Frank Cecchin

Boston Children's Hospital, Boston, Massachusetts, United States

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Publications (71)379.3 Total impact

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    ABSTRACT: Less is known about depression, anxiety and quality of life (QoL) in children and adolescents with pacemakers (PMs) and implantable cardioverter-defibrillators (ICDs) than is known in adults with these devices. A standardized psychiatric interview diagnosed anxiety/depressive disorders in a cross-sectional study. Self-report measures of anxiety, depression and post-traumatic stress disorder were obtained. Medical disease severity, family functioning and QoL data were collected. A total of 166 patients were enrolled (52 ICD, 114 PM; median age 15 years). Prevalence of current and lifetime psychiatric disorders was higher in patients with ICDs than PMs (Current: 27% vs. 11%, P = .02; Lifetime: 52% ICD vs. 34% PM, P = .01). Patients with ICDs had more anxiety than a healthy population (25% vs. 7%, P < .01). Patients with ICDs and PMs had similar levels of depression as a healthy population (ICD 10%, PM 4%, reference 4%, P = .29). In multivariate analysis including a medical disease score, demographics, exposure to beta-blockers, activity limitations, hospitalizations, shocks and procedures, the type of device (PM versus ICD) did not predict psychiatric diagnoses when age at implantation and the severity of medical disease were controlled for. Patients with ICDs and PMs had lower physical QoL scores (ICD 45, PM 47.5, Norm 53, P ≤ .03), but similar psychosocial functioning scores (ICD 49, PM 51, Norm 51, P ≥ .16) versus a normal reference population. Anxiety is highly prevalent in young patients with ICDs, but the higher rates can be attributed to medical disease severity and age at implantation instead of type of device.
    PEDIATRICS 03/2014; · 4.47 Impact Factor
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    ABSTRACT: Background Ebstein’s anomaly is associated with a high incidence of atrial and ventricular arrhythmias. The Cone procedure has become an effective hemodynamic intervention for this malformation. In response to two late postoperative sudden deaths in our early institutional Cone experience, a standardized plan for aggressive rhythm evaluation was instituted, including preoperative electrophysiologic studies (EPS) and intraoperative implantable loop recorder (ILR) placement. Objective The purpose of this study was to measure the yield of this diagnostic protocol for patients with Ebstein’s anomaly undergoing surgical repair and to describe its influence on patient management. Methods All patients at Boston Children’s Hospital with Ebstein’s anomaly who underwent the Cone procedure from December 2006 to September 2012 were reviewed. Pre- and postoperative arrhythmias and therapies were documented. For patients who underwent preoperative EPS, all arrhythmia substrates and interventions were recorded. Results A total of 74 patients were included, 42 of whom underwent preoperative EPS. Significant findings were documented during EPS in 29 of the 42 patients (69%), including eight patients with no prior suspicion of arrhythmias. Seventeen patients had successful catheter ablation during EPS, and EPS data guided one or more intraoperative rhythm interventions for the remainder. During follow-up, diagnostic yield from ILR was low. Since implementing a more aggressive diagnostic strategy, no further sudden deaths have occurred. Conclusion Preoperative EPS has a high diagnostic and therapeutic yield in patients with Ebstein’s anomaly undergoing the Cone operation. It is reasonable to recommend EPS as a routine preoperative test for this population.
