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Donnellan, Eoin, Oussama Wazni, Mohamed Kanj, Mohamed B. Elshazly, Ayman Hussein, Bryan Baranowski, Mazen Hanna, et al. "Atrial fibrillation ablation in patients with transthyretin cardiac amyloidosis." EP Europace 22, no. 2 (November 14, 2019): 259–64. http://dx.doi.org/10.1093/europace/euz314.
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Abstract Aims Atrial fibrillation (AF) occurs in as many as 70% of patients with transthyretin cardiac amyloidosis (ATTR CA). The aim of our study was to investigate the impact of AF ablation on freedom from recurrent arrhythmia, hospitalization for AF or heart failure (HF), and mortality. Methods and results This was a retrospective observational cohort study of 72 patients with ATTR CA and AF, of whom 24 underwent AF ablation and were matched in a 2:1 manner based on age, gender, ATTR CA stage, New York Heart Association functional class, ejection fraction, and date of AF diagnosis with 48 patients with ATTR CA and AF undergoing medical management. During a mean follow-up of 39 ± 26 months, 10 (42%) patients remained free of recurrent arrhythmia following ablation. Ablation was significantly more effective in those with Stage I or II ATTR CA, with 9/14 (64%) patients with Stage I or II ATTR CA remaining free of recurrent arrhythmia compared to only 1/10 (10%) patients with Stage III disease (P = 0.005). Death occurred in 7 (29%) patients in the ablation group compared to 36 (75%) in the non-ablation arm (P = 0.01). Rates of ischaemic stroke were similar in both groups. Ablation was associated with a significant reduction in the frequency of hospitalization for HF/arrhythmia (1.7 ± 2.4 hospitalizations vs. 4 ± 3.5, P = 0.005). On Cox proportional hazards analyses, ablation was associated with improved survival (hazard ratio 0.38, 95% confidence intervals 0.17–0.86; P = 0.02). Conclusion Atrial fibrillation ablation is associated with reduced mortality in ATTR CA and is most effective when performed earlier during the disease process.
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Nagarakanti, Rangadham, Kesavan Sankaramangalam, Sindhu Nagarakanti, Danyaal Moin, Kenneth Dulnuan, and Deepa B. Iyer. "Cardiac Amyloidosis Wild Type (ATTR-CAwt) and Associated Cardiac Arrhythmias: A Case Report and Literature Review." Indian Journal of Clinical Cardiology 2, no. 2 (March 8, 2021): 80–85. http://dx.doi.org/10.1177/2632463621998583.
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Cardiac amyloidosis wild type (ATTR-CAwt) is often the underdiagnosed cause of heart failure with preserved ejection fraction (HFpEF) and has a high mortality rate. There is usually a long delay between the appearance of clinical signs and the diagnosis of ATTR-CAwt but a short duration between diagnosis and death. ATTR-CAwt was associated with significant clinical arrhythmias. We report a case of ATTR-CAwt, the process of its diagnosis, and the associated clinical arrhythmias and their management. We also reviewed the literature of this underdiagnosed and potentially fatal condition and the current existing therapies.
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Khayambashi, S., C. Hahn, N. Fine, E. Mahe, and K. Elzinga. "P.045 Biopsies of the transverse carpal ligament and tenosynovium for tissue confirmation of transthyretin amyloidosis." Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 49, s1 (June 2022): S19. http://dx.doi.org/10.1017/cjn.2022.146.
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Background: Transthyretin Amyloidosis (ATTR) is a common cause of both cardiomyopathy and carpal tunnel syndrome, with many patients needing carpal tunnel release (CTR). Although tafamidis is now an approved treatment of ATTR cardiomyopathy, insurers in most provinces require biopsy confirmation of amyloidosis. Endomyocardial biopsy is often the chosen approach due to optimal sensitivity, albeit with risk of serious adverse events such as stroke, cardiac tamponade, and arrhythmias. CTR may present an ideal opportunity for obtaining amyloidosis biopsy confirmation. Methods: ATTR patients undergoing CTR had biopsy of their transverse carpal ligament (TCL) and/or flexor tenosynovium to assess the sensitivity of both sites for biopsy confirmation of amyloidosis. Results: Twelve patients consecutively underwent biopsies during CTR, with 4 (33%) having bilateral CTR and biopsy. Among 16 TCL biopsies and 14 tenosynovium biopsies, 100% demonstrated amyloid deposition. Another patient had isolated tenosynovium biopsy without CTR and also demonstrated amyloidosis. There were no serious adverse events, and 1/13 (8%) had wound dehiscence requiring repeat suturing. Conclusions: Biopsy of the TCL and/or tenosynovium during CTR is safe, cost-effective, and sensitive, and may represent an alternative to endomyocardial biopsy in patients requiring tissue confirmation for tafamidis approval. ATTR patients eligible for tafamidis may benefit from early neurology assessment.
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Aouate, David, Aymeric Menet, Dimitri Bellevre, Thibaud Damy, and Sylvestre Marechaux. "Deleterious effect of right ventricular pacing in patients with cardiac transthyretin amyloidosis: potential clinical benefit of cardiac resynchronization therapy." European Heart Journal - Case Reports 4, no. 3 (May 1, 2020): 1–5. http://dx.doi.org/10.1093/ehjcr/ytaa088.
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Abstract Background Cardiac amyloidosis involvement is associated with a detrimental outcome including frequent arrhythmias, heart failure, and conduction disturbances which may need permanent pacing. Cases summary We report two cases of patients with transthyretin amyloidosis (ATTR) who developed heart failure and depressed left ventricular ejection fraction (LVEF) following permanent right ventricular (RV) pacing but highly responded to cardiac resynchronization therapy (CRT). Discussion The impact of RV pacing and CRT in cardiac amyloidosis is not known. In our cases, the detrimental effect of permanent RV pacing on left ventricular (LV) systolic function and heart failure symptoms was suggested by both permanent RV pacing mediated functional and LV function decline and LV systolic dysfunction reversal following CRT along with QRS width reduction. Whether cardiac resynchronization should be readily recommended in ATTR patients who need ventricular pacing whatever the LVEF deserves further investigation.
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Juarez, Michel, Gaspar Del Rio-Pertuz, Kanak Parmar, Melanie C. Bois, Scott Shurmur, and Erwin Argueta-Sosa. "A Case of Early Hereditary Transthyretin Amyloid Cardiomyopathy Recognition With Genetic Screening: A Case Report." Journal of Primary Care & Community Health 13 (January 2022): 215013192110626. http://dx.doi.org/10.1177/21501319211062682.
