Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups

Eur Heart J. 2014 Mar;35(11):716-24. doi: 10.1093/eurheartj/eht072. Epub 2013 Mar 1.


Aims: This study compared the clinical, functional, and haemodynamic characteristics and current era survival of subgroups of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD): Eisenmenger syndrome (ES); PAH-CHD associated with systemic-to-pulmonary shunts (SPs); PAH with small defects (SDs); and PAH after defect correction (CDs).

Methods and results: Data from consecutive PAH-CHD patients referred to our centre from 1 January 1998 to 31 May 2011 were collected. A contemporary group of idiopathic PAH patients was utilized for comparison. Treatment was per PAH guidelines, including combination therapy, with approved PAH-specific drugs. Survival was assessed with Kaplan-Meier analysis from the first invasive haemodynamic confirmation of PAH and compared across subgroups by log-rank test. Of 192 patients (mean age 41 ± 17 years; 61% female), 90 had ES (aged 41 ± 16 years); 48 SP (aged 47 ± 18 years); 10 SD (aged 25 ± 21 years); and 44 CD (aged 36 ± 17 years). Patients with ES had the highest baseline pulmonary vascular resistance and the lowest exercise capacity. Seventy-eight per cent were treated with approved PAH-specific drugs, and 44% were treated with combination therapy. Kaplan-Meier survival estimates (95% confidence interval) at 20 years for ES, SP, and CD were 87% (77-93%), 86% (60-96%), and 36% (12-72%, P = 0.0001 vs. ES; P = 0.004 vs. SP), respectively, and at 15 years for SD was 66% (16-91%, P = 0.015 vs. ES; P = 0.016 vs. SP). The survival of the 278 patients with idiopathic PAH appeared to be worse when compared with the PAH-CHD subgroups.

Conclusion: Relevant clinical, functional, haemodynamic, and survival differences were observed among subgroups. In particular, patients with CD and SD had the worst survival. These findings should be considered when planning medical or interventional treatment strategies in PAH-CHD patients.

Keywords: Catheterization; Congenital heart defects; Follow-up studies; Haemodynamics; Pulmonary arterial hypertension.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Child
  • Exercise Tolerance / physiology
  • Familial Primary Pulmonary Hypertension
  • Female
  • Heart Defects, Congenital / complications
  • Heart Defects, Congenital / mortality*
  • Heart Defects, Congenital / therapy
  • Humans
  • Hypertension, Pulmonary / complications
  • Hypertension, Pulmonary / mortality*
  • Hypertension, Pulmonary / therapy
  • Kaplan-Meier Estimate
  • Male
  • Middle Aged
  • Referral and Consultation
  • Retrospective Studies
  • Survival Rate
  • Time-to-Treatment
  • Young Adult