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Aim: During surgery for an aortic arch aneurysm, aortic plaque in the descending aorta should be evaluated, but there are currently no suitable biomarkers for it. Surgeons should be especially aware of cerebral embolism from femoral perfusion and of peripheral embolism from stent graft deployment. Cystatin C is a known useful marker of renal dysfunction with a role as a biomarker for severity of coronary artery disease. In the absence of a suitable biomarker for aortic plaque in the descending aorta, we examine cystatin C as a candidate. Methods: In all, 75 patients who underwent surgery for an aortic arch aneurysm were enrolled. They were divided into two groups, depending on whether they had chronic kidney disease or not. The serum cystatin C value and creatinine value were evaluated preoperatively. The aortic plaque volume ratio and components in the descending aorta were calculated from preoperative enhanced computed tomography. Results: The soft plaque volume ratio was higher in patients with chronic kidney disease than in patients without it. Cystatin C positively correlated with the total aortic plaque volume ratio in all cases, and it positively correlated with the soft plaque volume ratio in both groups. Creatinine had no correlation with any type of plaque volume ratio in either group. In patients without chronic kidney disease, the soft plaque volume ratio was higher in patients with higher cystatin C levels than in patients with normal levels. Conclusion: The preoperative serum cystatin C level could be a biomarker of aortic plaque in the descending aorta in patients with an aortic arch aneurysm.

The location of the atrioventricular conduction axis in the setting of atrioventricular septal defect has previously been shown by histology and intraoperative recordings. We have now reassessed the arrangement using phase-contrast computed tomography, aiming to provide precise measurements so as to optimize future surgical repairs. We used the system based on an X-ray Talbot grating interferometer using the beamline BL20B2 in a SPring-8 synchrotron radiation facility available in Japan. We analyzed 18 specimens. The atrioventricular node was found within a nodal triangle 1.7 mm from the coronary sinus, with 95% confidence intervals from 1.45 to 2.0 millimeters. The depth of the node from the right atrial endocardium was 1.0 mm, with 95% confidence intervals from 0.73 to 1.34 mm. The overall length of the scooped-out ventricular septum was 30.8 mm, with 95% confidence intervals from 27.5 to 34.1 millimeters. The length from the inferior atrioventricular junction to the take-off of the right bundle branch was 12.8 mm, with 95% confidence intervals from 11.12 to 14.38 mm, giving a ratio of 0.43 for the extent of the axis along the inferior septum, with 95% confidence intervals of 0.38-0.48. The length of the non-branching bundle was 6.6 mm, with 95% confidence intervals from 5.57 to 7.7 mm. The proportion of septum occupied by the non-branching bundle was 0.22, with 95% confidence intervals from 0.18 to 0.26. Our findings confirm previous histological studies, extending them by providing precise measurements to guide placement of sutures during surgical repair.

The introduction of the arterial switch operation has improved the surgical outcome of transposition of the great arteries. However, coronary anomalies such as intramural coronary arteries, single coronary artery, or coronary arteries originating from a single arterial sinus have been reported as independent risk factors for early mortality and late morbidity after an arterial switch operation. We performed an arterial switch operation using a unique technique for translocation of the coronary arteries originating from a single left-side arterial sinus, to prevent coronary artery distortion and subsequent coronary malperfusion.

Simple tricuspid valve surgery for complex heart disease with systemic right ventricular dysfunction is a high‐risk procedure; however, staged tricuspid valve surgery following cardiac resynchronization therapy seems to be a beneficial method to expect reverse systemic ventricular remodeling.

Patients with left ventricular hypertrophy (LVH) have reportedly higher than normal mortality and incidences of cardiovascular events. Coronary microvascular pathophysiology also appears to differ from other populations. Such coronary microcirculation dysfunctions are considered strong causes of cardiac events. We compare the functional improvement of myocardial ischemia between LVH patients and other patients after successful coronary artery bypass surgery (CABG) using coronary flow velocity reserve (CFVR) by transthoracic echo cardiography. Patients who underwent isolated coronary artery bypass surgery, including left anterior descending artery (LAD) revascularization via "in situ" internal thoracic artery (ITA) between June 2008 and July 2017 (n = 155), were retrospectively reviewed. ITA grafts were patent in postoperative graft evaluation in all patients. CFVR was evaluated pre- and postoperatively, and data were compared between patients with severe LVH group and those without (non-LVH group). Preoperative mean CFVR was 1.77 ± 0.75 in LVH group and 1.91 ± 0.63 in non-LVH group (P = 0.188). After the operation, ITA to LAD graft patency was confirmed in all patients. Postoperative CFVR was 2.23 ± 0.70 in LVH group and 2.85 ± 0.71 in non-LVH group, respectively (P = 0.002). Significant difference was observed between the 2 groups. CFVR values improved after ITA to LAD bypass grafting in both LVH and non-LVH groups, but postoperative CFVR was significantly lower in patients with severe LVH than in patients without. Myocardial ischemia may exist in patients with LVH, despite patent graft, due to microvascular dysfunction. Comprehensive treatment, including long-term oral medication to improve microvascular dysfunction, is necessary for patients with LVH.

The selection of arterial cannulation site is an important decision to avoid cerebral complication for total arch replacement(TAR). We report the surgical outcome of TAR using bilateral axillary artery perfusion in our hospital.Between January 2012 and June 2020, 97 patients who underwent elective TAR for atherosclerotic aneurysms were enrolled in this study. Among them, bilateral axillary artery perfusion was used for 81 patients, and frozen elephant trunk( FET) procedure were used for 34 patients. In the case of FET procedure, translocated TAR was performed with distal anastomosis between the left common carotid artery and the left subclavian artery. The left subclavian artery was reconstructed by rerouting the graft used for the left axillary artery perfusion.There were no perioperative cerebral infarction and no hospital deaths. The mean operation time was 420 minutes. Compared to the conventional elephant trunk method, the FET method significantly reduced the operation time to 381 minutes.Bilateral axillary artery perfusion could contribute to reduce the cerebral infarction in TAR and facilitate the FET procedure.

