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Sample records for heart septal defects

  1. Ventricular Septal Defect (VSD)

    ... 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 ...

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

    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

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

    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

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

    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.

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

    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.

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

    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.

  7. Early closure of postinfarction ventricular septal defects.

    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.

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

    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

  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

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

    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

  11. Three-dimensional echocardiographic assessment of atrial septal defects

    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.

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

    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,...

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

    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.

  14. Angiographic differentiation of type of ventricular septal defects

    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

  15. Ventricular Septal Defect (For Teens)

    ... 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 ...

  16. Atrial Septal Defect (For Teens)

    ... 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 ...

  17. Facts about Ventricular Septal Defect

    ... 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 ( ...

  18. Facts about Atrial Septal Defect

    ... 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 ( ...

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

    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.

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

    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

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

    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.

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

    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.

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

    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.

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

    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.

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

    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)

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

    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.

  7. Familial Atrial Septal Defect and Sudden Cardiac Death

    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...

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

    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.

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

    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.

  10. MORTAL PULPOTOMY ON CHILDREN WITH VENTRICULAR SEPTAL DEFECT

    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.

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

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

    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.

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

    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

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

    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.

  15. Multiple ventricular septal defects: a new strategy.

    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

  16. MULTIPLE VENTRICULAR SEPTAL DEFECTS: A NEW STRATEGY

    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

  17. Diagnosis of atrial septal defect using magnetic resonance imaging

    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. Histopathologic analysis of atrial tissue in patients with atrial fibrillation: comparison between patients with atrial septal defect and patients with mitral valvular heart disease.

    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.

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

    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.

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

    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.

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

    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.

  2. Aspects of surgery for congenital ventricular septal defect

    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

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

    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.

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

    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 ...

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

    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.

  6. Current perspectives in percutaneous atrial septal defect closure devices

    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

  7. Facts about Atrioventricular Septal Defect (AVSD)

    ... 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 ...

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

    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.

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

    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.

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

    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.

  11. Pulmonary edema following transcatheter closure of atrial septal defect

    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.

  12. 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.

    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.

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

    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.

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

    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.

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

    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 ...

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

    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

  17. Facts about Congenital Heart Defects

    ... 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 ...

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

    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.

  19. Acquired ventricular septal defect due to infective endocarditis

    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.

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

    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.

  1. Congenital heart defects in Williams syndrome.

    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.

  2. Fourier ventricular amplitude ratio to evaluate atrial septal defect

    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

  3. Closure of secundum atrial septal defect in adults

    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)

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

    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.

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

    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

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

    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.

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

    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...

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

    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

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

    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.

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

    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

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

    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.

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

    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.)

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

    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.)

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

    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.

  15. 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

    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.

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

    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.

  17. Congenital Heart Defects and CCHD

    ... and more. Stony Point, NY 10980 Close X Home > Complications & Loss > Birth defects & other health conditions > Congenital heart defects and ... in congenital heart defects. You have a family history of congenital heart ... syndrome or VCF. After birth Your baby may be tested for CCHD as ...

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

    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.

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

    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

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

    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.

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

    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

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

    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.

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

    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.

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

    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).

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

    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.

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

    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)

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

    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

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

    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

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

    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.

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

    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.

  11. Congenital Heart Defects (For Parents)

    ... to be associated with genetic disorders, such as Down syndrome . But the cause of most congenital heart defects isn't known. While they can't be prevented, many treatments are available for the defects and related health ...

  12. Blood flow patterns underlie developmental heart defects.

    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.

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

    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

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

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

    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.

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

    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

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

    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.

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

    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.

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

    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)

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

    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.

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

    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.

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

    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

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

    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)

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

    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)

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

    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...

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

    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

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

    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

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

    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

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

    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.

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

    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.

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

    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

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

    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)

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

    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.

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

    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

  15. What Are Congenital Heart Defects?

    ... a baby with a congenital heart defect. Family history and genetics Congenital heart disease is not usually passed along ... you or your child to a specialist in genetic testing. Cardiac MRI to diagnose a ... Factors to review family history, smoking, and medicines that increase your risk of ...

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

    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

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

    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.

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

    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.

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

    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

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

    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.

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

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

    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.

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

    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.

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

    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.

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

    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 ...

  6. Natural history of ventricular septal defects in Nigerian children

    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 ...

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

    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%.

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

    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.

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

    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.

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

    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.

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

    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.

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

    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)

  13. Atrial septal defect in a Korean wild raccoon dog

    YIM, Soomi; CHOI, Sooyoung; KIM, Jongtaek; CHUNG, Jin-Young; PARK, Inchul

    2017-01-01

    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...

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

    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)

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

    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)

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

    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.

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

    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.

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

    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.

  19. From the Heart: Interatrial Septal Aneurysm Identified on Bedside Ultrasound

    Michael Butterfield

    2014-09-01

    Full Text Available A 61 year-old man presented to the Emergency Department for one day of nonspecific chest pain. Bedside echocardiogram performed by the emergency physician revealed normal systolic cardiac function but also showed a large ( > 10mm bicornuate interatrial septal aneurysm (IASA projecting into the right atrium (Figure 1, Video 1. There was no evidence of intraatrial thrombus. A formal echocardiogram performed later that day confirmed the diagnosis and also detected a patent foramen ovale (PFO with a left-to-right shunt that reversed with Valsalva maneuver. [West J Emerg Med. 2014;15(6:719–720

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

    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)

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

    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)

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

    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

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

    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.

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

    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.

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

    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.

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

    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)

  7. Congenital heart defects in children with oral clefts

    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.

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

    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.

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

    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.

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

    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

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

    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.

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

    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.

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

    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.

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

    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

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

    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

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

    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. Genetic Counseling for Congenital Heart Defects

    ... Artery Disease Venous Thromboembolism Aortic Aneurysm More Genetic Counseling for Congenital Heart Defects Updated:Jan 19,2018 ... with congenital heart disease considers having children. Genetic counseling can help answer these questions and address your ...

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

    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.

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

    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.

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

    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)

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

    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

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

    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

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

    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.

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

    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).

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

    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.

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

    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.

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

    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.

  8. The septal bulge--an early echocardiographic sign in hypertensive heart disease.

    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.

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

    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.

  10. Gerbode defect and multivalvular dysfunction: Complex complications in adult congenital heart disease.

    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.

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

    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)

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

    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.)

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

    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.

  14. Congenital heart defects in oculodentodigital dysplasia: Report of two cases.

    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.

  15. Congenital heart defects and extracardiac malformations.

    Rosa, Rosana Cardoso M; Rosa, Rafael Fabiano M; Zen, Paulo Ricardo G; Paskulin, Giorgio Adriano

    2013-06-01

    To review the association between congenital heart defects and extracardiac malformations. Scientific articles were searched in the Medline, Lilacs, and SciELO databases, using the descriptors "congenital heart disease," "congenital heart defects," "congenital cardiac malformations," "extracardiac defects," and "extracardiac malformations." All case series that specifically explored the association between congenital heart defects and extracardiac malformations were included. Congenital heart diseases are responsible for about 40% of birth defects, being one of the most common and severe malformations. Extracardiac malformations are observed in 7 to 50% of the patients with congenital heart disease, bringing a greater risk of comorbidity and mortality and increasing the risks related to heart surgery. Different studies have attempted to assess the presence of extracardiac abnormalities in patients with congenital heart disease. Among the changes described, those of the urinary tract are more often reported. However, no study has evaluated all patients in the same way. Extracardiac abnormalities are frequent among patients with congenital heart disease, and patients with these alterations may present an increased risk of morbimortality. Therefore, some authors have been discussing the importance and cost-effectiveness of screening these children for other malformations by complementary exams.

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

    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.

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

    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.

  18. Potential implications of the helical heart in congenital heart defects.

    Corno, Antonio F; Kocica, Mladen J

    2007-01-01

    The anatomic and functional observations made by Francisco Torrent-Guasp, in particular his discovery of the helical ventricular myocardial band (HVMB), have challenged what has been taught to cardiologists and cardiac surgeons over centuries. A literature debate is ongoing, with interdependent articles and comments from supporters and critics. Adequate understanding of heart structure and function is obviously indispensable for the decision-making process in congenital heart defects. The HVMB described by Torrent-Guasp and the potential impact on the understanding and treatment of congenital heart defects has been analyzed in the following settings: embryology, ventriculo-arterial discordance (transposition of great arteries), Ebstein's anomaly, pulmonary valve regurgitation after repair of tetralogy of Fallot, Ross operation, and other congenital heart defects. The common structural spiral feature is only one of the elements responsible for the functional interaction of right and left ventricles, and understanding the form/function relationship in congenital heart defects is more difficult than for acquired heart disease because of the variety and complexity of congenital heart defects. Individuals involved in the care of patients with congenital heart defects have to be stimulated to consider further investigations and alternative surgical strategies.

  19. Atrial septal stenting - How I do it?

    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.

  20. Adults with Congenital Heart Defects

    ... Ebstein's Anomaly l-Transposition of the Great Arteries Patent Ductus Arteriosus (PDA) Pulmonary Valve Stenosis and Regurgitation ... Sodium and Salt 3 Heart Attack Symptoms in Women 4 Warning Signs of a Heart Attack 5 ...

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

    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

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

    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.

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

    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.

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

    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)

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

    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.

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

    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.

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

    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.

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

    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

  9. Study of prevalence & risk factors of congenital heart defect (Review Article

    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.

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

    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.

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

    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.

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

    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.

  13. Congenital heart defect corrective surgeries

    ... make it bigger with a patch made of Gore-tex, a man-made (synthetic) material. Another way ... 434. Bhatt AB, Foster E, Kuehl K, et al; American Heart Association Council on Clinical Cardiology. Congenital ...

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

    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.

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

    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.

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

    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

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

    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.

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

    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.

  19. 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.

    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.

  20. Myocardial glucose metabolism is different between hypertrophic cardiomyopathy and hypertensive heart disease associated with asymmetrical septal hypertrophy

    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)

  1. Cyanotic Congenital Heart Defects – literature review

    Vlad Drăgoi

    2017-11-01

    Full Text Available Congenital heart defects are one of the most interesting and important chapters regarding abnormal fetal growth pathology. The objective of this article is to present a literature review for the main cyanotic congenital heart defects. The ones presented in the article are: tetralogy of Fallot, transposition of the great vessels, double outlet right ventricle, truncus arteriosus, total anomalous pulmonary venous circulation and additional information regarding very rare malformations such as pentalogy of Cantrell and Uhl anomaly. An early and precise identification of congenital heart defects is an important step in an accurate follow-up of a potential problematic pregnancy. Knowing the sonographic aspect, associated pathology and the current available treatment procedures are vital for the fetal outcome and for the physician to adapt the right management in every situation that might appear during the pregnancy and in the neonatal period.

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

    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.

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

    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.

  4. 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

    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

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

    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.

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

    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.

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

    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.

  8. Angio- and echocardiography: viewing heart defects in more detail

    Goodwin, J.K.; Lombard, C.W.

    1990-01-01

    Angio- and echocardiography can help to confirm the diagnosis or evaluate the severity of a heart defect. Angiograms of dogs with pulmonic stenosis and tetralogy of Fallot are shown. Echocardiographic features of common congenital heart defects are summarized

  9. If Your Child Has a Heart Defect (For Parents)

    ... Videos for Educators Search English Español If Your Child Has a Heart Defect KidsHealth / For Parents / If ... has any of these symptoms. Caring for Your Child Parenting kids with heart defects includes learning about ...

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

    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

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

    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. Left septal atrial tachycardia after open-heart surgery: relevance to surgical approach, anatomical and electrophysiological characteristics associated with catheter ablation, and procedural outcomes.

    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.

  13. Congenital Heart Defects and Coronary Anatomy

    Mawson, John B.

    2002-01-01

    Coronary artery anomalies are a well recognized feature of many cardiac malformations and have been catalogued in a number of reviews. This overview concentrates on 1) the interplay between congenital heart defects and coronary morphogenesis, examining how some of the embryology fits with the experiments of nature encountered in clinical practice; and 2) the influence of coronary anatomy on patient management. This overview uses, as examples, pulmonary atresia with intact ventricular septum, ...

  14. Transcatheter closure of post-operative residual ventricular septal defect using a patent ductus arteriosus closure device in an adult: a case report.

    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.

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

    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.

  16. Functional study of DAND5 variant in patients with Congenital Heart Disease and laterality defects.

    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.

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

    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

  18. 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.

    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.

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

    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.

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

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

    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.

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

    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

  3. 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?

    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.

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

    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.

  5. 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.

    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

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

    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.

  7. How Do We Define Congenital Heart Defects for Scientific Studies?

    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....

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

    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.

  9. Holt-Oram Syndrome in Adult Presenting with Heart Failure: A Rare Presentation

    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.

  10. Catheter Ablation of Atrial Fibrillation in Patients with Hardware in the Heart - Septal Closure Devices, Mechanical Valves and More.

    Bartoletti, Stefano; Santangeli, Pasquale; DI Biase, Luigi; Natale, Andrea

    2013-01-01

    Patients with mechanical "hardware" in the heart, such as those with mechanical cardiac valves or atrial septal closure devices, represent a population at high risk of developing AF. Catheter ablation of AF in these subjects might represent a challenge, due to the perceived higher risk of complications associated with the presence of intracardiac mechanical devices. Accordingly, such patients were excluded or poorly represented in major trials proving the benefit of catheter ablation for the rhythm-control of AF. However, recent evidence supports the concept that catheter ablation procedures might be equally effective in these patients, without a significant increase in the risk of procedural complications. This review will summarize the current state-of-the-art on catheter ablation of AF in patients with mechanical "hardware" in the heart.

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

    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.

  12. Risk factors predisposing to congenital heart defects

    Ul Haq, Faheem; Jalil, Fatima; Hashmi, Saman; Jumani, Maliha Iqbal; Imdad, Aamer; Jabeen, Mehnaz; Hashmi, Javad Tauseef; Irfan, Furqan Bin; Imran, Muhammad; Atiq, Mehnaz

    2011-01-01

    Congenital heart disease (CHD) is associated with multiple risk factors, consanguinity may be one such significant factor. The role of consanguinity in the etiology of CHD is supported by inbreeding studies, which demonstrate an autosomal recessive pattern of inheritance of some congenital heart defects. This study was done to find out the risk factors for CHD. A case-control study was done on pediatric patients at a tertiary care hospital, Aga Khan University Hospital, located in Karachi, Pakistan. A total of 500 patients, 250 cases and 250 controls were included in the study. Amongst the 250 cases (i.e. those diagnosed with CHD), 122 patients (48.8%) were born of consanguineous marriages while in the controls (i.e. non-CHD) only 72 patients (28.9%) showed a consanguinity amongst parents. On multivariate analysis, consanguinity emerged as an independent risk factor for CHD; adjusted odds ratio 2.59 (95% C. I. 1.73 - 3.87). Other risk factors included low birth weight, maternal co-morbidities, family history of CHD and first born child. On the other hand, medications used by the mother during the index pregnancy, maternal age and gender of the child did not significantly increase the risk of developing CHD. Analyses of our results show that parental consanguinity, family history of CHD, maternal co-morbidities, first born child and low birth weight are independent risk factors for CHD

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

    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

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

    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)

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

    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.

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

    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.

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

    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.

  18. Towards image-guided atrial septal defect repair: an ex vivo analysis

    Kwartowitz, David M.; Mefleh, Fuad N.; Baker, George H.

    2012-02-01

    The use of medical images in the operating room for navigation and planning is well established in many clinical disciplines. In cardiology, the use of fluoroscopy for the placement of catheters within the heart has become the standard of care. While fluoroscopy provides a live video sequence with the current location, it poses risks the patient and clinician through exposure to radiation. Radiation dose is cumulative and thus children are at even greater risk from exposure. To reduce the use of radiation, and improve surgical technique we have begun development of an image-guided navigation system, which can deliver therapeutic devices via catheter. In this work we have demonstrated the intrinsic properties of our imaging system, which have led to the development of a phantom emulating a childs heart with an ASD. Further investigation into the use of this information, in a series of mock clinical experiments, will be performed to design procedures for inserting devices into the heart while minimizing fluoroscopy use.

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

    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

  20. Congenital Heart Diseases in Adults: A Review of Echocardiogram ...

    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.

  1. Fabrication and characterization of chitosan nanoparticles and collagen-loaded polyurethane nanocomposite membrane coated with heparin for atrial septal defect (ASD) closure.

    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.

  2. Retrospective analysis of transthoracic echocardiography about the failure of transcather closure of ventricular septal defect

    Zhang Yigang; Li Shijie; Liu Ru; Li Zhihong; Fu Qiang

    2008-01-01

    Objective: To evaluate the value of echocardiography in investigation the failure of interventional therapy of VSDs and to increase the successful rate. Methods: 15 cases with failure of VSD closure through interventional approach were undertaken measurement of major parameters of the defect on left ventriculography and then followed by Philips 5500 color Doppler US for repeated multi-direction tangential measuring of the size, morphologic change, relation with peripheral structure and individual valvular regurgitation. Results: (1) Marginal membranous VSD 10 eases; membranance aneurysm 8 cases, ≥ 2 outlets 6 cases. Width of basal part of membranous aneurysm was 8-18 (10±2) mm with depth of 3 10 (6±2) mm, distance from aortic valve was 0-6 (2±1) mm and from tricuspid valve was 2-5 (2±1) mm; associated with slight tricuspid valvular regurgitation. Aortic valvular regurgitation 5 cases showed slight to mild amount and without membranous aneurysm occurred in 2 cases. (2) Intracristal VSD 5 cases showed the size of defect as 6-10 (7±1) mm, with distances of 2-3 (1±0.8) mm from pulmonary valve and 1-2 (1±0.6) mm from aortic valve associated with slight regurgitation in 4 cases, slight tricuspid valvular regurgitation in 5 eases and aortic valvular prolapse in 4 cases. Conclusions: Many factors can influence the direction tangential continuous scanning should be taken for accurate localization and measurement of VSD in order to select the very sight occludor providing high successful rate of occlusion and long term efficacy. (authors)

  3. Over-expression of DSCAM and COL6A2 cooperatively generates congenital heart defects.

    Tamar R Grossman

    2011-11-01

    Full Text Available A significant current challenge in human genetics is the identification of interacting genetic loci mediating complex polygenic disorders. One of the best characterized polygenic diseases is Down syndrome (DS, which results from an extra copy of part or all of chromosome 21. A short interval near the distal tip of chromosome 21 contributes to congenital heart defects (CHD, and a variety of indirect genetic evidence suggests that multiple candidate genes in this region may contribute to this phenotype. We devised a tiered genetic approach to identify interacting CHD candidate genes. We first used the well vetted Drosophila heart as an assay to identify interacting CHD candidate genes by expressing them alone and in all possible pairwise combinations and testing for effects on rhythmicity or heart failure following stress. This comprehensive analysis identified DSCAM and COL6A2 as the most strongly interacting pair of genes. We then over-expressed these two genes alone or in combination in the mouse heart. While over-expression of either gene alone did not affect viability and had little or no effect on heart physiology or morphology, co-expression of the two genes resulted in ≈50% mortality and severe physiological and morphological defects, including atrial septal defects and cardiac hypertrophy. Cooperative interactions between DSCAM and COL6A2 were also observed in the H9C2 cardiac cell line and transcriptional analysis of this interaction points to genes involved in adhesion and cardiac hypertrophy. Our success in defining a cooperative interaction between DSCAM and COL6A2 suggests that the multi-tiered genetic approach we have taken involving human mapping data, comprehensive combinatorial screening in Drosophila, and validation in vivo in mice and in mammalian cells lines should be applicable to identifying specific loci mediating a broad variety of other polygenic disorders.

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

    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.

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

    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.

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

    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

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

    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.

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

    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.

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

    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)

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

    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.

  11. Type 2 diabetes mellitus induces congenital heart defects in murine embryos by increasing oxidative stress, endoplasmic reticulum stress, and apoptosis.

    Wu, Yanqing; Reece, E Albert; Zhong, Jianxiang; Dong, Daoyin; Shen, Wei-Bin; Harman, Christopher R; Yang, Peixin

    2016-09-01

    Maternal type 1 and 2 diabetes mellitus are strongly associated with high rates of severe structural birth defects, including congenital heart defects. Studies in type 1 diabetic embryopathy animal models have demonstrated that cellular stress-induced apoptosis mediates the teratogenicity of maternal diabetes leading to congenital heart defect formation. However, the mechanisms underlying maternal type 2 diabetes mellitus-induced congenital heart defects remain largely unknown. We aim to determine whether oxidative stress, endoplasmic reticulum stress, and excessive apoptosis are the intracellular molecular mechanisms underlying maternal type 2 diabetes mellitus-induced congenital heart defects. A mouse model of maternal type 2 diabetes mellitus was established by feeding female mice a high-fat diet (60% fat). After 15 weeks on the high-fat diet, the mice showed characteristics of maternal type 2 diabetes mellitus. Control dams were either fed a normal diet (10% fat) or the high-fat diet during pregnancy only. Female mice from the high-fat diet group and the 2 control groups were mated with male mice that were fed a normal diet. At E12.5, embryonic hearts were harvested to determine the levels of lipid peroxides and superoxide, endoplasmic reticulum stress markers, cleaved caspase 3 and 8, and apoptosis. E17.5 embryonic hearts were harvested for the detection of congenital heart defect formation using India ink vessel patterning and histological examination. Maternal type 2 diabetes mellitus significantly induced ventricular septal defects and persistent truncus arteriosus in the developing heart, along with increasing oxidative stress markers, including superoxide and lipid peroxidation; endoplasmic reticulum stress markers, including protein levels of phosphorylated-protein kinase RNA-like endoplasmic reticulum kinase, phosphorylated-IRE1α, phosphorylated-eIF2α, C/EBP homologous protein, and binding immunoglobulin protein; endoplasmic reticulum chaperone gene

  12. Alarm!!! A UFO inside the heart.

    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.

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

    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.

  14. 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.

    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.

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

    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

  16. Comparison between transcatheter and surgical closure of secundum atrial septal defect in patients over 40 years old

    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)

  17. Percutaneous closure of patent foramen ovale and atrial septal defect in adults: the impact of clinical variables and hospital procedure volume on in-hospital adverse events.

    Opotowsky, Alexander R; Landzberg, Michael J; Kimmel, Stephen E; Webb, Gary D

    2009-05-01

    Percutaneous closure of patent foramen ovale/atrial septal defect (PFO/ASD) is an increasingly common procedure perceived as having minimal risk. There are no population-based estimates of in-hospital adverse event rates of percutaneous PFO/ASD closure. We used nationally representative data from the 2001-2005 Nationwide Inpatient Sample to identify patients >or-=20 years old admitted to an acute care hospital with an International Classification of Diseases, Ninth Revision code designating percutaneous PFO/ASD closure on the first or second hospital day. Variables analyzed included age, sex, number of comorbidities, year, same-day use of intracardiac or other echocardiography, same-day left heart catheterization, hospital size and teaching status, PFO/ASD procedural volume, and coronary intervention volume. Outcomes of interest included length of stay, charges, and adverse events. The study included 2,555 (weighted to United States population: 12,544 +/- 1,987) PFO/ASD closure procedures. Mean age was 52.0 +/- 0.4 years, and 57.3% +/- 1.0% were women. Annual hospital volume averaged 40.8 +/- 7.7 procedures (range, 1-114). Overall, 8.2 +/- 0.8% of admissions involved an adverse event. Older patients and those with comorbidities were more likely to sustain adverse events. Use of intracardiac echocardiography was associated with fewer adverse events. The risk of adverse events was inversely proportional to annual hospital volume (odds ratio [OR] 0.91, 95% confidence interval [CI] 0.86-0.96, per 10 procedures), even after limiting the analysis to hospitals performing >or=10 procedures annually (OR 0.91, 95% CI 0.85-0.98). Adverse events were more frequent at hospitals in the lowest volume quintile as compared with the highest volume quintile (13.3% vs 5.4%, OR 2.42, 95% CI 1.55-3.78). The risk of adverse events of percutaneous PFO/ASD closure is inversely correlated with hospital volume. This relationship applies even to hospitals meeting the current guidelines

  18. 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.

    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.

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

    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...

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

    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.

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

    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.

  2. 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.

    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.

  3. 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

    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.

  4. Surgical treatment of interrupted aortic arch associated with ventricular septal defect and patent ductus arteriosus in patients over one year of age.

    Li, Zhiqiang; Li, Bin; Fan, Xiangming; Su, Junwu; Zhang, Jing; He, Yan; Liu, Yinglong

    2014-01-01

    Interrupted aortic arch (IAA) is a rare congenital anomaly affecting 1.5% of infants with congenital heart disease. Neonatal repair of IAA is required to avoid irreversible pulmonary vascular lesion. However, in China, patients with IAA associated with ventricular septal defect (VSD) and patent ductus arteriosus (PDA) over one year of age are common. So we investigated the outcome of surgical treatment of IAA with VSD and PDA in patients over one year of age. From January 2009 to December 2012, 19 patients with IAA have undergone complete single-stage repair. The patients' mean age was 4.4 years, ranging 1 to 15 years; and their mean weight was 12.8 kg, ranging 4.2 to 36.0 kg. Fifteen IAA were type A, four were type B. Preoperative cardiac catheterization data were available from all patients. Mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) were measured. The measurements of postoperative pulmonary artery pressure were taken in the operating room at the end of the case. All patients underwent echocardiographic examinations before discharged from the hospital. In addition, cardiac catheterization and echocardiographic examinations were performed during follow-up. Selective brain perfusion through the innominate artery during aortic arch reconstruction was used in all patients. Mean follow-up was (1.6±0.8) years. There were two hospital deaths (2/19, 11%). One patient died of pulmonary hypertension crisis, and another died of postoperative low cardiac output. Five cases had other main postoperative complications but no postoperative neurologic complications. Seventeen survivors were followed up, and there were no late deaths or reoperation. Mean cross-clamp duration was (85±22) minutes and selective brain perfusion duration was (34±11) minutes. Two patients required delayed sternal closure at two days postoperatively. Intensive care unit and hospital stays were (9±8) days and (47±24) days, respectively. Pressure gradients across

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

    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.

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

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

    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.

  8. Congenital Anorectal Malformation Severity Does Not Predict Severity of Congenital Heart Defects.

    Jonker, Jara E; Liem, Eryn T; Elzenga, Nynke J; Molenbuur, Bouwe; Trzpis, Monika; Broens, Paul M A

    2016-12-01

    To determine the prevalence of congenital heart defects (CHDs) in patients with mild or severe congenital anorectal malformations (CARMs), and whether all patients with CARM need pediatric cardiology screening. We included 129 patients with CARM born between 2004 and 2013, and referred to University Medical Center Groningen. Recto-perineal and recto-vestibular fistulas were classified as mild CARMs, all others as severe. Significant patent foramen ovale, secundum atrial septal defect, and small ventricular septum defect were classified as minor CHDs, all others as major. Of 129 patients with CARM, 67% had mild CARM, 33% severe CARM, and 17% were additionally diagnosed with CHD. CHDs were distributed equally in patients with mild or severe CARMs. Patients with multiple congenital abnormalities were more frequently diagnosed with CHD (n = 16, 36%) than patients without multiple congenital malformations (n = 5, 9%, P = .001). Patients with CARM diagnosed with CHD using pediatric cardiac echo screening were younger than 3 months of age at diagnosis. Earlier general pediatric examinations missed 7 (50%) children with mild and 4 (50%) with severe CHDs. The severity of CARM could predict neither prevalence nor severity of CHD. More than one-half of CHDs were missed during the first physical examination. No new CHDs were found in patients older than 3 months of age at the time CARMs were diagnosed. We recommend screening all patients with CARM younger than 3 months of age for CHD at the time CARM is diagnosed. Preoperative echocardiography should be the rule in children younger than 3 months of age and with multiple congenital anomalies. Copyright © 2016 Elsevier Inc. All rights reserved.

  9. Maternal folic acid supplementation and dietary folate intake and congenital heart defects.

    Baohong Mao

    Full Text Available It has been reported that folic acid supplementation before and/or during pregnancy could reduce the risk of congenital heart defects (CHDs. However, the results from limited epidemiologic studies have been inconclusive. We investigated the associations between maternal folic acid supplementation, dietary folate intake, and the risk of CHDs.A birth cohort study was conducted in 2010-2012 at the Gansu Provincial Maternity & Child Care Hospital in Lanzhou, China. After exclusion of stillbirths and multiple births, a total of 94 births were identified with congenital heart defects, and 9,993 births without any birth defects. Unconditional logistic regression was used to estimate the associations.Compared to non-users, folic acid supplement users before pregnancy had a reduced risk of overall CHDs (OR: 0.42, 95% CI: 0.21-0.86, Ptrend = 0.025 after adjusted for potential confounders. A protective effect was observed for certain subtypes of CHDs (OR: 0.37, 95% CI: 0.16-0.85 for malformation of great arteries; 0.26, 0.10-0.68 for malformation of cardiac septa; 0.34, 0.13-0.93 for Atrial septal defect. A similar protective effect was also seen for multiple CHDs (OR: 0.49, 95% CI: 0.26-0.93, Ptrend = 0.004. Compared with the middle quartiles of dietary folate intake, lower dietary folate intake (<149.88 μg/day during pregnancy were associated with increased risk of overall CHDs (OR: 1.63, 95% CI: 1.01-2.62 and patent ductus arteriosus (OR: 1.85, 95% CI: 1.03-3.32. Women who were non-user folic acid supplement and lower dietary folate intake have almost 2-fold increased CHDs risk in their offspring.Our study suggested that folic acid supplementation before pregnancy was associated with a reduced risk of CHDs, lower dietary folate intake during pregnancy was associated with increased risk. The observed associations varied by CHD subtypes. A synergistic effect of dietary folate intake and folic acid supplementation was also observed.

  10. Congenital Heart Defects and Receipt of Special Education Services.

    Riehle-Colarusso, Tiffany; Autry, Andrew; Razzaghi, Hilda; Boyle, Coleen A; Mahle, William T; Van Naarden Braun, Kim; Correa, Adolfo

    2015-09-01

    We investigated the prevalence of receipt of special education services among children with congenital heart defects (CHDs) compared with children without birth defects. Children born from 1982 to 2004 in metropolitan Atlanta with CHDs (n = 3744) were identified from a population-based birth defect surveillance program; children without birth defects (n = 860 715) were identified from birth certificates. Cohorts were linked to special education files for the 1992-2012 school years to identify special education services. Children with noncardiac defects or genetic syndromes were excluded; children with CHDs were classified by presence or absence of critical CHDs (ie, CHDs requiring intervention by age one year). We evaluated the prevalence of receipt of special education services and prevalence rate ratios using children without birth defects as a reference. Compared with children without birth defects, children with CHDs were 50% more likely to receive special education services overall (adjusted prevalence rate ratio [aPRR] = 1.5; 95% confidence interval [CI]: 1.4-1.7). Specifically, they had higher prevalence of several special education categories including: intellectual disability (aPRR = 3.8; 95% CI: 2.8-5.1), sensory impairment (aPRR = 3.0; 95% CI: 1.8-5.0), other health impairment (aPRR = 2.8; 95% CI: 2.2-3.5), significant developmental delay (aPRR = 1.9; 95% CI: 1.3-2.8), and specific learning disability (aPRR = 1.4; 95% CI: 1.1-1.7). For most special education services, the excess prevalence did not vary by presence of critical CHDs. Children with CHDs received special education services more often than children without birth defects. These findings highlight the need for special education services and the importance of developmental screening for all children with CHDs. Copyright © 2015 by the American Academy of Pediatrics.

