Abstract: Congenital stenosis of airways is a rare and possibly life-threatening congenital defect with difficult treatment and uncertain prognosis. In our report, we describe a rare case of a 1-month-old newborn with concomitant stenosis of trachea and main bronchus and its successful treatment using slide plasty.
Abstract: 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.
Abstract: 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.
Abstract: OBJECTIVE: The aim of our study was to analyse experience with repair of truncus arteriosus with interrupted aortic arch. METHODS: Between 1993 and 2004, eight consecutive patients underwent repair of truncus arteriosus with interrupted aortic arch. The median age was 6.5 days (range 1-85 days) and median weight was 3.2 kg (range 2.6-4.8 kg). Five patients had type A and 3 patients had type B aortic arch interruption. The repair was performed in deep hypothermia with circulatory arrest in 4 patients and isolated selective low-flow perfusion of the head and the heart in the last 4 patients. The repair consisted in aortic arch reconstruction by direct anastomosis between descending and ascending aorta, closure of ventricular septal defect and reconstruction of the right ventricular to pulmonary artery continuity using a valved conduit. RESULTS: One (12.5%) patient died from sepsis and hepato-renal failure 18 days after surgery. Seven (87.5%) patients were followed up for 2.0-11.7 years (median 2.6 years). No patient died after the discharge from hospital. In 4 patients 1-3 reinterventions were required 0.6-10.0 years after repair. Reoperations were performed for conduit obstruction in 2 patients, aortic regurgitation in 2 patients, right pulmonary artery stenosis in 2 patients and airway obstruction in 1 patient. In 2 patients concommitant aortic valve and conduit replacement was required. Balloon angioplasty for aortic arch obstruction was necessary in 1 patient, and for bilateral pulmonary branch stenosis in 1 patient. Five (28.6%) surviving patients are in NYHA class I and 2 (71.4%) patients are in NYHA class II. CONCLUSIONS: Primary repair of persistent truncus arteriosus with interrupted aortic arch can be done with low mortality and good mid-term results. Aortic arch reconstruction in isolated low-flow perfusion of the head and the heart influences favourably the postoperative recovery. The main postoperative problems are associated with conduit obstruction and aortic insufficiency.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: AIM: To assess the outcome and mortality trend in newborns undergoing corrective surgery for congenital heart defect. METHOD: We reviewed the hospital records of 1,033 neonates under 30 days of life, who had congenital heart defects operated on at the Kardiocentrum, Motol University Hospital in Prague, Czech Republic, during 1977-2001. Early and late mortality and reoperation rates were analyzed. RESULTS: A total of 1,156 operations were performed in 1,033 neonates. Obstructive lesions were surgically treated in 56%, left-to-right shunts in 21%, and complex conotruncal lesions in 23% of the cases. Total correction has been achieved in 62% of the neonates. Most operations (75%) were performed in the first two weeks of neonate s life. Early 30-day hospital mortality was 13%. Late mortality, after the discharge from the hospital, was 10%. In the last three years, the hospital mortality rate decreased to about 2%. Out of 590 reinterventions in 379 neonates, with the mortality of 6%, 229 were secondary staged corrections and 190 further palliative procedures aimed mostly toward Fontan or Rastelli type of circulation. Residual or recurrent defects were solved in 62 neonates. There were 30 valve replacements, with 18 mechanical valves and 12 pulmonary valve autotransplantations (the Ross procedure). The homograft valved conduit was used in 38 children. CONCLUSION: Most newborns with critical congenital heart defects can undergo corrective operation under acceptable risk. Due to complex improvements in perioperative, anaesthetic, surgical, and postoperative care, contemporary hospital mortality can be reduced to 1-3%. Palliative procedures still play an important role in the staged treatment of severe complex heart defects in neonates.