Multimodality Imaging in Right Heart Tumors: Proposed Algorithm towards an Appropriate Diagnosis

Abstract

A right heart tumor can be identified by transthoracic echocardiography during a routine examination or due to cardiac symptoms. The first step is the assessment by echocardiography, with its multiple techniques, and the obtained information must be judged in a clinical and biological context. The second step comprises one, sometimes even two, of the more complex modality imaging methods. The choice is driven not only by the advantages of each imaging technique but also by local expertise or the preferred imaging modality in the center. This step is followed by staging, follow-up, and/or imaging-guided excision or biopsy, which is performed in selected cases in order to obtain anatomopathological confirmation. In the presence of features suggestive of malignancy or causing hemodynamic impairment, a transvenous biopsy is essential before the more complex imaging modalities (which are still relevant in the staging process). Using a structured imaging approach, it is possible to reach an appropriate diagnosis without a biopsy. Frequently, these imaging techniques have a complementary role, so an integrated imaging approach is recommended. This proposed algorithm for appropriate diagnosis of right heart tumors could serve as a practical guide for clinicians (not only imaging specialists).

Keywords: cardiac magnetic resonance; computer tomography; echocardiography; imaging; multimodality; positron emission tomography; right heart; tumor.

Figure 1

Two-dimensional transthoracic echocardiography (apical 4-chamber view) with color Doppler showing external compression of the free right ventricular wall (arrow) in a patient with hepatomegaly due to a hepatic tumor, which explains hemodynamic instability (similar to a localized cardiac tamponade). AO, aorta; IAS, interatrial septum; LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.

Figure 2

Two−dimensional transesophageal echocardiography (short-axis view at the level of the great vessels) showing a huge inhomogeneous mass (arrow) attached to the interatrial septum crossing the tricuspid valve (which, upon histology diagnosis, was a myxoma). AO, aorta; IAS, interatrial septum; LA, left atrium; RA, right atrium; RV, right ventricle.

Figure 3

Two-dimensional transthoracic echocardiography (apical 4-chamber view) showing a Chiari network (arrow) in the right atrium before and after (the last image) an electrophysiological study complicated by catheter entrapping in the Chiari apparatus. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.

Figure 4

Two-dimensional transthoracic echocardiography (modified parasternal view, long axis) showing a moderator band in the right ventricle. Ao, aorta; IVS, interventricular septum; LA, left atrium; LV, left ventricle; RV, right ventricle.

Figure 5

Two-dimensional transesophageal echocardiography (short-axis view at the level of the great vessels) showing a mass (arrow) on the port-a-cath in a patient with neoplasia and chemotherapy. It was an incidental finding. The mass disappeared after initiating anticoagulation treatment. AO, aorta; IVS, interventricular septum; LA, left atrium; LV, left ventricle; RV, right ventricle.

Figure 6

Two-dimensional transesophageal echocardiography (short-axis view at the level of the great vessels) showing a mass (arrow) in the right atrium on the pacemaker leads. PM, pacemaker; RA, right atrium; RV, right ventricle; TV, tricuspid valve.

Figure 7

Two−dimensional transesophageal echocardiography with color Doppler (high transesophageal view at 119°) showing two tumors (arrow) at the level of the interatrial septum with compression on the right superior pulmonary vein (right image). LA, left atrium; LV, left ventricle; PFO, permeable foramen oval; RA, right atrium; RSPV, right superior pulmonary vein.

Figure 8

Multimodality imaging diagnostic algorithm for right heart tumors. TTE, transthoracic echocardiography; TEE, transesophageal echocardiography; UEAs, ultrasound-enhancing agents; CT, computer tomography; CMR, cardiac magnetic resonance; PET–CT, positron emission tomography-computer tomography.