Persistent Oxygen Requirement beyond Prematurity: A Case of Acquired Pulmonary Vein Stenosis

Abstract

Acquired pulmonary vein stenosis is a rare cardiac defect and diagnosis can often be challenging, as many cases present with refractory or prolonged oxygen requirement over the expected course. Comorbid conditions can cloud this diagnosis further. Prognosis is poor for most patients. We present a case of idiopathic acquired pulmonary vein stenosis and discuss diagnostics, treatment options, and the need for further collaborative studies.

Figure 1

Transthoracic echocardiogram images demonstrating progressive pulmonary vein stenosis. (a) Color flow Doppler of pulmonary veins at initial diagnosis, with (b) measured mean pulmonary gradient (mean PG) of 5 mmHg and (c) increased mean pulmonary pressure gradient to 9 mmHg by 6 weeks after diagnosis.