Deletion of chromosome 22 and DiGeorge syndrome are associated with inappropriate development of the neural crest and abnormalities of the ventricular outflow tracts. Bis-diamine induces comparable cardiac anomalies by inhibiting the normal development of the neural crest. In order to clarify the effect of damage on the development of the neural crest and its relation to the cardiac anomalies, the effects of giving a small dose of bis-diamine were compared with the results of a large dose of bis-diamine. A small dose of bis-diamine (20 mg), therefore, was administered to 40 pregnant rats on the 10th day of pregnancy, and 330 fetal hearts were studied on the 21st day with the rapid whole-body freezing method. Intracardiac anomalies were rare, being found in only 55 fetuses. The anomalies discovered were: aberrant origin of the subclavian artery in 88 fetuses, right aortic arch in 26 fetuses, interruption of the aortic arch in 23 fetuses, and high location of the aortic arch in 8 fetuses. Anomalies of the arterial duct were also common; with a vascular ring formed by the aortic arch and contra-lateral duct being seen in 21 fetuses, isolation of the right subclavian artery associated with either bilateral ducts (9 fetuses) or a right duct and absent left duct (9 fetuses), absent duct with tetralogy of Fallot in 7 fetuses, and with common arterial trunk in one fetus. In conclusion, a small dose of bis-diamine induced extra cardiac anomalies of the aortic arch and arterial duct, indicating a greater susceptibility of the aortic arch and the duct to inappropriate formation of the neural crest cells in the early developing embryo. Clinically, these anomalies of the aortic arch and the duct should be searched for in all patients suspected of having deletion of their 22nd chromosome.

Fifty one years have passed since gross and Crafoord independently reported successful repair of aortic coarctation. One could be forgiven for assuming that all the surgical controversies have now been settled. This is far from the case, as the numerous publications (frequently with opposing view points) related to timing, technique and complications bear testament. Perhaps the passage of the golden anniversary of this operation should stimulate a degree of reflection among surgeons and cardiologists.

Abstract Hepatic venous blood has been thought to play some role as a vasoactive agent in the development of pulmonary arteriovenous fistulas in patients with congenital heart disease. During the last 15 years, we have observed pulmonary arteriovenous fistulas in 3, and systemic arteriovenous fistulas in 2, patients from our 16 cases of left isomerism. During the same period, neither pulmonary nor systemic arteriovenous fistulas were detected among 50 patients with right isomerism. Pulmonary arteriovenous fistulas had developed in the absence of surgery in 1 of the patients. Both pulmonary and systemic fistulas were detected in an another patient, in whom the hepatic venous blood bypassed the pulmonary circulation. The level of somatostatin, which is known to reduce splanchnic blood flow, was high in the systemic venous blood of this patient. Although the mechanism of development of the fistulas has yet to be clarified, we should be aware that not only pulmonary, but also systemic arteriovenous fistulas can be found in patients with left isomerism, even prior to any surgical intervention.

We evaluated the physical characteristics of the pulmonary trunk in 32 patients with complete transposition after the arterial switch operation, calculating the pulmonary input impedance. In 6 of the patients, the characteristic impedance was equal to, or less than, the value in patients with ventricular septal defect. In those patients with an intact ventricular septum, or those with a ventricular septal defect who had undergone the arterial switch operation or banding of the pulmonary trunk before 3 months age, the frequency of the first modulus minimum also agreed with the values obtained in patients with ventricular septal defect. The value was high, however, in patients with complete transposition with ventricular septal defect who had undergone an arterial switch operation or banding of the pulmonary trunk after 4 months of age. Our data suggest decreased distensibility of the proximal pulmonary trunk after the arterial switch operation, and mild changes in the pulmonary vasculature in patients with complete transposition with ventricular septal defect who had undergone either an arterial switch operation or banding of the pulmonary trunk after 4 months of age. Long-term follow-up is needed clarify whether or not these features affect right ventricular function by means of an increase in afterload.

Device closure of oval fossa atrial scptal defects with the Amplatzer Septal Occluder was performed in 26 patients ranging in age from 0.89 to 60.44 years. In eight additional patients no device implant was performed because of the presence of multiple defects or because the defect was of a size unsuitable for closure with the devices currently available. The strectched diameter of the defects that were closed ranged from 4 to 23 mm (mean 14±5.4 mm) and device sizes ranged from 4 to 24 mm. Two devices were unstable, of which one embolized to the right atrium after release. Both devices were retrived at the same procedure. One of these parients subsequently underwent a successful device closure of his defect using a larger (24-mm) device. Three patients had multiple defects, which were successfully closed with a single device. At 1-month follow-up 23/26 (88%) and at 3-month follow-up 22/24 (92%) patients had complete closure of their defects, while two had residual shunts. One further patient who had complete closure of his defect at 1-month post-implant had his device removed and his atrial septal defect patched surgically 8 weeks after device closure. This was done as a result of the development of a vegetation affecting the device after an episode of septicaemia, which was not relate to the cardiac problems. There was no procedure-related morbidty or martality and all patients remain well at the present time.

