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A 14-Year-Old Girl With a Systolic Murmur and Ventricular Extrasystole
A 14-year-old girl with no significant medical history is referred to a cardiologist by her primary care provider (PCP) after he notes a murmur during a routine physical examination. Her birth history is unremarkable, she has no family history of heart disease, and she reports normal exercise tolerance. Two months before presentation, however, the patient did experience a viral illness, with high fevers, cough, and fatigue.
On physical examination, her body weight and height fall in the 50th percentile for her age. A check of her vital signs shows a pulse of 90 bpm and a blood pressure of 120/70 mm Hg. Her lungs are clear to auscultation and normal respiratory effort is noted. A cardiac examination demonstrates a 1/6 soft systolic murmur along the left sternal border, without radiation. The peripheral arterial pulses in the upper and lower extremities are palpable and normal. No hepatosplenomegaly is found, and the remainder of the physical examination is unremarkable.
A 12-lead electrocardiogram (ECG) is performed, which reveals 1 ventricular extrasystolic beat but is otherwise unremarkable. Laboratory tests, including a complete blood cell count (CBC), erythrocyte sedimentation rate (ESR), electrolytes, serum glucose, serum creatinine, blood urea nitrogen, creatine kinase, lactate dehydrogenase, and liver transaminases, are all within normal limits. A chest radiograph is obtained and is normal. A second, prolonged ECG that is performed both while the patient is resting and during exercise reveals normal findings, without any extrasystole. The patient undergoes 24-hour Holter monitoring, which demonstrates rare monomorphic, monotopic ventricular extrasystoles. A 2-dimensional echocardiography examination is then performed, which reveals a V-shaped hyperechogenic mass in the right ventricle (see Figure 1) that appears firmly attached to the ventricular wall.
What is the mass in the right ventricle?
Hint: Look closely at the result of the 2-dimensional transthoracic echocardiography (Figure 1).
2,Right ventricular thrombus
Echocardiography revealed a hyperechogenic mass in the right ventricle of the heart that consisted of 2 parts: a spindle-shaped section approximately 3.5 cm long that radiated towards the apical side of the right ventricle, and a second section in the muscular portion of the interventricular septum. Both portions were about 1 cm wide and were attached firmly to the heart structures. No blood flow or right ventricular outflow obstruction was observed.
Cardiac lipomas are rare, benign, well-encapsulated tumors arising primarily from the myocardium or within a cardiac chamber. These types of cardiac masses are usually found in adult patients, but they can affect patients of all ages. These tumors consist of mature fat cells; they most commonly occur in the atrial septum as part of lipomatous hypertrophy of the interatrial septum. Occasionally, mature fat cells are intermingled with vacuolated, multilobulated fat cells. Diverse benign lipomatous lesions of the heart can be distinguished from one another based upon the size of the tumor and location: solitary, well-defined lipomas and its variants, intramuscular and intermuscular lipomas (infiltrating lipomas); subendocardial lipomas (large polypoid masses) with intracavity extension; lipomatous hypertrophy of the atrial septum, which is associated with myocardial atrophy and fibrosis; and extremely rare multiple large lipomas encircling the whole heart.
Among pediatric patients, primary cardiac neoplasms include rhabdomyoma (78%), fibroma (11%), pericardial teratoma (2%), epicardial lipoma (2%), multicystic hamartoma (2%), and unspecified tumors (5%). Most primary pediatric tumors are diagnosed within the first year of life.
Cardiac lipomas have no sex predilection. These tumors are frequently found during some diagnostic procedure but, rarely, they may present with heart murmur or arrhythmia in a previously healthy child. Subendocardial lipomas with intracavity extension may cause valve stenosis or insufficiency, while multiple large lipomas may compromise cardiac chambers. Lipomatous hypertrophy of the atrial septum, usually seen in advanced age, can cause cardiac arrhythmias. Conduction abnormalities can be evaluated by electrophysiology testing and may be amenable to radiofrequency ablation. Chest radiography may demonstrate the effects of intracardiac obstruction with features of pulmonary edema; however, it is more common to have a normal appearance.
