Impact of stent implantation on pulmonary artery growth.

BACKGROUND: Goals of stent implantation (SI) in children with pulmonary artery (PA) stenosis are to relieve obstruction and improve flow to the distal vasculature. We evaluated stent impact on distal PA growth. METHODS: We compared data of children who underwent unilateral SI using the nonstenotic contralateral PA (CPA) as a control (1998-2005; f/u data through 2009). Main/lobar diameters measured at initial and f/u catheterizations were analyzed. RESULTS: For single ventricle (SV) patients (N = 18), the stented PA diameter (SPA D) increased 118%. At initial f/u catheterization (14 ± 9.6 months), both upper lobe (UL) and lower lobe (LL) growth of SPA was comparable to those of the CPA (UL:7% vs. 7%; P = 0.97); (LL:5% vs. 10%; P = 0.33). Subsequent f/u in 11/18 patients (mean 26 ± 20 months) revealed similar results: both UL and LL growth of SPA were comparable to those of the CPA (UL:51% vs. 27%; P = 0.3); (LL:18% vs. 21%; P = 0.62). For two-ventricle (2V) patients (N = 21), the SPAD increased 100%. At f/u, UL, and LL growth on SPA was similar to those of the CPA (UL: 32% vs. 21%; P = 0.37); (LL: 17% vs. 18%; P = 0.88). Subsequent f/u in 10/21 patients (mean 34 ± 14 months) showed UL growth of SPA was significantly greater than that of the CPA (44% vs. 21%; P = 0.05). LL growth of SPA was similar to that of the CPA (19% vs. 14%; P = 0.56). CONCLUSION: SI for PA stenosis is effective in promoting normal lobar growth in SV and 2V patients. Greater lobar growth was seen in 2V compared to SV pts at first f/u. Early, aggressive PA stenting is beneficial in promoting lobar branch growth.

Catheter balloon adjustment of the pulmonary artery band: feasibility and safety.

The study aimed to assess the feasibility and safety of increasing pulmonary artery band (PAB) diameter by catheter-based PAB balloon dilation (PABBD). Eight dilations were performed between October 2006 and December 2008. Hemoclips were used to fix PABs surgically in a procedure designed to permit progressive clip dislodgment in a controlled manner. The PABBD resulted in gradual band loosening until the desired physiologic state was achieved. At time of PABBD, the patients had a mean age of 6 months (range 3-14 months) and a mean weight of 5 kg (range 2.6-7.3 kg). The median time from PAB placement until PABBD was 4.5 months (range 1-9 months). The single-balloon technique was used in seven cases (serial dilations in 5 cases) and the double-balloon technique in one case. The PABBDs were successful for all the patients, who experienced a mean saturation increase of 75-89% (P = 0.01) (mean increase of 20%), a mean PAB gradient decrease from 69 to 36 mmHg (P = 0.002) (mean decrease of 49%), and a mean band site diameter increase from 4.1 to 6.1 mm (P = 0.01) (mean increase of 45%). The only complication was transient pulmonary edema in one patient. The PABBD procedure is a feasible and safe method for increasing pulmonary blood flow in a staged manner and may eliminate the need for surgical band removal in some cases.

Deletion of JAM-C, a candidate gene for heart defects in Jacobsen syndrome, results in a normal cardiac phenotype in mice.

The 11q terminal deletion disorder (11q-) is a rare chromosomal disorder caused by a deletion in distal 11q. Fifty-six percent of patients have clinically significant congenital heart defects. A cardiac "critical region" has been identified in distal 11q that contains over 40 annotated genes. In this study, we identify the distal breakpoint of a patient with a paracentric inversion in distal 11q who had hypoplastic left heart and congenital thrombocytopenia. The distal breakpoint mapped to JAM-3, a gene previously identified as a candidate gene for causing HLHS in 11q-. To determine the role of JAM-3 in cardiac development, we performed a comprehensive cardiac phenotypic assessment in which the mouse homolog for JAM-3, JAM-C, has been deleted. These mice have normal cardiac structure and function, indicating that haplo-insufficiency of JAM-3 is unlikely to cause the congenital heart defects that occur in 11q- patients. Notably, we identified a previously undescribed phenotype, jitteriness, in most of the sick or dying adult JAM-C knockout mice. These data provide further insights into the identification of the putative disease-causing cardiac gene(s) in distal 11q, as well as the functions of JAM-C in normal organ development.

