Parents' perceptions of genetics services for congenital heart disease: the role of demographic, clinical, and psychological factors in determining service attendance.

Purpose: We sought to identify the demographic, clinical, and psychological factors associated with parents' attendance at clinical genetics services for congenital heart disease.

Methods: A survey assessing access to cardiac genetics services and a range of other variables was sent to the families of 213 children diagnosed with congenital heart disease between the years 2000 and 2009 at the Sydney Children's Hospital, Australia.

Results: Of the 114 respondents, 22% had accessed cardiac genetics services.

Prophylactic left thoracic sympathectomy to prevent electrical storms in CPVT patients needing ICD placement.

Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT), a life threatening arrhythmia induced by sympathetic stimulation in susceptible individuals is often refractory to antiarrhythmic agents. First line of treatment, beta-blockers can be ineffective in up to 50% with implantable cardioverter-defibrillator (ICD) placement for refractory cases. Paradoxically ICD can be arryhthmogenic from shock-associated sympathetic stimulation, initiating more shocks and "electrical storms".

An Australian tuberous sclerosis cohort: are surveillance guidelines being met?

Aim: This study aims to describe the phenotypic and genotypic characteristics of 45 Australian patients with tuberous sclerosis complex (TSC), to assess risk factors for intellectual disability, to compare patients with TSC1 and TSC2 mutations and to assess adherence to surveillance recommendations.

Methods: Phenotypic features were recorded in 45 patients who fulfilled established criteria for a diagnosis of definite TSC. All patients underwent TSC1 and TSC2 sequencing and multiplex ligand probe amplification.

Impact of early and standardized treatment with amiodarone on therapeutic success and outcome in pediatric patients with postoperative tachyarrhythmia.

Objective: Effects of timing of inception of amiodarone antiarrhythmic treatment after corrective surgery for congenital heart defects are not yet well defined.

Methods: In a 10-year prospective observational study, 71 of 2651 patients (2885 procedures, 2106 bypass procedures) received amiodarone for newly detected postoperative atrial (n = 70) or ventricular (n = 7) tachyarrhythmia: early treatment (initiation within 60 minutes of arrhythmia detection) and late treatment (after that). From an interim analysis, a treatment protocol was established (32 patients).

Acute hemodynamic effects of intravenous amiodarone treatment in paediatric cardiac surgical patients.

Introduction: The haemodynamic response after an IV-loading dose of amiodarone followed by continuous infusion for various supraventricular and ventricular tachycardias was studied in children immediately after corrective surgery for congenital heart defects.

Methods: Observational study over a 10-year period. Seventy-one out of 2651 patients (2,885 procedures, 2,106 bypass procedures) received amiodarone therapy for newly detected postoperative tachyarrhythmias.

Successful use of surfactant to treat severe intrapulmonary hemorrhage after iatrogenic lung injury--A case report.

Objective: To describe the beneficial clinical effect of the use of surfactant in a patient with severe unilateral pulmonary hemorrhage after iatrogenic lung injury during corrective surgery for congenital heart defects.

Design: Case report.

Setting: Pediatric cardiac intensive care unit of a tertiary care hospital.

Clinical review: thyroid hormone replacement in children after cardiac surgery--is it worth a try?

Cardiac surgery using cardiopulmonary bypass produces a generalized systemic inflammatory response, resulting in increased postoperative morbidity and mortality. Under these circumstances, a typical pattern of thyroid abnormalities is seen in the absence of primary disease, defined as sick euthyroid syndrome (SES). The presence of postoperative SES mainly in small children and neonates exposed to long bypass times and the pharmacological profile of thyroid hormones and their effects on the cardiovascular physiology make supplementation therapy an attractive treatment option to improve postoperative morbidity and mortality.

Successful treatment of oleander intoxication (cardiac glycosides) with digoxin-specific Fab antibody fragments in a 7-year-old child: case report and review of literature.

Unlabelled: A 7-year-old girl presented six hours after ingestion of a yellow oleander seed (Thevetia peruviana) with severe emesis, change in colour vision and complete heart block. Initial treatment with phenytoin and isoprenalin infusion led to intermittent supraventricular and ventricular tachycardia. The patient was then treated with two intravenous doses of 190 mg of digoxin-specific Fab antibody fragments (Digibind).

Beneficial hemodynamic response of transthoracic cardiac pacing in a 2 kg preterm neonate.

Objective: To describe the beneficial hemodynamic effect of transthoracic external pacing compared with epicardial pacing in a 2 kg premature neonate with congenital complete heart block, hydrops and complex congenital heart disease.

Methods: Transthoracic epicardial pacing was instituted at a rate of 120 beats/min to treat life-threatening bradycardia (HR 50-60 beats/min), hypotension (BP 45/20 mmHg) and severe lactic acidosis (pH 7.18, lactate 7.