The challenging diagnosis of isolated cardiac sarcoidosis

Non peer reviewe

Alkoholi ja sydän

Summary: Alcohol and the heart

External validation of the ROSC after cardiac arrest (RACA) score in a physician staffed emergency medical service system

Background: The return of spontaneous circulation (ROSC) after cardiac arrest (RACA) score may have implications as a quality indicator for the emergency medical services (EMS) system. We aimed to validate this score externally in a physician staffed urban EMS system. Methods: We conducted a retrospective cohort study. Data on resuscitation attempts from the Helsinki EMS cardiac arrest registry from 1.1.2008 to 31.12.2010 were collected and analyzed. For each attempted resuscitation the RACA score variables were collected and the score calculated. The endpoint was ROSC defined as palpable pulse over 30 s. Calibration was assessed by comparing predicted and observed ROSC rates in the whole sample, separately for shockable and non-shockable rhythm, and separately for resuscitations lead by a specialist, registrar or medical supervisor (i.e., senior paramedic). Data are presented as medians and interquartile ranges. Statistical testing included chi-square test, the Mann-Whitney U test, Hosmer-Lemeshow goodness of fit test and calculation of 95% confidence intervals (CI) for proportions. Results: A total of 680 patients were included of whom 340 attained ROSC. The RACA score was higher in patients with ROSC (0.62 [0.46-0.69] than in those without (0.46 [0.36-0.57]) (p <0.001). Observed against predicted ROSC indicated reasonable calibration overall (p = 0.30), with better calibration in patients with a shockable initial rhythm (p = 0.75) than in patients with a non-shockable rhythm (p = 0.04). There was no statistical difference between observed and predicted ROSC rates in resuscitations attended by a specialist (50% vs 53%, 95% CI 45-55) or registrar (55% vs 53%, 95% CI 48-62), but rates were lower than predicted in resuscitations lead by a medical supervisor (36% vs 49%, 95% CI 25-47). Discussion: Developing a practical severity-of-illness scoring system for out-of-hospital cardiac arrest patients would allow patient heterogeneity adjustment and measurement of quality of care in analogy to commoly used severity-of-illness-scores developed for the similar purposes for the general intensive care unit population. However, transferring RACA score to another country with different population and EMS system might affect the performance and generalizability of the score. Conclusions: This study found a good overall calibration and moderate discrimination of the RACA score in a physician staffed urban EMS system which suggests external validity of the score. Calibration was suboptimal in patients with a non-shockable rhythm which may due to a local do-not-attempt-resuscitation policy. The lower than expected overall ROSC rate in resuscitations attended by medical supervisors requires further study.Peer reviewe

Long-term outcome and its predictors in giant cell myocarditis

Non peer reviewe

Idiopathic giant cell myocarditis or cardiac sarcoidosis? A retrospective audit of a nationwide case series

Aims Cardiac sarcoidosis (CS) and giant cell myocarditis (GCM) are inflammatory cardiomyopathies sharing histopathological and clinical features. Their differentiation is difficult and susceptible of confusion and apparent mistakes. The possibility that they represent different phenotypes of a single disease has been debated. Methods and results We made a retrospective audit of 73 cases of GCM diagnosed in Finland since the late 1980s. All available histological material was reanalyzed as were other examinations pertinent to the distinction between GCM and CS. Finding granulomas in or outside the heart was considered diagnostic of CS and exclusive of GCM. Altogether 45 of the 73 cases of GCM (62%) were reclassified as CS. In all except one case, this was based on finding sarcoid granulomas that either had been originally missed (n = 29) or misinterpreted (n = 11) or were found in additional posttransplant myocardial specimens (n = 3) or samples of extracardiac tissue (n = 1) accrued over the disease course. Supporting the reclassification, patients relocated to the CS group had less heart failure at presentation (prevalence 20% vs. 46%, P = 0.017) and better 1 year transplant-free survival (82% vs. 45%, P = 0.011) than patients considered to represent true GCM. Conclusions Recognizing granulomas in or outside the heart remains a challenge for the pathologist. Given that CS and GCM are considered distinct diseases and granulomas exclusive of GCM, many cases of GCM, if thoroughly scrutinized, may need reclassification as CS. However, whether CS and GCM are truly different entities or parts of a one-disease continuum has not yet been conclusively settled.Peer reviewe

Magnetic Resonance Imaging as a Predictor of Survival Free of Life-Threatening Arrhythmias and Transplantation in Cardiac Sarcoidosis

