Validation of the Diagnostic Criteria for IgG4-Related Periaortitis/Periarteritis and Retroperitoneal Fibrosis (IgG4PA/RPF) 2018, and Proposal of a Revised 2023 Version for IgG4-Related Cardiovascular/Retroperitoneal Disease.

BACKGROUND: In 2018, diagnostic criteria were introduced for IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis (PA/RPF). This study assessed the existing criteria and formulated an improved version.Methods and Results: Between August 2022 and January 2023, we retrospectively analyzed 110 Japanese patients diagnosed with IgG4-related disease (IgG4-RD) involving cardiovascular and/or retroperitoneal manifestations, along with 73 non-IgG4-RD patients ("mimickers") identified by experts. Patients were stratified into derivation (n=88) and validation (n=95) groups. Classification as IgG4-RD or non-IgG4-RD was based on the 2018 diagnostic criteria and various revised versions. Sensitivity and specificity were calculated using experts' diagnosis as the gold standard for the diagnosis of true IgG4-RD and mimickers. In the derivation group, the 2018 criteria showed 58.5% sensitivity and 100% specificity. The revised version, incorporating "radiologic findings of pericarditis", "eosinophilic infiltration or lymphoid follicles", and "probable diagnosis of extra-PA/-RPF lesions", improved sensitivity to 69.8% while maintaining 100% specificity. In the validation group, the original and revised criteria had sensitivities of 68.4% and 77.2%, respectively, and specificities of 97.4% and 94.7%, respectively. CONCLUSIONS: Proposed 2023 revised IgG4-related cardiovascular/retroperitoneal disease criteria show significantly enhanced sensitivity while preserving high specificity, achieved through the inclusion of new items in radiologic, pathological, and extra-cardiovascular/retroperitoneal organ categories.

Comprehensive genetic screening for vascular Ehlers-Danlos syndrome through an amplification-based next-generation sequencing system.

Vascular Ehlers-Danlos syndrome (vEDS) is a hereditary connective tissue disorder (HCTD) characterized by arterial dissection/aneurysm/rupture, sigmoid colon rupture, or uterine rupture. Diagnosis is confirmed by detecting heterozygous variants in COL3A1. This is the largest Asian case series and the first to apply an amplification-based next-generation sequencing through custom panels of causative genes for HCTDs, including a specific method of evaluating copy number variations. Among 429 patients with suspected HCTDs analyzed, 101 were suspected to have vEDS, and 33 of them (32.4%) were found to have COL3A1 variants. Two patients with a clinical diagnosis of Loeys-Dietz syndrome and/or familial thoracic aortic aneurysm and dissection were also found to have COL3A1 variants. Twenty cases (57.1%) had missense variants leading to glycine (Gly) substitutions in the triple helical domain, one (2.9%) had a missense variant leading to non-Gly substitution in this domain, eight (22.9%) had splice site alterations, three (8.6%) had nonsense variants, two (5.7%) had in-frame deletions, and one (2.9%) had a multi-exon deletion, including two deceased patients analyzed with formalin-fixed and paraffin-embedded samples. This is a clinically useful system to detect a wide spectrum of variants from various types of samples.

Plasma Globotriaosylsphingosine and α-Galactosidase A Activity as a Combined Screening Biomarker for Fabry Disease in a Large Japanese Cohort.

Fabry disease is an X-linked disorder of α-galactosidase A (GLA) deficiency. Our previous interim analysis (1 July 2014 to 31 December 2015) revealed plasma globotriaosylsphingosine as a promising primary screening biomarker for Fabry disease probands. Herein, we report the final results, including patients enrolled from 1 January to 31 December 2016 for evaluating the potential of plasma globotriaosylsphingosine and GLA activity as a combined screening marker. We screened 5691 patients (3439 males) referred from 237 Japanese specialty clinics based on clinical findings suggestive of Fabry disease using plasma globotriaosylsphingosine and GLA activity as primary screening markers, and GLA variant status as a secondary screening marker. Of the 14 males who tested positive in the globotriaosylsphingosine screen (≥2.0 ng/mL), 11 with low GLA activity (<4.0 nmol/h/mL) displayed GLA variants (four classic, seven late-onset) and one with normal GLA activity and no pathogenic variant displayed lamellar bodies in affected organs, indicating late-onset biopsy-proven Fabry disease. Of the 19 females who tested positive in the globotriaosylsphingosine screen, eight with low GLA activity displayed GLA variants (six classic, two late-onset) and five with normal GLA activity displayed a GLA variant (one classic) and no pathogenic variant (four late-onset biopsy-proven). The combination of plasma globotriaosylsphingosine and GLA activity can be a primary screening biomarker for classic, late-onset, and late-onset biopsy-proven Fabry disease probands.

