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Sample records for atrioventricular septal defect

  1. Understanding atrioventricular septal defect: Anatomoechocardiographic correlation

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    Keirns Candace

    2008-06-01

    Full Text Available Abstract Objective Correlate the anatomic features of atrioventricular septal defect with echocardiographic images. Materials and methods Sixty specimen hearts were studied by sequential segmental analysis. Echocardiograms were performed on 34 patients. Specimen hearts with findings equivalent to those of echocardiographic images were selected in order to establish an anatomo-echocardiographic correlation. Results Thirty-three specimen hearts were in situs solitus, 19 showed dextroisomerism, 6 were in situs inversus and 2 levoisomerism. Fifty-eight had a common atrioventricular valve and 2 had two atrioventricular valves. Rastelli types were determined in 21 hearts. Nine were type A, 2 intermediate between A and B, 1 mixed between A and B, 4 type B and 5 type C. Associated anomalies included pulmonary stenosis, pulmonary atresia atrial septal defect, patent ductus arteriosus and anomalous connection of pulmonary veins. Echocardiograms revealed dextroisomerism in 12 patients, situs solitus in 11, levoisomerism in 7 and situs inversus in 4. Thirty-one patients had common atrioventricular valves and three two atrioventricular valves. Rastelli types were established in all cases with common atrioventricular valves; 17 had type A canal defects, 10 type B, 3 intermediate between A and B, 1 mixed between A and B and 3 type C. Associated anomalies included regurgitation of the atrioventricular valve, pulmonary stenosis, anomalous connection of pulmonary veins, pulmonary hypertension and pulmonary atresia. Conclusion Anatomo-echocardiographic correlation demonstrated a high degree of diagnostic precision with echocardiography.

  2. Atrioventricular septal defects among infants in Europe

    DEFF Research Database (Denmark)

    Christensen, Nikolas; Andersen, Helle; Garne, Ester

    2013-01-01

    2000-2008 were included. RESULTS: There was a total of 993 cases of atrioventricular septal defects, with a total prevalence of 5.3 per 10,000 births (95% confidence interval 4.1 to 6.5). Of the total cases, 250 were isolated cardiac lesions, 583 were chromosomal cases, 79 had multiple anomalies, 58...... had heterotaxia sequence, and 23 had a monogenic syndrome. The total prevalence of chromosomal cases was 3.1 per 10,000 (95% confidence interval 1.9 to 4.3), with a large variation between registers. Of the 993 cases, 639 cases were live births, 45 were stillbirths, and 309 were terminations...... of pregnancy owing to foetal anomaly. Among the groups, additional associated cardiac anomalies were most frequent in heterotaxia cases (38%) and least frequent in chromosomal cases (8%). Coarctation of the aorta was the most common associated cardiac defect. The 1-week survival rate for live births was 94...

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

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    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 incomplete atrioventricular septal defect

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    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.

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

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    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.

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

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    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.

  7. The Dorsal Mesenchymal Protrusion and the Pathogenesis of Atrioventricular Septal Defects

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    Burns, Tara; Yang, Yanping; Hiriart, Emilye; Wessels, Andy

    2017-01-01

    Congenital heart malformations are the most common type of defects found at birth. About 1% of infants are born with one or more heart defect on a yearly basis. Congenital Heart Disease (CHD) causes more deaths in the first year of life than any other congenital abnormality, and each year, nearly twice as many children die in the United States from CHD as from all forms of childhood cancers combined. Atrioventricular septal defects (AVSD) are congenital heart malformations affecting approximately 1 in 2000 live births. Babies born with an AVSD often require surgical intervention shortly after birth. However, even after successful surgery, these individuals typically have to deal with lifelong complications with the most common being a leaky mitral valve. In recent years the understanding of the molecular etiology and morphological mechanisms associated with the pathogenesis of AVSDs has significantly changed. Specifically, these studies have linked abnormal development of the Dorsal Mesenchymal Protrusion (DMP), a Second Heart Field-derived structure, to the development of this congenital defect. In this review we will be discuss some of the latest insights into the role of the DMP in the normal formation of the atrioventricular septal complex and in the pathogenesis of AVSDs. PMID:28133602

  8. Allelic Interaction between CRELD1 and VEGFA in the Pathogenesis of Cardiac Atrioventricular Septal Defects

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    Redig, Jennifer K.; Fouad, Gameil T.; Babcock, Darcie; Reshey, Benjamin; Feingold, Eleanor; Reeves, Roger H.; Maslen, Cheryl L.

    2014-01-01

    Atrioventricular septal defects (AVSD) are highly heritable, clinically significant congenital heart malformations. Genetic and environmental modifiers of risk are thought to work in unknown combinations to cause AVSD. Approximately 5–10% of simplex AVSD cases carry a missense mutation in CRELD1. However, CRELD1 mutations are not fully penetrant and require interactions with other risk factors to result in AVSD. Vascular endothelial growth factor-A (VEGFA) is a well-characterized modulator of heart valve development. A functional VEGFA polymorphism, VEGFA c.–634C, which causes constitutively increased VEGFA expression, has been associated with cardiac septal defects suggesting it may be a genetic risk factor. To determine if there is an allelic association with AVSD we genotyped the VEGFA c.–634 SNP in a simplex AVSD study cohort. Over-representation of the c.–634C allele in the AVSD group suggested that this genotype may increase risk. Correlation of CRELD1 and VEGFA genotypes revealed that potentially pathogenic missense mutations in CRELD1 were always accompanied by the VEGFA c.–634C allele in individuals with AVSD suggesting a potentially pathogenic allelic interaction. We used a Creld1 knockout mouse model to determine the effect of deficiency of Creld1 combined with increased VEGFA on atrioventricular canal development. Morphogenic response to VEGFA was abnormal in Creld1-deficient embryonic hearts, indicating that interaction between CRELD1 and VEGFA has the potential to alter atrioventricular canal morphogenesis. This supports our hypothesis that an additive effect between missense mutations in CRELD1 and a functional SNP in VEGFA contributes to the pathogenesis of AVSD. PMID:25328912

  9. Allelic Interaction between CRELD1 and VEGFA in the Pathogenesis of Cardiac Atrioventricular Septal Defects

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    Jennifer K. Redig

    2014-03-01

    Full Text Available Atrioventricular septal defects (AVSD are highly heritable, clinically significant congenital heart malformations. Genetic and environmental modifiers of risk are thought to work in unknown combinations to cause AVSD. Approximately 5–10% of simplex AVSD cases carry a missense mutation in CRELD1. However, CRELD1 mutations are not fully penetrant and require interactions with other risk factors to result in AVSD. Vascular endothelial growth factor-A (VEGFA is a well-characterized modulator of heart valve development. A functional VEGFA polymorphism, VEGFA c.-634C, which causes constitutively increased VEGFA expression, has been associated with cardiac septal defects suggesting it may be a genetic risk factor. To determine if there is an allelic association with AVSD we genotyped the VEGFA c.-634 SNP in a simplex AVSD study cohort. Over-representation of the c.-634C allele in the AVSD group suggested that this genotype may increase risk. Correlation of CRELD1 and VEGFA genotypes revealed that potentially pathogenic missense mutations in CRELD1 were always accompanied by the VEGFA c.-634C allele in individuals with AVSD suggesting a potentially pathogenic allelic interaction. We used a Creld1 knockout mouse model to determine the effect of deficiency of Creld1 combined with increased VEGFA on atrioventricular canal development. Morphogenic response to VEGFA was abnormal in Creld1-deficient embryonic hearts, indicating that interaction between CRELD1 and VEGFA has the potential to alter atrioventricular canal morphogenesis. This supports our hypothesis that an additive effect between missense mutations in CRELD1 and a functional SNP in VEGFA contributes to the pathogenesis of AVSD.

  10. Polymorphic haplotypes of CRELD1 differentially predispose Down syndrome and euploids individuals to atrioventricular septal defect.

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    Ghosh, Priyanka; Bhaumik, Pranami; Ghosh, Sujoy; Ozbek, Umut; Feingold, Eleanor; Maslen, Cheryl; Sarkar, Biswanath; Pramanik, Vishmadeb; Biswas, Priyanka; Bandyopadhyay, Biswajit; Dey, Subrata Kumar

    2012-11-01

    To explore the role of CRELD1 variants on congenital heart defects, we sequenced the entire reading frame of CRELD1 in the samples from Kolkata and adjoining areas. Nearly, 400 participants were included in the genetic association study and they were stratified as Down syndrome (DS) with atrioventricular septal defect (AVSD), DS without AVSD, euploid with AVSD, and euploid without AVSD. A significant association was found between AVSD and three polymorphisms, namely rs9878047 (c.1049-129T > C), rs3774207 (c.1119C > T), and rs73118372 (c.1136T > C) among the Down syndrome and euploid individuals. The polymorphism rs73118372, involves a transition (c.1136T > C) that leads to change in amino acid methionine to threonine which alters protein secondary structure as confirmed by the bioinformatics software SOPMA. In addition, two haplotypes, C-T-C and C-T-T, in the order of loci rs9878047-rs3774207-rs73118372 were associated with incidence of AVSD among euploid and Down syndrome, with a slightly higher odds ratio in the later group. We hypothesize that these haplotypes increase the risk of AVSD, and the susceptibility is exacerbated in DS, possibly due to the trisomy 21 genetic background. Moreover, we report for the first time on an interaction between the mutant alleles of rs3774207 and rs73118372 which could disrupt the delicate balance between different CRELD1 isoforms.

  11. Natural and modified history of complete atrioventricular septal defect--a 17 year study.

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    Frontera-Izquierdo, P; Cabezuelo-Huerta, G

    1990-01-01

    We reviewed 103 cases of isolated complete atrioventricular septal defect. These cases represented 4.4% of the cases of congenital heart disease diagnosed in our hospital by catheterisation and angiography during 1971-88. Most children (n = 76) had Down's syndrome. Banding of the pulmonary artery was performed in seven cases and complete repair in 67 cases. In the period 1971-82 the complete correction was performed at a mean age of 23 months with a surgical mortality of 88.8%. In the period 1983-8 the mean age at complete correction was 13 months, the mortality 43.2%, and the five year actuarial survival was 46.8%. The 22 patients that survived after complete correction were in functional classes I and II of the New York Heart Association classification. After a mean follow up of 10 years only eight (36%) of the 22 who were followed up and treated medically survived; all had developed pulmonary vascular obstructive disease and were in functional classes III or IV. Our findings stress the importance of early complete surgical repair. PMID:2221969

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

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    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

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

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    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.

  14. An Excess of Deleterious Variants in VEGF-A Pathway Genes in Down-Syndrome-Associated Atrioventricular Septal Defects

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    Ackerman, Christine; Locke, Adam E.; Feingold, Eleanor; Reshey, Benjamin; Espana, Karina; Thusberg, Janita; Mooney, Sean; Bean, Lora J.H.; Dooley, Kenneth J.; Cua, Clifford L.; Reeves, Roger H.; Sherman, Stephanie L.; Maslen, Cheryl L.

    2012-01-01

    About half of people with trisomy 21 have a congenital heart defect (CHD), whereas the remainder have a structurally normal heart, demonstrating that trisomy 21 is a significant risk factor but is not causal for abnormal heart development. Atrioventricular septal defects (AVSD) are the most commonly occurring heart defects in Down syndrome (DS), and ∼65% of all AVSD is associated with DS. We used a candidate-gene approach among individuals with DS and complete AVSD (cases = 141) and DS with no CHD (controls = 141) to determine whether rare genetic variants in genes involved in atrioventricular valvuloseptal morphogenesis contribute to AVSD in this sensitized population. We found a significant excess (p < 0.0001) of variants predicted to be deleterious in cases compared to controls. At the most stringent level of filtering, we found potentially damaging variants in nearly 20% of cases but fewer than 3% of controls. The variants with the highest probability of being damaging in cases only were found in six genes: COL6A1, COL6A2, CRELD1, FBLN2, FRZB, and GATA5. Several of the case-specific variants were recurrent in unrelated individuals, occurring in 10% of cases studied. No variants with an equal probability of being damaging were found in controls, demonstrating a highly specific association with AVSD. Of note, all of these genes are in the VEGF-A pathway, even though the candidate genes analyzed in this study represented numerous biochemical and developmental pathways, suggesting that rare variants in the VEGF-A pathway might contribute to the genetic underpinnings of AVSD in humans. PMID:23040494

  15. 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

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    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

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

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    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.

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

    Science.gov (United States)

    Priest, James R.; Osoegawa, Kazutoyo; Mohammed, Nebil; Nanda, Vivek; Kundu, Ramendra; Schultz, Kathleen; Girirajan, Santhosh; Scheetz, Todd; Waggott, Daryl; Haddad, Francois; Reddy, Sushma; Bernstein, Daniel; Burns, Trudy; Steimle, Jeffrey D.; Yang, Xinan H.; Moskowitz, Ivan P.; Hurles, Matthew; Lifton, Richard P.; Nickerson, Debbie; Bamshad, Michael; Eichler, Evan E.; Mital, Seema; Sheffield, Val; Quertermous, Thomas; Gelb, Bruce D.; Portman, Michael; Ashley, Euan A.

    2016-01-01

    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. PMID:27058611

  18. Genetics progress on atrioventricular septal defect%先天性房室间隔缺损的遗传学进展

    Institute of Scientific and Technical Information of China (English)

    李晓维

    2011-01-01

    先天性房室间隔缺损(atrioventricular sepial defect,AVSD)是一种常见的心血管畸形,由于存在房室间隔(房间隔下部、室间隔上部)以及中央心内膜垫组织的缺损,造成左右心腔之间的异常交通.虽然AVSD的胚胎学、病理生理学以及诊断和治疗目前都已基本阐明,但其确切的发病机制仍无突破性进展.随着分子生物学技术的广泛应用以及分子遗传学研究的深入开展,AVSD在遗传学的研究中取得一系列新进展,一些基因被证实与AVSD的发生发展存在一定的相关性.%Atrioventricular septal defect (AVSD) is a common cardiovascular malformation because of atrioventricular septal (lower atrial septum, ventricular septal upper) and the endocardial cushion defect,resulting in abnormal chambers of the heart. At the present, although the embryology, pathophysiology,diagnosis and treatment of the AVSD are clarified, but its precise pathogenesis has still no breakthrough progress.With the wide application of molecular biology and the depth research of molecular genetics, a series of new progress about AVSD has been made in the genetic study, and some genes are confirmed to be related to the occurrence and development of AVSD. The aim of this article is to review and discuss genetic mechanisms and related genes of AVSD, and to further identify the major genes causing AVSD.

  19. Ventricular septal defect (image)

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    Ventricular septal defect is a congenital defect of the heart, that occurs as an abnormal opening in ... wall that separates the right and left ventricles. Ventricular septal defect may also be associated with other ...

  20. Analysis of Selected Maternal Exposures and Non-Syndromic Atrioventricular Septal Defects in the National Birth Defects Prevention Study, 1997–2005

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    Patel, Sonali S.; Burns, Trudy L.; Botto, Lorenzo D.; Riehle-Colarusso, Tiffany J.; Lin, Angela E.; Shaw, Gary M.; Romitti, Paul A.

    2015-01-01

    Although the descriptive epidemiology of atrioventricular septal defects (AVSDs), a group of serious congenital heart defects (CHDs), has been recently reported, non-genetic risk factors have not been consistently identified. Using data (1997–2005) from the National Birth Defects Prevention Study, an ongoing multisite population-based case–control study, the association between selected non-genetic factors and non-syndromic AVSDs was examined. Data on periconceptional exposures to such factors were collected by telephone interview from 187 mothers of AVSD case infants and 6,703 mothers of unaffected infants. Adjusted odds ratios (aORs) and 95% confidence intervals (CIs) were estimated from logistic regression models. Mothers who reported cigarette smoking during the periconceptional period were more likely to have infants with AVSDs compared with non-smokers, independent of maternal age, periconceptional alcohol consumption, infant gestational age, family history of CHDs, and study site (aOR 1.5, 95% CI 1.1–2.4). The association was strongest in mothers who smoked more than 25 cigarettes/day. In addition, mothers with periconceptional passive smoke exposure were more likely to have infants with AVSDs than unexposed mothers, independent of maternal age, active periconceptional smoking, infant gestational age, and family history of CHDs (aOR 1.4, 95% CI 1.0–2.0). No associations were observed between AVSDs and maternal history of a urinary tract infection or pelvic inflammatory disease, maternal use of a wide variety of medications, maternal occupational exposure, parental drug use, or maternal alcohol consumption. If the results of this preliminary study can be replicated, minimizing maternal active and passive smoke exposure may decrease the incidence of AVSDs. PMID:22903798

  1. Correção biventricular em defeito do septo atrioventricular desbalanceado Corrección biventricular en defecto del septo atrioventricular desbalanceado Biventricular repair in unbalanced atrioventricular septal defect

    Directory of Open Access Journals (Sweden)

    Edmar Atik

    2009-04-01

    Full Text Available É apresentada a evolução favorável, após correção operatória biventricular, de criança com 2,5 anos de idade, com defeito do septo atrioventricular desbalanceado, com ventrículo esquerdo (VE pequeno (anel mitral de 10 mm em relação de 0,4 com o anel tricúspide, DDVE de 17 mm, Vd2 VE de 15 ml/m² e relação do índice longitudinal VE/VD de 0,71. Houve desenvolvimento normal do VE, verificado três meses após a operação (anel mitral de 22 mm, em relação de 0,84 com o da valva tricúspide e DDVE de 30 mm. Discutem-se os parâmetros atuais de utilização do ventrículo hipoplásico.Presenta evolución favorable después de corrección operatoria biventricular, de niño con 2,5 años de edad, con Defecto del Septo Atrioventricular Desbalanceado con ventrículo izquierdo (VI pequeño (anillo mitral de 10 mm en relación de 0,4 con el anillo tricúspide, DDVI de 17 mm, Vd2 VI de 15 ml/m2 y relación de ejes longitudinales VI/VD de 0,71. Hubo desarrollo normal del VI, verificado tres meses después de la operación (anillo mitral de 22 mm, en relación de 0,84 con el de la válvula tricúspide y DDVI de 30 mm. Se discuten los parámetros actuales de utilización del ventrículo hipoplásico.We present the case of a 2.5 year-old child with unbalanced atrioventricular septal defect due to a small left ventricle (LV (mitral annulus of 10mm and a 0.4 ratio in relation to the tricuspid annulus, LVDD: 17 mm, LV Vd2: 15 ml/m² and LV/RV long-axis ratio of 0.71; he had a favorable outcome after biventricular surgical repair. Normal LV development was observed three months after the operation (mitral annulus of 22 mm, with a 0.84 ratio in relation to the tricuspid annulus, and LVDD of 30 mm. Current parameters for utilization of the hypoplastic ventricle are discussed.

  2. Ventricular Septal Defect (For Parents)

    Science.gov (United States)

    ... Electrocardiogram) Anesthesia - What to Expect Tetralogy of Fallot Coarctation of the Aorta Patent Ductus Arteriosus (PDA) Getting ... Murmurs Atrial Septal Defect Cardiac Catheterization EKG (Video) Coarctation of the Aorta Atrial Septal Defect Ventricular Septal ...

  3. Congenital defect of the partial atrioventricular canal with Klinefelter syndrome

    OpenAIRE

    Zhang, Yejing

    2009-01-01

    The case of a 25-year-old man with a partial atrioventricular canal defect (PAVCD) with Klinefelter syndrome is reported here. The patient had Klinefelter syndrome associated with an atrial septal defect and the cleft of the anterior leaflet of the mitral valve.

  4. [Partial closure of the atrial septal primum defect and biventricular repair for a case of hypoplastic right ventricle with partial atrioventricular canal defect].

    Science.gov (United States)

    Oshima, Y; Yamaguchi, M; Imai, M; Ohashi, H; Tsukube, T; Hosokawa, Y

    1990-11-01

    An eight-year-old girl with partial atrioventricular canal defect and hypoplastic right ventricle was treated successfully by a palliative open-heart surgery. The preoperative right ventricular pressure was 58/7 mmHg (RVP/LVP = 0.67) and morphology of the right ventricle showed severe tricuspid stenosis and small outflow portion. The preoperative RVEDVI was 31 (41% of normal and tricuspid annulus was 17 mm (47% of normal). The right ventricular outflow was reconstructed with insertion of MVOP and the ASD was partially left open (the amplitude of the interatrial communication was 7 mm). Angiocardiogram two and half years after the operation demonstrated significant right ventricular growth with no right to left shunt through interatrial communication. The RVEDVI was 46 (56% of normal) and tricuspid annulus was 36 mm (90% of normal). This technique can be a procedure of choice in patient with right ventricular hypoplasia, who is not candidate for simple right ventricular reconstruction nor Fontan procedure, as the growth of the right ventricule is expected in the future.

  5. Postoperative follow up of patients with complete atrioventricular septal defect%完全性房室间隔缺损修补术后随访分析

    Institute of Scientific and Technical Information of China (English)

    肖婷婷; 李奋; 黄美蓉; 余志庆; 杨健萍; 陈笋; 张志芳

    2009-01-01

    Objective To observe the operative efficacy of patients with complete atrioventricular septal defect (CAVSD). Methods From January 2003 to June 2006, CAVSD patients underwent operative closure were included in this study. Color Doppler with apical four-chamber view was used to evaluate the degree of valve insufficiency before surgery and 2 days, 1 month, 6 months and 1 year after the surgery. Cardiac catheterization was performed to evaluate pulmonary artery pressure and pulmonary arteriolar resistance (PAR) before surgery in patients whose age were over 6 months. The time of staying at ICU, ventilation time after surgery and the occurrence of pulmonary artery hypertension crisis were recorded. Results 105 CAVSD patients underwent operative closure were enrolled in this study. The mean staying time at ICU was (4.7±2.4) days, and the mean ventilation time was (1.7±1.0) days, 9 patients (8.5%) developed pulmonary artery hypertension crisis after surgery. Patients with PAR > 8 Wood unit were older, staying time at ICU and ventilation time were longer compared patients with PAR 8 Wood unit compared patients with PAR 8 Wood unit was associated with increased risk of pulmonary artery hypertension crisis after surgery in patients with CAVSD.%目的 探讨完全性房室间隔缺损修补术的疗效.方法 对2003年1月至2006年6月行完全性房室间隔缺损修补术患儿进行随访分析.通过超声心动图心尖四腔切面评估术前,术后第2天、1个月、6个月及1年的房室瓣反流程度.大于6个月患儿术前行心导管检查了解肺动脉压力及肺小动脉阻力(PAR).记录术后监护时间、呼吸机使用时间及肺动脉高压危象发生情况.结果 105例完全性房室间隔缺损患儿术后监护(4.7±2.4)d,呼吸机使用(1.7±1.0)d,出现肺动脉高压危象9例(8.5%).PAR>8 Wood单位患儿与PAR≤8 Wood单位患儿比较,年龄较大,术后监护时间、呼吸机维持时间较长,肺动脉高

  6. Ventricular Septal Defect (For Teens)

    Science.gov (United States)

    ... upper filling chambers are the atria . In normal circulation, blood that returns from the body to the ... Causes a VSD? Ventricular septal defects occur during fetal heart development and are present at birth. During ...

  7. Atrial Septal Defect (For Teens)

    Science.gov (United States)

    ... septal defect (pronounced: AY-tree-ul SEP-tul DEE-fekt), or ASD for short, is sometimes referred ... can be treated with cardiac catheterization (pronounced: CAR-dee-ack cath-uh-turr-ih-ZAY-shun), in ...

  8. 室间隔缺损介入术后并发三度房室阻滞%Third-degree atrioventricular block after transcatheter closure of ventricular septal defect

    Institute of Scientific and Technical Information of China (English)

    白元; 宗刚军; 秦永文

    2009-01-01

    目的 分析国内室间隔缺损患者介入封堵术后三度房室阻滞的发生特点和原因.方法通过中国生物医学文献数据库和清华同方医学期刊数据库文献检索系统,以"室间隔缺损"和"传导"为任意字段进行检索,在纳入的文献中,重点对患者年龄、性别、术后三度房室阻滞的发生时间、恢复时间、处理措施等进行总结分析.结果 总计纳入23篇文献,共报道了73例术后出现三度房室阻滞的患者,女性和儿童多见.88%的三度房室阻滞发生于术后3~7 d.80%的病例均在症状出现后的7~15 d恢复窦性心律.有3例患者植入了永久起搏器.结论 室间隔缺损介入术后的三度房室阻滞有其发生发展的规律,多数为一过性,需植入永久性起搏器的患者极少.%Objective To observe clinical characteristics of third-degree atrioventricular block (AVB Ⅲ) after transcatheter closure of ventricular septal defect in China. Methods All the data were collected from the Chinese Bio-Medical Data-base and the Chinese Journal Full-text Data-base. The search term (all fields) used was "ventricular septal defect and conduction". The major items included age, sex, size of ven-tricular septal defect and the features of postoperative AVB Ⅲ. Results Twenty-three articles were reviewed in which the emphasis of analysis was conduction disorders after transcatheter closure of ventricular septal de-fect. There were 73 cases of postoperative AVB Ⅲ of the total 73 patients, most of them were women and chil-dren. In 88% of patients the AVB Ⅲ occurred in the first 3~7 day after surgery. AVB Ⅲ reversed during the 7~15 day after the onset of it in 80% of patients. Three patients required permanent pacemaker implantation due to irreversible AVB Ⅲ. Conclusion Postoperative AVB Ⅲ was transient in most of cases. The require-ment of permanent pacemaker implantation after closure is a rare but serious complication.

  9. Facts about Atrial Septal Defect

    Science.gov (United States)

    ... Living With Heart Defects Data & Statistics Tracking & Research Articles & Key Findings Free Materials Multimedia and Tools Links to Other Websites Information For... Media Policy Makers Facts about Atrial Septal Defect Language: English Español (Spanish) Recommend on Facebook Tweet Share Compartir ...

  10. Facts about Ventricular Septal Defect

    Science.gov (United States)

    ... Living With Heart Defects Data & Statistics Tracking & Research Articles & Key Findings Free Materials Multimedia and Tools Links to Other Websites Information For... Media Policy Makers Facts about Ventricular Septal Defect Language: English Español (Spanish) Recommend on Facebook Tweet Share Compartir ...

  11. 经胸房间隔缺损堵闭术对房室瓣反流的影响%Effect of transthoracic atrial septal defect closure on atrioventricular valve regurgitation

    Institute of Scientific and Technical Information of China (English)

    张家庆; 闫玉生; 莒瑞红; 陈坤棠

    2012-01-01

    Objectives To explore how transthoracic atrial septal defect closure affect atrioventricular valve regurgitation. Methods Retrospective reviews of 43 patients who underwent closure of atrial septal defects in Zhujiang Hospital from January 2002 to March 2011 were included in this study. Among them,40 patients were guided by transesophageal echocardiography, while 2 patients were observed by transthoracic echocardiography. All the patients' cardio parameters, complications and extent of atrioventricular valve regurgitation were followed up with echocardiography before and after operation. Results Of the 43 patients,41 (95.3%) cases were successful, 1 patient was given right transthoracic minimally invasive closure of atrial septal defects under cardiopulmonary bypass, and 1 patient died of cardiac arrest during operation. Acute renal failure appeared in 1 patient 2 days afer operation. Twelve patients were observed leakage from left atrial to right atrial immediately after operation, but the syndrome disappeared one month later. Transthoracic echocardiography demonstrated that right atrial diameter and right ventricular diameter decreased, while left ventricular diameter enlarged, pulmonary blood velocity reduced sharply. The differences were significant (P0.05). Mitral vaive regurgitation became worse at the time of 1 month and 6 months after operation. The difference was significant when compared with that before operation (rank; 2.01 vs. 2.17 vs. 1.77, ^ = 10.78 ,P=Q.04). Meanwhile, tricuspid valve regurgitation hardly changed (rank; 1.88 vs. 2.11 vs. 2.01, ^2=4.23 ,P=0.134). Conclusions Transthoracic closure of atrial septal defects may worsen mitral valve regurgitation, while it has no obvious influence on tricuspid valve regurgitation for a short time. Patients who had moderate or above mitral valve regurgitation or severe tricuspid valve regurgitation may not be suitable for isolated atrial septal defect closure.%目的 探讨经胸房间隔缺损封堵术

  12. Atrial – Ventricular Septal Defect

    Directory of Open Access Journals (Sweden)

    T Panagiotopoulos

    2009-05-01

    Full Text Available Atrial and ventricular septal defect constitute the most common congenital heart disease.Aim: Τhe aim of the present retrospective study was to record data and factors that affect atrial and ventricular septal defect.Method and material: The sample study included patients of both sexes who were hospitalized with diagnosis atrial and ventricular septal defect in a Cardiac Surgery hospital of Athens. A specially constructed printed form was used for data collection, where were recorded the demographic and personal variables, the pathological, surgical, cardiology and obstetric history, the habits of adults, as well as the personal characteristics of mothers. Analysis of data was performed by descriptive statistical analysis.Results: The sample study consisted of 101 individuals with diagnosis atrial or ventricular Septal Defect, of which 40% were boys and 60% girls. The 70% of the sample study suffered from atrial Septal Defect and the 30% suffered from ventricular Septal Defect. Regarding age, 12% of the sample study was 0-1 years old, 35% was >1 years old, 8% was >12-18 years old and 45% over than 18 years old. Regarding educational status of the adult participants, 9% was of 0-6 years education, 22%>6 -12 years, 13%>12 years. 14% of the adult paticipants smoked, 4% consumed alcohol and 5% smoked in conjunction with alcohol. In terms of the obstetric history of the sample studied, 32% of the cases had normal birth, 4% had a twin birth and 1% had a triplet one. According to the variables related to mothers, the mean age of the mother was 30 years and 3 months, 10% were smokers at pregnancy and 3% used chemical substance and mainly hair color. Also, the results of the present study showed that individuals of 12-18 and >18 years old did not suffer from ventricular Septal Defect, whereas the infants 0-1 years old did not suffer from Atrial Septal Defect. The mean value of age at the admission in intensive care unit was 7 months (12% for the infants

  13. Value of Prenatal Ultrasound in the Diagnosis of Fetal Complete Atrioventricular Septal Defect%产前超声诊断胎儿完全性房室间隔缺损的价值

    Institute of Scientific and Technical Information of China (English)

    刘志红; 冷萍; 曾宪敏

    2012-01-01

    目的 探讨产前超声心动图诊断胎儿完全性房室间隔缺损(cAVSD)的临床价值.资料与方法 回顾性分析2006~2010产前超声诊断的21例cAVSD胎儿的超声资料,分析其与染色体异常的关系及胎儿的临床结局.结果 产前超声显示12例为单纯cAVSD,9例合并其他结构异常,即复合型cAVSD.12例单纯cAVSD胎儿中2例核型异常,9例复合型房室间隔缺损胎儿中6例核型异常.经产前咨询后,9例孕妇选择终止妊娠;12例胎儿中,4例宫内死亡,出生的8例胎儿中,2例未接受手术在新生儿期死亡;6例接受手术,4例存活均为单纯cAVSD.结论 胎儿cAVSD常合并其他结构畸形,且与染色体异常关系密切.复合型cAVSD结局较差,单纯型cAVSD结局相对较好,但围生期胎儿丢失率超过50%.%Purpose To investigate the diagnostic value of prenatal ultrasound in fetal complete atrioventricular septal defect (cAVSD). Materials and Methods The ultrasonic data of 21 cases of cAVSD diagnosed by prenatal ultrasound from 2006 to 2010 were retrospectively analyzed, and the relationship with chromosomal abnormality as well as fetal clinical outcomes were also explored. Results There were 12 sole cAVSD and 9 complicated with other malformations (complicated cAVSD). Fetal karyotype analysis: 2 with sole cAVSD had abnormal karyotype, and 6 out of the 9 complicated cAVSD had chromosomal abnormalities. Following prenatal counseling, 9 couples chose termination. Of the 12 continuing pregnancies, 4 were lost to follow-up. Of the 8 live births, 2 were neonatal deaths without surgery while 6 babies underwent surgery and 4 had survived to date. All the survivors were sole cAVSD. Conclusion cAVSD is associated with chromosomal and other abnormalities. The outcome of complicated cAVSD is poor, and the outcome of sole cAVSD is better. But the survivors don't reach half of the sole cAVSD.

  14. 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....

  15. [Permanent cardiac stimulation in a patient with isolated dextrocardia and ventricular septal defect].

    Science.gov (United States)

    Słowiński, S; Derlaga, B; Kapusta, J

    A case of pacemaker implantation because of acquired atrioventricular block third degree in woman aged 39 years with rare isolated dextrocardia with inversion of cardiac ventricles, compensatory transposition of both aorta and pulmonary artery, and ventricular septal defect. The end of electrode has wedged in the apex of the arterial ventricle. Following pacemaker implantation, patients clinical course of gynaecological operation was uneventful.

  16. Facts about Birth Defects

    Science.gov (United States)

    ... Heart Defects Atrial Septal Defect Atrioventricular Septal Defect Coarctation of the Aorta D-Transposition of the Great ... Defects Fetal Alcohol Syndrome Disorders Gastroschisis Heart Defects Coarctation of the Aorta Hypoplastic left heart syndrome Tetralogy ...

  17. Echocardiographic assessment and percutaneous closure of multiple atrial septal defects

    Directory of Open Access Journals (Sweden)

    Timperley Jonathan

    2004-07-01

    Full Text Available Abstract Atrial septal defect closure is now routinely performed using a percutaneous approach under echocardiographic guidance. Centrally located, secundum defects are ideal for device closure but there is considerable morphological variation in size and location of the defects. A small proportion of atrial septal defects may have multiple fenestrations and these are often considered unsuitable for device closure. We report three cases of multiple atrial septal defects successfully closed with two Amplatzer septal occluders.

  18. National Birth Defects Prevention Study (NBDPS)

    Science.gov (United States)

    ... Heart Defects Atrial Septal Defect Atrioventricular Septal Defect Coarctation of the Aorta D-Transposition of the Great ... Defects Fetal Alcohol Syndrome Disorders Gastroschisis Heart Defects Coarctation of the Aorta Hypoplastic left heart syndrome Tetralogy ...

  19. Prenatal diagnosis of fetal aortopulmonary septal defect with ventricular septal defect by two-dimension echocardiography

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    @@ Fetal aortopulmonary septal defect (APSD) is an extremely rare condition, accounting for 0.1%-0.2% of all cardiac defects in live births world wide.1 Hospital mortality is 13% and 33% for simple and complex APSD, respectively.2 This rare cardiac defect refers to a congenital malformation in the development of the arteriosus truncus septum, and is usually associated with a wide variety of other structural cardiac anomalies such as ventricular septal defect (VSD), pulmonary valve stegnosis and so on.3 Prenatal diagnosis of an APSD is possible by echocardiography.

  20. Facts about Upper and Lower Limb Reduction Defects

    Science.gov (United States)

    ... Heart Defects Atrial Septal Defect Atrioventricular Septal Defect Coarctation of the Aorta D-Transposition of the Great ... Defects Fetal Alcohol Syndrome Disorders Gastroschisis Heart Defects Coarctation of the Aorta Hypoplastic left heart syndrome Tetralogy ...

  1. Muscular ventricular septal defects: A reappraisal of the anatomy

    NARCIS (Netherlands)

    Wenink, A.C.G.; Oppenheimer-Dekker, A.; Moulaert, A.J.

    1979-01-01

    Among 79 autopsy specimens of hearts with an isolated ventricular septal defect, there were 29 cases of muscular defect. Among 60 hearts with complete transposition of the great arteries and a ventricular septal defect, there were 13 cases with a muscular defect. All muscular defects could be classi

  2. 经胸房缺堵闭术对房室瓣反流的影响及术后并发症分析%Effects of transthoracic atrial septal defect occlusion on atrioventricular valve regurgitation and analysis of postoperative complications

    Institute of Scientific and Technical Information of China (English)

    张家庆; 闫玉生; 陈坤棠; 莒瑞红

    2012-01-01

    目的 探讨房室瓣反流程度与经胸房缺堵闭术术后并发症的关系.方法 回顾分析2002年1月~2011年3月我科经胸微创房缺堵闭术患者的临床资料,其中资料完全者43例,40例在食道超声、2例在经胸超声辅助下行房缺堵闭术.术后心脏超声随访,观察心脏各指标的变化,房室瓣反流程度及术后并发症等.结果 41例成功,1例术中改为右侧开胸小切口体外循环下房缺修补术,1例术中并发心跳骤停;1例术后并发肾衰,12例术后即时有少量残余漏,1个月后超声复查消失,手术成功率95.3%.术后超声随访显示:右室、右房直径较前缩小;左室直径较前增大;室间隔厚度、二尖瓣血流速度、主动脉瓣血流速度无明显改变;肺动脉瓣血流速度明显降低;二尖瓣反流程度在封堵术后加重,而三尖瓣的反流程度无明显改变;无瓣膜反流或合并二尖瓣轻度反流及三尖瓣中度以下反流的患者,术后未见严重并发症;合并二尖瓣中重度反流或三尖瓣重度反流的患者,封堵后可见严重的并发症,残余漏的发生几率也较大.结论 在无瓣膜反流或合并二尖瓣轻度反流或三尖瓣中度以下反流的患者中,经胸房缺堵闭术安全有效;而在二尖瓣中重度反流或三尖瓣重度反流的患者中,术后并发症发生率高,尤其是严重罕见并发症.%Objective To discuss the correlation between the regurgitation degree of atrioventricular valve and the occurrence of complications after transthoracic atrial septal defect occlusion. Methods A retrospective analysis was made in the clinical data of patients receiving the minimally invasive transthoracic atrial septal defect occlusion in our department from January 2002 to March 2011. Forty three patients had complete clinical data. Forty ones and 2 ones received the transthoracic atrial septal defect occlusion respectivelywith the assistance of transesophageal ultrasound and

  3. Ventricular Septal Defect from Takotsubo Syndrome

    Science.gov (United States)

    Caplow, Julie; Quatromoni, Neha

    2016-01-01

    Takotsubo Syndrome is a transient condition characterized by left ventricular systolic dysfunction with apical akinesis/dyskinesis and ballooning. Although the prognosis with medical management is excellent in most cases, rare cases of serious complications can occur. We present here a case of a 71-year-old woman presenting with acute decompensated heart failure with initial findings consistent with a myocardial infarction, who was found instead to have an acute ventricular septal defect as a complication of Takotsubo Syndrome. PMID:27563471

  4. Ventricular Septal Defect from Takotsubo Syndrome

    Directory of Open Access Journals (Sweden)

    Daniel Y. Lu

    2016-01-01

    Full Text Available Takotsubo Syndrome is a transient condition characterized by left ventricular systolic dysfunction with apical akinesis/dyskinesis and ballooning. Although the prognosis with medical management is excellent in most cases, rare cases of serious complications can occur. We present here a case of a 71-year-old woman presenting with acute decompensated heart failure with initial findings consistent with a myocardial infarction, who was found instead to have an acute ventricular septal defect as a complication of Takotsubo Syndrome.

  5. Resultados da correção simplificada com enxerto único no defeito septal atrioventricular completo

    Directory of Open Access Journals (Sweden)

    Ana Paula Tagliari

    2013-03-01

    Full Text Available FUNDAMENTO: Desde que Wilcox, em 1997, descreveu uma forma simplificada de correção do Defeito Septal Atrioventricular (DSAV com enxerto único, diversos estudos têm sido realizados comparando-a à técnica com duplo enxerto. OBJETIVO: Relatar os resultados em médio e longo prazos da correção de DSAV completo pela técnica simplificada de enxerto único. MÉTODOS: Estudo retrospectivo de 16 casos consecutivos arrolados entre janeiro de 2001 e dezembro de 2011. A idade média foi 18,31 ± 34,19 meses (2 meses - 11 anos e o peso 7,80 ± 6,12 Kg (3,77 - 25,0 Kg; 6 pacientes eram do sexo masculino e 14 eram portadores de Síndrome de Down. O tempo de seguimento médio foi de 54,97 ± 47,79 meses. RESULTADOS: O tempo médio de circulação extracorpórea foi 74,63 ± 18,48 min (49 - 112 min e o de pinçamento aórtico, de 46,44 ± 11,89 min (34 - 67 min. Foram observados dois óbitos hospitalares (12,5%, ambos por causa cardiovascular. Três pacientes foram reoperados por regurgitação da valva atrioventricular (VA esquerda e dois apresentaram bloqueio atrioventricular (BAV completo com necessidade de implante de marca-passo definitivo. Não houve nenhum caso de obstrução da via de saída do ventrículo esquerdo. Os 14 pacientes sobreviventes permanecem assintomáticos, 10 deles com insuficiência da valva VA esquerda leve (71,42%. CONCLUSÃO: A técnica simplificada com enxerto único para correção de DSAV completo mostrou-se factível, associada à correção adequada dos defeitos e à favorável evolução clínica e ecocardiográfica nos 57,97 meses de seguimento médio avaliados.

  6. Electrophysiologic abnormalities of children with ostium secundum atrial septal defect.

    Science.gov (United States)

    Ruschhaupt, D G; Khoury, L; Thilenius, O G; Replogle, R L; Arcilla, R A

    1984-06-01

    Sinus node (SN) and atrioventricular node (AVN) function were evaluated in 49 patients with secundum type atrial septal defect (ASD). Automaticity and conduction system function were assessed by intracardiac recording of the AH and HV intervals at rest, corrected SN recovery time, sinoatrial conduction time, AVN refractory period and the ability of the AVN to conduct rapidly paced atrial beats to the ventricles. Electrophysiologic abnormalities were found in 41% of the 34 patients who were studied before surgery. However, no preoperative abnormalities were present in children younger than 2.5 years. If only children older than 2.5 years were analyzed, the incidence of conduction abnormalities was similar for the patients studied before operation (62%) and those studied after operation (71%). The size and ejection fractions of the right and left ventricles, the magnitude of shunt flow and the size of the ASD did not differ between the patients with and those without electrophysiologic abnormalities. AVN dysfunction was present in 40% of the patients who were studied after surgical repair. While this frequency was more than twice the preoperative incidence of AVN dysfunction, it was not statistically significant. The data suggest that patient age is the major factor that influences the presence of conduction system dysfunction in patients with ASD.

  7. Postoperative follow-up of patients with complete atrioventricular septal defect complicated with pulmonary hypertension%完全性房室间隔缺损合并肺动脉高压术后75例预后分析

    Institute of Scientific and Technical Information of China (English)

    肖婷婷; 李奋; 沈捷; 黄美蓉

    2011-01-01

    objective To observe the operative efficacy in patients with complete atrioventricular septal defect compliated with pulmonary hypertension. Methods From January 2003 to March 2007, CAVSD patients with pulmonary hypertension who underwent operative closure were included in this study. Color Doppler with apical four-chamber view was used to evaluate the degree of pulmonary hypertension before surgery and 2 days, 1 month, 6 month and 1 year after the surgery. Cardiac catheterization was performed to evaluate pulmonary artery pressure and pulmonary arteriolar resistance (PAR) before surgery in patients whose age were over 6 months. The time of staying at ICU, ventilation time after surgery and the occurrence of pulmonary artery hypertension crisis were recorded. Results A total of 75 CAVSD patients who underwent operative closure were enrolled in this study. The mean staying time at ICU was (4.9 ± 2.1 )days, and the mean ventilation time was (2.1 ± 0.9) days; 12 patients developed pulmonary artery hypertension crisis after surgery. Patients with PAR > 8 wood unit were older, staying time at ICU and ventilation time were longer compared with patients with PAR < 8 wood unit (all P < 0.05). The incidence of pulmonary artery hypertension crisis after surgery was also significantly higher in patients with PAR > 8 wood unit compared with patients with PAR < 8 wood unit(P < 0.05). Five patients died. Four out of 5 hospital-dead patients had severe hypoplasia of the atrioventricular valve. Pulmonary artery pressure of 39 patients (52%) returned to normal two days after surgery, 51 patients (68%) returned to normal one month after surgery, 64 patients (85%) returned to normal 6 months after surgery,and 68 patients (91%) returned to normal one year after surgery; 7 patients have pulmonary hypertension one year after surgery. Conclusion Our results suggest that early surgical repair for CAVC is safe and beneficial.Preoperative PAR > 8 wood unit is

  8. High-septal pacing reduces ventricular electrical remodeling and proarrhythmia in chronic atrioventricular block dogs

    DEFF Research Database (Denmark)

    Winckels, Stephan K G; Thomsen, Morten Bækgaard; Oosterhoff, Peter

    2007-01-01

    This study was designed to analyze the relevance of ventricular activation patterns for ventricular electrical remodeling after atrioventricular (AV) block in dogs.......This study was designed to analyze the relevance of ventricular activation patterns for ventricular electrical remodeling after atrioventricular (AV) block in dogs....

  9. Familial Atrial Septal Defect and Sudden Cardiac Death

    DEFF Research Database (Denmark)

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

    2016-01-01

    OBJECTIVE: Atrial septal defect (ASD) is the second most common congenital heart defect (CHD) and is observed in families as an autosomal dominant trait as well as in nonfamilial CHD. Mutations in the NKX2-5 gene, located on chromosome 5, are associated with ASD, often combined with conduction...

  10. 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

  11. Echocardiographic evaluation of ventricular septal defect haemodynamics

    Directory of Open Access Journals (Sweden)

    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

  12. Atrial Septal Defect in a Very Old Woman

    Science.gov (United States)

    Pinho, Elika; Gomes, Andre Amaral; Silva, Maria Joao; Torres, Tiago Pinheiro; Coelho, Andreia; Almeida, Pedro Bernardo; Lourenco, Patricia; Bettencourt, Paulo

    2013-01-01

    Atrial Septal Defect (ASD) is one of the most frequently congenital heart diseases in adults and it is often asymptomatic until adulthood. We report a case of a 90-year-old woman admitted to hospital with dyspnea and orthopnea insidiously progressing over the preceding 5 years and becoming severe with dyspnea on minimal activities, orthopnea and paroxysmal nocturnal dyspnea, in the last 2 weeks. The transthoracic echocardiogram revealed an atrial septal defect ostium secundum type, with left-to-right shunt, moderate to severe tricuspid insufficiency, severe pulmonary hypertension (72 mmHg) and preserved biventricular function. With diuretic therapy optimization the patient showed symptomatic improvement. This present case represents and unusual and very late presentation of an atrial septal defect ostium secundum type, which is usually diagnosed at the mild adult age. Our patient lived symptom-free for over 80 years.

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

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    Kshitij Sheth

    2015-01-01

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

  14. Initial experience in Brazil with the Helex septal occluder for percutaneous occlusion of atrial septal defects

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    Pedra Carlos A.C.

    2003-01-01

    Full Text Available OBJECTIVE: To evaluate the initial clinical experience with the Helex septal occluder for percutaneous closure of atrial septal defects. METHODS: Ten patients underwent the procedure, 7 patients with ostium secundum atrial septal defects (ASD with hemodynamic repercussions and 3 patients with pervious foramen ovale (PFO and a history of stroke. Mean age was 33.8 years and mean weight was 55.4 kg. Mean diameter by transesophageal echocardiography and mean stretched ASD diameter were 11.33 ± 3.3mm, and 15.2 ± 3.8mm, respectively. The Qp/Qs ratio was 1.9 ± 0.3 in patients with ASD. RESULTS: Eleven occluders were placed because a patient with 2 holes needed 2 devices. It was necessary to retrieve and replace 4 devices in 3 patients. We observed immediate residual shunt (< 2mm in 4 patients with ASD, and in those with patent foramen ovale total occlusion of the defect occurred. No complications were noted, and all patients were discharged on the following day. After 1 month, 2 patients with ASD experienced trivial residual shunts (1mm. In 1 patient, we observed mild prolapse in the proximal disk in the right atrium, without consequences. CONCLUSION: The Helex septal occluder was safe and effective for occluding small to moderate atrial septal defects. Because the implantation technique is demanding, it requires specific training of the operator. Even so, small technical failures may occur in the beginning of the learning curve, but they do not involve patient safety.

  15. 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 ech

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

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    Sudhir S Shetkar

    2015-01-01

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

  17. Pulmonary edema following transcatheter closure of atrial septal defect

    Directory of Open Access Journals (Sweden)

    Singhi Anil

    2010-01-01

    Full Text Available Pulmonary edema after device closure of atrial septal defect (ASD is a rare complication. We present illustrative images of a case of pulmonary edema after device closure of ASD in a 53 year old adult. Older patients undergoing ASD closure can benefit from their left atrial and left ventricular end diastolic pressures measurement before and after temporary balloon occlusion of ASD.

  18. Echocardiographic evaluation of patent foramen ovale and atrial septal defect.

    Science.gov (United States)

    Hari, Pawan; Pai, Ramdas G; Varadarajan, Padmini

    2015-01-01

    Patent foramen ovale (PFO) is a common variant present in up to 25% of the population. Atrial septal defect (ASD) is a direct communication between the 2 atrial chambers, of which the ostium secundum variety is the most common. This manuscript is an in depth review of the complex atrial septation, the diagnosis of PFO and ASD and its clinical and therapeutic implications.

  19. Three-dimensional echocardiographic assessment of atrial septal defects

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    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.

  20. Transcatheter closure of secundum atrial septal defect using an Amplatzer septal occluder

    Institute of Scientific and Technical Information of China (English)

    孔祥清; 曹克将; 杨荣; 许迪; 盛燕辉; 黄峻; 马文珠

    2002-01-01

    Objective To evaluate the safety and efficacy of the Amplatzer septal occluder for transcatheter closure in patients with secundum atrial septal defect (ASD Ⅱ). Methods Patients with clinically confirmed ASD Ⅱ were recommended for transcatheter closure of ASD Ⅱ. Results 30 ASD Ⅱ patients (20 females) underwent transcatheter closure at a median age of 18.4 years (5-55 years). Both the stretched diameters of ASDs and the sizes of the devices were from 18 to 34?mm (25±7?mm). The successful placement rate was 100%. The rest shunt documented by color Doppler, was immediately after implantation in 40% of patients, in 9.9% after 24 hours, and in 3.3% trace at 3 months. No serious complications were observed. There was improvement in symptoms and in cardiac size. Septal motion abnormalities normalized in all patients after 3 months follow-up. Conclusion The Amplatzer septal occluder is a safe and effective device for transcatheter closure of ASD Ⅱ. Long-term follow-up is still required before widespread clinical use can be recommended.

  1. Complications Related to Transcatheter Occlusion of Atrial Septal Defect with Amplatzer Septal Occluder in Children

    Institute of Scientific and Technical Information of China (English)

    Wang Shushui; Li Yufen; Zhang Zhiwei; Wang Huishen; Qian Mingyang

    2004-01-01

    Objectives To investigate the causes, theraputic and preventive methods of complications associated with transcatheter occlusion of atrial septal defect (ASD) using the Amplatzer septal occluder (ASO) in children. Methods 289 cases underwent transcatheter closure of ASD with ASO. Complications occurred in 9 cases. The complications in procedure included systemic circulatory systemic air embolism in 2, pulmonary air embolism in 1, pericardial tamponade in 1, ASO malposition requiring emergency surgical removal in 1, transient atrial extrasystoles in 1 and sizing balloon rupture in 1 case. 2 cases with postoperative complications were found in the follow-up studies. These included perforation of mitral valve and ASO partially dislodged. In this study, 5children with intraoperative complication received emergency therapy including surgical intervention, and others needed only follow-up, as the complications were transient or asymptomatic. Results There were no children death in this study. The 5 cases who received treatment were completely healing, and the others with intra - procedure complications were also had no sequela existed. Cases with mitral valve and ASO partially dislodged were still in follow-up studies, as the 2 patients having no symptoms. Conclutions Air embolism were occurred easily in atrial septal defect cases who received ASO therapy. Complications mainly caused by inappropriate operative procedure and some complications needed emergency treatments. Follow-up studies were important to cases with transcatheter occlusion therapy.

  2. 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.

  3. Pulmonary edema following transcatheter closure of atrial septal defect

    Science.gov (United States)

    Chigurupati, Keerthi; Reshmi, Liza Jose; Gadhinglajkar, Shrinivas; Venkateshwaran, S.; Sreedhar, Rupa

    2015-01-01

    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. PMID:26139760

  4. Transcatheter Closure of Atrial Septal Defect with Amplatzer Septal Occluder in Adults: Immediate, Short, and Intermediate-Term Results

    Directory of Open Access Journals (Sweden)

    Majid Dehghani

    2011-05-01

    Full Text Available Background: The transcatheter closure of the atrial septal defect (ASD has become an alternative technique to surgical procedures. The aim of this study was to assess the immediate, short, and intermediate-term results of the transcatheter closure of the secundum ASD with the Amplatzer Septal Occluder (ASO in adult Iranian patients.Methods: Between December 2004 and July 2008, the transcatheter closure of the ASD using the ASO was attempted in 58consecutive, adult patients. The mean age of the patients was 37.1 ± 12.7 years (range = 19 - 75 years.All the procedures were performed under local anesthesia with transthoracic or transesophageal echocardiography and fluoroscopic guidance. The stretched diameter of the ASD was determined with a balloon sizing catheter, and device selection was based on and matched to the stretched diameter of the septal defect.Transthoracic echocardiography was performed immediately after the release of the device and before discharge.Further follow-up at one month, six months, and yearly thereafter included physical examination, electrocardiography, andtransthoracic echocardiography.Results: The mean ASD diameter, as measured by esophageal echocardiography, was 24.8 ± 5.4 mm (range = 13 - 34 mm.The mean stretched diameter, as measured by the balloon catheter, was 27.1 ± 6.4 mm (range = 12.5 - 39 mm. Deploymentof the ASO was successful in 52 (89.6% patients and failed in 6 (10.4%. Four patients experienced severe complications,1 had tamponade requiring drainage, 2 had device embolization to the left atrium and right ventricular outflow tract, and 1 had late wire fracture (surgical removal and repair of the ASD. The position of two large devices (34 mm and 36 mm was considered unsuitable and unstable after implantation and resulted in the removal of these devices.Minor complications included transient complete atrioventricular block in 1 patient, paroxysmal supra tachycardia in 3 patients, atrial flutter in 1 patient

  5. A case of hybrid closure of a muscular ventricular septal defect: anatomical complexity and surgical management.

    Science.gov (United States)

    Karimi, Mohsen; Hulsebus, Elise; Murdison, Kenneth; Wiles, Henry

    2012-06-01

    Complex muscular ventricular septal defect poses difficult surgical management and is associated with high morbidity and mortality despite advancements in surgical therapy. Device closure of muscular ventricular septal defect has been encouraging and has been used in hybrid approach at a few centres. However, device closure has some limitations in patients with complex muscular ventricular septal defect. We report a case of perventricular device closure of a complex muscular ventricular septal defect in a beating heart with entrapped right ventricular disc and its surgical management.

  6. [Left ventricular synchrony with septum stimulation vs. septal ventricular outflow tract in complete atrioventricular block].

    Science.gov (United States)

    Rodríguez-Serrano, Gustavo; Lara-Vaca, Susano; Pereyra-Nobara, Texar; Bernal-Ruiz, Enrique

    2016-01-01

    Introducción: el objetivo del presente estudio fue determinar si existe diferencia en la función sistólica y la sincronía del ventrículo izquierdo con estimulación del septum medio o tracto de salida del ventrículo derecho en pacientes con bloqueo auriculoventricular portadores de marcapaso. Métodos: estudio observacional, analítico, transversal. Se seleccionaron todos los pacientes mayores de 18 años portadores de marcapaso con diagnóstico de bloqueo auriculoventricular. Se analizaron dos grupos acorde al sitio de colocación del electrodo de estimulación en región septal media o tracto de salida del ventrículo derecho. Se determinó la fracción de expulsión y sincronía del ventrículo izquierdo. Resultados: se incluyeron 54 pacientes por cada grupo, siendo las características de ambos similares, excepto el tiempo de colocación del electrodo de estimulación ventricular (p = < 0.001). No hubo diferencia significativa en la fracción de expulsión o sincronía del ventrículo izquierdo. Conclusiones: no se encontró diferencia en la fracción de expulsión o sincronía del ventrículo izquierdo independientemente del sitio de colocación del electrodo de estimulación.

  7. [Non-puncture treatment of sinusitis in nasal septal defects].

    Science.gov (United States)

    Shilenkova, V V; Markov, G I; Shilenkov, A A

    1994-01-01

    The [symbol: see text] sinus-catheter was used to treat purulent sinusitis with septal perforation or defective septum of the sinuses. The procedure described implies simultaneous introduction of two sinus-catheters the functioning channels of which unite into one output by means of T-joint. The method has been successfully tried in the treatment of exudative and aggravated chronic purulent hemisinusitis (6 cases). The authors hold that communication between nasal sinuses is not a contraindication for sinus-catheter application. This fact widely extends potentialities of its use.

  8. Risk factors for ventricular septal defect in Finland.

    Science.gov (United States)

    Tikkanen, J; Heinonen, O P

    1991-03-01

    The possible effect of genetic and environmental factors during pregnancy on the occurrence of ventricular septal defect (VSD) in the offspring was studied in 150 cases and 756 controls. The cases represented all verified VSDs in Finland during 1982-1983. The controls were randomly selected from all babies born during the same period. Case and control mothers were interviewed by midwives approximately three months after delivery using a structured questionnaire. Congenital heart disease was more prevalent among parents of cases than those of controls. Maternal alcohol consumption during the first trimester of pregnancy was more common among the mothers of VSD infants (47.0%) than among those of controls (38.0%, P less than 0.05). Exposure to organic solvents at work showed in logistic regression analysis an adjusted relative odds ratio of 1.8 (95% confidence interval 1.0-3.4). The risk of VSD was not associated with any of the maternal habits monitored, e.g. smoking, or coffee, tea, cola, acetosalicylic acid or diazepam consumption. Whether the mother was employed during the first trimester of pregnancy, and her exposure to anesthetic gases, disinfectants, pecticides, wood preservatives or video display terminals were not factors associated with the risk of ventricular septal defect.

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

    Science.gov (United States)

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

    2012-01-01

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

  10. 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.

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

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    Yuksel Kaya

    2012-01-01

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

  12. Recurrence of atrial septal defect in three generations

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    Celso Ferreira

    1999-08-01

    Full Text Available Beginning with a patient presenting with an atrial septal defect (ASD of the secundum type, the genealogy was identified in four affected individuals who belonged to three successive generations of the same family. The defects were visually confirmed in all individuals and were found to be anatomically similar. No other congenital malformations were present in these individuals. The genealogy was identified in 1972, when ASD recurred in two generations, and it was concluded that the mechanism of transmission was autosomal recessive. The fifth individual, identified 21 years later, and having an anomaly identical to that of the others, was the child of a couple who had no consaguinity and whose mother was a member of the previously studied genealogy. Considering the absence of phenotype in the parents and the rarity of the ASD gene in the general population, the occurrence of the uniparental disomy for this family nucleus, and the same autosomal recessive mechanism of transmission by this affected individual is possible. This study reports the familial occurrence of ASD by genetic mechanisms of transmission, emphasizing the necessity for genetic-clinical studies in members of the familial nucleus in order to detect new carriers, who usually are asymptomatic, thereby allowing for early and adequate treatment of individuals who may be affected.

