Fetal cardiology in the Czech Republic: current management of prenatally diagnosed congenital heart diseases and arrhythmias.

Reliable diagnosis of congenital heart defects and arrhythmias in utero has been possible since the introduction of fetal echocardiography. The nation-wide prenatal ultrasound screening program in the Czech Republic enabled detection of cardiac abnormalities in 1/3 of patients born with any congenital heart disease and up to 83 % of those with critical forms. Prenatal frequency of individual heart anomalies significantly differed from the postnatal frequency. Fetal isolated complete atrioventricular block and supraventricular tachycardia may lead to heart failure and are important causes of fetal mortality. The regression of heart failure was achieved by a conversion to the sinus rhythm in the supraventricular tachycardia and by increase of ventricular rate in the complete atrioventricular block.

Nitrogen balance, 3-methylhistidine excretion, and plasma amino acid profile in infants after cardiac operations for congenital heart defects: the effect of early nutritional support.

OBJECTIVE: The objective of this study was to evaluate the effect of nutritional support on proteolysis and plasma amino acid profile in infants early after cardiac operations for congenital heart defects. METHODS: Thirty-seven patients, 2 to 12 months old, were randomized on postoperative day 1 for 24-hour isocaloric metabolic study. Group STANDARD (18 patients) received glucose as the maintenance fluid, and group PN (19 patients) received glucose and crystalloid amino acid solution at a dosage of 0.8 +/- 0.1 gm/kg per day. The nonprotein caloric intake in the two groups was 25 +/- 15 and 33 +/- 9 kcal/kg, respectively (p = not significant). RESULTS: The nitrogen balance was markedly less negative in group PN than in group STANDARD (-114 +/- 81 vs -244 +/- 86 mg/kg, respectively, p = 0.001). There was a highly significant inverse correlation between the nitrogen balance and urinary 3-methylhistidine excretion in both groups, but the muscle proteolysis was blunted more effectively in patients receiving amino acids. Concentrations of the plasmatic branched-chain amino acids, alanine, glycine, and proline, decreased significantly in group STANDARD but not in group PN on postoperative day 2. Glutamine and threonine levels declined significantly on postoperative day 2 in both groups. Low levels of arginine were observed in our patients before operation and in the early postoperative period. The amino acid concentrations normalized on postoperative day 7 in all patients. CONCLUSION: Significant proteolysis and hypoaminoacidemia were observed in infants early after cardiac operations. This hypercatabolic response was blunted by parenteral nutritional support.

Correction of aortico-left ventricular tunnel during the first day of life.

Two critically ill newborns with aortico-left ventricular tunnel and severe heart failure were operated on at six and 14 hours after birth. The diagnosis was established clinically by the auscultatory finding of systolic and diastolic murmurs and by two-dimensional and Doppler echocardiography. In the first newborn, the left aortic sinus was connected with the left ventricle below the aortic valve by an aneurysmatically dilated tunnel. In the second patient, the tunnel connected the right aortic sinus and the left ventricle. The repair was performed under deep hypothermia, total hemodilution, and cardiopulmonary bypass. The tunnel was closed with two patches of Gore-Tex on the aortic and ventricular orifices. Both children are free from symptoms and are developing normally 10 and 8 months after repair.

Congenital absence of aortic and pulmonary valve in a fetus with severe heart failure.

A case of congenital absence of both aortic and pulmonary valves with severe heart failure detected prenatally by cross-sectional and pulsed and colour Doppler echocardiography is reported in small for gestational age male fetus in 17th week of gestation. Additional double outlet right ventricle, hypoplastic left ventricle, and ventricular septal defect, as well as multiple extracardiac anomalies, were found by prenatal echocardiographic investigation and confirmed by necropsy examination. Retrograde diastolic Doppler waveforms retrieved from pulmonary artery, aorta, and umbilical arteries revealed massive insufficiency throughout both the great arteries, which eliminated diastolic placental perfusion, documented by absent anterograde diastolic flow in the umbilical vein. These prenatal echocardiographic findings may contribute to an understanding of the mechanism of rapid and progressive heart failure and growth retardation in the fetus. Severe cardiac failure may explain why congenital aplasia of both the aortic and the pulmonary valves has not been described postnatally, and only two fetal cases revealed by necropsy have been published.

