Central aortic blood pressure estimation in children and adolescents: results of the KidCoreBP study.

BACKGROUND: Central aortic SBP (cSBP) may have superior prognostic value compared with peripheral SBP (pSBP), but noninvasive cSBP measurement techniques have not been formally validated in children and adolescents. METHOD: This study assessed the accuracy of two automated devices and the radial tonometry/transfer function method (RT-TF) for estimating central pressures and pulse pressure amplification (PPA) in this population, with adherence to validation guidelines for central pressure devices. In 69 children/adolescents aged 3-18 years undergoing clinically indicated aortic catheterization, high fidelity ascending aortic cSBP was measured with a micromanometer-tipped wire and compared with values from SphygmoCor XCEL, Mobil-O-Graph (systolic/diastolic calibration, MoG-C1, or mean/diastolic calibration, MoG-C2) and RT-TF. Reference intra-arterial pSBP was derived from the tonometry pulse calibrated to central mean/diastolic pressures. RESULTS: XCEL, MoG-C1 and MoG-C2 overestimated cSBP by 7.9 ±â€Š6.8 mmHg (mean ±â€ŠSD), 5.7 ±â€Š10.3 mmHg, and 19.1 ±â€Š14.9 mmHg, exceeding the validation cut-off (5 ±â€Š8 mmHg). Brachial pSBP was also overestimated by XCEL (10.9 ±â€Š8.4 mmHg) and Mobil-O-Graph (11.5 ±â€Š12.3 mmHg). By contrast, central and brachial diastolic pressures were underestimated by the automated devices, albeit mostly within acceptable limits; pulse pressures were, therefore, substantially overestimated. Central-brachial PPA (4.5 ±â€Š4.4 mmHg) was overestimated by XCEL (8.7 ±â€Š3.2 mmHg) and MoG-C1 (11.1 ±â€Š6.4 mmHg), but underestimated by MoG-C2 (-3.0 ±â€Š6.6 mmHg). Given accurate pulse calibration, RT-TF achieved acceptable accuracy for cSBP (-0.2 ±â€Š4.6 mmHg) and central-radial PPA (1.9 ±â€Š5.1 mmHg). CONCLUSION: In conclusion, XCEL and Mobil-O-Graph overestimated pSBP and cSBP in children and adolescents. cSBP can be obtained via the same transfer function used in adults, but accurate pressure pulse calibration is critical.Video Abstracts: http://links.lww.com/HJH/B222.

Management of an unusual left coronary artery to right ventricular fistula in a neonate.

Congenital coronary arterial fistula is a rare cause of congestive heart failure in neonates. We describe a neonate who developed early symptoms because of an unusually large left coronary artery to right ventricular fistula. Computed tomography and coronary angiography delineated anatomy and facilitated successful surgical closure.

Intravascular stent implantation for the management of pulmonary artery stenosis.

Pulmonary artery stenosis is a challenging problem in the management of congenital heart disease. Untreated pulmonary artery stenosis may contribute to increased mortality and morbidity, and lead to suboptimal results following surgical repair of congenital heart disease. Intravascular stent implantation has emerged as one of the preferred treatment options for pulmonary artery stenosis. However, issues regarding the effectiveness and complications of stent implantation for pulmonary artery stenosis need to be identified. In addition, difficulties of stent implantation in the paediatric setting, as a consequence of small vessel size and subsequent vessel growth, are also important considerations. This review will evaluate the short and long-term effectiveness, the outcomes and complications, and discuss the potential problems of stent implantation for pulmonary artery stenosis.

Cardiac cirrhosis and hepatocellular carcinoma in a 13-year-old treated with doxorubicin microbead transarterial chemoembolization.

Increasing numbers of children are surviving into adulthood following surgery to correct or palliate congenital heart disease. This surgery can occasionally result in long-standing elevated right heart pressures and chronic hepatic venous congestion leading to cardiac cirrhosis. We report the first paediatric case of hepatocellular carcinoma in the setting of cardiac cirrhosis. A 13-year-old girl developed inoperable hepatocellular carcinoma and was treated with transarterial embolization with lipiodol and doxorubicin eluting microbeads. Promoting awareness of this association, even in younger patients, will hopefully result in better surveillance and screening of hepatic complications in survivors of complex cardiac surgery.

Intracardiac echocardiography during transcatheter interventions for congenital heart disease.

Although transesophageal echocardiography is often used for guidance during transcatheter interventions, few data exist regarding the use of the newer modality of intracardiac echocardiography. This brief report summarizes our single center experience using intracardiac echocardiographic guidance during transcatheter interventional procedures for congenital heart disease.

Transcatheter angioplasty for acquired pulmonary vein stenosis after radiofrequency ablation.

