Cardiac venous malformations (also known as cardiac haemangiomas) consists of a slow flow venous malformation and is composed of numerous non-neoplastic endothelial-lined thin-walled channels with interspersed fat and fibrous septae.
It is important to note that according to newer nomenclature (ISSVA classification of vascular anomalies) these lesions are merely known as venous malformations. Having said that it is probably helpful in reports to include the word 'haemangioma' in brackets as this term is ubiquitous in the literature and most familiar to many clinicians.
Venous malformations are found throughout the body. This article focuses on cardiac cavernous haemangiomas. For a general discussion please refer to the general article on cavernous venous malformation.
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)
- 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, the occurrence is possible anywhere from pericardium to the 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.
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 a 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 an 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.
Treatment and prognosis
Cardiac haemangiomas can be successfully excised, and surgical resection is the treatment of choice for symptomatic lesions or when the 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.
Potential complications include:
- development of cardiac arrhythmias
- intracavitatory growth may cause
- ventricular outflow tract obstruction
- valvular compromise
- disruption of intracardiac blood flow leading to congestive heart failure and hydrops
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)
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Vascular tumours and malformations
vascular malformations and tumours
- vascular tumours
- tufted angioma (with or without Kasabach Merritt syndrome)
- Kaposiform haemangioendothelioma (with or without Kasabach Merritt syndrome)
- spindle cell haemangioendothelioma
- other, rare hemangioendotheliomas
- dermatologic acquired vascular tumors
- slow flow vascular malformations
- capillary malformation (CM)
- venous malformation (VM)
- common sporadic venous malformation
cavernous venous malformation (cavernoma or cavernous haemangioma)
- cerebral cavernous venous malformation
- orbital cavernous venous malformation
- hepatic cavernous venous malformation (hepatic haemangioma)
- splenic cavernous venous malformation (splenic haemangioma)
- breast venous malformation (breast haemangioma)
- retroperitoneal venous malformation
- soft tissue venous malformation
- primary intraosseous venous malformation
- cardiac venous malformations
- cavernous venous malformation (cavernoma or cavernous haemangioma)
- Bean syndrome
- familial cutaneous and mucosal venous malformation
- glomuvenous malformation (glomangioma)
- Maffucci syndrome
- common sporadic venous malformation
- lymphatic malformation (LM)
- fast flow vascular malformations
- arterial malformation
- arterial malformation
- arteriovenous fistulae (with one or more shunts)
- arteriovenous malformations (with a nidus of multiple shunts)