P009. Cardiovascular Disease Burden Before Hereditary Transthyretin Amyloidosis Diagnosis

Background Hereditary transthyretin amyloidosis (hATTR or ATTRv [variant]) is a progressive and fatal disease often associated with infiltrative cardiac involvement, with cardiac symptoms sometimes occurring years before diagnosis. Because there are available treatments for hATTR, prompt recognition and early diagnosis is imperative. Nurses who work closely with patients with heart failure are positioned to recognize the symptoms and early signs that should raise clinical suspicion and uncover an underlying diagnosis of hATTR. Objective To determine whether patients with hATTR demonstrate significant cardiovascular symptom manifestations and health care utilization before diagnosis. Methods Patients with newly diagnosed hATTR were identified in IBM® MarketScan® Commercial and Medicare Supplemental data (United States) using a claims-based algorithm. Diagnosis required ≥1 claim with relevant amyloidosis diagnosis code (ICD-10-CM: E85.0-.4, E85.89, E85.9) during the identification (ID) period (January 2016-December 2017), and the occurrence of ≥1 qualifying criterion during 2011-2017: ≥15 days diflunisal use, liver transplant, or claim with code E85.1 (neuropathic heredofamilial amyloidosis) or E85.2 (heredofamilial amyloidosis unspecified). Index date was defined as date of first claim with amyloidosis code in the ID period, and preindex period was defined as the 5 years before index date. Patients with a preexisting ICD-9/10 amyloidosis code during this period were excluded to focus on patients who were newly diagnosed. Control subjects without hATTR were identified and matched (age, sex, region) to patients with hATTR (ratio 3:1); these subjects were assigned same index and met same continuous enrollment requirement during preindex period. Frequency of selected cardiovascular conditions and health care use were measured in the preindex period; demographics and Charlson comorbidity index (CCI) were measured 1 year preindex. Results Among 141 patients with hATTR and 423 matched controls, mean (SD) age was 62.5 (14.2) years and 53.9% were female. Mean CCI for patients with hATTR was 2.7 (3.0) versus 1.1 (1.9) for controls. In the preindex period, cardiovascular conditions/symptoms were more common among patients with hATTR relative to controls: chest pain (51.8 vs 28.1%; P < 0.001), dyspnea (49.6 vs 25.8%; P < 0.001), edema (27.0 vs 12.8%; P < 0.001), heart failure (23.4 vs 5.9%; P < 0.001), ventricular hypertrophy (19.9 vs 5.7%; P < 0.001), hypotension (18.4 vs 6.1%; P < 0.001), atrial fibrillation/flutter (18.0 vs 8.9%; P < 0.001), stroke (12.8 vs 6.1%; P = 0.011), and aortic stenosis (10.6 vs 4.5%; P = 0.008); these conditions were more prevalent for patients with hATTR compared with controls for each preindex year. Hospitalizations (47.5 vs 24.3%; P < 0.001), emergency department visits (60.3 vs 47.0%; P = 0.006) and cardiac imaging (56.7 vs 27.0%; P < 0.001) were also more frequent among patients with hATTR. Median time (months) from initial symptom manifestation to hATTR diagnosis was longest for chest pain (43.0), dyspnea (41.1), aortic stenosis (39.2), and atrial fibrillation/flutter (34.3). Conclusions Patients with hATTR have considerable cardiovascular disease burden in the 5 years preceding diagnosis. Increased awareness of characteristic cardiovascular manifestations among nurses may increase clinical suspicion, leading to early diagnosis and prompt intervention. Hereditary transthyretin amyloidosis (hATTR or ATTRv [variant]) is a progressive and fatal disease often associated with infiltrative cardiac involvement, with cardiac symptoms sometimes occurring years before diagnosis. Because there are available treatments for hATTR, prompt recognition and early diagnosis is imperative. Nurses who work closely with patients with heart failure are positioned to recognize the symptoms and early signs that should raise clinical suspicion and uncover an underlying diagnosis of hATTR. To determine whether patients with hATTR demonstrate significant cardiovascular symptom manifestations and health care utilization before diagnosis. Patients with newly diagnosed hATTR were identified in IBM® MarketScan® Commercial and Medicare Supplemental data (United States) using a claims-based algorithm. Diagnosis required ≥1 claim with relevant amyloidosis diagnosis code (ICD-10-CM: E85.0-.4, E85.89, E85.9) during the identification (ID) period (January 2016-December 2017), and the occurrence of ≥1 qualifying criterion during 2011-2017: ≥15 days diflunisal use, liver transplant, or claim with code E85.1 (neuropathic heredofamilial amyloidosis) or E85.2 (heredofamilial amyloidosis unspecified). Index date was defined as date of first claim with amyloidosis code in the ID period, and preindex period was defined as the 5 years before index date. Patients with a preexisting ICD-9/10 amyloidosis code during this period were excluded to focus on patients who were newly diagnosed. Control subjects without hATTR were identified and matched (age, sex, region) to patients with hATTR (ratio 3:1); these subjects were assigned same index and met same continuous enrollment requirement during preindex period. Frequency of selected cardiovascular conditions and health care use were measured in the preindex period; demographics and Charlson comorbidity index (CCI) were measured 1 year preindex. Among 141 patients with hATTR and 423 matched controls, mean (SD) age was 62.5 (14.2) years and 53.9% were female. Mean CCI for patients with hATTR was 2.7 (3.0) versus 1.1 (1.9) for controls. In the preindex period, cardiovascular conditions/symptoms were more common among patients with hATTR relative to controls: chest pain (51.8 vs 28.1%; P < 0.001), dyspnea (49.6 vs 25.8%; P < 0.001), edema (27.0 vs 12.8%; P < 0.001), heart failure (23.4 vs 5.9%; P < 0.001), ventricular hypertrophy (19.9 vs 5.7%; P < 0.001), hypotension (18.4 vs 6.1%; P < 0.001), atrial fibrillation/flutter (18.0 vs 8.9%; P < 0.001), stroke (12.8 vs 6.1%; P = 0.011), and aortic stenosis (10.6 vs 4.5%; P = 0.008); these conditions were more prevalent for patients with hATTR compared with controls for each preindex year. Hospitalizations (47.5 vs 24.3%; P < 0.001), emergency department visits (60.3 vs 47.0%; P = 0.006) and cardiac imaging (56.7 vs 27.0%; P < 0.001) were also more frequent among patients with hATTR. Median time (months) from initial symptom manifestation to hATTR diagnosis was longest for chest pain (43.0), dyspnea (41.1), aortic stenosis (39.2), and atrial fibrillation/flutter (34.3). Patients with hATTR have considerable cardiovascular disease burden in the 5 years preceding diagnosis. Increased awareness of characteristic cardiovascular manifestations among nurses may increase clinical suspicion, leading to early diagnosis and prompt intervention.