Clinical & Experimental Cardiology
Open Access
ISSN: 2155-9880
ISSN: 2155-9880
Gobinda Kanti Paul
Mymensingh Medical College and Hospital, Bangladesh
Posters & Accepted Abstracts: J Clin Exp Cardiolog
A dysmorphic feature is a distinctive morphology of body structure deviated from usual normal variant. It can be an isolated finding in an otherwise normal individual, or it can be related to a congenital disorder, genetic syndrome, or birth defect. Master X, seven years old boy has gradual increasing exertional dyspnoea during playing for the last 4 years with retarded growth was considered. Child��?s face looks triangular with low set of ears and depressed nasal bridge with upturned tip. Patient��?s teeth are mal-occluded with poor oral hygiene and high arched palate. Height 95.5 cm, weight 10.3 kg. Weight for age - 3.6 Z score, height for age -5.5 Z score(<3rd centile), weight for Height - 4.2 Z score, BMI - 4 Z score with wide carrying angle(about300). Precordium is slightly bulged with depressed sternum. A systolic thrill is in the left upper parasternal area. Left parasternal lift is present. Pulmonary component of second heart sound (p2) is soft. An ejection systolic murmur (ESM) grade, 4/6 is in the pulmonary area which radiates to infraclavicular region and back. Echocardiogram shows severe Pulmonary valvular stenosis (PS, PPG-128 mmHg) with concentric right ventricular hypertrophy (RVH).ECG also shows RVH. All the positive findings are in favor of Noonan syndrome (NS). NS is characterized by distinctive facial features, short stature, chest deformity, congenital heart disease, most commonly PS (about 70% cases). The incidence of NS is as 1 in 1,000 to 1 in 2,500 births. Diagnosis of Noonan syndrome is usually made after a doctor observes some key signs.
Gobinda Kanti Paul has completed his MD Cardiology, from NICVD under BSMMU, January 2008. He has more than 10 publications in different index journal one of the journals is in our medical college, named MMJ. One of his articles was published in International Journal of Clinical Medicine Research of AASCIT. He is also a Life Member of Indian Academy of Echocardiography. He has passion for nonintervention cardiology specially echocardiography and preventive cardiology. Currently, he is an Assistant Professor/Consultant Cardiology and Residential Physician in the Mymensingh Medical College and Hospital. He has special interest in rheumatic fever and rheumatic heart disease.