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To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.jtcvs.2005.08.047 Byline: Michael E. Mitchell (a), Richard F. Ittenbach (b), J. William Gaynor (c), Gil Wernovsky (d), Susan Nicolson (e), Thomas L. Spray (c) Abbreviations: CI, confidence interval; HLHS, hypoplastic left heart syndrome Abstract: This study was undertaken to investigate the intermediate outcomes after the Fontan operation in the current era. Author Affiliation: (a) Department of Surgery, Division of Cardiothoracic Surgery, University of Louisville, Louisville, Ky (b) Biostatistics and Data Management Core, The Children's Hospital of Philadelphia, Philadelphia, Pa (c) Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pa (d) Division of Pediatric Cardiology and Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pa (e) Division of Cardiothoracic Anesthesia, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pa. Article History: Received 12 April 2004; Revised 8 June 2005; Accepted 1 August 2005

Byline: J.William Gaynor, Margaret H. Collins, Jack Rychik, John P. Gaughan, Thomas L. Spray Abstract: Background and methods: Between January 1, 1984, and December 1, 1997, 73 infants with functional single ventricle and total anomalous pulmonary venous connection were admitted to our institution. A retrospective review was undertaken to determine factors influencing survival. Results: Heterotaxy syndrome was present in 52 patients and hypoplastic left heart syndrome in 14. Obstructed total anomalous pulmonary venous connection was present in 21 patients. The pulmonary venous connection was supracardiac in 32 patients, cardiac in 21 patients, infracardiac in 13, and mixed in 7. Twelve patients died before the operation. The remaining 61 patients underwent surgery at a median age of 5 days (range 1 day-2.5 years). Overall survival was 45% at 6 months of age, 37% at 1 year, and 19% at 5 years. Survival for patients undergoing surgery was 54% at 6 months of age, 44% at 1 year, and 23% at 5 years. By univariate analysis with the Cox proportional hazards model, younger age at the time of the initial operation and repair of total anomalous pulmonary venous connection were predictors of mortality. Lung tissue from 14 patients was available for histologic examination. The pulmonary veins were dilated and wall thickness was increased. Increased muscularization of the arteries was seen in 11 patients. Conclusions: The long-term prognosis for children undergoing staged reconstructive operations for single ventricle and total anomalous pulmonary venous connection is poor. Early mortality is high and late death is a continuing risk. Development of the pulmonary vasculature, especially the pulmonary veins, is abnormal, even in children without clinical evidence of pulmonary venous obstruction. (J Thorac Cardiovasc Surg 1999;117:506-14) Author Affiliation: From The Divisions of Cardiothoracic Surgery,.sup.a Pathology,.sup.b and Cardiology,.sup.c The Children's Hospital of Philadelphia, and the Department of Biostatistics,.sup.d Temple University School of Medicine, Philadelphia, Pa Article History: Received 8 May 1998; Revised 10 June 1998; Revised 27 October 1998; Accepted 3 November 1998 Article Note: (footnote) [star] Address for reprints: J. William Gaynor, MD, 34th St & Civic Center Blvd, Philadelphia, PA 19104., [star][star] 12/6/95597

Byline: Jack Rychik, Hara Levy, J.William Gaynor, William M. DeCampli, Thomas L. Spray Abstract: Objective:Pulmonary atresia with intact ventricular septum is an anatomically heterogeneous anomaly with a variety of surgical strategies possible. We sought to compare the outcome of patients with a single ventricle approach to those with a biventricular repair and to compare outcome of patients with coronary abnormalities to those with normal coronary arteries. Methods: A retrospective review of our surgical database revealed 67 patients with pulmonary atresia with intact ventricular septum operated on between 1981 and 1998. Patients were categorized on the basis of initial surgical strategy: strategy A, aortopulmonary shunt alone (n = 31); strategy B, right ventricular recruitment (n = 32); strategy C, heart transplantation (n = 4). Tricuspid valve size (Z-score) and coronary anatomy were determined. Right ventricular-coronary artery dependency was noted in 8 patients. Results: Overall actuarial survivals at 1, 5, and 8 years were 82%, 76%, and 76%. Mortality was highest in infancy (10 of 16 deaths). Outcome was equivalent for all 3 strategies. There was no difference in tricuspid valve size between survivors and nonsurvivors (mean Z-score -2.0 (2.5) vs -2.0 (1.9), P = .83). There was no difference in survival based on severity of coronary abnormality. Only one third of patients had a successful biventricular repair, and the tricuspid valve was significantly larger in these than in patients who had Fontan operation (mean Z-score -0.53 [1.6], range -3.5 to 1, versus mean Z-score -3.03 [2.7], range -5.5 to 0, P = .002). Conclusions: Surgical outcome for patients born with pulmonary atresia with intact ventricular septum is satisfactory. The strategies of biventricular repair, single ventricle palliation, and heart transplantation allow for equal outcome among all anatomic subtypes. (J Thorac Cardiovasc Surg 1998;116:924-31) Article History: Received 8 May 1998; Revised 18 June 1998; Revised 18 August 1998; Accepted 18 August 1998 Article Note: (footnote) [star] From the Divisions of Cardiology and Cardiothoracic Surgery, Children's Hospital of Philadelphia, and the Departments of Pediatrics and Surgery, University of Pennsylvania School of Medicine, Philadelphia, Pa., [star][star] Address for reprints: Jack Rychik, MD, Division of Cardiology, Children's Hospital of Philadelphia, 324 S 34th St, Philadelphia, PA 19104., a 12/6/93894

Byline: Nancy D. Bridges, Thomas L. Spray, Margaret H. Collins, Neil E. Bowles, Jeffrey A. Towbin Abstract: Objectives: Our goal was to examine the relationship between viral pneumonia and outcome in pediatric patients undergoing lung or heart-lung transplantation. Methods: Prospective surveillance for common respiratory viruses of childhood was performed in all patients undergoing lung or heart-lung transplantation. Specimens were examined for the presence of replicating virus (by culture), viral genome (by polymerase chain reaction), and viral antigen (by immunofluorescence and immunohistochemical staining). The relationship between viral infection and outcome was examined. Results: Sixteen patients underwent 19 transplants during the study period, with follow-up of 1 to 26 months. Virus was identified in the transplanted lung in 29 instances; adenovirus was identified most commonly (8/16 patients) and had the greatest impact on outcome. In 2 patients with early, fulminant infection, adenovirus was also identified in the donor. Adenovirus was significantly associated with respiratory failure leading to death or graft loss and with the histologic diagnosis of obliterative bronchiolitis (P [less than or equal to] .002 in each case). Conclusions: Adenovirus infection in the transplanted lung is significantly associated with graft failure, histologic obliterative bronchiolitis, and death. Health care personnel and families must be vigilant in preventing exposure of transplant recipients to this virus. Availability of a rapid and reliable test for adenovirus in donors and recipients would have an impact on management and could improve outcome for pediatric lung recipients. (J Thorac Cardiovasc Surg 1998;116:617-23) Author Affiliation: Philadelphia, Pa, and Houston, Tex Article History: Received 17 February 1998; Revised 26 May 1998; Revised 26 June 1998; Accepted 29 June 1998 Article Note: (footnote) [star] From the Divisions of Pediatric Cardiology,a Cardiothoracic Surgery,b and Pathology,c Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, and the Division of Pediatric Cardiology,d Texas Children's Hospital, Baylor College of Medicine, Houston, Tex., [star][star] Address for reprints: Nancy D. Bridges, MD, Medical Director, Thoracic Organ Transplantation, Children's Hospital of Philadelphia, 34th St and Civic Center Blvd, Philadelphia, PA 19104., a 12/1/92812

Byline: Robert D.B. Jaquiss, Rodney P. Lusk, Thomas L. Spray, Charles B. Huddleston Abstract: Long-segment stenosis of the trachea in infancy is a considerable surgical challenge because the infants are generally extremely ill and the airway is small. The optimal type of repair is not clearly defined. This report summarizes our experience with rib cartilage tracheoplasty done with cardiopulmonary bypass. Six patients underwent repair of long-segment tracheal stenosis between September 1987 and September 1994. The mean age was 14 weeks (range 1 to 58 weeks). Patients had stenosis of at least 70% of the tracheal length, typically with complete cartilaginous rings. In all patients stenosis was repaired by placement of a section of rib cartilage as an augmentation patch into the anterior surface of the trachea, which had been incised through the entire length of the stenosis. To avoid distal airway intubation, we used cardiopulmonary bypass for all procedures, with a mean bypass duration of 110 minutes (range 54 to 175 minutes). Mechanical ventilation was required for a median of 11 days after the operation (range 7 to 81 days), and the median postoperative hospital stay was 17 days (range 12 to 180 days). All patients are long-term survivors. Complications included the need for extracorporeal membrane oxygenation to treat ventricular dysfunction in one patient and graft dehiscence requiring revision of the distal graft in another. The latter patient has required several treatments with a bronchoscope for removal of granulation tissue. All other patients are free of symptoms and have normal growth with a mean follow-up of 4.7 years (range 5 months to 7.6 years). We conclude that rib cartilage tracheoplasty for long-segment tracheal stenosis provides excellent results in short and intermediate follow-up. In addition, the use of cardiopulmonary bypass allows an unobstructed view of the tiny infant airway and thus permits a precise repair. (J THORAC CARDIOVASC SURG 1995;110:1504-12) Author Affiliation: St. Louis, Mo., and Philadelphia, Pa Article Note: (footnote) [star] From the Division of Cardiothoracic Surgery, Department of Surgery,a and the Department of Otolaryngology,b Washington University School of Medicine, St. Louis, Mo., and the Division of Cardiothoracic Surgery, Department of Surgery,c University of Pennsylvania School of Medicine, Philadelphia, Pa., [star][star] Read at the Seventy-fifth Annual Meeting of The American Association for Thoracic Surgery, Boston, Mass., April 23-26, 1995., a Address for reprints: Charles B. Huddleston, MD 5W24, St. Louis Children's Hospital, 1 Children's Place, St. Louis, MO 63110., aa 0022-5223/95 $5.00 +0, acents 12/6/67805

Pediatric heart transplant recipients were previously reported to have higher early mortality and morbidity than do adult patients treated with triple immunosuppression therapy (steroids, azathioprine and cyclosporine). Nineteen patients (11 infants and 8 older children) underwent orthotopic transplantation using triple immunosuppression therapy. Surveillance for cellular rejection and coronary arteriopathy was performed with endomyocardial biopsy and selective coronary angiography in all patients, with continuous monitoring for hypertension and serious infection. Seventeen of 19 patients (89%; 10 infants and 7 older children) are current survivors, with a median follow-up of 29 months (range 17 to 94). There were S and 7 episodes of rejection in the first 12 months after transplantation in the infant and older groups, respectively, for actuarial freedom-from-rejection rates of 65% at 3 months and 54% at 12 months. Severe coronary arteriopathy was detected in 1 infant 11 months after transplantation. In the first 12 months after transplantation, there were 3 hospitalizations for infection, and 2 patients needed treatment for hypertension in the infant group, compared with 1 hospitalization for infection, and 4 patients on antihypertensives in the older group. An increased prevalence of noninfectious complications in the infant group led to significantly longer postoperative stays than in the older group (mean 27.3 vs 19.4 days; p

From July 1988 to March 1991, extracorporeal membrane oxygenation (ECMO) was used in 8 infants (newborn to 16 months old) with unoperated cyanotic congenital heart disease and cardiopulmonary collapse, associated with hypercyanotic spells (4 infants), pulmonary hypertensive crises (3) and sepsis (1). Indications for ECMO support were arterial saturations [is less than or equal to] 60% accompanied by hypotension and metabolic acidosis unresponsive to mechanical ventilation with 100% oxygen, paralysis and sedation, and pharmacologic support with inotropes or vasodilators, or both. Venoarterial bypass by carotid/jugular cannulation with flow rates of 100 to 840 ml/kg/min (mean 460) stabilized all patients. Duration of ECMO support ranged from 15 to 840 hours and was associated with transient seizures (1 patient) and renal failure (1). Seven patients underwent palliative (3 patients) or corrective (4) surgical procedures while on ECMO or within 48 hours of decannulation, including 1 patient bridged to double-lung transplantation with a long (840 hours) duration of ECMO. There was 1 operative and 2 late (>1 month after dacannulation) deaths, for an overall survival rate of 62%. These 5 survivors all have normal growth and development, and patent neck vessels at the site of cannulation. These early results indicate that ECMO can be effective mechanical support in cardiovascular crises untreatable with maximal conventional medical therapy and can be used as a bridge to successful surgical palliation or repair.

AbstractBackgroundVeno-arterial extracorporeal membrane oxygenation is frequently used in patients with cardiac disease. We evaluated short-term outcomes and identified factors associated with hospital mortality in cardiac patients supported with veno-arterial extracorporeal membrane oxygenation.MethodsA retrospective review of patients supported with veno-arterial extracorporeal membrane oxygenation at a university-affiliated children’s hospital was performed.ResultsA total of 253 patients with cardiac disease managed with extracorporeal membrane oxygenation were identified; survival to discharge was 48%, which significantly improved from 39% in an earlier era (1995–2001) (p=0.01). Patients were categorised into surgical versus non-surgical groups on the basis of whether they had undergone cardiac surgery before or not, respectively. The most common indication for extracorporeal membrane oxygenation was extracorporeal cardiopulmonary resuscitation: 96 (51%) in the surgical group and 45 (68%) in the non-surgical group. In a multiple covariate analysis, single-ventricle physiology (p=0.01), duration of extracorporeal membrane oxygenation (p<0.01), and length of hospital stay (p=0.03) were associated with hospital mortality. Weekend or night shift cannulation was associated with mortality in non-surgical patients (p=0.05).ConclusionWe report improvement in survival compared with an earlier era in cardiac patients supported with extracorporeal membrane oxygenation. Single-ventricle physiology continues to negatively impact survival, along with evidence of organ dysfunction during extracorporeal membrane oxygenation, duration of extracorporeal membrane oxygenation, and length of stay.

AbstractBackgroundWe have previously shown that the minor alleles of vascular endothelial growth factor A (VEGFA) single-nucleotide polymorphism rs833069 and superoxide dismutase 2 (SOD2)single-nucleotide polymorphism rs2758331 are both associated with improved transplant-free survival after surgery for CHD in infants, but the underlying mechanisms are unknown. We hypothesised that one or both of these minor alleles are associated with better systemic ventricular function, resulting in improved survival.MethodsThis study is a follow-up analysis of 422 non-syndromic CHD patients who underwent neonatal cardiac surgery with cardiopulmonary bypass. Echocardiographic reports were reviewed. Systemic ventricular function was subjectively categorised as normal, or as mildly, moderately, or severely depressed. The change in function was calculated as the change from the preoperative study to the last available study. Stepwise linear regression, adjusting for covariates, was performed for the outcome of change in ventricular function. Model comparison was performed using Akaike’s information criterion. Only variables that improved the model prediction of change in systemic ventricular function were retained in the final model.ResultsGenetic and echocardiographic data were available for 335/422 subjects (79%). Of them, 33 (9.9%) developed worse systemic ventricular function during a mean follow-up period of 13.5 years. After covariate adjustment, the presence of the VEGFAminor allele was associated with preserved ventricular function (p=0.011).ConclusionsThese data support the hypothesis that the mechanism by which the VEGFAsingle-nucleotide polymorphism rs833069 minor allele improves survival may be the preservation of ventricular function. Further studies are needed to validate this genotype–phenotype association and to determine whether this mechanism is related to increased vascular endothelial growth factor production.

AbstractObjectivesPulmonary lymphangiectasia associated with hypoplastic left heart syndrome with an intact or restrictive atrial septum may result from increased left atrial pressure, and is associated with worse outcomes following staged reconstruction due to lung dysfunction and significant hypoxaemia. Our objective was to characterise the incidence of pulmonary lymphangiectasia in cases of early mortality following stage 1 reconstructions.MethodsAn institutional cardiac surgical database was retrospectively searched for patients who died within 30 days following a stage 1 reconstruction between 1 January, 1984 and 31 December, 2013. During that period, 1669 stage 1 procedures were performed. Autopsy lung specimens were reviewed by a paediatric pathologist. Patients who died of suspected technical issues were excluded.ResultsA total of 54 patients were included, and of these seven cases (8.5%) of pulmonary lymphangiectasia were identified. The mean estimated gestational age was 38.2±2.4 weeks, and the mean birth weight was 3.0±0.6 kg. The median interval between surgery and death was 1 day (with a range from 0 to 18 days). The atrial septum was intact in one patient (14.3%), restrictive in three patients (42.9%), and unrestrictive in three patients (42.9%).ConclusionsPulmonary lymphangiectasia may develop in hypoplastic left heart syndrome with or without a restrictive atrial septum. As standard prenatal diagnostic evaluations and treatment methods for pulmonary lymphangiectasia are limited, this may be an important contributor to early and late mortality following stage 1 reconstruction for hypoplastic left heart syndrome.

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1067/mtc.2000.106033 Byline: Doff B. McElhinney, Stephen Paridon, Thomas L. Spray Author Affiliation: Philadelphia Pa From the Divisions of Cardiothoracic Surgery and Cardiology, The Children's Hospital of Philadelphia and University of Pennsylvania Medical School, Philadelphia, Pa Article History: Received 12 January 2000; Accepted 25 January 2000 Article Note: (footnote) [star] Address for reprints: Doff B. McElhinney, MD, Children's Hospital of Philadelphia, 34th St and Civic Center Blvd, Room 9557, Philadelphia, PA 19104 (E-mail: mcelhinney@email.chop.edu )., [star][star] J Thorac Cardiovasc Surg 2000;119:1284-5

Background: Anomalous aortic origin of a coronary artery (AAOCA) presents in varying age-groups. Assuming management algorithms differ between pediatric and adult institutions, we compared the perioperative management of patients with AAOCA at two such centers.Methods: A retrospective review was conducted at a pediatric and an adult institution of patients 14 years or older who underwent surgical repair of AAOCA between January 2000 and May 2014.Results: Twenty patients from the pediatric center (median age: 16.5 years, range: 14-18 years) and nine patients from the adult center (median age: 40 years, range: 37-52 years) were included. An anomalous aortic origin of a right coronary artery was the most frequent pathology at each institution. Chest pain was the most common presenting symptom at both institutions. Preoperative echocardiography was performed in 95% patients at the pediatric center and in 100% of patients at the adult center. Cardiac catheterization was utilized more frequently at the adult center, and cardiac magnetic resonance imaging more commonly employed at the pediatric center. Isolated coronary unroofing was performed in 19 of 20 cases at the pediatric center and in only 2 (22%) cases at the adult institution, both by congenitally trained cardiac surgeons. More concomitant cardiac procedures were performed at the adult center with associated longer operative times and hospital stays.Conclusion: Management strategies for AAOCA vary depending on both patient-specific factors and expertise of the managing team. Further studies are needed to optimally standardize diagnostic and treatment pathways regardless of location venue.

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Background:Sodium bicarbonate (NaHCO3) is a common treatment for metabolic acidemia; however, little definitive information exists regarding its treatment efficacy and cerebral hemodynamic effects. This pilot observational study quantifies relative changes in cerebral blood flow (?rCBF) and oxy- and deoxyhemoglobin concentrations (?HbO2and ?Hb) due to bolus administration of NaHCO3in patients with mild base deficits.Methods:Infants and children with hypoplastic left heart syndrome (HLHS) were enrolled before cardiac surgery. NaHCO3was given as needed for treatment of base deficit. Diffuse optical spectroscopies were used for 15?min postinjection to noninvasively monitor ?Hb, ?HbO2, and ?rCBF relative to baseline before NaHCO3administration.Results:Twenty-two anesthetized and mechanically ventilated patients with HLHS (aged 1 d to 4 y) received a median (interquartile range) dose of 1.1 (0.8, 1.8) mEq/kg NaHCO3administered intravenously over 10–20 s to treat a median (interquartile range) base deficit of -4 (-6, -3) mEq/l. NaHCO3caused significant dose-dependent increases in ?rCBF; however, population-averaged ?Hb and ?HbO2as compared with those of controls were not significant.Conclusions:Dose-dependent increases in cerebral blood flow (CBF) caused by bolus administration of NaHCO3are an important consideration in vulnerable populations wherein risk of rapid CBF fluctuations does not outweigh the benefit of treating a base deficit.

AbstractObjectiveLow weight at birth is a risk factor for increased mortality in infants undergoing surgery for congenitally malformed hearts. There has been a trend towards performing surgery in patients early, and for amenable lesions, in a single stage rather than following initial palliative procedures. Our goal was to report on the current incidences of morbidities and mortality in infants born with low weight and undergoing surgery for congenital cardiac disease.MethodsWe made a retrospective review of the data from patients meeting our criterions for entry from July, 2000, through July, 2004. The criterions for inclusion were weight at birth less than or equal to 2500 grams, and congenital cardiac malformations requiring surgery during the initial hospitalization. A criterion for exclusion was isolated persistent patency of the arterial duct. We assessed preoperative, intraoperative, and postoperative variables.ResultsWe found a total of 105 patients meeting the criterions for inclusion. The median weight at birth was 2130 grams, and median gestational age was 36 weeks. The most common morbidity identified was infections of the blood stream. Infections, and chronic lung disease, were associated with increased length of stay. Survival overall was 76%. Patients with hypoplastic left heart syndrome, or a variant thereof, had the lowest survival, of 62%. The needs for cardiopulmonary resuscitation, or extracorporeal membrane oxygenation, post-operatively were the only factors identified as independent risk factors for mortality.ConclusionPatients undergoing surgery during infancy for congenital cardiac disease who are born with low weight have a higher mortality and morbidity than those born with normal weight.

To define the modes of presentation, incidence of major organ dysfunction, predictors of hospital mortality, and adverse outcomes in neonates with critical heart disease admitted to a tertiary care center.

Neo-aortic insufficiency (neo-AI) has been noted following the Ross procedure. The purpose of this study was to evaluate the ability of intraoperative transesophageal echocardiography (TEE) to predict future neo-AI in pediatric patients undergoing the Ross from January 1995 to December 2003, who had an intraoperative TEE, and discharge and follow-up transthoracic (TTE) echocardiograms.

Microcephaly is a marker of abnormal fetal cerebral development, and a known risk factor for cognitive dysfunction. Patients with hypoplastic left heart syndrome have been found to have an increased incidence of abnormal neurodevelopmental outcomes. We hypothesized that reduced cerebral blood flow from the diminutive ascending aorta and transverse aortic arch in the setting of hypoplastic left heart syndrome may influence fetal growth of the brain. The purpose of our study, therefore, was to define the prevalence of microcephaly in full-term infants with hypoplastic left heart syndrome, and to investigate potential cardiac risk factors for microcephaly. We carried out a retrospective review of full-term neonates with hypoplastic left heart syndrome. Eligible patients had documented indexes of birth weight, and measurements of length, and head circumference, as well as adequate echocardiographic images for measurement of the diameters of the ascending aorta and transverse aortic arch. We used logistic regression for analysis of the data. A total of 129 neonates met the criterions for inclusion, with 15 (12) proving to have microcephaly. The sizes of their heads were disproportionately smaller than their weights (p less than 0.001) and lengths (p less than 0.001) at birth. Microcephaly was associated with lower birth weight (p less than 0.001), lower birth length (p equal to 0.007), and a smaller diameter of the ascending aorta (p equal to 0.034), but not a smaller transverse aortic arch (p equal to 0.619), or aortic atresia (p equal to 0.969). We conclude that microcephaly was common in this cohort of neonates with hypoplastic left heart syndrome, with the size of the head being disproportionately smaller than weight and length at birth. Microcephaly was associated with a small ascending aorta, but not a small transverse aortic arch. Impairment of somatic growth may be an additional factor in the development of microcephaly in these neonates.

Objectives: The purpose of our study was to assess the prevalence and progression, during childhood and adolescence, of dilation of the neo-aortic root, and neo-aortic valvar regurgitation, and to identify risk factors for such dilation and regurgitation, after the arterial switch operation. Methods: We included all patients who had undergone an arterial switch operation at The Children's Hospital of Philadelphia, and had been followed for a minimum of 4 years, and had at least 2 postoperative echocardiograms. Neo-aortic valvar regurgitation was quantitatively assessed, and measurements were made of the neo-aortic root at the level of the basal attachment of the leaflets, mid-sinusal level, and the sinutubular junction. Results: We found 82 patients who satisfied the criterions for inclusion, of whom 52 patients had transposition with an intact ventricular septum, and 30 had either an associated ventricular septal defect or double outlet right ventricle. The median follow-up time was 8.8 years (4.1 to 16.4 years). The neo-aortic valve had been replaced in 1 patient. Of the patients, 3 had moderate, 66 had trivial to mild, and 12 had no neo-aortic valvar regurgitation at their most recent follow-up. The regurgitation had progressed by at least 1 grade in 38 of the 82 patients (46.4%). Neo-aortic dilation was noted at the basal attachment of the leaflets, and at mid-sinusal level, which was out of proportion to somatic growth. Conclusions: At mid-term follow-up, significant neo-aortic valve regurgitation is present in 3.7%, and trivial to mild regurgitation in 81.4% of patients. The regurgitation progressed in almost half of the patients over time. We also noted progressive dilation of the neo-aortic root out of proportion to somatic growth.

Although extracorporeal membrane oxygenation (ECMO) is an acceptable strategy for children with refractory cardiac dysfunction after cardiac surgery, its role after stage I reconstruction for hypoplastic left heart syndrome and its variants is controversial. Our objective is to describe the outcome of “nonelective” ECMO after stage I reconstruction.

For patients requiring intervention because of progressive disease of the aortic valve, the perfect palliation will provide a valve that produces normal dynamics of flow, will not require anti-coagulation, will grow with the patient, and have long term durability. Current surgical interventions include aortic valvoplasty, or replacement with either a mechanical or tissue prosthesis. Options for tissue valves include insertion of a pulmonary autograft in the Ross procedure, a cadaveric homograft, or porcine or bovine xenograft valves. The optimal option is still debated.

In contrast to older patients, children and young adults rarely have isolated disease of the systemic atrioventricular valve. Stenosis and/or regurgitation of the systemic atrioventricular valve, however, frequently coexist with complex congenital cardiac disease. In addition, most patients undergoing surgery on the systemic atrioventricular valve have had previous intracardiac repairs.

The nomenclature and classification of patients with a functionally univentricular heart has been debated for decades. We review here the approach taken for dealing with this group of patients by the International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Cardiac Disease. We discuss the approach of this Nomenclature Working Group in the context of other historical and contemporary ideas about this topic.

Objectives: The Ross procedure is increasingly utilized in the treatment of aortic valvar disease in children and adolescents. Our purpose was to compare pre- and post-operative exercise state in this population. Methods: We included patients who underwent the Ross procedure at our institution between January, 1995, and December, 2003, and in whom we had performed pre- and post-operative exercise stress tests. We used a ramp bicycle protocol to measure consumption of oxygen and production of carbon dioxide. Cardiac output was estimated from effective pulmonary blood flow by the helium acetylene re-breathing technique. Results: We studied 26 patients, having a median age at surgery of 15.7 years, with a range from 7.5 to 24.1 years. The primary indication for surgery in two-thirds was combined aortic stenosis and insufficiency. Median time from the operation to the post-operative exercise stress test was 17.4 months, with a range from 6.7 to 30.2 months. There was a trend toward lower maximal consumption of oxygen after the procedure, at 36.3 plus or minus 7.6 millilitres per kilogram per minute (83.9% predicted) as opposed to 38.6 plus or minus 8.4 millilitres per kilogram per minute (88.5% predicted, p equal to 0.06). Patients after the procedure, however, had significantly increased adiposity, so that there was no difference in maximal consumption of oxygen indexed to ideal body weight before and after the operation. In 20 of the patients, aerobic capacity improved or was stable after the operation. There was no post-operative chronotropic impairment. Conclusions: In the majority of patients following the Ross procedure, exercise performance is stable and within the normal range of a healthy age and sex matched population, despite sedentary lifestyles and increased adiposity.

An increasingly complex group of children is now being followed as outpatients after surgery for congenital heart disease. A variety of complications and physiologic perturbations, both expected and unexpected, may present during follow-up, and should be anticipated by the practitioner and discussed with the patient and family. The purpose of this position article is to provide a framework for outpatient follow-up of complex congenital heart disease, based on a review of current literature and the experience of the authors.

There are many reasons for seeking to create a global database with which to record the outcomes of therapy for congenital heart disease. Such a database can function as a tool to support a variety of purposes:

For many years, surgeons have been able to implement new techniques and treatments with minimal oversight. Although regulatory control over application of new strategies for treatment is far different today, surgeons have enjoyed a greater degree of freedom than other specialists. The laboratory of the surgeon, and particularly the congenital cardiac surgeon, is often the operating room, since appropriate animal models are not available. Thus, the congenital cardiac surgeon has often been stimulated to modify existing techniques, or create new surgical approaches, to improve the perceived limitations of previously known surgical strategies.1 The introduction of a new surgical technique has often taken the strong ego and persistence of a surgical innovator, and has subjected the surgeon to criticism from medical colleagues. Although innovation is a proud heritage of the development of congenital cardiac, it now seems appropriate, as we are more cognizant of the past accomplishments in the development of repairs for congenital cardiac malformations, to evaluate how surgical techniques become widely adopted, often with only minimal data to suggest improvement over previously accepted procedures.

Background: Modified ultrafiltration has been shown to reverse haemodilution and improve ventricular function following cardiopulmonary bypass. There has been concern, however, about the safety and efficacy of modified ultrafiltration after the first stage of Norwood reconstruction for palliation of neonates with hypoplasia of the left heart and its variants. Methods: We reviewed the intraoperative course of all patients undergoing the first stage of Norwood reconstruction between September 1, 2000, and August 31, 2002. Results: The first stage of reconstruction was performed in 99 neonates, 78 with classical hypoplasia of the left heart, and 21 with variants. Mean weight at surgery was 3.1 plus or minus 0.7 kilograms. Genetic syndromes, weight less than or equal to 2.5 kilograms, and/or major additional cardiac or non-cardiac anomalies, were present in 44 patients. We deemed these patients to constitute the group at high risk. A modified Blalock–Taussig shunt was utilized in 95 patients, and a conduit from the right ventricle to the pulmonary arteries in 4. Deep hypothermic circulatory arrest was used in all patients for a mean period of 45 minutes, plus or minus 15 minutes. Total support time on cardiopulmonary bypass plus deep hypothermic circulatory arrest was 100 minutes plus or minus 26 minutes. Modified ultrafiltration was performed in all patients. The mean duration of modified ultrafiltration was 10 plus or minus 2 minutes, and the total volume of filtrate removed was 104 plus or minus 29 millilitres per kilogram. There were no complications from modified ultrafiltration, and no patient required discontinuation of modified ultrafiltration for haemodynamic instability. During modified ultrafiltration, the haematocrit increased from 31 percent plus or minus 4 to 46 percent plus or minus 6. Heart rate decreased from 170 plus or minus 17 beats per minute to 158 plus or minus 16 beats per minute. Systolic blood pressure increased from 57 plus or minus 12 to 63 plus or minus 13 millimetres of mercury, and diastolic blood pressure from 30 plus or minus 8 to 35 plus or minus 7 millimetres of mercury. All these values are significant at a p value of less than 0.0001. Hospital morality in the patients at low risk was 3 of 55 (5.5 percent), but was 12 of 44 (27.3 percent) in the patients deemed to be at high-risk. Conclusions: Modified ultrafiltration is safe procedure following the first stage of Norwood reconstruction, with improvement in all haemodynamic parameters measured. Modified ultrafiltration is an additional incremental strategy, which may contribute to the overall improvement in outcome following surgical palliation of patients with hypoplasia of the left heart or its variants.

To report an influenza B infection with associated myocarditis and severe skeletal myositis.

Objective: To explore whether prenatal diagnosis of congenital heart disease is associated with lower levels of parental distress and greater satisfaction with decisions about cardiothoracic surgery when compared to postnatal diagnosis. Methodology: A combined quantitative–qualitative design was used. Participants included the parents of 31 neonates (30 mothers and 22 fathers) admitted to the cardiac intensive care unit between 1 November 2001 and 1 May 2002 for repair of congenital cardiac malformations. Participants completed self-report measures of anxiety, optimism, and life events pre-operatively, and semi-structured qualitative interviews assessing satisfaction with decision-making within 1 week of the operation. Results: At the time of surgery, mothers of neonates receiving the diagnosis prenatally did not differ from mothers of neonates receiving the diagnosis postnatally on measures of anxiety, optimism, and life events. Fathers of neonates receiving the diagnosis prenatally, however, reported more optimism, lower state and trait anxiety, and fewer negative life events than fathers of neonates receiving the diagnosis postnatally. When we analyzed the interviews, we found that, regardless of the timing of the diagnosis, parents felt as though they made a genuine choice for their baby to have surgery. Conclusions: In this pilot study, fathers who learned prenatally that their child had a congenital cardiac malformation were less distressed than those who discovered this fact only postnatally. From the parental perspective, nonetheless, distress and urgency do not impair their ability to make decisions about neonatal cardiac surgery.

Objective:This study was undertaken to determine the demographic, anatomic, institutional, and surgical risk factors associated with outcomes after the Norwood operation. Methods:A total of 710 of 985 neonates with critical aortic stenosis or atresia enrolled in a prospective 29-institution study between 1994 and 2000 underwent the Norwood operation. Admission echocardiograms were independently reviewed for 64% of neonates. Competing risks analyses were constructed for outcomes after Norwood operation and after cavopulmonary shunt. Incremental risk factors for outcome events were sought. Results:Overall survivals after the Norwood operation were 72%, 60%, and 54% at 1 month, 1 year, and 5 years, respectively. According to competing risks analysis, 97% of neonates reached a subsequent transition state by 18 months after Norwood operation, consisting of death (37%), cavopulmonary shunt (58%), or other state (2%, cardiac transplantation, biventricular repair, or Fontan operation). Risk factors for death occurring before subsequent transition included patient-specific variables (lower birth weight, smaller ascending aorta, older age at Norwood operation), institutional variables (institutions enrolling ≤10 neonates, two institutions enrolling ≥40 neonates), and procedural variables (shunt originating from aorta, longer circulatory arrest time, and management of the ascending aorta). Of neonates undergoing cavopulmonary shunt, 91% had reached a subsequent transition state by 6 years after cavopulmonary shunt, consisting of Fontan operation (79%), death (9%), or cardiac transplantation (3%). Risk factors for death occurring before subsequent transition included younger age at cavopulmonary shunt and need for right atrioventricular valve repair. Conclusions:Competing risks analysis defines the prevalence of the various outcomes after Norwood operation and predicts improved outcomes with successful modification of controllable risk factors.

The technique of aortic valve replacement with the pulmonary autograft was originally described by Ross in 1967. Aortoventriculoplasty was first described by Konno in 1975 and was used for aortic valve replacement using a mechanical prosthesis. Since then, various refinements in both procedures as well as wider availability of cryopreserved allografts have permitted increasing application of the combined surgical approach in infants and small children. The Ross-Konno combination became particularly appealing in the treatment of complex left ventricular tract obstruction that is often not amenable to simple techniques such as open valvulotomy or balloon valvuloplasty. The Ross-Konno technique offers a number of advantages over the two alternatives of mechanical or allograft valve replacement. It has a low risk of thromboembolism and prosthetic valve endocarditis, and avoids the lifetime anticoagulation requirement and the size limitation seen with the mechanical valves. It also seems to be free of the rapid degeneration observed with the allografts, particularly when placed in the aortic position. However, the primary advantage of the pulmonary autograft over the other two choices is its apparent potential for growth in the aortic position. Confirmed in a number of short to intermediate term follow-up studies, the Ross-Konno technique is at this time the superior option for definitive treatment of a complex LVOTO at an early age.

Objective:This study was undertaken to evaluate factors contributing to a decrease in early mortality and morbidity after the Fontan procedure between January 1, 1992, and December 31, 1999. Methods:Outcomes evaluated were early survival, duration of pleural effusions, and duration of hospitalization. Potential predictors evaluated included anatomic diagnosis, presence of a common atrioventricular valve, preoperative pulmonary artery pressure, type of Fontan operation, type of intentional right-to-left shunt or baffle fenestration, and use of modified ultrafiltration. Results:The modified Fontan procedure was performed in 332 patients at a median age of 22 months (range, 11-380 months) and a median weight of 11 kg (range, 5.8-120 kg). Prior stage I reconstructive surgery for classic or variant hypoplastic left heart syndrome had been performed in 205 (53%) of 332 patients, and 318 (96%) had undergone an interim superior cavopulmonary connection. A lateral-tunnel Fontan operation was performed in 281 patients, and an extracardiac conduit Fontan operation was performed in 51 patients. An intentional right-to-left shunt was created in 298 (90%) patients. Between 1992 and 1999, the outcome after the modified Fontan operation improved significantly. Overall mortality was 6.6% (22/332), with only 2 deaths since 1994. Morbidity was also reduced, with a decreased duration of pleural effusions and decreased hospital stay. In a multivariable analysis of the entire cohort, only the presence of a common atrioventricular valve (odds ratio, 7.64; 95% confidence limits, 2.07-28.14; P= .0002) and increased preoperative pulmonary artery pressure (odds ratio, 1.46/1 mm Hg increase; 95% confidence limits, 1.2-1.78; P< .001) increased the risk of early death, whereas use of a single-punch fenestration in a lateral-tunnel Fontan (odds ratio, 0.06; 95% confidence limits, 0.01-0.65; P= .02) and use of modified ultrafiltration (odds ratio, 0.14; 95% confidence limits, 0.03-0.72; P= .019) decreased the risk of death. The risk of prolonged pleural effusions (>3 days) was increased in patients with hypoplastic left heart syndrome (odds ratio, 1.73; 95% confidence limits, 1.07-2.81; P= .03) and was decreased by use of a single-punch fenestration in a lateral-tunnel Fontan operation (odds ratio, 0.17; 95% confidence limits, 0.07-0.4; P< .001), as well as by the use of modified ultrafiltration (odds ratio, 0.25; 95% confidence limits, 0.15-0.40; P< .01). Conclusions:In a contemporary series of Fontan operations performed largely in patients with hypoplastic left heart syndrome or variants, systemic ventricle morphology had no effect on mortality. Some patient characteristics, however, continue to influence outcome. The decrease in mortality and morbidity in the current era is attributed to changes in management strategies, specifically the use of modified ultrafiltration and baffle fenestration.

Objective:The aim was to develop a consensus-based method of risk adjustment for in-hospital mortality among children younger than 18 years after surgery for congenital heart disease (designated RACHS-1). Methods:An 11-member national panel of pediatric cardiologists and cardiac surgeons used clinical judgment to place surgical procedures into six risk categories. Categories were refined after review of information from the Pediatric Cardiac Care Consortium and three statewide hospital discharge data sets. The effects of including additional clinical variables were explored by comparing areas under receiver-operator characteristic curves. Results:Among 4602 surgical patients in the Pediatric Cardiac Care Consortium data set and 4493 in the hospital discharge data, 3767 (81.9%) and 3832 (85.3%), respectively, had a single cardiac procedure, and 98.5% and 89.2%, respectively, were able to be assigned to one of six risk categories defined by the panel. Mortality rates showed expected trends (P<.001). For the Pediatric Cardiac Care Consortium data, mortality rates were 0.4% in category 1, 3.8% in 2, 8.5% in 3, 19.4% in 4, and 47.7% in 6; rates were similar in the hospital discharge data. There were too few cases in category 5 to estimate mortality rates. In multivariable models, younger age, prematurity, and the presence of a major noncardiac structural anomaly added to the risk of in-hospital death predicted by risk category alone. Best performance was obtained when cases with multiple procedures were placed in the risk category of the most complex procedure. Conclusion:The RACHS-1 method should adjust for baseline risk differences and allow meaningful comparisons of in-hospital mortality for groups of children undergoing surgery for congenital heart disease.

Protein-losing enteropathy (PLE), excessive serum protein loss within the gastrointestinal tract, after Fontan operation is a poorly understood disorder. Reported to occur anywhere from weeks to years after Fontan operation, there are no identifiable risk factors for its development, and its clinical manifestations vary widely from significant morbidity and mortality to mild-to-moderate hypoproteinemia with minimal functional impairment. Treatment strategies, tailored to the severity of the disease, include symptomatic relief with diuretics and supplemental protein, attempts at halting intestinal protein leak using steroids or heparin, and alteration of cardiovascular physiology via fenestration creation, atrial pacing, or heart transplantation. A better understanding of the pathophysiology of PLE will allow the development of more effective treatment modalities. We hypothesize an abnormality of local intestinal circulation in patients with PLE that may be related to low cardiac output. Compensatory flow redistribution takes place under conditions of low cardiac output. We studied superior mesenteric artery flow using Doppler ultrasound in 40 patients after Fontan operation, 13 of whom had clinical signs of PLE, and compared them with 25 normal control patients. Diastolic velocities were lower in Fontan subjects, and the ratio of systolic-to-diastolic velocities and the resistance index were higher in Fontan patients compared with the control group. Patients with PLE after Fontan operation had higher systolic-to-diastolic velocities and resistance index than patients with Fontan and no active PLE. However, subjects with Fontan circulation but without PLE had higher indices of mesenteric resistance than the normal controls, suggesting an abnormality of the mesenteric circulation even in those without overt signs of PLE. It is plausible to postulate that activation of the renin-angiotensin system with increased levels of circulating angiotensin II may be responsible for the increase in mesenteric vascular resistance seen after Fontan operation, thereby placing these patients at risk for development of PLE. Copyright © 2002 by W.B. Saunders Company

Objective:Mechanical cardiorespiratory support is occasionally required before or after pediatric thoracic organ transplantation. Extracorporeal membrane oxygenation is the most commonly used mechanical support technique in children. The goal of this study was to examine the indications for initiation and outcomes after peritransplant use of extracorporeal membrane oxygenation. Methods:A retrospective study was conducted of 65 patients who received peritransplant extracorporeal membrane oxygenation between November 1994 and June 2000. The pretransplant group included 45 patients (average age, 38 months) supported with extracorporeal membrane oxygenation and listed for transplantation (31 heart, 8 lung, and 6 heart-lung), and the post-transplant group included 20 patients (average age, 83 months) who required extracorporeal membrane oxygenation after thoracic organ transplantation (12 heart, 6 lung, and 2 heart-lung transplants). Hospital course and outcomes were evaluated. Results:With regard to pretransplant extracorporeal membrane oxygenation, patients listed for heart transplants were more likely to survive to transplantation than were those listed for lung or heart-lung transplants (12/31 [39%] vs 1/14 [7%], P=.03). There was no difference in long-term survival between heart transplant patients after extracorporeal membrane oxygenation and those without extracorporeal membrane oxygenation (12-month actuarial survival, 83% vs 73%; P=.68). Patients who survived for prolonged periods on extracorporeal membrane oxygenation (>250 hours) typically received heart transplants (7/8 [88%]). With regard to post-transplant extracorporeal membrane oxygenation, patients receiving lung or heart-lung transplants had better short-term outcomes than those receiving heart transplants (63% survived to discharge vs 33%). All 3 patients with early graft dysfunction receiving lung transplants survived to discharge. Conclusions:Long-term outcomes among those undergoing heart transplantation after support with an extracorporeal membrane oxygenator are comparable with those of patients not receiving extracorporeal membrane oxygenation. Extracorporeal membrane oxygenation can be a useful post-transplant support device, particularly in patients undergoing lung transplants.

Chronic lung disease and pulmonary failure are complications that can occur after bone marrow transplantation (BMT) and are associated with severe morbidity and mortality.

Background:Repair of aortic coarctation is often delayed in small infants because of the belief that such patients are at risk of recurrent arch obstruction and that growth will decrease this risk. To determine whether low weight was a risk factor for recurrent arch obstruction, we reviewed our experience with coarctation repair via left thoracotomy in infants less than 3 months of age. Methods:From 1990 to 1999, 103 patients less than 3 months of age underwent repair of aortic coarctation through a left thoracotomy. Median age was 18 days (1-90 days), with 45 patients less than 2 weeks. Median weight was 3.3 kg (1.0-6.4 kg) and 14 patients were less than 2 kg. The method of repair was resection and end-to-end anastomosis in 64 patients, subclavian flap angioplasty in 34, and patch augmentation of the arch in 5. Demographic, echocardiographic, and operative variables were analyzed for correlation with recurrent arch obstruction. Results:One early and 1 late death occurred, both in patients who had complications but no evidence of recoarctation. At median follow-up of 24 months, reinterventions for recurrent arch obstruction were performed in 15 patients. The median time to reintervention was 5 months and was less than 1 year in 12 patients. Kaplan-Meier freedom from arch reintervention was 88% at 1 year (95% confidence intervals: 82%-94%) and 82% at 5 years (95% confidence intervals: 72%-92%). Factors associated with shorter duration to arch reintervention by univariable Cox regression included younger age (continuous, P=.01; ||2 weeks, P=.005), smaller transverse arch (absolute diameter, P||.001; indexed to weight, P=.03; indexed to ascending aortic diameter, P=.02), and smaller ascending aorta (absolute diameter, P=.02). Smaller absolute transverse arch diameter and younger age were the only independent predictors of shorter time to arch reintervention by multivariable Cox regression analysis. Weight and type of repair did not correlate with risk of recoarctation. Conclusions:Low weight is not a risk factor for recurrent obstruction after repair of coarctation of the aorta in infants less than 3 months of age. Rather, risk of recoarctation is more a function of the anatomy of the arch. Thus, it is not indicated to delay repair in low weight infants with the goal of achieving growth.

Background. Thrombus formation is common after a Fontan operation. We investigated the frequency and location of thrombus in our population of children based on the type of Fontan operation performed.

Objectives:There is an increasing incidence of sinus node dysfunction after the Fontan procedure. Inability to maintain atrioventricular synchrony after the Fontan operation has been associated with an adverse late outcome. Although pacing may be helpful as a primary or adjunct modality after the Fontan procedure, the effects of performing a late thoracotomy or sternotomy for epicardial pacemaker implantation are unknown. In addition, little is known about the long-term effectiveness of epicardial leads in patients with single ventricles. The purpose of this study was to compare the hospital course and follow-up of epicardial pacing lead implantation in patients with Fontan physiology and patients with 2-ventricle physiology. Methods:We retrospectively reviewed all isolated epicardial pacemaker implantations and outpatient evaluations performed between January 1983 and June 2000. Results:There was no difference in the perioperative course for the 31 Fontan patients (27 atrial and 41 ventricular leads [68 total]) compared with the 56 non-Fontan subjects (9 atrial and 61 ventricular leads [70 total]). The median length of stay in Fontan and non-Fontan patients was 3 and 4 days, respectively. There was no early mortality in either group. Pleural drainage for 5 days or longer was reported in 4% of the Fontan cohort and 3% of the non-Fontan group. Late pleural effusions were identified in only 2 patients in the Fontan group and 2 patients in the non-Fontan group. There was no significant difference in epicardial lead survival between the Fontan group and the non-Fontan group (1 year, 96%; 2 years, 90%; 5 years, 70%). The overall incidence of lead failure was 17% (24/138). Conclusions:Epicardial leads can be safely placed in Fontan patients at no additional risk compared to patients with biventricular physiology. Sensing and pacing qualities were relatively constant in both the Fontan and non-Fontan groups over the first 2 years after implantation. (J Thorac Cardiovasc Surg 2001;121:804-11)

Intraoperative transesophageal echocardiography (IOTEE) is commonly used to assess for residual defect and the need to return to bypass after repair of ventricular septal defect (VSD). The frequency and significance of residual septal defects as noted on IOTEE has not been well defined. We evaluated the frequency of residual VSD via IOTEE and the relationship between size of a residual VSD and rate of reoperation. In addition, we looked at the relationship between the presence of a residual VSD via IOTEE and the presence of residual VSD at follow‐up transthoracic echocardiography (TTE). Residual VSD was measured via the largest width of the Doppler color jet diameter originating at the left ventricular septal surface. Of the 294 patients evaluated with IOTEE after VSD repair, one‐third had a residual defect by IOTEE Doppler color flow mapping. Two‐thirds of these defects closed spontaneously on TTE by the time of hospital discharge. There was no difference in frequency of residual VSD between simple (VSD closure alone, n = 90) and complex (VSD with associated lesions, n = 204) repair. Return to bypass with immediate reoperation was undertaken in nine patients, all of whom had significant shunt via oximetry (Qp/Qs > 1.5:1.0). All had residual VSD color jet diameters > 3 mm. Seven patients had residual color jet equal to 3 mm; however, hemodynamic studies did not reveal a significant shunt and none of these had reoperation. Seven patients with no VSD or < 3 mm residual VSD via had late reoperation to close residual VSD at 4 days to 5 months after initial operation. These were due to patch dehiscence or development of an “intramural” VSD in patients with conotruncal anomaly. A residual defect on IOTEE color Doppler measuring ≥ 4 mm predicts the need for immediate reoperation, while a 3 mm defect may be significant and requires additional intraoperative hemodynamic evaluation. The majority of small defects noted on IOTEE are not present at discharge TTE. Patients with conotruncal defect repair should be followed closely for development of late significant “intramural” defects.

Pulmonary vein stenosis describes a variety of entities with a variable clinical course. The basic pathologic process appears to be fibrous intimal thickening, which can gradually cause obliteration of the lumen of the pulmonary veins at the atrial junction. In its most severe form, congenital pulmonary vein stenosis is a progressive disease with rapid pulmonary hypertension and rare survival beyond the first year of life. Surgical intervention has not been successful in this group. Other forms of pulmonary vein stenosis, including unilateral types associated with congenital heart disease, may be managed by various surgical treatments or pneumonectomy in some cases. Although multiple surgical techniques have been described for repair of congenital vein stenosis, the “sutureless” techniques recently described may have a place in the surgical treatment of this condition. In the most severe forms, lung transplantation appears to be an important option that should be considered early in the course of the disease.

Objectives/Hypothesis: To review the clinical presentation and diagnostic evaluation of patients with symptomatic congenital vascular anomalies causing tracheobronchial compression and to establish the short‐ and long‐term results of surgical intervention with respect to postoperative complications, persistent symptoms, and ventilator and tracheostomy dependence. Study Design: Retrospective review. Methods: Chart review and telephone follow‐up. Results: Between 1987 and 1996, 35 children underwent surgical intervention to relieve symptomatic tracheobronchial compression resulting from a congenital vascular anomaly. Historically, the onset of symptoms occurs within the first months of life; however, only 12 (34%) of patients were diagnosed by 6 months of age and 13 (37%) were diagnosed at greater than 1 year of age. Excluding anomalous innominate artery, chest radiography or barium swallow was suggestive of a congenital vascular anomaly in 30 (94%) of the patients. Magnetic resonance imaging correctly delineated the anatomy of the vascular anomaly in 29 patients. Bronchoscopy was diagnostic in all three patients with anomalous innominate arteries causing tracheal compression. Postoperative follow‐up was obtained in 32 (91%) of patients; 25 (78%) of these were asymptomatic at the time of their most recent examination. The remaining patients had persistent stridor, recurrent respiratory tract infections, and/or chronic cough. In all three patients who underwent postoperative bronchoscopy for persistent symptoms, tracheomalacia was demonstrated in the region of previous compression. Conclusions: Tracheobronchial compression from congenital vascular anomalies is a rare but treatable cause of respiratory symptoms. Early diagnosis requires a prompt, thorough clinical and radiologic evaluation. Surgery affords excellent long‐term resolution of symptoms.

OBJECTIVES

Ten dogs underwent orthotopic cardiac transplantation after preservation of the donor heart for 24 hours in an oxygenated hypothermic, hypertonic, intracellular solution, either with (five dogs) or without (five dogs) continuous, oxygenated, low-pressure perfusion. Eight dogs survived for 24 hours after transplantation, at which time they were put to death. The two nonsurvivors were among the five with nonperfused hearts. Examination of all 10 donor hearts showed differences between the two groups: Four of five nonperfused hearts showed severe transmural myocardial coagulation necrosis but only small foci of contraction band necrosis (myofibrillar degeneration). The perfused hearts, however, showed more extensive subendocardial areas of contraction-band necrosis, but only minimal and focal coagulation necrosis, indicating less severe hypoxic damage. These results indicate that oxygenated perfusion with a hypothermic, hypertonic, intracellular solution may permit improved transplant survival after extended cardiac preservation.