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Sample records for atrioventricular septal defect

  1. Anatomical-embryological correlates in atrioventricular septal defect.

    OpenAIRE

    Allwork, S P

    1982-01-01

    Recent embryological studies have supported the consideration that the ventricular septum is multifocal in origin. These data have also provided excellent correlation of the morphology of malformed hearts with their embryology. In particular, atrioventricular septal defect correlates accurately with these observations on ventricular septation. Many of the names given to atrioventricular septal defect (for example ostium primum, persistent atrioventricular canal, endocardial cushion defect) in...

  2. The ostium primum or partial atrioventricular septal defect

    OpenAIRE

    Radermecker, Marc; Fontaine, Raphael; Limet, Raymond

    2007-01-01

    Often assimilated to simple inter-atrial communication, the ostium primum, or partial atrio-ventricular septal defect, is an entity that is characterized by a different embryological mechanism and requires some specific surgical expertise. Basically, knowledge of the morphology of the common atrioventricular valve with 5 components, the topography of the A-V node and His bundle, and the ventricular consequences of the absence of atrio-ventricular septal structures must be taken into account. ...

  3. [Fetal atrioventricular septal defect associated with Patau and Edwards syndromes, as well as trisomy 22].

    Science.gov (United States)

    Cesko, I; Hajdú, J; Marton, T; Tóth-Pál, E; Papp, C; Papp, Z

    1998-05-03

    The atrioventricular septal defect is usually associated with trisomy 21 and it may be observed in the heterotaxia syndromes. Atrioventricular septal defect may be associated with 8p deletion. There are reported cases of familial atrioventricular septal defect. Atrioventicular septal defect is rarely associated with other chromosomal abnormalities. We are reporting three unusual cases of atrioventricular septal defect that were associated with trisomy 13, 18 and 22. This association may be due to effect of genetic loci on the 13, 18 and 22 chromosome which could play the role in the development and fusion of endocardial cushion and atrioventricular septal defect.

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

    Directory of Open Access Journals (Sweden)

    Marcelo Felipe Kozak

    2015-09-01

    Full Text Available AbstractIntroduction:Left atrioventricular valve regurgitation is the most concerning residual lesion after surgical correction of atrioventricular septal defects.Objective:To determine factors associated with moderate or severe left atrioventricular valve regurgitation within 30 days of surgical repair of complete atrioventricular septal defect.Methods:We assessed the results of 53 consecutive patients 3 years-old and younger presenting with complete 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 atrioventricular valve regurgitation, abnormalities on the left atrioventricular valve and the use of annuloplasty. Median age was 6.7 months; median weight was 5.3 Kg; 86.8% had Down syndrome. At the time of preoperative evaluation, there were 26 cases with moderate or severe left atrioventricular valve regurgitation (49.1%. Abnormalities on the left atrioventricular valve were found in 11.3%; annuloplasty was performed in 34% of the patients.Results:At the time of postoperative evaluation, there were 21 cases with moderate or severe left atrioventricular valve regurgitation (39.6%. After performing a multivariate analysis, the only significant factor associated with moderate or severe left atrioventricular valve regurgitation was the absence of Down syndrome (P=0.03.Conclusion:Absence of Down syndrome was associated with moderate or severe postoperative left atrioventricular valve regurgitation after surgical repair of complete atrioventricular septal defect at our practice.

  5. Atrioventricular Septal Defect with Common Atrioventricular Junction Guarded by a Common Valve Consisting of Left Atrioventricular Trifoliate Valve

    Science.gov (United States)

    Krasniqi, Xhevdet; Gashi, Masar; Berisha, Blerim; Pllana, Ejup; Bakalli, Aurora; Abazi, Flora; Koçinaj, Dardan

    2013-01-01

    Introduction: Atrioventricular septal defect with common atrioventricular junction is a rare adult congenital cardiac syndrome. This occurrence with prolonged survival is exceptionally rare. Case report: We present the case of a patient who presented with this defect with common atrioventricular junction who survived to the age of 32. We describe a 32-year-old man with atrioventricular septal defect with common atrioventricular junction guarded by a common valve. His history, clinical course, and anatomic findings are discussed along with the factors which may have contributed to his longevity, which is unique in the medical literature. His management reflected the state of medical knowledge at the time when he presented, and although alternate approaches may have been utilized if the patient presented today. We discuss the findings, frequency, classifi cation, and management of congenital defects. Development of embryonic structure is altered by interaction between genetics and environmental factors toward a rare associated of congenital cardiac defects-complex congenital heart disease. Conclusion: This case demonstrates that patients with very complex congenital cardiac disease may survive to adulthood, presenting challenges in both medical and surgical treatment. PMID:24554809

  6. Atrioventricular septal defect with common atrioventricular junction guarded by a common valve consisting of left atrioventricular trifoliate valve.

    Science.gov (United States)

    Krasniqi, Xhevdet; Gashi, Masar; Berisha, Blerim; Pllana, Ejup; Bakalli, Aurora; Abazi, Flora; Koçinaj, Dardan

    2013-12-01

    Atrioventricular septal defect with common atrioventricular junction is a rare adult congenital cardiac syndrome. This occurrence with prolonged survival is exceptionally rare. We present the case of a patient who presented with this defect with common atrioventricular junction who survived to the age of 32. We describe a 32-year-old man with atrioventricular septal defect with common atrioventricular junction guarded by a common valve. His history, clinical course, and anatomic findings are discussed along with the factors which may have contributed to his longevity, which is unique in the medical literature. His management reflected the state of medical knowledge at the time when he presented, and although alternate approaches may have been utilized if the patient presented today. We discuss the findings, frequency, classifi cation, and management of congenital defects. Development of embryonic structure is altered by interaction between genetics and environmental factors toward a rare associated of congenital cardiac defects-complex congenital heart disease. This case demonstrates that patients with very complex congenital cardiac disease may survive to adulthood, presenting challenges in both medical and surgical treatment.

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

    Directory of Open Access Journals (Sweden)

    Marcelo Felipe Kozak

    2015-04-01

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

  8. Down Syndrome with Complete Atrioventricular Septal Defect, Hypertrophic Cardiomyopathy, and Pulmonary Vein Stenosis.

    Science.gov (United States)

    Mahadevaiah, Guruprasad; Gupta, Manoj; Ashwath, Ravi

    2015-10-01

    The prevalence of congenital heart disease in infants with Down syndrome is 40%, compared with 0.3% in children who have normal chromosomes. Atrioventricular and ventricular septal defects are often associated with chromosomal aberrations, such as in trisomy 21, whereas hypertrophic cardiomyopathy is chiefly thought to be secondary to specific gene mutations. We found only one reported case of congenital hypertrophic cardiomyopathy and atrioventricular septal defect in an infant with Down syndrome. Here, we report atrioventricular septal defect, hypertrophic cardiomyopathy, and pulmonary vein stenosis in a neonate with Down syndrome-an apparently unique combination. In addition, we discuss the relevant medical literature.

  9. Genome-Wide Association Study of Down Syndrome-Associated Atrioventricular Septal Defects

    National Research Council Canada - National Science Library

    Ramachandran, Dhanya; Zeng, Zhen; Locke, Adam E; Mulle, Jennifer G; Bean, Lora J H; Rosser, Tracie C; Dooley, Kenneth J; Cua, Clifford L; Capone, George T; Reeves, Roger H; Maslen, Cheryl L; Cutler, David J; Feingold, Eleanor; Sherman, Stephanie L; Zwick, Michael E

    2015-01-01

    .... We performed a genome-wide association study using logistic regression analysis on 452 individuals with Down syndrome, consisting of 210 cases with complete atrioventricular septal defects and 242...

  10. Contribution of copy-number variation to Down syndrome-associated atrioventricular septal defects.

    Science.gov (United States)

    Ramachandran, Dhanya; Mulle, Jennifer G; Locke, Adam E; Bean, Lora J H; Rosser, Tracie C; Bose, Promita; Dooley, Kenneth J; Cua, Clifford L; Capone, George T; Reeves, Roger H; Maslen, Cheryl L; Cutler, David J; Sherman, Stephanie L; Zwick, Michael E

    2015-07-01

    The goal of this study was to identify the contribution of large copy-number variants to Down syndrome-associated atrioventricular septal defects, the risk for which in the trisomic population is 2,000-fold more as compared with that of the general disomic population. Genome-wide copy-number variant analysis was performed on 452 individuals with Down syndrome (210 cases with complete atrioventricular septal defects; 242 controls with structurally normal hearts) using Affymetrix SNP 6.0 arrays, making this the largest heart study conducted to date on a trisomic background. Large, common copy-number variants with substantial effect sizes (OR > 2.0) do not account for the increased risk observed in Down syndrome-associated atrioventricular septal defects. By contrast, cases had a greater burden of large, rare deletions (P Down syndrome-associated atrioventricular septal defects, whereas large, common copy-number variants do not appear to increase the risk of Down syndrome-associated atrioventricular septal defects. The genetic architecture of atrioventricular septal defects is complex and multifactorial in nature.

  11. Cilia gene mutations cause atrioventricular septal defects by multiple mechanisms.

    Science.gov (United States)

    Burnicka-Turek, Ozanna; Steimle, Jeffrey D; Huang, Wenhui; Felker, Lindsay; Kamp, Anna; Kweon, Junghun; Peterson, Michael; Reeves, Roger H; Maslen, Cheryl L; Gruber, Peter J; Yang, Xinan H; Shendure, Jay; Moskowitz, Ivan P

    2016-07-15

    Atrioventricular septal defects (AVSDs) are a common severe form of congenital heart disease (CHD). In this study we identified deleterious non-synonymous mutations in two cilia genes, Dnah11 and Mks1, in independent N-ethyl-N-nitrosourea-induced mouse mutant lines with heritable recessive AVSDs by whole-exome sequencing. Cilia are required for left/right body axis determination and second heart field (SHF) Hedgehog (Hh) signaling, and we find that cilia mutations affect these requirements differentially. Dnah11avc4 did not disrupt SHF Hh signaling and caused AVSDs only concurrently with heterotaxy, a left/right axis abnormality. In contrast, Mks1avc6 disrupted SHF Hh signaling and caused AVSDs without heterotaxy. We performed unbiased whole-genome SHF transcriptional profiling and found that cilia motility genes were not expressed in the SHF whereas cilia structural and signaling genes were highly expressed. SHF cilia gene expression predicted the phenotypic concordance between AVSDs and heterotaxy in mice and humans with cilia gene mutations. A two-step model of cilia action accurately predicted the AVSD/heterotaxyu phenotypic expression pattern caused by cilia gene mutations. We speculate that cilia gene mutations contribute to both syndromic and non-syndromic AVSDs in humans and provide a model that predicts the phenotypic consequences of specific cilia gene mutations. © The Author 2016. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  12. Genome-Wide Association Study of Down Syndrome-Associated Atrioventricular Septal Defects.

    Science.gov (United States)

    Ramachandran, Dhanya; Zeng, Zhen; Locke, Adam E; Mulle, Jennifer G; Bean, Lora J H; Rosser, Tracie C; Dooley, Kenneth J; Cua, Clifford L; Capone, George T; Reeves, Roger H; Maslen, Cheryl L; Cutler, David J; Feingold, Eleanor; Sherman, Stephanie L; Zwick, Michael E

    2015-07-20

    The goal of this study was to identify the contribution of common genetic variants to Down syndrome-associated atrioventricular septal defect, a severe heart abnormality. Compared with the euploid population, infants with Down syndrome, or trisomy 21, have a 2000-fold increased risk of presenting with atrioventricular septal defects. The cause of this increased risk remains elusive. Here we present data from the largest heart study conducted to date on a trisomic background by using a carefully characterized collection of individuals from extreme ends of the phenotypic spectrum. We performed a genome-wide association study using logistic regression analysis on 452 individuals with Down syndrome, consisting of 210 cases with complete atrioventricular septal defects and 242 controls with structurally normal hearts. No individual variant achieved genome-wide significance. We identified four disomic regions (1p36.3, 5p15.31, 8q22.3, and 17q22) and two trisomic regions on chromosome 21 (around PDXK and KCNJ6 genes) that merit further investigation in large replication studies. Our data show that a few common genetic variants of large effect size (odds ratio >2.0) do not account for the elevated risk of Down syndrome-associated atrioventricular septal defects. Instead, multiple variants of low-to-moderate effect sizes may contribute to this elevated risk, highlighting the complex genetic architecture of atrioventricular septal defects even in the highly susceptible Down syndrome population. Copyright © 2015 Ramachandran et al.

  13. The left atrioventricular valve in partial atrioventricular septal defect: management strategy and surgical outcome.

    Science.gov (United States)

    Al-Hay, Amira A A; Lincoln, Christopher R; Shore, Darryl F; Shinebourne, Elliot A

    2004-10-01

    To test the hypothesis that in patients with a partial atrioventricular septal defect (PAVSD) and a competent left atrioventricular valve (LAVV), sutures should be placed across the line of apposition of the superior and inferior bridging leaflets, septal commissure (SC), to prevent the development of regurgitation. Outcome of surgery and risk factors for the need for LAVV reoperation of patients with mild or no LAVV regurgitation (LAVVR) were evaluated. Controversy over management of the LAVV in PAVSD. One hundred and forty seven children with PAVSD underwent surgical repair at the Royal Brompton Hospital between January 1983 and December 1999. Of this group, 21 (16.7%) had LAVVR of sufficient severity to require surgical intervention and were therefore excluded from analysis. The median age and weight at repair of those with mild or no LAVVR was 4.1 years and 15.4 kg. One hundred and eight had normal chromosomes, 13 Down syndrome and five other syndromes. The interatrial communication was closed using a pericardial patch in 62.7% and with synthetic material in the remainder. Intraoperative testing of LAVV competence was undertaken using saline injection into the left ventricle. In 80.9%, sutures were placed across the line of apposition of the left sided superior and inferior bridging leaflets partially to close the SC (sometimes incorrectly named the mitral valve cleft). The overall hospital mortality was 3.2% (95% confidence interval (CI) 1, 8.4%), which did not differ statistically in the last 20 years. No specific risk factors for early death were identified. Eleven patients (8.7%, 95% CI 4.7, 15.4%) required reoperation, 10 for LAVV repair and 1 resection of subaortic stenosis. Univariate analysis of risk factors for LAVV reoperation were low weight, relatively small size LAVV, the presence of a small preoperative interventricular interchordal communication and duration of ventilation. Ten (9.8%) of 102 patients in whom SC was sutured required LAVV

  14. 59. Early and late results of routine leaflet augmentation for complete atrio-ventricular septal defect repair

    OpenAIRE

    A. Arifi; Najm, H; Khan, A.; Ahmad, M; Khan, M A; M. Elanany

    2016-01-01

    Complete AVSD (CAVSD) is characterized by the presence of a common atrio-ventricular (AV) orifice, an inter-atrial communication, and a ventricular septal defect (VSD). Results of surgical correction of atrio-ventricular septal defects (AVSDs) have improved over the last decades; however, the need for reoperation for left atrio-ventricular valve regurgitation, after primary AVSD repair remains a major concern. The aim of our study is to assess the outcome of the routine leaflet augmentation t...

  15. Exome sequencing identifies rare variants in multiple genes in atrioventricular septal defect

    NARCIS (Netherlands)

    D'Alessandro, Lisa C. A.; Al Turki, Saeed; Manickaraj, Ashok Kumar; Manase, Dorin; Mulder, Barbara J. M.; Bergin, Lynn; Rosenberg, Herschel C.; Mondal, Tapas; Gordon, Elaine; Lougheed, Jane; Smythe, John; Devriendt, Koen; Bhattacharya, Shoumo; Watkins, Hugh; Bentham, Jamie; Bowdin, Sarah; Hurles, Matthew E.; Mital, Seema

    2016-01-01

    The genetic etiology of atrioventricular septal defect (AVSD) is unknown in 40% cases. Conventional sequencing and arrays have identified the etiology in only a minority of nonsyndromic individuals with AVSD. Whole-exome sequencing was performed in 81 unrelated probands with AVSD to identify

  16. Development of the cardiac conduction system in atrioventricular septal defect in human trisomy 21

    NARCIS (Netherlands)

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

    2005-01-01

    In patients with atrioventricular septal defect (AVSD), the occurrence of nonsurgical AV block has been reported. We have looked for an explanation in the development of the AV conduction system. Human embryos with AVSD and trisomy 21 and normal embryos were examined (age 5-16 wk gestation).

  17. Atrioventricular septal defect : advanced imaging from early development to long-term follow-up

    NARCIS (Netherlands)

    Calkoen, Emmeline E.

    2016-01-01

    The aim of this thesis is to review the current knowledge on atrioventricular septal defect (AVSD) (Part 1), to study the pathogenesis of AVSD (Part 2) and finally to analyze cardiac outcome long-term after AVSD correction (Part 3). Studies are performed with novel imaging techniques. In part 2 it

  18. Hemolytic anemia after atrioventricular septal defect repair without synthetic material.

    Science.gov (United States)

    Tsang, J C; Shum-Tim, D; Tchervenkov, C I; Jutras, L; Sinclair, B

    1999-11-01

    We report a rare case of severe hemolytic anemia following repair of a congenital heart defect without the use of prosthetic material. A review of the literature, diagnosis, and management are described. Although this is an unusual complication following congenital heart surgery, a high index of suspicion must be maintained and a possible mechanical cause should be sought and corrected.

  19. Near miss sudden cardiac death on a young patient with repaired atrioventricular septal defect.

    Science.gov (United States)

    Papadopoulou, Sofia A; Dimopoulos, Konstantinos; Gatzoulis, Michael A

    2008-11-28

    Patients with congenital heart disease often face the prospect of long-term haemodynamic or arrhythmic complications for which lifelong follow-up in specialist adult congenital heart disease (ACHD) centres is required. We describe the case of a 25-year-old man with repaired atrioventricular septal defect who was referred to our centre after a ventricular fibrillation arrest. Serial echocardiograms in previous years had shown progressive severe left ventricular outflow obstruction, but the patient had not been operated on as he was deemed asymptomatic and reluctant to consider surgery. Management and criteria for further intervention in ACHD patients often differ from those of patients with acquired heart disease and reliance on symptoms alone is not good practice and may prove catastrophic.

  20. Primary biventricular repair of atrioventricular septal defects: an analysis of reoperations.

    Science.gov (United States)

    Vohra, Hunaid A; Chia, Alicia X F; Yuen, Ho Ming; Vettukattil, Joseph J; Veldtman, Gruschen; Gnanapragasam, James; Roman, Kevin; Salmon, Anthony P; Haw, Marcus P

    2010-09-01

    The purpose of this study was to analyze the factors affecting reoperation after primary biventricular atrioventricular septal defect (AVSD) repair. Between April 1997 and April 2007, 93 consecutive patients underwent surgery for biventricular correction of AVSD with a median age of 5.8 months (range, 9 days to 68.9 years). Fifty-three patients had complete AVSD, 6 patients had an intermediate type, and 29 patients had partial AVSD; 4 patients had a complete AVSD with associated tetralogy of Fallot, and 1 patient had a complete AVSD with double-outlet right ventricle. There was no in-hospital mortality. There were 2 late deaths (2.2%). Forty-three reoperations were performed in 23 patients (24.7%), of which 18 were for repair of significant left atrioventricular valve regurgitation and 8 were mitral valve replacements. Seven patients (7.5%) required insertion of a permanent pacemaker. The overall 5-year freedom from reoperation after AVSD repair was 73.6% +/- 4.8%. In the multivariate analysis for complete AVSDs, Down syndrome (p = 0.01) and the presence of right ventricular dominance (p = 0.03) were independent predictors of reoperation. At last follow-up, 76 patients (83.5%) were in New York Heart Association class I, and 68 patients (74.7%) were not taking any heart failure medications. Echocardiographic examination showed absent to mild left atrioventricular valve regurgitation in 76.5%; moderate, in 19.8%; and severe, in 3.7% of patients. Down syndrome and right ventricular dominance are independent predictors of reoperation after complete AVSD repair. Biventricular repair of isolated AVSD with a small left ventricle can be successfully accomplished with no mortality. 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  1. The surgical anatomy of double-outlet right ventricle with concordant atrioventricular connection and noncommitted ventricular septal defect.

    Science.gov (United States)

    Stellin, G; Ho, S Y; Anderson, R H; Zuberbuhler, J R; Siewers, R D

    1991-12-01

    In describing hearts with double-outlet right ventricle, we have had problems with how best to use the term noncommitted as applied to the ventricular septal defect. We reviewed, therefore, 63 hearts with double-outlet right ventricle in the setting of usual atrial arrangement and a concordant atrioventricular connection. From these, 18 hearts with potentially noncommitted defects were identified and studied in detail. The defect was unequivocally remote from the ventricular outflow tracts in 16 of these hearts, being perimembranous with excavation to open into the inlet of the right ventricle in 12, two of these also having straddling of an atrioventricular valve. One heart had a muscular defect situated in the inlet part of the muscular septum, whereas the defect was the ventricular component of an atrioventricular septal defect in the other three. In the remaining two hearts the defect was anatomically juxtaposed to a subarterial outlet. The pathway to the outflow tract, however, was obstructed by leaflets of a straddling valve. Our study shows, therefore, the need to distinguish between anatomic "commitment" of the defect from the problems in terms of commitment that may confront the surgeon in the operating room. Not only does the distance between the interventricular communication and one of the subarterial outflow tract need to be assessed (the anatomic commitment), but also the presence and nature of any intervening extraneous tissues (the surgical commitment) requires assessment.

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

    NARCIS (Netherlands)

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

    2015-01-01

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

  3. Contribution of Copy Number Variation to Down Syndrome-associated Atrioventricular Septal Defects

    Science.gov (United States)

    Ramachandran, Dhanya; Mulle, Jennifer G.; Locke, Adam E.; Bean, Lora J.H.; Rosser, Tracie C.; Bose, Promita; Dooley, Kenneth J.; Cua, Clifford L.; Capone, George T.; Reeves, Roger H.; Maslen, Cheryl L.; Cutler, David J.; Sherman, Stephanie L.; Zwick, Michael E.

    2014-01-01

    Purpose The goal of this study was to identify the contribution of large copy number variants (CNV) to Down syndrome (DS) associated atrioventricular septal defects (AVSD), whose risk in the trisomic population is 2000-fold more compared to general disomic population. Methods Genome-wide CNV analysis was performed on 452 individuals with DS (210 cases with complete AVSD; 242 controls with structurally normal hearts) using Affymetrix SNP 6.0 arrays, making this the largest heart study conducted to date on a trisomic background. Results Large common CNVs with substantial effect sizes (OR>2.0) do not account for the increased risk observed in DS-associated AVSD. In contrast, cases had a greater burden of large rare deletions (p<0.01) and intersected more genes (p<0.007) when compared to controls. We also observed a suggestive enrichment of deletions intersecting ciliome genes in cases compared to controls. Conclusion Our data provide strong evidence that large rare deletions increase the risk of DS-associated AVSD, while large common CNVs do not appear to increase the risk of DS-associated AVSD. The genetic architecture of AVSD is complex and multifactorial in nature. PMID:25341113

  4. Surgical treatment of partial atrioventricular septal defect: functional analysis of the mitral valve in the postoperative period

    Directory of Open Access Journals (Sweden)

    Josué Viana Castro Neto

    2002-11-01

    Full Text Available OBJECTIVE: To study mitral valve function in the postoperative period after correction of the partial form of atrioventricular septal defect. METHODS: Fifty patients underwent surgical correction of the partial form of atrioventricular septal defect. Their mean age was 11.8 years and 62% of the patients were males. Preoperative echocardiography showed moderate and severe mitral insufficiency in 44% of the patients. The mitral valve cleft was sutured in 45 (90% patients (group II - GII. Echocardiographies were performed in the early postoperative period, and 6 and 12 months after hospital discharge. RESULTS: The patients who had some type of arrhythmia in the postoperative period had ostium primum atrial septal defect of a larger size (2.74 x 2.08 cm. All 5 patients in group I (GI, who did not undergo closure of the cleft, had a competent mitral valve or mild mitral insufficiency in the preoperative period. One of these patients began to have moderate mitral insufficiency in the postoperative period. On the other hand, in GII, 88.8% and 82.2% of the patients had competent mitral valve or mild mitral insufficiency in the early and late postoperative periods, respectively. CONCLUSION: The mitral valve cleft was repaired in 90% of cases. Echocardiography revealed competent mitral valve or mild mitral insufficiency in 88.8% and 82.2% of GII patients in the early and late postoperative periods, respectively.

  5. 59. Early and late results of routine leaflet augmentation for complete atrio-ventricular septal defect repair

    Directory of Open Access Journals (Sweden)

    A. Arifi

    2016-07-01

    Full Text Available Complete AVSD (CAVSD is characterized by the presence of a common atrio-ventricular (AV orifice, an inter-atrial communication, and a ventricular septal defect (VSD. Results of surgical correction of atrio-ventricular septal defects (AVSDs have improved over the last decades; however, the need for reoperation for left atrio-ventricular valve regurgitation, after primary AVSD repair remains a major concern. The aim of our study is to assess the outcome of the routine leaflet augmentation technique in CAVSD repair. A retrospective database and chart review analysis of all patients who underwent AV canal repair at king Abdul-Aziz Cardiac Center during period from 1999 to September 2014 was conducted. Demographic data, associated anomalies, operative data, ICU and hospital course were reviewed. Early outcomes were reviewed for postoperative complications (Chylothorax, complete AV block, Arrhythmias, early mortality and late outcomes were reviewed for Left AV valve regurgitation requiring for re-intervention and late mortality. Two hundred and sixty patients underwent leaflet augmentation technique to repair complete AVSD, between January 1999 and September 2014. The mean age was (131.5 months, and mean weight (6.06 kg. A variety of concomitant procedures were performed at the time of repair of the CAVSD, including a total of 49 patients (18.8% who underwent PDA ligation. Repair of TV (Right AV valve was performed in 11 patients (4.2%, 9 patients (3.46% required RVOTO resection, in 5 patients (1.92%, PA plasty was done and 2 patients (0.76% required ECMO after CAVSD repair. Regarding reoperations, a total of 17 patients (of 260 required reoperation after initial CAVSD repair. The most common indication for reoperation was left AV valve regurgitation in 16 patients (6% in the follow up period up to 15 years. One patient (0.38% required diaphragmatic plication. The overall mortality was 3 patients (1.1%. Leaflet augmentation for the repair of the

  6. Acquired Left Atrial-to-Right Ventricular Shunt with Mitral Valve Incompetence: A Rare Sequela after Repair of Atrioventricular Septal Defect

    OpenAIRE

    Mohapatra, Srikant; Minhas, Harpreet Singh; Virmani, Sanjula; Mishra, Bana Bihari; Mukherjee, Kaushik; Banerjee, Amit

    2009-01-01

    Acquired left ventricular-to-right atrial communication is encountered periodically. This condition is chiefly attributable to surgical mishaps, trauma, endocarditis, or endomyocardial biopsy. In a few instances, a Gerbode-like defect develops after the repair of an atrioventricular septal defect. Our search of the worldwide medical literature revealed just 1 report of a “mirror” occurrence of a Gerbode-like defect: a shunt between the left atrium and the right ventricle.

  7. The comparative relationships between locations of the papillary muscles and electrophysiologic QRS axis in patients with atrioventricular septal defect and common as opposed to separate orifices in the valve guarding the common atrioventricular junction.

    Science.gov (United States)

    Low, Liying; Idriss, Salim F; Anderson, Robert H; Maynard, Charles; Wagner, Galen; Hakacova, Nina

    2017-03-01

    Knowledge regarding factors that influence deviation of the QRS axis is important when seeking to differentiate between physiological and pathological changes. We hypothesised that, in contrast to those patients with an atrioventricular septal defect and common atrioventricular junction permitting only atrial shunting, those associated with ventricular shunting would show no relationship between the positions of the papillary muscles and the degree of the leftward deviation of the QRS axis. We compared the positions of endocardial origin of the papillary muscles, and the frontal plane QRS axis, in patients with atrioventricular septal defects and common atrioventricular junction permitting exclusively atrial as opposed to atrial and ventricular shunting. We analysed 18 patients with atrial and ventricular shunting and 23 patients with exclusively atrial shunting. The correlation coefficient between the ratio of distances of the papillary muscles from the mid-septum and the amount of leftward deviation in the frontal plane QRS axis was 0.1 (p=0.4) in those with ventricular shunting and 0.26 (p=0.01) in those with exclusively atrial shunting. In contrast to patients with the so-called primum form of atrioventricular septal defect, in whom the locations of the papillary muscles correlate with the degree of QRS axis, such relationships are lacking in patients with defects permitting both atrial and ventricular shunting. It may be, therefore, that the presence of ventricular shunting and/or their younger age causes pressure overload, which negates the leftward QRS forces caused by the abnormally positioned papillary muscles.

  8. Late complete atrioventricular block after closure of an atrial septal defect with a gore septal occluder (GSO™)

    Science.gov (United States)

    Sigler, Matthias; Priessmann, Helga

    2015-01-01

    Temporary intermittent complete heart block (CHB) occurred the day after interventional closure of an ASD with a 30 mm Gore Septal Occluder (GSO™) in a 2 years and 11‐month‐old female. CHB disappeared without further treatment and stable sinus rhythm recovered within 3 days. Only short episodes of 2nd degree AV‐block (Wenckebach periodicity) at rare intervals were documented in Holter‐monitors the following 2 months. Eleven months after device implantation the patient suffered from long lasting episodes of CHB. Surgical removal of the device resulted in incomplete recovery of AV‐conduction. Histopathological work‐up of the explanted GSO showed complete endothelialization of the device and regular scar formation. One year after surgery, the child had sinus rhythm during daytime but needed VVI‐pacing while sleeping. Young age, inferior localization of the defect, and use of a large device have been individual risk factors for CHB in this patient. Clinical course and histologic findings indicate that mechanical compression was the only cause for CHB. The cumulative number of reports of CHB after use of different ASD‐devices supports the recommendation to postpone the intervention in asymptomatic patients to preschool‐age. Early removal of a pushing device may increase the chance of complete recovery from CHB. © 2015 Wiley Periodicals, Inc. PMID:26354228

  9. Atrioventricular Canal Defect

    Science.gov (United States)

    ... tract infections. Atrioventricular canal defect can cause recurrent bouts of lung infections. Heart failure. Untreated, atrioventricular canal ... Leaky heart valves Narrowing of the heart valves Abnormal heart rhythm Breathing difficulties associated with lung damage ...

  10. Early Correction of Common Atrioventricular Septal Defects: A Single-Center 20-Year Experience.

    Science.gov (United States)

    Vida, Vladimiro L; Tessari, Chiara; Castaldi, Biagio; Padalino, Massimo A; Milanesi, Ornella; Gregori, Dario; Stellin, Giovanni

    2016-12-01

    Over the past 20 years our policy has been to electively repair common atrioventricular canal defects (CAVCD) in patients between 8 and 12 weeks of age. We sought to evaluate the results of our past 20-year experience. From January 1992 to April 2014, 159 consecutive patients underwent CAVCD repair (133 patients had complete CAVCD and 26 patients had a transitional form of CAVCD). Surgical repair was accomplished with a double-patch (n = 137 [86%]) or a modified single patch (n = 22 [14%]) technique. Median age at operation was 96 days (interquartile range [IQR], 73-128 days); 90 patients were younger than 3 months of age. There were 3 operative (1.9%) and 12 late (7.7%) deaths. Median follow-up time after repair was 8.2 years (IQR, 3.6-15 years). Twenty patients (13%) required reoperation-16 (10%) for left atrioventricular valve (LAVV) regurgitation. Reoperation on the LAVV was more frequent in patients with a dysplastic LAVV preoperatively (p = 0.01; odds ratio [OR], 4.2; 95% confidence interval [CI], 1.33-13.5) and in patients who underwent closure for an absent/incomplete cleft at the time of repair (p = 0.01; OR, 5.4; 95% CI, 1.4-21). Late LAVV performance (regurgitation greater than or equal to moderate or the need for reoperation), including late deaths and patients who underwent reoperation, was significantly worse in patients older than 3 months at repair (10 of 83 patients [12%] versus 20 of 73 patients [27%]; hazard ratio [HR], 2.71; 95% CI, 1.19-6.19) and in patients with LAVV dysplasia (19 of 68 patients [28%] versus 11 of 88 patients [12%]; HR, 3; 95% CI, 1.53-8.51). Individualized early repair of CAVCD is safe and beneficial, with good early and long-term results. Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  11. Complete atrioventricular block after percutaneous device closure of perimembranous ventricular septal defect: A single-center experience on 1046 cases.

    Science.gov (United States)

    Bai, Yuan; Xu, Xu-Dong; Li, Chang-Yong; Zhu, Jia-Qi; Wu, Hong; Chen, Shao-Ping; Chen, Feng; You, Xiao-Hua; Zhao, Xian-Xian; Qin, Yong-Wen

    2015-10-01

    Complete atrioventricular block (cAVB) has been deemed a rare complication after transcatheter closure for ventricular septal defect (VSD). However, this serious event appears to be underrecognized and is worth being investigated further. To determine the incidence and predisposing factors of cAVB associated with closure of VSD using a modified double-disk occluder (MDO). From December 21, 2001 to December 31, 2014, 1046 patients with perimembranous ventricular septal defect underwent percutaneous closure using the MDO. Electrocardiography was evaluated before the procedure, within 1 week after the procedure, and then at 1, 3, 6, and 12 months and every year thereafter. Other baseline and procedural parameters were also evaluated and a comparison between patients requiring pacemakers and those not suffering from cAVB was done. cAVB occurred in 17 patients (1.63%) after the procedure. Of the 17 patients, 8 underwent permanent pacemaker (PPM) implantation. The cAVB occurred within 30 days after the procedure in 14 patients and after 1 year in 3 patients. In comparison patients aged ≤18 years, patients aged >18 years were more prone to cAVB (P = .025). Logistic regression revealed no significant parameter to predict later requirement for PPM. The incidence of cAVB after transcatheter closure of VSD was acceptable, as part of the cAVB population recovered after administration of corticosteroid and application of a temporary pacemaker. Late cAVB (>1 year) appears to make it more difficult to restore normal conduction block. Because of the recurrence of cAVB, life-long follow-up with periodic electrocardiography examination may be mandatory. Copyright © 2015. Published by Elsevier Inc.

  12. Anesthetic management of a child with complete atrioventricular septal defect and single ventricle posted for noncardiac surgery

    Science.gov (United States)

    Mir, Aabid Hussain; Ali, Zulfiqar; Dar, Bashir Ahmad; Naqash, Imtiaz A.; Bashir, Samreena

    2016-01-01

    Congenital heart defects are associated with various physiological disturbances. They pose anesthetic challenges for both cardiac and noncardiac surgeries. Atrioventricular septal defects are due to a developmental failure in the separation of atria and the ventricles into separate chambers and failure in the separation of mitral and tricuspid valves. We present a case of a child (1½ years), weighing 10 kg, diagnosed as congenital hydrocephalus who was planned for ventriculoperitoneal shunt. Child was having an oxygen saturation of 76% on room air. Anesthesia was induced with morphine and propofol. After tracheal intubation, saturation improved to 93%. Anesthesia was maintained with a combination of oxygen and nitrous oxide along with isoflurane. Measures were taken to maintain normovolemia and avoid hypotension, hypoxia, tachycardia, cardiac dysrhythmias and acidosis. The patient remained hemodynamically stable, maintaining arterial blood gasses within normal limits. The overall intraoperative course remained uneventful. At the end of the procedure, patient was reversed with neostigmine 60 mcg/kg and glycopyrrolate 10 mcg/kg. Extubation was done after the child was alert and opening eyes and was shifted to intensive care on oxygen inhalation for further monitoring. PMID:27746571

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

    Science.gov (United States)

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

    2015-11-01

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

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

    Directory of Open Access Journals (Sweden)

    Eduardo Keller Saadi

    1993-06-01

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

  15. De Novo and Rare Variants at Multiple Loci Support the Oligogenic Origins of Atrioventricular Septal Heart Defects.

    Directory of Open Access Journals (Sweden)

    James R Priest

    2016-04-01

    Full Text Available Congenital heart disease (CHD has a complex genetic etiology, and recent studies suggest that high penetrance de novo mutations may account for only a small fraction of disease. In a multi-institutional cohort surveyed by exome sequencing, combining analysis of 987 individuals (discovery cohort of 59 affected trios and 59 control trios, and a replication cohort of 100 affected singletons and 533 unaffected singletons we observe variation at novel and known loci related to a specific cardiac malformation the atrioventricular septal defect (AVSD. In a primary analysis, by combining developmental coexpression networks with inheritance modeling, we identify a de novo mutation in the DNA binding domain of NR1D2 (p.R175W. We show that p.R175W changes the transcriptional activity of Nr1d2 using an in vitro transactivation model in HUVEC cells. Finally, we demonstrate previously unrecognized cardiovascular malformations in the Nr1d2tm1-Dgen knockout mouse. In secondary analyses we map genetic variation to protein-interaction networks suggesting a role for two collagen genes in AVSD, which we corroborate by burden testing in a second replication cohort of 100 AVSDs and 533 controls (p = 8.37e-08. Finally, we apply a rare-disease inheritance model to identify variation in genes previously associated with CHD (ZFPM2, NSD1, NOTCH1, VCAN, and MYH6, cardiac malformations in mouse models (ADAM17, CHRD, IFT140, PTPRJ, RYR1 and ATE1, and hypomorphic alleles of genes causing syndromic CHD (EHMT1, SRCAP, BBS2, NOTCH2, and KMT2D in 14 of 59 trios, greatly exceeding variation in control trios without CHD (p = 9.60e-06. In total, 32% of trios carried at least one putatively disease-associated variant across 19 loci,suggesting that inherited and de novo variation across a heterogeneous group of loci may contribute to disease risk.

  16. Incidence and risk factors of post-operative arrhythmias and sudden cardiac death after atrioventricular septal defect (AVSD) correction: Up to 47years of follow-up.

    Science.gov (United States)

    Kharbanda, Rohit K; Blom, Nico A; Hazekamp, Mark G; Yildiz, Pinar; Mulder, Barbara J M; Wolterbeek, Ron; Weijerman, Michel E; Schalij, Martin J; Jongbloed, Monique R M; Roest, Arno A W

    2018-02-01

    Atrioventricular septal defect (AVSD) has an incidence of 4-5.3 per 10.000 live births and is associated with Down syndrome (DS). Data on arrhythmias and sudden cardiac death (SCD) after AVSD correction is scarce. To analyse the incidence of post-operative arrhythmias and SCD after AVSD correction and explore risk factors. This is a retrospective multicenter study including patients after biventricular AVSD correction. Univariate and multivariate analyses were performed to explore risk factors. A total of 415 patients were included with a mean follow-up duration of 9years (range; operative SVTs were documented in 33 patients (8%) and late post-operative SVTs in 15 patients (3.6%). Non-syndromic AVSD (p=0.022, HR=2.64; 95% CI=1.15-6.04) and cAVSD (p=0.005, HR=3.7; 95% CI=1.39-7.51) were independent risk factors for early post-operative SVTs and significant more late post-operative SVTs occurred in non-syndromic patients (p=0.016, HR=6.38; 95% CI=1.42-28.71) and in pAVSD (p=0.045, HR=3.703; 95% CI=1.03-13.32). Fifteen patients (3.6%) received a pacemaker. Non-syndromic AVSD (p=0.008, HR=15.82; 95% CI=2.04-122.47), pAVSD (p=0.017, HR=6.26; 95% CI=1.39-28.28) and re-operation (p=0.007, HR=4.911; 95% CI=1.54-15.64) were independent risk factors for postoperative pacemaker implantation. Late life-threatening ventricular arrhythmias and SCD occurred in 0.5% and 1.7% respectively. There is good long-term survival after AVSD correction and incidence of SCD is low. Non-syndromic AVSD and cAVSD are independent risk factors for early post-operative SVTs. Non-syndromic AVSD patients have significant more early 3rd degree AVB and late post-operative SVTs. Non-syndromic patients with partial AVSD who have undergone reoperation have a significant higher risk of pacemaker implantation. Copyright © 2017 Elsevier B.V. All rights reserved.

  17. Ventricular septal defect.

    Science.gov (United States)

    Giboney, G S

    1983-05-01

    This article has discussed the ventricular septal defect, its occurrence, physiology, and therapy, and nursing concerns. The VSD, a communication allowing left-to-right shunting of blood at the ventricular level, is the most common congenital heart defect. Surgical correction is often required for large defects before the age of 12 months, and primary correction is now considered standard procedure. Small defects usually close spontaneously, and moderate defects are closely monitored for signs indicating the need for surgical intervention. Nursing care begins with child and family assessment and evaluation of the strengths and weaknesses of the family system. The child's developmental level is a major consideration in formulating interventions for his benefit. Play therapy is a useful vehicle in relating to the child in a nonthreatening manner preoperatively and in allowing the child to work through his hospitalization postoperatively. Maintaining the physical integrity of a child just out of the operating room is a challenge. Continuing support of the family system is a significant aspect of nursing's responsibility toward child and family. Discharge planning and intervention strive to prepare the family for the transition from hospital to home both physically and emotionally.

  18. Ventricular Septal Defect (VSD)

    Science.gov (United States)

    ... before getting pregnant. If you have a family history of heart defects or other genetic disorders, consider talking with a genetic counselor before getting pregnant. By Mayo Clinic Staff . Mayo Clinic Footer Legal Conditions and Terms ...

  19. Robot-Assisted Partial Atrioventricular Canal Defect Repair and Cryo-Maze Procedure.

    Science.gov (United States)

    Mandal, Kaushik; Srivastava, Aseem R; Nifong, L Wiley; Chitwood, W Randolph

    2016-02-01

    Atrial septal defect is one of the most common congenital heart anomalies in adults. Patients with partial atrioventricular canal defects, previously known as ostium primum atrial septal defect, usually present at an early age, and only a few reach adulthood without surgical correction. Herein, we describe a young woman who presented with an ostium primum defect and severe symptomatic mitral and tricuspid regurgitation with paroxysmal atrial fibrillation. A complex repair was successfully done through a left atrial approach using robot-assistance. Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  20. [Atrioventricular defect with 2 valves associated with absence of the pericardium. Report of a case].

    Science.gov (United States)

    Miranda-Chávez, Irma; Muñoz-Castellanos, Luis; Kimura-Hayama, Erick; Vidal-Rojo, Paola; Figueroa-Solano, Javier; de Micheli Serra, Alfredo; Buendía-Hernández, Alfonso

    2013-01-01

    We present a case of a patient with a cardiac malformation that represents a form of embryo-anatomical transition of an atrioventricular septal defect between a 2 valves form to a common valve form. This entity was associated with pericardium absence. Throughout several studies we have precisely established a diagnostic sequence by determining the adequate contribution of each method and we have been able to clear out the proper nomenclature of the atrioventricular cushion defect. Copyright © 2012 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. All rights reserved.

  1. Angiographic differentiation of type of ventricular septal defects

    Energy Technology Data Exchange (ETDEWEB)

    Cheon, Mal Soon; Park, Hee Young; Kim, Yang Sook [Marynoll Hospital, Busan (Korea, Republic of)

    1989-06-15

    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.

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

    DEFF Research Database (Denmark)

    Axelsson, Anna; Weibring, Kristina; Havndrup, Ole

    2014-01-01

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

  3. Cerebral infarction and ventricular septal defect.

    Science.gov (United States)

    Shuiab, A

    1989-07-01

    With the availability of contrast echocardiography, patent foramen ovale is frequently detected in patients with stroke, especially in those with no clear etiology and/or the young patient with stroke. Before this report, an association of stroke with ventricular septal defect had not been reported. In this communication, we describe a 38-year-old patient who developed an occipital lobe infarction and who, on investigation, was found to have a ventricular septal defect. Other investigations, which included four-vessel cerebral angiography, collagen disease workup, and coagulation profile, were all normal. We believe this case further extends the spectrum of cerebral ischemic events that may occur with intracardiac shunts.

  4. Anatomic biventricular repair in right isomerism with noncommitted ventricular septal defect.

    Science.gov (United States)

    Katewa, Ashish; Marwah, Ashutosh; Singh, Vishal; Ramaswamy, Arun; Sharma, Rajesh

    2012-07-01

    Biventricular repair in right atrial isomerism is rarely feasible due to associated anomalies of venous connection, ventricular imbalance, nonroutabilty of the interventricular communication, a common atrioventricular junction, and inadequate pulmonary arterial branches. These patients are also often not ideal for univentricular repair due to some of the above associations. We describe a novel surgical technique that was utilized in such a patient for biventricular repair of a child with right atrial isomerism with total anomalous pulmonary venous connection, regurgitant common atrioventricular valve, hypoplastic left ventricle, nonroutable ventricular septal defect, and pulmonary stenosis.

  5. Atrial Septal Defect (For Parents)

    Science.gov (United States)

    ... location, and severity of the defect. Very small ASDs might not need any treatment. In other cases, the cardiologist may recommend follow-up visits for observation. Usually, though, if an ASD hasn't closed on its own by the ...

  6. Anterograde conduction to the His bundle during right ventricular overdrive pacing distinguishes septal pathway atrioventricular reentry from atypical atrioventricular nodal reentrant tachycardia.

    Science.gov (United States)

    Nagashima, Koichi; Kumar, Saurabh; Stevenson, William G; Epstein, Laurence M; John, Roy M; Tedrow, Usha B; Koplan, Bruce A; Michaud, Gregory F

    2015-04-01

    Distinguishing orthodromic atrioventricular reciprocating tachycardia (ORT) using a retrograde septal accessory pathway (AP) from atypical atrioventricular nodal reentrant tachycardia (AVNRT) may be challenging. Specifically, excluding the presence and participation of an AP may require multiple diagnostic maneuvers. The purpose of this study was to assess the relative value of commonly used right ventricular (RV) pacing maneuvers, including identification of anterograde His-bundle activation with entrainment, to differentiate ORT using a retrograde septal AP from atypical AVNRT. From March 2009 to June 2014, 56 patients (28 female; age 43.9 ± 17.4 years) who underwent electrophysiologic study and ablation for supraventricular tachycardia (26 ORT using septal AP and 30 atypical AVNRT) that exhibited a concentric atrial activation pattern and a septal ventriculoatrial interval >70 ms were analyzed. Overdrive pacing maneuvers or ventricular extrastimuli failed on at least 1 occasion to correctly identify a septal AP. Overall, 16 ORT patients and 26 AVNRT patients had successful RV entrainment, and 12 (75%) ORT patients showed anterograde His capture (11 patients) and/or anterograde septal ventricular capture (3 patients). None of the patients with atypical AVNRT showed anterograde conduction to the His bundle with entrainment. RV pacing maneuvers are useful to exclude an AP in patients with AVNRT having concentric atrial activation sequence and a septal ventriculoatrial interval >70 ms; however, none are consistently diagnostic. When observed in this patient population, anterograde His-bundle or septal ventricular capture during RV entrainment was diagnostic for ORT using a septal AP. Copyright © 2015 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

  7. Circulatory adjustments after birth: effects on ventricular septal defect

    Science.gov (United States)

    Rudolph, Abraham M.

    1971-01-01

    The haemodynamic effects and clinical manifestations of congenital heart lesions may be greatly influenced by developmental changes in the circulation. The sequential changes associated with abnormal communications between the systemic and pulmonary circulations are well typified by the effect on ventricular septal defect. The possible influences of ventricular septal defect on the foetal circulation are first presented, and the interrelationship between ventricular septal defect and postnatal adjustments of the circulation are discussed. PMID:5572641

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

    Science.gov (United States)

    Nakanishi, Keisuke; Kawasaki, Shiori; Amano, Atsushi

    2017-01-01

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

  9. Transaortic closure of residual intramural ventricular septal defect.

    Science.gov (United States)

    Belli, E; Houyel, L; Serraf, A; Lacour-Gayet, F; Petit, J; Planché, C

    2000-05-01

    Residual intramural ventricular septal defect is an unusual cause of left-to-right shunt after biventricular repair of conotruncal anomalies. It results from the insertion of the patch within the trabeculated right ventricular free wall related to the ventriculoinfundibular fold creating a communication through the intertrabeculated spaces to the right ventricular cavity. This complication often leads to unsuccessful reoperations unless the exact mechanism of the shunt has been identified. Five patients presented with residual intramural ventricular septal defects. Three had double outlet right ventricle, one pulmonary atresia with ventricular septal defect, and one tetralogy of Fallot. One patient was unsuccessfully reoperated on for closure of the residual ventricular septal defect through the right ventricular approach. The surgical treatment, which consisted of patch closure of the residual intramural ventricular septal defect through aortotomy, was successful in 3 patients. In the 2 remaining patients the hemodynamically insignificant residual intramural ventricular septal defect remained untouched. No mortality or morbidity occurred. Residual intramural ventricular septal defect should be suspected in presence of a residual ventricular septal defect after biventricular repair of conotruncal anomalies. It is not accessible through either atriotomy or right ventriculotomy. The transaortic approach allows an easy treatment of this rare complication.

  10. Ventricular septal defect following blunt chest trauma

    Directory of Open Access Journals (Sweden)

    Lisa Ryan

    2012-01-01

    Full Text Available We present a 32-year-old male with ventricular septal defect (VSD following blunt chest trauma. Traumatic VSD is a rare but potentially life-threatening injury, the severity, course and presentation of which are variable. While the diagnosis of myocardial injury may be challenging, cardiac troponins are useful as a screening and diagnostic test. The proposed pathophysiological mechanisms in the development of traumatic VSD are early mechanical rupture and delayed inflammatory rupture. We conducted a literature review to investigate the pathogenesis, distribution of patterns of presentation, and the associated prognoses in patients with VSD following blunt chest trauma. We found that traumatic VSDs diagnosed within 48 hours were more likely to be severe, require emergency surgery and were associated with a higher mortality. Children with traumatic VSDs had an increased mortality risk. Smaller lesions may be managed conservatively but should be followed up to detect late complications. In both groups elective repair was associated with a good outcome.

  11. Atrial septal defects in Florida panthers.

    Science.gov (United States)

    Cunningham, M W; Dunbar, M R; Buergelt, C D; Homer, B L; Roelke-Parker, M E; Taylor, S K; King, R; Citino, S B; Glass, C

    1999-07-01

    Ostium secundum atrial septal defects (ASDs) were observed in six (3 M, 3 F) of 33 (20 M, 13 F) (18%) Florida panthers (Puma concolor coryi) necropsied by veterinary pathologists between 1985 and 1998. A seventh ASD was found in a female panther necropsied in the field and is included in the pathological description but not the prevalence of ASDs in Florida panthers. One panther (FP205) with severe ASD also had tricuspid valve dysplasia (TVD). Atrial septal defects and/or TVD are believed to have caused or contributed to the deaths of three (9%) Florida panthers in this study. Mean diameter +/- SD of ASDs was 9.0 +/- 4.7 mm (range 3 to 15 mm). Gross pathological changes attributed to ASDs/TVD in severely affected panthers (ASD > or = 10 mm) (n = 4) included mild right ventricular dilatation (n = 3) and hypertrophy (n = 2), mild to severe right atrial dilatation (n = 2), and acute pulmonary edema (n = 3). Panthers with mild ASDs (ASD < or = 5 mm) (n = 3) had no other detectable gross pathological changes associated with the ASDs. Histological examination of lungs of three panthers with severe ASDs revealed mild to moderate dilatation with fibrosis and smooth muscle atrophy of the tunica media of medium to large caliber arteries (n = 2), interstitial and/or pleural fibrosis (n = 2), perivascular fibrosis (n = 1), and acute to chronic edema (n = 3). Twenty-six necropsied panthers were examined one or more times while living; medical records were retrospectively evaluated. Antemortem radiographic, electrocardiographic, and echocardiographic examinations were performed on two panthers with severe ASDs (FP20 and FP205). Thoracic radiographic abnormalities in both included right heart enlargement, and in FP205 (severe ASD and TVD), mild pulmonary overperfusion. Electrocardiographic examination of FP205 revealed a right ventricular hypertrophy pattern, while FP205 had a normal electrocardiogram. Echocardiographic examination of FP20 revealed marked right atrial dilatation

  12. Outcome of transcatheter closure of muscular ventricular septal defects with the Amplatzer ventricular septal defect occluder.

    Science.gov (United States)

    Thanopoulos, B D; Rigby, M L

    2005-04-01

    To present further experience and intermediate term outcome in 30 patients with single muscular ventricular septal defects (MVSDs) who underwent transcatheter closure with the Amplatzer ventricular septal defect occluder (AVSDO). Thirty patients, aged 4 months to 16 years, with MVSDs underwent transcatheter closure with the AVSDO. The device consists of two low profile disks made of Nitinol wire mesh with a 7 mm connecting waist. The prosthesis size (waist diameter) was selected to be equal to the balloon "stretched" diameter of the defect. A 7-9 French sheath was used to deliver the AVSDO. Fluoroscopy and transoesophageal echocardiography guided the procedure. The stretched diameter of the defects ranged from 6-14 mm. The communication was completely occluded in 28 of 30 patients (93% closure rate). One patient (a 4 month old infant) with sustained complete left bundle branch block after the procedure went on to develop complete heart block one year later. No other complications were observed during a mean follow up of 2.2 years (range 0.25-4.5 years). The AVSDO is an efficient prosthesis that can be safely used in the majority of patients with a single MVSD. Further studies are required to establish long term results in a larger patient population.

  13. Spontaneous closure of muscular ventricular septal defects

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    Pejčić Ljiljana

    2017-01-01

    Full Text Available Introduction/Objective. Ventricular septal defect (VSD is the most frequently diagnosed congenital heart anomaly. The prognosis is usually good as it has spontaneous closure evolution, especially small muscular VSDs. The aim of this study was to determine the natural history of isolated muscular VSDs including the frequency of spontaneous closure in relation to location in the muscular septum and the age at the time of closure. Methods. The study included 96 children (52 girls and 44 boys with isolated muscular VSD diagnosed during the first month of life. We analyzed the tendency of spontaneous closure of these defects for the duration of childhood during a follow-up period of 16 years. Two-dimensional color Doppler echocardiography was performed to detect muscular VSD as a primary cardiac lesion. There was significant prevalence of small apical versus trabecular defects and their outcomes were evaluated. Results. Our study evaluated 91 children, 49 (53.8% girls and 42 (46.2% boys who did not undergo surgery. Apically located VSD was diagnosed in 68 (74.7%, while trabecular defects were found in 23 (25.3% children. Spontaneous closure occurred in 56 out of 91 cases (61.5%. The time of spontaneous closure was most commonly recorded during the first six months after birth (46.4%. The overall rate of spontaneous closure was 81.3% by the end of the first year. Apically located ventricular defects underwent spontaneous closure in the majority of patients, in comparison to trabecular ventricular defects (χ2 = 12.581; p < 0.001. Kaplan–Meier analysis demonstrated a significant difference in the average time required for spontaneous closure between the analyzed patient groups (log-rank = 9.64, p = 0.002. Conclusion. The frequency of spontaneous closure of muscular VSDs, especially apically located, is very high in the first six months, especially within the first year of life. It is advisable to detect them early on using color flow imaging and to

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

    Science.gov (United States)

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

    2016-09-01

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

  15. Ventricular septal defect due to blunt chest trauma | Olivier | South ...

    African Journals Online (AJOL)

    Blunt chest trauma may cause cardiac trauma, this possibility often being overlooked. Various anatomical structures may be affected. A case of ventr'icular septal defect due to blunt chest trauma is described and the relevant literature is reviewed.

  16. Recent advances in managing septal defects: atrial septal defects [version 1; referees: 2 approved

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    P Syamasundar Rao

    2017-11-01

    Full Text Available The purpose of this review is to discuss the management of atrial septal defects (ASD, paying particular attention to the most recent developments. There are four types of ASDs: ostium secundum, ostium primum, sinus venosus, and coronary sinus defects. The fifth type, patent foramen ovale—which is present in 25 to 30% of normal individuals and considered a normal variant, although it may be the seat of paradoxical embolism, particularly in adults—is not addressed in this review. The indication for closure of the ASDs, by and large, is the presence of right ventricular volume overload. In asymptomatic patients, the closure is usually performed at four to five years of age. While there was some earlier controversy regarding ASD closure in adult patients, currently it is recommended that the ASD be closed at the time of presentation. Each of the four defects is briefly described followed by presentation of management, whether by surgical or percutaneous approach, as the case may be. Of the four types of ASDs, only the ostium secundum defect is amenable to percutaneous occlusion. For ostium secundum defects, transcatheter closure has been shown to be as effective as surgical closure but with the added benefits of decreased hospital stay, avoidance of a sternotomy, lower cost, and more rapid recovery. There are several FDA-approved devices in use today for percutaneous closure, including the Amplatzer® Septal Occluder (ASO, Amplatzer® Cribriform device, and Gore HELEX® device. The ASO is most commonly used for ostium secundum ASDs, the Gore HELEX® is useful for small to medium-sized defects, and the cribriform device is utilized for fenestrated ASDs. The remaining types of ASDs usually require surgical correction. All of the available treatment modes are safe and effective and prevent the development of further cardiac complications.

  17. Atrial septal defects: Pattern, clinical profile, surgical techniques and ...

    African Journals Online (AJOL)

    Background: Atrial septal defect (ASD) is a congenital heart defect that leads to shunting of blood between left and right atria. It may be asymptomatic and sometimes may present with heart failure. Surgical repair is definitive, but currently non-surgical procedure is used to close the defect. Materials and Methods: It is a ...

  18. Unidirectional ventricular septal valved patch for repair of late presenting ventricular septal defect with aortopulmonary window

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    Neeti Makhija

    2016-01-01

    Full Text Available Management of long standing left to right shunt lesion resulting in elevated pulmonary vascular resistance (PVR is challenging. Limited surgical options are further complicated by an unpredictable postoperative period. Unidirectional valve patch (UVP closure has shown to be useful in cases of the large ventricular septal defect (VSD who present late. We report a case of large aortopulmonary window coexisting with a large VSD with severe pulmonary artery hypertension and significantly elevated PVR that was managed surgically by closure of the window by sandwich technique and closure of the septal defect with a UVP. This report emphasizes the importance of UVP in the management of such patients.

  19. Unidirectional ventricular septal valved patch for repair of late presenting ventricular septal defect with aortopulmonary window

    Science.gov (United States)

    Makhija, Neeti; Narula, Jitin; Keshri, Vikas Kumar; Gupta, Saurabh Kumar; Talwar, Sachin

    2016-01-01

    Management of long standing left to right shunt lesion resulting in elevated pulmonary vascular resistance (PVR) is challenging. Limited surgical options are further complicated by an unpredictable postoperative period. Unidirectional valve patch (UVP) closure has shown to be useful in cases of the large ventricular septal defect (VSD) who present late. We report a case of large aortopulmonary window coexisting with a large VSD with severe pulmonary artery hypertension and significantly elevated PVR that was managed surgically by closure of the window by sandwich technique and closure of the septal defect with a UVP. This report emphasizes the importance of UVP in the management of such patients. PMID:27011704

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

    African Journals Online (AJOL)

    Introduction. Ventricular septal defect (VSD) is a common congenital heart disease (CHD). Spontaneous closure of the VSD may occur, depending on the type and size of defects. This study was conducted to determine the natural history of VSD in a group of Nigerian children. Subjects and methods. Sixty-one children ...

  1. Natural history of ventricular septal defects in Nigerian children

    African Journals Online (AJOL)

    Introduction. Ventricular septal defect (VSD) is a common congenital heart disease (CHD). Spontaneous closure of the VSD may occur, depending on the type and size of defects. This study was conducted to determine the natural history of VSD in a group of Nigerian children. Subjects and methods. Sixty-one children ...

  2. MULTIPLE VENTRICULAR SEPTAL DEFECTS: A NEW STRATEGY

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    Antonio Francesco Corno

    2013-07-01

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

  3. Echocardiographic evaluation of ventricular septal defect haemodynamics

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    Miranović Vesna

    2007-01-01

    Full Text Available Introduction Ventricular septal defect (VSD is an opening in the interventricular septum. 30-50% of patients with congenital heart disease have VSD. Objective The aim of the study was to determine the dependence of the left ventricular diastolic dimension (LVD, left ventricular systolic dimension (LVS, shortening fraction (SF, left atrium (LA, pulmonary artery truncus (TPA on the body surface and compare their values among experimental, control and a group of healthy children. Values of maximal systolic gradient pressure (Pvsd of VSD were compared with children from one experimental and control group. Method Children were divided into three groups: experimental (32 children with VSD that were to go to surgery, control (20 children with VSD who did not require surgery and 40 healthy children. Measurements of LVD, LVS, SF, LA, TPA were performed in accordance to recommendations of the American Echocardiographic Association. The value of Pvsd was calculated from the maximal flow velocity (V in VSD using the following formula: Pvsd=4xVІ (mm Hg. Results For children from the experimental group, the relationship between the body surface and the variability of the LVD was explained with 56.85%, LVS with 66.15%, SF with 4.9%, TPA with 58.92%. For children from the control group, the relationship between the body surface and the variability of LVD was explained with 88.8%, LVS with 72.5%, SF with 0.42%, PA with 58.92%. For healthy children, the relationship between the body surface and the variabilitiy of the LVD was explained with 88.8%, LVS with 88.78%, SF with 5.25% and PA with 84.75%. There was a significant statistical difference between average values of Pvsd in the experimental and control group (p<0.02. Conclusion The presence of the large VSD has an influence on the enlargement of LVD, LVS, SF, TPA. The enlargement of the size of the pulmonary artery depends on the presence of VSD and there is a direct variation in the magnitude of the shunt

  4. An Unusual Mechanism of Closure of Muscular Ventricular Septal Defects

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    Soham Dasgupta

    2017-01-01

    Full Text Available Ventricular septal defects (VSDs are the most common congenital heart defects. Most of the small or moderate size (<6 mm muscular VSDs close spontaneously within the first two years of life. The usual mechanism of spontaneous closure involves muscular tissue encroachment with superimposed fibrosis or primary fibrous tissue formation around the margins of the defect. We describe an unusual mechanism of spontaneous closure of a muscular VSD.

  5. [Surgical correction of symptomatic ventricular septal defects in patients less than 6 months of age].

    Science.gov (United States)

    Cabrera Duro, A; Martínez Corrales, P; Llorente Urcullo, A; Aramburu Arriaga, N; Rodrigo Carbonero, D; Alcíbar Villa, J; Pastor Menchaca, E; Navarro Quintana, C

    1999-10-01

    Our objective was to evaluate the efficiency of a single surgical intervention in patients with symptomatic interventricular septal defects during the first six months of life. Between 1989 and 1997, 42 patients, 20 males and 22 females with an average age of 3.9 +/- 0.3 months and an average weight of 4 +/- 0.4 kg, were operated. Seven suffered from Down's syndrome. All of the patients became symptomatic during the first two months of life. The defect was localized by using Echo-Doppler in all of the cases. Thirty-six had perimembranous ventricular septal defects, 2 were muscular, 3 multiple and 1 was infundibular. The average defect size was 8 +/- 1.2 mm. A catheter was placed in 34 patient with the following results: Left to right shunt with 2.2 +/- 1.2, right ventricle systolic pressure of 57 +/- 20 mmHg (16 with systemic pulmonary pressure) and an average pulmonary pressure of 38 +/- 1.8 mmHg. The average pulmonary vascular resistance was 28 +/- 1.8 U/m2. Deep hypothermia (18 degrees C was applied during the surgery and the average cardiac arrest time was 31 +/- 4 minutes. None of the patients died during or after the surgical procedure. Patients required minimum ionotropic support during the first hours. The average time in the intensive care unit was 3.5 +/- 0.6 days, with an average hospitalization time of 11.2 +/- 2.1 days. Immediate complications included one hypertensive crisis, four junctional ectopic tachycardias, two atrio-ventricular blocks, 1 transient arrhythmia, two atelectasia-pneumonias, two patients with stridor and two sternal infections. During the follow-up period, two patients required a second intervention to repair the patch. We believe that one-time surgery is adequate to correct symptomatic ventricular septal defects.

