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Sample records for atrioventricular septal defect

  1. Unbalanced atrioventricular septal defect: definition and decision making.

    Science.gov (United States)

    Overman, David M; Baffa, Jeanne M; Cohen, Meryl S; Mertens, Luc; Gremmels, David B; Jegatheeswaram, Anusha; McCrindle, Brian W; Blackstone, Eugene H; Morell, Victor O; Caldarone, Christopher; Williams, William G; Pizarro, Christian

    2010-04-01

    Unbalanced atrioventricular septal defect is an uncommon lesion with widely varying anatomic manifestations. When unbalance is severe, diagnosis and treatment is straightforward, directed toward single-ventricle palliation. Milder forms, however, pose a challenge to current diagnostic and therapeutic approaches. The transition from anatomies that are capable of sustaining biventricular physiology to those that cannot is obscure, resulting in uneven application of surgical strategy and excess mortality. Imprecise assessments of ventricular competence have dominated clinical decision making in this regard. Malalignment of the atrioventricular junction and its attendant derangement of inflow physiology is a critical factor in determining the feasibility of biventricular repair in the setting of unbalanced atrioventricular septal defect. The atrioventricular valve index accurately identifies unbalanced atrioventricular septal defect and also brings into focus a zone of transition from anatomies that can support a biventricular end state and those that cannot.

  2. Long-term survival in children with atrioventricular septal defect and common atrioventricular valvar orifice in Sweden.

    Science.gov (United States)

    Frid, Christina; Björkhem, Gudrun; Jonzon, Anders; Sunnegårdh, Jan; Annerén, Göran; Lundell, Bo

    2004-02-01

    The survival for patients with atrioventricular septal defect has improved markedly over the last decades and, during the same period, the survival of children with Down's syndrome has also increased. The aim of our study was to investigate long-term survival in patients having atrioventricular septal defect with common valvar orifice, but without associated significant congenital heart defects, in the setting of Down's syndrome, comparing the findings to those in chromosomally normal children with the same malformation. In a population-based retrospective study, we scrutinised the medical records from 801 liveborn children with atrioventricular septal defect born in Sweden during the period 1973 through 1997. Data on gender, presence or absence of Down's syndrome, associated congenital heart defects, date of birth, operation and death were recorded and followed up until 2001. An isolated atrioventricular septal defect with common atrioventricular valvar orifice was present in 502 children, of whom 86% had Down's syndrome. We found a significant reduc tion over time in age at operation, and in postoperative mortality at 30 days, from 28 to 1%. Using a multiple logistic regression model, we found no significant differences in mortality between genders, nor between those with or without Down's syndrome. Early corrective surgery could not be identified as a significant independent factor for survival. The 5-year postoperative survival in patients with Down's syndrome increased from 65% over the period from 1973 through 1977, to about 90% in the period 1993 through 1997, and the same trend was observed in chromosomally normal patients. Survival in uncomplicated atrioventricular septal defect with common atrioventricular valvar orifice has greatly increased, and surgical correction is now equally successful in patients with Down's syndrome and chromosomally normal patients, and for both genders. Death in connection with surgery is no longer the major threat, and focus

  3. Recent advances in managing septal defects: ventricular septal defects and atrioventricular septal defects [version 1; referees: 3 approved

    Directory of Open Access Journals (Sweden)

    P Syamasundar Rao

    2018-04-01

    Full Text Available This review discusses the management of ventricular septal defects (VSDs and atrioventricular septal defects (AVSDs. There are several types of VSDs: perimembranous, supracristal, atrioventricular septal, and muscular. The indications for closure are moderate to large VSDs with enlarged left atrium and left ventricle or elevated pulmonary artery pressure (or both and a pulmonary-to-systemic flow ratio greater than 2:1. Surgical closure is recommended for large perimembranous VSDs, supracristal VSDs, and VSDs with aortic valve prolapse. Large muscular VSDs may be closed by percutaneous techniques. A large number of devices have been used in the past for VSD occlusion, but currently Amplatzer Muscular VSD Occluder is the only device approved by the US Food and Drug Administration for clinical use. A hybrid approach may be used for large muscular VSDs in small babies. Timely intervention to prevent pulmonary vascular obstructive disease (PVOD is germane in the management of these babies. There are several types of AVSDs: partial, transitional, intermediate, and complete. Complete AVSDs are also classified as balanced and unbalanced. All intermediate and complete balanced AVSDs require surgical correction, and early repair is needed to prevent the onset of PVOD. Surgical correction with closure of atrial septal defect and VSD, along with repair and reconstruction of atrioventricular valves, is recommended. Palliative pulmonary artery banding may be considered in babies weighing less than 5 kg and those with significant co-morbidities. The management of unbalanced AVSDs is more complex, and staged single-ventricle palliation is the common management strategy. However, recent data suggest that achieving two-ventricle repair may be a better option in patients with suitable anatomy, particularly in patients in whom outcomes of single-ventricle palliation are less than optimal. The majority of treatment modes in the management of VSDs and AVSDs are safe

  4. Repair of tetralogy of Fallot associated with atrioventricular septal defect.

    Science.gov (United States)

    Tláskal, T; Hucín, B; Kostelka, M; Chaloupecký, V; Marek, J; Tax, P; Janouàek, J; Kuèera, V; Hruda, J; Reich, O; Skovránek, J

    1998-01-01

    Tetralogy of Fallot, when associated with atrioventricular septal defect permitting shunting at ventricular level, represents a complex cyanotic congenital malformation. Experience with surgical repair is limited, and results vary considerably. Between 1984 and 1996, we repaired 14 consecutive patients with this combination seen in our center. Their ages ranged from 8 months to 21 years (median 7.4 years). Six (42.9%) had Down's syndrome. In eight patients the correct diagnosis was made using echocardiography alone. In the remaining six patients, who had previously-constructed arterial shunts and/or suspected pulmonary arterial stenosis, catheterization and angiocardiography were also performed. The repair consisted of double patch closure of the septal defect, reconstruction of two atrioventricular orifices, and relief of pulmonary stenosis at all levels. In five patients with a hypoplastic pulmonary trunk, a monocusp transannular patch (four patients) or an allograft (one patient) was used for restoration of continuity from the right ventricle to the pulmonary arteries. Patch enlargement of one or both pulmonary arteries was necessary in five patients. One patient (7.1%) died early, and another late. The twelve surviving (85.8%) patients have been followed for 1.2-12.5 years after surgery (median 4.9 years, mean 5.9+/-3.9 years). During the follow-up, reoperation was necessary for repair of residual ventricular septal defect and pulmonary regurgitation in two patients, and closure of an atrial septal defect and alteration to left atrioventricular valvar regurgitation in one patient. Seven patients are in class I of the New York Heart Association, four in class II, and one in class III. Tetralogy of Fallot associated with atrioventricular septal defect can be corrected with low mortality and good long-term results. Residual lesions, however, have a tendency to progress, especially when seen in combination. After surgery, all patients need long-term close follow-up.

  5. The angulation of the septal structures impacts ventricular imbalance in atrioventricular septal defects with a common atrioventricular junction.

    Science.gov (United States)

    Ahmad, Zaheer; Lim, Zek; Roman, Kevin; Haw, Marcus; Anderson, Robert H; Vettukattil, Joseph

    2016-02-01

    Multiplanar re-formatting of full-volume three-dimensional echocardiography data sets offers new insights into the morphology of atrioventricular septal defects. We hypothesised that distortion of the alignment between the atrial and ventricular septums results in imbalanced venous return to the ventricles, with consequent proportional ventricular hypoplasia. A single observer evaluated 31 patients, with a mean age of 52.09 months, standard deviation of 55, and with a range from 2 to 264 months, with atrioventricular septal defects, of whom 17 were boys. Ventricular imbalance, observed in nine patients, was determined by two-dimensional assessment, and confirmed at surgical inspection in selected cases when a univentricular strategy was undertaken. Offline analysis using multiplanar re-formatting was performed. A line was drawn though the length of the ventricular septum and a second line along the plane of the atrial septum, taking the angle between these two lines as the atrioventricular septal angle. We compared the angle between 22 patients with adequately sized ventricles, and those with ventricular imbalance undergoing univentricular repair. In the 22 patients undergoing biventricular repair, the septal angle was 0 in 14 patients; the other eight patients having angles ranging from 1 to 36, with a mean angle of 7.4°, and standard deviation of 11.1°.The mean angle in the nine patients with ventricle imbalance was 28.6°, with a standard deviation of 3.04°, and with a range from 26 to 35°. Of those undergoing univentricular repair, two patients died, with angles of 26 and 30°, respectively. The atrioventricular septal angle derived via multiplanar formatting gives important information regarding the degree of ventricular hypoplasia and imbalance. When this angle is above 25°, patients are likely to have ventricular imbalance requiring univentricular repair.

  6. Factors associated with moderate or severe left atrioventricular valve regurgitation within 30 days of repair of incomplete atrioventricular septal defect

    Directory of Open Access Journals (Sweden)

    Marcelo Felipe Kozak

    2015-04-01

    Full Text Available AbstractIntroduction:Left atrioventricular valve regurgitation is the most concerning residual lesion after surgical correction of atrioventricular septal defect.Objective:To determine factors associated with moderate or greater left atrioventricular valve regurgitation within 30 days of surgical repair of incomplete atrioventricular septal defect.Methods:We assessed the results of 51 consecutive patients 14 years-old and younger presenting with incomplete atrioventricular septal defect that were operated on at our practice between 2002 and 2010. The following variables were considered: age, weight, absence of Down syndrome, grade of preoperative left atrioventricular valve regurgitation, abnormalities on the left atrioventricular valve and the use of annuloplasty. The median age was 4.1 years; the median weight was 13.4 Kg; 37.2% had Down syndrome. At the time of preoperative evaluation, there were 23 cases with moderate or greater left atrioventricular valve regurgitation (45.1%. Abnormalities on the left atrioventricular valve were found in 17.6%; annuloplasty was performed in 21.6%.Results:At the time of postoperative evaluation, there were 12 cases with moderate or greater left atrioventricular valve regurgitation (23.5%. The variation between pre- and postoperative grades of left atrioventricular valve regurgitation of patients with atrioventricular valve malformation did not reach significance (P=0.26, unlike patients without such abnormalities (P=0.016. During univariate analysis, only absence of Down syndrome was statistically significant (P=0.02. However, after a multivariate analysis, none of the factors reached significance.Conclusion:None of the factors studied was determinant of a moderate or greater left atrioventricular valve regurgitation within the first 30 days of repair of incomplete atrioventricular septal defect in the sample. Patients without abnormalities on the left atrioventricular valve benefit more of the operation.

  7. A rare form of atrioventricular septal defect with severe subaortic stenosis.

    Science.gov (United States)

    Venugopalan, P; Agarwal, A K; Reyes, Z

    2001-06-01

    An unusual form of atrioventricular septal defect associated with severe subaortic obstruction is reported in a neonate who presented with intractable cardiac failure. The baby had a large defect in the atrioventricular septum allowing communication from the left ventricle to the right atrium, without interatrial or interventricular communication, and a cleft anterior mitral leaflet. The baby expired despite palliative surgery performed to bypass the subaortic stenosis.

  8. Cardiac complications relating to pregnancy and recurrence of disease in the offspring of women with atrioventricular septal defects

    NARCIS (Netherlands)

    Drenthen, W; Pieper, PG; van der Tuuk, K; Roos-Hesselink, JW; Voors, AA; Mostert, B; Mulder, BJM; Moons, P; Ebels, T; van Veldhuisen, DJ

    2005-01-01

    Aims In most pregnancy reports, atrioventricular septal defects (AVSD) are not differentiated from more simple septal defects, thus underestimating the risks of pregnancy. To investigate the magnitude and determinants of risk during pregnancy in female patients with balanced AVSD. Methods and

  9. Deficiency of the vestibular spine in atrioventricular septal defects in human fetuses with down syndrome

    NARCIS (Netherlands)

    Blom, Nico A.; Ottenkamp, Jaap; Wenink, Arnold G. C.; Gittenberger-de Groot, Adriana C.

    2003-01-01

    Data on the morphogenesis of atrioventricular septal defect (AVSD) in Down syndrome are lacking to support molecular studies on Down syndrome heart critical region. Therefore, we studied the development of complete AVSD in human embryos and fetuses with trisomy 21 using 3-dimensional graphic

  10. Surgical anatomy of the atrioventricular conduction bundle in anomalous muscle bundle of the right ventricle with subarterial ventricular septal defect

    NARCIS (Netherlands)

    Kurosawa, H.; Becker, A. E.

    1985-01-01

    A stillborn baby girl was found to have an anomalous muscle bundle of the right ventricle, associated with a doubly committed subarterial ventricular septal defect. The latter was separated from the area of the atrioventricular conduction bundle by muscle. Serial histologic sectioning of the

  11. Disturbed Intracardiac Flow Organization After Atrioventricular Septal Defect Correction as Assessed With 4D Flow Magnetic Resonance Imaging and Quantitative Particle Tracing

    NARCIS (Netherlands)

    Calkoen, Emmeline E.; de Koning, Patrick J. H.; Blom, Nico A.; Kroft, Lucia J. M.; de Roos, Albert; Wolterbeek, Ron; Roest, Arno A. W.; Westenberg, Jos J. M.

    2015-01-01

    Objectives Four-dimensional (3 spatial directions and time) velocity-encoded flow magnetic resonance imaging with quantitative particle tracing analysis allows assessment of left ventricular (LV) blood flow organization. Corrected atrioventricular septal defect (AVSD) patients have an abnormal left

  12. Surgical treatment of atrial and ventricular septal defects after unsuccessful interventional therapy: a retrospective analysis

    International Nuclear Information System (INIS)

    Han Hongguang; Zhang Nanbin; Wang Zengwei; Wang Huishan; Zhu Hongyu; Li Xinmin

    2010-01-01

    Objective: To investigate the causes of failure in treating atrial septal defect (ASD) and ventricular septal defect (VSD) with interventional procedures and to evaluate the clinical efficacy of surgical treatment in order to increase the successful rate. Methods: A total of 13 patients, who underwent surgical therapy because of unsuccessful interventional treatment for ASD or VSD during the period of January 2001-December 2007, were selected,and the clinical data were retrospectively analyzed. The surgical indications included the occluder abscission (n=7), III degree atrioventricular conduction block (n=3), valvular regurgitation (n=2), residual shunt (n=1) and interventional failure (n=1). The cardiac surgeries, including removal of the displaced occluder and / or the repair of atrioventricular septal defects, were performed with the help of cardiopulmonary bypass in all 13 cases. After surgical treatment, all patients were transferred into ICU for further supervision and treatment. Results: The average diameter of ASD on surgical exploration was 31 mm, which was greater than the preoperative average diameter (26 mm), with a significant difference (P 0.05). The III degree atrioventricular conduction block in 3 cases restored sinus rhythm after operation. All the procedures were successfully completed in all patients. No death occurred during the hospitalization period. Conclusion: Proper and timely cardiac surgery is an effective and safe measure for the treatment of the complications due to unsuccessful interventional therapy as well as the atrioventricular septal defect itself. (authors)

  13. Evaluation of atrial, ventricular and atrioventricular septal defects by cine magnetic resonance imaging

    International Nuclear Information System (INIS)

    Akagi, Teiji; Kato, Hirohisa; Kiyomatsu, Yumi; Saiki, Kuninobu; Suzuki, Kazushige; Eto, Takaharu

    1992-01-01

    Cine magnetic resonance imaging (MRI) was performed on 20 patients (mean age: 5.3±4.4 years) with atrial, ventricular, or atrioventricular septal defects for evaluation of cardiac structure and blood flow. Prior to cine MRI, electrocardiographycally gated MRI using multislice scquisition was performed on all patients to localize optimal slice location. Cine-MRI was obtained with a 30 deg flip angle, 15 msec echo time, and 30 msec pulse repetition time, on a 256 x 256 or 128 x 128 acquisition matrix. Abnormalities of cardiac structure were well defined in all patients by gated cardiac imaging. In 18 of the 20 patients, cine-MRI was able to detect shunt flow, visualized as a low intensity signal in comparison with the surrounding blood flow. Cine-MRI can provide not only accurate anatomy of cardiac structures but functional assessment of the cardiac chamber, wall topology and flow relations. Cine-MRI will become an important noninvasive technique for assessment of anatomy and physiology in congenital heart disease. (author)

  14. Análise dos fatores de risco na correção cirúrgica do defeito septal atrioventricular de forma total Risk factors analysis in the surgical repair of complete atrioventricular septal defect

    Directory of Open Access Journals (Sweden)

    Eduardo Keller Saadi

    1993-06-01

    Full Text Available Pacientes com defeito septal atrioventricular de forma total (DSAVT freqüentemente apresentam insuficiência cardíaca intratável e hipertensão arterial pulmonar nos primeiros meses de vida, e apenas uma minoria sobrevive sem tratamento cirúrgico precoce. Por essa razão, indica-se a correção definitiva para alterar favoravelmente a história natural da doença. Entretanto, vários fatores são responsáveis pela alta mortalidade cirúrgica. O presente trabalho estuda a experiência na correção cirúrgica do DSAVT com o objetivo de identificar alguns fatores de risco estatisticamente significativos para a ocorrência de morte operatória. Analisaram-se, retrospectivamente, 52 pacientes submetidos, entre janeiro de 1974 e dezembro de 1990, a cirurgia definitiva para correção de DSAVT no Royal Brompton and National Heart and Lung Institute, sendo estudadas as seguintes variáveis: idade, peso, sexo, ano da operação, presença de síndrome de Down, grau de regurgitação da valva AV, bandagem prévia do tronco pulmonar, presença de anomalias associadas, pressão sistólica pulmonar, duplo orifício mitral, classificação do defeito segundo Rastelli, emprego de parada circulatória e técnica de correção (1 x 2 retalhos. Todos os fatores foram avaliados isoladamente, mediante a análise univariada. Para determinar quais os fatores que, independentemente da ação de outros, contribuíram significativamente para maior mortalidade cirúrgica, foi utilizada a análise multivariada com regressão logística. A análise multivariada demonstrou que o baixo peso na época da operação e a técnica de correção com um retalho aumentam significativamente a mortalidade cirúrgica.Patients with complete atrioventricular septal defects (CAVSD frequently present with severe heart failure which cannot be controllable medically and pulmonary hypertension in infancy. Just a small number survives without early surgical treatment. For this reason

  15. Angiographic differentiation of type of ventricular septal defects

    International Nuclear Information System (INIS)

    Cheon, Mal Soon; Park, Hee Young; Kim, Yang Sook

    1989-01-01

    Defects of the ventricular septum are the commonest type of congenital cardiac malformations. A classification with axial angiography of the subtypes of ventricular septal defects is proposed on the study of 126 patients with defects of the ventricular septum. The results were as follows: 1. The incidence of the ventricular septal defects was 39.6% of congenital heart malformation. 2. The sex distribution of cases were 70 males and 56 females, the age ranged from 13 months to 26 years. 3. Angiographic features seen by axial angiography were as follows: a. Perimembranous defects as seen on long axial view of left ventriculogram were in continuity wity aortic valve. The relation of the defect to the tricuspid valve allows distinction of the extension of the preimembranous defect toward inlet, trabecular, or infundibular zones. This relation was determined angiographically, using the course of the contrast medium from the left ventricle through the ventricular septal defect, opacifying the right ventricle. In inlet excavation, the shunted blood opacified the recess between septal leaflet of tricuspid valve and interventricular septum in early phase, in infundibular excavation, opacified the recess between anterior leaflet of tricuspid valve and anterior free wall of right ventricle and in trabecular excavation, the shunted blood traversed anterior portion of tricuspid valve ring, opacified trabecular portion of right ventricle. b. Muscular defects were separated from the semilunar and atrioventricular valves. c, Subarterial defects were related to both semilunar valves, and they were best demonstrated on the elongated right anterior oblique view of the left ventriculogram. d. Total infundibular defects were profiled in right anterior oblique 30 and long axial view, subaortic in location in both views

  16. Echocardiographic definition and surgical decision-making in unbalanced atrioventricular septal defect: a Congenital Heart Surgeons' Society multiinstitutional study.

    Science.gov (United States)

    Jegatheeswaran, Anusha; Pizarro, Christian; Caldarone, Christopher A; Cohen, Meryl S; Baffa, Jeanne M; Gremmels, David B; Mertens, Luc; Morell, Victor O; Williams, William G; Blackstone, Eugene H; McCrindle, Brian W; Overman, David M

    2010-09-14

    Although identification of unbalanced atrioventricular septal defect (AVSD) is obvious when extreme, exact criteria to define the limits of unbalanced are not available. We sought to validate an atrioventricular valve index (AVVI) (left atrioventricular valve area/total atrioventricular valve area, centimeters squared) as a discriminator of balanced and unbalanced forms of complete AVSD and to characterize the association of AVVI with surgical strategies and outcomes. Diagnostic echocardiograms and hospital records of 356 infants with complete AVSD at 4 Congenital Heart Surgeons' Society (CHSS) institutions (2000-2006) were reviewed and AVVI measured (n=315). Patients were classified as unbalanced if AVVI≤0.4 (right dominant) or ≥0.6 (left dominant). Surgical strategy and outcomes were examined across the range of AVVI. Competing risks analysis until the time of commitment to a surgical strategy examined 4 end states: biventricular repair (BVR), univentricular repair (UVR), pulmonary artery banding (PAB), and death before surgery. A prediction nomogram for surgical strategy based on AVVI was developed. The majority of patients had balanced AVSD (0.4

  17. Complete atrioventricular canal.

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    Calabrò, Raffaele; Limongelli, Giuseppe

    2006-04-05

    Complete atrioventricular canal (CAVC), also referred to as complete atrioventricular septal defect, is characterised by an ostium primum atrial septal defect, a common atrioventricular valve and a variable deficiency of the ventricular septum inflow. CAVC is an uncommon congenital heart disease, accounting for about 3% of cardiac malformations. Atrioventricular canal occurs in two out of every 10,000 live births. Both sexes are equally affected and a striking association with Down syndrome was found. Depending on the morphology of the superior leaflet of the common atrioventricular valve, 3 types of CAVC have been delineated (type A, B and C, according to Rastelli's classification). CAVC results in a significant interatrial and interventricular systemic-to-pulmonary shunt, thus inducing right ventricular pressure and volume overload and pulmonary hypertension. It becomes symptomatic in infancy due to congestive heart failure and failure to thrive. Diagnosis of CAVC might be suspected from electrocardiographic and chest X-ray findings. Echocardiography confirms it and gives anatomical details. Over time, pulmonary hypertension becomes irreversible, thus precluding the surgical therapy. This is the reason why cardiac catheterisation is not mandatory in infants (less than 6 months) but is indicated in older patients if irreversible pulmonary hypertension is suspected. Medical treatment (digitalis, diuretics, vasodilators) plays a role only as a bridge toward surgery, usually performed between the 3rd and 6th month of life.

  18. Complete atrioventricular canal

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    Limongelli Giuseppe

    2006-04-01

    Full Text Available Abstract Complete atrioventricular canal (CAVC, also referred to as complete atrioventricular septal defect, is characterised by an ostium primum atrial septal defect, a common atrioventricular valve and a variable deficiency of the ventricular septum inflow. CAVC is an uncommon congenital heart disease, accounting for about 3% of cardiac malformations. Atrioventricular canal occurs in two out of every 10,000 live births. Both sexes are equally affected and a striking association with Down syndrome was found. Depending on the morphology of the superior leaflet of the common atrioventricular valve, 3 types of CAVC have been delineated (type A, B and C, according to Rastelli's classification. CAVC results in a significant interatrial and interventricular systemic-to-pulmonary shunt, thus inducing right ventricular pressure and volume overload and pulmonary hypertension. It becomes symptomatic in infancy due to congestive heart failure and failure to thrive. Diagnosis of CAVC might be suspected from electrocardiographic and chest X-ray findings. Echocardiography confirms it and gives anatomical details. Over time, pulmonary hypertension becomes irreversible, thus precluding the surgical therapy. This is the reason why cardiac catheterisation is not mandatory in infants (less than 6 months but is indicated in older patients if irreversible pulmonary hypertension is suspected. Medical treatment (digitalis, diuretics, vasodilators plays a role only as a bridge toward surgery, usually performed between the 3rd and 6th month of life.

  19. Familial Atrial Septal Defect and Sudden Cardiac Death

    DEFF Research Database (Denmark)

    Ellesøe, Sabrina Gade; Johansen, Morten Munk; Bjerre, Jesper Vandborg

    2016-01-01

    OBJECTIVE: Atrial septal defect (ASD) is the second most common congenital heart defect (CHD) and is observed in families as an autosomal dominant trait as well as in nonfamilial CHD. Mutations in the NKX2-5 gene, located on chromosome 5, are associated with ASD, often combined with conduction...... disturbances, cardiomyopathies, complex CHD, and sudden cardiac death as well. Here, we show that NKX2-5 mutations primarily occur in ASD patients with conduction disturbances and heritable ASD. Furthermore, these families are at increased risk of sudden cardiac death. RESULTS: We screened 39 probands...... with familial CHD for mutations in NKX2-5 and discovered a novel mutation in one family (2.5%) with ASD and atrioventricular block. A review of the literature revealed 59 different NKX2-5 mutations in 202 patients. Mutations were significantly more common in familial cases compared to nonfamilial cases (P = 7...

  20. Altered left ventricular vortex ring formation by 4-dimensional flow magnetic resonance imaging after repair of atrioventricular septal defects.

    Science.gov (United States)

    Calkoen, Emmeline E; Elbaz, Mohammed S M; Westenberg, Jos J M; Kroft, Lucia J M; Hazekamp, Mark G; Roest, Arno A W; van der Geest, Rob J

    2015-11-01

    During normal left ventricular (LV) filling, a vortex ring structure is formed distal to the left atrioventricular valve (LAVV). Vortex structures contribute to efficient flow organization. We aimed to investigate whether LAVV abnormality in patients with a corrected atrioventricular septal defect (AVSD) has an impact on vortex ring formation. Whole-heart 4D flow MRI was performed in 32 patients (age: 26 ± 12 years), and 30 healthy subjects (age: 25 ± 14 years). Vortex ring cores were detected at peak early (E-peak) and peak late filling (A-peak). When present, the 3-dimensional position and orientation of the vortex ring was defined, and the circularity index was calculated. Through-plane flow over the LAVV, and the vortex formation time (VFT), were quantified to analyze the relationship of vortex flow with the inflow jet. Absence of a vortex ring during E-peak (healthy subjects 0%, vs patients 19%; P = .015), and A-peak (healthy subjects 10% vs patients 44%; P = .008) was more frequent in patients. In 4 patients, this was accompanied by a high VFT (5.1-7.8 vs 2.4 ± 0.6 in healthy subjects), and in another 2 patients with abnormal valve anatomy. In patients compared with controls, the vortex cores had a more-anterior and apical position, closer to the ventricular wall, with a more-elliptical shape and oblique orientation. The shape of the vortex core closely resembled the valve shape, and its orientation was related to the LV inflow direction. This study quantitatively shows the influence of abnormal LAVV and LV inflow on 3D vortex ring formation during LV inflow in patients with corrected AVSD, compared with healthy subjects. Copyright © 2015. Published by Elsevier Inc.

  1. Percutaneous treatment of atrial septal defects, muscular ventricular septal defects and patent ductus arteriosus in infants under one year of age.

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    Prada, Fredy; Mortera, Carlos; Bartrons, Joaquim; Rissech, Miguel; Jiménez, Lorenzo; Carretero, Juan; Llevadias, Judit; Araica, Mireya

    2009-09-01

    Amplatzer devices are used for the percutaneous closure of ostium secundum atrial septal defects, muscular ventricular septal defects and patent ductus arteriosus. However, very little experience has been gained in using these devices in infants under 1 year of age. Between January 2001 and January 2008, 22 symptomatic infants aged under 1 year underwent percutaneous treatment: three had an ostium secundum atrial septal defect, 15 had patent ductus arteriosus, and four had a muscular ventricular septal defect. All the procedures were completed successfully. No immediate or medium-term complications were observed. Closure of these types of defect using an Amplatzer device in infants under 1 year of age, who would otherwise require surgery, is a safe and effective procedure.

  2. Defeito completo do septo atrioventricular com cianose Complete repair in total atrioventricular canal defect with cyanosis

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    Carla Tanamati

    2006-09-01

    Full Text Available Os defeitos do septo atrioventricular total (DSAVT representam 4% das mal formações cardíacas e acima de 50% dos defeitos observados na síndrome de Down (SD¹. A apresentação clínica é de insuficiência cardíaca precoce na infância e hipertensão pulmonar por hiperfluxo. Raramente a cianose é observada e sugere hipertensão pulmonar ou associação à tetralogia de Fallot³, dupla via de saída de ventrículo direito², anomalia de Ebstein4, drenagem anômala de cava esquerda persistente em átrio esquerdo (Barbero Marcial, comunicação pessoal. Crianças com SD são particularmente difíceis de avaliação por apresentarem obstrução de vias aéreas superiores, que podem contribuir com o aumento da resistência pulmonar observada no cateterismo cardíaco. A presença de cianose pré-operatória constitui-se um desafio ao tratamento cirúrgico devido ao risco de hipertensão pulmonar irreversível com falência ventricular direita com, a correção dos defeitos intracardíacos.Atrioventricular septal defects account for 4% of congenital cardiac malformations and over 50% of cardiac defects seen in Down syndrome¹. Clinical presentation is marked by congestive heart failure early in infancy. Cyanosis is rarely found in infants and suggests irreversible pulmonary hypertension or associated cardiac defects as tetralogy of Fallot, double outlet right ventricle², Ebstein anomaly³, persistent left superior vena cava draining in the left atrium (Barbero Marcial, personal communication. Children with Down's syndrome is particularly difficult to assess because they often suffer from upper airways obstruction4, which may contribute to the increased pulmonary vascular resistance determined at cardiac catheterization. This association of factors becomes a challenge for operability and, we will report one such case.

  3. Endocardial cushion defect

    Science.gov (United States)

    ... Philadelphia, PA: Elsevier; 2016:chap 426. Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Atrioventricular septal defect. In: Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK, eds. Kirklin/Barratt- ...

  4. Multiplanar transesophageal echocardiography for the evaluation and percutaneous management of ostium secundum atrial septal defects in the adult.

    Science.gov (United States)

    Sobrino, Ayax; Basmadjian, Arsène J; Ducharme, Anique; Ibrahim, Reda; Mercier, Lise-Andrée; Pelletier, Guy B; Marcotte, François; Garceau, Patrick; Burelle, Denis; O'Meara, Eileen; Dore, Annie

    2012-01-01

    The purpose of this paper is to review the usefulness of multiplanar transesophageal echocardiography before, during and after percutaneous transcatheter closure of secundum atrial septal defects. Transesophageal echocardiography imaging techniques,including their role in patient selection, procedural guidance and immediate assessment of technical success and complications are described and discussed in this review. Percutaneous transcatheter closure is indicated for ostium secundum atrial septal defects of less than 40 mm in maximal diameter. The defect must have a favorable anatomy, with adequate rims of at least 5 mm to anchor the prosthesis. Transesophageal echocardiography plays a critical role before the procedure in identifying potential candidates for percutaneous closure and to exclude those with unfavorable anatomy or associated lesions, which could not be addressed percutaneously. Transesophageal echocardiography is also important during the procedure to guide the deployment of the device. After device deployment, the echocardiographer must assess the device (integrity, position and stability), residual shunt, atrio-ventricular valve regurgitation, obstruction to systemic or venous return and pericardial effusion, in order to determine procedural success and diagnose immediate complications.

  5. Ventricular Septal Defect (VSD)

    Science.gov (United States)

    ... Call your doctor if your baby or child: Tires easily when eating or playing Is not gaining ... heart procedures. Risk factors Ventricular septal defects may run in families and sometimes may occur with other ...

  6. [Infections as a preoperative problem in patients with tetralogy of Fallot (TOF) associated with complete atrioventricular canal septal defect (AVC)].

    Science.gov (United States)

    Szydłowski, Lesław; Marek-Szydłowska, Teresa; Rudziński, Andrzej; Pajak, Jacek; Morka, Jacek; Stołtny, Ludwik

    2004-01-01

    Tetralogy of Fallot (TOF) coexisting with atrioventricular canal septal defect (AVC) is a rare combination of anomalies. Additionally, in cases with concomitant Down syndrome, recurrent infections can be a serious problem in patients (pts.) waiting for cardiosurgery treatment. The purpose of the study was an analysis of types of infections and other factors complicating the preoperative period in those patients. The study group consisted of 17 pts. with TOF and AVC aged from 1 day to 9 years (mean 9.4 month). In this group there were 11 pts. with Down syndrome. All of them were subjected to physical examinations, blood analysis, ECG, chest X-ray and echocardiographic study. Additionally, in 8 pts. we performed catheterization. The signs of different types of infection were analyzed and results were compared in two groups: with and without Down syndrome. The differences were observed in the frequency of recurrent or chronic infections (21 v/s 4), time of hospitalization before surgery (17 v/s 9 days), necessity (11 v/s 3) and duration of antibiotic therapy (19 v/s 7 days) in the two studied groups. Elevated body temperature of unknown etiology was noted in 8 cases with Down syndrome, compared to 1 patient without trisomy 21. Also, the children with Down syndrome had to wait 11 days longer (19 v/s 8) for discharge after operation. Infections in children with TOF, AVC and Down syndrome significantly complicate the natural course of this anomaly. Prolonged preoperation time is characteristic of Down syndrome pts. compared to patients without chromosomal abnormalities.

  7. Exclusive double outlet right ventricle with atrioventricular concordance and pulmonary stenosis. Results of reconstructive surgery.

    Science.gov (United States)

    Busquet, J; Fontan, F; Choussat, A; Caianiello, G; Fernandez, G

    1988-01-01

    Double outlet right ventricle associated with atrioventricular concordance, pulmonary stenosis and situs solitus of the atria is a subset of double outlet right ventricle related through the surgical treatment. From 1974 to 1985, 14 patients, 5 males, 9 females (mean age 8.9 years, range 13 months-22 years) were operated upon. All patients had infundibular stenosis and normal or large pulmonary arteries. The apex of the heart was to the right in 2 patients, the right and left ventricles were superior and inferior in 2 patients and 1 patient had both anomalies. The ventricular septal defect was subaortic in 11 patients (aorto-mitral discontinuity in 5) and non-committed in 3 patients. Three patients had 2 ventricular septal defects. The aorta was anterior in 3 patients and to the right of the pulmonary artery in 11 patients. All patients, through a transventricular and transatrial approach, had a reconstructive surgery. In 3 patients, an aortic homograft valved conduit was used. One patient had the ventricular septal defect enlarged. There was one early death (7.1%) from high residual right ventricle pressure and no late death. One patient had a transient atrioventricular block. One patient was reoperated upon for a residual ventricular septal defect. All survivors had a good clinical result. Re-evaluation in 8 patients confirmed excellent haemodynamics: the right ventricle to pulmonary artery pressure gradient decreased from 80 mm Hg (range 60-95) preoperatively to 24 mm Hg (range 3-32) postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)

  8. Facts about Congenital Heart Defects

    Science.gov (United States)

    ... types of CHDs. The types marked with a star (*) are considered critical CHDs. Atrial Septal Defect Atrioventricular ... for Disease Control and Prevention Email Recommend Tweet YouTube Instagram Listen Watch RSS ABOUT About CDC Jobs ...

  9. Transcatheter closure of membranous ventricular septal defects with home-made nitinol occluder

    Energy Technology Data Exchange (ETDEWEB)

    Yongwen, Qin; Xianxian, Zhao; Xing, Zheng; Jijun, Ding; Jiang, Cao [Second Military Medical Univ., Shanghai (China). Changhai Hospital, Dept. of Cardiology

    2004-04-01

    Objective: To evaluate the feasibility and efficacy of transcatheter perimembranous ventricular septal defects (VSD) occlusion with home-made nitinol occluder. Methods: Transcatheter closure was attempted in 196 patients with perimembranous VSD. The diameter of VSD measured by echocardiography was 3 to 15 mm, mean (4.94 {+-} 2.23) mm. The angiographic diameter of the VSD was 3 to 6 mm, mean (3.92 {+-} 1.44) mm. A 7-10 F delivery sheath was advanced across the perimembranous VSD over a wire from femoral vein to deploy the occluder with the guidance of echocardiography and fluoroscopy. The device diameter selected was from 4 to 20 mm, mean (6.68 {+-} 2.76) mm. Left ventriculography and transthoracic echocardiography were repeated to assess the closure of the defects 15 min after the procedure. Continuous electrocardiogram monitoring lasted for 5 days. The echocardiography and electrocardiogram examination were scheduled for 1, 6 and 12 months of follow-up. Results: The occluders were successfully deployed in 191 patients. There were five procedural failures, two with device-related aortic insufficiency, and three of inability to pass through VSD. After deployment of the devices, there were no residual shunt in 180 of 191 patients, 11 patients with a trivial residual shunt that disappeared in 8 patients after one month of follow up. 3 patients developed mild tricuspid insufficiency. 12 developed transient complete right bundle branch block, and 5 transient complete left bundle branch block, and 2 transient complete atrioventricular block. There were repetitive nonparoxysmal ventricular tachycardia in 4 patients 1 week after the procedure. One patient had a detached device embolized into the left pulmonary artery but with a successful catheter retrieval by snare and transcatheter closure. The devices were similarly applied to patients with VSD associated with patent ductus arteriosus, and 4 patients with VSD complicated by atrial septal defects. The fluoroscopy time

  10. Eisenmenger ventricular septal defect in a Humboldt penguin (Spheniscus humboldti).

    Science.gov (United States)

    Laughlin, D S; Ialeggio, D M; Trupkiewicz, J G; Sleeper, M M

    2016-09-01

    The Eisenmenger ventricular septal defect is an uncommon type of ventricular septal defect characterised in humans by a traditionally perimembranous ventricular septal defect, anterior deviation (cranioventral deviation in small animal patients) of the muscular outlet septum causing malalignment relative to the remainder of the muscular septum, and overriding of the aortic valve. This anomaly is reported infrequently in human patients and was identified in a 45-day-old Humboldt Penguin, Spheniscus humboldti, with signs of poor growth and a cardiac murmur. This case report describes the findings in this penguin and summarises the anatomy and classification of this cardiac anomaly. To the authors' knowledge this is the first report of an Eisenmenger ventricular septal defect in a veterinary patient. Copyright © 2016 Elsevier B.V. All rights reserved.

  11. Atrial septal defect in a Korean wild raccoon dog.

    Science.gov (United States)

    Yim, Soomi; Choi, Sooyoung; Kim, Jongtaek; Chung, Jin-Young; Park, Inchul

    2017-10-07

    An approximately two-year-old, male 6.1 kg body weight, Korean wild raccoon dog (Nyctereutes procyonoides koreensis) was captured by the wildlife medical rescue center of Kangwon National University. Upon physical examination, the heart rate was 87 beats per min and there were no clinical signs. The hematological, and blood biochemical profiles revealed no remarkable findings; however, thoracic radiographs showed cardiac enlargement, especially in the right atrium. On electrocardiogram, sinus node dysfunction and bradyarrhythmia were revealed. Echocardiography showed a left-to-right shunting atrial septal defect. Based on these findings, this Korean wild raccoon dog was diagnosed with atrial septal defect. This is the rare case report of atrial septal defect in wildlife.

  12. Ventricular septal defect closure in a patient with achondroplasia.

    Science.gov (United States)

    Nakanishi, Keisuke; Kawasaki, Shiori; Amano, Atsushi

    2017-01-01

    Achondroplasia with co-morbid CHD is rare, as are reports of surgical treatment for such patients. We present the case of a 13-year-old girl with achondroplasia and ventricular septal defect. Her ventricular septal defect was surgically repaired focussing on the cardiopulmonary bypass flow, healing of the sternum, and her frail neck cartilage. The surgery and recovery were without complications.

  13. Classification of Atrial Septal Defect and Ventricular Septal Defect with Documented Hemodynamic Parameters via Cardiac Catheterization by Genetic Algorithms and Multi-Layered Artificial Neural Network

    Directory of Open Access Journals (Sweden)

    Mustafa Yıldız

    2012-08-01

    Full Text Available Introduction: We aimed to develop a classification method to discriminate ventricular septal defect and atrial septal defect by using severalhemodynamic parameters.Patients and Methods: Forty three patients (30 atrial septal defect, 13 ventricular septal defect; 26 female, 17 male with documentedhemodynamic parameters via cardiac catheterization are included to study. Such parameters as blood pressure values of different areas,gender, age and Qp/Qs ratios are used for classification. Parameters, we used in classification are determined by divergence analysismethod. Those parameters are; i pulmonary artery diastolic pressure, ii Qp/Qs ratio, iii right atrium pressure, iv age, v pulmonary arterysystolic pressure, vi left ventricular sistolic pressure, vii aorta mean pressure, viii left ventricular diastolic pressure, ix aorta diastolicpressure, x aorta systolic pressure. Those parameters detected from our study population, are uploaded to multi-layered artificial neuralnetwork and the network was trained by genetic algorithm.Results: Trained cluster consists of 14 factors (7 atrial septal defect and 7 ventricular septal defect. Overall success ratio is 79.2%, andwith a proper instruction of artificial neural network this ratio increases up to 89%.Conclusion: Parameters, belonging to artificial neural network, which are needed to be detected by the investigator in classical methods,can easily be detected with the help of genetic algorithms. During the instruction of artificial neural network by genetic algorithms, boththe topology of network and factors of network can be determined. During the test stage, elements, not included in instruction cluster, areassumed as in test cluster, and as a result of this study, we observed that multi-layered artificial neural network can be instructed properly,and neural network is a successful method for aimed classification.

  14. Transcatheter closure of membranous ventricular septal defects with home-made nitinol occluder

    International Nuclear Information System (INIS)

    Qin Yongwen; Zhao Xianxian; Zheng Xing; Ding Jijun; Cao Jiang

    2004-01-01

    Objective: To evaluate the feasibility and efficacy of transcatheter perimembranous ventricular septal defects (VSD) occlusion with home-made nitinol occluder. Methods: Transcatheter closure was attempted in 196 patients with perimembranous VSD. The diameter of VSD measured by echocardiography was 3 to 15 mm, mean (4.94 ± 2.23) mm. The angiographic diameter of the VSD was 3 to 6 mm, mean (3.92 ± 1.44) mm. A 7-10 F delivery sheath was advanced across the perimembranous VSD over a wire from femoral vein to deploy the occluder with the guidance of echocardiography and fluoroscopy. The device diameter selected was from 4 to 20 mm, mean (6.68 ± 2.76) mm. Left ventriculography and transthoracic echocardiography were repeated to assess the closure of the defects 15 min after the procedure. Continuous electrocardiogram monitoring lasted for 5 days. The echocardiography and electrocardiogram examination were scheduled for 1, 6 and 12 months of follow-up. Results: The occluders were successfully deployed in 191 patients. There were five procedural failures, two with device-related aortic insufficiency, and three of inability to pass through VSD. After deployment of the devices, there were no residual shunt in 180 of 191 patients, 11 patients with a trivial residual shunt that disappeared in 8 patients after one month of follow up. 3 patients developed mild tricuspid insufficiency. 12 developed transient complete right bundle branch block, and 5 transient complete left bundle branch block, and 2 transient complete atrioventricular block. There were repetitive nonparoxysmal ventricular tachycardia in 4 patients 1 week after the procedure. One patient had a detached device embolized into the left pulmonary artery but with a successful catheter retrieval by snare and transcatheter closure. The devices were similarly applied to patients with VSD associated with patent ductus arteriosus, and 4 patients with VSD complicated by atrial septal defects. The fluoroscopy time for the

  15. Mirror Image Gerbode or Partial Atrioventricular Canal Defect?

    Directory of Open Access Journals (Sweden)

    Cem Ariturk

    2015-12-01

    Full Text Available Gebode defect, that can accurately be treated surgical repair, is defined as a true communication between left ventricle and right atrium. A 74-year-old woman with a worsening history of ortophnea and peripheral edema was hospitalised. A communication between right atrium and left ventricle was diagnosed using transeusophageal echocardiography. The defect was repaired and mitral valve was replaced with a biologic valve. It would be beter to tailor surgical strategy for each case with atrioventricular canal defect after preoperative transeusophageal echocardiography and peroperative direct sight.

  16. Transcatheter closure of ventricular septal defect with Occlutech Duct Occluder.

    Science.gov (United States)

    Atik-Ugan, Sezen; Saltik, Irfan Levent

    2018-04-01

    Patent ductus arteriosus occluders are used for transcatheter closure of ventricular septal defects, as well as for closure of patent ductus arteriosus. The Occlutech Duct Occluder is a newly introduced device for transcatheter closure of patent ductus arteriosus. Here, we present a case in which the Occlutech Duct Occluder was successfully used on a patient for the closure of a perimembraneous ventricular septal defect.

  17. Expression of the BMP receptor Alk3 in the second heart field is essential for development of the dorsal mesenchymal protrusion and atrioventricular septation

    NARCIS (Netherlands)

    Briggs, Laura E.; Phelps, Aimee L.; Brown, Elizabeth; Kakarla, Jayant; Anderson, Robert H.; van den Hoff, Maurice J. B.; Wessels, Andy

    2013-01-01

    The dorsal mesenchymal protrusion (DMP) is a prong of mesenchyme derived from the second heart field (SHF) located at the venous pole of the developing heart. Recent studies have shown that perturbation of its development is associated with the pathogenesis of atrioventricular (AV) septal defect.

  18. Transcatheter device closure of perimembranous ventricular septal defect in children treated with prophylactic oral steroids: acute and mid-term results of a single-centre, prospective, observational study.

    Science.gov (United States)

    Thakkar, Bhavesh; Patel, Nehal; Bohora, Shomu; Bhalodiya, Dharmin; Singh, Tarandeep; Madan, Tarun; Shah, Saurin; Poptani, Vishal; Shukla, Anand

    2016-04-01

    Background and Objective Although transcatheter closure of perimembranous ventricular septal defect is emerging as an accepted, viable alternative, conduction disturbances still remain a major concern. Although steroid treatment has shown encouraging results with complete recovery, efficacy of prophylactic use of steroids is still speculative. We aim to study the mid-term outcome of perimembranous ventricular septal defect closure in children who received prophylactic oral steroids. Materials and methods A prospective study was designed and antegrade device closure was attempted in eligible children who met the following inclusion criteria: age 3-18 years and weight >10 kg, defect diameter ⩽12 mm, and symptomatic, haemodynamic changes or history of infective endocarditis. Prophylactic steroid protocol consisted of 2 weeks oral prednisolone (1 mg/kg/day) initiated immediately after the procedure, and in the event of bradyarrhythmia it was escalated to 2 mg/kg. Patients were regularly followed-up at 1, 6, and 12 months and then annually. Patients with post-procedure heart block underwent Holter monitoring after a minimum of 1 year interval. Between May, 2007 and August, 2012, successful device closure was accomplished in 290/297 patients. Mean age and weight were 9±3.12 years and 21±8.27 kg, respectively. The defect measured 5±1.38 mm on echocardiography. Mean fluoroscopy time was 12.98±8.64 minutes. Eight patients with major complications included one each with device embolisation, haemolysis, severe aortic regurgitation, and five with bradyarrhythmias, including complete atrioventricular block in three, Mobitz II in one, and bifascicular block in one. Patients with complete atrioventricular block responded to high-dose steroid and temporary pacemaker. Minor complications included post-procedure heart block (n=22) and blood loss (n=2). At 18.23±13.15 months follow-up, 8/27 (five major, 22 minor) with arrhythmia had persistent post-procedure heart block of no

  19. Right atrial isolation associated with atrial septal closure in patients with atrial septal defect and chronic atrial fibrillation.

    Science.gov (United States)

    Minzioni, G; Graffigna, A; Pagani, F; Vigano, M

    1993-12-01

    To restore sinus rhythm in the remaining heart chambers of six adult patients with atrial septal defect and chronic or paroxysmal atrial fibrillation, electrical, right atrial isolation associated with surgical correction of the defect was performed. All but one patient was free from atrial fibrillation without medication 2-25 months after operation. The isolated right atrial appendages showed intrinsic rhythmical activity in five patients and no electrical activity in one. Right atrial isolation is a safe and effective procedure that abolishes atrial fibrillation in patients with arrhythmia after surgical correction of atrial septal defect.

  20. Percutaneous closure of a post-traumatic ventricular septal defect with a patent ductus arteriosus occluder

    Directory of Open Access Journals (Sweden)

    Er-Ping Xi

    2012-11-01

    Full Text Available OBJECTIVE: Ventricular septal defects resulting from post-traumatic cardiac injury are very rare. Percutaneous closure has emerged as a method for treating this disorder. We wish to report our experience in three patients who underwent percutaneous closure of a post-traumatic ventricular septal defect with a patent ductus arteriosus occluder. METHODS: We treated three patients with post-traumatic ventricular septal defects caused by stab wounds with knives. After the heart wound was repaired, patient examinations revealed ventricular septal defects with pulmonary/systemic flow ratios (Qp/Qs of over 1.7. The post-traumatic ventricular septal defects were closed percutaneously with a patent ductus arteriosus occluder (Lifetech Scientific (Shenzhen Co., LTD, Guangdong, China utilizing standard techniques. RESULTS: Post-operative transthoracic echocardiography revealed no residual left-to-right shunt and indicated normal ventricular function. In addition, 320-slice computerized tomography showed that the occluder was well placed and exhibited normal morphology. CONCLUSION: Our experiences indicate that closure of a post-traumatic ventricular septal defect using a patent ductus arteriosus occluder is feasible, safe, and effective.

  1. Percutaneous closure of a post-traumatic ventricular septal defect with a patent ductus arteriosus occluder.

    Science.gov (United States)

    Xi, Er-Ping; Zhu, Jian; Zhu, Shui-Bo; Yin, Gui-Lin; Liu, Yong; Dong, Yong-Qiang; Zhang, Yu; Xia, Feng

    2012-11-01

    Ventricular septal defects resulting from post-traumatic cardiac injury are very rare. Percutaneous closure has emerged as a method for treating this disorder. We wish to report our experience in three patients who underwent percutaneous closure of a post-traumatic ventricular septal defect with a patent ductus arteriosus occluder. We treated three patients with post-traumatic ventricular septal defects caused by stab wounds with knives. After the heart wound was repaired, patient examinations revealed ventricular septal defects with pulmonary/systemic flow ratios (Qp/Qs) of over 1.7. The post-traumatic ventricular septal defects were closed percutaneously with a patent ductus arteriosus occluder (Lifetech Scientific (Shenzhen) Co., LTD, Guangdong, China) utilizing standard techniques. Post-operative transthoracic echocardiography revealed no residual left-to-right shunt and indicated normal ventricular function. In addition, 320-slice computerized tomography showed that the occluder was well placed and exhibited normal morphology. Our experiences indicate that closure of a post-traumatic ventricular septal defect using a patent ductus arteriosus occluder is feasible, safe, and effective.

  2. Tetralogy of Fallot with restrictive ventricular septal defect by accessory tricuspid leaflet tissue

    OpenAIRE

    Mahipat Raj Soni; Deepak A. Bohara; Ajay U. Mahajan; Pratap J. Nathani

    2012-01-01

    In tetralogy of Fallot septal defect is usually large because of malalignment of outlet septum, restrictive defect has been reported rarely. We present a case of tetralogy of Fallot with accessory tricuspid leaflet tissue restricting ventricular septal defect. The report includes echocardiographic and catheter images of this rare presentation of tetralogy of Fallot.

  3. Hybrid closure of atrial septal defect: A modified approach

    Directory of Open Access Journals (Sweden)

    Kshitij Sheth

    2015-01-01

    Full Text Available A 3.5-year-old girl underwent transcatheter closure of patent ductus arteriosus in early infancy during which time her secundum atrial septal defect (ASD was left alone. When she came for elective closure of ASD, she was found to have bilaterally blocked femoral veins. The defect was successfully closed with an Amplatzer septal occluder (ASO; St. Jude Medical, Plymouth, MN, USA using a hybrid approach via a sub-mammary mini-thoracotomy incision without using cardiopulmonary bypass. At the end of 1-year follow-up, the child is asymptomatic with device in a stable position without any residual shunt.

  4. Anatomy of a wrong diagnosis: false Sinus Venosus Atrial Septal Defect

    Directory of Open Access Journals (Sweden)

    Montresor Graziano

    2003-11-01

    Full Text Available Abstract In contrast with transthoracic echocardiography, transesophageal echocardiography provides a sure way to make the diagnosis of sinus venosus atrial septal defect; on the other hand this abnormality is more complex than that seen with the secundum atrial septal defect, and inexperienced operators may fail to recognize properly the defect. In front of a high reported sensitivity using transesophageal echocardiography, specificity is difficult to assess, due to possible underreporting of diagnostic errors. We describe a false positive diagnosis of sinus venosus atrial septal defect, in the setting of enlarged right chambers of the heart because of pressure overload. Modified anatomy of the heart, together with the presence of a prominent linear structure(probably Eustachian Valve and an incomplete examination in this case made image interpretation very prone to misinterpretation. In this anatomical setting transesophageal longitudinal "bicaval" view may be sub-optimal for examining the atrial septum, potentially showing false images that need to be known for correct image interpretation. Nonetheless, a scan plane taken more accurately at the superior level would have demonstrated/excluded the pathognomonic feature of sinus venosus atrial septal defect in the high atrial septum, between the fatty limbus and the inferior aspect of the right pulmonary artery; moreover TEE allows morphological information about the posterior structures of the heart that need to be investigated in detail for a complete diagnosis.

  5. Early closure of postinfarction ventricular septal defects.

    Science.gov (United States)

    Martinelli, Luigi; Dottori, Vincenzo; Caputo, Enrico; Graffigna, Angelo; Pederzolli, Carlo

    2003-05-01

    According to the guidelines of the American College of Cardiology/American Heart Association early closure of postinfarction septal defects is now a class I indication although it still carries a relevant morbidity and mortality. The operative risk is related both to the critical hemodynamic conditions of the patient and to the technical difficulties posed by the friable tissue of the infarcted area. The most recent techniques involving the use of pericardial patches reinforced by acrylic glue have significantly reduced the hospital mortality. The aim of this study was to discuss the reliability of an aggressive, tissue-sparing surgical approach to this complication. We present a consecutive series of 12 patients operated upon between January 1998 and October 2001 within 12 hours of the onset of clinical evidence of postinfarction septal rupture. Repair was achieved with minimal septal debridement and the use of a large pericardial patch reinforced by a biological glue. Three cases of dehiscence required early reoperation with no hospital mortality. This procedure is technically feasible and allows early aggressive treatment of postinfarction septal rupture with satisfactory results.

  6. Percutaneous closure of a post-traumatic ventricular septal defect with a patent ductus arteriosus occluder

    OpenAIRE

    Xi, Er-Ping; Zhu, Jian; Zhu, Shui-Bo; Yin, Gui-Lin; Liu, Yong; Dong, Yong-Qiang; Zhang, Yu; Xia, Feng

    2012-01-01

    OBJECTIVE: Ventricular septal defects resulting from post-traumatic cardiac injury are very rare. Percutaneous closure has emerged as a method for treating this disorder. We wish to report our experience in three patients who underwent percutaneous closure of a post-traumatic ventricular septal defect with a patent ductus arteriosus occluder. METHODS: We treated three patients with post-traumatic ventricular septal defects caused by stab wounds with knives. After the heart wound was repaired,...

  7. Changes in Serum Natriuretic Peptide Levels after Percutaneous Closure of Small to Moderate Ventricular Septal Defects

    Directory of Open Access Journals (Sweden)

    Yuksel Kaya

    2012-01-01

    Full Text Available Background. B-type natriuretic peptide has been shown to be a very sensitive and specific marker of heart failure. In this study, we aimed to investigate the effect of percutaneous closure of ventricular septal defects with Amplatzer septal occluders on brain natriuretic peptide levels. Methods. Between 2008 and 2011, 23 patients underwent successfully percutaneous ventricular septal defect closure in 4 cardiology centers. Brain natriuretic peptide levels were measured in nine patients (4 male, mean ages were 25.3±14.3 who underwent percutaneous closure with Amplatzer occluders for membranous or muscular ventricular septal defects were enrolled in the study. Brain natriuretic peptide levels were measured one day before and one month after the closure. Patients were evaluated clinically and by echocardiography one month after the procedure. Results. Percutaneous closures of ventricular septal defects were successfully performed in all patients. There was not any significant adverse event in patients group during followup. Decrease in brain natriuretic peptide levels after closure were statistically significant (97.3±78.6 versus 26.8±15.6, =0.013. Conclusion. Brain Natriuretic Peptide levels are elevated in patients with ventricular septal defects as compared to controls. Percutaneous closure of Ventricular Septal Defect with Amplatzer occluders decreases the BNP levels.

  8. Radiological evaluation of ventricular septal defect with aortic insufficiency - An analysis of cineangiography in 15 cases -

    International Nuclear Information System (INIS)

    Park, Jae Hyung; Yeon, Kyung Mo; Han, Man Chung

    1981-01-01

    Fifteen cases of ventricular septal defect with aortic insufficiency were diagnosed radiographically and confirmed after operation at Seoul National University Hospital in recent two half years since 1979. Cineangiographies of ascending aorta and left ventricle were done in those cases and revealed some characteristic findings. The results of the analysis are as follow: 1. Among the 15 cases, 14 cases were male and 1 case was female. Age distribution was from 7 years to 23 years. 2. Those 15 cases were corresponded to 8% among total 193 cases of ventricular septal defect, to 11% among total 135 cases of aortic insufficiency and especially to 48% among 48 cases of aortic insufficiency below age of 20 years. 3. After operation, 11 cases were confirmed as subpulmonary type ventricular septal defect and 4 cases as subcristal type. The sizes of the ventricular septal defects were ranged between 0.6 and 2.5 cm in diameter. 4. Regurgitation of contrast media was noticed in cine aortography of all cases, and the grades of regurgitation were II-III/IV in 13 cases. 5. Various types of herniated aortic cusp through ventricular septal defect were seen. In the cases of subpulmonary ventricular septal defect characteristic saccular aneurysm was found in 7 cases. Asymmetry or mild bulging of aortic sinus was found in the cases of subcristal ventricular septal defect. 6. Infundibular stenosis was found in 3 cases with right ventriculography and those were caused by the herniated saccular aneurysm of aortic cusp. 7. It is essential for the diagnosis of ventricular septal defect with aortic insufficiency to undertake biplane cineangiography of ascending aorta and left ventricle in long axial view and right ventriculography should be done in suspicion of infundibular pulmonary stenosis

  9. Atrioventricular conduction after alcohol septal ablation for obstructive hypertrophic cardiomyopathy

    DEFF Research Database (Denmark)

    Axelsson, Anna; Weibring, Kristina; Havndrup, Ole

    2014-01-01

    .1-9.4) after ASA. Patients with high-grade atrioventricular block at follow-up had longer PR intervals at baseline [205 ms (200-230)] than the rest of the cohort [180 ms (140-200), P = 0.004] and a higher incidence of acute complete heart block (63 vs. 15%; P = 0.007) during ASA. A PR interval of at least 200...... ms at baseline was associated with higher prevalence of high-grade atrioventricular block at follow-up (30 vs. 2%; P = 0.0013). The incidence of late-onset complete heart block was 1.5% per year after ASA. CONCLUSION: We found normalized atrioventricular conduction at long-term follow-up, suggesting...... recovery in 6 of 14 patients with a pacemaker implanted in relation to ASA. Permanent atrioventricular conduction abnormalities were associated with baseline PR intervals of at least 200 ms and acute persistent complete heart block during ASA....

  10. Cor triatriatum dexter and atrial septal defect in a 43-year-old woman.

    Science.gov (United States)

    Vukovic, Petar M; Kosevic, Dragana; Milicic, Miroslav; Jovovic, Ljiljana; Stojanovic, Ivan; Micovic, Slobodan

    2014-08-01

    Cor triatriatum dexter is a rare congenital heart anomaly in which a membrane divides the right atrium into 2 chambers. We report the case of a 43-year-old woman who had cor triatriatum dexter and a large atrial septal defect. During attempted percutaneous closure, the balloon disrupted the membrane and revealed that the defect had no inferior rim, precluding secure placement of an Amplatzer Septal Occluder. Surgical treatment subsequently proved to be successful. In patients with an incomplete membrane and a septal defect with well-defined rims, percutaneous treatment can be the first choice. In patients who have cor triatriatum dexter and unfavorable anatomic features or concomitant complex heart anomalies, open-heart surgery remains the gold standard for treatment.

  11. Surgical removal of atrial septal defect occlusion device and mitral valve replacement in a 39-year-old female patient with infective endocarditis

    Directory of Open Access Journals (Sweden)

    A S Zotov

    2018-02-01

    Full Text Available Atrial septal defects represent the second most frequent congenital heart disease after ventricular septal defects. Transcatheter closure of an atrial septal defect is usually performed following strict indications on patients with significant left-to-right shunt. Infective endocarditis after transcatheter implantation of atrial septal defect occluder is an extremely rare complication. We report a case of infective endocarditis of the mitral valve (with severe mitral valve insufficiency in a 39-year-old female patient 13 years after transcatheter closure of an atrial septal defect. Complex prophylactic antibiotic coverage was performed prior to surgical intervention. Surgical removal of atrial septal defect occluder, mitral valve replacement, atrial septal defect closure and left atrial appendage resection were performed. Postoperative course was uneventful.

  12. Doubly committed subarterial ventricular septal defect with prolapsed right coronary cusp with moderate aortic regurgitation

    Directory of Open Access Journals (Sweden)

    Redoy Ranjan

    2017-11-01

    Full Text Available A 4 year old girl was presented with the respiratory tract infection, breathlessness after taking meal, failure to thrive, abnormal movement of the chest on left side overlying the area of heart and systolic murmur. She developed these symptoms gradually for the last 3.5 years. Echocardiography revealed doubly committed subarterial ventricular septal defect with moderate aortic regurgitation. The size of the ventricular septal defect was 7 x 9 mm at the left ventricular outflow tract. The right coronary cusp of the aortic valve was prolapsed. Left atrium and left ventricle were dilated. The pulmonary artery systolic pressure was 35 mm Hg. The ventricular septal defect was closed with the standard surgical procedure using cardiopulmonary bypass followed by aortotomy and right atriotomy. Immediate post-operative period of this case was uneventful and the patient was discharged on 9th post-operative day. Follow-up echocardiography showed no residual ventricular septal defect or aortic regurgitation and the ventricular function was good.

  13. Aspects of surgery for congenital ventricular septal defect

    NARCIS (Netherlands)

    G. Bol-Raap (Goris)

    2007-01-01

    textabstractIn chapter 1, an outline of the thesis is given. This thesis focuses on aspects of surgical closure of a congenital ventricular septal defect. In Chapter 2, the accuracy and the potential of 3-D echocardiography in the preoperative assessment of a congenital VSD were evaluated. 3-D

  14. The Fate of the Tricuspid Valve Following the Transatrial Closure of the Ventricular Septal Defect.

    Science.gov (United States)

    Giordano, Raffaele; Cantinotti, Massimiliano; Di Tommaso, Luigi; Comentale, Giuseppe; Tozzi, Andrea; Pilato, Emanuele; Iannelli, Gabriele; Palma, Gaetano

    2018-05-17

    The transatrial repair of the ventricular septal defect (VSD) requires an adequate exposure of its rim. We retrospectively evaluated the impact of adopting the tricuspid valve incision (TVI) technique, either with detachment or radial incision, on the postoperative outcome of children undergoing surgical VSD repair. From January 2008 to September 2017 we retrospectively enrolled 141 patients, divided into two groups: 97 patients were subjected to TVI (68.8%) and 44 patients (31.2%) were not subjected to TVI. All patients received an echocardiogram upon discharge from the hospital and after 1 month, 3 months, 6 months and a year from the treatment. No perioperative or late deaths resulted in our dataset. TVI was associated with a slightly longer cardio-pulmonary bypass and cross-clamp time but there were no differences in the surgical outcome between the two groups. Moreover, no differences occurred concerning residual VSD, atrioventricular block or tricuspid regurgitation at discharge. Echocardiograms at follow-up were available for 134 patients (95%) with a median of 5.3 years (range 0.5-9.3) and the degree of tricuspid regurgitation did not differ between groups. No patient required reoperation for tricuspid regurgitation or residual interventricular shunt. Finally, no difference was found even when comparing the two TVI subgroups. TVI should be used whenever intraoperative exposure of VSD is compromised in order to avoid a residual shunt and atrioventricular block. Here we show that this procedure does not significantly compromise the tricuspid function although a large, multicenter, randomized controlled trial is advised to validate this hypothesis. Copyright © 2018. Published by Elsevier Inc.

  15. Cost-effectiveness of procedures for treatment of ostium secundum atrial septal defects occlusion comparing conventional surgery and septal percutaneous implant.

    Science.gov (United States)

    da Costa, Márcia Gisele Santos; Santos, Marisa da Silva; Sarti, Flávia Mori; Simões e Senna, Kátia Marie; Tura, Bernardo Rangel; Correia, Marcelo Goulart; Goulart, Marcelo Correia

    2014-01-01

    The study performs a cost-effectiveness analysis of procedures for atrial septal defects occlusion, comparing conventional surgery to septal percutaneous implant. A model of analytical decision was structured with symmetric branches to estimate cost-effectiveness ratio between the procedures. The decision tree model was based on evidences gathered through meta-analysis of literature, and validated by a panel of specialists. The lower number of surgical procedures performed for atrial septal defects occlusion at each branch was considered as the effectiveness outcome. Direct medical costs and probabilities for each event were inserted in the model using data available from Brazilian public sector database system and information extracted from the literature review, using micro-costing technique. Sensitivity analysis included price variations of percutaneous implant. The results obtained from the decision model demonstrated that the percutaneous implant was more cost effective in cost-effectiveness analysis at a cost of US$8,936.34 with a reduction in the probability of surgery occurrence in 93% of the cases. Probability of atrial septal communication occlusion and cost of the implant are the determinant factors of cost-effectiveness ratio. The proposal of a decision model seeks to fill a void in the academic literature. The decision model proposed includes the outcomes that present major impact in relation to the overall costs of the procedure. The atrial septal defects occlusion using percutaneous implant reduces the physical and psychological distress to the patients in relation to the conventional surgery, which represent intangible costs in the context of economic evaluation.

  16. Cost-effectiveness of procedures for treatment of ostium secundum atrial septal defects occlusion comparing conventional surgery and septal percutaneous implant.

    Directory of Open Access Journals (Sweden)

    Márcia Gisele Santos da Costa

    Full Text Available OBJECTIVES: The study performs a cost-effectiveness analysis of procedures for atrial septal defects occlusion, comparing conventional surgery to septal percutaneous implant. METHODS: A model of analytical decision was structured with symmetric branches to estimate cost-effectiveness ratio between the procedures. The decision tree model was based on evidences gathered through meta-analysis of literature, and validated by a panel of specialists. The lower number of surgical procedures performed for atrial septal defects occlusion at each branch was considered as the effectiveness outcome. Direct medical costs and probabilities for each event were inserted in the model using data available from Brazilian public sector database system and information extracted from the literature review, using micro-costing technique. Sensitivity analysis included price variations of percutaneous implant. RESULTS: The results obtained from the decision model demonstrated that the percutaneous implant was more cost effective in cost-effectiveness analysis at a cost of US$8,936.34 with a reduction in the probability of surgery occurrence in 93% of the cases. Probability of atrial septal communication occlusion and cost of the implant are the determinant factors of cost-effectiveness ratio. CONCLUSIONS: The proposal of a decision model seeks to fill a void in the academic literature. The decision model proposed includes the outcomes that present major impact in relation to the overall costs of the procedure. The atrial septal defects occlusion using percutaneous implant reduces the physical and psychological distress to the patients in relation to the conventional surgery, which represent intangible costs in the context of economic evaluation.

  17. Three-dimensional echocardiographic assessment of atrial septal defects

    Directory of Open Access Journals (Sweden)

    Charles German

    2015-01-01

    Full Text Available Echocardiography provides a useful tool in the diagnosis of many congenital heart diseases, including atrial septal defects, and aids in further delineating treatment options. Although two-dimensional echocardiography has been the standard of care in this regard, technological advancements have made three-dimensional echocardiography possible, and the images obtained in this new imaging modality are able to accurately portray the morphology, location, dimensions, and dynamic changes of defects and many other heart structures during the cardiac cycle.

  18. Intraoperative closure of infant multiple muscular ventricular septal defects with Amplatzer occluder

    International Nuclear Information System (INIS)

    Liu Jinfen; Gao Wei; Zhu Zhongqun; Chen Huiwen; Zhang Yuqi

    2005-01-01

    Objective: To report the preliminary experience of intraoperative hybrid therapy for closure of multiple muscular ventricular septal defects (VSD) in a small infant. Methods: After median sternotomy, a AGA Amplatzer occluder was introduced through right ventricular surface to close 2 muscular ventricular septal defects under transesophageal echocardiographic guidance. Results: The infant survived after the treatment without residual shunting, and rehabilitated rapidly. Conclusions: Intraoperative hybrid therapy with combined surgical technique and interventional procedure for closure of multiple muscular VSD in small infant is a safe and effective method. (authors)

  19. Cor triatriatum dexter associated with atrial septal defect: Management in a complex clinical case.

    Science.gov (United States)

    Sozzi, Fabiola B; Montanaro, Claudia; Bacà, Laura; Viani, Giacomo M; Zilocchi, Massimo; Canetta, Ciro; Meazza, Roberto; Pavone, Laura; Lombardi, Federico

    2017-11-01

    The coexistence of an atrial septal defect and a prominent eustachian valve is a rare congenital anomaly, rarely reported in literature. Differentiation between a giant eustachian valve and cor triatriatum dexter can be difficult. A case of a large atrial septal defect associated with cor triatriatum dexter diagnosed by echocardiography in an asymptomatic woman is reported. A watchful waiting strategy was adopted. © 2017, Wiley Periodicals, Inc.

  20. Exercise thallium-201 imaging in complete left bundle branch block and the prevalence of septal perfusion defects

    International Nuclear Information System (INIS)

    Jazmati, B.; Sadaniantz, A.; Emaus, S.P.; Heller, G.V.

    1991-01-01

    To determine the prevalence of septal defects in a generalized referral population, the records of 93 consecutive patients with complete left bundle branch block (BBB) who underwent symptom-limited treadmill exercise testing with thallium-201 myocardial imaging over a 3-year period were reviewed. Segmental analysis of the planar thallium-201 images was performed in a blinded fashion with agreement by consensus. Computerized quantitative analysis of the images also was independently performed, and was correlated with the visual interpretations. Forty-seven patients (51%) had normal images, and 46 (49%) had defects of greater than or equal to 2 segments. In the abnormal studies, only 13 patients (14% of the total population) had septal defects, while a much higher number of patients, 33 (39%), had inferior or apical defects, or both. Coronary angiography was performed in 6 patients with septal defects: Significant narrowing of the left anterior descending coronary artery was found in 4 patients, a narrowed right coronary artery was found in 1, and normal coronary arteries were seen in the other patient. In conclusion, whereas previous studies have suggested a high percentage of false-positive septal defects in patients with left BBB, this study demonstrates a low prevalence (14%) of septal defects in a large population of unselected patients presenting for exercise thallium-201 imaging. Therefore, exercise thallium-201 imaging remains a useful procedure for evaluating patients with complete left BBB

  1. Right and Left Ventricular Volumes in Atrial Septal Defect Studied by Radiocardiography

    Energy Technology Data Exchange (ETDEWEB)

    Ivancevic, D. [Radioisotope Department, Internal Clinic, Rebro, Zagreb, Yugoslavia (Croatia); Vernejoul, P. de; Kellershohn, C. [CEA, Service Hospitalier Frederic Joliot, Departement de Biologie, Orsay (France)

    1971-02-15

    Radiocardiography with radioiodinated ({sup 131}I) human serum albumin and barium ({sup 137m}Ba) solution injected into the right subclavian vein has been performed in a group of 43 patients with atrial septal defect and left-to-right shunt. Data on the output and ejection index of each ventricle are essential for the estimation of the diastolic and residual volumes of the right and left ventricle. The systemic flow was therefore calculated according to Veall's formula and the pulmonary flow and the shunt How were determined using the method of de Vernejoul and co-workers. The formulas for the calculation of ventricular volumes were modified. The results show that many cases of atrial septal defect have an enlarged right ventricle whereas the left ventricle remains normal or is diminished. These changes correlate well with the amount of the shunt flow. In both ventricles the ventricular volumes show a good correlation with the stroke volumes. For the regulation of the pulmonary blood volume the right ventricle seems to be more important than the left ventricle. The operative closure of atrial septal defect (in 14 patients) has normalized the size of ventricular volumes. (author)

  2. Ventricular Septal Defect: Peculiarities of Early Neonatal and Postnatal Diagnosis, Clinical Manifestations, Treatment and Prognosis at the Contemporary Stage

    Directory of Open Access Journals (Sweden)

    K.A. Kalashnikova

    2016-05-01

    Full Text Available The article presents the literature data on the incidence, the main clinical manifestations, modern methods for early neonatal and postnatal diagnosis and treatment of ventricular septal defect in children, as well as the prognosis of this disease. According to the International Classification of Diseases, 10th revision, ventricular septal defect is classified as Q21.0 Ventricular septal defect. Incidence. In the overall structure of congenital malformations of the cardiovascular system, ventricular septal defect has about 20 %. Diagnosis. Moderate ventricular septal defect is manifested by shortness of breath, rapid fatigability during feeding, delay in physical development. Significant arterial-venous shunt in the first month of life is accompanied by a transient mild cyanosis when the baby is fed and cries. Infants develop high pulmonary hypertension, circulatory failure, malnutrition. Small noise intensity is typical for newborns in the first weeks or even months of life, which is due to physiologically increased intravascular pulmonary resistance. Systolic murmur is extended to the entire systole with maximum amplitude at the left edge of the sternum at the level of III–IV intercostal spaces. Sclerotic phase of pulmonary hypertension with ventricular septal defect is defined as Eisenmenger reaction. The clinical picture of this disorder depends on the degree of hemodynamic instability caused by the defect parameters, the pressure level in the pulmonary artery, vascular pulmonary resistance, the magnitude and direction of the shunt through the defect. Diagnosis is confirmed by characteristic changes in the electrocardiogram, echocardiography and chest radiograph. Treatment. Small muscular ventricular septal defects often close spontaneously during the first 2 years of life. Drug correction is needed in the development of congestive heart failure. The optimum age for surgery — 5–9 years.

  3. Comparison between minimal right vertical infra-axillary thoracotomy and standard median sternotomy for repair of atrial septal defects

    Directory of Open Access Journals (Sweden)

    Hafize Yaliniz

    2015-10-01

    Conclusion: Minimal right vertical infra-axillary thoracotomy can be performed with favorable cosmetic and clinical results for atrial septal defects closure. Infra-axillary thoracotomy provides a good alternative to standard median sternotomy for patients with atrial septal defects.

  4. Intermittent′ restrictive ventricular septal defect in Tetralogy of Fallot

    Directory of Open Access Journals (Sweden)

    Sudhir S Shetkar

    2015-01-01

    Full Text Available Ventricular septal defect (VSD in Tetralogy of Fallot (TOF is usually large and non-restrictive with equalization of right and left ventricular pressures. Restrictive VSD in TOF is rare. We present an unusual case of TOF with restriction to VSD caused by accessory tricuspid valve tissue that varied with respiration.

