Pulmonary Hypertension

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JACC March 27, 2012

Volume 59, Issue 13

HERITABLE PULMONARY ARTERIAL HYPERTENSION IS ASSOCIATED WITH WORSE PULMONARY

CAPACITANCE AND RIGHT VENTRICULAR STROKE WORK INDEX COMPARED TO IDIOPATHIC

PULMONARY ARTERIAL HYPERTENSION

ACC Moderated Poster ContributionsMcCormick Place South, Hall A

Sunday, March 25, 2012, 9:30 a.m.-10:30 a.m.

Session Title: Pulmonary Hypertension Prognosis/OutcomesAbstract Category: 30. Pulmonary Hypertension

Presentation Number: 1131-544

Authors: Evan L. Brittain, Meredith E. Pugh, Lisa A. Wheeler, Quinn S. Wells, Ivan M. Robbins, James E. Loyd, Eric D. Austin, Anna R. Hemnes,

Vanderbilt University Medical Center, Nashville, TN, USA, Vanderbilt Children’s Hospital, Nashville, TN, USA

Background: Recent data has shown that cardiac index (CI) and pulmonary vascular resistance (PVR) are worse in heritable pulmonary arterial

hypertension (HPAH) compared to Idiopathic PAH (IPAH) and that survival may be worse in HPAH compared to IPAH. We hypothesized that right

ventricular (RV)-specific indices of pulmonary capacitance and RV function would be worse in patients with HPAH compared to those with IPAH and

may offer insight into potentially different outcomes.

Methods: From a prospective registry of PAH patients seen at our institution, we measured conventional hemodynamic parameters and calculated

pulmonary arterial capacitance (PC) and RV stroke work index (RVSWI) at the time of diagnostic catheterization.

Results: Forty patients with HPAH (73% female, mean age 32.2 ± 10.5 years) and 81 patients with IPAH (78% female, mean age 46.1 ± 14.3

years) were analyzed. HPAH patients had lower CI (1.7 ± 0.5 vs. 2.3 ± 0.6 l/min/m2, p < 0.0001) and higher PVR (15.9 ± 10.3 vs. 11.1 ± 5.0 WU, p

= 0.001) as well as lower RVSWI (13.7 ± 4.4 vs. 18.6 ± 6.7 g-m/ml2) and lower PAC (0.9 ± 0.3 vs. 1.2 ± 0.5 ml/mmHg) compared to IPAH patients

(Figure). RVSWI (HR 0.9, CI 0.8-0.9) and PC (HR 0.15, CI 0.03-0.75) were both univariate predictors of death or transplant at 5 years for both groups

combined.

Conclusions: PC and RVSWI are lower in patients with HPAH at the time of diagnosis indicating less compliant pulmonary vasculature and failure

of RV compensation. These hemodynamic parameters may be useful predictors of survival in PAH.