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C. Basso and G. Thiene
Fig. 4.55 Functional mitral
valve incompetence in dilated
cardiomyopathy because of
annular dilatation (a) and in
postinfarction scarring, with
remodeling of left ventricular
cavity (b)
Fig. 4.56 Schematic drawings of left ventricle cavity
with a displaced papillary muscle and tethering. AO
aorta; LA left atrium; LV left ventricle
ab
Fig. 4.57 Left atrial myxoma
with smooth muscle
herniating into the mitral
valve orice. (a) 2D echo. (b)
View of the specimen after
successful surgical removal
ab
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Fig. 4.58 Giant villous left atrial myxoma, with obstruction of the
mitral valve orice, cardiogenic shock, pulmonary edema, and death,
occurred in pre echocardiography era
55
of the annular sphincteric contraction around it (Fig.4.59).
Since the risk of neoplastic or thrombotic embolism with
stroke is high, left atrial myxomas should be removed surgically on an urgent/emergency basis.
Before availability of echocardiography, 25% of surgically resected myxomas were incidentally found at surgery,
with a clinical diagnosis of rheumatic mitral valve stenosis.
Endocardial papillary broelastomas may grow up in the
mitral apparatus and account for valve dysfunction
(Fig.4.60).
Fig. 4.59 Left atrial myxoma
strangulated by sphincteric
contracting of the mitral
annulus. (a) echo and (b)
surgical pathology view
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C. Basso and G. Thiene
a b c
Fig. 4.60 Left ventricular papillary broelastoma (a) echo view; (b) gross appearance following surgical removal; (c) broelastic arborization at
histology. LV left ventricle; RV right ventricle
Tricuspid Valve
Acquired pathology of the tricuspid valve can be divided into
primary (Table4.1) and secondary:
“Primary”
Rheumatic Tricuspid Valve Disease
The tricuspid valve may be involved by rheumatic valvulitis,
although much less frequently than mitral and aortic valves
and never as an isolated form.
Rheumatic abacterial verrucae are located in the contact
borders of the leaets, the site of traumatic endocardial erosion during systolic closure. Organization of valvulitis is
similar to that occurring in the mitral valve: thickening and
retraction of the leaets with neovascularization, commissural fusion, shortening, and fusion of the chordae tendineae
(Figs.4.61 and 4.62) resulting in stenotic, steno- incompetent,
and incompetent tricuspid valve.
Involvement of the tricuspid valve occurs in the setting of
triple rheumatic valve disease (Fig.4.63).
Table 4.1 Classication of acquired tricuspid valve diseases
“Primary” structural tricuspid valve disease
Congenital See chapter congenital valve disease
Acquired Post-inammatory • Rheumatic
• Infective endocarditis
Carcinoid and carcinoid-like syndromes
Traumatic
Neoplastic
Carcinoid Valve Disease
Carcinoid tumor of the small intestine, mostly the appendix,
is an endocrine neoplasm secreting serotonin,
5- hydroxytryptophan, histamine, bradykinins, and prostaglandins (Table 4.2). in addition, tumor metastases to the
liver via the portal vein may release toxic substances. Before
reaching the lungs, these toxic agents cross the right cardiac
chambers and damage the endocardium of right atrium, tricuspid valve, right ventricle, and pulmonary valve, which
represent the target of their action as part of a carcinoid syndrome (Fig.4.64).
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Fig. 4.62 Severe rheumatic tricuspid valve disease with remarkable
cusp thickening and fusion of the anteroseptal commissure
57
a
b
Fig. 4.61 Rheumatic tricuspid valve disease. Note: commissural
fusion, cusp thickening, and chordal retraction-fusion with
steno-incompetence
The reaction of the involved structures is represented by
smooth muscle cell proliferation with onset of endocardial
plaques (Fig. 4.65) as well as thickening and retraction of
tricuspid and pulmonary leaets with valve incompetence.
The damage occurs on the endocardium of the right-sided
cavities (Fig.4.65): the auricularis of the tricuspid leaets
(Fig.4.66), the chordae tendineae, and the ventricularis of
the pulmonary cusps (Fig.4.67).
If the metastases reach the lungs and the neoplastic tissue
continues to secrete, the left-sided cardiac valves may also
be involved, developing similar lesions. Serotonin-like drugs
(ergotamine, methysergide, fenuramine-phentermine)
account for the so-called “fen-phen” phenomenon
(Table4.3).
