Repair of anomalous origin of the left coronary artery in the infant and small child.

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.

Two-dimensional echocardiography for catheter location and placement in infants and children.

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.

Mitral valve replacement in infants and children.

Thirteen patients, ranging in age from 10 months to 19 years (mean 7.8 years) and in weight from 6.6 to 60 kg (average 29.5 kg) underwent 14 operations for mitral valve replacement with a heterograft prosthesis between January 1, 1976 and July 1, 1979 for a variety of congenital or acquired lesions. Preoperative indications included severe refractory congestive heart failure in each patient with growth retardation, which was especially prominent in the younger patients. Operative mortality was 14% (2/14) with both deaths occurring within 48 hours of operation in patients less than 6 years of age. All surgical survivors had clinical improvement as manifested by relief of symptoms, decrease in heart size, and significant growth. Routine postoperative catheterization in five patients revealed good initial postoperative results in those studied, with one patient having a second study 20 months after operation. He was found to have had degeneration of his bovine prosthesis and had subsequent successful reoperation with a porcine prosthesis. Further long-term serial catheterizations are needed to further document the history of heterograft prosthesis in children, but they are preferred to mechanical valves became of the lack of need for long-term anticoagulants and the absence of thromboembolism complications. This series suggests that mitral valve replacement, when indicated by refractory congestive heart failure and growth retardation, can be successfully performed even in infants and small children. Surgery should not be postponed to allow for subsequent patient growth if the natural history of the disease is of progression.

Echocardiographically assisted balloon atrial septostomy.

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.

Cerebrovascular abnormalities in postoperative coarctation of aorta. Four cases demonstrating left subclavian steal on aortography.

Fifteen children, aged 0.3 to 10.5 years (mean 3.8 years) after repair of coarctation of the aorta, underwent cerebral arteriography as part of postoperative catheterization. Four manifested central nervous system symptoms postoperative catheterization. Four manifested central nervous system symptoms postoperatively: Two had persistent headaches, one had exercise-induced hemianopsia and one had major motor seizures. All four had greater blood pressure in the right arm than in the left and evidence of subclavian steal, with retrograde filling of the left vertebral and left subclavian arteries on selective right vertebral arteriography. Ligation of the left vertebral artery in three patients and left subclavian graft arterioplasty in one resulted in disappearance of symptoms. None of the 11 asymptomatic patients manifested cerebrovascular anomalies, and no patient in the series had berry aneurysm. This study suggests that patients with central nervous system symptoms and a disparity of blood pressure in the arms after surgery for coarctation of the aorta should be evaluated carefully to exclude subclavian steal as the cause of the symptoms.

Isolated partial anomalous pulmonary venous drainage associated with pulmonary vascular obstructive disease.

Five patients with partial anomalous pulmonary venous drainage with intact atrial septum are described. In two patients, pulmonary arterial hypertension and pulmonary vascular disease developed. Both had one or more right pulmonary veins draining anomalously to the right superior vena cava. It is postulated that a combination of increased pulmonary blood flow and reflex pulmonary vascular changes contributes to the production of pulmonary vascular obstructive disease.

Anticoagulation therapy in children with mechanical prosthetic cardiac valves.

From 1980 through 1984, 28 children younger than 19 years (mean 7.9) underwent cardiac valve replacement with 30 mechanical prostheses. Patients were followed for a total of 471 months (mean 15.7) and received either warfarin (mean 0.16 mg/kg/day) or acetylsalicylic acid and dipyridamole (mean 6.1 and 1.9 mg/kg/day, respectively) as thromboembolism prophylaxis. The frequency and incidence of thromboembolism and hemorrhage were compared. Warfarin-treated patients were at increased risk of hemorrhage (5 of 20 [25%], or 22 per 100 patient-years, vs 0 of 10 [0%], or 0 per 100 patient-years, p less than 0.05). Three of the 5 hemorrhagic episodes were mild, and in no case was hemorrhage life-threatening. Patients who did not receive warfarin had a greater risk of thromboembolism (2 of 10 [20%], or 12 per 100 patient-years, vs 0 of 20 [0%], or 0 per 100 patient-years, p less than 0.05). Both episodes of thromboembolism were life-threatening and necessitated emergency valve replacement. Although warfarin is associated with greater risk of hemorrhage than is acetylsalicylic acid and dipyridamole, warfarin is better than antiplatelet drugs in thromboembolism prophylaxis and is indicated for anticoagulation therapy in children with mechanical cardiac prostheses.

Left ventricular false tendons in children: prevalence as detected by 2-dimensional echocardiography and clinical significance.

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.

Small left atrium and change in contour of the ventricular septum in total anomalous pulmonary venous connection: a morphometric analysis of 22 infant hearts.

Morphometric measurements of 22 hearts with total anomalous pulmonary venous connection (TAPVC) were compared with measurements of 8 matched control specimens without heart disease. Each of the TAPVC specimens had a shorter left atrium, smaller left atrial surface area and larger diameter of the fossa ovalis. In addition to increased length of the right ventricle and larger circumferences for tricuspid and pulmonary valve anuli, the left ventricular contour of the ventricular septum was flat or convex in 18 of the 22 hearts; the septum was significantly longer than normal in these specimens and wider at its midportion. Because mitral and aortic valve anuli were normal in circumference, the data suggest that left ventricular volume is not decreased despite change in ventricular shape.

