Characteristics and long-term outcome for congenital left main coronary artery atresia.

The prevalence of congenital left main coronary artery atresia is very low. We report the characteristics and long-term outcomes of four children with left main coronary artery atresia. Three patients had heart murmurs due to mitral regurgitation at less than 1 year old. Their myocardial ischaemia worsened on exercise with aging. In the fourth patient, hypertrophic cardiomyopathy and Noonan syndrome were suspected at 1 year old. The development of communicating arteries between the conus branch and the left anterior descending artery was detected at 7 years old. The left main coronary artery atresia was confirmed by a selective coronary angiogram at 15 years old. Congenital left main coronary artery atresia could not be diagnosed by two-dimensional echocardiography; however, the left coronary arteries were small. Two patients underwent coronary artery bypass grafting of the left anterior descending artery using the left internal thoracic artery at 3 years and 6 years old, respectively. Two patients had an angioplasty with a cut back at the orifice of the left coronary artery at 2 years old and 17 years old, respectively. Two patients had no cardiac events without medication for more than 30 years after the operation. We must differentiate the diagnosis of left main coronary artery atresia in the small left coronary arteries with mitral regurgitation during the first year. Coronary artery revascularisation and mitral annuloplasty are needed. The long-term outcome of both coronary artery bypass grafting and angioplasty were good. The degree of mitral regurgitation after surgery may affect the prognosis.

[Robot-assisted Cardiac Surgery Council and Current Status and Future Prospect of Robotic Mitral Valve Surgery in Japan].

The Robot-assisted Cardiac Surgery Council (RACSC) was established in 2015, and consists of the members of three closely related surgical societies and associations. The RACSC conducts the review and approval of applicant surgeons and institutions. A registry system has also been established through the Japan Cardiovascular Surgery Database-National Clinical Database( JCVSD-NCD). For the last five years, including the time when robotic mitral valve surgery was first approved as a procedure covered by the Japanese social insurance system in 2018, a total of 1,161 patients were enrolled in the JCVSDNCD, with an increase of nearly 500 cases per year. These cases underwent operations in 25 approved hospitals. The mean patient age was 59±13 years( range:20-90 years), and the male to female patient ratio was 1.8 male dominant. The most common procedure was mitral valve reconstruction, and 79% of all patients underwent annuloplasty. The mean operative time was 238±100 min, the mean cardiopulmonary bypass (CPB) time was 158±69 min, and the mean aortic cross-clamp (AXC) time was 102±49 min. When these results were compared with those of the Cleveland Clinic, which treated more than 1,000 cases as an initial institutional experience, both CPB and AXC times were nearly the same. It is believed that 200 cases are needed to stabilize the CPB and AXC time. Currently in Japan, only 3 or 4 out of the 25 approved hospitals have performed more than 200 cases over the past 5 years. The 30-day hospital mortality and redo operation rate for persisting mitral regurgitation was 0.3% and 0.6%, respectively, almost equivalent to that of the Cleveland Clinic. We believe that the 5-year result for robotic mitral surgery in Japan is acceptable. It is of note, however, that the length of hospital stay was 10.8±7.6 days, much longer than that of patients at the Cleveland Clinic( 5.2±2.8 days), and the homologous blood transfusion rate was 40.3%, twofold higher than that of the Cleveland Clinic( 20.5%). Therefore, the full benefits of less invasive robotic mitral repair surgery have not yet been achieved. Further improvements in results are both essential and anticipated.

Mortality analysis of arterial switch operation for transposition of the great arteries with and without ventricular septal defect.