Adverse impact of vascular stent "mass effect" on airways.

OBJECTIVES: To report our experience with bronchial compression secondary to vascular stent implantation within the mediastinum of patients with congenital heart disease. BACKGROUND: Stents are routinely used to correct native and postsurgical vessel stenoses. However, the "mass effect" of stents on adjacent structures within the chest has not been consistently appreciated nor evaluated. METHODS: A retrospective review of patients who had stent implantation and subsequent computerized tomographic angiography (CTA) at Rady Children's Hospital was performed. RESULTS: From August 2005 to November 2007, 147 stents were implanted to correct vessel stenoses within the chests of 120 patients. Among these 120 patients, 21 had CT angiograms performed after stent implantation. Clinical indications for poststent CT imaging included inability to wean from ventilator support, significant coughing episodes, continued hypoxia, and follow-up study after complex repairs. Six of the twenty one patients (29%) had adverse mass effects from stents causing compression of adjacent bronchial structures. Two of the six patients expired. One patient required a tracheostomy, two patients were slow to wean from the ventilator, while one was clinically asymptomatic. Five of the six patients had had an aortic arch anomaly with subsequent arch reconstruction or hybrid intervention. CONCLUSIONS: Stent implantation in vascular structures within the chest may have adverse mass effects on adjacent bronchial structures. Clinical sequelae may range from silent to catastrophic. Pre-and/or poststent implantation imaging of vascular structures and airways should be considered in selected patients.

Left main coronary artery compression from right pulmonary artery stenting.

Complications related to pulmonary artery stenting include stent migration, jailing of vessels, vessel rupture, and compression of surrounding structures. Compression of the left main coronary artery (LMCA) as a result of stent placement in the right pulmonary artery (RPA) is extremely rare. We present two patients post repair of congenital heart disease who suffered LMCA compression following RPA stenting. The first patient experienced acute coronary insufficiency in the cardiac catheterization laboratory, whereas the second patient had a more chronic course. We also present a third patient who had a CT angiogram that demonstrated a close spatial relationship between the RPA and the LMCA. Based on our previous experiences, we felt that this patient was at significant risk for LMCA compression if the RPA were stented. Coronary compression is rare complication of pulmonary artery stenting but should be considered in cases with history of repaired congenital heart disease.

Simultaneous stent implantation to treat bifurcation stenoses in the pulmonary arteries: Initial results and long-term follow up.

BACKGROUND: Balloon angioplasty of bifurcating pulmonary artery (PA) stenoses is often inadequate, and stent treatment often requires simultaneous implantation of two stents. This study evaluates initial results and long-term follow up of transcatheter stent placement in bifurcating PAs. METHODS: This is a retrospective review of patients (pts) who had bifurcating PA stents placed in main and lobar branches from 1993 to 2007. RESULTS: Forty-nine pts had bifurcating PA stents placed at a median age of 10.9 years (range 1-43 years). The mean minimum vessel diameter increased from 5.7 +/- 2.5 mm to 11.0 +/- 3.6 mm (P < 0.001), the mean gradient across the stenoses decreased from 37.0 +/- 26.9 to 9.2 +/- 13 mm Hg (P < 0.001), whereas the mean RV:FA ratio decreased from 0.76 +/- 0.29 to 0.53 +/- 0.24 (P < 0.001). There was one death due to severe pulmonary hemorrhage. F/U data were available in 38 pts (mean duration 6.3 +/- 4.1 years, range 1.2-13.1 years). Thirty pts underwent repeat catheterizations (mean 2.3 +/- 2.2 years poststent), with 26 requiring further interventions: Fifteen had balloon angioplasty alone and 11 had additional stents placed. There were no complications at f/u catheterization. Six pts underwent further palliative surgeries, although none for repair of branch PA stenoses. CONCLUSIONS: Simultaneous transcatheter placement of bifurcating PA stents provides immediate gradient relief of bifurcating stenoses in the proximal or lobar branch PAs and reduces RV systolic pressure. Further interventions can be safely performed in future procedures, and the presence of stents does not complicate future surgeries.

