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CardioNerds co-founder Dr. Dan Ambinder, series chair Dr. Teodora Donisan, and Dr. Sukriti Banthiya discuss cardiac tumors with Dr. Juan Lopez-Mattei, a nationally recognized expert in the fields of cardio-oncology and the director of cardiac imaging at the Lee Health Heart Institute. Here, we explore the topic of cardiac tumors, with a focus on distinguishing between primary and secondary tumors. We delve into the symptoms, diagnostic methods, and treatment options. Show notes were drafted by Dr. Sukriti Banthiya and episode audio was edited by CardioNerds Intern and student Dr. Diane Masket.

This episode is supported by a grant from Pfizer Inc.

This CardioNerds Cardio-Oncology series is a multi-institutional collaboration made possible by contributions of stellar fellow leads and expert faculty from several programs, led by series co-chairs, Dr. Giselle Suero Abreu, Dr. Dinu Balanescu, and Dr. Teodora Donisan. 

Enjoy this Circulation 2022 Paths to Discovery article to learn about the CardioNerds story, mission, and values.

US Cardiology Review is now the official journal of CardioNerds! Submit your manuscript here.

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Pearls and Quotes – Cardiac Tumors

1. Keep it simple when approaching an intracardiac mass; start with transthoracic echocardiography (TTE) and use transesophageal echocardiography (TEE) or cardiac magnetic resonance (CMR) based on the clinical context. Use TEE when suspecting valvular vegetations or thrombi & CMR for intracavitary cardiac masses.
2. Cardiac tumors can manifest with a variety of symptoms; however, they are more commonly diagnosed as an incidental finding!
3. When faced with the dilemma of selecting the most suitable imaging modality for evaluating a cardiac mass, consider the following hierarchy: begin with TTE as the first choice, followed by CMR. If the patient cannot undergo CMR, the next step is cardiac computed tomography (CT) or Fluorodeoxyglucose F18 positron emission tomography (FDG-PET).
4. TEE is especially useful for the evaluation of small, highly mobile cardiac masses!
5. Imaging cannot substitute a tissue diagnosis of cardiac masses. However, in cases of advanced malignancy, it may not always be necessary.

Show notes – Cardiac Tumors

Segment One: A big “picture” Approach to Cardiac Tumors

Let’s start with an overview of cardiac masses

• Neoplastic vs non-neoplastic
  • Neoplastic lesions can be further classified into Primary Cardiac Tumors (PCT’s) & Secondary Cardiac Tumor (SCT’s)
  • A majority of PCTs are benign (up to 90%!); however, rarely, they may be malignant.
  • SCTs are more common than PCTs, and, by definition, they are malignant tumors.

Now, let’s look at the tools you can use to aid with the diagnosis of cardiac masses…

• Step 1: Investigate the cardiac mass initially with TTE.
• Step 2: Collect clues through history-taking & examination.
  • If suspecting valvular vegetations (as in infective endocarditis!) or left atrial appendage thrombus, characterize the mass further with TEE.
  • Consider the possibility of metastatic cardiac tumors in patients with a known malignancy, as they are more common than primary cardiac tumors.
  • In cases where it is uncertain if the mass is a cardiac tumor or thrombus, use CMR to differentiate the two entities.
    • Some findings on TTE that support the presence of a thrombus include left ventricular dysfunction with segmental wall motion abnormalities and/or apical aneurysm as these result in local pockets of stasis (think: Virchow’s triad)
• Step 3: Put it all together!
  • Think about whether a tissue biopsy will be needed. If yes, determine whether a negative margin or open biopsy will be required.

Segment Two: Symptoms, Symptoms, Symptoms!

Cardiac tumors may be symptomatic and present in the 3 key ways as outlined below (Think COD 🐟). However, they are more commonly identified as incidental findings!

• Constitutional symptoms (fever, arthralgias, weight loss, malaise/fatigue)
• Obstruction – Interfering with blood outflow, arrhythmias, interference with valves causing regurgitation, pericardial effusion +/- tamponade (presyncope, syncope, dyspnea, chest discomfort)
• Distal embolization (pulmonary or systemic thromboembolic phenomenon)

When a metastatic tumor is present, distinguishing symptoms originating from the heart becomes challenging due to potential overlap with symptoms caused by the primary malignancy. This stands in contrast to cases of primary cardiac tumors like myxomas, where symptom localization to the heart is more straightforward.

Segment Three: Multimodality Imaging

Imaging modality	Best used for	Advantages	Disadvantages
TTE	Initial diagnostic modality   Masses arising from valves	Good spatial resolution   Understanding of hemodynamic significance of mass	Lack of tissue characterization   Poor acoustic window in select cases
TEE	Small highly mobile valvular lesions (<1cm)	Visualization of structures with greater accuracy compared to TTE   Use of enhancing agents can help differentiate vascular tumors from non-vascular & thrombus	Lack of tissue characterization
CMR	Differentiates tumor from thrombus.   Identifies non-tumor masses or “pseudo-masses,” e.g. cysts, lipomas	Tissue characterization w/ T1, T2 weighted imaging and gadolinium enhancement	Lower temporal resolution   Limited availability   Interference from implanted electrical devices
CT & FDG-PET	Differentiates benign from malignant tumors	Alternative to CMR in pts. w/ claustrophobia & older generation cardiac devices	CT with limited soft tissue & temporal resolution compared to CMR   Dietary preparation before FDG

Segment Four: The Issue With Tissue!

