Right-sided Weakness in a Rwandan Patient with Unrepaired Tetralogy of Fallot


Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease encountered in pediatrics. Long-term survival after surgical repair has improved; however, reported mortality rates in unrepaired TOF are significant. Associated complications include neurological sequelae, most frequently brain abscess and stroke. In countries without early intervention for congenital heart disease including TOF, delayed presentations and complications require recognition by healthcare workers.

Case presentation

A 22 year old male with a history of unrepaired TOF presented to Rwanda’s tertiary university hospital, University Teaching Hospital of Kigali (CHUK) with acute right-sided hemiparesis. Diagnostic imaging identified a left-sided brain lesion consistent with brain abscess and cardiac mass concerning for an endocardial vegetation. He was managed with intravenous antibiotics, but subsequently died due to complications of septicemia.


In countries where surgical repair of TOF is not available, early recognition and medical management are key in temporizing the development of devastating sequelae. Describing the prevalence of CHD in Rwanda is urgent, requiring further research by which effective prevention and treatment strategies can be developed.

Management of Congenital Heart Disease in Low-Income Countries: The Challenges and the Way Forward

In this article, we will discuss the management of congenital heart disease in low-income and low-middle income countries. First, we will review the epidemiology of congenital heart disease in the low-income and low-middle income countries and compare it to that in the high-income countries; cardiac disease is the commonest cause of death globally. The challenges that are facing the delivery of pediatric cardiac services will be discussed and some solutions will be suggested to improve these services. Pediatric cardiac services face huge economic, financial, social, and health care system delivery challenges. Collaboration between countries and non-governmental and philanthropy organizations is strongly needed to improve delivery of pediatric cardiac services in low-income and low-middle income countries. Planning of pediatric cardiac services in these countries should consider the context of each country or region; some countries managed to transform their pediatric cardiac services to be better.

Counselling and pregnancy outcomes in women with congenital heart disease- current status and gap analysis from Madras Medical College Pregnancy And Cardiac disease (M-PAC) registry

Congenital heart disease (CHD) is becoming an increasingly important cause of heart disease in pregnancy in low- and middle – income countries (LMICs). Preconception and contraception counselling based on risk stratification has the potential to reduce maternal complications. Data is lacking from LMICs on the availability and effectiveness of preconception counselling (PCC) in women with CHD (WWCHD).

Madras Medical College Pregnancy and Cardiac disease (M-PAC) Registry is a single center prospective observational registry conducted at a tertiary referral institution in South India from July 2016 to December 2019. Baseline features and feto-maternal outcomes were compared in WWCHD with and without PCC. Predictors of post-delivery contraception were identified.

Of the 107 eligible pregnancies with data on counselling, only 49.5% had received PCC. Pregnancies involving women with corrected CHDs (62.3% vs 33.3%; P ​= ​0.006) and cyanotic CHD (20.8% vs 11.1%; P ​= ​0.042) were more likely to get PCC. High risk mWHO categories were non-significantly less likely to get PCC (32% vs 39%). Primary outcome of death or heart failure was non-significantly low in the PCC group (3.8% vs 7.4% P ​= ​0.4). Patients with high risk m WHO categories were less likely to get Tier I contraceptives post-delivery (46% vs 79.7% P ​= ​0.004).

Preconception and post conception counselling, which have the potential to improve outcome in WWCHD, are being underused in LMICs. Health care systems should ensure multidisciplinary pregnancy and heart team approach to offer timely lesion specific pre-conceptional counselling, shared decision making and appropriate peri-pregnancy care for WWCHD.

Survival of Left-to-Right Shunt Repair in Children with Pulmonary Arterial Hypertension at a Tertiary Hospital in a Low-to-Middle-Income Country

Background: In low-to-middle-income countries, repair of the left-to-right shunts congenital heart disease (CHD) are often done with existing pulmonary arterial hypertension (PAH). Long-term outcomes data of this condition in either low-to-middle or high-income countries are limited. We conducted a study to evaluate the outcomes of children with PAH related to left-to-right shunt CHD who underwent surgical or transcatheter repair.

Methods: All cases of PAH related to left-to-right shunt CHD repairs from 2015–2018 were retrospectively reviewed with additional new patients who underwent repair within our study period (2019–2020). Cases with complex congenital heart disease and incomplete hemodynamic echocardiography or catheterization measurements were excluded. Kaplan-Meier curves, log-rank test, Cox regression with Firth’s correction and restricted mean survival time were used for survival analysis.

