preventing embolization in inherited arrhythmia disorders pedro brugada, md, phd. chairman...
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PREVENTING EMBOLIZATION IN INHERITED ARRHYTHMIA
DISORDERS
Pedro Brugada, MD, PhD.Chairman Cardiovascular Division
UZ-Brussel, Brussels.
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Why this topic?
• Inherited arrhythmia disorders: An unknown cause of potential embolization.
• Guidelines on atrial fibrillation give no recommendations at all regarding inherited arrhythmia disorders (exception: HCM, WPW).
• NOACs may not be refunded for patients that may benefit the most.
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EMBOLIZATION IN INHERITED ARRHYTHMIA DISORDERS
• Atrial: ATRIAL FIBRILLATION– Familial atrial fibrillation– Short QT– Long QT– Brugada syndrome– HCM– PRKAG with HCM or CCM– ANP gene mutations
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EMBOLIZATION IN INHERITED ARRHYTHMIA DISORDERS
• Ventricular: STRUCTURAL– Left ventricular non-compaction– Post-op right ventricular dysplasia
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28 cM
I:1 I:2
11.7 cM
6.6 cM 0.5 cM
FAMILIAL ATRIAL FIBRILLATIONREGION SHARED ON 10q2
AFFECTED
NON AFFECTED
DEAD
Familial Atrial Fibrillation
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• Mean Age of Diagnosis 18 years• Range in age Diagnosis 1-35 years• Echocardiogram Normal• Chronic AF 41/42• Asymptomatic 36/42• Anticoagulation: 0• No embolization known: Future?
FAMILIAL ATRIAL FIBRILLATION CLINICAL FEATURES
Familial Atrial Fibrillation
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SHORT QT syndrome.
• Atrial fibrillation in 40-45% of cases.• Youngest reported case: In utero.• No known embolization.
Gussak, Brugada P, Brugada J, et al. Cardiology 2000
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LONG QT SYNDROME
• Atrial fibrillation: Yes, but incidence unknown
• Embolization: No reports
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BRUGADA SYNDROME
• Atrial fibrillation: 20-25%
• Embolization: Yes
• Youngest patients: 3-14 y
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Spontaneous atrial arrhythmias
→ Paroxysmal atrial fibrillation or SSS may be the first manifestation of Brugada syndrome: AJMALINE TEST!
→ Common cause of inappropriate ICD shocks:
14% of ICD recipients had inappropiate ICD shock because of asymptomatic, undiagnosed AF.
→ Marker of higher risk and/or of disease progress ?
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ATRIAL FIBRILLATION IN CHILDREN WITH BRUGADA SYNDROME
• 3/8 pts in 1992 paper were children with AF.• 3/3 had SSS and were pacemaker dependent.• 2/43 children had total atrial standstill.• 1/43 with right and left atriomegally.• 4/43 (10%) with atrial fibrillation or flutter.
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Transient ischemic attack in a 71 year-old man
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First consultation
• Patient comes for second opinion after TIA
• Admitted at local hospital during his holidays because of speech difficulties Rx: Aspirine, simvastatine
• Previous history: Appendicectomy. Similar episode 8 years ago.
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Admission at University Hospital
• Full neurological examination: Normal.• No carotid stenosis. CT, etc: Normal.• Cerebral MRI: Multiple microembolisms• Cardiological consultation: Normal, including
TEE, Holter, troponine, etc.
• Diagnosis: Cryptogenic TIA
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ECG
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Medical report local hospital
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ECG
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ECGs at admission
V1
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Ajmaline test
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TIA as a first manifestation of Brugada syndrome.
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Follow-up ECGs
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Follow-up
• DDD-ICD implanted.• Ablation of cavo-tricuspid isthmus.• Anticoagulation, sotalol.• No recurrence of AF after 2 years.
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EMBOLIZATION IN INHERITED ARRHYTHMIA DISORDERS
• Ventricular: STRUCTURE– Left ventricular non-compaction– Post-op right ventricular dysplasia
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Left Ventricular Non-compaction.
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Intratrabecular flow in LVNC
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Atrial Fibrillation in a 16 year-old boy.
