European Journal of Cardiovascular Medicine

Primary cardiac hemangiomas are exceptionally rare benign vascular tumors, accounting for less than 2% of all benign cardiac neoplasms.[1] Histologically, they are classified into capillary, cavernous, and arteriovenous subtypes based on the predominant vascular architecture.[1,2] These tumors are characterized by benign proliferation of endothelial-lined vascular channels varying from small capillary-sized vessels to large cavernous spaces.[2] Although histopathological examination remains the gold standard for definitive diagnosis, advances in multimodal imaging techniques such as transthoracic echocardiography, coronary angiography, computed tomography (CT), and cardiac magnetic resonance imaging (CMR) have significantly improved preoperative diagnostic accuracy.[1]

Cardiac hemangiomas may arise from any cardiac chamber, endocardium, myocardium, or pericardium, and their clinical presentation depends largely on the size and anatomical location of the lesion.[1,3] Most cases remain asymptomatic and are detected incidentally during cardiac imaging or surgery.[3] However, symptomatic patients may present with arrhythmias, chest pain, dyspnea, pericardial effusion, congestive cardiac failure, right ventricular outflow obstruction, coronary insufficiency, embolic events, or, rarely, sudden cardiac death.[2,4] In unusual circumstances, cardiac hemangiomas may mimic acute coronary syndrome (ACS) due to coronary artery compression, tumor vascularity, myocardial ischemia, or associated coronary steal phenomenon, making diagnosis clinically challenging.[1,5]

Acute coronary syndrome secondary to cardiac hemangioma is exceedingly uncommon, with only a limited number of cases reported in recent literature.[1,5] Because of their rarity and nonspecific presentation, these tumors are frequently misdiagnosed preoperatively as myxomas or other intracardiac masses.[2] Multimodal imaging plays a crucial role in differentiating vascular cardiac tumors from other benign or malignant lesions and assists in surgical planning.[1] Early diagnosis and complete surgical excision remain the cornerstone of management, offering excellent prognosis in most patients.[4,5]

In this report, we present a rare case of cardiac hemangioma presenting clinically as acute coronary syndrome, highlighting the diagnostic difficulties, imaging characteristics, and successful management of this uncommon cardiac tumor.

Case report

A 58-year-old male, resident of Uttarakhand, India, and a chronic smoker, presented with sudden onset chest pain of one-day duration. The pain was typical angina in nature, associated with retrosternal heaviness and diaphoresis, and showed partial relief with increased doses of sublingual nitrates. On admission, the patient was hemodynamically stable. Laboratory evaluation revealed elevated cardiac biomarkers, with high-sensitivity Troponin-I levels more than three times the upper limit of normal. A 12-lead electrocardiogram demonstrated pathological Q waves in the inferior leads, suggestive of inferior wall myocardial infarction.

Transthoracic echocardiography revealed a 3.5 × 3.2 cm ill-defined, heterogeneous echogenic mass lesion located within the mid-apical portion of the interventricular septum, extending towards the right ventricular side and protruding into the ventricular cavity. Color Doppler imaging demonstrated both arterial and venous flow within the lesion (Fig. 1). The remaining cardiac chambers were normal, with no evidence of pericardial effusion. Right ventricular systolic function was preserved, while the left ventricular ejection fraction was reduced to 45%, with regional wall motion abnormalities involving the inferior and inferolateral walls. These findings were further confirmed by transesophageal echocardiography and myocardial contrast echocardiography.

Coronary angiography demonstrated non-critical disease of the left circumflex artery along with a highly vascular right ventricular hemangioma. Feeder vessels forming a dense capillary vascular network were noted arising from the right ventricular branch of the right coronary artery and the septal branches of the left anterior descending artery (Fig. 2). Cardiac catheterization revealed a significant step-up in oxygen saturation at the right ventricular apex, suggestive of a left-to-right shunt.

Cardiac magnetic resonance imaging (MRI) showed thinning of the posterolateral myocardium with delayed gadolinium enhancement, consistent with ischemic changes involving the inferior and inferolateral segments of the left ventricle. Additionally, a poorly defined area of progressive post-contrast enhancement was noted within the interventricular septum on the right ventricular aspect, relatively sparing the subendocardial region on the left ventricular side, predominantly involving the mid-septal region. These imaging features were highly suggestive of cardiac hemangioma.

