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BACKGROUND The purpose of this study was to estimate survival and quality of outcome and assess factors associated with outcome for patients out 5 to 15 years from their Fontan operation. METHODS AND RESULTS We studied 352 patients who had the Fontan operation prior to 1985. The overall 1-, 5-, and 10-year survival was 77%, 70%, and 60%, respectively. The following factors were significantly associated with lower survival: univentricular heart or complex congenital anomalies other than tricuspid atresia, early calendar year of operation, heterotaxia syndromes, early age at operation, increased pulmonary artery pressure, atrioventricular valve dysfunction, and higher (worse) New York Heart Association class. Reoperations were necessary for 103 of the 352 patients. At least 20% of the survivors have or have had cardiac arrhythmias requiring antiarrhythmic medication or mechanical pacemaker insertion. Between 7% and 10% of the patients have had or had protein-losing enteropathy/hypoproteinemia. At 5 years postoperatively, 122 patients (34.7%) were alive with a better New York Heart Association functional classification than preoperatively. Fifty-eight patients (16.5%) were alive and in the same functional classification, but 126 (35.8%) died within the first 5 years or were in a worse functional classification. Thirty-nine patients were doing excellently and 29 patients poorly 5 years after the operation. Of the surviving patients, 43% can do as much exercise as their peers, whereas 3% are incapable of exercise. CONCLUSIONS To assure good functional long-term outcome in addition to survival, clinicians must exclude from selection for Fontan operation patients known to be at high risk for death or poor outcome.

Scalar electrocardiograms of 31 patients with double-outlet right ventricle (19 with and 12 without pulmonary stenosis) were correlated with the hemodynamic and anatomic findings. Atrioventricular conduction defects, intraventricular conduction delay, and counterclockwise frontal-plane loops were found less often than formerly reported. Counterclockwise frontal-plane loops were found with, as well as without, pulmonary stenosis. The presence or absence of left ventricular hypertrophy did not correlate with the severity of pulmonary vascular obstructive disease. Electrocardiographic evidence of left atrial enlargement was difficult to interpret. Additional intracardiac malformations could not be appreciated from the electrocardiographic findings. The predictive value of the electrocardiogram for the diagnosis of double-outlet right ventricle was considered to be low. In patients with double-outlet right ventricle without pulmonary stenosis, the electrocardiogram was not helpful in predicting the severity of pulmonary vascular obstructive disease.

Echocardiograms were performed on 20 children who were 1-14 years old. Ten children had secundum atrial septal defects (ASD 2°); four had partial and two had complete atrioventricular canal defects (AVC); and three had partial and one had total anomalous pulmonary venous connection (APVC). Twenty-eight normal children were also examined. Echocardiograms of nine patients with ASD 2° revealed increased right ventricular dimension index (RVDI) and abnormal (paradoxic) ventricular septal (VS) motion. The remaining patient with ASD 2°, who had a Qp/Qs of 1.2 (smallest in the series), had a normal echocardiogram. All four patients with APVC had increased RVDI, and three had abnormal VS motion. The patient with normal septal motion had associated mild aortic stenosis, but he too had a small left-to-right shunt (19%). The echocardiograms of two patients with partial AVC revealed features similar to ASD 2°, but in the third patient who had significant mitral regurgitation (MR) the VS motion was normal, indicating that the degree of associated MR influences the VS motion in this anomaly. The septal motion was normal in both patients with complete AVC. Three patients in the series had abnormal systolic anterior motion of mitral valve. Postoperative echocardiograms revealed persistence of increased RVDI and "paradoxic" VS motion.

Atrial septal defect (ASD) of the fossa ovalis type was found in 16 members of one family and confirmed in 15 members at autopsy or surgery or by cardiac catheterization. Sinus rhythm was present in 12 affected members, 11 of whom had electrocardiographic evidence of prolonged atrioventricular (A-V) conduction. Five other family members without ASD also had prolonged A-V conduction. The pedigree chart suggests that the syndrome ASD with prolonged A-V conduction is the manifestation of a single mutant autosomal gene with dominant effect, a high degree of penetrance, and some variation in expressivity. Earlier reports of familial ASD showing the autosomal-dominant pattern of inheritance reveal a similar frequency of prolonged A-V conduction among affected persons. The great majority of cases of ASD are sporadic, with little likelihood of recurrence in subsequent sibs or children. We suggest, however, that when ASD of the fossa ovalis type is accompanied by prolonged A-V conduction, the genetic prognosis may be drastically changed to a risk of almost 50% that the condition will recur in subsequent sibs or children of affected persons.

Simultaneous determinations of serum potassium and sodium, blood pH, blood pressure, and 12-lead electrocardiograms were obtained in hyperkalemic patients with chronic renal failure. Statistical investigation of the data showed a high correlation between serum potassium levels and ventricular repolarization abnormalities. "Mean intracellular potassium concentration" derived from total body potassium and total body water values was not accurate enough to show significant correlations to ECG changes and did not improve on the already significant correlations with serum potassium levels. Equations are presented which would permit the estimation of serum potassium levels (in a narrow range) from electrocardiographic findings in patients with chronic renal failure and suspected hyperkalemia.

Muscular obstruction to left ventricular outflow is a rare but serious occurrence. Eight cases in which this disease was anatomically proved are presented. The commonest symptoms are angina, dyspnea, and palpitations. Not uncommonly a family history of heart disease may be elicited. A systolic murmur along the left sternal border is the most characteristic physical finding, but a normal aortic second sound, the presence of third and fourth heart sounds, splitting of the second sound, and the absence of a diastolic murmur are all helpful clinical guides. Withdrawal pressures from the left ventricle reveal a characteristic infundibular pressure zone. Other hemodynamic findings include an anacrotic notch on the left ventricular pressure tracing, a rapidly ascending bisferious aortic pulse and a small compensatory beat after extrasystoles. Necropsy specimens reveal a grossly thickened interventricular septum as compared with normal and hypertrophied control hearts. Microscopic alterations are minimal. The etiology remains obscure but familial and developmental factors, either or both, appear to be implicated.

Direct surgical relief of diffuse subvalvular aortic stenosis has been accomplished in two cases by open operation through a left ventriculotomy.

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.

Progressive histologic changes occur in the pulmonary arteries and arterioles, as a complication of chronically elevated pulmonary arterial blood pressure, in patients with congenital septal defects of the heart. This progression is so stereotyped as to allow a division of the structural effects into 6 grades. The histologic features of each grade are described in detail in this communication. These results afford a basis for comparing the magnitude of these changes to the clinical findings.

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.