Pulmonary blood supply in bidirectional cavopulmonary anastomosis with pulsatile pulmonary blood flow: quantitative analysis using radionuclide angiocardiography.

OBJECTIVE: To establish a non-invasive method for quantitative analysis of pulmonary perfusion in patients with bidirectional cavopulmonary anastomosis (BCPA) and sources of pulsatile blood flow. The method should quantify left to right lung flow ratio and relative contribution of BCPA and sources of pulsatile blood flow to perfusion of each lung. DESIGN: A pilot study using radionuclide angiocardiography for quantitative analysis and for visualisation of cavo-caval collaterals. No criterion standard is available. SETTING: Tertiary care centre, ambulatory and hospital inpatient care. PATIENTS: Consecutive sample of 18 patients with BCPA and sources of pulsatile blood flow. RESULTS: In eight patients (44%) cavocaval collaterals prevented quantitative analysis. In 10 patients without cavo-caval collaterals, BCPA provided 42.3 (SEM 3.4)% of total pulmonary blood flow. From the total BCPA flow, 67.2 (4.3)% was directed to the ipsilateral lung. This lung received only 16.5 (3.3)% of all the blood from sources of pulsatile blood flow. The blood flow to the lung at the side of BCPA accounted for 35.3 (1.7)% of the total pulmonary blood flow. CONCLUSIONS: Radionuclide angiocardiography allows the quantitative analysis of pulmonary blood supply in BCPA with sources of pulsatile blood flow except in patients with cavo-caval collaterals or bilateral BCPA. Non-pulsatile flow from BCPA is mainly directed to the ipsilateral lung, whereas pulsatile flow to the contralateral lung. Total perfusion of the ipsilateral lung is less than the perfusion of the contralateral lung.

Long-term ventricular performance after intra-atrial correction of transposition: left ventricular filling is the major limitation.

OBJECTIVE: To establish the incidence of systolic and diastolic dysfunction of the right and left ventricle in a large cohort of patients after Mustard or Senning operations and to assess changes in the incidence on long term follow up. DESIGN: Postoperative case-control study using radionuclide ventriculography. Ejection fractions, peak filling rates, rapid filling periods and fractions, slow filling periods and fractions, and atrial contraction periods and fractions were studied. SETTING: Tertiary care centre, ambulatory and hospital inpatient care. PATIENTS: A convenience sample of 153 patients studied at median age of 6.9 years (median 4.4 years after surgery). In 99 cases another study was available at a median age of 15.3 years (median 13 years after surgery and 8.8 years after the first study). RESULTS: Respective incidences of dysfunction in the first and the second study were as follows: ejection fraction-right ventricle 7.8% and 8.1%, left ventricle 7.2% and 10.1%: peak filling rate-right ventricle 0% and 4.2%, left ventricle 14.3% and 29.5% (p < 0.05); rapid filling period-right ventricle 18.3% and 11.6%, left ventricle 30.2% and 30.5%; slow filling period-right ventricle 4.8% and 3.2%; left ventricle 11.9% and 23.2%; atrial contraction period-right ventricle 0.8% and 4.2%, left ventricle 15.1% and 26.3%; rapid filling fraction-right ventricle both 0%, left ventricle 82.5% and 79.0%; slow filling fraction-right ventricle 0.8% and 4.2%, left ventricle 37.3% and 30.5%; atrial contraction fraction-right ventricle both 0%, left ventricle 79.4% and 71.6%. CONCLUSIONS: The incidence of systolic ventricular dysfunction is 8% (right ventricle) and 10% (left ventricle) 13 years after surgery, without a significant increase over the eight year follow up. Diastolic filling is abnormal in up to 80% of patients and left ventricular peak filling rate deteriorates with time.