OBJECTIVES: We aimed to evaluate the 90-day mortality and effect of rescue or urgent coronary revascularization in children undergoing arterial switch operation for transposition of the great arteries with and without ventricular septal defect. METHODS: The 90-day mortality, risk factors for mortality and outcome of rescue or urgent coronary revascularization were analysed using 8 years of data from the Japan Cardiovascular Surgical Database. We only included patients with full data for all analyses. RESULTS: A total of 1084 patients (median weight: 3.0 kg; interquartile range: 2.8-3.3) underwent arterial switch operation at a median age of 10 days (interquartile range: 7-14). The 90-day mortality (5.2%, n = 56) was ∼1.6-fold higher than 30-day mortality (3.2%, n = 35). The cause was cardiac origin in 84% of non-survivors. Fifty-nine of the 1034 patients (5.7%) required extracorporeal membrane oxygenation (ECMO), with successful weaning in 44% (n = 26). Univariable or multivariable analyses revealed the following risk factors for mortality: body weight at operation <2.5 kg, aortic cross-clamp time, cardiac events and ECMO (P < 0.005-0.001). Ventricular septal defect was not a risk factor.Thirteen patients (1.2%) had either rescue or urgent coronary revascularization with salvage rates of 25% (2/8) and 100% (5/5), respectively. Only 5 ECMO patients (8%) underwent coronary revascularization with 1 survivor. CONCLUSIONS: The 90-day mortality represented perioperative outcomes better than 30-day mortality. Patients on ECMO, which extended the survival time, had 56% 90-day mortality. Coronary revascularization showed a salvaging effect, although the case number was small.

Benefits of robotically-assisted surgery for complex mitral valve repair.

OBJECTIVES: To determine whether robotic mitral valve repair can be applied to more complex lesions compared with minimally invasive direct mitral valve repair through a right thoracotomy. METHODS: We enrolled 335 patients over a 9-year period; 95% of the robotic surgeries were performed after experience performing direct mitral valve repair. RESULTS: The mean age in the robotic versus thoracotomy repair groups was 61 ± 14 vs 55 ± 11 years, respectively (P < 0.001); 97% vs 100% of the patients, respectively, had degenerative aetiologies. Repair complexity was simple in 106 (63%) vs 140 (84%), complex in 34 (20%) vs 20 (12%) and most complex in 29 (17%) vs 6 (4%) patients undergoing robotic versus thoracotomy repair, respectively. The average complexity score with robotic repair was significantly higher versus thoracotomy repair (P < 0.001). The robotic group underwent more chordal replacement using polytetrafluoroethylene and less resections. All patients underwent ring annuloplasty. Cross-clamp time did not differ between the groups, and no strokes or deaths occurred. More patients undergoing robotic repair underwent concomitant procedures versus the thoracotomy group (30% vs 14%, respectively; P < 0.001). The overall repair rate was 100%, with no early mortality or strokes in either group. Postoperative mean residual mitral regurgitation was 0.3 in both groups, and the mean pressure gradient through the mitral valve was 2.4 vs 2.7 mmHg (robotic versus thoracotomy repair, respectively; P = 0.031). CONCLUSIONS: Robotic surgery can be applied to repair more complex mitral lesions, with excellent early outcomes.

Effect of Therapeutic Modification on Outcomes in Heart Transplantation Over the Past Two Decades　- A Single-Center Experience in Japan.

BACKGROUND: During these 2 decades (1999-2019), many therapeutic strategies have been developed in the field of heart transplant (HTx) to improve post-HTx outcomes. In the present study, 116 consecutive HTx adults between 1999 and 2019 were retrospectively reviewed to evaluate the influences of a therapeutic modification on post HTx outcomes.Methods and Results:Patient survival, functional status and hemodynamics after HTx and modification of therapeutic strategies were reviewed. The overall cumulative survival rate at 10 and 20 years post-HTx was 96.4 and 76.7%, respectively. There were no significant differences in survival rate or exercise tolerance after HTx between extracorporeal and implantable continuous flow-LVAD. Post-HTx patient survival in patients, irrespective of the donor risk factors such as donor age, low LVEF, history of cardiac arrest, was equivalent across cohorts, while longer TIT and higher inotrope dosage prior to procurement surgery were significant risk factors for survival. In 21 patients given everolimus (EVL) due to renal dysfunction, serum creatinine significantly decreased 1 year after initiation. In 22 patients given EVL due to transplant coronary vasculopathy (TCAV), maximum intimal thickness significantly decreased 3 years after initiation. CONCLUSIONS: The analysis of a 20-year single-center experience with HTx in Japan shows encouraging improved results when several therapeutic modifications were made; for example, proactive use of donor hearts declined by other centers and the use of EVL in patients with renal dysfunction and TCAV.

Bilateral internal thoracic artery grafting in children under 5 years of age with Kawasaki disease: a case series.

