Predictors of exercise capacity in patients with atrial correction of transposition of great arteries.

OBJECTIVES: To examine the relationship of clinical, biochemical and imaging parameters to maximum oxygen uptake in patients after atrial correction of transposition of great arteries. BACKGROUND: Exercise tolerance is a key determinant of quality of life in patients with adult congenital heart disease. It is determined by a large scale of factors often different from general cardiology. METHODS: 86 consecutive patients after Senning correction of TGA were subjected to clinical and echocardiographic examination, Holter monitoring, blood tests of NT-proBNP, MRI of the heart and exercise test. Parameters of these examinations were correlated to VO2 max. RESULTS: The average age of patients was 28±3.5 years. The average systemic right ventricular function determined by MRI was 51.9±7.9 %. The average NT-proBPN was 124.3±23.6 ng/l, VO2 max. 31.7±6.5    ml/kg/min and the heart rate reserve 106±24 /min. Neither systemic right ventricular systolic function nor   NT-proBPN predicted VO2 max., whereas the heart rate reserve did (p=0.003). CONCLUSION: An inability to increase heart rate during exercise noted in a considerable number of patients after atrial switch of TGA caused a decreased exercise tolerance. It is not solely the global systolic function of either ventricle that influences the exercise performance, rather it is the ability to increase heart rate and overall cardiac output appropriately (Tab. 3, Fig. 6, Ref. 28).

Cardiac resynchronization therapy: a novel adjunct to the treatment and prevention of systemic right ventricular failure.

OBJECTIVES: This study aimed to evaluate the technical feasibility and hemodynamic benefit of cardiac resynchronization therapy (CRT) in patients with systemic right ventricle (RV). BACKGROUND: Patients with a systemic RV are at high risk of developing heart failure. Cardiac resynchronization therapy may improve RV function in those with electromechanical dyssynchrony. METHODS: Eight patients (age 6.9 to 29.2 years) with a systemic RV and right bundle-branch block (n = 2) or pacing from the left ventricle (LV) (n = 6) with a QRS interval of 161 +/- 21 ms underwent CRT (associated with cardiac surgery aimed at decrease in tricuspid regurgitation in 3 of 8 patients) and were followed-up for a median of 17.4 months. RESULTS: Change from baseline rhythm to CRT was accompanied by a decrease in QRS interval (-28.0%, p = 0.002) and interventricular mechanical delay (-16.7%, p = 0.047) and immediate improvement in the RV filling time (+10.9%, p = 0.002), Tei index (-7.7%, p = 0.008), estimated RV maximum +dP/dt(+45.9%, p = 0.007), aortic velocity-time integral (+7.0%, p = 0.028), and RV ejection fraction by radionuclide ventriculography (+9.6%, p = 0.04). The RV fractional area of change increased from a median of 18.1% before resynchronization to 29.5% at last follow-up (p = 0.008) without a significant change in the end-diastolic area (+4.0%, p = NS). CONCLUSIONS: The CRT yielded improvement in systemic RV function in patients with spontaneous or LV pacing-induced electromechanical dyssynchrony and seems to be a promising adjunct to the treatment and prevention of systemic RV failure.

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.

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.

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.

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.

An attempt to demonstrate the increased resistance of selected bacterial strains during repeated exposure to UV radiation at 254 nm.

Evidence was provided that in a repeatedly irradiated bacterial suspension of Escherichia coli K12: AB 1157, 1886, 2463, under the described experimental conditions, gradually mutants more resistant to UV radiation survive. Under these circumstances it may happen that selection and an increase of bacterial clones with an increased UV resistance develop, manifested by a reduced effectiveness of UV radiation during repeated irradiation. Although the cumulating damage of DNA in microbes can lead to a reduction of their survival as well as a reduction of their absolute numbers, it can be hardly assumed that during practical use in air conditioning units or during disinfection of drinking water all bacteria will be destroyed. In that case even repeated UV irradiation is not necessarily a reliable disinfection tool.

[Principles, methods and use of the National Register of Cardiovascular Interventions]. / Principy, metody a vyuzití Národního registru kardiovaskulárních intervencí.

The goal of cooperative project of the national registry of cardiovascular interventions is analysis of quality of cardiovascular care with risk stratification and assessment of clinical indicators at organizational and individual level. Bayes methodology of stratification and risk prediction is used.

