Key Points
- 1.
Cardiac tumors are rare. In general, a cardiac mass is more likely a vegetation or a thrombus than a tumor. Secondary (metastatic) tumors are far more common than primary cardiac tumors. Among primary cardiac tumors, benign lesions are more common than malignant tumors.
- 2.
Cardiac myxomas historically have been considered the most common benign cardiac tumor. Patients with myxomas typically exhibit signs and symptoms attributable to one of the triad of intracardiac obstruction, embolism, or constitutional symptoms.
- 3.
Papillary fibroelastomas are the most common valvular cardiac tumor and may be the most common benign lesion as well. Typically solitary, fibroelastomas occur most frequently on the mitral and aortic valve leaflets. Once considered an incidental, benign finding, they have a high incidence of coronary and cerebral embolization.
- 4.
Primary malignant cardiac tumors are less common than benign tumors. The overwhelming majority of primary malignant tumors are sarcomas.
- 5.
Metastatic cardiac tumors are far more common than primary tumors. Pericardial involvement is the most common.
- 6.
Carcinoid tumors are metastasizing neuroendocrine tumors. In patients with carcinoid syndrome, carcinoid heart disease is common and characterized by tricuspid regurgitation, mixed pulmonic regurgitation and stenosis, and right-sided heart failure. The mainstays of treatment are symptom management with somatostatin analogs, antitumor therapy, and cardiac surgical intervention.
- 7.
Cardiomyopathies are a heterogeneous group of diseases that may be acquired or genetic and may be confined to the heart (primary) or may be part of a systemic disorder (secondary). The American Heart Association subclassifies primary processes as genetic, acquired, or mixed.
- 8.
Dilated cardiomyopathy is the most common of the cardiomyopathies and may be acquired, hereditary, or idiopathic.
- 9.
Hypertrophic cardiomyopathy is likely the most common inherited cardiac disease and may progress along one or more of three pathways: (1) sudden cardiac death, (2) heart failure, or (3) atrial fibrillation, with or without cardioembolic stroke.
- 10.
The restrictive cardiomyopathies are heterogeneous and characterized by impaired myocardial relaxation and decreased ventricular compliance. Considering that their treatments are significantly different, restrictive cardiomyopathy and constrictive pericarditis must be distinguished.
- 11.
The management of an incidental patent foramen ovale found during cardiac surgery via transesophageal echocardiography continues to evolve; however, few data suggest that closure offers morbidity or mortality benefit and may actually increase the risk of postoperative stroke.
Although some of the diseases discussed are quite rare and unlikely to be encountered regularly outside of large referral centers, other conditions, such as chronic kidney disease, are exceedingly common and likely to be routinely found in the patient population. Regardless of the prevalence of the disease or condition, however, optimal anesthetic management will depend both on a thorough understanding of the underlying pathologic and pathophysiologic findings, and on the recognition that the disease process may affect the anesthetic just as much as the anesthetic may exacerbate the disease process.
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Cardiac Tumors
Cardiac tumors belong to the class of cardiac masses that includes vegetations and thrombi, for which tumors may be mistaken. Cardiac tumors may be classified as primary or secondary (metastatic). Primary tumors may be benign or malignant, whereas secondary tumors may involve the heart by direct extension (breast and lung), by venous extension (renal cell and hepatocellular carcinoma), or by hematogenous (melanoma, breast, and carcinoid) or lymphatic (lymphoma) spread.
