Left Innominate Vein Compression, Obstructing a Supra-cardiac Total Anomalous Pulmonary Venous Connection
Year: 2025
Type: article
Abstract: Dear Editor, Total anomalous pulmonary venous connection (TAPVC) constitutes 1%–2% of congenital heart diseases with an incidence of 0.6–1.2 per 10,000 live births.[1] Obstruction to the pulmonary venous pathway is commonly seen in the infracardiac TAPVC followed by a supra-cardiac type. We hereby report a case of supra-cardiac type of TAPVC with an unusual site of obstruction, that is, the left innominate vein (LIV) compressed by the arch of the aorta. The patient’s family has provided written consent to publish this report. A four-week-old female child was brought to our hospital with respiratory distress and cyanosis. The x-ray revealed a cardiomegaly with a pulmonary plethora. On transthoracic echocardiography, she was diagnosed with a supra-cardiac type of TAPVC with large atrial septal defect. All four pulmonary veins joined to form a common chamber behind the left atrium. The vertical vein formed from the common chamber ascent superiorly, anterior to the left pulmonary artery to drain into the LIV which further drains into the right superior vena cava and then into the right atrium [Figure 1; Video 1]. Interestingly we found a flow acceleration (FA) in the innominate vein [Figures 2 and 3; Videos 2 and 3]. A pulse wave Doppler placed at the FA revealed a peak velocity of 1.66 m/s and a mean pressure of 7 mm Hg [Figure 4]. On 2D echocardiography, LIV was found to be externally compressed by an arch of the aorta leading to an FA [Figures 2 and 3; Videos 2 and 3]. Because of the obstructed nature of TAPVC, urgent surgery was planned to correct the TAPVC. Post surgery the mean gradient across the narrowest part of LIV reduced to 2 mm Hg, hence no intervention was done. The child was discharged with an uneventful intensive care unit course and kept on a routine follow-up after discharge.Figure 1: A modified high parasternal view showing an unobstructed vertical vein (yellow arrow) passing anterior to the left pulmonary artery. MPA, Main Pulmonary ArteryFigure 2: Suprasternal view showing a compressed left innominate vein (LIV) (yellow arrow) by the arch of aorta (AOA)Figure 3: A modified suprasternal view showing a compressed left innominate vein (LIV) (yellow arrow) by the arch of aorta (AOA). SVC, Superior vena cavaFigure 4: Pulse wave Doppler placed at the compressed LIV showing a mean gradient of 7 mm HgThe site of obstruction to the pulmonary venous pathway depends mainly upon the type of TAPVC. The common sites of obstruction in the infra-cardiac TAPVC are at the level of the vertical vein joining either the hepatic venous sinusoids or ductus venous or the portal vein. Whereas in the supra-cardiac TAPVC, obstruction can lie at the level of the vertical vein to LIV, or left innominate to right superior vena cava junction or a dynamic compression can present when the vertical vein passes anterior to the left pulmonary. In the index case, an unusual site of obstruction (LIV with a mean gradient of 7 mm Hg) of the pulmonary venous drainage was observed. LIV compression can be caused due to mediastinal tumors, aortic arch aneurysms, or due to external vascular compression such as the aberrant right subclavian artery. However, LIV obstruction by a normal aortic arch is very rarely seen. It was first described by Bruwer et al.[2] in 1957 in a patient with systemic hypertension. Later on, few reports were published with similar pathophysiology; however, none of them were published in a pediatric age group. To the best of our knowledge, for the first time, a LIV obstruction by a normal aortic arch was reported in the neonatal age group and also in supra-cardiac TAPVC. A possible explanation for the LIV obstruction in the index case might be due to a reduction in the space between the aortic arch and the sternum. Guo et al.[3] in their study saw a high risk for LIV stenosis in patients with an older age, high body mass index, LIV crossing over the origin of the aortic arch, or smaller space between aortic arch and sternum. LIV compression is often asymptomatic, due to adequate compensation by collateral vasculature; however, it can present as facial congestion or neck swelling. It can be managed medically, surgically, or by transcatheter intervention depending upon the etiology.[4] No intervention was done in the index case after TAPVC repair as the gradient reduced to 2 mm Hg and the patient was kept on a regular follow-up. In conclusion, we report an unusual site, that is, LIV as a site of obstruction in the supra-cardiac TAPVC. Hence, scanning of the LIV should be performed routinely as part of an echocardiographic examination of TAPVC to rule out the potential site of obstruction. Abbreviations FA = Flow acceleration LIV = left nnominate vein TAPVC = Total anomalous pulmonary venous connection Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
Source: Annals of Cardiac Anaesthesia
Authors: Krishna Prasad Gourav, Richa Soni, Indranil Biswas, Sunder Negi, Arun Kutiappan
Language: English
Subfield: Epidemiology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4408651548