Results: Of the 118 patients, 103 patients were enrolled and 15 patients were excluded due to complex congenital heart disease and missing hemodynamic measurements prior to repair. Overall, median age at intervention was 44 months, mPAP mean was 43.17 ± 16.05 mmHg and Pulmonary Vascular Resistance index (PVRi) mean was 2.84 ± 2.09 (WU.m2). Nine patients died after repair. The survival rate for patients with PAH-CHD at 1 day, 30 days and 1400 days (4 years) was 96.1%, 92.1%, and 91.0% respectively. Patients with persisting PAH after correction had –476.1 days (95% confidence interval [CI]: –714.4, –237,8) shorter survival over 4 years of follow up compared to patients with reversed PAH. PVRi was found to be the influencing covariate of the difference of restricted mean survival time between these groups.

Conclusion: In low-to-middle income settings, with accurate PAH reversibility assessment prior to intervention, repair of left-to-right shunt CHD with existing PAH in children has a favourable outcome. Inferior survival is found in patients with persistence of PAH. PVRi at baseline predicts between-group survival difference.

How do caregivers of children with congenital heart diseases access and navigate the healthcare system in Ethiopia?

Surgery can correct congenital heart defects, but disease management in low- and middle-income countries can be challenging and complex due to a lack of referral system, financial resources, human resources, and infrastructure for surgical and post-operative care. This study investigates the experiences of caregivers of children with CHD accessing the health care system and pediatric cardiac surgery.

A qualitative study was conducted at a teaching hospital in Ethiopia. We conducted semi-structured interviews with 13 caregivers of 10 patients with CHD who underwent cardiac surgery. We additionally conducted chart reviews for triangulation and verification. Interviews were conducted in Amharic and then translated into English. Data were analyzed according to the principles of interpretive thematic analysis, informed by the candidacy framework.

The following four observations emerged from the interviews: (a) most patients were diagnosed with CHD at birth if they were born at a health care facility, but for those born at home, CHD was discovered much later (b) many patients experienced misdiagnoses before seeking care at a large hospital, (c) after diagnosis, patients were waiting for the surgery for more than a year, (d) caregivers felt anxious and optimistic once they were able to schedule the surgical date. During the care-seeking journey, caregivers encountered financial constraints, struggled in a fragmented delivery system, and experienced poor service quality.

Delayed access to care was largely due to the lack of early CHD recognition and financial hardships, related to the inefficient and disorganized health care system. Fee waivers were available to assist low-income children in gaining access to health services or medications, but application information was not readily available. Indirect costs like long-distance travel contributed to this challenge. Overall, improvements must be made for district-level screening and the health care workforce.

Global prevalence of congenital heart disease in school-age children: a meta-analysis and systematic review

Congenital heart disease (CHD) is the commonest birth defect. Studies estimating the prevalence of CHD in school-age children could therefore contribute to quantifying unmet health needs for diagnosis and treatment, particularly in lower-income countries. Data at school age are considerably sparser, and individual studies have generally been of small size. We conducted a literature-based meta-analysis to investigate global trends over a 40-year period.

Methods and results
Studies reporting on CHD prevalence in school-age children (4–18 years old) from 1970 to 2017 were identified from PubMed, EMBASE, Web of Science and Google Scholar. According to the inclusion criteria, 42 studies including 2,638,475 children, reporting the prevalence of unrepaired CHDs (both pre-school diagnoses and first-time school-age diagnoses), and nine studies including 395,571 children, specifically reporting the prevalence of CHD first diagnosed at school ages, were included. Data were combined using random-effects models. The prevalence of unrepaired CHD in school children during the entire period of study was 3.809 (95% confidence intervals 3.075–4.621)/1000. A lower proportion of male than female school children had unrepaired CHD (OR = 0.84 [95% CI 0.74–0.95]; p = 0.001). Between 1970–1974 and 1995–1999, there was no significant change in the prevalence of unrepaired CHD at school age; subsequently there was an approximately 2.5-fold increase from 1.985 (95% CI 1.074–3.173)/1000 in 1995–1999 to 4.832 (95% CI 3.425–6.480)/1000 in 2010–2014, (p = 0.009). Among all CHD conditions, atrial septal defects and ventricular septal defects chiefly accounted for this increasing trend. The summarised prevalence (1970–2017) of CHD diagnoses first made in childhood was 1.384 (0.955, 1.891)/1000; during this time there was a fall from 2.050 [1.362, 2.877]/1000 pre-1995 to 0.848 [0.626, 1.104]/1000 in 1995–2014 (p = 0.04).

