The survival of patients to 28 years with the Mustard repair has been good. Late sudden death is the most worrisome feature. There is a 97% freedom from right ventricular failure to date. The quality of life of late survivors is good, most achieving a normal level of education and employment.
In an attempt to achieve safe intracardiac surgery in severely ill babies with congenital heart defects, a technique of deep hypothermia with surface cooling and limited bypass has been used. Under halothane anesthesia, these infants were cooled to a nasopharyngeal temperature of 26 C on a circulating water blanket and the temperature was lowered further to 22 C by a short period of total body perfusion. After a period of circulatory arrest which averaged 48 minutes at 22 C, during which the intracardiac repair was carried out, rewarming to 32 C was achieved by 20 minutes of total body perfusion and final rewarming to 36 C by surface means.
Thirty-three of 37 infants under 10 kg in weight, with correctable lesions, survived this procedure, including 25 aged 8 days to 12 months. Conditions corrected were transposition (13), tetralogy of Fallot (9), total anomalous pulmonary venous connection (6), ventricular septal defect (6), and atrioventricular canal (3). The technique gave ideal operating conditions and is believed to have wide application in the neonatal and infant group.
Doppler echocardiography is more sensitive than clinical assessment in the detection of carditis in acute rheumatic fever, and can contribute to earlier diagnosis.
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