Form 1980 until January 1991, 76 patients with double outlet ventricle, aged from 1 year 7 months to 23.5 years (mean age 7.5± 5.0 years) underwent surgey at the A.N. Bakoulev Inste for Cardivascular Surgery. Associated heart defects were found in 77.6% patints. In 57 patients (75%), the left ventricle opened into the subaortic infundibulum; into the subpulmonary infudibulum in four (the Tassisg-Bing anomaly) while, in 12 (15.8%), it opened under the outflow tracts of both great arteries. In two cases, (2.6%) there was a mno-committed ventricular septact. In one case (1.3%)a non-committed defect was diagnosed as coexisting with a non-commited defect was dignosed as coexisting with a Taussig-Bing anomaly. There were eight operative deaths (10.9%) among the patients undergoing radical surgery.Another theree patients with the Taussig-Bing anomaly and obstructive pulmomnary vascular disease underwent a successful palliative Mustard operation. All deaths were associated with acute heart failure and were seen only among patiens with subaortic ventricular septal defects. The important factors, which increased the risk of correction and contributed to death were inappropriate pre-operative diagnosis of complex associated defecrs and techmical mistakes during surgical intervention, the latter due to inadequate surgical experience. During the last five years, mortality decreased significatly (one death among 34 patients undergoing surgery from 1986 through January 1991)

Surgical Management of straddling tricuspid valve and associated defects is a complex problem. Between August 1992 and August 1995, 5 patients with major straddling of the tricuspid valve underwent a complete or partial biventricular repair. All patients had either an inlet ventricular septal defect (n=4) or a ventricular septal defect with an inlet component (n=1), Co-existing cardiac lesions included hypoplasia of the right ventricle (n=3), discordant ventriculoarterial connections (n=1), tetralogy of Fallot (n=1), and multiple muscular vetricular septal defects (n=2). At the time of presentation to our institution, two of these patients had previously been palliated in preparation for a Fontan procedure, having undergone construction of a bidirectional superior cavopulmonary shunt. One patient was referred specifically for a Fontan procedure. The tricuspid valve was repaired by transecting all of the straddling cords and reattaching them in the right ventricle or onto the right side of the patch used to close the ventricular septal defect. Associated procedures included closure of the septum in all patients, an arterial switch procedure in one, repair of tetralogy of Fallot in one, and construction of a bidirectional superior cavopulmonary shunt in one. There has been no early or late mortality. Complete heart block requiring insertion of a pacemaker occurred after surgery in three patients. At a median follow-up of 32 months, functional integrity of the tricuspid valve is well maintained, with only one patient having moderate tricuspid regurgitation. None of the patients are receiving any cardiac medication.

Of the 12 children who represent the complete cohort of Norwegian patients surviving the complete palliative operative programme for hypoplastic left heart syndrome, 10 were studied by a pediatric cardiologist, a pediatric neurologist, a neuropsychologist and a child psychiatrist. We found significant morbidity. Hemodynamic function was relatively uncompromised in eight of the patients, but one had significant cyanosis due to use of an operative technique subsequently abandoned and one had obstruction of the flow of blood from the left to the right atrium. Five children had disturbances of rhythm, with one requiring insertion of a pacemaker. One child had epilepsy. Delayed motor development, most often to mild degree, was found in seven children, while six had attention problems. Mental retardation was found in two children and autistic traits in one. Despite these various problems, the parents were seemingly satisfied with their own lives, and the quality of life of their children.

From January 1988 through to July 1994, 54 consecutive infants underwent an arterial switch operation for simple or more complex forms of complete transposition (concordant atrioventricular and discordant ventriculo-arterial connections). They ranged in age from 2 to 180 days. The Lecompte maneuver was performed in all. In the first 19 patients the harvested sinuses of Valsalva were filled with two separate patches of autologous preserved pericardium, while, in the last 35 patients, a wide pantaloon patch of tanned pericardium was employed. Five babies died within 30 days after the procedure (operative mortality of 9.2%, 70% CL 4–17%). Four survivors were found to have developed a significant supravalvar pulmonary stenosis from 1 month to 21 months postoperatively. Echocardiographic data showed a transpulmonary peak systolic gradient from 60 mmHg to 101 mmHg, with a right-to-left ventricular systolic pressure ratio from 0.65 to 0.9. Reoperation was performed from 8 months to 39 months after the arterial switch procedure. Supravalvar pulmonary stenosis was located at the level of the pulmonary trunk, extending distally in two cases, due to the growth of fibrous scarring tissue with partial calcification. The pulmonary valvar leaflets were involved in two cases. Relief of the obstruction was obtained by insertion of a wide shield-tailored polytetrafluoroethylene patch after making an inverted Y-shaped longitudinal incision in the pulmonary trunk between the anterior sinuses of Valsalva. No mortality occurred at reoperation. Early and midterm echocardiographic measurements showed the effectiveness of this technique, with only trivial or mild residual transpulmonary gradients.

