13: Pulmonary Arteritis… The Great CTEPH Mimic


Figure 13.1

Lung scintigraphy demonstrating a number of unmatched perfusion defects, predominantly involving the upper lobes, lingula and right middle lobe


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Figure 13.2

(a) Right main pulmonary artery narrowing (white arrow) throughout its course with marked vessel narrowing of the left descending PA (chevron). Bronchial arterial collateral vessels also noted. (open arrow). (b) Proximal to distal (left to right) axial images of the CT angiogram shown in (a). Small right descending PA to attenuated proximal RLL segmental vessels to dilatation of the more distal segmental vessels of the right lower lobe. No CT evidence for chronic thrombus



On initial evaluation at a CTEPH center he reported some improvement in exercise tolerance, but remained WHO functional class III with one block DOE and exertional lightheadedness. Additional PMH included left spontaneous pneumothorax treated with VATS (report of evaluation for Marfan’s negative at that time), chronic hoarseness, mild thrombocytopenia, questionable Protein S deficiency in the setting of warfarin therapy, and abnormal LFTs with a liver ultrasound demonstrating early cirrhotic changes. His family history was remarkable for a sister who required surgery as a child for a “hole in her heart”. He previously drank 6–12 beers daily, but had decreased his alcohol intake substantially and continued to smoke marijuana.


Physical exam revealed a very tall and thin Hispanic male comfortable at rest. HR 65, BP 136/67, RR 16, SpO2 96% on room air. There was no JVD, but a carotid bruit vs. a transmitted murmur over his left carotid artery was auscultated. Lung fields were clear with loud pulmonary flow murmurs noted anteriorly and posteriorly in both lungs. There was no RV lift, S1 was normal with a loud second heart sound and a soft systolic murmur without gallop. Abdominal exam was normal without evidence of bruits. Extremities revealed no edema, no bruits over proximal arteries, and pulses were all strong and symmetric except for a diminished right dorsalis pedis and posterior tibialis pulses.


Laboratories were notable for elevated alkaline phosphatase (251 U/L) and ALT/AST (72/46 U/L) that were improved from prior labs, BNPP was normal. CBC was normal. A chest X-ray (Fig. 13.3) showed no cardiomegaly or central pulmonary artery enlargement, but blunting of the costophrenic angle with some diaphragmatic tenting was noted on the left.

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Figure 13.3

PA chest radiograph: no central pulmonary artery enlargement despite the severe pulmonary hypertension documented by right heart catheterization


An echocardiogram showed normal right and left sided chamber size and function, normal valvular function and estimated RV systolic pressure of 121 mmHg. Repeat right heart catheterization was performed: RA 5, RV 110/6, PA 110/16 (mean 43), PAOP 7, cardiac output 4.25 L/min, cardiac index 2.19 L/min/m2, PVR 678 dynes s cm−5, PA saturation 70%.


To better define pulmonary vessel anatomy, digital subtraction pulmonary angiography (Fig. 13.4a, b) was performed. On the right, a small main pulmonary artery was noted with absent perfusion to the posterior RUL, post-stenotic dilation of the apical RUL, proximal segmental obstruction of the RML, irregularity and narrowing of the RLL arteries with absent flow to the superior segment. The left main pulmonary artery was also small with marked narrowing of the descending PA, absent perfusion to the majority of the LUL and lingula and flow to the LLL confined to the posterior basal artery.

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Oct 30, 2020 | Posted by in Uncategorized | Comments Off on 13: Pulmonary Arteritis… The Great CTEPH Mimic

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