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Background Age-appropriate physical activity is an important basis for healthy physical and mental development for children and adolescents with congenital heart defects (CHD) Purpose The main goal of this study was to learn more about the physical activity of young and adolescent CHD-patients to understand patient's needs physical activity and to improve the medical advice regarding physical activity and sports by taking the CHD-severity into account. Methods An online-questionnaire was developed. CHD patients registered in the German National Register for Congenital Heart Defects were invited by email/post to join the study. In total, 1,718 patients took part. 1,262 patients have finished the questionnaire and are included in the statistical analysis (average age: 11.6±3.1 years; 588 women). CHD severity: 411 patients with simple CHD, 423 with moderate CHD, 364 with complex CHD and 64 with unclassified CHD. The study participants were asked to answer questions regarding physical activity and (school) sports to evaluate their activity level and their individual needs regarding sports. Results We identified 831 (65.9%) patients who reported they were exercising sports for at least three days or more for 60 minutes or longer per week (simple CHD: 68.9%; moderate CHD: 64.5%; complex CHD: 63.5%; other CHD: 68.8%). Over all 47 patients (3.7%) stated that they had no regular school sports and 12 patients (1%) reported to have less than one regular sports lessen per week. Out of the 1,215 patients who reported to have regular school sports (simple CHD: 97.3%; moderate CHD: 98.1%; complex CHD: 94.5%; other CHD: 87.5%), the patients stated about how hard they usually work in a regular sports lesson as follows: “without sweating and without shortness of breath” (188 patients, 15.5%; simple CHD: 15.4%; moderate CHD: 17.1%; complex CHD: 14.8%; other CHD: 7.1%), “a little bit sweaty and a bit short of breath” (825 patients, 67.9%; simple CHD: 68.8%; moderate CHD: 65.3%; complex CHD: 68.9%; other CHD: 75%), “a lot of sweat and a lot of shortness of breath” (202 patients, 16.6%; simple CHD: 15.8%; moderate CHD: 17.6%; complex CHD: 16.3%; other CHD: 17.9%). Conclusions Overall, young and adolescent patients with CHD appear to be exercising sports regularly and are well integrated into school sports. Although there are slight differences between the individual degrees of CHD severity. These differences can be interpreted as minor fluctuations.

Background: In pulmonary arterial hypertension (PAH), acute vasodilator response testing (AVT) is considered important to identify adult patients with favorable prognosis using calcium-chan...

The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for the targeted treatment of pulmonary arterial hypertension (PAH). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to the targeted therapy of PAH. This article summarizes the results and recommendations of the working group on targeted treatment of PAH.

BACKGROUND Hyperkalemia is frequently seen during the first days of life in premature infants with a gestational age at birth less than 28 weeks. Normally, these high concentrations of potassium are well tolerated of the premature infants. In a few cases hyperkalemia leads to life-threatening cardiac arrhythmias. CASE REPORT We report about a 800 grams weighing preterm infant born after 26 + 4 gestational weeks. 24 hours after birth the infant developed 2 : 1 atrioventricular block due to hyperkalemia with a heart rate about 75 bpm. The bradycardia continued about 45 minutes in spite of immediate therapy concomitant by circulatory failure that resulted in an intraventricular hemorrhage of grade III with periventricular intraparenchymal lesions. CONCLUSIONS The case report demonstrates the variations of the electrocardiogram that can be found in preterm infants with hyperkalemia and their potential risks. Therapy of symptomatic hyperkalemia is not able to interrupt early a life-threatening circulatory failure in any case.

Infections by Salmonella enteritidis commonly present with diarrhoea, vomiting and fever and complications such as septicaemia, pleural effusion and acute renal failure are usually rare. There are only few reports of cutaneous manifestations and especially septic shock in patients with Salmonella enteritidis infection. We report on a previously healthy seven-year-old boy suffering from Salmonella enteritidis septicaemia presenting with septic shock, pleural effusion, renal failure and an unusual maculopapular skin eruption on both wrists and ankles. The boy had no underlying immunodeficiency.

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiovascular malformation that is characterized by an absent connection of the pulmonary veins to the left atrium. Echocardiographic differentiation of TAPVC and persistent pulmonary hypertension of the newborn (PPHN) can be a great diagnostic challenge. The aim of our study was the assessment of a systematic echocardiographic approach to evaluate the feasibility and reliability of noninvasive diagnosis of TAPVC.Between January 1995 and January 2005, 15 consecutive patients with isolated TAPVC were diagnosed in our institution. 5 patients had supracardiac type, 5 cardiac type, 3 infracardiac and 2 had mixed type of TAPVC. Since 2001 preoperative diagnosis was performed exclusively by echocardiography (8/15 patients).TAPVC could be diagnosed correctly in all cases using a systematic echocardiographic approach with careful investigation of the proximal cardiac veins. Infracardiac type ofTAPVC was diagnosed using systematic evaluation of the liver with visualization of a pathological vessel with venous flow directed away from the heart. In all cases the echocardiographic findings were confirmed during surgery.Echocardiography with Doppler and color Doppler sonography is an effective method for noninvasive diagnosis of TAPVC. The presented systematic echocardiographic approach permits reliable noninvasive differentiation of TAPVC and PPHN.

Dimenhydrinate overdosage in a 3(1/2) year-old-girl with dilative cardiomyopathy. Dimenhydrinate (Vomex(R)) is frequently used in the treatment of sickness and vomiting. The symptoms of overdosage present like an anticholinergic syndrome. We report on the clinical findings of an intoxication with dimenhydrinate in a 3(1/2) year-old-girl with functional dilative cardiomyopathy following a congenital left ventricular diverticle. Especially in small children, with the application of 40 mg suppositories once or twice per day the maximum dose of 3.75 mg/kgBW/d is achieved.

