Papillary fibroelastoma

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Papillary fibroelastoma
Papillary fibroelastoma.jpg
Low magnification micrograph of an excised aortic valve papillary fibroelastoma showing the characteristic avascular branching papillae, H&E stain
Specialty Oncology, cardiology   OOjs UI icon edit-ltr-progressive.svg

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.

Contents

Signs and symptoms

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]

Diagnosis

Micrograph of an excised aortic valve papillary fibroelastoma showing that the avascular branching papillae are covered by endothelium. H&E stain. Papillary fibroelastoma2.jpg
Micrograph of an excised aortic valve papillary fibroelastoma showing that the avascular branching papillae are covered by endothelium. H&E stain.

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 ]

Treatment

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]

See also

Related Research Articles

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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.

<span class="mw-page-title-main">Aortic stenosis</span> Narrowing of the exit of the hearts left ventricle

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.

<span class="mw-page-title-main">Interventional radiology</span> Medical subspecialty

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.

<span class="mw-page-title-main">Chest pain</span> Discomfort or pain in the chest as a medical symptom

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.

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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.

<span class="mw-page-title-main">Myxoma</span> Myxoid tumor of primitive connective tissue

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<span class="mw-page-title-main">Valvular heart disease</span> Disease in the valves of the heart

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<span class="mw-page-title-main">Cardiac myxoma</span> Medical condition

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<span class="mw-page-title-main">Myocardial rupture</span> Medical condition

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<span class="mw-page-title-main">Cholesterol embolism</span> Medical condition

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.

<span class="mw-page-title-main">Syncope (medicine)</span> Transient loss of consciousness and postural tone

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.

<span class="mw-page-title-main">Left atrial appendage occlusion</span> Medical treatment

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.

<span class="mw-page-title-main">Cystic tumour of the atrioventricular nodal region</span> Medical condition

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References

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  4. Kumbala D, Sharp T, Kamalesh M (2008). ""Perilous pearl"--papillary fibroelastoma of aortic valve: a case report and literature review". Angiology. 59 (5): 625–8. doi:10.1177/0003319707305986. PMID   18388078. S2CID   22877350.
  5. Maestroni A, Zecca B, Triggiani M (2006). "Cardiac papillary fibroelastoma presenting with acute coronary syndrome and syncope". Acta Cardiol. 61 (3): 363–5. doi:10.2143/AC.61.3.2014843. PMID   16869462. S2CID   25041146.
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  7. Kalavakunta, Jagadeesh K.; Peddi, Prashanth; Bantu, Viswaroop; Tokala, Hemasri; Kodenchery, Mihas (2010-09-01). "Lambl's excrescences: a rare cause of stroke". The Journal of Heart Valve Disease. 19 (5): 669–670. ISSN   0966-8519. PMID   21053748.
  8. Kalavakunta, Jagadeesh K.; Martin, David; Gupta, Vishal (2011-01-01). "A rare location and presentation of papillary fibroelastoma". The American Heart Hospital Journal. 9 (2): 114–115. doi: 10.15420/ahhj.2011.9.2.114 . ISSN   1541-9215. PMID   24839649.
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  10. 1 2 Mutlu H, Demir IE, Leppo J, Levy WK (2008). "Nonsurgical Management of a Left Ventricular Pedunculated Papillary Fibroelastoma: A Case Report". J Am Soc Echocardiogr. 21 (7): 877.e4–7. doi:10.1016/j.echo.2007.10.001. PMID   18191538.
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