Challenges on the management of congenital heart disease in developing countries

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Abstract

Introduction

While in developed countries prenatal diagnosis is currently used to detect Congenital Heart Disease before (CHD) before birth, in developing countries only a minority of children with CHD is detected and few benefit from surgical treatment. Having created a referral unit for diagnosis and treatment of cardiovascular diseases in a resource-deprived country we designed a study aiming at describing the spectrum and characteristics of CHD and discuss the challenges of its management.

Population and methods

We studied retrospectively all patients assisted at a referral unit between 2001 and 2007, collecting epidemiological, clinical, echocardiographic and surgical data from hospital files.

Results

We studied 534 patients with median age at diagnosis of 4 years (range 0–79); 296 were females (55.4%). Only 282 (52.8%) patients were diagnosed under the age of two years, and complications were present in 155 (29.0%) at time of diagnosis. We operated 196 patients with mean age of 8 ± 10 years. Only 29 of the 111 complex defects were considered for surgery. The 30-days post-operative mortality was 8/196 (4.0%). The most important post-surgical complications were post pericardectomy syndrome (22).

Discussion

A pattern of late presentation accompanied by high rate of complications was found. In resource-deprived settings the approach to the management of CHD emphasizes the treatment of “curable” malformations. Surgery for CHD in these settings must be linked to early detection and a referral system in which general practitioners, pediatricians, obstetricians and cardiologists interact in the design and implementation protocols for diagnosis, management and follow-up of patients.

Introduction

Congenital Heart Disease (CHD) occur worldwide and although the incidence and prevalence may vary according to genetic and environmental factors there is no reason to think that they are lower in developing countries compared to developed areas. Considering that the pediatric population constitutes a large portion of the population in African countries, and the lack of correction of the congenital defects due to the absence of open-heart surgery, there is certainly an accumulation of large numbers of children and adults with CHD. Indeed hospital-based data indicate that CHD remains an important cause of heart failure in Africa [1], and a survey conducted in Mozambique in schoolchildren as shown that CHD are at least as common as in Caucasians [2]. Finally, a recent study on structural heart disease in children from Nigeria, showed that contrary to previous hospital reports CHD rather than RHD and other acquired heart diseases are dominant in some African settings [3].

CHD are defined as abnormalities in the cardiovascular structure or function that are present at birth, and remain one of the most frequent birth defects, with a live born prevalence of about 5–8 per 1000 (0.5–0.8%) in western countries. In these countries the diagnosis of CHD is established by one week of age in 40–50% of patients and by one month in up to 60% [4]. While in some developed countries prenatal diagnosis is currently used to detect most CHD before birth, in developing countries only a minority of children with CHD are detected, often at late stage, and few benefit from surgical treatment, because of delayed diagnosis, lack of skilled personnel and absence of facilities for surgery.

Having created a referral unit for cardiovascular diseases in a resource-deprived country we aimed at describing the spectrum and characteristics of CHD diagnosed in this unit and discuss the challenges of the management of these conditions in developing countries.

Section snippets

The setting

The Instituto do Coração is a cardiovascular service implanted in a poor country of Southern Africa, Mozambique. It serves 20 million people from all regions of the country, and is the only institution where cardiac catheterization and surgery could be performed in the period of the study. Surgery is performed by renowned surgeons who come periodically to our facilities from Europe, with a team of nurses and doctors for intensive care attention during the immediate post-operative phase.

Results

During the period of the study 534 patients with CHD were assisted. The median age at diagnosis was 4 years (range 0–79) and 296 patients were females (55.4%). Only 282 (52.8%) patients had their malformation diagnosed under the age of two years, while 179 (33.5%) were diagnosed between 2 and 17 years, and 73 (13.7%) after 18 years. Complications were present in 155 patients (29.0%) at time of diagnosis, namely severe policytemia 49 (9.2%), heart failure 47 (8.8%), fixed pulmonary hypertension 45

Discussion

The diagnosis of CHD is made late in life, mainly through complications such as heart failure, pulmonary vascular obstructive disease, severe cyanosis and infective endocarditis, frequently with long-term sequelae. This pattern of late presentation accompanied by high rate of complications is comparable to that found in studies done in similar settings [6], [7]. Associated malformations were frequent in children diagnosed with CHD and might be one of the calling signs for referral to

Conclusions

The approach to the management of CHD in poor settings is different from that in developed countries, emphasizing the treatment of diseases that can be “cured” and need no medication or close follow-up after surgery. Cardiologists are frequently confronted with patients with sequelae and severe complications who are no more operable.

Although surgery for CHD should be available in poor-countries it must be linked to a strategy of early detection and creation of a network that allows easy

Acknowledgments

We are grateful to the surgical teams from Chain of Hope (United Kingdom), Chain de L'Espoir (France), Coeur Pour Tous (Switzerland) and Cadeia da Esperança (Portugal) for their work at the Instituto do Coração.

The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology [16].

References (16)

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