Temporal Trends in Diagnostic Testing Patterns for Wild-Type Transthyretin Amyloid Cardiomyopathy in the Medicare Fee-for-Service Population

Introduction: Both hereditary (hATTR) and wild-type (wtATTR) transthyretin amyloidosis are under-recognized causes of cardiomyopathy (CM) and heart failure. Certain findings on Transthoracic Echocardiography (TTE) and cardiac Magnetic Resonance Imaging (cMRI) are suggestive but not diagnostic of ATTR. Although biopsy historically has been the gold standard for diagnosis, patients can be diagnosed with the highly sensitive and specific technetium-99m pyrophosphate scan (Tc-99m PYP). Genetic testing is recommended to confirm hATTR in patients diagnosed with ATTR cardiac amyloidosis. Despite growing awareness of this condition, many cases remain undiagnosed. This study evaluated if patients with TTEs concerning for infiltrative CM received appropriate diagnostic testing for ATTR-CM. Methods: Our echocardiography registry was queried from January 2011 to March 2020 for patients with our echo lab’s embedded infiltrative CM code. Data on demographics, comorbidities, TTE variables, cMRI results, PYP scans, genetic testing and biopsy results were retrieved from electronic medical records. Thorough manual chart review excluded other causes of CM. Data was expressed as mean ± SD and n (%). Results: We retrieved 510 patients (mean age 64 ± 16 years; 43% female) with TTEs suspicious for infiltrative CM revealing a mean interventricular septal diameter (IVSd) of 1.6 ± 0.3 cm. Only 67 (13%) patients underwent cMRI with 11 (16%) suggestive of cardiac amyloidosis. Of the patients with suspicious TTEs, 16 (3.1%) had PYP scans and 24 (4.7%) had tissue biopsy, with positive results in 7 (44%) and 11 (46%), respectively. Genetic testing in 31 (6%) patients revealed known hATTR mutations in 2 (6.5%) patients. Cardiac amyloidosis was diagnosed in 23 (4.5%) with 11 ATTR (2 hATTR), 5 amyloid light chain, and 7 unknown subtype. Conclusion: Despite clinical and TTE findings suspicious for ATTR-CM, many patients did not undergo appropriate confirmatory testing (see Figure 1).

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Wild-Type Transthyretin Amyloid Cardiomyopathy

Circulation ◽

10.1161/circulationaha.115.020351 ◽

2016 ◽

Vol 133 (3) ◽

pp. 245-247 ◽

Cited By ~ 17

Author(s):

Peter P. Liu ◽

David Smyth

Keyword(s):

Wild Type ◽

Amyloid Cardiomyopathy

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Abstract 60: National Temporal Patterns in Recurrent Stroke by Demographic Characteristics and Geographic Regions: 2001-2016

Stroke ◽

10.1161/str.52.suppl_1.60 ◽

2021 ◽

Vol 52 (Suppl_1) ◽

Author(s):

Judith H Lichtman ◽

Erica C Leifheit ◽

Yun Wang ◽

Larry B Goldstein

Keyword(s):

Proportional Hazards Model ◽

Recurrent Stroke ◽

Temporal Trends ◽

Healthcare Delivery ◽

Temporal Patterns ◽

Cox Proportional Hazards ◽

Cox Proportional Hazards Model ◽

Fee For Service ◽

The Us ◽

Over Time

Background: There have been important advances in secondary stroke prevention and a focus on healthcare delivery in the US over the past two decades. Yet, little is known about temporal patterns of recurrent stroke in the US. We examined temporal trends in recurrent stroke by sociodemographic characteristics and geographic areas using national Medicare data. Methods: We included fee-for-service Medicare beneficiaries aged ≥65y with a primary discharge diagnosis of ischemic stroke from 2001 to 2016. We fit a Cox proportional hazards model that censored for change in Medicare enrollment and accounted for death to evaluate the temporal trend in 1-year recurrent stroke, adjusting for demographic and clinical factors. Models were repeated for subgroups defined by age, sex, race, and state. We mapped smoothed rates of 1-year recurrent stroke by county to assess geographic variation over time. Results: There were 3,485,618 unique beneficiaries discharged with stroke during the study period. Demographic and clinical characteristics remained relatively stable over time, but the proportions discharged with home health services and inpatient rehabilitation increased. The observed 1-year recurrent stroke rate decreased from 11.2% in 2001-2004 to 9.3% in 2013-2016, with an adjusted annual reduction in recurrence from 2001-2016 of 1.49% (95% CI 1.40%-1.58%). There were significant reductions for all age, sex, and race groups (A). Geographic areas with persistently high rates were identified over time (B). In state-stratified analysis, the annual percentage reduction in recurrence ranged from -1.2% to 2.5% and was significant for all but 12 states. Conclusions: Recurrent strokes decreased over time overall and by sociodemographic subgroups; however, we identified geographic areas with persistently high recurrence rates. Such findings can target secondary prevention intervention opportunities for high-risk populations and communities.