Case records of 67 children who presented beyond infancy (57 male, 10 female) with congenital valvar aortic stenosis were reviewed to assess progression utilizing the Doppler derived peak gradient as the index of severity. Age at presentation ranged from 1–13 years, and mean follow-up was 67 ± 29 months (range 12–142 months). The patients were divided into three groups depending on the rate of progression. Those with the most rapid rate of progression were significantly older when aortic stenosis was detected and at the time of presentation compared to the other two groups, who progressed minimally or not at all (p < 0.002 and p < 0.01 respectively). There was no significant correlation between the progression of stenosis and other clinical or Doppler echocardiographic variables.

The cerebral abscess still occurs in children with cyanotic congenital heart disease. Mortality and morbidity have not yet been reduced in spite of new antibiotics and surgical techniques. We describe our experience with 185 patients having cyanotic congenital heart disease (117 with tetralogy of Fallot, 23 with complete transposition, 9 with tricuspid atresia, 4 with total abnormal venous return, 10 with double-inlet ventricle, 15 with double-outlet right ventricle and 7 with common arterial trunk) who have been followed regularly in our department. Their ages ranged between 3 years and 15 years. During the period of follow-up, cerebral abscess developed in 7 cases. Six of them had tetralogy of Fallot and one had complete transposition. One patient died before surgery could be performed. Operation was carried out in 6 patients and one died in the early postoperative stage. The others were as healthy as could be expected. Alpha-hemolytic streptococcus was isolated from the abscess in one patient, and Enterobacter in another. The abscesses were localized in the occipito-parietal region in 2 cases, the fronto-parietal region in 2 cases, and the parietal, temporal and occipital regions in one case each. Physical examination was normal apart from fever in 5 patients and, in these patients, the cause of fever could not be explained. Neurologic evaluations were all within normal range. If fever occurs unexpectedly in a patient with cyanotic congenital heart disease, therefore, it is important to rule out the possibility of a cerebral abscess.

Definitive repair of tetralogy of Fallot was performed on 74 patients under two years of age, including 40 under the age of one year and 13 less than 6 months of age. In six patients, there had been previous construction of a systemic-to-pulmonary arterial shunt. Patching across the ventriculo-pulmonary junction was required in 46 patients, placement of conduits in six, and procedures producing unifocaliza-tion in three. Three patients died during the 60-day postoperative period of observation. Of the 71 survivors, 64 recovered without complications. One patient had neurologic sequels, one required prolonged mechanical ventilation, two reoperation, and one balloon dilation of residual left pulmonary arterial stenosis. Morbidity and mortality were not significantly higher in those patients undergoing surgery at earlier than 6 months of age, although transjunctional patching was more common in that age group. Previous construction of a shunt was associated with a higher prevalence of distal stenosis and distortion of the pulmonary arteries, which needed surgical repair in two out of six patients (33%). Since the outcome of definitive repair is favourable in patients with tetralogy of Fallot younger than six months of age, we now advocate definitive surgery for all young symptomatic patients by the age of six months.

Construction of a modified Blalock-Taussig shunt using polytetrafluroethylene is frequently used for palliation in infants with complex cyanotic heart disease. This study was performed to assess angiographically the late patency rate of such prosthetic shunts. A modified Blalock-Taussig shunt had been constructed in 82 children and, of these, 63 (77%) patients had post-operative angiography and were included in this study. There were 29 males and 34 females, ranging in age from 1 day to 164 months. A second shunt was needed in 7 patients. Thus, 70 shunts were studied. Of the shunts, 16 were 4 mm in diameter, 28 shunts 5 mm in diameter, and 26 shunts 6 mm in diameter. Postoperative angiography was performed on 1 day to 132 months (median 39 months) after construction of the shunts. Acute post operative occlusion occurred in 2(3%) patients, who had 5 and 6 mm shunts, respectively. At the latest follow-up (median 39 ± 26 months), 6(9%) late occlusions were noticed. Overall patency was 89% for all the shunts, with 94% of the 4 mm shunts, 85% of the 5 mm and 88% of the 6 mm shunts being patent. Regression analysis showed a progressive reduction in diameter of the graft with time. This narrowing of the graft may have been related to the formation of a ‘neointima’ due to fibrous hyperplasia.

