T. B. Cartmill

We report a technique for the enlargement of a hypoplastic aortic root by an operation whereby the hypoplastic aortic root has been so enlarged by the insertion of a Dacron fabric gusset that it will accommodate a size 9A or larger Starr-Edwards prosthesis. Our experience in five patients is described. No matter what type of valve is used for replacement of a diseased aortic valve, and no matter what improved designs of valvular prosthesis are ultimately developed, it will be necessary (in the particular group described) to enlarge the aortic ring to accommodate a size which will function correctly without causing left ventricular outflow obstruction.

Five cases of the Hirschsprung's disease-congenital central hypoventilation syndrome (CCHS) association are presented and 41 other published cases reviewed. These children have a distinct pattern of associated features, an equal sex incidence, and a characteristic spectrum of disease severity which suggests that the condition is genetically distinct from other cases of Hirschsprung's disease. While approximately 1.5% of Hirschsprung's disease patients, and 10% of those with total colonic aganglionosis, will have CCHS, up to 50% of CCHS patients will have Hirschsprung's disease. Approximately 20% of CCHS/Hirschsprung patients will also have neuroblastoma or ganglioneuroma, usually multiple. Abnormalities of the eye and autonomic nervous system are also common. The ventilatory abnormality is usually evident on the first day of life. The aganglionosis is also severe, with more than half (59%) of the patients having aganglionosis extending into the small bowel.

OBJECTIVE: To assess the effectiveness and safety of amiodarone in the treatment of junctional ectopic tachycardia (JET) after open heart surgery in children. PATIENTS: Between January 1990 and December 1991, 16 consecutive patients aged 6 days to 14 years with JET associated with significant haemodynamic impairment after cardiopulmonary bypass were treated with amiodarone as the principal antiarrhythmic drug. INTERVENTIONS: Amiodarone 5 mg/kg was administered intravenously over one hour and the same dose was subsequently infused over 12 hours. This was reviewed every 12 hours and repeated as necessary until a satisfactory heart rate and stable haemodynamics were achieved. Atrial pacing was used whenever possible to provide atrioventricular synchrony. RESULTS: Except for one patient with a JET rate of 160/min, the maximum JET rate ranged from 180/min to 245/min with a mean(SD) of 200 (20)/min. After amiodarone, the heart rates reduced to a mean(SD) of 170 (20), 164 (27), 158 (27), 157 (24), and 153 (19)/min at two, four, eight, 12, and 24 hours respectively. A reduction in tachycardia rate allowing atrial pacing was achieved in 10 patients by two hours. Haemodynamic variables improved in most patients with an increase in mean systolic blood pressure by an average of 15 mm Hg and a decrease in atrial filling pressures by an average of 3.5 mm Hg at four hours after amiodarone administration. There were three deaths: one was a moribund patient who died soon after the onset of JET and the other two deaths were not directly related to JET. COMPLICATIONS: Late bradycardia with hypotension was recorded in one patient. Asymptomatic late sinus bradycardia was seen in several others. CONCLUSIONS: Amiodarone can be used safely and effectively to control JET with haemodynamic improvement in most patients. The addition of atrial pacing confers the advantage of atrioventricular synchrony.