Objective This study sought to determine the prevalence, predictors, prognostic relevance and evolution of pulmonary hypertension (PH) (mean pulmonary artery pressure ≥25 mm Hg) in adult patients with a subaortic right ventricle (RV) in a biventricular circulation (2V-RV).
Methods We analysed retrospective data from patients with 2V-RV undergoing cardiac catheterisation in our centre between 2000 and 2018. Echocardiographic assessment of subpulmonary ventricular pressures (left ventricular systolic pressure (LVSP)), age and B-type natriuretic peptide (BNP) were assessed as PH screening tools. Kaplan-Meier curves examined time to a composite outcome of death, transplant or ventricular assist device (VAD). Data from repeat catheterisations were analysed to evaluate PH changes over time, including the effects of therapy.
Results A total of 141 patients (median age 39 (IQR 33–45) years, 68% men) underwent 191 cardiac catheterisations. At baseline, 55% had PH (isolated postcapillary 24%, combined precapillary and postcapillary 26% and precapillary 5%). BNP (area under the curve 0.80; 95% CI 0.72 to 0.88; p<0.0001), but not age at catheterisation or echocardiographic estimates of LVSP were associated with the presence of PH. The absence of PH and BNP <100 pg/mL discriminated a subgroup at very low risk during short-term (2.5 (1.3–3.9) years) follow-up (p<0.0001). Diuretics, milrinone and VAD improved haemodynamics over time.
Conclusion PH is prevalent in patients with 2V-RV even when asymptomatic. It is difficult to identify by echocardiography and most importantly, is strongly associated with adverse outcomes. PH affects prognosis and transplant options for this patient group and yet is often amenable to treatment. Awareness of these results ought to lower the threshold for invasive haemodynamic assessment and may change the management of failing patients with 2V-RV.
- systemic right ventricle
- transposition of the great arteries
- pulmonary hypertension
- heart failure
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Contributors AVDB and LR: planned and conducted the study. WW: analysis and interpretation of data. WGW, NT, EJH, EH, LB and JTG: analysis, drafted the article and revised it critically. All authors gave final approval of the version to be submitted.
Funding AVDB was supported by a grant of the Frans van de Werf Fund for Clinical Cardiovascular Research and the Research Foundation Flanders (FWO). He was also the recipient of the Nevil Thomas Fellowship in Adult Congenital Heart Disease 2017.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
Patient consent for publication Not required.