Survival comparison in adults with congenital systemic to pulmonary shunt and borderline elevated pulmonary vascular resistance versus Eisenmenger syndrome
Issued Date
2024-12-01
Resource Type
eISSN
20452322
Scopus ID
2-s2.0-85211125378
Journal Title
Scientific Reports
Volume
14
Issue
1
Rights Holder(s)
SCOPUS
Bibliographic Citation
Scientific Reports Vol.14 No.1 (2024)
Suggested Citation
Sengsim J., Vijarnsorn C., Chanthong P., Chungsomprasong P., Kanjanauthai S., Thammasate P., Pacharapakornpong T., Chaiwangyen N., Bositthipichet D., Soongswang J., Tocharoenchok T., Nitiyarom E., Tantiwongkosri K., Subtaweesin T., Durongpisitkul K. Survival comparison in adults with congenital systemic to pulmonary shunt and borderline elevated pulmonary vascular resistance versus Eisenmenger syndrome. Scientific Reports Vol.14 No.1 (2024). doi:10.1038/s41598-024-81834-9 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/102395
Title
Survival comparison in adults with congenital systemic to pulmonary shunt and borderline elevated pulmonary vascular resistance versus Eisenmenger syndrome
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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.