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Outcomes of lead extraction in young adults.

Heart Rhythm. 2017 Apr;14(4):537-540

Authors: El-Chami MF, Sayegh MN, Patel A, El-Khalil J, Desai Y, Leon AR, Merchant FM

BACKGROUND: Extraction of pacemaker and defibrillator leads in young adults may be technically challenging because of more extensive fibrosis and calcification in this patient population.
OBJECTIVE: The purpose of this study was to examine outcomes of lead extraction (LE) in young adults at our institution.
METHODS: We retrospectively identified all patients who underwent LE at our institution between January 1, 2007, and May 31, 2016. Patients were divided by age into 2 groups: <40 years (group 1, n = 84) or ≥40 years (group 2, n = 690). Outcomes were determined by medical records review.
RESULTS: Patients in group 2 had a higher overall average number of leads extracted per procedure compared to group 1 (1.64 ± 0.80 vs 1.45 ± 0.64; P <.001). Lead dwell time was similar in the 2 groups (5.7 ± 5 years vs 5.6 ± 4.3 years; P = .95). The younger cohort tended to require femoral extraction techniques more frequently (9.5% vs 4.4%; P = .055). Extraction procedural success (group 1: 94.1%, group 2: 94.9%; P = .792), major complications (group 1: 0%, group 2: 1.3%; P = 1), and periprocedural mortality (group 1: 0%, group 2: 0.86%; P = 1) were similar in the 2 groups.
CONCLUSION: LE can be performed safely and effectively in young adults. Despite the lower number of leads extracted per procedure and the similar lead dwell time, younger adults more frequently required the use of femoral extraction tools, thus highlighting the importance of performing these procedures in centers with advanced expertise in extraction techniques.

PMID: 28189822 [PubMed - indexed for MEDLINE]

Initial in vitro testing of a paediatric continuous-flow total artificial heart.

Interact Cardiovasc Thorac Surg. 2018 Jan 19;:

Authors: Fukamachi K, Karimov JH, Horvath DJ, Sunagawa G, Byram NA, Kuban BD, Moazami N

OBJECTIVES: Mechanical circulatory support has become standard therapy for adult patients with end-stage heart failure; however, in paediatric patients with congenital heart disease, the options for chronic mechanical circulatory support are limited to paracorporeal devices or off-label use of devices intended for implantation in adults. Congenital heart disease and cardiomyopathy often involve both the left and right ventricles; in such cases, heart transplantation, a biventricular assist device or a total artificial heart is needed to adequately sustain both pulmonary and systemic circulations. We aimed to evaluate the in vitro performance of the initial prototype of our paediatric continuous-flow total artificial heart.
METHODS: The paediatric continuous-flow total artificial heart pump was downsized from the adult continuous-flow total artificial heart configuration by a scale factor of 0.70 (1/3 of total volume) to enable implantation in infants. System performance of this prototype was evaluated using the continuous-flow total artificial heart mock loop set to mimic paediatric circulation. We generated maps of pump performance and atrial pressure differences over a wide range of systemic vascular resistance/pulmonary vascular resistance and pump speeds.
RESULTS: Performance data indicated left pump flow range of 0.4-4.7 l/min at 100 mmHg delta pressure. The left/right atrial pressure difference was maintained within ±5 mmHg with systemic vascular resistance/pulmonary vascular resistance ratios between 1.4 and 35, with/without pump speed modulation, verifying expected passive self-regulation of atrial pressure balance.
CONCLUSIONS: The paediatric continuous-flow total artificial heart prototype met design requirements for self-regulation and performance; in vivo pump performance studies are ongoing.

PMID: 29365118 [PubMed - as supplied by publisher]

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Selection of the Best of 2017 in Congenital Heart Disease.

Rev Esp Cardiol (Engl Ed). 2018 Jan 20;:

Authors: García-Aranda B, Sarnago F, Velázquez MT, Mendoza A, López-Gude MJ, Alonso-González R

PMID: 29366713 [PubMed - as supplied by publisher]

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Multiple Roles of Pitx2 in Cardiac Development and Disease.

