Primary surgical repair of tetralogy of fallot at the Uganda Heart Institute: a ten-year review of 30day mortality and morbidity.

BACKGROUND: Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD) worldwide. It accounts for 7% of CHD cases in Uganda and leads to fatal outcomes in the long term without surgery. Surgery is often delayed in developing countries like Uganda due to limited resources. OBJECTIVE: This study aimed to determine the early surgical outcomes of patients with TOF who underwent primary intracardiac repair at the Uganda Heart Institute (UHI) and to identify factors associated. METHODOLOGY: This retrospective chart review evaluated outcomes of primary TOF repair patients at UHI from February 2012 to October 2022. Patient outcomes were assessed from surgery until 30 days post-operation. RESULTS: Out of the 104 patients who underwent primary TOF repair at UHI, records of 88 patients (84.6%) were available for review. Males accounted for 48.9% (n = 43). The median age at the time of operation was 4 years (with an interquartile range of 2.5-8.0 years), ranging from 9 months to 16 years. Genetic syndromes were present in 5/88 (5.7%). These included 2 patients with trisomy 21, 2 with Noonan's, and 1 with 22q11.2 deletion syndrome. Early postoperative outcomes for patients included: residual ventricular septal defects in 35/88 (39.8%), right ventricular dysfunction in 33/88 (37.5%), residual pulmonary regurgitation in 27/88 (30.7%), residual right ventricular outflow tract obstruction in 27/88 (30.0%), pleural effusion in 24/88 (27.3%), arrhythmias in 24/88(27.3%), post-operative infections in 23/88(26.1%) and left ventricular systolic dysfunction in 9/88 (10.2%). Out of the children who underwent surgery after one year of age, 8% (7 children) died within the first 30 days. There was a correlation between mortality and post-operative ventilation time, cardiopulmonary bypass (CPB) time, aortic cross-clamp time, preoperative oxygen saturations, RV and LV dysfunction and the operating team. CONCLUSION: The most frequent outcomes after surgery were residual ventricular septal defects and right ventricular failure. In our study, the 30-day mortality rate following TOF repair was 8%. Deceased patients had lower pre-operative oxygen levels, longer CPB and cross-clamp times, longer post-operative ventilation, RV/LV dysfunction, and were more likely operated by the local team.

Chest closure without drainage after open patent ductus arteriosus ligation in Ugandan children: A non blinded randomized controlled trial.

BACKGROUND: There is clinical equipoise regarding post-operative management of patients with patent ductus arteriosus (PDA) without insertion of a chest drain. This study evaluated post operative outcomes of chest closure with or without a drain following Patent Ductus Arteriosus ligation among childen at Uganda Heart Instritute (UHI). METHODS: This was an open label randomized controlled trial of 62 children 12 years of age and below diagnosed with patent ductus arteriosus at Mulago National Teaching and Referral Hospital, Uganda. Participants were randomized in the ratio of 1:1 with surgical ligation of patent ductus arteriosus to either thoracotomy closure with a chest tube or without a chest tube. All participants received standard care and were monitored hourly for 24 hours then until hospital discharge. The combined primary endpoint consisted of significant pleural space accumulation of fluid or air, higher oxygen need or infection of the surgical site. Analysis was conducted by multivariable logistic regression analysis at 5 % significance level. RESULTS: We enrolled 62 participants, 46 (74 %) of whom were females. Their median age was 12 months (IQR: 8-36). Participants in the no-drain arm significantly had less post-operative complications compared to the drain arm (Unadjusted odds ratio [uOR]: 0.21, 95 % CI: 0.06-0.73, p = 0.015). This "protective effect" remained without statistical significance in the multivariable regression model (Adjusted odds ratio [aOR]: 0.07, 95 % CI: 0.00-2.50, p = 0.144). CONCLUSION: Children aged below 6 years with patent ductus arterious can safely and effectively have thoracotomy closure without using a drain in uncomplicated surgical ligation of the PDA. Chest drain was associated with post-operative complications. TRIAL REGISTRATION: The trial was registered in the Pan African Clinical Trials registry on 1st/July/2012, retrospectively registered. Identifier number PACTR201207000395469 .

