tècniques d'imatge en miocardiopaties familiars.€¦ · tècniques d'imatge en...
TRANSCRIPT
Tècniques d'imatge en miocardiopaties familiars.
Poden canviar el pronòstic?
Noves tècniques d'imatge en patologies menys freqüents: pericarditis, miocarditis, endocarditis
Albert Teis, MDGabinet d’Imatge Cardíaca
Servei de Cardiologia
• Cardiomyopathies are defined by structural and functional abnormalities of the ventricular myocardium that are unexplained by flow limiting coronary artery disease or abnormal loading conditions
• Familiars à refers to the occurrence, in more than one family member, of either the same disorder or a phenotype that is (or could be) caused by the same genetic mutation and not to acquired cardiac or systemic diseases
Tècniques d’imatge en MCH
Poden canviar el pronòstic?
Noves tècniques d'imatge en patologies menys freqüents: pericarditis, miocarditis, endocarditis
MCHIncidència
• Miocardiopatia més freqüent• Incidència 1:500 (detectada per eco)• Primera causa de mort sobtada en
joves
• 2014 ESC: HCM is defined by the presence of increased LV wall thickness that is not solely explained by abnormal loading conditions
Diagnòstic
• Adults:LVW thickness ≥15 mm in one or more LV myocardial segments (echo, RM, TC) that is not explained solely by loading conditions. (1)
First-degree relatives: ≥13 mm (1)
• Children:LVW thickness >2SD than predicted mean (1)
Septal to posterior wall ratio >1.3 (2)
Circulation 2006;113:1807-1816(1) Eur Heart J. 2014;35(39):2733-79
(2) Circulation 1973;47:225–33
MCHIncidència
• Miocardiopatia més freqüent• Incidència 1:500 (detectada per eco)• Primera causa de mort sobtada en
joves
• 2014 ESC: HCM is defined by the presence of increased LV wall thickness that is not solely explained by abnormal loading conditions
Diagnòstic
• Adults:LVW thickness ≥15 mm in one or more LV myocardial segments (echo, RM, TC) that is not explained solely by loading conditions. (1)
First-degree relatives: ≥13 mm (1)
• Children:LVW thickness >2SD than predicted mean (1)
Septal to posterior wall ratio >1.3 (2)
Circulation 2006;113:1807-1816(1) Eur Heart J. 2014;35(39):2733-79
(2) Circulation 1973;47:225–33
Circulation 1997 Jul 1;96(1):214-9.
MCH – Eco i diagnòstic
• LVH >13mm en familiars
• Sensibilitat 61% • VVP 72%
Maron M et al J Am Coll Cardiol 2009;54:220–8Moon JC at al. Heart 2004;90:645-49
50% dels casos tenen hipertròfia localitzada
Massa VE pot ser normal
Eco infra-diagnostica en 10-15%
Rickers C at al. Circulation 2005;112:855-861
La Eco no diagnostica el 6% dels casos
Familiars de HCM (56)
Controls(279) P
Criptes 70% 19% <0.001
N criptes 3 1 <0.01
Profunditat 74% 59% <0.01
JACC 2006;48(12):2518-23
N=56Familiars portadors de mutacióSense hipertròfia (LVWH <13mm)
n criptes Sensibilitat Especificitat
1 70% 88%
≥2 51% 94%
≥3 40% 99%
≥5 9% 100%
Eur Heart J – Cardiovasc Imaging 2012;13:292-297
Journal of Cardiovascular Magnetic Resonance 2015, 17:64
N=72Portadors de mutació sense LVH vs controls
LGE: 0% 10% 60% 20%
n=69
n=95
n=23 n=23
Native T1 mappingAUC 0.97
Native T1 mappingAUC 0.90
HCM
Fabry
Amiloidosi
Diagnòstic Pronòstic
Necròpsia
Fa 50a - Cath Lab
1970’s - Eco
1990’s - RM - LGE
2000’s – Test genètic
2010’s – T1 mapping
MCH - Events CV
• FE• Classe funcional• ACxFA• Tamany auricular• Patró restrictiu• Fibrosià LGE a RM
•Tissue doppler imaging and plasma BNP levels to assess the prognosis in patients with HCM. J Am Soc Echocardiogr . 2011;24:1020–5•Tissue Doppler imaging and prognosis in asymptomatic or mildly symptomatic patients with HCM. Eur Heart J Cardiovasc Imaging. 2012;33:735•2014 ESC Guidelines on diagnosis and management of HCM. Eur Heart J. 2014;35:2733–2779.•Myocardial scar visualized by CMR imaging predicts major adverse events in patients with HCM. J Am Coll Cardiol. 2010;56:875–887. •Prognostic significance of myocardial fibrosis in hypertrophic cardiomyopathy. J Am Coll Cardiol. 2010;56:867–74.
