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Assembly

Hiding in Plain Sight

Author
Masooma Aqeel, MD 1; Jayshil J. Patel, MD 2
1 Assistant Professor, Division of Pulmonary, Critical Care & Sleep Medicine, Aga Khan University, Karachi, Pakistan
2Associate Professor, Division of Pulmonary, Critical Care & Sleep Medicine, Medical College of Wisconsin, Milwaukee, USA


Case

A 42-year-old non-smoking male presents with 6-months of fatigue, progressively worsening dyspnea and scant hemoptysis. Physical examination reveals resting room-air oxygen saturation (SpO2) of 92%, jugular venous distention (JVD) (11 cm H2O above right atrium), a loud pulmonic component of the 2nd heart sound (P2), S4 gallop, a left parasternal (right ventricular (RV)) heave and left leg pitting edema (1+). Lungs are clear. Echocardiography suggests severe pulmonary hypertension and severely reduced RV function. Computed tomography pulmonary angiogram (CTPA) reveals:

Computed tomography pulmonary angiogram

Question

What is the first-line treatment for this condition?

A. Pulmonary endarterectomy (PEA)
B. Lifelong anticoagulation
C. Supplemental oxygen, diuretics and spironolactone
D. Tissue plasminogen activator (tPA)
E. Riociguat

Answer