OBJECTIVES In upper-income countries, infants undergo low-risk ventricular septal defect (VSD) repair. Children in low- and middle-income countries frequently present at older ages with elevated pulmonary vascular resistance (PVR) and pulmonary arterial hypertension (PAH). Expensive interventions for pulmonary hypertensive crisis are not available, and children are often denied an operation due to the mortality risk. We report our early and late experiences with these patients who underwent VSD closure by traditional patch (TP) or double patch (DP) techniques. METHODS We extracted data from patients with VSD and PAH who underwent VSD closure operations from 1996 to 2016. Information regarding cardiac catheterizations, operations, time in the intensive care unit and follow-up information was found. We identified 129 children and analysed the differences between the TP (89/129) and the DP (40/129) in unidirectional groups. After 2005, the patients were pretreated with sildenafil 3 months before catheterization. RESULTS The TP group was younger (P  < 0.001). Hospital mortality (2.5%, DP; 10.1%,TP) was not significantly different (P  = 0.17) between the 2 groups despite significant differences in baseline PVR, PVR/systemic vascular resistance and the number of children with a saturation less than 90%. The long-term survival and regression of PAH were better in the DP group (survival over 97% vs 93% in the TP group). Moreover, 61% of children with DP and 75% of those with TP had normal to mild elevations in pulmonary artery pressures at the last follow-up examination (P  = 0.046). CONCLUSIONS The DP operation is a low-risk procedure. Moreover, the long-term outcome with the use of this technique in high-risk patients with VSD and high PVR and PAH is favourable with respect to survival and showed a decrease in PAH at the last follow-up.

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Introduction: Children with large ventricular septal defects, severe pulmonary hypertension (PHT), and elevated pulmonary vascular resistance (PVR) are frequently denied operation in low and middle-income countries secondary to concerns of high mortality and development of severe pulmonary hypertension following operation, early and late. Hypothesis: We hypothesized that closure with a uni-directional flap valve patch (DP) would provide lower mortality and better survival without severe PHT. Methods: From 5/96 - 12/2016 all patients undergoing VSD closure with severe PHT and elevated PVR by either traditional (TP) or DP closure were followed annually. Baseline and O 2 provocation catheterization were performed before operation. Echocardiograms were performed at discharge and follow-up. The decision on TP or DP closure was based upon age and surgeon preference. Beginning in 2005 all patients regardless of technique received sildenafil 3 mgs/kg/day for 3 months before operation. Results: The number of patients receiving operation was 129, of which 40 received the DP. Males were 66/129, age and weight differed significantly for DP group compared to the TP group (7.5 vs 2.6 years, p<0.001 and 20 vs 11.5 kgs, p<0.001).Catheterization baseline data, PVR 9.0 vs 7.4 for DP and TP respectively (p<0.001), negative vasoactive test 32.5% vs 13.5% for DP and TP respectively (p=0.016). Mortality was 1/40 for DP and 9/89 for TP. Pulmonary to Systemic systolic pressure ratio was lower at discharge vs baseline cath (0.38 vs 0.99, p<0.001). Predictors of late severe pulmonary hypertension were PVR/SVR >0.5, p<0.024; and a negative vasoactive test of <10% decrease, p<0.001. PHT at last follow-up, none or mild 86/115 (74.8%), moderate 16/115 (13.9%) or severe 13/115 (11.3%). Conclusions: DP patients had less risk of death and both groups had few patients with severe PHT at late follow-up..