Current procedural terminology codes generally do not distinguish between patients based on age or etiology of disease. As standardized payment schedules based upon such codes are being developed, the precise definition of a “typical” patient for each code becomes increasingly important. Since small size, young age, or presence of complex congenital disease could potentially render the work value of a physician's service different between children and adults, a study was jointly undertaken by the American College of Cardiology and the Cardiology Section of the American Academy of Pediatrics to compare relative work for the most commonly used Current Procedural Terminology codes in pediatric cardiology. A Technical Advisory Panel chose codes for evaluation and wrote clinical descriptors for each service. A separate rating panel of eight pediatric cardiologists (academic and private practice) and three adult cardiologists participated in a mail survey assigning work values to each service relative to current Health Care Financing Administration adult relative value units. The Panel then met and employed a modified Delphi process to arrive at consensus values. Of the 20 codes rated, only five were determined to be similar to adults. The pediatric median values were an average of 44% higher than the adult values (general cardiology codes 8% higher, echocardiography 90%, catheterization 49%, electrophysiology 35%). Thus, work values for cardiology were found to be different between children and adults in 75% of services assessed. The magnitude of these differences should be confirmed in a broader study. The Current Procedural Terminology Committee of the American Medical Association should consider, at a minimum, creating modifiers by specific age for certain procedures. Further study of the quantitative difference between services performed in adults and children is warranted.

Three children with pulmonary atresia and ventricular septal defect with absent central pulmonary arteries underwent successful complete correction following preliminary staging procedures for unifocalization and deletion of multiple major aortopulmonary collateral arteries. The unifocalization procedures were accomplished using valveless pulmonary arterial cryopreserved allografts. This technique was found to facilitate and simplify the final repair. All patients survived the complete correction. The patients have been followed for 1½-4 years following the final surgery. One child died two years after repair from pneumonia complicated by asthma. One patient appears to have progression of pulmonary vascular disease despite the complete repair, and one patient continues to do well both symptomatically and hemodynamically. The results suggest that this method of unifocalization solves many of the problems inherent to more classical techniques, but the overall prognosis of patients in this diagnostic entity has yet to be completely defined.

Replacement of the mitral valve was performed in 130 children less than 15 years of age—115 for rheumatic and 15 for congenital disease. Early mortality was 5.4%. Of 123 survivors, 112 were followed postoperatively for a mean period of nine years, with 80 of them on anticoagulants. Thromboembolism is the major late problem, with 25 thromboembolic accidents noted in 25 patients (22.3%). Anticoagulation was ineffective or lacking in 72% of them. Bacterial endocarditis was noted in five cases (4.4%) and a prosthetic leak in three (2.6%). Particular problems are observed in this group of young patients. First, recurrence of rheumatic fever in nine patients with an increasing severity of aortic valvar incompetence; second, with growth the prosthesis can become small as observed in six of our patients. A reoperation was needed in 15 patients, with one requiring two reoperations. Late mortality was 21%. Optimal timing for replacement of the valve is crucial. Appropriate anticoagulation and correct prophylaxis of rheumatic fever and bacterial endocarditis is mandatory. Conservative surgery is certainly preferable to valvar replacement, but if replacement proves necessary, large prostheses should be used.

Long-term left atrioventricular valvar function was evaluated in 95 of 110 survivors following surgical repair of atrioventricular septal defect between 1975 and 1984. A common or complete form was present in 40 and a partitioned or partial form in 55 patients. The patients have been followed for three to 13 years with a mean of 8.3 years. Pulmonary arterial banding was performed in 17 patients with a common atrioventricular valve prior to complete repair. The left atrioventricular valvar regurgitation was evaluated by clinical examination, Doppler and/or angiography. Three patients required valvar replacement postoperatively. Previous pulmonary arterial banding, pulmonary hypertension or pulmonary-to-systemic flow ratio did not affect the incidence or severity of left atrioventricular valvar regurgitation postoperatively. In this series left atrioventricular valvar regurgitation increased in the early postoperative period but rarely progressed at late follow-up.