  11. 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.

    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.

  12. Results of beating heart mitral valve surgery via the trans-septal approach Resultados da abordagem transeptal para a valva mitral com coração batendo

    Tomas A Salerno

    2009-03-01

    Full Text Available OBJECTIVE: Mitral valve surgery can be performed through the trans-atrial or the trans-septal approach. Although the trans-atrial is the preferred method, the trans-septal approach has also been used recently and has a particular value in beating-heart mitral valve surgery. Herein we report our experience with beating-heart mitral valve surgery via trans-septal approach, and discuss its advantages and pitfalls. METHODS: Between 2000 and 2007, 214 consecutive patients were operated upon utilizing beating heart technique for mitral valve surgery. The operation was performed via transseptal approach with the aorta unclamped, the heart beating, with normal electrocardiogram and in sinus rhythm. RESULTS: Mean age was 56.03 ± 13.93 years (range: 19-86 years; median: 56 years. There were 131 (61.2% males and 83 (38.8% females. Of the prostheses used, 108 (50.5% were biological, and 39 (18.2% were mechanical. Mitral repairs were performed in 67 (31.3% patients. Mean hospital stay was 17.4 ± 20.0 days (range: 3-135 days; median: 11 days. Intra-aortic balloon pump (IABP utilization was required in 12 (5.6% of 214 patients. One-month mortality was 7.4%, and re-operation for bleeding was needed in 15 (7% patients. CONCLUSIONS: Beating-heart mitral valve surgery is an option for myocardial protection in patients undergoing mitral valve surgery. This technique is facilitated by the trans-septal approach due to reduced aortic insufficiency and improved visualization of the mitral apparatus.OBJETIVO: A cirurgia da valva mitral pode ser feita via transatrial ou transeptal. Embora a transatrial seja a preferida, a via transeptal tem sido utilizada mais recentemente e tido um grande valor nas operações com o coração batendo. Mostramos a nossa experiência na cirurgia da valva mitral via transeptal com coração batendo e discutimos seus benefícios e problemas. MÉTODOS: Entre 2000 e 2007, 214 pacientes consecutivos foram operados com o coração batendo. A

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

    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.

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

    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.

  15. Warden repair for superior sinus venosus atrial septal defect and anomalous pulmonary venous drainage in children: Anesthesia and transesophageal echocardiography perspective

    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.

  16. Ambient air pollution and congenital heart defects in Lanzhou, China

    Jin, Lan; Qiu, Jie; Zhang, Yaqun; Qiu, Weitao; He, Xiaochun; Wang, Yixuan; Sun, Qingmei; Li, Min; Zhao, Nan; Cui, Hongmei; Liu, Sufen; Tang, Zhongfeng; Chen, Ya; Yue, Li; Da, Zhenqiang; Xu, Xiaoying; Huang, Huang; Liu, Qing; Bell, Michelle L.; Zhang, Yawei

    2015-07-01

    Congenital heart defects are the most prevalent type of birth defects. The association of air pollution with congenital heart defects is not well understood. We investigated a cohort of 8969 singleton live births in Lanzhou, China during 2010-2012. Using inverse distance weighting, maternal exposures to particulate matter with diameters ≤10 μm (PM10), nitrogen dioxide (NO2), and sulfur dioxide (SO2) were estimated as a combination of monitoring station levels for time spent at home and in a work location. We used logistic regression to estimate the associations, adjusting for maternal age, education, income, BMI, disease, folic acid intake and therapeutic drug use, and smoking; season of conception, fuel used for cooking and temperature. We found significant positive associations of Patent Ductus Arteriosus (PDA) with PM10 during the 1st trimester, 2nd trimester and the entire pregnancy (OR 1st trimester = 3.96, 95% confidence interval (CI): 1.36, 11.53; OR 2nd trimester = 3.59, 95% CI: 1.57, 8.22; OR entire pregnancy = 2.09, 95% CI: 1.21, 3.62, per interquartile range (IQR) increment for PM10 (IQR = 71.2, 61.6, and 27.4 μg m-3, respectively)), and associations with NO2 during 2nd trimester and the entire pregnancy (OR 2nd trimester = 1.92, 95% CI: 1.11, 3.34; OR entire pregnancy = 2.32, 95% Cl: 1.14, 4.71, per IQR increment for NO2 (IQR = 13.4 and 10.9 μg m-3, respectively)). The associations for congenital malformations of the great arteries and pooled cases showed consistent patterns. We also found positive associations for congenital malformations of cardiac septa with PM10 exposures in the 2nd trimester and the entire pregnancy, and SO2 exposures in the entire pregnancy. Results indicate a health burden from maternal exposures to air pollution, with increased risk of congenital heart defects.

  17. Ambient air pollution and congenital heart defects in Lanzhou, China

    Jin, Lan; Bell, Michelle L; Qiu, Jie; Qiu, Weitao; He, Xiaochun; Wang, Yixuan; Sun, Qingmei; Cui, Hongmei; Liu, Sufen; Tang, Zhongfeng; Chen, Ya; Yue, Li; Da, Zhenqiang; Xu, Xiaoying; Liu, Qing; Zhang, Yaqun; Li, Min; Zhao, Nan; Huang, Huang; Zhang, Yawei

    2015-01-01

    Congenital heart defects are the most prevalent type of birth defects. The association of air pollution with congenital heart defects is not well understood. We investigated a cohort of 8969 singleton live births in Lanzhou, China during 2010–2012. Using inverse distance weighting, maternal exposures to particulate matter with diameters ≤10 μm (PM 10 ), nitrogen dioxide (NO 2 ), and sulfur dioxide (SO 2 ) were estimated as a combination of monitoring station levels for time spent at home and in a work location. We used logistic regression to estimate the associations, adjusting for maternal age, education, income, BMI, disease, folic acid intake and therapeutic drug use, and smoking; season of conception, fuel used for cooking and temperature. We found significant positive associations of Patent Ductus Arteriosus (PDA) with PM 10 during the 1st trimester, 2nd trimester and the entire pregnancy (OR 1st trimester  = 3.96, 95% confidence interval (CI): 1.36, 11.53; OR 2nd trimester  = 3.59, 95% CI: 1.57, 8.22; OR entire pregnancy  = 2.09, 95% CI: 1.21, 3.62, per interquartile range (IQR) increment for PM 10 (IQR = 71.2, 61.6, and 27.4 μg m −3 , respectively)), and associations with NO 2 during 2nd trimester and the entire pregnancy (OR 2nd trimester  = 1.92, 95% CI: 1.11, 3.34; OR entire pregnancy  = 2.32, 95% Cl: 1.14, 4.71, per IQR increment for NO 2 (IQR = 13.4 and 10.9 μg m −3 , respectively)). The associations for congenital malformations of the great arteries and pooled cases showed consistent patterns. We also found positive associations for congenital malformations of cardiac septa with PM 10 exposures in the 2nd trimester and the entire pregnancy, and SO 2 exposures in the entire pregnancy. Results indicate a health burden from maternal exposures to air pollution, with increased risk of congenital heart defects. (letter)

  18. Maternal obesity and congenital heart defects: a population-based study123

    Mills, James L; Troendle, James; Conley, Mary R; Carter, Tonia; Druschel, Charlotte M

    2010-01-01

    Background: Obesity affects almost one-third of pregnant women and causes many complications, including neural tube defects. It is not clear whether the risk of congenital heart defects, the most common malformations, is also increased. Objective: This study was conducted to determine whether obesity is associated with an increased risk of congenital heart defects. Design: A population-based, nested, case-control study was conducted in infants born with congenital heart defects and unaffected controls from the cohort of all births (n = 1,536,828) between 1993 and 2003 in New York State, excluding New York City. The type of congenital heart defect, maternal body mass index (BMI; in kg/m2), and other risk factors were obtained from the Congenital Malformations Registry and vital records. Mothers of 7392 congenital heart defect cases and 56,304 unaffected controls were studied. Results: All obese women (BMI ≥ 30) were significantly more likely than normal-weight women (BMI: 19–24.9) to have children with a congenital heart defect [odds ratio (OR): 1.15; 95% CI: 1.07, 1.23; P heart defects with increasing maternal obesity (P heart syndrome, aortic stenosis, pulmonic stenosis, and tetralogy of Fallot. Conclusions: Obese, but not overweight, women are at significantly increased risk of bearing children with a range of congenital heart defects, and the risk increases with increasing BMI. Weight reduction as a way to reduce risk should be investigated. PMID:20375192

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

    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)

  20. Alpha-cardiac myosin heavy chain (MYH6) mutations affecting myofibril formation are associated with congenital heart defects.

    Granados-Riveron, Javier T; Ghosh, Tushar K; Pope, Mark; Bu'Lock, Frances; Thornborough, Christopher; Eason, Jacqueline; Kirk, Edwin P; Fatkin, Diane; Feneley, Michael P; Harvey, Richard P; Armour, John A L; David Brook, J

    2010-10-15

    Congenital heart defects (CHD) are collectively the most common form of congenital malformation. Studies of human cases and animal models have revealed that mutations in several genes are responsible for both familial and sporadic forms of CHD. We have previously shown that a mutation in MYH6 can cause an autosomal dominant form of atrial septal defect (ASD), whereas others have identified mutations of the same gene in patients with hypertrophic and dilated cardiomyopathy. In the present study, we report a mutation analysis of MYH6 in patients with a wide spectrum of sporadic CHD. The mutation analysis of MYH6 was performed in DNA samples from 470 cases of isolated CHD using denaturing high-performance liquid chromatography and sequence analysis to detect point mutations and small deletions or insertions, and multiplex amplifiable probe hybridization to detect partial or complete copy number variations. One non-sense mutation, one splicing site mutation and seven non-synonymous coding mutations were identified. Transfection of plasmids encoding mutant and non-mutant green fluorescent protein-MYH6 fusion proteins in mouse myoblasts revealed that the mutations A230P and A1366D significantly disrupt myofibril formation, whereas the H252Q mutation significantly enhances myofibril assembly in comparison with the non-mutant protein. Our data indicate that functional variants of MYH6 are associated with cardiac malformations in addition to ASD and provide a novel potential mechanism. Such phenotypic heterogeneity has been observed in other genes mutated in CHD.

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

    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.

  2. Ethnic and socioeconomic variation in incidence of congenital heart defects.

    Knowles, Rachel L; Ridout, Deborah; Crowe, Sonya; Bull, Catherine; Wray, Jo; Tregay, Jenifer; Franklin, Rodney C; Barron, David J; Cunningham, David; Parslow, Roger C; Brown, Katherine L

    2017-06-01

    Ethnic differences in the birth prevalence of congenital heart defects (CHDs) have been reported; however, studies of the contemporary UK population are lacking. We investigated ethnic variations in incidence of serious CHDs requiring cardiac intervention before 1 year of age. All infants who had a cardiac intervention in England and Wales between 1 January 2005 and 31 December 2010 were identified in the national congenital heart disease surgical audit and matched with paediatric intensive care admission records to create linked individual child records. Agreement in reporting of ethnic group by each audit was evaluated. For infants born 1 January 2006 to 31 December 2009, we calculated incidence rate ratios (IRRs) for CHDs by ethnicity and investigated age at intervention, antenatal diagnosis and area deprivation. We identified 5350 infants (2940 (55.0%) boys). Overall CHD incidence was significantly higher in Asian and Black ethnic groups compared with the White reference population (incidence rate ratios (IRR) (95% CIs): Asian 1.5 (1.4 to 1.7); Black 1.4 (1.3 to 1.6)); incidence of specific CHDs varied by ethnicity. No significant differences in age at intervention or antenatal diagnosis rates were identified but affected children from non-White ethnic groups were more likely to be living in deprived areas than White children. Significant ethnic variations exist in the incidence of CHDs, including for specific defects with high infant mortality. It is essential that healthcare provision mitigates ethnic disparity, including through timely identification of CHDs at screening, supporting parental choice and effective interventions. Future research should explore the factors underlying ethnic variation and impact on longer-term outcomes. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  3. Longitudinal evaluation of P-wave dispersion and P-wave maximum in children after transcatheter device closure of secundum atrial septal defect.

    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.

  4. 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.

    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.

  5. The application of transcatheter closure procedure in congenital heart diseases

    Guo Haoxue; Liu Shuyong; Jiang Rutong; Bai Hongcan; Wang Yanwei; Du Yuying; Yang Qiaoji; Qin Yongwen

    2003-01-01

    Objective: To explore and evaluate the value of transcatheter closure procedure in congenital heart diseases. Methods: Transcatheter closure was performed in 12 patients with congenital heart diseases including 6 ventricular septal defect (VSD), 4 atrial septal defect (ASD), 2 patent ductus arteriosus (PDA), by the Amplatzer occlusion device under local or general anesthesia. Results: The procedure was successful in all patients outcoming with the disappearance of cardiac murmur. All of them could get out of the bed within 6 to 12 hours postoperatively, and were discharged from hospital after 4 to 6 days. Conclusions: Transcatheter treatment of congenital cardiac defects by Amplatzer occlusion device is less traumatic, with good effect, simultaneously

  6. The application of transcatheter closure procedure in congenital heart diseases

    Haoxue, Guo; Shuyong, Liu; Rutong, Jiang; Hongcan, Bai; Yanwei, Wang; Yuying, Du; Qiaoji, Yang; Yongwen, Qin [Henan Provincial Corps Hospital, Chinese People' s Police Forces, Zhengzhou (China). Dept. of Surgery

    2003-10-01

    Objective: To explore and evaluate the value of transcatheter closure procedure in congenital heart diseases. Methods: Transcatheter closure was performed in 12 patients with congenital heart diseases including 6 ventricular septal defect (VSD), 4 atrial septal defect (ASD), 2 patent ductus arteriosus (PDA), by the Amplatzer occlusion device under local or general anesthesia. Results: The procedure was successful in all patients outcoming with the disappearance of cardiac murmur. All of them could get out of the bed within 6 to 12 hours postoperatively, and were discharged from hospital after 4 to 6 days. Conclusions: Transcatheter treatment of congenital cardiac defects by Amplatzer occlusion device is less traumatic, with good effect, simultaneously.

  7. A Novel Alpha Cardiac Actin (ACTC1 Mutation Mapping to a Domain in Close Contact with Myosin Heavy Chain Leads to a Variety of Congenital Heart Defects, Arrhythmia and Possibly Midline Defects.

    Céline Augière

    Full Text Available A Lebanese Maronite family presented with 13 relatives affected by various congenital heart defects (mainly atrial septal defects, conduction tissue anomalies and midline defects. No mutations were found in GATA4 and NKX2-5.A set of 399 poly(AC markers was used to perform a linkage analysis which peaked at a 2.98 lod score on the long arm of chromosome 15. The haplotype analysis delineated a 7.7 meganucleotides genomic interval which included the alpha-cardiac actin gene (ACTC1 among 36 other protein coding genes. A heterozygous missense mutation was found (c.251T>C, p.(Met84Thr in the ACTC1 gene which changed a methionine residue conserved up to yeast. This mutation was absent from 1000 genomes and exome variant server database but segregated perfectly in this family with the affection status. This mutation and 2 other ACTC1 mutations (p.(Glu101Lys and p.(Met125Val which result also in congenital heart defects are located in a region in close apposition to a myosin heavy chain head region by contrast to 3 other alpha-cardiac actin mutations (p.(Ala297Ser,p.(Asp313His and p.(Arg314His which result in diverse cardiomyopathies and are located in a totally different interaction surface.Alpha-cardiac actin mutations lead to congenital heart defects, cardiomyopathies and eventually midline defects. The consequence of an ACTC1 mutation may in part be dependent on the interaction surface between actin and myosin.

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

    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.

  9. Association between the European GWAS-identified susceptibility locus at chromosome 4p16 and the risk of atrial septal defect: a case-control study in Southwest China and a meta-analysis.

    Li Zhao

    Full Text Available Atrial septal defect (ASD is the third most frequent type of congenital heart anomaly, featuring shunting of blood between the two atria. Gene-environment interaction remains to be an acknowledged cause for ASD occurrence. A recent European genome-wide association study (GWAS of congenital heart disease (CHD identified 3 susceptibility SNPs at chromosome 4p16 associated with ASD: rs870142, rs16835979 and rs6824295. A Chinese-GWAS of CHD conducted in the corresponding period did not reveal the 3 susceptibility SNPs, but reported 2 different risk SNPs: rs2474937 and rs1531070. Therefore, we aimed to investigate the associations between the 3 European GWAS-identified susceptibility SNPs and ASD risk in the Han population in southwest China. Additionally, to increase the robustness of our current analysis, we conducted a meta-analysis combining published studies and our current case-control study. We performed association, linkage disequilibrium, and haplotype analysis among the 3 SNPs in 190 ASD cases and 225 age-, sex-, and ethnicity-matched healthy controls. Genotype and allele frequencies among the 3 SNPs showed statistically significant differences between the cases and controls. Our study found that individuals carrying the allele T of rs870142, the allele A of rs16835979, and the allele T of rs6824295 had a respective 50.1% (odds ratio (OR = 1.501, 95% confidence interval (CI = 1.122-2.009, PFDR-BH = 0.018, 48.5% (OR = 1.485, 95%CI = 1.109-1.987, PFDR-BH = 0.012, and 38.6% (OR = 1.386, 95%CI = 1.042-1.844, PFDR-BH = 0.025 increased risk to develop ASD than wild-type allele carriers in our study cohort. In the haplotype analysis, we identified a disease-risk haplotype (TAT (OR = 1.540, 95%CI = 1.030-2.380, PFDR-BH = 0.016. Our meta-analysis also showed that the investigated SNP was associated with ASD risk (combined OR (95%CI = 1.35 (1.24-1.46, P < 0.00001. Our study provides compelling evidence to motivate better understanding of the etiology

  10. Imaging techniques for visualizing and phenotyping congenital heart defects in murine models.

    Liu, Xiaoqin; Tobita, Kimimasa; Francis, Richard J B; Lo, Cecilia W

    2013-06-01

    Mouse model is ideal for investigating the genetic and developmental etiology of congenital heart disease. However, cardiovascular phenotyping for the precise diagnosis of structural heart defects in mice remain challenging. With rapid advances in imaging techniques, there are now high throughput phenotyping tools available for the diagnosis of structural heart defects. In this review, we discuss the efficacy of four different imaging modalities for congenital heart disease diagnosis in fetal/neonatal mice, including noninvasive fetal echocardiography, micro-computed tomography (micro-CT), micro-magnetic resonance imaging (micro-MRI), and episcopic fluorescence image capture (EFIC) histopathology. The experience we have gained in the use of these imaging modalities in a large-scale mouse mutagenesis screen have validated their efficacy for congenital heart defect diagnosis in the tiny hearts of fetal and newborn mice. These cutting edge phenotyping tools will be invaluable for furthering our understanding of the developmental etiology of congenital heart disease. Copyright © 2013 Wiley Periodicals, Inc.

  11. What’s new in genetics of congenital heart defects

    Maria Cristina Digilio

    2016-12-01

    Full Text Available Epidemiological studies, clinical observations and advances in molecular genetics are contributing to the understanding of the etiology of congenital heart defects (CHDs. Several phenotype-genotype correlation studies have suggested that specific morphogenetic mechanisms put in motion by genes can result in a specific cardiac phenotype. The use of new technologies has increased the possibility of identification of new genes and chromosomal loci in syndromic and non-syndromic CHDs. There are a number of methods available for genetic research studies of CHDs, including cytogenetic analysis, linkage and association studies, copy number variation (CNV and DNA micro-array analysis, and whole exome sequencing. The altered dosage of contigous genes included inside CNVs can produce new syndromic CHDs, so that several different new genomic conditions have been identified. These include duplication 22q11.2 syndrome, distal 22q11.2 deletion syndrome, deletion and duplication 1q21.1, deletion 1p36 syndrome. Molecular techniques as whole exome sequancing has lead to the identification of new genes for monogenic syndromes with CHD, as for example in Adams-Oliver, Noonan and Kabuki syndrome. The variable expressivity and reduced penetrance of CHDs in genetic syndromes is likely influenced by genetic factors, and several studies have been performed showing the involvement of modifier genes. It is not easy to define precisely the genetic defects underlying non-syndromic CHDs, due to the genetic and clinical heterogeneity of these malformations. Recent experimental studies have identified multiple CNVs contributing to non-syndromic CHD. The number of identified genes for non-syndromic CHDs is at this time limited and each of the identified gene has been shown to be implicated only in a small proportion of CHD. The application of new technologies to specific cases of CHD and pedigrees with familial recurrence and filtering genes mapping in CNV regions can probably

  12. Educational achievement among long-term survivors of congenital heart defects: a Danish population-based follow-up study

    Olsen, Morten; Hjortdal, Vibeke E.; Mortensen, Laust Hvas

    2011-01-01

    Congenital heart defect patients may experience neurodevelopmental impairment. We investigated their educational attainments from basic schooling to higher education.......Congenital heart defect patients may experience neurodevelopmental impairment. We investigated their educational attainments from basic schooling to higher education....

  13. Pediatric inpatient hospital resource use for congenital heart defects.

    Simeone, Regina M; Oster, Matthew E; Cassell, Cynthia H; Armour, Brian S; Gray, Darryl T; Honein, Margaret A

    2014-12-01

    Congenital heart defects (CHDs) occur in approximately 8 per 1000 live births. Improvements in detection and treatment have increased survival. Few national estimates of the healthcare costs for infants, children and adolescents with CHDs are available. We estimated hospital costs for hospitalizations using pediatric (0-20 years) hospital discharge data from the 2009 Healthcare Cost and Utilization Project Kids' Inpatient Database (KID) for hospitalizations with CHD diagnoses. Estimates were up-weighted to be nationally representative. Mean costs were compared by demographic factors and presence of critical CHDs (CCHDs). Up-weighting of the KID generated an estimated 4,461,615 pediatric hospitalizations nationwide, excluding normal newborn births. The 163,980 (3.7%) pediatric hospitalizations with CHDs accounted for approximately $5.6 billion in hospital costs, representing 15.1% of costs for all pediatric hospitalizations in 2009. Approximately 17% of CHD hospitalizations had a CCHD, but it varied by age: approximately 14% of hospitalizations of infants, 30% of hospitalizations of patients aged 1 to 10 years, and 25% of hospitalizations of patients aged 11 to 20 years. Mean costs of CHD hospitalizations were higher in infancy ($36,601) than at older ages and were higher for hospitalizations with a CCHD diagnosis ($52,899). Hospitalizations with CCHDs accounted for 26.7% of all costs for CHD hospitalizations, with hypoplastic left heart syndrome, coarctation of the aorta, and tetralogy of Fallot having the highest total costs. Hospitalizations for children with CHDs have disproportionately high hospital costs compared with other pediatric hospitalizations, and the 17% of hospitalizations with CCHD diagnoses accounted for 27% of CHD hospital costs. © 2014 Wiley Periodicals, Inc.

  14. Use of Non-Invasive Phase Contrast Magnetic Resonance Imaging for Estimation of Atrial Septal Defect Size and Morphology: A Comparison with Transesophageal Echo

    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

  15. Nasal septal hematoma

    ... 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 ...

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

    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

  17. Correção simultânea de defeito congênito intracardíaco e pectus excavatum Simultaneous repair of congenital heart defect and pectus excavatum

    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.

  18. 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

    Paulo Roberto B. Evora

    2004-12-01

    bypasses with less than 20 minutes of aortic cross-clamping. The causes of death were: non-occlusive intestinal ischemia, rupture of a cerebral aneurysm of the anterior communicating artery, cor pulmonale associated with pulmonary arterial hypertension and thromboembolism and probable cerebral thromboembolism in a child that needed to be reoperated to correct anomalous inferior vena cava drainage into the left atrium. CONCLUSION: Even being technically easy to treat, death due to atrial septal defects in extremely dramatic circumstances is possible, so "Do not underestimate the atrial septal in congenital heart surgery".

  19. Significance of MR imaging in congenital heart disease

    Mayr, H.; Globits, S.; Frank, H.; Glogar, D.; Nouhold, A.; Imhof, H.

    1989-01-01

    To determine the diagnostic impact of MR imaging in congenital heart disease, the authors used a 0.5- or 1.5-T magnet to examine 85 patients. Multisection spin-echo images were obtained in three planes. Diagnoses included atrial septal defect, trilogy and tetralogy of Fallot, ventricular septal defect, transposition (seven), single ventricle, and other complex disorders. Compared with other noninvasive techniques, MR imaging allowed a much better visualization of anatomic structures and the relationship of great vessels to shunt lesions in complex congenital heart disease. In 53 (63%) of 85 patients, MR imaging made a major contribution to establishing or modifying diagnoses

  20. Anatomic and functional imaging of congenital heart disease with digital subtraction angiography

    Buonocore, E.; Pavlicek, W.; Modic, M.T.; Meaney, T.F.; O'Donovan, P.B.; Grossman, L.B.; Moodie, D.S.; Yiannikas, J.

    1983-01-01

    Digital subtraction angiography (DSA) of the heart was performed in 54 patients for the evaluation of congenital heart diagnostic images and accurate physiologic shunt data that compared favorably with catheter angiography and nuclear medicine studies. Retrospective analysis of this series of patients indicated that DSA studies contributed sufficient informantion to shorten significantly or modify cardiac catheterization in 85% (79/93) of the defects that were identified. Interatrial septal defects were particularly well diagnosed, with identification occurring in 10 of 10 cases, wheseas intraventricular septal defects were identified in only 6 of 9 patients. Evaluation of postsurgical patients was accurate in 19 of 20 cases

  1. 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

    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

  2. Quantitative thallium-201 myocardial imaging in assessing right ventricular pressure in patients with congenital heart defects

    Rabinovitch, M.; Fischer, K.C.; Treves, S.

    1981-01-01

    Thallium-201 myocardial scintigraphy was performed in patients with congenital heart defects to determine whether, by quantification of right ventricular isotope uptake, one could assess the degree of right ventricular hypertrophy and so predict the level of right ventricular pressure. It is shown that quantitative analysis of myocardial imaging with thallium-201 is of use clinically in patients with congenital heart defects, in assessing the severity of pulmonary stenosis or the presence of pulmonary artery hypertension. (author)

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

    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.

  4. Usefulness of electrocardiography-gated dual-source computed tomography for evaluating morphological features of the ventricles in children with complex congenital heart defects

    Nakagawa, Motoo; Hara, Masaki; Sakurai, Keita; Asano, Miki; Shibamoto, Yuta; Ohashi, Kazuya

    2011-01-01

    Improved time resolution using dual-source computed tomography (DSCT) enabled adaptation of electrocardiography (ECG)-gated cardiac CT for children with a high heart rate. In this study, we evaluated the ability of ECG-gated DSCT (ECG-DSCT) to depict the morphological ventricular features in patients with congenital heart disease (CHD). Between August 2006 and March 2010, a total of 66 patients with CHD (aged 1 day to 9 years, median 11 months) were analyzed using ECG-DSCT. The type of anomaly was ventricular septal defect (VSD) in 32 (malaligned type in 20, perimembranous type in 7, supracristal type in 3, muscular type in 2), single ventricle (SV) in 11, and corrected transposition of the great arteries (cTGA) in 3. All patients underwent ECG-DSCT and ultrasonography (US). We evaluated the accuracy of diagnosing the type of VSD. For the cases with SV and cTGA, we evaluated the ability to depict anatomical ventricular features. In all 32 cases of VSD, DSCT could confirm the VSD defects, and the findings were identical to those obtained by US. Anatomical configurations of the SV and cTGA were correctly diagnosed, similar to that on US. Our study suggests that ECG-DSCT can clearly depict the configuration of ventricles. (author)

  5. Atrial septal defect as a cause of chronic cough and recurrent infections in a 4-year-old boy

    Piotr Fuss

    2017-09-01

    Full Text Available Recurrent infections of the respiratory system among children are the most common reason for ambulatory treatment and one of the main causes of hospitalisation. Out of many factors that can be potentially responsible for the recurrent infections in children it is necessary to consider the ones that are connected with the immaturity of the immune system of a child and ones that can disturb the proper functioning of this system. It seems that the most important observations are those pointing at links between recurrent infections of the respiratory tract and the allergic process. The present article describes a case of a boy with recurrent respiratory infections, who was diagnosed towards immune system malfunction and allergies. An echocardiographic examination revealed a major defect of the interatrial septum. This  heart condition was treated with cardiac surgery as a result of which infections occur less frequently and do not require the hospitalisation of the patient.

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

    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.

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

    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

  8. 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

    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.

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

    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.

  10. Investigation of Copy Number Variation in Children with Conotruncal Heart Defects

    Campos, Carla Marques Rondon; Zanardo, Evelin Aline; Dutra, Roberta Lelis; Kulikowski, Leslie Domenici; Kim, Chong Ae

    2015-01-01

    Congenital heart defects (CHD) are the most prevalent group of structural abnormalities at birth and one of the main causes of infant morbidity and mortality. Studies have shown a contribution of the copy number variation in the genesis of cardiac malformations. Investigate gene copy number variation (CNV) in children with conotruncal heart defect. Multiplex ligation-dependent probe amplification (MLPA) was performed in 39 patients with conotruncal heart defect. Clinical and laboratory assessments were conducted in all patients. The parents of the probands who presented abnormal findings were also investigated. Gene copy number variation was detected in 7/39 patients: 22q11.2 deletion, 22q11.2 duplication, 15q11.2 duplication, 20p12.2 duplication, 19p deletion, 15q and 8p23.2 duplication with 10p12.31 duplication. The clinical characteristics were consistent with those reported in the literature associated with the encountered microdeletion/microduplication. None of these changes was inherited from the parents. Our results demonstrate that the technique of MLPA is useful in the investigation of microdeletions and microduplications in conotruncal congenital heart defects. Early diagnosis of the copy number variation in patients with congenital heart defect assists in the prevention of morbidity and decreased mortality in these patients

  11. Investigation of Copy Number Variation in Children with Conotruncal Heart Defects

    Carla Marques Rondon Campos

    2015-01-01

    Full Text Available Background: Congenital heart defects (CHD are the most prevalent group of structural abnormalities at birth and one of the main causes of infant morbidity and mortality. Studies have shown a contribution of the copy number variation in the genesis of cardiac malformations. Objectives: Investigate gene copy number variation (CNV in children with conotruncal heart defect. Methods: Multiplex ligation-dependent probe amplification (MLPA was performed in 39 patients with conotruncal heart defect. Clinical and laboratory assessments were conducted in all patients. The parents of the probands who presented abnormal findings were also investigated. Results: Gene copy number variation was detected in 7/39 patients: 22q11.2 deletion, 22q11.2 duplication, 15q11.2 duplication, 20p12.2 duplication, 19p deletion, 15q and 8p23.2 duplication with 10p12.31 duplication. The clinical characteristics were consistent with those reported in the literature associated with the encountered microdeletion/microduplication. None of these changes was inherited from the parents. Conclusions: Our results demonstrate that the technique of MLPA is useful in the investigation of microdeletions and microduplications in conotruncal congenital heart defects. Early diagnosis of the copy number variation in patients with congenital heart defect assists in the prevention of morbidity and decreased mortality in these patients.