  6. Complete atrioventricular canal

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    Limongelli Giuseppe

    2006-04-01

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

  7. Sinus venosus atrial septal defect: a rare cause of misplacement of pacemaker leads

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    Bodian M

    2013-07-01

    Full Text Available Malick Bodian,1 Fatou Aw,1 Mouhamadou Bamba Ndiaye,1 Adama Kane,1 Modou Jobe,1 Alioune Tabane,1 Alassane Mbaye,2 Simon Antoine Sarr,1 Maboury Diao,1 Moustapha Sarr,1 Serigne Abdou Bâ1 1Department of Cardiology, Aristide Le Dantec Teaching Hospital, 2Grand Yoff General Hospital, Dakar, Senegal Abstract: Routine implantation of pacemakers and implantable cardioverter defibrillators is not commonly associated with complications. However, in some cases we see misplacement of pacemaker leads which is most often related to the presence of underlying cardiac anomalies. We report the case of misplacement of a pacemaker lead into the left ventricle of a 56-year-old patient paced in VVI/R mode and with a tined type pacemaker lead because of a symptomatic complete atrioventricular block. Electrocardiogram showed a pacemaker-generated rhythm with a right bundle branch block pattern. Chest X-ray showed the pacemaker lead located relatively high in relation to the diaphragm. Echocardiography visualized the pacemaker lead in the left heart chambers (atrium and ventricle, hence confirming its aberrant course. Further, the defect causing its passage to the left heart chambers was a sinus venosus atrial septal defect. The patient reported no complication related to the misplacement of the lead. After a brief period of oral anticoagulation, the lead was inserted into the right ventricle by percutaneous technique. Keywords: pacemaker, lead misplacement, sinus venosus atrial septal defect

  8. The floating harbor syndrome with cardiac septal defect.

    Science.gov (United States)

    Lazebnik, N; McPherson, E; Rittmeyer, L J; Mulvihill, J J

    1996-12-18

    The Floating Harbor syndrome of short stature, very delayed bone age, expressive language delay, and characteristic facial changes has not been associated with cardiac anomalies, except for one patient with pulmonic stenosis. We report on a 10-year-old boy with the syndrome and tetralogy of Fallot with atrial septal defect.

  9. Aspects of surgery for congenital ventricular septal defect

    NARCIS (Netherlands)

    G. Bol-Raap (Goris)

    2007-01-01

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

  10. Three-dimensional echocardiographic assessment of atrial septal defects

    Science.gov (United States)

    German, Charles; Nanda, Navin C.

    2015-01-01

    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. PMID:25566714

  11. Three-dimensional echocardiographic assessment of atrial septal defects

    Directory of Open Access Journals (Sweden)

    Charles German

    2015-01-01

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

  12. [A rare ventricular septal defect: a case report].

    Science.gov (United States)

    Notarangelo, Maria Francesca; Bontardelli, Federico; Taliani, Umberto; Agostinelli, Andrea; Vignali, Luigi; Ardissino, Diego

    2013-04-01

    Left ventricular-right atrial communications, known collectively as the Gerbode defect, are rare types of ventricular septal defects. Acquired forms of this defect have been described as a complication of cardiac surgery, bacterial endocarditis, chest trauma, or myocardial infarction. Diagnosis of this rare defect is challenging, but can be confirmed with echocardiography or cardiac magnetic resonance imaging. Until 6 years ago, these communications were corrected only surgically, often with relatively high mortality. However, few case reports of transcatheter closures of the defects have recently been reported with excellent results. We describe a 69-year-old patient with left ventricular-right atrial communication secondary to mitral valve surgery. The diagnosis was made by transesophageal and real-time three-dimensional echocardiography. The defect was closed percutaneously using an Amplatzer device. At follow-up, there was no residual flow and the patient improved clinically.

  13. Transesophageal echocardiography for incremental value of Amplatezer cribriform septal occluder for percutaneous transcatheter closure of complex septal defects: Case series

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    Shen Kou Tsai

    2017-06-01

    Conclusion: Our patients with complex septal defects were successfully treated by transcatheter closure using an Amplazter cribriform septal occluder device with careful planning based on patient presentation and close interdisciplinary collaboration. RT 3D color Doppler TEE provided precise information for the selection of the appropriate occluder device and facilitated the procedure by guiding the catheter through the often challenging patient anatomy.

  14. 50. Successful percutanous closure of spiral atrial septal defect

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    Mashail Abdulaziz Alobaidan

    2015-10-01

    An unusual morphology of atrial septal defect has been described where there is an apparently “double atrial septum” (Roberson, 2006. The terminology around this lesion has been attributed to be the wide separation of the primary atrial septum (primum septum from the secondary septum (septum secundum and the “spiral” spatial arrangement of the margins of the atrial septal defect (ASD has led to the term spiral ASD to describe this arrangement. This has been described to be associated with a high risk of device embolization or technical failure in the placement of an occluder device. We report the echocardiographic findings and outcome of a patient with this form of ASD in whom percutaneous occlusion was successful of which is considered up to date to be the first successful closure of this type of ASD.

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

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    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.

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

    Science.gov (United States)

    Roldán, Francisco-Javier; Vargas-Barrón, Jesús; Vázquez-Antona, Clara; Castellanos, Luis Muñoz; Erdmenger-Orellana, Julio; Romero-Cárdenas, Ángel; Martínez-Ríos, Marco-Antonio

    2008-01-01

    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. PMID:18638394

  17. The surgical anatomy of ventricular septal defect part IV: double outlet ventricle.

    Science.gov (United States)

    Anderson, R H; Ho, S Y; Wilcox, B R

    1996-01-01

    In this fourth part of our series of articles concerned with the surgical anatomy of ventricular septal defects (VSDs), we have analyzed the arrangements when both arterial trunks arise from the same ventricle. The essence of these anomalies is that the interventricular communication is an integral part of the circulation. Unless the surgeon constructs an alternative route of exit, closure of this defect would isolate one of the ventricles. The usual surgical approach, therefore, is to patch the hole between the ventricles into one or other of the subarterial outflow tracts. This means that all the components of the ventricular outflow tracts are of potential surgical importance. In hearts with double outlet right ventricle, the VSD can be categorized as being subaortic, subpulmonary, doubly committed, or non-committed. It is also important to determine whether its anatomical borders, as seen from the right ventricle, are in part fibrous or exclusively muscular, so as to establish the location of the atrioventricular conduction axis. It is possible, according to the nature of these borders, to place the defects into one of three groups, perimenbranous, muscular, or doubly committed and juxta-arterial. The size of the defect is another important surgical consideration. Double outlet left ventricle is a significantly more rare malformation, but the rules for determining the disposition of the conducting tissues are the same.

  18. Pulmonary edema following transcatheter closure of atrial septal defect

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    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.

  19. Transcatheter device closure of a traumatic ventricular septal defect

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    Mohamed Kasem

    2014-01-01

    Full Text Available A traumatic ventricular septal defect (VSD resulting from blunt chest injury is a very rare event in children. The clinical symptoms and timing of presentation are variable, so diagnosis and management of traumatic VSD may be challenging. Decision to close the traumatic VSD is usually based on a combination of severity of heart failure symptoms, hemodynamics, and defect size. We present a case of a 7-year-old boy who was run over by a truck and presented with head and liver injury initially. He was subsequently found to have a traumatic VSD. The VSD was closed percutaneously.

  20. Embryonic Ethanol Exposure Dysregulates BMP and Notch Signaling, Leading to Persistent Atrio-Ventricular Valve Defects in Zebrafish.

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    Swapnalee Sarmah

    Full Text Available Fetal alcohol spectrum disorder (FASD, birth defects associated with ethanol exposure in utero, includes a wide spectrum of congenital heart defects (CHDs, the most prevalent of which are septal and conotruncal defects. Zebrafish FASD model was used to dissect the mechanisms underlying FASD-associated CHDs. Embryonic ethanol exposure (3-24 hours post fertilization led to defects in atrio-ventricular (AV valvulogenesis beginning around 37 hpf, a morphogenetic event that arises long after ethanol withdrawal. Valve leaflets of the control embryos comprised two layers of cells confined at the compact atrio-ventricular canal (AVC. Ethanol treated embryos had extended AVC and valve forming cells were found either as rows of cells spanning the AVC or as unorganized clusters near the AV boundary. Ethanol exposure reduced valve precursors at the AVC, but some ventricular cells in ethanol treated embryos exhibited few characteristics of valve precursors. Late staged larvae and juvenile fish exposed to ethanol during embryonic development had faulty AV valves. Examination of AVC morphogenesis regulatory networks revealed that early ethanol exposure disrupted the Bmp signaling gradient in the heart during valve formation. Bmp signaling was prominent at the AVC in controls, but ethanol-exposed embryos displayed active Bmp signaling throughout the ventricle. Ethanol exposure also led to mislocalization of Notch signaling cells in endocardium during AV valve formation. Normally, highly active Notch signaling cells were organized at the AVC. In ethanol-exposed embryos, highly active Notch signaling cells were dispersed throughout the ventricle. At later stages, ethanol-exposed embryos exhibited reduced Wnt/β-catenin activity at the AVC. We conclude that early embryonic ethanol exposure alters Bmp, Notch and other signaling activities during AVC differentiation leading to faulty valve morphogenesis and valve defects persist in juvenile fish.

  1. Gerbode Defect—A Rare Defect of Atrioventricular Septum and Tricuspid Valve

    Science.gov (United States)

    Gangurde, Pranil; Mahajan, Ajay

    2015-01-01

    Left ventricular to right atrial communications (the Gerbode defect) are rare types of ventricular septal defect and present as direct or an indirect type. We hereby, report two cases, one direct and another indirect type. Cardiopulmonary bypass surgery was done and a successful suture closure of ventricularseptal defect using pericardial patch was performed. PMID:26500939

  2. [A case of tricuspid pouch associated with ventricular septal defect and functional left ventricular-right atrial communication].

    Science.gov (United States)

    Watanabe, H; Maeda, K; Minowa, T; Shimasaki, T; Shimanuki, T; Kasuya, S; Sakashita, I; Takano, S; Kato, K

    1992-03-01

    We reported a 60-year-old woman with tricuspid pouch associated with ventricular septal defect and subsequent left ventricular-right atrial communication. Preoperative angiographic findings suggested the presence of membranous septal aneurysm, ventricular and atrial septal defects, and tricuspid insufficiency. However, at operation, besides perimembranous inlet type ventricular septal defect, a pouch, 1.5 cm in diameter was found in the adjacent part to the septal leaflet within the anterior one of the tricuspid valve. The ventricular septal defect was closed with a patch. From these findings, it is speculated that the tricuspid pouch was formed by the effect of jet stream through the ventricular septal defect.

  3. Tetralogy of Fallot and atrial septal defect in a white Bengal Tiger cub (Panthera tigris tigris)

    OpenAIRE

    Pazzi, Paolo; Lim, Chee K; Steyl, Johan

    2014-01-01

    A 3-week-old female white Bengal Tiger cub (Panthera tigris tigris) presented with acute onset tachypnoea, cyanosis and hypothermia. The cub was severely hypoxaemic with a mixed acid–base disturbance. Echocardiography revealed severe pulmonic stenosis, right ventricular hypertrophy, high membranous ventricular septal defect and an overriding aorta. Additionally, an atrial septal defect was found on necropsy, resulting in the final diagnosis of Tetralogy of Fallot with an atrial septal defect ...

  4. Tetralogy of Fallot and atrial septal defect in a white Bengal Tiger cub (Panthera tigris tigris).

    Science.gov (United States)

    Pazzi, Paolo; Lim, Chee K; Steyl, Johan

    2014-03-04

    A 3-week-old female white Bengal Tiger cub (Panthera tigris tigris) presented with acute onset tachypnoea, cyanosis and hypothermia. The cub was severely hypoxaemic with a mixed acid-base disturbance. Echocardiography revealed severe pulmonic stenosis, right ventricular hypertrophy, high membranous ventricular septal defect and an overriding aorta. Additionally, an atrial septal defect was found on necropsy, resulting in the final diagnosis of Tetralogy of Fallot with an atrial septal defect (a subclass of Pentalogy of Fallot). This report is the first to encompass arterial blood gas analysis, thoracic radiographs, echocardiography and necropsy findings in a white Bengal Tiger cub diagnosed with Tetralogy of Fallot with an atrial septal defect.

  5. Right-to-left shunting through the unidirectional valved patch after closure of ventricular septal defect

    National Research Council Canada - National Science Library

    Sachin Talwar; Poonam Kapoor; Jitin Narula; Vikas Keshri; Shiv Choudhary; Balram AIran

    2017-01-01

    Postoperative transesophageal echocardiography images of a patient undergoing unidirectional valved patch closure of ventricular septal defect in the setting of severe pulmonary hypertension are presented...

  6. Acquired ventricular septal defect due to infective endocarditis

    Directory of Open Access Journals (Sweden)

    Randi E Durden

    2018-01-01

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

  7. Endocardial cushion defect

    Science.gov (United States)

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

  8. Klippel-Feil syndrome associated with atrial septal defect.

    Science.gov (United States)

    Bejiqi, Ramush; Retkoceri, Ragip; Bejiqi, Hana; Zeka, Naim; Maloku, Arlinda; Berisha, Majlinda

    2013-01-01

    Three major features result from this abnormality: a short neck, a limited range of motion in the neck, and a low hairline at the back of the head. Most affected people have one or two of these characteristic features. Less than half of all individuals with Klippel-Feil syndrome have all three classic features of this condition. The etiology of Klippel-Feil syndrome and its associated conditions is unknown. The syndrome can present with a variety of other clinical syndromes, including fetal alcohol syndrome, Goldenhar syndrome, anomalies of the extremities etc. Associated anomalies occur in the auditory system, neural axis, cardiovascular system, and the musculoskeletal system. Cardiovascular anomalies, mainly septal defects, were found in 7 patients in Hensinger's series, with 4 of these individuals requiring corrective surgery. In our case we have had registered a nonrestrictive atrial septal defect and corrective surgical intervention at age 18 months in the Santa Rosa Children's Hospital (USA) has been done successfully. Careful examinations of specialist exclude anomalies in other organs and systems. Radiographs and MRI of the thoracic and lumbosacral spine are obtained and other anomalies have been excluded.

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

    Directory of Open Access Journals (Sweden)

    Henry D I De′Ath

    2012-01-01

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

  10. Right ventricular remodeling in restrictive ventricular septal defect

    Science.gov (United States)

    Monreal, Gretel; Youtz, Dane J.; Phillips, Alistair B.; Eyman, Mahala E.; Gorr, Matthew W.; Velten, Christina; Lucchesi, Pamela A.; Wold, Loren E.; Gerhardt, Mark A.

    2014-01-01

    Restrictive ventricular septal defect (rVSD) presents with little/no hemodynamic aberrations despite a patent septal defect. Clinically, these patients are observed with the hope that the defect will functionally close over time without the need for surgical repair and development of heart failure. Without evidence supporting a definitive therapeutic strategy, rVSD patients may have increased risk of a poor outcome. We tested the hypothesis that rVSD results in subclinical RV diastolic dysfunction and molecular remodeling. Five pigs underwent surgical rVSD creation. Echocardiography, hemodynamics, myocyte contractility experiments, and proteomics/Western blot were performed 6-weeks post-rVSD and in controls. *p < 0.05. LV and RV hemodynamics in rVSD were comparable to controls. The tricuspid valve early/late diastolic inflow velocity ratio (TV E/A ratio) decreased from 1.6 ± 0.05 in controls to 1.0 ± 0.08* in rVSD, indicating RV diastolic dysfunction. rVSD RV myocytes showed abnormalities in contraction (departure velocity (Vd) − 51%*, Vd time +55%*) and relaxation (return velocity (Vr) −50%*, Vr time +62%*). Mitochondrial proteins (fatty acid, TCA cycle) increased 2-fold*, indicating heightened RV work. Desmin protein upregulated 285%* in rVSD RV myocardium, suggesting cytoskeletal remodeling. rVSD causes RV diastolic dysfunction, myocyte functional impairment, and mitochondrial/cytoskeletal protein upregulation in our model. Desmin upregulation may hinder sarcomeric organization/relaxation, representing a key subclinical early marker for future RV dysfunction. TV E/A measurements are a non-invasive modality to assess rVSD patients for diastolic dysfunction. Translational research applications may lead to fundamental changes in the clinical management of rVSD by providing evidence for early repair of the defect. PMID:20637777

  11. Percutaneous closure of atrial septal defect with situs solitus and dextrocardia.

    Science.gov (United States)

    Galal, Mohammed Omar; Khan, Muhammad Arif; El-Segaier, Milad

    2015-02-01

    Percutaneous closure of secundum atrial septal defect associated with situs solitus and dextrocardia has not been reported previously. We describe the technical difficulties encountered during transcatheter closure of a secundum atrial septal defect in a 19-month-old girl with situs solitus and dextrocardia. © The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  12. Value of the Ratio of Occluder Versus Atrial Septal Length for Predicting Arrhythmia Occurrence after Transcatheter Closure in Children with Ostium Secundum Atrial Septal Defect

    Directory of Open Access Journals (Sweden)

    Mei Jin

    2015-01-01

    Full Text Available Background: Transcatheter occlusion has been applied to treat ostium secundum atrial septal defect (OS ASD since 1997. During the clinical practice, several postoperative complications including arrhythmia have been reported. This study aimed to evaluate the value of the ratio of atrial septal occluder (ASO versus atrial septal length (ASL for predicting arrhythmia occurrence after transcatheter closure in children with OS ASD. Methods: Six hundred and fifty-one children diagnosed with OS ASD underwent occlusion procedures after completing routine examinations. The onsets and types of arrhythmia both during and after the occlusion procedures were monitored. Treatments were given based on the individual types of arrhythmia. The binary logistic regression analysis and receiver-operating characteristic (ROC curve were used in the analysis of value of the ratio of ASO/ASL for predicting postoperative arrhythmia occurrence. Results: Transcather occlusions were conducted in 651 children, among whom 7 children had different types and degrees of arrhythmia, with an incidence of 1.1%. The types of arrhythmia included sinus bradycardia, atrial premature beats, bundle branch block, and different degrees of atrioventricular block. Normal electrocardiograph findings were resumed in these 7 patients following active therapies such as corticoids, nutrition, and surgeries. The binary logistic regression and ROC analysis suggested that the ratio of ASO/ASL exhibited an intermediate predictive value for predicting arrhythmia occurrence after occlusion procedures. A cut-off value of 0.576 in the ratio provided a sensitivity of 87.5% and a specificity of 76.2% with an area under the ROC curve of 0.791 (95% confidence intervals, 0.655-0.926; P < 0.05 in predicting arrhythmia occurrence after the closure procedures. Conclusions: The ratio of ASO/ASL might be a useful index for predicting arrhythmia occurrence after closure procedures in children with OS ASD.

  13. Transcatheter Device Closure of Muscular Ventricular Septal Defect

    Directory of Open Access Journals (Sweden)

    Yun-Ching Fu

    2011-02-01

    Full Text Available Traditional treatment of muscular ventricular septal defect (VSD is surgical closure, but it carries considerable morbidity and mortality. The Amplatzer muscular VSD occluder (AGA Medical Corporation, Plymouth, MN, USA was approved by the United States Food and Drug Administration in September 2007 and by the Taiwan Department of Health in September 2009. It is a self-expandable double-disc device made from a nitinol wire mesh. Dacron polyester patches are sewn into each disc and the connecting waist to increase the thrombogenicity of the device. Many reports have shown that transcatheter device closure of muscular VSDs is effective and safe. It has no scar, less pain, shorter hospital stay, and less cost compared to the traditional open heart surgery.

  14. Totally thoracoscopic surgery for the treatment of atrial septal defect without of the robotic Da Vinci surgical system.

    Science.gov (United States)

    Liu, Gaoli; Qiao, Yanli; Ma, Liming; Ni, Liangchun; Zeng, Shanguang; Li, Qingchen

    2013-05-01

    More and more surgeons and patients focus on the minimally invasive surgical techniques in the 21st century. Totally thoracoscopic operation provides another minimal invasive surgical option for patients with ASD (atrial septal defect). In this study, we reported our experience of 61 patients with atrial septal defect who underwent totally thoracoscopic operation and discussed the feasibility and safety of the new technique. From January 2010 to October 2012, 61 patients with atrial septal defect underwent totally thoracoscopic closure but not traditional median sternotomy surgery. We divided the 61 patients into two groups based on the operation sequence. The data of group A (the first 30 cases) and group B (the last 31 cases). The mean age of the patients was 35.1 ± 12.8 years (range, 6.3 to 63.5 years), and mean weight was 52.7 ± 11.9 kg (range, 30.5 to 80 kg). Mean size of the atrial septal defect was 16.8 ± 11.3 mm (range, 13 to 39 mm) based on the description of the echocardiography. All patients underwent totally thoracoscopy successfully, 36 patients with pericardium patch and 25 patients were sutured directly. 7 patients underwent concomitant tricuspid valvuloplasty with Key technique. No death, reoperation or complete atrioventricular block occurred. The mean time of cardiopulmonary bypass was 68.5 ± 19.1 min (range, 31.0 to 153.0 min), the mean time of aortic cross-clamp was 27.2 ± 11.3 min (range, 0.0 to 80.0 min) and the mean time of operation was 149.8 ± 35.7 min (range, 63.0 to 300.0 min). Postoperative mechanical ventilation averaged 4.9 ± 2.5 hours (range, 3.5 to 12.6 hours), and the duration of intensive care unit stay 20.0 ± 4.8 hours (range, 15.5 to 25 hours). The mean volume of blood drainage was 158 ± 38 ml (range, 51 to 800 ml). No death, residual shunt, lung atelectasis or moderate tricuspid regurgitation was found at 3-month follow-up. The totally thoracoscopic operation is feasible and safe for

  15. Clinically silent atrial septal defects with evidence for cerebral embolization.

    Science.gov (United States)

    Harvey, J R; Teague, S M; Anderson, J L; Voyles, W F; Thadani, U

    1986-11-01

    The cause of stroke in young patients frequently cannot be established. Eleven consecutive patients, age 50 and younger, had clinical evidence of cerebral embolization. Results of physical, radiographic, electrocardiographic, and two-dimensional echocardiographic examinations were normal in all patients. During normal respiration, eight of the patients had right-to-left shunts at the atrial level shown by microcavitation contrast two-dimensional echocardiography. Six of the eight patients with positive contrast studies had cardiac catheterization. Five of six patients had an atrial septal defect, normal right and left heart pressures, and small right-to-left shunts during a Valsalva strain. Four patients had surgical closure of the defect, which ranged in size from 5 to 10 mm. The remaining patients received anticoagulants. Interatrial communications appear to be common in young patients with stroke, suggesting paradoxical embolization as a possible mechanism. Contrast two-dimensional echocardiography should be done in such patients because it is the only noninvasive technique that reliably finds these defects.

  16. An association between left axis deviation and an aneurysmal defect in children with a perimembranous ventricular septal defect.

    OpenAIRE

    Farrú-Albohaire, O; Arcil, G; Hernández, I

    1990-01-01

    Conspicuous left axis deviation was found in two thirds (27 patients) of 44 children with a perimembranous ventricular septal defect, echocardiographic signs of apposition of the septal tricuspid valve leaflet, and an aneurysm of the membranous septum. In 10 patients earlier electrocardiograms did not show left axis deviation; this feature appeared when the aneurysm of the membranous septum was first seen on the echocardiogram. None of the 44 controls with perimembranous ventricular septal de...

  17. MORTAL PULPOTOMY ON CHILDREN WITH VENTRICULAR SEPTAL DEFECT

    Directory of Open Access Journals (Sweden)

    Devi N. R. Devy

    2006-04-01

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

  18. Gerbode ventricular septal defect diagnosed at cardiac MR imaging: case report.

    Science.gov (United States)

    Cheema, Omar M; Patel, Ankit A; Chang, Su Min; Shah, Dipan J

    2009-07-01

    This report describes the ability of cardiac magnetic resonance (MR) imaging to depict an unusual cardiac defect. A type of ventricular septal defect called the Gerbode defect, which results in a communication between the left ventricle and the right atrium, is presented. To the authors' knowledge, this is the first time cardiac MR imaging has been utilized to characterize this defect. (c) RSNA, 2009.

  19. Sequential management of post-myocardial infarction ventricular septal defects.

    Science.gov (United States)

    Trivedi, Kalyani R; Aldebert, Philippe; Riberi, Alberto; Mancini, Julien; Levy, Gilles; Macia, Jean-Christophe; Quilicci, Jacques; Habib, Gilbert; Fraisse, Alain

    2015-05-01

    Ventricular septal defect (VSD) after acute myocardial infarction is a catastrophic event. We describe our multicentre experience of a defect closure strategy that combined surgery and transcatheter closure. Data were obtained by retrospective chart review. Twenty patients (mean age, 67 years) from three centres were studied. Median time from myocardial infarction to VSD was 6 (range, 3-9) days. Acute cardiogenic shock occurred in 12 (60%) patients. Median defect diameter by echocardiography was 18 (range, 12-28) mm. Median time to first surgical or percutaneous closure was 18 (range, 4-96) days. Twenty-seven procedures were performed in the 20 patients. Surgical closure was undertaken in 14 patients and contraindicated in eight, six of whom underwent percutaneous closure; the other two, after reconsideration, proceeded to surgical closure. No procedural complications occurred with percutaneous closure. Percutaneous closure patients were older than surgical patients (75 vs. 64 years; P=0.01) and had a higher mean logistic EuroSCORE (87% vs. 67%; P=0.02). Rates of residual shunt and mortality did not differ between surgical and percutaneous patients (P=0.12 and 0.3, respectively). Those who underwent early VSD closure (<21 days after myocardial infarction) had higher rates of residual shunt (P=0.09) and mortality (P=0.01), irrespective of closure strategy. The mortality rate was also higher after early percutaneous closure (P=0.001), but not after early surgery. Finally, predicted mortality (logistic EuroSCORE) was higher than hospital mortality (≤30 days) in our patient population (75% vs. 30%; P=0.01). Vigorous pursuit of closure of post-myocardial infarction VSD with a sequential surgical and/or percutaneous approach is recommended for improved outcomes. Copyright © 2015. Published by Elsevier Masson SAS.

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

    Directory of Open Access Journals (Sweden)

    Mustafa Yıldız

    2012-08-01

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

  1. Coronary artery to pulmonary artery communications in pulmonary atresia with ventricular septal defect

    National Research Council Canada - National Science Library

    Sridhar, Anuradha; Subramanyan, Raghavan; Cherian, Kotturathu Mammen

    2013-01-01

    ...% of patients with pulmonary atresia and ventricular septal defect (PA-VSD). A diligent look for these abnormal communications is important to prevent perioperative complications and achieve a complete repair...

  2. [Intraoperative transesophageal echocardiography in robotic perimembranous ventricular septal defect repair].

    Science.gov (United States)

    Wang, Yao; Gao, Chang-Qing; Wang, Gang; Shen, Yan-Song; Wang, Jia-Li; Xiao, Cang-Song; Yang, Ming

    2013-11-01

    To explore the role of intraoperative transesophageal echocardiography (TEE) in robotic perimembranous ventricular septal defect (VSD) repair. A retrospective analysis was conducted with intraoperative TEE data of 18 consecutive patients who underwent robotic perimembranous VSD repair from January 2009 to August 2012. (1) Before cardiopulmonary bypass (CPB), TEE was performed to document the anatomic types, numbers, and the size of VSD. The procedures were predetermined by the surgeon according to TEE information. (2) During the establishment of peripheral CPB, TEE was used to guide the placement of cannulae in inferior vena cava (IVC), superior vena cava (SVC), and ascending aorta (AAO). (3) After weaning from CPB, TEE was conducted to evaluate the effect of the procedure. (1) Accuracy of TEE was 100% for diagnosing the anatomic types of VSD. All the surgical procedures were performed based on the predetermined information. (2) Under TEE guidance, all the cannulae in the SVC, IVC and AAO were located in correct positions. (3) In all patients, TEE confirmed successful VSD repair. TEE is a useful tool in the assessment of robotic perimembranous VSD repair.

  3. Device Closure of Small Ventricular Septal Defects: When and Why?

    Directory of Open Access Journals (Sweden)

    IB Vijayalakshmi

    2014-10-01

    Full Text Available Ventricular septal defect (VSD accounts for approximately 20-30 % of all forms of congenital heart disease (CHD. They were traditionally closed surgically in the past. The surgery though safe carries the risk of morbidity, complete heart block, wound infection and thoracotomy scar on the chest. On weighing the risk and benefit, the small VSDs were not submitted to surgery in the past. So for many years it has been taught that the small VSDs are to be left alone and surgery is not recommended. Many clinicians believed in spontaneous closure of VSDs. Hence they advised the parents to wait for spontaneous closure till the child is 9 years old. But what if the VSD does not close by 10 years is the question. Are they normal as grown up congenital heart (GUCH. The Jane Somerville GUCH unit showed that spontaneous closure occurred only in 10% between the age of 17 and 45 (mean - 27 years in small VSDs in adults. What is worth noting is about 25% had serious complications: infective endocarditis (11%, progressive aortic regurgitation (5%, age-related symptomatic arrhythmias (8.5% like atrial fibrillation. This means that asymptomatic small VSDs in childhood is not necessarily benign during adult life. This raises the question, when we have a safe non-surgical device closure available to close the small VSDs, should we put the future lives of the young in danger by not giving the benefit of technology to them?

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

    DEFF Research Database (Denmark)

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

    2017-01-01

    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...... 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...... population cohort. The adjusted HR 30 days after closure was 1.4 (95% CI: 1.2-1.7), and it was 2.4 (95% CI: 2.0-2.9) for patients without closure. Conclusion: Overall, ASD patients had a higher long-term mortality than a general population cohort matched on birth year and gender. Our data indicate a lower...

  5. First successful totally endoscopic atrial septal defect closure with robotic assistance

    Directory of Open Access Journals (Sweden)

    А. Н. Архипов

    2015-10-01

    Full Text Available Median sternotomy has long been a conventional surgical approach in patients with atrial septal defects. However, with improvements in surgical techniques and introduction of new technologies, minimally invasive procedures are gaining more and more attention of surgeons managing congenital heart diseases. In this paper we introduce a case of successful totally thoracoscopic atrial septal defect closure performed by using the da Vinci surgical system in a 25-year old female.

  6. Total endoscopic robotic atrial septal defect repair in a patient with dextrocardia and situs inversus totalis

    Science.gov (United States)

    Iino, Kenji; Watanabe, Go; Ishikawa, Norihiko; Tomita, Shigeyuki

    2012-01-01

    Situs inversus with mirror-image of the heart is a rare condition. The present report describes a case of a patient with dextrocardia with situs inversus who had atrial septal defect with multiple holes in the fossa ovalis. The patient underwent total endoscopic atrial septal defect repair using the da Vinci surgical system. This procedure was achieved safely with good clinical and excellent cosmetic results. PMID:22200951

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

    Directory of Open Access Journals (Sweden)

    Er-Ping Xi

    2012-11-01

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

  8. A modified infarct exclusion technique for repair of anteroapical postinfarction ventricular septal defect.

    Science.gov (United States)

    Bayezid, Omer; Turkay, Cengiz; Golbasi, Ilihan

    2005-01-01

    Ventricular septal defects complicate approximately 1% to 2% of cases of acute myocardial infarction. Such postinfarction defects require urgent surgical treatment because, on medical treatment alone, 60% to 70% of patients die within the first 2 weeks. Despite the development of various surgical techniques for repair of postinfarction ventricular septal defect, the condition carries a high risk of recurrence and subsequent death. We describe a modification of the infarct exclusion technique in which the septal portion of the patch is reinforced by the right ventricular free wall. This modification appears to prevent leaks to the right ventricle through the ventricular septal defect, from anywhere around the patch. We applied this modified technique to 4 patients with anteroapical postinfarction ventricular septal defect. There was 1 early death, due to mesenteric artery occlusion secondary to embolus. No residual shunt was found during the postoperative period. We believe that our modification to the infarct exclusion technique might reduce both operative mortality and recurrence, by supporting friable endocardial tissue with right ventricular wall. We suggest that it be considered for use in patients with anteroapical ventricular septal defect and no severe right ventricular dysfunction.

  9. Transcatheter closure of perimembranous ventricular septal defects in infants and children using the Amplatzer perimembranous ventricular septal defect occluder.

    Science.gov (United States)

    Thanopoulos, Basil Vasilios D; Rigby, Michael L; Karanasios, Evangelos; Stefanadis, Christodoulos; Blom, Nico; Ottenkamp, Jaap; Zarayelyan, Armine

    2007-04-01

    There are very few published reports of the transcatheter closure of perimembranous ventricular septal defects (PMVSDs) using the Amplatzer PMVSD occluder with encouraging initial results. This report presents initial and 1-year results from 54 patients with PMVSDs who underwent transcatheter closure at 5 different institutions with the Amplatzer PMVSD occluder. Sixty-five patients with PMVSDs were enrolled at 5 European centers. Eleven of the 65 patients did not fulfill the patient selection criteria at the initial echocardiographic evaluation or at cardiac catheterization. As a result, a total of 54 patients underwent attempted transcatheter closure using the Amplatzer PMVSD occluder. The median age of the patients was 5.1+/-3.6 years (range 0.3 to 13), and the median weight 18.5+/-10.3 kg (range 5 to 45). Devices were permanently implanted in 49 of 54 patients. Complete occlusion of the communication at 1-year follow-up was observed in 46 of 49 patients (94%). Main early procedural complications included (1) device embolization (2 patients), (2) severe bradycardia with hemodynamic compromise (2 patients), and (3) Mobitz II (2:1) heart block (1 patient). Late procedural complications included complete heart block (1 patient). No other complications were observed during follow-up. In conclusion, the Amplatzer PMVSD occluder is promising device that can be used for transcatheter closure in selected patients with PMVSDs. Further studies and long-term follow-up are required before this technique enters routine clinical practice.

  10. Double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly) and other complex congenital cardiac malformations in an American Quarter Horse foal.

    Science.gov (United States)

    Kohnken, Rebecca; Schober, Karsten; Godman, Jennifer; Gardner, Alison; Jenkins, Tiffany; Schroeder, Eric; Baker, Peter; Dunbar, Laura

    2018-02-01

    A 4-week-old American Quarter Horse colt presented with a recent history of diarrhea and decreased activity level. On initial physical examination, the animal was bright and alert and major findings were limited to a loud systolic heart murmur radiating widely over both sides of the thorax. While in the hospital, the clinical condition of the foal warranted further imaging to determine the cause and extent of cardiac disease. A variety of congenital cardiac malformations were identified during echocardiographic examination and autopsy, including a double outlet right ventricle and a subpulmonary interventricular septal defect (Taussig-Bing anomaly), ventricular inversion with atrioventricular discordance, tricuspid valve atresia, a septum primum interatrial septal defect, mitral valve dysplasia with a cleft in the septal mitral valve cusp, aortic, and subaortic stenosis, tubular hypoplasia of the ascending aorta and the aortic arch, a patent ductus arteriosus, an aberrant circumflex coronary artery, and aberrant left and right subclavian arteries. Echocardiographic and postmortem findings of the cardiac defects in this foal are presented and discussed. Copyright © 2017 Elsevier B.V. All rights reserved.

  11. Robotic-assisted endoscopic atrial septal defect closure: analysis of 115 cases in a single center.

    Science.gov (United States)

    Yang, Ming; Gao, Changqing; Xiao, Cangsong

    2012-06-01

    To summarize the experience with the application of robotic technique in totally endoscopic atrial septal defect closure in a single center. Between January 2007 and September 2011, 115 patients with the diagnosis of secundum type atrial septal defects underwent robotic atrial septal defect repair with the assistance of da Vinci surgical system. The patients had a median age of 35 years and a median defect diameter of 28 mm. Cardiopulmonary bypass was established via peripheral cannulation. Via three 8-mm ports and one 15-mm port in the right chest, the surgeon manipulated the microinstruments to complete the defect closure with or without tricuspid valve plasty. Echocardiography was performed intraoperatively, before discharge and at 30 days after the operation. Atrial septal defect closure was completed on arrested heart in 44 patients and on beating heart in 61 patients. No deaths or conversions to alternate techniques occurred in these cases. No residual shunt was detected by intraoperative or postoperative echocardiography. The mean operating time and cardiopulmonary bypass time on bearing heart group were significantly shorter than those on arrested heart group. The median ventilation time, intensive care unit stay, drainage volume, or length of hospital stay showed no significant differences between the two groups. Secundum type atrial septal defect closure can be successfully performed with the assistance of the robotic system with good surgical results.

  12. Sinus Venosus Atrial Septal Defect Complicated by Eisenmenger Syndrome and the Role of Vasodilator Therapy

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    Amornpol Anuwatworn

    2016-01-01

    Full Text Available Sinus venosus atrial septal defect is a rare congenital, interatrial communication defect at the junction of the right atrium and the vena cava. It accounts for 5–10% of cases of all atrial septal defects. Due to the rare prevalence and anatomical complexity, diagnosing sinus venous atrial septal defects poses clinical challenges which may delay diagnosis and treatment. Advanced cardiac imaging studies are useful tools to diagnose this clinical entity and to delineate the anatomy and any associated communications. Surgical correction of the anomaly is the primary treatment. We discuss a 43-year-old Hispanic female patient who presented with dyspnea and hypoxia following a laparoscopic myomectomy. She had been diagnosed with peripartum cardiomyopathy nine years ago at another hospital. Transesophageal echocardiography and computed tomographic angiography of the chest confirmed a diagnosis of sinus venosus atrial septal defect. She was also found to have pulmonary arterial hypertension and Eisenmenger syndrome. During a hemodynamic study, she responded to vasodilator and she was treated with Ambrisentan and Tadalafil. After six months, her symptoms improved and her pulmonary arterial hypertension decreased. We also observed progressive reversal of the right-to-left shunt. This case illustrates the potential benefit of vasodilator therapy in reversing Eisenmenger physiology, which may lead to surgical repair of the atrial septal defect as the primary treatment.

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

    Science.gov (United States)

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

    2014-06-01

    We are aimed to investigate whether right ventricular mid-septal pacing (RVMSP) is superior to conventional right ventricular apical pacing (RVAP) in improving clinical functional capacity and left ventricular ejection fraction (LVEF) for patients with high-degree atrio-ventricular block and moderately depressed left ventricle (LV) function. Ninety-two patients with high-degree atrio-ventricular block and moderately reduced LVEF (ranging from 35% to 50%) were randomly allocated to RVMSP (n=45) and RVAP (n=47). New York Heart Association (NYHA) functional class, echocardiographic LVEF, and distance during a 6-min walk test (6MWT) were determined at 18 months after pacemaker implantation. Serum levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) were measured using an enzyme-linked immunosorbent assay (ELISA) kit. Compared with baseline, NYHA functional class remained unchanged at 18 months, distance during 6MWT (485 m vs. 517 m) and LVEF (36.7% vs. 41.8%) were increased, but BNP levels were reduced (2352 pg/ml vs. 710 pg/ml) in the RVMSP group compared with those in the RVAP group, especially in patients with LVEF 35%-40% (for all comparisons, Pblock and moderately depressed LV function whose LVEF levels ranged from 35% to 40%.

  14. [A case of subpulmonary membranous stenosis associated with atrial septal defect].

    Science.gov (United States)

    Ono, Y; Momokawa, T; Shuto, K; Munakata, M; Suzuki, S; Koie, H

    1993-09-01

    The patient was a 5 years old male who had had the cyanosis and congestive heart failure from his neonatal period. Dopamine, digitalis and diuretics disappeared his symptoms and he had been followed up as the out-patient. Preoperative cardiac catheterization revealed atrial septal defect and moderately pulmonary stenosis with two-staged systolic pressure gradient in a right ventricular cavity. Right ventriculogram showed subpulmonary crescent-shaped, linear filling defect. Ventricular septal defect was not detected. He was underwent open heart surgery and subpulmonary membranous stenosis was found out. Pressure gradient across the right ventricular outflow tract was diminished by the resection of the membranous structure. Atrial septal defect without lower margin was closed directly. Postoperative course was uneventful. Right ventricular apical systolic pressure was decreased to the degree of 27 mmHg postoperatively.

  15. Nickel hypersensitivity following closure of atrial septal defect: A case report and review of the literature.

    Science.gov (United States)

    Dickison, Philippa; Harris, Victoria; Smith, Saxon D

    2018-01-29

    We present an unusual case where symptoms of headache and chest pain persisted for 3 years following the implantation of a septal occluder device for an atrial septal defect despite endothelialisation of the device. The patient was found to have nickel hypersensitivity on patch testing. Following the removal of the device the patient had complete resolution of headaches and chest pain up to 10 months post-explantation. © 2018 The Australasian College of Dermatologists.

  16. Intramural Ventricular Septal Defect Is a Distinct Clinical Entity Associated With Postoperative Morbidity in Children After Repair of Conotruncal Anomalies

    National Research Council Canada - National Science Library

    Patel, Jyoti K; Glatz, Andrew C; Ghosh, Reena M; Jones, Shannon M; Natarajan, Shobha; Ravishankar, Chitra; Mascio, Christopher E; Spray, Thomas L; Cohen, Meryl S

    2015-01-01

    BACKGROUND—Intramural ventricular septal defects (VSDs) are interventricular communications through right ventricular free wall trabeculations that can occur after repair of conotruncal anomalies...

  17. Evaluation of morphological characteristics of septal rims affecting successful transcatheter atrial septal defect closure in children and adults.

    Science.gov (United States)

    Oflaz, Mehmet Burhan; Pac, Feyza Aysenur; Kibar, Ayse Esin; Balli, Sevket; Ece, Ibrahim

    2013-01-01

    Determining other echocardiographic predictors along with the measured atrial septal defect (ASD) size and evaluating the closure together with these predictors would increase the chance of success for transcatheter closure of ASD. To evaluate echocardiographic parameters affecting defect closure in children and adult patients with secundum ASD. In all patients, size of ASD, total length of atrial septum (TS), superior-posterior, inferior-posterior, superior-anterior and inferior-anterior rims surrounding the defect were measured by transesophageal echocardiography (TEE), and several measurement ratios were derived on the basis of TEE parameters. A total 216 patients with secundum ASD were included in this study. The device was successfully implanted in 65 children and 65 adults. Both in pediatric and adult cases, the ratio of successful closure was found to be significantly higher when the ratio of defect size to TS was ≤ 0.35, the ratio of superior-anterior (SA) rim to the defect size was > 0.75 and the ratio of inferior-posterior (IP) rim to the defect size was > 1.0. It was found that having more than one of these predictors in a single case increased the chance of closure success significantly (p rim to defect size > 0.75 and a ratio of IP rim to defect size > 1.0 were found to be echocardiographic predictors that could be used in successful transcatheter ASD closure both in children and adults.

  18. Successful closure of atrial septal defect by retrograde transarterial approach after unsuccessful transfemoral venous approach

    Directory of Open Access Journals (Sweden)

    Sadık Volkan Emren

    2016-06-01

    Full Text Available Although many of the ostium secundum atrial septal defects can be successfully closed by percutaneous transvenous approach, surgical method as well is preferred in some cases due to vascular reasons or due to the anatomical characteristics of the defect. Detecting ostium secundum atrial septal defect in a 26-year-old female patient, who presented with shortness of breath, percutaneous closure procedure was planned. During percutaneous closure, it was observed that guidewire and catheter persistently went towards patent foramen ovale because of quite flaccid structure of interatrial septum. For this reason, occluder could not be placed in the defect area. Before using the option of surgery for the patient, guidewire and simmons 1 diagnostic catheter were pushed forward towards to the left ventricle and then left atrium by transarterial approach through the femoral artery. Guidewire and the catheter were easily passed through atrial septal defect without passing through patent foramen ovale benefiting from the left atrial pressure. Occluder was retrogradely carried via transarterial route and successfully implanted in the defect area. Transarterial approach may be an alternative before surgery in selected cases in which percutaneous closure of atrial septal defect has failed.

  19. Risk Factors for Delayed Extubation after Ventricular Septal Defect Closure: a Prospective Observational Study

    Directory of Open Access Journals (Sweden)

    Divyakant Parmar

    Full Text Available Objective: The objective of our study was to determine the feasibility of early extubation and to identify the risk factors for delayed extubation in pediatric patients operated for ventricular septal defect closure. Methods: A prospective, observational study was carried out at our Institute. This study involved consecutive 135 patients undergoing ventricular septal defect closure. Patients were extubated if feasible within six hours after surgery. Based on duration of extubation, patients were divided two groups: Group 1= extubation time ≤ 6 hours, Group 2= extubation time >6 hours. Results: A total of 99 patients were in Group 1 and 36 patients in Group 2. Duration of ventilation was 4.4±0.9 hours in Group 1 and 25.9±24.9 hours in Group 2 (P<0.001. Univariate analysis showed that young age, low weight, low partial pressure of oxygen, trisomy 21, multiple ventricular septal defect, high vasoactive inotropic score, transient heart block and low cardiac output syndrome were associated with delayed extubation. However, regression analysis revealed that only trisomy 21 (OR: 0.248; 95%CI: 0.176-0.701; P=0.001, low cardiac output syndrome (OR: 0.291; 95%CI: 0.267-0.979; P=0.001, multiple ventricular septal defect (OR: 0.243; 95%CI: 0.147-0.606; P=0.002 and vasoactive inotropic score (OR: 0.174 95%CI: 0.002-0.062; P=0.039 are strongest predictors for delayed extubation. Conclusion: Trisomy 21, low cardiac output syndrome, multiple ventricular septal defect and high vasoactive inotropic score are significant risk factors for delay in extubation. Age, weight, pulmonary artery hypertension, size of ventricular septal defect, aortic cross-clamp and cardiopulmonary bypass time did not affect early extubation.

  20. Assessment of muscular ventricular septal defect closure by transcatheter or surgical approach: a three-dimensional echocardiographic study.

    Science.gov (United States)

    Acar, P; Abdel-Massih, T; Douste-Blazy, M-Y; Dulac, Y; Bonhoeffer, P; Sidi, D

    2002-09-01

    Previous classification of muscular ventricular septal defects (VSDs) visualized on two-dimensional echocardiography relied on artificial divisions of the septum. New visualization of the ventricular septum integrating the third dimension would facilitate communication between cardiologists and surgeons. The objectives of this study were (1) to assess in patients with muscular ventricular septal defects the accuracy of left ventricular three-dimensional echocardiographic reconstructions in demonstrating the position, the size and the tissue rims of the defects; (2) to compare findings by three-dimensional echocardiography with those obtained by surgical and transcatheter approaches. Twenty-six patients, aged from one month to 40 years, with muscular ventricular septal defects underwent three-dimensional echocardiographic study. From the left ventricular three-dimensional echocardiographic reconstructions, the localization, the maximal diameter and the tissue rim of the defect were analysed and compared with surgical or transcatheter findings. Optimal three-dimensional echocardiographic reconstructions were obtained in 22 patients. Nineteen had a single muscular ventricular septal defect and three had multiple muscular ventricular septal defects. The muscular ventricular septal defect localizations were the inlet septum in three, the outlet septum in three, the mid-muscular septum in 14 and the apex in eighth. In 10 patients who underwent surgical closure, the correlation between three-dimensional echocardiography and surgery for muscular ventricular septal defect maximal diameter was y=0 x 95 x +0.13 (r=0.98; Pventricular septal defect localization were complete. In five patients who underwent transcatheter closure, the mean difference between three-dimensional echocardiographic maximal diameter and stretched diameter was 1 x 8+/-0 x 5 mm. The three-dimensional echocardiographic left ventricular views provide a new and easily communicated visualization of various

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

    Directory of Open Access Journals (Sweden)

    Montresor Graziano

    2003-11-01

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

  2. Concordant familial segregation of atrial septal defect and Axenfeld-Rieger anomaly in father and son.

    Science.gov (United States)

    Calcagni, Giulio; Digilio, Maria Cristina; Capolino, Rossella; Dallapiccola, Bruno; Marino, Bruno

    2006-10-01

    The association of congenital heart defect and ocular malformations is involved in several genetic syndromes, metabolic diseases and environmental entities. We report here on father and son, both presenting with the combination of atrial septal defect and congenital ocular anomalies in Axenfeld-Rieger anomaly. The son had anterior iridotrabecular dysgenesis and posterior embryotoxon bilaterally, corneal leucoma and marked iridial vascularization at right. The father had bilateral anterior iridotrabecular dysgenesis, posterior embryotoxon and nystagmus, and corneal leucoma at left. No additional malformations were noted in these patients. The Axenfeld-Rieger syndrome seems to be a spectrum of developmental disorders. The present report confirms the existence of a specific Axenfeld-Rieger phenotype associated with congenital heart defect. Atrial septal defect is the anatomic type of congenital heart defect linked to this condition.

  3. Multicenter midterm follow-up results using the gore septal occluder for atrial septal defect closure in pediatric patients.

    Science.gov (United States)

    Grohmann, Jochen; Wildberg, Christian; Zartner, Peter; Abu-Tair, Tariq; Tarusinov, Gleb; Kitzmüller, Erwin; Schmoor, Claudia; Stiller, Brigitte; Kampmann, Christoph

    2017-06-01

    To assess the safety and efficacy of the Gore Septal Occluder (GSO) used for device-closure of significant secundum-type atrial septal defects (ASD II) focusing on pediatric patients. The GSO is a patch-like double disc device. Due to its design, it is assumed to be safe, even when implanted in ASDs with deficient retro-aortic rims. Multicenter retrospective analysis of consecutive children and adolescents with a GSO in situ for at least 12 months according to a 1- to 4-year midterm follow-up. Hundred and seventy three pediatric patients were enrolled. At implantation, median age was 6 years (range 0.7-17.9), median body weight and length were 21 kg (6.4-95) and 119 cm (65-193). Median follow-up period was 20 months (range 12-51). ASD anatomy was comprised of single defects in 131 patients (76%), multi-fenestrated defects in 42 (24%), and deficient retro-aortic rims in 33 (19%). Follow-up confirmed an overall closure-rate of 95.4%. Small residual shunts were reported in eight patients (4.6%) without need for any re-intervention. Complications were classified as minor events both during the initial procedure (9 patients, 5.2%) and on follow-up (another 9 patients), including transient AV block II in three patients (1.8%) and four snare-retrievals (2.4%) during the initial procedure. Periprocedural and midterm follow-up data have shown the GSO to be effective and safe for ASD device closure in children and adolescents. GSO may be considered the first-choice device in deficient retro-aortic rims and multi-fenestrated defects, when covering most of the atrial septum is necessary. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  4. RESIDUAL DEFECTS AFTER SURGICAL REPAIR OF VENTRICULAR SEPTAL DEFECTS IN CHILDREN:

    Directory of Open Access Journals (Sweden)

    K Sayadpour-Zanjani

    2008-12-01

    Full Text Available "nResidual ventricular septal defects (VSD are major complications after cardiac surgery. We studied the incidence of this complication, risk factors for its occurrence and short-term follow-up in 179 pediatric patients that underwent surgical closure of VSD from April 2003 until May 2004. All data were gathered retrospectively except measurements of shunt ratio. Studied risk factors included age, sex, weight, height, ejection fraction, VSD size, presence of pulmonary stenosis (PS, responsible surgeon, use of patch material for closing VSD, mean degree of hypothermia, cardiopulmonary bypass and aortic cross-clamp times, hemorrhage, documented infection, and surgical approach for defect closure. The incidence of all residual VSDs was 56% and significant ones (i.e. with Qp/Qs > 1.5 22%. The only statistically significant risk factors were higher age, weigh and height of the patients. There was notable but statistically insignificant differences in residual shunt incidence among the patients of different surgeons and with the use of different patch materials. During the median follow-up period of 9.5 months, 35% of the residual defects were closed spontaneously. Six patients underwent catheterization, three of which were candidates of residual VSD closure. As residual VSD is a hemodynamically and psychologically important complication, we recommend VSD closure at lower age and the use of intraoperative epicardial or transesophageal echocardiography to minimize its occurrence.

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

    Directory of Open Access Journals (Sweden)

    M. Cristina Digilio

    2011-07-01

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

  6. Short- and Mid-term Results of Atrial Septal Defect and Patent Foramen Ovale Occlusion with Starway Septal Occluder Device

    Directory of Open Access Journals (Sweden)

    J Kojuri

    2011-09-01

    Full Text Available Background: With a prevalence of almost 7% of all congenital heart diseases, atrial septal defect (ASD is a common condition. Patent foramen ovale (PFO is also a congenital heart disease which is frequently sustained into adulthood. Objectives: To study the feasibility of closure of ASD and PFU by Starway septal occluder device and the incidence of its inherent complications and procedural failure in 62 patients referred to our center. Methods: Starway septal occluder device was used for closure of ASD and PFO in 62 patients. After left and right heart catheterization, transesophageal echocardiography-guided closure was done for the patients with immediate recording of the results. Patients were followed for 6 months by transesophageal echocardiography for observing short- and mid-term complications. Results: The 62 studied patients were categorized into 2 groups. Group 1 included 31 patients (64% females with ASD (mean±SD age: 26.7±7.6 years. Group 2 consisted of 31 patients (35.6% females with PFO (mean±SD age: 53.5±12.4 years. Size of the right ventricle (RV annulus was significantly (P=0.005 decreased after the intervention in the ASD group. Overall 5 (8% patients developed post-intervention complications (transient ischemic attack, leg edema, and residual shunt and procedural failure—4 (13% in ASD group and 1 (3% in PFO group. None of the patients developed device-related thrombosis, significant arrhythmia, aortic regurgitation and pericardial effusion after intervention. Conclusion: Starway occluder device is effective and safe with very low short- and mid-term complication rates.

  7. Surgical Outcomes of Congenital Atrial Septal Defect Using da VinciTM Surgical Robot System.

    Science.gov (United States)

    Kim, Ji Eon; Jung, Sung-Ho; Kim, Gwan Sic; Kim, Joon Bum; Choo, Suk Jung; Chung, Cheol Hyun; Lee, Jae Won

    2013-04-01

    Minimally invasive cardiac surgery has emerged as an alternative to conventional open surgery. This report reviews our experience with atrial septal defect using the da VinciTM surgical robot system. This retrospective study included 50 consecutive patients who underwent atrial septal defect repair using the da VinciTM surgical robot system between October 2007 and May 2011. Among these, 13 patients (26%) were approached through a totally endoscopic approach and the others by mini-thoracotomy. Nineteen patients had concomitant procedures including tricuspid annuloplasty (n=10), mitral valvuloplasty (n=9), and maze procedure (n=4). The mean follow-up duration was 16.9±10.4 months. No remnant interatrial shunt was detected by intraoperative or postoperative echocardiography. The atrial septal defects were mainly repaired by Gore-Tex patch closure (80%). There was no operative mortality or serious surgical complications. The aortic cross clamping time and cardiopulmonary bypass time were 74.1±32.2 and 157.6±49.7 minutes, respectively. The postoperative hospital stay was 5.5±3.3 days. The atrial septal defect repair with concomitant procedures like mitral valve repair or tricuspid valve repair using the da VinciTM system is a feasible method. In addition, in selected patients, complete port access can be helpful for better cosmetic results and less musculoskeletal injury.

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

    DEFF Research Database (Denmark)

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

    2012-01-01

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

  9. Extracorporeal membrane oxygenation in a child with traumatic ventricular septal defect

    Directory of Open Access Journals (Sweden)

    Jennifer Y. Lam

    2018-01-01

    Full Text Available Traumatic ventricular septal defect is an uncommon event following blunt thoracic trauma. Within the pediatric trauma literature, extracorporeal membrane oxygenation is most commonly used for secondary acute respiratory distress syndrome. We present the first account of rescue extracorporeal membrane oxygenation to allow for safe transport and access to definitive operative repair in the setting of blunt cardiac injury.

  10. Exercise capacity and participation of children with a ventricular septal defect.

    NARCIS (Netherlands)

    Binkhorst, M.; Belt, T van de; Hoog, M. de; Dijk, Arie van; Schokking, M.; Hopman, M.

    2008-01-01

    Existing data on exercise performance in children with a ventricular septal defect (VSD) are scarce and inconclusive. We aimed to elucidate whether and why exercise capacity and physical activity level are decreased in children with VSD. Children 9 to 17 years of age with a surgically (operated, n =

  11. Repair of Posterior Infarct Ventricular Septal Defect in a Patient with Dextrocardia and Situs Inversus.

    Science.gov (United States)

    Nesta, Marialisa; Mazza, Andrea; Perri, Gianluigi; Bruno, Piergiorgio; Massetti, Massimo

    2016-03-01

    We report a patient with situs inversus who developed a large posterior interventricular septum pseudoaneurysm with a septal defect following a myocardial infarction. The ventricular septum was approached through the left ventricle and the entrance of the pseudoaneurysm was repaired with a strip of equine pericardium. © 2016 Wiley Periodicals, Inc.

  12. Deficient Surrounding Rims in Patients Undergoing Transcatheter Atrial Septal Defect Closure.

    Science.gov (United States)

    Kijima, Yasufumi; Akagi, Teiji; Takaya, Yoichi; Taniguchi, Manabu; Nakagawa, Koji; Kusano, Kengo; Sano, Shunji; Ito, Hiroshi

    2016-08-01

    The influence of deficient rims surrounding atrial septal defects (ASDs) in patients undergoing transcatheter closure has yet to be clarified. The aim of this study was to assess the influence of a deficient surrounding rim on the procedural success and clinical outcome of transcatheter ASD closure using an Amplatzer septal occluder. A total of 474 patients (mean age, 46 ± 22 years) with ostium secundum ASDs measuring ≤40 mm in diameter who had undergone attempted transcatheter closure using Amplatzer septal occluders from September 2007 to August 2013 were assessed. A comprehensive transesophageal echocardiographic examination was done to assess the morphologic characteristics of the defects in all patients. Subjects were classified into three groups by the extent and location of rim deficiency (rims (sufficient group, n = 101), patients with single deficient rims, (single group, n = 338), and patients with multiple rim deficiencies (multiple group, n = 35). There was a significant difference in the maximal defect diameter among the sufficient, single, and multiple groups (15 ± 6, 18 ± 6, and 29 ± 7 mm, respectively, P rim deficiencies as determined by transesophageal echocardiography, successful transcatheter ASD closure using Amplatzer septal occluders is more difficult to accomplish. However, if closure is successful, rim deficiencies rarely affect intermediate-term outcomes. Copyright © 2016 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

  13. [Aneurysm of the membranous ventricular septum with ventricular septal defect, mitral and tricuspid insufficiency].

    Science.gov (United States)

    Tanaka, K; Yasunaga, H; Egashira, A; Kumate, M; Kawara, T; Kosuga, K

    1998-10-01

    A seventy-year-old man was admitted at our hospital because of dyspnea. Echocardiogram and left ventriculogram showed an aneurysm formation of the membranous ventricular septum and small left-to-right shunt through ventricular septum defect and also severe mitral and tricuspid insufficiency. Operation was performed after medical therapy for congestive heart failure. During operation, mitral leaflets showed no organic lesions nor prolapse, but the annulus was dilated. The cause of mitral insufficiency, we thought, might be congenital, and the annulus dilatation was caused of mitral insufficiency, we thought, might be congenital, and the annulus dilatation was caused to produce tricuspid insufficiency secondary. The ventricular septal communication became small (diameter; 5 mm) and was associated with aneurysm formation of the remaining portion of the membranous septum. And the aneurysm, protruding to the septal leaflet of tricuspid valves, enhanced tricuspid insufficiency. It was reported by many authors that the aneurysm formation was related to spontaneous closure of ventricular septal defect. Patients with small ventricular septal defect, without any symptoms, must be followed intensively, or they might get cardiac complications, such as arrhythmia, right ventricular outflow obstruction, tricuspid insufficiency, and so on.

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

    African Journals Online (AJOL)

    dimensional echocardiographic examination revealed a mid‑muscular VSD. The connection between the defect and the trauma was not initially appreciated. Facilities for required urgent open‑heart surgery were not available. Cardiac failure ...

  15. Is Three-Dimensional Echocardiography Useful in Evaluation of Atrial Septal Defects?

    Directory of Open Access Journals (Sweden)

    Charles German

    2015-06-01

    Full Text Available Of all birth defects, Congenital Heart Disease (CHD remains the most prevalent. These malformations are largely multifactorial with both environmental and genetic components, but known chromosomal abnormalities and mutations of single genes account for less than 10% of all cardiac defects (1. They affect approximately 6 to 13 newborns per 1000 live births (2 and are made up of 5 major Atrial Septal Defects (ASDs, including primum and secundum type defects, sinus venosus and coronary sinus defects, and Patent Foramen Ovale (PFO. However, there is debate within the medical community regarding inclusion of PFOs and coronary sinus defects within the realm of CHD. PFOs do not have absent septal tissue, and coronary sinus defects, or unroofed coronary sinus, represent an abnormal communication between the superior portion of the coronary sinus and the neighboring left atrium. Regardless, these anomalies can go undetected at birth, particularly if asymptomatic. Though some defects may be innocuous at birth, the continued shunting of blood between the atria can lead to pulmonary hypertension, heart failure, and even death. Thus, finding an accurate and reliable means of diagnosis via echocardiography is necessary in establishing the optimal treatment and ultimately improving patient mortality.

  16. Mid-term Follow-up of the Transcatheter Closure of Perimembranous Ventricular Septal Defects in Children Using the Amplatzer

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    Mehdi Ghaderian

    2016-03-01

    Full Text Available Background: The ventricular septal defect (VSD is the most common form of congenital heart defects. The purpose of this study was to evaluate the results of the early complications and mid-term follow-up of the transcatheter closure of the VSD using the Amplatzer VSD Occluder.Methods: Between April 2012 and October 2013, 110 patients underwent the percutaneous closure of the perimembranous VSD. During the procedure, the size and type of the VSD were obtained via ventriculography. A device at least 2 mm larger than the VSD diameter measured via ventriculography was deployed. The size of the VSD, size of the Amplatzer, and device-size to VSD-size ratio were calculated. After the confirmation of the suitable position of the device via echocardiography and left ventriculography, the device was released. Follow-up evaluations were done at discharge as well as at 1, 6, and 12 months and yearly thereafter for the VSD occlusion and complete heart block.Results: The study population comprised 62 females and 48 males. The mean age and weight of the patients at procedure were 4.3 ± 5.6 years (range: 2 to 14 and 14.9 ± 10.8 kg (range: 10 to 43. The average device size was 7.0 ± 2.5 mm (range: 4 to 14. The VSD occlusion rate was 72.8% at the completion of the procedure and rose up to 99.0% during the follow-up. The most serious significant complication was complete atrioventricular block, which was seen in 2 patients. The mean follow-up duration was 10.9 ± 3.6 months.Conclusion: The transcatheter closure of the perimembranous VSD was a safe and effective treatment with excellent closure rates in our study population. This procedure had neither mortality nor serious complications. 

