Cardiac fibroma, also known as cardiac fibromatosis, cardiac fibrous hamartoma, fibroelastic hamartoma of heart and fibroma of heart is the second highest type of primary cardiac tumor seen in infants and children. This

benign tumor A benign tumor is a mass of Cell (biology), cells (tumor) that does not Cancer invasion, invade neighboring tissue or Metastasis, metastasize (spread throughout the body). Compared to Cancer, malignant (cancerous) tumors, benign tumors generally ...

made by

connective tissue Connective tissue is one of the four primary types of animal tissue, a group of cells that are similar in structure, along with epithelial tissue, muscle tissue, and nervous tissue. It develops mostly from the mesenchyme, derived from the mesod ...

and

fibroblast A fibroblast is a type of cell (biology), biological cell typically with a spindle shape that synthesizes the extracellular matrix and collagen, produces the structural framework (Stroma (tissue), stroma) for animal Tissue (biology), tissues, and ...

is largely observed in the ventricles of the

heart The heart is a muscular Organ (biology), organ found in humans and other animals. This organ pumps blood through the blood vessels. The heart and blood vessels together make the circulatory system. The pumped blood carries oxygen and nutrie ...

. The left ventricle is the most common location of cardiac fibroma and accounts for approximately 57% of cardiac fibroma cases followed by the right ventricle with 27.5% of cases.

Symptom Signs and symptoms are diagnostic indications of an illness, injury, or condition. Signs are objective and externally observable; symptoms are a person's reported subjective experiences. A sign for example may be a higher or lower temperature ...

s of the disease depend on the size of the tumor, its location relative to the conduction system, and whether it obstructs

blood flow Hemodynamics American and British English spelling differences#ae and oe, or haemodynamics are the Fluid dynamics, dynamics of blood flow. The circulatory system is controlled by homeostasis, homeostatic mechanisms of autoregulation, just as hydrau ...

. Two-thirds of children with this tumor are

asymptomatic Asymptomatic (or clinically silent) is an adjective categorising the medical conditions (i.e., injuries or diseases) that patients carry but without experiencing their symptoms, despite an explicit diagnosis (e.g., a positive medical test). P ...

, showing no signs and symptoms. Therefore the cause of cardiac fibroma is unexplained but has been associated with

Gorlin Syndrome Gorlin may refer to: People * Dan Gorlin, computer game programmer, designer and founder of Dan Gorlin Productions * Eitan Gorlin, filmmaker, author and actor * Mikhail Gorlin, Russian emigre poet * Richard Gorlin, American cardiologist, co-devel ...

Echocardiography Echocardiography, also known as cardiac ultrasound, is the use of ultrasound to examine the heart. It is a type of medical imaging, using standard ultrasound or Doppler ultrasound. The visual image formed using this technique is called an ec ...

is the primarily

diagnostic Diagnosis (: diagnoses) is the identification of the nature and cause of a certain phenomenon. Diagnosis is used in a lot of different academic discipline, disciplines, with variations in the use of logic, analytics, and experience, to determine " ...

method used to detect if an individual has cardiac fibroma. Resection of the tumor is recommended however heart transplantation is done if surgery is not possible. Overall prognosis of resection is favorable and the chance of recurrence is scarcely reported.

Signs and symptoms

Signs and symptoms in cardiac fibroma are nonspecific, some individuals experience

arrhythmias Arrhythmias, also known as cardiac arrhythmias, are irregularities in the heartbeat, including when it is too fast or too slow. Essentially, this is anything but normal sinus rhythm. A resting heart rate that is too fast – above 100 beats ...

dyspnea Shortness of breath (SOB), known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing discomfort that ...

cyanosis Cyanosis is the change of Tissue (biology), tissue color to a bluish-purple hue, as a result of decrease in the amount of oxygen bound to the hemoglobin in the red blood cells of the capillary bed. Cyanosis is apparent usually in the Tissue (bi ...

, chest-pain and sudden mortality. In addition, the majority of individuals with cardiac fibroma are asymptomatic therefore leading to signs and symptoms that depend on the size and location of the tumor. Tumors range from millimeters to extensive tumors in the range of 12.5 x 10.0 x 8.5 cm. For instance, individuals with larger tumors due to cardiac fibroma which include the conduction system of the heart can demonstrate cardiac dysrhythmias as a symptom.

Cause

The cause of cardiac fibroma is still unknown. However, some tumors have been found to be associated with

Gorlin syndrome Gorlin may refer to: People * Dan Gorlin, computer game programmer, designer and founder of Dan Gorlin Productions * Eitan Gorlin, filmmaker, author and actor * Mikhail Gorlin, Russian emigre poet * Richard Gorlin, American cardiologist, co-devel ...

which is also known as

Nevoid Basal Cell Carcinoma Syndrome Nevoid basal-cell carcinoma syndrome (NBCCS) is a rare inherited medical condition involving defects within multiple body systems such as the skin, nervous system, eyes, endocrine system, and bones. People with NBCCS are prone to developing vario ...

