Technetium Pyrophosphate Scintigraphy Research Articles

Efficacy of Computed Tomography-Based Evaluation of Myocardial Extracellular Volume Combined With Red Flags for Early Screening of Concealed Cardiac Amyloidosis in Patients With Atrial Fibrillation.

The prevalence of transthyretin amyloid cardiomyopathy (ATTR-CM) in atrial fibrillation (AF) patients remains unclear. We explored the efficacy of computed tomography-based myocardial extracellular volume (CT-ECV) combined with red flags for the early screening of concealed ATTR-CM in AF patients undergoing catheter ablation.Methods and Results: Patients referred for AF ablation at Oita University Hospital were prescreened using the red-flag signs defined by echocardiographic or electrocardiographic findings, medical history, symptoms, and blood biochemical findings. Myocardial CT-ECV was quantified in red flag-positive patients using routine pre-AF ablation planning cardiac CT with the addition of delayed-phase cardiac CT scans. Patients with high (>35%) ECV were evaluated using technetium pyrophosphate (99 mTc-PYP) scintigraphy. A cardiac biopsy was performed during the planned AF ablation procedure if 99 mTc-PYP scintigraphy was positive. Between June 2022 and June 2023, 342 patients were referred for AF ablation. Sixty-seven (19.6%) patients had at least one of the red-flag signs. Myocardial CT-ECV was evaluated in 57 patients because of contraindications to contrast media, revealing that 16 patients had high CT-ECV. Of these, 6 patients showed a positive 99 mTc-PYP study, and 6 patients were subsequently diagnosed with wild-type ATTR-CM via cardiac biopsy and genetic testing. CT-ECV combined with red flags could contribute to the systematic early screening of concealed ATTR-CM in AF patients undergoing catheter ablation.

Challenges associated with managing treatment complications in an older patient with cardiac amyloidosis

BackgroundAmyloidosis, particularly wild-type transthyretin amyloidosis (ATTRwt), is an increasingly recognized cause of heart failure with preserved ejection fraction in the aging population. The complexity of managing ATTRwt in older patients underscores the necessity for individualized treatment approaches, yet clinical guidelines are lacking. This case report contributes to the understanding of ATTRwt management in the elderly, emphasizing the intricacies of medication tolerance and therapeutic decision-making.Case presentationAn 83-year-old Korean man with a history of hypertension presented with dyspnea and peripheral edema. Investigations including electrocardiography, transthoracic echocardiography, cardiac magnetic resonance, and Technetium pyrophosphate scintigraphy led to a diagnosis of ATTRwt cardiac amyloidosis. Initial management with heart failure medications, including an angiotensin-converting enzyme inhibitor, diuretic, and mineralocorticoid receptor antagonist, was modified due to evolving clinical presentations, such as hypotension and onset of atrial fibrillation. Challenges included intolerance to beta-blockers and bleeding complications from direct oral anticoagulant therapy. The patient’s treatment journey highlighted the need for personalized management strategies in older ATTRwt patients.ConclusionsThis case illustrates the challenges in diagnosing and managing ATTRwt amyloidosis in the elderly, particularly the complexities in medication management due to the patient’s age, comorbid conditions, and side effects. It underscores the importance of a tailored approach in managing ATTRwt in older populations and highlights the need for ongoing research and development of treatment strategies tailored to this demographic.

Echocardiographic score for the screening of cardiac amyloidosis with positive 99m technetium pyrophosphate scintigraphy result.

Speckle-tracking-derived strains in cardiac chambers may provide better solutions for transthyretin amyloid cardiomyopathy (ATTR-CM) screening. This study aimed to evaluate the efficacy of biventricular strain measurements using speckle tracking for screening 99m Tc-pyrophosphate (99m Tc-PYP) scintigraphy-positive cardiomyopathy, which is nearly equivalent to ATTR-CM. We performed a retrospective analysis of transthoracic echocardiographic studies using vendor-independent speckle tracking analysis in older patients (≥65 years) who underwent 99m Tc-PYP scintigraphy to evaluate the etiology of suspected ATTR-CM in our institute between January 2019 and December 2022. The entire cohort (n=89) was divided into two subgroups positive 99m Tc-PYP scan results (n=34) and negative 99m Tc-PYP scan results (n=55). In the multivariate analysis, posterior wall thickness (p=.003, odds ratio [OR]:1.48, 95% confidence interval [CI]:1.14 -1.92), left ventricular longitudinal strain apical/basal ratio (LVLSapi/bas) (p=.015, OR: 2.78, 95% CI: 1.23-6.32, and right ventricular longitudinal strain (RVLS) (p=.003, OR: 1.15, 95% CI: 1.05 -1.26) were selected to be the most representative echocardiographic findings in 99m Tc-PYP positive cardiomyopathy. The receiver operating characteristic analysis indicated that posterior wall thickness (p<.0001, area under the curve [AUC]: .821, cut-off value: 14.0mm), LVLSapi/bas (p<.001, AUC: .802, cut-off value: 2,16), and RVLS (p<.001, AUC: .791, cut-off value: -18.7%) could significantly detect 99m Tc-PYP positive results with an excellent credibility. Echocardiographic score points calculated using the summary of these three parameters in each patient revealed that a 2-point score had a fair sensitivity (85%) and an excellent specificity (93%), while a 1-point score had an excellent sensitivity (91%) and a modest specificity (53%). Our proposed echocardiographic screening tool for 99m Tc-PYP scintigraphy-positive cardiomyopathy may help clinicians manage patients with suspected ATTR-CM.

