Outcomes post Ozaki procedure among children with aortic valve disease at Jakaya Kikwete Cardiac Institute, Dar es Salaam, Tanzania: a retrospective descriptive study.

BACKGROUND: Aortic valve reconstruction using glutaraldehyde-treated autologous pericardium, also called Ozaki procedure, is a surgical procedure for patients with aortic valve disease. Gratifying results have been reported in adult patients, however, limited published data is available in paediatric population. This study looked at clinical characteristics and early outcomes of children who underwent Ozaki procedure at our Institute. METHODS: This was a retrospective descriptive study conducted on children who underwent aortic valve reconstruction at Jakaya Kikwete Cardiac Institute (JKCI) from January 2019 through December 2022. Medical records of these children were reviewed to extract data on demographics, clinical characteristics, redo surgical interventions and survival. RESULTS: A total of 10 children underwent Ozaki procedure during the study period. Eight children had severe aortic regurgitation while 2 had severe aortic stenosis preoperatively. All children had either none or trivial aortic regurgitation immediately after surgery. None of them had redone operations throughout the follow-up period. There was no in-hospital mortality, however, one child died one-year after surgery. The mean follow-up period was 1.6 years with the longest follow-up time of 4 years. CONCLUSION: Ozaki procedure showed encouraging early results among children with aortic valve disease who underwent surgical repair by this technique. Future studies with larger sample sizes and longer follow up periods to evaluate long-term results in this population are recommended.

Mortality and associated factors among children admitted to an intensive care unit in muhimbili national hospital, from the time of admission to three months after discharge: a prospective cohort study.

BACKGROUND: Mortality of children admitted to Intensive Care Units (ICU) is higher in low-to-middle-income countries (LMICs) as compared to high-income countries (HICs). There is paucity of information on outcomes following discharge from ICU, especially from sub-Saharan Africa region. This study was conducted to determine mortality and its associated factors among children admitted to Pediatric ICU (PICU) at Muhimbili National Hospital, from admission to three months after discharge. METHODOLOGY: This was a hospital-based prospective cohort study conducted between July 2021 and May 2022, among children admitted to PICU who were followed up for 3-month after discharge. Structured questionnaires were used to collect data from their medical charts. Telephone interviews were made after discharge. Medical records and verbal autopsy were used to determine the cause of death after discharge. Cox regression analysis was performed to assess the association between variables. A p-value of < 0.05 was considered statistically significant. Survival after PICU discharge was estimated by Kaplan - Meier curve. RESULTS: Of 323 children recruited, 177(54.8%) were male, with a median age of 17 months (1-168). The leading cause of PICU admission was severe sepsis 90/323(27.9%). A total of 161/323 children died, yielding an overall mortality of 49.8%. Of 173 children discharged from PICU, 33(19.1%) died. The leading cause of death among children who died in the general ward or as readmission into PICU was sepsis 4/17(23.5%). Respiratory diseases 4/16(25.0%) were the commonest cause of death among those who died after hospital discharge. Independent predictors of overall mortality included single organ dysfunction with hazard ratio(HR):5.97, 95% confidence interval (CI)(3.05-12.26)] and multiple organ dysfunction [HR:2.77,95%CI(1.03-2.21)]. Chronic illness[HR:8.13,95%CI(2.45-27.02)], thrombocytosis [HR:3.39,95%CI(1.32-8.73)], single[HR:3.57,95%CI(1.42-9.03)] and multiple organ dysfunction[HR:3.11,95%CI(1.01-9.61)] independently predicted post-PICU discharge mortality. CONCLUSION: Overall mortality and post- PICU discharge mortality were high and more likely to affect children with organ dysfunction, chronic illness, and thrombocytosis. The leading causes of mortality post- PICU discharge were sepsis and respiratory diseases. There is a need for a focused follow up plan of children post- PICU discharge, further research on the long term survival and strategies to improve it.