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: Thanks to the co-operation with Czech and Slovak Transplant Centres and with some of the Departments of Pathology and Forensic Medicine 274 hearts were collected for allograft heart valves (AHV) processing during 1992-1995. The Cardiac surgeon dissected the aortic valve with the root and the pulmonary artery trunk with the valve. Tissues were antibiotically (ATB) sterilised in cultivation medium E 199 (24 hours at 37 degrees C). ATB concentrations (mg/ml): Cepharin 0.2, Azlocilin 0.2, Tobramycin 0.08 and Amphotericin B 0.1 for harvesting at post-mortem (P) or Miconazol 0.05 for sterile retrieval during multiorgan harvesting (MOH). After sterilisation AHV were stored at 4 degrees C. 49 AHV were infected even after ATB treatment-15 of 35 collected at P (43%) and 35 of 218 procured during MOH (16%)-(p < 0.01-ch2 test). After serological screening of the donor and microbiological testing the AHV were released for clinical use. Most AHV were programmed cooled to the temperature of liquid nitrogen (-196 degrees C), in which they were stored at the Allograft Heart Valves Cryobank. Cryoprotection was achieved by 10% dimethylsulphidoxide. CONCLUSION: A technology of harvesting, processing, storage and transportation of AHV, was introduced. It enabled the routine use in many cardiac surgical units. The AHV Cryobank was established. 131 AHV were used clinically between 1992 and 1995 (transportation as far as 1300 km). 108 AHV (82.4%) were used for repair of complex congenital heart defects, while 23 (17.6%) were used for aortic valve and/or root replacement.
Abstract: The cause of paresis of the diaphragm after cardiosurgery is damage of the phrenic nerve. The diagnosis of paresis is based on X-ray examination, sonography and electromyography of the diaphragm. Plication of the diaphragm is indicated only in those children with paresis of the diaphragm who develop during spontaneous ventilation severe respiratory insufficiency. In the Cardiocentre of the Faculty Hospital Prague-Motol between 1983 and 1996 of 5333 children operated on account of heart disease 29 children were subjected to plication of the diaphragm, incl. five where the operation was made during the neonatal stage (17%), 20 in infant age (69%) and four were older than one year (14%). By the third day after plication 9 children (38%) could be disconnected from the respirator, by the 5th day 20 children (70%) by the 7th day 22 children (75%). In neonates and infants with postoperative paresis of the diaphragm, where spontaneous ventilation cannot be induced, plication of the diaphragm is according to the authors the method of choice. It is a rapid and safe surgical operation which reduces the period of artificial ventilation and its complications.
Abstract: The authors describe the treatment of postoperative mediastinitis in cardiosurgery by the open method with local sucrose administration and subsequent delay of closure of the thorax. Universal antibacterial properties of sucrose are made use of. Sucrose dissolves in the wound in its crystalline form and creates a medium with low aqueous activity (high osmolarity), which inhibits bacterial growth. The wound is rapidly cleansed, the secretion from the wound is within 10 days bacteriologically negative and the chest can be closed secondarily. Simple resuture of the wound was performed four times, a plastic operation using the pectoral muscles twice and in one instance it was closed by a plastic operation of the transposed rectus abdominis muscle. The method was used successfully in seven patients.
Abstract: 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.
Abstract: 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.
Abstract: 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.
Abstract: METHODS AND RESULTS. From the group of 110 neonates born with transposition of the great arteries during 1991-1994 that were referred for the treatment to Kardiocentrum, University Hospital Prague-Motol, 46 neonates with simple transposition were operated on according to the criteria for anatomical correction (arterial switch) at the mean age of 9 days (4-20 days). Fourteen infants with transposition and large ventricular septal defect were corrected with arterial switch at the mean age of 2.5 months (5 weeks-9 months). Fifty neonates that did not meet criteria for arterial switch procedure were indicated for correction at the atrial level (Senning procedure) that has been performed at the mean age of 5 months (1-10 months). Out of 46 operated neonates 10 died following the operation and one child died 2 months later after surgery. There were 2 death out of last 20 neonates (10%). Thirty five children surviving 1-4 years after anatomical arterial correction of transposition are without complaints in excellent condition, NYHA class I. CONCLUSIONS. The procedure of the anatomical correction at the level of the great arteries (arterial switch) according to Jatene, that has been successfully introduced at Kardiocentrum, University Hospital Prague-Motol, has been reproducible and became the method of choice for operations of transposition of the great arteries in neonates with the suitable anatomy.