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Background: Transthyretin amyloid cardiomyopathy (ATTR-CM) is one of the most common types of cardiac amyloidosis. Amyloid cardiomyopathy more commonly affects men, elderly, and 3% to 4% of the African-American population. ATTR-CM suspicion and diagnosis is challenging; however, awareness of the disease is increasing, and best practices to identify it are being proposed. The approach to suspected cases of ATTR-CM relies on the presence of heart failure, red flag signs and symptoms, and age >65 or >70 for men and women respectively. Little is known about cases when it presents in early ages. Case: We report a 62-year-old African American male with past medical history of hyperlipidemia, prostate cancer, hypertension, bilateral carpal tunnel surgery that had debuted with a cardiac arrhythmia at age 55 and was diagnosed with heart failure several years later. Restrictive cardiomyopathy was suspected, and genetic screening was sent for ATTRm which confirmed a pathogenic trasnthyretin gene mutation. Endomyocardial biopsy was performed which confirmed cardiac amyloid deposition. Discussion: ATTR-CM is a rare disease with an increasing prevalence. Cases with out of proportion signs and symptoms of heart failure with preserved ejection fractions should raise the suspicion of ATTR-CM despite age.
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Kim, Eun-Jeong, Benjamin B. Holmes, Shi Huang, Ricardo Lugo, Asad Al Aboud, Stacey Goodman, Rebecca R. Hung, et al. "Outcomes in patients with cardiac amyloidosis and implantable cardioverter-defibrillator." EP Europace 22, no. 8 (June 8, 2020): 1216–23. http://dx.doi.org/10.1093/europace/euaa094.
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Abstract Aims Cardiac amyloidosis (CA) is associated with increased mortality due to arrhythmias, heart failure, and electromechanical dissociation. However, the role of an implantable cardioverter-defibrillator (ICD) remains unclear. We conducted case-control study to assess survival in CA patients with and without a primary prevention ICD and compared outcomes to an age, sex, and device implant year-matched non-CA group with primary prevention ICD. Methods and results There were 91 subjects with CA [mean age= 71.2 ± 10.2, female 22.0%, 49 AL with Mayo Stage 2.9 ± 1.0, 41 transthyretin amyloidosis (ATTR), 1 other] followed by Vanderbilt Amyloidosis centre. Patients with ICD (n = 23) were compared with those without (n = 68) and a non-amyloid group with ICD (n = 46). All subjects with ICD had implantation for primary prevention. Mean left ventricular ejection fraction was 36.2% ± 14.4% in CA with ICD, 41.0% ± 10.6% in CA without ICD, and 33.5% ± 14.4% in non-CA patients. Over 3.5 ± 3.1 years, 6 (26.1%) CA, and 12 (26.1%) non-CA subjects received ICD therapies (P = 0.71). Patients with CA had a significantly higher mortality (43.9% vs. 17.4%, P = 0.002) compared with the non-CA group. Mean time from device implantation to death was 21.8 months in AL and 22.8 months in ATTR patients. There was no significant difference in mortality between CA patients who did and did not receive an ICD (39.0% vs. 46.0%, P = 0.59). Conclusions Despite comparable event rates patients with CA had a significantly higher mortality and ICDs were not associated with longer survival. With the emergence of effective therapy for AL amyloidosis, further study of ICD is needed in this group.
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Antonelli, Giorgio, Valeria Cammalleri, Valeria Maria De Luca, Ombretta Annibali, Annunziata Nusca, Simona Mega, Myriam Carpenito, et al. "957 EMERGING FROM THE DARKNESS. SUDDEN CARDIAC DEATH IN CARDIAC AMYLOIDOSIS." European Heart Journal Supplements 24, Supplement_K (December 14, 2022). http://dx.doi.org/10.1093/eurheartjsupp/suac121.581.
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Abstract Cardiac amyloidosis (CA) manifests as infiltrative cardiomyopathy with a hypertrophic pattern, usually presenting with heart failure with preserved ejection fraction (HFpEF). In addition, degenerative valvular heart disease, particularly severe aortic stenosis, is commonly seen in patients with CA. However, amyloid fibril deposition may also infiltrate the conduction system and promote the development of electrical disorders, including ventricular tachyarrhythmias (VT), atrio-ventricular block, or acute electromechanical dissociation (EMD). These manifestations can increase the risk of sudden cardiac death (SCD). This review summarizes the pathophysiological mechanisms and risk factors for sudden cardiac death in cardiac amyloidosis and focuses on the major current concerns regarding medical and device management in this challenging scenario. Heart involvement is the major determinant of survival in patient with amyloidosis and a “sudden death” occurs in approximately two-thirds of patients with cardiac amyloidosis. Moreover, the AL amyloid fibrils are shown to be highly cytotoxic to the ventricular myocardial, explaining why ventricular arrhythmias appear more frequently in AL over ATTR. Proposed mechanisms driving electrophysiological manifestations of CA involve intramural coronaries, microvascular ischemia, or patchy myocardium infiltration of amyloid fibrils, causing the development of anatomical re-entrant circuits responsible for ventricular arrhythmia. The prevalence of non-sustained ventricular tachycardia (NSVT) in AL amyloidosis ranges from 5 to 27% with routine monitoring and 100% during the stem cell transplant period. The guidelines don't provide specific indications regarding insertion of implantabile cardioverter defibrillator (ICD) and they don't clarify whether some patients subtypes can benefit from them. Amyloid infiltration into the conduction system enhances the genesis of rhythm disturbances, including fatal ventricular arrhythmias and SCD. Current pharmacological anti-arrhythmic therapies are poorly tolerated in CA, and there are no robust recommendations on the management of ventricular arrhythmias in this subset of patients. Furthermore, the benefit of ICD implantation is highly variable according to the different clinical stages of the disease. Therefore, further studies are needed to create a standardized diagnostic algorithm and appropriate treatment strategy for this special population.
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Torselletti, Lorenzo, Stronati Giulia, Alessandro Barbarossa, Giuseppe Ciliberti, Francesca Coretti, Sara Belleggia, Francesca Coraducci, et al. "178 ARRHYTHMIC STORM IN ATTR WILD TYPE AMYLOIDOSIS: AN UNUSUAL COMBINATION." European Heart Journal Supplements 24, Supplement_K (December 14, 2022). http://dx.doi.org/10.1093/eurheartjsupp/suac121.087.
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Abstract Cardiac amyloidosis (CA) is characterized by extracellular protein fibril deposition in the myocardium leading to restrictive heart failure. Both atrial and ventricular arrhythmias are common in CA. Study have shown up to one half of patients with CA die suddenly. However, the most common cause of sudden death has been historically through to be secondary electromechanical dissociation rather than a lethal ventricular arrhythmia. We present the case of a 84 years old man, with a history of hypertension, dyslipidaemia, and prior smoking. In June 2020 the patient was admitted to the emergency room of our hospital due to an episode of hemodynamically unstable sustained ventricular tachycardia (SVT). The patient underwent electrical cardioversion with restoration of sinus rhythm. An echocardiogram that showed a slightly reduced ejection fraction (FE = 45%), severe concentric hypertrophy, grade 3 diastolic dysfunction with high pressures in the left ventricular (LV) cavity, and a reduced GLS (-13.8%) with a typical apical-sparing aspect. A cardiac magnetic resonance (Fig.1). was performed showing a diffuse area of LGE with a subepicardial pattern involving left ventricular and atrial segments, compatible with myocardial storage disease. In order to complete the diagnostic workout, we performed a bone scintigraphy (Fig.2). (Positive for CA, with Perugini score grade 2), a genetic test (negative for hATTR-CA mutations) and free light chain in serum (negative for AL-CA). The patient was discharged at home after ICD implantation in secondary prevention and prescribed appropriate heart failure therapy. In May 2022 the patient was readmitted in emergency room for dyspnea during an arrhythmic storm characterized by several SVTs and ICD interventions, and atrial fibrillation (FA). After stabilization of clinical parameters, the patient was hospitalized in our ward. We optimized medical therapy with metaprololo 100 mg 1 cp BID, cordarone 600 mg ev and mexiletina 200 mg cp BID. During the hospitalization we succeeded in reducing SVT burden. However, ventricular PVCs and slow SVT remained. For this reason, we decided to perform an electrophysiological study (EPS) followed by catheter ablation. The EPS found two low voltage areas (Fig.3). The first were found under the aortic valve; the second was an area of dense scar and fragmented potentials along the basal-posterior wall. After ablation of the first area we succeeded in removing clinical PVCs. During the ablation of the second area the procedure was complicated by ventricular fibrillation that required advanced life support and many electrical shocks. The patient was discharged at home in absence of further episodes of SVTs with optimized therapy At a three moths follow up no arrhythmic events were recorded.
