Heart transplantation for chyloptysis after Fontan operation.

Chyloptysis is a rare clinical problem, fewer than 10 patients having been reported in the literature. We report a patient with intractable chyloptysis associated with a Fontan procedure, who was palliated by heart transplantation.

The vectorcardiogram in 4 children undergoing the arterial switch repair.

Four children with complete transposition (concordant atrioventricular and discordant ventriculo-arterial connections), aged 1.2 to 16.4 years, underwent a delayed repair by the arterial switch procedure from March 1985 to October 1986. Three children had undergone previous atrial diversion repairs in early childhood. Two of these children had developed right ventricular dysfunction with tricuspid incompetence as well as having recurrent and symptomatic supraventricular tachyarrhythmias. They underwent banding of the pulmonary trunk 0.7 and 1 year prior to the final repair. The third child had dynamic obstruction of the left ventricular outflow tract and prior banding was not performed. A fourth child had right ventricular dysfunction on initial assessment in early infancy and then had banding of the pulmonary trunk. Two patients survived the arterial switch operation. Vectorcardiograms were performed on these 4 children before arterial switch repair. The left maximal spatial voltage did not correlate with elevated left to right ventricular pressure ratio at cardiac catheterization before arterial switch repair nor did it relate to survival after operation. The horizontal loop was clockwise in all patients preoperatively. The vectorcardiogram in these children was not a useful indicator that the left ventricle was suitably prepared for the arterial switch repair.

The vectorcardiogram in infants undergoing arterial switch repair.

Twenty-five patients with transposition of the great arteries underwent primary arterial switch repair between July 1985 to October 1986. Twenty infants had intact ventricular septum or small ventricular septal defect while 5 infants also had moderate or large ventricular septal defects. Median age of switch repair was 2.9 weeks in those with small ventricular septal defect or intact septum and 7.6 weeks in those with significant defects. Vectorcardiograms were performed weekly preoperatively and then up to 4 to 6 months postoperatively. Cardiac catheterisation was performed preoperatively. Correlation between a left-to-right ventricular pressure ratio of more or less than 0.7 was made with a vectorcardiogram taken near cardiac catheterization. Preoperative vectorcardiograms in patients with essentially intact ventricular septum showed normal maximal spatial voltages in 60 and 65% (left and right) although 70% had clockwise horizontal loops. There was poor correlation between maximal spatial voltages and loops of the vectorcardiogram with the left-to-right ventricular pressure ratio at catheterization. Three of 5 patients with large ventricular septal defects had decreased left maximal spatial voltages or clockwise loops preoperatively. This suggests low left ventricular pressure ratio but at catheterization all had elevated pressure ratios. Despite decreased left maximal spatial voltages or clockwise horizontal loops in many patients in both groups, all survived the arterial switch procedure. Postoperatively, the vectorcardiogram normalized in those with essentially intact septum within a mean of 4 weeks but took longer in the others. The vectorcardiogram is unreliable in assessing suitability for the arterial switch procedure in the first 3 months of life.(ABSTRACT TRUNCATED AT 250 WORDS)

Surgical excision of primary cardiac tumours in infancy.

Six cases of primary cardiac tumour have been operated upon in a 7 year period from 1 June 1979 until 1 June 1986. All patients were under 6 months of age at the time of operation and two of the patients were in their first week of life. The principal indication for surgery was obstruction mainly at the right or left ventricular outflow tract level. More recently echo evaluation alone has been adequate to define the problem prior to surgery. Surgical excision has been performed without mortality or significant complications. In most cases resection has been complete, although in one case residual tumour has been left because of attachment of the tumour to vital structures. Follow-up of this case has not resulted in further surgery being required because of regrowth of the tumour. In one case, with co-existent congenital heart disease, the tumour was brought to notice after palliative systemic to pulmonary artery shunt had been performed. From the cardiac view point gratifying results have been obtained both in the short and long term following surgical resection. However, for patients with rhabdomyoma, later development of symptomatic tuberosclerosis should be anticipated in 50% of cases.