01 March 2017 by Bahri Akdeniz

A rare cause of pulmonary hypertension: pulmonary artery involvement of Takayasu arteritis: two cases in a single centre experience

Introduction

Pulmonary involvement of Takayasu arteritis is a rare condition among pulmonary arterial hypertension population. We hereby are presenting two cases of pulmonary hypertension due to pulmonary artery involvement of Takayasu Arteritis

Case 1

A 27 year old woman was admitted to our clinic due to exertional dyspnea and fatigue. Precapillary PH was confirmed at catheterization Perfusion scan and Thorax CT was normal. We noticed a systolic murmur at 2nd intercostal space on right hemitorax.. Pulmonary angiography revealed a significant stenoses with an aneurysm formation at superior lobe of right pulmonary artery (Figure 1 a) as well as inferior lobar artery (Figure 1 b) .consisted with vacuitis Assessment of other arterial system revealed total occlusion of both carotid arteral system with a thickness of arterial wall (1c, 1d). Takayasu arteritis was diagnosed. All other arterial system was normal. Therapies with several immunosuppressive agents including Tocailizumab treatment were performed in th follow up period.. After 2 year of therapy the patients was stable condition with a good functional class and 6MWD. Despite this, narrowing of pulmonary artery was more prominent at control pulmonary angiogram (Figure 2)

Case 2 

A 28 year old woman referred to our clinic for catheterization due to pulmonary hypertension detected at echocardiography. She has been treated with Takayasu arteritis at our rheumatology department.. Fist diagnosis was established at 2010 with an aortic, renal internal carotid and subclavian artery involvement. Lupus anticoagulant was also detected Graft stent implantation of descending aorta and renal artery was made. One year later, stent in the left renal artery was occluded and renal failure was begun. She has been under hemodialysis treatment for 3 years due to renal artery stenoses. Her mean PAP, pulmonary vascular resistance and cardiac output were 60 mmHg, 10.6 Wood and 4.7 l/min/m2 subsequently. Her blood pressure was also high (171/121 mmHg) despite antihypertensive therapy. Pulmonary angiogram revealed a diffuse narrowing of right pulmonary artery, from main lobar brunch. Inferior descending of left pulmonary artery was also occluded as well as significant stenoses of upper lobe subsegmetal artery. Large perfusion defect was also present at late phase.

Conclusion

Pulmonary hypertension due to pulmonary artery involvement of Takayasu arteritis is a rare disease. This etiology should be taken into consideration especially among young women who had been investigated for pulmonary hypertension. Perfusion defect may not be present at lung scintigraphy and a simple murmur heard on the lung fields may be the only ominous sign for making diagnosis.

Key Contributors

Dr. Bahri Akdeniz*, Dr. Melih Birlik#, Dr. Gökce Kenar#, Dr . Mustafa Barış &, Dr. Kemal Can Tertemiz § Dr Ebru Özpelit Department of * Cardiology* , Rheumatology# Radiology& and Chest medicine§ Dokuz Eylül University School of Medicine Izmir Turkey


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