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Raymond W Sy,
Christian van der Werf,
Ishvinder S Chattha,
Priya Chockalingam,
Arnon Adler,
Jeffrey S Healey,
Mark Perrin,
Michael H Gollob,
Allan C Skanes,
Raymond Yee,
Lorne J Gula,
Peter Leong-Sit,
Sami Viskin,
George J Klein, Arthur A Wilde,
Andrew D Krahn
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ABSTRACT: Genetic testing can diagnose long-QT syndrome (LQTS) in asymptomatic relatives of patients with an identified mutation; however, it is costly and subject to availability. The accuracy of a simple algorithm that incorporates resting and exercise ECG parameters for screening LQTS in asymptomatic relatives was evaluated, with genetic testing as the gold standard.
Asymptomatic first-degree relatives of genetically characterized probands were recruited from 5 centers. QT intervals were measured at rest, during exercise, and during recovery. Receiver operating characteristics were used to establish optimal cutoffs. An algorithm for identifying LQTS carriers was developed in a derivation cohort and validated in an independent cohort. The derivation cohort consisted of 69 relatives (28 with LQT1, 20 with LQT2, and 21 noncarriers). Mean age was 35±18 years, and resting corrected QT interval (QTc) was 466±39 ms. Abnormal resting QTc (females ≥480 ms; males ≥470 ms) was 100% specific for gene carrier status, but was observed in only 48% of patients; however, mutations were observed in 68% and 42% of patients with a borderline or normal resting QTc, respectively. Among these patients, 4-minute recovery QTc ≥445 ms correctly restratified 22 of 25 patients as having LQTS and 19 of 21 patients as being noncarriers. The combination of resting and 4-minute recovery QTc in a screening algorithm yielded a sensitivity of 0.94 and specificity of 0.90 for detecting LQTS carriers. When applied to the validation cohort (n=152; 58 with LQT1, 61 with LQT2, and 33 noncarriers; QTc=443±47 ms), sensitivity was 0.92 and specificity was 0.82.
A simple algorithm that incorporates resting and exercise-recovery QTc is useful in identifying LQTS in asymptomatic relatives.
Circulation 11/2011; 124(20):2187-94. · 14.74 Impact Factor
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ABSTRACT: Cardiac channelopathies caused by SCN5A mutation are well tolerated by most patients. However, the dramatic presentation of a previously healthy 4-month-old girl with life-threatening arrhythmias and the subsequent findings in the child and her family provide evidence that loss-of-function sodium channel mutations can present very early in life. An SCN5A mutation was detected in the infant, her brother, and their father. Both the siblings manifested recurrent serious arrhythmias during febrile episodes, which followed immunization, as well as fever of nonspecific origin. Management consisted of prompt antipyretic measures, hospitalization with vigorous monitoring during immunization and febrile episodes, and prevention of tachycardia-induced conduction disturbance with β-blockers.
PEDIATRICS 01/2011; 127(1):e239-44. · 4.47 Impact Factor
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Niels J Verouden,
Joost D Haeck,
Karel T Koch,
José P Henriques,
Jan Baan,
René J van der Schaaf,
Marije M Vis,
Ron J Peters, Arthur A Wilde,
Jan J Piek,
Jan G Tijssen,
Robbert J de Winter
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ABSTRACT: The prognostic value of ST-segment resolution (STR) after initiation of reperfusion therapy has been established by various studies conducted in both the thrombolytic and mechanic reperfusion era. However, data regarding the value of STR immediately prior to primary percutaneous coronary intervention (PCI) to predict infarct-related artery (IRA) patency remain limited. We investigated whether STR prior to primary PCI is a reliable, noninvasive indicator of IRA patency in patients with ST-segment elevation myocardial infarction (STEMI).
The study population consisted of STEMI patients who underwent primary PCI at our institution between 2000 and 2007. STR was analyzed in 12-lead electrocardiograms recorded at first medical contact and immediately prior to primary PCI and defined as complete (> or =70%), partial (70%- 30%), or absent (<30%).
In 1253 patients with a complete data set, STR was inversely related to the probability of impaired preprocedural flow (P(for trend) < 0.001). Although the sensitivity of incomplete (<70%) STR to predict a Thrombolysis in Myocardial Infarction (TIMI) flow of <3 was 96%, the specificity was 23%, and the negative predictive value of incomplete STR to predict normal coronary flow was only 44%.
This study establishes the correlation between STR prior to primary PCI and preprocedural TIMI flow in STEMI patients treated with primary PCI. However, the negative predictive value of incomplete STR for detection of TIMI-3 flow is only 44% and therefore should not be a criterion to refrain from immediate coronary angiography in STEMI patients.
Annals of Noninvasive Electrocardiology 04/2010; 15(2):107-15. · 1.10 Impact Factor
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Heart rhythm: the official journal of the Heart Rhythm Society 03/2010; 7(6):863. · 4.56 Impact Factor
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N J Verouden,
K T Koch,
R J Peters,
J P Henriques,
J Baan,
R J van der Schaaf,
M M Vis,
J G Tijssen,
J J Piek,
H J Wellens, A A Wilde,
R J de Winter
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ABSTRACT: To describe patients with a distinct electrocardiogram (ECG) pattern without ST-segment elevation in the presence of an acute occlusion of the proximal left anterior descending (LAD) artery.
