Left ventricular hemangioma

Introduction

Cardiac hemangiomas are rare benign vascular tumors of the heart, with variable presentations and unpredictable outcomes. Complete surgical excision is recommended, with an expected good prognosis. Surgical resection aided by thoracoscopy is helpful.

Case report

A 20-year-old Saudi woman presented with a one-year history of palpitation, anxiety, and recurring near-syncope. Transthoracic echocardiography (TTE) showed an immobile 1.6 × 0.9-cm left ventricular (LV) mass attached to the apical 1/3 of the septum (Figure 1). Physical examination was unremarkable, without an appreciable cardiac murmur. An electrocardiogram showed sinus rhythm with frequent unifocal premature ventricular contractions and moderate left-axis deviation. Chest radiographs and laboratory findings were normal. Excisional biopsy was felt to be the most appropriate diagnostic and potentially curative therapy. Under general anesthesia, transesophageal echocardiography confirmed the presence of the LV mass. Under mild hypothermic cardiopulmonary bypass and a single dose of antegrade cold blood cardioplegic arrest, a transverse aortotomy was made. A Karl Storz 10-mm zero-degree thoracoscope was inserted into the LV cavity and the mass was visualized. It did not have any appreciable stalk, was grayish white in color, fragile, and multilobulated. It was easily detached from the septum, taking a thin layer of the ventricular septum with it (Figure 2). Off-bypass transesophageal echocardiography showed new onset of trace aortic regurgitation and normal septal motion. The patient’s recovery was unremarkable. TTE prior to discharge showed persistent trace aortic regurgitation which had resolved at the 3-month TTE follow-up. The final histologic diagnosis showed multiple dilated thin-walled capillary-sized vessels lined by bland endothelial cells and separated by myxoid stroma mildly infiltrated by mononuclear inflammatory cells. Immunohistochemistry showed a positive reaction of the cells lining the blood vessels for CD34, consistent with mixed-type cavernous-capillary hemangioma (Figure 3).

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Figure 1.

Transthoracic echocardiogram showing a left ventricular mass (crosses).

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Figure 2.

Intraoperative picture showing the left ventricular mass (surrounded by white arrows) being delivered out of the left ventricular cavity.

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Figure 3.

Histology showing (a) a mixture of dilated cavernous-type blood vessels and small capillaries. Hematoxylin and eosin stain, original magnification ×10. (b) Endothelial cells lining the blood vessels. Immunohistochemical CD34 stain, original magnification ×10.

Discussion

The incidence of primary cardiac tumors discovered at autopsy was 0.001% to 0.03%, with 75% of them being benign. ¹ More than half of these benign cardiac tumors were myxomas, the rest being lipomas, fibromas, hemangiomas, lymphangiomas, neurofibromas, granular cell tumors, rhabdomyomas, papillary fibroelastomas, teratomas, and mesotheliomas of the atrioventricular node. ¹ Metastatic involvement of the heart is approximately 30-times more common than a primary cardiac tumor. Primary cardiac tumors can occur in any age group, with a slight preponderance for the female sex and the right side of the heart. ² Hemangiomas usually occur in cutaneous structures or the gastrointestinal tract. Localization to the heart is extremely rare. ² Histologically, cardiac hemangiomas are classified into 3 categories: capillary, distinguished by small capillary-like vessels; cavernous, with multiple dilated thin-walled vessels; and mixed. The majority of cardiac hemangiomas have overlapping of all 3 components interspersed among fibrous tissues. ³

Most cardiac hemangiomas are either asymptomatic or present with dyspnea, arrhythmias, atypical angina, pericardial effusion, congestive heart failure, ventricular outflow tract obstruction, embolic events, or even sudden cardiac death. ² They can masquerade as sepsis with disseminated intravascular coagulation, or be associated with Kasabach-Merritt syndrome which is characterized by multiple systemic hemangiomas with thrombocytopenia and consumptive coagulopathy.^4,5 With wide application of TTE during the last few decades, more asymptomatic cardiac tumors have been identified, and close to 100 surgically treated cardiac hemangiomas have been reported. ³ Computed tomography angiography and magnetic resonance angiography are useful to assess the extent of myocardial and local invasion. Cardiac catheterization with coronary artery angiography can occasionally establish the diagnosis of hemangioma by its characteristic tumor vascular blush. The natural history of cardiac hemangiomas is unpredictable. They may continue to proliferate, cease to grow, or regress.^2,6 Preoperative diagnosis is rarely secured.

Complete excision of the tumor is recommended for tissue diagnosis and potential cure, with an expected good long-term prognosis. The incidence of recurrence after surgical resection is unknown. In 1993, Brizard and colleagues ⁷ reviewed 24 of the 28 surgically-treated patients (8 incisional biopsies, 4 partial excisions, 12 complete excisions) reported in the world literature. Only 13 patients were followed up, with one postoperative death. The remaining 12 patients (6 biopsies or partial excisions only) were followed up from 2 months to 6 years; all were alive and well. Various surgical approaches have been proposed. Median sternotomy with cardiopulmonary bypass is universally required for intracardiac tumors. While right-sided tumors can be approached by a right atriotomy, a left ventriculotomy for LV tumors is best avoided to prevent postoperative LV dysfunction and arrhythmia. The transaortic approach usually provides adequate exposure with minimal trauma to the heart. We utilized a Karl Storz 10-mm thoracoscope in this case, which provided excellent visualization, illumination, and magnification. No prolonged procedure-related aortic regurgitation was observed with this approach.