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The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past 9 years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases. The scheme to categorize a ventricular septal defect uses both its location and the structures along its borders, thereby bridging the two most popular and disparate classification approaches and providing a common language for describing each phenotype. Although the first-order terms are based on the geographic categories of central perimembranous, inlet, trabecular muscular, and outlet defects, inlet and outlet defects are further characterized by descriptors that incorporate the borders of the defect, namely the perimembranous, muscular, and juxta-arterial types. The Society recognizes that it is equally valid to classify these defects by geography or borders, so the emphasis in this system is on the second-order terms that incorporate both geography and borders to describe each phenotype. The unified terminology should help the medical community describe with better precision all types of ventricular septal defects., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The long-term outcome of modified Fontan operation concomitant with a valve operation for atrioventricular valve (AVV) regurgitation is not well described., Methods: Between 1977 and 2003, 500 children who underwent modified Fontan operation were subdivided into 192 with AVV plasty (group P) and 308 without AVV plasty (group N). Factors associated with patient outcome were investigated retrospectively., Results: Surgical techniques to correct valve incompetence included circular annuloplasty, partial annuloplasty with Kaye-Reed methods, edge-to-edge repair methods, and valvoplasty, which were combined according to the etiology of the valve lesion. The estimated actuarial survival rates at 10 and 20 years were, respectively, 82.0% and 76.6% in group P (p < 0.05) and 90.8% and 86.8% in group N (p = 0.001). The estimated actuarial survival rates at 10 years among patients with AVV plasty did not show a statistically significant difference (circular annuloplasty, 79.0%; partial annuloplasty, 81.6%; edge-to-edge, 83.3%; valvoplasty, 82.6%; p = 0.90). A Cox regression model revealed that a higher preoperative ventricular ejection fraction was associated with a significant reduction in long-term mortality in group P (hazard ratio, 0.921; 95% confidence interval, 0.873 to 0.972; p < 0.05). Each repair method showed acceptable durability at medium-term follow-up, without progression of lesions., Conclusions: Given worse results with poor ventricular function, early intervention against valve pathology before development of ventricular failure may improve long-term outcomes. Multiple methods are appropriate for a variety of valve lesions; however, circular annuloplasty remains a reliable repair option due to its technical simplicity., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Surgical results for functional univentricular heart with total anomalous pulmonary venous connection (TAPVC) have been unsatisfactory to date., Methods: During a 25-year period until December 2009, 207 TAPVC patients underwent surgical repair at our institute, including 56 with a univentricular heart. The 10-year survival rate was 51.1% with univentricular heart and 84.7% with biventricular heart (p<0.0001; log-rank, 27.6). Surgical outcomes and risk factors for early and late death after TAPVC repair in univentricular hearts were retrospectively analyzed., Results: Patients were aged 3.8±4.3 years and weighed 12.3±10.7 kg at operation. Preoperative diagnoses included heterotaxy syndrome in 55, asplenia in 48, preoperative pulmonary venous obstruction in 35, and pulmonary atresia in 20. TAPVC was classified as I in 22, II in 26, III in 5, and IV in 3. Concomitant procedures included Fontan procedure in 29, bidirectional Glenn procedure in 5, systemic-pulmonary shunt in 11, and pulmonary artery banding in 5. There were 17 hospital deaths and 11 late deaths. Fontan completion was undertaken in 31 (55.3%). Postoperative pulmonary venous obstruction was found in 15. Multivariate analysis identified TAPVC III and IV and pulmonary atresia as risk factors for hospital death. Univariate analysis identified postoperative pulmonary venous obstruction and concomitant systemic-pulmonary shunt as risk factors for hospital and late death., Conclusions: TAPVC III, IV, and pulmonary atresia are risk factors for early postoperative death. Intensive intervention, including perioperative management and operation, is required in these complex patients., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The optimal management of atrial tachyarrhythmia (AT) late after Fontan operation has not yet been established., Methods and Results: Of 199 patients who were followed for more than 10 years after Fontan operation, 60 patients in whom late postoperative arrhythmias were observed were the subjects of this study. These arrhythmias were managed with anti-arrhythmic drugs. Twenty-one of 60 patients (35%) did not respond to the drugs and they needed further interventions. Fourteen catheter ablation procedures were performed in nine patients (atrial fibrillation in one patient, AT in eight patients) and the success rate was 44%. Conversion to total cavopulmonary connection (TCPC) was performed in 14 patients and Maze operation was performed at the time of Fontan conversion in 6 patients. Sinus rhythm or pacemaker rhythm was maintained in 7 of 14 (50%) patients postoperatively., Conclusions: Although arrhythmogenic substrates accumulate and tachyarrhythmia becomes frequent over the long-term following Fontan operation, most patients with AT can be managed medically. The success rate of catheter ablation and Maze operation is low but those interventions may be indicated in patients with intractable arrhythmias.

