HAPPY NEW YEAR! IT BECOMES INCREASINGLY EVI-dent that it is a truism that years go past quicker as we become older. Ironically, as time goes by faster, we must devote more of our attention to the future. For readers of Cardiology in the Young, as we enter our fifth year of publication, the future is indeed bright. As of this issue you join readers on six continents and scores of countries. As editors, we are most grateful for your continued support. You will notice changes that we are incorporating into this volume, beginning with a complete redesign of our cover and minor changes in the size of the journal. Further substantive editorial changes will be incorporated throughout the year, and we will keep you fully informed as we go. Exciting changes will occur as we augment participation from colleagues in Japan, China and throughout Asia. Please share with us your ideas, as we are amenable to any change or addition that will further improve the quality of Cardiology in the Young.

BEFORE GOING INTO THE SPECIFIC AREAS OF PEDIATRIC cardiology and pediatric cardiac cardiology and pediatric cardiac surgery, an overview of the general health care organization in the Netherlands might be helpful. The Netherlands, a country with about 15 million inhabitants, spent 47 billion Dutch guilders (US$22 billion) for general health care in 1991. This is about 8.5% of the gross domestic product. Of this, 61% was spent on in-hospital costs, 39% on extramural costs, including the financing of organizations that deal with preventive (primary) medicine, such as clinics for infants and school children which were visited regularly. Almost all inhabitants, 99.6%, are covered for the expenses of health care, 60% through the “Sick Fund,” a state-insurance that is obliged for everyone with a yearly income of less than US$30,000. Employers of these people have to deduct an amount of money from the monthly salary to be paid to the “Sick Fund.” In case of unemployment this money is deducted from the amount paid by the social security office. The remaining 40% has some form of private insurance, that covers health care. Both diagnostic and therapeutic approaches as the timing of these are completely similar and independent from the type of insurance that applies for the individual patient.

Plasma leakage in polytetrafluoroethylene grafts arises through complex interactions between the prosthetic physical/chemical microstructure and patient environment. While many of the precipitating factors can be avoided by careful graft-handling and meticulous surgical technique, this unpredictable complication still contributes significant morbidity to shunt procedures. Protein sealing of the graft offers a possible solution to the problem.

To determine the long-term results in patients with critical aortic stenosis who survive initial intervention, and to identify factors which predict prognosis, we studied patients who underwent intervention between 1979 and 1992 for critical aortic stenosis treated within the first three months of life. Patients with a hypoplastic left ventricle or mitral stenosis who were not considered for a biventricular repair were excluded. Follow-up examination included cross-sectional and Doppler echocardiography. All initial and subsequent patient data were reviewed. Of the 64 patients with critical aortic stenosis, 41 (64%) survived more than one month after initial intervention (surgical valvotomy in 39, balloon valvoplasty in two). These survivors constitute the study group. Mild or moderate residual aortic stenosis or regurgitation without further intervention was found in 28 patients at a median duration of 3.1 years (range 0.2–15.0 years). A poor result with re-intervention (n=6) or death (n=7) occurred in 13 patients. The diameter of the aortic valve at presentation was smaller (p<0.02) in patients with a poor result (median 5.5; range 5–15 mm), than in those with a satisfactory result (median 8.0; range 5–10 mm). Significant residual aortic stenosis was present from the time of initial intervention in nine of the 13 patients (69%) with a poor result. No difference was found in the incidence of a duct-dependent systemic circulation, associated cardiac lesions, mechanical ventilation, acidosis or the use of inotropes preoperatively between patients with a satisfactory or a poor late outcome. Of patients with critical aortic stenosis, 64% survived for more than a month after initial intervention. A small aortic valvar diameter at presentation ( 6 mm) and residual stenosis after initial intervention were important determinants of long-term prognosis.

A one-stage repair was performed for correction of the intracardiac malformations associated with coarctation of aorta in five pateints or interrupted aortic arch in eight patients. The ages ranged from four to 294 (median 35) days. The anomalies within the heart were a ventricular septal defect with or without subaortic stenosis (n=7), an aortopulmonary window (n=2), common arterial trunk (n=2), aortic valvar stenosis (n=1) and the TaussigBing anomaly (n=1). Surgery was performed through a median sternotomy employing cardiopulmonary bypass with moderate to deep hypothermia. In terms of the aortic reconstruction, an extended direct anastomosis was performed in 10 patients, while a vascular graft was interposed in three. So long as the aortic cannula did not interfere with the proximal anastomotic site on the aorta, circulatory arrest was avoided. As for surgery within the heart, the ventricular septal defects were closed via the right atrium with myotomy and myectomy if a morphological substrate for subaortic stenosis was confirmed (n=4). There were two hospital deaths (15.4%) due to low cardiac output. In patients who underwent myotomy and myectomy for subaortic stenosis, the postoperative pressure gradient across the aortic valve was negligible. We conclude that surgical results of one-stage repair for the intracardiac malformations associated with interrupted aortic arch or aortic coarctation are reasonable. We suggest that the early relief of obstruction within the left ventricular outflow tract may have played some role in the favorable outcome.

