Rationale, Design, and the Baseline Characteristics of the RHDGen (The Genetics of Rheumatic Heart Disease) Network Study†.

BACKGROUND: The genetics of rheumatic heart disease (RHDGen) Network was developed to assist the discovery and validation of genetic variations and biomarkers of risk for rheumatic heart disease (RHD) in continental Africans, as a part of the global fight to control and eradicate rheumatic fever/RHD. Thus, we describe the rationale and design of the RHDGen study, comprising participants from 8 African countries. METHODS: RHDGen screened potential participants using echocardiography, thereafter enrolling RHD cases and ethnically-matched controls for whom case characteristics were documented. Biological samples were collected for conducting genetic analyses, including a discovery case-control genome-wide association study (GWAS) and a replication trio family study. Additional biological samples were also collected, and processed, for the measurement of biomarker analytes and the biomarker analyses are underway. RESULTS: Participants were enrolled into RHDGen between December 2012 and March 2018. For GWAS, 2548 RHD cases and 2261 controls (3301 women [69%]; mean age [SD], 37 [16.3] years) were available. RHD cases were predominantly Black (66%), Admixed (24%), and other ethnicities (10%). Among RHD cases, 34% were asymptomatic, 26% had prior valve surgery, and 23% had atrial fibrillation. The trio family replication arm included 116 RHD trio probands and 232 parents. CONCLUSIONS: RHDGen presents a rare opportunity to identify relevant patterns of genetic factors and biomarkers in Africans that may be associated with differential RHD risk. Furthermore, the RHDGen Network provides a platform for further work on fully elucidating the causes and mechanisms associated with RHD susceptibility and development.

Relationship between maternal and infant serum vitamin D levels in Jos, Plateau State, Nigeria: a cross-sectional study.

Introduction: there is growing interest in the link between maternal and infant vitamin D (VD) levels. Breast milk transmission and the fact that the mother and her child may share risk factors for VD, such as exposure to sunlight, diet, and sociocultural influences may impact VD status, the magnitude of which is largely unknown in our topical low-middle income setting. We assessed the connection between maternal and infant VD status including their correlates. Methods: this cross-sectional study investigated 95 maternal-infant pairs in Jos. Mothers were interviewed using a questionnaire. Blood sampling and analysis of serum total 25 hydroxy VD were performed using the chemiluminescent immunoassay method. Maternal and infant VD levels were classified as VD deficient (VDD), VD insufficient (VDI), and VD sufficient (VDS). The mean maternal and infant VD were compared, and the Spearman correlation between them was assessed, a stepwise linear regression was also performed with infant vitamin D as a dependent variable. For all statistical analysis, p<0.05 was considered significant. Results: the median maternal and infant VD was 29.68 ng/ml and 29.41 ng/ml, respectively. The mean infant VD (32.19 ± 10.61 ng/ml) was comparable to maternal VD (31.12 ± 12.94 ng/ml) (p=0.483), with a Spearman correlation coefficient of 0.3 (p=0.037). Maternal vitamin D (beta=0.539, duration of exclusive breastfeeding (beta=-3.490), and infant age (beta=1.655) were found to be significant independent predictors of infant vitamin. Conclusion: beyond neonatal age, a significant positive relationship between maternal and infants´ VD levels exists and suggests that family-focused vitamin D intervention might be an effective public health approach in the tropical city of Jos.

Data-independent acquisition mass spectrometry in severe rheumatic heart disease (RHD) identifies a proteomic signature showing ongoing inflammation and effectively classifying RHD cases.

BACKGROUND: Rheumatic heart disease (RHD) remains a major source of morbidity and mortality in developing countries. A deeper insight into the pathogenetic mechanisms underlying RHD could provide opportunities for drug repurposing, guide recommendations for secondary penicillin prophylaxis, and/or inform development of near-patient diagnostics. METHODS: We performed quantitative proteomics using Sequential Windowed Acquisition of All Theoretical Fragment Ion Mass Spectrometry (SWATH-MS) to screen protein expression in 215 African patients with severe RHD, and 230 controls. We applied a machine learning (ML) approach to feature selection among the 366 proteins quantifiable in at least 40% of samples, using the Boruta wrapper algorithm. The case-control differences and contribution to Area Under the Receiver Operating Curve (AUC) for each of the 56 proteins identified by the Boruta algorithm were calculated by Logistic Regression adjusted for age, sex and BMI. Biological pathways and functions enriched for proteins were identified using ClueGo pathway analyses. RESULTS: Adiponectin, complement component C7 and fibulin-1, a component of heart valve matrix, were significantly higher in cases when compared with controls. Ficolin-3, a protein with calcium-independent lectin activity that activates the complement pathway, was lower in cases than controls. The top six biomarkers from the Boruta analyses conferred an AUC of 0.90 indicating excellent discriminatory capacity between RHD cases and controls. CONCLUSIONS: These results support the presence of an ongoing inflammatory response in RHD, at a time when severe valve disease has developed, and distant from previous episodes of acute rheumatic fever. This biomarker signature could have potential utility in recognizing different degrees of ongoing inflammation in RHD patients, which may, in turn, be related to prognostic severity.