Abstract 15382: Geriatric HFpEF: When It Isn’t Hypertensive Heart Disease

Introduction: Transthyretin amyloid cardiomyopathy (ATTR) is thought to be a rare cause of HF, but recent studies showed this is the cause of 30% of HFpEF patients older than 75. With new treatments for amyloidosis, timely diagnosis has become critical. Case: A 78-female with HFpEF, HTN, and diabetes followed for progressive functional decline and hospitalizations for HFpEF exacerbations. EKG at baseline showed LBBB with normal QRS voltages. Serial echocardiogram (echo) showed an ejection fraction (EF) of 60-70%, mild LVH, and moderate TR with RVSP &gt;50 mmHg. Latest echo showed progressive LA enlargement, MR, a new small pericardial effusion, and EF 50%. Regadenoson nuclear scan showed normal perfusion with preserved LVEF. Despite adequate control of her HTN, dyspnea continued to worsen. Because of this, the diagnosis of Cardiac amyloidosis was considered. Further review revealed a history of Carpal Tunnel Syndrome (CTS) and speckled pattern within the ventricular wall on echo. A technetium pyrophosphate scintigraphy ( 99m Tc-PYP) confirmed ATTR with grade three uptake, AL amyloid was ruled out with normal SPEP/UPEP, and she was started on Tafamidis. Discussion: Cardiac amyloidosis should be considered in HFpEF patients with functional decline despite medical optimization. It can mimic LVH caused by HTN on echo and easily dismissed in patients with traditional risk factors, but presence of CTS or lumbar spinal stenosis can be supportive. Traditionally ATTR is associated with low-voltage EKG, however this patient presented with normal QRS voltages for years, making the diagnosis more elusive. Conclusion: Advances in noninvasive imaging and treatments allow for early diagnosis of cardiac amyloidosis, reducing morbidity and mortality. Diagnosis is often delayed or missed, as features could be subtle and mimic other more common cardiac disease. Screening&amp;nbsp; 99m Tc-PYP for elderly patients with HFpEF could translate to improved outcomes

Utility of scoring system including relative apical sparing pattern for screening cardiac amyloidosis in patients with left ventricular hypertrophy

Abstract Background Cardiac amyloidosis (CA) is an infiltrative disease mimicking left ventricular hypertrophy (LVH), although its prognosis is poorer than other diseases with LVH. Moreover, because CA is treatable, appropriate screening for CA is an important area of study for clinicians to prevent and treat the disease. Several imaging predictors of CA have been reported so far;. in particular, deformation parameters such as relative apical sparing patterns of longitudinal strain (RASP) may diagnose CA with better precision than conventional parameters. Accordingly, we hypothesized that the inclusion of deformation parameters into the established diagnostic parameters would permit derivation of a risk score for CA screening in patients with LVH. Thus, we aimed to 1) investigate the incremental benefits of deformation parameters over established diagnostic parameters for CA screening in patients with LVH; 2) determine the risk score to screen CA patients with LVH using all of these variables; and 3) externally validate the score. Methods We retrospectively studied 295 consecutive non-ischemic patients with LVH who underwent echocardiography as well as the detailed work-up for LVH (biopsy, technetium pyrophosphate scintigraphy (99mTc-PYP) or cardiac magnetic resonance imaging) (median age, 67 years; MWT, 12 mm). CA was diagnosed by biopsy or 99mTc-PYP. The base model consisted of age (≥65 [male], ≥70 [female]), low voltage in electrocardiography, and posterior wall thickness ≥14 mm in reference to previous studies. Continuous echocardiographic variables were binarized by the use of generally accepted external cutoff points to avoid best clinical scenario. Incremental benefits were assessed using receiver operating characteristic curve analysis and area under the curve (AUC) comparison. Multiple logistic regression analysis was performed to determine the risk score. The score was then validated in the external validation sample (N=178, median age, 70 years; MWT, 12 mm). Results CA was observed in 54 patients (18%) and of the several echocardiographic parameters studied, only RASP demonstrated a significant incremental benefit for the screening of CA over the base model (Figure A). After multiple logistic regression analysis in the prediction of CA with 4 variables (RASP and basal model components), each was assigned a numeric value based on its relative effect (Figure B). The incidence rate of CA clearly increased as the sum of the risk score increased (Figure C). The score had good discrimination ability, with an AUC of 0.87, a total score of ≥2 with 70% sensitivity and 90% specificity. Similarly, the discrimination ability of the score in the validation cohort was sufficient (AUC = 0.87). Conclusion Overall, we determined a simple risk score including RASP to screen CA. This score takes into account 4 common parameters used in daily practice, and therefore, has potential utility in risk stratification and management of patients with LVH. Figure 1 Funding Acknowledgement Type of funding source: Private hospital(s). Main funding source(s): Kitaishikai