  5. Animal experimental research of the endothelialization of home-made atrial septal defect occluder device

    International Nuclear Information System (INIS)

    Chen Mingwu; Zhou Aiqing; Li Feng; Gao Wei; Yu Zhiqing; Tang Ning; Zhang Lan

    2003-01-01

    Objective: To evaluate the endothelialization of Chinese nitinol atrial septal defect occluder device. Methods: Atrial septal defect with controllable size was created by the Brockenborough needle and Rashkind balloon atrial septostomy, the occluder devices were implanted in six piglets (mean weight 7.5 kg). Two pigs were killed each time after 1 month, 3 months and 6 months after the device implantation and then the explanted devices were examined by scanning electron microscope (SEM). Results: The devices were found covering with collagen fibrosis together with diffuse endothelial cells spreading over the primer 1 month after implantation. The implants were covered mostly by neointima 3 months after implantation and completely covered by confluent endothelial cells 6 months after the implantation. Endothelial cells were not found on the smooth marker band at 3 months, however, did exist by 6 months. Conclusions: Home-made atrial septal defect occluder devices were mostly endothelialised 3 months after the implantation and did completely at 6 months

  6. Transcatheter closure of secundum atrial septal defect with cardio SEAL septal occluder. A preliminary result of clinical application

    International Nuclear Information System (INIS)

    Zhang Gejun; Dai Ruping; Liu Yanling; Jiang Shiliang; Zeng Zheng; Huang Lianjun; Xie Ruolan

    2001-01-01

    Objective: To evaluate the efficiency and preliminary results of transcatheter closure of secundum atrial septal defect (ASD) with CardioSEAL septal occluder. Methods: There were 12 patients in this study. Trans-esophageal echocardiography (TEE) before the interventions confirmed the ASDs with a mean diameter of *13.14 +- 3.48) mm (ranging from 8 to 20 mm). There were 11 isolated ASDs and 1 multi-defects ASD in the group. Each ASD was occluded with CardioSEAL septal occluder through the percutaneous procedure. The closure procedure was guided by fluoroscopy and trans-esophageal echocardiography. The TEE was done immediately after the procedure to find whether there was residual shunt. Trans-thoracic-echocardiography (TTE), ECG, and X-ray examination were done 24 hours, 1 month, 3 months, and 1 year after the procedures as follow-up to evaluate the efficiency. Results: The placements of the occluders were successful in 11 cases. There were no mortality and no emergent surgery during the procedures. TEE confirmed that there were trivial and small residual shunts in 3 cases immediately after the procedures. TTE confirmed small residual shunts in 2 cases 24 hour after the procedures, and in 1 case 1 month, 3 month, and 1 year after the procedures. Conclusion: Transcatheter closure of secundum ASD with CardioSEAL septal occluder was an efficient nonsurgical method. It could be the method of choice in treating the ASDs with special anatomic variations. It had a high successful rate of device placement and satisfied preliminary results, but the long-term follow-up was needed

  7. Pulmonary valve endocarditis associated to a septal interventricular defect and infundibular and pulmonary valve Stenosis

    International Nuclear Information System (INIS)

    Echeverri, Juan G; Diaz, Alejandro; Jaramillo, Nicolas; Gonzalez, Sergio

    2004-01-01

    Ventricular septal defects generate 10% of all adult congenital cardiopathies. 4% to 8% of patients to whom the defect has not been corrected are in risk of developing endocarditis. Pulmonary valve endocarditis is a rare event (1.5% to 2% of all endocarditis cases) and its mean etiology is intravenous drug abuse. The most frequently isolated microorganism in these cases is staphylococcus aurous. We report a case of pulmonary valve endocarditis associated with ventricular septal defect and valvular and infundibular pulmonary stenosis caused by streptococcus sp. in a patient without past medical history of drug abuse, alcoholism or previous invasive procedures

  8. Long-term follow up after transcatheter closure of atrial septal defect and patent foramen ovale in adults

    Directory of Open Access Journals (Sweden)

    Jure Dolenc

    2014-01-01

    Full Text Available Background: The aim of our study was to define long-term electrocardiographic and echocardiographic changes and complications after transcatheter closure of atrial septal defect and patent foramen ovale in adults.Methods: The clinical, electrocardiographic and echocardiographic follow-up of 137 consecutive patients that underwent transcatheter closure of atrial septal defect (51 patients or patent foramen ovale (86 patients in a 10-year period was analyzed retrospectively.Results: In the patent foramen ovale group, we observed no significant postprocedural changes. There were no changes in heart rate, heart rhythm and PR or QRS duration in both groups. In the atrial septal defect group, we observed a leftward shift in the heart axis (p = 0.017, a decrease in the estimated systolic pulmonary artery pressure (p = 0.024, decreased tricuspid early diastolic flow velocity (p = 0.002, a decrease in the right chamber dimensions (p = 0.0004 and interventricular septal movement normalization (p < 0.0001. Most of the complications were mild and occurred early after the procedure. Three early serious complications were documented.Conclusions: No electrocardiographic or echocardiographic changes occurred after patent foramen ovale closure. Atrial septal defect closure is related to significant early morphological and hemodynamic improvement. Postprocedural complications are usually early and mild but serious late complications can occur. For that reason, long-term follow up is recommended in these patients.

  9. Maternal alcohol drinking pattern during pregnancy and the risk for an offspring with an isolated congenital heart defect and in particular a ventricular septal defect or an atrial septal defect

    DEFF Research Database (Denmark)

    Strandberg-Larsen, Katrine; Skov-Ettrup, Lise Skrubbeltrang; Grønbaek, Morten

    2011-01-01

    (VSD) or of an atrial septal defect (ASD). METHODS: Participants were 80,346 pregnant women who were enrolled into the Danish National Birth Cohort in 1996-2002 and gave birth to a live-born singleton without any chromosome anomalies. Twice during pregnancy these women were asked about their intake...... the early part of pregnancy was not statistical significantly associated with the prevalence of isolated VSD and ASD in offspring. Birth Defects Research (Part A), 2011. © 2011 Wiley-Liss, Inc....

  10. Time course of an X-ray picture following operation for the ventricular septal defect

    Energy Technology Data Exchange (ETDEWEB)

    Khenynya, R.L.; Latsis, A.T.; Rubene, M.Ya.

    1983-01-01

    An x-ray picture was studied over time in 80 pediatric patients by hemodynamic groups in accordance with the classification adopted in the A.I. Bakulev Institute of Cardiovascular Surgery, USSR AMS. Of them in 15 patients in addition to the ventricular septal defect, stenosis of the pulmonary artery was revealed, in 11 concomitant insufficiency of the tricuspid valve. The best results after radical correction of the defect were achieved in patients with insignificant or moderate disorder of the pulmonary hemodynamics; positive results in patients with a high pulmonary hypertension were observed over time later. X-ray studies on the regression of disorders of the pulmonary hemodynamics emphasize the necessity of early diagnosis of the ventricular septal defect followed by surgical correction.

  11. Prevention of congenital defects induced by prenatal alcohol exposure (Conference Presentation)

    Science.gov (United States)

    Sheehan, Megan M.; Karunamuni, Ganga; Pedersen, Cameron J.; Gu, Shi; Doughman, Yong Qiu; Jenkins, Michael W.; Watanabe, Michiko; Rollins, Andrew M.

    2017-02-01

    Nearly 2 million women in the United States alone are at risk for an alcohol-exposed pregnancy, including more than 600,000 who binge drink. Even low levels of prenatal alcohol exposure (PAE) can lead to a variety of birth defects, including craniofacial and neurodevelopmental defects, as well as increased risk of miscarriages and stillbirths. Studies have also shown an interaction between drinking while pregnant and an increase in congenital heart defects (CHD), including atrioventricular septal defects and other malformations. We have previously established a quail model of PAE, modeling a single binge drinking episode in the third week of a woman's pregnancy. Using optical coherence tomography (OCT), we quantified intraventricular septum thickness, great vessel diameters, and atrioventricular valve volumes. Early-stage ethanol-exposed embryos had smaller cardiac cushions (valve precursors) and increased retrograde flow, while late-stage embryos presented with gross head/body defects, and exhibited smaller atrio-ventricular (AV) valves, interventricular septum, and aortic vessels. We previously showed that supplementation with the methyl donor betaine reduced gross defects, improved survival rates, and prevented cardiac defects. Here we show that these preventative effects are also observed with folate (another methyl donor) supplementation. Folate also appears to normalize retrograde flow levels which are elevated by ethanol exposure. Finally, preliminary findings have shown that glutathione, a crucial antioxidant, is noticeably effective at improving survival rates and minimizing gross defects in ethanol-exposed embryos. Current investigations will examine the impact of glutathione supplementation on PAE-related CHDs.

  12. Pulmonary artery-to-left atrial fistula discovered after the closure of atrial septal defect: A rare clinical scenario

    Directory of Open Access Journals (Sweden)

    Akshay Chauhan

    2018-01-01

    Full Text Available A case of the right pulmonary artery-to- left atrial fistula with atrial septal defect (ASD is presented. The fistula was detected after the patient developed desaturation following surgical closure of the ASD. It was managed with a transcatheter (trans-RPA route closure of the fistula using a 12-mm Amplatzer ventricular septal defect closure device.

  13. Acquired ventricular septal defect: A rare sequel of blunt chest ...

    African Journals Online (AJOL)

    Ventricular septal defect (VSD) is the most common congenital cardiac lesion encountered worldwide. Only very rarely is it acquired, and causation through blunt injury in a child is extremely rare. A previously healthy 7‑year‑old boy suffered blunt chest trauma while at play. He presented 11 days later with features of acute ...

  14. Combined perventricular septal defect closure and patent ductus arteriosus ligation via the lower ministernotomy approach.

    Science.gov (United States)

    Voitov, Alexey; Omelchenko, Alexander; Gorbatykh, Yuriy; Bogachev-Prokophiev, Alexander; Karaskov, Alexander

    2018-02-01

    Over the past decade, minimally invasive approaches have been advocated for surgical correction of congenital defects to reduce costs related to hospitalization and for improved cosmesis. Minimal skin incisions and partial sternotomy reduce surgical trauma, however these techniques might not be successful in treating a number of congenital pathological conditions, particularly for combined congenital defects. We focused on cases with a combined presentation of ventricular septal defect and patent ductus arteriosus. We studied 12 infants who successfully underwent surgical treatment for a combined single-stage ventricular septal defect and patent ductus arteriosus closure through a lower ministernotomy without using cardiopulmonary bypass and X-rays. No intraoperative and early postoperative complications or mortality were noted. Postoperative echocardiography did not reveal residual shunts. The proposed technique is safe and reproducible in infants. © Crown copyright 2017.

  15. Aniridia--Wilms′ tumour association--a case with 11p 13-14.1 deletion and ventricular septal defect.

    OpenAIRE

    Rao S; Athale U; Kadam P; Gladstone B; Nair C; Pai S; Kurkure P; Advani S

    1992-01-01

    A two year old female child with bilateral wilms tumor (WT) along with multiple congenital anomalies like bilateral aniridia with congenital cataracts and nystagmus, microcephaly, mental retardation and ventricular septal defect has been described. The karyotype analysis revealed 46 xx, del 11p 13-14.1. Association of ventricular septal defect with the classical features of ′Aniridia-Wilms′ tumor association′ is an unusual feature in this case.

  16. Pulmonary edema following transcatheter closure of atrial septal defect

    Directory of Open Access Journals (Sweden)

    Keerthi Chigurupati

    2015-01-01

    Full Text Available We describe an incident of development of acute pulmonary edema after the device closure of a secundum atrial septal defect in a 52-year-old lady, which was treated with inotropes, diuretics and artificial ventilation. Possibility of acute left ventricular dysfunction should be considered after the defect closure in the middle-aged patients as the left ventricular compliance may be reduced due to increased elastic stiffness and diastolic dysfunction. Baseline left atrial pressure may be > 10 mmHg in these patients. Associated risk factors for the left ventricular dysfunction are a large Qp:Qs ratio, systemic hypertension, severe pulmonary hypertension and paroxysmal atrial fibrillation.

  17. Fourier ventricular amplitude ratio to evaluate atrial septal defect

    International Nuclear Information System (INIS)

    Makler, P.T. Jr.; McCarthy, D.M.; Adler, L.; Alavi, A.

    1985-01-01

    First harmonic Fourier analysis of gated blood pool scans results in the formation of two functional images, a phase and amplitude image. The authors have previously shown that the total amplitude values of the two ventricles can be used to quantitate valvular insufficiency. The ventricular amplitude ratio (VAR, left/right) in normals is 1.14 0.11 and patients with valvular insufficiency is elevated (0.3 0.77). In patients with atrial septal defect (ASD), the right ventricle has a larger stroke volume than the left ventricle, and the VAR should be less than unity. To evaluate whether the amplitude image would permit quantification of shunt flow in ASD, the authors compared the VAR to the OP/QS ratio determined by cardiac catheterization (cath) in 3 groups of patients; group I (n=9) had ASD without valvular insufficiency (one patient had right-to-left shunting due to tricuspid stenosis; group II (n=4) had ventricular septal defect; and group III (n=2) had ASD plus valvular insufficiency. QP/QS shunt flow is also determined in group I using standard first-pass radionuclide angiography (rna). The data suggest that the VAR technique accurately determines the magnitude of shunt flow in ASD patients without concomitant valvular insufficiency

  18. Is right ventricular mid-septal pacing superior to apical pacing in patients with high degree atrio-ventricular block and moderately depressed left ventricular function?

    Science.gov (United States)

    Chen, Kang; Mao, Ye; Liu, Shao-hua; Wu, Qiong; Luo, Qing-zhi; Pan, Wen-qi; Jin, Qi; Zhang, Ning; Ling, Tian-you; Chen, Ying; Gu, Gang; Shen, Wei-feng; Wu, Li-qun

    2014-06-01

    We are aimed to investigate whether right ventricular mid-septal pacing (RVMSP) is superior to conventional right ventricular apical pacing (RVAP) in improving clinical functional capacity and left ventricular ejection fraction (LVEF) for patients with high-degree atrio-ventricular block and moderately depressed left ventricle (LV) function. Ninety-two patients with high-degree atrio-ventricular block and moderately reduced LVEF (ranging from 35% to 50%) were randomly allocated to RVMSP (n=45) and RVAP (n=47). New York Heart Association (NYHA) functional class, echocardiographic LVEF, and distance during a 6-min walk test (6MWT) were determined at 18 months after pacemaker implantation. Serum levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) were measured using an enzyme-linked immunosorbent assay (ELISA) kit. Compared with baseline, NYHA functional class remained unchanged at 18 months, distance during 6MWT (485 m vs. 517 m) and LVEF (36.7% vs. 41.8%) were increased, but BNP levels were reduced (2352 pg/ml vs. 710 pg/ml) in the RVMSP group compared with those in the RVAP group, especially in patients with LVEF 35%-40% (for all comparisons, Pfunction capacity and LV function measurements were not significantly changed in patients with RVAP, despite the pacing measurements being similar in both groups, such as R-wave amplitude and capture threshold. RVMSP provides a better clinical utility, compared with RVAP, in patients with high-degree atrioventricular block and moderately depressed LV function whose LVEF levels ranged from 35% to 40%.

  19. The time course of an X-ray picture following operation for the ventricular septal defect

    International Nuclear Information System (INIS)

    Khenynya, R.L.; Latsis, A.T.; Rubene, M.Ya.

    1983-01-01

    An x-ray picture was studied over time in 80 pediatric patients by hemodynamic groUps in accordance with the classification adopted in the A.I.Bakulev Institute of Cardiovascular Surgery, USSR AMS. Of them in 15 patients in addition to the ventricUlar septal defect, stenosis of the pulmonary artery was revealed, in 11 concomitant insUfficiency of the tricuspid valve. The best results after radical correction of the defect were achieved in patients with insignificant or moderate disorder of the pulmonary hemdynamics; positive results in patients with a high pulmonary hypertension were observed over time later. X-ray studies on the regression of disorders of the pulmonary hemodynamics emphasize the necessity of early diagnosis of the ventricular septal defect followed by surgical correction

  20. Three-dimensional transesophageal echocardiography of the atrial septal defects

    Directory of Open Access Journals (Sweden)

    Romero-Cárdenas Ángel

    2008-07-01

    Full Text Available Abstract Transesophageal echocardiography has advantages over transthoracic technique in defining morphology of atrial structures. Even though real time three-dimensional echocardiographic imaging is a reality, the off-line reconstruction technique usually allows to obtain higher spatial resolution images. The purpose of this study was to explore the accuracy of off-line three-dimensional transesophageal echocardiography in a spectrum of atrial septal defects by comparing them with representative anatomic specimens.

  1. Self-expanding nanoplatinum-coated nitinol devices for atrial septal defect and patent ductus arteriosus closure: a swine model.

    Science.gov (United States)

    Lertsapcharoen, Pornthep; Khongphatthanayothin, Apichai; La-orkhun, Vidhavas; Supachokchaiwattana, Pentip; Charoonrut, Phingphol

    2006-01-01

    Our purpose was to evaluate self-expanding nanoplatinum-coated nitinol devices for transcatheter closure of atrial septal defects and patent ductus arteriosus in a swine model. The devices were braided from platinum-activated nitinol wires and filled with polyester to enhance thrombogenicity. The platinum activation of the nitinol wires was carried out with the help of Nanofusion technology. The coating of platinum covers the exposed surface of the nitinol wires and prevents the release of nickel into the blood stream after the implantation of the device but does not affect its shape memory, which makes the device self-expanding after it is loaded from the catheter. Atrial septal defects were created in 12 piglets by balloon dilation of the patent foramen ovale. The size of the device was selected on the basis of the diameter of the balloon and the size of the defect, measured by transthoracic echocardiography. The devices were successfully deployed in all 12 piglets under fluoroscopic study. Transthoracic color Doppler echocardiograms showed complete closure of the atrial septal defect within 15 minutes of device implantation. Twelve patent ductus arteriosus closure devices were deployed in the right or left subclavian arteries in 10 piglets. Angiograms showed complete occlusion of the subclavian arteries within a few minutes of device deployment. In the atrial septal defect cases, the autopsy findings showed complete organizing fibrin thrombus formation and complete neo-endothelialization on the outer surface of the devices within one week and six weeks of implantation, respectively. The use of self-expanding nanoplatinum-coated nitinol devices for the transcatheter closure of atrial septal defects and patent ductus arteriosus is feasible. The excellent occlusion result and complete neo-endothelialization of the devices in the swine model is an indication of the potential of these devices in human application.

  2. Successful anesthetic management of a child with blepharophimosis syndrome and atrial septal defect for reconstructive ocular surgery

    Directory of Open Access Journals (Sweden)

    Dalim Kumar Baidya

    2011-01-01

    Full Text Available Blepharophimosis syndrome is an autosomal dominant disorder characterized by eyelid malformation, involvement of reproductive system and abnormal facial morphology leading to difficult airway. We report a rare association of blepharophimosis syndrome and atrial septal defect in a 10-year-old girl who came for reconstruction surgery of eyelid. The child had dyspnea on exertion. Atrial septal defect was identified preoperatively by clinical examination and echocardiography. Anesthesia management was complicated by failure in laryngeal mask airway placement and Cobra perilaryngeal airway was subsequently used.

  3. Atrial activation during atrioventricular nodal reentrant tachycardia: studies on retrograde fast pathway conduction

    NARCIS (Netherlands)

    Katritsis, Demosthenes G.; Ellenbogen, Kenneth A.; Becker, Anton E.

    2006-01-01

    Detailed right and left septal mapping of retrograde atrial activation during typical atrioventricular nodal reentrant tachycardia (AVNRT) has not been undertaken and may provide insight into the complex physiology of AVNRT, especially the anatomic localization of the fast and slow pathways. The

  4. The effect of surgical treatment for secundum atrial septal defect in patients more than 30 years old.

    Science.gov (United States)

    Erkut, Bilgehan; Becit, Necip; Unlu, Yahya; Ceviz, Munacettin; Kocogullari, Cevdet Ugur; Ates, Azman; Karapolat, Bekir Sami; Kaygin, Mehmet Ali; Kocak, Hikmet

    2007-01-01

    We prospectively examined whether surgical treatment of secundum atrial septal defects in patients 30 years old improves their early- and mid-term clinical outcomes. Our clinical experience is reviewed to assess the importance of surgical management in elderly patients with atrial septal defect. We analyzed 41 patients older than 30 years of age who underwent surgical correction of a secundum atrial septal defect. To evaluate the effects of surgical treatment, we compared functional capacity, diuretic administration, rhythm status, and echocardiographic parameters of all patients before and after the operation. The median follow-up period was 4.2 years (range, 6 months-7 years). There were no operative deaths. Functional class in most of the patients improved after operation. Two patients reverted to normal sinus rhythm after the operation. There was only one new atrial fibrilation among patients in the postoperative term. Right atrial and right ventricular dimensions and pulmonary artery pressures were significantly decreased, and ejection fractions were significantly increased after the operation. The need for diuretic treatment was decreased after surgical repair. No residual intracardiac shunts were identified during follow-up. There were no cerebrovascular thromboembolic accidents in the early postoperative period. Surgical closure of atrial septal defects in patients over 30 years old can improve their clinical status and prevent right ventricular dilatation and insufficiency. The operation must be performed as soon as possible, even if the symptoms or the hemodynamic impact seem to be minimal.

  5. Acquired ventricular septal defect due to infective endocarditis

    Directory of Open Access Journals (Sweden)

    Randi E Durden

    2018-01-01

    Full Text Available Acquired intracardiac left-to-right shunts are rare occurrences. Chest trauma and myocardial infection are well-known causes of acquired ventricular septal defect (VSD. There have been several case reports describing left ventricle to right atrium shunt after infective endocarditis (IE. We present here a patient found to have an acquired VSD secondary to IE of the aortic and tricuspid valves in the setting of a known bicuspid aortic valve. This is the first case reported of acquired VSD in a pediatric patient in the setting of IE along with literature review of acquired left-to-right shunts.

  6. Supracristal ventricular septal defect with severe right coronary cusp prolapse

    International Nuclear Information System (INIS)

    Hussain, A.H.; Hanif, B.; Khan, G.; Hasan, K.

    2011-01-01

    The case of a 20 years old male, diagnosed as supracristal ventricular septal defect (VSD) for last 6 years is being presented. He came in emergency department with decompensated congestive cardiac failure. After initial stabilization, he underwent trans thoracic echocardiogram which showed large supracristal VSD, severely prolapsing right coronary cusp, severe aortic regurgitation and severe pulmonary hypertension. Right heart catheterization was performed which documented reversible pulmonary vascular resistance after high flow oxygen inhalation. He underwent VSD repair, right coronary cusp was excised and aortic valve was replaced by mechanical prosthesis. Post operative recovery was uneventful. He was discharged home in one week.The case of a 20 years old male, diagnosed as supracristal ventricular septal defect (VSD) for last 6 years is being presented. He came in emergency department with decompensated congestive cardiac failure. After initial stabilization, he underwent trans thoracic echocardiogram which showed large supracristal VSD, severely prolapsing right coronary cusp, severe aortic regurgitation and severe pulmonary hypertension. Right heart catheterization was performed which documented reversible pulmonary vascular resistance after high flow oxygen inhalation. He underwent VSD repair, right coronary cusp was excised and aortic valve was replaced by mechanical prosthesis. Post operative recovery was uneventful. He was discharged home in one week. (author)

  7. Novel de novo pathogenic variant in the NR2F2 gene in a boy with congenital heart defect and dysmorphic features.

    Science.gov (United States)

    Upadia, Jariya; Gonzales, Patrick R; Robin, Nathaniel H

    2018-04-16

    The NR2F2 gene plays an important role in angiogenesis and heart development. Moreover, this gene is involved in organogenesis in many other organs in mouse models. Variants in this gene have been reported in a number of patients with nonsyndromic atrioventricular septal defect, and in one patient with congenital heart defect and dysmorphic features. Here we report an 11-month-old Caucasian male with global developmental delay, dysmorphic features, coarctation of the aorta, and ventricular septal defect. He was later found to have a pathogenic mutation in the NR2F2 gene by whole exome sequencing. This is the second instance in which an NR2F2 mutation has been identified in a child with a congenital heart defect and other anomalies. This case suggests that some variants in NR2F2 may cause syndromic forms of congenital heart defect. © 2018 Wiley Periodicals, Inc.

  8. Radionuclide analysis of right and left ventricular response to exercise in patients with atrial and ventricular septal defects

    International Nuclear Information System (INIS)

    Peter, C.A.; Bowyer, K.; Jones, R.H.

    1983-01-01

    In patients with ventricular or atrial septal defect, the ventricle which is chronically volume overloaded might not appropriately respond to increased demand for an augmentation in output and thereby might limit total cardiac function. In this study we simultaneously measured right and left ventricular response to exercise in 10 normal individuals, 10 patients with ventricular septal defect (VSD), and 10 patients with atrial septal defect (ASD). The normal subjects increased both right and left ventricular ejection fraction, end-diastolic volume, and stroke volume to achieve a higher cardiac output during exercise. Patients with VSD failed to increase right ventricular ejection fraction, but increased right ventricular end-diastolic volume and stroke volume. Left ventricular end-diastolic volume did not increase in these patients but ejection fraction, stroke volume, and forward left ventricular output achieved during exercise were comparable to the response observed in healthy subjects. In the patients with ASD, no rest-to-exercise change occurred in either right ventricular ejection fraction, end-diastolic volume, or stroke volume. In addition, left ventricular end-diastolic volume failed to increase, and despite an increase in ejection fraction, left ventricular stroke volume remained unchanged from rest to exercise. Therefore, cardiac output was augmented only by the heart rate increase in these patients. Right ventricular function appeared to be the major determinant of total cardiac output during exercise in patients with cardiac septal defects and left-to-right shunt

  9. Assessment of atrial septal defects in adults comparing cardiovascular magnetic resonance with transoesophageal echocardiography

    Directory of Open Access Journals (Sweden)

    Brown Michael A

    2010-07-01

    Full Text Available Abstract Background Many adult patients with secundum-type atrial septal defects (ASDs are able to have these defects fixed percutaneously. Traditionally, this has involved an assessment of ASD size, geometry and atrial septal margins by transoesophageal echocardiography (TOE prior to percutaneous closure. This is a semi-invasive technique, and all of the information obtained could potentially be obtained by non-invasive cardiovascular magnetic resonance (CMR. We compared the assessment of ASDs in consecutive patients being considered for percutaneous ASD closure using CMR and TOE. Methods Consecutive patients with ASDs diagnosed on transthoracic echocardiography (TTE were invited to undergo both CMR and TOE. Assessment of atrial septal margins, maximal and minimal defect dimensions was performed with both techniques. Analyses between CMR and TOE were made using simple linear regression and Bland Altman Analyses. Results Total CMR scan time was 20 minutes, and comparable to the TOE examination time. A total of 20 patients (M:F = 5:15, mean age 42.8 years ± 15.7 were included in the analyses. There was an excellent agreement between CMR and TOE for estimation of maximum defect size (R = 0.87. The anterior inferior, anterior superior and posterior inferior margins could be assessed in all patients with CMR. The posterior superior margin could not be assessed in only one patient. Furthermore, in 1 patient in whom TOE was unable to be performed, CMR was used to successfully direct percutaneous ASD closure. Conclusions CMR agrees with TOE assessment of ASDs in the work-up for percutaneous closure. Potentially CMR could be used instead of TOE for this purpose.

  10. Prolonged postoperative desaturation in a child with Down syndrome and atrial septal defect

    Directory of Open Access Journals (Sweden)

    Renu Sinha

    2011-01-01

    Full Text Available We report prolonged desaturation in a child with Down syndrome (DS and atrial septal defect due to undiagnosed interstitial lung disease. An 18-month-old child with DS was scheduled for bilateral lens aspiration for cataract. The child had atrial septal defect and hypothyroidism. He also had delayed milestones and hypotonia with episodes of recurrent respiratory tract infection necessitating repeated hospitalization. Preoperative evaluation was unremarkable. General anaesthesia and controlled ventilation using proseal laryngeal mask airway was instituted. He had uneventful intraoperative period. In the postoperative period, the child had desaturation 1 hour after surgery on discontinuation of oxygen supplementation by face mask, which improved with oxygen therapy. Supplemental oxygen via face mask was continued and weaned off over several days. On further evaluation, the child was diagnosed as having interstitial lung disease. He improved and discharged from the hospital 15 days after the surgery with room air saturation of 90%.

  11. Budget impact analysis of the percutaneous septal occluder for treatment of ostium secundum atrial septal defects in the Brazilian Unified National Health System.

    Science.gov (United States)

    Senna, Kátia Marie Simões e; Sarti, Flavia Mori; Costa, Márcia Gisele Santos da; Nita, Marcelo Eidi; Santos, Marisa da Silva; Tura, Bernardo Rangel; Correia, Marcelo Goulart

    2015-08-01

    The aim of this study was to perform a budget impact analysis on the adoption of percutaneous occlusion of ostium secundum atrial septal defects in the Brazilian Unified National Health System. Costs were collected using micro-costing technique from medical records for each treatment technique (conventional surgery versus percutaneous septal occluder) at a public federal hospital specialized in high-complexity cardiology. The analysis showed that expenditures associated with percutaneous occlusion were lower than with conventional surgery, and sensitivity analysis confirmed the cost reduction in several scenarios, showing a significant budget impact with a 30% adoption rate for the percutaneous occluder (savings of approximately 1.5 million dollars per year). The study indicates that the adoption of the percutaneous septal occluder would mean cost savings of approximately 3.5 million dollars for the Brazilian public health system.

  12. Closure of secundum atrial septal defect in adults

    International Nuclear Information System (INIS)

    Elahi, M.M.; Pollock, J.C.S.

    2003-01-01

    Objective: To examine the outcome of ASD closure in adults and the effect of patients age on drug therapy, symptoms and incidence of atrial fibrillation. Results: There were no deaths. Five patients from early in the series were lost to follow-up. Large defect size was associated with patch rather than direct closure but there was extensive crossover. Analysis by age showed that patients over 49 had more postoperative atrial fibrillation (P 0.001), more chest pain (P>0.0001), more postoperative dyspnea (p = 0.021), greater use of diuretics (p = 0.20) and longer hospital stay (10.1 plus minis 2.6 vs. 8.5 plus minis 1.6 days; p = 0.007) than patients under 49. Conclusion: Operation for atrial septal defects in adults can be performed with no mortality and low morbidity. The age at which complications appear more frequent suggests that closer analysis of these patients is required. (author)

  13. Congenital Complete Atrioventricular Block : Clinical and Experimental Studies

    NARCIS (Netherlands)

    Blank, A.C.

    2014-01-01

    Complete atrioventricular block (CAVB) is a cardiac conduction defect wherein the trans¬mission of the normal electrical impulse from the atria to the ventricle is interrupted due to structural or functional impairment of the atrioventricular (AV) conduction system. If CAVB is diagnosed in utero or

  14. Novel mutation of GATA4 gene in Kurdish population of Iran with nonsyndromic congenital heart septals defects.

    Science.gov (United States)

    Soheili, Fariborz; Jalili, Zahra; Rahbar, Mahtab; Khatooni, Zahed; Mashayekhi, Amir; Jafari, Hossein

    2018-03-01

    The mutations in GATA4 gene induce inherited atrial and ventricular septation defects, which is the most frequent forms of congenital heart defects (CHDs) constituting about half of all cases. We have performed High resolution melting (HRM) mutation scanning of GATA4 coding exons of nonsyndrome 100 patients as a case group including 39 atrial septal defects (ASD), 57 ventricular septal defects (VSD) and four patients with both above defects and 50 healthy individuals as a control group. Our samples are categorized according to their HRM graph. The genome sequencing has been done for 15 control samples and 25 samples of patients whose HRM analysis were similar to healthy subjects for each exon. The PolyPhen-2 and MUpro have been used to determine the causative possibility and structural stability prediction of GATA4 sequence variation. The HRM curve analysis exhibit that 21 patients and 3 normal samples have deviated curves for GATA4 coding exons. Sequencing analysis has revealed 12 nonsynonymous mutations while all of them resulted in stability structure of protein 10 of them are pathogenic and 2 of them are benign. Also we found two nucleotide deletions which one of them was novel and one new indel mutation resulting in frame shift mutation, and 4 synonymous variations or polymorphism in 6 of patients and 3 of normal individuals. Six or about 50% of these nonsynonymous mutations have not been previously reported. Our results show that there is a spectrum of GATA4 mutations resulting in septal defects. © 2018 Wiley Periodicals, Inc.

  15. Percutaneous closure of congenital aortocaval fistula with a coexisting secundum atrial septal defect

    DEFF Research Database (Denmark)

    Loh, Poay Huan; Jensen, Tim; Søndergaard, Lars

    2012-01-01

    Congenital aortocaval fistula is a very rare anomaly. Clinically, it resembles conditions that cause left-to-right shunt of blood. We report a case of such anomaly in combination with a secundum atrial septal defect in a 13-month-old girl who presented with failure to thrive and exertional respir...

  16. Facts about Atrioventricular Septal Defect (AVSD)

    Science.gov (United States)

    ... through the valve. A leaky mitral valve can cause the heart to work harder to get enough blood to the rest of the body; a leaky mitral valve might have to be surgically repaired. A child or adult with an AVSD will need regular ...

  17. Diagnosis of atrial septal defect using magnetic resonance imaging

    International Nuclear Information System (INIS)

    Sakakibara, Makoto; Kobayashi, Shiro; Imai, Hitoshi; Watanabe, Shigeru; Masuda, Yoshiaki; Inagaki, Yoshiaki

    1987-01-01

    The capability of magnetic resonance imaging (MRI) to detect atrial septal defect (ASD) was evaluated in 28 ASD patients of diagnostic confirmation, including five with complicated anomaly. Findings of MRI obtained from 7 normal volunteers and 142 patients with acquired heart diseases were used as controls. Non-gated MRI, performed in three ASD patients, failed to reveal defect areas ; gated MRI, in the other 25 patients, depicted defects in the interatrial septum corresponding to findings of surgery and cardiac catheterizatioin, which allowed the site and size of ASD to be determined. Other findings of MRI included right atrial dilatation, right ventricular hypertrophy and dilatation, and pulmonary artery dilatation in majority of ASD patients. Complex anomalies associated with ADS were also clearly shown on MRI. The interatrial septum was shown on gated-MRI in 124 control subjects, 17 of whom had an extremely faint signal from the central portion of the interatrial septum. The results indicate that gated MRI is a valuable noninvasive method in the diagnosis of ASD and complicating anomalies. (Namekawa, K.)

  18. Quantification of left to right shunts in adults with atrial septal defects of secundum type, using radionuclide technique

    International Nuclear Information System (INIS)

    Sire, S.; Rootwelt, K.; Mangschau, A.

    1991-01-01

    Quantification of left to right shunt was carried out in 15 adult patients with a suspected ostium secundum atrial septal defect (ASD II). Radionuclide shunt quantitation correlated well with the results of righ heart catheterization. The radionuclide technique failed in two patients for technical reasons, but revealed no false negative or false positive results when technically satisfactory. The diagnosis was confirmed at operation. It is concluded that the radionuclide technique is a useful and reliable method which can also be used at follow-up after surgery in patients with artrial septal defects of secundum type. 20 refs., 3 figs., 1 tab

  19. MORTAL PULPOTOMY ON CHILDREN WITH VENTRICULAR SEPTAL DEFECT

    Directory of Open Access Journals (Sweden)

    Devi N. R. Devy

    2006-04-01

    Full Text Available Congenital heart disease is a condition of heart anomaly found since birth. The most common is ventricular septal defect whereby an aperture is found in the partition of heart chamber. It is estimated that 40,000 newborn in Indonesia have this defective condition. Dental treatment for patients with such condition must be undertaken in a very cautious way. Tooth with multiple caries can potentially lead to endocarditis bacteria. As such, dentist must be watchful on dental treatment that may worsen the patient’s condition. To prevent endocarditis bacteria, a prophylaxis antibiotic is required as recommended by the American Heart Association (AHA. In this particular case, a dental treatment was undertaken to a child patient with congenital heart disease. Previously, the patient has undergone dental treatment in the form of multiple extractions under general anesthesia before conducting cardiac surgery. In this case the treatment includes mortal pulpotomy, GIC restoration, and fissure sealant – all conducted in one visit under general anesthesia. A year after the treatment, there are no complaints from the patient and no irregularity on x-ray results.

  20. Posterior extensions of the human compact atrioventricular node: a neglected anatomic feature of potential clinical significance

    NARCIS (Netherlands)

    Inoue, S.; Becker, A. E.

    1998-01-01

    Catheter ablation procedures have revived interest in the detailed anatomy of the specialized atrioventricular (AV) septal junctional area. The compact AV node usually is considered to have a blunt posterior end. The objective of this study was to reconstruct the human compact AV node in relation to

  1. A large ventricular septal defect complicating resuscitation after blunt trauma

    Directory of Open Access Journals (Sweden)

    Henry D I De′Ath

    2012-01-01

    Full Text Available A young adult pedestrian was admitted to hospital after being hit by a car. On arrival to the Accident and Emergency Department, the patient was tachycardic, hypotensive, hypoxic, and acidotic with a Glasgow Coma Scale of 3. Despite initial interventions, the patient remained persistently hypotensive. An echocardiogram demonstrated a traumatic ventricular septal defect (VSD with right ventricular strain and increased pulmonary artery pressure. Following a period of stabilization, open cardiothoracic surgery was performed and revealed an aneurysmal septum with a single large defect. This was repaired with a bovine patch, resulting in normalization of right ventricular function. This case provides a vivid depiction of a large VSD in a patient following blunt chest trauma with hemodynamic compromise. In all thoracic trauma patients, and particularly those poorly responsive to resuscitation, VSDs should be considered. Relevant investigations and management strategies are discussed.

  2. Comunicação interatrial do tipo seio coronário, comunicação interventricular e ausência de veia cava superior esquerda Coronary sinus atrial septal defect and ventricular septal defect with no left superior vena cava

    Directory of Open Access Journals (Sweden)

    Fábio Alves Almeida

    1998-10-01

    Full Text Available Relatamos um caso raro de um paciente de 21 meses, portador de comunicação interatrial do tipo seio coronário, associada a comunicação interventricular perimembranosa, e ausência de veia cava superior esquerda. O diagnóstico foi realizado através da ecocardiografia e confirmado pela angiografia. O paciente foi operado sem intercorrências, ambos os defeitos foram fechados com patch de pericárdio bovino e o fluxo das veias coronárias ficou direcionado para o átrio esquerdo. Um ecocardiograma mostrou ausência de shunt residual através dos defeitos.We report a rare case of a 21 month old child with a coronary sinus atrial septal defect associated with perimembranous ventricular septal defect and no left superior vena cava. The diagnosis was made by transthoracic echocardiogram and confirmed by angiography. The patient was operated on uneventfully, both defects were closed with bovine pericardial patches and the flow from the coronary veins was directed towards the left atrium. An echocardiogram revealed complete closure of both defects.

  3. The unnatural history of the ventricular septal defect: Outcome up to 40 years after surgical closure

    NARCIS (Netherlands)

    M.E. Menting (Myrthe); J.A.A.E. Cuypers (Judith); P. Opic (Petra); E.M.W.J. Utens (Elisabeth); M. Witsenburg (Maarten); A.E. van den Bosch (Annemien); R.T. van Domburg (Ron); F.J. Meijboom (Folkert); H. Boersma (Eric); A.J.J.C. Bogers (Ad); J.W. Roos-Hesselink (Jolien)

    2015-01-01

    textabstractBackground Few prospective data are available regarding long-term outcomes after surgical closure of a ventricular septal defect (VSD). Objectives The objective of this study was to investigate clinical outcomes >30 years after surgical VSD closure. Methods Patients who underwent

  4. Transcatheter device closure of secundum atrial septal defect (ASD) in young children

    International Nuclear Information System (INIS)

    Sadiq, N.; Ullah, M.; Sultan, M.; Akhtar, K.; Akbar, H.

    2014-01-01

    To analyze the safety and efficacy of device closure of secundum atrial septal defect in children = 5 years of age. Study Design: Quasi-experimental study. Place and Duration of Study: The study was conducted at Armed Forces Institute of Cardiology / National Institute of Heart Diseases Rawalpindi, Pakistan from Dec 2010 - Dec 2012. Patients and Method: Forty eight patients = 5 years of age underwent transcatheter closure of secundum ASD during two years. All patients were evaluated with 2-D echocardiography before the procedure. The sizing balloon was used in 6% and general anaesthesia was given in 83% (n=40) of patients. Results: Ninety seven point nine percent (47/48) had successful closure of ASD. The mean age was 4.1 +- 6.8 years (range 2.5-5 years) and 58.4% (28/48) were females. The defect size and occluders used were between 5-20 mm (mean 12 +-- 3.5) and 8-22 mm (mean 15 +- 3.9) respectively, three patients had simultaneous procedures including pulmonary valvuloplasty in two and percutaneous transmitral commissurotomy (PTMC) in one. The major complications remained 2% (1/48) which included device embolization just after release of device while minor complication rate was 12.5%. The median procedure time was 30 min (15-100 min) and median fluoroscopic time was 6 min (1.50-45 min). There was no emergency surgical exploration or death during this period. Conclusion: Transcatheter device closure of suitable secundum atrial septal defect is effective and safe in young children in skilled and professional hands. (author)

  5. The unnatural history of the ventricular septal defect : outcome up to 40 years after surgical closure

    NARCIS (Netherlands)

    Menting, Myrthe E; Cuypers, Judith A A E; Opić, Petra; Utens, Elisabeth M W J; Witsenburg, Maarten; van den Bosch, Annemien E; van Domburg, Ron T; Meijboom, Folkert J; Boersma, Eric; Bogers, Ad J J C; Roos-Hesselink, Jolien W

    2015-01-01

    BACKGROUND: Few prospective data are available regarding long-term outcomes after surgical closure of a ventricular septal defect (VSD). OBJECTIVES: The objective of this study was to investigate clinical outcomes>30 years after surgical VSD closure. METHODS: Patients who underwent surgical VSD

  6. Off-Pump Repair of a Post Myocardial Infarction Ventricular Septal Defect

    Directory of Open Access Journals (Sweden)

    Feridoun Sabzi

    2014-01-01

    Full Text Available Refractory cardiogenic shock meant that traditional patch repairs requiring cardiopulmonary bypass would be poorly tolerated and external sandwich closure of post myocardial ventricular septal defect (VSD appears to be simple and effective after initial myocardial infarction (MI. The three cases presented with a VSD after of acute MI with or without thrombolysed with streptokinase during patient admission. The general condition of the three patients was poor with pulmonary edema, low cardiac output and renal failure. The heart was approached through a median sternotomy. Off-pump coronary artery bypass grafting of the coronary artery lesion was done first using octopus and beating heart surgery method and latero - lateral septal plication was performed using sandwich technique. Low cardiac output managed with intra-aortic balloon pump in these patients accompanied with inotropic drugs. Post-operative transesophageal echocardiography revealed that VSD was closed completely in one patient and in two patients small residual VSD remained. More experience is required to ascertain whether this technique will become an accepted alternative to patch repairs.

  7. A case difficult to diagnose in adults: High sinus venous atrial septal defect

    Directory of Open Access Journals (Sweden)

    Ozge Cetinarslan

    2018-01-01

    Full Text Available Sinus venous atrial septal defect (SVD is highly difficult to diagnose because of its location. Below, we report a case of SVD which is misdiagnosed as pulmonary hypertension and anomalous pulmonary venous return. A 57-year-old female patient was referred to congenital disease outpatient clinic of a tertiary center. She was admitted to the hospital with complaints of fatigue and exercise dyspnea which had started a year ago. She had transthoracic echocardiography (TTE examination done in another hospital which showed dilated right heart chambers and pulmonary hypertension. She underwent transesophageal echocardiography (TEE examination with the suspicion of atrial septal defect (ASD, but no defect was seen. As her symptoms persisted, we repeated the TTE and TEE examination in our center. TEE revealed 0.6 cm ASD on the upper side of the interatrial septum. All four pulmonary veins were draining into the left atrium. Right heart catheterization (RHC confirmed the diagnosis. A left-to-right shunt was detected and localized by a significant step-up in blood oxygen saturation found between mid and upper segments of the right atrium. According to our TEE and RHC results, we planned the surgical closure of the defect. Sinus venous ASD is deficiency of the superior portion of atrial septum adjacent to superior vena cava. Diagnosis of SVD is often more difficult than other forms of ASD and may require special imaging such as TEE, magnetic resonance imaging, or computed tomographic scanning. In conclusion, cardiologists must be aware about the possibility of SVD patients who have unexplained exertional dyspnea and fatigue, dilated right atrium and ventricle, pulmonary hypertension, paradoxical embolism, or atrial arrhythmias in their respective populations.

  8. Membranous Septal Aneurysm: An Unusual Case for Sub-Pulmonary Obstruction in cTGA

    Directory of Open Access Journals (Sweden)

    Onur Isik

    2013-10-01

    Full Text Available Aneurysm of the membranous portion of the interventricular septum is an uncommon congenital cardiac malformation that is rarely diagnosed during life. Perimembranous ventricular septal defects are the most common type of the ventricular septal defects and originates from morphologically membranous interventricular septum. Surgical exposure and accurate closure of a ventricular septal defect with a membranous septal aneurysm beneath the tricuspid septal leaflet carries a risk of tricuspid valve insufficiency and conduction disturbances. The current study presents a case with membranous septal aneursym with congenitally corrected transposition of the great arteries and sub-pulmonary obstruction, which was surgically corrected.

  9. The unnatural history of the ventricular septal defect: outcome up to 40 years after surgical closure

    NARCIS (Netherlands)

    Menting, Myrthe E.; Cuypers, Judith A. A. E.; Opić, Petra; Utens, Elisabeth M. W. J.; Witsenburg, Maarten; van den Bosch, Annemien E.; van Domburg, Ron T.; Meijboom, Folkert J.; Boersma, Eric; Bogers, Ad J. J. C.; Roos-Hesselink, Jolien W.

    2015-01-01

    Few prospective data are available regarding long-term outcomes after surgical closure of a ventricular septal defect (VSD). The objective of this study was to investigate clinical outcomes>30 years after surgical VSD closure. Patients who underwent surgical VSD closure during childhood between 1968

  10. Transcatheter closure of a small atrial septal defect with an Amplatzer™ patent foramen ovale occluder in a working dog with cyanosis and exercise intolerance at high altitude.

    Science.gov (United States)

    Shelden, A; Wesselowski, S; Gordon, S G; Saunders, A B

    2017-12-01

    A 6.5-year-old male Border Collie presented for transcatheter closure of an atrial septal defect due to exercise intolerance and cyanosis while working and training at altitude. A small, left-to-right shunting secundum atrial septal defect was confirmed with no evidence of significant right-sided volume overload. Pulmonary hypertension with subsequent right-to-left interatrial shunting occurring during exercise at high altitude was suspected and prompted the closure of the defect due to the dog's continued athletic requirements. The anatomy of the defect prompted use of a patent foramen ovale occluder rather than an atrial septal defect occluder, which was deployed using a combination of fluoroscopic and transesophageal echocardiographic guidance. The owner did not report continued exercise intolerance or cyanosis and the dog's lifestyle and residence at altitude was unchanged. Copyright © 2017 Elsevier B.V. All rights reserved.

  11. Fenestrated atrial septal defect percutaneously occluded by a single device: procedural and financial considerations.

    Science.gov (United States)

    Tal, Roie; Dahud, Qarawani; Lorber, Avraham

    2013-06-01

    A 45-year-old patient presented with a cerebrovascular attack and was subsequently found to have a multi-fenestrated atrial septal defect. Various therapeutic options for percutaneous transcatheter closure with their respective benefits and flaws are discussed, as well as procedural and financial considerations. The decision making process leading to a successful result using a single occlusive device is presented, alongside a review of the literature.

  12. Intracardiac Echocardiography Evaluation in Secundum Atrial Septal Defect Transcatheter Closure

    International Nuclear Information System (INIS)

    Zanchetta, Mario; Pedon, Luigi; Rigatelli, Gianluca; Carrozza, Antonio; Zennaro, Marco; Di Martino, Roberta; Onorato, Eustaquio; Maiolino, Pietro

    2003-01-01

    Purpose: This study was designed to assess the balloon sizing maneuvers and deployment of an Amplatzer Septal Occluder (ASO). In addition, intraprocedural balloon sizing was compared with off-line intracardiac echocardiographic measurements. Methods: The intracardiac echocardiography (ICE) measurements were: maximum transverse and longitudinal atrial septal defect (ASD) diameters in the aortic valve and four-chamber planes;area of the ASD and its equivalent circle diameter. Thirteen consecutive patients underwent transcatheter implantation of an ASO device using ICE guidance under local anesthesia. The device matching the balloon sizing diameter of the defect was implanted. Qualitative ICE assessment of the ASO devices implanted was performed off line. Results: The mean equivalent circle diameter predicted by ICE was 24.40 ± 5.61 mm and was significantly higher(p 0.027) than the ASD measured by balloonsizing (21.38 ± 5.28 mm). Unlike previous studies we did not find any correlation between the two measurements (correlation coefficient = 0.47). Only four of the 13 patients had optimal device positioning as shown by the qualitative ICE evaluation, whereas the remaining nine patients had inadequate device placement. This resulted in a waist diameter that was an average 26.1% undersized in seven patients and 12.7% oversized in two patients. Five of the seven patients with an undersized device had ASO-atrial septum misalignment with leftward device deviation. Conclusion: The ICE images allowed careful measurement of the dimensions of the ASD and accurately displayed the spatial relations of the ASO astride the ASD.Moreover, use of the ICE measurement led to selection of a different size of device in comparison with those of balloon sizing. The clinical benefit of this new approach needs to be rigorously tested

  13. A neonate with mitral stenosis due to accessory mitral valve, ventricular septal defect, and patent ductus arteriosus: changes in echocardiographical findings during the neonatal period.

    Science.gov (United States)

    Ito, Tadahiko; Okubo, Tadashi

    2002-12-01

    A female neonate with mitral stenosis due to accessory mitral valve with ventricular septal defect and patent ductus arteriosus is described. She was referred to our hospital because of neonatal asphyxia. Asphyxia was improved by ventilator support, but rapid deterioration of respiration with pulmonary congestion and hemorrhage appeared 8 days after birth. Echocardiography revealed an accessory mitral valve attached to the anterior mitral leaflet with a perimembranous ventricular septal defect and patent ductus arteriosus. Although there were no echocardiographical findings indicating mitral stenosis on admission, the mitral stenosis blood flow patterns were detected by color and pulsed Doppler examination performed on the eighth day after admission. Transaortic resection of accessory mitral valve tissue was performed with patch closure of the ventricular septal defect and ligation of the ductus arteriosus 35 days after birth. After operation, pulmonary congestion and hemorrhage were improved. Postoperative echocardiography showed complete resection of the accessory mitral valve and no mitral insufficiency. We concluded that the combination of the accessory mitral valve and left-to-right shunt due to ventricular septal defect or patent ducturs arteriosus might have led to a critical hemodynamic condition due to relative mitral stenosis in the neonatal period with the decrease in pulmonary vascular resistance.

  14. Increased pulmonary artery pressures during exercise are related to persistent tricuspid regurgitation after atrial septal defect closure.

    Science.gov (United States)

    De Meester, Pieter; Van De Bruaene, Alexander; Herijgers, Paul; Voigt, Jens-Uwe; Vanhees, Luc; Budts, Werner

    2013-08-01

    Although closure of an atrial septal defect type secundum often normalizes right heart dimensions and pressures, mild tricuspid insufficiency might persist. This study aimed at (1) identification of determinants explaining the persistence of tricuspid insufficiency after atrial septal defect closure, and (2) evaluation of functional capacity of patients with persistent mild tricuspid insufficiency. Twenty-five consecutive patients (age 42+17 y) were included from the outpatient clinic of congenital heart disease at the University Hospitals of Leuven. All underwent transthoracic echocardiography, semi-supine bicycle stress echocardiography and cardio-pulmonary exercise testing. Six patients (24%) had mild tricuspid insufficiency (2/4) compared to 19 patients (76%) with no or minimal tricuspid insufficiency ( 1/4) as assessed by semi-quantitative colour Doppler echocardiography. Mann-Whitney U and Fisher's exact tests were performed where applicable. Patients with persistent mild tricuspid insufficiency were significantly older than those with no or minimal tricuspid insufficiency (P = 0.042). At rest, no differences in right heart configuration, mean pulmonary artery pressure or right ventricular function were found. At peak exercise, mean pulmonary artery pressure was significantly higher in patients with mild persistent tricuspid insufficiency (P = 0.026). Peak oxygen uptake was significantly lower in patients with mild persistent tricuspid insufficiency (P = 0.019). Mild tricuspid insufficiency after atrial septal defect repair occurs more frequently in older patients and in patients with higher mean pulmonary artery pressure at peak exercise. In patients with mild tricuspid insufficiency, functional capacity was more reduced. Mild tricuspid insufficiency could be a marker of subclinical persistent pressure load on the right ventricle.

  15. Atrioventricular canal defect and associated genetic disorders: new insights into polydactyly syndromes

    Directory of Open Access Journals (Sweden)

    M. Cristina Digilio

    2011-07-01

    Full Text Available Atrioventricular canal defect (AVCD is a common congenital heart defect (CHD, representing 7.4% of all cardiac malformations, considered secondary to an extracellular matrix anomaly. The AVCD is associated with extracardiac defects in about 75% of the cases. In this review we analyzed different syndromic AVCDs, in particular those associated with polydactyly disorders, which show remarkable genotype-phenotype correlations. Chromo - some imbalances more frequently associated with AVCD include Down syndrome, deletion 8p23 and deletion 3p25, while mendelian disorders include Noonan syndrome and related RASopathies, several polydactyly syndromes, CHARGE and 3C (cranio-cerebello-cardiac syndrome. The complete form of AVCD is prevalent in patients with chromosomal imbalances. Additional cardiac defects are found in patients affected by chromosomal imbalances different from Down syndrome. Left-sided obstructive lesions are prevalently found in patients with RASopathies. Patients with deletion 8p23 often display AVCD with tetralogy of Fallot or with pulmonary valve stenosis. Tetralogy of Fallot is the only additional cardiac defect found in patients with Down syndrome and AVCD. On the other hand, the association of AVCD and tetralogy of Fallot is also quite characteristic of CHARGE and 3C syndromes. Heterotaxia defects, including common atrium and anomalous pulmonary venous return, occur in patients with AVCD associated with polydactyly syndromes (Ellis-van Creveld, short rib polydactyly, oral-facial-digital, Bardet-Biedl, and Smith-Lemli-Opitz syndromes. The initial clinical evidence of anatomic similarities between AVCD and heterotaxia in polydactyly syndromes was corroborated and explained by experimental studies in transgenic mice. These investigations have suggested the involvement of the Sonic Hedgehog pathway in syndromes with postaxial polydactyly and heterotaxia, and ciliary dysfunction was detected as pathomechanism for these disorders

  16. Transcatheter Retrieval of Embolized Atrial Septal Defect Occluder Device by Waist Capture Technique.

    Science.gov (United States)

    Her, Ae-Young; Lim, Kyung-Hun; Shin, Eun-Seok

    2018-01-27

    This case study describes the successful percutaneous transcatheter retrieval of an embolized Amplatzer occluder device using the "waist capture technique" in a patient with an atrial septal defect. This technique allowed for stability of the Amplatzer device, compression of the atrial discs for easier removal, prevention of further embolization, and minimal injury to vasculature during device retrieval. This novel and effective technique can be used safely for the retrieval of Amplatzer devices in the venous system.

  17. Betaine supplementation reduces congenital defects after prenatal alcohol exposure (Conference Presentation)

    Science.gov (United States)

    Karunamuni, Ganga; Gu, Shi; Doughman, Yong Qiu; Sheehan, Megan M.; Ma, Pei; Peterson, Lindsy M.; Linask, Kersti K.; Jenkins, Michael W.; Rollins, Andrew M.; Watanabe, Michiko

    2016-03-01

    Over 500,000 women per year in the United States drink during pregnancy, and 1 in 5 of this population also binge drink. As high as 20-50% of live-born children with prenatal alcohol exposure (PAE) present with congenital heart defects including outflow and valvuloseptal anomalies that can be life-threatening. Previously we established a model of PAE (modeling a single binge drinking episode) in the avian embryo and used optical coherence tomography (OCT) imaging to assay early-stage cardiac function/structure and late-stage cardiac defects. At early stages, alcohol/ethanol-exposed embryos had smaller cardiac cushions and increased retrograde flow. At late stages, they presented with gross morphological defects in the head and chest wall, and also exhibited smaller or abnormal atrio-ventricular (AV) valves, thinner interventricular septae (IVS), and smaller vessel diameters for the aortic trunk branches. In other animal models, the methyl donor betaine (found naturally in many foods such as wheat bran, quinoa, beets and spinach) ameliorates neurobehavioral deficits associated with PAE but the effects on heart structure are unknown. In our model of PAE, betaine supplementation led to a reduction in gross structural defects and appeared to protect against certain types of cardiac defects such as ventricular septal defects and abnormal AV valvular morphology. Furthermore, vessel diameters, IVS thicknesses and mural AV leaflet volumes were normalized while the septal AV leaflet volume was increased. These findings highlight the importance of betaine and potentially methylation levels in the prevention of PAE-related birth defects which could have significant implications for public health.

  18. Transcatheter closure of ventricular septal defects with nitinol wire occluders of type patent ductus arteriosus.

    Science.gov (United States)

    Wierzyk, Arkadiusz; Szkutnik, Małgorzata; Fiszer, Roland; Banaszak, Paweł; Pawlak, Szymon; Białkowski, Jacek

    2014-01-01

    Ventricular septal defects closure (VSD) depending on the anatomy and clinical setting can be performed surgically or by a hybrid and transcatheter approach. Two cases of children with VSD will be presented. Patients' defects were closed with various types of occluders made of nitinol wire mesh occluder, patent ductus arteriosus (PDA) type. The first case was a 2.5-year-old boy after cardiosurgical correction of tetralogy of Fallot (TOF). After the procedure, a significant haemodynamic residual VSD was observed, which was not successfully closed during the subsequent reoperation. Despite pharmacological treatment, symptoms of heart failure were observed in this patient. In echocardiographic images the residual VSD was presented as a tunnel-like dissection of the ventricular septum (length 6 mm and diameter 3.4 mm). The defect was closed via arterial access with an Amplatzer Duct Occluder II (ADO II). The procedure was successfully performed without any medical complications. In this child, a significant shunt reduction and a noticeable improvement in the patient's clinical status and diminished symptoms of heart failure were noticed. The second patient was a 4-year-old girl suffering from a multi-perforated perimembranous VSD accompanied by a ventricular septal defect with aneurysm. The defect was closed by a venous approach with a PDA Cardio-O-Fix occluder (very similar to ADO I). No short-term or long-term complications were visible during or after the procedure. Only a mild residual shunt through the VSD was observed 6 months afterwards. Transcatheter VSD closure with a proper morphology, with occluders of type Amplatzer Duct Occluder ADO I or ADO II, constitutes a safe and effective therapeutic alternative.

  19. Atrial and ventricular septal defect with pulmonary and tricuspid valvular anomalies in a dog

    International Nuclear Information System (INIS)

    Ishikawa, Y.; Wakao, Y.; Watanabe, T.; Minami, T.; Muto, M.; Suzuki, T.; Takahashi, M.; Une, Y.; Nomura, Y.; Ichioka, N.

    1989-01-01

    A 15-month-old male boxer dog weighing 22 kg was referred to Azabu University Animal Hospital for evaluation of the syncopal attack. There was no cardiac murmur, but electrocardiograms revealed an atrial fibrillation. Thoracic radiograph revealed enlargement of the right and left atrial regions. The medical treatment with digitalis and captopril was made for conversion from the atrial fibrillation to the sinus rhythm. By cardiac catheterization, atrial and ventricular septal defect with pulmonary stenosis was demonstrated. The patient died at 20 months from the first medical examination. At autopsy, there were severe enlargement of both atria, atrial defect, and pulmonary and tricuspid valvular anomalies. (author)

  20. Atrial and ventricular septal defect with pulmonary and tricuspid valvular anomalies in a dog

    Energy Technology Data Exchange (ETDEWEB)

    Ishikawa, Y. [Azabu Univ., Sagamihara, Kanagawa (Japan); Wakao, Y.; Watanabe, T.; Minami, T.; Muto, M.; Suzuki, T.; Takahashi, M.; Une, Y.; Nomura, Y.; Ichioka, N.

    1989-12-15

    A 15-month-old male boxer dog weighing 22 kg was referred to Azabu University Animal Hospital for evaluation of the syncopal attack. There was no cardiac murmur, but electrocardiograms revealed an atrial fibrillation. Thoracic radiograph revealed enlargement of the right and left atrial regions. The medical treatment with digitalis and captopril was made for conversion from the atrial fibrillation to the sinus rhythm. By cardiac catheterization, atrial and ventricular septal defect with pulmonary stenosis was demonstrated. The patient died at 20 months from the first medical examination. At autopsy, there were severe enlargement of both atria, atrial defect, and pulmonary and tricuspid valvular anomalies. (author)

  1. Double-chambered right ventricle, ventricular septal defect, patent ductus arteriosus in a dog

    Energy Technology Data Exchange (ETDEWEB)

    Morozumi, M. [Togasaki Animal Hospital, Misato, Saitama (Japan); Kurosu, Y.; Kogure, K.; Chimura, S.; Shibata, S.; Kanemoto, I.

    1989-12-15

    A 4-month-old female mongrel puppy was presented with an anophthalmos. On physical examination, systolic murmur was heard at the 4th left intercostal space near the sternum. However the dog appeared healthy without cyanosis and had no history of exercise intolerance. The phonocardiogram revealed a pansystolic murmur and a continuous murmur on the mitral area. A systolic ejection murmur was also recorded on the pulmonic area. The electrocardiogram indicated bi-ventricular hypertrophy. Left ventricular enlargement was seen on chest radiographs. Ventricular septal defect (VSD) and patent ductus arteriosus (PDA) were diagnosed from these findings. PDA closure was performed at 2 years of age. After 2 months from the operation, the dog died during an attempted repair of the VSD. At necropsy, it was found that the double-chambered right ventricle (DCRV) was formed by an anomalous septal band. The VSD was localized on the proximal conus and was 8 mm in diameter. (author)

  2. Double-chambered right ventricle, ventricular septal defect, patent ductus arteriosus in a dog

    International Nuclear Information System (INIS)

    Morozumi, M.; Kurosu, Y.; Kogure, K.; Chimura, S.; Shibata, S.; Kanemoto, I.

    1989-01-01

    A 4-month-old female mongrel puppy was presented with an anophthalmos. On physical examination, systolic murmur was heard at the 4th left intercostal space near the sternum. However the dog appeared healthy without cyanosis and had no history of exercise intolerance. The phonocardiogram revealed a pansystolic murmur and a continuous murmur on the mitral area. A systolic ejection murmur was also recorded on the pulmonic area. The electrocardiogram indicated bi-ventricular hypertrophy. Left ventricular enlargement was seen on chest radiographs. Ventricular septal defect (VSD) and patent ductus arteriosus (PDA) were diagnosed from these findings. PDA closure was performed at 2 years of age. After 2 months from the operation, the dog died during an attempted repair of the VSD. At necropsy, it was found that the double-chambered right ventricle (DCRV) was formed by an anomalous septal band. The VSD was localized on the proximal conus and was 8 mm in diameter. (author)

  3. Potts shunt in a child with end-stage pulmonary hypertension after late repair of ventricular septal defect

    DEFF Research Database (Denmark)

    Petersen, Cecilie; Helvind, Morten; Jensen, Tim

    2013-01-01

    We report on a 10-year-old boy with medically refractory pulmonary arterial hypertension (PAH) and end-stage right heart failure after closure of a ventricular septal defect. The boy was a candidate for lung transplantation (LTX), but an alternative option was to create an Eisenmenger physiology ...

  4. Hybrid management of a large atrial septal defect and a patent ductus arteriosus in an infant with chronic lung disease

    Directory of Open Access Journals (Sweden)

    Pedra Simone

    2010-01-01

    Full Text Available We report a case wherein a dysmorphic four-month-old infant (weighing 4.5 kgs with an 8 mm atrial septal defect (ASD, a 1.5 mm patent ductus arteriosus (PDA, a 2 mm mid-muscular ventricular septal defect (VSD associated with chronic lung disease, and severe pulmonary hypertension, was successfully managed using a hybrid approach, without the use of cardiopulmonary bypass (CPB. Through a median sternotomy, the PDA was ligated and the ASD was closed with a 9 mm Amplatzer septal occluder implanted through peratrial access. The VSD was left untouched. Serial echocardiograms showed complete closure of the ASD and PDA, with progressive normalization of the pulmonary artery (PA pressures within three months. The child rapidly gained weight and was weaned from sildenafil and oxygen administration. After 12 months, the VSD closed spontaneously and the child remained well, with normal PA pressures. A hybrid approach without the use of CPB should be considered in the management of infants with congenital heart disease, associated with chronic lung disease and pulmonary hypertension.

  5. Hybrid management of a large atrial septal defect and a patent ductus arteriosus in an infant with chronic lung disease

    International Nuclear Information System (INIS)

    Pedra, Simone F; Jatene, Marcelo; Pedra, Carlos AC

    2010-01-01

    We report a case wherein a dysmorphic four-month-old infant (weighing 4.5 kgs) with an 8 mm atrial septal defect (ASD), a 1.5 mm patent ductus arteriosus (PDA), a 2 mm mid-muscular ventricular septal defect (VSD) associated with chronic lung disease, and severe pulmonary hypertension, was successfully managed using a hybrid approach, without the use of cardiopulmonary bypass (CPB). Through a median sternotomy, the PDA was ligated and the ASD was closed with a 9 mm Amplatzer septal occluder implanted through peratrial access. The VSD was left untouched. Serial echocardiograms showed complete closure of the ASD and PDA, with progressive normalization of the pulmonary artery (PA) pressures within three months. The child rapidly gained weight and was weaned from sildenafil and oxygen administration. After 12 months, the VSD closed spontaneously and the child remained well, with normal PA pressures. A hybrid approach without the use of CPB should be considered in the management of infants with congenital heart disease, associated with chronic lung disease and pulmonary hypertension

  6. Hybrid management of a large atrial septal defect and a patent ductus arteriosus in an infant with chronic lung disease

    Energy Technology Data Exchange (ETDEWEB)

    Pedra, Simone F; Jatene, Marcelo; Pedra, Carlos AC, E-mail: carlosacpedra@hotmail.com [Hospital do Coração da Associação Sanatório Sírio, São Paulo (Brazil)

    2010-01-01

    We report a case wherein a dysmorphic four-month-old infant (weighing 4.5 kgs) with an 8 mm atrial septal defect (ASD), a 1.5 mm patent ductus arteriosus (PDA), a 2 mm mid-muscular ventricular septal defect (VSD) associated with chronic lung disease, and severe pulmonary hypertension, was successfully managed using a hybrid approach, without the use of cardiopulmonary bypass (CPB). Through a median sternotomy, the PDA was ligated and the ASD was closed with a 9 mm Amplatzer septal occluder implanted through peratrial access. The VSD was left untouched. Serial echocardiograms showed complete closure of the ASD and PDA, with progressive normalization of the pulmonary artery (PA) pressures within three months. The child rapidly gained weight and was weaned from sildenafil and oxygen administration. After 12 months, the VSD closed spontaneously and the child remained well, with normal PA pressures. A hybrid approach without the use of CPB should be considered in the management of infants with congenital heart disease, associated with chronic lung disease and pulmonary hypertension.

  7. Atrial septal defect closure with the new Cardia Ultrasept II™ device with interposed Goretex patch: Mexican experience - has the perforation of Ivalon's membrane been solved?

    Science.gov (United States)

    Mijangos-Vázquez, Roberto; García-Montes, Antonio J; Soto-López, Elena M; Guarner-Lans, Verónica; Zabal, Carlos

    2018-05-01

    The objective of this study was to demonstrate the safety and feasibility of using the new Cardia Ultrasept II™ device with interposed Goretex patch referring to the perforation of polyvinyl alcohol membrane. Great advances have been made in the development of devices for closure of atrial septal defect. The Cardia Ultrasept II™ with interposed Goretex patch is the modified last generation of Cardia devices, having the advantage of a super-low profile within the atria and an integral locking delivery-retrieval mechanism that ensures safe deployment. In addition, with the interposition of the Goretex, it has been possible to abolish perforation of Ivalon's membrane as a complication.Methods and resultsPatients with ostium secundum atrial septal defect with surrounding rims with a minimum length of 5 mm and who underwent atrial septal defect closure with the new Ultrasept II™ with Goretex patch were included from two paediatric cardiac centres. Primary end point was to determine perforation of the Goretex membrane at follow-up; secondary end point included right ventricular diastolic diameter. In total, 30 patients underwent atrial septal defect closure at a median age of 6 (1-29) years. At follow-up for 6 (range, 1-15) months, freedom from perforations was 100%. A continuous decrease in right ventricular diastolic diameter was found with an initial median of 30 (25-49) mm and after catheterisation of 27.5 (18-33) mm, p=0.01, and Z-score of 2.6 (1.7-3.6) versus 1.9 (1-2.9) after procedure, p=0.01. The new modified generation of the Ultrasept II™ device with interposed Goretex patch is a good alternative to achieve atrial septal defect closure safely and feasibly with no membrane perforation at follow-up.

  8. Unsuccessful lung scan due to major right-to-left shunt through a sinus venosus septal defect

    International Nuclear Information System (INIS)

    Brendel, A.J.; Larnaudie, B.; Lambert, B.; Leccia, F.; Barat, J.L.; Ducassou, D.; Fontan, F.

    1985-01-01

    In a patient with a prior history of cerebral abscess and cerebral ischemia, an unsuccessful perfusion lung scan led to a radionuclide angiocardiogram using an arm vein injection. This showed a total right-to-left (R-L) shunt from the superior vena cava (SVC) to the left atrium. Repeat radionuclide study, through a leg vein, demonstrated a moderate R-L shunt and an interpretable lung scan could be obtained. Catheterization and contrast cineangiogram did not provide the exact diagnosis, the preoperative conclusion being anomalous drainage of the SVC into the left atrium, with atrial septal defect (ASD) and partial anomalous pulmonary venous connection to the SVC. The operative diagnosis was high atrial (sinus venosus) septal defect. This example of major but clinically unsuspected R-L shunt emphasizes the value of performing a perfusion lung scan, preferably in conjunction with radionuclide angiocardiography in patients with a prior history of unexplained cerebral abscess or systemic ischemia. Implications of the site of an ASD on quantitation of L-R shunts by radionuclide methods are also discussed

  9. Late infectious endocarditis of surgical patch closure of atrial septal defects diagnosed by 18F-fluorodeoxyglucose gated cardiac computed tomography (18F-FDG-PET/CT): a case report.

    Science.gov (United States)

    Honnorat, Estelle; Seng, Piseth; Riberi, Alberto; Habib, Gilbert; Stein, Andreas

    2016-08-24

    In contrast to percutaneous atrial septal occluder device, surgical patch closure of atrial defects was known to be no infective endocarditis risk. We herein report the first case of late endocarditis of surgical patch closure of atrial septal defects occurred at 47-year after surgery. On September 2014, a 56-year-old immunocompetent French Caucasian man was admitted into the Emergency Department for 3-week history of headache, acute decrease of psychomotor performance and fever at 40 °C. The diagnosis has been evoked during his admission for the management of a brain abscess and confirmed using 18F-fluorodeoxyglucose gated cardiac computed tomography (18F-FDG-PET/CT). Bacterial cultures of surgical deep samples of brain abscess were positive for Streptococcus intermedius and Aggregatibacter aphrophilus as identified by the matrix-assisted laser desorption/ionization-time of flight (MALDI-TOF) mass spectrometry and confirmed with 16S rRNA gene sequencing. The patient was treated by antibiotics for 8 weeks and surgical patch closure removal. In summary, late endocarditis on surgical patch and on percutaneous atrial septal occluder device of atrial septal defects is rare. Cardiac imaging by the 18F-fluorodeoxyglucose gated cardiac computed tomography (18F-FDG-PET/CT) could improve the diagnosis and care endocarditis on surgical patch closure of atrial septal defects while transthoracic and transesophageal echocardiography remained difficult to interpret.

  10. Nuclear magnetic resonance zeugmatographic imaging of the heart: application to the study of ventricular septal defect

    International Nuclear Information System (INIS)

    Heneghan, M.A.; Biancaniello, T.M.; Heidel, E.; Peterson, S.B.; Marsh, M.J.; Lauterbur, P.C.

    1982-01-01

    The present work was undertaken to determine the applicability of nuclear magnetic resonance (NMR) imaging to the study of congenital heart disease. Three-dimensional proton density images of preserved lamb hearts with and without an artificially created ventricular septal defect were reconstructed and displayed in multiple planes. Sections obtained in the sagittal plane through the ventricular septum clearly showed the size, shape, and location of the defect. Results of these experiments suggest that NMR zeugmatography will become a valuable addition to existing imaging techniques for the study of congenital heart disease

  11. Ventricular Septal Dissection Complicating Inferior Wall Myocardial Infarction

    Directory of Open Access Journals (Sweden)

    Lindsey Kalvin

    2017-01-01

    Full Text Available Postmyocardial infarction ventricular septal defect is an increasingly rare mechanical complication of acute myocardial infarction. We present a case of acute myocardial infarction from right coronary artery occlusion that developed hypotension and systolic murmur 12 hours after successful percutaneous coronary intervention. Although preoperative imaging suggested a large ventricular septal defect and a pseudoaneurysm, intraoperative findings concluded a serpiginous dissection of the ventricular septum. The imaging technicalities are discussed.

  12. Virtual reality 3D echocardiography in the assessment of tricuspid valve function after surgical closure of ventricular septal defect

    NARCIS (Netherlands)

    G. Bol-Raap (Goris); A.H.J. Koning (Anton); T.V. Scohy (Thierry); A.D.J. ten Harkel (Arend); F.J. Meijboom (Folkert); A.P. Kappetein (Arie Pieter); P.J. van der Spek (Peter); A.J.J.C. Bogers (Ad)

    2007-01-01

    textabstractBackground. This study was done to investigate the potential additional role of virtual reality, using three-dimensional (3D) echocardiographic holograms, in the postoperative assessment of tricuspid valve function after surgical closure of ventricular septal defect (VSD). Methods. 12

  13. Right ventricular load and function during exercise in patients with open and closed atrial septal defect type secundum

    NARCIS (Netherlands)

    Pieter De Meester; Roselien Buys; Werner Budts; Marion Delcroix; Prof. Dr. Luc L.E.M.J. Vanhees; Jens-Uwe Voigt; Alexander Van de Bruaene

    2013-01-01

    Abstract Purpose: This study aimed at evaluating (1) right ventricular (RV) mean power during exercise, (2) the contribution of flow and pressure to RV mean power, and (3) the impact of pulmonary artery pressure on RV function during exercise. Methods: Fifty patients with atrial septal defect (ASD)

  14. Oclusión transitoria de comunicación interauricular en el síndrome de Lutembacher Temporary occlusion of atrial septal defect in the Lutembacher syndrome

    Directory of Open Access Journals (Sweden)

    Alejandro E. Contreras

    2011-08-01

    Full Text Available Se describe una paciente de 82 años de edad, con síntomas de insuficiencia cardíaca avanzada e hipertensión arterial pulmonar. Un ecocardiograma transtorácico mostró una comunicación interauricular tipo ostium secundum y estenosis valvular mitral concomitante (síndrome de Lutembacher. La valoración ecocardiográfica de la enfermedad mitral se vio dificultada por la presencia del defecto interauricular. Se realizó test de oclusión percutánea transitoria de la comunicación interauricular, observándose la aparición de estenosis valvular mitral grave. El tamaño del defecto interauricular modificó las manifestaciones clínicas y el test de oclusión transitoria ayudó a decidir la conducta terapéutica.We report the case of an 82 year-old woman with symptoms of advanced heart failure and pulmonary arterial hypertension. An echocardiogram showed an ostium secundum type atrial septal defect and concomitant mitral valve stenosis (Lutembacher syndrome. Echocardiographic assessment of mitral pathology was hampered by the interatrial septal defect. Transient percutaneous occlusion test of the atrial septal defect was performed and severe mitral valve stenosis was detected. Atrial septal defect size modified the clinical manifestations and the transient occlusion test helped to decide the therapeutic strategy.

  15. Long-term mortality in patients with atrial septal defect

    DEFF Research Database (Denmark)

    Nyboe, Camilla; Karunanithi, Zarmiga; Nielsen-Kudsk, Jens Erik

    2018-01-01

    Aims: In this nationwide cohort of atrial septal defect (ASD) patients, the largest to date, we report the longest follow-up time with and without closure in childhood and adulthood compared with a general population cohort. Methods and results: Using population-based registries, we included Danish...... individuals born before 1994 who received an ASD diagnosis between 1959 and 2013. All diagnoses were subsequently validated (n = 2277). Using the Kaplan-Meier estimates and Cox proportional hazards regression adjusted for sex, birth year, and a modified Charlson Comorbidity Index, we compared the mortality...... of ASD patients with that of a birth year and sex matched general population cohort. The median follow-up from ASD diagnosis was 18.1 years (range 1-53 years). Patients with ASD had a higher mortality [adjusted hazard ratio (HR): 1.7; 95% confidence interval (CI): 1.5-1.9] compared with the general...

  16. Patent ductus arteriosus closure using an Amplatzer™ ventricular septal defect closure device

    Science.gov (United States)

    Fernando, Rajeev; Koranne, Ketan; Loyalka, Pranav; Kar, Biswajit; Gregoric, Igor

    2013-01-01

    The ductus arteriosus originates from the persistence of the distal portion of the left sixth aortic arch. It connects the descending aorta (immediately distal to the left subclavian artery) to the roof of the main pulmonary artery, near the origin of the left pulmonary artery. Persistence of the duct beyond 48 h after birth is abnormal and results in patent ductus arteriosus (PDA). PDA is rare in adults because it is usually discovered and treated in childhood. Mechanical closure remains the definitive therapy because the patency of ductus arteriosus may lead to multiple complications, depending on the size and flow through the ductus. PDA closure is indicated in patients with symptoms and evidence of left heart enlargement, and in patients with elevated pulmonary pressures when reversal is possible. Transcatheter closure is the preferred technique in adults because it avoids sternotomy, reduces the length of hospital stay and is associated with fewer complications compared with surgery. First demonstrated in 1967, both the technique and the occluder devices used have since evolved. However, designing an ideal PDA occluder has been a challenge due to the variability in size, shape and orientation of PDAs. The present article describes a case involving a 35-year-old woman who presented to the Center for Advanced Heart Failure (Houston, USA) with congestive heart failure due to a large PDA, which was successfully occluded using an Amplatzer (St Jude Medical, USA) muscular ventricular septal defect closure device. The wider waist and dual-retention discs of these ventricular septal defect closure devices may be important factors to consider in the future development of devices for the occlusion of large PDAs. PMID:24294051

  17. Dilatation of the Great Arteries in an Infant with Marfan Syndrome and Ventricular Septal Defect

    Directory of Open Access Journals (Sweden)

    L. Rozendaal

    2011-01-01

    Full Text Available We describe an infant presenting with contractures of the fingers, a large ventricular septal defect (VSD, and severe pulmonary artery dilatation. He had clinical and echocardiographic features of both neonatal or infantile Marfan syndrome (MFS and congenital contractural arachnodactyly. After surgical VSD closure, the aortic root developed progressive dilatation while the size of pulmonary artery returned to normal limits. Eventually the diagnosis of MFS was confirmed by DNA analysis.

  18. Situs inversus totalis associated with subaortic stenosis, restrictive ventricular septal defect, and tricuspid dysplasia in an adult dog.