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D'Agostino, S., G. Gizzi, and V. Parato. "P289 EYESHOT OF CARDIAC AMYLOIDOSIS CASES PRESENTED TO A CARDIOLOGY UO IN CENTRAL ITALY IN 2021." European Heart Journal Supplements 24, Supplement_C (May 1, 2022). http://dx.doi.org/10.1093/eurheartj/suac012.278.
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Abstract Introduction ATTR–related cardiac amyloidosis, caused by the accumulation of insoluble fibrillar proteins derived from transthyretin misfolding, is an often underdiagnosed pathology, especially when neurological involvement is absent or minor. Transthoracic echocardiography represents the first fundamental step in the diagnostic process. Abstract During the year 2021, a total of 9 patients with an echocardiographic feature of hypertrophic heart disease suggestive of infiltrative pathology were referred to our Cardiology unit. Of these patients, 89% were male (8 out of 9), aged between 60 and 88 years (average age of 72 years). The clinic presentation was heart failure in 5 cases (55% of the total), arrhythmic storm in 1 case, history of amyloid polyneuropathy in 1 case and it was an occasional finding in 2 patients who required a cardiological evaluation for other reasons. The 12–lead ECG documented atrial fibrillation arrhythmia in 4 out of 9 cases (44%) and sinus rhythm in the remaining 5 cases (56%). Transthoracic echocardiography revealed left ventricular hypertrophy with granular sparkling aspect of the SIV in all patients, with indexes of global systolic function preserved in 4 out of 9 cases (44%), moderately reduced in 3 out of 9 cases (33%) and severely reduced in the remaining 2 cases (22%). Diastolic dysfunction was present in all patients, with a restrictive pattern in 4 of 9 cases (44%). Myocardial scintigraphy with DPD was performed in 7 patients, which confirmed the suspicion of aTTR amyloidosis in 6 cases (Perugini Score = 2), in the remaining 2 patients the diagnostic procedure was not performed due to severe comorbidities. At present, diagnostic confirmation of aTTR amyloidosis has been confirmed by biopsy in 2 patients; a patient‘s biopsy is currently under analysis; 1 patient is on Patisiran therapy. Conclusions Cardiac amyloidosis represents a frequent clinical condition especially in the elderly population. In the light of the most recent therapeutic strategies, a correct and early etiopathogenetic diagnosis is of fundamental importance.
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Cho, Jae Hyung, Natasha Cuk, Derek Leong, Ashkan Ehdaie, Eugenio Cingolani, Michael Shehata, and Jignesh Patel. "Abstract 16235: Rhythmic Disturbances in Patients With Cardiac Amyloidosis: Analysis From Cedars-Sinai Amyloidosis Registry." Circulation 142, Suppl_3 (November 17, 2020). http://dx.doi.org/10.1161/circ.142.suppl_3.16235.
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Introduction: Cardiac arrhythmias are frequent complications of patients with cardiac amyloidosis. The burden and nature of cardiac arrhythmias in patients with cardiac amyloidosis have not been investigated in a large patient population. Hypothesis: Cardiac amyloidosis is a highly arrhythmogenic disorder requiring implantation of cardiac electronic devices. Methods: Cedars-Sinai amyloidosis registry was analyzed to investigate the prevalence and nature of cardiac arrhythmias in patients with cardiac amyloidosis. Ambulatory ECG monitoring data, implantable cardioverter-defibrillator or pacemaker implantation, and interrogation data were reviewed to study the burden of cardiac arrhythmias in patients with cardiac amyloidosis. Results: A total of 156 patients were analyzed in the registry: 51 patients (32.7%) with AL amyloidosis, 101 patients (64.7%) with ATTR amyloidosis, and 4 patients with AA amyloidosis (2.6%). Thirty-seven patients (23.7%) were implanted with cardioverter-defibrillator; 23 patients (14.7%) for primary prevention, 11 patients (7.1%) for secondary prevention of ventricular tachycardia, and 3 patients (1.9%) for secondary prevention of ventricular fibrillation. Twenty-two patients (12.1%) needed pacemaker implantation; 9 patients (5.8%) for high-grade or complete heart block, and 13 patients (8.3%) due to sick sinus syndrome. The most common arrhythmias were atrial fibrillation occurring in 80 patients (51.3%) followed by first-degree AV block in 31 patients (19.9%). Sustained ventricular tachycardia occurred in 14 patients (8.9%) during hospitalization, device interrogation or ambulatory monitoring. Conclusions: Both atrial and ventricular arrhythmias are common manifestations of cardiac amyloidosis, frequently necessitating implantation of cardiac electronic devices.
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Zhang, K. W., R. Zhang, Y. Soyama, M. Karmpalioti, D. J. Lenihan, and J. Gorcsan. "P2724Diagnosis of transthyretin versus light chain cardiac amyloidosis by apical sparing strain ratio in patients with clinically suspected disease." European Heart Journal 40, Supplement_1 (October 1, 2019). http://dx.doi.org/10.1093/eurheartj/ehz748.1041.
