Biomedical subjects

J Marek

Publications and source records attributed to J Marek.

At least 73 records · Page 4Linked to original sources

PubMed1998

[Can normalization of vascular cytoadhesive activity be explained by the anti-atherosclerosis effect of growth hormone?].

BACKGROUND: The increased mortality caused by premature atherosclerosis has been shown among patients with hypopituitarism receiving conventional hormone treatment but with unsubstituted growth hormone deficiency. This experience belongs among the most important arguments in favour of replacement with growth hormone. The mechanisms of the antiatherogenic effect of growth hormone are poorly understood. The protective effect of growth hormone on the vascular endothel and its intervention in the clotting process, which have not been yet elucidated, may be the causative factors. METHODS AND RESULTS: The endothelial damage as given by measuring of circulating soluble cytoadhesive molecules sE-selectin, sP-selectin and intercellular adhesive molecule 1 (ICAM 1) was measured in 15 adult panhypopituitaric patients before and after 1 year treatment with recombinant human growth hormone. The blood levels of all of these cytoadhesive molecules decreased significantly (p < 0.01) during the treatment. None of the concomitantly followed coagulation tests (prothrombin time, activated partial thromboplastin time, fibrinogen, antithrombin III, von Willebrand's factor and D-dimer) was significantly changed during the treatment. The tendency to decrease (p = 0.054) was observed with antithrombin III. CONCLUSIONS: The decrease of circulating cytoadhesive molecules in blood during the treatment of growth hormone gives evidence for its protective effect, either direct or mediated, on the vascular endothel. These findings could bring an explantation for the premature atherosclerotic changes in hypopituitarism and antiatherogenic effect of growth hormone.

Adult↗

PubMed1998

[Treatment of hypopituitarism in adults with growth hormone improves improves the thickness of the arterial intima].

BACKGROUND: Increased mortality of hypopituitary adults with atherosclerotic vascular disease is commonly explained by growth hormone deficiency and forms the main argument for the growth hormone replacement. At the same time it is known that the growth hormone-IGF-I axis stimulates the vessel wall proliferation as an initial step of atherosclerosis. Investigation of the arterial intima thickness in hypopituitary adults with duplex ultrasound system and its follow up during the growth hormone therapy may clarify these effects of growth hormone on the vessel wall. METHODS AND RESULTS: Luminal diameter, maximal blood flow velocity and arterial intima thickness of common carotid arteries was measured with colour duplex ultrasound system in 15 adults with hypopituitarism before and after one year therapy with recombinant growth hormone. The results were compared with healthy controls matched for gender, age, blood pressure and weight. The luminal diameter did not changed during the therapy with growth hormone and was the same in patients and controls. Maximal blood velocity in hypopituitary patients before the therapy was lower than in control subjects (74.8 +/- 13.9 cm/s vs. 94.0 +/- 21.0 cm/s), but the statistical difference was only borderline. Arterial intima thickness was significantly thinner in hypopituitary patients when compared with controls (0.56 +/- 0.1 mm vs. 0.71 +/- 0.12 mm, p < 0.001) but became thicker after the treatment and was not any more different from the controls (0.56 +/- 0.1 mm vs. 0.66 +/- 0.1 mm, p < 0.001). CONCLUSIONS: In hypopituitary adults the arterial intima thickness of common carotid arteries is less than in healthy controls. The reduced thickness was normalised after one year treatment with growth hormone. Atheromatous plaques were not found either before or after the treatment. Duplex ultrasound measurement may be one of the means of checking of the effects of growth hormone therapy.

Adult↗

PubMed1998

Dexfenfluramine in psychotic patients.

Dexfenfluramine (DF) is contraindicated in severe psychiatric disorders and in depression. We used DF in 3 patients with chronic psychosis and severe overeating without changes in psychiatric pharmacotherapy. Two patients had paranoid schizophrenic psychosis with hallucinations, one patient mixed psychosis, beginning with lactation psychosis, and several attacks of hallucinations and depression later. Overeating was removed in all 3 patients without any negative effect on the psychotic state. All patients were able to maintain their body weight. Two patients with poorly controlled diabetes improved markedly their metabolic status. Doses up to 75 mg per day of DF were necessary during binge eating episodes in one patient. We conclude that DF can be used with care under close psychiatric supervision in psychotic patients with severe overeating.

