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Division
East Florida
Hospital
HCA Florida Westside Hospital
Specialty
Internal Medicine
Document Type
Poster
Publication Date
2024
Keywords
ventricular tachycardia, transthyretin amyloid cardiomyopathy, case report
Disciplines
Cardiology | Cardiovascular Diseases | Internal Medicine | Medicine and Health Sciences
Abstract
Objective: This case study aims to highlight the mechanisms, clinical presentation, diagnostic strategies, and management approach utilized for recurrent non-sustained ventricular tachycardias in a patient with suspected cardiac amyloidosis.
Background: Amyloidosis is a group of disorders characterized by the abnormal deposition of amyloid proteins in tissues and organs throughout the body. These proteins aggregate into insoluble fibrils that disrupt normal cellular function, leading to a range of clinical manifestations depending on the affected organs. Cardiac amyloidosis is characterized by the deposition of amyloid fibrils specifically in the myocardial tissue, leading to restrictive cardiomyopathy, progressive heart failure and arrhythmias. Ventricular tachycardia (VT) in amyloidosis presents significant clinical challenges due to the complex pathophysiology, increased arrhythmogenic potential associated with amyloid infiltration of the myocardium and the increased probability of sudden cardiac death.
Case Summary: We present the case of a 73-year-old male with a medical history of hypertension, diabetes mellitus, transient ischemic attack, transthyretin amyloidosis, and sporadic monomorphic ventricular tachycardias. He was scheduled for an outpatient cardiac MRI but was hospitalized to our facility before that. Initially admitted for facial numbness, the patient experienced multiple episodes of sustained ventricular tachycardia during the hospital stay. Given this clinical course, once stroke was ruled out and medical records were reviewed, the patient was successfully treated with an implantable cardioverter-defibrillator (ICD) to reduce the frequency of ventricular arrhythmias, enhance survival prospects, and lower the risk of sudden cardiac death.
Discussion: Patients with cardiac amyloidosis often present with symptoms related to heart failure, including exertional dyspnea, peripheral edema, and fatigue. Other manifestations may include syncope, arrhythmias such as atrial fibrillation or ventricular tachycardia, and conduction disturbances. Our patient had a history of sporadic ventricular tachycardia, and echocardiography showing decrease in LVEF from 55% to 45% but no symptoms of heart failure or syncopal episodes.
Key diagnostic tools for cardiac amyloidosis include ECG, Echocardiography, cardiac Magnetic Resonance Imaging (cMRI), biomarkers (troponin and N-terminal pro-B-type natriuretic peptide (NT-proBNP) and endomyocardial biopsy with Congo red staining. Our patient had past medical records demonstrating that biopsy done for carpal tunnel syndrome was positive for transthyretin amyloidosis (ATTR).
Treatment strategies may include: 1. Tafamidis which reduces the production and promotes clearance of the amyloid protein (ATTR). 2. Diuretics, Beta blockers and angiotensin-converting enzyme (ACE) inhibitors to manage heart failure. 3. Antiarrhythmic medications, catheter ablation, and the use of implantable cardioverter-defibrillators (ICDs) may be necessary to manage arrhythmias and prevent sudden cardiac death. This was the case with our patient. 4.There are advanced therapies where heart transplantation may be considered, particularly when cardiac involvement is the predominant manifestation and systemic amyloid burden is controlled.
Conclusion: Ventricular tachycardias in cardiac amyloidosis represent a significant clinical entity with high morbidity and mortality. Early recognition and comprehensive management, involving both antiarrhythmic strategies and disease-modifying treatments, are essential for improving patient outcomes. Further research is needed to optimize therapeutic protocols and explore novel interventions in this patient population
Original Publisher
HCA Healthcare Graduate Medical Education
Recommended Citation
Singh, Chinar; Xie, Meng; Choudhry, Poonam; and Jawaid, Asif, "Recurrent Non-Sustained Ventricular Tachycardias in a Patient with Suspicion of Transthyretin Amyloid Cardiomyopathy: A Case Report" (2024). East Florida Division GME Research Day 2024. 41.
https://scholarlycommons.hcahealthcare.com/eastflorida2024/41