Successful reoperation for severe left bronchus compression after repair of persistent truncus arteriosus with interrupted aortic arch.

Signs of the left bronchus compression, caused by aneurysmatic dilatation of the aortic root with severe aortic regurgitation, occurred 5 months after repair of the truncus arteriosus with interrupted aortic arch in an 85-day-old infant. At reoperation the dilated ascending aorta was replaced with a 14-mm Dacron tube. The aortic valve was replaced with an 18-mm Carbomedics valve. Compression of the left bronchus and the right pulmonary artery were released. The right pulmonary artery was enlarged with a pericardial patch and the original homograft was replaced with a new one. The patient remains in good clinical condition 2 years later.

Operative treatment of infective endocarditis in children.

A retrospective review of 11 children, aged 2 months to 15 years, demonstrates the feasibility of surgical treatment for infective endocarditis in childhood. Except for one case of perinatal infection, in all instances the infective endocarditis was a complication of a congenital heart defect. As medical treatment was not successful, surgery was indicated. Debridement of infected tissue and repair of the congenital heart defect was performed. There were no septic complications postoperatively although 8 patients were operated upon during the active stage of infection. One 2-month-old child did not survive excision of an infected tricuspid valve. The follow-up period of 8 years to 5 months (median 39 months) showed a good haemodynamic result (NYHA class I) in the remaining 10 patients. This included 4 patients with prosthetic valves.

Intermediate to late results of surgical relief of vascular tracheobronchial compression.

Since 1979, a total of 69 infants and children aged 0.1 to 11.9 (median 0.7) years required surgical intervention for: double aortic arch (26), anomalous origin of innominate artery (26), right aortic arch with left ligamentum arteriosum (9), pulmonary artery sling (5), retroesophageal right subclavian artery (3). Before operation, the morbidity was high: 84% of children were symptomatic in the first trimester of life with 24 patients requiring mechanical ventilation. Seven children had an accompanying heart defect. Left thoracotomy was the preferred approach. There were 2 early (asphyxic brain damage, postoperative pneumonia) and 2 late (tracheomalacia, complex heart defect) deaths. Five patients (2 originally operated elsewhere) needed reoperation for persistent symptoms. All 65 survivors are well 1 month to 11.9 (mean 3.9 +/- 3.62) years after surgery. Tracheobronchoscopy, magnetic resonance imaging, and lung function testing were helpful for postoperative evaluation. Minor tracheal compression was revealed in 4 patients despite their good clinical condition.

Bidirectional Glenn followed by total cavopulmonary connection or primary total cavopulmonary connection?

OBJECTIVE: Analysis of mortality and morbidity of patients treated by primary total cavopulmonary connection (TCPC)-Primary correction group, and comparison to patients treated by bidirectional Glenn (BDG) followed by total cavopulmonary connection-two stage TCPC group. METHODS: Retrospective study of 123 consecutive patients who underwent 144 different types of cavopulmonary connections between 1987-1995: bidirectional Glenn 59, HemiFontan operation 10, primary total cavopulmonary connection 54, and total cavopulmonary connection completion after previous bidirectional Glenn 21. Important preoperative risk factors: age, systemic outflow obstruction, pulmonary venous obstruction, pulmonary artery (PA) hypoplasia (McGoon ratio), PA stenosis/distortion, PA mean pressure, PA vascular resistance, atrioventricular valve regurgitation, systolic and diastolic ventricular function and ventricular hypertrophy were re-evaluated according to Texas Heart Institution Scoring System in both groups. Three different preoperative risk groups were established: low risk, score (0-3) moderate risk (4,5) and high risk score (> or = 6). RESULTS: Mean age was 85.2 month (range 16.1-229.5 months) and 106.6 months (range 42.6-178.9 months) in primary correction group and two stage TCPC group, respectively. Diagnosis was similar in both groups, majority having univentricular heart or hypoplastic one ventricle. Initial palliation (pulmonary artery banding, modified aortopulmonary shunt, coarctation repair etc.) was performed in 38 (70.3%) patients of primary correction group and in 12 (57.1%) two stage TCPC group. The mortality was 7.4% (4 out of 54) and 14.2% (3 out of 21) for primary correction and two stage TCPC group, respectively. There were two take down in the primary correction group. There was no late death in either group. Operative data and postoperative morbidity did not statistically differ in both groups. CONCLUSION: Until 1993 bidirectional Glenn was preferred to primary total cavopulmonary connection for high risk patients. High mortality 14.2% patients of two stage TCPC group vs. 7.4% of primary correction group in patients with the same preoperative hazard led us to change our policy. We now prefer primary TCPC for all patients with functional single ventricle and surgically correctable major associated defects. High risk patients undergo TCPC with fenestration. Patients not suitable for TCPC undergo either HemiFontan operation or some type of initial palliative procedure.

