Trends in Annual Incidence Rates of Newly Diagnosed Endomyocardial Fibrosis Cases at the Uganda Heart Institute: A 14-Year Review.

Background: First described in Uganda over seven decades ago, Endomyocardial fibrosis (EMF) is a rare form of restrictive cardiomyopathy found in the tropics. EMF occurs mainly in two phenotypes; biventricular involvement and right ventricular (RV) form. Previously endemic in several countries, there are reports suggesting that the disease is on the decline. Objectives: To describe trends in annual incidence rates of newly diagnosed EMF cases at the Uganda Heart Institute (UHI). Methods: This was a retrospective chart review of all newly diagnosed EMF cases at UHI from January 2007 to December 2020. Cases were divided into two groups A (2007-2013) and B (2014-2020). Results: A total of 155 cases were diagnosed during the period (Group A, n = 124; Group B, n = 31). There were no significant differences between the two groups A and B regarding median age at diagnosis (14 vs. 12 years, p = 0.0940), gender (48.4% female vs. 35.5%, p = 0.1987), and EMF type (66.9% RV EMF vs. 71.0%, p = 0.6634), respectively. The presence of complications such as intracardiac thrombus (5.6 vs. 32.2%, p = 0.0002) and pericardial effusion (57.3% vs. 80.6, p = 0.0172) were more frequent in group B than A, respectively. Pulmonary hypertension (PHT) was predominantly seen in cases with biventricular EMF compared to those with RV EMF (26 vs. 3.8%, p = 0.0001). The number of new cases diagnosed per year remained largely stable in the period 2007-2011, ranging 14-21 per year, peaked in 2012 (26 new cases), and thereafter declined from 10 cases seen in 2013 to 1-5 cases seen per year in the period 2017-2020. Similarly, the annual incidence rates of new EMF diagnosis remained relatively stable in the period 2007-2012, ranging between 22.7 and 29.7 per 10,000 patients seen in the echo labs, and then dramatically declined after 2012 to range between 1.0 and 4.5 new cases per 10,000 patients in the period between 2017 and 2020. Conclusion: There has been a steady decline in the number of new cases of EMF seen at the UHI. However, there were no significant differences in the gender, age at diagnosis and EMF subtype of cases during the period under review. Complication rates were more frequent in the later cohort.

Echocardiography in Indigenous Populations and Resource Poor Settings.

The majority of global cardiovascular disease burden occurs in low- and middle-income countries (LMIC) and indigenous populations. Although common diseases, such as ischaemic heart disease, cause significant burden, there are also neglected diseases. Forgotten by many, these diseases-including rheumatic heart disease, endomyocardial fibrosis and Chagas cardiomyopathy-continue to take a tremendous toll on a large proportion of the world's population. Whilst the technology of echocardiography continues to evolve in many high-income countries, low resource countries are working out how to make this vital tool available and affordable for the most remote and poorest populations. This paper aims to highlight the neglected cardiovascular diseases and their echocardiographic features. It also highlights the latest research in relation to portable echocardiography, task shifting and disease screening. The authors make recommendations in relation to future directions, including making echocardiography an affordable and accessible tool for all.

Pediatric cardiovascular care in Uganda: Current status, challenges, and opportunities for the future.

In many developing countries, concerted action against common childhood infectious diseases has resulted in remarkable reduction in infant and under-five mortality. As a result, pediatric cardiovascular diseases are emerging as a major contributor to childhood morbidity and mortality. Pediatric cardiac surgery and cardiac catheterization interventions are available in only a few of Sub-Saharan African countries. In Uganda, open heart surgeries (OHSs) and interventional procedures for pediatric cardiovascular disease are only possible at the Uganda Heart Institute (UHI), having been started with the help of expatriate teams from the years 2007 and 2012, respectively. Thereafter, independent OHS and cardiac catheterization have been possible by the local team at the UHI since the year 2009 and 2013, respectively. The number of OHSs independently performed by the UHI team has progressively increased from 10 in 2010 to 35 in 2015, with mortality rates ranging from 0% to 4.1% over the years. The UHI pediatric catheterization team has independently performed an increasing number of procedures each year from 3 in 2013 to 55 in 2015. We herein describe the evolution and current status of pediatric cardiovascular care in Uganda, highlighting the unique aspects of its establishment, existing constraints, and future plans.

Resolution of dilated cardiomyopathy in an adolescent with change of a failing highly active antiretroviral drug therapy.

BACKGROUND: Cardiovascular dysfunction is a recognized complication of HIV infection in children. Cardiac complications of HIV usually occur late in the course of the disease; they may be associated with drug therapy, and hence become more common as therapy and survival improve. Left ventricular (LV) dysfunction at baseline is a risk factor for death independent of the CD4 cell count, HIV viral load, and neurological disease. CLINICAL CASE: We present the case of a 15 year old girl with HIV who developed left ventricular dysfunction while non-compliant on highly active antiretroviral therapy (HAART). She presented with features of heart failure over a course of two months. Her laboratory evaluation was significant for leucopenia with a low CD4 count, high viral load, elevated ESR and CRP. The ECG showed a sinus tachycardia with diffuse ST-T segment changes and LVH with strain. Initial echo revealed dilated left heart chambers with poor LV systolic function and a small pericardial effusion with the development of an LV thrombus on follow up echo evaluation. She was started on heart failure medicines and had anticoagulation for the LV thrombus. She received adherence counseling and her HAART regimen was changed. Six months after presentation she became asymptomatic with higher CD4 counts and a normal LV size and function on echo. CONCLUSION: Immunological recovery following a switch of a failing or potentially cardiotoxic HAART in addition to improved HAART adherence may result in resolution of left ventricular dysfunction. Early and regular cardiology evaluation may improve outcomes in these patients.