  17. Percutaneous closure of perimembranous ventricular septal defect with an Amplatzer Duct Occluder in a dextrocardia patient.

    Science.gov (United States)

    Ergene, Oktay; Nazli, Cem; Kocabas, Ugur; Duygu, Hamza; Akyildiz, Zehra Ilke; Hijazi, Ziyad M

    2011-07-15

    In this article we report an unusual case of dextrocardia patient with perimembranous ventricular septal defect (VSD) whose defect is closed by percutaneous method with Amplatzer Duct Occluder-II device. To our best knowledge, this was the first time this device has been used to close a membranous defect in a patient with dextrocardia. Our case demonstrates the feasibility of percutaneous VSD closure in challenging patients by using appropiate techniques and devices for particular patients. Copyright © 2009 Elsevier Ireland Ltd. All rights reserved.

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

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    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%.

  19. Successful extensive enlargement of a non-committed ventricular septal defect in double outlet right ventricle.

    Science.gov (United States)

    Ishibashi, Nobuyuki; Aoki, Mitsuru; Fujiwara, Tadashi

    2005-08-01

    We performed an arterial switch operation in a patient with double outlet right ventricle with non-committed ventricular septal defect, and abnormal insertion of the tension apparatus of the tricuspid valve which produced moderate tricuspid regurgitation. This required extensive enlargement of the ventricular septal defect between the attachments of the cords of the tricuspid valve so as to create the interventricular rerouting that made possible the arterial switch operation. Postoperatively, we produced a straight, unobstructed, left ventricular outflow tract, improved the extent of tricuspid regurgitation, and achieved low right atrial pressures. Enlargement of the interventricular communication can set the scene for biventricular repair in this particular subset of patients with both arterial trunks arising from the morphologically right ventricle.

  20. Pregnancy in peritoneal dialysis and an infant with a ventricular septal defect

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    Abdullah Alhwiesh

    2015-01-01

    Full Text Available Fertility is markedly reduced in dialysis patients. Estimates of the frequency of conception in dialysis patients range from 1.4% per year in Saudi Arabia to 0.5% in the United States. The reasons for the rarity of pregnancy in dialysis patients are not well understood. In addition, there is a marked increase in the risk of pre-eclampsia, hydramnios, hypertension crisis, early uterine contractions and pre-term delivery. Herein, we report a 38-year-old Saudi woman with chronic renal failure who completed the full term of pregnancy uneventfully on peritoneal dialysis. Using a biocompatible dialysate solution, adequate metabolic and blood pressure control were achieved during pregnancy. The delivered infant was small for gestational age and was born with a ventricular-septal defect. To the best of our knowledge, this is the first case report in the literature of ventricular-septal defect in an infant born to a mother on peritoneal dialysis.

  1. The role of cardiac MRI in the diagnosis and management of sinus venosus atrial septal defect

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    Madhusudan Ganigara

    2014-01-01

    Full Text Available Sinus venosus atrial septal defects (SV-ASDs are inter-atrial communications caused by a deficiency of the common wall between the superior or inferior vena cava and the right-sided pulmonary veins. The diagnosis can be challenging, especially in adults with delayed presentation. We present images that illustrate an example of the role of cardiac magnetic resonance imaging (CMRI in the diagnosis and follow-up of a patient with SV-ASD.

  2. Post-Myocardial Infarction Ventricular Septal Defect Six Months following Coronary Artery Bypass Grafting.

    Science.gov (United States)

    Fiedler, Amy G; Sundt Iii, Thoralf M; Tolis, George

    2017-08-25

    Mechanical complications following acute myocardial infarction are associated with high mortality. We present the first reported case of a new post myocardial infarction ventricular septal defect (VSD) within six months of coronary artery bypass grafting. The patient underwent successful surgical correction of the VSD with the assistance of mechanical circulatory support (MCS). This case highlights the importance of mechanical circulatory support in the management of cardiogenic shock associated with rare complications of myocardial infarction, even after surgical revascularization.

  3. Ventricular septal defect and double-chambered right ventricle in an alpaca.

    Science.gov (United States)

    Poser, Helen; Dalla Pria, Angela; De Benedictis, Giulia M; Stelletta, Calogero; Berlanda, Michele; Guglielmini, Carlo

    2015-03-01

    A 20-month-old male alpaca was referred for evaluation of a cardiac murmur evident since birth. Echocardiography identified a ventricular septal defect (VSD) and a fibro-muscular band causing a stenosis of the right ventricular outflow tract. Right ventricular catheterization and selective angiography confirmed the diagnosis of VSD and double-chambered right ventricle with bidirectional shunting. Copyright © 2014 Elsevier B.V. All rights reserved.

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

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    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.

  5. Device closure in adults with atrial septal defect in Shiraz, a single center registry

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    Ostovan, Mohammad Ali; Kojuri, Javad; Dehghani, Pooyan; Razazi, Vida; Moarref, Alireza

    2016-01-01

    Introduction: Successful closure of atrial septal defect (ASD) improves patients? functional class and exercise capacity. In this study we evaluate the safety and feasibility of percutaneous device closure of ASDs. Methods: Two hundred fifty six patients with significant ASD according to our criteria were enrolled. The patients were treated using nitinol wire mesh transcatheter devices. Complications were followed for a median of 2.5 years. Results: Success rate was 98.4% with 3 unsuccessful ...

  6. Familial ebstein anomaly, left ventricular hypertrabeculation, and ventricular septal defect associated with a MYH7 mutation.

    Science.gov (United States)

    Bettinelli, Audra L; Mulder, Theodorus J; Funke, Birgit H; Lafferty, Katherine A; Longo, Sherri A; Niyazov, Dmitriy M

    2013-12-01

    Ebstein anomaly is a rare congenital heart defect that most often occurs sporadically within a kindred. Familial cases, although reported, are uncommon. At this time, the genetic etiology of Ebstein anomaly is not fully elucidated. Here, we describe clinical and molecular investigations of a rare case of familial Ebstein anomaly in association with a likely pathogenic mutation of the MYH7 gene. The severity of presentation varies, and Ebstein anomaly can be observed in association with such other heart defects as ventricular septal defect and left ventricular (LV) hypertrabeculation, as seen in our family of study. In our family of study, the 31-year-old father and four of his children have been diagnosed with Ebstein anomaly. Genetic testing revealed that the father was heterozygous for the Glu1220del variant detected in exon 27 of the MYH7 gene. The MYH7 gene encodes the β-myosin heavy chain and is expressed in cardiac muscle. DNA sequencing of three of his affected children confirmed that they carried the same variant while the fourth affected child was not available for testing. This is the first report of familial Ebstein anomaly associated with the Glu1220del mutation of the MYH7 gene. The mutation segregates with disease in a family with autosomal dominant transmission of congenital heart defects including Ebstein anomaly and other associated cardiovascular defects including LV hypertrabeculation and ventricular septal defect. © 2013 Wiley Periodicals, Inc.

  7. Robotically assisted perventricular closure of perimembranous ventricular septal defects: preliminary results in Yucatan pigs.

    Science.gov (United States)

    Amin, Zahid; Woo, Russell; Danford, David A; Froemming, Stacey E; Reddy, Vadiyala M; Lof, John; Overman, David

    2006-02-01

    Robotic systems allow surgeons to perform minimally invasive cardiac surgery in adults. Experience in the pediatric population, however, is limited. Perventricular closure of muscular ventricular septal defects has been reported in humans but requires a median sternotomy. The objective of this study was to assess the feasibility of robotically assisted closure of perimembranous ventricular septal defects by using the perventricular approach. The procedure was attempted in 7 pigs with naturally occurring perimembranous ventricular septal defects. Echocardiography was performed to confirm the presence and assess the size of the defect. A 3-armed da Vinci system consisting of two 8-mm instrument ports and a 12-mm endoscopy port was used. A pericardiotomy was performed, and the right ventricular free wall was visualized. A spinal needle was advanced into the right ventricular cavity. By using echocardiographic guidance, a glide wire was advanced through the angiocatheter and manipulated through the defect into the left ventricle or the ascending aorta. A delivery sheath was advanced over the wire. An appropriately sized Amplatzer device was deployed through the sheath. The procedure was successful in 5 pigs. One device was removed because it was smaller than the defect and an appropriately sized device was not available. The placement failed in the second pig in the series. Four pigs were followed up for 1 to 4 months. Angiograms performed before the pigs were killed documented complete occlusion in 3 and mild-to-moderate shunt in 1. Robotically assisted perventricular closure with the Amplatzer Membranous VSD Occluder is feasible. This approach avoids the associated morbidities of cardiopulmonary bypass and median sternotomy. Further investigation and refinements are needed, however, before application of this approach in humans.

  8. Comparison of transcatheter closure of secundum atrial septal defect using the Amplatzer septal occluder associated with deficient versus sufficient rims.

    Science.gov (United States)

    Du, Zhong-Dong; Koenig, Peter; Cao, Q-Ling; Waight, David; Heitschmidt, Mary; Hijazi, Ziyad M

    2002-10-15

    To evaluate the feasibility of transcatheter closure of secundum atrial septal defects (ASDs) associated with deficient rims (rim of 0 to 4 mm (n = 20), an inferior rim of 2 mm (n = 2), or a posterior rim of 4 mm (n = 1) as assessed by transesophageal echocardiography (TEE) or intracardiac echocardiography (ICE). Forty-eight patients with sufficient rims (>5 mm) who underwent closure served as controls. There were no differences between the 2 groups in ASD stretched diameter and device size (p >0.05). Of 23 patients with deficient rims, 17 (74%) had immediate complete closure compared with 44 of 48 patients (92%) with sufficient rims (p rims vs 94% for patients with sufficient rims at 24 hours and 100% vs 93% at 6 months, respectively). The fluoroscopic time and procedure time were longer in patients with deficient rims (13 +/- 7 and 72 +/- 26 minutes, respectively) compared with those with sufficient rims (10 +/- 4 and 61 +/- 22 minutes, respectively). No major complications were encountered either during or after the closure procedure in both groups. Thus, transcatheter closure of ASDs associated with small anterior, inferior, or posterior rims is feasible using an ASO. Long-term follow-up data are still needed to assess long-term safety and efficacy.

  9. Digital rectal examination in the evaluation of rectovaginal septal defects.

    Science.gov (United States)

    Rachaneni, Suneetha; Atan, Ixora Kamisan; Shek, Ka Lai; Dietz, Hans Peter

    2017-02-17

    The objective was to evaluate the diagnostic potential of digital rectal examination in the identification of a true rectocele. This is a retrospective observational study utilising 187 archived data sets of women presenting with lower urinary tract symptoms and/or pelvic organ prolapse between August 2012 and November 2013. Evaluation included a standardised interview, ICS-POPQ, rectal examination and 4D translabial ultrasound. The main outcome measure was the diagnosis of rectocele by digital rectal palpation on Valsalva manoeuvre. This diagnosis correlated with the sonographic diagnosis of rectocele to determine agreement between digital examination and ultrasound findings. Complete data sets were available for 180 participants. On imaging, the mean position of the rectal ampulla was 11.07 (-36.3 to 44.3) mm below the symphysis pubis; 42.8% (77) had a rectocele of a depth of ≥10 mm. On palpation, a rectocele was detected in 60 women (33%). Agreement between palpation and imaging was observed in 77%; the kappa was 0.52 (CI 0.39-0.65). On receiver operator characteristic analysis, the area under the curve was 0.854 for the relationship between rectocele pocket depth and the detection of rectocele on palpation. Moderate agreement was found between digital rectal examination for rectocele and translabial ultrasound findings of a "true rectocele". Digital rectal examination may be used to identify these defects in clinical practice. Extending the clinical examination of prolapse to include rectal examination to palpate defects in the rectovaginal septum may reduce the need for defecatory proctograms for the assessment of obstructive defecation and may help triage patients in the management of posterior compartment prolapse.

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

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    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.

  11. A New Coated Nitinol Occluder for Transcatheter Closure of Ventricular Septal Defects in a Canine Model

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    Yong Zhou

    2013-01-01

    Full Text Available Aims. This study evaluated feasibility and safety of implanting the polyester-coated nitinol ventricular septal defect occluder (pcVSDO in the canine model. Methods and Results. VSD models were successfully established by transseptal ventricular septal puncture via the right jugular vein in 15 out of 18 canines. Two types of VSDOs were implanted, either with pcVSDOs (n=8 as the new type occluder group or with the commercial ventricular septal defect occluders (VSDOs, n=7, Shanghai Sharp Memory Alloy Co. Ltd. as the control group. Sheath size was 10 French (10 Fr in two groups. Then the general state of the canines was observed after implantation. ECG and TTE were performed, respectively, at 7, 30, 90 days of follow-up. The canines were sacrificed at these time points for pathological and scanning electron microscopy examination. The devices were successfully implanted in all 15 canines and were retrievable and repositionable. There was no thrombus formation on the device or occurrence of complete heart block. The pcVSDO surface implanted at day 7 was already covered with neotissue by gross examination, and it completed endothelialization at day 30, while the commercial VSDO was covered with the neotissue in 30th day and the complete endothelialization in 90th day. Conclusion. The study shows that pcVSDO is feasible and safe to close canine VSD model and has good biocompatibility and shorter time of endothelialization.

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

    Science.gov (United States)

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

    2015-08-01

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

  13. Two pedigrees of autosomal dominant atrioventricular canal defect (AVCD): Exclusion from the critical region on 8p

    Energy Technology Data Exchange (ETDEWEB)

    Amati, F.; Mari, A.; Mingarelli, R. [Universita Tor Vergata, Rome (Italy)] [and others

    1995-07-03

    Atrioventricular canal defects (AVCD) constitute the predominant congenital heart defect in Down`s syndrome. For this reason, a candidate gene involved in atrioventricular canal development was previously searched and excluded in dominant pedigrees of AVCD, using linkage analysis of polymorphisms from chromosome 21. Because of the striking association between 8p deletion and AVCD, a search for an AVCD gene was carried out in two pedigrees of individuals with autosomal dominant AVCD using a set of DNA markers of the 8pter{r_arrow}q12 region. These two families include affected individuals and subjects who have transmitted the defect but are not clinically affected. Two-point lod scores were significantly negative for all markers at penetrance levels of 90% and 50%. Multipoint analysis excluded the region covered by the markers LPL-D8S262 and 30 cM to either side of this area. This result corroborates heterogeneity of this heart defect and indicates that the genetic basis of familial AVCD is different from AVCD associated to either trisomy 21 or 8p deletion. 25 refs., 3 figs., 2 tabs.

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

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    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.

  15. Iatrogenic intra-atrial macro-reenterant tachycardia following transcatheter closure of atrial septal defect treated by radiofrequency ablation

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    Ibrahim Marai

    2011-01-01

    Full Text Available Percutaneous closure of an atrial septal defect (ASD has been established as a safe and effective alternative to surgical management. We describe a case of a 41-year-old patient in whom an Amplatzer septal occluder device was used to close a moderately large ASD and who subsequently developed incessant intra-atrial macro-reenterant tachycardia. The tachycardia was terminated by radiofrequency ablation guided by electroanatomical mapping.

  16. Pulmonary atresia with dextroposition of the aorta and ventricular septal defect in three Arabian foals.

    Science.gov (United States)

    Vitums, A; Bayly, W M

    1982-03-01

    Three Arabian males foals were presented with cyanosis, heart murmur, and exercise intolerance, Results of clinical evaluation suggested a tentative diagnosis of ventricular septal defect in conjunction with malformations of the great arteries. Each foal had a poor prognosis and was killed at the owners' requests. At necropsy, the malformed hearts of the three foals were virtually identical. Each heart had a large defect in the upper interventricular septum. The aorta originated from the hypertrophied right ventricle and partially overrode the ventricular septal defect. The aortic ostium was guarded by three semilunar valvules. The position of the valvules and the ostia of the coronary arteries was abnormal. The pulmonary arteries arose from the persistent ductus arteriosus. The pulmonary trunk was reduced to a thin, fibrous cord extending from a short and narrow persistent ductus arteriosus to the level of the semilunar valve, where it fused with the wall of the aorta. No communication of the atretic pulmonary trunk with the cavity of the right ventricle was seen.

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

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    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.

  18. Challenges in treatment of postinfarction ventricular septal defect and heart failure

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    Mangovski Ljupčo

    2015-01-01

    Full Text Available Introduction. Acquired ventricular septal defect (VSD is uncommon, but serious mechanical complication of acute myocardial infarction with poor outcome and high mortality rate in surgically or medically treated patients. Case report. We report a 58-year-old male patient admitted to our hospital six days following acute inferior myocardial infarction complicated by ventricular septal rupture with signs of heart failure. Coronary angiography revealed 3-vessel disease, with proximally occluded dominant right coronary artery. Transthoracic echo exam revealed aneurysm of a very thin inferior septum and the basal portion of the inferior left ventricular wall, with septal wall rupture. One of the VSD dimensions was 15 mm and left- to right shunt was calculated 2 : 1. Since the patient was at too high risk for surgical closure, transcatheter closure of VSD was chosen as a better option. Under short intravenous sedation, 24 mm Amplatzer device was implanted percutaneously with transesophageal echo guidance. The post-procedural result revealed a small residual shunt, but it was followed by significant improvement of the patient’s clinical status. A 24h Holter ECG monitoring did not show cardiac rhythm or conduction disturbances. Coronary angiography was repeated ten days following the procedure, after hemodynamic stabilization of the patient, with direct stenting of the circumflex artery and the intermediate artery. Ostial left descending artery lesion was left for further functional significance assessment. Conclusion: Percutaneous closure with a septal occluder device can be definitive primary treatment for anatomically suitable patients or it can serve as a bridge to surgical treatment.

  19. Single Centre Experience for Percutaneous Closure of Secundum Atrial Septal Defect

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    Ramazan Aydemir

    2011-12-01

    Full Text Available Introduction: Atrial septal defect (ASD is the most common congenital heart disease in adults and constitutes 5-10% of all congenital heart disease. Primary surgical closure has been the standard approach for many years with high success rate. Transcatheter closure of ASD in selected patients has became the contemprorary practice that has the advantages of short hospital stay and relative ease of prosedure. In this article, we reported the results of percutaneous closure of ASD in our center.
Methods: Between 2009-2011, thirty-two patients (mean age 36±18 years and 24 females who had secundum type ASD which detected by transthoracic echocardiography (TTE and transesophageal echocardiography (TEE were included in this study. Twenty-nine (90,6% patients were closed percutaneously. Tree patients were referred for surgery because of failure of percutaneous closure. Amplatzer Septal Occluder was used in 25 (86,2% patients and Occlutech device was used in other patients. All procedures were performed under local anesthesia. All of the patients were examined with TTE before the procedure and, at the 1st, 3rd, 6th and 12th month follow-up visits.
Results: Pre-operative mean pulmonary artery pressure was 33,3±7,5 mmHg and mean pulmonary to systemic flow (Qp/Qs ratio was 2,9±1,1. Mean ASD diameter measured by TEE was 20,1±6,6 mm and mean streched diameter measured by balloon catheter was 22,1±5,9 mm. Mean device size 24±6 mm (range 12-36 mm. In the follow-up period no major complication was observed, but mild to moderate residual shunt flow was detected in two patients (% 6,8.
Conclusion: Percutaneous ASD closure has been performed with high success and low complication rates in patients with secundum atrial septal defect in our center.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2017-02-15

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

  1. Surgical Outcomes of a Modified Infarct Exclusion Technique for Post-Infarction Ventricular Septal Defects

    Directory of Open Access Journals (Sweden)

    In Sook Kim

    2015-12-01

    Full Text Available Background: Postinfarction ventricular septal defects (pVSDs are a serious complication of acute myocardial infarctions. The aim of this study was to analyze the clinical outcomes of the surgical treatment of pVSDs. Methods: The medical records of 23 patients who underwent operations (infarct exclusion in 21 patients and patch closure in two patients to treat acute pVSDs from 2001 to 2011 were analyzed. Intra-aortic balloon counterpulsation was performed in 19 patients (82.6%, one of whom required extracorporeal membrane support due to cardiogenic shock. The mean follow-up duration was 26.2±18.6 months. Results: The in-hospital mortality rate was 4.3% (1/23. Residual shunts were found in seven patients and three patients required reoperation. One patient needed reoperation due to the transformation of an intracardiac hematoma into an abscess. No patients required reoperation due to recurrence of a ventricular septal defect during the follow-up period. The cumulative survival rate was 95.5% at one year, 82.0% at five years, and 65.6% at seven years. Conclusion: The use of a multiple- patch technique with sealants appears to be a reliable method of reducing early mortality and the risk of significant residual shunting in patients with pVSDs.

  2. Transesophageal Echocardiographic Characteristics of Secundum-Type Atrial-Septal Defect in Adult Patients

    Directory of Open Access Journals (Sweden)

    Arezou Zoroufian

    2009-12-01

    Full Text Available Background: Given the dearth of data in the existing literature on the size and morphologic variability of secundumtype atrial-septal defect (ASD-II in adult patients, we aimed to address this issue in a series of consecutive adult patients evaluated by transesophageal echocardiography (TEE.Methods: A total of 50 patients (68.0% female with isolated ASD-II underwent TEE for the evaluation of the defect. The morphological characteristics of the defect were evaluated, and the largest defect size was measured. The ASD rim wasdivided into 6 sectors: the superior-anterior, superior-posterior, superior, inferior-anterior, inferior-posterior, and inferior.The minimal length of the defect rims was determined.Results: Mean age at the time of evaluation was 33.62±14.48 years. Mean defect diameter in the all the study patients was 20.80±8.17 mm. Thirteen morphological variations were detected. Deficiency of one rim was detected in 14 (28% patients,two in 16 (32%, three in 2 (4%, and four in 2 (4%. Deficiency of the superior anterior rim was found in 24% of the patients as the most frequent morphology. There was a significant correlation between the defect size and number of deficient rims (γ=0.558, P value<0.001. Forty-eight (96% patients emerged for defect closure: 22 (46.2% suitable for percutaneousclosure and 26 (53.8% for surgical closure. Two patients with small defects were recommended for medical treatment and follow-up.Conclusion: ASD-II is larger and more morphologically variable in adults than in children. Based on the findings of the present and previous studies and given the advantages of percutaneous treatment, it is advisable to make a decision on ASD-II closure as soon as possible before it outgrows the transcatheter closure suitability criteria.

  3. Relationships of the tricuspid valve to the membranous ventricular septum in Down's syndrome without endocardial cushion defect: study of 28 specimens, 14 with a ventricular septal defect.

    Science.gov (United States)

    Rosenquist, G C; Sweeney, L J; McAllister, H A

    1975-10-01

    The commissure between the anterior and medial leaflets of the tricuspid valve is commonly absent in Down's syndrome without endocardial cushion defect (19 of 28 specimens). As a result, aneurysm of the membranous ventricular septum may develop (eight of 14 specimens with ventricular septal defect limited to the membranous ventricular septum) and the potential for left ventricle-to-right atrial communication is increased.

  4. Current concept of transcatheter closure of atrial septal defect in adults.

    Science.gov (United States)

    Akagi, Teiji

    2015-01-01

    After the introduction of catheter intervention for atrial septal defect (ASD) in the pediatric population, therapeutic advantages of this less invasive procedure were focused on adult through geriatric populations. The most valuable clinical benefits of this procedure are the significant improvement of symptoms and daily activities, which result from the closure of left to right shunt without thoracotomy and cardiopulmonary bypass surgery. These benefits contribute to increase the number of adult patients of this condition who have hesitated over surgical closure. In terms of technical point of view for catheter closure of ASD, the difficulties still exist in some morphological features of defect, or hemodynamic features in the adult population. Morphological features of difficult ASD closure are (1) large (≥30 mm) ASD, (2) wide rim deficiency, and (3) multiple defects. Hemodynamic features of difficult ASD are (1) severe pulmonary hypertension, (2) ventricular dysfunction, and (3) restrictive left ventricular compliance (diastolic dysfunction) after ASD closure. To complete the catheter ASD closure under these difficult conditions, various procedural techniques have been introduced. These are new imaging modalities such as real-time three-dimensional imaging, new technical modifications, and new concepts for hemodynamic evaluation. Especially, real-time three-dimensional transesophageal echocardiography can provide the high quality imaging for anatomical evaluation including maximum defect size, surrounding rim morphology, and the relationship between device and septal rim. In adult patients, optimal management for their comorbidities is an important issue, which includes cardiac function, atrial arrhythmias, respiratory function, and renal function. Management of atrial arrhythmias is a key issue for the long-term outcome in adult patients. Because the interventional procedures are not complication-free techniques, the establishment of a surgical back

  5. Off-pump atrial septal defect closure using the universal cardiac introducer®: creation of models of atrial septal defects in the pig access and surgical technique.

    Science.gov (United States)

    Guiraudon, Gerard M; Jones, Douglas L; Bainbridge, Daniel; Moore, John T; Wedlake, Chris; Linte, Cristian; Wiles, Andrew; Peters, Terry M

    2009-01-01

    : Optimal atrial septal defect (ASD) closure should combine off-pump techniques with the effectiveness and versatility of open-heart techniques. We report our experience with off-pump ASD closure using the Universal Cardiac Introducer (UCI) in a porcine model. The goal was to create an ASD over the fossa ovale (FO) and position a patch over the ASD under ultrasound (US) imaging and augmented virtual reality guidance. : An US probe (tracked with a magnetic tracking system) was positioned into the esophagus (transesophageal echocardiographic probe) for real-time image-guidance. The right atrium (RA) of six pigs was exposed via a right lateral thoracotomy or medial sternotomy. The UCI was attached to the RA wall. A punching tool was introduced via the UCI, navigated and positioned, under US guidance, to create an ASD into the FO. A patch with its holder and a stapling device were introduced into the RA via the UCI. The patch was positioned on the ASD. Occlusion of the ASD was determined using US and Doppler imaging. : The FO membrane was excised successfully in all animals. US image-guidance provided excellent visualization. The patch was positioned in all cases with complete occlusion of the ASD. The stapling device proved too bulky, impeding circumferential positioning. : Using the UCI, ASD closure was safe and feasible. US imaging, combined with virtual and augmented reality provided accurate navigating and positioning. This study also provided valuable information on the future design of anchoring devices for intracardiac procedures.

  6. Pulmonary blood supply by a branch from the distal ascending aorta in pulmonary atresia with ventricular septal defect: differential diagnosis of fifth aortic arch.

    Science.gov (United States)

    Yoo, S J; Moes, C A; Burrows, P E; Molossi, S; Freedom, R M

    1993-10-01

    A patient with pulmonary atresia and a ventricular septal defect is described in whom an arterial branch from the distal ascending aorta supplied segments of both lungs. The branch is considered to represent a persistent fifth aortic arch. The possible morphogenesis and differential diagnosis of a communication between the ascending aorta and the pulmonary artery in pulmonary atresia with ventricular septal defect are discussed.

  7. A rare type of Gerbode defect.

    Science.gov (United States)

    Panduranga, Prashanth; Mukhaini, Mohammed

    2011-07-01

    A Gerbode defect is a left ventricle to right atrial communication. The type I defect (direct, acquired) results in a direct shunt through the atrioventricular part of membranous septum, while a type II (indirect, congenital) defect results in an indirect shunt through a perimembranous ventricular septal defect (VSD) and a defect in the septal tricuspid valve leaflet. We report a rare type of Gerbode defect wherein a small perimembranous VSD is completely covered by an elongated sail-like anterior tricuspid leaflet forming an aneurysm and directing the shunt into right atrium. © 2011, Wiley Periodicals, Inc.

  8. Simulation of Eisenmenger syndrome with ventricular septal defect using equivalent electronic system.

    Science.gov (United States)

    Korurek, Mehmet; Yildiz, Mustafa; Yüksel, Ayhan; Şahin, Alparslan

    2012-06-01

    In this study, we aim to investigate the simulation of the cardiovascular system using an electronic circuit model under normal and pathological conditions, especially the Eisenmenger syndrome. The Eisenmenger syndrome includes a congenital communication between the systemic and pulmonary circulation, with resultant pulmonary arterial hypertension and right-to-left reversal of flow through the defect. When pulmonary vascular resistance exceeds systemic vascular resistance, it results in hypoxaemia and cyanosis. The Westkessel model including Resistor-Inductance-Capacitance pi-segments was chosen in order to simulate both systemic and pulmonary circulation. The left and right heart are represented by trapezoidal shape stiffness for better simulation results. The Eisenmenger syndrome is simulated using a resistance (septal resistance) connected between the left ventricle and right ventricle points of the model. Matlab® is used for the model implementation. In this model, although there is a remarkable increase in the pulmonary artery pressure and right ventricle pressure, left ventricle pressure, aortic pressure, aortic flow, and pulmonary compliance decrease in the Eisenmenger syndrome. In addition, left-to-right septal flow reversed in these diseases. Our model is effective and available for simulating normal cardiac conditions and cardiovascular diseases, especially the Eisenmenger syndrome.

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

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    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.

  10. [The role of MRI for the evaluation of atrial septal defects before and after percutaneous occlusion with the amplatzer septal occluder(R)].

    Science.gov (United States)

    Weber, C; Dill, T; Mommert, I; Hofmann, T; Adam, G

    2002-11-01

    Evaluation of morphologic and functional MRI of atrial septal defects (ASD) before and after percutaneous occlusion with the Amplatzer Septal Occluder (AOC). Comparison of MRI with transesophageal echocardiography (TEE), balloon measurement (IVBM) and cardiac catheterization with shunt quantification (CCSQ). Twenty patients with ASD were examined before and three months after AOC implantation. ECG-triggered, breath-hold T1-weighted Turbo Spin Echo Segmented FLASH 2D and dynamic turbo-FLASH-GRE sequences after application of 0.2 mmol gadolinium DTPA per kg body weight were obtained in a 1.5 T MRI system. Defect size, and distance to coronal sinus (CS) and right upper pulmonary vein (RUPV) were determined for pre-interventional planning, and the AOC size was measured quantitatively for post-interventional follow-up. The shunts were evaluated qualitatively (occurrence of jets), semiquantitatively (jet length, turbulence square product) and quantitatively (flow measurement in the thoracic aorta and in the left and right pulmonary arteries). The average size of the ASD measured by MRI was 17.6 mm (11 - 24.8 mm) in the axial view, 15.9 mm (10.8 - 28.9 mm) in the sagittal view and 16.4 mm (12.1 - 24.8 mm) in the short axis view. In comparison, the average defect size was 15 mm (8 - 24 mm) by TEE and 20 mm (13 - 27 mm) by IVBM. The average distance to the RUPV was 17 mm (9.6 - 21.9 mm) and to the CS 11.2 mm (5 - 17 mm). The AOC was visualized with only minimal artifacts. Qualitative analysis of the MRI findings revealed an occurrence of jets in 17/20 patients. Semiquantitative analysis documented a high correlation for jet length and square product of the turbulence to defect size r = 0.81 resp. r = 0.82. Mean QP/QS-ratio measured by MR-volumetry was 1.6 +/- 0.29 and by MR-flow 1.6 +/- 0.26. The corresponding measurements were 1.7 +/- 0.3 for TEE and 1.5 +/- 0.5 for CCSQ. In comparison to TEE, the correlation coefficient was r = 0.96 for MR-volumetry and r = 0.85 for MR

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

    NARCIS (Netherlands)

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

    2007-01-01

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

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

    Science.gov (United States)

    Kohli, Vikas

    2015-02-01

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

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

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    Mangovski Ljupčo

    2015-01-01

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

  14. Onychomycosis due to Candida parapsilosis in a Child with Ventricular Septal Defect: An Unusual Predisposition

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    Supram Hosuru Subramanya

    2016-01-01

    Full Text Available Candida parapsilosis is emerging as a potential pathogen for onychomycosis. A 4-year-old male child with perimembranous ventricular septal defect (VSD was admitted with features of cystitis and was treated with broad spectrum antibiotics. Two weeks later, he developed yellowish discoloration of nails of both hands. The sloughed out nail, on microscopy, showed numerous yeast forms that were identified as Candida parapsilosis by both phenotypic and genotypic methods. Antifungal sensitivity testing of the isolate was performed by microbroth dilution method in accordance with CLSI guidelines. Patient was successfully treated with topical amphotericin B and oral fluconazole. Thus, one should have a high index of suspicion of C. parapsilosis onychomycosis, especially when the patient is in the paediatric age group, presenting with unusual predisposing condition like congenital heart disease, and is on broad spectrum antibiotics.

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

    Science.gov (United States)

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

    1998-04-01

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

  16. Ventricular septal defect and bidirectional shunting? Things are not what they seem.

    Science.gov (United States)

    Bonaque González, Juan Carlos; Navarro, Francisco; Valencia, Félix; Aguado, María J

    2013-01-01

    This report describes the case of a 19-year-old woman with a diagnosis of muscular ventricular septal defect. Bidirectional shunting was observed during a transthorathic echocardiography evaluation which also suggested normal pulmonary arterial pressure. Moreover, anomalous and hypertrophic right ventricular muscular bands were observed. After having ruled out other possibilities, the plausible explanation is one, which is not described in the literature. The findings may be explained as a sequestrated portion of the cavity of the right ventricle that remains isolated from the rest of the right ventricle (RV) by anomalous and hypertrophic right ventricular muscular bands, with communication only between the left ventricle and the sequestrated part of the RV. This is an unusual variant of two-chambered RV simulating two-chambered left ventricle.

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

    DEFF Research Database (Denmark)

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

    2011-01-01

    BACKGROUND: This cohort study examines the possible association between maternal alcohol intake, including binge drinking, during pregnancy, and the subsequent risk of having a child with an isolated congenital heart defect and, more specifically, with the isolated form of ventricular septal defect...... of alcohol. Few (if any) women with an excessive/abusive intake of alcohol were enrolled into the Danish National Birth Cohort. RESULTS: Through linkage with the National Hospital Discharge Registry, we identified 477 infants with a diagnosis of isolated congenital heart defect registered at any time during...... their first 3½-years of life; they included 198 infants with a VSD and 145 with an ASD. Neither the number of episodes of binge drinking nor binge drinking during three different developmental periods was associated with VSD or ASD. Women drinking ½-1½, 2, and 3+ drinks of alcohol per week had adjusted...

  18. Rare variants in NR2F2 cause congenital heart defects in humans

    NARCIS (Netherlands)

    Al Turki, Saeed; Manickaraj, Ashok K.; Mercer, Catherine L.; Gerety, Sebastian S.; Hitz, Marc-Phillip; Lindsay, Sarah; D'Alessandro, Lisa C. A.; Swaminathan, G. Jawahar; Bentham, Jamie; Arndt, Anne-Karin; Low, Jacoba; Breckpot, Jeroen; Gewillig, Marc; Thienpont, Bernard; Abdul-Khaliq, Hashim; Harnack, Christine; Hoff, Kirstin; Kramer, Hans-Heiner; Schubert, Stephan; Siebert, Reiner; Toka, Okan; Cosgrove, Catherine; Watkins, Hugh; Lucassen, Anneke M.; O'Kelly, Ita M.; Salmon, Anthony P.; Bu'lock, Frances A.; Granados-Riveron, Javier; Setchfield, Kerry; Thornborough, Chris; Brook, J. David; Mulder, Barbara; Klaassen, Sabine; Bhattacharya, Shoumo; Devriendt, Koen; Fitzpatrick, David F.; Wilson, David I.; Mital, Seema; Hurles, Matthew E.

    2014-01-01

    Congenital heart defects (CHDs) are the most common birth defect worldwide and are a leading cause of neonatal mortality. Nonsyndromic atrioventricular septal defects (AVSDs) are an important subtype of CHDs for which the genetic architecture is poorly understood. We performed exome sequencing in 13

  19. Atypical atrial septal defects in children: noninvasive evaluation by cardiac MRI

    Energy Technology Data Exchange (ETDEWEB)

    Beerbaum, Philipp; Parish, Victoria; Bell, Aaron [Guy' s and St. Thomas' Hospital, Division of Imaging Sciences, King' s College London, London (United Kingdom); Gieseke, Juergen [Philips Medical Systems, Best (Netherlands); Koerperich, Hermann; Sarikouch, Samir [Ruhr-University of Bochum, Department of Congenital Heart Disease and Institute for Magnetic Resonance Imaging, Heart and Diabetes Centre North Rhine-Westfalia, Bad Oeynhausen (Germany)

    2008-11-15

    Atypical left-to-right shunts at the level of the atrium in children such as sinus venosus atrial septal defects (ASDs) and partial anomalous pulmonary venous return (PAPVR) may be difficult to assess by transthoracic or transoesophageal echocardiography. Free-breathing cardiac MRI may be a powerful alternative. To assess the value of free-breathing cardiac MRI in the delineation of atypical ASDs in children. A total of 82 children (mean age 5.9 years, range 1.1-15.7 years) with suspected ASD and inconclusive transthoracic echocardiography underwent cardiac MRI under free-breathing, mostly sedated conditions. Phase-contrast MRI was used for defect visualization and shunt quantification, and multiphase inflow MR angiography for delineation of pulmonary/systemic venous connections. Of the 82 patients, 34 (41%) were diagnosed with atypical shunt lesions at the level of the atrium and 48 (59%) with simple secundum ASDs. No false-negative or false-positive findings were reported by MRI compared to cardiac catheterization and intraoperative findings. Superior sinus venosus ASD with partial anomalous PAPVR was present in 10 of the 82 children (12.2%), whereas 2 (2.4%) had a large posterior-inferior defect, 5 (6.1%) had isolated PAPVR, and 17 (20.7%) had multiple ASDs and/or associated vascular anomalies. Q{sub p}/Q{sub s} by phase-contrast MRI agreed well with oximetry values (mean difference 3%, limits of agreement {+-}21-25%; Bland/Altman analysis). Free-breathing cardiac MRI under sedation allows reliable identification of atypical left-to-right shunt defects at the level of the atrium in children in whom transcatheter ASD closure is unsuitable, including delineation of pulmonary or systemic venous anomalies and shunt quantification. (orig.)

  20. Totally robotic atrial septal defect closure: 7-year single-institution experience and follow-up.

    Science.gov (United States)

    Xiao, Cangsong; Gao, Changqing; Yang, Ming; Wang, Gang; Wu, Yang; Wang, Jiali; Wang, Rong; Yao, Minghui

    2014-12-01

    Robotic technology has been applied to atrial septal defect (ASD) repair for more than 10 years, but the number of cases reported is limited and results of long-term follow-up are not clear. This study reports on a large group of patients who underwent totally robotic ASD repair on an arrested or beating heart at a single institution with a 7-year follow-up. From 2007 to 2013, 160 patients (median age, 36 years; range, 11-66 years) at our centre underwent selective repair of secundum-type ASD using the da Vinci robotic system. The first 54 cases were performed on an arrested heart (arrested-heart group, n = 54) and the remainder on a beating heart (beating-heart group, n = 106). The mean diameter of defects was 2.9 cm (range, 1.1-4.1 cm). Cardiopulmonary bypass was achieved via cannulation of the femoral vessels and the right internal jugular vein. Blood cardioplegic arrest was induced using a transthoracic Chitwood clamp in the arrested-heart group. With the assistance of a robotic surgical system, atrial septal defect repairs were performed with or without tricuspid valvuloplasty via three 8-mm ports, a camera port and a working port in the right chest. Transoesophageal echocardiography was used to evaluate surgical results and follow-up. Complete ASD closure was verified by intraoperative transoesophageal echocardiography in all patients. None of the procedures was converted to an alternate technique and there were no major complications. There were significant learning curves for cross-clamp time, operative duration and cardiopulmonary bypass time. The beating-heart group had significantly shorter operative and cardiopulmonary bypass durations than the arrested-heart group (P = 0.000). The two groups had similar durations of mechanical ventilation and intensive care unit and hospital stays, and similar drainage volumes. During the 39 ± 21 months of follow-up, no patient required reoperation because of a residual shunt or tricuspid valve regurgitation. ASD can

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

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    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.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2015-06-01

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

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

    Science.gov (United States)

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

    2014-10-01

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

  4. Novel and Functional DNA Sequence Variants within the GATA6 Gene Promoter in Ventricular Septal Defects

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    Chunyu Li

    2014-07-01

    Full Text Available Congenital heart disease (CHD is the most common birth defect in humans. Genetic causes and underlying molecular mechanisms for isolated CHD remain largely unknown. Studies have demonstrated that GATA transcription factor 6 (GATA6 plays an essential role in the heart development. Mutations in GATA6 gene have been associated with diverse types of CHD. As GATA6 functions in a dosage-dependent manner, we speculated that changed GATA6 levels, resulting from DNA sequence variants (DSVs within the gene regulatory regions, may mediate the CHD development. In the present study, GATA6 gene promoter was genetically and functionally analyzed in large groups of patients with ventricular septal defect (VSD (n = 359 and ethnic-matched healthy controls (n = 365. In total, 11 DSVs, including four SNPs, were identified in VSD patients and controls. Two novel and heterozygous DSVs, g.22169190A>T and g.22169311C>G, were identified in two VSD patients, but in none of controls. In cultured cardiomyocytes, the activities of the GATA6 gene promoter were significantly reduced by the DSVs g.22169190A>T and g.22169311C>G. Therefore, our findings suggested that the DSVs within the GATA6 gene promoter identified in VSD patients may change GATA6 levels, contributing to the VSD development as a risk factor.

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

    Directory of Open Access Journals (Sweden)

    Yun Mi Kim

    2011-02-01

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

  6. A Rare Case of Double-Chambered Right Ventricle Associated with Ventricular Septal Defect and congenital Absence of the Pulmonary Valve

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    Georges Khoueiry

    2014-01-01

    Full Text Available Double-chambered right ventricle (DCRV is a rare congenital heart disorder involving 2 different right ventricle (RV pressure compartments that is often associated with ventricular septal defect (VSD. Usually, the obstruction is caused by an anomalous muscle bundle crossing the RV from the interventricular septum to the RV free wall. We are reporting a case of double-chambered right ventricle associated with ventricular septal defect and congenital absence of the pulmonary valve, a rare form of congenital infundibular pulmonary stenosis. In addition to ventricular septal defect, our patient had congenital absence of the pulmonary valve, which is very unusual and has never been reported to our knowledge.

  7. 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.

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    Strandberg-Larsen, Katrine; Skov-Ettrup, Lise Skrubbeltrang; Grønbaek, Morten; Andersen, Anne-Marie Nybo; Olsen, Jørn; Tolstrup, Janne

    2011-07-01

    This cohort study examines the possible association between maternal alcohol intake, including binge drinking, during pregnancy, and the subsequent risk of having a child with an isolated congenital heart defect and, more specifically, with the isolated form of ventricular septal defect (VSD) or of an atrial septal defect (ASD). 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 of alcohol. Few (if any) women with an excessive/abusive intake of alcohol were enrolled into the Danish National Birth Cohort. Through linkage with the National Hospital Discharge Registry, we identified 477 infants with a diagnosis of isolated congenital heart defect registered at any time during their first 3½-years of life; they included 198 infants with a VSD and 145 with an ASD. Neither the number of episodes of binge drinking nor binge drinking during three different developmental periods was associated with VSD or ASD. Women drinking ½-1½, 2, and 3+ drinks of alcohol per week had adjusted prevalence ratios of delivering an infant with a VSD of 1.22 (95% CI = 0.90-1.66); 1.38 (95% CI = 0.83-2.28); and 1.10 (95% CI = 0.54-2.23), respectively. The test for trend was 0.29. Prenatal exposure to low-to-moderate levels of alcohol on a weekly basis or occasional binge drinking during the early part of pregnancy was not statistical significantly associated with the prevalence of isolated VSD and ASD in offspring. Copyright © 2011 Wiley-Liss, Inc.

  8. Compound heterozygous or homozygous truncating MYBPC3 mutations cause lethal cardiomyopathy with features of noncompaction and septal defects

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    Wessels, Marja W; Herkert, Johanna C; Frohn-Mulder, Ingrid M; Dalinghaus, Michiel; van den Wijngaard, Arthur; de Krijger, Ronald R; Michels, Michelle; de Coo, Irenaeus FM; Hoedemaekers, Yvonne M; Dooijes, Dennis

    2015-01-01

    Familial hypertrophic cardiomyopathy (HCM) is usually caused by autosomal dominant pathogenic mutations in genes encoding sarcomeric or sarcomere-associated cardiac muscle proteins. The disease mainly affects adults, although young children with severe HCM have also been reported. We describe four unrelated neonates with lethal cardiomyopathy, and performed molecular studies to identify the genetic defect. We also present a literature overview of reported patients with compound heterozygous or homozygous pathogenic MYBPC3 mutations and describe their clinical characteristics. All four children presented with feeding difficulties, failure to thrive, and dyspnea. They died from cardiac failure before age 13 weeks. Features of left ventricular noncompaction were diagnosed in three patients. In the fourth, hypertrabeculation was not a clear feature, but could not be excluded. All of them had septal defects. Two patients were compound heterozygotes for the pathogenic c.2373dup p.(Trp792fs) and c.2827C>T p.(Arg943*) mutations, and two were homozygous for the c.2373dup and c.2827C>T mutations. All patients with biallelic truncating pathogenic mutations in MYBPC3 reported so far (n=21) were diagnosed with severe cardiomyopathy and/or died within the first few months of life. In 62% (13/21), septal defects or a patent ductus arteriosus accompanied cardiomyopathy. In contrast to heterozygous pathogenic mutations, homozygous or compound heterozygous truncating pathogenic MYBPC3 mutations cause severe neonatal cardiomyopathy with features of left ventricular noncompaction and septal defects in approximately 60% of patients. PMID:25335496

  9. Robotically assisted totally endoscopic atrial septal defect repair: insights from operative times, learning curves, and clinical outcome.

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    Bonaros, Nikolaos; Schachner, Thomas; Oehlinger, Armin; Ruetzler, Elisabeth; Kolbitsch, Christian; Dichtl, Wolfgang; Mueller, Silvana; Laufer, Guenther; Bonatti, Johannes

    2006-08-01

    Remote access perfusion and robotics have enabled totally endoscopic closure of atrial septal defect and patent foramen ovale. The aim of this study was to address learning curve issues of totally endoscopic atrial septal defect repair on the basis of a single-center experience and to investigate whether long cardiopulmonary bypass and aortic occlusion times influence intraoperative and postoperative outcomes. Seventeen patients (median age, 35 years; range, 16 to 55 years) underwent totally endoscopic atrial septal defect repair using remote access perfusion and robotic technology (da Vinci telemanipulation system). Learning curves were assessed by means of regression analysis with logarithmic curve fit. The effect of operative variables on clinical outcome was analyzed by linear regression using the Spearman's rho coefficient. No operative mortality or serious surgical complications were observed. No residual shunt was detected at intraoperative or postoperative echocardiography. Significant learning curves were noted for total operative time: y(min) = 406 - 49 ln(x) (r2 = 0.725; p = 0.002); cardiopulmonary bypass time: y(min) = 225 - 42 ln(x) (r2 = 0.699; p = 0.003); and aortic occlusion time: y(min) = 117 - 25 ln(x) (r2 = 0.517; p = 0.04), x = number of procedures. Median ventilation time, intensive care unit stay, and hospital length of stay were 7 hours (range, 2 to 19 hours), 26 hours (range, 15 to 120 hours), and 8 days (range, 5 to 14 days), respectively. No correlation was detected between cardiopulmonary bypass time and intubation time (r2 = 0.283; p = 0.326), intensive care unit stay (r2 = -0.138; p = 0.639), or total length of stay (r2 = 0.013; p = 0.962). Totally endoscopic atrial septal defect repair can be performed safely, and learning curves for operative times are steep. Longer cardiopulmonary bypass times had no negative impact on intraoperative and postoperative outcome.

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

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    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.

  11. Coronary to pulmonary fistula as the primary source of pulmonary blood supply in pulmonary atresia with ventricular septal defect

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    Isman Firdaus; Cholid T. Tjahjono; Ganesja H. Harimurti; Poppy S. Roebiono

    2004-01-01

    A communication between the coronary and pulmonary arteries, so called coronary to pulmonary fistula, is a rare source of pulmonary supply in pulmonary atresia (PA) with ventricular septal defect (VSD). A 4 year old girl referred to National Cardiovascular Center Harapan Kita, Jakarta with symptoms and signs of increased pulmonary blood flow since infancy and was confirmed by the chest x-rays. Heart examination revealed normal first heart sound with single loud second heart sound and an eject...

  12. Radiofrequency-assisted "reconstruction" of the right ventricular outflow tract in muscular pulmonary atresia with ventricular septal defect.

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    Hausdorf, G; Schulze-Neick, I.; Lange, P. E.

    1993-01-01

    A case of pulmonary atresia with ventricular septal defect is reported in which a communication was established between the right ventricle and the hypoplastic pulmonary artery by intervention, despite muscular atresia of the right ventricular outflow tract. The atresia was perforated with a special designed radiofrequency catheter (Osypka). After the creation of a canal within the muscular atresia, balloon dilatation (diameters 2, 3.5, and 7.2 mm) was performed. Arterial oxygen saturation in...

  13. Altered Pulmonary Venous Flow Pattern in Young Adults with Atrial Septal Defect

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    Mozhgan Parsaee

    2016-08-01

    Full Text Available Introduction: Atrial septal defect (ASD is a common congenital heart disease and causes left-to-right shunting and significant right ventricular (RV volume overload. The goal of this study was to evaluate the influence of these hemodynamic changes on pulmonary venous flow pattern in young adults.Methods: Complete echocardiographic examination was performed in a group of 40 young adults (aged < 40 years who had secundum type ASD and was compared to 40 age-matched individuals in control group who had no cardiac abnormality. Systolic and diastolic flow velocities in pulmonary veins (PV, superior vena cava (SVC, inferior vena cava (IVC and RV functional parameters were recorded and evaluated.Results: As opposed to healthy young individuals who showed distinct S and D waves with diastolic predominance in pulmonary vein Doppler, in patients with ASD a continuous flow with increased systolic peak that began in systole and continued to the late diastole was observed. The RV systolic function increased compared to the control group.Conclusions: In patients with ASD, the pattern of pulmonary veins flow transforms into a single continuous antegrade wave with systolic dominance due to persistent shunting of left atrial blood in to right heart chambers as well as increased RV pump function on pulmonary vein (by means of ASD, SVC and IVC, and could be used as a screening method for the presence of secundum type ASDs in young adults.

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

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    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, ploss were significantly correlated with BNP (r=0.75, 0.69 and 0.49, ploss 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. Towards high-throughput mouse embryonic phenotyping: a novel approach to classifying ventricular septal defects

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    Liang, Xi; Xie, Zhongliu; Tamura, Masaru; Shiroishi, Toshihiko; Kitamoto, Asanobu

    2015-03-01

    The goal of the International Mouse Phenotyping Consortium (IMPC, www.mousephenotype.org) is to study all the over 23,000 genes in the mouse by knocking them out one-by-one for comparative analysis. Large amounts of knockout mouse lines have been raised, leading to a strong demand for high-throughput phenotyping technologies. Traditional means via time-consuming histological examination is clearly unsuitable in this scenario. Biomedical imaging technologies such as CT and MRI therefore have started being used to develop more efficient phenotyping approaches. Existing work however primarily rests on volumetric analytics over anatomical structures to detect anomaly, yet this type of methods generally fail when features are subtle such as ventricular septal defects (VSD) in the heart, and meanwhile phenotypic assessment normally requires expert manual labor. This study proposes, to the best of our knowledge, the first automatic VSD diagnostic system for mouse embryos. Our algorithm starts with the creation of an atlas using wild-type mouse images, followed by registration of knockouts to the atlas to perform atlas-based segmentation on the heart and then ventricles, after which ventricle segmentation is further refined using a region growing technique. VSD classification is completed by checking the existence of an overlap between left and right ventricles. Our approach has been validated on a database of 14 mouse embryo images, and achieved an overall accuracy of 90.9%, with sensitivity of 66.7% and specificity of 100%.

  16. Peptidomic Analysis of Amniotic Fluid for Identification of Putative Bioactive Peptides in Ventricular Septal Defect

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    Xing Li

    2016-05-01

    Full Text Available Background: Ventricular septal defect (VSD is one of the most common congenital heart diseases and to date the role of peptides in human amniotic fluid in the pathogenesis of VSD have been rarely investigated. Methods: To gain insight into the mechanisms of protein and peptides in cardiovascular development, we constructed a comparative peptidomic profiling of human amniotic fluid between normal and VSD fetuses using a stable isobaric labeling strategy involving tandem mass tag reagents, followed by nano liquid chromatography tandem mass spectrometry. Results: We identified and quantified 692 non-redundant peptides, 183 of which were differentially expressed in the amniotic fluid of healthy and VSD fetuses; 69 peptides were up regulated and 114 peptides were down regulated. These peptides were imported into the Ingenuity Pathway Analysis (IPA and identified putative roles in cardiovascular system morphogenesis and cardiogenesis. Conclusion: We concluded that 35 peptides located within the functional domains of their precursor proteins could be candidate bioactive peptides for VSD. The identified peptide changes in amniotic fluid of VSD fetuses may advance our current understanding of congenital heart disease and these peptides may be involved in the etiology of VSD.

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

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    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 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.

  18. Pulmonary function in children after surgical and percutaneous closure of atrial septal defect.

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    Zaqout, Mahmoud; De Baets, Frans; Schelstraete, Petra; Suys, Bert; Panzer, Joseph; Francois, Katrien; Bove, Thierry; Coomans, Ilse; De Wolf, Daniel

    2010-11-01

    This study aimed to study differences in lung function after surgical and percutaneous atrial septal defect (ASD) closure. Several studies have demonstrated abnormalities of pulmonary function in adults and children with ASD. These abnormalities persist even a few years after correction. This study compared pulmonary function between patients who underwent ASD closure by surgery and those who had closure by device. This is the ideal pediatric population for studying changes in lung function caused by cardiopulmonary bypass or sternotomy. The 46 patients in this study were treated by percutaneous closure (group 1) or surgical closure (group 2) of ASD and then scheduled for pulmonary function testing an average of 5.8 years after ASD closure. The mean values of functional residual capacity, total lung capacity, and residual volume did not differ between the two groups. The surgical group showed a significant decrease in expiratory reserve volume (p function. Longitudinal lung function follow-up assessment after cardiac surgery is warranted to detect and measure restrictive abnormalities in this type of congenital heart disease and others.

  19. Conscious sedation using dexmedetomidine for percutaneous transcatheter closure of atrial septal defects: A single center experience

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    Pushkar Mahendra Desai

    2016-01-01

    Full Text Available Objective: The aim of this study is to determine safety and feasibility of conscious sedation using dexmedetomidine for transcatheter atrial septal defect (ASD device closure. Material and Methods: A retrospective institutional review of transcatheter ASD device closure without endotracheal intubation over 18 months. The protocol included topical oropharyngeal anesthesia using lignocaine followed by dexmedetomidine bolus 1 μg/kg intravenously over 10 min and maintenance dose 0.2-0.7 μg/kg/h. Ramsay sedation score 2-3 was maintained. Patients were analyzed regarding demographic profile, device size, procedure time, anesthesia time, recovery time, hospital stay, and any hemodynamic or procedural complications. Results: A total of 43 patients with mean age 31.56 ± 13.74 years (range: 12-56 years were analyzed. Mean anesthesia duration was 71.75 + 21.08 min. Mean recovery time was 7.6 ± 3.01 min. 16 females and one male patient required additional propofol with a mean dose of 30.8 ± 10.49 mg. No hemodynamic instability was noted. No patient required general anesthesia with endotracheal intubation. The procedure was successful in 93.02% of patients. Four patients developed atrial fibrillation. All patients were satisfied. Conclusion: Conscious sedation using dexmedetomidine is a safe and effective anesthetic technique for percutaneous ASD closure.

  20. Lung biopsy diagnosis of operative indication in secundum atrial septal defect with severe pulmonary vascular disease.

    Science.gov (United States)

    Yamaki, Shigeo; Kumate, Munetaka; Yonesaka, Susumu; Maeda, Katsuhide; Endo, Masato; Tabayashi, Koichi

    2004-10-01

    Surgical indication was determined by lung biopsy in 91 patients with secundum atrial septal defect (ASD) and severe pulmonary hypertension > 70 mm Hg of pulmonary arterial peak pressure and/or pulmonary vascular resistance of > 8 U/m(2). Pulmonary vascular disease (PVD) in ASD was classified into four types: (1) Musculoelastosis consisting of longitudinal muscle bundles and elastic fibers; surgery is indicated no matter how severely the peripheral small pulmonary arteries are occluded. Surgery was performed in all of the 20 patients, and the postoperative course was uneventful. (2) Plexogenic pulmonary arteriopathy: surgery is indicated for a PVD index < or = 2.3. Surgery was performed in 25 of the 32 patients. The remaining seven patients for whom surgery was not indicated are under follow-up observation. No deaths have occurred among the 32 patients. (3) Thromboembolism of small pulmonary arteries: Surgery is indicated for all such cases. Surgery was indicated in all of the five patients. (4) Mixed type of plexogenic pulmonary arteriopathy and musculoelastosis: Surgery is indicated if the collateral is not observed. Surgery was performed in 15 of the 25 patients. The remaining 10 patients for whom surgery was not indicated are under follow-up observation. Nine of these 91 patients associated with primary pulmonary hypertension were eliminated from this study. No deaths due to PVD occurred among the 82 patients who underwent lung biopsy diagnosis. Lung biopsy diagnosis is concluded to be very effective.

  1. Heart rate variability in patients with atrial septal defect and healthy children.

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    Bakari, Süleyman; Koca, Bülent; Oztunç, Funda; Abuhandan, Mahmut

    2013-06-01

    Heart rate variability (HRV) measures are altered in various cardiac and non-cardiac situations in children. The autonomic nervous system is assumed to have a role in the pathophysiology of atrial septal defect (ASD). In this study, we evaluated the autonomic system by measuring HRV in children with ASD. Twenty-eight patients with ASD and 32 healthy children (mean ages: 6.6±2.1 years and 6.4±2.2 years, respectively) were enrolled in the study. Twenty-four-hour ambulatory electrocardiographic recordings were obtained and the seven time-domain (SDNN, SDANN, rMSSD, SD, SDNN index, PNN50, and mean RR) and four frequency-domain (VLF, LF, HF, and LF/HF ratio) indices of HRV were analyzed. A significant decrease in calculated HRV variables was observed in children with ASD as compared to controls. The HRV alteration was found in both time-domain and frequency-domain parameters. Our results indicate that HRV is decreased in children with ASD, which implies parasympathetic withdrawal and sympathetic predominance. Copyright © 2013 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

  2. Infective endocarditis following patch closure of ventricular septal defect: a cross-sectional Doppler echocardiographic study.

    Science.gov (United States)

    Shrivastava, S; Radhakrishnan, S

    1989-10-01

    Cross-sectional and Doppler echocardiographic characteristics of infective endocarditis are described in six cases following patch closure of a ventricular septal defect. The patients presented to us with fever one to five months after surgery. Five of them also had congestive cardiac failure. Cross-sectional echocardiography showed large masses over the patch in all cases. Dehiscence of the lower end of the patch was identified in three of them, and, in two cases, the right sinus of Valsalva had ruptured into the right ventricle. Doppler detected turbulent flow in the right ventricle in five cases, and a continuous signal indicating an aorto-right ventricular communication in two cases. A signal indicative of aortic regurgitation was also found in the latter two cases. Staphylococcus aureus was cultured from the blood in three cases and Aspergillus was identified at autopsy in one. The echocardiographic findings were confirmed in three cases (one during surgery and two at autopsy). Dehiscence of the patch and large masses were associated with a poor prognosis.

  3. Efficacy of a novel IGS system in atrial septal defect repair

    Science.gov (United States)

    Mefleh, Fuad N.; Baker, G. Hamilton; Kwartowitz, David M.

    2013-03-01

    Congenital heart disease occurs in 107.6 out of 10,000 live births, with Atrial Septal Defects (ASD) accounting for 10% of these conditions. Historically, ASDs were treated with open heart surgery using cardiopulmonary bypass, allowing a patch to be sewn over the defect. In 1976, King et al. demonstrated use of a transcatheter occlusion procedure, thus reducing the invasiveness of ASD repair. Localization during these catheter based procedures traditionally has relied on bi-plane fluoroscopy; more recently trans-esophageal echocardiography (TEE) and intra-cardiac echocardiography (ICE) have been used to navigate these procedures. Although there is a high success rate using the transcatheter occlusion procedure, fluoroscopy poses radiation dose risk to both patient and clinician. The impact of this dose to the patients is important as many of those undergoing this procedure are children, who have an increased risk associated with radiation exposure. Their longer life expectancy than adults provides a larger window of opportunity for expressing the damaging effects of ionizing radiation. In addition, epidemiologic studies of exposed populations have demonstrated that children are considerably more sensitive to the carcinogenic effects radiation. Image-guided surgery (IGS) uses pre-operative and intra-operative images to guide surgery or an interventional procedure. Central to every IGS system is a software application capable of processing and displaying patient images, registration between multiple coordinate systems, and interfacing with a tool tracking system. We have developed a novel image-guided surgery framework called Kit for Navigation by Image Focused Exploration (KNIFE). In this work we assess the efficacy of this image-guided navigation system for ASD repair using a series of mock clinical experiments designed to simulate ASD repair device deployment.

  4. A HAND2 Loss-of-Function Mutation Causes Familial Ventricular Septal Defect and Pulmonary Stenosis

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    Yu-Min Sun

    2016-04-01

    Full Text Available Congenital heart disease (CHD is the most common developmental abnormality, and is the leading noninfectious cause of mortality in neonates. Increasing evidence demonstrates that genetic defects play an important role in the pathogenesis of CHD. However, CHD exhibits substantial heterogeneity, and the genetic determinants for CHD remain unknown in the overwhelming majority of cases. In the current study, the coding exons and flanking introns of the HAND2 gene, which encodes a basic helix-loop-helix transcription factor essential for normal cardiovascular development, were sequenced in 192 unrelated patients with CHD, and a novel heterozygous mutation, p.S65I, was identified in a patient with congenital ventricular septal defect (VSD. Genetic analysis of the index patient’s pedigree revealed that the mutation was present in all seven affected family members available, but absent in the 13 unaffected family members examined. Besides, in addition to VSD, five of the proband’s close relatives also had pulmonary stenosis (PS, and the proband’s son also had double outlet right ventricle (DORV. The missense mutation, which altered an evolutionarily conserved amino acid, was absent in 300 unrelated, ethnically matched healthy individuals. Biological analyses using a dual-luciferase reporter assay system showed that the mutant HAND2 was associated with significantly diminished transcriptional activity. Furthermore, the mutation abolished the synergistic activation between HAND2 and GATA4, as well as NKX2.5—two other cardiac core transcriptional factors that have been causally linked to CHD. These findings indicate that HAND2 loss-of-function mutation contributes to human CHD, perhaps via its interaction with GATA4 and NKX2.5.