Objective To visualize and quantify the atrioventricular conduction axis in the setting of ventricular septal defect using phase contrast computed tomography. Methods We used the SPring-8 synchrotron radiation facility in Hyogo prefecture in Japan, processing and reconstructing the data with 3-dimensional software. Results We studied 8 hearts obtained from patients known to have had ventricular septal defects, aged from 6 to 150 days, with a median of 24.5 days. None of the individuals, however, had undergone corrective surgery. The penetrating bundle was found at a median of 1.43 mm from the septal crest, with a range of 0.99 to 1.54 mm. The distance to the nonbranching bundle to the right ventricular endocardium was 1.10 mm, with a range from 0.49 to 2.49 mm, to the origin of the left bundle branch was 2.46 mm, with a range from 1.7 to 3.18 mm, and to the origin of the right bundle branch was 2.34 mm, with a range from 0.50 to 2.59 mm. The median distance from the edge of the caudal limb of the septomarginal trabeculation to the right bundle branch was 1.04 mm, with a range from 0.81 to 1.16 mm. Conclusions We were able to show the precise location of the axis, with our findings suggesting that longitudinal sutures placed along the posteroinferior rim should be effective in avoiding iatrogenic injury, but sutures should not be placed in the valley between the limbs of the septomarginal trabeculation.

We investigated the effect of ventricular septal defect (VSD) enlargement on long-term surgical results, late arrhythmia, and left ventricular (LV) function in the Rastelli procedure for D-transposition of the great arteries with LV outflow tract obstruction (LVOTO). From 1979 to 2001, 74 patients (D-transposition of the great arteries, n = 56; double outlet right ventricle, n = 18) underwent the Rastelli procedure. In group A, 46 patients underwent the Rastelli procedure with VSD enlargement, and in group B, 28 patients underwent the Rastelli procedure without enlargement. There were no hospital deaths. Actuarial survival at 20 years was 80% in group A and 91% in group B (P = 0.50). Freedom from reoperations at 20 years was 40.1% in group A and 52.0% in group B. Reoperations for LVOTO were performed in 2 patients in both groups. In postoperative catheterization, LV ejection fraction in group A was 57.1% ± 8.7% vs 57.2% ± 8.1% in group B (P = 0.97); LV end-diastolic volume, 150.0% ± 47.2% vs 142.0% ± 36.9% of the normal volume (P = 0.97). In long-term postoperative echocardiography, the pressure gradient of the LV to the aorta was 12.0 ± 12.8 vs 17.7 ± 26.0 mm Hg in groups A and B (P = 0.31). There were no differences between the groups regarding basal rhythms, anti-arrhythmic agents, and pacemaker implantation rate. VSD enlargement in the Rastelli procedure can be safely performed without early mortality and with long-term low mortality and morbidity regarding arrhythmia, LV function, and reoperation for late LVOTO. VSD enlargement should be considered as an option for avoiding long-term LVOTO.

OBJECTIVES To avoid cerebral infarction for aortic arch aneurysm and malperfusion for acute aortic dissection, the site of cannulation during total arch replacement remains important. Recently, we have used bilateral axillary artery perfusion in total arch replacement and in acute aortic dissection. Herein, we report the surgical outcomes. METHODS Seventy-eight patients with aortic arch aneurysm and 45 patients with acute aortic dissection were enrolled in this study. During surgery, translocation of the total arch was performed on 67 patients using a ‘frozen elephant trunk technique’. RESULTS In patients with aortic arch aneurysm, there was no postoperative cerebral infarction. New postoperative cerebral infarction was observed in only one patient who underwent acute aortic dissection. Two patients who had aortic arch aneurysm and 2 patients who had acute aortic dissection died at the hospital. Complications related to bilateral axillary perfusion were not observed. CONCLUSIONS The routine use of bilateral axillary artery perfusion in total arch replacement for aortic arch aneurysm to avoid cerebral infarction has the potential to be a useful procedure. It can facilitate the frozen elephant trunk procedure in acute aortic dissection.

BACKGROUND In patients with end-stage renal disease requiring hemodialysis (HD patients), myocardial ischemia after coronary artery disease is a major cause of mortality. Coronary pathophysiology, namely myocardial microvascular dysfunction, appears to differ from patients not requiring HD (non-HD patients). OBJECTIVES We compared functional improvement of myocardial ischemia after coronary artery bypass surgery (CABG) between HD and non-HD patients by transthoracic coronary flow velocity reserve (CFVR). METHODS We retrospectively reviewed isolated CABG patients from between 2008 and 2017. Finally, 161 patients were enrolled; each underwent pre- and postoperative CFVR assessment, and left anterior descending (LAD) artery revascularization with "in-situ" internal mammary artery (IMA). Graft patency was confirmed, and after successful CABG, postoperative CFVR improvement between the two groups was compared. RESULTS Preoperative CFVR value in group H was 1.81 ± 0.52, group N was 1.93 ± 0.66. There was no significant difference between the groups. IMA to LAD grafts were patent in postoperative evaluation in all patients. Postoperative CFVR in group H was 2.48 ± 0.72 and group N was 2.83 ± 0.73 (P = .042). Significant difference was observed. CONCLUSION In both groups, CFVR values improved after successful CABG, but postoperative CFVR values were significant different. In younger populations CFVR values are generally higher. Our HD group was significantly younger than the non-HD group, but CFVR values were postoperatively significantly lower. CFVR values are reportedly affected by both epicardial and microcoronary circulation. In this study population, as all grafts to the LAD were patent, the lower CFVR value in the HD group was considered to have resulted in microvascular disorders.

