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Background Presentation in shock and preoperative infection remain risk factors for neonatal cardiac surgery. This report describes bilateral pulmonary artery banding (bPAB) in ductal-dependent lesions with systemic outflow obstruction as rescue intervention before surgery with cardiopulmonary bypass in these high-risk neonates. Methods A retrospective chart review was conducted for 10 patients who underwent bPAB before conventional surgery with cardiopulmonary bypass. Patient characteristics including birth weight, gestational age, cardiac and noncardiac diagnoses, preoperative and postoperative markers of organ function, and outcome measures were examined. Results The majority of patients (8 of 10) were considered high-risk owing to multiorgan dysfunction syndrome. The median age at bPAB was 12 days (range, 5 to 26 days), and the median interval between bPAB and second surgery was 10.5 days (range, 5 to 79 days). Organ function improved after admission and continued to improve after bPAB in 9 of 10 patients. No patient experienced new complications between bPAB and subsequent operation. Of 8 patients who had stage I palliation, 5 have undergone or are awaiting completion Fontan, 1 underwent Kawashima procedure, 1 underwent orthotopic heart transplant, and 1 with hypoplastic left heart syndrome and intact atrial septum died at 44 days old. Both patients who underwent biventricular repair are alive and well. Median follow-up for survivors was 2.9 years (range, 0.25 to 6.25 years). Conclusions Bilateral pulmonary artery banding is safe in ductal-dependent lesions with systemic outflow obstruction. High-risk patients with preoperative organ dysfunction or infection can recover within a short period and become lower risk candidates for complex congenital heart surgery using cardiopulmonary bypass.

It is now recognized that a majority of single ventricle patients, those with functionally univentricular hearts, who have survived palliative cavopulmonary connection will experience circulatory failure and end-organ dysfunction due to intrinsic inadequacies of a circulation supported by a single ventricle. Thus, there are an increasing number of patients with functional single ventricles presenting with failing circulations that may benefit from mechanical circulatory support (MCS). The paucity of experience with MCS in this population, even at high volume cardiac centers, contributes to limited available data to guide MCS device selection and management. Thus, a registry of MCS in this population would be beneficial to the field. A conference was convened in January 2012 of pediatric and adult cardiologists, pediatric cardiac intensivists, congenital heart surgeons, and adult cardiothoracic surgeons to discuss the current state of MCS, ventricular assist device, and total artificial heart therapy for patients who have undergone cavopulmonary connection, either superior cavopulmonary connection or total cavopulmonary connection. Specifically, individual experience and challenges with VAD therapy in this population was reviewed and creation of a multiinstitutional registry of MCS/ventricular assist device in this population was proposed. This document reflects the consensus from the meeting and provides a descriptive overview of the registry referred to as Mechanical Support as Failure Intervention in Patients with Cavopulmonary Shunts.

Summary Background Pediatric congenital heart disease patients are at risk for vascular injuries during surgical procedures or when the arterial system is accessed for monitoring or diagnostic studies. Our treatment of emergent situations in this patient population using microvascular techniques shows the feasibility of such techniques. Methods A retrospective chart review of patients aged 0–18 years with congenital heart disease identified six patients who underwent microvascular surgery by the senior surgeon from June 2007 to May 2015. We studied this series, highlighting technical aspects of surgery and perioperative care to determine their effect on outcome. Results Six patients with congenital cardiac defects requiring cardiothoracic surgery were studied, body weight ranging from 3.2 to 19.1 kg at the time of surgery. Five suffered iatrogenic arterial injury to the heart or vessels used for access or diagnostic studies, including coronary artery laceration, brachial artery thrombosis, and external iliac artery avulsion. Interventions included direct end-to-end repair and vein grafting. Vessel diameter averaged 1 mm. Patients received vasopressors intraoperatively and were on vasopressors and antihypertensives postoperatively. One patient died because of disseminated intravascular coagulation on postoperative day 17, but bypass graft was patent prior to death. The rest survived with clinical evidence of patency of the repaired vessel for a long-term. Conclusions Microsurgical intervention may be life-saving as a revascularization procedure to the heart by direct coronary repair or bypass grafting. Iatrogenic injuries to the limb may cause critical ischemia; limbs can be salvaged by microsurgical repair. Despite technical and physiological challenges, microsurgery is feasible and sometimes crucial in this patient population.

We present a case of a 39-year-old woman with scimitar syndrome who had a 2-patch repair 3 decades previously and presented with a right-to-left shunt of the inferior vena cava (IVC) to the left atrium resulting from baffle dehiscence. We discuss details of our reoperative repair.