A
C
B
D
♂, 42-55a, LVH 19-21mm; AI 42-44mmNo LVOTO, sincope / TV / Mort sobtada
Adabag A t al. JACC 2008; 51(14): 1369-74
N=177Arítmies en Holter 24h95% pacients assimptomàtics
O’Hanlon et al. JACC 2010;56(11):867–74 Bruder et al. JACC 2010;56(11):875–87
All cause mortality
Cardiac mortality
SCD
Bruder et al. JACC 2010;56(11):875–87
Ismail TF, et al. Heart 2014;100:1851–1858
Cardiovascular Mortality
MCH - SCD
• Edat (anys)• Max LV thickness (mm) Eco• LA size (mm) M-Mode or 2D echo in parasternal long axis • Max LVOT gradient (mmHg)• Family History of SCD• Non-sustained VT• Unexplained syncope• Hypotension in Treadmill exercise test
2014 ESC Guidelines on diagnosis and management of HCM. Eur Heart J. 2014;35:2733–2779.http://www.doc2do.com/hcm/webHCM.html
O’Mahony C et al. European Heart Journal 2014:35:2010-2020
O’Mahony C et al. European Heart Journal 2014:35:2010-2020
Vriesendorp et al. Circ Arrhythm Electrophysiol 2015;8:829
AUC
2003 ACC / ESC 0.55
2011 ACC 0.6
2014 ESC 0.69
Ismail TF, et al. Heart 2014;100:1851–1858
Importància de la FE en la valoració del risc de mort sobtada
Briasoulis et al. Heart 2015;101:1406-11N = 3067 pacients
Briasoulis et al. Heart 2015;101:1406-11
All cause mortality
SCD – Aborted SCD
Estudi de RM Cardiovascular amb Realç Tardà
HCM - Events CV
• FE• Classe funcional• ACxFA• Tamany auricular• Patró restrictiu• Fibrosià LGE a RM
•Tissue doppler imaging and plasma BNP levels to assess the prognosis in patients with HCM. J Am Soc Echocardiogr . 2011;24:1020–5•Tissue Doppler imaging and prognosis in asymptomatic or mildly symptomatic patients with HCM. Eur Heart J Cardiovasc Imaging. 2012;33:735•2014 ESC Guidelines on diagnosis and management of HCM. Eur Heart J. 2014;35:2733–2779.•Myocardial scar visualized by CMR imaging predicts major adverse events in patients with HCM. J Am Coll Cardiol. 2010;56:875–887. •Prognostic significance of myocardial fibrosis in hypertrophic cardiomyopathy. J Am Coll Cardiol. 2010;56:867–74.
LGE – pronòstic HCMLimitacions
• Centres de referència (selecció de malalts)• Molt pocs events• Usem LGE: És una variable correcta??...
– Potser tenim altres millors marcadors de fibrosi. – Molta viariabilitat en el mètode d’anàlisi LGE: manual, FWHM, SD…. – Dificultat en trobar àrees de miocardi sense fibrosi per comparar
• Cal buscar altres mètodes? T1 mapping / GLS
• 48 HCM patients• Follow-up 42 ± 12 months• The primary endpoint composite
of SCD + VF or SVT + HF
Saito et al. European Heart Journal – Cardiovascular Imaging 2012;13:617–623
Hartlage G et al. Int J Cardiovasc Imaging 2015;31:557-565
N= 79 malalts HCMSeguiment: 22mesos (9-30 mesos)
Hartlage G et al. Int J Cardiovasc Imaging 2015;31:557-565
EndPoint: heart failure hospitalization, sustained ventricular arrhythmia, and all-cause death
Dass et al. Circ Cardiovasc Imaging. 2012;5:726-733
Circulation. 2012;126:1206-1216
Flett AS et al.Circulation. 2010;122:138-144
Tècniques d’imatge en DCM
Poden canviar el pronòstic?