Fig. 4.63 Multivalvular rheumatic disease (a) mitro-aortic; (b)
tricuspid
Table 4.2 Toxic chemical agents secreted by carcinoid tumor, accounting for endocardium damage
Carcinoid disease
Tumor products
• 5-hydroxytryptamine (serotonin)
• 5-hydroxytryptophan
• Histamine
• Bradykinins
• Tachykinins
• Prostaglandins
Ischemic
Postinfarction rupture of a papillary muscle of the tricuspid
valve occurs quite rarely. A healed posteroinferior myocardial infarction may retract the base of papillary muscles
causing distortion of the valve apparatus with secondary
incompetence (Fig.4.68).
Traumatic
Blunt chest traumas may cause rupture of a papillary muscle
with tricuspid valve incompetence. The most vulnerable
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C. Basso and G. Thiene
Fig. 4.66 Remarkable involvement of the tricuspid valve in carcinoid
syndrome, with thickening and retraction of the leaets as well as fused
chordae tendineae with disappearance of interchordal spaces
Fig. 4.64 Carcinoid syndrome with schematic representation of heart
involvement. CS coronary sinus; IVC inferior vena cava; LA left atrium;
LV left ventricle; MV mitral valve; RA right atrium; RV right ventricle;
SVC superior vena cava
Fig. 4.65 Carcinoid endocardial plaque in the pulmonary
infundibulum
Fig. 4.67 Histology of a carcinoid plaque of a semilunar pulmonary
cusp on the side of brosa. Note also thickening around chordae tendineae of the tricuspid valve
Table 4.3 “Carcinoid-like” valvular disease exposure to serotoninlike drugs
“Carcinoid-like”
Valvular disease
Exposure to serotonin-like drugs
• Ergotamine
• Methysergide
• Fenuramine-phentermine (“fen-phen” disease)
ab
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Fig. 4.68 (a) Secondary tricuspid valve incompetence due to postinfarct ventricular dilatation and myocardial translucent scarring. (b)
Arrhythmogenic right ventricular cardiomyopathy, with the pathognomonic transilluminated aneurysm of the posterior wall
structure is the tiny conal (Lancisi) papillary muscle
(Fig. 4.69) accounting for acute tricuspid insufciency,
which is initially well tolerated, being often discovered late
after the traumatic event, when signs of right heart failure
appear.
Neoplastic
Angiosarcoma of the heart usually arises at the level of right
AV junction and invades the tricuspid valve orice and apparatus with steno-incompetence (Fig.4.70).
Endocardial papillomas may grow upon the tricuspid
valve leaets (Fig.4.71). These are benign tumors, at the risk
of embolization, both neoplastic and thrombotic, the latter
because of thrombus lining the surface. Since the embolus is
of small size, the effect of a pulmonary infarct is negligible,
Fig. 4.69 Traumatic rupture of the conal (Lancisi) papillary muscle
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C. Basso and G. Thiene
ac
b d
Fig. 4.70 Tricuspid valve incompetence by cardiac angiosarcoma with typical growth at the right av junction at echo (a). Endomyocardial biopsy
immunohistochemistry stains in keeping with angiosarcoma (b, c, d) from Poletti etal., Cardiovascular Pathology 1991
unlike leiomyosarcoma of the pulmonary infundibulum, the
size of which may simulate a massive pulmonary
thromboembolism.
Currently, the most frequent cause of functional tricuspid
valve incompetence is pulmonary arterial hypertension,
whether primary or secondary to chronic increase of left
sided venous pressure like mitral valve disease or left ventricular failure. The reason why the right AV annulus dilates
Secondary: “Functional”
with enlargement of the tricuspid valve orice is the existence of a tiny brous AV ring, much thinner than that of the
There are some acquired myocardial diseases which may
cause tricuspid insufciency due to tethering of a still intact
mitral valve (Fig. 4.72). The more the pulmonary arterial
hypertension, the more the tricuspid valve regurgitation.
valve apparatus. This is the case with healed right ventricular
myocardial infarction (Fig.4.68a) and arrhythmogenic cardiomyopathy (Fig.4.68b).
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Fig. 4.71 Endocardial
broelastoma of the septal
tricuspid valve leaet at echo
(a) and after surgical removal
(b). AD right atrium; VD right
ventricle; Fbroelastoma;
VSleft ventricle
61
Fig. 4.72 Comparison
between gross (a) and
histology (b) of right
(tricuspid) and left (mitral) av
rings: the right one is thinner.