The Association of Depression with Type D Personality and Coping Strategies in Patients with Coronary Artery Disease.

Objective Depression in patients with coronary artery disease (CAD) has been a risk factor for adverse cardiovascular events. However, personality types, strategies for coping with stressors, and their associations with depression have not been fully elucidated in patients with CAD. This study explored depression in patients with CAD and examined its association with personality types and coping strategies. Methods A prospective observational study of 89 patients with CAD was conducted between August 2016 and July 2018. The presence of depression and type D personality and types of coping strategies were measured one month after percutaneous coronary intervention. A logistic regression analysis was performed to identify characteristics associated with depression. Results Generally, the incidence of depression and type D personality was 55.1% and 44.9%, respectively. The incidence of depression in patients with type D and non-type D personality was 72.5% and 40.8%, respectively. Patients with type D personality coped less frequently using a planning strategy but frequently using a responsibility-shifting strategy. A logistic regression analysis showed that the presence of depression was significantly associated with type D personality and inversely associated with a planning strategy. Conclusion The high prevalence of depression in patients with CAD was associated with type D personality and a low rate of adoption of a planning strategy. Specific coping interventions in patients with CAD with type D personality may be potential targets for improving coping skills and preventing the development of depression.

Geriatric Nutritional Risk Index Predicts Cardiovascular Events in Patients at Risk for Heart Failure.

BACKGROUND: The geriatric nutritional risk index (GNRI) is a simple and objective nutritional assessment tool for elderly patients. Lower GNRI values are associated with a worse prognosis in patients with heart failure (HF). However, few data are available regarding the prognostic effect of the GNRI value for risk stratification in patients at risk for HF.Methods and Results:We retrospectively investigated 1,823 consecutive patients at risk for HF (Stage A/B) enrolled in the IMPACT-ABI Study. GNRI on admission was calculated as follows: 14.89×serum albumin (g/dL)+41.7×body mass index/22. Patients were divided into 2 groups according to the median GNRI value (107.1). The study endpoint was a composite of cardiovascular (CV) events, including CV death and hospitalization for worsening HF. Over a 4.7-year median follow-up, CV events occurred in 130 patients. In the Kaplan-Meier analysis, patients with low GNRI (<107.1, n=904) showed worse prognoses than those with high GNRI (≥107.1, n=919) (20.2% vs. 12.4%, P<0.001). In the multivariable Cox proportional hazards analysis, low GNRI was significantly associated with the incidence of CV events (hazard ratio: 1.48, 95% confidence interval: 1.02-2.14; P=0.040). CONCLUSIONS: The simple and practical assessment of GNRI may be useful for predicting CV events in patients with Stage A/B HF.

Predictive Value of Underweight Status for Patients With Peripheral Artery Disease With Claudication.

