Cardiac amyloidosis screening using a relative apical sparing pattern in patients with left ventricular hypertrophy.

BACKGROUND: Cardiac amyloidosis (CA) mimics left ventricular hypertrophy (LVH). It is treatable, but its prognosis is poor. A simple screening tool for CA would be valuable. CA is more precisely diagnosed with echocardiographic deformation parameters (e.g., relative apical sparing pattern [RASP]) than with conventional parameters. We aimed to 1) investigate incremental benefits of echocardiographic deformation parameters over established parameters for CA screening; 2) determine the resultant risk score for CA screening; and 3) externally validate the score in LVH patients. METHODS: We retrospectively studied 295 consecutive non-ischemic LVH patients who underwent detailed diagnostic tests. CA was diagnosed with biopsy or 99mTc-PYP scintigraphy. The base model comprised age (≥65 years [men], ≥70 years [women]), low voltage on the electrocardiogram, and posterior wall thickness ≥ 14 mm in reference to the literature. The incremental benefit of each binarized echocardiographic parameter over the base model was assessed using receiver operating characteristic curve analysis and comparisons of the area under the curve (AUC). RESULTS: Fifty-four (18%) patients had CA. RASP showed the most incremental benefit for CA screening over the base model. After conducting multiple logistic regression analysis for CA screening using four variables (RASP and base model components), a score was determined (range, 0-4 points). The score demonstrated adequate discrimination ability for CA (AUC = 0.86). This result was confirmed in another validation cohort (178 patients, AUC = 0.88). CONCLUSIONS: We developed a score incorporating RASP for CA screening. This score is potentially useful in the risk stratification and management of LVH patients.

Semiquantitative assessment of the relative apical sparing pattern of longitudinal strain for cardiac amyloidosis identification.

BACKGROUNDS: The relative apical sparing pattern (RASP) of left ventricular (LV) longitudinal strain (LS) is frequently associated with cardiac amyloidosis (CA). However, the visual assessment of RASP is inconsistent, and the quantitative assessment of RASP is time-consuming. This study aimed to compare assessments of RASP for the identification of CA with conventional assessments and investigate their reproducibility and relevance on the assessments. METHODS: Forty patients with biopsy-proven CA were compared with 80 hypertrophied patients matched for mean LV wall thickness. We compared the discriminative abilities of three assessments of RASP to identify CA (visual, quantitative, and semiquantitative). Nine patterns of semiquantitative RASP were investigated; finally, it was defined as "reduction of LS" (≥ -10%) in ≥5 (of 6) basal segments, relative to "preserved LS" (< -15%) in at least one apical segment. RESULTS: The concordance between the two observers for visual RASP was modest (κ = 0.65). On the other hand, the consistency for semiquantitative RASP was perfect (κ = 1.00). The discriminative ability of semiquantitative RASP (area under the curve [AUC]  = 0.74) was significantly better than that of visual RASP (AUC = 0.65) and equivalent to that of binary quantitative RASP. CONCLUSION: Semiquantitative RASP assessment is reproducible and accurately discriminates CA. This simple assessment may help readily refine the risk stratification of patients with diffuse LV hypertrophy.

Assessment of Second- and Third-Generation Drug-Eluting Stents on Chronic Coronary Angioscopy　- Multicenter Study on Intra-Coronary AngioScopy After Stent (MICASA) Prospective Data Analysis.

