A long-term retrospective analysis of 248 patients, 11-25 years after surgical correction of coarctation of the aorta, revealed a high incidence of premature cardiovascular disease. Twelve percent of patients with follow-up have died. It is suggested that premature death in patients with adequate surgical repair may be related to the duration of preoperative hypertension. Fifty-nine patients were evaluated on a standard hospital protocol. Seventy-eight percent had evidence of cardiovascular disease and over 40% had no change or had increased blood pressure over the preoperative value. These data emphasize the importance of early diagnosis and treatment for patients with coarctation of the aorta as well as the need for close postoperative follow-up.

Subaortic stenosis is well known to complicate the clinical course of patients with single ventricle or univentricular hearts, and we have previously suggested that the development of subaortic stenosis in such patients may be causal to and/or accelerated by previous banding of the main pulmonary trunk. To further define the relationship between banding of the pulmonary artery in patients with univentricular hearts and the development of subaortic stenosis, we examined the morphologic substrate and timing of the development of subaortic stenosis in 43 patients seen at our institution from January 1, 1970, through June 30, 1985. These 43 patients include all patients in this period with an unequivocal univentricular heart whose longitudinal data was available for follow-up. We excluded patients who died within 1 week of surgery, patients lost to follow-up, and patients with evidence of subaortic stenosis before banding. Thirty-one of 43 patients (72. 1 %) developed subaortic stenosis subsequent to banding of the main pulmonary artery. The mean age at banding of those patients who developed subaortic stenosis was 0.21 years, and subaortic stenosis was recognized at a mean age of 2.52 years. For the specific cohort of patients whose ventricular morphology was a main chamber of left ventricular type supporting the pulmonary artery and a rudimentary right ventricle supporting the transposed aorta (32 patients), 27 developed subaortic stenosis (84.4%). Subaortic stenosis in the classic form of single ventricle usually results from progressive restriction of a wholly muscular interventricular communication. Banding of the pulmonary artery by producing myocardial hypertrophy undoubtedly accelerates the potential for subaortic stenosis in these patients. Furthermore, one must realize that subaortic stenosis may be present in the absence of a resting pressure gradient, and such subaortic stenosis can usually be unmasked by stimulation with isoprenaline. Finally, one must be guarded in advocating banding of the pulmonary artery in patients with single ventricle, realizing that subaortic stenosis strongly influences the outcome of more definitive surgery in these patients. Circulation 73, No. 4, 758-764, 1986. THE NATURAL HISTORY of patients with single ventricle (one-ventricle hearts, univentricular hearts, univentricular atrioventricular connection) has been irrevocably altered by those diverse surgical procedures that (1) augment pulmonary blood flow, (2) reduce pulmonary blood flow, (3) augment atrial mixing when intracardiac streaming is disadvantageous, or (4)

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A multidisciplinary team assessed 23 patients with various manifestations of the Noonan syndrome, including pulmonary valve stenosis (with leaflet dysplasia), "typical" facial appearance (including hypertelorism, epicanthic folds, flat nasal bridge, and apparently low-set ears), short stature, and mental retardation. Seven patients had a family history of the syndrome. A comprehensive scoring system was devised on the basis of frequency and severity of manifestations and results of invasive and noninvasive tests in these patients and those reported in the literature. The scoring system was condensed into a score card for clinical use and validated by "blind" application to patients with isolated pulmonary valve stenosis or suspected Noonan syndrome. Use of a scoring system to diagnose a syndrome for which there is no specific diagnostic test facilitates accuracy and decreases observer bias. In the case of unusual congenital disorders it is particularly valuable for a pediatrician in general practice.

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SUMMARY Serial changes in T-wave vector and polarity were assessed in 162 electrocardiograms, 117 from 44 healthy term neonates and 45 from 17 stressed neonates. Records were taken at 5 to 8 hours, 24 to 33 hours, and 71 to 96 hours after birth. Sequential changes in both T-wave amplitude and frontal and horizontal axes were found in both groups. A lag period was noted between healthy and stressed infants when comparing changes in T-wave amplitude, with greater flattening of T-waves for longer periods of time after birth in the stressed group. The normal changes in T-wave axis over time in the horizontal and frontal planes showed a similar lag in the stressed group. Alterations of T-wave amplitude and axis alone may be markers of myocardial ischaemia in neonates but are only reliable signs after the first 24 hours of life.