Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension

Alex Pizzini; Thomas Sonnweber; Renate Frank; Markus Theurl; Günter Weiss; Ivan Tancevski; Judith Löffler-Ragg

doi:10.1177/2045894019885352

Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension

Pulm Circ. 2020 Mar 26;10(1):2045894019885352. doi: 10.1177/2045894019885352. eCollection 2020 Jan-Mar.

Authors

Alex Pizzini¹, Thomas Sonnweber¹, Renate Frank², Markus Theurl³, Günter Weiss¹, Ivan Tancevski¹, Judith Löffler-Ragg¹

Affiliations

¹ Department of Internal Medicine II, Infectious Diseases, Pneumology, Rheumatology, Medical University of Innsbruck, Innsbruck, Austria.
² Department of Radiology, Medical University of Innsbruck, Innsbruck, Austria.
³ Department of Internal Medicine III (Cardiology, Angiology), Medical University of Innsbruck, Innsbruck, Austria.

Abstract

Isolated partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital heart anomaly that is sporadically associated with pulmonary arterial hypertension in the adult population. The diagnosis and therapy for this condition are challenging. We report on three cases of patients with unexpected severe precapillary pulmonary hypertension in single PAPVC treated with an upfront pulmonary arterial hypertension-specific combination therapy. Our cases indicate that the combination of PAPVC and pulmonary comorbidities may trigger the development of severe pulmonary arterial hypertension. The initiation of pulmonary arterial hypertension-targeted combination therapy revealed to be a safe and efficacious strategy for patients with PAPVC-associated severe pulmonary arterial hypertension.

Keywords: chronic obstructive pulmonary disease; congenital heart disease; partial anomalous pulmonary venous connection; pulmonary arterial hypertension; therapy.