Cardiac Fibroma - Causes, Symptoms, and Treatment

Introduction

Cardiac fibromas are also referred to as cardiac fibromatosis, which can be understood in simple words as cardiac tumors. Cardiac tumors come under the category of being rare in the population. Approximately 75 percent of these are benign tumors, and the incidence rate is roughly 0.32 percent. Cardiac fibromas usually manifest in children, with most of the cases being reported in infants or even before birth. They are the second most common cardiac anomaly in fetuses because they can be diagnosed at the prenatal stage. Cardiac fibroma occurs predominantly in males as compared to females. Such tumors reveal signs and symptoms only if there is an obstruction of blood flow in the cardiovascular system, leading to the formation of a coronary artery compromise, conduction defects, and thromboembolic events.

What Is Cardiac Fibroma?

Cardiac fibroma is a benign or noncancerous tumor in nature and one of the most frequently diagnosed intracardiac tumors in children. This benign proliferation is commonly seen in children but can show itself in adults. Additionally, a tumor of this kind can cause interference with the general functioning of the heart and the entire circulatory system by obstructing blood flow to vital organs of the body. Such blockages and obstructions may result in an abnormal heart rhythm known as arrhythmia. Cardiac fibroma is generally found in the left ventricle of the heart. This mass of tissue can be huge. Sometimes, the size can almost equal the size of the heart. Cardiac fibroma can be of genetic origin in cases of Gorlin syndrome. Gorlin syndrome is an autosomal dominant disease caused by mutations in a particular gene. Characteristics features of the syndrome include unusual brain tumors, macrocephaly (abnormally enlarged head in babies), and skeletal abnormalities.

What Causes Cardiac Fibroma?

The exact cause and etiology of cardiac fibroma are still unknown. There are several ongoing trials to get to the root cause of cardiac fibroma. Specific reasons are still under investigation, but they may prove to cause cardiac fibroma.

• Nevoid basal cell carcinoma (a genetic disorder characterized by the development of multiple basal cell carcinomas, a type of skin cancer).

• Heart fibroblasts (a type of connective tissue in the heart).

• Interventricular fibroblasts (fibroblast cells found in the interventricular septum of the heart).

• Genetic influence.

• Melanoma (skin cancer).

• Breast cancer.

• Lung cancer.

• Environment risk factors.

• Exposure to solid radioactivity.

• Mutation.

How to Diagnose Cardiac Fibroma?

Fibromas generally present at a very young age or can even occur before birth. They are rarely seen in an ultrasound. Diagnosis becomes clear if clinical features are evident clinically. Specific diagnostic tools and evaluation methods are mentioned below.

• CT imaging or computed tomography shows an abnormal appearance of the heart in case there is the presence of a fibroma. In around 25 percent of cases, calcification is seen, and a change in texture may be seen after the contrast administration. Cardiac fibromas appear as a homogeneous mass of well-demarcated soft tissue, but infiltration is also visible.

• Magnetic resonance imaging appears hypointense in the image on essential pulse imaging in case there is the presence of fibroma.

• On echocardiography, cardiac fibroma shows a homogeneous texture. It appears to be brighter than the surrounding tissue of the myocardium. There may be the presence of specks of calcium that give a shiny appearance to the fibroma.

• PET or positron imaging radiography

• Electrocardiogram.

• Histological studies.

• Chest X-rays.

• Intraoperative biopsy.

What Are the Signs and Symptoms of Cardiac Fibroma?

The heart is divided into four chambers. The top two chambers are the left and right atrium, and the lower two are called the left and right ventricles. Cardiac fibroma is most commonly seen in the left ventricle and the right ventricle. An increase in the size of cardiac fibroma is seen along with physiological cardiac growth. It stops at the time of complete development of the heart, approximately at the age of 17 to 20 years. The low cardiac output and abnormalities in the heart rhythm result in a poor prognosis. The classical signs and symptoms of cardiac fibroma are mentioned below.

• Tachycardia (increased heart rate).

• Bradycardia (decreased heart rate).

• Difficulty in breathing.

• Shortness of breath.

• Chest pain.

• Bluish discoloration of the skin.

• Abnormal pulse rates.

• Palpitations.

• Fatigue.

• Fainting.

• Swelling in the lungs.

• Imbalance of metabolic fluids.

• Collection of fluid in the lungs and other vital organs.

• Bronchitis.

• Sudden cardiac failure.

How to Treat Cardiac Fibroma?

The exact treatment modality for cardiac fibroma is not well defined as of today. There are still several pieces of research that are currently going on to develop a sure-shot management plan post-diagnosis of cardiac fibroma. Surgical intervention is a treatment of choice, which is done with a multidisciplinary approach. Other treatment options that are being studied are mentioned below.

• Single ventricle palliation.

• Partial surgical resection.

• Epicardial pace or sense leads.

• Endocardial defibrillator.

• Heart transplantation.

What Are the Risk Factors of Cardiac Fibroma?

Cardiac fibroma is a benign condition. This means it will not spread to other organs. However, being alert about this, there are still some risk factors that one should be aware of. The healthcare provider can assist a more substantial and more direct treatment plan by alerting the risk factors. Mentioned below are some of the risk factors associated with cardiac fibroma.

• Heart wall abnormalities.

• Increased ventricular diastolic volume.

• Reduced systolic functioning.

• Mitral valve regurgitation.

• Malfunctioning of the left ventricle.

• Reduction in overall hemodynamics.

• A decline in the ventricular fraction of ejection.

• A drop in cardiac index.

• Ventricular arrhythmias.

Conclusion

The cardiac fibroma tends to regress with time. Cardiac fibromas are the most common tumors in childhood. The most active region is the left ventricular wall. Fibromas have been associated with polyposis syndromes and Gorlin-Goltz syndrome. Typical clinical presentation, if not treated and diagnosed, is heart failure due to blockage of blood flow and obstruction in valve functioning. An echocardiogram is the first diagnostic step, as with many other diagnostic tools. Cardiac fibroma tumors may require surgical resection after an interprofessional approach is validated. Henceforth, coordinated multidisciplinary care and follow-up are mandatory from the patient’s end.