Pre-intervention wait time and survival in people with rheumatic heart disease in Uganda.

BACKGROUND: There is an unmet surgical burden among people living with rheumatic heart disease (RHD) in Uganda. Nevertheless, risk factors associated with time to first intervention and preoperative mortality is poorly understood. METHODS: Individuals with RHD who met indications for valve surgery were identified using the Uganda National RHD Registry (Jan. 2010- Aug. 2022). Kaplan-Meier estimates and multivariable Cox proportional hazard models were utilized. RESULTS: 64% of the cohort with clinical RHD (1452 of 2269) met criteria for index operation. Of those, 13.5% obtained surgical intervention while 30.6% died before surgery. The estimated likelihood of first surgery was 50% at 9.3 years of follow up (95% CI 8.1-upper limit not reached). Intervention was more likely in men vs. women (hazard ratio [HR] 1.78; 95% CI 1.21-2.64), those with post-secondary education vs. primary school or less (HR 3.60; 95% CI 1.88-6.89), and those with history of atrial fibrillation (HR 2.78; 95% CI 1.63-4.76). Surgery was less likely for adults (vs. those <18 years; HR 0.49; 95% CI 0.32-0.77) and those with NYHA class III/IV (vs. I/II; HR 0.51; 95% CI 0.32-0.83). The median preoperative survival time among those awaiting surgery was 4.6 years (95% CI, 3.9-5.7). History of infective endocarditis, RV dysfunction, pericardial effusion, atrial fibrillation, and having surgical indications for multiple valves were associated with increased mortality. CONCLUSIONS: Our analysis revealed a prolonged time to first surgical intervention and high pre-intervention mortality for RHD in Uganda, with factors such as age, sex, and education level remaining barriers to obtaining surgery.

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.

Clinical Profile and Outcomes of Rheumatic Heart Disease Patients Undergoing Surgical Valve Procedures in Uganda.

Background: Chronic valvular heart disease is a well-known, long-term complication of acute rheumatic fever (ARF), which remains a major public health problem in low- and middle-income countries. Access to surgical management remains limited. Outcomes of the minority proportion of patients that access surgery have not been described in Uganda. Objectives: To describe the volume and type of rheumatic heart disease (RHD) valvular interventions and the outcomes of operated patients in the Uganda RHD registry. Methods: We performed a retrospective cohort study of all valve surgery procedures identified in the Uganda RHD registry through December 2021. Results: Three hundred and sixty-seven surgical procedures were performed among 359 patients. More than half were among young (55.9% were ≤20 years of age), female (59.9%) patients. All patients were censored at 15 years. The median (IQR) follow up period was 43 (22,79) months. Nearly half of surgeries (46.9%) included interventions on multiple valves, and most valvular operations were replacements with mechanical prostheses (96.6%). Over 70% of the procedures were sponsored by charity organizations. The overall mortality of patients who underwent surgery was 13% (47/359), with over half of the mortalities occurring within the first year following surgery (27/47; 57.4%). Fifteen-year survival or freedom from re-operation was not significantly different between those receiving valve replacements and those receiving valve repair (log-rank p = 0.76). Conclusions: There has been increasing access to valve surgery among Ugandan patients with RHD. Post-operative survival is similar to regionally reported rates. The growing cohort of patients living with prosthetic valves necessitates national expansion and decentralization of post-operative care services. Major reliance on charity funding of surgery is unsustainable, thus calling for locally generated and controlled support mechanisms such as a national health insurance scheme. The central illustration (Figure 1) provides a summary of our findings and recommendations.

Clinical outcomes of children with rheumatic heart disease.