We evaluated whether underweight status is associated with poor prognosis in patients with peripheral artery disease (PAD) with claudication, excluding critical limb ischemia. We identified 441 claudicants hospitalized for cardiovascular disease between 2005 and 2012. Patients were divided into 4 groups according to body mass index (BMI): an underweight group (BMI < 18.5 kg/m2; n = 48), a normal group (BMI = 18.5-25.0 kg/m2; n = 286), an overweight group (BMI = 25.0-30.0 kg/m2; n = 92), and an obese group (BMI ≥ 30.0 kg/m2; n = 15). The mean follow-up period was 3.5 ± 1.9 years. The underweight group had significantly lower levels of hemoglobin, albumin, estimated glomerular filtration rate, triglycerides, and hemoglobin A1c; higher levels of C-reactive protein and B-type natriuretic peptide; and a higher prevalence of hemodialysis. The incidence of all-cause death and cardiovascular death was significantly higher in the underweight group (underweight vs normal, 77.1% vs 33.0%; P < .001 and 43.3% vs 14.4%; P < .001, respectively). In a multivariate Cox analysis, underweight status was an independent predictor of all-cause death (hazard ratio, 2.53; 95% confidence interval, 1.58-4.18; P < .001). Therefore, promoting weight gain, as well as managing cardiovascular disease, may be important for underweight patients with PAD.

Prognostic Value of Ankle-Brachial Index in Patients Undergoing Percutaneous Coronary Intervention: In-Hospital and 1-Year Outcomes From the SHINANO Registry.

Concomitant coronary and peripheral artery disease is associated with higher periprocedural and long-term percutaneous coronary intervention (PCI) complication rates. We evaluated in-hospital and 1-year clinical outcomes of patients with low or borderline ankle-brachial indexes (ABIs) undergoing PCIs in the drug-eluting stent era. We divided 1370 SHINANO registry patients into 3 groups-low (ABI ≤ 0.9), borderline (0.9 < ABI ≤ 1.0), and normal (1.0 ≤ ABI < 1.4). During the 1-year follow-up, more PCI-related complications occurred in the low and borderline ABI groups than in the normal ABI group (7.7% vs 8.8% vs 4.0%, respectively). Low ABI patients were more likely to experience adverse clinical events (6.3% vs 3.6% vs 3.0%, respectively; log-rank P = .020 for low vs normal ABI), with a hazard ratio of 2.27 (95% confidence interval, 1.12-4.61; P = .023), compared with patients with normal ABIs. Patients with abnormal ABIs had a significantly higher incidence of PCI-related complications and a less favorable 1-year prognosis. Routine ABI measurement before PCI may help predict PCI-related complication incidence and 1-year prognosis.

Ankle-brachial Index for the Prognosis of Cardiovascular Disease in Patients with Mild Renal Insufficiency.

Objective A low ankle-brachial index (ABI) is a known predictor for future cardiovascular events and mortality in patients with chronic kidney disease (CKD). While most prior studies have defined CKD as an estimated glomerular filtration rate (eGFR) <60 mL/min/1.73 m2, recent reports have suggested that the cardiovascular risk may be increased even in early stages of renal insufficiency. We hypothesized that a low ABI may predict future cardiovascular morbidity and mortality in patients with mild impairment of the renal function. Methods The IMPACT-ABI study was a retrospective, single-center, cohort study that enrolled and obtained ABI measurements for 3,131 patients hospitalized for cardiovascular disease between January 2005 and December 2012. From this cohort, we identified 1,500 patients with mild renal insufficiency (eGFR =60-89 mL/min/1.73 m2), and stratified them into 2 groups: ABI ≤0.9 (low ABI group; 9.2%) and ABI >0.9 (90.8%). The primary outcome measured was the cumulative incidence of major adverse cardiovascular events (MACE; cardiovascular death, myocardial infarction, and stroke). Results Over a mean follow-up of 5.0 years, 101 MACE occurred. The incidence of MACE was significantly higher in patients with low ABI than in those with ABI >0.9 (30.2% vs. 14.4%, log rank p<0.001). A low ABI was associated with MACE in a univariate Cox proportional hazard analysis. A low ABI remained an independent predictor of MACE in a multivariate analysis adjusted for cardiovascular risk factors (hazard ratio (HR): 2.27; 95% confidence interval (CI): 1.33-3.86; p=0.002). Conclusion Low ABI was an independent predictor for MACE in patients with mild renal insufficiency.

Prognostic Impact of Diastolic Wall Strain in Patients at Risk for Heart Failure.

