Double Ventricular Response with Aberrant Conduction Leading to Ventricular Dysfunction.

Double ventricular response (DVR), where a single P wave results in two QRS complexes, is a rare presentation of dual AV node physiology. It has been associated with ventricular dysfunction in the setting of incessant tachycardia. We present the case of an otherwise healthy adolescent who had frequent DVR without tachycardia leading to left ventricular dysfunction. Slow pathway modification led to a significant reduction in ectopy and normalization of ventricular function. This highlights that DVR without tachycardia might lead to ventricular dysfunction in pediatric patients. Slow pathway modification with reduction of ectopy may be sufficient to restore ventricular function.

Standardized Training for Physicians Practicing Pediatric Cardiac Critical Care.

OBJECTIVES: In the vast majority of Children's Hospitals, the critically ill patient can be found in one of three locations: the PICU, the neonatal ICU, and the cardiac ICU. Training, certification, and maintenance of certification for neonatology and critical care medicine are over seen by the Accreditation Council for Graduate Medical Education and American Board of Pediatrics. There is no standardization of training or oversight of certification and maintenance of certification for pediatric cardiac critical care. DATA SOURCES: The curricula from the twenty 4th year pediatric cardiac critical care training programs were collated, along with the learning objectives from the Pediatric Cardiac Intensive Care Society published "Curriculum for Pediatric Cardiac Critical Care Medicine." STUDY SELECTION: This initiative is endorsed by the Pediatric Cardiac Intensive Care Society as a first step toward Accreditation Council for Graduate Medical Education oversight of training and American Board of Pediatrics oversight of maintenance of certification. DATA EXTRACTION: A taskforce was established of cardiac intensivists, including the directors of all 4th year pediatric cardiac critical care training programs. DATA SYNTHESIS: Using modified Delphi methodology, learning objectives, rotational requirements, and institutional requirements for providing training were developed. CONCLUSIONS: In the current era of increasing specialized care in pediatric cardiac critical care, standardized training for pediatric cardiac critical care is paramount to optimizing outcomes.

Anaphylactic shock after amiodarone infusion resulting in haemodynamic collapse requiring a temporary ventricular assist device.

Acute heart failure related to anaphylactic shock is often reversible and necessitates aggressive support to ensure full recovery. We report the case of a 15-year-old boy who developed severe ventricular dysfunction and haemodynamic instability after administration of amiodarone and required temporary mechanical circulatory support with a left ventricular assist device. He had full recovery of cardiac function and returned to baseline neurologic status. This is the first report of successful left ventricular assist device use for recovery from cardiovascular collapse due to anaphylaxis.

Nodoventricular pathway associated with twin AV nodes: complexity of ablation in single ventricle physiology.

We report the case of a patient with heterotaxy syndrome including complex single ventricular morphology and interrupted IVC in association with twin conduction systems and a nodoventricular accessory pathway connection. The presence of 3 distinct QRS morphologies was inadvertently discovered during a hemodynamic catheterization study and prompted formal EP testing prior to hepatic venous inclusion into the Fontan circuit and loss of access to the atrial chamber for testing and therapy. This patient underscores the importance of close surveillance and high index of suspicion of arrhythmia mechanisms in patients with heterotaxy syndrome in conjunction with single ventricle morphology.

Linear lesion cryoablation for the treatment of atrioventricular nodal re-entry tachycardia in pediatrics and young adults.

BACKGROUND: Radiofrequency (RF) ablation is a relatively safe and effective method for treatment of atrioventricular nodal re-entry tachycardia (AVNRT), but carries a 1-2% risk of AV nodal injury. Cryothermal ablation reduces the risk of AV block, but has had decreased procedural success and increased recurrence of tachycardia. We sought to evaluate the technique of linear lesion cryoablation (LLC) for treatment of AVNRT. METHODS: Single institution retrospective cohort study. Each patient underwent slow pathway modification using either RF, single lesion cryoablation, or LLC. Procedural success, recurrence, freedom from tachycardia 12 months following ablation and fluoroscopy time were compared between ablation methods. RESULTS: A total of 125 patients, median age 15.5 (4.7-23.1) years, underwent ablation: 32 RF energy, 31 single lesion cryoablation, 62 LLC. Procedural success was obtained in 94% of the LLC group compared to 58% using single lesion cryoablation (P ≤ 0.001). Ninety-seven percent of the LLC group was free from tachycardia recurrence, significantly higher than with single lesion cryoablation (68%, P = 0.001) and equal to that of RF (97%, P = NS). Fluoroscopy time was reduced in the LLC group compared to both single lesion and RF groups (P = 0.02). There was no permanent AV nodal injury in the cryoablation groups. CONCLUSION: LLC is an effective means of treatment for AVNRT and is associated with significantly improved procedural success and freedom from recurrence compared to single lesion methods, while at the same time obtaining equivalent efficacy to RF.

