Survival of Patients after Left-to-Right Shunt Repaired of Congenital Heart Defect: A Comparison between Baseline Pulmonary Vascular Resistances

Abstract

Objective: This study compared the survival of patients with completely repaired pulmonary hypertension-associated congenital heart disease (PH-CHD) with a left-to-right shunt based on their pulmonary vascular resistance index (PVRi). Material and Methods: In this retrospective cohort study, the demographics, disease characteristics, laboratory tests, hemodynamic characteristics and survival of patients with PH-CHD in our institute between January 2004 and January 2016 were reviewed. Results: Of 298 patients, 216 had a low PVRi (72.5%), 57 had a moderate PVRi (19.1%) and 25 had a high PVRi (8.4%). In the overall population, the 1-, 5- and 10-year survival rates were 96.6±1.0%, 94.2±1.5%, and 91.1±3.3%, respectively. At 10 years after the operation, patients with a low PVRi had the best survival, but patients with a moderate PVRi didn’t have significantly better survival than the patients with a high PVRi (98.6±0.8% vs. 81.9±5.5% vs. 76.5±11.2%, respectively; p-value<0.001 in low vs. moderate or high PVRi; p-value=0.8 in moderate vs. high PVRi). The World Health Organization functional class and PVRi were predictors of survival at 10 years after the operation. Conclusion: This study supports the recent 2015 European Society of Cardiology and European Respiratory Society guideline, which says that patients with a PVRi <4 Wood units m2 have the best survival, a PVRi of 8 Wood units m2 was proposed as the limit for considering surgery, and a PVR of 4–8 Wood units m2 as the range in which the proper approach should be considered on a case by case basis.