Biomedical subjects

B Hucín

Publications and source records attributed to B Hucín.

At least 19 recordsLinked to original sources

PubMed2005

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.

Adolescent↗

PubMed2004

Demonstration of MyoD1 expression in oncocytic cardiomyopathy: report of two cases and review of the literature.

Oncocytic cardiomyopathy is a rare arrhythmogenic disorder usually associated with female sex, difficult-to-control arrhythmias, or sudden death of infants and children. Morphologically, it is characterized by the presence of oncocytic cells, which are diffusely distributed or form the nodular structures within the myocardium, occasionally involving the valves, with a large number of mitochondria in cytoplasms. We present two cases of oncocytic cardiomyopathy. The first case had a fatal clinical outcome, and the other case was surgically treated. The nuclear expression of skeletal muscle transcription factor MyoD1 was demonstrated in the first case, supporting the theory that oncocytic cardiomyopathy is a conduction system developmental disorder. To confirm this hypothesis, it is necessary to further investigate myogenic transcription factor program in human cardiac conduction system cells.

Cardiomyopathies↗

PubMed2004

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.

Child↗

PubMed2001

Resynchronization pacing is a useful adjunct to the management of acute heart failure after surgery for congenital heart defects.

The acute hemodynamic effect of atrioventricular (AV) and inter/intraventricular (IV) resynchronization accomplished by temporary pacing using multiple epicardial pacing wires was evaluated in 20 children (aged 3.4 months to 14.0 years) after surgery for congenital heart defects fulfilling the following criteria: (1) presence of AV and/or IV conduction delay, and (2) need for inotropic support. AV resynchronization (n = 13) was achieved by AV delay optimization during atrial synchronous right ventricular outflow tract pacing. IV resynchronization (n = 14) was accomplished by atrial synchronous pacing from the right ventricular lateral wall in 7 patients with right bundle branch block and normal AV conduction and by atrial synchronous multisite ventricular pacing in another 7 patients with previously performed AV resynchronization. Compared with baseline values, AV resynchronization resulted in an increase in arterial systolic, mean, and pulse pressures by 7.2 +/- 8.3% (p <0.01), 8.6 +/- 8.1% (p <0.005), and 6.9 +/- 13.5% (p = NS), respectively. IV resynchronization used either alone or added to previously performed AV resynchronization led to a pressure increase of 7.0 +/- 4.7%, 5.9 +/- 4.7%, and 9.4 +/- 7.8%, respectively (p <0.001 for all). The combined effect of AV and IV resynchronization resulted in a systolic, mean, and pulse pressure increase of 10.2 +/- 5.0% (range 4.0 to 19.1), 8.6 +/- 5.4% (range 0.8 to 14.8), and 15.2 +/- 8.5% (range 6.1 to 33.3), respectively (p <0.001 for all). The increase in systolic arterial pressure after IV resynchronization was positively correlated with the initial QRS duration (r = 0.62, p <0.05) and extent of QRS shortening (r = 0.66, p <0.05). In conclusion, resynchronization pacing led to a significant increase in arterial blood pressure and was a useful adjunct to the treatment of acute postoperative heart failure in patients with AV and/or IV conduction delay.

Adolescent↗

PubMed2000

Hemodynamically optimized temporary cardiac pacing after surgery for congenital heart defects.

