[Chest pain in children and adolescents - the heart?]. / Thoraxschmerzen bei Kindern und Jugendlichen - das Herz?

Chest pain in children and adolescents is a frequent observation, although potentially relevant disease is rather rare and then found in situations with acute presentation. In children with an inflammatory/infectious clinical context the differential diagnosis is oesophagitis, pleuropneumonia or pericarditis. Potentially dangerous complications may be found in youth with predisposing conditions for aortic dissection, pneumothorax or pulmonary embolism, or even in rare instances for an acute coronary complication. In these cases aggressive diagnostic work-up is mandatory. In the frequent elective outpatient evaluation of teenagers with long-lasting episodes of chest pain, relevant underlying cardiovascular disease only rarely can be found as the cause. In the elective outpatient evaluation for chest pain, usually patient history and clinical examination may be enough to track the problem, the main role of the physician is to provide reassurance with minimal but appropriate testing.

[Needle in the haystack - potentially dangerous syncope in childhood]. / Nadel im Heuhaufen - potentiell gefährliche Synkopen bei Kindern.

Syncope is a frequently observed symptom in pediatrics with teenagers being the age group most often affected. In contrast to older age, organic cardiac causes of syncope in child-hood are observed in a minority of only 2-5% of cases, in their majority pediatric syncopes thus are neurocardiogenic in origin. The rare organic cardiac causes that may manifest with syncope are all potentially dangerous entities such as cardiomyopathies, genetic primary electrical disease and some forms of structural heart disease, as well as some other rare diseases such as e.g. primary pulmonary hypertension. These diseases have to be actively looked for or excluded. Guidelines recommend patient evaluation including history, physical examination and ECG, which is sufficient to sort out suspect cases after a syncopal episode.

[Vasculitis as a reason of chronic headache]. / Vaskulitis als Ursache von Kopfschmerzen. Systemischer Lupus erythematodes.

A 13-year-old girl presented to our emergency with a one week history of fever and skin rash and new onset of chorea for the last three days. There was a long standing history of right predominant headache; followed by personality change, fatigue, arthralgia and weight loss over the last few months. Previous investigations by head CT and ophthalmological examination did not explain the symptoms. Further investigations revealed peri- and pancarditis with aortic insufficiency, a renal involvement with elevated creatinin, protein- and hematuria and a hemolytic anemia. Diagnosis of lupus eythematodes was confirmed by high ANA, anti-dsDNS and Anticardiolipin antibodies. Within the first 48 hours after admission there was significant deterioration with reduced vigilance and dysarthria. MRI of the brain and dopplersonography of cerebral vessels showed a complete thrombosis of the right medial cerebral artery with a small net of collaterals, irregularities of the left cerebral artery due to vasculitis and several subacute leftsided ischemias. Immunosuppressive therapy with high-dose corticosteroids and cyclophosphamid together with antithrombotic therapy induced an improvement of neurologic, renal and cardiac function.

[Practical management of common arrhythmias in children]. / Der Umgang mit Herzrhythmusstörungen beim Kind.

Premature supraventricular or ventricular beats are frequently seen in children of all age groups and are in most instances a benign condition. Intermittent and infrequent premature beats usually are not an indication for a cardiologic work-up. If ventricular or supraventricular premature beats are frequent and persist over a period of weeks or months, we usually recommend a cardiological evaluation. In those patients where ventricular or supraventricular tachycardias are found, the treatment is based on symptomatology, frequency of arrhythmias and impact on quality of life. In ventricular tachycardias, the presence of underlying cardiac disease is of prognostic importance and treatment more aggressive. Idiopathic ventricular tachycardia without structural heart disease usually shows a benign course and has a good prognosis.

[Cardiac emergencies in infancy and childhood]. / Kardiologische Notfälle im Kindesalter.

There are relatively few situations where cardiac emergencies can occur in a child. A special focus is directed to the cyanotic newborn. In these cases a very specific treatment plan is necessary which is limited to a few dedicated centres. The importance lies in the timely diagnosis of a cyanotic heart defect and the rapid and safe transport from the referring hospital. An outline is given on the causes and treatment of congestive heart failure in infancy. Management guidelines are given for the most frequent types of cardiac arrhythmias and the few occasions, where an arrhythmia can lead to a real emergency, mostly in infants. To complete the list of the most frequent cardiac emergencies in pediatrics, pericardial effusion/tamponade is mentioned as well as the occurrence of cyanotic spells typically in the baby with Fallot's tetralogy.

[Chest pain in children and adolescents]. / Thoraxschmerzen beim Kind und Jugendlichen.

