22 month old girl born at term via an uncomplicated delivery. Her postnatal course is complicated only by tachypnea.
She is now 15.8 kg. Vital signs at last exam were HR 120, BP 93/68, and RR 24. No SaO2 is reported. She has no central cyanosis, clubbing, or peripheral edema. She is in no respiratory distress. Her heart has a regular rate and rhythm, normal S1 and S2, and no audible murmurs. EKG is unchanged with sinus rhythm, L axis deviation, and nonspecific T-wave abnormality.
CXR and other subsequent studies were obtained leading to a diagnosis.
CXR - Patient is slightly rotated to right. Enlarged heart size with irregular borders. Lungs clear except small lung volume L
CT w/IV contrast - Intracardiac mass of the left ventricle. No pleural effusion or pericardial infiltration. (Mild disagreement between Echo and CT regarding interseptal involvement of the mass with echo showing mild superior interseptal involvement and CT showing none.)
Reconstructed CT image w/contrast - Very little perfusion to a large area of the left ventricular wall c/w large mass.
Radionuclear perfusion study - No evidence of perfusion to the lateral wall of the left ventricle. Accurate ejection fraction unable to be obtained.
Given the relatively benign clinical course thus far with a nonperfusing, very slowly growing intracardiac mass, a benign tumor is likely:
Rhabdomyoma - the most common intracardiac tumor of childhood, usually small and most commonly in the left ventricle.
Fibroma - Next most common (though very uncommon)intracardiac tumor of childhood, usually involves intraventricular septum but is also found in left ventricular free wall.
Myxoma - Rare in childhood and usually involves either atria.
The patient is now growing well without any new complaints and is being followed by cardiology and hematology/oncology every 3-6 months. She has remained asymtopmatic without murmurs and evidence of cardiovascular failure despite this large mass.
Options to remove the tumor are unlikely because of its size and LV wall involvement, arguably infiltrating the intraventricular septum. Further considerations for chemotherapy are being discussed if she becomes symptomatic. As a last option a heart transplant is also being considered.
Nearly all benign cardiac tumors found at birth or during early childhood are either a rhabdomyoma or fibroma. Rhabdomyomas are by far the most common benign cardiac tumor, most occuring before the age of 1 yr. They most often occur in within ventricular walls (left -80%, right -15%) but can occur on the atrioventricular valves. 80-90% of rhabdomyomas are associated with tuberous sclerosis. Typically, they regress spontaneously and require only outpatient follow-up with cardiology. Rarely they proliferate and cause obstruction and heart failure. Treatment for symptomatic children includes surgical resection, chemo, or rarely a heart transplant. Fibromas are less common and typically have a less benign course. They typically involve the intraventricular septum or the left ventricular free wall. There has been reported atrial involvement as well as tumor growth impeding either the mitral or aortic valves leading to stenosis and/or regurgitation. Fibromas, unlike rhabdomyomas, do not regress spontaneously but typically continue to grow. This can impede blood flow, cause conduction disturbances, and hinder chamber filling and overall cardiac function leading to heart failure. Patients can be followed clinically until they become symptomatic. They will likely need resection of the tumor, chemotherapy, or cardiac transplant.
This patient is currently asymptomatic. Her tumor is growing; however, she continues to show no signs of cardiac impairment. As mentioned above, recent studies were inconclusive regarding the cardiac output and efficiency. However, her tumor is growing and now occupies a larger volume of her left ventricle. Her tumor 3 days after birth measured 1.8 x 2.8 x 3.0 cm with a LV size (including tumor) approximately 3.1 x 4.0 x 3.9 cm. Eighteen months later her tumor measured 4.5 x 5.9 x 4.2 cm with a LV size (including tumor) of 6.1 x 5.8 x 4.2 cm. Her tumor volume was about 31% of the LV size at birth compared to 75% eighteen months later showing it is becoming more of a space occupying lesion and likely will soon become symptomatic. Given the size of her tumor treatment options are limited to a chemotherapy trial or a heart transplant.
Infantile cardiac fibromas are extrememly rare (1 cases per 2,000,000 births).
The key pathologic charateristic of cardiac fibroma is the proliferation of fibrous tissue which insinuates itself into the myocardial fibers of the heart wall. Rhabdomyomas often have a more distinct capsule. Debate continues as to whether this is a neoplastic or non-neoplastic entity.
The most common complications are related to cardiac failure likely due to mass effect sometimes complicated by hypoplasia of the involved ventricle. Fetuses may present with non-immune hydrops. Later complications include embolic events and conduction abnormalities.
There is not any well defined group of associated anomalies.
Some reports indicated that the tumors can be quite large in surprisingly assymptomatic patients.
Surgical excision may be useful for small tumors, but cardiac transplantation may be required.
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