Improving medical care and prevention in adults with congenital heart disease-reflections on a global problem-part II: infective endocarditis, pulmonary hypertension, pulmonary arterial hypertension and aortopathy.

Despite relevant residua and sequels, follow-up care of adults with congenital heart disease (ACHD) is too often not performed by/in specialized and/or certified physicians or centers although major problems in the long-term course may develop. The most relevant encompass heart failure, cardiac arrhythmias, heart valve disorders, pulmonary vascular disease, infective endocarditis (IE), aortopathy and non-cardiac comorbidities. The present publication emphasizes current data on IE, pulmonary and pulmonary arterial hypertension and aortopathy in ACHD and underlines the deep need of an experienced follow-up care by specialized and/or certified physicians or centers, as treatment regimens from acquired heart disease can not be necessarily transmitted to CHD.

Improving medical care and prevention in adults with congenital heart disease-reflections on a global problem-part I: development of congenital cardiology, epidemiology, clinical aspects, heart failure, cardiac arrhythmia.

Today most patients with congenital heart defects (CHD) survive into adulthood. Unfortunately, despite relevant residua and sequels, follow-up care of adults with congenital heart disease (ACHD) is not performed in specialized and/or certified physicians or centres. Major problems in the long-term course encompass heart failure, cardiac arrhythmias, heart valve disorders, pulmonary vascular disease, infective endocarditis, aortopathy and non-cardiac comorbidities.

Recommendations for improving the quality of the interdisciplinary medical care of grown-ups with congenital heart disease (GUCH).

Background/objectives: The number of adult congenital heart disease (ACHD) patients will be larger in the medium to long term than that of children and adolescents with congenital heart disease. The present structures for the medical care of ACHD patients are not sufficient and need to be improved. Therefore the task force's assignment and objective was to develop recommendations for the structure of the interdisciplinary medical care of adults with congenital heart disease (GUCH).

Recommendations for adult and paediatric cardiologists on obtaining additional qualification in "Adults with Congenital Heart Disease" (ACHD).

Background: The number of adult congenital heart disease (ACHD) patients will be larger in the medium to long term than that of children and adolescents with congenital heart disease. The present structures for the medical care of ACHD patients are not sufficient and need to be improved. Therefore the Task Force aimed at developing recommendations for adult and paediatric cardiologists to acquire the additional qualification "Adults with Congenital Heart Disease" (ACDH).

Liver transplantation in a subject with familial hypercholesterolemia carrying the homozygous p.W577R LDL-receptor gene mutation.

Mutations within the low density lipoprotein (LDL)-receptor gene result in familial hypercholesterolemia, an autosomal dominant inherited disease. Clinical homozygous affected subjects die of premature coronary artery disease as early as in early childhood. We identified a girl at the age of five yr with clinical homozygous familial hypercholesterolemia presenting with achilles tendon xanthomas and arcus lipoides.

A search for chromosome 22q11.2 deletions in a series of 176 consecutively catheterized patients with congenital heart disease: no evidence for deletions in non-syndromic patients.

Microdeletions in chromosome 22q11.2 are associated with DiGeorge syndrome (DGS), velo-cardio-facial syndrome (VCFS), and several other syndromes, collectively referred to as DG/VCF. Non-dysmorphic patients with cardiac defects have also been attributed to deletions in this chromosomal region.

Pulsed Doppler echocardiographic assessment of patterns of venous flow after the modified Fontan operation: potential clinical implications.

To assess the effect of a modified Fontan operation on systemic venous blood flow and the hepatic circulation, we compared 11 patients having an atriopulmonary connection and 35 with total cavopulmonary anastomosis. The Doppler echocardiographic study of the caval venous, hepatic venous and portal venous flow was performed so as to calculate the pulsatility ratio and the variation of flow with respiration. All patients had undergone cardiac catheterization.

Early and late atrial dysrhythmias after modified Fontan operation.

Unlabelled: Occurrence of supraventricular tachycardia and sinus node dysfunction was investigated pre- and postoperatively by serial ECG and Holter monitors in 63 consecutive patients with univentricular circulation after modified Fontan operation (total cavopulmonary connection 39 patients, atriopulmonary connection 24 patients). Mean age at operation was 7.2 (0.

Sotalol associated torsades de pointes tachycardia in a 15-month-old child: successful therapy with magnesium aspartate.

Torsades de points (Tdp) is a form of ventricular tachycardia, and its occurrence in childhood is very rare. In adult patients treated with sotalol, Tdp has been reported to the occur with an incidence of 2%-4%. In children who are treated with sotalol, occurrence of Tdp has been reported in only a single case.

[Evaluation of congenital vena cava anomalies and acquired vena cava obstructions after atrial switch operation using spiral computerized tomography and 3-dimensional reconstruction].

