Samuel Weinstein

The use of the Berlin heart EXCOR in patients with functional single ventricle

INTRODUCTION The frequency and successful use of pediatric ventricular assist devices (VADs) as a bridge to cardiac transplantation have been steadily increasing since 2003, but the experience in patients with complex congenital heart disease has not been well described. Using a large prospectively collected dataset of children supported with the Berlin Heart EXCOR VAD, we have reviewed the experience in children with single ventricular anatomy or physiology (SV), and compared the results with those supported with biventricular circulation (BV) over the same time period. METHODS The EXCOR Investigational Device Exemption study database was retrospectively reviewed. VAD implants under the primary cohort and compassionate use cohort between May 2007 and December 2011 were included in this review. RESULTS Twenty-six of 281 patients supported with a VAD were SV. The most common diagnosis was hypoplastic left heart syndrome (15 of 26). Nine patients were supported after neonatal palliative surgery (Blalock-Taussig shunt or Sano), 12 after a superior cavopulmonary connection (SCPC), and 5 after total cavopulmonary connection (TCPC). Two patients received biventricular assist devices, 1 after stage I surgery and 1 after stage II. SV patients were supported for a median time of 10.5 days (range, 1-363 days) versus 39 days (range, 0-435 days) for BV (P = .01). The ability to be bridged to transplant or recovery in SV patients is lower than for BV patients (11 of 26 [42.3%] vs 185 of 255 [72.5%]; P = .001). Three of 5 patients with TCPC were successfully bridged to transplant and were supported with 1 VAD. Seven of 12 patients with SCPC were bridged to transplant, and only 1 of 9 patients supported after a stage I procedure survived. CONCLUSIONS The EXCOR Pediatric VAD can provide a bridge to transplant for children with SV anatomy or physiology, albeit less successfully than in children with BV. In this small series, results are better in patients with SCPC and TCPC. VAD support for patients with shunted sources of pulmonary blood flow should be applied with caution.

A Novel Bioresorbable Film Reduces Postoperative Adhesions After Infant Cardiac Surgery

BACKGROUND Adhesions encountered in reoperative cardiac surgery can prolong operating time and increase risk. This study was designed to evaluate the ability of a novel bioresorbable barrier film to reduce adhesions in infants. METHODS A comparative, evaluator-masked, randomized, multicenter study design was used. Before chest closure, infants undergoing initial sternotomy for eventual staged palliative cardiac operations were randomized to barrier film placement (n = 54) or control (no treatment, n = 49) at 15 centers. At repeat sternotomy 2 to 13 months later, the extent and severity of adhesions at the investigational surgical site (ISS) were assessed. A four-grade adhesion severity scoring system was standardized as follows: none, mild (filmy, noncohesive, requiring blunt dissection), moderate (filmy, noncohesive, requiring sharp and blunt dissection), and severe (dense, cohesive, requiring extensive sharp dissection). RESULTS There were significantly fewer patients with any severe adhesions (29.6% vs 71.4%, p < 0.0001), and a significantly lower percentage of the ISS had severe adhesion involvement (21.1 +/- 36.9% vs 49.5 +/- 42.7%, p = 0.0005) in the barrier group compared with the control group at the second sternotomy. Delayed chest closure (p = 0.0101), Norwood procedure (p = 0.0449), and cardiopulmonary bypass (p = 0.0001) were univariate risk factors for more severe adhesions. Multivariate analysis revealed only control group to be a significant risk factor for more severe adhesions (p = 0.003). There were no statistically significant differences in adverse events between the groups. No adverse events were definitely attributed to the study device. CONCLUSIONS Use of a novel bioresorbable film was safe and effective in reducing the extent and severity of postoperative adhesions in infants undergoing repeat median sternotomy.

Anti-factor Xa assay is a superior correlate of heparin dose than activated partial thromboplastin time or activated clotting time in pediatric extracorporeal membrane oxygenation*.

