E-ISSN 1858-8360 | ISSN 0256-4408

Original Article 


2021; Vol 21, Issue No. 2


Clinical and echocardiographic patterns and outcomes of Sudanese children with cardiomyopathy

Amna Mamoun (1), Sulafa Ali (2,3)

(1) Department of Pediatric Cardiology, Pediatric Cardiology Fellow, Sudan Medical Specialization Board, Khartoum, Sudan

(2) Department of Pediatric Cardiology, Consultant Pediatric Cardiologist, Gaafar Ibnauf Children’s Hospital and Sudan Heart Center, Khartoum, Sudan

(3) Department of Paediatrics and Child Health, Faculty of Medicine, University of Khartoum, Khartoum, Sudan

Correspondence to:

Amna Mamoun

Pediatric Cardiology Fellow, Sudan Medical Specialization Board, Khartoum, Sudan

Email: amnamamoon [at] gmail.com

Received: 24 October 2020 | Accepted: 28 May 2021

How to cite this article:

Mamoun A, Ali S. Clinical and echocardiographic patterns and outcomes of Sudanese children with cardiomyopathy. Sudan J Paediatr. 2021;21(2):131–136.



Cardiomyopathy (CMP) constitutes a group of diseases of heart muscle that lead to significant mortality and morbidity, with limited data in Africa. This study aims to describe clinical and echocardiographic (echo) patterns and short-term outcomes of Pediatric CMP. The study was carried at Gaafar Ibnauf Children’s Hospital and included all children 0-18 years with an echo diagnosis of CMP. A retrospective part of the study was carried from January 2013 to 2019 and a prospective part from January to June 2019. Clinical and echo data were collected on presentation and on at least one follow up. A total of 146 patients were diagnosed with CMP during the study period. Most patients (48%) presented at 1-6 years of age, neonatal CMP was present in 4%. Familial incidence was detected in 11% of cases. Heart failure was the most common mode of presentation in 96% of patients; 65% needed hospitalisation; a third of them needed intensive care unit admission. The most common type of CMP was the dilated type (67%). Others included noncompaction CMP (19%), restrictive CMP and hypertrophic CMP (each of them in 6% of patients). Hospital mortality was 20% for the whole cohort and 100% for neonates. Other complications occurred in 21% of patients including arrhythmias, cerebrovascular accidents and end stage renal disease. Echo follow up showed that most patients (52%) remained the same, 26% worsened and 21% improved. Genetic and metabolic studies that could help to improve management and outcomes of CMP are needed.


Pediatric cardiomyopathy; Echocardiography; Outcome; Sudan.


Cardiomyopathy (CMP) constitutes a group of diseases of heart muscle affecting at least 100,000 children worldwide. They are heterogeneous and are associated with mechanical and/or electrical dysfunction, which usually exhibits abnormal ventricular hypertrophy or dilatation, due to a variety of aetiologies that frequently are genetic [1].

Cardiomyopathies are generally classified into primary and secondary, the most commonly used classification for primary CMP includes dilated CMP (DCM), hypertrophic CMP (HCM), restrictive CMP (RCM) and unclassified CMP that includes rarer types such as noncompaction CMP (NCCM) [2,3].

In Africa, CMP is among the major causes of cardiac morbidity and mortality, the commonest types in African patients are DCM and endomyocardial fibrosis (EMF) which is a tropical RCM linked to helminthic infestations [4].

In Sudan, CMP was reported to be responsible for 6% of hospital admissions to the main tertiary care hospital in Khartoum in the era of 1980s [5]. More recent studies found that CMP constituted up to 12% of hospital admissions to the same centre, probably indicating better availability of cardiac services, particularly cardiac imaging [6].

Data about paediatric CMP in Africa is scarce, mostly confined to case reports and small series [7]. Few studies from large series of children with heart disease from Sudan and Tanzania reported CMP to represent 7% of echocardiographic (echo) diagnoses at referral centres [8,9].

In this study, we report the initial clinical and echo patterns as well as the short-term outcomes of paediatric CMP from a registry that was established in 2018.


Sudan CMP Registry (SudaCaMP) was established in 2018 at three centres: Sudan Heart Center, Gaafar Ibnauf Children’s Hospital (GICH) and Zeinab Pediatric Cardiology Clinic, in Khartoum, Sudan. The current study is concerned with clinical and echo data from one centre (GICH). It includes a retrospective part from January 2013 to 2019 where hospital records were searched for all patients with echo diagnosis of CMP who have a contact telephone number. The second part is a prospective one from January to June 2019.

All children 0-18 years of age diagnosed as CMP by paediatric cardiologist seen at GICH were included. Primary CMP was defined as ‘primary disease of heart muscle in the absence of valvular, ischemic or congenital heart disease’. Patients were evaluated clinically (history and examination) and by chest X-ray and electrocardiogram (ECG). A complete echo study was performed using Esaote MyLab machine. In addition, blood samples were taken for thyroid function test (TFT), serum calcium and human immunodeficiency virus (HIV) screening.

Patients were followed up prospectively by clinical and echo examinations for at least 3 months using outpatient and inpatient records. Patients who did not show for follow up were called by telephone and invited to come for clinical and echo examinations.


