Pulmonary hypertension and congenital heart disease: An insight from the REHAP National Registry.

BACKGROUND

Pulmonary arterial hypertension (PAH) is common in patients with congenital heart disease (CHD). Although Eisenmenger syndrome may be on decline, other types of PAH-CHD are increasing and little is known on long-term outcome of this population. We report the PAH-CHD population of Spain via a national registry with focus on long-term survival.

METHODS AND RESULTS

A total of 240 consecutive patients (age 37.7 ± 14.1 years, 67.9% females) with PAH-CHD included in the REHAP registry were analysed. Patients were classified into 3 groups: 1) Eisenmenger syndrome, 2) postoperative-PAH and 3) PAH associated with small defects. Over a median follow-up time of 4.5[1.6-7.1]years, 50 patients (20.8%) died or underwent lung/heart-lung transplantation. Patients with Eisenmenger syndrome had better survival than postoperative-PAH (HR 0.1 95% CI: 0.2-0.9, p=0.048) but no advantage compared to small defects (HR 4.4, 95% CI 0.6-31.4, p=0.15). In the overall PAH-CHD population, patients in NYHA functional class III-IV had a 3-fold increased risk of death (HR 3.0, 95% CI: 1.5-5.9, p=0.001). Amongst patients with Eisenmenger syndrome, a pre-tricuspid shunt had a 2.6-fold increase risk of death (HR 2.6, 95% CI: 1.2-5.6, p=0.03). There was no significant difference in survival between patients with postoperative-PAH and patients with iPAH (HR 0.99 95% CI: 0.6-1.7, p=0.97).

CONCLUSION

PAH-CHD is associated with mid to long-term mortality. Outcome relates closely to functional class, type of PAH-CHD and within the Eisenmenger cohort, with location of the shunt. Adults with postoperative-PAH have the worse prognosis in the PAH-CHD cohort, reinforcing the need for lifelong close follow-up of such patients.