News
May 9, 2018

Interesting publications 2018 continues


 

....

One of the focus areas at Center for Cardiological Innovation is research on arrhythmogenic cardiomyopathy (AC), a severe disease that might lead to life-threatening arrhythmias. The emergence of family genetic screening of patients with AC has lead to improved patient care, but there are still many challenges that needs to be investigated.

Below you can read an overview of the following articles:

- Harmful Effects of Exercise Intensity and Exercise Duration in Patients With Arrhythmogenic Cardiomyopathy
- Reveal the Concealed: The Quest For Early Disease Detection In Family Members At Risk Of Developing Arrhythmogenic Cardiomyopathy.

- Use of leadless pacemakers in Europe: results of the European Heart Rhythm Association survey.
- Heart transplantation in arrhythmogenic right ventricular cardiomyopathy - Experience from the Nordic ARVC Registry.
- Mean Right Atrial Pressure for Estimation of Left Ventricular Filling Pressure in Patients with Normal Left Ventricular Ejection Fraction: Invasive and Noninvasive Validation.

Category: General
Posted by: Piritta

Lie ØH, Dejgaard LA, Saberniak J, Rootwelt C, Stokke MK, Edvardsen T, Haugaa KH
Harmful Effects of Exercise Intensity and Exercise Duration in Patients With Arrhythmogenic Cardiomyopathy
JACC Clin Electrophysiol 2018 March 28. [Epub ahead of print]

Abstract

Objectives: The goal of this study was to explore the association between exercise duration versus exercise intensity and adverse outcome in patients with arrhythmogenic cardiomyopathy (AC).

Background: Vigorous exercise aggravates and accelerates AC, but there are no data assessing the harmful effects of exercise intensity and duration in these patients.

Methods: Exercise habits at time of diagnosis were recorded by standardized interviews in consecutive AC patients. Exercise >6 metabolic equivalents was defined as high intensity, and exercise duration was categorized as long if above median. Life-threatening ventricular arrhythmia (VA) was defined as aborted cardiac arrest, documented sustained ventricular tachycardia, ventricular fibrillation, or appropriate implantable cardioverter-defibrillator therapy.

Results: We included 173 AC patients (53% probands; 44% female; 41 ± 16 years of age). Median weekly exercise duration was 2.5 h (interquartile range: 2.0 to 5.5 h), and 91 patients (52%) reported high-intensity exercise. VA had occurred in 83 patients (48%) and was more prevalent in patients with high-intensity exercise than low-intensity exercise (74% vs. 20%, p < 0.001), and more prevalent in long-duration than short-duration exercise (65% vs. 31%, p < 0.001). High-intensity exercise was a strong and independent marker of VA, even when adjusted for the interaction with long-duration exercise (odds ratio: 3.8; 95% confidence interval: 1.3 to 11.0, p < 0.001), whereas long-duration exercise was not.

Conclusions: High-intensity exercise was a strong and independent marker of life-threatening VA in AC patients, independent of exercise duration. AC patients could be advised to restrict their exercise intensity.

 

Haugaa KH, Lie ØH
Reveal the Concealed: The Quest For Early Disease Detection In Family Members At Risk Of Developing Arrhythmogenic Cardiomyopathy.
JACC Cardiovasc Imaging. 2018 Mar 12. pii: S1936-878X(18)30201-8.
doi: 10.1016/j.jcmg.2018.02.007. [Epub ahead of print] No abstract available
PMID: 29550321

Fifteen years ago, patients with arrhythmogenic cardiomyopathy (AC) were mostly probands with aborted cardiac arrest or with severe cardiac structural changes identified by cardiac imaging. In this inherited disease, the emergence of family genetic screening of patients with AC has resulted in a large group of mutation-positive family members at risk of developing AC disease. These family members are often young and healthy. With infliction of a genetic diagnosis on a young and healthy individual comes great responsibility for disease prevention. However, appropriate follow-up measures to detect early disease and to prevent life-threatening arrhythmic events and sudden cardiac death are challenging tasks. The transition of an AC mutation–positive individual from the concealed phase to a potentially life-threatening phase must not be missed. Physicians managing families with AC are focused on the correct timing of primary preventive insertion of an implantable cardioverter-defibrillator to avoid the catastrophe. However, recommendations for follow-up intervals in family members of patients with AC are not clearly defined, and they are definitely not individualized, thus making this follow-up demanding and often using a physician’s experience and intuition as its basis.

