Survival and Freedom From Reinterventions in Patients With Repaired Tetralogy of Fallot: Up to 42-Year Follow-Up of 917 Patients.

Background To evaluate long-term outcome of tetralogy of Fallot repair analyzing an unbiased country-wide surgically treated population with tetralogy of Fallot. Methods and Results Retrospective analysis of consecutive patients aged <18 years who underwent tetralogy of Fallot repair at a single nationwide pediatric cardiac center. Death from any cause and need for surgical or catheter reintervention were the study end points.

Long-Term Survival and Freedom From Coronary Artery Reintervention After Arterial Switch Operation for Transposition of the Great Arteries: A Population-Based Nationwide Study.

Background The aim of this study was to evaluate long-term survival and freedom from coronary artery reintervention after the arterial switch operation (ASO). Methods and Results This single-center nationwide retrospective study included consecutive children who underwent ASO between 1990 and 2016 (n=605). Long-term outcomes were obtained by cross-mapping individual data with the National Death Registry and the National Registry of Cardiovascular Interventions for adults.

[Czech paediatric cardiac surgery - history and presence].

The beginnings of the Paediatric Cardiac Surgery in the Czech Republic date back to the period immediately after the end of World War II. Its protagonists were Prof. Emerich Polák from the Surgical Clinic in Prague, Vinohrady, Prof.

Aggressive tumoral calcinosis in an infant thoracotomy scar.

The authors report a case of tumoral calcinosis (TC) in a six-month-old infant, which developed within the thoracotomy scar from previous aortic coarctation repair. After initial resection of the lesion, the child returned with a large mass of TC restricting movement of the left shoulder. Repeated total resection was successful with no recurrence in 12 months' follow-up.

Improved results after the primary repair of interrupted aortic arch: impact of a new management protocol with isolated cerebral perfusion.

Objective: The aim of this retrospective study was to analyse results and risk factors of death after the repair of the interrupted aortic arch, and to compare results obtained with the use of deep hypothermic circulatory arrest versus isolated cerebral perfusion.

Methods: The primary repair of the interrupted aortic arch and associated heart lesions was performed in 50 consecutive patients. The median age was 5 days and the mean weight was 3.

Long-term results after correction of persistent truncus arteriosus in 83 patients.

Objective: This study aims to analyse long-term results after correction of truncus arteriosus in all patients operated in one institution over 28 years.

Methods: Between 1981 and 2009, 83 patients, median age 54 days, underwent repair of truncus arteriosus. Interrupted aortic arch was present in 14 (17%), severe truncal valve insufficiency in 10 (12%) and non-confluent pulmonary arteries in five (6%) patients.

Repair of persistent truncus arteriosus with interrupted aortic arch.

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.

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.

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

Objectives: This study aimed to evaluate the technical feasibility and hemodynamic benefit of cardiac resynchronization therapy (CRT) in patients with systemic right ventricle (RV).

Background: Patients with a systemic RV are at high risk of developing heart failure. Cardiac resynchronization therapy may improve RV function in those with electromechanical dyssynchrony.

Phospholipid composition of myocardium in children with normoxemic and hypoxemic congenital heart diseases.

Samples of myocardial tissue were obtained during cardiac surgery from children operated for different types of normoxemic and hypoxemic congenital heart diseases. The phospholipid composition was analyzed by thin layer chromatography. The concentration of total phospholipids (PL), phosphatidylcholine and phosphatidylethanolamine (PE) was found lower in atrial tissue of both normoxemic and hypoxemic groups in comparison with the ventricles.

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

Oncocytic cardiomyopathy is a rare arrhythmogenic disorder usually associated with female sex, difficult-to-control arrhythmias, or sudden death of infants and children. Morphologically, it is characterized by the presence of oncocytic cells, which are diffusely distributed or form the nodular structures within the myocardium, occasionally involving the valves, with a large number of mitochondria in cytoplasms. We present two cases of oncocytic cardiomyopathy.