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Abstract Background Apical sparing by longitudinal strain imaging has reported utility for the diagnosis of cardiac amyloidosis. However, potential differences in the apical sparing pattern in light chain (AL) versus transthyretin (ATTR) amyloidosis in patients with high clinical suspicion for cardiac amyloidosis is not clear. Purpose Our objective was to test the hypothesis that echocardiographic strain imaging could determine differences in patients with clinically suspected AL and ATTR cardiac amyloidosis. Methods We studied 206 patients, aged 64±11, with clinically suspected cardiac amyloidosis. Routine longitudinal strain imaging analyses was performed (EchoPAC, GE Healthcare) with bulls-eye plots. After 27 exclusions (8 arrhythmia/frame rate, 19 missing/poor images), there were 179 patients. Included were 129 patients with cardiac amyloid: 42 by endomyocardial biopsy, 4 by technetium pyrophosphate scan, 65 by non-cardiac biopsy with suggestive cardiac imaging (interventricular septal thickness ≥1.2cm by echocardiography or characteristic cardiac MRI findings), 15 with multiple myeloma and suggestive cardiac imaging, and 3 by autopsy; 50 patients had a negative endomyocardial biopsy or autopsy for cardiac amyloid. The apical sparing ratio by strain imaging was calculated as the (average of apical segments) / (average of mid segments + average of basal segments). Results Cardiac amyloidosis patients were 79% with AL and 21% with ATTR. Applying the previously published apical sparing ratio cut-off of 1.0 for longitudinal strain imaging, sensitivity and specificity were 29% and 78%, respectively, for diagnosis of cardiac amyloidosis. Applying a ratio cut-off of 0.81 improved sensitivity to 72% with specificity of 64% and area under the curve (AUC) of 0.66. Positive and negative predictive values were 85% and 46%, respectively, at this ratio cut-off. The apical sparing ratio was significantly higher in AL and ATTR as compared to the biopsy negative group (p<0.001). Furthermore, the apical sparing ratio was significantly higher in ATTR as compared to AL (p<0.05). Apical sparing pattern and ratio Conclusions Among patients with high clinical suspicion for cardiac amyloidosis, the apical sparing ratio by echocardiographic strain imaging can demonstrate differences for AL and ATTR cardiac amyloidosis and has potential for clinical utility.
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Fede, Giuseppe, Giuseppe Abate, Paolo Belluardo, Nicoletta Guccione, Giovanni Tasca, Maria Luisa Guarrella, Carmelo Di Tommasi, Guglielmo Piccione, Giuseppe Sulsenti, and Sabina Ficili. "515 Exertional syncope and ventricular arrhythmia in a patient with cardiac amyloidosis." European Heart Journal Supplements 23, Supplement_G (December 1, 2021). http://dx.doi.org/10.1093/eurheartj/suab141.002.
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Abstract Aims Systemic amyloidosis is a multisystem disorder caused by the misfolding, aggregation, and deposition of certain proteins in various organs and tissues. Cardiac involvement is common and worsens the prognosis. Atrial fibrillation is the most frequent arrhythmia in cardiac amiloidosis (CA), but many cases of ventricular arrhythmias (VA) and sudden cardiac death (SCD) have been described. Methods and results We present a case of a 64-year-old man admitted following exertional syncope occurred after climbing a flight of stairs. In the previous 2 weeks he had experienced fatigue and poor tolerance to exertion; moreover, 2 days before he had another episode of transient loss of consciousness after exertion. His medical history included left gonarthrosis and surgery for right carpal tunnel syndrome one year before. Physical examination did not revealed signs of heart failure. A 12-lead electrocardiogram (ECG) showed a pseudoinfarction pattern with QS waves in V2 and V3 leads and low voltage in all limb leads (Figure 1). Routine blood tests revealed N-terminal pro-bran natriuretic peptide (NT-ProBNP) of 3436 pg/ml (n.v. 0–125) and high-sensitive troponin T of 67 pg/ml (n.v. < 58). A trans-thoracic echocardiogram showed left ventricle (LV) concentric thickening wall with granular and sparkling pattern, mild reduced ejection fraction, reduced global longitudinal strain with apical sparing, grade 3 diastolic disfunction, biatrial enlargement, mild mitral regurgitation, right ventricle free wall thickening (8 mm), mild reduced TAPSE, and mild pericardial effusion. Cardiac magnetic resonance (CMR) confirmed ventricular wall thickening with evidence at T1 mapping of interstitial infiltration more evident in the septum and inferior-lateral wall with apical savings (Figure 2). A total body 99mTc-HDP scintigraphy showed cardiac uptake with intensity similar to bone signal (Perugini Score 2) suggesting ATTR cardiac amyloidosis (Figure 3). Both kappa and lambda concentrations were normal. The genetic testing did not reveled mutations in the TTR gene. We concluded for a diagnosis of ATTR wild-type CA. The Holter ECG monitoring registered numerous ventricular ectopic beats and an episode of non-sustained ventricular tachycardia. A coronary angiography ruled out coronary artery disease. In consideration of the clinical-instrumental picture an ICD was implanted. Conclusions In conclusion, CA is still underdiagnosed. VA and SCD may further complicate the prognosis. Early diagnosis and adequate stratification of the arrhythmic risk are essential to ensure patients the best therapeutic strategies.
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Irabor, Biobelemoye, Jacqueline M. McMillan, and Nowell M. Fine. "Assessment and Management of Older Patients With Transthyretin Amyloidosis Cardiomyopathy: Geriatric Cardiology, Frailty Assessment and Beyond." Frontiers in Cardiovascular Medicine 9 (May 17, 2022). http://dx.doi.org/10.3389/fcvm.2022.863179.
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Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is commonly diagnosed in older adults, in particular the wild-type (ATTRwt), which is regarded as an age-related disease. With an aging population and improved diagnostic techniques, the prevalence and incidence of ATTR-CM will continue to increase. With increased availability of mortality reducing ATTR-CM therapies, patients are living longer. The predominant clinical manifestation of ATTR-CM is heart failure, while other cardiovascular manifestations include arrhythmia and aortic stenosis. Given their older age at diagnosis, patients often present with multiple age-related comorbidities, some of which can be exacerbated by ATTR, including neurologic, musculoskeletal, and gastrointestinal problems. Considerations related to older patient care, such as frailty, cognitive decline, polypharmacy, falls/mobility, functional capacity, caregiver support, living environment, quality of life and establishing goals of care are particularly important for many patients with ATTR-CM. Furthermore, the high cost ATTR treatments has increased interest in establishing improved predictors of response to therapy, with assessment of frailty emerging as a potentially important determinant. Multidisciplinary care inclusive of collaboration with geriatric and elder care medicine specialists, and others such as neurology, orthopedic surgery, electrophysiology and transcatheter aortic valve replacement clinics, is now an important component of ATTR-CM management. This review will examine current aspects of the management of older ATTR-CM patients, including shared care with multiple medical specialists, the emerging importance of frailty assessment and other considerations for using ATTR therapies.
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Lamb, Nick. "Why the Diagnosis of ATTR-Cardiomyopathy May Be a Challenge for Physicians." EMJ Cardiology, October 17, 2019, 66–71. http://dx.doi.org/10.33590/emjcardiol/10310030.