    Science.gov (United States)

    Piantedosi, Diego; Cortese, Laura; Meomartino, Leonardo; Di Loria, Antonio; Ciaramella, Paolo

    2011-11-01

    A rare association between situs inversus totalis (SIT), restrictive ventricular septal defect, severe subaortic stenosis, and tricuspid dysplasia was observed in an adult mixed-breed dog. Primary ciliary dyskinesia and Kartagener's syndrome were excluded. After 15 mo the dog died suddenly. The association between SIT and congenital heart diseases is discussed.

  19. Follow-up of P dispersion after transcatheter closure of an atrial septal defect in children

    International Nuclear Information System (INIS)

    Baspinar, O.; Kervancioglu, M.; Kilinc, M.; Irdem, A.; Koruk, S.

    2014-01-01

    Objectives: To determine in paediatric patients with atrial septal defects whether differences in P wave dispersion occurred with transcatheter closures using the Amplatzer septal occluder. Method: A total of 31 children who had undergone transcathater closures were evaluated. P maximum, P minimum, and P dispersion were measured with 12-lead surface electrocardiography, before the procedure and one week, one month, three months, six months and one year following the procedure. SPSS 10 was used for statistical analysis. Results: There were 23 (74.2%) females and 8 (25.8%) males with an overall mean age of 7.5+-4.1 years and mean weight of 26.2+-16.9kg. The P maximum and P minimum measurements differed between patients during the follow-up period. Both measurements decreased with time. However, P dispersion was not significantly different throughout the follow-up period (before the procedure P maximum 95.4+-15.6 ms, P minimum 64.5+-15.4 ms, and P dispersion 30.8+-11.4ms; one year later, P maximum 76.1+-14.6 ms, P minimum 47.1+-12.1 ms, and P dispersion 29.1+-9.1ms). Conclusion: Over time, there are no P dispersion differences in transcatheter closures using the Amplatzer septal occluder. (author)

  20. Situs inversus totalis associated with subaortic stenosis, restrictive ventricular septal defect, and tricuspid dysplasia in an adult dog

    OpenAIRE

    Piantedosi, Diego; Cortese, Laura; Meomartino, Leonardo; Di Loria, Antonio; Ciaramella, Paolo

    2011-01-01

    A rare association between situs inversus totalis (SIT), restrictive ventricular septal defect, severe subaortic stenosis, and tricuspid dysplasia was observed in an adult mixed-breed dog. Primary ciliary dyskinesia and Kartagener’s syndrome were excluded. After 15 mo the dog died suddenly. The association between SIT and congenital heart diseases is discussed.

  1. Atrial septal stenting - How I do it?

    Directory of Open Access Journals (Sweden)

    Kothandam Sivakumar

    2015-01-01

    Full Text Available A wide atrial communication is important to maintain hemodynamics in certain forms of congenital and acquired heart defects. In comparison to balloon septostomy or blade septostomy, atrial septal stenting provides a controlled, predictable, and long-lasting atrial communication. It often needs a prior Brockenbrough needle septal puncture to obtain a stable stent position. A stent deployed across a previously dilated and stretched oval foramen or tunnel form of oval foramen carries higher risk of embolization. This review provides technical tips to achieve a safe atrial septal stenting. Even though this is a "How to do it article," an initial discussion about the indications for atrial septal stenting is vital as the resultant size of the atrial septal communication should be tailored for each indication.

  2. THE RESTRICTED SURGICAL RELEVANCE OF MORPHOLOGIC CRITERIA TO CLASSIFY SYSTEMIC-PULMONARY COLLATERAL ARTERIES IN PULMONARY ATRESIA WITH VENTRICULAR SEPTAL-DEFECT

    NARCIS (Netherlands)

    DERUITER, MC; GITTENBERGERDEGROOT, AC; BOGERS, AJJC; ELZENGA, NJ

    1994-01-01

    Now that systemic-pulmonary collateral arteries are used for unifocalization in patients with pulmonary atresia and ventricular septal defect, the question arises whether morphologic criteria of these collateral arteries could help to provide better results. In an attempt to classify the morphologic

  3. Anaesthetic management of a child with "cor-triatriatum" and multiple ventricular septal defects - A rare congenital anomaly

    Directory of Open Access Journals (Sweden)

    Sriram Sabade

    2010-01-01

    Full Text Available Cor-triatriatum is a rare congenital cardiac anomaly. It accounts for 0.1% of congenital heart diseases. Its association with multiple ventricular septal defects (VSD is even rarer. A five-month-old baby was admitted with respiratory distress and failure to thrive. Clinical examination revealed diastolic murmur over mitral area. Chest X-ray showed cardiomegaly. Haematological and biochemical investigations were within normal limits. Electrocardiogram showed left atrial enlargement. 2D echo showed double-chambered left atrium (cor-triatriatum, atrial septal defect (ASD and muscular VSD with moderate pulmonary arterial hypertension. The child was treated with 100% oxygen, diuretics and digoxin and was stabilized medically. We used balanced anaesthetic technique using oxygen, air, isoflurane, fentanyl, midazolam and vecuronium. Patient was operated under cardiopulmonary bypass (CPB with moderate hypothermia. Through right atriotomy abnormal membrane in the left atrium was excised to make one chamber. VSD were closed with Dacron patches and ASD was closed with autologous pericardial patch. Patient tolerated the whole procedure well and was ventilated electively for 12h in the intensive care unit. He was discharged on the 10 th postoperative day.

  4. Anesthetic management of Amplatzer atrial septal defect closure device embolization to right ventricular outflow tract

    Directory of Open Access Journals (Sweden)

    S Das

    2016-01-01

    Full Text Available Percutaneous device closure of atrial septal defect (ASD is an alternative treatment to surgery with advantages of avoidance of surgery, short procedure time, early discharge from hospital, and lower rates of complications. However, percutaneous device closure is associated with infrequent life-threatening complications such as device embolization. We report a case device embolization of the ASD occlude device into right ventricular outflow tract resulting progressive hypoxia. The role of anesthesiologist as a team leader in managing such emergency is discussed.

  5. Clinical differentiation of patent foramen ovale and secundum atrial septal defect: a survey of pediatric cardiologists in Dallas, Texas, USA.

    Science.gov (United States)

    Scheuerle, Angela

    2011-01-01

    Public health birth defect surveillance registries rely on health care provider diagnosis and definition of congenital anomalies. Major anomalies are likely to have consistent diagnoses across providers; however, definition of some more common, often minor, defects can be problematic. Of particular frustration are the transient neonatal heart findings: patent ductus arteriosus, patent foramen ovale, and pulmonary artery branch stenosis. Under certain circumstances these findings may be considered true anomalies-patent foramen ovale (PFO) as a clinical finding overlaps significantly with atrial septal defect (ASD) of secundum type, the latter being considered a true congenital malformation. Some criteria must be established to separate these conditions in case ascertainment. It is therefore helpful to understand the clinical definitions of patent foramen ovale and secundum atrial septal defect. Pediatric cardiologists in the greater Dallas, Texas metropolitan area were surveyed by telephone, fax, and/or email and asked what criteria they use to distinguish a PFO from a secundum ASD. This was an open-ended question. No baseline parameters were suggested or introduced by the interviewer. Pediatric cardiology fellowship training was identified for each physician to examine the hypothesis that graduates of a given program would use the same diagnostic criteria. Responses were obtained from 22 of 23 pediatric cardiologists. Four measurement criteria were identified: size of the opening, presence or absence of a flap of septal tissue, appearance of the defect on echocardiogram and presence/absence/amount of blood shunting across through the opening. Though there was overlap, diagnostic criteria differentiating PFO and secundum ASD varied among pediatric cardiologists. Two fellowship programs were well represented by the respondent population. Eight respondents were trained at Fellowship 1 and 5 at Fellowship 2. Place of fellowship training was not a strong indicator of

  6. Patent ductus arteriosus closure using an Amplatzer(™) ventricular septal defect closure device.

    Science.gov (United States)

    Fernando, Rajeev; Koranne, Ketan; Loyalka, Pranav; Kar, Biswajit; Gregoric, Igor

    2013-01-01

    The ductus arteriosus originates from the persistence of the distal portion of the left sixth aortic arch. It connects the descending aorta (immediately distal to the left subclavian artery) to the roof of the main pulmonary artery, near the origin of the left pulmonary artery. Persistence of the duct beyond 48 h after birth is abnormal and results in patent ductus arteriosus (PDA). PDA is rare in adults because it is usually discovered and treated in childhood. Mechanical closure remains the definitive therapy because the patency of ductus arteriosus may lead to multiple complications, depending on the size and flow through the ductus. PDA closure is indicated in patients with symptoms and evidence of left heart enlargement, and in patients with elevated pulmonary pressures when reversal is possible. Transcatheter closure is the preferred technique in adults because it avoids sternotomy, reduces the length of hospital stay and is associated with fewer complications compared with surgery. First demonstrated in 1967, both the technique and the occluder devices used have since evolved. However, designing an ideal PDA occluder has been a challenge due to the variability in size, shape and orientation of PDAs. The present article describes a case involving a 35-year-old woman who presented to the Center for Advanced Heart Failure (Houston, USA) with congestive heart failure due to a large PDA, which was successfully occluded using an Amplatzer (St Jude Medical, USA) muscular ventricular septal defect closure device. The wider waist and dual-retention discs of these ventricular septal defect closure devices may be important factors to consider in the future development of devices for the occlusion of large PDAs.

  7. The establishment of atrial septal defect model with interventional management canine: its applied anatomy and technical points

    International Nuclear Information System (INIS)

    Zhu Yufeng; Huang Xinmiao; Bei Yuan; Wang Wei; Hu Jianqiang; Qin Yongwen

    2010-01-01

    Objective: To provide the relevant applied anatomic information for the preparation of atrial septal defect (ASD) model with transcatheter management in canine,and to discuss the technical points in making ASD model under DSA guidance. Methods: Anatomical measurements of the heart specimens,which were obtained from 15 healthy adult hybrid dogs (9 males and 6 females), were performed, from which the relevant anatomic parameters of the atrial septum were calculated. Cardiac 3D reconstruction with 64-sliced spiral CT scan was carried out in 5 dogs and the results were analyzed. According to the trans-illuminated position and angle obtained from 3D reconstruction images both the puncturing of the atrial septum with Brokenbrough needle and the balloon dilatation under fluoroscopic guidance were conducted in 20 dogs (body weight 17 -22 kg) to prepare ASD model. Results: The length and the width of the interauricular septum were (17.8 ± 4.3) mm and (14.5 ± 3.8) mm, respectively. The oval fossa was (11.2 ± 2.7) mm long and (8.7±1.9) mm wide. The distance from the central point of oval fossa to the central point of the orifice of coronary sinus was (7.2 ± 1.3) mm, which was (9.9 ± 1.5) mm to the center of the membranous atrial septum, (13.6 ± 3.1) mm to the middle point of septal tricuspid valve, (12.1 ± 2.3) mm to the central point of the bottom of aortic eminence and (11.3 ± 1.9) mm to the middle point of anterior bicuspid valve. The angle between atrial septal plane and sagittal plane was 15 degree ± 5 degree, and the angle between atrial septal plane and coronal plane was 75 degree ± 5 degree. Thus, the puncture of the interauricular septum was carried out with the dog in right anterior oblique position at 75 degree ± 5 degree. Of the total 20 dogs, ASD model was successfully established in 18, failure of the puncturing occurred in the remaining two, of which one died of cardiac tamponade after the procedure and the other one died of mistakenly puncturing

  8. Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease

    NARCIS (Netherlands)

    Engelfriet, Peter M.; Duffels, Marielle G. J.; Möller, Thomas; Boersma, Eric; Tijssen, Jan G. P.; Thaulow, Erik; Gatzoulis, Michael A.; Mulder, Barbara J. M.

    2007-01-01

    AIM: To investigate the role of pulmonary arterial hypertension (PAH) in adult patients born with a cardiac septal defect, by assessing its prevalence and its relation with patient characteristics and outcome. METHODS AND RESULTS: From the database of the Euro Heart Survey on adult congenital heart

  9. Congenital ventricular septal defects and prenatal exposure to cyclooxygenase inhibitors

    Directory of Open Access Journals (Sweden)

    F. Burdan

    2006-07-01

    Full Text Available Ventricular septal defects (VSDs are common congenital abnormalities which have been reported to be associated with maternal fever and various environmental factors. The aim of the present study was to evaluate the effect of prenatal exposure to cyclooxygenase (COX inhibitors on heart defects. A retrospective statistical analysis was performed using data collected in our laboratory during various teratological studies carried out on albino CRL:(WIWUBR Wistar strain rats from 1997 to 2004. The observations were compared with concurrent and historic control data, as well as findings from other developmental toxicological studies with selective and nonselective COX-2 inhibitors. Despite the lack of significant differences in the frequency of VSDs between drug-exposed and control groups, statistical analysis by the two-sided Mantel-Haenszel test and historical control data showed a higher incidence of heart defects in offspring exposed to nonselective COX inhibitors (30.06/10,000. Unlike other specific inhibitors, aspirin (46.26/10,000 and ibuprofen (106.95/10,000 significantly increased the incidence of the VSD when compared with various control groups (5.38-19.72/10,000. No significant differences in length or weight were detected between fetuses exposed to COX inhibitors and born with VSD and non-malformed offsprings. However, a statistically significant increase of fetal body length and decrease of body mass index were found in fetuses exposed to COX inhibitors when compared with untreated control. We conclude that prenatal exposure to COX inhibitors, especially aspirin and ibuprofen, increased the incidence of VSDs in rat offspring but was not related to fetal growth retardation.

  10. Small atrial septal defect associated with heart failure in an infant with a marginal left ventricle

    Directory of Open Access Journals (Sweden)

    Sandra D.K. Kingma

    2012-07-01

    Full Text Available Atrial septal defect (ASD is usually asymptomatic in infancy, unless pulmonary hypertension or severe co-morbidity is present. We report a case of a 4-week-old infant with moderate- sized ASD, small patent ductus arteriosus (PDA, and a borderline sized left ventricle that developed heart failure. Despite the relatively small diameter of the ASD, this defect influenced the mechanism of heart failure significantly. After surgical closure of both PDA and ASD, the signs of pulmonary hypertension resolved and the patient developed a normal sized left ventricle. This report illustrates that the presence of a small ASD in combination with a marginal left ventricle may result in inadequate left ventricular filling, pulmonary hypertension and heart failure.

  11. Waldmann's Disease (Primary Intestinal Lymphangiectasia) with Atrial Septal Defect.

    Science.gov (United States)

    Aroor, Shrikiran; Mundkur, Suneel; Kanaparthi, Shravan; Kumar, Sandeep

    2017-04-01

    Waldmann's disease or Primary Intestinal Lymphangiectasia (PIL) is a rare disorder of gastrointestinal tract characterized by dilated lymphatics and widened villi causing leakage of lymph into intestinal lumen. Loss of lymph leads to hypoalbuminemia, hyogammaglobulinemia and lymphopenia. Secondary lymphangiectasia occurs secondary to an elevated lymphatic pressure as in lymphoma, systemic lupus erythematosus, constrictive pericarditis, cardiac surgeries (Fontan's procedure), inflammatory bowel disease and malignancies. We, hereby present a five-year-old male child who presented with abdominal distension and poor weight gain. He had hypoalbuminemia, lymphocytopenia and hypogammaglobulinemia. Upper gastrointestinal endoscopy showed normal gastric mucosa and punctate white lesions in duodenal mucosa with biopsy confirming intestinal lymphangiectasia. Secondary causes of intestinal lymphangiectasia were ruled out. Echocardiography revealed atrial septal defect which is an uncommon association with Waldmann's disease. He was started on low fat, high protein diet and medium chain triglyceride supplementation following which he improved symptomatically. High index of suspicion, early diagnosis and appropriate dietary treatment are necessary to alleviate symptoms as well as to achieve a sustainable growth and development in these children.

  12. Use of sildenafil citrate in a cat with Eisenmenger’s syndrome and an atrial septal defect

    Directory of Open Access Journals (Sweden)

    Ke Shuan Chow

    2015-04-01

    Full Text Available A 2-year-old male neutered Siamese cat presenting with weakness and dyspnoea was diagnosed with an atrial septal defect and pulmonary hypertension, which resulted in right-to-left shunting (Eisenmenger’s syndrome. The cat was treated with sildenafil (0.25–0.6 mg/kg for 10 months. There were no apparent treatment-related adverse effects. Improvement in clinical signs was noted, although increasing doses of sildenafil were required. After 10 months the cat significantly deteriorated and was euthanased.

  13. Foxf genes integrate tbx5 and hedgehog pathways in the second heart field for cardiac septation.

    Directory of Open Access Journals (Sweden)

    Andrew D Hoffmann

    2014-10-01

    Full Text Available The Second Heart Field (SHF has been implicated in several forms of congenital heart disease (CHD, including atrioventricular septal defects (AVSDs. Identifying the SHF gene regulatory networks required for atrioventricular septation is therefore an essential goal for understanding the molecular basis of AVSDs. We defined a SHF Hedgehog-dependent gene regulatory network using whole genome transcriptional profiling and GLI-chromatin interaction studies. The Forkhead box transcription factors Foxf1a and Foxf2 were identified as SHF Hedgehog targets. Compound haploinsufficiency for Foxf1a and Foxf2 caused atrioventricular septal defects, demonstrating the biological relevance of this regulatory network. We identified a Foxf1a cis-regulatory element that bound the Hedgehog transcriptional regulators GLI1 and GLI3 and the T-box transcription factor TBX5 in vivo. GLI1 and TBX5 synergistically activated transcription from this cis-regulatory element in vitro. This enhancer drove reproducible expression in vivo in the posterior SHF, the only region where Gli1 and Tbx5 expression overlaps. Our findings implicate Foxf genes in atrioventricular septation, describe the molecular underpinnings of the genetic interaction between Hedgehog signaling and Tbx5, and establish a molecular model for the selection of the SHF gene regulatory network for cardiac septation.

  14. The Cardiac MR Images and Causes of Paradoxical Septal Motion

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Dong Hun [Soonchunhyang University Bucheon Hospital, Bucheon (Korea, Republic of); Choi, Sang Il; Chun, Eun Ju [Seoul National University Bundang Hospital, Seongnam (Korea, Republic of); Choi, Sung Hun [Ulsan University Hospital, Ulsan (Korea, Republic of); Park, Jae Hyung [Seoul National University Hospital, Seoul (Korea, Republic of)

    2010-10-15

    Real-time cine MRI studies using the steady-state free precession (SSFP) technique are very useful for evaluating cardiac and septal motion. During diastole, the septum acts as a compliant membrane between the two ventricles, and its position and geometry respond to even small alterations in the trans-septal pressure gradients. Abnormal septal motion can be caused by an overload of the right ventricle, delayed ventricular filling and abnormal conduction. In this study, we illustrate, based on our experiences, the causes of abnormal septal motion such as corrective surgery for tetralogy of Fallot, an atrial septal defect, pulmonary thromboembolism, mitral stenosis, constrictive pericarditis and left bundle branch block. In addition, we discuss the significance of paradoxical septal motion in the context of cardiac MR imaging.

  15. The Cardiac MR Images and Causes of Paradoxical Septal Motion

    International Nuclear Information System (INIS)

    Kim, Dong Hun; Choi, Sang Il; Chun, Eun Ju; Choi, Sung Hun; Park, Jae Hyung

    2010-01-01

    Real-time cine MRI studies using the steady-state free precession (SSFP) technique are very useful for evaluating cardiac and septal motion. During diastole, the septum acts as a compliant membrane between the two ventricles, and its position and geometry respond to even small alterations in the trans-septal pressure gradients. Abnormal septal motion can be caused by an overload of the right ventricle, delayed ventricular filling and abnormal conduction. In this study, we illustrate, based on our experiences, the causes of abnormal septal motion such as corrective surgery for tetralogy of Fallot, an atrial septal defect, pulmonary thromboembolism, mitral stenosis, constrictive pericarditis and left bundle branch block. In addition, we discuss the significance of paradoxical septal motion in the context of cardiac MR imaging

  16. Indentation in the Right Ventricle by an Incomplete Pericardium on 3-Dimensional Reconstructed Computed Tomography

    Directory of Open Access Journals (Sweden)

    Hak Ju Kim

    2017-08-01

    Full Text Available We report the case of a 17-year-old girl who presented with an indentation in the right ventricle caused by an incomplete pericardium on preoperative 3-dimensional reconstructed computed tomography. She was to undergo surgery for a partial atrioventricular septal defect and secundum atrial septal defect. Preoperative electrocardiography revealed occasional premature ventricular beats. We found the absence of the left side of the pericardium intraoperatively, and this absence caused strangulation of the diaphragmatic surface of the right ventricle. After correcting the lesion, the patient’s rhythm disturbances improved.

  17. One-stop shop assessment for atrial septal defect closure using 256-slice coronary CT angiography

    Energy Technology Data Exchange (ETDEWEB)

    Yamasaki, Yuzo; Kamitani, Takeshi; Sagiyama, Koji; Yamanouchi, Torahiko; Honda, Hiroshi [Kyushu University, Department of Clinical Radiology, Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka (Japan); Nagao, Michinobu; Kawanami, Satoshi [Kyushu University, Department of Molecular Imaging and Diagnosis, Graduate School of Medical Sciences, Fukuoka (Japan); Sakamoto, Ichiro [Kyushu University, Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Fukuoka (Japan); Yamamura, Kenichiro [Kyushu University, Department of Pediatrics, Graduate School of Medical Sciences, Fukuoka (Japan); Yabuuchi, Hidetake [Kyushu University, Department of Health Sciences, Graduate School of Medical Sciences, Fukuoka (Japan)

    2017-02-15

    To investigate the feasibility and accuracy of measurement of the pulmonary to systemic blood flow ratio (Qp/Qs) and defect and rim sizes in secundum atrial septal defects (ASDs) using 256-slice CT, compared to the reference transoesophageal echocardiography (TEE) and right heart catheterization (RHC) measurements. Twenty-three consecutive adult patients with secundum ASDs who underwent retrospective ECG-gated coronary CT angiography (CCTA), TEE and RHC were enrolled in this study. Right ventricular (RV) and left ventricular (LV) stroke volumes (SV) were calculated by biventricular volumetry of CCTA. Qp/Qs-CT was defined as RVSV/LVSV. The sizes of the defect and rim were measured by multi-planar reconstruction CT images. Correlations between Qp/Qs-CT and Qp/Qs-RHC and between the defect diameter obtained by CT and TEE were analyzed by Pearson's coefficient analysis. Rim sizes by CT and TEE were compared by paired t-test. Qp/Qs-CT was significantly correlated with Qp/Qs-RHC (r = 0.83, p < 0.0001), and the defect diameter by CT was significantly correlated with that by TEE (r = 0.95, p < 0.0001). There was no significant difference between CT and TEE in measurements of rim size. 256-slice CCTA allows measuring Qp/Qs and size of defects and rims in patients with secundum ASDs, accomplishing pretreatment evaluation non-invasively and comprehensively. (orig.)

  18. One-stop shop assessment for atrial septal defect closure using 256-slice coronary CT angiography

    International Nuclear Information System (INIS)

    Yamasaki, Yuzo; Kamitani, Takeshi; Sagiyama, Koji; Yamanouchi, Torahiko; Honda, Hiroshi; Nagao, Michinobu; Kawanami, Satoshi; Sakamoto, Ichiro; Yamamura, Kenichiro; Yabuuchi, Hidetake

    2017-01-01

    To investigate the feasibility and accuracy of measurement of the pulmonary to systemic blood flow ratio (Qp/Qs) and defect and rim sizes in secundum atrial septal defects (ASDs) using 256-slice CT, compared to the reference transoesophageal echocardiography (TEE) and right heart catheterization (RHC) measurements. Twenty-three consecutive adult patients with secundum ASDs who underwent retrospective ECG-gated coronary CT angiography (CCTA), TEE and RHC were enrolled in this study. Right ventricular (RV) and left ventricular (LV) stroke volumes (SV) were calculated by biventricular volumetry of CCTA. Qp/Qs-CT was defined as RVSV/LVSV. The sizes of the defect and rim were measured by multi-planar reconstruction CT images. Correlations between Qp/Qs-CT and Qp/Qs-RHC and between the defect diameter obtained by CT and TEE were analyzed by Pearson's coefficient analysis. Rim sizes by CT and TEE were compared by paired t-test. Qp/Qs-CT was significantly correlated with Qp/Qs-RHC (r = 0.83, p < 0.0001), and the defect diameter by CT was significantly correlated with that by TEE (r = 0.95, p < 0.0001). There was no significant difference between CT and TEE in measurements of rim size. 256-slice CCTA allows measuring Qp/Qs and size of defects and rims in patients with secundum ASDs, accomplishing pretreatment evaluation non-invasively and comprehensively. (orig.)

  19. Percutaneous closure of postoperative ventricular septal residual left-to-right shunt with the China made device

    International Nuclear Information System (INIS)

    Qin Yongwen; Zhao Xianxian; Wu Hong; Ding Jijun; Cao Jiang; Zheng Xin

    2007-01-01

    Objective: To evaluate the feasibility and efficacy of percutaneous transcatheter closure of residual ventricular septal defect (VSD)after surgical closure using China made-nitinol VSD device. Methods: Transcatheter closure was attempted in 11 patients (5 males, 6 females)with a residual VSD following surgical closure. The mean residual VSD narrowest diameter was(5.82 ± 2.09) mm (range from 3 to 9 mm)by echocardiography. A 6 F-9 F delivery sheath was advanced across the residual VSD over a guidewire from femoral vein to deploy the occluder under guidance of left ventriculography and transthoracic echocardiography. Results: The left ventriculography showed membranous part aneurism-like residual VSD in 8 patients and funnel type in 3 cases. There were multiple outlet in 5 cases and one outlets in 6 cases, with mean residual VSD narrowest diameter of (6.09 ± 1.58) mm (range from 3 to 9 mm)measured by left ventriculography. The diameter of occluder was (9.18 ± 2.79) mm (range from 8 to 12 mm). Complete closure of the defect was obtained in 10 cases, and another small residual shunt still remained in one case who had four outlets been treated by 2 occluders. No aortic valvular regurgitation occured in all patients except 1 patient presented complete atrioventricular block within 3 days after the procedure and recovered 2 weeks later with intravenous steroids therapy; and no other complications occured. The fluoroscopy time was(16.91 ± 4.23) min (range from 8 to 30 min). During follow-up from 1 m - 4 y, only 1 case showed residual shunt, and the other had no episodes of endocarditis, thromboembolism, hemolysis, infectious endocarditis, displacement of the occluder and aortic valvular regurgitation. Conclusion: Transcatheter closure of postoperative ventricular septal residual left-to-right shunt with China made-nitinol occluder is safe and effective. (authors)

  20. Tulip deformity with Cera atrial septal defect devices: a report of 3 cases.

    Science.gov (United States)

    Kohli, Vikas

    2015-02-01

    Device closure of secundum atrial septal defect (ASD) is the treatment of choice when anatomy is favourable. Amplatzer device has remained the gold standard for closure of ASD. Cobra deformity is a well-reported problem with devices. Recently, Tulip deformity has been reported in a single case. We report a series of cases where we noted Tulip deformity along with inability to retract the device in the sheath in Cera Lifetech devices. This resulted in prolongation of procedure, excessive fluoroscopic exposure and additional interventional procedures not usually anticipated in ASD device closure. We believe that the problem is due to the stiffness of the device resulting in its inability to be retracted into the sheath. We also report a unique way of retrieving the device.

  1. Visualization of a Small Ventricular Septal Defect at First-pass Contrast-enhanced Cardiac Magnetic Resonance Imaging

    Directory of Open Access Journals (Sweden)

    Francesco Secchi

    2013-01-01

    Full Text Available Ventricular septal defect (VSD is a congenital heart disease that accounts for up to 40% of all congenital cardiac malformations. VSD is a connection between right and left ventricle, through the ventricular septum. Echocardiography and magnetic resonance imaging (MRI help identify this entity. This case presents a 12-year-old male diagnosed with a small muscular apical VSD of 3 mm in diameter, at echocardiography. Cardiac MRI using first-pass perfusion sequence, combining the right plane of acquisition with a short bolus of contrast material, clearly confirmed the presence of VSD.

  2. Surgical repair of tricuspid valve leaflet tear following percutaneous closure of perimembranous ventricular septal defect using Amplatzer duct occluder I: Report of two cases

    Directory of Open Access Journals (Sweden)

    Saatchi Mahesh Kuwelker

    2017-01-01

    Full Text Available Tricuspid valve (TV injury following transcatheter closure of perimembranous ventricular septal defect (PMVSD with Amplatzer ductal occluder I (ADO I, requiring surgical repair, is rare. We report two cases of TV tear involving the anterior and septal leaflets following PMVSD closure using ADO I. In both the patients, the subvalvular apparatus remained unaffected. The patients presented with severe tricuspid regurgitation (TR 6 weeks and 3 months following the device closure. They underwent surgical repair with patch augmentation of the TV leaflets. Postoperatively, both are asymptomatic with a mild residual TR.

  3. Canonical wnt signaling regulates atrioventricular junction programming and electrophysiological properties

    NARCIS (Netherlands)

    Gillers, Benjamin S.; Chiplunkar, Aditi; Aly, Haytham; Valenta, Tomas; Basler, Konrad; Christoffels, Vincent M.; Efimov, Igor R.; Boukens, Bastiaan J.; Rentschler, Stacey

    2015-01-01

    Proper patterning of the atrioventricular canal (AVC) is essential for delay of electrical impulses between atria and ventricles, and defects in AVC maturation can result in congenital heart disease. To determine the role of canonical Wnt signaling in the myocardium during AVC development. We used a

  4. An Infectious Pseudoaneurysm Caused by Ventricular Septal Defect Occluder in Patent Ductus Arteriosus Closure in a Two-Year-Old Child.

    Science.gov (United States)

    Li, Dingyang; Qiu, Qiu; Jin, Jing; Zhang, Changdong; Wang, Lijun; Zhang, Gangcheng

    2017-12-12

    We present a case of an infectious pseudoaneurysm after patent ductus arteriosus (PDA) closure with a ventricular septal defect (VSD) occluder in a two-year-old child. The aneurysm grew rapidly but was successfully removed in time and the patient survived. To our knowledge, this is the first report of an infectious pseudoaneurysm caused by VSD occluder in PDA closure.

  5. Paediatrics Electrocardiography

    African Journals Online (AJOL)

    Prof Ezechukwu

    2011-11-28

    Nov 28, 2011 ... Right axis deviation- Tetralogy of Fallot, pulmonary atresia, transposition of the great arteries (TGA). Superior axis deviation- Atrioventricular septal defect, tricuspid atresia. QRS Complex. The QRS complex represents ventricular depolarization and the normal duration 0.10 seconds or less. It is meas-.

  6. Transcatheter occlusion of secundum atrial septal defect in elderly patients with pulmonary hypertension

    International Nuclear Information System (INIS)

    Hu Jian; Zhang Qi; Ding Fenghua; Yang Zhenkun; Zhang Ruiyan; Zhang Jiansheng; Shen Weifeng

    2008-01-01

    Objective: To evaluate the effect and safety of transcatheter occlusion for secundum atrial septal defect (ASD)in elderly patients with pulmonary hypertension. Methods: Thirty four patients underwent transcatheter occlusion of ASD from January 2002 to December 2006. Fifteen of them aged over 65 and accompanied with pulmonary hypertension (pulmonary hypertension group). All patients received thoracic cardiodynamic ultrasonography and 12 leads ECG before and 1 d, 1 mon, 3 mon, 6 mon, 12 mon after the procedure. Under the guidance of fluoroscopy and transthoracic cardio-ultrasonography, Amplatzer occluders was implanted for the atrial septal defect. Results: The successful rate of placement of the Amplatzer occluder was 100% and no complication was found during the procedure and follow-up period. There were significant differences between pulmonary hypertension group and non-pulmonary hypertension group in age (66.7 ± 5.0y vs 24.1 ± 9.0 y, P<0.001), classification of heart function (NYHA) (2.8 ± 0.7 vs 1.7 ± 0.7, P<0.001), diameter of ASD(30.5 ± 3.2 mm vs 14.2 ± 4.0 mm, P<0.001), size of Amplatzer occluder(35.3 ± 4.5 mm vs 18.2 ± 4.4 mm, P<0.001), systolic pulmonary artery pressure(65.2 ± 11.2 mmHg vs 29.5 ± 3.3 mmHg, P<0.001)and mean pulmonary artery pressure (31.5 ± 4.6 mmHg vs 17.9 ± 1.1 mmHg, P<0.001). After transcatheter closure of ASD, the parameters the systolic pulmonary artery pressure (36.6 ± 11.4 mmHg)and mean pulmonary artery pressure (21.6 ± 4.3 mmHg)decreased significantly in pulmonary hypertension group compared with those before procedure, and the classification of heart function (NYHA)improved (from 2.8 ± 0.7 to 1.8 ± 0.8, P<0.001). Conclusion: Transcatheter occlusion of secundum ASD in elderly patients with pulmonary hypertension is safe and effective. (authors)

  7. Genome-wide association study of multiple congenital heart disease phenotypes identifies a susceptibility locus for atrial septal defect at chromosome 4p16

    Science.gov (United States)

    Cordell, Heather J.; Bentham, Jamie; Topf, Ana; Zelenika, Diana; Heath, Simon; Mamasoula, Chrysovalanto; Cosgrove, Catherine; Blue, Gillian; Granados-Riveron, Javier; Setchfield, Kerry; Thornborough, Chris; Breckpot, Jeroen; Soemedi, Rachel; Martin, Ruairidh; Rahman, Thahira J.; Hall, Darroch; van Engelen, Klaartje; Moorman, Antoon F.M.; Zwinderman, Aelko H; Barnett, Phil; Koopmann, Tamara T.; Adriaens, Michiel E.; Varro, Andras; George, Alfred L.; dos Remedios, Christobal; Bishopric, Nanette H.; Bezzina, Connie R.; O’Sullivan, John; Gewillig, Marc; Bu’Lock, Frances A.; Winlaw, David; Bhattacharya, Shoumo; Devriendt, Koen; Brook, J. David; Mulder, Barbara J.M.; Mital, Seema; Postma, Alex V.; Lathrop, G. Mark; Farrall, Martin; Goodship, Judith A.; Keavney, Bernard D.

    2013-01-01

    We carried out a genome-wide association study (GWAS) of congenital heart disease (CHD). Our discovery cohort comprised 1,995 CHD cases and 5,159 controls, and included patients from each of the three major clinical CHD categories (septal, obstructive and cyanotic defects). When all CHD phenotypes were considered together, no regions achieved genome-wide significant association. However, a region on chromosome 4p16, adjacent to the MSX1 and STX18 genes, was associated (P=9.5×10−7) with the risk of ostium secundum atrial septal defect (ASD) in the discovery cohort (N=340 cases), and this was replicated in a further 417 ASD cases and 2520 controls (replication P=5.0×10−5; OR in replication cohort 1.40 [95% CI 1.19-1.65]; combined P=2.6×10−10). Genotype accounted for ~9% of the population attributable risk of ASD. PMID:23708191

  8. Limited access atrial septal defect closure and the evolution of minimally invasive surgery.

    Science.gov (United States)

    Izzat, M B; Yim, A P; El-Zufari, M H

    1998-04-01

    While minimizing the "invasiveness" in general surgery has been equated with minimizing "access", what constitutes minimally invasive intra-cardiac surgery remains controversial. Many surgeons doubt the benefits of minimizing access when the need for cardiopulmonary bypass cannot be waived. Recognizing that median sternotomy itself does entail significant morbidity, we investigated the value of alternative approaches to median sternotomy using atrial septal defect closure as our investigative model. We believe that some, but not all minimal access approaches are associated with reduced postoperative morbidity and enhanced recovery. Our current strategy is to use a mini-sternotomy approach in adult patients, whereas conventional median sternotomy remains our standard approach in the pediatric population. Considerable clinical experiences coupled with documented clinical benefits are fundamental before a certain approach is adopted in routine practice.

  9. Infective Endocarditis Complicated by Septic Pulmonary Emboli in a Case of a Ventricular Septal Defect

    Directory of Open Access Journals (Sweden)

    Roodpeyma

    2015-11-01

    Full Text Available Introduction Infective endocarditis (IE causes serious complications in patients. Congenital heart disease (CHD is an important underlying condition in children. Septic pulmonary embolism is an uncommon syndrome, and pulmonary valve IE is rare. The current study presented a case of right-sided IE with pulmonary valve involvement and its complications as pulmonary septic emboli in a child with CHD. Case Presentation A 6-year-old girl with a ventricular septal defect (VSD was presented. Echocardiography revealed large vegetation in the right ventricular outflow tract near the pulmonary valve. The patient showed clinical symptoms of lung involvement, and radiologic investigation was compatible with a diagnosis of septic pulmonary emboli. She had good response to antibacterial therapy and underwent a successful surgical closure of the heart defect. Conclusions Children with CHD are at risk of severe complications with the involvement of other organs. long-term febrile illness should be taken seriously in these children. They need hospitalization and careful evaluation.

  10. Preoperative cardiac computed tomography for demonstration of congenital cardiac septal defect in adults

    Energy Technology Data Exchange (ETDEWEB)

    Eom, Hye-Joung; Yang, Dong Hyun; Kang, Joon-Won; Lim, Tae-Hwan [University of Ulsan College of Medicine, Department of Radiology and Research Institute of Radiology, Cardiac Imaging Center, Asan Medical Center, Seoul (Korea, Republic of); Kim, Dae-Hee; Song, Jong-Min; Kang, Duk-Hyun; Song, Jae-Kwan [University of Ulsan College of Medicine, Department of Cardiology and Heart Institute, Cardiac Imaging Center, Asan Medical Center, Seoul (Korea, Republic of); Kim, Joon Bum; Jung, Sung-Ho; Choo, Suk Jung; Chung, Cheol Hyun; Lee, Jae Won [University of Ulsan College of Medicine, Department of Cardiothoracic surgery, Cardiac Imaging Center, Asan Medical Center, Seoul (Korea, Republic of)

    2015-06-01

    We aimed to evaluate the role of preoperative cardiac computed tomography (CT) for adults with congenital cardiac septal defect (CSD). Sixty-five consecutive patients who underwent preoperative CT and surgery for CSD were included. The diagnostic accuracy of CT and the concordance rate of the subtype classification of CSD were evaluated using surgical findings as the reference standard. Sixty-five patients without CSD who underwent cardiac valve surgery were used as a control group. An incremental value of CT over echocardiography was described retrospectively. Sensitivity and specificity of CT for diagnosis of CSD were 95 % and 100 %, respectively. The concordance rate of subtype classification was 91 % in CT and 92 % in echocardiography. The maximum size of the defect measured by CT correlated well with surgical measurement (r = 0.82), and the limit of agreement was -0.9 ± 7.42 mm. In comparison with echocardiography, CT was able to detect combined abnormalities in three cases, and exclusively provided correct subtype classification or clarified suspected abnormal findings found on echocardiography in seven cases. Cardiac CT can accurately demonstrates CSD in preoperative adult patients. CT may have an incremental role in preoperative planning, particularly in those with more complex anatomy. (orig.)

  11. Prenatal Isolated Ventricular Septal Defect May Not Be Associated with Trisomy 21

    Directory of Open Access Journals (Sweden)

    Ori Shen

    2014-04-01

    Full Text Available The aim of this study was to examine if isolated fetal ventricular septal defect (VSD is associated with trisomy 21. One hundred twenty six cases with prenatal VSD diagnosed by a pediatric cardiologist were reviewed. Cases with known risk factors for congenital heart disease, the presence of other major anomalies, soft signs for trisomy 21 or a positive screen test for trisomy 21 were excluded. Ninety two cases formed the study group. None of the cases in the study group had trisomy 21. The upper limit of prevalence for trisomy 21 in isolated VSD is 3%. When prenatal VSD is not associated with other major anomalies, soft markers for trisomy 21 or a positive nuchal translucency or biochemical screen, a decision whether to perform genetic amniocentesis should be individualized. The currently unknown association between isolated VSD and microdeletions and microduplications should be considered when discussing this option.

  12. Influence of CHDs on psycho-social and neurodevelopmental outcomes in children with Down syndrome.

    Science.gov (United States)

    Visootsak, Jeannie; Huddleston, Lillie; Buterbaugh, Allison; Perkins, Adrienne; Sherman, Stephanie; Hunter, Jessica

    2016-02-01

    To evaluate the family psycho-social outcomes of children with Down syndrome and atrioventricular septal defect, and examine the impact of these variables on the child's neurodevelopmental outcome. This was a cross-sectional study that consisted of 57 children with Down syndrome - 20 cases and 37 controls - of ~12-14 months of age. In both groups, we assessed the development of the child, the quality of the child's home environment, and parenting stress. Compared with the Down syndrome without CHD group, the atrioventricular septal defect group revealed lower scores in all developmental domains, less optimal home environments, and higher parental stress. Significant differences in development were seen in the areas of cognition (p=0.04), expressive language (p=0.05), and gross motor (pneurodevelopmental deficits. Finding that parental stress and home environment may play a role in the neurodevelopmental outcomes may prompt new family-directed interventions and anticipatory guidance for the families of children with Down syndrome who have a CHD.

  13. Guillain - Barre syndrome in a patient with acute myocardial infarction with ventricular septal defect repair treated with plasma exchange

    Directory of Open Access Journals (Sweden)

    Maitrey D Gajjar

    2015-01-01

    Full Text Available Guillain - Barre syndrome (GBS is an acute, frequently severe progressive illness of peripheral nervous system that is autoimmune in nature. GBS after myocardial infarction (MI with ventricular septal defect (VSD is uncommon with high mortality rate if not treated promptly. [1] We report a successful outcome of GBS post MI with VSD in a 60-year-old male patient who was on a ventilator treated successfully with therapeutic plasma exchange.

  14. Complex phenotype linked to a mutation in exon 11 of the lamin A/C gene: Hypertrophic cardiomyopathy, atrioventricular block, severe dyslipidemia and diabetes.

    Science.gov (United States)

    Francisco, Ana Rita G; Santos Gonçalves, Inês; Veiga, Fátima; Mendes Pedro, Mónica; Pinto, Fausto J; Brito, Dulce

    2017-09-01

    The lamin A/C (LMNA) gene encodes lamins A and C, which have an important role in nuclear cohesion and chromatin organization. Mutations in this gene usually lead to the so-called laminopathies, the primary cardiac manifestations of which are dilated cardiomyopathy and intracardiac conduction defects. Some mutations, associated with lipodystrophy but not cardiomyopathy, have been linked to metabolic abnormalities such as diabetes and severe dyslipidemia. Herein we describe a new phenotype associated with a mutation in exon 11 of the LMNA gene: hypertrophic cardiomyopathy, atrioventricular block, severe dyslipidemia and diabetes. A 64-year-old woman with hypertrophic cardiomyopathy and a point mutation in exon 11 of the LMNA gene (c.1718C>T, Ser573Leu) presented with severe symptomatic ventricular hypertrophy and left ventricular outflow tract obstruction. She underwent septal alcohol ablation, followed by Morrow myectomy. The patient was also diagnosed with severe dyslipidemia, diabetes and obesity, and fulfilled diagnostic criteria for metabolic syndrome. No other characteristics of LMNA mutation-related phenotypes were identified. The development of type III atrioventricular block with no apparent cause, and mildly depressed systolic function, prompted referral for cardiac resynchronization therapy. In conclusion, the association between LMNA mutations and different phenotypes is complex and not fully understood, and can present with a broad spectrum of severity. Copyright © 2017 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.

  15. Does septal thickness influence outcome of myectomy for hypertrophic obstructive cardiomyopathy?

    Science.gov (United States)

    Nguyen, Anita; Schaff, Hartzell V; Nishimura, Rick A; Dearani, Joseph A; Geske, Jeffrey B; Lahr, Brian D; Ommen, Steve R

    2018-03-01

    Patients with hypertrophic obstructive cardiomyopathy and basal septal thickness 21 mm, n = 505), were performed with the Kruskal-Wallis and the Pearson χ2 tests and semiparametric analysis of covariance. Median group ages were 57, 57 and 54 years (P = 0.007); men comprised 50.4%, 56.7% and 62.0%, respectively (P = 0.003). Intrinsic MV disease was present in 5.9%, 5.2% and 4.6%, respectively (P = 0.80). All patients underwent transaortic septal myectomy. Additional mitral procedures were performed in 7.6%, 7.8% and 8.1%, respectively (P = 0.90). Reasons for MV surgery included intrinsic MV disease (66.7%), residual mitral regurgitation (30.8%) and residual gradient (2.6%). All groups had postoperative gradient relief (median reduction: 51, 54 and 50 mmHg; P = 0.11). Ventricular septal defect occurred in 4 patients (0.3%), and risk did not differ by group (P = 0.24). Adequate relief of left ventricular outflow tract obstruction can be achieved via transaortic septal myectomy without concomitant MV procedures when septal thickness is < 18 mm, and the risk of ventricular septal defect is minimal. Concomitant MV repair/replacement should be reserved for patients with intrinsic MV disease or inadequate relief of mitral regurgitation/left ventricular outflow tract obstruction following adequate extended septal myectomy. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  16. Clinical significance of right ventricular ejection fraction in cases with atrial septal defect

    Energy Technology Data Exchange (ETDEWEB)

    Hamada, Seiki; Nishimura, Tsunehiko; Hayashida, Kouhei; Uehara, Toshiisa

    1989-04-01

    Right ventricular ejection fraction (RVEF) assessed by cardiac radionuclide angiography has been applied to evaluate ventricular function such as ischemic, valvular and congenital heart disease. Using this modality, previous reports also suggest that there is good correlation between RVEF and mean pulmonary arterial pressure (mPA) from catheterization findings in mitral valvular disease and chronic obstructive lung disease. In this study, cardiac RI angiography were performed on 33 adult patients with atrial septal defect (ASD). RVEF is not so good correlation (r=-0.42) with mPA, but in cases within pulmonary to systemic ratio (Qp/Qs) less than 2.0 limits, there is good correlation between RVEF and mPA (n=9, r=-0.71). As a conclusion, in ASD, both afterload assessed by mPA and preload assessed by Qp/Qs decrease RVEF. (author).

  17. Right ventricular pressure response to exercise in adults with isolated ventricular septal defect closed in early childhood.

    Science.gov (United States)

    Moller, Thomas; Lindberg, Harald; Lund, May Brit; Holmstrom, Henrik; Dohlen, Gaute; Thaulow, Erik

    2018-06-01

    We previously demonstrated an abnormally high right ventricular systolic pressure response to exercise in 50% of adolescents operated on for isolated ventricular septal defect. The present study investigated the prevalence of abnormal right ventricular systolic pressure response in 20 adult (age 30-45 years) patients who underwent surgery for early ventricular septal defect closure and its association with impaired ventricular function, pulmonary function, or exercise capacity. The patients underwent cardiopulmonary tests, including exercise stress echocardiography. Five of 19 patients (26%) presented an abnormal right ventricular systolic pressure response to exercise ⩾ 52 mmHg. Right ventricular systolic function was mixed, with normal tricuspid annular plane systolic excursion and fractional area change, but abnormal tricuspid annular systolic motion velocity (median 6.7 cm/second) and isovolumetric acceleration (median 0.8 m/second2). Left ventricular systolic and diastolic function was normal at rest as measured by the peak systolic velocity of the lateral wall and isovolumic acceleration, early diastolic velocity, and ratio of early diastolic flow to tissue velocity, except for ejection fraction (median 53%). The myocardial performance index was abnormal for both the left and right ventricle. Peak oxygen uptake was normal (mean z score -0.4, 95% CI -2.8-0.3). There was no association between an abnormal right ventricular systolic pressure response during exercise and right or left ventricular function, pulmonary function, or exercise capacity. Abnormal right ventricular pressure response is not more frequent in adult patients compared with adolescents. This does not support the theory of progressive pulmonary vascular disease following closure of left-to-right shunts.

  18. Wnt signaling regulates atrioventricular canal formation upstream of BMP and Tbx2

    NARCIS (Netherlands)

    Verhoeven, Manon C.; Haase, Christa; Christoffels, Vincent M.; Weidinger, Gilbert; Bakkers, Jeroen

    2011-01-01

    In the developing heart, the atrioventricular canal (AVC) is essential for separation and alignment of the cardiac chambers, for valve formation, and serves to delay the electrical impulse from the atria to the ventricles. Defects in various aspects of its formation are the most common form of

  19. Case report: an unstable wide QRS complexes tachycardia after ablation of a poster-septal accessory pathway: What is the mechanism?

    Science.gov (United States)

    Wang, Huan; Che, Xiaoru

    2018-03-01

    Differentiation of wide QRS complex tachycardia required repeated electrophysiological stimuli and mapping. However, instability of tachycardia would increase the difficulty in differential diagnosis. In this paper, we reported a wide QRS tachycardia following ablation of an atrioventricular reentrant tachycardia participated by a poster-septal accessory pathway. Limited differentiation strategy was performed because the wide QRS tachycardia was self-limited and with unstable hemodynamics. We analyzed the mechanism of the wide QRS tachycardia by only 4 beats ventricular overpacing. On the basis of the last ventricular pacing, an atypical atrioventricular nodal reentrant tachycardia was confirmed. After slow-pathway modification, the wide QRS tachycardia was eliminated. It was an atypical atrial-ventricular node reentrant tachycardia with right bundle branch block. Reasonable analysis based on electrophysiological electrophysiologic knowledge was the basis of successful diagnosis and treatment.

  20. Traumatic ventricular septal defect in a 4-year-old boy after blunt chest injury

    Directory of Open Access Journals (Sweden)

    Yun Mi Kim

    2011-02-01

    Full Text Available Traumatic ventricular septal defect (VSD resulting from blunt chest injury is a very rare event. The mechanisms of traumatic VSD have been of little concern to dateuntil now, but two dominant theories have been described. In one, the rupture occurs due to acute compression of the heart; in the other, it is due to myocardial infarction of the septum. The clinical symptoms and timing of presentation are variable, so appropriate diagnosis can be difficult or delayed. Closure of traumatic VSD has been based on a combination of heart failure symptoms, hemodynamics, and defect size. Here, we present a case of a 4-year-old boy who presented with a traumatic VSD following a car accident. He showed normal cardiac structure at the time of injury, but after 8 days, his repeated echocardiography revealed a VSD. He was successfully treated by surgical closure of the VSD, and has been doing well up to the present. This report suggests that the clinician should pay great close attention to the patients injured by blunt chest trauma, keeping in mind the possibility of cardiac injury.

  1. Serotonin potentiates transforming growth factor-beta3 induced biomechanical remodeling in avian embryonic atrioventricular valves.

    Directory of Open Access Journals (Sweden)

    Philip R Buskohl

    Full Text Available Embryonic heart valve primordia (cushions maintain unidirectional blood flow during development despite an increasingly demanding mechanical environment. Recent studies demonstrate that atrioventricular (AV cushions stiffen over gestation, but the molecular mechanisms of this process are unknown. Transforming growth factor-beta (TGFβ and serotonin (5-HT signaling modulate tissue biomechanics of postnatal valves, but less is known of their role in the biomechanical remodeling of embryonic valves. In this study, we demonstrate that exogenous TGFβ3 increases AV cushion biomechanical stiffness and residual stress, but paradoxically reduces matrix compaction. We then show that TGFβ3 induces contractile gene expression (RhoA, aSMA and extracellular matrix expression (col1α2 in cushion mesenchyme, while simultaneously stimulating a two-fold increase in proliferation. Local compaction increased due to an elevated contractile phenotype, but global compaction appeared reduced due to proliferation and ECM synthesis. Blockade of TGFβ type I receptors via SB431542 inhibited the TGFβ3 effects. We next showed that exogenous 5-HT does not influence cushion stiffness by itself, but synergistically increases cushion stiffness with TGFβ3 co-treatment. 5-HT increased TGFβ3 gene expression and also potentiated TGFβ3 induced gene expression in a dose-dependent manner. Blockade of the 5HT2b receptor, but not 5-HT2a receptor or serotonin transporter (SERT, resulted in complete cessation of TGFβ3 induced mechanical strengthening. Finally, systemic 5-HT administration in ovo induced cushion remodeling related defects, including thinned/atretic AV valves, ventricular septal defects, and outflow rotation defects. Elevated 5-HT in ovo resulted in elevated remodeling gene expression and increased TGFβ signaling activity, supporting our ex-vivo findings. Collectively, these results highlight TGFβ/5-HT signaling as a potent mechanism for control of biomechanical

  2. Atrioventricular Dissociation following Blunt Chest Trauma

    Directory of Open Access Journals (Sweden)

    Salim Surani

    2014-01-01

    Full Text Available Blunt chest trauma (BCT is a common clinical presentation seen in emergency departments. Few cases of cardiac conduction abnormalities due to BCT have been reported in the medical literature. This dysrhythmias may present as permanent conduction defects requiring permanent pacemaker or may have temporary conduction abnormalities requiring temporary pacemaker or supportive care. We present the case of a young woman who suffered from BCT after being kicked by a horse with the development of a significant substernal hematoma. She developed temporary atrioventricular block, which was completely resolved with the decrease in the size of the substernal hematoma suffered.

  3. Successful two-stage correction of ventricular septal defect and patent ductus arteriosus in a patient with fixed pulmonary hypertension.

    Science.gov (United States)

    Pac, Aysenur; Polat, Tugcin Bora; Vural, Kerem; Pac, Mustafa

    2010-01-01

    We report a case of a 6-year-old boy with fixed severe pulmonary artery hypertension secondary to a ventricular septal defect (VSD) together with a patent ductus arteriosus (PDA). As a preliminary step, PDA embolization was performed following therapy with inhaled prostacyclin over a period of 6 months. Further, the patient underwent successful surgical VSD closure. We postulate that a staged procedure with long-term prostaglandin therapy might be capable of reducing pulmonary artery resistance and permitting total correction in a patient once considered to have inoperable pulmonary arteriopathy.

  4. Complete atrio-ventricular septal defect and Wolf-Parkinson-White syndrome

    Directory of Open Access Journals (Sweden)

    Gudrun Björkhem

    2006-09-01

    Full Text Available A full term female infant was born after 41 weeks of gestation, with a birth weight of 3815g and normal Apgar scores (9-10-10. Immediately after birth tachycardia was noted. An electrocardiogram (ECG showed a heart rate of 300 beats per minute (bpm and narrow QRS complexes. The newborn was clinically stable. She was admitted to the neonatal intensive care unite and an intravenous line was inserted for treatment with Adenosine. While preparing for Adenosine infusion her heart rate spontaneously decreased. Her ECG at presentation with normal heart rate is shown in figure1a. On auscultation, a soft systolic murmur was heard.

  5. Septal Leaflet versus Chordal Detachment in Closure of Hard-To-Expose Ventricular Septal Defects.

    Science.gov (United States)

    Pourmoghadam, Kamal K; Boron, Agnieszka; Ruzmetov, Mark; Narasimhulu, Sukumar Suguna; Kube, Alicia; O'Brien, Michael C; DeCampli, William M

    2018-04-04

    Different techniques have been used for exposure of ventricular septal defect (VSD) margins when there is crowding of the VSD anatomy by tricuspid valve (TV) subvalvar apparatus. The aim of this study was to compare surgical outcomes, for the two techniques of TV leaflet detachment and the rarely described TV chordal detachment for hard-to-expose VSDs. Patients undergoing transatrial VSD repair were identified from our institutional database. Follow-up echocardiography and patient data were obtained from medical records. Between 1/2005-8/2016, 130 isolated conoventricular VSDs were repaired. Among these, 26patients had leaflet detachment, while 15 underwent chordal detachment, and 89 had regular VSD repair (reference group). There was no significant difference between the groups in age, weight, postoperative length-of-stay, genetic/syndromic abnormalities, time-to-extubation, and left and right ventricular systolic function. The cardiopulmonary bypass and cross-clamp time were significantly higher in leaflet detachment group, when compared with reference group (118+28vs102+32, p=0.02; and 73+20vs61+23, p=0.01, respectively). Echocardiographic follow-up were available for 87patients at a mean of 2.6years (1month-11years). Tricuspid regurgitation was rated as none or trivial in 66(76%), mild in 20(23%) and moderate in one reference group patient. There was no difference in presence of residual VSD, or degree of tricuspid regurgitation amongst the three groups. There was no reoperation for tricuspid regurgitation. Tricuspid valve leaflet and chordal detachment techniques provide equally viable and safe alternative to closure of hard-to-expose VSDs while maintaining appropriate TV function. Their use in our series did not lead to increased TV dysfunction at early-to-midterm echocardiographic assessment. Copyright © 2018. Published by Elsevier Inc.

  6. Current status of cardiovascular surgery in Japan 2013 and 2014: A report based on the Japan Cardiovascular Surgery Database. 2: Congenital heart surgery.

    Science.gov (United States)

    Hirata, Yasutaka; Hirahara, Norimichi; Murakami, Arata; Motomura, Noboru; Miyata, Hiroaki; Takamoto, Shinichi

    2018-01-01

    We analyzed the mortality and morbidity of congenital heart surgery in Japan using the Japan Cardiovascular Surgery Database (JCVSD). Data regarding congenital heart surgery performed between January 2013 and December 2014 were obtained from JCVSD. The 20 most frequent procedures were selected and the mortality rates and major morbidities were analyzed. The mortality rates of atrial septal defect repair and ventricular septal defect repair were less than 1%, and the mortality rates of tetralogy of Fallot repair, complete atrioventricular septal defect repair, bidirectional Glenn, and total cavopulmonary connection were less than 2%. The mortality rates of the Norwood procedure and total anomalous pulmonary venous connection repair were more than 10%. The rates of unplanned reoperation, pacemaker implantation, chylothorax, deep sternal infection, phrenic nerve injury, and neurological deficit were shown for each procedure. Using JCVSD, the national data for congenital heart surgery, including postoperative complications, were analyzed. Further improvements of the database and feedback for clinical practice are required.

  7. Atrioventricular block, ECG tracing (image)

    Science.gov (United States)

    ... an abnormal rhythm (arrhythmia) called an atrioventricular (AV) block. P waves show that the top of the ... wave (and heart contraction), there is an atrioventricular block, and a very slow pulse (bradycardia).

  8. Sensitivity and specificity of radionuclide equilibrium angiocardiography for detection of hemodynamically significant secundum atrial septal defect

    Energy Technology Data Exchange (ETDEWEB)

    Brunotte, F.; Laurens, M.H.; Itty, C.; Robert, J.; Cloez, J.L.; Marcon, F.; Pernot, C.

    1986-12-01

    To determine the value, of gated equilibrium angiography in secundum atrial septal defect (ASD) in children, the first pass pulmonic/systemic flow ratio (Qp/Qs) was compared with diastolic count ratio (DCR) and stroke count ratio (SCR) of the two ventricles. In 50 children we have found a correlation between Qp/Qs and DCR (r=0.71) and between Qp/Qs and SCR (r=0.66). For detection of significant atrial shunt (Qp/Qs>1.5) the sensitivity of DCR>2 was 0.81 and the specificity 0.75. For SCR>1.5 we sensitivity and specificity values of 0.87 and 0.71 respectively. Left and right ventricular ejection fractions were normal (0.67+-0.08 and 0.50+-0.07).

  9. Current perspectives in percutaneous atrial septal defect closure devices

    Directory of Open Access Journals (Sweden)

    Bissessor N

    2015-07-01

    Full Text Available N Bissessor1–4 1Department of Cardiology, The Epworth Hospital, Melbourne, VIC, Australia; 2Division of Interventional Cardiology, The Alfred Hospital, Melbourne, VIC, Australia; 3Department of Clinical Science, Charles Sturt University Albury Campus, NSW, Australia; 4Heart Foundation, Griffith University, QLD, Australia Abstract: In the last decade, percutaneous atrial septal defect (ASD closure has become the treatment of choice in most clinical presentations of ASD. Percutaneous ASD closure has established procedural safety through operator experience and improved device structure and deliverability. There have also been advances in diagnostic capabilities. Devices have evolved from large bulky meshes to repositionable, minimal residual mesh content that easily endothelializes and conforms well to surrounding structures. Biodegradable technology has been introduced and will be closely watched as a future option. The evolution of ASD closure device usage in the last four decades incorporates development that minimizes a wide range of serious side effects that have been reported over the years. Complications reported in the literature include thrombus formation, air embolization, device embolization, erosions, residual shunts, and nickel hypersensitivity. Modern devices have intermediate to long term data with outcomes that have been favorable. Devices are available in multiple sizes with improved delivery mechanisms to recapture, reposition, and safely close simple and complex ASDs amenable to percutaneous closure. In this review, commonly used devices and deployment procedures are discussed together with a look at devices that show promise for the future. Keywords: ASD, congenital, Amplatzer, Gore Helex, Biostar, Figulla

  10. Reversible chronic acquired complete atrioventricular block.

    Science.gov (United States)

    Rakovec, P; Milcinski, G; Voga, G; Korsic, L

    1982-01-01

    The return of atrioventricular conduction is reported in a case after nearly four years of complete acquired heart block. After recovery from atrioventricular block, right bundle branch block persisted, but P-R interval and H-V interval were normal. Three months later a relapse of second degree infranodal atrioventricular block was noted. A short review of similar cases from the literature is given.

  11. Genetic polymorphisms of the TYMS gene are not associated with congenital cardiac septal defects in a Han Chinese population.

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    Jian-Yuan Zhao

    Full Text Available BACKGROUND: Clinical research indicates that periconceptional administration of folic acid can reduce the occurrence of congenital cardiac septal defects (CCSDs. The vital roles of folate exhibits in three ways: the unique methyl donor for DNA expression regulation, the de novo biosynthesis of purine and pyrimidine for DNA construction, and the serum homocysteine removal. Thymidylate synthase (TYMS is the solo catalysis enzyme for the de novo synthesis of dTMP, which is the essential precursor of DNA biosynthesis and repair process. To examine the role of TYMS in Congenital Cardiac Septal Defects (CCSDs risk, we investigated whether genetic polymorphisms in the TYMS gene associated with the CCSDs in a Han Chinese population. METHOD: Polymorphisms in the noncoding region of TYMS were identified via direct sequencing in 32 unrelated individuals composed of half CCSDs and half control subjects. Nine SNPs and two insertion/deletion polymorphisms were genotyped from two independent case-control studies involving a total of 529 CCSDs patients and 876 healthy control participants. The associations were examined by both single polymorphism and haplotype tests using logistic regression. RESULT: We found that TYMS polymorphisms were not related to the altered CCSDs risk, and even to the changed risk of VSDs subgroup, when tested in both studied groups separately or in combination. In the haplotype analysis, there were no haplotypes significantly associated with risks for CCSDs either. CONCLUSION: Our results show no association between common genetic polymorphisms of the regulatory region of the TYMS gene and CCSDs in the Han Chinese population.

  12. The Effect on Somatic Growth of Surgical and Catheter Treatment of Secundum Atrial Septal Defects.

    Science.gov (United States)

    Chlebowski, Meghan M; Dai, Hongying; Kaine, Stephen F

    2017-10-01

    Historical studies suggest an association between atrial septal defect (ASD) and impaired growth with inconsistent improvement following closure. Limited data exist regarding the impact on growth in the era of transcatheter therapy. To evaluate the effect of closure on growth, we conducted a retrospective review of patients undergoing surgical or transcatheter closure during two time periods. Four hundred patients with isolated secundum ASD were divided into three cohorts: early surgical, contemporary surgical, and transcatheter. Data collected included demographics; height, weight, and body mass index (BMI) percentiles; catheterization hemodynamics; and co-morbidities. For all cohorts, there was no significant change in height or weight percentiles during two years after ASD closure. Age at repair was later for contemporary surgical and transcatheter cohorts (p indication for early ASD repair. However, early repair may be indicated in children with existing significant growth failure.

  13. Perforation of the right aortic valve cusp: complication of ventricular septal defect closure with a modified Rashkind umbrella.

    Science.gov (United States)

    Vogel, M; Rigby, M L; Shore, D

    1996-01-01

    An 18-month-old boy with a perimembranous ventricular septal defect (VSD) had undergone transcatheter closure of the defect with a modified 17 mm Rashkind umbrella device at age 4 months (weight 3.8 kg). The clinical signs of a VSD persisted, and he developed aortic incompetence, first detected 5 months after the procedure, which progressed from mild to moderate. A three-dimensional echocardiographic study demonstrated that one of the four arms holding the umbrella was protruding into the aortic valve and had perforated the right aortic valve cusp. This diagnosis was confirmed at subsequent surgery. Surgical repair of the perforated right aortic valve leaflet was necessary. The umbrella was adherent to the tricuspid valve and could not be removed. Instead it was left in situ, but three of the stainless steel arms were cut off. When umbrella closure of a perimembranous VSD is undertaken, the close proximity of part of the distal umbrella to the aortic valve can lead to aortic regurgitation.

  14. Inverse Relationship Between Membranous Septal Length and the Risk of Atrioventricular Block in Patients Undergoing Transcatheter Aortic Valve Implantation.

    Science.gov (United States)

    Hamdan, Ashraf; Guetta, Victor; Klempfner, Robert; Konen, Eli; Raanani, Ehud; Glikson, Michael; Goitein, Orly; Segev, Amit; Barbash, Israel; Fefer, Paul; Spiegelstein, Dan; Goldenberg, Ilan; Schwammenthal, Ehud

    2015-08-17

    This study sought to examine whether imaging of the atrioventricular (AV) membranous septum (MS) by computed tomography (CT) can be used to identify patient-specific anatomic risk of high-degree AV block and permanent pacemaker (PPM) implantation before transcatheter aortic valve implantation (TAVI) with self-expandable valves. MS length represents an anatomic surrogate of the distance between the aortic annulus and the bundle of His and may therefore be inversely related to the risk of conduction system abnormalities after TAVI. Seventy-three consecutive patients with severe aortic stenosis underwent contrast-enhanced CT before TAVI. The aortic annulus, aortic valve, and AV junction were assessed, and MS length was measured in the coronal view. In 13 patients (18%), high-degree AV block developed, and 21 patients (29%) received a PPM. Multivariable logistic regression analysis revealed MS length as the most powerful pre-procedural independent predictor of high-degree AV block (odds ratio [OR]: 1.35, 95% confidence interval [CI]: 1.1 to 1.7, p = 0.01) and PPM implantation (OR: 1.43, 95% CI: 1.1 to 1.8, p = 0.002). When taking into account pre- and post-procedural parameters, the difference between MS length and implantation depth emerged as the most powerful independent predictor of high-degree AV block (OR: 1.4, 95% CI: 1.2 to 1.7, p < 0.001), whereas the difference between MS length and implantation depth and calcification in the basal septum were the most powerful independent predictors of PPM implantation (OR: 1.39, 95% CI: 1.2 to 1.7, p < 0.001 and OR: 4.9, 95% CI: 1.2 to 20.5, p = 0.03; respectively). Short MS, insufficient difference between MS length and implantation depth, and the presence of calcification in the basal septum, factors that may all facilitate mechanical compression of the conduction tissue by the implanted valve, predict conduction abnormalities after TAVI with self-expandable valves. CT assessment of membranous septal anatomy provides

  15. Rest and exercise ventricular function in adults with congenital ventricular septal defects

    International Nuclear Information System (INIS)

    Jablonsky, G.; Hilton, J.D.; Liu, P.P.; Morch, J.E.; Druck, M.N.; Bar-Shlomo, B.Z.; McLaughlin, P.R.

    1983-01-01

    Rest and exercise right and left ventricular function were compared using equilibrium gated radionuclide angiography in 19 normal sedentary control subjects and 34 patients with hemodynamically documented congenital ventricular septal defect (VSD). Gated radionuclide angiography was performed at rest and during each level of graded supine bicycle exercise to fatigue. Heart rate, blood pressure, maximal work load achieved, and right and left ventricular ejection fractions were assessed. The control subjects demonstrated an increase in both the left and right ventricular ejection fractions with exercise. All study groups failed to demonstrate an increase in ejection fraction in either ventricle with exercise. Furthermore, resting left ventricular ejection fraction in Groups 2 and 3 was lower than that in the control subjects and resting right ventricular ejection fraction was lower in Group 3 versus control subjects. Thus left and right ventricular function on exercise were abnormal in patients with residual VSD as compared with control subjects; rest and exercise left ventricular ejection fractions remained abnormal despite surgical closure of VSD in the remote past; resting left and right ventricular function was abnormal in patients with Eisenmenger's complex; lifelong volume overload may be detrimental to myocardial function

  16. Atrioventricular Pacemaker Lead Reversal

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    Mehmet K Aktas, MD

    2007-01-01

    Full Text Available During cardiac surgery temporary epicardial atrial and ventricular leads are placed in case cardiac pacing is required postoperatively. We present the first reported series of patients with reversal of atrioventricular electrodes in the temporary pacemaker without any consequent deleterious hemodynamic effect. We review the electrocardiographic findings and discuss the findings that lead to the discovery of atrioventricular lead reversal.

  17. Prevention of congenital defects induced by prenatal alcohol exposure (Conference Presentation)

    Science.gov (United States)

    Sheehan, Megan M.; Karunamuni, Ganga; Pedersen, Cameron J.; Gu, Shi; Doughman, Yong Qiu; Jenkins, Michael W.; Watanabe, Michiko; Rollins, Andrew M.

    2017-02-01

    Over 500,000 women per year in the United States drink during pregnancy, and 1 in 5 of this population also binge drink. Up to 40% of live-born children with prenatal alcohol exposure (PAE) present with congenital heart defects (CHDs) including life-threatening outflow and valvuloseptal anomalies. Previously we established a PAE model in the avian embryo and used optical coherence tomography (OCT) imaging to assay looping-stage (early) cardiac function/structure and septation-stage (late) cardiac defects. Early-stage ethanol-exposed embryos had smaller cardiac cushions (valve precursors) and increased retrograde flow, while late-stage embryos presented with gross head/body defects, and exhibited smaller atrio-ventricular (AV) valves, interventricular septae, and aortic vessels. However, supplementation with the methyl donor betaine reduced gross defects, prevented cardiac defects such as ventricular septal defects and abnormal AV valves, and normalized cardiac parameters. Immunofluorescent staining for 5-methylcytosine in transverse embryo sections also revealed that DNA methylation levels were reduced by ethanol but normalized by co-administration of betaine. Furthermore, supplementation with folate, another methyl donor, in the PAE model appeared to normalize retrograde flow levels which are typically elevated by ethanol exposure. Studies are underway to correlate retrograde flow numbers for folate with associated cushion volumes. Finally, preliminary findings have revealed that glutathione, a key endogenous antioxidant which also regulates methyl group donation, is particularly effective in improving alcohol-impacted survival and gross defect rates. Current investigations will determine whether glutathione has any positive effect on PAE-related CHDs. Our studies could have significant implications for public health, especially related to prenatal nutrition recommendations.

  18. Transcatheter coil occluder for closure of ventricular septal defect (a report of 4 cases)

    International Nuclear Information System (INIS)

    Gao Wei; Zhou Aiqing; Yu Zhiqing; Li Fen; Zhong Yumin; Zhang Yuqi; Huang Meirong; Sun Kun

    2005-01-01

    Objective: To explore the indication, methodology and complication of transcatheter coil closure of ventricular septal defect (VSD) in children. Methods: Transcatheter closure of perimembranous VSD with coils was performed in 4 cases from 2003 to 2005. The Duct-Occlude (pfm) and detachable coil (Cook) were chosen for embolization depending on the results of the left ventricular angiogram. The coil size was generally about 1-4 mm larger than the diameter of VSD. Follow up was carried out with echocardiography, ultrasound and clinical examination. Results: The defect diameters of the four cases were 2.0 mm, 2.7 mm, 2.5 mm and 1.5 mm respectively. The Duct-Occlude were successfully implanted in 3 cases of perimembranous VSD with the same type coil (OD[mm]7-3-6, windings 5-3-4) for each. One detachable coil (Cook) (5 x 5) was implanted in the remaining case. All cases had trivial residual shunt immediately after implantation which disappeared 24 hours later. Follow-up for 2 months to one year showed no coil displacement and secondary bacterial arteritis. No tricuspid and aortic regurgitation, no emboli, no endocarditis, and no arrhythmia were found. Conclusions: Coil closure of some small VSD with membranate part pseudo-ventricular aneurysm has good efficacy with the advantages of simple operation, less metal content and mini-invasion also applicable for infants. (authors)

  19. Ventricular septal defect in children and adolescents in Angola: experience of a tertiary center.

    Science.gov (United States)

    Manuel, Valdano; Morais, Humberto; Manuel, Ana; David, Bruna; Gamboa, Sebastiana

    2014-10-01

    This is the first study in Angola with the aim of characterizing ventricular septal defect (VSD) among children and adolescents. A cross-sectional study based on echocardiographic records of the largest pediatric cardiology center in Angola included all children and adolescents (0 to 18 years old) with VSD between April 2010 and March 2011. The diagnosis was made by transthoracic and Doppler echocardiography with a Medison SA 8000 system. The sample was divided into two groups: Group 1, isolated VSD; and Group 2, VSD associated with other congenital heart defects (CHDs). Age, gender, type of VSD, associated CHDs and genetic syndromes were assessed. A total of 490 CHDs were diagnosed, of which 283 were VSDs. In Group 1 (140, 49%) the mean age was 29±36 months. The most frequent age (mode) at diagnosis was 24 months. There was no predominance of gender (ratio 1:1). The majority (127, 91%) had perimembranous VSD. In Group 2 (143, 51%) 113 patients (79%) had one, 27 patients (19%) had two and three patients (2%) had three other CHDs. Trisomy 21 was the most common genetic syndrome (23, 96%). The study shows that VSD is the most common CHD in childhood, the diagnosis is made late and almost half of VSDs are associated with other CHDs. Copyright © 2014 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  20. Familial pulmonary arterial hypertension, leucopenia, and atrial septal defect: a probable new familial syndrome with multisystem involvement.

    Science.gov (United States)

    Dursun, Ali; Ozgul, R Koksal; Soydas, Asli; Tugrul, Tugba; Gurgey, Aytemiz; Celiker, Alpay; Barst, Robyn J; Knowles, James A; Mahesh, Mansukhani; Morse, Jane H

    2009-01-01

    We present two siblings with identical clinical findings that seem to represent a previously unreported familial syndrome. Major findings involve three systems: pulmonary arterial hypertension, cardiac abnormalities including secundum-type atrial septal defect, and the hematopoietic system with intermittent neutropenia, lymphopenia, monocytosis, and anemia. The siblings also shared several minor abnormalities: pectus carinatum, long fingers, proximally placed thumb, broad nasal bridge, and high-arched palate. The male proband also had bilateral inguinal hernias and undescended testes. The same findings in two siblings suggest a genetic cause--either an autosomal recessive disorder or germline mosaicism in one parent for a dominant mutation. Investigations revealed a bone morphogenetic protein receptor 2 polymorphism in intron 4 in only one sibling, which was also present in unaffected maternal relatives.

  1. Decline in arterial partial pressure of oxygen after exercise: a surrogate marker of pulmonary vascular obstructive disease in patients with atrial septal defect and severe pulmonary hypertension.

    Science.gov (United States)

    Laksmivenkateshiah, Srinivas; Singhi, Anil K; Vaidyanathan, Balu; Francis, Edwin; Karimassery, Sundaram R; Kumar, Raman K

    2011-06-01

    To examine the utility of decline in arterial partial pressure of oxygen after exercise as a marker of pulmonary vascular obstructive disease in patients with atrial septal defect and pulmonary hypertension. Treadmill exercise was performed in 18 patients with atrial septal defect and pulmonary hypertension. Arterial blood gas samples were obtained before and after peak exercise. A decline in the arterial pressure of oxygen of more than 10 millimetres of mercury after exercise was considered significant based on preliminary tests conducted on the controls. Cardiac catheterisation was performed in all patients and haemodynamic data sets were obtained on room air, oxygen, and a mixture of oxygen and nitric oxide (30-40 parts per million). There were 10 patients who had more than a 10 millimetres of mercury drop in arterial partial pressure of oxygen after exercise and who had a basal pulmonary vascular resistance index of more than 7 Wood units per square metre. Out of eight patients who had less than a 10 millimetres of mercury drop in arterial partial pressure of oxygen after exercise, seven had a basal pulmonary vascular resistance index of less than 7 Wood units per square metre, p equals 0.0001. A decline in arterial partial pressure of oxygen of more than 10 millimetres of mercury predicted a basal pulmonary vascular resistance index of more than 7 Wood units per square metre with a specificity of 100% and a sensitivity of 90%. A decline in arterial partial pressure of oxygen following exercise appears to predict a high pulmonary vascular resistance index in patients with atrial septal defect and pulmonary hypertension. This test is a useful non-invasive marker of pulmonary vascular obstructive disease in this subset.

  2. MULTIPLE VENTRICULAR SEPTAL DEFECTS: A NEW STRATEGY

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    Antonio Francesco Corno

    2013-07-01

    Full Text Available INTRODUCTIONA multicenter prospective study was conducted to evaluate a new strategy for multiple Ventricular Septal Defects (VSDs.MATERIALS AND METHODSFrom 2004 to 2012 17 consecutive children (3 premature, 14 infants, mean age 3.2months (9 days to 9 months, mean body weight 4.2kg (3.1 to 6.1 kg, with multiple VSDs underwent Pulmonary Artery Banding (PAB with an adjustable FloWatch-PAB. Associated cardiac anomalies included patent ductus arteriosus (9, aortic coarctation (2, hypoplastic aortic arch (2 and left isomerism (1. Five patients (5/17 =29.4% required pre-operative mechanical ventilation, with a mean duration of 64 days (7 to 240 daysRESULTSThere were no early or late deaths during a mean follow-up of 48 months (7 to 98 months, with either FloWatch removal or last observation as end-points.FloWatch-PAB adjustments were required in all patients: a mean of 4.8 times/patient (2 to 9 to tighten the PAB, and a mean of 1.1 times/patient (0 to 3 to release the PAB with the patient’s growth. After a mean interval of 29 months (8 to 69 months 10/17 (59% patients underwent reoperation: 7/10 PAB removal, with closure of a remaining peri-membranous VSD in 6 and Damus-Kaye-Stansel, bi-directional Glenn, and atrial septectomy in 1; 3/9 patients required only PAB removal. All muscular multiple VSDs had closed in all 10 patients. PA reconstruction was required in 1/10 patient. In 5/7 of the remaining patients with the PAB still in situ, all muscular VSDs had already closed. The only 2 patients with persistent muscular multiple VSDs are the 2 patients with the shortest follow-up.CONCLUSIONS This reproducible new strategy with an adjustable PAB simplifies the management of infants with multiple VSDs and provides the following advantages: a good results (0% mortality, delayed surgery with a high incidence (15/17=88% of spontaneous closure of multiple muscular VSDs, and facilitated closure of residual peri-membranous VSD at an older age and h

  3. Radiological Diagnosis of Recirculatory Congenital Heart Disease with Increased Pulmonary Blood Flow

    International Nuclear Information System (INIS)

    Bartusevichiene, A.; Rulevichius, A.; Dobrovolskis, K.R.

