Aortic valve replacement for infective endocarditis in an adult with uncorrected tetralogy of Fallot and pulmonary atresia.

Infective endocarditis is one of the complications encountered in patients with uncorrected tetralogy of Fallot; however, there have been only limited reports on surgical treatment of this condition. A 38-year-old man with uncorrected tetralogy of Fallot with pulmonary atresia previously palliated with modified Blalock-Taussig shunt was diagnosed with aortic valve infective endocarditis and developed severe aortic regurgitation. He underwent urgent aortic valve replacement, during which intermittent hypothermic circulatory arrest was necessary to achieve adequate myocardial protection and bloodless surgical field due to massive arterial return into the left ventricle from the collateral pulmonary circulation.

A Novel Aortic Reconstruction for Anomalous Left Coronary Arising From the Right Pulmonary Artery in Hypoplastic Left Heart Syndrome: Successful Surgical Treatment.

An anomalous left coronary arising from the pulmonary artery (ALCAPA) in hypoplastic left heart syndrome (HLHS) is a very rare congenital malformation. The Norwood procedure and simultaneous direct reimplantation of the left coronary artery (LCA) to the ascending aorta have been previously used for treatment of ALCAPA, although mortality was very high, because it is difficult to reimplant the LCA to the neoaortic root without distortion or tension. We chose the modified Norwood procedure without reimplantation of the LCA to the neoaortic root, and 4-month-old male infant survived the operation and waiting Fontan procedure.

Novel aortic arch reconstruction using a modified Norwood procedure based on hypoplastic left heart syndrome-specific anatomical malformations.

OBJECTIVES: Postoperative left pulmonary artery (PA) or bronchus compression occasionally follows narrowing of the retroaortic space after the Norwood procedure. We examined hypoplastic left heart syndrome (HLHS)-specific anatomical malformations and hypothesized that a long main pulmonary arterial trunk might contribute to a larger retroaortic space. In this study, we introduced a modified Norwood procedure [pulmonary artery trunk-saving method (PATS)] and evaluated the results. METHODS: HLHS-specific anatomical malformations were examined using computed tomographic data from 23 consecutive patients who underwent bilateral pulmonary banding. Fourteen patients had HLHS or associated conditions (Group H), and 9 patients had other complex cardiac defects and underwent biventricular repair (Group B). Five consecutive HLHS patients underwent PATS as a modified Norwood operation, and 6 controls underwent a conventional Norwood procedure. We used computed tomography to measure the lengths of the aorta and PA and the angle of both pulmonary branches to evaluate the effect of PATS. RESULTS: Preoperative examination confirmed HLHS-specific right PA branching adjacent to the pulmonary valve and a trend towards a shorter main pulmonary arterial trunk with the conventional Norwood procedure. Also, both right and left pulmonary arterial branching from the dorsal aspect of the main pulmonary arterial trunk and the PA branch angle were minimal in HLHS patients. Postoperative data showed the retroaortic space behind the reconstructed neoaorta was significantly wider in PATS patients than in conventional Norwood patients. Longitudinal measurements (between the aortic arch and pulmonary valve) and sagittal measurements (ascending aorta to descending aorta) were larger in PATS patients, and no left PA or airway obstruction was observed following PATS. CONCLUSIONS: PATS resulted in a wider aortopulmonary space without left pulmonary arterial stenosis or bronchus obstruction. HLHS-specific anatomical malformations suggested that PATS may avoid extrinsic compression of the left PA and bronchus by the neoaorta and can be an alternative for HLHS patients.

[Transposition of Great Artery].

Transposition of the great artery is one of common congenital cardiac disease resulting cyanosis. Death occurs easily in untreated patients with transposition and intact ventricular septal defect (VSD) in infancy at a few days of age when posterior descending coronary artery (PDA) closed. Since there are 2 parallel circulations, flow from pulmonary to systemic circulation is necessary for systemic oxygenation, and Balloon atrial septostomy or prostaglandin infusion should be performed especially if patient do not have VSD. Although the advent of fetal echocardiography, it is difficult to diagnose the transposition of the great arteries (TGA) as abnormality of great vessels is relatively undistinguishable. The diagnosis of transposition is in itself an indication for surgery, and arterial switch procedure is performed in the case the left ventricle pressure remains more than 2/3 of systemic pressure. Preoperative diagnosis is important as associated anomalies and coronary artery branching patterns are important to decide the operative indication and timing of surgery.