We evaluated long-term outcomes of the arterial switch operation (ASO) conversion after a failed Senning/Mustard operation among patients with dextro-transposition of the great arteries.Between 1986 and 2006, 9 patients with dextro-transposition of the great arteries underwent ASO conversion at our institute. All patients had systemic right ventricle failure, 6 had supraventricular tachycardia, and 8 had moderate or severe tricuspid valve regurgitation. All patients had New York Heart Association classification II or III. The median age of patients at the ASO conversion operation was 7.4 years (range, 0.6 to 32.4 years). Pulmonary artery banding for left ventricle training was performed in 8 of the 9 patients before conversion.There was 1 early death from low-output syndrome at 5 days postoperatively and 1 late sudden death at 5 months. Median follow-up time was 23.1 years (range, 0.08 to 28.0 years). The actuarial survival rate was 76.1% at 20 years. Long-term survivors revealed good New York Heart Association classification (class I, 6 patients; class II, 1 patient), with less than mild tricuspid regurgitation and brain natriuretic peptide levels of 40.6 ± 16.2 pg/mL. Cardiac catheterization revealed significant improvements of right ventricle end-diastolic volume (decreased from 243.2% to 117.7% of normal), and right ventricle ejection fraction (increased from 0.42 to 0.572; p0.05). Three patients underwent new pacemaker implantation for sick sinus syndrome, and moderate neoaortic valve regurgitation developed in 1 patient.Excellent long-term outcomes can be achieved after ASO conversion; however, careful observation for new-onset sick sinus syndrome and aortic regurgitation is mandatory.

Objectives This study aimed to analyze long-term survival and functional outcomes after truncus arteriosus repair in a single institution with more than 40 years of follow-up. Methods Medical records were analyzed retrospectively in 52 patients who underwent the Rastelli procedure for truncus arteriosus repair between 1974 and 2002. Thirty-five patients survived the initial repair. The median age at the initial operation was 2.8 months (range, 0.1–123 months) and the body weight was 3.9 kg (range, 1.6 to 15.0 kg). Results The median age at follow-up was 23.6 years (range, 12.4 to 44.5 years). The median follow-up duration was 23.4 years (range, 12.3 to 40.7 years). The actuarial survival rate was 97% at 10 years and 93% at both 20 years and 40 years after the initial operation. At follow-up, most patients were in New York Heart Association (NYHA) functional classes I (73%) and II (24%). Thirty-six percent of patients had full-time jobs, 40% were students, and 21% were unemployed. Most patients (97%) had undergone conduit reoperations. Freedom from reoperation for right ventricular (RV) outflow and pulmonary artery (PA) stenosis was 59% at 5 years, 28% at 10 years, and 3% at 20 years after the initial operation. Freedom from catheter interventions for RV outflow and PA stenosis was 59% at 5 years, 47% at 10 years, and 38% at 20 years after the initial operation. Freedom from truncal valve replacement was 88% at 5 years, 85% at 10 years, and 70% at 20 years after the initial operation. Conclusions In this single-center retrospective study, with long-term follow-up after repair of truncus arteriosus, long-term survival and functional outcomes were acceptable, despite the requirement for reoperation and multiple catheter interventions for RV outflow and PA stenosis in almost all patients, and the frequent requirement for late truncal valve operations.

The Ross operation is an excellent surgical option for young children, regardless of aortic pathology type. However, failure rates are concerning during the second postoperative decade. We sought to determine the predictors of long-term outcomes after Ross and Ross-Konno operation performed in childhood.We performed 34 Ross and 9 Ross-Konno operations in pediatric patients (age 15 years) from 1996 to 2016, and retrospectively evaluated the long-term results.The postoperative follow-up period was 13.5 ± 3.9 years. In the Ross group, there were one inpatient death and one death after discharge. There were no inpatient deaths in the Ross-Konno group. There were 6 reoperations in the Ross group and 1 in the Ross-Konno group for left ventricular outflow tract (LVOT). Cumulative survival rates were 96.8% and 100% in the Ross and Ross-Konno groups, respectively. The reoperation free rate for LVOT /RVOT (right ventricular outflow tract) were 98.6/85.5% and 91.9/63.4% in the 5th and 10th years of follow-up, respectively. Patients who underwent the operations at age 8.6 years had higher risks of reoperation for LVOT. Aortic annulus measurements 24 mm or aorta/pulmonary artery diameter (Ao/PA) ratios 1.2 conferred higher risks of reoperation for LVOT.Long-term outcomes after Ross and Ross-Konno operations in children were satisfactory. However, new-onset aortic regurgitation was progressive and reoperation was needed in some children. Age, aortic annulus diameter, and Ao/PA ratio may be able to predict of long-term outcomes after Ross and Ross-Konno operations.

