Pulmonary hypertension is a mild comorbidity in end-stage cystic fibrosis patients

Background: This study investigated the prevalence of pulmonary hypertension (PH) in cystic fibrosis (CF) patients awaiting lung transplantation (LTx) and its influence on survival. We also explored the feasibility of using echocardiography as a first assessment for diagnosing PH.

Methods: The study included 93 CF patients (46 women [50%]) evaluated for LTx between 2001 and 2010. Median age was 29 years. PH was defined as a mean pulmonary artery pressure (mPAP) measured by right heart catheterization (mPAP(cath)) of ≥ 25 mm Hg with a wedge pressure of ≤ 15 mm Hg. Echocardiographic results were divided into 3 categories based on current guidelines as "unlikely," "possible," or "likely" to have PH.

Results: In 23 patients (25%) the mPAP(cath) was between 25 and 35 mm Hg, and 1 (1%) had severe PH (mPAP(cath) of ≥ 35 mm Hg). PH did not influence survival after enlistment (p = 0.7) and after LTx (p = 0.8). For 62 patients (67%), the sPAP(echo) could be measured, and PH was unlikely in 24 (39%). In another 19 patients (20%), PH was unlikely based on the absence of tricuspid regurgitation. The negative-predictive value (NPV) of measuring PH by echocardiography was 88% in whom PH was estimated to be unlikely (n = 43); whereas in 24 patients with a measurable low sPAP(echo), the NPV was 96%.

Conclusions: PH exists in 26% of end-stage CF patients and has no effect on survival on the waiting list for LTx or after LTx. Echocardiography might be used as the first tool to rule out PH, showing a NPV of 88%.