  12. Origins and consequences of congenital heart defects affecting the right ventricle.

    Woudstra, Odilia I; Ahuja, Suchit; Bokma, Jouke P; Bouma, Berto J; Mulder, Barbara J M; Christoffels, Vincent M

    2017-10-01

    Congenital heart disease is a major health issue, accounting for a third of all congenital defects. Improved early surgical management has led to a growing population of adults with congenital heart disease, including patients with defects affecting the right ventricle, which are often classified as severe. Defects affecting the right ventricle often cause right ventricular volume or pressure overload and affected patients are at high risk for complications such as heart failure and sudden death. Recent insights into the developmental mechanisms and distinct developmental origins of the left ventricle, right ventricle, and the outflow tract have shed light on the common features and distinct problems arising in specific defects. Here, we provide a comprehensive overview of the current knowledge on the development into the normal and congenitally malformed right heart and the clinical consequences of several congenital heart defects affecting the right ventricle. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.

  13. Investigation of Copy Number Variation in Children with Conotruncal Heart Defects

    Campos, Carla Marques Rondon, E-mail: carlamcampos@uol.com.br [Universidade Federal de Mato Grosso, Cuiabá, MT (Brazil); Zanardo, Evelin Aline; Dutra, Roberta Lelis [Departamento de Patologia - Laboratório de Citogenômica - LIM 03 - Universidade de São Paulo, São Paulo, SP (Brazil); Kulikowski, Leslie Domenici [Universidade de São Paulo, São Paulo, SP (Brazil); Departamento de Patologia - Laboratório de Citogenômica - LIM 03 - Universidade de São Paulo, São Paulo, SP (Brazil); Kim, Chong Ae [Universidade de São Paulo, São Paulo, SP (Brazil)

    2015-01-15

    Congenital heart defects (CHD) are the most prevalent group of structural abnormalities at birth and one of the main causes of infant morbidity and mortality. Studies have shown a contribution of the copy number variation in the genesis of cardiac malformations. Investigate gene copy number variation (CNV) in children with conotruncal heart defect. Multiplex ligation-dependent probe amplification (MLPA) was performed in 39 patients with conotruncal heart defect. Clinical and laboratory assessments were conducted in all patients. The parents of the probands who presented abnormal findings were also investigated. Gene copy number variation was detected in 7/39 patients: 22q11.2 deletion, 22q11.2 duplication, 15q11.2 duplication, 20p12.2 duplication, 19p deletion, 15q and 8p23.2 duplication with 10p12.31 duplication. The clinical characteristics were consistent with those reported in the literature associated with the encountered microdeletion/microduplication. None of these changes was inherited from the parents. Our results demonstrate that the technique of MLPA is useful in the investigation of microdeletions and microduplications in conotruncal congenital heart defects. Early diagnosis of the copy number variation in patients with congenital heart defect assists in the prevention of morbidity and decreased mortality in these patients.

  14. Ventricular Septal Dissection Complicating Inferior Wall Myocardial Infarction

    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.

  15. A systematic review of trends and patterns of congenital heart disease in children in Nigeria from 1964-2015.

    Abdulkadir, Mohammed; Abdulkadir, Zainab

    2016-06-01

    Congenital heart diseases cause significant childhood morbidity and mortality. Several restricted studies have been conducted on the epidemiology in Nigeria. No truly nationwide data on patterns of congenital heart disease exists. To determine the patterns of congenital heart disease in children in Nigeria and examine trends in the occurrence of individual defects across 5 decades. We searched PubMed database, Google scholar, TRIP database, World Health Organisation libraries and reference lists of selected articles for studies on patterns of congenital heart disease among children in Nigeria between 1964 and 2015. Two researchers reviewed the papers independently and extracted the data. Seventeen studies were selected that included 2,953 children with congenital heart disease. The commonest congenital heart diseases in Nigeria are ventricular septal defect (40.6%), patent ductus arteriosus (18.4%), atrial septal defect (11.3%) and tetralogy of Fallot (11.8%). There has been a 6% increase in the burden of VSD in every decade for the 5 decades studied and a decline in the occurrence of pulmonary stenosis. Studies conducted in Northern Nigeria demonstrated higher proportions of atrial septal defects than patent ductus arteriosus. Ventricular septal defects are the commonest congenital heart diseases in Nigeria with a rising burden.

  16. Prevalence of congenital heart defects among 54 Egyptian children ...

    Alyaa A. Kotby

    2017-10-19

    Oct 19, 2017 ... metabolic defect results in accumulation of branched-chain amino acids (BCAAs) (leucine, isoleucine and valine) and their keto acids [1]. Amino acids are key nutrient molecules essential for cell growth, survival, and normal function. In addition to providing building blocks for protein synthesis, many amino ...

  17. The helical ventricular myocardial band of Torrent-Guasp: potential implications in congenital heart defects.

    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.

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

    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.

  19. Ventricular septal necrosis after blunt chest trauma

    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.

  20. Congenital Anorectal Malformation Severity Does Not Predict Severity of Congenital Heart Defects

    Jonker, Jara E.; Liem, Eryn T.; Elzenga, Nynke J.; Molenbuur, Bouwe; Trzpis, Monika; Broens, Paul M. A.

    2016-01-01

    Objective To determine the prevalence of congenital heart defects (CHDs) in patients with mild or severe congenital anorectal malformations (CARMs), and whether all patients with CARM need pediatric cardiology screening. Study design We included 129 patients with CARM born between 2004 and 2013, and

  1. Familial co-occurrence of congenital heart defects follows distinct patterns

    Ellesøe, Sabrina G.; Workman, Christopher T.; Bouvagnet, Patrice; Loffredo, Christopher A.; McBride, Kim L.; Hinton, Robert B.; van Engelen, Klaartje; Gertsen, Emma C.; Mulder, Barbara J. M.; Postma, Alex V.; Anderson, Robert H.; Hjortdal, Vibeke E.; Brunak, Søren; Larsen, Lars A.

    2017-01-01

    Congenital heart defects (CHD) affect almost 1% of all live born children and the number of adults with CHD is increasing. In families where CHD has occurred previously, estimates of recurrence risk, and the type of recurring malformation are important for counselling and clinical decision-making,

  2. Dietary intake of B-vitamins in mothers born a child with a congenital heart defect

    Verkleij-Hagoort, A.C.; Vries, de J.H.M.; Ursem, N.T.C.; Jonge, de R.; Hop, W.C.J.; Steegers-Theunissen, R.P.M.

    2006-01-01

    BACKGROUND: Periconceptional use of multivitamins reduces the risk of a child with a congenital heart defect (CHD). Data on the impact of maternal diet, however, are lacking. AIM OF THE STUDY: We investigated the association between the maternal dietary intake of B-vitamins and having a child with a

  3. Usefulness of cutting balloon angioplasty for the treatment of congenital heart defects.

    Kusa, Jacek; Mazurak, Magdalena; Skierska, Agnieszka; Szydlowski, Leslaw; Czesniewicz, Pawel; Manka, Lukasz

    2018-01-01

    Patients with complex congenital heart defects may have different hemodynamic prob-lems which require a variety of interventional procedures including angioplasty which involves using high-pressure balloons. After failure of conventional balloon angioplasty, cutting balloon angioplasty is the next treatment option available. The purpose of this study was to evaluate the safety and efficacy of cutting balloon angioplasty in children with different types of congenital heart defects. Cutting balloon angioplasty was performed in 28 children with different congenital heart defects. The indication for cutting balloon angioplasty was: pulmonary artery stenosis in 17 patients, creating or dilatation of interatrial communication in 10 patients, and stenosis of left subclavian artery in 1 patient. In the pulmonary arteries group there was a significant decrease in systolic blood pressure (SBP) in the proximal part of the artery from the average 74.33 ± 20.4 mm Hg to 55 ± 16.7 mm Hg (p cutting balloon angioplasty was performed after an unsuccessful classic Rashkind procedure. After cutting balloon angioplasty there was a significant widening of the interatrial communication. Cutting balloon angioplasty is a feasible and effective treatment option in different con-genital heart defects.

  4. Ultrasonographic views for the screening of congenital heart defects in the first level of care

    Garcia Guevara, Carlos; Arenciabia Faife, Jakeline; Ley Vega, Lisset

    2009-01-01

    Congenital heart diseases are the main cause of infant mortality for congenital malformations in our country and they are the defects that more usually escape diagnosis in ultrasonographic screening, especially if we consider that associated risk factors call for a fetal echocardiogram are not identified in most pregnant women with fetuses affected with a heart disease. With this paper, we intend to bring within reach of both the specialists dedicated to this activity in primary care and the Masters in Genetic Counseling a review article about the principal aspects to be evaluated in each of the three echocardiography views that are used in Cuba as part of screening these defects, as well as the main signs of suspicion of congenital heart diseases that give reason for having a pregnant woman referred to the immediately higher level of care

  5. Obesity and Cardiometabolic Defects in Heart Failure Pathology.

    Halade, Ganesh V; Kain, Vasundhara

    2017-09-12

    Obesity is a major global epidemic that sets the stage for diverse multiple pathologies, including cardiovascular disease. The obesity-related low-grade chronic inflamed milieu is more pronounced in aging and responsive to cardiac dysfunction in heart failure pathology. Metabolic dysregulation of obesity integrates with immune reservoir in spleen and kidney network. Therefore, an integrative systems biology approach is necessary to delay progressive cardiac alternations. The purpose of this comprehensive review is to largely discuss the impact of obesity on the cardiovascular pathobiology in the context of problems and challenges, with major emphasis on the diversified models, and to study cardiac remodeling in obesity. The information in this article is immensely helpful in teaching advanced undergraduate, graduate, and medical students about the advancement and impact of obesity on cardiovascular health. © 2017 American Physiological Society. Compr Physiol 7:1463-1477, 2017. Copyright © 2017 John Wiley & Sons, Inc.

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

    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.

  7. Increased risk for congenital heart defects in children carrying the ABCB1 Gene C3435T polymorphism and maternal periconceptional toxicants exposure.

    Chuan Wang

    Full Text Available BACKGROUNDS: The etiology of congenital heart defect (CHD is commonly believed to involve the interaction of multiple environmental and genetic factors. This study aimed to explore the joint effects of the ABCB1 gene C3435T polymorphism and maternal periconceptional toxicants exposure on the CHD risk in a Han Chinese population. METHODS: An age and gender matched case-control study with standardized data collection involving 201 pairs was conducted. Periconceptional toxicants exposure was obtained through a structured questionnaire. A job exposure matrix (JEM was used for toxicants exposure assessment. Genotyping of the ABCB1 C3435T polymorphism was performed by sequencing. Logistic regression analysis was performed to assess the joint effects of the ABCB1 gene C3435T polymorphism and toxicants exposure on the risk of CHD. Placenta tissues and umbilical cords were collected to investigate the impact of C3435T polymorphism on the transcription and translation activities of ABCB1 gene. RESULTS: MATERNAL PERICONCEPTIONAL EXPOSURES TO PHTHALATES (ADJUSTED OR: 1.6; 95%CI: 1.0-2.6 and alkylphenolic compounds (adjusted OR:1.8; 95%CI:1.1-3.0 were associated with a higher incidence of CHDs in general. More cases were carriers of the ABCB1 CC/CT genotypes (OR: 2.0, 95%CI: 1.1-3.5, P-value: 0.021. Children carrying the CC/CT genotype and periconceptionally exposed to phthalates and alkylphenolic compounds suffered almost 3.5-fold increased risk of having CHD than non-exposed children with TT genotype (adjusted OR: 3.5, 95%CI: 1.5-7.9, P-value: 0.003, and the OR changed to 4.4 for septal defects (adjusted OR: 4.4,95%CI:1.8-10.9,P-value:0.001. The ABCB1 mRNA expression of the TT genotype was significantly higher than that of the CC genotype (P = 0.03. Compared with TT genotype, lower P-glycoprotein expression was observed for the CC/CT genotypes. CONCLUSION: The C3435T polymorphism in the ABCB1 gene of fetus increases the risks of CHD in a Han Chinese

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

    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.

  9. 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

    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

  10. Population-based evaluation of a suggested anatomic and clinical classification of congenital heart defects based on the International Paediatric and Congenital Cardiac Code

    Goffinet François

    2011-10-01

    Full Text Available Abstract Background Classification of the overall spectrum of congenital heart defects (CHD has always been challenging, in part because of the diversity of the cardiac phenotypes, but also because of the oft-complex associations. The purpose of our study was to establish a comprehensive and easy-to-use classification of CHD for clinical and epidemiological studies based on the long list of the International Paediatric and Congenital Cardiac Code (IPCCC. Methods We coded each individual malformation using six-digit codes from the long list of IPCCC. We then regrouped all lesions into 10 categories and 23 subcategories according to a multi-dimensional approach encompassing anatomic, diagnostic and therapeutic criteria. This anatomic and clinical classification of congenital heart disease (ACC-CHD was then applied to data acquired from a population-based cohort of patients with CHD in France, made up of 2867 cases (82% live births, 1.8% stillbirths and 16.2% pregnancy terminations. Results The majority of cases (79.5% could be identified with a single IPCCC code. The category "Heterotaxy, including isomerism and mirror-imagery" was the only one that typically required more than one code for identification of cases. The two largest categories were "ventricular septal defects" (52% and "anomalies of the outflow tracts and arterial valves" (20% of cases. Conclusion Our proposed classification is not new, but rather a regrouping of the known spectrum of CHD into a manageable number of categories based on anatomic and clinical criteria. The classification is designed to use the code numbers of the long list of IPCCC but can accommodate ICD-10 codes. Its exhaustiveness, simplicity, and anatomic basis make it useful for clinical and epidemiologic studies, including those aimed at assessment of risk factors and outcomes.

  11. Short-term outcomes of transcatheter closure of secundum atrial septal defect in children and adolescents: An experience of two centers in Upper Egypt

    Safaa H. Ali

    2018-01-01

    Conclusions: Transcatheter closure of ASDs in children and adolescents was feasible and safe in the first 4 years experience in our centers, with good short-term outcome. Balloon sizing is not necessary for transcatheter closure of secundum ASD. Multiple defects can be safety closed by a single device.

  12. During childhood unrecognized congenital heart defect in patient with Turner syndrome, and its implications

    Klaskova, E.; Kapralova, S.; Zapletalova, J.; Tuedoes, Z.

    2015-01-01

    Congenital heart disease affects approximately 50 % of individuals with Turner syndrome (TS). Bicuspid aortic valve, aortic coarctation, ascending aorta dilatation and arterial hypertension are important risk factors for life-threatening aortic dissection or rupture. Authors discuss the importance of a careful cardiac examination including cardiac magnetic resonance imaging study and life-long follow-up by experienced cardiologist in TS patients, and point out high maternal mortality and morbidity during pregnancy. They present a case report of woman with TS and the above-mentioned in childhood unrecognized congenital heart defects that underwent infertility treatment without pre conceptional counselling focused on cardiovascular risk for aortic dissection. (author)

  13. Differential clinical characteristics and prognosis of intraventricular conduction defects in patients with chronic heart failure

    Cinca, Juan; Mendez, Ana; Puig, Teresa; Ferrero, Andreu; Roig, Eulalia; Vazquez, Rafael; Gonzalez-Juanatey, Jose R.; Alonso-Pulpon, Luis; Delgado, Juan; Brugada, Josep; Pascual-Figal, Domingo; Brugada, J.; Batlle, M.; Berruezo, A.; Hevia, S.

    2013-01-01

    Aims Intraventricular conduction defects (IVCDs) can impair prognosis of heart failure (HF), but their specific impact is not well established. This study aimed to analyse the clinical profile and outcomes of HF patients with LBBB, right bundle branch block (RBBB), left anterior fascicular block (LAFB), and no IVCDs. Methods and results Clinical variables and outcomes after a median follow-up of 21 months were analysed in 1762 patients with chronic HF and LBBB (n = 532), RBBB (n = 134), LAFB ...

  14. Cirurgia valvar mitral e da comunicação interatrial: abordagem minimamente invasiva ou por esternotomia Mitral valve and atrial septal defect surgery: minimally invasive or sternotomy approach

    Josué V. Castro Neto

    2012-08-01

    atrial septal defect (ASD. METHODS: Forty patients underwent surgery for correction of MV disease or ASD. Patients were divided into group A (GA (n=20, access by right minithoracotomy and video-assistance; and group B (GB (n=20, access by full MS. Aortic cross-clamp and cardiopulmonary bypass time, intensive care unit (ICU time, hospital stay and morbidity were compared in this prospective study . RESULTS: Fifteen patients were submitted to MV procedures and five to ASD corrections in each group. There were 9 mitral replacements (7 bioprostetic and 2 mechanical and 6 repairs in GA, and 10 (all bioprostetic and five in GB. The mean aortic cross-clamp and cardiopulmonary bypass time, in minutes, were 65.1 ± 29.3 in GA and 50.2 ± 21.4 in GB (p=0.074; and 91.8±35 in GA and 63.7±27.3 in GB ( p=0.008. The mean ICU time, in hours, were 51.7 ± 16.3 in GA and 55.8±17.5 in GB (p=0.45. The in hospital stay, in days, were 5.2 ± 1 in GA and 6.4±1.5 in GB (p=0.009. CONCLUSION: MI access for correction of the MV disease and ASD implicated in a longer cardiopulmonary bypass time for finalization of the main procedure, nevertheless it didn´t affect patient's recuperation. MI treated patients were discharged earlier than sternotomy treated patients.

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

    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.

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

    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.

  17. Risk factors for congenital heart diseases in Alexandria, Egypt

    Bassili, A.; Mokhtar, S.A.; Dabous, N.I.; Zaher, S.R.; Mokhtar, M.M.; Zaki, A.

    2000-01-01

    A matched case control study has been conducted in the children's hospitals in Alexandria, Egypt, during 2 years-period, aiming at investigating the risk factors for the occurrence of congenital heart diseases. Our results showed that the significant risk factors for developing any type of congenital heart disease and ventricular septal defects were: older paternal age at birth, positive consanguinity, positive family history, female sex hormones, irradiation, hazardous maternal occupation, diabetes mellitus and suburban or rural residence. However, some environmental/teratogenic factors were not implicated in the etiology of atrial septal defects or pulmonary stenosis. These findings strongly suggest that environmental factors vary according to the specific type of congenital heart disease. This study emphasizes on the need to instruct the public about the importance of pre-marital counselling and the deleterious effects of various teratogens in the environment

  18. MRI of the heart, the great vessels and the mediastinum

    Obletter, N.; Picker, D.; Stirner, H.; Schmitt, R.; Helmberger, T.

    1994-01-01

    The article surveys the most important indications for MRT of the heart, the great vessels and the mediastinum. MRT is clearly indicated with search or clarification of aneurysms of the heart wall, atrial or ventriucular septal defects with shunting or other congenital vitia in children or in the newborn, aortic aneurysms as well as connatal defects. MRT is superior to other screening methods when clarifying mediastinal neoplasms because of its multiplanar presentation and its strong quality in contrasting soft tissue. With MRT impressive dynamic studies can be obtained through cine modes and gradient echo sequences, which are very successfully used for aortic and mitral valve stenoses, for aneurysms of the heart wall, for aortic aneurysms and atrial and septal shunts. (orig./MG) [de

  19. Congenital heart defects in newborns with apparently isolated single gastrointestinal malformation: A retrospective study.

    Schierz, Ingrid Anne Mandy; Pinello, Giuseppa; Giuffrè, Mario; La Placa, Simona; Piro, Ettore; Corsello, Giovanni

    2016-12-01

    Congenital gastrointestinal system malformations/abdominal wall defects (GISM) may appear as isolated defects (single or complex), or in association with multiple malformations. The high incidence of association of GISM and congenital heart defects (CHD) in patients with syndromes and malformative sequences is known, but less expected is the association of apparently isolated single GISM and CHD. The aim of this study was to investigate the frequency of CHD in newborns with isolated GISM, and the possibility to modify the diagnostic-therapeutic approach just before the onset of cardiac symptoms or complications. Anamnestic, clinical, and imaging data of newborns requiring abdominal surgery for GISM, between 2009 and 2014, were compared with a control group of healthy newborns. Distribution of GISM and cardiovascular abnormalities were analyzed, and risk factors for adverse outcomes were identified. Seventy-one newborns with isolated GISM were included in this study. More frequent GISM were intestinal rotation and fixation disorders. CHD were observed in 15.5% of patients, augmenting their risk for morbidity. Risk factors for morbidity related to sepsis were identified in central venous catheter, intestinal stoma, and H2-inhibitor-drugs. Moreover, 28.2% of newborns presented only functional cardiac disorders but an unexpectedly higher mortality. The high incidence of congenital heart disease in infants with apparently isolated GISM confirms the need to perform an echocardiographic study before surgery to improve perioperative management and prevent complications such as sepsis and endocarditis. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  20. The ubiquitin-proteasome system and autophagy are defective in the taurine-deficient heart.

    Jong, Chian Ju; Ito, Takashi; Schaffer, Stephen W

    2015-12-01

    Taurine depletion leads to impaired mitochondrial function, as characterized by reduced ATP production and elevated superoxide generation. These defects can fundamentally alter cardiomyocyte function and if left unchanged can result in cell death. To protect against these stresses, cardiomyocytes possess quality control processes, such as the ubiquitin-proteasome system (UPS) and autophagy, which can rejuvenate cells through the degradation of damaged proteins and organelles. Hence, the present study tested the hypothesis that reactive oxygen species generated by damaged mitochondria initiates UPS and autophagy in the taurine-deficient heart. Using transgenic mice lacking the taurine transporter (TauTKO) as a model of taurine deficiency, it was shown that the levels of ubiquitinated protein were elevated, an effect associated with a decrease in ATP-dependent 26S β5 proteasome activity. Treating the TauTKO mouse with the mitochondria-specific antioxidant, mitoTEMPO, largely abolished the increase in ubiquitinated protein content. The TauTKO heart was also associated with impaired autophagy, characterized by an increase in the initiator, Beclin-1, and autophagosome content, but a defect in the generation of active autophagolysosomes. Although mitoTEMPO treatment only restores the oxidative balance within the mitochondria, it appeared to completely disrupt the crosstalk between the damaged mitochondria and the quality control processes. Thus, mitochondrial oxidative stress is the main trigger initiating the quality control systems in the taurine-deficient heart. We conclude that the activation of the UPS and autophagy is another fundamental function of mitochondria.

  1. Anuloplastia de homoenxerto pulmonar criopreservado com anel de Delrin na atresia pulmonar com comunicação interventricular Annuloplasty of cryopreserved pulmonary homograft with Delrin stent in pulmonary atresia with ventricular septal defect

    Ulisses Alexandre Croti

    2007-05-01

    Full Text Available Criança de seis anos portadora de atresia pulmonar com comunicação interventricular, submetida a correção total com um ano, empregando monoválvula de pericárdio bovino na reconstrução da via de saída do ventrículo direito. Evoluiu com importante regurgitação valvar pulmonar (RVP e disfunção do ventrículo direito. Na reoperação foi implantado homoenxerto pulmonar criopreservado (HPC com anuloplastia, utilizando anel de Delrin com o intuito de evitar distorção geométrica do conduto. Após dois anos, o ecocardiograma, semelhante ao pós-operatório imediato, demonstra RVP discreta e função ventricular direita normal, sugerindo que essa manobra pode ser utilizada como coadjuvante para otimizar o resultado do implante do HPC.A six-year-old child was suffering from pulmonary atresia with ventricular septal defect after a total correction at 1 year of age using a bovine pericardial monocusp valve in the reconstruction of the right ventricle outflow tract. The infant evolved with significant pulmonary valve regurgitation (PVR and right ventricle dysfunction. On reoperation, a cryopreserved pulmonary homograft (CPH was implanted with annuloplasty utilizing a Delrin ring with the aim of avoiding geometric distortion of the vessel. After two years, an echocardiogram proved a similar state to the immediate postoperative period with slight pulmonary valve regurgitation and normal right ventricular function suggesting that this maneuver may be used as coadjuvant treatment to optimize the result of CPH implantation.

  2. 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®.

    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.

  3. Association between left ventricular perfusion defects and myocardial deformation indexes in heart transplantation recipients.

    D'Andrea, Antonello; De Rimini, Maria Luisa; America, Raffaella; Cirillo, Chiara; Riegler, Lucia; Limongelli, Giuseppe; D'Alto, Michele; Salerno, Gemma; Maiello, Ciro; Muto, Pietro; Russo, Maria Giovanna; Calabrò, Raffaele; Bossone, Eduardo; Pacileo, Giuseppe

    2017-10-01

    The aim of the study was to analyze possible correlations between strain echocardiography (STE) and PET myocardial perfusion in a population of heart transplantation (HTx) recipients showing preserved left ventricular (LV) ejection fraction. By STE, LV global longitudinal strain (LV GLS) was lower in HTx. PET showed no transient or chronic ischemia in 83 of 115 HTx (73%). Fixed perfusion defects were observed in 17% of HTx and reversible ischemia in 10%. Significant coronary stenosis was observed only in 10 cases. GLS was independently associated with age at HTx and fixed perfusion defects (HR 0.41; P<.001). Such relationships underline STE ability to early identify HTx pts with subclinical myocardial dysfunction during long-term follow-up. © 2017, Wiley Periodicals, Inc.

  4. Congenital heart defects: the 10-year experience at a single center.

    Aydin, Emine; Aypar, Ebru; Oktem, Ahmet; Ozyuncu, Ozgur; Yurdakok, Murat; Guvener, Murat; Demircin, Metin; Beksac, M Sinan

    2018-06-18

    We aimed to evaluate congenital heart disease (CHD) cases according to EUROCAT subgroup classification that were diagnosed during the prenatal period in our center. CHDs that were prenatally diagnosed using ultrasonography and confirmed by fetal echocardiography were reviewed over a 10-year period. Subgroup classification was finalized at the post-partum period in terms of the EUROCAT guide 1.3. Congenital heart defect subtypes and obstetric outcomes (gestational week at delivery, birth weight, gender, extracardiac structural abnormalities, karyotype results if performed) were analyzed. The data of 180 cases with CHD was examined. Left ventricular outflow tract obstruction (LVOT) was the most common CHD subtype (57/180; 31.6%), which included 48, five, and four cases of hypoplastic left heart syndrome (HLHS), coarctation of the aorta, and aortic valve atresia/stenosis, respectively. Eighteen pregnancies were terminated; the most common CHD subtype among patients of terminated pregnancies was hypoplastic left heart syndrome (HLHS) (n = 7, 38.8%). The most common extracardiac malformations were a single umbilical artery, esophageal atresia, and situs inversus in our study group. Eighteen of the 96 (18.75%) neonates with CHD died during the neonatal period. The most common CHD subtype was HLHS (7/18; 38%) among the newborns who died after birth. Prenatal diagnosis of a CHD and subgroup classification is very important for clinical decision making, including prenatal management, recommendations for termination of the pregnancy, postnatal management of the patient, and for early referral to pediatric cardiology and cardiovascular surgery centers.

  5. [Percutaneous catheter-based implantation of artificial pulmonary valves in patients with congenital heart defects].

    Wyller, Vegard Bruun; Aaberge, Lars; Thaulow, Erik; Døhlen, Gaute

    2011-07-01

    Percutaneous catheter-based implantation of artificial heart valves is a new technique that may supplement surgery and which may be used more in the future. We here report our first experience with implantation of artificial pulmonary valves in children with congenital heart defects. Eligible patients were those with symptoms of heart failure combined with stenosis and/or insufficiency in an established artificial right ventricular outflow tract. The valve was inserted through a catheter from a vein in the groin or neck. Symptoms, echocardiography, invasive measurements and angiography were assessed for evaluation of treatment effect. Our treatment results are reported for the period April 2007-September 2009. Ten patients (seven men and three women, median age 17 years) were assessed. The procedure reduced pressure in the right ventricle (p = 0.008) and resolved the pulmonary insufficiency in all patients. The median time in hospital was two days. No patients had complications that were directly associated with the implantation procedure. One patient developed a pseudoaneurysm in the femoral artery, another had a short-lasting fever two days after the procedure and one patient experienced a stent fracture that required surgery 9 months after the implantation. After 6 months all patients had a reduced pressure gradient in the right ventricular outflow tract (p = 0.008), the pulmonary insufficiency had improved (p = 0.006) and they all reported improval of symptoms. These results persisted for at least 24 months for the four patients who were monitored until then. Percutaneous catheter-based implantation of artificial pulmonary valves improves hemodynamics in the right ventricle of selected patients with congenital heart defects. A randomized controlled study should be undertaken to provide a stronger evidence-base for usefulness of this procedure.

  6. Genetics of Congenital Heart Disease: Past and Present.

    Muntean, Iolanda; Togănel, Rodica; Benedek, Theodora

    2017-04-01

    Congenital heart disease is the most common congenital anomaly, representing an important cause of infant morbidity and mortality. Congenital heart disease represents a group of heart anomalies that include septal defects, valve defects, and outflow tract anomalies. The exact genetic, epigenetic, or environmental basis of congenital heart disease remains poorly understood, although the exact mechanism is likely multifactorial. However, the development of new technologies including copy number variants, single-nucleotide polymorphism, next-generation sequencing are accelerating the detection of genetic causes of heart anomalies. Recent studies suggest a role of small non-coding RNAs, micro RNA, in congenital heart disease. The recently described epigenetic factors have also been found to contribute to cardiac morphogenesis. In this review, we present past and recent genetic discoveries in congenital heart disease.

  7. Endocardial cushion defect

    ... 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- ...