To assess the effect of a modified Fontan operation on systemic venous blood flow and the hepatic circulation, we compared 11 patients having an atriopulmonary connection and 35 with total cavopulmonary anastomosis. The Doppler echocardiographic study of the caval venous, hepatic venous and portal venous flow was performed so as to calculate the pulsatility ratio and the variation of flow with respiration. All patients had undergone cardiac catheterization. In addition, we included specific laboratory investigations to assess function of various organs.

Significantly lower maximum velocities of flow at inspiration (0.31±0.12 rn/sec vs 0.45±0.14 m/sec) and expiration (0.23±0.09 rn/sec vs 0.32±0.11 m/sec), less pulsatility (0.43 vs 0.16) and a lower ratio of systolic to diastolic velocity (1.22 vs 1.85) were found in the patients having a cavopulmonary as compared to an atriopulmonary anastomosis. Peak velocities of hepatic venous flow during inspiration and expiration were significantly lower in those with a cavopulmonary anastornosis (p=O.OOl and p<O.OOl, respectively). In these patients, forward flow was extremely dependent on respiration, with decrease or cessation of antegrade flow during expiration in 22 patients. The velocity of portal venous flow was also significantly lower in these patients, although the pulsatility ratio did not differ significantly between the groups (0.5 ± 0.21 and 0.57 ± 0.23, respectively). The ratio of inspiratory and expiratory velocities showed no significant difference between the groups, nor was there any correlation between the pulsatility ratio of the venous vessels or the ratio of peak flow velocities during expiration and the mean systemic venous/right atrial pressure on postoperative cardiac catheterization. Hypoproteinemia was found in 8 patients after total cavopulmonary anastomosis; 9 of 10 patients with protein C deficiency belonged to this group.

The dependence of hepatic venous flow on respiration in the presence of a chronically elevated systemic venous pressure in patients after the total cavopulmonary anastornosis may influence hepatic function in the postoperative period.

Pediatric cardiac surgery in Indonesia first developed thanks to the cooperation of various cardiac centers abroad. The establishment of the ‘Harapan Kita’ National Cardiac Center in 1985 was one of the most important initial steps. Thereafter, the discipline advanced remarkably in terms of the number of the operations performed and the variety of the diseases treated and, as a result, the surgical outcome also improved. Numerous problems remain to be solved. Only 1% of the children with congenital heart disease are today properly treated in Indonesia. Some of the underlying problems responsible for this situation include a shortage of pediatric cardiac professionals, the lack of the information and education on the part of the patients, and a shortage of funding, both privately and publicly. It would thus be welcome for pediatric cardiac surgeons, cardiologists and nurses in Indonesia to learn about congenital heart disease from doctors and nurses in advanced countries in order to improve the outlook at home.

Severe mechanical hemolysis ocurred in a 22 month old girl after placement of a 38-5-10 coil in the arterial duct. Shé had previously undergone percutaneous closure using the Rashkind technique 14 months before insertion of the coil, but remained with a moderate residual shunt. Surgical removal of the devices and division of the duct were required to abolish the hemolysis.

Advances in diagnosis, medical management and surgical intervention have improved the longevity and quality of life for children with congenital heart disease. Despite this, research studies specifically examining the psychosocial concerns of adolescents and young adults with congenital heart disease are few. To explore the subjective experiences and dilemmas of this population during the transition from adolescence to young adulthood, we interviewed, using a semi-structured protocol, a convenience sample of nine adolescents and young adults. Using analytic procedures inherent in Grounded Theory methodology, seven themes were identified: the dilemma of normality; dilemmas in disclosure; dilemmas in strategies for management of illness; the challenge of social integration versus social isolation; the challenge of dependence versus independence; the challenge of uncertainty; and strategies for coping. An understanding of these experiences by health professionals can be beneficial in helping this clinical population as they grow up and face the challenges of an uncertain, yet promising, future.