Abstract: During 47 re-operations on the open heart the authors used the method of autotransfusion, predeposition of the patient's blood and intravenous administration of aprotinin to reduce postoperative haemorrhage and blood consumption. The patients were classified according to the methods or their combinations used into three groups and the authors compared, using statistical methods the blood losses, blood consumption, haemolysis, renal function and the effect of the methods used on the morbidity. The results were compared with a control group where the mentioned methods of economizing on blood were not used. The authors recorded significantly higher haematocrit values in all three groups where economic methods were used at the end of the extracorporeal circulation (EC), as compared with the control group. The volume of the administered blood transfusion was significantly lower in group 1 where an autotransfusion apparatus Cell saver was used. The volume of the administered blood transfusion in the other groups did not differ when evaluated by statistical methods. The filling of the apparatus for extracorporeal circulation was blood free in 90% in group 1, in 75% in group 2, in 92.2% in group 3 and in 75% in the control group. Blood losses via thoracic drains did not differ significantly in different groups though there was a wide range of recorded values. In both groups 2 and 3 where patients were given aprotinin haemoglobinuria was more frequent, as confirmed by laboratory tests. The authors observed also a greater diuresis, without laboratory evidence of impairment of renal function.(ABSTRACT TRUNCATED AT 250 WORDS)
Abstract: Neonatal cardiosurgery is nowadays an integral part of treatment of congenital heart disease. At present it is possible to correct completely with a feasible risk heart diseases in the majority of neonates (73%) with a critical congenital disease. Most frequently these disease defects are operated during the first two weeks of life (78% of all operations). In the strategy of surgery optimal timing and selection of the most suitable operation is essential to ensure in time favourable conditions for child growth and development and a long high-quality life. The tactics and therapeutic results in contemporary neonatal cardiosurgery are illustrated by the authors' experience with 174 neonates operated on for congenital critical heart defect in 1991-1993 in the Pediatric Cardiocentre, University Hospital Prague-Motol.
Abstract: Primary correction of an interrupted aortic arch was performed in seven neonates aged 2 to 26 days. The operation was performed from median sternotomy with extracorporeal circulation using hypothermic arrest of the circulation for reconstruction of the aortic arch. In all neonates it proved possible to make a direct anastomosis of the ascendent and descendent aorta. At the same time in six children a defect of the ventricular septum was closed, in two the common arterial trunk was corrected, in one resection of subaortic stenosis was performed and in another child correction of the aortopulmonary window. After the operation three neonates died from sepsis and multiorgan failure. At present four patients are alive who, 10 to 20 months after operation, are in a good condition. All have a non-restrictive anastomosis of the aorta. Primary correction of an interrupted aortic arch and associated defects is preferred by the authors to two-stage surgery.
Abstract: The authors studied the surgical anatomy of atrioventricular valves (AV) in defects of the atrioventricular septum (AVSD) in a group of 73 preparations of the heart. Evidence was provided of a continual spectrum of changes of the entire valvular apparatus from a common AV orifice (71.2%) to two-separate orifices (28.8%). In the defect with a common orifice the authors differentiated six grades of overlapping of the left-sided component of the anterior common cusp with the right ventricle. In 12.3% hearts the authors revealed stenosis of the left AV orifice, which was most frequently caused by a thickened valve with reduced mobility. In 19.2% hearts the authors found a potentially stenotic configuration of the left AV orifice. From the surgical aspect it was possible to divide AVSD into the complete, transitional or partial type. The results of the morphological investigation were used during correction of 121 patients with AVSD. Of those 54 suffered from the complete form, 10 from the transitional and 57 from the partial form. During correction the authors closed the defect by using one or two patches and they performed an aimed plastic operation of the AV valves. The plastic repair involved closure of the cleft of the septal cusp of the left AV orifice, commissuroplasty, suture of abnormal commissures and straightening of irregular margins of the valve. In eight patients the plastic operation could not be performed because of a potentially stenotic configuration. The early mortality rate was 7.5% in the partial and transitional form and 18.5% in the complete form. The results were adversely affected by an unfavourable anatomy of the left AV orifice with severe regurgitation. From the great variability of the anatomy of AV valves and the authors' clinical experience ensues the necessity of an exact surgical solution of AVSD with an aimed and individually modified reconstruction of AV valves.