Association of Novel Locus With Rheumatic Heart Disease in Black African Individuals: Findings From the RHDGen Study.

Importance: Rheumatic heart disease (RHD), a sequela of rheumatic fever characterized by permanent heart valve damage, is the leading cause of cardiac surgery in Africa. However, its pathophysiologic characteristics and genetics are poorly understood. Understanding genetic susceptibility may aid in prevention, control, and interventions to eliminate RHD. Objective: To identify common genetic loci associated with RHD susceptibility in Black African individuals. Design, Setting, and Participants: This multicenter case-control genome-wide association study (GWAS), the Genetics of Rheumatic Heart Disease, examined more than 7 million genotyped and imputed single-nucleotide variations. The 4809 GWAS participants and 116 independent trio families were enrolled from 8 African countries between December 31, 2012, and March 31, 2018. All GWAS participants and trio probands were screened by use of echocardiography. Data analyses took place from May 15, 2017, until March 14, 2021. Main Outcomes and Measures: Genetic associations with RHD. Results: This study included 4809 African participants (2548 RHD cases and 2261 controls; 3301 women [69%]; mean [SD] age, 36.5 [16.3] years). The GWAS identified a single RHD risk locus, 11q24.1 (rs1219406 [odds ratio, 1.65; 95% CI, 1.48-1.82; P = 4.36 × 10-8]), which reached genome-wide significance in Black African individuals. Our meta-analysis of Black (n = 3179) and admixed (n = 1055) African individuals revealed several suggestive loci. The study also replicated a previously reported association in Pacific Islander individuals (rs11846409) at the immunoglobulin heavy chain locus, in the meta-analysis of Black and admixed African individuals (odds ratio, 1.16; 95% CI, 1.06-1.27; P = 1.19 × 10-3). The HLA (rs9272622) associations reported in Aboriginal Australian individuals could not be replicated. In support of the known polygenic architecture for RHD, overtransmission of a polygenic risk score from unaffected parents to affected probands was observed (polygenic transmission disequilibrium testing mean [SE], 0.27 [0.16] SDs; P = .04996), and the chip-based heritability was estimated to be high at 0.49 (SE = 0.12; P = 3.28 × 10-5) in Black African individuals. Conclusions and Relevance: This study revealed a novel candidate susceptibility locus exclusive to Black African individuals and an important heritable component to RHD susceptibility in African individuals.

Study of congenital heart defects among neonates in Jos, Nigeria: prevalence and spectrum.

BACKGROUND: There are few reports of the prevalence of CHD in the neonatal period in sub-Saharan Africa. The only available study in Nigeria was carried out before the widespread availability of echocardiography in the country. We sought to determine the prevalence and spectrum of congenital heart defects (CHD) among neonates in Jos, Nigeria. METHODS: This cross-sectional study enrolled neonates less than one week of age from the two largest hospitals and their immunisation centres. Relevant information was obtained and an echocardiogram was performed on each neonate. RESULTS: There were 3 857 neonates recruited over a two-year period; male-to-female ratio was 1.1:1. A total of 111 babies had CHD, with a prevalence of 28.8 per 1 000. Sixty-four neonates had mild CHD, with a prevalence of 16.6 per 1 000, while moderate and severe CHD were found in 27 (7.0 per 1 000) and 20 (5.2 per 1 000), respectively. CONCLUSIONS: CHD is prevalent in Nigerian neonates and there is therefore a need for advocacy to improve access to its diagnosis at birth for appropriate management.

Population and Individual-Level Double Burden of Malnutrition Among Adolescents in Two Emerging Cities in Northern and Southern Nigeria: A Comparative Cross-Sectional Study.