BACKGROUND: The vascular response, in terms of quality and quantity, of the second- and third-generation drug-eluting stents (2G- and 3G-DES, respectively) was assessed prospectively on coronary angioscopy (CAS).Methods and Results:The Multicenter study on Intra-Coronary AngioScopy After Stent (MICASA) is a multicenter CAS registry. A total of 107 DES (71 2G- and 36 3G-DES) were prospectively observed on CAS 8.7±2.7 months after percutaneous coronary intervention. Neointimal coverage (NC) grade was evaluated using a 4-point grading scale, from 0 (no coverage) to 3 (complete coverage). Plaque yellow color (YC) was also assessed using a 4-point grading system, from 0 (white) to 3 (bright yellow). Max-NC (2G-DES vs. 3G-DES: 2.14±0.68 vs. 2.44±0.73, P=0.023); min-NC (1.07±0.48 vs. 1.39±0.60, P=0.002), and dominant-NC (1.57±0.69 vs. 2.08±0.84, P=0.002) were significantly higher and the YC grade (1.23±0.82 vs. 0.86±0.76, P=0.031) significantly lower in the 3G-DES group than in the 2G-DES group. There was no significant difference in the presence of thrombus (28.2% vs. 22.2%, P=0.51) between the 2G- and 3G-DES groups. CONCLUSIONS: The higher NC grade and lower YC grade in 3G-DES than in 2G-DES might be associated with better long-term clinical outcome, which remains to be determined in future studies.

A Novel Truncating LMNA Mutation in Patients with Cardiac Conduction Disorders and Dilated Cardiomyopathy.

The cardiac phenotype of laminopathies is characterized by cardiac conduction disorders (CCDs) and dilated cardiomyopathy (DCM). Although laminopathies have been considered monogenic, they exhibit a remarkable degree of clinical variability. This case series aimed to detect the causal mutation and to investigate the causes of clinical variability in a Japanese family with inherited CCD and DCM.Of the five family members investigated, four had either CCD/DCM or CCD alone, while one subject had no cardiovascular disease and acted as a normal control. We performed targeted resequencing of 174 inherited cardiovascular disease-associated genes in this family and pathological mutations were confirmed using Sanger sequencing. The degree of clinical severity and variability were also evaluated using long-term medical records. We discovered a novel heterozygous truncating lamin A/C (LMNA) mutation (c.774delG) in all four subjects with CCD. Because this mutation was predicted to cause a frameshift mutation and premature termination (p.Gln258HisfsTer222) in LMNA, we believe that this LMNA mutation was the causal mutation in this family with CCD and laminopathies. In addition, gender-specific intra-familiar clinical variability was observed in this Japanese family where affected males exhibited an earlier onset of CCD and more severe DCM compared to affected females. Using targeted resequencing, we discovered a novel truncating LMNA mutation associated with CCD and DCM in this family characterized by gender differences in clinical severity in LMNA carriers. Our results suggest that in patients with laminopathy, clinical severity may be the result of multiple factors.

Comparison of Chronic Angioscopic Findings of Bare Metal Stents, 1st-Generation Drug-Eluting Stents and 2nd-Generation Drug-Eluting Stents　- Multicenter Study of Intra-Coronary Angioscopy After Stent (MICASA).

BACKGROUND: No previous study has reported a comprehensive comparison of the chronic angioscopic findings after bare metal stent (BMS), and 1st- and 2nd-generation drug-eluting stents (DES). METHODS AND RESULTS: The Multicenter Study on Intra-Coronary Angioscopy after Stent (MICASA) is a multicenter registry of coronary angioscopy. A total of 264 stents were observed by coronary angioscopy 1 year after PCI. There were 15 BMS, 90 1st-generation DES, and 159 2nd-generation DES. Neointimal coverage (NC) of the stent was classified into 4 grades from 0 (no coverage) to 3 (complete coverage). Yellow color (YC) of plaque at the stented segment was graded from 0 (white) to 3 (bright yellow). Minimum (Min-) and Maximum (Max-) NC grade were significantly lower with 1st- and 2nd-generation DES than with BMS. Although the Max-NC grade was similar, the Min-NC grade was significantly higher for 2nd-generation DES than for 1st-generation DES. Both the YC grade and the incidence of thrombus with 2nd-generation DES were lower than with the 1st-generation DES and were comparable to BMS. Multivariate analysis showed that low-density lipoprotein, 1st-generation DES, and acute coronary syndrome were independent factors for yellow plaque (YG2 or 3), and that hypertension and 1st-generation DES were independent factors for the incidence of thrombus. CONCLUSIONS: Coronary angioscopy revealed more homogeneous coverage with white neointima and less thrombus after 2nd-generation DES as compared with 1st-generation DES. These findings may explain the favorable clinical outcomes observed for patients treated with 2nd-generation DES. (Circ J 2016; 80: 1916-1921).