Initially, cardiac lipomas are often best evaluated by echocardiography. Areas behind the sternum, ribs, or lungs cannot be visualized adequately, and acoustic windows limit the structures imaged on any single plane or view, but the benefit of echocardiography is its portability and ease of use for a first-look approach to cardiac pathology. In patients with poor-quality transthoracic echocardiograms, transesophageal echocardiography usually provides better images, especially of masses in or adjacent to the left atrium. Differentiation of left atrial masses from thrombus may also be best achieved by using transesophageal echocardiography. Echocardiography permits the initial diagnosis of the heart mass. It is a relatively inexpensive and noninvasive method for monitoring the evolution of a mass, and it can be used to direct potential therapies.
Because they provide a larger field of view and greater anatomic detail, magnetic resonance imaging, (MRI), magnetic resonance angiography (MRA), and computed tomography (CT) scanning are preferred over echocardiography for cases that are less straightforward or for cases in which acoustic access is restricted. CT is accurate but requires the use of radiation and contrast material. In suitable patients, MRI has the fewest limitations, provided that the patient can remain calm during the examination. Contrast-enhanced MRI may define the extent of the tumor, but it is limited in its ability to discern benign from malignant disease. Cardiac contractility can be evaluated using MRI. In recent studies, echocardiography has been reported to be as useful as CT or MRI in evaluating cardiac masses. The imaging planes of CT or MRI are limited to those allowed by angulations of the gating; additionally, they are susceptible to motion artifacts, which occur more commonly in the pediatric population.
Some cardiac masses may not be tumors. Pseudotumors are abnormalities that mimic tumors and include thrombus or variants of cardiac anatomy (eg, prominent pulmonary vein orifice, valvular vegetation, thrombus within a left ventricular aneurysm, lipomatous hypertrophy of the interatrial septum, rheumatoid nodule, ruptured chordae tendineae, tumors outside the heart and pericardium).[7,8] When a cardiac mass is found, an endomyocardial biopsy should always be performed even if the tumor appears benign. Lipomas are typically surrounded by a capsule that makes them easy to delineate. In cases of cardiac lipoma, an endomyocardial biopsy should demonstrate mature fatty tissue cells with interlacing muscle fibers. Because normal fat deposition in the epicardium increases with advancing age, the clinician should take care to firmly establish the diagnosis of cardiac lipoma in an adult. Such deposition is primarily observed in the anterior surface of the ventricles, the anterior interventricular groove, and along the apical course of the coronary arteries. Excessive fat deposition is usually accompanied by fatty infiltration of the myocardium of the right ventricle and interatrial groove.
Surgical resection remains the treatment of choice for symptomatic lipomas. Incidental tumors can be resected, with generally good results. Surgical intervention is usually required only for children who develop relevant clinical symptoms resulting from large tumor masses or unfavorable localization of the tumor. Lipomas grow very slowly and usually do not require resection. When surgical excision of the lipoma is necessary but the location of the lipoma is not favorable, complete surgical correction may require ventricular reconstruction with a dynamic radiomyoplasty procedure. The malignant transformation of cardiac lipomas has never been convincingly documented, but the recurrence of resected lipomas has been reported. Sudden death from lipoma is rare and has only been reported in cases of lipomas located in the right atrial appendage or in more diffuse fatty infiltrates of the myocardium.[12,13]
In the patient in this case, an endomyocardial biopsy demonstrated mature fatty tissue cells with interlacing muscle fibers. Thoracic CT scanning was performed (Figure 2) over 4 years after her initial echocardiogram to monitor for progression. The scan revealed a hypodense expansive mass in the form of an inverse letter V, with an absorptive coefficient of fatty tissue (-50 to -75 Hounsfield units, which is consistent with adipose tissue). A repeat transthoracic echocardiogram (Figure 3) was also performed at this time, which revealed an excellent view of the lipoma as a hyperechogenic mass involving the apical part of the right ventricle, the muscular part of the interventricular septum, the anterior papillary muscle of the right ventricle, and a small part of the right ventricular wall near the tricuspid valve. The tricuspid and pulmonary valves were intact. Because this patient remained asymptomatic and the growth of the tumor was very slow, surgical excision of the tumor was not planned at that time.