Combination percutaneous and medical management of cardiac rhabdomyomas obstructing tricuspid valve inflow: Case report.

BACKGROUND: Cardiac rhabdomyomas can be prenatally diagnosed in patients with tuberous sclerosis complex. Many neonates require no intervention in early life other than close monitoring for regression of tumor over the period of months to years. In rare instances, cardiac rhabdomyomas can result in obstruction to blood flow or decreased ventricular function. CASE REPORT: We describe the case of a neonate who was prenatally diagnosed with multiple large cardiac rhabdomyomas, one of which caused clinically significant obstruction to prograde blood flow across the tricuspid valve in the newborn period. To address the disturbance to prograde pulmonary blood flow, the patient underwent successful ductal stent placement in the neonatal period. A troponin elevation was noted shortly after birth, but no evidence of coronary compression or involvement was demonstrated by coronary angiography. The patient has subsequently been treated with sirolimus over a period of three months, with noted regression in tumors and improvement in tricuspid valve inflow. CONCLUSIONS: A brief review of the literature regarding the diagnosis, treatment, and management of neonatal patients with cardiac rhabdomyomas is presented. A combined percutaneous and medical management approach may be of benefit in future cases of rhabdomyomas causing obstruction to pulmonary blood flow.

Long-term follow-up of the STARFlex device for closure of secundum atrial septal defect.

BACKGROUND: There is limited published outcome data on the STARFlex device for transcatheter closure of atrial septal defects (ASD). AIM: To contribute to the long term outcomes of ASD device closure with the STARFlex device. METHODS: Results of a prospective FDA approved clinical trial (1999-2001) from a single institution of the STARFlex device for simple ASD was reviewed. The inclusion criteria included age >or=2 years, isolated secundum ASD, evidence of right ventricular volume overload, and maximum stretched diameter <22 mm. A retrospective review of the original study data with most recent follow-up was performed. Clinical success in the trial was defined as complete closure or residual leak

Low prevalence and assay discordance of "aspirin resistance" in children.

BACKGROUND: Although "aspirin resistance" (AR-inadequate platelet inhibition as suggested by in vitro testing of aspirin-treated patients) has been widely studied in adults and linked to increased risk of adverse outcomes, its prevalence and clinical significance are largely unknown in children. PROCEDURE: To determine AR prevalence in children and its relationship to assay methodology, we undertook a cross-sectional study of 44 children (1-17 years, 24 male) on aspirin for various indications and considered three published definitions of AR in adults: platelet aggregation >/=70% to 10 microM adenosine diphosphate and >/=20% to 0.5 mg/ml arachidonic acid (AA), normal PFA-100(R) closure time and elevated urinary 11-dehydro thromboxane B(2) (11dhTxB(2)) concentration. RESULTS: Six subjects exhibited AR according to at least one of the criteria (5 by PFA-100(R), 1 by aggregometry and 11dhTxB(2) criteria); nearly all subjects had low levels of 11dhTxB(2) compared with controls. Subjects studied prior to therapy showed pronounced changes in AR parameters after aspirin dosing (e.g., mean aggregation to AA decreased from 82% to 6%, P < 0.001), confirming an aspirin effect. Subjects with AR did not differ from aspirin responsive subjects in terms of age, race, platelet count, or aspirin dose, indication or therapy duration. There was minimal correlation between assays. CONCLUSIONS: In this initial prevalence study of a clinically diverse group of pediatric patients, frequencies of AR were assay-dependent; however, the prevalence of true AR is likely low in children (2.3%; 95% CI 0.1-10.7%), in agreement with adult studies. To better define the clinical relevance of AR in children, multicenter, prospective cohort studies are imperative.

Comparison of two transcatheter closure methods of persistently patent arterial duct.

A randomized trial of arterial duct occlusion with a double umbrella (DU) or wire coil (WC) was undertaken for patients <18 years of age, weighing >10 kg with isolated ducts < or = 3 mm in diameter. Baseline, procedural, and outcome characteristics were compared in an intention-to-treat analysis according to randomization group. From 40 consecutively screened patients, 2 were not enrolled due to a ductal diameter of >3 mm on initial aortography, 38 patients were randomized to either the DU (n = 20) or WC (n = 18) groups. The groups did not differ significantly with respect to age, weight, gender, duct size, type, or branch pulmonary artery diameters. Crossover occurred only in the DU group, where 4 patients (20%) had a ductal diameter of < or = 1 mm and could not be entered for umbrella placement. All remaining DU group patients had ductal diameters of > or = 1.3 mm (p <0.0001). There were no embolizations or secondary implants in the DU group, but in the WC group there was 1 early and 1 late embolization, with 6 patients (33%) with > or = 2 coils. Mean times for the procedure (DU 68+/-19 minutes; WC 65+/-27 minutes; p = 0.70) and fluoroscopy (DU 14+/-4 minutes; WC 11+/-6 minutes; p = 0.22) did not differ significantly. Angiographic duct closure was documented in 4 of 13 patients (31%) of the DU group and 4 of 18 patients (22%) of the WC group (p = 0.69). Combined with an echocardiogram, closure in 11 of 17 patients with DU (65%) and 13 of 18 patients with WC (72%) (p = 0.64) was documented before hospital discharge. One WC group patient received thrombolytic therapy for a femoral artery thrombus. Follow-up at a median of 6.5 months (range 3.2 to 37) showed closure by Doppler echocardiography in 15 of 19 patients with DU (79%) versus 14 of 18 patients with WC (78%) (p = 1.0). Thus, with a tendency toward similar procedural characteristics and outcomes, the higher cost of the DU system compared with coil implants favors the use of coils for closure of the small arterial duct.

Use of covered stents in the management of coarctation of the aorta.

We report our experience with the use of covered stents for the management of coarctation of the aorta. From December 2001 to March 2004, nine patients (seven males; median age, 31 years; mean weight, 65 +/- 15 kg) underwent implantation. Indications included critical or atretic native coarctation (n = 4), patients >50 years of age (n = 2), associated patent ductus arteriosus (n = 1) or adjacent aneurysm (n = 1), and the presence of a circumferential fracture within a previously implanted stent (n = 1). The covered balloon-expandable Cheatham-Platinum stent and the self-expandable stent graft Braile were employed. Adequate implantation was observed in all patients. Gradients were reduced from 54 +/- 14 to 3 +/- 8 mmHg and the coarctation site increased from 2.4 +/- 2.9 to 15.9 +/- 4.3 mm. The patent ductus arteriosus was immediately closed and the aneurysm excluded. Two patients >35 years with aneurysmal ascending aorta and metallic aortic prosthesis had aneurysm formation at follow-up, with one undergoing aneurysm exclusion using a Braile stent. Although covered stents are useful in the management of selected patients with coarctation, aneurysm formation may still occur in patients with markers of aortic wall weakness. Refinements in the deployment technique and/or the stent design are needed to eliminate this risk.

Transcatheter creation of an atrial septal defect using radiofrequency perforation.

Transseptal perforation using radiofrequency energy was performed successfully in a patient with congenital heart disease and a thickened interatrial septum. This was followed by balloon dilatation of the atrial septal defect. Radiofrequency is presented as a alternative to standard transseptal needle puncture. Cathet Cardiovasc Intervent 2001;54:83-87.

Noninvasive imaging in congenital heart disease.

Imaging algorithms in congenital heart disease, as in the patient with acquired heart diseases continue to evolve, with more and more information gleaned noninvasively. The emphasis will be on the newer aspects of imaging, not cross sectional echocardiography with color Doppler.