Association of computed tomography-derived muscle mass and quality with delayed acquisition independent walking after cardiovascular surgery.

BACKGROUND: In the context of cardiovascular surgery, the foremost concern lies in delayed functional recovery, as typified by the acquisition of independent walking after surgery, among older patients with decline in skeletal muscle mass and quality. Computed tomography (CT), which is typically employed for the preoperative assessment of pathological conditions in patients undergoing cardiovascular surgery, is also suitable for screening for potential decline in skeletal muscle mass and quality. The aim of this study was to examine the predictive capabilities of CT-derived parameters such as muscle mass and muscle quality for the delayed acquisition of independent walking in the postoperative period. METHODS: This retrospective study enrolled consecutive Japanese patients who underwent elective cardiovascular surgery between May 2020 and January 2023. In total, 139 patients were included in the analyses. Based on the preoperative CT image, the psoas muscle volume index (PMVI) and psoas muscle attenuation (PMA) were calculated. Information on patient characteristics, including preoperative physical fitness such as handgrip strength/body mass index (GS/BMI), short physical performance battery (SPPB), and 6-min walking distance (6MWD), were obtained from the medical records. We defined delayed acquisition of independent walking after surgery as the inability to walk 100 m within 4 days after surgery. RESULTS: The median age of the patients was 72 (interquartile: 64-78) years, and 74.8% (104/139) were men; 47.5% corresponded to the delayed group. The areas under the curves of SPPB, GS/BMI, 6MWD, PMVI, and PMA against delayed acquisition of independent walking after surgery were 0.68 [95% confidence interval (CI): 0.59 to 0.77], 0.72 (95% CI: 0.63 to 0.80), 0.73 (95% CI: 0.65 to 0.82), 0.69 (95% CI: 0.60 to 0.78), and 0.78 (95% CI: 0.70 to 0.85), respectively. In the multivariate logistic regression analysis, low PMA was significantly associated with delayed acquisition of independent walking even after adjustment for patient characteristics including physical fitness [model 1: SPPB (OR, 1.14; 95% CI: 1.03-1.25), model 2: GS/BMI (OR, 1.13; 95% CI: 1.03-1.25), and model 3: 6MWD (OR, 1.14; 95% CI: 1.03-1.25)], but PMVI was not. CONCLUSIONS: Our study revealed a strong association between PMA, a marker of CT-derived muscle quality, and the postoperative delay in achieving independent walking in patients who underwent cardiovascular surgery. The technique to obtain information on muscle quality during the time period before surgery may be an option for timely therapeutic intervention in patients who may have delayed acquisition of independent walking after surgery.

Pseudo-hyperacute T Waves in a Giant Hiatal Hernia.

An 89-year-old woman with a giant hiatal hernia complained of persistent chest pain. An electrocardiogram (ECG) showed hyperacute T waves, suggesting the early phase of ST-elevation myocardial infarction. After endoscopic drainage for hiatal hernia, the chest pain disappeared, and the ECG abnormalities resolved. The present case illustrates that compression of the heart by a giant hiatal hernia can induce T wave elevation mimicking acute coronary syndrome.

Pseudoxanthoma elasticum resulting in acute coronary syndrome.