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

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    Stich Kathrin

    2009-01-01

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

  6. Aneurysm of the membranous septum in adult patients with perimembranous ventricular septal defect.

    Science.gov (United States)

    Yilmaz, A T; Ozal, E; Arslan, M; Tatar, H; Oztürk, O Y

    1997-02-01

    The aneurysm of the membranous septum (AMS) has often been considered as benign in the minds of many previous investigators. We have analyzed the complications with AMS in adult patients. Fifty-one cases (20%) of AMS in 254 adult patients with perimembranous ventricular septal defect (VSD) are described. The diagnosis of AMS was based on angiographic criteria. Thirty-nine (76.5%) of the 51 patients with AMS were aged between 20 and 29 years. All patients but one with AMS had a pulmonary-to-systemic flow (Qp/Qs) of less than 2.3 (range 1-2.1, mean 1.4). In a patient who had a ruptured aneurysm, the Qp/Qs was 2.7. There were six main complications affected by AMS and/or VSD; aortic valve prolapse in 24 patients (47%), aortic regurgitation in 15 (29.4%), tricuspid insufficiency in nine (17.6%), right ventricular outflow tract obstruction in two (4%), and rupture of the aneurysm in one patient (2%). Seven patients (13.7%) had prior bacterial endocarditis. All patients underwent surgery. Aneurysm and VSD were closed by direct suture in nine and with a patch in 42 patients. Aortic valve repair was performed in 13 patients in whom regurgitation was mild to moderate, and replacement was required in two patients with severe aortic regurgitation. There were no early or late deaths. Residual communication and recurrence of the aneurysm was noted three and seven years postoperatively in two patients where VSD had been closed by direct suture. According to present data, aneurysm formation functionally reduces the VSD size, but it has the potential consequence of promoting tricuspid insufficiency, aortic valve prolapse, right ventricular outflow tract obstruction, rupture and bacterial endocarditis. Therefore, we recommend that AMS should be resected completely and the defect produced closed with a patch in order to prevent further enlargement and consequent complications even if there are no cardiac symptoms.

  7. Repair of double outlet right ventricle with doubly-committed ventricular septal defect.

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    Uemura, H; Yagihara, T; Kadohama, T; Kawahira, Y; Yoshikawa, Y

    2001-07-01

    To investigate our surgical results of intraventricular rerouting in patients having double outlet right ventricle with doubly-committed ventricular septal defect. We undertook repair in 8 patients with this particular feature. Of these, 2 patients had pulmonary stenosis, and another had interruption of the aortic arch. The subarterial defect was unequivocally related to both the aortic and the-pulmonary orifices in all, albeit slightly deviated towards the aortic orifice in one, and towards the pulmonary orifice in another. Intraventricular rerouting was carried out via incisions to the right atrium and the pulmonary trunk. To ensure reconstruction of an unobstructed pulmonary pathway, a limited right ventriculotomy was made in 5. All patients survived the procedure, and are currently doing well, with follow-up of 25 to 194 months, with a mean of 117+/-68 months. Catheterization carried out 16+/-6 months after repair demonstrated excellent ventricular parameters. Mean pulmonary arterial pressure was 16+/-7 mmHg, being higher than 20 mmHg in 2 patients. No significant obstruction was found between the right ventricle and the pulmonary arteries. A pressure gradient across the left ventricular outflow tract became significant in one patient in whom a small outlet septum was present, and a heart-shaped baffle had been used for intraventricular rerouting. Reoperation was eventually needed in this patient for treatment of the obstruction, which proved to be progressive. Precise recognition of the morphologic features is of paramount importance when choosing the optimal options for biventricular repair in patients with double outlet right ventricle and doubly-committed interventricular communication.

  8. Closure of a high ventricular septal defect after transcatheter aortic valve implantation with an atrial septal occluder-hybrid treatment for a rare complication.

    Science.gov (United States)

    Hamm, Karsten; Reents, Wilko; Kerber, Sebastian; Diegeler, Anno

    2017-03-01

    A patient with porcelain aorta underwent transcatheter aortic valve implantation with a self-expandable prosthesis for severe aortic stenosis. After postdilatation trace paravalvular regurgitation was accepted. 10 weeks later the patient returned with complete heart block and underwent pacemaker implantation. A new heart murmur prompted further investigation. A ventricular septal defect from the left ventricular outflow tract into the right ventricle was detected. It was successfully closed under direct surgical visualization and total cardiopulmonary bypass in an aortic no touch approach. Closure was accomplished with a percutaneous Amplatzer-PFO-occluder. Functional result was excellent. © The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  9. Transcathether closure of a right pulmonary artery-to-left atrial fistula using Amplatzer muscular ventricular septal defect occluder.

    Science.gov (United States)

    Ece, Ibrahim; Uner, Abdurrahman; Cuce, Ferhat; Balli, Sevket

    2014-10-01

    A right pulmonary artery-to-left atrial fistula is a very rare cyanotic congenital heart defect and is characterized by cyanosis and normal auscultation of the heart. Interventional closure of the fistula using occluder devices and coils has been rarely reported. We report the successful closure of a RPA-to-left atrial fistula using an Amplatzer muscular ventricular septal defect occluder in a child with cyanosis. The two-dimensional echocardiogram with bubble contrast study demonstrated the communication between right pulmonary artery and left atrium. Computerized tomography confirmed the diagnosis and delineated the anatomy.

  10. Effects of surgical en bloc rotation of the arterial trunk on the conduction system in children with transposition of the great arteries, ventricular septal defect and pulmonary stenosis.

    Science.gov (United States)

    Prandstetter, Christoph; Tulzer, Andreas; Mair, Rudolf; Sames-Dolzer, Eva; Tulzer, Gerald

    2016-03-01

    The standard surgical management of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis is the Rastelli operation. Recently, en bloc rotation of the arterial trunk, by cutting out the aortic and the pulmonary root in one block and by rotating it 180°, has been introduced as a new option for anatomical repair. To evaluate the effects of this surgical method on the conduction system, pre-operative, post-operative, and follow-up electrocardiograms as well as patient charts were reviewed retrospectively. A total of 16 consecutive patients with transposition of the great arteries and left outflow tract obstruction were treated with en bloc rotation. During the post-operative period, there were two patients with complete atrio-ventricular block, one with junctional ectopic tachycardia, one with ventricular tachycardia, and one with supraventricular tachycardia. None of the patients had a typical right bundle branch block pattern before surgery; however, this pattern was detectable after surgery in eight out of 16 patients (50%), which persisted during the follow-up. All patients without typical right bundle branch block pattern showed a median QRS duration of 65 ms (54-112 ms) before surgery, 62 ms (54-122 ms) after surgery, and 84 ms (66-128 ms) at the last follow-up visit. This compares well with a similar Rastelli cohort, where a right bundle branch block prevalence of 77% was reported. Out of 16 patients, 12 showed non-specific ST changes and negative T-waves, which persisted during follow-up with an unknown significance for the future. Our data suggest that en bloc rotation of the arterial trunk seems not to have more negative effects on the conduction system than the Rastelli operation.

  11. TOGETHER WiTH ATRiAL SEPTAL DEFECT, BiCUSPiD AORTA, PECTUS EXCAVATUM AND MENTAL RETARDATiON : A CASE REPORT

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    Mustafa Necati Dagli

    2015-03-01

    Full Text Available In this case, patient with mental retardation accepted in our clinic complain with shortness of breath. Then physical examination inspection evident in the pectus excavatum and made Echocardiography Atrial septal defect (ASD and bicuspid aortic consistent with the view were identified.Congenital abnormalities in patients with the aim of further evaluation of transesophageal echocardiography (TEE planned. In TEE, 26 mm secundum atrial septal defect, bicuspid aortic valve was detected. Percutaneous transcatheter atrial septal defect closure with septal occluder device. Pectus excavatum, such as mental retardation or even cardiac abnormalities do not complain about aspects of the research must be done. Cardiac abnormalities regardless of the direction of the association needs to be done to show that we have a rigorous screening. [J Contemp Med 2015; 5(1.000: 48-50

  12. [Left ventricular-right atrial communication with a residual ventricular septal defect: a case report of successful VSD patch closure with tricuspid valvuloplasty using folded patch].

    Science.gov (United States)

    Kanazawa, M; Katoh, T; Suzuki, K; Fujimura, Y; Tsuboi, H; Esato, K

    1992-12-01

    A 18-year-old woman with combination of left ventricular right atrial communication (LV-RA communication) and residual ventricular septal defect (VSD) was presented. She underwent the radical operation of atrial septal defect, VSD and infundibular pulmonary stenosis at the age 11 years. Surgery was performed under the diagnosis of LV-RA communication and residual VSD. Two small defect were existed at the septal leaflet of the tricuspid valve, which communicated to left ventricle. The part of patch being used as closure of VSD at the previous operation, including perforated part of the septal leaflet was resected. A folded patch was used to reinforce the residual VSD and valvuloplasty was made using the remaining intact tricuspid tissues. Post-operative clinical course was uneventful. Shunt flow from LV to RA was not demonstrated in the postoperative left ventricular angiocardiography.

  13. Intermediate and long-term followup of percutaneous device closure of fossa ovalis atrial septal defect by the Amplatzer septal occluder in a cohort of 529 patients

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    Tomar Munesh

    2011-01-01

    Full Text Available Objectives : The aim of present study is to analyze the intermediate and long-term follow up results of percutaneous closure of fossa ovalis atrial septal defect (ASD with Amplatzer septal occluder (ASO in a large cohort of patients including children and adults. Methods : Between May 1998 and July 2008, 529 patients (age group 2-77 years, median 28 years underwent successful device closure with an ASO at single tertiary referral cardiac center in India.. This was out of an attempted 543 cases. The procedure was carried out in catheterization laboratory under transesophageal echocardiographic and fluoroscopy guidance. The mean size of ASD was 20 mm (7-40 mm while size of septal occluder was 10-40 mm (mean 24 mm. Two devices were deployed in four patients. Three patients developed transitory pulmonary edema in immediate postprocedure period requiring ICU care for 48 hrs. All patients were advised for Aspirin (3-5 mg/kg, maximum 150 mg once daily for 6 months. In patients with device 30 mm or larger, Clopidogril ( 75 mg once daily was given for 3 months in addition to Aspirin. Clinical evaluation, echocardiogram were done on 3 months, 6 months and then at 1, 3, 5, 7 and 10 years of follow up. Transesophageal echocardiography (TEE was performed in case of any doubt on clinical evaluation or on transthoracic echocardiography (n=10. Results : Followup data is available for 496 patients (93.7%. Followup period is from 12 months to 120 months (median 56 months. On followup, device was in position in all patients, no residual shunt and no evidence of thrombosis. Interventricular septal motion normalized on day of procedure in 89% patients, in 6% over 3 months while flat septal motion persisted in 5% (n=25, all in age group > 40 years of cases, though right ventricular dilatation persisted in 10% (n=50, age more than 40 years of patients. Symptom-free survival was 96.7 % (480/496 in patients who came for followup. Only one 68 year old patient with

  14. No-touch aorta robot-assisted atrial septal defect repair via two ports.

    Science.gov (United States)

    Ishikawa, Norihiko; Watanabe, Go; Tarui, Tatsuya

    2018-01-02

    Atrial septal defect (ASD) repairs have been successfully performed on arrested hearts with robotic assistance. The present study assessed the feasibility, safety, and efficacy of totally endoscopic cardiac surgery using a no-touch aorta technique for ASD via only 2 ports, and we named this procedure two-port robotic cardiac surgery (TROCS). Between May 2014 and June 2016, 8 consecutive patients underwent TROCS for ASD using the da Vinci surgical system (Intuitive Surgical Inc.) at our institute. All of the procedures were performed via only 2 port incisions in the right chest. One was the camera port, and the other was the port for the robotic instruments. Both robotic instruments were inserted through this port and crossed while being prevented from colliding with each other. The surgeon console was set to the reverse of default settings so that both masters would control the inverse instrument. TROCS for ASD was carried out under ventricular fibrillation induced by combinations of an electrical fibrillator, injection of potassium, and hypothermia without aortic cross-clamping. All cases were successfully repaired. The mean operation, cardiopulmonary bypass and ventricular fibrillation times were 129.6 ± 29.0 min, 66.9 ± 24.5 min and 9.6 ± 5.9 min, respectively, and the estimated blood loss volume was 28.1 ± 58.6 ml. No patients required blood transfusion during their hospital stay, and their cosmetic results were excellent. TROCS for ASD using no-touch aorta technique was achieved safely with good clinical results and excellent cosmetic results.

  15. A Complication following the Transcatheter Closure of a Muscular Ventricular Septal Defect

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    Mustafa Karaçelik

    2015-12-01

    Full Text Available Today, congenital heart diseases may be treated without surgery through advances in interventional cardiology. However, complications such as infection and thrombus formation may develop due to foreign materials used during these procedures. Surgical intervention may be required for the removal of the device utilized for the procedure. In this case report, we present the surgical treatment of a residual ventricular septal defect (VSD that had developed in a 6-year-old patient with an apical muscular VSD closed with the Amplatzer muscular VSD device. The patient was admitted to the emergency room with complaints of abdominal pain and high fever 5 days after discharge without any cardiac symptoms. When she arrived at our clinic, she had a heart rate of 95 bpm, blood pressure of 110/70 mmHg, and temperature of 38.5ºC. Examinations of the other systems were normal, except for a 3/6 pan-systolic murmur at the mesocardiac focus on cardiac auscultation. Echocardiography showed a residual VSD, and the total pulmonary blood flow to the total systemic blood flow ratio (Qp/Qs of the residual VSD was 1.8. In the operating room, the Amplatzer device was removed easily with a blunt dissection. The VSD was closed with an autologous fresh pericardial patch. Following the pulmonary artery debanding procedure, the postoperative period was uneventful. The condition of the patient at the time of discharge and in the first postoperative month’s follow-up was good. There was no residual VSD or infection. 

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

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    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

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

    Science.gov (United States)

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

    2017-12-01

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

  18. Analysis of MTHFR and MTRR Gene Polymorphisms in Iranian Ventricular Septal Defect Subjects

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    Ali Etemad

    2013-01-01

    Full Text Available Ventricular septal defect (VSD is one of the most common types of congenital heart defects (CHD. There are vivid multifactorial causes for VSD in which both genetic and environmental risk factors are consequential in the development of CHD. Methionine synthase reductase (MTRR and methylenetetrahydrofolate reductase (MTHFR are two of the key regulatory enzymes involved in the metabolic pathway of homocysteine. Genes involved in homocysteine/folate metabolism may play an important role in CHDs. In this study; we determined the association of A66G and C524T polymorphisms of the MTRR gene and C677T polymorphism of the MTHFR gene in Iranian VSD subjects. A total of 123 children with VSDs and 125 healthy children were included in this study. Genomic DNA was extracted from the buccal cells of all the subjects. The restriction fragment length polymorphism polymerase chain reaction (PCR-RFLP method was carried out to amplify the A66G and C524T polymorphism of MTRR and C677T polymorphism of MTHFR genes digested with Hinf1, Xho1 and Nde1 enzymes, respectively. The genotype frequencies of CC, CT and TT of MTRR gene among the studied cases were 43.1%, 40.7% and 16.3%, respectively, compared to 52.8%, 43.2% and 4.0%, respectively among the controls. For the MTRR A66G gene polymorphism, the genotypes frequencies of AA, AG and GG among the cases were 33.3%, 43.9% and 22.8%, respectively, while the frequencies were 49.6%, 42.4% and 8.0%, respectively, among control subjects. The frequencies for CC and CT genotypes of the MTHFR gene were 51.2% and 48.8%, respectively, in VSD patients compared to 56.8% and 43.2% respectively, in control subjects. Apart from MTHFR C677T polymorphism, significant differences were noticed (p < 0.05 in C524T and A66G polymorphisms of the MTRR gene between cases and control subjects.

  19. Combined double chambered right ventricle, tricuspid valve dysplasia, ventricular septal defect, and subaortic stenosis in a dog.

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    Scurtu, Iuliu; Tabaran, Flaviu; Mircean, Mircea; Giurgiu, Gavril; Nagy, Andras; Catoi, Cornel; Ohad, Dan G

    2017-11-29

    Double chambered right ventricle (DCRV) is a congenital heart anomaly where the right ventricle is divided into two chambers. We describe, for the first time, an unusual combination of DCRV combined with some other congenital heart defects. A 1.2-year-old Golden Retriever was presented with lethargy, exercise intolerance and ascites. Physical examination revealed an irregularly irregular pulse and a grade V/VI, systolic, right cranial murmur. Electrocardiography revealed widened and splintered QRS complexes with a right bundle-branch block pattern. Radiography demonstrated right-sided cardiomegaly. Two-dimensional echocardiography identified a DCRV with tricuspid valve dysplasia. The patient died despite abdominocentesis and 4 days of oral pharmacotherapy, and necropsy revealed an anomalous fibromuscular structure that divided the right ventricle into two compartments. Another finding was tricuspid valve dysplasia with hypoplasia of the posterior and septal leaflets. The anterior leaflet was prominent, being part of the anomalous structure that divided the right ventricle. Necropsy also identified a perimembranous ventricular septal defect and mild subaortic stenosis. Histopathological examination of the fibromuscular band that separated the right ventricle identified longitudinally oriented layers of dense fibrous connective tissue and myocardial cells arranged in a plexiform pattern. The muscular component was well represented at the ventral area of the fibromuscular band, and was absent in the central zone. Superficially, the endocardium presented areas of nodular hyperplasia covering mainly the fibrous part of the abnormal structure. The nodules were sharply demarcated and were composed by loosely arranged connective tissue with myxoid appearance, covered by discrete hyperplastic endocardium. Concomitant cardiac malformations involving DCRV, tricuspid valve dysplasia, perimembranous ventricular septal defect and mild subaortic stenosis have not been previously

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

    DEFF Research Database (Denmark)

    Petersen, Cecilie; Helvind, Morten; Jensen, Tim

    2013-01-01

    We report on a 10-year-old boy with medically refractory pulmonary arterial hypertension (PAH) and end-stage right heart failure after closure of a ventricular septal defect. The boy was a candidate for lung transplantation (LTX), but an alternative option was to create an Eisenmenger physiology...... on the right ventricle. The boy's clinical condition improved markedly, so he was discharged two weeks after the procedure. The ultimate therapeutic option for medically refractory PAH is LTX or heart-lung transplantation, but because of the short life span after LTX, time was bought by postponing the time...

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

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    Francesco Secchi

    2013-01-01

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

  2. Constrictive pericarditis in a patient with sinus venosus atrial septal defect and anomalous right upper pulmonary venous return

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    Uppu Santosh

    2009-01-01

    Full Text Available This is a report of a 49-year-old male, who presented with typical signs and symptoms of constrictive pericarditis. He was diagnosed with sinus venosus atrial septal defect (ASD and anomalous right upper pulmonary venous return during his adolescence, which was elected not to be repaired. During the attempted repair of the ASD it was noted there was a thick fibrous material covering the heart, which had progressed over time leading to frank constrictive pericarditis. His ASD spontaneously closed over time. There have been less than 10 cases reported with constrictive pericarditis of nonsurgical etiology in a patient with ASD, and none with sinus venosus ASD.

  3. Traumatic ventricular septal defect and flail tricuspid valve: successful management by an extracorporeal membrane oxygenator-supported hybrid approach.

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    Ling, Frederick S; Massey, H Todd

    2014-03-01

    We report the successful management of a critically ill patient with a traumatic ventricular septal defect (VSD) and flail tricuspid valve sustained in a motorcycle accident. Multiple orthopedic injuries prevented emergency cardiac surgery. The patient was stabilized by venous arterial extracorporeal membrane oxygenator support which allowed initial orthopedic repair. Repair of his cardiac injuries was then accomplished using a hybrid approach of percutaneous VSD closure using an Amplatzer post myocardial infarction VSD occluder which was also coil embolized followed by surgical tricuspid valve replacement. Copyright © 2013 Wiley Periodicals, Inc.

  4. Repair of postinfarct ventricular septal defect and total myocardial revascularization in a case of dextrocardia with situs inversus.

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    Kuthe, Sachin A; Mohite, Prashant N; Sarangi, Siddharth; Mathews, Sarin; Thingnam, Shyam K; Reddy, Sreenivas

    2011-01-01

    We report a case of an elderly man who suffered an acute myocardial infarction (MI) with the complication of a post-MI ventricular septal defect (VSD). Situs inversus with dextrocardia was diagnosed during the course of hospitalization. Total myocardial revascularization was achieved using saphenous vein conduits. The VSD was approached through the right ventricle and repaired with a polytetrafluoroethylene patch. Although several cases of coronary artery bypass grafting (CABG) in the presence of dextrocardia have been reported in the literature, this is the first case of repair of a post-MI VSD along with CABG.

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

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    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.

  6. Extracorporeal membrane oxygenation system as a bridge to reparative surgery in ventricular septal defect complicating acute inferoposterior myocardial infarction

    Science.gov (United States)

    Pascual, Isaac; Avanzas, Pablo; Hernandez-Vaquero, Daniel; Alvarez, Ruben; Díaz, Rocio; Díaz, Beatriz; Martín, María; Carro, Amelia; Muñiz, Guillermo; Silva, Jacobo; Moris, Cesar

    2017-01-01

    Post-infarction ventricular septal defect (VSD) is a rare but potentially lethal complication of acute myocardial infarction. Medical management is usually futile, so definitive surgery remains the treatment of choice but the risk surgery is very high and the optimal timing for surgery is still under debate. A 55-year-old man with no previous medical history attended the emergency-room for 12 h evolution of oppressive chest pain and strong anginal pain 7 days ago. On physical examination, blood pressure was 96/70 mmHg, pansystolic murmur over left sternal border without pulmonary crackles. An electrocardiogram revealed sinus rhythm 110 bpm, elevation ST and Q in inferior-posterior leads. Transthoracic echocardiogram showed inferoposterior akinesia, posterior-basal septal rupture (2 cm × 2 cm) with left-right shunt. Suspecting VSD in inferior-posterior acute myocardial infarction evolved, we performed emergency coronarography with 3-vessels disease and complete subacute occlusion of the mid segment of the right coronary artery. Left ventriculography demonstrated shunting of contrast from the left ventricule to the right ventricule. He was rejected for heart transplantation because of his age. Considering the high surgical risk to early surgery and his hemodynamic and clinical stability, delayed surgical treatment is decided, and 4 days after admission the patient suffered hemodynamic instability so venoarterial extracorporeal membrane oxygenation system (ECMO) is implanted as a bridge to reparative surgery. The 9th day after admission double bypass, interventricular defect repair with pericardial two-patch exclusion technique, and ECMO decannulation were performed. The patient’s postoperative course was free of complications and was discharged 10 days post VSD repair surgery. Follow-up 3-month later revealed the patient to be in good functional status and good image outcome with intact interventricular septal patch without shunt. ECMO as a bridge to reparative

  7. Miniinvasive hybrid closure of multiple muscular ventricular septal defects in a premature infant with novel use of Amplatzer Duct Occluder II – a case report

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    Ireneusz Haponiuk

    2011-03-01

    Full Text Available Muscular ventricular septal defects (mVSD appearing together with other septal defects are frequently regarded as “concomitant” pathologies, that nevertheless should be considered while the patient is referred for intervention. We followed a conception of mVSDs’ miniinvasive treatment with a hybrid approach based on perventricular implantation of occluding devices. In this paper we report a hybrid procedure performed in a premature infant referred for surgical correction of a large perimembranous VSD with a simultaneous perventricular approach for concomitant muscular ventricular septal defect. The device of choice, because of the patient’s small size and weight, was the Amplatzer Duct Occluder II. Colour Doppler showed complete closure of all VSDs 8 months after surgery with no complications related to the procedure.

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

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    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.

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

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    Jian-Yuan Zhao

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

  10. Transcatheter closure of secundum atrial septal defect in adults: report of our first experience in a developing country

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    Animasahun BA

    2014-04-01

    Full Text Available B Adeola Animasahun,1 Yele Aluko,2 Adeyemi Johnson,3 Kofo Ogunyankin,3 Sunita Maheshwari41Department of Paediatrics and Child Health, Lagos State University College of Medicine, Lagos, Nigeria; 2Mid Carolina Cardiology, Charlotte, NC, USA; 3First Cardiology Consultants, Lagos, Nigeria; 4Narayana Hrudayalaya Institute of Cardiac Sciences, Bangalore, IndiaBackground: Since the first report on device closure of atrial septal defect (ASD in 1976, the procedure has gained wide acceptance and has been used worldwide, including parts of Africa. The advantages when compared to surgical closure include a shorter procedure time and hospital stay and no scarring. This mode of treatment was not available in Nigeria prior to the cases reported here, requiring patients to travel abroad for the procedure.Methods: A cardiac catheterization laboratory became available in Lagos, Nigeria in 2009, and in December 2010 the laboratory collaborated with Lagos State University College of Medicine and Lagos State University Teaching Hospital to perform the device closure on two women (aged 34 and 62 years with secundum ASD. To the best of our knowledge, these are the first times the procedure has been performed in Nigeria.Results: Both patients made successful recovery and are stable.Conclusion: Transcatheter closure of ASD is now safe and available in Nigeria.Keywords: atrial septal defect, transcatheter closure, Nigeria

  11. Cardiac resynchronization therapy for heart failure induced by left bundle branch block after transcatheter closure of ventricular septal defect

    Science.gov (United States)

    Du, Rong-Zeng; Qian, Jun; Wu, Jun; Liang, Yi; Chen, Guang-Hua; Sun, Tao; Zhou, Ye; Zhao, Yang; Yan, Jin-Chuan

    2014-01-01

    A 54-year-old female patient with congenital heart disease had a persistent complete left bundle branch block three months after closure by an Amplatzer ventricular septal defect occluder. Nine months later, the patient suffered from chest distress, palpitation, and sweating at daily activities, and her 6-min walk distance decreased significantly (155 m). Her echocardiography showed increased left ventricular end-diastolic diameter with left ventricular ejection fraction of 37%. Her symptoms reduced significantly one week after received cardiac resynchronization therapy. She had no symptoms at daily activities, and her echo showed left ventricular ejection fraction of 46% and 53%. Moreover, left ventricular end-diastolic diameter decreased 6 and 10 months after cardiac resynchronization therapy, and 6-min walk distance remarkably increased. This case demonstrated that persistent complete left bundle branch block for nine months after transcatheter closure with ventricular septal defect Amplatzer occluder could lead to left ventricular enlargement and a significant decrease in left ventricular systolic function. Cardiac resynchronization therapy decreased left ventricular end-diastolic diameter and increased left ventricular ejection fraction, thereby improving the patient's heart functions. PMID:25593586

  12. Coronary to pulmonary fistula as the primary source of pulmonary blood supply in pulmonary atresia with ventricular septal defect

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    Isman Firdaus

    2004-12-01

    Full Text Available A communication between the coronary and pulmonary arteries, so called coronary to pulmonary fistula, is a rare source of pulmonary supply in pulmonary atresia (PA with ventricular septal defect (VSD. A 4 year old girl referred to National Cardiovascular Center Harapan Kita, Jakarta with symptoms and signs of increased pulmonary blood flow since infancy and was confirmed by the chest x-rays. Heart examination revealed normal first heart sound with single loud second heart sound and an ejection systolic murmur at the pulmonary area. ECG demonstrated sinus rhythm with normal axis and biventricular hypertrophy. Echocardiography was performed and truncus arteriosus (TA type I was suspected with perimembranus VSD, overriding of the aorta, and dilated main pulmonary artery. But on cardiac catheterization studies, a non obstructive fistula was found between the left coronary and main pulmonary artery coexisted with PA and VSD. A successful surgery was performed subsequently and confirmed the above diagnosis. Although there were episodes of pulmonary hypertension crisis during early post operative course, she was then discharge from the hospital in a good condition. Since irreversible pulmonary vascular disease may develop in a non restrictive coronary to pulmonary fistula, early recognition of this anomaly is very important for better surgical result. (Med J Indones 2004; 13: 237-40Keywords: coronary to pulmonary fistula, pulmonary atresia, ventricular septal defect

  13. Second natural history study of congenital heart defects. Quality of life of patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect.

    Science.gov (United States)

    Gersony, W M; Hayes, C J; Driscoll, D J; Keane, J F; Kidd, L; O'Fallon, W M; Pieroni, D R; Wolfe, R R; Weidman, W H

    1993-02-01

    Quality of life of patients with congenital heart defects is an important aspect of the assessment of outcome of medical and surgical treatment. All participants in the Second Natural History Study of Congenital Heart Defects who completed the study questionnaire were included in this analysis. The questionnaire completed by the patients included inquiries relative to medical history, marital and family life, self-perception of well-being, insurability, and employability. The following general observations were apparent. The study cohort reported a self-perception of health status that was similar to that of the general population. Second, 35-40% of patients had not had a cardiac evaluation within the previous 10 years. Third, 16% of the patients had no health insurance, and 35.7% of the patients had no life insurance. For the patients with pulmonary stenosis and ventricular septal defect, the percent who were married was less than that of the corresponding national age- and sex-specific population. For patients with aortic stenosis, the percent married was greater than that of the general population for some age groups. The proportion of divorced or separated individuals was similar to that of the general population. For all three defects, the level of educational attainment exceeded the national average. Last, unemployment rates were similar to national averages except for women with aortic stenosis, who had a significantly higher unemployment rate than did age- and sex-matched controls. Patients initially identified with aortic stenosis, pulmonary stenosis, or ventricular septal defect in 1959-1973 have a quality of life in the mid-1980s similar to that of the general US population.

  14. Preincision Initiation of Dexmedetomidine Maximally Reduces the Risk of Junctional Ectopic Tachycardia in Children Undergoing Ventricular Septal Defect Repairs.

    Science.gov (United States)

    Gautam, Nischal K; Turiy, Yuliya; Srinivasan, Chandra

    2017-04-10

    To evaluate whether initiation of dexmedetomidine (DEX) infusion before surgical incision and cardiopulmonary bypass (CPB) versus initiation after CPB had an impact on the incidence of junctional ectopic tachycardia (JET). Retrospective cohort study. Single tertiary-care cardiac center. Children undergoing cardiopulmonary bypass for repair of congenital heart disease involving ventricular septal defects between January 2010 and February 2013. None. One hundred thirty-four patients undergoing ventricular septal defect closure were included in the final analysis. Of the 99 patients (74%) exposed to DEX, intraoperative initiation was performed in 73 (pre-CPB, n = 39 patients [29%]; intraoperative post-CPB initiation, n = 34 patients [25%]), and postoperative initiation was performed on arrival to the intensive care unit (ICU) in 26 patients (19%). In 71 of the 73 patients, infusions that were initiated intraoperatively were continued in the postoperative period for up to the first 12 hours. Postoperative JET was observed in 22 of the 134 patients (15%). Of the 99 patients exposed to DEX in the perioperative period, JET was observed in 8 patients (11%). Of the 35 patients not exposed to any DEX, JET was observed in 12 patients (34%). Analysis was performed using DEX exposure and timing as predictor variables. Multivariable analysis modeled with DEX exposure as a predictor variable showed that when initiated preincision and continued through the postoperative period, DEX was associated with significant reduction in postoperative JET (odds ratio [OR] 0.09, 95% confidence interval [CI] 0.02-0.37, p = 0.002). Exposure to DEX in the postoperative period alone did not result in suppression of JET (OR 0.5, 95% CI 0.11-2.17, p = 0.366). When modeled by using timing of DEX initiation as the predictive variable, preincision initiation of DEX infusion resulted in significantly greater suppression of JET (OR 0.04, 95% CI 0.002-0.28, p = 0.006) compared with initiation

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

    Science.gov (United States)

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

    2013-10-09

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

  16. [Feasibility of device closure for multiple atrial septal defects using 3D printing and ultrasound-guided intervention technique].

    Science.gov (United States)

    Qiu, X; Lü, B; Xu, N; Yan, C W; Ouyang, W B; Liu, Y; Zhang, F W; Yue, Z Q; Pang, K J; Pan, X B

    2017-04-25

    Objective: To investigate the feasibility of trans-catheter closure of multiple atrial septal defects (ASD) monitored by trans-thoracic echocardiography (TTE) under the guidance of 3D printing heart model. Methods: Between April and August 2016, a total of 21 patients (8 male and 13 female) with multiple ASD in Fuwai Hospital of Chinese Academy of Medical Sciences underwent CT scan and 3-dimensional echocardiography for heart disease model produced by 3D printing technique. The best occlusion program was determined through the simulation test on the model. Percutaneous device closure of multiple ASD was performed follow the predetermined program guided by TTE. Clinical follow-up including electrocardiogram and TTE was arranged at 1 month after the procedure. Results: The trans-catheter procedure was successful in all 21 patients using a single atrial septal occluder. Mild residual shunt was found in 5 patient in the immediate postoperative period, 3 of them were disappeared during postoperative follow-up. There was no death, vascular damage, arrhythmia, device migration, thromboembolism, valvular dysfunction during the follow-up period. Conclusion: The use of 3D printing heart model provides a useful reference for transcatheter device closure of multiple ASD achieving through ultrasound-guided intervention technique, which appears to be safe and feasible with good outcomes of short-term follow-up.

  17. National time trends in congenital heart defects, Denmark, 1977-2005.

    Science.gov (United States)

    Oyen, Nina; Poulsen, Gry; Boyd, Heather A; Wohlfahrt, Jan; Jensen, Peter K A; Melbye, Mads

    2009-03-01

    Time trends in congenital heart defects (CHD) by specific phenotype and with long follow-up time are rarely available for an entire population. We present trends in national CHD prevalences over the past 3 decades. We linked information from the National Patient Register, the Causes of Death Register, and the Danish Cytogenetic Central Register for all persons born in Denmark, 1977 to 2005, and registered in the Civil Registration System, yielding a cohort of 1,763,591 persons-18,207 with CHD. Individuals with CHDs were classified by phenotype (heterotaxia, conotruncal defect, atrioventricular septal defect, anomalous pulmonary venous return, left and right ventricular outflow tract obstructions, septal defects, complex defects, associations, patent ductus arteriosus, unspecified, and other specified) by combining International Classification of Diseases codes using a hierarchical approach. From 1977 to 2005, the overall CHD birth prevalence increased from 73 to 113 per 10,000 live births. Generally, prevalence increased for defects diagnosed in infancy, until 1996-1997, and then stabilized. For each 5-year interval, isolated septal defects and severe defects increased by 22% (95% CI, 20%-25%) and 5% (95% CI, 4%-7%), respectively. Among the severe defects, conotruncal defects and atrioventricular septal defect showed the largest prevalence increases. Women had a lower prevalence of severe defects during the 1980s. The CHD prevalence increase was unchanged when persons with extracardiac defects or chromosomal aberrations were excluded. CHD birth prevalence increased from the beginning of the 1980s but stabilized in the late 1990s.

  18. Surgery for complications of trans-catheter closure of atrial septal defects : a multi-institutional study from the European Congenital Heart Surgeons Association

    NARCIS (Netherlands)

    Sarris, George E.; Kirvassilis, George; Zavaropoulos, Prodromos; Belli, Emre; Berggren, Hakan; Carrel, Thierry; Comas, Juan V.; Corno, Antonio F.; Daenen, Willem; Di Carlo, Duccio; Ebels, Tjark; Fragata, Jose; Hamilton, Leslie; Hraska, Viktor; Jacobs, Jeffrey; Lazarov, Stojan; Mavroudis, Constantine; Metras, Dominique; Rubay, Jean; Schreiber, Christian; Stellin, Giovanni

    Objective: This study aims to analyse the collective experience of participating European Congenital Heart Surgeons Association centres in the surgical management of complications resulting from trans-catheter closure of atrial septal defects (ASDs). Methods: The records of all (n = 56) patients,

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

    Directory of Open Access Journals (Sweden)

    Nageswara Rao Koneti

    2013-01-01

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

  20. A novel one-shot circular stapler closure for atrial septal defect in a beating-heart porcine model.

    Science.gov (United States)

    Tarui, Tatsuya; Tomita, Shigeyuki; Ishikawa, Norihiko; Ohtake, Hiroshi; Watanabe, Go

    2015-02-01

    In surgical atrial septal defect (ASD) closure, there are no techniques or devices that can close the ASD accurately in a short time under a beating heart. We have developed a simple and automatic ASD closure technique using a circular stapler. This study assessed the feasibility and efficacy of a new circular stapler closure for ASD. Under a continuous beating heart, hand-sewn patch plasty ASD closure was performed in 6 pigs (group A) and circular stapler ASD closure was performed in 6 pigs (group B). The time to close the ASD and the effectiveness of the closure were compared. Closure was significantly faster in group B (10.5 ± 1.0 seconds) than in group A (664 ± 10 seconds; p heart porcine model. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  1. Robotic atrial septal defect closure and tricuspid annuloplasty in a case of situs inversus totalis with dextrocardia.

    Science.gov (United States)

    Onan, Burak; Aydin, Unal; Kahraman, Zeynep; Bakir, Ihsan

    2017-03-01

    Situs inversus totalis (SIT) with dextrocardia is an uncommon congenital positional anomaly, which is characterized by a symmetrical 'mirror-image' orientation of all organs in relation to the midline. Although sternotomy and thoracotomy is traditionally used in patients with SIT with dextrocardia, a totally endoscopic robotic surgery is an alternative surgical approach to intracardiac anomalies. Placement of robotic ports, transthoracic aortic clamp, cardioplegia delivery, and peripheral vascular cannulation is made from the left side of the chest, as a mirror orientation of the right-sided robotic cardiac procedures. Here, we present a patient who underwent concomitant robotic atrial septal defect closure and tricuspid annuloplasty with posterior plication using the da Vinci surgical system.

  2. [Maze procedure in a case of dextrocardia with atrial septal defect and persistent left superior vena cava].

    Science.gov (United States)

    Muraoka, Arata; Kawada, Masaaki; Misawa, Yoshio

    2014-08-01

    A 52-year-old man was diagnosed with dextrocardia at the age of 1 year and was asymptomatic until 1 year before admission. He was transferred to our hospital for management of atrial fibrillation. A transthoracic echocardiogram showed dextrocardia with atrial septal defect;moderate tricuspid valve regurgitation; and a large, persistent left superior vena cava. A cardiac catheterization study revealed that pulmonary flow/systemic flow (Qp/Qs) was 3.6 and that pulmonary vascular resistance was 2.5 Wood U·m². Intracardiac repair with tricuspid annuloplasty and a maze procedure was scheduled. When establishing cardiopulmonary bypass, venous drainage was initially obtained from the inferior vena cava and the left superior vena cava, and the small superior vena cava was then directly cannulated after opening the right atrium. The patient's postoperative course was uneventful, and serial electrocardiograms have demonstrated maintenance of normal sinus rhythm for 3.5 years after the operation.

  3. [Radial diagnosis MRT and MSCT in assessing the outcomes of surgical correction for complicated congenital heart defects: discordant atrioventricular connections with double outlet right ventricle].

    Science.gov (United States)

    Mershina, E A; Frolova, Iu V; Seliavko, Iu A; Sinitsyn, V E

    2012-01-01

    Discordant atrioventricular connection with double outlet right ventricle is a rare complicated congenital heart defect (CHD) requiring accurate diagnosis and appropriate correction. Magnetic resonance imaging (MRI) and multiple spiral computed tomography (MSCT) were used to assess long-term outcomes of the «classical» repair of the double outlet right ventricle in a patient presenting with discordant atrioventricular connection. Using a modified segmental approach provided all necessary anatomical evidence concerning the condition of the heart, major vessels, and an extracardiac pulmonary valve-containing conduit. MSCT made it possible to evaluate the degree of calcinosis and stenosis of the conduit and to visualize the coronary arteries. MRI was employed to assess the pressure gradient at the level of stenosis of the conduit, the relationship between the pulmonary and systemic circulation, transvalvular regurgitation, and ventricular contractility. Comprehensive use of present-day tomographic methods of imaging made it possible to obtain complete anatomical and functional information about the condition of the heart, vessels, extracardiac vascular transplant, as well as to reveal complications and determine the indications for a repeat operative intervention.

  4. Transthoracic echocardiography is a safe alternative for assessment and guidance of transcatheter closure of secundum atrial septal defect in children.

    Science.gov (United States)

    Baruteau, Alban-Elouen; Hascoët, Sébastien; Fraisse, Alain

    2017-05-01

    2D-transesophageal echocardiography (TEE) is routinely performed to guide percutaneous ASD closure in children. We aimed to assess whether two-dimensional (2D)-transthoracic echocardiography (TTE) is a safe alternative for assessment and guidance of atrial septal defect (ASD) closure in unselected children. We performed a retrospective single-center study including 389 consecutive children aged less than 15-year-old who underwent percutaneous ASD closure under 2D-TEE (1998-2005, n=133) or 2D-TTE (2005-2014, n=256). A balloon calibration was performed in all cases for the Amplatz Septal Occluder choice. ASDs were larger and rims deficiencies were more frequent in the TTE-guided group. The procedure was successful in 376 patients [96.7%; 95% confidence interval (CI), 94.4-98.2%]. The success rate tended to be higher in the TTE- versus TEE-guided group (98.0% versus 94.0%, P=0.069). Device migration occurred in 4 patients (1.0%; 95% CI: 0.3-1.6%), all after TEE-guided procedure (P=0.013). Early major adverse events were observed in 5 patients (1.3%; 95% CI: 0.4-3.0%), all in the TEE group (P=0.004). Fluroroscopic time and irradiation dose were not different among the 2 groups (P=0.450 and P=0.130 respectively). After a median follow-up of 7 years (range, 1-16 years), no adverse events was reported. One (0.3%, 95% CI: 0-1.4%) 12-year-old patient developed atrial fibrillation 5 years after the procedure. Pregnancies were uneventful in 72 cases. When a balloon sizing is performed, 2D-TTE imaging is as efficient as 2D-TEE to guide percutaneous ASD closure in children. The procedure can safely be done in spontaneously breathing children under TTE guidance alone in experienced centers.

  5. Feasibility and safety of transthoracic echocardiography-guided transcatheter closure of atrial septal defects with deficient superior-anterior rims.

    Science.gov (United States)

    Li, Gui-Shuang; Li, Hai-De; Yang, Jie; Zhang, Wen-Quan; Hou, Zong-Shen; Li, Qing-Chen; Zhang, Yun

    2012-01-01

    Although previous studies showed that transthoracic echocardiography (TTE) can be used to guide transcatheter closure of atrial septal defect (ASD), whether TTE can be used to guide transcatheter closure of secundum ASD with a deficient superior-anterior rim is unknown and this critical issue was addressed in the present study. A total of 280 patients with secundum ASD who underwent transcatheter ASD closure were recruited and divided into groups A and B depending on ASD superior-anterior rim>4 mm (n = 118) or ≤4 mm (n = 162). TTE was used to guide Amplatzer-type septal occluder (ASO) positioning and assess residual shunt. Procedure success was defined as no, trivial and small residual shunt immediately after the procedure as assessed by color Doppler flow imaging. Group A and group B did not differ in complication rate (8.55% vs.7.55%), procedure success rate (98.3% vs. 95.0%) or complete closure rate immediately after the procedure (89.7% vs. 89.3%) or at 6-month follow-up (98.3% vs. 96.8%). The mean procedure and fluoroscopy time in group B were much longer than those in group A. In conclusion, the absence of a sufficient superior-anterior rim in patients undergoing percutaneous closure of secundum-type ASDs using fluoroscopic and TTE guidance is associated with slightly greater device malposition and migration as well as increased procedural and fluoroscopic times, but the overall complication rate did not differ with TTE guidance when compared to historical controls that used TEE guidance.

  6. Syndrome of extreme insulin resistance (Rabson-Mendenhall phenotype) with atrial septal defect: clinical presentation and treatment outcomes.

    Science.gov (United States)

    Dutta, Deep; Maisnam, Indira; Ghosh, Sujoy; Mukhopadhyay, Satinath; Chowdhury, Subhankar

    2013-01-01

    Syndrome of extreme insulin resistance (SEIR) is a rare spectrum disorder with a primary defect in insulin receptor signalling, noted primarily in children, and is often difficult to diagnose due to the clinical heterogeneity.SEIR was diagnosed in an adolescent girl with facial dysmorphism,exuberant scalp and body hair, severe acanthosis, lipoatrophy, dental abnormalities, and short stature (Rabson-Mendenhall phenotype). She had elevated fasting (422.95 pmol/L) and post-glucose insulin levels(>2083 pmol/L). Total body fat was decreased (11%; dual-energy X-ray absorptiometry). Basal growth hormone (GH) was increased (7.9 μg/L)with normal insuline-like growth factor 1 (37.6 nmol/L) suggestive of GH resistance. She had fatty liver and polycystic ovaries. Echocardiography revealed ostium secundum type atrial septal defect (ASD). Blood glucose normalized with pioglitazone (30 mg/day). Delayed development, severe insulin resistance, mild hyperglycemia, absence of ketosis, and remarkable response of hyperinsulinemia and hyperglycemia to pioglitazone which persisted even after 1 year of diagnosis are some of the notable features of this patient. This is perhaps the first report of occurrence of congenital heart disease (ASD) in a patient of SEIR (Rabson-Mendenhall phenotype). This report highlights the clinical features of SEIR and the role of insulin sensitizers like pioglitazone in the management of such patients.

  7. Automatic classification framework for ventricular septal defects: a pilot study on high-throughput mouse embryo cardiac phenotyping.

    Science.gov (United States)

    Xie, Zhongliu; Liang, Xi; Guo, Liucheng; Kitamoto, Asanobu; Tamura, Masaru; Shiroishi, Toshihiko; Gillies, Duncan

    2015-10-01

    Intensive international efforts are underway toward phenotyping the entire mouse genome by modifying all its [Formula: see text] genes one-by-one for comparative studies. A workload of this scale has triggered numerous studies harnessing image informatics for the identification of morphological defects. However, existing work in this line primarily rests on abnormality detection via structural volumetrics between wild-type and gene-modified mice, which generally fails when the pathology involves no severe volume changes, such as ventricular septal defects (VSDs) in the heart. Furthermore, in embryo cardiac phenotyping, the lack of relevant work in embryonic heart segmentation, the limited availability of public atlases, and the general requirement of manual labor for the actual phenotype classification after abnormality detection, along with other limitations, have collectively restricted existing practices from meeting the high-throughput demands. This study proposes, to the best of our knowledge, the first fully automatic VSD classification framework in mouse embryo imaging. Our approach leverages a combination of atlas-based segmentation and snake evolution techniques to derive the segmentation of heart ventricles, where VSD classification is achieved by checking whether the left and right ventricles border or overlap with each other. A pilot study has validated our approach at a proof-of-concept level and achieved a classification accuracy of 100% through a series of empirical experiments on a database of 15 images.

  8. Atrial septal defect in adults: echocardiography and cardiopulmonary exercise capacity associated with hemodynamics before and after surgical closure.

    Science.gov (United States)

    Suchon, Elzbieta; Tracz, Wieslawa; Podolec, Piotr; Sadowski, Jerzy

    2005-10-01

    The study aimed to evaluate pre and postoperative echocardiographic data and exercise capacity in relation to age and hemodynamics in adults with atrial septal defect (ASD). Fifty-two subjects with ASD (mean age: 38.6+/-15 years) were enrolled. Echocardiography and cardiopulmonary exercise test were performed before and a year after surgery. Pre and postoperative data were analyzed for the entire group and then compared in terms of age: or=40 years, right ventricular systolic pressure (RVSP): 30 mmHg and pulmonary to systemic flow ratio (Qp/Qs): or=2.5. After surgery right ventricle dimension decreased in all patients, although it remained significantly larger in patients over 40 years. There was a negative correlation between peak oxygen uptake and preoperative RVSP (r=-0.69, PExercise capacity improved irrespective of the age at surgery, preoperative RVSP and Qp/Qs, although it failed to normalize in patients with RVSP >30 mmHg. Adults with ASD benefit from surgical closure irrespective of the actual age at surgery. Patient's age at surgery and pulmonary hypertension crucially impact the results of surgical intervention. Early defect correction is therefore highly recommendable, specifically with a view to preventing the hemodynamic consequences of ASD.

  9. Can Computer Tomography Predict Compromise of Cardiac Structures After Percutaneous Closure of Interatrial Septal Defects?

    Science.gov (United States)

    Wagdi, Philipp

    2011-12-01

    Erosion of a cardiac structure after device closure of an interatrial septal communication (IASC-C), although rare, is a major and severe adverse event which may be underreported. On the other hand, unexplained episodes of transient chest pain occur more often and may be quite distressing. We sought to define the parameters relating the devices to the adjacent cardiac structures and to determine whether computer tomography (CT) could predict erosion of atrial or aortic wall or precordial pain symptoms occurring in the first months after device implantation. Retrospective observational study of 20 patients who underwent CT for de novo chest pain occurring after IASC-C or as a diagnostic test for suspected or proven coronary artery disease (CAD). Clinical follow up was for 20.5 ± 17.6 (6-84) months. CT was done 18 ± 10 (2-28) weeks after IASC-C. Indentation of the aortic root was found in 11 (55%) patients, the left atrial wall in 13 (65%) and the right atrial wall in eight (40%) of patients. Contact without indentation was found in nine (45%), 6 (30%) and 11 (55%) of patients respectively. Device indenting of the left and right atrial, as well as the aortic wall, occured in the majority of the patients examined after IASC-C. This finding may explain bouts of chest pain after the intervention in some patients, but does not predict clinically relevant erosion of a cardiac structure.

  10. Reversible complete atrioventricular block after percutaneous ASD device closure in a child <15 kg.

    Science.gov (United States)

    Rohit, Manoj Kumar; Puri, Kriti; Vadivelu, Ramalingam

    2014-01-01

    Transcatheter device closure of atrial septal defect (ASD) in small children less than 15 kg may be associated with increased complications. Complete atrioventricular heart block (CHB) is a rare complication of ASD device closure in such a setting. We report the case of a 2-year-old girl, less than 15 kg, who underwent device closure of ASD with Amplatzer Septal Occluder and subsequently developed CHB 12 h after the procedure which resolved completely with steroid treatment on fifth day. Case report of a similar kind is rarely reported in the literature. Despite adequate postero-inferior margin CHB may still occur in small children as in our case. Copyright © 2014 Cardiological Society of India. All rights reserved.

  11. Cardiac remodeling and effects on exercise capacity after interventional closure of atrial septal defects in different adult age groups.

    Science.gov (United States)

    Jategaonkar, Smita; Scholtz, Werner; Schmidt, Henning; Fassbender, Dieter; Horstkotte, Dieter

    2010-03-01

    Interventional closure of atrial septal defects (ASD) has become a standard procedure in pediatric and adult patients. We report immediate and mid-term results in different adult age groups. A retrospective analysis of 332 patients undergoing percutaneous ASD closure between 1998 and 2008 was performed. Beside echocardiographic and hemodynamic measurements, the NYHA functional class was assessed before and after ASD closure. The peak oxygen uptake (VO(2peak)) was available in a subgroup of 154 patients. The different age groups did not differ significantly in shunt volume or defect diameter, but a significant increase could be detected in mean pulmonary arterial pressure, mean left atrial pressure and pulmonary vascular resistance (PVR9 with increasing age). Right ventricular enlargement was present in all age groups, but the degree of enlargement increased with age. Transcatheter ASD closure was successful in 99.4%, major adverse events occurred in four patients (1.2%). At 3 months from closure, 123 patients reported an improvement, 7 of worsening and 163 of no change in their functional capacity. A significant increase in VO(2peak) could be registered in all subgroups after ASD closure. When classified by shunt volume no change could be detected in VO(2peak) in the patients with a Q(p):Q(s) 2 had a highly significant VO(2peak) increase. Patients benefit from interventional closure of hemodynamically significant ASD regardless of their age. However, the defect should be repaired as early as possible to prevent hemodynamic complications, such as the development of pulmonary hypertension and cardiac arrhythmias.

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

    Science.gov (United States)

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

    2013-01-01

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

  13. Pulmonary atresia and ventricular septal defect with collaterals to right lung associated with anomalous left pulmonary artery from the ascending aorta

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    Khositseth, Anant [Mahidol University, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Bangkok (Thailand); Siripornpitak, Suvipaporn; Pornkul, Ratanaporn [Mahidol University, Department of Radiology, Ramathibodi Hospital, Bangkok (Thailand)

    2010-12-15

    We present a 10-month-old boy with cyanosis. This is a rare case of pulmonary atresia, ventricular septal defect (VSD), major aorto-pulmonary collateral arteries (MAPCAs) to the right lung with absent native right pulmonary artery (RPA) in association with anomalous left pulmonary artery (LPA) from the ascending aorta (AAo). Echocardiography was unable to identify all of the cardiovascular abnormalities. Multidetector CT demonstrated all of these abnormalities and is the investigation of choice instead of cardiac catheterization. (orig.)

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

    Directory of Open Access Journals (Sweden)

    Fei-Feng Li

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

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

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    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.

  16. Important ECG diagnosis-aiding indices of ventricular septal defect children with or without congestive heart failure.

    Science.gov (United States)

    Guo, M; Huang, M N; Bai, Z; Hsieh, K S

    2001-04-15

    In this paper we perform a statistical study of the conventional RR intervals and two newly defined PR' and RT intervals of ECG data. A quadratic classification rule is applied to extract several important ECG diagnosis-aiding indices among normal children and children with ventricular septal defect (VSD) with or without congestive heart failure (CHF). The results show that certain statistics computed from PR', RR and RT intervals are important diagnosis-aiding indices. Best classification vectors are searched for pairwise classification. Two methods, minimum distance criterion and a two-stage classification procedure, are considered for three-way classification. Furthermore, logistic regression models based on transformations of these important diagnosis-aiding indices are proposed. The receiver operating characteristic curves of the proposed models show better performance than those of linear and quadratic logistic models. In order to proceed with this study, a computer algorithm to automatically detect the three intervals is developed and the related ECG data are collected and analysed. The algorithm is also enhanced with an outlier detection procedure for the automatic measurements of the PR' and RT intervals. Copyright 2001 John Wiley & Sons, Ltd.

  17. Feasibility, Safety and Long-Term Follow-Up of Transcatheter Closure of Secundum Atrial Septal Defects with Deficient Rims.

    Science.gov (United States)

    Cao, Chunhui; Wang, Zhonghua; Huang, Jun; Fan, Lingxia; Li, Ren; Wang, Shushui; Li, Yufen; Zhang, Zhiwei

    2016-01-01

    The aim of this work was to evaluate the feasibility and safety of transcatheter closure procedures for the treatment of atrial septal defects (ASDs) with insufficient rims. A total of 507 secondary ASDs were divided into two groups based on whether they had deficient rims or not (152 vs. 355 cases, respectively). Any complications, including residual shunt, heart arrhythmia, occluder translocation, etc., were followed up for 1-3 years. There were no differences in gender, weight, exposure time, ECG states, pulmonary pressure, the intervention success rate, occurrence of residual shunt, the operation time and occurrence of residual shunt during follow-up between the two groups (p > 0.05). However, the occurrence of rhythm disorders was significantly different between the two groups; ASDs with deficient rims were at an elevated risk (p rims group at 24 h postoperation, but no differences in arrhythmia incidence at any of the other follow-up time points (1, 3, 6, 12 and 36 months; p > 0.05). Patients with deficient rims experience a high success rate of ASD intervention and low rate of complications when the procedures are performed by experienced operators. © 2016 S. Karger AG, Basel.

  18. Radiofrequency-assisted "reconstruction" of the right ventricular outflow tract in muscular pulmonary atresia with ventricular septal defect.

    Science.gov (United States)

    Hausdorf, G; Schulze-Neick, I; Lange, P E

    1993-04-01

    A case of pulmonary atresia with ventricular septal defect is reported in which a communication was established between the right ventricle and the hypoplastic pulmonary artery by intervention, despite muscular atresia of the right ventricular outflow tract. The atresia was perforated with a special designed radiofrequency catheter (Osypka). After the creation of a canal within the muscular atresia, balloon dilatation (diameters 2, 3.5, and 7.2 mm) was performed. Arterial oxygen saturation increased from 64% to 78%. Lateral deviation of the radiofrequency catheter resulting in a lateral perforation of the atretic muscular infundibulum was well tolerated without later sequelae. Early restenosis within two weeks necessitated the implantation of a stent within the "recanalised" atresia resulting in an increased anterograde flow to the pulmonary artery. This case shows that "recanalisation" of muscular atresia of the pulmonary artery by radiofrequency is a promising technique. Additionally, stent implantation into the infundibulum to prevent restenosis is a first step to interventional right ventricular outflow tract reconstruction.

  19. Right Ventricular Outflow Tract Velocity Time Integral Determination in 570 Healthy Children and in 52 Pediatric Atrial Septal Defect Patients.

    Science.gov (United States)

    Koestenberger, Martin; Nage, Bert; Ravekes, William; Avian, Alexander; Burmas, Ante; Grangl, Gernot; Cvirn, Gerhard; Gamillscheg, Andreas

    2015-08-01

    Determination of the right ventricular outflow tract velocity time integral (RVOT VTI) is an important part of the noninvasive investigation of pulmonary blood flow in adults; however, age-related pediatric reference data are lacking. We examined growth-related changes of RVOT VTI values in children and the predictive value of RVOT VTI values in identifying enhanced pulmonary blood flow in children with secundum type atrial septal defect (ASD). A prospective study was conducted in a group of 570 healthy children and 52 children with a moderate-sized to large ASD. We determined the effects of age, body length (BL), body weight (BW), and body surface area (BSA) on RVOT VTI values. The predictive value of normal values stratified for age, BW, BL, and BSA was tested in our 52 ASD children. RVOT VTI values ranged from mean 9.7 ± 1.2 cm in neonates to 23.3 ± 2.7 cm in children with 18 years of age and showed a positive correlation with age, BL, BSA, and BW. In our population, RVOT VTI z-scores showed a high specificity for detecting ASD patients (>97 %) with sensitivity up to 71 %. We provide normal ranges and calculated z-scores of pediatric RVOT VTI values. Normal RVOT VTI z-scores might be additional predictors in identifying increased pulmonary blood flow in patients with ASD.

  20. Cardiac alpha-myosin (MYH6 is the predominant sarcomeric disease gene for familial atrial septal defects.

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    Maximilian G Posch

    Full Text Available Secundum-type atrial septal defects (ASDII account for approximately 10% of all congenital heart defects (CHD and are associated with a familial risk. Mutations in transcription factors represent a genetic source for ASDII. Yet, little is known about the role of mutations in sarcomeric genes in ASDII etiology. To assess the role of sarcomeric genes in patients with inherited ASDII, we analyzed 13 sarcomeric genes (MYH7, MYBPC3, TNNT2, TCAP, TNNI3, MYH6, TPM1, MYL2, CSRP3, ACTC1, MYL3, TNNC1, and TTN kinase region in 31 patients with familial ASDII using array-based resequencing. Genotyping of family relatives and control subjects as well as structural and homology analyses were used to evaluate the pathogenic impact of novel non-synonymous gene variants. Three novel missense mutations were found in the MYH6 gene encoding alpha-myosin heavy chain (R17H, C539R, and K543R. These mutations co-segregated with CHD in the families and were absent in 370 control alleles. Interestingly, all three MYH6 mutations are located in a highly conserved region of the alpha-myosin motor domain, which is involved in myosin-actin interaction. In addition, the cardiomyopathy related MYH6-A1004S and the MYBPC3-A833T mutations were also found in one and two unrelated subjects with ASDII, respectively. No mutations were found in the 11 other sarcomeric genes analyzed. The study indicates that sarcomeric gene mutations may represent a so far underestimated genetic source for familial recurrence of ASDII. In particular, perturbations in the MYH6 head domain seem to play a major role in the genetic origin of familial ASDII.

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

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    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

  2. Imaging atrial septal defects by real-time three-dimensional transesophageal echocardiography: step-by-step approach.

    Science.gov (United States)

    Saric, Muhamed; Perk, Gila; Purgess, Jan R; Kronzon, Itzhak

    2010-11-01

    There are currently no standardized three-dimensional (3D) transesophageal echocardiographic (TEE) views of the interatrial septum and atrial septal defects (ASDs). Without a standardized approach, it is difficult to ascertain the important anatomic relationships (such as the location of the aortic rim of an ASD), to perform relevant measurements (such as the size of an ASD or the size of its rims), or to guide the deployment of catheters and devices during atrial septal closure. Using a 3D TEE matrix-array transducer, 706 TEE studies were performed over a 14-month period. The purpose of the study was to develop a standardized protocol for anatomically correct orientation of 3D TEE images of the interatrial septum and ASDs. Among 706 TEE studies, there were 23 patients with ASDs, representing 3.3% of the study population. Eighteen patients had secundum ASDs, two had primum ASDs, and three had sinus venosus ASDs of the superior vena cava type. A protocol for properly orienting 3D TEE images of the interatrial septum and ASDs was developed. When the images are acquired at an angle of 0°, the septum is properly oriented by the tilt-up-then-left maneuver. The initial 3D TEE image in first tilted up to reveal the right atrial side of the septum. Then the image is tilted 180° around its vertical axis to reveal the left atrial side of the septum; the aortic rim is on the left, the superior vena cava on the top, and the right-sided pulmonary vein ostia on the right side of the screen. For acquisitions at a higher angle, the rotate-left-in-z-axis maneuver is used. The image is first tilted up to reveal the right atrial side of the septum, as in the tilt-up-then-left maneuver. The image is then rotated counterclockwise in the z axis until the superior vena cave is at 12 o'clock. Finally, the image is tilted 180° around its vertical axis to reveal the left atrial side of the septum. The use of standardized tilt-up-then-left and rotate-left-in-z-axis maneuvers enhances the

  3. [Atrioventricular canal defect, single atrium and tricuspid atresia as part of a case of Ellis-Van Creveld syndrome].

    Science.gov (United States)

    Gonzales Portillo, Sara Nila; Conde Sumire, Rosa; Gamio Vega Centeno, Fernando; Hernández-Córdova, Gustavo; Romaní Romaní, Franco

    2013-06-01

    Ellis-Van Creveld Syndrome or chondrectodermal dysplasia is produced by an autosomal recessive inheritance secondary to mutation in the short arm of chromosome 4. The syndrome affects multiple organs. It is described as a clinical tetrad that involves chondrodysplasia, ectodermal dysplasia, polydactyly and congenital heart defects. It is only known from reports and case series. We present a three months old male, without relevant family history, who presented chondrodysplasia, upper lip merged to palate, bilateral sinpolydactyly in the hands, developmental dysplasia of the hip, narrow chest with short ribs, and heart defects. This case is the first report of EVC in Peruvian literature.

  4. Robotic-assisted closure of atrial septal defect under real-time three-dimensional echo guide: in vitro study.

    Science.gov (United States)

    Suematsu, Yoshihiro; Kiaii, Bob; Bainbridge, Daniel T; del Nido, Pedro J; Novick, Richard J

    2007-10-01

    Several advances in robotic technology and imaging systems have enabled the broad application of minimally invasive techniques in cardiac surgery. We have previously demonstrated that real-time three-dimensional echocardiography (RT3DE) provided adequate imaging and anatomic detail to act as a sole guide for surgical task performance. In this study, we examined the feasibility of robotic-assisted RT3DE-guided repair of atrial septal defect (ASD) in an in vitro study. Exp. I: An RT3DE system with x4 matrix transducer (Sonos 7500, Philips Medical Systems, Andover, MA) was compared to two-dimensional echo (2DE) in the performance of common surgical tasks with the da Vinci Robotic Surgical System (Intuitive Surgical, Sunnyvale, CA). Completion times and deviation of suture from an echogenic target (mm) were measured. Exp. II: Porcine ASDs (n=10) were created and closed with robotic-assisted direct suturing in a water bath. During all experiments the operator was blinded to the target and operated only under ultrasonic guidance. Compared to 2DE guidance, completion times improved by 70% (probotic system was significantly smaller (2DE: 4+/-2mm, 3DE: 0.2+/-0.3mm, p=0.0002) in RT3DE-guided tasks. RT3DE provided satisfactory images and sufficient anatomical detail for suturing. All surgical tasks were successfully performed with accuracy. These initial experiments demonstrate the feasibility of robotic-assisted direct closure of ASD under RT3DE guidance. An endoscopic port access approach may be possible with refinements in telemanipulator technology and further development of the transesophageal echo transducer.