Predictors of 90-day mortality after congenital heart surgery: the first report of risk models from a Japanese database.

OBJECTIVE: The purpose of this study was to develop risk models for congenital heart surgery short-term and midterm outcomes from a nationwide integrated database drawn from hospitals in Japan. METHODS: The Japan Congenital Cardiovascular Surgery Database collects clinical information from institutions throughout Japan specializing in congenital heart surgery. Variables and definitions used in the Japan Congenital Cardiovascular Surgery Database are almost identical to those of the Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery database for congenital heart surgery. We used logistic regression to develop risk models, which were then validated through spilt-sample validation. In addition to procedural complexity categories by Risk Adjustment in Congenital Heart Surgery (RACHS-1) score, we incorporated patient characteristics to predict surgical outcome. RESULTS: Among 8923 congenital heart operations performed at 69 sites with cardiac surgical programs, 30-day mortalities by RACHS-1 category were as follows: I, 0.1% (n=1319); II, 0.5% (n=3211); III, 2.2% (n=3285); IV, 4.3% (n=818); and V and VI, 8.6% (n=290). From the test data set (n=7223), we developed 3 risk models (30-day mortality, 90-day mortality, and 90-day and in-hospital mortality) with 11 variables, including age category, RACHS-1 category, preoperative risk factors, number of surgical procedures, unplanned reoperations, status of surgery, surgery type, asplenia, and prematurity (<35 weeks). For the performance metrics of the risk models, C statistic values of 30-day, 90-day, and 90-day and in-hospital mortalities for the test data set were 0.85, 0.85, and 0.84, respectively. When only the RACHS-1 score was used for discrimination, the C statistic values of 30-day, 90-day, and 90-day and in-hospital mortalities for the validation data set were 0.73, 0.73, and 0.77, respectively. CONCLUSIONS: The proposed risk scores and categories have high discrimination power for predicting mortality, demonstrating improvement relative to existing consensus-based methods. Risk models incorporating these measures may be useful for comparing mortality outcomes cross institutions or countries with mixed cases.

Transcatheter closure of secundum atrial septal defect.

Since the introduction of transcatheter closure of atrial septal defect, device closure has become the alternative treatment of selected atrial defects. Although excellent results have been reported for transcatheter closure, concerns have arisen regarding complications, including residual shunt, systemic or pulmonary embolization of the device, and erosion and perforation of the cardiac chamber. Those complications are rare but potentially serious adverse events that may require immediate surgical intervention. This review summarizes the current trends in patient selection, result of device closure and typical complications. Comparison between transcatheter device closure and surgical closure is also made.

Manouguian double-valve replacement in a 6-year-old girl.

A 6-year-old girl with a diagnosis of aortic regurgitation with stenosis and mitral regurgitation because of short chordae was referred to us for surgery. Echocardiography revealed that the aortic and the mitral annular diameters were 16 and 23 mm, respectively. The Ross procedure and mitral valvuloplasty were scheduled. During the procedure, we were concerned that aggressive mitral valvuloplasty might result in mitral stenosis. We therefore converted the procedure to double-valve replacement using the Manouguian technique because it was necessary to enlarge both the aortic and mitral annuli. In children, the mitral prosthetic valve in Manouguian technique may override aortic annulus resulting in left ventricular outflow tract obstruction (LVOTO). Thus, it is important to decide the mitral prosthetic valve size. Measurements of both annuli showed 15 and 21 mm in aortic and mitral positions, respectively. Size #18 ATS AP mechanical valve (ATS Medical, Inc., Minneapolis, MN, USA) and size #23 ATS mechanical valve were implanted. We successfully performed two sizes up in the aortic position and one size up in the mitral position avoiding complications such as coronary orifice obstruction and LVOTO. To our knowledge, this is the youngest patient who underwent double-valve replacement by the Manouguian technique.

Long-term prognosis of double-switch operation for congenitally corrected transposition of the great arteries.