[Heart surgery in neonates: types and timing of surgery--present possibilities and results]. / Kardiochirurgie novorozeneckého veku: typy a nacasování operací--soucasné moznosti a výsledky.

Neonatal cardiosurgery is nowadays an integral part of treatment of congenital heart disease. At present it is possible to correct completely with a feasible risk heart diseases in the majority of neonates (73%) with a critical congenital disease. Most frequently these disease defects are operated during the first two weeks of life (78% of all operations). In the strategy of surgery optimal timing and selection of the most suitable operation is essential to ensure in time favourable conditions for child growth and development and a long high-quality life. The tactics and therapeutic results in contemporary neonatal cardiosurgery are illustrated by the authors' experience with 174 neonates operated on for congenital critical heart defect in 1991-1993 in the Pediatric Cardiocentre, University Hospital Prague-Motol.

[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.

[Radical anatomic correction of transposition of great arteries in neonates. A new program of treatment of critical congenital heart defects]. / Radikální anatomická korekce transpozice velkých arterií u novorozencu. Nový program lécby kritické vrozené srdecní vady.

METHODS AND RESULTS: From the group of 110 neonates born with transposition of the great arteries during 1991-1994 that were referred for the treatment to Kardiocentrum, University Hospital Prague-Motol, 46 neonates with simple transposition were operated on according to the criteria for anatomical correction (arterial switch) at the mean age of 9 days (4-20 days). Fourteen infants with transposition and large ventricular septal defect were corrected with arterial switch at the mean age of 2.5 months (5 weeks-9 months). Fifty neonates that did not meet criteria for arterial switch procedure were indicated for correction at the atrial level (Senning procedure) that has been performed at the mean age of 5 months (1-10 months). Out of 46 operated neonates 10 died following the operation and one child died 2 months later after surgery. There were 2 death out of last 20 neonates (10%). Thirty five children surviving 1-4 years after anatomical arterial correction of transposition are without complaints in excellent condition, NYHA class I. CONCLUSIONS: The procedure of the anatomical correction at the level of the great arteries (arterial switch) according to Jatene, that has been successfully introduced at Kardiocentrum, University Hospital Prague-Motol, has been reproducible and became the method of choice for operations of transposition of the great arteries in neonates with the suitable anatomy.

[Heart involvement in Lyme borreliosis. Case report]. / Postizení srdce pri lymeské borelióze. Kasuistika.

Lyme disease is a systemic illness with skin, neurologic, joint, and cardiac involvement. Absence of typical skin lesion--erythema chronicum migrans--during the first period of disease may cause the diagnosis difficult. Syncope due to complete atrioventricular block was the first symptom of disease in our patient, but no tick bite anamnestic data were available. Quick changes on EKG tracing during cardiac pacing were present. Echocardiography revealed left ventricular dysfunction. Specific immunological tests confirmed the diagnostic suspicion. Cardiac pacing, digitalis and antibiotic treatment ensued in complete recovery. No other systemic lesion appeared. Lyme carditis may be the only manifestation of the disease. Temporary cardiac pacing may be necessary.

[Complex plasty of the atrioventricular valve in defects with a functional single ventricle--surgical technic and midterm results]. / Komplexní plastika atrioventrikulárních chlopní u vad s funkcne jedinou komorou--operacní technika a strednedobé výsledky.