Results of primary and two-stage repair of interrupted aortic arch.

OBJECTIVE: Early results of primary and two-stage repair of interrupted aortic arch have improved. Experience with different surgical approaches should be analysed and compared. METHODS: Forty neonates and infants with interrupted aortic arch underwent primary repair (19 patients) or palliative operation (21 patients). Twenty (50%) patients were followed-up for 5.1+/-4.3 years. All patients were regularly examined with the aim of determining clinical development, presence of residual lesions or complications and need for re-intervention. Aortic arch and the left ventricular outflow tract growth were assessed by echocardiographic examination. Data from hospital and outpatient department records were analysed. RESULTS: The early mortality was 61.9% after palliative operations and 36.8% after the primary repair. Presence of complications (P < 0.001), earlier year of surgery (P < 0.01), bad clinical condition and acidosis (P < 0.05) represented statistically significant risk factors for death in the whole series. In seven (87.5%) out of eight early survivors, after the initial palliative operation, closure of ventricular septal defect and debanding were done, and in three (37.5%) patients, re-operation for aortic arch obstruction was also required. Out of 12 patients, after the primary repair, one required early re-operation for persistent left ventricular outflow tract obstruction and two needed late re-intervention for left bronchus obstruction. In three (25%) patients, after the primary repair, left ventricular outflow tract obstruction with a maximal systolic pressure gradient higher than 30 mmHg developed. At present, all 20 early survivors are alive. Five patients, after palliative operation, are in NYHA class 1, but in three patients, who are in class III or IV, the outcome is influenced by severe complications. All patients after the primary repair are in class I or II. CONCLUSIONS: Our experience confirmed better results after the primary repair of interrupted aortic arch, which was associated with lower mortality, prevalence of severe complications and need for re-intervention. Higher prevalence of subaortic stenosis after primary repair could be explained by patient selection early in our experience. We recommend the primary repair of interrupted aortic arch and associated heart lesions in neonates, however, in unfavourable conditions an individualised surgical approach with initial palliative surgery should be considered.

Phospholipid composition of myocardium in children with normoxemic and hypoxemic congenital heart diseases.

Samples of myocardial tissue were obtained during cardiac surgery from children operated for different types of normoxemic and hypoxemic congenital heart diseases. The phospholipid composition was analyzed by thin layer chromatography. The concentration of total phospholipids (PL), phosphatidylcholine and phosphatidylethanolamine (PE) was found lower in atrial tissue of both normoxemic and hypoxemic groups in comparison with the ventricles. When comparing the difference between hypoxemic and normoxemic defects, hypoxemia was found to increase the concentration of total PL, PE and phosphatidylserine in ventricles and total PL and PE in the atria. The increased level of particular phospholipid species may represent adaptive mechanisms to hypoxemia in children with congenital heart diseases.

Late complications and quality of life after atrial correction of transposition of the great arteries in 12 to 18 year follow-up.

