Abstract
BackgoundIdiopathic pulmonary arterial hypertension (IPAH) is a rare condition that requires lung transplantation in patients’ refractory to medical therapy. Pulmonary artery aneurysm (PAA) is a documented complication of IPAH however, optimal management and timing of intervention for this rare entity is not well understood.Case reportWe report a case of a 51-year-old female who underwent heart-lung transplantation for IPAH and giant PAA. The extreme size of the PAA and underlying pathology encountered in this case precluded both lung transplantation and conventional aneurysm repair.ConclusionThis case demonstrates that heart-lung transplantation is a good surgical option for IPAH complicated by giant sized PAA and right heart failure.
Highlights
Pulmonary arterial hypertension (PAH) is a chronic lung disorder characterised by a progressive and pathological remodelling of the resistance pulmonary arteries
This case demonstrates that heart-lung transplantation is a good surgical option for Idiopathic pulmonary arterial hypertension (IPAH) complicated by giant sized Pulmonary artery aneurysm (PAA) and right heart failure
There are several different causes of PAH as described under group 1 of the WHO classification of pulmonary hypertension [3], with the idiopathic subtype seen in this case accounting for approximately 39% of these [4]
Summary
This case demonstrates that heart-lung transplantation is a good surgical option for IPAH complicated by giant sized PAA and right heart failure.
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