Papillary fibroelastoma | |
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Low magnification micrograph of an excised aortic valve papillary fibroelastoma showing the characteristic avascular branching papillae, H&E stain | |
Specialty | Oncology, cardiology |
A papillary fibroelastoma is a primary tumor of the heart that typically involves one of the heart valves. [1] Papillary fibroelastomas, while considered uncommon, make up about 10 percent of all primary tumors of the heart. [2] They are the third most common type of primary tumor of the heart, [3] behind cardiac myxomas and cardiac lipomas.
A papillary fibroelastoma is generally considered pathologically benign, [4] however outflow obstruction or embolism can be associated with syncope, [5] chest pain, heart attack, stroke [6] [7] and sudden cardiac death.
Symptoms due to papillary fibroelastomas are generally due to either mechanical effects of the tumor or due to embolization of a portion of the tumor to a distal organ. In particular, chest pain or syncope may be due to transient occlusion of the left main coronary artery by the tumor, [8] while a heart attack or sudden cardiac death may be due to embolization of a portion of the tumor into a coronary artery. [9]
Papillary fibroelastoma are typically found and accurately diagnosed by imaging. The diagnosis is confirmed by pathology. Histologically, papillary fibroelastomas have branching avascular papillae, composed of collagen, that are covered by endothelium.[ citation needed ]
If the tumor is found incidentally in an asymptomatic person, the treatment approach is controversial. Certainly a conservative approach is warranted in certain individuals. [10] If the tumor is large, greater than 1 cm in asymptomatic patients, [11] and pedunculated, a case may be made for surgical excision prior to symptoms developing due to the higher risk of embolism. However, this is still considered controversial. [12]
If the papillary fibroelastoma is associated with symptoms, surgical excision is generally recommended for relief of symptoms. [10] A minimally invasive approach may be possible if the tumor involves the aortic valve [13] or right atrium. [14] In the case of aortic valve involvement, excision of the tumor is often valve-sparing, meaning that replacement of the valve with a prosthetic valve is not necessary. Repair of the native valve with a pericardial patch has been described. [15]
Coronary circulation is the circulation of blood in the arteries and veins that supply the heart muscle (myocardium). Coronary arteries supply oxygenated blood to the heart muscle. Cardiac veins then drain away the blood after it has been deoxygenated. Because the rest of the body, and most especially the brain, needs a steady supply of oxygenated blood that is free of all but the slightest interruptions, the heart is required to function continuously. Therefore its circulation is of major importance not only to its own tissues but to the entire body and even the level of consciousness of the brain from moment to moment. Interruptions of coronary circulation quickly cause heart attacks, in which the heart muscle is damaged by oxygen starvation. Such interruptions are usually caused by coronary ischemia linked to coronary artery disease, and sometimes to embolism from other causes like obstruction in blood flow through vessels.
Aortic stenosis is the narrowing of the exit of the left ventricle of the heart, such that problems result. It may occur at the aortic valve as well as above and below this level. It typically gets worse over time. Symptoms often come on gradually with a decreased ability to exercise often occurring first. If heart failure, loss of consciousness, or heart related chest pain occur due to AS the outcomes are worse. Loss of consciousness typically occurs with standing or exercising. Signs of heart failure include shortness of breath especially when lying down, at night, or with exercise, and swelling of the legs. Thickening of the valve without causing obstruction is known as aortic sclerosis.
Interventional radiology (IR) is a medical specialty that performs various minimally-invasive procedures using medical imaging guidance, such as x-ray fluoroscopy, computed tomography, magnetic resonance imaging, or ultrasound. IR performs both diagnostic and therapeutic procedures through very small incisions or body orifices. Diagnostic IR procedures are those intended to help make a diagnosis or guide further medical treatment, and include image-guided biopsy of a tumor or injection of an imaging contrast agent into a hollow structure, such as a blood vessel or a duct. By contrast, therapeutic IR procedures provide direct treatment—they include catheter-based medicine delivery, medical device placement, and angioplasty of narrowed structures.
Chest pain is pain or discomfort in the chest, typically the front of the chest. It may be described as sharp, dull, pressure, heaviness or squeezing. Associated symptoms may include pain in the shoulder, arm, upper abdomen, or jaw, along with nausea, sweating, or shortness of breath. It can be divided into heart-related and non-heart-related pain. Pain due to insufficient blood flow to the heart is also called angina pectoris. Those with diabetes or the elderly may have less clear symptoms.
Mitral regurgitation (MR), also known as mitral insufficiency or mitral incompetence, is a form of valvular heart disease in which the mitral valve is insufficient and does not close properly when the heart pumps out blood. It is the abnormal leaking of blood backwards – regurgitation from the left ventricle, through the mitral valve, into the left atrium, when the left ventricle contracts. Mitral regurgitation is the most common form of valvular heart disease.