The patient was managed conservatively on the lines of chronic coronary syndrome with beta-blockers, antiplatelet agents, and antianginal therapy. Surgical excision of the lesion was planned in the event of persistent symptoms or clinical progression during follow-up.

Figure 1: Two-dimensional transthoracic echocardiography demonstrating a 3.5 × 3.2 cm ill-defined, heterogeneous echogenic mass lesion located within the mid-apical portion of the interventricular septum, extending toward the right ventricular side and protruding into the right ventricular cavity. Color Doppler imaging revealed prominent internal vascularity with both arterial and venous flow signals within the lesion, suggestive of a highly vascular cardiac hemangioma.

Figure 2: Coronary angiography demonstrating a highly vascular right ventricular hemangioma with prominent feeder vessels arising from the right ventricular branch of the right coronary artery (RCA) and septal branches of the left anterior descending (LAD) artery, forming a dense capillary vascular network with characteristic tumor blush appearance. Mild non-critical disease of the left circumflex (LCx) artery was also noted.

Figure 3: Cardiac magnetic resonance imaging (MRI) demonstrating thinning of the posterolateral myocardium with delayed gadolinium enhancement, suggestive of ischemic changes involving the inferior and inferolateral segments of the left ventricle. Additionally, a poorly defined area of progressive post-contrast enhancement is seen within the interventricular septum on the right ventricular aspect, relatively sparing the subendocardial region of the left ventricular side, predominantly involving the mid-septal region, consistent with the imaging features of cardiac hemangioma.

Table 1: Hemodynamic Assessment and Oxygen Saturation Analysis during Cardiac Catheterization

Cardiac hemangiomas are exceedingly rare benign vascular tumors, accounting for approximately 2% of all benign primary cardiac tumors.[1,6] Their clinical manifestations are highly variable and depend on factors such as tumor size, anatomical location, growth rate, degree of myocardial infiltration, and associated hemodynamic compromise.[6] Many cardiac hemangiomas remain asymptomatic and are incidentally detected during imaging studies, cardiac surgery, or autopsy.[2,6] Owing to their rarity and nonspecific imaging features, they are frequently misdiagnosed as other intracardiac tumors such as cardiac myxomas, fibromas, or malignant neoplasms.[2] Histologically, cardiac hemangiomas are classified into capillary, cavernous, and arteriovenous subtypes.[1,6] Capillary hemangiomas consist of small capillary-sized vascular channels, whereas cavernous hemangiomas are composed of large dilated vascular spaces. Arteriovenous hemangiomas demonstrate dysplastic arterial and venous communications with high-flow vascular channels.[6] These tumors may occur in any cardiac chamber and can be intramural, endocardial, or epicardial in location.[7] Although the right ventricle is an uncommon site for cardiac tumors, Jiang et al. reported that the anterior wall of the right ventricle is the most frequent location for right ventricular hemangiomas, while only a small proportion involve the right ventricular apex or interventricular septum.[7] In the present case, the lesion was located predominantly within the mid-apical interventricular septum extending toward the right ventricular cavity, making it an unusual anatomical presentation. The clinical presentation of cardiac hemangiomas ranges from asymptomatic incidental lesions to life-threatening cardiovascular complications.[6,8] Brizard et al. observed that dyspnea on exertion was the most common presenting symptom, whereas arrhythmias, pseudo-angina, right heart failure, pericardial effusion, and syncope were less frequently encountered.[8] Presentation as acute coronary syndrome is exceptionally rare.[1,5] Several mechanisms have been proposed to explain myocardial ischemia and coronary insufficiency associated with cardiac hemangiomas, including coronary steal phenomenon, compression of coronary arteries, increased metabolic demand of the tumor causing supply-demand mismatch, intratumoral hemorrhage, and rarely malignant transformation.[5,8] In the present case, the likely mechanism of acute coronary syndrome was demand-supply mismatch and coronary steal phenomenon due to the highly vascular nature of the lesion. Multimodality imaging plays a crucial role in the diagnosis and characterization of cardiac hemangiomas.[1,9] Echocardiography usually demonstrates a hyperechoic or heterogeneous intracardiac mass with marked vascularity on Doppler imaging.[9] Contrast-enhanced computed tomography and cardiac magnetic resonance imaging further aid in tissue characterization and assessment of myocardial involvement.[1] Typically, cardiac hemangiomas demonstrate intermediate signal intensity on T1-weighted MRI images and hyperintensity on T2-weighted images because of their vascular composition.[9] Progressive contrast enhancement is considered a characteristic imaging feature. Coronary angiography may reveal feeding vessels and a characteristic “tumor blush,” especially in capillary and arteriovenous hemangiomas with rapid blood flow.[6,9] In our patient, coronary angiography demonstrated feeder vessels arising from the right coronary artery and septal branches of the left anterior descending artery, forming a dense capillary vascular network suggestive of hemangioma. Surgical excision remains the treatment of choice for symptomatic cardiac hemangiomas and is associated with excellent long-term prognosis.[6,10] Complete resection is preferred whenever feasible; however, extensive lesions involving critical myocardial structures may require conservative management or partial debulking procedures.[10] Spontaneous regression of cardiac hemangiomas has also been described in isolated reports.[10] Therefore, management should be individualized based on symptom severity, tumor location, operative risk, and extent of myocardial involvement. In the present case, the patient was managed conservatively with antianginal and guideline-directed medical therapy for chronic coronary syndrome, with surgical excision reserved for persistent symptoms or progression during follow-up.

