Background: Shone's complex is a rare lesion affecting the mitral valve (MV) and left ventricular outflow tract (LVOT). The objective of this study is to report the outcomes after Shone's complex repair, the growth of mitral and aortic valve and LVOT, and long-term survival. Methods: This retrospective study included all patients diagnosed with Shone's complex, who underwent biventricular repair. Data including patients' characteristics, type of the MV lesion and the associated lesions were collected. Patients were followed up regularly with echocardiography, and the changes in mitral and aortic valve z-score and LVOT z-score were recorded. Results: Thirty-seven patients were included in the study, the median age was 3.4 months, and 11 patients (30.6%) had pulmonary hypertension. The main procedure performed during the first surgical intervention was coarctation repair in 26 patients (70%). Twelve patients had MV repair, and five had MV replacement. Operative mortality occurred in 1 patient (2.7%), median follow up was 52 (25-75th percentile: 22-84) months. Survival at 1, 5, and 10 years was 94.4%, 90%, and 76.9%, respectively. Reoperation was required in 13 patients, mainly for LVOT repair (n = 8). Reoperation was significantly associated with associated aortic valve lesion (p = .044). The growth of the MV z-score was 0.35 per year; p < .001, aortic valve z-score 0.086 per year; p = 0.422, and the LVOT z-score was 0.53 per year; p = .01. Conclusion: Biventricular repair of Shone's complex has good outcomes. Reoperation is frequently encountered, especially with low aortic valve z-score. The MV and LVOT have significant growth following Shone's complex repair.
BackgroundIsolated right Superior Vena Cava drainage into the left atrium in the absence of other cardiac anomalies is an extremely rare condition. The vein of Galen aneurysmal malformation is a congenital vascular malformation. It comprises 1% of all pediatric congenital anomalies. The association vein of Galen aneurysmal malformation, with congenital heart disease has been described.Case presentationWe describe a 16-months old toddler presenting at 7-months of age with respiratory distress and cyanosis. CT brain showed Vein of Galen aneurysmal malformations. Echocardiography showed partial anomalous systemic venous drainage in the form of right superior vena cava drained into left atrium. Four sessions of Endovascular embolization were performed. Surgical repair of partial anomalous systemic venous drainage was done successfully.ConclusionsThe superior vena cava in our case overrides the atrial septum promoting direct drainage of venous return into the LA, thus causing dilated left ventricle instead of dilatation of right ventricle which is the usual presentation of VAGMs.
Background: Patent ductus arteriosus (PDA) stenting is an alternative to modified Blalock-Taussig shunt (MBTS) as first-stage palliation of duct-dependent lesions.The superiority of one approach over the other is still controversial. Our objective was to compare PDA stent versusMBTS for palliation in regard to safety, efficacy, and efficiency. Methods: From 2010 to 2021, 134 patients had first-stage palliation with either PDA stent (n = 83) or MBTS (n = 51). Twenty-seven patients failed the primary treatment and were converted to the other group. The study endpoints were hospital outcomes, interstage reintervention, and concomitant procedures at the second-stage palliation. Results: Patients with PDA stent were significantly younger. The prevalence of antegrade pulmonary blood flow (PBF) was higher in patients who had MBTS and graft thrombosis was higher in the PDA stent. Hospital stay was significantly longer in patients who had MBTS. Predictors of prolonged mechanical ventilation were lowweight, MBTS, and conversion. Intensive care unit stay significantly increased with conversion, low-weight, and antegrade PBF. The interstage intervention was required more frequently in PDA-stent group. Predictors of reintervention were conversion and pulmonary atresia with the intact interventricular septum. Pulmonary artery plasty was required more frequently during the second-stage palliation in PDA-stent group. Conclusion: PDA stent is an alternative to MBTS for first-stage palliation. It is associated with shorter hospital stays and avoidance of surgery at the expense of a high rate of stent thrombosis and interstage reintervention. Conversion increased the risk of the procedure. More studies are needed to determine factors that affect PDA-stent outcomes and patient selection criteria.
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