    Heart rhythm: the official journal of the Heart Rhythm Society 01/2014; 11(2):182–186. · 4.56 Impact Factor
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    ABSTRACT: We reported the outcomes of a single-institution experience using video-assisted thoracoscopic left cardiac sympathetic denervation as an adjunctive therapeutic technique in pediatric and young adult patients with life-threatening ventricular arrhythmias. We conducted a retrospective clinical review of all patients who underwent left cardiac sympathetic denervation by means of video-assisted thoracoscopic surgery at our institution. From August 2000 to December 2011, 24 patients (13 with long QT syndrome, 9 with catecholaminergic polymorphic ventricular tachycardia, and 2 with idiopathic ventricular tachycardia) were identified from the cardiology database and surgical records. There were no intraoperative complications. The median postoperative length of stay was 2 days (range, 1-32 days). There were no major perioperative complications. Longer-term follow-up was available in 22 of 24 patients at a median follow-up of 28 months (range, 4-131 months). Sixteen (73%) of the 22 patients experienced a marked reduction in their arrhythmia burden, with 12 (55%) becoming completely arrhythmia free after sympathectomy. Six (27%) of the patients were nonresponsive to treatment; each had persistent symptoms at follow-up. Video-assisted thoracoscopic left cardiac sympathetic denervation can be safely and effectively performed in most patients with life-threatening ventricular arrhythmias. This minimally invasive procedure is a promising adjunctive therapeutic option that achieves a beneficial response in most symptomatic patients. These results support the inclusion of thoracoscopic cardiac sympathetic denervation among the treatment armamentarium in all patients with ventricular arrhythmias refractive to conventional medical therapy.
    The Journal of thoracic and cardiovascular surgery 10/2013; · 3.41 Impact Factor
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    ABSTRACT: Objective: Early results for anatomic repair of congenitally corrected transposition of the great arteries (ccTGA) are excellent. However, the development of left ventricular dysfunction late after repair remains a concern. In this study we sought to determine factors leading to late left ventricular dysfunction and the impact of cardiac resynchronization as a primary and secondary (upgrade) mode of pacing. Methods: From 1992 to 2012, 106 patients (median age at surgery, 1.2 years; range, 2 months to 43 years) with ccTGA had anatomic repair. A retrospective review of preoperative variables, surgical procedures, and postop-erative outcomes was performed. Results: In-hospital deaths occurred in 5.7% (n ¼ 6), and there were 3 postdischarge deaths during a mean follow-up period of 5.2 years (range, 7 days to 18.2 years). Twelve patients (12%) developed moderate or severe left ventricular dysfunction. Thirty-eight patients (38%) were being paced at latest follow-up evaluation. Seven-teen patients had resynchronization therapy, 9 as an upgrade from a prior dual-chamber system (8.5%) and 8 as a primary pacemaker (7.5%). Factors associated with left ventricular dysfunction were age at repair older than 10 years, weight greater than 20 kg, pacemaker implantation, and severe neo-aortic regurgitation. Eight of 9 pa-tients undergoing secondary cardiac resynchronization therapy (upgrade) improved left ventricular function. None of the 8 patients undergoing primary resynchronization developed left ventricular dysfunction. Conclusions: Late left ventricular dysfunction after anatomic repair of ccTGA is not uncommon, occurring most often in older patients and in those requiring pacing. Early anatomic repair and cardiac resynchroniza-tion therapy in patients requiring a pacemaker could preclude the development of left ventricular dysfunction. (J Thorac Cardiovasc Surg 2013;-:1-5)
    Journal of Thoracic and Cardiovascular Surgery 10/2013; · 3.53 Impact Factor
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    ABSTRACT: Non-fluoroscopic imaging (NFI) devices are increasingly used in ablations. The objective was to determine the utility of intracardiac echocardiography (ICE) in ablating paediatric supraventricular tachycardias (SVTs) and assess whether its integrated use with electroanatomic mapping (EAM) resulted in lower radiation exposure than use of EAM alone.METHODS AND RESULTS: Prospective, controlled, single-centre study of patients (pts) age ≥10 years, weight ≥35 kg, with SVT and normal cardiac anatomy. Patients were randomized to ICE + EAM (ICE) or EAM only (no ICE). Both had access to fluoroscopy as needed. Eighty-four pts were enroled (42 ICE, 42 no ICE). Median age was 15 years (range 10.4-23.7 years); 57% had accessory pathways, 42% atrioventricular nodal reentry tachycardia. There was no difference in radiation dose (9 mGy ICE vs. 23 mGy no ICE, P = 0.37) or fluoroscopy time (1.1 min ICE vs. 1.5 min no ICE, P = 0.38). Transseptal punctures were performed in 25 pts (16 ICE, 9 no ICE), with ICE reducing radiation (8 mGy ICE vs. 62 mGy no ICE, P = 0.002) and fluoroscopy time (1.1 min ICE vs. 4.5 min no ICE, P = 0.01). Zero fluoroscopy was achieved in 13 pts (15% of total, 5 ICE, 8 no ICE), and low-dose cases (<50 mGy) in 57 pts (68% of total, 33 ICE, 24 no ICE). Acute success was 95% for ICE, 88% for no ICE.CONCLUSION: Use of an integrated EAM/ICE system was no better than EAM alone in limiting radiation, but can be helpful for transseptal punctures. Given the low dose savings, use of ICE may be weighed against its financial cost. Low-fluoroscopy cases are performed in most NFI procedures.
