(594) - Outcome of Heart-Lung or Double-Lung Transplantation in Pulmonary Hypertension Secondary to Congenital Heart Diseases

https://doi.org/10.1016/j.healun.2018.01.597Get rights and content

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Purpose

Heart and lung transplantation or double lung transplantation with shunt closure is a curative option for patients with pulmonary arterial hypertension (PAH) secondary to congenital heart diseases (CHD). Its benefit remains controversial given the low evolution of the disease. We aim to investigate outcome of patients with CHD and PAH listed for transplantation.

Methods

We investigated outcomes of 73 patients with PAH-CHD listed for transplantation in a single center. We compared data of patients with versus without transplantation after listing. We also assess outcome of patients being transplanted.

Results

Patients had Eisenmenger syndrome in 57 cases (78.1%) and PAH persisting post corrective surgery in 16 cases (21.9%). A WHO-functional class 4 was reported in 45 cases (61.5%). Age at listing was 33.4 years old [25.5-42.1]. Transplantation was performed in 50 cases (68.5%) at a median delay of 3.5 months [0.3-14.3]. Heart and Lung Transplantation (HLT) was performed in 44 cases and Double Lung Transplantation (DLT) in 6. DLT was performed in 4 patients with PAH persisting post corrective

Conclusion

Transplantation in patients with PAH-CHD is at high risk. Nevertheless, in selected patients with end-stage PAH-SDT, outcome of transplanted patients seems better.

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