Progressive worsening of aortic regurgitation due to detachment of the aortic valve commissure with multimodality imaging to elucidate pathogenesis: a case report.

Background: Aortic regurgitation (AR) associated with detachment of the aortic valve commissure is extremely rare. We present a case of progressively worsening severe chronic AR due to detachment of the aortic valve commissure during hospitalization that was confirmed with multimodality imaging. Case summary: A 50-year-old male with Marfan syndrome visited our hospital to receive treatment for cholelithiasis. Pre-operative examination revealed severe AR and aortic root aneurysm. Because the patient was asymptomatic, it was decided that cholecystectomy should be performed first. However, the patient's heart failure worsened acutely when his blood pressure increased just before induction of anaesthesia. The patient required intubation and management of heart failure. Five days later, the patient underwent cholecystectomy. He was treated for heart failure and underwent open heart surgery on the 35th hospital day. Intraoperative transoesophageal echocardiography revealed that his AR was caused by both enlargement of the aortic root and localized dissection of the aortic valve commissure, which was supported by intraoperative findings and histopathological evaluation. Aortic regurgitation was exacerbated by a new localized dissection, resulting in acute worsening of heart failure. Discussion: Aortic valve commissure detachment can easily lead to sudden onset of severe AR, deteriorating haemodynamics, and acute pulmonary oedema. Since delayed medical treatment leads to poor clinical outcomes, prompt and accurate diagnosis and appropriately timed surgical intervention are essential. This very rare case of severe AR worsening due to spontaneous aortic valve commissure dissection was evaluated with multiple modalities during hospitalization. Understanding this clinical condition will help cardiologists provide better medical care.

Non-rheumatic giant left atrium: An illustrative case successfully treated by surgical intervention.

A 45-year-old male presented to us with decompensated heart failure. He had been diagnosed as having atrial fibrillation when he was 31 years old. Transthoracic and transesophageal echocardiography revealed an excessive left atrial (LA) enlargement with left ventricular dysfunction and severe functional mitral regurgitation. There were no specific findings of rheumatic valve disease. He underwent surgical mitral valve replacement and LA volume reduction surgery after optimal medical therapy. Surgically-removed specimens of the LA and the anterior mitral leaflet were examined and there were no specific histopathological findings suggesting the specific etiology of the giant LA in this patient. The patient's condition significantly improved after the surgery without any cardiac events ever since. .

Effectiveness of the taut-line hitch knot in accurately determining the length of artificial chordae during repair for degenerative mitral insufficiency.

Taut-line hitch, a type of ropework used in outdoor activities, was adopted to tie the artificial chordae during mitral valve repair for degenerative mitral insufficiency. This knot-tying technique facilitated artificial chordae length determination during surgery. Nineteen patients with degenerative mitral insufficiency were successfully treated using this technique.

Safety profile of brentuximab vedotin in Japanese patients with relapsed/refractory Hodgkin lymphoma or systemic anaplastic large cell lymphoma: a post-marketing surveillance study.

Brentuximab vedotin (BV) was initially approved in Japan for the treatment of relapsed/refractory (R/R) CD30-positive Hodgkin lymphoma (HL) and systemic anaplastic large cell lymphoma (sALCL). As requested by the Japanese Ministry of Health, Labour and Welfare, we conducted a post-marketing surveillance (PMS) study to assess the safety of BV in Japanese patients with R/R HL or sALCL. PMS forms were collected from 284 patients (182 with HL, 101 with sALCL and one with another lymphoma) treated between April and September 2014. The median age was 62 (range 14-93) years and the median number of treatment cycles was 5.5 for HL and 4 for sALCL. Adverse drug reactions (ADRs) were reported in 74.3% of patients. The most commonly observed ADRs included peripheral sensory neuropathy (39.1%; grade ≥ 3, 6.3%), neutropenia (34.5%; grade ≥ 3, 22.2%) and lymphopenia (7.0%; grade ≥ 3, 5.3%). Ten patients had fatal ADRs including interstitial lung disease (n = 3). This study showed that BV has an acceptable safety profile in Japanese patients with R/R HL and R/R sALCL in the clinical practice setting. However, close monitoring rare, but potentially fatal, ADRs such as pulmonary toxicity may be warranted, especially in patients with prior or ongoing pulmonary disorders.

