Use of Palmaz stents in a newborn with congenital aneurysms and coarctation of the abdominal aorta.

Anomalies of the abdominal aorta are rare in the pediatric population limiting the reported knowledge base from which management decisions can be made. A 3-week-old male with congenital abdominal aortic coarctation and multiple aneurysms presented with malignant hypertension. We report the safe deployment of overlapping Palmaz stents using a 4-French catheter delivery system with significant relief of the coarctation gradient and restoration of adequate renal perfusion.

Calpain expression and infiltration of activated T cells in experimental allergic encephalomyelitis over time: increased calpain activity begins with onset of disease.

Calpain activity and expression at the protein level were examined in inflammatory cells, activated microglia, and astrocytes prior to or at onset of symptomatic experimental allergic encephalomyelitis (EAE), an animal model for the human demyelinating disease multiple sclerosis (MS). EAE was induced in Lewis rats by injection of guinea pig spinal cord homogenate and myelin basic protein (MBP) emulsified with Complete Freund's Adjuvant (CFA). Calpain translational expression, determined by Western blot and immunocytochemistry, was correlated with calpain activity, infiltration of inflammatory cells, and myelin loss at 2-11 days following challenge with antigen. Controls (CFA only) did not show any changes over time in these parameters and very few changes (CD11+ microglia/mononuclear phagocytes) were seen in either group from days 2 to 8 post-induction. In contrast, from days 9 to 11, the animals that developed the disease (at least grade 1) demonstrated extensive cellular infiltration (CD4+, CD25+, and CD11+ as well as increased calpain expression (content) and activity. This study demonstrates that cell infiltration and increased calpain activity do not begin in the CNS until the onset of clinical signs.

Insulin resistance syndrome in childhood: associations of the euglycemic insulin clamp and fasting insulin with fatness and other risk factors.

OBJECTIVE: Our objective was to describe in children the relation of fatness and insulin resistance to the risk factors associated with the insulin resistance syndrome and to compare fasting insulin with the euglycemic insulin clamp as a measure of insulin resistance in children. STUDY DESIGN: This was a random selection of participants after blood pressure screening of 12,043 students in the fifth through eighth grades. Euglycemic insulin clamp studies with an insulin infusion rate of 1 mU/kg/min and a variable infusion of 20% glucose to maintain euglycemia, that is, plasma glucose at 5.6 mmol/L. Insulin sensitivity (M(lbm)) is defined as the amount of glucose required to maintain euglycemia (milligrams of glucose infused per kilogram lean body mass per minute). RESULTS: Body mass index was significantly correlated with fasting insulin and significantly inversely correlated with M(lbm). Fasting insulin was significantly correlated with systolic blood pressure in both sexes, all lipids, except high-density lipoprotein-cholesterol in males and triglycerides and high-density lipoprotein-cholesterol in females, but after adjustment was done for body mass index, it was significantly related only to triglycerides. M(lbm) was significantly correlated only with triglycerides and high-density lipoprotein-cholesterol, and this did not change after adjustment was done for body mass index. A clustering effect for the risk factors was seen in children in the lowest quartile of M(lbm) (highest degree of insulin resistance) compared with children in the highest quartile of M(lbm) (lowest degree of insulin resistance). CONCLUSIONS: As defined by M(lbm), there is an early association of insulin resistance, independent of body fat, with the risk factors. There is a significant relation between fasting insulin, as an estimate of insulin resistance, and the risk factors, but this is significantly influenced by body fatness. The clustering of risk factors according to level of M(lbm) suggests that adult cardiovascular disease is more likely to develop in children with the greatest degree of insulin resistance.

Access to the left atrium for delivery of radiofrequency ablation in young patients: retrograde aortic vs transseptal approach.