  5. Does surgically induced right bundle branch block really effect ventricular function in children after ventricular septal defect closure?

    Science.gov (United States)

    Karadeniz, Cem; Atalay, Semra; Demir, Fikri; Tutar, Ercan; Ciftci, Omer; Ucar, Tayfun; Uysalel, Adnan; Eyileten, Zeynep

    2015-03-01

    In this prospective study, we aimed to assess left and right ventricular function in terms of the presence of right bundle branch block (RBBB) in the cases with repaired ventricular septal defect (VSD). Fifty-three patients who had VSD surgery at least 1-year preceding admission and 52 healthy controls were enrolled into the study. All the participants underwent electrocardiographic and echocardiographic examination. The cases with RBBB were determined. The conventional and tissue Doppler echocardiographic measurements of the patients with and without RBBB were compared with each other and healthy controls. Twenty-eight of VSD repair groups were male and 25 were female. Control group consisted of 30 males and 22 females. The mean age of the study and control groups was 7.5 ± 5.0 and 6.9 ± 4.3 years, respectively. RBBB was detected in 20 of 53 (37.7 %) operated patients. The only significant difference between the cases with and without RBBB was decreased right ventricular fractional area change (%) in the former group (33 ± 7 vs. 39 ± 5 p < 0.05). When compared to controls, operated group had statistically lower [corrected] tricuspid annular plane systolic excursion (p < 0.05), lower systolic, early diastolic, and late diastolic myocardial velocities, higher left and right ventricular myocardial performance indices, irrespective of the presence of RBBB. The ratios of mitral or tricuspid inflow to left or right ventricular myocardial in early diastolic velocities measured from lateral annular levels were increased in operated group (all p values <0.05). In conclusion, RBBB in the cases with surgical VSD repair might be associated with right ventricular dysfunction. Biventricular systolic and diastolic dysfunction may develop following VSD repair irrespective of the presence of RBBB. Tissue Doppler-derived myocardial performance indices are useful in detection of those subclinical dysfunctions.

  6. Clinical comparison of robotic minimally invasive surgery and transcatheter interventional occlusion for adult secundum atrial septal defect

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    Cheng WANG

    2016-06-01

    Full Text Available Objective  To assess the safety and efficiency of robotic minimally invasive surgery and transcatheter interventional occlusion for treatment of adult secundum atrial septal defect (ASD by comparing the early and recent postoperative follow-up results of the two minimally invasive surgery. Methods  Thirty adult patients with secundum ASD, who admitted to the General Hospital of PLA from Jan. 2008 to Dec. 2014 and received treatment of da Vinci Surgical System, were recruited as TEASD-R group, meanwhile, another 30 adult patients who received transcatheter interventional occlusion were recruited under the strict 1:1 criterion as TIASD-O group. The early postoperative complications, in-hospital conditions, recent postoperative follow-up results and the quality of life 30d and 6 months after operation were compared and retrospectively analyzed between the two groups. Results  The success rates of surgery were 100% in the both groups, no early and recent postoperative complications (residual shunt, pericardial effusion, cerebral infarction, peripheral vascular embolism, new arrhythmia, etc. were found in TEASD-R group. While some of corresponding complications existed in TISAD-O group, and the differences were of statistical significance (P<0.05 between the two groups in the incidence of postoperative new arrhythmia, tricuspid incompetence and pulmonary hypertension, as well as in the early size of right atrium and in-hospital time. SF-36 quality of life questionnaire showed that the difference of somatic pain 30d after operation was of statistical significance (P<0.05 between the two groups, but the difference disappeared 6 months after operation. Conclusion  Robotic minimally invasive surgery for adult secundum ASD is feasible, safe and efficacious since no postoperative complications occurred such as tricuspid incompetence and pulmonary hypertension, but the longer operative and inhosptial time are the shortages of the operation. DOI: 10

  7. Intramural Ventricular Septal Defect is a Distinct Clinical Entity Associated with Postoperative Morbidity in Children after Repair of Conotruncal Anomalies

    Science.gov (United States)

    Patel, Jyoti K; Glatz, Andrew C; Ghosh, Reena M; Jones, Shannon M; Natarajan, Shobha; Ravishankar, Chitra; Mascio, Christopher E; Spray, Thomas L; Cohen, Meryl S

    2015-01-01

    Background Intramural ventricular septal defects (VSDs) are interventricular communications through right ventricular free wall trabeculations that can occur after repair of conotruncal anomalies. We assessed the prevalence of residual intramural VSDs and their effect on postoperative course. Methods and Results Children who underwent biventricular repair of a conotruncal anomaly from 1/1/06 to 6/30/13 and had a post-operative transthoracic echocardiogram were included. Images were reviewed for residual intramural or non-intramural VSDs. The primary outcome was a composite of mortality, extracorporeal membrane oxygenation (ECMO) use, and need for subsequent catheter or surgical VSD closure. The secondary outcome was post-operative hospital length of stay (PLOS). A residual VSD was present in 256 of the 442 subjects (58%), of which 231 (90%) were <2mm in size. Forty-nine (11%) had intramural VSDs and 207 (47%) had non-intramural VSDs. Patients with intramural VSDs were more likely to reach the primary composite outcome compared to those with non-intramural VSDs or no residual VSD (14/49 [29%] vs 15/207 [7%] vs 6/186 [3%], p<0.0001). In addition, those with intramural VSDs had longer PLOS compared to those with non-intramural VSDs or no residual VSD (20 days [IQR 11-42] vs 7 days [5-14] vs 6 [4-11], p=0.0001). These associations remained significant after adjusting for known risk factors for poor outcomes, including residual VSD size and operative complexity. Conclusions Among residual VSDs after repair of conotruncal anomalies, intramural VSDs are uniquely associated with postoperative morbidity, mortality, and longer PLOS. It is important to recognize intramural VSDs in the postoperative period. PMID:26246174

  8. Echocardiographic Reference Values for Right Atrial Size in Children with and without Atrial Septal Defects or Pulmonary Hypertension.

    Science.gov (United States)

    Koestenberger, Martin; Burmas, Ante; Ravekes, William; Avian, Alexander; Gamillscheg, Andreas; Grangl, Gernot; Grillitsch, Marlene; Hansmann, Georg

    2016-04-01

    Right atrial (RA) size may become a very useful, easily obtainable, echocardiographic variable in patients with congenital heart disease (CHD) with right-heart dysfunction; however, according studies in children are lacking. We investigated growth-related changes of RA dimensions in healthy children. Moreover, we determined the predictive value of RA variables in both children with secundum atrial septal defect (ASD) and children with pulmonary hypertension (PH) secondary to CHD (PH-CHD). This is a prospective study in 516 healthy children, in 80 children with a secundum ASD (>7 mm superior-inferior dimension), and in 42 children with PH-CHD. We determined three RA variables, i.e., end-systolic major-axis length, end-systolic minor-axis length, and end-systolic area, stratified by age, body weight, length, and surface area. RA end-systolic length and area z scores were increased in children with ASD and PH-CHD when compared to those variables in the healthy control population. Using the Youden Index to determine the best cutoff scores in sex- and age-specific RA dimensions, we observed a sensitivity and specificity up to 94 and 91 %, respectively, in ASD children and 98 and 94 %, respectively, in PH-CHD children. We provide normal values (z scores -2 to +2) for RA size and area in a representative, large pediatric cohort. Enlarged RA variables with scores >+2 were predictive of secundum ASD and PH-CHD. Two-dimensional determination of RA size can identify enlarged RAs in the setting of high volume load (ASD) or pressure load (PH-CHD).

  9. Atrial septal defect

    Science.gov (United States)

    ... years old) ECG Heart MRI Transesophageal echocardiography (TEE) Treatment ASD may not need treatment if there are few or no symptoms, or ... often not cause problems, or will close without treatment. Larger ASDs (8 to 10 mm), often do not close ...

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

    Science.gov (United States)

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

    2016-08-24

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

  11. A case of an elderly man who required repeated repair of a ventricular septal defect and tricuspid rupture after blunt chest trauma.

    Science.gov (United States)

    Matsuyama, Shigefumi; Imazuru, Tomohiro; Nakagawa, Kaori; Ikeda, Tsukasa; Harada, Tadanori; Ota, Hiroo; Ozawa, Naomi; Iida, Mitsuru; Shimokawa, Tomoki

    2017-11-25

    Several cases of traumatic ventricular septal defect (VSD) have been reported. However, traumatic VSD complicated by tricuspid rupture is rare. We report a case of traumatic VSD with tricuspid rupture who required repeated repair of both conditions. A 69-year-old man was transferred to our hospital for emergent surgical repair of traumatic VSD and tricuspid rupture. Although emergent repair was performed, a new left-to-right shunt and moderate tricuspid regurgitation appeared during his postoperative course. A reoperation was performed 4 months after the first operation. The borders of the defect were very fibrotic and strong compared with those in the first operation. Surgical treatment of traumatic VSD should be postponed in hemodynamically stable patients. When emergent repair is performed, careful follow-up is necessary to diagnose new VSD.

  12. Congenital Complete Atrioventricular Block : Clinical and Experimental Studies

    NARCIS (Netherlands)

    Blank, A.C.

    2014-01-01

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

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

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    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.

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

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    Eiki Tayama

    2012-01-01

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

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

    Science.gov (United States)

    Tayama, Eiki; Fujita, Satoshi; Ueda, Tomohiro; Imasaka, Ken-Ich; Enomoto, Naofumi; Onitsuka, Hirofumi; Tomita, Yukihiro

    2012-01-01

    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.

  16. Cardiac Resynchronization for Corrected Transposition of the Great Arteries with Systemic Right Ventricle Failure after Tricuspid Valve Replacement and Ventricle Septal Defect Closure

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    Kosuke Fujii, MD

    2010-01-01

    Full Text Available A 32-year-old man developed systemic right ventricular (RV heart failure after ventricular septal defect (VSD closure and tricuspid valve replacement for corrected transposition of the great arteries with VSD and Ebstein anomaly. He subsequently experienced RV failure with wide QRS and atrial fibrillation (AF. Because corrective surgery for this condition seemed over risky, we decided to perform cardiac resynchronization therapy with implantation of an implantable cardioverter defibrillator (CRT-D. After CRT-D device implantation, the patient showed improved performance status in terms of New York Heart Association functional class, B-type brain natriuretic peptide levels, RV ejection fraction and cardiac electrical rhythm. CRT-D implantation is a useful approach for systemic RV failure with wide QRS duration showing right bundle branch block and AF.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    Nezafati, Pouya; Nezafati, Mohammad Hassan; Hoseinikhah, Hamid

    2015-01-01

    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.

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

    Science.gov (United States)

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

    2010-02-15

    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 approximately 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.

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

    Science.gov (United States)

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

    2010-01-01

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

  1. An inferior sinus venosus interatrial communication associated with a secundum atrial septal defect, clinically presenting in an adult patient: autopsy report

    Directory of Open Access Journals (Sweden)

    Silvana Maria Lovisolo

    2011-12-01

    Full Text Available Atrial septal defects (ASD are the most common congenital cardiac lesionin adults, representing up to 40% of acyanotic shunt lesions in patients olderthan 40 years. Secundum ASD comprises defects in the area of the ovalfossa, and may be associated with other types of interatrial communications.We present a case of a 25 year-old female patient who was asymptomaticuntil the age 22 when she started presenting exertion dyspnea and lowerlimbs edema, during her third pregnancy. The patient was admitted in theemergency department because of a febrile respiratory distress syndromedying in less than 36 hours after her arrival. The autopsy examination revealedpneumonia and a secundum ASD, associated with a rare form of interatrialcommunication, the inferior sinus venosus defect, which is characterized byan anomalous connection of the right pulmonary veins to the inferior caval veinat its opening in the right atrial cavity, while retaining partially their connectionto the left atrium. A dysplastic and thickened mitral valve was also present.Signs of pulmonary hypertension confirm the Eisenmenger syndrome in thisadult patient.

  2. Proximal isovelocity surface area (PISA) as a noninvasive method for the estimation of the shunt quantification in perimembranous ventricular septal defects.

    Science.gov (United States)

    Eren, M; Dagdeviren, B; Bolca, O; Polat, M; Gürlertop, Y; Norgaz, T; Tezel, T

    2001-02-01

    This study was designed to assess the reliability of the proximal isovelocity surface area (PISA) method for the estimation of shunt quantification in perimembranous ventricular septal defects (PVSD). The study group was composed of 30 patients (age 11 +/- 7 years, 13 female) with PVSD. The shunt flow (Qp-Qs) and the ratio of the pulmonary flow to the systemic flow (Qp/Qs) were calculated by spectral Doppler and catheterization. The Qp-Qs, the defect area (DA), and the shunt volume (SV) were obtained by the PISA method. The PISA method estimated the DA (cm(2)/m(2)), the SV (cm(3)/m(2)), and the Qp-Qs (L/min/m(2)) to be equal to (2 x pi x R(2) x NL)/(V(max) x Body surface area), DA x TVI(shunt), and to SV x Heart rate, respectively (R is the distance of the maximal PISA from the first aliasing line to the left ventricular side of the defect, NL is the nyquist limit, and V(max) and TVI(shunt) are the peak velocity and time-velocity integral of transdefect Doppler tracing obtained by continuous-wave Doppler). The PISA method (3.4 +/- 1.5 L/min/m(2)) underestimated the Qp-Qs according to spectral Doppler (r = 0.96, P PISA findings (Qp-Qs, DA, SV) and the catheterization Qp/Qs (r = 0.86, 0.84, and 0.86; P PISA findings in identifying large defects were high (0.90, 0.93, and 0.90 for cut-off values of Qp-Qs = 3.67 L/min/m(2), DA = 0.44 cm(2)/m(2), and SV = 43 cm(3)/m(2), respectively). As a result, the PISA method can be a simple and reliable alternative to the spectral Doppler method in the identification of large shunts in PVSD.

  3. Atrial flutter in a patient with atrial septal defect and anomalous venous drainage: unusual approach for ablation.

    Science.gov (United States)

    Roca-Luque, Ivo; Rivas, Nuria; Dos, Laura; Francisco, Jaume; Pérez-Rodon, Jordi; Pijuan, Antònia; Garcia-Dorado, David; Moya, Àngel

    2017-07-01

    Atrial flutter ablation in CHD (Congenital Heart Disease) patients is a challenging procedure because of the possibility of multiple circuits. Electroanatomical mapping and pacing maneuvers are crucial to determine critical isthmus. Moreover, vascular abnormalities and residual cardiac defects need to be known before the ablation to decide the better strategy for ablation.

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

    Science.gov (United States)

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

    2017-02-01

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

  5. Surgical technique of double switch procedure: Senning with arterial switch operation for congenitally corrected transposition of the great arteries with ventricular septal defect.

    Science.gov (United States)

    Ilin, Alexey S; Teplov, Pavel V; Sakovich, Valeriy A; Ohye, Richard G

    2016-01-01

    We present a case of 12-month-old boy with congenitally corrected transposition of great arteries with L-looped ventricles and L-transposition of great arteries and ventricular septal defect. When admitted to the hospital, the patient had the appearance of congestive heart failure due to moderate to severe tricuspid valve regurgitation and right ventricle dysfunction. The pulmonary artery (PA) banding was required first because of low systolic pressure in the morphological left ventricle less than 70% confirmed by catheterization. Three months later, the patient appeared to be a good candidate for anatomical repair and a double switch procedure-Senning with arterial switch-was performed. The early postoperative period was relatively smooth and uneventful. Tricuspid valve insufficiency was resolved immediately after surgery. Mild systolic dysfunction of the left ventricle with mild mitral insufficiency was confirmed by the 2D strain method of echocardiography on the second day of the postoperative period and it improved over the next 21 days. Thirty days later after the procedure, the patient underwent catheterization of his superior vena cava tunnel because of the slightly increased blood flow velocity diagnosed by echocardiography. In 3 months after the surgery, the boy was asymptomatic and was doing well. The patient's functional status was I according to the NYHA classification. © The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  6. Ventricular septal defect in infants and children with increased pulmonary vascular resistance and pulmonary hypertension--surgical management: leaving an atrial level communication.

    Science.gov (United States)

    Khan, Inam Ullah; Ahmed, Iftikhar; Mufti, Waqar A; Rashid, Azhar; Khan, Asif Ali; Ahmed, Syed Afzal; Imran, Muhammad

    2006-01-01

    To evaluate the surgical and medical efficacy of the patients operated for Ventricular Septal Defect (VSD) with Pulmonary Hypertension and Pulmonary Vascular Resistance (PVR). Infants and children with elevated PVR and Pulmonary Hypertension are associated with significant mortality and morbidity after surgical closure. Circulatory assist devices and sophisticated medicines may not be available to help in the management of infants and children with elevated Pulmonary artery pressure and resistance. We left Patent Foramen Ovale (PFO) or made atrial communication to decrease the morbidity and mortality associated with the closure of large VSD in this risky group. Sixteen infants and children were operated with median age of 12 months, operated by the same surgeon (IU), from January' 2004 to December' 2005. They were with large VSD of elevated PVR (3.9+0.3) and underwent VSD closure leaving PFO or artificial ASD (5mm). Surgical approach was through right atrium. Post operatively, all the patients were electively ventilated for 36 hours. They were given intravenous dilators (Glyceral Trinitrate + Phentolamine) and oral Sildenafil up to 1 mg /Kg, six hourly. Five cases went into acute pulmonary hypertensive crisis postoperatively, and were rescued by Prostacycline Nebulization. Sixteen patients had VSD as the primary lesion that underwent operation. The overall early mortality was 6.25% (1/16). There have been no late deaths. Closure of large VSD with elevated PVR can be performed, leaving PFO or artificial ASD, with acceptable mortality and morbidity.

  7. Right Ventricular Outflow Tract (RVOT) Changes in Children with an Atrial Septal Defect: Focus on RVOT Velocity Time Integral, RVOT Diameter, and RVOT Systolic Excursion.

    Science.gov (United States)

    Koestenberger, Martin; Ravekes, William; Avian, Alexander; Grangl, Gernot; Burmas, Ante; Raith, Wolfgang; Cvirn, Gerhard; Grillitsch, Marlene; Gamillscheg, Andreas

    2016-09-01

    Aim of the study was to determine the influence of right heart volume overload in children with atrial septal defect (ASD) on right ventricular outflow tract (RVOT) variables. A prospective study was conducted in 115 children (age range: 2 days-18.1 years) with a moderate to large ASD. We determined effects of age, body length (BL), body weight (BW), and body surface area (BSA) on the variables RVOT diameter, RVOT velocity time integral (VTI), and RVOT systolic excursion (SE), and tested the predictive value of published normal values for age, BW, BL, and BSA in our ASD patients. In our pediatric ASD patients, the age-specific RVOT diameter (z-score: +2.2, 95% CI: 2.0-2.4, P 2.0. The age-specific RVOT VTI z-score (z-score: +3.6, 95% CI: 3.2-3.9, P 2.0. The age-specific RVOT SE z-score was not increased but slightly lower compared to normal values (z-score: -0.5, 95% CI: -0.7 to -0.3, P 2.0 while 12% of the patients had a z-score children with moderate to large ASD. © 2016, Wiley Periodicals, Inc.

  8. Transcatheter closure of perimembranous ventricular septal defect with the Amplatzer(®) membranous VSD occluder 2: initial world experience and one-year follow-up.

    Science.gov (United States)

    Tzikas, Apostolos; Ibrahim, Reda; Velasco-Sanchez, Daniel; Freixa, Xavier; Alburquenque, Marcela; Khairy, Paul; Bass, John L; Ramirez, Juan; Aguirre, Daniel; Miro, Joaquim

    2014-03-01

    To describe the initial world experience and mid-term follow-up of perimembranous ventricular septal defect (pmVSD) closure with a newly designed occluder. Transcatheter closure of pmVSDs has been associated with a substantial risk of complete heart block, prompting many centers to abandon this intervention. A prospective multicenter cohort study was conducted on patients with pmVSD undergoing catheter closure using the Amplatzer(®) Membranous VSD Occluder 2 in the initial 4 pilot centers. Nineteen patients, median age 6 years (range 1.4-62 years), were enrolled and followed for 14 ± 3 months (range 8-20 months). The median weight was 26 kg (range 9.3-96 kg) and the mean Qp/Qs ratio was 1.8 ± 0.7. The defect on left ventricular side measured 9.9 ± 3.5 mm and the orifice on right ventricular side was 8.1 ± 2.8 mm by echocardiography. Mean device size was 9.4 ± 2.4 mm (range 5-14 mm). An eccentric device was employed in 9 patients (47%) and a concentric device in 10 (53%). Overall, 18 patients (95%) had successful device implants. Procedural time was 122 ± 39 min. There were no procedural complications. Mild residual shunting was initially observed in 14 (78%) patients. At last follow-up, mild residual shunting persisted in only 3 (17%) patients. There was no significant increase in aortic or tricuspid regurgitation. No patient had any degree of AV block, although one developed a transient left anterior fascicular block. Holter evaluation, obtained in all patients, was unremarkable in all. This early cohort experience using a novel adapted transcatheter closure device for pmVSD suggests that the procedure is feasible, safe, and effective. Copyright © 2013 Wiley Periodicals, Inc.

  9. Septal rhinoscleroma

    Directory of Open Access Journals (Sweden)

    Shoeib Mohamed

    2010-01-01

    Full Text Available Rhinoscleroma is a chronic granulomatous condition of the nose and other structures of the upper respiratory tract. Infection by the bacterium Klebsiella rhinoscleromatis is said to be the cause. A female patient aged 45 years, presented with a past history of trauma to the nose and swelling on her nose since last 1 year. There was nasal asymmetry and internal nasal examination showed a septal swelling protruding to the right nasal cavity with hypertrophied nasal mucosa and inferior turbinate. Open tip rhinoplasty approach was used to excise the mass, which examined pathologically revealing a rhinoscleroma, fibrotic infiltrative stage.

  10. Atrioventricular valve abnormalities in infancy: two-dimensional echocardiographic and angiocardiographic comparison.

    Science.gov (United States)

    Gutgesell, H P; Cheatham, J; Latson, L A; Nihill, M R; Mullins, C E

    1983-09-01

    The results of two-dimensional echocardiography and biplane angiocardiography from 47 infants with congenital atrioventricular (AV) valve abnormalities were compared. Eleven patients had atresia of the right AV valve, 10 had atresia of the left AV valve, 4 had hypoplasia of the right AV valve and 5 had hypoplasia of the left AV valve. Twelve patients had endocardial cushion defect, three had single ventricle and two had straddling of the left AV valve. There was agreement between the two techniques as to the number of AV valves present in each patient. The echocardiographic estimate of valve anular diameter was below normal in seven of the eight patients thought to have a hypoplastic anulus by angiocardiography. In 10 of the 12 patients with endocardial cushion defect, there was agreement between the two techniques as to the presence or absence of atrial and ventricular septal defect. The chordal attachments of straddling valves were better visualized by echocardiography; flow patterns and effective orifice size were better demonstrated by angiocardiography. The subcostal four chamber echocardiographic views and cranially angulated oblique angiocardiographic views were comparable and provided the best images for determination of the size and number of AV valves and their relation to the atrial and ventricular septa.

  11. Prevalence of congenital heart defects associated with Down syndrome in Korea.

    Science.gov (United States)

    Kim, Min-A; Lee, You Sun; Yee, Nan Hee; Choi, Jeong Soo; Choi, Jung Yun; Seo, Kyung

    2014-11-01

    Congenital heart defect (CHD) is common in infants with Down syndrome (DS), which is the principle cause of mortality. However, there is no data available for the frequency and types of CHD in infants with DS in Korea. We investigated the frequency of CHD in infants with DS in Korea. After the survey on birth defects was conducted throughout the country, the prevalence of CHD in DS in 2005-2006 was calculated. This study was conducted based on the medical insurance claims database of the National Health Insurance Corporation. The number of total births in Korea was 888,263 in 2005-2006; of them, 25,975 cases of birth defects were identified. The prevalence of DS was 4.4 per 10,000 total births, accounting for 1.5% of all birth defects. Of the 394 infants with DS, 224 (56.9%) had a CHD. Atrial septal defect was the most common defect accounting for 30.5% of DS followed by ventricular septal defect (19.3%), patent duct arteriosus (17.5%), and atrioventricular septal defect (9.4%). Our study will be helpful to demonstrate the current status of DS and to identify the distribution of CHD in infants with DS in Korea.

  12. Achievements and Limitations of a Strategy of Rehabilitation of Native Pulmonary Vessels in Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries.

    Science.gov (United States)

    Soquet, Jerome; Liava'a, Matthew; Eastaugh, Lucas; Konstantinov, Igor E; Brink, Johann; Brizard, Christian P; d'Udekem, Yves

    2017-05-01

    A strategy of rehabilitation for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) comprises repetitive shunting and patching procedures of the central pulmonary arteries. We wanted to determine the feasibility and limitations of a strategy of rehabilitation. The outcomes of 37 consecutive patients operated from June 2003 to December 2014 for PA/VSD/MAPCAs were reviewed. The patients were directed to a rehabilitation strategy, except when they presented in heart failure with very large collaterals. Four patients with very large MAPCAs underwent a one-stage repair with unifocalization of collateral vessels at a median age of 8.6 months. There was no mortality in this group after a median follow-up of 4.6 years. Following a strategy of staged rehabilitation, 33 patients had 2.01 ± 0.9 procedures before repair. Median age at primary shunting was 3.3 weeks (0.4 to 31.9 weeks). Repair rate was 73% (22 patients), at a median age of 1.7 years. Three patients (10%) were left palliated and 3 patients (10%) died. Median follow-up in this group was 4.5 years. Complementary procedures to the rehabilitation strategy consisted in pulmonary artery reconstruction in 25 patients (76%) and MAPCAs ligation in 7 patients (21%). Pulmonary balloon angioplasty was required in 12 patients (36%) and MAPCAs coil occlusion in 8 patients (24%). A strategy of rehabilitation can be implemented in almost 90% of the cases, with a low mortality rate. Following this strategy, 73% of the patients can be successfully repaired. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

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

    Science.gov (United States)

    Kaiser, Eva; Jaganathan, Saravana Kumar; Supriyanto, Eko; Ayyar, Manikandan

    2017-07-01

    Atrial septal defect (ASD) constitutes 30-40% of all congenital heart diseases in adults. The most common complications in the treatment of ASD are embolization of the device and thrombosis formation. In this research, an occluding patch was developed for ASD treatment using a well-known textile technology called electrospinning. For the first time, a cardiovascular occluding patch was fabricated using medical grade polyurethane (PU) loaded with bioactive agents namely chitosan nanoparticles (Cn) and collagen (Co) which is then coated with heparin (Hp). Fourier transform infrared spectrum showed characteristic vibrations of several active constituents and changes in the absorbance due to the inclusion of active ingredients in the patch. The contact angle analysis demonstrated no significant decrease in contact angle compared to the control and the composite patches. The structure of the electrospun nanocomposite (PUCnCoHp) was examined through scanning electron microscopy. A decrease in nanofiber diameter between control PU and PUCnCoHp nanocomposite was observed. Water uptake was found to be decreased for the PUCnCoHp nanocomposite against the control. The hemocompatibility properties of the PUCnCoHp ASD occluding patch was inferred through in vitro hemocompatibility tests like activated partial thromboplastin time (APTT), prothrombin time (PT) and hemolysis assay. It was found that the PT and APTT time was significantly prolonged for the fabricated PUCnCoHp ASD occluding patch compared to the control. Likewise, the hemolysis percentage was also decreased for the PUCnCoHp ASD patch against the control. In conclusion, the developed PUCnCoHp patch demonstrates potential properties to be used for ASD occlusion.

  14. Intracardiac echo-guided radiofrequency catheter ablation of atrial fibrillation in patients with atrial septal defect or patent foramen ovale repair: a feasibility, safety, and efficacy study.

    Science.gov (United States)

    Lakkireddy, Dhanunjaya; Rangisetty, Umamahesh; Prasad, Subramanya; Verma, Atul; Biria, Mazda; Berenbom, Loren; Pimentel, Rhea; Emert, Martin; Rosamond, Thomas; Fahmy, Tamer; Patel, Dimpi; Di Biase, Luigi; Schweikert, Robert; Burkhardt, David; Natale, Andrea

    2008-11-01

    Intracardiac Echo-Guided Radiofrequency Catheter. Patients with atrial septal defect (ASD) are at higher risk for atrial fibrillation (AF) even after repair. Transseptal access in these patients is perceived to be difficult. We describe the feasibility, safety, and efficacy of pulmonary vein antral isolation (PVAI) in these patients. We prospectively compared post-ASD/patent foramen ovale (PFO) repair patients (group I, n = 45) with age-gender-AF type matched controls (group II, n = 45). All the patients underwent PVAI through a double transseptal puncture with a roving circular mapping catheter technique guided by intracardiac echocardiography (ICE). The short-term (3 months) and long-term (12 month) failure rates were assessed. In group I, 23 (51%) had percutaneous closure devices and 22 (49%) had a surgical closure. There was no significant difference between group I and II in the baseline characteristics. Intracardiac echo-guided double transseptal access was obtained in 98% of patients in group I and in 100% of patients in group II. PVAI was performed in all patients, with right atrial flutter ablation in 7 patients in group I and in 4 patients in group II. Over a mean follow-up of 15 +/- 4 months, group I had higher short-term (18% vs 13%, P = 0.77) and long-term recurrence (24% vs 18%, P = 0.6) than group II. There was no significant difference in the perioperative complications between the two groups. Echocardiography at 3 months showed interatrial communication in 2 patients in group I and 1 patient in group II, which resolved at 12 months. Percutaneous AF ablation using double transseptal access is feasible, safe, and efficacious in patients with ASD and PFO repairs.

  15. Promoting Pulmonary Arterial Growth via Right Ventricle-to-Pulmonary Artery Connection in Children With Pulmonary Atresia, Ventricular Septal Defect, and Hypoplastic Pulmonary Arteries.

    Science.gov (United States)

    Rabinowitz, Edon J; Epstein, Shilpi; Kohn, Nina; Meyer, David B

    2017-09-01

    Complete repair of pulmonary atresia (PA) ventricular septal defect (VSD) with hypoplastic or absent native pulmonary arteries, often with major aortopulmonary collateral arteries (MAPCAs), involves construction of an adequate sized pulmonary arterial tree. We report our results with a previously described staged strategy using initial right ventricle (RV)-to-reconstructed pulmonary arterial tree (RV-PA) connection to promote pulmonary arterial growth and facilitate later ventricular septation. We retrospectively reviewed data for all patients (N = 10) with initial echocardiographic diagnosis of PA-VSD and hypoplastic pulmonary arteries operated in our center from October 2008 to August 2016. Pulmonary arterial vessel size measured on preoperative and postoperative angiography was used to calculate Nakata index. Seven patients had PA-VSD, three had virtual PA-VSD, and seven had MAPCAs. All underwent creation of RV-PA connection at a median age of 7.5 days and weight 3.6 kg. Eight patients had RV-PA conduits, two had a transannular patches, and seven had major pulmonary artery reconstruction simultaneously. There were no deaths or serious morbidity; one conduit required revision prior to complete repair. Complete repair with ventricular septation and RV pressure less than half systemic was achieved in all patients at a median age of 239 days. Nakata index in neonatal period was 54 mm2/m2 (range 15-144 mm2/m2) and at time of septation 184 mm2/m2 (range 56-510 mm2/m2; P = .004). Growth rates of right and left branch pulmonary arteries were similar. The 10 patients underwent 28 catheterizations with 13 interventions in 8 patients prior to full repair. Early palliative RV-PA connection promotes pulmonary arterial growth and facilitates eventual full repair with VSD closure with low RV pressure and operative risk.

  16. [Classification of congenital ventricular defects using echocardiography for transcatheter closure].

    Science.gov (United States)

    Lai, Yu-qiong; Huang, Xin-sheng

    2009-02-01

    To determine the anatomical variation and classification of ventricular septal defect (VSD) using echocardiography for percutaneous catheter closure in eligible cases. The isolated ventricular septal defect was diagnosed with echocardiography in 240 patients , and 167 patients screened by transthoracic echocardiography were suitable for percutaneous catheter closure, but only 62 with isolated perimembranous VSD voluntarily received the procedure. The procedure was successful in 58 patients, with a success rate of 93.5% with Amplatzer device. The diameter of VSD ranged from 2.4 to 13.9 (mean 5.3-/+2.0) mm with echocardiography, and the size of Amplatzer device ranged from 4-18 (mean 8.3-/+2.9) mm. Perimembranous ventricular septal defect was complicated by aneurysm formation in 22 patients. Residual trivial or mild shunt was seen in seven (12%) patients at 24 h and one (1.7%) patient at 3 months. Seven (12.1%) patients developed heart block, 3 (5.2%) had intermittence and transient complete heart block, and one had transient second degree atrioventricular block disappearing in 3 to 10 days, and 3 (5.2%) patients had complete right bundle branch block lasting for one month. None of the patients developed significant aortic regurgitation (P>0.05), although 22 showed a superior margin of the defect less than 3 mm from the aortic valve. The mean distance from the aortic valve was 3.7-/+2.7 (1.0 to 10.5) mm. No significant mitral and tricuspid regurgitation occurred in these patients. Four patients had unsuccessful procedures. Percutaneous closure with Amplatzer device can be carried out successfully in a majority of suitable defects screened using transthoracic echocardiography. Echocardiography can exactly demonstrate the anatomical variation and classification of ventricular septal defect in adults. Attention should be given to the misdiagnosis by echocardiography of a doubly committed defect as a perimembranous outflow defect. Heart block can be an important

  17. Outcomes and Prognostic Factors for Adult Patients With Congenital Heart Disease Undergoing Primary or Reoperative Systemic Atrioventricular Valve Surgery.

    Science.gov (United States)

    Stephens, Elizabeth H; Han, Jiho; Ginns, Jonathan; Rosenbaum, Marlon; Chai, Paul; Bacha, Emile; Kalfa, David

    2017-05-01

    Adults with congenital heart disease (ACHD) undergoing systemic atrioventricular valve (SAVV) surgery are a complex, understudied population. We assessed midterm outcomes and prognostic factors in ACHD undergoing SAVV surgery. We performed retrospective evaluation of ACHD undergoing SAVV surgery from January 2005 to February 2016: 14 (33%) patients with congenital mitral valve stenosis/regurgitation, 15 (35%) with atrioventricular septal defect (AVSD), and 14 (33%) with congenitally corrected transposition of the great arteries (ccTGA) with systemic tricuspid valve regurgitation. Adverse events were defined as mortality, reoperation on SAVV, and late more-than-moderate (> moderate) SAVV regurgitation. Statistical analysis was performed using Fisher's exact test and one-way analysis of variance as well as univariate and multivariate risk factor analysis. Fifteen (35%) patients had preoperative systemic ventricular dysfunction, including 13 patients with ccTGA (93%, P moderate SAVV regurgitation, and SAVV reoperation rates were 5% (n = 2), 2% (n = 1), 9% (n = 3), and 7% (n = 3), respectively. On multivariate analysis, predischarge SAVV regurgitation grade was the only significant predictor of adverse events (odds ratio = 8.2, 95% confidence interval: 1.1-63.8, P = .045). Overall outcomes in this challenging population are good. The single factor associated with adverse events was predischarge SAVV regurgitation grade.

  18. Association of certain chronic maternal diseases with the risk of specific congenital heart defects: a population-based study.

    Science.gov (United States)

    Vereczkey, A; Gerencsér, B; Czeizel, A E; Szabó, I

    2014-11-01

    Previous epidemiological studies have evaluated cases with all congenital heart defects (CHDs), rather than analysing different types of CHD. The objective of this study was to evaluate the possible association of certain chronic maternal diseases with the risk of different types of CHD, because the role of possible environmental factors in the origin of CHDs is unclear in the vast majority of patients. Different types of CHD, diagnosed after lethal outcome (autopsy report) or after surgical intervention (catheter or correction), were evaluated in order to estimate the possible role of chronic maternal diseases in their origin. This analysis was based on the rates of medically recorded chronic maternal diseases in 3562 live-born cases with CHDs, 38,151 population controls without any birth defects, and 16,602 malformed controls with other isolated congenital abnormalities, using the data set of the population-based Hungarian Case-Control Surveillance of Congenital Abnormalities (1980-1996). Maternal epilepsy treated with carbamazepine and migraine were found to be associated with higher risk of ventricular septal defect; panic disorders were associated with higher risk of hypoplastic left heart; type I diabetes mellitus was associated with higher risk of coarctation of the aorta; chronic hypertension was associated with higher risk of ventricular septal defect, common atrioventricular canal and common truncus; and paroxysmal supraventricular tachycardia was associated with higher risk of atrial septal defect secundum, common atrioventricular canal and ventricular septal defect. In conclusion, certain chronic maternal diseases were found to be associated with higher risk of specific CHDs. Appropriate treatment of these diseases may help to prevent these CHDs. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  19. Facts about Ventricular Septal Defect

    Science.gov (United States)

    ... a healthy weight gain, a special high-calorie formula might be prescribed. Some babies become extremely tired ... file Microsoft PowerPoint file Microsoft Word file Microsoft Excel file Audio/Video file Apple Quicktime file RealPlayer ...

  20. Atrial Septal Defect (For Kids)

    Science.gov (United States)

    ... People, Places & Things That Help Feelings Q&A Movies & More Quizzes Kids' Dictionary of Medical Words En ... hole is cardiac catheterization. This method uses a thin, flexible tube called a catheter (say: KATH-uh- ...

  1. Sinus Venosus Atrial Septal Defect

    Science.gov (United States)

    2010-04-01

    the atria. The presence of PAPVR compounds the problem with additional left-to-right shunt pathways. While the exact embryology is controversial...Cardiology 2001; 87:305-09. 4. Sadley TW. Langman’s Medical Embryology , 7th ed. Williams and Wilkins 1995: 191-201. 5. Al Zaghal AM et. al

  2. Atrial Septal Defect (For Teens)

    Science.gov (United States)

    ... ASDs happen during a baby's development in the mother's womb. Before birth, the heart develops from a ... around the mouth or on the lips and tongue loss of appetite weight loss feeling lethargic a ...

  3. Effect of Clopidogrel and Aspirin vs Aspirin Alone on Migraine Headaches After Transcatheter Atrial Septal Defect Closure: The CANOA Randomized Clinical Trial.

    Science.gov (United States)

    Rodés-Cabau, Josep; Horlick, Eric; Ibrahim, Reda; Cheema, Asim N; Labinaz, Marino; Nadeem, Najaf; Osten, Mark; Côté, Mélanie; Marsal, Josep Ramon; Rivest, Donald; Marrero, Alier; Houde, Christine

    2015-11-24

    The occurrence of new-onset migraine attacks is a complication of transcatheter atrial septal defect (ASD) closure. It has been suggested that clopidogrel may reduce migraine attacks after ASD closure. To assess the efficacy of clopidogrel, used in addition to taking aspirin, for the prevention of migraine attacks following ASD closure. Randomized, double-blind clinical trial performed in 6 university hospitals in Canada. Participants were 171 patients with an indication for ASD closure and no history of migraine. Patients were randomized (1:1) to receive dual antiplatelet therapy (aspirin + clopidogrel [the clopidogrel group], n = 84) vs single antiplatelet therapy (aspirin + placebo [the placebo group], n = 87) for 3 months following transcatheter ASD closure. The first patient was enrolled in December 2008, and the last follow-up was completed in February 2015. The primary efficacy outcome was the monthly number of migraine days within the 3 months following ASD closure in the entire study population. The incidence and severity of new-onset migraine attacks, as evaluated by the Migraine Disability Assessment questionnaire, were prespecified secondary end points. A zero-inflated Poisson regression model was used for data analysis. The mean (SD) age of the participants was 49 (15) years and 62% (106) were women. Patients in the clopidogrel group had a reduced mean (SD) number of monthly migraine days within the 3 months following the procedure (0.4 [95% CI, 0.07 to 0.69] days) vs the placebo group (1.4 [95% CI, 0.54 to 2.26] days; difference, -1.02 days [95% CI, -1.94 to -0.10 days]; incident risk ratio [IRR], 0.61 [95% CI, 0.41 to 0.91]; P = .04) and a lower incidence of migraine attacks following ASD closure (9.5% for the clopidogrel group vs 21.8% for the placebo group; difference, -12.3% [95% CI, -23% to -1.6%]; odds ratio [OR], 0.38 [95% CI, 0.15 to 0.89]; P = .03). Among patients with migraines, those in the clopidogrel group had less

  4. Types and distribution of congenital heart defects associated with trisomy 21 in Singapore.

    Science.gov (United States)

    Tan, Monica; Xu, Cunzhi; Sim, Sarah K R; Seow, Adeline L H; Tan, Teng Hong; Quek, Swee Chye

    2013-03-01

    Atrioventricular septal defect (AVSD) is widely accepted as the most common type of congenital heart defect in trisomy 21. Most of these studies, however, were conducted in Caucasian communities. The few Asian studies that had been conducted on this subject yielded different results. In the largest study of its kind in Asia, we described the distribution of types of congenital heart defects associated with trisomy 21 in Singapore. Five hundred and eighty-eight patients with trisomy 21 born in 1996-2010, and confirmed by karyotyping, were included in the study. The diagnosis of congenital heart defects were made on echocardiography. Variables extracted for analysis were demographics (race and gender) and the types of congenital heart defects. Except for complex cyanotic heart defects, haemodynamically significant lesions were accounted for separately in cases where more than one type of congenital heart defect coexisted in a patient. Ventricular septal defect (VSD) (39.2%) was the most common congenital heart defect associated with trisomy 21 in our study, followed by patent ductus arteriosus (34.3%), secundum atrial septal defect (23.4%) and AVSD (15.6%). This study validates previous smaller Asian studies identifying VSD as the most common cardiac lesion associated with trisomy 21. A high proportion (25.0%) of trisomy 21 patients with tetralogy of Fallot also had AVSDs. Coarctation of the aorta was uncommon. VSD was the most common congenital heart defect seen in trisomy 21 in our study. A high proportion (25.0%) of trisomy 21 patients with tetralogy of Fallot also had AVSDs. © 2013 The Authors. Journal of Paediatrics and Child Health © 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

  5. Experiencia multicéntrica colombiana durante tres años en el cierre percutáneo de la comunicación interventricular con diferentes dispositivos oclusores. Propuesta para la simplicación de la técnica en pacientes seleccionados Colombian multicentric experience during three years for percutaneous closure of interventricular septal defect with different occluder devices. Proposal for technique simplification in selected patients

    Directory of Open Access Journals (Sweden)

    Manuel R Téllez

    2010-09-01

    ,9% acusaron aparición o acentuación de episodios de cefalea vascular migrañosa. Hubo una complicación mayor dada por bloqueo atrioventricular completo y embolización del dispositivo. Conclusiones: el cierre endovascular de la comunicación interventricular es un procedimiento factible en nuestro medio, con el cual se alcanza un éxito cercano al 100%. El dispositivo para cierre de ductus de última generación puede implantarse con modificaciones en la técnica que aminora los tiempos de operatividad (hasta 60%, anestesia, utilización de medio de contraste y estancia hospitalaria. Por otra parte, su perfil hace posible el uso de sistemas de entregas 4 French que posibilitaría la inclusión de lactantes menores.Objectives: we describe the experience of the endovascular closure of interventricular communication performed for three years in different centers of interventional cardiology and highlight its result in a group of selected patients in whom such procedure was made by the use of a last generation device designed for the closure of ductus arteriosus. Methodology: between June 2006 and October 2009, a total of 34 patients underwent endovascular occlusion for interventricular septal defect. Indication of this procedure included volume overload, pressure and volume overload, chronic congestive heart failure, aortic valve prolapse with insufficiency and traumatic injury. Mean age was 12,9 years old and mean weight was 36 kg. A prevalence in male gender (53% was evidenced. Main blood flow calculation showed Qp / Qs of 1.71 to 1 and the pulmonary vascular resistance was 1.18 U Wood/m2. Materials and methods: PM VSD occluder device was implanted with the classic technique (62,5% while the Duct Occluder II (28,1% was used with the proposed simplified technique. General anesthesia was used in 98% cases; 60% were guided by fluoroscopy and transesophageal echocardiography and 40% with transthoracic echocardiography. Results: size of the defect varied from 4 to 12 mm. 91% of

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

    Science.gov (United States)

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

    2017-09-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2010-10-15

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

  8. Serotonin Potentiates Transforming Growth Factor-beta3 Induced Biomechanical Remodeling in Avian Embryonic Atrioventricular Valves

    Science.gov (United States)

    Buskohl, Philip R.; Sun, Michelle L.; Thompson, Robert P.; Butcher, Jonathan T.

    2012-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Philip R Buskohl

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

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

    Directory of Open Access Journals (Sweden)

    Luis A Gutiérrez

    2012-10-01

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

  11. Case report: airway and concurrent hemodynamic management in a neonate with oculo-auriculo-vertebral (Goldenhar) syndrome, severe cervical scoliosis, interrupted aortic arch, multiple ventricular septal defects, and an unstable cervical spine.

    Science.gov (United States)

    Char, Danton S; Gipp, Melanie; Boltz, M Gail; Williams, Glyn D

    2012-09-01

    We report the challenging case of a 1-week-old, term, 2.4 kg neonate with Goldenhar syndrome (including microcephaly, left microtia, left facial palsy, dextro-scoliosis of the cervical spine, and cervico-thoracic levoscoliosis), multiple ventricular septal defects, a type B interrupted aortic arch, a large patent ductus arteriosis, and radiographic and clinical signs concerning for an unstable cervical spine. Our anesthesia team was consulted for perioperative management of this patient during her surgical repair. This case report describes the use of the Air-Q size 1 laryngeal airway (LA) to assist fiberoptic intubation in an ASA 4 neonate with cardiac disease, an anticipated difficult airway with the addition of an unstable cervical spine, as well as the anesthetic techniques used to maintain hemodynamic stability while the airway was secured. © 2012 Blackwell Publishing Ltd.

  12. Dynamic characteristic mechanism of atrial septal defect using real-time three-dimensional echocardiography and evaluation of right ventricular functions.

    Science.gov (United States)

    Sharen, Gao-Wa; Zhang, Jun; Qin, Chuan; Lv, Qing

    2017-02-01

    The dynamic characteristics of the area of the atrial septal defect (ASD) were evaluated using the technique of real-time three-dimensional echocardiography (RT 3DE), the potential factors responsible for the dynamic characteristics of the area of ASD were observed, and the overall and local volume and functions of the patients with ASD were measured. RT 3DE was performed on the 27 normal controls and 28 patients with ASD. Based on the three-dimensional data workstations, the area of ASD was measured at P wave vertex, R wave vertex, T wave starting point, and T wave terminal point and in the T-P section. The right atrial volume in the same time phase of the cardiac cycle and the motion displacement distance of the tricuspid annulus in the corresponding period were measured. The measured value of the area of ASD was analyzed. The changes in the right atrial volume and the motion displacement distance of the tricuspid annulus in the normal control group and the ASD group were compared. The right ventricular ejection fractions in the normal control group and the ASD group were compared using the RT 3DE long-axis eight-plane (LA 8-plane) method. Real-time three-dimensional volume imaging was performed in the normal control group and ASD group (n=30). The right ventricular inflow tract, outflow tract, cardiac apex muscular trabecula dilatation, end-systolic volume, overall dilatation, end-systolic volume, and appropriate local and overall ejection fractions in both two groups were measured with the four-dimensional right ventricular quantitative analysis method (4D RVQ) and compared. The overall right ventricular volume and the ejection fraction measured by the LA 8-plane method and 4D RVQ were subjected to a related analysis. Dynamic changes occurred to the area of ASD in the cardiac cycle. The rules for dynamic changes in the area of ASD and the rules for changes in the right atrial volume in the cardiac cycle were consistent. The maximum value of the changes in the

  13. Is the prevalence of specific types of congenital heart defects different for non-Hispanic white, non-Hispanic black and Hispanic infants?

    Science.gov (United States)

    Nembhard, Wendy N; Salemi, Jason L; Wang, Tao; Loscalzo, Melissa L; Hauser, Kimberlea W

    2010-03-01

    Our purpose was to determine the prevalence of specific types of CHD among non-Hispanic (NH)-Black, NH-White, and Hispanic infants. We conducted a retrospective cohort study with 9,352 singleton infants diagnosed with conotruncal, right or left obstructive or septal CHDs from the Florida Birth Defects Registry, born 1998-2003 to resident NH-White, NH-Black, and Hispanic women aged 15-49. Defect-specific prevalence rates, prevalence ratios and P-values were calculated for each type of CHD and by number of defects for each racial/ethnic group. Compared to NH-Whites, NH-Blacks had higher rates of pulmonary valve atresia/stenosis but lower frequency of aortic valve atresia/stenosis and ventricular septal defect. Hispanics had lower rates of aortic valve atresia/stenosis and atrioventricular septal defects than NH-Whites. Although few racial/ethnic differences in prevalence are present among infants with major CHD, observed differences are clinically meaningful. However, the underlying etiologies for the observed differences remain unknown.

  14. Does the atrioventricular node conduct?

    NARCIS (Netherlands)

    Meijler, F.L.; Fisch, C.

    1989-01-01

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

  15. Experiência inicial no fechamento percutâneo da comunicação interatrial com a prótese de Amplatzer Initial experience in percutaneous occlusion of atrial septal defects with the Amplatzer device

    Directory of Open Access Journals (Sweden)

    Valmir F. Fontes

    1998-03-01

    Full Text Available OBJETIVO: Analisar a experiência inicial no fechamento percutâneo da comunicação interatrial ostium secundum (CIA OS com a prótese de Amplatzer. MÉTODOS: Sete pacientes foram submetidos ao procedimento através da via venosa anterógrada, orientados pela ecocardiografia transesofágica (ETE e sob anestesia geral. Uma criança era portadora de 2 CIA e de canal arterial (CA. As CIA medidas pelo ETE variaram de 8,7 a 20mm. Um ecocardiograma transtorácico foi realizado na manhã seguinte do procedimento. RESULTADOS: Oito próteses foram implantadas nos 7 pacientes com sucesso. Em um paciente, o CA foi ocluído na mesma sessão com mola de Gianturco, tendo surgido taquicardia supraventricular durante a oclusão de uma das CIA, controlada com adenosina. Todos receberam alta hospitalar na manhã seguinte, com oclusão total dos defeitos. CONCLUSÃO: O procedimento mostrou-se seguro, eficaz e versátil, podendo ser considerado como uma alternativa terapêutica inicial em pacientes selecionados com CIA OS.PURPOSE: To evaluate our initial experience with percutaneous closure of secundum type atrial septal defects (ASD with the Amplatzer septal occluder. METHODS: Seven patients underwent occlusion by anterograde approach, under general anesthesia and transesophageal echocardiography (TEE guidance. One child had 2 ASD and a patent ductus arteriosus (PDA. The ASD size ranged from 8,7 to 20mm as measured by TEE. A transthoracic echocardiogram was performed in the morning after the procedure. RESULTS: Eight devices were successfully implanted in 7 patients and the PDA was occluded with a Gianturco coil at the same session. In this patient, there was an episode of supraventricular tachycardia during the occlusion of one ASD which was reverted with adenosin. All patients were discharged the day after, with complete occlusion of all defects. CONCLUSION: The procedure is safe, effective and versatile. It can be applied as an initial alternative to the

  16. Three Achilles’ heels of alcohol septal ablation

    Directory of Open Access Journals (Sweden)

    M. G. Kashtanov

    2017-11-01

    Full Text Available This manuscript looks at basic limitations of alcohol septal ablation in obstructive hypertrophic cardiomyopathy. They include high-grade atrioventricular blockages, residual obstructions of the left ventricular outflow tract and the so-called proarrhythmic effects of alcohol septal ablation procedure. All these weaknesses are reviewed in the context of incidence, etiology, and prevention.Received 25 February 2017. Accepted 10 April 2017.Funding: The study did not have sponsorship.Conflict of interest: The authors declare no conflict of interest.Author contributionsConception and study design: M.G. Kashtanov.Data collection and analysis: M.G. Kashtanov.Drafting the article: M.G. Kashtanov, E.M. Idov.Final approval of the version to be published: M.G. Kashtanov, S.D. Chernyshev, L.V. Kardapoltsev, S.V. Berdnikov, E.M. Idov.Full text of the article is in the online version of this paper at http://dx.doi.org/10.21688/1681-3472-2017-3-12-22

  17. Atrioventricular Dissociation after Electroconvulsive Therapy

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    Siegfried William Yu

    2011-01-01

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

  18. Persistent iatrogenic atrial septal defect after a single-puncture, double-transseptal approach for pulmonary vein isolation using a remote robotic navigation system: results from a prospective study.

    Science.gov (United States)

    Rillig, Andreas; Meyerfeldt, Udo; Kunze, Markus; Birkemeyer, Ralf; Miljak, Tomislav; Jäckle, Sebastian; Hajredini, Bajram; Treusch, Fabian; Jung, Werner

    2010-03-01

    Persistent iatrogenic atrial septal defect (iASD) after transseptal puncture for pulmonary vein isolation (PVI) has been described recently as a complication of PVI. No data exists evaluating systematically the incidence and clinical implications of iASDs after PVI using a remote robotic navigation system (RNS) with sheaths with a distinct larger outer diameter. In this prospective study, 40 patients with either paroxysmal (n = 22, 55%) or persistent symptomatic atrial fibrillation were treated with circumferential PVI using an RNS. In all patients, a single-puncture, double-transseptal approach was used to access the left atrium. Transoesophageal echocardiography was performed before and the day after PVI as well as after a 3 and 6 months follow-up (FU). The day after ablation an iASD was detected in 38 of 40 (95%) patients with a mean diameter of 3.45 +/- 1.5 mm. At 6-month FU, the iASDs were closed in 30 of 39 (78.9%) patients. During the 6-month FU period, no patient died or suffered from cerebral or cardiac embolism. After a single-puncture, double-transseptal approach for PVI using the RNS, iASDs show a high spontaneous closure rate of 78.9% after a 6-month FU period. Persistent iASDs following PVI with the RNS are not associated with an increased rate of paradoxical embolism or with relevant shunting.

  19. Significant survival advantage of high pulmonary vein index and the presence of native pulmonary artery in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: results from preoperative computed tomography angiography.

    Science.gov (United States)

    Jia, Qianjun; Cen, Jianzheng; Zhuang, Jian; Zhong, Xiaomei; Liu, Xiaoqing; Li, Jiahua; Liang, Changhong; Huang, Meiping

    2017-08-01

    The prognosis of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA-VSD-MAPCAs) after surgery shows substantial clinical heterogeneity and predictors for outcomes are lacking. This study aimed to assess the predictive value of preoperative cardiac computed tomography angiography (CTA) for survival in patients with PA-VSD-MAPCAs. We retrospectively analysed PA-VSD-MAPCA patients with preoperative CTA who underwent both right ventricular outflow tract reconstruction and MAPCA unifocalization ( n  = 24) or pulmonary artery rehabilitation ( n  = 28). The end-point was overall survival. Prognostic values of CTA were assessed using Cox univariate and multivariate analyses. The significant threshold of independent parameters was calculated using receiver-operating characteristic (ROC) curves. During a median follow-up of 1145 days, a total of 13 deaths were observed. Multivariate analysis identified a high pulmonary vein index (PVI) [hazard ratio (HR) = 0.03; 95% confidence interval (CI): 0.03, 0.28; P  advantage in PA-VSD-MAPCA patients. A PVI ≥438 mm 2 /m 2 may be a reliable positive prognosticator that could improve the decision-making strategy for PA-VSD-MAPCA patients.

  20. Maternal smoking during pregnancy and the risk of congenital heart defects in offspring: a systematic review and metaanalysis.

    Science.gov (United States)

    Lee, Laura J; Lupo, Philip J

    2013-02-01

    Although a previous metaanalysis indicated that maternal smoking during pregnancy increased the risk of congenital heart defects (CHD) in offspring, the effect of smoking on individual CHD subtypes was not determined. Because CHDs are anatomically, clinically, epidemiologically, and developmentally heterogeneous, the authors conducted a systematic review and metaanalysis of the association between maternal smoking during pregnancy and the risk of CHDs, including CHD subtypes among offspring. Two types of summary relative risk (RR) estimates (any smoking vs no smoking and increasing categories of smoking, i.e., light, medium, and heavy) were calculated for CHDs as a group and for a number of CHD subtypes using both fixed- and random-effects models. Random effects estimates were reported if there was evidence of heterogeneity among the studies. Consistent with the previous metaanalysis, the authors observed a positive association between maternal smoking during pregnancy and the risk of CHDs as a group (RR, 1.11; 95 % confidence interval [CI], 1.02-1.21; number of cases [n] = 18,282). Additionally, women who smoked during pregnancy were more likely to have a child with 12 (71 %) of 17 CHD subtypes analyzed compared with women who did not smoke. The highest risk was for septal defects as a group (RR, 1.44; 95 % CI, 1.16-1.79; n = 2977). The evidence of dose response was observed for septal defects as a group, atrial septal defects, and atrioventricular septal defects. This systematic review and metaanalysis suggests that maternal smoking is modestly associated with an increased risk of CHDs and some CHD subtypes.

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

    Directory of Open Access Journals (Sweden)

    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.

  2. Anatomy of the human atrioventricular junctions revisited

    NARCIS (Netherlands)

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

    2000-01-01

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

  3. The influence of deficient retro-aortic rim on technical success and early adverse events following device closure of secundum atrial septal defects: An Analysis of the IMPACT Registry®.

    Science.gov (United States)

    O'Byrne, Michael L; Gillespie, Matthew J; Kennedy, Kevin F; Dori, Yoav; Rome, Jonathan J; Glatz, Andrew C

    2017-01-01

    Concern regarding aortic erosion has focused attention on the retro-aortic rim in patients undergoing device closure of atrial septal defects (ASD), but its effect on early outcomes is not well studied. A multicenter retrospective cohort study of patients undergoing device occlusion of ASD between 1/2011-10/2014 was performed, using data from the IMproving Pediatric and Adult Congenital Treatment Registry. Subjects were divided between those with retro-aortic rim technical failure and major early adverse events. Case times were measured as surrogates of technical complexity. The effect of deficient retro-aortic rim on primary outcomes was assessed using hierarchical logistic regression, adjusting for other suspected covariates and assessing whether they represent independent risk factors RESULTS: 1,564 subjects (from 77 centers) were included, with deficient retro-aortic rim present in 40%. Technical failure occurred in 91 subjects (5.8%) and a major early adverse event in 64 subjects (4.1%). Adjusting for known covariates, the presence of a deficient retro-aortic rim was not significantly associated with technical failure (OR: 1.3, 95% CI: 0.9-2.1) or major early adverse event (OR: 0.7, 95% CI: 0.4-1. 2). Total case (P = 0.01) and fluoroscopy time (P = 0.02) were greater in subjects with deficient rim, but sheath time was not significantly different (P = 0.07). Additional covariates independently associated with these outcomes were identified. Deficient retro-aortic rim was highly prevalent but not associated with increased risk of technical failure or early adverse events. Studies with longer follow-up are necessary to assess other outcomes, including device erosion. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  4. Right to Left Ventricular Diameter Ratio ≥0.42 is the Warning Flag for Suspecting Atrial Septal Defect in Preschool Children: Age- and Body Surface Area-Related Reference Values Determined by M-Mode Echocardiography.

    Science.gov (United States)

    Hashimoto, Ikuo; Watanabe, Kazuhiro; Ichida, Fukiko

    2016-04-01

    It is not always easy to observe and screen atrial septal defects (ASD) using echocardiography. In addition, there are no established echocardiographic reference indices for screening patients with ASDs. We retrospectively reviewed our database and recruited 151 isolated ASD patients and 2769 healthy subjects. In total, 307 echocardiographic studies were performed for ASD patients. Surgical repairs were done in 75 of the ASD patients. The ratio of right to left ventricular end-diastolic dimensions (RVD/LVD), which was determined by M-mode echocardiography, was used as an index of RV dilatation. After obtaining age- and body surface area (BSA)-related RVD/LVD nomograms in healthy subjects, we calculated the z-scores of RVD/LVD for all subjects and obtained the optimal cut-off values to differentiate patients with ASD from healthy subjects. The optimal cut-off values were high in neonates and gradually decreased with an increase in the age and BSA, but were almost constant in children aged >4 years or whose BSA was >0.65 m(2). The cut-off values of RVD/LVD for suspected ASD were ≥0.42 in children aged >4 years or those whose BSA was >0.65 m(2). Those for an ASD operation were ≥0.46 in those whose BSA > 0.65 m(2). The RVD/LVD determined by M-mode echocardiography is a useful index to evaluate RV dilatation in patients with ASDs. The RVD/LVD ≥ 0.42 is the warning flag for suspecting ASD in preschool children and that ≥0.46 may be a clinical important sign to determine ASD operation.

  5. Patterns in the prevalence of congenital heart defects, metropolitan Atlanta, 1978 to 2005.

    Science.gov (United States)

    Bjornard, Kari; Riehle-Colarusso, Tiffany; Gilboa, Suzanne M; Correa, Adolfo

    2013-02-01

    Knowledge of patterns in prevalence of congenital heart defects (CHDs) is important for clinical care, etiologic research, and prevention. We evaluated temporal and racial/ethnic trends in the birth prevalence of CHDs in metropolitan Atlanta from 1978 to 2005. Cases of CHDs were obtained from the Metropolitan Atlanta Congenital Defects Program among live born infants, stillborn infants, and pregnancy terminations of at least 20 weeks gestation. We calculated birth prevalence per 10,000 live births and used joinpoint regression analysis to calculate the average annual percent change for total CHDs and for 23 specific subtypes in the total population and among whites and blacks. To evaluate racial/ethnic variations, we calculated prevalence ratios among blacks and Hispanics compared with whites. Between 1978 and 2005, 7301 infants and fetuses with major structural CHDs were ascertained among 1,079,062 live births (67.7 per 10,000). The prevalence of all CHDs in aggregate increased from 50.3 per 10,000 in 1978-1983 to 86.4 per 10,000 in 2000-2005. The prevalence of septal defects and vascular rings increased and the prevalence of tricuspid atresia decreased, while other CHD prevalences were stable. Racial/ethnic prevalence differences were found for all CHDs combined and muscular ventricular septal defects, aortic stenosis, and atrioventricular septal defects. The prevalence of total CHDs, primarily common, less severe types, are increasing, with some racial/ethnic differences. Further studies could clarify the possible reasons for such variations including differences in ascertainment, risk factors, or susceptibility. Copyright © 2013 Wiley Periodicals, Inc.