Background-Cardiac magnetic resonance imaging has a key role in today's diagnosis of cardiac sarcoidosis. We set out to investigate whether cardiac magnetic resonance imaging also helps predict outcome in cardiac sarcoidosis. Methods and Results-Our work involved 59 patients with cardiac sarcoidosis (38 female, mean age 46 +/- 10 years) seen at our hospital since February 2004 and followed up after contrast-enhanced cardiac magnetic resonance imaging. The extent of myocardial late gadolinium enhancement (measured as percentage of left ventricular mass), the volumes and ejection fractions of the left and right ventricles, and the thickness of the basal interventricular septum were determined and analyzed for prognostic significance. By April 2015, 23 patients had reached the study's end point, consisting of a composite of cardiac death (n=3), cardiac transplantation (n=1), and occurrence of life-threatening ventricular tachyarrhythmias (n=19; ventricular fibrillation in 5 and sustained ventricular tachycardia in 14 patients). In univariate analysis, myocardial extent of late gadolinium enhancement predicted event-free survival, as did scar-like thinning (22% (third tertile) had positive and negative predictive values for serious cardiac events of 75% and 76%, respectively. Conclusions-Findings on cardiac magnetic resonance imaging and the extent of myocardial late gadolinium enhancement in particular help predict serious cardiac events in cardiac sarcoidosis.Peer reviewe

Cardiac magnetic resonance in giant cell myocarditis : a matched comparison with cardiac sarcoidosis

Peer reviewe

Restriction of lung volumes but normal function of pulmonary tissue in mulibrey nanism

Background Mulibrey nanism (MUL) is a rare growth restriction disorder with multiple organ manifestations caused by genetic defects affecting the TRIM37 protein. A perimyocardial heart disease is the most serious manifestation. Many MUL children appear to suffer from airway obstruction related to infection or exercise, prompting use of inhaled therapies. Asthma medication is continued up to adolescence or even to adulthood due to persisting of symptoms. The pulmonary pathophysiology has previously not been evaluated in any MUL cohort. Methods Thirty three finnish MUL patients (median age 20 years) were investigated with several lung function tests: spirometry with bronchodilatation test, single-breath diffusing capacity for carbon monoxide, single-breath lung volume measurements with helium dilution, and thoracic gas volume, airway resistance and specific conductance measurements with a body plethysmograph. As MUL typically affects body proportions, all variables were compared with reference values and with predicted values calculated from sitting height. Results Total lung capacity and forced vital capacity were markedly reduced (total lung capacity [TLC] and forced vital capacity [FVC], P = 12% and >= 200 mL) was found only in one patient. Conclusion MUL patients typically have volume restriction of the lungs, but function of the pulmonary tissue remains intact. Evidence of asthma in lung function testing at adult age is rare.Peer reviewe

Ischemic Heart Disease Selectively Modifies the Right Atrial Appendage Transcriptome

Background: Although many pathological changes have been associated with ischemic heart disease (IHD), molecular-level alterations specific to the ischemic myocardium and their potential to reflect disease severity or therapeutic outcome remain unclear. Currently, diagnosis occurs relatively late and evaluating disease severity is largely based on clinical symptoms, various imaging modalities, or the determination of risk factors. This study aims to identify IHD-associated signature RNAs from the atrial myocardium and evaluate their ability to reflect disease severity or cardiac surgery outcomes.Methods and Results: We collected right atrial appendage (RAA) biopsies from 40 patients with invasive coronary angiography (ICA)-positive IHD undergoing coronary artery bypass surgery and from 8 patients ICA-negative for IHD (non-IHD) undergoing valvular surgery. Following RNA sequencing, RAA transcriptomes were analyzed against 429 donors from the GTEx project without cardiac disease. The IHD transcriptome was characterized by repressed RNA expression in pathways for cell-cell contacts and mitochondrial dysfunction. Increased expressions of the CSRNP3, FUT10, SHD, NAV2-AS4, and hsa-mir-181 genes resulted in significance with the complexity of coronary artery obstructions or correlated with a functional cardiac benefit from bypass surgery.Conclusions: Our results provide an atrial myocardium-focused insight into IHD signature RNAs. The specific gene expression changes characterized here, pave the way for future disease mechanism-based identification of biomarkers for early detection and treatment of IHD.Peer reviewe

Modified Lipoprotein-Derived Lipid Particles Accumulate in Human Stenotic Aortic Valves

Peer reviewe