Background: Over the past three decades, double burden of malnutrition (DBM), a situation where high levels of undernutrition (stunting, thinness, or micronutrient deficiency) coexist with overnutrition (overweight and obesity), continues to rise in sub-Saharan Africa. Compared to other countries in the region, the evidence on DBM is limited in Nigeria. Objective: This paper aimed to determine the comparative prevalence of population-level and individual-level DBM among adolescents in two emerging cities in northern and southern Nigeria. Methods: This was a comparative cross-sectional study among apparently healthy secondary school adolescents aged 10-18 years in Gombe (northern Nigeria) and Uyo (southern Nigeria) between January 2015 and June 2017. A multistage random sampling technique was implemented to recruit adolescents from 24 secondary schools in both cities. Measures of general obesity (body mass index) and stature (height-for-age) were classified and Z-scores generated using the WHO AnthroPlus software, which is based on the WHO 2006 growth reference. Population-level DBM was defined as the occurrence of thinness and overweight/obesity within the population. Individual-level DBM was defined as the proportion of individuals who were concurrently stunted and had truncal obesity or stunted and were overweight/obese. Findings: Overall, at the population-level in both settings, 6.8% of adolescents had thinness, while 12.4% were overweight/obese signifying a high burden of population-level DBM. Comparatively, the population-level DBM was higher in Gombe compared to Uyo (thinness: 11.98% vs 5.3% and overweight/obesity: 16.08% vs 11.27% in Gombe vs Uyo respectively). Overall, at the individual level, 6.42% of stunted adolescents had coexisting truncal obesity, while 8.02% were stunted and had coexisting general overweight/obesity. Like the trend with population-level DBM, individual-level DBM was higher in Gombe (northern Nigeria) compared to Uyo (southern Nigeria). Conclusion: High levels of population-level and individual-level DBM exist in Gombe and Uyo. However, the level of DBM (under- and over-nutrition) is higher in Gombe located in northern Nigeria compared to Uyo in southern Nigeria.

Prevalence of rheumatic heart disease in North-Central Nigeria: a school-based cross-sectional pilot study.

OBJECTIVES: To present epidemiological data on rheumatic heart disease (RHD), the most common acquired heart disease in children and young adults in low- and middle-income countries, for North-Central Nigeria. METHODS: In this pilot study, we conducted clinical and echocardiography screening on a cross section of randomly selected secondary schoolchildren in Jos, North-Central Nigeria, from March to September 2016. For outcome classification into borderline or definite RHD, we performed a confirmatory echocardiography using the World Heart Federation criteria for those suspected to have RHD from the screening. RESULTS: A total of 417 secondary schoolchildren were screened, of whom 247 (59.2%) were female. The median age was 14 years (IQR: 13-15). Clinical screening detected 8/417 children, whereas screening echocardiography detected 42/417 suspected cases of RHD. Definitive echocardiography confirmed 9/417 with RHD corresponding to a prevalence of 21.6 per 1000 (95% CI, 6.7-36.5). All but one of the confirmed RHD cases (8/9) were borderline RHD corresponding to a prevalence of 19.2 per 1000 (95% CI, 8.3-37.5) for borderline RHD and 2.4 per 1000 (95% CI, 0.1-13.3) for definite RHD. RHD was more common in boys and cardiac auscultation missed over 50% of the cases. CONCLUSIONS: This study showed a high prevalence of RHD among secondary schoolchildren in North-Central Nigeria with a vast predominance of asymptomatic borderline lesions. Larger school-based echocardiography screening using portable or handheld echocardiography aimed at early detection of subclinical RHD should be adopted.

