Long-term outcomes of hypoplastic left heart syndrome with analysis of the Norwood procedure in infants following bilateral pulmonary artery banding.

Objective: To investigate the early and long-term outcomes of the deferred Norwood procedure by bilateral pulmonary artery banding (BPAB) versus the neonatal Norwood procedure. Methods: This retrospective study examined 46 patients with hypoplastic left heart syndrome and its variants undergoing the Norwood procedure for single ventricle physiology between 2004 and 2022 at 3 institutions. The patients were divided into 2 groups: neonatal Norwood procedure (group N; n = 23) and staged Norwood procedure in infants following BPAB (group I; n = 23). Preoperative risk factors, surgical results, survival rates, Fontan candidacy, and long-term complications were compared. Results: Early survival rates after the Norwood procedure were 91.3% (21 of 23) in both groups. Late survival rates after the Norwood procedure were similar at the 10-year follow-up (group N, 76.3%; group I, 68.7%; P = .63). Fontan completion rates also were comparable in the 2 groups (group N, 77.8%; group I, 85.7%; P = .67). Group N showed a higher median pulmonary artery (PA) index before bidirectional cavopulmonary connection (group N, 177 [interquartile range (IQR), 147-243] mm2/m2; group I, 152 [IQR, 146-163] mm2/m2; P = .03); this trend continued until 5 years after Fontan completion (P = .01). Group N also had a lower rate of freedom from protein-losing enteropathy (PLE) at 9.0 years after the Fontan operation (90.0% vs 52.5% for group I; P = .04), although the incidences of other Fontan-associated events were not significantly different. Conclusions: Fontan candidacy and survival rates were similar regardless of the timing of the Norwood procedure. Early performance of the Norwood procedure may lead to lower rates of late Fontan-associated events, such as PLE.

[Surgical Treatment of the Transposition of the Great Arteries].

Transposition of the great arteries( TGA) is a congenital heart disease in which the aorta originates from the right ventricle and the pulmonary artery from the left ventricle. In embryonic development, normally, the aorticopulmonary septum develops a spiral-like configuration resulting in the pulmonary trunk twisting around the ascending aorta. However, in TGA, there is lack of spiral rotation of the aorticopulmonary septum. TypeⅠ( 50%) without ventricular septal defect( VSD), typeⅡ( 25%) with VSD and left ventricular outflow tract stenosis (LVOTS), typeⅢ (25%) with VSD and LVOTS, and typeⅣ (very rare) with VSD but no LVOTS. All types present with cyanosis from the time of birth. Of these, typeⅠ is the most cyanotic, as systemic oxygen supply depends on the flow through the foramen ovale. Since the theme of this article is emergency surgery, we will discuss surgical treatment for typeⅠ, for which surgery is indicated in the early neonatal period.

Coarctation of the aorta with total anomalous pulmonary venous connection: a case report.

Total anomalous pulmonary venous connection (TAPVC) and coarctation of the aorta (CoA) rarely occur together. In affected patients, blood is supplied to the lower body by saturated ductal flow. Preoperative echocardiography may not show an acceleration of flow at the isthmus (coarctation), and the oxygen saturation (SpO2) at the feet may be satisfactory. Consequently, the severity of CoA is often underestimated before performing surgery. A 6-day-old boy weighing 2.6 kg with a diagnosis of supracardiac TAPVC was referred for surgical correction of his anomaly. The atrial septal defect (ASD) was 6.7 mm in diameter. There was a large patent ductus arteriosus (PDA) without flow acceleration at the preductal entry into the descending aorta. Only the TAPVC repair was planned, but immediately following ligation of the large PDA, the blood pressure in the lower extremity dropped to around 30 mmHg. The ligation was removed. The reason for the blood pressure discrepancy between the upper and the lower body was not clear as there was no arterial line in the upper extremity and a 6.7-mm-diameter ASD can support sufficient blood flow to the lower body without the PDA. A suspected CoA was found and repaired, followed by the TAPVC repair. Caution is necessary when repairing a TAPVC and coexisting large PDA as the severity of the CoA can easily be underestimated due to nonsignificant flow acceleration.

