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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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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
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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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CLEAR FILTERS
From Ambiguity to Clarity: The Critical Role of CMR in Diagnosing an Elusive Cardiomyopathy
Session:
SESSÃO DE CASOS CLÍNICOS DE MIOCARDIOPATIAS, VALVULOPATIAS, DOENÇAS DO PERICÁRDIO, CARDIOPATIAS CONGÉNITAS
Speaker:
Andre Lobo
Congress:
CPC 2025
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
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Subtheme:
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Session Type:
Sessão de Casos Clínicos
FP Number:
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Authors:
Andre Lobo; Mariana Brandão; Marta Catarina Almeida; Marisa Silva; Pedro Braga; Nuno Dias Ferreira; Ricardo Fontes-Carvalho
Abstract
<p>A 64-year-old female with multiple cardiovascular risk factors was referred due to exertional dyspnea and chest pain. Transthoracic echocardiography (TTE) revealed asymmetrical left ventricular hypertrophy (LVH) with septal predominance (13 mm), right ventricular hypertrophy, preserved left ventricular ejection fraction (LVEF), elevated filling pressures (E/e’: 17), and a small pericardial effusion.</p> <p>CCTA identified an 80% mid-LAD lesion, subsequently confirmed by invasive coronary angiography and treated with a drug-eluting stent. Despite successful revascularization, the patient’s symptoms persisted.</p> <p>CMR confirmed LVH, with a maximal wall thickness of 15 mm in the mid inferoseptal segment, and an LVEF of 71%. Tissue characterization revealed diffuse subendocardial late gadolinium enhancement (LGE) involving all left ventricular segments. Tissue mapping was also noteworthy for markedly elevated native T1 (1248 ms, MOLLI sequence, SIEMENS 1.5T) and a highly increased extracellular volume (ECV) of 45%.</p> <p>As CMR findings were highly suggestive of cardiac amyloidosis (CA), bone scintigraphy with 99mTc-DPD and hematologic tests were performed. The scan showed a Perugini score of zero, and hematologic tests were negative, deeming the diagnosis of CA unlikely. Due to the remaining diagnostic ambiguity, genetic testing with an HCM panel, including the most frequent phenocopies, was performed and returned negative results.</p> <p>Despite negative non-invasive tests, the patient’s clinical course, imaging findings, and thorough medical history—revealing bilateral carpal tunnel syndrome and lumbar spinal stenosis, both requiring surgical intervention years earlier—continued to raise strong suspicion for amyloidosis. An abdominal fat pad biopsy yielded no evidence of amyloid deposits. Consequently, an endomyocardial biopsy was performed in line with current recommendations, ultimately confirming the presence of myocardial amyloid deposits. This definitive diagnosis of CA allowed for the timely initiation of Tafamidis therapy.</p> <p>Currently, CA diagnosis mainly relies on the non-invasive algorithm proposed by Gilmore et al. While CMR is not considered a mandatory technique in this pathway, it played a central role in this case, with typical tissue characterization findings making the diagnosis very likely, even in the presence of negative scintigraphy. Despite the high sensitivity of nuclear imaging for ATTR diagnosis, this case underscores the importance of pursuing histologic confirmation with EMB when clinical suspicion remains high, as stated in the guidelines, as it may allow the initiation of disease-modifying therapy. The importance of CMR and the added value of mapping techniques are highlighted in this case.</p>
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