Aims  Sudden death and aborted sudden death have been observed in patients with biallelic variants in TECRL. However, phenotypes have only begun to be described and no data are available on medical therapy after long-term follow-up. Methods and results  An international, multi-centre retrospective review was conducted. We report new cases associated with TECRL variants and long-term follow-up from previously published cases. We present 10 cases and 37 asymptomatic heterozygous carriers. Median age at onset of cardiac symptoms was 8 years (range 1–22 years) and cases were followed for an average of 10.3 years (standard deviation 8.3), right censored by death in three cases. All patients on metoprolol, bisoprolol, or atenolol were transitioned to nadolol or propranolol due to failure of therapy. Phenotypes typical of both long QT syndrome and catecholaminergic polymorphic ventricular tachycardia (CPVT) were observed. We also observed divergent phenotypes in some cases despite identical homozygous variants. None of 37 heterozygous family members had a cardiac phenotype. Conclusion  Patients with biallelic pathogenic TECRL variants present with variable cardiac arrhythmia phenotypes, including those typical of long QT syndrome and CPVT. Nadolol and propranolol may be superior beta-blockers in this setting. No cardiac disease or sudden death was present in patients with a heterozygous genotype.

Background: Delayed hospital presentation is a hindrance to the optimum clinical outcome of modern therapies of Myocardial infarction (MI). This study aimed to investigate the significant factors associated with prolonged prehospital delay and the impact of this delay on in-hospital mortality among patients with MI in Northern Bangladesh. Methods: This cross sectional study was conducted in December 2019 in cardiology ward of a 1000-bed tertiary care hospital of Bangladesh. Patients admitted in the ward with the diagnosis of myocardial infarction were included in the study. Socio demographic data, clinical features and patients' health seeking behavior was collected in a structured questionnaire from the patients. Median with interquartile range (IQR) of pre hospital delay were calculated and compared between different groups. Chi-square (χ 2) test and binary logistic regression were used to estimate the determinants of pre-hospital delay and effect of pre-hospital delay on in-hospital mortality. Results: Three hundred thirty-seven patients was enrolled in the study and their median (IQR) pre-hospital delay was 9.0 (13.0) hours. 39.5% patients admitted in the specialized hospital within 6 h. In logistic regression, determinants of pre-hospital delay were patients age (for < 40 years aOR 2.43, 95% CI 0.73-8.12; for 40 to 60 years aOR 0.44, 95% CI 0.21-0.93), family income (for lower income aOR 5.74, 95% CI 0.89-37.06; for middle income aOR 14.22, 95% CI 2.15-94.17), distance from primary care center ≤5 km (aOR 0.42, 95% CI 0.12-0.90), predominant chest pain (aOR 0.15, 95% CI 0.05-0.48), considering symptoms as non-significant (aOR 17.81, 95% CI 5.92-53.48), referral from primary care center (for government hospital aOR 4.45, 95% CI 2.03-9.74; for private hospital OR 98.67, 95% CI 11.87-820.34); and not having family history of MI (aOR 2.65, 95% CI 1.24-5.71) (R2 = 0.528). Risk of in-hospital mortality was almost four times higher who admitted after 6 h compared to their counterpart (aOR 0.28, 95% CI 0.12-0.66); (R 2 = 0.303). Conclusion: Some modifiable factors contribute to higher pre-hospital delay of MI patients, resulting in increased in-hospital mortality. Patients' awareness about cardiovascular diseases and improved referral pathway of the existing health care system may reduce this unexpected delay.

Background: Acute inferior myocardial Infarction frequently involves the right ventricle and associated with significant morbidity and mortality. We hypothesized that right ventricular involvement may affect the prognosis of patients with inferior myocardial infarctions.Methodology: In 100 consecutive patients admitted to the hospital with acute inferior myocardial infarction, we assessed the incidence and prognostic factors for in-hospital outcome. RVMI (Right ventricular myocardial infarction) was diagnosed by ≥1mm ST elevation in lead V4R in right sided electrocardiogram.Result: RVMI was found in 31(31%) of patients of acute inferior myocardial infarctions. Major complications as hypotension and cardiogenic shock occurred in 96.7% and 64.5% patients respectively and in-hospital mortality was 41.9%. Whereas major complications as hypotension and cardiogenic shock occurred in 10.1% and 2.8% patients respectively and in hospital mortality was 2.8% of patients without right ventricular infarction among the inferior myocardial infarction. Multiple logistic regression analysis showed right ventricular infarction to be independent of and superior to all other clinical variables available on admission for the prediction of in hospital mortality (relative risk 88.37 percent, 95% confidence interval 7.33 to 1064.80; p=0.000) and major complications as hypotension (relative risk 394.22, 95% confidence interval 32.04 to 4849.07; p=0.000) and cardiogenic shock (relative risk 272.36, 95% confidence interval, 16.38 to 4526.35; p=0.000).Conclusion: RVMI commonly occurs in inferior myocardial infarction. It is a strong and independent predictor of major complications and in-hospital mortality. Early detection and appropriate monitoring can reduce its high mortality rate.TAJ 2013; 26: 8-13