Evaluation of Explanted CorMatrix Intracardiac Patches in Children With Congenital Heart Disease

Animal data demonstrate that intracardiac patches of decellularized porcine small intestine submucosa (CorMatrix; CorMatrix Cardiovascular, Inc, Atlanta, GA) become repopulated with native cells, suggesting the possibility of a substrate for regenerative tissue in humans. We report the only prospective series to date of explanted CorMatrix patches placed in infants with congenital heart disease

Pediatric Giant Right Atrial Aneurysm: A Case Series and Review of the Literature

Giant right atrial aneurysm is a rare form of congenital heart disease with a wide spectrum of clinical presentation varying from asymptomatic patients to those with refractory atrial arrhythmias or severe airway obstruction. Diagnosis is often confused with other causes of right atrial dilation such as E bstein disease. Because of its rare occurrence and variable clinical presentation, inconsistencies in medical and surgical management strategies exist between centers. We present five cases of giant right atrial aneurysm managed at our institution and discuss the clinical presentation, diagnostic challenges, and medical and surgical management.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/107528/1/chd12079.pd

Long-segment thoracoabdominal aortic occlusions in childhood

Developmental coarctation, hypoplasia, and occlusion of the abdominal aorta is a rare disease encompassing many differing etiologies and diverse methods of treatment. Long-segment thoracoabdominal aortic occlusion, an extreme manifestation of this disorder, has not previously been reported in children. Two pediatric patients with this entity, a 5- and 13-year-old with uncontrolled hypertension, underwent extensive arterial reconstructions for this entity and provided the impetus for this report. An ascending thoracic aorta to infrarenal aortic expanded polytetrafluoroethylene bypass was undertaken in the younger child. A distal thoracic aorto-bi-iliac artery expanded polytetrafluoroethylene bypass, with implantation of the left renal artery to one graft limb and a right renal artery bypass originating from the other limb, was performed in the older child. There were no major perioperative complications. Both patients were discharged with easily controlled blood pressures. They have remained normotensive at 13 and 14 months follow-up

Association of Digoxin With Interstage Mortality: Results From the Pediatric Heart Network Single Ventricle Reconstruction Trial Public Use Dataset

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/139107/1/jah31279.pd

Outcomes of 1½- or 2-ventricle conversion for patients initially treated with single-ventricle palliation

ObjectiveAs outcomes for the Fontan procedure have improved, it has become more difficult to select between a single-ventricle repair or biventricular repair for patients with complex anatomy and 2 ventricles. However, late complications after the Fontan procedure remain a concern. Our strategy, which has favored an aggressive preferential approach for biventricular repair in these patients, has also been applied to patients initially treated on a single-ventricle track elsewhere.MethodsNine patients (4 male patients) who had previously undergone the Fontan procedure (n = 3) or bidirectional cavopulmonary shunting (n = 6) with intent for a later Fontan procedure were referred to our center for complex 1½- or 2-ventricle repair over the last 10 years. Indications for conversion in these patients were protein-losing enteropathy (n = 2), pulmonary arteriovenous malformation (n = 1), and preference for biventricular anatomy (n = 6). The conversion mainly consisted of takedown of the Fontan procedure or bidirectional cavopulmonary shunt connection, reconstruction of 1 or both of venae cavae, creation of an intraventricular pathway for left ventricular output, and placement of a right ventricle–pulmonary artery conduit (Rastelli-type operation).ResultsFive patients underwent 1½-ventricle repair, and 4 had complete biventricular repair. Median cardiopulmonary bypass and aortic crossclamp times were 202 minutes (range, 169–352 minutes) and 129 minutes (range, 100–168 minutes), respectively. There were 2 early deaths and 1 late death. At a median follow-up of 27 months (range, 3.3–99.8 months), all survivors are in New York Heart Association class I.ConclusionsPatients initially treated with intent to perform single-ventricle palliation can be converted to 1½- or 2-ventricle physiology with acceptable outcomes

Haematological and infectious complications associated with the treatment of patients with congenital cardiac disease: consensus definitions from the Multi-Societal Database Committee for Pediatric and Congenital Heart Disease

A complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention. Operative and procedural complications include both intraoperative/intraprocedural complications and postoperative/postprocedural complications in this time interval. The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has set forth a comprehensive list of complications associated with the treatment of patients with congenital cardiac disease, related to cardiac, pulmonary, renal, haematological, infectious, neurological, gastrointestinal, and endocrinal systems, as well as those related to the management of anaesthesia and perfusion, and the transplantation of thoracic organs. The objective of this manuscript is to examine the definitions of operative morbidity as they relate specifically to the haematological system and to infectious complications. These specific definitions and terms will be used to track morbidity associated with surgical and transcatheter interventions and other forms of therapy in a common language across many separate databases. The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has prepared and defined a near-exhaustive list of haematological and infectious complications. Within each subgroup, complications are presented in alphabetical order. Clinicians caring for patients with congenital cardiac disease will be able to use this list for databases, quality improvement initiatives, reporting of complications, and comparing strategies for treatmen

Biventricular repair for aortic atresia or hypoplasia and ventricular septal defect