Late surgical ventricular septal defect closure in a low middle-income country setting: a case series.

BACKGROUND: Ventricular septal defect (VSD) is the commonest type of congenital heart lesion accounting for up to 40% of congenital heart defects. Well timed VSD closures are reported to yield excellent long-term outcomes. Late surgical VSD closures, particularly from the developing countries, are infrequently reported. CASE PRESENTATION: We report three cases of African children aged between 13 and 14 years who had late VSD presentations. They reported complaints of growth failure and recurrent respiratory infections since early infancy which necessitated frequent visits to primary health care facilities. They were found to have large ventricular septal defects by thoracic echocardiography. Diagnostic cardiac catheterization was done to all three patients to rule out irreversible pulmonary hypertension. After promising cardiac catheterization findings, they all underwent successful surgical VSD repair with good early outcomes. CONCLUSION: VSD surgical closure is ideal in children below 2 years, however, it can be done in children who present at advanced age despite being considered high risk patients. All three of our patients who presented late had successful surgical VSD repairs with promising immediate outcome. The role of genetics in the protection against developing irreversible pulmonary vascular disease in these patients is a possible area for future studies.

Sub-clinical rheumatic heart disease (RHD) detected by hand-held echocardiogram in children participating in a school-based RHD prevention program in Tanzania.

BACKGROUND: Rheumatic Heart Disease (RHD) continues to cause suffering and premature deaths in many sub-Saharan Africa (SSA) countries, where the disease is still endemic. RHD is largely preventable and determining its community burden is an important critical step in any RHD prevention program. METHODS: We conducted a cross-sectional study of 5-16 years old pupils from 11 primary schools participating in an RHD prevention program in 4 districts in Tanzania, between 2018 and 2019. At the school, all children were invited to participate after receiving consent from their parents/guardians. Participating children filled a questionnaire and were auscultated for cardiac murmurs. Echocardiographic screening was done by two experienced cardiologists, using a hand-held machine (V-Scan, GE®). All positive screening tests were stored for further examination by the same two cardiologists to reach to a consensus of definite, borderline or no RHD, using a modified World Heart Federation (WHF) criterion. RESULTS: Of the 6895 children invited, 4738 (68.7%) were screened and 4436 (64.3%) had complete data. The mean (SD) age was 10.04 (2.43) years, and 2422 (54.6%) were girls. Fifty three (1.2%) children were found to have a murmur. The proportion of children with trace or mild valvular regurgitation, sub-valvular/chordal thickening and valvular thickening/deformity were 8.3%, 1.3%, and 1.0%, respectively. Sub-clinical RHD was found in 95 children (59 definite and 36 borderline), giving a prevalence of 2.1%, [95% CI 1.7% - 2.6%]. Sub-clinical RHD was independently associated with female sex (aOR 1.83, 95% CI 1.18-2.85, p = 0.007), older age groups (aOR 1.73, 95% CI 1.10-2.72, p = 0.018 for age group 11-14 years; and aOR 3.02 95% CI 1.01-9.05, p = 0.048 for age group 15-16 years), as well as presence of a cardiac murmur, aOR 5.63 95% CI 2.31-13.69, p < 0.0001. None of the studied socio- or economic factors was associated with the presence of sub-clinical RHD in this study. CONCLUSION: The prevalence of sub-clinical RHD among primary school children in Tanzania is 2.1%, similar to previous reports in SSA. Efforts to prevent and control RHD in our communities are highly warranted.

Characteristics and immediate outcomes of patients who underwent percutaneous balloon mitral valvuloplasty at the Jakaya Kikwete Cardiac Institute, Tanzania.

