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Background: We are developing a total cavopulmonary support system for failing Fontan physiology using the percutaneous Wang-Zwische double-lumen cannula (DLC). Methods: We developed a sheep model of failing Fontan physiology through a right thoracotomy in 5 sheep. An extracardiac conduit was anastomosed to the inferior vena cava and superior vena cava, and a graft was applied to connect the conduit and the right pulmonary artery (PA) to create total vena cava-to-PA diversion, excluding the right heart. The DLC (commercialized as AvalonElite, Avalon Laboratories LLC, Rancho Dominguez, CA) was coupled with a CentriMag pump (Levitronix Waltham, MA) to form a cavopulmonary support system. The DLC was inserted through the right jugular vein and the superior vena cava into the extracardiac conduit with the infusion lumen opening aligned with the right PA bridge. Blood was withdrawn from the superior vena cava and the inferior vena cava through the drainage lumen and pumped into the right PA through the infusion lumen, with flow adjusted to 4.0 ± 0.5 L/min. Results: A successful Fontan model was created without cardiopulmonary bypass. After total venous blood diversion from the vena cava to the right PA artery, failing Fontan physiology developed, evidenced by elevated central venous pressure and dropping mean PA pressure and systolic arterial pressure. The DLC was successfully inserted, and hemodynamics were normalized in all 5 sheep for the duration of the 2-hour study. Conclusions: We created a model of failing Fontan circulation in sheep without cardiopulmonary bypass. The DLC system achieved total cavopulmonary support for 2 hours in our failing Fontan sheep model. [Copyright &y& Elsevier]

Background: Pulmonary artery reconstruction remains a challenging clinical problem. We sought to evaluate the use of intraoperative stenting in conjunction with open heart surgical repairs. Methods: We retrospectively analyzed data from 22 patients between 1998 and 2003 who underwent intraoperative pulmonary artery stenting. Results: The mean age was 9.3 years (range, 9 months to 24 years). The left pulmonary artery was stented in 8 patients, the right in 13, the main in 1, and bilateral in 1. Available echocardiograms in 20 patients revealed a mean preoperative diameter of 7.6 mm (range, 3 to 16.5 mm) and a mean peak gradient of 45.4 mm Hg (range, 20 to 120 mm Hg). Augmentation patch angioplasty of the contralateral pulmonary artery was performed in 11 patients. Thirteen patients had pulmonary valve replacement and 11 had conduit replacement or augmentation. Three patients underwent tricuspid valve repair, 6 had Fontan, and 4 underwent a modified Maze procedure. Follow-up echocardiograms demonstrated a decrease in mean peak gradient to 4.3 mm Hg (range, 0 to 15 mm Hg), a change in mean peak gradient of 41.1 mm Hg (93% reduction). After stenting the mean pulmonary artery diameter increased to 10.9 mm (range, 6 to 17.9 mm). There was no perioperative mortality. At a mean follow-up of 22.8 months (range, 6 to 57 months), there were no surgical or percutaneous reinterventions. Conclusions: Intraoperative pulmonary artery stenting is a safe and effective technique that can be used as an alternative to patch angioplasty. Close follow-up is needed to examine the long-term outcome of this procedure, with special attention to the growth and development of the stented vessel. [Copyright &y& Elsevier]

Background: We sought to validate a simple grading scheme for right ventricular hypoplasia in determining suitability for a biventricular repair. Methods: We reviewed the medical records for 106 patients with pulmonary atresia-intact ventricular septum (PA-IVS) treated between 1982 and 2001. Over this period, children were assigned to mild (>2/3 normal size, 23.7% of patients), moderate (1/3 to 2/3, 41.2%), or severe (1/3, 35.1%) right ventricular hypoplasia, and this grouping, along with severity of coronary anomalies (45% right ventricle to coronary fistulae, 16% with right ventricle dependent coronary circulation [RVDCC]), triaged children to eventual single ventricle (severe) or two-ventricle (mild or moderate) repair. Results: Actuarial 10-year survival was 86.3% with mortality predicted by severe hypoplasia (odds ratio [OR] 12.9, p < 0.001), RVDCC (OR 15.0, p < 0.001), and non-Caucasian race (OR 10.7, p < 0.001). Multivariate analysis with a Cox proportional hazards model confirmed only RVDCC (risk ratio [RR] 10.9, p = 0.0009} and non-Caucasian race (RR 6.9, p = 0.007) as significant. Although not an independent risk factor for survival, the degree of hypoplasia was the most important determinant for definitive repair. Severe hypoplasia virtually precluded two-ventricle repair (OR 33.1, p < 0.001 by χ2 analysis) and was the strongest risk factor for a one-ventricle system (OR 78.7, p < 0.001). Actuarial survival after either repair was 91%, and no biventricular repair later converted to a Fontan system. Conclusions: Surgical management of patients based on this three tier grade for right ventricular hypoplasia results in excellent survival and correctly predicts patients destined for eventual Fontan and biventricular repair. [Copyright &y& Elsevier]

