We report two female patients, one aged four years and the other a newborn, referred for evaluation of cyanosis with otherwise normal cardiovascular findings, who proved to have the right superior caval vein draining into the morphologically left atrium. In both patients, the diagnosis was made by color flow mapping. The older child underwent catheterization and subsequent successful surgical correction. A right superior caval vein draining into the left atrium, although very rare, should always be considered a diagnostic possibility in the presence of cyanosis and normal clinical findings. Color flow mapping is an excellent method with which to make the diagnosis.

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 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.

We describe two patients, aged seven and 21 years, with unilateral atresia of a solitary pulmonary vein. Until now, to the best or our knowledge, this condition has been reported only twice. Although symptoms, diagnostic measures and treatment have been described in detail, clinical diagnosis is still difficult.

We evaluated the clinical significance of the configuration of right ventricular pressure curves after balloon valvoplasty in 35 patients with pulmonary valvar stenosis. Right ventricular pressures were measured with a fluidfilled catheter. We divided the subjects into two groups according to the pressure curves seen after balloon valvoplasty. In eight patients, two peaks were found in the curves, with the higher peak occurring at late systole. The remaining 27 patients had a single peak observed during early to mid systole. In all patients in the group with a single peak, the ratio of ventricular pressures decreased by more than half, and no residual narrowing was seen in right ventricular outflow tract, the diameter after valvoplasty increasing by more than half over the diameter before the procedure. In contrast, in five of eight patients in the group with double peaks in the pressure curves, the ratio between ventricular pressures remained higher than 0.5, and the diameter of the right ventricular infundibulum was reduced to less than half the diameter prior to balloon valvoplasty. In three of these patients with double peaked pressure contours, to whom propranolol was administered intravenously, the pressure configuration changed to one with a single peak and the ventricular pressure ratio fell to below 0.5. The degree of obstruction of the right ventricular infundibulum also decreased. These data suggest that a high right ventricular pressure and two peaks in the pressure curve with the higher peak at late systole after balloon valvoplasty indicate, first, the presence of a significant narrowing in the right ventricular outflow tract and, second, effective balloon valvoplasty.

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.

Aneurysmal dilation of the right atrium is described in a four-year-old boy who presented with cardiomegaly and symptoms of congestive heart failure. Echocardiography revealed the aneurysmal dilation of the right atrium, but a definite diagnosis was obtained only after surgical exploration. This had caused compression of the other cardiac chambers resulting in congestive heart failure. Surgical resection was successful and the patient was discharged on the eleventh postoperative day with good results.

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.

We have now achieved transcatheter occlusion of the patent arterial duct as a day-case procedure in 26 patients. In all but two, who were judged not to be appropriate for the device, implantation was successful. Most of the patients arrived in the hospital at 8:00 a.m. and had to wait for a mean time of 154 minutes until they were taken for cardiac catheterization. The procedure, performed under sedation in 25 patients and under general anesthesia in two, took a mean of 91 minutes. The children were then monitored for two hours in the recovery room. The interval From the time of arrival in the Unit until waking was a mean of 196 minutes. Then, from the time of waking, a mean of 123 minutes elapsed prior to discharge. Admission of the patient was necessary in three instances (11.1%). Two patients vomited and remained too drowsy for safe discharge. A third patient had either occlusion or thrombosis of the femoral artery and received an infusion of heparin which necessitated admission overnight. All three patients were discharged the following morning. Patients from outside Riyadh stayed overnight in Riyadh before they went home. One patient complained of non-cardiac chest pains a few hours after discharge and was subsequently sent home. Total occlusion, as judged by mean follow-up of 13 months (5–26 months) using color flow Doppler was achieved in 19 of the 24 patients (79!) in whom devices were inserted. All patients are well and there are no complaints concerning the procedure done on a day-case basis. We conclude that the day-case protocol for transcatheter occlusion of the patent arterial duct is a safe and cost-effective option for selected patients.

Cerebrovascular accidents are a rare but feared complication of open heart surgery. We report a child with tricuspid atresia who suffered cerebral infarction after a Fontan procedure and discuss possible risk factors.

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.

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.

Flow in the descending aorta was studied to characterize aortic run-off in patients with shunts at the arterial level. Pulsed Doppler examination of descending aortic flow was performed with simultaneous estimation of ratios of shunt-to-systemic flow (shunt flow ratio) at cardiac catheterization. We studied 14 patients with shunt-dependent circulations, eight patients with isolated persistence of the arterial duct, 11 normals and five patients immediately after umbrella occlusion of their persistent arterial duct. Parameters of diastolic flow were standardized to systolic parameters. The ratio of diastolic velocity to the time integral (velocity/time integral retrograde diastolic flow ÷ velocity/time integral systolic flow) ranged from 1×10−1−10.8×10−1. Shunt flow ratios ranged from 0.1−3.9. Pandiastolic retrograde flow was seen in 21 of 22 patients with shunts at the arterial level (the ratio was 0.1 in the remaining patient). No normal patient exhibited pandiastolic retrograde flow. Of the five patients undergoing ductal closure, three exhibited persistent pandiastolic retrograde aortic flow following effective occlusion. The ratio of diastolic velocity to the time integral correlated with the ratio of shunt flow (r=0.76, standard error 0.7). A diastolic velocity/time integral ratio of greater than 8.5×10−1 (sensitivity 67%, specificity 94%) selected patients with shunt flow ratio greater than two, and a diastolic velocity/time integral ratio of less than 5×10−1 (sensitivity 100%, specificity 94%) selected shunt flow ratios of less than 0.5.

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.

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.

Complex congenital heart defects are accurately detectable antenatally using fetal echocardiography. In continuing pregnancies with fetal cardiac abnormalities, regular follow-up with advancing gestation is essential. This report documents the changes that occurred in the fetal cardiac anatomy in a fetus detected during antenatal scanning to have an atrioventricular septal defect with isomerism of the left atrial appendages.

A study was made of 26 adolescents, aged from 13 to 18 years, with different types of severe congenital heart disease, assessing their somatic condition and its impact on mental health and psychosocial functioning. Physical capacity was reduced, more pronounced in girls than in boys, along with an increased rate of psychiatric problems. Associations were found between physical capacity and psychosocial functioning, and between psychosocial functioning and chronic family difficulties. On the other hand, half the patients studied achieved fair function, both physically and with regard to their mental health. The degree of reduced physical capacity is an important etiological factor for impaired mental health in patients with congenital heart disease. This knowledge should be taken into account when dealing with this group of patients. A good social network seems to be a protective factor. It will probably be rewarding to give patients and their families not only an optimal medical follow-up, but also psychological, practical and financial support to improve or create such a network.

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.