J Cardiovasc Dev Dis. 2017 Oct 11;4(4):

Authors: Franco D, Sedmera D, Lozano-Velasco E

Cardiac development is a complex morphogenetic process initiated as bilateral cardiogenic mesoderm is specified at both sides of the gastrulating embryo. Soon thereafter, these cardiogenic cells fuse at the embryonic midline configuring a symmetrical linear cardiac tube. Left/right bilateral asymmetry is first detected in the forming heart as the cardiac tube bends to the right, and subsequently, atrial and ventricular chambers develop. Molecular signals emanating from the node confer distinct left/right signalling pathways that ultimately lead to activation of the homeobox transcription factor Pitx2 in the left side of distinct embryonic organ anlagen, including the developing heart. Asymmetric expression of Pitx2 has therefore been reported during different cardiac developmental stages, and genetic deletion of Pitx2 provided evidence of key regulatory roles of this transcription factor during cardiogenesis and thus congenital heart diseases. More recently, impaired Pitx2 function has also been linked to arrhythmogenic processes, providing novel roles in the adult heart. In this manuscript, we provide a state-of-the-art review of the fundamental roles of Pitx2 during cardiogenesis, arrhythmogenesis and its contribution to congenital heart diseases.

PMID: 29367545 [PubMed]

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Atrial Function after the Atrial Switch Operation for Transposition of the Great Arteries: Comparison with Arterial Switch and Normals by Cardiovascular Magnetic Resonance.

Congenit Heart Dis. 2016 Sep;11(5):426-436

Authors: Franzoso FD, Wohlmuth C, Greutmann M, Kellenberger CJ, Oxenius A, Voser EM, Valsangiacomo Buechel ER

OBJECTIVES: The atria serve as reservoir, conduit, and active pump for ventricular filling. The performance of the atrial baffles after atrial switch repair for transposition of the great arteries may be abnormal and impact the function of the systemic right ventricle. We sought to assess atrial function in patients after atrial repair in comparison to patients after arterial switch repair (ASO) and to controls.
METHODS: Using magnetic resonance imaging, atrial volumes and functional parameters were measured in 17 patients after atrial switch repair, 9 patients after ASO and 10 healthy subjects.
RESULTS: After the atrial switch operation, the maximum volume of the pulmonary venous atrium was significantly enlarged, but not of the systemic venous atrium. In both patients groups, independently from the surgical technique used, the minimum atrial volumes were elevated, which resulted in a decreased total empting fraction compared with controls (P < .01). The passive empting volume was diminished for right atrium, but elevated for left atrium after atrial switch and normal for left atrium after ASO; however, the passive empting fraction was diminished for both right atrium and left atrium after both operations (P < .01). The active empting volume was the most affected parameter in both atria and both groups and active empting fractions were highly significantly reduced compared with controls.
CONCLUSION: Atrial function is abnormal in all patients, after atrial switch and ASO repair. The cyclic volume changes, that is, atrial filling and empting, are reduced when compared with normal subjects. Thus, the atria have lost part of their capacity to convert continuous venous flow into a pulsatile ventricular filling. The function of the pulmonary venous atrium, acting as preload for the systemic right ventricle, after atrial switch is altered the most.

PMID: 26680275 [PubMed - indexed for MEDLINE]

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Obesity and Diabetes Mellitus Adversely Affect Outcomes after Cardiac Surgery in Children's Hospitals.