Pseudoxanthoma elasticum (PXE) is a rare hereditary disorder that causes elastic tissue degeneration in the skin, eyes, and cardiovascular system. Gastrointestinal bleeding and fundus hemorrhage are serious complications associated with PXE prognosis as well as cardiovascular involvement. This is a rare case of acute coronary syndrome in a PXE patient with high bleeding risk. Learning objective: Pseudoxanthoma elasticum (PXE) resulting in acute coronary syndrome (ACS) is rare. Given PXE patients are generally at very high bleeding risk, antithrombotic therapy as secondary prevention after ACS onset should be taken into full consideration.

Automated interpretation of the coronary angioscopy with deep convolutional neural networks.

BACKGROUND: Coronary angioscopy (CAS) is a useful modality to assess atherosclerotic changes, but interpretation of the images requires expert knowledge. Deep convolutional neural networks (DCNN) can be used for diagnostic prediction and image synthesis. METHODS: 107 images from 47 patients, who underwent CAS in our hospital between 2014 and 2017, and 864 images, selected from 142 MEDLINE-indexed articles published between 2000 and 2019, were analysed. First, we developed a prediction model for the angioscopic findings. Next, we made a generative adversarial networks (GAN) model to simulate the CAS images. Finally, we tried to control the output images according to the angioscopic findings with conditional GAN architecture. RESULTS: For both yellow colour (YC) grade and neointimal coverage (NC) grade, we could observe strong correlations between the true grades and the predicted values (YC grade, average r=0.80±0.02, p<0.001; NC grade, average r=0.73±0.02, p<0.001). The binary classification model for the red thrombus yielded 0.71±0.03 F1-score and the area under the receiver operator characteristic curve was 0.91±0.02. The standard GAN model could generate realistic CAS images (average Inception score=3.57±0.06). GAN-based data augmentation improved the performance of the prediction models. In the conditional GAN model, there were significant correlations between given values and the expert's diagnosis in YC grade but not in NC grade. CONCLUSION: DCNN is useful in both predictive and generative modelling that can help develop the diagnostic support system for CAS.

Clinical Utility of Diffusion-weighted Whole-body Magnetic Resonance Imaging with Background Body Signal Suppression for Assessing and Monitoring IgG4-related Disease.

A 77-year-old man with symptoms of chest pain was diagnosed with immunoglobulin G4 (IgG4)-related disease. Fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) revealed an intense uptake in the submandibular gland, lymph nodes and abdominal aortic wall. Diffusion-weighted imaging with background body signal suppression (DWIBS) revealed signal enhancements at the same location as those of the FDG-PET/CT findings. The DWIBS signal intensity decreased after steroid treatment, so we decreased the steroid dosage. Relapse did not occur. DWIBS makes it possible to adjust the medicine dosage while confirming the therapeutic effects and will likely be a useful method for monitoring IgG4-related disease.

Myocardial ischemia in acute coronary syndrome: assessment using 64-MDCT.

OBJECTIVE: We investigated the performance of 64-MDCT myocardial imaging in assessing myocardial ischemia in acute coronary syndrome (ACS). MATERIALS AND METHODS: Cardiac CT was performed in 35 patients with ACS: 24 patients with acute myocardial infarction (AMI) and 11 patients with unstable angina pectoris (UAP). We reconstructed 2D myocardial images at diastolic and systolic phases using the same raw data as those used for coronary CT angiography. The CT number in the myocardium was used as an estimate of ischemia. The myocardium was shown using a color scale that depicts faint low-density areas more clearly than gray scale. We evaluated the variations in myocardial enhancement during the cardiac cycle in the territory of the culprit lesion. In addition, we classified patients on the basis of the transmurality of myocardial enhancement and examined whether this feature correlates with myocardial damage. RESULTS: Myocardial imaging at systole showed myocardial hypoenhancement in territories of the culprit lesion in 91% of patients with ACS, 96% of patients with AMI, and 75% of patients with UAP. The hypoenhancement areas at systole tended to be more extensive than those at diastole. The transmural extent of hypoenhancement at systole correlated with myocardial damage, which was shown by myocardial biomarkers. CONCLUSION: CT myocardial imaging can be used to assess myocardial ischemia in the appropriate region of ACS with high sensitivity.

