Neonatal malignant arrhythmias refractory to conventional antiarrhythmic therapy are life-threatening and pose a major therapeutic challenge, particularly in premature infants. We report a premature infant born at 36 + 2 weeks of gestation who developed ventricular tachycardia and cardiac arrest following resuscitation for perinatal asphyxia. As lidocaine and amiodarone failed to control the rhythm, veno‑arterial ECMO was initiated shortly thereafter. After ECMO support, the rhythm converted to sinus and cardiac function improved, allowing successful weaning. Electrocardiography subsequently revealed a prolonged QT interval, which responded to oral mexiletine. Whole‑exome sequencing identified a heterozygous SCN5A c.1960G > A (p.Glu654Lys) variant inherited from the father, classified as a variant of uncertain significance (VUS). This case suggests that this specific VUS may be a candidate pathogenic variant, and that early ECMO can be an effective salvage therapy when conventional drugs fail. Additionally, when genetic testing yields a VUS, the clinical phenotype and treatment response appear valuable for assessing pathogenicity in neonatal cases.
Abstract Background Takayasu arteritis is a rare, chronic vasculitis affecting large arteries, primarily the aorta and its principal branches. The illness frequently presents with non-specific systemic symptoms, leading to a postponed diagnosis. Dysphagia resulting from aortic involvement is a rare manifestation and may resemble other cardiovascular or gastrointestinal disorders. Case presentation We report a 45-year-old Iranian woman with a one-year history of exertional dyspnoea, intermittent dysphagia for solid food, and diffuse musculoskeletal pain with morning stiffness. Initial evaluation showed elevated inflammatory markers, moderate aortic regurgitation, and aneurysmal dilation of the ascending aorta and aortic arch, and infective endocarditis was initially suspected. After serial negative blood cultures and resolution of a suspicious valvular finding on repeat transoesophageal echocardiography, upper gastrointestinal endoscopy and endoscopic ultrasonography localised her dysphagia to extrinsic oesophageal compression by the dilated thoracic aorta. Computed tomography angiography confirmed a non-dissecting aneurysm of the ascending aorta and arch, and whole-body ¹⁸F-FDG PET demonstrated markedly increased metabolic activity along the thoracic aorta and its supra-aortic branches, consistent with active large-vessel vasculitis. After systematic exclusion of infective and other inflammatory mimics, a clinical diagnosis of Takayasu arteritis was made. Conclusion This case illustrates an uncommon presentation of Takayasu arteritis with dysphagia produced by extrinsic oesophageal compression from thoracic aortic aneurysmal dilatation. Constitutional symptoms, elevated inflammatory markers, and unexplained cardiovascular findings should prompt evaluation for large-vessel vasculitis. Early multimodal imaging and a systematic approach to excluding infective and other inflammatory mimics are essential for accurate diagnosis and timely treatment.
