R A Elderkin1, D J Radford. 1. Queensland Centre for Congenital Heart Disease, Prince Charles Hospital, Chermside, Queensland, Australia.
Abstract
OBJECTIVE: To review the presentation, diagnosis, histology and outcome of primary cardiac tumours presenting to a paediatric cardiac unit over a 20-year period. METHODS: Hospital records and data bases were searched for the years 1980-2000. RESULTS: There were 12 patients with four histological tumour types including a predominance of rhabdomyoma, as well as myxoma, fibroma and myocardial hamartoma. Diagnosis was made in utero, by ultrasound in five cases and in the neonatal period in a further three cases. Six cases (50%) required surgical intervention and there were three tumour-related deaths. Two infants with large left ventricular tumours diagnosed in utero developed univentricular physiology, acting like hypoplastic left heart syndrome at birth. CONCLUSIONS: Despite an absence of malignant histology there was significant mortality and morbidity among the patients reviewed. The development of univentricular physiology in infants with large left ventricular tumours is rare and is a difficult management problem.
OBJECTIVE: To review the presentation, diagnosis, histology and outcome of primary cardiac tumours presenting to a paediatric cardiac unit over a 20-year period. METHODS: Hospital records and data bases were searched for the years 1980-2000. RESULTS: There were 12 patients with four histological tumour types including a predominance of rhabdomyoma, as well as myxoma, fibroma and myocardial hamartoma. Diagnosis was made in utero, by ultrasound in five cases and in the neonatal period in a further three cases. Six cases (50%) required surgical intervention and there were three tumour-related deaths. Two infants with large left ventricular tumours diagnosed in utero developed univentricular physiology, acting like hypoplastic left heart syndrome at birth. CONCLUSIONS: Despite an absence of malignant histology there was significant mortality and morbidity among the patients reviewed. The development of univentricular physiology in infants with large left ventricular tumours is rare and is a difficult management problem.