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75 results on '"von Alvensleben, Johannes C."'

56. Cardiac ryanodine receptor calcium release deficiency syndrome

Author

Sun, Bo, primary, Yao, Jinjing, additional, Ni, Mingke, additional, Wei, Jinhong, additional, Zhong, Xiaowei, additional, Guo, Wenting, additional, Zhang, Lin, additional, Wang, Ruiwu, additional, Belke, Darrell, additional, Chen, Yong-Xiang, additional, Lieve, Krystien V.V., additional, Broendberg, Anders K., additional, Roston, Thomas M., additional, Blankoff, Ivan, additional, Kammeraad, Janneke A., additional, von Alvensleben, Johannes C., additional, Lazarte, Julieta, additional, Vallmitjana, Alexander, additional, Bohne, Loryn J., additional, Rose, Robert A., additional, Benitez, Raul, additional, Hove-Madsen, Leif, additional, Napolitano, Carlo, additional, Hegele, Robert A., additional, Fill, Michael, additional, Sanatani, Shubhayan, additional, Wilde, Arthur A.M., additional, Roberts, Jason D., additional, Priori, Silvia G., additional, Jensen, Henrik K., additional, and Chen, S. R. Wayne, additional

Published
2021
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60. Novel measures of left ventricular electromechanical discoordination predict clinical outcomes in children with pulmonary arterial hypertension

Author

Frank, Benjamin S., primary, Schäfer, Michal, additional, Douwes, Johannes M., additional, Ivy, D. Dunbar, additional, Abman, Steven H., additional, Davidson, Jesse A., additional, Burzlaff, Sandra, additional, Mitchell, Max B., additional, Morgan, Gareth J., additional, Browne, Lorna P., additional, Barker, Alex J., additional, Truong, Uyen, additional, and von Alvensleben, Johannes C., additional

Published
2020
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62. Predictors of electrocardiographic screening failure for the subcutaneous implantable cardioverter-defibrillator in children: A prospective multicenter study

Author

Campbell, Matthew, Moore, Jeremy P., Sreeram, Narayanswami, von Alvensleben, Johannes C., Shah, Anjan, Batra, Anjan, Law, Ian, Sanatani, Shubhayan, Thomas, Vincent, Nik-Ahd, Farnoosh, Williams, Stephen, Nosavan, Nina, Maldonado, Jennifer, Hart, Amelia, Balaji, Seshadri, Campbell, Matthew, Moore, Jeremy P., Sreeram, Narayanswami, von Alvensleben, Johannes C., Shah, Anjan, Batra, Anjan, Law, Ian, Sanatani, Shubhayan, Thomas, Vincent, Nik-Ahd, Farnoosh, Williams, Stephen, Nosavan, Nina, Maldonado, Jennifer, Hart, Amelia, and Balaji, Seshadri

Abstract

BACKGROUND Subcutaneous implantable cardioverter-defibrillator (SICD) shows promise for select patients at risk of sudden cardiac death. However, patients need to pass an electrocardiographic (ECG) screening (ECG-S) test before they can receive an SICD. Predictors of ECG-S failure in children are unclear. OBJECTIVE The purpose of this study was to identify the incidence and predictive factors for failure of ECG-S in children. METHODS Patients 18 years and younger with a preexisting ICD underwent ECG-S for SICD. ECG and demographic data were analyzed for factors predictive of failure. RESULTS Seventy-three patients (mean age 14.2 +/- 3.3 years; range 5-18 years) with hypertrophic cardiomyopathy (n = 24, 33%), long QT syndrome (n =18, 25%), other inherited arrhythmia syndromes (n = 20, 27%), congenital heart disease (n = 9, 12%), and miscellaneous conditions (n = 2) with an existing transvenous ICD underwent prospective ECG-S. Nineteen (26%) failed ECG-S. Failed patients had a longer corrected QT (QTc) interval (457 ms vs 425 ms; P = .03), a longer QRS duration (120 ms vs 98 ms; P = .04), and a lower ratio of R-wave to T-wave amplitudes (R:T ratio) in lead aVF (4 vs 5; P = .001). Multivariable logistic regression identified QTc interval (odds ratio [OR] 4.31; P = .04), QRS duration (OR 4.93; P = .03), R:T ratio in lead aVF (OR 3.13; P = .08) as predictors of failure. A risk score with 1 point each for QTc interval >440 ms, QRS duration >120 ms, and R:T ratio <6.5 in lead aVF was associated with probability of failure of 15.4% (1 point), 47.4 0 /. (2 points), and 88.6% (3 points), respectively. CONCLUSION ECG-S failure for SICD occurred in 26% of children, which is higher than the reported incidence in adults. Factors predicting ECG-S failure included longer QTc interval, longer QRS duration, and lower R:T ratio in lead aVF.

Published
2018

64. Predictors of electrocardiographic screening failure for the subcutaneous implantable cardioverter-defibrillator in children: A prospective multicenter study

Author

Campbell, Matthew, primary, Moore, Jeremy P., additional, Sreeram, Narayanswami, additional, von Alvensleben, Johannes C., additional, Shah, Anjan, additional, Batra, Anjan, additional, Law, Ian, additional, Sanatani, Shubhayan, additional, Thomas, Vincent, additional, Nik-Ahd, Farnoosh, additional, Williams, Stephen, additional, Nosavan, Nina, additional, Maldonado, Jennifer, additional, Hart, Amelia, additional, Nguyen, Thuan, additional, and Balaji, Seshadri, additional

Published
2018
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72. Syncope and Palpitations: A Review.