Risk Factors for Mid-Term Liver Disease After the Fontan Procedure.

The Fontan procedure is a palliative surgery performed for patients with complex congenital heart disease who exhibit functional single ventricular physiology. Although clinical outcomes of the Fontan procedure have improved in recent years and most patients who undergo the procedure reach adulthood, Fontan-associated liver disease (FALD) is a noncardiovascular complication that has become increasingly common; its risk factors remain unknown.A total of 95 patients who underwent the Fontan procedure and who were followed up for at least three years at Gunma Children's Medical Center and Kitasato University Hospital between 1996 and 2015 were retrospectively enrolled in this study.The mean age of the patients at the time of Fontan procedure was 2.3 ± 1.4 years. Overall, 21 patients (23.1%) experienced FALD. All Fontan procedures were performed with extracardiac total cavopulmonary connection using 16-mm expanded polytetrafluoroethylene grafts. The presence of systemic right ventricle, requirement of pulmonary vasodilator, application of a non-fenestrated Fontan procedure, and absence of fenestration flow at the time of follow-up catheter examination were identified as predictors of FALD using univariate analysis. All these factors, except the requirement of pulmonary vasodilator, remained significant predictors of FALD in multivariate logistic regression analysis.Patients with a systemic right ventricle who undergo the Fontan procedure are at a high risk of FALD in the mid-term. Creating fenestration at the time of Fontan and maintaining the fenestration flow may reduce the mid-term risk of FALD.

[Effectiveness of Pulmonary Valve Replacement for the Patients with Pulmonary Valve Regurgitation after the Repair of Congenital Heart Diseases].

Pulmonary regurgitation(PR) and right ventricular dysfunction are the major complications in a longterm period after the repair of congenital heart diseases including tetralogy of Fallot(TOF). The purpose of this study is to investigate the effectiveness of pulmonary valve replacement (PVR) after the congenital heart repair. Twenty-five patients undergoing PVR for PR with clinical symptoms in a longterm period between March 2008 and December 2016 were retrospectively reviewed. The average age at the TOF repair was 2.6±2.6 years. The average age at the PVR was 21.6±11.2 years. The mean follow-up after the PVR was 52.8±27.5 months. There was neither hospital death nor late death. All the patients underwent PVR with a bio-prosthetic valve. Nine patients had concomitant tricuspid valve repair. PVR significantly reduced right ventricular end-diastolic volume( RVEDVI)[ 123±25 vs 101± 13% of normal, p=0.002], cardio-thoracic ratio (CTR) [56.0±6.9 vs 52.6±13.0%, p=0.03], and brain natriuretic peptide (BNP) [57.6±57.0 vs 38.7±31.3 pg/dl, p=0.03]. The relatively early PVR significantly improved RVEDVI, CTR and cardiac function.

Novel surgical strategy for complicated pulmonary stenosis using haemodynamic analysis based on a virtual operation with numerical flow analysis.

OBJECTIVES: A novel surgical strategy using haemodynamic analyses based on virtual operations with computational simulations has been introduced for complicated pulmonary stenosis. We evaluated the efficacy of this strategy. METHODS: Six patients were enrolled. Before surgery, the optimal pulmonary arteries were constructed based on computational fluid dynamics using 3-dimensional computed tomography. Energy loss (EL, mW) and wall shear stress (WSS, Pa) were calculated. We compared the shapes of preoperative and optimal pulmonary arteries to determine the surgical strategy, including the incision line and the shape of the patch (virtual surgery). EL and WSS were compared between virtual and actual surgeries using flow analysis. RESULTS: In both the virtual and actual surgeries, postoperative EL tended to be lower than the preoperative EL, although there were no significant differences (P = 0.12 and P = 0.17, respectively). The mean WSS in the virtual surgery was significantly reduced from 112 ± 130 Pa to 25 ± 24 Pa (P = 0.028). After the actual surgery, the mean WSS was also significantly reduced to 30 ± 23 Pa (P = 0.047). There were no significant differences in the values for EL and WSS before and after surgery or between virtual and actual surgery (P = 0.94 and P = 0.85, respectively). CONCLUSIONS: Pulmonary artery plasty, using computational fluid dynamics based on virtual surgery, is an efficient surgical strategy. This novel strategy can easily and successfully provide an optimal pulmonary artery plasty equivalent to that using the conventional approach, which is based on the surgeon's personal experience and judgement.