Congenit Heart Dis. 2016 Sep;11(5):409-414

Authors: Shamszad P, Rossano JW, Marino BS, Lowry AW, Knudson JD

OBJECTIVE: To assess how obesity or diabetes mellitus impacts outcomes in patients undergoing cardiac surgery in pediatric hospitals.
DESIGN: A multi-institutional, matched case-control study of the Pediatric Health Information System database was performed.
SETTING: Tertiary children's hospitals in the United States.
PATIENTS: All cardiac surgical cases in patients with obesity or diabetes mellitus between 2004 and 2012 were included. Cases were matched to controls by age, sex, race, and Risk Adjustment for Congenital Heart Surgery score.
OUTCOME MEASURES: Mortality, surgical complications, and hospital utilization. Differences in outcome measures were assessed by chi-square and Mann-Whitney tests. P value < .05 was significant.
RESULTS: Six hundred twenty-nine cardiac surgical cases (median age 17 years [IQR 12-32]) with obesity or diabetes mellitus were matched to 629 controls. Cases demonstrated lower median household income than those in the control group ($38,031 [IQR $31,900-$48,844] vs. ($41,896 [IQR $32,854-$56,020], P < .001). Mortality was similar between cases and controls (22% vs. 1.9%, P =.692). Surgical complications occurred similarly between cases and controls (13.5% vs. 12.4%, P = .535). Cases had longer intensive care unit length of stay than controls (3 vs. 2 days, P = .001), resulting in longer overall hospital length of stay (5 vs. 4 days, P < .001). Cases also had a higher odds of undergoing mechanical ventilation for >96 hours (OR 2.0, 95% CI 1.1-3.7) and higher rate of total parenteral nutrition use (7.2% vs. 4.5%, P = .040). Median hospital charges were higher in cases (clinical: $6,696 vs. $5,872; laboratory: $14,168 vs. $12,251; pharmacy: $12,971 vs. $10,426; imaging: $6,259 vs. $5,660; P ≤ .030 for all).
CONCLUSIONS: The presence of obesity or diabetes mellitus was associated with increased postoperative morbidity, hospital utilization, and cost in patients undergoing cardiac surgery in pediatric hospitals.

PMID: 26887350 [PubMed - indexed for MEDLINE]

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Incomplete Cor Triatriatum Dexter and Its Clinical and Technical Implications in Interatrial Shunt Device-Based Closure: An Intracardiac Echocardiography Study.

Congenit Heart Dis. 2016 Sep;11(5):420-425

Authors: Rigatelli G, Dell'Avvocata F, Giordan M, Vassilev D, Cardaioli P

OBJECTIVES: Cor triatriatum dexter (CTD) is a congenital anomaly in which the right atrium is divided into two parts by a membrane or fibromuscular band. Incomplete separation of the right atrium may occur when prominent venous valve remnants such as Eustachian valve (EV) or Chiari network (CN) incompletely divided the right atrium (incomplete CTD-iCTD). We sought to assess the incidence of EV/CN and iCTD and its clinical and technical implications in patients submitted to interatrial shunt transcatheter closure.
DESIGN: Retrospective analysis of single center registry.
SETTING: Secondary referral center.
PATIENTS: Five hundred eighty consecutive patients (mean age 44 ± 15.5 years, 385 females) who had been submitted over a 12 years period to intracardiac echocardiography-aided interatrial shunt catheter-based closure.
OUTCOMES MEASURES: Prevalence of iCTD and EV/CN, shunt grade, right ventricle diameter, incidence of intraprocedural complications.
RESULTS: In patients with PFO, a prominent EV or a large CN and iCTD have been diagnosed in was diagnosed on ICE in 51.1% and 5.2%, respectively. In ASD patients, a prominent EV or a large CN and iCTD were apparent in 13.7% and 5.6%, respectively. PFO patients with iCTD had more frequently a curtain pattern on TC Doppler and a larger right-to-left shunt graded than prominent EV/CN patients and patients without. ASD patients with iCTD had larger right ventricle diameter than both ASD patients with EV/CN and patients without. iCTD was associated with 45.1% of patients with intraoperative complications.
CONCLUSIONS: iCTD are not so infrequently observed by ICE during interatrial shunt closure procedure. Presence of this peculiar structure should be taken in account during device-based procedure in the right atrium.

PMID: 26887534 [PubMed - indexed for MEDLINE]

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Effect of Left Cardiac Sympathetic Denervation on the Electromechanical Window in Patients with either Type 1 or Type 2 Long QT Syndrome: A Pilot Study.

