Author + information
- Received February 12, 2016
- Revision received March 4, 2016
- Accepted March 18, 2016
- Published online August 1, 2016.
- Khawaja Afzal Ammar, MBBS, MSa,∗ (, )
- Bijoy K. Khandheria, MDa,
- Tanvir Bajwa, MDa,
- Steven C. Port, MDa,
- Suhail Allaqaband, MDa,
- Renuka Jain, MDa,
- Gary Neitzel, MDb and
- A. Jamil Tajik, MDa
- aAurora Cardiovascular Services, Aurora Sinai/Aurora St. Luke’s Medical Centers, Milwaukee, Wisconsin
- bDepartment of Pathology and Laboratory Medicine, Aurora St. Luke’s Medical Center, Milwaukee, Wisconsin
- ↵∗Reprint requests and correspondence:
Dr. Khawaja Afzal Ammar, Aurora Cardiovascular Services, Aurora St. Luke’s Medical Center, 2801 West Kinnickinnic River Parkway, Suite 840, Milwaukee, Wisconsin 53215.
Although mild to moderate mitral regurgitation is common in cardiac amyloidosis (CA), severe mitral regurgitation as the primary presenting finding is rare (1). We present 3 such cases of patients with severe mitral regurgitation who on further workup were discovered to have CA (2) (Figures 1, 2, 3, 4, 5, and 6). We found atypical nodularity along the coaptation margin of the anterior mitral leaflets as a mechanism behind severe mitral regurgitation, likely from local amyloid infiltration, creating an “amyloidoma.” Because multimodality imaging provided further evidence in favor of CA, the initial Congo red staining results, negative for CA, were ignored. Advanced histological techniques confirmed the diagnosis of CA. All cases markedly improved with unconventional therapeutic approaches (percutaneous mitral valve repair n = 2, and cardiac transplantation n = 1). This case series provides multiple insights regarding diagnosis of CA: inclusion of CA in the differential in patients presenting primarily with severe mitral regurgitation; utilization of multiple imaging modalities to increase the burden of evidence; and advanced histological techniques in order to avoid false-negative Congo red stain results.
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received February 12, 2016.
- Revision received March 4, 2016.
- Accepted March 18, 2016.
- American College of Cardiology Foundation