Conventional repair of total anomalous venous drainage without primary sutureless technique: surgical tips to prevent pulmonary vein obstruction.

OBJECTIVES: Although primary sutureless technique for total anomalous pulmonary venous drainage has been introduced to reduce postoperative pulmonary vein obstruction (PVO), controversy still exists about superiority of the procedure between the conventional repair and primary sutureless technique at the initial repair. In our unit, the conventional repair has been consistently used based on four important surgical policies: (1) mark incision lines between 2 chambers to gain anatomically natural alignment, (2) place precise stitches by "intima-to-intima" using monofilament suture, (3) adequate orifice size should be guaranteed in greater than expected mitral valve size, (4) do not hesitate to undertake a redo additional anastomosis by a different approach when an echocardiography shows the velocity more than 1.5 m/s. This study aims to evaluate mid-term outcome of the conventional repair for total anomalous pulmonary venous drainage. METHODS: Between 2004 and 2016, consecutive 15 patients who underwent the conventional repair without the primary sutureless technique were included in this study. Survival, Freedom from reoperation, and PVO were retrospectively reviewed. RESULTS: Mean follow-up period was 4.6 ± 3.7 years. Except for one patient who died of uncontrollable pleural effusion, all other patients survived with 5-year survival rate of 93.3%. For the 14 survivors, there was no PVO, nor reoperation. CONCLUSIONS: Following these policies, the mid-term outcome of the conventional total anomalous pulmonary venous drainage repair was excellent without the primary sutureless technique showing no obstruction. The conventional repair can be safely applied at the initial operation when the morphological condition allows for it.

Early Extubation in the Operating Room after Congenital Open-Heart Surgery.

Early extubation in the operating room after congenital open-heart surgery is feasible, but extubation in the intensive care unit after the operation remains common practice at many institutions. The purpose of this study was to evaluate retrospectively the adequacy of our early-extubation strategy and exclusion criteria through analysis based on the Risk Adjustment in Congenital Heart Surgery method (RACHS-1).This retrospective analysis included 359 cases requiring cardiopulmonary bypass (male, 195; female, 164; weight > 3.0 kg; aged 1 month to 18 years). Neonates and preoperatively intubated patients were excluded. Other exclusion criteria included severe preoperative pulmonary hypertension, high-dose catecholamine requirement after cardiopulmonary bypass, delayed sternal closure, laryngomalacia, serious bleeding, and delayed awakening. The early-extubation rates were compared between age groups and RACHS-1 classes.Overall, 83% of cases (298/359) were extubated in the operating room, classified by RACHS-1 categories as follows: 1, 59/59 (100%); 2, 164/200 (84%); 3, 61/78 (78%); and 4-6, 10/22 (45%). The early extubation rate in categories 1-3 (86%, 288/337) was significantly higher than for categories 4-6 (45.5%, 10/22) (P < 0.001). Because they met one of the exclusion criteria, 61 patients (17%) were not extubated in the operating room. Eight patients (2.7%) required re-intubation after early extubation in the operating room, and longer operation time was significantly associated with re-intubation (P < 0.001).Extubation in the operating room after congenital open-heart surgery was feasible based on our criteria, especially for patients in the low RACHS-1 categories, and involves a very low rate of re-intubation.

Surgical strategy for aortic arch reconstruction after the Norwood procedure based on numerical flow analysis.