  8. TWO-STAGE SURGICAL TREATMENT OF A CHILD OF ONE YEAR FROM CONGENITAL HEART DISEASE AND BILIARY CIRRHOSIS

    S. V. Gautier

    2014-01-01

    Full Text Available Aim: Clinical case of successful two-stage surgical treatment of a 1-year-old child with congenital heart disease and biliary cirrhosis is represented in this article. At the first day of life laparotomy was performed because of high intestinal obstruction. Kasai procedure and Roux-en-Y choledochojejunostomy were per- formed on 12th day and at the end of second month of life, respectively. Liver biopsy showed the signs of biliary cirrhosis. At the same time ventricular septal defect and atrial septal defect with pulmonary hyper- tension were diagnosed. The first step of treatment was the surgical septal defects closure. No complications during procedure, cardiopulmonary bypass and post-operative period were registered. There were no nega- tive effects on liver function after cardiac surgery. 11 months later living-donor liver transplantation was performed without any complications. Patient was discharged at 35th post-transplant day with stable graft function. 

  9. DGCR6 at the proximal part of the DiGeorge critical region is involved in conotruncal heart defects

    Gao, Wenming; Higaki, Takashi; Eguchi-Ishimae, Minenori; Iwabuki, Hidehiko; Wu, Zhouying; Yamamoto, Eiichi; Takata, Hidemi; Ohta, Masaaki; Imoto, Issei; Ishii, Eiichi; Eguchi, Mariko

    2015-01-01

    Cardiac anomaly is one of the hallmarks of DiGeorge syndrome (DGS), observed in approximately 80% of patients. It often shows a characteristic morphology, termed as conotruncal heart defects. In many cases showing only the conotruncal heart defect, deletion of 22q11.2 region cannot be detected by fluorescence in situ hybridization (FISH), which is used to detect deletion in DGS. We investigated the presence of genomic aberrations in six patients with congenital conotruncal heart defects, who show no deletion at 22q11.2 in an initial screening by FISH. In these patients, no abnormalities were identified in the coding region of the TBX1 gene, one of the key genes responsible for the phenotype of DGS. However, when copy number alteration was analyzed by high-resolution array analysis, a small deletion or duplication in the proximal end of DiGeorge critical region was detected in two patients. The affected region contains the DGCR6 and PRODH genes. DGCR6 has been reported to affect the expression of the TBX1 gene. Our results suggest that altered dosage of gene(s) other than TBX1, possibly DGCR6, may also be responsible for the development of conotruncal heart defects observed in patients with DGS and, in particular, in those with stand-alone conotruncal heart defects. PMID:27081520

  10. Evaluation of Pulmonary Perfusion Scan in Heart Disease

    Lee, J T; Kim, C K; Park, C Y; Choi, B S [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    1973-09-15

    Pulmonary perfusion scan with radioactive {sup 113m}In-iron hydroxide particle was performed in the 25 cases of heart disease which had been diagnosed by cardiac catheterization prior to surgery from July, 1972 to July, 1973 at the Department of Radiology and Nuclear Medicine, Yonsei Medical College. It consists of 7 mitral stenosis, 2 mitral insufficiency, 1 aortic insufficiency, 3 atrial septal defect, 5 ventricular septal defect, 2 patent ductus arteriosus, 1 transposition of great vessel and 4 Tetralogy of Fallot. Findings of pulmonary perfusion scan in relation to hemodynamic data of cardiac catheterization were examined. 1) Out of 10 cases of acquired valvular heart disease, In 6 cases of stenosis and 1 case of aortic insufficiency, radioactivity was increased at both upper lung. This finding is noted when pulmonary wedge or venous pressure was elevated above 22 mmHg and arterial systolic pressure above 33 mmHg. 2) Out of 15 cases of congenital heart disease. In almost all cases of artial septal defect and ventricular septal defect except 2 cases, radioactivity was even at both entire lung. In 2 cases of patent ductras arteriosus, radioactivity was decreased especially at the left lung. It is observed that in acyanotic congenital heart disease, radioactivity of lung is not related with pulmonary arterial pressure. In 3 cases of Tetralogy of Fallot, radioactivity was even at both entire lung and in 2 of them, extrapulmonary radioactivity of liver or kidney which depends on size of defect and volume of right to left shunt reversible, was noted.

  11. Evaluation of Pulmonary Perfusion Scan in Heart Disease

    Lee, J. T.; Kim, C. K.; Park, C. Y.; Choi, B. S.

    1973-01-01

    Pulmonary perfusion scan with radioactive 113m In-iron hydroxide particle was performed in the 25 cases of heart disease which had been diagnosed by cardiac catheterization prior to surgery from July, 1972 to July, 1973 at the Department of Radiology and Nuclear Medicine, Yonsei Medical College. It consists of 7 mitral stenosis, 2 mitral insufficiency, 1 aortic insufficiency, 3 atrial septal defect, 5 ventricular septal defect, 2 patent ductus arteriosus, 1 transposition of great vessel and 4 Tetralogy of Fallot. Findings of pulmonary perfusion scan in relation to hemodynamic data of cardiac catheterization were examined. 1) Out of 10 cases of acquired valvular heart disease, In 6 cases of stenosis and 1 case of aortic insufficiency, radioactivity was increased at both upper lung. This finding is noted when pulmonary wedge or venous pressure was elevated above 22 mmHg and arterial systolic pressure above 33 mmHg. 2) Out of 15 cases of congenital heart disease. In almost all cases of artial septal defect and ventricular septal defect except 2 cases, radioactivity was even at both entire lung. In 2 cases of patent ductras arteriosus, radioactivity was decreased especially at the left lung. It is observed that in acyanotic congenital heart disease, radioactivity of lung is not related with pulmonary arterial pressure. In 3 cases of Tetralogy of Fallot, radioactivity was even at both entire lung and in 2 of them, extrapulmonary radioactivity of liver or kidney which depends on size of defect and volume of right to left shunt reversible, was noted.

  12. Left ventricular hypertrabeculation/noncompaction with epilepsy, other heart defects, minor facial anomalies and new copy number variants

    Nagel Bert

    2012-07-01

    Full Text Available Abstract Background Left ventricular hypertrabeculation/noncompaction (LVHT is a cardiac abnormality of unknown etiology which has been described in children as well as in adults with and without chromosomal aberrations. LVHT has been reported in association with various cardiac and extracardiac abnormalities like epilepsy and facial dysmorphism. Case presentation A unique combination of LVHT, atrial septal defect, pulmonary valve stenosis, aortic stenosis, epilepsy and minor facial anomalies is presented in a 5.5 years old girl. Microarray-based genomic hybridization (array-CGH detected six previously not described copy number variants (CNVs inherited from a clinically unaffected father and minimally affected mother, thus, most likely, not clinically significant but rare benign variants. Conclusions Despite this complex phenotype de novo microdeletions or microduplications were not detected by array CGH. Further investigations, such as whole exome sequencing, could reveal point mutations and small indels as the possible cause.

  13. Conotruncal Heart Defect Repair in Sub-Saharan Africa: Remarkable Outcomes Despite Poor Access to Treatment.

    Edwin, Frank; Entsua-Mensah, Kow; Sereboe, Lawrence A; Tettey, Mark M; Aniteye, Ernest A; Tamatey, Martin M; Adzamli, Innocent; Akyaa-Yao, Nana; Gyan, Kofi B; Ofosu-Appiah, Ernest; Kotei, David

    2016-09-01

    The outcome of children born with conotruncal heart defects may serve as an indication of the status of pediatric cardiac care in sub-Saharan Africa (SSA). This study was undertaken to determine the outcome of children born with conotruncal anomalies in SSA, regarding access to treatment and outcomes of surgical intervention. From our institution in Ghana, we retrospectively analyzed the outcomes of surgery, in the two-year period from June 2013 to May 2015. The birth prevalence of congenital heart defects (CHDs) in SSA countries was derived by extrapolation using an incidence of 8 per 1,000 live births for CHDs. The birth prevalence of CHDs for the 48 countries in SSA using 2013 country data was 258,875; 10% of these are presumed to be conotruncal anomalies. Six countries (Nigeria, Democratic Republic of the Congo, Ethiopia, Tanzania, Uganda, and Kenya) accounted for 53.5% of the birth prevalence. In Ghana, 20 patients (tetralogy of Fallot [TOF], 17; pulmonary atresia, 3) underwent palliation and 50 (TOF, 36; double-outlet right ventricle, 14) underwent repair. Hospital mortality was 0% for palliation and 4% for repair. Only 6 (0.5%) of the expected 1,234 cases of conotruncal defects underwent palliation or repair within two years of birth. Six countries in SSA account for more than 50% of the CHD burden. Access to treatment within two years of birth is probably <1%. The experience from Ghana demonstrates that remarkable surgical outcomes are achievable in low- to middle-income countries of SSA. © The Author(s) 2016.

  14. [Value of chest x-ray films in the diagnosis of congenital heart defects in infants].

    Koczyński, A

    1982-01-01

    The respiratory distress and suspicion of the heart defects in newborns and infants is indicated by x-ray chest examinations. The right interpretation of the x-ray pictures is very important but it must be followed by other diagnostic procedures. In every child it is possible to take the linear measurements of the great vessels and arteries in parahilar lung areas as well as the heart and chest in two dimensions from x-ray plain films. The measurements let to establish the indices: cardio-thoracic (ICP), vasculo-cardiac (IVC) and sagittal one (IS), which play important role in radiological evaluation of the chest. It results from the investigated material, that the evaluation of the pulmonary vascular pattern and the indices particularly facilitate the diagnosis of heart deformities coexisting with higher blood flow in pulmonary circulation. Nevertheless the measurements and the indices play the relative role in establishing of the final opinion about the chest and should be considered together with clinical and cardiological data.

  15. Predicting congenital heart defects: A comparison of three data mining methods.

    Yanhong Luo

    Full Text Available Congenital heart defects (CHD is one of the most common birth defects in China. Many studies have examined risk factors for CHD, but their predictive abilities have not been evaluated. In particular, few studies have attempted to predict risks of CHD from, necessarily unbalanced, population-based cross-sectional data. Therefore, we developed and validated machine learning models for predicting, before and during pregnancy, women's risks of bearing children with CHD. We compared the results of these models in a large-scale, comprehensive population-based retrospective cross-sectional epidemiological survey of birth defects in six counties in Shanxi Province, China, covering 2006 to 2008. This contained 78 cases of CHD among 33831 live births. We constructed nine synthetic variables to use in the models: maternal age, annual per capita income, family history, maternal history of illness, nutrition and folic acid deficiency, maternal illness in pregnancy, medication use in pregnancy, environmental risk factors in pregnancy, and unhealthy maternal lifestyle in pregnancy. The machine learning algorithms Weighted Support Vector Machine (WSVM and Weighted Random Forest (WRF were trained on, and a logistic regression (Logit was fitted to, two-thirds of the data. Their predictive abilities were then tested in the remaining data. True positive rate (TPR, true negative rate (TNR, accuracy (ACC, area under the curves (AUC, G-means, and Weighted accuracy (WTacc were used to compare the classification performance of the models. Median values, from repeating the data partitioning 1000 times, were used in all comparisons. The TPR and TNR of the three classifiers were above 0.65 and 0.93, respectively, better than any reported in the literature. TPR, wtACC, AUC and G were highest for WSVM, showing that it performed best. All three models are precise enough to identify groups at high risk of CHD. They should all be considered for future investigations of other

  16. Assessing the influence of consanguinity on congenital heart disease

    Alan H Bittles

    2011-01-01

    Full Text Available Numerous articles have been published linking consanguineous marriage to an elevated prevalence of congenital heart disease, with ventricular septal defects and atrial septal defects the most commonly cited disorders. While initially persuasive, on closer examination many of these studies have fundamental shortcomings in their design and in the recruitment of study subjects and controls. Improved matching of cases and controls, to include recognition of the long-established community boundaries within which most marriages are contracted, and the assessment of consanguinity within specific levels and types of marital union would improve and help to focus the study outcomes. At the same time, major discrepancies between studies in their reported prevalence and types of congenital heart disease suggest an urgent need for greater standardization in the classification and reporting of these disorders.

  17. Assessing the influence of consanguinity on congenital heart disease

    Bittles, Alan H.

    2011-01-01

    Numerous articles have been published linking consanguineous marriage to an elevated prevalence of congenital heart disease, with ventricular septal defects and atrial septal defects the most commonly cited disorders. While initially persuasive, on closer examination many of these studies have fundamental shortcomings in their design and in the recruitment of study subjects and controls. Improved matching of cases and controls, to include recognition of the long-established community boundaries within which most marriages are contracted, and the assessment of consanguinity within specific levels and types of marital union would improve and help to focus the study outcomes. At the same time, major discrepancies between studies in their reported prevalence and types of congenital heart disease suggest an urgent need for greater standardization in the classification and reporting of these disorders

  18. Health-related quality of life in paediatric patients with congenital heart defects: association with the type of heart defect and the surgical technique.

    Heusch, A; Kahl, H J; Hensel, K O; Calaminus, G

    2017-11-01

    The aim of the study was to investigate the impact of a number of surgical interventions for a various congenital cardiac defects (CHDs) on self-reported HRQoL. Patients who had received corrective surgery of several congenital heart defects (surgical VSD closure, Fallot, TGA after atrial or arterial switch or Fontan-type circulation for univentricular AV-connection) were interviewed in the office of their home peadiatric cardiologist. HRQoL in children along 7 dimensions was assessed using a standardised questionnaire (PEDQoL); information on the medical case history of each respondent was also collected. HRQoL was assessed in the questionnaire by asking about the frequency (never, rarely, often, always) of specific negative experiences; more frequent experiences indicate a lower quality of life. Frequency expressions were transformed into numerical values (25, 50, 75, 100%), and mean values for HRQoL were calculated for each patient and for each domain. Differences in HRQoL among patients with different types of interventions were analysed using the Mann-Whitney Test or the Kruskal-Wallis Test as appropriate; p values <0.05 were considered to indicate significant differences, while p values <0.1 were considered to indicate notable trends. Patients: 169 patients (60% male, 40% female) were part of the study. The mean age was 11.6 years; 50 patients had surgical VSD closure, 52 surgeries for Tetralogy of Fallot (22 transannular patch, 18 no transannular patch, 12 inaccurate description), 40 had complete transposition of the great arteries (28 atrial switch, 12 arterial switch), 22 had a Fontan-type procedure for univentricular AV-connection. HRQoL differed little among patients with different CHDs for the items "relation to friends," "interactions in the affected families", and "own body image". For other items, notable differences emerged: patients with univentricular hearts rated their physical capacity worse and showed a tendency towards negative ratings in

  19. Between invisible defects and visible impact: the life experiences of adolescents and young adults with congenital heart disease.

    Chiang, Yueh-Tao; Chen, Chi-Wen; Su, Wen-Jen; Wang, Jou-Kou; Lu, Chun-Wei; Li, Yuh-Fen; Moons, Philip

    2015-03-01

    To describe the life experiences of adolescents and young adults with congenital heart disease. Owing to medical advances, most children with congenital heart disease are expected to survive into adulthood. The transitional development from adolescence to adult is the critical period for fostering self-care. Descriptive phenomenological study. Thirty-five patients of 15-24 years old with congenital heart disease were recruited from paediatric cardiology clinics by purposive sampling. They were individually interviewed between October 2012-February 2013 using a semi-structured interview guideline and joined adult congenital heart disease clinics at two medical centres in northern Taiwan. The data were analysed using descriptive phenomenological method developed by Giorgi. The essence of the life experience of adolescents and young adults with congenital heart disease involves a dynamic process of moving between invisible defects and coexistence with the disease. Six themes emerged: (1) invisible defects: the existence of imperfect understanding; (2) conflict: interpersonal frustrations; (3) imbalance: the loss of self-balance; (4) suffering: increasing anxiety; (5) encounters: meeting needs; and (6) coexistence: positive coping strategies. As patients with congenital heart disease transition from adolescence into adulthood, they must learn about their disease, overcome frustration and anxiety and develop self-care strategies for coexisting with congenital heart disease. Results of this study may serve as clinical care guidelines for adolescents and young adults with congenital heart disease and give a reference for developing transitional intervention strategies. © 2014 John Wiley & Sons Ltd.

  20. Familial co-occurrence of congenital heart defects follows distinct patterns

    Ellesøe, Sabrina G.; Workman, Christopher T.; Bouvagnet, Patrice

    2018-01-01

    Congenital heart defects (CHD) affect almost 1% of all live born children and the number of adults with CHD is increasing. In families where CHD has occurred previously, estimates of recurrence risk, and the type of recurring malformation are important for counselling and clinical decision......-making, but the recurrence patterns in families are poorly understood. We aimed to determine recurrence patterns, by investigating the co-occurrences of CHD in 1163 families with known malformations, comprising 3080 individuals with clinically confirmed diagnosis. We calculated rates of concordance and discordance for 41...... specific types of malformations, observing a high variability in the rates of concordance and discordance. By calculating odds ratios for each of 1640 pairs of discordant lesions observed between affected family members, we were able to identify 178 pairs of malformations that co-occurred significantly...

  1. Familial co-occurrence of congenital heart defects follows distinct patterns

    Ellesøe, Sabrina G.; Workman, Christopher T.; Bouvagnet, Patrice

    2017-01-01

    Congenital heart defects (CHD) affect almost 1% of all live born children and the number of adults with CHD is increasing. In families where CHD has occurred previously, estimates of recurrence risk, and the type of recurring malformation are important for counselling and clinical decision......-making, but the recurrence patterns in families are poorly understood. We aimed to determine recurrence patterns, by investigating the co-occurrences of CHD in 1163 families with known malformations, comprising 3080 individuals with clinically confirmed diagnosis. We calculated rates of concordance and discordance for 41...... specific types of malformations, observing a high variability in the rates of concordance and discordance. By calculating odds ratios for each of 1640 pairs of discordant lesions observed between affected family members, we were able to identify 178 pairs of malformations that co-occurred significantly...

  2. Enlarged Heart

    ... rheumatic fever, a heart defect, infections (infectious endocarditis), connective tissue disorders, certain medications or radiation treatments for cancer, your heart may enlarge. Disease of the heart ...

  3. Intellectual functioning in children with congenital heart defects treated with surgery or by catheter interventions

    Carmen Ryberg

    2016-11-01

    Full Text Available Background: Studies suggest that children with congenital heart defects (CHD are at risk for adverse intellectual functioning. However, factors related to lower intellectual functioning in this group are largely unknown. This study describes intellectual functioning in children with CHD in relation to severity of the heart defect, the child´s age and the socioeconomic status of the family (SES.Methods: 228 children treated with surgery or by catheter technique were tested using the Wechsler intelligence scales to determine Full Scale IQ (FSIQ. FSIQ was then analyzed in relation to age (3- 5- , 9-, and 15-year-olds, severity of the diagnosis (mild, moderate, and severe, and SES (low, medium, and high. The median age was 70 months (5.8 years with a range of 162 months (30 months (2.5 years to 192 months (16.0 years. Results: The total mean score on FSIQ was 100.8 (SD = 14.5. Children with severe CHD had significantly lower FSIQ than children with mild and moderate CHD, and 9- and 15-year-olds had significantly lower FSIQ compared to the 3-year-olds. Children from families with low SES had significantly lower FSIQ than children from medium SES and high SES families. No interaction between severity of diagnosis, age, and SES was found for FSIQ.Conclusions: 83% of the children with CHD performed at or above average with respect to FSIQ. SES and severity of diagnosis had significant main effects on FSIQ. These factors should be considered when planning interventions and follow-up programs for children with CHD.

  4. Congenital heart disease in adults and its problems

    Teddy Ontoseno

    2001-10-01

    Full Text Available There were 40 adult congenital heart disease (CHD patients seen in the Cardiology Division during 1 year (February 1993 - February 1994. The most frequently seen defect was atrial septal defect; however there were also cases with patent ductus arteriosus, pulmonary stenosis, ventricular septal defect, and tetralogy of Fallot. Hemodynamic disorder, serious hindrance to education achievement, and occupational threat due to limited physical capabilities as well as malnutrition are some of prominent issues to be closely anticipated. In general the older the patients the more serious hemodynamic disorder they suffer due CHD. It is worth thinking how to improve the quality of life of CHD patients who succeed to live their adult lives and minimize any possible fatal complication risks.

  5. The commonest mistakes in the treatment of adult patients with congenital heart disease

    Jure Dolenc

    2012-04-01

    Full Text Available Background: General practitioners and medical specialists are seeing adults with congenital heart disease in their everyday practice more frequently than ever. The lack of experience and knowledge in this field of cardiology often leads to mistakes in the treatment of these patients. The purpose of this article is to point out the commonest problems and mistakes in dealing with this group of patients. Conclusions: In the following article we pointed out the commonest problems and mistakes in dealing with adults with congenital heart disease, such as differing various types of atrial septal defects and differing atrial septal defect from patent foramen ovale. We also pointed out the commonest mistakes in dealing with patients with cyanotic heart disease, especially those with Eisenmenger syndrome.

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

    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

  7. Detection of chromosomal abnormalities and the 22q11 microdeletion in fetuses with congenital heart defects.

    Lv, Wei; Wang, Shuyu

    2014-11-01

    Chromosomal abnormalities and the 22q11 microdeletion are implicated in congenital heart defects (CHDs). This study was designed to detect these abnormalities in fetuses and determine the effect of genetic factors on CHD etiology. Between January 2010 and December 2011, 113 fetuses with CHD treated at the Beijing Obstetrics and Gynecology Hospital were investigated, using chromosome karyotyping of either amniotic fluid cell or umbilical cord blood cell samples. Fetuses with a normal result were then investigated for the 22q11 microdeletion by fluorescence in situ hybridization. Of the 113 patients, 12 (10.6%) exhibited chromosomal abnormalities, while 6 (5.3%) of the remaining 101 cases presented with a 22q11 microdeletion. The incidence of chromosomal abnormalities was significantly higher in the group of fetuses presenting with extracardiac malformations in addition to CHD (Pheart defects should also be considered for 22q11 microdeletion detection to evaluate fetal prognosis, particularly prior to surgery.

  8. [Nasal septal abscess].

    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.

  9. Maternal intake of fat, riboflavin and nicotinamide and the risk of having offspring with congenital heart defects

    Smedts, H.P.M.; Rakhshandehroo, M.; Verkleij-Hagoort, A.C.; Vries, de J.H.M.; Ottenkamp, J.; Steegers, E.A.P.; Steegers-Theunissen, R.P.M.

    2008-01-01

    With the exception of studies on folic acid, little evidence is available concerning other nutrients in the pathogenesis of congenital heart defects (CHDs). Fatty acids play a central role in embryonic development, and the B-vitamins riboflavin and nicotinamide are co-enzymes in lipid metabolism.

  10. Maternal intake of fat, riboflavin and nicotinamide and the risk of having offspring with congenital heart defects

    Smedts, Huberdina P. M.; Rakhshandehroo, Maryam; Verkleij-Hagoort, Anna C.; de Vries, Jeanne H. M.; Ottenkamp, Jaap; Steegers, Eric A. P.; Steegers-Theunissen, Régine P. M.

    2008-01-01

    BACKGROUND: With the exception of studies on folic acid, little evidence is available concerning other nutrients in the pathogenesis of congenital heart defects (CHDs). Fatty acids play a central role in embryonic development, and the B-vitamins riboflavin and nicotinamide are co-enzymes in lipid

  11. 118 SNPs of folate-related genes and risks of spina bifida and conotruncal heart defects

    Shaw Gary M

    2009-06-01

    Full Text Available Abstract Background Folic acid taken in early pregnancy reduces risks for delivering offspring with several congenital anomalies. The mechanism by which folic acid reduces risk is unknown. Investigations into genetic variation that influences transport and metabolism of folate will help fill this data gap. We focused on 118 SNPs involved in folate transport and metabolism. Methods Using data from a California population-based registry, we investigated whether risks of spina bifida or conotruncal heart defects were influenced by 118 single nucleotide polymorphisms (SNPs associated with the complex folate pathway. This case-control study included 259 infants with spina bifida and a random sample of 359 nonmalformed control infants born during 1983–86 or 1994–95. It also included 214 infants with conotruncal heart defects born during 1983–86. Infant genotyping was performed blinded to case or control status using a designed SNPlex assay. We examined single SNP effects for each of the 118 SNPs, as well as haplotypes, for each of the two outcomes. Results Few odds ratios (ORs revealed sizable departures from 1.0. With respect to spina bifida, we observed ORs with 95% confidence intervals that did not include 1.0 for the following SNPs (heterozygous or homozygous relative to the reference genotype: BHMT (rs3733890 OR = 1.8 (1.1–3.1, CBS (rs2851391 OR = 2.0 (1.2–3.1; CBS (rs234713 OR = 2.9 (1.3–6.7; MTHFD1 (rs2236224 OR = 1.7 (1.1–2.7; MTHFD1 (hcv11462908 OR = 0.2 (0–0.9; MTHFD2 (rs702465 OR = 0.6 (0.4–0.9; MTHFD2 (rs7571842 OR = 0.6 (0.4–0.9; MTHFR (rs1801133 OR = 2.0 (1.2–3.1; MTRR (rs162036 OR = 3.0 (1.5–5.9; MTRR (rs10380 OR = 3.4 (1.6–7.1; MTRR (rs1801394 OR = 0.7 (0.5–0.9; MTRR (rs9332 OR = 2.7 (1.3–5.3; TYMS (rs2847149 OR = 2.2 (1.4–3.5; TYMS (rs1001761 OR = 2.4 (1.5–3.8; and TYMS (rs502396 OR = 2.1 (1.3–3.3. However, multiple SNPs observed for a given gene showed evidence of linkage disequilibrium indicating

  12. Spectrum Of Congenital Heart Disease In Full Term Neonates.

    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

  13. The heart and great vessels

    Condon, V.

    1985-01-01

    Heart disease is the fifth most common cause of death in infants and children (preceded by anoxic and hypoxic conditions, gross congenital malformations, accidental death, and immaturity). Of all the cardiac lesions, congenital heart disease (CHD) makes up the gross majority, accounting for approximately 90% of all cardiac deaths. Approximately two-thirds of all infants who die from CHD do so within the first year of life; of these, approximately one-third die within the first month. The most common cause of death in the first month is hypoplastic left heart syndrome and lesions associated with it, i.e., aortic atresia/critical aortic stenosis and mitral atresia/critical mitral stenosis. Severe coarctation of the aorta (coarctation syndrome) and transposition of the great arteries are the other most important causes of death in this age group. CHD occurs as a familial condition in approximately 1-4% of cases; ventricular septal defects, patent ductus arteriosus, and atrial septal defect are particularly common forms. Parental age plays an important role, with a significantly increased risk of CHD in infants of mothers over 39 years of age. Patent ductus arteriosus is more prevalent in firstborn children, particularly those born prematurely to young mothers. Environmental factors, such as exposure to teratogenic agents, have also been shown to increase the incidence of CHD. Children with various syndromes also have increased incidence of CHD. Down syndrome is a classic example, as are other trisomies

  14. Novel Models to Study Effect of High-Altitude Hypoxic Exposure and Placental Insufficiency on Fetal Oxygen Metabolism and Congenital Heart Defects

    2017-10-01

    Metabolism and Congenital Heart Defects PRINCIPAL INVESTIGATOR: Steven Fisher, MD RECIPIENT: University of Maryland, Baltimore REPORT DATE...examined in fetal heart vs other tissues. Pregnant ODD-Luc dams age 3-6 months will inhale O2 concentrations ranging from 8-21% O2 x 4 h...Congenital Heart Defects in at-risk pregnancies. The first objective was to use a novel reporter of O2 concentrations, the ODDLuc mouse, to determine the

  15. Critical congenital heart defects and abnormal levels of routinely collected first- and second-trimester biomarkers.

    Borelli, Melissa; Baer, Rebecca J; Chambers, Christina D; Smith, Tyler C; Jelliffe-Pawlowski, Laura L

    2017-02-01

    We examined the association between maternal characteristics, routinely collected first- and second-trimester biomarkers and the risk of having an infant with a critical congenital heart defect (CCHD). Included were women who participated in the California Prenatal Screening Program who had nuchal translucency (NT) measurement and first- and second-trimester serum screening. All pregnancies ended in a live birth of an infant without aneuploidy or a neural tube defect. Poisson regression analyses were used to estimate the relative risk and 95% confidence interval of a CCHD by maternal characteristics, first- and second-trimester serum biomarkers or NT measurements. The sample included 118,194 mother-infant pairs; 284 infants had a CCHD. Women with preexisting diabetes were three-times as likely to have an infant with a CCHD. After adjusting for preexisting diabetes, women with first-trimester human chorionic gonatotropin (hCG) measurement <10th centile were 1.6-times as likely to have an infant with a CCHD (P = 0.011). Women with a NT measurement ≥95th centile were at two- to threefold higher risk of having an infant with a CCHD (P's = 0.004-0.007). Pregnancies with two risk factors for an infant with a CCHD were 5.6-times more likely to have an infant with a CCHD than women with no identified risk factors (P < 0.001). Despite the increased risk, performance testing demonstrated low sensitivity and specificity for screening use of these risk factors. Of the women with an infant with a CCHD, only 21.8% had an identified risk factor. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  16. Cataracts, radiculomegaly, septal heart defects and hearing loss in two unrelated adult females with normal intelligence and similar facial appearance : Confirmation of a syndrome

    Aalfs, CM; Oosterwijk, JC; VanSchooneveld, MJ; Begeman, CJ; Wabeke, KB; Hennekam, RCM

    Two unrelated, adult females with normal intelligence are described. They show a similar clinical picture with a long and narrow face, congenital cataract, microphthalmia, microcornea, a high nasal bridge, a short nose, a broad nasal tip, a long philtrum, bilateral hearing loss, persistent primary

  17. Dental plaque microbial profiles of children from Khartoum, Sudan, with congenital heart defects.

    Mohamed Ali, Hiba; Berggreen, Ellen; Nguyen, Daniel; Wahab Ali, Raouf; Van Dyke, Thomas E; Hasturk, Hatice; Mustafa, Manal

    2017-01-01

    Few studies have focused on the bacterial species associated with the deterioration of the dental and gingival health of children with congenital heart defects (CHD). The aims of this study were (1) to examine the dental plaque of children with CHD in order to quantify bacterial load and altered bacterial composition compared with children without CHD; and (2) to investigate the correlation between the level of caries and gingivitis and dental biofilm bacteria among those children. In this cross-sectional study, participants were children (3-12 years) recruited in Khartoum State, Sudan. A total of 80 CHD cases from the Ahmed Gasim Cardiac Centre and 80 healthy controls from randomly selected schools and kindergartens were included. Participants underwent clinical oral examinations for caries (decayed, missing, and filled teeth indices [DMFT] for primary dentition, and DMFT for permanent dentition), and gingivitis (simplified gingival index [GI]). Pooled dental biofilm samples were obtained from four posterior teeth using paper points. Real-time quantitative polymerase chain reaction was used for the detection and quantification of Streptococcus mutans , Streptococcus sanguinis, and Lactobacillus acidophilus . Checkerboard DNA-DNA hybridization was used for the detection of 40 additional bacterial species. CHD cases had a significantly higher caries experience (DMFT = 4.1 vs. 2.3, p  bacterial species exhibited significantly higher mean counts among CHD cases ( p  bacterial species in their dental plaque, which correlated with higher levels of caries and gingivitis.