  6. Cryoablation of septal accessory pathways in children: midterm results.

    Science.gov (United States)

    Karadeniz, Cem; Akdeniz, Celal; Turan, Ozlem; Tuzcu, Volkan

    2014-09-01

    Radiofrequency (RF) catheter ablation in the septal arrhythmia substrates has an increased risk of irreversible atrioventricular block. Despite its safety profile, several studies reported a lower acute success rate and a higher recurrence rate with cryoablation of septal accessory pathways (APs) when compared to RF ablation. The aim of this study was to assess the efficacy and safety of cryoablation of right septal APs using an electroanatomical mapping system guidance. A total of 43 consecutive patients (13.2 ± 5.5 years) underwent cryoablation for right septal APs. The EnSite system (St. Jude Medical, St. Paul, MN, USA) was used in all procedures. No fluoroscopy was used in 90% of patients (39/43). The mean fluoroscopy time in the remaining four patients was 3.7 ± 0.7 minutes. An electrophysiology catheter with 2-mm distal spacing was used to determine the precise AP location. The majority of the patients (20/43) had anteroseptal, and remaining of the patients had posteroseptal (15/43) and midseptal (8/43) APs. A 6-mm-tip catheter was used in 33 patients and 8-mm-tip catheter was used in nine patients (both catheters were used in one patient). The mean procedure duration and number of complete cryoablation lesions were 181.5 ± 60.6 minutes and 6.1 ± 3.1 minutes, respectively. Acute success was achieved in 40 of 43 patients (93%). No complications were noted. During a mean follow-up of 8.8 ± 4.8 months, five patients (12.5%) experienced recurrence. Our data suggest that cryoablation of septal APs can be performed safely with comparable efficacy to the reported RF ablation results using a limited fluoroscopy approach. ©2014 Wiley Periodicals, Inc.

  7. Prevalence of congenital heart defects in patients with Down syndrome in the municipality of Pelotas, Brazil.

    Science.gov (United States)

    Vilas Boas, Luciana T; Albernaz, Elaine P; Costa, Rafaéla Gonçalves

    2009-01-01

    To determine the prevalence of congenital heart defects in patients with Down syndrome in the municipality of Pelotas, Brazil, describing the most frequent types and assessing the associated factors. Cross-sectional study including children with Down syndrome who were born and lived in Pelotas from January 2000 to December 2005. Data were collected by means of home interviews with mothers or guardians. Univariate and bivariate analyses were carried out to analyze the factors related to congenital heart defect. Forty-seven mothers of patients with Down syndrome were interviewed. Twenty-two (46.8%) of the patients had a diagnosis of congenital heart defect. Of them, 28% had early cardiac evaluation before 3 months of life. The most frequent heart defect was interatrial communication (17%); atrioventricular septal defect affected five patients. Bivariate analysis between the outcome congenital heart defect and the predicting factors maternal age, paternal age, parents' and child's skin color, presence of other malformations and child's sex showed that the associations were not statistically significant. The prevalence of Down syndrome and congenital heart defects in our region is similar to the rates found by other authors; therefore, we highlight the importance of diagnostic suspicion and early referral by pediatricians to cardiac evaluation. Another relevant aspect is the small number of patients who underwent karyotype testing. In addition, the number of associated malformations was lower than that found by other authors.

  8. Consanguineous marriage and congenital heart defects: a case-control study in the neonatal period.

    Science.gov (United States)

    Yunis, Khalid; Khalid, Yunis; Mumtaz, Ghina; Ghina, Mumtaz; Bitar, Fadi; Fadi, Bitar; Chamseddine, Fadi; Fadi, Chamseddine; Kassar, May; May, Kassar; Rashkidi, Joseph; Joseph, Rashkidi; Makhoul, Ghaith; Makhoul, Ghaith; Tamim, Hala; Hala, Tamim

    2006-07-15

    The independent effect of consanguinity on the prevalence of congenital heart defects (CHDs), all and specific types, was investigated in newborns admitted to nine hospitals located in Beirut, Lebanon and members of the National Collaborative Perinatal Neonatal Network (NCPNN). Cases were 173 newborns admitted to the Neonatal Intensive Care Units (NICU) of participating hospitals during the 3-year period from January 1, 2000 to December 31, 2002 and diagnosed during their hospital stay as having one or more CHD. Cases with chromosomal abnormalities were excluded. Cases with more than one CHD were assigned one principal malformation. Controls consisted of a random sample of 865 newborns without a CHD admitted to the NICU during the same period. After controlling for confounders, first cousin consanguinity remained significantly associated with an increased risk of CHD: infants born to first cousin marriages had a 1.8 times higher risk of having a CHD diagnosed at birth compared to those born to unrelated parents (95% CI: 1.1-3.1). In particular, first-cousin marriage was a significant risk factor for ventricular septal defect (VSD), atrial septal defect (ASD), hypoplastic left heart (HLH), and single ventricle (SV). No association was found with d-transposition of the great arteries, coarctation, pulmonary atresia (PA), atrioventricular septal defect (AVSD), and tetralogy of Fallot (TOF). The results of this study suggest a familial factor in the multifactorial etiology of CHDs. Additional epidemiologic and family-based genetic studies are needed to understand the complex cause of CHDs. Copyright 2006 Wiley-Liss, 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.

    Directory of Open Access Journals (Sweden)

    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. Molecular signatures of cardiac defects in Down syndrome lymphoblastoid cell lines suggest altered ciliome and Hedgehog pathways.

    Directory of Open Access Journals (Sweden)

    Clémentine Ripoll

    Full Text Available Forty percent of people with Down syndrome exhibit heart defects, most often an atrioventricular septal defect (AVSD and less frequently a ventricular septal defect (VSD or atrial septal defect (ASD. Lymphoblastoid cell lines (LCLs were established from lymphocytes of individuals with trisomy 21, the chromosomal abnormality causing Down syndrome. Gene expression profiles generated from DNA microarrays of LCLs from individuals without heart defects (CHD(-; n = 22 were compared with those of LCLs from patients with cardiac malformations (CHD(+; n = 21. After quantile normalization, principal component analysis revealed that AVSD carriers could be distinguished from a combined group of ASD or VSD (ASD+VSD carriers. From 9,758 expressed genes, we identified 889 and 1,016 genes differentially expressed between CHD(- and AVSD and CHD(- and ASD+VSD, respectively, with only 119 genes in common. A specific chromosomal enrichment was found in each group of affected genes. Among the differentially expressed genes, more than 65% are expressed in human or mouse fetal heart tissues (GEO dataset. Additional LCLs from new groups of AVSD and ASD+VSD patients were analyzed by quantitative PCR; observed expression ratios were similar to microarray results. Analysis of GO categories revealed enrichment of genes from pathways regulating clathrin-mediated endocytosis in patients with AVSD and of genes involved in semaphorin-plexin-driven cardiogenesis and the formation of cytoplasmic microtubules in patients with ASD-VSD. A pathway-oriented search revealed enrichment in the ciliome for both groups and a specific enrichment in Hedgehog and Jak-stat pathways among ASD+VSD patients. These genes or related pathways are therefore potentially involved in normal cardiogenesis as well as in cardiac malformations observed in individuals with trisomy 21.

  11. Molecular Signatures of Cardiac Defects in Down Syndrome Lymphoblastoid Cell Lines Suggest Altered Ciliome and Hedgehog Pathways

    Science.gov (United States)

    Ripoll, Clémentine; Rivals, Isabelle; Ait Yahya-Graison, Emilie; Dauphinot, Luce; Paly, Evelyne; Mircher, Clothilde; Ravel, Aimé; Grattau, Yann; Bléhaut, Henri; Mégarbane, André; Dembour, Guy; de Fréminville, Bénédicte; Touraine, Renaud; Créau, Nicole; Potier, Marie Claude; Delabar, Jean Maurice

    2012-01-01

    Forty percent of people with Down syndrome exhibit heart defects, most often an atrioventricular septal defect (AVSD) and less frequently a ventricular septal defect (VSD) or atrial septal defect (ASD). Lymphoblastoid cell lines (LCLs) were established from lymphocytes of individuals with trisomy 21, the chromosomal abnormality causing Down syndrome. Gene expression profiles generated from DNA microarrays of LCLs from individuals without heart defects (CHD−; n = 22) were compared with those of LCLs from patients with cardiac malformations (CHD+; n = 21). After quantile normalization, principal component analysis revealed that AVSD carriers could be distinguished from a combined group of ASD or VSD (ASD+VSD) carriers. From 9,758 expressed genes, we identified 889 and 1,016 genes differentially expressed between CHD− and AVSD and CHD− and ASD+VSD, respectively, with only 119 genes in common. A specific chromosomal enrichment was found in each group of affected genes. Among the differentially expressed genes, more than 65% are expressed in human or mouse fetal heart tissues (GEO dataset). Additional LCLs from new groups of AVSD and ASD+VSD patients were analyzed by quantitative PCR; observed expression ratios were similar to microarray results. Analysis of GO categories revealed enrichment of genes from pathways regulating clathrin-mediated endocytosis in patients with AVSD and of genes involved in semaphorin-plexin-driven cardiogenesis and the formation of cytoplasmic microtubules in patients with ASD-VSD. A pathway-oriented search revealed enrichment in the ciliome for both groups and a specific enrichment in Hedgehog and Jak-stat pathways among ASD+VSD patients. These genes or related pathways are therefore potentially involved in normal cardiogenesis as well as in cardiac malformations observed in individuals with trisomy 21. PMID:22912673

  12. Right bundle branch block as a marker for interatrial septal abnormalities.

    Science.gov (United States)

    Bakalli, Aurora; Koçinaj, Dardan; Georgievska-Ismail, Ljubica; Bekteshi, Tefik; Pllana, Ejup; Sejdiu, Basri

    2012-02-01

    Interatrial septal anomalies, which include atrial septal defect, patent foramen ovale, and atrial septal aneurysm, are common disorders among adult patients. Early detection of interatrial septal anomalies is important in order to prevent haemodynamic consequences and/or thromboembolic events. Electrocardiogram offers some clues that should serve as hints for detection of interatrial abnormalities. The aim of our study was to analyse the interatrial septum by transoesophageal echocardiography in patients with electrocardiogram signs of right bundle branch block and in those without right bundle branch block. In a prospective study, 87 adult patients were included, that is, 41 with electrocardiogram signs of right bundle branch block forming the first group and 46 without right bundle branch block forming the second group. Interatrial septal anomalies were present in 80.5% of the patients with right bundle branch block, with patent foramen ovale (39.02%) being the most prevalent disorder, followed by atrial septal aneurysm (21.9%) and atrial septal defect (19.5%). Interatrial septal abnormalities were significantly more frequent in the first group compared with the second group (80.5% versus 6.5%, p value less than 0.001). Independently, patent foramen ovale was significantly more prevalent in patients with right bundle branch block (39.02% versus 4.3%, p value less than 0.001), as were atrial septal aneurysm (21.9% versus 2.2%, p value equal 0.01) and atrial septal defect (19.5% versus 0%, p value equal 0.004). Right bundle branch block should serve as a valuable indicator to motivate a detailed search for interatrial septal abnormalities.

  13. The first clinical experience with the new GORE® septal occluder (GSO)

    DEFF Research Database (Denmark)

    Søndergaard, Lars; Loh, Poay Huan; Franzen, Olaf

    2013-01-01

    Aims: A new GORE® septal occluder (GSO) was granted CE mark in Europe in June 2011 for the treatment of patent foramen ovale and atrial septal defect. Major changes have been made to the device and delivery system compared to the HELEX® device. The new delivery system has simplified...... the implantation procedure and the retrievability of the device after deployment if needed. The design of the GSO has improved the device apposition ability and tissue response whilst keeping its atraumatic design, low septal profile with minimal septal distortion and long-term biocompatibility. The first three...... of these patients had patent foramen ovale and one had secundum atrial septal defect. In all the cases, the GSO devices were successfully deployed in the first attempt without any complication. Only one patient had a minor residual shunt detected immediately after the device deployment. All the patients were re...

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

    Science.gov (United States)

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

    2016-03-01

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

  15. Congenital heart defects and parental occupational exposure to chemicals.

    Science.gov (United States)

    Snijder, Claudia A; Vlot, Ingrid J; Burdorf, Alex; Obermann-Borst, Sylvia A; Helbing, Willem A; Wildhagen, Mark F; Steegers, Eric A P; Steegers-Theunissen, Régine P M

    2012-05-01

    Congenital heart defects (CHDs) are the most common major malformations in newborns. In this study we examined the associations between the occurrence of CHDs in children and periconceptional occupational parental exposures to chemicals. In an age-matched case-control study with standardized data collection at c. 15 months after birth, 424 mothers and 421 fathers of a child with CHD and 480 mothers and 477 fathers of a non-malformed child, filled out questionnaires on periconceptional general and job characteristics. A job exposure matrix, which links the information on job title and a description of work tasks to an expert judgement on exposure to chemicals in the workplace, was used. The overall prevalence of occupational exposure to chemicals was 5.0 in cases and 6.2% in controls for mothers [odds ratio (OR) adjusted = 0.92; 95% confidence interval (CI): 0.26-3.25], while 22.3 and 15.9% for fathers, respectively (OR adjusted = 1.23; 95% CI: 0.39-3.91). No association of maternal occupational exposure to chemicals with risk of CHDs was found. Paternal exposure to phthalates was associated with a higher incidence of CHDs in general (OR adjusted = 2.08; 95% CI: 1.27-3.40). Paternal exposure to phthalates was associated with perimembranous ventricular septal defect (OR adjusted = 2.84; 95% CI: 1.37-5.92), to polychlorinated compounds with atrioventricular septal defect (OR adjusted = 4.22; 95% CI: 1.23-14.42) and to alkylphenolic compounds with coarctation of the aorta (OR adjusted = 3.85; 95% CI: 1.17-12.67). Periconceptional paternal (but not maternal) occupational exposure to certain chemicals is associated with an increased risk of CHDs in children. The results, however, must be interpreted cautiously as exposure probabilities are a crude measure of exposure.

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

    Science.gov (United States)

    Çelebi, Aksüyek Savaş; Amasyalı, Basri

    2017-04-01

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

  17. Septal myocardial perfusion imaging with thallium-201 in the diagnosis of proximal left anterior descending coronary artery disease

    Energy Technology Data Exchange (ETDEWEB)

    Pichard, A.D.; Wiener, I.; Martinez, E.; Horowitz, S.; Patterson, R.; Meller, J.; Goldsmith, S.J.; Gorlin, R.; Herman, M.V.

    1981-07-01

    The use of myocardial perfusion imaging (MPI) to identify obstructive coronary disease of the left anterior descending coronary artery proximal to the first septal perforator (prox LAD) was studied in 60 patients. Perfusion of the septum and anteroapical areas with thallium-201 injected during exercise was compared to results of coronary arteriography. Septal MPI defect was found in 92.3% of patients with obstruction of the proximal LAD, 27.7% of patients with obstruction of LAD distal to first septal perforator, 0% in patients with obstructions involving right or circumflex arteries, and in 10.5% of patients without coronary disease. Anteroapical MPI defects were found with similar frequency in the three groups with obstructive coronary disease. Septal MPI defect had a sensitivity of 92.3% and specificity of 85.4% in the diagnosis of proximal LAD disease. Normal septal perfusion with thallium-201 virtually excluded proximal LAD disease.

  18. Maternal injuries during the periconceptional period and the risk of birth defects, National Birth Defects Prevention Study, 1997-2005.

    Science.gov (United States)

    Tinker, Sarah C; Reefhuis, Jennita; Dellinger, Ann M; Jamieson, Denise J

    2011-09-01

    Maternal injuries during pregnancy are common (∼7% prevalence). However, few studies have examined the association between maternal injuries and birth defects. The National Birth Defects Prevention Study is a population-based case-control study of birth defects in 10 U.S. states. Cases were ascertained through surveillance; controls were randomly selected from infants delivered without major birth defects in the study regions. Mothers completed a telephone interview on exposures before and during pregnancy, including injuries. We assessed associations between periconceptional (month before until the end of the third month of pregnancy) maternal injuries and birth defects. We used logistic regression to estimate adjusted odds ratios (AORs) and 95% confidence intervals (CI). Periconceptional injuries were associated with interrupted aortic arch type B [AOR = 5.2, 95% CI 1.2, 23.2]; atrioventricular septal defect [AOR = 2.2, 95% CI 1.1, 4.4]; pulmonary atresia [AOR = 3.2, 95% CI 1.6, 6.4]; tricuspid atresia [AOR = 2.8, 95% CI 1.2, 6.7]; hypoplastic left heart syndrome [AOR = 2.0, 95% CI 1.1, 3.4]; anorectal atresia/stenosis [AOR = 1.7, 95% CI 1.0, 2.7]; longitudinal limb deficiency [AOR = 2.1, 95% CI 1.1, 3.9]; and gastroschisis [AOR = 1.8, 95% CI 1.2, 2.8]. Associations with longitudinal limb deficiency, gastroschisis and hypoplastic left heart syndrome were stronger for intentional injuries. Our results suggest maternal injury during the periconceptional period, particularly those inflicted intentionally, may be associated with select birth defects. This analysis was hypothesis-generating, with many associations tested. Further research is warranted. Published 2011. This article is a US Government work and is in the public domain in the USA.

  19. Association of interatrial septal abnormalities with cardiac impulse conduction disorders in adult patients: experience from a tertiary center in Kosovo.

    Directory of Open Access Journals (Sweden)

    Zaim Gashi

    2011-06-01

    Full Text Available Interatrial septal disorders, which include: atrial septal defect, patent foramen ovale and atrial septal aneurysm, are frequent congenital anomalies found in adult patients. Early detection of these anomalies is important to prevent their hemodynamic and/or thromboembolic consequences. The aims of this study were: to assess the association between impulse conduction disorders and anomalies of interatrial septum; to determine the prevalence of different types of interatrial septum abnormalities; to assess anatomic, hemodynamic, and clinical consequences of interatrial septal pathologies. Fifty-three adult patients with impulse conduction disorders and patients without ECG changes but with signs of interatrial septal abnormalities, who were referred to our center for echocardiography, were included in a prospective transesophageal echocardiography study. Intera trial septal anomalies were detected in around 85% of the examined patients.

  20. 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

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    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.

  1. Association of interatrial septal abnormalities with cardiac impulse conduction disorders in adult patients: experience from a tertiary center in Kosovo

    Science.gov (United States)

    Bakalli, Aurora; Pllana, Ejup; Koçinaj, Dardan; Bekteshi, Tefik; Dragusha, Gani; Gashi, Masar; Musliu, Nebih; Gashi, Zaim

    2011-01-01

    Interatrial septal disorders, which include: atrial septal defect, patent foramen ovale and atrial septal aneurysm, are frequent congenital anomalies found in adult patients. Early detection of these anomalies is important to prevent their hemodynamic and/or thromboembolic consequences. The aims of this study were: to assess the association between impulse conduction disorders and anomalies of interatrial septum; to determine the prevalence of different types of interatrial septum abnormalities; to assess anatomic, hemodynamic, and clinical consequences of interatrial septal pathologies. Fifty-three adult patients with impulse conduction disorders and patients without ECG changes but with signs of interatrial septal abnormalities, who were referred to our center for echocardiography, were included in a prospective transesophageal echocardiography study. Interatrial septal anomalies were detected in around 85% of the examined patients. Patent foramen ovale was encountered in 32% of the patients, and in combination with atrial septal aneurysm in an additional 11.3% of cases. Atrial septal aneurysm and atrial septal defect were diagnosed with equal frequency in 20.7% of our study population. Impulse conduction disorders were significantly more suggestive of interatrial septal anomalies than clinical signs and symptoms observed in our patients (84.91% vs 30.19%, P=0.002). Right bundle branch block was the most frequent impulse conduction disorder, found in 41 (77.36%) cases. We conclude that interatrial septal anomalies are highly associated with impulse conduction disorders, particularly with right bundle branch block. Impulse conduction disorders are more indicative of interatrial septal abnormalities in earlier stages than can be understood from the patient’s clinical condition. PMID:21977304

  2. Association of interatrial septal abnormalities with cardiac impulse conduction disorders in adult patients: experience from a tertiary center in Kosovo.

    Science.gov (United States)

    Bakalli, Aurora; Pllana, Ejup; Koçinaj, Dardan; Bekteshi, Tefik; Dragusha, Gani; Gashi, Masar; Musliu, Nebih; Gashi, Zaim

    2011-01-01

    INTERATRIAL SEPTAL DISORDERS, WHICH INCLUDE: atrial septal defect, patent foramen ovale and atrial septal aneurysm, are frequent congenital anomalies found in adult patients. Early detection of these anomalies is important to prevent their hemodynamic and/or thromboembolic consequences. The aims of this study were: to assess the association between impulse conduction disorders and anomalies of interatrial septum; to determine the prevalence of different types of interatrial septum abnormalities; to assess anatomic, hemodynamic, and clinical consequences of interatrial septal pathologies. Fifty-three adult patients with impulse conduction disorders and patients without ECG changes but with signs of interatrial septal abnormalities, who were referred to our center for echocardiography, were included in a prospective transesophageal echocardiography study. Interatrial septal anomalies were detected in around 85% of the examined patients. Patent foramen ovale was encountered in 32% of the patients, and in combination with atrial septal aneurysm in an additional 11.3% of cases. Atrial septal aneurysm and atrial septal defect were diagnosed with equal frequency in 20.7% of our study population. Impulse conduction disorders were significantly more suggestive of interatrial septal anomalies than clinical signs and symptoms observed in our patients (84.91% vs 30.19%, P=0.002). Right bundle branch block was the most frequent impulse conduction disorder, found in 41 (77.36%) cases. We conclude that interatrial septal anomalies are highly associated with impulse conduction disorders, particularly with right bundle branch block. Impulse conduction disorders are more indicative of interatrial septal abnormalities in earlier stages than can be understood from the patient's clinical condition.

  3. The contribution of chromosomal abnormalities to congenital heart defects: a population-based study.

    Science.gov (United States)

    Hartman, Robert J; Rasmussen, Sonja A; Botto, Lorenzo D; Riehle-Colarusso, Tiffany; Martin, Christa L; Cragan, Janet D; Shin, Mikyong; Correa, Adolfo

    2011-12-01

    We aimed to assess the frequency of chromosomal abnormalities among infants with congenital heart defects (CHDs) in an analysis of population-based surveillance data. We reviewed data from the Metropolitan Atlanta Congenital Defects Program, a population-based birth-defects surveillance system, to assess the frequency of chromosomal abnormalities among live-born infants and fetal deaths with CHDs delivered from January 1, 1994, to December 31, 2005. Among 4430 infants with CHDs, 547 (12.3%) had a chromosomal abnormality. CHDs most likely to be associated with a chromosomal abnormality were interrupted aortic arch (type B and not otherwise specified; 69.2%), atrioventricular septal defect (67.2%), and double-outlet right ventricle (33.3%). The most common chromosomal abnormalities observed were trisomy 21 (52.8%), trisomy 18 (12.8%), 22q11.2 deletion (12.2%), and trisomy 13 (5.7%). In conclusion, in our study, approximately 1 in 8 infants with a CHD had a chromosomal abnormality. Clinicians should have a low threshold at which to obtain testing for chromosomal abnormalities in infants with CHDs, especially those with certain types of CHDs. Use of new technologies that have become recently available (e.g., chromosomal microarray) may increase the identified contribution of chromosomal abnormalities even further.

  4. A randomized comparison of permanent septal versus apical right ventricular pacing: short-term results.

    Science.gov (United States)

    Victor, Frederic; Mabo, Philippe; Mansour, Hassan; Pavin, Dominique; Kabalu, Guillaume; de Place, Christian; Leclercq, Christophe; Daubert, J Claude

    2006-03-01

    This study compared chronic right ventricular (RV) pacing at the septum versus apex. Chronic RV apical pacing may be detrimental to ventricular function. This randomized, pilot study examined whether, compared with apical, permanent septal pacing preserves cardiac function. Ablation of the atrioventricular junction for permanent AF, followed by implantation of a DDDR pacemaker connected to two ventricular leads was performed in 28 patients. One lead screwed into the septum and another placed at the apex were connected to the atrial and ventricular port, respectively. Septum or apex was paced by programming AAIR or VVIR modes, respectively. Patients were randomly assigned, 4 months later, to pacing at one site for 3 months, and crossed over to the other for 3 months. New York Heart Association class, QRS width and axis, left ventricular ejection fraction (LVEF), exercise duration, and peak oxygen uptake were measured. Results in patients with LVEF > 45% and < or = 45% were compared. Septal pacing was associated with shorter QRS (145 +/- 4 msec vs 170 +/- 4 msec, P < 0.01) and normal axis (40 degrees +/- 10 degrees vs -71 +/- 4 degrees , P < 0.01). At 3 months, among patients with baseline LVEF < or = 45%, LVEF was 42 +/- 5% after septal pacing versus 37 +/- 4% after apical pacing (P < 0.001). In contrast to RV apical pacing, chronic RV septal pacing preserved LVEF in patients with baseline LVEF < or = 45%.

  5. Eventos catastróficos associados ao tratamento da comunicação interatrial tipo ostium secundum: razões para não se subestimar este tipo de cardiopatia congênita Catastrophic events associated to the surgical treatment of ostium secundum atrial septal defects: reasons for not underestimating this type of congenital cardiopathy

    Directory of Open Access Journals (Sweden)

    Paulo Roberto B. Evora

    2004-12-01

    Full Text Available OBJETIVO: O presente trabalho clínico foi motivado pela frustrante experiência de quatro pacientes operados para o tratamento cirúrgico da comunicação interatrial tipo ostium secundum (CIA-II, que vieram a falecer em condições extremamente dramáticas. MÉTODO: Estudo retrospectivo embasado em dados de prontuários. As pesquisas bibliográficas incluíram: tromboembolismo paradoxal (cerebral, pulmonar ou mesentérico, malformações vasculares do sistema nervoso central e conexões anômalas das veias cavas. Estas pesquisas da literatura foram embasadas em possíveis eventos, inesperados e catastróficos, que levaram quatro pacientes ao óbito. RESULTADOS: Os quatros pacientes, todos do sexo feminino, foram submetidos a atriosseptorrafia com tempo de parada cardíaca isquêmica inferior a 20 minutos, em circulação extracorpórea. As causas de óbito foram: isquemia intestinal não-oclusiva, ruptura de aneurisma cerebral de artéria comunicante anterior, cor pulmonale com hipertensão arterial e tromboembolismo e um provável tromboembolismo cerebral em uma criança que precisou ser reoperada pela drenagem da veia cava inferior em átrio esquerdo. CONCLUSÃO: A lição final deste trabalho é: "Não subestime a comunicação interatrial em cirurgia cardíaca!".OBJECTIVE: The present article was motivated by the frustrating experiences with four patients who underwent surgical treatment of ostium secundum atrial septal defect (ASD-II and who died in extremely dramatic circumstances. METHOD: This is a retrospective study based on clinical data. The bibliographical researche included: paradoxical thromboembolism (cerebral, lung or mesenteric, central nervous system vascular malformations and anomalous vena cava connections. This research was based on possible events, unexpected and catastrophic, that could have directly caused the patients' deaths. RESULTS: All patients were female, the operations were performed under cardiopulmonary

  6. Entrainment to distinguish orthodromic reciprocating tachycardia from atrioventricular nodal reentry tachycardia in children.

    Science.gov (United States)

    Kannankeril, Prince J; Bonney, William J; Dzurik, Matthew V; Fish, Frank A

    2010-04-01

    Studies in adults suggest that after entrainment from the right ventricle, a post-pacing interval (PPI) minus tachycardia cycle length (TCL), when corrected for atrioventricular node delay (cPPI-TCL), is useful to distinguish atrioventricular nodal reentry tachycardia (AVNRT) from orthodromic reciprocating tachycardia (ORT), but this has not been evaluated in children. In 100 children undergoing catheter ablation, entrainment of ORT or AVNRT was performed from the right ventricular apex. The atrial-His (AH) interval was measured on the return cycle (post-AH) and during tachycardia just prior to pacing (pre-AH). The cPPI-TCL was calculated as (PPI-TCL) - (post-AH - pre-AH). In the first 50 children, the best cutoff was identified and then validated in the next 50 children. In the first 50 children, cPPI-TCL was longer in AVNRT compared with ORT (122 +/- 19 ms vs 63 +/- 23 ms, P cPPI-TCL exceeded 95 ms in all AVNRT patients, but was less than cPPI-TCL cPPI-TCL > 95 ms was 95% specific for AVNRT. There was even greater separation of cPPI-TCL values comparing AVNRT with ORT utilizing a septal accessory pathway. The cPPI-TCL is a useful technique to distinguish AVNRT from ORT in children. Our data suggest that in children a cPPI-TCL 95 ms is rarely observed in ORT. This technique is particularly useful to distinguish AVNRT from ORT utilizing a septal accessory pathway. (PACE 2010; 469-474).

  7. Surgical treatment of atrial septal aneurysm.

    Science.gov (United States)

    Wos, S; Bachowski, R; Domaradzki, W; Jasinski, M; Matuszewski, M; Ceglarek, W; Deja, M

    1996-12-01

    The atrial septal aneurysm (ASA) is a morphologic abnormality known to cause peripheral and pulmonary embolism. 28-52% patients with ASA have embolic events. However ASA -- with no other concomittant cardiac patology has rarely been reported as the indication for open heart surgery. In this work, five cases of patients operated for ASA are presented. Embolic complications with cerebral symptoms were presented in three cases. The diagnosis of ASA was established with the use of transesophageal echocardiography. The surgical correction of the defect was performed in extracorporeal circulation. The aneurysmal part of interatrial septum was excised and replaced with a pericardial patch. The postoperative course was uneventful. The patients have no new embolic events during the follow up period of one year.

  8. Glue septal ablation: A promising alternative to alcohol septal ablation

    Directory of Open Access Journals (Sweden)

    Sercan Okutucu

    2016-03-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is defined as myocardial hypertrophy in the absence of another cardiac or systemic disease capable of producing the magnitude of present hypertrophy. In about 70% of patients with HCM, there is left ventricular outflow tract (LVOT obstruction (LVOTO and this is known as obstructive type of hypertrophic cardiomyopathy (HOCM. Cases refractory to medical treatment have had two options either surgical septal myectomy or alcohol septal ablation (ASA to alleviate LVOT gradient. ASA may cause some life-threatening complications including conduction disturbances and complete heart block, hemodynamic compromise, ventricular arrhythmias, distant and massive myocardial necrosis. Glue septal ablation (GSA is a promising technique for the treatment of HOCM. Glue seems to be superior to alcohol due to some intrinsic advantageous properties of glue such as immediate polymerization which prevents the leak into the left anterior descending coronary artery and it is particularly useful in patients with collaterals to the right coronary artery in whom alcohol ablation is contraindicated. In our experience, GSA is effective and also a safe technique without significant complications. GSA decreases LVOT gradient immediately after the procedure and this reduction persists during 12 months of follow-up. It improves New York Heart Association functional capacity and decrease interventricular septal wall thickness. Further studies are needed in order to assess the long-term efficacy and safety of this technique.

  9. Atrial tachycardia mimicking atrioventricular nodal reentry tachycardia.

    Science.gov (United States)

    Eilbert, Wesley P; Patel, Neal

    2013-07-01

    The term supraventricular tachycardia (SVT) is used to describe tachydysrhythmias that require atrial or atrioventricular nodal tissue for their initiation and maintenance. SVT can be used to describe atrioventricular nodal reentry tachycardia, atrioventricular reentry tachycardia, and atrial tachycardia (AT). AT is the least common of these SVT subtypes, accounting for only 10% of cases. Although the suggested initial management of each SVT subtype is different, they all can present with similar symptoms and electrocardiographic findings. Discuss the pathophysiology, diagnosis, and treatment of AT as compared with other types of SVT. We report a 56-year-old woman with symptoms and electrocardiographic findings consistent with SVT. Although standard treatment with intravenous adenosine failed to convert the SVT, it revealed AT as the cause of the tachydysrhythmia. The AT was successfully terminated with beta-blockade and the patient eventually underwent successful radioablation of three separate AT foci. AT frequently mimics other more common forms of SVT. AT might be recognized only when standard treatment of SVT has failed. Identification of AT in this setting is crucial to allow for more definitive therapy. Copyright © 2013 Elsevier Inc. All rights reserved.

  10. The sinus venosus myocardium contributes to the atrioventricular canal: potential role during atrioventricular node development?

    Science.gov (United States)

    Kelder, Tim P; Vicente-Steijn, Rebecca; Harryvan, Tom J; Kosmidis, Georgios; Gittenberger-de Groot, Adriana C; Poelmann, Rob E; Schalij, Martin J; DeRuiter, Marco C; Jongbloed, Monique R M

    2015-06-01

    The presence of distinct electrophysiological pathways within the atrioventricular node (AVN) is a prerequisite for atrioventricular nodal reentrant tachycardia to occur. In this study, the different cell contributions that may account for the anatomical and functional heterogeneity of the AVN were investigated. To study the temporal development of the AVN, the expression pattern of ISL1, expressed in cardiac progenitor cells, was studied in sequential stages performing co-staining with myocardial markers (TNNI2 and NKX2-5) and HCN4 (cardiac conduction system marker). An ISL1+/TNNI2+/HCN4+ continuity between the myocardium of the sinus venosus and atrioventricular canal was identified in the region of the putative AVN, which showed a pacemaker-like phenotype based on single cell patch-clamp experiments. Furthermore, qPCR analysis showed that even during early development, different cell populations can be identified in the region of the putative AVN. Fate mapping was performed by in ovo vital dye microinjection. Embryos were harvested and analysed 24 and 48 hrs post-injection. These experiments showed incorporation of sinus venosus myocardium in the posterior region of the atrioventricular canal. The myocardium of the sinus venosus contributes to the atrioventricular canal. It is postulated that the myocardium of the sinus venosus contributes to nodal extensions or transitional cells of the AVN since these cells are located in the posterior region of the AVN. This finding may help to understand the origin of atrioventricular nodal reentrant tachycardia. © 2015 The Authors. Journal of Cellular and Molecular Medicine published by John Wiley & Sons Ltd and Foundation for Cellular and Molecular Medicine.

  11. Late recovery of surgically-induced atrioventricular block in patients with congenital heart disease.

    Science.gov (United States)

    Bruckheimer, Elchanan; Berul, Charles I; Kopf, Gary S; Hill, Sharon L; Warner, Kenneth A; Kleinman, Charles S; Rosenfeld, Lynda E; Nehgme, Rodrigo A

    2002-06-01

    To assess the incidence and establish possible predictors of late recovery of post-surgical heart block, treated with pacemaker implantation, in patients with congenital heart defects. The American College of Cardiology/American Heart Association Task Force has recommended pacemaker implantation for advanced second or third degree atrioventricular block which persists for 7 to 14 days after surgery. The incidence of late recovery of post-surgical heart block following pacemaker implantation has not been reported. Records of 44 patients with post-surgical heart block who underwent pacemaker implantation at our institutions since 1976 were reviewed for demographic, anatomic, surgical and surface electrocardiographic data to assess the incidence of, and factors associated with, recovery of atrioventricular conduction on long-term follow-up. 32% (14) of patients recovered atrioventricular conduction at a median follow-up of 5.5 years while 68% (30) remained pacemaker dependent. The groups were similar in age and weight at surgery and period of follow-up p = 0.5). Types of defect and surgical repair were not significantly different (p > 0.1). There was a similar number of patients with second degree-type II block in both groups (p = 0.15). The groups did not differ in timing of pacemaker implantation (14 days p = 0.18). Late recovery of atrioventricular conduction following pacemaker implantation for postsurgical heart block is common. However, clinical predictors, with reference to current recommendations, could not be identified. Prospective electrophysiologic evaluations may be warranted to establish guidelines for long term pacemaker dependency and criteria for pacing.

  12. Canonical Wnt Signaling Regulates Atrioventricular Junction Programming and Electrophysiological Properties

    Science.gov (United States)

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

    2014-01-01

    Rationale Proper patterning of the atrioventricular canal (AVC) is essential for delay of electrical impulses between atria and ventricles, and defects in AVC maturation can result in congenital heart disease. Objective To determine the role of canonical Wnt signaling in the myocardium during AVC development. Methods and Results We utilized a novel allele of β-catenin that preserves β-catenin’s cell adhesive functions but disrupts canonical Wnt signaling, allowing us to probe the effects of Wnt loss of function independently. We show that loss of canonical Wnt signaling in the myocardium results in tricuspid atresia with hypoplastic right ventricle associated with loss of AVC myocardium. In contrast, ectopic activation of Wnt signaling was sufficient to induce formation of ectopic AV junction-like tissue as assessed by morphology, gene expression, and electrophysiologic criteria. Aberrant AVC development can lead to ventricular preexcitation, a characteristic feature of Wolff-Parkinson-White syndrome. We demonstrate that postnatal activation of Notch signaling downregulates canonical Wnt targets within the AV junction. Stabilization of β-catenin protein levels can rescue Notch-mediated ventricular preexcitation and dysregulated ion channel gene expression. Conclusions Our data demonstrate that myocardial canonical Wnt signaling is an important regulator of AVC maturation and electrical programming upstream of Tbx3. Our data further suggests that ventricular preexcitation may require both morphologic patterning defects, as well as myocardial lineage reprogramming, to allow robust conduction across accessory pathway tissue. PMID:25599332

  13. Tachycardia induction with ventricular extrastimuli differentiates atypical atrioventricular nodal reentrant tachycardia from orthodromic reciprocating tachycardia.

    Science.gov (United States)

    Obeyesekere, Manoj; Gula, Lorne J; Modi, Simon; Leong-Sit, Peter; Angaran, Paul; Mechulan, Alexis; Skanes, Allan C; Krahn, Andrew D; Yee, Raymond; Klein, George J

    2012-03-01

    Differentiating atypical atrioventricular nodal reentrant tachycardia (AVNRT) from septal orthodromic reentrant tachycardia (ORT(Septal)) is challenging in nonsustained tachycardia. When sustained, the postpacing interval minus tachycardia cycle length following entrainment (PPI(Entrainment) - TCL) and stimulation to atrial interval minus ventriculoatrial interval (Stim-A(Entrainment) - VA) are utilized. We hypothesized that the first tachycardia cycle after tachycardia induction with right ventricular apical extrastimulation would yield comparable information to entrainment, precluding the need for sustained tachycardia. Twenty-four patients with AVNRT (age 47 ± 18 years), 19 with ORT(Septal) (age 42 ± 17 years), and 15 with ORT over a left lateral accessory pathway (ORT(Left)) (age 41 ± 16 years) were included. The ventricular extrastimulus to atrial depolarization at tachycardia initiation (Stim-A(Initiation)) and tachycardia VA interval were measured to establish the Stim-A(Initiation) minus VA interval (Stim-A(Initiation) - VA). The ventricular extrastimulus to the subsequent right ventricular apical depolarization (postpacing interval at initiation, PPI(Initiation)) was utilized to obtain the PPI(Initiation) minus TCL (PPI(Initiation) - TCL). The AH interval associated with the PPI(Initiation) minus the AH in tachycardia was utilized to establish a corrected PPI(Initiation) minus TCL (cPPI(Initiation) - TCL). The intervals after tachycardia initiation were longer for AVNRT than for ORT: mean PPI(Initiation) - TCL (193 ± 44 vs 91 ± 73; P cPPI(Initiation) - TCL (174 ± 44 ms vs 88 ± 50 ms; P cPPI(Initiation) minus TCL against PPI(Entrainment) minus TCL was 0.71. cPPI(Initiation) minus TCL cPPI(Initiation) - TCL < 115 ms excludes AVNRT. Copyright © 2012 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

  14. Perinatal outcomes and congenital heart defect prognosis in 53313 non-selected perinatal infants.

    Science.gov (United States)

    Xie, Donghua; Wang, Hua; Liu, Zhiyu; Fang, Junqun; Yang, Tubao; Zhou, Shujin; Wang, Aihua; Qin, Jiabi; Xiong, Lili

    2017-01-01

    To evaluate perinatal outcomes and congenital heart defect (CHD) prognosis in a non-selected population. The population-based surveillance data used in this assessment of CHDs were based on birth defect surveillance data collected from 2010-2012 in Liuyang City, China. Infants living with CHDs were followed up for 5 years to determine their prognosis. Prevalence, prenatal diagnosis, perinatal outcomes, and total and type-specific prognosis data were assessed using SPSS 18.0. In total, 190 CHD cases were identified among the 53313 included perinatal infants (PIs), indicating a CHD prevalence of 35.64 per 10000 PIs in this non-selected population. The five most frequently identified types of CHDs were ventricular septal defects (VSDs, 38.95%), atrial septal defects (ASDs, 15.79%), cardiomegaly (7.89%), tetralogy of Fallot (TOF, 5.79%), and atrioventricular septal defects (AVSDs, 5.26%). Of the 190 CHD cases, 110 (57.89%) were diagnosed prenatally, 30 (15.79%) were diagnosed with associated malformations, and 69 (36.32%) resulted in termination of pregnancy (TOP). Moreover, 15 (7.89%) PIs died within 7 days after delivery, and 42 (22.10%) died within 1 year. In contrast, 79 (41.58%) were still alive after 5 years. When TOP cases were included, the 5-year survival rate of PIs with prenatally detected CHDs was lower than that of PIs with postnatally detected CHDs (25.45% vs. 63.75%). The CHD subtype associated with the highest rate of infant (less than 1 year old) mortality was transposition of the great arteries (100%). The subtypes associated with higher 5-year survival rates were patent ductus arteriosus (80%), ASD (63.33%), VSD (52.70%) and AVSD (50%). The rates of prenatal CHD detection and TOP were high in this study population, and the 5-year survival rate of PIs with CHDs was low. The government should strengthen efforts to educate pediatricians regarding this issue and provide financial assistance to improve the prognosis of infants living with CHDs, especially

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

    Science.gov (United States)

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

    2017-02-01

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

  16. [Complete atrioventricular block during galantamine therapy].

    Science.gov (United States)

    Leentjens, A F G; Kragten, J A

    2006-03-11

    A 77-year-old man who for 5 months had been being treated with galantamine for early Alzheimer's disease, suddenly became unwell. He was bradycardic and his ECG showed a complete atrioventricular block. This was treated by implantation of a pacemaker. Treatment with galantamine was continued. A month later he had no cardiac symptoms and his dementia score on a cognitive scale had improved. Cholinesterase inhibitors may have adverse effects on the heart. Patients starting treatment with cholinesterase inhibitors should be screened for an increased risk of cardiac arrhythmias by investigating their potassium concentrations and running an ECG.

  17. Endoscopic closure of septal perforations.

    Science.gov (United States)

    Alobid, Isam

    2017-05-26

    The management of septal perforations is a challenge for the surgeon. A wide variety of surgical techniques have been described, with different approaches. There is no scientific evidence to support a particular approach. The objective of this review is to present a practical guide on the technique of choice for each case of septal perforation. Inspection of the nasal mucosa, the size of the perforation, the location and especially the osteo-cartilaginous support, are the pillars of a successful surgery. For the sliding or rotating flaps of the mucosa of the septum it is essential to know in advance if the elevation of the mucopericondrio or mucoperiosteo of the septum is possible, otherwise the use of these flaps would not be indicated. The flaps of the lateral wall or nasal floor are the alternative. The pericranial flap may be indicated in total or near total perforations. The remnant of the nasal septum and status of osteo-cartilaginous support are the determining factors in the management of septal perforations. Each case should be evaluated individually and the approach chosen according to the size and location of the perforation, mucosal quality, personal history, previous surgery and the experience of the surgeon. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

  18. Prognostic importance of complete atrioventricular block complicating acute myocardial infarction

    DEFF Research Database (Denmark)

    Aplin, Mark; Engstrøm, Thomas; Vejlstrup, Niels G

    2003-01-01

    Third-degree atrioventricular block after acute myocardial infarction is considered to have prognostic importance. However, its importance in conjunction with thrombolytic therapy and its relation to left ventricular function remains uncertain. This report also outlines an important distinction...... between atrioventricular block in the setting of anterior and inferior wall acute myocardial infarction, with profound clinical and prognostic implications....

  19. Vector electrocardiographic alterations after percutaneous septal ablation in obstructive hypertrophic cardiomyopathy: possible anatomic causes

    Directory of Open Access Journals (Sweden)

    Pérez Riera Andrés Ricardo

    2002-01-01

    Full Text Available OBJECTIVE: Analyze the dromotropic disturbances (vector-electrocardiographic, and the possible anatomic causes, provoked by selective alcohol injection in the septal branch, for percutaneous treatment, of obstructive hypertrophic cardiomyopathy. METHODS: Ten patients with a mean age of 52.7 years underwent percutaneous septal ablation (PTSA from october 1998; all in functional class III/IV. Twelve-lead electrocardiogram was performed prior to and during PTSA, and later electrocardiogram and vectorcardiogram according to Frank's method. The patients were followed up for 32 months. RESULTS: On electrocardiogram (ECG prior to PTSA all patients had sinus rhythm and left atrial enlargement, 8 left ventricular hypertrophy of systolic pattern. On ECG immediately after PTSA, 8 had complete right bundle-branch block; 1 transient total atrioventricular block; 1 alternating transient bundle-branch block either right or hemiblock. On late ECG 8 had complete right bundle-branch block confirmed by vectorcardiogram, type 1 or Grishman. CONCLUSION: Septal fibrosis following alcohol injection caused a predominance of complete right bundle-branch block, different from surgery of myotomy/myectomy.

  20. Vertical zonality of septal nectaries of Monocots

    Directory of Open Access Journals (Sweden)

    Аnastasiya Odintsova

    2013-04-01

    Full Text Available Considering the septal nectary as a system of exogenous cavities inside the ovary and taking account of possibilities of various ways of the formation of nectary walls we propose to apply the concept of vertical zonality to the analysis of the septal nectary structure. The comparative analysis of the gynoecium with septal nectaries must include data about the nectary vertical zones and its location in the structural zones of the gynoecium.

  1. Incidental Discovery of a Membranous Ventricular Septal Aneurysm in Two Dissimilar Patients

    Directory of Open Access Journals (Sweden)

    Abhishek Naidu

    2012-01-01

    Full Text Available A ventricular septal aneurysm (VSA is a rare cardiac anomaly, and an accurate statistic of its prevalence has not been reported in the literature. True incidence is likely underestimated as most patients are thought to be asymptomatic. As a result, most VSAs are discovered incidentally on echocardiography, during angiography, or at autopsy. Potential complications include rupture, bacterial endocarditis, right ventricular outflow tract obstruction, and thromboembolic disease. It has been proposed that VSAs occur in association with ventricular septal defects (VSDs and other congenital cardiac abnormalities. It is uncommon for a VSA to exist in the absence of a known prior ventricular septal defect. We present two cases, each highlighting an incidental intact aneurysm involving the membranous interventricular septum. We discuss the contrast in the two patients with regard to their age, accompanying cardiac anomalies and cardiovascular fitness. Clinical implications of the condition are reviewed.

  2. Using optical coherence tomography to rapidly phenotype and quantify congenital heart defects associated with prenatal alcohol exposure.

    Science.gov (United States)

    Karunamuni, Ganga; Gu, Shi; Doughman, Yong Qiu; Noonan, Amanda I; Rollins, Andrew M; Jenkins, Michael W; Watanabe, Michiko

    2015-04-01

    The most commonly used method to analyze congenital heart defects involves serial sectioning and histology. However, this is often a time-consuming process where the quantification of cardiac defects can be difficult due to problems with accurate section registration. Here we demonstrate the advantages of using optical coherence tomography, a comparatively new and rising technology, to phenotype avian embryo hearts in a model of fetal alcohol syndrome where a binge-like quantity of alcohol/ethanol was introduced at gastrulation. The rapid, consistent imaging protocols allowed for the immediate identification of cardiac anomalies, including ventricular septal defects and misaligned/missing vessels. Interventricular septum thicknesses and vessel diameters for three of the five outflow arteries were also significantly reduced. Outflow and atrioventricular valves were segmented using image processing software and had significantly reduced volumes compared to controls. This is the first study to our knowledge that has 3D reconstructed the late-stage cardiac valves in precise detail to examine their morphology and dimensions. We believe, therefore, that optical coherence tomography, with its ability to rapidly image and quantify tiny embryonic structures in high resolution, will serve as an excellent and cost-effective preliminary screening tool for developmental biologists working with a variety of experimental/disease models. © 2014 Wiley Periodicals, Inc.

  3. Early pregnancy exposure to antihistamines and risk of congenital heart defects: results of two case-control studies.

    Science.gov (United States)

    Smedts, Huberdina P M; de Jonge, Linda; Bandola, Sarah J G; Baardman, Marlies E; Bakker, Marian K; Stricker, Bruno H C; Steegers-Theunissen, Régine P M

    2014-09-01

    We aimed to study the association between use of antihistamines in early pregnancy and congenital heart defects (CHD) in the offspring. Two case-control studies. HAVEN study, Erasmus MC, University Medical Centre, Rotterdam, and Eurocat Northern Netherlands (NNL), University Medical Center Groningen, Groningen, the Netherlands. We studied 361 children with CHD and 410 controls without congenital malformations from the HAVEN study and replicated the analyses in 445 children with CHD and 530 controls from the Eurocat NNL registry. Information about antihistamine use in early pregnancy and potential confounders was obtained from questionnaires postpartum. We calculated the association between antihistamines and CHD risk by multivariable logistic regression analysis. Odds ratios (OR) with 95% confidence intervals (CI). In the HAVEN study, 25 of 771 mothers used antihistamines that were associated with an increased CHD risk (OR 3.0, 95% CI 1.2-7.3), particularly atrioventricular septal defects (AVSD) (OR 5.1, 95 % CI 1.3-20.5) and perimembranous ventricular septal defects (pVSD) (OR 5.1, 95% CI 1.8-14.4). Mothers with severe nausea who did not use antihistamines had a reduced risk (OR 0.7, 95% CI 0.5-0.98), whereas nauseous mothers using antihistamines showed an almost fivefold increased risk of pVSD (OR 4.8, 95% CI 1.1-21.8). The association between antihistamines and AVSD was confirmed in the Eurocat cohort (OR 3.5, 95% CI 1.4-8.7), but we could not replicate the association with overall CHD risk. We found a positive association between antihistamine use in early pregnancy and CHD risk, particularly AVSD, which seemed to be independent of nausea/vomiting.

  4. Atrial septal aneurysm associated with additional cardiovascular comorbidities in two middle age female patients with ECG signs of right bundle branch block: two case reports.

    Science.gov (United States)

    Bakalli, Aurora; Kamberi, Lulzim; Pllana, Ejup; Gashi, Afrim

    2008-07-19

    Atrial septal aneurysm (ASA) is often associated with other atrial septal abnormalities, particularly with atrial septal defect type ostium secundum or patent foramen ovale. ECG signs of incomplete or complete right bundle branch block are known to be associated with atrial septal defects, however such correlation with other atrial septal abnormalities is not documented. We report here two cases of middle age female patients that presented with dyspnea on physical effort, right bundle branch block (RBBB) on ECG and ASA combined with other cardiac disorders. Transesophageal echocardiography revealed additional information to the ones obtained by surface echocardiography, in both cases. ASA associated with RBBB on ECG may serve as a hint for the presence of additional cardiac abnormalities, thus rousing the demand for a detailed cardiac investigation.

  5. Influence of thrombolytic therapy on the patterns of ventricular septal rupture after acute myocardial infarction

    OpenAIRE

    Rhydwen, G; Charman, S; Schofield, P

    2002-01-01

    Background: Post-myocardial infarction ventricular septal defect (VSD) complicates ∼2% of myocardial infarctions. Thrombolytic therapy may accelerate the time from myocardial infarction to VSD formation. The effects of thrombolytic therapy in patients with a post-myocardial infarction VSD were investigated.

  6. Fluorinated steroids do not improve outcome of isolated atrioventricular block

    NARCIS (Netherlands)

    Van den Berg, N. W. E.; Slieker, M. G.; van Beynum, I. M.; Bilardo, C. M.; de Bruijn, D.; Clur, S. A.; Cornette, J. M. J.; Frohn-Mulder, I. M. E.; Haak, M. C.; van Loo-Maurus, K. E. H.; Manten, G. T. R.; Rackowitz, A. B. M. H.; Rammeloo, L. A. J.; Reimer, A.; Rijlaarsdam, M. E. B.; Freund, M. W.

    2016-01-01

    Introduction: Congenital atrioventricular block (CAVB) is a rare disorder with a significant morbidity and mortality. Consensus regarding the prescription and efficacy of prenatal corticosteroids is lacking. This nationwide study was initiated to evaluate the effects of prenatal treatment with

  7. Swallow syncope caused by third-degree atrioventricular block

    DEFF Research Database (Denmark)

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

    2015-01-01

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

  8. Atrio-ventricular block requiring pacemaker in patients with late onset Pompe disease.

    Science.gov (United States)

    Sacconi, Sabrina; Wahbi, Karim; Theodore, Guillaume; Garcia, Jérémy; Salviati, Leonardo; Bouhour, Françoise; Vial, Christophe; Duboc, Denis; Laforêt, Pascal; Desnuelle, Claude

    2014-07-01

    Enzyme replacement therapy consistently improves cardiac function in infantile and juvenile onset patients with Pompe disease and cardiomyopathy, but is apparently not effective in preventing rhythm disorders, an emerging cardiac phenotype in long term survivors. In patients with late onset Pompe disease cardiomyopathy is an exceptional finding while heart rhythm disorders seem to be more frequent. We retrospectively identified, among a cohort of 131 French late onset Pompe disease patients, four patients with severe atrio-ventricular blocks requiring pacemaker implantation. These patients had no other risk factors for cardiovascular diseases or cardiomyopathy. In one patient the atrioventricular block was discovered while still asymptomatic. Cardiac conduction defects are relatively rare in late onset Pompe disease and may occur even in absence of cardiac symptoms or EKG abnormalities. However because of the possible life-threatening complications associated with these conduction defects, cardiac follow-up in patients with late onset Pompe disease should include periodical Holter-EKG monitoring. Copyright © 2014 Elsevier B.V. All rights reserved.

  9. Assessment of atrioventricular conduction following cryoablation of atrioventricular nodal reentrant tachycardia in children.

    Science.gov (United States)

    Kiplapinar, Neslihan; Ergul, Yakup; Akdeniz, Celal; Saygi, Murat; Ozyilmaz, Isa; Gul, Enes E; Tuzcu, Volkan

    2014-06-01

    Early-onset transient atrioventricular block (AVB) is a rare occurrence following cryoablation of atrioventricular nodal reentrant tachycardia (AVNRT), despite lack of any AVB at the end of the procedure. The purpose of this prospective study was to assess AVB shortly after successful cryoablation of AVNRT in children. A 6-mm-tip cryocatheter was used in 39 procedures. An 8-mm-tip catheter was used in 11 procedures. Twelve-lead electrocardiograms (ECGs) and 24-hour ambulatory ECGs were performed 24 hours prior to the procedure and immediately following the procedure. All procedures were done using the EnSite system (St. Jude Medical, St. Paul, MN, USA) without fluoroscopy. Although nine (18%) patients developed variable degrees of transient AVB during the procedure, all of them had normal atrioventricular (AV) conduction at the end of the procedure and did not require any intervention. Four of these patients had variable degrees of transient AVB following the procedure despite having normal AV conduction at the end of the procedure. One developed Mobitz type I AVB, which lasted for 11.5 hours, and the other three experienced 2:1 AVB, which lasted for 2, 8, and 24 hours, respectively. All patients had complete resolution of the AVB, which was also documented with the 24-hour ambulatory ECGs after the procedure. Early transient AVB can develop following AVNRT cryoablation even if AV conduction is normal at the end of the procedure. Despite the transient AVB in the initial 24 hours after the procedure in some cases, there is no evidence for ongoing AV nodal dysfunction. ©2014 Wiley Periodicals, Inc.

  10. Humeral septal aperture associated with supracondylar process: a case report and review of the literature.

    Science.gov (United States)

    Paraskevas, George K; Natsis, Konstantinos; Anastasopoulos, Nikolaos; Ioannidis, Orestis; Kitsoulis, Panagiotis

    2012-01-01

    The supracondylar process is usually a beak-like osseous prominence located at the anteromedial aspect of the distal portion of the humerus. It is usually asymptomatic but occasionally may compress underlying structures such as the median or ulnar nerve, the brachial artery or its branches. The term septal aperture defines an oval or round shaped bony defect of the septum that separates the olecranon from the coronoid fossa of the humerus. It is of significance for surgeons because it may alter the fracture pattern at the region and thus their management. We present a rare case of coexistence of supracondylar process and septal aperture in a macerated left humerus. The reported incidence of the supracondylar process alone varies from 0.28% to 2.78%, while that of the septal aperture from 6.9% to 60%. We have reviewed the literature and emphasized the radiological and surgical significance of the findings.

  11. Isorhythmic atrioventricular dissociation in Labrador Retrievers.

    Science.gov (United States)

    Perego, M; Ramera, L; Santilli, R A

    2012-01-01

    Isorhythmic atrioventricular dissociation (IAVD) is a rhythm disturbance in which atria and ventricles are driven by independent pacemakers at equal or nearly equal rates. To describe electrocardiographic and electrophysiologic features of IAVD in a group of 11 Labrador Retrievers and its possible correlation with focal junctional tachycardia (FJT). Between December 2004 and October 2010, medical records of 11 Labrador Retrievers with surface electrocardiographic findings compatible with IAVD were retrospectively analyzed. Twelve-lead surface electrocardiograms, thoracic radiographs, and echocardiographic findings of each dog and electrophysiologic mapping results of 3 dogs were retrospectively analyzed. In 10 of 11 dogs, the ECG pattern revealed the presence of IAVD with type I synchronization. In 5 of 10 dogs, IAVD with type I synchronization was interrupted by periods of junctional tachycardia with 1 : 1 ventriculo-atrial conduction. One of 11 dogs presented IAVD with type II synchronization. The ECG diagnosis of IAVD with type I and type II synchronization, and junctional rhythm with 1 : 1 ventriculo-atrial conduction was confirmed in 3 of 11 dogs with endocardial mapping in which the diagnosis of focal junctional tachycardia was made. IAVD with type I synchronization is more common than IAVD with type II synchronization in Labrador Retrievers, and a correlation between IAVD and FJT can be hypothesized. Copyright © 2012 by the American College of Veterinary Internal Medicine.

  12. Brucella Infection Associated with Complete Atrioventricular Block.

    Science.gov (United States)

    Bilici, Meki; Demir, Fikri; Yılmazer, Murat Muhtar; Bozkurt, Fatma; Tuzcu, Volkan

    2016-09-01

    The clinical spectrum of Brucella infection is quite diverse and characterized by multi-system involvement. Patients present with myocarditis, endocarditis, or pericarditis. Infective endocarditis is the most common cardiovascular complication in patients with brucellosis. Although conduction abnormalities are seen in cases with endocarditis, they are reported very rarely in the setting of cardiac Brucella infection. An eight and a half-year-old male patient was referred to our clinic due to inadequate response to cotrimaxazole plus streptomycin treatment at the 15th day of admission. Although local hospital records on the patient showed a heart rate of 80 bpm, we determined a heart rate of 46 bpm. The electrocardiogram showed complete atrioventricular (AV) block. The average heart rate was determined as 48 bpm with 24-hour Holter electrocardiogram (ECG) monitoring. The echocardiographic examination showed normal-sized heart chambers and the absence of valvular involvement. An agglutination test for brucellosis was found to be positive with a titer of 1/320. High fever, arthralgia, and splenomegaly regressed following doxycycline plus rifampicin therapy, but there was no improvement in the AV block. A permanent pacemaker was implanted because of the detection of an average heart rate of 48 bpm. Because cardiac failure and rhythm abnormalities are reported in the course of Brucella infection and may be associated with significant outcomes, cases with brucellosis should be evaluated carefully in terms of cardiac involvement. This report aims to draw attention to complete AV block as an extremely rare complication of Brucella infection.

  13. A Simple Method to Differentiate Atrioventricular Node Reentrant Tachycardia from Orthodromic Reciprocating Tachycardia.

    Science.gov (United States)

    He, Quan; Lei, Sen; Jia, Feng-Peng; Gao, Ling-Yun; W X Zhu, Dennis

    2018-01-27

    Discrimination between atrioventricular node reentry tachycardia (AVNRT) and orthodromic reciprocating tachycardia (ORT) during an electrophysiological study is sometimes challenging. This study aimed to investigate if the difference in the local VA (ventricle-atrium) interval during ventricular entrainment pacing and during tachycardia (DVA, defined as the shortest local VA interval of coronary sinus [CS] during entrainment minus the shortest local VA interval of CS during tachycardia) was different in patients with AVNRT and patients with ORT.Diagnoses of AVNRT or ORT through a concealed accessory pathway (AP) were made according to conventional electrophysiological criteria and ablation results. Entrainment by right ventricular (RV) pacing was performed in each patient before ablation and patients with successful entrainment were included in the study. The DVA was compared between patients with AVNRT and patients with ORT. The DVA in patients with AVNRT was significantly longer than that in patients with ORT (120 ± 20 versus 5.7 ± 9; P DVA was more than 48 ms. In each patient with ORT using a left free wall accessory pathway (AP), right free wall AP, and septal AP, the DVA was less than 20 ms.DVA was found to be a rapid, useful test in distinguishing patients with AVNRT from those with ORT.

  14. A Rare Association of Takotsubo Cardiomyopathy with High-Degree Atrioventricular Block

    Directory of Open Access Journals (Sweden)

    Eder Hans Cativo

    2017-01-01

    Full Text Available Here we present a case of a patient who got trapped in an elevator; on initial evaluation patient was found with bradycardia; on further evaluation electrocardiogram (EKG showed new onset 2nd-degree Mobitz type 2 AV block. On admission patient developed ischemic changes on EKG and troponin elevation. Transthoracic echocardiogram showed reduced ejection fraction as well as apical inferior, anterior, lateral, and septal hypokinesia. Coronary angiography showed nonobstructive coronary artery disease and ventriculogram demonstrated anterolateral and apical hypokinesia suggesting takotsubo cardiomyopathy (TCM. Atrioventricular block (AV is rarely seen as initial presentation of TCM and has a prevalence of about 2.9%. AV block during early presentation of TCM poses a therapeutic dilemma with regard to the timing and the need to place a temporary or permanent pacemaker. The decision to place a permanent pacemaker may be on a case-by-case basis and more research is needed on formulating standardized recommendations in patients with TCM and conduction tissue abnormalities.

  15. The development of pacing induced ventricular dysfunction is influenced by the underlying structural heart defect in children with congenital heart disease.

    Science.gov (United States)

    Balaji, Seshadri; Sreeram, Narayanswami

    Right ventricular pacing can cause pacing-induced ventricular dysfunction (PIVD) correctable with biventricular pacing (BiVP). Factors associated with PIVD are poorly understood. We reviewed children receiving epicardial dual-chamber pacemakers for complete heart block (CHB) after congenital heart disease (CHD) surgery. PIVD was defined as% fractional shortening hearts and underwent epicardial dual chamber pacemaker implantation. Nine of the 47 (19%) developed PIVD. PIVD occurred in 0/10 with ventricular septal defect (VSD), 0/6 with tetralogy of Fallot, 2/6 with double outlet right ventricle, 2/6 with transposition and VSD, 3/9 with atrioventricular canal defect, 1/2 with mitral valve replacement; 1/3 with congenitally corrected TGA repair; and 0/3 with atrioventricular canal plus tetralogy of Fallot and 0/1 with subaortic membrane. QRS duration (QRSD) was 84-170 (median 135ms) in the non PIVD group and 100-168 (median 124) ms in the PIVD group. Percentage fractional shortening (%FS) while paced was 16-46, median 30% in the non-PIVD group and 6-15 (median 11%) in the PIVD group.%FS post upgrade to BiVP (with an epicardial LV lead) in the 9 patients with PIVD was 23-33 (median 29%). PIVD occurred in certain CHD but not others. Prolonged QRSD was not associated with PIVD. The predilection for RV pacing to result in PIVD in certain types of CHD needs further study. Copyright © 2016. Published by Elsevier B.V.