Incremental benefits of echocardiographic indices over clinical parameters for screening cardiac amyloidosis in patients with left ventricular hypertrophy

Abstract Background Cardiac amyloidosis (CA), characterized by amyloid protein deposition in the heart, is a treatable disease. Although left ventricular (LV) wall thickness is the most established imaging predictor for CA, several echocardiographic indices including deformation parameters also contribute to the screening of CA. However, it is unclear whether additive values of echocardiographic indices have greater benefit over the conventional clinical predictors for the screening of CA. Therefore, we sought to compare the incremental benefits of echocardiographic indices over the clinical parameters for the screening of CA and externally validate their incremental benefits. Methods We retrospectively studied 295 consecutive patients (median age, 67 years; male, 65%; mean LV wall thickness (MWT), 12 mm) with LV hypertrophy who underwent echocardiography as well as the detailed work-up for myocardium (Biopsy, technetium pyrophosphate scintigraphy (99mTc-PYP) or cardiac magnetic resonance imaging). CA was diagnosed through biopsy or 99mTc-PYP. The clinical model considers patients' age and the low-voltage in electrocardiography in reference to previous studies. Continuous echocardiographic variables were represented in binary through generally accepted external cutoff points. The incremental benefits of the echocardiography findings over the clinical model were assessed using with the help of both receiver-operated characteristic curve analysis and comparison of area under the curves. Furthermore, these incremental benefits were validated in the external validation sample (median age, 70 years; male, 69%; MWT, 12 mm). Results Among the enrolled patients, CA was observed in 18% of cases. Table presents the results of this study. Of the echocardiographic parameters, relative apical sparing pattern (RASP) was the greatest contributor for improvement of diagnostic accuracy of the clinical model. The next greatest contributor was LV wall thickness, followed by left atrial reservoir strain (LAS), E/e', left atrial volume index, ejection fraction strain ratio, and pericardial effusion, respectively. Similarly, RASP, LV wall thickness, global longitudinal strain, ejection fraction, LAS, and granular sparkling showed significant incremental benefit in the validation cohort. Only mean wall thickness, LV wall thickness, LAS, E/e' and RASP consistently improved the diagnostic accuracy of the clinical model. Conclusion During the screening process, adding LV wall thickness, LAS, and RASP to the clinical parameters may be useful for the accurate diagnosis of CA in patients with LV hypertrophy. Figure 1 Funding Acknowledgement Type of funding source: None

Validation of the Kumamoto criteria for prediction of 99m technetium pyrophosphate scintigraphy positivity as a strategy for diagnosis of transthyretin cardiac amyloidosis: A retrospective cohort study in Kochi