    Europace 08/2013; · 2.77 Impact Factor
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    ABSTRACT: As mortality in patients with D-loop transposition of the great arteries (D-TGA) has decreased after the arterial switch operation (ASO), the focus has shifted to higher risk groups and outcomes that impact long-term morbidity and mortality, such as left ventricular (LV) dysfunction. We sought to examine the perioperative factors associated with LV dysfunction in patients with D-TGA and ventricular septal defects (VSD) after ASO. A retrospective study was made of all patients with D-TGA/VSD who underwent ASO/VSD closure from 2001 to 2011. Patients with prematurity, L-looped ventricles, and straddling atrioventricular valves were excluded. The primary endpoint was moderate or severe LV dysfunction measured by echocardiogram 2 months or more after surgery. A total of 112 patients underwent ASO/VSD closure at a median age of 5 days. Median time of follow-up was 6.5 months, with no mortality noted. Six patients (8%) were noted to have at least moderate LV dysfunction. Risk factors were heart block requiring pacemaker placement (p < 0.001) and length of intensive care unit admission (p = 0.04). All 6 patients with heart block had an epicardial lead on the right ventricular free wall; 4 had moderate or severe LV dysfunction and underwent upgrade to cardiac resynchronization therapy (CRT); median time from initial pacemaker to CRT was 5 months. With a median follow-up of 5 months after CRT, LV function improved to normal (2 patients) or mild dysfunction (2 patients). Left ventricular dysfunction after surgical repair for D-TGA/VSD is low, with heart block and pacemaker insertion playing a significant role. The LV function improved after patients were upgraded to a CRT device.
    The Annals of thoracic surgery 08/2013; · 3.74 Impact Factor
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    ABSTRACT: BACKGROUND: -Ventricular arrhythmia related sudden cardiac arrest in infants with structurally normal hearts is rare. There have been no previously published reports of infants less than 3 months of age with ventricular fibrillation in which a primary diagnosis could not be defined. METHODS AND RESULTS: -Retrospective chart review of 3 unrelated infants less than 2 months of age from 3 different tertiary care centers within the United States and Australia. All 3 infants survived sudden cardiac arrest secondary to multiple episodes of polymorphic ventricular tachycardia and ventricular fibrillation. Each infant demonstrated unique and transient ECG findings consisting of ST changes and QRS widening prior to arrhythmia onset that have not been previously reported. Amiodarone, sedation, sodium channel blocking agents and/or ventricular pacing were effective in suppressing acute events. Despite thorough investigation including genetic testing, the etiology of the ventricular arrhythmias in each of these infants remains unclear. CONCLUSIONS: -This is the first report of idiopathic ventricular fibrillation in young infants preceded by stereotypical transient ECG changes. These findings may represent a new, potentially treatable cause of sudden infant death. Recognition of these prodromal changes may be important in future management and survival of these infants.
