To determine if symptoms and electrocardiographic abnormalities relate to left ventricular mass, volume, and mass/volume ratio in children with aortic stenosis and/or insufficiency, we examined 42 patients (aged 11±6 years) with cross-sectional and Doppler echocardiography. Clinical symptoms included exertional chest pain, shortness of breath, exercise intolerance, congestive heart failure and syncope. Electrocardiographic abnormalities were defined as the presence of both left ventricular hypertrophy and ST-T wave changes. Left ventricular volumes and mass were measured from echocardiograms using paired orthogonal apical views and biplane Simpson's and truncated ellipsoid models, respectively. The peak and mean pressure gradients across the aortic valve, the aortic valvar area, and the degree of aortic insufficiency were determined using echo/Doppler techniques. Eighteen patients (ages 10±7 years) had pure aortic stenosis (peak gradient 52±29 mm Hg, mean gradient 28±18 mm Hg, and valve area 0.86±.46 cm2/m2). Of these 18, seven had symptoms and seven had an abnormal electrocardiogram. Twenty-four patients (aged 12±6 years) had aortic stenosis and aortic insufficiency (peak gradient 36±18 mm Hg, mean gradient 19±9 mm Hg, 18 with 1−2+ aortic insufficiency and six with 3−4+ aortic insufficiency). Of these 24, 10 had symptoms and 10 had an abnormal electrocardiogram. When symptomatic and asymptomatic patients were compared using an unpaired Student's t-test, symptomatic patients with pure aortic stenosis and combined aortic stenosis and insufficiency had left ventricular hypertrophy and inappropriately increased mass/volume ratios. However, due to differences in left ventricular volume, symptoms and electrocardiographic abnormalities occurred at a higher mass/ volume ratio in pure aortic stenosis (2.7±0.6) than in combined aortic stenosis and insufficiency (1.7±0.3).

We evaluated the modulation of the viability of immature cardiac myocytes by cardiac fibroblasts after hypothermic preservation using three types of storage solutions—saline, University of Wisconsin solution, and MCDB 107 medium. Cardiac myocytes and fibroblasts were isolated from neonatal rat ventricles, and cultures of myocytes only or co-cultures with fibroblasts (myocyte: fibroblast 2:1) were established. On the fourth day of culture, the cultures were incubated at 4 °C for 6, 12, 18 and 24 hours in the different storage solutions. Enzymes were measured in the storage solutions immediately before and after hypothermic incubation. The cultures were then incubated for an additional 24 hours at 37 °C to evaluate the recovery of the myocyte beating rate. The myocyte beating rate in the co-culture groups showed significantly higher recovery ratios than the corresponding groups in which only myocytes were cultured. Complete recovery was observed in the group co-cultured in MCDB medium 24 hours after hypothermic incubation (83.4% of control—beating rate prior to hypothermic incubation) compared to the other co-cultured groups (15.4, 0%, respectively). Release of enzymes in the co-cultures was significantly suppressed compared to the cultured myocytes, and the greatest suppression was found after 24 hours of incubation in MCDB medium (CPK: 36.6 mIU/flask, LDH: 281.2 mIU/flask) compared to the other two co-cultured groups (CPK: 181.1, 281.1; LDH: 501.7, 773.2). Cardiac fibroblasts diminished myocytic injury after hypothermic preservation using various storage solutions, in which MCDB 107 medium showed the best overall protective effect. Thus, cardiac fibroblasts may play an important role in controlling myocytic viability under various hypothermic conditions.

The purpose of our study was to assess the effect of administration of erythropoietin on postoperative anemia in patients with congenital heart disease who had undergone open heart surgery without the use of homologous blood. One intravenous and one subcutaneous dose of 300 units of erythropoietin per kg body weight was given to 10 patients at a mean of five (3–8) days after surgery. Another group of 10 patients, who had undergone open heart surgery but were not given erythropoietin postoperatively, were used as controls. There was a significant fall in all patients in hemoglobin from 13.6±1.3 gm/dl prior to surgery to 9.8±1.6 gm/dl after surgery. In patients treated with erythropoietin, there was an immediate significant rise in hemoglobin and hematocrit. Hemoglobin rose by 14% on the seventh day after treatment (the twelfth day after surgery) and by 25% on the tenth day after treatment (the fifteenth day after surgery). It had increased by only 8% 15 days after surgery in the controls. These findings suggest a beneficial effect of administration of recombinant erythropoietin on postoperative anemia in children who had undergone open heart surgery without the use of homologous blood.

