The Efficacy of Sildenafil in Children with Uni-Ventricle Congenital Heart Disease Post Bidirectional Cavopulmonary Shunt: An Evidence-Based Case Report

BACKGROUND: Univentricular congenital heart disease (CHD) is an anatomical heart defect where one of the ventricles does not develop. Management of univentricular defects is performed gradually; bidirectional cavopulmonary shunt (BCPS) is one of the surgical approaches conducted before the definite treatment in the Fontan procedure. Therefore, the average rate of pulmonary artery pressure and vasculature resistance is critical factors in determining good post-surgical outcomes. However, studies exploring the evidence that sildenafil administration can reduce pulmonary pressure in patients with univentricular defects are currently limited. AIM: This evidence-based case report aims to investigate whether sildenafil administration toward post-BCPS patients reduces mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) before undergoing the Fontan procedure. METHODS: Available evidence was screened through four databases in PubMed, Cochrane, Embase, and ProQuest on October 9, 2022. The keywords used were (((((single Ventricle) OR univentricular heart) AND BCPS) OR Glenn) AND sildenafil) AND hemodynamic. A result of three cohorts and one clinical trial was identified and critically appraised. RESULTS: Analytical testing of the two studies by Park I and Jeremiasen et al. shows that sildenafil significantly reduces mPAP from 19.5 ± 5.5 mmHg to 14.3 ± 3.0 mmHg (p = 0.023) and from 19 mmHg (SD = 3) to 14 mmHg (SD = 2) (p < 0.01). The studies from Hill KD and Mori et al. illustrate that sildenafil significantly reduces the PVR index by as much as 24% (p < 0.01) and from 3.2 ± 0.5 wood unit to 1.6 ± 0.6 wood unit (p < 0.0001). CONCLUSION: The administration of sildenafil decreases pulmonary artery pressure and vasculature resistance in post-BCPS pediatric patients, allowing patients to undergo the Fontan procedure.