OBJECTIFS: Présenter des données épidémiologiques sur la cardiopathie rhumatismale (CR), la maladie cardiaque acquise la plus courante chez les enfants et les jeunes adultes dans les pays à revenus faibles et intermédiaires, pour le centre-nord du Nigéria. MÉTHODES: Dans cette étude pilote, nous avons effectué un dépistage clinique et échocardiographique sur un échantillon transversal d'élèves du secondaire sélectionnés aléatoirement à Jos, dans le centre-nord du Nigéria, de mars à septembre 2016. Pour la classification des résultats en CR limite ou définitive, nous avons effectué une échocardiographie de confirmation en utilisant les critères de la Fédération Mondiale du Cœur pour les personnes suspectées d'avoir une CR lors du dépistage. RÉSULTATS: Au total, 417 élèves du secondaire ont été dépistés, dont 247 (59,2%) étaient des filles. L'âge médian était de 14 ans (IQR: 13-15). Un dépistage clinique a détecté 8/417 enfants, tandis qu'un dépistage par échocardiographie a détecté 42/417 cas suspects de CR. L'échocardiographie a confirmé une CR définitive chez 9/417, correspondant à une prévalence de 21,6 pour 1000 (IC95%: 6,7 à 36,5). Tous les cas confirmés de CR sauf un (8/9) étaient limites, correspondant à une prévalence de 19,2 pour 1000 (IC95%: 8,3 à 37,5) pour une CR limite et 2,4 pour 1000 (IC95%: 0,1 à 13,3) pour une CR définitive. La CR était plus fréquente chez les garçons et l'auscultation cardiaque a manqué plus de 50% des cas. CONCLUSIONS: Cette étude a montré une prévalence élevée de CR parmi les enfants du secondaire dans le centre-nord du Nigeria avec une forte prédominance de lésions asymptomatiques limites. Un dépistage échocardiographique à plus grande échelle en milieu scolaire utilisant une échocardiographie portable ou manuelle visant à la détection précoce de la CR subclinique devrait être adopté.

Profile of congenital heart disease in infants born following exposure to preeclampsia.

BACKGROUND: Events in pregnancy play an important role in predisposing the newborn to the risk of developing CHD. This study evaluated the association between maternal preeclampsia and her offspring risk of CHD. METHODS: This is a cohort study of 90 sex-matched neonates (45 each born to women with preeclampsia and normal pregnancy) in Jos, Nigeria. Anthropometry was taken shortly after delivery using standard protocols. Echocardiography was performed within 24 hours of life and repeated 7 and 28 days later. SPSS version 25 was used in all analyses. Statistical significance was set at p<0.05. RESULTS: Congenital heart disease (CHD) was observed in 27 (30.0%) of newborns of women with preeclampsia compared with 11 (12.1%) of newborns without preeclampsia (p<0.001) at the end of 7 days and in 19 (21.1%) of newborns of women with preeclampsia and 3 (3.3%) of newborns of women without preeclampsia by the end of the 4th week of life (p<0.001). Overall, ASD (4 newborns), PDA (21 newborns), patent foramen ovale (14 newborns) and VSD (2 newborns) were the prevalent lesions found among all the newborns studied in the first week of life. Isolated atrial and ventricular septal defects were seen in 4 (4.4%) of the newborns of women with preeclampsia. Being the infant of a woman with preeclampsia was associated with about 8-fold increased risk of having CHD (OR = 7.9, 95% CI = 2.5-24.9, p<0.001). CONCLUSION: CHD may be more common in newborns of women with preeclampsia underscoring the need for fetal and newborn screening for CHD in women with preeclampsia so as to improve their infant's well being.

Maternal Instruction About Jaundice and the Incidence of Acute Bilirubin Encephalopathy in Nigeria.

OBJECTIVE: To evaluate whether teaching mothers about neonatal jaundice will decrease the incidence of acute bilirubin encephalopathy among infants admitted for jaundice. STUDY DESIGN: This was a multicenter, before-after and cross-sectional study. Baseline incidences of encephalopathy were obtained at 4 collaborating medical centers between January 2014 and May 2015 (Phase 1). Structured jaundice instruction was then offered (May to November 2015; Phase 2) in antenatal clinics and postpartum. Descriptive statistics and logistic regression models compared 3 groups: 843 Phase 1 controls, 338 Phase 2 infants whose mothers received both antenatal and postnatal instruction (group A), and 215 Phase 2 infants whose mothers received no instruction (group B) either because the program was not offered to them or by choice. RESULTS: Acute bilirubin encephalopathy occurred in 147 of 843 (17%) Phase 1 and 85 of 659 (13%) Phase 2 admissions, which included 63 of 215 (29%) group B and 5 of 338 (1.5%) group A infants. OR for having acute bilirubin encephalopathy, comparing group A and group B infants adjusted for confounding risk factors, was 0.12 (95% CI 0.03-0.60). Delayed care-seeking (defined as an admission total bilirubin ≥18 mg/dL at age ≥48 hours) was the strongest single predictor of acute bilirubin encephalopathy (OR 11.4; 6.6-19.5). Instruction decreased delay from 49% to 17%. Other major risk factors were home births (OR 2.67; 1.69-4.22) and hemolytic disease (hematocrit ≤35% plus bilirubin ≥20 mg/dL) (OR 3.03; 1.77-5.18). The greater rate of acute bilirubin encephalopathy with home vs hospital birth disappeared if mothers received jaundice instruction. CONCLUSIONS: Providing information about jaundice to mothers was associated with a reduction in the incidence of bilirubin encephalopathy per hospital admission.