    Circulation Arrhythmia and Electrophysiology 06/2013; · 5.95 Impact Factor
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    ABSTRACT: BACKGROUND: Accessory pathways (APs) with intermittent preexcitation (IPX) are thought to be of lower risk, but there are reports of IPX patients presenting with rapidly conducted atrial fibrillation. METHODS: Retrospective study performed on patients with preexcitation who underwent an electro-physiological study (EPS). IPX was defined as loss of the delta wave on electrocardiogram prior to EPS. Patients with IPX were compared with those with persistent preexcitation (PPX) or suppression of the delta wave on exercise test (IPX-ET). Congenital heart disease and prior ablations were excluded. RESULTS: Of 328 patients with preexcitation, 41 (12.5%) had IPX. Patients with IPX or PPX were similar in age (12.9 years vs 13.0 years, P = 0.8) and AP location (left-sided 54% vs 50%, P = 0.7; septal 32% vs 35%, P = 0.4). Testing on isoproterenol was performed in 17 (41%) IPX and 41 (14%) PPX patients. Although IPX patients had a longer median refractory period compared to PPX patients (340 ms vs 310 ms, P = 0.001), the incidence of APs with refractory periods ≤250 ms was similar (10% vs 12%, P = 1.0). Exercise tests were performed on 208 patients and 24 (12%) had IPX-ET. Compared with IPX patients, IPX-ET had similar median AP refractory periods (320 ms, P = 0.4) and incidence of APs with refractory periods ≤250 ms (13%, P = 1.0). CONCLUSION: Patients with IPX had longer AP refractory periods than those with PPX, but the incidence of pathways with refractory periods ≤250 ms was not significantly different. The finding of IPX on a baseline electrocardiogram does not rule out potentially high-risk pathways.
    Pacing and Clinical Electrophysiology 04/2013; · 1.75 Impact Factor
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    ABSTRACT: BACKGROUND: An increasing number of adults with congenital heart disease (CHD) require implantable cardioverter-defibrillators (ICDs), yet little is known about their impact on psychological well being and sexual function. OBJECTIVE: We sought to assess shock-related anxiety in adults with CHD, and its association with depression and sexual function. METHODS: A prospective multicenter cross-sectional study was conducted on adult CHD patients with (ICD(+)) and without (ICD(-)) ICDs. The Florida Shock Anxiety Scale (FSAS) was administered to ICD(+) patients and the Beck Depression Inventory-II to all patients. Men completed the Sexual Health Inventory for Men and women the Female Sexual Function Index. RESULTS: A total of 180 adults with CHD (ICD(+) n=70; ICD(-) n=110), median age 32 years (27-40), 44% female, were enrolled. The complexity of CHD was classified as mild in 32 (18%), moderate in 93 (52%), and severe in 54 (30%) subjects. In ICD recipients, a high level of shock-related anxiety was identified (FSAS score 16, IQR 12-23.5), which was slightly higher than the median score for ICD recipients in the general population (p=0.057). A higher level of shock-related anxiety was associated with poorer sexual function scores in both men (ρ=-0.480, p<0.001) and women (ρ=-0.512, p<0.01). It was also associated with self-reported depressive symptomatology (ρ=0.536, p<0.001). CONCLUSION: Adults with CHD and ICDs demonstrate a high level of shock-related anxiety, which is associated with lower sexual functioning scores in men and women. These results underscore the need for increased clinical attention related to ICD-related shock anxiety and impaired sexual function in this population.
    Heart rhythm: the official journal of the Heart Rhythm Society 02/2013; · 4.56 Impact Factor
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    ABSTRACT: BACKGROUND: Patients with congenital heart disease carry a high burden of arrhythmias and may pose special challenges when these arrhythmias are addressed invasively. We sought to describe our early experience with radiofrequency (RF) needle transseptal perforation to facilitate ablation procedures in this population. METHODS: Retrospective chart review to identify all cases of attempted transseptal access with a commercial RF needle at Children's Hospital Boston between February 2007 and January 2010. RESULTS: A total of 10 patients had attempted RF transseptal perforation. Median age was 27 years. Five patients had undergone atrial switch procedures (Mustard/Senning), four had undergone Fontan operations, and one had atrial septal defect repair. The indication for left atrial access was mapping/ablation of atrial flutter in nine cases, and left-sided accessory pathway in one case. The RF needle was chosen primarily in eight of 10 cases, whereas in the remaining two cases RF was used only after failed attempts with a conventional Brockenbrough needle. Septal material was atrial muscle in five cases, pericardium in three, and synthetic fabric in two. In nine of 10 patients, RF transseptal perforation was successful, including both patients in whom a conventional needle had failed. There were no clinically significant complications. CONCLUSIONS: RF transseptal perforation can be an effective method of obtaining left atrial access for electrophysiologic procedures in patients with complex congenital heart disease, including cases where a conventional Brockenbrough needle has failed.
