Abstract
Background
Congenital heart disease (CHD) constitutes an important cause of mortality and morbidity in children. In limited resource settings, data regarding patterns and outcomes of CHD are deficient.
Objectives
To describe the clinical and echocardiographic (echo) features as well as outcomes of patients with CHD seen at Sudan Heart Center.
Methods
This is a study reporting results of the Sudan C ongenital He art Dis e ase R egistry ( Sudan CHEER ) from January 2021–September 2022. Clinical, echo, and interventional management data were collected from hospital records as well as through telephone calls.
Results
975 patients with CHD were included (males 53 %). The most common age group was 1–12 months (40 %), and only 7 % were neonates. Acyanotic CHD constituted 71 % of patients. Eisenmenger’s syndrome was present in 37 patients (3.8 %). Surgery was indicated in 472 patients (48 %) and performed in 167 (35 % of those in need). The Risk Adjustment Scale for CHD surgery was 2 for 70 % of cases. Interventional cardiac catheterization was indicated in 226 patients and done in 70 % of these. Only 56 % of patients were accessible for follow up period, mean of 12 months. Of unoperated patients, 25 died (6 %), mostly (43 %) having left to right shunts. The surgical operative mortality was 12 %; 80 % of those who underwent surgery did not have residual lesions. Interventional catheterization mortality was 0.6 %, and all survivors were well on follow up. Of those who had palliative interventional procedures, 70 % are still awaiting corrective surgery.
Conclusion
There are huge gaps in early diagnosis and access to interventions that need to be addressed to improve outcomes of patients with CHD in Sudan and similarly low-income countries.
Highlights
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In Sudan, congenital heart disease (CHD) presents late and only 6% of patients were diagnosed in the neonatal period.
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Surgical and trans catheter Interventions were performed in only 46% of patients in need.
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Of unoperated patients, 6% died in the follow up period of 12 months, mostly with left to right shunts.
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Only 56% of patients were accessible for follow up, of those who need a second operation, only 30% had access to surgery.
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There is a huge gap in early diagnosis and access to interventions that needs to be addressed to improve management of CHD.
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Introduction
Congenital heart diseases (CHD) are the most common congenital anomalies, with a prevalence of about 1 per 100 live births [ ]. As many as 50 % of these defects need some intervention, medical, surgical, or cardiac catheterization (cath) that can be lifesaving. The prevalence of CHD in limited resource countries is poorly documented due to poor access to medical care, leading to under diagnosis, particularly in neonates who might die with undiagnosed severe CHD. It is estimated that the number of children born with CHD in sub-Saharan Africa is nearly 3-fold more compared to that reported in the United States of America [ ]. Unfortunately, over 90 % of these children do not have access to cardiac care, and of those who need cardiac surgery, only 3 % receive it [ , ]. These findings indicate that the rest of these children either die or develop severe complications. It was found that the rank of CHD in the leading causes of disability–adjusted-life-years rose from 15th to 9th in countries with low socio demographic indices while it fell from second to fourth rank in high income counties emphasizing the poor access to cardiac care [ ]. Pediatric Cardiology (PC) programs require complex and expensive infrastructure, including trained personnel, diagnostic and interventional facilities including imaging, and surgical and cardiac cath equipment. Establishing and maintaining such services are challenging in resource limited settings due to the competing priorities of primary care services [ ].
A PC program was established in Sudan in 2002 that included basic diagnostic and interventional facilities [ ]. In 2021, a register for CHD ( Sudan C ongenital He art Dis e ase R egistry- Sudan CHEER ) was initiated, aiming at documentation of the clinical and echocardiographic (echo) patterns as well as the outcomes of children with heart disease. Such data are needed to evaluate the efficacy of the current service as well as developing and expanding PC facilities in order to meet the growing needs in the country.
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Methods
Sudan C ongenital He art Dis e ase R egistry ( Sudan CHEER ) is a database system initiated at Sudan Heart Center– Khartoum, Sudan, in 2021. This is one of 2 pediatric cardiac referral centers in Khartoum with facilities for outpatient, inpatient, cardiac cath, and cardiac surgery for CHD for pediatric and adult patients. This study is a pilot report of all patients with CHD seen in the period from January 1/2021 to September 30/2022. We excluded those with acquired heart disease, cardiomyopathies, pericardial disease, primary arrhythmias, and other non-congenital heart diseases. Patients’ data was obtained both retrospectively and prospectively from outpatient and inpatient departments using an electronic data sheet that includes demographic, clinical and echo findings at the first visit as well as follow up data on clinical and cardiac intervention outcomes. The International Coding and Nomenclature for Congenital Heart Disease was used to describe echo findings. [ ] Follow up data was obtained from hospital visits and telephone calls conducted by the research team and included symptoms, complications, type of intervention, and the need for further interventions. Data was analyzed using appropriate statistical methods.
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Ethical considerations
Ethical approval for the study was obtained from the Sudan Medical Specialization Board Ethics Committee. Informed written consent was obtained from patients seen at the center, and verbal consent was obtained from patients who were contacted by telephone. Approval to conduct the study was obtained from the Sudan Heart Center administration.
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Results
The study included 975 patients with CHD; males comprised 53 %. The most common age range of presentation was 1–12 months (40 %), with a mean age of 8 months. as shown in Fig. 1 .
The types and frequency of CHD are shown in Table 1 . Acyanotic CHD constituted 71 %, and cyanotic CHD 29 % of cases.
| Type of CHD | Number (%) |
|---|---|
| Acyanotic CHD | (71) |
| VSD | 289 (29) |
| ASD | 91 (9) |
| PDA | 86 (9) |
| AVSD | 81 (8) |
| Mitral Valve Disease | 54 (5) |
| Pulmonary valve stenosis | 41 (4) |
| Aortic Valve / Sub valve stenosis | 21 (2) |
| Coarctation of the aorta | 13 (1) |
| Cyanotic CHD | (29) |
| TOF | 120 (12) |
| TOF with Absent Pulmonary Valve | 4 |
| TGA | 28 (3) |
| TGA VSD | 10 |
| TGA Intact ventricular septum | 7 |
| TGA/VSD/ Pulmonary stenosis | 11 |
| Univentricular Hearts: | 54 (5) |
| Double inlet ventricle | 16 |
| Tricuspid atresia/hypoplasia | 10 |
| Hypoplastic left heart | 5 |
| Unbalanced AVSD | 23 |
| Pulmonary Atresia | 26 (2.5) |
| With VSD | 19 |
| With intact ventricular septum | 7 |
| Truncus arteriosus | 21 (2) |
| Anomalous Pulmonary Venous Drainage: | 16 (1.6) |
| Supracardiac Total Type | 3 |
| Cardiac Total Type | 4 |
| Mixed Total Type | 3 |
| Partial Type | 6 |
| Other CHD | |
| DORV: | 37 (3.7) |
| Sub aortic VSD / Pulmonary stenosis | 10 |
| Sub pulmonic VSD | 9 |
| Sub aortic VSD | 5 |
| Doubly committed VSD | 2 |
| Non-committed VSD | 11 |
| Isomeric hearts | 35 (3.5) |
| Left Atrial Isomerism | 25 |
| Right Atrial Isomerism | 10 |
| Congenitally Corrected TGA | 8 (1.8) |
| Ebstein anomaly | 2 (0.3) |
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