In-Hospital Mortality in Patients With Cardiogenic Shock Requiring Veno-Arterial Extracorporeal Membrane Oxygenation With Concomitant Use of Impella vs. Intra-Aortic Balloon Pump　- A Retrospective Cohort Study Using a Japanese Claims-Based Database.

BACKGROUND: Patients with refractory cardiogenic shock (CS) necessitating peripheral veno-arterial extracorporeal membrane oxygenation (VA-ECMO) often require an intra-aortic balloon pump (IABP) or Impella for unloading; however, comparative effectiveness data are currently lacking.Methods and Results: Using Diagnosis Procedure Combination data from approximately 1,200 Japanese acute care hospitals (April 2018-March 2022), we identified 940 patients aged ≥18 years with CS necessitating peripheral VA-ECMO along with IABP (ECMO-IABP; n=801) or Impella (ECPella; n=139) within 48 h of admission. Propensity score matching (126 pairs) indicated comparable in-hospital mortality between the ECPella and ECMO-IABP groups (50.8% vs. 50.0%, respectively; P=1.000). However, the ECPella cohort was on mechanical ventilator support for longer (median [interquartile range] 11.5 [5.0-20.8] vs. 9.0 [4.0-16.8] days; P=0.008) and had a longer hospital stay (median [interquartile range] 32.5 [12.0-59.0] vs. 23.0 [6.3-43.0] days; P=0.017) than the ECMO-IABP cohort. In addition, medical costs were higher for the ECPella than ECMO-IABP group (median [interquartile range] 9.09 [7.20-12.20] vs. 5.23 [3.41-7.00] million Japanese yen; P<0.001). CONCLUSIONS: Our nationwide study could not demonstrate compelling evidence to support the superior efficacy of Impella over IABP in reducing in-hospital mortality among patients with CS necessitating VA-ECMO. Further investigations are imperative to determine the clinical situations in which the potential effect of Impella can be maximized.

Right ventricular myocardial fibrosis evaluated by extracellular volume fraction by magnetic resonance imaging in patients with repaired tetralogy of Fallot: a meta-analysis.

OBJECTIVE: The objective of this meta-analysis was to assess the clinical utility of anomalous discoveries on cardiac magnetic resonance, particularly the right ventricular extracellular volume (RV-ECV), among individuals who underwent surgical repair for Tetralogy of Fallot (rTOF). METHODS: We conducted a systematic search of electronic databases including PubMed, Web of Science Core Collection, Cochrane advanced search, and EMBASE. Our analysis involved a comparison of ECV levels between rTOF patients and controls, as well as an evaluation of the predictive value of ECV for future adverse events. RESULTS: We identified 16 eligible studies that encompassed 856 rTOF patients and 283 controls. Our meta-analysis showed a significant increase in LV-ECV among rTOF patients compared to control subjects (MD = 2.63, 95%CI: 1.35 to 3.90, p < 0.0001, I2 = 86%, p for heterogeneity < 0.00001). Moreover, RV-ECV was found to be substantially higher in patients compared to LV-ECV. Our meta-analysis also revealed a significant association between RV-ECV and adverse events (HR = 1.15, 95% CI: 1.04 to 1.27, p = 0.005, I2 = 0%, p for heterogeneity = 0.62), while LV-ECV did not show any significant association with adverse events (HR = 1.12, 95% CI: 0.92 to 1.36, p = 0.16, I2 = 0%, p for heterogeneity = 0.46). CONCLUSION: The results of this meta-analysis on RV-ECV confirmed the presence of RV fibrosis as one of the prognostic factors in rTOF patients.

In-Hospital Death and End-of-Life Status Among Patients With Adult Congenital Heart Disease　- A Retrospective Study Using the JROAD-DPC Database in Japan.

