[Life expectancy in Down syndrome infants and children with congenital heart defects, 1974-1997]. / A Down-szindrómás szívbeteg csecsemók és gyermekek életkilátása 1974-1997 között.

Authors analysed 359 cases with Down's syndrome and congenital heart defects registered between 1974-1997 in Hungary. The total death rate was 19.9% (70 cases). Mortality in the operated group (85 cases) was 10.5% (9 patients), in the non-operated group (274 cases) 22.2% (61 patients). The death rate was lower in the group with early primary reconstruction (2.3%) than in the group with palliation + reconstruction (15.3%), or in the group with only palliative procedure (20%). These results indicate that the life expectancy of infants and children with Down's syndrome and congenital heart disease after early primary reconstructive procedure is the same as in Down syndrome patients without cardiac defects. The prognosis depends on the patient's social circumstances. The results after correct surgical procedure in patients with the same cardiac defect are similar to that of the patients with or without Down's syndrome.

[Rhabdomyoma as a first manifestation of childhood tuberous sclerosis]. / Rhabdomyoma mint a gyermekkori sclerosis tuberosa elsö manifesztációja.

Two dimensional echocardiography seems to be the best diagnostic tool for diagnosis of cardiac tumors. In our practice using 2 dimensional echocardiography in pediatric patients from 1984 11 cardiac tumors were diagnosed and followed-up. Four of them the cardiac rhabdomyomata (one of them diagnosed in utero) was the first manifestation of tuberous sclerosis. We summarized the follow-up data from our patients with cardiac rhabdomyomata. The detection of cardiac tumor in the fetal or infant period is of outmost importance in the early diagnosis of tuberous sclerosis and suggest careful follow-up and management. To the best of our knowledge no report of tuberous sclerosis based on echocardiographic diagnosis of cardiac rhabdomyomata has been described in our country.

[Closure of the patent ductus arteriosus by means of cardiac catheterization]. / Ductus Botalli persistens zárása szívkatéteres módszerrel.

Authors report their results with transcatheter closure of patent arterial duct during a period of 1 year. Thirty patients underwent the procedure at the mean age of 5.22 yrs (range 5 mos-22.3 yrs) and mean bodyweight of 19.1 kg (range 5.8-73 kg). There were 9 males and 21 females. The diagnosis of the patent arterial duct was established by physical examination and noninvasive techniques. The procedures were attempted in all patients above 5 kg bodyweight with patent arterial duct and normal pulmonary artery pressure, irrespective of the shunt-size. All procedures were performed under general anesthesia in one session with the diagnostic cardiac catheterisation. Transcatheter closure was successful in 29 patients. In one patient the device embolized into the left pulmonary artery, snaring was unsuccessful, surgical closure of the patent duct and removal of the device took place uneventfully. Control aortography 15 minutes after the coil placement showed insignificant residual shunt through the patent duct in 8 patients (27%), while echocardiography at 1 month proved complete closure in all. The patients were followed by noninvasive methods. No mortality was observed. Authors emphasize the low risk and cost-effectiveness of the procedure. They stress the importance of the patients selection. Authors applied the technique for the first time and introduced it to the regular patient-care practice in Hungary.

Candida endocarditis in an infant.

Infective endocarditis is a rare disease in infants. A 1-year-old boy with a large Candida albicans vegetation in the right atrium and superior vena cava was operated on successfully. During the newborn period he had had a right transverse colostomy for Hirschsprung's disease. Ten months later a subsequent rectosigmoidectomy and direct anastomosis were performed, but because of peritonitis that followed a leak at the site of the anastomosis parenteral nutrition was needed for 8 weeks. The probable source of Candida was an infected intravenous line.

[Management of aortic recoarctation by transluminal balloon angioplasty in childhood]. / A recoarctatio aortae kezelése transluminalis ballon-angioplasticával gyermekkorban.

Recoarctation balloon angioplasty is an interventional technique for the treatment of restenosis of the aortic isthmus after surgery. At present it is the treatment of choice prior to surgery. Authors report their experience with the first 30 patients in this country. Age less than 1 year at surgery is considered as a risk factor. The diagnosis of recoarctation was made by noninvasive techniques. Balloon angioplasty was performed in one session with the haemodynamic study. Twenty-nine successful procedures were performed, 1 patient only improved after the dilatation. Systemic hypertension decreased in all, but one patient, the diameter of the narrow segment increased in all patients. No mortality was observed. In one patient femoral artery injury developed without definite impairment of the circulation of the lower extremity. It is concluded, that balloon angioplasty is an effective and safe treatment, carries a lower risk for the patient, than surgery, and hospital stay is shorter. The procedure is cost-effective. Therefore, it is proposed for widespread use in the country.

[Total cavo-pulmonary anastomosis in heart surgery for the correction of univentricular heart]. / Teljes cavopulmonalis anasztomózis az univentricularis szív sebészi korrekciójában.

The total cavopulmonary anastomosis is one of the alternative surgical procedures which can be performed in the management of the most complicated congenital heart diseases. It was the first time in Hungary that this surgical management was performed successfully in a girl with univentricular heart, pulmonary valve stenosis, malposition of the great arteries, who was operated on palliative procedure previously.

[Non-invasive and invasive assessment of the function of aortic valve homografts in infancy and childhood]. / Homograft aorta billentyü conduit funkciójának noninvazív és invazív vizsgálata csecsemö- és gyermekkorban.

