A case report of systemic lupus erythematosus with severe pulmonary hypertension presenting as large pericardial effusion with early signs of cardiac tamponade: a diagnostic and therapeutic challenge.

Abstract

Background: Pulmonary hypertension is defined as resting arterial pressure >20 mmHg. Cardiac tamponade is a medical emergency where fluids accumulate in the pericardial sac compressing the heart pericardium leading to heart failure. Pericardiocentesis is challenging in patients with cardiac tamponade and severe pulmonary hypertension due to the risk of catastrophic haemodynamic collapse.

Case summary: An 18-year-old female who was recently diagnosed to have systemic lupus erythematosus (SLE) presented to the emergency department with shortness of breath, chest pain, fever, and fatigue. The physical examination revealed tachycardia, muffled heart sounds, and distended jugular venous pulse. Chest X-ray showed cardiomegaly, and transthoracic echocardiography showed a large circumferential pericardial effusion with signs of cardiac tamponade. There was severe pulmonary hypertension along with a dilated right ventricle with systolic dysfunction. The right ventricular systolic pressure was around 100 mmHg. The multidisciplinary team of cardiologists and pulmonologists decided to avoid pericardiocentesis due to the high risk of haemodynamic collapse. Aggressive medical therapy targeting pulmonary hypertension and SLE was started, which resulted in complete resolution of the pericardial effusion and normalization of pulmonary artery pressure.

Discussion: A conservative approach can be an alternative strategy to manage patients with large pericardial effusion and impending pericardial tamponade in the presence of severe pulmonary arterial hypertension as pericardiocentesis carries a high risk of haemodynamic collapse.