OBJECTIVES: Recently, the double-switch operation for congenitally corrected transposition of the great arteries has become the procedure of choice in our institute; however, the long-term follow-up is uncertain. METHODS: From 1983 to 2010, 90 patients with congenitally corrected transposition of the great arteries underwent the double-switch operation, which comprised of an atrial switch plus intraventricular rerouting (with or without extracardiac conduits) in 72 patients (group I), and an atrial switch plus arterial switch in 18 patients (group II). The mean age at operation was 7.4 years old in group I vs. 4.3 years old in group II. The mean follow-up period was 12.9 years in group I vs. 10.9 years in group II. Hospital and late mortality, reoperation, arrhythmia and NYHA status were analysed retrospectively. RESULTS: The Kaplan-Meier survival, including hospital and late mortality at 20 years, was similar (75.7% in group I vs. 83.3% in group II). The freedom from reoperation was 77.6% in group I (redo-Rastelli in five patients, subaortic stenosis resection in three, tricuspid valve replacement in one and mitral valve plasty in one) vs. 94.1% in group II (P < 0.05 vs. group I; aortic valve replacement in one). The freedom from arrhythmia was 57.1% in group I vs. 78.6% in group II (P < 0.05 vs. group I). The ratio of NYHA class I to II at outpatient clinic was similar (86% in group I vs. 86% in group II). CONCLUSIONS: The long-term prognosis of the double-switch operation for congenitally corrected transposition of the great arteries was acceptable. In particular, an atrial switch plus arterial switch could be performed with low morbidity, and it should be considered as the optimal procedure.

Surgical results for functional univentricular heart with total anomalous pulmonary venous connection over a 25-year experience.

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.

Impact of Fontan conversion with arrhythmia surgery and pacemaker therapy.

OBJECTIVE: In the long-term period after Fontan operation, atrial arrhythmia was one of the important factors to decide the postoperative quality of life. We reviewed the impact of Fontan conversion with arrhythmia surgery and pacemaker therapy. METHODS: Thirty-eight patients underwent Fontan conversion using extracardiac conduit from 1992, and 22 patients with atrial arrhythmia underwent maze procedure simultaneously using cryoablation or radiofrequency ablation and epicardial DDD pacemaker implantation and 16 patients had regular 'sinus' rhythm before Fontan conversion. Mean follow-up period was 52 months. Pre- and postoperative clinical course were analyzed. Average weight, age at Fontan conversion, and years after first Fontan operation were 49.0 kg, 25.8 years old, 14.7 years, respectively. Nineteen percent of patients were in New York Heart Association class I (NYHA I), and 74% of patients were in NYHA II, and 7% were in NYHA III, respectively. RESULTS: Except three early deaths, actual survival rate at 1 year and 5 years were 80% and 64%, respectively. In survivors, 80% of the patients obtained regular heart rhythm including artificial pacemaker rhythm, although only 43% of the patients had regular 'sinus' rhythm before the Fontan conversion. Postoperative average cardiothoracic ratio and oxygen saturation (SpO(2)) were 50% and 94%, and 74% of patients were in NYHA I and 26% were in NYHA II, respectively, after Fontan conversion. CONCLUSIONS: Mid-term results of Fontan conversion with arrhythmia surgery and pacemaker therapy were acceptable. Restoration of regular rhythm might improve the postoperative NYHA status and the activity of the daily life.

Performance of CryoValve SG decellularized pulmonary allografts compared with standard cryopreserved allografts.

BACKGROUND: There is no ideal option for pulmonary valve replacement in children. Cryopreserved pulmonary allografts frequently demonstrate early valve regurgitation and may elicit an immune response. To improve these shortcomings, the SynerGraft process (CryoLife, Kennesaw, GA) decellularizes an allograft, leaving only connective tissue, which then becomes repopulated with host cells. A previous study at our institution demonstrated superior short-term durability of the SynerGraft-processed CryoValve SG compared with standard allografts. Longer-term impact of the technology remains unknown. METHODS: A single institution review was performed of all CryoValve SGs implanted between 2001 and 2004. Forty-one CryoValve SG patients and 41 age and diagnosis-matched standard allograft controls were evaluated. Demographics, survival, reintervention, and echocardiographic findings were analyzed. RESULTS: There were no significant differences between groups in demographics, valve diameter, orthotopic-heterotopic allograft position, or follow-up. For the entire cohort, there was no difference in early or late insufficiency or stenosis at a mean follow-up of 46 +/- 14 months. However, freedom from moderate to severe insufficiency (>3+) was significantly better for CryoValve SG patients (p = 0.05). In addition, for patients greater than 2 years of age, CryoValve SGs were significantly less regurgitant (p = 0.045) and stenotic (p = 0.041). Long-term survival was identical at 85% (35 of 41). CONCLUSIONS: When compared with standard allografts, CryoValve SGs demonstrate superior freedom from significant insufficiency at intermediate follow-up. In older children, CryoValve SGs display less insufficiency and stenosis. For infants, patient age, valve diameter, previous conduit, and rapid somatic growth would likely be the predominant factors leading to allograft failure.