Adult↗

PubMed1998

Primary manifestation of Hodgkin's disease in the central nervous system.

A 62-year-old woman presented with loss of memory and a mild hemiparesis. Neuroradiology demonstrated a left frontoparietal tumour. Biopsy specimens of this lesion revealed intracerebral Hodgkin's lymphoma, a diagnosis supported by immunohistochemical reactions of the tumour cells for the CD30 antigen. Additional cell cycle studies revealed a high proliferative activity of the tumour cells in association with absence of apoptosis. There was no evidence that overexpression of bcl-2 or Epstein-Barr virus infection was involved in the pathogenesis of this neoplasm. Lymphomas in the lung were detected 3 months later. Following neurosurgical excision, radiotherapy, and chemotherapy, the patient had no evidence of Hodgkin's disease after 13 months of follow-up.

Apoptosis↗

PubMed1998

Evaluating institutional variability of duplex scanning in the detection of carotid artery stenosis.

PURPOSE: Duplex scanning is widely used to measure carotid artery stenosis, but the issue of variability between institutions must be raised. To examine for this potential variability, we evaluated two ATL Ultramark 9 duplex machines at two hospitals within our institution. METHODS: Stenosis was calculated angiographically as in the North American Symptomatic Carotid Endarterectomy Trial (NASCET). Internal carotid artery (ICA) to common carotid artery (CCA) peak systolic velocity (PSV) ratios were determined using the two ATL machines. Receiver operating characteristic (ROC) curves were generated for each hospital. RESULTS: ROC curves varied significantly. The area under the curve from hospital 1 was 0.95 (SE = 0.021); the area under the curve from hospital 2 was 0.87 (SE = 0.034). The curves were essentially statistically different (P = 0.0516). COMMENTS: We showed a difference in predicting diameter stenosis utilizing ICA PSV/CCA PSV ratios at two hospitals within our institution. Variability is likely due to technologists, since equipment and physician interpreters were the same. Criteria must be developed for each different machine in each laboratory. Quality assurance is critical to minimize variability.

Carotid Stenosis↗

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

[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↗

PubMed1998

[Limbal stem cell autograft transplantation for persistent epithelial corneal defect after chemical burn of the eye].

The aim of this paper is to describe the possibility of surgical treatment of persistent corneal epithelial defect caused by limbal stem cell deficiency after chemical burn of the eye. Limbal stem cells serve as a proliferative reserve and are responsible for a proper corneal re-epithelialization. Destruction of these cells leads to chronic irritation, epithelial erosions, corneal conjunctivalisation, vascularisation and stromal scarring. Conventional treatment in such cases is ineffective and prognosis for penetrating keratoplasty is very poor. We present a case of persistent epithelial defect after chemical burn that was successfully treated 4 weeks after the injury with limbal stem cell autograft transplantation. The operative technique involved transfer of two free grafts of limbal tissue from the healthy donor eye to the severely burned eye, prepared by superficial dissection of fibrovascular pannus. Three weeks after operation the cornea was completely covered by proper epithelium, stromal vascularisation and opacification was regressed and visual acuity was improved.

Adult↗

PubMed1997

[Spontaneous remission of corticosteroid osteopenia after successful surgical treatment of Cushing's syndrome. A cross-sectional study].

BACKGROUND: Osteoporosis is one of the most serious consequences of Cushing's syndrome. Only a small number of longitudinal observations on bone mineral density (BMD) in patients with treated Cushing's syndrome have been reported so far. To evaluate changes in bone mass in patients with Cushing's syndrome after surgical cure of the disease, BMD was evaluated cross-sectionally. METHODS AND RESULTS: BMD (DPX-L, Lunar) was measured in the lumbar spine and femoral neck (i.e. in skeletal areas with high proportion of trabecular and cortical bone, respectively) in 72 patients after successful surgical cure of Cushing's syndrome (8 men, 34 women before menopause and 30 women after menopause who were not on hormone replacement therapy). No other drug's interfering with skeletal metabolism were used. The reference group consisted of young healthy Czech women and/or men. The mean lumbar spine and femoral neck BMD increased to normal values within 3 and 5 years after surgery, respectively. In women after menopause, however, the significant positive relationship between BMD and time after surgery was negatively influenced by time after menopause. It is likely that several other factors contributed to the increase in bone mass in the patients (cure of hypercortisolism, recovery from hypogonadism and restoration of muscle strength). CONCLUSIONS: After surgical cure of Cushing's syndrome in premenopausal women and in men, BMD rapidly and substantially increases. The recovery is negatively affected by estrogen deficiency in postmenopause.