BACKGROUND: For rheumatic mitral stenosis (MS), a multidisciplinary evaluation is mandatory to determine the optimal treatment: medical, percutaneous balloon mitral valvuloplasty (PBMV) or valve surgery. Clinical and imaging evaluations are essential for procedural risk assessment and outcomes. PBMV interventions are increasingly available in Africa and are feasible options for selected candidates. Enhancing PBMV training/skills transfer across most of African countries is possible. OBJECTIVES: The aim of this study was to provide insight into the clinical practice of patients with rheumatic MS evaluated for PBMV in a Tanzanian teaching hospital and to define the role of imaging, and evaluate the heart team and training/skills transfer in PBMV interventions. METHODS: From August 2019 to May 2022, 290 patients with rheumatic MS were recruited consecutively in the Tanzania Mitral Stenosis study. In total, 43 (14.8%) patients were initially evaluated for eligibility for PBMV by a heart team. We carried out the clinical assessment, laboratory investigations, transthoracic/oesophageal echocardiography (TTE/TEE) and electrocardiography. RESULTS: The median age was 31 years (range 11-68), and two-thirds of the patients were female (four diagnosed during pregnancy). Two patients had symptomatic MS at six and eight years. Nine patients had atrial fibrillation with left atrial thrombus in three, and two were detected by TEE. Nine patients in normal sinus rhythm had spontaneous echo contrast. The mean Wilkins score was 8.6 (range 8-12). With re-evaluation by the local and visiting team, 17 patients were found to have unfavourable characteristics: Bi-commissural calcification (four), ≥ grade 2/4 mitral regurgitation (six), high scores and left atrial thrombus (three), left atrial thrombus (two), and severe pulmonary hypertension (two). Three patients died before the planned PBMV. Eleven patients were on a waiting list. We performed PBMV in 12 patients, with success in 10 of these, and good short-term outcomes [mean pre-PBMV (16.03 ± 5.52 mmHg) and post-PBMV gradients (3.08 ± 0.44 mmHg, p < 0.001)]. There were no complications. CONCLUSIONS: PBMV had good outcomes for selected candidates. TEE is mandatory in pre-PBMV screening and for procedural guidance. In our cohort, patients with Wilkins score of up to 11 underwent successful PBMV. We encourage PBMV skills expansion in low- and middle-income countries, concentrating on expertise centres.

Newborn Screening for Critical Congenital Heart Disease in a Low-Resource Setting; Research Protocol and Preliminary Results of the Tanzania Pulse Oximetry Study.

Background: Critical Congenital Heart Disease (CCHD) is the leading cause of early new-born mortality. Its early detection and intervention is crucial for the survival of affected new-born. Pulse Oximetry (POX) has shown to be one of the feasible, accurate and cost-effective tools in screening CCHD in developed nations, it is yet to be practiced and established as standard of care in a low-resource setting. Objectives: This paper reports on the research protocol and preliminary results of an ongoing study regarding the performance of POX in detecting CCHD in new-borns in a low resource setting. Secondary objectives include investigating the burdens of CCHD and outcome at 12 months of age. Methods: The Tanzanian Pulse Oximetry Study (TPOXS) is a prospective cohort study which plans to enrol 30,000 mothers and new-borns delivered at two referral hospitals in Tanzania. New-borns are offered POX test 12 hours after birth, those positively undergoes echocardiography examinations. Confirmed with CCHD are placed under observation for up to first birthday. Results: During a 5-months pilot period, a total of 1,592 infants at the Muhimbili National Hospital, received POX test .65% of them were post-caesarean section and 52% being male. Most babies delivered through Spontaneous Vertex Delivery (SVD) were promptly discharge and did not get screened. The detection-rate of CCHD was 2.5 per 1,000 live births (at 95% confidence interval [CI] 0.9 to 6.7 per 1000 live birth); with a POX false positive rate of 0.6%. Seven false-positive infants out of 10 were found to carry significant other neonatal conditions, including persistent pulmonary hypertension of the new-born, transient tachypnoeic and neonatal sepsis. Conclusion: This paper provides the protocol of the ongoing TPOXS with the preliminary results showing prevalence matching closely the global data. It shows acceptability of POX screening for CCHD in a well-prepared low resource setting. Highlight: This study addresses the utilization of pulse oximeter in detecting critical congenital heart disease (CCHD) in a low-resource setting (such as sub-Saharan African countries).

