Unusual drainage pattern of a supracardiac total anomalous pulmonary venous connection.
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ABSTRACT:
SUBMITTER: Ganiga Sanjeeva NC
PROVIDER: S-EPMC4368941 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description: Not available
| S-EPMC6058939 | biostudies-literature
Project description:A 6-year-old boy presented with dual drainage of left upper pulmonary vein, with connection to innominate vein inaddition to its normal connection to the left atrium. Despite relief of aortic stenosis at the age of 3 years, significant left to right shunt persisted. The dual drainage allowed successful percutaneous closure of the levoatriocardinal vein without obstruction to the pulmonary venous flow to the left atrium.
| S-EPMC5241853 | biostudies-other
Project description:OBJECTIVES: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease. This study aimed to evaluate the outcomes of TAPVC repair in neonates, controlling for anatomic subtypes and surgical techniques. METHODS: Between 1997 and 2013, 88 patients (median age: 16 days) underwent repair for supracardiac (31), cardiac (18), infracardiac (36), or mixed (3) TAPVC. All the patients underwent emergency operation due to obstructed drainage. Supracardiac and infracardiac TAPVC repair included a side-to-side anastomosis between the pulmonary venous confluence and left atrium. Coronary sinus unroofing was preferred for cardiac TAPVC repair. RESULTS: The early mortality rate was 2.3% (2/88 patients). The echocardiogram showed no obstruction in the pulmonary vein anastomosis, and flow rate was 1.1-1.42 m/s in the 3-year follow-up period. CONCLUSIONS: The accurate preoperative diagnosis, improved protection of heart function, use of pulmonary vein tissue to anastomose and avoid damage of the pulmonary vein, and delayed sternum closure can reduce the risk of mortality. The preoperative severity of pulmonary vein obstruction, the timing of the emergency operation, and infracardiac or mixed-type TAPVC can affect prognosis. Using our surgical technique, the TAPVC mortality among our patients was gradually reduced with remarkable results. However, careful monitoring of the patient with pulmonary vein restenosis and the timing and method of reoperation should also be given importance.
| S-EPMC4099118 | biostudies-other
Project description:BACKGROUND:Total anomalous pulmonary venous connection (TAPVC) is recognized as a rare congenital heart defect (CHD). With a high mortality rate of approximately 80%, the survival rate and outcomes of TAPVC patients are not satisfactory. However, the genetic aetiology and mechanism of TAPVC remain elusive. This study aimed to investigate the underlying genomic risks of TAPVC through next-generation sequencing (NGS). METHODS:Rare variants were identified through whole exome sequencing (WES) of 78 sporadic TAPVC cases and 100 healthy controls using Fisher's exact test and gene-based burden test. We then detected candidate gene expression patterns in cells, pulmonary vein tissues, and embryos. Finally, we validated these genes using target sequencing (TS) in another 100 TAPVC cases. FINDINGS:We identified 42 rare variants of 7 genes (CLTCL1, CST3, GXYLT1, HMGA2, SNAI1, VAV2, ZDHHC8) in TAPVC cases compared with controls. These genes were highly expressed in human umbilical vein endothelial cells (HUVECs), mouse pulmonary veins and human embryonic hearts. mRNA levels of these genes in human pulmonary vein samples were significantly different between cases and controls. Through network analysis and expression patterns in zebrafish embryos, we revealed that SNAI1, HMGA2 and VAV2 are the most important genes for TAPVC. INTERPRETATION:Our study identifies novel candidate genes potentially related to TAPVC and elucidates the possible molecular pathogenesis of this rare congenital birth defect. Furthermore, SNAI1, HMGA2 and VAV2 are novel TAPVC candidate genes that have not been reported previously in either humans or animals. FUND: National Natural Science Foundation of China.
| S-EPMC6306349 | biostudies-literature
Project description:We present the case of an infant with total anomalous pulmonary venous connection and a branching vertical vein with multiple points of narrowing, draining the confluence into the innominate vein. The embryology and clinical relevance of this interesting anatomy is discussed.
