SUMMARY The clinical and morphologic features of hypertrophic cardiomyopathy in 20 patients recognized as having cardiac disease in the first year of life are described. Fourteen of these 20 infants were initially suspected of having heart disease solely because a heart murmur was identified. However, the infants showed a variety of clinical findings, including signs of marked congestive heart failure (in the presence of nondilated ventricular cavities and normal or increased left ventricular contractility) and substantial cardiac enlargement on chest radiograph. Other findings were markedly different from those usually present in older children and adults with hypertrophic cardiomyopathy (e.g., right ventricular hypertrophy on the ECG and cyanosis). Consequently, in 14 infants, the initial clinical diagnosis was congenital cardiac malformation other than hypertrophic cardiomyopathy.Twelve of the 14 infants who underwent left-heart catheterization showed substantial obstruction to left ventricular outflow (peak systolic pressure gradient 35 mm Hg). However, unlike older patients with hypertrophic cardiomyopathy, infants with this condition commonly had marked obstruction to right ventricular outflow (35-106 mm Hg) (nine patients); in six patients, the magnitude of obstruction to right ventricular outflow was at least as great as that to left ventricular outflow.Asymmetric hypertrophy of the ventricular septum relative to the left ventricular free wall was present in the 16 patients who had echocardiographic or necropsy examination. Ventricular septal thickening was substantial in patients studied both before and after 6 months of age (mean 16 mm), indicating that in patients with hypertrophic cardiomyopathy, marked left ventricular hypertrophy may be present early in life and is probably congenital.The clinical course was variable in these patients, but the onset of marked congestive heart failure in the first year of life appeared to be an unfavorable prognostic sign; nine of the 11 infants with congestive heart failure died within the first year of life. In infants with hypertrophic cardiomyopathy, unlike older children and adults with this condition, sudden death was less common (two patients) than death due to progressive congestive heart failure.

Postoperative data were obtained at cardiac catheterization in twelve patients studied 6-29 months following Mustard's operation for transposition of the great arteries (TGA) to assess the incidence and severity of abnormalities of right ventricular (RV) function. Age at operation was 5-13 months in seven patients (infant group) and 19-25 months in the remaining five patients. RV end-diastolic volume (EDV) decreased in all patients following surgery and averaged 123% of normal in the postoperative group (NS). RV ejection fraction (EF) was depressed postoperatively averaging 0.45 (69% of normal (P less than 0.001) as was RV systolic output (78% of normal, P less than 0.01). LVEDV averaged 65% of normal (P less than 0.001), LVEF 0.67 (103% of normal, NS), and LV systolic output 67% of normal (P less than 0.001) following operation. Left atrial (systemic venous) volume was decreased in all postoperative patients averaging only 39% of normal (P less than 0.001). A high incidence (greater than 50%) of partial baffle obstruction was found and LV systolic output showed a significant negative correlation with baffle gradients. The low output postoperatively may be related to decreased LV filling pressure, a small LV reservoir, and thus a small atrial "booster pump." Pressure-velocity indices of RV contractile function in four patients showed a poor correlation with pump function. Long-term follow-up will be required to determine the clinical significance of the abnormalities of venous return and ventricular function.

