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!