    Pacing and Clinical Electrophysiology 02/2013; · 1.75 Impact Factor
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    ABSTRACT: In Fontan and atrial switch patients, transcatheter ablation is limited by difficult access to the pulmonary venous atrium. In recent years, transbaffle access (TBA) has been described, but limited data document its safety and utility. All ablative electrophysiological study cases of this population performed between January 2006 and December 2010 at Boston Children's Hospital were reviewed. Pre-case and follow-up clinical characteristics were documented. Adverse events were classified by severity and attributability to the intervention. We included 118 cases performed in 90 patients. TBA was attempted in 74 cases and was successful in 96%: in 20 via baffle leak or fenestration and in 51 (94%) of 54 using standard or radiofrequency transseptal techniques. There were 10 procedures with adverse events ranked as moderate or more severe. The event rate was similar in both groups (TBA 8% versus non-TBA 9%, P=1), and no events were directly attributable to TBA. There was a trend to higher proportion of cases having a >5-point drop in saturations from baseline in the TBA group versus the non-TBA group in Fontan cases (15% vs 0%, P=0.14). When cases with follow-up >90 and >365 days were analyzed, the median initial arrhythmia score of 5 significantly changed -3 points in both time periods (P≤0.001). TBA is feasible in this population; its use was not associated with a higher incidence of adverse events; and changes in clinical scores support its efficacy. Desaturation observed in some patients is of uncertain significance but warrants postablation monitoring and prospective study.
    Journal of the American Heart Association. 01/2013; 2(5):e000325.
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    ABSTRACT: The presence of multiple accessory pathways (MultAP) is described in structural heart disease (SHD) such as Ebstein's anomaly and cardiomyopathies. Structural defects can impact the tolerability of tachyarrhythmia and can complicate both medical management and ablation. In a large cohort of pediatric patients with and without SHD undergoing invasive electrophysiology study, we examined the prevalence of MultAP and the effect of both MultAP and SHD on ablation outcomes. Accessory pathway number and location, presence of SHD, ablation success, and recurrence were analyzed in consecutive patients from our center over a 16-year period. In 1088 patients, 1228 pathways (36% retrograde only) were mapped to the right side (TV) in 18%, septum (S) in 39%, and left side (MV) in 43%. MultAP were present in 111 pts (10%), involving 250 distinct pathways. SHD tripled the risk of MultAP (26% SHD vs 8% no SHD, P < .001). Multivariable adjusted risk factors for MultAP included Ebstein's (OR 8.7[4.4-17.5], P < .001) and cardiomyopathy (OR 13.3[5.1-34.5], P < .001). Of 1306 ablation attempts, 94% were acutely successful with an 8% recurrence rate. Ablation success was affected by SHD (85% vs 95% for no SHD, P < .01) but not by MultAP (91% vs 94% for single, P = .24). Recurrence rate was higher for SHD (17% SHD vs 8% no SHD, P < .05) and MultAP (19% MultAP vs 8% single, P < .001). MultAP are found in 10% of pediatric patients, and are more common in SHD compared to those with normal hearts. Both the presence of MultAP and SHD negatively influence ablation outcomes.