Abstract Introduction ST-segment elevation following coronary artery bypass grafting (CABG) is a clinically important postoperative finding that may indicate perioperative myocardial infarction, early graft failure, or acute coronary compromise. However, non-ischemic postoperative processes may produce similar electrocardiographic abnormalities, creating diagnostic uncertainty, particularly in clinically stable patients. Patients with immune thrombocytopenia (ITP) undergoing cardiac surgery present an additional therapeutic challenge because of the competing risks of bleeding and thrombosis. Thrombopoietin receptor agonists (TPO-RAs), such as romiplostim, may be used to increase platelet counts before invasive procedures, but their use requires careful assessment in patients with concomitant cardiovascular disease. Case presentation A 47-year-old man with chronic ITP and multivessel coronary artery disease underwent elective CABG following preoperative administration of romiplostim for platelet optimization. Forty-eight hours after receiving romiplostim, his platelet count increased from 57 × 10⁹/L to 125 × 10⁹/L. Four-vessel CABG was performed, with a pedicled left internal mammary artery (LIMA) grafted to the left anterior descending artery (LAD) and a right internal mammary artery (RIMA) used as a Y-graft arising from the LIMA and directed sequentially to the diagonal branch, ramus intermedius, and posterior descending artery (PDA). The operation was initially attempted off-pump but was converted to cardiopulmonary bypass because of challenging coronary anatomy and inadequate target-vessel exposure. On postoperative day one, routine electrocardiography demonstrated new ST-segment elevation in leads II, III, and aVF, accompanied by PR-segment depression. A pericardial friction rub was documented. The patient remained clinically asymptomatic and hemodynamically stable. Transthoracic echocardiography showed preserved left ventricular systolic function without new regional wall motion abnormalities or pericardial effusion. High-sensitivity cardiac troponin I (hs-cTnI) was 0.9 ng/mL on postoperative day one and declined to 0.3 ng/mL on postoperative day three (laboratory reference range, 0–0.6 ng/mL). C-reactive protein and erythrocyte sedimentation rate were elevated to 120 mg/L and 68 mm/h, respectively; preoperative inflammatory marker values were unavailable.Given the absence of clinical or echocardiographic evidence of ongoing ischemia, declining cardiac biomarkers, preserved ventricular function, and the additional procedural bleeding risk associated with ITP, immediate coronary angiography was deferred. The patient was managed with close monitoring, aspirin, prophylactic low-molecular-weight heparin, indomethacin, and intravenous nicorandil during the initial assessment. The ST-segment elevation resolved by postoperative day three without clinical evidence of ongoing myocardial ischemia. Conclusion Transient ST-segment elevation after CABG requires systematic evaluation for both ischemic and non-ischemic causes. In this patient, the combination of inferior ST-segment elevation, PR-segment depression, a documented pericardial friction rub, preserved ventricular function, absence of regional wall motion abnormalities, declining hs-cTnI, elevated inflammatory markers, and spontaneous ECG resolution favored an early postoperative inflammatory process. Romiplostim should be regarded as part of the patient’s overall thrombotic-risk profile rather than as a proven direct cause of the transient ECG abnormality.
Abstract Background Anomalies of coronary artery origin are uncommon congenital variants that may pose a challenge to selective engagement at invasive coronary angiography (CAG). Although their prevalence and anatomical spectrum are well described, prospective data on the diagnostic and guide catheter utilisation to engage each anomaly subtype, procedural implications and on the access strategy adopted in current radial-first practice, remain limited. Methods We prospectively enrolled 1,850 consecutive adults undergoing invasive CAG, with or without percutaneous coronary intervention (PCI). Anomalies of origin were defined by the modified Angelini classification; intrinsic anomalies and anomalies of termination were excluded. Baseline, angiographic, procedural data - including diagnostic and guide catheter use by subtype; and access strategy were compared between anomaly and non-anomaly groups. Results An anomaly of origin was identified in 85 of 1,850 patients (4.59%; 95% CI 3.71–5.63%); under a restricted definition that excluded two anatomical variants not consistently classified as anomalies in earlier invasive-angiography series, prevalence was 1.30% (95% CI 0.83–1.92%). The commonest anomaly was ectopic (high or anterior) right coronary artery (RCA) origin from the right coronary cusp ( n = 47; 55.3%), followed by separate ostia of left anterior descending (LAD) and left circumflex (LCx) arteries ( n = 14; 16.5%) and anomalous LCx from the right coronary cusp or RCA ( n = 14; 16.5%). PCI was performed in 52 of 85 anomaly patients (61.2%). Radial access was attempted in 67 patients: 53 (79.1%) completed radially, 14 (20.9%) required crossover to femoral; and 18 were attempted femorally. Anomaly patients required more diagnostic catheters per case (2.20 vs. 1.23), with the greatest burden in crossover cases (3.14 vs. 1.17 catheters); more access crossover (16.5% vs. 5.0%) and more guide catheter changes (44.2% vs. 6.5%; all p < 0.001),. No significant difference in major procedural complications was observed (4.7% vs. 2.7%; p = 0.43); however the study was not powered for rare procedural events.