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Cardiac amyloidosis is a rare but life-threatening group of disorders caused by the extracellular deposition of misfolded amyloid fibrils in cardiac tissue. Amyloid accumulation leads to cardiomyocyte toxicity, extracellular volume expansion, and ventricular pseudohypertrophy. Two types of amyloid protein are thought to be responsible for most disorders: immunoglobulin light chain, which causes light chain amyloidosis (AL); and transthyretin (TTR), which causes transthyretin amyloidosis (ATTR), of which there are two types: hereditary (hATTR) or wild-type (ATTRwt). Despite increasing clinical recognition of the disease, cardiac amyloidosis remains underdiagnosed. This article explores the epidemiology of AL and ATTR and the noninvasive techniques that help to improve diagnosis of the disorder. Cardiac amyloidosis is associated with mixed phenotype symptoms of polyneuropathy and cardiomyopathy which can lead to multiple misdiagnoses. As a result, patients can wait between 2 and 4 years for a correct diagnosis. Early diagnosis may be aided by recognising red flag symptom clusters. These include family history; neuropathy and sensory involvement; bilateral carpal tunnel syndrome; early autonomic dysfunction and gastrointestinal complaints; heart failure (HF) with preserved ejection fraction (HFpEF; without hypertension); cardiac hypertrophy, arrhythmias, ventricular blocks, right-sided or biventricular HF, or cardiomyopathy; renal abnormalities; and vitreous opacities. Noninvasive imaging techniques have increasingly been used as an alternative to biopsy to diagnose cardiac amyloidosis with the hope of allowing physicians to provide targeted therapy for these patients. Techniques include speckle tracking echocardiography, cardiac MRI, and nuclear scintigraphy, together with biomarkers such as N-terminal pro-brain natriuretic peptide and hepatocyte growth factor (HGF). It is hoped that greater understanding of patients with ATTR may lead to increased awareness of the disorder and improve patient outcomes.
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Rahman, Tanvir, Reihaneh C. Moghadam, Vikram V. Agarwal, and Craig K. Reiss. "Cardiac amyloidosis: diagnostic challenges and recent advancement in the treatment of transthyretin amyloidosis (ATTR)." Oxford Medical Case Reports 2021, no. 8 (August 1, 2021). http://dx.doi.org/10.1093/omcr/omab059.
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ABSTRACT ATTR-CA is an under-reported cause of congestive heart failure (CHF) and cardiac arrhythmias. Heightened clinical suspicion along with a multimodal investigative approach is often required in diagnosing this potentially fatal condition. Tafamidis and inotersen have shown promising results in terms of progression-free survival by ameliorating CHF symptoms and peripheral neuropathies in clinical trials. In this case series of five patients, we present three wild-type cardiac amyloidosis (ATTRwt-CA), one familial cardiac amyloidosis (ATTRm-CA) and one primary cardiac (AL-CA). The diagnostic modality was different for each patient. ATTRwt-CA, ATTRm-CA and AL-CA patients received tafamidis, inotersen and chemotherapy with bone marrow stem-cell transplantation, respectively.
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Guerra, F., F. Coretti, L. Torselletti, F. Coraducci, S. Belleggia, R. Manfredi, M. Silenzi, et al. "Prognostic role of low QRS voltages in patients with cardiac amyloidosis." European Heart Journal 43, Supplement_2 (October 1, 2022). http://dx.doi.org/10.1093/eurheartj/ehac544.1781.
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Abstract Introduction Cardiac amyloidosis (CA) is an underdiagnosed and heterogeneous cardiac disease characterized by the extracellular deposition of misfolded proteins in the cardiac tissue. Clinical manifestations are heterogeneous leading to progressive heart failure, often complicated by arrhythmias and conduction system disease. Among several sign and symptoms that are suspicious for the disease, named “red flags”, disproportionally low QRS voltages on the ECG has been described. Purpose The aim of this prospective observational study is to evaluate potential prognostic features of QRS amplitude in AL e ATTR CA patients. Methods All consecutive patients admitted to the Cardiomyopathy Clinic of our institution have been enrolled after receiving CA diagnosis, according to the current guidelines. We included all patients ≥18 years with a diagnosis of CA and written informed consent. A complete assessment including a standard 12-lead electrocardiogram (ECG) and echocardiogram was performed at enrollment. Low QRS voltages (LQRSV) was defined as a QRS total amplitude of ≤5 mm in every limb leads and ≤10 mm in every precordial lead. LQRSV was tested as an independent predictor of death from all causes (primary endpoint), hospitalization from cardiovascular causes, ventricular and supraventricular arrhythmias. Results Sixty patients (46 males, 77±12 years old) were enrolled, of which 18 (30%) met the criteria for LQRSV. Patients with LQRSV presented more frequently with an history of ventricular arrhythmia (27.8% vs. 6.7%, p=0.04), a lower left ventricular diastolic volume (31±7 vs. 44±18 ml/m2; p=0.04), and higher retinol-binding-protein 4 (9.3±2.2 vs 3.2±1.5 mg/dl; p=0.02). No differences were seen in the primary outcome (46% vs. 50%; p=NS; Figure 1) or in the secondary ones (cardiovascular hospitalization 25% vs. 21%; ventricular arrhythmias 12% vs 4%; supraventricular arrhythmias 29% vs 19%; all p=NS) between the two groups during a median follow up of 1.1 year. Conclusions In the present cohort of CA patients LQRSV did not emerge as independent predictor of all-cause mortality at 1 year. Although LQRSV is a recognized diagnostic “red-flag” in the work-up of CA, its role as prognostic marker remains unclear. Further studies with a longer follow-up are needed to better define the prognostic role of LQRSV among CA patients. Funding Acknowledgement Type of funding sources: None.
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Dale, Zack, Lana Rashdan, Pranav Chandrashekar, Stephen B. Heitner, Babak Nazer, and Ahmad Masri. "Abstract 16465: Mode of Death and Outcomes of Implantable Cardioverter Defibrillators in Transthyretin Cardiac Amyloidosis." Circulation 142, Suppl_3 (November 17, 2020). http://dx.doi.org/10.1161/circ.142.suppl_3.16465.
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Introduction: Patients with transthyretin cardiac amyloidosis (ATTR-CM) may have higher risk of sudden cardiac death yet the role of implantable cardioverter defibrillators (ICD) remains poorly defined. We report our experience with ICDs in a cohort with ATTR-CM. Methods: A single center observational cohort study of patients who had been seen at our Amyloidosis Center with either wild-type or hereditary ATTR-CM diagnosed between 2005-2019. ICD implant was at discretion of treating cardiologists. Device data was obtained from record review. Results: Ninety-seven patients with ATTR-CM (mean age 71.4±11 years, 89.7% male) had mean follow-up 2.2±1.9 years. Twenty-one patients (21.6%) got ICD - 19 for primary prevention and 2 for secondary prevention - with cumulative 137 interrogations over mean 2.0±2.7 years of device follow-up. In patients with primary prevention ICD, 1 patient got 2 inappropriate shocks - both for 1 episode of atrial flutter in the VT therapy zone; 1 patient got appropriate ATP on 3 occasions. In those with secondary prevention ICD, 1 patient got 15 appropriate shocks for 3 discrete episodes of VT storm; the other got ATP for 1 episode of monomorphic VT. In patients without ICD, 101 ambulatory monitors were reviewed (cumulative 12,313 hours) without sustained ventricular arrhythmia. In this group, 13/76 met at least a class IIB criterion for ICD per current guidelines. EF was higher in those without ICD versus with primary prevention ICD (45.0±13.9 vs 33.4±15.1 %, p=<0.01). Death was seen with similar frequency in those with primary prevention ICD and those without ICD (p=0.60) and mode of death did not vary between them (Table 1). Conclusions: Patients with ATTR-CM and primary prevention ICD have low rates of appropriate device therapy without differing in mode of death compared to those with no ICD. Use of ICD for secondary prevention was uncommon but all patients got appropriate device therapy. Further study on ICDs in ATTR-CM is needed to inform best care.
