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Surgery_Schwartz. by the history and physical. Most inflammatory markers and labo-ratory tests are nonspecific, but C-reactive protein may be useful in predicting recurrence risks and in guiding the duration of anti-inflammatory medications.272 Rarely, other imaging modalities, such as CT scanning, pericardial biopsies, or pericardiocentesis may aid in diagnosis.Treatment. The preferred treatment depends on the underly-ing cause of the pericarditis. The disease usually follows a self-limited and benign course and can be successfully treated with a short course of nonsteroidal anti-inflammatory agents (NSAIDs). The addition of colchicine may be beneficial.273 Some patients may require judicious use of steroids or IV anti-biotics. In cases of purulent pyogenic pericarditis, surgical exploration and drainage are occasionally necessary. Rarely, accumulation of fluid in the pericardium may lead to tampon-ade, requiring prompt evacuation of the pericardial space. While pericardiocentesis will typically


Surgery_Schwartz

Surgery_Schwartz. are occasionally necessary. Rarely, accumulation of fluid in the pericardium may lead to tampon-ade, requiring prompt evacuation of the pericardial space. While pericardiocentesis will typically suffice, surgical drain-age may be required for thick, viscous, or clotted fluid or in patients with significant scarring from previous operations. More commonly, surgical intervention is required to manage recurrent disease.8Table 21-14Features of acute pericarditis• Pleuritic and positional, retrosternal chest pain• Pericardial friction rub• EKG changes: diffuse ST elevation and PR depression• Pericardial effusionEKG = Electrocardiogram.Relapsing PericarditisAs many as one-third of patients with acute pericarditis will develop at least one episode of relapse.267 While many of these patients can be treated medically during their initial relapse and do not experience further episodes, a subset of patients experi-ence chronic relapsing pericarditis that can significantly impact their quality of


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Surgery_Schwartz. treated medically during their initial relapse and do not experience further episodes, a subset of patients experi-ence chronic relapsing pericarditis that can significantly impact their quality of life. Recurrence may develop either from the original etiology or from an autoimmune process that occurs as a consequence of damage from the initial episode. Relapsing pericarditis normally responds to a longer course of NSAIDS ± colchicine. While steroids may induce rapid symptomatic response, their use should be limited to patients who have mul-tiple relapses and are unresponsive to first-line agents, as several studies have suggested that steroid administration may favor relapse.273,274Pericardiectomy may be considered a last resort treatment in patients with relapsing pericarditis who are severely symp-tomatic despite optimal medical management, are unable to tol-erate steroids, or have recurrence with tamponade. Evidence for this approach is lacking, as few studies have described


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Surgery_Schwartz. are severely symp-tomatic despite optimal medical management, are unable to tol-erate steroids, or have recurrence with tamponade. Evidence for this approach is lacking, as few studies have described pericar-diectomy in this population.275-277 The largest study and the only one to compare surgical treatment with medical management for patients with persistent relapsing pericarditis was a report of 184 patients from the Mayo Clinic.276 About 58 patients were identi-fied as having undergone a pericardiectomy after failed medi-cal treatment, whereas the remainder were treated with medical management only. Compared to medical treatment only, peri-cardiectomy resulted in significantly fewer relapses (8.6% vs. 28.6%, P = 0.009) at long term follow-up, as well as a nonsig-nificant trend towards less medication and corticosteroid usage. Of note, 80% of patients in the pericardiectomy group who had relapses reported significant improvements in their symptoms and had fewer relapses than before


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Surgery_Schwartz. less medication and corticosteroid usage. Of note, 80% of patients in the pericardiectomy group who had relapses reported significant improvements in their symptoms and had fewer relapses than before pericardiectomy. No periop-erative deaths were observed, and only two patients (3%) had major complications. Hence, at experienced centers pericardi-ectomy may be a safe and viable option in select patients with relapsing pericarditis.Chronic Constrictive PericarditisEtiology, Pathology, and Pathophysiology. Constrictive pericarditis can occur after any pericardial disease process but remains a rare outcome of recurrent pericarditis. It results when chronic pericardial scarring and fibrosis cause adhesion of the visceral and parietal layers and resultant obliteration of the pericardial space. While the pericardium is often grossly thickened with either focal or diffuse calcification in chronic disease, constriction may occur with normal pericardial thick-ness in approximately 20% of


Surgery_Schwartz

Surgery_Schwartz. While the pericardium is often grossly thickened with either focal or diffuse calcification in chronic disease, constriction may occur with normal pericardial thick-ness in approximately 20% of cases.267,278 In developed nations, idiopathic causes and cardiac surgery (accounting for almost 40% of cases in some series) are the predominant underlying eti-ologies, followed by mediastinal radiation, pyogenic infections (i.e., Staphylococcus), and other miscellaneous causes. Tuber-culosis is an additional common cause in immunosuppressed patients and in developing or underdeveloped countries.Clinically, pericardial constriction limits diastolic filling of the ventricles and mimics right heart failure since the right-sided chambers are more affected by a rise in filling pressures. Subsequent increases in central venous pressure result in the progressive development of hepatomegaly, ascites, peripheral edema, abdominal pain, dyspnea on exertion, anorexia, and nausea (in part due to hepatic


