Repair of coarctation in a right-sided circumflex retroesophageal aortic arch.

We describe an unusual case of a 3-month-old infant with normal cardiac situs and coarctation of the aorta occurring proximal to the right subclavian artery in the presence of a right-sided retroesophageal circumflex aortic arch and aberrant left subclavian artery. Preoperative evaluation with magnetic resonance imaging and conventional aortography led us to surgically approach this lesion through a right thoracotomy with a successful outcome.

Transcatheter closure of patent ductus arteriosus with the Amplatzer duct occluder: short-term follow-up.

BACKGROUND: Transcatheter closure of the patent ductus arteriosus is a well established procedure. The objective of this study was to assess the effectiveness and the safety of the Amplatzer duct occluder. METHODS AND RESULTS: Occlusion of the patent ductus arteriosus was attempted in 23 patients. The median weight was 11.7 Kg (range, 5 kg - 42.4 kg) with a mean ductus diameter of 3.7 mm (range, 1.6 - 7.2 mm). The immediate closure rate was 86% with a closure rate of 100% at 6 months, 1 year and 2 years following device placement. There was one device embolization that occurred immediately following device placement. No patient had aortic narrowing or pulmonary artery stenosis following the procedure. CONCLUSIONS: The Amplatzer duct occluder is safe and effective in the closure of a patent ductus arteriosus up to 7.2 mm in diameter. Selecting a device at least 12 mm larger than the minimal ductal diameter can minimize embolization.

HIRA, a DiGeorge syndrome candidate gene, is required for cardiac outflow tract septation.

DiGeorge syndrome (DGS) is a congenital disease characterized by defects in organs and tissues that depend on contributions by cell populations derived from neural crest for proper development. A number of candidate genes that lie within the q11 region of chromosome 22 commonly deleted in DGS patients have been identified. Orthologues of the DGS candidate gene HIRA are expressed in the neural crest and in neural crest-derived tissues in both chick and mouse embryos. By exposing a portion of the premigratory chick neural crest to phosphorothioate end-protected antisense oligonucleotides, ex ovo, followed by orthotopic backtransplantation to the untreated embryos, we have shown that the functional attenuation of cHIRA in the chick cardiac neural crest results in a significantly increased incidence of persistent truncus arteriosus, a phenotypic change characteristic of DGS, but does not affect the repatterning aortic arch arteries, the ventricular function, or the alignment of the outflow tract.

Calf blood pressure: clinical implications and correlations with arm blood pressure in infants and young children.

OBJECTIVE: Indirect measurement of lower extremity blood pressure is often used in the clinical setting, although normative data after the newborn period are not readily available. METHODS: Indirect blood pressure (BP) measurement was obtained in the right arms and right calves of 148 healthy infants and young children 2 weeks to 3 years of age. All measurements were made using an oscillometric device. The infants and children are quiet or asleep and in the supine position. A BP cuff of proper size was chosen. Three measurements were made in both extremities; the average of the second and third measurements was used for all analyses. RESULTS: Age correlated better with calf systolic blood pressure (SBPc) than with arm SBP (SBPa) (r = .52 vs .17). Calf diastolic blood pressure (DBPc) and calf mean blood pressure (MBPc) correlated moderately poorly with age (r = .37 and .39, respectively). There was no order effect. SBPc correlated best with height (r = .53), then age (r = .52), and, finally, weight (r = .51). The correlation between BPc and BPa was moderately low. The correlation of SBPc with SBPa was r = .46; that of DBPc with DBPa was r = .37; and that of MBPc with MBPa was r = .41. From birth to 6 months, SBPc was slightly lower than SBPa (1 to 3 mm Hg). SBPc increased linearly relative to SBPa and began to exceed SBPa at 6 months of age. The pattern of DBP and MBP was similar. Wide variability of blood pressure parameters was noted between the infants and children at all ages. CONCLUSIONS: Reference data are presented for BPc and the difference between BPc and BPa in healthy infants and children from 2 weeks to 3 years of age. BPc is not equivalent to BPa and should not be arbitrarily substituted. Because of the wide variability among healthy infants and children, SBPc measurements should be interpreted with caution when evaluating for coarctation of the aorta.

Evaluation and recommendations for participation in athletics for children with heart disease.

Physical fitness is becoming increasingly emphasized in our society. Everyone is encouraged to participate in routine exercise, and many desire to compete in organized athletics. Patients with heart disease are living longer, healthier lives and may also desire sports participation. Because selected patients may be at risk for sudden death, a thorough evaluation should be performed. The purpose of this article is to familiarize the reader with basic principles of exercise physiology and preparticipation evaluation and to provide a guideline to recommending athletic involvement in patients with heart disease.