Comparative survival of operable chronic thromboembolic pulmonary hypertension patients as compared to inoperable medically treated or untreated patients

Abstract

Background: Pulmonary endarterectomy (PEA) is the treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH) patients. It is unclear whether the outcome of patients with inoperable CTEPH is influenced by the use of pulmonary arterial hypertension (PAH)-specific drugs. Aim: To compare the survival of operable CTEPH patients who underwent PEA (OP-PEA), inoperable CTEPH patients medically treated with PAH-specific drugs (INOP-MT) and a control group of inoperable untreated CTEPH patients (INOP-C) in a single center. Methods: Between July 1996 and February 2013 we included 261 consecutive patients with CTEPH. All patients underwent right heart catheterization and 6-minute walk test (6MWT). Kaplan-Meier curves were used to estimate the survival of the three groups. Results: The mean follow-up period was 43±37 months. One hundred and nine patients were included in the OP-PEA group, 118 in the INOP-MT group and 34 in the INOP-C group. In the INOP-MT group, 54 patients received phosphodiesterase type-5 inhibitors, 36 endothelin receptor antagonists, 5 prostanoids and 23 combination therapy. Age was 63±14, 64±17 and 57±16 years in INOP-C, INOP-MT and OP-PEA respectively (P = 0.063 and < 0.001 for OP-PEA vs INOP-C and INOP-MT respectively). Baseline 6MWT was 324±125, 354±138 and 387±123 m in INOP-C, INOP-MT and OP-PEA respectively (P = 0.042 and 0.057 for OP-PEA vs INOP-C and INOP-MT respectively). Pulmonary Vascular Resistance was 9.9±5.9, 9.8±4.9 and 9.7±0.4 WU in INOP-C, INOP-MT and OP-PEA respectively (NS). Kaplan-Meier survival of the three groups is reported in the table. Table 1 *P=0.01 vs OP-PEA; **P=0.13 vs INOP-C and OP-PEA. Conclusions: OP-PEA patients were younger, with a better baseline exercise capacity and had the best long term survival as compared to the inoperable groups. INOP-MT patients had an intermediate survival between OP-PEA and INOP-C groups.