SUMMARY Right heart volume data were obtained in 63 patients with tetralogy of Fallot. The patients were divided into three groups: 1) preoperative tetralogy (N=34); 2) post shunt procedure (N= 14); 3A) post repair without outflow patch (N= 10); 3B) post repair with an outflow patch (N=8). In Group 1 right ventricular end-diastolic volume (RVEDV), RV ejection fraction (EF), and RV systolic output (SO) were all mildly depressed. In post shunt patients, RVEDV was normal but RVEF remained depressed. RVEDV and RVSO increased following a shunt procedure, and these variables were larger in patients with a large versus a small shunt. In Group 3A RVEDV, ALTHOUGH TREATMENT for suspected right ventricular (RV) failure is common after tetralogy of Fallot repair, very little information is available on the direct measurement of RV performance in these patients. Such measurements may become increasingly important in the evaluation of current methods of treatment for both the infant and the older child with tetralogy of Fallot. The purpose of this investigation, therefore, was to analyze biplane cineangiocardiograms in patients before and after surgery for tetralogy of Fallot to determine right ventricular end-diastolic and end-systolic volumes, ejection fraction, systolic output, and maximum right atrial volume and to attempt to correlate these findings with clinical course and other pertinent hemodynamic data. MethodsAll patients studied at Vanderbilt Hospital from July 1971 to October 1975 with the diagnosis of tetralogy of Fallot and adequate biplane cineangiocardiograms for analysis were included. Tetralogy of Fallot was defined as the condition in which there is a large ventricular septal defect with equal right and left ventricular peak systolic pressures associated with right ventricular outflow tract obstruction, and bidirectional shunting. There were three basic patient groups.Group 1 consisted of patients prior to any surgical procedure. There were 34 studies performed on 32 patients in this group whose ages ranged from 3 days to 12 years with an average age of 2.1 ± 0.4 yr (mean ± SEM). Sixteen patients were less than one year of age and 24 were less than two years. Arterial oxygen saturation (02 SAT) ranged from 67-98% at rest and averaged 80 ± 2%. Only four patients had arterial 02 SATs at rest . 90%, and all four had a de- 417RVEF, and RVSO were normal. In contrast in patients in Group 3B, RVEDV was increased averaging 177 ± 15% of normal, RVEF was depressed averaging 0.45 ± 0.04, and RVSO was normal. RV size and pump function are abnormal in patients whose operation requires an outflow tract patch and the factors which may contribute to these abnormalities include a higher RV peak pressure, pulmonary incompetence, and a larger noncontractile outflow tract. Longitudinal studies relating these variables to clinical performance and exercise testing will be important in assessment of the importance of these abnormalities.crease in 02 SAT with crying. Hematocrits ranged from 36-73% with an average of 47 ± 2%, tabl...

Right ventricular (RV) volumes were calculated from biplane cineangiocardiograms in 46 patients undergoing diagnostic cardiac catheterization. Validation of methodology was performed by comparison of known and calculated volumes of postmortem RV casts as well as by comparison of cineangiocardiographic RV and left ventricular (LV) stroke volumes of patients without shunts or valvular insufficiency. Seven infants, <1 year of age, with normal right hearts as compared with older children showed smaller RV end-diastolic volumes (39 ± 8 vs 70 ± 13 ml/m 2 , P < 0.001) as well as decreased RV systolic indices (SI) (3.71 ± 0.68 vs 4.66 ± 1.10 liters/min/m 2 , P < 0.05). There were no differences between normal infants and older children for RV ejection fraction (EF), RVEDV/LVEDV = 1.01, RVSI/LVSI = 0.99, and RVEF/LVEF = 1.04 vs 0.99. In 13 patients with isolated pulmonary stenosis, RVEDV, RVEF, RVSI, RVEDV/LVEDV, and RVSI/LVSI were not different from normal, but RVEF/LVEF averaged 1.13 vs 0.99 in normal infants, P < 0.05. In contrast, 11 patients studied with atrial septal defect or total anomalous pulmonary venous connection had significant increases in RVEDV (128 ml/m 2 ), RVSI (9.34 liters/min/m 2 ), RVEDV/LVEDV (2.36), RVSI/LVSI (2.81), and RVEF/LVEF (1.17), but normal values for RVEF. There was a significant linear relationship between Q p /Q s from oxygen data and RVSI/LVSI. In three patients studied an average of 1 year following atrial septal defect (ASD) repair, RVEDV remained elevated. In volume overload, alterations in RV volume characteristics are apparent and can be useful in shunt estimation; adaptation to an RV pressure overload, however, is not associated with detectable volume alterations.