Chest pain is a frequent complaint in children and adolescents. True organic disease is rare, especially cardiac disease is infrequently found as the underlying cause. Organic disease is associated with acute onset, fever, chest pain exclusively with physical activity and with abnormal physical findings in the patient. In the majority the symptom is not caused by organic disease, in these cases longer duration of symptoms is typically found as well as higher patient age, typically teenagers. Stress factors such as troubles in school and family need to be asked for. Detailed history and a meticulous physical examination usually allow for a clear delineation of the problem, making additional diagnostic work-up unnecessary in most instances. Laboratory tests remain indicated only upon suspicious clinical or anamnestic signs and are not efficient as widely used screening tools.

Low incidence of respiratory syncytial virus hospitalisations in haemodynamically significant congenital heart disease.

BACKGROUND: Haemodynamically significant congenital heart disease (CHD) is a risk factor for severe respiratory syncytial virus (RSV) disease in young children. Population based data on the incidence of RSV hospitalisations in CHD patients are needed to estimate the potential usefulness of RSV immunoprophylaxis using palivizumab. AIMS: (1) To obtain population based RSV hospitalisation rates in children <24 months of age with CHD. (2) To compare these rates with non-CHD patients and with previous studies. (3) To determine the number of patients needed to treat (NNT) with palivizumab to prevent one RSV hospitalisation. METHODS: Six year, longitudinal, population based study at an institution, which is the sole provider of primary to tertiary in-patient care for a precisely defined paediatric population. RESULTS: RSV hospitalisation rates (per 100 child-years) in CHD patients aged <6, <12, 12-24, and <24 months of age were 2.5 (95% CI 0.8 to 5.6), 2.0 (0.8 to 3.8), 0.5 (0.1 to 1.8), and 1.3 (0.6 to 2.3), respectively, and the relative risk (RR) in comparison with non-CHD patients was 1.4 (0.6 to 3.1), 1.6 (0.8 to 3.2), 2.7 (0.7 to 9.7), and 1.8 (1.0 to 3.3), respectively. NNT was between 80 (35 to 245) and 259 (72 to 2140) for various age groups. CONCLUSION: RSV hospitalisation rates in CHD patients were fourfold lower than reported from the USA. Based on these low rates and RR, unrestricted use of palivizumab does not appear to be justified in this study area.

Impaired poststenotic aortic pulsatility after hemodynamically ideal coarctation repair in children.

Using echocardiographic quantification of aortic pulsatility distal to the site of the surgical anastomosis, we evaluated whether the preoperatively impaired poststenotic aortic pulsatility returned to normal after repair of coarctation with a hemodynamically ideal result. Patients who underwent repair of aortic coarctation without residual obstruction were compared to a matched group of normal children. A standardized M-mode echocardiographic evaluation of the aorta at the diaphragmatic level was performed for all patients. Measurements consisted of maximum and minimum aortic diameters, time intervals, and a calculated pulsatility index. Compared to normal children (n = 19), 20 children with operated coarctation and with a hemodynamically ideal result showed a significantly smaller increase in aortic diameter in systole (mean of 29 +/- 7% in patients versus 37 +/- 7% in normals; p < 0.01). In contrast to patients with coarctation in whom the maximum aortic distension is reached much later during the cardiac cycle, hemodynamically normalized, operated patients in our study had no such delay (maximum aortic pulsation at 28% of cardiac cycle time compared to 27% in normals; p = not significant). The pulsatility index of the poststenotic aorta was clearly lower in operated children (mean, 130 +/- 50%/sec) compared to a normal mean value of 202 +/- 33%/sec but was still significantly higher than that in patients with unoperated coarctation, who showed a low mean value of 51 +/- 24%/sec (p < 0.01). After correction of aortic coarctation with a hemodynamically ideal result, the pulsatility of the poststenotic aorta, severely impaired prior to repair, did not return to normal during the observation period in the patients studied.

[Arrhythmias in pediatric patients]. / Arrhythmien im Kindesalter.

Arrhythmias in children occur frequently as isolated phenomena, however, may also represent comorbidities of congenital heart disease. Therefore electrophysiologic assessments must include morphologic and hemodynamic evaluations. Besides the arrhythmias commonly seen in adults children present with arrhythmias unique to pediatric patients. Antiarrhythmic therapy depends on patient age, expected natural history of the arrhythmia and possible congenital heart disease. As in adult patients in addition to antiarrhythmic drug therapy radiofrequency catheter ablation is a preferred definitive treatment especially in patients with supraventricular tachycardias. Bradycardias can be successfully treated with pacing systems which can already be implanted in the newborn period. As antiarrhythmic treatment in pediatric patients may be very complex a referral to a pediatric cardiology center is recommended.

[Syncopes in children and adolescents]. / Synkopen bei Kindern und Jugendlichen.