A myxoma is a myxoid tumor of primitive connective tissue. It is most commonly found in the heart but can also occur in other locations.
Aortic valve replacement is a cardiac surgery procedure whereby a failing aortic valve is replaced with an artificial heart valve. The aortic valve may need to be replaced because of aortic regurgitation, or if the valve is narrowed by stenosis.
Valvular heart disease is any cardiovascular disease process involving one or more of the four valves of the heart. These conditions occur largely as a consequence of aging, but may also be the result of congenital (inborn) abnormalities or specific disease or physiologic processes including rheumatic heart disease and pregnancy.
Aortic valve repair or aortic valve reconstruction is the reconstruction of both form and function of a dysfunctional aortic valve. Most frequently it is used for the treatment of aortic regurgitation. It can also become necessary for the treatment of aortic aneurysm, less frequently for congenital aortic stenosis.
Cor triatriatum is a congenital heart defect where the left atrium or right atrium is subdivided by a thin membrane, resulting in three atrial chambers.
Primary tumors of the heart are extremely rare tumors that arise from the normal tissues that make up the heart. The incidence of primary cardiac tumors has been found to be approximately 0.02%. This is in contrast to secondary tumors of the heart, which are typically either metastatic from another part of the body, or infiltrate the heart via direct extension from the surrounding tissues. Metastatic tumors to the heart are about 20 times more common than primary cardiac tumors.
A myxoma is a rare benign tumor of the heart. Myxomata are the most common primary cardiac tumor in adults, and are most commonly found within the left atrium near the valve of the fossa ovalis. Myxoma may also develop in the other heart chambers. The tumor is derived from multipotent mesenchymal cells. Cardiac myxoma can affect adults between 30 and 60 years of age.
Myocardial rupture is a laceration of the ventricles or atria of the heart, of the interatrial or interventricular septum, or of the papillary muscles. It is most commonly seen as a serious sequela of an acute myocardial infarction.
Alcohol septal ablation (ASA) is a minimally invasive heart procedure to treat hypertrophic cardiomyopathy (HCM).
Shone's syndrome is a rare congenital heart defect described by Shone in 1963. In the complete form, four left-sided defects are present:
Cholesterol embolism occurs when cholesterol is released, usually from an atherosclerotic plaque, and travels as an embolus in the bloodstream to lodge causing an obstruction in blood vessels further away. Most commonly this causes skin symptoms, gangrene of the extremities and sometimes kidney failure; problems with other organs may arise, depending on the site at which the cholesterol crystals enter the bloodstream. When the kidneys are involved, the disease is referred to as atheroembolic renal disease. The diagnosis usually involves biopsy from an affected organ. Cholesterol embolism is treated by removing the cause and giving supportive therapy; statin drugs have been found to improve the prognosis.
Syncope, commonly known as fainting or passing out, is a loss of consciousness and muscle strength characterized by a fast onset, short duration, and spontaneous recovery. It is caused by a decrease in blood flow to the brain, typically from low blood pressure. There are sometimes symptoms before the loss of consciousness such as lightheadedness, sweating, pale skin, blurred vision, nausea, vomiting, or feeling warm. Syncope may also be associated with a short episode of muscle twitching. Psychiatric causes can also be determined when a patient experiences fear, anxiety, or panic; particularly before a stressful event, usually medical in nature. When consciousness and muscle strength are not completely lost, it is called presyncope. It is recommended that presyncope be treated the same as syncope.
Left atrial appendage occlusion (LAAO), also referred to as left atrial appendage closure (LAAC), is a procedure used to reduce the risk of blood clots from the left atrial appendage entering the bloodstream and causing a stroke in those with non-valvular atrial fibrillation.
Cystic tumour of the atrioventricular nodal region is a very rare tumour of the heart in the region of the atrioventricular node. It is also known as mesothelioma of the atrioventricular node.
Apicoaortic Conduit (AAC), also known as Aortic Valve Bypass (AVB), is a cardiothoracic surgical procedure that alleviates symptoms caused by blood flow obstruction from the left ventricle of the heart. Left ventricular outflow tract obstruction (LVOTO) is caused by narrowing of the aortic valve (aortic stenosis) and other valve disorders. AAC, or AVB, relieves the obstruction to blood flow by adding a bioprosthetic valve to the circulatory system to decrease the load on the aortic valve. When an apicoaortic conduit is implanted, blood continues to flow from the heart through the aortic valve. In addition, blood flow bypasses the native valve and exits the heart through the implanted valved conduit. The procedure is effective at relieving excessive pressure gradient across the natural valve. High pressure gradient across the aortic valve can be congenital or acquired. A reduction in pressure gradient results in relief of symptoms.