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Azevedo Coutinho, M. D. C., N. Cortez-Dias, G. Cantinho, S. Goncalves, N. Cunha, T. Rodrigues, L. Santos, I. Conceicao, and F. Pinto. "Diagnostic and prognostic contribution of 99mTc-DPD scintigraphy in transthyretin V30M cardiac amyloidosis." European Heart Journal 42, Supplement_1 (October 1, 2021). http://dx.doi.org/10.1093/eurheartj/ehab724.0278.
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Abstract Background Early diagnosis and prognostic stratification of hereditary transthyretin amyloidosis (ATTR) are crucial. Previous findings suggested that 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) scintigraphy presents suboptimal accuracy to detect ATTR cardiomyopathy caused by V30M mutation, particularly in patients with onset of symptoms under 50 years of age. Furthermore, its prognostic value has never been evaluated in ATTR caused by this mutation. Purpose To assess the diagnostic value of DPD scintigraphy to detect cardiomyopathy in a large cohort of patients with ATTR-V30M mutation and to explore its prognostic value regarding mortality. Methods Of the 305 ATTR-V30M mutation carriers followed prospectively and who underwent DPD scintigraphy, 288 individuals [median age 46 (39–56); 49% male] without myocardial thickening attributable to other causes were enrolled in the study. Amyloid cardiomyopathy was defined by septal thickness ≥13mm not attributable to other causes plus at least one of the following criteria: (1) late heart-to-mediastinum (H/M) 123I-metaiodobenzylguanidine (MIBG) ratio <1.60; (2) electrical heart disease (arrhythmia or cardiac conduction defect); or (3) amyloid infiltration documented in biopsy. Results Amyloid cardiomyopathy was identified in 41 (14.2%) patients and 44 (15.3%) individuals presented abnormal cardiac DPD uptake. Individuals with cardiac DPD retention had 27-fold higher likelihood of having amyloid cardiomyopathy (OR: 27.4; 95% CI 11.6–65.0; P<0.001). However, DPD scintigraphy presented suboptimal accuracy to detect ATTR cardiomyopathy (89.9%) with limitations in sensitivity (56.1%), specificity (95.6%), positive predictive value (67.7%) and negative predictive value (92.9%). During a mean follow-up 33.6±1.2 months, 16 patients died (5.6%). Mortality was 14 times higher in patients with amyloid cardiomyopathy (HR: 14.1; 95% CI 4.9–40.7; P<0.001), 13 times higher in those with abnormal cardiac DPD uptake (HR: 12.59; 95% CI % 4.56–34.72; P<0.001) and 10 times higher in those with H/M MIBG ratio <1.60 (HR: 10.40; 95% CI 2.95–36.69; P<0.001). The prognostic value of ventricular thickness and cardiac DPD uptake was additive: patients without septal thickening and no cardiac DPD retention had excellent prognosis (5-year mortality of 0.75%), while those with septal thickening and/or abnormal DPD retention presented 5-year mortality rates ranging from 39.9 to 53.3%. Conclusions DPD scintigraphy is valuable in the evaluation of ATTR-V30M mutation carriers, particularly for prognostic stratification purposes, identifying patients at higher risk of death. Funding Acknowledgement Type of funding sources: None.
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Damy, T., G. Bourel, M. Slama, V. Algalarrondo, O. Lairez, F. Pelcot, I. Durand-Zaleski, et al. "Epidemiology of transthyretin amyloid cardiomyopathy (ATTR-CM) in France, a study based on the systeme national des donnees de sante (SNDS) the French nationwide claims database." European Heart Journal 42, Supplement_1 (October 1, 2021). http://dx.doi.org/10.1093/eurheartj/ehab724.3131.
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Abstract Transthyretin (TTR) Amyloid Cardiomyopathy (ATTR-CM) is a rare, progressively debilitating, fatal disease with poor prognosis caused by amyloid deposition of fibrils derived from the serum protein TTR in the extracellular matrix of the heart. As amyloid infiltration in the heart progresses, atrial and ventricular walls thicken and become restrictive, resulting in diastolic dysfunction and further progression leads to heart failure, usually with preserved ejection fraction. Systolic dysfunction occurs in the late stages of the disease. Cardiac symptoms and conduction abnormalities also become increasingly clinically apparent (e.g. fatigue, shortness of breath, syncope or arrhythmias) with progression over time. Epidemiology of ATTR-CM is poorly understood as there are few existing studies that estimate its frequency in the general population. We aimed to estimate the prevalence and the incidence of ATTR-CM in France between 2011 and 2017, to describe demographic characteristics of incident cases and to assess patient survival. We used data from the SNDS database, which collects all national health insurance and hospital discharge data. As there is no specific ICD-10 marker code for ATTR -CM used in the SNDS, ATTR-CM diagnosis required both an amyloidosis and a cardiovascular condition, not necessarily reported at the same visit. Diagnostic date was defined when the features from amyloidosis and cardiovascular conditions were selected as events. Patients with a probable AL form of the disease were excluded. To remain conservative, patients younger than 50 yo were also excluded. Between 2011 and 2017, 4,815 patients with incident ATTR-CM were identified. Incidence rate was multiplied by more than 3 times, from 0.5 / 100,000 person-year in 2011 to 1.8 / 100,000 person-year in 2017, reaching 1,225 new cases in 2017. Sex-ratio remained stable (2:1). Most of the 4,815 identified patients were older than 70 yo. In the group ATTR CM >70 y.o, there were 3 times more men than women. Median age at diagnosis was 84.0 for women and 82.0 for men. The range of 80–89 yo represents about 50% of diagnose rate in overall population. Median survival was 33.7 months overall with minor differences between gender. Survival probability was 0.69 a year after diagnosis, 0.58 two years after, 0.48 three years after, and 0.41 four years after. Using exponential modeling the life expectancy of this population would be about 4 years for the 20 next years. This study is, to our knowledge, the first estimate of ATTR-CM incidence rates based upon an analysis of a national claims database. Our findings are consistent with existing data concerning the frequency of amyloidosis, even though were only identified the ICD-10 coded diagnosed cases, leading to possible underestimation of the full prevalence of the disease in France. SNDS demonstrated to be a scientifically valid data base to follow-up the standard of care of ATTR-CM. Funding Acknowledgement Type of funding sources: Private company. Main funding source(s): Pfizer France - SAS
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Dale, Z., P. Chandrashekar, L. Al-Rashdan, M. Kim, A. Masri, and B. Nazer. "Management strategies for atrial fibrillation and flutter in patients with transthyretin cardiac amyloidosis." European Heart Journal 42, Supplement_1 (October 1, 2021). http://dx.doi.org/10.1093/eurheartj/ehab724.1810.
