lesion-specific risk stratification of sudden …...2020/02/14 · sudden cardiac deaths (scd)...
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Lesion-specific Risk Stratification of
Sudden Cardiac Death and Ventricular Arrhythmias
in Adults with Congenital Heart Disease.
How to predict it?
Pastora Gallego, MD, PhDIntercenter Adult Congenital Heart Disease
Hospital Universitario Virgen del Rocio
Sevilla, Spain
On behalf of the Spanish ACHD Network Investigators
No conflict of interest to
disclose
European Heart Journal 2017
3311 consecutive ACHD FU 37608 person-years336 deathsAnnual Death Rate 0.89%
MODE OF DEATH
Sudden Cardiac Death is the leading mode of death at young age
incidence of sudden cardiac death in
adult congenital heart disease
Author Year PatientsFollow-up
person-years SCDAnnual Rate of
SCD
Silka 1998 3589 45857 41 0.089
Zomer 2012 8595 26500 51 0.192
Diller 2015 6969 70967 37 0.052
Engenligs 2016 2596 14114 55 0.389
Moore 2017 2935 85276 35 0.041
Oliver 2017 3331 37608 56 0.149
Total 28015 280322 275 0.098
sudden cardiac death and complexity
of underlying heart defect
Incidence of late SCD Simple CHD
0.04 / 1000 patient-years(1/25,000 patient-years)
Moderate CHD0.57 / 1000 patient-years(1/1,754 patient-years)
Severe CHD2.00/ 1000 patient-years
(1/500 patient-years)
Moore B. Int J Cardiol 2018 2935 CHD patients
Aged > 16 years old
35 cases of SCD
19%
19%
16%13%
11%
11%
4%
3.5% 3.5%
Eisenmenger
TGA (cc)
Fallot
LVOT lesions
Septal defects
Cianotic CHD
Fontan
Ebstein
Other
Distribution of SUDDen cardiac deaths by defect
Koyak Z. Circulation 2012
CLINICAL STUDYMultinational Case-Control Study
Surgical FU & Natural History of Inoperable Cases
N = 171 cases of presumed arrhythmic deaths
Sudden Cardiac Arrest
N=22SCD
n = 15
Resuscitated Cardiac Arrest
n = 5
Appropriate Shock
n = 2
Incidence
(2.6/1000 pts-year)10
1.2
3.7
2.11.4
0
3
6
9
12
TGA UVH CoAo Fallot Others
CHD TypesPrevalence and incidence were estimated for overall populationand for each diagnostic category.
Gallego P. Am J Cardiol 2012
POSTOPERATIVE ACHD POPULATION
936 postoperative ACHD patients
FU 8387 person-years
Systemic ventricular dysfunction
and Sudden cardiac arrest
Systemic VentricleFunction
SSVD(n=69)
Non-SSVD(n=867)
p
SCD(n & %) 16 (23%) 6 (0.7%) <0.001
All-cause death or TX(n & %) 28 (41%) 22 (2.5%) <0.001
SCD/All-cause death or TX Ratio
57% 27% <0.001
TX: Heart or Heart-Lung TransplantationSSVD: Severe systolic ventricular dysfunction
Gallego P el al. Am J Cardiol 2012
Variable ToF TGA LVOT lesions
Fontan
Clinical Age at repairPrior shuntVentriculotomy incisionTransannular patch (pre) syncope
Longer FU timeSyncopePalpitationsNYHA > II
Longer FU timeSyncope
Longer FU timeSyncopePalpitationsOlder Fontantechnique
Hemodynamic RVSP > 60 mmHgRVOT > 40 mmHgModerate-severe PRRV dilatation RV dysfunctionLV dysfunctionRVOT aneurysmSustained VT
Systemic RV dysfunctionModerate-severe TR
LVOT > 50 mmHgSignificant ARHigh LV EDP LV dysfunctionLV hypertrophy
Ventriculardysfunction
ECG QRS duration > 180 msQRS fragmentationQT dispersionNSVTAtrial arrhythmiasHR variabilityHR turbulenceLate potentials
Atrial arrhythmiasQRS duration > 140 msComplete heart block
Atrial arrhythmias
CMR RV LGE RV LGE
Electrophysiological Inducible VT NO
DISEASE-SPECIFIC STUDIES
Khairy P et al, Heart Rhythm 2014
Clase I
Clase IIA
Fisiología biventricular con fracción de eyección del VI
sistémico ≤35% yCF NYHA II o III
Tetralogía de Fallot con múltiples factores de riesgo:
• Disfunción sistólica o diastólica del VI.
•Taquicardia ventricular no sostenida.
• Duracción de QRS > 180 msg.
• Fibrosis miocárdica extensa.