Surgery_Schwartz

Surgery_Schwartz. in central venous pressure result in the progressive development of hepatomegaly, ascites, peripheral edema, abdominal pain, dyspnea on exertion, anorexia, and nausea (in part due to hepatic and bowel congestion). In many patients, these symptoms develop insidiously over a course Brunicardi_Ch21_p0801-p0852.indd 84001/03/19 5:32 PM 841ACQUIRED HEART DISEASECHAPTER 21of years. Since many of these symptoms are similar to those seen in patients with restrictive cardiomyopathy, the distinc-tion between the two entities is difficult, but it remains critical because the treatment is completely different for restriction. The primary difference is that restrictive cardiomyopathy is defined by a nondilated ventricle with a rigid myocardium that causes a significant decrease in myocardial compliance, which is not seen in constrictive pericarditis.Clinical and Diagnostic Findings. Classic physical exam findings include jugular venous distention with Kussmaul’s sign, diminished cardiac apical


Surgery_Schwartz

Surgery_Schwartz. which is not seen in constrictive pericarditis.Clinical and Diagnostic Findings. Classic physical exam findings include jugular venous distention with Kussmaul’s sign, diminished cardiac apical impulses, peripheral edema, ascites, pulsatile liver, a pericardial knock, and, in advanced disease, signs of liver dysfunction, such as jaundice or cachexia. The “pericardial knock” is an early diastolic sound that reflects a sudden impediment to ventricular filling, similar to an S3 but of higher pitch.Several findings are characteristic on noninvasive and invasive testing. CVP is often elevated 15 to 20 mmHg or higher. ECG commonly demonstrates nonspecific low voltage QRS complexes and isolated repolarization abnormalities. Chest X-ray may demonstrate calcification of the pericardium, which is highly suggestive of constrictive pericarditis in patients with heart failure, but this is present in only 25% of cases.274 Cardiac CT or MRI (cMRI) typically demonstrate increased pericardial


Surgery_Schwartz

Surgery_Schwartz. is highly suggestive of constrictive pericarditis in patients with heart failure, but this is present in only 25% of cases.274 Cardiac CT or MRI (cMRI) typically demonstrate increased pericardial thickness (>4 mm) and calcification, dilation of the inferior vena cava, deformed ventricular contours, and flattening or leftward shift of the ventricular septum. Pericardial adhesions may also be seen on tagged cine MRI studies.As discussed, it is most important to distinguish peri-cardial constriction from restrictive cardiomyopathy, which is best done with either echocardiography or right heart catheter-ization. Findings favoring constriction on echocardiography include respiratory variation of ventricular septal motion and mitral inflow velocity, preserved or increased mitral annulus early diastolic filling velocity, and increased hepatic vein flow reversal with expiration.267,274 Cardiac catheterization will show increased atrial pressures, equalization of end-diastolic pressure and


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Surgery_Schwartz. diastolic filling velocity, and increased hepatic vein flow reversal with expiration.267,274 Cardiac catheterization will show increased atrial pressures, equalization of end-diastolic pressure and early ventricular diastolic filling with a subsequent plateau, called the “square-root sign.” Additional findings upon cath-eterization that would favor constriction include respiratory variation in ventricular filling and increased ventricular inter-dependence, manifest as a discordant change in the total area of the LV and RV systolic pressure curve with respiration.Surgical Treatment. Transient constrictive pericarditis may occur weeks to months after an initial injury and follows a self-limiting course of weeks to a few months. These patients are best treated with medical therapy alone. They often lack calci-fication of their pericardium, and the degree of late gadolinium enhancement of the pericardium on cardiac MRI has shown promise in predicting which patients may have resolution of


Surgery_Schwartz

Surgery_Schwartz. often lack calci-fication of their pericardium, and the degree of late gadolinium enhancement of the pericardium on cardiac MRI has shown promise in predicting which patients may have resolution of the process.279 Still, there is no ideal way to distinguish these patients from those who will develop chronic constrictive peri-carditis, which is permanent. Therefore, if a newly diagnosed patient is hemodynamically stable, it is recommended that con-servative management is attempted for 2 to 3 months prior to performing a pericardiectomy.278 Surgical therapy should not be delayed indefinitely, however, as results are improved when the operation is performed earlier in the course of the disease. A series of 938 patients undergoing pericardiectomy reported by the Mayo Clinic, 355 of whom underwent pericardiec-tomy for constrictive pericarditis, showed significantly lower survival in patients with constrictive pericarditis compared with patients with effusive/relapsing pericarditis.280


