How to prevent sudden coronary death in the young CONGENITAL CORONARY ARTERY ANOMALIES Cristina Basso, MD, FESC University of Padua, Italy ESC Congress Paris August 29, 2011
DECLARATION OF CONFLICT OF INTEREST none
Coronary Artery Anomalies Prevalence Autopsy studies: 0.17% Angiographic studies: 1.2% Transthoracic 2-D echo: 0.17% The true prevalence in general population is unknown, but surely <0.2%
Congenital Coronary Artery Anomalies: Classification Ostia Sequestration Valve-like ridge Acute take-off Origin from the Pulmonary artery from the Aorta High take-off Single coronary artery Wrong sinus LCx from RCA or Right Valsalva sinus LDA from RCA Course ( myocardial bridge ) Fistulae Aneurysms
Coronary Ostia Sequestration
Valve-like Ridge
Congenital Coronary Artery Anomalies: Classification Ostia Sequestration Valve-like ridge Acute take-off Origin from the Pulmonary artery from the Aorta High take-off Single coronary artery Wrong sinus LCx from RCA or Right Valsalva sinus LDA from RCA Course ( myocardial bridge ) Fistulae Aneurysms
Origin from the Pulmonary Artery
Congenital Coronary Artery Anomalies: Classification Ostia Sequestration Valve-like ridge Acute take-off Origin from the Pulmonary artery from the Aorta High take-off Single coronary artery Wrong sinus LCx from RCA or Right Valsalva sinus LAD from RCA Course ( myocardial bridge ) Fistulae Aneurysms
High Take Off
High Take Off Purvis J et al. Heart 2010;96:1334-1334
LCx from RCA
M, 18 yrs old SD at rest F, 18 yrs old SD on emotion
Wrong Sinus Origin
SD in Athletes USA vs Italy Experience 3% 2% 2% 2% 2% 1% 1% 1% 2% 1% 2% 6% 2% 2% 2% 14% 3% 3% 3% 3% 26% 2% 12% 10% 2% 5% 7% 20% 24% 14% 20% 4% HCM Commotio Congenital CAD LV hypertrophy Myocarditis Aortic rupture ARVC Myocardial bridge AS CAD ATH DCM MVP Asthma Heat stroke Drug abuse Other cardiovascular Long QT Sarcoidosis Cerebral Pulmonary embolism Unexplained
Relative Risk of Sport-related SD 0,6 0,5 RR=2.6 (1.2-5.1)* in CAD Athletes Non-athletes RR=79.0 (10.1-3564.4)* p<.00001 p=.009 SD per 100,000 per year 0,4 0,3 0,2 0,1 0 CAD CCA Corrado et al, JACC 2003
ARRHYTHMIAS, SYNCOPE, SD Pathophysiology of SD Squeezing on effort Intramural aortic course Acute-angle take-off Transient Myocardial Ischemia Vasospasm
Surgical Unroofing Intracoronary Stenting
SD during or shortly after exercise: all Premonitory cardiac symptoms: 10 (37%) (syncope, chest pain, palpitations on effort) 12 lead ECG (available in 9): normal in all Stress test ECG (available in 6): normal in all Clinical diagnosis and sport disqualification: none
Clinical case Soccer referee, every year pre-participation screening for sport activity Dyspnea and angina during effort (training) Syncopal episode on tapis roulant Sport physician anaware of these symptoms regular sport activity
12 lead ECG
24 h Holter
Stress test ECG
PA LCA Ao RCA
Congenital Coronary Artery Anomalies: Classification Ostia Sequestration Valve-like ridge Acute take-off Origin from the Pulmonary artery from the Aorta High take-off Single coronary artery Wrong sinus LCx from RCA or Right Valsalva sinus LDA from RCA Course ( myocardial bridge ) Fistulae Aneurysms
Anomalous course Myocardial bridge
Myocardial Bridge and SD
MB Prevalence in the General Population Autopsy series:15-85% Angiographic series:0.5-2.5% The large discordance suggests that only a minority of patients with MB are at increased risk for clinical symptoms and cardiac events
Pathological Anatomy- University of Padua-I
Formulation of a diagnosis and the clinico-pathological summary It is important to accept that different degrees of certainty exist in defining the cause effect relationship between the cardiovascular substrate and the SCD event The commonest substrates of SCD have been classified as certain, highly probable or uncertain The clinical history and the circumstances of death may influence the decision making process
Pathological Anatomy- University of Padua-I