OBJECTIVES: Inefficient aortic flow after the Norwood procedure is known to lead to the deterioration of ventricular function due to an increased cardiac workload. To prevent the progression of aortic arch obstruction, arch reconstruction concomitant with second-stage surgery is recommended. The aim of this study was to determine the indications for reconstruction based on numerical simulation and to reveal the morphology that affects the haemodynamic parameters. METHODS: Fifteen patients who underwent the Norwood procedure or arch repair and Damus-Kaye-Stansel anastomosis were enrolled. The pressure gradient in aortic arch was 1.6 ± 3.9 mmHg (ranged from 0 to 12 mmHg) on catheter examination. Six patients who had prominent turbulent flow accompanied with a large flow energy loss index greater than 40 mW/m2 and high wall shear stress greater than 100 Pa underwent arch reconstruction. RESULTS: After arch reconstruction, the energy loss index significantly decreased from 88.5 ± 50.0 mW/m2 to 23.1 ± 10.4 mW/m2 (P = 0.026) and wall shear stress significantly decreased from 194.5 ± 87.4 Pa to 60.3 ± 40.5 Pa (P = 0.0062). There were 3 late deaths due to heart failure caused by progressive atrioventricular valve regurgitation during the follow-up period (60 months). The systemic ventricular function was preserved in the remaining patients without any pressure gradients in the arch. CONCLUSIONS: Determining the surgical strategy for arch reconstruction based on numerical flow analysis may effectively reduce the ventricular load even if no stenosis or pressure gradients are observed on catheter examination or echocardiography.

Exploring energy loss by vector flow mapping in children with ventricular septal defect: Pathophysiologic significance.

BACKGROUND: Vector flow mapping is a novel echocardiographic flow visualization method, and it has enabled us to quantitatively evaluate the energy loss in the left ventricle (intraventricular energy loss). Although intraventricular energy loss is assumed to be a part of left ventricular workload itself, it is unclear what this parameter actually represents. The aim of the present study was to elucidate the characteristics of intraventricular energy loss. METHODS: We enrolled 26 consecutive children with ventricular septal defect (VSD). On echocardiography vector flow mapping, intraventricular energy loss was measured in the apical 3-chamber view. We measured peak energy loss and averaged energy loss in the diastolic and systolic phases, and subsequently compared these parameters with catheterization parameters and serum brain natrium peptide (BNP) level. RESULTS: Diastolic, peak, and systolic energy loss were strongly and positively correlated with right ventricular systolic pressure (r=0.76, 0.68, and 0.56, p<0.0001, = 0.0001, and 0.0029, respectively) and right ventricular end diastolic pressure (r=0.55, 0.49, and 0.49, p=0.0038, 0.0120, and 0.0111, respectively). In addition, diastolic, peak, and systolic energy loss were significantly correlated with BNP (r=0.75, 0.69 and 0.49, p<0.0001, < 0.0001, and=0.0116, respectively). CONCLUSIONS: In children with VSD, elevated right ventricular pressure is one of the factors that increase energy loss in the left ventricle. The results of the present study encourage further studies in other study populations to elucidate the characteristics of intraventricular energy loss for its possible clinical application.

Energy dynamics of the intraventricular vortex after mitral valve surgery.

Mitral valve morphology after mitral valve surgery affects postoperative intraventricular flow patterns and long-term cardiac performance. We visualized ventricular flow by echocardiography vector flow mapping (VFM) to reveal the impact of different mitral valve procedures. Eleven cases of mechanical mitral valve replacement (nine in the anti-anatomical and two in the anatomical position), three bioprosthetic mitral valve replacements, and four mitral valve repairs were evaluated. The mean age at the procedure was 57.4 ± 17.8 year, and the echocardiography VFM in the apical long-axis view was performed 119.9 ± 126.7 months later. Flow energy loss (EL), kinetic pressure (KP), and the flow energy efficiency ratio (EL/KP) were measured. The cases with MVR in the anatomical position and with valve repair had normal vortex directionality ("Clockwise"; N = 6), whereas those with MVR in the anti-anatomical position and with a bioprosthetic mitral valve had the vortex in the opposite direction ("Counterclockwise"; N = 12). During diastole, vortex direction had no effect on EL ("Clockwise": 0.080 ± 0.025 W/m; "Counterclockwise": 0.083 ± 0.048 W/m; P = 0.31) or KP ("Clockwise": 0.117 ± 0.021 N; "Counterclockwise": 0.099 ± 0.057 N; P = 0.023). However, during systole, the EL/KP ratio was significantly higher in the "Counterclockwise" vortex than that in the "Clockwise" vortex (1.056 ± 0.463 vs. 0.617 ± 0.158; P = 0.009). MVP and MVR with a mechanical valve in the anatomical position preserve the physiological vortex, whereas MVR with a mechanical valve in the anti-anatomical position and a bioprosthetic mitral valve generate inefficient vortex flow patterns, resulting in a potential increase in excessive cardiac workload.