Globally, these data show an increased prevalence of CHD (mainly mild CHD conditions) recognised at birth/infancy or early childhood, but remaining unrepaired at school-age. In parallel there has been a decrease of first-time CHD diagnoses in school-age children. These together imply a favourable shift of CHD recognition time to earlier in the life course. Despite this, substantial inequalities between higher and lower income countries remain. Increased healthcare resources for people born with CHD, particularly in poorer countries, are required.

Considerations for Newborn Screening for Critical Congenital Heart Disease in Low- and Middle-Income Countries

We propose several considerations for implementation of critical congenital heart disease (CCHD) screening for low- and middle-income countries to assess health system readiness for countries that may not have all the downstream capacity needed for treatment of CCHD. The recommendations include: (1) assessment of secondary and tertiary level CHD health services, (2) assessment of birth delivery center processes and staff training needs, (3) data collection on implementation and quality surgical outcomes, (4) budgetary consideration, and (5) consideration of the CCHD screening service as part of the overall patient care continuum.

Incidence and Mortality Trend of Congenital Heart Disease at the Global, Regional, and National Level, 1990-2017

Congenital heart disease (CHD) is the most commonly diagnosed congenital disorder in newborns. The incidence and mortality of CHD vary worldwide. A detailed understanding of the global, regional, and national distribution of CHD is critical for CHD prevention.We collected the incidence and mortality data of CHD from the Global Burden of Disease study 2017 database. Average annual percentage change was applied to quantify the temporal trends of CHD incidence and mortality at the global, regional, and national level, 1990-2017. A sociodemographic index (SDI) was created for each location based on income per capita, educational attainment, and fertility.The incidence of CHD was relatively high in developing countries located in Africa and Asia, while low in most developed countries. Between 1990 and 2017, the CHD incidence rate remained stable at the global level, whereas increased in certain developed countries, such as Germany and France. The age-standardized mortality rate of CHD declined substantially over the last 3 decades, regardless of sex, age, and SDI region. The decline was more prominent in developed countries. We also detected a significant positive correlation between CHD incidence and CHD mortality in both 1990 and 2017, by SDI.The incidence of CHD remained stable over the last 3 decades, suggesting little improvement in CHD prevention strategies and highlighting the importance of etiological studies. The mortality of CHD decreased worldwide, albeit the greatly geographical heterogeneity. Developing countries located in Africa and Asia deserve more attention and priority in the global CHD prevention program.

Global Unmet Needs in Cardiac Surgery.

More than 6 billion people live outside industrialized countries and have insufficient access to cardiac surgery. Given the recently confirmed high prevailing mortality for rheumatic heart disease in many of these countries together with increasing numbers of patients needing interventions for lifestyle diseases due to an accelerating epidemiological transition, a significant need for cardiac surgery could be assumed. Yet, need estimates were largely based on extrapolated screening studies while true service levels remained unknown. A multi-author effort representing 16 high-, middle-, and low-income countries was undertaken to narrow the need assessment for cardiac surgery including rheumatic and lifestyle cardiac diseases as well as congenital heart disease on the basis of existing data deduction. Actual levels of cardiac surgery were determined in each of these countries on the basis of questionnaires, national databases, or annual reports of national societies. Need estimates range from 200 operations per million in low-income countries that are nonendemic for rheumatic heart disease to >1,000 operations per million in high-income countries representing the end of the epidemiological transition. Actually provided levels of cardiac surgery range from 0.5 per million in the assessed low- and lower-middle income countries (average 107 ± 113 per million; representing a population of 1.6 billion) to 500 in the upper-middle-income countries (average 270 ± 163 per million representing a population of 1.9 billion). By combining need estimates with the assessment of de facto provided levels of cardiac surgery, it emerged that a significant degree of underdelivery of often lifesaving open heart surgery does not only prevail in low-income countries but is also disturbingly high in middle-income countries.