Myocardial ventricular mass
attached to the annulus is less
at tricuspid level, accounting
for smaller sphincteric
constriction
a
b
Right AV Ring
Left AV Ring
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C. Basso and G. Thiene
Further Reading
Anderson RH, Becker AE.The heart: structure in health and disease.
London: Gower Medical Publishing; 1992.
Basso C, Valente M, Thiene G. Cardiac tumor pathology. NewYork:
Humana Press; 2013.
Becker AE, Anderson RH. Cardiac pathology: an integrated text and
colour atlas. Hon Kong: Gower Medical Publishing; 1983.
Buja LM, Butany J. Cardiovascular pathology, 5th ed. Elsevier
Academic Press; 2022.
Burke A, Virmani R.Atlas of tumor pathology. Tumors of the Heart
and Great Vessels. Washington DC: Armed Forces Institute of
Pathology; 1996.
Davies MJ. Colour atlas of cardiovascular pathology. 1st ed. United
States: Harvey Miller Publishers–Oxford University Press; 1986.
Edwards JE.Atlas of acquired diseases of the heart and great vessels,
vol. 1. United States of America: Saunders; 1961a.
Edwards JE.Atlas of acquired diseases of the heart and great vessels,
vol. 2. United States of America: Saunders; 1961b.
Edwards JE.Atlas of acquired diseases of the heart and great vessels,
vol. 3. United States of America: Saunders; 1961c.
Gallucci V, Bini RM, Thiene G. Selected topics in cardiac surgery.
Bologna: Pàtron Editore Bologna; 1980.
Gould SE.Patologia Del Cuore E Dei Vasi Sanguigni. Ed. italiana a
cura di Cavallero C.Vol. 1. Padova: Piccin Editore; 1972a.
Gould SE.Patologia Del Cuore E Dei Vasi Sanguigni. Ed. italiana a
cura di Cavallero C.Vol. 2. Padova: Piccin Editore; 1972b.
Lucena JS, García-Pavía P, Suarez-Mier MP, Alonso-Pulpon
LA. Clinico-pathological atlas of cardiovascular pathology.
Switzerland: Springer; 2015.
Masson & Cie, Paris, Roth & Cie, Lausanne editors. Mahaim I.Les
Tumeurs et les polypes du Coeur. Etude anatomo-clinique.
Lausanne. 1945;132(15):959.
McAllister HA, Fenoglio JJ. Tumors of the cardiovascular system.
(Atlas of Tumor Pathology, Second Series, Fascicle 15). Washington
DC: Armed Forces Institute of Pathology; 1978.
McManus BM (vol. editor). Braunwald E (Series editor). Atlas of
cardiovascular pathology for the clinician, 2nd edition. Springer:
China; 2008.
Pomerance A, Davies MJ, editors. The pathology of the heart. Great
Britain: Blackwell Scientic Publications; 1975.
Romero JL, Garcia-Pavia P, Suarez-Mier MP, Alonso-Pulpon L, edi-
tors. Atlas clínico-patológico de enfermedades cardiovasculares.
Barcelona: Esmon Publicidad; 2013.
Schoen FJ. Interventional and surgical cardiovascular pathology.
Clinical correlations and basic principles. United States of America:
Saunders; 1989.
Shenasa M, Hindricks G, Callans DJ, Miller JM, Josephson ME, edi-
tors. Cardiac Mapping. 5th ed. John Wiley & Sons Ltd; 2019.
Silver MD, editor. Cardiovascular pathology, vol. I. 2nd ed. United
Stated of America: Churchill Livingstone; 1991a.
Silver MD, editor. Cardiovascular pathology, vol. II. 2nd ed. United
Stated of America: Churchill Livingstone; 1991b.
Silver MD, Gotlieb AI, Schoen FJ, editors. Cardiovascular pathology.
3rd ed. NewYork: Churchill Livingstone; 2001.
Silver MD.Cardiovascular pathology, vol. 1. United Stated of America:
Churchill Livingstone; 1983a.
Silver MD.Cardiovascular pathology, vol. 2. United Stated of America:
Churchill Livingstone; 1983b.
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cura di Baroldi G, Gallo P, Thiene G.Padova: McGraw-Hill; 1994.