AbstractObjective: Aortic valve atresia or hypoplasia can present with a ventricular septal defect and a normal mitral valve and left ventricle. These patients may be suitable for biventricular repair, although the optimal initial management strategy remains unknown. Methods: From January 1991 through March 1999, 20 patients with aortic atresia or hypoplasia and ventricular septal defect underwent operation with the intent to achieve biventricular repair. Aortic atresia was present in 7 patients, and aortic valve hypoplasia was present in 13 patients. Among those patients with aortic hypoplasia, Z-scores of the aortic valve anulus ranged from –8.8 to –2.7. Associated anomalies included interrupted aortic arch (n = 12 patients), coarctation (n = 6 patients), aortopulmonary window (n = 1 patient), and heterotaxia (n = 1 patient). Nine patients were staged with an initial Norwood procedure followed by biventricular repair in 8 patients. One patient awaits biventricular repair after a Norwood procedure. The conditions of 11 patients were corrected with a single procedure. Results: Among the 9 patients who underwent staged repair, there were no deaths after the Norwood procedure and 1 death after biventricular repair. For the 11 patients who underwent a primary biventricular repair, there was 1 early death and 2 late deaths from noncardiac causes. Follow-up ranged from 1 to 85 months (mean, 28 months). Actuarial survival for the entire group was 78% ± 10% at 5 years and was not significantly different between staged repair (89%) and primary biventricular repair (73%). Conclusions: Both primary and staged biventricular repair for patients with aortic atresia or hypoplasia and ventricular septal defect may be performed with good late survival. Refinements in technique of conduit insertion and arch reconstruction have resulted in primary biventricular repair becoming our preferred approach. (J Thorac Cardiovasc Surg 1999;118:648-54

Early Surgical Morbidity and Mortality in Adults with Congenital Heart Disease: The University of Michigan Experience

Objectives.  To review early surgical outcomes in a contemporary series of adults with congenital heart disease (CHD) undergoing cardiac operations at the University of Michigan, and to investigate possible preoperative and intraoperative risk factors for morbidity and mortality. Methods.  A retrospective medical record review was performed for all patients ≥18 years of age who underwent open heart operations by a pediatric cardiothoracic surgeon at the University of Michigan Congenital Heart Center between January 1, 1998 and December 31, 2004. Records from a cohort of pediatric patients ages 1–17 years were matched to a subset of the adult patients by surgical procedure and date of operation. Results.  In total, 243 cardiac surgical operations were performed in 234 adult patients with CHD. Overall mortality was 4.7% (11/234). The incidence of major postoperative complications was 10% (23/234) with a 19% (45/23) minor complication rate. The most common postoperative complication was atrial arrhythmias in 10.8% (25/234). The presence of preoperative lung or liver disease, prolonged cardiopulmonary bypass and aortic cross clamp times, and postoperative elevated inotropic score and serum lactates were significant predictors of mortality in adults. There was no difference between the adult and pediatric cohorts in terms of mortality and morbidity. Conclusions.  The postoperative course in adults following surgery for CHD is generally uncomplicated and early survival should be expected. Certain risk factors for increased mortality in this patient population may include preoperative presence of chronic lung or liver dysfunction, prolonged cardiopulmonary bypass and aortic cross-clamp times, and postoperative elevated inotropic score and serum lactate levels.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/75596/1/j.1747-0803.2008.00170.x.pd

PLZF-expressing CD4 T cells show the characteristics of terminally differentiated effector memory CD4 T cells in humans

In humans, promyelocytic leukaemia zinc finger (PLZF)+CD161+CD4 T cells develop in the fetal thymus [1] but these cells were not detectable in thymi obtained from children (11 days to 5.5 years) (Fig. 1A). However, we found PLZF+CD4 T cells within CD45RA+RO+CD4 T cells from tonsils and spleens but rarely in blood (Fig. 1B, left panel and 1C; Supporting Information Fig. 1A and B). PLZF expression was also confirmed by mRNA analysis in these cells from tonsils (Fig. 1B, right panel). CD8 T cells had a very low frequency of CD45RA+RO+ cells compared to CD4 T cells and these cells did not express PLZF (Fig. 1B). We also validated that CD45RA+RO+CD4 T cells are not mucosalassociated invariant T cells [2] (data not shown). PLZF-expressing NKT cells were excluded from all our analyses by CD1d- Tetramer staining (Supporting Information Fig. 2

Risk factors for hospital morbidity and mortality after the Norwood procedure: A report from the Pediatric Heart Network Single Ventricle Reconstruction trial

ObjectivesWe sought to identify risk factors for mortality and morbidity during the Norwood hospitalization in newborn infants with hypoplastic left heart syndrome and other single right ventricle anomalies enrolled in the Single Ventricle Reconstruction trial.MethodsPotential predictors for outcome included patient- and procedure-related variables and center volume and surgeon volume. Outcome variables occurring during the Norwood procedure and before hospital discharge or stage II procedure included mortality, end-organ complications, length of ventilation, and hospital length of stay. Univariate and multivariable Cox regression analyses were performed with bootstrapping to estimate reliability for mortality.ResultsAnalysis included 549 subjects prospectively enrolled from 15 centers; 30-day and hospital mortality were 11.5% (63/549) and 16.0% (88/549), respectively. Independent risk factors for both 30-day and hospital mortality included lower birth weight, genetic abnormality, extracorporeal membrane oxygenation (ECMO) and open sternum on the day of the Norwood procedure. In addition, longer duration of deep hypothermic circulatory arrest was a risk factor for 30-day mortality. Shunt type at the end of the Norwood procedure was not a significant risk factor for 30-day or hospital mortality. Independent risk factors for postoperative renal failure (n = 46), sepsis (n = 93), increased length of ventilation, and hospital length of stay among survivors included genetic abnormality, lower center/surgeon volume, open sternum, and post-Norwood operations.ConclusionsInnate patient factors, ECMO, open sternum, and lower center/surgeon volume are important risk factors for postoperative mortality and/or morbidity during the Norwood hospitalization