    1995-01-01

    The number of patients with congenital diseases is increasing therefore early diagnosis of these diseases is of crucial importance. Radiological diagnostics of recirculatory congenital heart disease with increased pulmonary blood flow, i.e. atrial septal defect (ASD), ventricle septal defect (VSD), ductus arteriosus (Botalli) persistence (DAP) and atrioventricular communication (AVC) have been analysed. Recirculatory congenital heart disease with increased pulmonary blood flow (ASD, VSD, DAP)radiologically causes similar lung, lung roots and pulmonary arterial changes. After the radiomorphological and radiofunctional examination of chest organs the following symptoms of the disease were defined: all the patients had hypervolemy, enlarged structural lungs roots, enlarged pulmonary arterial arch. These radiofunctional symptoms help to differentiate congenital heart diseases case by case. (author). 7 refs., 6 figs., 1 tab

  4. Improving left ventricular outflow tract obstruction repair in common atrioventricular canal defects.

    Science.gov (United States)

    Myers, Patrick O; del Nido, Pedro J; Marx, Gerald R; Emani, Sitaram; Mayer, John E; Pigula, Frank A; Baird, Christopher W

    2012-08-01

    Left ventricular outflow tract obstruction (LVOTO) is the second most frequent reason for reoperation after atrioventricular canal (AVC) defect repair. Limited data are available on the mechanisms of LVOTO, their treatment, and outcomes. Between 1998 and 2010, 56 consecutive children with AVC underwent 68 LVOTO procedures. The AVC was partial in 4, transitional in 9, and complete in 43. The LVOTO procedure was required in 21 patients at the primary AVC repair, and the initial LVOTO procedure in 35 patients was a late reoperation after AVC repair. During a mean follow-up of 50±41 months, 5 patients (24%) with LVOTO repair at AVC repair required a reoperation for LVOTO, and 7 patients (20%) whose initial LVOTO repair was a reoperation required a second reoperation for LVOTO repair. Overall freedom from LVOTO reoperation was 98.5% at 1 year, 92.5% at 3 years, 81% at 5 years, 72.2% at 7 years, and 52.5% at 10 and 12 years. The freedom from reoperation was neither significantly different between partial, transitional, and complete AVC (p=0.78) nor between timing of the LVOT procedure (p=0.49). Modified single-patch AVC repair was associated with a higher LVOTO reoperation rate (p=0.04). Neither the mechanisms leading to LVOTO nor the surgical techniques used were independent predictors of reoperation. LVOTO in AVC is a complex and multifactorial disease. Aggressive surgical repair has improved late outcomes; however, risk factors for reoperation and the ideal approach for repair remain to be defined. Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  5. Experiences with surgical treatment of ventricle septal defect as a post infarction complication

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    Stich Kathrin

    2009-01-01

    Full Text Available Abstract Background Complications of acute myocardial infarction (AMI with mechanical defects are associated with poor prognosis. Surgical intervention is indicated for a majority of these patients. The goal of surgical intervention is to improve the systolic cardiac function and to achieve a hemodynamic stability. In this present study we reviewed the outcome of patients with post infarction ventricular septal defect (PVSD who underwent cardiac surgery. Methods We analysed retrospectively the hospital records of 41 patients, whose ages range from 48 to 81, and underwent a surgical treatment between 1990 and 2005 because of PVSD. Results In 22 patients concomitant coronary artery bypass grafting (CAGB was performed. In 15 patients a residual shunt was found, this required re-op in seven of them. The time interval from infarct to rupture was 8.7 days and from rupture to surgery was 23.1 days. Hospital mortality in PVSD group was 32%. The mortality of urgent repair within 3 days of intractable cardiogenic shock was 100%. The mortality of patients with an anterior VSD and a posterior VSD was 29.6% vs 42.8%, respectively. All patients who underwent the surgical repair later than day 36 survived. Conclusion Surgical intervention is indicated for a majority of patients with mechanical complications. Cardiogenic shock remains the most important factor that affects the early results. The surgical repair of PVSD should be performed 4–5 weeks after AMI. To improve surgical outcome and hemodynamics the choice of surgical technique and surgical timing as well as preoperative management should be tailored for each patient individually.

  6. Abordagem transxifóidea sem esternotomia para correção da comunicação interatrial Transxiphoid approach without sternotomy for the correction of atrial septal defect

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    Miguel Barbero-Marcial

    1996-12-01

    Full Text Available OBJETIVO: Avaliação de uma nova abordagem cirúrgica para fechamento da comunicação interatrial. MÉTODOS: A abordagem da comunicação interatrial através da janela xifóidea foi realizada em 6 pacientes do sexo masculino e 1 do sexo feminino e a idade variou de 6 meses a 14 anos, com média de idades de 5,1 anos. Em todos, foi utilizada acirculação extracorpórea, através de canulação da artéria femoral e átrio direito e videotoracoscopia para auxiliar a visibilização da aorta e do canal arterial, assim como das estruturas correspondentes às cavidades esquerdas. RESULTADOS: Não houve complicações intra-operatórias ou pós-operatórias e, em todos os pacientes, foi possível a extubação na sala de operações. CONCLUSÃO: A janela xifóidea, sem a abertura do esterno, permitiu a correção da comunicação interatrial com bons resultados, podendo constituir-se em uma nova abordagem, minimamente invasiva.PURPOSE: Evaluate a new access for surgical correction of atrial septal defects. METHODS: The access by xiphoid window was done in 7 cases with atria! septal defect type ostium secundum. The age ranged from 6 months to 14 years, mean-age 5.1 years old. In all cases the extracorporeal circulation was done with femoral artery and right atrial cannulation and aortic clamping. The video-assisted endoscopy was utilized to help the visualization of the aorta, ductus arteriosus and the anatomy of the left intracardiac structures. RESULTS: There was no intraoperative or postoperative complications and in our 7 cases the extubation at the operating room was possible. CONCLUSION: The xiphoid window without opening the sternum permited correction of the atrial septal defect with good results and without complications, and could be used as a new technique, less invasive, to correct these defects.

  7. Atrial Septal Aneurysm Presenting as Clubbing without Clinically Apparent Cyanosis.

    Science.gov (United States)

    Goyal, Laxmi Kant; Banerjee, S; Yadav, R N; Singh, Gajraj; Ganguli, Sujata; Isran, Rohit

    2015-09-01

    Atrial septal aneurysm (ASA) is a localised "saccular" deformity which protrudes to the right or the left atrium or on both sides. It is a rare, but well recognised cardiac abnormality. It is usually an incidental finding or may presents as atrial arrhythmias or arterial embolism. Though it is an acyanotic congenital heart disease but it may result in significant right to left shunt and cyanosis. We describe a patient of ASA with atrial septal defect who presented with clubbing and right to left shunt without clinically apparent cyanosis. © Journal of the Association of Physicians of India 2011.

  8. Transcatheter closure of atrial septal defect in a patient with Noonan syndrome after corrective surgery

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    Mangovski Ljupčo

    2015-01-01

    Full Text Available Introduction. Transcatheter atrial septal defect (ASD closure is considered to be a gold standard for patients with the suitable anatomy as compared to cardiac surgery. Reocurrence of ASD after surgical closure is a very rare late complication which can be successfully managed with transcatheter procedure. Case report. We reported a female patient with Noonan syndrome who presented with hemodinamically significant ASD 37 years after the corrective cardiac surgery. Due to numerous comorbidities which included severe kyphoscoliosis, pectus excavatum and multiple surgeries we decided to perform transcatheter closure of ASD. The procedure itself was very challenging due to the patient’s short stature and heart’s orientation in the chest, but was performed successfully. The subsequent follow-up was uneventful and the patient reported improvement in the symptoms. Conclusion. Transcatheter closure of ASD in a patient with Noonan syndrome with the history of surgically corrected ASD can be performed successfully, despite challenging chest anatomy.

  9. Transmembrane protein 2 (Tmem2) is required to regionally restrict atrioventricular canal boundary and endocardial cushion development

    NARCIS (Netherlands)

    Smith, K.; Lagendijk, A.K.; Courtney, A.D.; Chen, H.; Paterson, S.; Hogan, B.M.; Wicking, C.; Bakkers, J.

    2011-01-01

    The atrioventricular canal (AVC) physically separates the atrial and ventricular chambers of the heart and plays a crucial role in the development of the valves and septa. Defects in AVC development result in aberrant heart morphogenesis and are a significant cause of congenital heart malformations.

  10. The effect of residual ventricular septal defects on early clinical outcome: initial experience

    International Nuclear Information System (INIS)

    Zaman, H.; Cheema, M.A.; Jalal, A.

    2000-01-01

    Residual ventricular septal defect (VSD)after repair of isolated VSD or Fallot's tetralogy is one of the main causes of morbidity and re-operations. In this paper we have presented the results of out initial experience regarding the management of this problem. The data consists of 71 patients (22 isolated VSD and 29 fallout's tetralogy) operate during January 1991 to July, 1993. The incidence of residual VSD as shown by color doppler study at the time of discharge from hospital and at three month's follow-up was 3 out of 60 patients(73%) and 12 out of to (20%) respectively. Six out of these 12 patients were NYHA class III-IV who underwent cardiac catheterization which revealed patch dehiscence in two patients and residual right ventricular outflow tract obstruction in four patients. The two patients, with patch dehiscence were reported successfully with very good outcome. Simple color doppler mapping tends to over-diagnose residual VSDs since it can pick up small haemodynamically insignificant leaks around stitches and needle holes which seal off in due course. The findings of color doppler should, therefore, be correlated with actual clinical outcome in order to proceed with further investigation and re-operation. (author)

  11. Horizontal right axillary minithoracotomy: aesthetic and effective option for atrial and ventricular septal defect repair in infants and toddlers

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    Luciana da Fonseca da Silva

    2014-04-01

    Full Text Available Introduction: Congenital heart defects treatment shows progressive reduction in morbidity and mortality, however, the scar, resulting from ventricular (VSD and atrial septal defect (ASD repair, may cause discomfort. Right axillary minithoracotomy approach, by avoiding the breast growth region, is an option for correction of these defects that may provide better aesthetic results at low cost. Since October 2011, we have been using this technique for repairing VSD and ASD defects as well as associated defects. Objectives: To evaluate the efficacy of this method in children undergoing correction of VSD and ASD, to compare perioperative clinical outcomes with those repaired by median sternotomy, and to evaluate the aesthetic result. Methods: Perioperative clinical data of 25 patients submitted to axillary thoracotomy were compared with data from a paired group of 25 patients with similar heart defects repaired by median sternotomy, from October 2011 to August 2012. Results: Axillary approach was possible even in infants. There was no mortality and the main perioperative variables were similar in both groups, except for lower use of blood products in the axillary group (6/25 vs. control (13/25, with statistical difference (P =0.04. The VSD size varied from 7 to 15 mm in axillary group. Cannulation of the aorta and vena cavae was performed through the main incision, whose size ranged from 3 to 5 cm in the axillary group, with excellent aesthetic results. Conclusion: The axillary thoracotomy was effective, allowing for a heart defect repair similar to the median sternotomy, with more satisfactory aesthetic results and reduced blood transfusion, and it can be safely used in infants.

  12. Alergia al néquel manifestada como edema pulmonar no cardiogénico en paciente pos-cierre de comunicación interauricular con dispositivo tipo Amplatzer Nickel allergy manifested as noncardiogenic pulmonary edema in a patient post-closure of atrial septal defect with Amplatzer device

    Directory of Open Access Journals (Sweden)

    Luis A Gutiérrez

    2012-10-01

    Full Text Available El cierre percutáneo es la modalidad predilecta para el tratamiento de los defectos septales tipo ostium secundum cuando la anatomía es favorable, y reporta una tasa de éxito excelente así como también un bajo porcentaje de complicaciones. Se presenta el caso de un cierre exitoso de defecto septal tipo ostium secundum con dispositivo tipo Amplatzer en un paciente con antecedente de alergia a metales no detectada previamente, quien presentó edema pulmonar no cardiogénico, fiebre y pericarditis secundarios al níquel del dispositivo, pero tuvo mejoría y evolución satisfactoria con tratamiento médico.The percutaneous closure of ostium secundum septal defects is the preferred treatment modality when the anatomy is appropriate, as it shows high success and low complication rates. We present a case of a succesful percutaneous closure of an ostium secundum septal defect with an Amplatzer septal occluder device in a patient with an undetected metal allergy which led her to non cardiogenic pulmonary edema, fever and pericarditis related to the nickel contained in the device, with improvement and satisfactory evolution after medical treatment.

  13. Multiple ventricular septal defects: a new strategy.

    Science.gov (United States)

    Corno, Antonio F; Kandakure, Pramod R; Dhannapuneni, Ramana Rao V; Gladman, Gordon; Venugopal, Prem; Alphonso, Nelson

    2013-01-01

    A multicenter prospective study was conducted to evaluate a new strategy for multiple Ventricular Septal Defects (VSDs). From 2004 to 2012 17 consecutive children (3 premature, 14 infants), mean age 3.2 months (9 days-9 months), mean body weight 4.2 kg (3.1-6.1 kg), with multiple VSDs underwent Pulmonary Artery Banding (PAB) with an adjustable FloWatch-PAB(®). Associated cardiac anomalies included patent ductus arteriosus (1), aortic coarctation (2), hypoplastic aortic arch (2), and left isomerism (3). Five patients (5/17 = 29.4%) required pre-operative mechanical ventilation, with a mean duration of 64 days (7-240 days) There were no early or late deaths during a mean follow-up of 48 months (7-98 months), with either FloWatch removal or last observation as end-points. FloWatch-PAB(®) adjustments were required in all patients: a mean of 4.8 times/patient (2-9) to tighten the PAB, and a mean of 1.1 times/patient (0-3) to release the PAB with the patient's growth. After a mean interval of 29 months (8-69 months) 10/17 (59%) patients underwent re-operation: 7/10 PAB removal, with closure of a remaining unrestrictive VSD in 6 (peri-membranous in 3 patients, mid-muscular in 2, and inlet in 1) and Damus-Kaye-Stansel, bi-directional Glenn, and atrial septectomy in 1; 3/9 patients required only PAB removal. All muscular multiple VSDs had closed in all 10 patients. PA reconstruction was required in 1/10 patient. In 5/7 of the remaining patients with the PAB still in situ, all muscular VSDs had already closed. The only 2 patients with persistent muscular multiple VSDs are the 2 patients with the shortest follow-up. This reproducible new strategy with an adjustable PAB simplifies the management of infants with multiple VSDs and provides the following advantages: (a) good results (0% mortality), delayed surgery with a high incidence (15/17 = 88%) of spontaneous closure of multiple muscular VSDs, and facilitated closure of residual unrestrictive

  14. Consanguinity and isolated atrial septal defect in North East of Iran.

    Science.gov (United States)

    Moghaddam, Hasan Mottaghi; Esfehani, Reza Jafarzadeh; Panah, Nader Yazdan; Esfehani, Ali Jafarzadeh

    2014-01-01

    The rate of consanguineous marriage is high in Middle Eastern countries such as Iran. The relationship between consanguineous marriage and congenital heart disease is discussed in some studies, but there is not much data for relationship between atrial septal defect (ASD) and consanguineous marriage. The aim of this study was to evaluate the relationship between consanguineous marriage and ASD echocardiographic characteristics. This was a cross-sectional study approved by Mashhad University of Medical Sciences ethics committee and took place in Mashhad, Iran, for a period of 3 years from August 2008 till September 2011. In this cross-sectional study, 113 ASD patients participated and they were categorized into 3 groups on the basis of family relationship between their parents: first group-"no relationship," second group- "third degree relationship," and third group- "far relationship." Among the 54 male and 59 female ASD patients, the most prevalent type of ASD was ASD secundum (85.0%) followed by sinus venosus (8.8%). A total of 56% patients were present in the first group and 15% and 29% in the second group and the third group, respectively." The relationship between consanguinity and type of ASD (P relationship between the age of onset of disease and consanguinity (P=.003) was also observed. Considering the fact that there is a high prevalence of ASD and consanguineous marriage in Iran and bearing in mind the results of the present study, we recommend educating couples about the outcomes of consanguineous marriage in pre-marriage counseling.

  15. Estenose subaórtica associada a comunicação interventricular perimembranosa: acompanhamento clínico de 36 pacientes Subaortic stenosis associated with perimembranous ventricular septal defect: clinical follow-up of 36 patients

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    Maria da Gloria Cruvinel Horta

    2005-02-01

    Full Text Available OBJETIVO: Estudar o comportamento clínico da estenose subaórtica associada a comunicação interventricular perimembranosa. MÉTODOS: Foram acompanhadas, de janeiro 1979 a junho 2000, quanto às características anatômicas, caráter evolutivo e eventos clínicos, 36 crianças com comunicação interventricular perimembranosa e estenose subaórtica fixa. RESULTADOS: A idade de diagnóstico da estenose subaórtica fixa variou de seis meses a 170 meses, sendo abaixo de 1 ano apenas em duas crianças. Quanto ao sexo a distribuição foi de 2:1 com grande predomínio do masculino. A comunicação interventricular era de tamanho pequeno em 61,00% dos casos, médio em 30,56% e grande em 8,40%, apresentando diminuição do tamanho da comunicação durante o acompanhamento em 30,56% (11 casos. Em todos os pacientes a estenose subaórtica era fixa, em membrana. Durante o tempo de acompanhamento, 23 pacientes apresentaram progressão da estenose. Foi realizado tratamento cirúrgico em 21 casos, sendo um paciente reoperado por reestenose. Endocardite bacteriana ocorreu em dois casos, um deles faleceu. CONCLUSÃO: A estenose subaórtica ocorre na história natural da comunicação interventricular geralmente após o 1º ano de vida, apresentando caráter progressivo e necessitando de cirurgia na maioria dos casos.OBJECTIVE: To study the clinical pattern of subaortic stenosis associated with perimembranous ventricular septal defect. METHODS: From January 1979 to June 2000, 36 children with perimembranous ventricular septal defect and fixed subaortic stenosis were followed-up regarding anatomic characteristics, evolvement, and clinical events. RESULTS: Age at diagnosis of subaortic stenosis ranged from 6 months to 170 months, and it was less than 1 year in only 2 children. Regarding sex, the distribution was 2:1 with a greater predominance of males. Ventricular septal defect was small in 61.0% of cases, medium in 30.56%, and large in 8.40%; the size of the

  16. Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary arterial hypertension associated with congenital heart disease

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    Antonio Lopes

    2014-01-01

    Full Text Available Congenital heart disease (CHD with intracardiac/extracardiac shunts is an important etiology of pulmonary arterial hypertension (PAH. The majority of children with congenital cardiac shunts do not develop advanced pulmonary vasculopathy, as surgical repair of the anomalies is now performed early in life. However, if not repaired early, some defects will inevitably lead to pulmonary vascular disease (truncus arteriosus, transposition of the great arteries associated with a ventricular septal defect (VSD, atrioventricular septal defects remarkably in Down syndrome, large, nonrestrictive VSDs, patent ductus arteriosus and related anomalies. The majority of patients are now assigned to surgery based on noninvasive evaluation only. PAH becomes a concern (requiring advanced diagnostic procedures in about 2-10% of them. In adults with CHD, the prevalence of advanced pulmonary vasculopathy (Eisenmenger syndrome is around 4-12%. [1] This article will discuss the diagnostic and management approach for PAH associated with CHD (PAH-CHD.

  17. Prenatal detection of structural cardiac defects and presence of associated anomalies: a retrospective observational study of 1262 fetal echocardiograms.

    Science.gov (United States)

    Mone, Fionnuala; Walsh, Colin; Mulcahy, Cecelia; McMahon, Colin J; Farrell, Sinead; MacTiernan, Aoife; Segurado, Ricardo; Mahony, Rhona; Higgins, Shane; Carroll, Stephen; McParland, Peter; McAuliffe, Fionnuala M

    2015-06-01

    The aim of this study is to document the detection of fetal congenital heart defect (CHD) in relation to the following: (1) indication for referral, (2) chromosomal and (3) extracardiac abnormalities. All fetal echocardiograms performed in our institution from 2007 to 2011 were reviewed retrospectively. Indication for referral, cardiac diagnosis based on the World Health Organization International Classification of Diseases tenth revision criteria and the presence of chromosomal and extracardiac defects were recorded. Of 1262 echocardiograms, 287 (22.7%) had CHD. Abnormal anatomy scan in pregnancies originally considered to be at low risk of CHD was the best indicator for detecting CHD (91.2% of positive cardiac diagnoses), compared with other indications of family history (5.6%) or maternal medical disorder (3.1%). Congenital anomalies of the cardiac septa comprised the largest category (n = 89), within which atrioventricular septal defects were the most common anomaly (n = 36). Invasive prenatal testing was performed for 126 of 287 cases, of which 44% (n = 55) had a chromosomal abnormality. Of 232 fetuses without chromosomal abnormalities, 31% had an extracardiac defect (n = 76). Most CHDs occur in pregnancies regarded to be at low risk, highlighting the importance of a routine midtrimester fetal anatomy scan. Frequent association of fetal CHD and chromosomal and extracardiac pathology emphasises the importance of thorough evaluation of any fetus with CHD. © 2015 John Wiley & Sons, Ltd.

  18. Birth outcomes of cases with isolated atrial septal defect type II--a population-based case-control study.

    Science.gov (United States)

    Vereczkey, Attila; Kósa, Zsolt; Csáky-Szunyogh, Melinda; Urbán, Róbert; Czeizel, Andrew E

    2013-07-01

    In general, epidemiological studies have evaluated cases with congenital cardiovascular abnormalities together. The aim of this study is to describe the birth outcomes of cases with isolated/single atrial septal defect type II (ASD-II, i.e. only a fossa ovalis defect) after surgical correction or lethal outcome in the light of maternal sociodemographic data. Comparison of birth outcomes and maternal characteristics of cases with ASD-II and controls without defect. The population-based Hungarian Case-Control Surveillance of Congenital Abnormalities. Hungarian newborn infants with or without ASD-II. Medically recorded birth outcomes, maternal age and birth order were evaluated. Marital and employment status was based on maternal information. The lifestyle factors were analyzed in a subsample of mothers visited at home based on a personal interview with mothers and their close relatives, and the family consensus was accepted. Mean gestational age at delivery and birthweight, rate of preterm birth and low birthweight, maternal age, birth order, marital and employment status. The evaluation of 471 cases with ASD-II and 38,151 controls without any defects showed a female excess in cases with ASD-II, having shorter gestational age and lower mean birthweight, and thus a higher rate of preterm births and low birthweight. Intrauterine growth restriction and shorter gestational age were found in cases with ASD-II, particularly in female children. These factors may have a general developmental process in which there was not closure of the foramen ovale, thus echocardiographic screening of these babies might be of value. © 2012 The Authors Acta Obstetricia et Gynecologica Scandinavica © 2012 Nordic Federation of Societies of Obstetrics and Gynecology.

  19. Epicardial deployment of right ventricular disk during perventricular device closure in a child with apical muscular ventricular septal defect

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    Nageswara Rao Koneti

    2013-01-01

    Full Text Available We report a successful perventricular closure of an apical muscular ventricular septal defect (mVSD by a modified technique. An eight-month-old infant, weighing 6.5 kilograms, presented with refractory heart failure. The transthoracic echocardiogram showed multiple apical mVSDs with the largest one measuring 10 mm. perventricular device closure using a 12 mm Amplatzer mVSD occluder was planned. The left ventricular disk was positioned approximating the interventricular septum; however, the right ventricular (RV disk was deployed on the free wall of the RV due to an absent apical muscular septum and a small cavity at the apex. The RV disk of the device was covered using an autologous pericardium. His heart failure improved during follow-up.

  20. Congenital heart defects in Williams syndrome.

    Science.gov (United States)

    Yuan, Shi-Min

    2017-01-01

    Yuan SM. Congenital heart defects in Williams syndrome. Turk J Pediatr 2017; 59: 225-232. Williams syndrome (WS), also known as Williams-Beuren syndrome, is a rare genetic disorder involving multiple systems including the circulatory system. However, the etiologies of the associated congenital heart defects in WS patients have not been sufficiently elucidated and represent therapeutic challenges. The typical congenital heart defects in WS were supravalvar aortic stenosis, pulmonary stenosis (both valvular and peripheral), aortic coarctation and mitral valvar prolapse. The atypical cardiovascular anomalies include tetralogy of Fallot, atrial septal defects, aortic and mitral valvular insufficiencies, bicuspid aortic valves, ventricular septal defects, total anomalous pulmonary venous return, double chambered right ventricle, Ebstein anomaly and arterial anomalies. Deletion of the elastin gene on chromosome 7q11.23 leads to deficiency or abnormal deposition of elastin during cardiovascular development, thereby leading to widespread cardiovascular abnormalities in WS. In this article, the distribution, treatment and surgical outcomes of typical and atypical cardiac defects in WS are discussed.

  1. Prenatal diagnosis of two fetuses with deletions of 8p23.1, critical region for congenital diaphragmatic hernia and heart defects.

    Science.gov (United States)

    Keitges, Elisabeth A; Pasion, Romela; Burnside, Rachel D; Mason, Carla; Gonzalez-Ruiz, Antonio; Dunn, Teresa; Masiello, Meredith; Gebbia, Joseph A; Fernandez, Carlos O; Risheg, Hiba

    2013-07-01

    Microdeletions of 8p23.1 are mediated by low copy repeats and can cause congenital diaphragmatic hernia (CDH) and cardiac defects. Within this region, point mutations of the GATA4 gene have been shown to cause cardiac defects. However, the cause of CDH in these deletions has been difficult to determine due to the paucity of mutations that result in CDH, the lack of smaller deletions to refine the region and the reduced penetrance of CDH in these large deletions. Mice deficient for one copy of the Gata4 gene have been described with CDH and heart defects suggesting mutations in Gata4 can cause the phenotype in mice. We report on the SNP microarray analysis on two fetuses with deletions of 8p23.1. The first had CDH and a ventricular septal defect (VSD) on ultrasonography and a family history of a maternal VSD. Microarray analysis detected a 127-kb deletion which included the GATA4 and NEIL2 genes which was inherited from the mother. The second fetus had an incomplete atrioventricular canal defect on ultrasonography. Microarray analysis showed a 315-kb deletion that included seven genes, GATA4, NEIL2, FDFT1, CTSB, DEFB136, DEFB135, and DEFB134. These results suggest that haploinsufficiency of the two genes in common within 8p23.1; GATA4 and NEIL2 can cause CDH and cardiac defects in humans. Copyright © 2013 Wiley Periodicals, Inc.

  2. Device closure of secundum atrial septal defect's and the risk of cardiac erosion.

    Science.gov (United States)

    Thomson, J D R; Qureshi, S A

    2015-12-01

    Cardiac erosion related to transcatheter atrial septal defect closure devices is of increasing concern. Erosion is reported to have occurred with most of currently available occluder devices. Perhaps due to the very large number of implants worldwide, the Amplatzer (St Jude) occluder is associated with the majority of cardiac erosion events reported in the literature. Best current estimates of the incidence of erosion with the St Jude device are between one and three cases per 1000 implants. Most events occur early after implantation and it is rare, although not unheard of, for events to occur after a year following device insertion. It is important that those involved with closure programmes are vigilant for the problem, because device-related erosion is associated with a significant mortality risk. Despite considerable debate, the risk factors (either patient or device) for erosion remain unclear and require further investigation. Currently available data sets have focussed largely on erosion cohorts and are unable to place these cases in appropriate context with non-erosion closure cases. What is certain is that programmes implanting these devices must take care to implant appropriately sized devices and have in place plans to ensure that patients are both well informed and can access help and advice in the event of developing symptoms.

  3. Clinical long-term outcome of septal myectomy for obstructive hypertrophic cardiomyopathy in infants.

    Science.gov (United States)

    Schleihauf, Julia; Cleuziou, Julie; Pabst von Ohain, Jelena; Meierhofer, Christian; Stern, Heiko; Shehu, Nerejda; Mkrtchyan, Naira; Kaltenecker, Emanuel; Kühn, Andreas; Nagdyman, Nicole; Hager, Alfred; Seidel, Heide; Lange, Rüdiger; Ewert, Peter; Wolf, Cordula M

    2018-03-01

    Surgical septal myectomy is performed to relieve left ventricular outflow tract narrowing in severe drug-refractory obstructive hypertrophic cardiomyopathy. The objective of this study was to assess the perioperative and long-term clinical outcome of this procedure performed during infancy. Clinical, transthoracic echocardiographic, electrocardiographic, 24-h Holter, cardiopulmonary exercise test and genetic data were extracted by medical record review. A subset of patients underwent additional prospective detailed clinical evaluation including cardiac magnetic resonance imaging with contrast. Surgery was performed in 23 paediatric patients between 1978 and 2015 at the German Heart Centre Munich. Twelve patients had undergone surgery during infancy (≤ 1 year) (Group A), 11 between 1 and 18 years of age (Group B). The underlying genetic diagnosis was Noonan syndrome spectrum and non-syndromic hypertrophic cardiomyopathy. As compared to Group B, patients in Group A showed more concomitant cardiac procedures and received more homologous transfusions. One perioperative death occurred in Group A, and none in Group B. Two patients in Group A but no patient in Group B required redo septal myectomy. The long-term clinical outcome was similar between the 2 groups. One patient in Group B required cardioverter-defibrillator/pacemaker implantation for higher degree atrioventricular block and none in Group A. There was no evidence of differences in myocardial fibrosis between groups on long-term follow-up magnetic resonance imaging. Surgical septal myectomy can be performed safely during infancy with favourable perioperative and long-term clinical outcome but with a trend towards a higher reoperation rate later in life. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  4. Anatomy of the human atrioventricular junctions revisited

    NARCIS (Netherlands)

    Anderson, R. H.; Ho, S. Y.; Becker, A. E.

    2000-01-01

    There have been suggestions made recently that our understanding of the atrioventricular junctions of the heart is less than adequate, with claims for several new findings concerning the arrangement of the ordinary working myocardium and the specialised pathways for atrioventricular conduction. In

  5. No patch technique for complete atrioventricular canal repair.

    Science.gov (United States)

    Aramendi, José Ignacio; Rodriguez, Miguel Angel; Luis, Teresa; Voces, Roberto

    2006-08-01

    We describe our initial experience with a new technique, consisting in direct closure of the ventricular septal defect component of the AV canal, by directly attaching the common bridging leaflets to the crest of the ventricular septum with interrupted sutures. After closure of the cleft, the ostium primum defect was closed with a running suture suturing the border of the septum primum to the newly created AV valve annulus. Three patients were operated upon. There was no mortality. Mean ischemic time was 39 min and mean pump time 77 min. All patients remained in sinus rhythm. At follow-up only trivial or mild mitral regurgitation was observed. This new technique permits the repair of complete AV canal without the need for any patch. It is fast, simple and reproducible.

  6. Isl1 expression at the venous pole identifies a novel role for the second heart field in cardiac development.

    Science.gov (United States)

    Snarr, Brian S; O'Neal, Jessica L; Chintalapudi, Mastan R; Wirrig, Elaine E; Phelps, Aimee L; Kubalak, Steven W; Wessels, Andy

    2007-11-09

    The right ventricle and outflow tract of the developing heart are derived from mesodermal progenitor cells from the second heart field (SHF). SHF cells have been characterized by expression of the transcription factor Islet-1 (Isl1). Although Isl1 expression has also been reported in the venous pole, the specific contribution of the SHF to this part of the heart is unknown. Here we show that Isl1 is strongly expressed in the dorsal mesenchymal protrusion (DMP), a non-endocardially-derived mesenchymal structure involved in atrioventricular septation. We further demonstrate that abnormal development of the SHF-derived DMP is associated with the pathogenesis of atrioventricular septal defects. These results identify a novel role for the SHF.

  7. [Reversible first-degree atrioventricular block due to hyperthyroidism].

    Science.gov (United States)

    Çelebi, Aksüyek Savaş; Amasyalı, Basri

    2017-04-01

    Hyperthyroidism often causes tachyarrhythmia. Reversible atrioventricular block caused by hyperthyroidism is rare occurrence. Presently described is a case of atrioventricular block due to hyperthyroidism and recovery after antithyroid treatment.

  8. Managing Ventricular Septal Defect with Associated Aortic Regurgitation: Two Decades of Experience.

    Science.gov (United States)

    Sanoussi, Ahmed; Demanet, Helene; Dessy, Hughes; Massin, Martial; Biarent, Dominique; Deville, Andree; Wauthy, Pierre

    2015-09-01

    Ventricular septal defect (VSD) with aortic regurgitation (AR) is a well-known association. However, there is still no agreement about its management, particularly regarding the technical details of its operative treatment. The study aim was to describe all components of the syndrome and to evaluate the various techniques used with regards to its anatomical and functional features. A total of 31 patients (mean age 7.4 years; range: 1.0-14.3 years) who underwent repair of VSD and AR between 1990 and 2013 was reviewed. The VSD was perimembranous in 22 patients, and subarterial in nine. Trusler's valvuloplasty technique was used in 15 patients, Yacoub's technique in seven, and Carpentier's technique (triangular resection) in four. Two patients underwent aortic valve replacement (AVR), and three patients with no significant aortic valve lesions underwent a simple patch repair of the VSD. The aortic valvuloplasty results were generally good, with an initial aortic valvuloplasty avoiding AVR. During the immediate postoperative period, valvuloplasty failure occurred in three patients, regardless of the technique used, and all three patients were reoperated on. The mean duration of follow up was 8.5 years (range: 3.2-20.6 years). The initial result was maintained in all patients, except for four who underwent late AVR. The study findings contributed to an analysis of VSD and AR, and helped to clarify the best surgical strategy. The results obtained suggest that adequacy of the initial repair is the most important determinant of subsequent evolution.

  9. Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: A case report with review of literature

    Directory of Open Access Journals (Sweden)

    Lalita Nemani

    2014-01-01

    Full Text Available We report a case of type-A Coffin-Siris syndrome (CSS with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD, subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right ventricular infundibular resection, subaortic membrane resection, closure of atrial and ventricular septal defect, rerouting left superior vena cava to left pulmonary artery and aortic valve replacement.

  10. Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: A case report with review of literature.

    Science.gov (United States)

    Nemani, Lalita; Barik, Ramachandra; Patnaik, Amar Narayana; Mishra, Ramesh C; Rao, Amaresh M; Kapur, Pragati

    2014-09-01

    We report a case of type-A Coffin-Siris syndrome (CSS) with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD), subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right ventricular infundibular resection, subaortic membrane resection, closure of atrial and ventricular septal defect, rerouting left superior vena cava to left pulmonary artery and aortic valve replacement.

  11. Nasal septal hematoma

    Science.gov (United States)

    ... medlineplus.gov/ency/article/001292.htm Nasal septal hematoma To use the sharing features on this page, please enable JavaScript. A nasal septal hematoma is a collection of blood within the septum ...

  12. Surgical treatment of ventricular septal defect combined with tricuspid valve insufficiency

    Directory of Open Access Journals (Sweden)

    L. Maniuc

    2016-11-01

    Full Text Available The aim – to evaluate different methods of surgical treatment of ventricular septal defect (VSD, combined with failure of the tricuspid valve (TC, and to develop optimal algorithm for the treatment of patients with this pathology. Materials and methods. Between 2010 and 2014, 35 patients, average age 80.9±20.5 months, underwent tricuspidal annuloplasty within correction of VSD in Center of Cardiac Surgery of Republic of Moldova. Tricuspidal regurgitation of the II grade was diagnosed valve in 20 (57.0 % cases, III grade – in 8 (23.0 % cases, IV grade – in 7 (20.0 % of cases. Within correction of VSD plastics of tricuspidal valve was performed: in 4 cases (11.0 % of patients plastics by De Vega, in 14 cases (40.0 % of patients – comissuroplastics, in 6 cases (17.0 % – comissuroplastics and suture of cleft, in 1 case (4.0 % plastics by De Vega with comissuroplastics, in 10 cases (29.0 % – comissuroplastics and suture of cleft. Results. After operation the clinic status improved significantly: breathlessness reduced from 91.7 % to 8.3 % cases, tachycardia reduced from 91.7 % to 33.3 % cases and other cardiac failure symptoms – from 10.8 % to 4.2 % cases. The number of patients with NYHA class I heart failure after surgery was 54.2 % compared to its absence before operation, class 2 diminished from 60.0 % to 41.7 % cases, class 3 – from 36.0 % to 4.2 % cases. Conclusions. Anteroseptal comissuroplastics was used in majority of cases. This method is simple, reliable and inexpensive, requires not more than 5–10 min and significantly reduces tricuspidal valve insufficiency.

  13. Atrioventricular and Interventricular Groove and Septal Extension of Right Sinus of Valsalva Aneurysm: A Rare Cause of Complete Heart Block

    International Nuclear Information System (INIS)

    Khan, J.A.; Hussain, M.; Rizvi, N.H.; Fehmi, N.; Hussain, A.; Sial, J.A.

    2013-01-01

    A 26 years old male presented with vertigo and history of fall. The electrocardiogram revealed 2:1 second-degree heart block and later progression to complete heart block. Transthoracic echocardiography revealed aneurysm at the site of ascending aorta and computed tomographic scan showed an aneurysm of right sinsus of Valsalva extending into right atrioventricular and interventricular groove and causing complete heart block by compression on the conduction system. He also suffered from lymph node tuberculosis. This case report is unique because of rare presentation as complete heart block. (author)

  14. Repair of Double Orifice Left AV Valve (DOLAVV with Endocardial Cushion Defect in Adult

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    Vivek Velayudhan Pillai

    Full Text Available Abstract Double orifice left atrioventricular valve (DOLAVV or double orifice mitral valve (DOMV is a rare congenital cardiac anomaly manifesting either as an isolated lesion (mitral stenosis or mitral insufficiency or in association with other congenital cardiac defects. Signs of mitral valve disease are usually present along with the symptoms of associated coexistent congenital heart diseases. Mitral insufficiency due to annular dilatation is seen when DOLAVV is associated with endocardial cushion defects. Surgical intervention like mitral valve repair or replacement is required in 50% of patients and yields good results. We report a case of a 56-year-old lady who successfully underwent surgical correction of DOLAVV with partial atrioventricular canal defect.

  15. Deletion of ETS-1, a gene in the Jacobsen syndrome critical region, causes ventricular septal defects and abnormal ventricular morphology in mice

    Science.gov (United States)

    Ye, Maoqing; Coldren, Chris; Liang, Xingqun; Mattina, Teresa; Goldmuntz, Elizabeth; Benson, D. Woodrow; Ivy, Dunbar; Perryman, M.B.; Garrett-Sinha, Lee Ann; Grossfeld, Paul

    2010-01-01

    Congenital heart defects comprise the most common form of major birth defects, affecting 0.7% of all newborn infants. Jacobsen syndrome (11q-) is a rare chromosomal disorder caused by deletions in distal 11q. We have previously determined that a wide spectrum of the most common congenital heart defects occur in 11q-, including an unprecedented high frequency of hypoplastic left heart syndrome (HLHS). We identified an ∼7 Mb ‘cardiac critical region’ in distal 11q that contains a putative causative gene(s) for congenital heart disease. In this study, we utilized chromosomal microarray mapping to characterize three patients with 11q- and congenital heart defects that carry interstitial deletions overlapping the 7 Mb cardiac critical region. We propose that this 1.2 Mb region of overlap harbors a gene(s) that causes at least a subset of the congenital heart defects that occur in 11q-. We demonstrate that one gene in this region, ETS-1 (a member of the ETS family of transcription factors), is expressed in the endocardium and neural crest during early mouse heart development. Gene-targeted deletion of ETS-1 in mice in a C57/B6 background causes, with high penetrance, large membranous ventricular septal defects and a bifid cardiac apex, and less frequently a non-apex-forming left ventricle (one of the hallmarks of HLHS). Our results implicate an important role for the ETS-1 transcription factor in mammalian heart development and should provide important insights into some of the most common forms of congenital heart disease. PMID:19942620

  16. Brain Abscess Associated with Isolated Left Superior Vena Cava Draining into the Left Atrium in the Absence of Coronary Sinus and Atrial Septal Defect

    International Nuclear Information System (INIS)

    Erol, Ilknur; Cetin, I. Ilker; Alehan, Fuesun; Varan, Birguel; Ozkan, Sueleyman; Agildere, A. Muhtesem; Tokel, Kursad

    2006-01-01

    A previously healthy 12-year-old girl presented with severe headache for 2 weeks. On physical examination, there was finger clubbing without apparent cyanosis. Neurological examination revealed only papiledema without focal neurologic signs. Cerebral magnetic resonance imaging showed the characteristic features of brain abscess in the left frontal lobe. Cardiologic workup to exclude a right-to-left shunt showed an abnormality of the systemic venous drainage: presence of isolated left superior vena cava draining into the left atrium in the absence of coronary sinus and atrial septal defect. This anomaly is rare, because only a few other cases have been reported

  17. Transcatheter closure of large atrial septal defects with deficient aortic or posterior rims using the "Greek maneuver". A multicenter study.

    Science.gov (United States)

    Thanopoulos, Basil D; Dardas, Petros; Ninios, Vlasis; Eleftherakis, Nicholaos; Karanasios, Evangelos

    2013-10-09

    We report a modification ("Greek maneuver") of the standard atrial septal defect (ASD) closure technique using the Amplatzer septal occluder (ASO) to facilitate closure of large ASDs with deficient aortic or posterior rims. 185 patients (median 10.8, range 3 to 52 years) with large ASDs (mean diameter 26±7 mm, range 20-40 mm) with a deficient aortic (134 patients) or posterior (51 patients) rim underwent catheter closure with the ASO using the "Greek maneuver" under transesophageal guidance. The Greek maneuver is applied when protrusion of the aortic edge of the deployed left disk of the device in to the right atrium is detected by echo. To circumvent this left disk is recaptured and the whole delivery system is pushed inward and leftward into the left atrium where the left disk and the 2/3 of right disk are simultaneously released. This maneuver forces the left disk to become parallel to the septum preventing the protrusion of the device into the right atrium. The ASO was successfully implanted and was associated with complete closure in 175/185 (95%) of the patients. There were no early or late complications related to the procedure during a follow-up period ranging from 6 months to 7 years. The "Greek maneuver" is a simple quite useful trick that facilitates closure of large ASDs associated with or without deficient aortic or posterior rims. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  18. Technical modification enabling pulmonary valve-sparing repair of a severely hypoplastic pulmonary annulus in patients with tetralogy of Fallot.

    Science.gov (United States)

    Ito, Hiroki; Ota, Noritaka; Murata, Masaya; Tosaka, Yuko; Ide, Yujiro; Tachi, Maiko; Sugimoto, Ai; Sakamoto, Kisaburo

    2013-06-01

    Although pulmonary valve-sparing repair is preferable for patients with tetralogy of Fallot, the repair of very small pulmonary valves is challenging. The present study evaluates our modification for preserving severely hypoplastic pulmonary valves in patients with tetralogy of Fallot. Sixty-eight consecutive patients who underwent complete repair of a tetralogy of Fallot between 2005 and 2011 were retrospectively reviewed. Patients with pulmonary atresia, absence of a pulmonary valve, atrioventricular septal defect and/or subarterial ventricular septal defect were excluded. There were 19 (28%) patients with a severely hypoplastic pulmonary annulus determined by preoperative echocardiography (z-score -4 group. In the z tetralogy of Fallot could not be applied in all patients, this strategy enabled acceptable growth of the valve annulus, with only mild stenosis during the early to mid-term follow-up. This modification seems to be an option, even for a very small pulmonary valve.

  19. Right ventricular hypoplasia syndrome. Atresia of the right atrioventricular ostium (X-ray examination)

    International Nuclear Information System (INIS)

    Golonzko, R.R.; Chernova, M.P.; Berishvili, I.I.; Bulchinskij, S.A.

    1982-01-01

    Data on routine radiologic examination of 51 patients aged 3 days to 3 years with the right atrio-ventricular ostium atresia were presented. The evidence obtained was compared with angiographic and, in part of cases, with morphometric findings. All patients were divided in two groups, depending on pulmonary circulation functions. Characteristic radiologic features of the valvular defect were revealed: ''quadrangle'' heart configuration; increase of right auricle; ''sheared off'' profile of the right ventricle, absence of increase of leftauricle in children up to 1 year

  20. Late bacterial endocarditis of an Amplatzer atrial septal device

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    Bhavith Aruni

    2013-07-01

    Full Text Available A 59-year-old male with an secundum atrial septal defect status post repair with an Amplatzer occluder in 2001 was admitted with sepsis and MRSA bacteremia. Transesophageal Echocardiography (TEE showed presence of an overlying mobile echogenic structure on the left atrial surface of the device suggestive of a vegetation/infected thrombus. This is only the 3rd case description of late endocarditis involving the Amplatzer ASD closure device in an adult.

  1. Transcatheter occlusion of perimembranous ventricular septal defects with Amplatzer duct occluder

    International Nuclear Information System (INIS)

    Ren Sengen; Kang Kang; Wu Danning; Shi Hong; Wu Jingzhang; Yang Mei; Han Feizhou; Zhou Fei; Zhu Zhijun

    2003-01-01

    Objective: To study the feasibility of congenital perimembranous ventricular septal defect (VSD) occlusion by Amplatzer duct occluder (ADO) and to establish guidelines for its safe and effective application. Methods: From May 2001 to December 2002, percutaneous transcatheter occlusion of congenital perimembranous VSD was performed in 41 patients, in which 18 patients had an associated aneurysm of the membranous septum. There were 20 male and 21 female subjects, with age ranging from 6 to 38 years (median 14.9) and weights of 18 to 62 kg (median 41.5). The diameter of VSD ranged from 4 to 12.8 mm (median 6.0), and the distance of VSD to the aortic valve were 4.5 to 12 mm (median 6.6). The ratio of pulmonary to systemic blood flow (Qp/Qs) was 1.4 to 2.6 (median 1.7) and the pulmonary systolic pressure was 19 to 34 mmHg (median 25). Results: All the patients underwent successfully complete occlusion of VSD with ADO, and the size of ADO ranged from 6/4 to 16/14 mm. After the procedure, left ventricular angiography and transthoracic echocardiography showing the ADOs were precisely placed inside the VSDs with no residual shunt and also no abnormalities revealed by auscultation. Chest X-ray showed the relief of congestion of the lungs. Electrocardiogram demonstrated complete left bundle branch block in one case and incomplete right bundle branch block in other 12 cases, all disappeared within 1 to 2 weeks. Over a period of 2 to 21 months follow-up, all the devices maintained in good position and the patients' condition turned much better. No patient developed aortic or tricuspid regurgitation and other complications. Conclusions: ADO is a safe, effective and convenient occlusion device which could be practically applied for the congenital perimembranous VSDs

  2. Coexisting ventricular septal defect affects the features of ruptured sinus of Valsalva aneurysms

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    Yan Jin

    2017-03-01

    Full Text Available Objectives: To determine the correlation exists between ventricular septal defect (VSD and ruptured sinus of Valsalva aneurysm (RSVA. Methods: Between September 2003 and April 2014, 80 RSVA patients underwent surgical repair. These patients were retrospectively divided into two groups: the VSD group (38 cases and the non-VSD group (42 cases. Results: Rupture points of SVA originated more frequently in the right coronary sinus (RCS of patients in the VSD group than those in the non-VSD group (p=0.002. In the VSD group, more than 92.1% tended to rupture into the right ventricular outflow tract. The rupture points are diverse in the non-VSD group. A significant difference was found in rupture points of RSVA between the two groups (p<0.001. Patients in the VSD group presented with aortic valve disease more often than those in the non-VSD group (p<0.001. A total of 67 patients were repaired with a patch at the opening of RSVA; of those, all patients in VSD group and 29 patients in non-VSD group were repaired with a patch. Nine patients in non-VSD group received transcatheter closure of RSVA. Conclusion: The presence or absence of VSD affects the rupture points of SVA, aortic valve disease involved, and therapeutic schedule. Ruptured sinus of Valsalva aneurysm type should be clinically modified on the basis of presence or absence of VSD.

  3. The cardiac proteome in patients with congenital ventricular septal defect: A comparative study between right atria and right ventricles.

    Science.gov (United States)

    Bond, A R; Iacobazzi, D; Abdul-Ghani, S; Ghorbel, M T; Heesom, K J; George, S J; Caputo, M; Suleiman, M-S; Tulloh, R M

    2018-03-20

    Right ventricle (RV) remodelling occurs in neonatal patients born with ventricular septal defect (VSD). The presence of a defect between the two ventricles allows for shunting of blood from the left to right side. The resulting RV hypertrophy leads to molecular remodelling which has thus far been largely investigated using right atrial (RA) tissue. In this study we used proteomic and phosphoproteomic analysis in order to determine any difference between the proteomes for RA and RV. Samples were therefore taken from the RA and RV of five infants (0.34 ± 0.05 years, mean ± SEM) with VSD who were undergoing cardiac surgery to repair the defect. Significant differences in protein expression between RV and RA were seen. 150 protein accession numbers were identified which were significantly lower in the atria, whereas none were significantly higher in the atria compared to the ventricle. 19 phosphorylation sites (representing 19 phosphoproteins) were also lower in RA. This work has identified differences in the proteome between RA and RV which reflect differences in contractile activity and metabolism. As such, caution should be used when drawing conclusions based on analysis of the RA and extrapolating to the hypertrophied RV. RV hypertrophy occurs in neonatal patients born with VSD. Very little is known about how the atria responds to RV hypertrophy, especially at the protein level. Access to tissue from age-matched groups of patients is very rare, and we are in the unique position of being able to get tissue from both the atria and ventricle during reparative surgery of these infants. Our findings will be beneficial to future research into heart chamber malformations in congenital heart defects. Copyright © 2018. Published by Elsevier B.V.

  4. Left cardiac isomerism in the Sonic hedgehog null mouse.

    Science.gov (United States)

    Hildreth, Victoria; Webb, Sandra; Chaudhry, Bill; Peat, Jonathan D; Phillips, Helen M; Brown, Nigel; Anderson, Robert H; Henderson, Deborah J

    2009-06-01

    Sonic hedgehog (Shh) is a secreted morphogen necessary for the production of sidedness in the developing embryo. In this study, we describe the morphology of the atrial chambers and atrioventricular junctions of the Shh null mouse heart. We demonstrate that the essential phenotypic feature is isomerism of the left atrial appendages, in combination with an atrioventricular septal defect and a common atrioventricular junction. These malformations are known to be frequent in humans with left isomerism. To confirm the presence of left isomerism, we show that Pitx2c, a recognized determinant of morphological leftness, is expressed in the Shh null mutants on both the right and left sides of the inflow region, and on both sides of the solitary arterial trunk exiting from the heart. It has been established that derivatives of the second heart field expressing Isl1 are asymmetrically distributed in the developing normal heart. We now show that this population is reduced in the hearts from the Shh null mutants, likely contributing to the defects. To distinguish the consequences of reduced contributions from the second heart field from those of left-right patterning disturbance, we disrupted the movement of second heart field cells into the heart by expressing dominant-negative Rho kinase in the population of cells expressing Isl1. This resulted in absence of the vestibular spine, and presence of atrioventricular septal defects closely resembling those seen in the hearts from the Shh null mutants. The primary atrial septum, however, was well formed, and there was no evidence of isomerism of the atrial appendages, suggesting that these features do not relate to disruption of the contributions made by the second heart field. We demonstrate, therefore, that the Shh null mouse is a model of isomerism of the left atrial appendages, and show that the recognized associated malformations found at the venous pole of the heart in the setting of left isomerism are likely to arise from

  5. Ambient Air Pollution and Traffic Exposures and Congenital Heart Defects in the San Joaquin Valley of California

    Science.gov (United States)

    Padula, Amy M.; Tager, Ira B.; Carmichael, Suzan L.; Hammond, S. Katharine; Yang, Wei; Lurmann, Frederick; Shaw, Gary M.

    2013-01-01

    Background Congenital anomalies are a leading cause of infant morbidity and mortality. Studies suggest associations between environmental contaminants and some anomalies, although evidence is limited. Methods We used data from the California Center of the National Birth Defects Prevention Study and the Children's Health and Air Pollution Study to estimate the odds of 27 congenital heart defects with respect to quartiles of 7 ambient air pollutant and traffic exposures in California during the first two months of pregnancy, 1997–2006 (N=813 cases and N=828 controls). Results Particulate matter <10 microns (PM10) was associated with pulmonary valve stenosis (aOR4th Quartile=2.6; 95% CI: 1.2, 5.7) and perimembranous ventricular septal defects (aOR3rd Quartile=2.1; 95% CI: 1.1, 3.9) after adjusting for maternal race-ethnicity, education and multivitamin use. PM2.5 was associated with transposition of the great arteries (aOR3rd Quartile=2.6; 95% CI: 1.1, 6.5) and inversely associated with perimembranous ventricular septal defects (aOR4th Quartile=0.5; 95% CI: 0.2, 0.9). Secundum atrial septal defects were inversely associated with carbon monoxide (aOR4th Quartile=0.4; 95% CI: 0.2, 0.8) and PM2.5 (aOR4th Quartile=0.5; 95% CI: 0.3, 0.8). Traffic density was associated with muscular ventricular septal defects (aOR4th Quartile=3.0, 95% CI: 1.2, 7.8) and perimembranous ventricular septal defects (aOR3rd Quartile =2.4; 95% CI: 1.3, 4.6), and inversely associated with transposition of the great arteries (aOR4th Quartile=0.3; 95% CI: 0.1, 0.8). Conclusions PM10 and traffic density may contribute to the occurrence of pulmonary valve stenosis and ventricular septal defects, respectively. The results were mixed for other pollutants and had little consistency with previous studies. PMID:23772934

  6. Ventricular Septal Defect in an Octogenarian: A Case Report of VSD Surgical Repair Concomitant with Coronary Artery Bypass and Valvular Surgery

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    Eiki Tayama

    2012-01-01

    Full Text Available Finding an untreated or asymptomatic large ventricular septal defect (VSD in an elderly patient is uncommon. The present case was an 81-year-old man who suffered from acute myocardial infarction due to three-vessel coronary disease, mitral and tricuspid valve insufficiency, and high-flow perimembranous VSD (Qp/Qs 2.3. Although the patient was elderly and the VSD had been asymptomatic for a long time, we considered that high-flow VSD and valve diseases should be repaired simultaneously with coronary disease. Then, he underwent elective surgery, namely, VSD patch repair concomitant with coronary artery bypass grafting, and mitral and tricuspid annuloplasty. His postoperative course was uneventful. We conclude that, even for an octogenarian, surgical repair of VSD is recommendable, if surgical indications are appropriate.

  7. Suture fixation of migrated septal occluder device to prevent further migration: a simple surgical technique

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    Mohite Prashant N

    2013-01-01

    Full Text Available Abstract As the use of percutaneous intervention is increasing for the closure of the atrial septal defect, the procedure related complications are also on rise, migration of the device being most common. The migrated devices with failed percutaneous retrieval must be removed surgically under cardiopulmonary bypass. During establishment of cardiopulmonary bypass, the handling of heart may cause further migration of the device into other chambers of heart which leads to difficulty in finding and retrieval of the device. The authors propose a simple and unique technique to prevent further migration of the septal occluder device.

  8. Four-dimensional ultrasonography of the fetal heart with spatiotemporal image correlation.

    Science.gov (United States)

    Gonçalves, Luís F; Lee, Wesley; Chaiworapongsa, Tinnakorn; Espinoza, Jimmy; Schoen, Mary Lou; Falkensammer, Peter; Treadwell, Marjorie; Romero, Roberto

    2003-12-01

    This study was undertaken to describe a new technique for the examination of the fetal heart using four-dimensional ultrasonography with spatiotemporal image correlation (STIC). Volume data sets of the fetal heart were acquired with a new cardiac gating technique (STIC), which uses automated transverse and longitudinal sweeps of the anterior chest wall. These volumes were obtained from 69 fetuses: 35 normal, 16 with congenital anomalies not affecting the cardiovascular system, and 18 with cardiac abnormalities. Dynamic multiplanar slicing and surface rendering of cardiac structures were performed. To illustrate the STIC technique, two representative volumes from a normal fetus were compared with volumes obtained from fetuses with the following congenital heart anomalies: atrioventricular septal defect, tricuspid stenosis, tricuspid atresia, and interrupted inferior vena cava with abnormal venous drainage. Volume datasets obtained with a transverse sweep were utilized to demonstrate the cardiac chambers, moderator band, interatrial and interventricular septae, atrioventricular valves, pulmonary veins, and outflow tracts. With the use of a reference dot to navigate the four-chamber view, intracardiac structures could be simultaneously studied in three orthogonal planes. The same volume dataset was used for surface rendering of the atrioventricular valves. The aortic and ductal arches were best visualized when the original plane of acquisition was sagittal. Volumes could be interactively manipulated to simultaneously visualize both outflow tracts, in addition to the aortic and ductal arches. Novel views of specific structures were generated. For example, the location and extent of a ventricular septal defect was imaged in a sagittal view of the interventricular septum. Furthermore, surface-rendered images of the atrioventricular valves were employed to distinguish between normal and pathologic conditions. Representative video clips were posted on the Journal's Web

  9. Detection and quantification by deformation imaging of the functional impact of septal compared to free wall preexcitation in the Wolff-Parkinson-White syndrome.

    Science.gov (United States)

    De Boeck, Bart W L; Teske, Arco J; Leenders, Geert E; Mohamed Hoesein, Firdaus A A; Loh, Peter; van Driel, Vincent J; Doevendans, Pieter A; Prinzen, Frits W; Cramer, Maarten J

    2010-08-15

    Pacing experiments in healthy animal hearts have suggested a larger detrimental effect of septal compared to free wall preexcitation. We investigated the intrinsic relation among the site of electrical preexcitation, mechanical dyssynchrony, and dysfunction in human patients. In 33 patients with Wolff-Parkinson-White (WPW) syndrome and 18 controls, regional myocardial deformation was assessed by speckle tracking mapping (ST-Map) to assess the preexcitation site, shortening sequences and dyssynchrony, and the extent of local and global ejecting shortening. The ST-Map data in patients with accessory atrioventricular pathways correctly diagnosed as located in the interventricular septum (IVS) (n = 11) or left ventricular free wall (LFW) (n = 12) were compared to the corresponding control values. A local ejecting shortening of <2 SD of the control values identified hypokinetic segments. The localization of the atrioventricular pathways by ST-Map matched with the invasive electrophysiology findings in 23 of 33 patients and was one segment different in 5 of 33 patients. In both WPW-IVS and WPW-LFW, local ejecting shortening was impaired at the preexcitation site (p <0.01). However, at similar electrical and mechanical dyssynchrony, WPW-IVS had more extensive hypokinesia than did WPW-LFW (3.6 +/- 0.9 vs 1.8 +/- 1.3 segments, p <0.01). Compared to controls, the left ventricular function was significantly reduced only in WPW-IVS (global ejecting shortening 17 +/- 2% vs 19 +/- 2%, p = 0.01; ejection fraction 55 +/- 5% vs 59 +/- 3%, p = 0.02). In conclusion, preexcitation is associated with local hypokinesia, which at comparable preexcitation is more extensive in WPW-IVS than in WPW-LFW and could adversely affect ventricular function. ST-Map might have a future role in detecting and guiding treatment of septal pathways with significant mechanical effects.

  10. Cost comparison of transcatheter and operative closures of ostium secundum atrial septal defects

    Science.gov (United States)

    O’Byrne, Michael L.; Gillespie, Matthew J.; Shinohara, Russell T.; Dori, Yoav; Rome, Jonathan J.; Glatz, Andrew C.

    2015-01-01

    Background Clinical outcomes for transcatheter and operative closures of atrial septal defects (ASDs) are similar. Economic cost for each method has not been well described. Methods A single-center retrospective cohort study of children and adults cost of operative and transcatheter closures of ASD. A propensity score weight-adjusted multivariate regression model was used in an intention-to-treat analysis. Costs for reintervention and crossover admissions were included in primary analysis. Results A total of 244 subjects were included in the study (64% transcatheter and 36% operative), of which 2% (n = 5) were ≥18 years. Crossover rate from transcatheter to operative group was 3%. Risk of reintervention (P = .66) and 30-day mortality (P = .37) were not significantly different. In a multivariate model, adjusted cost of operative closure was 2012 US $60,992 versus 2012 US $55,841 for transcatheter closure (P cost favoring transcatheter closure were length of stay, medications, and follow-up radiologic and laboratory testing, overcoming higher costs of procedure and echocardiography. Professional costs did not differ. The rate of 30-day readmission was greater in the operative cohort, further increasing the cost advantage of transcatheter closure. Sensitivity analyses demonstrated that costs of follow-up visits influenced relative cost but that device closure remained favorable over a broad range of crossover and reintervention rates. Conclusion For single secundum ASD, cost comparison analysis favors transcatheter closure over the short term. The cost of follow-up regimens influences the cost advantage of transcatheter closure. PMID:25965721

  11. Síndrome brânquio-óculo-facial (BOFS e cardiopatias congênitas Síndrome branquio óculo facial (BOFS y cardiopatías congénitas Branchio-oculo-facial syndrome (BOFS and congenital heart defects

    Directory of Open Access Journals (Sweden)

    Rafael Fabiano Machado Rosa

    2009-02-01

    and decreased deep tendon reflexes. Echocardiography showed the presence of a type-A complete atrioventricular septal defect and patent ductus arteriosus. This description strengthens the possibility of congenital heart defects being part of the spectrum of anomalies seen in BOFS.

  12. Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: A case report with review of literature

    OpenAIRE

    Nemani, Lalita; Barik, Ramachandra; Patnaik, Amar Narayana; Mishra, Ramesh C; Rao, Amaresh M; Kapur, Pragati

    2014-01-01

    We report a case of type-A Coffin-Siris syndrome (CSS) with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD), subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right vent...

  13. Unruptured Aneurysm of Sinus of Valsalva Coexisting with the Large Ventricular Septal Defect and Severe Aortic Regurgitation in a Young Man

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    Pouya Nezafati

    2015-01-01

    Full Text Available Introduction. Unruptured sinus of valsalva aneurysm (SVA is a rare congenital anomaly, particularly, when it coexists with a ventricular septal defect (VSD and aortic regurgitation due to the prolapse of the elongated aortic cusp into the VSD. In this report, we present the case of a 19-year-old young man with VSD challenging in spite of dyspnea and lower limb edema. Presentation of Case. Its diagnosis was made on the basis of transthoracic echocardiography results. Surgical management consisted of replacing the SVA with mechanical valve prosthesis. A Gore-Tex patch repaired the VSD. Discussion. In the follow-up periods, clinical and echocardiographic tests showed that the patient was in excellent status. Conclusion. SVA requires a surgical procedure due to its high risk of mortality in unoperated patients and a good safety of surgery.

  14. Combining rhinoplasty with septal perforation repair.

    Science.gov (United States)

    Foda, Hossam M T; Magdy, Emad A

    2006-11-01

    A combined septal perforation repair and rhinoplasty was performed in 80 patients presenting with septal perforations (size 1 to 5 cm) and external nasal deformities. The external rhinoplasty approach was used for all cases and the perforation was repaired using bilateral intranasal mucosal advancement flaps with a connective tissue interposition graft in between. Complete closure of the perforation was achieved in 90% of perforations of size up to 3.5 cm and in only 70% of perforations that were larger than 3.5 cm. Cosmetically, 95% were very satisfied with their aesthetic result. The external rhinoplasty approach proved to be very helpful in the process of septal perforation repair especially in large and posteriorly located perforations and in cases where the caudal septal cartilage was previously resected. Our results show that septal perforation repair can be safely combined with rhinoplasty and that some of the routine rhinoplasty maneuvers, such as medial osteotomies and dorsal lowering, could even facilitate the process of septal perforation repair.

  15. Closure of large patent ductus arteriosus using the Amplatzer Septal Occluder.

    Science.gov (United States)

    García-Montes, José A; Camacho-Castro, Anahí; Sandoval-Jones, Juan P; Buendía-Hernández, Alfonso; Calderón-Colmenero, Juan; Patiño-Bahena, Emilia; Zabal, Carlos

    2015-03-01

    Percutaneous closure of patent ductus arteriosus has become the treatment of choice in many centres. In patients with large ducts and pulmonary hypertension, transcatheter closure has been achieved with success using the Amplatzer Duct Occluder or even the Amplatzer Muscular Ventricular Septal Defect Occluder. We present a series of 17 patients with large and hypertensive ductus arteriosus who were treated with an Amplatzer Septal Occluder. The group had 11 female patients (64.7%) and a mean age of 18.6±12.1 years. The haemodynamic and anatomical data are as follows: pulmonary artery systolic pressure 71.3±31.8 mmHg, pulmonary to systemic flow ratio 3.14±1.36, ductal diameter at the pulmonary end 12.5±3.8 mm, and at the aortic end 20.2±7.7 mm; 14 cases (82.3%) had type A ducts. In 11 patients, we began the procedure using a different device - six with duct occluder and five with ventricular septal occluder - and it was changed because of device embolisation in six (35.3%). All septal occluders were delivered successfully. Residual shunt was moderate in six patients (35.3%), mild in eight (47%), trivial in two (11.8%), and no shunt in one (5.9%). Pulmonary systolic pressure decreased to 48.9±10.8 mmHg after occlusion (p=0.0015). Follow-up in 15 patients (88.2%) for 28.4±14.4 months showed complete closure in all cases but one, and continuous decrease of the pulmonary systolic pressure to 31.4±10.5 mmHg. No complications at follow-up have been reported. The Amplatzer Septal Occluder is a good alternative to percutaneously treat large and hypertensive ductus arteriosus.

  16. Pre-operative evaluation with MR in tetralogy of Fallot and pulmonary atresia with ventricular septal defect

    International Nuclear Information System (INIS)

    Holmqvist, C.; Hochbergs, P.; Bjoerkhem, G.; Brockstedt, S.; Laurin, S.

    2000-01-01

    To assess whether MR imaging could replace angiography in pre-operative evaluation of patients with tetralogy of Fallot and pulmonary atresia with ventricular septal defect (VSD), especially since the surgical correction was done earlier than was previously the rule. Fourteen patients with tetralogy of Fallot (n=10) or pulmonary atresia with VSD (n=4), mean age 7.5±4.4 months, were evaluated with angiocardiography and MR before definitive surgical correction. There was good diagnostic agreement between the two modalities when evaluating right ventricular outflow obstruction; 86% for valvular and 93% for supra valvular stenosis, but the agreement was somewhat lower for the sub valvular obstruction (57%). Surgery findings, however, were in favour of MR in 5 patients concerning the sub valvular right ventricular outflow tract obstruction. MR images identified all stenoses in the right and left pulmonary arteries, but overlooked one stenosis in the main pulmonary artery. MR could evaluate patency in all palliative shunts. Even in this young age group, MR imaging offers a good alternative to angiocardiography for the pre-operative evaluation of the right ventricular outflow tract, the main pulmonary artery and the proximal right and left pulmonary arteries, before definitive surgical correction of tetralogy of Fallot and pulmonary atresia with VSD

  17. Pre-operative evaluation with MR in tetralogy of Fallot and pulmonary atresia with ventricular septal defect

    Energy Technology Data Exchange (ETDEWEB)

    Holmqvist, C.; Hochbergs, P. [Univ. Hospital, Lund (Sweden). Dept of Diagnostic Radiology; Bjoerkhem, G. [Univ. Hospital, Lund (Sweden). Dept of Paediatrics; Brockstedt, S.; Laurin, S. [Univ. Hospital, Lund (Sweden). Dept of Diagnostic Radiology

    2000-01-01

    To assess whether MR imaging could replace angiography in pre-operative evaluation of patients with tetralogy of Fallot and pulmonary atresia with ventricular septal defect (VSD), especially since the surgical correction was done earlier than was previously the rule. Fourteen patients with tetralogy of Fallot (n=10) or pulmonary atresia with VSD (n=4), mean age 7.5{+-}4.4 months, were evaluated with angiocardiography and MR before definitive surgical correction. There was good diagnostic agreement between the two modalities when evaluating right ventricular outflow obstruction; 86% for valvular and 93% for supra valvular stenosis, but the agreement was somewhat lower for the sub valvular obstruction (57%). Surgery findings, however, were in favour of MR in 5 patients concerning the sub valvular right ventricular outflow tract obstruction. MR images identified all stenoses in the right and left pulmonary arteries, but overlooked one stenosis in the main pulmonary artery. MR could evaluate patency in all palliative shunts. Even in this young age group, MR imaging offers a good alternative to angiocardiography for the pre-operative evaluation of the right ventricular outflow tract, the main pulmonary artery and the proximal right and left pulmonary arteries, before definitive surgical correction of tetralogy of Fallot and pulmonary atresia with VSD.

  18. Physiological differences between various types of Eisenmenger syndrome and relation to outcome.

    Science.gov (United States)

    Moceri, Pamela; Kempny, Aleksander; Liodakis, Emmanouil; Alonso Gonzales, Rafael; Germanakis, Ioannis; Diller, Gerhard-Paul; Swan, Lorna; Marino, Philip S; Wort, Stephen J; Babu-Narayan, Sonya V; Ferrari, Emile; Gatzoulis, Michael A; Li, Wei; Dimopoulos, Konstantinos

    2015-01-20

    Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) related to congenital heart disease. Several studies have suggested that the presence and location of the shunt defines the natural history of these patients by influencing right ventricular adaptation to PAH. We aimed to echocardiographically assess differences in cardiac physiology and outcome between various types of ES. In this longitudinal cohort study, 191 patients with ES and non-complex congenital heart disease were recruited, 36 with pre-tricuspid and 155 with post-tricuspid shunts. Patients with pre-tricuspid shunts were older, had higher BNP concentrations and lower exercise tolerance compared to patients with post-tricuspid shunts. Right ventricular (RV) function was impaired in patients with atrial septal defects, with larger right ventricles, impaired systolic function and adaptation. The left ventricular eccentricity index was significantly higher in pre-tricuspid defects. Within post-tricuspid shunts, patients with atrio-ventricular septal defects had better right ventricular function compared to ventricular septal defects, while in those with a patent ductus arteriosus this was worse. There was a trend towards lower mortality in patients with post versus pre-tricuspid shunts, which was significant for patients above the age of 48 years. The presence of a post-tricuspid shunt appears to carry physiological and possibly prognostic benefits in ES compared to patients with pre-tricuspid shunts. This should be borne in mind when management decisions and advanced therapies are considered. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  19. Headache in 25 consecutive patients with atrial septal defects before and after percutaneous closure--a prospective case series.

    Science.gov (United States)

    Riederer, Franz; Baumgartner, Helmut; Sándor, Peter S; Wessely, Peter; Wöber, Christian

    2011-09-01

    In contrast to patent foramen ovale that is highly prevalent in the general population, atrial septal defect (ASD) is a rare congenital heart defect. The effect of ASD closure on headache and migraine remains a matter of controversy. The objectives of our study were (1) to determine headache prevalence in consecutive patients with ASD scheduled for percutaneous closure for cardiologic indications, using the International Classification of Headache Disorders and (2) to compare headache characteristics before and after closure of ASD. In this observational case series no a priori power analysis was performed. Twenty-five consecutive patients were prospectively included over 27 months. Median duration of follow-up was 12 months [interquartile range 0]. Prevalence of active headache seemed to be higher compared with the general population: any headaches 88% (95% confidence interval 70-96), migraine without aura 28% (14-48), migraine with aura 16% (6-35). After ASD closure, we observed a slightly lower headache frequency (median frequency 1.0 [2.6] vs. 0.3 [1.5] headaches per month; P = .067). In patients with ongoing headaches, a significant decrease in headache intensity (median VAS 7 [3] vs. 5 [4]; P = .036) was reported. Three patients reporting migraine with aura before the intervention noted no migraine with aura attacks at follow-up, 2 of them reported ongoing tension-type headache, 1 migraine without aura. In summary, this prospective observational study confirms the high prevalence of headache, particularly migraine, in ASD patients and suggests a possible small beneficial effect of ASD closure. © 2011 American Headache Society.

  20. Swallow syncope caused by third-degree atrioventricular block

    DEFF Research Database (Denmark)

    Roust Aaberg, Anne Marie; Eriksson, Anna Elin; Madsen, Per Lav

    2015-01-01

    We report a case of a patient with more than 30 years of repeated syncopes, always following food intake. The patient was diagnosed with a swallow-related third-degree atrioventricular block and successfully treated with an artificial pacemaker.......We report a case of a patient with more than 30 years of repeated syncopes, always following food intake. The patient was diagnosed with a swallow-related third-degree atrioventricular block and successfully treated with an artificial pacemaker....

  1. Congenital heart defects in molecularly proven Kabuki syndrome patients.

    Science.gov (United States)

    Digilio, Maria Cristina; Gnazzo, Maria; Lepri, Francesca; Dentici, Maria Lisa; Pisaneschi, Elisa; Baban, Anwar; Passarelli, Chiara; Capolino, Rossella; Angioni, Adriano; Novelli, Antonio; Marino, Bruno; Dallapiccola, Bruno

    2017-11-01

    The prevalence of congenital heart defects (CHD) in Kabuki syndrome ranges from 28% to 80%. Between January 2012 and December 2015, 28 patients had a molecularly proven diagnosis of Kabuki syndrome. Pathogenic variants in KMT2D (MLL2) were detected in 27 patients, and in KDM6A gene in one. CHD was diagnosed in 19/27 (70%) patients with KMT2D (MLL2) variant, while the single patient with KDM6A change had a normal heart. The anatomic types among patients with CHD included aortic coarctation (4/19 = 21%) alone or associated with an additional CHD, bicuspid aortic valve (4/19 = 21%) alone or associated with an additional CHD, perimembranous subaortic ventricular septal defect (3/19 = 16%), atrial septal defect ostium secundum type (3/19 = 16%), conotruncal heart defects (3/19 = 16%). Additional CHDs diagnosed in single patients included aortic dilatation with mitral anomaly and hypoplastic left heart syndrome. We also reviewed CHDs in patients with a molecular diagnosis of Kabuki syndrome reported in the literature. In conclusion, a CHD is detected in 70% of patients with KMT2D (MLL2) pathogenic variants, most commonly left-sided obstructive lesions, including multiple left-sided obstructions similar to those observed in the spectrum of the Shone complex, and septal defects. Clinical management of Kabuki syndrome should include echocardiogram at the time of diagnosis, with particular attention to left-sided obstructive lesions and mitral anomalies, and annual monitoring for aortic arch dilatation. © 2017 Wiley Periodicals, Inc.

  2. Fatal Lyme carditis and endodermal heterotopia of the atrioventricular node.

    Science.gov (United States)

    Cary, N. R.; Fox, B.; Wright, D. J.; Cutler, S. J.; Shapiro, L. M.; Grace, A. A.

    1990-01-01

    A fatal case of Lyme carditis occurring in a Suffolk farmworker is reported. Post-mortem examination of the heart showed pericarditis, focal myocarditis and prominent endocardial and interstitial fibrosis. The additional finding of endodermal heterotopia ('mesothelioma') of the atrioventricular node raises the possibility that this could also be related to Lyme infection and account for the relatively frequent occurrence of atrioventricular block in this condition. Lyme disease should always be considered in a case of atrioventricular block, particularly in a young patient from a rural area. The heart block tends to improve and therefore only temporary pacing may be required. Images Figure 1 Figure 2 Figure 3 PMID:2349186

  3. Bone recycling in nasal septal reconstruction.

    Science.gov (United States)

    Apaydin, Fazil

    2013-12-01

    Septal reconstruction alone or together with rhinoplasty can be a very challenging operation. In situations where septal cartilage is used for grafting or is not enough, bony implants taken from the perpendicular plate of the ethmoid and vomer can be used as a filler material between the mucoperichondrial flaps to avoid from unwanted mucosal atrophy, flapping, and septal perforation. These bony implants can also be used for splinting the dorsal and/or caudal segment of the septal cartilage after reshaping by rongeurs. On rare occasions, they can even be used for subtotal reconstruction of the septum. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  4. New pulmonary vein Doppler echocardiographic index predicts significant interatrial shunting in secundum atrial septal defect.