Thrombosis of the right side of the heart has previously been a rarely reported early or late postoperetive complication after a modified Fontan-procedure.

In 4(5.5%) of 72 surviving patients who underwent a total cavopulmonary connection for various forms of univentricular atrioventricular connection or other complex lesion a thrombus within the right atrium was diagnosed by transthoracic echocardiogaphy either early (in 3 cases) or late (in one cases) postoperatively. Predisposing haemodynamic abnormalities could be identified in all four patients, but no patient was found to have severe abnormalities of coagulation. Two patients presented with acute clinical symptoms on the third and twelfth day after initial surgery. They underwent emergency reoperation with thrombectomy followed by prophylactic treatment with coumadin or aspirin after initial heparinization. In the other 2 patients, routine postoperative echocardiography revealed a right atrial thrombus without specific clinical symptoms 25 days or 3 months postoperatively. Thrombolytic therapy with an initial bolus of tissue plasminogen activator (0.1 mg/kg) followed by continuous infusion (1 mg/kg/day) combined with heparinization yielded complete resolution of the thrombus after 27 and 40 hours, respectively, as demonstrated on follow-up echocardiograms. Our own experience, coupled with a review of the literature, indicate that prophylactic anticoagulation therapy with coumadin should continue for at least 4 to 6 months for patients with an increased risk for postoperative thrombosis. For infants and young children with a low risk for postoperative thrombosis, aspirin should be given routinely for 6 months postoperatively.

The procedure of total cavopulmonary connexion includes trans-section and closure of the pulmonary trunk, the systemic venous blood flow being directed to the pulmonary vascular bed by an anastomosis of the superior caval vein with the right pulmonary artery. Following this procedure, there remains a small residual pulmonary trunk. The purpose of our study was to obtain information about the fate of this residual trunk and its contained pulmonary valve. Using transthoracic echocardiography, we examined 29 patients (mean age 7 years 4 months) after a total cavopulmonary connexion (mean interval 17 months). We were able to obtain adequate visualization of the pulmonary valve and the pulmonary trunk in 23 of the patients. Pulmonary regurgitation was demonstrated by colour Doppler echocardiography in 13 pts (57%). Formation of thrombus in the residual pulmonary trunk was detected in one patient (4%) 6 months after the operation. One cerebrovascular embolic event occurred in a patient who had pulmonary regurgitation without formation of thrombus in the residual trunk 3 months after the total cavopulmonary connexion. Our findings show that pulmonary regurgitation is frequently present in patients after total cavopulmonary connexion. Although formation of thrombus in the residual pulmonary trunk seems to be less common, we recommend surgical closure of the pulmonary valve during this procedure so as to exclude the residual pulmonary trunk as a possible source of systemic emboli.

Aneurysm of the arterial duct is well described when presenting in infants and children. The case we present were detected by fetal echocardiogram in late pregnancy, indicating the congenital nature of this lesion and excluding widely accepted explanations of the pathogenesis. Spontaneous closure may occur more frequently than the literature suggests. We recommend a conservative approach in asymptomatic patients.

Coexistence of atrioventricular block and Wolff-Parkinson-White syndrome is uncommon, but may occur in children, particularly with certain congenital heart defects. Our study describes 5 children who had pre-excitation and underlying disturbance of atrioventricular conduction. All of these patients had structural heart defects, consisting of discordant atrioventricular connections in three, double inlet left ventricle in one, and a ventricular septal defect associated with straddling of the tricuspid valve in the other. In 4 of the patients, the presence of atrioventricular block was initially masked by the pre-excitation. When the conduction through an accessory pathway is lost, the child may be left with atrioventricular block, which is poorly tolerated in the setting of complex cardiac anatomy. Awareness of this association of Wolff-Parkinson-White syndrome and atrioventricular block is important in understanding the natural history and the management of children with congenital heart defects that are associated with tenuous atrioventricular conduction.