Disturbance of normal AV synchrony and dyssynchronous ventricular contraction may be deleterious in patients with otherwise compromised hemodynamics. This study evaluated the effect of hemodynamically optimized temporary dual chamber pacing in patients after surgery for congenital heart disease. Pacing was performed in 23 children aged 5 days to 7.7 years (median 7.3 months) with various postoperative dysrhythmias, low cardiac output, and/or high inotropic support and optimized to achieve the highest systolic and mean arterial pressures. The following four pacing modes were used: (1) AV synchronous or AV sequential pacing with individually optimized AV delay in 11 patients with first- to third-degree AV block; (2) AV sequential pacing using transesophageal atrial pacing in combination with a temporary DDD pacemaker for atrial tracking and ventricular pacing in three patients with third-degree AV block and junctional ectopic tachycardia, respectively, who had poor signal and exit block on atrial epicardial pacing wires; (3) R wave synchronized atrial pacing in eight patients with junctional ectopic tachycardia and impaired antegrade AV conduction precluding the use of atrial overdrive pacing; (4) Atrio-biventricular sequential pacing in two patients. Pressures measured during optimized pacing were compared to baseline values at underlying rhythm (13 patients with first-degree AV block or junctional ectopic tachycardia) or during pacing modes commonly used in the given clinical situation: AAI pacing (1 patient with slow junctional rhythm and first-degree AV block during atrial pacing), VVI pacing (2 patients with third-degree AV block and exit block and poor sensing on epicardial atrial pacing wires) and dual-chamber pacing with AV delays set to 100 ms (atrial tracking) or 150 ms (AV sequential pacing) in 7 patients with second- to third-degree AV block and functional atrial pacing wires. Optimized pacing led to a significant increase in arterial systolic (mean) pressure from 71.5 +/- 12.5 (52.3 +/- 9.0) to 80.5 +/- 12.2 (59.7 +/- 9.1) mmHg (P < 0.001 for both) and a decrease in central venous (left atrial) pressure from 12.3 +/- 3.4 (10.5 +/- 3.2) to 11.0 +/- 3.0 (9.2 +/- 2.7) mmHg (P < 0.001 and < 0.005, respectively). In conclusion, several techniques of individually optimized temporary dual chamber pacing leading to optimal AV synchrony and/or synchronous ventricular contraction were successfully used to improve hemodynamics in patients with heart failure and selected dysrhythmias after congenital heart surgery.

Arrhythmias, Cardiac↗

PubMed2000

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.

Adolescent↗

PubMed2000

[Aortic reconstruction in neonates with hypoplastic left heart syndrome] .

The surgical technique and tactics of the Norwood operation in neonates with hypoplastic left heart syndrome represent a surgical challenge. The Norwood operation was performed from the midline sternotomy approach, in extracorporeal circulation and deep hypothermic circulatory arrest was used for reconstruction of the hypoplastic aorta. Operation consisted of reconstruction of the "neoaorta" and the aortic arch from the original hypoplastic ascendent aorta, pulmonary trunk and a patch cut from a pulmonary homograft or pericardium, excision of the atrial septum and an arterial shunt from a Goretex vascular graft 3.5 or 4 mm in diameter. In patients with well developed aortic arch it was possible to reconstruct the aorta using Damus-Kaye-Stansel operation without circulatory arrest. Out of 12 operated patients with this defect, two (16.7%) died during the early postoperative period, one patient died late. The postoperative course was often complicated. In 6 (50.0%) patients the second step of Norwood operation, the bidirectional cavopulmonary anastomosis, was performed without mortality. According to our experience, it was necessary to prepare patients adequately before the first surgery. Perfect reconstruction of the aorta and a well functioning shunt had the crucial significance.

Aorta↗

PubMed1999

[Occult fibrin production during surgery in cyanotic congenital heart defects and extracorporeal circulation].

In 10 patients with complex cyanotic congenital defects detailed coagulation examinations were made at the beginning and end of the extracorporeal circulation after neutralization of heparin by protamine and the results were compared with a control examination, made before general heparinization, after introduction into general anaesthesia. The authors examined the activated period of blood (ACT) by means of testing tubes with a celite activator (Hemochron) as well as the HR-ACT test with a kaolin activator (Medtronic) for comparison of the results. The authors assessed quantitatively plasma levels of heparin, antithrombin III and fibrinopeptide A which is a sensitive indicator of intravascular coagulation. They assessed also the fibrinogen level and total number of thrombocytes in the blood stream. The degree of haemodilution was recorded as well as the temperature at the periods of assessment. The values of both ACT test were within the range of values above 420 secs., evaluated according to the authors protocol as adequate for total heparinization during operations under conditions of extracorporeal circulation. Despite of this heparin levels lower than those recommended in the literature were found, as well as reduced antithrombin III levels during extracorporeal circulation and a rise of fibronopeptide A levels at the end of extracorporeal circulation which suggest latent fibrin production in the patients. Laboratory results were compared with clinical symptoms of post-operation bleeding. In 50% patients after surgery signs of increased haemorrhage in the surgical field and from thoracic drains were observed, in two patients the surgical wound had to be revised. Laboratory tests revealed in two patients thrombocytopenia after surgery, one patient had a prothrombin test reduced below 45% and in one patient there was a significantly reduced fibrinogen level calling for supplementation of this factor. After improvement of the laboratory results and surgical treatment haemostasis returned to normal. All patients survived the operation and were discharged from hospital to domestic treatment.