 

Haugaa KH, Dejgaard LA
Global Longitudinal Strain: Ready for Clinical Use and Guideline Implementation.
J Am Coll Cardiol. 2018 May 8;71(18):1958-1959. doi: 10.1016/j.jacc.2018.03.015.
PMID: 29724347

Cardiac function is a powerful prognostic marker. A great number of clinical decisions are based on the performance of the left ventricle, such as medication for heart failure (HF), primary prevention implantable cardiac defibrillator treatment, and timing of valve interventions, among others. The method for assessing left ventricular function has long been synonymous with the left ventricular ejection fraction (EF), a term with >70,000 hits on PubMed. Other methods to evaluate left ventricular function were introduced during the past years, but were most often discarded. Myocardial strain imaging was introduced in the 1990s and this method has persisted, although its path to acceptance by the medical community has been thorny 1 ;  2. Global longitudinal strain (GLS) has emerged as a fine-tuned, highly reproducible, and operator-friendly method for quantification of left ventricular function and prognostication in a wide spectrum of cardiac diseases (3). Long ago accepted in the cardiac imaging research community and headlining numerous conferences over the last decade, the way toward guideline implementation of GLS moves slowly ahead. Skepticism against the specifics of the underlying technology (vendor secrecy) and potential vendor differences is legitimate (4), but seems to overshadow the growing pile of evidence for the clinical use and added value of GLS beyond EF.

 

Boveda S, Lenarczyk R, Haugaa KH, Iliodromitis K, Finlay M, Lane D, Prinzen FW, Dagres N
Use of leadless pacemakers in Europe: results of the European Heart Rhythm Association survey.
Europace. 2018 Mar 1;20(3):555-559. doi: 10.1093/europace/eux381.
PMID: 29360974

Abstract

The purpose of this European Heart Rhythm Association (EHRA) survey is to provide an overview of the current use of leadless pacemakers (LLPM) across a broad range of European centres. An online questionnaire was sent to centres participating in the EHRA Electrophysiology Research Network. Questions dealt with standards of care and policies used for patient management, indications, and techniques of implantation of LLPM. In total, 52 centres participated in the survey. Most (86%) reported using LLPM, although 82% of these centres implanted <30 LLPM devices during the last 12 months. Non-availability (36%), lack of reimbursement (55%), and cost of the device (91%) were factors limiting the use of LLPM. The most commonly reported indications for LLPM were permanent atrial fibrillation (83%), a history of complicated conventional pacemaker (87%), or an anticipated difficult vascular access (91%). Implantation of LLPM is perceived as an easy-to-do and safe procedure by most implanters (64%), while difficult or risky in 28%, and comparable to conventional pacemakers by only a few (8%). Local vascular complications were the most frequently reported major problems (28%), but a significant number of respondents (36%) have never encountered any issue after LLPM implantation. Although cost and reimbursement issues strongly influence the use of LLPM, most respondents (72%) anticipate a significant increase in device utilization within next 2 years.

Proportion of respondents to the question: ‘How many patients were equipped with a LLPM during the last 12 months in your centre?’ Each bar represents one possible answer. A, None; B, <10 patients; C, 10–29 patients; D, 30–49 patients; E, 50–100 patients; and F, >100 patients. LLPM, leadless pacemaker.