  18. Chromosomal abnormalities and copy number variations in fetal left-sided congenital heart defects.

    Jansen, Fenna A R; Hoffer, Mariette J V; van Velzen, Christine L; Plati, Stephani Klingeman; Rijlaarsdam, Marry E B; Clur, Sally-Ann B; Blom, Nico A; Pajkrt, Eva; Bhola, Shama L; Knegt, Alida C; de Boer, Marion A; Haak, Monique C

    2016-02-01

    To demonstrate the spectrum of copy number variants (CNVs) in fetuses with isolated left-sided congenital heart defects (CHDs), and analyse genetic content. Between 2003 and 2012, 200 fetuses were identified with left-sided CHD. Exclusion criteria were chromosomal rearrangements, 22q11.2 microdeletion and/or extra-cardiac malformations (n = 64). We included cases with additional minor anomalies (n = 39), such as single umbilical artery. In 54 of 136 eligible cases, stored material was available for array analysis. CNVs were categorized as either (likely) benign, (likely) pathogenic or of unknown significance. In 18 of the 54 isolated left-sided CHDs we found 28 rare CNVs (prevalence 33%, average 1.6 CNV per person, size 10.6 kb-2.2 Mb). Our interpretation yielded clinically significant CNVs in two of 54 cases (4%) and variants of unknown significance in three other cases (6%). In left-sided CHDs that appear isolated, with normal chromosome analysis and 22q11.2 FISH analysis, array analysis detects clinically significant CNVs. When counselling parents of a fetus with a left-sided CHD it must be taken into consideration that aside from the cardiac characteristics, the presence of extra-cardiac malformations and chromosomal abnormalities influence the treatment plan and prognosis. © 2015 John Wiley & Sons, Ltd.

  19. Gene-Gene Interactions in the Folate Metabolic Pathway and the Risk of Conotruncal Heart Defects

    Philip J. Lupo

    2010-01-01

    Full Text Available Conotruncal and related heart defects (CTRD are common, complex malformations. Although there are few established risk factors, there is evidence that genetic variation in the folate metabolic pathway influences CTRD risk. This study was undertaken to assess the association between inherited (i.e., case and maternal gene-gene interactions in this pathway and the risk of CTRD. Case-parent triads (n=727, ascertained from the Children's Hospital of Philadelphia, were genotyped for ten functional variants of nine folate metabolic genes. Analyses of inherited genotypes were consistent with the previously reported association between MTHFR A1298C and CTRD (adjusted P=.02, but provided no evidence that CTRD was associated with inherited gene-gene interactions. Analyses of the maternal genotypes provided evidence of a MTHFR C677T/CBS 844ins68 interaction and CTRD risk (unadjusted P=.02. This association is consistent with the effects of this genotype combination on folate-homocysteine biochemistry but remains to be confirmed in independent study populations.

  20. First day of life pulse oximetry screening to detect congenital heart defects.

    Meberg, Alf; Brügmann-Pieper, Sabine; Due, Reidar; Eskedal, Leif; Fagerli, Ingebjørg; Farstad, Teresa; Frøisland, Dag Helge; Sannes, Catharina Hovland; Johansen, Ole Jakob; Keljalic, Jasmina; Markestad, Trond; Nygaard, Egil Andre; Røsvik, Alet; Silberg, Inger Elisabeth

    2008-06-01

    To evaluate the efficacy of first day of life pulse oximetry screening to detect congenital heart defects (CHDs). We performed a population-based prospective multicenter study of postductal (foot) arterial oxygen saturation (SpO(2)) in apparently healthy newborns after transfer from the delivery suite to the nursery. SpO(2) < 95% led to further diagnostic evaluations. Of 57,959 live births, 50,008 (86%) were screened. In the screened population, 35 CHDs were [corrected] classified as critical (ductus dependent, cyanotic). CHDs were prospectively registered and diagnosed in 658/57,959 (1.1%) [corrected] Of the infants screened, 324 (0.6%) failed the test. Of these, 43 (13%) had CHDs (27 critical), and 134 (41%) had pulmonary diseases or other disorders. The remaining 147 infants (45%) were healthy with transitional circulation. The median age for babies with CHDs at failing the test was 6 hours (range, 1-21 hours). For identifying critical CHDs, the pulse oximetry screening had a sensitivity rate of 77.1% (95% CI, 59.4-89.0), specificity rate of 99.4% (95% CI, 99.3-99.5), and a false-positive rate of 0.6% (95% CI, 0.5-0.7). Early pulse oximetry screening promotes early detection of critical CHDs and other potentially severe diseases. The sensitivity rate for detecting critical CHDs is high, and the false-positive rate is low.

  1. Elevated dimethylglycine in blood of children with congenital heart defects and their mothers.

    Alsayed, Ranwa; Al Quobaili, Faizeh; Srour, Samir; Geisel, Jürgen; Obeid, Rima

    2013-08-01

    Congenital Heart Defects (CHD) may be related to nutritional deficiencies affecting the methylation cycle. We aimed to study the metabolic markers of the betaine homocysteine methyl transferase (BHMT) pathway in children with CHD and their mothers compared to children without CHD and their mothers. Children with CHD (n=105, age DMG). Children with CHD had higher plasma SAM (131 vs. 100 nmol/L) and DMG (8.7 vs. 6.0 μmol/L) and lower betaine/DMG ratio (7.5 vs. 10.2) compared to the controls. Mothers of CHD children showed also higher DMG (6.1 vs. 4.1 µmol/L) and lower betaine/DMG ratio compared with the mothers of the controls. Higher SAM levels were related to higher cystathionine, MMA, betaine, choline, and DMG. MMA elevation in the patients was related to higher HCY, SAM, betaine and DMG. Elevated DMG in CHD children and their mothers compared to the controls can indicate upregulation of the BHMT pathway in this disease group. Nutritional factors are related to metabolic imbalance during pregnancy that may be related to worse birth outcome. Copyright © 2013 Elsevier Inc. All rights reserved.

  2. Idiopathic dilated cardiomyopathy: computerized anatomic study of relashionship between septal and free left ventricle wall.

    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.

  3. Spectrum of congenital heart defects and extracardiac malformations associated with chromosomal abnormalities: results of a seven year necropsy study

    Tennstedt, C; Chaoui, R; Korner, H; Dietel, M

    1999-01-01

    OBJECTIVE—To analyse the spectrum of congenital heart malformations, the frequency of extracardiac malformations, and the proportion of chromosome aberrations among fetuses sent for necropsy.
MATERIAL—Necropsies were performed on 815 fetuses—448 induced abortions (55%), 220 spontaneous abortions (27%), and 147 stillbirths (18%)—during a seven year period (1991-97) in the department of pathology of the Charité Medical Centre in Berlin. A congenital heart defect was identified in 129 cases (16%...

  4. Nonsustained Repetitive Upper Septal Idiopathic Fascicular Left Ventricular Tachycardia: Rare Type of VT

    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.

  5. Magnetic resonance imaging in complete type endocardial cushion defect

    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

  6. Late bacterial endocarditis of an Amplatzer atrial septal device

    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.

  7. [Temporal variation and geographical distribution: congenital heart defects in the Comunitat Valenciana].

    Cavero Carbonell, C; Zurriaga, O; Pérez Panadés, J; Barona Vilar, C; Martos Jiménez, C

    2013-09-01

    The objective of this study is to estimate the prevalence of congenital heart defects (CHD) in the Comunitat Valenciana (CV) in children less than one year old and identify whether there are temporal and geographic variations within this prevalence. The minimum basic data set from hospital discharge reports was used to select patients, who were born between 1999-2008, were less than one year old, and who lived in the CV with at least one hospital admission in which the primary diagnosis and/or any of the events were coded as CHD (codes 745-747 of the International Classification of Diseases 9th Revision Clinical Modification). The first hospital discharge report with CHD was selected, using the health card number to detect duplication. The prevalence and 95% confidence intervals were calculated, and the prevalence ratio (PR) and smoothed PR was obtained for each municipality to identify geographic patterns. In the period 1999-2008 there were 6.377 patients younger than one year with some CHD, representing the 43.2% of cases of congenital anomalies. The prevalence was 134.3 per 10.000 live births (95% CI: 131.1-137.6). There was a significant increase in the prevalence, from 115.8 in the 1999-2003 period to 149.5 in the 2004-2008 period. A higher risk was identified in the north of the CV, and in some municipalities of the province of Alicante, in the south. The observed increase in CHD agrees with the findings in other countries and it can be explained, at least in part, by improved diagnostic techniques. The geographic pattern identified requires a more detailed analysis that could explain the geographic variations found. Copyright © 2012 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  8. Application of new balloon catheters in the treatment of congenital heart defects

    Roland Fiszer

    2016-08-01

    Full Text Available Introduction : Balloon angioplasty (BAP and aortic or pulmonary balloon valvuloplasty (BAV, BPV are well-established treatment options in congenital heart defects. Recently, significant technological progress has been made and new catheters have been implemented in clinical practice. Aim: To analyze the results of BAP, BAV and BPV with the new balloon catheter Valver and its second generation Valver II, which the company Balton (Poland launched and developed. These catheters have not been clinically evaluated yet. Material and methods: We performed 64 interventions with Valver I and Valver II. With Valver I the following procedures were performed: 17 BPV (including 9 in tetralogy of Fallot – TOF, 10 BAV and 27 BAP in coarctations of the aorta (CoA – including 9 native and 18 after surgery. With Valver II ten interventions were done – 3 BPV, 2 pulmonary supravalvular BAP (after switch operations, 2 BAP of recoarctations and 3 other BAP. Age of the patients ranged from a few days to 40 years. Results: All procedures were completed successfully, without rupture of any balloon catheters. The pressure gradient drop was statistically significant in all groups: BPV in isolated pulmonary valvular stenosis 28.1 mm Hg (mean, BPV in TOF 18.7 mm Hg, BAV 32.8 mm Hg, BAP in native CoA 15.4 mm Hg and in recoarctations 18.6 mm Hg. In 3 cases during rapid deflation of Valver I, wrinkles of the balloons made it impossible to insert the whole balloon into the vascular sheath (all were removed surgically from the groin. No such complication occured with Valver II. Conclusions : Valver balloon catheters are an effective treatment modality in different valvular and vascular stenoses.

  9. Antecedents of Self-Care in Adults with Congenital Heart Defects

    McCabe, Nancy; Dunbar, Sandra B.; Butler, Javed; Higgins, Melinda; Book, Wendy; Reilly, Carolyn

    2015-01-01

    Background Adults with congenital heart defects (ACHD) face long-term complications related to prior surgery, abnormal anatomy, and acquired cardiovascular conditions. Although self-care is an important part of chronic illness management, few studies have explored self-care in the ACHD population. The purpose of this study is to describe self-care and its antecedents in the ACHD population. Methods Persons with moderate or severe ACHD (N=132) were recruited from a single ACHD center. Self-care (health maintenance behaviors, monitoring and management of symptoms), and potential antecedents including sociodemographic and clinical characteristics, ACHD knowledge, behavioral characteristics (depressive symptoms and self-efficacy), and family-related factors (parental overprotection and perceived family support) were collected via self-report and chart review. Multiple regression was used to identify antecedents of self-care maintenance, monitoring, and management. Results Only 44.7%, 27.3%, and 23.3% of participants performed adequate levels of self-care maintenance, monitoring and management, respectively. In multiple regression analysis, self-efficacy, education, gender, perceived family support, and comorbidities explained 25% of the variance in self-care maintenance (R2=.248, F(5, 123)=9.44, p<.001). Age, depressive symptoms, self-efficacy, and NYHA Class explained 23% of the variance in self-care monitoring (R2=.232, F(2, 124)=10.66, p<.001). Self-efficacy and NYHA Class explained 9% of the variance in self-care management (R2=.094, F(2, 80)=5.27, p=.007). Conclusions Low levels of self-care are common among persons with ACHD. Multiple factors, including modifiable factors of self-efficacy, depressive symptoms, and perceived family support, are associated with self-care and should be considered in designing future interventions to improve outcomes in the ACHD population. PMID:26340127

  10. The Prevalence of Celiac Disease in Down syndrome Children with and without Congenital Heart Defects

    Noor Mohammad Noori

    2016-07-01

    Full Text Available Background The prevalence of celiac disease (CD is remarkably varied in Down syndrome(DSpatientscompared with other diseases.  This study aimed to assess celiac disease prevalence in Down syndrome children with and without congenital heart defects (CHD and its comparison with controls. Materials and Methods This case-control study was performed at a single center on 132 participants in three groups. Clinical and genetic tests were performed on all patients suspected with Down syndrome to confirm their diseases.  After that in patients with confirmed Down syndrome echocardiography was carried out to diagnosis of CHD. Healthy children selected randomly among those who referred to the center for annual check-up. Statistical evaluation was done using SPSS-16. Results For the factors of age, weight, height and Body Mass Index (BMI not observed significant differences between three groups of participants, but it would be observed statistically differences for the variable of tTG- IgA.  For variables of weight, tTG- IgA and BMI was observed statistically different in the case and controls. The status of tTG- IgA (normal or 20 had significant correlation with three groups of controls, Down syndrome with and without CHD. The status of tTG- IgA also had significant correlation with groups of case and controls. In comparison of tTG- IgA in DS patients with and without CHD, no significant differences were observed. Conclusion The prevalence of CD in DS patients was higher compared the controls population; and in DS patients with CHD was higher compared the DS patients without CHD.

  11. Presence of plaque, gingivitis and caries in Sudanese children with congenital heart defects.

    Ali, Hiba Mohamed; Mustafa, Manal; Hasabalrasol, Siham; Elshazali, Osama Hafiz; Nasir, Elwalid Fadul; Ali, Raouf Wahab; Berggreen, Ellen; Skeie, Marit Slåttelid

    2017-05-01

    The objective of this study is to assess the presence of plaque, gingivitis, and caries in a group of Sudanese children with congenital heart defects CHDs (cases) and compare them to children without CHDs (controls). This analytical cross-sectional study included cases (N = 111, with a mean age of 7.2 ± 3.0 years) and controls (N = 182, with a mean age of 7.2 ± 2.8 years) from Khartoum, Sudan. Examinations were done by two calibrated dentists using plaque index, gingival index, and WHO (World Health Organization) caries diagnostic criteria (dmft/DMFT index: decayed, missing, and filled teeth). Children with CHDs (cases) had statistically significantly higher mean number of sites with plaque and gingivitis than children without CHDs (controls), although almost all children experienced plaque. Cases also experienced significantly higher mean dmft/DMFT than controls (age group 1, 3-7 years: 3.7 vs 2.3 and age group 2, 8-12 years: 1.3 vs 0.6). The Significant Caries Indices in cases (age groups 1 and 2) were also significantly higher than among controls (SiC 8.2 vs 5.9 and 1.8 vs 0.8, respectively). Fillings were totally lacking among cases and scarce among controls. The findings clearly showed that this group of Sudanese children with CHDs was more severely affected with gingivitis and caries than the control group without CHDs. These results are cause for concern in children at risk of developing systemic infections and serious complications related to poor oral health. These findings provide important baseline data for planning appropriate dental preventive strategies for Sudanese children with CHDs.

  12. Tessier Number 30 Median Mandibular Cleft With Congenital Heart Anomalies in Qena, Egypt.

    Ali, Ahmed Ali Abdelrahim

    2018-01-01

    Median cleft deformities of the lower lip and mandible are very rare congenital anomalies. Our patient had median cleft of the lower lip, mandible, and the chin with tongue duplication, ankyloglossia, and cleft strap muscles with 2 neck contracture bands. This anomaly was associated with congenital heart disease transposition of great vessels, large ventricular septal defect, and severe pulmonary stenosis. Early repair was done at 6 months to improve feeding.

  13. Mutations in STRA6 cause a broad spectrum of malformations including anophthalmia, congenital heart defects, diaphragmatic hernia, alveolar capillary dysplasia, lung hypoplasia, and mental retardation

    Pasutto, Francesca; Sticht, Heinrich; Hammersen, Gerhard; Gillessen-Kaesbach, Gabriele; Fitzpatrick, David R.; Nuernberg, Gudrun; Brasch, Frank; Schirmer-Zimmermann, Heidemarie; Tolmie, John L.; Chitayat, David; Houge, Gunnar; Fernandez-Martinez, Lorena; Keating, Sarah; Mortier, Geert; Hennekam, Raoul C. M.; von der Wense, Axel; Slavotinek, Anne; Meinecke, Peter; Bitoun, Pierre; Becker, Christian; Nuernberg, Peter; Reis, Andre; Rauch, Anita

    2007-01-01

    We observed two unrelated consanguineous families with malformation syndromes sharing anophthalmia and distinct eyebrows as common signs, but differing for alveolar capillary dysplasia or complex congenital heart defect in one and diaphragmatic hernia in the other family. Homozygosity mapping

  14. Introduction to the Congenital Heart Defects: Anatomy of the Conduction System.

    Moore, Jeremy P; Aboulhosn, Jamil A

    2017-06-01

    The position and course of the conduction system in congenital heart disease are intricately tied to the underlying congenital malformation. Although only subtle differences exist between the anatomy of the conduction axis for simple congenital heart lesions and normal anatomy, almost every patient with congenital heart disease harbors some important anatomic variation. This article summarizes the body of literature by retaining original classical concepts and by attempting to translate the available knowledge into useful points for the congenital heart disease specialist. This discussion spans the entire spectrum of simple to complex congenital heart disease. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. Proteomic-based detection of a protein cluster dysregulated during cardiovascular development identifies biomarkers of congenital heart defects.

    Anjali K Nath

    Full Text Available Cardiovascular development is vital for embryonic survival and growth. Early gestation embryo loss or malformation has been linked to yolk sac vasculopathy and congenital heart defects (CHDs. However, the molecular pathways that underlie these structural defects in humans remain largely unknown hindering the development of molecular-based diagnostic tools and novel therapies.Murine embryos were exposed to high glucose, a condition known to induce cardiovascular defects in both animal models and humans. We further employed a mass spectrometry-based proteomics approach to identify proteins differentially expressed in embryos with defects from those with normal cardiovascular development. The proteins detected by mass spectrometry (WNT16, ST14, Pcsk1, Jumonji, Morca2a, TRPC5, and others were validated by Western blotting and immunoflorescent staining of the yolk sac and heart. The proteins within the proteomic dataset clustered to adhesion/migration, differentiation, transport, and insulin signaling pathways. A functional role for several proteins (WNT16, ADAM15 and NOGO-A/B was demonstrated in an ex vivo model of heart development. Additionally, a successful application of a cluster of protein biomarkers (WNT16, ST14 and Pcsk1 as a prenatal screen for CHDs was confirmed in a study of human amniotic fluid (AF samples from women carrying normal fetuses and those with CHDs.The novel finding that WNT16, ST14 and Pcsk1 protein levels increase in fetuses with CHDs suggests that these proteins may play a role in the etiology of human CHDs. The information gained through this bed-side to bench translational approach contributes to a more complete understanding of the protein pathways dysregulated during cardiovascular development and provides novel avenues for diagnostic and therapeutic interventions, beneficial to fetuses at risk for CHDs.

  16. Outcome of patients undergoing open heart surgery at the Uganda heart institute, Mulago hospital complex.

    Aliku, Twalib O; Lubega, Sulaiman; Lwabi, Peter; Oketcho, Michael; Omagino, John O; Mwambu, Tom

    2014-12-01

    Heart disease is a disabling condition and necessary surgical intervention is often lacking in many developing countries. Training of the superspecialties abroad is largely limited to observation with little or no opportunity for hands on experience. An approach in which open heart surgeries are conducted locally by visiting teams enabling skills transfer to the local team and helps build to build capacity has been adopted at the Uganda Heart Institute (UHI). We reviewed the progress of open heart surgery at the UHI and evaluated the postoperative outcomes and challenges faced in conducting open heart surgery in a developing country. Medical records of patients undergoing open heart surgery at the UHI from October 2007 to June 2012 were reviewed. A total of 124 patients underwent open heart surgery during the study period. The commonest conditions were: venticular septal defects (VSDs) 34.7% (43/124), Atrial septal defects (ASDs) 34.7% (43/124) and tetralogy of fallot (TOF) in 10.5% (13/124). Non governmental organizations (NGOs) funded 96.8% (120/124) of the operations, and in only 4 patients (3.2%) families paid for the surgeries. There was increasing complexity in cases operated upon from predominantly ASDs and VSDs at the beginning to more complex cases like TOFs and TAPVR. The local team independently operated 19 patients (15.3%). Postoperative morbidity was low with arrhythmias, left ventricular dysfunction and re-operations being the commonest seen. Post operative sepsis occurred in only 2 cases (1.6%). The overall mortality rate was 3.2. Open heart surgery though expensive is feasible in a developing country. With increased direct funding from governments and local charities to support open heart surgeries, more cardiac patients access surgical treatment locally.

  17. POSTEROLATERAL DEFECT OF THE NORMAL HUMAN HEART INVESTIGATED WITH NITROGEN-13-AMMONIA AND DYNAMIC PET

    DEJONG, RM; BLANKSMA, PK; WILLEMSEN, ATM; ANTHONIO, RL; MEEDER, JG; PRUIM, J; VAALBURG, W; LIE, KI

    The posterolateral defect is a common artifact seen when static N-13-ammonia imaging with PET is used to assess myocardial perfusion. The aim of this study was to compare dynamic and static N-13-ammonia PET and to obtain more insight into the cause of the posterolateral defect. Methods: Dynamic

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

    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.

  19. Three-dimensional MR imaging of congenital heart disease

    Laschinger, J.C.; Vannier, M.W.; Knapp, R.H.; Gutierrez, F.R.; Cox, J.L.

    1987-01-01

    Contiguous 5-mm thick ECG-gated MR images of the thorax were edited using surface reconstruction techniques to produce three-dimensional (3D) images of the heart and great vessels in four healthy individuals and 25 patients with congenital heart disease (aged 3 months-30 years). Anomalies studied include atrial and ventricular septal defects, aortic coarctation, AV canal defects, double outlet ventricles, hypoplastic left heart syndrome, and a wide spectrum of patients with tetralogy of Fallot. The results were correlated with echocardiographic and cineradiographic studies, and with surgical findings or pathologic specimens. Three-dimensional reconstructions accurately localized the dimensions and locations of all cardiac and great vessel anomalies and often displayed anatomic findings not diagnosed or visualized with other forms of diagnostic imaging

  20. Kosovo’s Experience for Children with Feeding Difficulties after Cardiac Surgery for Congenital Heart Defect

    Ramush Bejiqi

    2017-11-01

    CONCLUSION: Feeding disorder is often and a frequent long-term sequel in children after neonatal or early infancy heart surgery. Patients with chromosomal and associated anomalies who underwent multiple cardiac surgeries are at risk of developing feeding difficulties.

  1. MR imaging of congenital heart disease

    Kersting-Sommerhoff, B.A.; Diethelm, L.; Teitel, D.F.; Sommerhoff, C.P.; Higgins, C.B.

    1988-01-01

    Sensitivity and specificity of MR imaging for the evaluation of congenital heart disease was assessed in 51 patients (31 male and 20 female, aged 3-69 years), with a total of 115 congenital heart lesions. The true diagnosis was established by angiocardiography, catheterization, or surgery. Sensitivity at a specificity level of 90% was determined by means of receiver operating characteristic curves for great vessel relationships (100%), thoracic aorta anomalies (94%), atrial (91%) and ventricular (100%) septal defects, visceroatrial situs (100%), loop (100%), right ventricular outflow obstructions (95%), aortic valve (52%), mitral valve (62%), and tricuspid valve (76%). Spin-echo MR imaging is a reliable method for the noninvasive evaluation of congenital heart disease but is limited in the assessment of some valvular anomalies

  2. Ultra high-resolution gene centric genomic structural analysis of a non-syndromic congenital heart defect, Tetralogy of Fallot.

    Douglas C Bittel

    Full Text Available Tetralogy of Fallot (TOF is one of the most common severe congenital heart malformations. Great progress has been made in identifying key genes that regulate heart development, yet approximately 70% of TOF cases are sporadic and nonsyndromic with no known genetic cause. We created an ultra high-resolution gene centric comparative genomic hybridization (gcCGH microarray based on 591 genes with a validated association with cardiovascular development or function. We used our gcCGH array to analyze the genomic structure of 34 infants with sporadic TOF without a deletion on chromosome 22q11.2 (n male = 20; n female = 14; age range of 2 to 10 months. Using our custom-made gcCGH microarray platform, we identified a total of 613 copy number variations (CNVs ranging in size from 78 base pairs to 19.5 Mb. We identified 16 subjects with 33 CNVs that contained 13 different genes which are known to be directly associated with heart development. Additionally, there were 79 genes from the broader list of genes that were partially or completely contained in a CNV. All 34 individuals examined had at least one CNV involving these 79 genes. Furthermore, we had available whole genome exon arrays from right ventricular tissue in 13 of our subjects. We analyzed these for correlations between copy number and gene expression level. Surprisingly, we could detect only one clear association between CNVs and expression (GSTT1 for any of the 591 focal genes on the gcCGH array. The expression levels of GSTT1 were correlated with copy number in all cases examined (r = 0.95, p = 0.001. We identified a large number of small CNVs in genes with varying associations with heart development. Our results illustrate the complexity of human genome structural variation and underscore the need for multifactorial assessment of potential genetic/genomic factors that contribute to congenital heart defects.

  3. Bone recycling in nasal septal reconstruction.

    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. Maternal obesity and tobacco use modify the impact of genetic variants on the occurrence of conotruncal heart defects.

    Tang, Xinyu; Nick, Todd G; Cleves, Mario A; Erickson, Stephen W; Li, Ming; Li, Jingyun; MacLeod, Stewart L; Hobbs, Charlotte A

    2014-01-01

    Conotruncal heart defects (CTDs) are among the most severe birth defects worldwide. Studies of CTDs indicate both lifestyle behaviors and genetic variation contribute to the risk of CTDs. Based on a hybrid design using data from 616 case-parental and 1645 control-parental triads recruited for the National Birth Defects Prevention Study between 1997 and 2008, we investigated whether the occurrence of CTDs is associated with interactions between 921 maternal and/or fetal single nucleotide polymorphisms (SNPs) and maternal obesity and tobacco use. The maternal genotypes of the variants in the glutamate-cysteine ligase, catalytic subunit (GCLC) gene and the fetal genotypes of the variants in the glutathione S-transferase alpha 3 (GSTA3) gene were associated with an elevated risk of CTDs among obese mothers. The risk of delivering infants with CTDs among obese mothers carrying AC genotype for a variant in the GCLC gene (rs6458939) was 2.00 times the risk among those carrying CC genotype (95% confidence interval: 1.41, 2.38). The maternal genotypes of several variants in the glutathione-S-transferase (GST) family of genes and the fetal genotypes of the variants in the GCLC gene interacted with tobacco exposures to increase the risk of CTDs. Our study suggests that the genetic basis underlying susceptibility of the developing heart to the adverse effects of maternal obesity and tobacco use involve both maternal and embryonic genetic variants. These results may provide insights into the underlying pathophysiology of CTDs, and ultimately lead to novel prevention strategies.

  5. Maternal intake of fat, riboflavin and nicotinamide and the risk of having offspring with congenital heart defects.

    Smedts, Huberdina P M; Rakhshandehroo, Maryam; Verkleij-Hagoort, Anna C; de Vries, Jeanne H M; Ottenkamp, Jaap; Steegers, Eric A P; Steegers-Theunissen, Régine P M

    2008-10-01

    With the exception of studies on folic acid, little evidence is available concerning other nutrients in the pathogenesis of congenital heart defects (CHDs). Fatty acids play a central role in embryonic development, and the B-vitamins riboflavin and nicotinamide are co-enzymes in lipid metabolism. To investigate associations between the maternal dietary intake of fats, riboflavin and nicotinamide, and CHD risk in the offspring. A case-control family study was conducted in 276 mothers of a child with a CHD comprising of 190 outflow tract defects (OTD) and 86 non-outflow tract defects (non-OTD) and 324 control mothers of a non-malformed child. Mothers filled out general and food frequency questionnaires at 16 months after the index-pregnancy, as a proxy of the habitual food intake in the preconception period. Nutrient intakes (medians) were compared between cases and controls by Mann-Whitney U test. Odds ratios (OR) for the association between CHDs and nutrient intakes were estimated in a logistic regression model. Case mothers, in particular mothers of a child with OTD, had higher dietary intakes of saturated fat, 30.9 vs. 29.8 g/d; P riboflavin and nicotinamide were lower in mothers of a child with an OTD than in controls (1.32 vs. 1.41 mg/d; P riboflavin (riboflavin and nicotinamide seems to contribute to CHD risk, in particular OTDs.

  6. A case of Wolf-Hirschhorn syndrome and hypoplastic left heart syndrome.

    von Elten, Kelley; Sawyer, Taylor; Lentz-Kapua, Sarah; Kanis, Adam; Studer, Matthew

    2013-06-01

    Wolf-Hirschhorn Syndrome (WHS) is a genetic syndrome that includes a typical facial appearance, mental retardation, growth delay, seizures, and congenital cardiac defects. A deletion of the terminal band of the short arm of chromosome 4, with a breakpoint at the 4p15 to 4p16 region, is the most common genetic mutation causing WHS. Congenital heart disease associated with WHS typically includes atrial and ventricular septal defects, though there are a few case reports of associated complex congenital heart disease. Here we report a case of an infant with a large 4p deletion, with a breakpoint at the 4p12 region, and hypoplasic left heart syndrome. We discuss a possible link between the size of the chromosomal deletion in WHS and the severity of the cardiac defect.

  7. Availability of cardiac surgical care in surgical correction of acquired heart defects in patients of older age group

    Kubatbek S. Urmanbetov

    2018-02-01

    Full Text Available Objective: A study of accessibility of surgical care to elderly patients (aged 60 and above with valvular heart disease has been conducted at the BSCCS "Bakulev Scientific Center of Cardiovascular Surgery» of the Ministry of Health of the Russian Federation. Methods: A retrospective analysis of structure of hospitalizations of 1726 patients, that were hospitalized between 2009 and 2010 at the BSCCS for surgical correction of valvular heart disease was performed. Results: Our study demonstrated that age, on one hand, is not the most significant barrier in the geographical accessibility of cardiac surgical care. On the other hand, it can influence the availability in general, taking into account other factors (urban / rural areas, the presence of cardiac surgical clinics, and clinical status. Provision of cardiac surgical care for patients with heart defects at the BSCCS per 1 million population varies considerably in the context of federal districts and is 0.4 for the Siberian Federal District 30 for the Central Federal District (the highest is 42 for the Moscow Region. Conclusion: Thus, our study demonstrated accessibility of surgical care for elderly patients is the highest for the urban areas with specialized cardiac surgery centers, where patients referred from rural regions

  8. Echocardiography for the diagnosis of congenital heart defects in the dog.

    Wingfield, W E; Boon, J A

    1987-05-01

    Echocardiography utilizing M-mode and real-time techniques is a safe, noninvasive diagnostic technique for use in veterinary cardiology. It provides a means to assess structural sizes and relationships and can be used to provide quantitative data for diagnosis of congenital heart disease. Although its availability is largely limited to veterinary colleges and specialty hospitals, the practicing veterinarian should know of its advantages and disadvantages. He or she is also encouraged to consider the use of ultrasound in the diagnosis and management of heart disease in all species of veterinary patients.

