Congenital diaphragmatic hernia associated with esophageal atresia, tracheoesophageal fistula, and truncus arteriosus in a premature newborn.

The occurrence of coexisting congenital diaphragmatic hernia (CDH) and esophageal atresia (EA) with distal tracheoesophageal fistula (TEF) is extremely rare and is considered highly lethal. The combination of CDH with EA/TEF and truncus arteriosus communis (TAC) has not been reported in the literature to date. The authors describe a premature neonate with this association.

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.

[Hypoplasia of trachea with tracheal bronchus as a complication of surgical correction of tetralogy of Fallot]. / Hypoplazie trachey s tracheálním bronchem jako prícina komplikací po korekci Fallotovy tetralogie.

A 10 months old infant underwent repair of tetralogy of Fallot with hypoplastic annulus of the pulmonary valve, diminutive pulmonary arteries, right aortic arch and left superior vena cava. The right ventricular outflow tract was reconstructed using a monocusp patch. The postoperative course was unfavourably influenced by respiratory complications due to tracheal bronchus and hypoplasia of trachea, which were not diagnosed preoperatively. Extreme emphysema of the right upper and middle lobes compromised haemodynamics. Repeated reoperations were required. The upper and the middle lobes of the right lung had to be resected, the hypoplastic trachea reconstructed with a pericardial patch and pulmonary homograft inserted. One year later, homograft had to be replaced and tricuspid annuloplasty performed for pulmonary and tricuspid regurgitation and right ventricular dilatation as a consequence of increased pulmonary artery pressure. Three years after the original surgery the patient remains in good clinical condition.

[Heart transplantation in children]. / Transplantace srdce u detí.

Heart transplantation represents the only effective method of treatment of intractable heart diseases. Cardiomyopathy with heart dilation, inoperable congenital heart diseases and heart failure after the heart surgery represent the most common indications for heart transplantation in pediatrics. Hypoplastic left heart syndrome is the most frequent indication for the heart transplantation in neonates and infants. In infants steroids-free immunosuppression is used or steroids are withdrawn 6 to 12 months after the transplantation. Graft failure, acute rejection and infection are the most common causes of death after transplantation. Long-term results and the quality of life depend on the occurrence of rejection, infection, graft vasculopathy, lymphoproliferative disease and side effects of immunosuppression. Posttransplant graft vasculopathy, however, represents the most dangerous late complication, which requires retransplantation. Improvement of the transplantation program improves the survival rate as well as the quality of life after transplantation. Survival after heart transplantation is 80% in one year and 50% in ten years interval. Better results can be achieved in neonates. Lack of donors, demanding economical and organisational conditions and the unresolved legislation problems preclude wider use of heart transplantation in children. Research for more effective, safer and cheaper pharmacotherapy in the prevention and treatment of rejection, infection and complications of immunosuppression is required. Long-term effects and the quality of life after heart transplantation in children, as well as possibilities of mechanical heart support and xenotransplantation must be also studied.

[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.

[Ethical, psycho-social, legislative and economic aspects of surgical treatment of the hypoplastic left heart syndrome]. / Etické, psycho-sociální, legislativní a ekonomické aspekty chirurgické lécby syndromu hypoplastického levého srdce.

Hypoplastic left heart syndrome (HLHS) represents a lethal congenital heart disease, which used to be considered inoperable. The improvement of results of cardiac surgery in neonates improved also the prognosis of patients with this heart lesion. HLHS, however, continues to be an unresolved problem from ethical, psychological, social and economical points of view. Opinions regarding the care for these patients depend on economical, political, cultural and religious factors. Based on recent experience it is evident, that HLHS is an operable disease in a child, who without surgery would otherwise die. Approximately 40% of patients with HLHS can live 15 years after surgery. The long-term prognosis remains, however, unclear. Because of the need of demanding care for a patient with HLHS, it is necessary to see also the quality of life and hear the opinion of parents. Psychomotor development and the quality of life may be altered by complications. An individualized approach in fetuses and neonates with HLHS on the basis of prenatal diagnosis, analysis of risk factors, provision of detailed informations to parents and indications for surgery in neonates having chance for an acceptable result is considered to be the most acceptable solution. In the future it will be necessary to concentrate the research on prenatal diagnostics, treatment and postoperative follow-up including psychomotor development and quality of life. In the preparation of legislative, which would reflect more sensitively the contemporary possibilities of medicine and at the same time regard the problems of health care organization, as well as social and economic aspects, narrow interdisciplinary cooperation will be required.

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.

