Mora, Bassem

Abstract Background Children with congenital heart disease remain at risk of being lost to follow-up. They may present at a later stage with complications related to underlying condition that usually require in-depth phenotyping for optimal management. We describe an unusual case of a double outlet right ventricle (DORV) who presented three decades after initial intervention. Case summary A 32-year-old female sought attention for worsening breathlessness. Available data suggested that she had double outlet right ventricle (DORV) and underwent pulmonary artery (PA) banding in infancy. On examination, she was frail, clubbed and cyanosed with oxygen saturation reduced to 75% at rest. Blood tests demonstrated elevated haemoglobin (208 g/L) and thrombocytopenia (70 *109/L). Echocardiography was limited but demonstrated DORV with increased gradient across both outflow tracts. Cardiac magnetic resonance (CMR) imaging clearly demonstrated DORV with doubly committed interventricular communication, subvalvular aortic valve stenosis and supravalvular pulmonary stenosis due to a previous PA band. CMR was also remarkable for demonstrating severely impaired left ventricle and difficulty achieving optimal contrast between myocardium and blood pool on late enhancement images. She underwent relief of subaortic stenosis with immediate improvement in left ventricular (LV) function and PA plasty with application of a new PA band. Conclusion Lost to follow-up congenital patients present a particular challenge due to complications of chronic unusual physiology. This requires careful assessment by a multidisciplinary team to define optimal management strategy.

The Middle East and North Africa (MENA) region has a high burden of morbidity and mortality due to premature (≤55 years in men; ≤65 years in women) myocardial infarction (MI) and acute coronary syndrome (ACS). Despite this, the prevalence of risk factors in patients presenting with premature MI or ACS is incompletely described. We compared lifestyle, clinical risk factors, and biomarkers associated with premature MI/ACS in the MENA region with selected non-MENA high-income countries. We identified English-language, peer-reviewed publications through PubMed (up to March 2018). We used the World Bank classification system to categorize countries. Patients with premature MI/ACS in the MENA region had a higher prevalence of smoking than older patients with MI/ACS but a lower prevalence of diabetes, hypertension, and dyslipidemia. Men with premature MI/ACS had a higher prevalence of smoking than women but a lower prevalence of diabetes and hypertension. The MENA region had sparse data on lifestyle, diet, psychological stress, and physical activity. To address these knowledge gaps, we initiated the ongoing Gulf Population Risks and Epidemiology of Vascular Events and Treatment (Gulf PREVENT) case–control study to improve primary and secondary prevention of premature MI in the United Arab Emirates, a high-income country in the MENA region.