Pulmonary hypertension and right ventricular function in Nigerian children with sickle cell anaemia.

BACKGROUND: Pulmonary hypertension (PH), a complication of sickle cell anaemia (SCA), results in considerable morbidity. This study aims to determine the prevalence and associations of echocardiography-suggested PH in children with SCA. METHODS: We performed a cross-sectional comparative study involving 100 systematically sampled SCA subjects 3-14 y of age in their steady state with matched haemoglobin AA phenotype controls. Clinical, laboratory and echocardiography data (including tricuspid regurgitation velocity [TRV], mean pulmonary arterial pressure [mPAP] and tricuspid annular plane systolic excursion [TAPSE]) were obtained from all patients. Statistical analyses were performed using SPSS version 22 (IBM, Armonk, NY, USA). A p-value <0.05 was considered statistically significant. RESULTS: Of the 100 SCA subjects studied, 22 (22%) had echocardiographic findings suggestive of PH compared with none in the controls. The median TAPSE was significantly lower in the PH group (2.55 cm [interquartile range {IQR} 2.2-2.8]) compared with the no PH group (2.77 cm [IQR 2.4-3.2]) (p=0.03). No significant correlation existed between mPAP and age, nor any laboratory parameters studied. The odds ratio (OR) suggested PH significantly increased with an increase in the frequency of hospitalizations for vaso-occlusive crises within a 12-month period (OR 15.15 [95% CI 1.57 to 146.35], p=0.02) and a lifetime history of blood transfusion (OR 5.44 [95% CI 1.09 to 27.24], p=0.04). CONCLUSIONS: Echocardiography-suggested PH is common in children with SCA and is associated with poorer right ventricular function, frequent vaso-occlusive crises and blood transfusions.

Discriminative ability of adiposity measures for elevated blood pressure among adolescents in a resource-constrained setting in northeast Nigeria: a cross-sectional analysis.

BACKGROUND: Several studies examining the association and discriminative ability of adiposity measures for prehypertension and hypertension among adolescents have reported varying outcomes. We aimed to determine the discriminative ability of the Body Mass index (BMI), Waist Circumference (WC), and Waist-to-Height Ratio (WHtR) adiposity measures for elevated blood pressure (prehypertension and hypertension combined) among adolescents in Gombe, northeast Nigeria. METHODS: This cross-sectional study used a multi-stage sampling technique and involved 367 secondary school adolescent (10-18 years) boys and girls in Gombe Local Government Area, Gombe State, northeast Nigeria from January to September 2015. We examined and compared the associations and discriminative ability of the BMI, WC and the WHtR for elevated blood pressure using multiple logistic regression and receiver operating characteristics (ROC) curves. Area under the curves (AUC), odds ratio (OR) and 95% confidence intervals (CI) are reported. RESULTS: All three measures of adiposity were strongly and positively associated with elevated blood pressure. The BMI obesity showed the strongest association with elevated blood pressure with odds that was double the odds of WC and triple that of WHtR [adjusted OR for BMI 15.3, 95% CI (4.8-27.9)]. The discriminative ability of adiposity measures for elevated blood pressure using AUC was comparable (0.786 for BMI, vs 0.780 for WC, vs 0.761 for WHtR). CONCLUSION: We provide evidence, here on the BMI, WC and WHtR to support the use of simple indirect measures of adiposity in evaluating adiposity-related risk including prehypertension and hypertension among Nigerian adolescents.

Factors influencing irradiance of locally fabricated phototherapy devices in Jos, north-central Nigeria.

Locally fabricated phototherapy devices (LFPDs) are widely used in Nigeria for the treatment of neonatal jaundice. Ours was a cross-sectional observational study of all LFPDs in major hospitals in Jos between January and March 2015. We evaluated a total of 24 LFPDs. The irradiance at the level of the baby was in the range of 2-23.9 µW/cm2/nm. Fourteen devices had the recommended irradiance of ≥10 µW/cm2/nm and none had irradiance in the intensive range. Decreasing distance from the baby, presence of reflectors and increasing number of flourecent tubes significantly contributed to higher irradiance. A combination of six tubes, presence of reflectors and a distance of 10 cm from the baby produced a mean irradiance of 23.40 µW/cm2/nm. The irradiance of LFPDs varies widely and can be improved by simple modifications.