Author

von Alvensleben JC

Subjects
Arrhythmias, Cardiac physiopathology, Child, Diagnosis, Differential, Humans, Syncope physiopathology, Arrhythmias, Cardiac diagnosis, Electrocardiography, Heart Conduction System physiopathology, Heart Rate physiology, Syncope diagnosis
Abstract

Syncope and palpitations are common complaints for patients presenting to their primary care provider. They represent symptoms that most often have a benign etiology but rarely can be the first warning sign of a serious condition, such as arrhythmias, structural heart disease, or noncardiac disease. The history, physical examination, and noninvasive testing can, in most cases, distinguish benign from pathologic causes. This article introduces syncope and palpitations, with emphasis on the differential diagnoses, initial presentation, diagnostic strategy, and various management strategies., Competing Interests: Disclosure The author has nothing to disclose., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Published
2020
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73. Novel measures of left ventricular electromechanical discoordination predict clinical outcomes in children with pulmonary arterial hypertension.

Author

Frank BS, Schäfer M, Douwes JM, Ivy DD, Abman SH, Davidson JA, Burzlaff S, Mitchell MB, Morgan GJ, Browne LP, Barker AJ, Truong U, and von Alvensleben JC

Subjects
Adolescent, Blood Pressure, Child, Electrophysiological Phenomena, Female, Hemodynamics, Humans, Magnetic Resonance Imaging, Male, Mechanical Phenomena, Myocardial Contraction, Pulmonary Arterial Hypertension diagnostic imaging, Retrospective Studies, Treatment Outcome, Ventricular Dysfunction, Left diagnostic imaging, Ventricular Dysfunction, Right physiopathology, Heart Function Tests, Pulmonary Arterial Hypertension physiopathology, Ventricular Dysfunction, Left physiopathology
Abstract

Adverse ventricle-ventricle interaction and resultant left ventricular (LV) dysfunction are a recognized pathophysiological component of disease progression in pulmonary arterial hypertension (PAH) and can be associated with electrical and mechanical dyssynchrony. The purpose of this study was to investigate the clinical and mechanistic implications of LV electromechanical dyssynchrony in children with PAH by using novel systolic stretch and diastolic relaxation discoordination indexes derived noninvasively from cardiac MRI (CMR). In children with PAH referred for CMR ( n = 64) and healthy controls ( n = 20), we calculated two novel markers of ventricular discoordination, systolic stretch fraction (SSF) and diastolic relaxation fraction (DRF). SSF and DRF were evaluated with respect to 1 ) electrical dyssynchrony, 2 ) functional status, and 3 ) composite clinical outcomes. SSF was increased in patients with PAH compared with controls ( P = 0.004). There was no difference in DRF between PAH and control groups. There were no differences between groups in standard mechanical dyssynchrony and LV global circumferential strain. Increased SSF was associated with greater electrical dyssynchrony (QRS duration) as well as worse WHO functional class. SSF, DRF, mechanical dyssynchrony, and right ventricular (RV) volumes were prognostic for worse clinical outcomes. LV dyssynchrony indexes are altered in pediatric patients with PAH compared with controls in proportion with greater degrees of RV dilation. Patients with PAH with greater dyssynchrony have worse clinical outcomes. RV-induced increased LV electromechanical dyssynchrony therefore may be an important link in the causal pathway from PAH to clinically significant LV dysfunction. Since dyssynchrony could precede overt LV dysfunction, addition of ventricular synchrony analysis to CMR postprocessing protocols may be of clinical benefit. NEW & NOTEWORTHY We demonstrate that left ventricular discoordination indexes are altered in pediatric patients with pulmonary arterial hypertension compared with controls and pediatric patients with pulmonary arterial hypertension with greater dyssynchrony have worse clinical outcomes. Furthermore, there is evidence for the mechanism of right ventricular-induced left ventricular discoordination to include a combination of delayed early systolic electromechanical activation, late-systolic septal shift, and prolonged, postsystolic septal thickening.

Published
2020
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74. Intraoperative Epicardial Triventricular Pacing in a Pediatric Patient.

Author

von Alvensleben JC, Pinder MA, Brateng C, Mitchell M, and Collins KK

Abstract

Cardiac resynchronization therapy (CRT) is used as an adjunctive therapy in adults with advanced heart failure but remains less commonly applied in pediatric patients. Further, CRT is traditionally conducted via biventricular transvenous pacing from the right ventricle and coronary sinus to activate the left ventricle and improve electromechanical synchrony; however, triventricular pacing, in which a third ventricular lead is utilized to activate an additional ventricular location, has been shown to be a feasible therapeutic alternative to typical CRT in patients with advanced heart failure or nonresponders. Limited adult studies involving triventricular pacing have been performed to date but no pediatric data are available. Thus, we present the case of a 12-month-old patient with congenital complete heart block and subsequent pacemaker-induced cardiomyopathy in whom triventricular epicardial pacing was applied in an effort to increase the available knowledge., Competing Interests: The authors report no conflicts of interest for the published content., (Copyright: © 2019 Innovations in Cardiac Rhythm Management.)

Published
2019
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