CASE REPORT: Biventricular Hypertrophic Cardiomyopathy in a 26-year-old Nigerian Woman with Noonan Syndrome: West Afr J Med. 2024 July; 41(7): 826-830 PMID:  39357066

O. S. Ogah; A. Aje; V. A. Obasuyi; O. A. Orimolade; I. T. Nebo; F. A. Ajao; D. A. Olawuyi; V. K. Olalekan; C. M. Ogah; F. E. Obiekwe; M. N. Odenigbo; O. M. Adebayo; A. M. Adeoye; O. O. Oladapo; A. Adebiyi

Authors

O. S. Ogah
A. Aje Cardiology Unit, Department of Medicine, University College Hospital Ibadan, Nigeria.
V. A. Obasuyi Cardiology Unit, Department of Medicine, University College Hospital Ibadan, Nigeria.
O. A. Orimolade Cardiology Unit, Department of Medicine, University College Hospital Ibadan, Nigeria.
I. T. Nebo Alexander Brown Hall, College of Medicine, University of Ibadan, Nigeria.
F. A. Ajao Alexander Brown Hall, College of Medicine, University of Ibadan, Nigeria.
D. A. Olawuyi Alexander Brown Hall, College of Medicine, University of Ibadan, Nigeria.
V. K. Olalekan Alexander Brown Hall, College of Medicine, University of Ibadan, Nigeria.
C. M. Ogah Ameyo Adedavoh Hall, Benjamin Carson (Snr) College of Health and Medical Sciences, Babcock University Ilishan-Remo, Ogun State, Nigeria.
F. E. Obiekwe Alexander Brown Hall, College of Medicine, University of Ibadan, Nigeria.
M. N. Odenigbo Alexander Brown Hall, College of Medicine, University of Ibadan, Nigeria.
O. M. Adebayo
A. M. Adeoye
O. O. Oladapo
A. Adebiyi

Keywords:

Dysmorphia, Hypertrophic cardiomyopathy, Noonan syndrome

Abstract

Background: Cardiac disorders are found in about half of cases of Noonan syndrome (NS). The most common congenital heart diseases in this syndrome include pulmonary valvular stenosis obstructive or nonobstructive hypertrophic cardiomyopathy (17%). Biventricular hypertrophic cardiomyopathy (HCM) is very rare in this condition.

Objective: The objective is to report a case of biventricular hypertrophic cardiomyopathy in a 26-year-old Nigerian female with the phenotype.

Methods: This is a descriptive case report.

Results: The patient presented with dyspnoea on exertion which started at the age of 7 years and has progressively worsened. There was associated precordial chest pain and palpitation. Clinical examination revealed a young woman, who is small for her age. She had some dysmorphic features such as a webbed neck, lowset ears, low posterior hairline, crowded teeth, high arched palate, a small and asymmetric chin and a high carrying angle at the elbows. The pulses were synchronous and there was no radio-radial or radiofemoral delay and her blood pressures were within normal limits. Cardiac auscultation was unremarkable. The 12-lead ECG showed biventricular hypertrophy with a strain pattern. The echocardiogram showed features in keeping with biventricular hypertrophic cardiomyopathy.

Conclusion: Biventricular HCM is relatively uncommon in Noonan syndrome. Patients with typical dysmorphia should have a full cardiac evaluation to look for these anomalies.