The aim of the study was to determine the optimal size and technique for construction of the systemic-to-pulmonary arterial shunt which will provide suitable pulmonary blood flow in first-stage Norwood palliation for hypoplastic left heart syndrome in neonates. Our clinical experience suggested that an arterial oxygen tension of about 30 mm Hg immediately after cardiopulmonary bypass, with the patients being ventilated at the lowest possible mean airway pressure with an FiO2 of 1.0, provided a suitable pulmonary-to-systemic flow ratio. We also aimed to clarify the characteristics of pulmonary blood flow in accordance with the size of the shunt and the change in the pulmonary vascular resistance in a simplified rigid model of the Norwood procedure. A hole of2.0 mm diameter proved adequate to provide a suitable pulmonary blood flow of 200−300 mlx002F;min in the presence of a pressure gradient of 20−40 mm Hg between the systemic and pulmonary circulations in neonates weighing 3 kg. A short central shunt with a prosthesis of4 mm in diameter produced an excessive flow of pulmonary blood. Our data suggest that using a smaller shunt than that commonly used is necessary to decrease the early and intermediate postoperative mortality. A prosthesis of 3.0 or 3.5 mm in diameter arising from the brachiocephalic artery would be acceptable and can be recommended for first-stage Norwood palliation in small infants, especially in view of the operative difficulties encountered in taking down the shunt at the time of subsequent operations.

Between January 1990 and March 1992, a bidirectional cavopulmonary shunt was performed in seven patients under one year of age for palliation of complex cyanotic congenital heart disease. Five cases had tricuspid atresia with a restrictive ventricular septal defect. One case presented with complete mirror imagery, right-sided heart, double inlet and double outlet right ventricle with pulmonary stenosis. Another case had isomerism of the left atrial appendages with common atrioventricular valve and double outlet right ventricle. All had a pulmonary arterial index greater than 250 mm/m2 (Nakata index). Age and weights before surgery averaged five months (one to 11 months) and 5.6 kg (range 2.8 to 8.5 kg) respectively. Preoperative mean arterial oxygen saturation measured by cardiac catheterization was 60.05±14% and postoperative mean arterial oxygen saturation on room air was 79.85±6.03% (p<0.05). There were no deaths, although postoperative complications occurred in three patients, namely pleural effusion and pneumothorax. Mean follow-up was 10±4.2 months. The cardiothoracic ratio decreased in all cases. On subsequent echocardiographic studies with pulsed Doppler sampling, low velocity systolic-diastolic forward flow was registered in the pulmonary trunk. The velocity of flow increased clearly during inspiration. The bidirectional cavopulmonary shunt can be considered as primary palliation for complex cardiac malformations with restricted pulmonary flow independent of age and body weight.

In 10 neonates with critical aortic stenosis who were treated with balloon dilation, we investigated retrospectively the predictive value for mortality of three echocardiographic parameters: early diastolic mitral valvar diameter, left ventricular end-diastolic diameter, and diameter of the aortic root. Valvoplasty was technically successful in each patient and the peak systolic ejection gradient decreased from 85±42 to 22±13 mm Hg, but clinical success was achieved in only six neonates, with four dying. The diameter of the aortic root was similar in survivors and non-survivors. The mitral valvar diameter and the left ventricular end-diastolic diameter, in contrast, were significantly smaller in non-survivors. The mitral valvar diameter and the left ventricular end-diastolic diameter, in contrast, were significantly smaller in non-survivors. The association of a mitral valvar diameter equal to, or less than, 9 mm with a left ventricular end-diastolic diameter equal to, or less than, 14 mm identified clearly all those who did not survive. In the future, we will recommend patients with these anatomical features for primary Norwood palliation. Neonates with a mitral valvar diameter equal to or greater than 12 mm and a left ventricular end-diastolic diameter equal to or greater than 17 mm, in contrast, are good candidates for balloon dilation. All our patients with these anatomical features survived and are doing well at follow-up (30±14.8 months). Simple echocardiographic measurements, therefore, can help in predicting outcome and choosing the best treatment in neonates with critical aortic stenosis.