  13. MORTAL PULPOTOMY ON CHILDREN WITH VENTRICULAR SEPTAL DEFECT

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    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.

  14. Staged repair of pentalogy of Cantrell with ectopia cordis and ventricular septal defect.

    Science.gov (United States)

    Sakasai, Yoshie; Thang, Bui Quoc; Kanemoto, Shinya; Takahashi-Igari, Miho; Togashi, Shinji; Kato, Hideyuki; Hiramatsu, Yuji

    2012-05-01

    Pentalogy of Cantrell is a rare congenital anomaly characterized by a combination of severe defects in the middle of the chest and abdomen including intracardiac defects. Survival rate after cardiac surgery is extremely low. We present a successful staged complete repair of an omphalocele, a ventricular septal defect and a sternal defect in a case of pentalogy of Cantrell.

  15. Ventricular Septal Defect Spontaneous Close Induced by Transcatheter: A Case Report

    Institute of Scientific and Technical Information of China (English)

    Qilian Xie; Jun Wang; Lei Gao; Zhen Wang; Milin Zhang; Kunshen Liu

    2007-01-01

    Congenital ventricular septal defect (VSD) spontaneous close induced by transcatheter treatment is rare and has not yet been reported.We report on one case of VSD spontaneous close induced by transcatheter treatment in a 10 years old girl.

  16. 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

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    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.

  17. 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

    2012-04-01

    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.

  18. Current perspectives in percutaneous atrial septal defect closure devices

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    Bissessor N

    2015-07-01

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

  19. Repetitive Delay in Diagnosis of Ventricular Septal Defect

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    Zahra Nikyar

    2011-12-01

    Full Text Available Objective: Although ventricular septal defect (VSD is the most common congenital heart disease, it is usually diagnosed late. The presentation of the disease is variable; sometimes it is so quiet and silent that might even improve and heal spontaneously, and in some certain cases if the appropriate, on time and early treatment is not done, this would lead to irreparable complications and mortality even in the early life period. This study reviews the diagnostic process, treatment and follow-up of the patients. It is hoped that the results of the present study be used to improve the patients' condition.Methods: This was a cross-sectional study done on 145 patients with VSD during 54 months in Isfahan. The disease was identified through color Doppler echocardiogram, cardiac catheterization and angiography if necessary. The required data were collected at the time of definite diagnosis.Findings: Mean age at initial and definite diagnosis of the disease was 17 months and 44 months, respectively. Heart murmur led to initial diagnosis in 85% of the cases. In 27.5% VSD was associated with other cardiac anomalies. Pulmonary artery hypertension existed in 16.5% of the cases. Fifty nine surgeries were performed on 40 patients.Conclusion: In routine physical examination of the infants, the probability of heart disease should be considered; conducting echocardiogram in suspected cases would lead to early diagnosis and eventually timely treatment. Appropriate follow-up of the patients will provide optimal care and treatment at proper time.

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

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    Er-Ping Xi

    2012-11-01

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

  1. Angiographic studies of atrioventricular discordance.

    Science.gov (United States)

    Attie, F; Soni, J; Ovseyevitz, J; Muñoz-Castellanos, L; Testelli, M R; Buendia, A

    1980-08-01

    The results and value of conventional and axial angiocardiography were studied in 26 cases of atrioventricular discordance (AVD). The angiographic anatomy of this malformation was analyzed to determine the precise characteristics of atrioventricular and ventriculoarterial connections. The left and right ventricles in atrial situs solitus and levocardia show angiographic features that differ from those seen in atrial situs solitus and dextrocardia. The same differences are found in atrial situs inversus. Regardless of the type of ventriculoarterial connections, the ventricles maintain the same angiographic characteristics. The ventricular septum in AVD in situs solitus and levocardia and situs inversus and dextrocardia has a peculiar orientation. The use of axial angiocardiography facilitates detection and localization of the ventricular septal defect. In cases of malposition of the heart, the conventional frontal view allows visualization of the ventricular septum in all of its length. The axial projection is useful in diagnosing left ventricular outflow tract obstruction.

  2. Percutaneous Interventional Treatment of Atrial Septal Defect Secundum in Macedonia

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    Ivan Milev

    2014-03-01

    Conclusion: In conclusion, the implantation of a septal occluder was found to be a safe procedure that resulted in improved hemodynamic parameters that result from right ventricular volume overload with favorable short- and mid-term results.

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

    Science.gov (United States)

    Anuwatworn, Amornpol; Bendaly, Edgard; Prescott-Focht, Julia A.; Clark, Richard; Jonsson, Orvar

    2016-01-01

    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. PMID:27974976

  4. 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.

  5. Atrioventricular and interventricular groove and septal extension of right sinus of valsalva aneurysm: a rare cause of complete heart block.

    Science.gov (United States)

    Khan, Javaid Arif; Hussain, Mushtaq; Rizvi, Nadeem H; Fehmi, Nadeem; Hussain, Akhtar; Sial, Jawaid A

    2013-10-01

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

  6. Lessons learnt from a series of hemodynamic and interventional complications during pulmonary valvotomy and device closure of ventricular septal defect

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    Anil Kumar Singhi

    2015-01-01

    Full Text Available In an adolescent girl with coexistent valvar pulmonary stenosis (PS and muscular ventricular septal defect (VSD causing right to left shunt and severe cyanosis, a series of complications were encountered during transcatheter intervention. After balloon pulmonary valvotomy (BPV and device closure of the VSD, dynamic infundibular hypercontractility elevated the right ventricular (RV systolic pressures leading to embolization of the device into the left ventricle. During retrieval of the device from the left ventricular outflow tract (LVOT, there was injury to the atrioventricular nodal tissue resulting in transient complete heart block. Even though the device was successfully retrieved out from the right femoral arterial access, there was a retroperitoneal hematoma causing severe anemia. A combination of dynamic infundibular obstruction, intravascular volume depletion caused by hemorrhage, low oxygen carrying capacity due to anemia, and inotropes resulted in a life-threatening hypercyanotic spell. Once this combination of suicidal hypercontractile right ventricle, anemia, and dehydration was recognized; management of the pathophysiology resulted in recovery of the patient. On a subsequent day, device closure of the VSD was complicated by unstable device position, but was finally achieved by an innovative device stabilization technique. We propose to explain the basis of hemodynamic and procedural complications, their recognition, and management. During preparatory stage before interventions in patients with multiple defects, which are linked by strong hemodynamic interdependence, meticulous planning should be done and multiple untoward events should be foreseen. While a few complications were completely unanticipated, some others could have been predicted.

  7. Morgagni Hernia with Partial A-V Canal Defect; A Rare Condition

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    Kunal

    2012-06-01

    Full Text Available Morgagni hernia is a rare diaphragmatic hernia usually due to congenital defects in the diaphragm. It is rarely associated with cardiac anomalies, most commonly atrial (ostium secundum or ventricular septal defects. We report a rare case of Morgagni hernia occurring in association with partial atrio-ventricular septal defect (ostium primum, and its successful surgical correction.

  8. Infective endocarditis affecting both systemic and pulmonary circulations predisposed by a ventricular septal defect.

    Science.gov (United States)

    Ando, M; Sakai, A; Nakamura, K; Iwata, Y; Sanae, T

    2000-07-01

    A 39-year-old woman was admitted to our hospital presenting persisting fever. An echocardiographic examination showed severe aortic and mitral valve regurgitation with moderate tricuspid regurgitation. Small left-to-right shunt through the ventricular septal defect was identified. Vegetation was also detected on the tricuspid, mitral, and aortic valves. At one month after admission, the patient showed sudden onset of headache and abdominal pain. A computed tomographic scan demonstrated cerebral and splenic infarction. A pulmonary perfusion scintigram demonstrated perfusion defects in left-S1 and right-S6 regions. At 4 months after admission, as operation was performed. The aortic valve was replaced with a #23 mm CarboMedics prosthesis and the mitral valve with a #29 mm Carbo Medics prosthesis. Tricuspid valve plasty was performed, with closure of He laceration and perforation of the anterior leaflet combined with a commissuroplasty, according to Kay's method. Ventricular septal defect was closed with a bovine pericardial patch. She was discharged at 19 days after the operation, and is leading a good life. Pervasion of the organism seemed to be initiated from the mitral valve which was conveyed by the blood stream to the aortic valve, and to the tricuspid valve through the ventricula septal defect. Left heart evaluation may be important in cases with infective endocarditis and ventricula septal defect.

  9. Is there any association between childhood cardiac septal defects and ROCK2 gene polymorphism?

    Science.gov (United States)

    Aksoy, M; Uygun, H; Baspinar, O; Demiryurek, S; Oztuzcu, S; Cengiz, B; Irdem, A; Araz, N C

    2014-03-17

    Rho/Rho-kinase pathway plays a critical role in the regulation of cellular functions such as proliferation and migration. One of the possible theories of the development of ventricular septal defects is cell migration disorder. The aim of this study was to analyze the genotype distributions and allele frequencies for the ROCK2 gene Thr431Asn polymorphisms in the development of cardiac septal defects in a Turkish population. In this case-control study, 300 patients with cardiac defects (150 patients with ventricular and 150 patients with atrial septal defects) and control group (150 healthy control subjects) were investigated. A single-nucleotide polymorphism in ROCK2 gene Thr431Asn was analyzed by real-time PCR using a Light-Cycler. Neither genotype distributions nor the allele frequencies for the Thr431Asn polymorphism showed a significant difference between the groups. These results suggest that there is no association of the ROCK2 gene Thr431Asn polymorphism with the development of cardiac septal defects in pediatric patients.

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

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    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.

  11. 80. Atrioventricular septal defect and tetralogy of Fallot: A 16-year experience

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    S. Al-Ahdal

    2015-10-01

    Conclusion: Shunt procedure is a reasonable initial option for many of patients with AVSD/TOF but primary repair can be performed in selected patients with low operative mortality and reasonable morbidity.

  12. Repair of a ventricular septal defect in a patient with left lung agenesis.

    Science.gov (United States)

    Guo, Hong-Wei; Pan, Shi-Wei; Song, Yun-Hu; Hu, Sheng-Shou

    2011-09-01

    Congenital heart disease combined with lung agenesis is extremely rare. We report a case of a 5-year-old female with a ventricular septal defect (VSD) and left lung agenesis with severe pulmonary hypertension who underwent successful closure of the VSD. 

  13. 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...

  14. Recurrence of cerebrovascular events in young adults with a secundum atrial septal defect

    NARCIS (Netherlands)

    Winkens, Bjorn; Dimopoulos, Konstantinos; Fernandes, Susan M.; Gatzoulis, Michael A.; Landzberg, Michael J.; Mulder, Barbara J. M.

    2010-01-01

    Background: The recurrence rate for cerebrovascular ischemic events in patients after a first TIA or CVA with an atrial septal defect type 2 (ASD2) remains unknown. At present, there are no guidelines with respect to appropriate treatment. The aim of this study was to determine incidence rates of re

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

    NARCIS (Netherlands)

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

    2015-01-01

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

  16. Perventricular device closure of residual muscular ventricular septal defects after repair of complex congenital heart defects in pediatric patients.

    Science.gov (United States)

    Zhu, Da; Tao, Kaiyu; An, Qi; Luo, Shuhua; Gan, Changping; Lin, Ke

    2013-01-01

    Residual muscular ventricular septal defects are surgical challenges, especially after the repair of complex congenital heart defects. We investigated perventricular device closure as a salvage technique in pediatric patients who had postoperative residual muscular ventricular septal defects. From February 2009 through June 2011, 14 pediatric patients at our hospital had residual muscular ventricular septal defects after undergoing surgical repair of complex congenital heart defects. Ten patients met our criteria for perventricular device closure of the residual defects: significant left-to-right shunting (Qp/Qs >1.5) or substantial hemodynamic instability (a defect ≥2 mm in size). The patients' mean age was 20.4 ± 13.5 months, and their mean body weight was 10 ± 3.1 kg. The median diameter of the residual defects was 4.2 mm (range, 2.5-5.1 mm). We deployed a total of 11 SQFDQ-II Muscular VSD Occluders (Shanghai Shape Memory Alloy Co., Ltd.; Shanghai, China) in the 10 patients, in accord with conventional techniques of perventricular device closure. The mean procedural duration was 31.1 ±9.1 min. We recorded the closure and complication rates perioperatively and during a 12-month follow-up period. Complete closure was achieved in 8 patients; 2 patients had persistent trivial residual shunts. No deaths, conduction block, device embolism, or other complications occurred throughout the study period. We conclude that perventricular device closure is a safe, effective salvage treatment for postoperative residual muscular ventricular septal defects in pediatric patients. Long-term studies with larger cohorts might further confirm this method's feasibility.

  17. Association of TBX5 gene polymorphism with ventricular septal defect in the Chinese Han population

    Institute of Scientific and Technical Information of China (English)

    LIU Cai-xia; SHEN A-dong; LI Xiao-feng; JIAO Wei-wei; BAI Song; YUAN Feng; GUAN Xiao-lei; ZHANG Xin-gen; ZHANG Gui-rong; LI Zhong-zhi

    2009-01-01

    Background Congenital heart disease is a diverse group of diseases determined by genetic and environmental factors. Considerable research has been done on genes associated with development of the heart. A recent focus is the role of transcription factor TBX5 in the development of atria, left ventricle and conduction system. As part of a larger study, high density, single nucleotide polymorphism (SNP) scanning was used to explore the relationship between TBX5 gene polymorphism and susceptibility to ventricular septal defect not associated with forelimb malformation in the Chinese Han population. Methods One hundred and ninety two paediatric patients with congenital ventricular septal defect and 192 matched healthy control subjects were studied. The haplotype reconstructions were calculated by PHASE2.0 software. Haploview software was used to 15erform linkage disequilibrium assessment and defining of haplotype blocks. The algorithm used for defining of blocks was the confidence interval method. Results The TBX5 gene region can be divided into 3 haplotype blocks of 27, 15 and 2 SNPs. Strong linkage disequilibrium exists within each block. SNP rs11067075 within the TBX5 gene had significant correlation with ventricular septal defect (P=0.0037) by single marker association analysis. In addition, a 20 kb haplotype composed of 27 SNPs correlated with ventricular septal defect (P=0.05, multiple loci regression analyses based on reconstructed haplotype blocks). Conclusions TBX5 is associated with the occurrence of ventricular septal defect and may be a predisposing gene to congenital heart disease in Hart Chinese. This finding has set a direction for further genetic and functional studies.

  18. Transcatheter closure of atrial septal defect protects from pulmonary edema: septal occluder device gradually reduces LR shunt.

    Science.gov (United States)

    Murakami, Tsutomu; Nakazawa, Gaku; Horinouchi, Hitomi; Torii, Sho; Ijichi, Takeshi; Ohno, Yohei; Amino, Mari; Shinozaki, Norihiko; Ogata, Nobuhiko; Yoshimachi, Fuminobu; Yoshioka, Koichiro; Ikari, Yuji

    2017-01-01

    A 56-year-old woman was diagnosed as atrial septal defect (ASD) with pulmonary hypertension; pulmonary blood flow/systemic blood flow (Qp/Qs) of 2.3, pulmonary artery pressure (PAP) of 71/23(39) mmHg and diastolic dysfunction of left ventricle. PAP was improved after medical therapy; therefore, transcatheter ASD closure was performed. Seven days later, left-sided heart failure occurred, however, the improvement of Qp/Qs (1.7) and PAP of 51/21(32) was confirmed. Diuretic therapy was introduced which led to further decrease of PAP 40/12(25) and Qp/Qs (1.1). Because of gradual decrease of Qp/Qs, this patient appeared to be protected from acute pulmonary edema.

  19. Effectiveness of balloon valvuloplasty for palliation of mitral stenosis after repair of atrioventricular canal defects.

    Science.gov (United States)

    Robinson, Joshua D; Marx, Gerald R; Del Nido, Pedro J; Lock, James E; McElhinney, Doff B

    2009-06-15

    Closure of a mitral valve (MV) cleft, small left-sided cardiac structures, and ventricular imbalance all may contribute to mitral stenosis (MS) after repair of atrioventricular canal (AVC) defects. MV replacement is the traditional therapy but carries high risk in young children. The utility of balloon mitral valvuloplasty (BMV) in postoperative MS is not established and may offer alternative therapy or palliation. Since 1996, 10 patients with repaired AVC defects have undergone BMV at a median age of 2.5 years (range 8 months to 14 years), a median of 2 years after AVC repair. At catheterization, the median value of mean MS gradients was 16 mm Hg (range 12 to 22) and was reduced by 34% after BMV. Before BMV, there was mild mitral regurgitation in 9 of 10 patients, which increased to severe in 1 patient. All patients were alive at follow-up (median 5.4 years). Repeat BMV was performed in 4 patients, 10 weeks to 18 months after initial BMV. One patient underwent surgical valvuloplasty; 3 underwent MV replacement 2, 3, and 28 months after BMV. In the 6 patients (60%) with a native MV at most recent follow-up (median 3.2 years), the mean Doppler MS gradient was 9 mm Hg, the median weight had doubled, and weight percentile had increased significantly. In conclusion, BMV provides relief of MS in most patients with repaired AVC defects; marked increases in mitral regurgitation are uncommon. Because BMV can incompletely relieve obstruction and increase mitral regurgitation, it will not be definitive in most patients but will usually delay MV replacement to accommodate a larger prosthesis.

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

    Directory of Open Access Journals (Sweden)

    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. 

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

    Directory of Open Access Journals (Sweden)

    Renu Sinha

    2011-01-01

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

  2. Transcatheter closure of atrial septal defect associated with arrhythmogenic right ventricular cardiomyopathy: a case report and literature review.

    Science.gov (United States)

    Wang, Haiyan; Ding, Hongyu; Lei, Lei; Zhang, Xiaohong; Gong, Yuling; Hou, Yinglong

    2015-03-01

    Arrhythmogenic right ventricular cardiomyopathy is characterised by progressive, fibrofatty replacement of myocardium, and ventricular arrhythmias, and its prognosis is usually poor. Arrhythmogenic right ventricular cardiomyopathy associated with atrial septal defect is very rare, and this combination may make the diagnosis, treatment, and prognosis difficult. We present a case of a patient with this association who underwent interventional treatment with a septal defect occluder. Transcatheter closure of atrial septal defect in a patient with arrhythmogenic right ventricular cardiomyopathy is hitherto unreported. During a 3-year follow-up he remained relatively stable. We also review the cases reported in the medical literature describing this uncommon association between arrhythmogenic right ventricular cardiomyopathy and atrial septal defect or patent foramen ovale.

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

    Directory of Open Access Journals (Sweden)

    L. Rozendaal

    2011-01-01

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

  4. EPIDURAL ANESTHESIA FOR CESAREAN SECTION IN ATRIAL SEPTAL DEFECT WITH PULMONARY HYPERTENSION

    Directory of Open Access Journals (Sweden)

    Rashmi

    2015-07-01

    Full Text Available Pregnant patients with atrial septal defect (ASD with pulmonary hypertension , occasionally present for anaesthesia . These patients are considered high risk for anaesthesia due to increased chances of per i operative cardiovascular complications . Anaesthesia requires intensive cardiovascular mo nitoring and maintenance of stable pulmonary and systemic haemodynamics . Here is a case of second gravida for LSCS which was managed under epidural anaesthesia successfully

  5. Association of Duodenal Atresia, Malrotation, and Atrial Septal Defect in a Down-Syndrome Patient

    Directory of Open Access Journals (Sweden)

    R Angotti

    2016-04-01

    Full Text Available Duodenal atresia is the frequent cause of neonatal intestinal obstruction. The association between duodenal atresia, intestinal malrotation, cardiac anomalies and Down syndrome is infrequently reported. We present a prenatally suspected case of duodenal atresia which was associated with malrotation and atrial septal defect in a patient of Down syndrome. Duodenotomy and resection of web was performed in addition to Ladd’s procedure. Postoperative course remained uneventful.

  6. Device closure of post-myocardial infarction ventricular septal defect three weeks after coronary angioplasty

    Science.gov (United States)

    Patnaik, A. N.; Barik, Ramachandra; Kumari, N. Rama; Gulati, A. S.

    2012-01-01

    Percutaneus device closure appears to be safe and effective in patients treated for a residual shunt after initial surgical closure, as well as after two to three weeks of index myocardial infarction. The index case presented with a ventricular septal defect on second of acute myocardial infarction thrombolysed with streptokinase. The general condition of the patient was fairly stable. Cardiac catheterization and coronary angiography showed significant left to right shunt and there was 90 % proximal stenosis of left anterior descending coronary artery. Other coronary arteries were normal. Angioplasty and stenting to the coronary artery lesion was done using drug eluting stent (DES) with very good angiographic result. Patient was discharged after four days in stable condtion. After 3 weeks his ventricular septal defect was closed percutaneusly using cardio -O-fix device with tiny residual shunt. The procedure was uneventful and of brief duration. He was discharged after 5 days of the post procedure in very stable condition with minimal residual shunt. A staged procedure is a better option if the condition of the patient allows strengthening ventricular septal defect border. PMID:22629038

  7. Oclusão percutânea das comunicações interventriculares: experiência inicial Interventricular septal defects percutaneous occlusion: initial experiment

    Directory of Open Access Journals (Sweden)

    Francisco José Araújo Chamié de Queiróz

    2005-09-01

    Full Text Available OBJETIVO: Avaliar a experiência inicial com o fechamento percutâneo das comunicações interventriculares (CIV, em especial, das CIV perimembranosas (CIVPM com a nova prótese de Amplatzer. MÉTODOS: Foram submetidos ao procedimento onze pacientes, seis com CIV perimembranosas (CIVPM e cinco com CIV musculares (CIVM. Dois apresentavam infecções repetidas do trato respiratório e tinham baixo ganho ponderal. Um apresentou história prévia de endocardite infecciosa. Os demais eram assintomáticos e foram selecionados pela ecocardiografia transtorácica (ETT. RESULTADOS: No grupo das CIVM (n=5, um paciente tinha uma CIV apical, dois tinham defeitos médio-septais e dois pacientes apresentavam defeitos múltiplos. Neste grupo, foi utilizada uma prótese de comunicação interatrial (CIA (Amplatzer Septal Occluder® e cinco próteses para CIVM (Amplatzer VSD-MUSC Occluder®, sendo que uma mesma paciente recebeu dois dispositivos. Todos os implantes foram bem sucedidos e transcorreram sem complicações. No grupo das CIVPM (n=6, dois tinham aneurismas de septo membranoso. Em 5, identificamos dois pertuitos com angiografia seletiva. Utilizamos próteses de Amplatzer para canal arterial (PCA (Amplatzer Duct Occluder® em 1 paciente e próteses específicas para CIVPM nos demais (Amplatzer VSD-MEMB Occluder®. Um oclusor perimembranoso migrou imediatamente depois de liberado, sendo retirado da aorta descendente. Outro paciente apresentou bloqueio atrioventricular total (BAVT, que reverteu após corticoterapia. CONCLUSÃO: O procedimento é tecnicamente complexo e deve ser executado apenas em centros especializados. Apesar disso, mostrou-se seguro e eficaz nos casos selecionados, e pode ser oferecido como alternativa ao tratamento cirúrgico tradicional.OBJECTIVE: To assess the initial experiment with percutaneous closure of interventricular septal defects (IVSD, especially perimembranous IVSD (PMIVSD with the new Amplatzer prosthesis. METHODS

  8. Transcatheter closure of perimembranous ventricular septal defects: single centre experience in China

    Institute of Scientific and Technical Information of China (English)

    LI Tian-chang; HU Da-yi; BIAN Hong; WANG Guo-hong; WANG Xian; ZHU Zheng-yan; XU Yu-yun

    2005-01-01

    @@ Ventricular septal defect (VSD) is one of the most common congenital heart malformation. As an isolated lesion, it accounts for 20%-30% of patients with congenital heart disease.1 Eighty percent of these defects are perimembranous involving the membranous septum and the adjacent area of muscular septum. At least 80% of these defects are small and close spontaneously,2,3 the larger defects often persist to cause significant shunt and right ventricular hypertension. Although conventional surgical repair of perimembranous ventricular septal defects (PMVSDs) is a safe, widely accepted procedure with negligible mortality. It is associated with morbidity, discomfort and a thoracotomy scar.1 As an alternative to surgery, a variety of devices for transcatheter closure of VSD have been developed. However, these devices were not specifically designed for this purpose and none has gained wide acceptance. Large delivery sheaths, inability to recapture and reposition, structural failure, dislodgement and embolization of the device, interference with the aortic valve resulting in aortic insufficiency and a high rate of residual shunting are the major limitations of the previously described techniques.2,3 The initial experiences with transcatheter closure of PMVSDs in patients with a new device that was especially designed for non-surgical occlusion of these defects, the Amplatzer asymmetric VSD occluder (AAVSDO, AGA Medical Co., USA) were encouraging.4-6 But the long-term results are not known.

  9. 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.

  10. 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...... (VSD) or of an atrial septal defect (ASD). METHODS: Participants were 80,346 pregnant women who were enrolled into the Danish National Birth Cohort in 1996-2002 and gave birth to a live-born singleton without any chromosome anomalies. Twice during pregnancy these women were asked about their intake...

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

    Directory of Open Access Journals (Sweden)

    Kátia Marie Simões e Senna

    2015-08-01

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

  12. 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.

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

    Directory of Open Access Journals (Sweden)

    Brown Michael A

    2010-07-01

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

  14. Low molecular weight heparin microcapsule coated occluder for atrial-septal defects

    Institute of Scientific and Technical Information of China (English)

    SUN Yong; WU Jian; ZHANG Ruo-xi; SHI Xiu-jie; LIU Hai-xia; ZHAO Yang; YU Bo

    2009-01-01

    Background Whether the low molecular weight heparin microcapsule coated occluder is helpful to endothelialization in atrial-septal defect models is uncertain. This study aimed to investigate the best conditions for low molecular weight heparin coated Nil-I alloy occluder and provide the evidence of the efficacy and safety of atrial-septal defect occluders in vivo.Methods Low molecular weight heparin microcapsules were investigated using gelatin as microcapsule material. The prepared low molecular weight heparin gelatin particles were subjected to nickel and titanium alloy occluder coating by sodium hyaluronate. A dog model of atrial septal defects was established after treatment with low molecular weight heparin microcapsule coated occluder (n=4) and uncoated occluder (n=4). Endotheliocytes and fibroblastic cells in occluders were observed. And the rate of endothelialization was detected.Results When the concentration of gelatin was 1%, the diameters of particles were mostly about 100 pm, and the particle size was uniform. The envelope efficiency of low molecular weight heparin microcapsule was about 80%. The endothelialization of occluder in the model was more obvious in the coated group than in the uncoated group (P <0.0001).Conclusions Low molecular weight heparin can be prepared into microcapsules with their particle size in nanometric grade. The antithrombotic properties are kept in the nickel and titanium alloy occluder successfully coated with sodium hyaluronate. The endothelialization after the interventional occlusion in the coated group is obvious, indicating that low molecular weight heparin is helpful to the growth of endothelial cells in the occlude and the healing after the interventional occlusion.

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

    OpenAIRE

    Luciana da Fonseca da Silva; José Pedro da Silva; Turquetto,Aida L R; Sonia Meiken Franchi; Cascudo,Cybelle M; Rodrigo Moreira Castro; Walter José Gomes; Christian Schreiber

    2014-01-01

    Introduction:Congenital heart defects treatment shows progressive reduction in morbidity and mortality, however, the scar, resulting from ventricular (VSD) and atrial septal defect (ASD) repair, may cause discomfort. Right axillary minithoracotomy approach, by avoiding the breast growth region, is an option for correction of these defects that may provide better aesthetic results at low cost. Since October 2011, we have been using this technique for repairing VSD and ASD defects as well as as...

  16. Congenital right pulmonary artery agenesis with atrial septal defect and pulmonary hypertension.

    Science.gov (United States)

    Orun, Utku Arman; Yilmaz, Osman; Bilici, Meki; Karademir, Selmin; Uner, Cigdem; Senocak, Filiz; Dogan, Vehbi

    2012-01-01

    Unilateral pulmonary artery agenesis is a rare congenital anomaly caused by a backward displacement of the conical artery of the truncus arteriosus. It is commonly associated with additional cardiovascular abnormalities. A 7-year-old girl was admitted to our clinic with the complaint of shortness of breath upon exertion. Chest radiography revealed a hypoplastic right lung. Absence of the right pulmonary artery with atrial septal defect and pulmonary hypertension was demonstrated by echocardiography, computed tomography, and cardiac catheterization. Bosentan is effectively used to treat pulmonary arterial hypertension.

  17. Occlusion of atrial septal defect utilizing occluder devise via minimally invasive right chest approach

    Institute of Scientific and Technical Information of China (English)

    励峰; 李伟; 康宁; 龚宝生; 吴东进; 徐方杰; 邱兆昆; 吴卫华

    2011-01-01

    Objective To evaluate atrial septal defect (ASD) occlusion employing a small right anterior thoracotomy approach. Methods A total of 21 patients with ASD underwent general anesthesia and 2 -3 cm incision was made in the fourth right intercostal space. Utilizing transesophageal or transthoracic echocardiography, the occluder was released using a monotube unit. Results All patients were occluded successfully. No patient required open surgery utilizing extracorporeal circulation. There were no major complications and no evidence of residual atrial shunt. Conclusion ASD occlusion via a minimal surgical incision is safe, less invasive, and has excellent outcomes.

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

    OpenAIRE

    Ori Shen; Sari Lieberman; Benjamin Farber; Daniel Terner; Amnon Lahad; Ephrat Levy-Lahad

    2014-01-01

    The aim of this study was to examine if isolated fetal ventricular septal defect (VSD) is associated with trisomy 21. One hundred twenty six cases with prenatal VSD diagnosed by a pediatric cardiologist were reviewed. Cases with known risk factors for congenital heart disease, the presence of other major anomalies, soft signs for trisomy 21 or a positive screen test for trisomy 21 were excluded. Ninety two cases formed the study group. None of the cases in the study group had trisomy 21. The ...

  19. Further insights into the syndrome of prolapsing non-coronary aortic cusp and ventricular septal defect

    Directory of Open Access Journals (Sweden)

    Akhlaque N Bhat

    2012-03-01

    Full Text Available Ventricular septal defect ( VSD with prolapse of the right coronary cusp and aortic regurgitation can be managed surgically with the anatomical correction technique . However when the VSD is located underneath the non coronary cusp surgical management differs due to anatomical constraints and secondary pathological changes seen in the non coronary cusp. It is therefore important that the location of the VSD and the morphology of prolapsing cusp be characterised preoperatively in order to plan appropriate surgical repair. We present a case study in which we discuss the salient differences in the surgical management of the prolapsing right and the prolapsing non coronary cusps.

  20. Quadricuspid pulmonary valve associated with atrial septal defects and pulmonary stenosis

    Science.gov (United States)

    Aboitiz-Rivera, Carlos Manuel; Blachman-Braun, Ruben; Ferrer-Arellano, Laura Graciela

    2015-01-01

    A 1-month-old Hispanic female was referred to the cardiology service. During physical examination, a systolic ejection murmur at the pulmonic area was auscultated. The echocardiography evaluation of the pulmonary valve demonstrated a quadricuspid pulmonary valve (QPV) with slightly thickened leaflets, associated atrial septal defects and mild pulmonary stenosis. QPV is a rare congenital anomaly variant that can remain asymptomatic with few or non-hemodynamic alterations. Associations with structural or functional alterations have been reported. This is the first case of QPV that was diagnosed in a Hispanic newborn. PMID:26138189

  1. ATRIAL SEPTAL DEFECT IN A 50 YEARS OLD MALE PRESENTING AS OBLIQUE SEPTAL CANAL THAT APPEARED TO BE REGULATED BY FIVE LUMINAL BANDS

    Directory of Open Access Journals (Sweden)

    Ainory Peter Gesase

    2014-09-01

    Full Text Available To date seven different types of atrial septal defects (ASD have been described and they include septum primum, septum secundum, superior vena cava, inferior vena cava, coronary sinus and patent foramen ovale types of ASD. One feature in common among these ASD’s is that they all present with a hole that may allow communication between the left and right atria. The current observation reports what appears to be a new type of ASD that is characterized by the presence of an oblique septal canal that opens into the right and left atria. The right atrial opening was D-shaped and measured about 0.9 cm wide and the left atrial opening was crescent-shaped and measuring about 0.5 cm wide. In addition to this the left atrial opening was associated with five luminal bands; the last three bands lies on the roof of the oblique septal canal. The action of pulling the first luminal band resulted into closure of the left atrial opening an indication that the bands prevented blood coming from the lungs from entering into the right atrium. The presence of oblique septal canal and luminal bands has not been reported in relation to the ASD’s. Continued documentation of such anomalies remains clinically important particularly in African settings where unexplained illnesses are easily attributed to endemic diseases.

  2. 室间隔缺损介入封堵治疗失败原因分析%Analysing on failure in transcatheter closure of ventricular septal defect

    Institute of Scientific and Technical Information of China (English)

    李军; 张军; 刘利勋; 朱霆; 刘丽文; 朱永胜; 李梅

    2010-01-01

    目的 分析室间隔缺损介入封堵失败病例,提高术前超声心动图筛选患者的手术成功率.方法 对50例室间隔缺损介入封堵手术失败患者的超声心动图检查结果进行分析.结果 14例术中出现房室传导阻滞,13例术后有残余分流,19例无法建立封堵轨道,2例嵴内置人封堵器后出现较明显的主动脉瓣反流,1例冠心病、1例急性心肌梗死伴室间隔穿孔,以上患者均未能完成手术.结论 应用超声心动图注意观察室间隔缺损的左右室侧大小及周边结构.对年龄较小、缺损复杂或年龄较大合并冠心病的患者,应慎用封堵方法.%Objective To analyse the failure reason in transcatheter closure of ventricular septal defect(VSD) ,in order to improve the succeeding rate of occlusive operation on screening VSD patients with echocardiography. Methods Echocardiographic datas were summarized in 50 cases who were failed in VSD occlusion. Results Atrio-ventricular block was occurred in 14 patients,residual shunt was visualized in 13 cases,transcatheter occlusive track could not be set up in 19 cases,arotic valve regurgitation in two cases were screened obviously after occlusive device was released into the intracristal VSD. One case was suffered from cononary heart disease(CAD) and another one was suffered from acute myocardial infarction with ventricular septal perforation. All these patients did not succeed in closure procedure. Conclusions The defect sizes of left and right sides of ventricular septal, structure close to defect must be considered in selecting patients before operation by echocardiography. Occlusion operation should be strictly chosen in cases who were children,or suffered from complex VSD,or senior patients with CAD.

  3. Cyanosis in atrial septal defect without pulmonary hypertension: a case of platypnea-orthodeoxya syndrome.

    Science.gov (United States)

    Di Bella, Isidoro; Pasquino, Stefano; Da Col, Uberto; Ragni, Temistocle

    2005-02-01

    Cyanosis in atrial septal defect typically occurs when pulmonary hypertension develops. Platypnea-orthodeoxya is an uncommon syndrome, still under debate, characterized by breathlessness and arterial oxygen desaturation exacerbated in the upright position. An interatrial communication is a common finding in this syndrome, but the absence of a right to left pressure gradient complicates the physiopathological picture. To explain the right to left shunt, it is generally advocated a concomitant condition that alternates the sterical relationship between inferior vena cava orifice and the atrial septal defect. A case of a 58-year-old male with platypnea-orthodeoxya syndrome related to a fenestrated redundant interatrial septum without any additional pathologic condition is reported. Possibly, this isolated anatomical abnormality could lead to a right to left shunt in the absence of other coexisting predisposing factors. It is reasonable to hypothesize the septum secundum bulging like a 'spinnaker' into the right atrium, so that it deviates the inferior vena cava venous blood towards the left atrium. Echocardiographic evaluation is mandatory to achieve a correct diagnosis and to decide the therapeutic strategy.

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

    Science.gov (United States)

    Baidya, Dalim Kumar; Khanna, Puneet; Kumar, Anil; Shende, Dilip

    2011-01-01

    Blepharophimosis syndrome is an autosomal dominant disorder characterized by eyelid malformation, involvement of reproductive system and abnormal facial morphology leading to difficult airway. We report a rare association of blepharophimosis syndrome and atrial septal defect in a 10-year-old girl who came for reconstruction surgery of eyelid. The child had dyspnea on exertion. Atrial septal defect was identified preoperatively by clinical examination and echocardiography. Anesthesia management was complicated by failure in laryngeal mask airway placement and Cobra perilaryngeal airway was subsequently used. PMID:22096296

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

    Directory of Open Access Journals (Sweden)

    Dalim Kumar Baidya

    2011-01-01

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

  6. Transcatheter closure of atrial septal defect in a patient with absent inferior caval vein connection: a novel technique using a steerable guide catheter.

    Science.gov (United States)

    Takaya, Yoichi; Akagi, Teiji; Ito, Hiroshi

    2016-06-01

    An alternative approach for transcatheter closure of atrial septal defect is necessary in patients with absent inferior caval vein connection. In this report, we describe the successful transcatheter atrial septal defect closure via the transjugular approach using a steerable guide catheter.

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

    Directory of Open Access Journals (Sweden)

    Feridoun Sabzi

    2014-01-01

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

  8. 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 da

  9. The unnatural history of an atrial septal defect: Longitudinal 35 year follow up after surgical closure at young age

    NARCIS (Netherlands)

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

    2013-01-01

    markdownabstractABSTRACT Objective: To describe the very long-term outcome after surgical closure of an atrial septal defect (ASD). Design: Longitudinal cohort study of 135 consecutive patients who underwent surgical ASD repair at age <15 years between 1968 and 1980. The study protocol included EC

  10. Severe hemolytic anemia after repair of primum septal defect and cleft mitral valve.

    Science.gov (United States)

    Alehan, D; Doğan, R; Ozkutlu, S; Elshershari, H; Gümrük, F

    2001-01-01

    Two cases are described in which severe mechanical hemolytic anemia developed after surgical repair of primum atrial septal defect (ASD) and cleft mitral valve. In both cases there was residual mitral regurgitation after repair. Moderate mitral regurgitation and collision of the regurgitant jet with the teflon patch used for repair of the primum ASD were detected by color-Doppler echocardiography imaging. Laboratory tests showed normochromic normocytic anemia, increased indirect serum bilirubin, decreased plasma haptoglobin and hemoglobinuria. The peripheral blood smear contained numerous fragmented red cells. Following another surgical correction of the mitral valve (repair or mitral valve replacement), there was no more hemolysis. The two presented cases show that foreign materials in association with localized intracardiac turbulence may cause severe hemolysis.

  11. Right-side pulmonary agenesis with atrial septal defect in adult.

    Science.gov (United States)

    Das Shukla, Amitabh; Agrawal, Neha; Chandra, Alok; Anantha, Shreenivasa; Chaudhary, Abhinav

    2016-04-01

    Pulmonary agenesis, a rare congenital condition, is incompatible with life when present bilateral, while unilateral agenesis is usually detected in infancy or early childhood. Rare asymptomatic patients may reach adulthood undiagnosed, with signs mimicking common conditions presenting as radiopaque hemithorax with ipsilateral mediastinal shift. Here, we describe a case of a young lady, with history of consanguinity, who presented with complaints, suggestive of lower respiratory tract infection, and was investigated and diagnosed to be a case of right-side pulmonary agenesis with large ostium secondum atrial septal defect. Our present case emphasizes the importance of presence of pulmonary agenesis with cardiac congenital anomaly, remaining asymptomatic until adulthood, particularly in patients born of parents with consanguineous marriages.

  12. 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.

  13. Diversion of the inferior vena cava following repair of atrial septal defect causing hypoxemia.

    Science.gov (United States)

    Thompson, Ellen; Moritz, Dennis; Perdue, Romaine; Cansino, Silvestre

    2004-05-01

    Atrial septal defects (ASDs) are a common congenital abnormality, and operative repair is a routine, safe procedure. Diversion of the inferior vena cava (IVC) into the left atrium is an unusual complication following ASD closure. We report a case that illustrates the problem created by this right-to-left shunt. A middle-aged woman underwent ASD repair. She developed hypoxemia postoperatively. A transthoracic echocardiogram confirmed a right-to-left shunt, found only with agitated saline injected into the femoral vein, not into the basilic vein. Surgical reexploration revealed a residual ASD diverting IVC flow into the left atrium, which was repaired with a pericardial patch. Echocardiography with agitated saline injected from the femoral vein is an easy method to diagnose this uncommon complication.

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

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    Ori Shen

    2014-04-01

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

  15. Use of steerable delivery catheter to successfully deliver a Ceraflex septal occluder to close an atrial septal defect in a child with interrupted inferior vena cava with azygos continuation.

    Science.gov (United States)

    Yücel, İlker K; Ballı, Şevket; Küçük, Mehmet; Çelebi, Ahmet

    2016-04-01

    The closure of a secundum atrial septal defect through the jugular vein in a child with interrupted inferior vena cava with azygos continuation by steerable delivery catheter is described in the present report. The steerable catheter can be used to correct the perpendicular position of the device over the margins of the defect, and is particularly useful in cases of large defects.

  16. Percutaneous closure of postinfarction ventricular septal defect: cardiac magnetic resonance-guided case selection and postprocedure evaluation.

    Science.gov (United States)

    Artis, Nigel J; Thomson, John; Plein, Sven; Greenwood, John P

    2011-01-01

    Despite modern surgical techniques, complications and early mortality remain high following postinfarction ventricular septal defect (VSD) repair. It is now possible to close these acquired defects percutaneously using, for example, the Amplatzer postinfarct muscular VSD device. Cardiovascular magnetic resonance is an important tool in determining appropriate case selection and device sizing as it can provide a multicomponent assessment of the VSD anatomy, ventricular volumes and function, infarct extent, and left-to-right shunt calculations.

  17. 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.)

  18. 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.)

  19. 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.

  20. Home-made fenestrated amplatzer occluder for atrial septal defect and pulmonary arterial hypertension

    Institute of Scientific and Technical Information of China (English)

    Fabio Dell'Avvocata; Gianluca Rigatelli; Paolo Cardaioli; Massimo Giordan

    2011-01-01

    We report the management of a patient with secundum atrial septal defect (ASD)and severe pulmonary hypertension.A 65-year-old male with recently diagnosed atrial serital defect was referred to our centre for decompensated right heart failure with rest and exercise induced dispnea and severe pulmonary hypertension.Right heart catheterization confirmed a mean pulmonary pressure of about 55 mmHg and a Qp/Qs of 2.7.An occlusion test with a compliant large balloon demonstrated partial fall of pulmonary arterial pressure.The implantation of a home-made fenestrated Amplatzer ASD Occluder (ASO) was planned in order to decrease lefttoright shunt and promote further decrease of pulmonary arterial pressure in the long-term.Thus,by means of mechanical intracardiac echocardiography study with a 9F 9 MHz Ultralce catheter(Boston Scientific Corp.),we selected a 34 mm ASO for implantation.Four millimeter fenestration was made inflating a 4 ntm non-compliant coronary balloon throughout the waist of the ASO,which was successfully implanted under intracardiac echocardiography.After six months,a decrease of pulmonary arterial pressure to 24 mmHg and full compensated right heart failure was observed on transthoracic echocardiography and clinical examination.This case suggests that transcatheter closure with home-made fenestrated ASD in elderly patients with severe pulmonary hypertension is feasible.

  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. Novel and functional DNA sequence variants within the GATA5 gene promoter in ventricular septal defects

    Institute of Scientific and Technical Information of China (English)

    Ji-Ping Shan; Xiao-Li Wang; Yuan-Gang Qiao; Hong-Xin Wan Yan; Wen-Hui Huang; Shu-Chao Pang; Bo Yan

    2014-01-01

    Background: Congenital heart disease (CHD) is the most common human birth defect. Genetic causes for CHD remain largely unknown. GATA transcription factor 5 (GATA 5) is an essential regulator for the heart development. Mutations in the GATA5 gene have been reported in patients with a variety of CHD. Since misregulation of gene expression have been associated with human diseases, we speculated that changed levels of cardiac transcription factors, GATA5, may mediate the development of CHD. Methods: In this study, GATA5 gene promoter was genetically and functionally analyzed in large cohorts of patients with ventricular septal defect (VSD) (n=343) and ethnic-matched healthy controls (n=348). Results: Two novel and heterozygous DNA sequence variants (DSVs), g.61051165A>G and g.61051463delC, were identified in three VSD patients, but not in the controls. In cultured cardiomyocytes, GATA5 gene promoter activities were significantly decreased by DSV g.61051165A>G and increased by DSV g.61051463delC. Moreover, fathers of the VSD patients carrying the same DSVs had reduced diastolic function of left ventricles. Three SNPs, g.61051279C>T (rs77067995), g.61051327A>C (rs145936691) and g.61051373G>A (rs80197101), and one novel heterozygous DSV, g.61051227C>T, were found in both VSD patients and controls with similar frequencies. Conclusion: Our data suggested that the DSVs in the GATA5 gene promoter may increase the susceptibility to the development of VSD as a risk factor.

  3. Defective T wave combined with incomplete right bundle branch block: a new electrocardiographic index for diagnosing atrial septal defect

    Institute of Scientific and Technical Information of China (English)

    WANG Mu-xuan; Andrew D.Michaels; MA Hong; WU Gui-fu; GU Jing-li; LI Li; LU Kun; YANG Da; CHEN Long; ZHANG Xi; LUO Fu-tian

    2012-01-01

    Background Incomplete right bundle branch block (ICRBBB) is commonly associated with atrial septal defect (ASD),but lacks sufficient diagnostic test characteristics.An abnormal T wave is also often observed in ASD,with horizontal or inverted displacement of the proximal T wave limb in the right precordial leads,termed "defective T wave" (DTW).Methods We examined the diagnostic test characteristics of combining ICRBBB with DTVV as a new index to diagnose ASD.A total of 132 consecutive patients with ASD and 132 cases of age/gender-matched controls without ASD were enrolled.Results Sensitivities of DTW,ICRBBB,and both were 87.1%-87.9%.Specificities were 97.0%,96.2%,and 100%,respectively.Positive predictive values were 1.3%,1.1%,and 100.0% respectively,while negative predictive values were 99.9% for each.Conclusion Combining ICRBBB with DTW in electrocardiogram (ECG) as a new index significantly increased the specificity and positive predictive values while maintaining a high sensitivity in diagnosing ASD.

  4. Novel Mutations in the Transcriptional Activator Domain of the Human TBX20 in Patients with Atrial Septal Defect

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    Irma Eloisa Monroy-Muñoz

    2015-01-01

    Full Text Available Background. The relevance of TBX20 gene in heart development has been demonstrated in many animal models, but there are few works that try to elucidate the effect of TBX20 mutations in human congenital heart diseases. In these studies, all missense mutations associated with atrial septal defect (ASD were found in the DNA-binding T-box domain, none in the transcriptional activator domain. Methods. We search for TBX20 mutations in a group of patients with ASD or ventricular septal defect (VSD using the High Resolution Melting (HRM method and DNA sequencing. Results. We report three missense mutations (Y309D, T370O, and M395R within the transcriptional activator domain of human TBX20 that were associated with ASD. Conclusions. This is the first association of TBX20 transcriptional activator domain missense mutations with ASD. These findings could have implications for diagnosis, genetic screening, and patient follow-up.

  5. Retraction: Selective cerebro-myocardial perfusion under mild hypothermia during primary repair for aortic coarctation with ventricular septal defect.

    Science.gov (United States)

    2013-04-01

    The following article from Artificial Organs, "Selective Cerebro-Myocardial Perfusion Under Mild Hypothermia During Primary Repair for Aortic Coarctation With Ventricular Septal Defect" by Huiwen Chen, Haifa Hong, Zhongqun Zhu and Jinfen Liu, published online on 2 November 2012 in Wiley Online Library (wileyonlinelibrary.com), has been retracted by agreement between the authors, the journal Editor-in-Chief, Paul S. Malchesky, the International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. The retraction has been agreed due to simultaneous publication of a substantially similar article, "Continuous Cerebral and Myocardial Perfusion During One-Stage Repair for Aortic Coarctation With Ventricular Septal Defect", by Huiwen Chen, Haifa Hong, Zhongqun Zhu and Jinfen Liu, in Pediatric Cardiology 7 November 2012 [Epub ahead of print].

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

    Directory of Open Access Journals (Sweden)

    Maitrey D Gajjar

    2015-01-01

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

  7. Hepatic and Renal Failure after Anterior Myocardial Infarction Induced Apical Ventricular Septal Defect

    Directory of Open Access Journals (Sweden)

    Dirk Lossnitzer

    2010-01-01

    Full Text Available We report the case of a 68-year-old man suffering from incremental hepatic and renal failure one month after anterior myocardial infarction. Cardiac MRI showed a pronounced apical post-AMI aneurysm, a moderate to severe mitral and tricuspid regurgitation as well as a hemodynamically highly significant 12 mm apical ventricular septal defect with a left-to-right ventricular shunt of almost 63% as the underlying cause. Heart X-ray revealed a severe LAD in-stent restenosis. CAPD catheter drainage of hydroperitoneum due to congestive liver and renal failure was provided in combination with intensified CAPD hemodialysis. Heart surgery was performed where the apical aneurysm was excised, the mitral valve was reconstructed, the IVSD was closed and the subtotally in-stent occluded LAD was bypassed. Post-surgery, the ascites were significantly reduced, and CAPD hemodialysis therapy could be terminated since the renal function gradually improved (MDRD = 25 mL/min. To our knowledge, for the first time we report successful CAPD catheter drainage of hydroperitoneum in combination with CAPD hemodialysis.

  8. Percutaneous closure of atrial septal defects leads to normalisation of atrial and ventricular volumes

    Directory of Open Access Journals (Sweden)

    Worthley Matthew I

    2008-12-01

    Full Text Available Abstract Background Percutaneous closure of atrial septal defects (ASDs should potentially reduce right heart volumes by removing left-to-right shunting. Due to ventricular interdependence, this may be associated with impaired left ventricular filling and potentially function. Furthermore, atrial changes post-ASD closure have been poorly understood and may be important for understanding risk of atrial arrhythmia post-ASD closure. Cardiovascular magnetic resonance (CMR is an accurate and reproducible imaging modality for the assessment of cardiac function and volumes. We assessed cardiac volumes pre- and post-percutaneous ASD closure using CMR. Methods Consecutive patients (n = 23 underwent CMR pre- and 6 months post-ASD closure. Steady state free precession cine CMR was performed using contiguous slices in both short and long axis views through the ASD. Data was collected for assessment of left and right atrial, ventricular end diastolic volumes (EDV and end systolic volumes (ESV. Data is presented as mean ± SD, volumes as mL, and paired t-testing performed between groups. Statistical significance was taken as p Results There was a significant reduction in right ventricular volumes at 6 months post-ASD closure (RVEDV: 208.7 ± 76.7 vs. 140.6 ± 60.4 mL, p Conclusion ASD closure leads to normalisation of ventricular volumes and also a reduction in right atrial volume. Further follow-up is required to assess how this predicts outcomes such as risk of atrial arrhythmias after such procedures.

  9. An evaluation of the left atrial/aortic root ratio in children with ventricular septal defect.

    Science.gov (United States)

    Lester, L A; Vitullo, D; Sodt, P; Hutcheon, N; Arcilla, R

    1979-08-01

    Echocardiograms were performed in 80 infants and children with isolated ventricular septal defect (VSD) who underwent cardiac catheterization. The pulmonary-to-systemic flow ratio (Qp/Qs) was correlated with the echocardiographic left atrial-to-aortic root diameter ratio (LA/Ao), and a relatively poor correlation (r = 0.62) was found. The end-systolic diameters of the left atrium and aorta at the level of the aortic root, obtained from lateral cineangiograms of 55 of the 80 patients, were compared with the corresponding echocardiographic dimensions. To assess the possible effect of transducer beam angulation upon the echocardiographic determinations, the angiographic measurements were made at 0 degrees position (perpendicular to the frontal plane) and at angles of 5 degrees, 10 degrees, 15 degrees and 20 degrees from zero, using the aortic root center as the point of intersection. The echocardiographic and angiographic aortic root measurements were comparable (r = 0.95), and the angiographically derived aortic diameter did not vary with different angle projections. However, the left atrial angiographic dimensions were significantly influenced by the angle of projection. We conclude that the echocardiographic LA/Ao ratio cannot reliably estimate the severity of the shunt flow in VSD.

  10. Transcatheter Closure of Patent Ductus Arteriosus and Atrial Septal Defects Using the Amplatzer Occluder

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective:To evalte the efficiency,safety and complications of transcatheter closure of patent ductus arteriosus (PDA) or secundum atrial septal defects (ASD)using the Amplatzer occluder device.Methods:30 patients underwent transcatheter closure of PDA or ASD with the Amplatzer occluder.The lateral descending aortographies were performed to evaluate immediate results in the 20 patients of PDA.Hemodynamics was studied before and after the procedure.X-ray and echocardiography were performed in order to detect residual shunt and recanalization.Results:The device was successfully implanted in 28 patients.There was on clinical evidence of hemolysis andon incidence of device emboliszation.The median operation time waw 56min and median fluoroscopy time was 11min .The devices'positions were optmal and on residual shunt was found 24h and 1month after the procedure No complicatios were observed during the 3-month follow-up in 25 patients.Conclusions:The Amplatzer occluder device is a highly efficient prosthesis that can be safely applied in most patients with PDA or ASD.

  11. Clinical Study of 147 Cases with Ventricular Septal Defect and Aortic Valve Insufficiency in Children

    Institute of Scientific and Technical Information of China (English)

    李渝芬; 李江林; 王树水; 庄建; 陈欣欣

    2004-01-01

    Objectives To introduce our experience in treatment of ventricular septal defects with aortic valve insufficiency. Methods A total of 147 cases was involved in a nine-year retrospective study. Age ranged from 5 months to 15 years (mean,7.60 ± 4.12 years). All had been diagnosed by thorough history, physical examinations, chest roentgenogram, two-dimensional echocardiogram.Some 103 patients had taken catheterization and angiocardiography. 91 (61.9%) cases underwent the procedure of VSD closure, 31 (21.08%) had aortic valvuloplasty simultaneously, and 25 (17.02%) were performed VSD closure plus aortic valve replacement.Results Among the 147 patients, 137 (93.19%) have fully recovered, 6 (4.08%) improved, and 4 patients died (2.73%). Conclusions As soon as being diagnosed as subarterial VSD, surgical intervention should be recommended at an early date. The cases of perimembranous VSD also need close follow-up. Once AI occurs, operations should be taken in time. The cases without AI ought to accept treatment during school age.

  12. Utilization of 3 amplatzer occluders for closure of post-myocardial infarction ventricular septal defect.

    Science.gov (United States)

    Kar, Saibal; Ibebuogu, Uzoma N; Conte, Antonio Hernandez

    2012-05-01

    This case report describes a patient who sustained a post-myocardial infarction ventricular septal defect (VSD) with an associated left ventricular aneurysm who developed cardiogenic shock and required an intra-aortic balloon pump for hemodynamic stabilization. After deployment of a single Amplatzer occluder (AGA Medical), a residual VSD measuring 0.5 cm was noted. Therefore, a second Amplatzer occluder was deployed and a minimal residual VSD remained. The patient remained hemodynamically stable throughout the procedure and was subsequently extubated with removal of intra-aortic balloon pump. Post-discharge, the patient was readmitted with congestive heart failure. A third Amplatzer device was deployed to ameliorate the recurrent VSD shunt. At 9-week follow-up, transthoracic echocardiogram was performed and findings included: 1) left ventricular ejection fraction of 62%; 2) appearance of 3 Amplatzer devices along the interventrcular septum seated well with no motion and residual shunt; 3) moderate diastolic dysfunction with pseudonormal left ventricular filling pattern; and 4) no valvular abnormalities. The patient had increased exercise tolerance with no shortness of breath at rest or with exertion. This case demonstrates the utility and viability of multiple Amplatzer device deployment as a means of repairing a large post-myocardial infarction VSD and recurrent VSDs.

  13. 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.

  14. Association of NFATc1 gene polymorphism with ventricular septal defect in the Chinese Han population

    Institute of Scientific and Technical Information of China (English)

    SHEN Lei; LI Zhong-zhi; SHEN A-dong; LIU Hui; BAI Song; GUO Jian; YUAN Feng

    2013-01-01

    Background Congenital heart disease (CHD) is a diverse group of diseases determined by genetic and environmental factors.Considerable research has been done on genes associated with the development of the heart.Recently,focus is on the role of transcription factor NFATc1 in the development of proper valve and septa.As part of a larger study,high density single nucleotide polymorphism (SNP) scanning was used to explore the relationship between NFATc1 gene polymorphism and susceptibility to ventricular septal defect (VSD) in the Chinese Han population.Methods One hundred and ninety-two pediatric patients with congenital VSD and 192 matching healthy control subjects were studied.The haplotype reconstructions were calculated by PHASE2.0 software.Haploview software was used to perform linkage disequilibrium assessment and define haplotype blocks.The algorithm used for defining the blocks was the confidence interval method.Results The NFATc1 gene region can be divided into 11 haplotype blocks.Strong linkage disequilibrium existed within blocks 6,8,9,and 11.Three SNPs (rs7240256,rs11665469,and rs754505) within the NFATc1 gene had significant correlation with VSD by single marker association analysis.In addition,two haplotypes correlated with VSD.Conclusions NFATc1 is associated with the occurrence of VSD and it may be a predisposing gene to CHD in Han Chinese.This finding has set a direction for further genetic and functional studies.

  15. 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.

  16. Right heart function assessment with real-time three-dimensional echocardiography before and after atrial septal defect surgery

    Institute of Scientific and Technical Information of China (English)

    Hua Zhao; Yi-Min Fu; Yong-Mei Jia

    2016-01-01

    Objective:To study the clinical value of right heart function assessment with real-time three-dimensional echocardiography before and after atrial septal defect surgery. Methods:Patients with atrial septal defect who received transcatheter closure in our hospital were selected for study and divided into non-pulmonary hypertension group and pulmonary hypertension group according to pulmonary artery systolic pressure (PASP), real-time three-dimensional echocardiography was conducted before and after operation, and right ventricular end-diastolic volume (RVEDV), right ventricular end-systolic volume (RVESV), right ventricular stroke volume (RVSV), right ventricular ejection fraction (RVEF) and right ventricular cardiac output (RVCO) were calculated;serum was collected, and brain natriuretic peptide (BNP), atrial natriuretic peptide (ANP), angiotensin I (AngI), angiotensin II (AngII) and endothelin (ET) contents were detected. Results:After operation, RVEDV, RVESV, RVSV, RVEF and RVCO as well as serum BNP, ANP, AngI, AngII and ET contents of both groups were lower than those before treatment;RVEDV, RVESV, RVSV, RVEF and RVCO were positively correlated with contents of BNP, ANP, AngI, AngII and ET. Conclusions:Using real-time three-dimensional echocardiography before and after atrial septal defect surgery can accurately assess right heart function, and it has good correlation with right heart volume load and pulmonary circulation blood flow.

