Management of Cardiovascular Disorders in Patients With Noonan Syndrome: A Case Report



Khorgami MR¹ ; Moradian M¹ ; Omidi N² ; Aarabi Moghadam MY¹ Show Affiliations
Source: Journal of Tehran University Heart Center Published:2017

Abstract

The Noonan syndrome is a rare disorder, one of whose major complications is cardiovascular involvement. A wide spectrum of congenital heart diseases has been observed in this syndrome. The most common cardiac disorder is pulmonary valve stenosis, which has a progressive nature. Hypertrophic cardiomyopathy is less common, but its morbidity and mortality rates are high. We herein introduce a 12-year-old boy with the typical findings of the Noonan syndrome. His symptoms began from infancy, and there was a gradual exacerbation in his respiratory and cardiac manifestations with age. The cardiac involvement included right ventricular outflow tract and pulmonary valve stenosis, hypertrophic cardiomyopathy, and subaortic valve stenosis. Due to the progressive course of the disease, surgical repair was done. Although the patient had a difficult postoperative period, his general condition improved and he was discharged. At 3 months’ follow-up, his symptoms showed improvement. Additionally, there was a reduction in the echocardiographic parameters of the outflow tract stenosis gradient as well as a significant improvement in the cardiac hemodynamic indices. © 2017, Tehran Heart Center. All rights reserved.