Congenital heart diseases with a functionally single ventricle can be surgically treated using total cavopulmonary connection. Regurgitation of atrioventricular valves represents one of risk factors for death and unfavourable long-term result after this operation. During 1996-2001 total cavopulmonary connection with a complex plastic repair of atrioventricular valves was performed in 8 patients at the age from 1.4 to 13.3 years (median 7.8 years). All patients had very complex congenital heart diseases with severe malformation of atrioventricular valves. All but one had pulmonary stenosis or atresia. Six patients had common atrioventricular orifice, 5 patients had bilateral superior caval veins and 4 patients had dextrocardia or mesocardia. In 6 (75%) patients one or two palliative operations had been performed earlier. Before surgery all patients were cyanotic with severe hypoxemia and polyglobulia. All patients had regurgitant atrioventricular valves. The surgery was performed from the midline sternotomy approach, in extracorporeal circulation and moderate hypothermia. The surgery consisted in excision of the atrial septum, construction of a direct connection of all superior and inferior caval veins with the pulmonary artery, transsection of the pulmonary trunk and complex plastic repair of atrioventricular valves. The inferior caval vein was derived to the pulmonary artery using an intraatrial lateral tunnel in 3 patients and using an external conduit from a Goretex vascular prosthesis in 5 patients. In 5 patients a fenestration in tunnel or conduit permitting decompression of the systemic venous return was done. Individualized plastic repair of atrioventricular valves consisted in the suture of the central portions of both common leaflets. Four patients required also commissuroplasty of one to four commissures and two patients required annuloplasty. All patients survived the early postoperative period. In the majority of them complications occurred: hypoxemia in 3, pleural effusions in 3, low cardiac output in 2 and bleeding in 1 patient. In two patients reintervention for severe hypoxemia was necessary. Postoperative echocardiographic examination proved good result in all patients. In 6 (75%) patients the grade of regurgitation of atrioventricular valves decreased immediately after the repair, in the two remaining patients the degree of regurgitation decreased during the follow up. One patient with the most severe residual regurgitation which progressed during the follow up died 2.5 years later under the signs of congestive heart failure after spontaneous closure of fenestration. The 7 (87.5%) living patients remain in good clinical condition in NYHA class I or II. Our experience proved that it is possible to perform total cavopulmonary connection with low mortality and good midterm results even in patients with hemodynamically significant regurgitation of atrioventricular valves. Persistent severe or progressive regurgitation, however, requires surgical reintervention.

[Surgical treatment of aorto-ventricular tunnel]. / Chirurgická lécba aortoventrikulárního tunelu.

The authors give an account of their experience with the treatment of aortoventricular tunnel and describe a successful operation in a four-month-old infant. They emphasize the importance of early indication and accurate surgical technique to preserve satisfactory function of the aortic valve.

[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.

[Bidirectional cavopulmonary anastomosis in palliative surgical treatment of complex congenital heart defects]. / Bidirekcní kavopulmonální anastomóza pri paliativní chirurgické lécbe komplexních vrozených srdecních vad.

Bidirectional cavopulmonary anastomosis was used during the past six years for palliative treatment of 34 children with complex cardiac disease with a low pulmonary blood flow and a functional common ventricle. The age at the time of operation was 5 months to 16 years (median 55 months). Two children (5.9%) died soon after operation and one patient died in conjunction with reoperation seven months later. After operation a significant increase of saturation and decrease of the haematocrit occurred in all patients. The condition of 31 patients during an out-patient check-up 2-64 months after operation was satisfactory and postoperative examinations revealed a satisfactory function of the anastomosis.

[Tactics and technique of primary correction of aortic arch interruption using a median sternotomy]. / Taktika a technika primární korekce interrupce aortálního oblouku ze strední sternotomie.

Primary correction of an interrupted aortic arch was performed in seven neonates aged 2 to 26 days. The operation was performed from median sternotomy with extracorporeal circulation using hypothermic arrest of the circulation for reconstruction of the aortic arch. In all neonates it proved possible to make a direct anastomosis of the ascendent and descendent aorta. At the same time in six children a defect of the ventricular septum was closed, in two the common arterial trunk was corrected, in one resection of subaortic stenosis was performed and in another child correction of the aortopulmonary window. After the operation three neonates died from sepsis and multiorgan failure. At present four patients are alive who, 10 to 20 months after operation, are in a good condition. All have a non-restrictive anastomosis of the aorta. Primary correction of an interrupted aortic arch and associated defects is preferred by the authors to two-stage surgery.

[Total cavopulmonary anastomosis in the surgical treatment of congenital heart defects with a functional single ventricle]. / Uplná kavopulmonální anastomóza pri chirurgické lécbe vrozených srdecních vad s funkcne spolecnou komorou.

Total cavopulmonary connection (TCPC) allows surgical treatment of patients with complex univentricular heart disease. Between 1991 and 1993 this operation was performed in 15 cyanotic children with complex heart defects. Age range was 3.5-14 (median 8.3) years. In 5 children with increased operative risk a calibrated interatrial fenestration was created intraoperatively to allow systemic venous decompression. Three patients (20%) died. In remaining 12 patients a significant increase in arterial oxygen saturation and decrease of polycythemia was encountered. All patients remain in a good clinical condition 1-25 months after operation. Noninvasive investigation including echocardiography confirmed good function of the heart.