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As targeted treatments for amyloid transthyretin cardiomyopathy (ATTR-CM) are becoming available, we aim to characterize the rates of ventricular arrhythmias (VAs), implantable cardioverter-defibrillator (ICD) utilization, and their impact on survival.This is a retrospective cohort study of 130 patients with ATTR-CM diagnosed at Emory University's Cardiac Amyloidosis Center between April 2012 and September 2020. VAs were defined as nonsustained or sustained ventricular tachycardia and ventricular fibrillation.Of 130 patients, 42 had wild-type disease (wtATTR) and 88 had hereditary variants (hATTR), most commonly Val122Ile (89%). At ATTR-CM diagnosis, 80 (62%) patients had EF ≤ 40% consistent with systolic heart failure. Of the 69 (53%) patients with documented VAs significantly higher rates occurred among those with EF ≤ 40% compared with EF 40% (67% vs. 28%, p = .001). Thirty-two patients (25 hATTR, 7 wtATTR) had primary prevention ICDs implanted. Eight (25%) of these patients received appropriate ICD therapy while two (6%) experienced inappropriate therapy. Comparing patients with EF ≤ 35% with and without ICDs did not reveal any survival difference (3.3 ± 0.5 vs. 2.8 ± 0.4 years, p = .699).High rates of VAs and appropriate ICD therapy were found among a unique cohort of largely hereditary ATTR-CM patients with a high rate of systolic heart failure.

Introduction: Hereditary Transthyretin Cardiac Amyloidosis (hATTR-CA) of the Valine 122 Isoleucine (Val122Ile) variant is prevalent among 3.4% African Americans. More commonly diagnosed in men, there is limited data on sex differences in clinical characteristics and outcomes in patients with Val122Ile hATTR-CA. Methods: Retrospective chart review identified a total of 90 patients with a genetic confirmation of Val122Ile hATTR-CA and either positive technetium pyrophosphate (PYP) scanning or endomyocardial biopsy. Cohorts were divided based on sex and clinical and demographic data was collected from time of diagnosis. Results: Among our cohort, 64 (71%) were males and 26 (28%) were females. All were African Americans. Women were significantly older at the time of diagnosis compared to men, 76 ± 6 vs 71 ± 7, p=0.001. New York Heart Association (NYHA) Functional Classification scores were similar between sexes with the majority presenting with NYHA class II and III symptoms. Men had significantly higher rates of coronary artery disease compared to women, 17 (26.6%) vs 2 (7.7%), p=0.047, but all other comorbidities and extra-cardiac manifestations were similar. Troponin I, BNP, eGFR, and prealbumin did not differ significantly between sexes. Electrocardiographic variables, arrhythmia rates, and cardiac implantable electronic distribution were also similar between gender. However, several echocardiographic variables at diagnosis significantly differed: left ventricular ejection fraction (LVEF, %) was lower in men compared to women (41 ±17 vs 33 ± 15, p=0.024) whereas left ventricular end-diastolic diameter (LVeDd, mm) and mass index (LVMI, g/m2) were both higher in men (44.3 ± 6.7 vs 38.4 7.6, p = 0.001 and 168 ± 63 vs 131 ± 38, p = 0.009 respectively). There was no difference in time to diagnosis or survival between men and women in our cohort. Conclusions: Consistent with prior data, women with Va1122Ile hATTR-CA were significantly older with higher LVEF at diagnosis when compared to men. Despite this, women were found to have similar NYHA classification, extra-cardiac manifestation rates, biomarkers, and survival. These findings support a delayed, but equally infiltrative phenotype that should be considered in women with preserved heart failure.

Introduction: The third generation transcatheter heart valve system aimed to improve the safety of transcatheter aortic valve replacement (TAVR). We performed systematic review and meta-analysis to compare clinical outcomes after TAVR with Edwards Sapien 3 (ES3) and Evolut series (MER). Methods: A systematic search was conducted by 2 independent researchers in MEDLINE and EMBASE for studies comparing clinical outcomes after TAVR with ES3 and MER. 1 randomized controlled trial and 11 observational studies met the inclusion criteria. Mantel-Haenszel odds ratios were calculated, and for heterogeneity I 2 statistics were reported. Results: 12 studies with total 7,749 patients were included for final analysis. ES3 and MER were implanted in 4,333 and 3,416 patients respectively. ES3 showed statistically significant decrease in permanent pacemaker implantation at 30-days (OR: 0.60; 95% CI: 0.52-0.70; p< 0.05). Furthermore, TAVR with ES3 showed non-statistically significant trend towards decreased all-cause mortality at 30 days (OR: 0.73; 95% CI: 0.51-1.02; p=0.07), and stroke at 30 days (OR: 0.55; 95% CI: 0.27-1.10; p=0.09). Cardiovascular mortality (OR: 1.03; 95% CI: 0.50-2.15; p=0.93), and acute kidney injury (OR: 1.08; 95% CI: 0.83-1.41; p=0.56) did not show statistically significant difference. Conclusions: While ES3 and MER had similar clinical outcomes, patients undergoing TAVR with ES3 had statistically significant decrease in permanent pacemaker implantation and hence may be favored in patients with pre-existing conduction system disease.

Introduction: Implantable cardioverter defibrillator (ICD) implantation in patients with cardiac amyloidosis is controversial, with no clear guidelines for clinical decision-making.. The role of ICD implantation in hereditary Transthyretin Amyloid Cardiomyopathy (hATTR-CM) is unclear. We sought to establish the burden of ventricular arrhythmias and the outcomes of ICD implantation in a single center cohort of hATTR-CM patients. Methods: A total of 69 patients with a confirmed diagnosis of hATTR-CM from genetic testing, and technetium pyrophosphate (PYP) scanning, or endomyocardial biopsy underwent retrospective chart review for demographic, clinical, and arrhythmia data. Results: Seventy-four percent of the cohort was male, with a mean age at diagnosis of 68 (SD=18 years). Sixty-five patients (94.2%) patients were African-American; all of whom carried the Valine 122 Isoleucine mutation. Most had systolic heart failure (New York Heart Association Staging II [18, 26%] and III [40, 58.8%]); 37 (54%) patients had an LVEF ≤ 35%. Thirty-six (52.2%) patients had documented episodes of non-sustained ventricular tachycardia (NSVT), three (4.3%) with ventricular tachycardia (VT), and one (1.5%) with ventricular fibrillation (VF). A total of 15 (21.7%) patients had ICDs placed for prevention of sudden cardiac death in the setting of low LVEF (EF Conclusions: In a cohort of patients with hATTR-CM, we observed a high incidence of NSVT, yet only 41% of patients with severe systolic heart failure had ICDs implanted. A high rate of successful defibrillation and no inappropriate ICD shocks were noted, suggesting that ICDs should be strongly considered, and may be underutilized, in patients with systolic heart failure and/or arrhythmias in the setting of hATTR-CM.

Non-alcoholic fatty liver disease (NAFLD) with cryptogenic liver cirrhosis is uncommonly linked to platypnea-orthodeoxia syndrome (POS). Traditionally, this syndrome has been described in correlation to intracardiac shunting like patent foramen ovale. We report a case of a 70-year-old female, with a previous history of NAFLD and heart failure presenting with acute hypoxic respiratory failure secondary to fluid overload. Further investigations revealed cryptogenic presentation of POS, which was masked by her heart failure. The patient was not able to maintain her oxygen saturation levels in an upright position, with marked improvement when lying down. Her echocardiogram was significant for positive bubble study without any intracardiac shunt, hence making NAFLD as a cause of this rare presentation of POS a more likely diagnosis.