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Year : 2011  |  Volume : 54  |  Issue : 3  |  Page : 646-647
Cardiac sub-pulmonary arteriovenous hemangioma

Departments of Pathology (Cardiovascular and Thoracic Division), Seth GS Medical College, Mumbai, India

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Date of Web Publication20-Sep-2011

How to cite this article:
Vaideeswar P. Cardiac sub-pulmonary arteriovenous hemangioma. Indian J Pathol Microbiol 2011;54:646-7

How to cite this URL:
Vaideeswar P. Cardiac sub-pulmonary arteriovenous hemangioma. Indian J Pathol Microbiol [serial online] 2011 [cited 2022 Jan 18];54:646-7. Available from: https://www.ijpmonline.org/text.asp?2011/54/3/646/85132


Tumors of the heart are more often metastatic than primary. Primary cardiac tumors have an incidence of 0.001-0.03% and almost 75% of them are benign. [1] Unlike myxomas, cardiac hemangiomas lag behind in their order of frequency, more so owing to their often asymptomatic nature. [2] These tumors are hence discovered incidentally at autopsy or clinical investigations. We report a rare arteriovenous hemangioma of the heart located in the right ventricular outflow tract, as an incidental finding at autopsy.

A 35-year-old morbidly obese male (135 kg) was admitted in the emergency medical services department in a gasping and cyanosed state. A chronic smoker and alcoholic, he had developed gradually increasing shortness of breath and edema feet for the past nine days. He subsequently developed focal ulcerations and pale blue-black discoloration up to the knees. The clinical diagnosis was Pickwickian syndrome with cor pulmonale and bilateral lower limb cellulitis. The patient expired within four hours of admission. At autopsy, the heart was markedly enlarged in size and weighed 710 g. There was marked increase in the epicardial fat, moderate right atrial hypertrophy and marked right ventricular hypertrophy. Just below the left posterior cusp of the pulmonary valve, there were four, slightly compressible red-brown nodules (0.3 to 0.8 cm in diameter, [Figure 1]). On histology, there were definite endocardial and subendocardial components [Figure 2]. The endocardial lesions showed a mound of connective tissue in which nestled many thick- and thin-walled vessels, some appearing cavernous [Figure 2]. Clusters of hemosiderophages were also present. These areas were distinctly separated by the endocardial fibroelastic tissue. The underlying myocardium showed aggregates of thick-walled arteries [Figure 2], one of which appeared to have a connection with the endocardial hemangiomatous formation and possibly represented a feeding vessel. Additional findings included lymphocytic myocarditis, focal pulmonary hemorrhage and cardiac cirrhosis of liver.
Figure 1: (a) Opened out right ventricular outflow tract showing four sessile polypoidal masses (arrows), situated just below the posterior cusp of the pulmonary valve. (b) A scanned glass slide of the histology section stained by elastic van Geison stain, showing a sub-pulmonary (arrows) location. (PT-Pulmonary trunk PV-Pulmonary valve, RV-Right ventricle, TV-Tricuspid valve)

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Figure 2: (a) Endocardial lesion demarcated from the subendocardial lesion by well-preserved fibroelastic tissue (Elastic van Geison, ×25). (b) Cavernous spaces seen on the endocardial aspect (H and E, ×400). (c) A thick-walled "grimacing" vessel identified in the underlying right ventricular myocardium (H and E, ×400)

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Despite being at the helm of the cardiovascular system, hemangiomas of the heart are uncommon and the first case was described in 1893. [3] The rarity of occurrence is reinforced by the fact that so far less than 100 cases have been reported, with an incidence of about 1-5% of all benign cardiac tumors. [2],[3] These tumors can arise in any part of the heart, including the pericardium. Hence, the symptomatology can be varied (depending on the location) ranging from dyspnea, palpitation and chest pain to features related to dysrrhythmia, outflow tract obstructions or cardiac failure. [2],[4] In a report of their case, Pigato et al.,[4] found that cardiac hemangiomas had a wide age-range of occurrence and a slight female predominance; only two of the 34 tumors that were reviewed (5.9%) were found to be asymptomatic. The hemangioma in this case was present in a 35-year-old male and was present in the right ventricular outflow tract and situated below the pulmonary valve. Such locations have been infrequently reported and Kann et al.,[5] found only six cases in their review. The lesions had been polypoidal and produced significant outflow tract obstruction. We had seen multiple polypoidal lesions which at autopsy did not appear to produce obstruction, but there would have definitely been an increase in size of the lesions during life in systole. We also noted that the endocardial lesion was composed of both cavernous vessels as well as thick-walled ones, features described for the rare arteriovenous hemangiomas. [6] In addition, the subendocardial region revealed presence of thick-walled and dysplastic vessels, which has not been described before. The natural history of hemangiomas is variable; some remain dormant while others may proliferate. Hence a surgical excision is advocated, especially when symptoms are present, and bearing in mind that there may a chance of recurrence.

   References Top

1.Butany J, Nair V, Naseemuddin A, Nair GM, Catton C, Yau T. Cardiac tumours: Diagnosis and management. Lancet Oncol 2005;6:219-28.  Back to cited text no. 1
2.Burke A, Johns JP, Virmani R. Hemangiomas of the heart. A clinicopathologic study of ten cases. Am J Cardiovasc Pathol 1990;3:283-90.  Back to cited text no. 2
3.Esmaeilzadeh M, Jalalian R, Maleki M, Givtaj N, Mozaffari K, Parsaee M. Cardiac cavernous hemangioma. Eur J Echocardiography 2007;8:487-9.  Back to cited text no. 3
4.Pigato JB, Subramaniam VA, McCaba JC. Cardiac hemangioma. A case report and discussion. Tex Heart Inst J 1998:25:83-5.  Back to cited text no. 4
5.Kann BR, Kim WJ, Cilley JH, Marra SW, Delrossi AJ. Hemangioma of the right ventricular outflow tract. Ann Thorac Surg 2000;70:975-7.  Back to cited text no. 5
6.Tse TS, Tsui KL, Ling LC, Chui WH, Choi MC, Li SK, et al. Necrotic cardiac hemangioma masquerading as sepsis with disseminated intravascular coagulation. Hong Kong Med J 2005;11:308-10.  Back to cited text no. 6

Correspondence Address:
Pradeep Vaideeswar
Department of Pathology (Cardiovascular and Thoracic Division), Seth GS Medical College, Parel, Mumbai - 400 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.85132

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