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Eight hundred twenty-two balloon pulmonary valvuloplasties were reported to the Valvuloplasty and Angioplasty of Congenital Anomalies Registry. Before and after systolic outflow gradients were recorded in 784 valvuloplasties, and the gradient decreased from 71 +/- 33 to 28 +/- 21 mm Hg. The sites of residual obstructions could be ascertained in 196 patients. In these, the total systolic outflow gradients decreased from 85 +/- 41 mm Hg to 33 +/- 27 mm Hg. Of this total residual gradient, 16 +/- 15 mm Hg was transvalvar and 18 +/- 24 mm Hg was infundibular. The degree to which infundibular obstruction subsequently resolved was not determined in this study. The procedure was less effective in reducing outflow gradients in patients with dysplastic valves with or without Noonan's syndrome. There were 5 major complications (0.6%), including 2 deaths (0.2%), a cardiac perforation with tamponade (0.1%) and 2 tricuspid insufficiencies (0.2%). There were 11 minor complications (1.3%) and 21 incidents (2.6%). The incidence of major complications, minor complications and incidents was inversely related to age; it was substantially higher in infants and, in particular, neonates. Balloon pulmonary valvuloplasty is a safe and effective method of lowering pulmonary outflow gradients in infants, children and adults. Small transvalvar and varied infundibular gradients commonly are present at the end of the procedure. Assessing the full effect of the procedure requires intermediate-term follow-up and assessing the duration of relief requires long-term follow-up.

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The results of hypospadias repair using 3.5 x magnification (53 cases) were compared with the results after using the Zeiss reconstruction microscope (59 cases). There were 33 MAGPI procedures, 47 flip-flap operations, 4 free graft patch repairs, 16 island pedicle patches, and 12 free graft tubed urethroplasties. Only one short-term complication occurred: a stricture after free graft urethroplasty using optical loupes. Although success of hypospadias repair is not totally dependent on use of the microscope, it is predicted that it will be used more often in the repair of hypospadias in the future because of its advantages.

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Endomyocardial biopsy was performed in 20 symptomatic pediatric patients with the following clinical diagnoses: dilated cardiomyopathy in 16, hypertrophic cardiomyopathy in 2 and myocarditis in 2. Transfemoral biopsy was performed without complications in patients aged 2 months to 16 years; 6 were less than 1 year old. An average of five right ventricular specimens were obtained for histologic and ultrastructure study from each patient. In 16 patients with the clinical diagnosis of dilated cardiomyopathy, biopsy findings were compatible with the diagnosis in 8, but changed the diagnosis as follows in the other 8: myocarditis, 4; hypertrophic cardiomyopathy, 2 and carnitine deficiency syndromes, 2. In two patients with the clinical diagnosis of hypertrophic cardiomyopathy, biopsy findings confirmed the diagnosis in one and were normal in the other who had an encapsulated cardiac fibroma at operation. In two patients with the clinical diagnosis of myocarditis, biopsy findings confirmed the diagnosis in one and suggested dilated cardiomyopathy in the other. In the entire series, 25% had biopsy evidence of inflammatory disease. Biopsy findings were confirmed at subsequent autopsy in five cases.

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The "buried penis" occurs in a spectrum which varies from slight with only ventral webbing to almost complete burying of the penis dorsally in the prepubic fat pad and ventrally in the scrotum. From 1979 to 1985, the author operated on 80 children with these problems. Indications for surgery varied from unsatisfactory appearance of the penis before or after circumcision to almost complete concealment of the penis. Satisfactory cosmetic and functional results were achieved in most cases utilizing several surgical principles. The limitations of these procedures and their possible complications are described.

