Heartline
IRCCS S. Martino
Genova
Cardiology meeting
14-15 novembre 2014
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STENOSI
INSUFFICIENZA

FORME MISTE
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Prevalente stenosi
Prevalente
insufficienza
•Secondo le stime più recenti, questa affezione colpisce il 4,6%
della popolazione oltre i 75 anni, un quinto dei quali deve essere
operato nel giro di 2-3 anni di anni, altrimenti non può
sopravvivere.
Nonostante la gravità, questa per gli esperti è una malattia
sottovalutata e spesso non trattata in maniera appropriata
•Da una ricerca dell’istituto britannico Opinion Matters su un
campione rappresentativo della popolazione italiana over 60 è
emerso che solo il 7,7% delle persone è preoccupata per le
valvole cardiache.
Sono i tumori (24,6%) e il morbo di Alzheimer (20%) le malattie
che spaventano di più.
The Huffington Post
 Congenite

Valvola uni/bicuspide
 Acquisite


Displasia senile fibro-calcifica
Malattia reumatica

Congenite

Valvola uni/bicuspide


Acquisite

Displasia senile fibro-calcifica



malattia aterosclerotica?
Malattia reumatica


patologia di per sé?
simile patogenesi?
Altre rare patologie
Patologie acquisite su base
congenita?
Fibrosi e calcificazione su
valvole tricuspidi/bicuspidi
asimmetriche?


Abbiamo in mente un aspetto
“ideale” di una valvola
cardiaca
Più ci si allontana da questo
ideale più aumenta il rischio di
patologia acquisita
Varianti anatomiche
 Senilità
sono perfetti substrati per vere
patologie

•
•
•
Anomalo numero delle
cuspidi
•
Unicuspide
•
Bicuspide
•
Quadricuspide
•
Atresia
Quadricuspid valve
Anomala proporzione fra le
cuspidi
Anomala struttura
•
Cuspidi displastiche
AO valve atresia
 Accentuazione
delle lunule
 Fenestrazioni
Vegetazioni marantiche
escrescenze di Lambl
formazioni trombotiche con evoluzione fibrotica
(A) Bicuspid and dysplastic valve with
both coronary arteries arising from
the anterior facing sinus.
(B) Bicuspid valve with the leaflets and
sinuses arranged in left–right fashion.
A raphe is present in the right leaflet
(C) A valve with three thickened
leaflets. One of the leaflets is
considerably larger than the other
two.
(D) This valve with three leaflets has
been cut longitudinally to show the
thick and gelatinous looking leaflets.
(E) There are abundant calcific nodules
in this severely stenotic valve from
an elderly patient.
(F) This unicommisural and unifoliate
valve has a tiny eccentric orifice.
Ho S Y Eur J Echocardiogr 2009;10:i3-i10
Lo stato della valvola riguarda il
cardiochirurgo?

Opzione chirurgica
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Morfologia della valvola



Solo la valvola
Valvola e radice aortica
Solo radice aortica
Numbero delle cuspidi
Fibrosi/calcificazione
Patologie associate


Coronarie
Bulbo/Aorta ascendente
Aortic bulbus
•The enlarged part where the cusps
are situated
•Diameter 1.5 superior in respect to
proximal ascending aorta
Sinutubular junction
Immediately above the three aortic
sinuses
Ascending aorta
From the bulbus up to the first
bifurcation of the aortic arch

1 –2 % della popolazione

maschio/femmina 3:1

Familiarità

Cuspidi spesso asimmetriche


Ostio di aspetto semilunare
Cuspidi:

“antero-posteriore”




2 coronarie nel seno anteriore
“lato a lato”
 1 coronaria per seno
30-40%
Presenza variabile di un rafe o falsa
commissura


60-70%
36-59%
Distribuzione coronarica a dominanza
sinistra 4 volte piu comune
origine sovracommissurale dell’ostio cor.
sin. 2 volte più comune
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Aortic valve disease is one of the most common congenital
cardiac defects, occurring in 5% of all children with heart
disease.
Bicuspid aortic valve (BAV) is the most common congenital
cardiac malformation, affecting 1-2% of the population,
with strong male predominance.
Individuals may have a normally functioning BAV, and may
be unaware of its presence and the potential risk of
impending complications.
They may typically remain asymptomatic until the third or
fourth decade of life, when the valve becomes
dysfunctional.
BAV is associated with both valve disease and aortic
disease, thereby leading to increased morbidity and
mortality, including

other cardiovascular malformations



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coronary anomalies, coartaction etc.
aortic valve disorders
aortic wall abnormalities
endocarditis
Bicuspid aortic valve phenotype and aortic disease: a magnetic
resonance study
David W Fitz*, James C Carr and Edwin Wu
J Cardiovasc MR 2011

Four valve morphologies on 217 cases:
type 1, fusion of the right and left cusps (n=152);
 type 2, fusion of the right and non-coronary cusps
(n=48);
 type 3, fusion of the left and non-coronary cusps
(n=9);
 and unicuspid, two fusions (n=8).


further characterized by the number of sinuses, two or
three, and the presence or absence of a raphe.
Conclusion
 Type 1 BAV is the most frequent phenotype.
 Two sinus valves are more common among type
2 and type 3 phenotypes.

Type 1
Type 2 is associated with
moderate to severe aortic stenosis
 a larger mid-ascending aortic diameter.