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Temporal Trends of Wild-type Attr Amyloidosis in the Transthyretin Amyloidosis Outcomes Survey

Journal of Cardiac Failure ◽

10.1016/j.cardfail.2020.09.239 ◽

2020 ◽

Vol 26 (10) ◽

pp. S82

Author(s):

Jose Nativi-Nicolau ◽

Alfonso Siu ◽

Angela Dispenzieri ◽

Mathew S. Maurer ◽

Claudio Rapezzi ◽

...

Keyword(s):

Temporal Trends ◽

Wild Type ◽

Transthyretin Amyloidosis ◽

Attr Amyloidosis

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Over-diagnosis of Rotavirus Infection in Infants Due to Detection of Vaccine Virus

Clinical Infectious Diseases ◽

10.1093/cid/ciz1196 ◽

2019 ◽

Vol 71 (5) ◽

pp. 1324-1326 ◽

Cited By ~ 1

Author(s):

David M Whiley ◽

Suifang Ye ◽

Sarah Tozer ◽

Julia E Clark ◽

Cheryl Bletchly ◽

...

Keyword(s):

Clinical Management ◽

Diagnostic Testing ◽

Vaccine Virus ◽

Vaccine Effectiveness ◽

Wild Type ◽

Virus Shedding ◽

Rotavirus Infection ◽

Routine Infant ◽

Positive Results ◽

Active Disease

Abstract An accurate rotavirus diagnosis is important for clinical management and monitoring active disease and vaccine effectiveness. Between 2016–2018, rotavirus-positive results in our laboratory were from vaccine virus shedding in 71/152 (46.7%) infants with a request for rotavirus testing. Routine infant diagnostic testing should ideally distinguish vaccine from wild-type viruses.

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MPC-02 REVIEW OF MEDULLOBLASTOMA FOR THE ASSESSMENT OF CONSENSUS IN PATHOLOGICAL DIAGNOSIS USING JPMNG CASES

Neuro-Oncology Advances ◽

10.1093/noajnl/vdz039.099 ◽

2019 ◽

Vol 1 (Supplement_2) ◽

pp. ii22-ii22

Author(s):

Atsushi Sasaki ◽

Jyunko Hirato ◽

Takeshi Inoue ◽

Yonehiro Kanemura ◽

Yoshinori Kodama ◽

...

Keyword(s):

Diagnostic Testing ◽

Molecular Data ◽

Pathological Diagnosis ◽

Nuclear Accumulation ◽

Variable Degree ◽

Wild Type ◽

Molecular Analyses ◽

Group 4 ◽

Group 3 ◽

Subgroup Classification

Abstract Medulloblastoma (MB) is now classified by WHO 2016 classification as “genetically defined” and “histologically defined” variants. The aim of this study is to search for consensus on pathological diagnosis and assess the correlation between the central pathological diagnosis and the molecular subgrouping. We performed the pathological and molecular analyses in a total of 176 JPMNG (The Japan Pediatric Molecular Neuro-Oncology Group) cases. The diagnosis of MB was made by three expert neuropathologists (AS, JH, and TH) without knowledge of the molecular data. Subgroup affiliation was determined by expression profiling of 22 medulloblastoma subgroup-specific genes using the nanoString nCounter system. Histologically, classic MBL accounted for approximately 80% of all MB cases. Genetic analyses of 176 cases revealed four distinct molecular subgroups: WNT (14%), SHH (27%), group 3 (16%), and group 4 (43%). The central review reached a diagnosis of AT/RT for 3 cases that had been diagnosed as MB by the local pathologists. Immunohistochemically, WNT MBs showed nuclear accumulation of β-catenin protein, but the immunoreactivity was patchy in approximately one-quarter of WNT cases. GAB1 often exhibited little or no reactivity in the SHH subgroup. No reliable staining was observed for YAP1. All D/N MBL (16 cases) or MBEN (6 cases) were defined as SHH tumors. All MBEN cases were in infants (<3 years of age), and genetically subdivided into SHH-TP53 wild-type tumors. Variable degrees of anaplasia, including LC/A MB, occur across the genetic subgroups, and the LC/A MB WNT type was rare (2/24=8.3%) among WNT subgroups. This study demonstrated that the combination of morphological and molecular analyses can precisely diagnose MB. More robust, surrogate markers should be developed as ancillary diagnostic testing for subgroup classification. Further exploration of the clinical significance of the variable degree of LC/A histology and some subtypes (i.e. LC/A, WNT) will be necessary for risk stratification.

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