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Isolated pulmonary artery (PA) stenosis in vasculitis is rare and a challenge to diagnose and treat. It carries high mortality due to risk of right heart failure and death.
A 21-year-old woman presented with 2 weeks of progressive shortness of breath. The physical exam was notable for II/VI systolic ejection murmur with split S2 and low blood pressure. Laboratory tests were notable for C reactive protein of 127mg/L. Transthoracic echocardiogram revealed moderate pericardial effusion (Figure 1A). Right heart catheterization was notable for mean PA systolic pressure distal to the stenosis of 17 mmHg and RV systolic pressure of 64 mmHg; RV angiography showed bilateral PA stenosis (Figure 1B). Pericardiocenthesis was non-diagnostic.18F FDG PET/CT showed isolated PA stenosis (Figure 1C).
The patient was referred for surgery. Visualized intraoperatively was severe thickening and obstruction of distal main, right, and left main PA with severe RV dilatation and dysfunction. Patient underwent proximal main PA to distal right main PA conduit. Histology revealed giant cells and the diagnosis of Takayasu vasculitis was made (Figure 1D). Patient was treated with anticoagulation, steroids and subsequently tocilizumab.
This case highlights a rare presentation of Takayasu vasculitis involving main pulmonary artery. It emphasizes successful use of PET imaging for diagnosis and surgical approach for symptom relief and treatment.
Sunday, March 17, 2019, 2:00 p.m.-2:20 p.m.
Session Title: FIT Forum: Stump the Professor
Abstract Category: Vascular Medicine
Presentation Number: 905-07
- 2019 American College of Cardiology Foundation