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.

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.

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.

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).

Atrioventricular septal defect with common valvar orifice and tetralogy of Fallot is a rare combination of congenital cardiac anomalies. Approaches to this lesion have tended to emphasize either staged repair or complete repair beyond infancy. Between July 1992 and August 1997, nine patients underwent repair of complete atrioventricular septal defect with tetralogy of Fallot. One patient, aged 9.6 years at the time of repair, had previously undergone construction of a modified Blalock-Taussig shunt. Primary complete repair was performed in the other 8 patients at ages ranging from 2.5 to 16 months (median 4.6 months), and all but one were infants. All patients had a Rastelli type C defect, a single ventricular septal defect with inlet and outlet components, and malalignment of the muscular outlet septum with subpul-monary stenosis. A single patch technique, with closure of the zone of apposition (‘cleft’) in the left atrioventricular valve, was used in all eight patients undergoing primary repair, while a double patch was employed in the previously palliated older patient. In all cases of repair using a single patch, the anterosu-perior bridging leaflet was divided obliquely to the right, following the malaligned outlet septum, in order to avoid subaortic obstruction. Repair of the right ventricular outflow tract included infundibular myectomy in eight, pulmonary valvotomy in four, infundibular or transannular patching in three and one, respectively, and reconstruction with a valved allograft conduit in two patients. There was no early mortality or significant morbidity. At a median follow-up of 45 months, there had been one death related to non-cardiac causes and no reinterventions. Left atrioventricular valvar regurgitation was moderate or mild in two patients, and right atrioventricular valvar regurgitation was mild in one patient. No patient had more than mild pulmonary regurgitation or a gradient across the right ventricular outflow tract in excess of 18 mm Hg. Our results demonstrate that primary repair of atrioventricular septal defect with tetralogy of Fallot can be performed with excellent early and mid-term results in young infants. Although it has been suggested that a technique utilizing oblique division of the anterosuperior bridging leaflet may lead to high rates of atrioventricular valvar regurgitation, medium-term atrioventricular valvar function in the present cohort of patients has been excellent.

We describe 4 cases of congenitally corrected transposition associated with atrioventricular septal defect, diagnosed by echocardiography and angiocardiography. Two had usual atrial arrangement and two had mirror imaged atrial arrangement. All cases were associated with subpulmonary valvar stenosis. All patients presented with cyanosis and were in sinus rhythm. Atrioventricular septal defect with common atrioventricular junction was easily diagnosed on the basis of a common atrioventricular valve permitting interatrial and interventricular communications. All patients had balanced right and left ventricles. The echocardiographic recognition of the ventricles was based on the presence of the moderator band within the morphologically right ventricle, the characteristics of the apical septal trabeculations, and the shape of the ventricles. Angiocardiographic recognition of the ventricles was achieved on the basis of right and left ven-triculography.

In one case with usual atrial arrangement, we recorded two His bundle potentials, one anteriorly and another posteriorly. Atrial stimulation revealed blocked atrioventricular conduction at the level of the pos terior bundle, and normal atrioventricular conduction through the anterior bundle. In both cases with atrial mirror-imagery, only a posterior His bundle potential was found, with normal atrioventricular conduction revealed by atrial stimulation

The clinical course with this combination depends on the other lesions present in addition to the common atrioventricular valve. Our electrophysiological studies show that the conduction system in presence of a common atrioventricular valve is as expected for congenitally corrected transposition with two atrioventric ular valves.

Children with functionally single ventricle in the setting of visceral heterotaxy (isomerism) may present a surgical challenge at the time of Fontan completion because of anomalies of systemic and pulmonary venous drainage. We have used an extracardiac conduit in this population to direct inferior caval venous blood to the pulmonary arteries.

Over the past five years, nine children with heterotaxy and a functionally single ventricle underwent correction by placement of an extracardiac synthetic (Gore-Tex) conduit. All patients had previously undergone a bidirectional Glenn procedure. Age and weight at the time of insertion of the extracardiac conduit were 26±15 months, and 11±2 kilograms, respectively.

