Korean Circ J.  2011 Apr;41(4):209-212. 10.4070/kcj.2011.41.4.209.

A Case of a Senile Systemic Amyloidosis Patient Presenting With Angina Pectoris and Dilated Cardiomyopathy

• Kang GH
• Ryu DR
• Song PS
• Song YB
• Hahn JY
• Choi SH
• Gwon HC

• KMID: 2028946
• DOI: http://doi.org/10.4070/kcj.2011.41.4.209

Abstract

A 77-year-old man visited our hospital complaining of aggravated exertional chest pain. He was diagnosed with syndrome X 7 years ago and underwent medical treatment in a regional hospital. Coronary angiography and echocardiography did not show any significant abnormalities. On the seventh in-hospital day, cardiogenic shock developed and echocardiography showed a dilated left ventricular (LV) cavity and severe LV systolic dysfunction. We thus inserted an intra-aortic balloon pump for hemodynamic support and were forced to maintain it because of weaning failure several times. Finally, heart transplantation was the decided necessary procedure. After successful heart transplantation, the biopsy specimen revealed a wild-type transthyretin deposition indicating senile systemic amyloidosis in the intramuscular coronary vessels and interstitium. Cardiac biopsy at the 4-year follow-up showed no recurrence of amyloid deposition.

Keyword

Amyloidosis; Heart transplantation; Microvascular angina

MeSH Terms

Aged
Amyloidosis
Angina Pectoris
Biopsy
Cardiomyopathy, Dilated
Chest Pain
Coronary Angiography
Coronary Vessels
Echocardiography
Follow-Up Studies
Heart Transplantation
Hemodynamics
Humans
Microvascular Angina
Plaque, Amyloid
Prealbumin
Recurrence
Shock, Cardiogenic
Weaning
Prealbumin

Figure

• 

  Fig. 1 Echocardiographic findings. A: end diastolic period of previous exam (July 7, 2002). B: end systolic period of previous exam (July 7, 2002). C: end diastolic period of this exam (June 23, 2005). D: end systolic period of this exam (June 23, 2005).

• 

  Fig. 2 Gross finding of the extracted heart specimen. There are no mural thrombi. The right ventricle, intraventricular septum and left ventricle were 0.9, 1.7 and 1.5 cm in thickness, respectively, all of which were hypertrophied. The cut surface of the heart muscles shows no infarcts or fibrosis.

• 

  Fig. 3 Light microscopic findings of vascular amyloid deposition. A: hematoxylin-eosin staining shows deposition of pale-staining amorphous material in both blood vessel and interstitium (original magnification ×100). B: congo red staining with cross-polarized microscopy shows that a green birefringence characteristic of amyloid is apparent (original magnification ×100).

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