A cardiac haemangioma consists of a vascular malformation, and is composed of numerous endothelial-lined thin-walled channels with interspersed fat and fibrous septae.
Cardiac haemangiomas are rare, benign lesions with an incidence of 1-10% among all detected benign heart tumours.
Mostly, cardiac haemangiomas are asymptomatic. However, they may cause a pericardial effusion, asymptomatic murmur, arrhythmia, haemopericardium or cardiac tamponade, complete heart block or even sudden death. In addition, these tumours have been incriminated for neurological manifestations.
These vascular malformations are composed of numerous endothelial-lined thin-walled channels with interspersed fat and fibrous septa. Histological classification is according to the size of their vascular channels into three types: capillary (composed of smaller capillary-like vessels), cavernous (composed of multiple thin-walled, dilated vessels), and arteriovenous (composed of thick-walled dysplastic arteries, venous-like vessels, and capillaries). Cavernous and capillary hemangioma are the most frequently reported forms, whereas a combination of all three features also is commonly seen, as well as fibrous and fatty components.
Cardiac haemangiomas have been reported to occur in variable locations of the heart involving any cardiac layer, namely endo-, myo-, or pericardium. The left atrium has previously been suggested as the predominant location for cardiac tumours. However, recent evidence has shown no chamber predilection. Hence, occurrence is possible anywhere from pericardium to endocardium.
Cardiac haemangiomas can occur in the clinical setting of Kasabach-Merritt syndrome, which is characterised by multiple systemic haemangiomas associated with recurrent thrombocytopenia and consumptive coagulopathy.
Ultrasound / Echocardiography
On echocardiography, haemangiomas typically appear hyperechoic.
Coronary arteriography demonstrates the blood supply to the tumor, which is characterized by a vascular blush, particularly in the capillary and arteriovenous types of hemangiomas, which exhibit rapid blood flow. Cavernous haemangiomas have large vascular spaces with very slow flow and therefore do not typically enhance at angiography 8.
Heterogeneous appearance on unenhanced chest CT, and, in most cases, intensely enhanced at CT performed after contrast material administration. Foci of calcification may also be seen be seen.8
It is usually seen as a heterogeneous mass, with intermediate-to-high signal on T1-weighted images because of slow flow. On T2-weighted images, a diffusely high signal is seen. Contrast enhancement is heterogeneous, intense and prolonged except in low-flow lesions 1.
- development of cardiac arrhythmias
- intra cavitatory growth may cause
- ventricular outflow tract obstruction
- valvular compromise
- disruption of intra-cardiac blood flow leading to congestive heart failure and hydrops
Treatment and prognosis
Cardiac haemangiomas can be successfully excised, and surgical resection is the treatment of choice for symptomatic lesions or when diagnosis is in question.The long-term outcome of patients with surgically treated symptomatic lesions is excellent. Spontaneous regression of cardiac haemangiomas have been reported, and, therefore, surgery may not always be necessary, particularly for extensive but asymptomatic haemangiomas that would require complex and potentially hazardous excision.
Differentiation from malignancies and thrombus is very important, since it greatly affects prognosis and necessity of (acute) treatment.
Other differential considerations include:
- thrombus - intracardiac thrombus
- primary benign tumours
- primary malignant tumours
- pericardial malignancy
- cardiac metastasis(es)
- 1- Grebenc ML, Rosado de christenson ML, Burke AP et-al. Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation. Radiographics. 20 (4): 1073-103. Radiographics (citation) - Pubmed citation
- 2- Hoey ET, Mankad K, Puppala S et-al. MRI and CT appearances of cardiac tumours in adults. Clin Radiol. 2009;64 (12): 1214-30. doi:10.1016/j.crad.2009.09.002 - Pubmed citation
- 3- Zerbo S, Argo A, Maresi E et-al. Sudden death in adolescence caused by cardiac haemangioma. J Forensic Leg Med. 2009;16 (3): 156-8. doi:10.1016/j.jflm.2008.08.020 - Pubmed citation
- 4- Luna A, Ribes R, Caro P et-al. Evaluation of cardiac tumors with magnetic resonance imaging. Eur Radiol. 2005;15 (7): 1446-55. doi:10.1007/s00330-004-2603-y - Pubmed citation
- 5- Marrone G, Sciacca S, D'Ancona G et-al. A rare case of left ventricular intramural hemangioma diagnosed using 1.5-T cardiac MRI with histopathological correlation and successfully treated by surgery. Cardiovasc Intervent Radiol. 2010;33 (1): 164-8. doi:10.1007/s00270-009-9533-7 - Pubmed citation
- 6- Perchinsky MJ, Lichtenstein SV, Tyers GF. Primary cardiac tumors: forty years' experience with 71 patients. Cancer. 1997;79 (9): 1809-15. Pubmed citation
- 7- Hrabak-Paar M, Hübner M, Stern-Padovan R et-al. Hemangioma of the interatrial septum: CT and MRI features. Cardiovasc Intervent Radiol. 2011;34 Suppl 2 (S2): S90-3. doi:10.1007/s00270-010-0062-1 - Pubmed citation
- 8- Brizard C, Latremouille C, Jebara VA et-al. Cardiac hemangiomas. Ann. Thorac. Surg. 1993;56 (2): 390-4. Pubmed citation
- 9- Grizzard JD, Ang GB. Magnetic resonance imaging of pericardial disease and cardiac masses. Magn Reson Imaging Clin N Am. 2007;15 (4): 579-607, vi. doi:10.1016/j.mric.2007.08.010 - Pubmed citation
- 10- Burke A, Johns JP, Virmani R. Hemangiomas of the heart. A clinicopathologic study of ten cases. Am J Cardiovasc Pathol. 1991;3 (4): 283-90. Pubmed citation
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Haemangioma of the heart||✗|
|Hemangioma of the heart||✗|