Infections by Salmonella enteritidis commonly present with diarrhoea, vomiting and fever and complications such as septicaemia, pleural effusion and acute renal failure are usually rare. There are only few reports of cutaneous manifestations and especially septic shock in patients with Salmonella enteritidis infection. We report on a previously healthy seven-year-old boy suffering from Salmonella enteritidis septicaemia presenting with septic shock, pleural effusion, renal failure and an unusual maculopapular skin eruption on both wrists and ankles. The boy had no underlying immunodeficiency.

PURPOSE/MATERIALS AND METHODS: The aorto-pulmonary window is a rare congenital cardiac malformation consisting of a connection between the ascending aorta and the main pulmonary artery. Based on 5 consecutive patients who were treated in our tertiary referral centre between 7/2000 and 11/2005, we report the echocardiographic features as well the limitations of this diagnostic method in the assessment of this rare malformation.Dilation of the left atrium and ventricle due to the large left-to-right-shunt was the characteristic echocardiographic feature in all patients with aorto-pulmonary window. In all patients we were able to visualise the window by 2D-echocardiography. Pulsed- and continuous-wave Doppler showed diastolic negative flow in the descending aorta and in systemic arteries due to left-to-right shunting in diastole via the aorto-pulmonary window. All 5 patients had significant associated cardiovascular malformations. They were accurately diagnosed by echocardiography. Coronary anomalies could be excluded noninvasively in all patients.Echocardiography is the method of choice for diagnosis of aortopulmonary window. Detection of aortopulmonary window, however, requires a systematic and careful investigation of all cardiovascular anatomic details, since this anomaly is frequently associated with substantial additional cardiac anomalies, which would by themselves be sufficient to explain the haemodynamic abnormalities of the patient. Cardiac catheterisation is required only in cases with inadequate information about coronary artery anatomy or in older patients with pulmonary hypertension, to determine the patient's suitability for surgical correction.

The compelling safety and efficacy data in numerous large, blinded trials on adult patients, and the progress in device- and leadtechnology have led to increasing use of implantable cardioverter defibrillators in pediatric patients. The purpose of our study was to assess the efficacy and safety of ICD in the pediatric age group of a tertiary referral centre.Between March 1998 and October 2003 12 patients underwent ICD-implantation. The mean age at implantation was 14,8 years with a range between 10-17 years. The underlying cardiac disorders included long QT-syndrome in 4 patients, ventricular fibrillation in 3 patients, dilated cardiomyopathy in 4 patients, and congenital heart disease in 1 patient (pulmonary atresia with ventricular septal defect after Rastelli repair). All patients received a transvenous ICD-system (VVI-ICD in 4 patients, DDD-ICD in 8 patients).The mean follow up was 35 months (6-68 months). During this period there were no severe complications nor mortality. We haven't seen infections, thromboembolic complications or lead-perforations. 2 patients (17 %) received appropriate DC-shocks, 1 patient (8 %) received an inappropriate DC-shock. 10 patients (83 %) had no malignant ventricular arrhythmia under medical therapy. 2 patients (17 %) required revision because of lead-dysfunction. In 2 patients with DCM the device was explanted during orthotopic heart transplantation.Our data demonstrate that advances in device- and leadtechnology have resulted in a decrease of severe complications in the pediatric age group. We conclude that ICD-implantation represents a safe and effective therapy for children and adolescents with lifethreatening ventricular dysrhythmias. Since it represents an invasive therapy, indication should be confined to patients with lifethreatening dysrhythmias according to the guidelines of the American Heart Association.

Hyperkalemia is frequently seen during the first days of life in premature infants with a gestational age at birth less than 28 weeks. Normally, these high concentrations of potassium are well tolerated of the premature infants. In a few cases hyperkalemia leads to life-threatening cardiac arrhythmias.We report about a 800 grams weighing preterm infant born after 26 + 4 gestational weeks. 24 hours after birth the infant developed 2 : 1 atrioventricular block due to hyperkalemia with a heart rate about 75 bpm. The bradycardia continued about 45 minutes in spite of immediate therapy concomitant by circulatory failure that resulted in an intraventricular hemorrhage of grade III with periventricular intraparenchymal lesions.The case report demonstrates the variations of the electrocardiogram that can be found in preterm infants with hyperkalemia and their potential risks. Therapy of symptomatic hyperkalemia is not able to interrupt early a life-threatening circulatory failure in any case.

Theaction of pig pancreatic phospholipase A2 (EC 3.1.1.4) on phosphatidylcholine bilayer is studied under a variety of substrate modification conditions including the incorporation of long chain alcohols (hexanol and several isomeric octanols) into the bilayer. The rate of hydrolysis shows a biphasic dependence upon the concentration of the activating alcohol. The hexanol to lipid molar ratio in the bilayer is approximately 1.4:1 at the optimal alkanol concentration. The lag phase at the beginning of hydrolysis has been shown to depend upon the nature of the bilayer as modified by different alkanols and by intrinsic differences in the unilamellar vesicles (approximate diameter approximately 250 A) compared to the multilamellar vesicles. The rate constant for the activation process responsible for the lag period is first order and does not depend upon the concentration of the enzyme, substrate, alkanol, and calcium. These and other experiments are interpreted in terms of a hypothesis that the pancreatic phospholipase interacts with the bilayer by a catalytic and a recognition site. The data suggest that the packing of the interface regulates the interaction of both the catalytic and the recognition site. It is postulated that the biphasic activation profile as a function of hexanol concentration may be a consequence of two-site interactions between the enzyme and the substrate interface.