Balloon dilation was performed in four patients with postoperative pulmonary stenosis who had undergone surgical creation of a coronary arterial tunnel in the pulmonary trunk. Two patients had complete transposition in whom the arterial switch operation had been performed using the modified Aubert method. The other two patients had anomalous origin of the left coronary artery from the pulmonary trunk treated with the Takeuchi procedure. Balloon dilation was performed at 11 locations. The pressure gradient decreased from 48 ± 22 to 24 ± 14mmHg (p<0.01), and the diameter of the narrowest segment increased from 5.3 ± 2.5 to 7.5 ± 2.8mm (p<0.01), respectively. Of the 11 procedures, 8 (73%) were judged successful with use of the criterion of success as a greater than 50% decrease in pressure gradient, and/or a greater than 50% increase in diameter. The inflated balloon must have compressed the coronary arterial tunnel in the pulmonary trunk, but there was no apparent myocardial damage in any patient, although transient and mild ST-T changes appeared on electrocardiographic monitoring during the procedure in 2 patients. Rupture of the wall of the pulmonary trunk occurred in two patients, one of whom required elective surgical intervention. These data suggest that balloon dilation should be performed with caution for management of postoperative pulmonary arterial stenosis in patients with a surgically created intrapulmonary coronary arterial tunnel, since tearing the wall of the pulmonary trunk may occur.

The finding of bland, sterile vegetations in children with severe tetralogy of Fallot is unexpected, and to our knowledge, has not been reported previously. Eight patients diagnosed with tetralogy between January 1993 and July 1997 had sterile vegetations proven by histological and microbiological evaluation, in their right ventricular outflow tracts. Four of these patients were experiencing severe hyper-cyanotic spells, and four had severely reduced effort tolerance at presentation. They all underwent cardiac catheterization and were submitted for surgical repair. At surgery, the vegetations were thought to be causing further narrowing of the already tight fibrotic infundibular stenosis. Two of these patients had evidence of damaged valves, without evidence of active endocarditis. Although initially sterile, these vegetations, may in some instances, become infected.

We studied factors that predict in children catecholamine induced vasoconstriction of the persistent arterial duct. Epinephrine at 2.0–10.0 (4.9 ± 1.3, mean ± standard deviation) μg/kg was injected intravenously into 30 patients with persistent arterial duct whose age ranged from 1 to 91 (27 ± 26) months. In 11 patients aged 10–66 (34 ± 23) months (responders), a continuous murmur had almost completely disappeared on auscultation, and both the diameter and the shunt flow area of the arterial duct had become smaller as shown by Doppler echocardiography. On the other hand, there was no such change in 19 patients aged 1–91 (23 ± 27) months (non-responders). Although there was no significant difference in the age, the body weight, the minimal diameter of the arterial duct, or the doses of epinephrine between responders and non-responders, the gestational age at birth was slightly less (p=0.09) and the birth weight was significantly smaller in responders than in non-responders (p<0.05). The persistent arterial duct of those who had a history of low birth weight always responded to epinephrine. In infants younger than 6 months, the persistent arterial duct was never constricted by epinephrine regardless of the birth weight and gestational age. In low birth weight infants, the vascular smooth muscle of the persistent arterial duct can usually constrict beyond infancy. There may be some age dependent difference in responsiveness to epinephrine until 6 months of age.

We sought to evaluate the saftey and efficacy of acure and chronic treatment with verapamil infanta with hypertrophic cardimyopathy. Prior studies have shown an improvement in adluts with hypertrophiuc cardiomyophathy who were treated with verapamil. Acutely, it reduced the degree of left ventricular outflow tract abstruction. Chronic therapy was associated with an improvement in sysmptoms ans incresed long-term survial. To date, no data are available on the efficacy of this drug in infants with hypertrophic cardiomyopathy. We evaluated prospectively the safety and efficacy of verapamil in infants. The acute and chronic effects of verapamil on infants with an echocardiographic diagnosis of hypertrophic cardiomyopathy were evaluated at a single institution between 1980 and 1994, with long-term follow-up available until 1996. Acute effects of an intravenous bolus of 0.1 mg/kg and infusion at 0.007 mg/kg/min were evaluated, where possible, in the cardiac catheterization laboratory. Oral verapamil at 3–5 mg/kg/day was started after catheterization. Follow-up included serial clinical, echocardiographic and Holter monitoring. A total of 22 patients were studied, 17 having a presumed diagnosis of primary hypertrophic cardiomyopathy including three with Noonan's syndrome. Acute infusion of the drug was well tolerated by all, without adverse electrophysiological effects. Haemodynamic effects were consistent with a negative inotropic action with significant falls in cardiac index (4.6±1.2 to 4.1±0.9 l/min/m2), systolic blood pressure (88±16 to 82±14 mmHg) and gradient across the left ventricular outflow tract (nine patients 48.2±30.4 to 28.4±24.1 mmHg). End-diastolic pressure was unchanged (14.0±6.8 to 13.9±4.7 mmHg). Three patients with primary hypertrophic cardiomyopathy died (two while being treated). In the group with primary hypertrophic cardiomyopathy continuing with long-term treatment, follow-up revealed regression in two, progression in three (two died) and stability in 10. For those treated, there was a trend towards improvement in clinical status. Verapamil is well tolerated acutely in infants with hypertrophic cardiomyopathy. The outcome was considerably better in these patients compared with prior reports, though careful long-term assessment is needed.