Objective: To assess the utility of activated clotting time, activated partial thromboplastin time, and anti-Factor Xa assay for the monitoring and dosing of heparin in pediatric patients requiring support with extracorporeal membrane oxygenation. Design: Retrospective chart review. Setting: PICU in a single, tertiary care, academic children’s hospital. Patients: Seventeen patients (age 1 d to 13.9 yr, median 0.83 yr) managed on pulmonary and cardiac extracorporeal membrane oxygenation between March 2010 and August 2012 by a single surgeon. Interventions: None. Measurements and Main Results: Twice daily measurements of anti-Factor Xa assay, activated clotting time, and activated partial thromboplastin time were determined from the same blood specimen. Data were analyzed using SAS system v9.2. Fourteen patients (82.4%) were successfully weaned from extracorporeal membrane oxygenation and 12 (70.6%) were discharged from the hospital. Pearson correlations were used to compare heparin dose and activated clotting time, activated partial thromboplastin time, and anti-Factor Xa assay. Analysis showed negative Pearson correlations in 11 of 17 patients between the activated clotting time and heparin, as compared with seven of 17 for activated partial thromboplastin time and only one for heparin and anti-Factor Xa assay. Only four patients had moderate to strong positive correlations between activated clotting time and heparin as compared with a moderate to strong positive correlation in 10 patients for anti-Factor Xa assay and heparin. Conclusions: The anti-Factor Xa assay correlated better with heparin dosing than activated clotting time or activated partial thromboplastin time. Activated clotting time has a poor correlation to heparin doses commonly associated with extracorporeal membrane oxygenation. In pediatric extracorporeal membrane oxygenation, anti-Factor Xa assay may be a more valuable monitor of heparin administration.

First reported use of the heartware HVAD in the US as bridge to transplant in an adolescent

D’Alessandro D, Forest SJ, Lamour J, Hsu D, Weinstein S, Goldstein D. First reported use of the heartware HVAD in the US as bridge to transplant in an adolescent.

Outcomes and risk factors for listing for heart transplantation after the Norwood procedure: An analysis of the Single Ventricle Reconstruction Trial

BACKGROUND Infants with hypoplastic left heart syndrome after palliation have the worst survival among heart transplant recipients. Heart transplantation is often reserved for use in patients with sub-optimal results after palliative surgery. This study characterized outcomes after listing in infants with a single ventricle who had undergone the Norwood procedure and identified predictors of the decision to list for heart transplantation. METHODS The public-use database from the multicenter, prospective randomized Single Ventricle Reconstruction trial was used to identify patients who were listed for heart transplantation. Outcomes on the waiting list and after transplantation were determined. Risk factors were compared between those who were listed and those who survived without listing. RESULTS Among 555 patients, 33 patients (5.9%) were listed and 18 underwent heart transplantation. Mortality was 39% while waiting for a heart and was 33% after heart transplantation. Overall, 1-year survival after listing (including death after transplantation) was 48%. Factors associated with listing were a lower right ventricular fractional area change at birth, non-hypoplastic left heart syndrome diagnosis, and a more complicated post-Norwood course, defined as a higher need for extracorporeal membrane oxygenation, longer intensive care unit stay, more complications, and a higher number of discharge medications. CONCLUSIONS Worse right ventricular function, non-hypoplastic left heart syndrome diagnosis, and complex intensive care unit stay were significant risk factors for listing for heart transplantation after the Norwood procedure. Heart transplantation as a rescue procedure after the Norwood procedure in the first year of life carries a significant risk of mortality.

Neonatal Myocardial Infarction: Case Report and Review of the Literature

Myocardial infarction in a neonate is rare. We describe the case of a full-term male who presented with respiratory distress. A chest radiograph demonstrated cardiomegaly. An electrocardiogram revealed ST segment changes suggestive of ischemia. Cardiac enzymes were elevated and an echocardiogram revealed a regional wall motion abnormality. Cardiac catheterization was performed demonstrating occlusion of the ramus intermedius branch of the left main coronary artery. The patient decompensated, requiring extracorporeal membrane oxygenation (ECMO). The infant was able to be decannulated from ECMO support in 5 days and was ultimately discharged on hospital day 25. We review this case as well as the literature on neonatal myocardial infarction.

