Jack is a 56-year-old orthodontist with a history of deep vein thrombosis (DVT) 12 years ago.
He was treated with anticoagulation for 3 months
8 years ago, Jack developed an acute pulmonary embolism (PE) after a long flight
Jack was started on anticoagulation subsequent to the PE, and he has been on it ever since
Jack’s HCP notes that Jack never completely “recovered” from the PE.
He has persistent exercise intolerance
Occasional palpitations and chest pressure—but no syncope
His father had DVT; otherwise, no meaningful family history
Jack’s past medical history includes systemic hypertension and mild arthritis.
He’s on an ACE inhibitor, warfarin, and a beta blocker
16 years ago, he had a knee arthroscopy but has had no other surgeries
On physical exam, Jack appeared generally well.
His lungs were clear, though a murmur was heard over the left posterior lung field
His heart showed a II/VI holosystolic murmur along the left lower sternal border and a somewhat loud P2
He had no edema or clubbing
On referral, initial evaluation of Jack revealed the following:
Chest x-ray showed mild cardiomegaly and clear lungs
Chest CT showed mosaic perfusion and potential irregularities of the right descending pulmonary artery; no luminal filling defects were evident
Echocardiogram showed moderate right ventricular (RV) enlargement and dysfunction
RV systolic pressure was estimated to be 64 + right atrial pressure
Failure to symptomatically return to baseline after an acute PE should raise suspicion for CTEPH1
V/Q scanning is highly reliable and should be ordered promptly2
Bilateral large defects are typical in surgically accessible CTEPH
Referral to a CTEPH center (several throughout the US and Europe) is recommended when CTEPH is suspected2
1. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. Circulation. 2009;119(116):2250-2294. 2. Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62(suppl D):D92-D99.