Five infants under three months of age with tetralogy of Fallot and absence of the leaflets of the pulmonary valve were successfully corrected using a one-stage repair. All patients suffered from respiratory failure preoperatively and surgery was performed somewhat urgently. The age at surgery ranged from 26 to 72 days, with an average of 47±17 days. The surgical procedures involved four steps. First, we plicated the aneurysmally dilated central pulmonary arteries. Second, we closed the ventricular septal defect. We then inserted a handmade heterograft pericardial valve at the site of the pulmonary valve, and completed the reconstruction of the right ventricular outflow tract with a patch. All patients survived. Two of them were successfully weaned from ventilation at early (two and 11 days, respectively) periods after surgery, but the remaining three patients required artificial ventilation for one to two months. We conclude that one-stage repair is possible for this anomaly, but that plication of the pulmonary trunk is an essential technique todeliver patients in early infancy from respiratory failure.

About 1.5 million children are born each year world-wide with congenital heart disease. The nine most frequently occurring lesions are patency of the arterial duct (ductus arteriosus), atrial septal defect within the oval fossa, atrioventricular septal defect, ventricular septal defect, coarctation of the aorta, valvar pulmonary stenosis, valvar aortic stenosis, tetralogy of Fallot, and complete transposition. Their natural history is presented and compared with representative results of surgical treatment. Surgery has markedly improved the outcome for all these lesions, although almost no patients have been followed postoperatively for more than 25 years. Because congenital heart lesions are likely to consume an increasing proportion of cardiological resources in future years, the long-term future in this field rests with prevention of these lesions if at all possible. Recent advances in molecular and developmental biology, some of which are discussed, will certainly aid in diagnosis and may well bring the time at which prevention can be achieved closer to reality.

To investigate the left ventricular systolic and diastolic function in patients with pulmonary atresia with intact ventricular septum without coronary-cardiac fistulas after a modified Blalock-Taussig shunt, 14 patients (age range 15 days-16.5 months, mean 4.03±5.6 months) and eight control subjects, matched for age, body surface area and heart rate were evaluated by cross-sectional and Doppler echocardiography. The follow-up interval after palliative procedures ranged from 12 days to 16.3 months (mean 3.67±5.6 months). Compared to controls, in the group of patients the ejection fraction was decreased (61±7% vs 68±5%, p=0.022) while the left ventricular end-diastolic volume indexed for body surface area was increased (72.7±10.8 cc/m2 46.1±12 cc/m2 p=0.0001) with normal values of left ventricular mass indexed for body surface area (67.88±20.9 g/m2 vs 76±10 g/m2 p=NS). Mass-to-volume ratio was lower in patients with pulmonary atresia (0.95±0.38 vs 1.24±0.3, p=0.031). The left ventricular shape index was increased in all patients with pulmonary atresia (1.27±0.26 vs 1±0.01, p=0.009). A significant inverse correlation was noted between the ejection fraction and follow-up (r=−0.71, p=0.04). as well as between the ejection fraction and shape index (r=−0.76, p=0.048). Moreover, the patients with pulmonary atresia had decreased E/A velocity ratio (0.65±0.16 vs 1.35±0.90, p=0.009), decreased normalized peak filling rate (4.16±0.13 sv/s vs 6.88±0.68 sv/s, p=0.0001), increased peak A velocity (0.95±0.17 m/s vs 0.51±0.16 m/s, p=0.0001) and prolonged isovolumic relaxation time (46±5.4 ms vs 34±6.2 ms, p=0.0001) and deceleration time (196.4±32.2 ms vs 116±21.4 ms, p=0.0001). There was a good correlation between the normalized peak filling rate and follow-up (r=−0.80, p=0.04). These data show a progressive compromise of the left ventricular systolic and diastolic function in patients with pulmonary atresia with intact ventricular septum without ventriculocoronary fistulas who had undergone systemic-to-pulmonary arterial shunting. Thus, an earlier biventricular or Fontan type procedure should be recommended.