: BackgroundHeart transplantation is the most accepted treatment for end-stage heart disease. A review of 1,083 consecutive transplants (1984 to 2001) was undertaken.: MethodsAdult recipients were divided into quartiles. The last 540 transplants were combined. Three eras were created from these, 1984 to 1991, 1991 to 1995, and 1995 to 2001, with three age groups: 0 to 18 years, 19 to 61 years, and 62 to 74 years. All patients have at least 1 year of follow-up time. End points were survival, rejection, and graft coronary artery disease.: ResultsThere were 1,012 patients. Donor age, graft ischemic time, and the proportion of elderly recipients and nonstandard donor hearts have increased in the current era. Actuarial 60-month survivals of recipients after 1995 were 80.7% (0 to 18 years); 75.3% (19 to 61 years); and 76.2% (>62 years). The current era children and younger adult groups demonstrated improved results when compared with previous eras (p = 0.003 and p = 0.05). Rejection episodes equal to or greater than ISHLT grade 3A per person per year improved to 0.15 in the current era (p < 0.001). During the three eras, older recipients (>62 years) demonstrated fewer episodes of rejection when compared with other adults (0.13 versus 0.58, p = 0.03). Deaths attributed to graft coronary artery disease decreased from 11% to 5% from era 2 to era 3. Regression analysis revealed a mild effect of donor age on survival and graft coronary artery disease (hazard ratio = 1.02, p = 0.001; hazard ratio = 1.039, p < 0.001, respectively). Recipient predictors of graft coronary artery disease were diagnosis of ischemic cardiomyopathy (hazard ratio = 1.6, p = 0.014) and congenital heart disease (hazard ratio = 3.41, p = 0.02).: ConclusionsImproved survival in the current era may be attributed to better organ preservation, improved immunosuppression and control of infection, and less life-threatening graft coronary artery disease. [Copyright &y& Elsevier]

We report on an acute myocardial injury caused by thrombotic emboli during off-pump bilateral sequential lung transplantation in a 15-year-old female patient with end-stage cystic fibrosis. A few minutes after reperfusion of the right (second sequential) allograft, the patient developed hemodynamic collapse, including hypotension, acute akinesis of the anterior and lateral myocardial walls, and severe mitral regurgitation. Thrombus was noted within the left atrium and ventricle on intraoperative transesophageal echocardiogram. After emergent cannulation for cardiopulmonary bypass, the patient underwent left cardiac catheterization with AngioJet rheolytic thrombectomy and removal of the clot from the distal left anterior descending coronary artery. Reperfusion was established without the need for stent placement or further intervention. The patient required short-term life support with venoarterial extracorporeal membrane oxygenation. [ABSTRACT FROM AUTHOR]

Background: Management of a ventricular septal defect (VSD) at time of coarctation of the aorta (CoA) repair remains controversial, with recent studies advocating concomitant repair of both defects. We evaluated the surgical management and mortality for patients undergoing CoA repair associated with a VSD., Methods: We retrospectively reviewed data submitted to the Pediatric Cardiac Care Consortium of patients undergoing repair of CoA from 1982 to 2007. The cohort was divided into three groups: CoA repair plus VSD closure (group 1); CoA repair plus pulmonary artery band (group 2); and CoA repair without repair of VSD (group 3). Variables reviewed included era, age, and weight at repair, and in-hospital mortality., Results: There were 7,860 patients who underwent repair of CoA, of whom 2,022 had an associated VSD (25.7%). Mortality after CoA repair with and without an associated diagnosis of VSD was 8.3% versus 2.1% (p < 0.001). Mean age at repair for group 1 (n = 286) and group 2 (n = 472) was 87.4 days and 21.6 days, respectively (p = 0.004), and median weight was 3.31 kg and 3.30 kg, respectively (p = 0.130). Discharge mortality for group 1 and group 2 was similar, at 8.7% and 9.1%, respectively (p = 0.852). Patients with CoA/VSD who had neither VSD closure nor pulmonary artery banding (group 3) had a hospital mortality of 7.9%., Conclusions: The association of CoA and VSD is common. A strategy of concomitant VSD closure at CoA repair does not result in worse discharge mortality when compared with pulmonary banding with anticipated staged repair of the VSD. These outcomes support continued evaluation of a one-stage approach., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)