Life-saving urgent intervention in a low-birth-weight newborn with obstructed supracardiac total anomalous pulmonary venous connection: stenting the vertical vein.
Life-saving urgent intervention in a low-birth-weight newborn with obstructed supracardiac total anomalous pulmonary venous connection: stenting the vertical vein.
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.
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.
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.
Project description:What do we call a vascular structure that is in the left pneumopericardial space, drains systemic venous blood, and the total pulmonary venous return, into the right atrium, through a dilated coronary sinus (CS)? Can we preserve the CS drainage while correcting the 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.
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.
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.