Transcript DGIP-SPS-PROLAY-PLCD-001-Aanhangsel 4-Power

Aortic Bicuspid Valve in Flight
Crew: Case-Reports and
Aeromedical Fitness
Med Maj DELRUE
Centre for Aviation Medicine
Brussels
Belgium
Bicuspid Aortic Valve
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Most common cardiac defect
Not only the valve but the whole aortic root
1 to 2 % of the population
Sex ratio 4 men/1 woman
Normal Aortic Valve
systole
diastole
Normal Aortic Valve
systole
diastole
Bicuspid Aortic Valve
Tricuspid
Bicuspid
Pathogenesis
• Defect fibrillin-1
• Hereditary? : autosomal dominant with
reduce penetrance
• screening of first degree relatives
Type of Bicuspid Aortic Valve
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Prof Sievers (Luebeck Germany)
Type of Bicuspid Aortic Valve
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Prof Sievers (Luebeck Germany)
Type 1 L/R
AB
RISKS of Bicuspid Ao Valve
• Serious complications 33 %
• Responsible for more complications and
death than all others cardiac
malformations
– Valvular stenosis
– Regurgitation
– Endocarditis
– Dilation and dissection Aortic Root (X9)
Associations
- 50 % of young people with normal bicuspid
functioning valve have aortic dilation.
- coarctation of the aorta (50-80%)
- septum anomalies
- Coronary anomalies
- More cerebral aneurysms ?
Aortic Dilation and Bicuspid Aortic
Valve
• Ascending Aorta :
D(mm) =31+0.16* age (years)
Aneurysm
40 mm 20-year-old
45 mm 40 year-old
50 mm 60 year-old
Follow-up
• Annual transthoracic of transesophageal
echocardiography (valve function)
• Annual MRI (or CT angiography/aortic
protocol) if >40 mm or above age-related
normal range
(6 months if rapid progression)
Ct scan
Surgery Repair
• No valvular indication
• a. max diam >= 50 mm
• b. rapid growth progession of >= 0.5
cm/year
• b. maximum aortic cross-sectional
area/body height >= 10 cm2/m
Surgery Repair
• If valvular indication
• Max diameter >=45 mm
• Max aortic cross-sectional area/body >= 89 cm2/m
• More Attention if pregnancy, sudden death
or aortic dissection in 1st degree relative
Screening
• Auscultation +- 60 % Aortic regurgitation
• 16% of severe not diagnostiqued
Screening?
• 2278 asymptomatic competitive Italian athletes
(Age 31+- 11 years) : echocardiography
• 58 (2,5%) BAV
• Only 9 normal function
• 45 Aortic regurgitation (14 mild, 25 moderate, 8
severe)
• Stenosis 2
• Aortic root > nl
• 15 disqualified for sport
Case Report 1
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Jan 2013 Military Pilot applicant Male 18 year
Sclerosis Aortic Valve
Aortic Bicuspid valve
Aortic Root dilatation 37 mm (nl 33.8 mm)
• Military Pilot Applicants : systematic echography
• UNFIT
• Classe 1 civilian ? EASA rules
(European Aviation Safety Agency)
• May be assessed as fit if no other cardiac
or aortic abnomaly
• Here : proposal FIT with annual
cardiologic review
(outcome?) licensing autority
Case Report 2
• Military ATC male 53 years
• 2002 : aortic murmur detected
: echography : Bicuspid Valve + calcifications no
dilation aorta + Aortic regurgitation ¼
• Follow up every year echography
• 2011 : dyspnea (stairs) progression of sclerosis
valve (0.9 cm2) Aortic replacement Ross
procedure (pulmonary autograft)
• 6 months unfit
• FIT with Annual cardiologic review
Case report 3
• 41 year old helicopter pilot Federal Police
• 1990 : military heli applicants no echography
• 2001 : Aortic murmur : echography Aortic
Bicuspid Valve + dilation Ao Root 41 mm
• Aorta Regurgitation 2/4
• Fit with 6 months cardiologic review
• 2005 Aortic Root : 46 mm CT scan
• 2010 48.5 mm Classe 1 OML(multicrew) SIC
Cardiologic evaluation 6 months
• 2011 51 mm unfit Classe 1
Bentall
• video
Read More
• Etz, Misfefd and al. Indication for Surgical
Repair in Patients with Bicuspid Aortic
Valve and Ascending Aortic Ectasia.
Cardiol Res Pract 2012; 313879.
• Fedak, Verma and Al. Clinical and
Pathophysiological Implications of a
Bicuspid Aortic Valve. Circulation 2002;
106: 900-904.