BACKGROUND: Although persistent coronary artery aneurysm of Kawasaki disease (KD) is rare, some patients develop a life-threatening myocardial infarction. In paediatric coronary artery bypass surgery (PCABS), the internal thoracic artery (ITA) graft is a reliable graft with favourable coronary outcomes. However, few studies have reported the outcomes and technical considerations of PCABS using bilateral ITAs in small children who have multivessel disease. CASE SUMMARY: We present the cases of three children under 5 years of age who underwent PCABS utilizing bilateral ITAs. All three patients had known bilateral giant coronary aneurysms associated with KD. Paediatric coronary artery bypass surgery was indicated after confirming multiple coronary lesions with myocardial ischaemia. One child underwent emergency PCABS because of circulatory collapse. The arterial anastomoses were performed under cardioplegic arrest. The left ITA was anastomosed to the left anterior descending artery (LAD) or the circumflex artery. The right ITA was anastomosed to the right coronary artery or the LAD. Post-operative coronary angiography revealed patent bilateral ITA grafts with an excellent run-off in all patients, and none of them have suffered any subsequent coronary event. DISCUSSION: Bilateral ITA grafting is a feasible procedure with favourable coronary outcomes for treating small children with multivessel disease, even in the setting of circulatory collapse.

Robotic minimally invasive direct coronary artery bypass for Kawasaki disease.

Case 1: 17-year-old boy developed severe stenosis at the proximal site of the coronary aneurysm in the left anterior descending artery (LAD). Case 2: 16-year-old boy developed severe stenosis at the proximal site of the coronary aneurysm in the LAD. Case 3: 30-year-old woman developed severe stenosis of the distal portion of the coronary aneurysm in the LAD. Minimally invasive direct coronary artery bypass (MIDCAB) with robot-assisted left internal thoracic artery harvest was successfully performed without cardiopulmonary bypass in these three young patients with Kawasaki disease. This is the first case report of robot-assisted MIDCAB for Kawasaki disease.

Long-Term Results of Intracardiac Mesenchymal Stem Cell Transplantation in Patients With Cardiomyopathy.

BACKGROUND: Mesenchymal stem cells (MSCs), which have the potential to differentiate into cardiomyocytes or vascular endothelial cells, have been used clinically as therapy for cardiomyopathy. In this study, we aimed to evaluate the long-term follow-up results.Methods and Results:We studied 8 patients with symptomatic heart failure (HF) on guideline-directed therapy (ischemic cardiomyopathy, n=3; nonischemic cardiomyopathy, n=5) who underwent intracardiac MSC transplantation using a catheter-based injection method between May 2004 and April 2006. Major adverse events and hospitalizations were investigated up to 10 years afterward. Compared with baseline, there were no significant differences in B-type natriuretic peptide (BNP) (from 211 to 173 pg/mL), left ventricular ejection fraction (LVEF) (from 24% to 26%), and peak oxygen uptake (from 16.5 to 19.2 mL/min/kg) at 2 months. During the follow-up period, no patients experienced serious adverse events such as arrhythmias. Three patients died of pneumonia in the 1st year, liver cancer in the 6th year, and HF in the 7th year. Of the remaining 5 patients, 3 patients were hospitalized for exacerbated HF, 1 of whom required heart transplantation in the 2nd year; 2 patients survived for 10 years without worsening HF. CONCLUSIONS: The results of this exploratory study of intracardiac MSCs administration suggest further research regarding the feasibility and efficacy is warranted.

Multiple Coronary Artery Bypass Grafting for Kawasaki Disease-Associated Coronary Artery Disease.

