A case report of successful complex percutaneous coronary intervention for acute coronary syndrome in a paediatric patient with familial hypercholesterolaemia.

BACKGROUND: Familial hypercholesterolaemia (FH) is a primary genetic dyslipidaemia characterized by elevation in serum low-density lipoprotein cholesterol and its deposition in systemic arteries, which causes premature atherosclerosis. CASE SUMMARY: A 10-year-old girl presented with severe symptomatic coronary artery disease. She demonstrated characteristic morphological features of FH. Despite aggressive medical management and lipid-lowering therapy, her symptoms were not relieved and she had dynamic electrocardiogram changes. Coronary angiography showed a distal left main coronary artery lesion along with significant lesions in ostio-proximal and mid-left circumflex artery which were managed by provisional left main coronary artery to left circumflex artery stenting technique, with good immediate- and short-term results and angina relief. DISCUSSION: To the best of our knowledge, this is the first reported case of a paediatric patient with FH and acute coronary syndrome treated with percutaneous coronary intervention to left main coronary artery and left circumflex artery using provisional stenting technique. Revascularization strategies for symptomatic coronary artery disease in paediatric patients with FH have multiple unique challenges and remain an unexplored and under-reported subject.

Arrhythmia spectrum and outcome in children with myocarditis.

INTRODUCTION: Myocarditis remains an under-diagnosed entity among children. We evaluated the spectrum of electrocardiogram (ECG) changes and arrhythmias in children with myocarditis. METHODS: A single-center prospective observational study was conducted over a period of 18 months at a public university hospital, which included all cases with myocarditis from the ages of 1 month to 12 years. Myocarditis was diagnosed according to standard criteria. Arrhythmias were detected by 12-lead ECG or by multiparameter monitors. RESULTS: There were 63 children with myocarditis. Sinus tachycardia remained the most important ECG finding (61, 96.8%) followed by ST-T changes (30, 47.6%), low voltage QRS complexes (23, 36.5%), and premature complexes (11, 17.4%). Sustained arrhythmias were seen in 14/63 (22.2%) of the children (Group A), while the remaining 49 patients were designated as Group B. There were 11 (17.5%) cases with sustained tachyarrhythmias, comprising 5 with supraventricular tachycardia, 4 with ventricular tachycardia, and 2 with atrial flutter/fibrillation. Bradyarrhythmias were seen in 3 patients, including 2 children with atrioventricular block and 1 with severe sinus bradycardia. A longer hospital stay of 18.5 (4.75) days vs. 13 (4) days, P = 0.001), and more ST-T changes [12 (85.7%) vs. 18 (36.73%), P = 0.003] were seen in Group A. Multivariate regression analysis found only the presence of ST-T changes as predictors for arrhythmia. CONCLUSIONS: A variety of arrhythmias and other ECG changes were commonly seen in children with myocarditis. Sustained arrhythmias were seen in one-fifth of the patients, being associated with ST-T changes and a longer hospital stay.

Phosphaturic Mesenchymal Tumors from Head to Toe: Imaging Findings and Role of the Radiologist in Diagnosing Tumor-Induced Osteomalacia.

BACKGROUND: This study aimed at evaluating the imaging findings of phosphaturic mesenchymal tumors and tumor-induced osteomalacia and assess the clinical and biochemical profiles of patients with tumor-induced osteomalacia. MATERIALS AND METHODS: Imaging findings in six patients with tumor-induced osteomalacia and histopathologically proven phosphaturic mesenchymal tumors were evaluated. Clinical and biochemical profiles of these patients were also assessed. RESULTS: Along with having a characteristic biochemical profile, patients with phosphaturic mesenchymal tumors also have certain imaging findings which can aid in the diagnosis such as increased uptake on DOTA PET-CT and homogeneous post-contrast enhancement on CT and MRI. CONCLUSION: Patients with tumor-induced osteomalacia have characteristic symptoms, imaging and biochemical profiles. For radiologists, raising the suspicion of a phosphaturic mesenchymal tumor in patients with refractory hypophosphatemic osteomalacia as well as localizing the tumor on imaging is crucial, as complete excision of the tumor leads to resolution of the osteomalacia and the patient's clinical symptoms.