A 64-element phased-array echocardiographic transducer mounted on a sliding carriage was used transthoracically in 11 patients with subaortic obstruction caused by various different morphological substrates. The transducer is moved in a plane from the outflow tract to the apex of the heart in steps of 0.5 to 1.3 mm and records a tomographic slice of the heart at each step. Parallel images are recorded at a frame rate of 25–30 images per second and triggered to heart rate and respiration. A complete cardiac cycle is recorded at each level. The images are digitized and stored in the image-processing computer, which reconstructs the anatomic structures of the heart in a three-dimensional format by means of different gray-scales. Good quality echocardiographic pictures were obtained in 10 of the 11 patients and three-dimensional reconstructions were possible in those 10 patients. The technique is able to display the heart in real-time in any desired plane, and in up to five planes simultaneously, without the need to change the position of the transducer on the chest. Thus the best views with which to display the substrate of subaortic stenosis could be selected. The left ventricular outflow tract could also be displayed in a view similiar to the one obtained by the surgeon during operative procedures for resection of the subaortic stenosis through an incision in the aortic root. Image acquisition took three to five minutes, and three-dimensional reconstruction of various cardiac structures took 20–90 minutes. Display of the subaortic area in three-dimensional format may enhance our understanding of cardiac anatomy and identify the different morphological lesions in the left ventricular outflow tract which cause subaortic obstruction.

Diffuse endocardial thickening in dilated hearts has been considered as a distinct pathological entity, usually being termed endocardial fibroelastosis. It is common in infants and children and rare in adults. Current opinion holds that the endocardial thickening is a non-specific reaction to stress in the ventricular wall. Our personal observations point to the most severe forms occurring in young infants. To determine the frequency and severity of endocardial fibroelastosis in children with dilated cardiomyopathy, we studied 21 necropsy hearts, both grossly and microscopically. Ages of the patients ranged from two to 175 months (mean 46.5, median 23 months). Using a graduated eyepiece, we measured the endocardial thickness in samples from three sites in the left ventricle and one from the right ventricle. The values were compared to those obtained from similar measurements of the endocardium in hearts from four children presenting with tetralogy of Fallot. All the dilated hearts presented some degree of endocardial thickening. The gross detection of fibroelastosis depended on the degree of thickening. There was a negative but linear correlation between age and the mean endocardial thickness (r=0.70; p>0.001). Our findings support the concept that endocardial cells have greater replicative and secretory potentials in young patients, and that endocardial fibroelastosis is a secondary phenomenon in dilated hearts.

Coronary angiograms were analyzed in 29 children after heart transplantation. Age at transplantation was one month to 14 years (mean 4.5 years) with follow-up from five months to six years (mean 2.9 years). Immunosuppression was based on cyclosporine and azathioprine; six patients also received maintenance steroids. Coronary angiograms were obtained after giving an intracoronary arterial bolus of nitroglycerin to avoid spasm. Coronary angiograms showed stenoses, luminal irregularities, loss of luminal diameter and obliteration of branches, and one example of myocardial bridging. The review of angiographic recordings in some patients showed a loss of normal motion of coronary arteries which, instead, appeared rigid. No abnormalities were seen in eight patients (28%). Coronary arterial changes were present between six months and four years after transplantation in 15 patients (52%). Isolated loss of normal motion of the coronary arteries was present in six patients (20%). The separate analysis of 15 patients who underwent more than one coronary angiogram showed a progression of lesions in eight cases. Coronary arterial disease is a frequent complication of heart transplantation in children and there is a progression of lesions in a large proportion of patients. Since there is no satisfactory therapy for this complication, heart transplantation should be reserved for children having no other reasonable therapeutic option.

The arterial switch operation has become the standard of surgical repair for total correction of complete transposition (concordant atrioventricular and discordant ventriculoarterial connections). A standard nomenclature for classification of the numerous anatomic relationships the coronary arteries may assume, however, has yet to be universally adopted. Inconsistent use of varied nomenclatures remain an obstacle to effective communication between physicians and surgeons. In this review we offer a unified nomenclature incorporating five important relationships: the configuration of the sinuses; their location; the configuration of the arterial orifices; the pattern of branching of the major coronary arteries; and the course of each artery.