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Abstract Background Atrial fibrillation (AF) and flutter (AFL) are common in transthyretin cardiac amyloidosis (ATTR-CM). Ventricular rate control drugs in ATTR-CM are poorly tolerated but data addressing tolerability and efficacy of rhythm control strategies is limited. Purpose We report characteristics and outcomes of AF/AFL in a cohort with ATTR-CM. Methods A single center observational study of patients seen at our Amyloidosis Center with wild-type or hereditary ATTR-CM diagnosed between 2005–2019. Treatment was prescribed as per treating cardiologists. Results Eighty-four patients with ATTR-CM (average age 74±10 years, 94% male) had mean follow-up of 27.6±22.8 months. AF/AFL occurred in 61 patients (73%). Clinically significant rapid ventricular response (RVR) was common as well attempted rate control with AV node blockers (Table 1). However, discontinuation was frequent (80%), often for adverse effects of hypotension (33%), bradycardia (15%), or presyncope/syncope (10%). Rhythm control was initiated in 64%, most often with cardioversion (DCCV) or ablation (Table 2). Post-DCCV recurrence was common (91%) and time to recurrence did not differ with use of anti-arrhythmic drugs (AAD; 5.8 months (IQR 1.9–12.5) vs without AAD 6.2 months (IQR 1.9–12.5) p=0.83). TEE was performed for 33% of DCCV with thrombus seen in 11% of cases – all patients who were not anticoagulated at the time. TEE was otherwise deferred due to known AF/AFL duration <48 hours (13%) or adequate anticoagulation (54%). Ablation was performed in 23% of patients with AFL (all for typical AFL) with 2 patients (14%) having recurrence after mean of 60.9 months. Pulmonary vein isolation for AF was performed in 12% (86% for persistent AF) with 86% recurrence after median of 6.2 months (IQR 5.6–12.3). Most patients (62%) with rhythm control had subjective improvement (≥1 NYHA class or resolved palpitations). Among AAD, amiodarone was most well tolerated with only 8% of patient discontinuing due to side effects. DCCV and ablation resulted in no direct complications although one patient had a perforation of a previously unknown Zenker diverticulum during TEE pre-DCCV. Conclusions In our ATTR-CM cohort, AF/AFL was common. Rate control was poorly tolerated and often abandoned. While rhythm control of AF/AFL had a favorable safety profile and successful conversion to sinus rhythm led to symptomatic improvement in a majority of cases, durable success with rhythm control was limited, often requiring multiple therapies. DCCV is only modestly successful and not significant improved with AAD. Ablation was successful in cases of cavo-tricuspid isthmus dependent AFL but had limited success in AF. Funding Acknowledgement Type of funding sources: None.
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Quarta, C. Cristina, Marianna Fontana, Thibaud Damy, Julia Catini, Damien Simoneau, Michele Mercuri, Pablo Garcia-Pavia, Mathew S. Maurer, and Giovanni Palladini. "Changing paradigm in the treatment of amyloidosis: From disease-modifying drugs to anti-fibril therapy." Frontiers in Cardiovascular Medicine 9 (December 20, 2022). http://dx.doi.org/10.3389/fcvm.2022.1073503.
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Cardiac amyloidosis is a rare, debilitating, and usually fatal disease increasingly recognized in clinical practice despite patients presenting with non-specific symptoms of cardiomyopathy. The current standard of care (SoC) focuses on preventing further amyloid formation and deposition, either with anti-plasma cell dyscrasia (anti-PCD) therapies in light-chain (AL) amyloidosis or stabilizers of transthyretin (TTR) in transthyretin amyloidosis (ATTR). The SoC is supplemented by therapies to treat the complications arising from organ dysfunction; for example, heart failure, arrhythmia, and proteinuria. Advancements in treatments have improved patient survival, especially for those whose disease is detected and for whom treatment is initiated at an early stage. However, there still are many unmet medical needs, particularly for patients with severe disease for whom morbidity and mortality remain high. There currently are no approved treatments to reverse amyloid infiltration and deplete the amyloid fibrils already deposited in organs, which can continue to cause progressive dysfunction. Anti-fibril therapies aimed at removing the deposited fibrils are being investigated for safety and efficacy in improving outcomes for patients with severe disease. However, there is no clinical evidence yet that removing deposited amyloid fibrils will improve organ function, thereby improving quality of life or extending life. Nevertheless, anti-fibril therapies are actively being investigated in clinical trials to evaluate their ability to complement and synergize with current SoC.
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De Michieli, Laura, Giulio Sinigiani, Petra Deola, Chiara Mainardi, Martina Perazzolo Marra, Cristina Basso, Sabino Iliceto, and Alberto Cipriani. "169 CARDIAC AMYLOIDOSIS AND CORONARY ARTERY DISEASE: INSIGHTS FROM A RETROSPECTIVE OBSERVATIONAL STUDY." European Heart Journal Supplements 24, Supplement_K (December 14, 2022). http://dx.doi.org/10.1093/eurheartjsupp/suac121.606.
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Abstract Introduction Patients with cardiac amyloidosis (CA) frequently present with heart failure with various extra-cardiac red flags. However, some patients can present with typical or atypical chest pain or even mimicking an acute coronary syndrome. Significant epicardial coronary artery disease (CAD) and CA can coexist, but microvascular dysfunction might also play a role. The aims of this study were to systematically investigate clinical indications for invasive coronary angiography (ICA) in patients with CA, to verify the prevalence of significant epicardial CAD and its relation to the symptoms at presentation, and to start investigating the role cardiac troponin (cTn) in this setting of patients with multiple possible causes of myocardial injury. Methods Retrospective observational study of patients with CA, both transthyretin (ATTR-CA) and light chain (AL-CA) evaluated in our Cardiac Amyloidosis Outpatient Clinic and undergoing ICA for clinical indication. Significant epicardial CAD was defined as stenosis ≥ 50% of the left main coronary artery and/or ≥ 70% of the other epicardial vessels. Results Of the 161 patients initially evaluated, 67 (42%) underwent ICA; after exclusion of those with ICA older than 5 years than CA diagnosis, the final cohort was of 61 patients, 12 (20%) AL-CA and 49 (80%) ATTR-CA. Patients with significant epicardial CAD at the index ICA had ATTR-CA and were more frequently affected by systemic hypertension and dyslipidemia. In 42 (69%) patients ICA was performed during an urgent hospitalization and in 38 (62%) patients CA diagnosis was already established/suspected at the time of the index ICA. The most common indication for ICA was chest pain (n=24, 39%), followed by dyspnea (n=21, 34%); electrocardiographic alterations/arrhythmias were the main indication for 4 patients (6.6%) while imaging for 6 (9.8%). In the remaining 6 patients (9.8%), there were other indications. Prevalence of significant epicardial CAD at the index ICA was 28% (n=17), up to 36% (n=22) if patients with previous coronary revascularization but without actual critical stenoses at index ICA were included. Among those presenting with chest pain (n=24), 9 (38%) had significant epicardial CAD at the index ICA whereas 15 (62%) did not. Considering those presenting with an acute event, the most frequent indication for ICA was chest pain (n=20, 48%); among these patients with chest pain, 55% did not have evidence of significant epicardial CAD whilst 45% did. Regarding cardiac troponin (cTn) values, concentrations at all timepoints were similar between patients with and without significant epicardial CAD at index ICA, also considering those presenting acutely and for chest pain. Conclusion In our cohort of CA patients undergoing ICA, the main clinical indication was chest pain; however, the majority of those presenting with chest pain did not have evidence of significant epicardial CAD. Significant epicardial CAD was present in around one third of patient, which is slightly less than what reported in non-amyloidotic HFpEF patients. The role of cTn in this setting is controversial, due to the possible concomitant causes of chronic and acute myocardial injury in these patients.