Clinical utility of non-contrast T1-weighted magnetic resonance imaging in percutaneous coronary intervention: A case report.

Coronary high-intensity plaques (HIPs) visualized by non-contrast T1-weighted imaging (T1WI) in cardiac magnetic resonance (CMR) are associated with slow-flow phenomena during percutaneous coronary intervention (PCI). We report a case of a 52-year-old man who had undergone left anterior descending artery stent implantation for unstable angina 5 years previously. He underwent CMR imaging for screening of vulnerable plaques. A lesion in the proximal right coronary artery showed HIP on non-contrast T1WI. Invasive coronary angiography showed progressive stenosis and PCI was performed. Non-contrast T1WI indicated a high risk for a slow-flow phenomenon. A distal protection device (Parachute™ (Tri-Med, Osaka, Japan)) was deployed at the distal site of the lesion. Following balloon dilation, a filter no-reflow phenomenon developed. Coronary flow was improved with removal of the Parachute™ after debris aspiration. Histological examination revealed aspirated debris composed of white thrombi, foamy macrophages, and cholesterol crystals. .

Massive pulmonary embolism with cardiac arrest in pregnancy: A case report.

A 39-year-old woman developed a pulmonary embolism at 28 weeks of gestation, after a 4-week period of bedrest, and required emergencycesarean section due to a decrease in fetal heart rate. Pulseless electrical activity (PEA) developed after intravenous anesthesia. The fetus was delivered 5 min after PEA onset, during cardiopulmonary resuscitation of the mother. Intravenous recombinant tissue-plasminogen activator injection, percutaneous cardiopulmonary support, and 24-h hypothermia therapy were administered to the mother, followed by inferior vena cava filter insertion, combined with catheter thrombus fragmentation and percutaneous thrombectomy. Both the patient and her baby survived. .

Quantification of myocardial perfusion by contrast-enhanced 64-MDCT: characterization of ischemic myocardium.

OBJECTIVE: Assessment of hemodynamic changes in ischemic cardiac segments at rest using CT has yet to be performed. We hypothesized that variations in subendocardial perfusion during the cardiac cycle might be related to the appearances of ischemia. The purpose of this study was to investigate myocardial perfusion in ischemic segments using contrast-enhanced 64-MDCT. SUBJECTS AND METHODS: We performed cardiac MDCT at rest and stress/rest (201)Tl myocardial perfusion scintigraphy (MPS) in 34 patients with suspected coronary artery disease. We reconstructed 2D long- and short-axis cardiac images in diastolic and systolic phases using raw data from coronary CT angiography. The attenuation value (in Hounsfield units) in the myocardium was used as an estimate of myocardial perfusion. We measured the subendocardial intensity of 17 segments according to the American Heart Association classification. Systolic perfusion or diastolic perfusion was calculated by dividing the subendocardial intensity at systole or diastole, respectively, for each segment by the mean value across all segments for each patient. We used stress/rest MPS to evaluate the variation in myocardial perfusion at systole and diastole for the segments diagnosed as ischemic or nonischemic. RESULTS: Systolic perfusion for ischemic segments was significantly lower than that for nonischemic segments in 15 of 17 segments. The difference between systolic perfusion and diastolic perfusion in ischemic segments was significantly lower than that in nonischemic segments (14 of 17 segments). There was no significant difference in diastolic perfusion between ischemic and nonischemic segments (15 of 17 segments). CONCLUSION: Our results suggest that a pattern of subendocardial hypoperfusion at systole and normal perfusion at diastole characterizes ischemic myocardium.

Analysis of microvascularity after reperfused acute myocardial infarction using the maximum slope method of contrast-enhanced magnetic resonance imaging.