Reported experience with the transseptal approach to the left atrium for delivery of radiofrequency energy in the young patient is limited. To compare two approaches for radiofrequency ablation (RFA) in the left atrium we reviewed our experience from January 1, 1991, through February 1, 1999, in 154 procedures performed on 136 patients (mean age 12.2 years). The patients were grouped by either the retrograde aortic route (R, n = 30) or the transseptal atrial route (T, n = 106). No significant differences were found in age, weight, height, supraventricular tachycardia cycle length, or electrocardiograph characteristics (manifest vs concealed accessory pathway) between the two approaches. Comparison of the transseptal group to the retrograde aortic group revealed a significant difference in the number of catheters (mean = 4 R vs 3 T, p < 0.0001), total fluoroscopic time (71.3 min R vs 43.0 min T, p = 0.0007), diagnostic fluoroscopic time (40.2 min R vs 16.6 min T, p < 0.0001), ablation fluoroscopic time (44.7 min R vs 25.3 min T, p = 0.019), and procedure time (5.0 hours R vs 4.1 hours T, p < 0.0001). No significant difference was found in success rate, number of radiofrequency applications, or major complication rate. These data suggest that although outcomes and major complication rates are similar for the two groups, the use of fewer catheters and shorter fluoroscopic times warrant consideration of the transseptal atrial approach in young patients.

Inducibility of intra-atrial reentrant tachycardia after the first two stages of the Fontan sequence.

OBJECTIVES: We sought to examine the incidence and possible factors for inducible intra-atrial reentrant tachycardia (IART) in a group of patients after two stages of the Fontan sequence but before the operation. BACKGROUND: Intra-atrial reentrant tachycardia occurs in 10% to 40% of patients after the Fontan operation. No data are available regarding the potential for IART after the first two stages of the Fontan sequence but before the operation. METHODS: The IART induction protocol included programmed extrastimulation and rapid atrial pacing, with and without isoproterenol. RESULTS: The median age of the study group (n = 44, 27 males) was 1.7 years (range 1.2 to 5.2). Forty patients were in sinus rhythm. Twelve patients (27%) had inducible, sustained (>1 min) IART. Three patients (8%) had inducible, nonsustained IART. Bivariate analysis revealed that patients with sustained IART were significantly older at their second operation (median 0.54 vs. 0.40 years, p = 0.05). Multivariate logistic modeling revealed that older age (> or =0.55 years) at the second palliative operation (p = 0.04), older age (> or =1.95 years) at evaluation before the Fontan sequence (p = 0.04) and female gender (p = 0.03) were independently associated with sustained IART. A trend toward a greater frequency of sustained IART was seen in those patients with moderate or severe atrioventricular valve regurgitation (p = 0.07) and in those with resection of the atrial septum (p = 0.06). CONCLUSIONS: The rate of inducible, sustained IART in a group of patients before the Fontan operation is 27% and is associated with older age at the time of second-stage palliation, older age at pre-Fontan evaluation and female gender.

Transcatheter closure of fontan fenestrations using the Amplatzer septal occluder: initial experience and follow-up.

We have recently used the Amplatzer septal occluder to close Fontan fenestrations. Between June 1998 and December 1999, 13 patients underwent transcatheter occlusion of their Fontan fenestrations. Systemic blood flow decreased significantly without a concomitant decrease in pulmonary blood flow. All residual shunts detectable by oximetry were at sites separate from those into which occlusion devices were implanted. One patient developed severe tricuspid regurgitation following the procedure requiring surgical removal of the device. At the last follow-up, all patients were doing well clinically. There were no shunts detectable through or around the devices by echocardiography. Our experience indicates that the location of the fenestration within the Fontan baffle is critical to avoiding device interference with other intracardiac structures. The Amplatzer septal occluder offers an effective means of transcatheter closure of Fontan baffle fenestrations. Although more experience is needed, our current follow-up data suggest that long-term outcomes will be favorable. Cathet. Cardiovasc. Intervent. 51:301-304, 2000.

Congenital Heart Surgery Nomenclature and Database Project: therapeutic cardiac catheter interventions.

The extant nomenclature for therapeutic cardiac catheter interventions is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Obesity hypertension, salt sensitivity and insulin resistance.

