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Background QRS axis deviation can occur during myocardial infarction (MI); to date, little is known about the significance of extreme right axis deviation (ERAD) in the frontal plane, i.e. a shift in QRS axis between +180° and +270°, during MI. We sought to investigate the clinical characteristics and outcomes of patients with new-onset ERAD in the absence of complete bundle branch blocks (BBB) in the setting of acute coronary syndromes (ACS). Methods A single-center retrospective observational study was conducted, including patients with new-onset ERAD in the absence of complete BBB admitted for ACS to our Cardiac Intensive Care Unit. Clinical, electrocardiographic, echocardiographic, angiographic features at baseline and cardiovascular events during hospitalization and at mid-term follow-up were collected. Results The study population consisted of 30 consecutive patients (23 men) from January 2014 to September 2018. The most frequent clinical presentation was ST-segment elevation MI (n = 22, 73.4%) and the most frequent electrocardiographic MI location was anterolateral (n = 11, 36.7%). Left anterior descending (LAD) was the most frequent infarct-related artery (n = 21, 70%); 15 patients (50%) had multivessel coronary artery disease. Cardiac arrest due to ventricular fibrillation (VF) at presentation (n = 5, 16.6%), cardiogenic shock during the hospital stay (n = 10, 33.3%), cardiac arrest due to VF after revascularization (n = 6, 20%) and cardiac death (n = 7, 23.3%) were common. Conclusion New-onset ERAD during MI may be related to extensive myocardial ischemia and/or necrosis causing an “electrical escaping” with an extreme dislocation of the QRS axis. In our limited series we found several acute arrhythmic and hemodynamic complications and high mortality.

Patient: Male, 72 Final Diagnosis: Acute myocardial infarction Symptoms: — Medication: Cardiac catheterization Clinical Procedure: — Specialty: Cardiology Objective: Unusual clinical course Background: New-onset extreme right axis deviation and right bundle branch block (RBBB) are rare during acute myocardial infarction (AMI), and has only been reported in several cases reflecting the severity of AMI. It could predict severe clinical complications and higher risks in coronary artery disease. Although there is little electrophysiological explanation, the complications are severe. They should be emphasized in newly diagnosed extreme right axis deviation and RBBB in AMI. Case Report: A 72-year-old male was admitted to our department with a chief complaint of intermittent retrosternal chest pain and was diagnosed with extensive anterior myocardial infarction with RBBB, by elevated myocardial enzymes and ECG. The main wave direction of QRS in lead aVR was positive and showed an extreme right axis deviation. After a month, the patient’s chest distress and the RBBB vanished, but a right axis deviation still existed. The echocardiogram showed prior extensive anterior myocardial infarction (including apex myocardia) and lower LVEF. Conclusions: New diagnosed RBBB and right axis deviation is uncommon and could be a useful clue to evaluate myocardial ischemia in AMI cases. This electrocardiographic marker can identify coronary artery occlusion where ST-segments are hard to evaluate, and hence, patients may benefit most from early and complete revascularization strategies such as primary angioplasty.

Background Costello syndrome (CS) and Cardiofaciocutaneous syndrome (CFCS) are both RASopathies, a group of developmental disorders caused by a mutation in genes encoding in the RAS/MAPK pathway. RASopathies are prone to cardiac malformations and sudden cardiac death. Description of electrocardiographic findings in these patients is rare. The objective of this study was to describe electrocardiogram and correlation with echocardiographic findings in a cohort of CS and CFC. Method A 12-lead electrocardiogram (ECG) and a trans-thoracic echocardiography (TTE) were performed for all patients during the same period in July 2016, and were analyzed by two different cardiologists. Results Twenty-four patients were included, 14 with CFCS and 10 with CS. The mean age was 10.3 ± 7.0 years. In 14 patients (58%), at least one abnormality was noticed on the electrocardiogram: axis deviation in 11 patients (46%); atrial hypertrophy in 5 patients (21%); left ventricular hypertrophy in 2 patients (8%); incomplete bundle-branch block in 3 patients (13%) and a significant Q-wave in 5 patients (21%). All patients with left axis deviation and/or left ventricular hypertrophy at ECG had a hypertrophic cardiomyopathy (sub-aortic septal, apical or concentric hypertrophy) on TTE. The patients with a significant Q wave (among them 2 had an extreme right axis deviation) had in fact a minimal hypertrophic cardiomyopathy (septal thickness Conclusion Significant Q-wave or extreme right axis deviation on the ECG may suggest the potential presence of myocardial fibrosis. Detection of fibrosis by MRI may play an important role for the risk stratification of sudden cardiac death in these patients.

A case report of a patient with persistent left anterior hemiblock admitted with acute ischemic heart disease is described. At effort during follow-up evaluation, the patient complained of retrosternal pain when the heart rate was 124 beats/min. No pathological ST-T changes were demonstrated at this time. At 133 beats/min, the precordial pain increased, the QRS axis displayed a marked shift to the right, and ischemic ST-T depression was recorded. In discussing this unreported phenomenon, it is pointed out that left anterior hemiblock does not necessarily represent an anatomical block of the atrioventricular bundle but may simply reflect a relative delay in conduction. In addition, acute ischemia may change the physiological behavior of the system resulting in slower conduction through the posterior rather than through the anterior atrioventricular bundle. The influence of left anterior and left posterior hemiblock on ischemic ST-T changes and on the coronary flow distribution is discussed.

Dysplastic pulmonary valve is usually associated with extreme right axis deviation. A mean manifest electrical QRS axis of + 160 degrees or less was present in 11 of 30 cases of DPV studied by us. In 9 of these cases associated left ventricular hypertrophic non-obstructive cardiomyopathy was found. We suggest that a normal mean manifest electrical QRS axis in the presence of dysplastic stenotic pulmonary valve indicates the presence of associated cardiomyopathy.