Miia Holmström

Late gadolinium enhanced cardiovascular magnetic resonance of lamin A/C gene mutation related dilated cardiomyopathy

BackgroundThe purpose of this study was to identify early features of lamin A/C gene mutation related dilated cardiomyopathy (DCM) with cardiovascular magnetic resonance (CMR). We characterise myocardial and functional findings in carriers of lamin A/C mutation to facilitate the recognition of these patients using this method. We also investigated the connection between myocardial fibrosis and conduction abnormalities.MethodsSeventeen lamin A/C mutation carriers underwent CMR. Late gadolinium enhancement (LGE) and cine images were performed to evaluate myocardial fibrosis, regional wall motion, longitudinal myocardial function, global function and volumetry of both ventricles. The location, pattern and extent of enhancement in the left ventricle (LV) myocardium were visually estimated.ResultsPatients had LV myocardial fibrosis in 88% of cases. Segmental wall motion abnormalities correlated strongly with the degree of enhancement. Myocardial enhancement was associated with conduction abnormalities. Sixty-nine percent of our asymptomatic or mildly symptomatic patients showed mild ventricular dilatation, systolic failure or both in global ventricular analysis. Decreased longitudinal systolic LV function was observed in 53% of patients.ConclusionsCardiac conduction abnormalities, mildly dilated LV and depressed systolic dysfunction are common in DCM caused by a lamin A/C gene mutation. However, other cardiac diseases may produce similar symptoms. CMR is an accurate tool to determine the typical cardiac involvement in lamin A/C cardiomyopathy and may help to initiate early treatment in this malignant familiar form of DCM.

Autologous bone marrow mononuclear cell transplantation in ischemic heart failure: A prospective, controlled, randomized, double-blind study of cell transplantation combined with coronary bypass

BACKGROUND Bone marrow mononuclear cell (BMMC) transplantation for heart failure has shown inconsistent therapeutic efficacy. METHODS We enrolled 104 ischemic heart failure patients scheduled for coronary artery bypass surgery (CABG). After 4- to 12-week pharmacotherapy optimization, 39 patients with left ventricular ejection fraction (LVEF) of ≤45% received injections of BMMC or vehicle intra-operatively into the myocardial infarction border area in a randomized, double-blind manner. RESULTS The median number of cells injected was 8.4 × 10(8) (interquartile range [IQR]: 5.2 × 10(8) to 13.5 × 10(8)). We measured LV function and myocardial scar size by magnetic resonance imaging (MRI), and viability by positron emission tomography (PET) and single-photon emission computed tomography (SPECT), pre-operatively and after 1-year follow-up. LVEF, the pre-defined primary end-point measure, improved by a median of 5.6% in the control group (IQR 0.2 to 10.1) and by 4.8% in the BMMC group (IQR -0.5 to 8.2) (p = 0.59). Wall thickening in injected segments rose by a median of 4.5% among controls (IQR -18.1 to 23.9) and by 5.5% in the BMMC group (IQR -6.6 to 26.5) (p = 0.68). Changes in viability by PET and SPECT did not differ between groups. Myocardial scar size by MRI in injected segments rose by a median of 5.1% among controls (IQR -3.3 to 10.8), but fell by 13.1% in the BMMC group (IQR -21.4 to -6.5) (p = 0.0002). CONCLUSIONS BMMC therapy combined with CABG failed to improve LV systolic function, or viability, despite reducing myocardial scar size.

Myocardial function in patients with Shwachman-Diamond syndrome: aspects to consider before stem cell transplantation.

Early studies have suggested increased risk of fatal cardiac complications in infants with Shwachman–Diamond syndrome (SDS), an inherited bone marrow failure syndrome. Patients undergoing stem cell transplantation (STC) have appeared susceptible to organ toxicity, including cardiac involvement.

Partial anomalous pulmonary venous return and atrial septal defect in adult patients detected with 128-slice multidetector computed tomography

The present series describes a group of adults with left-to-right shunts including partial anomalous pulmonary venous return (PAPVR) and/or an atrial septal defect (ASD) evaluated with ECG-gated 128-slice multidetector computed tomography (MDCT). PAPVR is defined as a left-to-right shunt where one or more, but not all, pulmonary veins drain into a systemic vein or the right atrium. PAPVR involving the right upper pulmonary vein can be associated with a sinus venosus ASD. The presence, course, number of anomalous veins and associated cardiovascular defects can be reliably observed by 128-slice MDCT angiography.

Magnetocardiographic Assessment of Healed Myocardial Infarction

Background: We evaluated the capability of multichannel magnetocardiography (MCG) to detect healed myocardial infarction (MI).

Eight-Row Multidetector Computed Tomography Coronary Angiography Evaluation of Significant Coronary Artery Disease in Patients with Severe Aortic Valve Stenosis

