A case of a 66 year-old female with coronary artery disease, coronary artery aneurysms, mitral valve prolapse with severe mitral regurgitation, and supravalvar aortic stenosis

Tóm tắt

Background

Supravalvar aortic stenosis (SVAS) is rare and is usually associated with Williams-Beuren syndrome, a developmental disorder. Non-syndromic SVAS has been associated with atherosclerotic cardiovascular disease (ASCVD) but has not been associated with rheumatic heart disease (RHD)

Methods

This study is a case report which defines and describes a clinical case and reviews the available diagnostic and management options

Results

A 66 year-old diabetic female, with history of RHD, presents with a 3 month history of angina and progressive heart failure symptoms. On physical exam, she was hemodynamically stable with a grade 4/6 systolic murmur at the left parasternal border and a grade 3/6 holosystolic murmur at the apex. Her LDL and NTproBNP was elevated. ECG and Chest XRay was supportive of chamber enlargement. 2D Echo showed concentric LVH with systolic anterior motion of anterior mitral leaflet suggestive of HOCM, posterior mitral leaflet prolapse, and moderate MR with dilated LA. Angiogram showed 2-vessel coronary artery disease with coronary artery aneurysms involving the left main coronary artery. A selective aortogram showed suspicious stenosis above the aortic root. Hemodynamic studies showed an LV-aorta and intra-LV pressure gradient. A cMR confirmed the presence of hourglass SVAS at the STJ, with no typical findings of HOCM. She is scheduled for mitral valve replacement, coronary artery bypass grafting with coronary aneurysmectomy, and patch aortoplasty.

Conclusion

This is the first reported case of a patient with concomitant CAD, CAA, SVAS, and severe MR with MVP. The surgical plan is a mitral valve replacement, coronary artery bypass grafting with aneurysmectomy, and ascending aorta patch aortoplasty.

Atherosclerotic cardiovascular disease and rheumatic heart disease remains a significant cause of morbidity and mortality in the Philippines. Recognition of these diseases warrants adequate treatment and primary prevention in order to mitigate possible complications. Here we present a case of a 66 year old female with rare complications of these two conditions.