  9. Meier-Gorlin syndrome: Report of an additional patient with congenital heart disease

    Rabah M. Shawky

    2014-10-01

    Full Text Available We report a 7 year old female child with the classical triad of Meier-Gorlin syndrome (MGS, (microtia, absent patella and short stature. She had the characteristic facial features, with normal mentality and defective speech, skeletal abnormalities, conductive hearing loss, cystitis and normal growth hormone level. She suffered from recurrent chest infection during the first year of life which improved gradually with age. Although congenital heart is rarely observed in MGS, our patient had in addition fenestrated interatrial septal defect.

  10. Parents' perceptions during the transition to home for their child with a congenital heart defect: How can we support families of children with hypoplastic left heart syndrome?

    March, Sarita

    2017-07-01

    The aim of the study was to explore the literature related to transitions in healthcare between the hospital and home that caregivers experience with a child who has a congenital heart defect (CHD), specifically related to hypoplastic left heart syndrome (HLHS). A systematic literature review was conducted searching OVID Medline, CINAHL, and PubMed to discover the caregivers' perceptions on their transitions between hospital care and home care of their child with a CHD. Articles included those with focus on the transitions of caregivers between hospital and home care for children with CHD. Excluded articles were studies focused on adolescents, transition to adult healthcare, mortality results, other diseases associated with CHDs, comparison of CHD treatments, feasibility studies, differences in care between hospitals, home monitoring, and comparison of videoconference and telephone home communication. Ten articles were selected. Many parents voiced their concerns with feeding their child, learning medical skills and knowledge, reported a disrupted relationship between parents and their child, and identified stress and anxiety associated with taking care of a child with a CHD. There were limited studies on caregivers' transitions with a child with HLHS, but there also was limited focus on the caregivers' experiences with transitions between hospital and home care for their child with any CHD. Research on the transition experience between hospital care and home care for caregivers of children born with a CHD, and a specific focus on HLHS from the caregivers' viewpoint, would provide insight into the perspective of caregivers during the numerous transitions. © 2017 Wiley Periodicals, Inc.

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

    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.

  12. Prevalence of congenital heart disease in rural communities of pakistan

    Rizvi, S.F.U.; Mustafa, G.; Khan, M.A.; Kundi, A.

    2015-01-01

    Prevalence of congenital heart disease (CHD) is well established in most of the developed countries, where childbirth is obligatory in hospital and allied facilities. In rural Pakistan the situation is reverse, where most of deliveries take place in homes by traditional birth attendants' therefor true prevalence of CHD in our population is unknown. in rural Pakistan almost 80% children are born at home hence the figures are unknown. This study was designed, to determine the prevalence of congenital heart disease in rural Pakistan. Methods: During a cross-sectional survey of rural population belonging to major ethnic groups living in three provinces of Pakistan to determine the prevalence of rheumatic heart disease (RHD), CHD rates were calculated as a sub study. Nine thousand four hundred and seventy-six (9476) subjects of all ages were screened using cluster sampling technique. Socio-demographic variables were recorded. Auscultation and short physical examination performed for initial screening and final diagnosis was confirmed on M-mode/2D/Doppler. Results: Thirty two patients had RHD, 25 Patients identified with CHD and another 7 patients had mixed CHD and RHD. Overall prevalence for CHD was 3.4/1000. The commonest lesion was Atrial Septal Defect (ASD) 40%, Ventricular Septal Defect (VSD) 35%, Aortic Stenosis (AS) 10%, Atrio Ventricular Septal Defect (AVSD) 5%. Conclusion: This is the first study to report CHD prevalence from multiethnic representative sample from rural communities of Pakistan. Apparently CHD rate seems less compared with facility based data because records of still stillbirths are not available and autopsies are not performed as routine. Very high infant mortality from rural areas of Pakistan also favours high prevalence for CHD; however these figures represent an overall picture of CHD in a community where medical facilities are lacking. (author)

  13. Heart defects and other features of the 22q11 distal deletion syndrome

    Fagerberg, Christina Ringmann; Graakjaer, Jesper; Heinl, Ulrike D

    2013-01-01

    patients with 22q11 distal deletions, of whom two have complex congenital heart malformation, thus broadening the phenotypic spectrum. We compare cardiac malformations reported in 22q11 distal deletion to those reported in the common 22q11 deletion syndrome. We also review the literature for patients...... with 22q11 distal deletions, and discuss the possible roles of haploinsufficiency of the MAPK1 gene. We find the most frequent features in 22q11 distal deletion to be developmental delay or learning disability, short stature, microcephalus, premature birth with low birth weight, and congenital heart...... malformation ranging from minor anomalies to complex malformations. Behavioral problems are also seen in a substantial portion of patients. The following dysmorphic features are relatively common: smooth philtrum, abnormally structured ears, cleft palate/bifid uvula, micro-/retrognathia, upslanting palpebral...

  14. Kosovo’s Experience for Children with Feeding Difficulties after Cardiac Surgery for Congenital Heart Defect

    Bejiqi, Ramush; Retkoceri, Ragip; Bejiqi, Hana; Maloku, Arlinda; Vuçiterna, Armend; Zeka, Naim; Gerguri, Abdurrahim; Bejiqi, Rinor

    2017-01-01

    BACKGROUND: A feeding disorder in infancy and during childhood is a complex condition involving different symptoms such as food refusal and faddiest, both leading to a decreased food intake. AIM: We aimed to assess the prevalence and predictor factors of feeding difficulties in children who underwent cardiac open heart surgery in neonatal period and infancy. We address selected nutritional and caloric requirements for children after cardiac surgery and explore nutritional interdependence ...

  15. Increased risk of severe congenital heart defects in offspring exposed to selective serotonin-reuptake inhibitors in early pregnancy

    Knudsen, Tanja Majbrit; Hansen, Anne Vinkel; Garne, Ester

    2014-01-01

    BACKGROUND: Previous studies suggest a possible association between maternal use of selective serotonin-reuptake inhibitors (SSRIs) during early pregnancy and congenital heart defects (CHD). The purpose of this study was to verify this association by using validated data from the Danish EUROCAT...... terminated due to congenital anomalies. The study population consisted of all registered pregnancies (n = 72,280) in Funen, Denmark in the period 1995-2008. SSRI-use was assessed using The Danish National Prescription Registry, information on marital status, maternal educational level, income, and country...... of origin from Statistics Denmark was used as indicators of socioeconomic situation, and the CHD were studied in subgroups defined by EUROCAT. Logistic Regression was used to investigate the association between redeemed prescriptions for SSRIs and CHD. RESULTS: The risk of severe CHD in the offspring...

  16. Anterolateral minithoracotomy versus median sternotomy for the treatment of congenital heart defects: a meta-analysis and systematic review.

    Ding, Chao; Wang, Chunmao; Dong, Aiqiang; Kong, Minjian; Jiang, Daming; Tao, Kaiyu; Shen, Zhonghua

    2012-05-04

    Anterolateral Minithoracotomy (ALMT) for the radical correction of Congenital Heart Defects is an alternative to Median Sternotomy (MS) due to reduce operative trauma accelerating recovery and yield a better cosmetic outcome after surgery. Our purpose is to conduct whether ALMT would bring more short-term benefits to patients than conventional Median Sternotomy by using a meta-analysis of case-control study in the published English Journal. 6 case control studies published in English from 1997 to 2011 were identified and synthesized to compare the short-term postoperative outcomes between ALMT and MS. These outcomes were cardiopulmonary bypass time, aortic cross-clamp time, intubation time, intensive care unit stay time, and postoperative hospital stay time. ALMT had significantly longer cardiopulmonary bypass times (8.00 min more, 95% CI 0.36 to 15.64 min, p = 0.04). Some evidence proved that aortic cross-clamp time of ALMT was longer, yet not significantly (2.38 min more, 95% CI -0.15 to 4.91 min, p = 0.06). In addition, ALMT had significantly shorter intubation time (1.66 hrs less, 95% CI -3.05 to -0.27 hrs, p = 0.02). Postoperative hospital stay time was significantly shorter with ALMT (1.52 days less, 95% CI -2.71 to -0.33 days, p = 0.01). Some evidence suggested a reduction in ICU stay time in the ALMT group. However, this did not prove to be statistically significant (0.88 days less, 95% CI -0.81 to 0.04 days, p = 0.08). ALMT can bring more benefits to patients with Congenital Heart Defects by reducing intubation time and postoperative hospital stay time, though ALMT has longer CPB time and aortic cross-clamp time.

  17. Congenital heart disease in adolescents with gluteal muscle contracture.

    You, Tian; Zhang, Xin-tao; Zha, Zhen-gang; Zhang, Wen-tao

    2015-02-01

    Gluteal muscle contracture (GMC), presented with hip abduction and external rotation when crouching, is common in several ethnicities, particularly in Chinese. It remains unclear that the reasons why these children are weak and have no choice to accept repeated intramuscular injection. Here, we found some unique cases which may be useful to explain this question. We describe a series of special GMC patients, who are accompanied with congenital heart disease (CHD). These cases were first observed in preoperative examinations of a patient with atrial septal defect (ASD), which was proved by chest X-ray and cardiac ultrasound. From then on, we gradually identified additional 3 GMC patients with CHD. The original patient with ASD was sent to cardiosurgery department to repair atrial septal first and received arthroscopic surgery later. While the other 3 were cured postoperative of ventricular septal defect (VSD), tetralogy of fallot (TOF), patent ductus arteriosus (PDA), respectively, and had surgery directly. The study gives us 3 proposals: (1) as to CHD children, it is essential to decrease the use of intramuscular injection, (2) paying more attention to cardiac examination especially cardiac ultrasound in perioperative period, and (3) taking 3D-CT to reconstruct gluteal muscles for observing contracture bands clearly in preoperation. However, more larger series of patients are called for to confirm these findings.

  18. Patient with congenital heart malformation and infective endocarditis

    Ramírez Ortiz, Zoraida

    2014-10-01

    Full Text Available We present the case of a 17 year-old-man, with no remarkable past medical history, who had a one month history of worsening functional class, fatigability and dyspnea, in addition to fever and systemic inflammatory response syndrome (SIRS; a de-novo congenital heart malformation, situs inversus type, with levocardia and a ventricular septal defect (VSD associated with transposition of great vessels were documented. During hospitalization he received several antibiotic treatments without microbiological isolation or identification of the cause of hemodynamic decompensation. An Aspergillus endocarditis with emboli to different organs was identified in the post-mortem examination.

  19. Pattern of pediatric heart diseases in Pakistan

    Sadiq, M.; Roshan, B.; Khan, A.; Latif, F.; Bashir, I.; Sheikh, S.A.

    2002-01-01

    Objective: To assess the pattern, age distribution and relative incidence of heart diseases in pediatric patients aged 16 years and less. Design: A five-year analysis of all children undergoing echocardiography for possible heart disease in a single center. Setting: Tertiary referral center for pediatric and adult cardiac services in the central and southern Punjab, Pakistan. Patients and Methods: Data of all new children undergoing detailed echocardiography was reviewed for type of lesion age at presentation and gender. Results: over a period of five years, (may 1996 to April 2001), 7400 patients underwent echocardiography. Of these, 6620 had cardiac lesions while 780 patients were normal and excluded from the study. Of 6620 patients, 4184 (63.2%) had congenital heart defects (CHD) while 2335 (35.3%) acquired heart disease (AHD) and 101 (1.5%) were placed in miscellaneous group. Of CHD, ventricular septal defect was the most common lesion (32% of all patients with CHD), followed by atrial septal defects (13.2%) and persistent arterial dust (12.8%). Majority was males (65%) and the mean age of presentation was 5.8 years for acyanotic and 4.8 years for cyanotic heart defects. Tetralogy of fallout was the most common cyanotic lesion (16.06%) with mean age of presentation being 4.2 years. The relative incidence of patients with critical health lesions was much less and only 586 patients (14%) were under the age of one year at presentation. Children presenting less than one month of age were only 3% (127 patients). Amongst AHD, 71.5% (1670) had rheumatic heart disease (RHD) while 24.5% (572) had mycocardial disease,clinically diagnosed as myocarditis or dilated cardiomyopathy. The mean age of presentation for myocarditis was 2.3 year and majority was clustered in the months of March, April, September and October. Amongst RHD, mitral regurgitation was the commonest lesion: 681 patients(40.8%), followed by mixed lesion of mitral and aortic regurgitation in 382 patients

  20. Congenital Critical Heart Defect Screening in a Health Area of the Community of Valencia (Spain: A Prospective Observational Study

    Elena Cubells

    2018-01-01

    Full Text Available Despite the progress in the fetal echocardiographic detection of congenital critical heart defects and neonatal physical examination, a significant number of newborn infants are discharged and readmitted to the hospital in severe condition due to cardiac failure or collapse. The aim of this study was to assess the incidence of undetected critical congenital heart disease (CCHD by a pulse oximetry-screening program in the maternity wards of hospitals with Perinatal Services in a specific geographic area. This is a prospective observational study performed in in the health area corresponding to the city of Valencia. Eligible infants were consecutively admitted newborn infants in the maternities of the participating hospitals with negative fetal echocardiography after normal physical examination in the delivery room. All patients were screened following a specific pulse oximetry protocol before discharge. A total of 8856 newborn infants were screened. A total of three babies presented with severe congenital cardiac malformation and two babies presented with early onset sepsis. Sensitivity was 100% and specificity was 99.97%, with a positive predictive value of 60% and negative predictive value of 100%. Pulse oximetry screening programs in the early neonatal period constitute a valuable tool to avoid inadvertent hospital discharge of severe cardiac malformations and the subsequent life-threatening complications derived.

  1. A maternal dietary pattern characterised by fish and seafood in association with the risk of congenital heart defects in the offspring

    Obermann-Borst, S.A.; Vujkovic, M.; Vries, de J.H.M.; Wildhagen, M.F.; Looman, C.W.; Jonge, de R.; Steegers, E.A.P.; Steegers-Theunissen, R.P.M.

    2011-01-01

    Objective To identify maternal dietary patterns related to biomarkers of methylation and to investigate associations between these dietary patterns and the risk of congenital heart defects (CHDs) in the offspring. Design Case–control study. Setting Western part of the Netherlands, 2003–08.

  2. Cine magnetic resonance imaging in congenital heart disease

    Akagi, Teiji; Kiyomatsu, Yumi; Kato, Hirohisa; Abe, Toushi; Nishimura, Hiroshi; Ohtake, Hisashi; Eto, Takaharu.

    1989-01-01

    Cine magnetic resonance imaging (MRI) was performed in 33 patients aged 19 days to 18 years (mean 5.1 years), who had congenital heart disease comfirmed at echocardiography or angiography. Prior to cine MRI, gated MRI with spin echo (SE) sequence was perfomed to evaluate cardiac structure. Cine MRI was demonstrated by fast low fip angle shot imaging technique with a 30 deg flip angle, 15 msec echo time, 30∼40 msec pulse repetition time, and 128X128 acquisition matrix. Abnormalities of cardiac structure were extremely well defined in all patients. Intracardiac and intravasucular blood flow were visualized with high signal intensity area, whereas ventricular filling flow and left to right shunt flow through ventricular septal defect and atrial septal defect were visualized with low signal intensity area. However, in the patients who had severe congestive heart failure or respiratory arrhythmia, the good recording of cine MRI was not obtained because of artifacts. Gated MRI with SE sequence provides excellent visualization of fine structures, and cine MRI can provide high spatial resolution imaging of flow dynamic in a variety of congenital heart disease, noninvasively. (author)

  3. Understand Your Risk for Heart Failure

    ... Heart.org Arrhythmia About Arrhythmia Why Arrhythmia Matters Understand Your Risk for Arrhythmia Symptoms, Diagnosis & Monitoring of ... Heart Defects The Impact of Congenital Heart Defects Understand Your Risk for Congenital Heart Defects Symptoms & Diagnosis ...

  4. Echocardiographic findings in children and adolescents with heart murmurs

    Amer Yazdanparast

    2007-02-01

    Full Text Available Background: Heart disease in infants and children may often lead to rapid decompensation. Therefore, early diagnosis is essential because modern medicine and surgery can cure many heart defects. A cardiac murmur can be the first sign of a serious structural cardiac disease. Abnormal auscultatory findings, usually murmurs, are the most common reason for referral to the pediatric cardiologist. Methods: In a case series study, children and adolescents with heart murmurs, referred to or admitted at Fatemeh Zahra (S Hospital of Bushehr University of Medical Sciences, were evaluated between 2001 and 2006. The age range of participants was from birth to 18 years old. Chest x-ray, ECG, and echocardiography were done for them in addition to history taking and physical examination. Results: A total of 715 (56.2% male and 43.8% female children and adolescents were included in our study (33.6% adolescents, 27.4% neonates, 22.7% infants and 16.3% children. A total of 59% of the cases had structural heart defects (organic, 27.3% had functional murmurs, and 13.7 % had acquired diseases. The most prevalent malformations were ventricular septal defects (26.2%, tetrology of Fallot (9.1%, and patent ductus arteriosus (8.7% 23.3% of participants had growth retardation. Conclusion: Heart murmur may be the first sign of an underlying heart disease, and timely referral of children with a murmur for echocardiography is essential for diagnosis.

  5. What determines nutritional recovery in malnourished children after correction of congenital heart defects?

    Vaidyanathan, Balu; Radhakrishnan, Reshma; Sarala, Deepa Aravindakshan; Sundaram, Karimassery Ramaiyar; Kumar, Raman Krishna

    2009-08-01

    Malnutrition is common in children with congenital heart disease (CHD), especially in developing countries. To examine the impact of corrective intervention on the nutritional status of children with CHD and identify factors associated with suboptimal recovery. Consecutive patients with CHD in a tertiary center in South India were evaluated for nutritional status before and 2 years after corrective intervention. Anthropometry was performed at presentation and every 6 months for 2 years, and z scores were compared. Malnutrition was defined as a weight-for-age, height-for-age, and weight/height z score nutritional status at presentation, and height of parents and not with type of cardiac lesion, dietary intake, or socioeconomic factors. This study from South India demonstrates severe malnutrition in over half of the patients with CHD and is not always reversed by corrective surgery or intervention. Persistent malnutrition after corrective intervention is predicted by nutritional status at presentation, birth weight, and parental anthropometry.

  6. Kosovo’s Experience for Children with Feeding Difficulties after Cardiac Surgery for Congenital Heart Defect

    Bejiqi, Ramush; Retkoceri, Ragip; Bejiqi, Hana; Maloku, Arlinda; Vuçiterna, Armend; Zeka, Naim; Gerguri, Abdurrahim; Bejiqi, Rinor

    2017-01-01

    BACKGROUND: A feeding disorder in infancy and during childhood is a complex condition involving different symptoms such as food refusal and faddiest, both leading to a decreased food intake. AIM: We aimed to assess the prevalence and predictor factors of feeding difficulties in children who underwent cardiac open heart surgery in neonatal period and infancy. We address selected nutritional and caloric requirements for children after cardiac surgery and explore nutritional interdependence with other system functions. METHODS: This was a retrospective study in a tertiary referral hospital, and prior approval from the institutional ethics committee was obtained. Information for 78 children (42 male and 36 female) was taken from patients charts. Data were analysed with descriptive statistics and logistic regression. RESULTS: From a cohort of analysed children with feeding problems we have occurred in 23% of such cases. At the time of the study, refusal to eat or poor appetite was reported as a significant problem in 19 children and subnormal height and weight were recorded in 11 children. Early neonatal intervention and reoperation were identified as risk factors for latter feeding difficulties or inadequate intake. Children with feeding problems also tended to eat less than children without feeding problems. There was a trend towards more feeding problems in patients with chromosomal abnormalities or other associated anomalies. CONCLUSION: Feeding disorder is often and a frequent long-term sequel in children after neonatal or early infancy heart surgery. Patients with chromosomal and associated anomalies who underwent multiple cardiac surgeries are at risk of developing feeding difficulties. PMID:29362619

  7. Interventional Cardiology for Congenital Heart Disease.

    Kenny, Damien

    2018-05-01

    Congenital heart interventions are now replacing surgical palliation and correction in an evolving number of congenital heart defects. Right ventricular outflow tract and ductus arteriosus stenting have demonstrated favorable outcomes compared to surgical systemic to pulmonary artery shunting, and it is likely surgical pulmonary valve replacement will become an uncommon procedure within the next decade, mirroring current practices in the treatment of atrial septal defects. Challenges remain, including the lack of device design focused on smaller infants and the inevitable consequences of somatic growth. Increasing parental and physician expectancy has inevitably lead to higher risk interventions on smaller infants and appreciation of the consequences of these interventions on departmental outcome data needs to be considered. Registry data evaluating congenital heart interventions remain less robust than surgical registries, leading to a lack of insight into the longer-term consequences of our interventions. Increasing collaboration with surgical colleagues has not been met with necessary development of dedicated equipment for hybrid interventions aimed at minimizing the longer-term consequences of scar to the heart. Therefore, great challenges remain to ensure children and adults with congenital heart disease continue to benefit from an exponential growth in minimally invasive interventions and technology. This can only be achieved through a concerted collaborative approach from physicians, industry, academia and regulatory bodies supporting great innovators to continue the philosophy of thinking beyond the limits that has been the foundation of our specialty for the past 50 years. Copyright © 2018. The Korean Society of Cardiology.

  8. Classificação anatômica e correção cirúrgica da atresia pulmonar com comunicação interventricular Anatomical classification and surgical repair of the pulmonary atresia with ventricular septal defect

    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

  9. Pediatric heart surgery

    Heart surgery - pediatric; Heart surgery for children; Acquired heart disease; Heart valve surgery - children ... There are many kinds of heart defects. Some are minor, and others are more serious. Defects can occur inside the heart or in the large blood vessels ...

  10. Disorder of Sexual Development and Congenital Heart Defect in 47XYY: Clinical Disorder or Coincidence?

    Hanane Latrech

    2015-01-01

    Full Text Available Background. 47XYY syndrome is a rare sex chromosome variation characterized by an additional Y chromosome. Most patients with 47XYY karyotype have normal phenotype. This disorder seems associated with a higher risk of developing behavioral and cognitive problems, tall stature, and infertility in adulthood. Sexual development disorder is a rare finding. We report a first case with an abnormal left coronary artery originating from the pulmonary artery in a 47XYY patient. Case. A one-month-old child was referred for ectopic testis and micropenis. Physical examination revealed facial dysmorphia, micropenis, and curvature of the penis with nonpalpable testis. Laboratory tests showed decreased total testosterone and anti-Mullerian hormone (AMH levels. Blood karyotyping revealed a 47XYY chromosomal formula. At the age of 3 months, the patient developed dyspnea and tachycardia. Echocardiography revealed an anomalous left coronary artery from pulmonary artery with left ventricular dysfunction requiring surgical revascularization by direct reimplantation of the left coronary artery system. Our second case was a 3-year-old child referred for hypospadias with nonpalpable left testicle. Physical examination showed hypertelorism. Blood karyotyping revealed a 47XYY chromosomal formula. Conclusion. To our knowledge, this is the first case of 47XYY syndrome associated with this congenital heart malformation and a sexual development disorder.

  11. Association between congenital heart defects and severe infections in children with Down syndrome.

    Faria, Paula Foresti; Nicolau, Juliana Augusta Zeglin; Melek, Marina Zaponi; de Oliveira, Nanci de Santa Palmieri; Bermudez, Beatriz Elizabeth Bagatin Veleda; Nisihara, Renato Mitsunori

    2014-01-01

    There is a high prevalence of congenital heart disease (CHD) in Down syndrome (DS) patients. Children with DS and CHD also present greater susceptibility to pulmonary infections than those without CHD. To investigate the prevalence and types of CHD and their association with severe infections in children with DS in southern Brazil seen in a reference outpatient clinic. Children aged between six and 48 months with a diagnosis of DS were included consecutively in the period May 2001 to May 2012, and the presence of CHD and severe infections (pneumonia and sepsis) was investigated, classified and analyzed. A total of 127 patients were included, of whom 89 (70.1%) had some type of CHD, 33 (37.7%) of them requiring surgical correction. Severe infections (pneumonia and sepsis) were seen in 23.6% and 5.5%, respectively. Of the cases of pneumonia, 70% had associated CHD (p=0.001) and of those with sepsis, 85% presented CHD (p=0.001). Our study showed a high prevalence of CHD and its association with severe infections in children with DS seen in southern Brazil. Copyright © 2013 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  12. Proximity to Pediatric Cardiac Surgical Care among Adolescents with Congenital Heart Defects in 11 New York Counties.

    Sommerhalter, Kristin M; Insaf, Tabassum Z; Akkaya-Hocagil, Tugba; McGarry, Claire E; Farr, Sherry L; Downing, Karrie F; Lui, George K; Zaidi, Ali N; Van Zutphen, Alissa R

    2017-11-01

    Many individuals with congenital heart defects (CHDs) discontinue cardiac care in adolescence, putting them at risk of adverse health outcomes. Because geographic barriers may contribute to cessation of care, we sought to characterize geographic access to comprehensive cardiac care among adolescents with CHDs. Using a population-based, 11-county surveillance system of CHDs in New York, we characterized proximity to the nearest pediatric cardiac surgical care center among adolescents aged 11 to 19 years with CHDs. Residential addresses were extracted from surveillance records documenting 2008 to 2010 healthcare encounters. Addresses were geocoded using ArcGIS and the New York State Street and Address Maintenance Program, a statewide address point database. One-way drive and public transit time from residence to nearest center were calculated using R packages gmapsdistance and rgeos with the Google Maps Distance Matrix application programming interface. A marginal model was constructed to identify predictors associated with one-way travel time. We identified 2522 adolescents with 3058 corresponding residential addresses and 12 pediatric cardiac surgical care centers. The median drive time from residence to nearest center was 18.3 min, and drive time was 30 min or less for 2475 (80.9%) addresses. Predicted drive time was longest for rural western addresses in high poverty census tracts (68.7 min). Public transit was available for most residences in urban areas but for few in rural areas. We identified areas with geographic barriers to surgical care. Future research is needed to determine how these barriers influence continuity of care among adolescents with CHDs. Birth Defects Research 109:1494-1503, 2017.© 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

  13. Anterolateral minithoracotomy versus median sternotomy for the treatment of congenital heart defects: a meta-analysis and systematic review

    Ding Chao

    2012-05-01

    Full Text Available Abstract Background Anterolateral Minithoracotomy (ALMT for the radical correction of Congenital Heart Defects is an alternative to Median Sternotomy (MS due to reduce operative trauma accelerating recovery and yield a better cosmetic outcome after surgery. Our purpose is to conduct whether ALMT would bring more short-term benefits to patients than conventional Median Sternotomy by using a meta-analysis of case–control study in the published English Journal. Methods 6 case control studies published in English from 1997 to 2011 were identified and synthesized to compare the short-term postoperative outcomes between ALMT and MS. These outcomes were cardiopulmonary bypass time, aortic cross-clamp time, intubation time, intensive care unit stay time, and postoperative hospital stay time. Results ALMT had significantly longer cardiopulmonary bypass times (8.00 min more, 95% CI 0.36 to 15.64 min, p = 0.04. Some evidence proved that aortic cross-clamp time of ALMT was longer, yet not significantly (2.38 min more, 95% CI −0.15 to 4.91 min, p = 0.06. In addition, ALMT had significantly shorter intubation time (1.66 hrs less, 95% CI −3.05 to −0.27 hrs, p = 0.02. Postoperative hospital stay time was significantly shorter with ALMT (1.52 days less, 95% CI −2.71 to −0.33 days, p = 0.01. Some evidence suggested a reduction in ICU stay time in the ALMT group. However, this did not prove to be statistically significant (0.88 days less, 95% CI −0.81 to 0.04 days, p = 0.08. Conclusion ALMT can bring more benefits to patients with Congenital Heart Defects by reducing intubation time and postoperative hospital stay time, though ALMT has longer CPB time and aortic cross-clamp time.

  14. Clinical pattern of heart diseases in children

    Ejaz, M.S.; Billoo, A.G.

    2000-01-01

    This study was done to determine various causes and clinical presentation of heart disease in children. It was a prospective hospital study conducted in Department of Pediatrics Civil Hospital, Karachi from August 1995 to February 1996. In this study, 70 patients of heart disease upto 12 years of age were inducted. There were 33 (47.14%) cases of congenital heart diseases and 37 (52.85%) cases of acquired heart diseases. The age distribution showed that heart disease was more frequent between 0-11 months of age (41.42%). Congenital heart diseases were also frequent between 0-11 months (28.57%). On the other hand acquired heart diseases were more common between 6-12 years (22.85%). In this study the males were predominantly involved, the male to female ratio was 1.05:1. In congenital heart disease it was 1.3:1 and in acquired heart diseases it was 0.85:1. Ventricular septal defect was the commonest congenital lesion reported (20%). Rheumatic fever and viral myocarditis were two frequently occurring acquired heart-diseases 17.14% each. The common presentation of heart diseases were respiratory distress (94.28%), fever (90%), feeding difficulty (57.14%) and failure to thrive (34.28%). In case of rheumatic fever, chorea was present in 8.57%, arthritis in 11.42% and S/C nodules (2.85%) cases respectively. The early management of the problem may help in decreasing morbidity and mortality due to these disease in children. Prenatal detection of congenital cardiac lesions by fetal echocardiography in high risk pregnancies, early intervention in neonatal period and counseling of the parents may help in prevention of congenital heart diseases in children. Primary prevention of rheumatic fever can be achieved by early diagnosis and treatment of streptococcal throat infection. (author)

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

    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.

  16. Upward creep of the heart: A frequent source of false-positive reversible defects during thallium-201 stress-redistribution SPECT

    Friedman, J.; Van Train, K.; Maddahi, J.; Rozanski, A.; Prigent, F.; Bietendorf, J.; Waxman, A.; Berman, D.S.