    American heart journal 01/2013; 165(1):87-92. · 4.65 Impact Factor
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    ABSTRACT: Background Ebstein anomaly is a rare and heterogeneous congenital heart defect affecting the tricuspid valve and right ventricular (RV) myocardium. Few studies have analysed the electrocardiographic features of Ebstein anomaly and none has addressed correlations with disease severity.Methods Patients with Ebstein anomaly who had undergone electrocardiography and cardiac magnetic resonance (CMR) within 6 weeks between 2001 and 2009 were included. Exclusion criteria were: associated congenital cardiac defect, previous RV myoplasty and/or reduction surgery, class I anti-arrhythmic drug therapy, and paced/pre-excited QRS. Standard electrocardiogram (ECG) findings were correlated with CMR-based RV measures and clinical profile.ResultsThe mean age of the 63 study patients was 22 ± 13 years. An RV conduction delay (rsR' pattern in right precordial leads) was present in 45 patients (71%). The QRS duration correlated with anatomic RV diastolic volume (r = +0.56, P < 0.0001) and inversely with RV ejection fraction (EF; r = -0.62, P < 0.0001). The presence of QRS fractionation predicted greater atrialized RV volume (80 ± 31 vs. 45 ± 37 mL/m(2), P < 0.001). Normal QRS duration was associated with smaller anatomic RV diastolic volume (150 ± 57 vs. 256 ± 100 mL/m(2); P < 0.0001), higher RV EF (48 ± 6 vs. 34 ± 14%; P < 0.0001), higher oxygen consumption (VO(2)) at cardiopulmonary exercise (25.8 vs. 21.8 mL/kg/min, P = 0.05) and lower incidence of oxygen desaturation with exercise (25 vs. 65%, P = 0.02).Conclusion Delayed and prolonged depolarization of the RV is common in patients with Ebstein anomaly. The QRS duration is a marker of RV enlargement and dysfunction. QRS fractionation is associated with a greater atrialized RV volume. A preserved surface ECG identifies a subset of patients with Ebstein anomaly with mild morphological and functional abnormalities and better clinical profile.
    European Heart Journal 11/2012; · 14.10 Impact Factor
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    ABSTRACT: Background: Adaptation of implantable cardioverter defibrillator (ICD) systems to the needs of pediatric and congenital heart patients is problematic due to constraints of vascular and thoracic anatomy. An improved understanding of the defibrillation energy and postshock pacing requirements in such patients may help direct more tailored ICD therapy. We describe the first prospective evaluation of defibrillation threshold (DFT) and postshock rhythm in this population. Methods: We prospectively studied patients ≤60 kg at time of ICD intervention. DFTs were obtained using a binary search protocol with three VF inductions. Postshock pacing was programmed using a stepwise protocol, lowering the rate prior to each VF induction. Results: Twenty patients were enrolled: 11 had channelopathy, five congenital heart disease, and four cardiomyopathy. The median age was 16 years, median weight 48 kg. Twelve patients had a transvenous high-voltage coil; eight had pericardial +/- subcutaneous coil(s). Median DFT was 7 J (range 3-31 J); 19/20 patients had DFT ≤15 J and all patients <25 kg had DFT ≤9 J (n = 6). There was no difference in DFT between patients with transvenous versus pericardial +/- subcutaneous coils (median 7 J vs 6 J, P = 0.59). No patient with normal atrioventricular conduction prior to defibrillation required postshock pacing (n = 16). There were no adverse events. Conclusions: These data suggest that many pediatric ICD patients have low DFTs and adequate postshock escape rhythm. This may help determine appropriate parameters for future design of pediatric-specific ICDs. (PACE 2012;00:1-7).
    Pacing and Clinical Electrophysiology 09/2012; · 1.75 Impact Factor
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    ABSTRACT: OBJECTIVE.: Although many Fontan patients undergo pacemaker placement, there are few studies characterizing this population. Our purpose was to compare clinical characteristics, functional status and measures of ventricular performance in Fontan patients with and without a pacemaker. PATIENTS AND DESIGN.: The National Heart, Lung, and Blood Institute funded Pediatric Heart Network Fontan Cross-Sectional Study characterized 546 Fontan survivors. Clinical characteristics, medical history and study outcomes (Child Health Questionnaire [CHQ]), echocardiographic evaluation of ventricular function, and exercise testing) were compared between subjects with and without pacemakers. RESULTS.: Of 71 subjects with pacemakers (13%), 43/71 (61%) were in a paced rhythm at the time of study enrollment (age 11.9 ± 3.4 years). Pacemaker subjects were older at study enrollment, more likely to have single left ventricles, and taking more medications. There were no differences in age at Fontan or Fontan type between the pacemaker and no pacemaker groups. There were no differences in exercise performance between groups. CHQ physical summary scores were lower in the pacemaker subjects (39.7 ± 14.3 vs. 46.1 ± 11.2, P =.001). Ventricular ejection fraction z-score was also lower (-1.4 ± 1.9 vs. -0.8 ± 2.0, P =.05) in pacemaker subjects. CONCLUSIONS.: In our cohort of Fontan survivors, those with a pacemaker have poorer functional status and evidence of decreased ventricular systolic function compared to Fontan survivors without a pacemaker.