Congenit Heart Dis. 2016 Sep;11(5):437-443

Authors: Schneider AE, Bos JM, Ackerman MJ

BACKGROUND: Left cardiac sympathetic denervation (LCSD) exerts significant antifibrillatory effects in patients with long QT syndrome (LQTS). Recently, electromechanical window (EMW) has emerged as a novel torsadogenic marker in LQTS, superior to QT interval (QTc) in distinguishing symptomatic from asymptomatic patients.
OBJECTIVE: To explore the hypothesis that LCSD improves EMW most favorably in patients with LQT1.
DESIGN: From September 2006 to July 2015, 44 LQT1 and 25 LQT2 patients underwent LCSD. Subset analysis was performed on the six LQT1 and seven LQT2 patients who had echocardiograms both pre-LCSD and ≥3 months post-LCSD. EMW is defined as the time difference (ms) between aortic valve closure and the end of the QT interval, measured from an ECG on the concurrent echocardiogram.
RESULTS: Compared to published normal EMW values of 22 ± 19 ms, pre-LCSD EMW mean values were -78 ± 36 ms in LQT1 and -71 ± 35 ms in LQT2 (P < .001). Following LCSD, there was a 57 ± 35 ms decrease in QTc in LQT1 (P = .16) and 23 ± 21 ms decrease in QTc in LQT2 (P = .3). Overall, there was a 35 ± 57 ms mean improvement in EMW post-LCSD (P = .04). Five of the 6 (83%) LQT1 subjects had a favorable EMW change post-LCSD (mean improvement 56 ± 25 ms, P = .04). Five of the 7 (71%) LQT2 subjects had a favorable EMW change post-LCSD (mean improvement 18 ± 19 ms, P = .2).
CONCLUSIONS: The precise mechanism of the LCSD therapeutic effect in LQTS patients is not fully understood. This pilot study raises the possibility that LCSD's antitorsadogenic effect in patients with LQT1 could be conferred in part by restoration of electromechanical order, evidenced by normalization of the EMW.

PMID: 26887900 [PubMed - indexed for MEDLINE]

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Needles in Hay II: Detecting Cardiac Pathology by the Pediatric Chest Pain Standardized Clinical Assessment and Management Plan.

Congenit Heart Dis. 2016 Sep;11(5):396-402

Authors: Kane DA, Friedman KG, Fulton DR, Geggel RL, Saleeb SF

OBJECTIVES: To determine if patients evaluated using the pediatric chest pain standardized clinical assessment and management plan (SCAMP) in cardiology clinic were later diagnosed with unrecognized cardiac pathology, and to determine if other patients with cardiac pathology not enrolled in the SCAMP would have been identified using the algorithm.
STUDY DESIGN: Patients 7-21 years of age, newly diagnosed with hypertrophic or dilated cardiomyopathy, coronary anomalies, pulmonary embolus, pulmonary hypertension, pericarditis, or myocarditis were identified from the Boston Children's Hospital (BCH) cardiac database between July 1, 2010 and December 31, 2012. Patients were cross-referenced to the SCAMP database or retrospectively assessed with the SCAMP algorithm.
RESULTS: Among 98 patients with cardiac pathology, 34 (35%) reported chest pain, of whom 10 were diagnosed as outpatients. None of these patients were enrolled in the SCAMP because of alternate chief complaints (n = 4) or referral to BCH for management of the new diagnosis (n = 6). Each of these patients would have had an echocardiogram recommended by retrospective application of the SCAMP algorithm. Two other patients with cardiac pathology were among the 1124 patients assessed by the SCAMP. One patient initially diagnosed with noncardiac chest pain presented 18 months later and was diagnosed with myocarditis as an inpatient. One patient seen initially in the emergency department was subsequently diagnosed with pericarditis as an outpatient.
CONCLUSIONS: Patients assessed by the chest pain SCAMP at BCH were not later diagnosed with cardiac pathology that was missed at the initial encounter. Nonenrolled outpatients with cardiac pathology and chest pain would have been successfully identified with the SCAMP algorithm.

PMID: 26918410 [PubMed - indexed for MEDLINE]

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A Review of Surgical Atrioventricular Block with Emphasis in Patients with Single Ventricle Physiology.

Congenit Heart Dis. 2016 Sep;11(5):462-467

Authors: Marshall AM

We perceived an increased incidence of surgical atrioventricular (AV) block in patients with single ventricle physiology undergoing two ventricle rehabilitation for hypoplastic left heart syndrome compared to the overall incidence of surgical AV block for our institution. Retrospective investigation of our center's data revealed a statistically significant increase in the incidence of surgical AV block in the single ventricle population and two ventricle rehabilitation population compared to the two ventricle population. Here we review the literature with respect to historic definitions, incidence, risk factors, pre- and post-op management, current indications for pacemaker placement and added cost and comorbidity associated with surgical AV block. We then offer possible strategies for decreasing the incidence of surgical AV block within both the single and two ventricle populations.

PMID: 27139742 [PubMed - indexed for MEDLINE]