Influence of preoperative right ventricular function on left ventricular remodeling and survival after subvalvular repair for functional mitral regurgitation.

OBJECTIVES: The objective of this study was to analyze our surgical experiences with mitral valve plasty (MVP) combined with subvalvular procedures (SVPs) for functional mitral regurgitation (FMR) and to determine which preoperative factors affected clinical outcomes. METHODS: This study retrospectively analyzed 33 patients who underwent MVP combined with SVPs for FMR with a left ventricular ejection fraction lower than 40% and advanced remodeled left ventricles. The mean follow-up period was 49 ± 33 months. RESULTS: The preoperative mean right ventricular fractional area change (RVFAC) used to quantify right ventricular (RV) systolic function was 26 ± 11%. Sixteen patients (48%) had an RVFAC < 26%. One patient died during hospital stay, and nine more patients died of cardiac causes during follow-up. The 3- and 5-year rates of freedom from cardiac-related mortality were 78% and 68%, respectively. RVFAC was the significant predictor of cardiac-related mortality in a univariate analysis (risk ratio [RR] = 0.92, 95% confidence interval [CI] 0.85-0.99, p = 0.03) and demonstrated a non-significant tendency to predict cardiac-related mortality in the Cox multivariate analysis (RR = 0.94, 95% CI 0.86-1.003, p = 0.08). Continued reverse left ventricular remodeling was associated with an RVFAC ≥ 26%. At 3 years, there was also a significant difference in survival rates of cardiac-related mortality between patients with an RVFAC ≥ 26% and < 26% (94% vs. 61%; p = 0.03). CONCLUSIONS: Preoperative RV function affected left ventricular remodeling and cardiac-related mortality after MV surgery. MVP combined with SVPs for FMR provided promising results for patients without severe RV dysfunction.

Clinical Outcomes of a Customized Mitral Valve Plasty for Functional Mitral Regurgitation with a Low Ejection Fraction and Implications for Preoperative Right Ventricular Function.

PURPOSE: To evaluate clinical outcomes of customized mitral valve plasty (MVP) for the treatment of functional mitral regurgitation (FMR) with a low ejection fraction (EF) and to determine which preoperative factors affected the clinical outcome. METHODS AND RESULTS: MVP was adjusted according to the degree of left ventricle (LV) remodeling. We performed mitral annuloplasty (MAP) alone in 14 patients and added subvalvular procedures (SVPs) in 22 patients at a high risk of recurrent MR. During follow-up, reverse LV remodeling was obtained and the 3-year and 5-year non-recurrence rates of MR grade ≥2 were 94% and 89%, respectively. Two patients died during their hospital stay, and four more patients died of cardiac causes during follow-up. The 3-year and 5-year rates of freedom from cardiac-related mortality were 86% and 81%, respectively; no significant difference was observed between the two treatment groups. Right ventricular fractional area change (RVFAC) was a significant predictor of cardiac mortality. Patients with an RVFAC of <26% had significantly poorer cardiac-related mortality (71% at 3 years) than those with an RVFAC of ≥26% (95% at 3 years). CONCLUSION: Customized MVP provided durable mitral competence and reverse LV remodeling. Preoperative RV function was associated with cardiac-related mortality.

Upregulated Kynurenine Pathway Enzymes in Aortic Atherosclerotic Aneurysm: Macrophage Kynureninase Downregulates Inflammation.

AIMS: Inflammation and hypertension contribute to the progression of atherosclerotic aneurysm in the aorta. Vascular cell metabolism is regarded to modulate atherogenesis, but the metabolic alterations that occur in atherosclerotic aneurysm remain unknown. The present study aimed to identify metabolic pathways and metabolites in aneurysmal walls and examine their roles in atherogenesis. METHODS: Gene expression using microarray and metabolite levels in the early atherosclerotic lesions and aneurysmal walls obtained from 42 patients undergoing aortic surgery were investigated (early lesion n=11, aneurysm n=35) and capillary electrophoresis-time-of-flight mass spectrometry (early lesion n=14, aneurysm n=38). Using immunohistochemistry, the protein expression and localization of the identified factors were examined (early lesion n=11, non-aneurysmal advanced lesion n=8, aneurysm n=11). The roles of the factors in atherogenesis were analyzed in macrophages derived from human peripheral blood mononuclear cells. RESULTS: Enrichment analysis using 35 significantly upregulated genes (log2 ratio, ＞3) revealed the alteration of the kynurenine pathway. Metabolite levels of tryptophan, kynurenine, and quinolinic acid and the kynurenine-to-tryptophan ratio were increased in the aneurysmal walls. Gene and protein expression of kynureninase and kynurenine 3-monooxygenase were upregulated and localized in macrophages in the aneurysmal walls. The silencing of kynureninase in the cultured macrophages enhanced the expression of interleukin-6 and indoleamine 2,3-dioxygenase 1. CONCLUSION: Our study suggests the upregulation of the kynurenine pathway in macrophages in aortic atherosclerotic aneurysm. Kynureninase may negatively regulate inflammation via the kynurenine pathway itself in macrophages.

