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Recurrence of Idiopathic Pulmonary Arterial Hypertension After Lung Transplantation FREE TO VIEW

Tathagat Narula, MD; Carol Farver, MD; Serpil Erzurum, MD; Micheala Aldred, PhD; Charles Lane, MD; Marie Budev, DO; Olufemi Akindipe, MD
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Cleveland Clinic, Cleveland, OH

Chest. 2014;145(3_MeetingAbstracts):624A. doi:10.1378/chest.1826554
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SESSION TITLE: Transplantation Case Report Poster

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM

INTRODUCTION: Lung transplantation is considered a curative therapy for idiopathic pulmonary arterial hypertension (IPAH). We report a case of recurrence of IPAH after lung transplantation.

CASE PRESENTATION: 62-year-old Caucasian man underwent bilateral sequential lung transplant for IPAH that was refractory to medical therapy. He had an uneventful post operative course and was discharged from the hospital on the 9th post operative day on routine immune-suppression. Histologic examination of the pneumonectomy specimens revealed marked pulmonary arterial hypertensive changes with intimal fibroplasia of the small, medium and large arteries with elastic remodeling of the larger arteries. No plexiform lesions were seen. Surveillance transbronchial biopsies over the next year were negative for any pathology and an echocardiogram 3 months after transplant revealed marked improvement from the pre-transplant studies. An year after lung transplantation, patient started having increasing exertional dyspnea and soon developed hypoxemic respiratory failure. Recurrence of pulmonary hypertension was confirmed with right heart catheterization. Investigations for all possible etiologies for recurrence of PH, including chronic thromboembolic pulmonary hypertension as well as possibility of pulmonary artery stenosis, were unrevealing. Patient was re-initiated on triple drug therapy for IPAH and emergently relisted for lung re-transplantation. Unfortunately, thirteen months after his lung transplant procedure, the patient died from refractory hypoxemia and right heart failure. At autopsy, histology of the lungs revealed pulmonary hypertensive vasculopathy with intimal fibrosis and medial hypertrophy of small, medium and large arteries. The histologic changes in the allograft were deemed similar to the changes in the explanted lungs removed at transplantation. In an effort to elucidate the potential etiology for IPAH recurrence, we performed mutational and chromosomal analysis on the original explant that was unrevealing.

DISCUSSION: Despite significant advances in the understanding and medical management of pulmonary vascular disease, particularly idiopathic pulmonary arterial hypertension, lung transplantation still remains an accepted therapy for severe pulmonary vascular disease not responding to medical therapy. Although recurrence of the pulmonary disease after lung transplantation has been reported, recurrence of pulmonary hypertension after lung transplantation is exceedingly rare. There are descriptions of pulmonary hypertension from anastomotic narrowing of the pulmonary artery after lung transplantation, requiring surgical revision. Recurrence has also been reported in a handful of cases with pulmonary capillary hemangiomatosis and pulmonary veno-occlusive disease. The absence of plexiform lesions in both the explanted and autopsy specimens in our patient is fascinating and raises questions about the possibility of a totally distinct phenotype of IPAH. To the best of our knowledge, there is only one previous case report describing the recurrence of idiopathic pulmonary arterial hypertension after bilateral lung transplantation. The fact that we have not able to elucidate an etiology for recurrence is a reflection of limitations and gaps in our understanding of IPAH.

CONCLUSIONS: One of the first reports on this front, this case highlights the limitations in our understanding of IPAH. Contrary to common belief, this case suggests that lung transplantation is not an infllible remedy for this confounding illness.

Reference #1: Soriano CM, Gaine SP, Conte JV, et al. Anastomotic pulmonary hypertension after lung transplantation for primary pulmonary hypertension: report of surgical correction. Chest. 1999;116(2):564-6.

Reference #2: Izbicki G, Shitrit D, Schechtman I, et al. Recurrence of pulmonary veno-occlusive disease after heart-lung transplantation. J Heart Lung Transplant. 2005;24(5):635-7.

Reference #3: Snow JL, Palevsky HI, Archer-Chicko C, et al. Idiopathic Pulmonary Arterial Hypertension Recurring Within One Year After Bilateral Lung Transplantation. Abstract presented at the 8th International Pulmonary Hypertension Association (PHA) conference and scientific sessions held in Houston, Texas June 20-22,2008.

DISCLOSURE: The following authors have nothing to disclose: Tathagat Narula, Carol Farver, Serpil Erzurum, Micheala Aldred, Charles Lane, Marie Budev, Olufemi Akindipe

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