Abstract: In Cardiocentrum of the University Hospital in Prague-Motol in 1988-1994 anatomical correction of transposition of the great arteries (arterial switch) was performed in 47 neonates aged 4 to 20 days. The aorta and pulmonary artery were transferred into the appropriate ventricle concurrently with reimplantation of the coronary arteries. The surgical technique was modified with regard to the anatomy of the coronary arteries and the presence of associated cardiac defects. Eleven of the 47 neonates (23%) died during the early postoperative period, one patient died two months after operation. Of the last 20 operated neonates only two died (10%) and both had an abnormal insertion of the coronary arteries. Thirty-five children are followed up for 2-45 months following anatomical correction. All are in a very good clinical condition without serious residual findings. The authors describe the protocol of the diagnostic and therapeutic procedure in transposition of the great arteries. They consider anatomical arterial correction in the neonatal period as the method of choice for this disease. The optimal age for anatomical correction in isolated transposition is between the 7th and 14th day and in transpositions with a major defect of the ventricular septum at the age of one month. The surgical results are steadily improving with accumulating experience. The medium-term results of anatomical correction of transposition of the great arteries are very favourable.
Abstract: Morphology of atrioventricular septal defect was studied on 73 heart specimens. All of them had a defect of atrioventricular septum and adjoining parts of atrial and ventricular septum, a shortening of inflow parts of both ventricles, a malformation of atrioventricular valves and changed position of aortic valve. There was a great variability of all the studied structures and nearly continuous spectrum of specimens was found as for the ventricles' size, localization and diameter of the defect, communication between atria and ventricles, anatomy of the left ventricle outflow tract as well as the whole atrioventricular valvular apparatus and occurrence of associated heart malformations. A common ostium was formed in 52 cases (71.2%), two separated ostia were found in 21 cases (28.8%) but a continuous transition between both groups was noticed. Both groups showed a great variety of single cusps of atrioventricular valves (in shape, number and anchoring), of papillary muscles and of the size of atrioventricular communication. The finding of frequently associated heart malformations was important from the clinical point of view, especially of the obstructive malformations of the left ventricle. A great morphological variability needs individual surgical solutions, namely reconstruction of valves, closure of septal defects and correction of associated heart malformations.
Abstract: Total cavopulmonary connection (TCPC) allows surgical treatment of patients with complex univentricular heart disease. Between 1991 and 1993 this operation was performed in 15 cyanotic children with complex heart defects. Age range was 3.5-14 (median 8.3) years. In 5 children with increased operative risk a calibrated interatrial fenestration was created intraoperatively to allow systemic venous decompression. Three patients (20%) died. In remaining 12 patients a significant increase in arterial oxygen saturation and decrease of polycythemia was encountered. All patients remain in a good clinical condition 1-25 months after operation. Noninvasive investigation including echocardiography confirmed good function of the heart.
Abstract: In the child cardiocentre in Prague 5-Motol in 1977-1993 a total of 420 neonates with critical inborn heart disease were operated. Obstructive defects of the left heart were found in 178 children, obstructive defects of the right heart in 87, defects with a left-right shunt with pulmonary hypertension in 75, conotruncal malformations in 73 and various operations were made in 7 children. Complete repair of the defect was achieved in 281 neonates, incl. 104 where extracorporeal circulation was used. Palliative operations were made in 139 children. Early mortality during the entire period was 26%, whereby a decrease from 40% to 16% was recorded during the last three years. At present it is possible to repair permanently critical inborn heart disease in the majority of neonates. This is made possible in particular by early non-invasive diagnosis, treatment with prostaglandins E in duct-dependent critical heart disease, optimal time for and selection of most suitable surgery, microsurgical technique, miniaturization of extracorporeal circulation and the method of deep hypothermia.
