Cardiac Fibroma with Asymptomatic Ventricular Arrhythmia in an Adolescent with Gorlin's Syndrome.

Nevoid basal cell carcinoma syndrome (NBCCS), also referred to as Gorlin's syndrome, is an autosomal dominant inherited condition that predisposes affected individuals to various tumors such as cardiac fibromas. Though technically benign, cardiac fibromas may result in malignant arrhythmias and sudden death. The pertinent literature pertaining to pediatric cases of cardiac fibromas and their clinical features were reviewed. We present the case of an asymptomatic teenage with de novo NBCCS who was diagnosed with both NBCCS and cardiac fibroma later in life. The patient was noted to have clinically significant ventricular arrhythmias that were eliminated with tumor resection. There are no established best practice guidelines for the management of cardiac fibromas in patients with NBCCS. Given the risk of sudden arrhythmic death, the presence of ventricular arrhythmias should prompt strong consideration of tumor resection.

Assessing Non-invasive Studies to Evaluate Resynchronization Pacing Effectiveness in the Young.

Appropriate non-invasive assessments (ECHO/ECG) of cardiac resynchronization pacing therapy (CRT) among younger patients (pts) with/without (w/wo) congenital heart disease (CHD) are not established. Ejection fraction (EF) and QRS can be unreliable due to anatomy, surgical repairs, and pre-existing pacemakers (PM). This study correlates updated non-invasive studies, including newer strain values, with clinical and invasive hemodynamic assessments of CRT response in the young. Sixteen pts (mean age 18.5 ± 6 years, 10/16 with pre-existing pacemakers) underwent CRT for heart failure (NYHA II-III). CHD included septal defects and Tetralogy of Fallot. Assessment of CRT efficacy was based on clinical findings, direct catheterization studies [pressures, contractility indices (dP/dt-max)], ECG changes, and ECHO studies [including updated global (GLS), left atrial strain (LAS), and sphericity indices] pre- and at 1-month and 1-year post-CRT. After 1 year following CRT, all pts improved (II-III to I-II) in clinical NYHA status. Contractility (dP/dt) increased (932 ± 351 vs 561 ± 178.7 mmHg-sec [p = 0.001]). QRS duration shortened only among pts with pre-existing PM (160 ± 25 vs 134 ± 25 ms [p = 0.02]). Standard ECHO parameters, including chamber dimensions and EF, showed no appreciable changes from pre-CRT values. However, endocardial GLS [(- 6.4 vs. - 9.6%) p = 0.0003] and LAS [(- 5.8 vs - 9.3%) p = 0.02] values significantly improved. Although CRT is applicable to younger pts, accurate non-invasive evaluations of response are lacking. This study establishes that newer strain values better correlate with clinical and hemodynamic changes over other parameters and offer more appropriate assessments of CRT response.

Anthracycline Treatment and Left Atrial Function in Children: A Real-Time 3-Dimensional Echocardiographic Study.

Anthracycline (AC) therapy is associated with left ventricular (LV) dysfunction. Left atrial (LA) size and function are used to assess LV diastolic function in heart failure in adults. Data on LA size and function following AC therapy in children is limited. We hypothesized that LA size and function will be abnormal in children following AC chemotherapy. This retrospective review included patients who received AC for pediatric cancers. Controls had normal echocardiograms performed for evaluation of chest pain, murmur, or syncope. Real-time three-dimensional echocardiography was performed to evaluate LA reservoir, conduit, and booster pump function parameters. In addition to LA volume data, LV shortening fraction, spectral and tissue Doppler variables assessing diastolic function as well as myocardial performance index was obtained. Groups with and without AC therapy were compared by student t-test and chi-square test. We evaluated 136 patients, 55 (40.4%) had received AC. There was no significant difference between the groups in LV shortening fraction, diastolic as well as global function indices. LA reservoir and conduit function parameters were significantly lower in AC group compared to controls. The booster function parameters showed variable results. It is intriguing that AC-treated children have smaller LA reservoir and abnormal booster function. We speculate that these findings may reflect early changes in LA compliance associated with AC exposure. Assessment of LA volumes and function as prognostic markers of AC-induced cardiotoxicity in children is warranted.

Use of 3D Electroanatomical Navigation (CARTO-3) to Minimize or Eliminate Fluoroscopy Use in the Ablation of Pediatric Supraventricular Tachyarrhythmias.

BACKGROUND: Radiation exposure related to medical procedures carries known medical risk. Electrophysiology (EP) and catheter ablation procedures are traditionally performed under fluoroscopic guidance. Three-dimensional (3D) electroanatomical navigation systems decrease or eliminate fluoroscopy use in EP procedures. OBJECTIVE: The aim of this study was to assess the efficacy and outcome of a minimal or no fluoroscopic electroanatomical mapping approach for catheter ablations for supraventricular tachycardia (SVT) in the pediatric population. METHODS: Patients were identified through our EP database. A retrospective chart review was performed at a single institution. RESULTS: Sixty-three pediatric patients underwent catheter ablations with a minimal fluoroscopic and 3D electroanatomical mapping using CARTO-3 system (Biosense Webster, Diamond Bar, CA, USA) between October 2012 and March 2015. We selected 20 age-matched patients who underwent ablations for SVT by the same operator prior to October 2012 with fluoroscopy use as our control group. The mean age in the study and control group was 13.9 years and 13.7 years, respectively. Mean procedure time was 208.7 minutes and 217.2 minutes in the study and control group (P = NS). Thirty-four (54%) in the study group had no fluoroscopy use. Mean fluoroscopy time was 4.1 minutes versus 35.4 minutes between the study and the control group (P < 0.001). Radiation dose was 6.7 mGy versus 209.3 mGy between the study and the control group (P < 0.001). Acute procedural success was achieved in 95% and 90% of patients in the study and control groups, respectively. On follow-up, the recurrence rate was 5.3% in the study group and 5.6% in the control group. CONCLUSION: Catheter ablation for SVT in children can be successfully performed in patients with normal cardiac anatomy using minimal or no fluoroscopy with favorable outcomes.