A new surgical technique is described to reduce the flow through acquired systemic-pulmonary collateral arteries, a complication which has been recognized as a risk factor in patients undergoing the Fontan operation. When systemic-pulmonary collateral arteries have developed subsequent to previous construction of an ipsilateral Blalock-Taussig shunt or a Glenn anastomosis, and are identified by angiography in advance of the Fontan operation, the technique is indicated as a preparative procedure. It consists of rethoracotomy aimed at isolating the lung from the thoracic wall (peeling), and wrapping of the upper half of the lung with expanded polytetrafluoroethylene sheets to avoid redevelopment of collateral vessels into the lung (wrapping). This decreases the systemic-collateral flow through small vessels which cannot be handled by conventional embolization using catheterization techniques, and permits the Fontan operation to be performed more safely.

The purpose of this study was to determine the cause, onset, clinical outcome and treatment of aortic insufficiency developing during treatment of complex cardiac anomalies. Aortic insufficiency associated with ventricular septal defect is a well-recognized entity. Very few studies, in contrast, have addressed the development of aortic insufficiency in the presence of more complex cardiac anomalies. Ten patients were selected from two clinical centers. All patients were diagnosed with a major cardiac anomaly requiring surgical treatment. Each patient developed aortic insufficiency requiring a surgical treatment of the valve after correction or palliation of their cardiac defect. There were six males, and mean age for the series was 17 years (range 5.5 to 33 years). All patients were initially operated for repair (eight patients) or palliation (two patients) of their congenital anomaly. Five patients had tetralogy of Fallot, one had tetralogy associated with pulmonary atresia, and two patients had double outlet right ventricle, and two had complete transposition. The degree of aortic insufficiency, the amount of pulmonary stenosis present before the original procedure and the diameter of the aortic root measured before the aortic surgical procedure on the valve were assessed by angiography and echocardiography. The mean time between the last surgical procedure and the appearance of aortic regurgitation was three years, with a mean time between the last intervention and the aortic procedure of 7.3 years. The etiology of the insufficiency was explained by prolapse of an aortic leaflet in three patients. Four patients had annular dilation with central regurgitation, two patients had traumatic perforation of a leaflet, one patient had lesions consistent with endocarditis. Eight patients had dilation of the aortic root. Surgical procedures included five replacements of the aortic valve, three aortic valvoplasties, one aortic valvar replacement associated with a Glenn shunt, and one Bentall procedure. One patient died two months following an aortic valvar replacement. All patients were assessed by transthoracic echocardiography before discharged. The two main causes of aortic regurgitation, leaflet prolapse and annular dilation are age-related complications. Most patients had dilation of the aortic root from longstanding increases in aortic flow. Early repair of congenital anomalies may decrease these complications.

The morphology of the aortic valve was studied in 1,022 heart specimens belonging to the collection of the Institute of Pathological Anatomy, University of Padua. Twenty specimens were found to have a unicommissural aortic valve, characterized by the presence of a single leaflet with only one functional commissure; however, the presence of two raphes enabled the recognition of a basically three-sinus arrangement. Age and gender were known in 19 cases: 14 male and five female, mean age nine days. In 19 cases, the unicommissural valve was dysplastic and resulted in a severe congenital aortic valvar stenosis. Only in two hearts was the unicommissural nature of the aortic valve an isolated finding; among the remaining 18, left ventricular fibroelastosis in 11, malformation of the mitral valve in 11, hypoplasia of the left ventricle in eight, ventricular septal defect in four, mitral atresia in three, and subaortic fibrous diaphragm in one. Furthermore, seven (35%) of these 20 unicommissural aortic valves were associated with coarctation of the aorta. Statistical analysis shows that this association is not a random event. Our findings support the hypothesis that the unicommissural aortic valve originates from the early fusion of the three mesenchymal valvar cushions or leaflet primordia. Although the present data do not exclude the possibility that reduction of the blood flow through the aorta during fetal life may play a role in the formation of the unicommissural aortic valve, they rather point in the direction that another etiologic factor, such as an anomalous migration of neural crest cells, may be responsible for the fusion of the valvar cushions.

The records of nine patients with double-chambered right ventricle, ventricular septal defect and subaortic narrowing were reviewed to clarify the natural history of the components of this pathological combination. All nine patients presented with heart failure related to a perimembranous ventricular septal defect, which underwent spontaneous reduction in eight and closed in one. Echocardiographic diagnoses of double-chambered right ventricle and subaortic narrowing were delayed in eight of the nine cases. The ratio of the distances from the pulmonary valve to moderator band/tricuspid valve annulus (the displacement index) was less than one in all patients. Subaortic narrowing was always discrete and was present at the diagnosis of double-chambered right ventricle. The subaortic narrowing was always recognized echocardiographically, and was seen in eight of nine patients at angiography. The subaortic gradient was 0–20 mm Hg and the subpulmonary gradient 0–110 mm Hg at catheterization. Five patients demonstrated aortic regurgitation. Subaortic narrowing should be diligently sought among patients with double-chambered right ventricle, and vice versa, even in the absence of a ventricular septal defect which may have closed. Both the subaortic narrowing and subpulmonary obstruction may progress, but probably at different rates. Patients with a perimembranous ventricular septal defect, particularly with a displacement index of less than one, should be monitored carefully for evolving subpulmonary obstruction, subaortic narrowing and aortic regurgitation. The subpulmonary obstruction should be resected at the time of any other surgical intervention. The indications for resection of the subaortic narrowing remain ill-defined.

