Development of pulmonary artery hypertension (PAH) worsens prognosis of systemic sclerosis (SSc) and can be either isolated precapillary PAH or secondary to interstitial lung disease (ILD). Early diagnosis is of crucial importance. There is scarcity of data on PAH in patients with SSc in Bangladesh. The objective if the study is to determine the Prevalence and clinical correlates of pulmonary hypertension in systemic sclerosis. Clinical and functional characteristics of 100 patients of systemic sclerosis were studied and they were evaluated by echocardiography to detect pulmonary artery hypertension. Our objective was to study the prevalence and the clinical correlation of PAH in SSc. PAH was found in 29% patients on echocardiography. Prevalence tend to increase with age of onset and duration of disease. However, it did not differ significantly between patients with limited cutaneous SSc (lcSSc) and patients with diffuse cutaneous SSc (dcSSc). On binary logistic regression analysis, none of the studied variables had any independent influence on development of PAH. PAH in SSc occurs in a remarkable proportion (29%) of patients without any ominous signs in early stages. Non-invasive screening of patients with SSc for PAH will help in early diagnosis and appropriate timely therapeutic intervention before significant end-organ damage occurs.

<p>A 4 year old girl was presented with the respiratory tract infection, breathlessness after taking meal, failure to thrive, abnormal movement of the chest on left side overlying the area of heart and systolic murmur. She developed these symptoms gradually for the last 3.5 years. Echocardiography revealed doubly committed subarterial ventricular septal defect with moderate aortic regurgitation. The size of the ventricular septal defect was 7 x 9 mm at the left ventricular outflow tract. The right coronary cusp of the aortic valve was prolapsed. Left atrium and left ventricle were dilated. The pulmonary artery systolic pressure was 35 mm Hg. The ventricular septal defect was closed with the standard surgical procedure using cardiopulmonary bypass followed by aortotomy and right atriotomy. Immediate post-operative period of this case was uneventful and the patient was discharged on 9^th post-operative day. Follow-up echocardiography showed no residual ventricular septal defect or aortic regurgitation and the ventricular function was good.</p>