AIM: To review human and animal data describing the complex interrelation between obesity hypertension, salt-sensitivity and insulin resistance. DATA SYNTHESIS: The first part of the article reviews the information available in the literature defining obesity hypertension as a salt sensitive form of hypertension and that links obesity hypertension to the presence of insulin. The second part reviews the four factors frequently cited as possible causes for this sodium retention: insulin resistance, alteration in the renin-angiotensin system, altered vascular responsiveness and alterations in the sympathetic nervous system. Special emphasis is given in an attempt to explain how each of these four factors can change pressure-natriuresis. CONCLUSIONS: There is clearly a strong relationship between obesity hypertension, sodium sensitivity and insulin resistance. The exact mechanism responsible for linking these three conditions is still unknown. However, there is ample evidence to suggest that insulin resistance, altered vascular responsiveness, altered function of the renin-angiotensin aldosterone system and altered function of the sympathetic nervous system are important in these three conditions. Further investigation is warranted to completely elucidate the complex relationship between insulin resistance, obesity hypertension and sodium sensitivity.

Left ventricular growth in selected hypoplastic left ventricles: outcome after repair of coarctation of aorta.

BACKGROUND: Models that predict survival in neonates with left ventricular hypoplasia and critical aortic stenosis may not be applicable to neonates with left ventricular hypoplasia and coarctation. METHODS AND RESULTS: We report 8 infants with severe aortic coarctation and left ventricular hypoplasia. Mean age was 18 days (range 1-48 days), and mean weight was 3.5 kg (range 2.7-4.3 kg). Associated diagnoses included mild aortic stenosis (4), ventricular septal defect (2), and venous anomalies (2). All had coarctation repair as a primary procedure (3 of these had concomitant intracardiac procedures); 7 had subsequent operations. All are alive and well 1.1-6.7 years (mean 3.1 years) after the first surgery. Progressive increases were observed in aortic and mitral diameters, and in left ventricular dimensions, areas, and volumes when the preoperative, earliest postoperative, and most recent echocardiograms were compared. CONCLUSIONS: Despite severe left ventricular hypoplasia, a two-ventricle repair is possible in selected cases. The prognostic criteria for left ventricular hypoplasia in critical aortic stenosis may not be applicable to infant coarctation. Relief of coarctation may result in the growth of the very small left ventricle, especially when the aortic root and mitral diameters are satisfactory.

Perventricular [correction of Periventricular] closure of ventricular septal defects without cardiopulmonary bypass.

BACKGROUND: Minimally invasive techniques are currently in use to close atrial and ventricular septal defects (VSD). Cardiopulmonary bypass (CPB) is instituted via the femoral vessels, which may cause injury to these vessels, especially in younger patients. The objectives of this study were to demonstrate the feasibility of perventricular [corrected] closure of muscular VSD (MVSD) and paramembranous VSD (PVSD) without CPB, using the Amplatz VSD device. METHODS: Five Yucatan pigs with naturally occurring PVSD (3- to 7-mm diameter) and 5 dogs with surgically created MVSD (6- to 14-mm diameter) were subjects of this study. The VSDs were closed intraoperatively with a 7-French delivery sheath inserted through the free wall of the right (n = 5) or left ventricle (n = 5), under epicardial echocardiogram guidance. The animals were followed for 3 months. RESULTS: There was no operative mortality. All MVSD closed after placement of the device. Closure rate of PVSD was 4 of 5 after placement and 3 of 5 after 3 months. One pig developed aortic incompetence at the last follow-up. CONCLUSIONS: Perventricular closure of MVSD and PVSD is feasible. Avoidance of CPB can decrease recovery time, its complications, and trauma to the femoral vessels.

Snare-assisted vascular access: a new technique.

Access to the central circulation can be difficult in small infants, particularly when normal anatomic landmarks have been altered. We describe a new technique that utilizes any existing central catheter to establish additional sites of vascular access. A 4 Fr end hole catheter is advanced under fluoroscopic guidance to the desired site of new vascular access. A 10-mm Amplatz snare catheter is advanced through the end hole catheter and the loop opened within the target vessel lumen. The snare is then used to guide percutaneous placement of a Cope wire through a 21-gauge needle and then to pull the wire into the central circulation. New access is then achieved by introducing an additional catheter over the guidewire. This technique has now been successfully utilized 16 times in 13 patients. Snare assistance is a safe and effective technique that provides a reliable means of establishing additional secure vascular access. Cathet. Cardiovasc. Intervent. 47:315-318, 1999.

