Reconstrucion of the Aortic Valve and Root: A practical approach,
September 18, 2019, Homburg-Saar, Germany
Aortic regurgitation and aortic
aneurysm- epidemology and
guidelines
Nina Wunderlich
Director, Cardiac Non-Invasive Laboratory
Cedars-Sinai Medical Center, Los Angeles
Professor of Medicine UCLA & CSMC
Robert J. Siegel, M.D.
Actor & TV moderator Alain
Thieke
PhysicistAlbert Einstein
Ski-coachWolfgang Graßl
Politician (FPÖ, Austria) Kurt Lukasek
Sergej Dorenko,Russia,
Journalist:
Professional soccerplayer Michael Novac
US ambassadorRichard Holbrooke
What do these people have in common?
Aortic regurgitation and aneurysm
Aortic aneurysms
– Etiologies
– Definition of aortic aneurysms
– Imaging modalities & measures
– Clinical presentation of aortic aneurysms
– Recommendations for surgery
Aortic regurgitation and aneurysm
Aortic aneurysms
– Etiologies
– Definition of aortic aneurysms
– Imaging modalities & measures
– Clinical presentation of aortic aneurysms
– Recommendations for surgery
Aortic AneurysmsEtiology
Degenerative:
Atherosclerosis (most common), hypertension
Hereditary disorders:
Marfan syndrome
Loeys-Dietz syndrome
Ehlers-Danlos syndrome
Turner’s & Noonan’s syndromes
Hereditary vascular conditions:
Bicuspid aortic valve syndrome (2nd most common)
Aortic coarctation
Familial aortic root dilatation, & annuloaortic ectasia
Infection- “itis”(syphilis, tuberculosis, salmonella),
Aortitis (Takayasu arteritis, Giant cell arteritis, Behcet disease, Ankylosing
spondylitis)
Aortic AneurysmsEtiologies
GuidelinesAHA/ACC 2010 Guidelines on Thoracic Aortic Disease 2010
Hiratzka et al; Guidelines on Thoracic Aortic disease; Circulation 2010
Aortic AneurysmsHereditary disorders
1896 Dr. Antoine Marfan Pt had arachnodactyly, scoliosis, chest asymmetry
Incidence 1/3,000-10,000
All ethnic groups
Clinical Dx- major/minor criteria: skeletal, ocular, cardiovascular, lungs, dura, skin, family history
Mutation Fibrillin-1 gene, chromosome 15
↑ TGF-beta signaling in the vessel wall
Aortic AneurysmsMarfan syndrome
Jakob Erdheim, MD.
1874-1937
Erdheim’s
Normal Marfan
Aortic AneurysmsMarfan syndrome- “cystic medial necrosis”
Aortic AneurysmsMarfan syndrome
Life expectancy determined by cardiovascular
involvement, especially the aorta
Prophylactic aortic root replacement ↑ prognosis.
Low risk if aorta <4 cm.
High risk if aorta >5 cm.
Life expectancy was 35 yrs → now 72 yrs
Aortic AneurysmsMarfan syndrome- Prognosis
Autosomal dominant syndrome described in
2006
Many features similar to Marfan syndrome but
worse prognosis
D/t gene mutations encoding transforming growth
factor beta receptors: (TGFBR1) or (TGFBR2).
Aortic AneurysmsLoeys Dietz Syndrome
Widely spaced eyes (hypertelorism)
Cleft palate or bifid uvula
Aortic & arterial aneurysms/dissections
Arterial tortuosity (corkscrew structure)
Aortic AneurysmsLoeys Dietz Syndrome- main clinical characteristics
At increased risk for aortic dissection & arterial dissection
& rupture
Screen with CT or MRA from head to pelvis at baseline &
1 yr- then depending on findings 2 to 3 yrs
Prophylactic aortic surgery is indicated when the asc aorta
is ≥ 4.2cm by echo or 4.5cm by CT / MRI
Aortic AneurysmsLoeys Dietz Syndrome- Treatment
A group of inherited connective tissue disorders d/t a defect in
synthesis of collagen
Depending on individual mutation, severity of the syndrome
varies from mild to life-threatening
No cure & treatment is only supportive, monitoring of
cardiovascular system
Corrective surgery may help with some of the problems, but
try to avoid surgical therapy→ weak tissue
Aortic AneurysmsEhlers-Danlos-Syndrome (EDS)
Bicuspid aortic valve (BAV) syndrome- a BAV with
an enlarged aortic root or ascending aorta.