BACKGROUND: The end-of-life (EOL) status, including age at death and treatment details, of patients with adult congenital heart disease (ACHD) remains unclear. This study investigated the EOL status of patients with ACHD using a nationwide Japanese database.Methods and Results: Data on the last hospitalization of 26,438 patients with ACHD aged ≥15 years, admitted between 2013 and 2017, were included. Disease complexity (simple, moderate, or great) was classified using International Classification of Diseases, 10th Revision codes. Of the 853 deaths, 831 patients with classifiable disease complexity were evaluated for EOL status. The median age at death of patients in the simple, moderate, and great disease complexity groups was 77.0, 66.5, and 39.0 years , respectively. The treatments administered before death to patients in the simple, moderate, and great complexity groups included cardiopulmonary resuscitation (30.1%, 35.7%, and 41.9%, respectively), percutaneous cardiopulmonary support (7.2%, 16.5%, and 16.3%, respectively), and mechanical ventilation (58.7%, 72.2%, and 75.6%, respectively). Overall, 70% of patients died outside of specialized facilities, with >25% dying after ≥31 days of hospitalization. CONCLUSIONS: Nationwide data showed that patients with ACHD with greater disease complexity died at a younger age and underwent more invasive treatments before death, with many dying after ≥1 month of hospitalization. Discussing EOL options with patients at the appropriate time is important, particularly for patients with greater disease complexity.

A Simple Risk Score to Differentiate Between Coronary Artery Obstruction and Coronary Artery Spasm of Patients With Acute Coronary Syndrome Without Persistent ST-Segment Elevation.

BACKGROUND: The aim of this study was to create a risk scoring model to differentiate obstructive coronary artery (CA) from CA spasm in the etioology of acute coronary syndrome (ACS).Methods and Results: We included 753 consecutive patients with ACS without persistent ST-segment elevation (p-STE). The exclusion criteria were: (1) out-of-hospital cardiac arrest; (2) cardiogenic shock; (3) hemodialysis; (4) atrial fibrillation/flutter; (5) severe valvular disease; (6) no coronary angiography; (7) non-obstructive coronary artery without "definite" vasospastic angina definition; and/or (8) missing data. From the multivariate logistic regression analysis for prediction of obstructive CA, an integer score of 2 to each 0.5 increment in odds ratio was given, and values were divided into quartiles according to the total score. The scores were as follows: age >70 years (6 points), non-STE myocardial infarction (9 points), diabetes mellitus (5 points), B-type natriuretic peptide >90 pg/mL (7 points), neutrophil to lymphocyte ratio >2 (5 points), and high-density lipoprotein cholesterol <50 mg/dL (5 points). CA spasm-induced ACS occurred in 50.0% in Quartile 1 (total score: 0-13), 20.5% in Quartile 2 (total score: 14-19), 4.9% in Quartile 3 (total score: 20-26), and 2.2% in Quartile 4 (total score: 27-37) (P<0.001), indicating that a total score of <20 was a potential clinical indicator of CA spasm-induced ACS. CONCLUSIONS: CA spasm-induced ACS should be suspected if a total score of <20, and a spasm provocation test was being considered.

Impaired Left Atrial Function in Patients with Atrial Septal Defect and History of Atrial Fibrillation.

In patients with atrial septal defect (ASD), atrial left-to-right shunting causes left atrial (LA) remodeling and dysfunction, leading to atrial fibrillation (AF). In adults with ASD and concomitant AF, LA function should be evaluated after ASD closure plus AF radiofrequency catheter ablation (RFCA).This multicenter retrospective cohort study included patients who underwent transcatheter ASD closure at one of the four leading hospitals. Patients with a history of AF also underwent preceding RFCA. The association between AF history and LA ejection fraction (EF) (indicating LA global function) at 6-12 months following ASD closure was evaluated. To account for differences in baseline characteristics between patients with and without a history of AF, we conducted the following statistical methods: (1) multivariate regression analysis in the prepropensity score (PS)-matched cohort and (2) univariate comparisons in the PS-matched cohort.Overall, this study included 231 patients (30 with AF history, 201 without). Multiple regression analysis showed that AF history was independently associated with impaired LAEF (ß = -10.425, P < 0.001, model created prior to propensity matching). A one-to-one PS matching (25 pairs) showed that the LAEF at 6-12 months following ASD closure was significantly impaired in patients with ASD and AF history compared to that in patients without history of AF (median LAEF, 37.5% (interquartile range [IQR] 29.4%-48.5%) versus 52.3 [IQR 50.0%-56.6%]; P < 0.001).LA function was impaired in patients with ASD and a history of AF at 6-12 months after successful transcatheter ASD closure and on maintenance of sinus rhythm by RFCA.