MRI and computed tomography of cardiac and pulmonary complications of tetralogy of fallot in adults.

Tetralogy of Fallot (TOF) represents the most common form of cyanotic congenital heart disease, accounting for 6.8% of all congenital heart disease. As surgical techniques and medical management of patients with TOF have improved, most affected patients are reaching adulthood. Though surgical outcomes are favorable (<2% early mortality rate), adults with TOF may experience complications from the long-term sequelae of congenital heart disease and complications related to treatment. We describe common and uncommon findings in adults with TOF, including pulmonary insufficiency, central and peripheral pulmonary artery stenosis and aneurysms, in addition to graft and shunt-related complications. Pulmonary function abnormalities and lung parenchymal imaging findings will be detailed. The diagnostic value of computed tomography and magnetic resonance imaging in adults with complications of TOF will be illustrated.

Congenitally corrected transposition of the great arteries and concomitant coronary artery and valvular disease in the adult patient.

Congenitally corrected transposition of the great arteries (ccTGA) accounts for less that 1% of cardiac anomalies, and is defined as ventriculoarterial and atrioventricular (AV) discordance. The double discordant connection allows for survival with the right ventricle performing as the systemic ventricle, and the left ventricle as the pulmonary ventricle. We report a case of ccTGA in a 35-year-old male with situs inversus totalis status post repair of a ventricular septal defect (VSD) with a residual VSD, severe systemic AV valve regurgitation, and coronary artery disease who presented with chest pain. He subsequently underwent tricuspid valve replacement and VSD repair, followed by percutaneous coronary revascularization. This case highlights many important issues of adults with congenital cardiac disease, as well as the specific surgical management of anomalies associated with ccTGA. We review the literature and discuss the management of these complicated patients.

Forming a Consortium to Promote Pediatric Cardiac Care in a Developing Country

a a r A M S P D C he Federation of Pediatric Organizations has identified pediatric global health care as 1 of its 5 priority areas for the next 5 years. Consistent with this goal, there has een a growing effort to provide heart surgery for children in reas of the world where it has not yet been available. This sually takes the form of a medical group traveling to an undererved area and operating on small numbers of children. We escribe a collaborative effort among 4 charitable organizations o provide these requisite surgical and medical services, train the ocal medical team to operate independently year round, and ffer a global training experience to fellows and faculty based in he United States. The Variety Children’s Lifeline (VCL) was founded in 982 under the executive directorship of Salah M. Hassanein, ith the mission of sending teams to developing countries to erform heart surgery on impoverished children with no access o such care otherwise. Several other organizations provide care or children with congenital heart disease (CHD) in areas of eed throughout the world. For example, the Association of hildren with Heart Disease in the World sponsored 76 surgical issions in 12 developing countries and performed 412 operaions over a 10-year period. They stressed the importance of a oncomitant educational program with a partnering institution hat is committed to treating children with CHD over the long erm. Likewise, the Save a Child’s Heart Foundation was estabished to provide heart care to indigent children and is funded by oth government subsidies and private donors. This program, ased in Israel, has sent teams to 5 countries in Africa and also o China. Their model is a regional center to which children can e sent that also serves as a training center for medical personnel rom the host country. The difficulties encountered when attempting to establish uch cardiac care for children in the developing world have everal common themes. First is the high cost of heart surgery nd catheterization, with or without interventions. Most of these ountries struggle to fulfill even the basic needs of large segments f the population who live in poverty. High incidences of infec-

Tracheal rupture in complicated delivery: A case report and review of the literature

A case of distal tracheal rupture is described, literature review reveals two previously reported cases of neonatal distal tracheal rupture, as well as 14 cases of anterior subglottic rupture. All patients had shoulder dystocia, and 59% had associated brachial plexus injury. Delayed diagnosis (>3 days) was common in the distal tracheal group (66%), compared to 0% in the anterior subglottic group. The 2 distal tracheal rupture patients were initially managed conservatively, but ultimately required open repair. Distal tracheal rupture is exceedingly rare and more difficult to diagnose and manage than the more common anterior subglottic rupture.