Levoatrial cardinal vein with normal left ventricle: A forgotten cause of pulmonary arterial hypertension

Abstract

Levoatrial cardinal vein (LACV) is anomalous connection between left atrium or pulmonary veins and systemic veins such as innominate vein or superior vena cava. This persistence of splanchnic circulation occurs when there is left-sided obstructive cardiac lesions such as hypoplastic left heart or mitral atresia. In this report we present three cases of LACV with well-developed left heart, without any obstructive lesions. All our cases presented with pulmonary arterial hypertension (PAH) and had associated intracardiac shunt such as ventricular/atrial septal defect and supracardiac partial anomalous pulmonary venous connection. Apart from the above shunts, LACV contributed to PAH in these cases. It is important to detect and report LACV as this may require surgical correction along with other defects. If LACV goes undetected during imaging workup, it may cause persistent PAH postoperatively.

Levoatriocardinal Vein With Multiple Ventricular Septal Defects and Without Left-Sided Valvular Atresia

“…Although instances of the levoatriocardinal vein in association with well-developed left heart without any obstructive lesions have been reported, 2 concomitant presence of VSD and mitral regurgitation are unique aspects of our case.…”

Levoatrial Cardinal Vein: In two siblings with normal heart morphology

“…In the literature, treatment modalities are selected depending on the amount and direction of the shunt flow. Transcatheter occlusion (8) and surgical correction treatments are applied (3), (5). In our first case, since the flow direction was from the innominate vein to the left atrium, surgical closure was preferred using the double ligation technique in terms of paradoxical embolism and infection risk.…”

Trans-catheter closure of a rare cause of pre-tricuspid left-to-right shunt: A “double” levoatriocardinal vein without left heart obstructive lesions

“…The closure allowed a normalization of the right ventricular volume with consequent improvement of signs and symptoms. A non-diagnosed levoatriocardinal vein may be responsible for a significant pulmonary blood-flow overload with consequent development of pulmonary arterial hypertension [6]. An early detection is necessary to avoid the progression of the disease over time.…”