We aim to describe the clinical course of a series of patients with hypoplastic left heart syndrome and refractory systolic heart failure supported with a HeartWare ventricular assist device (HVAD) following Fontan palliation. This is a retrospective review of three consecutive patients supported with a HVAD following Fontan palliation through February 2016. Data include patient characteristics, operative variables, postimplantation hemodynamic/device parameters, event outcomes, and duration of HVAD support. Patient ages were 11.7, 13.5, and 17.5 years, respectively, at the time of HVAD implant. The duration of HVAD support was 148, 272, and 271 days, respectively, of which 86, 222, and 211 were outpatient days. Inflow cannula position was the morphologic right ventricle with depth adjustment and manipulation of the tricuspid subvalvar apparatus to ensure good inflow. Echocardiographic, hemodynamic, and noninvasive oximetric monitoring resulted in high RPM settings for all patients. Despite various complications, all patients were successfully transplanted and discharged home alive. We present three patients bridged to transplantation using the HVAD following Fontan palliation. We demonstrate potential for durable support with transition to outpatient care while awaiting heart transplantation in a subset of patients status post Fontan surgery.

To describe the management of a giant cardiac malignancy initially diagnosed as an anterior mediastinal mass.A nine-year-old female with right facial swelling and chronic cough was diagnosed with a large right mediastinal mass. Intermittent ventricular and supraventricular arrhythmias were noted on admission electrocardiograms. Empiric corticosteroid and radiation therapy did not reduce the size of the tumour, and initial tissue biopsies were non-diagnostic. Due to worsening tamponade physiology and persistent arrhythmias, the patient was scheduled for tumour debulking with potential resection. Prior to surgery, a multidisciplinary team was assembled to delineate team member responsibilities and treatment algorithms. The procedure was performed under general anesthesia with spontaneous ventilation preserved during endotracheal intubation and invasive line placement. The team was prepared to provide extracorporeal mechanical support if needed. The child required inotropic and vasoactive medications after transitioning to positive pressure ventilation, but her hemodynamics improved with sternotomy. The lesion was identified as a malignant cardiac clear-cell tumour that was unresectable. Her sternum was left open, as attempted closure led to the re-creation of tamponade physiology. She underwent delayed sternal closure days later. After months of chemotherapy that resulted in significant tumour involution, she underwent successful surgical resection.Giant primary cardiac tumours may present similarly to large anterior mediastinal masses. The care of patients with these lesions requires an understanding of the risks associated with mediastinal masses as well as those unique to cardiac tumours. A multidisciplinary approach is critical to providing safe and effective care throughout this process.

Our objective is to describe the use of a ventricular assist device (VAD) in single-ventricle patients with circulatory failure following superior cavopulmonary anastomosis (SCPA). We performed a retrospective chart review of all single-ventricle patients supported with a VAD following SCPA. Implantation techniques, physiologic parameters while supported, medical and surgical interventions postimplant, and outcomes were reviewed. Four patients were supported with an EXCOR Pediatric (Berlin Heart Inc., The Woodlands, TX, USA) following SCPA for a median duration of 10.5 days (range 9-312 days). Selective excision of trabeculae and chords facilitated apical cannulation in all patients without inflow obstruction. There were two pump exchanges in the one patient supported for 312 days. Two patients were evaluated by cardiac catheterization while supported. Three of four patients were successfully bridged to transplantation. One patient died while supported. All patients had significant bleeding at the time of transplantation, and one required posttransplant extracorporeal membrane oxygenation with subsequent full recovery. VAD support can provide a successful bridge to transplantation in patients with single-ventricle circulation following SCPA. A thorough understanding of the challenges encountered during this support is necessary for successful outcomes.

