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The fourth universal definition of MI defines requires presence of j point elevation in two contiguous leads except v2–3 where the elevation should be equal to or >2 mm in men (2.5 mm in <40 years) and 1.5 mm in women.(1) We present two cases of patients who presented with electrocardiographic manifestations of occlusion of septal perforator of left anterior descending artery and discuss the salient feature of ECG in such patients. We also present the limitations of STEMI criteria given the dynamic nature of acute coronary occlusion and stress on early recognition of this MI.

The cardiac causes predominantly include rheumatic heart disease, mitral valve prolapse and congenital heart disease. Since the patient presented in junctional rhythm, our main differential included either transient causes (new onset atrial fibrillation, transient electrolyte abnormalities (hyperkalaemia), digoxin toxicity, acute myocarditis with atrial involvement, dilated cardiomyopathy) or permanent causes (associated with muscular dystrophies and genetic cardiomyopathies). Atrial standstill is a rare condition with absence of atrial mechanical/electrical activity characterized by “(1) the absence of P waves in the ECG and of A waves in the intracardiac recordings, (2) narrow QRS complexes, (3) evidence of atrial paralysis (absence of A waves in the jugular venous pulse, in the atrial pressure recording and in the mitral Doppler recording), and (4) inability to stimulate the atria.” Germline pathogenic variations in the HCN4 gene have been shown to be associated with familial sick sinus syndrome, specifically the autosomal dominant type 2 variety.5 The mechanism by which sick sinus syndrome due to gene mutations result in atrial standstill have been best described for SCN5A mutation which is the most common genetic cause in paediatric patients with atrial standstill.1 In patients with SCN5A mutations, slowing down of both pacemaker function and conduction of action potential across the SA node-atrium occurs.6 Histological examination of the atrial wall have been described to show presence of dense collagenous tissue, different stages of fibrosis with scattered myocardium.7 A combination of reducing pacemaking activity and atrial fibrosis contributes to the atrial standstill.

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.