Survival comparison in adults with congenital systemic to pulmonary shunt and borderline elevated pulmonary vascular resistance versus Eisenmenger syndrome

Abstract

Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a consequence of unrepaired large systemic-to-pulmonary shunts. The long-term data of adult patients who have PAH-CHD with elevated pulmonary vascular resistance (PVR) are limited. We aimed to investigate the survival of adults who had PAH-CHD with predominantly left-to-right (L-R) shunts with (1) borderline-to-high PVR and (2) treat-and-repair compared with those with Eisenmenger syndrome (ES). From 1995 to 2021, 99 adults with ES (age 34.1 ± 11.2 years) and 118 adults with PAH-CHD with predominantly L-R shunts (age 39.1 ± 13.7 years) were eligible. The PVR in the ES group was 21.0 ± 13.1 WU. Most ES patients (97%) received pulmonary vasodilator therapy. Among the 118 patients with PAH-CHD with predominantly L-R shunts, the baseline PVR was 7.6 ± 4.6 WU, and 78 patients (66.1%) had borderline to high PVR (≥ 5 WU). In the group, 105 patients (88.9%) underwent repair; 84 had defect closure, and 21 had fenestrated closure. Treat-and-repair was used to treat 53 patients with a preoperative final PVR of 3.58 ± 2.63 WU. No early postoperative deaths were reported. At a median follow-up time of 5.4 years (range 0.1–23.6 years), the 10- and 15-year survival rates of adults with borderline PVR were 82.3% and 82.3%, respectively, which were not inferior to the rates for patients with ES, which were 77.8% and 71.2%, respectively (p = 0.41). The survival rate of patients who underwent treat-and-repair was slightly better than that of patients who underwent ES, although the difference was not statistically significant (p = 0.19). Independent mortality risk factors were functional class III-IV at initial presentation (hazard ratio 5.7, 95% CI 1.2–26.6; p = 0.02) and oxygen saturation < 94% at the most recent visit (hazard ratio 9.4, 95% CI 2.1–42.9; p = 0.004). Trial registration: TCTR20200420004.