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Funabashi, N., and Y. Kobayashi. "Prediction of sites of wall thickening and abnormal late enhancement on cardiac CT and magnetic resonance imaging using electrocardiography findings in patients with confirmed cardiac amyloidosis." European Heart Journal 42, Supplement_1 (October 1, 2021). http://dx.doi.org/10.1093/eurheartj/ehab724.0309.
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Abstract Background Left ventricular (LV) wall thickening and diastolic dysfunction on a transthoracic echocardiogram (TTE) without high voltage R wave in V5 leads on ECG leads to a diagnosis of cardiac amyloidosis. A final diagnosis is made by endomyocardial biopsy. However, amyloid sometimes invade the right ventricle (RV), and left (LA) and right (RA) atria to cause ECG changes such as sick sinus syndrome (SSS), arrhythmia, and QRS wave axis deviation. Purpose To predict sites of wall thickening and abnormal late enhancement (LE) on cardiac computed tomography (CT) and magnetic resonance imaging (MRI), suggesting amyloid invasion, using cardiac rhythm and other ECG findings in patients with cardiac amyloidosis confirmed by biopsy. Methods A total of 26 patients (11 females) with suspected cardiac amyloidosis, showing LV wall thickening by TTE without a high voltage R wave in V5 leads on ECG, underwent cardiac enhanced CT. LV wall thickening on CT in the early phase led to late phase acquisition to detect LE. Five patients (3 females, mean age 73 years) were diagnosed with cardiac amyloidosis: complicated multiple myeloma, 2; senile ATTR (transthyretin) amyloidosis, 1; immunoglobulin light chain (AL) amyloidosis 1; and transthyretin mutation, 1. Four patients underwent cardiac MRI. Results Two patients (cases 1 and 2) had SSS (junctional rhythm), one had atrial tachycardia, and the remaining two (cases 4 and 5) had a normal sinus rhythm. In case 1, ECG showed a left axis QRS wave deviation, no low voltage R wave in limb leads and a mild LA load. Wall thickening in the basal interventricular septum (IVS), LV inferior-posterior wall, LA on CT, LE in the endocardium in whole LV, RV, and RA on CT, and LE in the endocardium in whole LV, RV, LA, and IVS on MRI were observed. In case 2, ECG showed a normal QRS wave axis, no low voltage R wave in limb leads, no LA load, wall thickening in whole LV, RV, LA, and IVS on CT, and unclear (CT) or no (MRI) LE. In case 3, ECG showed a normal QRS wave axis, with low voltage R wave in limb leads, no LA load, wall thickening in LA and basal IVS on CT, LE in LA and basal IVS on CT, and LE in LA only on MRI. In case 4, ECG showed left axis QRS wave deviation, a low voltage R wave in limb leads, and no LA load, wall thickening in the LA and RV moderator band on CT, unclear LE on CT, and LE in whole LV, endocardium in the RV, and whole IVS on MRI. In case 5, ECG showed a right axis QRS wave deviation, low voltage R wave in limb leads, and a mild LA load, wall thickening in the IVS, LV lateral wall, LV anterior wall, RA, RV outflow tract, and RA appendage, and no LE on CT (MRI not performed). Conclusions This pilot study of a few patients with cardiac amyloidosis revealed few links between cardiac rhythm and other ECG findings with sites of wall thickening and abnormal LE. However, a longer-term study of more patients may lead to detecting an association between these variables with this methodology. Funding Acknowledgement Type of funding sources: None.
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Funabashi, N., and Y. Kobayashi. "Comparison of sites of wall thickening and abnormal late enhancement on cardiac CT and magnetic resonance imaging with electrocardiography findings in patients with confirmed cardiac amyloidosis." European Heart Journal 43, Supplement_1 (February 1, 2022). http://dx.doi.org/10.1093/eurheartj/ehab849.010.
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Abstract Funding Acknowledgements Type of funding sources: None. Background Left ventricular (LV) wall thickening and diastolic dysfunction on a transthoracic echocardiogram (TTE) without a high voltage R wave on V5 leads on an ECG leads to a diagnosis of cardiac amyloidosis. A final diagnosis is made by endomyocardial biopsy. However, amyloid sometimes invades the right ventricle (RV), and left (LA) and right (RA) atria, causing ECG changes such as sick sinus syndrome (SSS), arrhythmia, and QRS wave axis deviation. Purpose To elucidate the relationship between sites of wall thickening and abnormal late enhancement (LE) on cardiac computed tomography (CT) and magnetic resonance imaging (MRI), suggesting amyloid invasion, with ECG findings in patients with cardiac amyloidosis confirmed by biopsy. Methods A total of 26 patients (11 females) with suspected cardiac amyloidosis, who had LV wall thickening by TTE without a high voltage R wave in V5 leads on ECG, underwent cardiac CT. LV wall thickening observed on CT in the early phase led to a late phase acquisition. Five patients (3 females, mean age 73 years) were diagnosed with cardiac amyloidosis: complicated multiple myeloma, 2; senile ATTR (transthyretin) amyloidosis, 1; immunoglobulin light chain (AL) amyloidosis, 1; and transthyretin mutation, 1. Four patients underwent cardiac MRI. Results Case 1 had wall thickening in the basal interventricular septum (IVS), LV inferior-posterior wall, LA on CT, abnormal LE in the endocardium in whole LV, RV, and RA on CT, and LE in the endocardium in whole LV, RV, LA, and IVS on MRI. ECG showed SSS (junctional rhythm), left axis QRS wave deviation, no low voltage R wave in limb leads, and a mild LA load. Case 2 had wall thickening in whole LV, RV, LA, and IVS on CT, and unclear (CT) or no (MRI) abnormal LE. ECG revealed SSS (junctional rhythm), a normal QRS axis, no low voltage R wave in limb leads, and no LA load. Case 3 had wall thickening in the LA and basal IVS on CT, abnormal LE in the LA and basal IVS on CT, and LE in the LA only on MRI. ECG revealed atrial tachycardia, a normal QRS axis with low voltage R wave in limb leads, and no LA load. Case 4 had wall thickening in the LA, an RV moderator band on CT, an unclear LE on CT, and LE in whole LV, endocardium in the RV, and whole IVS on MRI. ECG showed a normal sinus rhythm, left axis QRS wave deviation, with low voltage R wave in limb leads, and no LA load. Case 5 had wall thickening in the IVS, LV lateral wall, LV anterior wall, RA, RV outflow tract, and RA appendage, and no abnormal LE on CT (MRI not performed). ECG revealed a normal sinus rhythm, right axis QRS wave deviation, with low voltage R wave in limb leads, and a mild LA load. Conclusions In this pilot study of a small number of patients with cardiac amyloidosis, few relationships between sites of wall thickening and abnormal LE on ECG were found. However, a long-term follow-up study with more patients may reveal relationships between such parameters using this methodology. Abstract Figure. Classification by wall thickening on CT