    1989-01-01

    A new cause of artifactual 201 Tl defects on single photon emission computed tomography (SPECT) termed upward creep of the heart is described. In 102 consecutive patients undergoing 201 Tl SPECT, 30 (29%) demonstrated upward creep defined by an upward movement of the heart of greater than or equal to 2 pixels during acquisition. In 45 consecutive patients with a less than 5% likelihood of coronary artery disease, 17 (38%) had upward creep. Of these nine had reversible 201 Tl defects localized to the inferior and basal inferoseptal walls, while none of the 28 without upward creep had defects. The 17 low likelihood patients with upward creep had longer exercise duration and higher peak heart rate than those without upward creep. In five additional low likelihood patients with upward creep in whom imaging was immediately repeated, the upward creep pattern disappeared on the repeated images. After we changed our test protocol to begin imaging 15 min postexercise, only five (14%) of 36 low likelihood patients tested demonstrated upward creep. Upward creep is probably related to a transient increase in mean total lung volume early following exhaustive exercise, resulting in a mean lower position of the diaphragm (and thus the heart) at the beginning of imaging. The frequency of this source of false-positive 201 Tl studies can be reduced by delaying SPECT acquisition until 15 min postexercise

  17. Nkx2-5 Mutations in Patients With Nonsyndromic Congenital Heart Disease

    Fariborz Soheili

    2016-01-01

    Full Text Available Background Congenital heart diseases (CHD are the most common of all birth defects, affecting nearly 0.9% of all live births. Nkx2-5 mutations were reported to cause CHD but data in Kurdish populations of Iran are limited. Objectives In this experimental study, we performed high resolution melt (HRM mutation scanning of Nkx2-5 exons of non-syndrome patients. Patients and Methods Thirty nine patients with atrial septal defect and 57 patients with ventricular septal defect, 4 patients possessing both defects as case groups and 50 healthy controls. Then we grouped samples according to HRM graph and sequenced several samples from each group. Results HRM analysis showed 2 deviated curves for exon 1 and one group for exon 2A and exon 2B. Then, 2 samples of exon 1 that showed different HRM curves, 3 samples of another group from this exon and 5 samples of exon 2A, 2B and healthy controls were randomly sequenced. The results of sequencing confirmed the HRM analysis, and one polymorphism (A65G was identified in 2 atrial septal defects with deviated curves. Conclusions The environmental and effective factors on the heart development within embryonic evolution as well as the possibility of the existence of the mutation in coding genes of the other cardiac transcription factors such as GATA4 and TBX5 can be the reasons for the lack of the pathogenic mutation in this study. It is suggested in further related studies to investigate normal and abnormal cardiac tissue samples of these studied patients and coding genes of the other cardiac transcription factors.

  18. Combining rhinoplasty with septal perforation repair.

    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.

  19. Cine magnetic resonance imaging for evaluation of cardiac structure and flow dynamics in congenital heart disease

    Akagi, Teiji; Kiyomatsu, Yumi; Ohara, Nobutoshi; Takagi, Junichi; Sato, Noboru; Kato, Hirohisa; Eto, Takaharu.

    1989-01-01

    Cine magnetic resonance imaging (Cine MRI) was performed in 20 patients aged 19 days to 13 years (mean 4.0 years), who had congenital heart disease confirmed at echocardiography or angiography. Prior to cine MRI, gated MRI was performed to evaluate for cardiac structure. Cine MRI was demonstrated by fast low fip angle shot imaging technique with a 30deg flip angle, 15 msec echo time, 30-40 msec pulse repetition time, and 128 x 128 acquisition matrix. Abnormalities of cardiac structure were extremely well defined in all patients by gated MRI. Intracardiac or intravascular blood flow were visualized in 17 (85%) of 20 patients by cine MRI. Left to right shunt flow through ventricular septal defect, atrial septal defect, and endocardial cushion defect were visualized with low signal intensity area. Low intensity jets flow through the site of re-coarctation of the aorta were also visualized. However, the good recording of cine MRI was not obtained because of artifacts in 3 of 20 patients (15%) who had severe congestive heart failure or respiratory arrhythmia. Gated MRI provides excellent visualization of fine structure, and cine MRI can provide high spatial resolution imaging of flow dynamic in a variety of congenital heart disease, noninvasively. (author)

  20. Syndromic Hirschsprung's disease and associated congenital heart disease: a systematic review.

    Duess, Johannes W; Puri, Prem

    2015-08-01

    Hirschsprung's disease (HD) occurs as an isolated phenotype in 70% of infants and is associated with additional congenital anomalies or syndromes in approximately 30% of patients. The cardiac development depends on neural crest cell proliferation and is closely related to the formation of the enteric nervous system. HD associated with congenital heart disease (CHD) has been reported in 5-8% of cases, with septation defects being the most frequently recorded abnormalities. However, the prevalence of HD associated with CHD in infants with syndromic disorders is not well documented. This systematic review was designed to determine the prevalence of CHD in syndromic HD. A systematic review of the literature using the keywords "Hirschsprung's disease", "aganglionosis", "congenital megacolon", "congenital heart disease" and "congenital heart defect" was performed. Resulting publications were reviewed for epidemiology and morbidity. Reference lists were screened for additional relevant studies. A total of fifty-two publications from 1963 to 2014 reported data on infants with HD associated with CHD. The overall reported prevalence of HD associated with CHD in infants without chromosomal disorders was 3%. In infants with syndromic disorders, the overall prevalence of HD associated with CHD ranged from 20 to 80 % (overall prevalence 51%). Septation defects were recorded in 57% (atrial septal defects in 29%, ventricular septal defects in 32%), a patent ductus arteriosus in 39%, vascular abnormalities in 16%, valvular heart defects in 4% and Tetralogy of Fallot in 7%. The prevalence of HD associated with CHD is much higher in infants with chromosomal disorders compared to infants without associated syndromes. A routine echocardiogram should be performed in all infants with syndromic HD to exclude cardiac abnormalities.

  1. Germline mutations in ABL1 cause an autosomal dominant syndrome characterized by congenital heart defects and skeletal malformations.

    Wang, Xia; Charng, Wu-Lin; Chen, Chun-An; Rosenfeld, Jill A; Al Shamsi, Aisha; Al-Gazali, Lihadh; McGuire, Marianne; Mew, Nicholas Ah; Arnold, Georgianne L; Qu, Chunjing; Ding, Yan; Muzny, Donna M; Gibbs, Richard A; Eng, Christine M; Walkiewicz, Magdalena; Xia, Fan; Plon, Sharon E; Lupski, James R; Schaaf, Christian P; Yang, Yaping

    2017-04-01

    ABL1 is a proto-oncogene well known as part of the fusion gene BCR-ABL1 in the Philadelphia chromosome of leukemia cancer cells. Inherited germline ABL1 changes have not been associated with genetic disorders. Here we report ABL1 germline variants cosegregating with an autosomal dominant disorder characterized by congenital heart disease, skeletal abnormalities, and failure to thrive. The variant c.734A>G (p.Tyr245Cys) was found to occur de novo or cosegregate with disease in five individuals (families 1-3). Additionally, a de novo c.1066G>A (p.Ala356Thr) variant was identified in a sixth individual (family 4). We overexpressed the mutant constructs in HEK 293T cells and observed increased tyrosine phosphorylation, suggesting increased ABL1 kinase activities associated with both the p.Tyr245Cys and p.Ala356Thr substitutions. Our clinical and experimental findings, together with previously reported teratogenic effects of selective BCR-ABL inhibitors in humans and developmental defects in Abl1 knockout mice, suggest that ABL1 has an important role during organismal development.

  2. Germline mutations in ABL1 cause an autosomal dominant syndrome characterized by congenital heart defects and skeletal malformations

    Wang, Xia; Charng, Wu-Lin; Chen, Chun-An; Rosenfeld, Jill A.; Shamsi, Aisha Al; Al-Gazali, Lihadh; McGuire, Marianne; Mew, Nicholas Ah; Arnold, Georgianne L.; Qu, Chunjing; Ding, Yan; Muzny, Donna M.; Gibbs, Richard A.; Eng, Christine M.; Walkiewicz, Magdalena; Xia, Fan; Plon, Sharon E.; Lupski, James R.; Schaaf, Christian P.; Yang, Yaping

    2017-01-01

    ABL1 is a proto-oncogene well known as part of the fusion gene BCR-ABL in the Philadelphia chromosome of leukemia cancer cells1. Inherited germline ABL1 changes have not been associated with genetic disorders. Here we report ABL1 germline variants co-segregating with an autosomal dominant disorder characterized by congenital heart disease, skeletal abnormalities, and failure to thrive. The variant c.734A>G (p.Tyr245Cys) was found as de novo or co-segregating with disease in five individuals (families 1-3). Additionally, a de novo c.1066G>A (p.Ala356Thr) variant was identified in the sixth individual (family 4). We overexpressed the mutant constructs in HEK 293T cells and observed increased tyrosine phosphorylation, suggesting increased ABL1 kinase activities associated with both p.Tyr245Cys and p.Ala356Thr substitutions. Our clinical and laboratory findings, together with previously reported teratogenic effects of selective BCR-ABL inhibitors in humans2-5 and developmental defects in Abl1 knock-out mice6,7, suggest ABL1 plays an important role during organismal development. PMID:28288113

  3. Well-being in mothers of children with congenital heart defects: a 3-year follow-up.

    Grønning Dale, Maria T; Solberg, Øivind; Holmstrøm, Henrik; Landolt, Markus A; Eskedal, Leif T; Vollrath, Margarete E

    2013-10-01

    This prospective case-cohort study compared subjective well-being (SWB) among mothers whose children had various degrees of congenital heart defects (CHD) with mothers of children without CHD (controls). Nationwide CHD registry data were linked to data collected from the Norwegian Mother and Child Cohort Study at gestational week 30, 6 months, and 36 months postpartum. A total of 175 mothers of children with mild, moderate, and severe CHD were identified in a cohort of 44,144 mothers. The SWB index was operationalized by means of three subscales: a cognitive aspect, positive affect, and negative affect. Mothers of children with severe CHD reported significantly lower SWB than the controls at 6 months postpartum (p = .003), with further decrease in SWB at 36 months postpartum (p = .001). SWB levels in the mild and moderate CHD group did not deviate significantly from controls. The findings suggest a pattern in which all stressors concerning the severely ill child lead to significant deterioration of maternal well-being. Having a child with mild or moderate CHD, which is a less severe and shorter-term stressor, did not reduce mothers' well-being.

  4. Assessment of congenital heart disease (CHD): Is there a role for fetal magnetic resonance imaging (MRI)?

    Manganaro, L. [Department of Radiological Sciences, UMBERTO I Hospital, SAPIENZA University of Rome, Viale del Policlinico 155, 00161 Rome (Italy); Savelli, S. [Department of Radiological Sciences, UMBERTO I Hospital, SAPIENZA University of Rome, Viale del Policlinico 155, 00161 Rome (Italy)], E-mail: sarasavelli@hotmail.it; Di Maurizio, M.; Perrone, A.; Francioso, A.; La Barbera, L.; Totaro, P.; Fierro, F.; Tomei, A.; Coratella, F. [Department of Radiological Sciences, UMBERTO I Hospital, SAPIENZA University of Rome, Viale del Policlinico 155, 00161 Rome (Italy); Giancotti, A. [Department of Gynaecological Sciences, UMBERTO I Hospital, SAPIENZA University of Rome, Viale del Policlinico 155, 00161 Rome (Italy); Ballesio, L. [Department of Radiological Sciences, UMBERTO I Hospital, SAPIENZA University of Rome, Viale del Policlinico 155, 00161 Rome (Italy); Ventriglia, F. [Department of Pediatric Cardiology, UMBERTO I Hospital, SAPIENZA University of Rome, Viale del Policlinico 155, 00161 Rome (Italy)

    2009-10-15

    Purpose: To review our experience with fetal magnetic resonance imaging (MRI) to evaluate congenital heart disease (CHD). Methods: We performed fetal MRI in 32 fetuses with an echocardiographically assessed CHD. Both direct and indirect signs of CHD were investigated. Direct signs considered were: morpho-volumetric abnormalities of the heart; malrotations; ventricular and atrial septal defects; anomalies of the origin, size and course of the great arteries. Indirect signs considered were: difficulty to recognize a 'normal' anatomical structures in the reference projections; increase of the vascular size before a stenosis; hypertrophy of the papillary muscles; cardiomegaly and pericardial effusion. All MRI findings were compared with postnatal or autoptic findings. Results: MRI allowed the CHD to be visualised by direct signs in 17 fetuses, indirect signs in 5 and both direct and indirect signs in 9 fetuses, excluding the prenatal echocardiographic suspect of hypoplastic left heart syndrome in 1 fetus. Postnatal echocardiograms or autoptic findings confirmed a normal heart in 1 fetus and CHD in 31 fetuses including a single cardiac anomaly or syndrome in 19 fetuses, 2 associated cardiac abnormalities in 11 and 3 cardiac anomalies in 1 fetus. However, in 2 fetuses MRI detected a ventricular septal defect successively disclosed by gold standard. Conclusions: MRI is a promising method for further assessment of the cardiovascular pathologies diagnosed by echocardiography, and may be a valuable tool in assessing associated extracardiac anomalies.

  5. Profile of congenital heart disease and correlation to risk adjustment for surgery; an echocardiographic study

    Akhtar, K.; Ahmed, W.

    2008-01-01

    To determine the pattern and profile of Congenital Heart Diseases (CHD) in paediatric patients (age 1 day to 18 years) presenting to a paediatric tertiary referral centre and its correlation to risk adjustment for surgery for congenital heart disease. Over a period of 6 months, 1149 cases underwent 2-D echocardiography. It was a non-probability purposive sampling. This study showed 25% of all referrals had normal hearts. A male preponderance (38%) was observed from 1 year to 5 years age group. Nineteen percent of the cases were categorized as cyanotic CHD with the remaining as acyanotic variety. Tetralogy of Fallot (TOF) represented 10%, Ventricular Septal Defects (VSD) 24%, followed by Patent Ductus Arteriosus (PDA) and Atrial Septal Defect (ASD), which comprised 6.6% and 6.5% respectively. VSD was the most common association in patients with more complex CHD (10%) followed by PDA in 3% and ASD in 1.2% of the cases. Most of the cases were category 2 in the RACHS-1 scoring system. VSD and TOF formed the major groups of cases profiled. Most of the cases recommended for surgery for congenital heart disease belonged to the risk category 2 (28.1%) followed by the risk category 1 (12.7%) of the RACHS-1 scoring system. (author)

  6. A case of residual inferior sinus venosus defect after ineffective surgical closure.

    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.

  7. Dynamic three-dimensional display of common congenital cardiac defects from reconstruction of two-dimensional echocardiographic images.

    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.

  8. Retrospective review of congenital heart disease in 976 dogs.

    Oliveira, P; Domenech, O; Silva, J; Vannini, S; Bussadori, R; Bussadori, C

    2011-01-01

    Knowledge of epidemiology is important for recognition of cardiovascular malformations. Review the incidence of congenital heart defects in dogs in Italy and assess breed and sex predispositions. Nine hundred and seventy-six dogs diagnosed with congenital heart disease (CHD) of 4,480 dogs presented to Clinica Veterinaria Gran Sasso for cardiovascular examination from 1997 to 2010. A retrospective analysis of medical records regarding signalment, history, clinical examination, radiography, electrocardiography, echocardiography, angiography, and postmortem examination was performed. Breed and sex predisposition were assessed with the odds ratio test. CHD was observed in 21.7% of cases. A total of 1,132 defects were observed with single defects in 832 cases (85%), 2 concurrent defects in 132 cases (14%), and 3 concurrent defects in 12 cases (1%). The most common defects were pulmonic stenosis (PS; 32.1%), subaortic stenosis (SAS; 21.3%), and patent ductus arteriosus (20.9%), followed by ventricular septal defect (VSD; 7.5%), valvular aortic stenosis (AS; 5.7%), and tricuspid dysplasia (3.1%). SAS, PS, and VSD frequently were associated with other defects. Several breed and sex predispositions were identified. The results of this study are in accordance with previous studies, with slight differences. The breed and sex predilections identified may be of value for the diagnosis and screening of CHD in dogs. Additionally, the relatively high percentage of concurrent heart defects emphasizes the importance of accurate and complete examinations for identification. Because these data are from a cardiology referral center, a bias may exist. Copyright © 2011 by the American College of Veterinary Internal Medicine.

  9. Does maternal environmental tobacco smoke interact with social-demographics and environmental factors on congenital heart defects?

    Liu, Xiaoqing; Nie, Zhiqiang; Chen, Jimei; Guo, Xiaoling; Ou, Yanqiu; Chen, Guanchun; Mai, Jinzhuang; Gong, Wei; Wu, Yong; Gao, Xiangmin; Qu, Yanji; Bell, Erin M; Lin, Shao; Zhuang, Jian

    2018-03-01

    Congenital heart defects (CHDs) are a major cause of death in infancy and childhood. Major risk factors for most CHDs, particularly those resulting from the combination of environmental exposures with social determinants and behaviors, are still unknown. This study evaluated the main effect of maternal environmental tobacco smoke (ETS), and its interaction with social-demographics and environmental factors on CHDs in China. A population-based, matched case-control study of 9452 live-born infants and stillborn fetuses was conducted using the Guangdong Registry of Congenital Heart Disease data (2004-2014). The CHDs were evaluated by obstetrician, pediatrician, or cardiologist, and confirmed by cardia tomography/catheterization. Controls were randomly chosen from singleton newborns without any malformation, born in the same hospital as the cases and 1:1 matched by infant sex, time of conception, and parental residence (same city and town to ensure sufficient geographical distribution for analyses). Face-to-face interviews were conducted to collect information on demographics, behavior patterns, maternal disease/medication, and environmental exposures. Conditional logistic regression was used to estimate odds ratios and 95% confidence intervals of ETS exposure on CHDs while controlling for all risk factors. Interactive effects were evaluated using a multivariate delta method for maternal demographics, behavior, and environmental exposures on the ETS-CHD relationship. Mothers exposed to ETS during the first trimester of pregnancy were more likely to have infants with CHD than mothers who did not (aOR = 1.44, 95% CI 1.25-1.66). We also observed a significant dose-response relationship when mothers were exposed to ETS and an increasing number of risk factors and CHDs. There were greater than additive interactions for maternal ETS and migrant status, low household income and paternal alcohol consumption on CHDs. Maternal low education also modified the ETS

  10. [Combined G-banded karyotyping and multiplex ligation-dependent probe amplification for the detection of chromosomal abnormalities in fetuses with congenital heart defects].

    Liu, Yang; Xie, Jiansheng; Geng, Qian; Xu, Zhiyong; Wu, Weiqin; Luo, Fuwei; Li, Suli; Wang, Qin; Chen, Wubin; Tan, Hongxi; Zhang, Hu

    2017-02-10

    To assess the value of G-banded karyotyping in combination with multiplex ligation-dependent probe amplification (MLPA) as a tool for the detection of chromosomal abnormalities in fetuses with congenital heart defects. The combined method was used to analyze 104 fetuses with heart malformations identified by ultrasonography. Abnormal findings were confirmed with chromosomal microarray analysis (CMA). Nineteen (18%) fetuses were found to harbor chromosomal aberrations by G-banded karyotyping and MLPA. For 93 cases, CMA has detected abnormalities in 14 cases including 10 pathogenic copy number variations (CNVs) and 4 CNVs of uncertain significance (VOUS). MLPA was able to detect all of the pathogenic CNVs and 1 VOUS CNV. Combined use of G-banded karyotyping and MLPA is a rapid, low-cost and effective method to detect chromosomal abnormalities in fetuses with various heart malformations.

  11. Three-dimensional surface reconstruction imaging for evaluation of congenital heart disease from ECG-triggered MR images

    Vannier, M.W.; Laschinger, J.; Knapp, R.H.; Gutierrez, F.R.; Gronnemeyer, S.A.

    1987-01-01

    Three-dimensional surface reconstruction images of the heart and great vessels were produced from contiguous sequences of electrocardiographically triggered MR images in 25 patients with congenital heart disease and in three healthy subjects. The imaging data were semiautomatically processed to separate the epicardial and endocardial surfaces and to define the outline of the enclosed blood volumes on a section by section basis. Images were obtained at 5-mm intervals in patients aged 3 months to 30 years with anomalies of the great vessels, tetralogy of Fallot, septal defects, pulmonary atresia, and other congenital heart malformations. The results were used to facilitate the surgical treatment of these patients and were compared with echocardiographic and cineradiographic studies, and with surgical findings or pathologic specimens. These surface reconstruction images were useful for communicating the results of diagnostic examinations to cardiac surgeons, for sizing and location of intracardiac defects, for imaging the pulmonary venous drainage, and for assessing regional and global function

  12. Estudo comparativo entre a miniesternotomia em "L" invertido e esternotomia longitudinal total na correção cirúrgica da comunicação interatrial Comparative study between inverted "L" mini-sternotomy and complete sternotomy for the surgical treatment of the atrial septal defect (ASD

    Luiz Cláudio Nery Sampaio

    2005-03-01

    Full Text Available OBJETIVO: Comparar os resultados obtidos entre duas vias de acesso cirúrgico em pacientes submetidos à correção cirúrgica de comunicação interatrial (CIA. MÉTODO: Foram distribuídos 20 pacientes, com média de idade de 24,1±14,2 anos, em dois grupos. No grupo A, 10 pacientes (80% do sexo feminino, com média de idade de 20,9 ± 12,0 anos foram submetidos à correção da CIA por meio de uma esternotomia longitudinal total. No grupo B, 10 pacientes (80% do sexo feminino, com média de idade de 27,4 ± 16,1 anos foram submetidos à correção da CIA através de miniesternotomia em "L" invertido. Foi considerado significativo p OBJECTIVE: To compare the results obtained from two distinct surgical approaches in patients undergoing surgical correction of an atrial septal defect (ASD. METHOD: The study series consisted of 20 patients, with a mean age of 24.1±14.2 years, distributed in two groups. In group A, 10 patients (80% female, with a mean age of 20.9±12.0 years underwent surgical correction of ASD through a complete sternotomy. In group B, 10 patients (80% female, with mean age of 27.4±16.1 years, were operated through an inverted "L" mini-sternotomy. A p-value < 0.05 was considered statistically significant. RESULTS: There was no statistical significance for demographics variables, duration of surgery, cardiopulmonary bypass time, clamping time, amount of cardioplegia administered, thoracic drainage, ICU stay, duration of mechanical ventilatory support, amount of blood and its components transfused, arrhythmia or pacemaker usage. There was statistical significance (p= 0.00001 between incision size performed by complete sternotomy (group A and inverted "L" mini-sternotomy (group B, with mean incision size of 15.7±0.8 e 6.8±0.6cm, respectively. There was a difference in the mean hospital stay between the groups but without statistical significance (7.5±1.6 days in group A and 6.4±1.3 days in group B, with a p-value = 0.12. There

  13. Expression of Id2 in the Second Heart Field and Cardiac Defects in Id2 Knock-Out Mice

    Jongbloed, M. R. M.; Vicente-Steijn, R.; Douglas, Y. L.; Wisse, L. J.; Mori, K.; Yokota, Y.; Bartelings, M. M.; Schalij, M. J.; Mahtab, E. A.; Poelmann, R. E.; Gittenberger-De Groot, A. C.

    2011-01-01

    The inhibitor of differentiation Id2 is expressed in mesoderm of the second heart field, which contributes myocardial and mesenchymal cells to the primary heart tube. The role of Id2 in cardiac development is insufficiently known. Heart development was studied in sequential developmental stages in

  14. Diagnosis of common congenital heart anomalies in the dog using survey and nonselective contrast radiography

    Stickle, R.L.; Anderson, L.K.

    1987-01-01

    The most common canine congenital heart anomalies include patient ductus arteriosus, ventricular septal defects, tetralogy of Fallot, pulmonic stenosis, and aortic stenosis. Survey radiography and nonselective (venous) angiography can allow the practicing veterinarian to confirm the diagnosis in many of these patients. Typical radiographic findings using these diagnostic procedures are reviewed. Nonselective angiocardiography is a relatively easy, rapid, and noninvasive procedure which can be performed using conventional equipment. The major disadvantage of this special procedure is that the superimposition of opacified structures can make the identification of some left-to-right shunts difficult. Dilution of contrast medium can occur if a rapid bolus injection is not made

  15. Down's syndrome with Ventricular septal Defect (VsD)

    InTRodUcTIon. Down's syndrome (DS) (Trisomy 21) occurs in about one in 800 live births without predilection for race or socioeconomic class with a male to female ratio of. 1:1. It is the most common chromosomal abnormality in newborns and one of the most frequent genetic causes of mild to moderate mental retardation1, ...

  16. Natural history of ventricular septal defects in Nigerian children

    Most (35 – 57.4%) of the patients were female, their mean age at presentation was 11.2±5.2 months, and the most common type of VSD ..... 26. 13 (50.0). Nutritional status. Malnutrition. 27. 8 (29.6). 0.104. Normal nutrition. 28. 19 (67.9). VSD type. Muscular. 17. 14 (82.4). 0.003. Perimembranous. 39. 14 (35.9). Subaortic. 5. 0.

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

    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.

  18. Tratamento das cardiopatias congênitas em Sergipe: proposta de racionalização dos recursos para melhorar a assistência Heart defects treatment in Sergipe: propose of resources' rationalization to improve care

    Debora Cristina Fontes Leite

    2012-06-01

    postoperative diagnosis was more frequent closure of the interventricular communication (20.5%, closure of patent ductus arteriosus (20.2% and atrial septal defect (19%. There was a statistically significant correlation between the expected mortality RACHS-1 and observed in the sample. The evaluation of RACHS-1 as a predictor of hospital mortality by ROC curve showed area of 0.860 95% CI 0.818 to 0.902 with P <0.0001. CONCLUSION: The results of this study indicate that the centralization and organization of existing resources are needed to improve the performance of surgical correction of congenital heart diseases.

  19. Septal graft in laryngeal reconstruction

    Bahannan, Abdulrahman; Slavicek, A.; Taudy, M.; Chovanec, M.

    2006-01-01

    A 62-year-old woman presented with symptoms of dyspnea. Ultrasonography and computed tomography examinations revealed mass extending from the cricoid cartilage to the left lobe of thyroid gland and thyroid cartilage. Cytology revealed possibility of cartilaginous origin, which was proven to be chondrosarcoma (Grade 1) from the biopsy specimen obtained during panendosopy. She underwent one stage radical resection and immediate reconstruction of laryngeal skeleton defect by mucocartilaginous graft from the nasal septum. Her postoperative course was optimal with preservation of the laryngeal functions. Twenty-eight months postoperatively, she had to undergo total laryngectomy as a salvage procedure for the advanced local recurrence. We report on the relatively easy technique for functional reconstruction of the large laryngeal defect with the employment cartilage graft from the nasal septum. (author)

  20. Application of muscular ventricular septal defect occluders in the therapy of uderweight infants and young children with patent dutus arterriosus%应用肌部室间隔缺损封闭器封堵低体质量婴幼儿动脉导管未闭

    戴辰程; 郭保静; 金梅

    2011-01-01

    Objective: To evaluate the safety and advantage of application of muscular ventricular septal defect in the therapy of uderweight infants and young children with patent dutus arterriosus. Methods: Nine uderweight infants and young children (1 male and 8 fomale) with patent dutus arterriosus, aged from 6 to 21 months and weighted from 5. 5 to 10kg,underwent transcathter intervention with muscular VSD occluders from October2010 to June 2011. All patients accept transthoracic echocardiography,cardiac catheterization and angiocardiography. Results: 8 patients were associated with pulmonary hypertension. There are 2,5,and 1 patients were diganosed as mild,middle and severe pulmonary hypertension respectively. QP/QS ranged from 1.94 to 2. 87 and total pulmonary resistance index ranged from 1. 82 to 4. 7 Wood. M2. Angiocardiography indicated there were 4 large PDAs, 1 PDA with large infundibulum,and 4 PDAs with short and small infundibulum,one of which was right aortic arch and left PDA. Nine PDAs were successfully closed with muscular ventricular septal defect occluders. Pulmonary tension decreased to normal or nearly normal. No residual shunt, occluders falling off,vascular injury,or iatrogenic stenosis of pulmonary artery and descending aorta were found. 3 of 4 patients with mitral regurgitation relieved. Conclusion: Muscular VSD occluder is available and effective to close large PDA,PDA with short and small infundibulum or PDA which can not be successfully closed with Amplatzer PDA occluders in uderweight infants and young children.%目的:探讨肌部室间隔缺损(VSD)封闭器在低体质量婴幼儿动脉导管未闭(PDA)介入治疗中临床应用的安全性及优点.方法:2010年10月至2011年6月,应用肌部VSD封闭器对9例低体质量婴幼儿PDA进行介入治疗,男性1例,女性8例,年龄6-21个月,体质量5.5 ~10 kg.本组病例均行经胸超声心动图、心导管检查及造影检查.结果:合并肺动脉高压者8例,

  1. Evaluation of congenital heart disease by three-dimensional MR imaging

    Vannier, M.W.; Gutierrez, F.R.; Canter, C.E.; Yoffie, R.L.; Hildebolt, C.F.

    1988-01-01

    In an evaluation of the detectability of intra-and extracardiac morphologic defects with electrocardiographically gated magnetic resonance (MR) imaging, 100 patients were studied, including ten without and 90 with abnormalities. The abnormalities included septal defects, tetrology of Fallot, pulmonary atresia, transposition of the great vessels, and others. The patients with abnormalities were studied angiographically and with echocardiographic analyses. Two radiologists with cardiac MR imaging experience evaluated the scans in a blinded fashion, and the results were analyzed by means of receiver operating characteristic analysis. The diagnostic value of routine two-dimensional cardiac MR imaging was compared with that of three-dimensional reconstruction imaging and with the results of cardiac catheterization and echocardiography. The reported sensitivity and specificity of echocardiography in the detection of congenital heart disease is comparable to that of MR imaging. The differences in diagnostic value between various modalities for the imaging of congenital heart disease may be determined from the results of the preliminary series

  2. When Your Child Needs a Heart Transplant

    ... transplant. Why Do Kids Need Heart Transplants? A child's heart might not work right for many reasons. Sometimes, babies are born with heart defects (malformations) that cause their hearts to fail. These defects are the ...

  3. 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

    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.

  4. Molecular Diffusion through Cyanobacterial Septal Junctions.

    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

  5. Removing intranasal splints after septal surgery.

    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.

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

    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.

  7. Pulmonary artery closure in combination with patch technique for treating congenital heart disease combined with large patent ductus arteriosus: A clinical study of 9 cases.