Atrioventricular accessory pathways in 89 dogs: Clinical features and outcome after radiofrequency catheter ablation.

BACKGROUND: Atrioventricular accessory pathways (APs) in dogs have been reported rarely. Data regarding clinical presentation and long-term outcome after radiofrequency catheter ablation (RFCA) are limited. HYPOTHESIS/OBJECTIVES: To study clinical features, electrophysiologic characteristics, and outcome of RFCA in dogs with APs. ANIMALS: Eighty-nine dogs presented consecutively for RFCA of APs. METHODS: Case series. RESULTS: Labrador retrievers (47.2% of dogs) and male dogs (67.4% of dogs) were most commonly affected. Labrador retrievers were more likely to be male than non-Labrador breeds (P = .043). Clinical signs were nonspecific and most commonly included lethargy and gastrointestinal signs. Concealed APs were more prevalent in Labrador retrievers than other breeds (P = .001). Right-sided APs (91.7%) predominated over left-sided (8.3%). Tachycardia-induced cardiomyopathy (TICM) occurred in 46.1% of dogs, with complete resolution or substantial improvement noted on one-month postablation echocardiograms. Radiofrequency catheter ablation successfully eliminated AP conduction long term in 98.8% of dogs in which it was performed. Complications occurred in 5/89 dogs. Recurrence in 3 dogs was eliminated long term with a second procedure. CLINICAL IMPORTANCE/CONCLUSIONS: Accessory pathways are challenging to recognize in dogs because of nonspecific clinical signs, frequency of concealed APs that show no evidence of their presence during sinus rhythm, and intermittent occurrence of tachyarrhythmias resulting from APs. Tachycardia-induced cardiomyopathy commonly occurs with AP-mediated tachycardias and should be considered in any dog presenting with a dilated cardiomyopathic phenotype because of its good long-term prognosis with rhythm control. Radiofrequency catheter ablation is a highly effective method for eliminating AP conduction and providing long-term resolution.

Utility and safety of the SafeSept™ transseptal guidewire for electrophysiology studies with catheter ablation in pediatric and congenital heart disease.

PURPOSE: The atrial transseptal procedure is used in catheter ablation of left-sided arrhythmias. Studies in adult patients have shown the SafeSept™ transseptal guidewire (SSTG) to be effective in atrial transseptal procedures. We analyzed our 5-year experience with SSTG use in pediatric and congenital heart disease patients undergoing catheter ablation. METHODS: This is a single-center retrospective analysis of patients undergoing catheter ablation from 2009 to 2014. We identified all procedures where SSTG was used for atrial transseptal or trans-baffle access. Success of transseptal access and complications were recorded and compared to the standard transseptal approach without the SSTG. RESULTS: One hundred twenty-seven patients underwent 132 attempted atrial transseptal or trans-baffle procedures using SSTG. Median age was 14 (1.2-38) years. Arrhythmia substrates included AV reentrant tachycardia (90.2%), atrial tachycardia (4.5%), ventricular tachycardia (2.3%), and AV nodal reentrant tachycardia (2.3%). Transseptal or trans-baffle access was successful in 96.2% of the SSTG cases compared to 98.9% in the standard transseptal group without SSTG (p = NS). The youngest patient with successful atrial transseptal procedure using SSTG was 4 years old. SSTG was used to successfully cross a surgically created atrial baffle in a patient who had undergone the Mustard procedure. There was one major complication in both groups, 0.8% in the SSTG group compared to the standard transseptal group without SSTG, 1.1% (p = NS). The major complication in the SSTG group occurred when the SSTG crossed the aorta into the coronary artery system and mimicked placement in the left atrial appendage, with subsequent placement of a transseptal sheath into the aorta, requiring sternotomy and surgical intervention. CONCLUSIONS: SSTG is effective for use in atrial transseptal and surgical trans-baffle access in pediatric and congenital heart disease patients. Placement of the SSTG into the pulmonary vein is necessary to avoid major complications, and if not achieved requires additional methods to determine appropriate left atrial placement.

