Pattern of congenital heart disease among children presenting to the Uganda Heart Institute, Mulago Hospital: a 7-year review

Background: Congenital heart disease (CHD) is the most common congenital anomaly in children. Over half of the deaths due to CHD occur in the neonatal period. Most children with unrepaired complex heart lesions do not live to celebrate their first birthday. We describe the spectrum of congenital heart disease in Uganda. Methods: We retrospectively reviewed the data of children with CHD who presented to the Uganda Heart Institute (UHI), Mulago Hospital Complex from 2007 to 2014. Results: A total of 4621 children were seen at the UHI during the study period. Of these, 3526 (76.3%) had CHD; 1941(55%) were females. Isolated ventricular septal defect (VSD) was the most common CHD seen in 923 (27.2%) children followed by Patent ductus arteriosus (PDA) 760 (22%) and atrial septal defects (ASD) 332 (9.4%). Tetralogy of Fallot (TOF) and Truncus arteriosus were the most common cyanotic heart defects (7% and 5% respectively). Dysmorphic features were diagnosed in 185 children, of which 61 underwent genetic testing (Down syndrome=24, 22q11.2 deletion syndrome n=10). Children with confirmed 22q11.2 deletion had conotruncal abnormalities. Conclusion: Isolated VSD and Tetralogy of Fallot are the most common acyanotic and cyanotic congenital heart defects. We report an unusually high occurrence of Truncus arteriosus

Health Related Quality of Life of Ugandan Children Following Valve Replacement Surgery for Rheumatic Heart Disease

Background: Valve replacement surgery (VRS) improves clinical outcomes in patients with severe rheumatic heart disease (RHD). However, lifelong anticoagulation and frequent monitoring are required, which potentially impacts health-related quality of life (HRQoL). In this study, we assessed the HRQoL of people with RHD in Uganda following VRS. Methods: This was a hospital-based, cross-sectional study conducted between March and August 2021. Eligible participants were individuals who had VRS before the age of 18 years. The Pediatric Quality of Life Inventory–Cardiac Module (PedsQL-Cardiac module) was used to evaluate HRQoL. A total mean score of ≥ 80% was considered as optimal HRQoL. Results: Of the 83 eligible participants, 52 (60.5%) were female, with a median age of 18 (interquartile range: 14–22) years. Most participants had NYHA I functional status (n = 79, 92%). Most (n = 73, 92.4%) surgeries were performed outside of Uganda, and 61 (72.6%) were single mechanical valve replacement. Almost half (n = 45, 54%) expressed no concern about being on life-long warfarin therapy. However, 24 (29.3%) feared bleeding. The optimal mean score of cardiac-specific HRQoL was achieved in 50 (60.2%) of participants. Factors associated with optimal HRQoL were body mass index (BMI) (adjusted odds ratio (aOR), 1.2, 95% Confidence Interval: 1.1–1.3, p = 0.006), being afraid of bleeding or bruising (aOR: 1.5, 95% CI: 1.21–2.47, p = 0.004), acceptance of having an artificial valve (aOR: 2.7, 95% CI; 1.64–3.81, p < 0.001). Conclusion: HRQoL was optimal in about three in five participants following VRS. Increasing BMI and acceptance of artificial valve were significantly associated with optimal HRQoL

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

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

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

Prevalence and characteristics of primary left-sided valve disease in a cohort of 15,000 patients undergoing echocardiography studies in a tertiary hospital in Uganda

Abstract Background Although rheumatic heart disease remains the leading cause of valve heart disease (VHD) in developing countries, other forms of valve disease have been over shadowed and not regarded as a public health problem. However, several facts suggest that the role of non-rheumatic VHD as a significant cardiovascular disease should be reconsidered. We aimed to assess the prevalence and characteristics of different forms of primary left sided valve diseases from a series of 15,009 echocardiographic studies. Methods This was a retrospective review of echocardiographic reports for studies performed between January 2012 and December 2013 (24 months) at Uganda Heart Institute. All patients with primary left-sided valve disease were classified into one of five major diagnostic categories and in each diagnostic category; patients were sub-classified into stages A-D of primary valve disease as defined by the American College of Cardiology. Results Three thousand five hundred eighty-two echocardiography reports qualified for final data analysis. The “sclerotic valve changes with normal valve function”, a Stage A sub-class of “degenerative valve disease” overwhelmingly overshadowed all the other diagnostic categories in this stage. “Rheumatic Heart Disease”, “Degenerative Valve Disease”, “Bicuspid Aortic Valve”, “Mitral Valve Prolapse” and “Endomyocardial Fibrosis” diagnostic categories accounted for 53.0%, 41.8%, 2.2%, 1.4% and 1.7% respectively in stages B-D of primary VHD. Rheumatic heart disease disproportionately affected the young, productive age groups. It was the major risk factor for infective endocarditis; and was the indication for valve surgery in 44 of 50 patients who had undergone valve replacement procedures. Conclusions We acknowledge that rheumatic heart disease remains a leading cause of progressive and severe primary left-sided valve disease among young adults in Uganda. But we bring to light the contemporary footprints of other forms of primary valve disease that require coordinated multidisciplinary approach to research, education and clinical management to ensure improved patient outcomes