Aortic cusp involvement causing severe aortic regurgitation in a case of relapsing polychondritis

Abstract

A case with relapsing polychondritis is described where primary involvement of the aortic valve cusps produced severe aortic regurgitation requiring valve replacement. An aneurysmal dilatation of the ascending aorta developing later led to disruption of the prosthesis requiring re-operation. Superior vena caval obstruction, an abdominal aortic aneurysm which ruptured and required resection, and obstructive lesions in common iliac arteries, presumably the result of the same process that involved the aorta and the cartilaginous structures, were also seen.

Patient Perception of Disease‐Related Symptoms and Complications in Relapsing Polychondritis

“…RP is defined by inflammation of cartilaginous structures and the disease course and clinical manifestations are highly variable. End organ complications due to inflammation of cartilage and proteoglycan rich structures are diverse and include deformity of the ears(2), nose(3) and chest(4), subglottic stenosis (5), obstructive lung disease, tracheomalacia(6), bronchomalacia(7), recurrent pneumonia(8), mitral and aortic regurgitation(9, 10), large and medium vessel aneurysms(11), blindness(12), deafness(13), renal insufficiency(14), chronic pain, and sudden death(15–17). The protean aspects of RP pose diagnostic challenges.…”

Multiple Cardiovascular Complications in a Patient With Relapsing Polychondritis

“…15 The postulated causes are aortic root dilatation, cusp retraction, and occasionally cusp rupture and perforation. 16 Transesophageal echocardiographic measurement of the aortic root in our patient indicated mild dilatation with thickening but without retraction of the cusps. However, leaflet abnormalities are likely to have been present because, in addition to "central regurgitation," other pericommissural regurgitant jets were also observed.…”