Associated abnormalities:
ASD
VSD
PDA
& aortic coarctation.
Aortic AneurysmsBicuspid aortic valve syndrome- Definition
Probably inherited:
Abnormal in the NOTCH 1 gene & associated with
hypoplastic left heart
But genetics is not understood
> ½ of BAV pts have aortic dilatation
1/3 of first degree relatives have aortic
dilatation in the absence of a BAV
Aortic AneurysmsBicuspid aortic valve syndrome- Etilology
Bicuspid aortic valve – 2 commissures, no raphe
Organic AR- bicuspid AVAortic AneurysmsBicuspid aortic valve syndrome- Etilology
Live 3D- also an aortic root disease
Organic AR- bicuspid AVAortic AneurysmsBicuspid aortic valve sysndrome- Etilology
Aortic regurgitation and aneurysm
Aortic aneurysms
– Etiologies
– Definition of aortic aneurysms
– Imaging modalities & measures
– Clinical presentation of aortic aneurysms
– Recommendations for surgery
Aortic regurgitation
Berrebi A et al. Annals of cardiothoracic surgery, Vol 8, No 3 May 2019
Aortic Aneurysm
Aortic aneurysm
dilatation of an aortic segment ≥1.5 x normal diameter
In healthy subjects aortic diameters do not usually exceed
40mm and taper gradually downstream
Low, but progressive aortic dilation: expansion is about 0.9 mm
in men and 0.7 mm in women for each decade of life
Normal Diameters (2D)- male vs. female:
Annulus: 2.6cm & 2.2cm
Sinus of Valsalva: 3.4cm & 3.0cm
Sinotubular junction: 2.9cm & 2.6cm
Prox. ascending aorta 3.0cm & 2.7cm • Nomograms for sinuses of Valsalva relating size to age & BSA
Aortic aneurysmsDefinitions
Lang RM et al. EHJ- Cardiovascular Imaging (2015) 16, 233–271.
Aortic regurgitation and aneurysm
Aortic aneurysms
– Etiologies
– Definition of aortic aneurysms
– Imaging modalities & measures
– Clinical presentation of aortic aneurysms
– Recommendations for surgery
Segments of the AortaMeasurements
Erbel et al. European Heart Journal (2014) 35, 2873–2926
Differing methods used
for aortic root dilatation
Diastolic vs. Systolic
Inner to inner edge
Outer to outer edge
leading edge to leading
edge
Perpendicular to the
centerline of the vessel
Z score calculator Differences of 2-3mm may alter therapeutic decisions
Standardized measurents are needed!
Segments of the AortaMeasurements
Erbel et al. European Heart Journal (2014) 35, 2873–2926
Differing methods used
for aortic root dilatation
Diastolic vs. Systolic
Inner to inner edge
Outer to outer edge
leading edge to leading
edge
Perpendicular to the
centerline of the vessel
Z score calculator
Proposal to get better reproducibility: inner-inner edge
technique at end diastole on the strictly transverse plane by
double oblique reconstruction perpendicular to the axis of
blood flow
Berrebi A et al. Ann Cardiothorac Surg 2019;8(3):331-341
Imaging modalities
All imaging modalities have limitations!