Adenoma↗

PubMed1997

[Effect of estrogens on cell adhesion molecules in thrombophilic states associated with high blood estrogen levels].

BACKGROUND: Incidence of thromboembolism in pregnancy is relatively low regarding to many predisposing factors, inclusively the thrombophilia induced changes in coagulation. To evaluate this contradiction we presumed: 1. still not adequately evaluated role of the fibrinolytic system, 2. possible suppression of cytoadhesive molecules in bloodstream and consequently decreased activation of vascular endothelia by high levels of estrogens in the course of pregnancy. METHODS AND RESULTS: The tests of fibrinolysis (serum levels of fibrinogen, D-dimers, tissue plasminogen activator (tPA) and plasminogen inhibitor 1 (PAI-1) and circulating cytoadhesive molecules (sE-selectin, sP-selectin and ICAM-1) were followed in a group of 66 pregnant women and compared with the unpaired Student's t-test with a group of 16 women after uncomplicated ovarectomy for benign disease. Results were compared with the serum 17 beta-estradiol levels. In pregnancy, significantly decreased levels of both selectins were found: sP-selectin: 159 +/- 39 micrograms/l (SD) vs. 205 +/- 69 micrograms/l (p < 0.05), sE-selectin: 33 +/- 12 micrograms/l (SD) vs. 43 +/- 17 micrograms/l (p < 0.05), meanwhile no significant difference in the concentration of ICAM-1 was found: 248 +/- 80 micrograms/l (SD) vs 285 +/- 101 micrograms/l (SD) (p > 0.05). In concordance, there were found significant negative correlations between serum levels of 17 beta-estradiol and sP selectin (r = -0.35, p < 0.01) and between serum levels of 17 beta-estradiol and sE-selectin (r = -0.21, p < 0.05) but not between 17 beta-estradiol and ICAM-1 (r = -0.1, p > 0.05). There were significant correlations between serum levels of 17 beta-estradiol fibrinogen (r = 0.46, p < 0.01), plasminogen activator I (PAI-1) (r = 0.38) and tissue plasminogen activator I (tPA) (r = 0.22, p < 0.05). No difference was found in serum levels of lipoprotein Lp(a) and naturally occurring inhibitor of the receptor for interleukin 1 (IL-Ira). CONCLUSIONS: This data supports the concept that the decreased serum levels of cytoadhesive molecules of sP-selectin and sE-selectin are dependent on serum estrogen levels and together with a new, estrogen induced, equilibrium of the fibrinolytic system suggest an explanation for the relatively low incidence of thromboembolic events in pregnancy. Decrease of cytoadhesive molecules may be one of explanations of favourable effects of estrogens on the development of atherosclerotic vascular changes.

Adolescent↗

PubMed1997

[Reference values, metabolism and findings of abnormal values of magnesium in childhood].

In the introduction we summarize specifics of reference values and metabolism of magnesium in childhood. We studied the frequency of abnormal values of magnesium in both children and adults hospitalized in Faculty Hospital Motol, Prague. We analyzed 6761 tests of S-Mg and 356 dU-Mg results located in archives of Department of clinical biochemistry (1. 1. 1992-1. 11. 1995). The frequency of low level of S-Mg (< 0.65 mmol/l) was 14%. Hypermagnesemia was present in 1.6% of the total number of cases. We found low values of dU-Mg in 20% of the total number of cases, hypermagnesiuria was present in 8.4% cases. We also proved that hypomagnesemia is more frequent in both boys and men than in girls and women. Critical values of S-Mg below 0.5 mmol/l are less frequent (84 cases). Estimated frequency of clinical significant hypomagnesemia in hospitalized patients was about 1.25%.

Adolescent↗

PubMed1997

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.

Adolescent↗

PubMed1997

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.

Adolescent↗

PubMed1997

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.

Aorta, Thoracic↗

PubMed1997

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.

Abnormalities, Multiple↗