Ann Thorac Surg 2000;70:975-977
© 2000 The Society of Thoracic Surgeons
Case report
Hemangioma of the right ventricular outflow tract
Brian R. Kann, MDa,
William J. Kim, BAa,
Jonathan H. Cilley, Jr, MDa,
Steven W. Marra, MDa,
Anthony J. DelRossi, MDa
a Division of Cardiothoracic Surgery, Department of Surgery, UMDNJ-Robert Wood Johnson Medical School, Cooper Hospital/University Medical Center, Camden, New Jersey, USA
Address reprint requests to Dr Marra, UMDNJ-Robert Wood Johnson Medical School, Cooper Hospital, University Medical Center, Three Cooper Plaza, Suite 411, Camden, NJ 08103
e-mail: marra-steve{at}cooperhealth.edu
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Abstract
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Obstruction of the right ventricular outflow tract by a primary cardiac tumor is rare. Six cases of right ventricular outflow tract obstruction by a primary cardiac hemangioma have been reported; all but one were detected before the age of 25 years. In this report, we review the literature and describe what we believe to be only the second reported case of right ventricular outflow tract obstruction produced by a cardiac hemangioma that presented in late adulthood.
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Introduction
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Primary cardiac tumors are exceedingly rare. Three-quarters of all primary cardiac tumors are benign; most of these are atrial myxomas. Primary cardiac hemangiomas are extremely uncommon. Taken as a separate entity, primary tumors of the right ventricle are also very rare. When there is obstruction to right ventricular outflow, a variety of symptoms that mimic valvular disease, angina, or conduction abnormalities may be seen. We are aware of only six prior cases of a cardiac hemangioma producing right ventricular outflow tract (RVOT) obstruction in the literature: two in children, three in adults less than 25 years of age, and one in a 59-year-old female [1–6]. We present herein what we believe to be only the second case of RVOT obstruction due to a primary cardiac hemangioma that presented in late adulthood.
A previously healthy 59-year-old African American male presented with signs and symptoms of unstable angina. He underwent cardiac catheterization, which revealed three-vessel coronary artery disease as well as a tumor blush in the right ventricle. Subsequent transesophageal echocardiography revealed left ventricular and septal hypertrophy in addition to a discrete 4.0-cm right ventricular mass that nearly filled the RVOT during diastole (Fig 1). Magnetic resonance imaging (MRI) of the chest demonstrated a 4.0 x 2.0 cm contrast-enhancing mass associated with the anterior aspect of the RVOT (Fig 2).
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Fig 1. Transesophageal echocardiogram, demonstrating a 4.0-cm lesion in the right ventricular outflow tract.
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Fig 2. MRI of the chest, demonstrating a 4.0 x 2.0 cm contrast-enhancing mass involving the anterior aspect of the right ventricular outflow tract.
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The patient underwent three single-vessel bypass grafts and excision of the right ventricular mass. The entire procedure was performed with a single aortic cross-clamp due to the marked atherosclerotic nature of the aorta. On gross inspection, the ventricular mass measured 4.0 x 3.0 x 2.0 cm in size and demonstrated extensive neovascularization on the surface. After excision of the mass, the resulting defect in the RVOT was closed using a Hemashield patch (Meadox Medical, Oakland, NJ) and nonabsorbable suture. Permanent histologic sections revealed the tumor to be a hemangioma.
Postoperatively, the patient was noted to have isolated right upper extremity weakness with no other neurologic deficits. Computed tomography (CT) of the head revealed a small infarct in the distribution of the distal left middle cerebral artery, which was presumably embolic in nature. The remainder of his postoperative course was uneventful, and at the time of discharge, on the sixth postoperative day, he had begun to regain right upper extremity strength.
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Comment
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Hemangiomas are tumors of vascular origin. The appearance may vary from that of a capillary type to that of a cavernous type. Capillary hemangiomas tend to occur in the skin, subcutaneous tissues, and mucous membranes of the oral cavity and lips. Cavernous hemangiomas have a predilection for the skin of the head and neck as well as other mucosal surfaces of the body. Visceral involvement of both types is fairly common. Cardiac involvement, however, is extremely rare.
Right ventricular tumors are also fairly uncommon. A review of the literature in 1972 revealed 62 reported cases of primary right ventricular tumors. Of these tumors, 56 could be classified histologically; only two of these were classified as hemangiomas [4]. A more recent review revealed only 34 reported cases of primary cardiac hemangiomas in the literature [7]. Six prior cases of RVOT obstruction due to a hemangioma have been reported. Two of these presented in childhood [1, 2], three presented in adults less than 25 years of age [3–5], and one presented in late adulthood [6]. We believe that the case we have described herein represents only the second such reported case presenting in late adulthood.
Most cardiac hemangiomas are discovered incidentally. Common clinical presentations include dyspnea on exertion, arrhythmias, right-sided heart failure, pericarditis, pericardial effusion, and failure to thrive. Asymptomatic presentations have also been described [5]. As one would expect, the presenting symptoms typically vary with the size and location of the tumor. In a review of cardiac hemangiomas by Brizard and associates, the most frequent locations were the lateral wall of the left ventricle (21%), the anterior wall of the right ventricle (21%), and the interventricular septum (17%). Only 5 of the 24 cardiac hemangiomas described in this review were specifically localized to the RVOT [5].
Preoperative diagnosis of the cardiac hemangioma is difficult. Whereas chest roentgenograms are abnormal in the majority of cases, the diagnosis of a cardiac tumor is rarely suspected on the basis of plain radiographs alone. The diagnosis of a cardiac hemangioma is suggested by a characteristic tumor blush on coronary arteriography. Echocardiography, either transthoracic or transesophageal, should be done as an initial imaging modality. More recently, contrast-enhanced CT and MRI have been suggested as radiographic imaging modalities of choice [8]. With the wide availability of newer, noninvasive diagnostic methods, the diagnosis of these tumors is being made more frequently.
When technically feasible, surgery is indicated to both confirm the diagnosis and excise the tumor [2]. The natural history of these tumors is extremely variable. They may remain dormant and clinically silent or may proliferate indefinitely. One case of spontaneous resolution has been reported [9]. Long-term prognosis after surgical resection is extremely favorable. More recently, it has been suggested that when extensive resection requires hazardous surgical procedures, it could possibly be foregone except in severely symptomatic patients in whom resection is necessary to eliminate compression of vital cardiac structures or outflow tract obstruction [5].
In conclusion, cardiac hemangiomas are extremely rare primary cardiac tumors. A variety of clinical presentations may be seen, depending upon the size and location of the tumor. Very rarely, hemangiomas of the right heart may produce RVOT obstruction; this usually is detected early in life. We have described what we believe to be only the second reported case of RVOT obstruction due to a cardiac hemangioma that presented in late adulthood.
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References
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Accepted for publication March 23, 2000.
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Abstract
Introduction