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Surgery_Schwartz. pericardiec-tomy for constrictive pericarditis, showed significantly lower survival in patients with constrictive pericarditis compared with patients with effusive/relapsing pericarditis.280 Patients with left ventricular systolic dysfunction or right ventricular dilata-tion are at increased risk of early mortality.281 Additional fac-tors that predict adverse long-term outcomes include older age and prior ionizing radiation, as well as cardiopulmonary and renal dysfunction.274,281 Surgery should therefore be approached cautiously in patients with advanced, “end-stage” constrictive pericarditis, mixed constrictive-restrictive disease (often from radiation), and significant myocardial or renal dysfunction, as those patients are at increased risk from surgery and may not experience improvement of symptoms.In order to minimize recurrence following pericardiec-tomy, complete pericardial resection is desirable. This is typi-cally performed through either a median sternotomy or left


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Surgery_Schwartz. of symptoms.In order to minimize recurrence following pericardiec-tomy, complete pericardial resection is desirable. This is typi-cally performed through either a median sternotomy or left anterolateral thoracotomy while on cardiopulmonary bypass. Radical pericardiectomy involves wide resection of the con-stricting pericardium from the anterior surface of the heart between the phrenic nerves and the diaphragmatic surface. Decortication of the right atrium and vena cavae is not univer-sally performed, but doing so improves the risk of persistent disease or relapse.282,283The extent of myocardial involvement may also affect long-term outcomes, and, thus, the depth of decortication is an important consideration.282 Even when an adequate pericardi-ectomy is performed, epicardial sclerosis can cause persistent hemodynamic instability or a delayed response to surgery. Scle-rotic epicardium is often thin and nearly transparent, but in cases of severe chronic constrictive pericarditis it can


Surgery_Schwartz

Surgery_Schwartz. cause persistent hemodynamic instability or a delayed response to surgery. Scle-rotic epicardium is often thin and nearly transparent, but in cases of severe chronic constrictive pericarditis it can be difficult to remove it without injury to the heart.Surgical Results. While most patients experience significant improvement in their symptoms following pericardiectomy, symptomatic relief may take several months. Since there is a significant perioperative morbidity and mortality, pericardiec-tomy is best performed by experienced surgeons at high-volume centers. Between 1970 and 1985, the operative mortality was reported to be 12%, but a lower mortality of approximately 4% to 8% was noted between 1977 and 2006 at several experienced centers.278,283-287Long-term survival is in part determined by etiology of the disease. In a report from the Cleveland Clinic, 7-year survival rates following pericardiectomy for idiopathic, postsurgical, and radiation-induced constrictive pericarditis were


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Surgery_Schwartz. by etiology of the disease. In a report from the Cleveland Clinic, 7-year survival rates following pericardiectomy for idiopathic, postsurgical, and radiation-induced constrictive pericarditis were 88%, 66%, and 27%, respectively.284 Results are worst for radiation-induced disease because ionizing radiation is often associated with myo-cardial injury as well as pericardial disease.Despite the risks, many patients experience significant benefits from surgical treatment. In one large series, 83% of patients were reported to be free of symptoms at last follow-up.287 This is in agreement with other studies that have shown a signif-icant improvement in NYHA functional status from class III/IV preoperatively to class I/II following pericardiectomy in >95% of patients.283,285-287CARDIAC NEOPLASMSOverview and General Clinical FeaturesCardiac neoplasms are rare, with an incidence ranging from 0.001% to 0.3% in autopsy studies and a 0.15% incidence in major echocardiographic series.288,289 In


Surgery_Schwartz

Surgery_Schwartz. and General Clinical FeaturesCardiac neoplasms are rare, with an incidence ranging from 0.001% to 0.3% in autopsy studies and a 0.15% incidence in major echocardiographic series.288,289 In one large autopsy series, 99.2% of cardiac tumors were metastatic in origin; however, Brunicardi_Ch21_p0801-p0852.indd 84101/03/19 5:32 PM 842SPECIFIC CONSIDERATIONSPART IIthese patients almost never present for surgical management as they usually have fatal diffuse metastatic disease.290 As a result, a majority of surgical series describe management of primary cardiac neoplasms. Benign cardiac tumors are most common and account for 75% of primary neoplasms. Approximately 50% of benign cardiac tumors are myxomas, with the remainder being papillary fibroelastomas, lipomas, rhabdomyomas, fibro-mas, hemangiomas, teratomas, lymphangiomas, and others, in order of decreasing frequency. Most malignant primary cardiac tumors are sarcomas (angiosarcoma, rhabdomyosarcoma, fibro-sarcoma, leiomyosarcoma,


