Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension. (1st March 2020)

• Record Type: Journal Article
• Authors: Pizzini, Alex; Sonnweber, Thomas; Frank, Renate; Theurl, Markus; Weiss, Günter; Tancevski, Ivan; Löffler‐Ragg, Judith
• Is Part Of: Pulmonary circulation. Volume 10:Number 1(2020)
• Page Start: 1
• Page End: 5
• Publication Date: 2020-03-01
• Subjects: partial anomalous pulmonary venous connection -- pulmonary arterial hypertension -- chronic obstructive pulmonary disease -- congenital heart disease -- therapy; Pulmonary circulation -- Periodicals; Pulmonary circulation; Electronic journals -- Sciences; Periodicals; 616.24005
• DOI: 10.1177/2045894019885352
• Languages: English
• ISSNs: 2045-8932

Abstract

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.