There has been no report observing the LV function of Konno incision. We examined the outcomes of the Konno operations over 20 years. We investigated 63 Konno operations with mechanical valves performed from 1984. This study aimed to evaluate the effects of Konno incision including LV function changes and the long-term outcomes of patient survival and reoperation rate (RVOT and LVOT complications). The postoperative follow-up period was 20.6 ± 6.2 years. Early death occurred in 1 case and late death occurred in 9 cases. The cumulative survival rate was 89.6, 87.5, and 84.5% in the 5th, 10th, and 20th follow-up years. Five patients required reoperation for the aortic valve. The recurrence avoidance rate for LVOT reoperation was 96.6, 94.7, and 92% in the 5th, 10th, and 20th follow-up years of the Konno operation. Konno incision did not show significant decline in postoperative LV ejection fraction. (Pre, 62% vs 20 years later, 58%; P = 0.052.) There were no cases requiring intervention at the event of ventricular arrhythmia, but 13 cases were Complete Right bundle branch block, and 11 cases were Left bundle branch block. The survival rate associated with Konno operation throughout 20 years was excellent. Konno incision can secure sufficient LV outflow tract and is a safe treatment for complex LVOTO without deteriorating LV ejection fraction.

OBJECTIVES Isolated total anomalous pulmonary venous connection (TAPVC) is a relatively rare congenital cardiac defect, while pulmonary venous obstruction (PVO) is associated with poor prognosis. We reviewed the long-term outcome of total correction for isolated TAPVC at our institute and analysed the risk factors for mortality and morbidity. METHODS A total of 290 isolated TAPVC patients evaluated between 1965 and 2016 were divided into 2 groups: Group Early (n = 151) underwent surgery before 1989; Group Late (n = 139) underwent surgery after 1990. The mean age at operation was 10.4 ± 30.2 months (range 0 day to 23 years), and the mean body weight was 5.5 ± 6.0 kg (range 1.6-48 kg). Group Late included more patients with the infracardiac type of TAPVC and preferably used the posterior approach. RESULTS There were 53 hospital deaths and 16 late deaths. Postoperative PVO was recognized in 28 patients. The mean follow-up time was 18.2 ± 9.7 years (range 2 months to 42.4 years). The actuarial survival rate was improved to 87.8% at 20 years in Group Late. Multivariable analysis revealed that death rate was significantly increased in patients of Group Early, with a body weight

A 3-month-old female with ventricular septal defect underwent definitive repair. In the operation, a venting tube was inserted through the incised patent foramen ovale. The ventricular septal defect was closed using a patch and the incised patent foramen ovale was directly closed. A transesophageal echography showed no abnormal findings in the operating room. On 1st postoperative day, the patient's heart rate and pressure of the superior vena cava increased while blood pressure decreased. A transthoracic echocardiography revealed an atrial septal hematoma. After conservative therapy, the heart rate and pressure of the superior vena cava gradually decreased while blood pressure increased. The atrial septal hematoma disappeared spontaneously. The patient was discharged on 16th postoperative day. The atrial septal hematoma appears to have a good prognosis, but sometimes can be poor resulting and death. Meticulous and constant observation is required to detect an atrial septal hematoma.

Background The Fontan procedure is rarely performed in adults and adolescents in the present era. We review our results with the Fontan procedure in adolescents and young adults. Methods Between 1974 and 2010, 79 consecutive patients underwent the Fontan procedure at an age ≥ 15 years (mean age at Fontan operation, 20.3 years ± 4.5 years). Forty-five patients underwent atriopulmonary connection, 11 underwent the Bjork procedure, and 23 underwent total cavopulmonary connection. Results Ten hospital deaths (HDs) and/or early Fontan takedowns (TDs) occurred. The median follow-up period was 18.2 years (range, 0.6-37.6 years). The estimated freedom from death or TD rates was 79.7% at 5 years, 77.0% at 10 years, 73.9% at 15 years, and 63.9% at 20 years. Age was not a predictor of HD and/or TD. Freedom from death or TD after 1998 was 69.1% at 5 years, 69.1% at 10 years, and 69.1% at 15 years, and before 1997 was 82.3% at 5 years, 79.0% at 10 years, 75.5% at 15 years, and 65.1% at 20 years; there were no significant differences between the two groups. In 19 late-death patients, nine (47.4%) experienced sudden death. Among these patients, five had known arrhythmias before sudden death. Conclusions In patients who were ≥15 years old, the surgical results of the Fontan operation were acceptable. Approximately half of the late deaths were sudden deaths, mainly occurring 10-20 years postoperatively.

The unilateral absence of a proximal pulmonary artery (UAPPA) is rare and is most frequently accompanied by cardiovascular anomalies such as tetralogy of Fallot or septal defects. We report a patient with truncus arteriosus with UAPPA in which we performed a two-stage surgical repair. During the first palliative operation, a right modified Blalock-Taussig shunt was constructed to develop the hypoplastic right pulmonary artery. At 10 months, the patient underwent patch closure of a ventricular septal defect with integration of both pulmonary arteries, and reconstruction of the right ventricular outflow tract using a conduit. Four years postoperatively, he continues to do well.

Background This study aimed to evaluate Fontan circulation which was observed over 30 years. Methods Forty patients who underwent a Fontan operation between 1974 and 1986 and survived (group S, n = 20) or died in the late period (group LD, n = 20) were evaluated. The median age at operation was 10 years (range 2–32 years). The diagnoses were tricuspid atresia in 21, single ventricle in 9, and others in 10. The Fontan procedure was a right atrium-pulmonary artery graft in 2, atriopulmonary connection in 28, and right atrium-right ventricle anastomosis in 10 (Björk in 4, graft in 6). Results Causes of late death were congestive heart failure in 6, sudden death in 4, arrhythmia in 4, and others in 6. Sixteen patients underwent reoperation 23.3 ± 6.5 years after Fontan. Cardiac catheterization was performed at 1 month and 12 years. There were no significant differences in central venous pressure, ventricular ejection fraction, or pulmonary vascular resistance between the two groups. However, changes in ventricular end-diastolic volume suggested volume and pressure overload in group LD. In group S, the latest chest radiographs showed cardiothoracic ratio 51.3% ± 7.6%, oxygen saturation 94% ± 2%, brain natriuretic peptide 153 ± 111 pg ċ mL−1, and Holter monitoring revealed sinus rhythm in 10 patients. New York Heart Association class was I in 12 patients, II in 6, and III in 2. Conclusions For long-term Fontan survival, timely total cavopulmonary connection conversion and medication to decrease ventricular volume and pressure load might be important.

