IntroductionSideroblastic cardiomyopathy secondary to repeated blood transfusions is a feared complication in thalassaemia. Control of myocardial iron is thus becoming the cornerstone of thalassaemia management. Recent evidence suggests a role for L-type Ca2+ channels in mediating iron uptake by the heart. Blocking the cellular iron uptake through these channels may add to the benefit of therapy to standard chelation in reducing myocardial iron. We aim to determine the efficacy of amlodipine (a calcium channel blocker) as an adjunct to standard aggressive chelation in retarding myocardial iron deposition in thalassaemics with or without cardiomyopathy.OutcomesThe primary outcome is to compare the efficacy of amlodipine+chelation (intervention) versus standard chelation (control) in retarding myocardial iron deposition. Secondary outcomes include the effect of amlodipine therapy on systolic and diastolic function, strain and strain rate and liver iron content.Methods and analysisThis is a single-centre, parallel-group, prospective randomised control trial. Twenty patients will be randomised in a 1:1 allocation ratio into the intervention and control arms. In addition to conventional echocardiography, MRI T2* values for assessment of cardiac and liver iron load will be obtained at baseline and at 6 and 12 months. Cardiac T2* will be reported as the geometric mean and per cent coefficient of variation, and an increase in cardiac T2* values from baseline will be used as an end point to compare the efficacy of therapy. A p Value of <0.05 will be considered significant.Study settingDepartment of Pediatric and Child Health, Aga Khan University Hospital, Karachi, Pakistan.Ethics and disseminationThis study has been approved by the Ethics Review Committee and Clinical Trials Unit at The Aga Khan University with respect to scientific content and compliance with applicable research and human subjects regulations. Findings will be reported through scientific publications and research conferences and project summary papers for participants.Trial registration numberClinicalTrials.Gov. Registration no: NCT02065492.

There is a high prevalence of severe myocardial iron load in Pakistani TM patients. Serum ferritin did not correlate with T2* values. Lower T2* was the only clinical factor associated with the NYHA functional class.

Aims: To identify the changes in cardiovascular disease presentation, emergency room triage and inpatient diagnostic and therapeutic pathways. Methods: We conducted a retrospective cohort study at the Aga Khan University Hospital, Karachi. We collected data for patients presenting to the emergency department with cardiovascular symptoms between March–July 2019 (pre-COVID period) and March–July 2020 (COVID period). The comparison was made to quantify the differences in demographics, clinical characteristics, admission, diagnostic and therapeutic procedures, and in-hospital mortality between the two periods. Results: Of 2976 patients presenting with cardiac complaints to the emergency department (ED), 2041(69%) patients presented during the pre-COVID period, and 935 (31%) patients presented during the COVID period. There was significant reduction in acute coronary syndrome (ACS) (8% [95% CI 4–11], p < 0.001) and heart failure (↓6% [95% CI 3–8], p < 0.001). A striking surge was noted in Type II Myocardial injury (↑18% [95% CI 20–15], p < 0.001) during the pandemic. There was reduction in cardiovascular admissions (coronary care unit p < 0.01, coronary step-down unit p = 0.03), cardiovascular imaging (p < 0.001), and procedures (percutaneous coronary intervention p = 0.04 and coronary angiography p = 0.02). No significant difference was noted in mortality (4.7% vs. 3.7%). The percentage of patients presenting from rural areas declined significantly during the COVID period (18% vs. 14%, p = 0.01). In the subgroup analysis of sex, we noticed a falling trend of intervention performed in females during the COVID period (8.2% male vs. 3.3 % female). Conclusions: This study shows a significant decline in patients presenting with Type I myocardial infarction (MI) and a decrease in cardiovascular imaging and procedures during the COVID period. There was a significant increase noted in Type II MI.

Objectives: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart-muscle disease, characterized by fibro-fatty replacement and ventricular arrhythmias, that primarily affects the right ventricle (RV). We aimed to look at the clinical presentation, cardiac magnetic resonance (CMR) imaging findings and prognosis of patients with ARVC in Pakistan. Material and Methods: It is a retrospective observational study, 17 consecutive patients with CMR and other findings consistent with ARVC, were enrolled from 2010 to 2019 at a single center. Results: Out of 17 patients, 12 (70.6%) were male with a mean age of 33.5 ± 17.5 years. Family history of sudden cardiac death was present in 3 (17.7%) patients while one (5.9%) patient had family history of ARVC. Syncope was the first presenting symptom in eight (47.1%) patients. On 12 leads ECG, T wave inversion in precordial leads was found in 6 (35.4%) patients, and epsilon wave was present in only 3 (17.7%) patients. On echocardiogram, 13 (76.5%) patients had dilated RV with reduced systolic function. On CMR, majority of patients (n = 14, 82.4%) were found to have RV dilatation with regional dyskinesia and fatty infiltration, 9 (52.9%) of them had left ventricular involvement also. Follow-up was available for 14 patients (82.4%) with a mean follow-up period of 35.5 ± 19.7 months. Three (21.4%) of them died and 10 (71.4%) got admissions for heart failure during follow-up period. Conclusion: Arrhythmia related events are the main presenting symptoms of ARVC in this region, and left ventricular involvement in ARVC is not rare in this population. The mortality is relatively high, probably due to advanced disease at the time of presentation and less medical facilities available.

A 48 years old male presented to clinic with 12 months of low grade fever with shortness of breath which has progressively worsened with no associated weight loss, night sweats or loss of appetite. There was no prior history of chronic illness before the current illness. Laboratory workup revealed a high white blood cell count with predominant eosinophils. Chest X-ray was normal. Transthoracic echocardiography and Cardiac Magnetic Resonance showed biventricular thrombi. On further extensive workup the findings were consistent with hypereosinophilic syndrome. The patient was started on oral steroids, hydroxyurea, imatanib mesylate and oral anticoagulation. The patient responded to the treatment with complete resolution of his symptoms over the course of few months. The repeat Echocardiogram after a year showed normal left ventricular systolic and diastolic function with complete resolution of biventricular thrombi.