Diastolic wall strain (DWS) is based on the linear elastic theory, according to which decreased wall thinning during diastole reflects reduced left ventricular compliance and thus increased diastolic stiffness. Increased diastolic stiffness as assessed by DWS is associated with a worse prognosis in patients who have heart failure (HF) with preserved ejection fraction. However, there are no data about the prognostic value of DWS derived by M-mode echocardiography in patients at risk for HF. We retrospectively enrolled 1829 consecutive patients without prior HF who were hospitalized for cardiovascular (CV) diseases in our hospital between 2005 and 2012. Patients were divided into two groups stratified by DWS (median value 0.34). The study endpoint was the composite of major adverse cardiovascular events (MACE), including all-cause death, myocardial infarction, stroke, and hospitalization for HF. Over a 4.2-year median follow-up, adverse events were observed in 322 patients (17.6%). In Kaplan-Meier analysis, patients with low DWS (≤ 0.34, n = 915) showed worse prognoses than those with high DWS (> 0.34, n = 914) (MACE incidence 39.4% versus 31.9%, P = 0.011). In multivariate Cox proportional hazards analysis after the adjustment for age, sex, and echocardiographic parameters, low DWS (≤ 0.34) was significantly associated with the incidence of MACE (hazard ratio: 1.26, 95% confidence interval: 1.01-1.59; P = 0 .045). In patients without prior HF, DWS is an independent predictor of MACE. Simple assessment of DWS might improve risk stratification for CV events in those patients.

Comparison of mid-term outcomes between patients with and without atrial fibrillation undergoing coronary stenting in the second-generation drug-eluting stent era: from the SHINANO registry.

Little is known about the mid-term outcomes of patients with atrial fibrillation (AF) who undergo coronary stenting in the second-generation drug-eluting stent (DES) era. We evaluated the 1-year outcomes of AF patients undergoing percutaneous coronary intervention (PCI) with second-generation DES. This retrospective cohort analysis used integrated data from the SHINANO registry, a prospective observational multicenter cohort study, which enrolled 1923 consecutive patients undergoing PCI for any coronary artery disease. We retrospectively recruited 917 of these patients (mean age, 71.3 ± 10.0 years; male, 77 %) who received PCI with 2nd generation DES. The primary endpoint was net adverse clinical events (NACE: cardiac death, stroke, MI, stent thrombosis, and major bleeding) at 1 year. The secondary endpoints were major adverse cardiovascular events (MACE: cardiac death, stroke, and MI), stroke, MI, and major bleeding at 1 year. One-year follow-up was completed in 871 (94.9 %) patients, of whom 85 had AF. The incidence of NACE (15.4 vs. 7.3 %, P = 0.008), MACE (10.6 vs. 5.4 %, P = 0.047), and major bleeding (6.0 vs. 2.3 %, P = 0.049) were all significantly higher in AF compared to non-AF patients. On multivariate analysis, AF was an independent predictor of NACE (HR 2.32, 95 % CI 1.24-4.34, P = 0.008). In the second-generation DES era, patients with AF undergoing PCI still have a poorer prognosis, with more thrombotic and bleeding events, than those without AF. More attention should be paid to the thrombotic and bleeding risk in AF patients undergoing PCI.

Failure of steroid withdrawal guided by 18F-FDG PET in a patient with cardiac sarcoidosis.

A 71-year-old man diagnosed with cardiac sarcoidosis (CS) 11 years previously underwent implantation of an implantable cardioverter defibrillator due to sustained ventricular tachycardia. Over past decade, his condition of CS did not progress on the maintenance steroid dose of 7.5 mg per day. We attempted to taper and discontinue steroids according to the results of fluorine-18-fluorodeoxyglucose positron emission tomography (18F-FDG PET). On the basis of the results, we reduced the oral steroid dose slowly. In spite of no abnormal 18F-FDG uptake in the myocardium, advanced atrioventricular conduction block and deterioration of the ventricular pacing threshold occurred during the course of steroid withdrawal. Plasma brain natriuretic peptide (BNP) increased from 94 to 842 pg/ml. It was necessary to add new ventricular and atrial leads to synchronize atrial and ventricular contractions, and the pacing mode for bradycardia was changed to dual-chamber DDD-60 ppm. Fatigue disappeared, and BNP levels decreased to 147 pg/ml. 18F-FDG PET might have a limit to detect small scattered inflammatory foci. This case highlights the need for caution when reducing steroid doses in CS patients, guided by 18F-FDG PET only. .

