Sudden Death (SD) and hypertrophic...
Transcript of Sudden Death (SD) and hypertrophic...
Sudden Death (SD) and hypertrophic cardiomyopathy (HCM) –Attempt of risk stratification
84th Annual Scientific Meetingof the Aerospace Medical AssociationMay 12-16, 2013Sheraton Chicago Hotel & Towers, Chicago
Dr. med. Franz H. HauerFlugmedizin Oberkassel (Düsseldorf)
Flugmedizinischer Sachverständiger Klasse 1Senior Aviation Medical Examiner (AME-FAA)
Innere Medizin - KardiologieEuropean Cardiologist - FESC
Flugmedizin – VerkehrsmedizinHypertensiologe® DHL
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Disclosure Information
84th Annual AsMA Scientific MeetingFranz H. Hauer
I have no financial relationships to disclose.
I will not discuss off-label use and/or investigational use in my presentation.
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Hypertrophic Cardiomyopathy (HCM)
Hypertrophic Cardiomyopathy (HCM) is a relatively common primary heart
disease
and quite often the cause of sudden cardiac death (SCD)
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
DefinitionDefinition Risk Faktors
Case Report
Examination
Requirements of ICAO
Results
Proposal for special issuance
.
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Definition
Absence of another cardiac disease producing leftventricular hypertrophy (LVH)
Exclusion of Athlete´s heart
Autosomal dominant mutation
Prevalence of 0.2 % (i.e. 1:500) in the general population
Echocardiography: septal thickness more than 15 mm, 13 - 14 mm are
considered as borderline if there exists a family history of hypertrophic
cardiomyopathy or unexplained sudden cardiac death (SCD)
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Prevalence
Prevalence of 0.2 % (i.e. 1:500) in the general population
Estimated frequency seems much higher than the occurence in cardiologic
practices
Affected persons mostly remain unidentified, in most cases symptom free and
without shortened life expectancy (Guideline 2011 ACCF/AHA)
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
DefinitionDefinition
Risk Faktors Case Report
Examination
Requirements of ICAO
Results
Proposal for special issuance
.
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Major Risk Factors
Cardiac arrest (ventricular fibrillation)
Sustained ventricular tachycardia (sVT)
Non-sustained ventricular tachycardia (nsVT)
Septal thickness > 30 mm
Family history of sudden cardiac death (SCD)
Syncope
Abnormal blood pressure (BP) under exercise
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Potential Risk Factors
Left ventricular outflow tract (LVOT)
Resting gradient more than 33 mmHg;
5 mmHg increase doubles the risk of
sudden cardiac death (SCD);
(NB: after myektomy very low rates of sudden cardiac death were
seen)
LGE (Late Gadolinium Enhancement) in CMR (Cardio-MRI):
Represents fibrosis and scarring; LGE has been associated with nsVT
and ventricular ectopy, but not with sudden cardiac death (SCD)
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Potential Risk Factors
Genetic Mutation
More than 500 mutations in 14 genes are known.
In the european collective mutations can be found in
80% in both major genes MYH7 und MYBPC3
Atrial fibrillation (AFIB)
AFIB generally is an important cause of severe symtoms as for instance stroke and
heart failure
Factors for development are age, heart failure, size and volume of left atrium (LA)
AFIB is seldom seen in patients below 30 years of age
In some cases AFIB is not associated with symptoms, but is often poorly tolerated
in others
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
DefinitionDefinition
Risk Faktors
Case Report Examination
Requirements of ICAO
Results
Proposal for special issuance
.
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Case Report
37 y/o airline pilot
Non-smoker
height 189 cm, weight 81 kg, Body-Mass-Index 22,7 kg/qm
Free of symptoms, active sportsman
No cardiac risk factors
No medication
One of his uncles (father´s brother) died by a not identified heart disease
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
DefinitionDefinition
Risk Faktors
Case Report
Examination Requirements of ICAO
Results
Proposal for special issuance
.