    Science.gov (United States)

    Lam, Yat-Yin; Fang, Fang; Yip, Gabriel Wai-Kwok; Li, Zhi-An; Yang, Ya; Yu, Cheuk-Man

    2012-09-20

    The relation between pulmonary venous flow (PVF) pattern and degree of left-to-right interatrial shunting (IAS) in patients with secundum atrial septal defect (ASD) is unknown. Fifty consecutive ASD patients (14 males, 36 ± 17 years) received transthoracic echocardiography (TTE) before and 1 day after transcatheter closure and their results were compared to 40 controls. The ratio of pulmonary-to-systemic flows (Qp/Qs) was assessed by TTE and invasive oximetry. Pre-closure PV systolic (PVs), diastolic (PVd) velocities and velocity-time integral (PV-VTI) increased, time from onset of ECG Q-wave to the peak PV diastolic wave (Q-PVd) shortened and atrial reversal (PVar) velocity significantly decreased as compared to normals. These findings normalized after closure. Patients with large IAS (defined as invasive Qp/Qs ≥ 2) had higher PVs, PVd and PV-VTI, shorter Q-PVd but lower PVar (all pIAS. Invasive Qp/Qs ratios correlated with PVs, PVd, PV-VTI, Q-PVd and TTE-derived Qp/Qs ratios, ASD sizes and RV end-diastolic dimensions (all pIAS after multivariate analysis. The corresponding sensitivity, specificity and AUC were 89%, 82% and 0.90 respectively for a PV-VTI of 30 cm (pIAS have distinguishable PVF features. Doppler evaluation of PV-VTI is a novel additional tool for assessing the magnitude of shunting in these patients non-invasively. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

  5. Surgical ablation of ventricular tachycardia secondary to congenital ventricular septal aneurysm.

    Science.gov (United States)

    Graffigna, A; Minzioni, G; Ressia, L; Vigano, M

    1994-04-01

    Three patients underwent surgical ablation for ventricular tachycardia resulting from an aneurysm of the membranous portion of the ventricular septum. Two patients had a definite history of cardiac murmur during infancy, and one of them was found at the time of operation to have a left-to-right shunt through the apex of the aneurysm. The earliest ventricular activation sites were located around the neck of the aneurysm and were ablated in 1 patient by encircling the endocardial ventriculotomy and by cryoablation in the remaining 2. After focus resection had been completed, aneurysm resection and ventricular septal reconstruction were performed. All patients were alive and free of ventricular tachycardia and did not need medication as of 61, 66, and 88 months postoperatively. Spontaneous closure of a ventricular septal defect may lead to the formation of an aneurysm in the ventricular septum that may sustain ventricular tachycardias. Such arrhythmias can be effectively treated using electrically guided surgical techniques.

  6. An exploratory analysis of the relationship between ambient ozone and particulate matter concentrations during early pregnancy and selected birth defects in Texas

    International Nuclear Information System (INIS)

    Vinikoor-Imler, Lisa C.; Stewart, Thomas G.; Luben, Thomas J.; Davis, J. Allen; Langlois, Peter H.

    2015-01-01

    We performed an exploratory analysis of ozone (O 3 ) and fine particulate matter (PM 2.5 ) concentrations during early pregnancy and multiple types of birth defects. Data on births were obtained from the Texas Birth Defects Registry (TBDR) and the National Birth Defects Prevention Study (NBDPS) in Texas. Air pollution concentrations were previously determined by combining modeled air pollution concentrations with air monitoring data. The analysis generated hypotheses for future, confirmatory studies; although many of the observed associations were null. The hypotheses are provided by an observed association between O 3 and craniosynostosis and inverse associations between PM 2.5 and septal and obstructive heart defects in the TBDR. Associations with PM 2.5 for septal heart defects and ventricular outflow tract obstructions were null using the NBDPS. Both the TBDR and the NBPDS had inverse associations between O 3 and septal heart defects. Further research to confirm the observed associations is warranted. - Highlights: • Air pollution concentrations combined modeled air data and air monitoring data. • No associations were observed between the majority of birth defects and PM 2.5 and O 3 . • Estimated associations between PM 2.5 and certain heart defects varied by dataset. • Results were suggestive of an inverse association between O 3 and septal heart defects. • Higher O 3 concentrations may be associated with increased odds of craniosynostosis. - Although most observed associations between ozone and fine particulate matter concentrations and birth defects were null, some were present and warrant further consideration

  7. Tibial and fibular developmental fields defects

    International Nuclear Information System (INIS)

    Khoury, N.J.; Haddad, M.C.; Hourani, M.H.

    1999-01-01

    Malformations of the lower limbs are rare and heterogeneous anomalies. To explain the diversity and complexity of these abnormalities, authors introduced the concept of tibial and fibular developmental fields. Defects in these fields are responsible for different malformations, which have been described, to our knowledge, in only one report in the radiology literature. We present a case of a newborn with femoral bifurcation, absent fibulae and talar bones, ankle and foot malformations, and associated atrial septal defect. Our case is an example of defects in both fibular and tibial developmental fields. (orig.)

  8. Long-term Associations of an Early Corrected Ventricular Septal Defect and Stress Systems of Child and Mother at Primary School Age.

    Science.gov (United States)

    Stonawski, Valeska; Vollmer, Laura; Köhler-Jonas, Nicola; Rohleder, Nicolas; Golub, Yulia; Purbojo, Ariawan; Moll, Gunther H; Heinrich, Hartmut; Cesnjevar, Robert A; Kratz, Oliver; Eichler, Anna

    2017-01-01

    Ventricular septal defect (VSD) is the most common congenital heart defect, with larger VSDs typically being corrected with an open-heart surgery during infancy. Long-term consequences of a VSD-corrective surgery on stress systems of child and mother are still unknown. The aim of the present study is to investigate the associations of an early corrected VSD and diurnal cortisol release of child and mother. 26 children (12 boys) between 6 and 9 years old, who underwent surgery for an isolated VSD within the first 3 years of life, and their mothers participated in the study. Their diurnal cortisol profiles were compared to a sex-, age-, and socioeconomic status-matched healthy control group. Within the VSD group, associations between cortisol and characteristics of surgery and hospitalization were investigated. Child and mother psychopathological symptoms were considered as a possible interfering mechanism of altered cortisol profiles. Diurnal cortisol profiles of children with an early corrected VSD did not differ from those of controls. However, mothers of affected children exhibited higher cortisol levels in the morning ( p  early corrected VSD, in terms of comparable diurnal cortisol profiles with healthy controls, according to a comparable mother-rated psychopathology. Mothers of affected children reveal altered diurnal cortisol levels, without differences in self-rated psychopathology. This divergence should be clarified in future research.

  9. Does the atrioventricular node conduct?

    NARCIS (Netherlands)

    Meijler, F.L.; Fisch, C.

    1989-01-01

    It is difficult to be certain wh en the term "conduction" was first applied to the transfer of atrial activation to the ventricles .' In 1894, Engelmann used the word "Leitung", which can be translated as "connection" or as "conduction" .2 In 1906, Tawara described the atrioventricular node,

  10. [Percutaneous closure of ductus arteriosus and muscular ventricular defect with amplatzer occluder in a patient with severe pulmonary hypertension].

    Science.gov (United States)

    García-Montes, José Antonio; Zabal Cerdeira, Carlos; Calderón-Colmenero, Juan; Espínola, Nilda; Fernández de la Reguera, Guillermo; Buendía Hernández, Alfonso

    2005-01-01

    Surgical treatment of multiple muscular ventricular septal defects with associated lesions and severe pulmonary hypertension has a high morbility and mortality. Closure of these defects by the Amplatzer muscular VSD occluder is an alternative to surgery, avoiding the need of cardiopulmonary bypass. We present the case of a 38 year-old woman with signs of heart failure in NYHA functional class IV, with two muscular ventricular septal defects, patent ductus arteriosus and severe pulmonary hypertension, that were treated with three Amplatzer muscular VSD occluders, with significant reduction of pulmonary pressure and functional class improvement.

  11. Virtual reality 3D echocardiography in the assessment of tricuspid valve function after surgical closure of ventricular septal defect

    Directory of Open Access Journals (Sweden)

    Kappetein A Pieter

    2007-02-01

    Full Text Available Abstract Background This study was done to investigate the potential additional role of virtual reality, using three-dimensional (3D echocardiographic holograms, in the postoperative assessment of tricuspid valve function after surgical closure of ventricular septal defect (VSD. Methods 12 data sets from intraoperative epicardial echocardiographic studies in 5 operations (patient age at operation 3 weeks to 4 years and bodyweight at operation 3.8 to 17.2 kg after surgical closure of VSD were included in the study. The data sets were analysed as two-dimensional (2D images on the screen of the ultrasound system as well as holograms in an I-space virtual reality (VR system. The 2D images were assessed for tricuspid valve function. In the I-Space, a 6 degrees-of-freedom controller was used to create the necessary projectory positions and cutting planes in the hologram. The holograms were used for additional assessment of tricuspid valve leaflet mobility. Results All data sets could be used for 2D as well as holographic analysis. In all data sets the area of interest could be identified. The 2D analysis showed no tricuspid valve stenosis or regurgitation. Leaflet mobility was considered normal. In the virtual reality of the I-Space, all data sets allowed to assess the tricuspid leaflet level in a single holographic representation. In 3 holograms the septal leaflet showed restricted mobility that was not appreciated in the 2D echocardiogram. In 4 data sets the posterior leaflet and the tricuspid papillary apparatus were not completely included. Conclusion This report shows that dynamic holographic imaging of intraoperative postoperative echocardiographic data regarding tricuspid valve function after VSD closure is feasible. Holographic analysis allows for additional tricuspid valve leaflet mobility analysis. The large size of the probe, in relation to small size of the patient, may preclude a complete data set. At the moment the requirement of an I

  12. Complete atrioventricular block in acute coronary syndrome: prevalence, characterisation and implication on outcome.

    Science.gov (United States)

    Aguiar Rosa, Silvia; Timóteo, Ana Teresa; Ferreira, Lurdes; Carvalho, Ramiro; Oliveira, Mario; Cunha, Pedro; Viveiros Monteiro, André; Portugal, Guilherme; Almeida Morais, Luis; Daniel, Pedro; Cruz Ferreira, Rui

    2018-04-01

    The aim was to characterise acute coronary syndrome patients with complete atrioventricular block and to assess the effect on outcome. Patients admitted with acute coronary syndrome were divided according to the presence of complete atrioventricular block: group 1, with complete atrioventricular block; group 2, without complete atrioventricular block. Clinical, electrocardiographic and echocardiographic characteristics and prognosis during one year follow-up were compared between the groups. Among 4799 acute coronary syndrome patients admitted during the study period, 91 (1.9%) presented with complete atrioventricular block. At presentation, group 1 patients presented with lower systolic blood pressure, higher Killip class and incidence of syncope. In group 1, 86.8% presented with ST-segment elevation myocardial infarction (STEMI), and inferior STEMI was verified in 79.1% of patients in group 1 compared with 21.9% in group 2 ( Pblock was observed in 7.3% in contrast to 2.5% in patients submitted to primary percutaneous coronary intervention ( Pblock was an independent predictor of hospital mortality (odds ratio 3.671; P=0.045). There was no significant difference in mortality at one-year follow-up between the study groups. Complete atrioventricular block conferred a worse outcome during hospitalisation, including a higher incidence of cardiogenic shock, ventricular arrhythmias and death.

  13. Minimally invasive or interventional repair of atrial septal defects in children: experience in 171 cases and comparison with conventional strategies.

    Science.gov (United States)

    Formigari, R; Di Donato, R M; Mazzera, E; Carotti, A; Rinelli, G; Parisi, F; Pasquini, L; Ballerini, L

    2001-05-01

    The goal of this study was to evaluate percutaneous interventional and minimally invasive surgical closure of secundum atrial septal defect (ASD) in children. Concern has surrounded abandoning conventional midline sternotomy in favor of the less invasive approaches pursuing a better cosmetic result and a more rational resource utilization. A retrospective analysis was performed on the patients treated from June 1996 to December 1998. One hundred seventy-one children (median age 5.8 years, median weight 22.1 kg) underwent 52 device implants, 72 minimally invasive surgical operations and 50 conventional sternotomy operations. There were no deaths and no residual left to right shunt in any of the groups. The overall complication rate causing delayed discharge was 12.6% for minimally invasive surgery, 12.0% for midline sternotomy and 3.8% for transcatheter device closure (p appeal of the percutaneous and minimally invasive approaches must be weighed against their greater exposure to technical pitfalls. Adequate training is needed if a strategy of surgical or percutaneous minimally invasive closure of ASD in children is planned in place of conventional surgery.

  14. Incremental cost-effectiveness of percutaneous versus surgical closure of atrial septal defects in children under a public health system perspective in Brazil.

    Science.gov (United States)

    Costa, Rodrigo; Pedra, Carlos A C; Ribeiro, Marcelo; Pedra, Simone; Ferreira-Da-Silva, André Luis; Polanczyk, Carisi; Berwanger, Otávio; Biasi, Alexandre; Ribeiro, Rodrigo

    2014-11-01

    Cost-effectiveness (CE) studies of percutaneous (PC) versus surgical (SC) atrial septal defect closure are lacking. A systematic literature review in children and a CE analysis based on a model of long-term outcomes were performed. Direct costs of PC and SC were US$8700 (defined arbitrarily) and US$5700 (actually paid), respectively. Three-times the Brazilian GDI (US$28,700) per year of life saved (with a discount rate of 5%) was used as a limit for willingness-to-pay. PC had a high (US$104,500) incremental CE ratio despite lower complication rates, shorter hospital stay and better (nonsignificant) adjusted life expectancy. PC would be cost-effective if it cost US$6400 or SC had an 8% loss of utility or its indirect costs were US$2250. Costs of PC should be reduced to be cost-effective in the Brazilian public health system. Indirect costs and impact on quality of life should be further assessed.

  15. Characterization of SMAD3 Gene Variants for Possible Roles in Ventricular Septal Defects and Other Congenital Heart Diseases.

    Directory of Open Access Journals (Sweden)

    Fei-Feng Li

    Full Text Available Nodal/TGF signaling pathway has an important effect at early stages of differentiation of human embryonic stem cells in directing them to develop into different embryonic lineages. SMAD3 is a key intracellular messenger regulating factor in the Nodal/TGF signaling pathway, playing important roles in embryonic and, particularly, cardiovascular system development. The aim of this work was to find evidence on whether SMAD3 variations might be associated with ventricular septal defects (VSD or other congenital heart diseases (CHD.We sequenced the SMAD3 gene for 372 Chinese Han CHD patients including 176 VSD patients and evaluated SNP rs2289263, which is located before the 5'UTR sequence of the gene. The statistical analyses were conducted using Chi-Square Tests as implemented in SPSS (version 13.0. The Hardy-Weinberg equilibrium test of the population was carried out using the online software OEGE.Three heterozygous variants in SMAD3 gene, rs2289263, rs35874463 and rs17228212, were identified. Statistical analyses showed that the rs2289263 variant located before the 5'UTR sequence of SMAD3 gene was associated with the risk of VSD (P value=0.013 <0.05.The SNP rs2289263 in the SMAD3 gene is associated with VSD in Chinese Han populations.

  16. Bronchial compression in an infant with isolated secundum atrial septal defect associated with severe pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Sung-Hee Park

    2012-08-01

    Full Text Available Symptomatic pulmonary arterial hypertension (PAH in patients with isolated atrial septal defect (ASD is rare during infancy. We report a case of isolated ASD with severe PAH in an infant who developed airway obstruction as cardiomegaly progressed. The patient presented with recurrent severe respiratory insufficiency and failure to thrive before the repair of the ASD. Echocardiography confirmed volume overload on the right side of heart and severe PAH (tricuspid regurgitation [TR] with a peak pressure gradient of 55 to 60 mmHg. The chest radiographs demonstrated severe collapse of both lung fields, and a computed tomography scan showed narrowing of the main bronchus because of an intrinsic cause, as well as a dilated pulmonary artery compressing the main bronchus on the left and the intermediate bronchus on the right. ASD patch closure was performed when the infant was 8 months old. After the repair of the ASD, echocardiography showed improvement of PAH (TR with a peak pressure gradient of 22 to 26 mmHg, and the patient has not developed recurrent respiratory infections while showing successful catch-up growth. In infants with symptomatic isolated ASD, especially in those with respiratory insufficiency associated with severe PAH, extrinsic airway compression should be considered. Correcting any congenital heart diseases in these patients may improve their symptoms.

  17. Congestive Heart Failure Leads to Prolongation of the PR Interval and Atrioventricular Junction Enlargement and Ion Channel Remodelling in the Rabbit

    Science.gov (United States)

    Nikolaidou, Theodora; Cai, Xue J.; Stephenson, Robert S.; Yanni, Joseph; Lowe, Tristan; Atkinson, Andrew J.; Jones, Caroline B.; Sardar, Rida; Corno, Antonio F.; Dobrzynski, Halina; Withers, Philip J.; Jarvis, Jonathan C.; Hart, George; Boyett, Mark R.

    2015-01-01

    Heart failure is a major killer worldwide. Atrioventricular conduction block is common in heart failure; it is associated with worse outcomes and can lead to syncope and bradycardic death. We examine the effect of heart failure on anatomical and ion channel remodelling in the rabbit atrioventricular junction (AVJ). Heart failure was induced in New Zealand rabbits by disruption of the aortic valve and banding of the abdominal aorta resulting in volume and pressure overload. Laser micro-dissection and real-time polymerase chain reaction (RT-PCR) were employed to investigate the effects of heart failure on ion channel remodelling in four regions of the rabbit AVJ and in septal tissues. Investigation of the AVJ anatomy was performed using micro-computed tomography (micro-CT). Heart failure animals developed first degree heart block. Heart failure caused ventricular myocardial volume increase with a 35% elongation of the AVJ. There was downregulation of HCN1 and Cx43 mRNA transcripts across all regions and downregulation of Cav1.3 in the transitional tissue. Cx40 mRNA was significantly downregulated in the atrial septum and AVJ tissues but not in the ventricular septum. mRNA abundance for ANP, CLCN2 and Navβ1 was increased with heart failure; Nav1.1 was increased in the inferior nodal extension/compact node area. Heart failure in the rabbit leads to prolongation of the PR interval and this is accompanied by downregulation of HCN1, Cav1.3, Cx40 and Cx43 mRNAs and anatomical enlargement of the entire heart and AVJ. PMID:26509807

  18. Congestive Heart Failure Leads to Prolongation of the PR Interval and Atrioventricular Junction Enlargement and Ion Channel Remodelling in the Rabbit.

    Directory of Open Access Journals (Sweden)

    Theodora Nikolaidou

    Full Text Available Heart failure is a major killer worldwide. Atrioventricular conduction block is common in heart failure; it is associated with worse outcomes and can lead to syncope and bradycardic death. We examine the effect of heart failure on anatomical and ion channel remodelling in the rabbit atrioventricular junction (AVJ. Heart failure was induced in New Zealand rabbits by disruption of the aortic valve and banding of the abdominal aorta resulting in volume and pressure overload. Laser micro-dissection and real-time polymerase chain reaction (RT-PCR were employed to investigate the effects of heart failure on ion channel remodelling in four regions of the rabbit AVJ and in septal tissues. Investigation of the AVJ anatomy was performed using micro-computed tomography (micro-CT. Heart failure animals developed first degree heart block. Heart failure caused ventricular myocardial volume increase with a 35% elongation of the AVJ. There was downregulation of HCN1 and Cx43 mRNA transcripts across all regions and downregulation of Cav1.3 in the transitional tissue. Cx40 mRNA was significantly downregulated in the atrial septum and AVJ tissues but not in the ventricular septum. mRNA abundance for ANP, CLCN2 and Navβ1 was increased with heart failure; Nav1.1 was increased in the inferior nodal extension/compact node area. Heart failure in the rabbit leads to prolongation of the PR interval and this is accompanied by downregulation of HCN1, Cav1.3, Cx40 and Cx43 mRNAs and anatomical enlargement of the entire heart and AVJ.

  19. Transthoracic device closure of ventricular septal defects without cardiopulmonary bypass: experience in infants weighting less than 8 kg.

    Science.gov (United States)

    Xing, Quansheng; Wu, Qin; Pan, Silin; Ren, Yueyi; Wan, Hao

    2011-09-01

    Both surgical and percutaneous device closure of ventricular septal defect (VSD) have drawbacks and limitations in infants. We report our experiences and midterm results of transthoracic device closure of VSDs (TDCVs) without cardiopulmonary bypass (CPB) in infants. Between September 2007 and September 2009, 32 patients, with a mean age of 7.2 ± 4.7 months, body weight of 6.8 ± 2.8 kg, underwent this procedure. The procedure was performed in the operating room. A small subxiphoid incision was made. A purse-string suture was placed on the right ventricular free wall. The free wall was punctured using a trocar, then a guide wire was inserted and advanced to cross the VSD into the left ventricle under transesophageal echocardiographic guidance. A modified delivery sheath was then introduced over the guide wire. The device was delivered and deployed in position along the sheath to close the defect. A total of 30 cases (94%) were successfully closed, and the remaining two cases (6%) were converted to open heart repair. No patients received transfusion. There was no perioperative mortality, or any major complication. The mean size of the devices was 7.6 ± 3.4mm. The total operative time was less than 60 min, and the mean time for device implantation was 18.3 ± 9.4 min. All patients were extubated within 2h, and were discharged within 5 days after operation. The follow-up period ranged from 6 to 31 months (18.3 ± 9.6 months). There was no late major complication detected. Minimally invasive TDCV without CPB is a safe and effective alternative to the conventional operation in low-body-weight infants. Copyright © 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

  20. Síndrome de Wolff-Parkinson-White associada a comunicação interatrial tipo seio venoso Wolff-Parkinson-White syndrome and the sinus venosus atrial septal defect association

    Directory of Open Access Journals (Sweden)

    Patrícia Lopes Moraes

    2005-02-01

    Full Text Available A associação de comunicação interatrial (CIA tipo seio venoso com síndrome de Wolff Parkinson White (WPW é muito rara e ainda não descrita na literatura médica especializada. Descreve-se o caso de uma jovem portadora dessa associação de patologias tratada com ablação da via acessória por radiofreqüência, seguida de correção cirúrgica do defeito do septo interatrial.The Wolff-Parkinson-White syndrome (WPW and sinus venosus atrial septal defect (ASD association is very rare and not yet reported in the literature. It is the main basis for this case report.

  1. Swiss cheese ventricular septal defect with myocarditis - A rare coexistence in a neonate

    Directory of Open Access Journals (Sweden)

    A R Saboo

    2012-01-01

    Full Text Available Myocarditis is defined as acute inflammation of the myocardium, usually following a non-specific flu-like illness, and encompasses a wide range of clinical presentations ranging from mild or subclinical disease to heart failure. We report a 12-day-old healthy full-term neonate who presented with abrupt onset of congestive cardiac failure (CCF following a viral prodrome. Examination revealed persistent sinus tachycardia, lymphocytosis, gross cardiomegaly, nonspecific electrocardiogram changes with echocardiography showing Swiss cheese ventricular septal defect (VSD. VSD alone very rarely presents as early-onset cardiac failure in the absence of other precipitating factors like anemia, sepsis, hypoglycemia etc. Myocarditis, however, can mimic VSD and can present as fulminant cardiac failure in an otherwise healthy newborn. Myocarditis is usually diagnosed based on circumstantial evidence such as a recent viral infection and the sudden onset of cardiac dysfunction while ruling out other diagnostic possibilities. Elevated troponin T level is one of the most crucial noninvasive diagnostic modalities. Several trials have concluded that levels >0.055 ng/ml are statistically significant for diagnosing myocarditis in children. In our case an abrupt onset of cardiac failure following a viral prodrome and markedly elevated cardiac troponin T without sepsis and in the presence of normal coronary anatomy clinched the diagnosis of myocarditis. An early and aggressive treatment for CCF along with regular long-term follow-up plays a key role in the management of myocarditis. Role of high-dose Intravenous immunoglobulin in myocarditis has been studied by many trials with different outcomes. This is the first case report showing coexistence of VSD with myocarditis in a neonate presenting as early-onset acute cardiac failure. The report highlights the importance of screening for myocarditis in all previously normal babies presenting primarily with cardiogenic

  2. Characterization of circulating microRNA expression in patients with a ventricular septal defect.

    Directory of Open Access Journals (Sweden)

    Dong Li

    Full Text Available Ventricular septal defect (VSD, one of the most common types of congenital heart disease (CHD, results from a combination of environmental and genetic factors. Recent studies demonstrated that microRNAs (miRNAs are involved in development of CHD. This study was to characterize the expression of miRNAs that might be involved in the development or reflect the consequences of VSD.MiRNA microarray analysis and reverse transcription-polymerase chain reaction (RT-PCR were employed to determine the miRNA expression profile from 3 patients with VSD and 3 VSD-free controls. 3 target gene databases were employed to predict the target genes of differentially expressed miRNAs. miRNAs that were generally consensus across the three databases were selected and then independently validated using real time PCR in plasma samples from 20 VSD patients and 15 VSD-free controls. Target genes of validated 8 miRNAs were predicted using bioinformatic methods.36 differentially expressed miRNAs were found in the patients with VSD and the VSD-free controls. Compared with VSD-free controls, expression of 15 miRNAs were up-regulated and 21 miRNAs were downregulated in the VSD group. 15 miRNAs were selected based on database analysis results and expression levels of 8 miRNAs were validated. The results of the real time PCR were consistent with those of the microarray analysis. Gene ontology analysis indicated that the top target genes were mainly related to cardiac right ventricle morphogenesis. NOTCH1, HAND1, ZFPM2, and GATA3 were predicted as targets of hsa-let-7e-5p, hsa-miR-222-3p and hsa-miR-433.We report for the first time the circulating miRNA profile for patients with VSD and showed that 7 miRNAs were downregulated and 1 upregulated when matched to VSD-free controls. Analysis revealed target genes involved in cardiac development were probably regulated by these miRNAs.

  3. Characterization of circulating microRNA expression in patients with a ventricular septal defect.

    Science.gov (United States)

    Li, Dong; Ji, Long; Liu, Lianbo; Liu, Yizhi; Hou, Haifeng; Yu, Kunkun; Sun, Qiang; Zhao, Zhongtang

    2014-01-01

    Ventricular septal defect (VSD), one of the most common types of congenital heart disease (CHD), results from a combination of environmental and genetic factors. Recent studies demonstrated that microRNAs (miRNAs) are involved in development of CHD. This study was to characterize the expression of miRNAs that might be involved in the development or reflect the consequences of VSD. MiRNA microarray analysis and reverse transcription-polymerase chain reaction (RT-PCR) were employed to determine the miRNA expression profile from 3 patients with VSD and 3 VSD-free controls. 3 target gene databases were employed to predict the target genes of differentially expressed miRNAs. miRNAs that were generally consensus across the three databases were selected and then independently validated using real time PCR in plasma samples from 20 VSD patients and 15 VSD-free controls. Target genes of validated 8 miRNAs were predicted using bioinformatic methods. 36 differentially expressed miRNAs were found in the patients with VSD and the VSD-free controls. Compared with VSD-free controls, expression of 15 miRNAs were up-regulated and 21 miRNAs were downregulated in the VSD group. 15 miRNAs were selected based on database analysis results and expression levels of 8 miRNAs were validated. The results of the real time PCR were consistent with those of the microarray analysis. Gene ontology analysis indicated that the top target genes were mainly related to cardiac right ventricle morphogenesis. NOTCH1, HAND1, ZFPM2, and GATA3 were predicted as targets of hsa-let-7e-5p, hsa-miR-222-3p and hsa-miR-433. We report for the first time the circulating miRNA profile for patients with VSD and showed that 7 miRNAs were downregulated and 1 upregulated when matched to VSD-free controls. Analysis revealed target genes involved in cardiac development were probably regulated by these miRNAs.

  4. Atrial Fibrillation, Atrioventricular Blocks and Bundle Branch Blocks in Hemodialysis Patients

    OpenAIRE

    Mandic, Ante; Tomic, Monika; Petrov, Bozo; Romic, Zeljko

    2012-01-01

    Atrial fibrillation is one of the most frequent arrhythmias diagnosed in clinical practice and it is also relatively common in dialysis patients. Atrioventricular and intraventricular conduction disturbances are less investigated in hemodialysis patients and data about their prevalence are insufficient. The objective of this study was to determine the prevalence of atrial fibrillation, atrioventricular blocks and bundle branch blocks in hemodialysis patients and to analyze different clinical ...

  5. Exploring energy loss by vector flow mapping in children with ventricular septal defect: Pathophysiologic significance.

    Science.gov (United States)

    Honda, Takashi; Itatani, Keiichi; Takanashi, Manabu; Kitagawa, Atsushi; Ando, Hisashi; Kimura, Sumito; Oka, Norihiko; Miyaji, Kagami; Ishii, Masahiro

    2017-10-01

    Vector flow mapping is a novel echocardiographic flow visualization method, and it has enabled us to quantitatively evaluate the energy loss in the left ventricle (intraventricular energy loss). Although intraventricular energy loss is assumed to be a part of left ventricular workload itself, it is unclear what this parameter actually represents. The aim of the present study was to elucidate the characteristics of intraventricular energy loss. We enrolled 26 consecutive children with ventricular septal defect (VSD). On echocardiography vector flow mapping, intraventricular energy loss was measured in the apical 3-chamber view. We measured peak energy loss and averaged energy loss in the diastolic and systolic phases, and subsequently compared these parameters with catheterization parameters and serum brain natrium peptide (BNP) level. Diastolic, peak, and systolic energy loss were strongly and positively correlated with right ventricular systolic pressure (r=0.76, 0.68, and 0.56, p<0.0001, = 0.0001, and 0.0029, respectively) and right ventricular end diastolic pressure (r=0.55, 0.49, and 0.49, p=0.0038, 0.0120, and 0.0111, respectively). In addition, diastolic, peak, and systolic energy loss were significantly correlated with BNP (r=0.75, 0.69 and 0.49, p<0.0001, < 0.0001, and=0.0116, respectively). In children with VSD, elevated right ventricular pressure is one of the factors that increase energy loss in the left ventricle. The results of the present study encourage further studies in other study populations to elucidate the characteristics of intraventricular energy loss for its possible clinical application. Copyright © 2017 Elsevier B.V. All rights reserved.

  6. Complete atrioventricular block following radiation therapy for malignant thymoma

    International Nuclear Information System (INIS)

    Nakao, Takeshi; Kanaya, Honin; Namura, Masanobu; Ohsato, Kazuo; Araki, Tsutomu; Ohka, Takio; Sugihara, Norihiko; Takeda, Ryoyu.

    1990-01-01

    Complete atrioventricular block following radiation is very rare. We present a case which developed after radiation therapy for malignant thymoma. The etiology of conduction disturbances due to radiation is unknown. In our case, serial electrocardiograms showed stepwise progression of the conduction disturbance, and his bundle electrocardiograms revealed new prolongation of the H-V interval. Endomyocardial biopsy specimens demonstrated occlusion in small arteries and diffuse degenerative changes in the myocardium. We therefore attributed the complete atrioventricular block in our patient to secondary damage to the conduction system, caused by radiation-induced occlusive changes in the small arteries supplying the conduction system. (author)

  7. Utilizing Three-Dimensional Printing Technology to Assess the Feasibility of High-Fidelity Synthetic Ventricular Septal Defect Models for Simulation in Medical Education.

    Science.gov (United States)

    Costello, John P; Olivieri, Laura J; Krieger, Axel; Thabit, Omar; Marshall, M Blair; Yoo, Shi-Joon; Kim, Peter C; Jonas, Richard A; Nath, Dilip S

    2014-07-01

    The current educational approach for teaching congenital heart disease (CHD) anatomy to students involves instructional tools and techniques that have significant limitations. This study sought to assess the feasibility of utilizing present-day three-dimensional (3D) printing technology to create high-fidelity synthetic heart models with ventricular septal defect (VSD) lesions and applying these models to a novel, simulation-based educational curriculum for premedical and medical students. Archived, de-identified magnetic resonance images of five common VSD subtypes were obtained. These cardiac images were then segmented and built into 3D computer-aided design models using Mimics Innovation Suite software. An Objet500 Connex 3D printer was subsequently utilized to print a high-fidelity heart model for each VSD subtype. Next, a simulation-based educational curriculum using these heart models was developed and implemented in the instruction of 29 premedical and medical students. Assessment of this curriculum was undertaken with Likert-type questionnaires. High-fidelity VSD models were successfully created utilizing magnetic resonance imaging data and 3D printing. Following instruction with these high-fidelity models, all students reported significant improvement in knowledge acquisition (P 3D printing technology to create high-fidelity heart models with complex intracardiac defects. Furthermore, this tool forms the foundation for an innovative, simulation-based educational approach to teach students about CHD and creates a novel opportunity to stimulate their interest in this field. © The Author(s) 2014.

  8. A prenatally diagnosed pentalogy of cantrell case with encephalocele: A rare variant

    Directory of Open Access Journals (Sweden)

    Melih Atahan Güven

    2009-06-01

    Full Text Available AIM: The aim of this study is to present a prenatally diagnosed and postnatally confirmed Pentalogy of Cantrell case also with neural tube defect. CASE: Characteristic features of Cantrell Pentalogy are omphalocele due to the defect of anterior diaphragm and lower sternum, absence of pericardium and cardiac anomaly. We are presenting here a case with encephalocele and omphalocele containing the heart with atrioventricular septal defect detected during prenatal ultrasonography. There is no consanguinity and history of drug usage or toxin exposure during pregnancy. As these malformations cause a very low chance of survival, pregnancy was terminated after an informed consent. Postmortem genetic evaluation of the fetus confirmed the prenatal findings. CONCLUSION: It is easy to diagnose omphalocele during pregnancy but if it associates with heart anomalies, Cantrell Pentalogy must be remembered. Encephalocele and other types of neural tube defects very rarely associate with this disorder and there were fewer than 20 cases reported in the literature.

  9. Cardiac imaging : X-ray, magnetic resonance and ultrasound in congenital heart diseases

    International Nuclear Information System (INIS)

    Rigby, M.L.

    1991-01-01

    In many instances, the management of simple or common congenital cardiac anomalies, such as patient arterial duct, atrial septal defect or ventricular septal defect, can be based upon clinical evaluation, the chest radiograph and conventional transthoracic echocardiography and Doppler investigation. Angiography is usually required for those conditions, such as the tetralogy of Fallot, in which the size and anatomy of the pulmonary arteries influence the timing or the type of surgical intervention. Interventional cardiac catheterization, however, requires high quality X-ray screening and, frequently, transesophageal echocardiography. The investigation of more complex anomalies, such as those in hearts with a univentricular atrioventricular connection, often involves several methods of investigation. These complex anomalies require complete morphological and physiological assessment in order to provide optimum medical and surgical management. Increasingly, the diagnosis is established in utero. Should termination of pregnancy then be encouraged? Or, should all patients be subjected to an aggressive medical and surgical treatment regimen to optimize the chances of a later successful Fontan operation? Finally, what is the role of cardiac transplantation or the Norwood operation in those infants in whom the long term prognosis of conventional treatment is considered to be poor? (author). 20 refs.; 6 figs

  10. Effectiveness of simulator-based echocardiography training of noncardiologists in congenital heart diseases.

    Science.gov (United States)

    Wagner, Robert; Razek, Vit; Gräfe, Florentine; Berlage, Thomas; Janoušek, Jan; Daehnert, Ingo; Weidenbach, Michael

    2013-07-01

    Congenital heart diseases (CHD) are responsible for substantial morbidity and mortality in neonates. The preliminary diagnosis often is made by noncardiologists. For this reason, there is a huge demand of training in echocardiography of CHD. This is difficult to achieve due to limited resources of specialized centers. The goal of this study was to investigate the training effect of the echocardiography simulator EchoCom on trainee's ability to diagnose CHD. We enrolled 10 residents for simulator-based training in echocardiography of CHD. All participants were instructed on the simulator's basic handling and had one hour to scan the first 9 datasets information (ventricular septal defect, atrial septal defect, atrioventricular septal defect, Tetralogy of Fallot, transposition of great arteries, congenital corrected transposition of great arteries, common arterial trunk, hypoplastic left heart syndrome, normal anatomy) and establish a diagnosis. No help was given except for support regarding simulator related issues. Afterward, 2 rounds of structured simulator based echocardiography training focused on echocardiographic anatomy, spatial orientation, standard views, and echocardiographic anatomy of different CHD followed. All participants completed a standardized questionnaire containing 10 multiple-choice (MC) questions focusing on basic theoretical knowledge in echocardiographic anatomy and common CHD. Almost all of the residents invited from the affiliated children's hospital had little (20%) or no experience (80%) in echocardiography of CHD. Their Pretest and Posttest scores showed significant improvement for both, MC test and performance test, respectively. Our study showed that simulator-based training in echocardiography in CHD could be very effective and may assist with training outside the scope of CHD. © 2013, Wiley Periodicals, Inc.

  11. MicroRNA expression, target genes, and signaling pathways in infants with a ventricular septal defect.

    Science.gov (United States)

    Chai, Hui; Yan, Zhaoyuan; Huang, Ke; Jiang, Yuanqing; Zhang, Lin

    2018-02-01

    This study aimed to systematically investigate the relationship between miRNA expression and the occurrence of ventricular septal defect (VSD), and characterize the miRNA target genes and pathways that can lead to VSD. The miRNAs that were differentially expressed in blood samples from VSD and normal infants were screened and validated by implementing miRNA microarrays and qRT-PCR. The target genes regulated by differentially expressed miRNAs were predicted using three target gene databases. The functions and signaling pathways of the target genes were enriched using the GO database and KEGG database, respectively. The transcription and protein expression of specific target genes in critical pathways were compared in the VSD and normal control groups using qRT-PCR and western blotting, respectively. Compared with the normal control group, the VSD group had 22 differentially expressed miRNAs; 19 were downregulated and three were upregulated. The 10,677 predicted target genes participated in many biological functions related to cardiac development and morphogenesis. Four target genes (mGLUR, Gq, PLC, and PKC) were involved in the PKC pathway and four (ECM, FAK, PI3 K, and PDK1) were involved in the PI3 K-Akt pathway. The transcription and protein expression of these eight target genes were significantly upregulated in the VSD group. The 22 miRNAs that were dysregulated in the VSD group were mainly downregulated, which may result in the dysregulation of several key genes and biological functions related to cardiac development. These effects could also be exerted via the upregulation of eight specific target genes, the subsequent over-activation of the PKC and PI3 K-Akt pathways, and the eventual abnormal cardiac development and VSD.

  12. Nasal septum configuration as a basis for novel septal splints.

    Science.gov (United States)

    Furbish, Nina; Kühnel, Thomas S

    2017-03-01

    The objective is to use anatomical determinations of nasal septum shape and surface area in adults as a design basis for silastic septal splints of universal size and fit and offering maximum possible surface coverage. The objective is also to devise a method of securing the septal splints, so that surface pressure on septal mucosa is distributed as evenly as possible while not interfering with capillary perfusion. Nasal septum area was determined in 21 Caucasian body donors, and nasal septum thickness was measured in 20 CT scans. Septal splints of universal size and shape were prepared from silastic sheeting. The holding force of various neodymium-iron-boron (NdFeB) magnets, and the surface pressure exerted by magnet-containing septal splints was calculated. These septal splints of novel design offer a satisfactory fit in routine clinical practice. The splints can be securely attached with built-in NdFeB magnets, and surface pressure can be distributed evenly across the nasal septum while not interfering with mucosal tissue perfusion. With their simple intranasal insertion, these magnet-containing septal splints of universal size and optimised shape offer maximum possible septum coverage following septoplasty/septorhinoplasty. The absence of interference with septal tissue perfusion means that they are likely to be associated with fewer postoperative complications and better outcomes.

  13. A null mutation of Hhex results in abnormal cardiac development, defective vasculogenesis and elevated Vegfa levels.

    Science.gov (United States)

    Hallaq, Haifa; Pinter, Emese; Enciso, Josephine; McGrath, James; Zeiss, Caroline; Brueckner, Martina; Madri, Joseph; Jacobs, Harris C; Wilson, Christine M; Vasavada, Hemaxi; Jiang, Xiaobing; Bogue, Clifford W

    2004-10-01

    The homeobox gene Hhex has recently been shown to be essential for normal liver, thyroid and forebrain development. Hhex(-/-) mice die by mid-gestation (E14.5) and the cause of their early demise remains unclear. Because Hhex is expressed in the developing blood islands at E7.0 in the endothelium of the developing vasculature and heart at E9.0-9.5, and in the ventral foregut endoderm at E8.5-9.0, it has been postulated to play a critical role in heart and vascular development. We show here, for the first time, that a null mutation of Hhex results in striking abnormalities of cardiac and vascular development which include: (1) defective vasculogenesis, (2) hypoplasia of the right ventricle, (3) overabundant endocardial cushions accompanied by ventricular septal defects, outflow tract abnormalities and atrio-ventricular (AV) valve dysplasia and (4) aberrant development of the compact myocardium. The dramatic enlargement of the endocardial cushions in the absence of Hhex is due to decreased apoptosis and dysregulated epithelial-mesenchymal transformation (EMT). Interestingly, vascular endothelial growth factor A (Vegfa) levels in the hearts of Hhex(-/-) mice were elevated as much as three-fold between E9.5 and E11.5, and treatment of cultured Hhex(-/-) AV explants with truncated soluble Vegfa receptor 1, sFlt-1, an inhibitor of Vegf signaling, completely abolished the excessive epithelial-mesenchymal transformation seen in the absence of Hhex. Therefore, Hhex expression in the ventral foregut endoderm and/or the endothelium is necessary for normal cardiovascular development in vivo, and one function of Hhex is to repress Vegfa levels during development.

  14. Echocardiographic effects of changing atrioventricular delay in cardiac resynchronization therapy based on displacement

    DEFF Research Database (Denmark)

    Valeur, Nana; Fritz-Hansen, Thomas; Risum, Niels

    2010-01-01

    In studies showing benefits of cardiac resynchronization therapy (CRT), individual atrioventricular (AV) delays have been optimized using echocardiography. However, the method for AV delay optimization remains controversial.......In studies showing benefits of cardiac resynchronization therapy (CRT), individual atrioventricular (AV) delays have been optimized using echocardiography. However, the method for AV delay optimization remains controversial....

  15. Efficacy and Safety of Transthoracic Echocardiography Alone in Transcatheter Closure of Secundum-Type Atrial Septal Defects in Adults.

    Science.gov (United States)

    Ding, Cheng; Chang, Jia-Kan; Lin, Chang-Chyi; Wu, Yong-Jian; Hsieh, Kai-Sheng

    2016-04-01

    On-site transthoracic echocardiography (TTE) to guide the transcutaneous closure of secundum-type atrial septal defects (ASDs) in the catheterization laboratory remains unclear, especially in adults. Between 2005 and 2012, a total of 82 adults underwent transcutaneous closure of ASDs. The initial 15 cases underwent the procedure with both on-site transesophageal echocardiography (TEE) and TTE monitoring. Since January 2008, a total of 67 patients underwent on-site TTE alone to guide the procedure. Among the 82 adult patients who underwent a transcutaneous closure of the secundum-type ASD procedure, all had successful closure of the defects, and no periprocedural adverse complications occurred. No statistical significance was observed in the successful complete shunt closure rate between the TEE plus TTE and TTE groups during sequential follow-up (postprocedure 24 hour [87% vs. 92%],1 month [93% vs. 95%], 3 month [93% vs. 97%], and 12 month [93% vs. 97%], P > 0.05, respectively) nor was a significant difference observed between the two groups, including decreased right ventricular dimension (29.5 ± 3.3 vs. 32.0 ± 4.9 mm, 26.5 ± 3.0 vs. 28.7 ± 4.6 mm, 26.2 ± 3.1 vs. 28.2 ± 4.8 mm, and 25.6 ± 2.8 vs. 27.7 ± 4.7 mm, P > 0.05, respectively) or increased left ventricular end-diastolic dimension (41.1 ± 2.0 vs. 42.6 ± 3.0 mm, 44.3 ± 2.7 vs. 45.5 ± 3.1 mm, 44.2 ± 2.8 vs. 45.4 ± 3.1 mm, 44.9 ± 2.7 vs. 45.8 ± 2.6 mm, P > 0.05, respectively) before the procedure, and at the 3-, 6-, and 12-month follow-up evaluations. This study showed that TTE guidance alone may be considered efficacious and safe as TEE during a transcutaneous ASD occlusion procedure in select adults. © 2015, Wiley Periodicals, Inc.

  16. Atrioventricular conduction abnormality and hyperchloremic metabolic acidosis in toluene sniffing

    Directory of Open Access Journals (Sweden)

    Jian-Hsiung Tsao

    2011-10-01

    Full Text Available Toluene is an aromatic hydrocarbon with widespread industrial use as an organic solvent. As a result of the euphoric effect and availability of these substances, inhalation of toluene-based products is popular among young adults and children. Chronic or acute exposure is known to cause acid–base and electrolyte disorders, and to be toxic to the nervous and hematopoietic systems. We report a 38-year-old man who suffered from general muscular weakness of all extremities after toluene sniffing, which was complicated with hypokalemic paralysis, atrioventricular conduction abnormality, and normal anion gap hyperchloremic metabolic acidosis. Renal function, serum potassium and acid–base status normalized within 3 days after aggressive potassium chloride and intravenous fluid replacement. Electrocardiography showed regression of first-degree atrioventricular block. Exposure to toluene can lead to cardiac arrhythmias and sudden sniffing death syndrome. Tachyarrhythmia is the classical manifestation of toluene cardiotoxicity. Atrioventricular conduction abnormalities have been rarely mentioned in the literature. Knowledge of the toxicology and medical complications associated with toluene sniffing is essential for clinical management of these patients.

  17. Risk Analysis of the Long-Term Outcomes of the Surgical Closure of Secundum Atrial Septal Defects

    Directory of Open Access Journals (Sweden)

    Hong Rae Kim

    2017-04-01

    Full Text Available Background: Closure of a secundum atrial septal defect (ASD is possible through surgical intervention or device placement. During surgical intervention, concomitant pathologies are corrected. The present study was conducted to investigate the outcomes of surgical ASD closure, to determine the risk factors of mortality, and establish the effects of concomitant disease correction. Methods: Between October 1989 and October 2009, 693 adults underwent surgery for secundum ASD. Their mean age was 40.9±13.1 years, and 199 (28.7% were male. Preoperatively, atrial fibrillation was noted in 39 patients (5.6% and significant tricuspid regurgitation (TR in 137 patients (19.8%. The mean follow-up duration was 12.4±4.7 years. Results: There was no 30-day mortality. The 1-, 5-, 10-, and 20-year survival rates were 99.4%, 96.8%, 94.5%, and 81.6%, respectively. In multivariate analysis, significant preoperative TR (hazard ratio [HR], 1.95; 95% confidence interval [CI], 1.09 to 3.16; p=0.023 and preoperative age (HR, 1.04; 95% CI, 1.01 to 1.06; p=0.001 were independent risk factors for late mortality. The TR grade significantly decreased after ASD closure with tricuspid repair. However, in patients with more than mild TR, repair was not associated with improved long-term survival (p=0.518. Conclusion: Surgical ASD closure is safe. Significant preoperative TR and age showed a strong negative correlation with survival. Our data showed that tricuspid valve repair improved the TR grade effectively. However, no effect on long-term survival was found. Therefore, early surgery before the development of significant TR mat be beneficial for improving postoperative survival.

  18. Dental management of pediatric patients affected by pulmonary atresia with ventricular septal defect: A scoping review.

    Science.gov (United States)

    Garrocho-Rangel, A; Echavarría-García, A-C; Rosales-Bérber, M-A; Flores-Velázquez, J; Pozos-Guillén, A

    2017-07-01

    Congenital Heart Diseases (CHD) involves a wide range of pathological conditions, such as Pulmonary Atresia with Ventricle Septal Defect (PA/VSD). This disorder leads to the systemic circulation of oxygen-poor blood (cyanosis), with associated features and consequences in the oral cavity. Using scoping review methodology for screening and article selection, the primary objectives of this paper were as follows: first, to pose a research question; second, to identify relevant studies in order to answer the research question; third, to select and retrieve the studies; fourth, to chart the critical data, and finally, to collate, summarize, and report the results from the most important articles on the dental management of children affected with PA/VSD. Relevant articles (Randomized Controlled Trials [RCT], reviews, observational studies, and clinical case reports) published over a 10-year period were identified and retrieved from four Internet databases: PubMed; Embase/Ovid; Cochrane Library, and Google Scholar. By title and abstract screening and after removing duplicates, 24 articles were finally included in the present scoping review. According to the extracted data, the following are the most important clinical issues to be considered when treating children with PA/VSD in the dental setting: prevalence of dental caries; prevention of dental disease (oral hygiene and diet); bacteremia and infective endocarditis risk, and child behavior control and treatment under general anesthesia. Pediatric Dentists should bear in mind that early diagnosis and treatment, together a long-term follow-up of children with PA/VSD, continue to be the best approaches for achieving enhanced patient psychological well-being and, in consequence, their good quality of life.

  19. Epidemiological study of congenital heart defects in children and adolescents: analysis of 4,538 cases

    Directory of Open Access Journals (Sweden)

    Nelson Itiro Miyague

    2003-03-01

    Full Text Available OBJECTIVE: To analyze the frequency and prevalence of congenital heart defects in a tertiary care center for children with heart diseases. METHODS: We carried out an epidemiological assessment of the first medical visit of 4,538 children in a pediatric hospital from January 1995 to December 1997. All patients with congenital heart defects had their diagnoses confirmed at least on echocardiography. The frequency and prevalence of the anomalies were computed according to the classification of sequential analysis. Age, weight, and sex were compared between the groups of healthy individuals and those with congenital heart defects after distribution according to the age group. RESULTS: Of all the children assessed, 2,017 (44.4% were diagnosed with congenital heart disease, 201 (4.4% with acquired heart disease, 52 (1.2% with arrhythmias, and 2,268 (50% were healthy children. Congenital heart diseases predominated in neonates and infants, corresponding to 71.5% of the cases. Weight and age were significantly lower in children with congenital heart defects. Ventricular septal defect was the most frequent acyanotic anomaly, and tetralogy of Fallot was the most frequent cyanotic anomaly. CONCLUSION: Children with congenital heart defects are mainly referred during the neonatal period and infancy with impairment in gaining weight. Ventricular septal defect is the most frequent heart defect.

  20. Atrioventricular Dissociation after Electroconvulsive Therapy

    Directory of Open Access Journals (Sweden)

    Siegfried William Yu

    2011-01-01

    Full Text Available Electroconvulsive therapy (ECT is increasingly used as a treatment for psychiatric disorders. Cardiac effects are the principal cause of medical complications in these patients. We report a case of atrioventricular (AV dissociation that occurred after ECT that was treated with pacemaker implantation. The mechanisms contributing to the onset of AV dissociation in this patient, and the management and rationale for device therapy, in light of the most recent guidelines, are reviewed.

  1. Natural history of ventricular septal defects in Nigerian children

    African Journals Online (AJOL)

    Spontaneous closure of some of small and medium-sized defects within the first 2 ... Permission for the study was obtained from the UBTH Ethical. Committee. .... may be due to the fact that most babies in our environment are born outside ...

  2. Effect of atrioventricular conduction on heart rate variability

    KAUST Repository

    Ahmad, Talha Jamal; Ali, Hussnain; Majeed, S. M Imran; Khan, Shoab A.

    2011-01-01

    This paper discusses the effect of atrioventricular conduction time (AVCT) on the short-term Heart Rate Variability (HRV) by computing HRV parameters using intervals between the onsets of successive P waves (PP time series) for three groups: normal

  3. Natural history of ventricular septal defects in Nigerian children ...

    African Journals Online (AJOL)

    There were 2 (3.3%) deaths from bronchopneumonia and bacterial endocarditis. Conclusion. Spontaneous closure readily occurs in small-sized defects and muscular VSDs. However, most patients with moderate to large VSDs are confined to long-term medical management, highlighting the need for indigenous surgical ...

  4. Prospective Study of Adenosine on Atrioventricular Nodal Conduction in Pediatric and Young Adult Patients After Heart Transplantation.

    Science.gov (United States)

    Flyer, Jonathan N; Zuckerman, Warren A; Richmond, Marc E; Anderson, Brett R; Mendelsberg, Tamar G; McAllister, Jennie M; Liberman, Leonardo; Addonizio, Linda J; Silver, Eric S

    2017-06-20

    Supraventricular tachycardia is common after heart transplantation. Adenosine, the standard therapy for treating supraventricular tachycardia in children and adults without transplantation, is relatively contraindicated after transplantation because of a presumed risk of prolonged atrioventricular block in denervated hearts. This study tested whether adenosine caused prolonged asystole after transplantation and if it was effective in blocking atrioventricular nodal conduction in these patients. This was a single-center prospective clinical study including healthy heart transplant recipients 6 months to 25 years of age presenting for routine cardiac catheterization during 2015 to 2016. After catheterization, a transvenous pacing catheter was placed and adenosine was given following a dose-escalation protocol until atrioventricular block was achieved. The incidence of clinically significant asystole (≥12 seconds after adenosine) was quantified. The effects of patient characteristics on adenosine dose required to produce atrioventricular block and duration of effect were also measured. Eighty patients completed adenosine testing. No patient (0%; 95% confidence interval, 0-3) required rescue ventricular pacing. Atrioventricular block was observed in 77 patients (96%; 95% confidence interval, 89-99). The median longest atrioventricular block was 1.9 seconds (interquartile range, 1.4-3.2 seconds), with a mean duration of adenosine effect of 4.3±2.0 seconds. No patient characteristic significantly predicted the adenosine dose to produce atrioventricular block or duration of effect. Results were similar across patient weight categories. Adenosine induces atrioventricular block in healthy pediatric and young adult heart transplant recipients with minimal risk when low initial doses are used (25 μg/kg; 1.5 mg if ≥60 kg) and therapy is gradually escalated. URL: http://www.clinicaltrials.gov. Unique identifier: NCT02462941. © 2017 American Heart Association, Inc.

  5. Gastrointestinal system malformations in children are associated with congenital heart defects.

    Science.gov (United States)

    Orün, Utku Arman; Bilici, Meki; Demirçeken, Fulya G; Tosun, Mahya; Ocal, Burhan; Cavuşoğlu, Yusuf Hakan; Erdoğan, Derya; Senocak, Filiz; Karademir, Selmin

    2011-03-01

    To determine the frequency of congenital heart defects (CHD) in children with gastrointestinal malformations (GISM) and mortality rates in patients with GISM. Two hundred and forty two consecutive children patients with GISM followed up in Pediatric Surgery Clinics of our hospital were examined for cardiovascular anomaly by the Department of Pediatric Cardiology, and the CHD incidence was investigated by examining the records of the patients retrospectively. Chi-square test was used for the statistical analysis of data. Two hundred and forty two patients with gastrointestinal system malformations were included in the study. Of 242 patients, 135 (55.8%) were male and 107 (44.2%) were female, and their age range was 0-15 years. The most frequent GISM were anorectal malformations (43.2%), atresia involving stomach, ileum or colon (21%) and esophageal atresia/tracheoesophageal fistula (18.3%). Congenital heart defects were observed in 28.5% of the participants. The most frequent defects were as follows; atrial septal defect (31 patients, 44.9%) a, ventricular septal defect (17 patients, 24.6%) and patent ductus arteriosus (5 patients, 7.2%). There was no significant difference (p>0.05) in mortality rate in patients with CHD (16.7%) and without CHD (13.3%) undergoing operations for GISM. We would like to emphasize the importance of the earliest possible cardiological evaluation of all patients with gastrointestinal system malformations.

  6. Removing intranasal splints after septal surgery.

    Science.gov (United States)

    Aksoy, Elif; Serin, Gediz Murat; Polat, Senol; Kaytaz, Asm

    2011-05-01

    The aim of this retrospective chart review of the patients who had septal surgery with or without turbinate surgery was to compare the postoperative complication rates according to the time of intranasal-splint (INS) removal. The data of 137 patients who underwent septal surgery with or without turbinate surgery at 2 different hospitals of Acıbadem Health Care Group between January 2007 and March 2009 were retrospectively evaluated. The patients who had these risk factors were eliminated, and 96 patients were included in this study. The patients were divided into 2 groups according to splint-removal time. The first group comprises patients whose nasal splints were removed in 24 hours after surgery, and the second group comprises patients whose splints were removed 5 days after the surgery. Any bleeding, septal hematoma, and synechia after pack removal were recorded. Analysis of the rate of complications was done with the χ test. Sixty-five male and 31 female patients with a mean age of 32.4 years (range, 18-57 years) were included in the study groups. Septal surgeries were performed in association with turbinate surgery in all 96 patients. These patients were divided into 2 groups. In the first group (n = 50), INSs were removed in 24 hours after surgery. In the second group (n = 46), INSs were removed 5 days after surgery. Bleeding within the first postoperative week was not recorded in both groups. Late bleeding was recorded in 2% (n = 1) of group 1 and in 2.17% (n = 1) in group 2. Septal hematoma and synechia were not recorded in none of the groups. The results were not statistically significant (P = 1). The routine use of INSs after septoplasty and removing them 24 hours after septoplasty are sufficient to avoid postoperative complications, and it minimizes postoperative discomfort.

  7. Syndromes and Disorders Associated with Omphalocele (III: Single Gene Disorders, Neural Tube Defects, Diaphragmatic Defects and Others

    Directory of Open Access Journals (Sweden)

    Chih-Ping Chen

    2007-06-01

    Full Text Available Omphalocele can be associated with single gene disorders, neural tube defects, diaphragmatic defects, fetal valproate syndrome, and syndromes of unknown etiology. This article provides a comprehensive review of omphalocele-related disorders: otopalatodigital syndrome type II; Melnick–Needles syndrome; Rieger syndrome; neural tube defects; Meckel syndrome; Shprintzen–Goldberg omphalocele syndrome; lethal omphalocele-cleft palate syndrome; cerebro-costo-mandibular syndrome; fetal valproate syndrome; Marshall–Smith syndrome; fibrochondrogenesis; hydrolethalus syndrome; Fryns syndrome; omphalocele, diaphragmatic defects, radial anomalies and various internal malformations; diaphragmatic defects, limb deficiencies and ossification defects of skull; Donnai–Barrow syndrome; CHARGE syndrome; Goltz syndrome; Carpenter syndrome; Toriello–Carey syndrome; familial omphalocele; Cornelia de Lange syndrome; C syndrome; Elejalde syndrome; Malpuech syndrome; cervical ribs, Sprengel anomaly, anal atresia and urethral obstruction; hydrocephalus with associated malformations; Kennerknecht syndrome; lymphedema, atrial septal defect and facial changes; and craniosynostosis- mental retardation syndrome of Lin and Gettig. Perinatal identification of omphalocele should alert one to the possibility of omphalocele-related disorders and familial inheritance and prompt a thorough genetic counseling for these disorders.

  8. Ventricular septal necrosis after blunt chest trauma

    Directory of Open Access Journals (Sweden)

    Alireza Ahmadi

    2012-07-01

    Full Text Available Ventricular Septal Defect (VSD after blunt chest trauma is a very rare traumatic affection.We report here a case of blunt chest injury-related VSD and pseudoaneurysm.A 30-year old male truck driver was referred from a trauma center to our hospital seven days after a blunt chest trauma and rib fracture. The patient had severe pulmonary edemaand echocardiography showed large VSD. Several mechanisms are involved in the pathogenesis of this affection including an acute compression of the heart muscle between the sternum and the spine, leading to excessive changes in the intrathoracic and most likely theintracardiac pressure after blunt chest injury. Traumatical patients with the same symptoms may be at risk of sudden death. Therefore, a high grade of suspicion is mandatory even without solid evidence of myocardial damage on the initial evaluation. In continue somehidden angles of this case was discussed. Given the prognostic implications of traumatic VSD with associated pseudoaneurysm, its detection has critical value for preventing its clinicalsequelae.

  9. Utility of pulmonary venous flow diastolic deceleration time in an adult patient undergoing surgical closure of atrial septal defect and coronary artery bypass grafting

    Directory of Open Access Journals (Sweden)

    Dharmesh R Agrawal

    2013-01-01

    Full Text Available Acute left ventricular (LV failure has been reported after surgical closure of atrial septal defect (ASD in adult patients. We report acute LV failure in a 56 year old gentleman following coronary artery bypass grafting (CABG and surgical closure of ASD. Transesophageal echocardiography examination of the patient following closure of ASD and CABG showed a residual ASD and a shunt (Qp :Qs = 1.5. The residual ASD was closed after re-institution of cardiopulmonary bypass (CPB under cardioplegic cardiac arrest. However, the patient did not tolerate closure of the residual ASD. The CPB was re-established and under cardioplegic cardiac arrest residual ASD was reopened to create a fenestration. This time patient was weaned easily from CPB. Postoperatively, 16 hours after extubation, patient became hemodynamically unstable, the patient was electively put on ventilator and intra-aortic balloon pump. Later the patient was weaned off successfully from ventilator. Retrospective analysis of pulmonary venous flow diastolic deceleration time (PVDT D recorded during prebypass period measured 102 msec suggestive of high left atrial pressure which indicate possibility of LV failure after ASD closure.

  10. Direct type gerbode defect with cleft in anterior mitral leaflet: A rarest of rare combination

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    Rakesh Jain

    2016-01-01

    Full Text Available A 15-year-old boy was evaluated for the new onset of dyspnea. He had no significant medical or surgical history. On echocardiography, he was found to have a direct communication between the left ventricle and the right atrium through the atrioventricular (AV septum (a rare type of Gerbode defect with an associated cleft in the anterior mitral leaflet. The combination of direct type Gerbode defect with the cleft of the anterior mitral leaflet is extremely rare.

  11. Repair of Tetralogy of Fallot in Infancy via the Atrioventricular Approach

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    Hamid Bigdelian

    2016-02-01

    Full Text Available Background: Tetralogy of Fallot (TOF is a well-recognized congenital heart disease. Despite improvements in the outcomes of surgical repair, the optimal timing of surgery and type of surgical management of patients with TOF remains controversial. The purpose of this study was to assess outcomes following the repair of TOF in infants depending on the surgical procedure used. Methods: This study involved the retrospective review of 120 patients who underwent TOF repair between 2010 and 2013. Patients were divided into three groups depending on the surgical procedure that they underwent. Corrective surgery was done via the transventricular approach (n=40, the transatrial approach (n=40, or a combined atrioventricular approach (n=40. Demographic data and the outcomes of the surgical procedures were compared among the groups. Results: In the atrioventricular group, the incidence of the following complications was found to be significantly lower than in the other groups: complete heart block (p=0.034, right ventricular failure (p=0.027 and mediastinal bleeding (p=0.007. Patients in the atrioventricular group had a better postoperative right ventricular ejection fraction (p=0.001. No statistically significant differences were observed among the three surgical groups in the occurrence of tachycardia, renal failure, and tricuspid incompetence. The one-year survival rates in the three groups were 95%, 90%, and 97.5%, respectively (p=0.395. Conclusion: Combined atrioventricular repair of TOF in infancy can be safely performed, with acceptable surgical risk, a low incidence of reoperation, good ventricular function outcomes, and an excellent survival rate.

  12. Safety and efficacy of nano lamellar TiN coatings on nitinol atrial septal defect occluders in vivo

    International Nuclear Information System (INIS)

    Zhang, Zhi xiong; Fu, Bu fang; Zhang, De yuan; Zhang, Zhi wei; Cheng, Yan; Sheng, Li yuan; Lai, Chen; Xi, Ting fei

    2013-01-01

    Atrial septal defect (ASD) occlusion devices made of nickel–titanium (NiTi) have a major shortcoming in that they release nickel into the body. We modified NiTi occluders using Arc Ion Plating technology. Nano lamellar titanium–nitrogen (TiN) coatings were formed on the surfaces of the occluders. The safety and efficacy of the modified NiTi occluders were evaluated in animal model. The results showed that 38 out of 39 rams (97%) survived at the end of the experiment. Fibrous capsules formed on the surfaces of the devices. Gradual endothelialization took place through the attachment of endothelial progenitor cells from the blood and the migration of endothelial cells from adjacent endocardium. The neo-endocardium formed more quickly in the coated group than in the uncoated group, as indicated by the evaluation of the six month study group. After TiN coating, there was no significant difference in endothelial cell cycle. TiN coating significantly reduced the release of nickel in both in vivo and in vitro indicating an improved biocompatibility of the nitinol ASD occluders. Superior and modified ASD occluders may provide a good choice for people with nickel allergies after sFDA registration, which is expected in one to two years. - Highlights: ► The nano lamella TiN coating did not change the shape-memory behavior and flexibility of the nitinol occluder. ► Nano lamella TiN coating modifications significantly reduced nickel release from nitinol ASD occluder. ► The new ASD occluder was found to be superior to nitinol ASD occluder with respect to both safety and efficacy

  13. [Neonatal anatomical repair of transposition of great vessels associated with atrial septal defect. Apropos of 42 cases].

    Science.gov (United States)

    Planché, C; Serraf, A; Bruniaux, J; Lacour-Gayet, F; Bouchart, F; Losay, J; Touchot, A

    1991-05-01

    The good results obtained by anatomic correction of simple transposition of the great arteries (TGA) in the neonatal period have incited some surgical teams to widen the indications to neonates with TGA associated with ventricular septal defect (VSD). The classical management of these patients is a two stage procedure: banding of the pulmonary artery followed by detransposition, which carries a certain risk. Between January 1985 and June 1990, 42 neonates with TGA and VSD underwent a combined procedure consisting in anatomic correction of the TGA and closure of the VSD. The average age of these patients was 16 days, and the average weight was 3.3 kg. Ten patients had coarctation and 6 underwent a complete one stage correction by an anterior approach. The surgical technique consisted in closing the VSD from the right atrium in 20 patients, from the right ventricle in 11 patients and from the pulmonary artery in 11 patients, associated with detransposition of the great arteries and coronary artery reimplantation. Three children died in the preoperative period (7.1%). In two cases, death was related to malposition of the coronary artery. The third fatality was the result of haemorrhage. There has been one late death three years after surgery. Four patients have been reoperated for stenosis of the right ventricular outflow tract (1 case), recurrence of coarctation (2 cases) and stenosis of the superior vena cava (1 case) and have survived. All patients were followed up for an average period of 26.4 +/- 19 months. They are all in the NYHA Class I without treatment. One patient has mild aortic regurgitation.(ABSTRACT TRUNCATED AT 250 WORDS)

  14. Safety and efficacy of nano lamellar TiN coatings on nitinol atrial septal defect occluders in vivo

    Energy Technology Data Exchange (ETDEWEB)

    Zhang, Zhi xiong, E-mail: Top5460@163.com [Research Institute of Peking University in Shenzhen, Shenzhen 518057 (China); Fu, Bu fang, E-mail: fubnicpbp@163.com [National Institutes for Food and Drug Control, Beijing (China); Zhang, De yuan, E-mail: Deyuanzhangcn@yahoo.com.cn [Lifetech Scientific (Shenzhen) Co., Ltd., Shenzhen (China); Zhang, Zhi wei, E-mail: Zhzhx65@163.com [Guangdong Cardiovascular Institute, Guangzhou (China); Cheng, Yan, E-mail: chengyan@pku.edu.cn [Academy for Advanced Interdisciplinary Studies, Peking University, Beijing (China); Sheng, Li yuan, E-mail: lysheng@yeah.net [Research Institute of Peking University in Shenzhen, Shenzhen 518057 (China); Lai, Chen, E-mail: laichen1110@163.com [Research Institute of Peking University in Shenzhen, Shenzhen 518057 (China); Xi, Ting fei, E-mail: Xitingfie@pku.edu.cn [Research Institute of Peking University in Shenzhen, Shenzhen 518057 (China); Academy for Advanced Interdisciplinary Studies, Peking University, Beijing (China)

    2013-04-01

    Atrial septal defect (ASD) occlusion devices made of nickel–titanium (NiTi) have a major shortcoming in that they release nickel into the body. We modified NiTi occluders using Arc Ion Plating technology. Nano lamellar titanium–nitrogen (TiN) coatings were formed on the surfaces of the occluders. The safety and efficacy of the modified NiTi occluders were evaluated in animal model. The results showed that 38 out of 39 rams (97%) survived at the end of the experiment. Fibrous capsules formed on the surfaces of the devices. Gradual endothelialization took place through the attachment of endothelial progenitor cells from the blood and the migration of endothelial cells from adjacent endocardium. The neo-endocardium formed more quickly in the coated group than in the uncoated group, as indicated by the evaluation of the six month study group. After TiN coating, there was no significant difference in endothelial cell cycle. TiN coating significantly reduced the release of nickel in both in vivo and in vitro indicating an improved biocompatibility of the nitinol ASD occluders. Superior and modified ASD occluders may provide a good choice for people with nickel allergies after sFDA registration, which is expected in one to two years. - Highlights: ► The nano lamella TiN coating did not change the shape-memory behavior and flexibility of the nitinol occluder. ► Nano lamella TiN coating modifications significantly reduced nickel release from nitinol ASD occluder. ► The new ASD occluder was found to be superior to nitinol ASD occluder with respect to both safety and efficacy.

  15. Outcome of cardiac surgery in patients with congenital heart disease in England between 1997 and 2015.

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    Aleksander Kempny

    Full Text Available The number of patients with congenital heart disease (CHD is increasing worldwide and most of them will require cardiac surgery, once or more, during their lifetime. The total volume of cardiac surgery in CHD patients at a national level and the associated mortality and predictors of death associated with surgery are not known. We aimed to investigate the surgical volume and associated mortality in CHD patients in England.Using a national hospital episode statistics database, we identified all CHD patients undergoing cardiac surgery in England between 1997 and 2015.We evaluated 57,293 patients (median age 11.9years, 46.7% being adult, 56.7% female. There was a linear increase in the number of operations performed per year from 1,717 in 1997 to 5,299 performed in 2014. The most common intervention at the last surgical event was an aortic valve procedure (9,276; 16.2%, followed by repair of atrial septal defect (9,154; 16.0%, ventricular septal defect (7,746; 13.5%, tetralogy of Fallot (3,523; 6.1% and atrioventricular septal defect (3,330; 5.8% repair. Associated mortality remained raised up to six months following cardiac surgery. Several parameters were predictive of post-operative mortality, including age, complexity of surgery, need for emergency surgery and socioeconomic status. The relationship of age with mortality was "U"-shaped, and mortality was highest amongst youngest children and adults above 60 years of age.The number of cardiac operations performed in CHD patients in England has been increasing, particularly in adults. Mortality remains raised up to 6-months after surgery and was highest amongst young children and seniors.

  16. Evaluation of Paradoxical Septal Motion Following Cardiac Surgery with Gated Cardiac Blood Pool Scan

    International Nuclear Information System (INIS)

    Shin, Seong Hae; Chung, June Key; Lee, Myung Chul; Cho, Bo Youn; Koh, Chang Soon; Suh, Kyung Phil

    1985-01-01

    The development of paradoxical interventricular septal motion is a common consequence of cardiopulmonary bypass operation. The reason for this postoperative abnormal septal motion is not clear. 41 patients were studied preoperatively and postoperatively with radionuclide blood pool scan to evaluate the frequency of development of paradoxical septal motion with right ventricular volume overload before surgery and the frequency of development of paradoxical septal motion after cardiac surgery with cardiopulmonary bypass, and to evaluate the change of EF related to the development of paradoxical septal motion after cardiac surgery. The results were as follows; 1) 7 of 41 patients with right ventricular volume overload (that is 17%) showed paradoxical septal motion before surgery. But 13 of 34 patients (that is 42%) had paradoxical septal motion after cardiac surgery with cardiopulmonary bypass. So open heart surgery with cardiopulmonary bypass related the development of paradoxical septal motion after surgery. 2) EF significantly decreased in patients who developed paradoxical septal motion after surgery, whereas the EF did not change in the patients who retained normal interventricular septal motion after surgery. So paradoxical septal motion usually reflected some diminution of left ventricular function, immediately after cardiac surgery.

  17. Evaluation of Paradoxical Septal Motion Following Cardiac Surgery with Gated Cardiac Blood Pool Scan

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Seong Hae; Chung, June Key; Lee, Myung Chul; Cho, Bo Youn; Koh, Chang Soon; Suh, Kyung Phil [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1985-03-15

    The development of paradoxical interventricular septal motion is a common consequence of cardiopulmonary bypass operation. The reason for this postoperative abnormal septal motion is not clear. 41 patients were studied preoperatively and postoperatively with radionuclide blood pool scan to evaluate the frequency of development of paradoxical septal motion with right ventricular volume overload before surgery and the frequency of development of paradoxical septal motion after cardiac surgery with cardiopulmonary bypass, and to evaluate the change of EF related to the development of paradoxical septal motion after cardiac surgery. The results were as follows; 1) 7 of 41 patients with right ventricular volume overload (that is 17%) showed paradoxical septal motion before surgery. But 13 of 34 patients (that is 42%) had paradoxical septal motion after cardiac surgery with cardiopulmonary bypass. So open heart surgery with cardiopulmonary bypass related the development of paradoxical septal motion after surgery. 2) EF significantly decreased in patients who developed paradoxical septal motion after surgery, whereas the EF did not change in the patients who retained normal interventricular septal motion after surgery. So paradoxical septal motion usually reflected some diminution of left ventricular function, immediately after cardiac surgery.

  18. Intraoperative device closure of perimembranous ventricular septal defects in the young children under transthoracic echocardiographic guidance; initial experience

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    Cao Hua

    2011-12-01

    Full Text Available Abstract Objectives This study aimed to assess the safety and feasibility of intraoperative device closure of perimembranous ventricular septal defects (VSD in young children guided by transthoracic echocardiography (TTE. Methods We enrolled 18 patients from our hospital to participate in the study from June 2011 to September 2011. A minimal inferior median incision was performed after full evaluation of the perimembranous VSD by real-time TTE, and a domestically made device was inserted to occlude the perimembranous VSD. The proper size of the device was determined by means of transthoracic echocardiographic analysis. Results Implantation was ultimately successful in 16 patients using TTE guidance. In these cases, the complete closure rate immediately following the operation and on subsequent follow-up was 100%. Symmetric devices were used in 14 patients, and asymmetric devices were used in two patients. Two patient were transformed to surgical treatment, one for significant residual shunting, and the other for unsuccessful wire penetration of the VSD. The follow-up periods were less than nine months, and only one patient had mild aortic regurgitation. There were no instances of residual shunt, noticeable aortic regurgitation, significant arrhythmia, thrombosis, or device failure. Conclusions Minimally invasive transthoracic device closure of perimembranous VSDs is safe and feasible, using a domestically made device under transthoracic echocardiographic guidance, without the need for cardiopulmonary bypass. This technique should be considered an acceptable alternative to surgery or device closure guided by transesophageal echocardiography in selected young children. However, a long-term evaluation of outcomes is necessary.

  19. Study on transcatheter ASD occlusion using modified atrial septal defect occluder with no stainless steel screw in canine model

    International Nuclear Information System (INIS)

    Xuan Bin; Qin Yongwen; Hu Jianqiang; Wu Hong

    2006-01-01

    Objective: To evaluate the safety, biocompatibility and efficacy of transcatheter closure of atrial septal defect (ASD) with no stainless-steel-screw occluder in canine model. Methods: The device was constructed from superelastic Nitinol wires tightly woven into two flat disks and sewed with polyester fibers inside, with a pliable loop on the right-atrial-disk of the device, connecting to the delivery cable. ASD was created by transcatheter puncture and balloon dilatation and then closed by occluder under fluoroscopy in the catheterization laboratory. the location and the influence of the implanted device on function of tricuspid valve and mitral valve were evaluated by echocardiography. At 1, 2, 3 and 6 months after the operation, the animals were killed and autopsy was conducted. Results: Eight dogs with puncture-produced ASD underwent ASD closing procedure successfully. the occluder showed no influence on the function of MV and AV demonstrated by echocardiogram. The two disks of the implanted device were covered with a smooth intact neogenesis layer in all dogs. Endocardial cells fully covered the surface of the two disk without inflammating reaction 3 months later. There was no evidence of corrosion on the surface of the nitinol wire removed from the dog after 6 months. Light microscopic examination of the liver, kidney, lung and spleen showed no evidence of embolization and inflammation. Conclusion: Transcatheter ASD occlusion with new-type occluder is safe, feasible, effective and good biocompatibility with a good prospective clinical application. (authors)

  20. Cartilage link protein 1 (Crtl1), an extracellular matrix component playing an important role in heart development.

    Science.gov (United States)

    Wirrig, Elaine E; Snarr, Brian S; Chintalapudi, Mastan R; O'neal, Jessica L; Phelps, Aimee L; Barth, Jeremy L; Fresco, Victor M; Kern, Christine B; Mjaatvedt, Corey H; Toole, Bryan P; Hoffman, Stanley; Trusk, Thomas C; Argraves, W Scott; Wessels, Andy

    2007-10-15

    To expand our insight into cardiac development, a comparative DNA microarray analysis was performed using tissues from the atrioventricular junction (AVJ) and ventricular chambers of mouse hearts at embryonic day (ED) 10.5-11.0. This comparison revealed differential expression of approximately 200 genes, including cartilage link protein 1 (Crtl1). Crtl1 stabilizes the interaction between hyaluronan (HA) and versican, two extracellular matrix components essential for cardiac development. Immunohistochemical studies showed that, initially, Crtl1, versican, and HA are co-expressed in the endocardial lining of the heart, and in the endocardially derived mesenchyme of the AVJ and outflow tract (OFT). At later stages, this co-expression becomes restricted to discrete populations of endocardially derived mesenchyme. Histological analysis of the Crtl1-deficient mouse revealed a spectrum of cardiac malformations, including AV septal and myocardial defects, while expression studies showed a significant reduction in versican levels. Subsequent analysis of the hdf mouse, which carries an insertional mutation in the versican gene (CSPG2), demonstrated that haploinsufficient versican mice display septal defects resembling those seen in Crtl1(-/-) embryos, suggesting that reduced versican expression may contribute to a subset of the cardiac abnormalities observed in the Crtl1(-/-) mouse. Combined, these findings establish an important role for Crtl1 in heart development.

  1. Zero net flux estimates of septal extracellular glucose levels and the effects of glucose on septal extracellular GABA levels

    OpenAIRE

    Krebs-Kraft, Desiree L.; Rauw, Gail; Baker, Glen B.; Parent, Marise B.

