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Curso de Atualização em Medicina Cardiovascular 2019
Reunião Anual Conjunta dos Grupos de Estudo de Cirurgia Cardíaca, Doenças Valvulares e Ecocardiografia da SPC
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0 Topics
A. Basics
B. Imaging
C. Arrhythmias and Device Therapy
D. Heart Failure
E. Coronary Artery Disease, Acute Coronary Syndromes, Acute Cardiac Care
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
G. Aortic Disease, Peripheral Vascular Disease, Stroke
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01. History of Cardiology
02. Clinical Skills
03. Imaging
04. Arrhythmias, General
05. Atrial Fibrillation
06. Supraventricular Tachycardia (non-AF)
07. Syncope and Bradycardia
08. Ventricular Arrhythmias and Sudden Cardiac Death (SCD)
09. Device Therapy
10. Chronic Heart Failure
11. Acute Heart Failure
12. Coronary Artery Disease (Chronic)
13. Acute Coronary Syndromes
14. Acute Cardiac Care
15. Valvular Heart Disease
16. Infective Endocarditis
17. Myocardial Disease
18. Pericardial Disease
19. Tumors of the Heart
20. Congenital Heart Disease and Pediatric Cardiology
21. Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure
22. Aortic Disease
23. Peripheral Vascular and Cerebrovascular Disease
24. Stroke
25. Interventional Cardiology
26. Cardiovascular Surgery
27. Hypertension
28. Risk Factors and Prevention
29. Rehabilitation and Sports Cardiology
30. Cardiovascular Disease in Special Populations
31. Pharmacology and Pharmacotherapy
32. Cardiovascular Nursing
33. e-Cardiology / Digital Health
34. Public Health and Health Economics
35. Research Methodology
36. Basic Science
37. Miscellanea
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Abstract
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CLEAR FILTERS
When the heart is the key to Pandora’s box
Session:
Sessão de Casos Clínicos - I
Speaker:
Cátia Costa Oliveira
Congress:
CPC 2019
Topic:
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Theme:
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Subtheme:
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Session Type:
Sessão de Casos Clínicos
FP Number:
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Authors:
Cátia Costa Oliveira; Isabel Durães Campos; Catarina Vieira; Carla Rodrigues; Paulo Medeiros; Carlos Galvão Braga; Cristina Ângela; Herlander Marques; Alberto Salgado; Jorge Marques
Abstract
<p>A 67 years old woman with a previous history of hypertension, atrial fibrillation (AF) and known severe concentric hypertrophy (more pronounced at the interventricular septum) with moderate pulmonary hypertension and diastolic dysfunction of the left ventricle (LV) resorted to the urgency service for weight loss, asthenia and dyspnea for minor efforts. Her physical examination was innocent. Her ECG showed AF and low voltage in limb and precordial leads. Patient was admitted at the Cardiology Department for investigation. A new TTE revealed fast progression of the hypertrophic pattern (IVS 26 mm, and posterior wall 18 mm) with a rest outflow tract gradient of 18 mmHg; free right ventricular wall was also hypertrophied with 12 mm and a type II diastolic dysfunction was present. A complete investigation for ventricular hypertrophy and constitutional syndrome was done. CMR demonstrated preserved biventricular systolic function, LV hypertrophy with septal predominance (24 mm) with elevated myocardial mass indexed to body mass (114 g/m2) and late difuse subendocardial gadolinium enhancement. (99m)Tc -DPD scintigraphy was negative for ATTR; Anderson-Fabry disease was excluded by genetic testing. Blood analysis revealed a normocytic/ normochromic anemia and a disproportional ratio of free light immunoglobulin chains K/λ, with a predominance of lambda chains. β2-microglobulin was also elevated. 24 hours urine analyses were normal. The hypothesis of non-secretor multiple myeloma (MM) with light chain immunoglobulin amyloidosis (AL) was then admitted. The patient underwent bone marrow and salivary gland biopsies which confirmed MM and deposition of amyloid, respectively. The FISH analyses demonstrated a delection of RB1 gene on 13q14.3 sequence and of TP53 on 17p13.1, both associated with a poorly prognosis in MM. The patient was referred for oncology where she is currently undergoing chemotherapy for MM and AL amyloidosis.</p> <p>Amyloidosis represents a large spectrum of systemic diseases. Heart failure may be its first sign. This case reflects the diagnostic work-up in the presence of a hypertrophic LV pattern as well as the importance of high clinical suspicious and early diagnosis. While AL amyloidosis occurs in isolation, 10% of patients with multiple myeloma develop systemic AL amyloid with cardiac involvement. The distinction between these entities is not only semantic, but has extremely important prognosis and therapeutic considerations.</p>
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