Abstract: Anatomical correction with an intraventricular tunnel, as suggested by Kawashima, was performed in 14 children with a double-outlet right ventricle and a subpulmonary (4) or non-committed (10) defect of the ventricular septum with one early and one late death. Nine children had previous palliative operations. Complications after radical surgery were residual ventricular defects (4), infectious endocarditis (2) and impaired rhythm (one sudden death). Twelve patients are after medium- term or long-term follow-up free from subjective complaints, ten children report good performance, in two the performance is slightly reduced. Anatomical intraventricular correction of double-outlet right ventricle restores the mitral valve and left ventricle to the systemic circulation. The prognosis of children with double-outlet right ventricle and subpulmonary or remote ventricular defect improves substantially.
Abstract: The adolescent generation of patients operated in childhood on account of congenital heart disease is a new health and social problem. Kardiocentrum for children in Motol started its activities in 1977. A concept of care for children with congenital heart disease was elaborated and gradually all surgical methods used worldwide were introduced. The advances in cardiosurgery along with the development of diagnostic methods and intervention catheterization in particular balloon valvuloplasty and angioplasty make high standard and effective treatment of practically the entire spectrum of congenital heart disease in children of all age groups possible. The cardiac surgeon tries nowadays to resolve problems of inborn heart disease already in early childhood. Before a possible re-operation in adult age he must take into account how the result of the first operation affected the patient's growth and development. He is interested in particular in the fate of implants, surgically induced pathology and the influence of growth on the reconstruction. Despite intensive research, so far the long-term prognosis of patients after some types of surgery is not clear (e.g. transposition of the great arteries, Fontan's operation and its modifications). An adult patient congenital hearth disease should receive care of a specialized cardiologist who has personal experience with the diagnosis and treatment of congenital heart disease. If surgery is necessary it should be performed in a specialised department equipped as regards staff, technical devices and funds for these special operations.
Abstract: Four children with double outlet right ventricle type Taussig-Bing were operated in 1988-1990 by anatomical correction-arterial switch. Primary correction of the defect was performed in one infant at the age of three months. Three children had a previous palliative operation (banding of the pulmonary artery, resection of coarctation of the aorta and ductus arteriosus). Anatomical correction of the defect was performed in a second stage at the age of 14, 15 and 19 months. One child died from Gram-negative septicaemia on the 8th day after operation. Three children are completely free from complaints and develop normally. The authors discuss some technical aspects of the arterial switch operation in infants with double outlet right ventricle.
Abstract: Forty four children underwent surgery for congenital vascular anomalies causing tracheal compression in Kardiocentrum--Motol during last ten years. Double aortic arch was encountered 18 times, tracheal compression by the innominate artery in 12 instances, right aortic arch with left ligamentum arteriosum and possibly aberrant left subclavian artery 7 times, pulmonary artery sling in 5, finally aberrant right subclavian artery twice. The age of the children was 2 weeks to 11 years (median 6 months). Stridor was present in 38 infants under 3 months, 17 children with severe dyspnoea had artificial ventilation or were resuscitated or hospitalized at the intensive care department. In four an associated cardiac defects was present. The diagnosis was made by oesophagography, echography, laryngotracheoscopy, in the majority of children it was confirmed by angiography of the sometime with tracheobronchography. The operation was performed in 41 children from left-sided thoracotomy, the vascular ring was released by resection or retraction of the compromising vessel and connective tissue from the trachea. Serious stenosis of the distal trachea in one patient found before operation persists one year after operation, the patient breathes through an individual tracheostomic cannula. Four children could not be extubated for prolonged periods after operation, they were re-operated, the annulus was released. Three patients died, one from bronchopneumonia during the postoperative period, the second one from hypoxic brain damage and the third one after operation of a complex heart disease. In 40 children satisfactory long-term results were achieved, the operation resolved respiratory complaints. The prerequisite of satisfactory results in an accurate diagnosis, early indication of the operation and a perfect surgical operation and good postoperative care.