Mitral valve repair in Barlow's disease by chordal reconstruction using the adjustable slip-knot technique.

The adjustable slip-knot technique-a procedure for facilitating repair of the mitral valve prolapse-was first performed and reported by us in 2015. This technique comprises six steps: anchor institution in the papillary muscle, flexible band positioning, saline injection to confirm the prolapsing site, neochorda setting between the anchor and leaflet, fine adjustment of the neochordal length, and knot-fixing to confirm repair. Here, we have described the first case of successful mitral valve repair using the adjustable slip-knot technique in a 51-year-old man with Barlow's disease.

Novel quantitative and objective structured assessment of technical skill for slip knotting.

OBJECTIVE: Currently, no quantitative and objective method has been established for evaluating competencies in basic surgical techniques. The aim of this study was to develop a structured assessment tool for slip knotting and verify how well current board certification system discriminates the level of basic surgical skill. METHODS: We examined 171 cardiovascular surgical fellows using a novel assessment method for slip knotting that was developed by the committee of the Under-Forty of the Japanese Society of Cardiovascular Surgery. We compared the scores and examinees' surgical experience for validation. We analyzed the relationship between board certification and the scores. RESULTS: The scores differentiated the general surgical board-certified surgeons from those without certification. Surgical experiences such as training years and number of operated cases and scores were correlated. Among the board-certified surgeons, the group with daily off-the-job training, or simulator-based skill training had a significantly higher mean score (67.4 ± 3.0 vs 55.4 ± 3.1, p = 0.008) and lower rate of poor scorers (7.1% vs 38.5%, p = 0.004). A multivariate analysis revealed that board certification did not predict high scores. Daily off-the-job training was the only independent predictor of high scores (odds ratio: 2.41, 95% confidence interval: 0.01-1.20, p = 0.014). CONCLUSIONS: This novel quantitative and objective assessment tool for technical skill in slip knotting was found to be valid to examine the skill for slip knotting. In this study, current board certification discriminated the level of basic surgical skill. However, it could not distinguish extremely low scorers perfectly. Some board-certified surgeons showed poor technical competency, especially those without off-the-job training.

Correction to: Novel quantitative and objective structured assessment of technical skill for slip knotting.

The article "Novel quantitative and objective structured assessment of technical skill for slip knotting", written by.

Significance of preoperative right ventricular function on mid-term outcomes after surgical ventricular restoration for ischemic cardiomyopathy.

OBJECTIVES: To analyze our surgical experiences with surgical ventricular restoration (SVR) for dilated ischemic cardiomyopathy (ICM) and to determine the significance of preoperative right ventricular (RV) function on outcomes. METHODS AND RESULTS: This study retrospectively analyzed 19 patients who underwent SVR between April 2010 and May 2016. Their mean age and New York Heart Association functional class were 62 ± 11 years and 2.9 ± 0.8, respectively. The preoperative mean left ventricular (LV) end-systolic volume index and LV ejection fraction (LVEF) were 134 ± 56 mL/m2 and 24 ± 7%, respectively. The preoperative mean RV fractional area change (RVFAC) to quantify RV systolic function was 33 ± 13%, as assessed by transthoracic echocardiography. The mean follow-up period was 47 ± 20 months. Three patients died of cardiac causes during the follow-up, with the 3-year and 5-year freedoms from cardiac-related death of 89% and 79%, respectively. Major adverse cardiac events (MACEs) occurred in ten patients, with the 3-year and 5-year MACE-free survival rates of 58% and 41%, respectively. RVFAC (risk ratio [RR] = 0.92, 95% confidence interval [CI] 0.86-0.98, p = 0.01) and LVEF (RR = 0.83, 95% CI 0.68-0.97, p = 0.02) were significant predictors of MACEs in the multivariate analysis. Patients with RVFAC of < 35% had significantly poorer MACE-free survival rates (33% at 3 years) than those with RVFAC of ≥ 35% (80% at 3 years). CONCLUSION: SVR for ICM provided acceptable freedom from cardiac-related death; however, MACEs commonly occurred and was associated with RV dysfunction.