BACKGROUND: Most adolescents and young adults born with transposition of the great arteries and survivors of Mustard operation are alive today and are leading ordinary lives. How far is the quality of their life influenced by residual findings? METHODS: We have analysed the oldest group of 177 children operated on with the Mustard procedure during 1979-1984 from our total experience of 609 corrections of transposition of the great arteries until 1996. In the long-term follow-up 12 to 18 years after Mustard operation the clinical investigation, 24 hours ECG Holter monitoring, 2D and colour flow Doppler echocardiography, radionuclide angiocardiography and exercise testing were performed. RESULTS: Basic sinus rhythm had 61% of 137 survivors, sinus node dysfunction had 51%. All but 4 patients needed no antiarrhythmic therapy. There were 10 sudden deaths. Severe tricuspid valve regurgitation developed in 15.9% of children, and decreased to 6% after intensive medical treatment. The right ventricular systolic dysfunction was found on radionuclide ventriculography in 8% of children after surgery. The left ventricular ejection fraction was subnormal in 10% and left ventricular diastolic function expressed by rapid filling fraction was subnormal in 80% of patients and may cause inability to increase cardiac output at exercise The obstruction of caval veins was found in 9%. There were neurological complications in 13.3% (10% already preoperatively) but severe brain damage was found in 4.4% only. CONCLUSIONS: Exercise tolerance with the maximum oxygen consumption over 30 ml/kg/min in 96% of investigated children suggests the good ability and full efficiency for recreation sports. Most survivors (84%) are reported to lead ordinary lives and function in NYHA class I.

Individualized repair of the left atrioventricular valve in spectrum of atrioventricular septal defect.

From September 1977 to October 1995, 287 patients with atrioventricular septal defect (AVSD) aged from 2 months of 21 years underwent total repair in Kardiocentrum in prague. In 97 patients complete, in 20 transitional and in 170 patients partial form of AVSD was present. The repair consisted of closure of the defect and individually modified reconstruction of two atrioventricular (AV) orifices. In cases with a common orifice a two-patch technique was used. Fixation of undivided anterior and posterior common leaflets to patches in an appropriate level was essential in combination with complete closure of the cleft. Incomplete closure of the cleft was performed if potentially stenotic morphology was present. Commissuroplasty with pladgeted mattress stitches was done in patients with dilated annulus and commissuroplasty with a single stitch was performed if the annulus was not dilated. The methods were similar in cases with two AV orifices. The AV valve repair was difficult in the presence of severe regurgitation in valves with potentially stenotic morphology. Of the 287 operated patients 26 (9.1%) died during the early postoperative period. Mortality was 19.6% in the complete form and 3.7% in the partial and transitional forms. The mortality depended on morphology of the left atrioventricular valve. Potentially stenotic valvar morphology represented an important risk factor for death and reoperation. It was necessary to reoperate on 18 (6.3%) patients for significant "mitral" valve regurgitation. Reconstruction of a competent left AV valve is the most important step of AVSD repair which must always be modified according to individual morphological and functional abnormalities.

Primary repair of interrupted aortic arch and associated heart lesions in newborns.

Primary repair of interrupted aortic arch and associated heart lesions was performed in 13 patients aged from 1 to 85 days. The surgery was performed through the midline sternotomy approach in extracorporeal circulation and deep hypothermia. Hypothermic circulatory arrest at 14 to 19 degrees C was used for reconstruction of the aortic arch. In all patients it was possible to perform a direct anastomosis between the ascendent and descendent aorta. At the same time closure of the ventricular septal defect was performed in 11 patients, closure of the atrial septal defect in 4, correction of persistent truncus arteriosus in 3, resection of subaortic stenosis in 2, arterial switch repair of transposition of the great arteries in 1, correction of double outlet right ventricle in 1 and patch closure of aortico-pulmonary window in 1 patient. Three (23.1%) newborns died in the early postoperative period: two from sepsis and one from multiple organ failure. Ten patients (76.9%) were followed up for 1 to 29 months postoperatively. All of them are in very good condition with a nonrestrictive aortic anastomosis. Primary one-stage repair of interrupted aortic arch and associated heart lesions is preferred to the two-stage repair in all newborns with this critical congenital heart disease.

