Open AccessNarrowing of the dark valve: aortic stenosis in alkaptonuria
Author(s) -
Ketak Nagare,
Mohammed Idhrees,
Mohammed Ibrahim,
Aju Jacob,
Bashi Velayudhan
Publication year - 2020
Publication title -
indian journal of thoracic and cardiovascular surgery/indian journal of thoracic and cardiovascular surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.114
H-Index - 9
eISSN - 0973-7723
pISSN - 0970-9134
DOI - 10.1007/s12055-020-01059-z
Subject(s) - alkaptonuria , medicine , stenosis , asymptomatic , cardiac surgery , cardiology , aortic valve replacement , ascending aorta , ochronosis , prosthesis , surgery , aortic valve , aortic valve stenosis , aorta , dermatology
The commonest cardiac pathology in patients with alkaptonuria is aortic stenosis. Patients with alkaptonuria and aortic stenosis may remain asymptomatic until the 6th decade. Surgeons may have to deal with per-operative difficulties as alkaptonuria is a systemic disease. Proper preoperative planning is important. The mechanical valve prosthesis is advisable in a patient with alkaptonuria and aortic stenosis considering disease pathophysiology. We report a 70-year-old male diagnosed with alkaptonuria and aortic stenosis, who underwent aortic valve (mechanical valve prosthesis) and ascending aorta replacement.