Corrective surgery of congenital heart defects in neonates: the Prague experience.

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.

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.

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

The surgical technique and tactics of the Norwood operation in neonates with hypoplastic left heart syndrome represent a surgical challenge. The Norwood operation was performed from the midline sternotomy approach, in extracorporeal circulation and deep hypothermic circulatory arrest was used for reconstruction of the hypoplastic aorta. Operation consisted of reconstruction of the "neoaorta" and the aortic arch from the original hypoplastic ascendent aorta, pulmonary trunk and a patch cut from a pulmonary homograft or pericardium, excision of the atrial septum and an arterial shunt from a Goretex vascular graft 3.

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.

Late complications and quality of life after atrial correction of transposition of the great arteries in 12 to 18 year follow-up.

Background: Most adolescents and young adults born with transposition of the great arteries and survivors of Mustard operation are alive today and are leading ordinary lives. How far is the quality of their life influenced by residual findings?

Methods: We have analysed the oldest group of 177 children operated on with the Mustard procedure during 1979-1984 from our total experience of 609 corrections of transposition of the great arteries until 1996. In the long-term follow-up 12 to 18 years after Mustard operation the clinical investigation, 24 hours ECG Holter monitoring, 2D and colour flow Doppler echocardiography, radionuclide angiocardiography and exercise testing were performed.

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

In 10 patients with complex cyanotic congenital defects detailed coagulation examinations were made at the beginning and end of the extracorporeal circulation after neutralization of heparin by protamine and the results were compared with a control examination, made before general heparinization, after introduction into general anaesthesia. The authors examined the activated period of blood (ACT) by means of testing tubes with a celite activator (Hemochron) as well as the HR-ACT test with a kaolin activator (Medtronic) for comparison of the results. The authors assessed quantitatively plasma levels of heparin, antithrombin III and fibrinopeptide A which is a sensitive indicator of intravascular coagulation.

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

The authors present the case-history of a two-day-old neonate with clinical signs of critical coarctation of the aorta where echocardiography revealed thrombosis of the aortic arch and isthmus. By surgery it was impossible to remove the organized thrombus from the aorta, therefore the portion of the aorta with the thrombus was resected and the aorta was reconstructed by an end-to-end anastomosis. After surgery long-term anticoagulation treatment with Warfarin was started.

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

Objective: To test the effect of heart disease and heart surgery on lung function.

Design: A pulmonary function study of children undergoing surgery for atrial septal defect (ASD).

Settings: University hospital.

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).

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.

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

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

Successful reoperation for severe left bronchus compression after repair of persistent truncus arteriosus with interrupted aortic arch.

Signs of the left bronchus compression, caused by aneurysmatic dilatation of the aortic root with severe aortic regurgitation, occurred 5 months after repair of the truncus arteriosus with interrupted aortic arch in an 85-day-old infant. At reoperation the dilated ascending aorta was replaced with a 14-mm Dacron tube. The aortic valve was replaced with an 18-mm Carbomedics valve.

[Primary repair of interrupted aortic arch in neonates].

In the Kardiocentrum, University Hospital Motol, Prague, protocol of the primary repair of interrupted aortic arch was introduced, and between 1993-1997, 15 neonates aged 1-26 days (median 5 days) were operated on. Treatment with prostaglandins E for maintenance of the ductal patency, correction of metabolic acidosis, and treatment of all complications were necessary before surgery. The correction was performed from the midline sternotomy approach, in extracorporeal circulation and deep hypothermia with circulatory arrest.

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

Background: Reoperations in cardiac surgery of congenital heart defects represent not only the difficult technical problem but also a prognostic one. They are demanding reconstructive procedures with broad spectrum of hazards and are definitely cumulating the operative risk.

Methods And Results: At the Pediatric Kardiocentrum, University Hospital Motol, Prague, there were 2250 children operated on for congenital heart defects in the first year of life and followed up during 1969-1996.