Ludivine Eliahou

Circadian rhythm of blood pressure reflects the severity of cardiac impairment in familial amyloid polyneuropathy

BACKGROUND Cardiac amyloidosis due to familial amyloid polyneuropathy (FAP) includes restrictive cardiomyopathy, thickened cardiac walls, conduction disorders and cardiac denervation. Impaired blood pressure variability has been documented in FAP related to the Val30Met mutation. AIMS To document blood pressure variability in FAP patients with various mutation types and its relationship to the severity of cardiac involvement. METHODS Blood pressure variability was analysed in 49 consecutive FAP patients and was compared with a matched control population. Cardiac evaluation included echocardiography, right heart catheterization, electrophysiological study, Holter electrocardiogram and metaiodobenzylguanidine (MIBG) scintigraphy. RESULTS A non-dipping pattern was found in 80% of FAP patients and in 35% of control patients (P<0.0001); this was due to a significantly lower diurnal blood pressure in FAP patients (FAP group, 113 ± 21 mmHg; control group, 124 ± 8 mmHg; P<0.0001), whereas nocturnal blood pressures were similar. Among FAP patients, a non-dipping pattern was significantly associated with haemodynamic involvement, cardiac thickening or conduction disorders. These associations did not depend on the average blood pressure levels. Impaired blood pressure variability was more frequent and more pronounced in patients with multiple criteria for severe cardiac amyloidosis. CONCLUSION Low blood pressure variability is common in cardiac amyloidosis due to FAP. A non-dipping pattern was more frequently observed in FAP patients with haemodynamic impairment, cardiac thickening or conduction disorders. It is suggested that impairment of circadian rhythm of blood pressure reflects the severity of cardiac amyloidosis due to FAP.

Prediction of Long-Term Survival After Liver Transplantation for Familial Transthyretin Amyloidosis

Familial transthyretin amyloidosis (ATTR) is a rare, life-threatening, autosomal dominant disease involving mainly the heart and the peripheral nervous system due to a point mutation of the transthyretin ( TTR ) gene. By removing the main source of the mutated TTR , liver transplantation (LT) has

Indications of anti-inflammatory drugs in cardiac diseases.

Throughout the history of cardiology, physicians have attempted to treat cardiac inflammatory diseases in a multitude of different ways. In recent years, three major developments have confirmed the important role of antiinflammatory drugs in cardiology: the development of new, more powerful drugs, the advent of evidence-based medicine, and the decline of rheumatic disease in western countries. Thus, we aim to review the indications for anti-inflammatory drugs in pericarditis and myocarditis. The management of pericarditis has been improved following the publication of the European guidelines in 2004. Indeed, recent randomized controlled trials highlighted the role of colchicine to i) prevent and treat recurrences of acute pericarditis and ii) prevent post pericardiectomy syndrome and its complications. With regard to the management of myocarditis, significant advances have been made towards further understanding the mechanisms involved, and in the identification of its underlying causes (especially viral vs. autoimmune). In addition, cardiac MRI and endomyocardial biopsy are now used to detect rare etiologies of myocarditis, which may benefit from immunosuppressive therapy (giant cell and eosinophilic myocarditis, cardiac sarcoidosis). Although broad consensus has yet to be reached regarding the management of acute myocarditis, identifying viral vs. autoimmune myocarditis allows a tailored treatment using antiviral or immunosuppressive drugs.

Can Nuclear Imaging Techniques Predict Patient Outcome and Guide Medical Management in Hereditary Transthyretin Cardiac Amyloidosis

Purpose of ReviewNuclear imaging recently gained a key role in the diagnosis and prognostic assessment of transthyretin (TTR)-related cardiac amyloidosis. This review aims at summarizing the state-of-the art regarding the implementation of nuclear imaging in the management of hereditary mutated TTR-cardiac amyloidosis (mTTR-CA).Recent FindingsAlthough cardiac uptake of bone tracers is acknowledged as a specific marker of TTR amyloid cardiac burden, recent studies validated the implementation of bone scan in the flow chart for non-invasive diagnosis and follow-up of CA in multicenter trials. Simultaneously, cardiac denervation evidenced by MIBG scintigraphy proved to be a strong and independent prognostic marker of poor outcome in mTTR-CA.SummaryBy its unique ability to assess both amyloid burden and cardiac denervation, nuclear imaging may prove useful as part of multimodality imaging tools to trigger treatment initiation and monitoring in patients with mTTR-CA.

Multi-modality imaging in cardiac ATTR amyloidosis: agreement between echocardiography, MRI and DPD-scintigraphy

Methods Seventy seven consecutive patients with multimodality imaging evaluation (echocardiography, 1.5T MRI and 99mTc-DPD scintigraphy) to diagnose cardiac amyloidosis were identified from the database of the French National Reference Center for Amyloidosis. Patients with pacemaker or severe renal failure did not undergo cardiac MRI and were analyzed on the basis of the echocardiography and scintigraphy (n=17). Three groups were compared: patients with positive agreement to diagnose cardiac ATTR (PA-ATTR group), patients with positive agreement to exclude cardiac ATTR (PA-normal) and patients with negative agreement (NA).

Comparison of MIBG and Diphosphonate scintigraphy in cardiac involvement of aTTR-FAP

Background In familial aTTR amyloid polyneuropathy (FAP) cardiac involvement is of major prognostic value. Two approaches using radionuclide imaging proved relevant in the assessment of aTTR-related cardiac amyloidosis: detection of amyloid deposits with disphosphonates (DPD) and of sympathetic denervation with MIBG. The study aimed to compare the respective value of both approaches in patients with aTTR-FAP with suspected cardiac involvement.