Background/Purpose: Laparoscopic Nissen fundoplication (LNF) is being utilized more extensively in the management of symptomatic gastroesophageal reflux disease in infants and children. The traditional approach utilizes 5 3- to 5-mm cannulas for telescope and instrument access to the peritoneal cavity. The purpose of this study is to report the technique and document the results using a single 5-mm umbilical cannula for LNF, stab incisions for placement of the instruments, and robotic telescope assistance. Methods: From November 1999 through March 2002, 154 patients underwent LNF by the senior author for pathologic gastroesophageal reflux disease. All operations were performed with a single 5-mm umbilical cannula through which a 4- or 5-mm telescope was placed for operative visualization. Four stab incisions were made through the upper/lateral abdominal wall under direct visualization avoiding the epigastric vessels. Through these stab incisions, instruments were inserted into the peritoneal cavity. The maximum insufflation pressure was 15 mm Hg in all cases. The ability to perform the procedure in the absence of additional operative cannula placement, complications during instrument insertion, the ability to maintain adequate pneumoperitoneum, the patient's age, weight, operating time, and the addition of a gastrostomy were recorded. Results: All but one of the 154 LNFs were completed successfully using this technique. The mean age at operation and mean operating time was 23.9 months (range, 3 weeks to 180 months) and 91 minutes (31 to 160 minutes), respectively. Patients weight ranged from 2.4 to 57 kg (mean, 10.4 kg). Gastrostomies were placed in 52 cases. There were no complications associated with the stab incisions or insertion of the operative instruments through the abdominal wall. Pneumoperitoneum was maintained adequately in all cases. Conclusions: LNF can be performed safely and effectively with a single umbilical cannula. We recommend its use for pediatric patients who require LNF. J Pediatr Surg 38:111-115. Copyright 2003, Elsevier Science (USA). All rights reserved.

A 20-year-old man with a temporary epicardial pacing wire retained after infant repair of an atrioventricular septal defect presented with cardiopulmonary arrest. After resuscitation with extracorporeal membranous oxygenation, a coronary angiogram demonstrated a retained epicardial pacing wire compressing the left anterior descending artery and obtuse marginals. Owing to poor prognostic findings on a computed tomography scan of his head, he was terminally withdrawn from mechanical support. This case demonstrates a potential devastating complication of a retained temporary epicardial pacing wire.

Transapical-to-aorta double lumen cannula inserted from apex into left ventricle-aorta for a less-invasive and more reliable left ventricular assist device.

The externally supported Ross (supported Ross), consisting of a Dacron (DuPont, Wilmington, DE) graft to support the neoaortic root, has been used in adolescent and adult patients to prevent neoaortic dilatation. Outcomes after the supported Ross technique were compared with the Ross procedure using the standard aortic root replacement technique (standard Ross).This was a retrospective analysis of 36 adolescent and young adult patients who underwent the Ross procedure between 1992 and 2013. The outcomes of supported Ross procedures in 26 patients were compared with the Ross procedure in 10 patients. End points included survival, neoaortic root dilatation, development of neoaortic regurgitation, and the need for reintervention.The median age at operation was 14 years (range, 11 to 31 years), and indications for the operation were mixed lesions (47%), followed by aortic regurgitation (42%) and stenosis (11%). There were no early deaths. The mean follow-up was 2.2 years (range, 1 to 11 years). At the 1-year (p = 0.01) and 3-year (p0.05) follow-up, patients in the supported Ross cohort had a smaller neoaortic root z-score. Neither cohort had a large number of patients with significant neoaortic regurgitation, with 1 patient in the supported cohort compared with 3 patients in the standard cohort. Overall, 4 patients (40%) in the standard Ross cohort had required reintervention, including 3 directed at the neoaortic root. One patient in the supported Ross cohort required early reintervention for revision of the right coronary artery.At intermediate follow-up, patients who underwent the supported Ross technique were less likely to have neoaortic root dilatation compared with patients who underwent a standard Ross procedure. Further studies are needed to evaluate the long-term durability of this technique, particularly in regards to the development of significant aortic regurgitation, the rate of reintervention, and application to younger and smaller patients.

Cor triatriatum dexter is a rare congenital heart defect that can lead to cyanosis in a newborn with an otherwise normal exam. The initial evaluation of these patients typically focuses on searching for a pulmonary etiology for arterial desaturation, which often leads to a negative work up. When cardiac evaluation is performed, it may be challenging because the heart lesion can be difficult to visualize on an echocardiogram. The diagnosis requires a high index of suspicion and thorough echocardiographic imaging. Once diagnosed, surgical repair can alleviate the shunt created by the defect. This case series describes all patients (3) with cor triatriatum dexter seen at Children's Hospital of Wisconsin from 2000 to 2013.