Synchronization of the Flow and Pressure Waves Obtained With Non-Simultaneous Multipoint Measurements.

The use of measured data as boundary conditions renders hemodynamic simulations more patient-specific. However, synchronized acquisition of data at multiple locations is often difficult in clinical practice. This study proposes a method for resynchronizing measured data for use as boundary conditions for flow simulations using frequency analyses, and discusses the optimal cut-off frequency for differentiating cardiac and respiratory variation in hemodynamic data during resynchronization. To demonstrate the utility of the method, a Fontan circulation, which is the final palliative result with single-ventricle physiology, was used. The results suggest that it is optimal to set a cut-off frequency that gives a local minimum in the power spectrum that is slightly lower than the peak frequency of the heartbeat. Additionally, the total energy loss depended on the cut-off frequency, although the overall flow patterns appeared to be similar. The method is applicable to cardiovascular systems other than the Fontan circulation, where hemodynamic data with multifactorial fluctuations are required at various locations but simultaneous measurements are not possible.

Contributions of Respiration and Heartbeat to the Pulmonary Blood Flow in the Fontan Circulation.

BACKGROUND: In the Fontan circulation, driving forces with respiration, heartbeat, and lower limb muscle pump are relevant. However, the mechanics of these forces has not been proven, and their effects on the Fontan circulation remain unclear. METHODS: We performed catheter examinations and measured pressure and flow velocity simultaneously in the bilateral pulmonary arteries of 12 Fontan patients 1 year after the operation. The pulmonary pressure and flow velocity data were decomposed into respiratory and heartbeat components by discrete Fourier analysis. We then calculated respiratory and cardiac wave intensity (WI) based on the respiratory and heartbeat components of pressure and flow velocity data. RESULTS: Respiratory WI formed 2 negative peaks, a backward expansion wave during the inspiratory phase, and then a backward compression wave during the expiratory phase. In 2 phrenic nerve palsy cases and 1 case of a patient on a respirator, respiratory WI showed disturbed patterns and a negative pattern, respectively. Cardiac WI showed 2 or 4 negative peaks, the time phase of which matched that of the atrial contractions. CONCLUSIONS: WI analysis elucidated that inspiration acts as a sucking driving force and increases the pulmonary blood flow in the Fontan circulation. Respiratory complications compromise efficiency in the Fontan circulation. It was also revealed that the pulmonary blood flow was mutually dammed up and sucked in by increases and decreases in atrial pressure.

Timing of Haemophilus influenzae type b vaccination after cardiac surgery.

BACKGROUND: The best time for vaccination in infants with congenital heart disease (CHD) after cardiopulmonary bypass (CPB) surgery is unclear, but it is important to prevent Haemophilus influenzae type b (Hib) infection in infants with CHD after CPB surgery. To identify the best time for Hib vaccination in infants with CHD after CPB surgery, we investigated the immunological status, and the efficacy and safety of Hib vaccination after CPB surgery. METHODS: Sixteen subjects who underwent surgical correction of ventricular septal defect with CPB were investigated. Immunological status and cytokines were analyzed before surgery, 2 months after surgery, and before Hib booster vaccination. Hib-specific IgG was also measured to evaluate the effectiveness of vaccination. RESULTS: Immunological status before and 2 months after surgery (e.g. whole blood cells and lymphocyte subset profile) was within the normal range and no subjects had hypercytokinemia. Additionally, all subjects who received Hib vaccination at 2-3 months after CPB surgery had effective serum Hib-specific IgG level for protection against Hib infection without any side-effects. CONCLUSIONS: CPB surgery does not influence acquired immunity and Hib vaccination may be immunologically safe to perform at 2 months after CPB surgery. Hib vaccination at 2-3 months after CPB surgery was effective in achieving immunization for infants with simple left-right shunt-type CHD.

Norwood Procedure Performed on a Patient With Trisomy 13.

