Markku Kupari

A reduction of intrathoracic pressure during the Mueller manoeuvre (MM) has been shown to decrease left ventricular (LV) forward stroke volume in patients with systolic heart failure. To assess the underlying mechanisms, we analyzed MM‐induced changes in LV inflow and outflow dynamics in 13 healthy individuals (mean age 42 y, 8 men) and in 13 patients with dilated cardiomyopathy (DCM) (mean age 44 y, 8 men) having LV ejection fraction 10 s. Doppler echocardiography was used to record systolic and diastolic flow velocities at the aortic and mitral valve annulus, respectively, during quiet respiration and the MM. The respective flow velocity‐time integrals (VTI) were measured. Compared with control subjects, patients with DCM had a larger MM‐induced decrease in the aortic VTI (−4.2 ± 0.3 vs. −1.6 ± 0.5 cm; p < 0.001) combined with a tendency to a smaller decrease in the mitral VTI (−1.4 ± 0.5 vs. –2.2 ± 0.3 cm; p = 0.070). The mitral‐to‐aortic VTI ratio, an indirect mitral regurgitant index, increased in DCM from 0.78 ± 0.04 to 1.03 ± 0.08 ( p = 0.006) but decreased in the control group from 0.70 ± 0.03 to 0.61 ± 0.03 ( p = 0.025). The group x MM interaction was statistically highly significant ( F = 16.8, p < 0.001). We conclude that, in DCM‐related heart failure, a reduction of intrathoracic pressure during the MM increases the mitral‐to‐aortic VTI ratio suggesting appearance of functional mitral regurgitation.

Background: There is no universal agreement on the diagnostic criteria for cardiac sarcoidosis (CS). In 2024, the American Heart Association (AHA) introduced a new diagnostic scheme featuring a quaternary likelihood stratification for CS instead of dichotomy. Its clinical implications are unknown. Methods: We analyzed 252 patients with CS and proven histology (mean age, 49 years; 61% women). Following the AHA diagnostic stratification, CS was retrospectively considered definite given sarcoid granulomas on myocardial biopsy (n=109), highly probable with both clinical and cardiac imaging findings being CS consistent (n=93), and probable with either clinical or imaging findings alone being CS consistent (n=50). The groups were compared for clinical characteristics and the composite incidence of death, transplantation, implantation of a left ventricular assist device, and ventricular tachyarrhythmia on follow‐up (median, 5.0 years). Results: In AHA definite, highly probable, and probable CS: 94%, 99%, and 22% of patients ( P P P P P P P =0.201). Conclusions: The 2024 AHA stratification of CS likelihood distinguishes groups with differences in clinical characteristics but did not have independent prognostic value in our statistical analysis.

BACKGROUND: Cardiac sarcoidosis involves a significant but difficult-to-define risk of sudden cardiac death (SCD). Current guidelines recommend consideration of an implantable cardioverter defibrillator for patients with extensive or significant myocardial late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging. However, extensive/significant LGE is not defined. METHODS: A nationwide cardiac sarcoidosis registry was screened for patients entered before 2020 with cardiac magnetic resonance imaging done before or P <0.001), with receiver operating characteristic analyses identifying LGE mass ≥9.9% and ≥6 LGE segments as discriminative thresholds. At presentation, 70 patients were free of class I and class IIa implantable cardioverter defibrillator indications unrelated to LGE. Their 5-year rate of SCD/VT was 6.3% (0.0–14.8%) with LGE mass <9.9% versus 21.5% (6.5–36.6%) with higher LGE mass, and 6.9% (0.0–16.3%) with <6 LGE segments versus 20.5% (5.9–35.2%) with ≥6 segments. CONCLUSIONS: In cardiac sarcoidosis, myocardial LGE making up ≥9.9% of LV mass or affecting ≥6 LV segments may suggest prognostically significant LV involvement and a high risk of SCD. However, prospective validation of the thresholds is needed.

Cardiac sarcoidosis (CS) results from epithelioid cell granulomas infiltrating the myocardium and predisposing to conduction disturbances, ventricular tachyarrhythmias, and heart failure. Manifest CS, however, constitutes only the top of an iceberg as advanced imaging uncovers cardiac involvement 4 to 5 times more commonly than what is clinically detectable. Definite diagnosis of CS requires myocardial biopsy and histopathology, but a sufficient diagnostic likelihood can be achieved by combining extracardiac histology of sarcoidosis with clinical manifestations and findings on cardiac imaging. CS can appear as the first or only organ manifestation of sarcoidosis or on top of pre-existing extracardiac disease. Due to the lack of controlled trials, the care of CS is based on observational evidence of low quality. Currently, the treatment involves corticosteroid-based, tiered immunosuppression to control myocardial inflammation with medical and device-based therapy for symptomatic atrioventricular block, ventricular tachyarrhythmias, and heart failure. Recent outcome data indicate 90% to 96% 5-year survival in manifest CS with the 10-year figures ranging from 80% to 90%. Major progress in the care of CS awaits the key to its molecular–genetic pathogenesis and large-scale controlled clinical trials.

Aims: Giant cell myocarditis (GCM) is an inflammatory cardiomyopathy akin to cardiac sarcoidosis (CS). We decided to study the findings of GCM on cardiac magnetic resonance (CMR) imaging and to compare GCM with CS.Methods and results: CMR studies of 18 GCM patients were analyzed and compared with 18 CS controls matched for age, sex, left ventricular (LV) ejection fraction and presenting cardiac manifestations. The analysts were blinded to clinical data. On admission, the duration of symptoms (median) was 0.2 months in GCM vs. 2.4 months in CS (P = 0.002), cardiac troponin T was elevated (>50 ng/L) in 16/17 patients with GCM and in 2/16 with CS (P < 0.001), their respective median plasma B-type natriuretic propeptides measuring 4488 ng/L and 1223 ng/L (P = 0.011). On CMR imaging, LV diastolic volume was smaller in GCM (177 ± 32 mL vs. 211 ± 58 mL, P = 0.014) without other volumetric or wall thickness measurements differing between the groups. Every GCM patient had multifocal late gadolinium enhancement (LGE) in a distribution indistinguishable from CS both longitudinally, circumferentially, and radially across the LV segments. LGE mass averaged 17.4 ± 6.3% of LV mass in GCM vs 25.0 ± 13.4% in CS (P = 0.037). Involvement of insertion points extending across the septum into the right ventricular wall, the “hook sign” of CS, was present in 53% of GCM and 50% of CS.Conclusion: In GCM, CMR findings are qualitatively indistinguishable from CS despite myocardial inflammation being clinically more acute and injurious. When matched for LV dysfunction and presenting features, LV size and LGE mass are smaller in GCM.