Infantile Cardiac Hemangioma: A Rare Case Presentation

Introduction

The incidence of primary cardiac tumors is rare in all age groups. They account for 0.0017% to 0.28% of all cardiac tumors in autopsies. The incidence of primary cardiac tumors in fetal life is 0.14%. ¹ Most of these tumors are benign in nature with rhabdomyoma (60%) being the most common followed by fibroma and teratoma. ² Infantile cardiac hemangiomas are even rarer accounting for only 2.8% of cardiac tumors. ³ Hemangiomas are slow-growing benign tumors which are mainly seen in the skin but occasionally involve the heart. We present a case of an intracardiac hemangioma in a two-month-old child who presented with impending tamponade due to an associated large pericardial effusion. The patient underwent surgical excision of the mass and had a satisfactory postoperative recovery.

Case Presentation

A two-month-old female patient weighing 2.9 kg presented with poor feeding and failure to thrive. An electrocardiogram and echocardiogram (ECHO) were done to rule out cardiac pathology. Electrocardiogram showed normal sinus rhythm, but on ECHO a large homogenous intracardiac mass of approximately 30 mm×25 mm was detected in the right atrium (RA) (Figure 1A). There was an associated large pericardial effusion with features of impending tamponade. A contrast-enhanced cardiac computed tomography (CT) scan was done which showed a large single heterogenous intracardiac mass attached to the free wall of the RA (Figure 1B). Due to the large size of the mass and impending tamponade, the patient was scheduled for exploration and surgical removal of the mass.

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Figure 1.

(A) Echocardiogram shows a large intracardiac mass in the RA. There is associated pericardial effusion. (B) Contrast-enhanced CT scan shows a large intracardiac heterogeneously enhancing lesion (arrow) located along the free wall of the RA causing its expansion. Abbreviations: CT, computed tomography; LA, left atrium; LV, left ventricle; PE, pericardial effusion; RA, right atrium; RV, right ventricle.

The chest was opened with a median sternotomy. Pericardiotomy revealed a huge enlarged RA with the mass within its cavity. The RA wall was found to be intact (Figure 2A). Pericardial fluid was noted to be serous in nature. Cardiopulmonary bypass was initiated, aortic cross clamp applied, and the heart was arrested with Del Nido cardioplegia. The RA mass was of firm consistency with a smooth round contour densely adherent to a wide area of the RA free wall (Figure 2B). There was no evidence of internal rupture. The mass was removed “in toto” by a circumferential incision over the RA free wall which contained the base of the mass. We preserved as much of the RA wall tissue as possible (Figure 3A and B). After the RA was closed and cross clamp removed, the patient was gradually weaned off bypass with minimal support and stable hemodynamics.

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Figure 2.

Intraoperative finding of (A) enlarged right atrum with intracardiac mass in situ. (B) Open right atrium containing the mass. Abbreviation: RA, right atrium

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Figure 3.

(A) Gross pathology of tumor following excision. (B) Cross section of tumor showing foci of hemorrhage with necrosis.

Histological examination revealed lobules of small thin-walled capillary sized blood vessels lined by plump endothelial cells with interstitial fibrosis. Nuclear atypia or mitotic figures were absent. This was consistent with the diagnosis of capillary hemangioma. The patienthad an uneventful postoperative course and was discharged successfully.

Discussion

Cardiac tumors are rare in children and are mostly benign in nature. The most common cardiac tumors in fetuses and neonates are rhabdomyoma, teratoma, and fibroma. In infants and children, the most common cardiac tumors are rhabdomyoma and fibroma. ⁴ Cardiac and pericardial hemangiomas are uncommon benign vascular tumors in neonates and children. These can occur at any location in the heart such as ventricles, atrium, epicardial suface, and pericardium, with the most common being the RA.^3,5

Histologically, hemangiomas are classified as cavernous (composed of multiple dilated thin-walled vessels), capillary (lobules of smaller vessels resembling capillaries), and arteriovenous types (dysplastic malformed arteries and veins). The most common pathological type is cavernous hemangioma (58.5%) and simple capillary hemangioma (9.2%). Cardiac hemangiomas often combine features of more than one subtype. ³

Cardiac hemangiomas usually do not cause symptoms due totheir slow and benign growth. But neonates may present with features of congestive heart failure such as respiratory distress and failure to thrive, cardiac arrythmias, and pericardial effusion. The latter is mainly because of the secretory nature of the tumor. ⁶ The incidence of death can be high in these neonates and children due to high-output cardiac failure secondary to arteriovenous shunting through the hemangioma, massive hemorrhage from the fragile vessels, and involvement of the central nervous system (CNS). ⁵

Noninvasive procedures such as ECHO have a very high accuracy of detecting these tumors. Magnetic resonance imaging and CT scan are often helpful for better evaluation of the tumor size, location, and extracardiac involvement.

The treatment modality depends on the symptoms of the patient. Most authors consider surgical resection as the treatment of choice for symptomatic patients.^3,4 But many reports also suggest conservative management of these tumors with radiotherapy, corticosteroids, and beta blockers.^3,7 In our case study, surgical resection was carried out idue to the large size of the intracardiac tumor and the impending tamponade.

Conclusion

Cardiac hemangioma is a rare benign tumor in neonates and children. These patients are usually asymptomatic but can present with features of congestive heart failure and arrythmias. In some patients, they can be life-threatening due to high-output cardiac failure, hemorrhage, or CNS involvement. Echocardiography is an accurate and noninvasive method to detect these tumors while final diagnosis is based upon the histopathological examination of the specimen. Surgical resection of the tumor in symptomatic patients often has a favorable outcome.