Trisomy 13 is associated with a variety of congenital anomalies, some of which are life-threatening and related to poor prognosis. Therefore, cardiac surgery is rarely offered to these patients, especially to those with complex cardiac anomalies. We report the case of a neonate weighing 2324 g who was born with severe congenital heart defects. Transthoracic echocardiography revealed the diagnoses of asplenia, single ventricle, aortic stenosis, coarctation of the aorta, hypoplastic aortic arch, and total anomalous pulmonary venous return. She was hemodynamically unstable. Palliative Norwood procedure with right ventricle-pulmonary artery conduit (RV-PA conduit) was performed at the age of 1 day to save her life. On postoperative day 7, chromosome analysis revealed trisomy 13. Echocardiography revealed good heart function; stable hemodynamic status was achieved with minimal amounts of inotropic agents. However, she developed anuria, which did not improve despite situational possible interventions, including peritoneal dialysis and continuous hemodiafiltration. On postoperative day 37, she succumbed to sudden cardiorespiratory failure. Nevertheless, this case indicates that a neonate with trisomy 13 can have a better chance at survival with cardiac surgery such as the Norwood procedure with an RV-PA conduit.

Impact of top end anastomosis design on patency and flow stability in coronary artery bypass grafting.

For coronary artery bypass grafting (CABG), free grafts such as a saphenous vein or radial artery are often used for grafts to the lateral and posterior walls. However, the relationship between top-end anastomosis design and long-term patency remains unknown. Because coronary artery blood flow is dominant during diastole, top-end anastomosis may work better if the graft is directed towards the apex, whereas the shortest graft pathway appears to be most efficient. Using computational fluid dynamic models, we evaluated the hemodynamic variables that were affected by the angle of the top-end anastomosis. We created three-dimensional geometries of the aortic root with coronary arteries that involved 75 % stenosis in the obtuse marginal and postero-lateral branches. Two bypass models under vasodilator administration were created: in a"Model A", the top-end anastomosis is parallel to the long axis of the ascending aorta and the graft passed over the conus directed towards the apex; in a "Model B", the top-end anastomosis is directed toward the shortest pathway, and form near the right angles to the long axis of the ascending aorta. Wall shear stress (WSS) and its fluctuation, an oscillatory shear index (OSI) were evaluated to predict fibrosis progression at the anastomosis site and graft flow. Graft flow was 197.3 ml/min and 207.3 ml/min in the "Model A" and "Model B", respectively. The minimal WSS value inside the graft with the "Model A" and "Model B" was 0.53 Pa and 4.09 Pa, respectively, and the OSI value was 0.46 and 0.04, respectively. The top-end anastomosis of a free graft should be directed vertically towards the aorta to achieve the shortest graft pathway to maintain a high graft flow rate and to avoid the risks of endothelial fibrosis and plaque progression over the long-term after CABG.

Optimal Graft Size of Modified Blalock-Taussig Shunt for Biventricular Circulation in Neonates and Small Infants.

The modified Blalock-Taussig shunt (mBTS) is one of the most important palliative procedures in congenital heart surgery. However, in neonates and small infants, operative mortality and morbidity due to excessive pulmonary blood flow or shunt failure remains high. In this study, a small shunt graft (3.0-mm diameter) was estimated to determine the optimal shunt graft size of BTS as an initial palliation for ultimate biventricular circulation. Eighteen patients weighing an average 3.5 kg who underwent mBTS from July 2004 to January 2013 at our institute were reviewed. We divided the study cohort into two groups: group S (n = 10) included patients with 3.0-mm diameter shunt grafts, and group L (n = 8) included patients with 3.5-mm diameter shunt grafts. There were no hospital deaths or shunt occlusion in either group. One group L patient (12.5%) had cardiogenic shock due to excessive pulmonary blood flow. There were no differences in postoperative arterial oxygen saturation (SaO2) between the groups. There were no differences in body weight at intracardiac repair (ICR) between the groups. During the interstage to ICR, body weight gain was significantly greater in group S than in group L (P = 0.008). The small shunt graft (3.0-mm diameter) in BTS was safe, provided adequate pulmonary blood flow, and led to significant weight gain between mBTS and ICR for ultimate biventricular circulation in neonates and small infants with low body weight.