Syncopes are frequent in the pediatric age group. The majority of these events are benign and are so called neurocardiogenic syncopes. In a minority of affected children, a cardiac pathology is the underlying cause, these cases need to be recognized promptly as almost all of the underlying cardiac causes are potentially fatal conditions. Usually detailed history of syncopal events allows for differentiation between neurocardiogenic and other causes of syncope. In case the physical examination is normal, a simple ECG as the only additional laboratory test is adequate. Other laboratory tests are performed on the basis of suspicious findings in both history and physical examination. The typical neuricardiogenic syncopes rarely require medication in childhood, usually addressing some behavioural aspects with the patient are sufficient as only therapeutic measures.

Cardiopulmonary exercise parameters in children with atrial septal defect and increased pulmonary blood flow: short-term effects of defect closure.

UNLABELLED: Markedly increased pulmonary blood flow because of a relevant atrial septal defect (ASD) leads to impaired cardiopulmonary function during maximum exercise in adults. No comparative preoperative and postoperative data are available on the short-term effects of shunt closure on cardiorespiratory function at peak exercise in children. Pulmonary function testing at rest and cardiopulmonary exercise testing together with haemodynamic assessment was done prospectively in children with an ASD preoperatively and again after full recovery at 3-4 mo postoperatively and compared with a matched normal population. Sixteen children, aged 6.8-16.1 y, with a defect of 8-23 mm (median 15 mm) and a pulmonary/systemic flow ratio of 1.5-3.5 (median 2.2) were tested and compared with 15 healthy children. Preoperatively, baseline pulmonary function parameters and exercise capacity were no different from normals. At peak exercise, patients with a shunt had increased pulmonary resistance, especially of the distal airways (p = 0.04), with a significantly larger proportion of children having a paradoxical increase in total airway resistance during exercise (p < 0.05). Maximum serum lactate at peak exercise was elevated (p < 0.05) in patients. In patients, maximum oxygen uptake was impaired (p = 0.03) and remained so at repeat evaluation postoperatively. The same observation was made for chronotropic response to exercise. CONCLUSION: Cardiopulmonary exercise parameters in patients with ASD differed only slightly from those in normal children. The most important deviations were a lower maximum oxygen uptake and an increase in airway resistance at maximum exercise.

Delayed recognition of haemodynamically relevant congenital heart disease.

UNLABELLED: Delayed recognition of congenital heart defects may have a serious impact on the long-term outcome of the children affected. It was the aim of the present study, to evaluate the proportion of children with delayed cardiac diagnosis out of a large cohort of consecutive paediatric patients requiring treatment for congenital heart disease. A prospective study was performed over a 3-year period. Of all 323 paediatric patients requiring surgical (n = 291) or catheter interventional (n = 32) treatment for congenital heart disease, patients with delayed diagnosis of their cardiac defects were observed and especially examined for the presence of clinical cardiac findings other than systolic murmurs, not recognized as such prior to referral. Of all the patients, 32 (10%) had delayed diagnosis of heart defects. Surprisingly, the proportion of late diagnoses was not different in the group of patients with cyanotic heart disease where 7/72 patients were referred with delay, compared to 25 delayed referrals among 251 children with acyanotic heart defects. Of the 32 patients with delayed diagnosis, 7 had complications due to delayed referral, but there was no mortality associated with late diagnosis. CONCLUSION: A substantial proportion of all paediatric patients requiring intervention for heart disease were diagnosed with relevant delay. In all study patients with late diagnosis, clinical cardiac findings other than systolic murmurs were present that should have alerted the physician on the possible presence of underlying heart disease.

Dilation of the ascending aorta in childhood: 4 cases without obvious predisposing disease.

Dilation of the ascending aorta is rare in childhood. When seen, it is usually associated with some form of connective tissue disease or predisposing cardiac malformations, especially an aortic valve with two leaflets. We describe four children in whom significant dilation of the ascending aorta was encountered as an incidental finding. No patient had any sign of an associated connective tissue disease, nor did we detect any predisposing cardiac anomalies. One patient had undergone surgical ligation of the arterial duct in infancy, whilst another had undergone repair of aortic coarctation, also in infancy. A third child has had repair of an atrioventricular septal defect with exclusively atrial shunting, whereas the fourth patient had a structurally normal heart. The aortic valve had three leaflets, and was functionally normal in all. The dilation of the ascending aorta was progressive in all patients, and finally surgical treatment was recommended, relying on the guidelines established for the management of patients affected with the Marfan syndrome.

[New approaches in cardiac surgery for the therapy of congenital heart disease]. / Neue kardiochirurgische Aspekte bei der Therapie kongenitaler Herzfehler.