Mid-term results of mitral valve repair for ischemic mitral regurgitation adjusted according to the degree of remodeling progression.

OBJECTIVES: Mitral annuloplasty (MAP) for ischemic mitral regurgitation (IMR) with advanced remodeling is often associated with recurrent mitral regurgitation (MR). We performed surgeries adjusted according to the degree of remodeling; i.e., for advanced remodeling cases, we added subvalvular repair (SVR) to MAP. This study aimed to evaluate our experience with patients with IMR. METHODS AND RESULTS: Thirty patients with IMR (MR grade ≥ 2) were retrospectively analyzed. We performed MAP with SVR for 13 patients (MAP + SVR group) and MAP alone for 17 patients (MAP group). The MAP + SVR group had significantly larger left ventricular dimension and tenting height than the MAP group. Four patients (two in each group) experienced recurrence of MR (MR grade ≥ 2), and the 3-year and 5-year freedom from recurrence of MR rates were 97% and 83%, respectively. Six patients (two in the MAP group and four in the MAP + SVR group) died of cardiac causes during follow-up, and the 3-year and 5-year freedom from cardiac-related death rates were 81% and 74%, respectively. There was no difference between the 2 groups regarding freedom from recurrent MR (p = 0.6) and freedom from cardiac-related death (p = 0.1). The preoperative left ventricular end-systolic dimension was a significant predictor of cardiac death in the multivariate analysis. CONCLUSION: Mitral valve repair adjusted according to the degree of remodeling progression provided durable mitral competence; however, it remains unclear if such a strategy is related to a high probability of freedom from cardiac-related death.

Comparison of mitral competence after mitral repair with papillary muscle approximation versus papillary muscle relocation for functional mitral regurgitation.

The purpose of this study was to evaluate the surgical results of papillary muscle approximation (PMA) and papillary muscle relocation (PMR) for functional mitral regurgitation (FMR) and to compare the effects of both procedures on the change in mitral regurgitation (MR) and echocardiogram parameters associated with tethering. Eighteen patients with moderate-to-severe FMR (MR grade ≥2) who underwent PMA or PMR were retrospectively analyzed. Underlying diseases were ischemic cardiomyopathy, idiopathic dilated cardiomyopathy, and aortic valve disease for seven, six, and five patients, respectively. Eleven patients underwent PMA and seven patients underwent PMR. Mitral annuloplasty and surgical ventricular restoration were performed concomitantly for 18 and 6 patients, respectively. None of these patients died in the hospital. Three patients died during the late period; two of these deaths were cardiac related. The rate of 3 years of freedom from cardiac-related death was 89%. After a mean follow-up of 33 months, MR grade was significantly improved compared with preoperative values (3.0 ± 0.8 to 0.7 ± 1.2; p < 0.01). Recurrence of MR grade ≥2 occurred in three patients and the rate of 3 years of freedom from recurrence of MR grade ≥2 was 87%. During follow-up, tenting height (1.1 ± 0.2 to 0.7 ± 0.2 cm; p < 0.01), tenting area (2.2 ± 0.7 to 0.9 ± 0.5 cm2; p < 0.01), and anterior leaflet tethering angle (39° ± 11° to 26° ± 8°; p < 0.01) were significantly improved compared with preoperative values. Posterior leaflet tethering angle significantly deteriorated from 40° ± 7° to 53° ± 15° (p < 0.01); however, it did not further deteriorate compared with the early postoperative value of 55° ± 16° (p = 0.7). There was no difference in echocardiogram parameters associated with tethering between PMA and PMR throughout the observation period. Both methods were associated with lasting relief of MR and reverse left ventricular remodeling. There was no difference between PMA and PMR regarding the effect on mitral valve competence. Both methods allowed durable mitral repair and good clinical outcomes.