Inflammatory Cytokine Levels After Endovascular Therapy in Patients With Peripheral Artery Disease.

We evaluated the impact of endovascular therapy (EVT) on inflammatory cytokine levels and its relationship with in-stent restenosis in patients with peripheral artery disease. The study prospectively enrolled 35 patients with intermittent claudication who underwent EVT of the iliofemoral artery. Levels of interleukin 6 (IL-6), monocyte chemoattractant protein-1 (MCP-1), and tumor necrosis factor α (TNF-α) were measured using enzyme-linked immunosorbent assay before and at 2 hours, 4 hours, and 3 months after EVT. All cytokine levels increased significantly after EVT (IL-6 [pg/mL]: from 1.51 [0.84-1.93] before EVT to 6.97 [4.05-20.41] at 2 hours and 13.29 [4.57-31.88] at 4 hours; MCP-1 [pg/mL]: from 326.65 [265.60-406.55] before EVT to 411.18 [341.21-566.27] at 2 hours and 519.36 [383.58-644.85] at 4 hours; TNF-α [pg/mL]: from 1.08 [0.77-1.29] before EVT to 1.25 [0.94-1.81] at 2 hours and 1.27 [0.95-1.59] at 4 hours, all P < .001). However, cytokine levels did not differ significantly between lesions with and without in-stent restenosis. Overall, our results suggest that EVT significantly increases IL-6, MCP-1, and TNF-α levels in the ischemic leg, but this effect is not associated with a higher rate of in-stent restenosis.

Diagnostic advantage of stress computed tomography myocardial perfusion over single-photon emission computed tomography for the assessment of myocardial ischemia.

BACKGROUND: This study compared adenosine stress computed tomography myocardial perfusion (CTP) with single-photon emission computed tomography (SPECT) in the diagnosis of functionally significant coronary artery stenosis using fractional flow reserve (FFR) as reference standard. METHODS: We included a total of 93 coronary arteries from 31 patients in whom at least one vessel with ≥50% stenosis was detected with computed tomography coronary angiography. All patients underwent both SPECT and adenosine stress CTP, followed by invasive coronary angiography (ICA) and FFR. Diagnostic accuracy between CTP and SPECT was compared according to positive findings of either ≥99% stenosis on ICA or FFR ≤0.8. RESULTS: Among 78 vessels eligible for the quantitative analyses, significant coronary artery disease (CAD) was diagnosed in 22 vessels of 19 patients. Comparison of CTP vs. SPECT for sensitivity, specificity, positive predictive value (PPV), negative predictive value, and accuracy in detecting significant CAD were 59% vs. 18%, 96% vs. 93%, 87% vs. 50%, 86% vs. 74%, and 86% vs. 72%, respectively. CONCLUSIONS: CTP demonstrated a significant diagnostic advantage over SPECT in the identification of significant CAD, especially in terms of sensitivity and PPV. Adenosine stress CTP is useful for the noninvasive diagnosis of functionally significant CAD.

Prediction of 1-year clinical outcomes using the SYNTAX score in patients with prior heart failure undergoing percutaneous coronary intervention: sub-analysis of the SHINANO registry.

Although coronary artery disease (CAD) is common in patients with heart failure (HF), little is known about the prognostic significance of coronary lesion complexity in patients with prior HF undergoing percutaneous coronary intervention (PCI). The aim of this study was to investigate whether the coronary Synergy between Percutaneous Coronary Intervention with TAXus and Cardiac Surgery (SYNTAX) score could improve risk stratification in HF patients with CAD. Two hundred patients (mean age 73 ± 11 years, left ventricular ejection fraction 49 ± 15 %) with prior HF who underwent PCI were divided into two groups stratified by SYNTAX score (median value 12) and tracked prospectively for 1 year. The study endpoint was the composite of major adverse cardiovascular events (MACE), including all-cause death, myocardial infarction, stroke, and hospitalization for worsening HF. Adverse events were observed in 39 patients (19.5 %). Patients with high SYNTAX scores (n = 100) showed worse prognoses than those with low scores (n = 100) (26.0 vs. 13.0 %, respectively, P = 0.021). In multivariate Cox-regression analysis, SYNTAX score ≥12 was significantly associated with MACE (hazard ratio: 1.99, 95 % confidence interval: 1.02-3.97; P = 0.045). In patients with prior HF and CAD, high SYNTAX scores predicted a high incidence of MACE. These results suggest that the SYNTAX score might be a useful parameter for improving risk stratification in these patients.

