Atrioventricular conduction time in fetuses assessed by Doppler echocardiography.

We performed measurement of mechanical atrioventricular conduction time intervals in human fetuses assessed by Doppler echocardiography and provided reference values. We found that atrioventricular conduction time interval was prolonged with gestational age and decreased with increasing fetal heart rate. No correlation between gestational age and heart rate was found. Using normal limits established by this study, mechanical atrioventricular interval >135 ms in the 20(th) week and/or >145 ms in the 26(th) week of gestation could be suspected of having the first-degree AV block. We compared reference values with fetuses of mothers with anti-SSA Ro/SSB La autoantibodies, being in risk of isolated congenital heart block development. One of 21 fetuses of mothers with positive autoantibodies was affected by prolonged atrioventricular interval according to the established limits, with sinus rhythm after the birth.

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.

Coagulation profile and liver function in 102 patients after total cavopulmonary connection at mid term follow up.

OBJECTIVE: To examine coagulation factors and liver function test abnormalities in patients after total cavopulmonary connection (TCPC). DESIGN: Cross sectional study comprising clinical and echocardiographic evaluation, and biochemical and coagulation profile screening. SETTING: Tertiary referral centre. METHODS: 102 patients aged 4-24 years (median 10 years) at one to eight years (median five years) after TCPC were examined. All patients were maintained on a low dose of aspirin. 96% of patients were in a good clinical condition (New York Heart Association class I or II). No intracardiac thrombi were detected on echocardiography and ventricular function was good in 91% of patients. RESULTS: Total bilirubin was increased in 27% and gamma glutamyltransferase in 54% of patients. Serum total protein, albumin, and prealbumin were normal in almost in all patients. Compared with the control group, patients after TCPC had significantly lower fibrinogen, factor V, factor VII, and protein C concentrations, prolonged international normalised ratio, and increased antithrombin III concentration. Factor V concentration was abnormally decreased in 35%, factor VII in 16%, and protein C in 28% of patients. Antithrombin III was increased in 23% of patients. Factor VII, factor V, protein C, and antithrombin III correlated significantly with serum prealbumin. There was also a significant correlation between procoagulant factor VII and both anticoagulant protein C and antithrombin III. CONCLUSIONS: Almost half of patients after TCPC had laboratory signs of mild cholestasis. Decreased liver synthesis of procoagulant and anticoagulant factors was observed but overall coagulation homeostasis appeared to be in balance in this selected group of patients with a good clinical outcome.

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.

Long term results of percutaneous balloon valvoplasty of congenital aortic stenosis: independent predictors of outcome.

OBJECTIVE: To evaluate long term results and independent predictors of outcome of aortic valvoplasty. DESIGN: Retrospective follow up study. Independent predictors of outcome identified by multiple logistic regression. SETTING: Tertiary referral centre. PATIENTS: 269 consecutive patients treated at the median age of 8 months (0-23 years): 80 (30%) under 4 weeks, 59 (22%) between 4 weeks and 1 year, and 130 (48%) over 1 year. The follow up period was up to 14.8 years (median 5.3, in survivors 6.4 years). INTERVENTIONS: Percutaneous balloon valvoplasty with mean (SD) balloon to annulus ratio 0.97 (0.08). MAIN OUTCOME MEASURES: Restenosis > or = 70 mm Hg, grade 3 aortic insufficiency, cusps disruption, surgery, death, and valvoplasty failure (significant restenosis or insufficiency or surgery or death). RESULTS: The mortality rate was 10.4% (n = 28), the restenosis rate was 16.7% (n = 45), significant insufficiency developed in 22.3% (n = 60), surgery was needed in 20.1% (n = 54), and "valvoplasty failure" occurred in 41.6% (n = 112) patients. Mean (SEM) survival probability 14.4 years after the procedure was 0.89 (0.02) and mean (SEM) probability of surgery-free survival was 0.50 (0.08). The independent predictors were as follows. For restenosis: small aortic annulus; for cusp disruption: large aortic annulus; for insufficiency: bicuspid aortic valve; for need for surgery: bicuspid aortic valve; for death: small aortic annulus, low left ventricular shortening fraction, and low sequential number of the valvoplasty; and for valvoplasty failure: small aortic annulus, bicuspid aortic valve, and high grade of mitral insufficiency. CONCLUSION: Independent predictors of unfavourable outcome are small aortic annulus, bicuspid aortic valve, poor function of left ventricle or mitral valve, and limited operator experience.

[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.

Are cyanosed adults with congenital cardiac malformations depressed?

