Echocardiographic Diagnosis of Severe Pulmonary Arterial Hypertension in Systemic Sclerosis in a Resource-Constrained Somali Healthcare Setting

Background Systemic sclerosis (scleroderma) is a chronic autoimmune connective-tissue disorder characterized by progressive microvascular injury and multiorgan fibrosis. Cardiopulmonary involvement—including pulmonary arterial hypertension (PAH), right-sided heart failure, and pericardial disease—remains a leading cause of morbidity and mortality. Early diagnosis of PAH is critical, yet it is frequently delayed, particularly in resource-limited settings where access to advanced diagnostics and targeted therapies is limited. Case Presentation We report the case of a 40-year-old woman with established systemic sclerosis, diagnosed 5 years prior, who presented with progressive exertional dyspnea, orthopnea, and lower-limb edema. Physical examination revealed signs consistent with right heart failure (jugular venous distention and peripheral edema), along with SSc-related features including sclerodactyly, but notably lacking telangiectasias or calcinosis cutis. Transthoracic echocardiography demonstrated severe pulmonary hypertension (estimated PASP >70 mmHg), gross right atrial and right ventricular dilatation, severely impaired right-ventricular systolic function (TAPSE ≈ 0.8 cm), and a D-shaped left ventricle. Additional findings included severe tricuspid and pulmonic regurgitation, reduced left-ventricular ejection fraction (29%), and mild circumferential pericardial effusion. The patient received supportive medical therapy and was referred to a specialized center for advanced PAH evaluation and right-heart catheterization due to limited local access to targeted therapies. Conclusion This case illustrates the severe cardiopulmonary complications of systemic sclerosis, particularly advanced PAH leading to progressive right-heart failure. Echocardiography played a central role in diagnosis by demonstrating hallmark findings such as right-sided chamber enlargement, severe tricuspid and pulmonic regurgitation, and interventricular septal flattening. Given the limited availability of targeted PAH therapies and advanced diagnostics in resource-restricted settings, early recognition, systematic screening, and timely referral to specialized centers are essential to improving outcomes in patients with connective-tissue disease–associated PAH.