Abstract: A group of post-mortem specimens of 82 hearts with a defect of the atrioventricular septum (atrioventricular canal) was retrospectively examined with special attention to the morphology of the defect in relation to the left atrioventricular anastomosis. In 42 preparations the finding was compared with previous angiocardiography and post-mortally with X-ray pictures of the specimens. Correlation of the findings revealed malformation of the left atrioventricular orifice as part of a complex malformation affecting the valvular apparatus in conjunction with the atrioventricular septal defect. In the very variable anatomical morphology a major part is played by the superior cusp of the left atrioventricular anastomosis. The relationship of the superior cusp to the outflow tract of the left ventricle and to the atrioventricular septal defect has an impact on the variability of the angiocardiographic picture, and last but not least, also on the results of surgical treatment. The anteroposterior, left oblique and hepatoclavicular projections are optimal for visualization of the anomaly.
Abstract: Total repair of tetralogy of Fallot was performed in 69 patients after the primary construction of a Blalock (28 patients) or a Waterston (41 patients) anastomosis. A total of 7 patients (10.1%) died. One to 5 yrs after correction, 62 patients (89.9%) survive: 52 (75.4%) are in a very good condition, 7 (10.1%) are in a good condition and the condition of 3 (4.4%) is unsatisfactory. 8 (11.6%) patients have serious late complications. The result of correction is not much influenced by the previous Blalock anastomosis (mortality rate 6.9%). After the Waterston anastomosis, however, there are frequent serious complications (pulmonary hypertension in 25.0% of patients), which adversely affect the result and increase mortality (12.5%) of the subsequent correction. The authors prefer two-stage repair of tetralogy of Fallot in the first two years of life. By administration of beta-blockers and prostaglandins they endeavour to postpone the construction of a Blalock anastomosis or an anastomosis performed by means of a polytetrafluoroethylene vascular prosthesis up to the age when the pulmonary artery branches are at least 4 mm in diameter. They plan the subsequent correction to be performed between the 2nd and the 5th year of age.
Abstract: During a 5-year period (1978-1983) corrective surgery for tetralogy of Fallot was performed in 114 patients, ranging in age from 2 to 24 years (mean 8.4 years). Forty-one of them (35.9%) had previously undergone a palliative operation. Insertion of a transannular patch was necessary in 48 patients (42.1%). Eleven children died in the immediate postoperative period (early mortality 9.6%); no deaths occurred later. 103 survivors were followed-up for periods ranging from 5 months to 5 years mean 2.4 years) after surgery. The condition in 91 patients was excellent (88.7%), in 11 good (10.7%) and in one poor. Residual ventricular septal defect persisted in 9 patients (8.7%). Postoperative ECG revealed complete right bundle branch block in 71 patients (59%), incomplete block in 18 (18%), 5 patients (5%) had complete RBBB with left anterior hemiblock. Postoperative dysrhythmia requiring treatment was found in 7 patients (6%). Of 82 patients thoroughly examined by echocardiography, 37 (45%) had pulmonary regurgitation, 29 (35%) pulmonary plus tricuspid regurgitation, and 3 (4%) isolated tricuspid regurgitation. 83% of the patients had impaired lung function.
Abstract: The author analyse results of anastomosis in 120 children with cyanotic heart disease operated on in the first two years of life. At the age up to 3 months of life 21 infants were operated on, 11 (52.4%) of whom died in connection with the procedure. Between the 3rd and 24th month of life 99 children were operated on with a death rate of 11.1%. In 94 children hypoxic spells disappeared, cyanosis and hypoxia were relieved. In the majority of patients, however, the duration of an improved general condition was limited to five years. Both immediate and long-term results depended first of all on the age of the child and on the type of heart lesion. Patients with tetralogy of Fallot had the lowest operative mortality (7.1%) and the best long-term results. In pulmonary atresia, which has poor prognosis without operation, the results of anastomosis were the least gratifying. The authors consider an anastomotic operation, especially the Blalock and Waterston shunt, to be an important life-saving procedure in infants with cyanotic congenital heart disease with hypoxic spells and critical hypoxia.