BackgroundEarly diagnosis of transthyretin cardiac amyloidosis (ATTR-CA) is important. The aim of this study was to validate the ‘Kumamoto criteria’ for prediction of technetium-99m pyrophosphate (99mTc-PYP) scintigraphy positivity. MethodsOne hundred fifty patients (median age: 79.4 years, 117 males) with the possibility of ATTR-CA who underwent 99mTc-PYP scintigraphy were assessed. We divided the patients into 4 groups (groups with score of 0–3) according to the Kumamoto criteria by total points for the following 3 factors: high-sensitivity cardiac troponin T (hs-cTnT) ≥0.0308 ng/ml, left ventricle posterior wall thickness ≥13.6 mm, and wide QRS (QRS ≥ 120 ms). ResultsSeventy patients (46.7%) were positive for 99mTc-PYP scintigraphy. 99mTc-PYP positivity rates in the groups with score of 0, 1, 2, and 3 were 4%, 39%, 69%, and 89%, respectively. Compared with the original Kumamoto cohort, our patients in the score 1 group showed a relatively high rate of 99mTc-PYP positivity because hs-cTnT as one of the positive factors had high ability to discriminate the disease. The sensitivity and negative predictive value of hs-cTnT ≥0.0308 ng/ml for 99mTc-PYP positivity were 97.1% and 93.9%. ConclusionsIn the Kochi validation cohort, the Kumamoto criteria were useful for predicting 99mTc-PYP positivity. However, patients in the score 1 group should be assessed cautiously for the possibility of ATTR-CA if the hs-cTnT value is high.

Transthyretin Cardiac Amyloidosis as Diagnosed by 99mTc-PYP Scanning in Patients with Acute Heart Failure and Preserved Ejection Fraction.

Transthyretin amyloid deposition is present in 17% of autopsies of patients with heart failure and a preserved ejection fraction (HFpEF). Technetium-pyrophosphate scintigraphy (Tc-PYP) is sensitive and specific to diagnose cardiac transthyretin amyloid deposition (ATTR). The prevalence of ATTR by Tc-PYP was evaluated along with echocardiographic parameters in patients with HFpEF. One-hundred consecutive patients with HFpEF, who had Tc-PYP, were retrospectively evaluated. Echocardiographic variables were analyzed to compare patients with positive versus negative ATTR infiltration. Myocardial ATTR was present in 19% of patients. Individuals with ATTR were older with a mean age of 82 ± 7 versus 75 ± 13 years (P = 0.03), had increased left ventricular hypertrophy with the interventricular septum measuring 1.6 (IQR, 1.4-2.0) versus 1.4 (IQR, 1.3-1.6) cm (P = 0.002), had a greater mean left ventricular mass index of 160 ± 50 g/m versus 131 ± 44 g/m (P = 0.01), and a reduced global longitudinal strain measuring -11% (IQR, -9 to -12) versus -12% (IQR, -10 to -16), P = 0.04. The prevalence of ATTR myocardial deposition demonstrated by Tc-PYP in patients with HFpEF is comparable to that of autopsy studies. It is more common in older patients, with increased left ventricular hypertrophy and reduced global longitudinal strain.

3078Usefulness of a semi-quantitative and layer-specific assessment of the relative apical sparing pattern of longitudinal strain for the identification of cardiac amyloidosis

Abstract Background The relative apical sparing pattern (RASP) of left ventricular longitudinal strain (LS) is determined using a strain polar map, while global longitudinal strain is measured using speckle-tracking echocardiography, and it is frequently associated with cardiac amyloidosis (CA). However, the definition of visual RASP is ambiguous, and this leads to insufficient reproducibility, whereas quantitative RASP takes time and leads to difficulty in the clinical application. Generally, amyloid predominantly accumulates in the endo-myocardial layer. As such, layer-specific analysis of RASP may more accurately identify CA. Therefore, the aims of this study were to explore the reproducible and easy definition of RASP for identifying CA and investigate the effect of layer-specific analysis on the assessment. Methods A total of 40 patients with CA diagnosed by biopsy and technetium pyrophosphate scintigraphy were compared with 120 control patients matched for mean left ventricular wall thickness (40 aortic stenosis, 40 hypertrophic cardiomyopathy, and 40 hypertensive heart disease). We compared the discriminative abilities of three definitions of RASP (visual, quantitative, and semi-quantitative). According to a previous paper, visual RASP was defined as visual reduction of LS in the basal and middle LS segments (light red or blue) relative to the apical LS (red). Quantitative RASP was calculated using the following formula: average apical LS/(average basal LS + average mid-ventricle LS), then binarized by the optimal cut-off value for predicting CA. Semi-quantitative RASP was defined as reduction of LS (≥-10%) in five or more segments out of the basal six segments, relative to apical LS (≤-15%). Sample cases are shown in Figure (left). Visual and semi-quantitative RASP were independently assessed by two blinded sonographers. The RASP at the endo-myocardial and all layers was evaluated using customized software. The concordance was assessed using the kappa statistic, whereas the discriminative ability was assessed using receiver operating characteristic curve analysis. Results The concordance of visual RASP was modest but its semi-quantitative RASP was perfect (Table right). The discriminative ability of semi-quantitative RASP at each layer was significantly better than that of visual RASP and close to that of the binary quantitative RASP. Additionally, the discriminative abilities of visual (p=0.10) and semi-quantitative (p=0.11) RASP at the endo-myocardial layer appeared to be better than those at all layers. Conclusions The assessment method of semi-quantitative RASP is easy and highly reproducible. Furthermore, it accurately discriminates CA. In addition, assessment at the endo-myocardial layer potentially improves the discriminative ability.