Congenital Heart Defects in Nigerian Children: Preliminary Data From the National Pediatric Cardiac Registry.

BACKGROUND: Congenital heart defects (CHDs) are common birth defects with significant impact on morbidity and mortality. We aimed to compare regional patterns of CHDs in Nigeria using a registry-based approach. METHODS: Children with echocardiography-confirmed CHDs at 17 medical centers across the country were enrolled in a pilot National Pediatric Cardiac Registry from January to December 2014. RESULTS: A total of 1,296 children (52.9% male; median age 0.9 years) with CHDs were enrolled. Patients enrolled in Northern Nigeria constituted 34.6% of the study population and were older compared to those enrolled from Southern Nigeria (2.9 ± 3.6 vs 2.4 ± 3.5 years; P = .02). Ventricular septal defects were significantly more prevalent in the North (37.4%) compared with the South (18.5%; P < .0001), while severe CHDs were more prevalent in the South ( P = .004). Of the 208 (16.0%) children who received corrective cardiac intervention, only 43 (20.7%) of them had the intervention done in country. More patients in the South received intervention compared to the North (19.02% vs 10.5%; P < .0001). CONCLUSION: This is the first prospective, registry-based, multicenter study of CHDs in Nigerian children. We demonstrate important differences between the Northern and the Southern geographical regions of the country in terms of age at diagnosis, type, and severity of lesion as well as access to cardiac surgery. The findings demonstrate the utility of a national CHDs registry for understanding clinical epidemiology of CHDs in low- and middle-income countries and its potential to serve as a basis for research and planning.

Serum lipid and glucose profiles in HIV-positive Nigerian children.

OBJECTIVES: To describe the fasting serum lipid and glucose profiles of HIV-positive Nigerian children and determine the prevalence and risk factors for dyslipidaemia and hyperglycaemia, which are risk factors for cardiovascular diseases. METHODS: This was a comparative cross-sectional study carried out at the Paediatric Infectious Disease Clinic (PIDC) of the Jos University Teaching Hospital (JUTH) for HIV-positive children and at two primary schools in Jos for HIV-negative children as controls. One hundred and forty-two HIV-positive children aged 6-18 years and an equal number of controls were studied by determining their fasting serum lipid and glucose levels. The prevalence of dyslipidaemia and hyperglycaemia was determined and their risk factors obtained using multivariate logistic regression. P values of less than 0.05 were considered statistically significant. RESULTS: Mean triglyceride levels were significantly higher in HIV-positive children compared with controls at 87.2 mg/dL (95% confidence interval [CI] 79.4-95.0) and 68.1 mg/dL (95% CI 62.5-72.7), respectively (P<0.001). There were no significant differences in mean glucose levels. Dyslipidaemia was significantly higher in HIV-positive children (21.8%) compared with controls (12.7%; P=0.04). Total serum cholesterol was elevated in 17 (12.0%) HIV-positive participants compared with seven (4.9%) of controls (P=0.02). Children on lopinavir/ritonavir (LPV/r) and those with no significant or mild disease had a significantly higher prevalence of hypercholesterolaemia (33.3% vs 4.8% and 14.5% vs 0.0%, respectively; P<0.001). CONCLUSION: HIV-positive children on antiretroviral (ARV) drugs, especially LPV/r, should have their lipids regularly monitored as those with dyslipidaemia stand the risk of subsequently developing cardiovascular diseases.

Status and Challenges of Care in Africa for Adults With Congenital Heart Defects.

The 54 countries in Africa have an estimated total annual congenital heart defect (CHD) birth prevalence of 300,486 cases. More than half (51.4%) of the continental birth prevalence occurs in only seven countries. Congenital heart disease remains primarily a pediatric health issue in Africa because of the deficient health-care systems: the adults with CHD made up just 10% of patients with CHD in Ghana, and 13.7% of patients with CHD presenting for surgery in Mozambique. With Africa's population projected to double in the next 35 years, the already deficient health systems for CHD care will suffer unbearable strain unless determined and courageous action is undertaken by the African leaders.

