|Topic:||26. Physiology/Pathophysiology: Pulmonary Vascular and Right Ventricular Health and Disease / Adult / Clinical Studies / Pulmonary Circulation (PC)|
|Authors:||N. Maniar, C. Clarke, E. McDaniel, J.S. Smith, K. El-Kersh; Louisville, KY/US|
Pulmonary arterial hypertension (PAH) is a fatal disease with variable progression. Despite therapeutic advances, it continues to portend a poor prognosis. Per the American College of Chest Physicians (ACCP) guidelines, patients with pulmonary hypertension should be evaluated in centers with expertise in the diagnosis and management. We sought to report our experience and the outcomes in a tertiary referral center.
This was a single center retrospective study that included all patients with the diagnosis of PAH and chronic thromboembolic pulmonary hypertension (CTEPH) treated in our pulmonary vascular disease center in the University of Louisville in the period between 2011 and 2015. We examined patient demographics, initial right heart catheterization (RHC) hemodynamics, pulmonary function test, and patient outcomes. Descriptive analysis was used.
A total of 96 patients were included in the study. Twelve patients (12.5%) had a diagnosis of CTEPH and the rest (87.5%) had a diagnosis of PAH. The mean age was 54.9±13.9 years and 68.8% of the patients were females. The average mean right atrial pressure was 9.6 ± 5.5 mmHg, average mean pulmonary artery pressure was 45.33 ±14.13 mmHg, mean pulmonary vascular resistance was 8.5±5.22 Wood Units, and the mean cardiac index was 2.66 ± 0.93 L/min/m2 by thermodilution. The average FEV1/FVC ratio was 88.6%, average total lung capacity was 82.5%, and the average DLCO was 46.5% of the predicted.
The majority of our patients (83.4%) were receiving combination therapy and only 16.6% were on oral monotherapy. One third of the patients were receiving inhaled therapy and one third were receiving parenteral therapy. Tadalafil was the most commonly used oral agent (61.4%) and treprostinil was the most commonly used parenteral agent (32.2%) with an average dose of 43.7 ng/kg/min. Eight patients lost follow up and the mortality at four years was 22.7%. Three patients underwent double lung transplantation and all of them were alive at the time of this analysis. All of our CTEPH patients underwent surgical evaluation for pulmonary thromboendarterectomy (PTE) but only four out of the twelve patients underwent surgery. The rest of CTEPH patients (8 patients) were not surgical candidates due to either distal disease or associated co-morbid conditions.
In our pulmonary vascular disease center, the majority of our patients were females. Most of our patients were receiving combination therapy. Our 4-year mortality was 22.7%, 3 patients received double lung transplantation and 4 patients underwent PTE.