(NBCCS). NBCCS is a

genetic condition A genetic disorder is a health problem caused by one or more abnormalities in the genome. It can be caused by a mutation in a single gene (monogenic) or multiple genes (polygenic) or by a chromosome abnormality. Although polygenic disorders are ...

that affects various parts of the body and increases the possibility of acquiring distinct cancerous and non cancerous tumors.

Mechanism

The mechanism behind cardiac

fibroma Fibromas are benign tumors that are composed of fibrous or connective tissue. They can grow in all organs, arising from mesenchyme tissue. The term "fibroblastic" or "fibromatous" is used to describe tumors of the fibrous connective tissue. Wh ...

s is still unclear. Fibromas have a

homogeneous Homogeneity and heterogeneity are concepts relating to the uniformity of a substance, process or image. A homogeneous feature is uniform in composition or character (i.e., color, shape, size, weight, height, distribution, texture, language, i ...

mass of

s mixed with an abundance of

collagen Collagen () is the main structural protein in the extracellular matrix of the connective tissues of many animals. It is the most abundant protein in mammals, making up 25% to 35% of protein content. Amino acids are bound together to form a trip ...

and

elastic fiber Elastic fibers (or yellow fibers) are an essential component of the extracellular matrix composed of bundles of proteins (elastin) which are produced by a number of different cell types including fibroblasts, endothelial, smooth muscle, and ai ...

s. These masses represent

mesenchymal Mesenchyme () is a type of loosely organized animal embryonic connective tissue of undifferentiated cells that give rise to most tissues, such as skin, blood, or bone. The interactions between mesenchyme and epithelium help to form nearly ever ...

growth, but lack other mesenchymal elements, such as

blood vessel Blood vessels are the tubular structures of a circulatory system that transport blood throughout many Animal, animals’ bodies. Blood vessels transport blood cells, nutrients, and oxygen to most of the Tissue (biology), tissues of a Body (bi ...

cartilage Cartilage is a resilient and smooth type of connective tissue. Semi-transparent and non-porous, it is usually covered by a tough and fibrous membrane called perichondrium. In tetrapods, it covers and protects the ends of long bones at the joints ...

bone, and muscle. These masses often entrap

cardiomyocytes Cardiac muscle (also called heart muscle or myocardium) is one of three types of vertebrate muscle tissues, the others being skeletal muscle and smooth muscle. It is an involuntary, striated muscle that constitutes the main tissue of the wall of ...

, which are muscle cells that make up the cardiac muscle; cells decrease while collagen content increase in these masses. The growth of cardiac fibromas are also slow and produce detrimental physical effects. This is due to infiltration and replacement of myocardium which protrude into the cavity of the heart. These tumors usually occur within the anterior wall of the left ventricle or the

interventricular septum The interventricular septum (IVS, or ventricular septum, or during development septum inferius) is the stout wall separating the ventricle (heart), ventricles, the lower chambers of the heart, from one another. The interventricular septum is di ...

and rarely involves the

right ventricle A ventricle is one of two large chambers located toward the bottom of the heart that collect and expel blood towards the peripheral beds within the body and lungs. The blood pumped by a ventricle is supplied by an atrium (heart), atrium, an adjace ...

Diagnosis

Early and precise diagnosis of cardiac fibroma can lead to healing and avoidance of

surgery Surgery is a medical specialty that uses manual and instrumental techniques to diagnose or treat pathological conditions (e.g., trauma, disease, injury, malignancy), to alter bodily functions (e.g., malabsorption created by bariatric surgery s ...

is the preferred initial method of diagnosis for cardiac fibroma. This is due to the exams non-invasiveness, reduced cost and extensive availability. Echocardiography depicts the morphologic appearance, region and motion of the tumor. On the other hand, additional examinations such as

Computed Tomography A computed tomography scan (CT scan), formerly called computed axial tomography scan (CAT scan), is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers or ...

(CT) and

Magnetic Resonance Imaging Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and ...

(MRI) are also conducted for additional diagnostic information. The CT exam provides precise

spatial resolution In physics and geosciences, the term spatial resolution refers to distance between independent measurements, or the physical dimension that represents a pixel of the image. While in some instruments, like cameras and telescopes, spatial resoluti ...

of the tumor meanwhile an MRI provides a comprehensive overview of tissue characterization and does not expose the patient to

radiation In physics, radiation is the emission or transmission of energy in the form of waves or particles through space or a material medium. This includes: * ''electromagnetic radiation'' consisting of photons, such as radio waves, microwaves, infr ...