    Congenital Heart Disease 07/2012; · 1.01 Impact Factor
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    ABSTRACT: Myocardial fibrosis is a hallmark of hypertrophic cardiomyopathy (HCM) and a risk factor for ventricular arrhythmia. Fibrosis can be reflected in circulating matrix remodeling protein concentrations. We explored differences in circulating markers of extracellular matrix turnover between young HCM patients with versus without history of serious arrhythmia. Using multiplexed and single ELISA, matrix metalloproteinases (MMPs) 1, 2, 3, and 9; tissue inhibitor of metalloproteinases (TIMPs) 1, 2, and 4; and collagen I carboxyterminal peptide (CICP) were measured in plasma from 45 young HCM patients (80% male patients; median age, 17 years [interquartile range, 15-20]). Participants were grouped into serious ventricular arrhythmia history (VA) versus no ventricular arrhythmia history (NoVA). Differences in MMPs between groups were examined nonparametrically. Relationships between MMPs and ventricular arrhythmia were assessed with linear regression, adjusted for interventricular septal thickness, family history of sudden death, abnormal exercise blood pressure, and implantable cardioverter-defibrillator (ICD). In post hoc sensitivity analysis, age was substituted for ICD. The 14 VA patients were older than 31 NoVA patients (median, 19 versus 17 years; P=0.03). All 14 VA and 12 NoVA patients had an ICD. MMP3 concentration was significantly higher in the VA group (VA median, 12.9 μg/mL [interquartile range, 5.7-16.7 μg/mL] versus NoVA, 5.8 μg/mL [interquartile range, 3.7-10.0 μg/mL]; P=0.01). On multivariable analysis, VA was independently associated with increasing MMP3 (standardized β, 0.37; P=0.01). Post hoc adjustment for age attenuated this association. Circulating MMP3 may be a marker of ventricular arrhythmia in adolescent patients with HCM. Because of our role as pediatric providers, we cannot exclude age-related confounding.
    Circulation Heart Failure 05/2012; 5(4):462-6. · 6.68 Impact Factor
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    ABSTRACT:   We report our analysis of conventional surgery and the cone procedure for Ebstein's malformation (EM) of the tricuspid valve at a single institution. Previous conventional surgery for EM, including use of bioprosthetic valves, has inherent problems especially in pediatrics. The newer cone procedure aims to construct a funnel-like valve out of native leaflets, obviating problems with artificial valves.   This is a retrospective cohort study to examine short-term outcomes of both surgeries for EM.   Nineteen patients (our initial cohort) had the cone procedure, and 13 had conventional tricuspid valve repair or replacement. No early deaths occurred in either group. Three cone and one conventional repair patients required reoperation. Two of 19 patients in the cone and one of 13 in the conventional group died suddenly >30 days after operation, assumed secondary to dysrhythmias. At discharge, by two-dimensional echocardiography, the cone group had 85% reduction in tricuspid valve regurgitation (TVR), and the conventional group had 56% reduction, P= .004. This decrease of TVR persisted to a greater extent in the cone group.   Short-term results for the cone procedure are similar to conventional surgery. The cone procedure uses autologous tissue; hypothetically, early favorable improvement in reduction of TVR should persist.
