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

Abstract

a b s t r a c tAnomalous 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.

Two‐ and four‐dimensional echocardiography with high‐definition flow imaging and spatiotemporal image correlation in the diagnosis of fetal isolated partial anomalous pulmonary venous connection

“…PAPVC is an uncommon congenital heart disease in which one or more (but not all) of the PVs connect to the systemic veins instead of connecting into left atrium. 6 It can be isolated or associated with Scanning around the left atrium carefully makes it possible to identify the drainage of the PVs into the left atrium by conventional 2DE, CDFI, or better by HDFI. Usually, the connection of the RPVs to the left atrium is close to the interatrial septum, while the LPVs are usually superior to the appendage and on the leftward/posterior aspect of the left atrium.…”

Undiagnosed case of right ventricular dilatation in a teenager

“…Because of the redirection of blood flow to the RA, right heart dilatation from volume overload may occur; however, that is dependent on the size and magnitude of the left to right shunt. Clinical findings may not be helpful in securing a diagnosis because they may be absent or subtle, yet, over time, the persistent increase in pulmonary blood flow can lead to remodeling of the pulmonary vasculature, an increase in the pulmonary vascular resistance and pulmonary arterial hypertension leading to RV failure …”

“…Clinical findings may not be helpful in securing a diagnosis because they may be absent or subtle, yet, over time, the persistent increase in pulmonary blood flow can lead to remodeling of the pulmonary vasculature, an increase in the pulmonary vascular resistance and pulmonary arterial hypertension leading to RV failure. 4,6 It is imperative that the diagnosis and surgical intervention of PAPVD is performed early in the clinical course in order to prevent possible irreversible right ventricular failure and pulmonary vascular disease. Transthoracic echocardiography is the first line investigation in the diagnosis of PAPVD.…”

A Wide Comparison of Techniques for Repair of PAPVCs: One Institution's 20-Year Experience

“…6 It is defined as a drainage of one or more pulmonary veins to the right atrium or to one of its tributaries. 7,8 The right upper pulmonary veins are the most frequently affected. 9,10 The location of drainage determines the type of PAPVC; whether it is supracardiac, cardiac, or infracardiac.…”

“…20 Pulmonary hypertension is a possible consequence of a left-to-right-shunt. 5,8,21 Furthermore, a leftto-right-shunt can lead to ventricular failure and death. 8,19,21 The purpose of this analysis is to compare the outcome of the four surgical therapies for PAPVC, single-patch, doublepatch, Warden technique and direct-anastomosis, regarding their complications, such as pulmonary hypertension, right ventricular dysfunction, heart rhythm disorders and venous stenosis.…”