When using an imaging modality, strengths and
limitations should be taken into consideration
Erbel et al. European Heart Journal (2014) 35, 2873–2926
Imaging modalities
TTE is sufficient in most cases with AAA for serial FU`s and
for decision making
TEE more accurate than TTE for distal asc aortic aneurysm,
arch & desc aorta
TEE accurate for sizing & defining aortic wall pathology
CT is most accurate to assess true aortic dimensions and to
detect aortic dissection
MRI is particularly useful for serial FU`s in younger pts
A PET may be useful in pts with inflammatory disease
Aortic regurgitation and aneurysm
Aortic aneurysms
– Etiologies
– Definition of aortic aneurysms
– Imaging modalities & measures
– Clinical presentation of aortic aneurysms
– Recommendations for surgery
Aortic diseases
Most chronic disorder of the aorta
long period of subclinical development
40% of pts with ascending aortic aneurysm are
asymptomatic, as are most pts with Marfan syndrome,
pseudoaneurysm, & sinus of Valsalva aneurysms
Acute aortic syndrome is often the 1st sign of the
disease
needs rapid diagnosis and decision making to reduce
the extremely poor prognosis
Aortic Aneurysms
Acute aortic disorders (dissection / rupture, intramural
hematoma, & penetrating aortic ulcer) generally present
with
severe chest pain
back, retrosternal or interscapular pain
dyspnea
dysphagia
or extremity pain
Aortic dissection, rupture of ao sinus of Valsalva, or
trauma with flail aortic cusps → severe acute AR & CHF
Physical exam in chronic or acute disorders of the aorta
of limited Dx value
Acute Aortic Disorders
Age: majority between 40 and 70 years
Sex: 2-5 times more common in men
Race: Common in African Americans, rare in
Orientals
Temporal: 6-10 am,
early afternoon
Seasonal: winter/spring
Acute Aortic DissectionEpidemiology
Tear in intima results in blood entering the media,
dissecting aortic wall and forming false lumen
Acute Aortic DissectionDefinition
Acute Aortic DisordersClassification of aortic dissection localization
Erbel et al. European Heart Journal (2014) 35, 2873–2926
“No physician can diagnose a
condition they never think about.”
Dr. Michael De Bakey
aortic dissection
5%
35%
Misdiagnosis:
myocardial infarction
Ascending Aortic DissectionChoice of imaging modality
Sensitivity 100%
Specifitiy 98%Sensitivity 98%
Specifitiy 98%
Sensitivity 100%
Specifitiy 98%
J Am Soc Echo 2015; 28:119
Aortic AneurysmsExample- Type A dissection
Aortic regurgitation and aneurysm
Aortic aneurysms
– Etiologies
– Definition of aortic aneurysms
– Imaging modalities & measures
– Clinical presentation of aortic aneurysms
– Recommendations for surgery
Ascending Aortic aneurymsRecommendations on interventions
ESC Guidelines2014
Director, Cardiac Non-Invasive Laboratory
Cedars-Sinai Medical Center, Los Angeles
Professor of Medicine UCLA & CSMC
Robert J. Siegel, M.D.
Actor & TV moderator Alain
Thieke
PhysicistAlbert Einstein
Ski-coachWolfgang Graßl
Politician (FPÖ, Austria) Kurt Lukasek
Sergej Dorenko,Russia,
Journalist:
Professional soccerplayer Michael Novac
US ambassadorRichard Holbrooke
What do these people have in common?
5 year survival 27% if symptomatic
58% for asymptomatic
61% < 6.0cm,
38% if > 6.0cm
Law of La Place: As size of aneurysm
increases wall tension rises; disease
progression is self propagating
Aortic Dissection- Prevention
Size determines prognosis!
It can be monitored!
Pts with weakened aortic wall or dilated aorta
Hypertensives
Connective tissue disease
Family history aortic diseases
Bicuspid aortic valve/ Coarctation
Trauma
Aortic Dissection- PreventionWho to screen?
The apple does not fall far from the tree
Thank you!