The traditional approach to repair of tetralogy of Fallot involves a right ventriculotomy for closure of ventricular septal defect. During the past two decades, reports of progressive right ventricular dilation and dysfunction, and late occurrence of ventricular arrhythmias, have led investigators to re-evaluate this approach and advocate instead the transatrial-transpulmonary approach, hoping to preserve global right ventricular function. We studied the short term effects on right ventricular function of either of the two approaches through a prospective randomised study, involving two comparable groups of patients operated in the same time frame.

Between June 1993 and February 1994, 40 patients having tetralogy of Fallot with comparable preoperative characteristics, were assigned randomly to each of two groups for surgical correction.

In 20 patients, correction was achieved via the transatrial-transpulmonary route. In the other 20 patients, transventricular correction was the chosen option. Six months after surgery, patients were evaluated clinically, by Doppler echocardiography, cardiac catheterisation, first pass radionuclide angiography and by 24 hours ambulatory electrocardiographic monitoring, taking note of hemodynamics, abnormalities in rhythm, and global right ventricular function.

There were no early deaths or morbidity in either group. Mean immediate postoperative ratio between peak right ventricular and systemic pressures was 0.62 ± 0.22 after transatrial and 0.70 ± 0.007 after transventricular correction. All patients were in functional class I. Six months after surgery the mean ratio between peak ventricular pressures was similar in the two groups (transatrial group: 0.37 ± 0.02, transventricular group: 0.38 ( 0.01), but significantly lower than that measured in the operating room. There were no significant arrythmias in either group. Mean right ventricular ejection fraction was nearly the same in both groups (transatrial group versus transventricular group; 44.83 ± 5.65% versus 42.37 ± 8.70%). Significant global hypokinesia of the right ventricle was documented in three patients, and mild hypokinesia in another three, undergoing repair through the transventricular route while in the group undergoing transatrial repair only one patient had mild hypokinesia.

We conclude that comparable hemodynamic results are obtained on short term follow-up after repair of tetralogy of Fallot by either the transatrial or transventricular route. Although more patients in the transventricular group were found to have global hypokinesia of the right ventricle, longer follow-up is necessary to establish the clinical relevence of these findings.

Morphometric methods were used to study the great arterial trunks in a total of 22 human fetuses and newborn subjects weighing from 1 to 3.8 kg (mean 2.22 ± 0.85 kg). All specimens displayed concordant atrioventricular and ventriculoarterial connections, and all had perimembranous ventricular septal defects. Thirteen different parameters were measured in each specimen, comprising the orifices of the great arterial trunks, ascending aorta, vessels branching from the aortic arch, the aortic isthmus, descending aorta, pulmonary trunk, orifices of the pulmonary arteries, and arterial duct. The values were Compared with patterns of normality established in an earlier study using hearts from 496 human fetuses and neonates weighing form 60 to 5000 g. Minimum square regression analyses were used to study the relationships between fetal and neonatal log body weight in kilograms, and the various cardiometric parameters. We believe that these morphometric data have immediate clinical and surgical applications in the treatment of fetal and neonatal cardiovascular disorders.

Mortality from infectious endocarditis in the general paediatric population had decreased significantly over the last two decades. This is attributable to the increased awareness of the health care team in recognizing the early signs of this once fatal disease such that early antibiotic intervention is possible. Two groups, however, remain at high risk for infectious endocarditis. Advances in cardiac surgery have dramatically improved the prognosis for survival in many children with otherwise fatal congenital heart diseases. This group of children, along with the seriously ill who have central venous catheters in place, are currently at the highest risk for development of infectious endocarditis.1-5