Importance of cystatin C and uric acid levels in the association of cardiometabolic risk factors in Japanese junior high school students.

BACKGROUND: Serum cystatin C (CysC), a novel marker of renal function, is associated with the components of metabolic syndrome in adults. Little is known about the utility of CysC and its association with cardiometabolic risks in young subjects. METHODS AND RESULTS: In a cohort of 454 Japanese junior high school students, the distribution of serum CysC levels and associated variables were analyzed. CysC levels were significantly higher in boys than in girls (0.92±0.10mg/L vs. 0.77±0.08mg/L, p<0.001). CysC was significantly correlated with serum creatinine (r=0.473, p<0.001), and serum uric acid (SUA) (r=0.546, p<0.001). Multivariable regression analysis revealed significant associations between CysC and SUA in all subjects (ß=0.241, p<0.001), and in boys and girls separately (ß=0.264 and 0.240, respectively, both p<0.001). Importantly, subjects with elevation of both serum CysC and SUA levels had the highest ratio of triglyceride to high-density lipoprotein cholesterol. CONCLUSIONS: CysC had significant associations with both creatinine and SUA in Japanese junior high school students. The concomitant elevation of serum CysC and SUA levels was associated with subclinical lipid metabolism dysregulation, and suggested the presence of cardiometabolic risk accumulation.

Prognostic value of ankle brachial index for future incident heart failure in patients without previous heart failure: data from the impressive predictive value of ankle brachial index for clinical long term outcome in patients with cardiovascular disease examined by ABI study.

The ankle brachial index (ABI) is regarded as a predictor of future cardiovascular events. However, the relationship between ABI and incident heart failure (HF) in patients without previous HF is poorly understood. This study aimed to assess the prognostic value of ABI for incident HF in patients without previous HF. The IMPACT-ABI study was a retrospective, single-center, cohort study that enrolled and measured ABI in 3131 patients hospitalized for cardiovascular disease between January 2005 and December 2012. From this cohort, 307 patients were excluded because of previous HF and high (>1.4) ABI. The remaining 2824 patients were stratified into three groups: low ABI (≤0.9), borderline ABI (0.91-0.99), and normal ABI (1.0-1.4). The primary endpoint was hospitalization for HF. Over a mean 4.8-year follow-up, 105 cases of HF occurred. The cumulative incidence of HF was significantly higher in patients with low and borderline ABIs than in those with normal ABI (19.3 vs. 21.0 vs. 10.4 %, log rank P <0.001). In multivariate Cox proportional hazard analysis, low ABI and borderline ABI were independent predictors of incident HF [hazard ratio (HR) 3.00; 95 % confidence interval (CI) 1.70-5.28; P < 0.001 and HR 2.68; 95 % CI 1.35-5.34; P = 0.005, respectively]. In conclusion, low and borderline ABI were strong predictors for future incident HF in patients without previous HF.

Clinical Characteristics and Outcomes of Patients with High Ankle-Brachial Index from the IMPACT-ABI Study.