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From January 1983 to December 1985, 14 procedures were performed in which a free graft of skin from either the foreskin or elsewhere was substituted for the flipped flap in the repair of hypospadias. This obviates the problem of thin skin below the urethral meatus, since a flipped flap in such a case will result in devascularization of the flap. Moderate chordee was not necessarily a contraindication to the use of this procedure, provided the chordee could be corrected without moving the meatus proximally. Cosmetic and functional results have been good, and there was only 1 urethral fistula. Nevertheless, because of the need for a scrotal flap of fat to prevent fistula formation and to ensure vascularization of the graft, and because of the availability of a vascularized flap from the foreskin in most cases, the free graft patch procedure is best reserved for cases in which the foreskin is either absent or deficient.

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Anomalous origin of the left coronary artery from the pulmonary artery is associated with myocardial infarction, left ventricular dysfunction, mitral valve dysfunction and, occasionally, intracardiac congenital abnormalities. A technique that utilizes a flap of the anterior wall of the pulmonary artery to serve as a neocoronary artery to direct aortic flow from a created aortopulmonary window to the pulmonary artery orifice of the anomalous left coronary artery was used in five patients aged 2.5 months to 4.75 years. Two patients were less than 4 months of age at operation. There was one death 2 days after operation and one late death. The two youngest patients required mitral valve replacement. Two of the three surviving patients are well at follow-up at 7 to 44 months. One patient has been lost to follow-up study. One patient had postoperative catheterization which showed an intact repair. The pulmonary artery neocoronary procedure is applicable to infants and small patients with anomalous origin of the left coronary artery from the pulmonary artery.

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Successful surgical repair of a 4-year-old boy with situs inversus [I, D, D] and corrected transposition of the great vessels (TGV) as well as hemodynamically significant ventricular septal defect (VSD), systemic atrioventricular (tricuspid) valve regurgitation, and atrial septal defect is described. Unique technical aspects of VSD and tricuspid valve repair in association with corrected TGV and situs solitus or inversus are discussed and clarified.

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Any surgeon performing hypospadias repair must be prepared to manage the surgical complications. From January 1976 to December 1982, 176 cases of hypospadias treated with a number of different operations were critically analyzed for complications. There were no complications in 143 cases (81 per cent). However, there were 44 surgical complications in the remaining 33 patients (19 per cent), which included stricture formation (11), urethral fistula (11), severe skin edema (2), subcutaneous hematoma (2), persistent chordee (2), scrotal abscess (1) and bladder calculus (1). Treatment of the various complications is discussed along with suggestions on how to avoid them. When only 3 operations are considered (meatal advancement and glanuloplasty, flip-flap procedure and free graft tubed urethroplasty), surgical repair of all types of hypospadias was accomplished, without any complications in 89 per cent of the cases. The results of hypospadias repair have improved because of the availability of these more reliable procedures that provide a lower incidence of complications and improved cosmetic appearance.

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During a 28-month period, consecutive 2-dimensional echocardiograms were reviewed to determine the prevalence of left ventricular (LV) false tendons, their associated anomalies and clinical significance. LV false tendons were found in 31 (0.8%) of 3,847 consecutive 2-dimensional echocardiograms. Of 31 LV false tendons, 30 passed longitudinally from papillary muscle to septum and 1 went from free wall to free wall. The 31 patients were aged 1 day to 15 years. Associated heart disease, most often ventricular septal defect, bicuspid aortic valve and coarctation of the aorta, was present in 48%, of whom 73% were girls. Of those without heart disease, 69% were boys. In patients with heart disease, precordial murmurs were due to the underlying cardiac anomaly. Of those without heart disease, 15 of 16 (94%) had a precordial murmur, usually of the Still's type over the lower left sternal border. Four of 31 (13%), 1 with and 3 without heart disease, had unifocal premature ventricular contractions that were rate-dependent in the 2 patients undergoing stress testing. LV false tendons appear to occur in 0.8% of pediatric patients and usually are accompanied by a Still's type innocent murmur if unassociated with heart disease. Some LV false tendons are associated with rate-dependent premature ventricular contractions.