A 52-year-old man underwent surgical repair for coronary arteriovenous fistula including patch closure of two exitus of the fistula, double ligation of the dilated right coronary artery, and coronary bypass grafting to the right coronary artery with saphenous vein graft at the age of 25. Then, twenty-seven years later, a multi-detector computed tomographic scan revealed an aneurysmally dilated right coronary artery (4.8 cm), fistula, and saphenous vein graft. The aneurysm was resected and histologically mucoid degeneration in the media of the aneurysmal wall was detected.

Several hemostat hydrogels are clinically used, and some other agents are studied for safer, more facile, and more efficient hemostasis. In the present paper, we proposed a novel method to evaluate local hemostat hydrogel on tissue surface. The procedure consisted of the following steps: (step 1) a mouse was fixed on a cork board, and its abdomen was incised; (step 2) serous fluid was carefully removed because it affected the estimation of the weight gained by the filter paper, and parafilm and preweighted filter paper were placed beneath the liver (parafilm prevented the filter paper's absorption of gradually oozing serous fluid); (step 3) the cork board was tilted and maintained at an angle of about 45 degrees so that the bleeding would more easily flow from the liver toward the filter paper; and (step 4) the bleeding lasted for 3 min. In this step, a hemostat was applied to the liver wound immediately after the liver was pricked with a needle. We found that (1) a careful removal of serous fluid prior to a bleeding and (2) a quantitative determination of the amount of excess aqueous solution that oozed out from a hemostat were important to a rigorous evaluation of hemostat efficacy. We successfully evaluated the efficacy of a fibrin-based hemostat hydrogel by using our method. The method proposed in the present study enabled the quantitative, accurate, and easy evaluation of the efficacy of local hemostatic hydrogel which acts as tissue-adhesive agent on biointerfaces.

In a 57-year-old man with Wolff-Parkinson-White syndrome, a left ventricular pseudoaneurysm developed, induced by the pop phenomenon, after radiofrequency catheter ablation of the posterolateral accessory pathway. The pseudoaneurysm was positioned at the atrioventricular groove with moderate pericardial adhesions, and the rupture site was located just below the P3 annulus of the posterior mitral leaflet. With this anatomy and in the presence of adhesions, we repaired the rupture site from within the heart through an incision made in the posterior mitral leaflet. This transmitral approach might be a reasonable surgical option for treating this type of submitral left ventricular pseudoaneurysm.

Surgical treatment of arterial Behcet's disease (BD) has a higher incidence of graft-related complications such as anastomotic pseudoaneurysm or graft occlusion. A 64-year-old man presented with a rupture of the thoracoabdominal aortic aneurysms associated with BD. Evaluation shows a large hematoma in the retroperitoneum and multiple aneurysms of the thoracoabdominal aorta. Physical examination and past history fulfills the diagnostic criteria of BD. The abdominal aorta was replaced with an allograft and the major branches were reconstructed with its branches. The postoperative course was uneventful. A 10-month follow-up computed tomographic scan did not show any graft-related complications. This case suggests the usefulness of an allograft for arterial involvement of BD.

The most concrete and universal outcome measure used in databases, whether governmental, professional society, research, or third-party payer, is operative mortality. To assure congruous data entry by multiple users of The Society of Thoracic Surgeons and the European Association for Cardiothoracic Surgery congenital heart surgery databases, operative mortality must be clearly defined. Traditionally, operative mortality has been defined as any death, regardless of cause, occurring (1) within 30 days after surgery in or out of the hospital, and (2) after 30 days during the same hospitalization subsequent to the operation. Differing hospital practices result in problems in use of the latter part of the definition (eg, the pediatric hospital that provides longer-term care will have higher mortality rates than one which transfers patients to another institution for such care). In addition, because of the significant number of pediatric multiple operation hospitalizations, issues of assignment of mortality to a specific operation within the hospitalization, calculation of operative mortality rates (operation based vs patient admission based), and discharge other than to home must be addressed and defined. We propose refinements to the definition of operative mortality which specifically meet the needs of our professional societies' multi-institutional registry databases, and at the same time are relevant and appropriate with respect to the goals and purposes of administrative databases, government agencies, and the general public.

