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BACKGROUND: The Fontan procedure is the final stage of a three-stage palliation process in patients born with a univentricular heart as part of hypoplastic left heart syndrome (HLHS) or other pathologies with a univentricular heart. As essential as this procedure has proven to be for such cases, the Fontan physiology diminishes cardiac output and expands systemic venous pressure, which then leads to venous congestion that can be complicated by protein-losing enteropathy (PLE). This retrospective study aimed to identify the predictors of such complications in all patients who underwent completion of the Fontan procedure at our center (Sheikh Khalifa Medical City/SKMC) in the past eight years. METHODS: This study examined the medical records of patients who underwent completion of Fontan repair at our center since the inauguration of the cardiac surgery program of SKMC in the United Arab Emirates (UAE) - 01 Jan 2012 to 31 Dec 2020. Exclusion criteria included the absence of any of the undermentioned data in patient files. Patients were divided into two groups: those who developed PLE and those who did not. For each group, the following data were collected: demographics data (current age and age at completion of Fontan), clinical and laboratory data (oxygen saturation, serum albumin), echocardiographic data (classification of original cardiac diagnosis, degree of atrio-ventricular valve regurgitation, ventricular functions), hemodynamic data (mean pressures of superior vena cava and pulmonary arteries before Fontan completion), operative data (type of initial palliation, type of Fontan, presence of fenestrations and its size) and the need for any cardiac intervention prior to Fontan completion, such as atrio-ventricular valve repair, peripheral pulmonary stenting and arch balloon dilatation. RESULTS: Of the 48 included patients,13 (25%) developed PLE. Multivariate regression analysis proved that the best predictors of PLE were superior vena cava mean pressure (P = 0.012) and the degree of atrio-ventricular valve regurgitation (P = 0.013). An oxygen saturation <83% prior to Fontan completion was 92% sensitive in predicting PLE after Fontan completion. CONCLUSION: This is a single-center study of the predictors of PLE after Fontan procedure and, as expected from similar studies, SVC pressure higher than 11 mmHg and moderate-to-severe atrio-ventricular valve regurgitation were predictors of Fontan failure. The higher prevalence of PLE in our cohort, as well as lower cut-offs of SVC pressure that can predict complications, may be related to the predominance of hypoplastic left heart in the operated patients, which has been the main referral center for cardiac surgeries in UAE in the last decade.
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7-year-old boy, who underwent aortic valve replacement two years previously, suffered from idiopathic dilated cardiomyopathy. Because of poor condition (NYHA-IV), heart transplantation was performed on 18th October 2007. It was the first pediatric heart transplantation in Hungary. It was an uneventful early postoperative period, 6 months after the operation he is doing well, no biopsy-proven and tissue Doppler echocardiography (TDI-derived velocities measurement) rejection was detected. The immunosuppression was based on triple-drug therapy (tacrolimus+mycophenolate mofetil+corticosteroid) with use of induction therapy with interleukin-2 receptor blocker (basiliximab).
Assuntos
Cardiomiopatia Dilatada/cirurgia , Transplante de Coração , Criança , Transplante de Coração/métodos , Humanos , Hungria , MasculinoRESUMO
Congenitally corrected transposition of the great arteries is a rare defect characterized by discordant atrioventricular and ventriculoarterial connections. Symptoms result from one or a combination of associated cardiovascular malformations, including ventricular septal defect, pulmonary stenosis or atresia, tricuspid valve dysfunction, dextrocardia, hypoplastic left or right ventricle. Correcting exclusively the associated defects, leaving the morphologic right ventricle in systemic position, will determine the patient's life-long prognosis. Anatomic repair by double switch technique may improve survival of patients with congenitally corrected transposition of the great arteries by establishing the morphologic left ventricle in the systemic circulation. A 3-year-old girl with congenitally corrected transposition of the great arteries, ventricular septal defect, hypoplastic right ventricle, and previous palliative procedure was corrected by double switch technique and patch closure of ventricular septal defect. The authors prefer the double switch procedure inspite of its many surgical challenges because it has very good long-term outcome. The more simple surgery namely the operation of only associated cardiac defects will involve the possibility of deterioration of right ventricular function.
Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Transposição dos Grandes Vasos/fisiopatologia , Transposição dos Grandes Vasos/cirurgia , Procedimentos Cirúrgicos Vasculares/métodos , Pré-Escolar , Dextrocardia/cirurgia , Feminino , Comunicação Interventricular/cirurgia , Ventrículos do Coração/patologia , Humanos , Atresia Pulmonar/cirurgia , Estenose da Valva Pulmonar/cirurgia , Resultado do Tratamento , Insuficiência da Valva Tricúspide/cirurgia , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/prevenção & controleRESUMO
INTRODUCTION: For infants and children with congenital aortic valve disease root replacement with pulmonary allograft (Ross procedure) is the preferred method of choice. PATIENTS/RESULTS: The authors have successfully applied this operation in 12 children (age range from 2.5 to 17 years--mean 9 years, body weight from 12 to 58 kg--mean 46 kg), one of whom has also required a Konno extension for long segment left ventricular outflow tract obstruction. The operation was complicated by early postoperative endocarditis in one case, and the child required redo homograft root replacement on the ninth postoperative day. All patients, including this one survived, and are doing well at present. CONCLUSIONS: In the Hungarian literature this is the first report on the Ross and Konno procedure in children. On the basis of our excellent early results, Ross procedure is the method of choice in aortic valve disease in children.