This study aimed to analyze long-term survival and functional outcomes after truncus arteriosus repair in a single institution with more than 40 years of follow-up.Medical records were analyzed retrospectively in 52 patients who underwent the Rastelli procedure for truncus arteriosus repair between 1974 and 2002. Thirty-five patients survived the initial repair. The median age at the initial operation was 2.8 months (range, 0.1-123 months) and the body weight was 3.9 kg (range, 1.6 to 15.0 kg).The median age at follow-up was 23.6 years (range, 12.4 to 44.5 years). The median follow-up duration was 23.4 years (range, 12.3 to 40.7 years). The actuarial survival rate was 97% at 10 years and 93% at both 20 years and 40 years after the initial operation. At follow-up, most patients were in New York Heart Association (NYHA) functional classes I (73%) and II (24%). Thirty-six percent of patients had full-time jobs, 40% were students, and 21% were unemployed. Most patients (97%) had undergone conduit reoperations. Freedom from reoperation for right ventricular (RV) outflow and pulmonary artery (PA) stenosis was 59% at 5 years, 28% at 10 years, and 3% at 20 years after the initial operation. Freedom from catheter interventions for RV outflow and PA stenosis was 59% at 5 years, 47% at 10 years, and 38% at 20 years after the initial operation. Freedom from truncal valve replacement was 88% at 5 years, 85% at 10 years, and 70% at 20 years after the initial operation.In this single-center retrospective study, with long-term follow-up after repair of truncus arteriosus, long-term survival and functional outcomes were acceptable, despite the requirement for reoperation and multiple catheter interventions for RV outflow and PA stenosis in almost all patients, and the frequent requirement for late truncal valve operations.

In this study, we perform a detailed analysis of the microglial and macrophage responses in a model of spinal cord ischemia and reperfusion (SCI/R) injury in Wistar rats. The rats underwent occlusion across the descending aorta for 13min, causing paraplegia or paresis of varying severity. They were divided into four groups based on neurological assessment: sham, mild paresis, moderate paresis, and severe (complete) paraplegia. To examine the origin of microglia and macrophages in the ischemic lesion, bone marrow from rats expressing green fluorescent protein (GFP) was transplanted into test subjects one month before performing SCI/R. Many GFP(+)/CD68(+) microglia and macrophages were present 7d after SCI/R. Resident (GFP(-)/Iba1(+)/CD68(-)) microglia and bone marrow-derived macrophages (BMDMs; GFP(+)/Iba1(+)/CD68(+)) colocalized in the mild group 7d after SCI/R. In the moderate group, BMDMs outnumbered resident microglia. A greater accumulation of BMDMs expressing insulin-like growth factor-1 (IGF-1) was observed in lesions in the severe group, relative to the moderate group. BMDMs in the severe group strongly expressed tumor necrosis factor α, interleukin-1β, and inducible nitric oxide synthase, in addition to IGF-1. A robust accumulation of BMDMs occupying the entire ischemic gray matter was observed only in the severe group. These results demonstrate that the magnitude of the microglial and BMDM responses varies considerably, and that it correlates with the severity of the neurological dysfunction. Remarkably, BMDMs appear to have a beneficial effect on the spinal cord in paresis. In contrast, BMDMs seem to exhibit both beneficial and harmful effects in severe paraplegia.

Congenital coronary artery fistula (CAF) is a rare anomaly involving communication between the coronary artery and any cardiac chamber. We retrospectively studied 23 patients with CAF who were surgically treated at 2 institutes over the past 38 years. All patients had continuous murmur and were diagnosed using echocardiography and cardiac catheterization. Eighteen patients had no other heart anomalies, and 5 had an associated anomaly. Fifteen patients were treated using cardiopulmonary bypass via differential surgical approaches (6 patients, CAF orifice closure through cardiac chamber; 6, coronary arteriotomy for CAF orifice closure; and 3, CAF ligation). Eight patients underwent CAF ligation without cardiopulmonary bypass. CAF originated from the right coronary artery in 11 patients; the left coronary artery in 10, and both arteries in 2. Drainage occurred at the following sites: the right ventricle (10 patients), right atrium (6), left ventricle (4), left atrium (2), and pulmonary artery (1). All surgeries were performed through a median sternotomy. There were no mortalities during or after the hospital stay. Aortic root replacement was performed in 1 patient 30 years after the CAF surgery. Three of the 6 patients who underwent coronary arteriotomy for CAF orifice closure showed coronary artery occlusion at the distal coronary arteriotomy site with long-term collateral formation. Surgical management of CAF was thus effective, resulting in 100% long-term survival and closure rates. Dilated CAF-associated vessels have been normalized for the long term. CAF should be therefore considered even in asymptomatic patients because of the risk of future complications.

