Heart-lung transplantation for idiopathic pulmonary arterial hypertension and giant pulmonary artery aneurysm – case report

Abstract

Backgound: Idiopathic 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 report: We 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. Conclusion: This case demonstrates that heart-lung transplantation is a good surgical option for IPAH complicated by giant sized PAA and right heart failure.

Is it time for a paradigm shift: Should double‐lung transplant be considered the treatment of choice for idiopathic pulmonary arterial hypertension and giant pulmonary aneurysm?

“…2,5 In the case of IPAH and significant (>6 cm) PAA, especially when right ventricle dysfunction is also present, HLT can be considered. 4 Our case presented indeed a giant aneurysm of the pulmonary artery (8.8 cm) and was further complicated, as rarely reported in such conditions, 6 by a massive pulmonary valve regurgitation and right ventricle dysfunction. HLT has been usually considered, in such a scenario, one option 2,4,7 and our patient was actually first listed for it.…”

“…In the case of IPAH, aggressive medical therapy is often unsatisfactory and double‐lung transplantation (DLT) remains the only treatment option 3 . When IPAH is associated with significant PAA, heart–lung transplantation (HLT) is usually considered one of the available techniques 2,4 . Here, we report a unique case of severe IPAH, giant pulmonary aneurism, massive pulmonary valve regurgitation (PVR), and right ventricle dysfunction that we originally listed for HLT, but, due to the lack of heart–lung block availability, we successfully treated using an original combined procedure based on DLT.…”

Pulmonary artery dissection in long standing idiopathic pulmonary arterial hypertension: A case report

“…Pulmonary arterial hypertension (PAH) is a chronic lung disease characterized by progressive and pathologic remodeling of the resistance in the pulmonary arteries [ 1 , [3] , [4] , [5] , [6] ]. This group of disorders is described under the WHO group 1 classification of PH [7] .…”

“…In this case complicated by the presence of a marked PAA and PAD, additional pulmonary valve replacement and placement of the proximal conduit was performed. Alternatively combined lung and heart transplantation has also been performed to treat long standing IPAH complicated by a marked PAA [6] …”

ERS International Congress 2020: highlights from the Thoracic Surgery and Transplantation Assembly

“…Giant Pulmonary Artery Aneurysm (PAA) is a late complication of idiopathic pulmonary arterial hypertension (IPAH). Bilateral LTx with aneurysmal repair is possible in such cases with replacing recipient PAA with donor pulmonary artery (PA) or aorta [48][49][50]. Heart-Lung Transplantation (HLTx) remains an option for IPAH complicated by giant sized PAA and right heart failure.…”