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Chapter 26
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Cardiac Neoplasms
FernandoRamirezDel Val andMichaelJ.Reardon
Incidence
Neoplasms of the heart can be primary (benign or malignant) or secondary (metastatic). Secondary neoplasms are many times more common than primary tumors.
Primary cardiac tumors have an incidence of 1% and a prevalence of 0.001–0.03in
autopsy series [1, 2]. As many as 20% of patients with terminal metastatic disease
have cardiac involvement. In these patients, surgery is mainly palliative and often
limited to drainage of pericardial effusions [3]. Common sites of metastasis to the
heart are shown in Table26.1. Primary heart tumors can require cardiac surgery of
varying complexity to achieve complete resections. In adults, the most common
cardiac neoplasms are atrial myxoma and papillary broelastoma [4, 5]. Up to 25%
of all primary cardiac neoplasms are malignant and 75% of these are sarcomas [6,
7]. Table26.2 shows a list of primary cardiac tumors as well as the structures from
which they most often arise.
F. R. Del Val (*)
Harvard Medical School, Massachusetts General Hospital, Boston, MA, USA
e-mail: framirezdelval@mgh.harvard.edu
M. J. Reardon
Cardiothoracic Surgery, Houston Methodist Hospital, Houston, TX, USA
e-mail: mreardon@houstonmethodist.org
Switzerland AG 2024
J. P. Bloom, T. M. Sundt (eds.), Cardiac Surgery Clerkship, Contemporary
Surgical Clerkships, https://doi.org/10.1007/978-3-031-41301-8_26
299© The Author(s), under exclusive license to Springer Nature
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F. R. Del Val and M. J. Reardon
Table 26.1 Common sites of
primary tumors in metastatic
heart disease
Table 26.2 Types of primary cardiac tumors
Benign
Valves
Atria
Ventricle
Pericardium
Malignant
Atria
Ventricles
Pericardium
Vasculature
Breast carcinoma
Esophageal carcinoma
Gastric carcinoma
Germ cell tumors (malignant)
Hepatocellular carcinoma
Leukemia/lymphoma
Lung carcinoma
Melanoma
Mesothelioma
Renal cell carcinoma
Sarcoma
Papillary broelastoma
Myxoma
Lipomatous hypertrophy
Paraganglioma
Schwannoma
Rhabdomyoma
Fibroma
Hemangioma
Granular cell tumor
Epithelioid hemangioendothelioma
Hamartoma of mature cardiac myocytes
Inammatory myobroblastic tumor
Lipoma
Germ cell tumor
Undifferentiated pleomorphic sarcoma
Angiosarcoma
Osteosarcoma
Myxobrosarcoma
Rhabdomyosarcoma
Liposarcoma
Mesothelioma
Lymphoma
Synovial sarcoma
Solitary brous tumor
Leiomyosarcoma
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301
Workup
An important differential diagnosis of a cardiac mass is tumor versus thrombus.
Cardiac MRI looking for perfusion of the mass can help differentiate tumor from
thrombus [8]. Thrombus is more likely to be associated with indwelling catheters,
pacemakers, mechanical devices, and hypercoagulable syndromes. The predominant symptoms in patients with cardiac neoplasms result from embolic events,
mechanical disruption of normal blood ow, alterations in the normal conduction,
or systemic constitutional symptoms. Symptoms, therefore, depend on tumor location, size mobility, and friability.
Both atrial and ventricular tumors can impede normal blood ow from the atrium to
the ventricle or the ventricular outow tract. Atrial tumors may mimic valvular stenosis
and are more likely to produce intermittent obstruction depending on the tumor’s position. Arrhythmias are seen due to direct tumor inltration or myocardial irritation.
Hemopericardium is more often observed in malignant neoplasms. Sudden cardiac
death secondary to obstruction of blood ow or ventricular arrhythmias may occur,
thus most neoplasms (benign and malignant) benet from resection when possible.
