CONGENITAL CORONARY ARTERY ANOMALIES

Transcription

1 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

2 DECLARATION OF CONFLICT OF INTEREST none

3 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%

4 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

5 Coronary Ostia Sequestration

6 Valve-like Ridge

7 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

8 Origin from the Pulmonary Artery

9 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

10 High Take Off

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12 High Take Off Purvis J et al. Heart 2010;96:

13 LCx from RCA

14 M, 18 yrs old SD at rest F, 18 yrs old SD on emotion

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16 Wrong Sinus Origin

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19 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

20 Relative Risk of Sport-related SD 0,6 0,5 RR=2.6 ( )* in CAD Athletes Non-athletes RR=79.0 ( )* p< p=.009 SD per 100,000 per year 0,4 0,3 0,2 0,1 0 CAD CCA Corrado et al, JACC 2003

21 ARRHYTHMIAS, SYNCOPE, SD Pathophysiology of SD Squeezing on effort Intramural aortic course Acute-angle take-off Transient Myocardial Ischemia Vasospasm

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24 Surgical Unroofing Intracoronary Stenting

25 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

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27 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

28 12 lead ECG

29 24 h Holter

30 Stress test ECG

31 PA LCA Ao RCA

32 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

33 Anomalous course Myocardial bridge

34 Myocardial Bridge and SD

35 MB Prevalence in the General Population Autopsy series:15-85% Angiographic series: % The large discordance suggests that only a minority of patients with MB are at increased risk for clinical symptoms and cardiac events

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37 Pathological Anatomy- University of Padua-I

38 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

39 Pathological Anatomy- University of Padua-I