Outcomes of women with congenital heart disease admitted to acute-care hospitals for delivery in Japan: a retrospective cohort study using nationwide Japanese diagnosis procedure combination database.

BACKGROUND: The number of women with congenital heart disease (CHD) who are of childbearing age is increasing due to advancements in medical management. Nonetheless, data on the outcomes of delivery in women with CHD remain limited. Therefore, we conducted a retrospective cohort study using a nationwide database of deliveries by women with CHD. METHODS: Deliveries by women with CHD discharged from acute-care hospitals between April 2017 and March 2018 were identified based on the Diagnosis Procedure Combination database which covers almost all acute-care hospitals in Japan. By using this database, we tried to include relatively high-risk deliveries by women with CHD. Subjects were divided into three groups according to the underlying disease complexity: simple, moderate, and great complexity. The clinical characteristics and incidence of peripartum cardiovascular events were compared among the three groups. RESULTS: A total of 249 deliveries from 107 hospitals were included. The largest facility had 29 deliveries per year. Given the uncertainty of underlying cardiac anomalies, 48 women were excluded, and the remaining 201 women (median age, 32 years) were analyzed. In-hospital maternal death, use of extracorporeal membrane oxygenation, intra-aortic balloon pump, pacemaker, and direct current cardioversion were not observed. Nine patients (4.5%) required intravenous diuretic administration. However, the difference in the frequency of diuretic use was not significant among the three groups (simple, 1.9%; moderate, 7.2%; great, 6.9%; P = 0.204). One participant required valve replacement surgery at 22 days after a successful cesarean section. As the disease complexity increased, deliveries occurred more frequently at university hospitals (simple, 41.7%; moderate, 52.2%; great, 72.4%; P = 0.013) and the length of hospitalization was significantly longer, with median durations of 9.0 (interquartile range [IQR] 7.0-11.0) days, 10.0 (IQR 8.0-24.0) days, and 11.0 (IQR 8.0-36.0) days in the simple, moderate, and great complexity groups, respectively (P = 0.002). CONCLUSIONS: Appropriate patient selection and management by specialized tertiary institutions may contribute to positive outcomes in pregnancies in women with CHD.

Real-time three-dimensional trans-oesophageal echocardiography-guided balloon dilation of pulmonary venous pathway obstruction in a patient with dextro-transposition of the great arteries after atrial switch surgery: a case report.

A percutaneous transcatheter balloon dilation of a pulmonary venous pathway obstruction was successfully performed in a 40-year-old patient after a Mustard procedure. During the procedure, real-time three-dimensional trans-oesophageal echocardiography demonstrated the morphology of the obstruction. Our case highlights the usefulness of real-time three-dimensional trans-oesophageal echocardiography as a guide for transcatheter intervention in the increasing number of adults with CHD.

The AB Portable Driver Generates Higher Drive-Line Pressures Possibly Leading to Accelerated Hemolysis.

The AB5000 Circulatory Support System is paracorporeal pulsatile ventricular assist device. The AB Portable Driver is a portable console for this system. We experienced two cases with accelerated hemolysis while receiving support by the AB Portable Driver. The purpose of this study was to clarify the mechanical differences associated with the hemolysis between the AB5000 console and the AB Portable Driver. The mock circulatory system modeled by an AB5000 ventricle and a blood sampling bag of vinyl chloride was run with an AB5000 console or AB Portable Driver. The peak drive-line pressure, the mean arterial cannula pressure and the pumping rate of the VAD were recorded. The AB5000 console generated a peak drive-line pressure of 280-300 mm Hg in LVAD mode and 210-220 mm Hg in RVAD mode, approximately 100 mm Hg lower than officially documented. In contrast, the AB Portable Driver generated pressures of 310-330 mm Hg in LVAD mode and 230-250 mm Hg in RVAD mode, 65-95 mm Hg higher than officially documented. The AB Portable Driver console generates higher drive-line pressures than the AB5000 console, possibly explaining the accelerated hemolysis.

