Angiojet thrombectomy for Blalock-Taussig shunt and pulmonary artery thrombus in an infant with tetralogy of fallot.

We describe a new technique for treatment of shunt thrombosis in infants with complex anatomical defects. A 2-month-old girl with Tetralogy of Fallot underwent placement of a modified Blalock-Taussig shunt (MBTS) at day of life (DOL) 6 with revision at DOL 20. Following this surgery, the patient became hypotensive and hypoxic with new evidence of lack of flow through the MBTS on echocardiography. Angiography showed an occluded MBTS and right pulmonary artery with patent distal branches with normal pulmonary venous return. Balloon angioplasty was attempted but failed to fully recanalize the right pulmonary artery (RPA) and MBTS. An AngioJet catheter was then passed through the shunt and RPA to perform rheolyticthrombectomy. Subsequent angiogram showed a widely patent RPA and MBTS. An echocardiogram at 1-month post-thrombectomy showed a widely patent MBTS with continuous flow seen entering both branch pulmonary arteries. The AngioJet system for thrombectomy provides a viable option for complex thrombus removal in patients refractory to other methods.

Cardiac function in congenital adrenal hyperplasia: a pattern of reversible cardiomyopathy.

OBJECTIVE: To evaluate cardiac function in infants with congenital adrenal hyperplasia (CAH) before and after corticosteroid replacement therapy. STUDY DESIGN: This prospective, case-control study included 9 infants with CAH. Cardiac function was assessed by echocardiography at presentation and after corticosteroid replacement therapy. Six term infants underwent 2 echocardiograms each and served as the control group. Data on fractional shortening (FS), rate-corrected velocity of circumferential fiber shortening (Vcf), wall stress, tissue Doppler indices, myocardial performance index, left ventricular mass, and Vcf/wall stress were obtained. RESULTS: The infants with CAH exhibited myocardial dysfunction at baseline and lower systolic blood pressure (SBP) compared with the control group. FS, a measure of systolic contractility, differed significantly from before to after corticosteroid treatment (mean, 32.3%±4.7% pretreatment, 39.9%±5.0% posttreatment). Vcf, a preload-independent measure of cardiac contractility, also differed significantly before and after treatment (mean, 1.23±0.16 circumferences/second pretreatment, 1.45±0.22 circumferences/second posttreatment). SBP was also lower (mean, 84±9.3 mmHg) and improved with treatment (mean, 95±4.8 mmHg). The control group demonstrated no statistically significant changes in FS, Vcf, or SBP. There was a change in left ventricular mass in the control group between the 2 studies. CONCLUSION: Newborns with CAH have evidence for cardiac dysfunction at baseline that reverses with corticosteroid replacement therapy. These data suggest that corticosteroids play a direct role in modulating cardiac function in the newborn.

Cardiac catheterization and operative outcomes from a multicenter consortium for children with williams syndrome.

Williams syndrome is a multifaceted disorder that includes a spectrum of cardiovascular anomalies. Due to its rare occurrence, outcome data for operations and cardiac catheterization are limited. We reviewed data from 242 individuals from the Pediatric Cardiac Care Consortium (PCCC) with Williams syndrome and associated cardiovascular lesions, and their frequency, and assessed their effects on mortality. In the PCCC, from 1984 to 1999 there were approximately 100,000 entries for cardiac procedures, involving more than 62,000 patients. The diagnosis of Williams syndrome was based on clinical features and determined by each site. Most patients were diagnosed with the availability of the FISH probe for region 7q11.23. Using a spreadsheet application, Microsoft Excel, the selected patients were analyzed for various types of cardiac anomalies. The most common cardiovascular lesions and the mortality rate in patients with Williams syndrome were examined. A complete tabulation of all cardiovascular lesions was assembled. There were 292 catheterizations and 143 operations reported to the PCCC. One hundred six patients had both an operation and a catheterization. The three main cardiovascular anomalies were supravalvular aortic stenosis (SVAS; 169), pulmonary artery stenosis (PAS; 130), and coarctation or aortic arch hypoplasia (Arch; 32). One hundred five patients had a single lesion, 70 with SVAS, 29 with PAS, and 6 with an arch anomaly. Ninety-two had more than one lesion: 80 with SVAS and PAS, 7 with PAS and Arch, and 5 with SVAS and Arch. Seventy individuals have only SVAS, 29 PAS, and 6 Arch alone. There was a total of 15 deaths. The mortality rate was highest in the group with the combination of SVAS and PAS (7 surgical and 5 catheter; 12 of 80 patients [15%]; p = 0.0001, chi(2)). In conclusion, our data represent the largest collection of individuals with Williams syndrome who underwent cardiac catheterization and/or operation. The data suggest that children with Williams syndrome and bilateral outflow tract obstruction have statistically and clinically significantly higher mortality associated with catheterization or operation.

