Although most patients with coarctation of the aorta survive to early adult life, it has been adequately demonstrated that some patients die in infancy and early childhood as a result of their coarctation. In selected cases, then, operation is advisable at a very early age. Cardiac failure in a 10 week old infant necessitated operation at this age in the case reported.
Pulmonic stenosis unassociated with a ventricular septal defect of significant size is often a complex abnormality. The stenosis may be valvular, infundibular, or both, and may be associated with an atrial septal defect or even with a small ventricular septal defect. Preoperative studies cannot accurately predict these variations. The surgical approach selected for such patients should permit the correction of each of the cardiac defects encountered. That this can be accomplished by means of extracorporeal circulation and open cardiotomy is demonstrated by the presented series of 10 cases without operative mortality.
Long-term outcome of patients undergoing surgical repair of isolated pulmonary valve stenosis. Follow-up at 20-30 years.
In a group of 191 consecutive patients who underwent operation at the Mayo Clinic for isolated pulmonary valve stenosis between 1956 and 1967, late results were excellent. In patients surviving operation before the age of 21 years, long-term survival was similar to that in an age- and sex-matched control population. In the other patients, late survival and functional status were good, but survival was poorer than in the control population. This late attrition, most likely due to the sequelae of long-standing right ventricular hypertrophy, suggests the need for continued surveillance. Late sudden death occurred in only three patients, between 10 and 21 years postoperatively. The finding of normal life expectancy for such patients who survive operation before age 21 is important for actuarial purposes and for the patients' career and employment plans.