Giant left atrial appendage aneurysm in a neonate.

Giant aneurysm of the left atrial appendage (LAA) is a rare condition typically presenting in adulthood. This case report describes the investigations and surgical management of a giant LAA aneurysm in a neonate, emphasizing the role of echocardiography and cardiovascular magnetic resonance imaging in preoperative evaluation as well as challenges in surgical repair of this defect.

Fears and anxiety in children with long-QT syndrome compared to children with asthma.

OBJECTIVE: To compare children with asthma to children with long-QT syndrome (LQTS) in terms of anxiety and medical fears. METHOD: Forty children (25 males/15 females) with asthma and their mothers participated, along with seven children with LQTS (four males/three females) and their mothers. RESULTS: Children with asthma had significantly more medical fears, fear of danger/death, and fear of minor injury and small animals compared to children with LQTS. Children with LQTS tended to have more fear of failure and criticism, and tended to keep their feelings to themselves and minimize their real feelings of anxiety. Children with LQTS had significantly more internalizing problems, and their mothers had significantly higher anxiety. CONCLUSION: Fear and uncertainty can be overwhelming in LQTS. Children with LQTS do not seem to be able to share their feelings openly. Examining the psychosocial adjustment of affected children may assist professionals to help families to cope more effectively.

QT intervals in metabolic dilated cardiomyopathy.

OBJECTIVES: The present study determined the prevalence of dilated cardiomyopathy together with prolonged corrected QT (QTc) intervals in children. The study also examined whether an association exists between prolonged QTc intervals and ventricular dysrhythmia in a patient cohort with dilated cardiomyopathy. BACKGROUND: The morbidity and mortality for pediatric patients with dilated cardiomyopathy remains high and is a clinical challenge. The patient population includes a significant number of Hutterite patients with metabolic disease associated with dilated cardiomyopathy. METHODS: Thirty-eight pediatric patients with dilated cardiomyopathy were reviewed for the presence of prolonged QTc and dysrhythmias. Eleven patients had a metabolic etiology for their dilated cardiomyopathy. RESULTS: Thirty-six per cent of the patient cohort had a long QTc interval. After 50 months of follow-up, the probability of survival for a child with a long QTc interval was approximately 50%. The probability of survival for a child with a normal QTc interval was 72%. Seventy per cent of the patients who died had a metabolic etiology for their dilated cardiomyopathy and a long QTc. CONCLUSIONS: Dilated cardiomyopathy may be associated with a prolonged QTc and may increase the patient's risk for sudden death. The presence of a metabolic etiology for dilated cardiomyopathy increases the risk of death.

Electrocardiogram interpretation and management in a pediatric emergency department.

OBJECTIVES: To determine the accuracy of electrocardiogram (ECG) interpretation by pediatric emergency physicians through comparison with a pediatric cardiologist and to determine the intrarater and interrater reliability for pediatric emergency physicians and cardiologists. METHODS: This was a prospective cohort study in which pediatric emergency physicians ordering an ECG completed a standardized questionnaire. The same emergency physician, a second emergency physician, and a pediatric cardiologist also completed the questionnaire for all ECGs at a later time. A randomly selected subset of ECGs was also interpreted by the same cardiologist and a second pediatric cardiologist. Major outcome variables were (1) whether the ECG was normal or abnormal, and if abnormal, (2) whether the abnormality represented a minor or major concern, and (3) whether the ECG warranted referral to a pediatric cardiologist. RESULTS: For pediatric emergency physicians, the intrarater and interrater kappa values were 0.56 and 0.24 for the presence of an abnormality, 0.49 and 0.36 for level of concern, and 0.63 and 0.25 for need of cardiology follow-up. For pediatric cardiologists, the intrarater and interrater kappa values were 0.82 and 0.92 for the presence of an abnormality, 0.71 and 1.00 for level of concern, and 0.82 and 0.91 for need of cardiology follow-up. A comparison of the initial emergency physician and cardiologist interpretations yielded kappa values of 0.42 for the presence of an abnormality, 0.16 for level of concern, and 0.31 for need of cardiology follow-up. CONCLUSIONS: When compared with interpretation by a pediatric cardiologist, ECG interpretation by pediatric emergency physicians was relatively inaccurate; intrarater and interrater agreement among emergency physicians was good and poor, respectively, and the intrarater and interrater agreement among pediatric cardiologists was excellent.

Surgical outcome following treatment of isolated subaortic obstruction.

Surgical and nonsurgical patients with isolated subaortic stenosis (SAS) were compared to determine the important factors contributing to the timing of surgical intervention. This study reviews 49 consecutive patients (27 surgical and 22 nonsurgical) aged 1.8 to 15.9 years with isolated SAS. The preoperative peak left ventricular outflow tract (LVOT) gradient in surgical patients was significantly higher than the gradient in nonsurgical patients (59.0 +/- 30.4 vs 22.77 +/- 13.9 mm Hg, P = .0001). The progression in LVOT gradient analyzed by echo Doppler was significantly higher in the surgical group compared with the nonsurgical group (10.48 +/- 9.7 vs 1.56 +/- 6.5 mm Hg/y, P = .007). Repeat surgical intervention was required in 22% of patients in the surgical group for recurrence of SAS, and 4% needed a third surgery. The progression in the severity of aortic regurgitation (AR) was not significantly different in the surgical and nonsurgical groups. There was a significant association between the development of AR and patients undergoing surgery (P = .045). AR may not be a reliable indication for early operative intervention in isolated SAS as there was no significant difference in its progression with surgical and nonsurgical patients. Asymptomatic patients with isolated SAS may warrant surgical intervention on the basis of progression of LVOT gradient, rather than the development or progression of AR.

Congenital right ventricular diverticulum in association with disseminated neonatal hemangiomatosis.

The case of a term infant with disseminated neonatal hemangiomatosis and congenital right ventricular diverticulum is reported. Visceral hemangiomas were present in the liver and kidneys and the patient had lower gastrointestinal bleeding that resolved. The right ventricular diverticulum remained clinically silent. The association of right ventricular diverticula and disseminated neonatal hemangiomatosis has not been previously documented in the literature.