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AA, born 16-03-1995
• 1998: diagnosis of left ventricular non-compaction
Rx: Metroprolol, asymptomatic.
• 3/2011: Atrial fibrillation with fatigue and dyspnea Rx: Sotalol (vomiting, low blood pressure)
Amiodarone (hyperthyroidism) Verapamil, Lasix, Spirolactone, Warfarine.
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Echocardiography
DATE LA (mm) LVEF (%) SPAP (mmHg)
19-1-2011 53 63 40
24-8-2011 56 61 70
23-9-2011 49 59 70
5-12-2011 (B) 57 30 75
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After optimal medical RX
DATE LA (mm) LVEF (%) SPAP (mmHg)
19-1-2011 53 63 40
24-8-2011 56 61 70
23-9-2011 49 59 70
5-12-2011 (B) 57 30 75
21-12-11 - MI 3/4 ! 40 95
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After optimal medical therapy
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13-12-2011
• Severe heart failure with caquexia• Persistent atrial fibrillation• Diagnosis of LV non compaction confirmed• Severe mitral regurgitation with dilatation of
the annulus en massive tricuspid insufficiency
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0
10
20
30
40
50
60
70
80
90 <3-month duration of atrial fibrillation prior to cardioversion
>12-month duration of atrial fibrillation prior to cardioversion
The longer one waits to initiate a rhythm-control strategy, the harder it is to regain sinus rhythm
1 month
P<.026 months
P<.07
Electrical cardioversion82%
36%
Patie
nts
in s
inus
rh
ythm
(%)
67%
27%<3m
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Electroanatomical mapping
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CRYOBALLOON ABLATION OF ATRIAL FIBRILLATION
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Success rate of single procedure catheter ablation
● PAF ranges from 38% to 78%. Most series > 60%.
● Persistent AF ranges from 22% to 45%. Most centers < 30%.
Success rate of mutiple procedure catheter ablation
● PAF ranges from 54% to 80%. Most series > 70%.
● Persistent AF ranges from 37% to 88%. Most centers around 50%.
Centre for Heart and Vessel Disease, University Hospital Brussels
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HYBRID THERAPY FOR ATRIAL FIBRILLATION
• Simultaneous endo and epicardial ablation.• Combination of RF and cryoablation possible.• Allows reduction of left atrium dimensions.• Allows exclusion of the left atrial appendage.
• Can be combined with other procedures: Valve repairICD implantation
• Success rate in persistent AF of 80-90%.
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After CRT-D
DATE LA (mm) LVEF (%) SPAP (mmHg)
19-1-2011 53 63 40
24-8-2011 56 61 70
23-9-2011 49 59 70
5-12-2011 (B) 57 30 75
21-12-11 - MI 3/4 ! 40 95
14-5-2012 - MI 1/4 62 60
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After CRT-D
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What about the future?
• Embolization• Heart failure • Atrial and ventricular arrhythmias.
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Best strategy to treat AF in inherited arrhythmia disorders?
• Medical: Disease specific therapies and disease specific pro-arrhythmic drugs.
THE MIRROR-IMAGE PATHOPHYSIOLOGY
• Electrical: Pacing for SSS.• Ablation: RF ablation versus cryoablation.• Surgical: Hybrid therapy for persistent AF.
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ISSUES ABOUT ANTICOAGULATION IN INHERITED ARRHYTHMIA DISORDERS.
• IADs not diagnosed as the cause of AF.• Incidence and prevalence of AF in IADs unclear.• Indications for anticoagulation in IADs unclear.• Role of CHADS2 and CHAD2DS2-VASC scores?
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With AF, the younger you are the greater the risk?
CHADS2 y/risk y to 100%
0 2 50
1 3 33
2 4 25
3 6 16
4 9 11
5 12 9
6 18 6
0
10
20
30
40
50
60
y/risk y to 100%
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Conclusions
• Because of the low embolization score NOACs are not reimbursed for patients with IADs in spite of the high cumulative life-long risk.
• Studies to understand and guidelines on how to manage AF in IADs are urgently required.
• In young individuals with AF, and in those with a structurally normal heart, IADs have to be excluded as the cause of AF.