Analysis of in-hospital deaths in patients with critical limb ischemia necessitating invasive treatments: based on a Japanese nationwide database.

Critical limb ischemia (CLI) is associated with systemic cardiovascular and non-cardiovascular diseases. Treatments primarily targeting limb-related outcomes may not improve overall life prognosis. We aimed to describe in-hospital mortality and the underlying etiologies in Japanese patients with CLI. We analyzed the Diagnosis Procedure Combination (DPC) database from approximately 1200 Japanese acute-care hospitals between April 2018 and March 2020. The definition of patients with CLI was based on the diagnostic codes listed as the most resource-intensive diagnosis and information regarding invasive procedures (endovascular treatment, bypass, or amputation). The DPC database provides information on whether in-hospital death was caused by the most resource-intensive diagnosis. Among 15,228 distinct patients with CLI, we identified 18,970 records, including 5,378 amputations. In-hospital death occurred in 1238 (6.5%) patients. Among them, 811 (65.5%) were due to causes unrelated to CLI. In patients who underwent amputation (n = 5378), causes unrelated to CLI accounted for 70.0% of in-hospital deaths, whereas among patients who did not undergo amputation (n = 13,592), this proportion was 60.1%. When compared to patients who died due to causes related to CLI, the prevalence of male patients was higher (62.6% vs 52.7%, p = 0.001), and amputation was more frequently performed (58.0% vs 47.1%, p < 0.001) in those who died due to causes unrelated to CLI. The majority of in-hospital deaths among patients with CLI necessitating endovascular treatment, bypass, or amputation were attributable to factors unrelated to the primary condition of CLI. Managing systemic cardiovascular and non-cardiovascular diseases beyond the affected limb is crucial to improve the prognosis of these patients.

Network Meta-Analysis of C5 Palsy After Anterior Cervical Decompression of Three to Six Levels: Comparing Three Different Procedures.

STUDY DESIGN: Systematic review and meta-analysis. OBJECTIVE: Using a network meta-analysis (NMA), this study aimed to compare the risks of C5 palsy after three different procedures of anterior cervical decompression. SUMMARY OF BACKGROUND DATA: C5 palsy is a well-known complication affecting the quality of life after anterior procedures. Due to the limited evidence on the various procedures available, we evaluate the basis for selection to prevent palsy and achieve maximal decompression in cases spanning 3-6 levels. MATERIALS AND METHODS: We conducted a comprehensive search for C5 palsy and complications after 3representative procedures, including anterior cervical discectomy and fusion (ACDF), anterior cervical corpectomy and fusion (ACCF), and their combination (hybrid), involving 3 to 6 intervertebral levels. The incidence of C5 palsy was compared using a NMA. RESULTS: We identified 1655 patients in 11 studies who met inclusion criteria. Sixty-nine patients (4.2%) developed delayed C5 palsies. The incidences among ACDF, ACCF, and hybrid cases were 2.3% (16/684, 95% CI: 1.4% to 3.8%), 6.4% (39/613, 95% CI: 4.7% to 8.6%), and 3.9% (14/358, 95% CI: 2.3% to 6.5%), respectively ( P < 0.01). A NMA was performed for 15 pairwise comparisons across the 3 procedure arms: ACDF versus hybrid, 7/232 (3.0%) versus 11/234 (4.7%); hybrid versus ACCF, 14/301 (4.3%) versus 18/224 (8.0%); ACCF versus ACDF, 38/523 (7.8%) versus 16/619 (2.6%). Compared with ACDF, the risk of C5 palsy was significantly higher in ACCF (odds ratio: 2.72, 95% CI: 1.47 to 5.01), whereas ACDF versus hybrid did not significantly differ in risk (odds ratio: 1.56, 95% CI: 0.68 to 3.60). CONCLUSION: We determined that ACCF was associated with a higher risk of postoperative C5 palsy than ACDF in cases spanning 3 to 6 intervertebral levels. If practicable, ACDF surgery may be an appropriate choice for cases requiring anterior decompression of 3 to 6 levels. LEVEL OF EVIDENCE: Level III.

Precautions When Performing Pericardiocentesis in Patients with Cardiac Tamponade-complicated Malignancy: A Case Report and Review of the Literature.