Background: This study evaluates the relationship of morbidity and resource utilization with the timing of early neonatal repair of transposition of the great arteries and intact ventricular septum (d-TGA/IVS). Methods: All patients with d-TGA/IVS who underwent arterial switch in the first 14 days of life, between January 2000 and May 2011, were reviewed. Patients undergoing repair at ≤4 days of age were categorized as group I, 5 to 7 days as group II, and 8 to 14 days as group III. Outcomes included mortality, morbidity, and resource utilization. Results: Hospital survival was 69 (98.6%) of 70. The length of stay (LOS) and total charges were lowest in group I—15.5 days compared to group II—18.0 days and group III—23.5 days ( P = .005); group I—US$128,219 compared to group II—US$141,729 and group III—US$217,427 ( P = .0006). Using regression analysis to account for potentially confounding effects of multiple variables and treating time as a continuous variable demonstrated that age at surgery was significantly associated with total LOS ( P = .029), hospital charges ( P = .029) and intensive care unit charges ( P = .002). Younger age at repair was not associated with worse outcomes for any measure of morbidity. Conclusions: Earlier repair of d-TGA/IVS was associated with decreased resource utilization and no detriment to clinical outcomes. Further analysis based on a larger cohort of patients is needed to verify these results that have important implications for improving the value of care.

Pediatric patients supported on ventricular assist devices (VADs) require systemic anticoagulation and are at risk for intracranial hemorrhage (ICH). Little is known about the incidence or outcomes of pediatric patients with ICH while supported on a VAD. A retrospective chart review of all patients receiving VAD support was completed. Patients diagnosed with ICH while supported on a VAD were identified. Significant factors prior to diagnosis of ICH, medical/surgical treatment of ICH, and patient outcomes were assessed. Five of 30 (17%) patients supported on a VAD from January 2000 to November 2012 were diagnosed with an ICH. Four patients had an identified cerebral thromboembolic injury prior to the ICH. Four patients required interruption in their anticoagulation regimen due to other bleeding concerns prior to ICH. Neurosurgical intervention consisted of evacuation of hemorrhage in one, whereas two others required management of hydrocephalus with external ventricular drainage. Three of the five patients died on VAD support. Two deaths were directly related to ICH, whereas the third was unrelated. Two patients were successfully transplanted; one remains with a significant neurological impairment, and the other has recovered with minimal residual impairment following neurosurgical evacuation of a large subdural hematoma. ICH is a devastating complication of VAD support. Prior ischemic infarcts and interruptions to anticoagulation may put a patient at risk for ICH. Prompt neurosurgical evaluation/intervention can result in positive outcomes.

Primary cardiac sarcomas are rare and carry a poor prognosis. The standard of care is complete resection. Outcomes for patients without complete resection are dismal, and the benefit of adjuvant therapy is uncertain. A 9-year-old female presented with a large right-sided cardiac mass. Following biopsy, the tumor was classified as an undifferentiated sarcoma. Resection was not feasible due to apparent invasion of the right ventricle and atrioventricular groove. Treatment with oral etoposide resulted in a 97% reduction in tumor volume and allowed for complete resection of residual tumor. She is alive with no evidence of disease 25 months from diagnosis.

Objective Experiences of mothers of infants undergoing complex heart surgery were explored to build evidence-based family-centered interventions. Background Congenital heart disease is the most frequent birth defect in the United States and is common worldwide. Methods Eight mothers recalled through journal entries their experiences of the days before, during, and after their infant's surgery and shared advice for other mothers. Colaizzi's phenomenological method was utilized for data analysis. A validation survey of seven additional mothers from a support group occurred via email. Results Six themes were identified and validated: Feeling Intense Fluctuating Emotion; Navigating the Medical World; Dealing with the Unknown; Facing the Possibility of My Baby Dying, Finding Meaning and Spiritual Connection, and the umbrella theme of Mothering Through It All. Conclusions Through a clearer understanding of experiences as described by mothers, health-care providers may gain insight as to how to better support mothers of infants undergoing heart surgery.