BACKGROUND: Pulmonary vein stenosis has recently been recognized as a complication of radiofrequency ablation for atrial fibrillation. This study evaluates the presentation of affected patients and the role of transcatheter therapy for this patient population. METHODS AND RESULTS: This study used a retrospective review of data from 19 patients (age, 51+/-13 years) with pulmonary vein stenosis who underwent catheterization and angiography between December 2000 and December 2002. Quantitative perfusion and spiral CT scans were performed for initial diagnosis and follow-up. The median duration between radiofrequency ablation and the reported onset of respiratory symptoms for 18 of 19 patients was 7.5 weeks (0.1 to 48). After the onset of symptoms, all but two patients were initially misdiagnosed with a symptoms-to-diagnosis duration of 16 weeks (2-59). At initial catheterization, 17 of 19 patients had angioplasty in 30 veins with stent placement in 5 vessels when a flap occurred. Overall vessel diameter increased from 2.6+/-1.6 to 6.6+/-2.4 mm (P<0.0001). There were 4 procedure-related adverse events but no long-term sequelae. Immediate follow-up showed improved flow to involved lung segments. At a median follow-up of 43 weeks (2-92), although repeat angioplasty for restenosis was necessary in 8 of 17 patients, 15 of 17 patients currently have no or minimal persistent symptoms. CONCLUSIONS: Pulmonary vein stenosis after radiofrequency ablation for atrial fibrillation is often misdiagnosed. Although further follow-up is necessary to determine long-term success, our data indicate better pulmonary vein flow and symptomatic improvement in the majority of patients undergoing dilation of postablation pulmonary vein stenosis.

Results of the double switch operation for congenitally corrected transposition of the great arteries.

OBJECTIVE: To determine the outcomes for a program that utilizes the double switch operation as the primary approach for congenitally corrected transposition. METHODS: The records of 46 consecutive patients from a single institution who had undergone a double switch operation from October 1993 to March 2002 were reviewed. The records of 24 patients who were evaluated during the same period and felt not to be candidates for the double switch operation or who are awaiting double switch after pulmonary artery banding were also reviewed. RESULTS: The median age at operation was 28 months (range 2 months to 16.3 years). Associated defects included ventricular septal defect 40, pulmonic stenosis 13 and pulmonary atresia 16. Twenty-six patients underwent an arterial switch operation combined with a Senning procedure while 20 patients underwent combined Rastelli and Senning procedures. Before the double switch, 12 patients had required pulmonary artery banding and 21 patients had systemic to pulmonary artery shunts. The median duration of stay in the intensive care unit was 3.5 days (range 2-60 days) and the median duration of total hospital stay was 8 days (range 5-60 days). There were no hospital deaths; one patient died 5 months after discharge due to an arrhythmogenic cardiac arrest during a median follow-up of 24 months [long-term survival 98% (95% CI 89-100%)]. CONCLUSIONS: The double switch operation may be performed with excellent hospital and long-term survival. The theoretical advantages of this procedure which enables the morphologic left ventricle and mitral valve to support a systemic pressure load must be established by careful follow-up of these patients.

Transcatheter interventions across an inferior vena cava filter.

Patients who suffer from an embolic event often undergo placement of an inferior vena caval filter. Few data are available regarding treatment of this patient population with concurrent right-to-left shunting across an atrial communication. We report four cases of transcatheter device closure of an atrial communication across an inferior vena cava filter.

Invasive echocardiography: the use of catheter imaging by the interventional cardiologist.

Ultrasound imaging is frequently used for diagnostic purposes or guidance during procedures in the pediatric and congenital cardiac catheterization laboratory. As new imaging modalities emerged, many interventional cardiologists rather than noninvasive specialists are now performing the ultrasound imaging as part of the catheterization. The focus of this discussion will be to detail the technique and application of echocardiography by the interventional cardiologist.

Cutting balloon angioplasty for children with small-vessel pulmonary artery stenoses.

Patients with complex congenital heart disease may have pulmonary artery stenoses that are either congenital or associated with scarring following surgical procedures. This study evaluates cutting balloon angioplasty for small-vessel pulmonary artery stenoses resistant to standard balloon angioplasty. Between October 1998 and December 1999, patients were enrolled in an FDA-approved compassionate-use protocol. During four catheterizations, there were seven lesions found resistant to standard balloon angioplasty (mean lesion diameter was unchanged: 1.8 mm +/- 0.8 mm to 1.9 +/- 0.8 mm). A cutting balloon was inflated twice in each of these lesions. Standard balloon angioplasty was then repeated. Final mean lesion diameter was increased significantly (1.9 mm +/- 0.8 mm to 3.8 +/- 1.3 mm; P