A 51-year-old woman who had previously undergone left mastectomy for left breast cancer accompanied by multiple metastasis experienced worsening dyspnea. Physical and imaging assessments of the hemodynamics suggested cardiac tamponade, and emergency pericardiocentesis was successfully performed. However, immediately after the procedure, the patient's condition deteriorated rapidly and showed pulseless electrical activity. Contrast-enhanced computed tomography with continuous mechanical support demonstrated massive thrombi in both pulmonary arteries. An abrupt decrease in the central venous pressure and an increase in the venous return following pericardiocentesis might result in the migration of a deep venous thrombus and fatal acute pulmonary thromboembolism.

Association between sarcopenia and exercise capacity in patients with pulmonary hypertension without left heart disease.

BACKGROUND: Pulmonary hypertension (PH) has recently been described as a complex clinical syndrome affecting multiple organ systems, including the heart, lungs, and skeletal muscle, each of which plays an important role in exercise capacity. However, the relationship between exercise capacity and skeletal muscle abnormalities in patients with PH has not been fully elucidated. METHODS: We retrospectively analysed the exercise capacity and measures of skeletal muscle of 107 patients with PH without left heart disease (mean age 63 ± 15 years, 32.7% males, n = 30/6/66/5 in the clinical classification Group 1/3/4/5). RESULTS: Sarcopenia, low appendicular skeletal muscle mass index, low grip strength, and slow gait speed, determined by international criteria, were found in 15 (14.0%), 16 (15.0%), 62 (57.9%), and 41 (38.3%) patients, respectively. The mean 6-min walk distance of all patients was 436 ± 134 m and was independently associated with sarcopenia (standardised ß = -0.292, p < 0.001). All patients with sarcopenia showed reduced exercise capacity defined as 6-min walk distance < 440 m. Multivariable logistic regression analysis showed that each of the components of sarcopenia was associated with reduced exercise capacity (adjusted odds ratio and 95% confidence interval of appendicular skeletal muscle mass index: 0.39 [0.24-0.63] per 1 kg/m2, p = 0.006, grip strength: 0.83 [0.74-0.94] per 1 kg, p = 0.003, and gait speed: 0.31 [0.18-0.51] per 0.1 m/s, p < 0.001). CONCLUSIONS: Sarcopenia and its components are associated with reduced exercise capacity in patients with PH. A multifaceted evaluation may be important in the management of reduced exercise capacity in patients with PH.

A case of subacute thrombosis associated with clopidogrel resistance after implantation of a zotarolimus-eluting stent.

A 73-year-old woman was admitted to our hospital with anterior acute myocardial infarction due to subacute thrombosis after coronary stenting with a zotarolimus-eluting stent (ZES), which is a newly developed drug-eluting stent that has been widely used since May 2009 in Japan. Five days before, she underwent implantation with a ZES in the left anterior descending artery due to stable angina pectoris. After stenting, the intravascular ultrasonography showed no malapposition from the proximal to the distal edge of the stent. She received aspirin 100 mg/day and clopidogrel 75 mg/day from 2 weeks before the stent was implanted. When we investigated the single nucleotide polymorphisms of CYP2C19 in this patient, both CYP2C19*2 and CYP2C19*3 were detected, and she was classified as a poor metabolizer. This report is the first to describe subacute stent thrombosis following the implantation of a newly developed ZES in a Japanese patient, which may be related to clopidogrel resistance.

Ingenuity of precordial leads in diagnosing a patient with dextroversion of the heart complicated by Takotsubo syndrome: A case report.

A 71-year-old male with history of a right lung lobectomy for cancer of the right lung complained of resting chest pain. Through the typical echocardiographic findings, takotsubo syndrome was suspected; however, because of dextroversion of the heart, the standard 12­lead electrocardiogram did not show the typical findings of takotsubo syndrome. Based on the finding of the chest-X-ray, in order to adjust for his dextroversion of the heart, the electrodes were then placed on the right side of his chest as modified right-sided precordial leads, in which leads V1-2 were equivalent to basal portion and V5-6 to the apex of the dextroversion of his heart. Negative T waves in the apical leads (V5-6) as a typical finding of takotsubo syndrome were clearly seen. Based on coronary angiogram and left ventriculogram, takotsubo syndrome was definitively diagnosed. Learning objectives: The proper modification of the precordial leads with consideration of the heart position can provide a valuable finding and may be very useful in diagnosing patients with cardiac malposition complicated by cardiac diseases in which identification of impaired site is important.