  17. Improvement of the Technique for Transcatheter Closure of Atrial Septal Defect in Children

    Institute of Scientific and Technical Information of China (English)

    Wang Huishen; Qian Mingyang; Zhang Zhiwei

    2005-01-01

    Objectives To improve experience of procedure and success rate of interventional treatment of atrial septal defect (ASD) in children, applying the technique of controlling release of devices in the pulmonary vein (controlling two disc of device opening for subsequence) in children cases with ASD who can not be occluded by regularly interventional treatment. Methods Since 2000 year 182 child cases (male 70 and 112 female) underwent the procedure of controlling release of devices in the pulmonary vein. The patients' age was from 2 to 14years old (average 3.77±1.55). The body weight was from 9 to 48 Kg (average 21.53±10.63). When the devices were placed on the right position with difficulty and failure in some cases with short and soft rims of the defect and large defect and the angle between the device and the interval atrial septal (IAS), It could be helpful to put the device into the left upper pulmonary vein, and to make right atrium (proximal) disc opened before the left atrium (distal) disc naturally fall down.At the end the double disc of the device clamped and stood up at the right position of the IAS. After closure of ASD, patients were followed up regularly by echocardiography, X-ray and ECG in the 1,3,6,12month and 3,5 years. Results The successful rate of device implantation in the improving group (98.4%)was obviously higher than that in the regularly group (68%). The techniques improved in this group with the smaller age, the lighter weight, the larger defect and the larger device comparing with the regularly group.The velocity of the pulmonary vein before occlusion procedure was (0.54±0.15)m/s; after procedure was (0.56±0.16)m/s, P > 0.05,there were no significant difference. All cases couldn't found pulmonary congestion by follow up. Conclusions The method of controlling release of device in the pulmonary vein has been used more than 5 years in the occlusion of ASD with double disc device. It is feasible and safety. The aim of the improvement is

  18. Percutaneous closure of secundum atrial septal defects: Experience of a tertiary referral center

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    Ali Hikmet Kırdök

    2012-03-01

    Full Text Available Objectives: This study was aimed to evaluate our clinical experiences and investigate results of percutaneous closure of secundum atrial septal defects (ASD in our clinic.Results: We retrospectively included 70 patients (19 male, 51 female undergoing percutaneous ASD closure procedure with mean age of 36±14.8 (17-75 in our clinic between March 2008 and January 2010. Defect diameter measured by transesophageal echocardiography was 19.7±6.3 mm (6-32 mm. Device size used for percutaneous closure of ASD was 23.7±6.2 (12-36. Devices used for percutaneous closure were nitinol-based devices including Amplatzer (64.7%, Cardiofix (26.7% and biodegredable BioStar (8.6%. The percutaneous closure procedure was successful at 68 of 70 (97% patients. In 2 patients procedure failed. Failure reason was device embolization in one patient and device strut fracture in other; so these 2 patients referred to surgery. During follow up 2 more patients also referred to surgery because of device embolization in first day control. In 3 patients (4.2% supraventricular arrythmias that are converted to sinus by cardioversion are observed. Mean follow up interval was 18.6±9.6 months (1-44 and during this period peripheral vascular complications, cerebrovascular accidents, thrombus on devices, device erosion or death is not observed.Conclusion: Percutaneous closure of secundum ASD which started to replace surgical treatment of ASD in last decades is safe and effective method in short to mid-term period. However because of potentially serious complication risks it should be performed in special centers by operators who are experienced in treating structural heart diseases.

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

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    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.

  20. 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.

  1. Repair of Atrial Septal Defect via Right Submammary Minithoracotomy in Children

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    Hamid Bigdelian

    2015-03-01

    Full Text Available Background: Atrial Septal Defect (ASD is one of the most common congenital heart diseases that may lead to pulmonary hypertension. Advantages of ASD closure by limited thoracotomy as a minimally invasive approach includes reduction of post-operative complications and improvement of post-operative recovery. Objectives: The present study aimed to assess the safety of right submammary minithoracotomy for repair of ASD in children and to evaluate the cosmetic and functional results of this approach. Patients and Methods: Between August 2010 and August 2013, 35 children underwent heart operations for ASD closure via right submammary thoracotomy. The standard anterolateral thoracotomy technique entailed a 4 - 5 cm right submammary incision. After establishment of cardiopulmonary bypass, the right atrium was opened and defect was closed by pericardial patch. The thoracotomy was closed in a routine fashion. Results: The study patients included 5 males (14.29% and 30 females (85.71% whose age ranged from 1 to 7 years. Among the patients, 30 had ASD and 5 had a sinus venosus type. There were no intraoperative complications regarding exposure, cannulation, or bleeding. There were also no deaths in the post-operative period. Postoperative complications included significant hemorrhage from the suture line in one case and sick sinus syndrome requiring pacemaker implantation in another case. The mean length of stay in the intensive care unit was 2 days and the mean length of hospital stay was 4.5 days. Conclusions: Our study findings indicated that use of right submammary thoracotomy technique to repair ASD could be accomplished safely and provided good short-term results in terms of cosmetics, especially in female patients.

  2. 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

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

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    Pedra Simone

    2010-01-01

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

  4. Surgical Closer of Atrial Septal Defect in Adults after 40 Years Old

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    Salehi Rezvanieh

    2014-01-01

    Full Text Available Objective: Atrial septal defect (ASD is the most common congenital heart disease in adults. The aim of this study was to determine the value of surgical closure of ASD in patients over 40 years of age. Materials and Methods: Retrospective analysis of 96 patients with the mean age of 47.58 ± 6.59, who had undergone surgical repair of ASD, was carried out. Pre and postoperative clinical status, New York Heart Association (NYHA functional class and systolic pulmonary artery pressure before and after surgical repair, complications of post operation were assessed and analyzed. The follow-up period was between 1 month and 16 years. Results: Before the operations, 62 patients (89.8% were placed in NYHA functional classes of II and III. However, after the surgeries most of the patients could be placed in the functional classes I and II. The mean of pulmonary artery pressure before the surgeries was about 46.68 ± 14.18 and dropped to 32 ± 11.89 mm Hg after the operations (P < 0.0001. Atrial fibrillation rhythm was present in 18 cases preoperatively which was reduced to 3 patients at the discharge time (16.6%. The mean right ventricular (RV sizes were reduced from 4.1 to 2.5 cm after the surgical repairs. In 35 cases (36.64%, we had no tricuspid valve regurgitation after surgery for RV systolic presser estimation. Conclusion: Surgical closure of ASD in patients over 40 years of age could improve their clinical status, and lead to a reduced pulmonary systolic as well as smaller RV sizes.

  5. 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

  6. Conduction Disorders in Continuous Versus Interrupted Suturing Technique in Ventricular Septal Defect Surgical Repair

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    Gholampour-Dehaki

    2016-01-01

    Full Text Available Background Ventricular septal defects (VSD is one of the most frequent congenital cardiac malformations and cardiac conduction disorders are still one of the serious postoperative complications in this surgery. Objectives This study aimed to compare the incidence of conduction disorders with the use of continuous compared to interrupted suturing techniques in VSD surgical repair. Patients and Methods Previously recorded data of 231 patients who underwent surgical closure of VSD between January 2009 and January 2012 at the Rajaie cardiovascular medical and research center were retrospectively reviewed. VSD surgical repair was performed using continues suturing technique in group A patients (n = 163, 70.6% and interrupted suturing technique in group B patients (n = 68, 29.4%. Results The most common concomitant congenital anomaly was Tetralogy of Fallot (27.3%. Twenty-four (10.4% patients had intraoperative cardiac arrhythmia, including 19 (8.2% transient and 5 (2.2 % permanent arrhythmia. During their ICU stay, ventricular arrhythmia and complete heart block were observed in 34 (14.7% and 5 patients (2.2%, respectively. At the time of the last follow-up, incomplete right bundle branch block (RBBB, complete RBBB, RBBB with left anterior hemi-block, and complete heart block were identified in 84 (36.4%, 42 (18.2%, 29 (12.6%, and 5 patients (2.2%, respectively. The results revealed that group A patients were most likely to have had cardiac arrhythmias during their ICU stay and at the time of last follow-up (P < 0.001, while the intraoperative incidence of cardiac arrhythmia during surgery was not statistically significant between the two groups (P = 0.06. Conclusions In the absence of any statistical differences in the other risk factors between the two groups, the difference in the incidence of conduction disorders can be attributed to the type of suturing used during the procedure.

  7. QUANTIFICATION OF RIGHT VENTRICULAR FUNCTION IN ATRIAL SEPTAL DEFECT USING ULTRASOUND-BASED STRAIN RATE IMAGING

    Institute of Scientific and Technical Information of China (English)

    MENG Xiang-chun; SUN Kun; ZHANG Yu-qi; HUANG Mei-rong; GAO Wei; ZHANG Zhi-fang; SHEN Rong; CHEN Shu-bao

    2005-01-01

    Objective To study the validation of ultrasound-based strain rate imaging in the quantitative assessment of right ventricular (RV) function in atrial septal defect (ASD). Methods Tissue Doppler images (TDI) of RV longitudinal and short axes were recorded from the apical 4-chamber view and the subcostal short-axis view in 18 normal controls, 28 children with ASD and 14 children after Amplazter closure of ASD respectively. Peak systolic velocities (V), peak systolic strain rates (SR), peak systolic strains (S) at the basal segment, middle segment of RV lateral wall and the basal septum from the longitudinal axis, the middle segment of RV free wall from the short axis were quantitatively measured using QLAB TM tissue velocity quantification software system respectively. Peak dp/dt from the RV isovolumic contraction determined during the right cardiac catheterization in 28 ASD patients was used as the gold standard of RV contractility. Peak systolic indices were compared against max dp/dt by linear correlation. Results Peak systolic indices at the basal and middle segments of RV lateral wall from the longitudinal axis increased significantly in 28 ASD patients.Peak systolic indices at the basal septum also increased in patient group, but not significantly. Significant decreases in peak systolic indices at the basal and middle segments of RV lateral wall were observed after the Amplatzer closure in 14 ASD patients. There was no significant difference at the middle segment of RV free wall from the short axis between patient group and normal control. A strong correlation was found between max dp/dt and peak systolic indices at the basal and middle segments of RV lateral wall (P<0.05). Conclusion Ultrasound-based strain rate imaging can assess quantitatively RV function in CHD. Peak systolic strains determined at the basal and middle segments of RV lateral wall are strong noninvasive indices of RV contractility.

  8. 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. 

  9. Measurement of Atrial Septal Defect Size: A Comparative Study between Transesophageal Echocardiography and Balloon Occlusive Diameter Method

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    Bahareh Eslami

    2010-05-01

    Full Text Available Background: Transcatheter closure of atrial septal defect secundum (ASD-II has become an alternative method for surgery. We sought to compare the two-dimensional transesophageal echocardiography (TEE method for measuring atrial septal defect with balloon occlusive diameter (BOD in transcatheter ASD-II closure.Methods: A total of 39 patients (71.1% female, mean age: 35.31 ± 15.37 years who underwent successful transcatheter closure of ASD-II between November 2005 and July 2008 were enrolled in this study. Transthoracic echocardiography (TTE and TEE were performed to select suitable cases for device closure and measure the defect size before the procedure, and BOD measurement was performed during catheterization via TEE. The final size of the selected device was usually either equal to or 1 – 2 mm larger than the BOD of the defect.Results: The mean defect size obtained by TEE and BOD was 18.50 ± 5.08 mm and 22.86 ± 4.76 mm, respectively. The mean difference between the values of ASD size obtained by TEE and BOD was 4.36 ± 2.93 mm. In comparison with BOD, TEE underestimated the defect size in 94.9%, but TEE value being equal to BOD was observed in 5.1%. There was a good linear correlation between the two measurements: BOD = 0.773 × ASD size by TEE+8.562; r2 = 67.9.1%. A negative correlation was found between TEE sizing and the difference between BOD and TEE values (r = -0.394, p value = 0.013.Conclusion: In this study, BOD was larger than ASD size obtained by two-dimensional TEE. However, TEE maximal defect sizing correlates with BOD and may provide credible information in device size selection for transcatheter ASD closure.

  10. Device closure of secundum atrial septal defect in a 4.5 kilogram infant: Novel use of the Amplatzer DuctOccluder II device.

    Science.gov (United States)

    McCrossan, B A; Walsh, K P

    2016-02-01

    Modest secundum atrial septal defects (2°ASD) may cause significant pulmonary over perfusion during infancy, particularly in conjunction with left heart obstructive lesions. Amplatzer Septal Occluders are not ideal in this setting especially given recent concerns regarding device erosion. We report the first use of the Amplatzer Duct Occluder II device (ADO2) to close a 2°ASD in a 4.5 kg infant.

  11. Percutaneous closure of atrial septal defects without balloon sizing in adults: Experience of a tertiary referral center

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    Hasan Kaya

    2013-03-01

    Full Text Available Objective: We aimed to evaluate our clinical experienceand short-term results of percutaneous closure of secundumtype atrial septal defects (ASD in adults.Methods: We studied 71 patients (49 female, 22 male,mean age 35±14 years undergoing percutaneous closureof ASD between January 2010-October 2012 in ourclinic. All procedures were performed under sedoanalgesiawithout balloon sizing. Clinical characteristics of patients,properties of percutaneous closure intervention,complications and short-term results are evaluated.Results: Defect diameter measured by transesophagealechocardiographic examination was 19.8±6.4 mm. Devicesize used for percutaneous closure was 24.7±6.7mm. Procedure was successfully performed in 67 patients(94%. Four patients were referred for surgery because ofprocedural failure. During procedure, a patient developedtransient 2nd degree AV block, and another developeddevice thrombosis. Residual shunt was detected in threepatients at first day control echocardiographic examination.In the follow-up of 13±8 months, no residual shunt,embolic or arrhythmic complications were observed.Conclusion: Percutaneous closure of secundum ASD isa safe and effective method with high success and lowcomplication rates in experienced centers. J Clin Exp Invest2013; 4 (1: 67-72Key words: Secundum atrial septal defect, percutaneousclosure, sedoanalgesia, transesophageal echocardiography

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

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    Ke Shuan Chow

    2015-04-01

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

  13. Maternal MTHFR C677T genotype and septal defects in offspring with Down syndrome: A pilot study

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    Ghada M. Elsayed

    2014-01-01

    Conclusions: MTHFR 677CT genotype could be implicated as a maternal risk factor for septal defects especially in children with DS. Carriers of this genotype may have more risk of development of AV canal in their children. A major limitations of this study was the small sample size and so further studies on a larger sample of patients and their mothers in addition to measurement of homocysteine level in this population is needed to investigate this theory and to clarify the actual role of MTHFR polymorphism and the risk of development of CHD in DS.

  14. Ectopia cordis with a double outlet right ventricle, large ventricular septal defect, malposed great arteries and left ventricular hypoplasia.

    Science.gov (United States)

    Malik, Rabiya; Zilberman, Mark V; Tang, Liwen; Miller, Susan; Pandian, Natesa G

    2015-03-01

    Ectopia cordis, defined as partial or complete displacement of the heart outside of the thoracic cavity, is a rare congenital malformation. If not surgically corrected during the early years of life, ectopia cordis can prove to be a fatal abnormality. However, due to the presence of multiple intracardiac and extracardiac malformations, a corrective surgery might not always be successful. The pathology of ectopia cordis with a double outlet right ventricle, large ventricular septal defect, malposed great arteries and left ventricular hypoplasia is discussed, highlighting the complexities involved in such a rare disorder.

  15. 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...

  16. Post-traumatic ventricular septal defect: a rare indication for extracorporeal membrane oxygenation as a bridge to transplant

    Science.gov (United States)

    Hernández-Pérez, Francisco José; Gómez-Bueno, Manuel Francisco; Escudier-Villa, Juan Manuel; Castedo, Evaristo; Segovia, Javier; Alonso-Pulpón, Luis Antonio

    2017-01-01

    A ventricular septal defect (VSD) is a rare complication of blunt chest trauma. This report presents the case of a 44-year-old man who developed a VSD as a result of high-energy closed chest trauma. We describe the initial surgical and medical management of the cardiac rupture. After failed repair surgery, extracorporeal membrane oxygenation (ECMO) was used as a bridge to heart transplantation. We discuss the successful use of ECMO to improve the prognosis results in this rare and complex entity. PMID:28164015

  17. Circulating ghrelin was negatively correlated with pulmonary arterial pressure in atrial septal defect patients

    Institute of Scientific and Technical Information of China (English)

    LI Zhao-feng; ZHOU Da-xin; PAN Wen-zhi; ZHANG Lei; GE Jun-bo

    2013-01-01

    Background Ghrelin was found to attenuate the magnitude of pulmonary arterial hypertension and pulmonary vascular remodeling in rats.The objective of this study was to explore the fasting plasma ghrelin level and the relationships between ghrelin and pulmonary arterial pressure (PAP) in atrial septal defect (ASD) patients with pulmonary arterial hypertension (PAH).Methods Fasting plasma ghrelin,obestatin,and insulin levels were measured by enzyme linked immunosorbent assay (ELISA) method in ASD patients with or without PAH according to the manufacturer's instructions.Insulin resistance was calculated by the homeostasis model of assessment for insulin resistance (HOMA-IR) approach,calculated as fasting insulin (microunits/ml)× fasting blood glucose (mmol/L)/22.5.Comparisons between the parameters of patients with PAH and those of patients with normal PAP were performed with an unpaired Student's t test.The relationships between ghrelin and various clinical parameters were examined by bivariate correlations and multiple regression analysis.Results We found that the fasting plasma ghrelin level and the ratio of ghrelin to obestatin were significantly lower in the PAH group compared with the control group ((582.4±12.8) pg/ml vs.(1045.2±95.5) pg/ml,P <0.05 and 30.5±4.9 vs.70.0±9.7,P <0.01).The fasting plasma obestatin level was higher in the PAH group compared with the control group,but the difference between them was not significant ((23.2±3.1) pg/ml vs.(16.3±1.6) pg/ml,P >0.05).In a multiple regression model analysis,only mean PAP was an independent predictor of ghrelin and the ratio of ghrelin to obestatin (standardized coefficient=0.737,P <0.001 and standardized coefficient=-0.588,P=0.006,respectively).Conclusion Ghrelin is negatively correlated with mean PAP and this suggests that circulating ghrelin might predict the severity of pulmonary hypertension in ASD patients with PAH.

  18. Percutaneous closure of secundum type atrial septal defects:More than 5-year follow-up

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    Roel; JR; Snijder; Maarten; J; Suttorp; Jurri?n; M; Ten; Berg; Martijn; C; Post

    2015-01-01

    AIM: To investigate long-term efficacy of two different devices more than five years after percutaneous atrial septal defect(ASD) closure in adults.METHODS: All patients who underwent percutaneous closure of an ASD in the St. Antonius Hospital, Nieuwegein, The Netherlands, between February 1998 and December 2006 were included. Percutaneous closure took place under general anaesthesia and transesophageal echocardiographic moni toring. Transthoracic echocardiography(TTE) was performed 24 h post-procedure to visualize the device position and to look for residual shunting using color Doppler. All complications were registered. All patients were invited for an outpatient visit and contrast TTE more than 5-years after closure. Efficacy was based on the presence of a residual right-to-left shunt(RLS), graded as minimal, moderate or severe. The presence of a residual left-to-right shunt(LRS) was diagnosed using color Doppler, and was not graded. Descriptive statistics were used for patients’ characteristics. Univariate analysis was used to identify predictors for residual shunting.RESULTS: In total, 104 patients(mean age 45.5 ± 17.1 years) underwent percutaneous ASD closure using an Amplatzer device(ASO) in 76 patients and a Cardioseal/Starflex device(CS/SF) in 28 patients. The mean follow-up was 6.4 ± 3.4 years. Device migration occurred in 4 patients of whom two cases occurred during the index hospitalization(1 ASO, 1 CS/SF). The other 2 cases of device migration occurred during the first 6 mo of follow-up(2 CS/SF). The recurrent thrombo-embolic event rate was similar in both groups: 0.4% per follow-up year. More than 12 mo post-ASD closure and latest follow-up, new-onset supraventricular tachyarrhythmia’s occurred in 3.9% and 0% for the ASO and CS/SF group, respectively. The RLS rate at latest follow-up was 17.4%(minimal 10.9%, moderate 2.2%, severe 4.3%) and 45.5%(minimal 27.3%, moderate 18.2%, severe 0%) for the ASO- and CS/SF groups, respectively. There was no

  19. 经静脉闭合房间隔缺损的疗效观察%Transcatheter occlusion of secundum atrial septal defect with the Amplatzer septal occluder

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    秦永文; 丁继军; 等

    2001-01-01

    Objective:To evaluate the efficiency of transvenous oclusion of secundum atrial septal defect (ASD) using Amplatzer occluder.Method:There were 24 patients ( 6 male and 18 female, mean age 26.6 years). Echocardiography confirmed the ASD with a diameter of (18.96±7.03)mm (ranging from 6 to 30 mm). All patients with ASD were treated using Amplatzer occluder under guiding fluoroscopy and echocardiography through the percutaneous procedure.Result:The mean balloon stretched diameter of the ASDs in 24 patients was 15~36(26.25±7.57)mm.The mean diameter of the occluder selected was 14~36(26.52±7.15)mm.The successful rate of placement of the Amplatzer occluder was 100%. No complication was found during the procedures and during follow up. Trivial residual shunt was found in 7 patients immediately after the procedures, and there was no residual shunt 1 week after the procedures and during follow-up.Conclusion:Transvenous closure of secundum ASD using Amplatzer septal occluder is an efficient nonsurgical method.%目的:评价经静脉置入Amplatzer封堵器治疗继发孔型房间隔缺损(ASD)的疗效。方法:24例患者术前经多普勒超声心动图检查ASD直径为6~30(18.96±7.03)mm。在透视及经超声心动图引导下经静脉置入Amplatzer封堵器闭合ASD。 结果:24例ASD直径的球囊测量值为15~36(26.25±7.57)mm,选择的封堵器直径为14~36(26.52±7.15)mm。24例封堵器置入均获得成功 ,术中无并发症, 1例在术后第3天出现Ⅱ度Ⅰ型房室传导阻滞,2周后恢复 。7例术后即刻超声检查显示微量残余分流,术后1周复查均无分流。结论:经静脉置入Amplatzer封堵器治疗ASD是一种有效的非外科手术方法 。

  20. Transcatheter Closure of Atrial Septal Defect with Amplatzer Device in Children and Adolescents: Short and Midterm Results; an Iranian Experience

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    Saiyed-Habibollah Hosseini

    2011-06-01

    Full Text Available Objective:Transcatheter closure of secundum atrial septal defect (ASD with Amplatzer device is an alternative procedure to surgical repair, with some limitations. The aim of this study was to assess the initial and mid-term results of the treatment of ASD with Amplatzer septal occluder in children and adolescents. Methods:From May 2003 to January 2008 sixty three consecutive children and adolescent patients underwent transcatheter closure of ASD at a mean±SD age of 8.5±4.8 years (range 2.2 to 18 years. All procedures were performed under local anesthesia and moderate sedation or general anesthesia with transthoracic echocardiography and fluoroscopic guidance. Stretch diameter of ASD was determined by balloon sizing catheter. Device selection was based on and matched to the standard diameter of the septal defect. Follow up at 24 hours, 1 month, 6 months, 12 months and yearly thereafter included physical examination, electrocardiography and transthoracic echocardiography. Findings:The mean ASD diameter, measured with transthoracic echocardiography and balloon catheter were 19.5± 5.5 mm and 20.9± 6.2 mm, respectively. The mean follow up period was 32.4±18.8 months. Deployment of the device was successful in 57 (90.5% and failed in 6 (9.5% patients. The major complication included dislodgement of device in 1 patient and device embolization to right ventricular inlet (surgically removed in 1 patient. The minor complication included transient atrial tachycardia in 10 patients, paroxysmal supraventricular tachycardia in 2 patients during procedure, successfully terminated with medication. At 24 hour, 1 month, 6 month and 1 year follow up, total occlusion rates were 73.6 %, 91%, 94.7%, and 94.7%, respectively. Conclusion:Transcatheter occlusion of ASD with Amplatzer device is an effective and safe procedure with minimal complication rate and short hospital stay, as well as excellent short and intermediate outcome in children and adolescents.

  1. 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.

  2. Three-dimensional transesophageal echocardiography with agitated saline injection to differentiate between atrial septal defects and echo drop-out artifacts.

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    Alherbish, Aws; Shanks, Miriam; Choy, Jonathan

    2014-12-01

    The diagnosis of multiple atrial septal defects is less challenging with 3-D transesophageal echocardiography. However, the common occurrence of echo drop-out (acoustic shadow) artifacts with 3-D echocardiography can make the differentiation between a second defect and an artifact challenging. Agitated saline injection with direct visualization using 3-D echocardiography can help resolve this by allowing visualization of the bubbles crossing from true defects.

  3. Sinus Venosus Atrial Septal Defect as a Cause of Palpitations and Dyspnea in an Adult: A Diagnostic Imaging Challenge

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    Michael S. Donovan

    2015-01-01

    Full Text Available Sinus venosus atrial septal defects (SV-ASD have nonspecific clinical presentations and represent a diagnostic imaging challenge. Transthoracic echocardiography (TTE remains the initial diagnostic imaging modality. However, detection rates have been as low as 12%. Transesophageal echocardiography (TEE improves diagnostic accuracy though it may not detect commonly associated partial anomalous pulmonary venous return (PAPVR. Cardiac magnetic resonance (CMR imaging provides a noninvasive, highly sensitive and specific imaging modality of SV-ASD. We describe a case of an adult male with exercise-induced, paroxysmal supraventricular tachycardia who presented with palpitations and dyspnea. Despite nondiagnostic imaging results on TTE, CMR proved to be instrumental in visualizing a hemodynamically significant SV-ASD with PAPVR that ultimately led to surgical correction.

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

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    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.

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

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    Saatchi Mahesh Kuwelker

    2017-01-01

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

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

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    Kuwelker, Saatchi Mahesh; Shetty, Devi Prasad; Dalvi, Bharat

    2017-01-01

    Tricuspid valve (TV) injury following transcatheter closure of perimembranous ventricular septal defect (PMVSD) with Amplatzer ductal occluder I (ADO I), requiring surgical repair, is rare. We report two cases of TV tear involving the anterior and septal leaflets following PMVSD closure using ADO I. In both the patients, the subvalvular apparatus remained unaffected. The patients presented with severe tricuspid regurgitation (TR) 6 weeks and 3 months following the device closure. They underwent surgical repair with patch augmentation of the TV leaflets. Postoperatively, both are asymptomatic with a mild residual TR. PMID:28163430

  7. RARE ASSOCIATION OF POSTERIOR EMBRYOTOXON WITH MAXILLARY HYPOPLASIA, VENTRICULAR SEPTAL DEFECT, PULMONARY ATRESIA AND PATENT DUCTUS ARTERIOSUS

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    Pandey

    2014-12-01

    Full Text Available BACKGROUND: Posterior embryotoxon is a congenital anomaly, considered to be a relatively mild disorder and can occur in 15% of normal eyes. Bilateral posterior embryotoxon associated with maxillary hypoplasia, Ventricular Septal Defect (VSD, Pulmonary Atresia (PA with Patent Ductus Arteriosus (PDA is of rare occurrence and hasn’t been reported in literature till date. CASE: We report a case of 12 year old female who came to us in eye Out Patient Department for routine eye checkup and on detailed ocular examination we found anteriorly displaced Schwalbe’s line. On detailed physical examination and investigations she was found to have pan-systolic murmur and continuous machinery murmur. On Echocardiography, the patient was found to have large peri-membranous VSD with PA and PDA. On Oro-dental examination she was found to have maxillary hypoplasia. CONCLUSION: The present case is reported due to the rarity and sporadic character of the condition and its rare association with cardiac defect and maxillary hypoplasia.

  8. A novel two-stage complete repair method for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

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    MEI Ju; DING Fang-bao; ZHU Jia-quan; BAO Chun-rong; XIE Xiao; ZHANG Yun-jiao

    2010-01-01

    Background Pulmonary atresia with ventricular septal defect (PA-VSD) and major aortopulmonary collateral arteries (MAPCAs) remains a challenging complex congenital heart disease nowadays. In the present study, we aimed to develop a two-stage surgical method and to evaluate outcomes of this method in managing PA-VSD and MAPCAs.Methods Between December 2003 and December 2008, 7 female and 4 male patients between the age of 5 and 10 years who were suffering from PA-VSD and MAPCAs were selected and recruited. The native pulmonary artery trunks were absent in all patients; the lungs were solely supplied by major aortopulmonary collaterals, and the numbers of supplied lung segments ranged from 15 to 20 (17.9±1.6). There were a total of 43 MAPCAs in all the patients (3-5 (3.9±0.7) MAPCAs per patient). The accumulated Nakata index was (222.9±29.9) mm~2/m~2 (ranged from 182 to 272). All the patients underwent two sequential operations. Stage one included left major aortopulmonary collateral unifocalization and modified Blalock-Taussig shunt from left posterior lateral thoracotomy; stage two comprised right unifocalization, ligation of the shunt, followed by ventricular septal defect closure and right ventricular outflow tract reconstruction assisted with cardiopulmonary bypass from midline stemotomy.Results All the patients survived the initial surgery, but one of them died of low cardiac output syndrome on the third day after the second operation. Postoperative complications included pneumonia in one case and capillary leak syndrome in another. Postoperative oxygen saturation maintained about 95%-100%, which was significantly higher than pre-operation (P <0.01). During the follow-up period of 3-51 (25.4±15.2) months, there were no late death and no need for re-intervention. All the patients enjoyed their lives with good conditions.Conclusions This two-stage complete repair strategy was well-tolerated and effective with good outcome, thus offering an alternative

  9. Association of coronary to left ventricular microfistulae (vessels of Wearn) with atrial septal defect in an adult without cyanotic heart disease.

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    Hussain, Munem; Roberts, Elved Bryn

    2015-07-02

    Vessels of Wearn are rare findings during coronary angiography in adults. They are known to be associated with forms of cyanotic congenital heart disease in infants but we are not aware of any published cases of association with non-cyanotic left to right shunts in adults. We present the case of a 69-year-old man with angiographically evident vessels of Wearn draining from the left and right coronary arteries into the left ventricle associated with an asymptomatic atrial septal defect. We postulate a developmental phase association between atrial septal maturation and closure of perfusing microchannels from the ventricular cavities to the epicardial coronary arteries on the same spectrum as that which leads to more widespread defects in infants. We also highlight a common medication side effect that might have been mistaken as a manifestation of the congenital anomalies.

  10. 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.

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

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    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.

  12. Pulmonary Valve Infective Endocarditis in an Adult Patient with Severe Congenital Pulmonary Stenosis and Ostium Secundum Atrial Septal Defect

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    Juan Lacalzada

    2010-01-01

    Full Text Available A hypertensive 76-year-old man with severe pulmonary valve stenosis (PVS and recent initiation of haemodialysis was referred with fever, chills, and asthenia. One month prior, he had been admitted with similar symptoms. Transthoracic echocardiography (TTE had shown a PVS and no valve vegetations were observed. Following discharge, he was readmitted with fever and blood cultures positive for Staphylococcus haemolyticus. A new TTE revealed two pulmonary valve vegetations and a previously undetected ostium secundum-type atrial septal defect (ASD, confirmed by transesophageal echocardiography. The clinical course was uneventful with intravenous antibiotic treatment and the patient was safely discharged. This is a case of pulmonary valve infective endocarditis (IE. The incidence of right-sided IE is on the rise due to the increased number of patients using central venous lines, pacing, haemodialysis and other intravascular devices. Pulmonary valve IE is extremely rare, especially in structurally normal hearts. The case reported here, presents a combination of predisposing factors, such as severe congenital PVS, the presence of a central venous catheter, and haemodialysis. The fact that it was an older patient with severe congenital PVS and associated with a previously undiagnosed ASD, is also an unusual feature of this case, making it even more interesting.

  13. Surgical closure of apical multiple muscular septal defects via right ventriculotomy using a single patch with intermediate fixings

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    LI Gang; WANG Qiang; LIU Ying-long; XU Yu-lin; GUO Yan

    2013-01-01

    Background Multiple apical muscular ventricular septal defects (VSDs) remain a challenge for surgeons because of their anatomical features.We used single patch with intermediate fixations to repair multiple apical muscular VSDs through right ventriculotomy.Methods We analysed the data of 16 children (median age 8 months,range 2 months to 144 months) with multiple apical muscular VSDs who underwent a single patch technique via apical right ventriculotomy.Perioperative data were collected and analysed,and the patients were followed up for three months to 66 months (median,46 months) to investigate the outcomes.Results All patients recovered from cardiopulmonary bypass easily with median of cardiopulmonary bypass time 87 minutes and of aortic crossclamp time 53 minutes.No surgically related death occurred and no patient required reoperation.One patient died of pseudomonas pyocyanea infection on day 11 postoperatively.Residual shunt happened in one patient with a diameter of 2 mm and spontaneously closed in 12 months after operation.Two patients presented slightly reduced right ventricular volume and apical hypokinesia postoperatively and recovered 24 and 36 months later respectively.No other complication was found during the three months to 66 months follow-up.Conclusion Our experiences using a single patch technique with intermediate fixations via right ventriculotomy in the management of multiple muscular VSDs had favourable outcomes.

  14. Acute left ventricular failure after transcatheter closure of a secundum atrial septal defect in a patient with hypertrophic cardiomyopathy

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    ZHANG Cao-jin; HUANG Yi-gao; HUANG Xin-sheng

    2011-01-01

    We report a case of acute left ventricular failure at one hour after transcatheter closure of a secundum atrial septal defect (ASD) in a 28-year-old man with hypertrophic cardiomyopathy. Afforded noninvasive mechanical ventilation and the administration of intravenous morphine and high doses of furosemide, the patient exhibited improvement of his clinical condition, redtion of pulmonary congestion at chest X-ray, and satisfactory blood gas analyses in twelve hours. Twentyfour hours later, the patient received oral administration of furosemide and metoprolol. After 7 days the patient was discharged in good clinical condition. At follow-up at 12 months, the patient had remained symptomatically improved from NYHA Class Ⅲ symptoms before the procedure to Class Ⅱ symptoms. There was no latent arrhythmia at the follow-up examination. Follow-up transthoracic echocardiography estimated an improvement of the left ventricular function. So,transcatheter closure of a secundum ASD in a patient with hypertrophic cardiomyopathy is feasible, and a thorough understanding of the hemodynamic condition of ASD and hypertrophic cardiomyopathy will reduce the complication of ASD closure.

  15. Ventricular Septal Defect: a Literature Review Comunicación interventricular: revisión de la literatura

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    Luis Ángel Osorio Góngora

    2011-12-01

    Full Text Available Congenital heart defects occur in approximately eight of every 1000 live births. Ventricular septal defect occupies a large percentage of them and is the most commonly associated with chromosomal syndromes presenting multifactorial etiology. In 1879, Roger was the first to define the underlying clinical signs. New milestones in its history are marked by Einsenmenger, Dalrymple, Heath and Edwards. In 1979, the use of two-dimensional echocardiography in real time, dramatically improved non-invasive anatomical diagnosis. The main pathophysiologic alteration is part of the injuries that produce volume overload and left to right short-circuit. It is a model for the study of anatomical and physiological relationship in the different injuries that allow communication between the pulmonary and systemic circuits. The imperfections of the sites of origin of the interventricular septum result in different types of ventricular septal defects. The most commonly used classification is the anatomical, from Soto. Homodynamic effects after birth depend on the physiological changes of systemic and pulmonary circulation, as well as on the diameter of the hole. The clinical case depends on the defect and the pulmonary and vascular resistance. The diagnosis is clinical and echocardiographic. Treatment is medical, palliative, surgical and with rehabilitation. Currently, rehabilitation is approached as an essential part of care and therapy.Las cardiopatías congénitas se presentan aproximadamente en 8 de cada 1000 recién nacidos vivos. La comunicación interventricular ocupa un gran porcentaje entre todas, y es la que más se asocia a los síndromes cromosómicos con etiología multifactorial. En 1879 Roger fue el primero en definir los signos clínicos subyacentes. Nuevos hitos en su historia marcan: Einsenmenger, Dalrymple, Heath y Edwards, hasta 1979 en que, con el uso de la ecocardiografía bidimensional en tiempo real, se mejora dramáticamente el diagn

  16. Utility of balloon assisted technique in trans catheter closure of very large (≥35 mm) atrial septal defects

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    Rangaswamy Balasubramanian, Vidhyakar; Selvaraj, Raja; Saktheeswaran, Maheshkumar; Satheesh, Santhosh; Jayaraman, Balachander

    2014-01-01

    Background Very few published data is available on the outcomes of balloon assisted techniques (BATs) for trans catheter closure (TCC) of very large (Defined as ≥35 mm size) ostium secundum atrial septal defect (ASD). Objective To study the utility of BAT as against conventional techniques (CT) in TCC of very large ostium secundum ASD (≥35 mm) over the past 5-year period and to find out the association of different morphological features of the defects in relation to TCC outcomes. Study design and methods Descriptive single center retrospective study of patients with very large ostium secundum ASD (≥35 mm size) who were subjected to TCC. Results Thirty-three out of 36 patients with ≥35 mm ASD and complex morphological features underwent successful TCC. The study patients had high prevalence of absent aortic and posterior rims with posterior mal-alignment of the septum. BAT was successful in 28/31 (90.3%) patients while CT had a success rate of 16%. The mean trans-esophageal echocardiography (TEE) ASD size with BAT success 37 (SD 1.3) mm and CT failure 36.2 (SD 1.1) mm was not different (P=0.06). On univariate analysis of different morphological features, posterior mal alignment of the septum was associated failure of CT (P=0.01). There was no urgent referral for surgery and patients did well on follow up. Conclusions Balloon assisted device closure of (≥35 mm) ASD had 90% success rate. BAT helps in controlled delivery and device alignment in very large ASD with posterior malalignment of the septum and is often helpful when CT fails. PMID:24649421

  17. 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

  18. Clarifying the morphology of the ostium primum defect.

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    Anderson, Robert H; Mohun, Timothy J; Brown, Nigel A

    2015-03-01

    The 'ostium primum' defect is still frequently considered to be the consequence of deficient atrial septation, although the key feature is a common atrioventricular junction. The bridging leaflets of the common atrioventricular valve, which are joined to each other, are depressed distal to the atrioventricular junction, and fused to the crest of the muscular ventricular septum, which is bowed in the concave direction towards the ventricular apex. As a result, shunting across the defect occurs between the atrial chambers. These observations suggest that the basic deficiency in the 'ostium primum' defect is best understood as a product of defective atrioventricular septation, rather than an atrial septal defect. We have now encountered four examples of 'ostium primum' defects in mouse embryos that support this view. These were identified from a large number of mouse embryo hearts collected from a normal, outbred mouse colony and analysed by episcopic microscopy as part of an ongoing study of normal mouse cardiac development. The abnormal hearts were identified from embryos collected at embryonic days 15.5, 16.5 and 18.5 (two cases). We have analysed the features of the abnormal hearts, and compared the findings with those obtained in the large number of normally developed embryos. Our data show that the key feature of normal atrioventricular septation is the ventral growth through the right pulmonary ridge of a protrusion from the dorsal pharyngeal mesenchyme, confirming previous findings. This protrusion, known as the vestibular spine, or the dorsal mesenchymal protrusion, reinforces the closure of the primary atrial foramen, and muscularises along with the mesenchymal cap of the primary atrial septum to form the ventro-caudal buttress of the oval foramen, identified by some as the 'canal septum'. Detailed analysis of the four abnormal hearts suggests that in each case there has been failure of growth of the vestibular spine, with the result that the common

  19. Transcatheter Closure of Atrial Septal Defects Improves Cardiac Remodeling and Function of Adult Patients with Permanent Atrial Fibrillation

    Institute of Scientific and Technical Information of China (English)

    Liang Chen; Yuan Bai; Fei-Yu Wang; Zhi-Gang Zhang; Xing-Hua Shan; Tao Chen; Xian-Xian Zhao

    2015-01-01

    Background:Permanent atrial fibrillation (AF) is the most common form of dysrhythmia associated with atrial septal defects (ASDs) in patients older than 40 years.However,little is known about cardiac remodeling after transcatheter closure in patients with permanent AF.This study was designed to compare cardiac events and remodeling effects after transcatheter closure in such patients.Methods:Clinical data of 289 adult patients older than 40 years who underwent ASD closure at our center were analyzed retrospectively.Of them,63 patients with permanent AF were assigned to the case group,and the other 226 patients without permanent AF were assigned to the control group.Cardiac events and changes in left and right cardiac cavity dimensions before the procedure and 6 months after the procedure were compared between the two groups.Results:Patients in the case group were significantly older than those in the control group.The right ventricular (RV) volume and right atrial (RA) volume were decreased significantly in both the groups during a median follow-up period of 6 months after closure (P < 0.001).The left atrial dimensions,left ventricular end-systolic dimensions,left ventricular end-diastolic dimensions and left ventricular ejection fraction showed no significant change before and after the procedure in both the groups.Changes of the RV volume and RA volume in the case group were significantly smaller than those in the control group (P =0.005 and P < 0.001).The New York Heart Association cardiac function was improved in both the groups during the 6 months follow-up period.Conclusions:The transcatheter closure of ASD can improve the cardiac remodeling and cardiac function in patients with or without AF.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2013-04-01

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

  1. Transcatheter Closure of Atrial Septal Defects Improves Cardiac Remodeling and Function of Adult Patients with Permanent Atrial Fibrillation

    Directory of Open Access Journals (Sweden)

    Liang Chen

    2015-01-01

    Full Text Available Background: Permanent atrial fibrillation (AF is the most common form of dysrhythmia associated with atrial septal defects (ASDs in patients older than 40 years. However, little is known about cardiac remodeling after transcatheter closure in patients with permanent AF. This study was designed to compare cardiac events and remodeling effects after transcatheter closure in such patients. Methods: Clinical data of 289 adult patients older than 40 years who underwent ASD closure at our center were analyzed retrospectively. Of them, 63 patients with permanent AF were assigned to the case group, and the other 226 patients without permanent AF were assigned to the control group. Cardiac events and changes in left and right cardiac cavity dimensions before the procedure and 6 months after the procedure were compared between the two groups. Results: Patients in the case group were significantly older than those in the control group. The right ventricular (RV volume and right atrial (RA volume were decreased significantly in both the groups during a median follow-up period of 6 months after closure (P < 0.001. The left atrial dimensions, left ventricular end-systolic dimensions, left ventricular end-diastolic dimensions and left ventricular ejection fraction showed no significant change before and after the procedure in both the groups. Changes of the RV volume and RA volume in the case group were significantly smaller than those in the control group (P = 0.005 and P < 0.001. The New York Heart Association cardiac function was improved in both the groups during the 6 months follow-up period. Conclusions: The transcatheter closure of ASD can improve the cardiac remodeling and cardiac function in patients with or without AF.

  2. 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

  3. Usefulness of myocardial performance index for assessing right ventricular function after percutaneous closure of atrial septal defect

    Institute of Scientific and Technical Information of China (English)

    Jingdong Ding; Genshan Ma; Yaoyao Huang; Xiaoli Zhang; Jian Zhu; Rong Yang; Fengxiang Lu

    2007-01-01

    Objective Assessment of right ventricular function in patients with atrial septal defect (ASD) is difficult. The Doppler myocardial performance index (MPI) may provide a method of assessing function in these patients. The purposes of this study were to evaluate the right ventricular function and its changes in patients with ASD after transcatheter closure of ASD. Methods MPI, defined as the sum of isovolumic relaxation time and isovolumic contraction time derived by ejection time, was measured from tricuspid inflow and right ventricular outflow; Doppler velocity profiles recorded during routine echocardiography. Twenty nine patients ( 13 men, 16 women;mean age 25.28 ± 12.69, range 6 to 57 years) were diagnosed to secundum ASD [the stretched diameters of ASD were from 9 To 36 (24.91 ±7.98) mm], and had a successfully placed Amplatzer septal occluder (ASO) (the sizes of ASO were from 11 to 40 mm ); there were 81 sex-matched, age-matched healthy people (control group 41men, 40 women; mean age 29.02 ± 14.22, range 4 to 45 years).MPI was measured again on 3 days and 1 month after closure of ASD. Change in the study group was assessed and compared to the control subjects with structurally normal hearts. A complete 2- dimensional and Doppler echocardiographic examination was performed in all study groups. Results 1) The isovolumic relaxation and isovolumic contraction times [respectively(77.59 ± 14.39)ms vs (60.93 ± 12.94)ms, P<0.0001; (28.28 ± 10.88)ms vs (23.64 ± 9.01)ms, P=0.027] were prolonged, and ejection time [(260.65 ± 21.86 )ms vs (271.85 ± 21.92)ms, P=0.033] was shortened in patients with ASD compared with that in control subjects, resulting in a marked increase in the MPI(0.40 ± 0.07 vs 0.31 ± 0.05, P<0.0001) from normal values; 2) by Pearson's correlations, the MPI had no correlation with heart rate and blood pressure in control subjects and patients with ASD, but it correlated positively with age in patients with ASD; 3) by Pearson

  4. Comunicação interatrial e hipertensão pulmonar em atleta profissional de futebol Atrial septal defect and pulmonary hypertension in professional soccer player

    Directory of Open Access Journals (Sweden)

    Fernando Bianchini Cardoso

    2010-08-01

    Full Text Available Relatamos um caso raro de diagnóstico tardio de comunicação interatrial (CIA com hipertensão pulmonar (HP, através da ecocardiografia Doppler bidimensional (ECO, em atleta profissional de futebol que após correção cirúrgica retornou ao esporte competitivo de alto rendimento.This study reports a rare case of late diagnosis of atrial septal defect (ASD with pulmonary hypertension (PH through two-dimensional echocardiography (ECHO in a professional soccer player who resumed high-performance sports activity after surgery.

  5. 婴幼儿室间隔缺损并肺动脉高压的外科治疗%Surgical treatment for ventricular septal defect infants with pulmonary hypertension

    Institute of Scientific and Technical Information of China (English)

    吕瑛; 李小兵; 张会军; 闫芳; 孙丽颖; 李志杰; 申红; 黄建成; 李红英

    2013-01-01

    Objective To evaluate the efficiency of urgent surgical correction for ventricular septal defect (VSD) infants with pulmonary hypertension(PH).Methods Two hundred and fifteen infants (109 male,106 female)with VSD and PH received surgical correction from Department of Cardiac Surgery,Center of Congenital Heart Disease,the First Hospital of Hebei Medical University.The age of patients ranged from 1 to 36 months on admission,the body mass ranged from 1.5 to 10.0 kg.Among all the cases,the perimembranous type was found in 156 children,the conal septal defect in 18 cases,and subpulmonary VSD in 41 cases.The diameter of defect was 0.8-2.1 cm.Sixteen patients were associated with atrial septal defect (ASD),8 cases with patent foramen ovale,15 cases with ductus arteriosus (PDA),14 cases with ASD and PDA,and 3 cases with PDA and coarctation of the aorta,14 cases with pulmonary valve stenosis,33 cases with stricture of right ventricular outflow tract,22 cases with incompetence of mitral valve directly ligation,42 cases with incompetence of tritral valve directly ligation,and the rest 12 cases with persistent left superior vena cava.The pressure of pulmonary artery was 4.66-11.31 kPa.All patients received cardiopulmonary bypass under hypothermic anaesthesia through extracorporeal blood circulation.Results Urgent operations were performed in all patients,but 3 patients died after surgery.Postoperative complications included low cardiac output syndrome (15 cases),among whom 2 cases died ; right bundle-branch block (10 cases),supraventricular tachycardia (6 cases),transient atrioventricular block of grade Ⅲ (5 cases),inhalation of nitric oxide was given to 5 cases for the management of significant postoperative PH,among whom 1 case died during surgery,3 cases were pneumothorax,2 cases were atelectasis.Chest radiography,echocardiography,and ECG were performed during this period.As a result,most children had good cardiac functions.Conclusions Low cardiac output syndrome and

  6. Ventricular septal defect

    Science.gov (United States)

    ... Hard breathing Paleness Failure to gain weight Fast heart rate Sweating while feeding Frequent respiratory infections ... looks to see if there is a large heart with fluid in the lungs ECG : shows signs of an enlarged left ventricle Echocardiogram : ...

  7. Percutaneous closure of huge patent ductus arterious associated with anomalous inferior vein cava drainage and dextrocardia with muscular ventricular septal defect occluder

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    @@ Transcatheter occlusion of patent ductus arterious (PDA) using various occluders and coils has been a well-established method1-5 since Porstmann and colleagues6 reported the first case in 1967. However, when patients associated with anomalous inferior vein cava drainage or/and huge high pulmonary artery pressure ductus (HPAP-PDA), the method is not suitable. First, it is unfeasible to carry out the procedure via femoral vein. Second, in the presence of high pulmonary artery pressure such devices including the Amplatzer ductu occluder carry the risk of embolising into the aorta.7 The muscular ventricular septal defect occluder (MVSDO), which is a device for transcatheter closure of muscular ventricular septal defect, may be more suitable for using with HPAP-PDA as its double disk tends to anchor the device, preventing embolisation into the aorta. We present a patient, who is suffering from huge PDA associated with anomalous inferior vein cava drainage and dextrocardia, in whom percutaneous closure of PDA using MVSDO was successfully accomplished via transjugular approach.

  8. Iatrogenic erosion of the septum primum resulting in an atrial septal defect with left-to-right shunt: a rare pitfall of patent foramen ovale percutaneous closure.

    Science.gov (United States)

    Scacciatella, Paolo; Biava, Lorenza Michela; Marra, Sebastiano

    2014-09-01

    Iatrogenic erosion of the septum primum after foramen ovale closure is an anecdotal event. We report the case of a 39-year-old woman admitted to our institution for multifocal cryptogenic cerebral ischemia and a patent foramen ovale (PFO) associated with an aneurysm of the septum primum. The patient underwent percutaneous closure of the PFO with an Amplatzer PFO Occluder device. At the 6-months follow up, the device was in the right position, but a jagged defect of the septum primum and evidence of significant left-to-right shunting was detected. The atrial septal defect was then repaired by a surgical approach. Although this event is not life-threatening, it should be considered as a therapeutic pitfall, resulting in a risk of paradoxical embolism recurrences and long-term hemodynamic impairment. © 2013 Wiley Periodicals, Inc.

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

    Directory of Open Access Journals (Sweden)

    Fábio Alves Almeida

    1998-10-01

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

  10. [Results of surgical therapy of the partial form of persistent common atrioventricular canal (author's transl)].

    Science.gov (United States)

    Morino, F; Possati, F; Calafiore, A M; Santarelli, P; Malara, D; Ottino, G M; de Nunno, T

    1975-01-01

    20 patients with partial form of persistent common atrioventricular canal underwent surgery at the Centro di Cardiochirurgia "A. Blalock", University of Turin, between 1969 and 1974. In each case the treatment of lesions took place in the following order: the repair or replacement (one case) of the mitral valve; the closing with a patch of the atrial septal defect; the repair, in one case, of the tricuspid. The most frequent post-operative complications were arrhythmias, which in two cases consisted of a total atrioventricular block. Only one patient died postoperatively for an acute pulmonary oedema following an imperfect mitral valve correction. Six months after surgery, another patient died from bacterial endocarditis. Thirteen of the eighteen surviving patients showed excellent results at the clinical and/or hemodynamic check; two had fair results and three had poor results. In two cases, mitral insufficiency was hemodynamically aggravated, whilst in another five it disappeared. In eight, a slight apex systolic murmor 1-2/6 persisted, which was not correlated with any radiological or electrocardiographical sign of mitral insufficiency (and for three of these, not even a hemodynamic one). The authors consider that the most difficult problem in surgical therapy for these malformations is still the treatment of mitral lesions.

  11. Amplatzer封堵器治疗儿童伴有假性室隔瘤的膜周部室间隔缺损%Transcatheter closure of aneurysm of perimembrane ventricular septal defect with Amplatzer duct occluder in children

    Institute of Scientific and Technical Information of China (English)

    唐礼江; 高伟; 方崇峰; 林仙方; 江建军; 刘志国

    2004-01-01

    我们应用Amplatzer不对称的膜周部室间隔缺损封堵器介入治疗伴有假性室隔瘤的膜周部室间隔缺损(Aneurysm of perimembrane ventricular septal defect)28例,现报告如下。

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

    Directory of Open Access Journals (Sweden)

    Alejandro E. Contreras

    2011-08-01

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

  13. A Rare Case of Pulmonary Atresia with Ventricular Septal Defect with a Right Sided Aortic Arch and a Calcified Pulmonary AVM Presenting in an Adult without Cyanosis

    Directory of Open Access Journals (Sweden)

    Devendra V. Kulkarni

    2014-01-01

    Full Text Available Pulmonary atresia with ventricular septal defect (PA-VSD with pulmonary arterial supply arising from the aorta representing large MAPCAs associated with a right sided aortic arch is an uncommon anomaly. Most of the patients succumb to severe respiratory compromise or congestive cardiac failure very early. We report the clinical details and imaging findings of a case of PA-VSD with a right sided aortic arch and a calcified pulmonary arteriovenous malformation (AVM in a 21-year-old postpartum female with no previous episodes of cyanosis who was diagnosed as having a cardiac anomaly on echocardiography when she presented with breathlessness during the 8th month of the pregnancy.

  14. Mid-to-long-term follow-up results of transcatheter closure of atrial septal defect in patients older than 40 years.

    Science.gov (United States)

    Wang, Jou-Kou; Chiu, Shuenn-Nan; Lin, Ming-Tai; Chen, Chun-An; Lu, Chun-Wei; Wu, Mei-Hwan

    2016-08-16

    We investigated the mid-to-long-term results of transcatheter closure of atrial septal defect (ASD) in patients ≥40 years since there are issues with patients presenting with pulmonary hypertension and arrhythmia at the time of closure. In an 8.8 year period, transcatheter closure of ASD was successful in 179 patients aged ≥40 years, but failed in 2. Of the 179 patients (44 males, median 53 years), NYHA functional class, presence of arrhythmia and severity of pulmonary hypertension were compared before and after closure. Patients with pulmonary hypertension (n = 43, 24 %) were significantly older (60 ± 11 vs. 52 ± 9 years, p atrial fibrillation (AF), 4 atrial flutter and 5 supraventricular tachycardia. Patients with AF or atrial flutter (n = 26) were significantly older (63 ± 10 vs. 53 ± 10 years, p = 0.048) and had a higher pulmonary artery mean pressure (29.2 ± 12.6 vs. 20.2 ± 7.6 mmHg, p = 0.041) than those without. The mean follow-up period was 3.8 ± 2.1 years. Early new-onset arrhythmia was documented in 23 patients of whom 1 had persistent AF, 1 developed sick sinus syndrome and others were in sinus rhythm at latest visit. There was significant improvement in NYHA functional class after closure (p paroxysmal AF, and 11 had persistent AF. Pulmonary hypertension persisted in 13 patients. Transcatheter closure of atrial septal defect in patients above 40 years is beneficial in terms of NYHA functional class, pulmonary artery pressure and cardiac rhythm.

  15. Hypothermia During Cardiopulmonary Bypass Increases Need for Inotropic Support but Does Not Impact Inflammation in Children Undergoing Surgical Ventricular Septal Defect Closure.

    Science.gov (United States)

    Schmitt, Katharina Rose Luise; Fedarava, Katsiaryna; Justus, Georgia; Redlin, Mathias; Böttcher, Wolfgang; Delmo Walter, Eva Maria; Hetzer, Roland; Berger, Felix; Miera, Oliver

    2016-05-01

    Minimizing the systemic inflammatory response caused by cardiopulmonary bypass is a major concern. It has been suggested that the perfusion temperature affects the inflammatory response. The aim of this prospective study was to compare the effects of moderate hypothermia (32°C) and normothermia (36°C) during cardiopulmonary bypass on markers of the inflammatory response and clinical outcomes (time on ventilator) after surgical closure of ventricular septal defects. During surgical closure of ventricular septal defects under cardiopulmonary bypass, 20 children (median age 4.9 months, range 2.3-38 months; median weight 7.2 kg, range 5.2-11.7 kg) were randomized to a perfusion temperature of either 32°C (Group 1, n = 10) or 36°C (Group 2, n = 10). The clinical data and blood samples were collected before cardiopulmonary bypass, directly after aortic cross-clamp release, and 4 and 24 h after weaning from cardiopulmonary bypass. Time on ventilation as primary outcome did not differ between the two groups. Other clinical outcome parameters like fluid balance or length of stay in the intensive care were also similar in the two groups. Compared with Group 2, Group 1 needed significantly higher and longer inotropic support (P bypass temperature does not influence time on ventilation or inflammatory marker release. However, in the present study, with a small patient cohort, patients operated under hypothermic bypass needed higher and longer inotropic support. The use of hypothermic cardiopulmonary bypass in infants and children should be approached with care.

  16. Postoperative Residual Shunt After Repair of Ventricular Septal Defect Among Children%小儿室间隔缺损术后残余漏

    Institute of Scientific and Technical Information of China (English)

    蒋雄刚

    1995-01-01

    8例小儿室间隔缺损修补术后残余漏再次手术病例分析:残余漏表现为5例术后残留心前区Ⅲ级以上收缩期杂音,7例血红蛋白尿,2例右心衰,8例超声心动图(UCG)证实心室水平残余左向右分流.均于术后2~112天行残余漏修补.术中见残余漏原因为不同部位缝针处组织撕裂6例,修补不全2例.8例均经带垫片褥式缝合加固,7例痊愈,1例再次发生残余漏.本文对残余漏特点、诊断、手术适应证及时机进行了讨论.%Eight cases of postoperative residual shunt after repair of venticular septal defect (VSD) were analysed in this paper.Their clinical features disclosed:hemofiubinuria in 7 cases,cardiac murmur in 5 and residual shunt signs on echocardiograph in 8.In the operation for residual shunt,it was found:the tear in septal leaflet in aneurysm-like VSD in 2 cases,the tear in septal leaflet of tricuspid in 4,infundibular muscular 2 and residual triangle defect under the patch repair in VSD 2.Residual shunt in all the 8 cases were repaired with mattress suture under cardiopulmonary bypass,seven patients recovered without hemogiobinuria or cardiac murmur,but one had residual shunt again and cerebral insufficiency,which was improved by medical therapy.The harmfulness,the diagnosis and prevention of postoperative residual shunt,the operative indication,optimal time and surgical procedures were discussed.