A total of 146 patients were diagnosed with CMP during the study period. They represent 9% of total admissions to the cardiology unit (1,560 patients during the study period). Thirteen patients (9%) developed their symptoms before their first birthday, 71 (48%) developed their symptoms at the age of 1-6 years and 62 (42%) presented after 6 years of age. The male-to-female ratio was 1:1.3.

Clinical presentation

Most patients (96%) were symptomatic with heart failure at the time of presentation. Two patients with NCCM presented with cerebrovascular accident (CVA).

There were six patients (4%) who presented in neonatal period, one patient had HCM and five had DCM, all of them died during admission. Fifty patients (34%) are outcomes of consanguineous marriages. Seventeen patients (11%) had positive family history of CMP; 5 out of them have more than one family member affected.

Five patients had genetic syndromes including two with mucopolysaccharidosis (both had DCM), one had Noonan’s syndrome with Hypertrophic obstructive cardiomyopathy (HOCM), one had trisomy 21 and HCM and one with Duchene muscular dystrophy and DCM.

ECG was available for review in 45 patients, 18 of them (40%) showed arrhythmias; the commonest was supraventricular tachycardia, 2 patients had bradycardia 1 of them (with HCM) needed pacemaker insertion, 16 patients had ventricular hypotrophy and 1 had low voltage ECG due to pericardial effusion.

Types of CMP and echo findings

Table 1 shows the types of CMP in 146 patients; 142 had primary and 4 had secondary CMP. Causes of secondary CMP (all DCM) include arrhythmia-induced CMP, sickle cell disease, systemic lupus erythematosus and diphtheria (one patient each). Echo detected a thrombus in 16 patients (10.4%), 11 of them already had a CVA. Ejection fraction was low in 120 (82%), and was normal in 26 patients.

Table 1. Types of CMP seen in 146 patients.

Type of CMP Frequency (%)
Post myocarditis
98 (67%)
14/98 (14%)
NCCM (Figure 1) 28 (19%)
HCM 9 (6%)
Combined CMP 2 (1%)
RCM (Figure 2) 9 (6%)

Figure 1. Echo apical four chamber view (enlarged) showing a large thrombus (arrow) at the left ventricle apex in a patient with NCCM.

Other investigations

HIV screening was done for 10 patients (2 were positive) and TFT was done for 43 patients, it was normal in 34, showed hyperthyroidism in 6 and hypothyroidism in 3 patients, none had clinical signs of thyroid dysfunction. Serum calcium was done for 90 patients; hypocalcaemia was detected in 2 of them.


Most patients were on furosemide (151, 96%) and angiotensin converting enzyme inhibitors (122, 78%), 37 patients (23%) were on beta blockers (carvedilol) and 30 (19%) on digoxin. In addition, 20 patients (12%) were treated empirically with L-Carnitine, none of them improved and the drug was discontinued after 2 months. Thirteen percent of patients were not compliant with medications.

Clinical and echo outcomes

Of the 146 patients, 142 had a follow up clinical and echo examination. The mean duration of follow up was 8 months (range 3-18 months). Twenty-nine patients (20%) passed away during their hospital stay. Tables 2 and 3 show outcomes and complications.

Figure 2. Echo four chamber view showing a large thrombus (arrows) in a hugely dilated right atrium in a patient with RCM.

Table 2. Clinical and echo outcomes of 142 patients with CMP.

Outcome Clinical (%) Echo (%)
Improved 28 (20%) 30 (21%)
Remained the same 76 (54%) 74 (52%)
Worsened 38 (27%) 38 (27%)

Table 3. Complications seen on follow up of patients with primary CMP.

Complication Frequency (%)
Intensive care unit admission
95/146 (65%)
32/95 (34%)
31/95 (33%)
Arrhythmias 18/45 (40%)
CVAs 11/142 (8%)
End stage renal disease 2/142 (1%)
(% out of type)
29/146 (20%)
21/ 98 (21%)
4/28 (14%)
3/9 (33%)
2/9 (22%)


This study is the largest, to the authors’ knowledge, to describe the clinical and echo features of children with CMP and their outcomes in Africa. The frequency of CMP in paediatric cardiac wards is similar to previously published literature [6]. There is a significant number of patients needing frequent and intensive care unit admissions, and this has important implications on the limited health services in low-resource settings. Utilising high resources, CMP has a high mortality rate (20%), challenging the prioritisation of intensive care admission selection when facing other treatable medical conditions.

Neonatal CMP is rare and can often be identified in the foetus; its prognosis is poor as has been documented in our patients who had a mortality rate of 100% [10].

Studies from the United States of America had shown that there is a male dominance in the incidence of CMP [11]; however, in the current study, as well as our previous report of NCCM, we demonstrated that females are more affected in contrast to western literature [12]. Genetic studies are needed to investigate families with history of CMP and identify possible mutations that could be distinct from those in other parts of the world.