OBJECTIVE: To evaluate the long-term clinical outcomes of children with rheumatic heart disease (RHD) in Uganda, and determine characteristics that predict adverse outcomes. METHODS: This retrospective cohort study evaluated the risk of death in Ugandan children with clinical RHD from 2010 to 2018; enrolling children aged 5-18 years old from an existing registry. Demographic data and clinical data (baseline complications, RHD severity, cardiac interventions) were collected. The primary outcome was survival. Univariable and multivariable hazard ratios (HR) were obtained from Cox proportional hazards regression. Survival probabilities were developed using Kaplan-Meier curves; log-rank tests compared survival based on cardiac interventions, disease severity and time of enrolment. RESULTS: 612 cases met inclusion criteria; median age 12.8 years (IQR 5.3), 37% were male. Thirty-one per cent (187 of 612) died during the study period; median time to death 7.8 months (IQR 18.3). In univariable analysis, older age (HR 1.26, 95% CI=1.0 to 1.58), presence of baseline complications (HR 2.06, 95% CI=1.53 to 2.78) and severe RHD (HR 5.21, 95% CI=2.15 to 12.65) were associated with mortality. Cardiac intervention was associated with a lower risk of mortality (HR 0.06, 95% CI=0.02 to 0.24). In multivariable models, baseline complications (HR 1.78, 95% CI=1.31 to 2.41), severe RHD (HR 4.58, 95% CI=1.87 to 11.23) and having an intervention (HR 0.05, 95% CI=0.01 to 0.21) remained statistically significant. Kaplan-Meier survival curves demonstrated >25% mortality in the first 30 months, with significant differences in mortality based on intervention status and severity of disease. CONCLUSIONS: The mortality rate of children with clinical RHD in Uganda exceeds 30%, over an 8-year time frame, despite in-country access to cardiac interventions. Children at highest risk were those with cardiac complications at baseline and severe RHD.

An unusual occurrence of penetrating aortic arch injury by a ball-point pen: a case report and review of the literature.

BACKGROUND: Aortic arch injuries account for about 8% of thoracic aortic injuries. Penetrating zone I neck injuries account for 18% of vascular injuries in the neck and have great potential to traverse to involve thoracic vascular structures as well. The hard and soft signs of vascular injury facilitate triage of patients on an individual basis. We present a case of a ball-point pen traversing through zone I of the neck and causing penetrating aortic arch injury with minimal mediastinal haemorrhage. CASE PRESENTATION: We present a polytrauma patient who was admitted with traumatic brain injury and a ball-point pen lodged above the sternal notch in zone I of the neck following a road traffic accident. He underwent mediastinal exploration via a median sternotomy. The ball-point pen was found penetrating the anterior wall of the aortic arch and resting in its lumen. The ball-point pen was successfully explanted and primary repair of the penetrating aortic arch injury was done. He had an uneventful recovery without any added secondary neurological complications. CONCLUSION: Penetrating aortic arch injuries are rare compared to injuries of the ascending aorta and descending aorta. They require a high index of suspicion, rapid investigation and urgent intervention in view of their high associated fatality. The ball-point pen in this case assumed the shape of a plug which acted as a seal at the site of injury preventing catastrophic exsanguination.

Makerere University-Uganda Heart Institute collaborative cardiovascular disease training and research since 1988.

Background: Makerere University College of Health Sciences has been collaborating with the Uganda Heart Institute to build capacity for research, training and clinical care in cardiovascular medicine for the last 34 years to appropriately respond to rising societal needs for advanced cardiovascular care which was lacking before this period. Aim: To describe the major milestones in the MakCHS-UHI cardiovascular training collaboration and chart way for future collaborations. Method: This short communication highlights some of the salient features and important milestones in the collaboration journey of the two institutions. Conclusion: Clinical centres of excellence in specialised fields of health care, such as the Uganda Heart Institute for Cardiology, provide a conducive academic environment for MakCHS clinical scientists to provide high quality evidence-based care to meet societal needs.

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.

Reimplantation of the two coronary ostia as a single button in Ross procedure.

A 10-year-old boy presented with severe aortic regurgitation due to a dysplastic aortic valve. He underwent a Ross procedure employing a mini-root replacement technique. At surgery, he was found to have eccentrically located coronary ostia which were both reimplanted as a single button into the pulmonary autograft. Postoperatively, multislice computed tomographic coronary angiography demonstrated satisfactory reimplantation of the coronary artery ostia with no evidence of kinking or compression of the coronary arteries. This case report supports the technique of transfer of coronary ostia as a single button where coronary anatomy is unfavorable for their individual transfer.

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.

Deep venous thrombosis after major abdominal surgery in a Ugandan hospital: a prospective study.

