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Carcinoid tumors are rare with an incidence of about 4 per 100,000 general population per year. They originate from enterochromaffin cells, which are localized in 70% of cases in the gastrointestinal system and more rarely in the bronchopulmonary system. Carcinoid tumors are characterized by excess secretion of serotonin and other vasoactive substances. The primary tumor is often asymptomatic until hepatic metastases take place, which then produce…

Immune-mediated systemic diseases can create valvular lesions, particularly systemic lupus erythematosus, primary antiphospholipid syndrome, rheumatoid arthritis, and ankylosing spondylitis. SYSTEMIC LUPUS ERYTHEMATOSUS Systemic lupus erythematosus is an immune complex–mediated inflammatory disease that can cause a variety of cardiac manifestations including valvular heart disease, coronary artery disease, myocarditis, and pericarditis. Valvular lesions, characterized by leaflet thickening and fibrosis, involve predominantly the aortic and mitral valves. Echocardiography typically…

Primary cardiac valve tumors are rare and comprise about 10% of all cardiac tumors, which are rare themselves. The most common tumors are papillary fibroelastoma followed by myxoma and fibroma . Extremely rare are hemangioma, sarcoma, and histiocytoma. In asymptomatic patients the diagnosis is suspected often incidentally during a systematic echocardiography. In symptomatic patients, stroke or myocardial infarction attributable to peripheral embolization and congestive heart failure…

Although rare, various types of trauma can be responsible for valvular lesions that may not be recognized unless a systematic search is conducted after major penetrating or blunt trauma or following accidents. Traumatic valve injury can also be manifested as iatrogenic lesions resulting from surgery or interventional cardiology. PATHOLOGY AND CLINICAL PRESENTATION Penetrating cardiac trauma can affect the right ventricle, the left ventricle, and the great…

Endomyocardial fibrosis is characterized by progressive fibrosis and thickening of the ventricular endocardium that result in restrictive cardiomyopathy. The prevalence of this disease is greater in tropical and subtropical countries where parasitosis is frequently encountered. Endomyocardial fibrosis can also be encountered in temperate countries and is then associated with hypereosinophilia as part of Löffler's syndrome. The disease process involves the left (30%), the right (10%), or…

Hypertrophic cardiomyopathy is a disease of the left and/or right ventricle characterized by the development of myocardial hypertrophy predominant at the interventricular septum. There is no direct cause, such as volume or pressure overload. In most instances, the disease is genetically transmitted in an autosomal dominant manner. Ventricular hypertrophy is often asymmetrical and its localization and severity influence clinical manifestations. In the majority of cases, ventricular…

The World Health Organization restricts the use of the term “dilated cardiomyopathy” to diseases of unknown cause that primarily involve the myocardium. The term “idiopathic cardiomyopathy” would be more accurate to define a myocardial disease with no discernible cause using the current state of knowledge, while the term “dilated cardiomyopathy” refers to a cardiac remodeling process ultimately increasing the volume of the ventricular chambers with significant…

The term “ischemic valvular disease” defines valve regurgitation due to ventricular ischemia or ventricular infarction is classified by the term “ischemic valvular disease.” This definition excludes preexisting valvular diseases in patients presenting with coronary artery disease. The leaflet tissue often appears normal. For this reason, the valve dysfunction is sometimes called “functional regurgitation,” a terminology that might suggest a potential reversibility of the regurgitation (which is…

Extensive calcification of the mitral valve annulus is a pathological entity that may or may not be associated with other mitral valve diseases. In its most characteristic configuration, it forms a semilunar deposit of calcium within the posterior annulus with limited extension to the leaflet tissue. This process differs from calcification in rheumatic valve disease, which usually involves the commissures and the leaflet tissue with only…

Degenerative valvular diseases are the main cause of mitral valve dysfunction in developed countries today and the “pain quotidien” * * See Glossary . of the valvular surgeon. They are also the source of regrettable confusion; this is because they are often grouped under the general term “mitral valve prolapse,” which for some authors defines a specific disease, for others a group of several diseases, and…

Before 1950 rheumatic fever was one of the most common epidemics in the world, with considerable socioeconomic consequences. As an example, acute rheumatic fever was the most common cause of draft rejection in the United States Armed Forces during World War II. Dramatic changes took place when in the mid-1940s sulfanilamides were found to be efficient in the treatment of streptococcal pharyngitis, the cause of rheumatic…

Tricuspid valve malformations are very rare. The most frequent is the anomaly described by Ebstein in 1866, which represents only 0.5% of patients with congenital heart disease. Other tricuspid valve malformations are exceptional. They include annular dilatation, papillary muscle elongation, leaflet agenesis, absence of papillary muscle, or “parachute” * * See Glossary . valve. Tricuspid valve malformations rarely require surgery within the first years of life.…

Mitral valvular malformations are rare, diverse, and complex. They are often associated with other cardiac or great vessel anomalies that may hide or be hidden by the valvular malformation. They may also be encountered as a persisting anomaly when other, more dominant malformations have been corrected. “Formes frustes” * * See Glossary . are frequent and usually not diagnosed during childhood; instead, they are discovered after…

Several repair techniques have been described to correct aortic valve dysfunction. In contrast to mitral valve repair techniques, few of them have become the gold standard because of the unpredictability of their results. This is primarily attributable to the small amount of tissue and consequent small surface of coaptation present in the aortic leaflets, as compared to the large amount of tissue found in the mitral…

The same diseases that affect the mitral and tricuspid valves may also affect the aortic valve. According to the pathophysiological triad ( Table 21-1 ), a clear distinction should be made between etiology, lesions, and dysfunctions. TABLE 21-1 Pathophysiological Triad Etiology —The cause of the disease ↓ Lesions —Result from the disease ↓ Dysfunctions —Result from the lesions ETIOLOGY Aortic valve diseases involve either the leaflets…

The aortic valve is one component of the aortic root, which connects the left ventricular outflow tract to the ascending aorta. The function of the aortic root is to guide the unidirectional flow of large volumes of blood with minimal resistance while maintaining laminar flow. Another function is to optimize coronary blood flow. These functions require complex dynamic interactions between different anatomical components that may be…

Reconstruction of the tricuspid valve should be guided by the same basic principles described for the mitral valve—that is, preserving or restoring normal leaflet motion, ensuring a large surface of leaflet coaptation, and remodeling the dilated and deformed annulus. The three fundamental principles of tricuspid valve reconstruction are: 1. Restore or preserve full leaflet mobility. 2. Provide a large surface of leaflet coaptation. 3. Remodel and…

The pathophysiological triad introduced for the mitral valve ( Chapter 6 ) is also relevant to the other valves, including the tricuspid valve. It provides an accurate description of the valve pathophysiology by a clear separation between etiology, lesions, and dysfunctions ( Table 18-1 ). TABLE 18-1 Pathophysiological Triad Etiology — The cause of the disease ↓ Lesions — Result from the disease ↓ Dysfunctions —…

The tricuspid valve is part of a complex functional system that also includes the right atrium, the right ventricle, and the pulmonary circulation. With the tricuspid valve exposed, the surgeon observes sequentially the right atrial cavity, the atrio-valvular junction, the tricuspid leaflets, and the subvalvular apparatus. RIGHT ATRIUM The right atrium consists of a curved posterior groove continuous with the superior and inferior vena cavae, a…