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
ECG / Stress-ECG
ECG
Sinusrhythm, 77 beats/min, indifference typ, no alterations of T-
waves
Stress-Testing (bicycle)
Maximal load 225 Watt
Rise of frequency from 79 to 170 beats/min
Rise of blood pressure from 128/73 to 172/84 mmHg
no alterations of T-waves, no rhythm disturbances
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
ECG (resting)
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Echocardiography
Results
normal left ventricular enddiastolic diameter (LVEDD)
No wall motion abnormalities
No SAM (Systolic Anterior Movement), Mitralinsufficiency I°
normal systolic pulmonary pressure
Septal thickness 24 mm , no pressure gradient in left ventricular
outflow tract (LVOT) ,under exercise not more than 6 mmHg
normal size of left atrium (LA)
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Echocardiography
Stress echocardiography
Maximal load 175 Watt
Flow velocity in LVOT not more than 2,5 m/s (i.e. 25 mmHg
maximum)
No SAM unter maximal effort, no signs of obstruction
Conclusion
Completely normal findings
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
CMR (Cardio-MRI)
Normal enddiastolic diameter (LVEDD)
No wall motion abnormalities
Marked midventricular septal hypertrophy of 21 mm
Normal size of left atrium (LA)
No enlargement of right ventricle with normal function
Normal size of right atrium (RA)
No heart valve defects, no pericardial effusion
No apical aneurysm
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Adenosin-Perfusion-Scan
No perfusion abnormalities resting and under exercise
Late Gadolinium Enhancement:
patchy contrasting agent enrichement in the area of the hypertrophic septum
Conclusion
normal systolic function
No signs of ischemia
According CMR-Criteria: hypertrophic
non-obstructive cardiomyopathie (HNCM)
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Myocardial Biopsy
9 biopsies from the right-ventricular septum to assess morphological changes
Histologic Findings
moderate hypertrophy of the heart muscle fibers
focally marked fibrosis of the endocard
no specific findings for the diagnosis of cardiomyopathy
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Cardiopulmonary (metabolic) Testing
Reaching the necessary exercise level
No ventilatory limitation, good oxygen absorption
normal PO2 resting and under exercise
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Reveal-Recorder
Results
Normal findings
One ventricular, some premature ventricular beats
No atrial fibrillation (AFIB)
No ventricular tachycardia (VT)
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Reveal-Recorder
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
DefinitionDefinition
Risk Faktors
Case Report
Examination
Requirements of ICAO Results
Proposal for special issuance
.
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Requirements of ICAO
Manual of Civil Aviation Medicine (Doc 8984-AN/895), 3rd.Edition 2012
Blood Pressure Response normal
no VT
no family history of Sudden Death (SCD)
IVS < 25 mm
OML-Operation
AFIB paroxysmal or systemic is disqualifiing
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
DefinitionDefinition
Risk Faktors
Case Report
Examination
Requirements of ICAO
Results Proposal for special issuance
.
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Results
Normal exercise test without electrical instability or fall in BP (which may be
predictive of SCD)
No ventricular tachycardia (VT)
No atrial fibrillation (AFIB) (Circulation 2001, 104:2518 ff.)
No family history of sudden cardiac death (SCD)
IVS (interventricular septum) < 25 mm
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Results
CMR confirms diagnosis and shows LGE
Research of main risk factors is negative
No LA dilatation
No outflow tract gradient
genetic Screening (MYH7 und MYBPC3)
actual: mutation in gene MYBPC3
(Myosin-Binding Protein Typ 3,
heartspecific), hybrid pattern;
No changes in MYH7 (Myosin,heavy chain 7)
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Statistics
Risk of sudden cardiac death in the general population is approximately
1% (< 0,5% - 1,5%>)
55% of the whole population with HCM show no maior risk factors
Regarding these data the mortality of this group can be compared with the
mortality of the normal population
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
DefinitionDefinition
Risk Faktors
Case Report
Examination
Requirements of ICAO
Results
Proposal for special issuance
.
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Proposal for Special Issuance(VO(EU) 1178/2011 , Acceptable Means of Compliance and Guidance Material to Part-MED (EC) No 216/2008)
TML (six months)
OML-Operation
Holter-Monitoring or data of implanted Reveal-Recorder every six months
CMR (Cardio-MRT) every two years
84th AsMA Annual Scientific Meeting, Chicago May 12-16, 2013 Dr. med. Franz H. Hauer, Düsseldorf, Germany
Routine Examination (yearly)
Resting ECG
Stress Testing (bicycle)
Echocardiography with determination of outflow tract gradient
24-hour Holter Monitoring or Data of Reveal-Recorder
Lab findings including common risk factors
Dr. med. Franz H. HauerFlugmedizin Oberkassel (Düsseldorf)
Flugmedizinischer Sachverständiger Klasse 1Senior Aviation Medical Examiner (AME-FAA)
Innere Medizin - KardiologieEuropean Cardiologist - FESC
Flugmedizin – VerkehrsmedizinHypertensiologe® DHL
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