Open heart surgery for infants with low body weight (BW) remains still a challenge. Pulmonary artery banding (PAB) is a useful surgical palliation for small neonates and early infants with excessive pulmonary blood flow who are unable to withstand a heart surgery. This study retrospectively reviewed neonates and infants who underwent PAB to assess the surgical results and the validity of our PAB. We selected 38 acyanotic infants and neonates and divided them into 2 groups: low BW (2.5 kg, n = 15, group L) and normal or high BW (≥ 2.5 kg, n = 23, group NH). The average BW at the time of PAB was 2.8 ± 1.1 kg (range, 1.2-5.8 kg), and the average age at the time of PAB was 41.8 ± 44.8 days (range, 2-151 days). Using a 3-mm-wide polyester tape, we tightened the main pulmonary artery to obtain the circumference of (19 mm + 1 mm for each kg of BW). There was no early death but one late death in each group. Postoperative BW continuously increased 1 month after PAB in both groups, although BW was significantly lower in group L than in group NH. Intracardiac repair (ICR) was accomplished in 31 patients (13 in group L and 18 in group NH) at average ages of 1.5 years, while the remaining 5 patients are awaiting ICR. In conclusion, PAB using our formula for the infants even weighing2.5 kg has low mortality and is effective as a bridge to ICR.

Here we report a neonatal case of total anomalous pulmonary venous connection with left lung agenesis. Diagnostic imaging demonstrated that the left pulmonary veins were totally absent and the right pulmonary veins connected with the common pulmonary chamber. Drainage from the common pulmonary venous chamber entered the persistent left suerior vena cava. In addition, it revealed complete absence of the left main bronchus and left lung vessels. The neonate successfully underwent surgical repair 18 days after birth.

Anomalous subaortic left brachiocephalic vein (ASLBV) is a rare systemic venous anomaly. We review our experience with patients associated with ASLBV who underwent cardiac surgery at three institutions. From 1989 to 2009, the medical records of surgically treated patients with ASLBV were analyzed; the incidence of ASLBV, clinical characteristics, and associated anatomical findings were assessed. Fifteen patients had ASLBV. All ASLBVs coursed left lateral to the aortic arch, passed under the ascending aorta anterior to the central pulmonary artery, and joined the right brachiocephalic vein. Fourteen patients had congenital heart disease (CHD), and the remaining patient did not have cardiac anomalies. Its incidence was 0.57% (14 of 2,449) in patients with CHD and only 0.02% (1 of 4,805) in patients without CHD. In patients with CHD, 73.3% (11 of 15) of the patients had conotruncal cardiac anomalies such as tetralogy of Fallot, ventricular septal defect with pulmonary atresia, truncus arteriosus, and interruption of the aortic arch. Eight patients had aortic arch anomalies, including right aortic arch and cervical aortic arch. The deletion of chromosomal 22q11.2 was confirmed in two patients, and one patient was diagnosed with DiGeorge syndrome. ASLBV was clinically silent even without any surgical intervention. ASLBV is a very rare anomaly and is highly associated with conotruncal cardiac anomalies and aortic arch anomalies, including right aortic arch and cervical aortic arch. Preoperative diagnosis is important when any surgical interventions are intended, especially, in patients with conotruncal cardiac anomalies.

SummaryIn the neonatal period, the surgical mortality of palliation is extremely high for asplenia syndrome complicated by single ventricle combined with total anomalous pulmonary venous connection (TAPVC). Recently, stent implantation for the pulmonary venous drainage route soon after birth has been used instead of surgery to prevent pulmonary venous occlusion and to maintain stable hemodynamics in the neonatal period or in early infancy. Here, we successfully implanted stents in the ductus venosus (DV) in 2 neonates with asplenia syndrome complicated by infracardiac type TAPVC. The first patient was a 3-day-old male neonate with severe cyanosis. Immediately after TAPVC was diagnosed, we implanted a stent in the DV. The second patient was a 0-day-old female neonate. She was diagnosed as TAPVC by fetal echocardiogram. After the scheduled delivery, a stent was successfully implanted. We believe that stent implantation in the DV in the neonatal period is effective and less invasive than surgery in patients with infracardiac type TAPVC.

Anatomically corrected malposition of the great arteries (ACMGA) is rare congenital cardiac anomaly. In a heart with ACMGA, both great arteries arise from the appropriate ventricle but they exit parallel to each other. The pulmonary trunk is located posterior and to the right of the aorta. ACMGA associated with tubular hypoplasia of the aortic arch is an extremely rare cardiac malformation and it has previously been reported in only four patients. In addition, successful surgical repair for this combination of anomalies was achieved in only one patient. We describe clinical and morphologic features in a neonate with this complex anomaly. The patient successfully underwent the staged operation. (J Card Surg 2010;25:412-415)

Background Rastelli and reparation a l'etage ventriculaire (REV) procedures have been applied for biventricular repair of transposition of great arteries with ventricular septal defects and pulmonary stenosis (TGA III). In some patients with TGA III, whose tricuspid valve (TV) chordae tendineae are attached to the infundibular septum, abnormal TV chorda insertion may cause intracardiac rerouting obstruction. For such cases, we performed a conal flap" (CF) procedure involving mobilization of the infundibular septum including TV chordae as a pedicled flap. This study evaluated the long-term outcomes of this CF method. Methods Between October 1985 and December 1999, 17 consecutive patients with TGA III underwent a Rastelli-type or an REV-type procedure using CF. Median age at operation was 4.8 years (range, 1.5 to 14.0 years). Results The mean follow-up period was 17.8 ± 7.2 years. One hospital death and one late death occurred. The freedom from death rates were 94.1%, 94.1%, and 85.6% at 5, 10, and 15 years, respectively. The freedom from death or reoperation rates were 94.1%, 55.8%, and 46.5% at 5, 10, and 15 years, respectively. No reoperations were performed for the left ventricular outflow tract (LVOT) or TV. The average LVOT maximum flow velocity was 1.26 ± 0.50 m/s. TV regurgitation was greater than mild in 3 patients (20%) and trivial to mild in 12 patients (80%). Thirteen patients were in New York Heart Association (NYHA) functional class I, and 2 patients were in NYHA functional class II. Conclusions The long-term outcomes, including the daily life activities, LVOT pressure gradients, and TV regurgitation grades, were excellent in patients who underwent Rastelli-type and REV-type procedures using CF for TGA III.