Background: The procedure of quadrangular resection and suture for prolapsed posterior leaflet of the mitral valve is a reliable and reproducible method that achieves excellent long-term results. However, triangular resection and suture of a prolapsed anterior leaflet is not widely supported and different techniques have been advocated. The aim of this study was to review our experience of mitral valve repair in which resection of the anterior and/or posterior leaflets was performed., Methods: Between October 1991 and September 2003, 105 patients with mitral regurgitation underwent mitral valve reconstruction with leaflet resection, including 55 patients with quadrangular resection of the posterior leaflet (P), 32 patients with triangular resection of the anterior leaflet (A), and 18 patients with resection of both leaflets (A+P)., Results: The mean follow-up period was 63.6 (1 to 139) months. Reoperation was required in 2 patients, each after resection of the anterior or posterior leaflet. The freedom from reoperation rates at 10 years in 93% +/- 5% of patients after triangular resection of the anterior leaflet, 96% +/- 3% after quadrangular resection of the posterior leaflet, and 100% after resection of both leaflets. There were no significant differences of survival or risk of reoperation among these three groups. The postoperative mitral valve area was significantly smaller than the preoperative area in all three groups, but remained large enough (A: 2.84 +/- 1.07; P: 2.6 +/- 0.87; A+P: 3.09 +/- 1.20 cm2) for adequate valve function., Conclusions: Triangular resection of a prolapsed anterior mitral leaflet is a reliable, reproducible, and durable procedure, like quadrangular resection of a prolapsed posterior leaflet.

A patient with transposition of the great arteries accompanied by Shaher type 9 coronary anatomy experienced the development of progressive neoaortic valvular regurgitation with a small annulus, supravalvular stenosis, and neopulmonary valvular and supravalvular stenoses 15 years after an arterial switch operation. To implant a prosthetic valve clinically adequate in size, the Konno procedure was necessary. However, the right coronary anatomy precluded the original Konno procedure. My colleagues and I accomplished neoaortic anterior annular enlargement in this case by using atrioventricular groove patch plasty without jeopardizing the right coronary artery, and this resulted in a satisfactory outcome.

Background: In conventional conduit operations, longevity has been essentially limited by the inevitable need for conduit replacement. This study was undertaken to compare long-term results of the use of equine pericardial conduits, autologous pericardial conduits, and direct anastomosis repair., Methods: Between 1982 and 2001, 366 patients underwent primary establishment of right ventricle-pulmonary artery continuity at our institution. The mean age at the time of operation was 6.2 years (range, 4 days to 28 years) and mean weight was 17.2 kg (range, 1.6 to 61 kg). Three different repair techniques were used for connection: hand-made valved equine pericardial conduits (n = 179), autologous pericardial conduits (n = 71), and direct anastomosis without a conduit (n = 116). Mean follow-up period for early survivors was 8.6 years in the equine group, 6.1 years in the direct anastomosis group, and 5.1 years in the autologous pericardium group., Results: Direct anastomosis repair (p = 0.0002) was associated with significantly better freedom from late events (conduit replacement or late death) than equine pericardial conduits. The hazard ratio was less with the autologous pericardium conduit than with the equine pericardium, but the difference was not statistically significant (p = 0.2122). Younger age at operation, and postoperative pressure ratio from right to left ventricle were also predictors of conduit longevity., Conclusions: To decrease the probability of late events, direct anastomosis is an encouraging technique compared with traditional equine pericardium extracardiac conduit repair. An autologous pericardial conduit, because of its benefits, would be an alternative when direct anastomosis is not suitable.