  17. 间隔缺损性先天性心脏病胎儿脐动脉及大脑中动脉搏动指数的变化%Change of Umbilical Artery and Middle Cerebral Artery Pulsatility Index in Fetuses with Congenital Heart Diseases of Septal Defect

    Institute of Scientific and Technical Information of China (English)

    刘金蓉; 吕国荣; 吴秀明; 候敏

    2011-01-01

    Objective To evaluate the significance of change in umbilical artery(UA)and middle cerebral artery (MCA)pulsatility index(PI)in fetuses with congenital heart disease(CHD)of septal defect. Methods Umbilical artery (UA)and middle cerebral artery(MCA) pulsatility index(PI) were retrospectively analyzed in 82 fetuses with CHD, including 25 cases of atrioventricular septal defect(AVSD) ,51 cases of ventricular septal defect(VSD)and 6 cases of atrial septal defect(ASD). The control group comprised 660 healthy fetuses of uncomplicated pregnancies matched for gestational age. Individual PI measurements were converted into Z-scores for statistical analysis. Results Fetuses with AVSD、VSD and all CHD had an increased UA-PI compared to the controls respectively(P<0. 05), but no difference was observed in UA-PI between fetuses with ASD and the controls(P>0. 05). There was no significant difference in MCA-PI between fetuses with AVSD,VSD,ASD,all CHD and the controls(P>0. 05). Conclusions UA-PI in fetuses with AVSD and VSD may increase,however, MCA-PI does not change remarkably.%目的 探讨几种间隔缺损性的先天性心脏病胎儿的脐动脉搏动指数(UA-PI)、大脑中动脉搏动指数(MCA-PI)的变化.方法 回顾性分析82例间隔缺损性的先天性心脏病胎儿(疾病组)的脐动脉(UA)和大脑中动脉(MCA)的搏动指数(PI),其中包括25例房室隔缺损(又称心内膜垫缺损,AVSD组)、51例室间隔缺损(VSD组),6例房间隔缺损(ASD组);另随机选择660例按孕龄分组的正常的胎儿作为正常对照组.对所有PI值进行标准正态转换后再进行统计分析.结果 疾病组AVSD组,VSD组胎儿的UA-PI均比正常对照组胎儿高(P<0.05),而ASD组胎儿与正常对照组胎儿的UA-PI无明显差别(P>0.05);疾病组AVSD组、VSD组、ASD组胎儿与正常对照组胎儿的MCA-PI均无明显差别(P>0.05).结论 AVSD、VSD的胎儿在官内可能会出现UA-PI增高,而MCA-PI无明显改变.

  18. Interventional treatment of common congenital heart diseases the common view of Chinese medical experts.Part Two——Interventional treatment of ventricular septal defect%常见先天性心脏病介入治疗中国专家共识二、室间隔缺损介入治疗

    Institute of Scientific and Technical Information of China (English)

    中国医师协会心血管内科分会先心病工作委员会

    2011-01-01

    室间隔缺损(VSD)为最常见的先天性心脏畸形,占先天性心脏病的25% ~ 30%.介入治疗的关键在于适应证的选择,操作技巧和并发症的防治.根据左心室造影图像判断VSD的形态和类型,选择不同类型的封堵器.操作中要避免右房室瓣腱索的缠绕、瓣膜的损伤和心律紊乱的发生.术后应严密观察,及早处理房室传导阻滞等并发症.本文详细介绍了膜部和肌部VSD的诊断,规范化的操作方法,术中和术后可预测的情况处理.%Ventricular septal defect(VSD)is the most common congenital heart disease. it accounts for 25 ~ 30% of all congenital heart diseases. The key points of interventional treatment for VSD are the careful selection of indications. the rich experience in manipulating skill and the effective prevention of complications. According to the imaging f'indings on the selective left sided cardiography and echocardiography, the morphology and type of VSD, mainly including the precise size and location of the defect. can be accurately determined. on this account the proper transcatheter device closure can be rationally selected. During the interventional management, the twine and damage of the right atrio-ventricular valve as well as its tendinous cords should be avoided. and the ventricular arrhythmias and new aortic or tricuspid regurgitation should be prevented. After the treatment, the patient should be closely observed, the postoperative complications such as atrioventricular block should be promptly dealt with. In this chapter, the diagnosis of membranous and muscular VSD. the standard interventional procedure of transcatheter device closure implantation and the management of predictable events occurred during and after the procedure will be systemically described. (J Intervent Radiol, 2011 , 20 : 87-92)

  19. 供体先天性室间隔缺损心脏移植1例%Donor congenital ventricular septal defect heart transplantation in one case

    Institute of Scientific and Technical Information of China (English)

    仲崇俊; 王春生; 薛群; 陆晨希; 许一鸣; 高增栋

    2008-01-01

    本例心脏移植受者为终末期扩张性心肌病患者,供体为先天性室间隔缺损患者,进行原位双腔心脏移植,随访4年结果满意.在当前供体紧缺的情况下,对于一些存在先天性缺陷的供体,适当处理后应用也能取得满意的临床效果.%The receptor of the heart transplantation was a patient with terminal dilated cardiomyopathy, the donor was a patient with congenital ventricular septal defect, in situ double-chamber heart transplantation was performed, and the result of the four-year follow-up was satisfactory. At present, donor is deficient,and those donors with congenital defect can also obtain satisfactory clinical application effects after appropriate handling.

  20. Isolation, characterization and genetic analysis of canine GATA4 gene in a family of Doberman Pinschers with an atrial septal defect

    Indian Academy of Sciences (India)

    Shin-Aeh Lee; Seung-Gon Lee; Hyeong-Sun Moon; Lopeti Lavulo; Kyoung-Oh Cho; Changbaig Hyun

    2007-12-01

    GATA4 is expressed early in the developing heart where it plays a key role in regulating the expression of genes encoding myocardial contractile proteins. Gene mutations in the human GATA4 have been implicated in various congenital heart defects (CHD), including atrial septal defect (ASD). Although ASD is the third most common CHD in humans, it is generally rare in dogs and cats. There is also no obvious predilection for ASD in dogs and cats, based on sex or breed. However, among dogs, the incidence rate of ASD is relatively high in Samoyeds and Doberman Pinschers, where its inheritance and genetic aetiology are not well understood. In this study, we identified and investigated the genetic aetiology of an ASD affected family in a pure breed dog population. Although the GATA4 gene was screened, we did not find any mutations that would result in the alteration of the coding sequence and hence, the predicted GATA4 structure and function. Although the aetiology of ASD is multifactorial, our findings indicate that GATA4 may not be responsible for the ASD in the dogs used in this study. However, this does not eliminate GATA4 as a candidate for ASD in other dog breeds.

  1. 隔瓣后室间隔缺损并假性室隔瘤的手术治疗%Surgical Treatment of Ventricular Septal Defect With False Aneurysm of Membrane Septum

    Institute of Scientific and Technical Information of China (English)

    毛志福; 高尚志; 陈俊堂; 姚震; 涂仲凡

    1996-01-01

    Objective:To introduce the procedure for better exposure of the ventricular septal defect(VSD)from fused septal leaflet pouch.Method:Among the 28 cases,the septal leaflet of tricuspid valve in 22 cases was incised and the papillary muscle in 6 cases was dissected to expose the rim of the hidden VSD for proper anatomic repair.Results:There was no death,no significant intraoperative or postoperative complications and tricuspid valve dysfunction.Conclusion: Opening the fused septal leaflet pouch is safe and beneficial to a better exposure and repair of the hidden defect.%采用隔瓣切开或圆锥乳头肌切断术治疗假性室隔瘤型VSD并予以评价.全组共28例,其中22例采用隔瓣切开,6例采用圆锥乳头肌切断片显露缺损,再以涤纶片或自体心包片修补VSD.全组无手术死亡,无重大并发症及三尖瓣功能不全.此法安全,有利于VSD的显露和对VSD准确可靠的修补,值得推荐使用.

  2. Transcatheter closure with double Amplatzer septal occluder device in treatment of multiple atrial septal defects%双封堵器介入治疗多孔性房间隔缺损

    Institute of Scientific and Technical Information of China (English)

    孙鑫; 杨延坤; 郑宏; 徐争鸣; 徐仲英; 蒋世良; 赵世华; 张戈军; 吴文辉

    2013-01-01

    Objective To evaluate the clinical feasibility and effectiveness of transcatheter closure of multiple atrial septal defects (ASD) using double occluders.Methods Seventeen patients underwent transcatheter closure of multiple ASD (2 or 3) with double devices under X-ray fluoroscopy and TTE.Electrocardiogram,X-ray chest radiography as well as echocardigraphy were performed to evaluate the effectiveness after the procedure.Results All patients had multiple ASD (12 with 2 defects and 5 with 3 defects).TTE during the procedures showed that the mean diameters of the larger,medium and smaller defects was (13.94±3.21)mm,(9.65±2.64)mm and (4.80±1.92)mm,respectively.Tanscatheter closure of multiple ASD succeeded in 16 patiets (16/17,94.12 %),1 failed because the second defect was not suitable for intervention,and was cured by surgery,and totally 32 occluder devices were implanted and closed 37 defects.The mean diameters of the larger and the smaller occluders was (20.75 ± 4.07)mm and (16.94 ± 3.75)mm,respectively,larger than the value showed by TTE during procedures by (7.06±2.65)mm (larger defects) and (7.44±3.25)mm (medium defects).Immediately after the procedure,3 patients had trivial shunt (2 patients with 3 defects),which disappeared 2,3 days and 3 month later,respectively.No severe complication occurred,while femoral arteriovenous fistula was observed in 1 patient and disappeared 1 month later.The cardiothoracic ratio of X-ray chest radiography and the right ventricle internal diameter measured by echocardiography dropped during follow-up (all P<0.01).Conclusion Transcatheter closure of multiple ASD with double devices is feasible,safe and effective.%目的 对双封堵器介入治疗多孔性房间隔缺损(ASD)的可行性与有效性进行临床评估.方法 对17例多孔性ASD(12例为2孔,5例为3孔)患者在X线透视和超声心动图监测下植入Amplatzer封堵器,同时闭合2~3个缺损.术后复查ECG、X线平

  3. Usefulness of early diastolic mitral annular velocity to predict plasma levels of brain natriuretic peptide and transient heart failure development after device closure of atrial septal defect.

    Science.gov (United States)

    Masutani, Satoshi; Taketazu, Mio; Mihara, Chihiro; Mimura, Yuko; Ishido, Hirotaka; Matsunaga, Tamotsu; Kobayashi, Toshiki; Senzaki, Hideaki

    2009-12-15

    Device closure of atrial septal defect (ASD) is sometimes followed by elevation of plasma brain natriuretic peptide (BNP), a marker of heart failure, and progression to heart failure. This study tested the hypothesis that the underlying diastolic dysfunction, assessed on tissue Doppler images (TDI) before device closure, can predict BNP level after ASD closure. The study subjects were 39 consecutive patients (age 27.5 +/- 16.3 years, range 5 to 63) who underwent device closure for ASD. Echocardiographic evaluation using TDI and 2-dimensional and pulse wave Doppler were performed, together with plasma BNP measurement 1 day before and 2 days after ASD closure. Before ASD closure, an age-dependent decrease was noted in left ventricular relaxation, assessed by early diastolic mitral annular velocity. ASD closure resulted in a decrease in early diastolic mitral annular velocity (from 14.7 to 12.3 cm/s, p linear regression identified early diastolic mitral annular velocity before ASD closure and age as independent predictors of BNP levels after ASD closure (p annular velocity developed exertional dyspnea after the procedure. In conclusion, our results indicate that TDI measurements could be useful to detect underlying diastolic dysfunction that can potentially cause heart failure after ASD closure and emphasize the importance of ASD closure at a young age before impairment of left ventricular relaxation.

  4. Timing in resolution of left heart dilation according to the degree of mitral regurgitation in children with ventricular septal defect after surgical closure

    Directory of Open Access Journals (Sweden)

    Hwa Jin Cho

    2014-01-01

    Full Text Available OBJECTIVE: Children with ventricular septal defects (VSD can have chronic volume overload, which can result in changes of left heart echocardiographic parameters. To evaluate the changes before and after surgical closure, the children were divided into three groups according to the degree of mitral regurgitation (MR, and their echocardiographic characteristics were reviewed at serial follow-up after surgical closure. METHODS: The preoperative, and one-, three-, and 12-month postoperative echocardiographic data of 40 children who underwent surgical closure of VSD were retrospectively reviewed. Left ventricular end-diastolic volume (LVEDV, left ventricular end-diastolic dimension (LVEDD, left ventricular end-systolic dimension (LVESD, mitral valvular characteristics, including degree of MR and mitral valve annulus, and left atrial (LA characteristics, including volume and dimensions, were observed. RESULTS: Preoperative LVEDV, LVEDD, LVESD, mitral valvular annulus, LA volume, and LA dimensions were significantly larger in children with MR. Additionally, there were significant decreases in LVEDV, LVEDD, LA volume, and LA dimensions at one, three, and 12 months postoperatively. The degree of MR also improved to a lower grade after surgical closure of the VSD without additional mitral valve repair. CONCLUSION: The echocardiographic parameters of left heart dilation and MR in children with VSD improved within the first year after surgical closure without additional mitral valve repair. Furthermore, in all of the patients with VSD, regardless of MR, LA dilation was reduced within three months after surgical closure of the VSD; however, LV and mitral valve annular dilatation decreased within 12 months.

  5. Transcatheter closure of ventricular septal defect in patients with aortic valve prolapse and mild aortic regurgitation:feasibility and preliminary outcome

    Institute of Scientific and Technical Information of China (English)

    Guan-Liang Chen; Hai-Tao Li; Hai-Rong Li; Zhi-Wei Zhang

    2015-01-01

    Objective: To evaluate the feasibility, safety and efficacy of transcatheter closure of ventricular septal defect (VSD) in patients with aortic valve prolapse (AVP) and mild aortic regurgitation (AR). Methods: Between January 2008 and July 2014, transcatheter closure of VSD was attempted in 65 patients. Results: The total intermediate closure successful rate in all subjects was 96.9%. During the perioperative period, no death, major bleeding, pericardial tamponade, occluder dislodgement, residual shunt or hemolysis occurred. Two procedures had been forced to suspend due to significant aggregation of device related aortic regurgitation, three cases of transient complete left bundle branch block occurred but did not sustain. At 1-year follow-up, no patients had residual shunts and complications. Furthermore, grade of residual AR were relieved in 61.9% (39/63) cases and degree of AVP were ameliorated in 36.5% (23/63) patients; Conclusions: Transcatheter closure VSD in selected patients with AVP and mild AR is technically feasible and highly effective. Long term safety and efficacy needs to be assessed.

  6. [Right ventricular outflow tract reconstruction using monocusp valved outflow patch for pulmonary atresia with ventricular septal defect: influence of the presence of major aorto-pulmonary collateral arteries].

    Science.gov (United States)

    Hisamochi, K; Ishino, K; Kawada, M; Ohshima, Y; Aoki, A; Arai, S; Sano, S

    2001-07-01

    We have preferably utilized monocusp valved outflow patch (MVOP) for right ventricular outflow tract (RVOT) reconstruction in pulmonary atresia with ventricular septal defect (PA + VSD). The purpose of this study was to evaluate the influence of the presence of major aorto-pulmonary collateral arteries (MAPCAs) on probability of MVOP reconstruction and development of RVOT restenosis in midterm. 49 patients underwent complete repair (either MVOP reconstruction or Rastelli procedure) of PA + VSD in our service. These patients were divided into 2 groups: group 1; 21 patients with MAPCAs, group 2; 28 patients without MAPCAs. There was one operative death (group 1). The probably of MVOP reconstruction was similar between group 1 and group 2 (71 vs 79%, p = 0.57, chi 2 test). Follow-up was completed for 48 survivors with the period ranged 3-108 months (mean 47 months). In group 1, one patient died suddenly at home 10 months after surgery. For 47 long-term patients, the ratio of freedom from RVOT restenosis was 72% (95% CI: 52-92%, Kaplan-Meier method) at 5 year. There was no difference between 2 groups (group 1; 73%, 95% CI: 45-100%, group 2; 74%, 95% CI: 48-99%, respectively, p = 0.85 by Log-Rank test). The presence of MAPCAs in PA + VSD was not a risk factor for either the probably of MVOP reconstruction or development of RVOT restenosis in midterm.

  7. Disturbances in atrial rhythm and conduction following the surgical creation of an atrial septal defect by the Blalock-Hanlon technique.

    Science.gov (United States)

    Hamilton, S D; Bartley, T D; Miller, R H; Schiebler, G L; Marriott, H J

    1968-07-01

    Disturbances in atrial conduction or rhythm, or both, were found in 16 of 27 patients undergoing the surgical creation of an atrial septal defect by the Blalock-Hanlon technique. These included P-wave aberrations of intra-atrial block and ectopic atrial rhythm, A-V rhythm, A-V dissociation, sinus bradycardia, atrial flutter, escape-capture bigeminy, and atrial premature beats. Some of these were transient and occurred within 2 weeks after surgery. It seems likely that tissue trauma engendered by the clamp and resection of the atrial septum with possible injury to the internodal conducting pathways may be the genesis of these early postoperative disturbances. Of the eight cases with transient disturbances, six occurred in this period. Later changes, such as intra-atrial block, sinus bradycardia, and atrial flutter, may be attributed to changes in atrial size secondary to the postoperative pathophysiology. Digitalis intoxication and congestive heart failure cannot be excluded as contributory factors in either the early or the late disturbances. It was not possible to correlate the incidence and nature of these disturbances with morbidity and mortality. In such severely ill, cyanotic infants, irregularities, however slight, may have altered cardiac function significantly and contributed to their deaths.

  8. Midterm results of diagnostic treatment and repair strategy in older patients presenting with nonrestrictive ventricular septal defect and severe pulmonary artery hypertension

    Institute of Scientific and Technical Information of China (English)

    Liu Aijun; Li Zhiqiang; Li Xiaofeng; Fan Xiangming; Su Junwu; Zhang Jing; He Yan

    2014-01-01

    Background Congenital heart disease with severe pulmonary arterial hypertension (SPAH),previously thought to have irreversible pulmonary vascular disease (PVD),has been recently successfully corrected using diagnostic treatment and repair strategy by our surgery team.This study aimed to evaluate the midterm results of a selected cohort of older patients with nonrestrictive ventricular septal defect (VSD) and SPAH using diagnostic treatment and repair strategy.Methods The records of 56 patients older than six years with nonrestrictive VSD and SPAH undergoing diagnostic treatment and repair strategy from 2006 to 2012 were retrospectively reviewed.All patients received advanced pulmonary arterial hypertension (PAH) therapy and radical repairs were performed when transcutaneous oxygen saturation (SPO2) increased up to 93%.Results There were no operative deaths.SPO2 and baseline six-minute walk test (SMWT) distance of all selected patients increased significantly and mean pulmonary artery pressure (MPAP) regressed significantly after diagnostic treatment and at late follow-up (P <0.01).The incidence of late postoperative PAH was seen in six (10.7%) patients and by Logistic regression analysis,early postoperative PAH was an independent risk factor related to late postoperative PAH.Conclusions Diagnostic treatment and repair strategy was effective and safe for treatment of nonrestrictive VSD and SPAH and the midterm results were excellent.Diagnostic treatment strategy was effective in assessing the reversibility of SPAH in older patients associated with nonrestrictive VSD and the PVD in these selective patients is generally reversible.

  9. Postprocedural Outcomes and Risk Factors for Arrhythmias Following Transcatheter Closure of Congenital Perimembranous Ventricular Septal Defect: A Single-center Retrospective Study

    Science.gov (United States)

    Zhao, Li-Jian; Han, Bo; Zhang, Jian-Jun; Yi, Ying-Chun; Jiang, Dian-Dong; Lyu, Jian-Li

    2017-01-01

    Background: Currently, transcatheter closure of perimembranous ventricular septal defect (pmVSD) is a widely accepted therapeutic modality. However, arrhythmias, especially postprocedural heart blocks, are a concern and outcomes are not very clear. This study explored the outcomes and risk factors of arrhythmias associated with transcatheter device closure of pmVSD. Methods: A total of 395 patients diagnosed with pmVSD who successfully underwent transcatheter intervention between January 2010 and December 2015 in our center were retrospectively reviewed. Electrocardiographic data before and after the procedure were collected and analyzed. We first evaluated the potential risk factors including gender, age, weight, inlet and outlet diameters of defect, subaortic rim length, occluder size, corrected occluder size into body surface area, fluoroscopy time, presence of aneurysm, and deployment position. We compared the potential risk factors between arrhythmia and nonarrhythmia groups using univariate analysis, followed by logistic analysis for independent risk factors. Results: Various arrhythmias were detected in 95 cases (24.1%) following transcatheter closure procedure. Logistic regression analysis revealed that eccentric (odds ratio [OR] 2.9, 95% confidence interval [CI]: 1.2–7.2) and large occluders (OR 2.0, 95% CI: 1.6–2.5), as well as long fluoroscopy time (OR 1.1, 95% CI: 1.1–1.2), were correlated with postprocedural arrhythmia. During 35.5 months (range: 9–80 months) of follow-up, most of the patients (74 out of 95) reverted to normal heart rhythm. Conclusions: The mid-term outcome of patients with arrhythmias after transcatheter closure of pmVSD was satisfactory as most of the patients recovered normal rhythm. Eccentric, large device and long fluoroscopy time increase the risk of arrhythmias after transcatheter closure of pmVSD. PMID:28229981

  10. Dextrocardia, atrial septal defect, severe developmental delay, facial anomalies, and supernumerary ribs in a child with a complex unbalanced 8;22 translocation including partial 8p duplication.

    Science.gov (United States)

    Pope, Kathleen; Samanich, Joy; Ramesh, K H; Cannizzaro, Linda; Pan, Qiulu; Babcock, Melanie

    2012-03-01

    We report on a child with dextrocardia, atrial septal defect (ASD), severe developmental delay, hypotonia, 13 pairs of ribs, left preauricular choristoma, hirsutism, and craniofacial abnormalities. Prenatal cytogenetic evaluation showed karyotype 46,XY,?dup(8p)ish del(8)pter. Postnatal array CGH demonstrated a 6.8 Mb terminal deletion at 8p23.3-p23, an interstitial 31.1 Mb duplication within 8p23.1-p11, and a terminal duplication of 0.24 Mb at 22q13.33, refining the karyotype to 46,XY,der(8)dup(8)(p23.1p11.1)t(8;22)(p23.1;q13.1).ish der(8)dup(8)(p23.1p11.1)t(8;22)(p23.1;q13.1) (D8S504-,MS607 + ,ARSA + ,D8Z1 + , RP115713 + +). Previous reports of distal 8p deletion, 8p duplication, and distal 22q duplication have shown similar manifestations, including congenital heart disease, intellectual impairment, and multiple minor anomalies. We correlate the patient's clinical findings with these particular areas of copy number. This case study supports the use of aCGH to identify subtle chromosomal rearrangement in infants with cardiac malformation as their most significant or only apparent birth defect. Additionally, it illustrates why aCGH is essential in the description of chromosome rearrangements, even those seemingly visible via routine karyotype. This method shows that there is often greater complexity submicroscopically, essential to an adequate understanding of a patient's genotype and phenotype.

  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. 胎儿先天性室间隔缺损的染色体核型分析%Study of chromosome karyotype in congenital ventricular septal defect of fetus

    Institute of Scientific and Technical Information of China (English)

    高金梅; 关云萍; 项宇识; 闫峰; 李冬梅

    2013-01-01

    目的:通过产前诊断分析先天性胎儿室间隔缺损与染色体核型的相关性。方法:回顾性分析我院产前超声诊断胎儿发育异常并进行脐静脉血穿刺染色体核型分析的患者387例,对其中单纯室间隔缺损或室间隔缺损伴发其他结构异常的病例74例进行分析。结果:74例病例中,脐血穿刺血细胞培养成功72例,培养失败2例。72例病例中,产前超声诊断单纯室间隔缺损12例,染色体核型结果均正常;产前超声诊断室间隔缺损伴发其他结构异常60例,其中染色体核型异常者17例(18-三8例、13-三体3例、21-三体4例、其他2例),染色体核型正常者43例。结论:胎儿心脏室间隔缺损伴发其他结构异常时较单纯室间隔缺损出现染色体异常的几率大。%Objective:To analyse the correlations between congenital ventricular septal defects and fetal karyotype through prenatal diagnosis.Methods:A retrospective analysis of 387 cases who diagnosed with fetal abnormalities and carried on umbilical vein puncture for karyotype analysis in our hospital through prenatal ultrasound diagnosis. among which, 74 cases with the ventricular septal defect or ventricular septal defect simply associated with other structural abnormalities were analyzed.Results:In the 74 cases, 72 cases were successful y cultured in the umbilical cord blood cells, 2 cases were cultured failure. 12 cases were diagnosed with ventricular septal defect simply in prenatal ultrasound diagnosis in the 72 cases, karyotype of the 12 cases were al normal; 60 cases were diagnosed with ventricular septal defect associated with other structural abnormalities in prenatal ultrasound diagnosisin the 72 cases, in which chromosome abnormalities were 17 cases (8 cases of 18-trisomy syndrome;3 cases of 13-trisomy syndrom;4 cases of 21-trisomy syndrom;2 cases of other chromosomal abnormalities), normal karyotype were 43 cases.Conclusion:Fetal cardiac

  13. Atrial septal stenting - How I do it?

    Directory of Open Access Journals (Sweden)

    Kothandam Sivakumar

    2015-01-01

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

  14. Surgical treatment of isolated atrial septal defect secundum in the elderly%老年房间隔缺损病人的手术治疗

    Institute of Scientific and Technical Information of China (English)

    韩林; 张宝仁; 郝家骅; 朱家麟; 梅举; 邹良建

    2001-01-01

    目的总结老年房间隔缺损病人手术治疗经验。方法对12例60岁以上继发孔型房间隔缺损病人进行手术治疗,年龄为60~66岁,平均(62.3±2.4)岁,全部合并三尖瓣关闭不全,其中还合并二尖瓣关闭不全10例;房颤9例,房扑1例;术前采用右心导管进行肺动脉测压,肺动脉平均压为(5.31±3.12)kPa,最高为8.4 kPa。本组房缺修补采用心包片或涤纶补片修补,同期行三尖瓣成形术12例,其中3例行Kay’s二叶瓣成形术,9例行De vage成形术;二尖瓣成形术6例,二尖瓣置换术2例,3例合并有房颤者同期行右房迷宫术。结果 12例病人术后全部存活,心功能均有明显改善,但与其他年龄组手术病人比较,术后多巴胺使用时间和住院时间明显延长,6例发生胸腔积液,术后随访3~60个月。结论老年房缺病人修补手术成功的关键在于合并症的同期手术处理和手术后的心功能支持。%Objective To review the clinical experiences of surgical treatment of isolated atrial septal defect secundum in elderly patients. Methods 12 patients were over 60 years of age (range, 60~ 66, with a mean of 62.3 years). Coexislent disorders were moderate to severe tricuspid regurgitation in all 12 patients, mitral valve regurgitation in 10, atrial fibrillation in 9 and atrial flutter in 1. Preoperative mean pulmonary artery pressure in these 12 patients was 5.31 + 3.12 kPa. All patients underwent closure by Dacron and pericardial patch. 12 patients underwent either De vage or Kay tricuspid annuloplasty for TVR, and 6 mitral valve repair, 2 mitral valve replacements. Three patients with atrial fibrillation underwent right-sided maze procedure. Results All patients survived the operation, heart function being improved in most patients. Conclusion Old age is not a contradiction to repair of atrial septal defect. The key element in successful operation relies on concomitent correction of the

  15. Down 综合征患儿室间隔缺损的手术治疗%Surgical Treatment of Ventricular Septal Defect in Children with Down Syndrome

    Institute of Scientific and Technical Information of China (English)

    吴士强

    2014-01-01

    目的:总结 Down 综合征患儿室间隔缺损的外科治疗经验。方法48例 Down 综合征患儿室间隔缺损在全身麻醉低温体外循环下行一期根治术。术前对于合并肺部感染、心功能不全、肺动脉高压者,给予有效的抗感染、强心及利尿剂、降肺动脉压等治疗;加强静脉营养,提高机体免疫力。术中提高手术技巧,操作轻柔,选择合适的手术切口及修补材料,避免发生传导阻滞等并发症。术后入 CICU,加强呼吸道管理,合理使用呼吸机;合理使用血管活性药物,维持循环系统的稳定;合理使用抗生素,减少肺部感染的发生。结果48例中无1例死亡,并发呼吸机相关性肺炎10例,心律失常2例,气胸1例,心包填塞1例,低心排1例。术后随访6个月至6年,生长发育好,无死亡。结论Down 综合征室间隔缺损患儿应尽早手术,选择适当的手术时机,合理有效的围术期处理,可提高手术成功率,延长患儿寿命。%Objective To summarize the experience with the surgical treatment of ventricular septal defect in children with Down syndrome.Methods Forty-eight children with Down syn-drome underwent one-stage radical operation under general anesthesia during hypothermic car-diopulmonary bypass.Patients with lung infection,heart failure,and pulmonary hypertension were given anti-infection,cardiotonics and diuretics,and anti-pulmonary hypertension,respectively.In addition,intravenous nutrition was strengthened and immunity was improved in all patients.Dur-ing operation,gentle operation technique was carried out and appropriate surgical incision and re-pair material were selected to avoid conduction block and other complications.After operation,pa-tients were admitted to CICU,and respiratory tract management and rational use of breathing ma-chine were strengthened.Moreover,vasoactive drugs and antibiotics were rationally used to main-tain the stability of

  16. Effects of VAT and DDD pacing on left cardiac function in the patients of high right ventricular septal pacing with third degree atrioventricular block%Ⅲ度房室传导阻滞患者高位右室间隔部起搏VAT与DDD模式对左心功能的影响

    Institute of Scientific and Technical Information of China (English)

    王燕; 章蓉; 王旭; 邵一兵

    2012-01-01

    目的 通过研究Ⅲ度房室传导阻滞患者高位右室间隔部起搏( HRVS)时,VAT与DDD模式对心功能的影响,探讨心房的生理收缩和舒张对左心功能的作用.方法 32例Ⅲ度房室传导阻滞患者,在HRVS时,分别给予DDD模式或VAT模式工作,于调控即刻通过超声心动图测定二尖瓣口快速充盈期峰值血流速度(Ep),二尖瓣口左房收缩期峰值血流速度(Ap),二尖瓣环后壁处收缩期脉冲组织多普勒峰值速度平均值(Vs),二尖瓣环后壁处舒张早期脉冲组织多普勒峰值速度平均值(Ve),二尖瓣环后壁处舒张晚期脉冲组织多普勒峰值速度平均值(Va),二尖瓣血流频谱等容舒张时间(IVRT),通过Ep/Ap,Ve/Va,Ep/Ve和IVRT评价左室舒张功能,通过Vs,LVEF和LVFS评价收缩功能.结果 HRVS时DDD较VAT模式Ep/Ap、Ve/Va、Ep/Ve,IVRT差异有显著性(0.97 ±0.11 vs 1.01±0.11,0.89 ±0.09 vs 0.97 ±0.07,6.00 ±0.45 vs 6.24 ±0.36,100.4±14.32 vs 89.99 ±7.94;P均<0.01),Vs、左室射血分数和左室短轴缩短率无显著性差异.结论 Ⅲ度房室传导阻滞时,HRVS起搏时,DDD模式较VAT模式使左室舒张功能下降,收缩功能无影响,表明了心房生理性起搏的重要性.%Objective To analyze the effects of physiologic systole and diastole of atrium, through the influence of VAT and DDD pacing mode on left cardiac function in patients of high right ventricular septal( HRVS) pacing with third degree atrioventricular block. Methods Thirty-two patients of HRVS pacing with third degree atrioventricular block with pacemaker mode of VAT or DDD underwent complete standard echocardiography (2D,M-mode, pulsed Doppler) and PW-DTI. Transmitral inflow velocity Ep and Ap, peak systolic velocity ( Vs ) , peak early diastolic velocity ( Ve) , peak late diastolic velocity (Va) of posterior mitral annulus and the isovolumic relaxation time(IVRT) were obtained . Then left ventricular diastolic function were evaluated through Ep/Ap, Ve/Va and Ep

  17. Re-operation of residual ventricular septal defect in 12 cases%残余室间隔缺损的再手术治疗12例

    Institute of Scientific and Technical Information of China (English)

    胡晓峰; 周运乾; 曹庆亨; 邱兆崑

    2001-01-01

    Objective To analyze the causation, surgical indications and pivotal points in reoperation of residual ventricular septal defect (VSD). Methods Collecting the clinical data of 12 cases of reoperated residual VSD in this hospital from 1988 to 1998 and investigating into insight of their causation, surgical indications and keys elements of reoperation in residual VSD. Results This group consisted of 12 patients of whom 8 were operated in this hospital from 1977 to 1997, and 4 were operated in other hospital. The re-operative rate of VSD in our hospital was 0.18 %.The defects in the first operation were 8 cases of simple VSD and 3 tetralogy of Fallot. The mean interval between the two operations was 4.1 years (5 months-12 years). Residual shunting in 10 patients were adjacent to the base of anterior and septal leaflets of tricuspid valve. The mean longitudinal diameter of residual VSD was 1.2 cm(0.3~2.5 cm).Three patients died after reoperation. Causes of death included low cardiac output syndrome, cardiac arrhythmia and cardiac tamponade. Conclusion 1. The common location of residual VSD is around the base of anterior and septal leaflets of tricuspid valve, this area involves some important structures like aortic valve and AV bundle which are not easily exposed. 2. Adequate exposure of entire VSD margin is the key element to avoid leakage by laceration. 3. Early re-operation is required for all residual VSD with diameter above 0.5 cm. 4. The key points of reoperation are timely operation, atrial incisional approach, patch repairment and careful hemostasis.%目的分析残余室间隔缺损(VSD)的发生原因、防治措施、手术指征和手术要点。方法对12例施行残余VSD再手术治疗患者的发生原因、预防措施、手术指征和手术要点进行分析。结果 12例中8例首次手术于1977年至1997年在我院施行,4例在外院施行,我院VSD再手术率为0.18%。首次手术时单纯VSD 9例,法洛四联症(TOF)3

  18. Atrioventricular Dissociation following Blunt Chest Trauma

    Directory of Open Access Journals (Sweden)

    Salim Surani

    2014-01-01

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

  19. Relationships between left heart chamber dilatation on echocardiography and left-to-right ventricle shunting quantified by cardiac catheterization in children with ventricular septal defects.

    Science.gov (United States)

    Gokalp, Selman; Guler Eroglu, Ayse; Saltik, Levent; Koca, Bulent

    2014-04-01

    Left atrium and/or left ventricle dilatation on echocardiography is considered to be an indication for closure of ventricular septal defects (VSD). No study has addressed the accuracy of using dilated left heart chambers when defining significant left-to-right shunting quantified by cardiac catheterization in isolated small or moderate VSDs. In this study, the relation between dilated left heart chambers, measured by echocardiography, and left-to-right ventricle shunting, quantified by cardiac catheterization, was evaluated in patients with isolated VSD. The medical records of all patients with isolated VSD who had undergone catheterization from 1996 to 2010 were examined retrospectively. Normative data for left heart chambers adjusted for body weight (BW) and body surface area (BSA) were used. The pulmonary-to-systemic flow ratio (Qp:Qs) was calculated by an oximetry technique. A total of 115 patients (mean age 7.3 ± 5 years) fulfilled the inclusion criteria. There was a statistically significant difference in terms of Qp:Qs between the patient groups with normal and dilated left heart chambers, when adjusted for BW and BSA (p = 0.001 and p = 0.002, respectively). But the relationships between Qp:Qs and left heart chamber sizes on echocardiography were not strong enough to be useful for making surgical decisions, as left heart chamber dilatation was not significantly associated with Qp:Qs ≥ 2 (p = 0.349 when adjusted for BW, p = 0.107 when adjusted for BSA). Left heart chamber dilatation was significantly associated with Qp:Qs ≥ 1.5 only when it was adjusted for BSA (for BW p = 0.022, for BSA p = 0.006). As a result, left heart chamber dilatation measured by echocardiography does not show significant left-to-right ventricle shunting, as quantified by catheterization. We still advocate that catheter angiography should be undertaken when left heart chambers are dilated in echocardiography in order to make decisions about closing small- to moderate-sized VSD.

  20. Robotic-assisted endoscopic atrial septal defect closure:analysis of 115 cases in a single center%单中心、机器人辅助下房间隔缺损修补术

    Institute of Scientific and Technical Information of China (English)

    杨明; 高长青; 肖苍松

    2012-01-01

    目的 总结单中心、由同一术者完成的机器人辅助下房间隔缺损修补术的临床经验.方法 2007年1月至2011年9月,115例继发孔型房间隔缺损患者接受了机器人辅助下房缺修补术.患者中位年龄35岁,中位缺损直径为28 mm.术中采用外周体外循环技术,右侧胸壁开3个直径8mm的小孔及1个15mm的工作孔,术者于机器人系统操控台前、三维手术视野下遥控机械臂完成房缺修补或同期三尖瓣成形.术中食道超声评估手术效果,出院前及术后1个月常规心脏超声复查.结果 本组病例中无手术死亡或术中术式转化,其中54例房缺修补在心脏停跳下完成,61例于心脏不停跳下完成.术中检查及术后超声复查未见房缺残余分流.平均手术时间为(257.4 ±79.5) min,平均体外循环时间(81.6±32.7)min.心脏不停跳组的手术时间和体外循环较心脏停跳组明显缩短;两组患者的呼吸机辅助时间、ICU时间、术后引流量和住院时间无明显差别.结论 利用机器人系统可在心脏停跳或不停跳下安全完成继发孔房间隔缺损修补,手术效果良好.%Objective To summarize the experience with the application of robotic technique in totally endoscopic atrial septal defect closure in a single center.Methods Between January 2007 and September 2011,115 patients with the diagnosis of sectmdum 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.Results Atrial septal defect closure was completed on

  1. Atrial Septal Defect (For Parents)

    Science.gov (United States)

    ... Development Infections Diseases & Conditions Pregnancy & Baby Nutrition & Fitness Emotions & Behavior School & Family Life First Aid & Safety Doctors & ... indicate a problem include: a bluish tinge or color (cyanosis) to the skin around the mouth or ...

  2. Atrial Septal Defect (For Kids)

    Science.gov (United States)

    ... which records the electrical activity of the heart echocardiogram (echo), which uses sound waves to create a ... one, the kid will get special medicine called anesthesia that causes sleepiness and prevents pain during the ...

  3. 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...Many radiology corner articles are also MedPix™ cases of the week where CME credits may be obtained. http://rad.usuhs.mil/amsus.html References...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

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

    Directory of Open Access Journals (Sweden)

    Maria da Gloria Cruvinel Horta

    2005-02-01

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

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

    Institute of Scientific and Technical Information of China (English)

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

    2014-01-01

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

  6. Totally robotic atrial septal defect closure using da vinci S surgical system on beating heart%全机器人心脏不停跳下房间隔缺损修补术

    Institute of Scientific and Technical Information of China (English)

    杨明; 高长青; 肖苍松; 王刚; 王加利

    2011-01-01

    目的 总结使用"达芬奇S"(da Vinci S)机器人手术系统,心脏不停跳下房间隔缺损修补或房间隔缺损修补+三尖瓣成形术的经验体会.方法 2009年3月至2010年12月,使用da Vinic S机器人系统,心脏不停跳下完成继发孔型房间隔缺损修补或房间隔缺损修补+三尖瓣成形术40例.患者女23例,男17例;年龄平均(38±13)岁.房间隔缺损直径为1.5~3.5 cm,平均(2.8±1.3)cm,无右向左分流,伴有或不伴有三尖瓣重度关闭不全.手术经股动、静脉及右侧颈内静脉插管建立体外循环.于右侧胸壁打直径为0.8 cm的器械臂孔3个,直径为2 cm工作孔1个,术中不阻断升主动脉,经内窥镜套管持续给予二氧化碳,心脏跳动下,术者于操作台前遥控机器人进行房间隔缺损修补,三尖瓣重度关闭不全患者同期行三尖瓣成形术.其中直接缝合房间隔缺损22例,心包补片修补房间隔缺损18例,同期三尖瓣成形9例.术中食管超声评估修补及三尖瓣成形效果.对比不停跳与心脏停跳下全机器人房间隔缺损修补术的手术时间及体外循环时间.结果 所有患者均成功接受全机器人心脏不停跳下房间隔缺损修补术或房间隔缺损修补+三尖瓣成形术,无体循环气体栓子及残余分流等并发症.不停跳组的手术时间、机器人使用时间或体外循环时间少于停跳组.结论 机器人心脏不停跳下房间隔缺损修补术无需阻断升主动脉,简化了全机器人手术过程,手术效果安全可靠.%Objective To Summary the first 40 cases underwent robotic atrial septal defect (ASD) closure or atrial septal defect closure combined bicuspid valve plasty (TVP) using "da Vinci S" surgical System on beating heart. Methods 40 cases of atrial septal defect or combined sever tricuspid valve regurgitation were repaired using "da Vinic S" surgical system on beating heart from March 2009 to December 2010 in cardiovascular department of PLA general

  7. Perioperative management of the ventricular septal defect with moderate or severe pulmonary hypertension%室间隔缺损并中重度肺动脉高压患者围手术期处理

    Institute of Scientific and Technical Information of China (English)

    李占伟; 程兆云; 张国报; 权晓强; 乔刚; 刘传振

    2011-01-01

    目的:探讨室间隔缺损并中重度肺动脉高压患者的手术时机及围手术期处理.方法:28例室间隔缺损并中重度肺动脉高压患者,术前给予吸氧、前列腺素E1等药物治疗后均行室间隔缺损修补术,部分重度肺动脉高压患者应用单向活瓣补片修补室间隔缺损.结果:术后死亡2例,其中1例为低心排血量综合征并肺动脉高压危象,1例为低心排血量综合征并肺部感染.余26例安全度过围手术期并痊愈出院.结论:选择手术时机,术中完善手术修复和恰当的围术期处理是室间隔缺损并中重度肺动脉高压患者治疗的关键.%Objective To study the surgical opportunity and perioperative management of the ventricular septal defect with moderate or severe pulmonary hypertension. Methods After medicinal therapy, 28 cases of ventricular septal defect received operation. One-way valve patch and further myocardial protection were applied on patients with severe pulmonary hypertension. Results Two patients died after the operation, in which one died of low cardiac output syndrome and pulmonary hypertension crisis, and the other died of low cardiac output syndrome and pulmonary infection. Twenty-six patients went through perioperative period smoothly. Conclusion Proper choice of surgical opportunity, complete surgical repair and good perioperative management are the keys to the successful operation of ventricular septal defect with moderate or severe pulmonary hypertension.

  8. Short-and Mid-term Follow-up Study on Tricuspid Valve Detachment in Correction of Ventricular Septal Defect%三尖瓣适当切开修补室间隔缺损的近中期随访研究

    Institute of Scientific and Technical Information of China (English)

    陈仁伟; 罗金文; 刘剑; 黄鹏; 曾德斌; 刘平波; 周文武

    2012-01-01

    目的 探讨三尖瓣适当切开技术(tricuspid valve detachment,TVD)在室间隔缺损(ventricular septal defect,VSD)修补手术中的临床价值. 方法 回顾性分析我院2006年1月~2010年12月行VSD修补的1 021例患者的临床资料,其中211例采用TVD技术(TVD组),810例未采用TVD技术(非TVD组).所有患者在术前与术后3d、1个月、6个月及1年行超声心动图观察心脏结构,评估三尖瓣返流(tricuspic regurgitation,TR)情况. 结果 695例补片修补VSD,其中TVD组209例(128例使用单独缝线修补瓣叶切口,81例使用VSD补片缝线),非TVD组486例;另326例直接缝合VSD,其中TVD 组2例(使用VSD补片缝线直接修补瓣叶切口),非TVD组324例.671例进行了合并畸形手术,其中TVD组115例(占TVD组54.5%),非TVD组556例(占非TVD组68.6%).两组CPB时间、主动脉阻断时间及术后住院时间比较,均无统计学差异(P>0.05).两组均无围手术期死亡病例.TVD组无完全性房室传导阻滞出现,非TVD组出现3例,均于术后10 d内恢复窦性.术后3 d VSD残余漏11例,其中TVD组3例,非TVD组8例;TVD组轻度以上的TR病例14例,非TVD组有47例.两组均无因TR和残余漏而再手术者.两组术后并发症发生率比较无统计学差异(8.1% VS 7.2%,P>0.05).所有病人随访1年,无远期死亡病例,均无残余漏需远期再手术者.术后1年TVD组无轻度以上TR,非TVD组有8例轻度以上TR,组间比较差异无统计学意义(P>0.05).两组均无因TR再手术者.结论 TVD是一种安全、有效的技术,能改善VSD的暴露,与传导阻滞、手术时间延长以及TR无相关性.TVD能保护三尖瓣瓣叶结构,减少术后严重TR的发生.%Objective To investigate the clinical value of temporary tricuspid valve detachment (TVD) in the correlation of ventricular septal defect (VSD). Methods A total of 1 021 VSD patients admitted January 2006 to December 2010 in our hospital were analyzed retrospectively, including 211 of TVD

  9. Investigation of Influence Factors and Nutritional Status in Ventricular Septal Defect Children before Surgery%室间隔缺损患儿术前的营养状况及其影响因素

    Institute of Scientific and Technical Information of China (English)

    焦文娟; 陈京立; 李庆印

    2014-01-01

    探讨室间隔缺损患儿术前的营养状况及其可能的影响因素.方法采用便利抽样法,选取2011年5-12月在北京阜外医院就诊并接受室间隔缺损修补术的患儿143例,采用人体测量法、访谈法和问卷法调查患儿营养不良发生的情况及其影响因素.结果(1)室间隔缺损修补术前患儿急性营养不良发生率为44.1%,慢性营养不良发生率为21.0%,总营养不良发生率为50.3%;(2)Logistic回归分析结果显示,患儿出生体质量、术前肺动脉压力水平、能量摄入情况及患儿母亲是否通过网络或图书获取营养知识是影响患儿术前营养状况的独立预测因素.结论室间隔缺损修补术前患儿营养不良发生率高,护理人员应全面评估患儿的出生体质量、术前肺动脉压力水平、能量摄入情况等,并有针对性地向高危患儿家属进行相关宣教,以改善患儿的营养状况,提高手术成功率.%Objective To investigate the nutritional status in ventricular septal defect children before surgery and to analyze its influence factors.Methods By convenience sampling,totally 1 4 3 ventricular septal defect children were selected and evaluated by using body measurement,interviews and ques-tionnaires to investigate the incidence of malnutrition and its influencing factors.Results ①The inci-dence of acute malnutrition and chronic malnutrition were 4 4 .1%,2 1%.The incidence of malnutrition was 5 0 .3%.②Logistic analysis showed that the influence factors of nutritional status in ventricular septal defect infants were birth weight,pulmonary artery pressures,dietary intake and whether the mother required nutritional information through e-books was the independent predictive factor.Conclu-sion There is a high incidence of malnutrition in ventricular septal defect children before surgery. Nurses should comprehensively evaluate the birth weight,pulmonary artery pressures,dietary intake before

  10. Fourier analysis of multi-gated blood-pool data in patients with congenital heart disease, (1). Assessment of disease with left to right shunt, especially ventricular septal defect

    Energy Technology Data Exchange (ETDEWEB)

    Takeda, Kan; Maeda, Hisato; Yamaguchi, Nobuo; Nakamura, Kazuyoshi; Nakagawa, Tsuyoshi (Mie Univ., Tsu (Japan). School of Medicine)

    1983-07-01

    The ventricular emptying performance in patients with congenital heart disease with left to right (L-to-R) shunt was investigated by temporal Fourier analysis of multi-gated cardiac blood-pool data. Functional images are constructed with parameters of each pixel's phase angle and amplitude at fundamental frequency. Using global time-activity curves of both ventricles, phase angle and amplitude of left and right ventricles (LV and RV) were computed. Values of interventricular phase difference (D (phase)) and amplitude ratio of RV to LV (R (amp)) were calculated in individual cases. In 18 subjects with normal cardiac function, mean ( +- standard deviation) values of D (phase) was 1.7 +- 5.8 degree and that of R (amp) was 0.54 +- 0.20, respectively regardless of heart rate. In 22 patients of ventricular septal defect (VSD) with L-to-R shunt, D (phase) became larger in proportion to the ratio of pulmonary to systemic blood flow (Qp/Qs) (r = 0.899, p < 0.001). Especially, in those with large L-to-R shunt (Qp/ Qs < 2.0), significant RV phase lag over 18 degrees was recognized and types of VSD might be possible to be differentiated by phase images. In 9 patients with patent ductus arteriosus (PDA), no RV phase delay was seen. Mean value of R (amp) was considerably smaller in patients with PDA and significantly larger in 11 patients with atrial septal defect (ASD), as compared with that of subjects with normal cardiac functions. However, cases with VSD took the values within normal range. This method is highly valuable for pathophysiologic investigation and differential diagnosis of congenital heart disease with L-to-R shunt.

  11. Comparative analysis of early and middle outcomes of the arterial switch operation in children with complete transposition of the great arteries with ventricular septal defect and severe pulmonary artery hypertension

    Institute of Scientific and Technical Information of China (English)

    LIU Cheng-hu; SU Jun-wu; LI Zhi-qiang; FAN Xiang-ming; CHEN Yan; HE Yan; LIU Ying-long

    2013-01-01

    Background The best age for the arterial switch operation (ASO) in complete transposition of great arteries with ventricular septal defect is usually considered to be within six months.This is because of severe pulmonary arterial hypertension and pulmonary arterial obstructive pathological changes.There are few reports on ASO surgery in children older than three years old.Methods We studied 41 children,including 24 males and 17 females,from January 2010 to December 2011.They were divided into three groups by operation age; 15 patients were <1 year old,13 were 1-3 years old,and 13 were >3 years old.Associated cardiac abnormalities included patent ductus arteriosus in six cases,atrial septal defect in five cases,and mitral regurgitation in two cases.All the patients had echocardiography before the operation.Seventeen patients underwent a coronary computed tomography examination and five patients underwent right heart catheterization.All ASO surgeries were performed under inhalation anesthesia and hypothermic cardiopulmonary bypass.Results Three operative deaths occurred.Two were in the <1 year old group,who died from severe postoperative low cardiac output.The other was two years old and died of postoperative multiple organ failure.There was no significant difference in postoperative mortality and the recent mid-term survival rate among the three groups.Thirty-eight cases were followed up for an average of 11.2 months,ranging 6-20 months.One seven years old patient died of acute diarrhea and electrolyte disturbance arrhythmia caused by food poisoning.Three patients more than three years old still had residual pulmonary arterial hypertension.Conclusion Children older than three years old can still undergo the ASO procedure,but residual pulmonary hypertension is present.

  12. Straddling mitral valve with hypoplastic right ventricle, crisscross atrioventricular relations, double outlet right ventricle and dextrocardia: morphologic, diagnostic and surgical considerations.

    Science.gov (United States)

    Geva, T; Van Praagh, S; Sanders, S P; Mayer, J E; Van Praagh, R

    1991-06-01

    The clinical, surgical and morphologic findings in five cases of a rare form of straddling mitral valve are presented. Three patients were diagnosed by two-dimensional echocardiography, cardiac catheterization and angiocardiography and two had diagnostic confirmation at autopsy. All five cases shared a distinctive and consistent combination of anomalies: 1) dextrocardia; 2) visceroatrial situs solitus, concordant ventricular D-loop and double outlet right ventricle with the aorta positioned to the left of and anterior to the pulmonary artery; 3) hypoplasia of right ventricular inflow (sinus) with tricuspid valve stenosis or hypoplasia; 4) large right ventricular infundibulum (outflow); 5) malalignment conoventricular septal defect; 6) straddling mitral valve with chordal attachments to the left ventricle and right ventricular infundibulum; 7) severe subpulmonary stenosis with well developed pulmonary arteries; and 8) superoinferior ventricles with crisscross atrioventricular (AV) relations. The degree of malalignment between the atrial and ventricular septa was studied quantitatively by measuring the AV septal angle projected on the frontal plane. The AV septal angle in the two postmortem cases was 150 degrees, reflecting marked malalignment of the ventricles relative to the atria. This AV malalignment appears to play an important role in the morphogenesis of straddling mitral valve. As judged by a companion study of seven postmortem cases, the more common form of straddling mitral valve with a hypertrophied and enlarged right ventricular sinus had less severe ventricular malposition than did the five rare study cases with hypoplastic right ventricular sinus. A competent mitral valve, low pulmonary vascular resistance and low left ventricular end-diastolic pressure were found at cardiac catheterization in the three living patients who underwent a modified Fontan procedure and are doing well 2.2 to 5.8 years postoperatively.

  13. 经胸微创封堵术治疗婴幼儿室间隔缺损的疗效评价%Evaluation of the efficacy of transcatheter closure of ventricular septal defect in infants and young children

    Institute of Scientific and Technical Information of China (English)

    宋毅; 钟前进; 陈建明; 李志平; 李福平; 胡义杰

    2015-01-01

    Objective To investigate the therapeutic effect of transcatheter closure of ventricular septal defect in infants oryoung children with minimally invasive surgery,and to explore the clinical application value. Methods 50 cases of children with ventricular septal defect were confirm by Colour Doppler Ultrasound. Then selected the appropriate occluder type spare and had an operation under general anesthesia and tracheal intubation.The transesophageal echocardiography probe is inserted through the mouth. Exposed the right ventricle with 3 ~ 4 cm small incision from the xiphoid to the third intercostal level on median sternotomy. And the transesophageal echocardiography was used for occluder closure of ventricular septal defect. In addition,Membranous ventricular septal defect choose occluder diameter larger than the defect diameter of 0 ~ 2 mm; with membranous aneurysm,the selection of the occluder diameter should be 3 ~ 4 mm larger than the diameter of the defect. Conventional heparin anticoagulation and oral aspirin anticoagulation were used after the surgery. Results The Operation time was(62.23±11.23)min. All the closures were successful with no residual shunt and no regurgitation in the aortic valve,mitral valve and tricuspid valve. And the Hospitalization were(9.11±1.84)days. Postoperative follow-up was(24.11±6.42)months; all follow-up to the patients,there were no occluder detachment,infective endocarditis,thromboembolism,sudden death and other serious complications occurred. Conclusion Minimally invasive closure is an effective method for the treatment of ventricular septal defect in infants and young children.%目的:探讨经胸微创婴幼儿室间隔缺损封堵术治疗效果,探讨其临床应用价值。方法室间隔缺损的患儿50例,彩超明确室间隔缺损情况,选取相应的封堵器型号备用,在全麻、气管插管下进行手术,经口腔插入食道超声探头,胸骨正中作上至第三肋间水平下至剑突3~4

  14. Thrombosis and prevention after transcatheter occlusion of atrial septal defect%房间隔缺损介入封堵术后血栓形成及其防治

    Institute of Scientific and Technical Information of China (English)

    刘倩

    2012-01-01

    目前,介入治疗已逐渐取代外科开胸手术成为房间隔缺损首选的治疗方法.但各种类型封堵器植入均有血栓形成报道,并引起脑栓塞、心肌梗死、肺栓塞等严重并发症.封堵器相关血栓形成主要与心房颤动、房间隔膨胀瘤、封堵器类型、凝血功能激活、封堵器内皮化过程等密切相关.该文总结了封堵器血栓形成的相关因素及目前房间隔缺损介入术后各种血栓预防方案,为介入封堵术后血栓防治提供理论基础及实验依据.%Transcatheter closure as an alternative to medical surgery has become a preferred therapeutic method to atrial septal defect (ASD).However,thrombopoiesis after occluder implantation has been reported comprehensively and causes a series of serious complications such as cerebral embolism、myocardial infarction,pulmonary embolism and so on.Reasons of device thrombopoiesis are closely associated with atrial fibrillation,persistent atrial septal aneurysm,occluder type,activated coagulation system and occluder endothelialization process.This paper summarizes correlation factors of occluder thrombopoiesis and current related prevention proposal after transcatheter closure of ASD,and provides theoretical and experimental foundation for prevenition of device thrombopoiesis after occluder implantation of ASD.

  15. Orthodromic atrioventricular reciprocating tachycardia conducted with intraventricular conduction disturbance mimicking ventricular tachycardia in an English Bulldog.

    Science.gov (United States)

    Santilli, Roberto A; Diana, Alessia; Baron Toaldo, Marco

    2012-01-01

    Electrocardiographic tracings of an English Bulldog referred for cardiogenic shock due to an orthodromic atrioventricular reciprocating tachycardia conducted with intraventricular conduction disturbance and mimicking ventricular tachycardia (VT) are presented. At admission the surface ECG showed a wide QRS complex tachycardia (WCT) that was converted to sinus rhythm using manual cardioversion (chest thump). This change revealed pre-existing right bundle branch block, and a final diagnosis of supraventricular tachycardia (SVT) with intraventricular conduction disturbance was made. Electrophysiologic study defined the SVT mechanism as an atrioventricular macroreentrant tachycardia mediated by a single mid-septal accessory pathway. The differentiation between various types of WCT is essential when antiarrhythmic therapy is considered. The surface ECG should be systematically evaluated in order to recognize the characteristic features of SVT and VT. Moreover chest thump procedure can be very helpful in the attempt to convert the rhythm to sinus rhythm and to correctly recognize the underlying arrhythmia.

  16. Mammoth interatrial septal aneurysm in the ICE age

    Directory of Open Access Journals (Sweden)

    Timperley John

    2007-09-01

    Full Text Available Abstract Background Intracardiac echocardiography (ICE is a useful imaging modality that is now being used more widely to assist in the percutaneous closure of atrial septal defects (ASD and patent foramen ovales (PFO. Case presentation A 42 year old lady with a history of transient ischaemic attacks and migraine underwent percutaneous closure of an ASD. Intraprocedural ICE demonstrated a mammoth billowing multiperforated interatrial septal aneurysm in association with a secondum ASD. Conclusion ICE provides excellent adjuvant imaging during percutaneous closure of intracardiac shunts, in this case demonstrating a 'mammoth' interatrial septal aneurysm.

  17. 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

  18. Facts about Gastroschisis

    Science.gov (United States)

    ... Heart Defects Atrial Septal Defect Atrioventricular Septal Defect Coarctation of the Aorta D-Transposition of the Great ... Defects Fetal Alcohol Syndrome Disorders Gastroschisis Heart Defects Coarctation of the Aorta Hypoplastic left heart syndrome Tetralogy ...

  19. Facts about Microcephaly

    Science.gov (United States)

    ... Heart Defects Atrial Septal Defect Atrioventricular Septal Defect Coarctation of the Aorta D-Transposition of the Great ... Defects Fetal Alcohol Syndrome Disorders Gastroschisis Heart Defects Coarctation of the Aorta Hypoplastic left heart syndrome Tetralogy ...