| S-EPMC3959071 | biostudies-literature
Project description:ObjectivesA meta-analysis was performed to investigate the risk factors for postoperative pulmonary venous obstruction (PVO) after surgical repair of total anomalous pulmonary venous connection (TAPVC).MethodsData bases including PubMed, Embase, Web of Science and Cochrane Library were searched systematically. The goal was to discuss the risk factors for postoperative PVO after TAPVC. Publications were screened by 2 authors independently for criteria inclusion, methodological quality assessment and data extraction. The Newcastle-Ottawa Scale and the Agency for Healthcare Research and Quality checklist were obtained to assess the quality of the studies. Data were pooled by the random effect model or the fixed effect model according to the heterogeneity test.ResultsA total of 16 studies (2,385 participants) were included in the meta-analysis. All included studies were retrospective studies. Six potential risk factors were pooled, 5 of which were significantly associated with postoperative PVO. Patients with preoperative PVO were more likely to suffer from postoperative PVO [odds ratio (OR)=5.27, 95% confidence interval (CI) = (2.75, 10.11), P < 0.01]. Compared with a sutureless procedure, the conventional operative procedure was associated with postoperative PVO [OR = 1.80, 95% CI=(1.20, 2.71), P < 0.01]. A mixed type TAPVC plays a critical role in postoperative PVO [OR = 3.78, 95% CI=(1.08, 13.18), P = 0.04]. Inverse variance analysis showed that longer cardiopulmonary bypass time [hazard ratio (HR)=1.01, 95% CI=(1.01, 1.02), P < 0.00001] and aortic cross-clamp time [HR = 1.01, 95% CI=(1.01, 1.02), P < 0.01] were significantly associated with postoperative PVO. Heterotaxy [OR = 1.18, 95% CI = 0.13, 10.45, P = 0.88] was not statistically significant as a risk factor for postoperative PVO.ConclusionsThis meta-analysis may provide a perspective on the risk factors for postoperative PVO after TAPVC, thus leading to more studies predicting postoperative PVO after TAPVC with our findings.
| S-EPMC9270848 | biostudies-literature
Project description:Total anomalous systemic venous return is a very rare anomaly, where vena cava inferior, vena cava superior, and coronary sinus drain into left atrium. Two-day-old male baby was admitted with cyanosis and tachypnea after the birth. Left atrial isomerism with anomalous systemic venous drainage was found on echocardiographic examination. We present an unusual case of total anomalous systemic venous drainage in to the left atrium.
| S-EPMC4145383 | biostudies-literature
Project description:IntroductionObstructed total anomalous pulmonary venous connection (TAPVC) is one of the commonest seen emergencies in pediatric cardiology centers.Case presentationOur case was diagnosed to have this anomaly, showing early respiratory distress resulting from severe pulmonary congestion. Palliative stenting of the obstruction was done, which helped in stabilizing the debilitated hemodynamics of the baby before surgery, thus a good surgical outcome and prognosis are expected.ConclusionThis intervention may be listed as a vital measurement in the preoperative cardiac stabilization plan for infants with obstructed TAPVC.
| S-EPMC7331390 | biostudies-literature
Project description: Not available
| S-EPMC8303077 | biostudies-literature
Project description:Anomalous pulmonary venous return is an uncommon congenital malformation which can be broadly categorized into partial or total, of which the former is more common. The anomaly is considered to be partial if some of the pulmonary veins drain into the systemic circulation and total if all the pulmonary veins drain into systemic circulation. Isolated partial anomalous pulmonary venous return (PAPVC) is an uncommon finding and is a very uncommon cause of pulmonary arterial hypertension. Whilst many patients with PAPVC remain asymptomatic, some may present at a later age with symptoms related to left-to-right shunt, pulmonary hypertension and right heart failure. We are presenting an interesting case report of an 18 years old patient who presented with exertional dyspnea and fatigue conforming to NYHA class II symptom status. Trans-esophageal echocardiography revealed isolated obstructive PAPVC as the cause for pulmonary hypertension without other demonstrable left-to-right shunts.
| S-EPMC3953709 | biostudies-literature