Background: The activation of inflammatory cells and the production of matrix metalloproteinases (MMPs) are important in the pathogenesis of abdominal aortic aneurysm (AAA). Previous studies have demonstrated that the antiplatelet agent trapidil has multiple actions, including suppression of MMP expression through the inhibition of the CD40-CD40 ligand (CD40-CD40L) pathway in cultured cells. A recent clinical study suggested that trapidil might have functions beyond its antiplatelet action. Methods and results In the present study, we performed immunohistochemical analysis and semiquantitative reverse transcription-polymerase chain reaction to evaluate the effect of trapidil on the production of MMPs in cultured aortic tissues from patients with infrarenal AAA (n = 9) and control patients with aortoiliac occlusive disease (n = 7). The tissue concentrations of both MMP-2 and MMP-9 were significantly higher in AAA walls than in control aortic walls. Both trapidil and an anti-CD154 (CD40L) antibody significantly suppressed the protein production and mRNA expression of MMP-2 but did not inhibit those of MMP-9 in organ cultures of AAA wall specimens. MMP-9 was produced by macrophages and a lot of neutrophils in AAA tissues, whereas MMP-2 was derived from macrophages. CD40 was expressed on macrophages but not on neutrophils, and this expression could explain the differential effect of trapidil on the production of MMP-2 and MMP-9., Conclusions: Trapidil, a CD40-CD40L pathway inhibitor, suppressed mRNA expression and protein production of MMP-2 in AAA tissues, suggesting a potential therapeutic approach for the prevention or treatment of AAA.

A 7-year-old girl with unilateral absence of the pulmonary artery underwent autologous tissue limited reconstructive surgery. The proximal portion of the artery was reconstructed by rotating a reverse U-shaped cut opposite the pulmonary arterial wall and covering the anterior surface with autologous pericardium. Follow-up catheterization at 5.8 years after surgery revealed no stenosis. This procedure could become one of the preferred methods for this unusual clinical condition.

Two patients with double inlet left ventricles were successfully treated by ventricular septation. Patient 1, a 38-year-old woman, underwent operation in 1971. This patient has been a full-time worker. Patient 2, a 2-year-old boy, had a relatively small left ventricle with an estimated volume of 134% of normal value. This patient underwent ventricular septation in 1995. He is now in New York Heart Association class II and enjoying school life.

Background: Plasma pheresis and reinfusion of platelet-rich plasma has not been shown to reduce blood loss in cardiac patients. Recently, freshly prepared autologous platelet concentrates (PC) can be made from patient's blood and has a higher concentration than conventional platelet rich plasma. In this study, the effects of autologous PC reinfusion were examined after open heart surgery in patients with congenital heart disease., Methods: Eight patients with noncyanotic congenital heart disease, who underwent open heart surgery and reinfusion of autologous PC, were classified as the PC group. Eight other patients with noncyanotic congenital heart disease, who underwent only open heart surgery, were defined as the control group. Ages ranged from 2 to 24 years and were not significantly different between the two groups (9.3 +/- 5.1 years in the PC group and 12.6 +/- 7.9 years in the control group, p = 0.33). In the PC group, blood was collected from the femoral vein through a 6F catheter introducer; 9 to 20 U (13.0 +/- 5.4 U, 0.42 +/- 0.22 U/kg) of autologous PC were prepared and were reinfused after protamine administration. The time course of platelet counts was examined until postoperative day 7. Aggregation responses to adenosine diphosphate; (4 micromol/L and 8 micromol/L), collagen (1 micromol/L and 5 micromol/L), and epinephrine (5 micromol/L and 10 micromol/L) were evaluated after induction of anesthesia (individual references), after protamine administration, at the end of the operation; these responses were shown as recovery ratios., Results: Blood loss during surgery in the PC group was significantly less than in the control group (4.8 +/- 3.0 mL/kg versus 7.8 +/- 1.7 mL/kg, p = 0.044). Similarly blood loss on postoperative day 1 in the PC group was significantly less than in the control group (3.6 +/- 1.2 mL/kg versus 7.2 +/- 3.1 mL/kg, p = 0.013). The platelet counts in the PC group were larger than those in the control group until postoperative day 5, after reinfusion of prepared autologous PC. The recovery ratios of the aggregation responses to adenosine diphosphate, collagen, and epinephrine after protamine administration were not significantly different between the two groups. However, recovery in the PC group after reinfusion of the prepared autologous PC was greater than in the control group., Conclusions: Reinfusion of the freshly prepared autologous PC was followed by good aggregation responses and low blood loss in patients with noncyanotic congenital heart disease after open heart surgery. This procedure may be useful in pediatric open heart surgery without blood transfusion or with little administration of homologous blood products.