    Congenital Heart Disease 12/2011; 7(1):50-8. · 1.01 Impact Factor
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    ABSTRACT: The aim of this study was to describe a large experience with primary cardiac tumors in pediatric patients, characterize associated arrhythmias, and expand knowledge of natural history and treatment options. Primary cardiac tumors in children are rare. The incidence of arrhythmias is not well-defined, and management plans vary widely. We employed a retrospective single-center review of patients ≤21 years of age diagnosed with a primary cardiac tumor between 1968 and 2010. Clinically significant arrhythmias were defined as: 1) sudden cardiac arrest; 2) nonsustained and sustained ventricular tachycardia (VT); 3) pre-excitation; and 4) sustained supraventricular tachycardia of any mechanism. A total of 173 patients were identified: 106 rhabdomyoma, 25 fibroma, 14 myxoma, 6 vascular, 4 teratoma, 3 lipoma, and 15 other. Median age at diagnosis was 7 months (prenatal to 21 years). Of these, 42 (24%) had clinically significant arrhythmias. Patients with large fibromas were the highest-risk group, with VT occurring in 64%. Among rhabdomyoma patients, 10% had pre-excitation, and 6% had VT. Over a mean follow-up of 6 years (1 day to 34 years, median 4 years), surgical excision was performed in 62 cases, with rhythm treatment being 1 of the indications in 20. Post-operatively, clinically significant arrhythmias were eliminated in 18 of these 20, including all 13 fibroma patients. Clinically significant arrhythmias occurred in 24% of pediatric patients with cardiac tumors, VT being the most common type. Surgical excision for VT associated with rhabdomyomas and fibromas in selected patients is an important and effective management strategy in these patients.
    Journal of the American College of Cardiology 10/2011; 58(18):1903-9. · 14.09 Impact Factor
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    ABSTRACT: Atrial tachycardias (AT) are common after palliation or repair of congenital heart disease. The electroanatomic mechanism of AT in postoperative tetralogy of Fallot (TOF) and double outlet right ventricle (DORV) patients has not been fully explored. Retrospective analysis of TOF or DORV patients was performed in the electrophysiology (EP) lab from January 1997 to March 2010. Sustained ATs were mapped using the Carto system (Biosense Webster, Diamond Bar, CA, USA). Fifty-eight patients were identified with 82 EP studies performed and 127 ATs identified. The first EP study for AT was performed at a median age of 35 years (2-58 years). Ninety-five IART circuits were identified, 5 in a figure-of-8 pattern. There were 13 focal ATs, 4 ectopic ATs, and 15 presentations of atrial fibrillation (AF). The cavotricuspid isthmus (CTI) was the critical area for ablation in the majority of TOF and DORV patients (53%). The CTI, along with the lateral RA wall, made up 85% of IART circuits. Excluding AF, the acute success rate for ablation was 90%. Of the 58 patients, 20 had additional ablation attempts, 19 within 3 years of their first ablation. The CTI and lateral RA wall are critical corridors of conduction in 85% of IART circuits in TOF and DORV patients. The acute success rate for AT ablations is high, but a substantial number of patients have required additional ablation procedures. Recurrences may be reduced if both the CTI and lateral RA wall are targeted and blocked, even if the mapped circuit points only to 1 region. 
    Journal of Cardiovascular Electrophysiology 05/2011; 22(9):1013-7. · 3.48 Impact Factor
  • Journal of The American College of Cardiology - J AMER COLL CARDIOL. 01/2011; 57(14).

Publication Stats

1k Citations
101 Downloads
379.30 Total Impact Points

Institutions

  • 2003–2014
    • Boston Children's Hospital
      • • Department of Pediatrics
      • • Department of Cardiac Surgery
      Boston, Massachusetts, United States
  • 2013
    • University of Massachusetts Boston
      Boston, Massachusetts, United States
    • Stanford University
      Palo Alto, California, United States
  • 2002–2012
    • Harvard Medical School
      • Department of Pediatrics
      Boston, MA, United States
  • 2009
    • Brigham and Women's Hospital
      • Department of Medicine
      Boston, MA, United States
  • 2008
    • Université de Montréal
      Montréal, Quebec, Canada
    • Montreal Heart Institute
      Montréal, Quebec, Canada
  • 2006
    • Vibra Hospital of San Diego
      San Diego, California, United States