[Echocardiography methods in the diagnosis of cardiomyopathies in children]. / Echokardiographische Methoden in der Diagnostik von Kardiomyopathien bei Kindern.

We investigated 59 children with cardiomyopathy (CMP) by echocardiographic methods (ECHO). 29 children with dilated CMP showed typical enlargement of the left ventricle 159 +/- 31%, xxx; (% of normal value +/- SD) and of the left atrium (143 +/- 27%, xxx). Systolic and diastolic function of the left ventricle were decreased, systolic change in size of the left ventricle % delta D = 16 +/- 7%; xxx; systolic change in area of the left ventricle % delta A = 20 +/- 8%; xxx; maximum filling rate of the left ventricle in the phase of quick filling E max = 0.37 +/- 0.09 m. sec-1, xxx; the percentage of filling in the first third of diastole 0.33 DA = 37 +/- 9%, xx. Mitral insufficiency was found by us in 81% of children. In 25 children with hypertrophic CMP the size of left ventricle was decreased (81 +/- 13%, xxx). Asymmetric septal hypertrophy was found by us in 69% of children, symmetric hypertrophy in 27%, local hypertrophy of the apex in 4%. Systolic function of the left ventricle was increased (% delta D = 47 +/- 10%, xxx; % delta A = 70 +/- 10%, xxx). Diastolic dysfunction manifested itself by reduced E max (0.71 +/- 0.19 m.sec-1, xx) and 0.33 DA (45 +/- 12%, x). Mitral insufficiency was found in 54% of the children. In the case of the 5 children with restrictive CMP an extreme enlargement of left atrium (195 +/- 41%, xxx) was typical, as well as diastolic dysfunction of the left ventricle (0.33 DA = 67 +/- 6%, x).(ABSTRACT TRUNCATED AT 250 WORDS)

[Intraumbilical therapy of fetal supraventricular tachycardia]. / Intraumbilikální lécba supraventrikulární tachykardie plodu.

The supraventricular tachycardia represents the life threatening disease, which may cause severe heart failure or even during foetal life. The authors present case report of the foetus aged 23 weeks of gestation in whom the supraventricular tachycardia was resistant to standard transplacental treatment by using digoxin and sotalol. The successful rhythm conversion was achieved by intracordal infusion of amiodarone. Further uncomplicated course of pregnancy reached term and healthy boy was subsequently born without having additional psychomotoric complications.

[The infundibular septum in congenital heart defects. Morphologico-angiocardiographic study of the double-outlet heart ventricle]. / Infundibulární septum u vrozených srdecních vad. Morfologicko-angiokardiografická studie dvojvýtokové pravé komory.

The authors investigated the infundibular septum in 40 archival heart preparations with a double outlet right ventricle. They compared the post-mortem findings with the previous angiocardiographic examination and the skiagram of the preparation made post mortem. They present also the corresponding findings obtained by echocardiography. The infundibular septum was in all instances the dominating intracardiac structure influencing the direction of the blood flow from the ventricles into outflow tracts in the direction towards the large arteries. In 15 instances the infundibular septum was shifted in an anterosuperior direction beneath the orifice of the pulmonary artery and it derived the blood current from the left ventricle through the defect in the ventricular septum into the aorta. The restricted blood flow through the aorta led to its hypoplasia, which in 11 instances was associated with coarctation or interruption of the aorta. In seven instances of posteroinferior shift the infundibular septum was straight. These cases were associated with coarctation or interruption of the aorta. In ventricular septum and with stenosis of the pulmonary artery. Based on these findings the authors assume that the shift of the infundibular septum in a double outlet right ventricle has a decisive position as regards haemodynamic manifestations of the defect and in the development of outflow tracts and large arteries. Last not least, information on the infundibular septum is also important for surgery of the defect.