    Wen, Bing; Yang, Junya; Liu, Huiruo; Jiao, Zhouyang; Zhao, Wenzeng

    2016-01-01

    To document clinical experience of treating congenital heart disease combined with large patent ductus arteriosus with pulmonary artery closure in combination with patch technique. Thirty-six patients (8 males and 28 females) who suffered from congenital heart disease and underwent hybrid surgery in the First Affiliated Hospital of Zhengzhou University from October 2010 to February 2014 were selected for this study. They aged 14 to 39 years and weighed 32.20 to 61.50 kg. Diameter of arterial duct was between 10 mm and 13 mm; 28 cases were tube type, 4 cases were funnel type and four cases were window type. All patients had moderate or severe pulmonary arterial hypertension; besides, there were 28 cases of ventricular septal defect, 16 cases of atrial septal defect, eight cases of aortic insufficiency, four cases of mitral stenosis and insufficiency and four cases of infectious endocarditis. Cardz Pulmonary Bypass (CPB) was established after chest was opened along the middle line. With the help of Transesophageal echocardiography, large patent ductus arteriosus was blocked off through pulmonary artery. Pulmonary artery was cut apart after blocking of heart. Large patent ductus arteriosus on the side of pulmonary artery was strengthened with autologous pericardial patch. Of 36 patients, 32 patients had patent ductus arteriosus closure device and four patients had atrial septal defect closure device. Pulmonary arteries of 36 cases were all successfully closed. Systolic pressure declined after closure ((54.86±19.23) mmHg vs (96.05±23.07) mmHg, pclosure ((39.15±14.83) mmHg vs (72.88±15.76) mmHg, ppatent ductus arteriosus and congenital heart disease, which decreases surgical problems, shortens surgical time and lowers the incidence of complications.

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

    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.

  9. Perioperative Anaesthetic Management of a Patient of Gilbert’s Syndrome with Adult Congenital Heart Disease - A Rare Presentation

    Sambhunath Das

    2014-11-01

    Full Text Available Gilbert's syndrome is a hereditary condition with the genetic mutation of the enzyme uridine diphosphate glucuronosyltransferase, characterized by intermittent jaundice in the absence of hemolysis or underlying liver disease. These patients develop jaundice when subjected to fasting, stress and exercise. Majority of anaesthetics are metabolized by liver. Anaesthesia, surgery and cardiopulmonary bypass (CPB can act as triggers to hepatic injury. The successful perioperative management of an adult congenital heart disease patient for atrial septal defect closure under cardiopulmonary bypass was discussed in this report.

  10. Congenital Heart Information Network

    ... heart defects. Important Notice The Congenital Heart Information Network website is temporarily out of service. Please join ... and Uwe Baemayr for The Congenital Heart Information Network Exempt organization under Section 501(c)3. Copyright © ...

  11. Does asymptomatic septal agenesis exist? A review of 34 cases

    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

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

    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.

  13. ECG-manifest and ECG-silent dipyridamole technetium-99m sestamibi SPET perfusion defects in patients with ischaemic heart disease

    Galli, M.; Marcassa, C.; Bosimini, E.; Zoccarato, O.; Comazzi, F.; Giannuzzi, P.

    1997-01-01

    To investigate the relationship between ECG changes and perfusion abnormalities, body surface maps were recorded during dipyridamole infusion in 55 subjects (11 normals and 44 patients with ischaemic heart disease) undergoing dipyridamole technetium-99m sestamibi single-photon emission tomography (SPET). All had a normal resting ECG. The extent and severity of the sestamibi defect were quantified. New negative areas in the isointegral maps and rest-dipyridamole map differences >2 SD from normal limits were considered abnormal. After dipyridamole in normals, neither perfusion defects nor ≥1 mm ST segment depression on 12-lead ECG nor new negative areas in isointegral maps occurred. In patients, dipyridamole induced new perfusion defects in 35 (80%) but ST segment depression in only 18 (41%, P<0.001). Of the 35 patients with perfusion defects, 17 (49%, group 1) showed ST segment depression, while the other 18 (51%, group 2) did not. Abnormal body surface maps were found in 100% of group 1 and 88% of group 2 patients (NS). In group 1, the provoked hypoperfusion was of greater extent (P=0.007) and severity (P=0.01) and the onset of map abnormalities was significantly earlier (P<0.001) than in group 2; time to map abnormalities was also significantly shorter than time to ST segment depression (P=0.01). In the 35 patients with complete scintigraphic, body map and angiographic data, the severity of reversible perfusion defect proved to be the strongest correlate of ST segment depression upon logistic regression analysis. Thus, sestamibi SPET abnormalities after dipyridamole are almost always associated with electrical changes on body surface maps, suggesting myocardial ischaemia as their cause. The much less common 12-lead ECG changes are slower to appear and reflect a more severe hypoperfusion. (orig./MG). With 5 figs., 4 tabs

  14. An Introduction to the Septal Extension Graft

    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.

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

    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.

  16. A Rare de novo Interstitial Duplication at 4p15.2 in a Boy with Severe Congenital Heart Defects, Limb Anomalies, Hypogonadism, and Global Developmental Delay.

    Liang, Liyang; Xie, Yingjun; Shen, Yiping; Yin, Qibin; Yuan, Haiming

    2016-01-01

    Proximal 4p deletion syndrome is a relatively rare genetic condition characterized by dysmorphic facial features, limb anomalies, minor congenital heart defects, hypogonadism, cafe-au-lait spots, developmental delay, tall and thin habitus, and intellectual disability. At present, over 20 cases of this syndrome have been published. However, duplication of the same region in proximal 4p has never been reported. Here, we describe a 2-year-5-month-old boy with severe congenital heart defects, limb anomalies, hypogonadism, distinctive facial features, pre- and postnatal developmental delay, and mild cognitive impairments. A de novo 4.5-Mb interstitial duplication at 4p15.2p15.1 was detected by chromosomal microarray analysis. Next-generation sequencing was employed and confirmed the duplication, but revealed no additional pathogenic variants. Several candidate genes in this interval responsible for the complex clinical phenotype were identified, such as RBPJ, STIM2, CCKAR, and LGI2. The results suggest a novel contiguous gene duplication syndrome. © 2016 S. Karger AG, Basel.

  17. Cellular cardiomyoplasty for a patient with heart failure

    Zhang Fumin; Chen Yijiang; Yang Zhijian; Gao Xiang; Ma Wenzhu; Li Chuanfu; Kao, Race L.

    2003-01-01

    Background: A 73-year-old man with a history of myocardial infarction and hypertension for 5 years suffered heart failure (NYHA III-IV). Methods: 2D echo indicated hypokinesia at septal, left ventricular anterior wall and apical regions. Coronary angiograms demonstrated 60% stenosis in distal left main and 99% stenosis in proximal and distal left anterior descending coronary arteries (LAD). Both proximal artery and middle left circumflex coronary artery (LC) had 90% stenosis, and diffuse stenosis of right coronary artery (RC) was found. Myocardial perfusion imaging using 99m Tc-MIBI indicated defective perfusion of left ventricular apex, anterior wall and septal region and severe reduced perfusion of posterior inferior wall. Myocardial metabolic activities ( 18 F-deoxyglucose) also showed comparable reductions. After exposing the heart, LAD, LC, and RC were all completely occluded and bypass procedure could not be completed. Autologous satellite cells were implanted without any complication and the patient had an uneventful recovery. Results: During the first 2 months, he remained in heart failure, and by the third month, he gradually improved and reached NYHA II. At fifth month after the procedure, significant increased ejection fraction (37.1-48.6%) and wall movement with modest reduction of left ventricular systolic diameter (48-45 mm) were observed. Imaging with 18 F-deoxyglucose showed dramatic improvement in myocardial metabolic activity with similar improvement in myocardial perfusion ( 99m Tc-MIBI). Conclusion: This is the first successful case of cellular cardiomyoplasty without any conjunctional procedure for patient with severe coronary heart disease and heart failure

  18. Divergent Requirements for EZH1 in Heart Development Versus Regeneration.

    Ai, Shanshan; Yu, Xianhong; Li, Yumei; Peng, Yong; Li, Chen; Yue, Yanzhu; Tao, Ge; Li, Chuanyun; Pu, William T; He, Aibin

    2017-07-07

    Polycomb repressive complex 2 is a major epigenetic repressor that deposits methylation on histone H3 on lysine 27 (H3K27me) and controls differentiation and function of many cells, including cardiac myocytes. EZH1 and EZH2 are 2 alternative catalytic subunits with partial functional redundancy. The relative roles of EZH1 and EZH2 in heart development and regeneration are unknown. We compared the roles of EZH1 versus EZH2 in heart development and neonatal heart regeneration. Heart development was normal in Ezh1 -/- ( Ezh 1 knockout) and Ezh2 f/f ::cTNT -Cre ( Ezh 2 knockout) embryos. Ablation of both genes in Ezh1 -/- ::Ezh2 f/f ::cTNT -Cre embryos caused lethal heart malformations, including hypertrabeculation, compact myocardial hypoplasia, and ventricular septal defect. Epigenome and transcriptome profiling showed that derepressed genes were upregulated in a manner consistent with total EZH dose. In neonatal heart regeneration, Ezh1 was required, but Ezh2 was dispensable. This finding was further supported by rescue experiments: cardiac myocyte-restricted re-expression of EZH1 but not EZH2 restored neonatal heart regeneration in Ezh 1 knockout. In myocardial infarction performed outside of the neonatal regenerative window, EZH1 but not EZH2 likewise improved heart function and stimulated cardiac myocyte proliferation. Mechanistically, EZH1 occupied and activated genes related to cardiac growth. Our work unravels divergent mechanisms of EZH1 in heart development and regeneration, which will empower efforts to overcome epigenetic barriers to heart regeneration. © 2017 American Heart Association, Inc.

  19. Should MR imaging be used as the first line of investigation in adult congenital heart disease

    Sivananthan, M.U. Jr.; Rees, M.R.; Verma, S.P.; Gundroo, G.M.; Ridgway, J.; Bann, K. Jr.

    1991-01-01

    This paper investigates the adequacy of MR imaging in the display of anatomy and flow in adult congenital heart disease. Seventeen adult patients with congenital heart disease were studied with a 1-T Siemens Magnatom imager. Gated spin-echo images in three orthogonal as well as selected oblique planes and gradient cine angiographic images were obtained. The results were compared with the results of echocardiography and conventional angiography. There were 9 patients with coarctation of the aorta, 3 of which were postoperative studies. MR images were adequate in the postoperative cases, and the need for angiography was avoided. Seven additional lesions (2 atrial septal defects (ASD), 2 ventricular septal defects (VSD), and 3 bicuspid aortic valves) were demonstrated that were not demonstrated with echocardiography. Four postoperative Blalock shunts were evaluated, which could not be catheterized with echocardiography (2 occlusions, 2 stenoses), and additional flow and anatomic information of the pulmonary vasculature was obtained. In the other 5 cases, 5 additional lesions were demonstrated compared with echocardiography

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

    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.

  1. Spectrum of Congenital Heart Diseases in Eastern Nepal: A tertiary care hospital experience

    Prashant Shah

    2017-01-01

    Full Text Available Background & Objectives: Congenital heart diseases are neglected especially in world’s poorest nations and appear to be ignored and unexplored dimension of health. The exact prevalence and spectrum of congenital heart diseases in Nepal is largely unknown. The aim of this study was to describe the local experience on the magnitude and the pattern of congenital heart disease in order to increase the awareness of the public and health policy makers on its burden in Nepal.Materials & Methods: This is an observational hospital based study carried out in a tertiary care hospital in Eastern Nepal. The duration of this study was from April 2015 to July 2016. The echocardiography reports of all patients clinically suspected of having congenital heart disease were retrieved, and their diagnostic details were extracted. Only patients of day one of life to 14 years of age were included. Congenital heart diseases like bicuspid aortic valve, mitral valve prolapse and various inherited cardiomyopathies were excluded.Results: A total of 330 echocardiograms were performed for clinically suspected congenital heart disease.  The mean age of study population was 22.31±34.08 months with male to female ratio of 1.2:1. 23% of clinically suspected congenital heart disease cases turned out to have normal echocardiography. Acyanotic congenital heart disease was most common (81.5% followed by cyanotic congenital heart disease (14.2% and obstructive congenital heart disease (4.3%. Atrial septal defect was found to be the most common form of acyanotic congenital heart disease (52% which was followed by ventricular septal defect (28.8% and patent ductus arteriosus (14.8%. Tetralogy of Fallot and double outlet right ventricle were the most common form of cyanotic CHD representing 44.4% of all cyanotic patients. Pulmonary stenosis was the most common obstructive congenital heart disease observed in this study population (63.6%. Rarer entities, like d-transposition of great

  2. Incidence of congenital heart disease among neonates in a neonatal unit of a tertiary care hospital

    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)

  3. Open heart surgery in Nigeria; a work in progress.

    Falase, Bode; Sanusi, Michael; Majekodunmi, Adetinuwe; Animasahun, Barakat; Ajose, Ifeoluwa; Idowu, Ariyo; Oke, Adewale

    2013-01-12

    There has been limited success in establishing Open Heart Surgery programmes in Nigeria despite the high prevalence of structural heart disease and the large number of Nigerian patients that travel abroad for Open Heart Surgery. The challenges and constraints to the development of Open Heart Surgery in Nigeria need to be identified and overcome. The aim of this study is to review the experience with Open Heart Surgery at the Lagos State University Teaching Hospital and highlight the challenges encountered in developing this programme. This is a retrospective study of patients that underwent Open Heart Surgery in our institution. The source of data was a prospectively maintained database. Extracted data included patient demographics, indication for surgery, euroscore, cardiopulmonary bypass time, cross clamp time, complications and patient outcome. 51 Open Heart Surgery procedures were done between August 2004 and December 2011. There were 21 males and 30 females. Mean age was 29 ± 15.6 years. The mean euroscore was 3.8 ± 2.1. The procedures done were Mitral Valve Replacement in 15 patients (29.4%), Atrial Septal Defect Repair in 14 patients (27.5%), Ventricular Septal Defect Repair in 8 patients (15.7%), Aortic Valve Replacement in 5 patients (9.8%), excision of Left Atrial Myxoma in 2 patients (3.9%), Coronary Artery Bypass Grafting in 2 patients (3.9%), Bidirectional Glenn Shunts in 2 patients (3.9%), Tetralogy of Fallot repair in 2 patients (3.9%) and Mitral Valve Repair in 1 patient (2%). There were 9 mortalities (17.6%) in this series. Challenges encountered included the low volume of cases done, an unstable working environment, limited number of trained staff, difficulty in obtaining laboratory support, limited financial support and difficulty in moving away from the Cardiac Mission Model. The Open Heart Surgery program in our institution is still being developed but the identified challenges need to be overcome if this program is to be sustained. Similar

  4. O conduto valvulado bovino contegra, um biomaterial para o tratamento cirúrgico de cardiopatias congênitas The contegra valved bovine conduit: a biomaterial for the surgical treatment of congenital heart defects

    Shi-Min Yuan

    2012-01-01

    congenital heart defects, especially as a conduit for the right ventricular outflow tract reconstruction. This article aims to make a comprehensive review on the clinical outcomes of Contegra. Reports of Contegra published since 2002 were comprehensively retrieved, collected and analyzed. There were 1718 Contegra, applied in 1705 patients. The sizes of the conduits were 8-22 mm. The patients aged from newborn to 74.5 years, prevailed by pediatrics. The primary diagnosis was congenital heart defects in all cases, with Tetralogy of Fallot, truncus arteriosus and pulmonary atresia being the first three diagnoses, representing 25.6%, 16.7%, and 13.1%, respectively. Contegra was used as a tube graft in the pulmonary position in 1635 (95.9% patients, as a monocuspid patch in 12 (0.7%, as a graft in the position of the pulmonary valve or a monocusps in 40 (2.3%, and as an inferior vena cava-pulmonary artery conduit in the Fontan procedure in 18 (1.1% patients, respectively. Conduit reimplantation was performed in 141 (8.3% patients 33.8 ± 37 (8.6-106.8 months after the initial conduit insertion. Conduit plasty was necessary in 6 (0.4%, and reintervention in 83 (4.9% patients. Indications for conduit reimplantation included severe stenosis of the distal anastomosis, pseudoaneurysm of the proximal anastomosis and severe conduit regurgitation. As for the good performance, availability and longevity, Contegra is a biomaterial suitable for the right ventricular outflow tract reconstruction and for patch repair for ventricular septal defect, but not apt for Fontan procedure.

  5. O conduto valvulado bovino contegra, um biomaterial para o tratamento cirúrgico de cardiopatias congênitas The contegra valved bovine conduit: a biomaterial for the surgical treatment of congenital heart defects

    Shi-Min Yuan

    2012-12-01

    congenital heart defects, especially as a conduit for the right ventricular outflow tract reconstruction. This article aims to make a comprehensive review on the clinical outcomes of Contegra. Reports of Contegra published since 2002 were comprehensively retrieved, collected and analyzed. There were 1718 Contegra, applied in 1705 patients. The sizes of the conduits were 8-22 mm. The patients aged from newborn to 74.5 years, prevailed by pediatrics. The primary diagnosis was congenital heart defects in all cases, with Tetralogy of Fallot, truncus arteriosus and pulmonary atresia being the first three diagnoses, representing 25.6%, 16.7%, and 13.1%, respectively. Contegra was used as a tube graft in the pulmonary position in 1635 (95.9% patients, as a monocuspid patch in 12 (0.7%, as a graft in the position of the pulmonary valve or a monocusps in 40 (2.3%, and as an inferior vena cava-pulmonary artery conduit in the Fontan procedure in 18 (1.1% patients, respectively. Conduit reimplantation was performed in 141 (8.3% patients 33.8 ± 37 (8.6-106.8 months after the initial conduit insertion. Conduit plasty was necessary in 6 (0.4%, and reintervention in 83 (4.9% patients. Indications for conduit reimplantation included severe stenosis of the distal anastomosis, pseudoaneurysm of the proximal anastomosis and severe conduit regurgitation. As for the good performance, availability and longevity, Contegra is a biomaterial suitable for the right ventricular outflow tract reconstruction and for patch repair for ventricular septal defect, but not apt for Fontan procedure.

  6. Congenital Heart Diseases in the Newborns of Diabetic Mothers: an Echocardiographic Study

    S Rahimpour

    2011-10-01

    Full Text Available Introduction: Despite the discovery of insulin and current improvement in diabetics care, congenital malformations in diabetics are still more frequent than in the general population. The aim of this study was to identify congenital heart dieases (CHD in the newborns of diabetic mothers (IDMS. Methods: In our prospective study, color doppler echocardiography was performed in 75 consecutive full- term newborns of diabetic mothers by GE Vivid3 echocardiographic device. Newborns were classified into two subgroups according to the type of the mothers’ diabetes: pre-gestational and gestational. They were also those were classified into three subgroups according to their birth weight: appropriate, large and small for gestational age. Data analysis was made by Fisher exact test and Chi-Square test. Results: Forty nine (65% and thirty six (35% of subjects were infants of gestational (IGDM and pre-gestational diabetic mothers (IPDM, respectively. Fifty five Newborns (73% were apropriate, fourteen (19% were large and six (8% were small for gestational age. The most common echocardiographic findings included: patent ductus arteriosus (PDA: 54.7%, hypertrophic cardiomyopathy (HCMP: 24%, ventricular septal defect (VSD: 4%, atrial septal defect (ASD: 2.7%, transposition of great arteries (TGA: 1.3% and coarctation of the Aorta (COA: 1.3%. Overall incidence of congenital heart diseases was 9.3 after exclusion of PDA and HCMP cases. The incidence of congenital heart diseases was higher in macrosomic than nonmacrosomic infants of diabetic mothers (P<0.001. Congenital heart diseases were more common in infants of pre-gestational than gestational diabetic mothers (P=0.004. Conclusion: Our results showed that diabetic mothers are at increased risk of giving birth to a newborn with congenital heart disease, and transthoracic echocardiography is recommended for all infants of diabetic mothers.

  7. Management of tricuspid regurgitation in congenital heart disease: is survival better with valve repair?

    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.

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

    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.

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

    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.

  10. Evolution and development of ventricular septation in the amniote heart.

    Robert E Poelmann

    evolution and development of this part of the vertebrate heart as well as understand the etiology of septal defects in human congenital heart malformations.

  11. Generation of human iPSC line from a patient with laterality defects and associated congenital heart anomalies carrying a DAND5 missense alteration

    Fernando Cristo

    2017-12-01

    Full Text Available A human iPSC line was generated from exfoliated renal epithelial (ERE cells of a patient affected with Congenital Heart Disease (CHD and Laterality Defects carrying tshe variant p.R152H in the DAND5 gene. The transgene-free iPSCs were generated with the human OSKM transcription factor using the Sendai-virus reprogramming system. The established iPSC line had the specific heterozygous alteration, a stable karyotype, expressed pluripotency markers and generated embryoid bodies that can differentiate towards the three germ layers in vitro. This iPSC line offers a useful resource to study the molecular mechanisms of cardiomyocyte proliferation, as well as for drug testing.

  12. Translation and Testing of the Swedish Version of Iceland-Family Perceived Support Questionnaire With Parents of Children With Congenital Heart Defects.

    Bruce, Elisabeth; Dorell, Åsa; Lindh, Viveca; Erlingsson, Christen; Lindkvist, Marie; Sundin, Karin

    2016-08-01

    There is a need for a suitable instrument for the Swedish context that could measure family members' perceptions of cognitive and emotional support received from nurses. The purpose of this study was to translate and test the psychometric properties of the Swedish version of the Iceland-Family Perceived Support Questionnaire (ICE-FPSQ) and, further, to report perceptions of support from nurses by family members of children with congenital heart defects (CHDs). A sample of 97 parents of children with CHD, living in Sweden, completed the Swedish translation of ICE-FPSQ. The Swedish version of ICE-FPSQ was found to be reliable and valid in this context. Parents scored perceived family support provided by nurses working in pediatric outpatient clinics as low, which suggests that nurses in these outpatient contexts in Sweden offered family nursing only sparingly. © The Author(s) 2016.

  13. Clinical, demographic characteristics and results of the long term follow-up in adolescents and adults with congenital heart disease

    I.G. Lebid

    2016-05-01

    Full Text Available The aim – to analyze clinical and demographic indicators in adolescents and adults with congenital heart disease (CHD to provide strategy of cardiac care for these patients, to assess risk of cardiological and cardiac surgery interventions in patients with congenital heart malformations. Materials and methods. 2569 consecutive patients, aged 16–88 years, mean age 24.14 ± 0.20 years, were selected in electronic database from April 01, 2011 to December 31, 2015. The majority (92.57 % of the included patients (n = 2378 were younger than 40 years. Results. Among all CHD patients, a significant majority had septal defects (39 % and left heart lesions (24 %, followed by congenital lesions of thoracic arteries and veins (16 % and right heart lesions (10 %. The annual number of the examined patients with CHD progressively increased (from 210 in 2011 to 656 in 2015. The number of patients aged 18 years or older mostly increased. The number of patients older than 40 years increased from nine patients in 2011 to 75 adults in 2015. Patients after cardiac surgery and percutaneous transcatheter interventions dominated (n=1553, 60.45 %, compared to the patients without any interventions for CHD (n = 1016, 39.55 %. Only one intervention for CHD was performed in the majority of these patients (n = 1255, 80.81 %, 12.94 % needed two interventions, 3.99 % – three, 2.26 % – three or more interventions. Conclusions. Among patients with congenital heart disease, patients younger than 40 years old were prevalent (92.57 %, with no significant gender differences. Septal defects (ASD, VSD, left heart lesions (congenital aortic valve stenosis and insufficiency, congenital lesions of thoracic arteries and veins (patent ductus arteriosus and aorta coarctation were registered most often in adolescents and adults.

  14. Radiation dose and risk to children undergoing cardiac catheterization for the treatment of a congenital heart disease using Monte Carlo simulations

    Yakoumakis, E; Kostopoulou, H.; Dimitriadis, T.; Georgiou, E.; Makri, T.; Tsalafoutas, I.

    2012-01-01

    Background and objective: Children diagnosed with congenital heart disease often undergo cardiac catheterization for their treatment, which involves the use of ionizing radiation and therefore a risk of radiation induced cancer. Our objective was to calculate the effective (E) and equivalent organ doses (HT) in those children and estimate the risk of radiation induced cancer (REID). Materials and methods: Fifty three children were divided into three groups, atrial septal defect (ASD), ventricular septal defect (VSD), and patent ductus arteriosus (PDA), depending on their heart condition. In all procedures, the exposure conditions and the Dose-Area Product (DAP) meters readings were recorded for each individual acquisition. Monte Carlo simulations were run using the PCXMC 2.0 code and mathematical phantoms simulating children anatomy. The HT values to all irradiated organs and the resulting E and REID values were calculated. Results: The average DAP values were respectively 40±12 Gy·cm 2 for the ASD, 17.5±0.7 Gy·cm 2 for the VSD and 9.5±1 Gy·cm 2 for the PDA group. The average E values were 40±12, 22±2.5 and 17±3.6 mSv for ASD, VSD and PDA groups, respectively. The respective estimated REID values per procedure were 0.109, 0.106 and 0.067 %. For all groups the most heavily irradiated organs were the thymus, heart, breast, lung and stomach. (authors)

  15. Novas técnicas cirúrgicas para o tratamento da atresia pulmonar com comunicação interventricular e anomalias de artérias pulmonares incluindo o assim chamado truncus tipo IV New surgical techniques for treatment of pulmonary atresia with ventricular septal defect and pulmonary arteries anomalies including the so-called tipo IV truncus

    Miguel Barbero-Marcial

    1987-04-01

    Full Text Available Entre janeiro de 1975 e outubro de 1986, 42 pacientes com atresia pulmonar e comunicação interventricular, com idade entre 2 e 18 anos, foram submetidos a correção parcial, ou total. Foram divididos em: tipo A com todos os segmentos broncopulmonares conectados às artérias pulmonares (AP's, 34 pacientes; tipo B com alguns dos segmentos broncopulmonares conectados às AP's, 6 pacientes; tipo C com todos os segmentos broncopulmonares conectados às colaterais sistêmico-pulmonares, 2 pacientes. A correção foi planejada em uma a três etapas. No tipo A, 17 foram corrigidos em uma etapa, com três óbitos; em 9, na primeira etapa, as AP's foram reconstruídas e o Blalock (BT, realizado, tendo ocorrido um óbito. Em 2, a segunda etapa de correção total foi realizada, sem óbitos. No tipo B, a primeira etapa de unificação das colaterais intra ou extra-hilares foi realizada em 6 casos, sem óbitos; em 2, a segunda etapa da correção total foi realizada, com um óbito. No tipo C, 2 pacientes foram operados; 1 em três etapas; a primeira constou de construção de segmento arterial intermediário entre as artérias lobares e o BT; a segunda compreendeu unificação das colaterais contralaterais e a terceira, restabelecimento da continuidade ventrículo direito - circulação pulmonar; o paciente teve boa evolução. No segundo caso, a correção foi realizada após somente uma intervenção prévia. A evolução foi satisfatória. Estudos hemodinâmicos seriados foram realizados em 32 pacientes. As técnicas propostas permitem obter condições para correção total com adequada relação pós-operatória das pressões ventrículo direito - ventrículo esquerdo.Fourty-two patients with pulmonary atresia and interventricular septal defect were submited to a partial or total correction, between January 1975 and October 1986, with a range of 2 months to 18 years of age. Three groups were identified: Group A: 34 patients with all bronco

  16. The pulmonary vascular blood supply in the pulmonary atresia with ventricular septal defect and its implications in surgical treatment O suprimento sangüíneo vascular pulmonar na atresia pulmonar com comunicação interventricular e suas implicações no tratamento cirúrgico

    Ulisses Alexandre Croti

    2003-10-01

    Full Text Available OBJECTIVE: With base in the studies cineangiocardiography of pacients with pulmonary atresia (PA with ventricular septal defect (VSD, to identify in the groups proposed by BARBERO MARCIAL, subgroups with similar morphological characteristics, to measure their central pulmonary arteries (CPA and major aortopulmonary collateral arteries (MAPCA, thereby establishing their implications in surgical treatment. METHOD: Sixty three patients were classified in groups A (15, B (40 and C (8 between january 1990 and june 2001. Patients with complete cineangiocardiograms prior to the first surgical intervention were included in this study, being calculated the pulmonary arterial index (PAI, the major aortopulmonary collateral arterial index (MAPCAI and the total neopulmonary arterial index (TNPAI = PAI + MAPCAI. Surgical treatment was considered palliative (PT, definitively palliative (DPT and definitive (DT. RESULTS: Nine subgroups were identified, A (A1 and A2, B (B1, B2, B3, B4 and B5 and C (C1 and C2. In group A, the PAI of patients for DT was higher than for PT patients (p=0,0092. In group B, the TNPAI of DT patients was greater than for PT patients (p=0,0959. In group C, the MAPCAI in DPT patients was lower than in PT and DT patients. In the group A was not mortality, in the group B was of 17,5% and in the group C was of 12,5%. CONCLUSIONS:Among the groups A, B e C was possible to identifiy nine subgroups, the morphologic and morphometric characteristics allowed to suggest the surgical treatment in the patients of the group A had larger chance of TD, the group B of TP and the group C of TPD. The mortality presented larger correlation with the morphologic characteristics that with the morphometric.OBJETIVO: Com base nos estudos cineangiocardiográficos de pacientes portadores de atresia pulmonar (AP com comunicação interventricular (CIV, identificar nos grupos propostos pela classificação de BARBERO MARCIAL, subgrupos com suprimento sang

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

    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.

  18. [Clinical Experience of Beating Heart Atrial Septostomy Using a Device for Coronary Artery Anastomosis Site Creator].

    Takahashi, Goro; Sai, Sadahiro; Konishi, Akinobu

    2015-09-01

    Intra-atrial communication was mandatory for several congenital cardiac diseases, such as pulmonary atresia with intact ventricular septum (PA/IVS), and either sided aortoventricular valve atresia. We assessed whether the new methods of atrial septal defect(ASD)creation was effective. We experienced 4 cases of the surgical atrial septostomy performed under on-pump beating. We used a new device, a circular punch out defect creator. All cases were alive. The mean ASD diameter was enlarged from 4.37 mm to 5.55 mm and the mean ASD shunt flow was significantly decreased from 1.47 m/s to 1.11 m/s. We performed the surgical atrial septostomy using an aortic puncher under beating heart effectively and safely.

  19. Utility of Capture-Recapture Methodology to Estimate Prevalence of Congenital Heart Defects Among Adolescents in 11 New York State Counties: 2008 to 2010.

    Akkaya-Hocagil, Tugba; Hsu, Wan-Hsiang; Sommerhalter, Kristin; McGarry, Claire; Van Zutphen, Alissa

    2017-11-01

    Congenital heart defects (CHDs) are the most common birth defects in the United States, and the population of individuals living with CHDs is growing. Though CHD prevalence in infancy has been well characterized, better prevalence estimates among children and adolescents in the United States are still needed. We used capture-recapture methods to estimate CHD prevalence among adolescents residing in 11 New York counties. The three data sources used for analysis included Statewide Planning and Research Cooperative System (SPARCS) hospital inpatient records, SPARCS outpatient records, and medical records provided by seven pediatric congenital cardiac clinics from 2008 to 2010. Bayesian log-linear models were fit using the R package Conting to account for dataset dependencies and