Repetitive ventricular tachycardia in a syncopal child: Cause or incidental finding ?

A 15-year-old girl suffered recurrent syncopal episodes during 7 years. Events were precipitated by exercise or emotional stress, leading to the diagnosis of reflex syncope. Exercise testing induced recurrent salvos of nonsustained right ventricular outflow tract tachycardia. This arrhythmia is often asymptomatic, reflex syncope is very frequent and both causes are related to the same triggering situations. It was therefore essential to obtain recordings during syncopal events and to observe the clinical evolution under effective treatment in order to make the right diagnosis.

Chronotropic impairment after surgical or percutaneous closure of atrial septal defect.

BACKGROUND: In previous studies, an attenuated heart rate response to exercise has been noted in patients after surgical closure of atrial septal defect. The aim of this study was to compare the prevalence of chronotropic impairment after surgical and percutaneous closure of atrial septal defect. METHODS: Thirty-eight pediatric patients who underwent a surgical (group A, n = 18) or transcatheter closure (group B, n = 20) of atrial septal defect in our institution were prospectively included in the study. Treadmill exercise testing was performed using the Bruce walking treadmill protocol to voluntary exhaustion, with continuous monitoring of heart rate and oxygen consumption. RESULTS: All the children were in sinus rhythm, and had normal values for peak oxygen uptake and endurance time. Exercise heart rate was significantly lower than normal in group A at the end of stage 2 (Z-score = -0.71 +/- SD 1.02), and in both groups at the end of stage 3 (Z-score = -2.06 +/- 1.76 in A and -1.00 +/- 0.71 in B) and at peak exercise (Z-score = -2.78 +/- 2.14 in A and -0.81 +/- 0.75 in B). However, the heart rate response to exercise was significantly less attenuated in group B than in group A. Moreover, maximal heart rate was <-2 SD in 8/18 surgical patients but in no patient of the group B. CONCLUSION: The chronotropic impairment is significantly less important after transcatheter closure of atrial septal defect than after surgical closure. It is an additional argument in favor of the interventional catheterization.

Eculizumab in anti-factor h antibodies associated with atypical hemolytic uremic syndrome.

Atypical hemolytic uremic syndrome (aHUS) is a life-threatening multisystemic condition often leading to end-stage renal failure. It results from an increased activation of the alternative pathway of the complement system due to mutations of genes coding for inhibitors of this pathway or from autoantibodies directed against them. Eculizumab is a monoclonal antibody directed against complement component C5 and inhibiting the activation of the effector limb of the complement system. Its efficacy has already been demonstrated in aHUS. The present article reports for the first time the use of eculizumab in a patient presenting with aHUS associated with circulating anti-complement Factor H autoantibodies and complicated by cardiac and neurologic symptoms. Our observation highlights the efficacy of eculizumab in this form of aHUS not only on renal symptoms but also on the extrarenal symptoms. It also suggests that eculizumab should be used very promptly after aHUS presentation to prevent life-threatening complications and to reduce the risk of chronic disabilities. To obtain a complete inhibition of the effector limb activation, the advised dosage must be respected. After this initial therapy in the autoimmune aHUS form, a long-term immunosuppressive treatment should be considered, to prevent relapses by reducing anti-complement Factor H autoantibody plasma levels.

Workload of ambulatory activities in a tertiary paediatric cardiac centre.

BACKGROUND: Few data are available on the evolution in the number of referrals and the spectrum and frequency of issues addressed in paediatric cardiac outpatient clinics. AIM: To assess the volume and range of symptoms and diagnoses in patients, referred to a paediatric cardiac outpatient clinic in an academic hospital setting. METHODS: Data were collected prospectively over 6 months. RESULTS: Historical comparison showed that the number of outpatient visits increased from 819 during the first semester of 2004 to 865, 1045 and 1391 during the first semesters of 2005, 2006 and 2007, respectively. During the 6-month study period in 2007, 854/1391 visits concerned patients with known heart disease; the reason for the visit was follow-up of congenital heart disease (n=616 children, 128 adults), arrhythmia (n=91) or acquired heart disease (n=19). During the visit, the decision to perform diagnostic or therapeutic cardiac catheterization or a surgical procedure was taken in 47 cases. Foetal echocardiography was performed in 60 foetuses and was abnormal in 21 cases. Among the other 477 visits, which concerned patients without known heart disease, the most frequent clinical concern was cardiac murmur (n=193) and there were new diagnoses of congenital heart disease (n=28), ventricular dysfunction (n=2) and arrhythmia (n=7). CONCLUSIONS: The ambulatory paediatric cardiology workload in tertiary academic hospital settings is increasing alarmingly. These data may be helpful in future planning of consultant manpower and in curriculum development for cardiac training of students and residents.