Bicuspid aortic valve phenotype and aortic disease:
a magnetic resonance study
David W Fitz*, James C Carr and Edwin Wu
J Cardiovasc MR 2011
Type 2
Anatomo-Clinical demonstration
of progressive aortic dilatation
 Abnormalities in components of
the extracellular matrix or
aberrant vascular matrix
remodeling might contribute to
abnormal valvulogenesis and a
structurally weakened aortic
root.
 Other studies supports the
concept of an independent aortic
remodeling process
 throughout childhood among
patients with a bicuspid aortic
valve
 may precede frank aneurysm
formation.


Fibrosis
Calcification
Thrombosis
Infections
Hemolisis
DISSECTION
Sudden death?

Fibrosis and Calcification




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
Differences in clinical presentation between patients with
tricuspid aortic valves (TAVs) or bicuspid aortic valves (BAVs)
and aortic valve disease are evident.
 METHODS:

702 patients with aortic valve and/or ascending aortic pathology; 202
also had concomitant coronary artery disease.
RESULTS:
 A BAV was commonly found in patients with isolated valve disease
(BAV 47%, TAV 53%) and frequently associated with ascending
aortic dilatation (BAV 80%, TAV 20%).
 In patients with coronary artery disease, a TAV was commonly found
(TAV 84%, BAV 16%).
 The combination of ascending aortic dilatation and coronary artery
disease was markedly rare regardless of valve morphology (TAV, 7
out of 38; BAV, 6 out of 127).
 The distribution of valve pathology and clinical parameters was
similar in patients with TAV and BAV with coronary artery disease (P
≥ .12).
 Without coronary artery disease, parameters associated with
cardiovascular risks were more often seen in patients with TAV than
in patients with BAV (P ≤ .0001).

J Thorac Cardiovasc Surg. 2014 Aug 17. pii: S0022-5223(14)01114-3. doi:
Jackson V1, Eriksson MJ2, Caidahl K2, Eriksson P3, Franco-Cereceda
A4
CONCLUSIONS:
 Coronary artery disease is uncommon in
surgical patients with BAV, but it is
associated with TAV, advanced age, and
male gender.
 Coronary artery disease and
ascending aortic dilatation rarely coexist,
regardless of valve phenotype.
 Differences in the prevalence of coronary
artery disease or
ascending aortic dilatation between
patients with TAV and BAV are not
explained by differences in
cardiovascular risks or the distribution
of valve pathology.
J Thorac Cardiovasc Surg. 2014 Aug 17. pii: S00225223(14)01114-3. doi:
Jackson V1, Eriksson MJ2, Caidahl K2, Eriksson P3, Franco-Cereceda
A4.
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The process of valve disease is believed to be initiated with the
development of atherosclerosis along the aortic surface of the valve
which, subsequent to aortic valvular osteoblast differentiation, calcifies.
Early in the disease process, the thickened aortic valve is said to be
sclerotic; that is, although thickening of the valves is present, there is
not yet obstruction of the valve orifice.
Later in the disease process, as the thickening of the valve progresses
and the valve orifice becomes significantly obstructed, the valve is said
to be frankly calcific.
Comparsa di elementi
infiammatori
-Ruolo patogenetico?
-Secondaria alla fibrosi?



In rheumatic fever,
antistreptococcal antibodies
produced by B lymphocytes crossreact with host-tissue epitopes,
producing inflammation in a
number of organ systems,
including the heart and its mitral
and aortic valves. Any or all 4
valves may be involved.
Inflammation induces angiogenesis
in the normally avascular valve
layers; over a period of months or
years, thickening of the valve
develops as inflamed elastic tissue
becomes replaced by irregular
masses of collagen fibers.
Recurrent episodes of rheumatic
fever produce progressive damage
to the host’s valves.
renal failure,
 familial hypercholesterolemia,
 Paget disease,
 systemic lupus erythematosus,
 ochronosis with alkaptonuria,
 Radiation
 left ventricular noncompaction

systemic lupus erythematosus
IgG4-related disease of the aortic valve: a report of
two cases and review of the literature.
Cardiovasc Pathol. 2014 Sep 28. pii: S1054-8807(14)00085-4. doi:
10.1016/j.carpath.2014.08.001.
Maleszewski JJ1, Tazelaar HD2, Horcher HM3, Hinkamp TJ4, Conte
JV5, Porterfield JK6, Halushka MK7.
A case of aortic and mitral valve involvement in
granulomatosis with polyangiitis.
Cardiovasc Pathol. 2014 Aug 4.
Espitia O1, Droy L2, Pattier S3, Naudin F4, Mugniot A5, Cavailles A4, Hamidou
M6, Bruneval P7, Agard C6, Toquet C2.
Aortic valve replacement in systemic
sclerosis.
J Cardiovasc Med (Hagerstown). 2014 Mar 12.
Ferrari G1, Pratali S, Pucci A, Bortolotti U.


Patogenesi simile?
Forme infiammatorie


Calcificazione



Fusione delle cuspidi
Inizia dal margine
aortico
Forme diverse all’inizio
Alla fine si somigliano
tutte

Bicuspidia


Post-infiammatorie


Associazione con lesioni aortiche
Possibili recidive, quadri sistemici
Forme senili

Progressivo aumento