  20. Facts about Hypospadias

    Science.gov (United States)

    ... Heart Defects Atrial Septal Defect Atrioventricular Septal Defect Coarctation of the Aorta D-Transposition of the Great ... Defects Fetal Alcohol Syndrome Disorders Gastroschisis Heart Defects Coarctation of the Aorta Hypoplastic left heart syndrome Tetralogy ...

  1. Facts about Omphalocele

    Science.gov (United States)

    ... Heart Defects Atrial Septal Defect Atrioventricular Septal Defect Coarctation of the Aorta D-Transposition of the Great ... Defects Fetal Alcohol Syndrome Disorders Gastroschisis Heart Defects Coarctation of the Aorta Hypoplastic left heart syndrome Tetralogy ...

  2. Research of cardiac troponin I release after transcatheter closure of atrial septal defect in children%房间隔缺损患儿介入治疗前后肌钙蛋白I变化的研究

    Institute of Scientific and Technical Information of China (English)

    陈轶维; 张志芳; 李奋; 周爱卿; 吉炜; 胡晶晶

    2012-01-01

    Objective To investigate whether transcatheter closure of atrial septal defect makes cardiac troponin I(cTnI) realease in children. Methods Clinical data, laboratory tests, imagings, diagnosis, the information of interventional therapy and postoperative follow-up from 146 children with secundum atrial septal defect who were diagnosed and get treatment of interventional were collected. The median age was(5. 87 ± 3. 48) years( range,2. 25-12. 5) with 87 boys and 59 girls. The device size ranged from 8 to 22 mm. These patients were divided to 2 groups according to the level of cTnI after intervention. The cTnI serum concentrations were examined immediately eafter intervention ,6 hours later,24 hours later and 3 days after intervention. Results The level of cTnI were normal before intervention,and the peak level of cTnI was found at 6 hours after intervention(Z = - 3. 410,P =0. 005) . All of the patients had a normal level of cTnI 3 days after operation ( Z = - 0. 332, P = 0. 74 ) . Univariate analysis showed age, height,device size,size/BSA ratio to be contributing factors for level of cTnI. The device size/body surface area ratio was identified by demonstration positive correlation with cTnI elevation by multivariate logistic regression analysis ( r = 0. 31, P =0. 022 ). Conclusions The transcatheter closure of atrial septal defect in children could lead to reversible increase of cTnI or induce minor myocardial lesion, the extent of which depends on the ratio of device size/ body surface area. Interventional therapy of atrial septal defect is safe and effective for children.%目的 总结并分析儿童继发孔型房间隔缺损患者接受介入封堵治疗前后肌钙蛋白I(cTnI)变化的原因及相关因素,提高临床医师对介入封堵技术治疗儿童房间隔缺损相关适应证及手术风险的了解.方法 收集146例2009年9月至2012年4月在上海儿童医学中心心内科介入封堵的继发孔型房间隔缺损患儿的临床资料、辅

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

    Science.gov (United States)

    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; Louw, Jacoba; 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

    2014-04-01

    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 parent-offspring trios and 112 unrelated individuals with nonsyndromic AVSDs and identified five rare missense variants (two of which arose de novo) in the highly conserved gene NR2F2, a very significant enrichment (p = 7.7 × 10(-7)) compared to 5,194 control subjects. We identified three additional CHD-affected families with other variants in NR2F2 including a de novo balanced chromosomal translocation, a de novo substitution disrupting a splice donor site, and a 3 bp duplication that cosegregated in a multiplex family. NR2F2 encodes a pleiotropic developmental transcription factor, and decreased dosage of NR2F2 in mice has been shown to result in abnormal development of atrioventricular septa. Via luciferase assays, we showed that all six coding sequence variants observed in individuals significantly alter the activity of NR2F2 on target promoters.

  4. 200 Open Heart Operations on Patients with Ventricular Septal Defects%200例室间隔缺损(VSD)手术体会

    Institute of Scientific and Technical Information of China (English)

    李仲智; 郑德珍; 高玲; 郭志和; 高国庆; 陆进; 姜娟; 刘岚

    1989-01-01

    Two hundred open heart operations for VSD were performed between 1979.8 and 1987.5.77% of the patients aged below 5 years,and 68% weighed below 15kg and 16% below 10kg.98 out of 163(60%),on whom cardiac catherization was performed,showed moderate or severe pulmonary hypertension.197 patients su cvived,and 3 died duting hospitalization.Discussions involve:1) The age for operation.Patients with large defects and henrt failure;or repeated infection of respivatory tract,or hyposomia should be operated on earlier,better before the age of 2 years.As for patients with small defects operationts may be delayed to Presehool age.2) The type of operation.The defect with a diameter bigger than 1cm should be patched.The pladget mattress suture gives a better security sealing than the simple running suture does.3) The treatment of the associated cardiac deformities.PDA may be ligated before or after the cardiopulmonary bypass.4) The consi derations of a re-operation.Based on the experiences of 2 successful re-operations,it is suggested that in case of a serious complication responding Poorly to the medicaltreatmeat,a re-operation had better not be hesitated.%作者自1979年8月至1987年5月共进行了200例VSD心内直视手术,其中5岁以下占77%,体重15公斤以下占68%.手术取得良好的效果,存活率达到98.5%. 通过200例VSD手术经验,作者从手术时机的选择,修补方法的选择,合并畸形的处理以及再手术的决定等四个方面谈谈体会.

  5. [Surgical Treatment of Large Muscular Ventricular Septal Defect nearby the Moderator Band Using the Sandwich Method;Report of a Case].

    Science.gov (United States)

    Motokawa, Mamika; Sasahara, Akihiro; Terakawa, Katsunari; Miyamoto, Takashi

    2016-09-01

    We describe the rare case of a 1-year-old girl who had large muscular ventricular defect (VSD) nearby the moderator band. We experienced the patch closure using sandwich method. A 1-month-old girl was referred to our institution for treatment of muscular VSD. At the age of 2 month, she underwent the pulmonary artery banding to control the pulmonary high flow. After follow up, the patient have reached 70 cm tall and weighed 7 kg. One year after the "sandwich operation", cardiac catheterization revealed the tiny residual shunt. Nevertheless, the cardiac function was good and the growth was in fine fettle. Sandwich method is a useful surgical technique to close the muscular VSD without resect the right ventricular trabeculation.

  6. Genetic analysis of a spinocerebellar ataxia family combined with atrial septal defects%脊髓小脑性共济失调合并房间隔缺损家系的遗传学研究

    Institute of Scientific and Technical Information of China (English)

    周丹; 郭洪; 白云; 迟路湘

    2013-01-01

    目的 对本科收治的1个脊髓小脑共济失调(spinocerebellar ataxias,SCA)合并先天性房间隔缺损(atrial septal defects,ASD)家系进行临床资料分析和分子遗传学研究.方法 分别对SCA和ASD两个疾病表型进行临床诊断、家系调查和系谱分析,通过聚合酶链式反应和直接测序的方法对收集到的家系成员分别进行SCA和ASD相关致病基因进行突变检测和共分离分析.结果 该家系4代共有SCA患者15例,其中已故患者5例,呈常染色体显性遗传模式,病史回顾示家系患者均无明确诱因出现行走不稳、饮水呛咳、言语不清等共济失调的临床特征,基因诊断发现该家系患者SCA3致病基因CAG异常扩增.此外,在家系中还发现包括先证者在内的5例先天性ASD患者,对ASD相关基因进行突变筛查发现3例ASD患者均携带有MYH6基因编码区的1个错义突变c.1154C>T,导致第385位氨基酸由丝氨酸变为亮氨酸(Ser385Leu),其余家系成员无此突变.结论 该家系中SCA和ASD 2种独立的疾病表型都分别以常染色体显性遗传的方式传递,并且分别由SCA3的致病基因和MYH6基因的突变导致.%Objective To make a genetic and clinical analysis in an autosomal dominant spinocerebellar ataxia (SCA) family combined with congenital atrial septal defect (ASD).Methods Family investigation,pedigree analysis and clinical diagnosis were performed on the 2 disease phenotypes respectively.Mutation detection of the disease genes associated with SCA and ASD by polymerase chain reaction (PCR) with direct DNA sequencing.Results There were 15 SCA familial patients of 4 generations,including 5 having dead.Both SCA and ASD were inherited in an autosomal dominant mode in this family.They presented walking instability,dysphagia and slurred speech after being 30 years old.Genetic diagnosis demonstrated the CAG repeat sizes of SCA3 causative gene were abnormally expanded.There were 5 ASD patients among these SCA

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

    Directory of Open Access Journals (Sweden)

    Ulisses Alexandre Croti

    2007-05-01

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

  8. Laterality Defects in the National Birth Defects Prevention Study (1998–2007): Birth Prevalence and Descriptive Epidemiology

    Science.gov (United States)

    Lin, Angela E.; Krikov, Sergey; Riehle-Colarusso, Tiffany; Frías, Jaime L.; Belmont, John; Anderka, Marlene; Geva, Tal; Getz, Kelly D.; Botto, Lorenzo D.

    2015-01-01

    Little is known epidemiologically about laterality defects. Using data from the National Birth Defects Prevention Study (NBDPS), a large multi-site case-control study of birth defects, we analyzed prevalence and selected characteristics in children born with laterality defects born from 1998 to 2007. We identified 517 nonsyndromic cases (378 heterotaxy, 73.1%; 139 situs inversus totalis [SIT], 26.9%) resulting in an estimated birth prevalence of 1.1 per 10,000 live births (95% confidence interval 1.0–1.2). Prevalence did not differ significantly across sites, over time, or by inclusion of pregnancy termination. Laterality defects were more common among preterm cases compared to term cases, and in children born to mothers who were non-white or younger than 20 years compared to white mothers or those age 25–29 years. The distribution of associated cardiac and extracardiac defects, excluding the expected heterotaxy anomalies, varied by type of laterality defect. Cases with heterotaxy were significantly more likely than those with SIT to have double outlet right ventricle, atrioventricular canal defects, pulmonary stenosis, non-tetralogy of Fallot pulmonary atresia with ventricular septal defect, totally and partially anomalous pulmonary venous return; also more likely to have orofacial clefts, esophageal atresia, bowel atresias, and omphalocele, though not reaching statistical significance. Relatively more common among cases with SIT were Dandy-Walker malformation, anotia/microtia, and limb deficiency. The similarity in the demographic characteristics of heterotaxy and SIT supports the hypothesis that they are part of a continuum of abnormal left-right axis patterning. These findings on laterality defects may help guide clinical care, future research, and prevention strategies. PMID:25099286

  9. 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

  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. Atrioventricular node and input pathways: a correlated gross anatomical and histological study of the canine atrioventricular junctional region.

    Science.gov (United States)

    Racker, D K

    1989-07-01

    To determine the architecture of the atrioventricular (AV) junctional region, structures in atrial preparations were correlated to those in serial sections made either parallel or perpendicular to the long axis of the AV node (AVN)/AV bundle complex. The results demonstrated the following for the first time: 1) A right medial atrial wall (MAW) extends anteriorly from the interatrial septum, superior to the interventricular septum (IVS). 2) An atrial interventricular septum (A-IVS) groove is located between the base of the MAW and the crest of the IVS. 3) Three atrionodal bundles converge to form a proximal AV bundle (PAVB), which in turn is contiguous with the AVN. The atrionodal bundles are associated with the MAW or the superomedial and inferolateral margins of the coronory sinus. Terminal portions of the atrionodal bundles and the PAVB reside within the A-IVS groove. The AV bundle was termed distal (DAVB) to avoid confusion. 4) The location of the AVN/DAVB complex topographically is deep to the apex of the septal cusp of the tricuspid valve subjacent to the MAW. Intracardially, the AVN/DAVB complex is within the central fibrous body. Significantly, this study resulted in the first unequivocal demonstration of discrete bundles of myocardial fibers associated with the atrial end of the AV node. Moreover, it appears likely that the atrionodal AV bundles are continuous with the sinoatrial nodal extensions, thereby forming internodal tracts.

  12. 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.

  13. Late endocarditis of Amplatzer atrial septal occluder device in a child

    Institute of Scientific and Technical Information of China (English)

    Neerod; K; Jha; Laszlo; Kiraly; John; SK; Murala; Csaba; Tamas; Haitham; Talo; Hazem; El; Badaoui; Magdi; Tofeig; Malaika; Mendonca; Sameer; Sajwani; Mary; A; Thomas; Sura; Ahmed; Al; Doory; Mohammad; D; Khan

    2015-01-01

    Bacterial endocarditis following atrial septal defect closure using Amplatzer device in a child is extremely rare. We report a 10-year-old girl who developed late bacterial endocarditis, 6 years after placement of an Amplatzer atrial septal occluder device. Successful explantation of the device and repair of the resultant septal defect was carried out using a homograft patch. The rare occurrence of this entity prompted us to highlight the importance of a closed long-term follow up, review the management and explore preventive strategies for similar patients who have multiple co-morbidities and a cardiac device. A high index of suspicion is warranted particularly in pediatric patients.

  14. 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...... 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...

  15. Surgical treatment of ventricular septal defect with pulmonary hypertension in infants younger than 1 yearold%1岁以下婴儿室间隔缺损伴肺动脉高压的外科治疗

    Institute of Scientific and Technical Information of China (English)

    许刚; 张镜芳; 庄建; 陈欣欣; 陈寄梅; 岑坚正

    2001-01-01

    目的探讨婴儿室间隔缺损(VSD)伴肺动脉高压(PH)外科治疗的结果和经验。方法对60例1岁以下婴儿室缺伴肺高压的手术治疗进行总结。年龄2~11个月,平均(6.23±2.60)个月,体重3~10 kg,平均(5.50±1.26)kg。术前中度以上肺高压53例(88.8%)。结果全组手术死亡3例(5%),手术死亡的主要原因是肺动脉高压危象和急性心包填塞。术后主要并发症为肺动脉高压危象,术后出血和肺部感染。结论婴儿室缺伴肺高压应尽早手术治疗。术前、术后支持疗法,术中加强心肌保护、提高手术技巧,术后注意呼吸、循环监测,加强呼吸道管理,防治可能出现的并发症是手术治疗成功的关键。%Objective To review the experience of surgical treatment of ventricular septal defect (VSD) with pulmonary hypertension (PH) in infants younger than 1 year old.Methods 60 infants with VSD+PH received operations. Their ages ranged from 2 to 11 months[(6.23±2.60) months]. The mean body weight was [(5.50±1.26)kg]. 53 patients (88.8%) had moderate or severe pulmonary hypertension before operation.Results Three patients died after operation. The operative mortality was 5%. The main reasons were pulmonary hypertensive crisis and acute cardiac tamponade. The main complications were pulmonary hypertensive crisis, bleeding and pulmonary infection. Conclusion Surgical treatment of VSD with PH in infants may have a satisfactory outcome. The improvement of surgical techniques and proper perioperative management are important factors influencing surgical outcome.

  16. 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

  17. Platypnea-orthodeoxia induced by fenestrated atrial septal aneurysm.

    Science.gov (United States)

    Giombolini, Claudio; Notaristefano, Salvatore; Santucci, Stefano; Savino, Ketty; Pasquino, Stefano; Ragni, Temistocle; Ambrosio, Giuseppe

    2005-02-01

    Platypnea-orthodeoxia is a peculiar syndrome characterized by a right-to-left shunt, which occurs in the upright position. The diagnosis is made by contrast transesophageal echocardiography, paying attention to include contrast visualization in the orthostatic decubitus. The association of this syndrome with a fenestrated atrial septal aneurysm is rare and probably underlies a peculiar and also rare mechanism of shunting in presence of normal pulmonary pressure. We report of a case of a 58-year-old man with a fenestrated atrial septal aneurysm and platypnea-orthodeoxia syndrome treated by surgical closure of the atrial defect.

  18. The predictive value of fetal FO/IAS for postpartum secundum atrial septal defect%胎儿卵圆孔与房间隔比值对产后继发孔型房间隔缺损的预测价值

    Institute of Scientific and Technical Information of China (English)

    吴华雯

    2015-01-01

    Objective To investigate the predictive value of ratio (FO/IAS) in fetal for postpartum secundum atrial septal de-fect.Methods The ultrasound examination results of postpartum secundum atrial septal defect (study group, n =42)and postpartum normal newborns (control group, n =458)were retrospective analyzed.The ratio of FO/AS and value of prenatal ultrasonography in di-agnosis were analyzed.Results The ratio of FO/IAS in study group was higher than that in control group( P <0.01);The veracity of FO/IAS ratio in the diagnosis of secudum atrial septal defects were higher than that of conventional form ( P <0.05).Conclusion Fetal FO/IAS ratio can be a useful index for predicting the occurrence of postpartum neonatal secundum atrial septal defect and is wor-thy of clinical application.%目的:探讨胎儿卵圆孔与房间隔比值(FO/IAS)对产后继发孔型房间隔缺损的预测价值。方法回顾性分析我院产科42例产后继发孔型房间隔缺损患儿(观察组)和458例产后正常新生儿(对照组)产前及产后超声检查结果,分析胎儿 FO/IAS 与产前常规超声对疾病的诊断价值。结果观察组胎儿 FO/IAS 高于对照组( P <0.01);胎儿 FO/IAS 检测灵敏度、准确度均高于产前常规形态检测( P <0.05)。结论胎儿 FO/IAS 可在一定程度上预测产后新生儿继发孔型房间隔缺损发生情况,值得临床推广应用。

  19. 小儿膜周部室间隔缺损封堵术后心电图长期随访%Long-term follow-up of electrocardiogram after transcatheter closure of perimembranous ventricular septal defect in children

    Institute of Scientific and Technical Information of China (English)

    王慧深; 李淑娟; 林约瑟; 李轩狄; 李运泉; 孙芸芸

    2012-01-01

    目的 长期随访儿童经导管介入封堵膜周部室间隔缺损(ventricular septal defect,VSD)后心电图(ECG)各值,了解ECG改变规律及其转归.方法 对患膜周部VSD并成功介入封堵的290例患儿进行术后ECG随访.所有患儿术前术后均检查ECG,术后分别于1、3、6个月及1、2、3、5、8年复查ECG,电脑自动测算心率(V)、P-R间期、QRS间期、QT间期及校正的QT(QTc)值,观察心律及ST-T的变化.结果 随访时间:1~96个月[平均(40.16±29.2)个月].行多因素分析发现小儿膜周部VSD引起心电改变的危险因素是年龄在6周岁以内,膜部瘤样VSD,选择封堵器大于VSD直径3 mm以上者.封堵术后290例中115例(39.7%)出现各种轻重不等的ECG改变,进入5年以上长期随访的200例中持续ECG改变者43例(21.5%).但这些患儿均无症状,生长发育良好.随访80例经过应用激素和营养心肌治疗者中仅17例仍存在ECG的改变(21.3%),未经过治疗的35例,持续存在ECG改变者有26例(74.3%).随访5年以上仍需特别关注并定期随访的ECG改变者有25例(25/200例,12.5%).持续ECG改变组与术前组及无ECG改变组的各值对比,均有显著差异.对于出现重度房室传导阻滞(AVB)的患儿及时放置临时起搏器以及激素和营养心肌等治疗的病例预后较好.结论 本组随访提示封堵膜周部VSD慎重选择封堵器型号至关重要.对封堵术后ECG出现异常改变者,应用激素、果糖和维生素等营养心肌治疗,对心律失常的恢复或缓解有帮助.封堵术后早期发生ECG改变大部分轻微或可恢复,对一些明显的ECG改变,随访中未发现有转为三度AVB并影响患儿生长发育的病例,但对于一些ECG值的改变需要更长期随访以了解其转归和预后.%Objective To observe the long-term follow-up data of electrocardiographic (ECG) changes and prognosis after transcatheter closure of perimembranous ventricular septal defect (VSD)in children. Methods 290

  20. Surgical Treatment of Large Ventricular Septal Defect Complicated with Pneumonia in Infants%大型室间隔缺损并肺炎婴幼儿的外科治疗

    Institute of Scientific and Technical Information of China (English)

    马千里; 陈子英; 石凤梧; 陈立华; 梁宜武; 李倩

    2011-01-01

    目的 探讨大型室间隔缺损(VSD)并肺炎婴幼儿早期手术治疗的可行性、手术时机、适应证以及术中术后的处理.方法 选择2009年10月-2010年7月本院收治的大型VSD并肺炎患儿19例.男11例,女8例;年龄3~15(7.3±2.8)个月;体质量5~12(7.6±1.4) kg .其中VSD并房间隔缺损5例、并动脉导管未闭1例;膜周部VSD 16例,肺动脉瓣下VSD 3例.患儿均在低温、体外循环、冷晶体停跳液灌注下行VSD直视修补术及并发畸形矫治.16例膜周部VSD患儿均经右房切口修补;3例肺动脉瓣下VSD患儿中,2例经肺动脉横切口修补,1例经右室流出道切口修补.19例患儿VSD修补均采用补片修补法,其中15例应用牛心包补片,4例应用自体心包补片(戊二醛固定).患儿术中均应用膜式氧合器和术中平衡超滤.结果 19例患儿手术均获成功,术后主要并发症为肺部感染3例,切口愈合不良2例,肺不张和室间隔少量残余分流各1例,无全身感染、肺动脉高压危象以及严重低心排病例,19例患儿均治愈出院.门诊随访2~6个月,上呼吸道感染显著减少,营养发育状况改善.结论 把握手术时机和适应证,早期手术、防治术中和术后肺损伤,是治疗大型VSD 并肺炎婴幼儿的有效方法.%Objective To investigate the feasibility,operation time,indication,management during and after operation of early repair in infants with large ventricular septal defect(VSD) complicated with pneumonia. Methods From Oct. 2009 to Jul. 2010,19 infants [ 11 male and 8 female, aged 3 - 15 (7.3 ± 2.8 ) months, weighted 5- 12 (7.6 ± 1.4) kg ] with large VSD complicated with pneumonia were admitted to the Second Hospital of Hebei Medical University. Five cases were associated with atrial septal defects , 1 case was associated with patent ductus arteriosus; 16 cases were membrana type,3 cases were subpulmonary valve type. All patients underwent cardiopulmonary bypass with systemic hypothermia

  1. 超声评价继发孔型房间隔缺损封堵治疗后心功能变化的价值%Evaluated Transcatheter Closure Effectiveness of Secundum Atrial Septal Defect by Echocardiogram

    Institute of Scientific and Technical Information of China (English)

    范东晨; 李开龙; 王聪; 孟慧敏

    2015-01-01

    目的:探讨经胸超声心动图(TTE)在继发孔型房间隔缺损(ASD)介入封堵治疗前后评价心脏结构及心脏收缩功能变化的价值。方法:选取2012年1-12月在我院成功行介入封堵术,并定期随诊的继发孔型ASD患者47例,分别在术前7d ,术后3d、1个月、3个月、6个月、12个月进行 TTE检查,观察右房左右径(RAD)、右室左右径(RVDd)、右心室长轴缩短分数(RVLFS)、三尖瓣环收缩期位移(TAPSE)、主肺动脉内径(PAD)、左房前后径(LAD)、左室前后径(LVDd);左室射血分数(EF)、短轴缩短分数(FS)、每搏量(SV)的动态改变。结果:与术前比较,术后1、3、6个月RAD、RVDd、PAD逐渐减小,LAD、LVDd、TAPSE、SV、EF、FS、RVLFS逐渐增加,恢复正常大小,以术后1个月及3个月变化较为明显。术后6个月、12个月复查各项指标,无明显变化,趋于稳定。结论:继发孔型房间隔缺损应用T T E介入封堵治疗,创伤小、恢复快,效果确切。对于ASD介入封堵治疗术前病例筛选、术中实时监视、术后定期随访以及封堵治疗后心脏结构及收缩功能的改善状况提供可靠依据。%Objective :To investigate the value of transthoracic echocardiography (TTE) in atrial septal defect (ASD) interventional closure therapy before and after to evaluate changes of cardiac structure and cardiac systolic function . Methods :47 cases of patients with secundum ASD from 2012 January to December in our hospital underwent successful transcatheter closure ,and regular follow‐up .Respectively in the 7 days before operation and after operation 3 days ,1 , 3 ,6 and 12 months examination TTE ,dynamic changes in right atrial diameter (RAD) ,right ventricular diastolic di‐ameter (RVDd) ,right ventricular longitudinal fractional shortening (RVLFS) ,tricuspid annular plane systolic excur‐sion (TAPSE) ,main pulmonary artery diameter (PAD

  2. Ethanol exposure alters early cardiac function in the looping heart: a mechanism for congenital heart defects?

    Science.gov (United States)

    Karunamuni, Ganga; Gu, Shi; Doughman, Yong Qiu; Peterson, Lindsy M; Mai, Katherine; McHale, Quinn; Jenkins, Michael W; Linask, Kersti K; Rollins, Andrew M; Watanabe, Michiko

    2014-02-01

    Alcohol-induced congenital heart defects are frequently among the most life threatening and require surgical correction in newborns. The etiology of these defects, collectively known as fetal alcohol syndrome, has been the focus of much study, particularly involving cellular and molecular mechanisms. Few studies have addressed the influential role of altered cardiac function in early embryogenesis because of a lack of tools with the capability to assay tiny beating hearts. To overcome this gap in our understanding, we used optical coherence tomography (OCT), a nondestructive imaging modality capable of micrometer-scale resolution imaging, to rapidly and accurately map cardiovascular structure and hemodynamics in real time under physiological conditions. In this study, we exposed avian embryos to a single dose of alcohol/ethanol at gastrulation when the embryo is sensitive to the induction of birth defects. Late-stage hearts were analyzed using standard histological analysis with a focus on the atrio-ventricular valves. Early cardiac function was assayed using Doppler OCT, and structural analysis of the cardiac cushions was performed using OCT imaging. Our results indicated that ethanol-exposed embryos developed late-stage valvuloseptal defects. At early stages, they exhibited increased regurgitant flow and developed smaller atrio-ventricular cardiac cushions, compared with controls (uninjected and saline-injected embryos). The embryos also exhibited abnormal flexion/torsion of the body. Our evidence suggests that ethanol-induced alterations in early cardiac function have the potential to contribute to late-stage valve and septal defects, thus demonstrating that functional parameters may serve as early and sensitive gauges of cardiac normalcy and abnormalities.

  3. The clinical application of symmetrical occluder in treating intercristal ventricular septal defect%嵴内型室间隔缺损介入封堵术中对称型封堵器的临床应用

    Institute of Scientific and Technical Information of China (English)

    许美珍; 罗来树; 李颐; 程应樟; 李运德; 吴延庆; 吴清华; 程晓曙; 彭强

    2014-01-01

    Objective To investigate the interventional treatment strategy for occluding the intercristal ventricular septal defect (VSD) in order to improve the surgical safety and success rate. Methods During the period from January 2012 to December 2013, a total of 31 patients with intercristal VSD were admitted to authors’ hospital to receive interventional catheter occlusion therapy. Preoperative color Doppler ultrasound echocardiography showed that on the short axis view of the aorta the VSD interrupted port was situated at 12:00 - 1:00 o’clock region. Left ventricular and above aortic valve angiography indicated that the VSD location, shape and size, the split vent size on the left ventricle side and its distance from the aortic valve could be correctly measured when the VSD shunt was visualized , which were very helpful in guiding the operator to select the suitable occluder as well as to adjust the release pattern of the occluder. Postoperative imaging findings of the left ventricular and above aortic valve angiography were compared with the preoperative ones. Results Successful occlusion of VSD was obtained in 22 patients , in 13 among them the left ventricular angiography showed that the direction of blood flow beam at the defect hole was from the left ventricle to the right ventricle in an obliquely upward direction. The basal width of the defect on the left ventricle side was (5.12 ± 1.38) mm, and(6 - 10) mm occluder was employed. In the remaining 9 patients the left ventricular angiography showed that the direction of blood flow beam at the defect hole was from the left ventricle to the right ventricle in a direction almost parallel to the aortic valve , and the basal width of the defect on the left ventricle side was (7.18 ± 1.26) mm, and (9 - 12) mm zero-bias occluder was adopted. Interventional occlusion of VSD was unsuccessful in 9 cases as the intercristal hole was rather larger, and two of them had coexisting aortic sinus aneurysm complicated by mid

  4. Atrioventricular block and wiry hair in Teebi hypertelorism syndrome.

    Science.gov (United States)

    Han, Xiao-Dong; Cox, Vicki; Slavotinek, Anne

    2006-09-15

    We report on a 4 1/2-year-old girl with clinical features of Teebi hypertelorism syndrome (THS), including a prominent forehead with a widow's peak, heavy and broad eyebrows, hypertelorism, long palpebral fissures, ptosis, a thin upper lip, a grooved chin, and a left preauricular cyst. She required a pacemaker for third degree atrioventricular (AV) block, a finding that has not been previously reported in 36 other patients with THS and for which we were unable to identify other causes. We have reviewed the previous reports of THS and note a characteristic facial appearance with hypertelorism, heavy, broad, and arched eyebrows, a thin upper lip with a long and deep philtrum, and a prominent forehead. Structural cardiac defects were present in five patients, implying that cardiac investigations are warranted in patients with a cardiac murmur and a clinical diagnosis of THS.

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

    NARCIS (Netherlands)

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

    2011-01-01

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

  6. 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.

  7. Application value of echocardiography in perventricular device closure of ventricular septal defect%超声心动图在微创外科室间隔缺损封堵治疗中的应用价值

    Institute of Scientific and Technical Information of China (English)

    康彧; 唐红; 白文娟; 安琪; 赁可; 宋海波

    2011-01-01

    目的 探讨超声心动图在微创外科室间隔缺损(VSD)封堵治疗中的应用价值.方法 经胸超声心动图(TTE)筛选拟行微创外科VSD封堵治疗患者169例,术中经食管超声心动图(TEE)再次评估,对具备封堵条件者进行实时引导和监控.术后3~5 d及3、6、12个月 TTE随访.结果 术中TEE评估对15例不适宜封堵者直接改行VSD修补术.行微创封堵术154例,成功137例,成功率88.9%;封堵失败改行VSD修补术17例.术中即刻观察10例有微量残余分流,3例主动脉瓣有微量反流,7例三尖瓣反流较术前减轻或消失,11例三尖瓣出现少量反流.术后TTE随访封堵器无移位,4例残余分流消失,1例三尖瓣反流较术前略有加重,左心室重构改善.结论 超声心动图在微创外科VSD封堵的术前诊断、术中监控及术后效果评价中均发挥了重要作用.%Objective To explore the application value of echocardiography in perventricular device closure of ventricular septal defect ( VSD ). Methods One hundred and sixty - nine patients with VSD were selected by transthoracic echocardiography ( TTE ) and prepared to receive off pump surgical treatment-perventricular device closure. All the patients were re-evaluated in cardiac surgical procedures by transesophageal echocardiography ( TEE ). The patients suitable to the perventricular device closure were guided and observed. After operation, 3 ~5d,3,6,12 months follow-up were performed by TTE. Results Fifteen cases were not suitable to the device closure by intraoperative TEE re-evaluation and were directly repaired under cardiopulmonary bypass. 137 of 154 cases treated with perventricular device closure were succeeded( 137/154, 88.9% ), 17 cases were failed and received the repair surgery. TEE was performed immediately after occluder release. The tricuspid regurgitation was decreased or disappeared in 7 patients, while the closure caused new mild tricuspid regurgitation in 11 patients, and there were 10

  8. 老年继发孔型房间隔缺损患者封堵前后心电图特点%The characteristics and alterations of ECG in elderly patients before and after occlusion of atrial septal defects

    Institute of Scientific and Technical Information of China (English)

    陈锦铷; 赵明; 韩秀敏; 武琼; 全薇

    2016-01-01

    Objective To summarize the ECG characteristics and atrial fibrillation influences on hemodynamics and cardiac cavity diameter in elderly patients with atrial septal defect before and after occlusion. Methods We record the ECG parameter variations of 114 elderly patients with ASD per-operation and a week after post-operation. Results (1)Peroperative:QRS right axis deviation in 27 cases (23.7%), 75 cases (65.8%) without deviation and left axis deviation in 12 cases (10.5%); V1(V3R) in 82 cases of rsR type (71.9) , CRBBB in 11 cases (9.6%), 2 cases of Rs type (1.8%), 19 cases of rS type (16.7%);9 cases of inversion of TV5-6 (7.9%);30 cases of atrial fibrillation (26.3%), 3 cases of ventricular premature beat (2.6%) and 2 cases of sick sinus syndrome (1.85%). (2)Postoperation:7 cases of QRS right axis deviation turned to normal (25.9%), 3 cases of right ventricular hypertrophy (2.6%) vanished, 3 cases of TV5-6 inversion turned to normal(33.3%). Although there were 2 cases of Af converted to sinus rhythm, 4 new cases of Af occured. (3)Hemodynamics:The mPAP of 114 cases was 10~46(24.6±7.4)mmHg, Af group 13~46(28.2±1.2)mmHg, non-Af group 13~45(24.2±0.8)mmHg , there was no significance difference between them(P>0.05). (4)Echocardiography showed that the left atrial diameter Af group were significantly increased compared with non-Af (P0.05)。(4)超声心动图检测结果显示左心房内径房颤组较非房颤组明显增大(P<0.01)。结论与其他年龄组比较,老年患者术前QRS电轴不偏、左偏、V1(V3R)导联呈CRBBB型和房颤常见;术后QRS电轴右偏转正常(25.9%)和缺血性T波改善(33.3%)较明显,但右室肥厚消退和心房颤动下降率不明显。房颤患者左房内径增大明显,与mPAP无关。

  9. The effect of oversized occluder on endothelialization after percutaneous closure of experimental atrial septal defect in dogs%过大房间隔缺损封堵器对犬术后内皮化的影响

    Institute of Scientific and Technical Information of China (English)

    张婷婷; 张玉顺; 万陈; 成革胜; 王军; 和旭梅; 杜亚娟

    2014-01-01

    目的:探讨过大房间隔缺损封堵器对术后封堵器表面内皮化的影响。方法在经胸超声心动图的引导下,在18只实验犬中建立直径为(6.0±0.2)mm的房间隔缺损模型。采用完全随机化方法将实验犬分为正常组及过大组(每组各9只实验犬),正常组置入直径8 mm封堵器,过大组置入直径12 mm封堵器。术后3、6、14个月分别处死实验犬,通过扫描电镜观察各组封堵器表面的内皮化进程。结果4只实验犬在术后1个月左右死亡。正常组术后3个月封堵器表面已基本内皮化,术后6和14个月均为完全内皮化;过大组术后3个月封堵器表面内皮化程度不佳,术后6个月基本内皮化,术后14个月才完全内皮化。结论采用过大封堵器治疗实验犬房间隔缺损,术后6个月封堵器表面尚未完全内皮化。%Objective To observe the effect of oversized occluder on endothelialization post percutaneous closure of experimental atrial septal defect ( ASD) in dogs.Methods ASD was established with the help of transthoracic echocardiography in 18 dogs.ASD size was ( 6.0 ±0.2 ) mm.Dogs were randomly divided into normal size group ( implanted with 8 mm occlude , n =9 ) and oversized group (implanted with 12 mm occluder, n =9).Dogs were randomly killed at 3, 6 and 14 months after percutaneous closure.The endothelialization process on device surface was observed by scanning electron microscope.Results Four animals died around 1 month post procedure.Microscopic sections from normal group showed nearly complete endothelialization at 3 months after device implantation and complete endothelialization at 6 and 14 months after device implantation.While microscopic sections showed lack of endothelialization at 3 months post implantation , nearly endothelialization at 6 months, and complete endothelialization at 14 months after device implantation in oversized group.Conclusion Incomplete endothelialization of

  10. Alterations in coagulation in children with atrial septal defect before and after treatment with transcatheter closure%儿童房间隔缺损介入术前后凝血机能的变化

    Institute of Scientific and Technical Information of China (English)

    刘倩; 瞿平; 覃军; 易岂建; 李谧; 吕铁伟; 刘晓燕; 蒲晓芳; 计晓娟; 白永红

    2012-01-01

    Objective To investigate the changes in coagulation in children with atrial septal defect ( ASD) after transcatheter closure and the safety of interventional treatment in ASD. Methods Serum levels of prothrombin fragmentl + 2 ( F1 + 2 ) ,β-thromboglobulin ( 3-TG ) , tissue-type plasmingen activator ( t-PA) , D-dimer were measured by enzyme linked immunosorbent assays ( ELISA ) before and after transcatheter closure. Results Compared with the results of pre-occlusion, the levels of serum Fl + 2,t-PA, D-dimer raised by the end of the closure procedure ( P 0. 05 ). The levels gradually returned to the baseline levels 1 months after occlusion. Β-TG levels also raised by the end of the closure procedure (P < 0. 05), and returned to the baseline level at 24 hour after closure. Conclusions The function of coagulation is activated in early and recovered in a short time after transcatheter closure in children with ASD; Aspirin is enough to anticoagulant after occluder theraphy in children with ASD. Interventional treatment in children with ASD is safety and effective.%目的 探讨儿童房间隔缺损(ASD)介入治疗封堵器植入前后凝血机能的变化及ASD介入治疗的安全性.方法 采用酶联免疫吸附法( ELISA)检测19例儿童ASD患者介入封堵前后血浆凝血酶原片段1 +2(F1 +2)、β-血小板球蛋白(β-TG)、血浆组织型纤溶酶原激活物(t-PA)、D二聚体(D-dimer)浓度.结果 与封堵术前比较,血浆F1 +2、t-PA、D-dimer浓度术后即刻明显升高,术后1日较术前水平仍高,但差异无统计学意义,术后1个月基本恢复至术前水平;β-TG术后即刻明显升高,术后1天基本恢复至术前水平.结论 儿童ASD介入封堵术后出现了凝血酶、纤溶系统的激活及血小板的活化,短期内恢复至术前水平;儿童ASD介入术后使用阿司匹林抗凝已足够;介入治疗ASD安全、有效.

  11. 心室间隔膜部及嵴内型缺损动物模型的建立%Animal models of intracristal and membranous ventricular septal defect

    Institute of Scientific and Technical Information of China (English)

    周达新; 管丽华; 王晓彦; 王宜青; 黄国倩; 葛均波; 诸骏仁

    2008-01-01

    BACKGROUND:Surrounding structure of intracristal ventricular septal defects(IVSD) and membranous ventricular septal defects(MVSD)is complicated,and it is difficult to perform transcatheter closure treatment.Corresponding animal models should be established to define an optimal interventional therapy.OBJECTIVE:To prepare the animal models of IVSD and MVSD under echoeardiogram location.DESIGN:Animal modeling experiment.SETTING:Department of Cardiology,Zhongshan Hospital Affiliated to Fudan University.MATERIALS:Six healthy adult dogs,irrespective of gender,were purchased from Shanghai Experimental Animal Center.Aorta and great saphenous vein stapler-puncher(St.Jude corporation,USA)was used.The main improvement is to cut the plastic shell even,thin,or shorter in order to reduce the resistance when puncturing the"purse"in the fight ventficle and the interventficular septum.Sonos 5500 multifunction ultrasonoscope(Philips,USA)was used,and the frequency of the probe was between 2.5-3.5 MHz.METHODS:Experiments were performed at the Zhongshan Hospital Affiliated to Fudan University and Central Laboratory of Shanghai Institute of Cardiovascular Disease from April to July 2006(biosaflety level 2).After anaesthesia,6 adult dogs were fixed at decumbent position.The interventricular septum was punetured to make the preparation with the location of echocardiography(ECHO)by using the modified aorta and great saphenous vein proximal stapler-puncher.Animal intervention met the Animal Ethical Committee of Fudan University.MAIN OUTCOME MEASURES:One week after the surgery,transthoracic echocardiography examination was used to check the VSD site,the highest shunt flow rate,the pressure difference between both sides of the VSD,as well as pulmonary artenal pressure.RESULTS:The VSD models were successfully established in three survivals and included in the final result.Two models of IVSD and one model of MVSD were examined by ECHO one week after the surgery.The diameter of VSD ranges from 1

  12. 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.

  13. Congenital heart defect - corrective surgery

    Science.gov (United States)

    ... Hypoplastic left heart repair; Tetralogy of Fallot repair; Coarctation of the aorta repair; Atrial septal defect repair; ... done in the neonatal intensive care unit (NICU). Coarctation of the aorta repair: Coarctation of the aorta ...

  14. 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.

  15. Septal splint with wax plates.

    Directory of Open Access Journals (Sweden)

    Nayak D

    1995-07-01

    Full Text Available To pack or not to pack, has always been a debate, especially after septal and functional endoscopic sinus surgery. The authors have studied the symptoms of packing versus not packing in their series of 100 patients having undergone nasal surgery. They advocate the use of dental wax for the fashioning of septal splints, since they are easy to introduce, cheap and malleable. The patients postoperative comfort is greatly enhanced with the use of dental wax plate splints instead of nasal packing.

  16. 膜周部室间隔缺损介入治疗术后三尖瓣损伤11例%Tricuspid injury in 11 patients after interventional transcatheter closure for perimembranous ventricular septal defect

    Institute of Scientific and Technical Information of China (English)

    曹黎明; 王凤鸣; 秦玉明; 赵乃琤; 周凯; 莫绪明; 朱善良

    2016-01-01

    Objective To investigate the characteristics of the newly discovered tricuspid injury during the fol-low -up of the patients with interventional transcatheter closure for perimembranous ventricular septal defect (VSD). Methods A retrospective analysis of clinical data was performed in the successful completion of membranous VSD in-tervention from March 2003 to April 2015,and 11 cases of serious tricuspid injury in children were found during the fol-low -up.Results Among 11 children,10 cases underwent surgery again,and in 7 cases of them with pseudoaneurysm of perimembranous septum,the occluders were deviated toward the right ventricul which caused valvular inadequacy for plate winding closures,extrusion and wear of tricuspid chordae and /or leaflet by right ventricul(RV)disc.Among them,1 case was caused by leaflet fusion tear and tricuspid valve insufficiency;3 cases without pseudoaneurysm were caused by a spindle like RV disc recovery structure protruding into the right ventricle,and then the disc squeezed the tricuspid tendinous cord or leaflet and the right ventricular disc nut wrapped tendon of tricuspid valve finally leading to tricuspid valve insufficiency.There was a small amount of tricuspid regurgitation after surgery;the cardiac size and car-diac function returned to normal.One case did not receive surgery,and at present the right atrium and right ventricle were significantly enlarged,and the activity was limited.Conclusions Tricuspid injury discovered newly after perimem-branous VSD interventional therapy is more common in pseudoaneurysm with large tumor occluding the right ventricular disc resulting in poor formation,and it is important to consider these factors in choosing the size of the occluder and the operation.Long term follow -up mechanism should be established for the treatment of VSD after interventional therapy.%目的:探讨膜周部室间隔缺损(VSD)介入术后随访过程中新发现的三尖瓣损伤病例特点,以期发现其

  17. Application of the transthoracic occlusion surgery of atrial septal defect under the guidance of transesophageal echocardiography%食管超声引导下经胸房间隔缺损封堵术的应用研究

    Institute of Scientific and Technical Information of China (English)

    李奎; 张郁林; 辜志英; 周波; 杨卫东; 黄烨; 张秉宜

    2015-01-01

    目的:探讨食管超声引导下经胸房间隔缺损封堵术的可行性及其优越性。方法:选取我科2009年9月至2014年8月经胸及经食管超声诊断为房间隔缺损患者共54例为试验组,选取同期行体外循环下房缺修补术31例患者为对照组,应用SPSS 16.0统计软件在手术时间、呼吸机辅助时间、术后住院天数、伤口大小及心理认可状况方面进行对比研究。结果:试验组手术时间(81.13±15.91) min,呼吸机辅助时间(193.67±72.74) min,伤口大小(3.41±1.13) cm,术后住院天数(3.37±0.49) d,心理认可状况54例均良好。对照组手术时间(109.13±18.19) min,呼吸机辅助时间(425.06±40.67) min,伤口大小(10.78±1.37) cm,术后住院天数(9.61±1.69) d。患者心理认可状况:良好8例,一般10例,较差13例。各项对比结果试验组明显优于对照组( P<0.05)。结论:食管超声引导下经胸房间隔缺损封堵术既缩短手术时间和呼吸机辅助时间,降低术后住院天数和费用,又符合美容要求,患者心理认可状况良好,是一种安全可行的手术方法。%Objective: To explore the feasibility and advantage of the transthoracic occlusion surgery of atrial septal defect ( ASD ) under the guidance of transesophageal echocardiography ( TEE ) .Methods: In experimental group,fifty-four patients with ASD were examined by transthoracic echocardiography and TEE from September 2009 to August 2014 in our hospital.In control group, thirty-one patients were eligible for repairing of ASD under cardiopulmonary bypass.For the comparative study, we used SPSS 16.0 to study these indicators: the operation time, the breathing machine auxiliary time, the postoperative hospitalization days, the wound length and psychological recognition.Results: In experimental group, the operation time was ( 81.13 ±15.91 ) min, the breathing

  18. 小儿先天性室间隔缺损介入治疗心电改变及处理%Change of electrocardiogram and treatment in the children with congenital ventricular septal defect by interventional processes

    Institute of Scientific and Technical Information of China (English)

    王慧深; 李运泉; 陈国桢; 覃有振; 李淑娟; 李格丽; 李渝芬; 钱明阳

    2008-01-01

    目的 分析小儿先天性室间隔缺损(VSD)介入治疗后的心电图(ECG)变化,探索避免严重心律失常的发生.方法 记录260例介入封堵VSD术前后的ECG,据此分成术后心律失常组和无心律失常组(对照组),对发生完全性或重度房室传导阻滞(AVB)的患儿及时营养心肌和减轻心肌水肿治疗,必要时放置临时起搏器.结果 心律失常组82例(31.5%),对照组178例.心律失常组中4例(1.5%)为完全性或重度AVB.36例(13.8%)给予营养心肌等药物处理.两组VSD径比较无显著差异(P>0.05);心律失常组较对照组年龄小,体质量轻(P<0.05),选用封堵器较对照组大(P<0.05).心率、QRS时限、PR间期、QT间期和VTc封堵术前后差异有统计学意义(P<0.05).结论 VSD介入封堵术中和术后需密切注意ECG改变,避免选择过大封堵器.出现完全性右束支传导阻滞(CRBBB)、完全性左束支传导阻滞(CLBBB)、二度AVB者,应尽早营养心肌治疗;完全性或重度AVB者,尽早安装临时起搏器.%Objective To analysis the change of electrocardiogram(ECG)in the children with congenital ventricular septal defect(VSD)by interventional processes and to explore how to decrease the incidence of serious arrhythmias.Methods To record the ECG before and after interventional closure in the 260 patients that hey were divided into arrhythmia group and no arrhythmia group(control group).The patients were treated with nutrition of cardiac muscle and medicines of reducing the cardiac muscle dropsy when they suffered from complete or serious complete or severe atrioventricuhr block(AVB)even underwent the temporary pacemaker quickly.Results There were 178 csses in control group.82 cuses(31.5%)had arrhythmiag after the interventional,in which 4 cases(1.5%)were complete/serious AVB.36 cases(13.8%)were given medicine above.The age was smaller and the body weigh was less in the arrhythmia group than that of control group(P<0.05).The device occluder was bigger in

  19. 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

    Directory of Open Access Journals (Sweden)

    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.

  20. Inlay Approach for the Infant with Ventricular Septal Defect%镶嵌技术治疗婴幼儿室间隔缺损的效果观察

    Institute of Scientific and Technical Information of China (English)

    姜明泽; 游昕; 莫绪明

    2015-01-01

    Objective To investigate the feasibility and safety of inlay approach treatment for infant with ventricular septal defect(VSD).Methods Retrospective analysis on 279 infant from PLA 454th Hospital with VSD who received inlay approach from Jun.2010 to Dec.2013,was done.According to whether cardiop-ulmonary bypass was involved, they were divided into off-pump group ( 201 cases ) , and on-pump group (78 cases) .And 65 cases who received conventional VSD repair on-pump during the same period were included as control group.Operation time, postoperative mechanical ventilation time, intensive care unit (ICU) stay time,postoperative hospital stay,the rate of complications in perioperative period and the total cost of hospitalization of the three groups were compared .Results All of the three groups had no death dur-ing perioperative period.Operation time,postoperative mechanical ventilation time,ICU stay time,postopera-tive hospital stay of off-pump inlay approach group were (1.3 ±0.4) h,(2.5 ±0.8) h,(17.8 ±3.8) h, (6.7 ±1.7) d,which were shorter than those in the control group [(3.0 ±0.5) h,(13.6 ±6.6) h, (38.4 ±4.2) h,(12.0 ±1.8) d] and inlay approach group with cardiopulmonary bypass [(2.7 ±0.4) h, (47.1 ±22.8) h,(58.9 ±26.0) h,(11.6 ±3.0) d],the difference were statistically significant(P <0.05).The rate of complications in perioperative period of off-pump inlay approach group were lower than control group and on-pump inlay approach group(P<0.05).Conclusion Inlay approach has the advanta-ges of good safety,fewer complications for the infant with VSD.%目的:探讨镶嵌技术治疗婴幼儿室间隔缺损( VSD)的可行性与安全性。方法回顾性分析2010年6月至2013年12月解放军第四五四医院心胸外科采用镶嵌技术治疗279例婴幼儿 VSD的临床资料,按是否有体外循环参与分为非体外循环组(201例)、体外循环组(78例)。以同期常规体外循环下VSD修补手术65例作为对照组。对比3

  1. 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

  2. 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

  3. Hybrid procedure for infants with ventricular septal defect and coarctation of aorta: a review of 20 cases%“杂交”手术治疗20例小婴儿主动脉缩窄合并室间隔缺损

    Institute of Scientific and Technical Information of China (English)

    陈纲; 贾兵; 刘芳; 吴琳; 叶明; 成梦遇

    2012-01-01

    Objective To evaluate the early clinical efficaoy of hybrid procedure for infants less than six months old with ventricular septal defect and coarctation of aorta.Methods From January 2010 to July 2011,20 patients with ventricular septal defect and coarctation of aorta received hybrid procedure in our center.The body weight was (4.5 ± 1.6) kg ( ranged from 1.9 kg to 6.5 kg) and the age was ( 56 ± 45 ) days ( ranged from 18 days to 6 months).The pressure gradient of the coarctation of the aorta ranged from 30 mm Hg to 56 mm Hg,5 patients of them were diagnosed as hypoplasty of aortic arch.The size of the ventricular septal defect ranged from 8 mm to 16 mm.Results The mortality was zero in all the 20 cases during the surgery,and the mobidity was 20% (4/20).The complications were pneumonia in 2 cases,infective endocarditis in 1 case and pneumothorax in 1 case.The diameter of coarctation of the aorta ranged from 1.5 mm to 3.4 mm,and the size of the balloon ranged from 4 mm to 12 mm.The pressure gradient of the coarctation of the aorta decreased to 0 to 27 mm Hg.The bypass time ranged from 40 minutes to 87 minutes,and the crossclamp time of the aorta ranged from 20 minutes to 41minutes.The atrial septal defects were repaired and the patent ductuses were ligated during the surgery without leaving the sternum open.The total operation time was (4.0 ± 0.7 ) hours ( ranging from 3.0 hours to 5.2 hours).The mean ventilation time was (2.2 ± 1.4) days and mean ICU stay time was (5 ± 3 ) days.All the patients were followed up for ( 10.0 ± 3.6) months without aneurysm in arch and obstruction in airway.The residual obstructive pressurc gradicnt in the aortic arch ranged from 12 mm Hg to 35 mm Hg and 2 patients received reintervention.One patient received re-balloon dilation and the other received surgery.The cardiac function reached NYHA Ⅰ - Ⅱ in all eases.Conclusion The early outcome of the hybrid procedure (balloon dilation of the coartation of the aorta and surgical

  4. Aorta-pulmonary septal defect and aortic origin of the right pulmonary artery with interruption of the aortic arch: a clinical analysis of 5 cases%主-肺动脉间隔缺损及右肺动脉起源于升主动脉合并主动脉弓离断(附5例报告)

    Institute of Scientific and Technical Information of China (English)

    张刚成; 韩霞; 李艳萍; 陶凉

    2011-01-01

    Objective Reviewing the experience in the diagnosis and treatment of cases with aorta-pulmonary septal defect and aortic origin of right pulmonary artery complicated with interruption of the aortic arch. Methods Reviewed clinical data from medical records for5 patients (4 boys and 1 girl, age from 1 to 18 years on admission) who had been treated in Wuhan Asia Heart Hospital between 2005 and 2009. Results All cases had type A interruption of the aortic arch ( according to Celoria and Patron's Classification) , type Ⅱ aorta-pulmonary septal defect( according to Mori Classification) and aortic origin of right pulmonary artery complicated with interruption of the aortic arch. Large patent ductus arteries were observed in all cases,and no cardiac abnormality such as ventricular septal defect was found. All patients received echocardiography and spiral CT examination, and 3 of them over 10 years of age received cardiac catheterization and angiography for the evaluation of the pulmonary artery pressure. Two patients, at the age of 14 and 18 years, had severe pulmonary hypertension and declined to receive the operation. One of the 2 cases had hemoptysis and was given prostacyclin ( Iloprost solution for inhalation, a drug for pulmonary artery hypertention ) for reducing the pulmonary pressure. The patient now has an improved cardiac fu0ction without recurrent hemoptysis. One 14-year-old case gave up the operation because of the financial problem and failed to communicate with us after discharge. Radical surgery was performed in the remaining three cases, one had serious infection in the lung and died 11 days after the operation, in one case ( 2 years old) pulmonary artery hypertension has been persisted even though drug therapy was given for a long time and was found at a poor cardiac function 18 months after the operation. One patient recovered well under routine drug therapy and has been followed-up. Conclusion Aorta-pulmonary septal defect and aortic origin of right

  5. 76. Profile and spectrum of congenital heart defect in pediatric patient with down syndrome

    Directory of Open Access Journals (Sweden)

    G. Alsuhaibani

    2016-07-01

    Full Text Available Down syndrome is one of the most common chromosomal abnormality worldwide. It occurs in 1 of every 800 live births. Almost one-half of patients with Down Syndrome have congenital heart defect. Our objective is to describe the frequency and spectrum of congenital heart defect (CHD among children with Down Syndrome in Saudi Arabia and identify the rate of primary and secondary pulmonary hypertension among pediatric patients with Down syndrome. Cross-sectional, retrospective study of the cardiac anomalies among 331 pediatric patients (0–18 years with Down Syndrome in King Khalid University Hospital (KKUH from August 2001 till October 2014. The demographic data, reason for referral, echocardiography data including systolic function parameters, the presence of CHD, type and details of CHD, presence of pulmonary hypertension (PHTN, history of cardiac surgeries or transcatheter interventions. Among the 331 pediatric patients with Down Syndrome; 230 patients (69.5% have Congenital Heart Defect (CHD. The patients with CHD were significantly younger (median age 3 months with lower weight (P-value <0.05 and height (P-value <0.05 compared to patients with no CHD. The median age at first assessment was 3 months. The most common type of CHDs among DS pediatric patients was atrial septal defect secundum (ASD II which account for 33.5% of all CHD followed by ventricular septal defect (VSD which account for 26.5%, then atrioventricular septal defect (AVSD 21.7% and moderate to large patent ductus arteriosus (PDA 21.7%. There is another (11.7% who have other CHDs. Pulmonary hypertension was present in 32% of patients with CHD vs 4% among patients with no CHD. There is significant relationship between CHD and pulmonary hypertension with odds ratio 11.3 (CI 3.99–31.83, P-value <0.05. 15% of patients underwent either cardiac surgery or transcatheter intervention. Almost two thirds of Down Syndrome patients have CHD with pulmonary hypertension affecting almost

  6. Intermittent Atrioventricular Block following Fingolimod Initiation

    Directory of Open Access Journals (Sweden)

    E. Gialafos

    2014-01-01

    Full Text Available A 47-year-old female patient with multiple sclerosis (MS developed symptomatic intermittent 2nd degree atrioventricular block (AVB of five-hour duration, five hours after the first two doses of fingolimod, that resolved completely. Frequency domain analysis of heart rate variability (HRV revealed increased parasympathetic activity and decreased sympathetic tone, while modified Ewing tests were suggestive of impaired cardiac sympathetic function. We hypothesize that expression of this particular arrhythmia might be related to autonomic nervous system (ANS dysfunction due to demyelinating lesions in the upper thoracic spinal cord, possibly augmented by the parasympathetic effect of the drug.

  7. 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....

  8. 常见先天性心脏病介入治疗中国专家共识一、房间隔缺损介入治疗%Interventional treatment of common congenital heart diseases: the common view of Chinese medical experts. Part one: Interventional treatment of atrial septal defect

    Institute of Scientific and Technical Information of China (English)

    中国医师协会心血管内科分会先心病工作委员会

    2011-01-01

    房间隔缺损(ASD)约占先天性心脏病的10%,多见于成人女性.绝大多数ASD能够采用介入治疗的方法得到治愈.超声心动图能够明确诊断,准确测量缺损的位置和大小.本文系统介绍ASD介入治疗的适应证、禁忌证、操作方法、封堵器选择与并发症防治.对合并肺动脉高压,多孔型,房间隔膨出瘤型及边缘不好等特殊情况下ASD的处理均进行详细的论述.%Atrial septal defect (ASD) , a congenital heart disease more commonly recognized in female adults. accounts for 10% of all congenital heart diseases. Echocardiographic study can accurately provide the precise information about the size and location of the defect in detail. Most ASDs can be cured by transcatheter device closure. In this chapter, the indications. contraindications, procedures and device sizing for ASDs of interventional management will be systemically described, meanwhile , the complications caused by device occlusion and their preventions will also be discussecl. The treatment for ASD patients accompanied with pulmonary arterial hypertension, multiple-orif'ice defects, aneurysmatic. defects or defects with poor edge will be involved in this chapter. (J Intervent Radiol. 2011, 20: 3-9)

  9. [Acute atrioventricular block in chronic Lyme disease].

    Science.gov (United States)

    Wagner, Vince; Zima, Endre; Gellér, László; Merkely, Béla

    2010-09-26

    The tick bite transmitted Lyme disease is one of the most common antropozoonosis, about 10 000 new infections are reported in Hungary each year. The progress and clinical presentation can vary, and carditis can occur in later stages. A serologically verified Lyme disease caused third degree atrioventricular block in young male presenting with presyncope. Based on the tick-bites mentioned a few weeks prior to hospital admission, Lyme carditis was considered with the administration of antibiotics and monitor observation. Typical skin lesions were not recognized and laboratory findings showed no pathology. An electrophysiological study recorded a predominant supra-His atrioventricular block. Total regression of conduction could be detected later and the serological tests established an underlying Lyme disease. Currently no definite treatment recommendation is available for the potentially reversible Lyme carditis. The tick bite seemed to be the key on our way to diagnosis; however, serological tests proved the disease to be older than one year. A detailed medical history and serological tests are essential in identifying the cause and pacemaker implantation can be avoided.