Background The arterial switch operation (ASO) for the transposition of the great arteries (TGA) with intramural coronary arteries has been performed using several techniques to avoid coronary events. We mainly performed ASO without coronary relocation by creating an aortopulmonary fenestration (Imai technique). Coronary circulation was rerouted by covering the aortopulmonary window and coronary orifices with a nonfacing sinus flap. Long-term results have not been reported. We describe our early and late results. Methods Among 551 patients who underwent an ASO between 1985 and 2014, intramural coronary arteries were detected in 15 of them. Coronary arteries were managed using 2 techniques: the double-button method in 5 patients (with unroofing and trapdoor incision in 1 patient) and the Imai technique in 10 patients. Results There were 3 hospital deaths and 3 deaths after discharge, 5 of which showed coronary complications. Actual survival and freedom from coronary complications at 15 years were 70% and 67%, respectively, with the Imai technique and 40% and 20%, respectively, with the double-button method. Late coronary intervention was performed for a long intramural coronary artery stenosis in 1 patient who underwent the Imai technique. In the others, late aortography showed good patency of the aortopulmonary window and growth of the coronary pouch after the Imai technique. Conclusions The Imai technique can be an option for coronary management in the presence of high-risk coronary anatomy, particularly distal intramural coronary artery stenosis and inseparable coronary arteries with an almost single orifice. Adequate neopulmonary artery augmentation must be performed to prevent right ventricular outflow stenosis.

We treated a 6-year-old boy who had polysplenia syndrome and tetralogy of Fallot with a small right ventricle (RV), an atrial septal defect, a hemiazygos connection, and bilateral superior vena cava. Because the RV was too small for a biventricular repair to be performed, the patient underwent a total cavopulmonary shunt operation although his heart was biventricular and a pacemaker (VVI) had been implanted for management of the sick sinus syndrome complicated by polysplenia syndrome. After the operation, marked asynchronous contraction was noted between the morphological right and left ventricles and was probably responsible for the low cardiac output noted in this patient. In order to clarify the significance of the asynchronous contraction, we determined the cause of the low cardiac output by studying the time course of the volume changes in the morphological right and left ventricles during a cardiac cycle by using angiograms. In addition, we studied the interventricular flow dynamics by using pulsed-Doppler echocardiography. After a Fontan-type operation is performed on patients with a biventricular heart, the 2 ventricles may not function in perfect coordination when they have to work as 1 unit. These patients are likely to develop cardiac dysfunction due to interventricular to-and-fro flow dynamics. Asynchronous contraction between the 2 ventricles caused by abnormal interventricular conduction impaired the cardiac performance in the present case.

We provided emergency treatment to a 1-day-old neonate (1600 g) with tracheoesophageal fistula (gross classification, type C) and total anomalous pulmonary venous drainage (infracardiac type) complicated by pulmonary venous obstruction. Emergency surgery was required because the tracheoesophageal fistula would have caused respiratory failure. Here we report the perioperative management techniques we used, including the surgical strategy.

We report a surgical case of dextrocardia complicated with annuloaortic ectasia (AAE) and mitral regurgitation, which induced congestive heart failure. Preoperative electrocardiography-gated multidetector-row computed tomography (MDCT) showed the following complex cardiovascular abnormalities without motion artifacts: dextrocardia, situs inversus, polysplenia, AAE, absence of the inferior vena cava, azygos vein continuation, drainage of the hepatic vein into the right atrium, and bilateral superior venae cavae. On the basis of the MDCT data, we established a cardiopulmonary bypass; and a modified Bentall procedure (Piehler method) and mitral valve replacement were performed without complications.

We provided a left ventricular assist device (LVAD) for a 22-year-old man with congenital L-transposition of the great arteries after anatomic repair at the age of 7 years. He was hospitalized for progressive low-output syndrome caused by intractable biventricular failure. He received LVAD in his morphologic left ventricle with a concomitant pulmonary valve replacement. After the surgery, critical multiorgan failure with severe right heart failure occurred. It took three postoperative months to normalize all organ function following improvement of morphologic right ventricular function. He has remained stable with LVAD support for 1.5 years.

The cellular mechanisms of abnormal calcium regulation and excitation-contraction coupling in relation to glucose metabolism in the hypertrophied heart are not well understood. The present study evaluated the myocardial mechanics of 6-7-week-old pressure overload hypertrophied rabbit hearts in response to dobutamine by (1) serial echocardiograms in vivo and (2) isolated Langendorff perfusion. Cytosolic Ca2+([Ca2+]i) and sarcoplasmic reticulum Ca2+-ATPase (SERCA2) expression were measured by fluorescence spectroscopy and Western immunoblotting, respectively. The effect of glycolytic inhibition by 2-deoxy-D-glucose +/- pyruvate was also evaluated. Both systolic and diastolic [Ca2+]i tended to be higher and diastolic calcium removal (tauCa) significantly slower in the hypertrophied heart. The myocardial response to dobutamine was blunted and dobutamine insignificantly improved tauCa. The SERCA2 protein level was higher in early hypertrophy, but was significantly reduced by 6 weeks of age, with progressive contractile failure. Inhibition of glycolysis or SERCA2 caused an increase in [Ca2+]i as well as a slower tauCa. Pyruvate completely preserved myocardial function and [Ca2+]i handling during glycolytic inhibition. It was concluded that in this model of advanced pressure overload hypertrophy, contractile failure and inotrope insensitivity are associated with increased [Ca2+]i, slower tauCa and reduced sensitivity of the contractile proteins to Ca2+. These changes occur in association with downregulation of the SERCA2, probably caused by impaired glucose metabolism.