Audit of availability and distribution of paediatric cardiology services and facilities in Nigeria.

BACKGROUND: Paediatric cardiac services in Nigeria have been perceived to be inadequate but no formal documentation of availability and distribution of facilities and services has been done. OBJECTIVE: To evaluate and document the currently available paediatric cardiac services in Nigeria. METHODS: In this questionnaire-based, cross-sectional descriptive study, an audit was undertaken from January 2010 to December 2014, of the personnel and infrastructure, with their distributions according to geopolitical zones of Nigeria. RESULTS: Forty-eight centres participated in the study, with 33 paediatric cardiologists and 31 cardiac surgeons. Echocardiography, electrocardiography and pulse oximetry were available in 45 (93.8%) centres while paediatric intensive care units were in 23 (47.9%). Open-heart surgery was performed in six (12.5%) centres. South-West zone had the majority of centres (20; 41.7%). CONCLUSIONS: Available paediatric cardiac services in Nigeria are grossly inadequate and poorly distributed. Efforts should be intensified to upgrade existing facilities, establish new and functional centres, and train personnel.

Clinical Outcomes in 3343 Children and Adults With Rheumatic Heart Disease From 14 Low- and Middle-Income Countries: Two-Year Follow-Up of the Global Rheumatic Heart Disease Registry (the REMEDY Study).

BACKGROUND: There are few contemporary data on the mortality and morbidity associated with rheumatic heart disease or information on their predictors. We report the 2-year follow-up of individuals with rheumatic heart disease from 14 low- and middle-income countries in Africa and Asia. METHODS: Between January 2010 and November 2012, we enrolled 3343 patients from 25 centers in 14 countries and followed them for 2 years to assess mortality, congestive heart failure, stroke or transient ischemic attack, recurrent acute rheumatic fever, and infective endocarditis. RESULTS: Vital status at 24 months was known for 2960 (88.5%) patients. Two-thirds were female. Although patients were young (median age, 28 years; interquartile range, 18-40), the 2-year case fatality rate was high (500 deaths, 16.9%). Mortality rate was 116.3/1000 patient-years in the first year and 65.4/1000 patient-years in the second year. Median age at death was 28.7 years. Independent predictors of death were severe valve disease (hazard ratio [HR], 2.36; 95% confidence interval [CI], 1.80-3.11), congestive heart failure (HR, 2.16; 95% CI, 1.70-2.72), New York Heart Association functional class III/IV (HR, 1.67; 95% CI, 1.32-2.10), atrial fibrillation (HR, 1.40; 95% CI, 1.10-1.78), and older age (HR, 1.02; 95% CI, 1.01-1.02 per year increase) at enrollment. Postprimary education (HR, 0.67; 95% CI, 0.54-0.85) and female sex (HR, 0.65; 95% CI, 0.52-0.80) were associated with lower risk of death. Two hundred and four (6.9%) patients had new congestive heart failure (incidence, 38.42/1000 patient-years), 46 (1.6%) had a stroke or transient ischemic attack (8.45/1000 patient-years), 19 (0.6%) had recurrent acute rheumatic fever (3.49/1000 patient-years), and 20 (0.7%) had infective endocarditis (3.65/1000 patient-years). Previous stroke and older age were independent predictors of stroke/transient ischemic attack or systemic embolism. Patients from low- and lower-middle-income countries had significantly higher age- and sex-adjusted mortality than patients from upper-middle-income countries. Valve surgery was significantly more common in upper-middle-income than in lower-middle- or low-income countries. CONCLUSIONS: Patients with clinical rheumatic heart disease have high mortality and morbidity despite being young; those from low- and lower-middle-income countries had a poorer prognosis associated with advanced disease and low education. Programs focused on early detection and the treatment of clinical rheumatic heart disease are required to improve outcomes.

Seven key actions to eradicate rheumatic heart disease in Africa: the Addis Ababa communiqué.

