CARDIAC HEMANGIOMACARDIAC HEMANGIOMA
M. Hekmat MD,* R. Khodaverdain MD,* Z.H. Ahmadi MD,* Mohammad Khani MD,** P. Shishineh MD***
*Department of Cardiac Surgery, Modarres Hospital, Shahid Beheshti University of Medical Sciences Tehran, **Department of Cardiology, Zandjan University of Medical Sciences, Zandjan, and***Department of Pathology, Modarres Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
A 32-year-old female referred with
gradually progressive dyspnea for 9 months duration. 2D
echocardiography showed a right atrial tumor. With
impression of atrial myxoma the patient underwent
resection of tumor under cardiopulmonary by-pass, and
cardiac arrest. A 5x3 cm polypoid mass was found adherent
to interatrial septum which was then excised with a rim
of septum. The defect was then repaired with a patch of
pericardium. The patient had an uneventful post-operative
course and was discharged in the 8th
post-operative day.
Histologically the tumor was consistent with cavernous
hemangioma. Hemangiomas are uncommon primary tumors of
the heart. The most common site of involvement is the
right ventricle; it is however, rarely seen in the right
atrium.
Keywords · Cardiac Hemangioma · right atrium · cardiopulmonary by-pass
Introduction
Among primary cardiac tumors, hemangiomas are relatively rare. McAllister reviewed 533 cases of primary cardiac tumors; there were only 15 cases of hemangioma.1 To date, less than forty cases are reported in litrature.
Case Report
A 32-year-old female presented with the chief complaint of shortness of breath of 9 months duration, which had gradually increased in intensity. She had also noticed episodes of palpitation. There was no history of cough, no cyanosis or syncope. General Physical examination was unremarkable except for mild cardiomegaly which was evident on Chest X-Ray. The ECG was normal. Two-dimensional echocardiography showed a right atrial tumor, which was echodense, and adherent to inter-atrial septum. Moderate TR was also noted. The patient was operated upon under cardiopulmonary by-pass, and moderate hypothermia with the preoperative diagnosis of atrial myxoma. The right atrium was opened and a 5x3 cm polypoid mass was found which was severely adherent to interatrial septum (Figure 1). The mass was excised with a rim of septum and the defect was then repaired with a patch of pericardium. The patient had an uneventful post-operative course and was discharged on the 8th post-operative day. The specimen consisted of a round piece of tissue measuring 5 cm in diameter and was partially covered by a thin capsule of 0.2 cm thickness. Serial cut sections showed red to brown small cystic structures filled with blood. Histological examination revealed a highly vascular tumor with infiltration of chronic inflammatory elements and multiple tortuous vascular channels filled with RBCs. There were many large cystic spaces lined with endothelial cells, compatible with cavernous hemangioma (Figure2).
During a 14-month follow-up, the patient was free of symptoms and serial echocardiograph examinations revealed no evidence of recurrence.
Discussion
Cardiac hemangiomas are composed of a benign proliferation of endothelial cells that are histologically identical to hemangiomas elsewhere in the body. These lesions may involve the endocardium, myocardium, or epicardium. Bizzard et al found that 21% of cardiac hemangioma involved the anterior wall of the right ventricle and 21% the lateral wall of the left ventricle.2 In one study, the locations of the tumors in order of frequency were: right ventricle, left ventricle, atrial septum and right atrium.3-7
The symptoms depend on the anatomic location of the tumor. The most common symptoms are dyspnea, palpitation, atypical chest pain and arrhythmia. When the tumor is situated on the right side of the interventricular septum, it may simulate an infundibular pulmonary stenosis.5 In some instances, tumors developing in the pericardium have produced pericardial effusion.8 Histologically, vascular tumors of the heart are classified according to the predominant type of the proliferating vascular channels. They are lined by endothelial cells with moderately pleomorphic, and sometimes atypical nuclei with focal tuft formation.
Mitosis is extremely rare. Most reported hemangiomas are small, and range from 2 to 3.5 cm in diameter. The lesions are sessile or polypoid and usually single. At times CH has been associated with other angiomas, mainly cutaneous or visceral; a condition that constitutes diffuse angiomatosis.5
Surgical excision is the mainstay of treatment because of the benign nature of the tumor. Wide excision is not necessary while incomplete excision may cause tumor recurrence.10 Resectable cases usually have a good prognosis, in contrast to unresectable tumors cases which have a poor prognosis and may lead to ventricular arrhythmia, local progression and sudden death.
References