  16. Morphological aspects of atrioventricular valves in the ostrich (Struthio camelus

    Directory of Open Access Journals (Sweden)

    Marco A. Pereira-Sampaio

    2013-11-01

    Full Text Available Heart anatomy in the ostrich has been reported, but there are few information on the histological features of the atrioventricular valves. Hearts of young ostriches were fixed in 10% formaldehyde for 24 h and dissected to characterize their macroscopic anatomy. Samples of valves were harvested and stained with Mallory’s trichrome, Gomori’s trichrome, and Picro-Sirius red, for later analysis. The right atrioventricular valve consists of a muscle flap with two fixations. The left atrioventricular valve consists of two layers of endocardium with a layer of connective tissue between them. The free border of the tricuspid valve supports a varying number of chordae tendineae. One of the cusps is attached to the septum, while the other two cusps are attached to the opposite wall. The aortic valve, as well as the pulmonary trunk valve, consists of three cusps. The right atrioventricular valve showed up only as a muscle flap of myocardium coated with a thin layer of dense connective tissue, with two fixations. In the connective tissue, we find a predominance of type I collagen fibers and a lesser amount of type III, with a small presence of elastic fibers. The presence of Purkinje fibers were also usual in the valvular subendocardium, suggesting that they directly participate in the transmission of nervous stimulation to the muscle fibers within the valves. The left atrioventricular valve consisted of 3 cusps, a dorsal, a left, and a right.

  17. Human fingernail as interpositional graft material in the treatment of nasal septal perforations.

    Science.gov (United States)

    Akçal, Arzu; Karsidag, Semra; Ozkaya, Ozay; Sirvan, Selami Serhat; Sevim, Kamuran Zeynep; Kabukcuoglu, Fevziye

    2014-04-01

    The etiology of nasal septal perforations involves iatrogenic, traumatic, inflammatory, infectious, neoplastic, and caustic causes. To ensure successful closure, an appropriate interpositional graft material should be selected, and this graft material should be covered with healthy tissue. The study included 18 New Zealand white rabbits weighing 2 to 2.5 kg. Nasal septal perforations were created in group 1. After the creation of defects in group 2, repair was performed with cartilage graft and bilateral mucoperichondrial advancement flaps. After septal nasal perforations in group 3, the defect was covered with fingernail and bilateral mucoperichondrial flaps. At week 12, the rabbits were sacrificed. The septum site that had been repaired with fingernail was intact. No nail exposition, wound site decomposition, or re-perforation was observed. No findings of a breach of the structural integrity of the fingernails or disintegration were encountered. Fingernails can be used as an interpositional graft material in place of cartilage in eligible cases for the repair of nasal septal perforations. Fingernails have several properties that enable their use in such cases, such as form preservation that is similar to cartilage, the lack of live cells, easy availability, and a lack of donor-site morbidity at removal. Copyright © 2014 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

  18. The role of temporalis fascia for free mucosal graft survival in small nasal septal perforation repair.

    Science.gov (United States)

    Jeon, Eun-Ju; Choi, Jin; Lee, Joo-Hyung; Kim, Sung-Won; Nam, In-Chul; Park, Yong-Su; Jin, Sang-Gyun; Cheon, Byung-Jun

    2014-01-01

    Temporalis fascia has been used widely as a interposition graft for mucosal rotation flap in nasal septal perforation repair. However, the exact role of temporalis fascia in healing process has not yet been clarified. For the pedicle of rotation flap has been considered as a major vehicle for nutrition distribution, the role of temporalis fascia has been devaluated. In this study, we experienced small nasal septal perforation repairs using free mucosal graft not having pedicles but covered by temporalis fascia. Three patients with small nasal septal perforations not larger than 1 × 1 cm were included. In 2 patients, the perforations were repaired using free composite grafts from the inferior turbinate mucosa covered by continuous temporalis fascia not divided, and the surgical results were successful with complete healings. In 1 patient, however, the temporalis fascia was divided into 2 parts to better fit the shape of the perforation, and the graft failed to survive. These surgical results suggest that the temporalis fascia might have an important role in healing process of nasal septal defect and could be used as a beneficial options for small mucosal defect repair surgeries using free mucosal grafts.

  19. Right ventricular electrical and mechanical synchronization by properly timed septal pacing in a patient with right bundle branch block and first degree AV block--a case report.

    Science.gov (United States)

    Siliste, Calin; Suran, Maria-Claudia-Berenice; Margulescu, Andrei-Dumitru; Vinereanu, Dragos

    2015-03-01

    We present a case of near-normalization of the QRS by septal pacing in a patient with dual-chamber pacemaker and underlying complete right bundle branch block and first degree atrioventricular block. The right ventricular mechanical synchronization suggested by the ECG was validated as such by strain echo. To the best of our knowledge, this is the first time it has been shown that the narrowing of the QRS corresponds to mechanical synchronization in a case of this seldom-recognized phenomenon. © 2014 Wiley Periodicals, Inc.

  20. Influence of Septal Thickness on the Clinical Outcome After Alcohol Septal Alation in Hypertrophic Cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten K; Jacobsson, Linda; Almaas, Vibeke Marie

    2016-01-01

    BACKGROUND: We assessed the influence of interventricular septal thickness (IVSd) on the clinical outcome and survival after alcohol septal ablation (ASA) in patient with hypertrophic cardiomyopathy. METHODS AND RESULTS: We analyzed 531 patients with hypertrophic cardiomyopathy (age: 56±14 years...

  1. Familial Atrial Septal Defect and Sudden Cardiac Death

    DEFF Research Database (Denmark)

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

    2016-01-01

    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.......1 × 10(-9) ). The majority of patients (74%) had ASD with conduction disturbance. Nineteen patients (15%) of 120 with familial ASD and conduction disturbance died from sudden cardiac death of which nine (8%) were confirmed mutation carriers, and 10 were possible carriers. CONCLUSIONS: NKX2-5 mutations...... mainly occur in familial CHD, the signature phenotype is ASD with conduction disturbances and mutation carriers are at increased risk of sudden cardiac death. We suggest that familial ASD patients should be screened for NKX2-5 mutations and, if they are mutation carriers, implantation of an implantable...

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

    African Journals Online (AJOL)

    In Down's syndrome, 95% of all cases are caused by non disjunction: one cell has two 21st chromosomes instead of one, so the resulting fertilized egg has three. 21st chromosomes. Hence the scientific name, trisomy. 21. The cause of the non disjunction error isn't known, but there is definitely connection with maternal age.

  3. Transcatheter device closure of postmyocardial infarction ventricular septal defect

    Directory of Open Access Journals (Sweden)

    You-Lin Nie

    2017-01-01

    Conclusion: Our findings indicate that transcatheter device closure of PMIVSD is technically feasible, safe, and effective to reduce the shunt. The crucial prognostic factors were ascertained to be age ≥ 80 years, systolic blood pressure ≤ 90 mmHg, and procedure time ≥180 minutes.

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

    African Journals Online (AJOL)

    Date of Acceptance: 20‑Jul‑2014. Address for correspondence: Dr. OO Ogunkunle,. Department of ... a large VSD in the muscular portion measuring 2.57 cm. [Figure 1]. At the time, the connection between the ... also, the absence of clinical symptoms suggestive of a large VSD prior to the trauma in our patient, should have.

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

    African Journals Online (AJOL)

    2014-07-20

    Jul 20, 2014 ... He presented 11 days later with features of acute congestive cardiac failure. ... trauma to the chest. Any such patient should undergo careful echocardiographic evaluation. There is an ... complained of chest pains. At a private ...

  6. Straddling tricuspid valve without a ventricular septal defect.

    OpenAIRE

    Isomatsu, Y; Kurosawa, H.; Imai, Y

    1989-01-01

    A four year old girl with pulmonary atresia had a straddling tricuspid valve without an interventricular communication. The overriding tricuspid valve had two orifices, which connected with the right and the left ventricles. Valve tissue separated both orifices and was firmly connected to the crest of the ventricular septum, thus sealing off the expected interventricular communication. Surgical correction was performed and the outcome was satisfactory.

  7. Straddling tricuspid valve without a ventricular septal defect.

    Science.gov (United States)

    Isomatsu, Y; Kurosawa, H; Imai, Y

    1989-09-01

    A four year old girl with pulmonary atresia had a straddling tricuspid valve without an interventricular communication. The overriding tricuspid valve had two orifices, which connected with the right and the left ventricles. Valve tissue separated both orifices and was firmly connected to the crest of the ventricular septum, thus sealing off the expected interventricular communication. Surgical correction was performed and the outcome was satisfactory.

  8. Utility of dual source CT with ECG-triggered high-pitch spiral acquisition (Flash Spiral Cardio mode) to evaluate morphological features of ventricles in children with complex congenital heart defects.

    Science.gov (United States)

    Nakagawa, Motoo; Ozawa, Yoshiyuki; Nomura, Norikazu; Inukai, Sachiko; Tsubokura, Satoshi; Sakurai, Keita; Shimohira, Masashi; Ogawa, Masaki; Shibamoto, Yuta

    2016-04-01

    We evaluated the ability of dual source CT (DSCT) with ECG-triggered high-pitch spiral acquisition (Flash Spiral Cardio mode) to depict the morphological features of ventricles in pediatric patients with congenital heart defects (CHD). Between July 2013 and April 2015, 78 pediatric patients with CHD (median age 4 months) were examined using DSCT with the Flash Spiral Cardio mode. The types of ventricular abnormalities were ventricular septal defect (VSD) in 42 (the malaligned type in 11, perimembranous type in 23, supracristal type in 2, atrioventricular type in 2, and muscular type in 4), single ventricle (SV) in 11, and congenital corrected transposition of the great arteries (ccTGA) in 4. We evaluated the accuracy of the diagnosis of the VSD type. In cases of SV and ccTGA, we assessed the detectability of the anatomical features of both ventricles for a diagnosis of ventricular situs. DSCT confirmed the diagnoses for all VSDs. The type of defect was precisely diagnosed for all patients. The anatomical features of both ventricles were also depicted and ventricular situs of SV and ccTGA was correctly diagnosed. The results suggest that DSCT has the ability to clearly depict the configuration of ventricles.

  9. Alcohol septal ablation in patients with hypertrophic obstructive cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten K; Prinz, Christian; Horstkotte, Dieter

    2013-01-01

    The infarction induced by alcohol septal ablation (ASA) may predispose to arrhythmia and sudden cardiac death (SCD).......The infarction induced by alcohol septal ablation (ASA) may predispose to arrhythmia and sudden cardiac death (SCD)....

  10. A Mobitz type II atrioventricular block in multicentric ischemic stroke ...

    African Journals Online (AJOL)

    A Mobitz type II atrioventricular block in multicentric ischemic stroke. Utku Murat Kalafat, Canan Akman, Turker Karaboga, Tarik Ocak. Abstract. Cardiac and cerebrovascular illnesses are major causes of mortality and morbidity. Thromboembolisms, which are the result of cardiac arrhythmia, are important causes of ischemic ...

  11. A Mobitz type II atrioventricular block in multicentric ischemic stroke

    Science.gov (United States)

    Kalafat, Utku Murat; Akman, Canan; Karaboga, Turker; Ocak, Tarik

    2016-01-01

    Cardiac and cerebrovascular illnesses are major causes of mortality and morbidity. Thromboembolisms, which are the result of cardiac arrhythmia, are important causes of ischemic stroke. In this study, we present a rare case of multicentric ischemic stroke induced by Mobitz type II atrioventricular block. PMID:28154620

  12. Age changes in the structure of human Atrioventricular annuli | El ...

    African Journals Online (AJOL)

    Atrioventricular annuli are important in hemodynamic stability and support to tricuspid and mitral valves. Anatomical features of the annuli such as circumference, organization of connective tissue fibers, myocardium and cellularity may predispose to annular insufficiency and valvular incompetence. These pathologies ...

  13. Swallow syncope caused by third-degree atrioventricular block.

    Science.gov (United States)

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

    2015-10-27

    We report a case of a patient with more than 30 years of repeated syncopes, always following food intake. The patient was diagnosed with a swallow-related third-degree atrioventricular block and successfully treated with an artificial pacemaker. 2015 BMJ Publishing Group Ltd.

  14. Morphological aspects of atrioventricular valves in the ostrich (Struthio camelus

    Directory of Open Access Journals (Sweden)

    Marcelo Abidu-Figueiredo

    2013-08-01

    Full Text Available http://dx.doi.org/10.5007/2175-7925.2013v26n4p203 Heart anatomy in the ostrich has been reported, but there are few information on the histological features of the atrioventricular valves. Hearts of young ostriches were fixed in 10% formaldehyde for 24 h and dissected to characterize their macroscopic anatomy. Samples of valves were harvested and stained with Mallory’s trichrome, Gomori’s trichrome, and Picro-Sirius red, for later analysis. The right atrioventricular valve consists of a muscle flap with two fixations. The left atrioventricular valve consists of two layers of endocardium with a layer of connective tissue between them. The free border of the tricuspid valve supports a varying number of chordae tendineae. One of the cusps is attached to the septum, while the other two cusps are attached to the opposite wall. The aortic valve, as well as the pulmonary trunk valve, consists of three cusps. The right atrioventricular valve showed up only as a muscle flap of myocardium coated with a thin layer of dense connective tissue, with two fixations. In the connective tissue, we find a predominance of type I collagen fibers and a lesser amount of type III, with a small presence of elastic fibers. The presence of Purkinje fibers were also usual in the valvular subendocardium, suggesting that they directly participate in the transmission of nervous stimulation to the muscle fibers within the valves. The left atrioventricular valve consisted of 3 cusps, a dorsal, a left, and a right.

  15. Virtual Cardiac Surgery Using CFD: Application to Septal Myectomy in Obstructive Hypertrophic Cardiomyopathy

    Science.gov (United States)

    Vedula, Vijay; Mittal, Rajat; Abraham, Theodore

    2011-11-01

    Obstructive hypertrophic cardiomyopathy (HOCM) is characterized by ventricular wall thickening, diastolic dysfunction, and dynamic outflow tract obstruction, all of which strongly influence the vortex dynamics and pressure distribution in the left ventricle (LV). Severe cases of HCM are usually managed through septal myectomy where the surgeon resects the hypertrophic mass. Surgeons currently try to remove as much tissue as possible in order to optimize the post surgical result. However, excessive debulking increases the chance of ventricular septal defects, bundle branch block or complete heart block, and aneurysmal septal thinning. On the other hand, insufficient tissue removal also leads to unsatisfactory outcomes in terms of reduction of outflow tract pressure gradient. Knowing how much muscle to remove and where to remove it from could reduce the likelihood of complications and suboptimal outcomes. In the present study, we employ an immersed boundary solver to model the effect of septal myectomy for ventricles with HOCM and demonstrate the potential of such an approach for surgical planning. Computational resources were provided by the National Institute of Computational Science under Tergrid grant number TG-CTS100002.

  16. The Tbx2(+) Primary Myocardium of the Atrioventricular Canal Forms the Atrioventricular Node and the Base of the Left Ventricle

    NARCIS (Netherlands)

    Aanhaanen, W.T.J.; Brons, J.F.; Domínguez, J.N.; Rana, M.S.; Norden, J.; Airik, R.; Wakker, V.; de Gier-de Vries, C.; Brown, N.A.; Kispert, A.; Moorman, A.F.M.; Christoffels, V.M.

    2009-01-01

    The primary myocardium of the embryonic heart, including the atrioventricular canal and outflow tract, is essential for septation and valve formation. In the chamber-forming heart, the expression of the T-box transcription factor Tbx2 is restricted to the primary myocardium. To gain insight into the

  17. Improved septal contraction and coronary flow velocity after cardiac resynchronization therapy elucidated by strain imaging and pulsed wave Doppler echocardiography.

    Science.gov (United States)

    Kayano, Hiroyuki; Ueda, Hiroaki; Kawamata, Tomoaki; Miyoshi, Fumito; Toshida, Tsutomu; Watanabe, Norikazu; Hirano, Yuichi; Kawamura, Mitsuharu; Asano, Taku; Kou, Shyhaku; Tanno, Kaoru; Ozawa, Masaki; Kobayashi, Youichi; Katagiri, Takashi

    2006-02-01

    The effects of cardiac resynchronization therapy (CRT) with various atrioventricular conduction delay settings were investigated on cardiac hemodynamic changes involved in coronary flow velocity using color and pulsed wave Doppler modalities and myocardial regional contractility using a novel echocardiographic technique (strain imaging). Seven patients with advanced heart failure (left ventricular ejection fraction or = 140 msec) were treated with CRT. Color and pulsed wave Doppler imaging were performed from the apical four-chamber view to examine the cardiac functions such as stroke volume, cardiac output, mitral regurgitant volume and coronary flow velocity. Strain imaging was performed to quantify the asynchrony of both intraventricular and interventricular time delay between the septum and left ventricular free wall (posterior wall) and to assess the regional contractile function. Wall motion was also evaluated. Intraventricular and interventricular asynchrony were improved from 173 +/- 18 to 60 +/- 6 msec, and 69 +/- 25 to 12 +/- 3 msec, respectively. Stroke volume (55.2 +/- 6.2 to 76.8 +/- 10.8 ml; 39% up), cardiac output (3.9 +/- 0.3 to 5.4 +/- 0.5 I/min; 38% up) and coronary flow velocity (24 +/- 3 to 36 +/- 5 cm/sec; 50% up) were greatly increased and mitral regurgitant volume (59.7 +/- 18.0 to 38.9 +/- 11.3 ml; 35% down)was clearly decreased. Septal wall shortening was greatly increased from 10.2 +/- 2.3% to 17.0 +/- 1.8% and septal wall motion (radial thickening)was also improved simultaneously. Atrioventricular interval settings influenced all above parameters. CRT improved the cardiac hemodynamics involved in coronary flow significantly due to both resynchronization of inter and intra asynchrony, and improvement of the regional myocardial contraction in patients with severe congestive heart failure and complete left bundle branch block.

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

    Science.gov (United States)

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

    2014-09-01

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

  19. Marked First Degree Atrioventricular Block: an extremely prolonged PR interval associated with Atrioventricular Dissociation in a young Nigerian man with Pseudo-Pacemaker Syndrome: a case report.

    Science.gov (United States)

    Ogunlade, Oluwadare; Akintomide, Anthony O; Ajayi, Olufemi E; Eluwole, Omotayo A

    2014-11-04

    The diagnosis of Marked First Degree Atrioventricular Block is made with electrocardiogram when PR interval ≥0.30 s. A PR interval of up to 0.48 s had been reported in literature. Data is sparse on an extremely prolonged PR interval associated with Atrioventricular Dissociation and Pseudo-Pacemaker Syndrome. Electrocardiogram with this type of uncommon features poses diagnostic and management challenges in clinical practice. We report a case of a 22 year old Nigerian male from Igbo ethnic group who presented himself for medical screening with a history of exercise intolerance, occasional palpitation and fainting spells. He has no history of cough, orthopnoea, paroxysmal nocturnal dyspnoea nor body swelling. A physical examination revealed that the patient has a pulse rate of 64 beats per minute, blood pressure of 110/70 mmHg and soft heart sounds. Standard 12-lead electrocardiogram showed an uncommon Marked First Degree Atrioventricular Block with an extremely prolonged PR interval of 0.56 s. Long rhythm strips of the electrocardiogram showed extremely prolonged PR interval associated with Atrioventricular Dissociation and variable degrees of Atrioventricular Block (Mobitz type I and II). An extremely prolonged PR interval may occur in First Degree Atrioventricular Block and it may be associated with Atrioventricular Dissociation and Pseudo-Pacemaker Syndrome which may pose diagnostic and management challenges. This suggests that not all cases of First Degree Atrioventricular Block are benign and so should be sub-classified based on degree of PR interval prolongation and associated electrical abnormalities.

  20. Minimally invasive septal myectomy for the treatment of hypertrophic obstructive cardiomyopathy and intrinsic mitral valve disease.

    Science.gov (United States)

    Gilmanov, Daniyar Sh; Bevilacqua, Stefano; Solinas, Marco; Ferrarini, Matteo; Kallushi, Enkel; Santarelli, Philippo; Farneti, Pier Andrea; Glauber, Mattia

    2015-01-01

    Transaortic left ventricular septal myectomy described by Morrow is a classical procedure for the treatment of systolic anterior motion of the mitral apparatus associated with hypertrophic obstructive cardiomyopathy (HOCM). We aimed to review our results of transmitral septal myectomy and mitral valve repair/replacement in patients with intrinsic mitral valve disease associated with HOCM, operated on through a minimally invasive approach. Between 2005 and 2014, 19 patients [7 men (37%); mean (SD) age, 69.4 (14.5) years] were treated with minimally invasive approach for degenerative mitral regurgitation and HOCM. Preoperative peak left ventricular outflow tract (LVOT) gradient was 66 (24) mm Hg. Severe mitral regurgitation was diagnosed in 16 cases (84%). New York Heart Association functional class III to IV heart failure was present in 13 patients (68%). Fifteen patients (79%) underwent mitral valve replacement, and four patients (21%) underwent mitral valve repair. Left ventricular outflow tract obstruction was corrected directly in all patients via the mitral valve with septal myectomy/myotomy, avoiding aortotomy in majority of the patients. No significant prolongation of extracorporeal circulation/aortic cross-clamping times was observed (P = 0.41 and P = 0.67, respectively) when compared with a similar population without HOCM. No iatrogenic ventricular septal defect developed in treated patients. No hospital mortality occurred. Resting LVOT gradient reduced at discharge to 13 (22) mm Hg (P = 0.025). Transmitral left ventricular septal myectomy in patients with degenerative mitral valve disease is quite a simple, feasible, and effective technique and does not require aortotomy in most cases. It can be performed with low early mortality and satisfactory resolution of LVOT obstruction in a minimally invasive setting.

  1. Effects of enhanced parasympathetic tone on atrioventricular nodal conduction during atrioventricular nodal reentrant tachycardia.

    Science.gov (United States)

    Belz, M K; Stambler, B S; Wood, M A; Pherson, C; Ellenbogen, K A

    1997-10-01

    The effects of various physiologic and pharmacologic stimuli on the anterograde slow pathway in patients with atrioventricular nodal reentrant tachycardia are well characterized. We sought to further characterize the nature of anterograde and retrograde conduction during tachycardia and to define the differential input of the parasympathetic nervous system to these pathways. A custom-made neck suction collar was placed to stimulate the carotid body baroreceptors during supraventricular tachycardia. Neck suction at -60 mm Hg was applied and changes in tachycardia cycle length, AH, and ventriculoatrial intervals were measured in 20 patients. These measurements were repeated after intravenous administration of 10 mg of edrophonium to enhance vagal tone. We observed a 15 +/- 6 ms increase in tachycardia cycle length from baseline (p <0.0001) and a 14 +/- 6 ms increase in AH interval (p <0.0001), but no change in the VA interval with neck suction alone. The tachycardia cycle length prolonged 26 +/- 55 ms (p <0.0001) with edrophonium and an additional 12 +/- 43 ms (p <0.001) with neck suction after edrophonium. There was no change in the VA interval before or after edrophonium during neck suction. There were 10 tachycardia terminations in 8 patients during anterograde slow pathway block during neck suction, with tachycardia cycle length prolongation and mean AH prolongation before termination of 45 +/- 37 ms (vs 15 +/- 7 ms increase in AH interval without tachycardia termination, p = 0.10). There were 12 tachycardia terminations in 4 patients with retrograde block during neck suction, only after edrophonium, without any preceding change in tachycardia cycle length during 11 episodes. We conclude that anterograde slow pathway demonstrates gradual conduction slowing with parasympathetic enhancement, whereas retrograde fast pathway responds with abrupt block.

  2. Prenatal diagnosis of congenital heart defects: accuracy and discrepancies in a multicenter cohort.

    Science.gov (United States)

    van Velzen, C L; Clur, S A; Rijlaarsdam, M E B; Pajkrt, E; Bax, C J; Hruda, J; de Groot, C J M; Blom, N A; Haak, M C

    2016-05-01

    To examine the accuracy of fetal echocardiography in diagnosing congenital heart disease (CHD) at the fetal medicine units of three tertiary care centers. This was a multicenter cohort study of tertiary echocardiography referrals between 2002 and 2012. Prenatal and postnatal diagnoses were compared and the degree of agreement was classified as 'correct' (anatomy correct and the postnatal diagnosis led to a similar outcome as expected), 'discrepant' (anatomical discrepancies present but the severity and prognosis of the defect were diagnosed correctly) or 'no similarity' (the pre- and postnatal diagnoses differed completely). We included 708 cases with CHD for which both prenatal and postnatal data were available. The prenatal diagnosis was correct in 82.1% of cases and discrepancies present were present in 9.9%; however, these did not result in a different outcome. In 8.1% there was no similarity between prenatal and postnatal diagnoses. Disagreement between pre- and postnatal diagnoses occurred significantly more frequently in cases that presented with a normal four-chamber view than in those with an abnormal four-chamber view (5.5% vs 1.9%). Incorrect identification of the outflow tracts and incorrect differentiation between unbalanced atrioventricular septal defect and hypoplastic left heart syndrome were relatively commonly encountered. In many cases with disagreement, trisomy 21, extracardiac anomaly or a high maternal body mass index was present. The prenatal diagnosis and estimated prognosis of fetal echocardiography in our tertiary referral centers were appropriate in 92% of cases. Some types of CHD remain difficult to diagnose or rule-out prenatally, therefore awareness and education are of considerable importance. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

  3. Complete Atrioventricular Block in an Adolescent With Rheumatic Fever

    OpenAIRE

    Yoo, Gyeong-Hee

    2009-01-01

    Rheumatic fever is an acute inflammatory sequela following a group A, ?-hemolytic streptococcal infection. Rheumatic fever is characterized by polyarthritis, carditis, chorea, subcutaneous nodules, and erythema marginatum as the major diagnostic criteria. Rarely, advanced heart block may also occur. A 13-year-old boy was admitted to the Pediatric Department for evaluation and management of complete atrioventricular block. The patient had exertional dyspnea for 1 month. Based on the findings o...

  4. Ion channel transcript expression at the rabbit atrioventricular conduction axis.

    Science.gov (United States)

    Greener, Ian D; Tellez, James O; Dobrzynski, Halina; Yamamoto, Mitsuru; Graham, Gillian M; Billeter, Rudi; Boyett, Mark R

    2009-06-01

    Little is known about the distribution of gap junctions and ion channels in the atrioventricular node, even though the physiology and pathology of the atrioventricular node is ultimately dependent on them. The abundance of 30 transcripts for markers, gap junctions, ion channels, and Ca(2+)-handling proteins in different regions of the rabbit atrioventricular node (nodal extension and proximal and distal penetrating bundle of His as well as atrial and ventricular muscle) was measured using a novel quantitative polymerase chain reaction technique and in situ hybridization. The expression profile of the nodal extension (slow pathway into penetrating bundle) was similar to that of the sinoatrial node. For example, in the nodal extension, in contrast to the atrial muscle and as expected for a slowly conducting tissue with pacemaker activity, there was no or reduced expression of Cx43, Na(v)1.5, Ca(v)1.2, K(v)1.4, KChIP2, and RYR3 and high expression of Ca(v)1.3 and HCN4. The expression profile of the penetrating bundle was less specialized. In situ hybridization revealed a transitional zone with reduced expression of Cx43, Na(v)1.5, and KChIP2 that may form the fast pathway into the penetrating bundle. At the atrioventricular node, the expression of gap junctions and ion channels in the nodal extension (slow pathway) and a transitional zone (putative fast pathway) as well as the penetrating bundle (output pathway) is specialized and heterogeneous and roughly matches the electrophysiology of the different regions.

  5. Complete atrioventricular block in an adolescent with rheumatic Fever.

    Science.gov (United States)

    Yoo, Gyeong-Hee

    2009-03-01

    Rheumatic fever is an acute inflammatory sequela following a group A, beta-hemolytic streptococcal infection. Rheumatic fever is characterized by polyarthritis, carditis, chorea, subcutaneous nodules, and erythema marginatum as the major diagnostic criteria. Rarely, advanced heart block may also occur. A 13-year-old boy was admitted to the Pediatric Department for evaluation and management of complete atrioventricular block. The patient had exertional dyspnea for 1 month. Based on the findings of mitral regurgitation, fever, elevated acute phase reactants, and a high antistreptolysin O titer, the patient was diagnosed with rheumatic fever. A benzathine penicillin injection was administered, as well as salicylate therapy. On the 5(th) day of hospitalization, the electrocardiogram revealed a normal sinus rhythm with a 1(st) degree atrioventricular block. After discharge, the electrocardiogram normalized with the monthly penicillin injections. Herein we report a case of complete atrioventricular block associated with rheumatic fever. The heart block resolved without specific cardiac treatment, other than a non-steroidal anti-inflammatory medication.

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

    Energy Technology Data Exchange (ETDEWEB)

    Belhocine, Ouardia; Andre, Christine; Kalifa, Gabriel; Adamsbaum, Catherine [St Vincent de Paul Hospital, Radiology Department, Paris (France)

    2005-04-01

    Primary septal agenesis (PSA) is a rare brain malformation that can be isolated or part of developmental brain abnormalities (holoprosencephaly, septo-optic dysplasia or cortical malformation). Such associated malformation can be subtle, leading to difficulties in the prenatal management of PSA. Moreover, the neurological prognosis of isolated PSA remains debatable. The aims of the study were to specify the patterns and frequency of brain malformations associated with septal agenesis (SA), to identify the clinical prognosis, and to discuss the aetiology of PSA with the new insights provided by molecular genetics. The study consisted of a 14-year retrospective review of brain MRI in 34 patients having PSA (mean age, 5 years). Chiasm and optic nerves were not evaluated. Post-hydrocephalus SA or incomplete data were excluded. The clinical data were correlated to the MRI patterns. The study disclosed 82.5% associated lesions with MRI (28/34): 11 neuronal migration disorders, 9 holoprosencephalies (HP), 7 pituitary stalk interruptions, 1 corpus callosum partial agenesis; 17.5% (6/34) of cases were apparently isolated PAS. Clinically, the patients had motor dysfunction in 68% (23/34), mental retardation in 65% (22/34), blindness in 24% (8/34), endocrinological defects in 21% (7/34) and epilepsy in 18% (6/34) of cases. Nine percent of patients (3/34) were neurologically normal (including one with scoliosis and two infants younger than 2 years at the last follow-up). Patients with bilateral cortical anomalies and HP (even if mild) had the worst neurological prognosis. A severe motor impairment was present without evidence of hemispheric anomaly in 12% of patients (4/34). Interestingly, the frontal lobes were involved in 90% of cortical anomalies and HP, supporting the malformative aetiology of PSA. PSA rarely appears isolated and severe psychomotor impairment may occur in apparently isolated forms. These unfavourable results should be highlighted and need to be confirmed

  7. Hypertrophic Obstructive Cardiomyopathy: Surgical Myectomy and Septal Ablation.

    Science.gov (United States)

    Nishimura, Rick A; Seggewiss, Hubert; Schaff, Hartzell V

    2017-09-15

    Hypertrophic cardiomyopathy is a genetic disorder characterized by marked hypertrophy of the myocardium. It is frequently accompanied by dynamic left ventricular outflow tract obstruction and symptoms of dyspnea, angina, and syncope. The initial therapy for symptomatic patients with obstruction is medical therapy with β-blockers and calcium antagonists. However, there remain a subset of patients who have continued severe symptoms, which are unresponsive to medical therapy. These patients can be treated with septal reduction therapy, either surgical septal myectomy or alcohol septal ablation. When performed by experienced operators working in high-volume centers, septal myectomy is highly effective with a >90% relief of obstruction and improvement in symptoms. The perioperative mortality rate for isolated septal myectomy in most centers is <1%. Alcohol septal ablation is a less invasive treatment. In many patients, the hemodynamic and clinical results are comparable to that of septal myectomy. However, the results of alcohol septal ablation are dependent on the septal perforator artery supplying the area of the contact between the hypertrophied septum and the anterior leaflet of the mitral valve. There are some patients, particularly younger patients with severe hypertrophy, who do not uniformly experience complete relief of obstruction and symptoms. Both techniques of septal reduction therapy are highly operator dependent. The final decision as to which approach should be selected in any given patient is dependent up patient preference and the availability and experience of the operator and institution at which the patient is being treated. © 2017 American Heart Association, Inc.

  8. Determinants of early dilated cardiomyopathy in neonates with congenital complete atrioventricular block.

    Science.gov (United States)

    Silvetti, Massimo Stefano; Drago, Fabrizio; Ravà, Lucilla

    2010-09-01

    Dilated cardiomyopathy (DCM) can occur in infants with congenital complete atrioventricular block (CCAVB) treated by permanent pacemaker (PM), even without other congenital heart defects. The objective is to find the risk factors of this complication. Retrospective analysis of a single-centre experience. Since 1992, 25 patients, aged 25 (1-355) days [median (range)], with normal ejection fraction (EF), underwent PM implantation (13 DDD, 12 VVIR) with an RV-pacing site. Follow-up was 4 (0.3-16) years. DCM occurred after 4 (3-23) months in eight patients (32%). Univariate analysis identified the following risk factors: younger age at implantation [5 (1-85) days vs. 90 (1-355) P = 0.007], a broad QRS (50 vs. 18% P = 0.03), prolonged QTc at implantation (63 vs. 0%, P = 0.001), and greater duration of heart rate >160 bpm during the first month after implantation (18 vs. 2%, P = 0.03). By multivariate analysis prolonged QTc was the only significant risk factor for DCM (hazard ratio: 23, P mode to allow predominant narrow QRS junctional rhythm (one patient each). Neonates with CCAVB without other congenital heart defects and prolonged QTc are at high risk for DCM possibly due to electromechanical dyssynchrony induced by high-rate RV pacing. In patients in whom RV pacing was discontinued, EF became normal.

  9. Incidence and predictors of late complete heart block after alcohol septal ablation treatment of hypertrophic obstructive cardiomyopathy.

    Science.gov (United States)

    Schuller, Joseph L; Zipse, Matthew M; Krantz, Mori J; Blaker, Brian; Salcedo, Ernesto; Groves, Bertron M; Messenger, John C; Beaty, Brenda; Sauer, William H

    2015-02-01

    This study was designed to identify the incidence of late complete heart block (CHB) first identified at least 48 hours post alcohol septal ablation (ASA). Septal reduction with ASA is a therapeutic option for patients with symptomatic hypertrophic obstructive cardiomyopathy (HCM). CHB, resulting from the septal infarct, is a known complication with a reported incidence of 9-22%. The incidence of CHB more than 48 hours post-procedure is unknown. Consecutive patients who underwent ASA were analyzed and clinical characteristics associated with late CHB were assessed. Late CHB was defined as first identification of CHB more than 48 hours after ASA. From 2002-2013, 145 subjects underwent 168 ASA procedures and were followed for a mean of 3.2 +/- 2.3 years. The incidence of late CHB was 8.9% (15/168 ASA procedures). Heart block occurred from 48 hours to 3-years post-procedure. In a multivariable model, patients with any CHB were more likely to have had multiple ASA procedures (OR 4.14; 95% CI: 1.24, 13.9; P < 0.05) and high resting and provoked left ventricular outflow tract (LVOT) gradient assessed by catheterization (OR per 10 mmHg gradient 1.14; 95% CI: 1.0, 1.20; P < 0.05). After multivariable adjustment, only a high provokable LVOT gradient remained an independent predictor of late CHB (OR per 10 mmHg gradient 1.14 [95% CI 1.02-1.29]). Late CHB is a common complication of ASA for treatment of symptomatic HCM. Post-discharge electrocardiographic surveillance for atrioventricular conduction disease should be considered after ASA, especially for those with a high provokable LVOT gradient. © 2015, Wiley Periodicals, Inc.

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

    Directory of Open Access Journals (Sweden)

    Rafael Fabiano Machado Rosa

    2009-02-01

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

  11. Atrioventricular nodal reentrant tachycardia ablation and inferior vena cava agenesis.

    Science.gov (United States)

    Galand, Vincent; Pavin, Dominique; Behar, Nathalie; Mabo, Philippe; Martins, Raphaël P

    2016-10-01

    Congenital anomalies of the inferior vena cava (IVC) are rare and very often diagnosed in asymptomatic patients during computed tomography performed for other purposes. These anomalies can have significant clinical implications, for example if electrophysiology procedures are needed. Diagnostic and ablation procedures are difficult since catheter manipulation and positioning are more complex. We present here a case of successful atrioventricular nodal reentrant tachycardia ablation in a patient with unexpected IVC agenesis, using an azygos route. Copyright © 2016 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.

  12. Occult etiologies of complete atrioventricular block: Report of two cases

    Directory of Open Access Journals (Sweden)

    Neeta Bachani

    2016-09-01

    Full Text Available In patients presenting with complete atrioventricular (AV block, the common causes are degeneration of the conduction system, acute myocardial infarction, congenital and metabolic disorders (such as azotemia. However, at times, no cause can be ascribed and the label congenital or degenerative is applied depending on the patient's age and the QRS complex width. We present two cases of patients with complete AV block, who were subsequently found to have rare etiologies – sarcoidosis (with isolated feature of AV block and non-Hodgkin's lymphoma.

  13. Coronary sinus and atrioventricular groove avulsion after motor vehicle crash

    Directory of Open Access Journals (Sweden)

    Bradley M Dennis

    2014-01-01

    Full Text Available Simultaneous cardiac and pericardial rupture from blunt chest trauma is a highly lethal combination with rarely reported survival. We report of a case of young patient with a right atrioventricular groove injury, pericardial rupture and a unique description of a coronary sinus avulsion following blunt chest trauma. Rapid recognition of this injury is crucial to patient survival, but traditional diagnostic adjuncts such as ultrasound, echocardiography and computed tomography are often unhelpful. Successful repair of these injuries requires high suspicion of injury, early cardiac surgery involvement of and possible even placement of the patient on cardiopulmonary bypass.

  14. 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

    Directory of Open Access Journals (Sweden)

    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

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

    DEFF Research Database (Denmark)

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

    2010-01-01

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

  16. Development of the myocardium of the atrioventricular canal and the vestibular spine in the human heart

    NARCIS (Netherlands)

    Kim, J. S.; Virágh, S.; Moorman, A. F.; Anderson, R. H.; Lamers, W. H.

    2001-01-01

    To establish the morphogenetic mechanisms underlying formation and separation of the atrioventricular connections, we studied the remodeling of the myocardium of the atrioventricular canal and the extracardiac mesenchymal tissue of the vestibular spine in human embryonic hearts from 4.5 to 10 weeks

  17. Possible Common Aetiology behind Maternal Preeclampsia and Congenital Heart Defects in the Child: a Cardiovascular Diseases in Norway Project Study.

    Science.gov (United States)

    Brodwall, Kristoffer; Leirgul, Elisabeth; Greve, Gottfried; Vollset, Stein Emil; Holmstrøm, Henrik; Tell, Grethe S; Øyen, Nina

    2016-01-01

    The aetiology of congenital heart defects (CHD) is mostly unknown, but maternal factors may modify the infant risk of CHD. We investigated the association between maternal preeclampsia and offspring risk of severe CHD in a nation-wide cohort study. Information on all births registered in the Medical Birth Registry of Norway, 1994-2009, was completed with information on CHD diagnoses from national health registries and the Cardiovascular Diseases in Norway Project (CVDNOR). Among 914 703 singleton births without chromosomal abnormalities, 32 864 (3.6%) were born after a pregnancy with preeclampsia. The preeclampsia was diagnosed before the 34th week of pregnancy (early-onset preeclampsia) in 2618 (8.0% of preeclamptic pregnancies). CHDs were diagnosed in 10 691 infants; of these, 2473 had severe CHD. The risk of severe CHD was compared between births with and without maternal preeclampsia and estimated with binomial log-linear regression. When adjusting for year of birth, maternal age, parity, and pregestational diabetes, the risk ratio (RR) for severe CHD in offspring of mothers with any preeclampsia was 1.3 [95% confidence interval (CI) 1.1, 1.5], and in pregnancies with early-onset preeclampsia, the RR was 2.8 (95% CI 1.8, 4.4). The association between early-onset preeclampsia and specific types of severe CHD was stronger for atrioventricular septal defects (AVSD), with adjusted RR 13.5 (95% CI 6.8, 26.8). Early-onset preeclampsia was strongly associated with infant risk of severe CHD, specifically; the risk of AVSD was 15-fold higher if the mother was diagnosed with early-onset preeclampsia, suggesting common aetiological factors for early-onset preeclampsia and erroneous fetal heart development. © 2015 John Wiley & Sons Ltd.

  18. Cryoballoon ablation for paroxysmal atrial fibrillation in the presence of an Amplatzer Septal Occluder device

    Directory of Open Access Journals (Sweden)

    Jubran A. Rind

    2016-09-01

    Full Text Available Cryoballoon ablation of the pulmonary veins (CAPV has been demonstrated to be non-inferior to radiofrequency (RF ablation for paroxysmal atrial fibrillation (AFib. As CAPV requires a larger transseptal sheath than RF ablation, it can be challenging in the presence of an Amplatzer™ Septal Occluder (ASO device. Real-time three-dimensional transesophageal echocardiography (RT3DTEE provides enhanced visualization of various complex cardiac defects and has revolutionized interventional procedures by guiding catheter positioning. We describe successful RT3DTEE guided transseptal puncture for CAPV of paroxysmal AFib in the presence of an ASO in a 53-year-old male.

  19. Congenital heart defects in Kabuki syndrome.

    Science.gov (United States)

    Yuan, Shi-Min

    2013-01-01

    Kabuki syndrome (KS) is an entity of multiple congenital malformations with mental retardation with undetermined etiology. Congenital heart defects are one of the clinical manifestations of KS with insufficient elucidations. Literature of congenital heart defects associated with KS was comprehensively retrieved, collected and reviewed. The clinical features of the congenital heart defects in the patients with KS were summarized. Congenital heart defects were one of the clinical manifestations of KS with 90.6% of the patients being diagnosed prenatally or at an early age. Left-sided obstructions/aortic dilation and septal defects were the fi rst two types of anomalies, accounting up to 46.1% and 32.9%, respectively. The most common congenital heart defects were coarctation of the aorta, and atrial and ventricular septal defects. Fifteen (19.7%) patients received surgical repair of congenital heart defects at a mean age of 0.8 ± 1.3 years. Congenital heart defects are one of the clinical manifestations of KS with 90.6% of the patients being diagnosed prenatally or at an early age. About 20% of the patients warranted surgical repair of the heart defects. Patients with KS require close follow-up in terms of their etiology, clinical presentations and long-term prognosis.

  20. Septal alcoholization in hypertrophic cardiomyopathy: about 11 cases

    African Journals Online (AJOL)

    Outcomes of septal alcoholization in hypertrophic obstructive cardiomyopathy are not enough studied in all centers. The purpose of this study was to determine the outcomes of septal alcoholization in hypertrophic obstructive cardiomyopathy in our hospital. A retrospective and prospective descriptive study focused on all ...

  1. [Candida albicans endocarditis after treatment of complete atrioventricular canal].

    Science.gov (United States)

    El Alami, S; Handor, N; Moutaki Allah, Y; Bouchrik, M; El Mellouki, W; Boulahya, A; Lmimouni, B

    2013-09-01

    Infective endocarditis is rare in children, it is rarer after a surgical treatment of atrioventricular canal, and it is exceptional that Candida albicans is the etiologic agent. This is a serious infection found in congenital heart disease with or without surgery. It is potentially lethal, despite diagnostic and therapeutic advances. We report a case of infective endocarditis due to C. albicans after the treatment of a congenital systemic atrioventricular canal in a child with trisomy 21. The diagnosis was suspected on clinical manifestations and cardiac auscultation. Confirmation was provided by positive blood cultures and echocardiography. The large size of the vegetation in the patient was in favor of a fungal etiology, blood cultures allowed to identify the fungus. This observation illustrates a poorly understood disease, with very poor prognosis and which is a potential complication of heart surgery. The improved prognosis should be achieved by shortening the time to diagnosis and optimizing the therapeutic support. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  2. Molecular Diffusion through Cyanobacterial Septal Junctions

    Directory of Open Access Journals (Sweden)

    Mercedes Nieves-Morión

    2017-01-01

    Full Text Available Heterocyst-forming cyanobacteria grow as filaments in which intercellular molecular exchange takes place. During the differentiation of N2-fixing heterocysts, regulators are transferred between cells. In the diazotrophic filament, vegetative cells that fix CO2 through oxygenic photosynthesis provide the heterocysts with reduced carbon and heterocysts provide the vegetative cells with fixed nitrogen. Intercellular molecular transfer has been traced with fluorescent markers, including calcein, 5-carboxyfluorescein, and the sucrose analogue esculin, which are observed to move down their concentration gradient. In this work, we used fluorescence recovery after photobleaching (FRAP assays in the model heterocyst-forming cyanobacterium Anabaena sp. strain PCC 7120 to measure the temperature dependence of intercellular transfer of fluorescent markers. We find that the transfer rate constants are directly proportional to the absolute temperature. This indicates that the “septal junctions” (formerly known as “microplasmodesmata” linking the cells in the filament allow molecular exchange by simple diffusion, without any activated intermediate state. This constitutes a novel mechanism for molecular transfer across the bacterial cytoplasmic membrane, in addition to previously characterized mechanisms for active transport and facilitated diffusion. Cyanobacterial septal junctions are functionally analogous to the gap junctions of metazoans.

  3. Chlamydial conjunctivitis presenting as pre septal cellulitis

    Directory of Open Access Journals (Sweden)

    Diaper Charles JM

    2007-03-01

    Full Text Available Abstract Chlamydia conjuctivitis results from infection by chlamydia trachomatis, the commonest treatable sexually transmitted infection in Europe. Its clinical manifestations involve the conjunctiva and the cornea. The inflammation under the upper eyelid may be sufficient to present as ptosis, however previously it has not been documented to cause a preseptal cellulitis. We present such a case. A 15-year-old girl was diagnosed with a left viral conjunctivitis. Five days later, she returned with marked oedema of the left upper and lower lids accompanied by erythema. The tarsal conjunctiva revealed follicles and large papillae and extra ocular movements revealed discomfort on elevation. A secondary diagnosis of bacterial pre septal cellulitis was made and the treatment was changed a broad spectrum oral antibiotic. On review at two days, the patient now complained of a large amount of purulent discharge in association with the marked pre septal swelling. As previous bacteriology and virology had been negative, the patient was re swabbed for chlamydia. This proved positive and her symptoms completely resolved following administration of Azithromycin. In this particular case recognition of the pathogen is important to alert the patient to the likelihood of unknown genital infestation. In all cases of positive culture, the patient should be counselled to attend a genitourinary clinic and to alert any sexual partners to the need to do likewise.

  4. Olfactory receptors in the mouse septal organ.

    Science.gov (United States)

    Kaluza, Jan F; Gussing, Fredrik; Bohm, Staffan; Breer, Heinz; Strotmann, Jörg

    2004-05-15

    In this study we have identified a repertoire of chemosensory receptors expressed in the septal organ (SO). The results suggest that septal organ neurons are specified to express receptor genes belonging to class II olfactory receptors that are also expressed in the main olfactory epithelium. We found no evidence for the expression of members from the vomeronasal receptor gene families. In the SO, no topography analogous to the receptor expression zones of the main olfactory epithelium was evident. The majority of identified receptors corresponds to genes with restricted expression in the medial and lateral zones of the main olfactory epithelium. This coincides with the expression of olfactory cell adhesion molecule (OCAM) throughout the SO, which is considered as a marker for the medial-lateral zones. In contrast, NADPH:quinone oxidoreductase 1 expression, a characteristic marker for the dorsal zone, was lacking in the SO. Most of the receptor types were found to be expressed in rather few SO neurons; as an exception, the receptor mOR244-3 was observed in a very high proportion of cells. Although a very high fraction of SO neurons expressed mOR244-3, we found no evidence for the coexpression of different receptors in individual cells. Copyright 2004 Wiley-Liss, Inc.

  5. Atrial enlargement in symptomatic heart block patients with preserved left ventricular function: possibly related to atrioventricular dyssynchrony.

    Science.gov (United States)

    Lin, Yu-Sheng; Guo, G Bih-Fang; Chen, Yung-Lung; Tsai, Tzu-Hsien; Pan, Kuo-Li; Liu, Wen-Hao; Chen, Mien-Cheng

    2011-05-05

    Right ventricular apical pacing may possibly induce atrial dilatation as a consequence of atrioventricular dyssynchrony. However, atrial enlargement associated with atrioventricular dyssynchrony due to atrioventricular block has never been studied. This case-control survey involved 90 patients with symptomatic atrioventricular block [29 patients with Mobitz type 2 atrioventricular block, 22 patients with high degree of atrioventricular block and 39 patients with complete atrioventricular block]. The control group comprised 54 age- and sex-matched patients with sick sinus syndrome and intact intrinsic atrioventricular conduction. The M-mode measurements were obtained before implant and the left and right atrial areas were measured by planimetry. The peri-implant right atrial area (17.4±3.7 vs. 15.3±3.4 cm2, p=0.002), left atrial area (24.9±4.2 vs. 21.0±3.7 cm2, pblock patients than in sick sinus syndrome patients. The two groups had similar left ventricular ejection fraction. The right atrial area (p=0.01) and left atrial area (p=0.006) remained significantly greater in atrioventricular block patients than in sick sinus syndrome patients after adjustments for age, gender, body surface area, left ventricular dimension and left ventricular ejection fraction in multiple logistic regression analysis. Atrial enlargement occurs in patients with symptomatic atrioventricular block. This phenomenon is possibly related to atrioventricular dyssynchrony. Copyright © 2009 Elsevier Ireland Ltd. All rights reserved.

  6. Alcohol septal ablation to overcome shock.

    Science.gov (United States)

    Galle, Katalien; De Sutter, Johan; Cornelis, Kristoff

    2010-04-01

    A 69-year-old man, known with hypertrophic obstructive cardiomyopathy (HOCM), was referred to our hospital because of progressive hypoxaemia and sepsis after admission for respiratory infection. Once at the emergency department, cardiopulmonary resuscitation, intubation and mechanical ventilation were necessary. Despite vasopressors and colloids the patient remained haemodynamically unstable. Because of the conviction that the distributive shock, caused by sepsis, was worsened by an associated obstructive shock related to the HOCM, an alcohol septal ablation (ASA) was attempted in these acute circumstances. Immediately after the ASA the gradient over the left ventricular outflow tract disappeared and the mean arterial pressure and oxygenation increased. Despite his cardiovascular recuperation the patient died a couple of days later. Nevertheless we achieved an improvement of the haemodynamic situation of this patient with HOCM by performing an urgent ASA.

  7. Effect of atrioventricular conduction on heart rate variability

    KAUST Repository

    Ahmad, Talha Jamal

    2011-08-01

    This paper discusses the effect of atrioventricular conduction time (AVCT) on the short-term Heart Rate Variability (HRV) by computing HRV parameters using intervals between the onsets of successive P waves (PP time series) for three groups: normal, arrhythmia and sudden cardiac death (SCD) patients. A very precise wavelet transform based ECG delineator was developed to detect PP, PR and RR time series. Mean PR variation in arrhythmia and SCD group was found to be significantly high as compared to the normal group. It was observed that when PR variations in arrhythmia and SCD cases crossed a certain threshold, RR variability no longer provided a very accurate estimate of HRV. In such cases, PP variability was able to provide a better assessment of HRV. © 2011 IEEE.

  8. Rapidly Progressive Atrioventricular Block in a Patient with Sarcoidosis

    Directory of Open Access Journals (Sweden)

    Nagham Saeed Jafar

    2014-01-01

    Full Text Available Cardiac sarcoidosis is a major cause of death in patients with systemic sarcoidosis. Cardiac manifestations are seen in 2.3% of the patients. Atrioventricular (AV block is one of the common manifestations of cardiac sarcoidosis. Other presentations of cardiac involvement include congestive heart failure, ventricular arrhythmias, and sudden cardiac death. The presence of AV block in young patients should raise the suspicion of sarcoidosis. AV block may be the only manifestation and patients may not have clinical evidence of pulmonary involvement. Here we describe a young male presented with exercise induced AV block rapidly progressing to complete heart block with recurrent syncope needing urgent pacemaker implantation. Factors that suggested an infiltrative process included his young age, rapidly progressive conduction abnormalities in the ECG in the absence of coronary disease, and previous history of cutaneous sarcoidosis.

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

    Science.gov (United States)

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

    2015-01-01

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

  10. What Next After Failed Septal Ventricular Tachycardia Ablation?

    Directory of Open Access Journals (Sweden)

    Laurent Roten, MD

    2012-07-01

    Full Text Available Ablation of ventricular tachycardia (VT by conventional radiofrequency ablation can be impossible if the ventricular wall at the targeted ablation site is very thick, as for example the ventricular septum. We present a case of a patient with incessant, non-sustained slow VT originating from the septal part of the lower outflow tracts. Radiofrequency catheter ablation from both ventricles as well as from the anterior cardiac vein were not successful. Both high power radiofrequency ablation and bipolar radiofrequency ablation neither were successfull. Finally, ethanol ablation of the first septal perforator successfully terminated arrhythmia. We discuss the possibilities to overcome failed conventional radiofrequency VT ablation of a septal focus.

  11. His or para-His pacing preserves left ventricular function in atrioventricular block: a double-blind, randomized, crossover study.

    Science.gov (United States)

    Kronborg, Mads B; Mortensen, Peter T; Poulsen, Steen H; Gerdes, Jens C; Jensen, Henrik K; Nielsen, Jens C

    2014-08-01

    To compare left ventricular function after a long-term His or para-His pacing (HP) and right ventricular septal pacing (RVSP) in patients with atrioventricular block (AVB). We included consecutive patients with AVB, a narrow QRS 0.40, in a prospective, randomized, double-blinded, crossover design. All patients were treated with 12 months HP and 12 months RVSP. A total of 38 patients [mean age, 67 ± 10 years; 30 (79%) men] were included. The primary endpoint was LVEF, which was significantly lower after a 12 months RVSP (0.50 ± 0.11) than after 12 months of HP (0.55 ± 0.10), P = 0.005. We measured the difference in time-to-peak systolic velocity between opposite basal segments in the apical views by using tissue Doppler imaging. In the four-chamber view, the difference was 58 (±7) ms after RVSP and 49 (±7) ms after HP, P = 0.27; in the two-chamber view, the difference was 45 (±5) ms after RVSP and 31 ±(4) ms after HP, P = 0.02, and in the apical long-axis view, the difference was 63 (±6) after RVSP and 44 (±7) after HP, P = 0.03. There was no difference in New York Heart Association class, 6-min hall walk test, quality-of-life assessments, or device-related complications. The mean threshold was significantly higher in HP leads than in RVSP leads. His or para-His pacing preserves LVEF and mechanical synchrony as compared with RVSP after 12 months pacing in patients with AVB, narrow QRS, and LVEF > 0.40. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.

  12. Permanent pacemaker implantation for late atrioventricular block in patients receiving catheter ablation for atrioventricular nodal reentrant tachycardia.

    Science.gov (United States)

    Liao, Jo-Nan; Hu, Yu-Feng; Wu, Tsu-Juey; Fong, Ann-Ning; Lin, Wei-Shiang; Lin, Yenn-Jiang; Chang, Shih-Lin; Lo, Li-Wei; Tuan, Ta-Chuan; Chang, Hung-Yu; Li, Cheng-Hung; Chao, Tze-Fan; Chung, Fa-Po; Hanafy, Dicky Armein; Lin, Wen-Yu; Huang, Jin-Long; Huang, Chin-Chou; Leu, Hsin-Bang; Lee, Pi-Chang; Chiang, Chern-En; Chen, Shih-Ann

    2013-02-15

    The present study investigated the incidence and predictors of permanent pacemaker (PPM) implantation for late atrioventricular block (AVB) in patients with atrioventricular nodal reentrant tachycardia (AVNRT) who received ablation. The data from 3,442 patients with AVNRT who received ablation were analyzed. Those who developed late AVB (>1 month after ablation) and received a PPM were identified. The incidence of PPM implantation in 1,148 matched patients with Wolff-Parkinson-White syndrome and in the whole population of Taiwan were compared. Of the patients with AVNRT receiving ablation (mean follow-up duration 128.3 ± 62.5 months), 15 (0.4%) received PPM implantation for late AVB (mean interval after catheter ablation 95.4 ± 55.0 months). Only age (odds ratio 1.05, p = 0.02) and transient AVB (odds ratio 8.55, p = 0.01) during the procedure were independently associated with PPM implantation for late AVB. The patients with AVNRT had a greater incidence of PPM implantation due to late AVB compared to the matched patients with Wolff-Parkinson-White syndrome. The annual incidence of PPM implantation for AVB was also greater in the patients with AVNRT than in the general population. In conclusion, the incidence of PPM implantation for late AVB in patients with AVNRT who received catheter ablation was low but still greater than that in patients with Wolff-Parkinson-White syndrome and the general population in Taiwan. Copyright © 2013 Elsevier Inc. All rights reserved.

  13. Concomitant ablation for atrial fibrillation during septal myectomy in patients with hypertrophic obstructive cardiomyopathy.

    Science.gov (United States)

    Bogachev-Prokophiev, Alexander V; Afanasyev, Alexander V; Zheleznev, Sergei I; Pivkin, Alexei N; Fomenko, Michael S; Sharifulin, Ravil M; Karaskov, Alexander M

    2017-09-01

    The appearance of atrial fibrillation is associated with significant clinical deterioration in patients with obstructive hypertrophic cardiomyopathy; therefore, maintenance of sinus rhythm is desirable. Guidelines and most articles have reported the results of catheter ablation and pharmacologic atrial fibrillation treatment; nevertheless, data regarding concomitant procedures during septal myectomy are limited. The aim of this study was to assess the outcomes of concomitant atrial fibrillation treatment in patients with obstructive hypertrophic cardiomyopathy. Between 2010 and 2013 in our clinic, 187 patients with obstructive hypertrophic cardiomyopathy underwent extended myectomy. In 45 cases, concomitant Cox-Maze IV procedure was performed; however, obstructive hypertrophic cardiomyopathy was the primary indication for surgery. Atrial fibrillation was paroxysmal in 26 patients (58%) and nonparoxysmal in 19 patients (42%). The mean age of patients was 52.8 ± 14.2 years (range, 22-74 years). Mean peak gradient was 90.7 ± 24.2 mm Hg, and interventricular septum thickness was 26.1 ± 4.3 mm. Mean atrial fibrillation duration was 17.3 ± 8.5 months. There were no early deaths. No procedure-related complications occurred with regard to ablation procedure. Complete atrioventricular block was achieved in 2 patients (4.0%). Mean crossclamping time was 61 ± 36 minutes. Peak left ventricular outflow tract gradient was 12.6 ± 5.5 mm Hg based on transesophageal echocardiography. The Maze IV procedure was used for ablation in all patients (radiofrequency ablation with bipolar clamp + cryolesion for mitral and tricuspid lines). Because of the atrial wall thickness (5-6 mm), applications were performed 8 to 10 times on each line. There were no cases of pacemaker implantation due to sinus node dysfunction. All patients were discharged in stable sinus rhythm. Mean follow-up was 23.7 ± 1.3 months. The rate of atrial fibrillation freedom was 100% (45

  14. Intra-cardiac echocardiography in alcohol septal ablation

    DEFF Research Database (Denmark)

    Cooper, Robert M; Shahzad, Adeel; Newton, James

    2015-01-01

    Alcohol septal ablation (ASA) in hypertrophic obstructive cardiomyopathy reduces left ventricular outflow tract gradients. A third of patients do not respond; inaccurate localisation of the iatrogenic infarct can be responsible. Transthoracic echocardiography (TTE) using myocardial contrast can...

  15. Congenital complete atrioventricular block associated with QT prolongation: Description of a patient with an unusual outcome.

    Science.gov (United States)

    Mendoza, Alberto; Belda, Silvia; Salguero, Rafael; Granados, Miguel Angel

    2010-08-01

    The association of a complete atrioventricular block with long QT syndrome is relatively common and carries a high risk of torsades de pointes (TdP) and sudden death. It is probably due to a downregulation of potassium channel currents (I (Ks) and I (Kr)) that impairs ventricular repolarization, prolongs the QT interval and increases susceptibility to TdP, so it must be considered a channelopathy. This report describes a 6 year-old boy, with a complete atrioventricular block diagnosed at 5 months of age, who at the age of 1 year started having episodes of TdP associated with a prolonged QT interval. He was treated successfully with propranolol and with a pacemaker implant. At age 3 the complete atrioventricular block reversed spontaneously to a first degree atrioventricular block.

  16. Valproic Acid as a Cause of Transient Atrio-Ventricular Conduction Block Episodes.

    Science.gov (United States)

    Davutoglu, Vedat; Neyal, Munife; Altunbas, Gokhan

    2017-01-01

    Herein we share, to our knowledge for the first time, a a case of valproic acid use complicated by symptomatic atrio-ventricular conduction block episodes on Holter monitoring. Symptomatic atrio-ventricular block episodes should be considered as an unusual side effect of valproic acid despite normal blood therapeutic level. Before consideration of pacemaker implantation in such cases, valproic acid usage should be investigated, and dose reduction should be attempted.

  17. [Informed consent in congenital defect heart surgery].

    Science.gov (United States)

    Oka, Norihiko; Miyaji, Kagami

    2014-09-01

    In pediatric cardiac surgery, informed consent is often obtained from the patient's parents. The parents are commonly under great stress due to being in a situation where they have to make the important decision of whether their child will undergo cardiac surgery. Cardiac surgery does not necessarily determine the patient's prognosis if he or she has other organ anomalies or systemic diseases. In such cases, communication among the cardiac surgeon, pediatrician, and family becomes even more crucial. In pediatric cardiac surgery, it is important to obtain informed consent with adequate explanations, an understanding of the parents' stressful situation, and knowledge of the patient's background. In this article, we introduce two examples of obtaining informed consent for two common pediatric cardiac operations, atrial septal defect closure and ventricular septal defect closure.

  18. Percutaneous closure of congenital Gerbode defect using Nit-Occlud? L? VSD coil

    OpenAIRE

    Phan, Quang T; Kim, Sang-Wook; Nguyen, Hieu L

    2017-01-01

    We present a case report about percutaneous closure of a congenital Gerbode defect using Nit-Occlud? L? VSD coil. The patient was referred to our hospital with a diagnosis of ventricular septal defect (VSD) and severe pulmonary arterial hypertension. But transthoracic echocardiography revealed a communication between the left ventricle (LV) and the right atrial (RA), called Gerbode defect. Catheterization confirmed the shunt from the LV to the RA. We successfully closed the defect with a VSD ...