Recent developments in the field of pediatric cardiac surgery and pediatric cardiology have led to significant changes in the surgical approach to the various cardiac malformations. There is a clear trend towards surgical treatment at a younger age of the patient, towards complete correction of malformations instead of staged procedures with initial palliation and delayed correction. Perioperative cardioprotection has improved significantly as has postoperative management in specialized pediatric cardiac intensive care units. As a result of this, mortality and morbidity of pediatric cardiac surgery have been in steady decline, although complexity of cardiac surgery in even smaller children has increased. Preoperative diagnostic work-up of the cardiac malformation can nowadays be achieved in an always increasing number of patients without invasive cardiac catheterization and based exclusively on ultrasound findings alone. A new element comes from the possibilities of therapeutic cardiac catheterization, which now allows for a combined therapeutic approach using therapeutic catheter procedures either to perform preparatory interventions prior to surgery or to treat surgical complications in the postoperative course. Refinements in the use of surgical implants have led to new possibilities of correction of complex malformations early in life, especially with regard to the use of biological prosthesis for valvar or outflow tract replacement.

[Diagnosis and treatment of common pediatric supraventricular tachycardias]. / Diagnose und Therapie häufiger supraventrikulärer Tachykardien.

The incidence of paediatric supraventricular tachycardias is approximately 0.1-0.4%. AV node and AV reentrant tachycardias represent the most common supraventricular tachycardias in children. Atrial ectopic tachycardias are the second most common group of pediatric arrhythmias with a predominance in younger children. All these arrhythmias may result in a significant morbidity and necessitate therapy. During the last decade the widespread use of newer antiarrhythmic agents and the invention of radiofrequency catheter ablation have revolutionized the treatment of supraventricular tachycardias in children. A thorough knowledge of these various treatments options is essential to achieve a safe and effective management of supraventricular tachycardias in children. This article focuses on diagnostic and therapeutic aspects of the most frequent supraventricular tachycardias.

Modified repair of mixed total anomalous pulmonary venous connection.

Mixed total anomalous pulmonary venous connection (TAPVC) is a rare condition, accounting for only 5% of diagnosed TAPVC. It is associated with a poor prognosis unless surgically corrected by connecting the pulmonary venous sinus to the left atrium and optionally dividing the abnormal connection to the systemic venous system. We report a modified technique of complete intracardiac repair of mixed supracardiac and cardiac TAPVC in a 3-year-old child without pulmonary venous sinus-to-left atrium anastomosis.

Treatment of supraventricular tachycardias in the new millennium--drugs or radiofrequency catheter ablation?

UNLABELLED: Supraventricular tachycardias are a cause of significant morbidity in children. Newer anti-arrhythmic drugs and the invention of radiofrequency catheter ablation have revolutionised anti-arrhythmic therapy in children. CONCLUSION: With the availability of these very effective therapeutic options, an improved knowledge of these methods and their indications is required.

Surgical management of complications after transcatheter closure of an atrial septal defect or patent foramen ovale.

OBJECTIVE: During recent years, transcatheter closure has become an alternative to operations for the treatment of atrial septal defects and patent foramen ovale. However, this procedure may be unsuccessful or complicated and requires surgical treatment. METHODS: We retrospectively analyzed the outcomes of patients who needed surgical treatment after failed or complicated transcatheter closure of an atrial septal defect or a patent foramen ovale. RESULTS: Between April 1994 and March 1999, 124 patients were treated with transcatheter closure of an atrial septal defect or a patent foramen ovale at our institution. We report the results of 10 (8%) patients of this series who required operations after transcatheter closure attempts. In 8 of these 10 patients a significant shunt caused by malposition or dislocation of the device persisted, leading to surgical closure of the defect. In 2 patients injury of the femoral artery at the puncture site required surgical repair. In one patient the device had to be removed surgically from the iliac vein after retraction. One patient died of left ventricular perforation after dislocation of the device and several surgical attempts to close the left ventricular rupture. All other patients recovered well. CONCLUSION: An operation was required after transcatheter closure of an atrial septal defect or a patent foramen ovale in 8% of patients. After device complications, the atrial septal defect and the patent foramen ovale can still successfully be closed surgically with good results and low morbidity. However, serious complications like cardiac perforation may have a fatal outcome. Residual shunt, dislocation, or vascular complications are the most frequent problems that require surgical interventions.

Myopericarditis associated with central European tick-borne encephalitis.

UNLABELLED: The case of an 11-year-old child with acute myopericarditis associated with central European tick-borne encephalitis is presented. Cardiac involvement was demonstrated by pericardial effusion, elevated serum concentration of troponin-I and cardiac arrhythmia. Co-infections with enteroviruses, Borrelia burgdorferi or the agent of human granulocytic ehrlichiosis were excluded. Recovery was uneventful. CONCLUSION: Central European tick-borne encephalitis can be complicated by cardiac involvement.