Midterm clinical result of tissue-engineered vascular autografts seeded with autologous bone marrow cells.

OBJECTIVE: Prosthetic and bioprosthetic materials currently in use lack growth potential and therefore must be repeatedly replaced in pediatric patients as they grow. Tissue engineering is a new discipline that offers the potential for creating replacement structures from autologous cells and biodegradable polymer scaffolds. In May 2000, we initiated clinical application of tissue-engineered vascular grafts seeded with cultured cells. However, cell culturing is time-consuming, and xenoserum must be used. To overcome these disadvantages, we began to use bone marrow cells, readily available on the day of surgery, as a cell source. The aim of the study was to assess the safety and feasibility of this technique for creating vascular tissue under low-pressure systems such as pulmonary artery or venous pressure. METHODS: Since September 2001, tissue-engineered grafts seeded with autologous bone marrow cells have been implanted in 42 patients. The patients or their parents were fully informed and had given consent to the procedure. A 5-mL/kg specimen of bone marrow was aspirated with the patient under general anesthesia before the skin incision. The polymer tube serving as a scaffold for the cells was composed of a copolymer of l -lactide and -caprolactone (50:50). This copolymer is degraded by hydrolysis. The matrix is more than 80% porous, and the diameter of each pore is 20 to 100 microm. Polyglycolic acid woven fabric with a thickness of 0.5 mm was used for reinforcement. Twenty-three tissue-engineered conduits (grafts for extracardiac total cavopulmonary connection) and 19 tissue-engineered patches were used for the repair of congenital heart defects. The patients' ages ranged from 1 to 24 years (median 5.5 years). All patients underwent a catheterization study, computed tomographic scan, or both, for evaluation after the operation. The patients received anticoagulation therapy for 3 to 6 months after surgery. RESULTS: Mean follow-up after surgery was 490 +/- 276 days (1.3-31.6 months, median 16.7 months). There were no complications such as thrombosis, stenosis, or obstruction of the tissue-engineered autografts. One late death at 3 months after total cavopulmonary connection was noted in patient with hypoplastic left heart syndrome; this was unrelated to the tissue-engineered graft function. There was no evidence of aneurysm formation or calcification on cineangiography or computed tomography. All tube grafts were patent, and the diameter of the tube graft increased with time (110% +/- 7 % of the implanted size). CONCLUSION: Biodegradable conduits or patches seeded with autologous bone marrow cells showed normal function (good patency to a maximum follow-up of 32 months). As living tissues, these vascular structures may have the potential for growth, repair, and remodeling. The tissue-engineering approach may provide an important alternative to the use of prosthetic materials in the field of pediatric cardiovascular surgery. Longer follow-up is necessary to confirm the durability of this approach.

Extracardiac total cavopulmonary connection using a tissue-engineered graft.

OBJECTIVE: Extracardiac and lateral tunnel total cavopulmonary connection are currently 2 major options for patients with a single ventricle physiology. However, each procedure has some disadvantages over the other. We developed a new technique of extracardiac total cavopulmonary connection using a tissue-engineered graft to overcome some of the disadvantages previously associated with both the extracardiac and lateral tunnel procedures. METHODS: Between February 2001 and October 2002, 8 patients underwent an extracardiac total cavopulmonary connection using a tissue-engineered graft in our institution. Collected bone marrow cells (1 x 10(8) mononucleocytes) from a patient (approximately 1-4 mL/kg body weight) were seeded onto a biodegradable scaffold composed of polycaprolactone-polylactic acid copolymer reinforced with woven polylactic acid. After a 2- to 4-hour cultivation, the seeded scaffold was implanted as an extracardiac conduit during the total cavopulmonary connection operation. RESULTS: There were no hospital or late deaths. At a mean follow-up of 13.4 months (range 4-25 months), all patients are alive and asymptomatic with no need for repeat surgery. A postoperative catheter examination or computed tomography showed all tissue-engineered grafts to be patent and revealed no stenosis, obstruction, or aneurysmal change in the 8 patients. CONCLUSION: We believe that extracardiac total cavopulmonary connection using a tissue-engineered graft has the potential to overcome some of the disadvantages previously associated with extracardiac or lateral tunnel total cavopulmonary connection. However, an extended follow-up period is required to clarify the long-term clinical outcome for the tissue-engineered graft.