The role of cross-sectional echocardiography is demonstrated in defining the anatomy of the coronary arteries in 29 patients with complete transposition (concordant atrioventricular and discordant ventriculoarterial connections). The study revealed a relationship between some of the less frequent patterns and the spatial relationship of the arterial trunks, along with the presence or absence of a ventricular septal defect. It suggests that a side-by-side relationship of the arterial trunks due to infundibular maldevelopment may be related to some of the less common patterns.

Balloon dilation of the aortic valve was performed in 20 consecutive neonates with critical aortic stenosis using an approach achieved by cutting down on the right carotid artery. The age of the patients ranged from one to 25 days (mean seven days) and their weight from 2.1 to 4.0 kg (mean 3.16 kg). All patients were evaluated before cardiac catheterization with cross-sectional and Doppler echocardiography so as to keep the catheterization procedure as short as possible. Balloon dilation was accomplished in all patients. The only complication was an apical perforation by the guide wire in two cases. The ensuing pericardial effusion was immediately drained with pericardiocentesis and the subsequent course of the procedure was uneventful. Immediate results showed dramatic improvement in cardiovascular conditions. The transvalvar pressure gradient fell from 80±40 to 27±20 mm Hg (p<0.001). Left ventricular ejection fraction evaluated by echocardiography increased from 30±21% before dilation to 54±18% 24-48 hours after the procedure (p<0.001). In all patients, the procedure was free from vascular complications. Aortic regurgitation was documented after the procedure in 11 patients, being severe in one, moderate in five and trivial in five. Seven patients died, although in only one was the death related directly to the procedure itself. Six patients died because of associated lesions despite an immediate satisfactory result of the balloon valvoplasty. The 13 surviving patients are doing well, and are receiving no medications. During a mean follow-up of 25 months (range 2-54 months), four patients have developed restenosis. One underwent surgical valvotomy at one year of age. The second was successfully redilated through the same approach at two months of age. The other two have a significant gradient, as assessed by Doppler measurements (60 and 70 mm Hg), with normal systolic ventricular function. Two patients have moderate aortic regurgitation. Balloon dilation achieved through cutdown on the right carotid artery is a safe and effective alternative to surgery in neonates with isolated aortic stenosis. The unfavorable results are mainly due to associated anomalies.

The management of pulmonary atresia with intact ventricular septum remains controversial. The plan for management at our institution is based on the echocardiographic identification of a well-developed infundibulum. Neonates with such a well-developed infundibulum are prepared for a biventricular repair, whereas those without an infundibulum are groomed for a Fontan operation. In all, 48 neonates with the lesion were admitted to our unit between 1980 and 1992. In 31 neonates with a well-formed infundibulum, the initial palliation consisted mainly of pulmonary valvotomy without cardiopulmonary bypass and construction of a polytetrafluoroethylene shunt from the left subclavian artery to the pulmonary trunk. In most of these patients, growth of the right ventricle could be promoted by establishing antegrade flow through the ventricle. In seven patients in this group, nonetheless, the right ventricle failed to grow satisfactorily. In this subgroup, additional intermediate procedures were performed on the apical component and infundibulum of the ventricle together with the tricuspid and pulmonary valves which could collectively be termed as “right ventricular overhaul.” Five of these patients have gone on to further successful biventricular repairs at a median interval of 24 months after the overhaul. There was no operative mortality in this group. Following the final biventricular repair, there have been no late deaths or reoperations over a total follow-up of 145.4 patient months (mean 22.2 months).

We studied 52 children in order to assess the prevalence, natural history, and electrophysiologic effects of cardiac rhabdomyoma in tuberous sclerosis. Their ages ranged from one day to 12 years (median age 1.33 year). The tumors in the heart were found in 22 patients through cross-sectional echocardiography. The overall prevalence of cardiac rhabdomyoma was 42%. The prevalence of cardiac tumors in patients first seen in infancy was 77%, and that in patients over one year of age was 31%. Those with cardiac tumors diagnosed in infancy had more tumors per person than did the patients diagnosed after infancy, and the tumors were smaller in the patients diagnosed over one year of age. Follow-up cross-sectional echocardiography showed that almost all tumors showed spontaneous regression. Complete regression occurred more commonly in younger patients, and in those with smaller tumors. About 70% of patients with cardiac tumors had either normal findings or infrequent premature contractions on 24hour Holter monitorings. The rest of the patients with cardiac tumors showed either frequent premature contractions, atrioventricular block, or atrial tachycardia, but antiarrhythmic medication was rarely required. Cross-sectional echocardiography, therefore, seems to be the best diagnostic tool for screening cardiac rhabdomyomas in patients with tuberous sclerosis. The tumors may be managed more conservatively in view of their high rate of spontaneous regression and rather rare association with symptomatic arrhythmias.

