Transcatheter interventions can be successfully performed in the early postoperative period. These procedures can have a positive impact on patient outcome; however, they should be performed only by a pediatric interventional cardiologist supported by a multi-disciplinary team.

Initiation of a high-risk program might decrease interstage mortality after high-risk neonatal palliative operations. Such an approach might contribute to earlier detection of significant residual/recurrent lesions amenable to therapy.

Patients undergoing congenital heart surgery may occasionally require additional surgical procedures in the form of tracheostomy and gastrostomy. These procedures are often performed in an attempt to diminish hospital morbidity and length of stay. We reviewed the Web-based medical records of all patients undergoing congenital heart surgery at Miami Children's Hospital from February 2002 through August 2007. Patients who were deemed preterm and had undergone closure of a patent ductus arteriosis were eliminated. The records of all other patients were queried for the terms gastrostomy, g-tube, Nissan, fundal plication, tracheostomy, or tracheotomy. Patients' medical records in which these terms appeared in any portion were completely reviewed. There were 1660 congenital heart operations performed in the study period. There were 592 operations performed on patients whose age ranged from 1 month to 1 year and 441 neonatal operations. Mortality was 2%. Median postoperative stay was 8 days (range, 1-191 days), 12 days for neonates (range, 3-142 days), and 19 days for neonates undergoing RACHS-1 category 6 operations (range, 4-142 days). Tracheostomies were performed in four patients (0.2%). Gastrostomies were performed on eight patients (0.4%), representing 0.8% of patients <1 year of age, 1.4% of neonates, and 2.4% of patients undergoing RACHS-1 category 6 operations. The rate of patients undergoing either tracheostomy or gastrostomy after congenital heart surgery at our institution was quite low. Avoidance of either of these two procedures was achieved without increased morbidity or length of stay. The rate at which these procedures need to be performed may reflect the magnitude of the patients' lifetime trauma related to their underlying condition and acute and total surgical experiences.

To define the incidence, course, and etiology of hematologic abnormalities in children on tacrolimus-based immunosuppression, we reviewed records of 106 transplant patients (70 heart, 16 heart and lung, 20 double lung), 0-21 yr of age, who were transplanted at the Children's Hospital of Pittsburgh from 1989 to 1997. Fifty-four of the 106 patients (51%) developed 65 abnormal hematologic episodes (32 anemia, nine neutropenia, nine thrombocytopenia, 15 simultaneous anemia and neutropenia with or without thrombocytopenia). Common etiologies included: infections, post-transplant lymphoproliferative disease, and medications. Eleven episodes (seven anemia, one neutropenia, and three simultaneous anemia and neutropenia) had unclear etiologies and process of elimination suggested an association with tacrolimus. Interventions included filgrastim (effective in 15 of 15 patients, with resolution of neutropenia in a median of 5 days) and epoetin alfa (effective in five of 16 patients, including four of four patients with anemia possibly related to tacrolimus). Five patients (two with neutropenia and three with simultaneous neutropenia and anemia) were switched to cyclosporin A (CsA); rapid resolution occurred in four of the five patients, suggesting a possible association of the hematologic abnormalities with tacrolimus. In summary, hematologic abnormalities are common in children on tacrolimus-based immunosuppression. Most of these hematologic abnormalities are caused by common etiologies; however, a sub-population exists where tacrolimus may be the etiologic agent. Anemia and neutropenia respond to treatment with epoetin alfa and filgrastim. After thorough investigation, a trial switch to CsA may be warranted.

We report the first implantation of two Helex septal occluder devices in a 10-year-old boy with complex atrial septal anatomy. The unique characteristics of this new device are discussed in the following case report.

Mutations in PRKAG2 gene that regulates the γ2 subunit of the adenosine monophosphate (AMP) dependent protein kinase have been associated with the development of atrioventricular (AV) accessory pathways, cardiac hypertrophy, and conduction system abnormalities. These patients can potentially be misdiagnosed as hypertrophic cardiomyopathy (HOCM) and/or Wolf-Parkinson White (WPW) syndrome due to similar clinical phenotype. Early recognition of this disease entity is very important as ablation of suspected accessory pathways is not effective and the natural history of the disease is very different from HOCM and WPW syndrome.

Children born with hypoplastic left heart syndrome are at high risk for serious morbidity, growth failure, and mortality during the interstage period, which is the time from discharge home after first-stage hypoplastic left heart syndrome palliation until the second-stage surgical intervention. The single-ventricle circulatory physiology is complex, fragile, and potentially unstable. Multicenter initiatives have been successfully implemented to improve outcomes and optimize growth and survival during the interstage period. A crucial focus of care is the comprehensive family training in the use of home surveillance monitoring of oxygen saturation, enteral intake, weight, and the early recognition of "red flag" symptoms indicating potential cardiopulmonary or nutritional decompensation. Beginning with admission to the intensive care unit of the newborn with hypoplastic left heart syndrome, nurses provide critical care and education to prepare the family for interstage home care. This article presents detailed nursing guidelines for educating families on the home care of their medically fragile infant with single-ventricle circulation.

Sinus of Valsalva aneurysm (SVA) is defined as a weakness in the aortic valve wall, immediately above the attachments of each of the aortic cusps. This weakness can rupture and create an aortocardiac fistula. There are many congenital heart defects associated with chromosome 22q11 deletion, especially involving the aortic arch and its branches. SVA is not an anomaly usually associated with chromosome 22 deletion. We report the case of a 19-year-old female who presented to our institution with SVA rupture. She was subsequently diagnosed with chromosome 22q11 deletion syndrome. Despite dysmorphic facial features and a learning disability, our patient had not been diagnosed with the chromosome abnormality. SVA is a rare congenital heart defect and has only once previously been reported in a child with a chromosome 22q11 deletion. We report the first case where aneurysm rupture preceded the chromosomal findings. Chromosome 22q11 deletion could be missed due to either the unfamiliarity of physicians with the syndrome or the variability and subtlety of the phenotype. This was demonstrated by our patient who, at age 19 after presenting with an SVA rupture, prompted physicians to find an explanation for her coexisting dysmorphic features and her learning disability.