Similar to other studies in children and adults, DCM is the commonest type of CMP in our patients [9,12]. NCCM is relatively common in our series representing 18% of cases; this is higher than that reported in the Australian CMP registry which was 9% [12]. We reported previously a large series of NCCM; the disease can be overlooked by echo and needs to be carefully looked for especially in familial CMP [13]. NCCM is associated with a higher rate of stroke, this has been reported before and can be related to the morphology of NCCM which is characterised by myocardial trabeculations and deep inter trabecular recesses. In addition, the ejection fraction can improve in patients with NCCM leading to systemic embolisation [14].

Although EMF has been reported to represent about 18% of paediatric CMP in the same institution in 2008 [15], we only encountered one case in the current study, which may indicate a decreasing incidence of this tropical CMP. Endemic infectious disease such as tuberculosis and diphtheria that are prevalent in resource-limited countries can lead to secondary CMP as had been reported in adult African patients [16]. Therefore, efforts should be directed to control such diseases in order to prevent this devastating complication.

Follow-up duration in this study is short and showed that the majority of patients remained the same. The mortality rate of 20% in a short duration of follow up in this study is relatively high compared with a similar mortality rate in a cohort of children followed for 36 months [17]. The mortality is reported to increase to 36% in a 2-year time [18]. This could be related to the facilities for intensive care management including cardiac replacement therapy. In our patients, HCM was associated with a relatively higher mortality, similar to other studies that reported a mortality of 5% per year, even with surgical treatment [19]. Cardiac assist devices and resynchronisation therapy are promising treatments for end stage heart failure. Although there had been reports of their use in children, however, the cost and technical demands as well as the high morbidity associated with these devices prohibits their use in limited resource countries [20]. Heart transplant is currently the optimal treatment for DCM and RCM in children, with a 5 year and 15-year survival of 92% and 53%, respectively. However, unavailability of donors and need for lifelong immunosuppressive therapy are limiting factors [21].

In conclusion, we described the clinical and echo features of a large cohort of children with CMP and revealed that the commonest type is DCM followed by NCCM, and demonstrated a high burden on hospital resources and a high mortality. More studies are needed to delineate genetic and metabolic features of African children with CMP. Limitations of the study are the lack of genetic and metabolic workup which was not done for our patients due to technical and financial constraints.


The authors are grateful to the paediatric cardiology fellows and residents for contribution to data collection.




The authors declare that there is no conflict of interest regarding the publication of this article.


Ethics Committee approval was obtained from Sudan Medical Specialisation Board and permission to conduct the study was taken from the hospital administration. Study data were used for the research purposes only and privacy issues were meticulously considered. Written informed consent for the prospective part was obtained from children’s care givers. Participants’ consent is not required according to the approved research guidelines of the committees for the retrospective (record-based) study, and confidentiality was ensured at all levels.


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How to Cite this Article
Pubmed Style

Mamoun A, Ali S. Clinical and echocardiographic patterns and outcomes of Sudanese children with cardiomyopathy. Sudan J Paed. 2021; 21(2): 131-136. doi:10.24911/SJP.106-1600099020

Web Style

Mamoun A, Ali S. Clinical and echocardiographic patterns and outcomes of Sudanese children with cardiomyopathy. https://www.sudanjp.com/?mno=134980 [Access: December 04, 2022]. doi:10.24911/SJP.106-1600099020

AMA (American Medical Association) Style

Mamoun A, Ali S. Clinical and echocardiographic patterns and outcomes of Sudanese children with cardiomyopathy. Sudan J Paed. 2021; 21(2): 131-136. doi:10.24911/SJP.106-1600099020

Vancouver/ICMJE Style

Mamoun A, Ali S. Clinical and echocardiographic patterns and outcomes of Sudanese children with cardiomyopathy. Sudan J Paed. (2021), [cited December 04, 2022]; 21(2): 131-136. doi:10.24911/SJP.106-1600099020

Harvard Style

Mamoun, A. & Ali, . S. (2021) Clinical and echocardiographic patterns and outcomes of Sudanese children with cardiomyopathy. Sudan J Paed, 21 (2), 131-136. doi:10.24911/SJP.106-1600099020

Turabian Style

Mamoun, Amna, and Sulafa Ali. 2021. Clinical and echocardiographic patterns and outcomes of Sudanese children with cardiomyopathy. Sudanese Journal of Paediatrics, 21 (2), 131-136. doi:10.24911/SJP.106-1600099020

Chicago Style

Mamoun, Amna, and Sulafa Ali. "Clinical and echocardiographic patterns and outcomes of Sudanese children with cardiomyopathy." Sudanese Journal of Paediatrics 21 (2021), 131-136. doi:10.24911/SJP.106-1600099020

MLA (The Modern Language Association) Style

Mamoun, Amna, and Sulafa Ali. "Clinical and echocardiographic patterns and outcomes of Sudanese children with cardiomyopathy." Sudanese Journal of Paediatrics 21.2 (2021), 131-136. Print. doi:10.24911/SJP.106-1600099020

APA (American Psychological Association) Style

Mamoun, A. & Ali, . S. (2021) Clinical and echocardiographic patterns and outcomes of Sudanese children with cardiomyopathy. Sudanese Journal of Paediatrics, 21 (2), 131-136. doi:10.24911/SJP.106-1600099020

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