Sulaiman Lubega

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.

Brief Report: Prevalence of Latent Rheumatic Heart Disease Among HIV-Infected Children in Kampala, Uganda.

Abstract:Rheumatic heart disease (RHD) remains highly prevalent in resource-constrained settings around the world, including countries with high rates of HIV/AIDS. Although both are immune-mediated diseases, it is unknown whether HIV modifies the risk or progression of RHD. We performed screening echocardiography to determine the prevalence of latent RHD in 488 HIV-infected children aged 5–18 in Kampala, Uganda. The overall prevalence of borderline/definite RHD was 0.82% (95% confidence interval: 0.26% to 2.23%), which is lower than the published prevalence rates of 1.5%–4% among Ugandan children. There may be protective factors that decrease the risk of RHD in HIV-infected children.

Echocardiographic pattern and severity of valve dysfunction in children with rheumatic heart disease seen at Uganda Heart Institute, Mulago hospital.

BACKGROUND Rheumatic heart disease (RHD) is the commonest acquired heart disease in children worldwide but in Uganda, data is scarce regarding its morbidity and mortality. The disease has a progressive course and patients usually require valve repair/replacement in the future. OBJECTIVES To describe the frequency of echocardiographic valvular dysfunction in children with RHD To explore the relationship between the severity of valvular dysfunction by the age and sex of the children with RHD. METHODS Echocardiographic findings of children ≤15 years with RHD seen at Uganda Heart Institute from January 2007 to December 2011 were retrospectively analyzed. RESULTS 376 children had a diagnosis of RHD. The mean age of the children was 11.0 ± 2.7 years and 216 (57.4%) were females. Mitral regurgitation was the commonest lesion seen in 98.9% (severe in 73.1%) of the children. Aortic regurgitation (AR) was found in 51.3% (severe in 7.2%), mitral stenosis (MS) was found in 10.6% (severe in 5.9%), tricuspid regurgitation was found in 86.7% (severe in 8.2%) while aortic stenosis was seen in 1.3% (severe in 0.3%). Severe AR was less common in females (OR=0.32, 95%CI 0.13-0.78) and children with MS were older than those without MS (12.7 ± 2.0 Vs. 10.7 ± 2.7 years, p<0.00). CONCLUSIONS Mitral valve dysfunction was found in almost all the cases of RHD and majority of the children had severe valve disease at the time of their first presentations. Children with MS were predominantly above 10 years and severe AR was more common in males.

Latent Rheumatic Heart Disease: Identifying the Children at Highest Risk of Unfavorable Outcome.

Background: Screening echocardiography has emerged as a potentially powerful tool for early diagnosis of rheumatic heart disease (RHD). The utility of screening echocardiography hinges on the rate of RHD progression and the ability of penicillin prophylaxis to improve outcome. We report the longitudinal outcomes of a cohort of children with latent RHD and identify risk factors for unfavorable outcomes. Methods: This was a prospective natural history study conducted under the Ugandan RHD registry. Children with latent RHD and ≥1 year of follow-up were included. All echocardiograms were re-reviewed by experts (2012 World Heart Federation criteria) for inclusion and evidence of change. Bi- and multivariable logistic regression, Kaplan-Meier analysis, and Cox proportional hazards models, as well, were developed to search for risk factors for unfavorable outcome and compare progression-free survival between those treated and not treated with penicillin. Propensity and other matching methods with sensitivity analysis were implemented for the evaluation of the penicillin effect. Results: Blinded review confirmed 227 cases of latent RHD: 164 borderline and 63 definite (42 mild, 21 moderate/severe). Median age at diagnosis was 12 years and median follow-up was 2.3 years (interquartile range, 2.0–2.9). Penicillin prophylaxis was prescribed in 49.3% with overall adherence of 84.7%. Of children with moderate-to-severe definite RHD, 47.6% had echocardiographic progression (including 2 deaths), and 9.5% had echocardiographic regression. Children with mild definite and borderline RHD showed 26% and 9.8% echocardiographic progression and 45.2% and 46.3% echocardiographic improvement, respectively. Of those with mild definite RHD or borderline RHD, more advanced disease category, younger age, and morphological mitral valve features were risk factors for an unfavorable outcome. Conclusions: Latent RHD is a heterogeneous diagnosis with variable disease outcomes. Children with moderate to severe latent RHD have poor outcomes. Children with both borderline and mild definite RHD are at substantial risk of progression. Although long-term outcome remains unclear, the initial change in latent RHD may be evident during the first 1 to 2 years following diagnosis. Natural history data are inherently limited, and a randomized clinical trial is needed to definitively determine the impact of penicillin prophylaxis on the trajectory of latent RHD.

Cardiac Dysfunction Among Ugandan HIV-infected Children on Antiretroviral Therapy.

Background: Despite effective antiretroviral therapy (ART), HIV-infected children on treatment have been observed to have cardiac abnormalities. We sought to determine the prevalence, types and factors associated with cardiac abnormalities among HIV-infected Ugandan children on combination ART. Methods: We carried out a cross-sectional study from July 2012 to January 2013, at Joint Clinical Research Centre among HIV-infected children aged 1–18 years. Cardiac abnormalities were assessed using electrocardiography and echocardiography. CD4 counts, viral load and complete blood count were performed at enrollment. The prevalence of cardiac abnormalities was determined using simple proportions with the associated factors ascertained using logistic regression. Results: Among 285 children recruited, the median (interquartile range) age was 9 (6–13) years, 54% were female; 72% were on first line combination ART. Their mean (±SD) CD4 count was 1092 (±868.7) cells/mm3; median (interquartile range) viral load was 20 (20–76) copies/mL. Ninety-four percent had adherence to ART of more than 95%. Cardiac abnormalities were detected in 39 (13.7%) children. The most common abnormalities by electrocardiography and echocardiography were nonspecific T wave changes (4.6%) and pericardial disease (thickened pericardium with or without pericardial effusion; 2.8%), respectively. No factor assessed was found to be significantly associated occurrence of cardiac dysfunction. Conclusions: The prevalence of cardiac dysfunction among the HIV-infected children on ART was 13.7%, which was high, with nonspecific T wave changes and pericardial disease being the most frequent abnormalities observed. No factor assessed was found to be associated with cardiac dysfunction.

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.

Autoimmune vasculitis in a child following tetralogy of Fallot repair in Uganda

Tetralogy of Fallot is the most common cyanotic congenital heart disease worldwide [1]. Among other conotruncal abnormalities, 13-16% of Tetralogy of Fallot individuals are reported to have 22q11.2 deletion syndrome which is characterized by thymic hypoplasia, endocrine abnormalities and hypocalcaemia [2]. Individuals with micro deletion 22q11.2 deletion syndrome are prone to auto immune disorders. Defects in thymic development predispose them to impaired immune function especially T cell deficiency [3]. However autoimmune vasculitis has not been well documented in children with Tetralogy of Fallot. We report on an eleven-year-old child post Tetralogy of Fallot repair who presented with persistent fevers and an over whelming infection.

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.