Transthyretin Cardiac Amyloidosis in the Black Population: A Ten-Year Experience

Introduction Transthyretin (ATTR) cardiac amyloidosis (CA) is becoming increasingly recognized as an underlying pathology of heart failure with preserved ejection fraction (HFpEF) in elderly patients. One in 25 African Americans are carriers of the hereditary variant (ATTRv) Val-122-Ile point mutation, increasing the risk of developing ATTR-CA. Age-related wild-type (ATTRwt) is the most common cause of ATTR-CA overall, seen most often in white males. We present the racial distribution of ATTR-CA at our institution from 2008-2018, focusing on the black subpopulation. Methods This is a retrospective review of patients with ATTR-CA seen between 2008 and 2018 at the Cleveland Clinic. Diagnosis of ATTR-CA was established via endomyocardial biopsy or non-biopsy protocol with technetium pyrophosphate scintigraphy. Genetic typing was obtained when possible. Results Of 350 patients with ATTR-CA, 110 (31%) identified as racially black or African American. In the black population with available genetic testing (n = 96), 76% of cases were due to Val-122-Ile ATTRv and 22% were due to ATTRwt. Variants Asp-18-Asn and Glu-54-Gln were also represented with one case each. Average age at diagnosis in the black subpopulation was 75 ±9 years and 78% of patients were male. History of hypertension was seen in 83% of patients, and diabetes mellitus was seen in 38%. Carpal tunnel syndrome was present in 46% of patients. There were no statistically significant differences in demographics, clinical or lab parameters between blacks with ATTRv Val-122-Ile versus ATTRwt. Conclusions Over the course of a decade, black patients accounted for nearly one-third of all patients with ATTR-CA seen at our institution. The majority had hereditary ATTR due to the Val-122-Ile mutation, but nearly one quarter had ATTRwt. Additionally, there were two rarer mutations found. Current estimates of the prevalence of ATTR-CA in the black population focus on those with the Val-122-Ile variant at risk for development of hereditary ATTR, but do not account for blacks who may develop ATTRwt. The significant prevalence of ATTRwt in our cohort suggests ATTR-CA may be more prevalent in the black population. With accessibility to non-invasive diagnostic methods, recognition of ATTR-CA in black patients is critical as therapies become available for this progressive disease.

Noninvasive diagnosis of hereditary transthyretin-related cardiac amyloidosis

Rationale:Cardiac transthyretin amyloidosis is a progressive and fatal cardiomyopathy for which several promising therapies are in development. The diagnosis is frequently delayed or missed because of the limited specificity of clinical manifestations, routine electrocardiogram, echocardiography and the traditional requirement for endomyocardial biopsy confirmation.Patient concerns:A 68-year-old female had suffered from lumbago for 5 years with progressive weakness, numbness in both lower limb.Diagnosis:The patient's clinical signs were not specific, but cardiac amyloidosis was suspected based on relative left ventricular apical sparing of longitudinal strain on echocardiography and continuous elevated serum levels of cardiac biomarkers (ultrasensitive cardiac troponin I and NT-proBNP). She was finally diagnosed hereditary transthyretin-related cardiac amylodosis by specific findings of cardiovascular magnetic resonance imaging (CMR), 99m-technetium pyrophosphate (99mTc-PYP) scintigraphy and genetic testing.Interventions:The patient received medications including diuretics, beta-blockers and angiotensin-converting enzyme inhibitors at the time of hospitalization. Ultimately, however, she refused further treatments and requested discharge from our hospital.Outcomes:A series of noninvasive technique enables the diagnosis of hereditary transthyretin-related cardiac amyloidosis.Lessons:While endomyocardial biopsy is not able to performed, this case demonstrates that a combination of noninvasive techniques, especially CMR, nuclear imaging, and genetic testing, may help us to make a correct diagnosis of hereditary transthyretin-related cardiac amyloidosis.