Objective and Personalized Assessment of Disease-Related Knowledge Among Patients With Congenital Heart Disease　- Development and Validation of the Japanese Version of the Leuven Knowledge Questionnaire for Congenital Heart Disease.

Background: Disease understanding in patients with congenital heart disease is important in transitional and lifelong care. This study aimed to develop the Japanese version of the Leuven Knowledge Questionnaire for Congenital Heart Disease (LKQCHD) and identify factors associated with disease-related knowledge. Methods and Results: After confirming the content and face validity of the scale, a questionnaire including the LKQCHD was distributed to 59 eligible patients aged >16 years attending a university hospital. For the 58 participants who responded (30 males, 28 females; median age 22 years), the mean (±SD) LKQCHD total score was 53.7±15.4, with mean (±SD) scores for each domain as follows: Disease and Treatment, 68.3±19.7; Preventing Complications, 45.8±19.0; Physical Activity, 74.1±34.1; Sex and Heredity, 37.9±35.4; and Contraception and Pregnancy, 40.2±29.1. Regarding known-groups validity, we found a positive correlation between the LKQCHD score and age (ρ=0.268, P=0.042), and a significantly low LKQCHD score in the moderate/severe disease group (η2=0.131, P=0.021). Regarding convergent validity, the LKQCHD score was positively correlated with the total and subscale scores of the Resilience Assessment Tool (r=0.213 [P=0.109] and r=0.405 [P=0.002], respectively). Conclusions: We confirmed the validity of the Japanese version of the LKQCHD, concluding that patient education regarding long-term complications, prevention methods, heredity, pregnancy, and childbirth is needed.

Characteristics of patients with adult congenital heart disease treated by non-specialized doctors: The potential loss of follow-up.

BACKGROUND: In the treatment of adult congenital heart disease (ACHD), the transfer of patients from pediatric cardiologists to ACHD cardiologists is of relevance. However, little is known about the clinical courses of ACHD patients that have been referred by non-CHD-specialized doctors (n-CSDs). METHODS: This retrospective cohort study included 230 patients (average age: 37 ± 15.2 years, male: 97) who were referred to a single specialized ACHD center between April 2016 and July 2019. We compared the characteristics and clinical courses between patients referred by n-CSDs and those referred by CHD-specialized-doctors (CSDs). RESULTS: Overall, 121 (53%) patients were referred by n-CSDs. Among them, 91 (75%) patients were referred by adult cardiologists. Univariate analysis showed that the patients referred by n-CSDs were older than those referred by CSDs (41.6 ± 16.3 vs. 32.0 ± 12.0 years, p < 0.01), were more likely to have simple CHD, and less likely to have severe CHD (27.0% vs. 12.8% and 16.5% vs. 40.4%, respectively, p < 0.01). Patients referred by n-CSDs were also more likely to have a history of loss of follow-up (16.5% vs. 3.7%, p < 0.01) and to require invasive treatments after referral, including cardiac surgeries and transcatheter interventions (47.9% vs. 26.6 %, p < 0.01). Notably, unintended invasive treatments that were not designated by the referring doctors were more frequently required in patients with moderate complexity referred by n-CSDs (50.0% vs. 23.3%, p = 0.02). CONCLUSIONS: Patients with moderate CHD complexity referred by n-CSDs are more likely to require unintended invasive treatments. Referrals to specialized ACHD centers may be most beneficial for these patients.

A mechanism of a cardiac murmur with respiratory variation in a patient with straight back syndrome.