• Inducible taquicardia ventricular sostenida
Clase IIB
Ventrículo único o VD sistémico con fracción de eyección
<35%, particularmente si:
• arritmias ventriculares complejas
• síncope inexplicado
• CF NYHA II o III
• Duración de QRS > 140 msg
• Insuficiencia grave de válvula AV sistémica
Fracción de eyección del ventrículo sistémico < 35% en
ausencia de síntomas (CF NYHA I) u otros factores de riesgo
conocidos
Síncope de origen desconocido con Inducible TV sostenida
o FV en EEF
Síncope y CC moderada o compleja con alta sospecha
clínica de arritmia ventricular aunque el EEF no haya podido
definir la causa
Clase III
Enfermedad vascular pulmonar avanzada o síndrome
Eisenmenger
Priory SG et al. Eur Heart J 2015
Circ Arrhythm Electrophysiol 2017
Population: 157 deceased patients and controls36% cases and 16% controls: ICD recommendations according to guidelines
Applying guidelines: 59% cases and 65% controls, unrecognized
Spanish achd network
C. Hospitalario Jaén
H. Virgen del Rocio Sevilla
H. Virgen de las Nieves, Granada
• H. Vall d’Hebron, BCN• H Clinic, BCN
• H. U. La Paz, Madrid• H. Gregorio Marañón, Madrid• H. Ramón y Cajal, Madrid• H. 12 octubre, Madrid
H. La Fe, Valencia
H. Juan Canalejo, Coruña
H. Insular Las Palmas
H. Virgen de la Salud, Toledo
H. U. Salamanca
H. Universitario Elche
muticenter study on sudden cardiac death
207 confirmed sudden cardiac deaths
2014-2020
METHODS Gallego P, et al
ESC Congress 2019
Sudden cardiac deaths (SCD) & life-threatening ventricular
arrhythmias (LTVA) by specific defect
N = 71 Lesion-specific risk stratification
Actuarial Survival free
from SCD<VA
Gallego P, et al
ESC Congress 2019
Clusters HR (95%
CI)
p
Very-low Reference –
Low 2.8 (1.0-7.7) 0.048
Moderate 16 (6-45) <0.001
Severe 68 (26-181) <0.001
MULTICENTER CASE-CONTROL STUDY ON SUDDEN CARDIAC DEATH
21 centers
• 203 cases
• 2287 controls
SPANISH NETWORK FOR RESEARCH ON ACHD
Gallego P, et al
ESC Congress 2019
Adjusted R2 = 0.89
P<0.001
Gallego P, et al
ESC Congress 2019
Lesion-Specific Risk Stratification
C-indexStudy cohort Multicenter Study
ROC area lesion-specific risk stratification
AUC 0.811
95% CI 0.77-0.85
P<0.001
AUC 0.784
95% CI 0.75-0.82
P<0.001
Appropriate
shock excluded
Ventricular
arrhythmias
excluded
Gallego P, et al
ESC Congress 2019
Risk Prediction Model for SCD(National Institute of Health Carlos III, Research Project 17/01327)
1. Merging SCD with episodes of life-threatening
ventricular arrhythmias into a composite end-point.
2. Incorporating the baseline lesion-specific risk
stratification.
3. Including easily available demographic, clinical,
ECG, and echo variables
Oliver JM, et al
Submitted
Statistical Flow Chart
Oliver JM, et al
Submitted
Multivariate Risk ModelOliver JM, et al
Submitted
Model Performance in the Validation Dataset
CALIBRATION DISCRIMINATION
NRI = 1.17
95% CI = 0.98-1.36
p < 0.001
Oliver JM, et al
Submitted
Electronic Calculator
(http://cardioim.iisgmsai.org:48080/calc/)
Oliver JM, et al
Submitted
Prospective Evaluation
Control Group
N= 2,287 pts
FU 2017-2022
RECC Dataset
N≈ 6,000 pts
FU 2019-2022Risk Model
Outcomes at 3&5 yr.
•SCD
•LTVA
MetricsAnnual Incidence <0.2%
50 events
3 yrs – N > 8,000 pts
5 yrs – N > 5,000 pts
Spanish ACHD Network Investigators
José M Oliver, Pastora Gallego, Ana González, Pablo Ávila, Andrés
Alonso, Diego García-Hamilton, Rafael Peinado, Laura Dos, Antonia
Pijuan, Joaquín Rueda, Maria José Rodríguez Puras, Rocío García
Orta, Efrén Martínez Quintana, Raquel Prieto, Tomas Datino, José
Ruiz Cantador, Beatriz Bouzas Zubeldia, Isaac Martínez Bendayán,
Blanca Gordon, Javier Cantalapiedra, Víctor González Fernández,
Aleix Olivella, Francisco Buendía, Eduardo Moreno, Juan L
Rodríguez Hernández, Juan Robledo Carmona, Marta López,
Antonio García Honrubia, María Bastos Fernández, Gemma Lacuey,
Nuria Hernandez, Luis F Valenzuela, Joaquín Cano-Nieto, Iris de la
Puerta, Marta Noris, Beatriz García Aranda, Silvia Montserrat,
Inmaculada Sánchez, Javier Bermejo, Francisco Fernández-Avilés.
Lesion-specific Risk Stratification of
Sudden Cardiac Death and Ventricular Arrhythmias
in Adults with Congenital Heart Disease.
How to predict it?
Pastora Gallego, MD, PhDIntercenter Adult Congenital Heart Disease
Hospital Universitario Virgen del Rocio
Sevilla, Spain
On behalf of the Spanish ACHD Network Investigators
No conflict of interest to
disclose