Doppler echocardiography with extended transesophageal atrial pacing: predicting the efficacy of permanent atrial pacing in the patient with a small left ventricle and sinus node dysfunction.

A 2100-g neonate underwent a two-ventricular surgical repair of a right ventricle-dominant unbalanced atrioventricular septal defect associated with the heterotaxy syndrome and sinus node dysfunction. Postoperative congestive heart failure persisted despite bradycardia management by temporary ventricular pacing. Spectral Doppler echocardiographic analysis of pulmonary venous inflow and aortic outflow patterns demonstrated significant improvement with transesophageal atrial pacing. Extended transesophageal pacing was performed for two days, resulting in dramatic clinical improvement. This is the first report of extended transesophageal atrial pacing complementing Doppler echocardiography predicting an improved outcome with permanent atrial pacing.

Impact of arrhythmia circuit cryoablation during Fontan conversion for refractory atrial tachycardia.

Refractory atrial arrhythmias in late postoperative Fontan patients are usually associated with residual hemodynamic abnormalities and result in significant morbidity and mortality. Surgical revision of the Fontan anastomosis may improve hemodynamics without eliminating tachycardia. This study sought to assess the impact of surgical cryoablation of the arrhythmia circuit at the time of Fontan conversion on the clinical recurrence of tachycardia. Sixteen consecutive atriopulmonary Fontan patients with refractory atrial arrhythmias underwent surgical conversion to lateral tunnel total cavopulmonary anastomosis (15) or Fontan revision (1 patient). The initial 4 patients underwent Fontan conversions alone, without specific arrhythmia surgery. The subsequent 12 patients underwent electrophysiologically guided cryoablation of the tachycardia circuits at the time of surgical conversion. The mean age at Fontan revision was 15.6 +/- 3.8 years. Cryoablation was directed to 3 identified major tachycardia circuits: the inferomedial right atrium, the superior rim of the prior atrial septal defect patch, and along the lateral right atrial wall. Transmural antitachycardia pacemakers were implanted in 11 of the 16 patients. There was no surgical mortality in either group, and all patients improved in functional classification. All patients not undergoing cryoablation experienced recurrent symptomatic tachycardia requiring antiarrhythmic therapy (median follow-up, 54 months) versus 2 of 12 patients receiving cryoablation (median follow-up, 25 months; p <0.02). Thus, surgical cryoablation of the arrhythmia circuit at the time of Fontan conversion is highly effective in the management of refractory atrial arrhythmias, and is superior to Fontan conversion alone.

Clonidine prevents insulin resistance and hypertension in obese dogs.

The role that the central sympathetic nervous system plays in the development of obesity hypertension and insulin was evaluated by feeding dogs a high fat diet with or without clonidine treatment. Thirteen adult mongrel dogs were chronically instrumented and randomly assigned to receive either a high fat diet and no clonidine (n=6) or a high fat diet plus clonidine (n=7), 0.3 mg BID. Blood pressure, heart rate, plasma insulin, and electrolytes were measured daily. Insulin resistance was assessed with a multiple-dose euglycemic clamp (1, 2, and 30 mU. kg-1. min-1) before and after 1, 3, and 6 weeks of the high fat diet. Clonidine prevented the hypertension, tachycardia, and insulin resistance associated with feeding dogs the high fat diet but did not affect weight gain. The present study suggests that the central sympathetic nervous system plays a critical role in the development of both insulin resistance and hypertension associated with feeding dogs a high fat diet.

Raising heart-healthy children.

The current article will describe how the cardiovascular risk factors of obesity, increased blood pressure, hyperlipidemia, cigarette smoking, poor physical fitness and prenatal environment impact the development of cardiovascular disease and what types of therapy can be used in the pediatric patient to modify them.

Repair of mitral valve and subaortic mycotic aneurysm in a child with endocarditis.

Endocarditis requiring surgical intervention in children is uncommon. Individualized operative therapy must be fashioned to the particular pathology of each case. In this case we describe mitral anterior leaflet homograft patch augmentation valvuloplasty, subaortic homograft patch closure of a large mycotic aneurysm, and homograft aortic root replacement in a 3-year-old patient with endocarditis after remote repair of complete atrioventricular canal.