Transforming growth factor beta mimetics: discovery of 7-[4-(4-cyanophenyl)phenoxy]-heptanohydroxamic acid, a biaryl hydroxamate inhibitor of histone deacetylase.

Transforming growth factor beta (TGF-beta) is a multifunctional protein that has been shown to possess potent growth-inhibitory activity. To identify small molecular weight compounds with TGF-beta-like activities, high throughput screening was performed using mink lung epithelial cells stably transfected with a TGF-beta-responsive plasminogen activator inhibitor 1 promoter/luciferase construct. Biaryl hydroxamate compounds were identified that demonstrated TGF-beta-like activities. 7-[4-(4-cyanophenyl)phenoxy]-heptanohydroxamic acid (A-161906) demonstrated complete TGF-beta-like agonist activity in the plasminogen activator inhibitor 1/luciferase construct. A-161906 inhibited the proliferation of multiple cell lines in a concentration-dependent manner. Cells were growth arrested at the G1-S checkpoint similar to TGF-beta. Consistent with the G1-S arrest, A-161906 induced the expression of the cyclin-dependent kinase inhibitor p21waf1/cip1. A-161906 produced many cellular effects similar to that of TGF-beta but did not displace labeled TGF-beta from its receptors. Cells with mutations in either of the TGF-beta receptors I or II were growth-arrested by A-161906. Therefore, the site of action of A-161906 appears to be distal to the receptors and possibly involved with the signaling events controlled by TGF-beta. The TGF-beta mimetic effect of A-161906 can be partially, if not entirely, explained by its activity as a histone deacetylase (HDAC) inhibitor. A-161906 demonstrated potent HDAC-inhibitory activity (IC50 = 9 nM). A-161906 is a novel small molecular weight compound (< 400 MW) having TGF-beta mimetic activity as a result of its potent HDAC-inhibitory activity. These results and those of others demonstrate the importance of HDACs in regulation of the TGF-beta signaling pathway(s).

Relation of systemic venous return, pulmonary vascular resistance, and diastolic dysfunction to exercise capacity in patients with single ventricle receiving fontan palliation.

Fontan patients have a reduced exercise capacity, primarily owing to limitations in the ability to augment pulmonary blood flow and stroke volume. To date, the mechanism of peak exercise pulmonary blood flow restriction has not been elucidated. We performed a single-center, prospective, crossover trial of supine and upright exercise in Fontan patients and healthy controls to determine the mechanisms of exercise limitation in the Fontan-palliated patient. A total of 29 Fontan patients and 16 control subjects completed the protocol. The duration of exercise, percentage of predicted peak oxygen consumption (VO(2)) and peak work were reduced in the Fontan group, regardless of posture (p < or = 0.03). The percentage of predicted oxygen pulse, a surrogate for pulmonary stroke volume, was not increased with supine posture in the Fontan cohort (upright, 82.3 + or - 18.8% vs supine, 82.4 + or - 19.7%; p = 0.6). In both groups, the percentage of predicted peak VO(2) was lower with supine exercise than with upright exercise (p < or =0.002). Diastolic dysfunction was present in 57% of the Fontan patients and was associated with a reduced percentage of predicted peak VO(2) (p = 0.04) and supine peak work (p = 0.008). Six Fontan patients who underwent supine exercise with indwelling catheters failed to demonstrate the expected decrease in pulmonary vascular resistance characteristically seen with peak exercise (at rest, 2.8 + or - 0.7 mm Hg/L/min/m(2) vs at peak, 2.8 + or - 0.9 mm Hg/L/min/m(2); p = 0.9). In conclusion, supine exercise in Fontan patients does not result in an increased VO(2) or oxygen pulse, suggesting that inadequate venous return might not be the primary limitation of exercise capacity in this population. Diastolic dysfunction and relatively excessive peak exercise pulmonary vascular resistance might be more important factors in Fontan exercise limitation.