Rheumatic Heart Disease Treatment Cascade in Uganda.

BACKGROUND: Rheumatic heart disease (RHD) is a leading cause of premature death and disability in low-income countries; however, few receive optimal benzathine penicillin G (BPG) therapy to prevent disease progression. We aimed to comprehensively describe the treatment cascade for RHD in Uganda to identify appropriate targets for intervention. METHODS AND RESULTS: Using data from the Uganda RHD Registry (n=1504), we identified the proportion of patients in the following care categories: (1) diagnosed and alive as of June 1, 2016; (2) retained in care; (3) appropriately prescribed BPG; and (4) optimally adherent to BPG (>80% of prescribed doses). We used logistic regression to investigate factors associated with retention and optimal adherence. Overall, median (interquartile range) age was 23 (15-38) years, 69% were women, and 82% had clinical RHD. Median follow-up time was 2.4 (0.9-4.0) years. Retention in care was the most significant barrier to achieving optimal BPG adherence with only 56.9% (95% confidence interval, 54.1%-59.7%) of living subjects having attended clinic in the prior 56 weeks. Among those retained in care, however, we observed high rates of BPG prescription (91.6%; 95% confidence interval, 89.1%-93.5%) and optimal adherence (91.4%; 95% confidence interval, 88.7-93.5). Younger age, latent disease status, and access to care at a regional center were the strongest independent predictors of retention and optimal adherence. CONCLUSIONS: Our study suggests that improving retention in care-possibly by decentralizing RHD services-would have the greatest impact on uptake of antibiotic prophylaxis among patients with RHD in Uganda.

A case of anomalous origin of the left coronary artery presenting with acute myocardial infarction and cardiovascular collapse.

BACKGROUND: Though a rare clinical entity, anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) a common cause of myocardial infarction in children. Unrecognized and untreated it leads to progressive left ventricular dilatation and systolic dysfunction. In settings of high infectious burden, ALCAPA may erroneously be diagnosed as myocarditis, dilated cardiomyopathy or other common childhood disorders. CLINICAL CASE: We present the case of a 10 weeks old male infant who presented to the inpatient unit with marked restlessness and irritability. He was inconsolable, had marked respiratory distress, cool extremities, central and peripheral cyanosis oxygen. The radial and brachial pulses were absent. The mean arterial pressure was 65mmHg, Heart rate of 160 beats per minute with a third heart sound. The liver was enlarged 4cm below the costal margin and tender, with a splenomegaly. He had an elevated Creatinine Kinase-MB of 112.5 u/L. ECG revealed deep Q waves in leads I, aVL, V5, V6 with ST elevation in the anterolateral leads. Echo showed a dilated left ventricle LVEDd of 40mm, with paradoxical interventricular septal motion, severe LV systolic dysfunction (FS=15%, EF=28%), LV anterolateral wall echo brightness and flow reversal in the Left coronary artery with its origin from the pulmonary trunk. He was admitted to the coronary care unit as a case of acute myocardial infarction with cardiovascular collapse. He received fluid resuscitation, inotropic support and standard management of heart failure. Six days later he was discharged home with a plan to refer abroad. He died at home after one week. CONCLUSION: A combination of a high index of suspicion, typical ECG and echocardiographic findings in a young infant presenting with LV dysfunction could lead to an earlier diagnosis of ALCAPA.

Outcome of patients undergoing open heart surgery at the Uganda heart institute, Mulago hospital complex.

BACKGROUND: Heart disease is a disabling condition and necessary surgical intervention is often lacking in many developing countries. Training of the superspecialties abroad is largely limited to observation with little or no opportunity for hands on experience. An approach in which open heart surgeries are conducted locally by visiting teams enabling skills transfer to the local team and helps build to build capacity has been adopted at the Uganda Heart Institute (UHI). OBJECTIVES: We reviewed the progress of open heart surgery at the UHI and evaluated the postoperative outcomes and challenges faced in conducting open heart surgery in a developing country. METHODS: Medical records of patients undergoing open heart surgery at the UHI from October 2007 to June 2012 were reviewed. RESULTS: A total of 124 patients underwent open heart surgery during the study period. The commonest conditions were: venticular septal defects (VSDs) 34.7% (43/124), Atrial septal defects (ASDs) 34.7% (43/124) and tetralogy of fallot (TOF) in 10.5% (13/124). Non governmental organizations (NGOs) funded 96.8% (120/124) of the operations, and in only 4 patients (3.2%) families paid for the surgeries. There was increasing complexity in cases operated upon from predominantly ASDs and VSDs at the beginning to more complex cases like TOFs and TAPVR. The local team independently operated 19 patients (15.3%). Postoperative morbidity was low with arrhythmias, left ventricular dysfunction and re-operations being the commonest seen. Post operative sepsis occurred in only 2 cases (1.6%). The overall mortality rate was 3.2. CONCLUSION: Open heart surgery though expensive is feasible in a developing country. With increased direct funding from governments and local charities to support open heart surgeries, more cardiac patients access surgical treatment locally.