ABSTRACT
Pentalogy of Cantrell is a rare disease. Approximately 185 cases have been reported around the world. The authors performed a retrospective study that reviewed the clinical files and pathological samples of 22 cases of pentalogy of Cantrell treated at the Hospital Infantil de México Federico Gómez. Thirteen patients had ectopia cordis associated with pentalogy of Cantrell (group I), and there were 9 cases without ectopia cordis (group II). In group I, the following types of congenital heart disease were found: single ventricle (4), double-outlet right ventricle (4), ventricular septal defect (3), aortic coarctation (1), and atrial septal defect (1). In group II, the following types of congenital heart disease were found: double-outlet right ventricle (3), double-inlet left ventricle (2), ventricular septal defect (2), tetralogy of Fallot (1), and hypoplastic right ventricle syndrome (1). Nine cases had a ventricular diverticulum (40%). Ten patients (45%) had some other congenital anomaly associated with pentalogy of Cantrell. Thirteen patients underwent surgery (59%), which included cardiac surgery in 10 cases (45%). Sixteen patients died (73%): 11 from group I and 5 from group II (P < .05). Little more than 50 years since it was first described, pentalogy of Cantrell remains a disease with high mortality, especially in patients with associated ectopia cordis.
ABSTRACT
OBJECTIVE: To evaluate the effect of carvedilol administrated during 8 months in children with heart failure secondary to dilated cardiomyopathy. METHODS: We initiated carvedilol in patients who, despite optimization of standard treatment, had persistent left ventricular ejection fraction < or = 40% and evaluated the systolic and diastolic left ventricular function before and after 4 and 8 months of treatment. RESULTS: 19 patients were included in the study, 10 women; median age: 6.7 years and 17 +/- 12.4 months after the diagnosis. The ejection fraction improved significantly from entry to eight months (median: 33.5 to 52.6%, p = 0.01) as did the shortening fraction (m: 14.8 to 25.8%, p = 0.01). The myocardial performance index abnormal in all 19 patients became normal in 5 at the end of study. In addition mitral Doppler flow which was altered in 9 improved in 3 and pulmonary venous flow abnormal in all patients improved in 13. Only 1 patient presented minimal adverse effects. CONCLUSIONS: Carvedilol added to standard therapy for dilated cardiomyopathy improves left ventricular function, is well tolerated, and has minimal adverse effects in childhood.
Subject(s)
Carbazoles/therapeutic use , Cardiomyopathy, Dilated/drug therapy , Propanolamines/therapeutic use , Adolescent , Carvedilol , Child , Child, Preschool , Female , Humans , Infant , Male , Prospective StudiesABSTRACT
OBJECTIVE: To evaluate the effect of carvedilol administrated during 8 months in children with heart failure secondary to dilated cardiomyopathy. METHODS: We initiated carvedilol in patients who, despite optimization of standard treatment, had persistent left ventricular ejection fraction < or = 40% and evaluated the systolic and diastolic left ventricular function before and after 4 and 8 months of treatment. RESULTS: 19 patients were included in the study, 10 women; median age: 6.7 years and 17 +/- 12.4 months after the diagnosis. The ejection fraction improved significantly from entry to eight months (median: 33.5 to 52.6%, p = 0.01) as did the shortening fraction (m: 14.8 to 25.8%, p = 0.01). The myocardial performance index abnormal in all 19 patients became normal in 5 at the end of study. In addition mitral Doppler flow which was altered in 9 improved in 3 and pulmonary venous flow abnormal in all patients improved in 13. Only 1 patient presented minimal adverse effects. CONCLUSIONS: Carvedilol added to standard therapy for dilated cardiomyopathy improves left ventricular function, is well tolerated, and has minimal adverse effects in childhood.