[Morphology of the hypoplastic left heart syndrome from the surgical perspective]. / Morfologie syndromu hypoplastického levého srdce z chirurgického hlediska.

Hypoplastic left heart syndrome (HLHS) is found in a wide spectrum of changes of the mitral valve, left ventricle and aorta. It represents a critical congenital heart disease with a small left ventricle and stenosis or atresia of the aortic and the mitral valve. The knowledge of a detailed anatomy of this lesion is necessary from the viewpoint of assessment of all possibilities of the surgical treatment, interventional cardiological procedures or foetal cardiac surgery. Sixty one hearts with HLHS from the collection of heart specimens with congenital heart malformations were reviewed. The existence of a wide spectrum of malformations of all heart structures was proved. In all the cases enlarged right atrium and right ventricle was found. In 23.0% of specimens foramen ovale was restrictive or closed. The left ventricle was small in all the hearts, extreme hypoplasia was observed in 26.2% in presence of a combination of the mitral and the aortic atresia. Most commonly, the combination of mitral stenosis with aortic atresia (36.1%) and combination of mitral and aortic stenosis (32.8%) were present. Mitral atresia with aortic stenosis was identified in 4.9% cases. The mitral valve was usually dysplastic with short and thick chordae and smaller annulus. In 39.3% of all hearts fibroelastosis of the left ventricular endocardium was present. In these cases moderate degree hypoplasia of the left ventricle in combination with mitral and aortic stenosis was often found (65.0%). All the aorta was hypoplastic starting from the aortic valve, across the ascendent part and aortic arch until the isthmus. Its diameter usually varied between 2 and 4 mm. Coarctation of the aorta was found in 16.4% hearts. Persistent left superior caval vein represented the most common associated heart lesion. From the surgical point of view, nearly in all reviewed hearts Norwood surgery, consisting in aortic reconstruction from aorta, main pulmonary artery and a patch from the pulmonary homograft could be performed. Reconstruction would be very difficult in presence of an extreme hypoplasia of the ascending aorta. Until present, possibilities to prevent the development of the left ventricular hypoplasia by a foetal surgical intervention or possibility of the surgical augmentation of the left ventricle by excision of a thick endocardium do not cross the horizons of theory.

[Surgical technique and results of correction of partial, transitional and total forms of atrioventricular septal defects]. / Operacní technika a výsledky korekce cástecné, prechodné a úplné formy defektu atrioventrikulárního septa. Osobní zkusenosti.

During 1982-2001 correction of atrioventricular septal defect was performed by the author in 180 patients: 74 (41.1%) patients with partial, 11 (6.1%) with transitional and 95 (52.8%) with complete form. The age was in range from 1 month to 17 years and 7 months (mean 3.3 +/- 3.8 years). 71 (39.4%) patients were younger than 1 year. The repair consisted in closure of the defect with one or two patches and plastic reconstruction of both atrioventricular valves. In 70.0% patients the "cleft" of the left anterior leaflet was completely closed, in 17.2% commissuroplasty of one and in 6.1% commissuroplasty of both commissures was required. In two patients with stenotic and regurgitant valve mechanical prosthesis was inserted. The surgical mortality was 7.8% (5.4% in partial, 0.0% in transitional and 10.5% in complete form). Out of 115 patients operated before 1994, 14 (12.2%) patients died, but out of 65 patients (including 42 patients with complete form) operated in the period 1995-2001, no patient died. Low cardiac output represented the most common cause of death. Stenotic valvar morphology, complete form and severe clinical condition represented risk factors of death during the early period. Influence of all risk factors was, however, neutralized with time and the early mortality decreased bellow 1% even in the complete form. Surgery performed before 1995 represents the only risk factor of early mortality. After surgery long-term follow-up concentrated to early detection of severe residual or recurrent left atrioventricular valvar incompetence and serious late complications is necessary.

[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.

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.

[Polytetrafluoroethylene vascular prostheses and patches in pediatric cardiac surgery]. / Cévní protézy a záplaty z polytetrafluoroetylénu (PTFE) v pediatrické kardiochirurgii.

The author reviews the indications for the use of vascular grafts, patches, membranes and stitches from polytetrafluoroethylene (PTFE) in pediatric cardiac surgery. Vascular grafts are used for construction of arterial shunts in neonates with complex cyanotic heart defects, for palliative reconstruction of interrupted aortic arch, for construction of extraanatomic aortic by-pass and total cavopulmonary connection. The PTFE patches are suitable for closure of septal defects, plastic reconstruction of the right and the left ventricular outflow tracts, correction of coarctation of the aorta and plastic repair of stenoses on the main pulmonary artery and its branches. PTFE stitches can be used for plastic reconstruction of atrioventricular valves. Membranes represent very good pericardial substitutes after complex surgeries with valve replacement or the use of a valved conduit, where reoperation must be suspected. In neonates and small children with signs of heart failure after surgery, it is possible to use PTFE membranes for transient wound cover when the chest is left opened.