Silver MD.Patologia Cardiovascolare, 2° edizione. Vol 2. Ed. italiana a
cura di Baroldi G, Gallo P, Thiene G.Padova: McGraw-Hill; 1994.
Virmani R, Atkinson JB, Fenoglio JJ.Cardiovascular pathology. United
States of America: Saunders; 1991.
Virmani R, Burke A, Farb A.Atlas of cardiovascular pathology. United
States of America: Saunders; 1996.
Congenital Anomalies oftheCardiac
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Valves
CarlaFrescura andGaetanoThiene
5
Mitral Valve
The congenital malformations of the mitral valve can affect
the mitral leaets and/or the subvalvular apparatus
(Table5.1).
Parachute deformity of the mitral valve is characterized
by the presence of a single papillary muscle to which the
chordae tendineae of both the mitral leaets are connected
(Fig.5.1a, b). The mitral valve looks like a parachute. All
chordae tendineae are usually attached to the posteromedial
papillary muscle, with the anterolateral papillary muscle
hypoplastic or even absent. The leaets and chordae are well
differentiated, and the interchordal spaces represent the
effective valvular orice. If the leaets or tensor apparatus
are dysplastic, the valve appears severely stenotic.
This malformation is rarely isolated. More frequently it is
associated with ventricular septal defect and obstruction of
the aortic arch (Fig.5.1c).
Mitral arcade (known also as the “congenital mitral stenosis”) is a malformation characterized by absent or defective differentiation of the chordae tendineae originating from
the tip of the papillary muscles, with preservation of the
embryonic muscularization (Fig.5.2). The muscularization
of the chordae from the apex of both the papillary muscles to
the anterior leaet gives the valve the look of an arcade mimicking “rheumatic” mitral disease. The leaets lose their
movement and there is absence of the interchordal spaces.
The cleft of the anterior leaet of the mitral valve is rarely
an isolated defect. It can be observed in association with
atrial or ventricular septal defects, tetralogy of Fallot, and
transposition of the great arteries.
The cleft is usually located in the central part of the anterior mitral leaet and is shaped like an inverted “V” with the
apex pointing toward the valvular anulus (Fig. 5.3). The
C. Frescura (*) · G. Thiene
Department of Cardiac, Thoracic, Vascular Sciences and Public
Health, University of Padua Medical School, and Cardiovascular
Pathology Unit, University Hospital of Padua, Padua, Italy
e-mail: gaetano.thiene@unipd.it
extension of the cleft from the free margin of the leaet to the
anulus is variable. The deeper and wider the cleft, the more
the valve results insufcient.
The cleft is also part of partial atrioventricular septal
defect (also known as ostium primum defect), characterized
by interatrial communication and mitral valve insufciency
(Fig.5.4).
Two types of double orice mitral valve can be found: the
“bridge type” and the “hole type.” In the “bridge type,” the
mitral valve is divided into two orices because of the partial
fusion of the anterior and posterior leaets as to form a
brous bridge (Fig.5.5). In the “hole-type” double orice,
there is a variable deciency in the substance of a valvular
leaet (Fig.5.6). Usually, the affected leaet is the anterior
one. In both cases, the accessory orice shows along the circumference the insertion of chordae tendineae arising from
papillary muscles.
In addition, the double orice mitral valve rarely is an
isolated anomaly, more often being associated with atrioventricular or ventricular septal defects and obstruction of the
aortic arch.
The supravalvular mitral ring is a circumferential brous
ring located in the left atrium, a few mm over the mitral valve
and below the mouth of left atrial appendage (Figs.5.7 and
5.8). The ring is associated with a central orice that allows
the transit of blood ow from the atrium to the left ventricle.
Depending upon the diameter of the orice, various degrees
of obstruction may result. The ring reduces the effective orice of the mitral valve.
In some cases, a supravalvular mitral ring is associated
with a parachute mitral valve and aortic isthmic coarctation
(so-called Shone malformation).
Cases exist where the ring is located around the same
mitral leaets (Fig.5.8).
Quite rare is the occurrence of the “Ebstein”-type anomaly of the mitral valve. The “mitral” Ebstein must be distinguished from the Ebstein of the tricuspid valve in hearts with
left-sided tricuspid valve due to the development of an
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
G. Thiene et al. (eds.), Pathology of Cardiac Valve Disease, https://doi.org/10.1007/978-3-031-35498-4_5
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