Transcatheter occlusion of giant congenital coronary cameral fistulae: a case series.

BACKGROUND: A coronary cameral fistula is a rare connection between a coronary artery and a cardiac chamber or vein bypassing the cardiac capillary bed system. Most of these fistulae are congenital and solitary, although they can be acquired and multiple. CASES PRESENTATION: Case 1: A 10-year-old black South African boy presented with a long-standing history of fatigue; he had a heart murmur, and a bounding pulse and wide pulse pressure. An echocardiogram demonstrated a large coronary cameral fistula involving his left coronary artery and his left ventricle. This was also confirmed on ascending aortogram. Surgical ligation was done and his symptoms improved afterward, but a small residual fistula remained. Case 2: A 7-year-old black South African boy had decreased effort tolerance and a heart murmur on the mid-sternal border. He had cardiomegaly on chest roentgenogram and a dilated left coronary artery origin on echocardiogram. An ascending aortogram confirmed a large left coronary cameral fistula draining to the left ventricle. Case 3: A 28-year-old black South African woman with decreased effort tolerance and chest pain on exertion had a continuous murmur over the lower sternal border. Echocardiography demonstrated a dilated right coronary artery with a fistulous connection to her right ventricle. An ascending aortogram demonstrated a tortuous coronary cameral fistula arising from her right coronary artery to her right ventricle. All three patients were successfully treated percutaneously using the Amplatzer vascular plug type II device. CONCLUSION: The availability of numerous vascular closure devices has made transcatheter occlusion the treatment of choice for the majority of coronary cameral fistulae, rather than the traditional surgical ligation.

Transcranial Doppler and Magnetic Resonance in Tanzanian Children With Sickle Cell Disease.

Background and Purpose- We determined prevalences of neurological complications, vascular abnormality, and infarction in Tanzanian children with sickle cell disease. Methods- Children with sickle cell disease were consecutively enrolled for transcranial Doppler; those with slightly elevated (>150 cm/s), low (<50 cm/s) or absent cerebral blood flow velocity (CBFv) were invited for brain magnetic resonance imaging and magnetic resonance angiography. Results- Of 200 children (median age 9; range 6-13 years; 105 [2.5%] boys), 21 (11%) and 15 (8%) had previous seizures and unilateral weakness, respectively. Twenty-eight (14%) had elevated and 39 (20%) had low/absent CBFv, all associated with lower hemoglobin level, but not higher indirect bilirubin level. On multivariable analysis, CBFv>150 cm/s was associated with frequent painful crises and low hemoglobin level. Absent/low CBFv was associated with low hemoglobin level and history of unilateral weakness. In 49 out of 67 children with low/absent/elevated transcranial Doppler undergoing magnetic resonance imaging, 43% had infarction, whereas 24 out of 48 (50%) magnetic resonance angiographies were abnormal. One had hemorrhagic infarction; none had microbleeds. Posterior circulation infarcts occurred in 14%. Of 11 children with previous seizure undergoing magnetic resonance imaging, 10 (91%) had infarction (5 silent) compared with 11 out of 38 (29%) of the remainder ( P=0.003). Of 7 children with clinical stroke, 2 had recurrent stroke and 3 died; 4 out of 5 had absent CBFv. Of 193 without stroke, 1 died and 1 had a stroke; both had absent CBFv. Conclusions- In one-third of Tanzanian children with sickle cell disease, CBFv is outside the normal range, associated with frequent painful crises and low hemoglobin level, but not hemolysis. Half have abnormal magnetic resonance angiography. African children with sickle cell disease should be evaluated with transcranial Doppler; those with low/absent/elevated CBFv should undergo magnetic resonance imaging/magnetic resonance angiography.