On physical examination, signs and symptoms of left (chest pain, orthopnea,
dyspnea) or right heart failure (peripheral edema, ascites, hepatomegaly) are often
observed. Left heart failure is a more common presentation both because left heart
tumors are more common and because large right heart tumors are generally malignant and tend to grow exophytically rather than into the right atrium. A characteristic neoplasm plop (early diastolic low pitched sound after S2) has been described in
association with cardiac tumors. It is thought to be caused as the neoplasm strikes
the myocardium or as the mass prolapses from the atrium to the ventricle [9].
Transthoracic echocardiography is often the initial imaging study in the assessment of cardiac tumors as it is readily available, inexpensive, and provides good
spatial and temporal resolution [10]. Transesophageal echocardiography provides a
better denition of cardiac structures and is less limited by poor acoustic windows,
thus playing an integral part during preoperative and intraoperative planning. The
role of cardiac computer tomography (CCT) and cardiac magnetic resonance (CMR)
imaging in the assessment of cardiac tumors has grown over the last two decades [11].
Both CCT and CMR provide a multiplanar reconstruction of the heart and adjacent structures but CCT provides the best special resolution but limited tissue characterization and no functional assessment of the heart. In contrast, CMR has the
highest tissue characterization and provides a comprehensive functional assessment
of the cardiovascular structures [12].
Differential Diagnosis
Myxoma
Myxomas are the most common primary cardiac neoplasm in adults [13]. These
gelatinous benign neoplasms originate in the sub-endocardium and are formed by
lepidic cells (polygonal/stellate myxoma cells with abundant eosinophilic
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cytoplasm) [14]. They are most commonly solitary and are located in the left atrium
(within the fossa ovalis) in >80% of the time, but can also be present in the right
atrium or rarely in the ventricles [15]. Myxomas are more common in women (2:1)
and present between the third and sixth decade of life. Most are sporadic and single,
but familial syndromes such as Carney syndrome are well described and are associated with multiple or recurrent myxomas [16].
The classic presentation of systemic symptoms such as weight loss and fatigue,
valvular obstruction, and embolization occurs in 30% of the patients. Smaller, irregular, more friable tumors have a higher risk of embolization [17]. The most common
sequela of these embolic events are neurologic, most of which result in permanent
decits, although they can also result in visceral or lower extremity ischemia.
Myxomas can cause outow obstruction and will mimic right- or left-sided valvular disease depending on their location. Left atrial myxomas may present with
elevated left atrial and pulmonary pressures raising concerns for mitral stenosis
which leads to imaging workup and diagnosis of the tumor. Myxomas that completely occlude the mitral valve result in syncopal episodes (if transient) or sudden
cardiac death. Tumors located in the right atrium mimic tricuspid stenosis and can
produce paradoxical embolization if a patent foramen ovale is present. Similarly,
left and right ventricular outow tract obstructions can be seen when these neoplasms arise from the ventricles.
Surgical resection with a negative margin is recommended in all myxomas due
to the risk of embolization. Recurrence is rare in sporadic myxomas (5%) and is
associated with positive margins. Resection of these tumors is achieved via median
sternotomy with aortic and bi-caval cannulation or using minimally invasive or
robotic approaches. Exposure of the tumor varies depending on the cavity it is
located. Left atrial lesions are approached by incising the anterior wall of the left
atrium via Sondergaard’s groove. Biatrial exposure through an additional parallel
right atrial incision is generally reserved for large tumors. Venous cannulation can
be challenging in right atrial tumors. If the size or location of the myxoma precludes
central venous cannulation, peripheral femoral and jugular venous cannulas can be
placed to initiate cardiopulmonary bypass.
F. R. Del Val and M. J. Reardon
Papillary Fibroelastoma
Papillary broelastomas (PFE) account for 10% of all primary cardiac tumors [14]
and are the most common valve tumor but can present anywhere in the endocardium. Histologically, they are formed by papillary folds and reassemble valvular
chordae tendinea [18]. PFE are more common in patients in their seventh decade of
life [19]. They most commonly occur in the aortic valve, but can also occur in rightsided vales, are generally small (<1cm), and present as a single lesion in 80% of the
patients [20]. They are most commonly asymptomatic and incidentally found during cardiac workup for other disease processes occasionally present with embolic
events. Surgery is indicated in all left-sided lesions given the risk of systemic
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embolization. The timing of resection is driven by the size of the tumor (>1cm),
embolic events, or mobile masses. Valve sparing resections are generally possible
by employing conservative resection margins with excellent outcomes [14].