OBJECTIVE: To assess the incidence of depression, and the ability to interact socially, in adult patients with chronic cyanosis and congenital cardiac malformations. DESIGN: Prospective study of consecutive patients. SETTING: Single institution, tertiary referral centre. PATIENTS: Between 1993 and 2000, we assessed 76 patients with congenital cardiac malformations and persistent cyanosis, having a median age of 36.5 years, with a range from 19 to 64 years, at the time of referral. Female patients accounted for just under half (48.6%) of the sample. Just under two-fifths of the cohort (39.5%) had functionally univentricular cardiac anatomy, while 14.8% had tetralogy of Fallot with pulmonary atresia and aorto-pulmonary collateral arteries, and 17% had the Eisenmenger syndrome. During the period of follow-up, 17 (22.4%) of the patients died. ASSESSMENT: We used clinical interviews and non-invasive assessment, employing Zung's questionnaire which provides a scale for the self-rating of depression. On this scale, a score above 50 points is indicative of depression. RESULTS: Of the survivors, 32 (54%) completed the self-rating questionnaires. Of these, 20 responders (63%) considered that they lead full lives, including sexual activities, while 26 (81%) had never harboured suicidal thoughts. Depression was diagnosed in 11 responders (34%), with a mean score of 66.9, standard deviation of 8.7, and a range from 53 to 89. The remaining 21 patients (66%) were without signs of depression, scoring a mean of 41.5, with standard deviation of 5.5, and a range from 35 to 46. Depression was associated with older age (40.5 years versus 33.5 years, p = 0.01), worse functional state in the classification of the New York Heart Association (2.95 versus 2.48, p = 0.03), and unemployment (p < 0.0001), but independent from the severity of cyanosis, the level of the haematocrit, the saturation of oxygen, or previous surgical treatment. CONCLUSIONS: To our knowledge, this is the first evidence suggesting a relatively high incidence of depression in adults with congenital cardiac malformations and persistent cyanosis. Larger, multi-centric studies will be needed to confirm or refute these findings.

[Adult patients after surgery of ostium primum type of atrial septal defects in childhood: echocardiography study]. / Dospelí pacienti po operaci defektu septa síní typu ostium primum v detském veku: echokardiografická studie.

The authors examined, using transthoracic and transoesophageal echocardiography, 36 adult patients (15 men) aged 22 +/- 3.1 years (18-29 years) who were operated 12.2 +/- 3.7 years previously on account of a defect of the atrial septum type ostium primum. In these patients no other congenital cardiac defect was present. In addition to closure of the defect in the patients complete suture of the "cleft" of the anterior cusp of the mitral valve was performed, in 7 partial suture of the "cleft" of the anterior cusp of the mitral valve and in 4 commissuroplasty. In one instance later reoperation with replacement of the mitral valve by a mechanical prosthesis was performed. The control group was formed by 16 healthy volunteers (5 men) aged 22.1 +/- 3 years (19-31 years). Patients operated in childhood on account of an atrial defect of the ostium primum type have on echocardiographic examination, as compared with healthy volunteers, larger atria and the left ventricle, a thicker interventricular septum and left ventricular wall and a higher velocity of left ventricular filling during the late diastole. Higher values of parameters of the size and volume of the left ventricle are associated with the presence of mitral regurgitation. More marked changes of systolic or diastolic left ventricular function are not present, there are not even any echocardiographic signs of higher pressure in the atria and pulmonary artery. In none of the patients a residual shunt at the level of the atrial septum is present. Mitral regurgitation is found in two thirds of the patients, only in one case it was however moderately severe (grade 3). From the results it does not ensue which type of surgery of the "cleft" of the mitral valve has the best long-term results. In none of the patients tricuspid regurgitation of a higher grade than grade 1 is present.

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.

[Hypoplastic left heart syndrome (general review)]. / Syndrom hypoplastického levého srdce (Souborný referát).

The hypoplastic left heart syndrome (HLHS) is a critical congenital heart lesion with a small left ventricle in combination with stenosis or atresia of the aortic and the mitral valve. This heart disease is associated with a nearly 100% mortality at neonatal age. Until recently, this heart lesion was considered inoperable because of extremely unfavourable morphology. Introduction of the Norwood three-step reconstructive operation on one hand, and heart transplantation in neonates, on the other hand, improved the prognosis of patients with this complex heart lesion. In our country, however, this heart disease, if found during the first months of pregnancy, represents an indication for its termination, and neonates with HLHS are offered only symptomatic medical treatment. On basis of good experience from several most experienced cardiac centres we believe that it is necessary to reassess the statement considering HLHS as an inoperable disease. The main change from this aspect necessitates, however, a detailed analysis of the whole problem not only from the morphological, clinical and surgical point of view but also from philosophical, psychological, socio-economic and health care organisation aspects.

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.

Repair of tetralogy of Fallot associated with atrioventricular septal defect.