    2009-01-01

    Although hippocampal infusions of glucose enhance memory, we have found repeatedly that septal glucose infusions impair memory when γ-aminobutyric acid (GABA) receptors are activated. For instance, hippocampal glucose infusions reverse the memory-impairing effects of co-infusions of the GABA agonist muscimol, whereas septal glucose infusions exacerbate memory deficits produced by muscimol. One potential explanation for these deleterious effects of glucose in the septum is that there are highe...

  2. Molecular Diffusion through Cyanobacterial Septal Junctions.

    Science.gov (United States)

    Nieves-Morión, Mercedes; Mullineaux, Conrad W; Flores, Enrique

    2017-01-03

    Heterocyst-forming cyanobacteria grow as filaments in which intercellular molecular exchange takes place. During the differentiation of N 2 -fixing heterocysts, regulators are transferred between cells. In the diazotrophic filament, vegetative cells that fix CO 2 through oxygenic photosynthesis provide the heterocysts with reduced carbon and heterocysts provide the vegetative cells with fixed nitrogen. Intercellular molecular transfer has been traced with fluorescent markers, including calcein, 5-carboxyfluorescein, and the sucrose analogue esculin, which are observed to move down their concentration gradient. In this work, we used fluorescence recovery after photobleaching (FRAP) assays in the model heterocyst-forming cyanobacterium Anabaena sp. strain PCC 7120 to measure the temperature dependence of intercellular transfer of fluorescent markers. We find that the transfer rate constants are directly proportional to the absolute temperature. This indicates that the "septal junctions" (formerly known as "microplasmodesmata") linking the cells in the filament allow molecular exchange by simple diffusion, without any activated intermediate state. This constitutes a novel mechanism for molecular transfer across the bacterial cytoplasmic membrane, in addition to previously characterized mechanisms for active transport and facilitated diffusion. Cyanobacterial septal junctions are functionally analogous to the gap junctions of metazoans. Although bacteria are frequently considered just as unicellular organisms, there are bacteria that behave as true multicellular organisms. The heterocyst-forming cyanobacteria grow as filaments in which cells communicate. Intercellular molecular exchange is thought to be mediated by septal junctions. Here, we show that intercellular transfer of fluorescent markers in the cyanobacterial filament has the physical properties of simple diffusion. Thus, cyanobacterial septal junctions are functionally analogous to metazoan gap junctions

  3. Gerbode defect: A comprehensive review of its history, anatomy, embryology, pathophysiology, diagnosis, and treatment

    Directory of Open Access Journals (Sweden)

    Erfanul Saker

    2017-10-01

    Full Text Available The purpose of this paper is to survey the literature on Gerbode defect and provide an overview of its history, anatomy, development, pathophysiology, diagnosis, and treatment options. The available literature on this topic, including case reports, was thoroughly reviewed. Gerbode defect is defined as abnormal shunting between the left ventricle and right atrium resulting from either a congenital defect or prior cardiac insults. The pathophysiology underlying the development of Gerbode defect is a disease process that injures the atrioventricular septum and leads to the abnormal shunting of blood. Although the most prevalent cause of Gerbode defect has historically been congenital, an increasing trend towards acquired cases has recently been reported owing to improved diagnostic capabilities and a greater number of invasive cardiac procedures. In conclusion, Gerbode defect is an increasingly recognized condition that warrants further study.

  4. Zero-fluoroscopy cryothermal ablation of atrioventricular nodal re-entry tachycardia guided by endovascular and endocardial catheter visualization using intracardiac echocardiography (Ice&ICE Trial).

    Science.gov (United States)

    Luani, Blerim; Zrenner, Bernhard; Basho, Maksim; Genz, Conrad; Rauwolf, Thomas; Tanev, Ivan; Schmeisser, Alexander; Braun-Dullaeus, Rüdiger C

    2018-01-01

    Stochastic damage of the ionizing radiation to both patients and medical staff is a drawback of fluoroscopic guidance during catheter ablation of cardiac arrhythmias. Therefore, emerging zero-fluoroscopy catheter-guidance techniques are of great interest. We investigated, in a prospective pilot study, the feasibility and safety of the cryothermal (CA) slow-pathway ablation in patients with symptomatic atrioventricular-nodal-re-entry-tachycardia (AVNRT) using solely intracardiac echocardiography (ICE) for endovascular and endocardial catheter visualization. Twenty-five consecutive patients (mean age 55.6 ± 12.0 years, 17 female) with ECG-documentation or symptoms suggesting AVNRT underwent an electrophysiology study (EPS) in our laboratory utilizing ICE for catheter navigation. Supraventricular tachycardia was inducible in 23 (92%) patients; AVNRT was confirmed by appropriate stimulation maneuvers in 20 (80%) patients. All EPS in the AVNRT subgroup could be accomplished without need for fluoroscopy, relying solely on ICE-guidance. CA guided by anatomical location and slow-pathway potentials was successful in all patients, median cryo-mappings = 6 (IQR:3-10), median cryo-ablations = 2 (IQR:1-3). Fluoroscopy was used to facilitate the trans-septal puncture and localization of the ablation substrate in the remaining 3 patients (one focal atrial tachycardia and two atrioventricular-re-entry-tachycardias). Mean EPS duration in the AVNRT subgroup was 99.8 ± 39.6 minutes, ICE guided catheter placement 11.9 ± 5.8 minutes, time needed for diagnostic evaluation 27.1 ± 10.8 minutes, and cryo-application duration 26.3 ± 30.8 minutes. ICE-guided zero-fluoroscopy CA in AVNRT patients is feasible and safe. Real-time visualization of the true endovascular borders and cardiac structures allow for safe catheter navigation during the ICE-guided EPS and might be an alternative to visualization technologies using geometry reconstructions. © 2017 Wiley Periodicals, Inc.

  5. Accuracy of Transthoracic Echocardiography in Assessing Retro-aortic Rim prior to Device Closure of Atrial Septal Defects.

    Science.gov (United States)

    O'Byrne, Michael L; Glatz, Andrew C; Goldberg, David J; Shinohara, Russell; Dori, Yoav; Rome, Jonathan J; Gillespie, Matthew J

    2015-01-01

    Deficient retro-aortic rim has been identified as a risk factor for device erosion following trans-catheter closure of atrial septal defects (ASDs). Transthoracic echocardiography (TTE) is the primary screening method for subjects for possible device closure of ASD, but its reliability in measuring retro-aortic rim size has not been assessed previously. A single-institution cross-sectional analysis of children and adults referred for trans-catheter device closure of single ostium secundum ASD from January 1, 2005 to April 1, 2012 with reviewable TTE and trans-esophageal echocardiogram images was performed. Inter-rater reliability of measurements was tested in a 24% sample. Accuracy of TTE measurement of retro-aortic rim was assessed using a Bland-Altman plot with trans-esophageal echocardiogram measurement as the gold standard. Test characteristics of TTE detection of deficient retro-aortic rim were calculated. Risk factors for misclassification of deficient retro-aortic rim were assessed using receiver operator characteristic curves. Risk factors for measurement error were assessed through multivariate linear regression. In total, 163 subjects of median age 5 years (range: 0.3-46 years) were included. Trans-thoracic echocardiography had 90% sensitivity, 84% specificity, 90% positive predictive value, and 83% negative predictive value to detect deficient retro-aortic rim. Bland-Altman plot demonstrated no fixed bias (P = .23), but errors in measurement increased on average as the aortic rim increased in size (P affect receiver operator characteristic curve area under the curve, nor were any patient-level risk factors independently associated with increased measurement error on TTE. TTE is a sensitive and specific screening test for deficient retro-aortic rim across a range of patient ages and sizes. © 2014 Wiley Periodicals, Inc.

  6. Lah is a transmembrane protein and requires Spa10 for stable positioning of Woronin bodies at the septal pore of Aspergillus fumigatus.

    Science.gov (United States)

    Leonhardt, Yannik; Kakoschke, Sara Carina; Wagener, Johannes; Ebel, Frank

    2017-03-10

    Woronin bodies are specialized, fungal-specific organelles that enable an immediate closure of septal pores after injury to protect hyphae from excessive cytoplasmic bleeding. In most Ascomycetes, Woronin bodies are tethered at the septal pore by so-called Lah proteins. Using the pathogenic mold Aspergillus fumigatus as a model organism, we show that the C-terminal 288 amino acids of Lah (LahC 288 ) bind to the rim of the septal pore. LahC 288 essentially consists of a membrane spanning region and a putative extracellular domain, which are both required for the targeting to the septum. In an A. fumigatus rho4 deletion mutant that has a severe defect in septum formation, LahC 288 is recruited to spot-like structures in or at the lateral membrane. This suggests that LahC is recruited before Rho4 starts to govern the septation process. Accordingly, we found that in wild type hyphae Lah is bound before a cross-wall emerges and thus enables a tethering of Woronin bodies at the site of the newly formed septum. Finally, we identified Spa10, a member of a recently described family of septal pore-associated proteins, as a first protein that directly or indirectly interacts with LahC to allow a stable positioning of Woronin bodies at the mature septum.

  7. Variation in practice patterns in device closure of atrial septal defects and patent ductus arteriosus: An analysis of data from the IMproving Pediatric and Adult Congenital Treatment (IMPACT) registry.

    Science.gov (United States)

    O'Byrne, Michael L; Kennedy, Kevin F; Rome, Jonathan J; Glatz, Andrew C

    2018-02-01

    Practice variation is a potentially important measure of healthcare quality. The IMPACT registry provides a representative national sample with which to study practice variation in trans-catheter interventions for congenital heart disease. We studied cases for closure of atrial septal defect (ASD) and patent ductus arteriosus (PDA) in IMPACT between January 1, 2011, and September 30, 2015, using hierarchical multivariate models studying (1) the distribution of indications for closure and (2) in patients whose indication for closure was left (LVVO) or right ventricular volume overload (RVVO), the factors influencing probability of closure of a small defect (either in size or in terms of the magnitude of shunt). Over the study period, 5233 PDA and 4459 ASD cases were performed at 77 hospitals. The indications for ASD closure were RVVO in 84% and stroke prevention in 13%. Indications for PDA closure were LVVO in 57%, endocarditis prevention in 36%, and pulmonary hypertension in 7%. There was statistically significant variability in indications between hospitals for PDA and ASD procedures (median rate ratio (MRR): 1.3 and 1.1; both Pclosure of PDA and ASD. Further research is necessary to study whether this affects outcomes or resource utilization. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. Spectrum Of Congenital Heart Disease In Full Term Neonates.

    Science.gov (United States)

    Bibi, Saima; Hussain Gilani, Syed Yasir; Bibi, Shawana

    2018-01-01

    Congenital heart disease is a significant problem world over especially in neonates. Early diagnosis and prompt interventions in neonatal period precludes the mortality associated with this disorder. The objective of this study was to highlight the diversity of congenital cardiac defects in our region so that appropriate interventions are devised to minimize significant morbidity and mortality associated with this disorder. This descriptive cross-sectional study was conducted at the Neonatology Unit of Department of Paediatrics, Ayub Teaching Hospital from January 2015 to December 2016. Approval of ethical committee was taken. All fullterm neonates of either gender who presented in department of neonatology including those delivered in hospital or received from other sources (private settings, home deliveries), diagnosed as having congenital heart disease on echocardiography were included in the study. Preterm neonates of either gender were excluded from the study. Patient characteristics were recorded in a designed proforma. Data was entered in SPSS version 20 and analysed. A total of 89 neonates were included in the study. Mean age of presentation was 6.34±7.058 days and range of 1-28 days. There was a male preponderance with 57 (64%) male patients as compared to 32 (36%) female patients. Ventricular septal defect (VSD) was the commonest cardiac lesion being present in 34 (38.2%) patients. Other defects included complex congenital heart disease in 8 (9%), atrial septal defect (ASD) and transposition of great arteries (TGA) in 7 (7.9%) each, atrioventricular septal defect (AVSD) in 6 (6.7%) and Fallots's tetralogy (TOF) and hypoplastic left heart syndrome in 5 (5.6%) each.. Congenital heart disease is a problem of profound importance. It constitutes approximately one third of the total major congenital malformations. There is a diversity of cardiac lesions in our region that warrant early and prompt interventions so that the disease is recognized and treated at

  9. Three-dimensional image analysis of plugging at the septal pore by Woronin body during hypotonic shock inducing hyphal tip bursting in the filamentous fungus Aspergillus oryzae

    International Nuclear Information System (INIS)

    Maruyama, Jun-ichi; Juvvadi, Praveen Rao; Ishi, Kazutomo; Kitamoto, Katsuhiko

    2005-01-01

    We observed that the filamentous fungus, Aspergillus oryzae, grown on agar media burst out cytoplasmic constituents from the hyphal tip soon after flooding with water. Woronin body is a specialized organelle known to plug the septal pore adjacent to the lysed compartment to prevent extensive loss of cytoplasm. A. oryzae Aohex1 gene homologous to Neurospora crassa HEX1 gene encoding a major protein in Woronin body was expressed as a fusion with DsRed2, resulting in visualization of Woronin body. Confocal microscopy and three-dimensional reconstruction of images visualized the septal pore as a dark region surrounded by green fluorescence of EGFP-fused secretory protein, RNase T1, on the septum. Dual fluorescent labeling revealed the plugging of the septal pores adjacent to the lysed apical compartments by Woronin bodies during hypotonic shock. Disruption of Aohex1 gene caused disappearance of Woronin bodies and the defect to prevent extensive loss of cytoplasm during hypotonic shock

  10. How Do We Define Congenital Heart Defects for Scientific Studies?

    DEFF Research Database (Denmark)

    Garne, Ester; Olsen, Morten Smaerup; Johnsen, Søren Paaske

    2012-01-01

    of echocardiography in neonatal intensive care, a patent ductus arteriosus (PDA) or flow over the atrial septum will often be visible. These findings may be coded as CHD at discharge and in this way falsely increase the CHD prevalence in the population. There are several purposes for which population-based data...... practice. We include PDA and atrial septal defects as CHD cases if these defects are still open 2 months after birth. International consensus on how to define CHD would improve the validity and comparability of epidemiological studies on CHD....

  11. Avaliação pós-operatória imediata da influência da desinserção da valva tricúspide no tratamento da comunicação interventricular

    Directory of Open Access Journals (Sweden)

    Francisco Fernandes MOREIRA NETO

    1998-10-01

    ão afeta a competência da valva tricúspide nem aumenta a incidência de comunicação interventricular residual ou bloqueio atrioventricular.Although the tricuspid valve and its attachments can sometimes obscure the margins of the ventricular septal defect from the vision of the surgeon, concern for valvular competence has made surgeons hesitate to take down the tricuspid valve. In this paper, the records of 34 patients divided into two groups (comparable by gender, age and associated anomalies were reviewed retrospectively. Treatment of the ventricular septal defect was done without (Group I, with 19 patients and with (Group II, with 15 patients takedown of the tricuspid valve to determine the degree of the tricuspid regurgitation and also the incidence of residual ventricular septal defects and atrioventricular block. The anterior and septal leaflets of the valve were taken down at the annulus and resuspended after VSD repair with running 6-0 polypropylene sutures. The degree of valvular regurgitation and the presence of residual VSD were determined by echo done on post-operative day one and at the the patient's discharge. Valvular regurgitation was graded as none in 12 patients of the Group I and in 10 patients of the Group II; trivial regurgitation in 5 patients of Group I and 4 of Group II; mild to moderate regurgitation in 2 cases of Group I and 1 in Group II. No patient had severe regurgitation. Even with a small number of cases, there was no statistically significant difference between the groups. There was only one case of residual VSD in Group I with spontaneous closure. The mortality in Group I was 10.5% and 6.6% in Group II. The authors conclude that takedown and re-suspension of the tricuspid valve in VSD repair surgery does not adversely affect valve competence neither increases the incidence of residual VSD or the incidence of A-V block.

  12. Maternal steroid therapy for fetuses with second-degree immune-mediated congenital atrioventricular block: a systematic review and meta-analysis.

    Science.gov (United States)

    Ciardulli, Andrea; D'Antonio, Francesco; Magro-Malosso, Elena R; Manzoli, Lamberto; Anisman, Paul; Saccone, Gabriele; Berghella, Vincenzo

    2018-03-07

    To explore the effect of maternal fluorinated steroid therapy on fetuses affected by second-degree immune-mediated congenital atrioventricular block. Studies reporting the outcome of fetuses with second-degree immune-mediated congenital atrioventricular block diagnosed on prenatal ultrasound and treated with fluorinated steroids compared with those not treated were included. The primary outcome was the overall progression of congenital atrioventricular block to either continuous or intermittent third-degree congenital atrioventricular block at birth. Meta-analyses of proportions using random effect model and meta-analyses using individual data random-effect logistic regression were used. Five studies (71 fetuses) were included. The progression rate to congenital atrioventricular block at birth in fetuses treated with steroids was 52% (95% confidence interval 23-79) and in fetuses not receiving steroid therapy 73% (95% confidence interval 39-94). The overall rate of regression to either first-degree, intermittent first-/second-degree or sinus rhythm in fetuses treated with steroids was 25% (95% confidence interval 12-41) compared with 23% (95% confidence interval 8-44) in those not treated. Stable (constant) second-degree congenital atrioventricular block at birth was present in 11% (95% confidence interval 2-27) of cases in the treated group and in none of the newborns in the untreated group, whereas complete regression to sinus rhythm occurred in 21% (95% confidence interval 6-42) of fetuses receiving steroids vs. 9% (95% confidence interval 0-41) of those untreated. There is still limited evidence as to the benefit of administered fluorinated steroids in terms of affecting outcome of fetuses with second-degree immune-mediated congenital atrioventricular block. © 2018 Nordic Federation of Societies of Obstetrics and Gynecology.

  13. Atrioventricular Canal Defect

    Science.gov (United States)

    ... viral illness during a mother's early pregnancy Alcohol consumption during pregnancy Poorly controlled diabetes during pregnancy Smoking during pregnancy Certain medications taken during pregnancy — talk to your doctor before taking any drugs while you're pregnant or trying to become ...

  14. Transcatheter device closure of multiple defects in ruptured sinus of Valsalva aneurysm

    Directory of Open Access Journals (Sweden)

    A. Vamsidhar

    2015-12-01

    Full Text Available Ruptured sinus of Valsalva aneurysm (SOVA with multiple communications, ventricular septal defect, and aortic regurgitation are still best treated surgically. We report a case of 30-year-old male with right SOVA, with two communications with right ventricle. Both communications were successfully closed using antegrade and retrograde approaches, respectively.

  15. Transcatheter closure of post-operative residual ventricular septal defect using a patent ductus arteriosus closure device in an adult: a case report.

    Science.gov (United States)

    Djer, Mulyadi M; Idris, Nikmah S; Alwi, Idrus; Wijaya, Ika P

    2014-07-01

    Transcatheter closure of perimembranous and muscular ventricular septal defect (VSD) has been performed widely and it has more advantages compare to surgery. However, transcatheter closure of residual VSD post operation of complex congenital heart disease is still challenging because of the complexity of anatomy and concern about device stability, so the operator should meticulously choose the most appropriate technique and device. We would like to report a case of transcatheter closure of residual VSD post Rastelli operation in a patient with double outlet right ventricle (DORV), sub-aortic VSD, severe infundibulum pulmonary stenosis (PS) and single coronary artery. The patient had undergone operations for four times, but he still had intractable heart failure that did not response to medications. On the first attempt. we closed the VSD using a VSD occluder, unfortunately the device embolized into the descending aorta, but fortunately we was able to snare it out. Then we decided to close the VSD using a patent ductus arteriosus (PDA occluder). On transesophageal echocardiography (TEE) and angiography evaluation, the device position was stable. Post transcatheter VSD closure, the patient clinical condition improved significantly and he could finally be discharged after a long post-surgery hospitalization. Based on this experience we concluded that the transcatheter closure of residual VSD in complex CHD using PDA occluder could be an effective alternative treatment.

  16. Autoshaping a leverpress in rats with lateral, medial, or complete septal lesions.

    Science.gov (United States)

    Poplawsky, A; Phillips, C L

    1986-05-01

    Rats with either control operations or lateral, medial, or complete septal lesions received 600 trials of leverpress training using an autoshaping procedure, i.e., food delivery followed a 10 s illuminated lever presentation, or occurred immediately after a leverpress. Rats with complete septal lesions acquired the leverpress faster than controls and had more food-tray entries per minute during the first 100 trials than the other groups. Rats with lateral or medial septal lesions had leverpress and food-tray entries equivalent to controls. The facilitation of autoshaping a leverpress may partially be explained by the general increase in motor reactivity to stimuli found following septal lesions.

  17. Warden repair for superior sinus venosus atrial septal defect and anomalous pulmonary venous drainage in children: Anesthesia and transesophageal echocardiography perspective

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    Neelam Aggarwal

    2016-01-01

    Full Text Available Objective: Review of intraoperative anesthetic challenges and the role of transesophageal echocardiography in children with sinus venosus atrial septal defect and partial anomalous pulmonary venous drainage undergoing Warden repair. Design: A retrospective observational case series. Methodolgy: Pediatric patients who underwent Warden repair between October 2011-September 2015 were recruited. Their preoperative clinical details, anesthetic techniques, intraoperative TEE findings and postoperative events were recorded from the medical records. The categorical variables and the continuous variables were expressed as number (percentages and mean ΁ SD respectively. Results: A total of 35 patients were operated for Warden repair during the study period. Anesthesia was induced with the aim to prevent any fall in pulmonary vascular resistance. The right internal jugular vein was cannulated under ultrasound guidance using a short length cannula to monitor right superior vena cava pressure. Intraoperative TEE revealed the drainage of PAPVC high into RSVC in 22 patients. Persistent LSVC was found in 9 patients. After repair, TEE imaging detected a high gradient at Warden anastomotic site in 5 patients and 3 of them required revision of surgery. Rerouted pulmonary veins required surgical correction in 2 patients in view of obstruction. None of them had pulmonary venous and SVC obstruction in the postoperative period. Conclusion: The primary aim of anesthesia is to avoid any fall in PVR. Right IJV cannulation can be beneficial. The intraoperative TEE can help in delineating the anatomy of lesion and detecting anastomotic site obstruction.

  18. Diprosopus with multiple craniofacial, musculoskeletal, and cardiac defects in a purebred Suffolk lamb.

    Science.gov (United States)

    Kerr, Nancy J

    2007-10-01

    A stillborn Suffolk ewe lamb with 2 heads was found to have tarsal arthrogryposis, kypholordosis, craniorachischisis totalis, cheiloschisis, palatoschisis, deviation of the right maxilla, prognathia, patent ductus arteriosus, 3 ventricular septal defects, and 4 great vessels. The lamb was born twin to a normal lamb. No definitive etiology was established.

  19. Diprosopus with multiple craniofacial, musculoskeletal, and cardiac defects in a purebred Suffolk lamb

    OpenAIRE

    Kerr, Nancy J.

    2007-01-01

    A stillborn Suffolk ewe lamb with 2 heads was found to have tarsal arthrogryposis, kypholordosis, craniorachischisis totalis, cheiloschisis, palatoschisis, deviation of the right maxilla, prognathia, patent ductus arteriosus, 3 ventricular septal defects, and 4 great vessels. The lamb was born twin to a normal lamb. No definitive etiology was established.

  20. Magnetic resonance imaging in complete type endocardial cushion defect

    International Nuclear Information System (INIS)

    Li Kuncheng; Yang Xiaoping; Yao Xinyu; Yang Yunxia; Liu Yuqing; Pang Zhixian

    1999-01-01

    Objective: To evaluate the value and limitation of magnetic resonance imaging (MRI) in complete type endocardial cushion defect (ETECD). Methods: Eight patients (male 5, female 3) were scanned by MRI with ECG-gated spin echo (SE) and gradient echo (GE) cine techniques. The diagnosis of CTECD was made by X-ray plain film, echocardiography, X-ray right cardiac catheterization and angiocardiography, as well as MRI four patients were treated with operation. Results: Every chamber of the heart was enlarged, more serious in the right atrium and ventricle, with thickening of right ventricular wall on MRI in majority of cases. The endocardial cushion disappeared, so the four chambers of the heart were directly connected with each other, and presented a cross shape. Ventricular septal membrane defect and type I atrial septal defect were depicted also. The normal structure of mitral and tricuspid valves were not intact. There were 6 cases with pulmonary hypertension, 2 cases with Tetralogy of Fallot, 4 cases with right aorta arch and descending aorta, 1 patient with dextroverted heart and another with persistent left superior vena cava connected with coronary sinus. The cine MRI could directly depict the situation of left-right or right-left shunt at atrial and ventricular levels, and regurgitation from ventricles to atria, meanwhile the valves were depicted clearly. Conclusions: MRI can clearly demonstrate all anatomic deformation and complicated hemodynamic change of CTECD, as well as other coexistent deformations

  1. Clinically oriented device programming in bradycardia patients: part 2 (atrioventricular blocks and neurally mediated syncope). Proposals from AIAC (Italian Association of Arrhythmology and Cardiac Pacing).

    Science.gov (United States)

    Palmisano, Pietro; Ziacchi, Matteo; Biffi, Mauro; Ricci, Renato P; Landolina, Maurizio; Zoni-Berisso, Massimo; Occhetta, Eraldo; Maglia, Giampiero; Botto, Gianluca; Padeletti, Luigi; Boriani, Giuseppe

    2018-04-01

    : The purpose of this two-part consensus document is to provide specific suggestions (based on an extensive literature review) on appropriate pacemaker setting in relation to patients' clinical features. In part 2, criteria for pacemaker choice and programming in atrioventricular blocks and neurally mediate syncope are proposed. The atrioventricular blocks can be paroxysmal or persistent, isolated or associated with sinus node disease. Neurally mediated syncope can be related to carotid sinus syndrome or cardioinhibitory vasovagal syncope. In sinus rhythm, with persistent atrioventricular block, we considered appropriate the activation of mode-switch algorithms, and algorithms for auto-adaptive management of the ventricular pacing output. If the atrioventricular block is paroxysmal, in addition to algorithms mentioned above, algorithms to maximize intrinsic atrioventricular conduction should be activated. When sinus node disease is associated with atrioventricular block, the activation of rate-responsive function in patients with chronotropic incompetence is appropriate. In permanent atrial fibrillation with atrioventricular block, algorithms for auto-adaptive management of the ventricular pacing output should be activated. If the atrioventricular block is persistent, the activation of rate-responsive function is appropriate. In carotid sinus syndrome, adequate rate hysteresis should be programmed. In vasovagal syncope, specialized sensing and pacing algorithms designed for reflex syncope prevention should be activated.

  2. The use of non-invasive fetal electrocardiography in diagnosing second-degree fetal atrioventricular block.

    Science.gov (United States)

    Lakhno, Igor; Behar, Joachim A; Oster, Julien; Shulgin, Vyacheslav; Ostras, Oleksii; Andreotti, Fernando

    2017-01-01

    Complete atrioventricular block in fetuses is known to be mostly associated with autoimmune disease and can be irreversible if no steroids treatment is provided. Conventional methods used in clinical practice for diagnosing fetal arrhythmia are limited since they do not reflect the primary electrophysiological conduction processes that take place in the myocardium. The non-invasive fetal electrocardiogram has the potential to better support fetal arrhythmias diagnosis through the continuous analysis of the beat to beat variation of the fetal heart rate and morphological analysis of the PQRST complex. We present two retrospective case reports on which atrioventricular block diagnosis could have been supported by the non-invasive fetal electrocardiogram. The two cases comprised a 22-year-old pregnant woman with the gestational age of 31 weeks and a 25-year-old pregnant woman with the gestational age of 41 weeks. Both women were admitted to the Department of Maternal and Fetal Medicine at the Kyiv and Kharkiv municipal perinatal clinics. Patients were observed using standard fetal monitoring methods as well as the non-invasive fetal electrocardiogram. The non-invasive fetal electrocardiographic recordings were analyzed retrospectively, where it is possible to identify the presence of the atrioventricular block. This study demonstrates, for the first time, the feasibility of the non-invasive fetal electrocardiogram as a supplementary method to diagnose of the fetal atrioventricular block. Combined with current fetal monitoring techniques, non-invasive fetal electrocardiography could support clinical decisions.

  3. Experiência inicial no fechamento percutâneo da comunicação interatrial com a prótese de Amplatzer Initial experience in percutaneous occlusion of atrial septal defects with the Amplatzer device

    Directory of Open Access Journals (Sweden)

    Valmir F. Fontes

    1998-03-01

    Full Text Available OBJETIVO: Analisar a experiência inicial no fechamento percutâneo da comunicação interatrial ostium secundum (CIA OS com a prótese de Amplatzer. MÉTODOS: Sete pacientes foram submetidos ao procedimento através da via venosa anterógrada, orientados pela ecocardiografia transesofágica (ETE e sob anestesia geral. Uma criança era portadora de 2 CIA e de canal arterial (CA. As CIA medidas pelo ETE variaram de 8,7 a 20mm. Um ecocardiograma transtorácico foi realizado na manhã seguinte do procedimento. RESULTADOS: Oito próteses foram implantadas nos 7 pacientes com sucesso. Em um paciente, o CA foi ocluído na mesma sessão com mola de Gianturco, tendo surgido taquicardia supraventricular durante a oclusão de uma das CIA, controlada com adenosina. Todos receberam alta hospitalar na manhã seguinte, com oclusão total dos defeitos. CONCLUSÃO: O procedimento mostrou-se seguro, eficaz e versátil, podendo ser considerado como uma alternativa terapêutica inicial em pacientes selecionados com CIA OS.PURPOSE: To evaluate our initial experience with percutaneous closure of secundum type atrial septal defects (ASD with the Amplatzer septal occluder. METHODS: Seven patients underwent occlusion by anterograde approach, under general anesthesia and transesophageal echocardiography (TEE guidance. One child had 2 ASD and a patent ductus arteriosus (PDA. The ASD size ranged from 8,7 to 20mm as measured by TEE. A transthoracic echocardiogram was performed in the morning after the procedure. RESULTS: Eight devices were successfully implanted in 7 patients and the PDA was occluded with a Gianturco coil at the same session. In this patient, there was an episode of supraventricular tachycardia during the occlusion of one ASD which was reverted with adenosin. All patients were discharged the day after, with complete occlusion of all defects. CONCLUSION: The procedure is safe, effective and versatile. It can be applied as an initial alternative to the

  4. Correção simultânea de defeito congênito intracardíaco e pectus excavatum Simultaneous repair of congenital heart defect and pectus excavatum

    Directory of Open Access Journals (Sweden)

    João Roberto Breda

    2007-09-01

    Full Text Available Relatamos tratamento simultâneo de pectus excavatum e defeito congênito intracardíaco representado por comunicação interatrial ostium secundum. Paciente do sexo masculino, 8 anos de idade, com diagnóstico clínico e ecocardiográfico de comunicação interatrial, associada à deformidade da parede torácica tipo pectus excavatum. Foi encaminhado para operação com correção simultânea do defeito congênito intracardíaco associado ao reparo do pectus. O tratamento operatório simultâneo do pectus excavatum e defeitos congênitos intracardíacos torna difícil o acesso ao coração. Foi feita a correção simultânea dessas alterações, com satisfatório resultado, sobretudo estético, para o paciente.The author describes the simultaneous treatment of pectus excavatum and congenital intracardiac defect (atrial septal defect represented by the interatrial foramen secundum. An 8-year-old boy, with clinical and echocardiography diagnosis of atrial septal defect associated with pectus excavatum was referred to a simultaneous surgical treatment of both abnormalities. The simultaneous surgical treatment of both pectus excavatum and congenital intracardiac defects make it difficult to access the heart. In this case, the simultaneous surgical treatment of atrial septal defect and pectus excavatum was a valuable alternative to surgical repair of both abnormalities, mainly due to its cosmetic outcome.

  5. [The nonpharmacological treatment of tachyarrhythmias. The surgery of supraventricular arrhythmias not due to pre-excitation].

    Science.gov (United States)

    Viganò, M; Graffigna, A; Pagani, F; Salerno, J A

    1991-12-01

    Surgery can provide treatment for supraventricular tachyarrhythmias without operative risk and with a definite improvement of patients' quality of life. Ectopic atrial tachycardia is a rare but invalidating arrhythmia that may lead to cardiomyopathy: intraoperative mapping is necessary for the location of the location of the ectopic focus; in our experience, surgical, cryothermal or isolation ablation of the area were effective in 9 patients out of 9, with regression of the cardiomyopathy. Atrioventricular node reentry tachycardia is a reentry tachycardia which is often associated with Wolff-Parkinson-White syndrome and is amenable to surgical treatment. Discrete cryolesions around the Koch triangle are effective in interrupting the atrial inputs to the atrioventricular node and therefore the reentry mechanism: in our experience, 7 patients were successfully treated without operative mortality: 3 patients underwent surgical ablation of Kent bundles as well. Atrial fibrillation is not based on a well-defined mechanism, and therefore does not permit an electrically-guided surgical treatment. However, in case of atrial septal defect it is possible to isolate the enlarged right atrium in order to allow sinus rhythm to activate the left atrium and ventricles.(ABSTRACT TRUNCATED AT 250 WORDS)

  6. NMDA-dependent phase synchronization between septal and temporal CA3 hippocampal networks.

    Science.gov (United States)

    Gu, Ning; Jackson, Jesse; Goutagny, Romain; Lowe, Germaine; Manseau, Frédéric; Williams, Sylvain

    2013-05-08

    Increasing evidence suggests that synchronization between brain regions is essential for information exchange and memory processes. However, it remains incompletely known which synaptic mechanisms contribute to the process of synchronization. Here, we investigated whether NMDA receptor-mediated synaptic plasticity was an important player in synchronization between septal and temporal CA3 areas of the rat hippocampus. We found that both the septal and temporal CA3 regions intrinsically generate weakly synchronized δ frequency oscillations in the complete hippocampus in vitro. Septal and temporal oscillators differed in frequency, power, and rhythmicity, but both required GABAA and AMPA receptors. NMDA receptor activation, and most particularly the NR2B subunit, contributed considerably more to rhythm generation at the temporal than the septal region. Brief activation of NMDA receptors by application of extracellular calcium dramatically potentiated the septal-temporal coherence for long durations (>40 min), an effect blocked by the NMDA antagonist AP-5. This long-lasting NMDA-receptor-dependent increase in coherence was also associated with an elevated phase locking of spikes locally and across regions. Changes in coherence between oscillators were associated with increases in phase locking between oscillators independent of oscillator amplitude. Finally, although the septal CA3 rhythm preceded the oscillations in temporal regions in control conditions, this was reversed during the NMDA-dependent enhancement in coherence, suggesting that NMDA receptor activation can change the direction of information flow along the septotemporal CA3 axis. These data demonstrate that plastic changes in communication between septal and temporal hippocampal regions can arise from the NMDA-dependent phase locking of neural oscillators.

  7. Complete paroxysmal atrioventricular block in a 2-year-old girl

    DEFF Research Database (Denmark)

    Holst, Line Marie; Dixen, Ulrik; Jeppesen, Dorthe L

    2015-01-01

    We present a case of atypical syncope in a 2-year-old, otherwise healthy girl. The patient presented with three episodes of syncope without any precipitating factors and no family history of sudden unexpected death. Holter monitoring revealed 24 events of complete atrioventricular block lasting up...

  8. Fabrication and characterization of chitosan nanoparticles and collagen-loaded polyurethane nanocomposite membrane coated with heparin for atrial septal defect (ASD) closure.

    Science.gov (United States)

    Kaiser, Eva; Jaganathan, Saravana Kumar; Supriyanto, Eko; Ayyar, Manikandan

    2017-07-01

    Atrial septal defect (ASD) constitutes 30-40% of all congenital heart diseases in adults. The most common complications in the treatment of ASD are embolization of the device and thrombosis formation. In this research, an occluding patch was developed for ASD treatment using a well-known textile technology called electrospinning. For the first time, a cardiovascular occluding patch was fabricated using medical grade polyurethane (PU) loaded with bioactive agents namely chitosan nanoparticles (Cn) and collagen (Co) which is then coated with heparin (Hp). Fourier transform infrared spectrum showed characteristic vibrations of several active constituents and changes in the absorbance due to the inclusion of active ingredients in the patch. The contact angle analysis demonstrated no significant decrease in contact angle compared to the control and the composite patches. The structure of the electrospun nanocomposite (PUCnCoHp) was examined through scanning electron microscopy. A decrease in nanofiber diameter between control PU and PUCnCoHp nanocomposite was observed. Water uptake was found to be decreased for the PUCnCoHp nanocomposite against the control. The hemocompatibility properties of the PUCnCoHp ASD occluding patch was inferred through in vitro hemocompatibility tests like activated partial thromboplastin time (APTT), prothrombin time (PT) and hemolysis assay. It was found that the PT and APTT time was significantly prolonged for the fabricated PUCnCoHp ASD occluding patch compared to the control. Likewise, the hemolysis percentage was also decreased for the PUCnCoHp ASD patch against the control. In conclusion, the developed PUCnCoHp patch demonstrates potential properties to be used for ASD occlusion.

  9. O efeito da morfina na inibição vagal do coração: demonstração da dupla via do nodo atrioventricular - DOI: 10.4025/actascihealthsci.v28i2.1102 The effect of morphine on vagal inhibition of the heart: demonstration of dual atrioventricular nodal pathways - DOI: 10.4025/actascihealthsci.v28i2.1102

    Directory of Open Access Journals (Sweden)

    José Geraldo Pereira da Cruz

    2006-03-01

    Full Text Available A estimulação elétrica do nervo vago diminui a freqüência cardíaca e aumenta o intervalo PQ no eletrocardiograma, podendo ativar a via anterógrada do nodo atrioventricular. Neste experimento é investigada a interação da morfina sobre o controle nervoso parassimpático cardíaco em relação à dupla condução do nodo atrioventricular. A estimulação do nervo vago induz uma significativa bradicardia e um bloqueio atrioventricular de terceiro grau, com a onda P aparecendo negativa na derivação II. Devido aos efeitos moduladores da anestesia sobre o coração, administração de injeções de morfina induz o aparecimento de onda P positiva através de uma ação inibitória sobre a reentrância do nodo atrioventricular. Tudo sugere a presença de duas vias atrioventriculares, anatômica e funcionalmente distintasThat electrical stimulation of the vagus decreased heart rate and increased the PQ interval in electrocardiogram, consistent with anterograde dual atriventricular conduction. The present experiments were investigating the interaction of the morphine with cardiac parasympathetic nervous control in dual atrioventricular node. Electrical stimulation of the vagus nerve induced significant bradycardia and of third degree atrioventricular block and the P wave appearance was negative in leads II. Because background anesthesia modulates the heart, administered morphine injection the P waves appearance was positive through one inhibitory action on atrioventricular nodal reentry. All suggest the presence of two functionally and anatomically distinct atrioventricular pathways

  10. Echocardiographic effects of changing atrioventricular delay in cardiac resynchronization therapy based on displacement

    DEFF Research Database (Denmark)

    Valeur, Nana; Fritz-Hansen, Thomas; Risum, Niels

    2010-01-01

    In studies showing benefits of cardiac resynchronization therapy (CRT), individual atrioventricular (AV) delays have been optimized using echocardiography. However, the method for AV delay optimization remains controversial....

  11. Atrioventricular node reentrant tachycardia (AVNRT) after mitral valvuloplasty during cardiac rehabilitation.

    Science.gov (United States)

    Fallavollita, Luca; Santillo, Elpidio; Marini, Luciano; Balestrini, Fabrizio

    2012-12-01

    We descrive a patient who presents palpitations during cardiac rehabilitation after mitral valvuloplasty. ECG showed regular narrow QRS tachycardia compatible with Atrioventricular Node Reentrant Tachycardia. After slow pathway radiofrequency catheter ablation, the patient completed the rehabilitation program remained tachycardia and palpitations-free.

  12. Flail Tricuspid Valve in an Adult Patient with Congenitally Corrected Transposition of the Great Arteries.

    Science.gov (United States)

    Meloni, Luigi; Abbruzzese, Piero A.; Pirisi, Raimondo; Cherchi, Angelo

    1997-01-01

    We describe a case of a 50-year-old woman with congenitally corrected transposition of the great vessels, in whom severe left-sided tricuspid (systemic atrioventricular) valve insufficiency was the only associated anomaly. The tricuspid valve was dysplastic and abnormally oriented toward the interventricular septum, without the downward displacement of Ebstein's anomaly. The mechanism of atrioventricular regurgitation was unusual in that it consisted of the rupture of chordae tendineae of both the anterior and septal leaflets. The left-sided tricuspid valve was replaced with a St. Jude prosthesis and the postoperative course was uneventful.

  13. Comparison of human septal nuclei MRI measurements using automated segmentation and a new manual protocol based on histology

    Science.gov (United States)

    Butler, Tracy; Zaborszky, Laszlo; Pirraglia, Elizabeth; Li, Jinyu; Wang, Xiuyuan Hugh; Li, Yi; Tsui, Wai; Talos, Delia; Devinsky, Orrin; Kuchna, Izabela; Nowicki, Krzysztof; French, Jacqueline; Kuzniecky, Rubin; Wegiel, Jerzy; Glodzik, Lidia; Rusinek, Henry; DeLeon, Mony J.; Thesen, Thomas

    2014-01-01

    Septal nuclei, located in basal forebrain, are strongly connected with hippocampi and important in learning and memory, but have received limited research attention in human MRI studies. While probabilistic maps for estimating septal volume on MRI are now available, they have not been independently validated against manual tracing of MRI, typically considered the gold standard for delineating brain structures. We developed a protocol for manual tracing of the human septal region on MRI based on examination of neuroanatomical specimens. We applied this tracing protocol to T1 MRI scans (n=86) from subjects with temporal epilepsy and healthy controls to measure septal volume. To assess the inter-rater reliability of the protocol, a second tracer used the same protocol on 20 scans that were randomly selected from the 72 healthy controls. In addition to measuring septal volume, maximum septal thickness between the ventricles was measured and recorded. The same scans (n=86) were also analysed using septal probabilistic maps and Dartel toolbox in SPM. Results show that our manual tracing algorithm is reliable, and that septal volume measurements obtained via manual and automated methods correlate significantly with each other (pautomated methods detected significantly enlarged septal nuclei in patients with temporal lobe epilepsy in accord with a proposed compensatory neuroplastic process related to the strong connections between septal nuclei and hippocampi. Septal thickness, which was simple to measure with excellent inter-rater reliability, correlated well with both manual and automated septal volume, suggesting it could serve as an easy-to-measure surrogate for septal volume in future studies. Our results call attention to the important though understudied human septal region, confirm its enlargement in temporal lobe epilepsy, and provide a reliable new manual delineation protocol that will facilitate continued study of this critical region. PMID:24736183

  14. Nasal septal angiofibroma, a subclass of extranasopharyngeal angiofibroma.

    Science.gov (United States)

    Garcia-Rodriguez, Laura; Rudman, Kelli; Cogbill, Christopher H; Loehrl, Todd; Poetker, David M

    2012-01-01

    Extranasopharyngeal angiofibromas (ENA) arising from the nasal septum or nasal septal angiofibromas are extremely rare; only 13 such cases have been reported in the international literature. Our objective is to describe the presentation, workup, and surgical management of these lesions. Case reports were done. The setting was a tertiary care referral center and the Veterans Affairs Medical Center. PATIENTS, INTERVENTIONS, AND RESULTS: We present 2 cases of extranasopharyngeal angiofibroma occurring on the nasal septum. In this report, we discuss the occurrence, the histopathologic findings, and the treatment of nasal septal angiofibroma. Copyright © 2012 Elsevier Inc. All rights reserved.

  15. Delayed ventricular septal rupture complicating acute inferior wall myocardial infarction

    OpenAIRE

    Cho, Jae Hyung; Sattiraju, Srinivasan; Mehta, Sanjay; Missov, Emil

    2013-01-01

    Background Ventricular septal rupture is a potentially fatal complication of acute myocardial infarction. Its incidence has declined with modern reperfusion therapy. In the era of percutaneous coronary interventions, it occurs a median of 18?24?hours after myocardial infarction and is most commonly associated with anterior myocardial infarction. We present a case of delayed ventricular septal rupture complicating acute inferior wall myocardial infarction. Case presentation A 53-year-old Cauca...

  16. Morfologia da junção atrioventricular em Iguana iguana (Reptilia-Iguanidae

    Directory of Open Access Journals (Sweden)

    Sonia Regina Jurado

    2006-06-01

    Full Text Available The atrioventricular junctional area (AVJA, including atrioventricular (AV node and bundle was investigated in seven hearts of common or green iguana (Iguana iguana using the light microscopy. Adult animals, both sexes, were captured in the Pantanal, Brazil. All hearts were fixed in buffered formaldehyde 10% (pH 7.2 for 24 hours, embedded in paraplast according to routine methods, and serially cut at 5 µm thickness. In the Iguana iguana, the AVJA consists of a mass of the fibers intermingled with variable amount of connective tissue and blood vessels surrounded by adjacent myocardium and the attachment of the right atrioventricular valve in the fibrous skeleton. By light microscopy, conducting cells of the AV node and bundle can be distinguished from working cells by their much smaller size, paler staining reaction and the presence of a sheath of connective tissue. The AV node and bundle and its branches were found to constitute a continuous tract. Histochemically, we found elastic fibers between cells of the conduction, mainly in the AV node. The PAS method reveals absence of glycogen in specialized cells. The fibrous skeleton, mainly the right trigone, showed a well-developed chondroid tissue, made by hyaline like cartilage (binucleated condrocytes included in the big lacunas and extracellular matrix with fibrillar collagen. In conclusion, the nodal and Purkinje cells in heart iguana presented poorly morphological differentiation comparing mammals and birds, however the skeleton fibrous has a different cartilage kind.

  17. Functional study of DAND5 variant in patients with Congenital Heart Disease and laterality defects.

    Science.gov (United States)

    Cristo, Fernando; Inácio, José M; de Almeida, Salomé; Mendes, Patrícia; Martins, Duarte Saraiva; Maio, José; Anjos, Rui; Belo, José A

    2017-07-24

    Perturbations on the Left-Right axis establishment lead to laterality defects, with frequently associated Congenital Heart Diseases (CHDs). Indeed, in the last decade, it has been reported that the etiology of isolated cases of CHDs or cases of laterality defects with associated CHDs is linked with variants of genes involved in the Nodal signaling pathway. With this in mind, we analyzed a cohort of 38 unrelated patients with Congenital Heart Defects that can arise from initial perturbations in the formation of the Left-Right axis and 40 unrelated ethnically matched healthy individuals as a control population. Genomic DNA was extracted from buccal epithelial cells, and variants screening was performed by PCR and direct sequencing. A Nodal-dependent luciferase assay was conducted in order to determine the functional effect of the variant found. In this work, we report two patients with a DAND5 heterozygous non-synonymous variant (c.455G > A) in the functional domain of the DAND5 protein (p.R152H), a master regulator of Nodal signaling. Patient 1 presents left isomerism, ventricular septal defect with overriding aorta and pulmonary atresia, while patient 2 presents ventricular septal defect with overriding aorta, right ventricular hypertrophy and pulmonary atresia (a case of extreme tetralogy of Fallot phenotype). The functional analysis assay showed a significant decrease in the activity of this variant protein when compared to its wild-type counterpart. Altogether, our results provide new insight into the molecular mechanism of the laterality defects and related CHDs, priming for the first time DAND5 as one of multiple candidate determinants for CHDs in humans.

  18. An Introduction to the Septal Extension Graft

    Directory of Open Access Journals (Sweden)

    Myung-Hoon Kim

    2014-01-01

    Full Text Available The septal extension graft is a very useful method of controlling nasal lengthening and tip projection, rotation, and shape by fixing a graft to the septum, which leads to a strong supporting structure. Enhancing graft stability is important for better long-term outcomes and minimizing complications or relapse, and even more efficient application of these methods is needed for East Asians who lack enough cartilage to be harvested in addition to possessing a weak cartilage framework. In this paper, the methods for overcoming the drawbacks of the septal extension graft, such as instability, a fixed tip, and insufficiency of cartilage, are presented, and the applications of each method for greater satisfaction with surgical outcomes are also discussed.

  19. Use of Orbital Conformer to Improve Speech in Patients with Confluent Maxillectomy and Orbital Defects

    Science.gov (United States)

    Colebeck, Amanda C.; Kase, Michael T.; Nichols, Cindy B.; Golden, Marjorie; Huryn, Joseph M.

    2016-01-01

    The basic objective in prosthetic restoration of confluent maxillary and orbital defects is to achieve a comfortable, cosmetically acceptable prosthesis that restores speech, deglutition, and mastication. It is a challenging task complicated by the size and shape of the defects. The maxillary obturator prosthesis often satisfies the objective of adequate deglutition; however, orbital defects that are not obturated in the medial septal or posterior walls allow air to escape, negatively impacting phonation. This article describes a technique to achieve favorable prosthetic rehabilitation in a patient with a maxillectomy and ipsilateral orbital exenteration. The prosthetic components include maxillary obturator, orbital conformer, and orbital prosthesis connected using rigid magnetic attachments. PMID:25953143

  20. Comparison between transcatheter and surgical closure of secundum atrial septal defect in patients over 40 years old

    International Nuclear Information System (INIS)

    Wang Cheng; Zhao Shihua; Jiang Shiliang; Huang Lianjun; Xu Zhongying; Ling Jian; Zheng Hong; Zhang Gejun; Lv Bin; Zhang Yan; Jin Jinglin; Yan Chaowu; Dai Ruping

    2007-01-01

    Objective: To compare the safety and efficacy of transcatheter closure of secundum atrial septal defect (ASD) with surgical closure in patients over 40 years old. Methods: A single center, nonrandomized concurrent study was performed in 233 consecutive adults from January, 2004 to December, 2005. The patients were assigned to either the device or surgical closure group according to the patients options. Technical success rate, complications, residual shunt, hospital stay, amount of blood transfusion and cost were compared. Results: A total of 137 patients were in the group undergoing device closure, whereas 96 patients were in the surgical group. There was no differences in age, sex distribution or baseline cardiac function between the two groups. The sizes of the ASD were(18.9±5.4) mm for the device group and (24.9 ± 6.8)mm for the surgical group (P<0.001). The technical success rates were 97.1% for the device group and 100% for the surgical group (P=0.151). The residual shunt rates were 0.7% for the device group and 0% for the surgical group (P=0.583). Mortality was zero for both groups. The complication rates were 16.1% for the device group and 30.2% for the surgical group (P=0.015). The blood transfusion amounts were (273.1 ± 491.5)ml for the surgical group and 0 ml for the device group (P<0.001). The lengths of hospital stay were (4.6 ± 3.3)days for the device group and (12.0 ± 4.0) days for the surgical group (P<0.001). The costs of hospital stay were 39 570.0±5 929.5 RMB for the device group and 29 839.6±7 533.1 RMB for the surgical group (p<0.001). Conclusions: The technical success rates for surgical versus device closure of ASD were of significantly different, however, the complication rate was lower and the length of hospital stay was shorter for device closure than those for surgical repair. Transcatheter closure of secundum ASD is a safe and effective alternative to surgical repair in selected patients. (authors)

  1. Nonsustained Repetitive Upper Septal Idiopathic Fascicular Left Ventricular Tachycardia: Rare Type of VT

    Directory of Open Access Journals (Sweden)

    Gokhan Aksan

    2016-05-01

    Full Text Available Upper septal fascicular ventricular tachycardia is a very rare form of idiopathic fascicular ventricular tachycardia. Upper septal fascicular tachycardia uses the posterior fascicle as the anterograde limb and the septal fascicle as the retrograde limb. When evaluating the electrocardiography for this form of tachycardia, the presence of narrow QRS morphology and normal axis may be misinterpreted as supraventricular tachycardia. Here, we report a very rare subtype of fascicular tachycardia that originates more proximally in the His-Purkinje system at the base of the heart.

  2. Letter by Gourdie and Sedmera regarding article, "Abnormal conduction and morphology in the atrioventricular node of mice with atrioventricular canal-targeted deletion of Alk3/Bmpr1a receptor"

    Czech Academy of Sciences Publication Activity Database

    Gourdie, R. G.; Sedmera, David

    2008-01-01

    Roč. 118, - (2008), e106-e106 ISSN 0009-7322 Institutional research plan: CEZ:AV0Z50450515 Keywords : conduction system * heart development * atrioventricular canal Subject RIV: FA - Cardiovascular Diseases incl. Cardiotharic Surgery Impact factor: 14.595, year: 2008

  3. Id4 functions downstream of Bmp signaling to restrict TCF function in endocardial cells during atrioventricular valve development.

    Science.gov (United States)

    Ahuja, Suchit; Dogra, Deepika; Stainier, Didier Y R; Reischauer, Sven

    2016-04-01

    The atrioventricular canal (AVC) connects the atrial and ventricular chambers of the heart and its formation is critical for the development of the cardiac valves, chamber septation and formation of the cardiac conduction system. Consequently, problems in AVC formation can lead to congenital defects ranging from cardiac arrhythmia to incomplete cardiac septation. While our knowledge about early heart tube formation is relatively comprehensive, much remains to be investigated about the genes that regulate AVC formation. Here we identify a new role for the basic helix-loop-helix factor Id4 in zebrafish AVC valve development and function. id4 is first expressed in the AVC endocardium and later becomes more highly expressed in the atrial chamber. TALEN induced inactivation of id4 causes retrograde blood flow at the AV canal under heat induced stress conditions, indicating defects in AV valve function. At the molecular level, we found that id4 inactivation causes misexpression of several genes important for AVC and AV valve formation including bmp4 and spp1. We further show that id4 appears to control the number of endocardial cells that contribute to the AV valves by regulating Wnt signaling in the developing AVC endocardium. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. Structural and functional alterations in the atrioventricular node and atrioventricular ring tissue in ischaemia-induced heart failure.

    Science.gov (United States)

    Yanni, Joseph; Maczewski, Michal; Mackiewicz, Urszula; Siew, Samuel; Fedorenko, Olga; Atkinson, Andrew; Price, Marcus; Beresewicz, Andrzej; Anderson, Robert H; Boyett, Mark R; Dobrzynski, Halina

    2014-07-01

    Heart failure (HF) causes dysfunction of the atrioventricular node (AVN) - first or second-degree heart block is a risk factor for sudden cardiac death in HF patients. The aim of the study was to determine if HF causes remodelling of the AVN and right atrioventricular ring (RAVR). HF was induced in rats (n=4) by ligation of the proximal left coronary artery, which resulted in a large infarct of the left ventricle. Sham-operated rats (n=4) were used as controls. Eight weeks after surgery, functional experiments were performed and the hearts were frozen. The body weight of HF rats was similar to control rats, but the mean heart weight of HF rats was significantly enlarged. In HF rats compared to controls, the left ventricle was dilated, left ventricular end-diastolic pressure elevated (21.0 ± 0.6 and 5.4 ± 0.2 mm Hg), left ventricular ejection fraction reduced (0.2 ± 0.02 and 0.5 ± 0.02) and left ventricular end-systolic pressure reduced (102 ± 4.2 and 127 ± 3.1 mm Hg). In HF rats, the in vivo and in vitro PR intervals were increased (41% and 20%), as was the Wenckebach cycle length, indicative of AVN dysfunction. The collagen content was significantly increased in the AVN and RAVR indicating fibrosis. Immunolabelling of caveolin3 (cell membrane marker) showed that there was hypertrophy in HF (cell diameter was increased by 63%, 39% in AVN, RAVR). The TUNEL assay showed that the myocytes of the AVN and RAVR in HF undergo apoptotic cell death. Immunolabelling showed that expression of HCN4 was significantly decreased in the AVN and RAVR (43% and 47%) in HF. We conclude that in HF there is remodelling of the AVN and RAVR and this remodelling may explain the AVN dysfunction.

  5. Classical fluoroscopy criteria poorly predict right ventricular lead septal positioning by comparison with echocardiography.

    Science.gov (United States)

    Squara, Fabien; Scarlatti, Didier; Riccini, Philippe; Garret, Gauthier; Moceri, Pamela; Ferrari, Emile

    2018-03-13

    Fluoroscopic criteria have been described for the documentation of septal right ventricular (RV) lead positioning, but their accuracy remains questioned. Consecutive patients undergoing pacemaker or defibrillator implantation were prospectively included. RV lead was positioned using postero-anterior and left anterior oblique 40° incidences, and right anterior oblique 30° to rule out coronary sinus positioning when suspected. RV lead positioning using fluoroscopy was compared to true RV lead positioning as assessed by transthoracic echocardiography (TTE). Precise anatomical localizations were determined with both modalities; then, RV lead positioning was ultimately dichotomized into two simple clinically relevant categories: RV septal or RV free wall. Accuracy of fluoroscopy for RV lead positioning was then assessed by comparison with TTE. We included 100 patients. On TTE, 66/100 had a septal RV lead and 34/100 had a free wall RV lead. Fluoroscopy had moderate agreement with TTE for precise anatomical localization of RV lead (k = 0.53), and poor agreement for septal/free wall localization (k = 0.36). For predicting septal RV lead positioning, classical fluoroscopy criteria had a high sensitivity (95.5%; 63/66 patients having a septal RV lead on TTE were correctly identified by fluoroscopy) but a very low specificity (35.3%; only 12/34 patients having a free wall RV lead on TTE were correctly identified by fluoroscopy). Classical fluoroscopy criteria have a poor accuracy for identifying RV free wall leads, which are most of the time misclassified as septal. This raises important concerns about the efficacy and safety of RV lead positioning using classical fluoroscopy criteria.

  6. Septo-Hippocampo-Septal Loop and Memory Formation

    Directory of Open Access Journals (Sweden)

    Fatemeh Khakpai

    2013-01-01

    Full Text Available   Cholinergic and GABAergic fibers in the medial septal/diagonal band of Broca (MS/DB area project to the hippocampus and constitute the septo-hippocampal pathway, which has been proven in learning and memory. In addition, the hippocampus has bidirectional connections with the septum, which use this relation for self-regulation of cholinergic input.   The activity of septal and hippocampal neurons is modulated by several neurotransmitters including glutamatergic neurons from the entorhinal cortex, serotonergic fibers from the raphe nucleus, dopaminergic neurons from the ventral tegmental area (VTA, histaminergic cells from the tuberomammillary nucleus and adrenergic fibers from the locus coeruleus (LC. Thus, changes in the glutamatergic, serotonergic and etc. mediated transmission in the MS/DB may influence cholinergic or GABAergic transmission in the hippocampus.

  7. Septo-Hippocampo-Septal Loop and Memory Formation

    Directory of Open Access Journals (Sweden)

    Fatemeh Khakpai

    2013-02-01

    Full Text Available The Cholinergic and GABAergic .bers of the medial septal/diagonal band of Broca (MS/DB area project to the hippocampus and constitute the septo-hippocampal pathway, which has been proven to play a role in learning and memory. In addition, the hippocampus has bidirectional connections with the septum so that to self-regulate of cholinergic input. The activity of septal and hippocampal neurons is modulated by several neurotransmitter systems including glutamatergic neurons from the entorhinal cortex, serotonergic .bers from the raphe nucleus, dopaminergic neurons from the ventral tegmental area (VTA, histaminergic cells from the tuberomammillary nucleus and adrenergic .bers from the locus coeruleus (LC. Thus, changes in the glutamatergic, serotonergic and other systems- mediated transmission in the MS/DB may in.uence cholinergic or GABAergic transmission in the hippocampus.

  8. Ultrastructural aspects in perithecia hyphae septal pores of Glomerella cingulata F. SP. Phaseoli

    Directory of Open Access Journals (Sweden)

    Roca M. María Gabriela

    2000-01-01

    Full Text Available Glomerella cingulata (Stonem. Spauld. & Schrenk f. sp. phaseoli, better known in its anamorphic state Colletotrichum lindemuthianum (Sacc. & Magn. Briosi & Cav., is a causal agent of anthracnose in beans (Phaseolus vulgaris L.. Ultrastructural aspects of the perithecial hyphae of this pathogen were studied. The perithecia hyphae septal pores were found either plugged by a vesicle or unplugged. Some perithecia hyphae septa presented no pore. The Woronin bodies, close to the septal pores, appeared as globose structures which were more electron dense than the occlusions plugging the septal pore.

  9. Measurement of shunt amount using radionuclide angiocardiography: accuracy according to level of shunt and associated lesion

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Yang Min [Sejong General Hospital, Bucheon (Korea, Republic of)

    2006-08-15

    Determination of pulmonary to systemic blood flow ratio (QP/QS) is important for the management of patients with left-to-right shunt. This study was performed to assess the agreement of Qp/Qs ratio using the radionuclide method and oxymetry, to investigate the factors influencing the agreement, and to know how interchangeable the results of each technique. We compared the Qp/Qs measured by single-pass radionuclide angiocardiography and oxymetry during catheterization in 207 patients who underwent both studies. In radionuclide method, Qp/Qs was calculated from the pulmonary time-activity curves using a gamma variate fit. The correlation and Bland-Altman analysis were performed according to the levels of shunt and associated lesions. The mean Qp/Qs was 1.83 {+-} 0.50 by radionuclide, and 1.74 {+-} 0.51 by oxymetry. The overall correlation coefficient was 0.86 ({rho} 0.001), and Bland-Altman range of agreement encompassing 4SD was 1.05. For atrial septal defect, ventricular septal defect, patent ductus arteriosus, tricuspid and mitral insufficiency, the correlation coefficient was 0.78, 0.90, 0.84, 0.63 and 0.44 and Bland-Altman range was 1.52, 0.74, 0.96, 1.57 and 1.50, respectively. There is good agreement but wide variance between the Qp/Qs ratios by radionuclide method and oxymetry. Associated atrioventricular valvar insufficiency decreases the correlation coefficient and widens the variance. Wide overall variance suggests that Qp/Qs measurements by two techniques should not be used interchangeably.

  10. Measurement of shunt amount using radionuclide angiocardiography: accuracy according to level of shunt and associated lesion

    International Nuclear Information System (INIS)

    Kim, Yang Min

    2006-01-01

    Determination of pulmonary to systemic blood flow ratio (QP/QS) is important for the management of patients with left-to-right shunt. This study was performed to assess the agreement of Qp/Qs ratio using the radionuclide method and oxymetry, to investigate the factors influencing the agreement, and to know how interchangeable the results of each technique. We compared the Qp/Qs measured by single-pass radionuclide angiocardiography and oxymetry during catheterization in 207 patients who underwent both studies. In radionuclide method, Qp/Qs was calculated from the pulmonary time-activity curves using a gamma variate fit. The correlation and Bland-Altman analysis were performed according to the levels of shunt and associated lesions. The mean Qp/Qs was 1.83 ± 0.50 by radionuclide, and 1.74 ± 0.51 by oxymetry. The overall correlation coefficient was 0.86 (ρ 0.001), and Bland-Altman range of agreement encompassing 4SD was 1.05. For atrial septal defect, ventricular septal defect, patent ductus arteriosus, tricuspid and mitral insufficiency, the correlation coefficient was 0.78, 0.90, 0.84, 0.63 and 0.44 and Bland-Altman range was 1.52, 0.74, 0.96, 1.57 and 1.50, respectively. There is good agreement but wide variance between the Qp/Qs ratios by radionuclide method and oxymetry. Associated atrioventricular valvar insufficiency decreases the correlation coefficient and widens the variance. Wide overall variance suggests that Qp/Qs measurements by two techniques should not be used interchangeably

  11. Predictive factors for red blood cell transfusion in children undergoing noncomplex cardiac surgery.

    Science.gov (United States)

    Mulaj, Muj; Faraoni, David; Willems, Ariane; Sanchez Torres, Cristel; Van der Linden, Philippe

    2014-08-01

    Red blood cell (RBC) transfusion is frequently required in pediatric cardiac surgery and is associated with altered outcome and increased costs. Determining which factors predict transfusion in this context will enable clinicians to adopt strategies that will reduce the risk of RBC transfusion. This study aimed to assess predictive factors associated with RBC transfusion in children undergoing low-risk cardiac surgery with cardiopulmonary bypass (CPB). Children undergoing surgery to repair ventricular septal defect or atrioventricular septal defect from 2006 to 2011 were included in this retrospective study. Demography, preoperative laboratory testing, intraoperative data, and RBC transfusion were reviewed. Univariate and multivariate logistic regression analysis were used to define factors that were able to predict RBC transfusion. Then, we employed receiver operating characteristic analysis to design a predictive score. Among the 334 children included, 261 (78%) were transfused. Age (43 mL/kg), type of oxygenator used, minimal temperature reached during CPB (<32°C), and preoperative hematocrit (<34%) were independently associated with RBC transfusion in the studied population. A predictive score 2 or greater was the best predictor of RBC transfusion. The present study identified several factors that were significantly associated with perioperative RBC transfusion. Based on these factors, we designed a predictive score that can be used to develop a patient-based blood management program with the aim of reducing the incidence of RBC transfusion. Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  12. Caudal Septal Stabilization Suturing Technique to Treat Crooked Noses.

    Science.gov (United States)

    Baykal, Bahadir; Erdim, Ibrahim; Guvey, Ali; Oghan, Fatih; Kayhan, Fatma Tulin

    2016-10-01

    To rotate the nasal axis and septum to the midline using an L-strut graft and a novel caudal septal stabilization suturing technique to treat crooked noses. Thirty-six patients were included in the study. First, an L-strut graft was prepared by excising the deviated cartilage site in all patients. Second, multiple stabilization suturing, which we describe as a caudal septal stabilization suturing technique with a "fishing net"-like appearance, was applied between the anterior nasal spine and caudal septum in all patients. This new surgical technique, used to rotate the caudal septum, was applied to 22 I-type and 14 C-type crooked noses. Correction rates for the crooked noses were compared between the 2 inclination types with angular estimations. Deviation angles were measured using the AutoCAD 2012 software package and frontal (anterior) views, with the Frankfurt horizontal line parallel to the ground. Nasal axis angles showing angle improvement graded 4 categories as excellent, good, acceptable, and unsuccessful for evaluations at 6 months after surgery in the study. The success rate in the C-type nasal inclination was 86.7% (±21.9) and 88% (±16.7) in the I-type. The overall success rate of L-strut grafting and caudal septal stabilization suturing in crooked nose surgeries was 87.5% (±18.6). "Unsuccessful" results were not reported in any of the patients. L-strut grafting and caudal septal stabilization suturing techniques are efficacious in crooked noses according to objective measurement analysis results. However, a longer follow-up duration in a larger patient population is needed.

  13. Congenital intermittent atrio-ventricular dissociation in tetralogy of Fallot (a case report.

    Directory of Open Access Journals (Sweden)

    Karande S

    1991-01-01

    Full Text Available A case of tetralogy of fallot with congenital intermittent atrioventricular dissociation is reported. A review of standard postgraduate books of cardiology fails to describe this condition, thus showing its extreme rarity. The therapeutic dilemma in treating such a case is also discussed.

  14. Gerbode defect and multivalvular dysfunction: Complex complications in adult congenital heart disease.

    Science.gov (United States)

    Ruivo, Catarina; Guardado, Joana; Montenegro Sá, Fernando; Saraiva, Fátima; Antunes, Alexandre; Correia, Joana; Morais, João

    2017-07-01

    We report a clinical case of a 40-year-old male with surgically corrected congenital heart disease (CHD) 10 years earlier: closure of ostium primum, mitral annuloplasty, and aortic valve and root surgery. The patient was admitted with acute heart failure. Transesophageal echocardiography (TEE) revealed a dysmorphic and severely incompetent aortic valve, a partial tear of the mitral valve cleft repair and annuloplasty ring dehiscence. A true left ventricular-to-right atrial shunt confirmed a direct Gerbode defect. The authors aim to discuss the diagnostic challenge of adult CHD, namely the key role of TEE on septal defects and valve regurgitations description. © 2017, Wiley Periodicals, Inc.

  15. Intra-cardiac echocardiography in alcohol septal ablation

    DEFF Research Database (Denmark)

    Cooper, Robert M; Shahzad, Adeel; Newton, James

    2015-01-01

    Alcohol septal ablation (ASA) in hypertrophic obstructive cardiomyopathy reduces left ventricular outflow tract gradients. A third of patients do not respond; inaccurate localisation of the iatrogenic infarct can be responsible. Transthoracic echocardiography (TTE) using myocardial contrast can...

  16. Evolution and scaling of atrioventricular conduction time in mammals. [Pt. 1

    NARCIS (Netherlands)

    Meijler, F.L.; Strackee, J.

    2006-01-01

    Scaling can be defined as the adjustment of a structure, a function, or an organ to the size of the mammalian body. An example is the size of the heart in relation to the size of the body. The duration of the PR interval on the electrocardiogram (atrioventricular delay) in relation to the size of

  17. Idiopathic dilated cardiomyopathy: computerized anatomic study of relashionship between septal and free left ventricle wall.

    Science.gov (United States)

    Juliani, Paulo Sérgio; Costa, Eder França da; Correia, Aristides Tadeu; Monteiro, Rosangela; Jatene, Fabio Biscegli

    2014-01-01

    A feature of dilated cardiomyopathy is the deformation of ventricular cavity, which contributes to systolic dysfunction. Few studies have evaluated this deformation bearing in mind ventricular regions and segments of the ventricle, which could reveal important details of the remodeling process, supporting a better understanding of its role in functional impairment and the development of new therapeutic strategies. To evaluate if, in basal, equatorial and apical regions, increased internal transverse perimeter of left ventricle in idiopathic dilated cardiomyopathy occurs proportionally between the septal and non-septal segment. We performed an anatomical study with 28 adult hearts from human cadavers. One group consisted of 18 hearts with idiopathic dilated cardiomyopathy and another group with 10 normal hearts. After lamination and left ventricle digital image capture, in three different regions (base, equator and apex), the transversal internal perimeter of left ventricle was divided into two segments: septal and not septal. These segments were measured by proper software. It was established an index of proportionality between these segments, called septal and non-septal segment index. Then we determined whether this index was the same in both groups. Among patients with normal hearts and idiopathic dilated cardiomyopathy, the index of proportionality between the two segments (septal and non-septal) showed no significant difference in the three regions analyzed. The comparison results of the indices NSS/SS among normal and enlarged hearts were respectively: in base 1.99 versus 1.86 (P=0.46), in equator 2.22 versus 2.18 (P=0.79) and in apex 2.96 versus 3.56 (P=0.11). In the idiopathic dilated cardiomyopathy, the transversal dilatation of left ventricular internal perimeter occurs proportionally between the segments corresponding to the septum and free wall at the basal, equatorial and apical regions of this chamber.

  18. Survival and sudden cardiac death after septal ablation for hypertrophic obstructive cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten Kvistholm; Havndrup, Ole; Hassager, Christian

    2011-01-01

    Reports of long-term survival and the risk of sudden cardiac death (SCD) after percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy (HOCM) are sparse.......Reports of long-term survival and the risk of sudden cardiac death (SCD) after percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy (HOCM) are sparse....

  19. Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management.

    Science.gov (United States)

    Baruteau, Alban-Elouen; Pass, Robert H; Thambo, Jean-Benoit; Behaghel, Albin; Le Pennec, Solène; Perdreau, Elodie; Combes, Nicolas; Liberman, Leonardo; McLeod, Christopher J

    2016-09-01

    Atrioventricular block is classified as congenital if diagnosed in utero, at birth, or within the first month of life. The pathophysiological process is believed to be due to immune-mediated injury of the conduction system, which occurs as a result of transplacental passage of maternal anti-SSA/Ro-SSB/La antibodies. Childhood atrioventricular block is therefore diagnosed between the first month and the 18th year of life. Genetic variants in multiple genes have been described to date in the pathogenesis of inherited progressive cardiac conduction disorders. Indications and techniques of cardiac pacing have also evolved to allow safe permanent cardiac pacing in almost all patients, including those with structural heart abnormalities. Early diagnosis and appropriate management are critical in many cases in order to prevent sudden death, and this review critically assesses our current understanding of the pathogenetic mechanisms, clinical course, and optimal management of congenital and childhood AV block. • Prevalence of congenital heart block of 1 per 15,000 to 20,000 live births. AV block is defined as congenital if diagnosed in utero, at birth, or within the first month of life, whereas childhood AV block is diagnosed between the first month and the 18th year of life. As a result of several different etiologies, congenital and childhood atrioventricular block may occur in an entirely structurally normal heart or in association with concomitant congenital heart disease. Cardiac pacing is indicated in symptomatic patients and has several prophylactic indications in asymptomatic patients to prevent sudden death. • Autoimmune, congenital AV block is associated with a high neonatal mortality rate and development of dilated cardiomyopathy in 5 to 30 % cases. What is New: • Several genes including SCN5A have been implicated in autosomal dominant forms of familial progressive cardiac conduction disorders. • Leadless pacemaker technology and gene therapy for

  20. Double-outlet right ventricle revisited.

    Science.gov (United States)

    Ebadi, Ameneh; Spicer, Diane E; Backer, Carl L; Fricker, F Jay; Anderson, Robert H

    2017-08-01

    Double-outlet right ventricle is a form of ventriculoarterial connection. The definition formulated by the International Society for Nomenclature of Paediatric and Congenital Heart Disease is based on hearts with both arterial trunks supported in their greater part by a morphologically right ventricle. Bilateral infundibula and ventricular septal defects are highly debated criteria. This study examines the anatomic controversies surrounding double-outlet right ventricle. We show that hearts with double-outlet right ventricle can have atrioventricular-to-arterial valvular continuity. We emphasize the difference between the interventricular communication and the zone of deficient ventricular septation. The hearts examined were from the University of Florida in Gainesville; Johns Hopkins All Children's Hospital, St Petersburg, Fla; and Lurie Children's Hospital, Chicago, Ill. Each specimen had at least 75% of both arterial roots supported by the morphologically right ventricle, with a total of 100 hearts examined. The morphologic method was used to assess anatomic features, including arterial-atrioventricular valvular continuity, subarterial infundibular musculature, and the location of the hole between the ventricles. Most hearts had fibrous continuity between one of the arterial valves and an atrioventricular valve, with bilateral infundibula in 23%, and intact ventricular septum in 5%. Bilateral infundibula are not a defining feature of double-outlet right ventricle, representing only 23% of the specimens in our sample. The interventricular communication can have a posteroinferior muscular rim or extend to become perimembranous (58%). Double-outlet right ventricle can exist with an intact ventricular septum. Copyright © 2017 The American Association for Thoracic Surgery. All rights reserved.

  1. Radiological evaluation of double-outlet right ventricle - An analysis of cinecardioangiography in 44 cases -

    Energy Technology Data Exchange (ETDEWEB)

    Park, Cheong Hee; Yeon, Kyung Mo; Han, Man Chung [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1984-03-15

    Double-outlet right ventricle is defined as follows: both great arteries arise completely or nearly completely from the right ventricle; neither semilunar valve is in fibrous continuity with either atrioventricular valve; and usually a ventricular septal defect is present and the only outlet from the left ventricle. A total of 44 cases of double-outlet right ventricle is analyzed, in which cineangiocardiographies were done at the Department of Radiology, Seoul National University Hospital in recent 4 year and 6 months, with specific reference to the segmental combinations, the height of conus, the relationship of great arteries, the location of ventricular septal defects, and associated anomalies. The results were as follows; 1. Among 44 cases, 36 cases had normal cardiac position, 4 cases had dextrocardia with situs inversus 2 cases had dextrocardia with situs solitus, 1 case had levocardia with situs inversus, and another 1 case had mesocardia with situs ambiguus. 2. Segmental sets were [S,D,D] in 36 cases, [I,L,L] in 3 cases, [I,D,D] in 2 cases, [S,D,L], [S,L,L] and [A,D,D] in 1 case respectively. 3. All cases had bilateral conus. Aortic valve rings were same level as pulmonary valve rings in 25 cases, lower than pulmonary valve rings in 17 cases in which 15 cases were type A., and higher than pulmonary valve rings in 2 cases. 4. The relation of the great arteries were normal in 15 cases, side-by-side in 13 cases, dextromalposition in 13 cases, and levomal position in 3 cases. 5. The position of the ventricular septal defects with respect to the origins of the great arteries is subaortic (type A and type B) in 23 cases, subpulmonary (type C) in 13 cases, doubly committed (type D) in 3 cases, and uncommitted (type E) in 5 cases. 6. Associated cardiac malformations are pulmonary stenosis in 24 which had all cases of type A and type E, aortic stenosis in 6 which were only in type C, left SVC in 6, abnormality of atrioventricular valve in 5, single coronary artery

  2. [Complete atrioventricular block in Duchenne muscular dystrophy].

    Science.gov (United States)

    Kuru, Satoshi; Tanahashi, Tamotsu; Matsumoto, Shinjirou; Kitamura, Tetsuya; Konagaya, Masaaki

    2012-01-01

    We report a case of complete atrioventricular (AV) block in a 40-year-old patient with Duchenne muscular dystrophy (DMD). While he was bed-ridden and required mechanical ventilation, his cardiac involvement was mild. He had the deletion of exon 45-52 in the dystrophin gene. He underwent transient complete AV block and came to require pacemaker implantation due to recurrence of complete AV block ten days after the first attack. Electrophysiological study revealed mild prolonged AH and HV interval. Although DMD patients with AV block have been rarely reported so far, attention should be paid to AV block for patients who prolonged their lives.

  3. [Nasal septal abscess].

    Science.gov (United States)

    Barril, María F; Ferolla, Fausto M; José, Pablo; Echave, Cecilia; Tomezzoli, Silvana; Fiorini, Sandra; López, Eduardo Luis

    2008-12-01

    A nasal septal abscess (NA) is defined as a collection of pus between the cartilage or bony septum and its normally applied mucoperichondrium or mucoperiostium. It is an uncommon disease which should be suspected in a patient with acute onset of nasal obstruction and recent history of nasal trauma, periodontal infection or an inflammatory process of the rhinosinusal region. We report a case of an 8-year-old boy with bilateral NA caused by community-acquired methicillin-resistant Staphylococcus aureus(MR-CO) in order to emphasize the importance of prompt diagnosis and adequate treatment to prevent the potentially dangerous spread of infection and the development of severe functional and cosmetic sequelae.