PURPOSE: The aim of this study was to analyze microvas-cularity after reperfused acute myocardial infarction (AMI) using the maximum slope method of contrastenhanced cardiac magnetic resonance imaging (CMR). MATERIALS AND METHODS: CMR and resting (201)T1 single photon emission computed tomography (SPECT) images were obtained in 30 consecutive patients after reperfused AMI and 10 controls. After bolus injection of gadolinium diethylenetriamine pentaacetic acid, first-pass CMR images were obtained using the True-FISP sequence. Time-intensity curves were generated by measuring the signal intensity in the myocardium and left ventricle. The arterial input function was obtained from the left ventricular time-intensity curve. On the basis of the maximum slope method, the microvascular index (MVI) was calculated by dividing the maximum initial upslope of the myocardium by the initial upslope of the left ventricle. RESULTS: The MVI was significantly lower in the segments related to the occluded coronary artery. MVIs in segments with (201)Tl uptake of 50%-59% of peak were significantly lower than in those with (201)Tl uptake of 60%-69%. MVIs in segments with (201)Tl uptake of <50% of peak were significantly lower than in those with (201)Tl uptake of 50%-59%. CONCLUSION: This study presents a method that directly assesses microvascularity after reperfused AMI.

Evaluation of acute myocardial infarction with late enhancement pattern on MRI compared with 201Tl and 99mTc-hydroxymethylenediphosphonate (HMDP) dual single photon emission computed tomography (SPECT) images.

UNLABELLED: The purpose of this study is to evaluate reperfused acute myocardial infarction with late enhancement (LE) pattern on contrast-enhanced magnetic resonance imaging compared with myocardial single photon emission computed tomography (SPECT) images. MATERIALS AND METHODS: Magnetic resonance and 201Tl and 99mTc-hydroxymethylenediphosphonate SPECT images were obtained from 40 patients within 6 days of reperfused myocardial infarction. We assessed the myocardial LE pattern using the true fast imaging with steady-state free precession sequence after the injection of Gd-DTPA. Patients were classified into 3 groups: group 1 included patients with localized endocardial enhancement; group 2, patients with transmural enhancement; and group 3, patients with LE and a residual defect. RESULTS: There were 9 patients (23%) in group 1, 15 (38%) in group 2, and 15 (38%) in group 3. In 1 patient, LE was not detected (3%). The %201Tl uptake for the infarcted area was 60.7 +/- 7.2 (mean +/- SD) for group 1, 49.5 +/- 12.3 (P < 0.05 vs. group 1) for group 2, and 36.9 +/- 8.2 (P < 0.0001 vs. group 1, P = 0.005 vs. group 2) for group 3. An overlap pattern of 201Tl and 99mTc was observed in 9 of the group 1 patients (100%) and 9 of the group 2 patients (60%), but was not evident in group 3 (0%). CONCLUSIONS: LE with residual defect is an important indicator of microvascular obstruction after reperfusion therapy.

A case of in-stent restenosis with pathologically proven chronic inflammation seven years after sirolimus-eluting stent implantation.

A 67-year-old man was admitted to our hospital due to chest pain at rest. Seven years previously, the patient underwent percutaneous coronary intervention (PCI) of the left ascending artery and implanted sirolimus-eluting stent (SES). Coronary angioscopy (CAS) performed at that time showed a white plaque at the SES site. Two years after the first PCI, repeat CAS demonstrated light yellow plaques at the SES site. At the time of his presentation to our hospital, coronary angiography showed in-stent restenosis at the SES site, and CAS demonstrated the plaque rupture with presence of dense yellow plaque and various thrombi. After distal protection, drug-eluting balloon treatment was performed. Collected specimens from culprit sites included foamy macrophages, cholesterin crystals, neutrophils, and fibrin, suggesting that progressive neoatherosclerosis at the SES site triggered the acute coronary syndrome. This study highlights the importance of ensuring careful patient follow-up after SES implantation. .