Vnitr Lek 2007, 53(1):71-77

Pulmonary hypertension in Takayasu arteritis

M. Škvařilová^1,*, J. Lindner², B. Hanzová³, J. Dušková³, P. Jansa¹, T. Paleček¹, M. Aschermann¹

¹ Centrum pro plicní arteriální hypertenzi II. interní kliniky kardiologie a angiologie 1. lékařské fakulty UK a VFN v Praze, přednosta prof. MUDr. Aleš Linhart, DrSc.

² II. chirurgická klinika 1. lékařské fakulty UK a VFN v Praze, přednosta doc. MUDr. Jan Tošovský, CSc.

³ Ústav patologie 1. lékařské fakulty UK a VFN v Praze, přednosta prof. MUDr. Cyril Povýšil, DrSc.

Takayasu arteritis (TA) is a rare chronic vasculitis primarily involving the aorta and its main branches. The disease affects women much more frequently than men, the ratio being 8: 1. The onset occurs in the teenage years, always before age of 40. Aortic regurgitation is rare. The pulmonary artery stenosis may also rarely occur. We report the case of a 49-year old female patient with Takayasu arteritis who presented with severe left pulmonary trunk stenosis resulting in pulmonary hypertension, severe aortic regurgitation due to the dilatation of the ascending aorta, mitral insufficiency, critical left renal artery stenosis, and probably with left main coronary artery stenosis. No severe stenosis or occlusion in the mid portion of mid subclavian artery lesion were present. Because the patient presented with serious congestive heart failure, she underwent mitral valvuloplasty and aortic valve replacement. However, the patient died in early postoperative period due to pulmonary hypertension which failed to respond to pharmacotherapy.

Keywords: pulmonary hypertension; Takayasu arteritis; aorta; aortic regurgitation

Received: June 15, 2006; Accepted: September 26, 2006; Published: January 1, 2007  Show citation