A SUSPICIOUS CASE OF HEART DYSFUNCTION
DOI:
https://doi.org/10.58043/rphrc.25Keywords:
cardiac amyloidosis, pleural effusion, diagnosisAbstract
Introduction: Amyloid fibrils are polymers of proteins involved in various physiological processes. When there is an accumulation of defective amyloid fibrils, a condition called amyloidosis occurs. This can have several subtypes.
Case Report: The authors report a case of a 78-year-old male, independent in his daily life, came to the medical center complaining of aggravating dyspnea in the last months. He has consultations regularly in his medical center with this history: congestive heart failure NYHA class III, hyperuricemia, permanent atrial fibrillation, controlled essential hypertension and a pleural effusion two months earlier. In physical examination we suspected a right pleural effusion. He was sent to hospital’s emergency room for guidance. There he was admitted and stayed for 2 months in investigation regarding etiology of these pleural effusions. The pleural fluid and blood were analyzed, as well as thoracic-abdominal-pelvic CT scans were ordered to further investigate, among other exams. Nothing was found. A few months later he had another pleural effusion in a consultation and a cardiac magnetic resonance was ordered showing very typical signs of cardiac amyloidosis. Later we sent the patient to hospital for further investigation of a possible systemic amyloidosis, with an abdominal fat biopsy that concluded for the most likely diagnosis of amyloidosis. The patient is currently under further specification of amyloidosis type.
Discussion: In family medicine we tend to think first of the most likely diagnosis for our patient’s complaints. However, sometimes, the investigation should consider other rare causes for recurrent problems. A family physician is in the front line to think of the patients as a whole, both along time and also general physiology.
Downloads
References
Witteles RM. Cardiac Amyloidosis - American College of Cardiology [Internet]. 2016
COHEN A, Kyle RA. History of amyloidosis. J Intern Med [Internet]. 1992 Dec 1 232(6):509–10.
Rapezzi C, Quarta CC, Riva L, Longhi S, Gallelli I, Lorenzini M, et al. Transthyretin-related amyloidoses and the heart: A clinical overview [Internet]. Vol. 7, Nature Reviews Cardiology. Nat Rev Cardiol; 2010. p. 398–408.
Falk RH, Comenzo RL, Skinner M. The systemic amyloidoses. N Engl J Med [Internet]. 1997 Sep 25;337(13):898–909.
Desai H V., Aronow WS, Peterson SJ, Frishman WH. Cardiac amyloidosis: Approaches to diagnosis and management [Internet]. Vol. 18, Cardiology in Review. Cardiol Rev; 2010. p. 1–11.
Halwani O, Delgado DH. Cardiac amyloidosis: An approach to diagnosis and management [Internet]. Vol. 8, Expert Review of Cardiovascular Therapy. Expert Reviews Ltd.; 2010. p. 1007–13.
Wechalekar AD, Goodman HJB, Lachmann HJ, Offer M, Hawkins PN, Gillmore JD. Safety and efficacy of risk-
adapted cyclophosphamide, thalidomide, and dexamethasone in systemic AL amyloidosis. Blood [Internet]. 2007 Jan 15;109(2):457–64.
Jorge AJL, Ávila DX de, Vilar EG, Ribeiro ML, Bruno KEH, Pires AC. Cardiac Amyloidosis with Heart Failure and Middle Range Ejection Fraction. Int J Cardiovasc Sci [Internet]. 2018; 31(4):457–60.
Maceira AM, Joshi J, Prasad SK, Moon JC, Perugini E, Harding I, et al. Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation [Internet]. 2005 Jan 18;111(2):186– 93.
Shah KB, Inoue Y, Mehra MR. Amyloidosis and the heart: A comprehensive review [Internet]. Vol. 166, Archives of Internal Medicine. Arch Intern Med; 2006. p. 1805–13.
Lachmann HJ, Booth DR, Booth SE, Bybee A, Gilbertson JA, Gillmore JD, et al. Misdiagnosis of hereditary amyloidosis as AL (primary) amyloidosis. N Engl J Med [Internet]. 2002 Jun 6; 346(23):1786–91.
Fernandes A, Caetano F, Almeida I, Paiva L, Gomes P, Mota P, et al. Amiloidose cardíaca - abordagem diagnóstica, a propósito de um caso clínico. Rev Port Cardiol. 2016 May 1;35(5):305.e1-305.e7.