Ann Thorac Surg 2003;76:2097-2099
© 2003 The Society of Thoracic Surgeons
Case report
Cavernous hemangioma of the tricuspid valve: minimally invasive surgical resection
Elisabetta Lapenna, MD*a,
Michele De Bonis, MDa,
Lucia Torracca, MDa,
Giovanni La Canna, MDa,
Giacomo Dell’Antonio, MDb,
Ottavio Alfieri, MDa
a Department of Cardiac Surgery, Milan, Italy
b Department ofSurgical Pathology, "San Raffale" University Hospital, Milan, Italy
Accepted for publication April 23, 2003.
* Address reprint requests to Dr Lapenna, Divisione di Cardiochirurgia, IRCCS Ospedale San Raffaele, Via Olgettina, 60, 20132 Milan, Italy
e-mail: elisabettalapenna{at}tiscalinet.it
 |
Abstract
|
|---|
Cardiac hemangioma is a rare, benign vascular tumor, occurring with an incidence of 1% to 2% of all detected benign heart neoplasms. Hemangioma of the tricuspid valve has never been previously reported. We describe the successful excision of this tumor through a right anterolateral mini-thoracotomy in a 49-year-old woman. Competency of the valve was confirmed intraoperatively and at discharge by transesophageal echocardiography.
|
Introduction
|
|---|
Cardiac hemangiomas are rare, benign neoplasms, with only 37 reported cases involving surgical treatment and none primarily arising from the tricuspid valve. A variety of clinical presentations may be seen, depending on the size and location of the tumor. These tumors can be completely asymptomatic, but they can also precipitate conductive and hemodynamic abnormalities resulting in sudden death. Because of the natural, unpredictable history of these tumors, surgical intervention is mandatory for resectable lesions. We report the case of a cavernous hemangioma of the septal leaflet of the tricuspid valve successfully resected using a minimally invasive surgical approach.
A healthy 49-year-old white woman was referred to our department with a history of syncopal episodes and an echocardiographic diagnosis of cardiac tumor localized on the tricuspid valve. No apparent abnormality was found on electrocardiogram and chest roentgenogram. A transesophageal echocardiogram demonstrated a 3 x 2 cm large mass with a thin capsule and an echo-free content, which was attached to the base of the septal leaflet of the tricuspid valve or possibly to the annulus (Fig 1A).
The mass appeared peduncolated, mobile, and prolapsing into the right ventricle (Fig. 1B). A mild tricuspid regurgitation was documented. Coronary angiogram showed normal coronary arteries. We did not perform computed tomography or magnetic resonance imaging, because we believed that these would not provide any additional, relevant information to decide whether or not to operate. Therefore the patient was taken to the operating room for resection of the lesion.
View larger version (46K):
[in this window]
[in a new window]
|
Fig 1. (A) Transesophageal echocardiogram demonstrating a bubble-like 2-cm mass (arrow) with a thin capsule and echo-free content, which was attached to the base of the septal leaflet of the tricuspid valve and prolapsed in the right ventricle during diastole. (AO = aortic valve; LA = left atrium; RA = right atrium; RV = right ventricle; TV = tricuspid valve; X = hemangioma.) (B) Transthoracic echocardiogram showing significant alteration of the diastolic flow across the tricuspid valve with two evident eccentric jets (arrows) around the mass. (H = hemangioma; LV = left ventricle.)
|
|
A 7-cm right anterolateral mini-thoracotomy was performed. The right femoral artery and vein and the right internal jugular vein were used for cannulation. Mild hypothermic cardiopulmonary bypass was established, the aorta was clamped directly using the transthoracic clamping (Chitwood) technique, and cold blood cardioplegia was delivered antegradely. After conventional right atriotomy, a gross inspection demonstrated a bluish round-shaped mass of about 2 cm in diameter, arising from the septal tricuspid valve leaflet close to the annulus (Fig. 2A).
This peduncolated lesion was excised en bloc and appeared fairly well capsulated (Fig 2B). The aortic clamping time was 15 minutes and the cardiopulmonary bypass time was 40 minutes. Postoperative transesophageal echocardiogram demonstrated a normal appearing tricuspid valve with no tricuspid regurgitation. The patient had an uncomplicated recovery and was discharged home on postoperative day 4. Histopathologic examination revealed a plurilocular, well-demarcated lesion with cavernous cysts filled with blood, having thick or thin fibroelastic walls. Areas of capillary type vessels and focal papillary endothelial hyperplasia were present. Immunohistochemical staining with the endothelial marker CD31 (DAKO, Denmark, Europe) confirmed the diffuse presence of an internal layer of thin endothelial cells. Thus the diagnosis of benign cavernous hemangioma was made (Fig 3).
