ESPE Abstracts (2016) 86 P-P1-48

Cardiac Function in Pediatric Patients with Congenital Adrenal Hyperplasia

Christiaan F. Mooija, Milanthy S. Pourierb, Gert Weijersc, Chris L. de Kortec, Hedi L. Claahsen - van der Grintena & Livia Kapustad,e


aDivision of Pediatric Endocrinology, Department of Pediatrics, Amalia Children’s Hospital, Radboud University Medical Center, Nijmegen, The Netherlands; bDepartment of Pediatrics, Amalia Children’s Hospital, Radboud University Medical Center, Nijmegen, The Netherlands; cMedical Ultrasound Imaging Center, Department of Radiology, Radboud University Medical Center, Nijmegen, The Netherlands; dPediatric Cardiology Unit, Dana-Dwek Children’s Hospital, Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel; eDepartment of Pediatric Cardiology, Amalia Children’s Hospital, Radboud University Medical Center, Nijmegen, The Netherlands


Background: Congenital adrenal hyperplasia (CAH) is characterized by cortisol deficiency and hyperandrogenism. Both hyperandrogenism and treatment with supraphysiological doses of glucocorticoids may cause unfavorable changes in the cardiovascular risk profile of CAH patients. Data on the cardiac function in CAH patients is scarce.

Objective and hypotheses: To evaluate the cardiac function in pediatric CAH patients.

Method: The cardiac function of 27 pediatric CAH patients, aged 8–16 years, was evaluated by conventional echocardiography and 2D myocardial strain (rate) imaging. Results were compared to a selected cohort of 27 age and gender matched healthy controls. Data on hydrocortisone dosage in CAH patients were collected. Blood was withdrawn in CAH patients to evaluate 17-OH-progesterone and androstenedione concentrations.

Results: Left ventricular parameters (IVSd, LVIDd, LVIDs, EF and FS) are normal in all CAH patients, and do not differ from controls. In contrast, LVPWd is significantly lower in CAH patients compared to controls (5.55 vs 6.53 mm; P=0.009). The LVPWd Z-score is significantly lower in CAH patients yet within the normal range (−1.12 vs −0.35; P=0.002). LV mass and LV mass index are normal and comparable in both patients and controls. Isovolumetric relaxation time is significantly lower in CAH patients (49 vs 62 ms; P=0.003). No associations were found between left ventricular parameters and BMIS SDS, hydrocortisone dose, androstenedione and 17-OH-progesterone, respectively. Global longitudinal, radial and circumferential strain were normal compared to controls. Global radial strain rate was significantly higher compared to healthy controls (2.58 vs 2.06 1/s). Time to peak global longitudinal, radial and cicumferential strain did not differ between CAH patients and controls.

Conclusion: Cardiac evaluation of pediatric CAH patients showed no signs of left ventricular hypertrophy or ventricular dilatation. We found a thinner LVPWd in CAH patients, this finding was not associated with treatment or hyperandrogenism. A shorter isovolumetric relaxation time in CAH patients suggested increased left atrial pressure.

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