Apr 24, 2017

Recent publications



Results from several recent studies have been published in international scientific journals this year, including an expert consensus document on multi-modality imaging approaches in arrhythmogenic cardiomyopathies.

Below is a short overview of the following articles;
- Comprehensive multi-modality imaging approach in arrhythmogenic cardiomyopathy-an expert consensus document of the European Association of Cardiovascular Imaging
- Echocardiographic comparison between left ventricular non-compaction and hypertrophic cardiomyopathy
- Soluble ST2 is associated with disease severity in arrhythmogenic right ventricular cardiomyopathy
- A retrospective analysis of cardiovascular outcomes in patients treated with ASV

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Haugaa KH, Basso C, Badano LP, Bucciarelli-Ducci C, Cardim N, Gaemperli O, Galderisi M, Habib G, Knuuti J, Lancellotti P, McKenna W, Neglia D, Popescu BA, Edvardsen T
Comprehensive multi-modality imaging approach in arrhythmogenic cardiomyopathy-an expert consensus document of the European Association of Cardiovascular Imaging.
Eur Heart J Cardiovasc Imaging. 2017 Jan 9. pii: jew229. doi: 10.1093/ehjci/jew229. [Epub ahead of print]
PMID: 28069601

Arrhythmogenic cardiomyopathy (AC) is a progressive disease with high risk of life-threatening ventricular arrhythmias. A genetic mutation is found in up to 50–60% of probands, mostly affecting desmosomal genes. Diagnosis of AC is made by a combination of data from different modalities including imaging, electrocardiogram, Holter monitoring, family history, genetic testing, and tissue properties. Being a progressive cardiomyopathy, repeated cardiac imaging is needed in AC patients. Repeated imaging is important also for risk assessment of ventricular arrhythmias. This expert consensus document gives clinical recommendations for how to use multi-modality imaging in the different aspects of AC disease, including diagnosis, family screening, follow-up, risk assessment, and differential diagnosis.


Haland TF, Saberniak J, Leren IS, Edvardsen T, Haugaa KH
Echocardiographic comparison between left ventricular non-compaction and hypertrophic cardiomyopathy.
Int J Cardiol. 2017 Feb 1;228:900-905. doi: 10.1016/j.ijcard.2016.11.162
PMID: 27894062


Modern imaging technology has improved detection of left ventricular non-compaction cardiomyopathy (LVNC). Hypertrophic cardiomyopathy (HCM) shares morphological features with LVNC, but prognosis and treatment strategies differ between LVNC and HCM.

Left ventricular non-compaction cardiomyopathy (LVNC) is a rare condition with high morbidity and mortality due to malignant arrhythmias, systemic thrombotic embolism and heart failure. Both sporadic and familial forms of LVNC have been described. In familial disease, LVNC is a genetically heterogeneous disorder and shares genetic mutations with hypertrophic cardiomyopathy (HCM), including mutations in genes encoding for sarcomere proteins. LVNC is diagnosed with increasing frequency which may be due to higher awareness and more sensitive diagnostic tools. The use of modern ultrasound technology and cardiac magnetic resonance (CMR) has increased the detection of morphological features of LVNC.

Strain echocardiography can assess regional LV function, and be helpful in differentiating between cardiomyopathies and reveal changes in myocardial function, also when ejection fraction (EF) is relatively preserved. The purpose of this study was to compare echocardiographic parameters in LVNC and HCM patients and to investigate regional cardiac function. We hypothesized that apical function is reduced in LVNC due to the embryonic uncompleted process of compaction.


Increased number of trabeculations, thinner MWT and lower EF were characteristics of LVNC. Myocardial function was homogeneously reduced in LVNC, while an apical to basal gradient with relatively preserved apical function was present in HCM. These characteristics may help to discriminate between LVNC and HCM.



Broch K, Leren IS, Saberniak J, Ueland T, Edvardsen T, Gullestad L, Haugaa KH
Soluble ST2 is associated with disease severity in arrhythmogenic right ventricular cardiomyopathy.
Biomarkers. 2017 Jan 24:1-8. doi: 10.1080/1354750X.2016.1278266. [Epub ahead of print]
PMID: 28067540

Diagnostic and prognostic evaluation remains challenging in arrhythmogenic right ventricular cardiomyopathy (ARVC). Soluble ST2 (sST2) is a decoy receptor for interleukin-33 (IL-33). Soluble ST2 reflects hemodynamic stress in non-ischemic heart failure, and plasma sST2 may mirror not only left ventricular function, but also the degree of right ventricular failure. We measured plasma concentration of soluble ST2 (sST2) and assessed its association with myocardial function and ventricular arrhythmias in patients with ARVC.

We included patients with ARVC and genotype positive relatives.

Soluble ST2 was determined by ELISA (enzyme-linked immunosorbent assay). We assessed myocardial function by echocardiography including strain by speckle tracking technique. The study concluded that soluble ST2 was associated with both right ventricular and left ventricular function in patients with ARVC. Soluble ST2 may aid in the determination of disease severity in ARVC.


Figure 1. Associations between soluble ST2 and cardiac function. Scatter plots depicting associations between logarithmically transformed soluble ST2 (lnsST2) and indices of left and right ventricular function. Panel A shows the association with left ventricular ejection fraction (LVEF); panel B the association with left ventricular global longitudinal strain (LV GLS); panel C the association with tricuspid annular plane systolic excursion (TAPSE); and panel D the association with right ventricular (RV) strain. From Broch K et al, Biomarkers 2017 Jan 24:1-8 [Epub ahead of print].

Hetland A, Haugaa KH, Vistnes M, Liland KH, Olseng M, Jacobsen MB, Edvardsen T
A retrospective analysis of cardiovascular outcomes in patients treated with ASV.
Scand Cardiovasc J. 2017 Apr;51(2):106-113. doi: 10.1080/14017431.2016.1262546
PMID: 27854123


Sleep disordered breathing (SDB) is common among patients with chronic heart failure (CHF). The presence of severe SDB in CHF patients, in particular central sleep apnea (CSA), also clinically known as Cheyne–Stokes respiration (CSR), includes a significant increased risk of death, independent of other established risk factors. CSR is a disorder characterized by recurrent central apneas during sleep alternating with a crescendo–decrescendo pattern of tidal volume. CSR is linked to chronic heart failure (CHF) and appears to be a marker for the severity of CHF. Continuous positive airway pressure (CPAP) has been found to improve cardiac function. Based on the results from our small prospective, randomized and controlled 3 months results we retrospectively wanted to explore the cardiovascular effects of long-term use of ASV treatment on mortality and hospital admissions in CHF patients with CSR on our outpatient clinic in an observational study. We hypothesized that 18 months treatment with ASV would decrease mortality or hospital readmission rates for these patients, based on our earlier findings.


Cardiovascular mortality or combined cardiovascular mortality or hospital readmissions did not decrease significantly during 18 months of ASV treatment in CHF patients with CSR, but the latter showed a trend toward better outcome for the ASV treatment group. Sleep parameters improved considerably after only 1 week of ASV use. Patients receiving ASV treatment experienced fewer hospital days due to heart-related conditions. We could not confirm that ASV treatment affected mortality significantly.

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