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Sarcoma
Primary cardiac malignancies are exceedingly uncommon, angiosarcomas and
rhabdomyosarcomas account for more than half of all primary cardiac sarcomas.
These tumors are most common in women during their fth decade of life. Most
sarcomas arise from the atrium and pulmonary vessels, followed by the ventricles,
mitral valve, and epicardium [21] Surgical resection has been associated with
improved survival for primary cardiac sarcoma [6].
Angiosarcomas, are often found in the right atrium, are more common in men,
and often present between 20 and 50years of age [22]. A majority of patients present with metastatic disease (to the lung, liver, and brain) with poor 12-month survival. Rhabdomyosarcomas are equally distributed among the right and left
chambers of the heart.
The overall prognosis for cardiac sarcomas is poor because both metastatic disease and mechanical circulatory collapse from local spread are common [17]. Oneand ve-year survival for these tumors are 47% and 16%, respectively [23]. The
most common cause of death without surgery is local disease progression. The most
common cause after surgical resection is distant metastatic disease [7, 24].
Surgical Resection ofPrimary Malignancies
Treatment is individualized as complete surgical resection is not always possible.
Multimodality management includes surgical resection, chemotherapy, palliative
radiation for non-resectable tumors, and heart transplantation in selected cases [17].
Right-sided sarcomas tend to be bulky, metastasize early, and grow exophytically
(resulting in a lower incidence of heart failure or mechanical circulatory collapse).
These characteristics provide a valuable time window for neoadjuvant chemotherapy [25, 26]. This strategy aims to decrease the size of the tumor, thereby facilitating R0 resection in as many as one-third of the patients [26]. Ride-sided lesions are
easily accessible via a median sternotomy and are amendable to standard resection
techniques.
Left-sided sarcomas are less likely to metastasize early, are less inltrative, and
are more likely to present with heart failure or circulatory collapse compared to
their right-sided counterparts. A common presentation is that of a patient with an
incomplete rst resection where the tumor was misdiagnosed as a myxoma, followed by early recurrence. These tumors are often managed with primary resection
followed by adjuvant chemotherapy as heart failure symptoms at presentation often
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F. R. Del Val and M. J. Reardon
preclude the use of neoadjuvant treatment [26]. Best outcomes are seen with R0
resection but this is particularly challenging in posterior left atrial wall tumors,
where exposure limits adequate excision. Autotransplantation (excision of the native
heart from its anatomical position to remove the tumor exvivo) has been employed
to improve exposure and facilitate complete resection [27]. The procedure is done
via midline sternotomy with bi-caval and aortic cannulation. The temperature goal
is set for 28°C.The inferior and superior vena cava, great vessels, and left atria are
divided. The heart is placed on a back table with iced saline to remove the tumor and
reconstruct the atrium when necessary followed by re-implantation of the heart [28,
29]. Pulmonary artery sarcoma has been treated with both total excision and endar-
terectomy. Total excision when possible provides a better oncologic resection [7].
These tumors usually arise from the level of the pulmonary valve and extend distally along the arteries. Pulmonary root replacement is often necessary for which we
use pulmonary root allograft.
Role ofCardiac Surgery intheManagement
ofMetastatic Disease
The incidence of cardiac metastasis is 9.1%. Mesothelioma, melanoma, lung cancer, and breast cancer are the most common primary tumor sites [30, 31]. The pericardium, followed by the epicardium are the most frequently involved sites of
metastasis. Endocardial metastases are most common on the right side and are associated with tumors with endovascular growth such as liver and renal cancers [31].
