Introduction
Cardiac rhabdomyoma is an uncommon tumor. It develops from striated muscles. Congestive heart failure symptoms might emerge. Pharmacotherapy and surgical resection are needed. Cardiac rhabdomyoma is a unique and benign striated muscle tumor. It affects the head and neck.
What Is the Classification of Cardiac Rhabdomyoma?
The three types of extracardiac tumors are:
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Adult cardiac rhabdomyoma.
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Fetal cardiac rhabdomyoma.
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Germ cell cardiac rhabdomyoma.
The most common juvenile heart tumor is cardiac rhabdomyoma (CR), which generally develops before one year. They are classified as hamartomas anatomically.
What Is the Occurrence of Cardiac Rhabdomyoma?
Most cardiac rhabdomyoma are numerous and regress independently; surgical excision is not recommended unless the patient is symptomatic. The symptoms result from a blockage in blood input or outflow, which leads to congestive heart failure. Arrhythmias (irregular heartbeat) can also develop from bradycardia (low heart rate) caused by sinus or atrioventricular node (AVN) dysfunction to the atrioventricular node (AVN) reentrant tachycardia, atrial or ventricular tachycardia, or ventricular pre-excitation.
Epidemiology of Cardiac Rhabdomyoma
Primary cardiac tumors are uncommon, occurring in about 0.2 % of children. Heart rhabdomyomas are 45 % of all cardiac tumors in children and are the most frequent. It is noticed in males and women who do not prefer race.
What Is the Cause of Cardiac Rhabdomyoma?
Rhabdomyoma may be caused by genetic changes that occur during striated muscle development. There are no other known causes. Tuberous sclerosis is linked to 80 to 90 percent of cardiac rhabdomyoma.
What Is the Pathophysiology of Cardiac Rhabdomyoma?
Cardiac rhabdomyoma is a form of hamartomatous tumor. It is most typically detectable between 20 and 30 weeks of gestation. Tumors are seen more frequently in prenatal series than in postnatal series. The rate of fetal discovery has increased with the advancement of imaging technologies. The interventricular septum, atrioventricular valves, and left and right ventricles are typically implicated. It develops in the heart muscle's myocardium. Although it is infrequent, there is a clear clinical link between cardiac rhabdomyoma and tuberous sclerosis, an autosomal dominant disorder characterized by benign hamartomas in numerous organ systems. Tuberous sclerosis is generally diagnosed clinically, and subsequent genetic testing to check for TSC1 and TSC2 gene abnormalities is confirmed.
What Are the Signs and Symptoms of Cardiac Rhabdomyoma?
Ultrasonography can detect cardiac rhabdomyoma as a mass in utero. Heart blockages, hydrops fetalis, and pericardial effusion are all possible presentations. If the tumor is big enough, intrauterine fetal death may occur. They are usually asymptomatic after birth; if they are, they commonly manifest with signs and symptoms of heart failure and ventricular dysfunction caused by a blockage in the outflow or inflow of blood. Dyspnea (shortness of breath) is one of the most common first-patient symptoms. Tumors are identified more frequently in fetal series than postnatal series, increasing fetal echocardiogram sensitivity. In addition, physical manifestations may be present in the patient.
How Are Cardiac Rhabdomyoma Diagnosed?
Electrocardiogram (EKG or ECG)
As cardiac rhabdomyoma is most usually associated with tuberous sclerosis, a baseline 12-lead electrocardiogram (EKG) is required at the time of diagnosis, even if no cardiac symptoms are present, with regular EKGs every two to five years after that.
Echocardiography
It is the preferred imaging modality (ECHO). Multiple echogenic and nodular masses in the ventricular myocardium characterize cardiac rhabdomyoma. Rhabdomyoma of the heart can extend into the ventricular cavity. If the location is unusual or there is a single substantial solitary tumor, the results may be misconstrued as a cardiac tumor-like atrial myxoma.
Cardiac Magnetic Resonance Imaging (MRI)
In situations of ambiguous results, other imaging modalities, such as cardiac magnetic resonance imaging (MRI), might be employed as a supplement to ECHO. The MRI improves tumor delineation and can be highly useful in situations with planned surgical resection. It also gives a more accurate measure of ventricular systolic function.
What Is the Treatment of Cardiac Rhabdomyoma?
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Cardiac rhabdomyomas are asymptomatic and spontaneously regress.
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Pharmacotherapy with angiotensin-converting enzyme inhibitors, Digitalis, and diuretics is appropriate in rare hemodynamic compromise and congestive heart failure cases. In addition, prostaglandin E can be given to control hemodynamic instability in a severely unwell baby.
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When there are conduction problems or any other type of ventricular arrhythmia, antiarrhythmics can help.
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Surgical excision of the mass is indicated in hemodynamic compromise and congestive heart failure caused by a giant tumor.
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If there is a high chance of cardiac mass or significant structural injury from total excision, partial resection is suggested.
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Orthotopic heart transplantation is explored when the tumor is so enormous that it replaces normal cardiac tissue.
What Is the Differential Diagnosis of Cardiac Rhabdomyoma?
The differential diagnosis includes the following:
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Fibroma of the heart.
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Myxoma of the atrium.
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Teratoma.
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Thrombus.
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Myofibroblastic inflammatory tumor.
Prognosis of Cardiac Rhabdomyoma
Patients undergoing rhabdomyoma removal surgery have a decent to favorable prognosis. However, cardiovascular rhabdomyomas have the highest risk. This is because they can expand and impede the left ventricular outflow tract, producing flow irregularities or creating ventricular tachycardias or heart blocks.
As fetal cardiac rhabdomyoma is frequently the first symptom of tuberous sclerosis, other structures, such as the renal or brain parenchyma, should be evaluated for tumors because cardiac rhabdomyoma is frequently the first symptom of tuberous sclerosis, followed by neurological involvement and impairment.
Complications of Cardiac Rhabdomyoma
The complications of cardiac rhabdomyoma:
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Infections.
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Arrhythmias.
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Tachycardia.
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Bradycardia.
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Compromise in hemodynamics.
Conclusion
The approach to cardiac rhabdomyoma therapy is multidisciplinary. Inter-professional contact among general practitioners, cardiologists, and cardiothoracic surgeons is critical. A growing body of data supports the use of Everolimus in treating cardiac rhabdomyoma. Every management stage must be conveyed to the family, which is essential to patient-centered care. Patient safety should always be a top concern, necessitating exceptional teamwork and performance.
