PO95 - Hemodynamic and Histopathologic Features of Pulmonary Hypertension in Explants from Advanced Pulmonary Sarcoidosis
Jin Sun Kim (United States)1; Colin Dunn (United States)2; Amandeep Aneja (United States)3; Rohit Gupta (United States)1;
1 - Lewis Katz School of Medicine Temple University Hospital; 2 - Temple University Hospital; 3 - Cooper University Hospital;
Keywords: SAPH; Explant; Histopathology;
Select the theme: Challenges in Sarcoidosis Diagnosis and Staging
Type: Original Papers
Presentation: Poster Presentation

Introduction: Pulmonary hypertension (PH) is a significant complication of advanced pulmonary sarcoidosis. A variety of mechanisms can lead to precapillary, postcapillary and combined pulmonary hypertension. Few studies have assessed pathological findings with hemodynamic changes. Its prevalence and characteristics in advanced pulmonary sarcoidosis (APS) remain incompletely characterized.

Objectives: This study examined clinical features including hemodynamics and histopathologic features of pulmonary vasculature in explanted lungs from advanced pulmonary sarcoidosis (APS).

Materials and Methods: A single-center retrospective analysis of 24 patients who underwent lung transplantation from 2012-2020 for APS was conducted. Demographic data, right heart catheterization parameters, echocardiogram findings, and explant histopathologic features of vascular changes, vessel granulomas, and intraluminal thrombus were analyzed. PH was defined as mean pulmonary artery pressure (mPAP) >20 mmHg, with severe PH defined as pulmonary vascular resistance (PVR) >5 Wood units. 

Results: The cohort was 50% female, 87.5% Black, with median age at transplant listing of 58.1 years. PH was present in 20 patients (83.3%), with severe disease in 13 (65%). Mean hemodynamic parameters included: RAP 4.8±3.8 mmHg, mPAP 33.5±11.4 mmHg, PCWP 9.8±4.6 mmHg, cardiac index 2.8±1.3 L/min/m², and PVR 5.1±2.6 Wood units. Right heart dysfunction was evident with right atrial dilation in 63.6% and reduced TAPSE (1.7 cm) in 46.2%. Histopathologic examination revealed vascular changes consistent with PH in all explants, with intraluminal thrombotic material in 16.7% (4) cases and pulmonary vascular granulomas in 20% of PH patients.

Conclusions: Sarcoidosis-associated pulmonary hypertension is highly prevalent in advanced pulmonary sarcoidosis requiring transplantation. The majority demonstrated severe hemodynamic impairment and right ventricular dysfunction. Histology revealed vascular changes in all explanted patients with varying numbers of intraluminal thrombi and pulmonary vascular granulomas. These findings reflect the heterogeneous nature by which the SAPH can affect the pulmonary vasculature.