Only 14% of all surgically resected cardiac tumors are metastatic [32]. These are
usually located on the right side. They result from direct hematogenous extension
from the cava or as hematogenous spread from distant tumors. Resection is palliative for the latter. Direct cavo-atrial spread in renal cell carcinoma is rare (<10%)
and well documented [33, 34]. These patients have a survival benet from complete
oncologic resection as survival is not dependent on the presence of direct hematogenous spread [35].
Lung cancer can metastasize to the heart by local invasion, lymphatic, and hematogenous spread [36]. Resection of the great vessels and the atrium in patients with
pathological N0–1 and T4 lung cancer to obtain R0 resection has been described
with a median survival of 14months [37, 38].
References
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12,485 consecutive autopsies. Arch Pathol Lab Med. 1993;117(10):1027–31.
2. Sütsch G, etal. Heart tumors: incidence, distribution, diagnosis. Exemplied by 20,305 echocardiographies. Schweiz Med Wochenschr. 1991;121(17):621–9.
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3. Smith C.Tumors of the heart. Arch Pathol Lab Med. 1986;110(5):371–4.
4. Silverman NA.Primary cardiac tumors. Ann Surg. 1980;191(2):127–38.
5. Reynen K.Cardiac myxomas. N Engl J Med. 1995;333(24):1610–7.
6. Yin K, etal. Survival outcomes in patients with primary cardiac sarcoma in the United States.
J Thorac Cardiovasc Surg. 2021;162(1):107–115.e2.
7. Chan EY, etal. Surgical Management of Primary Pulmonary Artery Sarcoma. Semin Thorac
Cardiovasc Surg. 2021;35(1):53–64.
8. Wyler von Ballmoos MC, Chan EY, Reardon MJ.Imaging and surgical treatment of primary
pulmonary artery sarcoma. Int J Cardiovasc Imaging. 2019;35(8):1429–33.
9. Keren A, etal. The etiology of tumor plop in a patient with huge right atrial myxoma. Chest.
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10. Auger D, et al. Cardiac masses: an integrative approach using echocardiography and other
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multicentre outcomes study. Eur Heart J. 2021;43(1):71–80.
12. Hoey ET, et al. MRI and CT appearances of cardiac tumours in adults. Clin Radiol.
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13. Burke, A. and R. Virmani, Atlas of tumor pathology: tumors of the heart and great vessels. 1996.
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surgical patients. Tex Heart Inst J. 2016;43(2):148–51.
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Heart J. 2003;146(3):404–10.
20. Reynen K.Frequency of primary tumors of the heart. Am J Cardiol. 1996;77(1):107.
21. Zhang PJ, et al. Primary cardiac sarcomas: a clinicopathologic analysis of a series with
follow-up information in 17 patients and emphasis on long-term survival. Hum Pathol.
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22. McAllister HA Jr, Hall RJ, Cooley DA. Tumors of the heart and pericardium. Curr Probl
Cardiol. 1999;24(2):57–116.
23. Oliveira GH, etal. Characteristics and survival of malignant cardiac tumors: a 40-year analysis
of >500 patients. Circulation. 2015;132(25):2395–402.
24. Chan EY, etal. Primary cardiac sarcomas: treatment strategies. J Thorac Cardiovasc Surg.
2022;166(3):828–838.e2.
25. Abu Saleh WK, etal. Improved outcomes with the evolution of a neoadjuvant chemotherapy
approach to right heart sarcoma. Ann Thorac Surg. 2017;104(1):90–6.
26. Blackmon SH, Reardon MJ.Surgical treatment of primary cardiac sarcomas. Tex Heart Inst
J. 2009;36(5):451–2.
27. Blackmon SH, etal. Cardiac autotransplantation for malignant or complex primary left-heart
tumors. Tex Heart Inst J. 2008;35(3):296–300.
28. Conklin LD, Reardon MJ.Autotransplantation of the heart for primary cardiac malignancy:
development and surgical technique. Tex Heart Inst J. 2002;29(2):105–8; discussion 108.
29. Ramlawi B, et al. Autotransplantation for the resection of complex left heart tumors. Ann
Thorac Surg. 2014;98(3):863–8.