BACKGROUND: Although coronary artery bypass grafting (CABG) is the preferred choice for advanced Kawasaki disease (KD)-associated coronary artery disease, graft design such as number of grafts or type of conduits has not been fully established. We reviewed a series of patients who underwent single or multiple CABG for coronary artery disease of KD sequelae to investigate the optimum revascularization strategy. METHODS: We enrolled a consecutive series of 102 CABG surgeries in 92 patients during the last 36 years. Mean patient age at CABG was 14.9 ± 10.4 years. Internal thoracic artery, radial artery, and gastroepiploic artery were used in 100 (98%), 18 (15%), and 4 (4%) cases, respectively. Patients were divided into 2 groups by single (n = 53) or multiple (n = 49) CABG. RESULTS: Actuarial survival was 93% in single CABG and 91% in multiple CABG at 30 years (P = .71). There was no in-hospital mortality, but 6 deaths occurred long term, with no significant difference between the groups. Freedom from cardiac events was 45.2% in single CABG and 68.5% in multiple CABG at 25 years (P = .228), and reintervention to the left anterior descending (LAD) artery territory was the most common event. Graft patency of the internal thoracic artery-LAD artery graft was 81% in single CABG and 85% in multiple CABG at 25 years. Patency of the radial artery in the non-LAD artery territories was significantly greater than that of the saphenous vein graft at 10 years (91% vs 46%, P = .013). CONCLUSIONS: Multiple CABG using arterial conduits is feasible, safe, and therapeutically effective long term for patients with advanced KD-associated coronary artery disease.

Decreased perfusion of the posterior cingulate gyri shown by a cingulate island score is a possible marker of vulnerability to behavioural and psychological symptoms of Alzheimer's disease: a pilot study.

BACKGROUND: The cingulate island score (CIScore), which indicates the Z-score ratio of the posterior cingulate gyri to the medial occipital area, has been shown to be useful for differentiating dementia with Lewy bodies from Alzheimer's disease (AD). Our aim was to investigate associations between the clinical symptoms of AD and the CIScore as an index of the relative decrease in perfusion of the posterior cingulate gyri that occurs in the early stages of AD. METHODS: Seventeen patients with early-stage AD and 13 patients with amnesic mild cognitive impairment were examined. Z-score maps of technetium-99m ethyl cysteinate dimer single-photon emission computed tomography images acquired from the patients were converted, and the CIScore was determined by using the easy Z-score imaging system. The relationships between the CIScore and clinical symptom scores were tested. RESULTS: A significant correlation was identified between the CIScore and the Neuropsychiatric Inventory Questionnaire score. No significant correlations were identified between the CIScore and other measures of cognitive function. Based on a CIScore of 0.39, we correctly differentiated patients with and without behavioural and psychological symptoms of dementia (BPSD), with a sensitivity of 72.2% and specificity of 75.0%. DISCUSSION: Using technetium-99m ethyl cysteinate dimer single-photon emission computed tomography, we observed that decreased posterior cingulate gyri perfusion, relative to the medial occipital area, in prodromal and early AD was closely associated with behavioural and psychological symptoms of dementia. Therefore, our findings suggest that CIScore is not only useful for discriminating dementia with Lewy bodies from AD, but it can also be clinically used as a specific indicator of the vulnerability to behavioural and psychological symptoms of dementia in the early stages of AD.

Pediatric Coronary Artery Bypass Surgery for Congenital Heart Disease.

BACKGROUND: Pediatric coronary artery bypass surgery (PCABS) for congenital heart disease has become increasingly important in infants and children undergoing modern cardiac surgical procedures, because of its life-saving potential in unsuccessful coronary transfer operation. This review summarizes the current surgical role of PCABS for treating congenital heart diseases. METHODS: Databases, mainly PubMed, were searched for relevant publications about coronary bypass operation in the pediatric population, from 1970 (commencement) to March 2018. RESULTS: The five main indications were (1) anomalous origin of the left coronary artery from the pulmonary artery, (2) left main coronary trunk atresia, (3) acute and late coronary events in the arterial switch operation for transposition of the great arteries, (4) the Ross operation for congenital aortic stenosis complicated by destructive infective endocarditis, and (5) inadvertent coronary artery injury during heart operation. Direct coronary reimplantation (coronary transfer) in most and surgical angioplastic procedures in selected patients were the first choice; however, PCABS with internal thoracic artery (ITA) grafts can be lifesaving in emergency or severe myocardial hypoperfusion conditions. Because the patency of saphenous vein grafts is poorer than that of ITA grafts, their use should be avoided in growing children. The procedure can be performed safely in neonates, infants, and small children, using high-power magnifying glasses or a surgical microscope. CONCLUSIONS: Long-term results of survival and ITA graft function are excellent in growing children and adults. Congenital heart surgeons should be trained in quick graft takedown and in coping with emergency and late devastating complications in coronary transfer operation.