Aortic valve atresia: a new classification based on necropsy study of 73 cases.

Certain clinical and morphologic observations are described in 73 necropsy patients with aortic valve atresia. The mean age at death was 5 days; 80 percent died during the first week of life, and 70 percent were boys. Of the 73 patients, 69 (95 percent) had a hypoplastic left ventricle with intact ventricular septum and either an atretic (25 patients) or hypoplastic (44 patients) mitral valve. The other four patients had a well developed left ventricle with one or more defects in the ventricular septum and either an atretic (one patient) or well developed (three patients) mitral valve. Review of previous reports on aortic valve atresia disclosed that a well developed left ventricle or ventricular septal defect in association with absence of the aortic valve was extremely rare. A new classification for aortic valve atresia is presented based on the status of the ventricular septum, which in turn appears to determine the size of the left ventricular cavity. The predilection for male subjects for all types of aortic valve disease, including atresia, is emphasized.

Costs and results of cardiac operations in infants less than 4 months old. Are they worthwhile?

From 1979 through 1983, 328 of 1,388 pediatric cardiac operations involved patients undergoing their first procedure at less than 4 months of age. Of these, 220 patients had 265 nonductal procedures, and their case histories are reviewed for results and total hospital cost. Initial operative mortality was 20% (43 patients). Infants with lower operative age and operative weight tended to have closed procedures. Mortality and cure were not related to gestational age, birth weight, age at operation, number of operations, or type of operation. Lower operative weight was associated with a greater mortality. Evaluated survivors (142 patients) were followed for a mean of 24 months. Fifteen percent (33 patients) died during follow-up. Of survivors, 80% (114 patients) had optimized general health; a subset of 29% had normal cardiac function, and 17% were cured. Lower birth weight was associated with curable lesions and normalcy (p less than 0.04). Longer preoperative hospital stay and lower weight at operation were associated with higher hospital cost (p less than 0.05). Hospital cost was not related to type of operation, gestational age, birth weight, age at operation, mortality, cure, or normalcy. Acquired neurologic dysfunction and long-term disability were uncommon. The mean hospital cost for surviving infants was +80,000 (1984 dollars). Effective hospital cost per survivor was +110,000. Mortality, cure, and normal function after cardiac operations in infants less than 4 months of age were not related to gestational age, birth weight, or age at operation. Mortality was higher in patients with a lower weight at operation. Separation into distinct fiscal cost groups is not reasonable in this series. Because most survivors are in normal or optimized cardiac health, intensive cardiovascular care in this population is justified.

Aortico-left ventricular tunnel. Clinical profile, diagnostic features, and surgical consideration.

Case histories of 2 patients with aortico-left ventricular tunnel are reported. A review of the literature on this subject shows a unique clinical profile. The electrocardiographic (ECG) and roentgenographic features as well as the hemodynamic and angiographic data of 18 previously reported patients are reviewed. The anatomy and pathophysiology of the defect are discussed and surgical management is described. It is recommended that surgical correction be carried out as soon as the diagnosis is established.

Early failures of Ionescu-Shiley bioprosthesis after mitral valve replacement in children.

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.

Surgical approach to corrected transposition of the great vessels and situs inversus [I, D, D] with ventricular septal defect and systemic atrioventricular valve regurgitation.

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.

Experience with repair of complete atrioventricular canal.

Twenty-nine patients at out institution have undergone repair of complete atrioventricular canal since 1969. There were 3 operative and 5 late deaths, 4 of which were of infectious etiology. Age at operation ranged from 2 months to 12 years (mean, 50 months). Weight ranged from 3.6 kg to 30 kg (mean, 12 kg). Before repair, catheterization studies revealed pulmonary hypertension in all patients with unobstructed pulmonary arteries. Pulmonary to systemic flow ratio ranged from 1.5 to 10.6 (mean, 3.5). Pulmonary vascular resistance (PVR) ranged from 0.7 to 21.7 (mean, 5.3) Wood units/m2. At repair, 14 patients had Rastelli type A anatomy, 14 had type C, and 1 patient had a variant with crossing chordae and double-outlet right ventricle (DORV). Postoperative catheterization has been done in 16 patients 2 months to 8 years (mean, 30 months) after repair. One patient had residual ventricular shunting and later underwent successful repair. A 2-year-old patient had severe mitral regurgitation and died following mitral valve replacement. One patient required a permanent pacemaker. The 21 surviving patients have been followed from 7 months to 10 years 7 months and have excellent hemodynamic status. Long-term studies are needed to assess the ultimate effect on patients with high PVR.

Subclavian flap aortoplasty for treatment of coarctation in early infancy.

In a 2-yr period 12 infants less than 2 mo of age underwent surgery for critical coarctation of the aorta. All had severe congestive failure with unsatisfactory response to medical therapy. In all patients the coarctation was located in the preductal or preligamentous region. Eleven patients had an associated ventricular septal defect (VSD). Early (1-mo) survival in the patients with VSD was 82%. Two early deaths occurred; there have been five late deaths on follow-up of up to 30 mo. In all patients who expired, the cause of death was unrelated to the coarctation repair. Recurrent coarctation has occurred in one patient. Early aggressive surgical intervention is indicated in the management of coarctation of the aorta both with and without VSD.