Capillary Hemangioma of the Left Ventricle.

We present the case of a young woman complaining of aspecific symptoms of malaise and dyspnea admitted to our Cardiology Department for investigations. Two-dimensional (2D)/3D transthoracic echocardiography showed an echogenic, sessile mass adhering to the midsegment of the posterior interventricular septum. The patient refused transesophageal echocardiography. For further investigation, a cardiac magnetic resonance imaging was performed, which raised suspicion of a benign tumor. Ultimately, the patient underwent uncomplicated cardiac surgery with total excision of the mass. Histopathology examination revealed a capillary hemangioma. Copyright:

CASE REPORT

A 33-year-old woman presented to our Cardiology Department for aspecific symptoms of malaise and dyspnea. She had a history of drug-addiction syndrome with borderline personality disorder, an emphysematous chronic obstructive pulmonary disease and a chronic hepatitis C virus-related hepatitis. Physical examination, chest X-ray, and electrocardiogram were normal. A two-dimensional (2D)/3D transthoracic echocardiography shown an echogenic, sessile mass (dimension 13 mm × 15 mm) adhering to the midsegment of the posterior interventricular septum; no evidence of myocardial infiltration, no valvular impairment, and no pericardial effusion; and preserved left ventricular ejection fraction with normal right chambers [Figure 1]. The patient refused transesophageal echocardiography.

Figure 1

Two-dimensional/three-dimensional transthoracic echocardiogram

Cardiac magnetic resonance (CMR) was performed on a 3T system (Skyra, Siemens Medical Solutions, Erlangen, Germany). The cine CMR images (steady-state free-precession sequence) confirmed the presence of a mobile small lesion, with regular borders (dimension 11 mm × 10 mm) attached to the midsegment of posterior interventricular septum [Figure 2]. The mass was isointense on T1-weight sequences and hyperintense on T2-weight sequences [Figure 3]. First-pass perfusion imaging with 0.1 mmol/kg of gadolinium-based contrast demonstrated partial vascularization of the mass. Late gadolinium enhancement images demonstrated a marked contrast uptake [Figure 3]. These findings suggested for a benign tumor.

Figure 2

Cardiac magnetic resonance steady-state free-precession sequences – four-chamber and short-axis view

Figure 3

Cardiac magnetic resonance T1, T2, and late gadolinium enhancement sequences – four-chamber view

Moreover, the patient underwent to a cerebral magnetic resonance, in which multiple hyperintensities of the white matter on T2 sequences were highlighted and interpreted as previous microembolic lesions in the presence of the cardiac mass. Therefore, an interdisciplinary cardiological and surgical evaluation was taken during which, by balancing risks and benefits, including the young age of the patient and the potential risk of other embolization, the decision of a surgical excision was taken.

The patient underwent surgical excision of the mass with extracirculatory support at 34°C through atriotomy approach avoiding left ventriculotomy, which provided adequate exposure of the mass with the least cardiac trauma. The mass appeared red, soft, with a small peduncle; it was adjacent to the interventricular septum and partially fused with the posteromedial papillary muscle. Total excision of the mass was performed, and its peduncle was thoroughly cauterized [Figure 4]. Recovery was uneventful. Histopathologic examination concluded for a capillary and cavernous hemangioma.

Figure 4

Intraoperatory image of the mass

DISCUSSION

Benign and malignant cardiac tumors are rare, with an estimated prevalence of only 0.002%–0.3% at autopsy. However, approximately 75% of all primary cardiac tumors are benign, and the most common in the adult population are myxomas (50%), papillary elastomas (20%), lipomas (15%–20%), and hemangiomas (5%). The other 25% of primary cardiac tumors are malignant, 95% of these are sarcomas and 5% are lymphomas. Metastases involving the heart and pericardium (secondary cardiac tumors) from direct invasion or hematologic spread are 20–40 times more common than primary cardiac tumors. However, the most common types of cardiac mass are, in fact, “pseudotumors,” such as intracardiac thrombus or misinterpreted normal anatomic variants.[1]

Even when benign, cardiac tumors can exert significant clinical effects by altering cardiac hemodynamics and by acting as embolic or arrhythmia substrates. Hemangiomas are benign tumors originating from the endothelial cells to form blood vessels and account approximately for 2.8% of benign cardiac tumors, have an equal age distribution, a female preponderance, and they are usually incidentally discovered.[2]

Cardiac hemangiomas are classified as capillary, cavernous, and arteriovenous. Endocardial hemangiomas are usually capillary or mixed capillary-cavernous. Intramural hemangiomas might be capillary, cavernous, or arteriovenous. Cardiac hemangiomas can occur in any chamber. They may be predominantly intramural or endocardial.

Macroscopically, they can be either polypoid or sessile. Histologically, they are nonencapsulated tumors.[3]

These formations may enlarge, remain stationary, or undergo involution.[456] Hemangiomas are mainly asymptomatic but can develop various severe and dangerous presentations including valvular obstruction, heart failure, chest pain, pericardial effusion with tamponade, systemic embolism, and even conduction disturbances with possible sudden death consequent to infiltration of the conduction pathways.

The gold standard diagnosis is made through transthoracic echocardiography, where cardiac hemangiomas are seen as hyperechogenic lesions, even though microscopic examination of the tissues provides definitive diagnosis. CMR can be essential in the differential diagnosis within other benign heart tumors by giving additional information regarding the soft tissues around the tumor and can be highly useful as a preoperative planning evaluation. These tumors typically have intermediate signal intensity on T1-weighted images and are hyperintense on T2-weighted images owing to the high vascularity of the mass.

Coronary angiography can occasionally diagnose hemangioma by showing the blood supply of the tumor (vascular blush), particularly in the capillary and arteriovenous types of hemangiomas, which exhibit rapid blood flow.

Surgical resection is the treatment of choice for symptomatic lesions or if the diagnosis is in question. In most cases, complete tumor excision is suggested to prevent serious complications of valvular, left or right ventricular outflow tract obstruction, or systemic embolism.

The long-term prognosis of patients with surgically treated lesions is usually excellent,[6] and the rate of recurrence after surgical resection is rather small. Spontaneous regression of a cardiac hemangioma though has also been reported.[7]

Surgery might not always be necessary, particularly for extensive and asymptomatic hemangiomas that would require complex and potentially hazardous excision.

The surgery techniques depend on structure's localization in the heart and the surgeon experience.

CONCLUSIONS

We describe a case of capillary hemangioma in a patient referred for aspecific symptoms of malaise and dyspnea. Magnetic resonance imaging plays a key role for establishing an accurate diagnosis and for guiding staging, prognosis, and appropriate therapy in confirmed tumors. Complete surgery excision of the mass was performed successfully avoiding serious complications such as systemic embolization and allowing the achievement of the definitive diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.