  19. Transitory complete atrioventricular block associated to ingestion of Nerium oleander.

    Science.gov (United States)

    Nishioka, S de A; Resende, E S

    1995-01-01

    Self-medication with plants can lead to severe poisoning. Oleander (Nerium oleander) is an ornamental plant whose toxicity to man is due to a mixture of nondigitalis cardiac glycosides. The clinical manifestations of oleander poisoning combine cardiac and gastrointestinal symptoms, and are similar to those of a digitalis overdose. Following the ingestion of tea made of the leaves of oleander, a 33-year-old woman developed dizziness, vomiting and abdominal cramps as main symptoms, and complete atrioventricular block that reverted within 24 hours. She remained with bradycardia, dizziness and diarrhea for about 6 days. On admission she had a serum potassium of 6.7 mEq/L and a serum creatinine of 2.3 mg%, that progressively returned to normal levels. Clinicians must include oleander poisoning in the differential diagnosis of bradyarrhythmias, particularly in children and young people without known cardiovascular disease, in areas where this plant either is used as a herbal medicine or is known as poisonous.

  20. Congenital complete atrioventricular block in the early pediatric population

    Directory of Open Access Journals (Sweden)

    Riccardo Cappato

    2010-05-01

    Full Text Available Congenital complete atrioventricular block (CCAVB is a rare potentially lethal disease with an estimated incidence of 1 every 15.000 to 20.000 live born infants. In structurally normal hearts two kinds of congenital heart block can be identified: one usually diagnosed in utero associated with the circulating maternal anti-SS-A/Ro and anti-SS-B/La antibodies, the other kind is detected later in the neonatal period or during the infancy or childhood and present no clear relation with maternal antibodies. Nowadays, the diagnosis can be made in utero as early as between week 16 and 28 of gestation by foetal echocardiography. The mortality in isolated CCAVB is estimated between 8 and 16% and between 4 and 8% in children and adults. The mortality and morbidity of patients diagnosed outside the neonatal period is significantly lower than those with a in utero diagnosis. Risk factors for worse outcome in CCAVB are the foetal diagnosis, the presence of hydrops fetalis, delivery at

  1. Fetal tachyarrhythmia with 1:1 atrioventricular conduction. Adenosine infusion in the umbilical vein as a diagnostic test

    Directory of Open Access Journals (Sweden)

    Tiago L. L. Leiria

    2000-07-01

    Full Text Available This is the report of a case of fetal tachyarrhythmia with 1:1 atrioventricular conduction detected by pre-natal echocardiography in a fetus at 25-weeks gestation. Adenosine infusion via cordocentesis was performed as a diagnostic test to differentiate between atrioventricular nodal reentrant supraventricular tachyarrhythmia and atrial flutter. After infusion, transient 2:1 atrioventricular dissociation was obtained and the diagnosis of atrial flutter was made. Transplacental therapy with digoxin and amiodarone was then successfully used.

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

    Science.gov (United States)

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

    2017-06-01

    The aim was to characterise acute coronary syndrome patients with complete atrioventricular block and to assess the effect on outcome. Patients admitted with acute coronary syndrome were divided according to the presence of complete atrioventricular block: group 1, with complete atrioventricular block; group 2, without complete atrioventricular block. Clinical, electrocardiographic and echocardiographic characteristics and prognosis during one year follow-up were compared between the groups. Among 4799 acute coronary syndrome patients admitted during the study period, 91 (1.9%) presented with complete atrioventricular block. At presentation, group 1 patients presented with lower systolic blood pressure, higher Killip class and incidence of syncope. In group 1, 86.8% presented with ST-segment elevation myocardial infarction (STEMI), and inferior STEMI was verified in 79.1% of patients in group 1 compared with 21.9% in group 2 ( Pcoronary intervention ( P<0.001). During hospitalisation group 1 had worse outcomes, with a higher incidence of cardiogenic shock (33.0% vs. 4.5%; P<0.001), ventricular arrhythmias (17.6% vs. 3.6%; P<0.001) and the need for invasive mechanical ventilation (25.3% vs. 5.1%; P<0.001). After a propensity score analysis, in a multivariate regression model, complete atrioventricular block was an independent predictor of hospital mortality (odds ratio 3.671; P=0.045). There was no significant difference in mortality at one-year follow-up between the study groups. Complete atrioventricular block conferred a worse outcome during hospitalisation, including a higher incidence of cardiogenic shock, ventricular arrhythmias and death.

  3. Morphometric analysis of septal aperture of humerus

    Directory of Open Access Journals (Sweden)

    Raghavendra K, Anil kumar Reddy Y, Shirol VS, Daksha Dixit, Desai SP

    2014-04-01

    Full Text Available Introduction: Lower end of humerus shows olecranon and coronoid fossae separated by a thin bony septum, sometimes it may deficient and shows foramen which communicates both the fossae called Septal aperture, which is commonly referred as supratrochlear foramen (STF. Materials & Methods: We have studied 260 humeri (126 right side and 134 left side, measurements were taken by using vernier caliper, translucency septum was observed by keeping the lower end of humerus against the x-ray lobby. Results: A clear cut STF was observed in 19.2% bones, translucency septum was observed in 99 (91.6% humeri on the right side and 95 (93.1% humeri on the left sides respectively (Table – 1. Clinical significance: The presence of STF is always associated with the narrow medullary canal at the lower end of humerus, Supracondylar fracture of humerus is most common in paediatric age group, medullary nailing is done to treat the fractures in those cases the knowledge about the STF is very important for treating the fractures. It has been observed in x-ray of lower end of the humerus the STF is comparatively radiolucent, it is commonly seen as a type of ‘pseudolesions’ in an x-ray of the lower end of humerus and it may mistake for an osteolytic or cystic lesions. Conclusion: The present study can add data into anthropology and anatomy text books regarding STF and it gives knowledge of understanding anatomical variation of distal end of the humerus, which is significant for anthropologists, orthopaedic surgeons and radiologists in habitual clinical practice.

  4. Five-year clinical and echocardiographic evaluation of the Das AngelWings atrial septal occluder.

    Science.gov (United States)

    Kay, Joseph D; O'Laughlin, Martin P; Ito, Kristin; Wang, Andrew; Bashore, Thomas M; Harrison, J Kevin

    2004-02-01

    The late outcome of patients treated with atrial septal occluder devices remains incompletely defined. The purpose of this study was to assess the late outcome (range 4-7 years postprocedure) of patients in whom the Das AngelWings septal occluder device was implanted in the atrial septum. We report the clinical and echocardiographic outcome, at an average of 5 years following the procedure, of patients treated with the Das AngelWings device used to close either a secundum atrial septal defect (ASD) or a patent foramen ovale (PFO). Thirty-two patients underwent successful percutaneous closure of an atrial septal closure, patent foramen ovale, or fenestration in the lateral tunnel of their Fontan with the Das AngelWings device between June 1995 and March 1998 at Duke University Medical Center. Two of the 32 patients were lost to follow-up. The remaining 30 patients were divided into 3 groups based on indication for device implantation. Group 1 consisted of 14 patients with a secundum ASD and predominantly left-to-right atrial shunting. Group 2 consisted of 8 patients who had a PFO and who suffered a thromboembolic event. Group 3 (compassionate use) consisted of 10 patients with multiple comorbid medical problems with predominantly right-to-left shunting at the atrial level causing hypoxemia. Eight of the patients in group 3 were severely ill at the time of device implantation. The 2 remaining patients in group 3 underwent AngelWings implantation for closure of right-to-left shunting through a Fontan fenestration. Mean follow-up was 59 months. There was no device embolization. No patient in the ASD or PFO/stroke group had a clinical complication. By radiographic examination, 2 of 27 patients had evidence of fracture of the nitinol framework at 2-year follow-up. Residual shunting was present in 44% at 24 hours, 20% at 1 year, and 18.8% at 2 years by use of Doppler color flow imaging and/or microcavitation echocardiographic studies. Mild mitral regurgitation caused by

  5. Nasal Septal Deviations: A Systematic Review of Classification Systems

    Directory of Open Access Journals (Sweden)

    Jeffrey Teixeira

    2016-01-01

    Full Text Available Objective. To systematically review the international literature for internal nasal septal deviation classification systems and summarize them for clinical and research purposes. Data Sources. Four databases (including PubMed/MEDLINE were systematically searched through December 16, 2015. Methods. Systematic review, adhering to PRISMA. Results. After removal of duplicates, this study screened 952 articles for relevance. A final comprehensive review of 50 articles identified that 15 of these articles met the eligibility criteria. The classification systems defined in these articles included C-shaped, S-shaped, reverse C-shaped, and reverse S-shaped descriptions of the septal deviation in both the cephalocaudal and anteroposterior dimensions. Additional studies reported use of computed tomography and categorized deviation based on predefined locations. Three studies graded the severity of septal deviations based on the amount of deflection. The systems defined in the literature also included an evaluation of nasal septal spurs and perforations. Conclusion. This systematic review ascertained that the majority of the currently published classification systems for internal nasal septal deviations can be summarized by C-shaped or reverse C-shaped, as well as S-shaped or reverse S-shaped deviations in the anteroposterior and cephalocaudal dimensions. For imaging studies, predefined points have been defined along the septum. Common terminology can facilitate future research.

  6. Pathology of complete atrioventricular block in chronic chagas' myocarditis

    Directory of Open Access Journals (Sweden)

    Zilton A. Andrade

    1988-03-01

    Full Text Available Sclero-atrophy, fibrosis, vascular ectasia, phlebosclerosis and mild non-specific chronic inflammatory changes were observed in variable location and proportion involving the atrioventricular conducting tissue of the heart in five human cases of chronic Chagas' myocarditis associated with complete atrioventricular block. One case presented complete destruction of the A-V conduction system. In three cases the lesions were disseminated all along the conducting tissue but did not cause anywhere a complete disruption in the continuity of the system. The distal portion of the bundle branches were the most damaged sector of the system, exceptfor the fasciculi of the posterior division of the left bundle branch which were relatively preserved. One case exhibited bilateral sclero-atrophy of the bundle branches as the main change; and another showed early and mild fibrocalcific damage of the penetrating portion of the His bundle. The A-V node appeared as the least involved part of the conducting system in the cases studied. Demonstration of the lesions in this series of cases seems important because: a it reveals that complete atrioventriculr block in chronic Chagas' disease results from disseminated lesions and not from focal disruptive change as has been commonly observed in cases of other etiologies; b it shows that chronic inflammation can produce at the end variable and widespread vascular, degenerative andfibrotic alterations within the conducting tissue of the heart, which may lead to its total destruction.O estudo do sistema de condução atrioventricular através cortes seriados completos em cinco casos humanos de miocardite crônica chagásica e bloqueio A-V total revelou a presença de lesões de esclero-atrofia, fibrose, ectasia vascular, fleboesclerose e inflamação crônica inespeclfica envolvendo o sistema de maneira disseminada, mas com distribuição e intensidade variáveis de caso para caso. Em um caso, todo o sistema, do nódulo A

  7. Volume of the human septal forebrain region is a predictor of source memory accuracy.

    Science.gov (United States)

    Butler, Tracy; Blackmon, Karen; Zaborszky, Laszlo; Wang, Xiuyuan; DuBois, Jonathan; Carlson, Chad; Barr, William B; French, Jacqueline; Devinsky, Orrin; Kuzniecky, Ruben; Halgren, Eric; Thesen, Thomas

    2012-01-01

    Septal nuclei, components of basal forebrain, are strongly and reciprocally connected with hippocampus, and have been shown in animals to play a critical role in memory. In humans, the septal forebrain has received little attention. To examine the role of human septal forebrain in memory, we acquired high-resolution magnetic resonance imaging scans from 25 healthy subjects and calculated septal forebrain volume using recently developed probabilistic cytoarchitectonic maps. We indexed memory with the California Verbal Learning Test-II. Linear regression showed that bilateral septal forebrain volume was a significant positive predictor of recognition memory accuracy. More specifically, larger septal forebrain volume was associated with the ability to recall item source/context accuracy. Results indicate specific involvement of septal forebrain in human source memory, and recall the need for additional research into the role of septal nuclei in memory and other impairments associated with human diseases.

  8. Myocardial infarction, symptomatic third degree atrioventricular block and pulmonary embolism caused by thalidomide: a case report.

    Science.gov (United States)

    Zhang, Shengyu; Yang, Jing; Jin, Xiaofeng; Zhang, Shuyang

    2015-12-18

    Thalidomide has been reported to cause numerous thromboembolic events. Deep vein thrombosis and pulmonary embolism are more common. It can also cause bradycardia and even total atrioventricular block. Rarely, it causes coronary artery spasm and even myocardial infarction. But almost simultaneous onset of myocardial infarction, third degree atrioventricular block and pulmonary embolism in one patient has not been reported so far. A 53-year old man presented because of chest pain, nausea and then syncope for several minutes. Previous medical history included neurodermitis for which thalidomide was given and hypercholesterolemia with simvastatin taking. The patient didn't exhibit any other established risk factors for coronary artery disease. Electrocardiography showed sinus rhythm with third degree atrioventricular block and complete right bundle branch block, and precordial leads ST segment elevation. The diagnosis of acute coronary syndrome was suspected, but further coronary angiography demonstrated no flow-limiting lesions in coronary arteries, and temporary pacemaker was implanted. After admission, low SpO2 and elevated D-dimer level was mentioned. Further computed tomography pulmonary angiography revealed pulmonary embolism. Thalidomide was thought to be the cause of hypercoagulability and coronary spasm, so it was ceased immediately. Therapeutic low molecule weight heparin was initiated and then switched to warfarin with appropriate INR, and nifedipine was described for coronary spasm. The patient's symptoms completely relived and SpO2 recovered, and atrioventricular block had disappeared during hospitalization with pacemaker removed. This is the very first case in which myocardial infarction, third degree atrioventricular block and pulmonary embolism almost simultaneously developed. We should be ware that anti-thrombotic prophylaxis, which needs further investigation for optimal drug and dosage, may be beneficial in thalidomide therapy. And it is also

  9. Lah is a transmembrane protein and requires Spa10 for stable positioning of Woronin bodies at the septal pore of Aspergillus fumigatus.

    Science.gov (United States)

    Leonhardt, Yannik; Kakoschke, Sara Carina; Wagener, Johannes; Ebel, Frank

    2017-03-10

    Woronin bodies are specialized, fungal-specific organelles that enable an immediate closure of septal pores after injury to protect hyphae from excessive cytoplasmic bleeding. In most Ascomycetes, Woronin bodies are tethered at the septal pore by so-called Lah proteins. Using the pathogenic mold Aspergillus fumigatus as a model organism, we show that the C-terminal 288 amino acids of Lah (LahC288) bind to the rim of the septal pore. LahC288 essentially consists of a membrane spanning region and a putative extracellular domain, which are both required for the targeting to the septum. In an A. fumigatus rho4 deletion mutant that has a severe defect in septum formation, LahC288 is recruited to spot-like structures in or at the lateral membrane. This suggests that LahC is recruited before Rho4 starts to govern the septation process. Accordingly, we found that in wild type hyphae Lah is bound before a cross-wall emerges and thus enables a tethering of Woronin bodies at the site of the newly formed septum. Finally, we identified Spa10, a member of a recently described family of septal pore-associated proteins, as a first protein that directly or indirectly interacts with LahC to allow a stable positioning of Woronin bodies at the mature septum.

  10. High Prevalence of Associated Birth Defects in Congenital Hypothyroidism

    Directory of Open Access Journals (Sweden)

    Alok Sachan

    2010-01-01

    Full Text Available Aim. To identify dysmorphic features and cardiac, skeletal, and urogenital anomalies in patients with congenital hypothyroidism. Patients and Methods. Seventeen children with congenital primary hypothyroidism were recruited. Cause for congenital hypothyroidism was established using ultrasound of thyroid and T99mc radionuclide thyroid scintigraphy. Malformations were identified by clinical examination, echocardiography, X-ray of lumbar spine, and ultrasonography of abdomen. Results. Ten (59% patients (6 males and 4 females had congenital malformations. Two had more than one congenital malformation (both spina bifida and ostium secundum atrial septal defect. Five (29% had cardiac malformations, of whom three had only osteum secundum atrial septal defect (ASD, one had only patent ductus arteriosus (PDA, and one patient had both ASD and PDA. Seven patients (41% had neural tube defects in the form of spina bifida occulta. Conclusion. Our study indicates the need for routine echocardiography in all patients with congenital hypothyroidism.

  11. Effectiveness of caudal septal extension graft application in endonasal septoplasty.

    Science.gov (United States)

    Karadavut, Yunus; Akyıldız, Ilker; Karadaş, Hatice; Dinç, Aykut Erdem; Tulacı, Gökçe; Tastan, Eren

    Septal deviation is a common disease seen in daily otorhinolaryngology practice and septoplasty is a commonly performed surgical procedure. Caudal septum deviation is also a challenging pathology for ear, nose, and throat specialists. Many techniques are defined for caudal septal deviation. To evaluate the effectiveness of caudal septal extension graft (CSEG) application in patients who underwent endonasal septoplasty for a short and deviated nasal septum. Forty patients with nasal septal deviation, short nasal septum, and weak nasal tip support who underwent endonasal septoplasty with or without CSEG placement between August 2012 and June 2013 were enrolled in this study. Twenty patients underwent endonasal septoplasty with CSEG placement. The rest of the group, who rejected auricular or costal cartilage harvest for CSEG placement, underwent only endonasal septoplasty without any additional intervention. Using the Nasal Obstruction Symptom Evaluation (NOSE) and Rhinoplasty Outcome Evaluation (ROE) questionnaires, pre- and post-operative acoustic rhinometer measurements were evaluated to assess the effect of CESG placement on nasal obstruction. In the control group, preoperative and postoperative minimal cross-sectional areas (MCA1) were 0.44±0.10cm2 and 0.60±0.11cm2, respectively (pCirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  12. left ventricular inflow obstruction by giant atrial septal aneurysm

    African Journals Online (AJOL)

    ANEURYSM IN A NEONATE WITH HYPOPLASTIC RIGHT HEART. SYNDROME: CASE REPORT. C. YUKO-JOWI and C. A. OKELLO. SUMMARY. Atrial septal aneurysm remains a rare congenital cardiac malformation. In the neonatal age group it can occur as an isolated cardiac malformation or in association with complex.

  13. Left Ventricular Inflow Obstruction by Giant Atrial Septal Aneurysm in ...

    African Journals Online (AJOL)

    Atrial septal aneurysm remains a rare congenital cardiac malformation. In the neonatal age group it can occur as an isolated cardiac malformation or in association with complex hypoplastic cardiac malformations of the right and left heart. In the adult population most aneurysms have been described in association with ...

  14. Prevalência de cardiopatias congênitas em portadores da síndrome de Down na cidade de Pelotas (RS Prevalence of congenital heart defects in patients with Down syndrome in the municipality of Pelotas, Brazil

    Directory of Open Access Journals (Sweden)

    Luciana T. Vilas Boas

    2009-10-01

    -sectional study including children with Down syndrome who were born and lived in Pelotas from January 2000 to December 2005. Data were collected by means of home interviews with mothers or guardians. Univariate and bivariate analyses were carried out to analyze the factors related to congenital heart defect. RESULTS: Forty-seven mothers of patients with Down syndrome were interviewed. Twenty-two (46.8% of the patients had a diagnosis of congenital heart defect. Of them, 28% had early cardiac evaluation before 3 months of life. The most frequent heart defect was interatrial communication (17%; atrioventricular septal defect affected five patients. Bivariate analysis between the outcome congenital heart defect and the predicting factors maternal age, paternal age, parents' and child's skin color, presence of other malformations and child's sex showed that the associations were not statistically significant. CONCLUSIONS: The prevalence of Down syndrome and congenital heart defects in our region is similar to the rates found by other authors; therefore, we highlight the importance of diagnostic suspicion and early referral by pediatricians to cardiac evaluation. Another relevant aspect is the small number of patients who underwent karyotype testing. In addition, the number of associated malformations was lower than that found by other authors.

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

    Directory of Open Access Journals (Sweden)

    Vivek Velayudhan Pillai

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

  16. Percutaneous closure of large VSD using a home-made fenestrated atrial septal occluder in 18-year-old with pulmonary hypertension.

    Science.gov (United States)

    Kamali, Hacer; Saritas, Turkay; Erdem, Abdullah; Akdeniz, Celal; Tuzcu, Volkan

    2014-06-09

    Hemodynamically significant muscular ventricular septal defects in children after the infantile period are a rare occurrence and ideal for transcatheter closure. In cases of severe concomitant pulmonary hypertension, it may be necessary to fenestrate the device. In this report, we present an 18-year old patient with a large mid-trabecular ventricular septal defect and severe pulmonary hypertension that underwent percutaneous closure of the defect with a home-made fenestrated atrial septal occluder. An 18-year-old female patient referred to us with complaints of dyspnea (NYHA score of 2-3). Physical examination revealed an apical rumble and a harsh second heart sound. Echocardiographic examination revealed a large mid-trabecular ventricular septal defect with bidirectional shunt and the widest diameter measuring 22 mm on 2D echocardiography. Left and right heart cavities were enlarged. Before and after the vasoreactivity test performed during cardiac catheterization, average aortic pressure was 65 → 86 mmHg, average pulmonary artery pressure: 58 → 73 mmHg, Qp/Qs: 1.6 → 3.2, PVR: 4.6 → 4.3 Wood/U/m2 and PVR/SVR: 0.5 → 0.2. On left-ventricular angiocardiogram, the largest end-diastolic defect diameter was 21 mm. The closure procedure was performed with transthoracic echocardiographic guidance, using a 24 mm Cera septal occluder and a 14 F sheath dilator to make a 4.5-5 mm opening. Measured immediately after the procedure and during cardiac catheterization one month later, average aortic pressure was 75 → 75 mmHg, average pulmonary artery pressure: 66 → 30 mmHg, Qp/Qs 1.5 → 1.4, PVR: 4.4 → 2.9 Wood/U/m2 and PVR/SVR: 0.4 → 0.2. Transthoracic echocardiographic examination performed 24 hours after the procedure showed a max 35-40 mmHg gradient between the left and right ventricles through the fenestration. After the procedure, we observed sporadic early ventricular systoles and a nodal rhythm disorder

  17. Increased sarcolemmal Na+/H+ exchange activity in hypertrophied myocytes from dogs with chronic atrioventricular block

    NARCIS (Netherlands)

    van Borren, Marcel M. G. J.; Vos, Marc A.; Houtman, Marien J. C.; Antoons, Gudrun; Ravesloot, Jan H.

    2013-01-01

    Dogs with compensated biventricular hypertrophy due to chronic atrioventricular block (cAVB), are more susceptible to develop drug-induced Torsade-de-Pointes arrhythmias and sudden cardiac death. It has been suggested that the increased Na+ influx in hypertrophied cAVB ventricular myocytes

  18. Atrioventricular muscular dystrophy in a 5-month-old English springer spaniel.

    Science.gov (United States)

    Lai, Serene R

    2009-12-01

    Primary persistent atrial standstill due to atrioventricular muscle dystrophy is a rare familial disease in dogs. The diagnosis of this disorder in a 5-month-old English springer spaniel is the earliest in dogs that have been presented at the Ontario Veterinary College.

  19. Longitudinal echocardiographic follow-up in children with congenital complete atrioventricular block

    NARCIS (Netherlands)

    Beaufort-Krol, Gertie C. M.; Leeuwen, Miek J. M. Schasfoort-Van; Stienstra, Ymkje; Bink-Boelkens, Margreet Th. E.

    2007-01-01

    Background: Due to a low heart rate (HR) in children with congenital complete atrioventricular block (CCAVB), a larger stroke volume of the left ventricle (LV) may be expected. If so, end-diastolic (LVEDD) and end-systolic (LVESD) diameters may be enlarged and even dilated cardiomyopathy (DCM) may

  20. Evolution and scaling of atrioventricular conduction time in mammals. [Pt. 2

    NARCIS (Netherlands)

    Meijler, F.L.; Strackee, J.

    2006-01-01

    Changes of the PR interval (atrioventricular delay) in relation to changes of heart size in mammalian species (scaling) confront us with a perplexing lack of understanding of an essential funetion of the heart. The PR interval controls the duration of late diastolic blood flow from the atria to the

  1. Complete paroxysmal atrioventricular block in a 2-year-old girl

    DEFF Research Database (Denmark)

    Holst, Line Marie; Dixen, Ulrik; Jeppesen, Dorthe L

    2015-01-01

    We present a case of atypical syncope in a 2-year-old, otherwise healthy girl. The patient presented with three episodes of syncope without any precipitating factors and no family history of sudden unexpected death. Holter monitoring revealed 24 events of complete atrioventricular block lasting u...

  2. Septal and Anterior Reverse Mismatch of Myocardial Perfusion and Metabolism in Patients With Coronary Artery Disease and Left Bundle Branch Block

    Science.gov (United States)

    Wang, Jian-Guang; Fang, Wei; Yang, Min-Fu; Tian, Yue-Qin; Zhang, Xiao-Li; Shen, Rui; Sun, Xiao-Xin; Guo, Feng; Wang, Dao-Yu; He, Zuo-Xiang

    2015-01-01

    Abstract The effects of left bundle branch block (LBBB) on left ventricular myocardial metabolism have not been well investigated. This study evaluated these effects in patients with coronary artery disease (CAD). Sixty-five CAD patients with complete LBBB (mean age, 61.8 ± 9.7 years) and 65 without LBBB (mean age, 59.9 ± 8.4 years) underwent single photon emission computed tomography, positron emission tomography, and contrast coronary angiography. The relationship between myocardial perfusion and metabolism and reverse mismatch score, and that between QRS length and reverse mismatch score and wall motion score were evaluated. The incidence of left ventricular septum and anterior wall reverse mismatching between the two groups was significantly different (P < 0.001 and P = 0.002, respectively). The incidences of normal myocardial perfusion and metabolism in the left ventricular lateral and inferior walls were also significantly different between the two groups (P < 0.001 and P < 0.001, respectively). The incidence of septal reverse mismatching in patients with mild to moderate perfusion was significantly higher among those with LBBB than among those without LBBB (P < 0.001). In CAD patients with LBBB, septal reverse mismatching was significantly more common among those with mild to moderate perfusion than among those with severe perfusion defects (P = 0.002). The correlation between the septal reverse mismatch score and QRS length was significant (P = 0.026). In patients with CAD and LBBB, septal and anterior reverse mismatching of myocardial perfusion and metabolism was frequently present; the septal reverse mismatch score negatively correlated with the QRS interval. PMID:25997045

  3. Septo-Hippocampo-Septal Loop and Memory Formation

    Directory of Open Access Journals (Sweden)

    Fatemeh Khakpai

    2013-01-01

    Full Text Available   Cholinergic and GABAergic fibers in the medial septal/diagonal band of Broca (MS/DB area project to the hippocampus and constitute the septo-hippocampal pathway, which has been proven in learning and memory. In addition, the hippocampus has bidirectional connections with the septum, which use this relation for self-regulation of cholinergic input.   The activity of septal and hippocampal neurons is modulated by several neurotransmitters including glutamatergic neurons from the entorhinal cortex, serotonergic fibers from the raphe nucleus, dopaminergic neurons from the ventral tegmental area (VTA, histaminergic cells from the tuberomammillary nucleus and adrenergic fibers from the locus coeruleus (LC. Thus, changes in the glutamatergic, serotonergic and etc. mediated transmission in the MS/DB may influence cholinergic or GABAergic transmission in the hippocampus.

  4. Septo-Hippocampo-Septal Loop and Memory Formation

    Directory of Open Access Journals (Sweden)

    Fatemeh Khakpai

    2012-12-01

    Full Text Available Cholinergic and GABAergic fibers in the medial septal/diagonal band of Broca (MS/DB area project to the hippocampus and constitute the septo-hippocampal pathway, which has been proven in learning and memory. In addition, the hippocampus has bidirectional connections with the septum, which use this relation for self-regulation of cholinergic input. The activity of septal and hippocampal neurons is modulated by several neurotransmitters including glutamatergic neurons from the entorhinal cortex, serotonergic fibers from the raphe nucleus, dopaminergic neurons from the ventral tegmental area (VTA, histaminergic cells from the tuberomammillary nucleus and adrenergic fibers from the locus coeruleus (LC. Thus, changes in the glutamatergic, serotonergic and etc. mediated transmission in the MS/DB may influence cholinergic or GABAergic transmission in the hippocampus.

  5. [The crooked nose: correction of dorsal and caudal septal deviations].

    Science.gov (United States)

    Foda, H M T

    2010-09-01

    The deviated nose represents a complex cosmetic and functional problem. Septal surgery plays a central role in the successful management of the externally deviated nose. This study included 800 patients seeking rhinoplasty to correct external nasal deviations; 71% of these suffered from variable degrees of nasal obstruction. Septal surgery was necessary in 736 (92%) patients, not only to improve breathing, but also to achieve a straight, symmetric external nose. A graduated surgical approach was adopted to allow correction of the dorsal and caudal deviations of the nasal septum without weakening its structural support to the nasal dorsum or nasal tip. The approach depended on full mobilization of deviated cartilage, followed by straightening of the cartilage and its fixation in the corrected position by using bony splinting grafts through an external rhinoplasty approach.

  6. Defect branes

    NARCIS (Netherlands)

    Bergshoeff, Eric A.; Ortin, Tomas; Riccioni, Fabio

    2012-01-01

    We discuss some general properties of "defect branes", i.e. branes of co-dimension two, in (toroidally compactified) IIA/IIB string theory. In particular, we give a full classification of the supersymmetric defect branes in dimensions 3

  7. MR demonstration of septal involvement in arrhythmogenic right ventricular dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Malhaire, Caroline; Rahmouni, Alain [Centre Hospitalo-Universitaire Henri Mondor, Service de Radiologie et d' Imagerie Medicale, Creteil Cedex (France); Garot, Jerome [Centre Hospitalo-Universitaire Henri Mondor, Service de Cardiologie, Creteil Cedex (France)

    2005-05-01

    Arrhythmogenic right ventricular dysplasia (ARVD) is a heart muscle disease of unknown origin. Although MR imaging is regarded as the best technique for the demonstration of functional and structural abnormalities in ARVD, fat deposits in the interventricular septum have never been documented on MR imaging. We report the case of interventricular septal fatty deposition demonstrated by fat-suppressed MR imaging in a 48-year-old man. (orig.)

  8. Polypropylene mesh for nasal septal perforation repair: an experimental study.

    Science.gov (United States)

    Yücebaş, Kadir; Taşkın, Ümit; Oktay, Mehmet Faruk; Tansuker, Hasan Deniz; Erdil, Mehmet; Altınay, Serdar; Kozanoğlu, Erol; Kuvat, Samet Vasfi

    2017-01-01

    The aim of this study is to determine the effectiveness and biocompatibility of polypropylene mesh for the repair of nasal septal perforations in an animal model on rabbits. A full-thickness nasal septal perforation with a diameter of nearly 10 × 10 mm was created on 12 rabbits, and then the perforation was reconstructed with two different methods. We used mucosal flaps and polypropylene mesh as an interpositional graft in group 1. Only mucosal flaps were used for reconstruction and are identified as group 2. After 4 weeks, we removed the nasal septum of the rabbits and performed histopathological examinations for acute rejection, infection, inflammatory response, fibrosis, and granuloma formation. We found perforation closure rates of 75 and 25 % in groups 1 and 2, respectively. Inflammatory response was seen in all specimens of group 1 (100 %). The inflammatory response was +1 in five of the specimens (62.5 %), +2 in one specimen (12.5 %), and +3 in two specimens (25 %). Mild fibrosis around the mesh was detected in four specimens (50 %), medium-level fibrosis was detected in one (12.5 %), and no fibrosis was detected in three (37.5 %). Severe fibrosis was not seen in any specimens. The foreign-body reaction was limited to a few giant cells, and granuloma formation was seen in two specimens (25 %). The propylene mesh showed excellent biocompatibility with the septal mucosa, and it can, therefore, be used for the repair of septal perforation as an interpositional graft safely.

  9. Percutaneous transluminal alcohol septal myocardial ablation after aortic valve replacement

    Science.gov (United States)

    Sitges, M.; Kapadia, S.; Rubin, D. N.; Thomas, J. D.; Tuzcu, M. E.; Lever, H. M.

    2001-01-01

    When left ventricular outflow tract obstruction develops after aortic valve replacement, few treatment choices have been available until now. We present a patient with prior aortic valve replacement who developed left ventricle outflow tract obstruction that was successfully treated with a percutaneous transcoronary myocardial septal alcohol ablation. This technique is a useful tool for the treatment of obstructive hypertrophic cardiomyopathy, especially in those patients with prior heart surgery. Copyright 2001 Wiley-Liss, Inc.

  10. Effect of right ventricular pacing lead site on left ventricular function in patients with high-grade atrioventricular block: results of the Protect-Pace study.

    Science.gov (United States)

    Kaye, Gerald C; Linker, Nicholas J; Marwick, Thomas H; Pollock, Lucy; Graham, Laura; Pouliot, Erika; Poloniecki, Jan; Gammage, Michael

    2015-04-07

    Chronic right ventricle (RV) apical (RVA) pacing is standard treatment for an atrioventricular (AV) block but may be deleterious to left ventricle (LV) systolic function. Previous clinical studies of non-apical pacing have produced conflicting results. The aim of this randomized, prospective, international, multicentre trial was to compare change in LV ejection fraction (LVEF) between right ventricular apical and high septal (RVHS) pacing over a 2-year study period. We randomized 240 patients (age 74 ± 11 years, 67% male) with a high-grade AV block requiring >90% ventricular pacing and preserved baseline LVEF >50%, to receive pacing at the RVA (n = 120) or RVHS (n = 120). At 2 years, LVEF decreased in both the RVA (57 ± 9 to 55 ± 9%, P = 0.047) and the RVHS groups (56 ± 10 to 54 ± 10%, P = 0.0003). However, there was no significant difference in intra-patient change in LVEF between confirmed RVA (n = 85) and RVHS (n = 83) lead position (P = 0.43). There were no significant differences in heart failure hospitalization, mortality, the burden of atrial fibrillation, or plasma brain natriutetic peptide levels between the two groups. A significantly greater time was required to place the lead in the RVHS position (70 ± 25 vs. 56 ± 24 min, P < 0.0001) with longer fluoroscopy times (11 ± 7 vs. 5 ± 4 min, P < 0.0001). In patients with a high-grade AV block and preserved LV function requiring a high percentage of ventricular pacing, RVHS pacing does not provide a protective effect on left ventricular function over RVA pacing in the first 2 years. ClinicalTrials.gov number NCT00461734. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.

  11. Anatomia da valva atrioventricular esquerda: I. As cúspides Anatomy of the left atrio-ventricular valve: I. The cusps

    Directory of Open Access Journals (Sweden)

    Antônio B. Prado Fortuna

    1988-12-01

    Full Text Available Os resultados de um estudo anatômico da valva atrioventricular esquerda, realizado em 30 corações aparentemente normais, conservados em formalina, são comparados aos dados da literatura, tendo por objetivo adequar a nomenclatura e os conceitos anatômicos clássicos aos atuais, baseados na ultra-sonografia e na prática cirúrgica corrente. O presente artigo tratará apenas das cúspides, sendo que os demais elementos anatômicos dessa valva atrioventricular esquerda fazem parte de uma "unidade aortoventricular", onde distinguimos a cúspide anterior, em continuidade com o trígono intervalvar e o anel aórtico, e um grupo de cúspides posteriores, em relação com a parede posterior do ventrículo esquerdo. A cúspide anterior está separada do grupo de cúspides posteriores, tanto no polo valvar superior como no inferior, por uma estrutura anatômica bem definida: a "lâmina juncional", superior e inferior, respectivamente. As cúspides do grupo posterior são designadas pela ordem numeral ordinal, a partir da lâmina juncional superior. Esta nomenclatura tem por objetivo destacar a cúspide como unidade funcional da valva e facilitar as referências ecocardiográficas e cirúrgicas, particularmente nos casos em que mais de 3 cúspides posteriores são encontradas.A review of the left atrio-ventricular valve anatomy is compared to a clinical oriented morphological study on 30 normal adult heart, preserved in formalin. The results will be presented in five separated sections: I. The cusps; II. The comissures; III. The chordae tendinae; IV. The papilary muscles; V. The valvar annulus. The present publication will deal with the cusps. The terms "left atrio-ventricular valve" and "cusps" are to be prefered to the more descriptive and traditional "mitral valve" and "leaflets". The anterior cusp, in continuity with the aortic annulus, and a group of posterior cusps, attached to the posterior left wall, are described. An ordinal numerical

  12. Effects of beta-blockade on atrial and atrioventricular nodal refractoriness, and atrial fibrillatory rate during atrial fibrillation in pigs

    NARCIS (Netherlands)

    van den Berg, MP; van de Ven, LLM; Witting, W; Crijns, JGM; Haaksma, J; Bel, KJ; de Langen, CDJ; Lie, KI

    1997-01-01

    Despite their widespread use in atrial fibrillation, the effects of beta-adrenoceptor blockers on atrial and atrioventricular (AV) nodal refractoriness, and atrial fibrillatory rate during atrial fibrillation have been incompletely characterised. In particular, it is unknown whether additional

  13. Prevalence of cardiac dyssynchrony and correlation with atrio-ventricular block and QRS width in dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Anzouan-Kacou, J B; Ncho-Mottoh, M P; Konin, C

    2012-01-01

    Cardiac dyssynchrony causes disorganised cardiac contraction, delayed wall contraction and reduced pumping efficiency. We aimed to assess the prevalence of different types of dyssynchrony in patients with dilated cardiomyopathy (DCM), and to establish the correlation between atrio-ventricular blo...

  14. Septal co-infusions of glucose with a GABAB agonist impair memory

    OpenAIRE

    Erickson, Erika J.; Watts, Kelly D.; Parent, Marise B.

    2005-01-01

    Septal infusions of glucose exacerbate memory deficits produced by co-infusions of drugs that increase γ-aminobutyric acid (GABA)A receptor activity. To further understand the interaction between glucose and GABA, this experiment tested whether glucose would also potentiate spatial working memory deficits produced by septal infusions of the GABAB receptor agonist baclofen. Fifteen minutes prior to assessing spontaneous alternation (SA), male Sprague–Dawley derived rats were given septal infus...

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

    OpenAIRE

    Gokhan Aksan

    2016-01-01

    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 th...

  16. Atrioventricular block in coronary artery bypass surgery: perioperative predictors and impact on mortality

    Directory of Open Access Journals (Sweden)

    Ricardo Medeiros Piantá

    2015-04-01

    Full Text Available AbstractIntroduction:Disturbances of the cardiac conduction system are frequent in the postoperative period of coronary artery bypass surgery. They are mostly reversible and associated with some injury of the conduction tissue, caused by the ischemic heart disease itself or by perioperative factors.Objective:Primary: investigate the association between perioperative factors and the emergence of atrioventricular block in the postoperative period of coronary artery bypass surgery. Secondary: determine the need for temporary pacing and of a permanent pacemaker in the postoperative period of coronary artery bypass surgery and the impact on hospital stay and hospital mortality.Methods:Analysis of a retrospective cohort of patients submitted to coronary artery bypass surgery from the database of the Postoperative Heart Surgery Unit of the Sao Lucas Hospital of the Pontifical Catholic University of Rio Grande do Sul, using the logistic regression method.Results:In the period from January 1996 to December 2012, 3532 coronary artery bypass surgery were carried out. Two hundred and eighty-eight (8.15% of the total sample patients had atrioventricular block during the postoperative period of coronary artery bypass surgery, requiring temporary pacing. Eight of those who had atrioventricular block progressed to implantation of a permanent pacemaker (0.23% of the total sample. Multivariate analysis revealed a significant association of atrioventricular block with age above 60 years (OR=2.34; CI 95% 1.75-3.12; P<0.0001, female gender (OR=1.37; CI 95% 1.06-1.77; P=0.015, chronic kidney disease (OR=2.05; CI 95% 1.49-2.81; P<0.0001, atrial fibrillation (OR=2.06; CI 95% 1.16-3.66; P=0.014, functional class III and IV of the New York Heart Association (OR=1.43; CI 95% 1.03-1.98; P=0.031, perioperative acute myocardial infarction (OR=1.70; CI 95% 1.26-2.29; P<0.0001 and with the use of the intra-aortic balloon in the postoperative period of coronary artery bypass

  17. Do All Children with Congenital Complete Atrioventricular Block Require Permanent Pacing ?

    Directory of Open Access Journals (Sweden)

    Christian Balmer

    2003-07-01

    Full Text Available With an incidence of 1 in 20’000 live born infants1, congenital complete atrioventricular block (CCAVB is a rare disease. The aetiology is not completely understood. However, CCAVB may be isolated or combined with congenital heart diseases in up to 53% of affected individuals2. Isolated CCAVB is in up to 98% of the children associated with positive autoimmune antibodies in the maternal serum (anti-Ro/SS-A and anti-LA/SS-B3,4. Interestingly, these antibodies are not specifically directed against the conduction system but also against normal myocardial cells and may cause myocarditis5,6. Affection of the conduction system can occur at different levels7. Histologically, the atrioventricular node tissue may be replaced by fibrous fatty tissue with variable involvement of the distal conduction system8.

  18. Radiofrequency Catheter Ablation of Coexistent Idiopathic Left Ventricular Tachycardia and Atrioventricular Nodal Reentrant Tachycardia

    Directory of Open Access Journals (Sweden)

    Ken-Pen Weng

    2005-10-01

    Full Text Available A healthy 15-year-old male patient presented with a 6-month history of recurrent attacks of palpitations. On multiple emergency room visits, a sustained wide QRS complex tachycardia with a right bundle branch block and northwest axis deviation was documented. The tachycardia was not terminated by intravenous adenosine, but was suppressed with intravenous verapamil. There was no evidence of structural heart disease, myocarditis, long QT syndrome, or electrolyte imbalance after a series of standard examinations. Idiopathic left ventricular tachycardia (ILVT was suspected. Electrophysiologic studies revealed 2 inducible tachycardias, which were shown to represent atrioventricular nodal reentrant tachycardia (AVNRT and ILVT. Transformation from AVNRT to ILVT occurred spontaneously following atrial pacing. Successful ablation of ILVT and the slow atrioventricular nodal pathway resulted in cure of the double tachycardia.

  19. Novel insights on effect of atrioventricular programming of biventricular pacemaker in heart failure – a case series

    Directory of Open Access Journals (Sweden)

    Rafique Asim M

    2006-10-01

    Full Text Available Abstract Background Echocardiography plays an integral role in the diagnosis of congestive heart failure including measurement of left heart pressure as well as mechanical dyssynchrony. Methods In this report we describe novel therapeutic uses of echo pulsed wave Doppler in atrioventricular pacemaker optimization in patients who had either not derived significant symptomatic benefit post biventricular pacemaker implantation or deteriorated after deriving initial benefit. In these patients atrioventricular optimization showed novel findings and improved cardiac output and symptoms. Results In 3 patients with Cheyne Stokes pattern of respiration echo Doppler showed worsening of mitral regurgitation during hyperpneac phase in one patient, marked E and A fusion in another patient and exaggerated ventricular interdependence in a third patient thus highlighting mechanisms of adverse effects of Cheyne Stokes respiration in patients with heart failure. All 3 patients required a very short atrioventricular delay programming for best cardiac output. In one patient with recurrent congestive heart failure post cardiac resynchronization, mitral inflow pulse wave Doppler showed no A wave until a sensed atrioventricular delay of 190 ms was reached and showed progressive improvement in mitral inflow pattern until an atrioventricular delay of 290 ms. In 2 patients atrioventricular delay as short as 50 ms was required to allow E and A separation and prevent diastolic mitral regurgitation. All patients developed marked improvement in congestive heart failure symptoms post echo-guided biv pacemaker optimization. Conclusion These findings highlight the value of echo-guided pacemaker optimization in symptomatic patients post cardiac resynchronization treatment.

  20. Catheter ablation of atrial tachycardia after interatrial defect repair with patch apposition.

    Science.gov (United States)

    Nguyen, B L; Garante, C M; Tersigni, F; Sergiacomi, R; Petrassi, M; Di Matteo, A; Tufano, F; Alessandri, N

    2012-02-01

    A 54-year-old woman with history of septal atrial mixoma surgically treated and drug-refractory supraventricular tachyarrhythmia underwent catheter ablation of macro-reentry areas near the pericardial patch placed to repair an interatrial defect. The use of ablative therapy has been successful to cure this arrhythmia.

  1. Dual Atrioventricular Nodal Pathways Physiology: A Review of Relevant Anatomy, Electrophysiology, and Electrocardiographic Manifestations

    OpenAIRE

    Mani, Bhalaghuru Chokkalingam; Pavri, Behzad B.

    2014-01-01

    More than half a century has passed since the concept of dual atrioventricular (AV) nodal pathways physiology was conceived. Dual AV nodal pathways have been shown to be responsible for many clinical arrhythmia syndromes, most notably AV nodal reentrant tachycardia. Although there has been a considerable amount of research on this topic, the subject of dual AV nodal pathways physiology remains heavily debated and discussed. Despite advances in understanding arrhythmia mechanisms and the wides...

  2. A case of advanced second-degree atrioventricular block in a ferret secondary to lymphoma

    OpenAIRE

    Menicagli, F.; Lanza, A; F. Sbrocca; Baldi, A.; Spugnini, E.P.

    2016-01-01

    A female ferret was referred as an emergency for severe respiratory distress symptoms. At presentation, the patient was listlessness, dyspnoeic, and hyper-responsive. The clinical examination evidenced dyspnea with cyanosis, altered cardiac rhythm, and hepatomegaly. Electrocardiography showed an advanced second-degree atrioventricular (AV) block. The liver aspirate was diagnostic for lymphoma. The patient did not respond to supportive therapy and rapidly died. Post-mortem exams confirmed the ...

  3. Morfologia da junção atrioventricular em Iguana iguana (Reptilia-Iguanidae

    Directory of Open Access Journals (Sweden)

    Sonia Regina Jurado

    2006-06-01

    Full Text Available The atrioventricular junctional area (AVJA, including atrioventricular (AV node and bundle was investigated in seven hearts of common or green iguana (Iguana iguana using the light microscopy. Adult animals, both sexes, were captured in the Pantanal, Brazil. All hearts were fixed in buffered formaldehyde 10% (pH 7.2 for 24 hours, embedded in paraplast according to routine methods, and serially cut at 5 µm thickness. In the Iguana iguana, the AVJA consists of a mass of the fibers intermingled with variable amount of connective tissue and blood vessels surrounded by adjacent myocardium and the attachment of the right atrioventricular valve in the fibrous skeleton. By light microscopy, conducting cells of the AV node and bundle can be distinguished from working cells by their much smaller size, paler staining reaction and the presence of a sheath of connective tissue. The AV node and bundle and its branches were found to constitute a continuous tract. Histochemically, we found elastic fibers between cells of the conduction, mainly in the AV node. The PAS method reveals absence of glycogen in specialized cells. The fibrous skeleton, mainly the right trigone, showed a well-developed chondroid tissue, made by hyaline like cartilage (binucleated condrocytes included in the big lacunas and extracellular matrix with fibrillar collagen. In conclusion, the nodal and Purkinje cells in heart iguana presented poorly morphological differentiation comparing mammals and birds, however the skeleton fibrous has a different cartilage kind.

  4. Olfactory Bulb Volume Changes in Patients With Nasal Septal Deviation.

    Science.gov (United States)

    Özkiriş, Mahmut; Gencer, Zeliha Kapusuz; Aydin, Reha; Açikgöz, Mustafa; Saydam, Levent

    2017-05-01

    The olfactory bulb (OB) plays a pivotal role in the processing of olfactory information. The aim of this study was to investigate the OB volume changes and its possible associations with nasal septal deviation. Cross-sectional study. Otolaryngology Department of Bozok University School of Medicine and Neurology Department of Yozgat State Hospital. Ninety patient's cranial magnetic resonance imaging (MRI) studies (46 males and 44 females, mean age 36 ± 13.4 years; range 18-56 years) with isolated nasal septal deviations were recruited for the study. Olfactory bulb volumes in all study subjects were evaluated in T2-weighted coronal MRI images by planimetric manual contouring. Nasal septal deviation angles were found to range between 5° and 23.21° (mean 13.6° ± 3.58°). The right-sided deviations included 17 mild (Olfactory bulb volumes were calculated in both right- and left-sided deviation groups. In the patients with left-sided septal deviations of Groups I, II, and III, the left OB volumes of Groups I, II, and III were 46.49 ± 3.87, 47.46 ± 3.36, and 60.68 ± 5.65 mm and the right OB volumes were 53.37 ± 3.76, 56.47 ± 4.43, and 76.69 ± 6.84 mm, respectively. The statistical evaluation of the right OB volumes did not produce significant difference between Groups I and II (P = 0.73). The authors demonstrated statistically significant differences in comparison of Groups I to III and Groups II to III (P = 0.002 and P = 0.016, respectively). In the right septal deviation group for Groups I, II, and III, mean volumes of right OB volumes were 45.59 ± 4.46, 48.63 ± 3.78, and 61.35 ± 5.84 mm, respectively, and the left OB volumes were 54.67 ± 4.73, 57.65 ± 4.53, and 75.84 ± 7.67 mm, respectively. There was no statistically significant difference between Groups I and II (P = 0.95) left OB volumes in the right-sided deviation group, but statistically significant difference was

  5. Closure of patent foramen ovale defects using GORE® CARDIOFORM septal occluder

    DEFF Research Database (Denmark)

    Hardt, Stefan E; Eicken, Andreas; Berger, Felix

    2017-01-01

    (2.6%) were diagnosed new onset paroxysmal atrial fibrillation, which were successfully treated. No thrombembolic events occurred. Closure was successful in 94.2% of subjects at discharge evaluation and 96.9% at 6 months follow-up. CONCLUSION: This prospective, multicenter study adds to previous...

  6. Discharge planning for children with ventricular septal defect and pulmonary arterial hypertension in China

    Directory of Open Access Journals (Sweden)

    Xiaoxiao Wu

    2015-06-01

    Conclusions: Discharge planning improves the maternal discharge readiness, maternal caring knowledge and maternal caring behaviors. However, this planning did not reduce the readmission rate of children with CHD-PAH.

  7. Maternal MTHFR C677T genotype and septal defects in offspring ...

    African Journals Online (AJOL)

    Ghada M. Elsayed

    2013-10-09

    Oct 9, 2013 ... Abstract Background: While abnormal folate/homocysteine metabolism has been implicated as an etiology for the development of both CHD and DS, recent studies and meta-analyses did not consider MTHFR C677T genotype as a maternal risk factor for either of these conditions alone. Aim of work: To ...

  8. Maternal MTHFR C677T genotype and septal defects in offspring ...

    African Journals Online (AJOL)

    Abstract. Background: While abnormal folate/homocysteine metabolism has been implicated as an etiology for the development of both CHD and DS, recent studies and meta-analyses did not consider MTHFR C677T genotype as a maternal risk factor for either of these conditions alone. Aim of work: To investigate if ...

  9. Clinical Characteristics of Adult Uncorrected Secundum Atrial Septal Defect, A Pilot Study

    Directory of Open Access Journals (Sweden)

    Lucia Krisdinarti

    2016-12-01

    Defek septum atrium (DSA merupakan penyakit jantung bawaan yang paling sering dijumpai pada usia dewasa karena tanda dan gejalanya yang samar. Hipertensi paru (HP merupakan komplikasi DSA yang mendorong pasien mendatangi rumah sakit karena munculnya gejala yang berat. Hampir semua pasien DSA dewasa mengalami HP yang menyebabkan angka kesakitan dan kematian yang bermakna. Tujuan penelitian ini adalah untuk mengetahui karakteristik klinis pasien dengan DSA dan HP. Disain penelitian adalah potong lintang. Subjek dicuplik secara berurutan dari klinik rawat jalan dan rawat inap. Data demografi, medis dan pencitraan dikumpulkan dan dicatat dalam suatu case report form. Penelitian deskriptif dilakukan untuk menilai karakteristik subjek. Sebanyak tujuh puluh enam subjek dicuplik. Mayoritas subjek adalah wanita (77,6 % dalam usia muda, produktif dan subur (63,2 %. Gejala paling sering adalah sesak saat aktivitas, mudah lelah, dan berdebar. Sebagian besar pasien masuk dalam kelas fungsional WHO II (70,2 %. Rerata saturasi oksigen 96,4 %. Berdasar hasil ekhokardiografi, sebanyak 77,6 subjek telah mengalami HP. Rerata diameter terpanjang defek adalah 2,7 centimeter. Arah aliran darah melewati defek sebagian besar kiri ke kanan (77,6 %. Fungsi ventrikel kanan dan kiri dalam batas normal. Kateterisasi jantung kanan menunjukkan rerata tekanan atrium kiri sebesar 11,5 mmHg yang menandakan HP prekapiler/arteri. Rerata tekanan arteri pulmonalis sebesar 42.0 mmHg. Nilai indeks tahanan arteri pulmonalis sebagian besar kurang dari 4 Wood unit/m2 (63,7 % yang menunjukkan kemungkinan bisa dilakukan penutupan defek. Sedangkan 24,6 % subjek kontraindikasi untuk penutupan. Hipertensi arteri pulmonalis (HAP didiagnosis pada 77,6 % subjek, sedangkan 13,2 % subjek mengalami HAP borderline. Sebagai kesimpulan, sebagian besar DSA dewasa telah mengalami HAP, sebagian besar wanita muda yang berusia produktif, sebagian besar mengunjungi rumah sakit karena gejala HP, arah aliran sebagian besar dari kiri ke kanan dan sebagian besar telah mengalami penurunan kapasitas fungsional.

  10. Multiple coronary arteriovenous fistulae combined with ventricular septal defect: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Jung, Kun Sik; Zeon, Seok Kil; Kim, Ki Sik [Keimyung University, School of Medicine, Daegu (Korea, Republic of); Oh, Yeon Hee [Pohang Hospital, Dongguk University, Pohang (Korea, Republic of)

    1992-01-15

    Congenital coronary arteriovenous fistulas are all of those anomalies and malformations that result in a direct communication of a coronary artery and/or any of its branches with a cardiac chamber or extra cardiac vessel resulting in a shunt of varying proportions. The majority of these fistulas form an A-V connection between a coronary artery and the right heart chambers, and rarely their is communication with the left heart chambers. In most reported cases, the abnormal fistula involves only one coronary artery which developed alone. This report describes a unique case in which multiple coronary fistulas associated with VSD were encountered.

  11. [Congenital ventricular septal defect with late detection. Apropos of a case in an adult].

    Science.gov (United States)

    Bonnefoy, L; Chapoutot, L; Metz, D; Laudinat, J M; Doucet, J; Gandon, T; Guérin, R; Bajolet, A

    1989-03-01

    The authors report the case of a congenital interventricular communication discovered in a 75 year-old female patient. The shunt was identified on the cardiac Doppler and confirmed by catheterization. It was a type IIa interventricular communication which decompensated in the course of a pulmonary embolism. This case points out how rare is the diagnosis of congenital interventricular communication made during adulthood, and emphasizes the advantage of the Doppler in the diagnosis of ventricular shunts.

  12. Interruption of the aortic arch, ventricular septal defect, aortic atresia and aortopulmonary fistulous communication.

    Science.gov (United States)

    De Caro, E; Pongiglione, G; Ribaldone, D

    1998-06-01

    Interruption of the aortic arch in association with aortic atresia is a rare condition. We report the second case in literature in which survival was provided by an aortopulmonary fistulous communication.

  13. A hybrid technique for ventricular septal defect closure in young infants.

    Science.gov (United States)

    Bartel, Thomas; Tuzcu, E Murat

    2017-08-01

    Perventricular device closure is a hybrid surgical and transcatheter technique. Perventricular device closure is a safe and effective technique to close muscular VSDs even in infants. TEE guidance is the principal tool for intra-procedural guidance. © 2017 Wiley Periodicals, Inc.

  14. Effectiveness of caudal septal extension graft application in endonasal septoplasty

    Directory of Open Access Journals (Sweden)

    Yunus Karadavut

    Full Text Available Abstract Introduction Septal deviation is a common disease seen in daily otorhinolaryngology practice and septoplasty is a commonly performed surgical procedure. Caudal septum deviation is also a challenging pathology for ear, nose, and throat specialists. Many techniques are defined for caudal septal deviation. Objective To evaluate the effectiveness of caudal septal extension graft (CSEG application in patients who underwent endonasal septoplasty for a short and deviated nasal septum. Methods Forty patients with nasal septal deviation, short nasal septum, and weak nasal tip support who underwent endonasal septoplasty with or without CSEG placement between August 2012 and June 2013 were enrolled in this study. Twenty patients underwent endonasal septoplasty with CSEG placement. The rest of the group, who rejected auricular or costal cartilage harvest for CSEG placement, underwent only endonasal septoplasty without any additional intervention. Using the Nasal Obstruction Symptom Evaluation (NOSE and Rhinoplasty Outcome Evaluation (ROE questionnaires, pre- and post-operative acoustic rhinometer measurements were evaluated to assess the effect of CESG placement on nasal obstruction. Results In the control group, preoperative and postoperative minimal cross-sectional areas (MCA1 were 0.44 ± 0.10 cm2 and 0.60 ± 0.11 cm2, respectively (p < 0.001. In the study group, pre- and postoperative MCA1 values were 0.45 ± 0.16 cm2 and 0.67 ± 0.16 cm2, respectively (p < 0.01. In the control group, the nasal cavity volume (VOL1 value was 1.71 ± 0.21 mL preoperatively and 1.94 ± 0.17 mL postoperatively (p < 0.001. In the study group, pre- and postoperative VOL1s were 1.72 ± 0.15 mL and 1.97 ± 0.12 mL, respectively (p < 0.001. Statistical analysis of postoperative MCA1 and VOL1 values in the study and the control groups could not detect any significant intergroup difference (p = 0.093 and 0.432, respectively. In the study group, mean nasolabial angles were

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

    Directory of Open Access Journals (Sweden)

    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

  16. Saddle nose deformity and septal perforation in granulomatosis with polyangiitis.

    Science.gov (United States)

    Coordes, A; Loose, S M; Hofmann, V M; Hamilton, G S; Riedel, F; Menger, D J; Albers, A E

    2018-02-01

    Patients who have granulomatosis with polyangiitis (GPA, syn. M. Wegener) often develop an external nose deformity which may have devastating psychological effects. Therefore, reconstruction of nasal deformities by rhinoplasty may become necessary to achieve a normal appearance. The aim of this systematic review was to investigate the efficacy and safety of surgical reconstruction in external nasal deformities and septal perforation in GPA patients. A systematic literature search with defined search terms was performed for scientific articles archived in the MEDLINE-Database up to 10 June 2016 (PubMed Advanced MEDLINE Search), describing management of cases or case series in GPA patients with saddle nose deformity and/or septal perforation. Eleven of 614 publications met the criteria for this analysis including 41 GPA patients undergoing external nasal reconstruction and/or septal reconstruction with a median follow-up of 2.6 years. Overall, saddle nose reconstruction in GPA patients is safe even if an increased rate of revision surgery has to be expected compared with individuals without GPA undergoing septorhinoplasty. Most implanted grafts were autografts of calvarial bone or costal cartilage. For septal perforation reconstruction, few studies were available. Therefore, based on the available data for surgical outcomes, it is impossible to make evidence-based recommendations. All included GPA patients had minimal or no local disease at the time of reconstructive surgery. Therefore, the relationship between disease activity and its impact on surgical outcomes remains unanswered. The potential impact of immune-modulating medications on increased complication rates and the impact of prophylactic antibiotics are unknown. This study systematically reviews the efficacy and safety of surgical reconstruction of external nasal deformities in GPA patients for the first time. Saddle nose reconstruction in GPA patients with minimal or no local disease is a safe procedure

  17. Slow-fast Form of Atrioventricular Nodal Reentrant Tachycardia with Unusual Retrograde Activation in the Right Atrium —Possible Conduction Disturbance across the Tendon of Todaro Related to the Genesis of Positive Component of Biphasic Retrograde P Wave—

    Directory of Open Access Journals (Sweden)

    Kazuya Ishibashi, MD

    2006-01-01

    Full Text Available We report a rare case of slow-fast form of atrioventricular nodal reentrant tachycardia with delayed activation in the low septal right atrium (His bundle area. During supraventricular tachycardia (SVT, electrocardiogram showed “pseudo-positive P waves” in II, III, and aVF leads. SVT was induced by atrial extrastimulus with marked AH prolongation (i.e., jump phenomenon. Ventricular pacing showed a decremental retrograde conduction without jump phenomenon. Double atrial potentials were observed in the His bundle area during SVT and during ventricular pacing. The first electrogram of these split potentials, which was the earliest activation during SVT and during ventricular pacing, showed a dull and small deflection, whereas the second electrogram was sharp and clear. The interval of these discrete potentials was 70 msec during SVT. After a standard slow pathway ablation, SVT could never be induced by any programmed stimuli. It was concluded that in this case, the conduction disturbance across the tendon of Todaro was likely to cause the delayed atrial activation in the His bundle area, which created the pseudo-positive (biphasic. retrograde P wave.

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

    Directory of Open Access Journals (Sweden)

    Erfanul Saker

    2017-10-01

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

  19. Survival and sudden cardiac death after septal ablation for hypertrophic obstructive cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten Kvistholm; Havndrup, Ole; Hassager, Christian

    2011-01-01

    Reports of long-term survival and the risk of sudden cardiac death (SCD) after percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy (HOCM) are sparse.......Reports of long-term survival and the risk of sudden cardiac death (SCD) after percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy (HOCM) are sparse....

  20. Lower rate of selected congenital heart defects with better maternal diet quality: a population-based study.

    Science.gov (United States)

    Botto, Lorenzo D; Krikov, Sergey; Carmichael, Suzan L; Munger, Ronald G; Shaw, Gary M; Feldkamp, Marcia L

    2016-01-01

    To evaluate whether better diet quality in mothers is associated with lower risk for major non-syndromic congenital heart defects in their children. Multicentre population-based case-control study, the National Birth Defects Prevention Study. Ten sites in the USA. Mothers of babies with major non-syndromic congenital heart defects (n=9885) and mothers with unaffected babies (n=9468) with estimated date of delivery from 1997 to 2009. Adjusted ORs for specific major congenital heart defects by quartiles of maternal diet quality in the year before pregnancy, assessed by the Diet Quality Index for pregnancy (DQI-P) and the Mediterranean Diet Score. Quartile 1 (Q1) reflecting the worst diet quality and Q4 the best diet quality. Better diet quality was associated with reduced risk for some conotruncal and atrial septal heart defects. For DQI-P, estimated risks reductions (Q4 vs Q1) for conotruncal defects were 37% for tetralogy of Fallot (OR 0.63, 95% CI 0.49 to 0.80) and 24% overall (OR 0.76, 95% CI 0.64 to 0.91); and for septal defects, 23% for atrial septal defects (OR 0.77, 95% CI 0.63 to 0.94) and 14% overall (OR 0.86, 95% CI 0.75 to 1.00). Risk reductions were weaker or minimal for most other major congenital heart defects. Better diet quality is associated with a reduced occurrence of some conotruncal and septal heart defects. This finding suggests that a reduction in certain cardiac malformations may be an additional benefit of improved maternal diet quality, reinforcing current preconception care recommendations. 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/