[Extracorporeal membrane oxygenation in the treatment of severe pulmonary hypertension in a neonate after surgery for laparoschisis]. / Mimotelní membránová oxygenace v lécbe tezké plicní hypertenze u novorozence po operaci laparoschízy.

The authors describe the case of newborn with laparoschisis in whom severe idiopathic pulmonary hypertension during postoperative period developed and initiation of extracorporeal membrane oxygenation (ECMO) to maintain circulatory stability and adequate oxygenation was necessary. ECMO was performed for 75 hours with maximum extracorporeal support up to 50% of cardiac output (Biomedicus pump BP 50, Jostra oxygenator M8). Patient was successfully weaned and switched to conventional ventilation and nitric oxide inhalation with consequent extubation. No bleeding complications were observed during ECMO in connection with surgical repair of laparoschisis.

[Aortic reconstruction in neonates with hypoplastic left heart syndrome] . / Rekonstrukce aorty u novorozencu se syndromem hypoplastického levého srdce.

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.

[Extracorporeal membrane oxygenation in the treatment of acute respiratory failure in full-term neonates]. / Pouzití mimotelní membránové oxygenace v terapii akutního respiracního selhání u donosených novorozencu.

OBJECTIVE OF STUDY: Evaluation of success of extracorporeal membrane oxygenation, EMCO) in the treatment of acute respiratory failure in mature neonates. TYPE OF STUDY: Clinical pilot study of the EMCO centre. SETTING: Institute for the Care of Mother and Child, Prague. MATERIAL AND METHOD: In 12 consecutive patients with severe acute respiratory distress syndrome and/or circulatory failure of different etiology who met the indication criteria the method of venoarterial EMCO was used. RESULTS: Venoarterial extracorporeal membrane oxygenation was successful in 75% patients who survived. Four patients died (1x syndrome of cerebral death associated with severe hypoxic-ischaemic encephalopathy, 2x severe irreversible haemorrhage, into the abdominal and thoracic cavity, 1x periventricular intraventricular haemorrhage grade III.). The mean period of EMCO was 71.4 +/- 31.7 hours (range 25-130 hours). On comparison of the surviving group (S) and the non-surviving group (NS) there was a significant difference in the necessity of continuous inotropic adrenaline support during EMCO. In patients who died necropsy confirmed irreversible multiorgan failure. CONCLUSION: In indicated cases extracorporeal membrane oxygenation remains the method of choice in critically ill mature neonates. A prognostically adverse factor is the necessity of inotropic support and haemodialysis during EMCO.

[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.

[Occult fibrin production during surgery in cyanotic congenital heart defects and extracorporeal circulation]. / Skrytá produkce fibrinu pri operacích cyanotických vrozených srdecních vad v mimotelním obehu.

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.

[Initial experience with surgical treatment of tracheal stenosis in small children using extracorporeal circulation]. / Prvé zkusenosti s chirurgickým resením stenózy trachey u malých detí s pouzitím mimotelního obehu.

Severe tracheal stenosis represents a life threatening malformation which necessitates early surgery. Two patients aged one and two years, respectively, were followed-up for signs of congenital stridor. Following respiratory infection both children became critically ill with severe dyspnoea necessitating intubation and artificial ventilation. In the first patient, echocardiography, tracheobronchoscopy and other investigations revealed a pulmonary artery sling with tracheal compression and hypoplasia of the whole trachea. In the second child, presence of a short local tracheal stenosis was found, the cause of which could not be clarified. In the patient with the pulmonary sling, resection and reimplantation of the anomalous left pulmonary artery was performed first. In both children, however, surgical reconstruction of the lower airways was necessary. Surgery was performed from a midline sternotomy approach in extracorporeal circulation. The hypoplastic trachea with circular rings in the first child was enlarged with a pericardial patch. In the second child, the local tracheal stenosis was resected and a direct anastomosis of the trachea was performed. In both patients, transient formation of granulations was observed. Both children, however, survived and their clinical condition remains good 18 and 9 months, respectively, after surgery. Tracheobronchoscopic controls show very good result. Our experience confirms the possibility of successful surgical reconstruction of lower airways in young children using extracorporeal circulation. Good interdisciplinary cooperation between the surgeon and other specialists is an important prerequisite of good surgical results.

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.