Endomyocardial fibrosis in Cameroon: Echocardiographic and clinical description of a pediatric series in Yaoundé

Background: Endomyocardial fibrosis is a restrictive cardiomyopathy that causes heart failure. It is characterized by the fibrotic thickening of the endocardium, sometimes involving the myocardium as well. The lesion generally lies at the apices or inflow tracts of one or both ventricles, associated with more or less severe alteration of the valves. It is a disease of the intertropical regions but is not well known in Cameroon. In this study we describe the first series seen in a pediatric hospital in Cameroon. Patients and methods: A retrospective study was conducted in a pediatric hospital in Yaoundé involving children who had been diagnosed with endomyocardial fibrosis after echocardiographic investigation. We collected the clinical and paraclinical data from consultation records and medical files. Results: Between January 2006 to December 2013, we registered 1430 patients with a cardiac anomaly in our center. Endomyocardial fibrosis was found in 46 patients. Neither sex predominated. Ages at the time of diagnosis varied between 2 and 17 years. Most of the patients were between 5 and 15 years old (80.4 %), with a median of 10 years (interquartile range, 7-13 years). The main complaints were breathlessness, cough, abdominal distension, abdominal, and loss of appetite. Apart from the hyperpigmentation of the lips observed in all our patients, dyspnea was the most frequent physical sign and the diagnosis was made at a time when signs of heart failure were preponderant. Growth retardation was found in all the children examined. All patients were underweight with a median weight for age found below the 25th percentile of the norms according to the National Health Statistics. Lower limb edema was absent even in the presence of voluminous ascites. All subjects had hyperpigmented lips. Despite the cyanotic appearance of the lips, pulse oximetry always gave a normal oxygen saturation level and no cyanosis was seen elsewhere. None of the patients had nail clubbing. Fibrosis more often affected the right ventricle (45/46 patients). The apical obliteration by fibrotic material was found in 43 (93.5 %) patients. Moreover, 36 (78.3 %) patients had pericardial effusion: mild to moderate in 32 subjects and abundant in four subjects. Hypereosinophilia was noted in 57.5 % of the patients. Atrial fibrillation was found in six out of 15 patients who had an electrocardiogram done. Conclusion: The modes of clinical presentation of endomyocardial fibrosis are not sufficiently well known in our context. Despite its insidious progression, certain signs such as weight loss and hyperpigmented lips could be very helpful for screening and easing orientation of parents and heath personnel, thus enabling early referral for appropriate investigation. The presence of bulky ascites without edema of the lower extremities should be viewed as strongly suggestive.