BackgroundThe aim of this study was to evaluate whether 8-row multidetector computed tomography coronary angiography (MDCT-CA) could replace invasive conventional coronary angiography (CCA) in patients with acquired severe aortic valve stenosis (AS). Coronary artery disease (CAD) diagnosis should be obtained with a noninvasive method in patients with AS undergoing valvular replacement. We evaluated the diagnostic accuracy of MDCT-CA in detecting high-grade (≥50%) stenoses in the main coronary arteries in patients with AS.MethodsTwenty-three patients with acquired severe AS underwent both CCA and MDCT-CA. We calculated the total and volumetric calcium scores and evaluated the image quality of each coronary segment as assessable or nonassessable for stenosis. The images of the arteries were evaluated for the occurrence of artifacts and the presence of high-grade stenoses (≥50%) by visual estimation and comparison with that of CCA.ResultsOf the 322 segments screened 224 were assessable for stenosis. Heavy calcium load rendered 37 (38%) of the 98 coronary segments nonassessable.Compared to CCA, MDCT-CA had a sensitivity of 63%, a specificity of 96%, a positive predictive value of 52%, and a negative predictive value of 98% for ≥50% stenoses in the main coronary arteries.ConclusionsEight-row MDCT-CA revealed a low sensitivity in detecting significant coronary artery disease in patients with acquired severe AS. High calcium burden decreased visualization of the lumen and complicated most often a correct assessment. In this patient group, CCA should still remain the primary pre-surgical test to rule out coronary lesions requiring revascularization.

Clinical disease presentation and ECG characteristics of LMNA mutation carriers.

Objective Mutations in the LMNA gene encoding lamins A and C of the nuclear lamina are a frequent cause of cardiomyopathy accounting for 5–8% of familial dilated cardiomyopathy (DCM). Our aim was to study disease onset, presentation and progression among LMNA mutation carriers. Methods Clinical follow-up data from 27 LMNA mutation carriers and 78 patients with idiopathic DCM without an LMNA mutation were collected. In addition, ECG data were collected and analysed systematically from 20 healthy controls. Results Kaplan-Meier analysis revealed no difference in event-free survival (death, heart transplant, resuscitation and appropriate implantable cardioverter-defibrillator therapy included as events) between LMNA mutation carriers and DCM controls (p=0.5). LMNA mutation carriers presented with atrial fibrillation at a younger age than the DCM controls (47 vs 57 years, p=0.003). Male LMNA mutation carriers presented with clinical manifestations roughly a decade earlier than females. In close follow-up non-sustained ventricular tachycardia was detected in 78% of LMNA mutation carriers. ECG signs of septal remodelling were present in 81% of the LMNA mutation carriers, 21% of the DCM controls and none of the healthy controls giving a high sensitivity and specificity for the standard ECG in distinguishing LMNA mutation carriers from patients with DCM and healthy controls. Conclusions Male LMNA mutation carriers present clinical manifestations at a younger age than females. ECG septal remodelling appears to distinguish LMNA mutation carriers from healthy controls and patients with DCM without LMNA mutations.

Temporal Analysis of the Depolarization Wave of Healed Myocardial Infarction in Body Surface Potential Mapping

Background: We studied the ability of different time segments of the depolarization wave recorded with body surface potential mapping (BSPM) to detect and localize myocardial infarction (MI).

Cardiac MRI in patients with cardiac pacemakers: practical methods for reducing susceptibility artifacts and optimizing image quality

Background Magnetic resonance imaging (MRI) of pacemaker patients has become available despite of previous contraindications. However, pacing systems containing ferromagnetic material may hamper the diagnostic quality of cardiac MR (CMR) images. Purpose To study methods for reducing susceptibility-based artifacts in CMR examinations of pacemaker patients. Material and Methods Altogether 16 patients were scanned with 1.5T MRI scanner using cine balanced steady-state free-precession (bSSFP) and spoiled gradient echo (SPGR) sequences. The use of frequency-scout was also evaluated. For myocardial late gadolinium-enhanced (LGE) imaging, SPGR or bSSFP readout inversion-recovery prepared gradient echo sequences were used with and without phase-sensitive inversion-recovery (PSIR). Two radiologists subjectively compared the image quality (IQ) and the ranges of susceptibility artifacts were evaluated objectively. Results The IQ proved adequate for diagnosing each patient, although in a few patients with a left-side implanted generator, artifacts hampered IQ in the anterior and anteroseptal segments of the myocardium in bSSFP cine and LGE sequences. In bSSFP cine, the use of frequency-scout could often transfer the banding artifacts away from the left ventricular myocardium. In LGE imaging, the artifacts were more pronounced in IR-bSSFP and PSIR than in IR-SPGR sequences. The ranges of generator-based artifacts were greater in bSSFP (10–12 cm) than in SPGR (6 cm) sequences due to banding artifacts. Conclusion The artifacts caused by pacemakers typically did not compromise the diagnostic IQ. The use of frequency-scout prior to bSSFP cine or the use of SPGR-based sequences could also improve IQ.

Wall Motion and Perfusion Analysis of Transmyocardial Laser Revascularization

Objective Objective—Transmyocardial laser revascularization (TMLR) creates channels in the myocardium. The aim of the treatment is to relieve angina in patients with end-stage coronary artery disease. We studied the effect of TMLR on myocardial function and perfusion with the combination of cine magnetic resonance Imaging (MRf) and thallium scintigraphy. Design—Eight patients with severe triple-vessel coronary artery disease were studied with MRI and thallium scintigraphy before and 6 months after laser treatment. Results—TMLR did not improve global left ventricular (LV) function or myocardial perfusion. However, systolic wall thickening deprived in segments with fixed perfusion defects in 6 months and la ertreatment prevented this deprivation (p = 0.03). In addition single Photon emission computed tomography (SPECT) Imaging indicated that TMLR prevented conversion of reversible into fixed defects. Conclusion—In severe, progressing coronary artery disease TMLR does not improve global LV function or myocardial perfusion, but it preserves systolic wall thickening in fixed defects (scar). It also prevents changes from ischemic myocardial regions to scar.