This study was designed to determine, first, the stiffness of the pulmonary arteries and, second, the relationship between the stiffness of the pulmonary arteries and the success rate of balloon angioplasty in patients with complete transposition after an arterial switch operation. Indexes of pulmonary arterial wall stiffness, percent change in the radius during a cardiac cycle and the pressure elastic modulus, were calculated from the pulmonary arterial pressure and radius measured from a cineangiogram. Of the patients, 13 had no significant stenosis, while 25 had significant stenoses and, therefore, underwent balloon angioplasty. In all, we dilated 33 stenotic lesions. In the presence or absence of postoperative pulmonary stenosis, the percent change in the radius of the pulmonary trunk was significantly less than the normal value. The pressure elastic modulus of the trunk and its branches was significantly greater than normal, and increased significantly with time after the operation. In patients without pulmonary stenosis, the systolic pulmonary arterial pressure increased concomitantly with the pressure elastic modulus. Balloon dilation was successful at 17 locations and unsuccessful at 16 locations. The percent change in radius was significantly less for the failures than for the successes. Prior to 3.5 years after the operation, the pressure elastic modulus was < 400 g/cm2 at most locations and balloon dilation was successful in 88%. More than 3.5 years after the operation, the pressure elastic modulus was < 400 g/cm2 in 11 of 15 locations, and balloon dilation was successful in only one location. The increased stiffness of the pulmonary artery may result in increased pulmonary systolic pressure in patients without pulmonary stenosis, and result in a low success rate for balloon angioplasty in patients with pulmonary stenosis.

Anatomic study of 54 autopsied cases of tricuspid atresia revealed mitral valvar anomalies in 9 (16.7%). The morphologic findings in these 9 cases are presented and compared with 45 cases having a normal mitral valvar apparatus. The anomalies included a cleft valve in 2, parachute valve in 2, straddling valve in 3, and a valve with dual orifice in 2.Two of these cases had more than one anomaly. Data from echocardiography and cardiac catheterisation, when available, had failed to diagnose the anomalies during life. The anomalies were more commonly associated with discordant ventriculo-arterial connections (33.3% versus 13.3%, p < 0.01), were associated significantly with a large ventricular septal defect (33.3% versus 4.4%, p < 0.01), and with pulmonary hypertension (33.3% versus 2.2%, p < 0.01). Two of 3 post operative deaths among the 9 were due to hemodynamic consequences arising from the uncorrected mitral valvar anomaly (parachute valve in both ). Diagnosis of these anomalies prior to surgical correction is imperative, as their presence may necessitate repair or replacement of the mitral valve. Failure to do so may result in increased perioperative mortality, congestive cardiac failure and arrhythmias at a later date. Mitral valvar anomalies may be a contributory factor to the early onset of cardiac failure seen in patients with tricuspid atresia undergoing palliative shunts.

Aortic atresia is rare in the setting of a normally developed left ventricle with a ventricular septal defect. In this combination, as far as we know, it has been described only with concordant ventriculo-arterial connections, for which seven one-stage biventricular repairs have now been described. We describe here the case of a 3½-month-old male infant with a similar combination but with discordant ventriculo-arterial connections and severe pulmonary hypertension. It was the right ventricle which achieved normal size in this arrangement, presumably because of the ventricular septal defect. One-stage biventricular correction was accomplished, employing a single pulmonary allograft, aided by massive doses of Prostaglandin El. As far as we know this is the first report of the combination of aortic atresia, discordant ventriculo-arterial connections, a ventricular septal defect and balanced ventricles. We complement our surgical account, nonetheless, with a description of a comparable specimen from our anatomic museum.

We recently reported our experience with exercise stress echocardiography in children with prior history of Kawasaki disease and coronary arterial involvement. Dobutamine stress echocardiography has been used in adults (doses up to 50 μg/kg/min) for diagnosis and risk stratification of coronary arterial disease. To date, no studies have assessed the safety and utility of high-dose dobutamine for the detection of abnormalities of wall motion in children, although low dose dobutamine (5 μg/kg/min) has been used to assess left ventricular function after use of anthracycline in children. Our study group consisted of 10 children known to have Kawasaki disease, and 16 children who had undergone heart transplantation. After baseline transthoracic echocardiographic imaging, dobutamine was infused in an incremental fashion, 10–40 μg/kg/min intravenously, to attain peak heart rate. Heart rate and blood pressure were monitored continuously, and 4 standard echocardiographic images were acquired at each stage of dobutamine infusion and at recovery. Images were digitized, and the left ventricular segments were reviewed to assess wall motion. No patients suffered serious side effects such as arrhythmias or hypotension. Studies were complete in all 26 patients. One study proved positive, with a new abnormality of wall motion identified corresponding to a critical right coronary arterial stenosis. Peak heart rate and blood pressure were similar for the 2 groups. High dose dobutamine stress echocardiography, therefore, is feasible and safe in children, and may detect flow-limiting coronary arterial stenoses noninvasively. The technique may safely be applied to other subsets of children where assessment of ischemia and contractile reserve is warranted.