Superior caval venous syndrome is one of the late problems known to occur after Mustard repair of complete transposition. Reoperation may leave residual stenosis, and carries substantial risk for the patient. It is now feasible to use intravascular stents to overcome systemic venous baffle obstructions, and such an approach is probably more effective. The purpose of our study therefore, was to assess immediate and medium term results of inserting stents subsequent to gradual balloon enlargement of acquired atresia of the intraatrial baffle in patients who had undergone an atrial switch operation. We investigated five patients with complete obstruction of the superior caval venous pathway at perforation of the atretic segment was achieved using a guide wire technique. The procedure was successful in all patients. Gradual angioplasty was performed and intravascular stents were implanted. The pressure in the superior caval vein dropped to normal values, symptoms improved, and the patency of the newly created venoatrial communication was proven at mid-term follow-up. Thus critical obstructions at the superior caval venous pathway after the Mustard procedure can be reopened by interventional catheterization. Implantation of balloon-expandable intravascular stents is safe and effective in the acute relief of the obstructions, but careful long-term follow-up is mandatory.

It has already been suggested that redundancy of the primary atrial septum could be associated with primary fetal arrhythmias. There are few reports, however, concerning the mobility of the primary septum and its behavior during fetal life. In this study, fifteen fetuses without anatomic heart disease were serially evaluated by fetal echocardiography. The time interval between three separate examinations was at least two weeks. A redundancy index was calculated as the ratio between the maximum excursion of the flap valve of the septum and the maximal left atrium as seen in a four chamber view. The indexes observed in the three examinations were: 0.55 ± 0.15, 0.48 ± 0.13 and 0.41 ± 0.13, respectively. Analysis of variance for normal distribution and Tukey's test were performed. A significant decrease (p < 0.05) in the redundancy index was observed with increasing gestational age. We postulate that this finding may be related to the decrease in frequency of premature atrial beats throughout fetal life.

Management of pulmonary vascular resistance in neonates with congenital heart disease is important for stabilization before and after surgical interventions. Thus, we determined which combina tion of positive end-expiratory pressure ventilation and fraction of oxygen in the inspired air increases pulmonary vascular resistance without compromising delivery of oxygen to the tissue. Eight piglets were anesthetized, intubated and ventilated. Pulmonary flow and pulmonary arterial and left atrial pressures were monitored continuously. At all levels of inspired oxygen (1.00, 0.21 and 0.15), ventilation at a pressure of 15 cm of water increased pulmonary vascular resistance. At all levels of positive pressure ventilation, a fraction of 0.15 of inspired oxygen increased pulmonary vascular resistance. The combination of a ventilatory pressure of 15 cm of water and inspired oxygen of 1.00, or ventilatory pressure at 5 cm of water and oxygen delivery of 0.15, produced similar changes in pulmonary vascular resistance (19.1 ± 2.8 vs. 20.0 ± 3.8 mmHg/(L/min)) and cardiac output (0.78 ± 0.07 vs. 0.93 ± 0.10 L/min) but, the higher level of positive pressure plus 1.00 inspired oxygen gave a significantly higher arterial oxygen saturation (0.99 ± 0.03 vs. 0.72 ± 0.19%) and delivery of oxygen to the tissues (13.7 ± 2.9 vs. 7.4 ± 1.5 ml 02/min, p<0.05). Thus, both high positive pressure ventilation and hypoxia increase pulmonary vascular resistance. Only high pressure ventilation plus high concentrations of inspired oxygen, however, increased pulmonary vascular resistance without compromising delivery of oxygen, suggesting that this combination is a superior means of increasing pulmonary vascular resistance.