There are a wide variety of reported techniques with few comparative trials and no current data available by which surgeons can compare their myopreservation strategies across the specialty. We therefore surveyed congenital heart surgeons to develop a profile of current practice.One hundred twenty-two members of the Congenital Heart Surgeons' Society were surveyed, and 56 responses were analyzed. The survey focused on cardioplegia formulations, dosage and administration, and perfusion strategies for four age groups: neonates, infants, children, and adolescents. All percentages are expressed as percentage of the entire reporting cohort (n=56).Eighty-six percent of surgeons use blood-based cardioplegia versus crystalloid cardioplegia. Microplegia is used in 5%. Blood-based cardioplegia additives include del Nido (38%), customized solutions (32%), St. Thomas, Plegisol, or Baxter (11%), and microplegia (5%). Crystalloid cardioplegia types are Custodiol (7%), St. Thomas, Plegisol, or Baxter (5%), and customized solutions (2%). Cold (10°C) cardioplegia is most common (93%), and "hot shots" are used in 21%. Moderate (26° to 30°C) hypothermic cardiopulmonary bypass is more common in neonates and infants compared with older children and adolescents. Antegrade administration is most common (89%). Longer intervals between cardioplegia doses were associated with surgeons using del Nido and Custodiol solutions, and these solutions were commonly administered with a single dose regardless of aortic cross-clamp time.Myocardial protection techniques still remained highly variable among congenital heart surgeons. This survey demonstrates that there is a perception that del Nido and Custodiol solutions can offer appropriate myocardial protection for longer intervals with decreased repeat dosing. An observational study correlating markers of postoperative myocardial performance with myocardial preservation strategies should be considered.

Our aim is to develop and validate an accurate method for estimating TCV using standard echocardiographic imaging that can be easily employed to aid in donor-recipient size matching in pediatric heart transplantation. Thirty patients who underwent Echo and cardiac magnetic resonance imaging (cMRI) were identified. TCV was measured on cMRI. TCV was determined echocardiographically by two methods: a volume measurement using the modified Simpson's method on a four-chamber view of the heart; and a calculated volume measurement which assumed a true-elliptical shape of the heart. These two methods where compared with the value obtained by cMRI using the concordance correlation coefficient (CCC). TCV by method 1 correlated well with cMRI (CCC = 0.98%, CI = 0.97, 0.99). TCV by method 2 had a CCC = 0.90 (CI = 0.9464, 0.9716) when compared to cMRI. Left ventricular end-diastolic volume (LVEDV) also correlated as a predictor of TCV in patients with structurally normal hearts and could be described by the equation: TCV = 6.6 (LVEDV) + 12 (R(2) = 0.97). Echocardiographic assessment of TCV for recipients and their potential donors is a simple process and can be prospectively applied as part of donor evaluation.

It is now recognized that a majority of single ventricle patients, those with functionally univentricular hearts, who have survived palliative cavopulmonary connection will experience circulatory failure and end-organ dysfunction due to intrinsic inadequacies of a circulation supported by a single ventricle. Thus, there are an increasing number of patients with functional single ventricles presenting with failing circulations that may benefit from mechanical circulatory support (MCS). The paucity of experience with MCS in this population, even at high volume cardiac centers, contributes to limited available data to guide MCS device selection and management. Thus, a registry of MCS in this population would be beneficial to the field. A conference was convened in January 2012 of pediatric and adult cardiologists, pediatric cardiac intensivists, congenital heart surgeons, and adult cardiothoracic surgeons to discuss the current state of MCS, ventricular assist device, and total artificial heart therapy for patients who have undergone cavopulmonary connection, either superior cavopulmonary connection or total cavopulmonary connection. Specifically, individual experience and challenges with VAD therapy in this population was reviewed and creation of a multiinstitutional registry of MCS/ventricular assist device in this population was proposed. This document reflects the consensus from the meeting and provides a descriptive overview of the registry referred to as Mechanical Support as Failure Intervention in Patients with Cavopulmonary Shunts.

Objectives The purpose of the present study was to evaluate the association of open and closed Fontan fenestration status with event-free survival. Methods All patients who underwent a fenestrated Fontan procedure at our institution from January 1994 through June 2007 were reviewed. Patient information was obtained from the medical records. The patients were assigned to 1 of 2 study groups, open or closed, according to their most recent fenestration status. Clinically relevant morbid events were tabulated, and Kaplan-Meier event analysis was used to create event-free probability curves with log-rank comparisons. Results A total of 161 patients were classified as open and 51 as closed. The median interval to an event was 1.1 years (interquartile range, 0.1-3.3 years) after the Fontan procedure. The median interval to closure was 1.2 years (interquartile range, 0.7-3.3 years). The median interval to an event was 1.5 years (interquartile range, 0.1-4.6 years) in the closed group and 1.1 years (interquartile range, 0.1-3.3 years) in the open group. Event-free probability analysis revealed no significant difference between the 2 groups ( P = .15). The median follow-up arterial oxygen saturation was greater in the closed group (96.0%; interquartile range, 94.0%-97.0%) than in the open group (91.0%; interquartile range, 86.0%-95.0%; P Conclusions Fenestration closure was associated with greater arterial oxygen saturation but not greater event-free survival. The interval to an event was slightly less than the interval to fenestration closure, suggesting potential merit in the evaluation of earlier fenestration closure. Adoption of specific fenestration management guidelines might help improve the overall outcomes and enhance the quality of future studies.