Between 1983 and 1995, 546 Blalock-Taussig shunt procedures were performed in 472 patients: 128 (23.0%) were classical shunts, 90 of them on the same side as to the aortic arch, and 418 (77.0%) were modified shunts, 182 on the same side of the arch. At the time of surgery, 78 patients were aged below one week, 270 from one week to 12 months, and 198 patients were over one year of age. The mean pre-operative arterial saturation (71.7%±16.5%) was significantly increased to 83%±17.9% imme-diately after the procedure (p=0.017). The overall hospital mortality rate was 2.9% (16/546), with rates of 2.3% (3/128) for the classical, and 3.1% (13/418) for the modified shunts (p= not significant). The rate was significantly higher, however, for classical shunts when the pulmonary arterial diameter was less than 4 mm (15.4% versus zero; p=0.047), though this relationship was reversed for modified shunts (zero versus 3.6%; p=0.338). Early mortality was significantly influenced by the age at surgery, 5/78 (6.4%) in patients aged below 1 week, 3.7% between 1 week and 1 year, and 0.5% over 1 year (p=0.019). Early mortality was also significantly increased in patients weighing 3kg or less, 8/156 (5.1%), versus 3/303 (1.0%), p=0.037. Overall, 51 shunts failed (9–3%), 10 early and 41 late. Early failure was significantly increased in patients weighing 3kg or less, 8/156 (5.1%) versus 3/303 (1.0%), p=0.016. The overall early failure rate was 1.4% (3/215) when heparin was administered intra-operatively and for 48 hours postoperatively, in contrast to an early failure rate of 3.4% (7/203) when heparin was not used (p = 0.294). Overall rates of failure during follow-up were 9.1% (17/188) in heparinized patients versus!3.6% (24/177), (p=0.173) in non-heparinized patients. Failure of classical shunts was 10.2% (13/128), compared with 6.7% (28/418) for modified shunts (p=0.195). Failure was more common overall if the pulmonary arterial diameter was less than 4 mm, 14.7% (9/61), as opposed to 8.7% (26/300) when the diameter was 4 mm or greater, (p=0.144). Administration of aspirin during follow-up after the modified shunt procedure reduced failure from 11 % (18/163) to 6.7% (10/150), p=0.176. Classical or modified Blalock-Taussig shunts, either on the same side or opposite to the aortic arch, can be performed on patients of any age with minimum postoperative complications and low operative mortality. The use of intra- and post-operative heparin appears to reduce the overall rate of failure, and the administration of aspirin during follow-up appears to reduce failure of modified Blalock-Taussig shunts.

This study compared anxiety, fears, depression and behavioural problems as occurring in children with congenital heart disease, comparing them with samples of normal children. It further considered the influence of maternal anxiety, as well as analyzing a subgroup of children with cyanotic forms of congenital heart disease to determine if they were at higher risk than acyanotic children for the problems identified.

Muscular ventricular septal defects were diagnosed by echocardiography in 97 neonates within 7 days of birth. In 82 of the neonates (84.5%), the defect was solitary, while 15 had multiple defects. The solitary defects was located at mid-septal, apical, anterior and inlet locations in 42 (51.2%), 21 (25.6%), 14 (17.1%) and 5 (6.1%) neonates, respectively. Multiple defects occurred in the apical, anterior and mid-septal areas. The diameter of the solitary defects ranged from 1 to 6 mm (2.3 ± 0.8 mm), while the multiple lesions were 1 to 4 mm in diameter (2.1 2.3 ± 0.8 mm 0.8 mm) in 28 instances in which they could measured. It proved possible to follow 79 of the patients for period of 10 to 13 months. The defects closed spontaneously in 56 (84.8%) of 66 patients with a single defect, and in 7 (53.8%) of 13 of those with multiple defects (P<0.05). For the solitary defects, the position and size were factors determining the likelihood and speed of closure. Defects located at the apical septum, or defects larger than 4 mm in diameter, closed slowly and at a later stage. Echocardiography is an useful technique in establishing of natural history of muscular ventricular septal defects encountered in neonates.

Pericardiocentesis is usually an easy and uncomplicated procedure when guided by cross-sectional echocardiography, but an abnormal intracardiac or extrapericardial position of the puncture system can occur, especially in children. Injection of echo contrast medium through the puncture needle is a very sensitive, quick, easy and harmless procedure which can be performed at the bedside in all cases in which doubt remains concerning the location of the needle. Prompt enhancement of signal intensity of the peri-cardial fluid validates the correct intrapericardial position, and permits visualization of the tip of the needle. Absence of echodense formations excludes an intrapericardial position. We recommend the use of echo contrast medium during pericardiocentesis whenever blood is aspirated through the needle, or if there is any doubt concerning its location.

A 41-day-old boy was transferred to our department with severe congestive heart failure. Digital subtraction counter current aortography, and antegrade aortography, revealed coarctation of a persistent right fifth aortic arch, stenosis of the origin of an aberrant left subclavian artery, and sequestration of the lower lobe of the right lung.

As his heart failure seemed to be caused not only by pressure overload to the left ventricle following coarctation, but also by the volume load associated with the shunting effect of the pulmonary sequestration, we performed transcatheter balloon dilation of the coarctation and the origin of the aberrant subclavian artery, together with embolization of the aberrant pulmonary artery. His heart failure responded dramatically to these procedures.