  10. Fatal septicemia in a patient with cerebral lymphoma and an Amplatzer septal occluder: a case report

    Directory of Open Access Journals (Sweden)

    Stöllberger Claudia

    2011-11-01

    Full Text Available Abstract Introduction The Amplatzer septal occluder is frequently used for percutaneous closure of an atrial septal defect. Complications include thrombosis and embolism, dislocation, cardiac perforation, and, rarely, infection. We report the case of a patient who had survived an occluder-related thromboembolism two years previously. Case presentation A 72-year-old Caucasian woman had received a septal occluder because of an atrial septal defect seven years ago. Two years ago, she underwent chemotherapy of a non-Hodgkin lymphoma, developed atrial fibrillation, and experienced a left-sided occluder thrombosis with stroke and peripheral embolism. Now, she presented with cerebral lymphoma, received glucocorticoids, and subsequently developed skin lesions. Swabs from the lesions and blood cultures were positive for methicillin-resistant Staphylococcus aureus and Pseudomonas aeruginosa. Endocarditis, however, was considered only two months later and echocardiography suggested aortic valve endocarditis. Despite antibiotic therapy, she died three days later because of septicemia, and no post-mortem investigation was carried out. It remains uncertain whether the septal occluder was endothelialized or infected and whether explantation might have changed the outcome. Conclusions If infections occur in patients with a septal occluder, endocarditis should be considered and echocardiography should be performed early. To prevent a fatal outcome, explantation of the septal occluder should be considered, especially in patients with problems that suggest delayed endothelialization. Post-mortem investigations, including bacteriologic studies, should be carried out in patients with a septal occluder in order to assess the focal and global long-term effects of these devices.

  11. Endoscopic closure of large septal perforations with bilateral Hadad-Bassagasteguy flaps.

    Science.gov (United States)

    Morera Serna, Eduardo; Ferrán de la Cierva, Luis; Fernández, Meritxell Tomás; Canut, Santiago Quer; Mesquida, Jacoba Alba; Purriños, Francisco José García

    2017-03-01

    Surgical closure of nasal septal perforations is one of the most challenging procedures in nasal surgery. Defects greater than 2 cm are especially difficult to repair with the traditional approaches due to the challenges of mobilizing enough mucosa to close big gaps, and avoiding airway stenosis in the process. We present a new technique to endoscopically close major septal perforations using bilateral Hadad-Bassagasteguy flaps. Four consecutive patients with septal perforations greater than 2 cm were operated at our institution. Bilateral Hadad-Bassagasteguy flaps were endoscopically raised, one of them above the defect and the other one below it, and rotated to partially cover the defect on each side. A deep temporalis fascia graft was sandwiched between the two flaps to provide a scaffold for schneiderian mucosa growth. Both flaps were covered with a silicone sheet for 3 weeks. A complete closure of the defect was accomplished in three of the four patients; partial closure was achieved in the other. Nasal crusting, epistaxis, and nasal breathing improved in all patients. Large defects of the nasal septum may be closed in selected cases by rotation of bilateral pedicled septal mucosal flaps partially covering the defect area, followed by secondary healing of the nasal mucosa over an autologous graft acting as an epithelial scaffold.

  12. Vector electrocardiographic alterations after percutaneous septal ablation in obstructive hypertrophic cardiomyopathy: possible anatomic causes

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    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.

  13. 单向活瓣补片治疗合并重度肺动脉高压双向分流型室间隔缺损%One - way - valved patch for repair of ventricular septal defect with severe pulmonary hypertension with Bi-directional shunt

    Institute of Scientific and Technical Information of China (English)

    邵双印

    2009-01-01

    目的 总结本院自2003年至2008年用自制单向活瓣补片治疗合并重度肺动脉高压双向分流型室间隔缺损15例的经验.方法 对收治的15例合并重度肺动脉高压双向分流的室间隔缺损患者行单向活瓣补片修补室间隔缺损.结果 本组15例病人无手术死亡,疗效满意,1周时有7例右向左分流,3周时右向左分流消失.随访3个月病人自觉症状改善,肺动脉压下降.结论 单向活瓣补片能降低肺动脉高压危象的发生率,降低围术期死亡率,围术期的管理也非常重要.%Objective To summerize the surgical effect with one - way - valved patch to treat vetricular septal de-feet with severe pulmonary hypertension with bi - directional shunt since 2003. Methods The materials of 15 patients with ventricular septal defect and severe PH with bi- directional shunt who were treated by one- way- valved patch were ana-lyzed. Results No patient died in hospital after operation due to pulmonary hypertention crisis and the low output of left ventrical. Most patients felt better than preoperation and their pulmonary artery pressure decreased gradually. Conclusions One -way -valved patch is useful in decreasing the rate of perioparative pulmonary hypertension crisis and perioperation mortality. The perioperative therapy and prioperative evaluation are also essential to raise surgical operation effect.

  14. Acute left ventricular dysfunction secondary to right ventricular septal pacing in a woman with initial preserved contractility: a case report

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    Gribaa Rim

    2011-10-01

    Full Text Available Abstract Introduction Right ventricular apical pacing-related heart failure is reported in some patients after long-term pacing. The exact mechanism is not yet clear but may be related to left ventricular dyssynchrony induced by right ventricular apical pacing. Right ventricular septal pacing is thought to deteriorate left ventricular function less frequently because of a more normal left ventricular activation pattern. Case presentation We report the case of a 55-year-old Tunisian woman with preserved ventricular function, implanted with a dual-chamber pacemaker for complete atrioventricular block. Right ventricular septal pacing induced a major ventricular dyssynchrony, severe left ventricular ejection fraction deterioration and symptoms of congestive heart failure. Upgrading to a biventricular device was associated with a decrease in the symptoms and the ventricular dyssynchrony, and an increase of left ventricular ejection fraction. Conclusion Right ventricular septal pacing can induce reversible left ventricular dysfunction and heart failure secondary to left ventricular dyssynchrony. This complication remains an unpredictable complication of right ventricular septal pacing.

  15. Effect of continuous positive airway pressure during one lung ventilation on pulmonary function in patients undergoing video-assisted thoracoscopic repair of atrial septal defect%单肺通气期间连续气道正压通气对胸腔镜房间隔缺损修补术患者肺功能的影响

    Institute of Scientific and Technical Information of China (English)

    王磊; 陈宇; 钱燕宁; 丁正年; 孙杰

    2010-01-01

    目的 探讨单肺通气期间连续气道正压通气(CPAP)对胸腔镜房间隔缺损修补术患者肺功能的影响.方法 拟行房间隔缺损修补术的患者20例,年龄16~30岁,体重41~64 kg,性别不限,ASA分级Ⅱ级,随机分为2组(n=10):对照组和CPAP组.两组单肺通气时VT 8 ml/kg,呼吸频率12~16次/min,吸呼比1:2,维持PET CO2 35~40 mm Hg.CPAP组单肺通气期间,非通气侧肺采用CPAP,压力为6 cm H2O.术中监测氧合指数、肺顺应性和气道压,记录拔管时间、单肺通气期间心血管事件和低氧血症的发生情况.结果 与对照组比较,CPAP组氧合指数和肺顺应性升高,拔管时间缩短,低氧血症发生率低(P<0.01),两组患者气道压力在正常范围且未发生心血管事件.结论 单肺通气期间行CPAP(6 cm H2O)可改善胸腔镜房间隔缺损修补术患者的肺功能.%Objective To investigate the effect of continuous positive airway pressure(CPAP)during one lung ventilation on pulmonary function in patients undergoing video-assisted thoracoscopic repair of atrial septal defect.Methods Twenty ASA Ⅱ patients of both sexes,aged 16-30 yr,weighing 41-64 kg,scheduled for video-assisted thoracoscopic repair of atrial septal defect,were randomly divided into 2 groups(n = 10 each): control group and CPAP group.One lung ventilation(VT 8 ml/kg,RR 12-16 bpm,I:E 1:2,PETCO2 35-40 mm Hg)was perform in both groups.CPAP(6 cm H2O)was perform during one lung ventilation in group CPAP.The oxygenation index,pulmonary compliance and airway pressure were recorded during operation.The cardiovascular events,hyoxemia and extubation time were recorded.Results The oxygenation index and pulmonary compliance were significantly higher,extubation time was shorter,and the incidence of hyoxemia was lower in CPAP group than in control group(P < 0.01).The airway pressure was in the normal range in both groups.No cardiovascular events was found in both groups.Conclusion CPAP(6cm H2O)during one lung

  16. Incidental Discovery of a Membranous Ventricular Septal Aneurysm in Two Dissimilar Patients

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    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.

  17. 经胸与经食管超声心动图在室间隔缺损外科封堵中的应用价值%APPLICATION VALUE OF TTE AND TEE IN DEVICE CLOSURCE OF VENTRICULAR SEPTAL DEFECTS

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    张小杉; 哈斯; 吴晓萍; 王晓磊; 朱宪明; 刘志平

    2012-01-01

    目的:评价经胸与经食管超声心动图在室间隔缺损外科封堵中的价值.方法:2009 - 08~2011 - 06经我院心外科确诊的室间隔缺损病人29例.膜周型缺损21例,嵴内型缺损8例.缺损直径3~9mm,平均5.35±3.12mm.术前用经胸超声心动图观察缺损大小及缺损与主动脉瓣的距离.在经食管超声的引导下,经右室游离壁置入国产室缺封堵器.术后用经食管超声即刻评价封堵效果,出院前及封堵3 mo后用经胸超声心动图随访观察.结果:25例封堵成功,使用等边封堵器18例,7例偏心封堵器,术后即刻超声及术后超声观察均无分流信号,2例病人术后出现少量三尖瓣反流.2例膜周型缺损TEE发现缺损处有多处破口无法进行封堵.2例嵴内型封堵失败,即刻术中行开胸修补术.结论:经胸与经食管超声心动图在室间隔缺损外科封堵术前评估、术中监测及术后随访中发挥重要的价值.%objective: To access the application value of TTE and TEE in device closurce of ventricular septal defects. Methods: From August 2009 to June 2011, A total of 29 patients diagnosised with VSD(17 males and 12 females) underwent TTE preoperative assessment in the cardial surgical department in our hospital. 21 cases with Perimembranous VSD,other 8 cases with intracristal type VSD. The defect diameter ranges from 3mm to 9mm,the average value is 5. 35 ± 3.12mm. Used TTE to access defect size and distance from the aortic valve in preoperative. Introduce domestic ventricular septal defect occluder through right ventricular free wall under the guidance of TEE. Immediate evaluate postoperative effect with TEE, TTE was used to follow - up study in the prior to dischargr and three months after. Results:The procedures were successful in 25 cases. Equilateral occluders were used in 18 cases, eccentric occluders in 7 cases. There was no signal of shunt observed by immediate postoperative TEE in the 25 cases,2 patients had mild

  18. Medical assessment of atrial septal defect in fighter pilots —2 cases attached%歼击机飞行员继发孔型房间隔缺损医学鉴定的探讨——附二例临床病例

    Institute of Scientific and Technical Information of China (English)

    崔丽; 郑军; 徐先荣; 李利; 熊巍; 刘晶; 付兆君; 徐蜀宣; 刘红巾

    2012-01-01

    Objective To explore the treatment,aviation medical follow-up and assessment principles of secundum atrial septal defect cases in fighter pilots.Methods The clinical treatment,aviation assessment process and the assessment conclusion of two cases of fighter pilot with secundum atrial septal defect were analyzed and related literature were reviewed.Results The two fighter pilots were casually discovered having secundum atrial septal defect during annual physical examination with no clinical syndromes or signs.The transesophageal echocardiography showed that the defects were less than 10 mm in diameter and the sizes of the heart chambers were normal.The brain images showed that there was no infarction caused by paradoxical embolization.Both pilots successfully underwent percutaneous closure of the defect by using Amplatzer device.By 9~13 months follow-up,the echocardiography showed normal heart functions and no residual shunt.Also no arrthymia was detected by ambulatory electrocardiogram.The Amplatzer devices were stable and the heart functions were normal in human-centrifuge examination and pressure breathing test.Both pilots were wavered for flight.One had gotten the waiver flying with other qualified pilot for 120 h then resumed to his normal duties due to his proper adaptation and normal heart rhythm in air.The other one was still in the limit of co-flying.Conclusion The fighter pilots may get waiver permission to fly as they have successful closure of secundum atrial septal defect and with stable cardio-hemodynamic.The evaluation of flight adaptability should include not only routine postoperative auxiliary examinations,such as echocardiography,ambulatory electrocardiogram and myocardium enzyme studies,but also the examinations that reflect flight endurance,such as sub-maximal exercise test,human centrifuge test and pressure breathing test.Flying certification should be given carefully and progressively.Appropriate aerial physiological monitoring may be

  19. Genetic abnormalities in FOXP1 are associated with congenital heart defects.

    Science.gov (United States)

    Chang, Sheng-Wei; Mislankar, Mona; Misra, Chaitali; Huang, Nianyuan; Dajusta, Daniel G; Harrison, Steven M; McBride, Kim L; Baker, Linda A; Garg, Vidu

    2013-09-01

    The etiology for the majority of congenital heart defects (CHD) is unknown. We identified a patient with unbalanced atrioventricular septal defect (AVSD) and hypoplastic left ventricle who harbored an ~0.3 Mb monoallelic deletion on chromosome 3p14.1. The deletion encompassed the first four exons of FOXP1, a gene critical for normal heart development that represses cardiomyocyte proliferation and expression of Nkx2.5. To determine whether FOXP1 mutations are found in patients with CHD, we sequenced FOXP1 in 82 patients with AVSD or hypoplastic left heart syndrome. We discovered two patients who harbored a heterozygous c.1702C>T variant in FOXP1 that predicted a potentially deleterious substitution of a highly conserved proline (p.Pro568Ser). This variant was not found in 287 controls but is present in dbSNP at a 0.2% frequency. The orthologous murine Foxp1 p.Pro596Ser mutant protein displayed deficits in luciferase reporter assays and resulted in increased proliferation and Nkx2.5 expression in cardiomyoblasts. Our data suggest that haploinsufficiency of FOXP1 is associated with human CHD.

  20. Successful retrieval of a Figulla Occlutech septal occluder - embolized device stability and potential solutions

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    Masood Sadiq

    2016-01-01

    We report a case of an atrial septal defect where a Figulla Occlutech device embolized into the right ventricle. As this device has no left atrial hub, we brought the bare device into the inferior vena cava and used a novel two-snare technique to slenderize the device into the sheath. This report highlights additional tips in transcatheter device retrieval.

  1. Intraoperative blood oxygenation continuous pulmonary artery perfusion lung protection of ventricular septal defect combined children with pulmonary hypertension%术中氧合血持续肺动脉灌注对室间隔缺损合并肺动脉高压患儿的肺保护作用

    Institute of Scientific and Technical Information of China (English)

    章晔; 李春玲; 胡建明; 林庆; 陈艰

    2014-01-01

    目的:研究在体外循环手术中使用氧合血进行持续肺动脉灌注对合并肺动脉高压的室间隔缺损患儿的肺保护作用。方法30例室间隔缺损合并肺动脉高压的患儿,随机均分为试验组和对照组。均在体外循环下进行室间隔缺损修补手术。试验组在体外循环中采用氧合血持续肺动脉灌注,对照组未行肺动脉灌注。两组患儿在体外循环前,主动脉开放后6、12、24h时抽取动脉血3ml.采用酶联免疫吸附法(ELISA法)检测丙二醛(MDA)的水平。并抽取动脉血进行血气分析,计算体外循环前,主动脉开放后6、12、24h时的氧合指数(QI),并记录气道峰压和呼吸机辅助时间,进行统计分析。结果试验组术后12、24h的 QI高于对照组(P<0.05),试验组术后6、12h的气道峰压明显低于对照组(P<0.05)。试验组术后呼吸机辅助时间明显低于对照组(P<0.05)。试验组术后6、12、24h时MDA水平均低于对照组(P<0.05)。结论氧合血持续肺动脉灌注能减轻室间隔缺损合并肺动脉高压患儿在体外循环中的肺损伤。%Objective To study the effects of blood oxygenation in extracorporeal circulation operation on continuous pul-monary artery perfusion of merger pulmonary hypertension of ventricular septal defect with pulmonary protection. Methods 30 cases of ventricular septal defect combined pulmonary hypertension were randomly divided into experimental group and control group,15 cases in each group. Both ventricular septal defect repair surgery under extracorporeal circulation. Patients with blood oxygenation in extracorporeal circulation continuous pulmonary artery perfusion, the control group no pulmonary artery perfusion. Two groups of children in front of the extracorporeal circulation, aorta open after 6,12 and 24 h when extracting arterial blood 3 ml. By using enzyme-linked immunosorbent (ELISA) to detect malondialdehyde (MDA

  2. Septal Junctions in Filamentous Heterocyst-Forming Cyanobacteria.

    Science.gov (United States)

    Flores, Enrique; Herrero, Antonia; Forchhammer, Karl; Maldener, Iris

    2016-02-01

    In the filaments of heterocyst-forming cyanobacteria, septal junctions that traverse the septal peptidoglycan join adjacent cells, allowing intercellular communication. Perforations in the septal peptidoglycan have been observed, and proteins involved in the formation of such perforations and putative protein components of the septal junctions have been identified, but their relationships are debated.

  3. Atrioventricular valve replacement in functional single-ventricle patients

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    ZHANG Zhong; CEN Jian-zheng; XU Gang; WEN Shu-sheng; CHEN Ji-mei

    2016-01-01

    Background This article summarized the experience of atrioventricular valve replacement operation for functional single ventricle patients whose atrioventricular valve was insufficiency.The follow-up time of operation was arranged from early-to medium-term.Methods From July 2009 to July 2015,there were 40 patients of functional single ventricle receiving the operation of atrioventricular valve replacement,including 23 male and 17 female patients.The age at A-Ⅴ valve replacement was ranged from 6 days to 32 years (the medium age of 9 years),and the body weight ranged from 2.7 to 57 kg (the medium body weight of 21.5 kg).Moreover,the atrioventricular valve replacement was performed on all patients under extracorporeal circulation.Results Atrioventricular valve replacement was performed on 40 patients with function single ventricle in hospital,of which eight (20.0%) died in hospital including two neonate and infant (age range:6 days-2 years old) accounting for 25% (2/8),Among them,seven patients died of low cardiac output syndrome two patients with repeated hypoxemia concurrent infection after operation,and one with bilateral diaphragmatic paralysis and malignant arrhythmia.For the patients suffering the severe perivalvular leakage,there was a reoperation,and the cardiac function before the first operation was class Ⅳ.One patient received heart transplantation finally for severe heart failure after AV valve replacement.For the rest of patients,their cardiac function recovered to class Ⅰ-Ⅱ after operation.Conclusions For function single ventricle patients with atrioventricular valve regurgitation,the mortality rate of atrioventricular valve replacement can be accepted if the operation was indicated.For patients with severe atrioventricular value regurgitation,the result of the operation in the early and medium term was satisfying.Thus,the atrioventricular valve replacement served as a useful treatment for patients diagnosed of A-Ⅴ valve regurgitation and

  4. Morphological aspects of atrioventricular valves in the ostrich (Struthio camelus

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    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.

  5. Late erosion of Amplatzer septal occluder device resulting in cardiac tamponade.

    Science.gov (United States)

    Tchantchaleishvili, Vakhtang; Melvin, Amber L; Ling, Frederick S; Knight, Peter A

    2014-12-01

    Transcatheter device closure of atrial septal defects (ASDs) is a minimally invasive technique that offers an alternative to conventional surgical repair. There are risks imposed by this technique; however, they compare favourably with risks of surgical closure. Here, we present a case of a 59-year old male with late erosion of an Amplatzer septal occluder device resulting in cardiac tamponade 5 years after device placement. To the best of our knowledge, cardiac tamponade this late after device placement has not yet been reported. Septal occlusion device erosion remains a major issue among the risks imposed by device closure of an ASD. More data are needed to better understand its true causes and possible solutions.

  6. Brucella Infection Associated with Complete Atrioventricular Block

    Science.gov (United States)

    Bilici, Meki; Demir, Fikri; Yılmazer, Murat Muhtar; Bozkurt, Fatma; Tuzcu, Volkan

    2016-01-01

    Background: 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. Case Report: 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. Conclusion: 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. PMID:27761286

  7. 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.

  8. Tratamento cirúrgico da rotura do septo interventricular pós infarto agudo do miocárdio Surgical treatment of ventricular septal defect post acute myocardial infart

    Directory of Open Access Journals (Sweden)

    Luiz Fernando Leite Tanajura

    1987-08-01

    Full Text Available A rotura do septo interventricular (RSI reduz a perspectiva de sobrevida do paciente com infarto agudo do miocárdio (IAM. Entre 1968 e 1987, atendemos 48 pacientes (p com este diagnóstico, sendo 16 (33% mantidos clinicamente e 32 (67% submetidos a cirurgia. Todos os p clínicos faleceram durante a internação. Dentre os p operados, o IAM se localizava na parede anterior em 18 (56% e na inferior em 14 (44%, com mortalidades respectivas de 6 (33% e 4 (29%. Disfunção ventricular esquerda severa (Killip III e IV foi encontrada em 26 (81%. Em 18 (56% p foi introduzido o cateter de Swan-Ganz. Dos 31 (97% p que se submeteram a cinecoronariografia, 22 (71% apresentavam lesão uniarterial e 9 (29%, doença multiarterial. Entre os p com lesões isoladas, a descendente anterior foi acometida em 15 (68%, a coronária direita em 6 (27% e a circunflexa em 1 (5%. A cirurgia foi realizada nas 2 primeiras semanas de evolução em 8 (25% e, após este período, em 24 (75%, com mortalidades de 6 (75% e 4 (17%, respectivamente. O balão intra-aórtico foi usado em 7 (22%. Os procedimentos cirúrgicos associados ao fechamento da RSI foram revascularização miocárdica em 10 (31%, aneurismectomia de VE em 17 (53% e infartectomia e 6 (19%. Na evolução tardia, dos 22 sobreviventes, 14 (64% encontram-se assintomáticos, 1 (5% sintomático, ocorreram 4 (18% óbitos e perdeu-se a evolução de 3 (13%. Concluímos que, apesar do risco cirúrgico, a cirurgia precoce ainda é a melhor opção terapêutica desta complicação.The ventricular septal rupture (VSR following acute myocardial infarction (AMI is a rare and catasthrophic complication with a high mortality rate. Surgery is the only effective procedure to try to control this complication. However the surgical mortality is elevated and is closely related to the post-infarct period thus making the indication for the precise time for surgery one the fundamental aspects for its success. In a population of 48

  9. Ventricular septal rupture imaged with real-time three-dimensional transesophageal echocardiography: diagnosis at a glance.

    Science.gov (United States)

    Squeri, Angelo; Conti, Rita; Bosi, Stefano

    2014-01-01

    Ventricular septal rupture is a rare complication of acute myocardial infarction and its diagnosis can be really challenging especially in the case of complex lesions. Echocardiography is the technique of choice for the detection of mechanical complications following myocardial infarction. The introduction of three-dimensional echocardiography offers new imaging possibilities with precise localization and easiest definition of the defect anatomy. This information is of paramount importance in the setting of a percutaneous closure procedure. We describe a case where real-time three-dimensional transesophageal echocardiography correctly defined the complex anatomy of a postmyocardial infarction septal defect with an associated pseudoaneurysm.

  10. Combining rhinoplasty with septal perforation repair.

    Science.gov (United States)

    Foda, Hossam M T; Magdy, Emad A

    2006-11-01

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

  11. Individual contrast medium injection protocol for coronary CT angiography in atrial septal defect patients%房间隔缺损患者行冠状动脉CTA检查的个性化对比剂注射方案研究

    Institute of Scientific and Technical Information of China (English)

    宋会军; 王宏宇; 侯志辉; 吴言伶; 韩磊; 吕滨

    2012-01-01

    目的:探讨房间隔缺损患者行冠状动脉CTA检查的理想对比剂注射方案.方法:将连续90例拟行介入治疗或外科手术治疗中老年房间隔缺损患者,于术前行冠状动脉CTA检查,依据不同的对比剂注射方案将患者随机分为3组.A组:单期相注射方案(仅注射基础量对比剂,不注射生理盐水);B组:双期相注射方案(先注射基础量对比剂,后以40mL生理盐水冲洗);C组:三期相注射方案(先注射基础量对比剂,后注射30%生理盐水与对比剂的混合物30mL,最后注射40mL生理盐水冲洗).基础量对比剂按1mL/kg计算,注射流率均为5.0mL/s.比较3组方案CTA图像上冠状动脉及左、右心系统的CT值及房间隔缺损的可评估性.结果:3组患者在平均年龄、体重及心率方面差异无显著性意义.各组患者冠状动脉、升主动脉及左心房、左心室的CT值差异无显著性意义(P>0.05),但各组右心房、右心室及肺动脉的CT值均数大于250HU;此外,B组对比剂用量明显低于其它2组(P<0.05),房间隔缺损量化评分高于C组(P<0.05).结论:双期注射方案由于对比剂用量减少,利于房间隔缺损评估,因而是房间隔缺损患者行冠状动脉CTA检查的理想的对比剂注射方案.%Objective:To determine the individual protocol for dual source CT contrast enhanced cardiac imaging for better visualization of both coronary artery and atrial septal defect. Methods; A total of ninety consecutive patients were in eluded in this prospective study. Each 30 patients were assigned to use a different contrast protocol: group Ⅰ: monophasic protocol (basic contrast injection without saline chaser) with a single syringe injector; group Ⅱ: biphasic protocol (basic contrast injection + saline chaser 40mL) with a dual syringe injector; group Ⅲ:triphasic protocol (basic contrast media+ 30% saline to contrast medium mixture 30mL+ saline chaser 40mL) with a dual syringe injector. The basic contrast media was

  12. 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.

  13. 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;

    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...

  14. "Distal common pathway in atrioventricular node reentrant tachycardia "

    Directory of Open Access Journals (Sweden)

    "Moghaddam M

    2001-06-01

    Full Text Available Anotomical boundary of atrioventricular node reentrant tachycardia (AVNRT is composed of fast and slow pathways right atrium in upper turnaround and common distal pathway in lower turnaround. We performed electophsiologic study (EPS in 152 patients and could show the existence of distal common pathway with decremental conduction properties in approximately 40 patients.

  15. Atrioventricular nodal reentrant tachycardia treatment using novel potential.

    Science.gov (United States)

    Ardashev, Andrey V; Makarenko, Alexandr S; Zhelyakov, Eugeny G; Shavarov, Andrey A

    2010-12-01

    Radiofrequency ablation of atrioventricular nodal reentrant tachycardia is commonly guided by slow and sharp bipolar potentials of the atrioventricular slow nodal pathway. We optimized the morphology of the guiding potential by unipolar mapping of the slow nodal pathway. We identified a novel unipolar dual-component atrial electrogram at the anterior limb of the coronary sinus ostium. The first component was a positive delta-wave type that corresponded to the isoelectric phase on a bipolar electrogram. The second component had fast biphasic morphology and corresponded to the R wave on a bipolar atrial electrogram. Of 104 consecutive patients with typical atrioventricular nodal reentrant tachycardia, 51 were treated with ablation guided by the novel potential, and 53 underwent ablation using the conventional technique. There was no recurrence of tachycardia in any of these patients. In those treated by the novel potential, there was significantly less radiofrequency power applied and a shorter duration of application than in patients treated by the traditional approach. The novel approach to mapping and ablation of the slow nodal pathway in atrioventricular nodal reentrant tachycardia guided by unipolar recording was safe and effective, and comparable to the traditional technique.

  16. INFERIOR-SEPTAL MYOCARDIAL INFARCTION MISDIAGNOSED AS ANTERIOR-SEPTAL MYOCARDIAL INFARCTION: ELECTROCARDIOGRAPHIC, SCINTIGRAPHIC, AND ANGIOGRAPHIC CORRELATIONS

    Institute of Scientific and Technical Information of China (English)

    Ji-lin Chen; Zuo-xiang He; Zai-jia Chen; Jin-qing Yuan; Yue-qin Tian; Shu-bin Qiao; Rong-fang Shi; Yi-da Tang; Zong-lang Lu

    2007-01-01

    To explore the infarct sites in patients with inferior wall acute myocardial infarction (AMI) concomitant with ST segment elevation in leads V1-V3 and leads V3R-V5R.Methods Five patients diagnosed as inferior, right ventricular, and anteroseptal walls AMI at admission were enrolled. Electrocardiographic data and results of isotope 99mTc-methoxyisobutylisonitrile (MIBI) myocardial perfusion imaging and coronary angiography (CAG) were analyzed.Results Electrocardiogram showed that ST segment significantly elevated in standard leads Ⅱ, Ⅲ, aVF, and leads V1-V3 , V3R-V5R in all five patients. The magnitude of ST segment elevation was maximal in lead V1 and decreased gradually from lead V1 to V3 and from lead V1 to V3R-V5R. There was isotope 99mTc-MIBI myocardial perfusion imaging defect in inferior and basal inferior-septal walls. CAG showed that right coronary artery was infarct-related artery.Conclusions The diagnostic criteria for basal inferior-septal wall AMI can be formulated as follows: ( 1 ) ST segment elevates ≥2 mm in lead V1 in the clinical setting of inferior wall AMI; (2) the magnitude of ST segment elevation is the tallest in lead V1 and decreases gradually from lead V1 to V3 and from lead V1 to V3R-V5R. With two conditions above, the basal inferior-septal wall AMI should be diagnosed.

  17. The clinical value of spatio-temporal image correlation in the diagnosis of fetal ventricular septal defect%四维超声时间-空间关联成像对孕期胎儿室间隔缺损诊断的临床价值

    Institute of Scientific and Technical Information of China (English)

    贾立娟; 王智培

    2016-01-01

    Objective To explore the clinical value of four-dimensional ultrasound spatio-temporal correlation image (STIC) in the identification of the diagnosis room septal defect (VSD) in prenatal.Method Selected Court Ultrasound 2011 to 2015 by the two-dimensional ultrasound screening technology and STIC get 38 cases of fetal VSD were retrospectively analyzed to or after the final autopsy results or postnatal follow-up results as the gold standard to calculate two STIC-dimensional ultrasound and clinical diagnostic value of the VSD.Result STIC sample inspection time for each case, each image to obtain the required time were significantly shorter than the two-dimensional ultrasound (P<0.05); 36 patients were diagnosed VSD fetuses, STIC correctly diagnosed 35 cases, one case of missed diagnosis, misdiagnosis does not appear case; correct dimensional ultrasound fetal diagnosis VSD in 28 cases, 8 cases of missed diagnosis, one case of misdiagnosis; calculated according to table 2 to give STIC fetal diagnosis VSD sensitivity of 97.22%, specificity of 100%, misdiagnosis rate was 2.78%, misdiagnosis rate 0.00%; two-dimensional ultrasound diagnosis of fetal VSD sensitivity of 77.78% and a specificity of 50.00%, 22.22% rate of missed diagnosis, misdiagnosis rate was 50.00%.ConclusionPrenatal screening for VSD application STIC technology has the advantages of fast and accurate.%目的探讨四维超声时间-空间关联成像(spatio-temporal image correlation,STIC)技术在产前胎检中鉴别诊断室间隔缺损(ventricular septal defect,VSD)的临床价值。方法选取本院超声科2011年3月至2015年4月经二维超声和STIC技术筛查得到的38例VSD胎儿资料进行回顾性分析,以最终引产后尸体解剖结果或出生后随访结果作为金标准,分析二维超声和STIC诊断VSD的临床价值。结果 STIC对每例样本检查时间、获取每幅图像所需要时间均显著短于二维超声(P<0.05);36例

  18. Determination of the optimal atrioventricular and interventricular delays in cardiac resynchronization therapy

    Institute of Scientific and Technical Information of China (English)

    Hongxia NIU; Wei HUA; Shu ZHANG; Fangzheng WANG; Keping CHEN; Xin CHEN

    2005-01-01

    In order to provide the maximum benefit of cardiac resynchronization therapy (CRT), we tried to use an echocardiography method to optimize the atrioventricular and interventricular delay. Methods The study included 6 patients who underwent implantation of biventricular pacemakers for drug-resistant heart failure. Two-dimensional echocardiography and tissue Doppler imaging were carried out before and after the pacemaker implantation. The optimal AV delay was defined as the AV delay resulting in maximum timevelocity integral (TVI) of transmitral filling flow, the longest left ventricular filling time (LVFT) and the minimum mitral regurgitation(MR). The optimal VV delay was defined as the VV delay producing the maximum LV synchrony and the largest aortic TVI. Results CRT was successfully performed in all patients. After pacemaker implantation, an acute improvement in left ventricular ejection fraction (LVEF) was observed from 26.5% to 35%. Meanwhile, the QRS duration decreased from 170ms to 150ms. The optimal AV delay was programmed at 130, 120, 120, 120, 150 and 110ms respectively with heart rate corrected, LVFT significantly lengthened and TVI of MR decreased (non-optimal vs optimal AV delay: LVFT: 469ms vs 523ms; TVI of MR: 16.43cm vs 13.06cm, P<0.05). The optimal VV delay was programmed at 4, 4, 4, 8, 12 and 8ms with LV preactivation respectively. Programming the optimal VV delay increased the aortic TVI from 17.33cm up to 21.42cm (P<0.05). In the septal and lateral wall, peak systolic velocities improved from2.70cm/s to 3.02cm/s (P>0.05) and froml.31cm/s to 2.50cm/s (P<0.05) respectively. The septal-to-lateral delay in peak velocity improved from 56.4ms to 13.3ms after CRT (P<0.01). Conclusions Optimization of AV and VV delays may further enhance the efficacy of CRT. However, there was interindividual variability of optimal values, warranting individual patient examination.

  19. [Asymptomatic myxoma of the tricuspid valve septal leaflet].

    Science.gov (United States)

    Jedliński, Ireneusz; Jamrozek-Jedlińska, Maria; Bugajski, Paweł; Kalawski, Ryszard; Poprawski, Kajetan; Słomczyński, Marek

    2012-01-01

    We presented a case of asymptomatic myxoma of the tricuspid valve septal leaflet. The tumour was diagnosed accidentally during rutine transthoracic echocardiography and confirmed by transesophageal echocardiography. It was resected and the septal leaflet repaired during surgery.

  20. 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)....

  1. The study on the effect of single disease charges on the use of consumable materials in transcatheter closure of atrial septal defect%按病种收费方式对房间隔缺损封堵术中耗材使用的影响研究

    Institute of Scientific and Technical Information of China (English)

    张超群; 陈默蓉; 侯宇; 杨涵; 张凤勤

    2016-01-01

    Objective:To discuss on the effect of single disease charging mode on the cost of material consumption by using medical consumable material.Methods: Based on the use of the materials of the disease by reviewing the historical cost of diseases, the type and reasonable quantity of the materials used in the disease are consulted by Delphi method.Results: Through analyzing the cost of the disease, the cost of materials accounted for 43.7% in the total cost. According to the experts’ advice, we make a list of medical consumable materials of atrial septal defect closer by 13 valid questionnaires, in order to regulate the use of supplies and reduce the cost of diseases.Conclusion: Single disease charges will encourage hospitals to focus on the control of medical services to control output and outcome indicators and promote the hospitals to reduce the cost of medical services.%目的:以医用耗材为切入点探讨按病种收费方式对病种耗材费用和使用的影响。方法:回顾病种历史费用,以房间隔缺损封堵术耗材使用范围为基础,通过德尔菲法征询专家对该病种耗材使用的种类和合理数量。结果:分析病种费用,其中材料费占全部费用的43.7%。通过15份问卷调查,其中12份有效问卷,结合专家意见制定房间隔缺损封堵术耗材使用清单。以此规范耗材使用,降低病种成本。结论:按病种收费方式将激励医院对于医疗服务的监管重心转向控制产出和结果指标,促使医院自主降低医疗服务成本。

  2. 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.

  3. Occurrence and outcome of tricuspid regurgitation after transcatheter closure of perimembranous ventricular septal defect%膜周部室间隔缺损经导管封堵术后三尖瓣反流的发生与转归

    Institute of Scientific and Technical Information of China (English)

    马进举; 姚青; 宋治远; 仝识非; 景涛; 李永华; 张倩; 张志辉; 李华康

    2013-01-01

    目的 探讨膜周部室间隔缺损(perimembranous ventricular septal defect,PMVSD)经导管封堵术后三尖瓣反流的发生与转归.方法 选取2007年1月至2012年3月在我院心内科诊断为PMVSD并成功行经导管介入封堵治疗的患者332例,其中男182例,年龄(15.4±12.3)岁.分别在经导管封堵术前、术后3天及1、3、6、12个月行经胸超声心动图(transthoracic echocardiography,TTE)检查,观察有无三尖瓣反流及其程度.并根据所用封堵器类型分为2组:对称型封堵器组和细腰型封堵器组,并比较两组三尖瓣反流的发生率.结果 332例患者中181例使用对称型封堵器,另151例使用细腰型封堵器.术后共有14例(4.2%)新出现三尖瓣反流或原有三尖瓣反流加重,其中对称型封堵器组8例(4.4%),细腰型封堵器组6例(4.0%),二者之间无明显差异(P=0.84);随访时间(12.1±7.9)个月,有3例患者三尖瓣反流程度较出院时减轻.无因三尖瓣腱索断裂或瓣膜毁损导致三尖瓣关闭不全的患者.结论 PMVSD经导管封堵术后少数患者可发生三尖瓣反流,其发生与封堵器类型无关,反流程度一般不随时间推移而加重,预后较好.%Objective To investigate the occurrence and outcome of tricuspid regurgitation after transcatheter closure of perimembranous ventricular septal defect (PMVSD).Methods From January 2007 to March 2012,332 patients diagnosed as PMVSD and successfully underwent transcatheter interventional therapy in the Southwest Hospital were enrolled,including 182 male patients with an average age of 15.4 ± 12.3 years.Transthoracic echocardiography (TTE) was applied to observe the tricuspid regurgitation and its degree before procedure,in 3 d after procedure,and during 1-,3-,6-and 12-month follow-up.The patients were divided into two groups based on the devices applied:a symmetric occluder group and a small-waist occluder group,and the incidence of tricuspid regurgitation in the two groups were

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

    Institute of Scientific and Technical Information of China (English)

    戴辰程; 郭保静; 金梅

    2011-01-01

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

  5. 低龄低体重患儿经胸小切口封堵膜周部室间隔缺损的策略及早期疗效%Strategy and Short-term Outcomes of Transthoraic Occlusion via a Small Incision in Low Birth-weight Infants with Ventricular Septal Defect

    Institute of Scientific and Technical Information of China (English)

    李小波; 万亚红; 邬云龙; 肖明第

    2012-01-01

    To study the surgical techniques and efficacy of transthoracic occlusion through a small chest incision for low birth-weight infants with ventricular septal defect (VSD). Methods Totally 51 low birth-weight infants with VSD, who received transthoracic occlusion in our hospital during October 2010 to November 2011, were enrolled into this study. The shunt diameter was 4-8 mm, as shown by echocardiography, and was over 1 mm away from the aortic valve. The patients aged from 4 to 12 months with a mean of (8.6 ±2. 1) months; the mean body weight was (7.4 ±2. 1) kg (ranged from 6 to 11 kg). Via a 4- to 5-mm incision below the sternum, under the guidance by TEE, we introduced a guide wire through the right ventricular anterior wall, and then placed occlusion device at the VSD site. The surgical procedure and efficacy was analyzed retrospectively afterwards. Results No mortality occurred during peri-operative period. The success rate of transthoracic occlusion was 90% (46/51). The five patients, who failed in the procedure, were transferred to cardiopulmonary bypass (CPB) for a VSD repair in a direct surgical field. In the 46 successful cases, no residual leakage, dislocation of occlusion device, or arrhythmia occurred; echocardiography showed no aortic regurgitation, however, 5 (13%) patients developed mild tricuspid valve regurgitation. Three patients (5. 8%) received blood transfusion of 50 ml. The patients were discharged from hospital in a mean of (3. 9 ± 0. 7) days (ranged from 3 to 7 days). Afterwards, all the 46 successful cases were followed up for (3. 6 ± 1. 4) months ( ranged from 1 to 12 months) , during which no patient died,and ECG, echocardiography, and chest X-ray found nothing abnormal, the cardiac faction was in grade I in all the cases. Conclusions For low birth-weight infants with VSD with a shunt diameter of 4 - 8 mm, and > 1 mm away from the aortic valve, transthoracic occlusion through a small chest incision is feasible and effective.%目的

  6. 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....

  7. Robotic trans-atrial and trans-mitral ventricular septal resection

    Science.gov (United States)

    2017-01-01

    Localized ventricular septal hypertrophy, also known as idiopathic hypertrophic subaortic stenosis or idiopathic hypertrophic subaortic septal obstruction (IHSS), can create severe ventricular outflow obstruction. This often results in a high sub-aortic pressure gradient with potentially lethal symptoms. In 1960, Braunwald described the hemodynamic characteristics of IHSS, and thereafter, Morrow developed a trans-aortic approach to resect a large part of the ventricular septum, enlarging the outflow tract. The Morrow operation has remained the gold standard for surgically treating this pathology. However, in patients with a small aortic annulus, a severely hypertrophied septum, and a long anterior mitral leaflet, the trans-aortic approach may be more difficult, resulting in an inadequate resection and/or systolic anterior leaflet motion. The latter usually increases the obstruction and can cause mitral regurgitation. Herein, we describe a minimally invasive trans-left atrial robotic approach to treat IHSS. First, the anterior mitral leaflet is incised radially to reveal the aortic outflow tract and ventricular septum, which are located posteriorly. Thereafter, a deep “block” of septum is excised, beginning at the right valve cusp nadir and continued counterclockwise toward the left fibrous trigone. This excision is extended to the anterior papillary muscle base, where any septal connections must be divided. Subsequently, the anterior leaflet is re-suspended and the repair is completed with a flexible annuloplasty band. If necessary, the anterior leaflet can be augmented with a pericardial patch. The “wristed” robotic instruments and magnified 3-D vision definitely facilitate an adequate septal resection and anterior papillary muscle mobilization. Moreover, it is possible that this fine control helps to reduce complications, such as heart block or a ventricular septal defect. PMID:28203542

  8. One-year cardiac morphological and functional evolution following permanent pacemaker implantation in right ventricular septal position in chagasic patients

    Directory of Open Access Journals (Sweden)

    Otaviano da Silva Júnior

    2012-06-01

    Full Text Available INTRODUCTION: The septal position is an alternative site for cardiac pacing (CP that is potentially less harmful to cardiac function. METHODS: Patients with Chagas disease without heart failure submitted to permanent pacemaker (PP implantation at the Clinics Hospital of the Triângulo Mineiro Federal University (UFTM, were selected from February 2009 to February 2010. The parameters analyzed were ventricular remodeling, the degree of electromechanical dyssynchrony (DEM, exercise time and VO2 max during exercise testing (ET and functional class (NYHA. Echocardiography was performed 24 to 48h following implantation and after one year follow-up. The patients were submitted to ET one month postprocedure and at the end of one year. RESULTS: Thirty patients were included. Patient mean age was 59±13 years-old. Indication for PP implantation was complete atrioventricular (AV block in 22 (73.3% patients and 2nd degree AV block in the other eight (26.7%. All patients were in NYHA I and no changes occurred in the ET parameters. No variations were detected in echocardiographic remodeling measurements. Intraventricular dyssynchrony was observed in 46.6% of cases and interventricular dyssynchrony in 33.3% of patients after one year. CONCLUSIONS: The findings of this work suggest that there is not significant morphological and functional cardiac change following pacemaker implantation in septal position in chagasic patients with normal left ventricular function after one year follow-up. Thus, patients may remain asymptomatic, presenting maintenance of functional capacity and no left ventricular remodeling.

  9. 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

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

    Directory of Open Access Journals (Sweden)

    Lalita Nemani

    2014-01-01

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

  11. Could early septal involvement in the remodeling process be related to the advance hypertensive heart disease?

    Directory of Open Access Journals (Sweden)

    Fatih Yalçin

    2015-06-01

    Conclusions: LV diastolic dysfunction becomes more severe in septal wall than lateral wall in hypertensive LVH. Septal myocardial performance is more dominantly affected by hypertension possibly due to earlier septal involvement in disease course. Septal MPI is correlated moderately with septal wall thickness.

  12. 经食管超声心动图引导下微创小切口室间隔缺损封堵治疗%Transcatheter closure of ventricular septal defects through minimally invasive port -access surgery guided by transesophageal echocardiography

    Institute of Scientific and Technical Information of China (English)

    侯永波; 王国祥; 谭胜; 刘金东

    2015-01-01

    目的:总结经食管超声心动图(TEE)引导下,微创经胸室间隔缺损(VSD)封堵治疗的临床经验。方法11例 VSD 患者接受微创经胸封堵治疗,年龄3~6岁,体重12~65 kg,VSD 直径3~8 mm。手术在 TEE 引导,胸骨下段小切口微创下进行,于右心室表面选择适当的穿刺点,建立输送轨道并完成 VSD 封堵。术后密切随访1~6个月。结果11例中9例一次性安放成功,2例首次安放有残余分流,术中更换大号伞;心内操作时间10~36 min,平均(21±12)min。全部患者均于术后4~5天出院,随访1~6个月,效果良好。11例患者均无心律失常、残余分流等并发症发生。结论TEE 引导下微创经胸 VSD 封堵是一种安全有效的治疗方法,但远期结果有待进一步观察。%Abstrat: Objectve To summarize the clinical experience of transcatheter closure of ventricular septal defects (VSD) through minimally invasive port -access surgery guided by transesophageal echocardiography (TEE).Methods A total of 11 patients aged from 3 to 6, weighting 12 kg to 65 kg were subjected to transcatheter closure of VSD with a diameter ranging from 3 mm to 8 mm.The surgery was performed under the guidance of TEE, where a small transthoracic incision was made.A proper site was chosen at the surface of the right ventricle for punctuation, in order to establish de-livery pathway.Patients were closely followed up for 1 to 6 months.Results Among the patients were nice ones who successfully completed the surgery at one time.Residual shunts were seen in two cases which required the replacement of larger occluders.The time of intracardiac procedures was 10 -36 min [(21 ±12)min].All patients were discharged 4 to 5 days after operation.During fellow -up period, neither arrhythmia nor residual shunt occurred.Conclusion Tran-sthoracic minimally invasive closure is a safe and efficient method for the treatment of VSD.Meanwhile, long -term fel

  13. 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....

  14. 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.

  15. [Complete atrioventricular block as the first clinical manifestation of a tick bite (Lyme disease)].

    Science.gov (United States)

    Bacino, Luca; Gazzarata, Massimo; Siri, Giambattista; Cordone, Stefano; Bellotti, Paolo

    2011-03-01

    A 52-year-old male patient presented to the emergency department because of malaise and frequent dizziness. The ECG revealed high-grade atrioventricular block that required placement of a temporary pacemaker. There were no other abnormalities in physical and echocardiographic examination, and coronary angiography excluded the presence of coronary artery disease. IgM and IgG antibodies against Borrelia were positive, and antibiotic therapy with ceftriaxone at the dose of 2 g/die for 15 days resulted in rapid regression of atrioventricular block. Seven-day ECG recording immediately after discharge and 24h ECG monitoring at 40 days confirmed the total disappearance of atrioventricular block. This represents a case of atrioventricular block as the first manifestation of Borrelia infection (Lyme disease). A prompt diagnosis and antibiotic therapy usually result in complete resolution of atrioventricular block without the need for a permanent pacemaker.

  16. Heart rhythm and conduction disturbances in early postoperative period after surgical correction for congenital heart defects in infants

    Directory of Open Access Journals (Sweden)

    Bockeria L. A.

    2012-03-01

    Full Text Available Objective. The study aimed to determine the range of rhythm and conduction disturbances in early postoperative period in infants and prove the prognostic value of some pre- and intraoperative factors for development of such complications. Material and methods. The study included 235 children aged from 1 to 3 years who underwent surgeries for congenital heart diseases (CHD under cardiopulmonary bypass (CPB. Exclusion criteria were: age less than 1 year or more than 3 years, presence of confirmed heart rhythm disturbances before surgery except incomplete or complete right bundle branch block in presence of right ventricular myocardial hypertrophy, left anterior bundle branch block in presence of partial or complete atrioventricular canal defect and sinus tachycardia equal to circulatory failure (CF. Results. Ventricular arrhythmias such as premature ventricular contraction (PVC of different character were frequently seen in early postoperative period after surgical correction of CHD. They were noted in 36% of overall number postoperative arrhythmias. Supraventricular extrasystole occurred in 29% cases, different types of supraventricular tachycardias – in 17%, AV-blocks of different grades – in 10%. We noted the following predictors for postoperative rhythm disturbance development: age more than 25 months at the moment of surgery, prolonged CPB and aortic cross-clamping during ventricular septal defect (VSD (70 and 39 minutes, respectively and Fallot’s tetralogy correction (100 and 60 minutes, respectively and exceeding the critical end-point of aortic cross-clamping during atrial septal defect correction (ASD (24 minutes, presence of IIA and IIb grade for CF before surgery, surgical correction of ASD under hypothermia of less than 32 °C. Exceeding the critical end-points of CPB and aortic clamping in patients with Fallot’s tetralogy and exceeding the critical end-points of aortic clamping in children with VSD and ASD were shown to be the

  17. Controversies in the Management of Isolated Congenital Atrioventricular Block.

    Science.gov (United States)

    DeNoble, Anna E; Kuller, Jeffrey A; Rhee, Eleanor J

    2015-08-01

    Congenital atrioventricular block (CAVB) affects approximately 2% of fetuses of mothers with anti-Ro or anti-La antibodies, regardless of maternal rheumatologic symptoms. Anti-Ro and anti-La antibodies are antinuclear antibodies commonly found in autoimmune diseases. Congenital atrioventricular block is associated with a relatively high fetal morbidity and mortality, particularly more advanced degrees of block. There is significant controversy surrounding surveillance of anti-Ro/La-positive pregnancies and treatment of fetuses diagnosed with CAVB. Studies of dexamethasone in the treatment of CAVB have yielded conflicting results, with most suggesting only a limited potential benefit in first- and seconddegree CAVB and in cases complicated by fetal hydrops. Larger prospective studies are needed to further evaluate the efficacy of intravenous immunoglobulin in the treatment of CAVB and of intravenous immunoglobulin and hydroxychloroquine in the prevention of CAVB in fetuses of at-risk mothers. Surveillance and treatment regimens should be determined on a case-by-case basis, taking into consideration the degree of CAVB, costs, and potential adverse effects of treatment.

  18. Effects of urokinase type plasminogen activator and plasminogen activator inhibitor-1 expressions on the formation of aneurysm of perimembranous ventricular septal defect%尿激酶型纤溶酶原激活物及其抑制物表达在膜周型室间隔缺损自发闭合中的作用

    Institute of Scientific and Technical Information of China (English)

    钱娟; 李本尚; 殷敏智; 沈萍; 孙锟

    2015-01-01

    0.05).结论 uPA及抑制物系统在VSA形成过程中起重要作用,参与瘤体的形成和纤维增殖过程.%Objective The exact mechanisms of defect closure in patients with perimembranous ventricular septal defect (PMVSD) remain unknown.We hypothesized that the expression of urokinase type plasminogen activator (uPA) and plasminogen activator inhibitor-1 (PAI-1) may mediate extracellular matrix (ECM) remodeling in aneurysms.Method Seven normal heart tricuspid septal leaflet and 33 aneurysms were collected in Shanghai Renji Hospital and Shanghai Children's Medical Center from January 2008 to June 2010.Immunohistochemical expression of uPA and PAI-1 in 4 normal heart valvular tissues and 15 aneurysms was detected with immunohistochemical methods.The expression of uPA and PAI-1 mRNA in 3 normal heart valvular tissues and 7 aneurysms was studied by real time fluorescent PCR;the protein expression of uPA and PAI-1 in 4 normal heart valvular tissues and 11 aneurysms was tested with Western blotting.Result The surface of the aneurysms were completely covered by endothelial cells.Two types of granulation tissue,myxoid and fibrous,were associated with the aneurismal formation.uPA were recognized predominantly in valvar interstitial cells (VICs) which located mainly in regions adjacent to the endothelium and smooth muscle cells of blood vessels.PAI-1 was found in both VICs which located mainly in granulation tissue and endothelial cells.Nine aneurysms expressed a higher uPA activity than 4 normal valvular tissues ((74.6 ± 11.8) % vs.(49.5 ± 7.4) %;t =3.87,P =0.003) and six aneurysms expressed a low uPA activity ((10.3±3.1)% vs.(49.5±7.4)%;t=11.78,P=0.000) andahighPAI-1 activity ((55.2±1.7) % vs.(50.8 ± 3.8) %;t =2.55,P =0.034) using immunohistochemical methods.uPA / PAI-1 ratio of protein expression tested by Western blot was 0.88 ± 0.22 in four normal heart vavular tissues;five aneurysms expressed high uPA activity and low PAI-1 activity and u

  19. An Analysis of the Causes of the Emerging Tricuspid Regurgitation after Minimally Invasive Transthoracic Closure of the Perimembranous Ventricular Septal Defects%膜周部室间隔缺损微创封堵术后新发三尖瓣反流程度变化的原因分析

    Institute of Scientific and Technical Information of China (English)

    万浩; 段书华; 周彩萍; 邢泉生

    2016-01-01

    Objective To analyze the cause of changes of emerging tricuspid regurgitation after minimally invasive transthoracic device closure of perimembranous ventricular septal defects (pmVSD).Methods 281 patients with pmVSD were selected to assess for tricuspid regurgitation with transesophageal echocardiography during preoperative and postoperative periods. Transthoracic echocardiography was used postoperatively during the 1-wk, 1-mo, 3-mo, 6-mo, 12-mo, 24-mo, 48-mo, 60-mo follow-up to evaluate the effectiveness and to assess for any complication emerging after the surgery.ResultsThere were 19 cases with tricuspid regurgitation with varying degrees of aggravation emerging immediately after closure. During the follow-ups, in the mild-relfux group, 12 patients’ relfux symptom mitigated;one case aggravated to mild to moderate relfux. In the mild to moderate relfux group, 2 cases alleviated to mild relfux; the other case aggravated to moderate relfux.Conclusion The aggravation of tricuspid regurgitation after minimally invasive transthoracic device closure of pmVSD may be caused by the occluder oppression on the tricuspid chordae or lealfets, tendons damage resulted fromdelivery sheath, and abnormal origin of part of the anterior tricuspid valve chordate. Mitigation of tricuspid regurgitation after surgery may be caused by remodeling of the occluder, which leads to oppressed chordae or lealfets to restore to the physiological state. Preoperative and postoperative ultrasound allows precise assessment of the position of tricuspid valve chordae and helps with understanding of the construction of the structure of defect edge tissues, which provides guidance for selecting the appropriate occluder and thus reduce or mitigate tricuspid regurgitation and other complications.%目的:分析经胸微创封堵膜周部室间隔缺损(pmVSD)术后三尖瓣反流程度的变化原因。方法对经胸微创封堵成功的281例pmVSD患儿进行术前、术后即用食道超声心

  20. The one-stage rhinoplasty septal perforation repair.

    Science.gov (United States)

    Foda, H M

    1999-08-01

    A combined septal perforation repair and rhinoplasty was performed in 20 patients (12 males, eight females; age range 16-36, mean age 29.6) presenting with septal perforations (size 1-4 cm) and external nasal deformities. The external rhinoplasty approach was used for all cases and the perforation was repaired using bilateral intranasal mucosal advancement flaps with a connective tissue interposition graft in between. The perforation was totally closed in 18 cases (90 per cent) with complete resolution of the pre-operative symptoms occurring in 16 (80 per cent). Cosmetically, 19 cases (95 per cent) were very satisfied with their aesthetic result. The exposure provided by the external approach proved to be very helpful in the process of septal perforation repair. Our results show that septal perforation repair could safely be combined with rhinoplasty and that some of the rhinoplasty manoeuvres used could even facilitate the process of septal perforation repair.

  1. Rapidly Progressive Atrioventricular Block in a Patient with Sarcoidosis

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    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.

  2. Reentry confined to the atrioventricular node: electrophysiologic and anatomic findings.

    Science.gov (United States)

    Sheinman, M M; Gonzalez, R; Thomas, A; Ullyot, D; Bharati, S; Lev, M

    1982-05-01

    A patient with recurrent disabling, paroxysmal supraventricular tachycardia refractory to drug treatment underwent electrophysiologic studies. The paroxysmal supraventricular tachycardia was found to be due to atrioventricular (A-V) nodal reentry. The patient died shortly after surgical His bundle section and detailed anatomic studies were performed. These showed fatty infiltration of the approaches to the sinoatrial node, atrial preferential pathways, and A-V node and common bundle. The A-V node was mechanically damaged and the common His bundle was completely severed. These abnormalities were clearly delineated and there was no evidence of an atrio-His bundle bypass tract to an accessory A-V node. Specifically, the central fibrous body and pars membranacea were defined and no atrial muscular fibers pierced these structures to joint the A-V bundle. It is concluded that paroxysmal supraventricular tachycardia due to A-V nodal reentry can be confined to the A-V node.

  3. 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.