Hereditary cardiac amyloidosis associated with Pro24Ser transthyretin mutation: a case report

BackgroundTransthyretin amyloidosis is a systemic disorder caused by extracellular deposition of insoluble amyloid fibrils in peripheral and autonomic nerves, heart, kidney, gastrointestinal tract, and other organs. Hereditary transthyretin amyloidosis is an autosomal dominant disease. More than 120 mutations have been reported in the transthyretin gene with considerable phenotypic heterogeneity and geographic diversity. Among them, a sporadic case of hereditary transthyretin amyloidosis with cardiac-predominant phenotype is very rare, progressive, and potentially fatal if left undiagnosed. However, a clinical diagnosis of cardiac amyloidosis still remains challenging due to non-specific symptoms, and less sensitivity and specificity of medical examinations.Case presentationA 60-year-old Japanese man with a history of embolic stroke and hypertrophic cardiomyopathy visited our department for heart failure. The present case exhibited only cardiomyopathy without any clinical signs of systemic amyloidosis manifested as carpal tunnel syndrome, polyneuropathy, or autonomic dysfunction. An echocardiogram revealed severe asymmetric left ventricular hypertrophy, biatrial dilatation, pericardial effusion, and preserved left ventricular ejection fraction of 50% with severe diastolic dysfunction. Technetium pyrophosphate scintigraphy indicated marked diffuse myocardial uptake of technetium pyrophosphate, strongly suggesting transthyretin cardiac amyloidosis, which was firmly confirmed by a left ventricular endomyocardial biopsy. Genetic analysis demonstrated a transthyretin C70T (Pro24Ser) heterozygous mutation. Tafamidis, a transthyretin stabilizer, was started. His cardiac symptoms remained unchanged for 12 months.ConclusionsHere we report the case of a patient with hereditary cardiac amyloidosis associated with a Pro24Ser mutation in transthyretin, which is the first case reported in Japan. Technetium pyrophosphate scintigraphy was extremely useful for definitive diagnosis. Thus, we propose that the nuclear imaging technique should be taken into account even for an exploratory diagnosis of transthyretin cardiac amyloidosis.

Tenosynovial and Cardiac Amyloidosis in Patients Undergoing Carpal Tunnel Release

BackgroundPatients with cardiac amyloidosis often have carpal tunnel syndrome that precedes cardiac manifestations by several years. However, the prevalence of cardiac involvement at the time of carpal tunnel surgery has not been established. ObjectivesThe authors sought to identify the prevalence and type of amyloid deposits in patients undergoing carpal tunnel surgery and evaluate for cardiac involvement. The authors also sought to determine if patients with soft tissue transthyretin (TTR) amyloid had abnormal TTR tetramer kinetic stability. MethodsThis was a prospective, cross-sectional, multidisciplinary study of consecutive men age ≥50 years and women ≥60 years undergoing carpal tunnel release surgery. Biopsy specimens of tenosynovial tissue were obtained and stained with Congo red; those with confirmed amyloid deposits were typed with mass spectrometry and further evaluated for cardiac involvement with biomarkers, electrocardiography, echocardiography with longitudinal strain, and technetium pyrophosphate scintigraphy. Additionally, serum TTR concentration and tetramer kinetic stability were examined. ResultsOf 98 patients enrolled (median age 68 years, 51% male), 10 (10.2%) had a positive biopsy for amyloid (7 ATTR, 2 light chain [AL], 1 untyped). Two patients were diagnosed with hereditary ATTR (Leu58His and Ala81Thr), 2 were found to have cardiac involvement (1 AL, 1 ATTR wild-type), and 3 were initiated on therapy. In those patients who had biopsy-diagnosed ATTR, there was no difference in plasma TTR concentration or tetramer kinetic stability. ConclusionsIn a cohort of patients undergoing carpal tunnel release surgery, Congo red staining of tenosynovial tissue detected amyloid deposits in 10.2% of patients. Concomitant cardiac evaluation identified patients with involvement of the myocardium, allowing for implementation of disease-modifying therapy. (Carpal Tunnel Syndrome and Amyloid Cardiomyopathy; NCT02792790)

PREDICTORS OF A POSITIVE TECHNETIUM PYROPHOSPHATE SCAN IN PATIENTS WITH SUSPECTED CARDIAC AMYLOIDOSIS

Background: Transthyretin cardiac amyloidosis (ATTR CA) is an underdiagnosed cause of heart failure with reported worse outcomes. Technetium Pyrophosphate scintigraphy (PYP) has re-emerged as a tool to non-invasively diagnose ATTR CA. We sought to determine the clinical and echocardiographic

Serial scanning with technetium pyrophosphate (99mTc-PYP) in advanced ATTR cardiac amyloidosis