We analyzed tricuspid insufficiency noninvasively using cross-sectional and continuous wave Doppler echocardiography in 49 newborn infants free of structural cardiac and pulmonary disease. In 41(83.6%) of the cases, mild insufficiency was detected using the continuous wave Doppler technique. All the infants with valvar insufficiency had normal electrocardiographic tracings and, in 25 (61%) of them, there was no audible murmur. We detected transient ductal patency in five (12.1%). The ratio of right ventricular preejection period to ventricular ejection time was in the normal ranges (below 0.05) in 39 (95.1%) of the neonates. Of the 30 cases who underwent continued surveillance over a mean period of 37.9 days, the incompetence disappeared in 15 (50%). Our findings show that, in neonates, the lack of a murmur does not eliminate the possibility of neonatal tricuspid incompetence. Furthermore, abnormalities such as postnatal transient pulmonary hypertension, fetal closure of the arterial duct and perinatal asphyxia cannot readily explain the finding of tricuspid incompetence.

To check a possible mechanism for residual patency of arterial ducts closed with umbrellas, we studied retrospectively all 25 implantations of the Rashkind device carried out in our institution. In all instances, the smaller umbrella had been used. Residual patency occurred in 40%. There was a significant correlation (p<0.001) between the way the umbrella was rotated and the rate of residual patency. In the larger subgroup, the residual patency was 75% when two arms were pointing towards the aortic blood stream. In contrast, when only one arm pointed upwards, there was only one patient (8.3%) with residual shunt. This potential precipitating mechanism should be considered when implanting the Rashkind devices in future.

We present a case of anomalous origin of the left pulmonary artery from a left-sided brachiocephalic artery via an arterial duct in a patient with a right aortic arch. Associated intracardiac anomalies were a large perimembranous ventricular septal defect and the persistence of a small vertical vein, connecting the pulmonary veins normally incorporated into the left atrium to the brachiocephalic vein. Spontaneous closure of the arterial duct occurred in the first days of life and was responsible for the circulatory “isolation” of the left pulmonary artery. The clinical course, surgical strategy and possible embryological explanation are described.

We describe a two-year-old boy suffering from pulmonary hypertension due to pulmonary venous stenosis. Previous reports suggest that it is not easy to diagnose this entity during life, and most cases are diagnosed at operation or autopsy. Our patient was diagnosed relatively early in life and treated successfully by pericardial patch venoplasty. Histologic examination of the lung biopsy performed at operation was considered to be useftil prognostically. The minimal pulmonary vascular changes noted were consistent with a good outcome after the surgical repair.

Isolated noncompaction of ventricular myocardium is a rare cardiomyopathy with persistence of a myocardial architecture such as seen early in fetal development with numerous prominent trabeculations and deep intertrabecular spaces. Symptoms are related to cardiac failure, arterial embolism, and severe arrhythmias. Familial recurrence has been described. We describe a case where isolated noncompaction of ventricular myocardium could be recognized on an ultrasound study at 23 weeks gestation, thus supporting the developmental hypothesis.

We describe a neonate who presented with aortic atresia, a hypoplastic left ventricle and aorto-left ventricular tunnel. These findings, recognized by transthoracic and transesophageal cross-sectional echocardiography complemented by Doppler color flow imaging, were confirmed at surgery by direct inspection. This complex of associations has not been described before and suggests a primary abnormality of the left ventricle because diastolic filling from the aorto-left venticular tunnel and mitral flow should tend to ensure fetal left ventricular growth.

We report a patient with a mediastinal cyst compressing the left atrium that was diagnosed prenatally by fetal echocardiography. Postnatally, attempted aspiration guided by computerized tomography was unsuccessful. Because of respiratory distress, the cyst was removed surgically. Pathological examination revealed a bronchogenic cyst.

We describe a three-year-old girl in whom arthritis developed ten days after the onset of illness, followed by clinical features consistent with rheumatic carditis and pneumonia. Treatment with prednisone proved satisfactory. This case illustrates the persistence of patterns of rheumatic fever in northeast Brazil which are most unusual in developed countries

while preferential blood flow to the right pulmonary artery has been described in transposition of the great arteries with or without obstruction of the left ventricular outflow tract, this disparity of pulmonary blood flow is not present in newborns.1 We report a newborn with transposition in whom there was discontinuity of the pulmonary arteries and ductal blood supply to the left pulmonary artery. To our knowledge, this entity has not been described in newborns with transposition of the great arteries {S,D,D}.