  4. 血流向量成像技术在房间隔缺损右房流场可视化及肺体循环血流量比定量中的价值%Visualizaion of Right Atrial Flow Patterns and Quantification of Pulmonary to Systemic Flow Ratio in Patients with Atrial Septal Defect by Vector Flow Mapping

    Institute of Scientific and Technical Information of China (English)

    韦馨; 梁玉佳; 朱蔚; 唐红; 陈柳; 张炬倩; 李晨; 冯沅; 周文霞; 彭瑛; 黄鹤

    2012-01-01

    Objective To evaluate the ability of vector flow mapping (VFM) in visualizing right atrial flow and quantifying pulmonary to systemic flow ratio in patients with atrial septal defect (ASD). Methods VFM was performed on 30 patients with ASD and 50 healthy volunteers. The pulmonary to systemic flow ratio (Qp/Qs) was calculated using VFM and spectral doppler compared with that obtained through oximetric data derived from cardiac catheterization. Resnlts Blood streams from superior and inferior caval veins did not collide but turned forward, contributing to a forward clockwise vortex during systole on the subcostal bi-atrial plane in the healthy volunteers. The vortex was disrupted continuously by a left to right shunt in the patients with ASD during the hole cardiac cycle. The Qp/Qs measured by VFM was less angle-dependent in all the cases than the other methods. The mean value of Qp/Qs of the healthy volunteers calculated by apical three-chamber view and apical five-chamber view ranged from 0. 84 :1 to 1. 15 :1, which is consistent with the theoretical value 1 of no left to right shunts. The values of Qp/Qs of patients with ASD measured by VFM and spectral doppler showed no significant differences with those obtained through oximetric data C(2. 18±0. 48) vs. (2. 29±0. 76), P = 0. 29; (2.30±0. 91) vs. (2. 29± 0. 76), P=0. 863. However, the Qp/Qs value measured by VFM had a better correlation with the oximetric data than that measured by spectral doppler (r=0. 71, P<0. 001 vs. R=0. 38, P<0. 05). The inter-observer and intra-observer variability of Qp/Qs measured by VFM was 9. 84% and 9. 86% respectively. Conclusion VFM can visualize right atrial flow field in patients with ASD and quantify Qp/Qs in a more accurate way than spectral dopier.%目的 探讨血流向量成像(VFM)技术观察房间隔缺损(ASD)患者右房流场分布的可行性及定量肺、体循环血流量比(Qp/Qs)的准确性.方法 应用VFM观察50例健康体检者(正常组)和30例ASD患

  5. Evaluation on global left ventricular systolic function of children with ventricular septal defect by two-dimensional speckle tracking imaging%二维斑点追踪成像评价室间隔缺损患儿左室整体收缩功能

    Institute of Scientific and Technical Information of China (English)

    李莎; 张立敏; 马春燕; 刘爽; 张妍; 杨军

    2014-01-01

    目的:探讨应用二维斑点追踪成像(2D-STI)评价室间隔缺损(VSD)患儿左室整体收缩功能的价值。方法单纯VSD患儿42例,按缺损大小与主动脉瓣环比值分为小VSD组、中VSD组、大VSD组;30例体检健康儿童为对照组。超声心动图常规测量左房收缩末期内径(LAD)、左室舒张末期内径(LVEDD)、左室舒张末期容积(LVEDV)、左室收缩末期容积(LVESV)及左室射血分数(LVEF),计算左房容积。2D-STI测量左室整体纵向峰值应变(GLPS)、收缩期峰值应变率(GLSRS),左室整体径向峰值应变(GRPS)、收缩期峰值应变率(GRSRS)及左室整体圆周峰值应变(GCPS)、收缩期峰值应变率(GCSRS)。结果大VSD组和中VSD组的LAD、LAV、LVEDD、LVEDV、LVESV较小VSD组和对照组均增大(P<0.05),大VSD组LAD、LAV、LVEDD、LVEDV、LVESV较中VSD组均增大(P<0.05)。与对照组和小VSD组的GLPS、GRPS、GCPS、GLSRS、GRSRS、GCSRS比较,中VSD组均增大(P<0.05),大VSD组均减小(P<0.05);中VSD组GLPS、GRPS、GCPS、GLSRS、GRSRS、GCSRS亦大于大VSD组(P<0.05)。结论中度以上VSD影响左室整体收缩功能。2D-STI的应变、应变率对VSD患儿左室整体收缩功能的评价有重要意义。%Objective To explore the value of two-dimensional speckle tracking imaging (2D-STI) in evaluating the global left ventricular systolic function of children with ventricular septal defect. Methods Forty-two children with simple VSD were divided into small, moderate and large VSD group according to the ratio of VSD size to aortic annulus diameter. And 30 healthy children were enrolled as control group. The left atrial systolic diameter (LAD), left ventricular end-diastolic diameter (LVEDD), left ventricular end-diastolic volume (LVEDV), left ventricular end systolic volume (LVESV) and left ventricular ejection fraction (LVEF) were routinely

  6. 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

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

    Directory of Open Access Journals (Sweden)

    Miguel Barbero-Marcial

    1987-04-01

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

  8. Cardiac Defects and Results of Cardiac Surgery in 22q11.2 Deletion Syndrome

    Science.gov (United States)

    Carotti, Adriano; Digilio, Maria Cristina; Piacentini, Gerardo; Saffirio, Claudia; Di Donato, Roberto M.; Marino, Bruno

    2008-01-01

    Specific types and subtypes of cardiac defects have been described in children with 22q11.2 deletion syndrome as well as in other genetic syndromes. The conotruncal heart defects occurring in patients with 22q11.2 deletion syndrome include tetralogy of Fallot, pulmonary atresia with ventricular septal defect, truncus arteriosus, interrupted aortic…

  9. 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 b...

  10. Septal Myectomy Surgery to Treat Obstructive Hypertrophic Cardiomyopathy (HCM)

    Science.gov (United States)

    Septal Myectomy Surgery to Treat Obstructive Hypertrophic Cardiomyopathy (HCM) Click Here to view the BroadcastMed, Inc. Privacy Policy and Legal Notice © 2017 BroadcastMed, Inc. All rights reserved.

  11. LEFT VENTRICULAR INFLOW OBSTRUCTION BY GIANT ATRIAL SEPTAL ANEURYSM IN A NEONATE WITH HYPOPLASTIC RIGHT HEART SYNDROME: CASE REPORT.

    Science.gov (United States)

    Yuko-Jowi, C; Okello, C A

    2013-02-01

    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 stroke. Baby H.N delivered on 10/05/2008 by C/S, was cyanosed at birth with systemic desaturation. Chest X-ray showed oligaemic lung fields while two dimensional echocardiograms showed tricuspid atresia with hypoplastic right ventricle, large secundum atrial septal defect, and highly mobile gigantic aneurysms of the atrial septum obstructing the inflow of the mitral valve and entering the left ventricle in diastole. Surgical intervention was not possible and child died on second day.

  12. Nitric oxide synthase-3 promotes embryonic development of atrioventricular valves.

    Directory of Open Access Journals (Sweden)

    Yin Liu

    Full Text Available Nitric oxide synthase-3 (NOS3 has recently been shown to promote endothelial-to-mesenchymal transition (EndMT in the developing atrioventricular (AV canal. The present study was aimed to investigate the role of NOS3 in embryonic development of AV valves. We hypothesized that NOS3 promotes embryonic development of AV valves via EndMT. To test this hypothesis, morphological and functional analysis of AV valves were performed in wild-type (WT and NOS3(-/- mice at postnatal day 0. Our data show that the overall size and length of mitral and tricuspid valves were decreased in NOS3(-/- compared with WT mice. Echocardiographic assessment showed significant regurgitation of mitral and tricuspid valves during systole in NOS3(-/- mice. These phenotypes were all rescued by cardiac specific NOS3 overexpression. To assess EndMT, immunostaining of Snail1 was performed in the embryonic heart. Both total mesenchymal and Snail1(+ cells in the AV cushion were decreased in NOS3(-/- compared with WT mice at E10.5 and E12.5, which was completely restored by cardiac specific NOS3 overexpression. In cultured embryonic hearts, NOS3 promoted transforming growth factor (TGFβ, bone morphogenetic protein (BMP2 and Snail1expression through cGMP. Furthermore, mesenchymal cell formation and migration from cultured AV cushion explants were decreased in the NOS3(-/- compared with WT mice. We conclude that NOS3 promotes AV valve formation during embryonic heart development and deficiency in NOS3 results in AV valve insufficiency.

  13. Coexistent Types of Atrioventricular Nodal Re-Entrant Tachycardia

    Science.gov (United States)

    Marine, Joseph E.; Latchamsetty, Rakesh; Zografos, Theodoros; Tanawuttiwat, Tanyanan; Sheldon, Seth H.; Buxton, Alfred E.; Calkins, Hugh; Morady, Fred; Josephson, Mark E.

    2015-01-01

    Background— There is evidence that atypical fast–slow and typical atrioventricular nodal re-entrant tachycardia (AVNRT) do not use the same limb for fast conduction, but no data exist on patients who have presented with both typical and atypical forms of this tachycardia. We compared conduction intervals during typical and atypical AVNRT that occurred in the same patient. Methods and Results— In 20 of 1299 patients with AVNRT, both typical and atypical AVNRT were induced at electrophysiology study by pacing maneuvers and autonomic stimulation or occurred spontaneously. The mean age of the patients was 47.6±10.9 years (range, 32–75 years), and 11 patients (55%) were women. Tachycardia cycle lengths were 368.0±43.1 and 365.8±41.1 ms, and earliest retrograde activation was recorded at the coronary sinus ostium in 60% and 65% of patients with typical and atypical AVNRT, respectively. Thirteen patients (65%) displayed atypical AVNRT with fast–slow characteristics. By comparing conduction intervals during slow–fast and fast–slow AVNRT in the same patient, fast pathway conduction times during the 2 types of AVNRT were calculated. The mean difference between retrograde fast pathway conduction during slow–fast AVNRT and anterograde fast pathway conduction during fast–slow AVNRT was 41.8±39.7 ms and was significantly different when compared with the estimated between-measurement error (P=0.0055). Conclusions— Our data provide further evidence that typical slow–fast and atypical fast–slow AVNRT use different anatomic pathways for fast conduction. PMID:26155802

  14. Nitric oxide synthase-3 promotes embryonic development of atrioventricular valves.

    Science.gov (United States)

    Liu, Yin; Lu, Xiangru; Xiang, Fu-Li; Lu, Man; Feng, Qingping

    2013-01-01

    Nitric oxide synthase-3 (NOS3) has recently been shown to promote endothelial-to-mesenchymal transition (EndMT) in the developing atrioventricular (AV) canal. The present study was aimed to investigate the role of NOS3 in embryonic development of AV valves. We hypothesized that NOS3 promotes embryonic development of AV valves via EndMT. To test this hypothesis, morphological and functional analysis of AV valves were performed in wild-type (WT) and NOS3(-/-) mice at postnatal day 0. Our data show that the overall size and length of mitral and tricuspid valves were decreased in NOS3(-/-) compared with WT mice. Echocardiographic assessment showed significant regurgitation of mitral and tricuspid valves during systole in NOS3(-/-) mice. These phenotypes were all rescued by cardiac specific NOS3 overexpression. To assess EndMT, immunostaining of Snail1 was performed in the embryonic heart. Both total mesenchymal and Snail1(+) cells in the AV cushion were decreased in NOS3(-/-) compared with WT mice at E10.5 and E12.5, which was completely restored by cardiac specific NOS3 overexpression. In cultured embryonic hearts, NOS3 promoted transforming growth factor (TGFβ), bone morphogenetic protein (BMP2) and Snail1expression through cGMP. Furthermore, mesenchymal cell formation and migration from cultured AV cushion explants were decreased in the NOS3(-/-) compared with WT mice. We conclude that NOS3 promotes AV valve formation during embryonic heart development and deficiency in NOS3 results in AV valve insufficiency.

  15. Acidosis slows electrical conduction through the atrio-ventricular node

    Directory of Open Access Journals (Sweden)

    Ashley Muir Nisbet

    2014-06-01

    Full Text Available Acidosis affects the mechanical and electrical activity of mammalian hearts but comparatively little is known about its effects on the function of the atrio-ventricular node (AVN. In this study, the electrical activity of the epicardial surface of the left ventricle of isolated Langendorff-perfused rabbit hearts was examined using optical methods. Perfusion with hypercapnic Tyrode’s solution (20% CO2, pH 6.7 increased the time of earliest activation (Tact from 100.5+7.9 to 166.1+7.2ms (n=8 at a pacing cycle length (PCL of 300ms (37oC. Tact increased at shorter PCL, and the hypercapnic solution prolonged Tact further: at 150ms PCL, Tact was prolonged from 131.0+5.2 to 174.9+16.3ms. 2:1 AVN block was common at shorter cycle lengths. Atrial and ventricular conduction times were not significantly affected by the hypercapnic solution suggesting that the increased delay originated in the AVN. Isolated right atrial preparations were superfused with Tyrode’s solutions at pH 7.4 (control, 6.8 and 6.3. Low pH prolonged the atrial-Hisian (AH interval, the effective and functional refractory periods and Wenckebach cycle length significantly. Complete AVN block occurred in 6 out of 9 preparations. Optical imaging of conduction at the AV junction revealed increased conduction delay in the region of the AVN, with less marked effects in atrial and ventricular tissue. Thus acidosis can dramatically prolong the AVN delay, and in combination with short cycle lengths, this can cause partial or complete AVN block and is therefore implicated in the development of brady-arrhythmias in conditions of local or systemic acidosis.

  16. 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

  17. Impact of Down syndrome on the surgical treatment of congenital heart defects%Down综合征对先天性心脏病外科治疗的影响

    Institute of Scientific and Technical Information of China (English)

    张伟志; 杨一峰; 黄灿; 赵天力

    2012-01-01

    Objective: To evaluate the impact of Down syndrome (DS) on surgical management in patients with congenital heart defects (CHD).Methods: We retrospectively analyzed the clinical data from 35 children with DS and CHD, who underwent cardiac surgery between 2004 and 2009. The data on surgical mortality, complications and follow-up results are emphasized.Results: All of the patients underwent primary repair. One child (2.9%) with DS and complete atrioventricular septal defect (CAVSD) died early postoperatively because of pulmonary hypertension. Two patients (5.7%) had low cardiac output syndrome, and 15 (42.9%) suffered pulmonary complications. III° atrioventricular block (AVB) occurred in 4 patients (11.5%). Thirty children who were followed up 10 months to 6 years [(3.8 ±1.1) years] are in NYHA class I or II. There were no reoperations or later death.Conclusion: CHD in DS children can be repaired with a low risk of mortality, although a high incidence of severe infections and III ° AVB can result in a complicated postoperative course. The results of mid-term follow up are satisfactory.%目的:评估Down综合征对先天性心脏病手术治疗的影响.方法:回顾性分析于2004年至2009年行心脏手术治疗的35例合并先天性心脏病的Down综合征患儿的临床资料,总结此类患儿的手术死亡率、并发症和随访结果.结果:所有患儿均接受一期手术修复.术后早期死亡1例(2.9%);低心排出量综合征2例(5.7%);肺部并发症15例(42.9%).术后早期Ⅲ°房室传导阻滞4例(11.4%).对30例随访10个月~6年[(3.8±1.1)年],无远期死亡和再次手术病例,存活者心功能为NYHA Ⅰ或Ⅱ级.结论:Down综合征增加了先天性心脏病手术的早期风险性,但不增加死亡率,术后中期生存状态良好.

  18. Nasal Septal Deviations: A Systematic Review of Classification Systems

    Science.gov (United States)

    Teixeira, Jeffrey; Certal, Victor; Chang, Edward T.; Camacho, Macario

    2016-01-01

    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. PMID:26933510

  19. 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.

  20. 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.

  1. Department of Defense Birth and Infant Health Registry: Annual Report on Birth Defects Among Infants Born to U.S. Military Families, January 1, 2000 Through December 31, 2000

    Science.gov (United States)

    2005-03-01

    745.4 Ventricular septal defect 745.5 Atrial septal defect 745.6 Endocardial cushion defects 746.01-746.02 Pulmonary valve...left heart syndrome 747.0 Patent ductus arteriosus 747.1 Coarctation of aorta 747.3 Anomalies of pulmonary artery Resp Respiratory...Biliary atresia Male Male Reproductive 752.6 Hypospadias, epispadias Urinary Urinary 753.0 Renal agenesis /dysgenesis 753.2/753.6 Obstructive

  2. Reversible cardiogenic shock caused by atrioventricular junctional rhythm after percutaneous coronary intervention

    Institute of Scientific and Technical Information of China (English)

    Jun Wang; Qi-Gao Zhang; Xiao-Min Cai; Li-Jun Wang; Jian-Bin Gong; Shi-Sen Jiang

    2012-01-01

    An 82-year-old female patient undergoing cardiogenic shock caused by atrioventricular junctional rhythm immediately after percutaneous coronary intervention (PCI) is described. Pharmacotherapy was invalid, and subsequent application of atrial pacing reversed the cardiogenic shock. PCI-related injury of sinuatrial nodal artery leading to acute atrial contractility loss, accompanied by atrioventricular junctional arrhythmia, was diagnosed. We recommend that preoperative risk evaluation be required for multi-risk patients. Likewise, emergent measures should to be established in advance. This case reminds us that atrial pacing can be an optimal management technique once cardiogenic shock has occurred.

  3. Tubular hypoplasia of the aorta and right atrioventricular valve dysplasia in a Bulldog.

    Science.gov (United States)

    Robinson, Nicholas A; Armíen, Aníbal G

    2010-07-01

    A Bulldog puppy that died at 1 day of age was presented for postmortem evaluation. Macroscopically, there was marked hypoplasia of the ascending, transverse, and proximal segments of the descending thoracic aorta and almost complete secondary thrombosis of the left ventricle causing a functional stenosis of the left atrioventricular valve. Separately, there was right atrioventricular valve dysplasia with secondary dilation of the right atrium. Microscopically, the left ventricular outflow tract was occluded by chondroid metaplasia, fibrosing recanalization of a left-ventricular thrombus, and isolated Purkinje fiber degeneration and necrosis.

  4. [Exercise-induced atrioventricular block. Significance of the ischemic component. Report of 4 new cases].

    Science.gov (United States)

    Rumoroso, J R; Montes Orbe, P M; Cembellín, J C; Pérez-García, P; González-Liébana, J; Gómez-Varela, S; Bodegas, A; Barrenetxea, J I

    1997-04-01

    We report four new cases of exercise-induced atrio-ventricular block (appearing during treadmill exercise testing). The mechanism was ischemia in two patients and the conduction disturbance disappeared after coronary artery bypass grafting. The literature on this matter is reviewed. Also the etiology, the natural history and management are discussed in these cases.

  5. Potential additional indicators for pacemaker requirement in isolated congenital atrioventricular block.

    NARCIS (Netherlands)

    Breur, J.M.; Udink ten Cate, F.E.; Kapusta, L.; Boramanand, N.; Cohen, M.I.; Crosson, J.E.; Lubbers, L.J.; Friedman, A.H.; Brenner, J.I.; Vetter, V.L.; Meijboom, E.J.

    2006-01-01

    Low heart rate is the predominantly used indication for pacemaker intervention in patients with isolated congenital atrioventricular block (CAVB). The aim of this study was to compare the difference in heart rates recorded with ECG and Holter monitoring between paced (PM) and nonpaced (NPM) patients

  6. Potential additional indicators for pacemaker requirement in isolated congenital atrioventricular block

    NARCIS (Netherlands)

    J.M.P.J. Breur; F.E.A.U. ten Cate; L. Kapusta; N. Boramanand; M.I. Cohen; J.E. Crosson; L.J. Lubbers; A.H. Friedman; J.I. Brenner; V.L. Vetter; E.J. Meijboom

    2006-01-01

    Low heart rate is the predominantly used indication for pacemaker intervention in patients with isolated congenital atrioventricular block (CAVB). The aim of this study was to compare the difference in heart rates recorded with ECG and Holter monitoring between paced (PM) and nonpaced (NPM) patients

  7. 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 o

  8. Deterministic model of the canine atrio-ventricular node as a periodically perturbed, biological oscillator

    NARCIS (Netherlands)

    Tweel, I. van der; Herbschleb, J.N.; Borst, C.; Meijler, F.L.

    1986-01-01

    The atrio-ventricular (AV) node may be regarded as a periodically perturbed, biologicaL oscillator. In that case the ventricular response to atrial excitation can be described by a latency-phase curve. The phase is approximated by the time between a QRS-complex and an atrial stimulus S (R-S interval

  9. Exercise capacity in children with isolated congenital complete atrioventricular block: does pacing make a difference?

    NARCIS (Netherlands)

    Blank, A.C.; Hakim, S.; Strengers, J.L.; Tanke, R.B.; Veen, T.A. van; Vos, M.E. de; Takken, T.

    2012-01-01

    The management of patients with isolated congenital complete atrioventricular block (CCAVB) has changed during the last decades. The current policy is to pace the majority of patients based on a variety of criteria, among which is limited exercise capacity. Data regarding exercise capacity in this p

  10. Exercise Capacity in Children With Isolated Congenital Complete Atrioventricular Block : Does Pacing Make a Difference?

    NARCIS (Netherlands)

    Blank, A. Christian; Hakim, Sara; Strengers, Jan L.; Tanke, Ronald B.; van Veen, Toon A.; Vos, Marc A.; Takken, Tim

    2012-01-01

    The management of patients with isolated congenital complete atrioventricular block (CCAVB) has changed during the last decades. The current policy is to pace the majority of patients based on a variety of criteria, among which is limited exercise capacity. Data regarding exercise capacity in this p

  11. Congenital intermittent atrio-ventricular dissociation in tetralogy of Fallot (a case report.

    Directory of Open Access Journals (Sweden)

    Karande S

    1991-01-01

    Full Text Available A case of tetralogy of fallot with congenital intermittent atrioventricular dissociation is reported. A review of standard postgraduate books of cardiology fails to describe this condition, thus showing its extreme rarity. The therapeutic dilemma in treating such a case is also discussed.

  12. Congenital intermittent atrio-ventricular dissociation in tetralogy of Fallot (a case report).

    OpenAIRE

    Karande S; Sheth S; Dalvi B; Lahiri K; Shah M

    1991-01-01

    A case of tetralogy of fallot with congenital intermittent atrioventricular dissociation is reported. A review of standard postgraduate books of cardiology fails to describe this condition, thus showing its extreme rarity. The therapeutic dilemma in treating such a case is also discussed.

  13. Cardiovascular magnetics resonance diagnosis of cystic tumor of the atrioventricular node

    Directory of Open Access Journals (Sweden)

    Wang Xuedong

    2009-04-01

    Full Text Available Abstract Late gadolinium enhanced (LGE cardiovascular magnetic resonance (CMR has proven to be the gold standard for viability assessment. LGE CMR is also useful for identifying the nature of cardiac masses or lesions. We report a case of a rare primary cystic tumor of the atrioventricular node, in which CMR proved to be valuable.

  14. Electrophysiologic and antiarrhythmic effects of intravenous bisoprolol in atrioventricular nodal reentry tachycardia

    NARCIS (Netherlands)

    vandeVen, LLM; Crijns, HJGM; deMuinck, ED; VanGelder, IC; VanWijk, LM; Lie, KI

    1996-01-01

    Beta-blockade may be useful in the termination and prevention of atrioventricular nodal reentry tachycardia (AVNRT), An electrophysiologic study was performed in 9 patients (4 men and 5 women; mean +/- SD age, 56 +/- 16 years) with documented AVNRT before and after the intravenous administration of

  15. Mobitz type I atrio-ventricular block in dengue myocarditis, requiring temporary pacing

    Directory of Open Access Journals (Sweden)

    S. de Mel

    2015-01-01

    Full Text Available We present a case of dengue myocarditis related Mobitz type I atrio-ventricular (A-V block. To our knowledge, this is the first report of such a patient requiring pacing. An early response to methylprednisolone suggests the possibility of a therapeutic role for steroids in these patients.

  16. Evolution and scaling of atrioventricular conduction time in mammals. [Pt. 1

    NARCIS (Netherlands)

    Meijler, F.L.; Strackee, J.

    2006-01-01

    Scaling can be defined as the adjustment of a structure, a function, or an organ to the size of the mammalian body. An example is the size of the heart in relation to the size of the body. The duration of the PR interval on the electrocardiogram (atrioventricular delay) in relation to the size of th

  17. Comparative aspects of the dual role of the human atrioventricular node

    NARCIS (Netherlands)

    Meijler, F.L.

    1986-01-01

    As well as transmitting the impulse from the atria to the ventric1es the atrioventricular node has two other important functions namely: (a) synchronisation of atrial and ventricular contractions by a varying delay; and (b) protection of the ventricles from rapid atrial arrhythmias. The relative imp

  18. [Successful selective electrical ablation of the retrograde pathway in atrioventricular nodal reentry tachycardia associated with syncope].

    Science.gov (United States)

    Lukl, J; Cíhalík, C

    1992-01-01

    A 55-year-old man was admitted to the intensive care unit on account of repeatedly occurring syncopes which developed at the peak of physical exertion. The attack was reproduced by exercise on a bicycle ergometer: the patient developed paroxysmal tachycardia with a narrow QRS and a frequency of 160/min leading after 20 sec. to severe hypotension and loss of consciousness. The same tachycardia caused by programmed atrial stimulation caused a drop of tension in the recumbent position by 30 mmHg and after more detailed analysis during electrophysiological examination it was evaluated as atrioventricular nodal reentrant tachycardia. By an electric discharge of 300 J administered by means of a stimulation electrode 7F USCI into the area of the AV node the retrograde conduction through the perinodal rapid pathways was completely interrupted and 1st. degree atrioventricular block developed. Repeated electrophysiological examination and exercise tests on a bicycle ergometer provided evidence of the disappearance of the retrograde pathway and the impossibility to elicit AVNRT. The authors express the view that the rapid perinodal pathway is interrupted in successful cases in both directions and the 1st. degree AV block is due to conduction along a slow pathway and not incidental slowing of conduction along the rapid pathway which is the generally accepted interpretation. Modification of the atrioventricular conduction by interruption of the rapid pathway by fulguration is according to data in the literature and the described patient a method which makes is possible to cure severe atrioventricular nodal reentrant tachycardias.

  19. Time-resolved tracking of the atrioventricular plane displacement in Cardiovascular Magnetic Resonance (CMR) images

    DEFF Research Database (Denmark)

    Seemann, Felicia; Pahlm, Ulrika; Steding-Ehrenborg, Katarina

    2017-01-01

    BACKGROUND: Atrioventricular plane displacement (AVPD) is an indicator for systolic and diastolic function and accounts for 60% of the left ventricular, and 80% of the right ventricular stroke volume. AVPD is commonly measured clinically in echocardiography as mitral and tricuspid annular plane...

  20. Fetal atrioventricular and outflow tract flow velocity waveforms during conducted and blocked supraventricular extrasystoles

    NARCIS (Netherlands)

    K. van der Mooren (K.); J.W. Wladimiroff (Juriy); Th. Stijnen (Theo)

    1992-01-01

    textabstractMaximum flow velocity waveforms at atrioventricular and outflow tract level were studied cross‐sectionally in 19 human fetuses with conducted and/or blocked supraventricular extrasystoles ranging from 25 to 38 weeks of gestation. At outflow tract level, peak systolic velocity and acceler

  1. 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

  2. The road to right ventricular septal pacing: techniques and tools.

    Science.gov (United States)

    Mond, Harry G

    2010-07-01

    Prolonged right ventricular (RV) apical pacing is associated with progressive left ventricular dysfunction due to dysynchronous ventricular activation and contraction. RV septal pacing allows a narrower QRS compared to RV apical pacing, which might reflect a more physiological and synchronous ventricular activation. Previous clinical studies, which did not consistently achieve RV septal pacing, were not confirmatory and need to be repeated. This review summarizes the anatomy of the RV septum, the radiographic appearances of pacing leads in the RV, the electrocardiograph correlates of RV septal lead positioning, and the techniques and tools required for implantation of an active-fixation lead onto the RV septum. Using the described techniques and tools, conventional active-fixation leads can now be reliably secured to either the RV outflow tract septum or mid-RV septum with very low complication rates and good long-term performance. Even though physiologic and hemodynamic studies on true RV septal pacing have not been completed, the detrimental effects of long-term RV apical pacing are significant enough to suggest that it is now time to leave the RV apex and secure all RV leads onto the septum.

  3. Echocardiography for the Assessment of Congenital Heart Defects in Calves.

    Science.gov (United States)

    Mitchell, Katharyn Jean; Schwarzwald, Colin Claudio

    2016-03-01

    Congenital heart disease should be considered when evaluating calves with chronic respiratory signs, failure to thrive, poor growth, or if a murmur is heard on physical examination. Echocardiography is currently the gold standard for diagnosing congenital heart defects. A wide variety of defects, either alone or in combination with a ventricular septal defect, are possible. A standardized approach using sequential segmental analysis is required to fully appreciate the nature and severity of more complex malformations. The prognosis for survival varies from guarded to poor and depends on the hemodynamic relevance of the defects and the degree of cardiac compensation.

  4. Clinical analysis of 73 pediatric patients with completely atrioventricular conduction block%儿童完全性房室传导阻滞73例

    Institute of Scientific and Technical Information of China (English)

    曹黎明; 周凯; 秦玉明; 杨世伟; 赵乃铮; 王凤鸣; 钱建华; 龚晓平

    2016-01-01

    CAVB patients with myocarditis undergoing ventricular septal defect (VSD)closure operation.All congenital CAVB patients were re-fractory to drug therapy.Electrocardiogram and echocardiogram were performed in 1 9 cases without clinical symptoms during follow -up,but 2 cases had permanent pacemaker implanted.Among 27 fulminant myocarditis,Adams -Stokes attacks were found in 1 5 cases,3 cases had Adams -Stokes attack in 1 5 cases with sequelae of myocarditis,and 2 out of 6 cases undergoing VSD closure operation had Adams -Stokes attack,and other 4 cases without clinical symptoms were followed up periodically.The acquired CAVB patients were given energy composition and intravenous megavitamin C. The cases with fulminant myocarditis were given adrenal cortical hormone and intravenous gamma globulins simulta-neously.A total of 27 acquired CAVB patients were implanted temporary pacemaker and 5 with permanent pacemaker. Among 52 acquired CAVB patients,31 cases were cured,9 cases were improved,1 1 cases were ineffective,and 1 case died.Conclusions Most congenital CAVB children without clinical symptoms need clinical follow -ups.Myocarditis is a major cause of acquired CAVB.The CAVB prognosis caused by fulminant myocarditis may be related to antimely im-planting the temporary pacemaker timely.Permanent pacemaker should be implanted in patients who have no response to drug therapy with frequent Adams -Stokes or heart failure.

  5. 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...

  6. A Case of Adult-Onset Acute Rheumatic Fever With Long-Lasting Atrioventricular Block Requiring Permanent Pacemaker Implantation.

    Science.gov (United States)

    Oba, Yusuke; Watanabe, Hiroaki; Nishimura, Yoshioki; Ueno, Shuichi; Nagashima, Takao; Imai, Yasushi; Shimpo, Masahisa; Kario, Kazuomi

    2015-01-01

    A 45-year-old hypertensive Japanese woman presented with epigastric pain on inspiration, fever, complete atrioventricular block and polyarthritis. Her antistreptolysin O levels were markedly elevated. A diagnosis of rheumatic fever was made according to the modified Jones criteria. She was prescribed loxoprofen sodium, which was partially effective for her extracardiac clinical symptoms. However, she had syncope due to complete atrioventricular block with asystole longer than 10 seconds. Consequently, we implanted a permanent pacemaker. Although we prescribed prednisolone, the efficacy of which was limited for the patient's conduction disturbance, the complete atrioventricular block persisted. In our systematic review of 12 similar cases, the duration of complete heart block was always transient and there was no case requiring a permanent pacemaker. We thus encountered a very rare case of adult-onset acute rheumatic fever with persistent complete atrioventricular block necessitating permanent pacemaker implantation.

  7. 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 sodiu

  8. 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

  9. 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.

  10. 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.

  11. Congenital heart defect in a patient with deletion of chromosome 7q.

    Science.gov (United States)

    Tiller, G E; Watson, M S; Duncan, L M; Dowton, S B

    1988-02-01

    We describe a premature male infant with a terminal deletion of 7q [del(7) (pter----q34:)]. Manifestations include low birth weight, hypertelorism, bilateral cleft lip and palate, cryptorchidism, and a complex congenital heart defect. The latter consisted of hypoplasia of the main pulmonary artery, absent pulmonary valve, ventricular septal defect, and anomalous right pulmonary artery. We briefly review the spectrum of heart defects seen with chromosome 7 deletions, and comment on the incidence of this unusual heart lesion.

  12. Ablation of left-deviated dual atrioventricular nodal pathway from coronary sinus

    Institute of Scientific and Technical Information of China (English)

    HUANG Wei-bin; HONG Jiang; WANG Yan; ZHOU Fa-guang; ZENG Zhao-pin; GONG Yan; SUN Bao-gui; WANG Le-xin

    2009-01-01

    @@ Atrioventricular nodal reentrant tachycardia (AVNRT) is one of the most common types of paroxysmal supraventricular tachycardia. The mechanism of AVNRT is reentry associated with dual or multiple atrioventricular nodal (AVN) pathways. Typical AVNRT pathways,including fast and slow pathways, are confined in the right atrium. Radiofrequency catheter ablation of the slow pathway, and occasionally the fast pathway, has become the definitive treatment of choice for most symptomatic patients. Besides typical AVNRT, there exists some atypical AVNRT with various manifestations. Several groups have reported successful ablation of the leftward dual AVN pathway from the left side of the heart.1-3 We present one case of left-sided AVN as well as dual AVN pathway. The tachycardia was successfully eliminated by ablation of the slow pathway deep in the coronary sinus.

  13. [Innervation of the atrioventricular portion of the interatrial septum in the rat].

    Science.gov (United States)

    Pavlovich, E R

    1984-03-01

    A quantitative electron microscopical investigation has revealed reliable differences in contents of muscular, connective tissue and neural elements in the conductive system, on the one hand (in the atrioventricular node and in the internodal specialized pathways) and in the working myocardium of the inferior third of the interatrial septum, on the other hand. Morphometrical analysis of the neuromuscular interrelations, performed separately for every type of specialized conductive myocytes (II, III) and of working cardiomyocytes of the interatrial septum, reveals some differences in size of the neural fibers and their terminals and in distance between them and myocytes. The synaptic vesicles of the efferent terminals are qualitatively different in all types of the myocytes. The afferent terminals situating near specialized cardiac cells are characterized. Criteria for distinguishing small and large granular vesicles in the terminals are discussed. A suggestion is made that the innervational peculiarities revealed reflect certain specificity in regulation the conduction in the atrioventricular area of the heart.

  14. Atrioventricular block induced by mad-honey intoxication: confirmation of diagnosis by pollen analysis.

    Science.gov (United States)

    Cagli, Kumral Ergun; Tufekcioglu, Omac; Sen, Nihat; Aras, Dursun; Topaloglu, Serkan; Basar, Nur; Pehlivan, Sevil

    2009-01-01

    An unusual type of food poisoning, mad-honey intoxication, can be observed in the Black Sea region of Turkey and various other parts of the world. It can occur after ingestion of grayanotoxin-contaminated honey produced from the nectar of Rhododendron ponticum and other plant species, chiefly of the Ericaceae and Sapindaceae families. Mad-honey intoxication can result in severe cardiac complications, such as complete atrioventricular block. The diagnosis is generally reached on the basis of the patient's history of honey intake. In this report, we describe the case of a patient who had mad-honey-related complete atrioventricular block; in this instance, the diagnosis was confirmed by a pollen analysis of the suspect honey.

  15. A Rare Heart Rhythm Problem in Acute Rheumatic Fever: Complete Atrioventricular Block

    Directory of Open Access Journals (Sweden)

    Ayse Kibar

    2014-03-01

    Full Text Available Rheumatic heart disease remains the most important cause of acquired heart disease in children and young adults. Different kinds of rhythm and conduction disturbances may be seen during the course of acute rheumatic fever (ARF. Long PR intervals are found commonly in rheumatic fever, but complete atrioventricular (AV block is an exceptionally rare manifestation. This case report is about a 14 year-old-female patient diagnosed as ARF based on migratory arthralgia and mild carditis who also developed complete heart block on admission. Electrocardiogram on the 3rd day of hospitalization depicts 2nd degree atrioventricular block (Mobitz I combined with PR prolongation. The ECG revealed a normal sinus rhythm with PR prolongation on the 4th day of hospitalization. Rarely, complete AV heart block can occur as a complication of ARF and may develop during the acute phase

  16. Biological Glue Application in Repair of Atrioventricular Groove Rupture: A Case Report

    Directory of Open Access Journals (Sweden)

    Ahmet Baris-Durukan

    2015-10-01

    Full Text Available Atrioventricular groove rupture is a rare, albeit mortal, complication following mitral valve surgery. Avoidance is the best strategy but it cannot fully prevent the occurrence of this complication. Several repair techniques have been described with varying success rates; however, the rarity of the complication precludes consensus about the safest technique.Here we report two cases of posterior atrioventricular groove rupture. Both cases were diagnosed immediately after the cessation of cardiopulmonary bypass. Repair was performed successfully with a technique involving the use of biological glue. The postoperative course was uneventful for both of them. Both cases are well with normally functioning mitral prostheses; one with a follow-up time of 5.5 years and the other 10 months. We believe that the glue provides additional hemostasis and support to the repaired area.

  17. Atrium-atrioventricular node block: an unusual complication during catheter ablation of persistent atrial fibrillation

    Institute of Scientific and Technical Information of China (English)

    MIAO Cheng-long; SANG Cai-hua; DONG Jian-zeng; MA Chang-sheng

    2011-01-01

    Ablation of persistent atrial fibrillation is still a challenge for the ablationist. Extensive ablation is required under some conditions and could lead to some unintended complications. Here we report a case of atrium-atrioventricular node block complicating multiple catheter ablation procedures for persistent atrial fibrillation. After extensive ablation, including circumferential pulmonary vein ablation, linear ablation at the left atrial roof, mitral isthmus, atrial septum, cavotricuspid isthmus, and complex fractionated atrial electrogram ablation, conduction obstacle was found, and sinus impulse could not travel from the right atrium, atrial septum and left atrium to atrioventricular node. The case indicated that intensive ablation at some key sites, especially the interatrial septum, should be careful during ablation of atrial fibrillation.

  18. Electrical Injury-Induced Complete Atrioventricular Block: Is Permanent Pacemaker Required?

    Directory of Open Access Journals (Sweden)

    Osman Beton

    2015-01-01

    Full Text Available A considerable percentage of electrical injuries occur as a result of work activities. Electrical injury can lead to various cardiovascular disorders: acute myocardial necrosis, myocardial ischemia, heart failure, arrhythmias, hemorrhagic pericarditis, acute hypertension with peripheral vasospasm, and anomalous, nonspecific ECG alterations. Ventricular fibrillation is the most common arrhythmia resulting from electrical injury and is the leading cause of death in electrical (especially low voltage alternating current injury cases. Asystole, premature ventricular contractions, ventricular tachycardia, conduction disorders (various degrees of heart blocks, bundle-brunch blocks, supraventricular tachycardia, and atrial fibrillation are the other arrhythmic complications of electrical injury. Complete atrioventricular block has rarely been reported and permanent pacemaker was required for the treatment in some of these cases. Herein, we present a case of reversible complete atrioventricular block due to low voltage electrical injury in a young electrical technician.

  19. Complete paroxysmal atrioventricular block in a 2-year-old girl

    DEFF Research Database (Denmark)

    Holst, Line M; Dixen, Ulrik; Jeppesen, Dorthe L

    2014-01-01

    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...... to 6 seconds. A normal 12-lead electrocardiogram does not necessarily exclude cardiac disease, and Holter monitoring is warranted in children with atypical syncope....

  20. Bloqueo atrioventricular completo y reversible en un paciente con corazón estructuralmente sano

    Directory of Open Access Journals (Sweden)

    Oswaldo Gutiérrez-Sotelo

    2008-06-01

    Full Text Available Se presenta el caso de un paciente joven, deportista, quien presentó un episodio de síncope con pródromo breve, durante ejercicio físico. Se encontró el corazón estructuralmente normal y que durante la prueba de inclinación presentó bloqueo atrioventricular completo, que revirtió rápidamente con la posición de decúbito dorsal.

  1. The anatomy and histology of the atrioventricular conducting system in the hedgehog (Hemiechinus auritus) heart

    OpenAIRE

    NABIPOUR, Abolghasem

    2010-01-01

    This study examined the atrioventricular conducting system in 4 adult male hedgehogs (Hemiechinus auritus). The histological structure of these components was studied using routine histological methods. The AVN was located at the lower and anterior part of the interatrial septum, near the root of the aorta. It was almost oval and consisted of twisted cells. Internodal pathways in the hedgehog heart were not observed, but there were numerous purkinje-like fibers within the myocardium of the at...

  2. [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.

  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. Abnormalities of atrioventricular conduction in patients with the Lown-Ganong-Levine syndrome.

    Science.gov (United States)

    Pavlovic, J; Fabián, J; Belán, A

    1978-01-01

    In 18 patients with the Lown-Ganong-Levine syndrome and in 11 control subjects the response of the atrioventricular conduction system to heart rate acceleration by right atrial pacing, and, with the aid of His bundle electrogram, the behaviour of individual levels of the atrioventricular conduction system were studied. At spontaneous sinus rhythm A-H interval was shorter in the patients with the Lown-Ganong-Levine syndrome than in the controls. In the latter the A-H interval became markedly prolonged, up to a Wenckebach-type IInd-degree AV block, already at a minor pacing-induced acceleration of the heart rate, whereas in the patients with the Lown-Ganong-Levine syndrome the atrioventricular propagation of excitation at a 1:1 ratio remained preserved even at substantially higher heart-rate values. On the basis of the responses of the A-H interval to pacing the patients with the Lown-Ganong-Levine syndrome were classed into four types.

  6. Atrial activation during atrioventricular nodal reentrant tachycardia: Studies on retrograde fast pathway conduction

    NARCIS (Netherlands)

    D.G. Katritsis; K.A. Ellenbogen; A.E. Becker

    2006-01-01

    BACKGROUND Detailed right and left septal mapping of retrograde atrial activation during typical atrioventricutar nodal reentrant tachycardia (AVNRT) has not been undertaken and may provide insight into the complex physiology of AVNRT, especially the anatomic localization of the fast and stow pathwa

  7. Nasal septal abscess--retrospective analysis of 14 cases from University Hospital, Kuala Lumpur.

    Science.gov (United States)

    Jalaludin, M A

    1993-10-01

    Fourteen patients who presented to the University Hospital of Kuala Lumpur between June 1981 and June 1991 were reviewed retrospectively. Nasal septal abscesses are uncommon and therefore there are limited reports in the medical literature. Early diagnosis and immediate therapy is mandatory to avoid cosmetic nasal deformity or intracranial infection. Two out of the fourteen patients developed saddle nose deformity and septal perforation because of delay in treatment, the cases were misdiagnosed by non-otolaryngologist as turbinates swelling. The leading cause of nasal septal abscess was non-surgical trauma which accounted for about 85.7%. The commonest pathogenic organism isolated from the pus of nasal septal abscess was Staphylococcus aureus.

  8. Permanent pacemaker implantation and follow-up in 23 infants and children with perioperative complete atrioventricular block%23例先天性心脏病患儿围术期永久起博器的植入及随访

    Institute of Scientific and Technical Information of China (English)

    张惠丽; 李守军; 花中东; 杨克明; 胡盛寿; 张浩; 陈柯萍; 华伟

    2014-01-01

    目的:总结先天性心脏病(congenital heart diseases, CHD)儿童围术期植入永久性起搏器治疗完全性房室传导阻滞(atrioventricular block, AVB)的经验。方法选择需植入永久起搏器CHD患儿23例,男13例,女10例。年龄4个月~8岁,平均(2.8±2.5)岁,体重5~27 kg,平均(10.7±6.8)kg。结果植入心内膜起博器7例、心外膜起博器16例。起搏方式为频率应答心室按需起搏(VVIR)1例,固定频率心室按需起搏(VVI)22例,起搏频率为70~160次/min,平均(111±25.5)次/min,起搏阈值为0.5~0.75V,平均(0.74±0.30)V,感知灵敏度为2.5~2.8 mV,平均(2.78±0.11)mV,电极阻抗为271~726Ω,平均(445.5±148.9)Ω。21例(91.3%)获得随访,随访1个月~5.5年,起搏阈值术后早期略升高,后稳定。1例术后6个月猝死,原因不明。1例术后4.5年重新更换电池。1例随访中恢复窦性心律。4例术后6个月~2年心脏扩大、心功能低下,左室射血分数(LVEF)<50%。结论 CHD患儿围术期植入永久性起搏器治疗AVB是安全可行的,永久起搏器植入对其远期预后的影响需要长期随访。%Objectives We analyzed the results of 23 children with congenital heart diseases (CHD) associated complete atrioventricular block (AVB)who underwent pacemaker (PM) implantation at our center. Methods Between Jan-uary 2007 and July 2012, 23 children with CHD (13 males, 10 females, aged from 4 months to 8 years with the average of 2.8±2.5 years. Their weight ranged from 5 to 27 kg with the average of 10.7±6.8kg) underwent PM implantation for com-plete AVB at our center. Endocardial (ENDO) or epicardial (EPI) pacing systems were implanted in 7 and 16 patients re-spectively. Three patients (1 atrial septal defect(ASD)/pulmonary stenosis(PS),1 partial endocardial cushion defect (PECD) and 1 corrected transposition of the great arteries (CTGA) had complete AVB

  9. Ectopia cordis with endocardial cushion defect: Prenatal ultrasonographic diagnosis with autopsy correlation.

    Science.gov (United States)

    Balakumar, K; Misha, K

    2010-07-01

    The prenatal ultrasonographic diagnosis of ectopia cordis associated with a complex intra-cardiac defect (common atrium, common atrioventricular valve with single ventricle) is illustrated in a 32-week gestation fetus. The fetus showed associated features of amniotic band disruption sequence. The cardiac autopsy findings correlated with the antenatal diagnosis. The association of ectopia cordis with amniotic band disruption is rare and infrequently reported in literature.

  10. Ectopia cordis with endocardial cushion defect: Prenatal ultrasonographic diagnosis with autopsy correlation

    OpenAIRE

    Balakumar K; Misha K

    2010-01-01

    The prenatal ultrasonographic diagnosis of ectopia cordis associated with a complex intra-cardiac defect (common atrium, common atrioventricular valve with single ventricle) is illustrated in a 32-week gestation fetus. The fetus showed associated features of amniotic band disruption sequence. The cardiac autopsy findings correlated with the antenatal diagnosis. The association of ectopia cordis with amniotic band disruption is rare and infrequently reported in literature.

  11. Ectopia cordis with endocardial cushion defect: Prenatal ultrasonographic diagnosis with autopsy correlation

    Directory of Open Access Journals (Sweden)

    Balakumar K

    2010-01-01

    Full Text Available The prenatal ultrasonographic diagnosis of ectopia cordis associated with a complex intra-cardiac defect (common atrium, common atrioventricular valve with single ventricle is illustrated in a 32-week gestation fetus. The fetus showed associated features of amniotic band disruption sequence. The cardiac autopsy findings correlated with the antenatal diagnosis. The association of ectopia cordis with amniotic band disruption is rare and infrequently reported in literature.

  12. Iron deficiency and hemolytic anemia reversed by ventricular septal myectomy

    Science.gov (United States)

    Costa, Steven M.; Cable, Christian

    2015-01-01

    Hemolytic anemia has been reported to occur in the setting of aortic stenosis and prosthetic heart valves, but much more rarely in association with obstructive hypertrophic cardiomyopathy (HC). Of the few descriptions of hemolytic anemia secondary to HC, all but one case involved bacterial endocarditis contributing to left ventricular outflow tract obstruction. We present the case of a 67-year-old man with recurrent hemolytic anemia and HC, without infective endocarditis. Attempts at iron repletion and augmentation of beta-blocker therapy proved his anemia to be refractory to medical management. Ventricular septal myectomy led to the resolution of hemolysis, anemia, and its coexisting symptoms. PMID:26424952

  13. 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

  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. Ventricular septal rupture and right ventricular intramyocardial dissection secondary to acute inferior myocardial infarction.

    Science.gov (United States)

    Bittencourt, Márcio Sommer; Seltmann, Martin; Muschiol, Gerd; Achenbach, Stephan

    2010-01-01

    A 61-year-old female patient presented with sub acute myocardial infarction with an occluded right coronary artery on invasive evaluation and a ventricular septal rupture on echocardiogram. Cardiac computed tomography (CT) was performed to better define the septal anatomy. As the anatomy on cardiac CT was considered unfavorable for percutaneous intervention, the patient underwent successful surgical repair.

  16. Complete atrioventricular block in adult Sjögren's syndrome with anti-Ro autoantibody.

    Science.gov (United States)

    Sung, Myung Jun; Park, Sung-Hoon; Kim, Seong-Kyu; Lee, Young-Soo; Park, Chul-Yeon; Choe, Jung-Yoon

    2011-06-01

    Anti-Ro autoantibody is associated with Sjögren's syndrome (SS), systemic lupus erythematosus (SLE), and neonatal lupus syndrome (i.e., congenital complete heart block in newborns). Generally, the adult atrioventricular (AV) node is believed to be relatively resistant to the scarring effects of anti-Ro/anti-La autoantibodies. However, there have been some reports of adult complete AV block in SS and SLE patients. Here, we report a case of complete heart block in primary SS with anti-Ro autoantibodies, with no other risk factor for the development of heart block, and review their etiological association.

  17. Konno procedure using atrioventricular groove patch plasty after arterial switch operation.

    Science.gov (United States)

    Kosaka, Yoshimichi; Kurosawa, Hiromi; Nagatsu, Masayoshi

    2004-11-01

    A patient with transposition of the great arteries accompanied by Shaher type 9 coronary anatomy experienced the development of progressive neoaortic valvular regurgitation with a small annulus, supravalvular stenosis, and neopulmonary valvular and supravalvular stenoses 15 years after an arterial switch operation. To implant a prosthetic valve clinically adequate in size, the Konno procedure was necessary. However, the right coronary anatomy precluded the original Konno procedure. My colleagues and I accomplished neoaortic anterior annular enlargement in this case by using atrioventricular groove patch plasty without jeopardizing the right coronary artery, and this resulted in a satisfactory outcome.

  18. 房间隔部位介入治疗的焦点与思考%Focus and thinking of atrial septal interventional therapy

    Institute of Scientific and Technical Information of China (English)

    胡大一; 王显

    2009-01-01

    @@ 房间隔是先天性心脏病的好发部位之一,不少先天性心脏病的介入治疗是在房间隔部位完成的,如房间隔缺损(atrial septal defect,ASD)封堵术、卵圆孔未闭(patent foramen ovale,PFO)封堵术等.尽管目前房间隔部位的介入治疗成功率较高,但临床仍面临诸多难点,现提出一些热点问题与思考.

  19. Observed Prevalence of Congenital Heart Defects From a Surveillance Study in China

    Science.gov (United States)

    Zhang, Yali; Riehle-Colarusso, Tiffany; Correa, Adolfo; Li, Song; Feng, Xinheng; Gindler, Jacqueline; Lin, Hui; Webb, Catherine; Li, Wei; Trines, Jean; Berry, Robert J.; Yeung, Lorraine; Luo, Ying; Jiang, Meifang; Chen, Hua; Sun, Xiamei; Li, Zhu

    2015-01-01

    Objectives The purpose of this study was to estimate the prevalence of major and minor congenital heart defects among fetuses and neonates using sonography in a general population of 4 areas surrounding Shanghai, China. Methods Pregnant women were recruited between April 2004 and December 2005 in Jiaxing City, Suzhou City, Changshu County, and Haining County. All participants could have 3 sonographic examinations performed by specially trained physicians regardless of medical indication: a fetal sonographic screen and fetal echocardiography between 20 and 28 weeks’ gestation and neonatal echocardiography. Diagnoses of congenital heart defects were made on the basis of review of all available scans by an international group of experts in pediatric cardiology. Prevalence rates were calculated per 1000 births. Results Among 4006 scanned fetuses and neonates, there were 75 congenital heart defects, including 12 major defects. The observed prevalence for all congenital heart defects was 18.7 (95% confidence interval, 14.8–23.5) per 1000 births, and the prevalence for major defects was 3.0 (95% confidence interval, 1.6–5.2) per 1000 births. The most common defects were ventricular septal defects (n = 47 [62.7%]), atrial septal defects (n = 14 [18.7%]), tetralogy of Fallot (n = 4 [5.3%]), and hypoplastic left heart syndrome (n = 3 [4.0%]). Conclusions The prevalence of all congenital heart defects in the 4 areas of China studied was higher than that reported in other countries, with ventricular septal defects being the most frequent defects. Our data likely reflect a better estimate of the total prevalence of congenital heart defects in China than reported previously. PMID:21705732

  20. Redefining the Septal L-Strut to Prevent Collapse

    Science.gov (United States)

    Ha, Dong-Heon; Kim, Sung Won; Cho, Dong-Woo

    2016-01-01

    During septorhinoplasty, septal cartilage is frequently resected for various purposes but the L-strut is preserved. Numerous materials are inserted into the nasal dorsum during dorsal augmenation rhinoplasty without considering nasal structural safety. This study used a finite element method (FEM) to redefine the septal L-strut, to prevent collapse as pressure moved from the rhinion to the supratip breakpoint on the nasal dorsum and as the contact percentage between the caudal L-strut and the maxillary crest changed. We designed a 1-cm-wide L-strut model based on computed tomography data. At least 45% of the width of the L-strut in the inferior portion of the caudal strut must be preserved during septoplasty to stabilize the septum. In augmentation rhinoplasty, the caudal L-strut must either be preserved perfectly or reinforced to prevent collapse or distortion of the L-strut. The dorsal augmentation material must be fixed in an augmentation pocket to prevent movement of graft material toward the supratip breakpoint, which can disrupt the L-strut. We conducted a numerical analysis using a FEM to predict tissue/organ behavior and to help clinicians understand the reasons for target tissue/organ collapse and deformation. PMID:27073993

  1. Pheochromocytoma-induced atrial tachycardia leading to cardiogenic shock and cardiac arrest: resolution with atrioventricular node ablation and pacemaker placement.

    Science.gov (United States)

    Shawa, Hassan; Bajaj, Mandeep; Cunningham, Glenn R

    2014-12-01

    Pheochromocytoma should be considered in young patients who have acute cardiac decompensation, even if they have no history of hypertension. Atrioventricular node ablation and pacemaker placement should be considered for stabilizing pheochromocytoma patients with cardiogenic shock due to atrial tachyarrhythmias. A 38-year-old black woman presented with cardiogenic shock (left ventricular ejection fraction, cardiogenic shock and cardiac arrest unresolved by the placement of 2 different ventricular assist devices, but that was completely reversed by radiofrequency ablation of the atrioventricular node and the placement of a temporary pacemaker. We present the patient's clinical, laboratory, and imaging findings, and we review the relevant literature.

  2. A case of Kearns-Sayre sindrome with autoimmune thyroiditis and complete atrio-ventricular block.

    Science.gov (United States)

    Berio, A; Piazzi, A

    2006-06-01

    The Kearns-Sayre syndrome, (characterized by its onset before the age of 20 years, chronic ophthalmoplegia, pigmentary retinal degeneration and at least one of the following symptoms: ataxia, heart block and high protein content in the cerebrospinal fluid) is a severe variant of chronic progressive external ophthalmoplegia with frequent rearrangements of the mitochondrial DNA (mtDNA). The aim of this paper is to report a sporadic paediatric case of Kearns-Sayre syndrome with mtDNA heteroplasmic deletion, absence of cytochrome c-oxidase in many muscle fibers, autoimmune thyroiditis, complete atrio-ventricular heart block in which the diagnosis of subclinical hypothyroiditis associated with autoimmune thyroid disease was made. The subclinical hypothyroidism, more severe in the presence of thyroid antibodies, may have contributed to the pathogenesis of cardiovascular disease. We hypothesized that in this patient, predisposed by mitochondrial deletion, anti-thyroid antibodies may have interfered with the mitochondrial function of conduction heart system, causing atrio-ventricular heart block. It seems important to study anti-thyroid antibodies in every case of Kearn-Sayre syndrome, specially if cardiac rhythm disturbances are present.

  3. Ruptured Sinus of Valsalva Aneurysm Initially Misdiagnosed as Ventricular Septal Defect by Echocardiography

    Directory of Open Access Journals (Sweden)

    GR Rezaian

    2009-06-01

    Full Text Available Aneurysms of sinus of valsalva are rare cardiac lesions and most of them are in congenital origin. The malformation consists of a separation or lack of fusion between the media of the aorta and the annulus fibrosis of the aortic valve. The structure becomes aneurysmal and may rupture to form a fistula. We present a case of ruptured sinus of valasalva aneurysm in a 25-year-old man. The diagnosis was made by echocardiography and confirmed at operation.

  4. Sinus venosus atrial septal defect with severe mitral stenosis: A rare presentation

    Directory of Open Access Journals (Sweden)

    Shweta Shende

    2015-01-01

    Sinus venosus ASD are uncommon and constitute 2-3% of interatrial communication. However, sinus venosus ASD with mitral stenosis is an extremely rare condition which was treated successfully by patch closure with left atrialization of superior pulmonary vein and mitral valve replacement.

  5. [Prognostic factors associated with postoperative morbidity in children with isolated ventricular septal defect].

    Science.gov (United States)

    Castro-Rodríguez, Claudia Olimpia; Rodríguez-Hernández, Lydia; Estrada-Loza, María Jesús; Herrera-Márquez, Julia Rocío; Gómez-Salvador, Maricela; Flores-Lujano, Janet; Núñez-Enríquez, Juan Carlos

    2015-01-01

    Introducción: la comunicación interventricular aislada (CIV) es una de las cardiopatías congénitas más frecuentes a nivel mundial. Se desconoce cuáles son los factores pronósticos asociados a la morbilidad postoperatoria de los pacientes con CIV aislada en el Hospital de Pediatría del Centro Médico Nacional Siglo XXI. Métodos: se realizó un estudio de cohorte retrospectivo del 1 de enero de 2009 al 31 de diciembre de 2013. Se utilizó estadística descriptiva y comparaciones entre los grupos mediante chi cuadrada, U de Mann Whitney y regresión lineal. Se calcularon riesgos relativos (RR) e intervalos de confianza al 95 % (IC 95%). Resultados: se incluyeron 64 pacientes a quienes se les realizó cierre de comunicación interventricular aislada. El 12.5 % (n = 8) tenían antecedente de cerclaje pulmonar y 22 (34.3 %) pacientes tenían síndrome de Down. Las CIV perimembranosas y la subaórtica se presentaron en 70 % de los casos. Los factores asociados a una mayor morbilidad y estancia prolongada en terapia intensiva fueron: lactato al final de la circulación extracorpórea (CEC) mayor a 3mmol/L (p = 0.02) y el síndrome de Down (p = 0.02) quienes presentaron una mayor frecuencia de complicaciones postoperatorias (p = 0.02). Conclusiones: se encontró una asociación estadísticamente significativa: cuanto mayor era la clase funcional preoperatoria, mayor era su estancia intrahospitalaria, con mayor número de días en terapia intensiva y mayor tiempo de ventilación mecánica asistida.

  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. Anesthetic considerations of an emergency decompressive craniotomy complicated with Ebstein's anomaly and atrial septal defect

    Institute of Scientific and Technical Information of China (English)

    XIN Xin; TANG Shuai; WANG Ling; ZHAO Jing; LI Gui-lin; GUO Li-lin; HUANG Yu-guang

    2011-01-01

    Despite considerable published papers regarding Ebstein's anomaly (EA) patients receiving open-heart tricuspid valve replacement, non-cardiac emergency surgeries were rarely reported. We report a case of emergency decormpressive craniotormy in a patient with EA. Anesthesiologists should pay special attention to the complications and anesthetic management during the non-cardiac surgeries performed in EA patients.hile papers regarding Ebstein's anomaly (EA)patients receiving open-heart tricuspid valve replacement are numerous, the reports of non-cardiac emergency surgeries have not been to the same degree.We report a case of emergency decompressive craniotomy in a patient with EA. This case report describes the symptoms and anesthetic management during the surgery.