[Can atrial septal defects be reliably diagnosed by echocardiography?]. / Lze echokardiografií spolehlive diagnostikovat defekt sínového septa?

The diagnostic reliability of echocardiography (ECHO) was analyzed in a group of 179 children operated on account of an atrial septal defect (ASD) and partial anomalous pulmonary venous drainage (PAPVD). A total of 223 individual types of ASD and PAPVD and their mutual combinations were proved on operation. In all patients indirect signs of a left-to-right shunt at the atrial level was proved. The sensitivity and specificity of ECHO examinations in the ASD type fossa ovalis was 0.97 and 0.80 resp., in ASD type sinus venosus it was 0.47 and 0.98, in PAPVD 0.38 and 0.99. In both types of ASD type sinus coronarius assessed on operation the ECHO diagnosis was correct. The application of colour Doppler technique increased the reliability of the ECHO examination. The accuracy of ECHO in ASD and PAPVD depends indirectly on age, body weight and body surface. No relationship was proved between reliability of ECHO diagnosis of the defect and the magnitude of the left-to-right shunt assessed by radiocirculography and the size of the defect assessed during operation. In no instance the diagnostic inaccuracy affected the surgical approach and the result of the operation. Despite certain diagnostic pitfalls, it is therefore possible to operate, using the comprehensive non-invasive examination procedure, children with these anomalies in our department without previous cardiac catheterization and angiography.

[Reoperation in congenital heart defects after primary surgery in the neonatal period and infancy]. / Reoperace vrozených srdecních vad po primárních operacích v novorozeneckém a kojeneckém veku.

BACKGROUND: Reoperations in cardiac surgery of congenital heart defects represent not only the difficult technical problem but also a prognostic one. They are demanding reconstructive procedures with broad spectrum of hazards and are definitely cumulating the operative risk. METHODS AND RESULTS: At the Pediatric Kardiocentrum, University Hospital Motol, Prague, there were 2250 children operated on for congenital heart defects in the first year of life and followed up during 1969-1996. There were 1701 primary corrections (75%) and 549 palliative procedures (25%). After operation 1912 survivors (85%) were followed-up to 25 years. Later on 524 children (27%) were reoperated with 672 procedures. There were three main reasons for reoperation: 1. Staged procedures of complex heart defects in 477 children. 2. One hundred seventy four reoperations for residual defects were performed after primary procedure in 123 infants. There were 11 corrections of residual intracardiac shunts and 120 repairs of residual stenoses. There were 58 repairs of recoarctation, 40 pulmonary artery reconstructions after arterial shunts and 43 reoperations on valves for restenosis or worsened regurgitation. 3. Other 21 extracardiac complications of primary operation were solved by pacemaker implantation (8), plication of paralysed diaphragm (6), chylothorax (3) and other (4). The causes of reoperations represent 7 main categories: 1. Solid scars, adhesions and fibrous bands cause stenoses. 2. Palliative procedures influence positively the hemodynamics, but compromise the development and growth of myocardial wall. 3. Synthetic materials are nor growing but produce obstructions. 5. Incomplete primary correction and technical errors. 6. Iatrogenic injury of the phrenic nerve or the bundle of His. 7. False diagnosis. The hazards of reoperations are: 1. High risk of resternotomy with catastrophic bleeding. 2. Difficult cannulation for heart-lung bypass, and problematic induction of cardioplegia. 3. Difficult dissection of all structures with the high risk of bleeding on the site of aorta, lung hilum or coronary arteries. 4. Difficult left heart decompression and defibrillation of the heart. 5. Paralysis of the diaphragm. CONCLUSIONS: Staged procedures are the major source of reoperations. Their volume does not change much and it is difficult to influence it because still a large number of complex heart defects are operated every year. On the contrary the residual or recurrent defects as well as a number of further complications can be influenced by preventive measures during primary operation.