To evaluate the influence of the size of the defect and the age of surgical repair on left ventricular mechanics, including geometry, shape, diastolic and systolic function as well as myocardial contractility, we used cross-sectional echo-Doppler to study 20 patients (12 males, 8 females) who had undergone successful surgical closure of a ventricular septal defect. The patients were divided in two groups, corrected early and late, on the basis of the degree of left-to-right shunting (ratio of pulmonary to systemic output of greater or less than 2.5/1) and the age at the surgical repair (older or younger than 2 years of age). The group undergoing early correction included 11 patients, mean age 7.1 ± 1.8 years (range 4.2–11.8 years) having surgery at mean age of 1.3±0.6 years for a large ventricular septal defect (mean ratio of pulmonary to systemic output of 3.1/1; range 3.4–2.7/1) with a mean postoperative follow-up 4.6±1.9 years. The group of nine patients undergoing late correction had a mean age of 11.3±4.9 years (range 6.7–17.2 years), with a later surgical repair (mean age 4.7±2.7 years) for a moderate-sized ventricular septal defect (mean pulmonary/systemic output ratio 2.1/1; range 2.3–1.7) and a mean postoperative follow-up of 7±4.2 years. Each group of surgically repaired patients was compared with a control group matched for age, body surface area and gender. No significant differences were found between the normal controls and those undergoing early correction for any assessed functional index regarding left ventricular geometry (normalized volumes and mass for body surface area, mass/volume and thickness/radius ratios), shape (long axis–short axis ratio), diastolic (mitral and pulmonary venous flow patterns) and systolic (fractional shortening and rate-corrected mean velocity of circumferential fibre shortening) function. In addition, the data points for each patient for the rate-corrected mean velocity of circumferential fibre shortening to end-systolic stress relationship were within the 95% confidence limits of normal, suggesting normal left ventricular contractility. On the other hand, the patients undergoing surgery at a later age showed a persistent increase of the normalized left ventricular end-diastolic volume and mass, with an higher mass/volume ratio and reduced end-systolic stress compared with normal controls. Furthermore, left ventricular shape (long axis–short axis ratio) was abnormal at end-diastole but with its normal values at end-systole. Our data suggest that, in the presence of a large ventricular septal defect, early successful surgical repair <2 years of age results in complete recovery of left ventricular mechanics in the postoperative follow-up. In ntrast, surgical closure at >2 years of age, even for a moderately sized ventricular septal defect, deleteriously affects postoperative left ventricular geometry and shape. Since prolonged volume overload may be detrimental to myocardial function, earlier surgical repair should be recommended.

We reviewed retrospectively the clinical and surgical data from 9 children with postoperative chylothorax secondary to cardiac surgery for congenital heart disease seen over a 3 year period. Mean age was 26 months and 6 patients were female. The procedures performed were 2 Blalock-Taussig shunts, 6 Bidirectional Glenn anastomoses and 1 modified Fontan procedure. Diagnosis of chylothorax was made by classical means. The overall incidence of chylothorax was 0.8%, with the relative incidences being 2.5% for Blalock-Taussig, 2.7% for Fontan, and 11.7% for the Glenn procedures. All patients were initially treated conservatively with chest tube drainage and hypercaloric diet suplemented with medium chain triglycerides. Parenteral nutrition was needed in 2 patients, one because of sepsis and the other because of progressive malnourishment. High output fistulas and long periods of drainage were noted after cavo-pulmonary procedures. Up to 12% weight loss was observed in 6 patients, one patient was successfully treated of pneumonia and another died of Pseudomonas aeruginosa sepsis. Two patients required chemical pleurodesis because of unabated drainage, with the others being successfully treated conservatively. Chylothorax is a common complication after bidirectional Glenn procedures, and is associated with considerable morbidity and mortality. Earlier operative intervention may be required in these patients. Pleurodesis is a simple, safe, and effective procedure to stop chylous flow.

Anomalous origin of one pulmonary artery from the ascending aorta is an uncommon lesion with an uncertain outcome. We reviewed 16 consecutive children (9 males) presenting with this lesion over a 36 year period at a single institution. Median age at presentation was 2 days (range, birth to 3.2 years). The anomalous pulmonary was the right in 12 and the left in 4, all originating from the proximal ascending aorta, with no patient having stenosis at the origin of the anomalous pulmonary artery. Associated cardiac anomalies were noted in 9 patients. No intervention was attempted in 2 patients: one was deemed inoperable due to complex associated lesions and pulmonary vascular obstructive disease, while the other one died before repair. Surgical intervention was attempted in 14 patients, with 3 intraoperative deaths (21%). Of 11 operative survivors, 8 developed pulmonary arterial stenosis graded severe in 2, moderate in 1 and mild in 5. Patients with severe stenosis required surgical angioplasty, 1 after unsuccessful dilation combined with placement of an endovascular stent. One patient with moderate, and one with mild, stenosis underwent successful transcatheter balloon dilation. The remaining 4 patients with mild stenosis remain unchanged during follow-up. One patient had biopsy evidence of pulmonary vascular obstructive disease at 3.3 years of age. There were no late deaths, giving a total mortality of 25% (4/16).