J. Martijn Bos
Mayo Clinic
QT intervalInternal medicinePhenotypeGenotypeCardiologyPediatricsSudden cardiac deathImplantable cardioverter-defibrillatorCatecholaminergic polymorphic ventricular tachycardiaCardiomyopathyLong QT syndromeElectrocardiographyHypertrophic cardiomyopathyGenetic testingSudden deathAnesthesiaGeneticsMedicineCohortBiology
Publications 146
#1Hao Cui (Mayo Clinic)H-Index: 4
#2Hartzell V. Schaff (Mayo Clinic)H-Index: 131
Last. Joseph J. Maleszewski (Mayo Clinic)H-Index: 41
view all 12 authors...
Abstract Background Hypertrophic cardiomyopathy (HCM) is characterized by multiple pathological features including myocyte hypertrophy, myocyte disarray, and interstitial fibrosis. Objectives This study sought to correlate myocardial histopathology with clinical characteristics of patients with obstructive HCM and post-operative outcomes following septal myectomy. Methods The authors reviewed the pathological findings of the myocardial specimens from 1,836 patients with obstructive HCM who under...
1 CitationsSource
#1J. Martijn Bos (Mayo Clinic)H-Index: 32
#2Zachi I. Attia (Mayo Clinic)H-Index: 12
Last. Michael J. Ackerman (Mayo Clinic)H-Index: 144
view all 6 authors...
Importance Long QT syndrome (LQTS) is characterized by prolongation of the QT interval and is associated with an increased risk of sudden cardiac death. However, although QT interval prolongation is the hallmark feature of LQTS, approximately 40% of patients with genetically confirmed LQTS have a normal corrected QT (QTc) at rest. Distinguishing patients with LQTS from those with a normal QTc is important to correctly diagnose disease, implement simple LQTS preventive measures, and initiate prop...
4 CitationsSource
#1Steven M. Dotzler (Mayo Clinic)H-Index: 2
#2C.S. John Kim (Mayo Clinic)H-Index: 2
Last. Michael J. Ackerman (Mayo Clinic)H-Index: 144
view all 9 authors...
Background: Type 1 long QT syndrome (LQT1) is caused by loss-of-function variants in the KCNQ1-encoded Kv7.1 potassium channel α-subunit which is essential for cardiac repolarization, providing the...
#1John R. Giudicessi (Mayo Clinic)H-Index: 22
#2Matthew SchramH-Index: 2
Last. Michael J. Ackerman (Mayo Clinic)H-Index: 144
view all 14 authors...
Background: Heart rate-corrected QT interval (QTc) prolongation, whether secondary to drugs, genetics including congenital long QT syndrome (LQTS), and/or systemic diseases including SARS-CoV-2-mediated COVID19, can predispose to ventricular arrhythmias and sudden cardiac death. Currently, QTc assessment and monitoring relies largely on 12-lead electrocardiography. As such, we sought to train and validate an artificial intelligence (AI)-enabled 12-lead electrocardiogram (ECG) algorithm to determ...
2 CitationsSource
#1Gurukripa N. Kowlgi (Mayo Clinic)H-Index: 3
#2John R. Giudicessi (Mayo Clinic)H-Index: 22
Last. Michael J. Ackerman (Mayo Clinic)H-Index: 144
view all 5 authors...
BACKGROUND Unfortunately, some patients with long QT syndrome (LQTS) experience breakthrough cardiac events (BCEs) despite maximal therapy. Small studies have shown that refractory LQTS cases may benefit from intentional permanent atrial pacing (IPAP). As such, we sought to determine the genotype-specific utilization and efficacy of IPAP in a single-center LQTS registry. METHODS AND RESULTS In this retrospective study, electronic medical records from 1,065 patients diagnosed with LQTS were used ...
#1Roddy Walsh (UvA: University of Amsterdam)H-Index: 23
#2Najim Lahrouchi (UvA: University of Amsterdam)H-Index: 9
Last. Connie R. Bezzina (UvA: University of Amsterdam)H-Index: 68
view all 119 authors...
Purpose: Stringent variant interpretation guidelines can lead to high rates of variants of uncertain significance (VUS) for genetically heterogeneous disease like long QT syndrome (LQTS) and Brugada syndrome (BrS). Quantitative and disease-specific customization of American College of Medical Genetics and Genomics/Association for Molecular Pathology (ACMG/AMP) guidelines can address this false negative rate. Methods: We compared rare variant frequencies from 1847 LQTS (KCNQ1/KCNH2/SCN5A) and 333...
4 CitationsSource
#1Alan Sugrue (Mayo Clinic)H-Index: 12
#2Martin van Zyl (Mayo Clinic)H-Index: 4
Last. Michael J. Ackerman (Mayo Clinic)H-Index: 144
view all 9 authors...
Abstract Background The ability to identify those patients at the highest phenotypic risk for long QT syndrome (LQTS)–associated life-threatening cardiac events remains suboptimal. Objectives This study sought to validate the association between electromechanical window (EMW) negativity, as derived from echocardiography, and symptomatic versus asymptomatic status in patients with LQTS. Methods We analyzed a cohort of 651 patients with LQTS (age 26 ± 17 years; 60% females; 158 symptomatic; 51% LQ...
3 CitationsSource
#1Talha Niaz (Mayo Clinic)H-Index: 8
#2J. Martijn Bos (Mayo Clinic)H-Index: 32
Last. Michael J. Ackerman (Mayo Clinic)H-Index: 144
view all 5 authors...
Background - Videoscopic left cardiac sympathetic denervation (LCSD) is an effective anti-fibrillatory, minimally invasive therapy for patients with potentially life-threatening arrhythmia syndromes like long QT syndrome (LQTS). Although initially used primarily for treatment intensification following documented LQTS-associated breakthrough cardiac events (BCEs) while on beta-blockers, LCSD as one-time monotherapy for certain patients with LQTS requires further evaluation. We are presenting our ...