In general, cardiac tumors are rare, and a cardiac mass encountered echocardiographically or radiographically is more likely to be a thrombus or a vegetation than a tumor. Metastatic tumors are more common than primary cardiac tumors, with an incidence at autopsy between 2.3% and 18.3%, whereas primary tumors have an incidence rate between 0.0014% and 0.33%. Among primary tumors, benign lesions are more common than malignant masses. In adults, the most common primary benign tumors are myxomas, although several series now suggest that papillary fibroelastomas may, in fact, be more common ( Table 18.1 ). In children, rhabdomyomas are the most common benign tumor. Approximately 15% to 25% of primary cardiac tumors are malignant, with sarcomas being the most common in both adults and children. Tumors with high rates of cardiac metastases include pleural mesothelioma, melanoma, lung adenocarcinoma and squamous cell carcinoma, and breast carcinoma. Although metastases may involve the pericardium, epicardium, myocardium, or endocardium, pericardial involvement is most common.
| Neoplasms | Incidence (%) | |
|---|---|---|
| Adults | Children | |
| Myxoma | 45 | 15 |
| Lipoma | 20 | — |
| Papillary fibroelastoma | 15 | — |
| Angioma | 5 | 5 |
| Fibroma | 3 | 15 |
| Hemangioma | 5 | 5 |
| Rhabdomyoma | 1 | 45 |
| Teratoma | <1 | 15 |
Although cardiac tumors may be clinically silent and diagnosed only at autopsy, advancements in imaging have facilitated both their often-incidental antemortem diagnosis and their characterization once detected. The increasing sophistication of two-dimensional echocardiography, the advent of three-dimensional echocardiographic imaging, and the continued refinement of computed tomography (CT) and magnetic resonance imaging (MRI) have all allowed earlier, more frequent, and more complete assessment of cardiac tumors. Although malignant primary lesions and metastatic tumors may produce constitutional symptoms, even histologically benign masses may cause concerning signs and symptoms associated with intracardiac obstruction and extracardiac embolization.
The most effective treatment of primary tumors is generally surgical resection with an approximate 2% operative mortality. Recurrence rate in these tumors varies between 3% and 13% but appears to be related to a biologic propensity rather than the surgical technique, as was previously believed. Orthotopic cardiac transplantation has been recommended for unresectable tumors, but the benefit is indeterminate. Although malignant lesions are less common, the surgical risk and outcome for their resection, compared with benign tumor resection, is usually significantly worse, especially in younger patients.
Primary Benign Tumors
Myxoma
Often a diagnostic challenge, myxomas are benign, solitary, and slow-proliferating neoplasms. Microscopically, they often resemble organized thrombi, which may obscure their identity as a primary cardiac tumor. The pedunculated mass is believed to arise from undifferentiated cells in the fossa ovalis and adjoining endocardium, projecting into the left atrium (LA) and the right atrium (RA) 75% and 20% of the time, respectively. However, myxomas appear in other locations of the heart, even occupying more than one chamber. Myxomas predominate in the 30- to 60-year-old age range, but any age group may be affected. More than 75% of the affected patients are women. Although most cases occur sporadically, 7% to 10% of atrial myxomas will occur in a familial pattern with an autosomal dominant transmission pattern known as Carney complex.
Occasionally an incidental finding on echocardiography, myxomas may produce a variety of symptoms. The classic triad includes embolism, intracardiac obstruction, and constitutional symptoms. Approximately 80% of individuals will exhibit one component of the triad. The most common initial symptom, dyspnea on exertion, reflects mitral valve obstruction associated with left atrial myxomas ( Fig. 18.1 ). Because of the pedunculated nature of some myxomas, temporary obstruction of blood flow may cause hemolysis, hypotension, syncope, or sudden death. Other symptoms of mitral obstruction, similar to mitral stenosis, may occur, including hemoptysis, systemic embolization, fever, and weight loss. If the tumor is obstructing the mitral valve, then a tumor plop may be heard after the second heart sound on chest auscultation. The persistent sinus rhythm in the presence of such symptoms may help distinguish an atrial myxoma from mitral stenosis. Severe pulmonary hypertension without significant mitral valve involvement suggests recurrent pulmonary emboli, which is known to occur with a myxoma in the RA or right ventricle (RV). Occasionally, right-sided heart tumors may appear as cyanotic congenital heart lesions attributable to intracardiac shunting.