Effect of preoperative left ventricular diastolic dysfunction on mid-term outcomes after surgical ventricular restoration for ischemic cardiomyopathy.

OBJECTIVES: The impact of surgical ventricular restoration (SVR) on survival and major adverse cardiac events (MACEs) is still controversial. The purposes of this study were to analyze our surgical experience with SVR for ischemic cardiomyopathy and to determine the effect of preoperative left ventricular diastolic dysfunction on mid-term outcomes after SVR. METHODS AND RESULTS: Between April 2010 and May 2016, 19 patients underwent SVR. The mean age was 60 ± 11 years and the mean New York Heart Association functional class was 2.9 ± 0.8. Preoperative mean left ventricular end systolic volume index (LVESVI) and ejection fraction (LVEF) were 134 ± 56 mL/m2 and 24 ± 7%, respectively. The early-to-late mitral valve flow ratio (E/A) on echocardiogram was 2.4 ± 1.8 and 9 patients had E/A ≥2, excluding 2 patients with atrial fibrillation. The mean follow-up period was 29 ± 16 months. One patient died of heart failure at 6 months postoperative; the overall survival rate at 3 years was 95%. MACEs requiring hospitalization occurred in 10 patients; E/A ≥2, or restrictive filling pattern, was the only significant predictor of MACE in multivariate analysis. Reverse remodeling was associated with E/A <2, but not E/A ≥2. There was also a significant difference between patients with E/A <2 vs. ≥2 with respect to MACE-free survival rates at 3 years (100 vs. 10%; p = 0.001). CONCLUSION: The degree of preoperative diastolic dysfunction can influence the outcome after SVR. Patients with E/A ≥2 may not be good candidates for SVR.

Multiple 18F-Fluorodeoxyglucose Positron Emission Tomography Scans Showing Progression of Abdominal Aortic Aneurysm: A Case Report.

Although the precise mechanisms underlying the pathogenesis of abdominal aortic aneurysm (AAA) remain unclear, aortic wall inflammation has been implicated in AAA development. Several studies have reported the use of fluoro-deoxyglucose (F-FDG)/positron emission tomography (PET) to assess the nature of AAA.We present a case of 77-year-old Japanese male with juxta-anastomotic AAA who was followed up with multiple F-FDG-PET/CT scans over 7 years. The scans revealed chronological changes in aortic wall inflammation leading to progress and eventual rupture.This case supports a notion that aortic wall inflammation plays a role in AAA progression and rupture.

The adjustable slipknot technique for implanting neochordae in the repair of mitral valve prolapse.

The adjustable slipknot technique is a novel procedure for implanting neochordae. The neochorda acts like a lasso. The procedure facilitates accurate assessment of the length of the neochordae. We performed repairs for 30 of 31 (97.7%) patients using this technique. Less than mild residual regurgitation was observed in all patients at hospital discharge.

Unroofed coronary sinus detected by 2D/3D echocardiography in a patient referred to catheter ablation for atrial fibrillation.

A 67-year-old female visited our echo lab for a preoperative screening exam before radiofrequency catheter ablation for atrial fibrillation. Transthoracic echocardiography revealed abnormal color flow signal in the right atrium, and following 2D/3D transesophageal echocardiography proved partial type of unroofed coronary sinus. Absence of associated congenital abnormalities was ruled out by Doppler and contrast echo examinations. Surgical findings confirmed exactly the same anatomical abnormalities as preoperative 2D/3D echocardiography. Echocardiography plays an important role in finding unexpected underlying cardiac diseases in patients who are referred to arrhythmia clinics. In the present case with atrial fibrillation, we could accurately diagnose an unroofed coronary sinus syndrome during preoperative transthoracic and transesophageal echocardiography prior to planned radiofrequency catheter ablation. .

Two dehiscences of the aortic valve commissure and cusp with progressive acute aortic regurgitation.

A 54-year-old female with acute heart failure due to aortic regurgitation (AR) was admitted to our hospital. Following admission, her condition worsened progressively; thus, surgery was performed prematurely. During surgery, two dehiscences were visualized in the aortic valve commissure between the right and left cusps and the upper part of the left coronary artery ostium. However we scheduled aortic valve replacement (AVR) at first, we made the shift to perform the aortic root replacement for reinforcement of the aortic wall around the left coronary artery ostium. We describe a rare case of two dehiscences at the aortic root, which is the first report.