A 20-year-old male without any symptoms was referred for heart murmur on a medical examination. A thrill was palpable at the upper left sternal border. His cardiac murmur showed respiratory variation. The systolic murmur was louder (Levine grade IV/VI) during expiration and diminished during inspiration (Levine grade I/VI). He was thin and had a narrow thoracic cage in the anteroposterior direction due to straight back syndrome (SBS). An echocardiogram and a right ventriculogram showed changes in the diameter of the right ventricular outflow tract (RVOT) on respiration. During expiration, the RVOT was compressed and narrow, while it was expanded during inspiration. Cardiac catheterization demonstrated a 10-mmHg of pressure gradient across the RVOT during expiration but no pressure gradient during inspiration. Thus, respiratory compression to the RVOT by a narrow thoracic cage due to SBS was the cause of the cardiac murmur with respiratory alterations. Our case highlights the importance of physical examination, including an inspection of the patient's physique. .

Unusual double-chambered right ventricle induced by ruptured sinus of Valsalva aneurysm: A case report.

A 54-year-old male with a history of unrepaired ventricular septal defect (VSD) suffered from easy fatigability on exertion. A Levine grade V/VI continuous murmur was auscultated. Transthoracic echocardiogram showed a ruptured sinus of Valsalva aneurysm (SVA) and a significant left-to-right shunting from the ascending aorta to the right ventricle (RV). In addition, a 36 mmHg of pressure gradient was observed between the inflow and outflow tract in the RV, suggesting double-chambered RV (DCRV). Cardiac catheterization also revealed 33 mmHg of the pressure gradient in the mid-potion of the RV, which was coincident with DCRV. A calculated pulmonary-to-systemic flow ratio was 3.0. Therefore, the patient was offered surgical repair of the ruptured SVA and VSD, which was successfully performed. During the surgery, an anomalous muscle band, which is usually the cause of DCRV, was not found, instead, a thickened RV free-wall due to the exposure of the left-to-right shunt flow, so-named jet lesion, was found. Therefore, surgical resection of the anomalous muscle band was not required. The protruded SVA toward the RV, the jet lesion, and the increased RV stroke volume, which could induce relative stenosis, were the causes of the unusual DCRV. .

Importance of pleural findings in patients with amyloid cardiomyopathy complicated with refractory pleural effusion.

A 76-year-old male was admitted to our hospital for progressive bilateral pleural effusion. Because of typical echocardiographic findings such as left ventricular (LV) hypertrophy, thickness of the mitral valve, and a granular sparkling appearance of the LV wall, amyloid cardiomyopathy was suspected. Regardless of up-titration of several diuretic agents, the bilateral pleural effusion did not improve. Because the histological findings of the right ventricular septum (direct-fast-scarlet staining) obtained by biopsy that demonstrated amyloid deposits in perivascular and pericellular lesions, amyloid cardiomyopathy was diagnosed. However, cardiac catheterization revealed normal right and left atrial pressure and normal right and left ventricular end-diastolic pressure. Therefore, hemodynamic deterioration was less likely to be the cause of persistent pleural effusion. Amyloid deposits were also detected in the pleural biopsy specimen, so pleural amyloidosis was diagnosed and may have played an important role in the refractoriness of the pleural effusion. .

An early atherosclerotic change detected by an aortic angioscopy in a young patient with coarctation of the aorta: a case report.

The patient was a 19-year-old woman who had experienced headache for 1 year. Soon after birth, ventricular septal defects were diagnosed, the size of which were small, therefore not requiring surgical repair. She also noticed hypertension, with up to 184/110 mmHg of blood pressure. Her physical examination revealed a difference in blood pressure between her upper and lower limbs (160/108 and 92/65 mmHg, respectively). A cardiac computed tomography image clearly demonstrated the narrowing of the aortic isthmus. Coarctation of the aorta (CoA) was definitively diagnosed and was the cause of the upper limb hypertension and headache. Cardiac catheterization revealed 3.8 mm of the aortic isthmus and 65 mmHg of the peak-to-peak pressure gradient across the CoA. The patient was offered endovascular therapy of the CoA. A non-covered stent implantation was successfully performed and the pressure gradient across the aortic isthmus disappeared. Her upper limb hypertension also improved. Aortic angioscopy revealed a yellow plaque on the aortic intima, located proximal to the coarctation site, which was exposed owing to high blood pressure. Our case highlights that an atherosclerotic change can develop even in young patients with hypertension. .