. Tumor

biopsy A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, an interventional radiologist, or an interventional cardiology, interventional cardiologist. The process involves the extraction of sampling (medicine), sample ...

is utilized in cardiac fibroma as the

gold standard A gold standard is a backed currency, monetary system in which the standard economics, economic unit of account is based on a fixed quantity of gold. The gold standard was the basis for the international monetary system from the 1870s to the ...

to confirmation a patient diagnosis with cardiac fibroma. Family medical history and

physical examination In a physical examination, medical examination, clinical examination, or medical checkup, a medical practitioner examines a patient for any possible medical signs or symptoms of a Disease, medical condition. It generally consists of a series of ...

of an individual is also used as a diagnostic method, close examination of the heart is concluded along with careful detection of any abnormal heart sounds.

Treatment

Cardiac fibroma is commonly treated through

surgical excision Surgery is a medical specialty that uses manual and instrumental techniques to diagnose or treat pathological conditions (e.g., trauma, disease, injury, malignancy), to alter bodily functions (e.g., malabsorption created by bariatric surgery ...

procedures. The removal of the cardiac tumor requires open heart surgery. During surgery, the tumor and tissues around it is removed to reduce the risk of the tumor returning. Recovery after surgery is between 4–5 days in the hospital and 6 weeks in total. An

echocardiogram Echocardiography, also known as cardiac ultrasound, is the use of ultrasound to examine the heart. It is a type of medical imaging, using standard ultrasound or Doppler ultrasound. The visual image formed using this technique is called an echo ...

is taken every year after the surgery to make sure the tumor has not returned or formed any new growth. On the other hand, if surgery is not possible, a

heart transplantation A heart transplant, or a cardiac transplant, is a surgical transplant procedure performed on patients with end-stage heart failure when other medical or surgical treatments have failed. , the most common procedure is to take a functioning heart ...

is required.

Prognosis

Cardiac fibromas in infancy which are unable to be removed surgically due to size and extensive

myocardial Cardiac muscle (also called heart muscle or myocardium) is one of three types of vertebrate muscle tissues, the others being skeletal muscle and smooth muscle. It is an involuntary, striated muscle that constitutes the main tissue of the wall of ...

infiltration, have a poor prognosis. The is due to the high ratio of tumor-to-heart size that may produce low

cardiac output In cardiac physiology, cardiac output (CO), also known as heart output and often denoted by the symbols Q, \dot Q, or \dot Q_ , edited by Catherine E. Williamson, Phillip Bennett is the volumetric flow rate of the heart's pumping output: tha ...

and as a result lead to a poorer

prognosis Prognosis ( Greek: πρόγνωσις "fore-knowing, foreseeing"; : prognoses) is a medical term for predicting the likelihood or expected development of a disease, including whether the signs and symptoms will improve or worsen (and how quickly) ...

. As a result,

defibrillator Defibrillation is a treatment for life-threatening cardiac arrhythmias, specifically ventricular fibrillation (V-Fib) and non-perfusing ventricular tachycardia (V-Tach). Defibrillation delivers a dose of electric current (often called a ''count ...

implantation or cardiac transplantation may be required. However, tumors that are able to be surgically removed even incompletely have a good prognosis. Long-term survival after surgical resection is favorable, and the chance of recurrence is scarcely reported.

Epidemiology

Cardiac fibromas account for 12%-16% of primary cardiac tumors in children. Therefore, leading it to be the second most common benign cardiac tumor in children. However, it has rarely been found in adults but mainly found in infants under the age of one.

Research directions

Literature Survey on Epidemiology and Pathology of Cardiac Fibroma: During this study,

research Research is creative and systematic work undertaken to increase the stock of knowledge. It involves the collection, organization, and analysis of evidence to increase understanding of a topic, characterized by a particular attentiveness to ...

ers searched through the literature

database In computing, a database is an organized collection of data or a type of data store based on the use of a database management system (DBMS), the software that interacts with end users, applications, and the database itself to capture and a ...

s on cardiac fibroma to find factors that predict poor outcomes that lead to

death Death is the end of life; the irreversible cessation of all biological functions that sustain a living organism. Death eventually and inevitably occurs in all organisms. The remains of a former organism normally begin to decompose sh ...

