With increasing use of echocardiography, an increasing number of patients are being identified as having pulmonary artery hypertension (PAH). In patients with normal systolic left ventricular (LV) function, extensive work up is often performed, even in patients in whom a comprehensive history, physical examination and chest radiograph does not suggest an underlying diagnosis. This descriptive study investigates the characteristics of these patients and the outcomes of diagnostic testing.
Pulmonary consult sheets over a period of 4 years were reviewed, and patients with newly diagnosed PAH, by echocardiography, identified. Patients with a likely etiology for PAH on history or examination were excluded from the study. Medical records were then reviewed and the following data abstracted: patient demographics, co-morbid conditions, medications taken, and results of echocardiography, chest CT scan, PFT, polysomnogram, serologies for connective tissue disorders, and tests for pulmonary embolism (PE) (ventilation-perfusion scan or PE protocol chest CT).
Twenty-six patients were included in the study. Twenty (77%) were women. Mean age was 66 (±11) years and 19 (73%) were hypertensive. The mean pulmonary artery systolic pressure was 60 (±12) mmHg and LVH was present in 19 (73%). An average of 3.5 diagnostic studies were done. Three of 22 chest CT scans were abnormal: 2 with interstitial lung disease and one with severe emphysema. One of 18 PE studies was abnormal. Nine of 12 polysomnograms revealed obstructive sleep apnea. Two of 12 serologies were abnormal. 23 patients had PFTs: 16 had minor abnormalities, 4 had severe restriction and one severe obstruction. Five (19%) had relatively normal studies except for elevated PAP on echocardiography. Seven (27%) patients had LVH and no other abnormality that adequately explained PAH. Six (23%) patients had LVH and OSA.
The prevalence of hypertension and LVH was unexpectedly high in our cohort of PAH. Except for polysomnograms, the other studies had poor diagnostic yields.
LVH with diastolic dysfunction may be the underlying etiology in a significant proportion of patients with PAH.
Vinay Sharma, None.