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) remain major causes of heart failure, stroke and death among African women and children, despite being preventable and imminently treatable. From 21 to 22 February 2015, the Social Cluster of the Africa Union Commission (AUC) hosted a consultation with RHD experts convened by the Pan-African Society of Cardiology (PASCAR) in Addis Ababa, Ethiopia, to develop a 'roadmap' of key actions that need to be taken by governments to eliminate ARF and eradicate RHD in Africa. Seven priority areas for action were adopted: (1) create prospective disease registers at sentinel sites in affected countries to measure disease burden and track progress towards the reduction of mortality by 25% by the year 2025, (2) ensure an adequate supply of high-quality benzathine penicillin for the primary and secondary prevention of ARF/RHD, (3) improve access to reproductive health services for women with RHD and other non-communicable diseases (NCD), (4) decentralise technical expertise and technology for diagnosing and managing ARF and RHD (including ultrasound of the heart), (5) establish national and regional centres of excellence for essential cardiac surgery for the treatment of affected patients and training of cardiovascular practitioners of the future, (6) initiate national multi-sectoral RHD programmes within NCD control programmes of affected countries, and (7) foster international partnerships with multinational organisations for resource mobilisation, monitoring and evaluation of the programme to end RHD in Africa. This Addis Ababa communiqué has since been endorsed by African Union heads of state, and plans are underway to implement the roadmap in order to end ARF and RHD in Africa in our lifetime.

Characteristics, complications, and gaps in evidence-based interventions in rheumatic heart disease: the Global Rheumatic Heart Disease Registry (the REMEDY study).

AIMS: Rheumatic heart disease (RHD) accounts for over a million premature deaths annually; however, there is little contemporary information on presentation, complications, and treatment. METHODS AND RESULTS: This prospective registry enrolled 3343 patients (median age 28 years, 66.2% female) presenting with RHD at 25 hospitals in 12 African countries, India, and Yemen between January 2010 and November 2012. The majority (63.9%) had moderate-to-severe multivalvular disease complicated by congestive heart failure (33.4%), pulmonary hypertension (28.8%), atrial fibrillation (AF) (21.8%), stroke (7.1%), infective endocarditis (4%), and major bleeding (2.7%). One-quarter of adults and 5.3% of children had decreased left ventricular (LV) systolic function; 23% of adults and 14.1% of children had dilated LVs. Fifty-five percent (n = 1761) of patients were on secondary antibiotic prophylaxis. Oral anti-coagulants were prescribed in 69.5% (n = 946) of patients with mechanical valves (n = 501), AF (n = 397), and high-risk mitral stenosis in sinus rhythm (n = 48). However, only 28.3% (n = 269) had a therapeutic international normalized ratio. Among 1825 women of childbearing age (12-51 years), only 3.6% (n = 65) were on contraception. The utilization of valvuloplasty and valve surgery was higher in upper-middle compared with lower-income countries. CONCLUSION: Rheumatic heart disease patients were young, predominantly female, and had high prevalence of major cardiovascular complications. There is suboptimal utilization of secondary antibiotic prophylaxis, oral anti-coagulation, and contraception, and variations in the use of percutaneous and surgical interventions by country income level.

Childhood acquired heart diseases in Jos, north central Nigeria.

BACKGROUND: The patterns of childhood acquired heart diseases (AHD) vary in different parts of the world and may evolve over time. We aimed to compare the pattern of childhood AHD in our institution to the historical and contemporary patterns in other parts of the country, and to highlight possible regional differences and changes in trend. MATERIALS AND METHODS: Pediatric echocardiography records spanning a period of 10 years were reviewed. Echocardiography records of children with echocardiographic or irrefutable clinical diagnoses of AHD were identified and relevant data extracted from their records. RESULTS: One hundred and seventy five children were diagnosed with AHD during the period, including seven that had coexisting congenital heart disease (CHD). They were aged 4 weeks to 18 years (mean 9.84΁4.5 years) and comprised 80 (45.7%) males and 95 (54.3%) females. Rheumatic heart disease (RHD) was the cause of the AHD in 101 (58.0%) children, followed by dilated cardiomyopathy (33 cases, 18.9%) which was the most frequent AHD in younger (under 5 years) children. Other AHD encountered were cor pulmonale in 16 (9.1%), pericardial disease in 15 (8.6%), infective endocarditis in 8 (4.6%) and aortic aneurysms in 2 (1.1%) children. Only one case each of endomyocardial fibrosis (EMF) and Kawasaki Disease were seen during the period. CONCLUSIONS: The majority of childhood acquired heart diseases in our environment are still of infectious aeitology, with RHD remaining the most frequent, particularly in older children. Community-based screening and multicenter collaborative studies will help to better describe the pattern of AHD in our country. More vigorous pursuit of the Millennium development goals will contribute to reducing the burden of childhood acquired heart diseases in the country.