Tetralogy of Fallot, when associated with atrioventricular septal defect permitting shunting at ventricular level, represents a complex cyanotic congenital malformation. Experience with surgical repair is limited, and results vary considerably. Between 1984 and 1996, we repaired 14 consecutive patients with this combination seen in our center. Their ages ranged from 8 months to 21 years (median 7.4 years). Six (42.9%) had Down's syndrome. In eight patients the correct diagnosis was made using echocardiography alone. In the remaining six patients, who had previously-constructed arterial shunts and/or suspected pulmonary arterial stenosis, catheterization and angiocardiography were also performed. The repair consisted of double patch closure of the septal defect, reconstruction of two atrioventricular orifices, and relief of pulmonary stenosis at all levels. In five patients with a hypoplastic pulmonary trunk, a monocusp transannular patch (four patients) or an allograft (one patient) was used for restoration of continuity from the right ventricle to the pulmonary arteries. Patch enlargement of one or both pulmonary arteries was necessary in five patients. One patient (7.1%) died early, and another late. The twelve surviving (85.8%) patients have been followed for 1.2-12.5 years after surgery (median 4.9 years, mean 5.9+/-3.9 years). During the follow-up, reoperation was necessary for repair of residual ventricular septal defect and pulmonary regurgitation in two patients, and closure of an atrial septal defect and alteration to left atrioventricular valvar regurgitation in one patient. Seven patients are in class I of the New York Heart Association, four in class II, and one in class III. Tetralogy of Fallot associated with atrioventricular septal defect can be corrected with low mortality and good long-term results. Residual lesions, however, have a tendency to progress, especially when seen in combination. After surgery, all patients need long-term close follow-up.

[Primary repair of interrupted aortic arch in neonates]. / Zkusenosti s primární korekcí interrupce aortálního oblouku u novorozencu.

In the Kardiocentrum, University Hospital Motol, Prague, protocol of the primary repair of interrupted aortic arch was introduced, and between 1993-1997, 15 neonates aged 1-26 days (median 5 days) were operated on. Treatment with prostaglandins E for maintenance of the ductal patency, correction of metabolic acidosis, and treatment of all complications were necessary before surgery. The correction was performed from the midline sternotomy approach, in extracorporeal circulation and deep hypothermia with circulatory arrest. Direct anastomosis between the ascending and the descending aorta was possible in all the patients. At the same time, associated heart lesions were corrected (ventricular septal defect in 13, persistent truncus arteriosus in 3, subaortic stenosis in 2, transposition of the great arteries, double-outlet right ventricle and aortico-pulmonary window in 1 patient, each). Four (26.7%) patients died after surgery. Out of the first 6 neonates 3 (50.0%) died, but out of the subsequent 9 patients only 1 (11.1%) died. Reoperation was necessary in 2 patients. All 11 early survivors are alive and doing well 8-54 months after the repair. In one of them restenosis at the site of aortic anastomosis and hemodynamically significant subaortic stenosis occurred. All the remaining patients have a nonrestrictive aortic anastomosis. Primary repair of interrupted aortic arch and associated heart lesions can be performed in a neonate with reasonable mortality. Treatment of complications is necessary before surgery. Results depend especially on the patient's clinical condition and experience of the center.

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.

[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.

[Congenital thrombosis of the aortic arch simulating aortic coarctation in a neonate]. / Vrozená trombóza aortálního oblouku u novorozence imitující koarktaci aorty.

The authors present the case-history of a two-day-old neonate with clinical signs of critical coarctation of the aorta where echocardiography revealed thrombosis of the aortic arch and isthmus. By surgery it was impossible to remove the organized thrombus from the aorta, therefore the portion of the aorta with the thrombus was resected and the aorta was reconstructed by an end-to-end anastomosis. After surgery long-term anticoagulation treatment with Warfarin was started. Examination of haemocoagulation factors, incl. proteins C and S and antithrombin III revealed normal findings. The test for lupus erythematosus was also negative. From the case-history of the mother's pregnancy the cause of the serious congenital aortic thrombosis could not be traced. The child is all right after surgery and thrives.

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.

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.

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.

[Plication of the diaphragm--a method of surgical treatment of diaphragmatic paralysis in neonates and infants after heart surgery]. / Plikace bránice--metoda chirurgické lécby parézy bránice u novorozencu a kojencu po kardiochirurgických operacích.

The cause of paresis of the diaphragm after cardiosurgery is damage of the phrenic nerve. The diagnosis of paresis is based on X-ray examination, sonography and electromyography of the diaphragm. Plication of the diaphragm is indicated only in those children with paresis of the diaphragm who develop during spontaneous ventilation severe respiratory insufficiency. In the Cardiocentre of the Faculty Hospital Prague-Motol between 1983 and 1996 of 5333 children operated on account of heart disease 29 children were subjected to plication of the diaphragm, incl. five where the operation was made during the neonatal stage (17%), 20 in infant age (69%) and four were older than one year (14%). By the third day after plication 9 children (38%) could be disconnected from the respirator, by the 5th day 20 children (70%) by the 7th day 22 children (75%). In neonates and infants with postoperative paresis of the diaphragm, where spontaneous ventilation cannot be induced, plication of the diaphragm is according to the authors the method of choice. It is a rapid and safe surgical operation which reduces the period of artificial ventilation and its complications.