BACKGROUND: Reduced ankle-brachial index (ABI) is a predictor of cardiovascular events. However, the significance of high ABI remains poorly understood. This study aimed to assess the characteristics and outcomes of patients with high ABI. METHODS: The IMPACT-ABI study was a retrospective cohort study that enrolled and examined ABI in 3,131 patients hospitalized for cardiovascular disease between January 2005 and December 2012. From this cohort, 2,419 patients were identified and stratified into two groups: high ABI (> 1.4; 2.6%) and normal ABI (1.0-1.4; 97.3%). The primary endpoint was the cumulative incidence of major adverse cardiovascular events (MACE), including cardiovascular-associated death, myocardial infarction, and stroke. RESULTS: Compared with the normal ABI group, patients in the high ABI group showed significantly lower body mass index (BMI) and hemoglobin level, but had higher incidence of chronic kidney disease and hemodialysis. Multivariate logistic regression analysis revealed that hemodialysis was the strongest predictor of high ABI (odds ratio, 6.18; 95% confidence interval (CI), 3.05-12.52; P < 0.001). During the follow-up (median, 4.7 years), 172 cases of MACE occurred. Cumulative MACE incidence in patients with high ABI was significantly increased compared to that in those with normal ABI (32.5% vs. 14.5%; P = 0.005). In traditional cardiovascular risk factors-adjusted multivariate Cox proportional hazard analysis, high ABI was an independent predictor of MACE (hazard ratio, 2.07; 95% CI, 1.02-4.20; P = 0.044). CONCLUSION: Lower BMI, chronic kidney disease, and hemodialysis are more frequent in patients with high ABI. Hemodialysis is the strongest predictor of high ABI. High ABI is a parameter that independently predicts MACE.

Comparison of the standard and speckle tracking echocardiographic features of wild-type and mutated transthyretin cardiac amyloidoses.

AIMS: To compare cardiac function in patients with the two types of transthyretin (TTR)-related amyloidoses [wild-type (wt) and mutated (m) TTR amyloidoses (ATTR)] using standard and speckle tracking echocardiography (STE). METHODS AND RESULTS: Twenty-one consecutive patients with biopsy-proved ATTRwt were compared with 21 patients with ATTRm from the database, matched by age and left ventricular (LV) wall thickness (n = 135, ATTRm). All patients were examined using 2D echocardiography. Apical four- and two-chamber, and long-axis views and basal, mid, and apical short-axis views were used to examine LV longitudinal, circumferential, and radial strains. LV ejection fraction (EF), LV basal circumferential/radial strain, and mid-radial strain were significantly lower in patients with ATTRwt compared with patients with ATTRm. There was no significant difference between the two groups in the other parameters. In the receiver-operating characteristic curve analysis, LVEF and LV basal mean radial strain were the best parameters for distinguishing between the two groups. CONCLUSION: Patients with ATTRwt are characterized by lower LVEF, LV basal, and LV mid-radial strains compared with patients with ATTRm. LVEF and LV radial strain are useful in distinguishing between ATTRwt and ATTRm when TTR has been proved in biopsy specimens.

Regression of left ventricular hypertrabeculation is associated with improvement in systolic function and favorable prognosis in adult patients with non-ischemic cardiomyopathy.

BACKGROUND: We sometimes experience regression of left ventricular hypertrabeculation (LVHT), which is compatible with the diagnosis of LV non-compaction cardiomyopathy (LVNC) in adult patients. However, little is known about the association between LVHT regression and LV systolic function in adult patients. METHODS: We prospectively examined 23 consecutive adult patients who fulfilled the echocardiographic criteria for LVNC. LV reverse remodeling (RR) was defined as an absolute increase in LV ejection fraction of >10% at 6 months follow-up. LVHT area was calculated by subtraction from the outer edge to the inner edge of the LVHT at end-systole. RESULTS: The mean follow-up period was 61 months. LVRR was observed in 9 patients (39%). The changes in the mean LVHT area showed significant correlation with the changes in LV ejection fraction (r=-0.78, p<0.0001). Cardiac death occurred in 7 patients (50%) without LVRR, but no patients with LVRR died (log-rank, p=0.003). Furthermore, composite of cardiac death and hospitalization for heart failure occurred in 10 patients (71%) without LVRR, whereas there was one patient with LVRR (log-rank, p<0.001). CONCLUSIONS: Regression of LVHT is associated with improvement in LV systolic function. LVRR might be associated with a favorable prognosis in patients with LVHT.