Myoung Hyun Choi, Frances Greathouse, Amir Darki
Loyola University Medical Center.
United States
Cureus
Cureus 2024; 16:
DOI: 10.7759/cureus.62779
Abstract
Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital heart disease in which one or more pulmonary veins drain into the systemic venous circulation. The abnormal connection between the pulmonary vein and the right atrium can result in a right-sided volume overload due to a left-to-right shunt, followed by eventual right-sided pressure overload and right ventricular failure. PAPVC is usually associated with an atrial septal defect but can present as an isolated finding. We present a case of isolated PAPVC resulting in right heart failure and predominantly pre-capillary pulmonary hypertension. We discuss the challenges in the diagnosis and medical management of isolated PAPVC and highlight the clinical and hemodynamic indications for pulmonary vasodilators and diuretics.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes