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Myxoma, the most common primary cardiac tumor in adults, is rare in neonates. We describe a myxoma arising from the infundibulum of the right ventricle causing significant outflow tract obstruction in an otherwise normal newborn. Serial echocardiograms revealed an increasing gradient across the right ventricular outflow tract prompting surgery. The patient underwent successful excision of the myxoma with an uneventful recovery.

Background: This study was performed to evaluate and compare the early, intermediate, and long-term outcomes of the bidirectional Glenn procedure and Fontan procedure in patients who live at moderately high altitude., Methods: The outcome of each method of palliation for patients with a functionally single ventricle was retrospectively evaluated from a review of medical records., Results: The bidirectional Glenn procedure was performed in 177 patients from October 1984 to June 2004. The Fontan procedure was performed in 149 patients from June 1978 to June 2004. Cardiovascular death or heart transplantation occurred in 8% of patients after the bidirectional Glenn procedure and 17% of patients after the Fontan procedure. Complications of systemic thromboembolic events, bleeding associated with anticoagulation therapy, protein losing enteropathy, and arrhythmias requiring implantation of a pacemaker, cardioversion, or radiofrequency ablation occurred in 7% of patients after the bidirectional Glenn procedure and 47% of patients after the Fontan procedure. Cardiovascular deaths and heart transplantation occurred less frequently when the Fontan procedure was performed in patients with a previous bidirectional Glenn procedure. However, the actuarial transplant-free survival and freedom from complications was not superior for a subgroup of patients who had a Fontan procedure after a bidirectional Glenn procedure in comparison to a subgroup of patients who had a bidirectional Glenn procedure alone., Conclusions: The bidirectional Glenn procedure can be used for long-term palliation of patients with a functionally single ventricle. Additional palliation with a Fontan procedure may increase the risk of stroke, protein losing enteropathy and arrhythmias without improving survival.

We describe a case of a 3-year-old child with an intrapericardial bronchogenic cyst arising from the pericardium overlying the pulmonary artery found incidentally at the time of elective repair of a sinus venosus atrial septal defect with partial anomalous pulmonary venous return. A cystic mass overriding the right pulmonary artery was resected and diagnostic studies revealed multiple loculated cysts lined by respiratory mucosa with cartilage and smooth muscle in the wall consistent with a bronchogenic cyst. The cyst was completely resected and postoperative recovery was uneventful.

Extracorporeal membrane oxygenation (ECMO) has become a valuable adjunct in caring for infants and children with heart disease. Since the initial reports of ECMO support for cardiac failure in children, the number of centers providing cardiac support and the number of cases of cardiac ECMO have steadily increased. The International Registry for Extracorporeal Life Support Organization has reported survival statistics for cardiac cases in neonates, children, and adults ranging from 33% to 43%. These numbers are similar to the survival from recent reports by Morris (39%) and Chaturvedi (49%). Survival is influenced by ability to be weaned from bypass in the operating room and by residual structural disease and multi-organ system failure but not by cardiac arrest and single ventricle physiology. To improve results in the future, we need to focus on better predicting the need for support and avoiding multi-organ system failure before initiating ECMO. Rapid deployment of ECMO may further improve results for patients who deteriorate suddenly in the intensive care unit.

Background: The incidence of rheumatic heart disease (RHD) has increased recently in the western United States. We reviewed our 18-year surgical experience with RHD in children to examine current surgical techniques and results., Methods: From 1985 until 2003, 596 children (<21 years) with rheumatic fever were seen at Primary Children's Medical Center. Rheumatic carditis was diagnosed in 366 patients (61.4%). Twenty-six with carditis (26/366, 7.1%) required operation for rheumatic valve disease including 8 for mitral regurgitation, 7 for mitral and aortic regurgitation, 4 for aortic regurgitation, 4 for mitral regurgitation and stenosis, 2 for combined mitral stenosis and regurgitation with aortic insufficiency, and 1 for mitral and tricuspid regurgitation., Results: Mean age at operation was 13.5 +/- 4 years. Three patients required operation during the acute phase of rheumatic fever (< 6 weeks), 2 during the subacute phase (< 6 months), and 21 during the chronic phase after the episode of rheumatic fever (6.7 +/- 3 years). Mitral valve repair was possible in 19 of 22 patients who required mitral operation. Aortic valve repair was possible in 4 patients whereas replacement was necessary in 9, including 2 Ross procedures. No operative deaths were recorded and 2 late deaths occurred at 4.6 and 10 years. Actuarial survival was 94% at 5 years and 78% at 10 years. Six patients required reoperation; actuarial freedom from reoperation was 78% at 5 years, 65% at 10 years, and 49% at 15 years. All survivors are in New York Heart Association class I or II., Conclusions: Children with RHD in the United States uncommonly require valve operation. Mitral repair with a technique that allows annular growth is possible in most children with good long-term functional results. Long-term surveillance of children with RHD is necessary because of the possible need for late valve operation.

Background: Recognition of the immunogenicity of standard cryopreserved allografts has led to the development of new decellularized allografts (CryoValve SG; CryoLife, Inc, Kennesaw, Ga). This preliminary study examined the HLA antibody response to these decellularized allografts and compared it with the response to standard allograft material., Methods: We prospectively measured the frequency of panel-reactive HLA class I (HLA-A, HLA-B, and HLA-C) and class II (HLA-DR/DQ) alloantibodies in 14 children (age 8.5 +/- 7.9 years) receiving decellularized, cryopreserved allografts, including 6 undergoing allograft patch insertion and 8 with a valved pulmonary allograft. We compared them with 20 historical control subjects (age 1.7 +/- 2.4 years) undergoing implantation of standard cryopreserved allografts, 8 with valves and 12 with allograft patch. All patients had panel-reactive antibody levels measured before and at 1, 3, and 12 months after the operation. HLA class I and class II panel-reactive antibody levels were determined with a sensitive flow cytometry technique., Results: We found panel-reactive antibody levels in decellularized allografts to be elevated slightly from preoperative levels for both class I and class II antibodies at 1, 3, and 12 months (P >.05). The panel-reactive antibody level for both class I and class II antibodies were significantly lower for decellularized allografts as compared to standard allografts. Functionally, the allografts were similar with decellularized valved grafts showing a peak echo-determined systolic gradient of 13 +/- 15 mm Hg at 8 +/- 2.6 months postoperatively as compared to a gradient of 24 +/- 18 mm Hg measured 12 +/- 6 months postoperatively in standard allografts (P =.11)., Conclusions: Decellularized grafts elicited significantly lower levels of class I and class II HLA antibody formation at 1, 3, and 12 months after implantation than did standard cryopreserved allografts. Early hemodynamic function of decellularized grafts was similar to that of standard cryopreserved allograft valves. Further experience is necessary to determine whether the reduced immunogenicity of decellularized allografts will truly allow tissue ingrowth and improved long-term durability in patients.

Children with dilated cardiomyopathy awaiting transplantation who fail maximal pharmacologic therapy may benefit from intra-aortic balloon pumping. Between July 1993 and August 1999, a total of 4 children with dilated cardiomyopathy underwent pre-transplant balloon pumping for 6.0 +/- 5.8 (1 to 12) days. One child (pumped for 12 days) died awaiting transplant, and the remaining 3 were successfully transplanted. Intra-aortic balloon pumping timed precisely with M-mode echocardiographic markers offers a relatively simple and safe intermediate level of mechanical support for children with dilated cardiomyopathy who fail pharmacologic support.

Cardiac transplantation provides the best option for neonates with congenital heart disease that is not amenable to surgical repair or palliation. The scarcity of suitable organs for this group has resulted in prolonged waiting times; many infants die awaiting transplantation. We present the case of a newborn with severe Ebstein's anomaly and low cardiac output who was supported with extracorporeal membrane oxygenation for 1,126 hours, until an appropriate organ became available.

Ebstein's anomaly of the tricuspid valve is characterized by a regurgitant tricuspid valve with a large anterior leaflet and septal and tricuspid leaflets displaced into the right ventricle. Associated anomalies are common, especially atrial septal communication and right ventricular outflow tract obstruction. Clinical presentation includes a spectrum from minimal hemodynamic derangement and mild exercise intolerance to severe cardiopulmonary abnormalities and grave illness. The natural history of the disease is extremely variable; the worst prognosis is in the youngest patients, who often have associated cardiac abnormalities. Operative procedures range from ligation of a patent ductus arteriosus to cardiac transplantation. Tricuspid valvuloplasty has been advanced by several investigators and can be considered in nearly all cases. Repairs are based on the creation of a monocuspid or bicuspid valve with reduction of the enlarged annulus. While tricuspid valve repair or replacement is indicated for symptomatic children and adults, the management of the symptomatic neonate is difficult. Patients are stratified according to clinical status and morphology, then treated with observation, valve repair, conversion to single ventricle, or transplantation. We review the anatomy and pathophysiology of Ebstein's anomaly, the indications for surgery, and the appropriate timing of surgical intervention. Current techniques of tricuspid valvuloplasty are described and unresolved issues are discussed, including the role for superior cavopulmonary anastomosis, the management of symptomatic infants, and the necessity of ventricular plication in valve repair. Copyright 1999 by W.B. Saunders Company