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

• Published in: Pulmonary circulation Vol. 10; no. 1; pp. 1 - 5
• Main Authors: Pizzini, Alex, Sonnweber, Thomas, Frank, Renate, Theurl, Markus, Weiss, Günter, Tancevski, Ivan, Löffler-Ragg, Judith
• Format: Journal Article
• Language: English
• Published: London, England SAGE Publications 01-01-2020; John Wiley & Sons, Inc; Wiley
• Subjects: Case Report; chronic obstructive pulmonary disease; congenital heart disease; Interferon; partial anomalous pulmonary venous connection; Potassium; pulmonary arterial hypertension; Pulmonary hypertension; therapy; congenital heart disease; pulmonary arterial hypertension; therapy; partial anomalous pulmonary venous connection; chronic obstructive pulmonary disease
• ISSN: 2045-8940; 2045-8932; 2045-8940
• DOI: 10.1177/2045894019885352

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.