There is little information on the indications for balloon dilation angioplasty for aortic coarctation. To determine the indications for balloon dilation in aortic coarctation, we evaluated prospectively the outcome of children treated according to our therapeutic protocol. The protocol included patients with postoperative recoarctation and isolated native coarctation with risks for surgical repair as conditions for which balloon intervention was absolutely indicated, and native coarctation without a significant arterial duct as a condition possibly qualifying for balloon dilation. Patients with duct-dependent coarctation and diffuse hypoplasia of the aortic arch were not considered to require balloon intervention and were treated surgically. We entered 47 patients into this protocol between January 1988 and December 1993. In 21, dilation was performed successfully and provided significant relief of pressure gradients. The peak systolic pressure gradients across the coarcted site in native coarctation and postoperative recoarctation decreased significantly from 40±19 to 13±10 mm Hg (p<0.0001) and 38±24 to 14±75 mm Hg (p<0.01) immediately after the procedure, respectively. Similarly, the diameters of the coarcted site increased significantly from 3.8±1.8 to 6.6±2.43 mm (p<0.0001) and 4.6±1.9 to 6.0±2.0 mm (p<0.005), respectively. Five patients with native coarctation, and one with postoperative recoarctation, developed restenosis during the period of follow-up and were treated successfully with repeat dilation. It may be possible to change the therapeutic strategy for aortic coarctation with advances in the use of balloon dilation. Our protocol for this technique appears to be reasonable, but further evaluation of its indications is needed.

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.

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.

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.

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.

Morphometric methods were used to study hearts with perimembranous ventricular septal defects in 22 human fetuses and neonates weighing from one to 3.8 kg (2.221±0.852 kg). All specimens displayed concordant atrioventricular and ventriculoarterial connections, and all had perimembranous ventricular septal defects. A total of 17 interventricular parameters (nine in the right ventricle and eight in the left) were measured in each specimen. The values were compared with patterns of normality established by Rowlatt and her colleagues in hearts from 83 children ranging in age from birth to 15 years. These patterns were found to be less reliable than our own data from fetal and perinatal hearts from children ranging in age from birth to one month. Our morphological data, when appropriately interpreted, have immediate clinical and surgical applications in the treatment of fetal and perinatal cardiovascular disorders.

Combined antiarrhythmic drug therapy is an occasionally necessary but problematic approach to the child with recalcitrant supraventricular tachycardia. There is little experience with the combined use of amiodarone and class 1-C agents (flecainide, propafenone and encainide) in children. To judge the efficacy and safety of this combination, we reviewed the case notes and results of investigation in all nine children with supraventricular tachycardia who received such therapy between 1984 and 1993. These nine children received combined therapy on 12 occasions. Five were infants with either atrioventricular reentrant tachycardia (n=3) or atrial ectopic tachycardia (n=2), and four were older children with atrial flutter seen after a Fontan procedure. Amiodarone was combined with flecainide on eight occasions, with propafenone on three occasions, and with encainide on one occasion. Both infants with atrial ectopic tachycardia were successfully controlled, but only one of three infants with atrioventricular reentrant tachycardia had successful control on combination therapy. In three of the four patients with atrial flutter, the combination was useful in reducing the number of arrhythmic episodes. Three infants suffered side effects. At electrophysiologic study to judge efficacy, ventricular tachycardia was induced in two patients (one infant and one Fontan patient), necessitating a change in the 1-C agent. One patient had skin rash due to flecainide and was placed on propafenone with success. One Fontan patient died of complications after an elective surgical procedure. No deaths occurred attributable to proarrhythmia. Thus, combined therapy with amiodarone and 1-C agents was found to be safe and fairly effective in children with certain types of intractable supraventricular tachycardia.

Aneurysms of the aortic sinus of Valsalva are rare and generally do not produce symptoms unless the aneurysm ruptures. When rupture occurs, the clinical findings depend to an extent on the site of the aneurysm and the cardiac chamber into which the aneurysm ruptures. We report a case of acute rupture of an aneurysm of the right aortic sinus, which was misdiagnosed as a ventricular septal defect. The clinical condition of the patient deteriorated rapidly, and transcatheter closure of the defect with an umbrella was associated with a fatal outcome.

A 33-year-old woman known to have tetralogy of Fallot and a straddling tricuspid valve had bilateral systemic-to-pulmonary arterial shunts constructed at the age of six months. She subsequently developed infundibular atresia. Antegrade flow of blood from the right ventricle to pulmonary trunk was re-established by percutaneous laser perforation of the outflow tract, later followed by implantation of a stent.

We describe a male adolescent with co-existence of quadricuspid aortic and pulmonary valves and rheumatic heart disease. As far as we know, the presence of quadricuspid aortic and pulmonary valves in the same patient has not previously been described. Furthermore, the presence of rheumatic heart disease in this case makes it a true rarity.

A fistula from the pulmonary artery to the left atrium is a very rare congenital anomaly. The communication, almost exclusively arising from the right pulmonary artery, may give rise to a variety of symptoms with cyanosis as the predominant sign. To our knowledge only seven neonates have been reported with this anomaly, and five of them died postoperatively. We present another two neonates with such a fistula which was closed by simple ligation using a transsternal approach and cardiopulmonary bypass. Both patients had an uneventful postoperative course and are alive and well at the age of two and three years, respectively.

A neonate presenting with persistent pulmonary hypertension and severe hypoxemia was treated with nitric oxide administered into a head-box. Improvement of clinical, biochemical and echocardiographic parameters were observed during therapy, suggesting an acute decrease of pulmonary arterial pressure. It is feasible to administer nitric oxide to non-ventilated neonates provided all recommended safety precautions are observed