Assessment of cardiac function in children with congenital adrenal hyperplasia: a case control study in Cameroon

Abstract

BackgroundHigh level of androgens found in congenital adrenal hyperplasia (CAH) seems to have a deleterious effect on heart function. We therefore evaluate cardiac function of children with CAH in comparison with a healthy group.MethodsWe carried out a case-control study in the single endocrinology unit of the Mother and Child Center of Chantal Biya’s Foundation. Cases were matched for age and genotypic sex to 2 healthy controls. We analyzed the ejection fraction (LVEF), fractional shortening and left ventricular mass; output and cardiac index; E and A waves velocities, E/A ratio and the mitral deceleration time and diameter of the left atrium; tricuspid annular plane systolic excursion and pulmonary artery systolic pressure were also measured.ResultsWe included 19 patients with a median age of 6.26 ± 3.75 years and 38 controls stackable distribution. The left ventricular mass of cases was greater than that of controls. A case of reversible cardiomyopathy on hormone replacement therapy was found.For the cases, the average ejection fraction was 71.95 ± 7.88%; the average fractional shortening was 40.67 ± 7.02%. All these values ​​were higher than those of controls, although the difference was not statistically significant. Diastolic left ventricular function was more impaired among the cases.Right ventricular function was similar in both groups. These abnormalities were highly correlated to the late age at diagnosis and duration of treatment.ConclusionThis study shows an altered cardiac function in CAH compared to healthy control and highlights importance of an early diagnosis of cases, a tight control of androgens levels and a regular monitoring of cardiac function.

Cardiac function in paediatric patients with congenital adrenal hyperplasia due to 21 hydroxylase deficiency

“…The finding of impaired left ventricular diastolic function was based on the presence of a lower ratio of Doppler‐derived E/A wave, prolonged mitral deceleration time and isovolumetric deceleration times in studied patients with CAH compared to controls. Left ventricular diastolic dysfunction was associated with testosterone levels and the late age of diagnosis in three of the studies; in one study, no correlation with androgen levels was found . Left ventricular hypertrophy, indicated by an increased left ventricular mass index, was described by two studies .…”

“…None of the studies used the newer echocardiographic technique, for example myocardial deformation, for the detection of early signs of myocardial damage. Left ventricular diastolic dysfunction was described in Italian adolescent male patients with CAH (n = 10)and in paediatric patients with CAH from Cameroon (n = 19), Egypt (n = 32) and Turkey (n = 25) . The finding of impaired left ventricular diastolic function was based on the presence of a lower ratio of Doppler‐derived E/A wave, prolonged mitral deceleration time and isovolumetric deceleration times in studied patients with CAH compared to controls.…”

“…Seven of the nine patients had an abnormal fractional shortening and rate‐corrected velocity of circumferential fibre shortening at baseline that was improved in six of the patients after glucocorticoid treatment. The other previous studies on cardiac function in patients with CAH evaluated cardiac function in paediatric and adolescent patients, mean age ranges of 6.3‐13.6 years, using conventional echocardiography and, in most studies, tissue Doppler imaging . None of the studies used the newer echocardiographic technique, for example myocardial deformation, for the detection of early signs of myocardial damage.…”

Homocysteine Level in Children with Classic Congenital Adrenal Hyperplasia: Relationship to Carotid Intimal Wall Thickness and Left Ventricular Function

“…Similarly, Tony Nengom et al [37] reported positive correlations between homocysteine levels and mitral DcT. It has been suggested that homocysteine directly promotes cardiac fibrosis and cardiac matrix metalloproteinase activity, resulting in diastolic dysfunction [38].…”

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“…where αn is the n-Womersley number, a is the artery radius of the inlet, n is the number of modes, and ω is the angular frequency obtained from the period of the cardiac cycle. In our study, we have chosen eight modes and an angular frequency of 7.703 s-1 (period of the cardiac cycle was 0.857 s) [23]. The Womersley solution depends on the Womersley number, and it is a measure of the ratio of the momentum equation to the viscous part [21].…”