  4. Longitudinal evaluation of P-wave dispersion and P-wave maximum in children after transcatheter device closure of secundum atrial septal defect.

    Science.gov (United States)

    Grignani, Robert Teodoro; Tolentino, Kim Martin; Rajgor, Dimple Dayaram; Quek, Swee Chye

    2015-06-01

    Transcatheter device closure of the secundum atrial septal defect (ASD) in children prevents atrial arrhythmias in older age. However, the benefits of favourable atrial electrocardiographic markers in these children remain elusive. We aimed to review the electrocardiographic markers of atrial activity in a longitudinal fashion. We retrospectively reviewed longitudinal data of all children who underwent transcatheter device closure at the National University Hospital between 2004 and 2013. The inclusion criteria included the presence of a secundum-type ASD with left to right shunt and evidence of increased right ventricular volume load (Q p/Q s ratio >1.5 and/or right ventricular dilatation). A total of 25 patients with a mean follow-up of 44.7 ± 33.47 (7.3-117.4) months were included. P maximum and P dispersion decreased at 2 months, P amplitude at 1 week and remained so until last follow-up. A positive trend was seen with a correlation coefficient of +0.12 for P maximum, +0.08 for P dispersion and 0.34 for P amplitude. There was a higher baseline P amplitude and P dispersion in patients who were older than 10 years and a non-significant trend to support an increase in both P maximum (71.0 ± 8.8 vs. 73.2 ± 12.7), P dispersion (17.0 ± 6.5 vs. 22.0 ± 11.3) and P amplitude (0.88 ± 0.25 vs. 1.02 ± 0.23) in patients with an ASD more than 15 mm compared with an ASD <15 mm. There is reduction in both P maximum and P dispersion as early as 2 months, which persisted on follow-up. Earlier closure may result in more favourable electrocardiographic results.

  5. Nasal airway and septal variation in unilateral and bilateral cleft lip and palate.

    Science.gov (United States)

    Starbuck, John M; Friel, Michael T; Ghoneima, Ahmed; Flores, Roberto L; Tholpady, Sunil; Kula, Katherine

    2014-10-01

    Cleft lip and palate (CLP) affects the dentoalveolar and nasolabial facial regions. Internal and external nasal dysmorphology may persist in individuals born with CLP despite surgical interventions. 7-18 year old individuals born with unilateral and bilateral CLP (n = 50) were retrospectively assessed using cone beam computed tomography. Anterior, middle, and posterior nasal airway volumes were measured on each facial side. Septal deviation was measured at the anterior and posterior nasal spine, and the midpoint between these two locations. Data were evaluated using principal components analysis (PCA), multivariate analysis of variance (MANOVA), and post-hoc ANOVA tests. PCA results show partial separation in high dimensional space along PC1 (48.5% variance) based on age groups and partial separation along PC2 (29.8% variance) based on CLP type and septal deviation patterns. MANOVA results indicate that age (P = 0.007) and CLP type (P ≤ 0.001) significantly affect nasal airway volume and septal deviation. ANOVA results indicate that anterior nasal volume is significantly affected by age (P ≤ 0.001), whereas septal deviation patterns are significantly affected by CLP type (P ≤ 0.001). Age and CLP type affect nasal airway volume and septal deviation patterns. Nasal airway volumes tend to be reduced on the clefted sides of the face relative to non-clefted sides of the face. Nasal airway volumes tend to strongly increase with age, whereas septal deviation values tend to increase only slightly with age. These results suggest that functional nasal breathing may be impaired in individuals born with the unilateral and bilateral CLP deformity. © 2014 Wiley Periodicals, Inc.

  6. Full Atrioventricular Block Secondary to Acute Poisoning Mercury: A Case Report

    Directory of Open Access Journals (Sweden)

    Amelia Geraldine Peregrina-Chávez

    2018-04-01

    Full Text Available Background: The biological behaviour and clinical significance of mercury toxicity vary according to its chemical structure. Mercury differs in its degree of toxicity and in its effects on the nervous, digestive and immune systems as well as on organs such as the lungs, kidneys, skin, eyes and heart. Human exposure occurs mainly through inhalation of elemental mercury vapours during industrial and artisanal processes such as artisanal and small-scale gold mining. Case presentation: A 52-years-old female, housewife, with a body mass index of 25.3 kg/cm2, without smoking or alcohol habits or any important clinical or chronic cardiovascular history, was admitted to the emergency room due to probable accidental poisoning by butane gas. Clinical manifestations with a headache, dizziness, cough, and dyspnoea of medium to small efforts. An initial physical exploration with Glasgow scored at 15, with arrhythmic heart sounds, pulmonary fields with bilateral subcrepitant rales and right basal predominance. Electrocardiographic findings were as follows: a cardiac frequency of 50 beats per minute and atrioventricular dissociation. Laboratory parameters were: white blood cells at 15.8 × 109/L; aspartate aminotransferase at 38 U/L; lactate dehydrogenase at 1288 U/L; creatine-kinase at 115 U/L; CK-MB fraction at 28 U/L; and other biochemical parameters were within the reference values. A radiographic evaluation showed flow cephalization, diffuse bilateral infiltrates with right basal predominance. In addition, the patient presented data of low secondary expenditure to third-degree atrioventricular (AV block for which the placement of a transvenous pacemaker was decided, substantially improving the haemodynamic parameters. Subsequently, after a family interrogation, the diagnosis of mercury inhalation poisoning was established. An initial detection of mercury concentration (Hg(0 was carried out, reporting 243.5 µg/L. In view of this new evidence, mercury

  7. Radiofrequency Catheter Ablation of Coexistent Idiopathic Left Ventricular Tachycardia and Atrioventricular Nodal Reentrant Tachycardia

    Directory of Open Access Journals (Sweden)

    Ken-Pen Weng

    2005-10-01

    Full Text Available A healthy 15-year-old male patient presented with a 6-month history of recurrent attacks of palpitations. On multiple emergency room visits, a sustained wide QRS complex tachycardia with a right bundle branch block and northwest axis deviation was documented. The tachycardia was not terminated by intravenous adenosine, but was suppressed with intravenous verapamil. There was no evidence of structural heart disease, myocarditis, long QT syndrome, or electrolyte imbalance after a series of standard examinations. Idiopathic left ventricular tachycardia (ILVT was suspected. Electrophysiologic studies revealed 2 inducible tachycardias, which were shown to represent atrioventricular nodal reentrant tachycardia (AVNRT and ILVT. Transformation from AVNRT to ILVT occurred spontaneously following atrial pacing. Successful ablation of ILVT and the slow atrioventricular nodal pathway resulted in cure of the double tachycardia.

  8. A case of residual inferior sinus venosus defect after ineffective surgical closure.

    Science.gov (United States)

    Uga, Sayuri; Hidaka, Takayuki; Takasaki, Taiichi; Kihara, Yasuki

    2014-10-03

    A 38-year-old woman presented with cyanosis and heart failure 34 years after patch closure of an atrial septal defect and partial anomalous pulmonary venous connection. CT and cardiac catheterisation showed a residual defect that caused right-to-left shunting. The patch almost blocked the inferior vena cava from the right atrium, resulting in uncommon drainage of the inferior vena cava into the left atrium. Other anomalies included the coronary-to-pulmonary artery fistula and duplicate inferior vena cava with dilated azygos venous system. A second surgery was performed, and we confirmed an inferior sinus venosus defect, which is rare and can be misdiagnosed. The ineffective patch closure had caused a haemodynamic status that rarely occurs. We describe the diagnostic process and emphasise the importance of correctly understanding the entity. 2014 BMJ Publishing Group Ltd.

  9. Growth activity in human septal cartilage: age-dependent incorporation of labeled sulfate in different anatomic locations

    International Nuclear Information System (INIS)

    Vetter, U.; Pirsig, W.; Heinze, E.

    1983-01-01

    Growth activity in different areas of human septal cartilage was measured by the in vitro incorporation of 35 S-labeled NaSO 4 into chondroitin sulfate. Septal cartilage without perichondrium was obtained during rhinoplasty from 36 patients aged 6 to 35 years. It could be shown that the anterior free end of the septum displays high growth activity in all age groups. The supra-premaxillary area displayed its highest growth activity during prepuberty, showing thereafter a continuous decline during puberty and adulthood. A similar age-dependent pattern in growth activity was found in the caudal prolongation of the septal cartilage. No age-dependent variations could be detected in the posterior area of the septal cartilage

  10. Does asymptomatic septal agenesis exist? A review of 34 cases

    Energy Technology Data Exchange (ETDEWEB)

    Belhocine, Ouardia; Andre, Christine; Kalifa, Gabriel; Adamsbaum, Catherine [St Vincent de Paul Hospital, Radiology Department, Paris (France)

    2005-04-01

    Primary septal agenesis (PSA) is a rare brain malformation that can be isolated or part of developmental brain abnormalities (holoprosencephaly, septo-optic dysplasia or cortical malformation). Such associated malformation can be subtle, leading to difficulties in the prenatal management of PSA. Moreover, the neurological prognosis of isolated PSA remains debatable. The aims of the study were to specify the patterns and frequency of brain malformations associated with septal agenesis (SA), to identify the clinical prognosis, and to discuss the aetiology of PSA with the new insights provided by molecular genetics. The study consisted of a 14-year retrospective review of brain MRI in 34 patients having PSA (mean age, 5 years). Chiasm and optic nerves were not evaluated. Post-hydrocephalus SA or incomplete data were excluded. The clinical data were correlated to the MRI patterns. The study disclosed 82.5% associated lesions with MRI (28/34): 11 neuronal migration disorders, 9 holoprosencephalies (HP), 7 pituitary stalk interruptions, 1 corpus callosum partial agenesis; 17.5% (6/34) of cases were apparently isolated PAS. Clinically, the patients had motor dysfunction in 68% (23/34), mental retardation in 65% (22/34), blindness in 24% (8/34), endocrinological defects in 21% (7/34) and epilepsy in 18% (6/34) of cases. Nine percent of patients (3/34) were neurologically normal (including one with scoliosis and two infants younger than 2 years at the last follow-up). Patients with bilateral cortical anomalies and HP (even if mild) had the worst neurological prognosis. A severe motor impairment was present without evidence of hemispheric anomaly in 12% of patients (4/34). Interestingly, the frontal lobes were involved in 90% of cortical anomalies and HP, supporting the malformative aetiology of PSA. PSA rarely appears isolated and severe psychomotor impairment may occur in apparently isolated forms. These unfavourable results should be highlighted and need to be confirmed

  11. Device and method for treatment of openings in vascular and septal walls

    Science.gov (United States)

    Singhal, Pooja; Wilson, Thomas S.; Cosgriff-Hernandez, Elizabeth; Maitland, Duncan J.

    2017-06-06

    A device, system and method for treatment of an opening in vascular and/or septal walls including patent foramen ovale. The device has wings/stops on either end, an axis core covered in a shape memory foam and is deliverable via a catheter to the affected opening, finally expanding into a vascular or septal opening where it is held in place by the expandable shape memory stops or wings.

  12. Histopathologic analysis of atrial tissue in patients with atrial fibrillation: comparison between patients with atrial septal defect and patients with mitral valvular heart disease.

    Science.gov (United States)

    Kwak, Jae Gun; Seo, Jeong-Wook; Oh, Sam Se; Lee, Sang Yun; Ham, Eui Keun; Kim, Woong-Han; Kim, Soo-Jin; Bae, Eun Jung; Lim, Cheoung; Lee, Chang-Ha; Lee, Cheul

    2014-01-01

    Atrial fibrillation (AF) in adult patients with atrial septal defect (ASD) accompanies an enlarged right atrium (RA) with a less enlarged left atrium (LA), which is the opposite situation in patients with AF and mitral valvular disease. This study was to compare the histopathological change in the atrium of patients with AF of two different etiologies: ASD and mitral disease. Twenty-four patients were enrolled. Group 1 included patients with ASD (8), Group 2 included patients with ASD with AF (6), and Group 3 included patients with mitral disease with AF (10). Preoperative atrial volumes were measured. Atrial tissues were obtained during surgical procedures and stained with periodic acid-Schiff, smooth muscle actin, Sirius red, and Masson's trichrome to detect histopathologic changes compatible with AF. The severity of histopathological changes was represented with "positivity" and "strong positivity" after analyzing digitalized images of the staining. We investigated the relationship between the degree of atrial dilatation and severity of histopathological changes according to the groups and tissues. Group 2 and Group 3 patients showed a tendency toward an enlarged RA volume and enlarged LA volume, respectively, compared with each others. However, in the histopathologic analysis, "positivity" and "strong positivity" showed no significant positive correlations with the degree of atrial volume in special staining. A similar degree of histopathologic changes was observed in both atria in patients with AF (Group 2 and 3) regardless of the degree of dilatation of atrial volume and disease entities. Crown Copyright © 2014. Published by Elsevier Inc. All rights reserved.

  13. Complete Atrioventricular Block Complicating Mitral Infective Endocarditis Caused by Streptococcus Agalactiae

    OpenAIRE

    Arai, Masaru; Nagashima, Koichi; Kato, Mahoto; Akutsu, Naotaka; Hayase, Misa; Ogura, Kanako; Iwasawa, Yukino; Aizawa, Yoshihiro; Saito, Yuki; Okumura, Yasuo; Nishimaki, Haruna; Masuda, Shinobu; Hirayama, Atsushi

    2016-01-01

    Patient: Male, 74 Final Diagnosis: Infective endocarditis Symptoms: Apetite loss ? fever Medication: ? Clinical Procedure: Transesophageal echocardiography Specialty: Cardiology Objective: Rare co-existance of disease or pathology Background: Infective endocarditis (IE) involving the mitral valve can but rarely lead to complete atrioventricular block (CAVB). Case Report: A 74-year-old man with a history of infective endocarditis caused by Streptococcus gordonii (S. gordonii) presented to our ...

  14. Accessory atrioventricular myocardial connections in the developing human heart: relevance for perinatal supraventricular tachycardias

    NARCIS (Netherlands)

    Hahurij, Nathan D.; Gittenberger-de Groot, Adriana C.; Kolditz, Denise P.; Bökenkamp, Regina; Schalij, Martin J.; Poelmann, Robert E.; Blom, Nico A.

    2008-01-01

    BACKGROUND: Fetal and neonatal atrioventricular (AV) reentrant tachycardias can be life-threatening but resolve in most cases during the first year of life. The transient presence of accessory AV myocardial connections during annulus fibrosus development may explain this phenomenon. METHODS AND

  15. Molecular Diffusion through Cyanobacterial Septal Junctions

    Directory of Open Access Journals (Sweden)

    Mercedes Nieves-Morión

    2017-01-01

    Full Text Available Heterocyst-forming cyanobacteria grow as filaments in which intercellular molecular exchange takes place. During the differentiation of N2-fixing heterocysts, regulators are transferred between cells. In the diazotrophic filament, vegetative cells that fix CO2 through oxygenic photosynthesis provide the heterocysts with reduced carbon and heterocysts provide the vegetative cells with fixed nitrogen. Intercellular molecular transfer has been traced with fluorescent markers, including calcein, 5-carboxyfluorescein, and the sucrose analogue esculin, which are observed to move down their concentration gradient. In this work, we used fluorescence recovery after photobleaching (FRAP assays in the model heterocyst-forming cyanobacterium Anabaena sp. strain PCC 7120 to measure the temperature dependence of intercellular transfer of fluorescent markers. We find that the transfer rate constants are directly proportional to the absolute temperature. This indicates that the “septal junctions” (formerly known as “microplasmodesmata” linking the cells in the filament allow molecular exchange by simple diffusion, without any activated intermediate state. This constitutes a novel mechanism for molecular transfer across the bacterial cytoplasmic membrane, in addition to previously characterized mechanisms for active transport and facilitated diffusion. Cyanobacterial septal junctions are functionally analogous to the gap junctions of metazoans.

  16. Safe management of cesarean section in a patient of Eisenmenger syndrome

    Directory of Open Access Journals (Sweden)

    Silvia Minicucci

    2012-01-01

    Full Text Available We report our experience of a 29-year-old female with a complete atrio-ventricular septal defect leading to a single ventricle physiology and Eisenmenger syndrome. The patient successfully underwent spinal anesthesia for cesarean section in the 31 st week of pregnancy. A multidisciplinary approach involving cardiologist, cardiac surgeon, obstetrician, and anesthesiologist was utilized to achieve a safe pregnancy and cesarean for the delivery of the baby. A close clinical assessment is required, especially during the third trimester when the risk of acute right ventricular dysfunction increases. The use of extracorporeal membrane oxygenation (ECMO (as a bridge to recovery or bridge to salvage was planned to support oxygenation and circulation in case of acute biventricular dysfunction. The delivery/cesarean section was performed in a cardiac surgery operating room, and to reduce the time-frame for ECMO institution the femoral vessels were exposed surgically before the cesarean section.

  17. Identifying Septal Support Reconstructions for Saddle Nose Deformity: The Cakmak Algorithm.

    Science.gov (United States)

    Cakmak, Ozcan; Emre, Ismet Emrah; Ozkurt, Fazil Emre

    2015-01-01

    The saddle nose deformity is one of the most challenging problems in nasal surgery with a less predictable and reproducible result than other nasal procedures. The main feature of this deformity is loss of septal support with both functional and aesthetic implications. Most reports on saddle nose have focused on aesthetic improvement and neglected the reestablishment of septal support to improve airway. To explain how the Cakmak algorithm, an algorithm that describes various fixation techniques and grafts in different types of saddle nose deformities, aids in identifying saddle nose reconstructions that restore supportive nasal framework and provide the aesthetic improvements typically associated with procedures to correct saddle nose deformities. This algorithm presents septal support reconstruction of patients with saddle nose deformity based on the experience of the senior author in 206 patients with saddle nose deformity. Preoperative examination, intraoperative assessment, reconstruction techniques, graft materials, and patient evaluation of aesthetic success were documented, and 4 different types of saddle nose deformities were defined. The Cakmak algorithm classifies varying degrees of saddle nose deformity from type 0 to type 4 and helps identify the most appropriate surgical procedure to restore the supportive nasal framework and aesthetic dorsum. Among the 206 patients, 110 women and 96 men, mean (range) age was 39.7 years (15-68 years), and mean (range) of follow-up was 32 months (6-148 months). All but 12 patients had a history of previous nasal surgeries. Application of the Cakmak algorithm resulted in 36 patients categorized with type 0 saddle nose deformities; 79, type 1; 50, type 2; 20, type 3a; 7, type 3b; and 14, type 4. Postoperative photographs showed improvement of deformities, and patient surveys revealed aesthetic improvement in 201 patients and improvement in nasal breathing in 195 patients. Three patients developed postoperative infection

  18. Holt-Oram Syndrome in Adult Presenting with Heart Failure: A Rare Presentation

    Directory of Open Access Journals (Sweden)

    Rupesh Kumar

    2014-01-01

    Full Text Available Holt-Oram syndrome is a rare inherited disorder involving the hands, arms, and the heart. The defects involve carpal bones of the wrist and the thumb and the associated cardiac anomalies like atrial or ventricular septal defects. Congenital cardiac and upper-limb malformations frequently occur together and are classified as heart-hand syndromes. The most common amongst the heart-hand disorders is the Holt-Oram syndrome, which is characterized by septal defects of the heart and preaxial radial ray abnormalities. Its incidence is one in 100,000 live births. Approximately three out of four patients have some cardiac abnormality with common associations being either an atrial septal defect or ventricular septal defect. Herein, we report a rare sporadic case of Holt-Oram syndrome with atrial septal defect with symptoms of heart failure in a forty-five-year-old lady who underwent emergency cardiac surgery for the symptoms.

  19. Disinfection By-Product Exposures and the Risk of Specific Cardiac Birth Defects

    Science.gov (United States)

    Wright, J. Michael; Evans, Amanda; Kaufman, John A.; Rivera-Núñez, Zorimar; Narotsky, Michael G.

    2016-01-01

    Background: Epidemiological studies suggest that women exposed to disinfection by-products (DBPs) have an increased risk of delivering babies with cardiovascular defects (CVDs). Objective: We examined nine CVDs in relation to categorical DBP exposures including bromoform, chloroform, dibromochloromethane (DBCM), bromodichloromethane (BDCM), monobromoacetic acid (MBAA), dichloroacetic acid (DCAA), trichloroacetic acid (TCAA), and summary DBP measures (HAA5, THMBr, THM4, and DBP9). Methods: We calculated adjusted odds ratios (aORs) in a case–control study of birth defects in Massachusetts with complete quarterly 1999–2004 trihalomethane (THM) and haloacetic acid (HAA) data. We randomly matched 10 controls each to 904 CVD cases based on week of conception. Weight-averaged aggregate first-trimester DBP exposures were assigned to individuals based on residence at birth. Results: We detected associations for tetralogy of Fallot and the upper exposure categories for TCAA, DCAA, and HAA5 (aOR range, 3.34–6.51) including positive exposure–response relationships for DCAA and HAA5. aORs consistent in magnitude were detected between atrial septal defects and bromoform (aOR = 1.56; 95% CI: 1.01, 2.43), as well as DBCM, chloroform, and THM4 (aOR range, 1.26–1.67). Ventricular septal defects (VSDs) were associated with the highest bromoform (aOR = 1.85; 95% CI: 1.20, 2.83), MBAA (aOR = 1.81; 95% CI: 0.85, 3.84), and DBCM (aOR = 1.54; 95% CI: 1.00, 2.37) exposure categories. Conclusions: To our knowledge, this is the first birth defect study to develop multi-DBP adjusted regression models as well as the first CVD study to evaluate HAA exposures and the second to evaluate bromoform exposures. Our findings, therefore, inform exposure specificity for the consistent associations previously reported between THM4 and CVDs including VSDs. Citation: Wright JM, Evans A, Kaufman JA, Rivera-Núñez Z, Narotsky MG. 2017. Disinfection by-product exposures and the risk of specific

  20. Dynamic three-dimensional display of common congenital cardiac defects from reconstruction of two-dimensional echocardiographic images.

    Science.gov (United States)

    Hsieh, K S; Lin, C C; Liu, W S; Chen, F L

    1996-01-01

    Two-dimensional echocardiography had long been a standard diagnostic modality for congenital heart disease. Further attempts of three-dimensional reconstruction using two-dimensional echocardiographic images to visualize stereotypic structure of cardiac lesions have been successful only recently. So far only very few studies have been done to display three-dimensional anatomy of the heart through two-dimensional image acquisition because such complex procedures were involved. This study introduced a recently developed image acquisition and processing system for dynamic three-dimensional visualization of various congenital cardiac lesions. From December 1994 to April 1995, 35 cases were selected in the Echo Laboratory here from about 3000 Echo examinations completed. Each image was acquired on-line with specially designed high resolution image grazmber with EKG and respiratory gating technique. Off-line image processing using a window-architectured interactive software package includes construction of 2-D ehcocardiographic pixel to 3-D "voxel" with conversion of orthogonal to rotatory axial system, interpolation, extraction of region of interest, segmentation, shading and, finally, 3D rendering. Three-dimensional anatomy of various congenital cardiac defects was shown, including four cases with ventricular septal defects, two cases with atrial septal defects, and two cases with aortic stenosis. Dynamic reconstruction of a "beating heart" is recorded as vedio tape with video interface. The potential application of 3D display of the reconstruction from 2D echocardiographic images for the diagnosis of various congenital heart defects has been shown. The 3D display was able to improve the diagnostic ability of echocardiography, and clear-cut display of the various congenital cardiac defects and vavular stenosis could be demonstrated. Reinforcement of current techniques will expand future application of 3D display of conventional 2D images.

  1. Tbx20 interacts with smads to confine tbx2 expression to the atrioventricular canal

    NARCIS (Netherlands)

    Singh, Reena; Horsthuis, Thomas; Farin, Henner F.; Grieskamp, Thomas; Norden, Julia; Petry, Marianne; Wakker, Vincent; Moorman, Antoon F. M.; Christoffels, Vincent M.; Kispert, Andreas

    2009-01-01

    RATIONALE: T-box transcription factors play critical roles in the coordinated formation of the working chambers and the atrioventricular canal (AVC). Tbx2 patterns embryonic myocardial cells to form the AVC and suppresses their differentiation into chamber myocardium. Tbx20-deficient embryos, which

  2. Atrioventricular depolarization differences identify coronary artery anomalies in Kawasaki disease.

    Science.gov (United States)

    Cortez, Daniel; Sharma, Nandita; Jone, Pei-Ni

    2017-03-01

    Kawasaki disease (KD) is the leading cause of acquired heart disease in children. Signal average electrocardiogram changes in patients during the acute phase of KD with coronary artery anomalies (CAA) include depolarization changes. We set out to determine if 12-lead-derived atrioventricular depolarization differences can identify CAA in patients with KD. A blinded, retrospective case-control study of patients with KD was performed. Deep Q waves, corrected QT-intervals (QTc), spatial QRS-T angles, T-wave vector magnitudes (RMS-T), and a novel parameter for assessment of atrioventricular depolarization difference (the spatial PR angle) and a two dimensional PR angle were assessed. Comparisons between groups were performed to test for significant differences. One hundred one patients with KD were evaluated, with 68 having CAA (67.3%, mean age 3.6 ± 3.0 years, 82.6% male), and 32 without CAA (31.7%, mean age 2.7 ± 3.2 years, 70.4% male). The spatial PR angle significantly discriminated KD patients with CAA from those without, 59.7° ± 31.1° versus 41.6° ± 11.5° (p differences, measured by the spatial or two dimensional PR angle differentiate KD patients with CAA versus those without. © 2016 Wiley Periodicals, Inc.

  3. Incidence of congenital heart disease among neonates in a neonatal unit of a tertiary care hospital

    International Nuclear Information System (INIS)

    Hussain, S.; Sabir, M.U.

    2014-01-01

    Objectives: To determine the incidence and pattern of various congenital heart disease in a neonatal unit of a tertiary care hospital. Methods: The prospective study was carried out in the neonatal unit of Combined Military Hospital, Rawalpindi, from September 2008 to August 2011. All 5800 neonates admitted with gestational age of >28 weeks irrespective of birthweight were included in the study. Neonatologist/Paediatrician carried out the neonatal examination during the first 12 hours of life. Neonates suspected of having congenital heart disease were further evaluated by pulse oxymetry, X-ray chest and echocardiography to ascertain final diagnosis and type of lesion. Data was collected on a predesigned proforma containing information regarding gender, mode of delivery, gestational age, weight at birth, family history, and associated malformations. SPSS 16 was used for statistical analysis. Results: Of the 5800 neonates, 87 (1.5%) were found to have congenital heart disease with an incidence of 15/1000. There was a male preponderance. Most common lesion was ventricular septal defect 27(31.3%), followed by atrial septal defect 20 (22.9%), patent ductus arteriosus 13 (14.94%), tetralogy of fallot 06 (6.89%), transposition of great arteries 04 (4.59%), Pulmonary stenosis 05 (5.79%) and 03(3.44%) had atrioventricular canal defects. Conclusion: Congenital heart disease is a common congenital anomaly. Its incidence varies from centre to centre due to different factors like nature of the sample, method of detection and early examination by a neonatologist/paediatrician. In this study a higher incidence is reported because it was carried out in a tertiary care unit, which is a referral hospital and all the neonates admitted in the unit were included in the study. (author)

  4. Aortic intracardiac echocardiography-guided septal puncture during mitral valvuloplasty.

    Science.gov (United States)

    Akkaya, Emre; Vuruskan, Ertan; Zorlu, Ali; Sincer, Isa; Kucukosmanoglu, Mehmet; Ardic, Idris; Yilmaz, Mehmet Birhan

    2014-01-01

    Transoesophageal echocardiography (TEE) and venous intracardiac echocardiography (ICE) are traditionally used to visualize the interatrial septum (IAS) and the tenting effect of the fossa ovalis in patients undergoing percutaneous balloon mitral valvuloplasty (PBMV). The aim of the present study was to assess the comparative efficacy and safety of arterial (intra-aortic) ICE and venous ICE, compared with TEE (traditional approach), in the patients undergoing PBMV. TEE, aortic ICE, and venous ICE were consecutively performed in 50 patients (40 ± 9 years, 86% female). The images of intracardiac structures were obtained from both aortic and right atrial loci. The IAS was visualized using TEE, aortic ICE, and venous ICE. The mean mitral valve area was 1.14 ± 0.2 cm(2), and the mean left atrial volume index was 57.5 ± 12 mL/m(2). The mean size of the visualized septal length was 48 ± 5 mm by TEE, 51 ± 5 mm by aortic ICE, and 33 ± 6 mm by venous ICE. The Bland-Altman test indicated that the 95% limits of agreement for the measurement of septal diameter ranged from -11.0 to +5.9 mm (mean -2.5 mm) between TEE and aortic ICE, -2.8 to +33.5 mm (mean +15.3 mm) between TEE and venous ICE, and -36.6 to +0.8 mm (mean -17.9 mm) between venous and aortic ICE. Standard venous ICE generally tended to yield smaller values compared with TEE and aortic ICE for the measurement of septal length. Furthermore, the view of fossa ovalis and 'tenting effect' was optimal in 11 patients on venous ICE; however, the fossa ovalis and tip of the needle were well visualized in all patients on aortic ICE (P < 0.001). There were no major complications with the use of aortic ICE. Aortic ICE is a superior alternative to venous ICE and facilitates trans-septal puncture in patients with mitral stenosis.

  5. Electrocardiographic characteristics of atrioventricular block induced by tilt testing.

    Science.gov (United States)

    Zyśko, Dorota; Gajek, Jacek; Koźluk, Edward; Mazurek, Walentyna

    2009-02-01

    The electrocardiographic (ECG) characteristics of atrioventricular (AV) block during reflex syncope may be unique due to the presence of hypervagotonia. The aim of the present study was to define the ECG characteristics of the AV block induced by neurocardiogenic reflex provoked by tilt testing (TT). A series of 31 patients with presumed vasovagal syncope and AV block provoked by TT was studied. The duration of PP and PR interval, AV block grade and type, concomitant arrhythmias, and timing of the AV block occurrence were assessed. The AV block occurred at TT termination in 26 patients, in the recovery in 4 patients, and in both periods in 1 patient. Atrioventricular block was preceded by sinus slowing, and sinus rhythm during AV block was slow and instable. Mobitz I, 2:1 second-degree AV block, and advanced second-degree AV block were recognized in 35.5, 48.4, and 67.8% of patients, respectively. Third-degree AV block was diagnosed in 41.9% of patients. Twenty-one patients had at least two AV block forms. The most prevalent concomitant arrhythmia was junctional escape rhythm (61.3%). (i) The occurrence of the AV block during neurocardiogenic reaction induced by TT is always preceded by sinus rhythm slowing and usually by PR interval prolongation. (ii) The AV block provoked by TT usually occurs at TT termination, but may occur even in the recovery period in a supine position. Sometimes the AV block may be present both at TT termination and during the recovery period.

  6. Echocardiography-guided Radiofrequency Catheter Ablation of Atrioventricular Node and VVI Pacemaker Implantation

    Directory of Open Access Journals (Sweden)

    T Guo

    2014-05-01

    Full Text Available Objective: This study is to evaluate the feasibility and safety of intracardiac radiofrequency catheter ablation (RFCA of the atrioventricular node (AVN and pacemaker implantation using transthoracic echocardiography. Methods: Eleven patients – six males and five females (mean age 66 years – with persistent or permanent atrial fibrillation/atrial flutter received RFCA of AVN and VVI pacemaker implantation (paces and senses the ventricle and is inhibited if it senses ventricular activity. Under transthoracic echocardiography, the electrode catheters were positioned intracardiac, and target ablation was performed, with the permanent pacemaking catheter in the left subclavian vein and the ablation catheter in the right femoral vein. The multi-view imaging and dynamic observation applied during the stable AV dissociation were successful. Results: Atrioventricular node ablation and permanent pacemaker implantation in 11 patients were completed successfully without X-ray exposure. The operation success rate was 100%. All patients recovered well within the follow-up period. Conclusions: Radiofrequency catheter ablation of AVN and VVI pacemaker implantation under transthoracic echocardiography guidance is a safe, easy and feasible approach. This procedure could be an important supplemental measure to catheter ablation of arrhythmia under routine X-ray fluoroscopy.

  7. Successful catheter ablation of ventricular premature complexes from the right atrial side of the atrioventricular septum with good contact force.

    Science.gov (United States)

    Arai, Marina; Fukamizu, Seiji; Kawamura, Iwanari; Miyazawa, Satoshi; Hojo, Rintaro; Sakurada, Harumizu; Hiraoka, Masayasu

    2018-04-01

    The acquisition of good contact force for radiofrequency catheter ablation of ventricular premature complexes (VPCs) originating from the basal septum of the left ventricle (LV) is often difficult. We describe a case of VPCs originating from the basal septum of the LV, which were successfully eliminated by applying radiofrequency at the right atrium (RA) side of the atrioventricular septum (AVS) without causing any significant impairment of atrioventricular conduction because the ablation catheter could obtain better contact force through the RA approach. Moreover, intracardiac echocardiography (ICE) and RA angiography effectively demonstrated the AVS.

  8. Mechanism of polyuria and natriuresis in atrioventricular nodal tachycardia.

    Science.gov (United States)

    Canepa-Anson, R; Williams, M; Marshall, J; Mitsuoka, T; Lightman, S; Sutton, R

    1984-01-01

    A woman with tachycardia associated with polyuria was investigated. Electrophysiological analysis showed that the tachycardia was an atrioventricular nodal re-entrant tachycardia. Programmed stimulation was then used to provoke and sustain the tachycardia for 40 minutes. Polyuria, with an appreciable increase in free water clearance, was observed. This was associated with reduction in plasma and urinary arginine vasopressin concentrations. Appreciable natriuresis also developed. These results support the hypothesis that the polyuria with increased free water clearance and the natriuresis occurring during sustained tachycardia in man are due to inhibition of secretion of vasopressin and the release of natriuretic factor. PMID:6434116

  9. Occult etiologies of complete atrioventricular block: Report of two cases

    Directory of Open Access Journals (Sweden)

    Neeta Bachani

    2016-09-01

    Full Text Available In patients presenting with complete atrioventricular (AV block, the common causes are degeneration of the conduction system, acute myocardial infarction, congenital and metabolic disorders (such as azotemia. However, at times, no cause can be ascribed and the label congenital or degenerative is applied depending on the patient's age and the QRS complex width. We present two cases of patients with complete AV block, who were subsequently found to have rare etiologies – sarcoidosis (with isolated feature of AV block and non-Hodgkin's lymphoma.

  10. Radial bone graft usage for nasal septal reconstruction

    Directory of Open Access Journals (Sweden)

    Yakup Cil

    2011-01-01

    Full Text Available Background: Although various techniques have been described for correction of crooked and saddle nose deformities, these problems are challenging with high recurrence and revision rates. Conventional septal surgery may not be adequate for nose reconstruction in crooked and saddle nose deformities. Materials and Methods: Between December 2005 and October 2009, six patients with crooked nose and five patients with saddle nose deformities underwent corrective surgery in our clinic. All patients were male, and the mean age was 21 years (range, 19-23 years. We used rigid radial bone graft to prevent redeviation and recurrence following corrective nasal septal surgery. Results: The mean follow-up period was 28 months, ranging from 18 to 46 months. Mean operation time was 4 hours (3-4.5. All patients healed uneventfully. None of the patients required secondary surgery. Conclusions: We believe that radial bone grafts offer a long lasting support in treatment of challenging cases with crooked and saddle nose deformities.

  11. [Parahisian atrial tachycardia or atrioventricular nodal reentrant tachycardia with tendon of Todaro breakthrough?].

    Science.gov (United States)

    Orczykowski, Michał; Jaworska-Wilczyńska, Maria; Urbanek, Piotr; Bodalski, Robert; Derejko, Paweł; Gajek, Jacek; Hryniewiecki, Tomasz; Szumowski, Lukasz; Walczak, Franciszek

    2010-08-01

    We present a case of a 61 year-old woman with tachycardia originating close to the His bundle where radiofrequency (RF) ablation may bear potential risk of atrioventricular (AV) block. In this case report we discuss the possibility of a AV nodal reciprocating tachycardia with tendon of Todaro breakthrough. Patient was safely and effectively treated with RF catheter ablation.

  12. Increased sarcolemmal Na+/H+ exchange activity in hypertrophied myocytes from dogs with chronic atrioventricular block

    NARCIS (Netherlands)

    van Borren, Marcel M. G. J.; Vos, Marc A.; Houtman, Marien J. C.; Antoons, Gudrun; Ravesloot, Jan H.

    2013-01-01

    Dogs with compensated biventricular hypertrophy due to chronic atrioventricular block (cAVB), are more susceptible to develop drug-induced Torsade-de-Pointes arrhythmias and sudden cardiac death. It has been suggested that the increased Na+ influx in hypertrophied cAVB ventricular myocytes

  13. Urgent surgical management for embolized occluder devices in childhood: single center experience

    Directory of Open Access Journals (Sweden)

    Gokaslan Gokhan

    2012-12-01

    Full Text Available Abstract Background In this study, we sought to analyze our experience in urgent surgical management for embolized cardiac septal and ductal occluder devices resulting from trans-catheter closure of atrial septal defect, ventricular septal defect and patent ductus arteriosus in childhood patient group. Methods We retrospectively reviewed 9 patients (aged 2–15 years who underwent urgent surgery due to cardiac septal and ductal occluder embolization between January 2007 and December 2010. Congenital defects were atrial septal defect (n = 6, ventricular septal defect (n = 1, and patent ductus arteriosus (n = 2. Risk factors for device embolization and urgent surgical management techniques for embolized device removal were discussed. Results Removal of embolized devices in all cases and repair of damaged tricuspid valve in 2 patients were performed. Inevitably, all congenital defects were closed or ligated up to the primary defect. Total circulator arrest necessitated in 1 patient with ascending aortic device embolization. All operations were completed successfully and no hospital mortality or morbidity was encountered. Conclusions Although closure of left to right shunting defects by percutaneous occluder devices has a lot of advantages, device embolization is still a major complication. If embolized device retrieval fails with percutaneous intervention attempts, surgical management is the only method to remove embolized devices. In this circumstance, to provide an uneventful perioperative course, urgent management strategies should be well planned.

  14. Atrioventricular valve repair in patients with functional single-ventricle physiology: impact of ventricular and valve function and morphology on survival and reintervention.

    Science.gov (United States)

    Honjo, Osami; Atlin, Cori R; Mertens, Luc; Al-Radi, Osman O; Redington, Andrew N; Caldarone, Christopher A; Van Arsdell, Glen S

    2011-08-01

    This study was to determine whether atrioventricular valve repair modifies natural history of single-ventricle patients with atrioventricular valve insufficiency and to identify factors predicting survival and reintervention. Fifty-seven (13.5%) of 422 single-ventricle patients underwent atrioventricular valve repair. Valve morphology, regurgitation mechanism, and ventricular morphology and function were analyzed for effect on survival, transplant, and reintervention with multivariate logistic and Cox regression models. Comparative analysis used case-matched controls. Atrioventricular valve was tricuspid in 67% and common in 28%. Ventricular morphology was right in 83%. Regurgitation mechanisms were prolapse (n = 24, 46%), dysplasia (n = 18, 35%), annular dilatation (n = 8, 15%), and restriction or cleft (n = 2, 4%). Postrepair insufficiency was none or trivial in 14 (26%), mild in 33 (61%), and moderate in 7 (13%). Survival in repair group was lower than in matched controls (78.9% vs 92.7% at 1 year, 68.7% vs 90.6% at 3 years, P = .015). Patients with successful repair and normal ventricular function had equivalent survival to matched controls (P = .36). Independent predictors for death or transplant included increased indexed annular size (P = .05), increased cardiopulmonary bypass time (P = .04), and decreased postrepair ventricular function (P = .01). Ventricular dilation was a time-related factor for all events, including failed repair. Survival was lower in single-ventricle patients operated on for atrioventricular valve insufficiency than in case-matched controls. Patients with little postoperative residual regurgitation and preserved ventricular function had equivalent survival to controls. Lower grade ventricular function and ventricular dilation correlated with death and repair failure, suggesting that timing of intervention may affect outcome. Copyright © 2011 The American Association for Thoracic Surgery. Published by Mosby, Inc. All

  15. There Is No Structural Relationship between Nasal Septal Deviation, Concha Bullosa, and Paranasal Sinus Fungus Balls

    Directory of Open Access Journals (Sweden)

    Tung-Lung Tsai

    2012-01-01

    Full Text Available This study aims to determine the relationship between nasal septal deviation, concha bullosa, and chronic rhinosinusitis by using a definitive pathological and simplified model. Fifty-two consecutive sinus computed tomography scans were performed on patients who received endoscopic sinus surgery and whose final diagnosis was paranasal sinus fungus balls. The incidences of nasal septal deviation and concha bullosa for patients diagnosed with paranasal sinus fungus balls among the study group were 42.3% and 25%, respectively. About 63.6% sinuses with fungus balls were located on the ipsilateral side of the nasal septal deviation, and 46.2% were located on the ipsilateral side of the concha bullosa. When examined by Pearson’s chi-square test and the chi-squared goodness-of-fit test, no significant statistical difference for the presence of paranasal sinus fungus balls between ipsilateral and contralateral sides of nasal septal deviation and concha bullosa was noted (P=0.292 and P=0.593, resp.. In conclusion, we could not demonstrate any statistically significant correlation between the location of infected paranasal sinus, the direction of nasal septal deviation, and the location of concha bullosa, in location-limited rhinosinusitis lesions such as paranasal sinus fungal balls. We conclude that the anatomical variants discussed herein do not predispose patients to rhinosinusitis.

  16. Neurogenesis in the septal and temporal part of the adult rat dentate gyrus.

    Science.gov (United States)

    Bekiari, Chryssa; Giannakopoulou, Aggeliki; Siskos, Nikistratos; Grivas, Ioannis; Tsingotjidou, Anastasia; Michaloudi, Helen; Papadopoulos, Georgios C

    2015-04-01

    Structural and functional dissociation between the septal and the temporal part of the dentate gyrus predispose for possible differentiations in the ongoing neurogenesis process of the adult hippocampus. In this study, BrdU-dated subpopulations of the rat septal and temporal dentate gyrus (coexpressing GFAP, DCX, NeuN, calretinin, calbindin, S100, caspase-3 or fractin) were quantified comparatively at 2, 5, 7, 14, 21, and 30 days after BrdU administration in order to examine the successive time-frames of the neurogenesis process, the glial or neuronal commitment of newborn cells and the occurring apoptotic cell death. Newborn neurons' migration from the neurogenic subgranular zone to the inner granular cell layer and expression of glutamate NMDA and AMPA receptors were also studied. BrdU immunocytochemistry revealed comparatively higher numbers of BrdU(+) cells in the septal part, but stereological analysis of newborn and total granule cells showed an identical ratio in the two parts, indicating an equivalent neurogenic ability, and a common topographical pattern along each part's longitudinal and transverse axis. Similarly, both parts exhibited extremely low levels of newborn glial and apoptotic cells. However, despite the initially equal division rate and pattern of the septal and temporal proliferating cells, their later proliferative profile diverged in the two parts. Dynamic differences in the differentiation, migration and maturation process of the two BrdU-incorporating subpopulations of newborn neurons were also detected, along with differences in their survival pattern. Therefore, we propose that various factors, including developmental date birth, local DG microenvironment and distinct functionality of the two parts may be the critical regulators of the ongoing neurogenesis process, leading the septal part to a continuous, rapid, and less-disciplined genesis rate, whereas the quiescent temporal microenvironment preserves a quite steady, less

  17. Blood flow patterns underlie developmental heart defects.

    Science.gov (United States)

    Midgett, Madeline; Thornburg, Kent; Rugonyi, Sandra

    2017-03-01

    Although cardiac malformations at birth are typically associated with genetic anomalies, blood flow dynamics also play a crucial role in heart formation. However, the relationship between blood flow patterns in the early embryo and later cardiovascular malformation has not been determined. We used the chicken embryo model to quantify the extent to which anomalous blood flow patterns predict cardiac defects that resemble those in humans and found that restricting either the inflow to the heart or the outflow led to reproducible abnormalities with a dose-response type relationship between blood flow stimuli and the expression of cardiac phenotypes. Constricting the outflow tract by 10-35% led predominantly to ventricular septal defects, whereas constricting by 35-60% most often led to double outlet right ventricle. Ligation of the vitelline vein caused mostly pharyngeal arch artery malformations. We show that both cardiac inflow reduction and graded outflow constriction strongly influence the development of specific and persistent abnormal cardiac structure and function. Moreover, the hemodynamic-associated cardiac defects recapitulate those caused by genetic disorders. Thus our data demonstrate the importance of investigating embryonic blood flow conditions to understand the root causes of congenital heart disease as a prerequisite to future prevention and treatment. NEW & NOTEWORTHY Congenital heart defects result from genetic anomalies, teratogen exposure, and altered blood flow during embryonic development. We show here a novel "dose-response" type relationship between the level of blood flow alteration and manifestation of specific cardiac phenotypes. We speculate that abnormal blood flow may frequently underlie congenital heart defects. Copyright © 2017 the American Physiological Society.

  18. Gerbode defect following endocarditis and misinterpreted as severe pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Allajbeu Iris

    2010-09-01

    Full Text Available Abstract A Gerbode -type defect is a ventricular septal defect communicating directly between the left ventricle and right atrium. It is usually congenital, but rarely is acquired, as a complication of endocarditis. This can be anatomically possible because the normal tricuspid valve is more apically displaced than the mitral valve. However, identification of an actual communication is often extremely difficult, so a careful and meticulous echocardiogram should be done in order to prevent echocardiographic misinterpretation of this defect as pulmonary arterial hypertension. The large systolic pressure gradient between the left ventricle and the right atrium would expectedly result in a high velocity systolic Doppler flow signal in right atrium and it can be sometimes mistakably diagnosed as tricuspid regurgitant jet simulating pulmonary arterial hypertension. We present a rare case of young woman, with endocarditis who presented with severe pulmonary arterial hypertension. The preoperative diagnosis of left ventricle to right atrial communication (acquired Gerbode defect was suspected initially by echocardiogram and confirmed at the time of the surgery. A point of interest, apart from the diagnostic problem, was the explanation for its mechanism and presentation. The probability of a bacterial etiology of the defect is high in this case.

  19. Treatment of septal hematomas and abscesses in children

    NARCIS (Netherlands)

    Menger, Dirk Jan; Tabink, Ivar; Nolst Trenité, Gilbert J.

    2007-01-01

    The cartilaginous part of the nasal septum of a child with a septal hematoma or abscess is at risk of destruction. Consequently, the noses of these children can collapse, causing a saddle nose deformity, and in time, the normal outgrowth of both the nose and maxilla win be disturbed. In adulthood,

  20. Surgical treatment for ectopic atrial tachycardia.

    Science.gov (United States)

    Graffigna, A; Vigano, M; Pagani, F; Salerno, G

    1992-08-01

    Atrial tachycardia is an infrequent but potentially dangerous arrhythmia which often determines cardiac enlargement. Surgical ablation of the arrhythmia is effective and safe, provided a careful atrial mapping is performed and the surgical technique is tailored to the individual focus location. Eight patients underwent surgical ablation of ectopic atrial tachycardia between 1977 and 1990. Different techniques were adopted for each patient according to the anatomical location of the focus and possibly associated arrhythmias. Whenever possible, a closed heart procedure was chosen. In 1 patient a double focal origin was found and treated by separate procedures. In 1 patient with ostium secundum atrial septal defect and atrial flutter, surgical isolation of the right appendage and the ectopic focus was performed. In all patients ectopic atrial tachycardia was ablated with maintenance of the sinoatrial and atrioventricular nodal function as well as internodal conduction. In follow-up up to December 1991, no recurrency was recorded.

  1. Modelling the heart with the atrioventricular plane as a piston unit.

    Science.gov (United States)

    Maksuti, Elira; Bjällmark, Anna; Broomé, Michael

    2015-01-01

    Medical imaging and clinical studies have proven that the heart pumps by means of minor outer volume changes and back-and-forth longitudinal movements in the atrioventricular (AV) region. The magnitude of AV-plane displacement has also shown to be a reliable index for diagnosis of heart failure. Despite this, AV-plane displacement is usually omitted from cardiovascular modelling. We present a lumped-parameter cardiac model in which the heart is described as a displacement pump with the AV plane functioning as a piston unit (AV piston). This unit is constructed of different upper and lower areas analogous with the difference in the atrial and ventricular cross-sections. The model output reproduces normal physiology, with a left ventricular pressure in the range of 8-130 mmHg, an atrial pressure of approximatly 9 mmHg, and an arterial pressure change between 75 mmHg and 130 mmHg. In addition, the model reproduces the direction of the main systolic and diastolic movements of the AV piston with realistic velocity magnitude (∼10 cm/s). Moreover, changes in the simulated systolic ventricular-contraction force influence diastolic filling, emphasizing the coupling between cardiac systolic and diastolic functions. The agreement between the simulation and normal physiology highlights the importance of myocardial longitudinal movements and of atrioventricular interactions in cardiac pumping. Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved.

  2. Trans-Catheter Therapy of Lutembacher Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Hossein Nough

    2011-05-01

    Full Text Available Lutembacher syndrome refers to the rare combination of a congenital atrial septal defect and acquired mitral stenosis. Traditionally, Lutembacher syndrome has been corrected by surgical treatment. We describe two patients treated percutaneouly with a combined Inoue balloon valvuloplasty and septal defect closure using the Amplatzer septal occlusion device.

  3. NJP VOLUME 40 No 4 Corrected

    African Journals Online (AJOL)

    PROF. EZECHUKWU

    2013-03-01

    Mar 1, 2013 ... heart defects (e.g Tetralogy of Fallot, Truncus arteriosus and Transposition of the great arteries) typically present with cyanosis while acyanotic heart defects (e.g Ven- tricular septal defect, Atrial septal defect, Pulmonary stenosis) do not normally manifest with cyanosis. Children with CHD have impaired ...

  4. Prevalence of cardiac dyssynchrony and correlation with atrio-ventricular block and QRS width in dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Anzouan-Kacou, J B; Ncho-Mottoh, M P; Konin, C

    2012-01-01

    Cardiac dyssynchrony causes disorganised cardiac contraction, delayed wall contraction and reduced pumping efficiency. We aimed to assess the prevalence of different types of dyssynchrony in patients with dilated cardiomyopathy (DCM), and to establish the correlation between atrio-ventricular block...

  5. Mondini defect in association with multiple congenital anomalies.

    Science.gov (United States)

    Sekhar, H K; Sachs, M

    1976-01-01

    A case of bilaterally symmetrical genetic aplasia conforming to Mondini type of congenital deformity in a 12-day-old child is presented with the help of temporal bone sections. Cochlear changes include a stunted modiolus, deficient interscalar septum between the middle and upper coils forming a scala communis cochleae, a degenerated organ of Corti and reduced spiral ganglion cells and dendrites. The vestibule is malformed, with membranous labyrinth being deficient. The utricle and semicircular canals are absent. There is no oval window or stapedial footplate, and the facial nerve is hypoplastic. An interesting feature is the unusual association of bilateral bony choanal atresia, atrial septal defect, cleft lip, absence of olfactory bulbs in the brain, and congenital ophthalmic anomalies.

  6. A case of typical atrioventricular nodal (AVN) reentrant tachycardia confined to the compact AV node, showing a variety of rare electrophysiological findings, including eccentric AVN echoes

    OpenAIRE

    Tanaka, Kazushi; Shiotani, Shinji; Fukuda, Keisuke; Fujihara, Masahiko; Higashimori, Akihiro; Morioka, Nobuyuki; Yokoi, Yoshiaki; Fujimura, Osamu

    2014-01-01

    Herein, we report the case of a 49-year-old woman with typical atrioventricular nodal (AVN) reentrant tachycardia, confined to the compact atrioventricular node, showing numerous rare electrophysiological findings such as unique AVN reentrant echoes, double ventricular responses, latent retrograde dual AVN pathways, antegrade triple AVN pathways, and longitudinal dissociation within the lower final common pathway.

  7. A case of typical atrioventricular nodal (AVN reentrant tachycardia confined to the compact AV node, showing a variety of rare electrophysiological findings, including eccentric AVN echoes

    Directory of Open Access Journals (Sweden)

    Kazushi Tanaka, M.D., Ph.D.

    2014-12-01

    Full Text Available Herein, we report the case of a 49-year-old woman with typical atrioventricular nodal (AVN reentrant tachycardia, confined to the compact atrioventricular node, showing numerous rare electrophysiological findings such as unique AVN reentrant echoes, double ventricular responses, latent retrograde dual AVN pathways, antegrade triple AVN pathways, and longitudinal dissociation within the lower final common pathway.

  8. Effect of atrioventricular conduction on heart rate variability

    KAUST Repository

    Ahmad, Talha Jamal

    2011-08-01

    This paper discusses the effect of atrioventricular conduction time (AVCT) on the short-term Heart Rate Variability (HRV) by computing HRV parameters using intervals between the onsets of successive P waves (PP time series) for three groups: normal, arrhythmia and sudden cardiac death (SCD) patients. A very precise wavelet transform based ECG delineator was developed to detect PP, PR and RR time series. Mean PR variation in arrhythmia and SCD group was found to be significantly high as compared to the normal group. It was observed that when PR variations in arrhythmia and SCD cases crossed a certain threshold, RR variability no longer provided a very accurate estimate of HRV. In such cases, PP variability was able to provide a better assessment of HRV. © 2011 IEEE.

  9. The helical ventricular myocardial band of Torrent-Guasp: potential implications in congenital heart defects.

    Science.gov (United States)

    Corno, Antonio F; Kocica, Mladen J; Torrent-Guasp, Francisco

    2006-04-01

    The new concepts of cardiac anatomy and physiology, based on the observations made by Francisco Torrent-Guasp's discovery of the helical ventricular myocardial band, can be useful in the context of the surgical strategies currently used to manage patients with congenital heart defects. The potential impact of the Torrent-Guasp's Heart on congenital heart defects have been analyzed in the following settings: ventriculo-arterial discordance (transposition of the great arteries), double (atrio-ventricular and ventriculo-arterial) discordance (congenitally corrected transposition of the great arteries), Ebstein's anomaly, pulmonary valve regurgitation after repair of tetralogy of Fallot, Ross operation, and complex intra-ventricular malformations. The functional interaction of right and left ventricles occurs not only through their arrangements in series but also thanks to the structural spiral features. Changes in size and function of either ventricle may influence the performance of the other ventricle. The variety and complexity of congenital heart defects make the recognition of the relationship between form and function a vital component, especially when compared to acquired disease. The new concepts of cardiac anatomy and function proposed by Francisco Torrent-Guasp, based on his observations, should stimulate further investigations of alternative surgical strategies by individuals involved with the management of patients with congenital heart defects.

  10. [The crooked nose: correction of dorsal and caudal septal deviations].

    Science.gov (United States)

    Foda, H M T

    2010-09-01

    The deviated nose represents a complex cosmetic and functional problem. Septal surgery plays a central role in the successful management of the externally deviated nose. This study included 800 patients seeking rhinoplasty to correct external nasal deviations; 71% of these suffered from variable degrees of nasal obstruction. Septal surgery was necessary in 736 (92%) patients, not only to improve breathing, but also to achieve a straight, symmetric external nose. A graduated surgical approach was adopted to allow correction of the dorsal and caudal deviations of the nasal septum without weakening its structural support to the nasal dorsum or nasal tip. The approach depended on full mobilization of deviated cartilage, followed by straightening of the cartilage and its fixation in the corrected position by using bony splinting grafts through an external rhinoplasty approach.

  11. Structure of Masera's Septal Olfactory Organ in Cat (Felis silvestris f. catus - Light Microscopy in Selected Stages of Ontogeny

    Directory of Open Access Journals (Sweden)

    I. Kociánová

    2006-01-01

    Full Text Available The septal organ /SO/ (Masera's organ /MO/ is a chemoreceptor presently considered one of three types of olfactory organs (along with the principal olfactory region and vomeronasal organ. Notwithstanding the septal organ having been first described by Rodolfo Masera in 1943, little is known of the properties of sensory neurons or of its functional significance in chemoreception. Until now the septal organ has been described only in laboratory rodents and some marsupials. This work refers to its existence in the domestic cat (Felis silvestris f. catus. The septal organ can be identified at the end of embryonic period - 27 or 28 days of ontogenesis in cats (the 6th developmental stage of Štěrba - coincident with formation of the principal olfactory region in nasal cavity. At 45 days of ontogenesis (the 9th developmental stage of Štěrba, this septal olfactory organ is of circular or oval shape, 120 μm in diameter, in ventral part of septum nasi, lying caudally to the opening of ductus incisivus. The structure of the epithelium of septal olfactory organ is clearly distinct from the respiratory epithelium of the nasal cavity. It varies in thickness, cellular composition, as well as free surface appearance, and even lack the typical structure of sensory epithelium, in this developmental period. Nerve bundles and glandular acini are lacking in the lamina propria mucosae of the septal organ and in the adjacent tissues. Glands appear as the single non-luminized cords of epithelia extending from the surface. The adjacent respiratory epithelium contains numerous goblet cells.

  12. Ablación miocárdica septal transluminal percutánea en pacientes con miocardiopatía hipertrófica septal obstructiva: Resultados agudos y seguimiento a 3 años

    OpenAIRE

    Llamas-Esperón,Guillermo A.; Loera Pinales,Armando; Sandoval Navarrete,Santiago; Zamora Muciño,Alberto; Ramírez Robledo,Miguel A.; Varela Ortiz,Samuel; Casas Juárez,Ulices; Fuantos Delgado,María de la Luz; Albarrán Domínguez,Javier; Muñoz Sandoval,Rocío; Sandoval Rodríguez,Eufracino; Ruiz Esparza,M. Eugenia

    2009-01-01

    Objetivo: Reportar los resultados agudos y a largo plazo de la ablación miocárdica septal transluminal percutánea (AMSTP) como tratamiento de la miocardiopatía hipertrófica septal obstructiva (MHSO). La AMSTP se considera una alternativa del tratamiento quirúrgico en pacientes con MHSO y sintomatología refractaria a otras opciones de tratamiento. La respuesta aguda suele ser satisfactoria, pero los resultados a largo plazo no se han descrito de forma suficiente. Métodos: Realizamos AMSTP en 8...

  13. Septal penetration correction in I-131 imaging following thyroid cancer treatment

    Science.gov (United States)

    Barrack, Fiona; Scuffham, James; McQuaid, Sarah

    2018-04-01

    Whole body gamma camera images acquired after I-131 treatment for thyroid cancer can suffer from collimator septal penetration artefacts because of the high energy of the gamma photons. This results in the appearance of ‘spoke’ artefacts, emanating from regions of high activity concentration, caused by the non-isotropic attenuation of the collimator. Deconvolution has the potential to reduce such artefacts, by taking into account the non-Gaussian point-spread-function (PSF) of the system. A Richardson–Lucy deconvolution algorithm, with and without prior scatter-correction was tested as a method of reducing septal penetration in planar gamma camera images. Phantom images (hot spheres within a warm background) were acquired and deconvolution using a measured PSF was applied. The results were evaluated through region-of-interest and line profile analysis to determine the success of artefact reduction and the optimal number of deconvolution iterations and damping parameter (λ). Without scatter-correction, the optimal results were obtained with 15 iterations and λ  =  0.01, with the counts in the spokes reduced to 20% of the original value, indicating a substantial decrease in their prominence. When a triple-energy-window scatter-correction was applied prior to deconvolution, the optimal results were obtained with six iterations and λ  =  0.02, which reduced the spoke counts to 3% of the original value. The prior application of scatter-correction therefore produced the best results, with a marked change in the appearance of the images. The optimal settings were then applied to six patient datasets, to demonstrate its utility in the clinical setting. In all datasets, spoke artefacts were substantially reduced after the application of scatter-correction and deconvolution, with the mean spoke count being reduced to 10% of the original value. This indicates that deconvolution is a promising technique for septal penetration artefact reduction that

  14. Coronary sinus and atrioventricular groove avulsion after motor vehicle crash

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    Bradley M Dennis

    2014-01-01

    Full Text Available Simultaneous cardiac and pericardial rupture from blunt chest trauma is a highly lethal combination with rarely reported survival. We report of a case of young patient with a right atrioventricular groove injury, pericardial rupture and a unique description of a coronary sinus avulsion following blunt chest trauma. Rapid recognition of this injury is crucial to patient survival, but traditional diagnostic adjuncts such as ultrasound, echocardiography and computed tomography are often unhelpful. Successful repair of these injuries requires high suspicion of injury, early cardiac surgery involvement of and possible even placement of the patient on cardiopulmonary bypass.

  15. Congenital heart defects in children with oral clefts

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    Nahvi H.

    2007-09-01

    Full Text Available   Background: Oral clefts are among the most common congenital anomalies. Infants with oral clefts often have other associated congenital defects, especially congenital heart defects. The reported incidences and the types of associated malformations and congenital heart defects vary between different studies. The purpose of this study was to assess the incidence of associated congenital heart defects in children with oral clefts. Methods: All infants with cleft lip and palate referred to the Children's Medical Center and Bahramy; the teaching Hospitals of the Tehran University of Medical Sciences from 1991 to 2005 were prospectively enrolled in this study group. All patients were examined and noted by an academic cleft team contain; a pediatrician and a pediatric surgeon, and received cardiac consultation and echocardiography by a pediatric cardiologist. non cardiac associated anomalies, still born and patients without echocardiography were excluded from the study.  Data including age, gender, exposure to contagions and high risk elements ,consanguinity and familial history of oral cleft, type of oral cleft, results of cardiac consultation and echocardiography and associated cardiac anomalies were cumulated and analyzed by SSPS version 13.5Results: Among the 284 infants with oral clefts, 162 were male (57% and 122 were female (43%. Seventy-nine patients (27.8% had cleft lip, 84 (29.5% had cleft palate and 121 (42.6% had both cleft lip and palate. Of all the patients, 21.1% had congenital heart defects. the most common type Of these congenital heart defects(28.3%  was atrial septal defect.Conclusions: For patients with cleft lip and palate, we recommend preoperative cardiac consultation, careful examination and routine echocardiography for associated cardiac anomalies, as well as appropriate management and prophylactic antibiotic therapy for those with associated congenital heart anomaly.

  16. Myocardial Tbx20 regulates early atrioventricular canal formation and endocardial epithelial-mesenchymal transition via Bmp2

    NARCIS (Netherlands)

    Cai, Xiaoqiang; Nomura-Kitabayashi, Aya; Cai, Weibin; Yan, Jianyun; Christoffels, Vincent M.; Cai, Chen-Leng

    2011-01-01

    During early embryogenesis, the formation of the cardiac atrioventricular canal (AVC) facilitates the transition of the heart from a linear tube into a chambered organ. However, the genetic pathways underlying this developmental process are poorly understood. The T-box transcription factor Tbx20 is

  17. Role of adenosine 99MTc sestamibi myocardial perfusion spect in evaluation of symptomatic hypertensives with recent onset left bundle branch block

    International Nuclear Information System (INIS)

    Padma, S.; Sundaram, P.S.; Zachariah, M.; Hariads, K.K.

    2004-01-01

    Objectives: Adenosine pharmacological stress Myocardial Perfusion Imaging (AMPI) is the preferred imaging modality for IHD evaluation in pts with Left Bundle Branch Block (LBBB). Good prognosis has been reported in recent onset LBBB pts with normal MPI. Our aim was to evaluate the utility and analyze the predictive value of SestaMIBI AMPI in symptomatic recent onset LBBB hypertensives for coronary artery disease evaluation. Methods: 29 hypertensive pts, age range 36-72yrs, mean 54+ 6 yrs, M:F- 21:8 with recent onset complete LBBB, under evaluation for typical/atypical angina who had AMPI between Nov 02-Nov 03, were retrospectively analyzed. LBBB was diagnosed ECG wise if there is prolonged QRS complex i.e. 0.125sec or more in sinus/supraventricular rhythm, a QS or rS complex in lead 1, and an R-wave peak time of 0.06sec in leads 1, V5 or V6 with no Q wave in same lead. Pts with prior Myocardial Infarct / revasularisation, pacemaker induced LBBB were excluded. Single day 6 min. Adenosine infusion Stress-rest 99m Tc MIBI gated MPI was performed with a dual head variable angle Gamma Camera at 76-degree angle. Coronary Angiogram (CAG) correlation was available in 24 pts. Results: Myocardial perfusion SPECT images were assessed visually and by a 20-segment model. Location, extent and severity of the LV perfusion defects were evaluated. 6/29 pts (20.7%) had normal stress-rest perfusion in all LV segments.12 (41.4%) had isolated septal fixed defect with normal perfusion in rest of the segments.3 pts (10.3%)showed isolated reversible septal defects while 8(27.6%) had reversible septal defects with reversible defects in other LV segments also. Pts with fixed septal perfusion defect showed moderate to good septal wall thickening in gated SPECT images. While all pts with multiple reversible perfusion defects had angiographically proven coronary artery disease (i.e. 3pts had LAD lesion, 2 had Triple vessel and 3 showed double vessel lesions), 1 out of 3 pts with isolated

  18. The double switch for atrioventricular discordance.

    Science.gov (United States)

    Brawn, William J

    2005-01-01

    Conventional surgery for atrioventricular discordance usually associated with ventricular arterial discordance leaves the morphologic right ventricle in the systemic circulation. Long-term follow-up results with this approach reveal a high incidence of right ventricular failure. The double switch procedure was introduced to restore the morphologic left ventricle to the systemic circulation. This operation is performed in two main ways: the atrial-arterial switch and the atrial switch plus Rastelli procedure. This double switch approach has been successful at least in the medium term in abolishing morphologic right ventricular failure and its associated tricuspid valve regurgitation. In the atrial-arterial switch group, there is an incidence of morphologic left ventricular dysfunction, sometimes associated with neoaortic valve regurgitation, and the minority of cases need aortic valve replacement. The long-term function of the morphologic left ventricle and the aortic valve need careful surveillance in the future. The atrial-Rastelli group of patients has not in the medium term shown evidence of ventricular dysfunction but will require change on a regular basis of their ventricular to pulmonary artery valved conduits.

  19. Limited diagnostic accuracy of gated myocardial perfusion SPECT for wall motion analysis in patients with asymmetric septal hypertrophy

    International Nuclear Information System (INIS)

    Seo, J.H.; Ahn, B.C.; Bae, J.H.; Jeong, S.Y.; Lee, J.; Lee, K.B.

    2004-01-01

    Objective: Although gated SPECT(G-SPECT) using Tc-99m MIBI is well-known diagnostic modality in the evaluation of myocardial perfusion and wall motion analysis, there were limited reports for subjects with asymmetric septal hypertrophy (ASH). This study was performed to evaluate the clinical usefulness of G-SPECT for assessments of myocardial perfusion and wall motion analysis in patients with ASH on 2D-echocardiography(Echo). Methods: Thirty patients (male 18, 59 12 years) with ASH on Echo (septal wall thickness 13 mm and 1.3 times as thick as that of posterior wall) underwent Tc-99m MIBI G-SPECT. Two studies were performed within one month. No patient had experienced any significant cardiac event, nor had changed medical and surgical therapy during the studies. Functional parameters of the left ventricle were acquired with QGS software(AutoQUANTTM). Three experts performed visual interpretation for the presence of septal thickening and perfusion abnormalities on G-SPECT and two experienced cardiologists measured dimension, thickness and wall motion of the left ventricle on Echo. Results: Mean septum thickness measured by Echo was 1.90 0.50 cm, and the septum/posterior wall thickness ratio was 1.85 0.51. On visual SPECT analysis, 14 patients (46.7%) were interpreted as with thickened septum and 17 patients (57%) as with abnormal perfusion. All 3 patients who underwent coronary angiography showed significant luminal stenosis and also had perfusion abnormalities on SPECT. On Echo, only one patient showed septal hypokinesia, who showed anteroseptal infarction on SPECT, and the others showed normal septal wall motion. But 13 patients (54%) among 24 patients showed septal hypokinesia on G-SPECT. Patients with thickened septum on SPECT had thicker septum (2.3 vs 1.6 cm) and higher septum/posterior wall thickness ratio (2.2 vs 1.6) on Echo, compared with patients without septal thickening on SPECT. Conclusions: Although G-SPECT could proffer diagnostic accuracy for

  20. Unusual diagnosis of a persistent third-degree atrioventricular block during anesthesia in a "healthy" pediatric patient

    Directory of Open Access Journals (Sweden)

    Yasser Alsayegh

    2012-01-01

    Full Text Available We present an unusual diagnosis of a permanent third-degree atrioventricular block under general anesthesia in an otherwise healthy and asymptomatic child. This diagnosis of unclear causality represented a serious rare finding, requiring judicious management and resulting in the placement of a permanent pacemaker.

  1. Transcatheter closure, mini-invasive closure and open-heart surgical repair for treatment of perimembranous ventricular septal defects in children: a protocol for a network meta-analysis.

    Science.gov (United States)

    You, Tao; Yi, Kang; Ding, Zhao-Hong; Hou, Xiao-Dong; Liu, Xing-Guang; Wang, Xin-Kuan; Ge, Long; Tian, Jin-Hui

    2017-06-21

    Both transcatheter device closure and surgical repair are effective treatments with excellent midterm outcomes for perimembranous ventricular septal defects (pmVSDs) in children. The mini-invasive periventricular device occlusion technique has become prevalent in research and application, but evidence is limited for the assessment of transcatheter closure, mini-invasive closure and open-heart surgical repair. This study comprehensively compares the efficacy, safety and costs of transcatheter closure, mini-invasive closure and open-heart surgical repair for treatment of pmVSDs in children using Bayesian network meta-analysis. A systematic search will be performed using Chinese Biomedical Literature Database, China National Knowledge Infrastructure, PubMed, EMBASE.com and the Cochrane Central Register of Controlled Trials to include random controlled trials, prospective or retrospective cohort studies comparing the efficacy, safety and costs of transcatheter closure, mini-invasive closure and open-heart surgical repair. The risk of bias for the included prospective or retrospective cohort studies will be evaluated according to the risk of bias in non-randomised studies of interventions (ROBINS-I). For random controlled trials, we will use risk of bias tool from Cochrane Handbook version 5.1.0. A Bayesian network meta-analysis will be conducted using R-3.3.2 software. Ethical approval and patient consent are not required since this study is a network meta-analysis based on published trials. The results of this network meta-analysis will be submitted to a peer-reviewed journal for publication. CRD42016053352. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  2. Intracardiac echo-guided radiofrequency catheter ablation of atrial fibrillation in patients with atrial septal defect or patent foramen ovale repair: a feasibility, safety, and efficacy study.

    Science.gov (United States)

    Lakkireddy, Dhanunjaya; Rangisetty, Umamahesh; Prasad, Subramanya; Verma, Atul; Biria, Mazda; Berenbom, Loren; Pimentel, Rhea; Emert, Martin; Rosamond, Thomas; Fahmy, Tamer; Patel, Dimpi; Di Biase, Luigi; Schweikert, Robert; Burkhardt, David; Natale, Andrea

    2008-11-01

    Intracardiac Echo-Guided Radiofrequency Catheter. Patients with atrial septal defect (ASD) are at higher risk for atrial fibrillation (AF) even after repair. Transseptal access in these patients is perceived to be difficult. We describe the feasibility, safety, and efficacy of pulmonary vein antral isolation (PVAI) in these patients. We prospectively compared post-ASD/patent foramen ovale (PFO) repair patients (group I, n = 45) with age-gender-AF type matched controls (group II, n = 45). All the patients underwent PVAI through a double transseptal puncture with a roving circular mapping catheter technique guided by intracardiac echocardiography (ICE). The short-term (3 months) and long-term (12 month) failure rates were assessed. In group I, 23 (51%) had percutaneous closure devices and 22 (49%) had a surgical closure. There was no significant difference between group I and II in the baseline characteristics. Intracardiac echo-guided double transseptal access was obtained in 98% of patients in group I and in 100% of patients in group II. PVAI was performed in all patients, with right atrial flutter ablation in 7 patients in group I and in 4 patients in group II. Over a mean follow-up of 15 +/- 4 months, group I had higher short-term (18% vs 13%, P = 0.77) and long-term recurrence (24% vs 18%, P = 0.6) than group II. There was no significant difference in the perioperative complications between the two groups. Echocardiography at 3 months showed interatrial communication in 2 patients in group I and 1 patient in group II, which resolved at 12 months. Percutaneous AF ablation using double transseptal access is feasible, safe, and efficacious in patients with ASD and PFO repairs.

  3. Transposition of the great vessels

    Science.gov (United States)

    ... as atrial septal defect, ventricular septal defect, or patent ductus arteriosus) and how much the blood can ... develop, get worse, or continue after treatment. Prevention Women who plan to become pregnant should be immunized ...

  4. Comparison of right ventricular septal pacing and right ventricular apical pacing in patients receiving cardiac resynchronization therapy defibrillators: the SEPTAL CRT Study

    Science.gov (United States)

    Leclercq, Christophe; Sadoul, Nicolas; Mont, Lluis; Defaye, Pascal; Osca, Joaquim; Mouton, Elisabeth; Isnard, Richard; Habib, Gilbert; Zamorano, Jose; Derumeaux, Genevieve; Fernandez-Lozano, Ignacio; Dupuis, Jean-Marc; Rouleau, Frédéric; Tassin, Aude; Bordachar, Pierre; Clémenty, Jacques; Lafitte, Stephane; Ploux, Sylvan; Reant, Patricia; Ritter, Philippe; Defaye, Pascal; Jacon, Peggy; Mondesert, Blandine; Saunier, Carole; Vautrin, Estelle; Kacet, Salem; Guedon-Moreau, Laurence; Klug, Didier; Kouakam, Claude; Marechaux, Sylvestre; Marquie, Christelle; Polge, Anne Sophie; Richardson, Marjorie; Chevallier, Philippe; De Breyne, Brigitte; Lotek, Marcin M.; Nonin, Emilie; Pineau, Julien; Deharo, Jean-Claude; Bastard, Emilie; Franceschi, Frédéric; Habib, Gilbert; Jego, Christophe; Peyrouse, Eric; Prevot, Sebastien; Saint-Joseph, Hôpital; Bremondy, Michel; Faure, Jacques; Ferracci, Ange; Lefevre, Jean; Pisapia, Andre; Davy, Jean-Marc; Cransac, Frederic; Cung, Tien Tri; Georger, Frederic; Pasquie, Jean-Luc; Raczka, Franck; Sportouch-Dukhan, Catherine; Sadoul, Nicolas; Blangy, Hugues; Bruntz, Jean-François; Freysz, Luc; Groben, Laurent; Huttin, Olivier; Bammert, Antoine; Burban, Marc; Cebron, Jean-Pierre; Gras, Daniel; Frank, Robert; Duthoit, Guillaume; Hidden-Lucet, Françoise; Himbert, Caroline; Isnard, Richard; Lacotte, Jérôme; Pousset, Françoise; Zerah, Thierry; Leclercq, Christophe; Bellouin, Annaïk; Crocq, Christophe; Deplace, Christian; Donal, Erwan; Hamon, Cécile; Mabo, Philippe; Romain, Olivier; Solnon, Aude; Frederic, Anselme; Bauer, Fabrice; Bernard, Mathieu; Godin, Benedicte; Kurtz, Baptiste; Savoure, Arnaud; Copie, Xavier; Lascault, Gilles; Paziaud, Olivier; Piot, Olivier; Touche, Thierry; Delay, Toulouse Marc; Chilon, Talia; Detis, Nicolas; Duparc, Alexandre; Hebrard, Aurélien; Massabuau, Pierre; Maury, Philippe; Mondoly, Pierre; Rumeau, Philippe; Pasteur, Clinique; Boveda, Serge; Adrover, Laurence; Combes, Nicolas; Deplagne, Antoine; Marco-Baertich, Isabelle; Fondard, Olivier; Martínez, Juan Gabriel; Ibañez Criado, José Luis; Ortuño, Diego; Mont, Lluis; Berruezo, Antonio; Eduard, Belu; Martín, Ana; Merschon, Franco M.; Sitges, Marta; Tolosana, José María; Vidal, Bárbara; Hebron, H. Valle; i Mitjans, Angel Moya; Rodriguez, Oscar Alcalde; Rodriguez Palomares, José Fernando; Rivas, Nuria; Teixidó, Gisela; de Hierro, H. Puerta; Lozano, Ignacio Fernández; Ruiz Bautista, Maria Lorena; Castro, Victor; Cavero, Miguel Angel; Gutierrez, Carlos; Ros, Natalia; de la Victoria, H. Virgen; Alzueta Rodriguez, Francisco Javier; Cabrera, Fernando; Cordero, Alberto Barrera; Peña, José Luis; de Valme Sevilla, H.; Gonzáles, Juan Lealdel Ojo; Garcia Medina, Mª Dolores; Jiménez, Ricardo Pavón; Villagomez, David; de la Salud Toledo, H. Virgen; Castellanos Martinez, Eduardo; Alcalá, Juan; Maicas, Carolina; Arias Palomares, Miguel Angel; Puchol, Alberto; Valencia, H. La Fé; OscaAsensi, Joaquim; Carmona, Anastasio Quesada; De Carranza, Mª José Sancho-Tello; De Ros, José Olagüe; Pareja, Enrique Castro; Pérez, Oscar Cano; Saez, Ana Osa; Hortega, H. Rio; Guilarte, Benito Herreros; Muñoz San Jose, Juan Francisco; Pérez Sanz, Teresa Myriam; Logeart, Damien; Gil, Maria Lopez; Leclercq, Christophe; Lozano, Ignacio Fernandez; de Hierro, H. Puerta; Derumeaux, Genevieve

    2016-01-01

    Abstract Aims Cardiac resynchronization therapy (CRT) is a recommended treatment of heart failure (HF) patients with depressed left ventricular ejection fraction and wide QRS. The optimal right ventricular (RV) lead position being a matter of debate, we sought to examine whether RV septal (RVS) pacing was not inferior to RV apical (RVA) pacing on left ventricular reverse remodelling in patients receiving a CRT-defibrillator. Methods and results Patients (n = 263, age = 63.4 ± 9.5 years) were randomly assigned in a 1:1 ratio to RVS (n = 131) vs. RVA (n = 132) pacing. Left ventricular end-systolic volume (LVESV) reduction between baseline and 6 months was not different between the two groups (−25.3 ± 39.4 mL in RVS group vs. −29.3 ± 44.5 mL in RVA group, P = 0.79). Right ventricular septal pacing was not non-inferior (primary endpoint) to RVA pacing with regard to LVESV reduction (average difference = −4.06 mL; P = 0.006 with a −20 mL non-inferiority margin). The percentage of ‘echo-responders’ defined by LVESV reduction >15% between baseline and 6 months was similar in both groups (50%) with no difference in the time to first HF hospitalization or death (P = 0.532). Procedural or device-related serious adverse events occurred in 68 patients (RVS = 37) with no difference between the two groups (P = 0.401). Conclusion This study demonstrates that septal RV pacing in CRT is non-inferior to apical RV pacing for LV reverse remodelling at 6 months with no difference in the clinical outcome. No recommendation for optimal RV lead position can hence be drawn from this study. ClinicalTrials. gov number NCT 00833352. PMID:26374852

  5. The Role of Cerl2 in the Establishment of Left-Right Asymmetries during Axis Formation and Heart Development

    Directory of Open Access Journals (Sweden)

    José A. Belo

    2017-12-01

    Full Text Available The formation of the asymmetric left-right (LR body axis is one of the fundamental aspects of vertebrate embryonic development, and one still raising passionate discussions among scientists. Although the conserved role of nodal is unquestionable in this process, several of the details around this signaling cascade are still unanswered. To further understand this mechanism, we have been studying Cerberus-like 2 (Cerl2, an inhibitor of Nodal, and its role in the generation of asymmetries in the early vertebrate embryo. The absence of Cerl2 results in a wide spectrum of malformations commonly known as heterotaxia, which comprises defects in either global organ position (e.g., situs inversus totalis, reversed orientation of at least one organ (e.g., situs ambiguus, and mirror images of usually asymmetric paired organs (e.g., left or right isomerisms of the lungs. Moreover, these laterality defects are frequently associated with congenital heart diseases (e.g., transposition of the great arteries, or atrioventricular septal defects. Here, reviewing the knowledge on the establishment of LR asymmetry in mouse embryos, the emerging conclusion is that as necessary as is the activation of the Nodal signaling cascade, the tight control that Cerl2-mediates on Nodal signaling is equally important, and that generates a further regionalized LR genetic program in the proper time and space.