Tissue diagnosis is essential for the diagnosis of primary cardiac tumors; however, it may be less important for metastatic tumors to the heart in cases of known advanced-stage malignancies such as melanoma, breast, and lung.

An overview of a rare primary cardiac malignancy: Carney Complex!

A complex hereditary syndrome that affects multiple organs, including the heart, skin & endocrine organs.

• Epidemiology:
  • Autosomal dominant
  • Young age groups in both sexes
• Clinical presentation
  • Intra-cardiac/extra-cardiac myxomas; intra-cardiac myxomas are multiple, bilateral (atrial and ventricular) & multicentric.
  • Skin findings commonly include lentigines and blue nevi.
  • Endocrine abnormalities include Cushing syndrome, pituitary & adrenal adenomas, thyroid dysfunction.
• Diagnosis
  • Clues on CMR
    • SSFP (dteady-state free precession) cine imaging, an association of punctiform areas of high & low signal intensity consistent with “Blackberry appearance”
    • Hypoperfused enhancement pattern at first-pass perfusion imaging
    • High signal T2-imaging, iso-intense on T1-weighted imaging
• Treatment
  • Surgical resection
  • Annual surveillance for Carney complex as they frequently recur (compared with surveillance every 3-5 years for non-Carney myxomas)
  • Genetic testing for PRKAR1 mutations in 1st degree family members

Segment Five: “Secondary” to None

Secondary cardiac tumors (SCT’s)

• Epidemiology:
  • Cardiac metastasis is 20-40 times more common than primary cardiac tumors.
• Etiology:
  • Routes of spread: hematogenous, lymphatic, transvenous, direct invasion
  • Most common malignancies to metastasize to heart include melanomas, carcinomas of breast, lung and esophageal.
• Clinical Presentation
  • Pericardial effusion, tamponade
  • Arrhythmias – “resistant” to antiarrhythmic drugs
  • Heart failure due to myocardial infiltration
  • Valvular dysfunction due to intracavitary masses that impede blood flow.
• Diagnosis
  • Echocardiography is the initial test of choice.
  • MRI
    • Most malignancies exhibit low signal on T1-weighted imaging & high signal intensity on T2-weighted imaging.
    • Exception, metastaticmelanoma, which appears hyperintense on T1-weighted imaging due to paramagnetic T1 shortening effects of melanin.
  • FDG-PET/CT
    • In cardiac metastasis, the myocardium has high metabolic activity and can mimic FDG uptake seen in a tumor.
    • Ensure adequate dietary preparation to suppress glucose uptake of normal healthy myocardium.
• Multi-disciplinary approach to management
  • Tissue diagnosis is necessary.
    • Referral to an interventional cardiologist for transvenous biopsy, or
    • Referral to a cardiac surgeon for minimally invasive surgery
    • Note – tissue diagnosis can come from another metastatic site.
  • Pathologist to confirm the malignant nature of the tumor.
  • Cardio-oncologist to facilitate multidisciplinary team discussion involving oncologist and cardiac surgeon on the best approach to treatment.
    • Neoadjuvant chemotherapy or radiotherapy vs. cardiac surgery

References – Cardiac Tumors

1. Tyebally, Sara, Daniel Chen, Sanjeev Bhattacharyya, Abdallah Mughrabi, Zeeshan Hussain, Charlotte Manisty, Mark Westwood, Arjun K. Ghosh, and Avirup Guha. “Cardiac Tumors: JACC CardioOncology State-of-the-Art Review.” JACC. CardioOncology 2, no. 2 (June 2020): 293–311. https://doi.org/10.1016/j.jaccao.2020.05.009.

• Basson, Craig T., and H. Thomas Aretz. “Case 11-2002: A 27-Year-Old Woman with Two Intracardiac Masses and a History of Endocrinopathy.” Edited by Richard C. Cabot, Nancy Lee Harris, William F. McNeely, Jo-Anne O. Shepard, Sally H. Ebeling, Stacey M. Ellender, and Christine C. Peters. New England Journal of Medicine 346, no. 15 (April 11, 2002): 1152–58. https://doi.org/10.1056/NEJMcpc010057.

• Colin, Geoffrey C., Bernhard L. Gerber, Mihaela Amzulescu, and Jan Bogaert. “Cardiac Myxoma: A Contemporary Multimodality Imaging Review.” The International Journal of Cardiovascular Imaging 34, no. 11 (November 2018): 1789–1808. https://doi.org/10.1007/s10554-018-1396-z.