A rare case of severe Ebstein's anomaly and hepatoblastoma in a neonate.

Primary liver tumours in neonates with single-ventricle palliation are exceedingly rare. We present the first reported case of neonatal hepatoblastoma with severe Ebstein's anomaly following Starnes procedure. The patient's postoperative course highlights the challenges and complications in simultaneous management of these diagnoses. Transition from shunted single-ventricle physiology to bidirectional cavopulmonary connection improved end-organ function, permitting more aggressive hepatic malignancy treatment.

Weight loss supplement causing acute heart block in a child.

A 16-year-old male was admitted to the paediatric ICU with acute onset of vomiting, somnolence, and chest pain, and electrocardiogram showing 2nd degree heart block after ingesting an Aleurites moluccana (Candlenut) seed as a herbal weight loss supplement. Electrocardiogram showed progressively worsening heart block with down-sloping of the ST segments, resembling digoxin toxicity. After 2 days of ICU observation, his symptoms began to improve and eventually resolved. The side effects of herbal supplements are often unknown but by analysing cases such as these, physicians can develop a better understanding of these substances to help guide management.

Congenital long QT syndrome in children identified by family screening.

The diagnosis of congenital long-QT syndrome (LQTS) in the relatives (nonprobands) of index patients (probands) is increasing because of screening. This report documents the clinical courses and outcomes of nonproband pediatric patients with LQTS. All patients aged <18 years with LQTS were identified at 3 pediatric centers. Demographic data, personal and family histories, electrocardiographic data, and genetic diagnoses (if available) were obtained. Probands were defined as the first patients in their families diagnosed with LQTS and nonprobands as those diagnosed by screening. Of 144 patients with LQTS, 84 (58%) were nonprobands aged 6.5+/-5.4 years with QTc intervals of 479+/-34 ms. No nonproband presented with resuscitated sudden death, atrioventricular block, or ventricular arrhythmia, but 7 (8.3%) had histories of syncope at presentation. All nonproband patients were treated. During a follow-up period of 4.7+/-3.9 years, there were no deaths in the nonproband group, but device implantation was performed in 13 (15%), 4 of whom had appropriate shocks. As expected, compared with probands, nonprobands were less symptomatic. Additionally, nonprobands were younger and had shorter QTc intervals. Although device implantation was more common in probands, there was no difference in appropriate implantable cardioverter-defibrillator shocks or mortality between the probands and their affected relatives. In conclusion, children are increasingly identified with LQTS as a result of family screening. Although phenotypic differences exist between probands and nonprobands, survival is excellent in the 2 groups with therapy. Appropriate implantable cardioverter-defibrillator discharges in the nonproband group underscore the importance of follow-up in this relatively asymptomatic population.

Long QT syndrome in children in the era of implantable defibrillators.

OBJECTIVES: We sought to assess the spectrum and outcome of young long QT syndrome (LQTS) patients, addressing treatment including device indications, risks, and benefits. BACKGROUND: Long QT syndrome has a phenotype ranging from asymptomatic electrocardiogram (ECG) abnormalities to sudden death. Treatments include beta-blockers and device implantation in high-risk individuals. Despite genetic testing, accurate risk stratification remains challenging. METHODS: A database search at 3 institutions identified all pediatric LQTS patients. Records were reviewed for demographics, criteria for diagnosis, treatment, follow-up, and ECG and device data. RESULTS: We identified 128 patients ages 8.0 +/- 5.4 years with QTc of 487 +/- 39 ms and follow-up of 4.4 +/- 3.5 years. Most were diagnosed because of an abnormal ECG in a patient with a family history (53%). Genetic mutations were identified in 51 patients. Beta-blockers were used in 126 (98%) and pacemaker/implantable cardioverter-defibrillator implantation in 27 (21%) patients, usually because of symptoms despite use of beta-blockers. Pacing was common; 22% received an appropriate shock but device-related re-intervention occurred in 48%. Device patients had longer QTc intervals (p = 0.03) and more symptoms (p < 0.001). No one with an isolated KCNQ1 and all patients with an SCN5A mutation had device implantation. During the study period, there were 2 deaths. CONCLUSIONS: Long QT syndrome without symptoms is increasingly recognized as family members are screened. Evaluation of this minimally symptomatic population offers an evolving understanding of LQTS. Previous studies of highly symptomatic patients were more worrisome. In the era of genetic testing and device implantation, overall mortality is low with treatment. Device therapy, although effective, is not without complications and should be reserved for high-risk patients.