A new arena in cardiac surgery: Pediatric coronary artery bypass surgery.

Prior to the 1970s, pediatric coronary artery bypass surgery (PCABS) was seldomly performed due to the lack of compelling surgical indications. The advent of coronary sequelae secondary to Kawasaki disease (KD) and the occurrence of coronary artery complications due to newly developed procedures, such as the arterial switch operation and early repair for intrinsic congenital coronary malformations, necessitated the development of PCABS. Because children grow rapidly and their life expectancy is very long, with increasing exercise capability requirements, the strategy for PCABS should differ from that for bypass surgery in adults. PCABS utilizing unilateral and bilateral internal thoracic arteries (ITA) has become the most reliable surgical method for children because of the distinct structure of ITAs being resistant to KD, growth potential according to the child's somatic growth and long-term patency without wall degeneration. This operation utilizing ITA grafts is now being performed worldwide and is referred to as the "Kitamura operation" for KD coronary sequelae. Notably, the use of vein grafts should be avoided in children. Likewise, this operation can now be successfully performed in infants using a surgical microscope, for congenital coronary disorders. Currently, PCABS with ITAs has been established as a new arena in cardiac surgery, following our initial attempts.

[(11)C]TASP457, a novel PET ligand for histamine H3 receptors in human brain.

PURPOSE: The histamine H3 receptors are presynaptic neuroreceptors that inhibit the release of histamine and other neurotransmitters. The receptors are considered a drug target for sleep disorders and neuropsychiatric disorders with cognitive decline. We developed a novel PET ligand for the H3 receptors, [(11)C]TASP0410457 ([(11)C]TASP457), with high affinity, selectivity and favorable kinetic properties in the monkey, and evaluated its kinetics and radiation safety profile for quantifying the H3 receptors in human brain. METHODS: Ten healthy men were scanned for 120 min with a PET scanner for brain quantification and three healthy men were scanned for radiation dosimetry after injection of 386 ± 6.2 MBq and 190 ± 7.5 MBq of [(11)C]TASP457, respectively. For brain quantification, arterial blood sampling and metabolite analysis were performed using high-performance liquid chromatography. Distribution volumes (V T) in brain regions were determined by compartment and graphical analyses using the Logan plot and Ichise multilinear analysis (MA1). For dosimetry, radiation absorbed doses were estimated using the Medical Internal Radiation Dose scheme. RESULTS: [(11)C]TASP457 PET showed high uptake (standardized uptake values in the range of about 3 - 6) in the brain and fast washout in cortical regions and slow washout in the pallidum. The two-tissue compartment model and graphical analyses estimated V T with excellent identification using 60-min scan data (about 16 mL/cm(3) in the pallidum, 9 - 14 in the basal ganglia, 6 - 9 in cortical regions, and 5 in the pons), which represents the known distribution of histamine H3 receptors. For parametric imaging, MA1 is recommended because of minimal underestimation with small intersubject variability. The organs with the highest radiation doses were the pancreas, kidneys, and liver. The effective dose delivered by [(11)C]TASP457 was 6.9 µSv/MBq. CONCLUSION: [(11)C]TASP457 is a useful novel PET ligand for the investigation of the density of histamine H3 receptors in human brain.

Pediatric Coronary Artery Revascularization Surgery: Development and Effects on Survival, Cardiac Events and Graft Patency for Children With Kawasaki Disease Coronary Involvements.

Pediatric coronary artery bypass surgery gained wide acceptance with the introduction of internal thoracic arteries (ITAs) for bypass operations for post Kawasaki disease (KD) lesions. The technique is now established as the standard surgical choice, and its safety even in infancy, graft patency, growth potential, graft longevity and clinical efficacy have been well documented. In this article the author reviews the development of pediatric coronary bypass as the main indication for the treatment of coronary lesions due to KD. I believe that coronary revascularization surgery in pediatric population utilizing uni- or bilateral ITAs is the current gold-standard as the most reliable treatment, although percutaneous coronary intervention with or without a stent has been tried with vague long-term results in children.