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Multimodal imaging in a rare malformation of bilateral supracardiac partial anomalous pulmonary venous connection

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PROVIDER: S-EPMC10281625 | biostudies-literature | 2023 Jun

REPOSITORIES: biostudies-literature

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Unusual drainage pattern of a supracardiac total anomalous pulmonary venous connection.

Project description: Not available

| S-EPMC4368941 | biostudies-literature

Quantification of 3-Dimensional Confluence-Atrial Morphology in Supracardiac Total Anomalous Pulmonary Venous Connection.

Project description:BackgroundPulmonary vein stenosis (PVS) continues to be a major complication after surgical repair of total anomalous pulmonary venous connection (TAPVC). Recent studies suggest that the morphology of pulmonary venous confluence and the left atrium (LA) is associated with PVS. However, there are limited data on the prognostic value of integrating quantitative confluence-atrial morphology into risk stratification.ObjectivesThis study sought to evaluate the prognostic impact of novel imaging metrics derived from 3-dimensional (3D) computed tomography angiography (CTA) modeling on postsurgical PVS (PPVS) in the supracardiac TAPVC (sTAPVC) setting.MethodsPatients undergoing sTAPVC repair in 2017 to 2022 from 3 centers were retrospectively reviewed. Study investigators developed 3D CTA modeled geometric features to quantify confluence-atrial morphology that were analyzed with regard to PPVS.ResultsOf the 162 patients (median age 61 days; 55% having preoperative pulmonary venous obstruction [prePVO]) included, 47 (29%) with PPVS at a median of 1.5 months ([quartile 1-quartile 3: 1.5-3.0 months]). In the univariable analysis, the indexed total volume of the LA and confluence (iTVLC) and the ratio of the corresponding confluence length to the mean distance between the LA and confluence (CCL/mDBLC ratio) were significantly associated with PPVS. In a multivariable model adjusting for prePVO and age, the iTVLC and CCL/mDBLC ratio independently predicted PPVS (HR: 1.15; 95% CI: 1.06-1.25; and HR: 1.20; 95% CI: 1.08-1.35, respectively, all P < 0.01). Specifically, an iTVLC ≥20 cm3/m2 and a CCL/mDBLC ratio ≥7.7 were significantly associated with a reduced risk of PPVS.ConclusionsQuantification of 3D confluence-atrial morphology appears to offer a deeper and better metric to predict PPVS in patients with sTAPVC.

| S-EPMC11328765 | biostudies-literature

Cardiovascular MR angiography in partial anomalous pulmonary venous connection.

Project description: Not available

| S-EPMC4369040 | biostudies-literature

A Case of Late Presentation of Supracardiac Total Anomalous Pulmonary Venous Connection in an Adult.

Project description:Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart disease and their survival into adulthood is even rarer. Here, we present the case of a 26-year-old female who was incidentally diagnosed with a case of supracardiac TAPVC during her pregnancy. All four pulmonary veins were joining to form a common venous confluence which drained into a left-sided vertical vein which drained into the innominate vein.

| S-EPMC10936703 | biostudies-literature

Branching vertical vein with multiple sites of obstruction in supracardiac total anomalous pulmonary venous connection.

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

Interventional therapy for partial anomalous pulmonary venous connection with dual drainage.

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-literature

Successful surgical repair in an older adult with supracardiac total anomalous pulmonary venous connection: A case report.

Project description:Total anomalous pulmonary venous connection (TAPVC) is a rare, cyanotic and critical congenital heart disease where the entire left and right pulmonary veins fail to drain into the left atrium directly. Also, TAPVC-induced tissue hypoxia gradually worsens after birth. Thus, timely surgical repairs are recommended once diagnosed, particularly with pulmonary venous drainage obstruction(s). Nonetheless, in sporadic cases, patients with TAPVC survive to adulthood with no surgical treatment. Herein, we report a 46-year-old female with TAPVC, where the four pulmonary veins drain into to the innominate vein (IV) via the vertical vein. The patient developed palpitations and non-anginal chest pain following routine activities for over three months. The patient had a successful surgical correction with excellent postoperative recovery.

| S-EPMC10073726 | biostudies-literature

Modified L-shaped incision technique for supracardiac total anomalous pulmonary venous connection as an alternative to sutureless technique.

Project description:BackgroundModified L-shaped incision technique (MLIT) was successfully applied to the repair of supracardiac total anomalous pulmonary venous connection (TAPVC) with promising mid-term outcomes. It is, however, unclear whether or not MLIT could be an alternative to sutureless technique (ST).MethodsAll patients (n=141) who underwent MLIT or ST repair for supracardiac TAPVC between June 2009 to June 2022 were included and a propensity score-matched analysis was performed to reduce the heterogeneity.ResultsMLIT was performed in 80.9% (114/141), whereas ST was performed in 19.1% (27/141). Patients who underwent MLIT repair had a lower incidence of pulmonary veinous obstruction (PVO)-related reintervention (1.8% vs. 18.5%, P=0.002), and late mortality (2.6% vs. 18.2%, P=0.006). Overall survival at 10 years was 92.5% (87.7%-97.7%) for MLIT and 66.8% (44.4%-100%) for ST (P=0.012). Freedom from postoperative PVO at 10 years was 89.1% (83.2%-95.5%) for MLIT and 79.9% (65.6%-97.4%) for ST (P=0.12). Cox proportional hazards regression identified prolonged mechanical ventilation duration, postoperative PVO, respiratory dysfunction and low cardiac output syndrome were associated with postoperative death and PVO-related reintervention.ConclusionsThe MLIT strategy is a safe, technologically feasible, and effective approach for supracardiac TAPVC, which is associated with more favourable and promising freedom from death and PVO-related reintervention.

| S-EPMC10720858 | biostudies-literature

Prenatal diagnosis of obstructed supracardiac total anomalous pulmonary venous connection at 23 weeks with successful immediate postnatal surgical correction.

Project description:Diagnosis of isolated total anomalous pulmonary venous connections (TAPVCs) is relatively rare in fetal life, especially in early gestation. We report a case of a fetus diagnosed with the supracardiac type of TAPVC at 23 weeks gestation, with evidence of obstruction to connection of the common vertical vein to the superior vena cava. The neonate had a critical presentation at birth and underwent an emergency surgical repair immediately after birth with excellent outcome on short term follow-up with the resolution of pulmonary artery hypertension.

| S-EPMC8457286 | biostudies-literature

Pulmonary hypertension due to presence of isolated partial anomalous pulmonary venous connection: A case report.

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

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