Stenoses of the systemic venous pathways are a potentially dangerous complication after the Mustard operation. The acute and intermediate-term efficacy of balloon dilation of these stenoses has not previously been studied in a large group of patients. A retrospective analysis of clinical and angiographic data, with prospective clinical and echocardiographic follow-up, was performed in a consecutive group of 25 patients (17 male, 8 female) who underwent balloon dilation of the superior or inferior caval venous pathways. For 17 dilations of stenoses in the superior pathway, the mean pressure gradient decreased from 9.1±5.7 to 3.4±2.3 mm Hg (p<0.01), while the diameter of the stenosis increased from 5.9±2.9 to 11.2±3.0 mm (p<0.01). For 25 dilations of the inferior pathway, the mean gradient decreased from 4.3±1.8 to 1.4±1.1 mm Hg (p<0.01), while the diameter of the stenosis increased from 7.3±1.5 to 10.6±2.3 mm (p<0.01). Over a median follow-up of 3.5 years, 16 patients have been without symptoms or signs suggestive of recurrence of stenoses. In the remaining nine patients, 11 further procedures were performed at a median of 3.1 years after initial dilation. In five patients who had previously undergone dilation of the superior (3), inferior (1) or both (1) pathways, further dilations of the same pathway(s) and/or stent implantations were performed. Three patients, including one in whom superior caval venous stenosis had recurred, had developed a new stenosis of a previously undilated pathway, while in two patients no further dilations were required. Balloon dilation offers an alternative to reoperation for stenoses of the systemic venous pathways after the Mustard operation, and appears to provide satisfactory intermediate-term palliation. Recurrences of stenoses in previously dilated pathways, and new stenoses in previously unobstructed pathways, are commonly seen at follow-up. Alternative measures, such as placement of stents, need to be considered in the long-term management of these patients.

The purpose of this study was to evaluate the functional and biochemical effects of cardioplegic solutions on immature cardiac myocytes incubated under hypothermic conditions. In addition, the effects of insulin as an additive were evaluated in each solution. Cardiac myocytes were isolated from neonatal rat ventricles and cultured for four days; 12.5 x 105 myocytes/flask were then incubated at 4 °C for three, six and 12 hours in three types of cardioplegic solutions—glucose-potassium solution (glucose: 50 gm/l, NaHCO3: 20 mEq, KCl: 20 mEq), lactated Ringer's solution (KCl: 20 mEq) and St. Thomas' Hospital solution. After each hypothermic incubation, enzymes were measured in the incubation solutions. The myocytes were then cultured for an additional 24 hours at 37 °C to evaluate the recovery of the myocyte beating rate. In the Ringer's group, the recovery ratio of the myocyte beating rate was complete at three hours, then decreased to 48.8 percent of control (beating rate prior to hypothermic incubation) at 12 hours. The glucose-potassium and St. Thomas' groups had significantly lower recovery ratios than the Ringer's group, beginning at three hours (63.4, 72.9, 95.6 percent, respectively). Release of enzymes (CPK and LDH) in the Ringer's group gradually increased and at 12 hours was 29.0 mIU/flask and 260.0 mIU/flask, respectively. The St. Thomas' group, in contrast, had significantly increased values for CPK at 12 hours to 116.0 mIU/flask, and the greatest increases of both enzymes were observed in the glucose-potassium group at 12 hours (CPK: 115.5, LDH: 1163.9). By addition of 20 IU/l insulin, marked improvements were observed in the Ringer's and glucose-potassium groups both functionally and biochemically. Thus, the lactated Ringer's solution had the least cytotoxic effects that might be suitable for a basic solution of various cardioplegic solutions during the neonatal period, and insulin may have beneficial effects on immature myocardium under hypothermic conditions.

Maternal hyperthermia induces malformations in human and laboratory animals, but there are only a few reports of cardiovascular anomalies induced by maternal hyperthermia. Pregnant Wistar Imamichi rats were exposed to hyperthermia on day nine of gestation by immersion in a hot-water bath (43.0–43.5 °C) twice a day. Their fetuses were examined on day 18 after fixing with 2.5% glutaraldehyde solution. According to the average body temperature of the dam, the fetuses were divided into five groups, each group increasing by 0.1 °C from 42.5 °C to 42.9 °C. The incidence of cardiovascular anomalies was increased from 24 to 100% with the rise in body temperature of the dams. In the groups with average temperatures of 42.8 and 42.9 °C, most of the fetuses (70–80% of cases with anomalies) showed visceroatrial heterotaxy associated with severe cardiovascular anomalies, including common atrioventricular orifices with double outlet right ventricle or discordant ventriculo-arterial connections. The visceral structures of those fetuses showed a tendency to left isomerism, such as bilateral left atrial appendages, single lobation of the lung on both sides, a symmetrical liver with interruption of the posterior caval vein, hypoplasia of the spleen, and malrotation of the intestinal loop. Day nine of gestation in the rat may be a critical period for determining left-right sidedness of the viscera. The results indicate that the determination of morphological left-sidedness of visceral structures is related in some way with maternal hyperthermia.