30. Paraskevaidis IA, etal. Cardiac tumors. ISRN Oncologia. 2011;2011:208929.
31. Bussani R, etal. Cardiac metastases. J Clin Pathol. 2007;60(1):27–34.
32. Murphy MC, etal. Surgical treatment of cardiac tumors: a 25-year experience. Ann Thorac
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33. Zustovich F, etal. Cardiac metastasis from renal cell carcinoma without inferior vena involvement: a review of the literature based on a case report. Two different patterns of spread? Int J
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34. Kearney GP, et al. Results of inferior vena cava resection for renal cell carcinoma. J Urol.
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35. Sidana A, etal. Determinants of outcomes after resection of renal cell carcinoma with venous
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36. Tamura A, etal. Cardiac metastasis of lung cancer. A study of metastatic pathways and clinical
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37. Fukuse T, Wada H, Hitomi S.Extended operation for non-small cell lung cancer invading great
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38. Tsuchiya R, etal. Extended resection of the left atrium, great vessels, or both for lung cancer.
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F. R. Del Val and M. J. Reardon
Chapter 27
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Hypertrophic Cardiomyopathy
BoatengKubi andThoralfM.Sundt
Clinical Presentation
• HCM may or may not be symptomatic. When symptoms occur, they may be due
to left ventricular outow tract (LVOT) obstruction secondary to systolic anterior
motion (SAM) of the mitral valve. They may also be due to inow impairment
secondary to chamber hypertrophy and decreased compliance of the left ventricle (diastolic dysfunction).
• A patient with HCM is considered to have signicant LVOT obstruction when
the maximal instantaneous subaortic pressure gradient is ≥30mmHg either at
rest (basal obstruction) or with physiologic provocation (labile obstruction). The
conventionally accepted threshold for intervention (surgical or percutaneous) is
≥50mmHg (Fig.27.1).
• Dyspnea and chest pain are the most common presenting symptoms of
HCM.Other symptoms include syncope, heart palpitations, paroxysmal nocturnal dyspnea, pedal edema, and sudden cardiac death .
• Due to impaired compliance of the left ventricle in HCM, physical exam may
reveal a normal s1, split s2, and audible s3in the setting of decompensated heart
failure. Patients may also have a double apical impulse due to forceful left atrial
contraction against the low-compliance ventricle.
• HCM is typically diagnosed in the fth decade of life.
B. Kubi · T. M. Sundt (*)
Department of Surgery, Massachusetts General Hospital, Boston, MA, USA
e-mail: bkubi@mgb.org; tsundt@mgh.harvard.edu
Switzerland AG 2024
J. P. Bloom, T. M. Sundt (eds.), Cardiac Surgery Clerkship, Contemporary
Surgical Clerkships, https://doi.org/10.1007/978-3-031-41301-8_27
307© The Author(s), under exclusive license to Springer Nature
308
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Fig. 27.1 3-D printed model demonstrating focal hypertrophy at interventricular septum. Dashed
red line demonstrates the extent of myectomy that would be performed to correct LVOT obstruction
B. Kubi and T. M. Sundt
Diagnostic Evaluation ofHCM
• After a comprehensive history and physical exam, an EKG should be obtained.
In 75–95% of cases of HCM, a 12-lead EKG demonstrates changes consistent
with left ventricular hypertrophy [1]. However, a normal EKG does not
exclude HCM.
• Conventional 2-dimensional echocardiography is the most utilized imaging
modality in HCM due to its widespread availability.
• Cardiac magnetic resonance (CMR) is increasingly used to conrm the diagnosis
particularly when echocardiographic images are inconclusive. In addition to aiding preoperative planning, CMR can identify myocardial ischemia in the absence
of epicardial coronary arterial disease—an important feature of HCM [2].
• An LV wall thickness>15mm on imaging (in the absence of another etiology)
is generally considered diagnostic of HCM in adults, especially if associated
with a family history of HCM.
Management ofHCM
• Medical management with beta-blockers, verapamil, and/or disopyramide is the
recommended initial therapy for obstructive HCM (Fig.27.2) [3].
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