  8. Defeito do septo atrioventricular forma total associado a tricuspidização da valva atrioventricular esquerda na infância

    Directory of Open Access Journals (Sweden)

    DOBRIANSKYJ Aleksander

    1997-01-01

    Full Text Available Entre maio de 1987 e dezembro de 1996, o reparo do defeito do septo atrioventricular, forma total (DSAVT, com duplo retalho de pericárdio bovino e tricuspidização da valva atrioventricular (VAV esquerda, foi realizado em 34 pacientes consecutivos com mortalidade global de 5 (14,7% pacientes (pac.. Grupo I: abaixo de 6 meses (m com 12 pac. (1 óbito; 8,3%; Grupo II: entre 7m e 12m, com 15 pac. (4 óbitos; 26,6% e Grupo III: entre 1 e 5 anos (a com 7 pac. sem óbitos. Ocorreram 2 óbitos tardios (1 por Insuficiência mitral residual + insuficiência cardíaca congestiva e outro por pneumonia + insuficiência respiratória. Duas crianças receberam implante de marcapasso definitivo (após 45 dias e 4 anos. Nenhum paciente foi reoperado, apesar de mais 2 apresentarem insuficiência da VAV esquerda e 1 VAV direita, controlada clinicamente, e as restantes encontram-se nos graus I e II da New York Heart Association (NYHA. Apesar de 29 pacientes apresentarem peso abaixo de 10 kg, utilizou-se circulação extracorpórea (CEC e hipotermia a 25°C sem parada circulatória total. Iniciou-se o reparo pela zona de "aposição" da VAV única com um ou dois pontos em "x", mantendo tricúspide. Sutura-se o primeiro retalho de pericárdio bovino (PB em forma de "gota", insinuando-o entre as cordas do lado direito do septo; a seguir, sutura-se o segundo retalho, fazendo uma aposição concomitante com a VAV única, septando-se, desta forma, as quatro cavidades. As vantagens desta técnica são: preservação da integridade valvar, maior durabilidade funcional da VAV esquerda, menor possibilidade de reoperação mesmo em pacientes mais jovens e que devem ser preferencialmente operados em torno de 6º mês de vida.

  9. Prevalence of Congenital Heart Defects in Metropolitan Atlanta, 1998–2005

    Science.gov (United States)

    Reller, Mark D.; Strickland, Matthew J.; Riehle-Colarusso, Tiffany; Mahle, William T.; Correa, Adolfo

    2008-01-01

    Objective To determine an accurate estimate of the prevalence of congenital heart defects (CHD) using current standard diagnostic modalities. Study design We obtained data on infants with CHD delivered during 1998–2005 identified by the Metropolitan Atlanta Congenital Defects Program, an active, population-based birth defects surveillance system. Physiologic shunts in infancy and shunts associated with prematurity were excluded. Selected infant and maternal characteristics of the cases were compared with those of the overall birth cohort. Results From 1998–2005 there were 398 140 births, of which 3240 infants had CHD, for an overall prevalence of 81.4/10 000 births. The most common CHD were muscular ventricular septal defect, perimembranous ventricular septal defect, and secundum atrial septal defect, with prevalence of 27.5, 10.6, and 10.3/10 000 births, respectively. The prevalence of tetralogy of Fallot, the most common cyanotic CHD, was twice that of transposition of the great arteries (4.7 vs. 2.3/10 000 births). Many common CHD were associated with older maternal age and multiple-gestation pregnancy; several were found to vary by sex. Conclusion This study, using a standardized cardiac nomenclature and classification, provides current prevalence estimates of the various CHD subtypes. These estimates can be used to assess variations in prevalence across populations, time or space. PMID:18657826

  10. Atrial Fibrillation in Hypertrophic Cardiomyopathy: Is the Extent of Septal Hypertrophy Important?

    Science.gov (United States)

    Park, Kyoung-Min; Im, Sung Il; Kim, Eun Kyoung; Lee, Sang-Chol; Park, Seung-Jung; Kim, June Soo; On, Young Keun

    2016-01-01

    Hypertrophic cardiomyopathy (HCM) is a cardiac disease associated with a high incidence of atrial fibrillation (AF). Recent studies have suggested that interventricular septum thickness may influence the risk stratification of patients with AF. We evaluated the effects of septal hypertrophy on morbidity and mortality in patients with HCM. Patients were followed for a median of 6.1 years and were divided into two groups according to the extent of septal hypertrophy. A total of 1,360 HCM patients were enrolled: 482 (33%) apical or apicoseptal, 415 (28%) asymmetric septal, 388 (27%) basal septal, 38 (2.6%) concentric, and 37 (2.5%) diffuse and mixed type. Ninety-two all-cause deaths and 21 cardiac deaths occurred. The total event rates were significantly higher for patients with HCM with more extensive septal hypertrophy (group A) compared to those with HCM ± focal septal hypertrophy (group B), regardless of type (p<0.001). Arrhythmias occurred in 502 patients, with a significantly higher incidence in group A than in group B (p<0.001). Among patients with arrhythmias, the incidence of AF was significantly higher in group A than group B (p<0.001). In univariate Cox analysis, a greater extent of septal hypertrophy (p<0.001), E/E´ ratio (p = 0.011), and mitral regurgitation grade (p = 0.003) were significantly associated with developing AF. In multivariate Cox analyses, a greater extent of septal hypertrophy [odds ratio (OR) 5.44 (2.29-12.92), p<0.001] in patients with HCM was significantly associated with developing AF. In conclusion, a greater extent of septal hypertrophy is an independent predictor of progression to AF in patients with HCM.

  11. EFFECT OF METOPROLOL ON ATRIAL FIBRILLATORY RATE, ATRIOVENTRICULAR NODAL CONCEALED CONDUCTION, AND VENTRICULAR RESPONSE DURING ATRIAL-FIBRILLATION IN PIGS

    NARCIS (Netherlands)

    VANDENBERG, MP; DELANGEN, CDJ; CRIJNS, HJGM; HAAKSMA, J; BEL, KJ; WESSELING, H; LIE, KI

    1994-01-01

    We wished to elucidate the effect of beta-blockade on fibrillatory rate and atrioventricular (AV) nodal concealed conduction during atrial fibrillation (AF). Subsequent to determination of the effect on atrial functional refractoriness with the extrastimulus technique (basic cycle length 400 ms), th

  12. [Trans-septal endoscopic approach of pituitary tumors].

    Science.gov (United States)

    Muñoz del Castillo, F; Jurado Ramos, A; De la Riva Aguilar, A; López Villarejo, P

    2003-10-01

    The advances in endoscopic instruments have eased the approach to the sellar region through the nasal cavity. We carry out an analysis of the surgical results on 20 patients that underwent surgery for sellar tumours through a transeptal-transphenoidal approach in the last 2 years in our hospital. The average was 45.6 years old, and 75% were females. 30% of cases were pituitary adenomas and another 30% acromegaly, 25% Cushing's disease and 10% prolactinomas. No complications were encountered during surgery being the most common postoperative complications, diabetes insipida in two cases (10%) and CSF leak in one case. At present 2 patients are having hormonal treatment for panhypopituitarism. No patients developed a septal perforation, nasal deformity, epistaxis, meningitis, lip numbness or oronasal fistula. The rest did have good results noith no recurrence and hormonal values back to normal.

  13. Leiomyosarcoma with coronary fistulae and ventricular septal perforation:A case study

    Institute of Scientific and Technical Information of China (English)

    Dang-Sheng HUANG; Yu-Mei WANG; Yu CHEN

    2014-01-01

    Coronary fistulae and ventricular septal perforation are very rare clinically, and even less caused by cardiac leiomyosarcoma. A case is reported that a 67-year-old female had cardiac leiomyosarcoma with progressive heart failure and coronary fistulae and ventricular septal perforation. This case was special since all ante-mortem examinations and cardiac surgery failed to detect the presence of any abnormal car-diac mass. Therefore, the malignant cardiac tumors could appear in an invasive form without mass and be one of the causes of the coronary fistulae and ventricular septal perforation.

  14. Cx30.2 enhancer analysis identifies Gata4 as a novel regulator of atrioventricular delay

    Science.gov (United States)

    Munshi, Nikhil V.; McAnally, John; Bezprozvannaya, Svetlana; Berry, Jeff M.; Richardson, James A.; Hill, Joseph A.; Olson, Eric N.

    2009-01-01

    Summary The cardiac conduction system comprises a specialized tract of electrically coupled cardiomyocytes responsible for impulse propagation through the heart. Abnormalities in cardiac conduction are responsible for numerous forms of cardiac arrhythmias, but relatively little is known about the gene regulatory mechanisms that control the formation of the conduction system. We demonstrate that a distal enhancer for the connexin 30.2 (Cx30.2, also known as Gjd3) gene, which encodes a gap junction protein required for normal atrioventricular (AV) delay in mice, is necessary and sufficient to direct expression to the developing AV conduction system (AVCS). Moreover, we show that this enhancer requires Tbx5 and Gata4 for proper expression in the conduction system, and Gata4+/- mice have short PR intervals indicative of accelerated AV conduction. Thus, our results implicate Gata4 in conduction system function and provide a clearer understanding of the transcriptional pathways that impact normal AV delay. PMID:19592579

  15. [Supra-His complete atrioventricular block in a patient with subclinical hyperthyroidism].

    Science.gov (United States)

    Amasyalı, Basri; Barçın, Cem; Kılıç, Ayhan

    2011-12-01

    Subclinical hyperthyroidism is a difficult entity to diagnose because of silent clinical features and it may be easily underdiagnosed unless it is suspected and thyroid hormone levels are examined. Although atrioventricular (AV) conduction abnormalities such as complete heart block may occasionally be seen in hyperthyroidism, its association with subclinical hyperthyroidism has not been reported previously. We report on a 50-year-old female patient who did not have any systemic or cardiovascular disease or history of drug use that could affect AV conduction and presented with presyncope and complete heart block with narrow QRS complexes. Thyroid function tests showed subclinical hyperthyroidism and an electrophysiological study showed the supra-His level as the site of complete AV block. After initiation of antithyroid treatment (propylthiouracil), the patient's rhythm improved to second-degree AV block on the third day and returned to normal sinus rhythm on the fourth day.

  16. RADIOFREQUENCY CURRENT CATHETER ABLATION OF THE LEFT ATRIOVENTRICULAR ACCESSORY PATHWAYS WITH PAROXYSMAL SUPRAVENTRICULAR TACHYCARDIA

    Institute of Scientific and Technical Information of China (English)

    王静毅; 郭继鸿; 吴益明; 朱继红; 王伟民; 赵红; 刘喜荣; MichaelA.Lee

    1994-01-01

    Seventy patients with left atrioventricular accessory pathways and paroxysmal supraventricular tachycardia (PSVT) underwent radiofrequency catheter ablation (RFCA).The success rate was 94.3%.Among these pa-tients,26 had manifest preexcitation syndrome,and 44 had concealed preexcitation.Eighteen patients with con-cealed preexcitation underwent coronary sinus(CS) pacinga,and delta wave appeared in 15.The keys to successful RFCA were correct positioning of the radiofrequency (RF) catheter tip,A/V amplitude ratio,AV interval(in si-nus rhythm)and VA interval(during SVT or ventricular pacing).After 1-14 months of follow-up,two pa- tients had supraventricular tachycardia(SVT)recurrence.

  17. Patologia do bloqueio atrioventricular na cardiomiopatia por depósito de desmina

    Directory of Open Access Journals (Sweden)

    Luiz Alberto Benvenuti

    2012-01-01

    Full Text Available Geralmente, a cardiomiopatia restritiva por deposição de desmina é caracterizada pela restrição ao enchimento diastólico ventricular e por diferentes graus de bloqueio atrioventricular (BAV. Neste relato, são descritas as alterações anatomopatológicas do sistema de condução cardíaco relacionadas ao BAV. O nó sinusal, o nó compacto e o feixe penetrante (feixe de His não apresentavam anormalidades, entretanto, havia extensa fibrose das porções terminais do feixe ramificante e do início dos feixes esquerdo e direito, no topo do septo ventricular. A patogenia dessa substituição fibrosa é provavelmente a mesma que origina a extensa fibrose do miocárdio ventricular contrátil, e permanece por ser elucidada.

  18. O nódulo atrioventricular e o feixe de his no homem

    Directory of Open Access Journals (Sweden)

    C. Magarinos Torres

    1961-09-01

    Full Text Available A demonstração do nódulo atrioventricular e do feixe de His, no coração humano, não oferece dificuldade especial, uma vez que seja seguida a técnica consagrada. A negação da sua existência, feita há cêrca de 20 anos atraz, é atribuível à não observância dêsse cuidado. A documentação apresentada refere-se a um paciente do sexo feminino, de côr branca, com 36 anos de idade, não infectado pelo Schizotrypanum cruzi, cujo coração pesava 310 g, sendo considerado macro e microscòpicamente, normal. Destina-se ela a servir de têrmo de comparação para a que será publicada, oportunamente, sôbre a cardiopatia crônica na doença de Chagas. Confirmamos o que dizem muitos autores quando assinalam a ausência completa de células ganglionares e de nervos, no nódulo atrioventricular, no tronco comum do feixe His e em seus dois ramos, quando observados no homem. Contrasta isso, fortemente, com o que existe, nas mesmas estruturas, no coração dos ungulados. Verificamos, não raramente, continuidade entre fibras musculares do tecido específico, no ramo esquerdo do feixe de His e fibras musculares ventriculares, sendo o ramo esquerdo constituído, em certa proporção, por fibras com caracters intermediários entre fibras musculares ordinárias e fibras de Purkinje (fibras de transição. Algumas vêzes fibras com características das de Purkinie aparecem em grupos musculares do septo ventricular imediatamente adjacentes ao ramo esquerdo, dificultando a demarcação exata daquele ramo. Reproduzimos, textualmente, pontos de mior interêsse, na literatura consultada, quando receavamos que a tradução não pudesse traduzir o pensamento exato do autor ou pudesse dar tal impressão.No particular difficulty was met in the demonstration of the atrioventricular node and bundel of His in the human heart when standard methods such as that described by RÉNON and GÉRAUDEL (posteriorly referred as MAHAIM's method was followed. The figures

  19. Transient complete atrioventricular block in a preterm neonate with congenital myotonic dystrophy: case report.

    Science.gov (United States)

    Kim, Hee Na; Cho, Young Kuk; Cho, Joo Hyun; Yang, Eun Mi; Song, Eun Song; Choi, Young Youn

    2014-06-01

    Congenital myotonic dystrophy (CMD) is an inherited neuromuscular disorder with cardiac rhythm abnormalities that may occur as a child grows. No report has described complete atrioventricular (AV) block detected in a neonate with CMD. We report a floppy infant of 31(+4) weeks gestation with complete AV block at birth, who was diagnosed with CMD by Southern analysis. She recovered from complete AV block 32 hr after temporary transcutaneous pacing was applied. To the best our knowledge, this is the first recorded case of a complete AV block accompanied by CMD during the neonatal period. When a newborn has a complete AV block, the physician should consider the possibility of the CMD and conduct a careful physical examination.

  20. TAQUICARDIA POR REENTRADA DEL NODO AURÍCULO-VENTRICULAR / Atrioventricular nodal reentrant tachycardia

    Directory of Open Access Journals (Sweden)

    Jéssica Mirella Mercedes

    2013-01-01

    Full Text Available Resumen La taquicardia por reentrada del nodo aurículo–ventricular representa una parte significativa de las taquiarritmias que los médicos asisten en los servicios de urgencias. Constituye la taquicardia paroxística más frecuente, y es característico que se presente sobre todo en mujeres entre la cuarta y la quinta década de la vida. El diagnóstico puede hacerse con alta fiabilidad mediante el electrocardiograma de superficie y algunos casos complejos o dudosos, mediante los estudios electrofisiológicos. Su tratamiento debe ser dirigido hacia la curación definitiva mediante la técnica de ablación endocavitaria, pero de acuerdo a su disponibilidad o las preferencias del paciente se puede indicar tratamiento farmacológico. En este artículo se resumen las técnicas diagnósticas, los tipos de taquicardia por reentrada intranodal y los principales aspectos del tratamiento. / Abstract Atrioventricular nodal reentrant tachycardia represents a significant part of tachyarrhythmias seen by attending physicians in emergency departments. It is the most common paroxysmal tachycardia, and it occurs primarily in women between their fourth and fifth decades of life. Diagnosis can be made with high reliability by surface electrocardiogram and complex or borderline cases by electrophysiological studies. Its treatment should be aimed towards definitive healing by endocardial ablation technique, but depending on availability or patient preferences drug treatment may be indicated. This article summarizes the diagnostic techniques, types of atrioventricular nodal reentrant tachycardia and major aspects of treatment.

  1. [Funtcional and morphological changes in nasal mucosa in patients presenting with septal deformation].

    Science.gov (United States)

    Piskunov, V S; Mezentseva, O Iu

    2011-01-01

    This study was designed to obtain morpho-functional characteristics of nasal mucosa associated with septal deformation. It has demonstrated disturbances in the mucociluary transport, compromised mucosal immunity, and morphological changes in the nasal cavity.

  2. Short-term prognosis and risk factors of ventricular septal rupture following acute myocardial infarction

    Institute of Scientific and Technical Information of China (English)

    胡小莹

    2013-01-01

    Objective To analyze the short-term prognosis and risk factors of ventricular septal rupture(VSR)following acute myocardial infarction(AMI).Methods A total of 70 consecutive VSR patients following AMI hospitalized in

  3. Short-term prognosis and risk factors of ventricular septal rupture following acute myocardial infarction

    Institute of Scientific and Technical Information of China (English)

    胡小莹

    2013-01-01

    Objective To analyze the short-term prognosis and risk factors of ventricular septal rupture(VSR)following acute myocardial infarction(AMI).Methods A total of 70 consecutive VSR patients following AMI

  4. Microvascular permeability changes might explain cardiac tamponade after alcohol septal ablation for hypertrophic cardiomyopathy.

    Science.gov (United States)

    Hsu, Jen-Te; Hsiao, Ju-Feng; Chang, Jung-Jung; Chung, Chang-Min; Chang, Shih-Tai; Pan, Kuo-Li

    2014-04-01

    Various sequelae of alcohol septal ablation for hypertrophic obstructive cardiomyopathy have been reported. Of note, some cases of cardiac tamponade after alcohol septal ablation cannot be well explained. We describe the case of a 78-year-old woman with hypertrophic obstructive cardiomyopathy in whom cardiac tamponade developed one hour after alcohol septal ablation, probably unrelated to mechanical trauma. At that time, we noted a substantial difference in the red blood cell-to-white blood cell ratio between the pericardial effusion (1,957.4) and the peripheral blood (728.3). In addition to presenting the patient's case, we speculate that a possible mechanism for acute tamponade--alcohol-induced changes in microvascular permeability--is a reasonable explanation for cases of alcohol septal ablation that are complicated by otherwise-unexplainable massive pericardial effusions.

  5. Asymmetric septal hypertrophy - a marker of hypertension in aortic stenosis (a SEAS substudy)

    DEFF Research Database (Denmark)

    Tuseth, Nora; Cramariuc, Dana; Rieck, Ashild E

    2010-01-01

    Some patients with aortic stenosis develop asymmetric septal hypertrophy (ASH) that may influence the surgical approach and is associated with higher perioperative morbidity. The aim of this analysis was to characterize further this subtype of aortic stenosis patients....

  6. Interventricular Septal Hematoma and Coronary-Ventricular Fistula: A Complication of Retrograde Chronic Total Occlusion Intervention

    Directory of Open Access Journals (Sweden)

    Abdul-rahman R. Abdel-karim

    2016-01-01

    Full Text Available Interventricular septal hematoma is a rare complication of retrograde chronic total occlusion (CTO percutaneous coronary interventions (PCI with a typically benign course. Here we report two cases of interventricular septal hematoma and coronary-cameral fistula development after right coronary artery (RCA CTO-PCI using a retrograde approach. Both were complicated by development of ST-segment elevation and chest pain. One case was managed actively and the other conservatively, both with a favorable outcome.

  7. Asymmetric septal hypertrophy - a marker of hypertension in aortic stenosis (a SEAS substudy)

    DEFF Research Database (Denmark)

    Tuseth, Nora; Cramariuc, Dana; Rieck, Ashild E

    2010-01-01

    Some patients with aortic stenosis develop asymmetric septal hypertrophy (ASH) that may influence the surgical approach and is associated with higher perioperative morbidity. The aim of this analysis was to characterize further this subtype of aortic stenosis patients.......Some patients with aortic stenosis develop asymmetric septal hypertrophy (ASH) that may influence the surgical approach and is associated with higher perioperative morbidity. The aim of this analysis was to characterize further this subtype of aortic stenosis patients....

  8. Interventricular Septal Hematoma and Coronary-Ventricular Fistula: A Complication of Retrograde Chronic Total Occlusion Intervention

    Science.gov (United States)

    Abdel-karim, Abdul-rahman R.; Main, Michael L.

    2016-01-01

    Interventricular septal hematoma is a rare complication of retrograde chronic total occlusion (CTO) percutaneous coronary interventions (PCI) with a typically benign course. Here we report two cases of interventricular septal hematoma and coronary-cameral fistula development after right coronary artery (RCA) CTO-PCI using a retrograde approach. Both were complicated by development of ST-segment elevation and chest pain. One case was managed actively and the other conservatively, both with a favorable outcome. PMID:27668097

  9. Msx1 and Msx2 are required for endothelial-mesenchymal transformation of the atrioventricular cushions and patterning of the atrioventricular myocardium

    Directory of Open Access Journals (Sweden)

    Maxson Robert E

    2008-07-01

    Full Text Available Abstract Background Msx1 and Msx2, which belong to the highly conserved Nk family of homeobox genes, display overlapping expression patterns and redundant functions in multiple tissues and organs during vertebrate development. Msx1 and Msx2 have well-documented roles in mediating epithelial-mesenchymal interactions during organogenesis. Given that both Msx1 and Msx2 are crucial downstream effectors of Bmp signaling, we investigated whether Msx1 and Msx2 are required for the Bmp-induced endothelial-mesenchymal transformation (EMT during atrioventricular (AV valve formation. Results While both Msx1-/- and Msx2-/- single homozygous mutant mice exhibited normal valve formation, we observed hypoplastic AV cushions and malformed AV valves in Msx1-/-; Msx2-/- mutants, indicating redundant functions of Msx1 and Msx2 during AV valve morphogenesis. In Msx1/2 null mutant AV cushions, we found decreased Bmp2/4 and Notch1 signaling as well as reduced expression of Has2, NFATc1 and Notch1, demonstrating impaired endocardial activation and EMT. Moreover, perturbed expression of chamber-specific genes Anf, Tbx2, Hand1 and Hand2 reveals mispatterning of the Msx1/2 double mutant myocardium and suggests functions of Msx1 and Msx2 in regulating myocardial signals required for remodelling AV valves and maintaining an undifferentiated state of the AV myocardium. Conclusion Our findings demonstrate redundant roles of Msx1 and Msx2 in regulating signals required for development of the AV myocardium and formation of the AV valves.

  10. Collagen immunostains can distinguish capsular fibrous tissue from septal fibrosis and may help stage liver fibrosis.

    Science.gov (United States)

    Chen, Wei; Rock, Jonathan B; Yearsley, Martha M; Hanje, A James; Frankel, Wendy L

    2014-01-01

    Core-needle biopsy remains essential for diagnosis of cirrhosis; however, evaluation of fibrosis in such biopsies is often challenging due to the fragmented nature of cirrhotic liver specimens. It is also common to see portions of liver capsules present in the biopsy which adds to the diagnostic challenge. The distinction between capsular/subcapsular fibrous tissue and septal fibrosis is critical to avoid potential overstaging of liver fibrosis. We compared the differential immunostaining in liver capsular and septal areas for collagens III, IV, V, VI, vitronectin, laminin, Orcein, and Trichrome in 15 whole sections of explanted cirrhotic livers and 5 simulated liver biopsies. Collagens III, IV, V, VI, Trichrome, and Orcein show distinct staining patterns in capsular fibrous tissue and septal fibrosis. Collagen IV shows strong diffuse septal staining and consistently weak to negative capsular staining. Collagens III and VI stain similar to IV for septal fibrosis, whereas collagen V, Trichrome, and Orcein show strong staining in both areas. Collagen IV, possibly with III or VI in addition to the routine Trichrome and hematoxylin and eosin stain, is useful in differentiating capsular fibrous tissue from septal fibrosis on challenging and fragmented liver biopsies.

  11. [Septal alcohol ablation in patients with hypertrophic cardiomyopathy].

    Science.gov (United States)

    López-Aburto, Gustavo; Palacios-Rodríguez, Juan Manuel; Cantú-Ramírez, Samuel; Galván-García, Eduardo; Tolosa-Dzul, Gonzalo; Morán-Benavente, Armando; Ontiveros-Martínez, Raúl

    2013-01-01

    Objetivo: conocer características demográficas, clínicas y hemodinámicas de los pacientes con ablación con alcohol para tratar la miocardiopatía hipertrófica septal obstructiva (MHSO). Métodos: estudio observacional, longitudinal, descriptivo de 21 pacientes con MHSO resistente a tratamiento o con gradiente = 30 mm Hg en reposo o = 60 mm Hg provocado y con movimiento sistólico anterior o insuficiencia mitral > grado II. Resultados: edad de 50 ± 16 años; hombres 38.1 % y mujeres 61.9 %. Los síntomas fueron angor 42.9 %, disnea 85.7 % y síncope 23.8 %. La clase funcional NYHA preablación fue grados III y IV en 61.9 %; al año todos tenían grados I y II. Preablación y al año, el espesor del septo interventricular fue de 22.7 ± 4.9 y 20.7 ± 3.1 mm. Preablación, después y al año, la fracción de eyección fue de 65.5 ± 7, 62.2 ± 6.5 y 68.7 ± 6.2 %. Preablación, después y al año, el gradiente del tracto de salida del ventrículo izquierdo fue de 106.9 ± 29.9, 44.6 ± 24.3 y 22.0 ± 5.7 mm Hg. Preablación, la insuficiencia mitral fue grados III y IV en 33.3 y 47.6 % y al año fue grados 0 en 52.4 %, I en 28.6 % y II en 19 %. No hubo defunciones intrahospitalarias. Conclusiones: la ablación septal con alcohol en pacientes con MHSO fue exitosa.

  12. Department of Defense Birth and Infant Health Registry: Birth Defects Among Infants Born to US Military Families: 2001 Annual Report

    Science.gov (United States)

    2008-05-30

    large intestinal atresia/stenosis 751.2 Anophthalmia/microphthalmia 743.0, 743.1 Pyloric stenosis 750.5 Congenital cataract 743.30-743.34...occurred in US military families worldwide. Data on birth defects were gathered using nationally standardized definitions for major congenital ...745.4 Congenital hip dislocation 754.30, .31, .35 Atrial septal defect 745.5 Reduction deformity, upper limbs 755.20-755.29 Endocardial cushion

  13. Asymmetrical septal hypertrophy in newborn infants of diabetic mothers.

    Science.gov (United States)

    Vela-Huerta, M M; Vargas-Origel, A; Olvera-López, A

    2000-01-01

    The objective of this paper is to determine the frequency and outcome of asymmetrical septal hypertrophy (ASH) in large-for-gestational-age infants (LGA) born to diabetic (DM) and nondiabetic mothers (NDM), and to establish the relationship between ASH and maternal diabetes control. A comparative study was design to assess ASH in infants born to DM and NDM. The study was conducted in the Departments of Neonatology and Pediatric Cardiology of the "Hospital de Gineco-Pediatria 48", Instituto Mexicano del Seguro Social from January to December 1997. Eighty-five full-term infants of DM (group A) and 85 LGA infants of NDM (group B) were included. As a control group (group C), we studied 85 healthy, full-term infants. In all cases a Doppler echocardiogram was obtained in the first 48 h after birth, and for the ASH infants, at 2 and 4 months. Chest X ray, electrocardiogram, and laboratory tests were performed as complementary studies. ASH was present in 38.8% of LGA infants of DM and in 7.1% of NDM. The difference was significant (p < 0.01). Interventricular septum (IVS) and IVS/ posterior wall of left ventricle ratio were significantly different between groups A and B with C. There was no correlation between Hb A1 level and the presence of ASH in group A. ASH is a common finding in infants of DM. We could not find a relationship between the degree of metabolic control during pregnancy and the incidence and severity of ASH.

  14. Ablation of the atrioventricular node executed after paranodal ablation of the atrioventricular node for the treatment of paroxysmal atrial-ventricular node of reentry tachycardia in conditions of artificial blood circulation

    Directory of Open Access Journals (Sweden)

    Melikulov A.Kh.

    2014-03-01

    Full Text Available In this clinical observation is shown the data of the patient who was previously undergone paranodal ablation of atrial-ventricular junction for the treatment of atrioventricular (AV nodal reentrant tachycardia. Radiofrequency ablation of right lower isthmus for treatment of the paroxysmal form of atrial flutter was made for the patient. Sick sinus node syndrome and paroxysmal form of atrial fibrillation were diagnosed. Then dual-chamber pacemaker was implanted. Antiarrhythmic therapy about the persistent form of atrial fibrillation had no effect. The decision for the implementation of radio frequency modification of atrioventricular connection using right ventriclar access failed because of the lack of verification of the His bundle's spike. Using retrograde access through the aorta we managed to create AV blockade of III degree. Taking into account the fact that in 1990-ies patients with atrioventricular nodal reentrant tachycardia were operated using paranodal ablation of the AV node using extracorporeal circulation, this case has a practical significance when endovascular catheter modification of AV nodal conduction in this category of patients is made.

  15. Epidemiology of Complex Congenital Heart Disease in Defective Stillbirth Fetus%复杂性先天性心脏病在非活产缺陷儿中的发生状况

    Institute of Scientific and Technical Information of China (English)

    欧艳秋; 刘小清; 麦劲壮; 韩凤珍; 陈寄梅; 潘微; 庄建

    2012-01-01

    Objective:To investigate the prevalence and diagnosis of complex congenital heart disease( CCHD )in defective stillbirth fetus. Methods : We retrospectively studied 14342 fetuses and new born babies in our hospital from 2005 to 2010,that including 249 cases of defective stillbirth. All diagnosis was confirmed by fetal echocardiography. The defective rate and the component ratio of CCHD in stillbirth fetuses were analyzed. Results:The total detective rate of CHD in all birth was 10. 95‰( 157/14342 ),the detective rate in defective stillbirth was 1. 74%( 249/14342 )and CCHD in defective stillbirth was 17. 27%( 43/249 ). The CCHD occurrence as complete atrioventricular septal defect in 52‰( 13/249 ) cases,double outlet of right ventricle in 36‰ cases( 9/249 ), single ventricle in 32‰ cases( 8/ 249 ) and great artery conversion in 24‰ cases( 6/249 ). 95. 35% parents of CCHD fetuses chose to terminate the pregnancy. The detective rate for CCHD was higher in female fetuses than that in male( 30. 38% vs. 14. 55% ,P = 0. 0086 ). The average diagnosis time for CCHD fetuses was( 26. 9±4. 9 )weeks of gestation,and 67. 44% of diagnosis was made between 20 ~ 30 weeks of gestation. Conclusion:There was a high detective rate for CCHD in defective stillbirth. Fetal echocardiography was an effective and helpful method for CCHD diagnosis in fetuses.%目的:了解复杂性先天性心脏病(CCHD)在非活产缺陷儿中的检出和诊断情况.方法:通过病历回顾的方法选取我院2005-01-01 至2010-12-31胎儿及新生儿14 342例,非活产缺陷儿249例.主要诊断手段为胎儿超声心动图.分析CCHD在非活产缺陷儿中的检出率及构成比例.结果:14 342例胎儿及新生儿的先天性心脏病总检出率为10.95‰(157例),非活产缺陷儿的249例(1.74%),CCHD 43例占非活产缺陷儿的17.27%.43例中CCHD的亚型比例分布依次是完全性房室间隔缺损52‰(13例);右心室双出口36‰(9例)、单心室32‰(8

  16. Paravaginal defect

    DEFF Research Database (Denmark)

    Arenholt, Louise T S; Pedersen, Bodil Ginnerup; Glavind, Karin;

    2016-01-01

    , arcus tendineus fascia pelvis (ATFP), pubocervical fascia, and uterosacral/cardinal ligaments. Studies conclude that physical examination is inconsistent in detecting paravaginal defects. Ultrasound (US) and magnetic resonance imaging (MRI) have been used to describe patterns in the appearance...

  17. O efeito da morfina na inibição vagal do coração: demonstração da dupla via do nodo atrioventricular = The effect of morphine on vagal inhibition of the heart: demonstration of dual atrioventricular nodal pathways

    Directory of Open Access Journals (Sweden)

    José Geraldo Pereira da Cruz

    2006-07-01

    Full Text Available A estimulação elétrica do nervo vago diminui a freqüência cardíaca e aumenta o intervalo PQ no eletrocardiograma, podendo ativar a via anterógrada do nodo atrioventricular. Neste experimento é investigada a interação da morfina sobre o controle nervoso parassimpático cardíaco em relação à dupla condução do nodo atrioventricular. Aestimulação do nervo vago induz uma significativa bradicardia e um bloqueio atrioventricular de terceiro grau, com a onda P aparecendo negativa na derivação II. Devido aos efeitos moduladores da anestesia sobre o coração, administração de injeções de morfina induz o aparecimento de onda P positiva através de uma ação inibitória sobre a reentrância do nodo atrioventricular. Tudo sugere a presença de duas vias atrioventriculares, anatômica e funcionalmente distintas.That electrical stimulation of the vagus decreased heart rate and increased the PQ interval in electrocardiogram, consistent with anterograde dual atriventricular conduction. The present experiments were investigating the interaction of the morphine with cardiac parasympathetic nervous control in dual atrioventricular node. Electrical stimulation of the vagus nerve induced significantbradycardia and of third degree atrioventricular block and the P wave appearance was negative in leads II. Because background anesthesia modulates the heart, administered morphine injection the P waves appearance was positive through one inhibitory action on atrioventricular nodal reentry. All suggest the presence of two functionally and anatomically distinct atrioventricular pathways.

  18. Use-dependent properties of flecainide acetate in accessory atrioventricular pathways.

    Science.gov (United States)

    Goldberger, J; Helmy, I; Katzung, B; Scheinman, M

    1994-01-01

    Flecainide acetate has been shown to have use-dependent properties. The use-dependent properties of flecainide were evaluated in 20 patients (13 men and 7 women, mean age 32 +/- 11 years) with accessory atrioventricular connections. Twenty to 30 stimulus drive trains were introduced in either the atrium or ventricle at progressively faster rates. The range of cycle lengths over which anterograde and retrograde conduction block occurred in the accessory pathway was assessed in the drug-free state and after oral loading with flecainide acetate. The block cycle length index was defined as the shortest cycle length during which 1:1 conduction was maintained in the accessory pathway minus the longest cycle length during which block in the accessory pathway occurred on the second paced beat. In the drug-free state, the (mean +/- SD) anterograde and retrograde block cycle length indexes were 20 +/- 12 and 20 +/- 9 ms, respectively. After flecainide therapy, the anterograde and retrograde block cycle length indexes increased to 80 +/- 33 and 65 +/- 29 ms, respectively (p = 0.002 compared with the drug-free state). The block cycle length index did not correlate with serum flecainide levels, but did correlate with other electrophysiologic markers of drug effect on accessory pathway conduction. The change in the block cycle length index demonstrates that flecainide has a progressive effect on accessory pathway conduction at more rapid rates, consistent with its in vitro use-dependent properties. This index is an excellent marker of drug efficacy.

  19. An improved in vivo method for atrioventricular node ablation via thoracotomy

    Directory of Open Access Journals (Sweden)

    R.H. MacIver

    2010-02-01

    Full Text Available The atrioventricular (AV node is permanently damaged in approximately 3% of congenital heart surgery operations, requiring implantation of a permanent pacemaker. Improvements in pacemaker design and in alternative treatment modalities require an effective in vivo model of complete heart block (CHB before testing can be performed in humans. Such a model should enable accurate, reliable, and detectable induction of the surgical pathology. Through our laboratory’s efforts in developing a tissue engineering therapy for CHB, we describe here an improved in vivo model for inducing chronic AV block. The method employs a right thoracotomy in the adult rabbit, from which the right atrial appendage may be retracted to expose an access channel for the AV node. A novel injection device was designed, which both physically restricts needle depth and provides electrical information via electrocardiogram interface. This combination of features provides real-time guidance to the researcher for confirming contact with the AV node, and documents its ablation upon formalin injection. While all animals tested could be induced to acute AV block, those with ECG guidance were more likely to maintain chronic heart block >12 h. Our model enables the researcher to reproduce both CHB and the associated peripheral fibrosis that would be present in an open congenital heart surgery, and which would inevitably impact the design and utility of a tissue engineered AV node replacement.

  20. Surgical correction of hypertrophic obstructive cardiomyopathy in a patient with severe hypertrophy and septal myocardial fibrosis.

    Science.gov (United States)

    Borisov, Konstantin Valentinovitch

    2012-10-01

    In patients with hypertrophic cardiomyopathy, myocardial fibrosis is an independent predictor of an adverse outcome. A new technique of hypertrophic obstructive cardiomyopathy (HOCM) surgical correction in patients with severe hypertrophy and septal myocardial fibrosis has been proposed. This approach avoids mechanical damage to the heart conduction system, and for the surgeon it improves visual inspection of the area to be resected. We present a case report of a 33-year old female patient with biventricular obstruction, extreme hypertrophy, septal myocardial fibrosis and episodes of ventricular tachycardia who underwent surgical correction according to this novel procedure. The advantage of the approach is an effective surgical treatment of HOCM in patients with severe hypertrophy and septal myocardial fibrosis who cannot be treated with the current surgical techniques.

  1. Nasal airway and septal variation in unilateral and bilateral cleft lip and palate.

    Science.gov (United States)

    Starbuck, John M; Friel, Michael T; Ghoneima, Ahmed; Flores, Roberto L; Tholpady, Sunil; Kula, Katherine

    2014-10-01

    Cleft lip and palate (CLP) affects the dentoalveolar and nasolabial facial regions. Internal and external nasal dysmorphology may persist in individuals born with CLP despite surgical interventions. 7-18 year old individuals born with unilateral and bilateral CLP (n = 50) were retrospectively assessed using cone beam computed tomography. Anterior, middle, and posterior nasal airway volumes were measured on each facial side. Septal deviation was measured at the anterior and posterior nasal spine, and the midpoint between these two locations. Data were evaluated using principal components analysis (PCA), multivariate analysis of variance (MANOVA), and post-hoc ANOVA tests. PCA results show partial separation in high dimensional space along PC1 (48.5% variance) based on age groups and partial separation along PC2 (29.8% variance) based on CLP type and septal deviation patterns. MANOVA results indicate that age (P = 0.007) and CLP type (P ≤ 0.001) significantly affect nasal airway volume and septal deviation. ANOVA results indicate that anterior nasal volume is significantly affected by age (P ≤ 0.001), whereas septal deviation patterns are significantly affected by CLP type (P ≤ 0.001). Age and CLP type affect nasal airway volume and septal deviation patterns. Nasal airway volumes tend to be reduced on the clefted sides of the face relative to non-clefted sides of the face. Nasal airway volumes tend to strongly increase with age, whereas septal deviation values tend to increase only slightly with age. These results suggest that functional nasal breathing may be impaired in individuals born with the unilateral and bilateral CLP deformity.

  2. Percutaneous septal ablation for left mid-ventricular obstructive hypertrophic cardiomyopathy: a case report

    Directory of Open Access Journals (Sweden)

    Alioglu Emin

    2006-04-01

    Full Text Available Abstract Background Mid-ventricular obstructive hypertrophic cardiomyopathy (MVOHC is a rare type of cardiomyopathy. The diagnosis is based on the hourglass appearance on the left ventriculogram and the presence of pressure gradient between apical and basal chamber of the ventriculum on the hemodynamic assessment. Case presentation The present case represents successful percutaneous treatment with septal ablation to patient with MVOHC associated with systolic anterior motion of the mitral valve and obstruction at both the mid-ventricular and outflow levels. Conclusion Alcohol septal ablation has been proposed as less invasive alternatives to surgery in patients with MVOHC.

  3. Functional Capacity of Patients with Pacemaker Due to Isolated Congenital Atrioventricular Block

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    Roberto Márcio de Oliveira Júnior

    2015-01-01

    Full Text Available Background: Isolated congenital atrioventricular block (CAVB is a rare condition with multiple clinical outcomes. Ventricular remodeling can occur in approximately 10% of the patients after pacemaker (PM implantation. Objectives: To assess the functional capacity of children and young adults with isolated CAVB and chronic pacing of the right ventricle (RV and evaluate its correlation with predictors of ventricular remodeling. Methods: This cross-sectional study used a cohort of patients with isolated CAVB and RV pacing for over a year. The subjects underwent clinical and echocardiographic evaluation. Functional capacity was assessed using the six-minute walk test. Chi-square test, Fisher's exact test, and Pearson correlation coefficient were used, considering a significance level of 5%. Results: A total of 61 individuals were evaluated between March 2010 and December 2013, of which 67.2% were women, aged between 7 and 41 years, who were using PMs for 13.5 ± 6.3 years. The percentage of ventricular pacing was 97.9 ± 4.1%, and the duration of the paced QRS complex was 153.7 ± 19.1 ms. Majority of the subjects (95.1% were asymptomatic and did not use any medication. The mean distance walked was 546.9 ± 76.2 meters and was strongly correlated with the predicted distance (r = 0.907, p = 0.001 but not with risk factors for ventricular remodeling. (Arq Bras Cardiol. 2014; [online].ahead print, PP.0-0 Conclusions: The functional capacity of isolated CAVB patients with chronic RV pacing was satisfactory but did not correlate with risk factors for ventricular remodeling.

  4. Primary percutaneous coronary intervention ameliorates complete atrioventricular block complicating acute inferior myocardial infarction

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    Lee SN

    2014-11-01

    Full Text Available Su Nam Lee, You-Mi Hwang, Gee-Hee Kim, Ji-Hoon Kim, Ki-Dong Yoo, Chul-Min Kim, Keon-Woong MoonDepartment of Internal Medicine, St Vincent’s Hospital, The Catholic University of Korea, Suwon, South KoreaObjective: Complete atrioventricular block (CAVB in acute inferior ST-segment elevation myocardial infarction (STEMI is associated with poor clinical outcomes after noninvasive treatment. This study was designed to determine the effect of primary percutaneous coronary intervention (PCI in patients with CAVB complicating acute inferior STEMI, at a single center.Methods: We enrolled 138 consecutive patients diagnosed with STEMI involving the inferior wall; of these, 27 patients had CAVB. All patients received primary PCI. The clinical characteristics, procedural data, and clinical outcomes were compared in patients with versus without CAVB. Results: Baseline clinical characteristics were similar between patients with and without CAVB. Patients with CAVB were more likely to present with cardiogenic shock, and CAVB was caused primarily by right coronary artery occlusion. Door-to-balloon time was similar between those two groups. After primary PCI, CAVB was reversed in all patients. The peak creatinine phosphokinase level, left ventricular ejection fraction and in-hospital mortality rate were similar between the two groups. After a median follow up of 318 days, major adverse cardiac events did not differ between the groups (8.1% in patients without CAVB; 11.1% in patients with CAVB (P=0.702.Conclusion: We conclude that primary PCI can ameliorate CAVB-complicated acute inferior STEMI, with an acceptable rate of major adverse cardiac events, and suggest that primary PCI should be the preferred reperfusion therapy in patients with CAVB complicating acute inferior myocardial infarction. Keywords: major adverse cardiac events, PCI-capable hospital

  5. An analysis of cardiac defects and surgical interventions in 84 cases with full trisomy 18.

    Science.gov (United States)

    Bruns, Deborah A; Martinez, Alyssa

    2016-02-01

    Trisomy 18 (Edwards syndrome) is the second most common autosomal trisomy after trisomy 21. Medical issues commonly include cardiac defects, such as ventricular septal defect (VSD) and atrial septal defect (ASD). If untreated, these conditions can contribute to the associated infant mortality. The objective of the study was review parent-reported information on 84 cases with full trisomy 18 focusing on prenatal and postnatal assessment and confirmation of cardiac defects and on subsequent treatment with cardiac surgery and post-surgery outcomes. At birth, 65 parent responses indicated the presence of VSD (77.4%), 38 ASD (45.2%), and 50 patent ductus arteriosus (PDA) (59.5%). The presence of multiple cardiac defects was also analyzed including 25 cases with VSD, ASD, and PDA at birth. The total reduced to 18 at survey completion. Twenty-four cases had one or more cardiac defects repaired for a total of 34 corrective surgeries. Age at surgery varied from 2 weeks to 41 months of age with most performed under 1 year of age. Twenty-one cases were still living at the time of survey completion (87.5%). From these date we provide recommendations and implications.

  6. Normalisation of left ventricular systolic function after change from VVI pacing to biventricular pacing in a child with congenital complete atrioventricular block, long-QT syndrome, and congenital muscular dystrophy

    DEFF Research Database (Denmark)

    Ellesøe, Sabrina G; Reimers, Jesper I; Andersen, Henrik

    2013-01-01

    Development of dilated cardiomyopathy in patients with congenital complete atrioventricular block with or without pacemaker is well described. We report a case of dilated cardiomyopathy in a child with congenital complete atrioventricular block, long-QT syndrome, and VVI pacemaker. Temporary pacing...

  7. Sobrepunta nasal como expresión de una comunicación septal Supratip as expression of a septal communication

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    J.H. Soria

    2009-12-01

    Full Text Available La deformidad del tercio medio nasal caracterizada por convexidad como resultado desafortunado de una rinoplastia estética, es una de las causas que con mayor frecuencia induce a una revisión quirúrgica. Se conoce con la denominación suprapunta, del inglés supratip y obedece a distintos orígenes. En su etiología, intervienen elementos anatómicos del dorso del tercio medio nasal. Mencionamos en este artículo una causa inédita: una comunicación interfosas nasales. Atribuimos su etiopatogenia a la síntesis insuficiente de la incisión intersepto-columelar; la insuficiente resección de la mucosa del tabique en su borde inferior y en su ángulo anterior, unida a la mucosa excedente contralateral, forman una comunicación que se torna definitiva. Hacemos hincapié en la necesidad de un examen clínico exhaustivo de las fosas nasales para descartar esta etiología. El tratamiento quirúrgico consistió en la resección de la comunicación y la cobertura del defecto creado con colgajos de vecindad del mismo tabique. Enumeramos las medidas preventivas para evitar este tipo de contratiempo.The supratip prominence is one of the most common deformity that requires a revision rhinoplasty. Their etiology can result from a variety of causes, that including poor management of skeletal tissue in the supratip region. The authors add an unpublished cause: septal fistula (septal communication. The overstepped mucosal at the inferior edge and the anterior septal angle, with the exceeding mucosal from opposite side, producing a permanent septal communication. The diagnosis is based on the clinical exploration; we recommend an exhaustive inspection of the nasal fosses (septum. Surgical procedure consist on surgical resection of the communication using local septal flaps for plastic repair. Finally we describe the preventive management.

  8. Pathogenesis of hepatic septal fibrosis associated with Capillaria hepatica infection of rats.

    Science.gov (United States)

    Santos, A B; Tolentino, M; Andrade, Z A

    2001-01-01

    Septal fibrosis is a common form of hepatic fibrosis, but its etiology and pathogenesis are poorly understood. Rats infected with the helminth Capillaria hepatica constitute a good experimental model of such fibrosis. To investigate the pathogenetic contribution of the several parasitic factors involved, the following procedures were performed in rats: a) regarding the role of eggs, these were isolated and injected either into the peritoneal cavity or directly into the liver parenchyma; b) for worms alone, 15-day-old infection was treated with mebendazole, killing the parasites before oviposition started; c) for both eggs and worms, rats at the 30th day of infection were treated with either mebendazole or ivermectin. Eggs only originated focal fibrosis from cicatricial granulomas, but no septal fibrosis. Worms alone induced a mild degree of perifocal septal fibrosis. Systematized septal fibrosis of the liver, similar to that observed in the infected controls, occurred only in the rats treated with mebendazole or ivermectin, with dead worms and immature eggs in their livers. Thus, future search for fibrogenic factors associated with C. hepatica infection in rats should consider lesions with both eggs and worms.

  9. Lateral septal vasopressin in rats : Role in social and object recognition?

    NARCIS (Netherlands)

    Everts, HGJ; Koolhaas, JM

    1997-01-01

    The capacity of male rats to remember familiar conspecifics is called social recognition. It is a form of short-term memory modulated by lateral septal (LS) vasopressin (VP). The specificity of this phenomenon was studied by examining whether recognition of previously investigated objects is also un

  10. Direct transatrial pericardiocentesis for tamponade caused by left atrial perforation after trans-septal puncture.

    Science.gov (United States)

    Picard, Fabien; Millán, Xavier; de Hemptinne, Quentin; L L'allier, Philippe

    2016-07-07

    Trans-septal puncture is associated with risks of serious complications. We report a case of an obese 52-year-old man with hypertrophic cardiomyopathy who underwent preoperative coronary angiography and cardiac catheterisation complicated by left atrial perforation. We describe a direct transatrial pericardiocentesis approach to treating cardiac tamponade.

  11. Structure and function of the septal pore cap of Schizophyllum commune

    NARCIS (Netherlands)

    van Peer, A.F.

    2008-01-01

    Filamentous fungi form networks (mycelia) of long, tubular structures (hyphae) that extend at their tip (apex) and branch subapically (behind the tip). Hyphae are compartmentalized by cross walls (septa) that contain a central pore which is covered by a septal pore cap (SPC) in fungi belonging to th

  12. Lupus vulgaris of external nose with septal perforation--a rarity in antibiotic era.

    Science.gov (United States)

    Garg, Ajay; Wadhera, Raman; Gulati, S P; Singh, Jagjit

    2010-07-01

    Lupus vulgaris (LV) is the commonest morphological variant of cutaneous tuberculosis. Case of LV of external nose extending to internal nose causing septal perforation is documented here. Histopathology of biopsy taken confirmed the diagnosis of LV. Patient responded well to Anti-tubercular therapy (ATT).

  13. Transcoronary ablation of septal hypertrophy compared with surgery in the treatment of hypertrophic obstructive cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    姜腾勇; 吴学思; 吕强; 孟旭; 贾长琪; 张银

    2004-01-01

    @@ Transcoronary ablation of septal hypertrophy (TASH)can lead to sustained improvement in both hemodynamics and symptoms in patients with hypertrophic obstructive cardiomyopathy ( HOCM ) . 1-4 However, there have been few reports about its efficacy and safety compared with traditional surgical procedures. This study sought to compare TASH with surgery in the treatment of HOCM.

  14. Syndromes and disorders associated with omphalocele (III): single gene disorders, neural tube defects, diaphragmatic defects and others.

    Science.gov (United States)

    Chen, Chih-Ping

    2007-06-01

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

  15. Successful radiofrequency catheter ablation of atrioventricular nodal reentrant tachycardia in a patient with dextrocardia due to unilateral pulmonary agenesis: a case report

    Directory of Open Access Journals (Sweden)

    Aksu T

    2015-02-01

    Full Text Available Tolga Aksu, Tumer Erdem Guler, Ebru Golcuk, Ismail Erden, Kazim Serhan Ozcan Department of Cardiology, Kocaeli Derince Education and Research Hospital, Derince, Kocaeli, Turkey Abstract: Radiofrequency catheter ablation of the slow pathway is considered to be the treatment of choice for patients with atrioventricular nodal reentrant tachycardia. We report a 34-year-old female with mirror image dextrocardia due to unilateral pulmonary agenesis who underwent successful slow pathway ablation for typical atrioventricular nodal reentrant tachycardia. Using contrast injection, cardiac anatomy was identified in a short time and successfully ablated. Keywords: dextrocardia, AVNRT, ablation, pulmonary agenesis

  16. Right ventricular septal pacing: Safety and efficacy in a long term follow up

    Institute of Scientific and Technical Information of China (English)

    Eraldo; Occhetta; Gianluca; Quirino; Lara; Baduena; Rosaria; Nappo; Chiara; Cavallino; Emanuela; Facchini; Paolo; Pistelli; Andrea; Magnani; Miriam; Bortnik; Gabriella; Francalacci; Gabriele; Dell’Era; Laura; Plebani; Paolo; Marino

    2015-01-01

    AIM: To evaluate the safety and efficacy of the permanent high interventricular septal pacing in a long term follow up, as alternative to right ventricular apical pacing. METHODS: We retrospectively evaluated:(1) 244 patients(74 ± 8 years; 169 men, 75 women) implanted with a single(132 pts) or dual chamber(112 pts) pacemaker(PM) with ventricular screw-in lead placed at the right ventricular high septal parahisian site(SEPTAL pacing);(2) 22 patients with permanent pacemaker and low percentage of pacing(< 20%)(NO pacing);(3) 33 patients with high percentage(> 80%) right ventricular apical pacing(RVA). All patients had a narrow spontaneous QRS(101 ± 14 ms). We evaluated New York Heart Association(NYHA) class, quality of life(Qo L), 6 min walking test(6MWT) and left ventricular function(end-diastolic volume, LV-EDV; end-systolic volume, LVESV; ejection fraction, LV-EF) with 2D-echocardiography. RESULTS: Pacing parameters were stable duringfollow up(21 mo/patient). In SEPTAL pacing group we observed an improvement in NYHA class, Qo L score and 6MWT. While LV-EDV didn’t significantly increase(104 ± 40 m L vs 100 ± 37 m L; P = 0.35), LV-ESV slightly increased(55 ± 31 m L vs 49 ± 27 m L; P = 0.05) and LV-EF slightly decreased(49% ± 11% vs 53% ± 11%; P = 0.001) but never falling < 45%. In the RVA pacing control group we observed a worsening of NYHA class and an important reduction of LV-EF(from 56% ± 6% to 43% ± 9%, P < 0.0001).CONCLUSION: Right ventricular permanent high septal pacing is safe and effective in a long term follow up evaluation; it could be a good alternative to the conventional RVA pacing in order to avoid its deleterious effects.

  17. Endoscope-assisted repair of large nasal septal perforation using a complex mucoperichondrial flap and free tissue graft

    Institute of Scientific and Technical Information of China (English)

    张庆泉; 张杰; 李树峰

    2003-01-01

    Objective To improve the closure rate of large nasal septal perforations. Methods Using an endoscope, complex mucoperichondrial/mucoperiosteal flaps on one side and free tissue graft on the other, we designed a procedure to repair large nasal septal perforations.Results In our series, 8 patients were operated on with this procedure, resulting in complete closure of the perforation and subsequent relief of symptoms. Conclusion This technique may be used as an alter*$native for the repair of large nasal septal perforations.

  18. Maternal Exposure to Methotrexate and Birth Defects: a Population-Based Study

    Science.gov (United States)

    Dawson, April L.; Riehle-Colarusso, Tiffany; Reefhuis, Jennita; Arena, J. Fernando

    2015-01-01

    Methotrexate is an anti-folate medication that is associated with increased risk of multiple birth defects. Using data from the National Birth Defects Prevention Study, a case-control study of major birth defects in the United States, we examined mothers exposed to methotrexate. The study population included mothers of live-born infants without major birth defects (controls) and mothers of fetuses or infants with a major birth defect (cases), with expected dates of delivery between October 1997 and December 2009. Mothers of cases and controls were asked detailed questions concerning pregnancy history, demographic information, and exposures in a telephone interview. Approximately 0.06% (n=16/27,623) of case and 0.04% (n=4/10,113) of control mothers reported exposure to methotrexate between three months prior to conception through the end of pregnancy. Of the 16 case infants, 11 (68.8%) had a congenital heart defect (CHD). The observed CHDs included atrial septal defects, tetralogy of Fallot, valvar pulmonary stenosis, ventricular septal defects (VSDs), and total anomalous pulmonary venous return. One case infant had microtia in addition to a VSD and another had VACTER association. Exposed cases without a CHD had one of the following birth defects: cleft palate, hypospadias, congenital diaphragmatic hernia, or craniosynostosis. Based on a limited number of methotrexate-exposed mothers, our findings support recent case reports suggesting an association between early pregnancy exposure to methotrexate and CHDs. Because of the rarity of maternal periconceptional exposure to methotrexate, long-term, population-based case-control studies are needed to confirm these findings and better evaluate the association between methotrexate and birth defects. PMID:24898111

  19. Maternal exposure to methotrexate and birth defects: a population-based study.

    Science.gov (United States)

    Dawson, April L; Riehle-Colarusso, Tiffany; Reefhuis, Jennita; Arena, J Fernando

    2014-09-01

    Methotrexate is an anti-folate medication that is associated with increased risk of multiple birth defects. Using data from the National Birth Defects Prevention Study, a case-control study of major birth defects in the United States, we examined mothers exposed to methotrexate. The study population included mothers of live-born infants without major birth defects (controls) and mothers of fetuses or infants with a major birth defect (cases), with expected dates of delivery between October 1997 and December 2009. Mothers of cases and controls were asked detailed questions concerning pregnancy history, demographic information, and exposures in a telephone interview. Approximately 0.06% (n = 16/27,623) of case and 0.04% (n = 4/10,113) of control mothers reported exposure to methotrexate between 3 months prior to conception through the end of pregnancy. Of the 16 case infants, 11 (68.8%) had a congenital heart defect (CHD). The observed CHDs included atrial septal defects, tetralogy of Fallot, valvar pulmonary stenosis, ventricular septal defects (VSDs), and total anomalous pulmonary venous return. One case infant had microtia in addition to a VSD and another had VACTER association. Exposed cases without a CHD had one of the following birth defects: cleft palate, hypospadias, congenital diaphragmatic hernia, or craniosynostosis. Based on a limited number of methotrexate-exposed mothers, our findings support recent case reports suggesting an association between early pregnancy exposure to methotrexate and CHDs. Because of the rarity of maternal periconceptional exposure to methotrexate, long-term, population-based case-control studies are needed to confirm these findings and better evaluate the association between methotrexate and birth defects.

  20. Estudio descriptivo sobre bloqueos atrio-ventriculares en infartos posteroinferiores en el Hospital de Caldas ESE.

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    Wilmar Alberto Díaz

    2009-11-01

    Full Text Available Introducción: El infarto agudo de miocardio (IAM es un problema de salud pública a escala mundial y nacional, que merece una prevención, diagnóstico y tratamiento oportunos. El IAM de cara inferior presenta entre sus complicaciones el bloqueo atrio-ventricular (bloqueo AV en sus diversas manifestaciones. Materiales y métodos: El presente es un estudio de tipo descriptivo, retrospectivo realizado en el Hospital Universitario de Caldas ESE, por medio de la revisión de historias clínicas corresponismo atrio-DACCION diagnóstico, de alta calidad y de gran importancia. dientes a los años 1999 a 2002, de los pacientes que presentaron infarto agudo de miocardio de cara inferior. Se tomaron de las historias variables como edad, sexo, consumo de alcohol, tabac