Adolescent↗

PubMed1998

[Reoperation in congenital heart defects after primary surgery in the neonatal period and infancy].

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.

Child↗

PubMed1998

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.

Aorta, Thoracic↗

PubMed1998

Primary repair of transposition of the great arteries with interrupted aortic arch.

Primary repair of transposition of the great arteries with interrupted aortic arch was performed in a 2 day old neonate weighing 2.1 kg. The repair consisted of a direct anastomosis between the ascending and the descending aorta and an arterial switch repair without Lecompte procedure because of the side by side position of the great arteries and an anomaly of the coronary arteries. A total of 28 months after the repair, the patient remains in excellent clinical condition.

Aorta, Thoracic↗

PubMed1998

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.

Anastomosis, Surgical↗

PubMed1998

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.

Adolescent↗

PubMed1998

Pulmonary function in children with atrial septal defect before and after heart surgery.

OBJECTIVE: To test the effect of heart disease and heart surgery on lung function. DESIGN: A pulmonary function study of children undergoing surgery for atrial septal defect (ASD). SETTINGS: University hospital. PATIENTS: 26 children tested before surgery (at mean (SD) age 11.8 (3.8) years) and 24 patients tested 1.8 (0.2) years after surgical correction. METHODS: Lung volumes, lung elasticity, and airway patency indices were measured using standard techniques. RESULTS: Before surgery: pulmonary function test abnormalities were found in 18 of the 26 patients. Stiff lung was found in 12, lung hyperinflation in five, and indices of decreased airway patency in four. Total lung capacity decreased in only two patients. After surgery: pulmonary function test abnormalities were found in 12 of the 24 patients (informed consent not given for two patients). Stiff lung was detected in nine and indices of peripheral airway obstruction in four. Mean values of specific airway conductance and peak expiratory flow were all normal. Lung hyperinflation was found only in one of 24 patients. No correlation between perioperative events and pulmonary function test data was found. CONCLUSIONS: Pulmonary function test abnormalities persist in half the patients almost two years after surgery for ASD. A decrease in the total frequency of pulmonary function test abnormalities (in 19% of the patients), with a decrease in stiff lung in 8% and lung hyperinflation in 15%, was not significant. Impairment of lung function related to ASD is associated with the disease itself rather than the surgical procedure.

Adolescent↗

PubMed1998

[Congenital thrombosis of the aortic arch simulating aortic coarctation in a neonate].

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.

Aorta, Thoracic↗

PubMed1998

[Primary repair of interrupted aortic arch in neonates].

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.

Aorta, Thoracic↗

PubMed1997

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.

Amino Acids↗

PubMed1997

Good exercise tolerance and impaired lung function after atrial repair of transposition.

OBJECTIVE: Evaluation of long-term results of atrial correction of transposition of the great arteries (TGA), focusing on the relationship between pulmonary function and exercise tolerance. METHODS: A prospective population-based study-56 out of 60 survivors of Mustard/Senning repair, born in Bohemia in 1980-1984 (median age at surgery 0.85 years) were followed up over 13.4 +/- 1.2 years and studied by complete lung function and bicycle exercise testing. RESULTS: Maximum heart rate on exercise reached 181 +/- 14 bpm (Z-score: -1.06 +/- 1.66, range -6.7 to +1.6); VO2max: 40.6 +/- 6.7 ml/kg per min (Z-score: -0.97 +/- 1.4, range -5 to +1.8). A total of 73.6% had abnormal lung function, most frequently features of stiff lung (39.6%) and lung restriction (32.1%). Static recoil pressure of the lungs measured at 100, 90 and 60% of total lung capacity reached 137, 126 and 130%, respectively (Z-score: 1.93 +/- 2.33, 1.64 +/- 1.96, and 1.14 +/- 1.95, respectively). There was an inverse relationship between static recoil pressure of the lungs and VO2max (r = -0.306, P = 0.043), indicating the impact of lung function abnormalities on exercise tolerance. Comparison with lung function study of the group of 'pioneer' Mustard patients operated at the mean age of 4.4 years revealed a similar pattern and frequency (73.6% in current series vs. 88%, NS). CONCLUSIONS: Long-term follow-up has shown good exercise tolerance in the majority of patients after atrial correction of TGA performed in infancy. Many of them, however, have impaired lung function, especially stiff lung, although less frequently than original patients operated at a later age. The stiff lung can impair the exercise tolerance.

Analysis of Variance↗