Pulmonary Vein Stenosis in a Newborn: A Commonly Overlooked Diagnosis.

Nathalie Jeanne Magioli Bravo-Valenzuela,Guilherme Ricardo Nunes Silva,Marcela Pinto Varella

doi:10.1155/2015/870257

Nathalie Jeanne Magioli Bravo-Valenzuela, Guilherme Ricardo Nunes Silva + Show 1 more

Open Access

https://doi.org/10.1155/2015/870257

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Journal: Case reports in cardiology	Publication Date: Jan 1, 2015
Citations: 6	License type: CC BY 3.0

Affiliation: Universidade de Taubaté

Abstract

The diagnosis of primary pulmonary vein stenosis is often overlooked because its symptoms overlap lung diseases and pulmonary arterial hypertension. Its diagnosis may be difficult because the condition is progressive and associated with other defects. We present a case of pulmonary vein stenosis in a newborn with stenosis of the left-sided common pulmonary vein, diffuse hypoplasia of the superior right pulmonary vein, and atresia of the inferior right pulmonary vein.

Highlights

Pulmonary vein stenosis is a rare condition with high morbidity and mortality and a frequency of 1.7 among 100,000 children less than 2 years of age [1]
We report on a rare case of a newborn with stenosis of the left-sided common pulmonary vein, diffuse hypoplasia of the superior right pulmonary vein, and atresia of the inferior right pulmonary vein
Developmental abnormalities of the pulmonary venous plexus and common pulmonary vein absorption into the left atrium (LA) as well as the atresia or stenosis of the common pulmonary vein result in a spectrum of pulmonary venous anomalies

Summary

Introduction

Pulmonary vein stenosis is a rare condition with high morbidity and mortality and a frequency of 1.7 among 100,000 children less than 2 years of age [1]. 50% of patients with primary pulmonary vein stenosis have other congenital heart defects (CHD), most commonly atrial and ventricular septal defects [2]. Secondary pulmonary vein stenosis can develop because of external compression, cardiac catheterization, or surgical repair [1, 3]. Primary pulmonary vein stenosis is generally progressive and has been associated with neoproliferation of myofibroblast cells [3]. Pulmonary arterial hypertension predominates as stenosis progresses. We report on a rare case of a newborn with stenosis of the left-sided common pulmonary vein, diffuse hypoplasia of the superior right pulmonary vein, and atresia of the inferior right pulmonary vein

Clinical Presentation

Findings

Discussion