BackgroundDevelopment of noninvasive imaging modalities to quantify amyloid burden over time is an unmet clinical need. Technetium pyrophosphate (99mTc-PYP) scintigraphy is a simple and widely available radiotracer useful to differentiate transthyretin from light-chain amyloidosis in patients with advanced cardiac amyloidosis. We examined the utility of serial 99mTc-PYP scanning to quantify amyloid burden over time in TTR cardiac amyloidosis (ATTR-CA). Methods and ResultsTwenty subjects with ATTR-CA (10 wild type, 10 mutant) underwent serial 99mTc-PYP planar cardiac imaging. Cardiac retention was assessed both semiquantitatively (visual score 0, no uptake to 3, uptake greater than bone) and quantitatively (region of interest drawn over the heart, copied, and mirrored over the contralateral chest) to calculate a heart-to-contralateral (H/CL) ratio. Index scan mean visual score and H/CL were 3.0 ± 0.2 and 1.79 ± 0.2, respectively, and after an average 1.5 ± 0.5 years follow-up, did not differ, 3.0 ± 0.2, P = .33 and 1.76 ± 0.2, P = .44. H/CL change was minimal, 0.03 ± 0.17, did not correlate with time between scans, r = 0.19, P = .43, and was observed despite obvious clinical progression (increase in troponin ≥ 0.1 ng/mL, BNP ≥ 400 pg/mL, NYHA class, and/or death). ConclusionsSerial 99mTc-PYP scanning in subjects with advanced ATTR-CA does not show significant changes over an average 1.5 years of follow-up despite obvious clinical progression.

The Incidence of Perioperative Myocardial Infarction in Men Undergoing Noncardiac Surgery

To determine the incidence of and risk factors for perioperative myocardial infarction with noncardiac surgery and to test the accuracy of a risk stratification system. Prospective cohort study. A large urban Veterans Affairs hospital. A total of 1487 men older than 40 years undergoing major, nonemergent, noncardiac operations. Infarction was established by at least two of the following: development of new Q waves, typical change in creatine kinase MB, and positive technetium pyrophosphate scintigraphy. Patients were stratified preoperatively into high-, intermediate-, low-, and negligible-risk strata based on clinical markers corresponding to different levels of coronary artery disease prevalence. Patients with coronary disease (high-risk stratum) had a 4.1% incidence of infarction (13 of 319; 95% CI, 1.8% to 6.4%); patients with peripheral vascular disease but no evidence of coronary disease (intermediate-risk stratum) had a 0.8% incidence (2 of 260, upper bound of CI, 2.0%); patients with high atherogenic risk factor profiles but no clinical atherosclerosis (low-risk stratum) had a 0% incidence (0 of 256, upper bound of CI, 1.2%). No cardiac deaths occurred in 652 men who had no atherosclerosis and low atherogenic risk factor profiles (the negligible-risk stratum). Factors independently associated with infarction included age more than 75 years (adjusted odds ratio, 4.77; CI, 1.17 to 19.41), signs of heart failure on the preoperative examination (adjusted odds ratio, 3.31; CI, 0.96 to 11.38), coronary disease (adjusted odds ratio, 10.39; CI, 2.27 to 47.46), and a planned vascular operation (adjusted odds ratio, 3.72; CI, 1.12 to 12.37). Coronary artery disease is the major risk factor for perioperative infarction. The stratification scheme identifies subsets of patients with different risks, and finer within-stratum distinctions can be made using additional variables.

Further evidence supporting the concept of T-wave memory: observation in patients having undergone high-energy direct current catheter ablation of the Wolff-Parkinson-White syndrome.

Following successful high-energy direct current catheter ablation of posterior septal accessory pathways, serial T-wave changes were observed in seven patients with Wolff-Parkinson-White syndrome, but not in two patients with a concealed accessory bypass pathway. Following catheter ablation in the patients with Wolff-Parkinson-White syndrome, the previously upright T-waves in the inferior electrocardiographic leads became inferiorly directed. Serial electrocardiograms following catheter ablation revealed slow regression of these T-wave changes over time. In contrast, in the two patients in whom a concealed accessory pathway was present, the T-waves prior to and following the procedure were upright. In these two patients, serial electrocardiograms showed no evolution of the T-wave vector over the same time period. Although a small rise in myocardial CK enzymes was observed, ischaemic injury in the area of catheter ablation could not be documented by post-procedure echocardiography, tomographic thallium scintigraphy, technetium pyrophosphate scintigraphy or coronary angiography with left ventriculography. We conclude that the observed T-wave changes in the patients with Wolff-Parkinson-White syndrome may be a result of the change in depolarization sequence resulting from loss of pre-excitation, and that the time-dependent nature of these T-wave changes is consistent with the concept of T-wave 'memory'.