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A left ventricular, mass-like echocardiographic density (a pseudotumor), present only during diastole and not attached to any intracardiac structure, was present in 77 of 1,820 consecutive 2-dimensional echocardiograms performed with a rotating-head transducer. Evaluation of the patients with a pseudotumor demonstrated that aortic insufficiency of mild to moderate degree was the most commonly associated lesion. Of the 61 patients with aortic insufficiency in our series, 69% had a left ventricular pseudotumor. Of the cases with a pseudotumor and aortic insufficiency, none had severe aortic insufficiency. Of the 1,759 patients without aortic insufficiency, a left ventricular pseudotumor was present in 2%. In our series, the sensitivity of left ventricular pseudotumor for aortic insufficiency was 69% and the specificity was 98%.

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Balloon atrial septostomy is an accepted method for palliation of certain types of congenital heart disease. However, malposition of the balloon may lead to cardiac perforation, avulsion of an atrioventricular valve, or laceration of the systemic or pulmonary veins. Inasmuch as single-phase fluoroscopy may not identify balloon position correctly and as biplane fluoroscopy adds significant radiation exposure, two-dimensional echocardiography has been used to assist in balloon atrial septostomy in ten infants. The catheter is advanced from the inferior vena cava to the right atrium across the foramen ovale to the left atrium with the echo transducer in the subxiphoid position. The balloon is inflated and its position within the left atrium is confirmed by echo. The catheter is withdrawn according to the technique of Rashkind. Withdrawal is halted when the balloon traverses the atrial septum. Adequate septostomy is indicated on echo by a defect at least 5 mm in diameter and by flapping of the inferior rim of the atrial septum. There were no complications using this technique and a clinically adequate septostomy was achieved in each patient. Two-dimensional echocardiography-assisted balloon atrial septostomy minimizes risk of complications and decreases exposure to ionizing radiation.

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Two infants, 101/2 and 11 1/2 months of age, underwent mitral valve replacement with Ionescu-Shiley bovine bioprosthesis for congenital cardiac defects. Both patients had early valve failure, 19 months and 4 months after implantation, due to the growth of excessive collagen on the ventricular surface of the bioprosthesis with adherence of the collagen primarily to the sewing rings and struts of the valves. The cause of this excessive collagen reaction is unknown. Further study is needed to document the true incidence of this problem.

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Two-dimensional echocardiography (2DE) was performed on 13 bioprosthetic mitral valves in 11 pediatric patients, 1 week to 46 months following implantation. Cardiac catheterization confirmed the echocardiographic findings of seven valves. The 11 normally functional bioprosthetic mitral valves by 2DE had leaflet thickness not exceeding 2.0 mm, freely moving leaflets, and normal sewing ring motion. The left atrium (LA) was enlarged only in the presence of mitral valve obstruction, atrial fibrillation, or left ventricular (LV) dysfunction. One bovine valve was stenotic due to fibrous tissue beneath normal valve leaflets; 2DE demonstrated limited leaflet motion, tissue beneath a thin valve leaflet, increase rocking motion of the sewing ring, and LA enlargement. In another patient, a bovine valve produced subaortic LV obstruction due to growth of fibrous tissue outside the stent; 2DE showed that the valve leaflets were thin and moved normally without excessive rocking of the valve ring, and identified the abnormal fibrous tissue in the LV outflow tract.

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This paper presents our experience with two-dimensional echocardiography in the detection of 61 intravascular catheters or wires in a series of 39 infants and children. It was possible to identify intravascular wires as small as 0.5 mm in diameter and intravascular catheters as small as 0.9 mm in diameter. In no instance, was an intravascular catheter not detected. The two-dimensional echocardiogram is demonstrated to be a tool to assist the physician in placement of intravascular catheters. In the catheterization laboratory, the two-dimensional echocardiogram is useful in determining catheter location, particularly in the presence of complex congenital defects. Two-dimensional echocardiographic guidance of catheters minimizes exposure to ionizing radiation.

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