Regression of pulmonary artery hypertension due to development of a pulmonary arteriovenous malformation

Abstract

Idiopathic Pulmonary Hypertension (IPAH) is characterized by elevated pulmonary arterial pressure in the absence of an identifiable underlying cause. The condition is usually relentlessly progressive with a short survival in the absence of treatment.(1) We describe a patient of IPAH in whom the pulmonary artery pressures significantly abated with complete disappearance of symptoms, following spontaneous development of a pulmonary arterio-venous malformation (PAVM).

Intrapulmonary arteriovenous anastomoses in dogs with severe Angiostrongylus vasorum infection: clinical, radiographic, and echocardiographic evaluation

“…It has been shown that individuals able to open IPAVAs during exercise have lower PAP [34]. Isolated case reports have documented a hemodynamic and clinical benefit of macroscopic intrapulmonary arteriovenous malformations in diseased people with severe PH [35,36], similarly the ability to recruit IPAVAs may be an important defensive mechanism against PH in disease [37]. Whereas the PAP lowering effect may have advantages in health and disease, reduced gas exchange may worsen oxygenation which may contribute to exercise-induced arterial hypoxemia [38] and may be critical in severely hypoxic patients [39,40].…”

Pulmonary Arterial Hypertension and Pregnancy

“…An interesting case report was published by Hasan et al wherein pulmonary artery pressures significantly abated, following spontaneous development of pulmonary arteriovenous malformation in an IPAH patient. 48…”