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.

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.

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.

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. .

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. .

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.

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.

Intracoronary Optical Coherence Tomography-Derived Virtual Fractional Flow Reserve for the Assessment of Coronary Artery Disease.

Fractional flow reserve (FFR) is widely used for the assessment of myocardial ischemia. Optical coherence tomography (OCT) provides accurate visualization of coronary artery morphology. The aim of this study was to investigate the relation between FFR and OCT-derived FFR. We retrospectively analyzed 31 lesions (25 left anterior descending arteries, 2 left circumflex arteries, and 4 right coronary arteries) in 31 patients with moderate-to-severe coronary stenosis, who underwent OCT and FFR measurements simultaneously. OCT-derived FFR was calculated by the original algorithm, which was calculated using the following equation based on fluid dynamics: ΔP = FV + SV2, where V is the flow velocity, F is the coefficient of pressure loss because of viscous friction (Poiseuille resistance), and S is the coefficient of local pressure loss because of abrupt enhancement (flow separation). Mean values of % diameter stenosis by quantitative coronary angiography and FFR were 55.2 ± 14.0% and 0.70 ± 0.14, respectively. OCT-derived FFR showed a stronger linear correlation with FFR measurements (r = 0.89, p <0.001; root mean square error = 0.062 FFR units) than quantitative coronary angiography % diameter stenosis (r = -0.65, p <0.001), OCT measurements of minimum lumen area (r = 0.68, p <0.001), and % area stenosis (r = -0.70, p <0.001). OCT-derived FFR has the potential to become an alternative method for the assessment of functional myocardial ischemia, and may elucidate the relation between coronary morphology and FFR.

Characterization of high-intensity plaques on noncontrast T1-weighted magnetic resonance imaging by coronary angioscopy.

BACKGROUND: A recent study showed that coronary high-intensity plaques (HIPs) visualized by noncontrast T1-weighted imaging (T1WI) in cardiac magnetic resonance were associated with coronary events. We used coronary angioscopy to analyze HIP plaque morphology. METHODS AND RESULTS: A total 17 lesions from 17 patients with stable or unstable angina pectoris were evaluated at the culprit lesion by noncontrast T1WI using 1.5-T magnetic resonance; of them, nine (53%) were HIPs and eight (47%) were non-HIPs, and all were analyzed by coronary angioscopy. We assessed the existence of thrombus and plaque yellow color grade (YG). YG was assessed visually according to a four-grade scale: 0, white; 1, light yellow; 2, yellow; 3, intense yellow. The frequency of thrombus was significantly higher in HIPs than in non-HIPs (89% vs. 25%, respectively; p=0.007). YG was significantly more frequent in HIPs than in non-HIPs (2.2±0.4 vs. 0.7±0.7, respectively; p=0.01). CONCLUSIONS: These data indicated that HIPs on noncontrast T1WI were associated with the presence of high-grade yellow plaque with thrombus.

Relationship between yellow plaque grade and tissue protrusion after stent implantation: A coronary angioscopy study.

BACKGROUND: Tissue protrusion detected with optical coherence tomography after percutaneous coronary intervention using stents is one of the risk factors for early stent thrombosis. However, tissue protrusion features have not been described. The aim of this study was to compare tissue morphology at stented sites with or without tissue protrusion by using coronary angioscopy. METHODS: Using optical coherence tomography and coronary angioscopy, we assessed 42 patients [31 men, 11 women; age, 70.7±7.4years; acute coronary syndrome (ACS), n=19; effort angina pectoris (EAP), n=23] after stenting. RESULTS: Twenty patients had tissue protrusion. ACS patients had a higher incidence of tissue protrusion than EAP patients (70.0% vs 29.4%; p=0.002). The plaque at the protrusion site had higher-grade yellow plaque with thrombus than those without protrusion (2.35±0.67 vs 1.40±0.67; p<0.001). The plaque at protrusion sites developed more thrombi (60.0% vs 22.7%; p=0.041). CONCLUSIONS: Tissue protrusion after stent implantation was associated with high-grade yellow plaque with thrombi.

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. .