The aortopulmonary window concomitant with an interrupted aortic arch is a rare occurrence. We successfully performed an emergency one-stage surgical repair of the aortopulmonary window (type 1) concomitant with an interrupted aortic arch (type A) in the case of a very low-birth-weight (1230-g) premature 2-day-old neonate. We describe the diagnosis, surgery, and postoperative course of this rare occurrence.

Isolated congenital tricuspid regurgitation without downward displacement of the leaflet is uncommon in adults, and repair of such valves often requires a procedure that is more complex than simple tricuspid annuloplasty. We describe a technique of tricuspid valvoplasty using the neo-papillary loop technique to reconstruct multiple artificial chords for isolated congenital tricuspid regurgitation associated with a dysplastic anterior leaflet with agenesis of the chordae tendineae and a hypoplastic anterior papillary muscle. This technique provides a simple and valuable option for the repair of dysplastic valves lacking chordae, even those with a hypoplastic papillary muscle.

The vascular wall of patent ductus arteriosus in adults is usually fragile due to atherosclerosis with calcification. In adults, surgical treatment, such as direct or patch closure, is sometimes required for a wide, short, or calcified patent ductus arteriosus. We present a novel technique for patent ductus arteriosus closure with a pedicled patch created from the wall of a dilated pulmonary artery. We believe that this technique, in which only an autologous tissue is used, is safe and has excellent durability.

Andersen-Tawil syndrome is an uncommon inherited autosomal disorder characterized by a prolonged QT interval, periodic paralysis, and dysmorphic features. The deleterious effects of cardioplegia on periodic paralysis and cardiac arrhythmia are unknown, and no studies have reported the performance of cardiac surgery in patients with Andersen-Tawil syndrome. We present a case of successful cardiac surgery in a patient with Andersen-Tawil syndrome, without using cardioplegia.

Occlusion of the right coronary artery (RCA) ostium by an aortic cusp is a rare anomaly and can be a cause of sudden death. We report the case of a child with progressive stenosis of the right coronary ostium caused by a hypoplastic right coronary cusp that adhered to the aortic wall resulting in severe myocardial ischemia. The patient underwent a Ross-Konno operation with mitral valvuloplasty for congenital aortic valvular stenosis and mitral regurgitation caused by ischemia-induced posteromedial papillary muscle dysfunction. The myocardial ischemia was relieved by resection of the deformed pouch-like cusp to disclose the RCA ostium. Postoperative myocardial scintigraphy demonstrated no myocardial ischemia and multidetector computed tomography showed no coronary ostial stenosis.

A two-stage surgical repair of a one-month-old infant with truncus arteriosus with unilateral absence of a proximal pulmonary artery (PA) with a closed ipsilateral ductus arteriosus was successfully performed. In the first palliation, translocation of the discontinuous, closed, and undeveloped PA to the adjacent area of the other PA was useful for making a pulmonary arterial confluence at timing of the subsequent radical operation.(J Card Surg 2010;25:90-92)

Objectives: It is controversial whether immature cyanotic hearts are more susceptible to ischemic injury than normoxemic hearts. Acutely induced alveolar hypoxic stress before cardiopulmonary bypass has been used as a model of cyanosis and is reported to worsen recovery of immature hearts after subsequent ischemic insult by means of a free radical injury mechanism. Because of concerns about the relevance of acute alveolar repair to the chronic cyanosis encountered clinically, we assessed the effects of chronic cyanosis without alveolar hypoxia, acute alveolar hypoxia, and normoxemia on recovery of cardiac function after deep hypothermic circulatory arrest. Methods: A chronic cyanosis model was created in 8 lambs by an anastomosis between the pulmonary artery and the left atrium (cyanosis group). Eight lambs underwent sham operation (control). One week later, the animals underwent cardiopulmonary bypass with 90 minutes of deep hypothermic circulatory arrest at 18°C. Another 8 lambs underwent 45 minutes of hypoxic ventilation before bypass, with arterial oxygen tension being maintained at 30 mm Hg (acute hypoxia group). Cardiac index, preload recruitable stroke work, and tau were measured. Malondialdehyde and nitrate-nitrite, nitric oxide metabolites, were also measured in the coronary sinus. Myocardial antioxidant reserve capacity at 2 hours of reperfusion was assessed by measuring lipid peroxidation in left ventricular tissue samples incubated with t-butylhydroperoxide at 37°C. Results: Oxygen tension was 35 ± 3 mm Hg in the acute hypoxia group versus 93 ± 7 mm Hg in the control group. In the acute hypoxia group the recovery of cardiac index, preload recruitable stroke work, and tau were significantly worse than that found in both the control and cyanosis groups. Preload recruitable stroke work at 2 hours of reperfusion was slightly but significantly lower in the cyanosis group than in the control group. The postischemic level of nitric oxide metabolites was significantly lower in the acute hypoxia group than in the cyanosis and control groups. However, malondialdehyde levels in the coronary sinus and myocardial antioxidant reserve capacity were not significantly different among the groups. Conclusion: Recovery of left ventricular function after deep hypothermic circulatory arrest in neonatal lambs with chronic cyanosis was slightly worse than that found in acyanotic animals. Acute hypoxia before bypass was associated with significantly worse recovery of left ventricular function, and the mechanism of injury may be related to an impairment of nitric oxide production. Free radical injury does not appear to explain any differences among cyanotic, acyanotic, and acutely hypoxic animals in recovery of left ventricular function after ischemia. (J Thorac Cardiovasc Surg 2000;120:238-46)