Myocardial Performance Index in Childhood Onset Essential Hypertension and White Coat Hypertension.

BACKGROUND: As a global measure of ventricular systolic and diastolic function, the myocardial performance index (MPI) can be an early indicator of hypertensive cardiomyopathy in children with essential hypertension (EH). METHODS: Children with untreated newly diagnosed EH and white coat hypertension (WCH) by a 24-hour ambulatory blood pressure monitoring (ABPM), both groups without any identifiable etiology for the hypertension, were enrolled for the study. Echocardiograms and vascular ultrasounds for carotid artery intimal medial thickness were performed on all children prior to therapy. Diastolic function (peak E and A velocities, E/A ratio, isovolumic relaxation time, and deceleration times) and MPI were evaluated by simultaneous transmitral and transaortic spectral Doppler flow velocities. Systolic function was evaluated by shortening fraction and ejection fraction. RESULTS: A cohort of 66 children (24 with EH, 42 with WCH, males 61%, median age of 13 years, range 10-17 years) were enrolled in the study. The demographic, anthropometric, laboratory tests, vascular ultrasound, and conventional echocardiographic parameters were similar between the 2 groups. There was a very small difference in MPI between the EH and WCH children (0.28 SD: 0.07 vs. 0.31 SD: 0.08, P = 0.045). However, in EH children, MPI increased by 0.14 units for every 10 unit increase in mean ABPM systolic BP (95% confidence interval: 0.03-0.25). CONCLUSIONS: We found the increasing MPI was associated with increasing 24-hour mean systolic BP in children with EH. Therefore, MPI may have utility as a single, quick, noninvasive method of detection and tracking of subclinical hypertensive heart disease.

Modified Senning Procedure for Correction of Atrioventricular Discordance With Total Anomalous Pulmonary Venous Return, Atrial Situs Inversus, Dextrocardia, and Bilateral Superior Venae Cavae.

The Senning and Mustard baffles remain important techniques for the treatment of congenitally corrected transposition (cc-TGA), isolated ventricular inversion, and D-transposition of the great arteries with delayed presentation. We describe the treatment of an 8-month old infant with atrioventricular discordance, ventriculoarterial concordance, and dextrocardia with atrial situs inversus. A modified Senning procedure was performed through the "left-sided" atrium. Modifications of the Senning and Mustard baffles remain important tools in the treatment of rare conditions like isolated ventricular inversion.

"Wire-target" technique for precise vascular access.

Herein, we describe a technique that facilitates percutaneous vascular access when the traditional method of achieving access is unsuccessful. For multiple reasons, gaining access to small vessels in pediatric patients is sometimes difficult. In instances of atrial, ventricular, or great arterial communications, a wire can be positioned from a vein or artery across the communications into an artery or vein to which access needs to be gained. This wire then serves as a target for vascular access. All pediatric patients who underwent cardiac catheterization at Mattel Children's Hospital from July 2003 through June 2006, and at Rady Children's Hospital from July through December 2006, were considered for the wire-target technique when access could not be achieved in vessels of interest via traditional methods. Fifteen wire-target procedures were undertaken in 14 patients (ages, 4 d-11 yr). By use of a directional catheter, a Wholey or 0.014-inch coronary wire was positioned in a vessel to which access was desired. Anterior-posterior and lateral fluoroscopic views were used to target the wire and attain vascular access. The patients' diagnoses, ages, vessels to which access was gained via traditional methods and via the wire-target technique, and wire routes were retrospectively recorded, and outcomes were noted. In all instances, the technique was performed successfully and without complications. In selected pediatric patients in whom percutaneous vascular access is difficult, the wire-target technique may be used safely and effectively to establish arterial, venous, or transhepatic access.