Double-Chambered Left Ventricle Diagnosed in Fetus and Follow-up During the First Year of Life: A Case Report.

Double-chambered left ventricle (DCLV) is a rare congenital cardiac lesion. It is usually an incidental finding in children and young adults. Diagnosis during fetal life is exceedingly rare. We report a case of DCLV diagnosed in the prenatal period associated with a ventricular septal defect (VSD). Transthoracic echocardiographic and magnetic resonance imaging confirmed the prenatal findings. At seven-month follow-up, the baby was asymptomatic. Transthoracic echocardiographic check-up revealed spontaneous closure of the VSD and stable aspect of the main left ventricle and the accessory chamber.

The yield of 24-hour ambulatory electrocardiography in the assessment of symptomatic school-age children.

INTRODUCTION: Lightheadedness, syncope, dyspnea, chest pain, palpitation, loss of consciousness (LOC) or malaise are a frequent reason for consultation in school-age children (SAC). The yield of holter monitoring (HM) in the investigation of these symptoms in SAC is still controversial given the scarce studies. AIM: To determine the prevalence of baseline ECG abnormalities and those detected in HM in SAC, and to assess the predictive factors of abnormal HM test. METHODS: We conducted a retrospective descriptive study in which we had included all the consecutive SAC (6 to 12 years) presenting: syncope or lightheadedness or palpitation, dyspnea, or chest pain, malaise, LOC and referred to our department for HM. RESULTS: We included 366 children. Mean age was 9.7 ± 1.88 years. The major symptoms experienced by the patients were: palpitation (50.7%), chest pain (16.9%), lightheadedness (11.9%), syncope (6.9%), LOC (5.3%), ECG was abnormal in 8.7%. The two most common baseline ECG abnormalities were premature ventricular contractions (PVCs) (1.8%) and right bundle branch block (1.6%). HM was positive in 101 (26.6%) patients. The most common abnormalities in HM were vagal hypertonia in 61 patients (16.1%), PVCs in 29 patients (7.7%), Malignant abnormalities were encountered in 16 patients (13.9%) whereas benign abnormalities in 99 patients (86.1%). In the multivariate analysis: Age≥10 years, mean heart rate <94 beats per minute, the presence of syncope, LOC, malaise and the presence of PVCs in baseline ECG were identified as independent risk factors of abnormal holter. CONCLUSION: HM has an important diagnostic value in symptomatic SAC. It allows the identification of benign as well as malignant arrhythmias, which require urgent management.

Giant aortic arch aneurysm complicating Kawasaki's disease.

Kawasaki disease (KD) is a common acute vasculitis in pediatric population that usually involves small- and middle-sized arteries, commonly coronary arteries. Although the incidence and natural course of coronary aneurysms after KD are well documented in studies, related reports on peripheral arterial and aortic aneurysms are scarce. We report the occurrence of a giant aortic aneurysm involving the horizontal part of aortic arch in a 28-month-old boy diagnosed with KD. This complication was managed by steroids therapy in the beginning. Because of mechanical complication and potential risk of rupture, surgery was undertaken.

Clinical and genetic investigation of pediatric cases of Wolff-Parkinson-White syndrome in Tunisian families.

BACKGROUND: Wolff-Parkinson-White (WPW) syndrome is an autosomal-dominant heart disease characterized by an accessory pathway that arises from an aberrant conduction from the atria to the ventricles. Several mutations within the PRKAG2 gene were shown to be responsible for WPW. This gene encodes the γ2 regulatory subunit of adenosine monophosphate (AMP)-activated protein kinase, which functions as a metabolic sensor in cells, responding to cellular energy demands. METHODS: This first study of WPW in a North African population comprises the clinical and genetic investigation of 3 Tunisian families, including 11 affected members. The involvement of the PRKAG2 and NKX2-5 genes was investigated. RESULTS: Mutation screening showed that with the exception of two already reported single-nucleotide polymorphisms, no mutations were detected within the coding region of PRKAG2 or in the NKX2-5 gene. CONCLUSIONS: This study provides further evidence of the genetic heterogeneity of WPW.

[Anatomic repair of transposition of the great arteries or arterial switch operation. Report of 62 cases]. / La reparation anatomique de la transposition des gros vaisseaux ou switch arteriel. A propos de 62 cas.

Between January 1990 and September 2003, 62 patients underwent anatomic repair of a transposition of the great arteries. Mean operative age is 40 days. Transposition of the great arteries was simple in 38 cases and associated to a large ventricular septal defect in 24 cases. 44 patients have had an atrial septostomy of Rashkind and 45 an infusion of prostaglandin E 1.5 patients with simple transposition of the great arteries have had left ventricular retraining before arteriel switch. In association to arterial switch, were performed closure of ventricular septal defect in 24 cases, cure of coarctation of the aorta in 4 cases and cure of an abnormal partial pulmonary venous return in 1 case. Early mortality was 6,45%. After a mean follow up of 3 years, one patient died suddenly (late mortality is 1.72%) and one patient had to have 2 reoperations. Results of anatomic repair are now excellent. Late mortality is essentially related to coronary complications so that a careful follow-up is mandatory.