Impact of the entry site on late outcome in acute Stanford type B aortic dissection†.

OBJECTIVES: This study aimed to investigate whether the entry site of acute type B aortic dissection affects late outcomes. METHODS: Inpatient and outpatient records were retrospectively reviewed. RESULTS: We identified 224 cases of acute type B aortic dissection between 1998 and 2013. Of these 224 patients, 168 were men and the age was 64.2 ± 12.6 (range 23-94) years, from which 130 presented with the entry at a location downstream of the distal aortic arch, 67 with the entry at the outer curvature of the distal aortic arch and 27 with the entry at the inner curvature. At the initial presentation, 127 patients had descending false lumen thrombosis. The 30-day mortality rate was 2%, and 8% of patients had malperfusion. The entry at the outer curvature was associated with a higher risk of 30-day mortality. Patients with the entry at a location downstream were significantly older, and had a higher chance for primarily thrombosed descending false lumen and a lower risk of malperfusion. At follow-up (6.0 ± 4.1 years), the actuarial survival rates were 97, 83 and 60%, freedoms from open aortic surgery were 96, 91 and 86%, aortic intervention were 73, 66 and 63% and aortic events were 71, 60 and 52% at 1, 5 and 10 years, respectively. Multivariate logistic regression analysis revealed that the outer curvature entry and maximum aortic diameter were correlated with open aortic surgery, aortic intervention and aortic events. Of the 127 patients with primarily thrombosed false lumen, the outer curvature entry was significantly correlated with aortic events. CONCLUSIONS: The primary entry at the outer curvature of the distal aortic arch, as well as the large aortic diameter, is associated with a higher risk of late open aortic surgery, aortic intervention and aortic events in acute type B aortic dissection. Thus, the entry site should be taken into consideration in the establishment of an appropriate treatment indication of type B aortic dissection.

Seventeen-month-long paracorporeal biventricular mechanical support as a bridge to transplantation for severe dilated cardiomyopathy.

The long-term management of paracorporeal biventricular assist devices (BiVAD) is difficult because of significant risks of bleeding, thrombosis, and infection. Here we report the case of a 41-year-old woman with severe dilated cardiomyopathy who developed serious cerebral bleeding after receiving a paracorporeal BiVAD but recovered well after treatment. She eventually underwent cardiac transplantation 17 months after implantation of the paracorporeal BiVAD.

Clinical utility of the plasma brain natriuretic peptide level in monitoring tetralogy of fallot patients over the long term after initial intracardiac repair: considerations for pulmonary valve replacement.

Clinicians are currently encountering an increasing number of patients in the long-term period after tetralogy of Fallot (TOF) repair presenting with pulmonary valve regurgitation (PR) or right ventricular (RV) dysfunction. The purpose of this study was to evaluate the clinical utility of the plasma brain natriuretic peptide (BNP) level and consider surgical indications and timing of pulmonary valve replacement (PVR). We examined 33 patients (21 males, 12 females, mean age 14.5 ± 2.8 years) who underwent TOF repair at Kitasato University Hospital. All patients were evaluated using echocardiography and blood sampling. The mean age at the time of initial repair was 1.3 ± 0.7 years. The patients with moderate-severe PR exhibited significantly higher plasma BNP levels than the patients with trivial-mild PR (mean 37.5 ± 33.1 vs. 17.3 ± 6.6 pg/ml, p = 0.013). The mean plasma BNP level with cardiac symptoms was higher than that observed in the patients without any symptoms (71.4 ± 46.1 vs. 25.0 ± 14.0 pg/ml, p = 0.005). The mean BNP level was significantly decreased after PVR (71.3 ± 46.1-26.1 ± 13.2 pg/ml, p = 0.009), and the plasma BNP level was found to be positively correlated with the RV end-diastolic pressure (r = 0.851; p = 0.008). The optimal BNP cut-off value for considering PVR was 32.15 pg/ml (sensitivity, 85.7 %; specificity, 83.3 %). The plasma BNP level may become a useful diagnostic tool for considering the indications and optimal timing of PVR over the long term after TOF repair.