Active cardiac sarcoidosis: Diagnosis and adjustment of therapy with FDG PET/CT

National audienc

210 An unusual case of Mahaim fibers in hypertrophic cardiomyopathy

IntroductionThe coexistence of Mahaim fibbers (MF) and hypertrophic cardiomyopathy (HCM) is exceptional. We report an unusual case of a young female patient presenting with HCM and MF.Case-reportA 17 y/o woman was referred in 1998 for a familial HCM screening. She had no symptoms; her surface EKG revealed a preexcitation suggesting a right anteroseptal WPW syndrome. The transthoracic echocardiogram (TTE) demonstrated an asymmetric septal HCM without LV outflow tract (LVOT) obstruction. The electrophysiological study (EPS) demonstrated the presence of MF (normal AH interval, a short and fixed HV interval and atrial pacing produced AH prolongation without change of preexcitation degree, indicating fasciculo-ventricular MF).Eight years later (25 y/o) while the patient was doing well, the ECG was similar and TEE demonstrated the same aspect of asymmetric septal HCM without obstruction. Three months later the patient was pregnant and gave birth to a healthy baby. (During pregnancy: Delta wave was present and there was no LVOT obstruction). After delivery, Surprisingly there was no more preexcitation on her ECG and the TEE demonstrated now a significant LVOT obstruction with a systolic anterior motion of the mitral valve and an LVOT gradient of 36 mmHg. A second EPS was performed in 2008 and showed no more preexcitation. We speculate the hypothesis that FV fiber played in this case a similar pathogenic role as does the apical right ventricular pacing when it is used to decrease LVOT gradient in patients with HCM.ConclusionThis case deserves to be reported because the association of MF and HCM is rare (only 3 cases reported in english litterature), the disappearance of MF during pregnancy has never been reported and the hypothesis that the RV preexcitation via FV Mahaim fiber played a role in decreasing LVOT gradient in this patient with HCM

Reliability of QRS duration and morphology on surface electrocardiogram to identify ventricular dyssynchrony in patients with idiopathic dilated cardiomyopathy

International audienceUsing Fourier phase analysis of equilibrium radionuclide angiography in both ventricles, inter- and intraventricular delays were correlated with QRS morphology and duration in 103 patients with idiopathic dilated cardiomyopathy. We found that left bundle branch block on the left axis may reflect a high intra-left ventricular dyssynchrony. A precise evaluation of the dyssynchrony appears useful because a high proportion of patients with incomplete bundle branch block, left anterior hemiblock, or "normal" QRS exhibit a marked intra-LV dyssynchrony and may respond to cardiac resynchronization therapy

Ventricular Dyssynchrony and Risk Markers of Ventricular Arrhythmias in Nonischemic Dilated Cardiomyopathy:. A Study with Phase Analysis of Angioscintigraphy

International audienceBiventricular pacing is a new form of treatment for patients with dilated cardiomyopathy and ventricular dyssynchrony. Limited information is available regarding the relationship between ventricular dyssynchrony and risk markers of ventricular arrhythmias in idiopathic dilated cardiomyopathy (IDC). In 103 patients with IDC, Fourier phase analysis of both ventricles was performed from equilibrium radionuclide angiography (ERNA). The difference between the mean phase of the LV and RV was a measure of interventricular dyssynchrony, and the standard deviations of the mean phases in each ventricle measured intraventricular dyssynchrony. There were no significant differences in inter- and intraventricular dyssynchrony between patients with versus without histories of sustained VT or VF, nonsustained VT, abnormal signal-averaged ECG, or induced sustained monomorphic VT. Dyssynchrony was not related to decreased heart rate variability (HRV). LV and interventricular dyssynchrony were weakly related to QT duration and QT dispersion. During a follow-up of 27 +/- 23 months, 21 patients had major adverse cardiac events (MACE), including 7 cardiac deaths, 11 progression of heart failure leading to cardiac transplantation, and 3 sustained VT/VF. The only independent predictors of MACE were an increased standard deviation of LV mean phase (P = 0.003), a decreased HRV (standard deviation of normal-to-normal intervals, P = 0.004), and histories of previous VT/VF (P = 0.03) or nonsustained VT (P = 0.04). In conclusion, left intraventricular dyssynchrony evaluated with ERNA was an independent predictor of MACE in IDC and was not related to usual risk markers of ventricular arrhythmias. This may have implications for resynchronization therapy and/or the use of implantable cardioverter defibrillators in IDC

Cardiac amyloidosis: Updates in diagnosis and management

SummaryAmyloidosis is a severe systemic disease. Cardiac involvement may occur in the three main types of amyloidosis (acquired monoclonal light-chain, hereditary transthyretin and senile amyloidosis) and has a major impact on prognosis. Imaging the heart to characterize and detect early cardiac involvement is one of the major aims in the assessment of this disease. Electrocardiography and transthoracic echocardiography are important diagnostic and prognostic tools in patients with cardiac involvement. Cardiac magnetic resonance imaging better characterizes myocardial involvement, functional abnormalities and amyloid deposition due to its high spatial resolution. Nuclear imaging has a role in the diagnosis of transthyretin amyloid cardiomyopathy. Cardiac biomarkers are now used for risk stratification and staging of patients with light-chain systemic amyloidosis. Different types of cardiac complications may occur, including diastolic followed by systolic heart failure, atrial and/or ventricular arrhythmias, conduction disturbances, embolic events and sometimes sudden death. Senile amyloid and hereditary transthyretin amyloid cardiomyopathy have better prognoses than light-chain amyloidosis. Cardiac treatment of heart failure is usually ineffective and is often poorly tolerated because of its hypotensive and bradycardiac effects. The three main types of amyloid disease, despite their similar cardiac appearance, have specific new aetiological treatments that may change the prognosis of this disease. Cardiologists should be aware of this disease to allow early treatment

Segmental wall motion abnormalities in idiopathic dilated cardiomyopathy and their effect on prognosis

International audienceThere is considerable variability in segmental wall motion abnormalities and in the prognosis of idiopathic dilated cardiomyopathy (IDC). Radionuclide ventriculography with Fourier analysis was performed in 107 patients with angiographically proved IDC. Amplitude analysis located the wall motion abnormalities. Using phase analysis in the left and right ventricles, the interventricular delay between the mean phase of the right and left ventricles was used to assess interventricular dyssynchrony and SDs of the mean phase in each ventricle was used to assess intraventricular dyssynchrony. Hypokinesis was global in 56 patients (52%) and localized in the anteroseptal wall in 34 (32%), the inferior wall in 12 (11%), the anteroseptal and inferior walls in 2 (2%), and the lateral wall in 3 (3%). Patients with localized wall motion abnormalities had larger left ventricular (LV) end-diastolic diameters (70 +/- 9 vs 66 +/- 8 mm, p = 0.009) and lower LV ejection fractions (25 +/- 9% vs 31 +/- 12%, p = 0.005). Intraventricular dyssynchrony was lower in patients with global hypokinesis (SD of LV mean phase 67 +/- 35 vs 48 +/- 22 ms, p = 0.002). With a follow-up of 27 +/- 23 months, increased SD of the LV phase (p = 0.005), decreased right ventricular ejection fraction (p = 0.006), decreased LV ejection fraction (p = 0.04), and localized wall motion abnormality (p = 0.009) were independent predictors of cardiac death or worsening heart failure leading to heart transplantation. Thus, segmental wall motion abnormalities are frequent in IDC and are associated with severe systolic dysfunction and a worse prognosis