Microdissection of the oval fossa from its right and left atrial aspects showed that the rim of the fossa was formed from an infolding of the atrial wall, which began between the base of the superior caval vein and the insertion of the right pulmonary vein to the left atrium. Because the rim varied in shape from circular to oval, we used morphometric methods to obtain patterns of normality of the diameters of the oval fossa, oval foramen, and the area of right and left atrial aspects of the septum. These values were found to correlate with log fetal and neonatal weight. Due to technical advances in prenatal diagnosis, these structures can now be examined in fetal life. Therefore, our morphometric data, when appropriately interpreted, have immediate clinical and surgical applications in the treatment of fetal and neonatal cardiovascular disorders.

The aim of this study was to clarify those morphological features and hemodynamic stress factors which influence the electrocardiographic findings in atrioventricular septal defect. In 64 patients with the incomplete form of atrioventricular septal defect (separate valvar orifices) with usual atrial arrangement, the length from the left ventricular apex to the aortic valve (outlet dimension), to the so-called cleft (scoop dimension), and to the lowest point of the left atrioventricular valve (inlet dimension) were measured by biplanar cineangiography. The size of the ostium primum defect, the ventricular pressure ratio, and the pulmonary/systemic flow ratio were also evaluated. The results showed that the lower the scoop/outlet ratio (r=0.60, p<0.001), or the higher the ventricular pressure ratio (r= −0.55, p<0.001), the more deviated to the left and superior was the mean frontal QRS axis. These factors also had significant correlation with the QRS patterns in the Frank vectorcardiogram. We conclude that the scoop/outlet ratio, supposedly reflecting the degree of scooping of the interventricular septum, is one of the most significant factors which influence the electrocardiographic findings in atrioventricular septal defect.

Double orifice left atrioventricular valve is an uncommon condition. This paper describes 10 consecutive patients with the anomaly encountered over 20 months. Diagnosis was assisted by cross-sectional echocardiography and supplementary color Doppler, often requiring non-standard views and a high index of suspicion. There were three anatomical variants—those in which the orifices were equal in size (three of 10 patients), those in which they were unequal and associated with an atrioventricular septal defect (four of 10 patients), and those in which they were unequal in the absence of an atrioventricular septal defect (three of 10 patients). The unequal orifice in both anatomical situations was frequently complicated by valvar regurgitation (six of seven patients). Conservative repair was possible in three of the four patients requiring surgery to the left atrioventricular valve. This condition should be thought of more often in the differential diagnosis of left atrioventricular regurgitation in childhood.

A case of a complete atrioventricular septal defect was detected in a 20-week gestation fetus, in whom the ventricular component of the defect was subsequently noted to close spontaneously with advancing gestational age. The antenatal and postnatal findings are discussed.

A congenital defect of the right coronary leaflet (fenestration) is an unusually rare cause for aortic incompetence in childhood. We report an eight-year-old child with such an anomaly who presented with infective endocarditis of the left ventricle. The importance of excision of the vegetation prior to the occurrence of life-threatening embolic complications is discussed.

We present a patient with primary pulmonary hypertension who had unusually high pulmonary arterial pressure prior to double-lung transplantation. Obstruction of the right ventricular outflow tract developed after transplantation and progressed over the subsequent two years.

We report an unusual form of aortic atresia with a normal sized left ventricle, intact ventricular septum and isolated cleft of the left atrioventricular valve, with abnormal attachments creating both subaortic atresia and massive regurgitation across the left atrioventricular valve. This combination, never described before as far as we are aware, can be considered as an extreme form of subaortic stenosis complicating a very particular type of atrioventricular septal defect.

A seven-year-old patient with severe valvar pulmonary stenosis developed a mycotic aneurysm of the pulmonary trunk, which was followed closely with echocardiography. The aneurysm increased rapidly in size over a period of three weeks and required surgical removal.

We report a patient with tetralogy of Fallot and pulmonary atresia in whom the predominant extracardiac supply of arterial blood was via an aortopulmonary window. Major aortopulmonary collateral arteries originating from the descending thoracic aorta provided an additional source of pulmonary blood flow. The patient underwent successful correction and the postoperative recovery was uneventful.