Association of endomyocardial fibrosis and minor myocarditis sequelae with intracardiac thrombus and Ebstein like valvulopathy in a patient with Behçet disease: a case report.

Background: Cardiac complications occur in 1-6% of cases of Behçet disease (BD) with intracardiac thrombus being the most frequent complication. Endomyocardial fibrosis, less common and occasionally associated with intracardiac thrombus, is reported in <20 case reports of BD, among which, three cases are described to mimic Ebstein disease based on echocardiography. We present the first case in the literature of a 34-year-old man with BD diagnosed with multiple cardiovascular complications, highlighting the challenging diagnosis and treatment of this pathology, especially regarding anticoagulation therapy. Case summary: A 34-year-old man, diagnosed with BD, presented to the Emergency Room with haemoptysis. Computed tomography study of the thorax diagnosed pulmonary arterial aneurysm with multiple arterial thrombi, associated with multiple intracardiac thrombi in the right ventricle and atrium. The echocardiography confirmed the presence of voluminous thrombi in the right ventricle and atrium and showed hypertrabeculation of the right ventricle and a high insertion of the posterior leaflet of the tricuspid valve inducing a moderate tricuspid insufficiency compatible with an Ebstein disease. The cardiac MRI later revealed right ventricular fibrosis consistent with endomyocardial fibrosis and sequelae of myocarditis, also described as BD rare cardiac manifestations. The patient had a favourable outcome under anticoagulant treatment and immunosuppressive drugs. Discussion: The association of multiple cardiovascular complications can occur in a single patient with BD. The endomyocardial fibrosis in the right heart chambers acting as a substrate for thrombus formation and subsequent pulmonary embolism; fibrosis extending to the tricuspid valve inducing an Ebstein-like morphology.

Dobutamine stress echocardiography in patients with moderate coronary artery disease detected by coronary computed tomography angiography could reduce the rate of unnecessary coronary angiography.

AIMS: To test the hypothesis that dobutamine stress echocardiography (DSE) reduces the rate of unnecessary invasive coronary angiography (CA) in patients with chronic stable coronary artery disease (CAD) and moderate to severe stenosis detected by coronary computed tomography angiography (CCTA). METHODS: This study included 49 consecutive, symptomatic CAD patients with coronary lesions greater than 50% detected by CCTA who underwent all DSE and a CA with pressure wire evaluation and FFR measurement. The DSE operators was aware of the CCTA results, but invasive physicians were blinded to DSE results. The primary endpoint was the negative predictive value of a CCTA followed by a DSE test for detecting significant coronary artery disease (CAD). This was defined by the presence of significant coronary lesions (>90% stenosis) or moderate coronary lesions (50-90%) with abnormal FFR value of less than 0.80 evaluated by invasive angiogram (CA). Secondary endpoints included major adverse cardiovascular events (MACEs). RESULTS: In patients with abnormal CCTA followed by CA, 33 patients (67.34%) had non-significant CAD lesions. In patients with both abnormal CCTA and DSE only 6 patients (12.24%) presented non-significant CAD. The negative predictive value of a CCTA followed by a DSE was significantly increased to 92.5%, when compared with CCTA alone. Thus DSE on top of abnormal CCTA could reduce unnecessary CA by 5.5 fold. During follow-up (mean 38.75 ± 12.25 months) 1 (2.1%) patient had a cardiac sudden death, 3 (6.12%) patients had an unplanned myocardial revascularization and 1 (2.1%) patient had a stroke, none of which occurred in patients with normal DSE. No patients experienced a myocardial infarction or needed un unplanned surgical revascularization. CONCLUSIONS: The addition of DSE in case of abnormal CCTA increases significantly the negative predictive value for detecting significant CAD in need for revascularisation and thus reduces markedly the number of unnecessary CA. This diagnostic strategy has a higher diagnostic accuracy and negative predictive value to the opposite approach where an abnormal CCTA mandates a CA without additional functional testing.

Publisher Correction: Accurate Detection of Dobutamine-induced Haemodynamic Changes by Kino-Cardiography: A Randomised Double-Blind Placebo-Controlled Validation Study.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Accurate Detection of Dobutamine-induced Haemodynamic Changes by Kino-Cardiography: A Randomised Double-Blind Placebo-Controlled Validation Study.

Non-invasive remote detection of cardiac and blood displacements is an important topic in cardiac telemedicine. Here we propose kino-cardiography (KCG), a non-invasive technique based on measurement of body vibrations produced by myocardial contraction and blood flow through the cardiac chambers and major vessels. KCG is based on ballistocardiography and measures 12 degrees-of-freedom (DOF) of body motion. We tested the hypothesis that KCG reliably assesses dobutamine-induced haemodynamic changes in healthy subjects. Using a randomized double-blinded placebo-controlled crossover study design, dobutamine and placebo were infused to 34 volunteers (25 ± 2 years, BMI 22 ± 2 kg/m², 18 females). Baseline recordings were followed by 3 sessions of increasing doses of dobutamine (5, 10, 20 µg/kg.min) or saline solution. During each session, stroke volume (SV) and cardiac output (CO) were determined by echocardiography and followed by a 90 s KCG recording. Measured linear accelerations and angular velocities were used to compute total Kinetic energy (iK) and power (Pmax). KCG sorted dobutamine infusion vs. placebo with 96.9% accuracy. Increases in SV and CO were correlated to iK (r = +0.71 and r = +0.8, respectively, p < 0.0001). Kino-cardiography, with 12-DOF, allows detecting dobutamine-induced haemodynamic changes with a high accuracy and present a major improvement over single axis ballistocardiography or seismocardiography.