Aortic Bicuspid Valve in Flight Crew:  Case-Reports and

Aeromedical Fitness

Med Maj DELRUE

Centre for Aviation Medicine

Brussels

Belgium

Bicuspid Aortic Valve

• 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 Valvesystole diastole

Normal Aortic Valvesystole 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

• Prof Sievers (Luebeck Germany)

Type of Bicuspid Aortic Valve

• Prof Sievers (Luebeck Germany)

Type 1 L/R

A B

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 >= 8-9 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

• 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.