Objective We sought to describe early outcomes of aortic valve replacement in neonates and infants across a large multicenter cohort. Methods Neonates and infants in the Society of Thoracic Surgeons Congenital Heart Surgery Database undergoing nontruncal aortic valve replacement with the Ross-Konno procedure, Ross procedure, or homograft replacement from 2000 to 2009 were included. Preoperative characteristics, operative data, and early outcomes are described. Results A total of 160 patients (43 neonates, 117 infants) from 47 centers were included. Society of Thoracic Surgeons–defined preoperative risk factors were present in 76 patients (48%) and were most prevalent in neonates (67%) and patients undergoing homograft aortic valve replacement (93%). Concomitant arch repair or mitral valve surgery was performed in 30 patients (19%) and 19 patients (12%), respectively. Postoperative mechanical circulatory support was used in 17 patients (11%). Overall in-hospital mortality was 18% and was highest for neonates (28%) and patients undergoing homograft aortic valve replacement (40%). Concomitant arch repair was associated with higher in-hospital mortality (33% vs 15%, P = .02), whereas concurrent mitral valve surgery was not (21% vs 18%, P = .73). Postoperative mechanical circulatory support was also associated with increased in-hospital mortality (65% vs 13%, P Conclusions Neonates and infants undergoing aortic valve replacement are a high-risk group, with hospital mortality comparable with some of the highest risk procedures in this age group. The requirement for arch repair or postoperative mechanical circulatory support was associated with an increased risk of death in this cohort.

Background We reviewed our experience with valve-sparing aortic root reconstruction (VSARR) using the sinus of Valsalva graft in children, teenagers, and young adults with connective tissue disorders. Methods Results of a single-center experience with VSARR in children, teenagers, and young adults were retrospectively analyzed. End points were death, freedom from reintervention, and freedom from valve dysfunction. Results Between 2003 and 2010, 16 patients (Marfan, 9; Loeys Dietz syndrome, 6; conotruncal, 1) underwent VSARR. The mean age was 20 ± 7.4 (range, 9 to 36 years). Indications for VSAAR were aortic root enlargement in 14 (sinus of Valsalva Z-score, 6.2 ± 2) and aortic dissection in 2. Additional procedures included replacement of the ascending aorta in 7, with additional replacement of the aortic arch in 2. No early or late deaths occurred. One patient required a pacemaker. One patient with Loeys-Dietz syndrome required reoperation for aneurysmal dilatation of the coronary buttons. Two patients underwent replacement of the thoracoabdominal aorta for chronic dissection. Follow-up by echocardiography or magnetic resonance imaging at a mean of 33 ± 29 months showed more than mild aortic regurgitation in 2 patients. Both patients with moderate aortic insufficiency also had a bicuspid aortic valve. Conclusions VSARR using the sinus of Valsalva graft is a reproducible technique that achieves acceptable early and intermediate results. It is suitable for children, teenagers, and young adults. Anticoagulation is avoided. The procedure is appropriate for emergency operations but should be used with caution in patients with a bicuspid aortic valve.

We present a successful case of the use of a HeartWare ventricular assist device as a bridge to transplantation in an 11-year-old with a hypoplastic left heart and failed Fontan circulation.

We report an alternative technique for achieving hemostasis in the conversion from bypass to post-repair mechanical cardiac support.

Purpose Perceptions on the role and timing of surgical intervention for high-output or massive chylothorax in small and premature babies remain varied. We reviewed our experience with this challenging group of patients to help refine our thinking and devise a more consistent strategy for management. Methods We conducted a retrospective analysis of all patients in our institutions' neonatal populations diagnosed with chylothorax from June 2000 to April 2008. Results Of a total of 23 patients (birth weight, 0.54-4.5 kg; gestational age, 23-41 weeks), 17 were treated conservatively, and 6 with massive chylothorax (>50 mL/kg per day) were treated surgically. Surgical treatment varied, including en masse thoracic duct ligation, mechanical pleurodesis, and application of fibrin glue. Survival in the surgically treated group was 83% vs 59% in the conservatively treated group. Median duration of chest tube drainage was 5 days (postoperative, range, 4-16) in the surgically treated group vs 14 days (range, 1-68) in the conservatively treated group. Conclusion Surgery has a definitive role in the care of small babies with massive chylothorax. Daily output exceeding 50 mL/kg per day with no or minimal response to 3 days of maximal medical therapy may indicate a potential therapeutic benefit of surgery.