BACKGROUND: Deep venous thrombosis (DVT) is a major cause of morbidity and mortality among postoperative patients. Its incidence has been reported to range between 16% and 38% among general surgery patients and may be as high as 60% among orthopaedic patients. The most important clinical outcome of DVT is pulmonary embolism, which causes about 10% of hospital deaths. In over 90% of patients, occurrence of DVT is silent and presents no symptoms until onset of pulmonary embolism and/or sudden death. The only effective way of guarding against this fatal condition is therefore prevention/prophylaxis. However, prophylaxis programs are usually based on the estimated prevalence of DVT in that particular community. There is currently no data concerning rates of postoperative DVT in Uganda.The purpose of the study was therefore to determine the prevalence of DVT among postoperative patients at Mulago Uganda's National Referral Hospital. METHODS: A cross sectional descriptive study was conducted between March and June 2011. Eligible patients were identified and screened and patient details were collected. Clinical examinations were done on postoperative days (PODs) 1, 2, and 4 and Doppler ultrasounds were done on POD 7 and POD 21 to assess for DVT. Patients found with DVT were treated appropriately according to local treatment guidelines. RESULTS: A total of 82 patients were recruited, 4/82 (5%) had DVT. The most common risk factor was cancer. The overall mean age was 45 years (range 20-83 years). The male to female ratio was 1.6:1. Participants with more than one risk factor for DVT were 16/82 (20%). CONCLUSIONS: Prevalence of DVT among major post-abdominal surgery patients was low (5%). Cancer was the most common associated factor apart from surgery.

Tricuspid reoperation after left-sided rheumatic valve operations.

BACKGROUND: The management of late tricuspid regurgitation after left-sided valve operations in rheumatic patients remains controversial. The aim of this study was to analyze clinical and echocardiographic outcomes of tricuspid valve procedures after left-sided valve operations in rheumatic patients. METHODS: This study enrolled 106 rheumatic patients with a history of left-sided valve operations who were undergoing tricuspid valve procedures (53 replacements, 53 repairs). Follow-up was 97% complete, with a mean follow-up of 62 ± 42 months. Clinical and echocardiographic data were analyzed. RESULTS: The early mortality rate was 1.9% (2 of 106 patients). There was no significant difference in cumulative survival at 10 years between patients who underwent tricuspid valve replacement (63.1% ± 13.2%) or repair (80.7% ± 0.8%, p = 0.317). Multivariable Cox regression analysis revealed that old age (hazard ratio [HR], 6.5; p = 0.007), anemia (HR, 10.9; p = 0.004), and left ventricular ejection fraction of less than 0.4 (HR, 10.3; p = 0.001) were predictors of major adverse cardiac events. Among patients who underwent tricuspid valve repair, multivariate analysis revealed that the aortic transprosthetic mean pressure gradient at late follow-up was an independent predictor of late tricuspid regurgitation. CONCLUSIONS: Tricuspid valve procedures after left-sided valve operations in rheumatic patients can be performed at low risk with good clinical outcomes. For improved clinical outcomes, early surgical intervention should be considered before the development of anemia and left ventricular dysfunction. A lower aortic transprosthetic mean pressure gradient may help prevent late progression of tricuspid regurgitation in a clinical setting.

Destructive rib lesions in an HIV sero-negative male: an unusual presentation of tuberculosis in a high tuberculosis prevalence setting.

Tuberculosis (TB) of the rib is a very rare form of skeletal TB and its diagnosis may be difficult because of a low index of suspicion by clinicians. The presentation often mimics malignant disease clinically and radiologically and diagnosis may only be confirmed by tissue biopsy. We present a 32-year-old HIV-negative man who presented with a three-month history of progressively worsening pleuritic chest pain, weight loss, fatigue, anorexia and low-grade fever with night sweats. A chest computerized tomography (CT) scan showed destructive lesions in the right fourth and seventh ribs with no pulmonary lesions. A diagnosis of TB of the rib was made after surgical resection and histopathology of the affected tissue. There was significant improvement when anti-TB therapy was initiated. This case report emphasizes the importance of a high index of suspicion of TB in patients presenting with destructive bone lesions in regions with high prevalence of TB.