 

Gilljam T, Haugaa KH, Jensen HK, Svensson A, Bundgaard H, Hansen J, Dellgren G, Gustafsson F, Eiskjær H, Andreassen AK, Sjögren J, Edvardsen T, Holst AG, Svendsen JH, Platonov PG
Heart transplantation in arrhythmogenic right ventricular cardiomyopathy - Experience from the Nordic ARVC Registry.
Int J Cardiol. 2018 Jan 1;250:201-206. doi: 10.1016/j.ijcard.2017.10.076. Epub 2017 Oct 21.
PMID: 29107359

Abstract

Objective: There is a paucity of data on heart transplantation (HTx) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC), and specific recommendations on indications for listing ARVC patients for HTx are lacking. In order to delineate features pertinent to HTx assessment, we explored the pre-HTx characteristics and clinical history in a cohort of ARVC patients who received heart transplants.

Methods: Data from 31 ARVC/HTx patients enrolled in the Nordic ARVC Registry, transplanted between 1988 and 2014 at a median age of 46 years (14–65), were compared with data from 152 non-transplanted probands with Definite ARVC according to 2010 Task Force Criteria from the same registry.

Results: The HTx patients were younger at presentation, median 31 vs. 38 years (p = 0.001). There was no difference in arrhythmia-related events. The indication for HTx was heart failure in 28 patients (90%) and ventricular arrhythmias in 3 patients (10%). During median follow-up of 4.9 years (0.04–28), there was one early death and two late deaths. Survival was 91% at 5 years after HTx. Age at first symptoms under 35 years independently predicted HTx in our cohort (OR = 7.59, 95% CI 2.69–21.39, p < 0.001).

Conclusion: HTx in patients with ARVC is performed predominantly due to heart failure. This suggests that current 2016 International Society for Heart and Lung Transplantation heart transplant listing recommendations for other cardiomyopathies could be applicable in many cases when taking into account the haemodynamic consequences of right ventricular failure in conjunction with ventricular arrhythmia.

Time from 1st ARVC symptom to HTx and post-HTx time in individual patients. † Deceased patients.

 

Nagueh SF, Smiseth OA, Dokainish H, Andersen OS, Abudiab MM, Schutt RC, Kumar A, Gude E, Sato K, Harb SC, Klein AL
Mean Right Atrial Pressure for Estimation of Left Ventricular Filling Pressure in Patients with Normal Left Ventricular Ejection Fraction: Invasive and Noninvasive Validation.
J Am Soc Echocardiogr. 2018 Mar 23. pii: S0894-7317(18)30070-1. doi: 10.1016/j.echo.2018.01.025. [Epub ahead of print]
PMID: 29580694

Highlights:

- Mean RAP provides useful information about mean PCWP in patients with normal LV EF.
- RAP may be used to draw conclusions about PCWP if one of three variables is missing.
- RAP may be an inaccurate measure of PCWP with predominant RV disease, severe TR, or MR.

Background: There is a paucity of data on the utility of right atrial pressure (RAP) for estimating pulmonary capillary wedge pressure (PCWP) in patients with normal ejection fraction (EF), including patients with heart failure with preserved EF.

Methods: Mean RAP was compared with PCWP in 129 patients (mean age, 61 ± 11 years; 45% men) with exertional dyspnea enrolled in a multicenter study. Measurements included left ventricular volumes, EF, and mitral inflow velocities.

Results: Mean PCWP was 14 ± 7 mm Hg, and mean RAP was 8 ± 5 mm Hg. A significant relation was present between mean RAP and mean PCWP (r2 = 0.5, P < .001). RAP > 8 mm Hg had 76% sensitivity and 86% specificity in detecting mean PCWP > 12 mm Hg. In 101 patients with inconclusive mitral filling pattern (defined according to American Society of Echocardiography/European Association of Cardiovascular Imaging 2016 diastolic function recommendations), RAP by catheterization had sensitivity of 73% and specificity of 91%. In a subset of 59 patients with echocardiographic assessment of mean RAP, RAP by echocardiography had sensitivity of 76% and specificity of 89%.

Conclusions: Mean RAP provides useful information about mean PCWP in many patients with normal left ventricular EF. There is good sensitivity and excellent specificity when combining invasive or noninvasive RAP and mitral velocities to determine if PCWP is elevated.

Regression plot of mean RAP versus mean PCWP in all 129 patients.

Heart SFI