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Objective: This study was designed to determine whether either of 2 alternative methods of extracardiac Fontan reconstruction provides superior results. Methods: We reviewed 58 consecutive Fontan procedures performed between 1995 and 2001 with a pedicled pericardial tunnel (group P, n=21) or an extracardiac conduit of polytetrafluoroethylene or allograft aorta (group C, n=37). Operations were performed with cardiopulmonary bypass at 32°C; an aortic crossclamp was applied in only 6 patients. All group P patients and 33 (89%) group C patients received fenestrations. Results: The groups were similar in terms of age, weight, anatomy, and preoperative hemodynamics. There were 3 hospital deaths (5%; 70% confidence limit, 2%-30%), all in group C. Median durations of mechanical ventilation (group P, 1 day; group C, 1 day), intensive care unit stay (group P, 3 days; group C, 3 days), chest tube drainage (group P, 8 days; group C, 7 days), and hospitalization (group P, 10 days; group C, 9 days) were not significantly different. There were no late deaths. All patients received warfarin sodium, and there were no late strokes. Before the Fontan procedure, 1 patient in group P and 3 patients in group C required pacemaker implants. Of the 51 surviving patients in sinus rhythm before the Fontan procedure, only 1 patient in group C subsequently required a pacemaker. Conclusions: Extracardiac Fontan procedures with either a pericardial baffle or conduit are associated with low operative mortality and low risks of arrhythmia and late thromboembolic complication. J Thorac Cardiovasc Surg 2003;125:465-71

Background. The Nuss repair of pectus excavatum is a relatively new, minimally invasive surgical (MIS) alternative to the traditional open “Ravitch-type” operation. We have one of the larger single-center experiences to date, and we conducted this clinical study to evaluate our early experience, emphasizing initial outcome and technical modifications designed to minimize complications. Methods. A retrospective chart review was performed on 112 patients who underwent 116 pectus excavatum repairs between January 1995 and January 2001. The Nuss procedure was performed in 80 patients, and open repair was performed in 32 patients. Information about demographics, deformity, operative course, complications, and early outcome was recorded. Results. Operative duration was 143 minutes for the open group and 53 minutes for the Nuss MIS group (P

Background. Vascular rings are uncommon anomalies in which preferred strategies for diagnosis and treatment may vary among institutions. In this report, we offer a description of our approach and a review of our 25-year experience. Methods. A retrospective review was conducted of all pediatric patients with symptomatic tracheoesophageal compression secondary to anomalies of the aortic arch and great vessels diagnosed from 1974 to 2000. Results. Thirty-one patients (38%) of eighty-two patients (mean age, 1.7 years), were identified with double aortic arch, 22 patients (27%) with right arch left ligamentum, and 20 patients (24%) with innominate artery compression. Our diagnostic approach emphasized barium esophagram, along with echocardiography. This regimen was found to be reliable for all cases except those with innominate artery compression for which bronchoscopy was preferred, and except those with pulmonary artery sling for which computed tomography or magnetic resonance imaging, in addition to bronchoscopy, were preferred. Left thoracotomy was the most common operative approach (70 of 82; 85%). Ten patients (12%) had associated heart anomalies, and 6 (7%) patients underwent repair. Complications occurred in 9 (11%) patients and led to death in 3 (4%) patients. Conclusions. In our practice, barium swallow and echocardiography are sufficient in diagnosing and planning the operative strategy in the majority of cases, with notable exceptions. Definitive intraoperative delineation of arch anatomy minimizes the risk of misdiagnosis or inadequate treatment.

We report a technique for repair of more challenging variants of partial anomalous pulmonary venous return to the superior vena cava.