We report spiral-CT findings in adult patients with congenital abnormalities of the vena cava and systemic venous obstructions after atrial switch operation. Especially systemic venous obstruction is a well-known complication following Mustard procedure for transposition of the great arteries. The results demonstrate that computed tomography, particularly with the use of 3-dimensional surface reconstruction, is very useful as a highly sensitive procedure for the detailed depiction of abnormalities of the vena cava or of residua and sequelae after inflow correction for complete transposition.

[Total cavopulmonary anastomosis for definitive palliation of complex univentricular cardiovascular systems--potential preoperative risk factors].

The modified Fontan operation for definitive palliation has been performed in an increasing number of patients with various forms of a functionally univentricular heart. To eliminate the influence of different surgical techniques only patients after total cavopulmonary anastomosis (TCPA) were included in this retrospective analysis of preoperative risk factors. The age at operation in 72 patients varied from 7 to 219 months (median 54 months); 29 patients had been younger than 4 years.

[Coronary sinus defects: rare form of interatrial communication].

Unlabelled: A coronary sinus defect results in a communication of variable size between the coronary sinus (CS) and the left atrium (LA). It is in most cases associated with a persistent left superior vena cava (l-SVC) and an atrial septal defect of the coronary sinus type and often part of a more complex cardiac malformation. If the CS to LA fenestration is the single cardiac defect, this anomaly provides the basis of an unusual form of interatrial communication, which may cause diagnostic difficulties.

[Spiral CT and 3-dimensional reconstruction in evaluation of systemic venous obstruction after atrial switch operation for complete transposition of great vessels].

We report findings in spiral-CT from 11 adolescent or adult patients after atrial switch operation. The results demonstrate that computed tomography, particularly with the use of three-dimensional surface reconstruction, is very useful as a highly sensitive procedure for the detailed depiction of residua and sequelae after inflow correction for complete transposition. Especially systemic venous obstruction (SVO) is a well know complication following Mustard procedure for transposition of the great arteries.

Modified Fontan operation in functionally univentricular hearts: preoperative risk factors and intermediate results.

Background: The modified Fontan operation has been proposed as definitive palliation for an increasing variety of hearts with complex univentricular anatomy. To eliminate the influence of different surgical strategies, only patients undergoing total cavopulmonary anastomosis were included in this retrospective study.

Methods: Seventy-two patients had been operated on at ages ranging from 7 to 219 months, with 29 patients younger than 4 years.

Isolated aortic coarctation in neonates and infants: results of resection and end-to-end anastomosis.

Background: Operative resection is the treatment of choice for native aortic coarctation in most institutions. The ideal timing for elective repair is still a matter of debate. This study evaluated one institution's results with resection and end-to-end anastomosis in the first year of life.

[Intermediate and long-term outcome after percutaneous balloon dilatation of valvular pulmonary stenoses in childhood].

Pulmonary balloon valvuloplasty was performed in 52 patients aged 7 days to 19 years (mean 5.5 years). Mean balloon/anulus ratio was 1.

[Atresia of the left-sided atrioventricular connection with single ventricle and transposition of great vessels in a adult patients].

Mitral atresia is an extremely rare congenital anomaly in which survival into adulthood is an exception. Without treatment most patients with this anomaly will die in early childhood and only few reach adolescence or early adulthood. The here described patient with mitral atresia, atrial septal defect, single ventricle and transposition of the great arteries survived to age 24 years.

Familial idiopathic atrial fibrillation with bradyarrhythmia.

Unlabelled: A 10-year-old boy is reported who presented with idiopathic atrial fibrillation and bradyarrhythmia. After history of intrauterine and postnatal bradycardia, atrial fibrillation was first documented electrocardiographically at 16 months of age. An underlying structural heart disease was not evident.

[Atrial flutter in young patients after corrective operation of congenital heart defects: therapy by high frequency catheter ablation].

Unlabelled: Radiofrequency catheter ablation has been used successfully in adult patients for treatment of atrial flutter. Three young patients (mean age 14.6 years) with common type atrial flutter (n = 2) and uncommon type atrial flutter (n = 1) underwent electrophysiologic study.

[2D and Doppler echocardiography after atrial switch operation for transposition of great arteries; a review].

Introduction of the atrial baffle procedure (Mustard, Senning) more than 30 years ago has dramatically improved the prognosis of patients with transposition of the great arteries. However, a range of late complications can occur even in clinically asymptomatic patients including: superior and inferior limb obstruction, pulmonary venous obstruction, residual baffle-leakage, left ventricular outflow tract obstruction, tricuspid valve regurgitation, right ventricular dysfunction and postoperative dysrhythmia. As an increasing number of patients has reached adulthood an accurate noninvasive technique is needed for routine follow-up studies.

Total cavopulmonary anastomosis in patients less than three years of age.

Background: As young age at modified Fontan operation was thought to be a preoperative risk factor for poorer survival, we studied early and intermediate outcome in our young patient group.

Methods: Results in children less than 3 years of age (group I; n = 26; age range, 7 to 35 months) were compared with those in older patients (group II; n = 46; age range, 36 to 219 months). For both groups there was no significant difference with regard to preoperative pathology and hemodynamics.