View larger version (89K):
[in this window]
[in a new window]
|
Fig 2. Operative view. (a) Cavernous hemangioma (arrow) arising from the septal leaflet of the tricuspid valve. (b) The resected tumor, approximately 2 cm in diameter.
|
|
View larger version (160K):
[in this window]
[in a new window]
|
Fig 3. Histologic examination showing large vessels connected irregularly and filled with blood. (Hematoxylin & eosin, x100, original magnification.) Inset: strong and diffuse positivity at CD31 immunohistochemical staining. (x400, original magnification.)
|
|
At 3 months follow-up the patient is doing well, and the echocardiogram reveals no tumor recurrence and no evidence of valve regurgitation.
|
Comment
|
|---|
Histologically, hemangiomas are benign vascular tumors, composed either by capillaries or cavernous channels and localized frequently in the liver, skin, subcutaneous tissues, and mucous membranes [1]. Although any organ can be involved, it is extremely rare to occur as a primary tumor of the heart. In a review by McAllister [2] of 533 primary tumors and cysts of the heart and pericardium, hemangiomas were present in only 2.8% of patients. Even though this tumor can be localized in any portion of the heart and pericardium, its growth on the tricuspid valve, as in the present case, is exceptional. Among 37 reported cases of surgically resected cardiac hemangiomas, none involved primarily the tricuspid valve except one attached to the papillary muscle of the tricuspid valve [3–4]. Therefore, we believe that this is the first case of cardiac hemangioma arising from the tricuspid valve leaflet ever described.
Hemangiomas can present in every age group with a modest female predominance and a broad spectrum of symptoms depending on the location and extention of the tumor (pericardial effusion, dysrhythmia, embolization, outflow tract obstruction, and signs of heart failure). Pseudo-angina has been reported in cases of compression of epicardial coronary arteries by the tumor tissue. Some asymptomatic presentations have also been described [5]. Echocardiography, either transthoracic or transesophageal, usually reveals the mass and should be used as an initial imaging modality. In our case, the echocardiographic findings of a thin-walled mass with an echo-free space within it and its location near the fibrous skeleton of the heart could also suggest the diagnosis of a blood-filled cyst, making the differential diagnosis really difficult [6]. More recently, contrast-enhanced computed tomographic scans and magnetic resonance imaging have been suggested as better techniques to evaluate the extracardiac extension of the tumor and the amount of myocardial involvement [7]. Coronary angiography may establish the diagnosis of hemangioma due to tumor vascularity, by showing the characteristic "tumor blush" [5]. The natural history of these tumors is extremely variable. They may remain dormant and clinically silent or may proliferate indefinitely [8]. A spontaneous tumor resolution during a 2-year follow-up time has also been reported [2]. However, because hemangiomas may cause sudden death due to arrhythmias or pericardial effusion [5], surgery is indicated to confirm the diagnosis and to excise the tumor when technically feasible [8]. Usually long-term prognosis is extremely favorable after surgical resection [5]. In this specific case, considering the young age of the patient and the favorable location and shape of the mass, a minimally invasive approach could be successfully used with both excellent cosmetic and clinical results.
|
References
|
|---|
- Matsumoto Y., Watanabe G., Endo M., Sasaki H. Surgical treatment of a cavernous hemangioma of the left atrial roof. Eur J Cardiothorac Surg 2001;20:633-635.[Abstract/Free Full Text]
- McAllister H. Tumors of the heart and pericardium. In: Silver M.D., ed. Cardiovascular pathology. New York: Churchill Livingstone, 1983:909-943.
- Burke A., Virmani R. Tumors of the heart and great vessels. In: Rosai J., Sobin L.H., eds. Atlas of tumor pathology, 3rd series, fascicle 16. Washington, DC: Armed Forces Institute of Pathology, 1996:80-86.
- Cunningham T., Lawrie G.M., Stavinoha J., Jr, Quinones M.A., Zoghbi W.A. Cavernous hemangioma of the right ventricle: echocardiographic-pathologic correlates. J Am Soc Echocardiogr 1993;6(3 Pt 1):335-340.[Medline]
- Brizard C., Latremoulli C., Jebara V.A., et al. Cardiac hemangiomas. Ann Thorac Surg 1993;56:390-394.[Abstract/Free Full Text]
- Sim E.K., Wong M.L., Tan K.T., Sim S.K. Blood cyst of the tricuspid valve. Ann Thorac Surg 1996;61:1012-1013.[Abstract/Free Full Text]
- Kemp J.L., Kessler R.M., Raizada V., Williamson M.R. MR and CT appearance of cardiac hemangioma. J Comput Assist Tomogr 1996;58:184-185.
- Kann B.R., Kim W.J., Cilley J.H., Marra S.W., DelRossi A.J. Hemangioma of the right ventricular outflow tract. Ann Thorac Surg 2000;70:975-977.[Abstract/Free Full Text]
This article has been cited by other articles:
|
|
|
|
 
X. Chen, A. J. Lodge, L. R. Dibernardo, and C. A. Milano
Surgical treatment of a cavernous haemangioma of the heart
Eur J Cardiothorac Surg,
December 21, 2011;
(2011)
ezr153v1.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Ayadi-Kaddour, M. Abid, Y. Harrath, B. Smati, T. Kilani, and F. El Mezni
Asymptomatic hemangioma of the interatrial septum.
J. Thorac. Cardiovasc. Surg.,
September 1, 2006;
132(3):
691 - 692.
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