Single-centre observational study.
Patients with acute anterior wall myocardial infarction who were referred for primary percutaneous coronary intervention (PCI) between 1998 and 2008.
We identified patients with a static, distinct ECG pattern without ST-segment elevation and an occlusion of the proximal LAD artery during urgent coronary angiography before PCI. Of 1890 patients who underwent primary PCI of the LAD artery, we could identify 35 patients (2%) with this distinct ECG pattern. The ECG showed ST-segment depression at the J-point of at least 1 mm in precordial leads with upsloping ST-segments continuing into tall, symmetrical T-waves. Patients with this distinct ECG pattern were younger, more often male and more often had hypercholesterolaemia compared to patients with anterior myocardial infarction and ST-segment elevation.
In patients presenting with chest pain, ST-segment depression at the J-point with upsloping ST-segments and tall, symmetrical T-waves in the precordial leads of the 12-lead ECG signifies proximal LAD artery occlusion. It is important for cardiologists and emergency care physicians to recognise this distinct ECG pattern, so they can triage such patients for immediate reperfusion therapy.
Heart (British Cardiac Society) 08/2009; 95(20):1701-6. · 4.22 Impact Factor
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Heart rhythm: the official journal of the Heart Rhythm Society 07/2009; 6(10):1501-3. · 4.56 Impact Factor
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Heart Rhythm 04/2007; 4(4):508-11. · 4.10 Impact Factor
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Heart Rhythm 01/2006; 2(12):1365-8. · 4.10 Impact Factor
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Ayten Erol-Yilmaz,
Hein J Verberne,
Tim A Schrama,
Jana Hrudova,
Robbert J De Winter,
Berthe L F Van Eck-Smit,
Rianne De Bruin,
Jeroen J Bax,
Martin J Schalij, Arthur A Wilde,
Raymond Tukkie
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ABSTRACT: The aim of this article is to examine whether cardiac resynchronization therapy (CRT) induces improvements in the neurohumoral system.
Thirteen patients with HF (left ventricular (LV) ejection fraction <35%) were included. Before and after 6 months of CRT, myocardial (123)I-metaiodobenzylguanidine ((123)I-MIBG) uptake indices, used as an index of neural norepinephrine reuptake and retention, and brain natriuretic peptide (BNP) levels, used as an index of LV end-diastolic pressure, NYHA classification and echocardiographic indices were assessed. Six months of CRT resulted in significant improvement in (1) NYHA classification and reduction in QRS width (P < 0.001), (2) decrease of LV end-diastolic diameter (P = 0.005), LV end-systolic diameter (P = 0.005), septal to lateral delay (P = 0.01) and mitral regurgitation (MR, P = 0.04), (3) delayed (123)I-MIBG heart/mediastinum ratios improved (P = 0.03) and (123)I-MIBG washout decreased (P = 0.001), and (4) BNP levels decreased (P = 0.001).
Parallel to significant functional improvement and echocardiographic reverse remodeling and resynchronization, our data indicate that CRT induces favorable changes in the neurohumoral system.
Pacing and Clinical Electrophysiology 04/2005; 28(4):304-10. · 1.35 Impact Factor
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ABSTRACT: Programmable pacemaker sensor features are frequently used in default setting. Limited data are available about the effect of sensor optimization on exercise capacity and quality of life (QOL). Influence of individual optimization of sensors on QOL and exercise tolerance was investigated in a randomized, single blind study in patients with VVIR, DDDR, or AAIR pacemakers.
Patients with > or =75% pacing were randomized to optimized sensor settings (OSS) or default sensor setting (DSS). Standardized optimization was performed using three different exercise tests. QOL questionnaires (QOL-q: Hacettepe, Karolinska, and RAND-36) were used for evaluation of the sensor optimization. One month before and after optimization, exercise capacity using chronotropic assessment exercise protocol and the three QOL-q were assessed.
Fifty-four patients (26 male, 28 female) with a mean age of 65 +/- 16 years were enrolled in the study. In each group (OSS and DSS) 27 patients were included. One month after sensor optimization, the achieved maximal heart rate (HR) and metabolic workload (METS) were significantly higher in OSS when compared with DSS (124 +/- 28 bpm vs 108 +/- 20 bpm, P = 0.036; 7.3 +/- 4 METS vs 4.9 +/- 4 METS, P = 0.045). Highest HR and METS were achieved in patients with pacemakers with accessible sensor algorithms. In patients with automatic slope settings (33%), exercise capacity did not improve after sensor optimization. QOL did not improve in OSS compared with DSS.
After 1 month of individual optimization of rate response pacemakers, exercise capacity was improved and maximum HR increased, although QOL remained unchanged. Accessible pacemaker sensor algorithms are mandatory for individual optimization.
Pacing and Clinical Electrophysiology 02/2005; 28(1):17-24. · 1.35 Impact Factor