Simultaneous mechanical clot fragmentation and pharmacologic thrombolysis in acute massive pulmonary embolism

that technetium pyrophosphate scintigraphy has a very low sensitivity for cardiac amyloidosis. Our results suggest that none of the noninvasive parameters evaluated in this study for diagnosis of cardiac amyloidosis are highly sensitive. Thus, low voltage had a sensitivity of 80%, myocardial sparkling 45%, and technetium-99m-pyrophosphate uptake 23%. This low sensitivity, coupled with the previously mentioned low specificity, makes these findings unattractive for diagnosis of amyloidosis. An alternative approach to noninvasive diagnosis of cardiac amyloidosis was suggested by Carrol et al’ who noted that patients with cardiac amyloidosis tend to have low electrocardiographic voltages and high echocardiographic estimates of left ventricular mass. When the voltage/mass relation for cardiac amyloid patients is compared with that for patients with aortic valve (high voltage, high mass) or pericardial (low voltage, low mass) disease, clear differences emerge. Plotting voltage/mass relations for the patients in this study on a diagram developed by Carrol et al (Figure 1) would place 12 patients (80%) in a cardiac amyloid range (voltage 10 cm2/m2). This result makes the voltage/mass relation an attractive approach for diagnosis of cardiac amyloidosis. However, there are no reliable estimates of specificity for this technique. In conclusion, the present study examined several frequently used findings obtained by noninvasive techniques with regard to their sensitivity for diagnosis of cardiac amyloidosis. Our results suggest that the sensitivity ‘of both myocardial technetium uptake and echocardiographic observation of sparkling are low. However, voltage/mass relation diagrams appear to be a very promising technique for noninvasive diagnosis of these patients. The use and specificity of this technique need further study.

Postoperative haemodynamic and pharmacological responses in patients with positive technetium pyrophosphate single-photon emission computed tomography following CABG.

The aim of this prospective study was to evaluate the postoperative haemodynamic variables and medication requirements in patients with perioperative myocardial infarction (PMI), following elective coronary artery bypass graft (CABG) surgery, as documented by technetium pyrophosphate scintigraphy using single-photon emission computed tomography (TcPPi-SPECT). A high-dose fentanyl anaesthetic technique was applied. Twelve of 58 patients (21%) developed PMI with an infarcted myocardial mass of 35.7 +/- 3.9 g. Over the 48 hr postoperative period, patients with positive TcPPi-SPECT (n = 12) did not differ from those with negative TcPPi-SPECT (n = 46) in mean heart rate (below 100 bpm), systolic blood pressure (100-120 mmHg) or central venous pressure (8-16 mmHg). However, patients with positive TcPPi-SPECT had higher pulmonary artery diastolic pressures at 5-8 hr after surgery. No differences were found in the incidence and dosage requirements for postoperative sedative or vasoactive drugs (morphine, diazepam, propranolol, lidocaine, nitroglycerin and nitroprusside) between the two groups. There was no difference in the incidence of dopamine requirement between the groups (positive-scan: 16.7%, negative-scan: 13.0%). However, the dopamine dosage for inotropic support was higher in the positive TcPPi-SPECT group over 24 hr (318.5 +/- 125.2 mg vs 71.2 +/- 24.7 mg, P less than 0.05) and 48 hr (869.1 +/- 19.0 mg vs 142.3 +/- 49.4 mg, P less than 0.001) periods after surgery. We postulate that careful control of postoperative haemodynamic variables did not prevent but may limit the extent of PMI in elective CABG patients.

Usefulness of technetium pyrophosphate scintigraphy in demonstrating cardiac amyloidosis in persons aged 85 years and older

Necropsy examination detects cardiac amyloidosis in at least 20% of persons 85 years of age or older. 1 The amount detected is rarely extensive enough to produce clinical findings. 2 Technetium-99m pyrophosphate scintigraphy, a diagnostic tool for the identification of acute myocardial infarction, often demonstrates dense biventricular uptake of tracer in patients with cardiac amyloidosis. 3 We postulated that technetium-99m pyrophosphate scanning might also identify “senile” amyloidosis of the myocardium. If that were true, it might also be useful for assessing the prevalence of cardiac amyloid in the very elderly and less valuable for recognizing myocardial infarction in that age group. To ascertain the prevalence of a positive technetium-99m pyrophosphate scan in an elderly population, we undertook the following study.