Background Mandatory fecal diversion has been advocated as an appropriate measure to prevent infection in the clinical setting of an open pelvic fracture. However, the efficacy of this practice has not been verified by prospective investigation and has received only inconsistent support from retrospective analyses. Objective To determine whether fecal diversion is associated with a substantially lower incidence of abdominopelvic infectious complications in patients with open pelvic fractures. Design Case-control study. Setting University-based tertiary care, level I trauma center. Methods The current study reviews our experience with 60 cases admitted from 1987 to 1993 to Harborview Medical Center, a regional level I trauma center. Data collected on each patient included age, sex, Injury Severity Score, Glasgow Coma Scale, initial heart rate and systolic blood pressure, location and severity of wound, fracture pattern, pelvic stability, time to open reduction internal fixation or external fixation, mortality, use of fecal diversion, and incidence and location of infection. Review of the literature produced an additional 186 patients amenable to analysis. Results Fecal diversion was performed in 19 patients, 5 (26%) of whom experienced subsequent abdominopelvic infectious morbidity. Of the remaining 41 patients, 7 patients (17%) experienced infectious complications. The 2 groups (diversion vs no diversion) were comparable with regard to relevant demographic and clinical characteristics of injury severity. Combining the present series with those reported by others gave a composite series of 246 patients. For the composite series, diversion was performed in 70% of patients. Infection developed in 27% of patients who underwent diversion vs 29% in patients who did not. In the present series, only mechanical instability was determined by stepwise logistic regression to be significantly associated with pelvic infection. This association was not altered by diversion status. Conclusions Diversion of the fecal stream to protect open pelvic fractures is not associated with a lower incidence of abdominopelvic infectious complications. Diversion may offer protection to a select group of patients with extensive soft tissue injury or posterior wounds. Mechanical instability was independently associated with infection.

Purpose Heart transplant (HTx) in the setting of a pos crossmatch (XM) is associated with worse outcomes. Since 2004, we have used a virtual XM to evaluate organ offers. We risk stratify based on usual organ criteria (fxn, size, distance) along with the perceived pt specific wait risk and likelihood of a more favorable offer given the patient’s strength and breadth of antibodies. We describe our experience focusing on management and outcomes. Methods and Materials We reviewed records of all HTx pts at our center. PRA at HTx, wait times and XM results were recorded. We reviewed peri-op care for pts with pos XM along with outcomes: rejection and death. Results 100 pts underwent HTx between 8/2004 and 8/2012. 43 were sensitized at HTx, 57 were not. Comparisons shown below ( Table 1 ). When considering any DSA at HTx as VXM pos: 17 were VXM pos and 26 were neg. Of 17 with any DSA at HTx, 12 were flow XM pos and 5 neg. All VXM pos pts received pheresis in OR; post op B cell therapy and induction was based on flow XM results ( Table 2 ). Conclusions A judicious virtual XM strategy can increase the chance for sensitized pts to have a neg XM with acceptable wait times. Aggressive early B cell therapy can produce excellent outcomes for highly sensitized, critically ill pts unable to wait for a neg XM. Pts with DSA at HTx but a neg flow XM may still be at risk for early AMR. Table 1 . Comparision of sensitized vs non-sensitized patients Gender Age (years) Diagnosis Wait Time (days) median (range) Sensitized (43) 24M 56% 7.1 14 CM, 27 CHD, 2 ReTx 59 (2-1899) Non sensitized (57) 33M 58% 6.6 30 CM, 26 CHD, 1 Tumor 38 (2-971) Table 2 . Virtual Crossmatch Positive (ie + DSA) managment and outcomes Pheresis in OR Postop Pheresis Thymo Rituxan Cell Rej Yr 1 AMR Yr 1 Survival (3 yr) Flow XM Pos (12) 12 9 11 9 3 3: 1 hyperacute (ECMO), 2 Rx Bortezomib 100% Flow XM Neg (5) 5 0 2 0 0 1: POD 8 (died) 60% * see below * 2 deaths, both flow XM neg, 1 with AMR POD 8, 1 with early MSOF

An n-dimensional (n-D) filtered backprojection image reconstruction algorithm has been developed and used in the reconstruction of 4-D spectral-spatial magnetic resonance imaging (MRI) data. The algorithm uses n-1 successive stages of 2-D filtered backprojection to reconstruct an n-D image. This approach results in a reduction in computational time on the order of N/sup n-2/ relative to the single-stage technique, where N/sup n/ is the number of elements in an n-D image. The authors describe implementation of the algorithm, including digital filtering and sampling requirements. Images obtained from simulated data are presented to illustrate the accuracy and potential utility of the technique. >

A brief description of 4-dimensional (4-D) filtered backprojection image reconstruction algorithm that has been developed for use in spectral-spatial magnetic resonance imaging (MRI) is presented. The algorithm uses three successive stages of 2-D filtered backprojection to reconstruct a 4-D image. This approach results in a reduction in computational time on the order of N2 relative to the single-stage technique, where N4 is the number of hypervoxels in the image. Design of digital filters used to filter projections is discussed. Images obtained from simulated data are presented to illustrate the accuracy and potential utility of the technique.