Abstract
Case report.
60-year-old man without previous history of coronary artery disease was admitted to our hospital. He complained of weakness, low extremities edema. Physical examination revealed also enlargement of the liver, positive hepatojugular reflux, multiple ecchymoses on patient’s face, especially in periorbital area. The arterial blood pressure was 100/60 mm Hg. ECG showed sinus rhythm 83/min, low R waves in V2, V3 with biphasic T waves in V4-V6. The last coronary angiography revealed normal coronary arteries, it was performed 6 months ago because of atypical chest pain and inconclusive stress test.
Echocardiography revealed severe tricuspid regurgitation (TR) due to leaflets restriction and malcoaptation. TR gradient was about 10 mm Hg. Interventricular septum 12 mm, left ventricle posterior wall 9 mm, mass index 87 g/m2, relative wall thickness 0.38; no LV regional wall motion abnormalities was noted, left ventricular ejection fraction (LV EF) was about 50%, mitral valve E/A ratio was 1,4, but average E/E" = 17. Left atrium volume 33 ml/m2. Insignificant amount of pericardial effusion also was found.
3D evaluation of tricuspid valve (TV) showed no leaflets defects and chordal ruptures. LV global longitudinal strain (LV GLS) was – 11,1 % with the apex/(mid + base) ratio 1,3 - apical sparing pattern. Cardiac MRI with gadolinium also showed severe TR and diffuse late subendocardial gadolinium enhancement in both ventricles. We suspected cardiac amyloidosis with significant tricuspid valve involvement, torrential TR, and right ventricle volume overload. The rectum biopsy was negative. The cardiac muscle biopsy with congo red straining was positive for amyloidosis. The patient was transferred to hematology clinic where the diagnosis of AL-amyloidosis was confirmed. The treatment with lenalidomide and prednisone was started. Unfortunately, one month later the patient died. The autopsy was not performed due to religious reasons.
Discussion
AL-amyloidosis is a systemic disease characterized by multiple organ and tissue changes and associated with poor prognosis. Cardiac involvement is a major prognostic factor as it accounts for approximately 75% of death due to heart failure or arrhythmias. Infiltration of myocardium with amyloid leads to diastolic than systolic dysfunction of the heart and to developing of the heart failure. The most common presentation of heart involvement in AL-amyloidosis is fatigue and dyspnea. In this case the main complaint of the patient was peripheral edema, echocardiography showed damaged TV and preserved LV systolic function. In literature, we found only one case report describing cardiac amyloidosis presented as severe TR.
Conclusion
In patients presenting with significant isolated valvular dysfunction and heart failure the cardiac amyloidosis can be suspected. The comprehensive echocardiography is the most useful tool to detect this problem.
Abstract P1684 Figure. 3D picture of tricuspid valve
Unsuccessful Transfemoral Tricuspid Valve-in-Ring Implantation: Case Report and Literature Review