Background: Achieving unobstructed blood flow from the systemic ventricle to the aorta is important during the Fontan procedure for complex cyanotic congenital heart disease when there is systemic ventricular outflow obstruction (SVOO). Because SVOO can progress after the Fontan procedure if there is morphologic obstruction, we have adopted a policy of relieving obstructions to systemic blood flow., Methods: Twenty-five patients were treated by the Fontan procedure with SVOO. Twenty-one patients had undergone prior pulmonary artery banding and 10 patients had undergone prior arch repair. Systemic ventricular outflow obstruction progressed in 5 patients after the Fontan procedure. Main diagnosis was single ventricle in 12, tricuspid atresia in 5, transposition of the great arteries in 4, double-outlet right ventricle in 3, and common atrioventricular canal in 1. Mean age at operation was 6.5 years (range 1 to 15 years) and the average preoperative pressure gradient across the ascending aorta and systemic ventricle was 29 mm Hg (range 0 to 100 mm Hg). The Damus-Kaye-Stansel procedure was performed in 18 patients (double-barrel anastomosis in 13, end to side anastomosis in 5), and subaortic resection or ventricular septal defect or bulboventricular foramen enlargement was performed in 7. Double-barrel anastomosis has been our first choice since 1994, if the pulmonary valve is intact. Follow-up has ranged from 4 months to 14 years (average 5.0 years). Twenty-three of the 25 patients have undergone recatheterization (average 21.4 months later)., Results: No early deaths were found; one late death was reported of a patient with single right ventricle (4.0%). The postoperative average pressure gradient was 1.1 mm Hg (0 to 10 mm Hg), and the average right atrial pressure was 14 mm Hg (9 to 20 mm Hg). In all patients who underwent ventricular septal defect or bulboventricular foramen enlargement, regular sinus rhythm was maintained postoperatively. Regarding the Damus-Kaye-Stansel procedure, there was minimal progression of semilunar valve insufficiency except in 1 patient who underwent end-to-side anastomosis with moderate pulmonary regurgitation postoperatively., Conclusions: The midterm results of the Fontan procedure with SVOO have been satisfactory. Because SVOO might progress after the Fontan procedure if there is morphologic obstruction, an appropriate strategy to relieve obstruction to systemic blood flow should therefore be performed concomitantly with the Fontan procedure.

Background: Pulmonary vascular resistance (PVR) is closely related with patients' hemodynamics after the Fontan procedure and endothelin-1 (ET-1) may play an important role in pulmonary circulation. Modified ultrafiltration (MUF) is known to remove inflammatory mediators after cardiopulmonary bypass (CPB) surgery. The time courses of plasma ET-1 and PVR were examined before and after the Fontan procedure with MUF., Methods: Twenty-two patients who underwent the Fontan procedure were divided into two groups: a dilutional ultrafiltration/modified ultrafiltration (DUF/MUF) group (n =11) and a control group (n = 11). Conventional ultrafiltration was performed during CPB in the control group. DUF was performed semicontinuously during CPB and MUF was continued until 15 to 20 minutes after the CPB with polyacrylonitonile membrane in the DUF/ MUF group. The plasma ET-1 concentration was measured before and after CPB, after MUF in the DUF/MUF group, and 6 and 24 hours after CPB. PVR was calculated simultaneously using a thermodilutional catheter., Results: Plasma ET-1 levels increased significantly after CPB in the control group but they did not increase immediately after CPB in the DUF/MUF group. Similarly, PVR increased significantly after CPB in the control group but it did not increase after CPB in the DUF/MUF group and remained low at 6 and 24 hours after CPB., Conclusions: DUF and MUF suppress the increase in the plasma ET-1 concentration that occurs immediately after the completion of the Fontan procedure and may be an effective intervention for maintaining low PVR after the procedure.

We evaluated the utility of in-situ hybridization (ISH) for the rapid diagnosis of sepsis. We applied this approach to polymorphonuclear neutrophil (PMN)-rich smears from patients with suspected bacterial infection. Positive results by ISH were obtained in the smears of 123 of 292 patients (42%), while only 32 of the 292 (11%) were positive by blood culture. These findings indicate that ISH is almost four times more sensitive than the culture method for the detection of sepsis. ISH results are obtained within 1 day, while 1 day to 2 weeks is required for the results of blood culture. Blood culture and ISH methods detected the same bacteria in two patients. ISH also successfully identified the same bacteria in blood and PMN-rich body fluid (bronchoalveolar lavage samples) in 6 patients. In 19 patients, ISH of blood detected the same bacteria as those found in subcultures from other sources (e.g., stool, sputum, nasal cavity). We discuss these results in comparison with blood culture results in terms of evaluating a rapid approach to the management of patients with sepsis.