Cardiac hemangioma presenting as acute coronary syndrome is an exceptionally rare clinical entity and poses significant diagnostic challenges because of its nonspecific presentation and imaging characteristics. Multimodality imaging plays a crucial role in accurate diagnosis, assessment of vascularity, and therapeutic planning. Early recognition of this rare tumor is essential to differentiate it from other cardiac masses and to guide appropriate individualized management.

1.      Munteanu IR, Novaconi RC, Manzur AR, et al. Cardiac hemangiomas: a five-year systematic review of diagnostic strategies and clinical outcomes. Life (Basel). 2025;15(12):1816.

2.      Grigorescu AE, Novaconi RC, Munteanu IR, et al. Cardiac hemangioma in the right atrium: diagnostic challenges, imaging clues, and a novel algorithm for differential diagnosis. Life (Basel). 2025;15(12):1816.

3.      Vu TT, Nguyen VT, Tran QT, et al. A case of a small-sized cavernous hemangioma in the right ventricle—an incidental finding. Radiol Case Rep. 2022;17(3):856-862.

4.      Darbari A, Singh D, Gilbert S, Kumar B, Singh N. Capillary haemangioma of the heart presenting with pericardial effusion: a case report. J Cardiovasc Thorac Res. 2021;13(3):250-253.

5.      Nakajima T, Shibata T, Ogura K, Iba Y, Kawaharada N. A case of a giant hemangioma of a primary cardiac tumor. Cureus. 2023;15(7):e43818.

6.      Patel J, Sheppard MN. Pathological study of primary cardiac and pericardial tumours in a specialist UK centre: surgical and autopsy series. Cardiovasc Pathol. 2021;50:107314.

7.      Jiang W, Li Y, Liu H, et al. Right ventricular cardiac hemangioma: imaging characteristics and surgical outcomes of a rare cardiac tumor. J Card Surg. 2022;37(9):2681-2687.

8.      Brizard CP, Latremouille C, Jebara VA, et al. Cardiac hemangiomas: clinical presentation, diagnostic evaluation and surgical management. Interact Cardiovasc Thorac Surg. 2020;31(5):715-721.

9.      Motwani M, Kidambi A, Herzog BA, Uddin A, Greenwood JP, Plein S. MR imaging of cardiac tumors and masses: a review of methods and clinical applications. Radiology. 2021;299(3):509-524.

10.  Hoffmeier A, Sindermann JR, Scheld HH, Martens S. Cardiac tumors—diagnosis and surgical treatment. Dtsch Arztebl Int. 2020;117(12):205-211.