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Neoplastic Disorders Involving the Aortic Valve
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aortic regurgitation has been studied. Routine transthoracic echocardiography demonstrated
the presence of a circular structure attached to the ventricular surface of the coronary cusp of
the aortic valve, which needed surgery resection. The pathological examination recognized
the lesion as a giant blood cyst [95].
Endocarditis
Valvular endocarditic vegetations are an important differential diagnosis with PFE since
they can be very similar at the echocardiographic evaluation [25, 96]. They have an infectious
origin, so they are generally associated with symptoms of endocarditis and their aspect can
change quite quickly with time and therapies; they can also cause severe damage to the
valvular leaflets. Valvular masses are an important finding in endocarditis, and they are called
“vegetations” in this clinical setting. Depending on the infectious or non-infectious origin of
endocarditis, vegetations can be sterile or not. Infective endocarditis (IE) is a cardiovascular
infection of primary relevance because of the frequent complications on cardiac valves,
devices and extracardiac complications. Etiology can be various, depending on epidemiology
and localization of the vegetation (right or left heart), but in general there is a vast array of
microorganisms that may cause IE: streptococci, staphylococci, enterococci, HACEK
organisms, fungi. Vegetations are more commonly located on the mitral valve (41%),
followed by the aortic valve (38%). The clinical presentation encompasses a broad spectrum
of symptoms: fever, chills, weakness, dyspnea, cough and edema (cause to valve regurgitation
and congestive heart failure), stroke and chest pain (cause to septic embolization). Signs must
be find for the definition of IE with the modified Duke Criteria [25].
TTE imaging can characterize vegetations with sensitivity in the range of 90-100% and
specificity of 90%, but in some cases TEE is necessary [25, 96]. One of the principal
pathogenetic aspects of IE is the prior presence of non-bacterial thrombotic endocarditis
(NBTE) characterized by endothelial disruption, platelet and fibrin deposition especially in
points of cardiac structural abnormality that represent a nidus for subsequent adhesion by
bacteria or fungi in the bloodstream.
NBTE or Marantic Endocarditis refers to the presence of sterile vegetations on the heart
valves that are composed of an amorphous mixture of platelets and fibrin. Other than being
susceptible to infections, these lesions can cause cardiac symptomatology or embolize to the
pulmonary and systemic circulation (spleen, kidney, brain, coronary arteries). The diagnosis
is more likely reached with TEE than with TTE [97–99].
Rheumatoid Nodules
Cardiac involvement in rheumatoid diseases is not uncommon, especially in rheumatoid
arthritis [100]. In most cases, the involvement consists of non-specific inflammatory changes
and specific granuloma formation that usually affects the pericardium. Specific rheumatoid
nodules in the heart are not frequent, but there are cases reporting their localization also on
the aortic valve and causing regurgitation and heart failure [100, 101]. Vegetations are more
common in patients with systemic lupus erythematosus or primary antiphospholipid antibody
syndrome and are called Libman-Sacks vegetations, usually evaluated with TEE [102]. These
lesions are of variable shape and size, generally of soft tissue and located predominantly on
the vessel side of aortic cusps. They have irregular borders, heterogeneous echogenicity and
can complicate with thrombus and embolism or IE.
Angela Pucci, Alessandra Burini, Enrica Manzato et al.
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CONCLUSION
Although aortic valve tumors are rare, mainly benign and often asymptomatic, they may
have severe clinical consequences because of embolic or obstructive phenomena, thus
requiring surgical excision. Malignant primary tumors are only exceptionally reported and are
mainly represented by sarcomas. The aortic valve can also present tumor-like lesions or
pseudo-tumors, including thrombi, Lambl’s excrescences and endocarditic vegetations. Even
though TEE represents the main diagnostic tool and CT and CMR can add useful information
about tumor size, shape, location and characteristics, definitive and differential diagnoses
require histology.
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In: Perspectives in Aortic Valve Disease ISBN: 978-1-53618-769-4
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Editor: Giovanni Concistrè © 2020 Nova Science Publishers, Inc.