Study population involved 21 pts with complex congenital heart disease after corrective surgery using homografts between the years 1986 and 1992. Diagnoses included double outlet right ventricle, tetralogy of Fallot, transposition of great arteries, truncus arteriosus, pulmonary atresia with VSD, corrected transposition with pulmonary stenosis, and absent pulmonary valve. Pts age at surgery ranged from 18 days--to 15 yrs mean, 6.7 yrs. Time interval between surgery and diagnostic procedures was 8 days--6 yrs (mean 11 months). All pts were studied by Echo/Doppler. 2 pts by TEE and 3 pts by MRI. 6 pts had cardiac catheterisation. Distal (5) or proximal (1) stenosis was present in 6 cases. The severity and the type of stenosis was correctly identified by noninvasive technique compared to invasive findings. Homograft valve regurgitation was mild (4) or moderate (3). In two pts severe insufficiency was associated to homograft endocarditis. Postoperative residual shunts were found in 8 pts. Homograft stenosis can correctly be diagnosed using Echo/Doppler technique. Distal stenosis was more frequent than proximal. Homograft tends to become insufficient, but severe incompetence did not occur except in endocarditis.

[Development of a nationwide system for harvesting and using homograft heart valves: Establishment of the Hungarian Homograft Bank]. / Homograft szívbillentyük begyüjtési és beültetési rendszerének kifejlesztése. A Magyar Homograft Bank felállítása.

Fourty homografts have been implanted for correction of congenital heart disease in children. Since 1992 cryopreserved homografts are used. Mid-term follow-up data are encouraging: owing to the improved procurement and preservation techniques and the cryopreservation a remarkably enhanced graft-survival is achieved. Preprocessing ischemic time reduction and cryopreservation are the foremost determinants for the freedom of reoperation. Despite the very strict donor selection criteria the capacity of our program (ie. the number of the homografts harvested and processed) seems to be increasing progressively. In order to meet the country-wide need for homografts the Hungarian Homograft Bank is to be established in cooperation with the other cardiac and transplant teams in Hungary. Its objectives, working standards, responsibilities and facilities are outlined.

[Successful surgical correction of persistent truncus arteriosus by an aortic homograft]. / Truncus arteriosus communis sikeres sebészi korrekciója aorta homograft beültetéssel.

After palliative procedure a 2 years old girl with congenital heart disease was corrected with aortic homograft. Truncus arteriosus is a rare congenital heart disease and this is the first successfully corrected case in our country. Using homograft in this age group improves the early and late result of this type of corrective surgery.

[Vascular ring and pulmonary loop: experience with the surgical treatment]. / Sosudistoe kol'tso i legochnaia petlia: opyt khirurgicheskogo lechenia.

Operation was carried out on 143 children (aged from 5 days to 16 years) for tracheobronchial obstruction caused by vascular rings. A double arch of the aorta was found in 50 patients, double arch of the aorta with atresia of the left arch--in 50 patients, right arch of the aorta with ligamentum arteriosum--in 30, left arch of the aorta with an aberrant right subclavian artery--in 10, and a pulmonary loop in 3 patients. Stridor was the main clinical manifestation of the vascular ring. The diagnosis was established by means of two-projection chest radiography and esophagography with a barium meal, bronchoscopy, and angiography. The surgical approach was gained through a posterolateral (left or right) thoracotomy. No deaths occurred during the operation. Two infants died in the postoperative period from a severe bacterial infection of the respiratory tract. On the basis of a rich clinical experience the authors came to the conclusion that the presence of a right aortic arch (except for cases with situs inversus) is always pathognomic because it either causes the appearance of a vascular ring without any intracardiac anomaly, or it may be combined with a congenital heart disease in which a vascular ring is usually not encountered (tetralogy of Fallot, truncus arteriosus communis, etc.). The authors never encountered atresia of a segment of the right aortic arch. The last-named was either patent or absent.

Postoperative echocardiographic study of patients following valvulotomy for critical valvular aortic stenosis.

Authors report on long term follow up of 12 patients operated with critical valvular aortic stenosis. They could perform control echocardiography in 11 patients 4-83 (mean 29) months after valvulotomy. The size and function of the left ventricle was found to be satisfactory, with elevated ejection fraction. The cause of the significant pressure gradient between the left ventricle and the aorta is discussed emphasizing the importance of echocardiography in determining the optimal time for valve replacement or homograft implantation.

Surgical management of pulmonary atresia with intact ventricular septum. Right ventricular size as a guideline for surgical intervention.

61 infants with critical valvular pulmonary stenosis (21 cases) or pulmonary atresia with intact ventricular septum (40 cases) were operated on between 1975-1989 in the Semmelweis University Medical School. Right ventricular volume, area, outflow tract dimension and tricuspid anulus diameter was measured by angiocardiography and echocardiography to evaluate right ventricular size, and to predict the operative outcome using these data. Our results suggested, that if the normalized right ventricular volume was less than 3 ml/m2, the normalized right ventricular area was less than 2.5 cm2/m2, and the normalized right ventricular area was less than 2.5 cm2/m2 and the normalized tricuspid anulus diameter was less than 1.2 cm2/m2/3 only a systemopulmonary shunt procedure is needed. In all other cases pulmonary valvotomy is necessary to decompress the right ventric and to help increase the right ventricular size. Where a different part of the right ventricle is hypoplastic a systemopulmonary shunt procedure is needed too. In the follow up period the right ventricular dimension and tricuspid valve diameter was measured by echocardiography. This noninvasive assessment can predict the preoperative diagnosis, postoperative outcome and demonstrates an adequate growth of the right ventricle after pulmonary valvotomy.