  6. Interventricular Septal Hematoma and Coronary-Ventricular Fistula: A Complication of Retrograde Chronic Total Occlusion Intervention

    OpenAIRE

    Abdul-rahman R. Abdel-karim; Minh Vo; Michael L. Main; J. Aaron Grantham

    2016-01-01

    Interventricular septal hematoma is a rare complication of retrograde chronic total occlusion (CTO) percutaneous coronary interventions (PCI) with a typically benign course. Here we report two cases of interventricular septal hematoma and coronary-cameral fistula development after right coronary artery (RCA) CTO-PCI using a retrograde approach. Both were complicated by development of ST-segment elevation and chest pain. One case was managed actively and the other conservatively, both with a f...

  7. Use of Non-Invasive Phase Contrast Magnetic Resonance Imaging for Estimation of Atrial Septal Defect Size and Morphology: A Comparison with Transesophageal Echo

    International Nuclear Information System (INIS)

    Piaw, Chin Sze; Kiam, Ong Tiong; Rapaee, Annuar; Khoon, Liew Chee; Bang, Liew Houng; Ling, Chan Wei; Samion, Hasri; Hian, Sim Kui

    2006-01-01

    Background: Transesophageal echocardiography (TEE) is a trusted method of sizing atrial septal defect (ASD) prior to percutaneous closure but is invasive, uncomfortable, and may carry a small risk of morbidity and mortality. Magnetic resonance imaging (MRI) may be useful non-invasive alternative in such patients who refuse or are unable to tolerate TEE and may provide additional information on the shape of the A0SD. Purpose: To validate the accuracy of ASD sizing by MRI compared with TEE.Method: Twelve patients (mean age 30 years; range 11-60 years) scheduled for ASD closure underwent TEE, cine balanced fast field echo MRI (bFFE-MRI) in four-chamber and sagittal views and phase-contrast MRI (PC-MRI) with reconstruction using the two orthogonal planes of T2-weighted images as planning. The average of the three longest measurements for all imaging modalities was calculated for each patient. Results: Mean maximum ASD length on TEE was 18.8 ± 4.6 mm, mean length by bFFE-MRI was 20.0 ± 5.0 mm, and mean length by PC-MRI was 18.3 ± 3.6 mm. The TEE measurement was significantly correlated with the bFFE-MRI and PC-MRI measurements (Pearson r = 0.69, p = 0.02 and r = 0.59, p = 0.04, respectively). The mean difference between TEE and bFFE-MRI measurements was -1.2mm (95% CI: -3.7, 1.3) and between TEE and PC-MRI was 0.5 mm (95% CI: -1.9, 2.9). Bland-Altman analysis also determined general agreement between both MRI methods and TEE. The ASDs were egg-shaped in two cases, circular in 1 patient and oval in the remaining patients. Conclusion: ASD sizing by MRI using bFFE and phase-contrast protocols correlated well with TEE estimations. PC-MRI provided additional information on ASD shapes and proximity to adjacent structures

  8. Post-operative ventricular flow dynamics following atrioventricular valve surgical and device therapies: A review.

    Science.gov (United States)

    Nguyen, Yen Ngoc; Ismail, Munirah; Kabinejadian, Foad; Tay, Edgar Lik Wui; Leo, Hwa Liang

    2018-04-01

    Intra-ventricular flow dynamics has recently emerged as an important evaluation and diagnosis tool in different cardiovascular conditions. The formation of vortex pattern during the cardiac cycle has been suggested to play important epigenetic and energy-modulation roles in cardiac remodelling, adaptations and mal-adaptations. In this new perspective, flow alterations due to different cardiovascular procedures can affect the long-term outcome of those procedures. Especially, repairs and replacements performed on atrioventricular valves are likely to exert direct impact on intra-ventricular flow pattern. In this review, current consensus around the roles of vortex dynamics in cardiac function is discussed. An overview of physiological vortex patterns found in healthy left and right ventricles as well as post-operative ventricular flow phenomenon owing to different atrioventricular valvular procedures are reviewed, followed by the summary of different vortex identification schemes used to characterise intraventricular flow. This paper also emphasises on future research directions towards a comprehensive understanding of intra-cardiac flow and its clinical relevance. The knowledge could encourage more effective pre-operative planning and better outcomes for current clinical practices. Copyright © 2018. Published by Elsevier Ltd.

  9. Anatomy of right superior septal artery demonstrated on the coronary CT scan

    Energy Technology Data Exchange (ETDEWEB)

    Takeguchi, Takaya (Mitsui Memorial Hospital, Department of Diagnostic Radiology, Tokyo (Japan); Tokyo Medical and Dental University, Department of Diagnostic Radiology, Tokyo (Japan)); Ibukuro, Kenji; Fukuda, Hozumi; Tobe, Kimiko; Abe, Shoko (Mitsui Memorial Hospital, Department of Diagnostic Radiology, Tokyo (Japan)), Email: kj-ibkr@qd6.so-net.ne.jp

    2012-02-15

    Background. A coronary CT scan allows for non-invasive visualization of the anatomy of a coronary artery in three dimensions compared to the two dimensions afforded by conventional angiography. The septal artery, the main blood source of the interventricular septum, is usually derived from the left anterior descending artery; however, it is occasionally derived from the right coronary artery. Purpose. To analyze the prevalence, origin, diameter, and length of the right superior septal artery (RSSA) demonstrated on a coronary CT scan. Material and Methods. The right superior septal artery was retrospectively reviewed on the reconstructed axial scan images (0.5-mm thickness, 0.25-mm interval) in 1290 consecutive patients who underwent coronary CT scans. All patients were scanned on a 320-row CT scanner. The images were transferred to a workstation to trace the vessel to analyze the origin, diameter, and length. We also compared the length of the RSSA between patients with and without coronary artery stenosis. Results. The RSSA was identified in 51 (3.9%) of 1290 patients. The origin was the proximal portion of the right coronary artery (n = 40) or the right sinus of Valsalva (n 11). The artery co-existed with the conus artery in 15 (29%) of 51 patients. The length was 16-62 mm (mean 31.2 mm +- 10.5), and the diameter was 0.8-2.0 mm (mean 1.3 mm +- 0.2). Longer RSSAs tended to be demonstrated in the patients with coronary artery stenosis rather than with normal coronary arteries (P < 0.05). Conclusion. The right superior septal artery and its anatomical variant could be analyzed with a coronary CT scan. The ability to demonstrate this artery on the coronary CT scan was the same as with coronary angiography. The recognition of this vessel is useful for physicians managing with the diagnosis and treatment of the coronary artery disease

  10. Successful radiofrequency catheter ablation of atrioventricular nodal reentrant tachycardia in a patient with dextrocardia due to unilateral pulmonary agenesis: a case report

    Directory of Open Access Journals (Sweden)

    Aksu T

    2015-02-01

    Full Text Available Tolga Aksu, Tumer Erdem Guler, Ebru Golcuk, Ismail Erden, Kazim Serhan Ozcan Department of Cardiology, Kocaeli Derince Education and Research Hospital, Derince, Kocaeli, Turkey Abstract: Radiofrequency catheter ablation of the slow pathway is considered to be the treatment of choice for patients with atrioventricular nodal reentrant tachycardia. We report a 34-year-old female with mirror image dextrocardia due to unilateral pulmonary agenesis who underwent successful slow pathway ablation for typical atrioventricular nodal reentrant tachycardia. Using contrast injection, cardiac anatomy was identified in a short time and successfully ablated. Keywords: dextrocardia, AVNRT, ablation, pulmonary agenesis

  11. Fallot's tetralogy in combination with an open common atrioventricular canal

    International Nuclear Information System (INIS)

    Alekyan, B.G.; Dvinyaninova, N.B.; Chiaureli, M.R.; Arzhanukhina, S.Yu.

    1987-01-01

    The methods and clinics of diagnostics of Fallot tetrad (FT) combined with the opened general atrioventricular canal (AVC) of the heart are described on the basis of observation of a 6.5-year old patient and analysis of literary data. On the basis of studying clinical data, electro-, phono-, echocardiography and roentgenography, as well as heart catheterization and angiocardiography the conclusion is made that nowadays the most informative method of diagnostics of FT and AVC combination before angiocardiography is a dimer echocardiography which permits to diagnoze AVC. Angiocardiographic study proves this diagnosis and gives a precise anotomical picture of various components of this combination of heart failure

  12. VACTERL association with double-chambered left ventricle: A rare occurrence

    Directory of Open Access Journals (Sweden)

    Abdulla Al-Farqani

    2013-01-01

    Full Text Available VACTERL association is a non-random association of birth defects of unknown etiology derived from structures of embryonic mesoderm. The common cardiac defects seen with VACTERL association are ventricular septal defects, atrial septal defects, and tetralogy of Fallot. We present a 2-year-old child with VACTERL association in whom we detected double-chambered left ventricle on transthoracic echocardiography.

  13. The role of septal surgery in management of the deviated nose.

    Science.gov (United States)

    Foda, Hossam M T

    2005-02-01

    The deviated nose represents a complex cosmetic and functional problem. Septal surgery plays a central role in the successful management of the externally deviated nose. This study included 260 patients seeking rhinoplasty to correct external nasal deviations; 75 percent of them had various degrees of nasal obstruction. Septal surgery was necessary in 232 patients (89 percent), not only to improve breathing but also to achieve a straight, symmetrical, external nose as well. A graduated surgical approach was adopted to allow correction of the dorsal and caudal deviations of the nasal septum without weakening its structural support to the dorsum or nasal tip. The approach depended on full mobilization of deviated cartilage, followed by straightening of the cartilage and its fixation in the corrected position by using bony splinting grafts through an external rhinoplasty approach.

  14. Thoracic aortic dissection and rupture in conotruncal cardiac defects: A population-based study.

    Science.gov (United States)

    Frischhertz, Benjamin P; Shamszad, Pirouz; Pedroza, Claudia; Milewicz, Dianna M; Morris, Shaine A

    2015-04-01

    Although the risk of thoracic aortic dissection and rupture (TAD) is well-known in bicuspid aortic valve (BAV), the risk of TAD in other congenital heart diseases (CHD), particularly conotruncal lesions like tetralogy of Fallot (TOF), truncus arteriosus, D-transposition of the great arteries (D-TGA), and double outlet right ventricle is currently unknown. The primary purpose of this study was to describe TAD in conotruncal CHD, and the secondary purpose was to explore whether an association exists between TAD and conotruncal CHD. Using the Texas Inpatient Public Use Data File, an administrative database of all Texas hospitalizations, including >37.9 million hospitalizations from January 1999 through June 2012, 12,016 cases of TAD and 214 cases of TAD in CHD were identified. The most common lesions were BAV (42%), atrial septal defect (21%), aortic coarctation (7%), ventricular septal defect (6%), and patent ductus arteriosus (4%). Three patients with TOF, 2 with D-TGA, and 1 with truncus arteriosus were admitted with TAD. An exploratory case-control study in patients older than 1 year using multilevel logistic regression models to evaluate the association between CHD and TAD that controlled for known TAD risk factors demonstrated a significant association between TAD and BAV (OR 10, 95% CI 8.2-13) but not coarctation of the aorta or any conotruncal lesion. TAD in conotruncal CHD is exquisitely rare. In our hospitalized population, there was no increased occurrence of TAD in conotruncal CHD above what would be expected in the rest of the hospitalized population. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  15. Effect of amiodarone-induced hyperthyroidism on left ventricular outflow obstruction after septal myectomy for hypertrophic cardiomyopathy.

    Science.gov (United States)

    Pokorney, Sean D; Stone, Neil J; Passman, Rod; Oyer, David; Rigolin, Vera H; Bonow, Robert O

    2010-12-01

    Patients with obstructive hypertrophic cardiomyopathy who undergo septal myectomy are at risk for developing postoperative atrial fibrillation. Amiodarone is effective in treating this arrhythmia but is associated with multiple adverse effects, often with delayed onset. A novel case is described of a patient who developed type 2 amiodarone-induced hyperthyroidism that presented as recurrence of outflow obstruction after septal myectomy. The patient's symptoms and echocardiographic findings of outflow obstruction resolved substantially with the treatment of the amiodarone-induced hyperthyroidism. Amiodarone-induced hyperthyroidism of delayed onset can be a subtle diagnosis, requiring a high index of suspicion. In conclusion, recognition of this diagnosis in patients with recurrence of outflow obstruction by symptoms and cardiac imaging after septal myectomy may avoid unnecessary repeat surgical intervention. Copyright © 2010 Elsevier Inc. All rights reserved.

  16. Myocardial glucose metabolism is different between hypertrophic cardiomyopathy and hypertensive heart disease associated with asymmetrical septal hypertrophy

    International Nuclear Information System (INIS)

    Shiba, Nobuyuki; Kagaya, Yutaka; Ishide, Nobumasa; Takeyama, Daiya; Yamane, Yuriko; Chida, Masanobu; Otani, Hiroki; Shirato, Kunio; Ido, Tatsuo.

    1997-01-01

    Myocardial glucose metabolism has been shown to be heterogeneous in patients with hypertrophic cardiomyopathy (HCM). We tested the hypothesis that myocardial glucose metabolism differs between patients with HCM and those with hypertensive heart disease (HHD) associated with asymmetrical septal hypertrophy. We studied 12 patients with HCM, 7 HHD patients associated with asymmetrical septal hypertrophy using 18 F 2-deoxyglucose (FDG) and positron emission tomography. We calculated % FDG fractional uptake in the interventricular septum and posterolateral wall. Heterogeneity of FDG uptake was evaluated by % interregional coefficient of variation of FDG fractional uptake in each wall segment. In both the interventricular septum and posterolateral wall, % FDG fractional uptake was not significantly different between the two groups. The % interregional coefficient of variation for both interventricular septum (10.6±1.6 vs. 4.1±0.5, p<0.01) and posterolateral wall (5.9±0.7 vs. 3.8±0.5, p< 0.05) was significantly larger in patients with HCM than in HHD patients associated with asymmetrical septal hypertrophy. Echocardiography demonstrated that the degree of asymmetrical septal hypertrophy was similar between the two groups. These results suggest that myocardial glucose metabolism may be more heterogeneous in patients with HCM compared to HHD patients associated with asymmetrical septal hypertrophy, although the left ventricular shape is similar. The difference in the heterogeneity might have resulted from differences in the pathogeneses of the two diseases. (author)

  17. Reversible complete atrioventricular block in patient with wegener's granulomatosis - a report on fortunate outcome with long term follow-up.

    Science.gov (United States)

    Steckiewicz, Roman; Rosiak, Marek; Stolarz, Przemysław; Świętoń, Elżbieta B; Grabowski, Marcin; Kosior, Dariusz A

    Reversible complete atrioventricular block in patient with Wegener's granulomatosis - a report on a positive outcome with long term follow-up. Atrioventricular (AV) block is a rare complication of Wegener's granulomatosis (WG), thus there are no standards of management in such cases. We present a case of a patient with a dual-chamber pacemaker (DDD) implanted due to complete AV block in the course of Wegener's granulomatosis (WG). An immunosuppressive therapy resulted in the resolution of non-cardiac and AV conduction disorders. The diagnostic functions of the pacemaker enabled us to evaluate AV conduction over a five-year follow-up period. The resolution of AV conduction disorders, which accompanied WG remission, suggests that careful monitoring with temporary cardiac pacing may be considered in some patients before permanent pacemaker implantation.

  18. Alarm!!! A UFO inside the heart.

    Science.gov (United States)

    Santoro, Giuseppe; Castaldi, Biagio; Iacono, Carola; Giugno, Luca; Gaio, Gianpiero; Russo, Maria G

    2012-10-01

    An 8-year-old asymptomatic child was referred for surgical repair of coronary sinus atrial septal defect resulting in significant left-to-right shunt and right chamber volume overload. The septal fenestration was located near to its drainage site into the right atrium. Due to this seemingly favourable anatomy, transcatheter closure of the septal defect was performed using an Amplatzer Septal Occluder device. The echocardiographic postprocedural evaluation imaged the occluding device almost perpendicular to the atrial septum, seemingly floating above the mitral valve orifice, like an alien spaceship inside the heart.

  19. Postural Tachycardia Syndrome and Vasovagal Syncope: A Hidden Case of Obstructive Cardiomyopathy without Severe Septal Hypertrophy.

    Science.gov (United States)

    Mayuga, Kenneth A; Ho, Natalie; Shields, Robert W; Cremer, Paul; Rodriguez, L Leonardo

    2018-01-01

    A 36-year-old female with symptoms of orthostatic intolerance and syncope was diagnosed with vasovagal syncope on a tilt table test and with postural tachycardia syndrome (POTS) after a repeat tilt table test. However, an echocardiogram at our institution revealed obstructive cardiomyopathy without severe septal hypertrophy, with a striking increase in left ventricular outflow tract gradient from 7 mmHg at rest to 75 mmHg during Valsalva, with a septal thickness of only 1.3 cm. Cardiac MRI showed an apically displaced multiheaded posteromedial papillary muscle with suggestion of aberrant chordal attachments to the anterior mitral leaflet contributing to systolic anterior motion of the mitral valve. She underwent surgery with reorientation of the posterior medial papillary muscle head, resection of the tethering secondary chordae to the A1 segment of the mitral valve, chordal shortening and tacking of the chordae to the A1 and A2 segments of the mitral valve, and gentle septal myectomy. After surgery, she had significant improvement in her prior symptoms. To our knowledge, this is the first reported case of obstructive cardiomyopathy without severe septal hypertrophy with abnormalities in papillary muscle and chordal attachment, in a patient diagnosed with vasovagal syncope and POTS.

  20. Postural Tachycardia Syndrome and Vasovagal Syncope: A Hidden Case of Obstructive Cardiomyopathy without Severe Septal Hypertrophy

    Directory of Open Access Journals (Sweden)

    Kenneth A. Mayuga

    2018-01-01

    Full Text Available A 36-year-old female with symptoms of orthostatic intolerance and syncope was diagnosed with vasovagal syncope on a tilt table test and with postural tachycardia syndrome (POTS after a repeat tilt table test. However, an echocardiogram at our institution revealed obstructive cardiomyopathy without severe septal hypertrophy, with a striking increase in left ventricular outflow tract gradient from 7 mmHg at rest to 75 mmHg during Valsalva, with a septal thickness of only 1.3 cm. Cardiac MRI showed an apically displaced multiheaded posteromedial papillary muscle with suggestion of aberrant chordal attachments to the anterior mitral leaflet contributing to systolic anterior motion of the mitral valve. She underwent surgery with reorientation of the posterior medial papillary muscle head, resection of the tethering secondary chordae to the A1 segment of the mitral valve, chordal shortening and tacking of the chordae to the A1 and A2 segments of the mitral valve, and gentle septal myectomy. After surgery, she had significant improvement in her prior symptoms. To our knowledge, this is the first reported case of obstructive cardiomyopathy without severe septal hypertrophy with abnormalities in papillary muscle and chordal attachment, in a patient diagnosed with vasovagal syncope and POTS.

  1. Tricuspid annuloplasty with the MC3 ring and septal plication technique.

    Science.gov (United States)

    Isomura, Tadashi; Hirota, Masanori; Hoshino, Joji; Fukada, Yasuhisa; Kondo, Taichi; Takahashi, Yu

    2015-01-01

    Functional tricuspid regurgitation is caused by annular dilation mainly in the posterior annulus. However, ring annuloplasty does not always prevent the recurrence of tricuspid regurgitation due to dilation of the septal annulus. We developed a septal plication technique with a 3-dimensional MC3 ring. Between 2006 and 2011, 76 patients (male/female 30/46; mean age 68 ± 11 years) with functional tricuspid regurgitation received tricuspid ring annuloplasty. After placement of the annular sutures, the 3 commissural ring portions were fixed on the equivalent commissures to plicate the anterior and posterior annulus. The end of the septal ring portion was fixed at the optimal annular position to obtain minimal tricuspid regurgitation. All patients were followed-up for a mean of 47 ± 18 months; the longest duration was 79 months. Although there was no operative death, one patient died of sepsis during hospitalization (hospital mortality 1.3%). After implantation of the MC3 ring (mean size 31.0 ± 3.3 mm), additional edge-to-edge sutures were required for minor leakage in 5 (7%) patients. The degree of tricuspid regurgitation was significantly reduced at discharge (0.5 ± 0.6) and midterm (0.6 ± 0.6) compared to 2.5 ± 0.7 before the operation (p tricuspid ring annuloplasty with a 3-dimensional MC3 ring. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  2. Study of prevalence & risk factors of congenital heart defect (Review Article

    Directory of Open Access Journals (Sweden)

    ali dehghani

    2017-09-01

    Full Text Available Abstract Background: Congenital heart defects are known as the state that comes from birth and influences on structure and function of baby's heart, The different types of defects can range from mild (e.g., a small hole between the heart chambers to hard (like a flaw or weakness in a part of the heart. Method: This article is a review article in which the articles published in Farsi and English that the bases valid as Medline, Google Scholar, Pubmed, Springer, SID index has been used, as well as for the study of keywords associated with the use of MESH keywords in identifying and no time limit listed in the databases were searched. Result The prevalence of congenital heart defect, in general, less than one per cent in newborn. The ventricular wall abnormalities defect (VSD, atrial septal defect of (ASD, patent ductus arteriosus (PDA and tetralogy of Fallot (TOF Top among the most types of congenital heart anomalies. Factors such as the age of the parents at conception, maternal risk of diabetes, influenza and febrile illness during pregnancy, drug use during pregnancy and taking a multivitamin before and during the Pregnancy were influenced of newborns with congenital heart defects. Conclusion: According to studies need to be conducted in the presence of multiple risk factors for these disorders, seem to have a detailed plan to Study of More about the factors that affect the risk of developing these disorders, as well as interventions to reduce risk factors identified particularly during pregnancy.

  3. Eventos catastróficos associados ao tratamento da comunicação interatrial tipo ostium secundum: razões para não se subestimar este tipo de cardiopatia congênita Catastrophic events associated to the surgical treatment of ostium secundum atrial septal defects: reasons for not underestimating this type of congenital cardiopathy

    Directory of Open Access Journals (Sweden)

    Paulo Roberto B. Evora

    2004-12-01

    Full Text Available OBJETIVO: O presente trabalho clínico foi motivado pela frustrante experiência de quatro pacientes operados para o tratamento cirúrgico da comunicação interatrial tipo ostium secundum (CIA-II, que vieram a falecer em condições extremamente dramáticas. MÉTODO: Estudo retrospectivo embasado em dados de prontuários. As pesquisas bibliográficas incluíram: tromboembolismo paradoxal (cerebral, pulmonar ou mesentérico, malformações vasculares do sistema nervoso central e conexões anômalas das veias cavas. Estas pesquisas da literatura foram embasadas em possíveis eventos, inesperados e catastróficos, que levaram quatro pacientes ao óbito. RESULTADOS: Os quatros pacientes, todos do sexo feminino, foram submetidos a atriosseptorrafia com tempo de parada cardíaca isquêmica inferior a 20 minutos, em circulação extracorpórea. As causas de óbito foram: isquemia intestinal não-oclusiva, ruptura de aneurisma cerebral de artéria comunicante anterior, cor pulmonale com hipertensão arterial e tromboembolismo e um provável tromboembolismo cerebral em uma criança que precisou ser reoperada pela drenagem da veia cava inferior em átrio esquerdo. CONCLUSÃO: A lição final deste trabalho é: "Não subestime a comunicação interatrial em cirurgia cardíaca!".OBJECTIVE: The present article was motivated by the frustrating experiences with four patients who underwent surgical treatment of ostium secundum atrial septal defect (ASD-II and who died in extremely dramatic circumstances. METHOD: This is a retrospective study based on clinical data. The bibliographical researche included: paradoxical thromboembolism (cerebral, lung or mesenteric, central nervous system vascular malformations and anomalous vena cava connections. This research was based on possible events, unexpected and catastrophic, that could have directly caused the patients' deaths. RESULTS: All patients were female, the operations were performed under cardiopulmonary

  4. Left ventricular filling pressure by septal and lateral E/e' equally predict cardiovascular events in the general population

    DEFF Research Database (Denmark)

    Wang, Joanna Nan; Biering-Sørensen, Tor; Jørgensen, Peter Godsk

    2017-01-01

    /e'lateral were equally strong predictors of cardiac events; in age- and sex-adjusted models they did not differ in AUC (septal: 0.8385, lateral: 0.8389; p = 0.94) or in continuous NRI (p = 0.84). Models using E/e'average did not improve AUC or NRI, and the intra-individual difference between sites had...... no predictive value (p = 0.79). E/e'septal was generally higher than E/e'lateral, thus age- and sex-specific normal values were reported for both sites for a population free of cardiac events during 10 years of follow-up. CONCLUSIONS: Septal and lateral E/e' are equally useful in predicting cardiac events...

  5. Left septal atrial tachycardia after open-heart surgery: relevance to surgical approach, anatomical and electrophysiological characteristics associated with catheter ablation, and procedural outcomes.

    Science.gov (United States)

    Adachi, Toru; Yoshida, Kentaro; Takeyasu, Noriyuki; Masuda, Keita; Sekiguchi, Yukio; Sato, Akira; Tada, Hiroshi; Nogami, Akihiko; Aonuma, Kazutaka

    2015-02-01

    Septal atrial tachycardia (AT) can occur in patients without structural heart disease and in patients with previous catheter ablation of atrial fibrillation. We aimed to assess septal AT that occurs after open-heart surgery. This study comprised 20 consecutive patients undergoing catheter ablation of macroreentrant AT after open-heart surgery. Relevance to surgical approach, mechanisms, anatomic and electrophysiological characteristics, and outcomes were assessed. Septal AT was identified in 7 patients who had all undergone mitral valve surgery. All septal ATs were localized in the left atrial septum, whereas 10 of 13 nonseptal ATs originated from the right atrium. Patients with left septal AT had a thicker fossa ovalis (median, 4.0; 25th-75th percentile, 3.6-4.2 versus 2.3; 1.6-2.6 mm; P=0.006) and broader area of low voltage (open-heart surgery was characterized by a thicker septum, more scar burden in the septum, and repeated prolongations of the tachycardia cycle length during ablation. Such an arrhythmogenic substrate may interfere with transmural lesion formation by ablation and may account for higher likelihood of recurrence of left septal AT. © 2014 American Heart Association, Inc.

  6. Effectiveness of caudal septal extension graft application in endonasal septoplasty

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    Yunus Karadavut

    Full Text Available Abstract Introduction Septal deviation is a common disease seen in daily otorhinolaryngology practice and septoplasty is a commonly performed surgical procedure. Caudal septum deviation is also a challenging pathology for ear, nose, and throat specialists. Many techniques are defined for caudal septal deviation. Objective To evaluate the effectiveness of caudal septal extension graft (CSEG application in patients who underwent endonasal septoplasty for a short and deviated nasal septum. Methods Forty patients with nasal septal deviation, short nasal septum, and weak nasal tip support who underwent endonasal septoplasty with or without CSEG placement between August 2012 and June 2013 were enrolled in this study. Twenty patients underwent endonasal septoplasty with CSEG placement. The rest of the group, who rejected auricular or costal cartilage harvest for CSEG placement, underwent only endonasal septoplasty without any additional intervention. Using the Nasal Obstruction Symptom Evaluation (NOSE and Rhinoplasty Outcome Evaluation (ROE questionnaires, pre- and post-operative acoustic rhinometer measurements were evaluated to assess the effect of CESG placement on nasal obstruction. Results In the control group, preoperative and postoperative minimal cross-sectional areas (MCA1 were 0.44 ± 0.10 cm2 and 0.60 ± 0.11 cm2, respectively (p < 0.001. In the study group, pre- and postoperative MCA1 values were 0.45 ± 0.16 cm2 and 0.67 ± 0.16 cm2, respectively (p < 0.01. In the control group, the nasal cavity volume (VOL1 value was 1.71 ± 0.21 mL preoperatively and 1.94 ± 0.17 mL postoperatively (p < 0.001. In the study group, pre- and postoperative VOL1s were 1.72 ± 0.15 mL and 1.97 ± 0.12 mL, respectively (p < 0.001. Statistical analysis of postoperative MCA1 and VOL1 values in the study and the control groups could not detect any significant intergroup difference (p = 0.093 and 0.432, respectively. In the study group, mean nasolabial angles were

  7. Classificação anatômica e correção cirúrgica da atresia pulmonar com comunicação interventricular Anatomical classification and surgical repair of the pulmonary atresia with ventricular septal defect

    Directory of Open Access Journals (Sweden)

    Ulisses Alexandre CROTI

    2001-12-01

    Full Text Available OBJETIVO: Analisar as características anatômicas, o resultado das técnicas empregadas na correção cirúrgica de acordo com o número de procedimentos, assim como a mortalidade em cada grupo da classificação de Barbero-Marcial para atresia pulmonar com comunicação interventricular. CASUÍSTICA E MÉTODOS: De janeiro de 1990 a novembro de 1999, 73 pacientes que foram submetidos a estudo cineangiocardiográfico previamente à primeira intervenção cirúrgica, foram analisados. As características anatômicas das artérias pulmonares e artérias colaterais sistêmico-pulmonares, assim como as técnicas cirúrgicas que propiciaram tratamento paliativo, "paliativo definitivo" e definitivo foram estudadas. As causas de mortalidade também foram descritas. RESULTADOS: Dezenove pacientes apresentavam os segmentos pulmonares supridos por artérias pulmonares (grupo A, 45 por artérias pulmonares e artérias colaterais sistêmico-pulmonares (grupo B e 9 somente por artérias colaterais sistêmico-pulmonares (grupo C. O grupo A apresentou maior proporção de tratamentos definitivos, o grupo B maior proporção de paliativos e o grupo C, maior proporção de "paliativos definitivos" (pOBJECTIVE: To analyze the morphological aspects, the surgical results obtained according to the number of procedures, and the mortality in each group of Barbero-Marcial´s classification of the pulmonary atresia with ventricular septal defect. MATERIAL E METHODS: From January 1990 to November 1999, 73 patients submitted to cardiac catheterization and detailed pulmonary angiographic study before the first surgical intervention were analyzed. The anatomical characteristics of the pulmonary arteries and major aorticopulmonary collaterals, as the surgical techniques of definitive, palliative and "definitive palliative" were studied. The causes of mortality were also described. RESULTS: Nineteen patients had all the pulmonary segments supplied by pulmonary arteries (group

  8. Congenital heart defects in oculodentodigital dysplasia: Report of two cases.

    Science.gov (United States)

    Izumi, Kosuke; Lippa, Andrew M; Wilkens, Alisha; Feret, Holly A; McDonald-McGinn, Donna M; Zackai, Elaine H

    2013-12-01

    Oculodentodigital dysplasia is caused by mutations in the GJA1 gene. Oculodentodigital dysplasia presents with a spectrum of clinical features including craniofacial, ocular, dental, and limb anomalies. Although recent findings implicate the major role of GJA1 during cardiac organogenesis, congenital heart defects are infrequently reported in oculodentodigital dysplasia. Here we report on two patients with GJA1 mutations presenting with cardiac malformations and type III syndactyly. Patient 1 presented with pulmonary atresia, an intact septum, right ventricular hypoplasia and tricuspid stenosis. The infant had a small nose, thin columella and bilateral 4-5 syndactyly of the fingers. A de novo c.226C>T (p.Arg76Cys) mutation was identified. Patient 2 presented at 6 months with a ventricular septal defect. The child had hypoplastic alae nasi with a thin columella and bilateral 4-5 syndactyly of the digits. A de novo missense mutation, c.145C>G (p.Gln49Glu) was found. Our two patients underscore the importance of cardiac evaluations as part of the initial workup for patients with findings of oculodentodigital dysplasia. Conversely, those patients with type III syndactyly and congenital heart defect should be screened for GJA1 mutations. © 2013 Wiley Periodicals, Inc.

  9. Management of tricuspid regurgitation in congenital heart disease: is survival better with valve repair?

    Science.gov (United States)

    Said, Sameh M; Dearani, Joseph A; Burkhart, Harold M; Connolly, Heidi M; Eidem, Ben; Stensrud, Paul E; Schaff, Hartzell V

    2014-01-01

    Tricuspid valve (TV) regurgitation in congenital heart disease includes a heterogeneous group of lesions, and few series have documented the outcomes. We reviewed the records of 553 patients with congenital heart disease who had undergone TV surgery for tricuspid regurgitation from January 1993 to December 2010. Patients with Ebstein malformation were excluded. Their mean age was 32 ± 21 years, and 300 were female (54%). The most common diagnoses were conotruncal anomaly in 216 patients (39%), previous ventricular septal defect closure in 83 (15%), atrioventricular septal defect in 77 (14%), and pulmonary atresia with an intact ventricular septum in 11 (2%). Preoperative right-sided heart failure was present in 124 patients (22%), and 55 patients (10%) had pulmonary hypertension. TV repair was performed in 442 (80%) and TV replacement in 111 (20%) patients. Repeat sternotomy was performed in 415 patients (75%). Previous TV repair was present in 44 patients (8%); of these, 17 (38.6%) underwent repeat TV repair. The overall early mortality was 3.1% (17 patients) and was 2.5% for TV repair and 5.4% for TV replacement (P = .001). The mean follow-up period was 4.5 ± 4.1 years (maximum, 18). The overall survival at 1, 5, and 10 years was 97%, 93%, and 85%, respectively. Survival was better for patients with repair than with replacement. TV repair was an independent predictor of better survival (P = .001). Important tricuspid regurgitation can occur with a variety of congenital diagnoses. Early mortality is low and late survival is superior with tricuspid repair than with valve replacement. Surgical treatment of tricuspid regurgitation in congenital heart disease should be performed before the onset of heart failure. Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  10. Yield Strength Testing in Human Cadaver Nasal Septal Cartilage and L-Strut Constructs.

    Science.gov (United States)

    Liu, Yuan F; Messinger, Kelton; Inman, Jared C

    2017-01-01

    To our knowledge, yield strength testing in human nasal septal cartilage has not been reported to date. An understanding of the basic mechanics of the nasal septum may help surgeons decide how much of an L-strut to preserve and how much grafting is needed. To determine the factors correlated with yield strength of the cartilaginous nasal septum and to explore the association between L-strut width and thickness in determining yield strength. In an anatomy laboratory, yield strength of rectangular pieces of fresh cadaver nasal septal cartilage was measured, and regression was performed to identify the factors correlated with yield strength. To measure yield strength in L-shaped models, 4 bonded paper L-struts models were constructed for every possible combination of the width and thickness, for a total of 240 models. Mathematical modeling using the resultant data with trend lines and surface fitting was performed to quantify the associations among L-strut width, thickness, and yield strength. The study dates were November 1, 2015, to April 1, 2016. The factors correlated with nasal cartilage yield strength and the associations among L-strut width, thickness, and yield strength in L-shaped models. Among 95 cartilage pieces from 12 human cadavers (mean [SD] age, 67.7 [12.6] years) and 240 constructed L-strut models, L-strut thickness was the only factor correlated with nasal septal cartilage yield strength (coefficient for thickness, 5.54; 95% CI, 4.08-7.00; P cadaver nasal septal cartilage, L-strut thickness was significantly associated with yield strength. In a bonded paper L-strut model, L-strut thickness had a more important role in determining yield strength than L-strut width. Surgeons should consider the thickness of potential L-struts when determining the amount of cartilaginous septum to harvest and graft. NA.

  11. Cardiac surgery in Africa: a thirty-five year experience on open heart surgery in Cote d'Ivoire.

    Science.gov (United States)

    Yangni-Angate, Koffi Herve; Meneas, Christophe; Diby, Florent; Diomande, Manga; Adoubi, Anicet; Tanauh, Yves

    2016-10-01

    Few centers for open heart surgery (OHS) are in Sub-Saharan Africa. Lack of OHS results is also noted. By reporting our African experience on OHS, the aim of this study was to fill the gap. It is a retrospective study on 2,612 patients who were subject to an OHS between 1978 and 2013. Data were collected from demographical, clinical, investigative studies, surgical and outcomes parameters. There were 1,475 cases of rheumatic heart diseases (RHD), 126 endomyocardial fibrosis (EMF), 741 congenital heart diseases (CHDs) and 270 various affections. Related to rheumatic valvular surgery we enumerated 1,175 monovalvular (mitral n=778, aortic n=336, tricuspid n=61); 280 bivalvular (mitral + aortic n=150, mitral + tricuspid n=130) and 20 trivalvular. For RHD, average age was 26±10.1 years (4-69 years) and 60% of our patients presented a functional class III or IV according to New York Heart Association (NYHA) classification. A total of 1,481 valvular replacements (bioprostheses n=489, mechanical prostheses n=992) and 445 valvular repair were carried out with a global and late mortality surgery respectively at 7% and 8%. One hundred and twenty-six [126] cases of EMF with right sided form 39, left sided form 40, and bilateral form 47 were colligated. Average age was 12±0.6 years (2-15 years). All patients with EMF underwent surgery; an endocardectomy in all patients combined with valvular reconstruction (n=36) or valvular replacement (n=90) was carried out with a hospital mortality at 16% (n=20). Concerning CHD, the most frequent were ventricular septal defect (VSD) (n=240), atrial septal defect (ASD) (n=200), partial atrio-ventricular sepal defect (n=30) and tetralogy of Fallot (T4F) (n=220), a total correction was performed for those CHD with an early mortality at 6.4% (n=44). OHS in Cote d'Ivoire was successfully performed in most of our patients, the spectrum of acquired valvular heart diseases and CHDs in our country is similar to others in Sub-Saharan Africa.

  12. Anatomy of right superior septal artery demonstrated on the coronary CT scan

    International Nuclear Information System (INIS)

    Takeguchi, Takaya; Ibukuro, Kenji; Fukuda, Hozumi; Tobe, Kimiko; Abe, Shoko

    2012-01-01

    Background. A coronary CT scan allows for non-invasive visualization of the anatomy of a coronary artery in three dimensions compared to the two dimensions afforded by conventional angiography. The septal artery, the main blood source of the interventricular septum, is usually derived from the left anterior descending artery; however, it is occasionally derived from the right coronary artery. Purpose. To analyze the prevalence, origin, diameter, and length of the right superior septal artery (RSSA) demonstrated on a coronary CT scan. Material and Methods. The right superior septal artery was retrospectively reviewed on the reconstructed axial scan images (0.5-mm thickness, 0.25-mm interval) in 1290 consecutive patients who underwent coronary CT scans. All patients were scanned on a 320-row CT scanner. The images were transferred to a workstation to trace the vessel to analyze the origin, diameter, and length. We also compared the length of the RSSA between patients with and without coronary artery stenosis. Results. The RSSA was identified in 51 (3.9%) of 1290 patients. The origin was the proximal portion of the right coronary artery (n = 40) or the right sinus of Valsalva (n 11). The artery co-existed with the conus artery in 15 (29%) of 51 patients. The length was 16-62 mm (mean 31.2 mm ± 10.5), and the diameter was 0.8-2.0 mm (mean 1.3 mm ± 0.2). Longer RSSAs tended to be demonstrated in the patients with coronary artery stenosis rather than with normal coronary arteries (P < 0.05). Conclusion. The right superior septal artery and its anatomical variant could be analyzed with a coronary CT scan. The ability to demonstrate this artery on the coronary CT scan was the same as with coronary angiography. The recognition of this vessel is useful for physicians managing with the diagnosis and treatment of the coronary artery disease

  13. Complete Atrioventricular Block Complicating Mitral Infective Endocarditis Caused by Streptococcus Agalactiae.

    Science.gov (United States)

    Arai, Masaru; Nagashima, Koichi; Kato, Mahoto; Akutsu, Naotaka; Hayase, Misa; Ogura, Kanako; Iwasawa, Yukino; Aizawa, Yoshihiro; Saito, Yuki; Okumura, Yasuo; Nishimaki, Haruna; Masuda, Shinobu; Hirayama, Astushi

    2016-09-08

    BACKGROUND Infective endocarditis (IE) involving the mitral valve can but rarely lead to complete atrioventricular block (CAVB). CASE REPORT A 74-year-old man with a history of infective endocarditis caused by Streptococcus gordonii (S. gordonii) presented to our emergency room with fever and loss of appetite, which had lasted for 5 days. On admission, results of serologic tests pointed to severe infection. Electrocardiography showed normal sinus rhythm with first-degree atrioventricular block and incomplete right bundle branch block, and transthoracic echocardiography and transesophageal echocardiography revealed severe mitral regurgitation caused by posterior leaflet perforation and 2 vegetations (5 mm and 6 mm) on the tricuspid valve. The patient was initially treated with ceftriaxone and gentamycin because blood and cutaneous ulcer cultures yielded S. agalactiae. On hospital day 2, however, sudden CAVB requiring transvenous pacing occurred, and the patient's heart failure and infection worsened. Although an emergent surgery is strongly recommended, even in patients with uncontrolled heart failure or infection, surgery was not performed because of the Child-Pugh class B liver cirrhosis. Despite intensive therapy, the patient's condition further deteriorated, and he died on hospital day 16. On postmortem examination, a 2×1-cm vegetation was seen on the perforated posterior mitral leaflet, and the infection had extended to the interventricular septum. Histologic examination revealed extensive necrosis of the AV node. CONCLUSIONS This rare case of CAVB resulting from S. agalactiae IE points to the fact that in monitoring patients with IE involving the mitral valve, clinicians should be aware of the potential for perivalvular extension of the infection, which can lead to fatal heart block.

  14. Effect of right ventricular pacing lead site on left ventricular function in patients with high-grade atrioventricular block: results of the Protect-Pace study.

    Science.gov (United States)

    Kaye, Gerald C; Linker, Nicholas J; Marwick, Thomas H; Pollock, Lucy; Graham, Laura; Pouliot, Erika; Poloniecki, Jan; Gammage, Michael

    2015-04-07

    Chronic right ventricle (RV) apical (RVA) pacing is standard treatment for an atrioventricular (AV) block but may be deleterious to left ventricle (LV) systolic function. Previous clinical studies of non-apical pacing have produced conflicting results. The aim of this randomized, prospective, international, multicentre trial was to compare change in LV ejection fraction (LVEF) between right ventricular apical and high septal (RVHS) pacing over a 2-year study period. We randomized 240 patients (age 74 ± 11 years, 67% male) with a high-grade AV block requiring >90% ventricular pacing and preserved baseline LVEF >50%, to receive pacing at the RVA (n = 120) or RVHS (n = 120). At 2 years, LVEF decreased in both the RVA (57 ± 9 to 55 ± 9%, P = 0.047) and the RVHS groups (56 ± 10 to 54 ± 10%, P = 0.0003). However, there was no significant difference in intra-patient change in LVEF between confirmed RVA (n = 85) and RVHS (n = 83) lead position (P = 0.43). There were no significant differences in heart failure hospitalization, mortality, the burden of atrial fibrillation, or plasma brain natriutetic peptide levels between the two groups. A significantly greater time was required to place the lead in the RVHS position (70 ± 25 vs. 56 ± 24 min, P function requiring a high percentage of ventricular pacing, RVHS pacing does not provide a protective effect on left ventricular function over RVA pacing in the first 2 years. ClinicalTrials.gov number NCT00461734. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.

  15. [Comparison of ablation of left-sided accessory pathway by atrial septal and retrograde arterial approach].

    Science.gov (United States)

    Zhu, J G; Bao, Z Y; Gu, X

    2017-03-07

    Objective: To compare the advantages and disadvantages of radiofrequency ablation of left-sided accessory pathways by via atrial septal approach with retrograde through aortic approach. Methods: A total of 184 patients of left-side accessory pathways were treated in Taizhou People's Hospital and the Subei People's Hospital from March 2012 to August 2015.A total of 103 cases were treated by aortic retrograde approach as through arterial group, 81 cases were treated by punctured atrial septal to left atrial for mapping and ablation as through atrial septal group.Comparison of ablation procedure time, total and pathways of different parts(subgroup) at instant success and relapse rates, safety (serious complications), and statistics other complications in operation and postoperative. Results: Through arterial group and through atrial septal group were no significant difference ( P >0.05) in the ablation procedure time((25±18 ) vs (22±15)min ), instant success(98.1% vs 97.5%) and relapse rates(1.0% vs 1.2%), security(1 vs 0 case). There was no statistical difference in septal part subgroups (all P >0.05) in the ablation procedure time((22±18)vs (25±19)min), instant success(91.7% vs 89.9 %) and relapse rates(0 vs 11.1%); posterior wall subgroup had no statistical difference in the ablation procedure time((18±15)vs (16±12)min), instant success(100% vs 100 %) and relapse rates(0 vs 0)(all P >0.05); side wall subgroup had no statistical difference in the ablation procedure time((29±20)vs (21±18) min), instant success (98.3% vs 98.1%)and relapse rates(1.7% vs 0%)(all P >0.05). Conclusion: Ablation of left-sided accessory pathways by transseptal approach and transaortic approach has no statistical difference in the procedure time, instant success and relapse rates, security.In a particular case, there is a certain complementarity between the two methods.

  16. Lutembacher's syndrome: A rare combination of congenital and ...

    African Journals Online (AJOL)

    Because the mitral stenosis was, in fact, rheumatic in aetiology, the syndrome was defined eventually as a combination of congenital atrial septal defect and acquired, almost always rheumatic, mitral stenosis. Keywords:Lutembacher's syndrome, congenital heart disease, valvular heart disease, atrial septal defect, mitral ...

  17. Lateral septal vasopressin in rats : Role in social and object recognition?

    NARCIS (Netherlands)

    Everts, H.G J; Koolhaas, J.M.

    1997-01-01

    The capacity of male rats to remember familiar conspecifics is called social recognition. It is a form of short-term memory modulated by lateral septal (LS) vasopressin (VP). The specificity of this phenomenon was studied by examining whether recognition of previously investigated objects is also

  18. Co-occurrence of hypertrophic cardiomyopathy and myeloproliferative disorder in a neonate with Noonan syndrome carrying Thr73Ile mutation in PTPN11.

    Science.gov (United States)

    Yagasaki, Hideaki; Nakane, Takaya; Hasebe, Youhei; Watanabe, Atsushi; Kise, Hiroaki; Toda, Takako; Koizumi, Keiichi; Hoshiai, Minako; Sugita, Kanji

    2015-12-01

    Most cases of Noonan syndrome (NS) result from mutations in one of the RAS-MAPK signaling genes, including PTPN11, SOS1, KRAS, NRAS, RAF1, BRAF, SHOC2, MEK1 (MAP2K1), and CBL. Cardiovascular diseases of varying severity, such as pulmonary stenosis and hypertrophic cardiomyopathy (HCM), are common in NS patients. RAF1 mutations are most frequent in NS with HCM, while PTPN11 mutations are also well known. Thr73Ile is a gain-of-function mutation of PTPN11, which has been highly associated with juvenile myelomonocytic leukemia and NS/myeloproliferative disease (MPD), but has not previously been reported in HCM. Here, we report a Japanese female infant with NS carrying the PTPN11 T73I mutation with NS/MPD, complete atrio-ventricular septal defect, and rapidly progressive HCM. No other HCM-related mutations were detected in PTPN11, RAF1, KRAS, BRAF, and SHOC2. This patient provides additional information regarding the genotype-phenotype correlation for PTPN11 T73I mutation in NS. © 2015 Wiley Periodicals, Inc.

  19. lutembacher's syndrome: a rare combination of congenital and ...

    African Journals Online (AJOL)

    FOBUR

    Lutembacher's syndrome is defined as the rare combination of congenital atrial septal defect and acquired ... atrial septal defect. The definition of Lutembacher's syndrome has undergone many changes. The earliest description in medical literature was found in a letter written ... atrial fibrillation with her chest x-ray showing.

  20. Defeito do septo atrioventricular forma total associado a tricuspidização da valva atrioventricular esquerda na infância

    Directory of Open Access Journals (Sweden)

    Aleksander DOBRIANSKYJ

    1997-10-01

    Full Text Available Entre maio de 1987 e dezembro de 1996, o reparo do defeito do septo atrioventricular, forma total (DSAVT, com duplo retalho de pericárdio bovino e tricuspidização da valva atrioventricular (VAV esquerda, foi realizado em 34 pacientes consecutivos com mortalidade global de 5 (14,7% pacientes (pac.. Grupo I: abaixo de 6 meses (m com 12 pac. (1 óbito; 8,3%; Grupo II: entre 7m e 12m, com 15 pac. (4 óbitos; 26,6% e Grupo III: entre 1 e 5 anos (a com 7 pac. sem óbitos. Ocorreram 2 óbitos tardios (1 por Insuficiência mitral residual + insuficiência cardíaca congestiva e outro por pneumonia + insuficiência respiratória. Duas crianças receberam implante de marcapasso definitivo (após 45 dias e 4 anos. Nenhum paciente foi reoperado, apesar de mais 2 apresentarem insuficiência da VAV esquerda e 1 VAV direita, controlada clinicamente, e as restantes encontram-se nos graus I e II da New York Heart Association (NYHA. Apesar de 29 pacientes apresentarem peso abaixo de 10 kg, utilizou-se circulação extracorpórea (CEC e hipotermia a 25°C sem parada circulatória total. Iniciou-se o reparo pela zona de "aposição" da VAV única com um ou dois pontos em "x", mantendo tricúspide. Sutura-se o primeiro retalho de pericárdio bovino (PB em forma de "gota", insinuando-o entre as cordas do lado direito do septo; a seguir, sutura-se o segundo retalho, fazendo uma aposição concomitante com a VAV única, septando-se, desta forma, as quatro cavidades. As vantagens desta técnica são: preservação da integridade valvar, maior durabilidade funcional da VAV esquerda, menor possibilidade de reoperação mesmo em pacientes mais jovens e que devem ser preferencialmente operados em torno de 6º mês de vida.Between May 1987 and December 1996, repair of the "Ostium Atrioventricularis Communis" (OAVC, with total shaping by means of a double bovine pericardial patch and tricuspidation of the left atrioventricular valve (AVV was performed on 34 consecutive

  1. Decellularization of Human Nasal Septal Cartilage for the Novel Filler Material of Vocal Fold Augmentation.

    Science.gov (United States)

    Kang, Dae-Woon; Shin, Sung-Chan; Jang, Jeon-Yeob; Park, Hee-Young; Lee, Jin-Choon; Wang, Soo-Geun; Lee, Byung-Joo

    2017-01-01

    The clinical application of allogenic and/or xenogenic cartilage for vocal fold augmentation requires to remove the antigenic cellular component. The objective of this study was to assess the effect of cartilage decellularization and determine the change in immunogenicity after detergent treatment in human nasal septal cartilage flakes made by the freezing and grinding method. Human nasal septal cartilages were obtained from surgical cases. The harvested cartilages were treated by the freezing and grinding technique. The obtained cartilage flakes were treated with 1% Triton X-100 or 2% sodium dodecyl sulfate (SDS) for decellularization of the cartilage flakes. Hematoxylin and eosin stain (H&E stain), surface electric microscopy, immunohistochemical stain for major histocompatibility complex I and II, and ELISA for DNA contents were performed to assess the effect of cartilage decellularization after detergent treatment. A total of 10 nasal septal cartilages were obtained from surgical cases. After detergent treatment, the average size of the cartilage flakes was significantly decreased. With H&E staining, the cell nuclei of decellularized cartilage flakes were not observed. The expression of major histocompatibility complex (MHC)-I and II antigens was not identified in the decellularized cartilage flakes after treatment with detergent. DNA content was removed almost entirely from the decellularized cartilage flakes. Treatment with 2% SDS or 1% Triton X-100 for 1 hour appears to be a promising method for decellularization of human nasal septal cartilage for vocal fold augmentation. Copyright © 2017 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

  2. Case report of complete atrioventricular block following therapeutic irradiation for breast cancer

    Energy Technology Data Exchange (ETDEWEB)

    Nishizawa, Tadashi; Suzuki, Ichiro; Kobayashi, Jun; Terashima, Masafumi

    1987-12-01

    A 49-year-old woman developed complete atrioventricular (A-V) block 16 years after radiotherapy for breast cancer. The patient had received 60 Gy of cobalt 60 irradiation for left breast cancer arising 3 years after contracting right breast cancer. It took one year before complete A-V block was fixed, during which two-degree A-V block, atrial fibrillation, and sinus rhythm were predominant. She had myocardial insertion of pacemaker electrode. Fibrosis and hyaloid degeneration of the pericardium and thickened sclerosis of the epicardium seemed to be radiation-induced. There was no evidence of recurrence of breast cancer. (Namekawa, K.).

  3. Association between septal deviation and sinonasal papilloma.

    Science.gov (United States)

    Nomura, Kazuhiro; Ogawa, Takenori; Sugawara, Mitsuru; Honkura, Yohei; Oshima, Hidetoshi; Arakawa, Kazuya; Oshima, Takeshi; Katori, Yukio

    2013-12-01

    Sinonasal papilloma is a common benign epithelial tumor of the sinonasal tract and accounts for 0.5% to 4% of all nasal tumors. The etiology of sinonasal papilloma remains unclear, although human papilloma virus has been proposed as a major risk factor. Other etiological factors, such as anatomical variations of the nasal cavity, may be related to the pathogenesis of sinonasal papilloma, because deviated nasal septum is seen in patients with chronic rhinosinusitis. We, therefore, investigated the involvement of deviated nasal septum in the development of sinonasal papilloma. Preoperative computed tomography or magnetic resonance imaging findings of 83 patients with sinonasal papilloma were evaluated retrospectively. The side of papilloma and the direction of septal deviation showed a significant correlation. Septum deviated to the intact side in 51 of 83 patients (61.4%) and to the affected side in 18 of 83 patients (21.7%). Straight or S-shaped septum was observed in 14 of 83 patients (16.9%). Even after excluding 27 patients who underwent revision surgery and 15 patients in whom the papilloma touched the concave portion of the nasal septum, the concave side of septal deviation was associated with the development of sinonasal papilloma (p = 0.040). The high incidence of sinonasal papilloma in the concave side may reflect the consequences of the traumatic effects caused by wall shear stress of the high-velocity airflow and the increased chance of inhaling viruses and pollutants. The present study supports the causative role of human papilloma virus and toxic chemicals in the occurrence of sinonasal papilloma.

  4. Atrial Septal Aneurysm and Patent Foramen Ovale as Risk Factors for Cryptogenic Stroke in Patients Less Than 55 Years of Age: A Study using Transesophageal Echocardiography

    Science.gov (United States)

    Cabanes, L.; Mas, J. L.; Cohen, A.; Amarenco, P.; Cabanes, P. A.; Oubary, P.; Chedru, F.; Guerin, F.; Bousser, M. G.; deRecondo, J.

    1993-01-01

    Background and Purpose: An association between atrial septal aneurysm and embolic events has been suggested. Atrial septal aneurysm has been shown to be associated with patent foramen ovale and,.in some reports, with mitral valve prolapse. These two latter cardiac disorder; have been identified as potential risk factors for ischemic stroke. The aim of this prospective study was to assess the role of atrial septal aneurysm as an independent risk factor for stroke, especially for cryptogenic stroke. Methods: We studied the prevalence of atrial septal aneurysm, patent foramen ovale, and mitral valve prolapse in 100 consecutive patients ischemic stroke who underwent extensive etiological investigations. We compared these results with those in a control group of 50 consecutive patients. The diagnosis of atrial septal aneurysm and patent foramen ovale relied on transesophageal echocardiography with a contrast study and that of mitral valve prolapse, on two-dimensional transthoracic echocardiography. Results: Stepwise logistic regression analysis showed that atrial septal aneurysm (odds ratio, 4.3; 95% confidence interval, 1.3 to 14.6; P=.01) and patent foramen ovale (odds ratio, 3.9; 95% confidence interval, 1.5 to 10; P=.003) but not mitral valve prolapse were significantly associated with the diagnosis of cryptogenic stroke. The stroke odds of a patient with both atrial septal aneurysm and patent foramen ovale were 33.3 times (95% confidence interval, 4.1 to 270) the stroke odds of a patient with neither of these cardiac disorders. For a patient with atrial septal aneurysm of >lo-mm excursion, the stroke odds were approximately 8 times the stroke odds of a patient with atrial septal aneurysm of stroke and that their association has a marked synergistic effect. Atrial septal aneurysms of >lo-mm excursion are associated with a higher risk of stroke. (Stroke. 1993;24:1865-1873.) KEY WORDS aneurysm echocardiography foramen ovale, patent mitral valve prolapse o young adults

  5. Ablation of hypertrophic septum using radiofrequency energy: an alternative for gradient reduction in patient with hypertrophic obstructive cardiomyopathy?

    Science.gov (United States)

    Riedlbauchová, Lucie; Janoušek, Jan; Veselka, Josef

    2013-06-01

    Alcohol septal ablation and surgical myectomy represent accepted therapeutic options for treatment of symptomatic patients with hypertrophic obstructive cardiomyopathy. Long-term experience with radiofrequency ablation of arrhythmogenic substrates raised a question if this technique might be effective for left ventricular outflow tract (LVOT) gradient reduction. We report on a 63-year-old patient with recurrence of symptoms 1 year after alcohol septal ablation (ASA) leading originally to a significant reduction of both symptoms and gradient. Due to a new increase of gradient in the LVOT up to 200 mm Hg with corresponding worsening of symptoms and due to refusal of surgical myectomy by the patient, endocardial radiofrequency ablation of the septal hypertrophy (ERASH) was indicated. Radiofrequency ablation was performed in the LVOT using irrigated-tip ablation catheter; the target site was identified using intracardiac echocardiography and electroanatomical CARTO mapping. ERASH caused an immediate gradient reduction due to hypokinesis of the ablated septum. At 2-month follow-up exam, significant clinical improvement was observed, together with persistent gradient reduction assessed with Doppler echocardiography. Echocardiography and magnetic resonance revealed persistent septal hypokinesis and slight thinning of the ablated region. Septal ablation using radiofrequency energy may be a promising alternative or adjunct to the treatment of hypertrophic obstructive cardiomyopathy. Intracardiac echocardiography and electroanatomical CARTO mapping enable exact lesion placement and preservation of atrioventricular conduction.

  6. The septal bulge--an early echocardiographic sign in hypertensive heart disease.

    Science.gov (United States)

    Gaudron, Philipp Daniel; Liu, Dan; Scholz, Friederike; Hu, Kai; Florescu, Christiane; Herrmann, Sebastian; Bijnens, Bart; Ertl, Georg; Störk, Stefan; Weidemann, Frank

    2016-01-01

    Patients in the early stage of hypertensive heart disease tend to have normal echocardiographic findings. The aim of this study was to investigate whether pathology-specific echocardiographic morphologic and functional parameters can help to detect subclinical hypertensive heart disease. One hundred ten consecutive patients without a history and medication for arterial hypertension (AH) or other cardiac diseases were enrolled. Standard echocardiography and two-dimensional speckle-tracking-imaging analysis were performed. Resting blood pressure (BP) measurement, cycle ergometer test (CET), and 24-hour ambulatory BP monitoring (ABPM) were conducted. Patients were referred to "septal bulge (SB)" group (basal-septal wall thickness ≥ 2 mm thicker than mid-septal wall thickness) or "no-SB" group. Echocardiographic SB was found in 48 (43.6%) of 110 patients. In this SB group, 38 (79.2%) patients showed AH either by CET or ABPM. In contrast, in the no-SB group (n = 62), 59 (95.2%) patients had no positive test for AH by CET or ABPM. When AH was solely defined by resting BP, SB was a reasonable predictive sign for AH (sensitivity 73%, specificity 76%). However, when AH was confirmed by CET or ABPM the echocardiographic SB strongly predicted clinical AH (sensitivity 93%, specificity 86%). In addition, regional myocardial deformation of the basal-septum in SB group was significantly lower than in no-SB group (14 ± 4% vs. 17 ± 4%; P heart disease. Sophisticated BP evaluation including resting BP, ABPM, and CET should be performed in all patients with an accidental finding of a SB in echocardiography. Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

  7. Morphologic-echocardiographic correlates of Ebstein's malformation.

    Science.gov (United States)

    Rusconi, P G; Zuberbuhler, J R; Anderson, R H; Rigby, M L

    1991-07-01

    The cross-sectional echocardiographic findings were analysed retrospectively in 26 patients with Ebstein's malformation in the light of studies of autopsied specimens from different patients showing this lesion. The salient anatomical feature in diagnosis is the finding of the hinge point of the septal and mural leaflets of the valve within the inlet component of the right ventricle rather than at the atrioventricular junction. The other important feature is the nature of the distal attachment of the leaflets, particularly the anterosuperior one, which can either be in focal or linear fashion. The hinge point of the septal leaflet was noted echocardiographically to be displaced in 19 patients but, significantly, the leaflet was absent in the other seven. Also significant was that the hinge point of the mural leaflet at the crux had been visualized in only 15 of the patients. The anterosuperior leaflet had a distal linear attachment in 20 of the patients, with the anteroseptal commissure becoming a keyhole in six of these through which blood passed to the functional right ventricle. The valve remained a competent structure, even though closing at the junction of atrialized and functional components of the right ventricle rather than at the atrioventricular junction. Cross-sectional echocardiography is the technique of choice with which to display the salient morphological features of Ebstein's malformation.

  8. Ventricular Septal Defect (For Teens)

    Science.gov (United States)

    ... of the heart and surrounding organs an electrocardiogram (EKG) , which records the electrical activity of the heart ... couch doing quiet activities like watching TV or reading. Everyone heals differently. Your doctor will let you ...

  9. Atrial Septal Defect (For Teens)

    Science.gov (United States)

    ... of the heart and surrounding organs an electrocardiogram (EKG), which records the electrical activity of the heart ... on the couch doing quiet activities such as reading, sleeping, and watching TV. Within a week or ...

  10. Facts about Ventricular Septal Defect

    Science.gov (United States)

    ... of the Aorta D-Transposition of the Great Arteries Hypoplastic Left Heart Syndrome Pulmonary Atresia Tetralogy of ... Privacy FOIA No Fear Act OIG 1600 Clifton Road Atlanta , GA 30329-4027 USA 800-CDC-INFO ( ...

  11. Facts about Atrial Septal Defect

    Science.gov (United States)

    ... of the Aorta D-Transposition of the Great Arteries Hypoplastic Left Heart Syndrome Pulmonary Atresia Tetralogy of ... Privacy FOIA No Fear Act OIG 1600 Clifton Road Atlanta , GA 30329-4027 USA 800-CDC-INFO ( ...

  12. Septal membrane localization by C-terminal amphipathic α-helices of MinD in Bacillus subtilis mutant cells lacking MinJ or DivIVA.

    Science.gov (United States)

    Ishikawa, Kazuki; Matsuoka, Satoshi; Hara, Hiroshi; Matsumoto, Kouji

    2017-10-18

    The Min system, which inhibits assembly of the cytokinetic protein FtsZ, is largely responsible for positioning the division site in rod-shaped bacteria. It has been reported that MinJ, which bridges DivIVA and MinD, is targeted to the cell poles by an interaction with DivIVA, and that MinJ in turn recruits MinCD to the cell poles. MinC, however, is located primarily at active division sites at mid-cell when expressed from its native promoter. Surprisingly, we found that Bacillus subtilis MinD is located at nascent septal membranes and at an asymmetric site on lateral membranes between nascent septal membranes in filamentous cells lacking MinJ or DivIVA. Bacillus subtilis MinD has two amphipathic α-helices rich in basic amino acid residues at its C-terminus; one of these, named MTS1 here, is the counterpart of the membrane targeting sequence (MTS) in Escherichia coli MinD while the other, named MTS-like sequence (MTSL), is the nearest helix to MTS1. These amphipathic helices were located independently at nascent septal membranes in cells lacking MinJ or DivIVA, whereas elimination of the helices from the wild type protein reduced its localization considerably. MinD variants with altered MTS1 and MTSL, in which basic amino acid residues were replaced with proline or acidic residues, were not located at nascent septal membranes, indicating that the binding to the nascent septal membranes requires basic residues and a helical structure. The septal localization of MTSL, but not of MTS1, was dependent on host cell MinD. These results suggest that MinD is targeted to nascent septal membranes via its C-terminal amphipathic α-helices in B. subtilis cells lacking MinJ or DivIVA. Moreover, the diffuse distribution of MinD lacking both MTSs suggests that only a small fraction of MinD depends on MinJ for its localization to nascent septal membranes.

  13. The influence of deficient retro-aortic rim on technical success and early adverse events following device closure of secundum atrial septal defects: An Analysis of the IMPACT Registry®.

    Science.gov (United States)

    O'Byrne, Michael L; Gillespie, Matthew J; Kennedy, Kevin F; Dori, Yoav; Rome, Jonathan J; Glatz, Andrew C

    2017-01-01

    Concern regarding aortic erosion has focused attention on the retro-aortic rim in patients undergoing device closure of atrial septal defects (ASD), but its effect on early outcomes is not well studied. A multicenter retrospective cohort study of patients undergoing device occlusion of ASD between 1/2011-10/2014 was performed, using data from the IMproving Pediatric and Adult Congenital Treatment Registry. Subjects were divided between those with retro-aortic rim technical failure and major early adverse events. Case times were measured as surrogates of technical complexity. The effect of deficient retro-aortic rim on primary outcomes was assessed using hierarchical logistic regression, adjusting for other suspected covariates and assessing whether they represent independent risk factors RESULTS: 1,564 subjects (from 77 centers) were included, with deficient retro-aortic rim present in 40%. Technical failure occurred in 91 subjects (5.8%) and a major early adverse event in 64 subjects (4.1%). Adjusting for known covariates, the presence of a deficient retro-aortic rim was not significantly associated with technical failure (OR: 1.3, 95% CI: 0.9-2.1) or major early adverse event (OR: 0.7, 95% CI: 0.4-1. 2). Total case (P = 0.01) and fluoroscopy time (P = 0.02) were greater in subjects with deficient rim, but sheath time was not significantly different (P = 0.07). Additional covariates independently associated with these outcomes were identified. Deficient retro-aortic rim was highly prevalent but not associated with increased risk of technical failure or early adverse events. Studies with longer follow-up are necessary to assess other outcomes, including device erosion. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  14. [Prevalence of birth defects according to the level of care in two hospitals, Cali, Colombia, 2012-2013].

    Science.gov (United States)

    Pachajoa, Harry; Villota, Vania A; Cruz, Luz Marina; Ariza, Yoseth

    2015-01-01

    Birth defects are morphologic alterations diagnosed prenatal or postnatally. Surveillance systems have been used to estimate the prevalence in high complexity care centers; however, the variation of the prevalence among different complexity care centers remains unknown. To compare the prevalence of birth defects among two different complexity care centers in Cali, Colombia. A descriptive hospital-based study following the methodology of the Latin American Collaborative Study of Congenital Malformations was conducted during 20 months in a medium complexity hospital and a high complexity hospital. During the study period, 7,140 births were attended of which 225 had at least one birth defect. The prevalence of these was of 1.7% (IC95% 1.3-2.0) and 7.4% (IC95% 6.2-8.7) for the medium complexity hospital and the high complexity hospital, respectively. The highest frequencies for the high complexity care center were: ventricular septal defect, 10%; congenital hydronephrosis, 7%; abdominal wall defects, 6%, and hydrocephalus, 5%, while for the medium complexity were: polydactyly, 15%; preauricular skin tags, 8%; congenital talipes equino varus, 7%, and hemangioma, 6%. The prevalence of birth defects among different complexity care centers varies in quantity, type and severity of the anomaly diagnosed. The surveillance of birth defects is a useful tool for any level of care. It allows estimating more accurately the prevalence of the city, as well being a base for the planning and targeting of resources according to the prevalence of different congenital defects.

  15. Congenital Heart Diseases in Adults: A Review of Echocardiogram ...

    African Journals Online (AJOL)

    The most common congenital anomalies were ventricular septal defects (VSD) ‑ 31.3%, (36/115), atrial septal defects ‑ 28.7% (33/115) and tetralogy of fallot ‑ 10.4% (12/115). Conclusion: VSD are the most common congenital heart diseases in adults presenting for echocardiographic examination in Enugu, Nigeria.

  16. Noninvasive quantification of left-to-right shunt by phase contrast magnetic resonance imaging in secundum atrial septal defect: the effects of breath holding and comparison with invasive oximetry.

    Science.gov (United States)

    Yamasaki, Yuzo; Kawanami, Satoshi; Kamitani, Takeshi; Sagiyama, Koji; Sakamoto, Ichiro; Hiasa, Ken-Ichi; Yabuuchi, Hidetake; Nagao, Michinobu; Honda, Hiroshi

    2018-01-16

    To investigate the effect of breath-holding on left-to-right shunts in patients with a secundum atrial septal defect (ASD). Thirty-five consecutive patients with secundum ASDs underwent right heart catheterization and invasive oximetry. Phase-contrast magnetic resonance imaging (MRI) was performed for the main pulmonary artery and ascending aorta. All measurements were obtained during free breathing (FB) (quiet breathing; no breath-hold), expiratory breath-hold (EBH), and inspiratory breath-hold (IBH). Pulmonary circulation flow (Qp) and systemic circulation flow (Qs) were calculated by multiplying the heart rate by the stroke volume. Measurements during FB, EBH, and IBH were compared, and the differences compared to invasive oximetry were evaluated. There were significant differences among the measurements during FB, EBH, and IBH for Qp (FB, 7.70 ± 2.68; EBH, 7.18 ± 2.34; IBH, 6.88 ± 2.51 l/min); however, no significant difference was found for Qs (FB, 3.44 ± 0.74; EBH, 3.40 ± 0.83; IBH, 3.40 ± 0.86 l/min). There were significant differences among the measurements during FB, EBH, and IBH for Qp/Qs (FB, 2.38 ± 1.12; EBH, 2.24 ± 0.95; IBH, 2.14 ± 0.97). Qp/Qs during FB and EBH correlated better with Qp/Qs measured by invasive oximetry than did IBH. The limit of agreement was smaller for EBH than for FB and IBH. In patients with secundum ASDs, Qp/Qs significantly decreased with breath-holding. The accuracy of the Qp/Qs measurement by MRI compared with invasive oximetry during EBH was higher than during FB and IBH.

  17. Bloqueio atrioventricular no pós-operatório de cirurgia cardíaca valvar: incidência, fatores de risco e evolução hospitalar Atrioventricular block in the postoperative period of heart valve surgery: incidence, risk factors and hospital evolution

    Directory of Open Access Journals (Sweden)

    Andres Di Leoni Ferrari

    2011-09-01

    Full Text Available INTRODUÇÃO: Distúrbios do sistema de condução cardíaco são complicações potenciais e conhecidas dos procedimentos de cirurgia cardíaca valvar. OBJETIVOS: Investigar a associação entre fatores peri-operatórios com bloqueio atrioventricular (BAV e a necessidade de estimulação cardíaca artificial temporária (ECAT e, se necessário, implante de marcapasso definitivo no pós-operatório de cirurgia cardíaca (POCC valvar. MÉTODOS: Coorte histórica de pacientes submetidos a cirurgia cardíaca valvar, sendo realizada análise de banco de dados por regressão logística. RESULTADOS: No período de janeiro de 1996 a dezembro de 2008, foram realizadas 1102 cirurgias cardíacas valvares: 718 (65,2% na valva aórtica e 407 (36,9% na valva mitral; destas, 190 (17,2% cirurgias de revascularização miocárdica associadas à cirurgia valvar e 23 (2,1% cirurgias valvares combinadas (aórtica+mitral. Cento e oitenta e sete (17% pacientes apresentaram quadro clínico e eletrocardiográfico de BAV durante o POCC valvar, necessitando de ECAT. Quatorze (7,5% pacientes evoluíram para implante de marcapasso definitivo (1,27% do total da amostra. A análise multivariada evidenciou associação significativa de BAV com cirurgia de valva mitral (OR=1,76; IC 95% 1,08-2,37; P=0,002, implante de prótese biológica (OR=1,59; IC 95% 1,02-3,91; P= 0,039, idade maior que 60 anos (OR = 1,99; IC 95% 1,35-2,85; PINTRODUCTION: Disturbances of the cardiac conduction system are potential complications after cardiac valve surgery. OBJECTIVES: This study was designed to investigate the association between perioperative factors and atrio-ventricular block, the need for temporary cardiac artificial pacing and, if necessary, permanent pacemaker implantation after cardiac valve surgery. METHODS: Retrospective analysis of the Cardiac Surgery Database - Hospital São Lucas/PUCRS. The data are collected prospectively and analyzed retrospectively. RESULTS: Between January

  18. Acute oral administration of low doses of methylphenidate targets calretinin neurons in the rat septal area.

    Directory of Open Access Journals (Sweden)

    Alvaro eGarcía-Aviles

    2015-03-01

    Full Text Available Methylphenidate (MPD is a commonly administered drug to treat children suffering from attention deficit hyperactivity disorder (ADHD. Alterations in septal driven hippocampal theta rhythm may underlie attention deficits observed in these patients. Amongst others, the septo-hippocampal connections have long been acknowledged to be important in preserving hippocampal function. Thus, we wanted to ascertain if methylphenidate administration, which improves attention in patients, could affect septal areas connecting with hippocampus. We used low and orally administered methylphenidate doses (1.3; 2.7 and 5mg/Kg to rats what mimics the dosage range in humans. In our model, we observed no effect when using 1.3mg/Kg methylphenidate; whereas 2.7 and 5 mg/Kg induced a significant increase in c-fos expression specifically in the medial septum, an area intimately connected to the hippocampus. We analyzed dopaminergic areas such as nucleus accumbens and striatum, and found that only 5mg/Kg induced c-fos levels increase. In these areas tyrosine hydroxylase correlated well with c-fos staining, whereas in the medial septum the sparse tyrosine hydroxylase fibres did not overlap with c-fos positive neurons. Double immunofluorescence of c-fos with neuronal markers in the septal area revealed that co-localization with choline acethyl transferase, parvalbumin, and calbindin with c-fos did not change with MPD treatment; whereas, calretinin and c-fos double labeled neurons increased after MPD administration. Altogether, these results suggest that low and acute doses of methylphenidate primary target specific populations of caltretinin medial septal neurons.

  19. Inhibitory effects of Urtica dioica L. root on electrophysiological properties of isolated rabbit atrioventricular node

    OpenAIRE

    A. Enayati; V. Khori*; M. Azadbakhat; M. Zahedi

    2017-01-01

    Background and objectives: The ideal drug for treatment of a wide range of supraventricular arrhythmia hasn't yet been developed. Previous studies have shown antihypertensive and negative inotropic effects of the Urtica dioica L. (nettle). Therefore, the aim of present study is to determine the rate dependent inhibitory effects of ethanol extract of nettle root and investigate the role of adrenoceptors in the anti-arrhythmic mechanism of nettle on the isolated rabbit atrio-ventricular node. M...

  20. The anatomy and histology of the atrioventricular conducting system in the hedgehog (Hemiechinus auritus) heart

    OpenAIRE

    NABIPOUR, Abolghasem

    2014-01-01

    This study examined the atrioventricular conducting system in 4 adult male hedgehogs (Hemiechinus auritus). The histological structure of these components was studied using routine histological methods. The AVN was located at the lower and anterior part of the interatrial septum, near the root of the aorta. It was almost oval and consisted of twisted cells. Internodal pathways in the hedgehog heart were not observed, but there were numerous purkinje-like fibers within the myocardium of the at...