Chapter 8
AORTIC ROOT INVOLVEMENT IN CONGENITAL
HEART DEFECTS: SPECIAL SURGICAL TOPICS
Vitali Pak, Elisa Barberi and Duccio Federici
Pediatric Cardiac Surgery, Heart Hospital,
Monasterio Foundation, Massa, Italy
ABSTRACT
The aortic root can be involved in many complex congenital heart diseases. Valvular
anomalies, along with structural diseases of the aortic root, can present themselves as
integral part of the congenital defect or might be the expression of secondary injury
starting from another cardiac lesion. In this chapter we wish to describe three conditions
in which structural and functional anomalies of the aortic root imply special surgical
considerations. Aortic regurgitation secondary to restrictive ventricular septal defect,
aortic valve anomaly in the setting of Truncus Arteriosus and the Aortico-Left
Ventricular Tunnel are here elucidated in terms of pathophysiology, clinical picture and
surgical management. Surgical results, long-term outcomes and current perspectives of
each of these conditions are provided according to the most recent literature.
Keywords: congenital aortic disease, aortic regurgitation, ventricular septal defect, truncus
arteriosus, aortic-left ventricular tunnel
TRUNCUS ARTERIOSUS AND TRUNCAL VALVE REGURGITATION
Vitali Pak
Introduction
Anatomy and Classification
Truncus arteriosus accounts for fewer than 3% of all congenital heart defects. Truncus
arteriosus is an anomaly of the conotruncus and characterized by the presence of one vessel
Corresponding Author’s Email: federici@ftgm.it.
Vitali Pak, Elisa Barberi and Duccio Federici
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arises from the base of both ventricles with single truncal valve, a high large subarterial
ventricular septal defect and by the origination of the pulmonary arteries from the truncus.
Anatomy of the truncus arteriosus includes description of the origin of the pulmonary
arteries, type of ventricular septal defect, morphology of truncal valve, pathway of the
coronary arteries and associated malformation. There are two classification systems in use:
one from Collett and Edwards and another from Van Praagh and Van Praagh. (Figure 1).
Collett and Edwards divide truncus arteriosus in types I–IV. In type I a short main pulmonary
artery arise from the truncus and then bifurcates into a right and left pulmonary artery. In type
II the right and left pulmonary artery arise as separate orifices from posterior aspect of the
truncus. In type III the right and left pulmonary artery arise from the opposite sides of the
truncus. In type IV there is no main pulmonary artery and the lungs are supplied by
aortopulmonary collaterals [1, 2].
Van Praagh and Van Praagh divide truncus arteriosus according to the existence or
absence of the conotruncal septum and define type A as cases with and type B as cases
without ventricular septal defect. In type A1 a common origin of pulmonary arteries from the
truncus. In type A2 two separate right and left branch pulmonary arteries from the dorsal
truncus. In type A3 only one branch pulmonary artery arises from the truncus, the other
orginates from a ductus-like structure from the aortic arch. Type A4, the truncus continues via
a ductus arteriosus into the descending aorta. The right and left pulmonary arteries come off
the truncus before the ductus. The ascending aorta is small. It appears to arise from the main
pulmonary artery. The aortic arch is interrupted [1, 2].
Figure 1. Truncus arteriosus classifications.
Most commonly an intermediate type of these two forms (type I-II and type A1-A2) is
found and the central and peripheral pulmonary arteries are well developed without stenosis
in most clinical cases. Another more practical classification of truncus arteriosus include three
types: truncus arteriosus with confluent pulmonary arteries, truncus arteriosus with absence of
one pulmonary artery and truncus arteriosus with associated interrupted aortic arch or aortic
coarctation.
The ventricular septal defect in truncus arteriosus is located usually under the truncus and
usually separated from the tricuspid valve by the posterior limb of the septal band. Two types
of arrangement can be seen posteriorly. In approximately 80% of patients with truncus
arteriosus, the posterior margin of the defect is muscular, completely separated from the
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