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 valves of the heart. Papillary fibroelastomas, while considered generally rare, make up about 10 percent of all primary tumors of the heart. [1] They are the third most common type of primary tumor of the heart, [2] behind cardiac myxomas and cardiac lipomas.
A papillary fibroelastoma is generally considered pathologically benign, [3] however outflow obstruction or embolism can be associated with syncope, [4] chest pain, heart attack, stroke [5] [6] 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, [7] while a heart attack or sudden cardiac death may be due to embolization of a portion of the tumor into a coronary artery. [8]
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. [9] If the tumor is large, greater than 1 cm in asymptomatic patients, [10] 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. [11]
If the papillary fibroelastoma is associated with symptoms, surgical excision is generally recommended for relief of symptoms. [9] A minimally invasive approach may be possible if the tumor involves the aortic valve [12] or right atrium. [13] 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. [14]
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 narrowing is known as aortic sclerosis.
Aortic dissection (AD) occurs when an injury to the innermost layer of the aorta allows blood to flow between the layers of the aortic wall, forcing the layers apart. In most cases, this is associated with a sudden onset of severe chest or back pain, often described as "tearing" in character. Also, vomiting, sweating, and lightheadedness may occur. Other symptoms may result from decreased blood supply to other organs, such as stroke, lower extremity ischemia, or mesenteric ischemia. Aortic dissection can quickly lead to death from insufficient blood flow to the heart or complete rupture of the aorta.
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.
Aneurysm of the aortic sinus, also known as the sinus of Valsalva, is a rare abnormality of the aorta, the largest artery in the body. The aorta normally has three small pouches that sit directly above the aortic valve, and an aneurysm of one of these sinuses is a thin-walled swelling. Aneurysms may affect the right (65–85%), non-coronary (10–30%), or rarely the left coronary sinus. These aneurysms may not cause any symptoms but if large can cause shortness of breath, palpitations or blackouts. Aortic sinus aneurysms can burst or rupture into adjacent cardiac chambers, which can lead to heart failure if untreated.
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.
Libman–Sacks endocarditis (LSE) is a form of non-bacterial endocarditis that is seen in association with systemic lupus erythematosus (SLE), antiphospholipid syndrome, and malignancies. It is one of the most common heart-related manifestations of lupus.
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.
The mini-maze procedures are cardiac surgery procedures intended to cure atrial fibrillation (AF), a common disturbance of heart rhythm. They are procedures derived from the original maze procedure developed by James Cox, MD.
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. Myxomata 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 percutaneous, minimally invasive procedure performed by an interventional cardiologist to relieve symptoms and improve functional status in eligible patients with severely symptomatic hypertrophic cardiomyopathy (HCM) who meet strict clinical, anatomic and physiologic selection criteria. In carefully selected patients, when performed by an experienced interventional cardiologist, the procedure is successful in relieving symptoms in over 90% of patients.
Shone's syndrome is a rare congenital heart disease 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, 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 treatment strategy to reduce the risk of left atrial appendage blood clots from entering the bloodstream and causing a stroke in patients with non-valvular atrial fibrillation (AF).
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 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.