Noves tècniques d'imatge en patologies menys freqüents: pericarditis, miocarditis, endocarditis
Gulati A et al. JAMA. 2013;309(9):896-908
N=472Seguiment 8 anysLGE a MRI
HR 2.43 [95% CI, 1.50-3.92]; P<0.001
HR 5.24 [95%CI, 3.15-8.72]; P<0.001
Gulati A et al. JAMA. 2013;309(9):896-908
26% de reclassificació si haguessim usat el LGE com a factor pronòstic
Kuruvilla S et al. Circ Cardiovasc Imaging. 2014;7:250-258
9 estudis1488 malalts i seguiment 30 mesos
Kuruvilla S et al. Circ Cardiovasc Imaging. 2014;7:250-258
All-causemortality
HF
SCD
Indicacions de DAI en MCP dilatada
Predictors de MS en NIDCM
OR
EEF positiu 2.49
FEVI 2.87
TVNS 2.92
Alternança ona T 4.66
Realç tardà en RM 5.32
Fragmentació QRS 6.73
Priori SG. ESC Guidelines. Eur Heart J.2015 Nov 1;36(41):2793-867Goldberger et al. JACC 2014;63:1879-1889Kuruvilla S et al. Circ Cardiovasc Imaging 2014;7:250-258
210 pacients DCMSeguiment 5.3 anys
Mort Cardíaca + trasplantament + SCD
Buss S et al. European Heart Journal – Cardiovascular Imaging 2015;16:307–315
Tècniques d’imatge en DAVD
Poden canviar el pronòstic?
Noves tècniques d'imatge en patologies menys freqüents: pericarditis, miocarditis, endocarditis
Major Criteria
Minor Criteria
Marcus FI et al. Circulation 2010;121:1533-1541
Vermes E et al. JACC Imaging 2011;4:282-7
ARVC
Estratificació de risc i indicacions de DAI
• Factors de risc de MS:• Síncope inexplicat• TVNS• Història familiar de MS• Afectació extensa de VD o
afectació de VI (per RM)• Inducibilitat de TV en EEF
*
*
2015 ESC Guidelines management patients with VT and prevention of SCD. Eur Heart J 2015;36:2793-2867Lemola et al. Heart 2005;91:1167–1172
Conclusions• Per millorar el pronòstic cal millorar el diagnòstic.• El correcte diagnòstic de les mateixes farà canviar el
pronòstic conegut fins ara.• Existeix una desproporció entre les dades de
pronòstic clàssiques i tot el que podem valorar actualmentà Falta temps.
• Cal ser crític amb les guies: HCM i DCM• Prou evidència per a valorar la presència de captació
tardana de contrast com a factor pronòstic en HCM i dilatada.
• T1 mapping / ECV / Strain longitudinal global
Gràcies
Què és una miocardiopatia?• 1980, the World Health Organization (WHO): "heart muscle diseases of unknown cause”
• 1995 expanded the classification to include all diseases affecting heart muscle and to take into consideration etiology as well as the dominant pathophysiology. In this 1995 classification, the cardiomyopathies were defined as "diseases of the myocardium associated with cardiac dysfunction." They were classified according to anatomy and physiology into the following types, each of which has multiple different causes:
– Dilated cardiomyopathy (DCM)– Hypertrophic cardiomyopathy (HCM)– Restrictive cardiomyopathy (RCM)– Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D)– Unclassified cardiomyopathies
Etiologies include genetic, inflammatory, metabolic, toxic, and other diseases.The 1995 WHO/ISFC classification system included ischemic, valvular, and hypertensive disease among the causes of cardiomyopathy.
Què és una miocardiopatia?
• 2006 AHA: "Cardiomyopathies are a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilation and are due to a variety of causes that frequently are genetic. Cardiomyopathies either are confined to the heart or are a part of generalized systemic disorders, often leading to cardiovascular death or progressive heart failure-related disability"
• Primary cardiomyopathies (predominantly involving the heart)• Secondary cardiomyopathies (accompanied by other organ system involvement)
Mordi I at al. Eur Heart J Cardiovasc Imaging. 2015 Sep 10