A novel approach incorporating sildenafil in the management of symptomatic neonates with Ebstein's anomaly.

The neonatal presentation of Ebstein's anomaly is a distinct cyanotic lesion for which little can be done other than to maintain ductal patency or perform palliative surgery if improvement does not occur. We report two cases in which sildenafil was introduced to assist previously failed attempts at weaning of prostaglandin.

Impact of conventional versus biventricular pacing on hemodynamics and tissue Doppler imaging indexes of resynchronization postoperatively in children with congenital heart disease.

OBJECTIVES: We sought to evaluate the effects of biventricular (BDOO) pacing compared with conventional (CDOO) atrioventricular (AV) sequential and atrial (AOO) pacing in children and infants in the early postoperative period after open heart surgery for congenital heart disease (CHD). BACKGROUND: Biventricular pacing using right ventricular (RV) and left ventricular (LV) leads can improve hemodynamics in patients with CHD, but it is unclear whether this occurs in early postoperative children with CHD. METHODS: Nineteen children (age, 5 days to 5.4 years; median, 5.5 months) with a definitive biventricular repair for CHD underwent AOO, CDOO, and BDOO pacing with temporary epicardial leads for 10 min each. The AV delay was 80% of the PR interval for the CDOO and BDOO modes. Lead placement was two right atrial, two RV, and one LV. Blood samples for cardiac index (arterial and venous) and tissue Doppler (TDI) traces were obtained in each pacing mode with a Vivid 7 BT04 digital ultrasound system (GE/VingMed, Horten, Norway) from an apical four-chamber view and analyzed with EchoPac software. RESULTS: The QRS duration was significantly shorter for BDOO compared with CDOO, and the cardiac index was higher with BDOO compared with CDOO. Systemic blood pressure was not different between the three modes of pacing (AOO, CDOO, BDOO). The TDI-derived strain rate showed minimal dyssynchrony in AOO as seen by isovolumic tensing (IVT) and peak systolic contraction (PSC) timing differences between RV and LV. The CDOO worsened dyssynchrony with prolonged DeltaIVT and PSC. The BDOO showed improved synchrony as seen by DeltaIVT and PSC. CONCLUSIONS: The TDI-derived strain rate showed worsened ventricular dyssynchrony with CDOO and improvement with BDOO. Cardiac index and QRS duration were improved by BDOO compared with CDOO. This suggests that short-term pacing with BDOO may benefit children with CHD needing pacing in the postoperative period.

Advances in perioperative pacing.

Cardiac resynchronization therapy with biventricular pacing has become a significant management tool in adults with heart failure. In children, right rather than just left ventricular failure, is a key problem in the postoperative period. Also, congenital heart defects vary widely in their nature and prognosis. There are now preliminary reports in the literature of the use of multiple temporary pacing sites after congenital heart surgery and acute comparison of the effects of unsynchronized versus synchronized pacing in the postoperative period. These studies support the use of cardiac resynchronization pacing at least as a temporary measure in cases of acute heart failure after surgery in patients with congenital heart disease.