. Researchers found that patients who did not survive were significantly younger than those who did survive. These results suggest that younger individuals diagnosed with cardiac fibroma are associated with a poorer outcome. Researchers found no significant difference between the maximum

diameter In geometry, a diameter of a circle is any straight line segment that passes through the centre of the circle and whose endpoints lie on the circle. It can also be defined as the longest Chord (geometry), chord of the circle. Both definitions a ...

of the

tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

between age groups. However, although younger individuals have smaller

s, the high ratio of tumor-to-heart sizes may generate low

, which leads to a poor outcome. In addition, literature revealed that 18 of 178 patients with cardiac fibroma were diagnosed during

prenatal Prenatal development () involves the embryonic development, development of the embryo and of the fetus during a viviparity, viviparous animal's gestation. Prenatal development starts with fertilization, in the germinal stage of embryonic develop ...

and

neonatal In common terminology, a baby is the very young offspring of adult human beings, while infant (from the Latin word ''infans'', meaning 'baby' or 'child') is a formal or specialised synonym. The terms may also be used to refer to Juvenile (orga ...

periods, resulting in the tumor having a certain size regardless of the child's age. These findings suggest that cardiac fibromas may be a

congenital disorder A birth defect is an abnormal condition that is present at childbirth, birth, regardless of its cause. Birth defects may result in disability, disabilities that may be physical disability, physical, intellectual disability, intellectual, or dev ...

. Successful Surgical Excision of a Large Cardiac Fibroma in an Asymptomatic Child: A 3-year-old girl, who was

, underwent a successful

of a large cardiac fibroma. The individual had frequent

cough A cough is a sudden expulsion of air through the large breathing passages which can help clear them of fluids, irritants, foreign particles and Microorganism, microbes. As a protective reflex, coughing can be repetitive with the cough reflex fol ...

s, and therefore underwent several diagnostic exams to diagnose the cause of her frequent cough. A cardiac mass was found on the

echocardiograph Echocardiography, also known as cardiac ultrasound, is the use of ultrasound to examine the heart. It is a type of medical imaging, using standard ultrasound or Doppler ultrasound. The visual image formed using this technique is called an echoc ...

and later was confirmed by magnetic resonance imaging (

MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and rad ...

). The cardiac mass was monitored and after 24 hours, it showed

sinus rhythm A sinus rhythm is any cardiac rhythm in which depolarisation of the cardiac muscle begins at the sinus node. It is necessary, but not sufficient, for normal electrical activity within the heart. On the electrocardiogram (ECG), a sinus rhythm ...

s of normal variability. In addition, the mass dimensions were 38 X 28 mm in the apical area of the left ventricle. A surgical procedure was recommended due to the risk of ventricular arrhythmias and sudden

cardiac death Clinical death is the medical term for cessation of blood circulation and breathing, the two criteria necessary to sustain the lives of human beings and of many other organisms. It occurs when the heart stops beating in a regular rhythm, a conditio ...

. The surgery was a success and physicians were able to remove the entire tumor without any complications. Follow-up evaluations at six-months and a year showed the patient in good health and no signs of tumor recurrence. Primary Cardiac Tumors in Children: A Center's Experience: The Department of Cardiac Surgery Children's Hospital in China conducted a study to analyze different characteristics and outcomes of

pediatric Pediatrics (American English) also spelled paediatrics (British English), is the branch of medicine that involves the medical care of infants, children, adolescents, and young adults. In the United Kingdom, pediatrics covers many of their youth ...

patients who have primary cardiac tumors treated in their center. They had sixteen patients with primary cardiac tumors between the ages of 1–13 years. All patients were diagnosed by echocardiography, MRI, and CT. 15 of the 16 patients were able to get successful remove of their mass and one patient had partial resection. However, one patient did die during surgery due to low cardiac output

syndrome A syndrome is a set of medical signs and symptoms which are correlated with each other and often associated with a particular disease or disorder. The word derives from the Greek language, Greek σύνδρομον, meaning "concurrence". When a sy ...

after five days of their initial surgery to remove their mass. The

pathological Pathology is the study of disease. The word ''pathology'' also refers to the study of disease in general, incorporating a wide range of biology research fields and medical practices. However, when used in the context of modern medical treatme ...

examination of the cardiac masses showed that rhabdomyoma is the most frequent tumor in children, followed by

myxoma A myxoma (New Latin from Ancient Greek, Greek 'mucus') is a myxoid tumor of primitive connective tissue. It is most commonly found in the heart (and is the most common primary tumor of the heart in adults) but can also occur in other locations ...

and fibromas;

morbidity A disease is a particular abnormal condition that adversely affects the structure or function of all or part of an organism and is not immediately due to any external injury. Diseases are often known to be medical conditions that are asso ...

of rhabdomyomas and fibromas were reported higher in infancy, while myxomas were more frequent in older children.

References

External links

{{Heart and thymus neoplasia Pediatrics Heart neoplasia