Infantile cardiac hemangioma causing right ventricular inflow obstruction

Introduction

Cardiac hemangioma is a rare primary cardiac tumor, accounting for approximately 2.8% of all benign cardiac tumors. ¹ There have been almost 30 cases of these tumors reported in neonates and infants, but only a few that presented with a life-threatening condition. ² Herein, we report a case of obstructive cardiac hemangioma in an infant who was treated successfully with emergency tumor removal.

Case report

A 4-month-old girl with no underlying disease presented with sudden dyspnea during breastfeeding. She appeared lethargic and cyanotic on physical examination. Her initial oxygen saturation was 63% on room air. She was in sinus rhythm with a heart rate of 152 beats·min⁻¹, blood pressure of 80/50 mm Hg, and a respiratory rate of 56 breaths·min⁻¹. No cardiac murmur was observed. The patient was intubated with ventilator assistance and admitted to the intensive care unit. Chest radiography revealed mild cardiomegaly with normal lung parenchyma. Electrocardiography was normal. Bedside echocardiography revealed an approximately 3 × 3.5-cm hetero-echoic mass at the right atrium, which was causing right ventricular inflow obstruction, and a 1.46-cm atrial septal defect. With the suspicion of cardiac myxoma, the patient was referred to our unit for surgery. Because she had symptoms and signs of tumor obstruction, which was a life-threatening condition, emergency tumor resection was indicated. The approach was made via a median sternotomy. Cardiopulmonary bypass was established with ascending and bicaval cannulation under mild hypothermia. We avoided manipulating the right atrium before crossclamping the aorta. Cold blood cardioplegia was administered, and when the heart stopped, a right atriotomy was performed. The tumor appeared as a round, reddish, cystic mass. It originated from the right atrial free wall (Figure 1a, 1b) and extend to the lateral wall of the inferior vena caval orifice. The tumor was completely resected with part of the right atrial wall. The atrial septal defect was closed with a synthetic patch. The patient was hemodynamically stable after weaning off bypass. The postoperative course was uneventful and she was discharged after 5 days. There was no evidence of recurrence after 5 months of follow-up.

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Figure 1.

(a) Surgeon’s view demonstrating the large tumor that occupied the right atrium. The tumor originated from the right atrial free wall. The site where the tumor originated and attached to the right atrium exhibited a rough surface. (b) The gross specimen was a well-circumscribed globular mass with a smooth surface, measuring 3 × 3.5 cm.

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Figure 2.

The vascular neoplasm comprising mixed small-sized veins, venules, and capillaries. (a) Hematoxylin and eosin stain, original magnification ×100. (b) Hematoxylin and eosin stain, original magnification ×400. The vascular spaces were lined by uniform flat endothelium cells without any cytological atypia. Endothelial markers stained positive for (c) CD31, (d) CD34, and (e) factor VIII (original magnification ×400).

Discussion

The overall incidence of primary cardiac tumors is approximately 0.01%–0.03% at autopsy, and cardiac hemangiomas constitute 2.8% of primary cardiac tumors. ³ The majority of patients present with tumors in their fifth decade of life. Tumors presenting in infancy, such as in our case, are extremely rare. Clinical manifestations of cardiac hemangioma are varied and nonspecific. They are determined by the size and location of the tumors. The majority of cardiac hemangioma patients are asymptomatic. One-fifth of patients may develop serious complications such as syncope, angina, or embolic stroke. ¹ In very rare cases, patients may present with cyanosis, ⁴ tumor obstruction, ² or sudden death.^5,6 Therefore, both a high level of awareness of the possibility of this disease and early diagnosis are important for preventing a fatal outcome.

Echocardiography is an important and noninvasive tool that provides preliminary anatomical and dynamic details of the tumor. Computed tomography and magnetic resonance imaging increase the accuracy of diagnosis by revealing the hypervascularity of the tumor. Recent studies have reported the successful diagnosis of cardiac hemangioma during the prenatal period,^2,7,8 which can allow early management to prevent any serious complications that may occur. Pathological examination is important for the definitive diagnosis of cardiac hemangioma. There are 3 types hemangioma according to microscopic examination: capillary, cavernous, and arteriovenous. Cavernous hemangioma is the most predominant. However, cardiac hemangioma often combines features of more than one subtype, which was the case in our patient who had a mixed capillary and cavernous hemangioma (See Figure 2).

The natural history of cardiac hemangioma is unpredictable. Complete resection is preferable due to the potential risk of dangerous complications. ³ Because our patient presented with tumor obstruction, there was no doubt that emergency tumor removal was necessary. Typically, cardiac hemangioma is a noninvasive tumor and simple resection can be performed with or without cardiopulmonary bypass. However, in some cases, complete resection may be difficult or impossible, depending on the site of tumor involvement. Although cardiac hemangioma has a low incidence of recurrence, post-surgical follow-up with echocardiography is warranted.