Surgery_Schwartz

Surgery_Schwartz. hemangiomas, teratomas, lymphangiomas, and others, in order of decreasing frequency. Most malignant primary cardiac tumors are sarcomas (angiosarcoma, rhabdomyosarcoma, fibro-sarcoma, leiomyosarcoma, and liposarcoma), with a small inci-dence of malignant lymphomas.Clinical Presentation. The clinical presentation of cardiac neoplasms varies greatly depending on the location of the tumor, as well as its size, rate of growth, invasiveness, and fri-ability. While as many as 10% of patients are asymptomatic, most manifest some combination of symptoms from the classic triad resulting from blood flow obstruction, tumor embolization, and constitutional symptoms.291,292 Systemic manifestations of disease include fever, myalgias, chills, night sweats, weight loss, and fatigue and occur in up to one-third of patients.Obstruction of cardiac blood flow accounts for the major-ity of presenting symptoms.292 When the tumor is located in the left atrium, symptoms tend to mimic mitral valve disease


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Surgery_Schwartz. one-third of patients.Obstruction of cardiac blood flow accounts for the major-ity of presenting symptoms.292 When the tumor is located in the left atrium, symptoms tend to mimic mitral valve disease with dyspnea and pulmonary edema; although more severe presenta-tions with syncopal episodes, hypotension, and sudden cardiac death have been reported from temporary valve orifice occlu-sion. When the tumor is located in the right atrium, symptoms may mimic right heart failure and include hepatomegaly, asci-tes, and peripheral edema. Outflow tract obstruction is rare but may be caused by large ventricular tumors.293Tumor lysis and embolization may also lead to neurologic presentations such as stroke, retinal artery occlusion, or cere-bral aneurysms, particularly in the case of pedunculated tumors and those with frond-like projections.294 Embolic tumor cells are able to lodge and penetrate distant vessel walls via subintimal growth, which leads to weakening of the arterial wall and


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Surgery_Schwartz. tumors and those with frond-like projections.294 Embolic tumor cells are able to lodge and penetrate distant vessel walls via subintimal growth, which leads to weakening of the arterial wall and sub-sequent aneurysm formation. This has been documented as late as 5 years after successful primary myxoma resection.295 Alter-natively, embolic implants may metastasize and create space occupying lesions. While rare, myxomatous tumor emboli have also been identified in the coronary arteries, common iliac and femoral arteries, kidney, spleen, pancreas, and liver.294Certain clinical features may be helpful in distinguishing benign from malignant primary cardiac tumors.292 Malignant tumors, primarily sarcomas, do not demonstrate a gender pref-erence and tend to present after the fourth decade of life. They are often multifocal within the right atrium, and intramyocardial invasion can lead to refractory congestive heart failure, arrhyth-mias, hemopericardium, and ischemia. Conversely, benign


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Surgery_Schwartz. life. They are often multifocal within the right atrium, and intramyocardial invasion can lead to refractory congestive heart failure, arrhyth-mias, hemopericardium, and ischemia. Conversely, benign tumors, primarily myxomas, are typically unifocal in the left atrium, have a 3:1 female preference, and occur in younger patients. Arrhythmias and pericardial effusions are very rare in this population.Diagnosis and Characterization of Cardiac Masses. Trans-thoracic echocardiography is the mainstay imaging technique for the detection of cardiac tumors.292 However, echocardiogra-phy is limited by dependence on an acoustic window, subopti-mal visualization of extracardiac extension, and poor soft-tissue visualization. TEE is generally only beneficial for small local-ized tumors due to its limited field of view. cMRI is therefore the current standard for delineating the anatomical extent of the tumor and assessing the paracardiac space and great vessels. Advantages of cMRI over CT scans


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Surgery_Schwartz. field of view. cMRI is therefore the current standard for delineating the anatomical extent of the tumor and assessing the paracardiac space and great vessels. Advantages of cMRI over CT scans include better soft-tissue evaluation without the need for iodinated contrast and no expo-sure to ionizing radiation.It is important in the initial workup to distinguish a cardiac tumor from an intracardiac thrombus, which may be common in the atria of patients with AF and can mimic echocardiographic features of atrial myxomas. This determination is critical, as an atrial thrombus may be expected to resolve with anticoagula-tion, whereas a tumor requires surgical intervention. Moreover, anticoagulation can potentially increase the risk of peripheral embolization in patients with cardiac tumors. Delayed enhance-ment cMRI is the best modality to separate these two entities. cMRI may show vascularization, areas of necrosis, hemorrhage, or calcification in cardiac tumors that are not present in


Surgery_Schwartz

Surgery_Schwartz. Delayed enhance-ment cMRI is the best modality to separate these two entities. cMRI may show vascularization, areas of necrosis, hemorrhage, or calcification in cardiac tumors that are not present in thrombi.MyxomaPathology and Genetics. Cardiac myxomas are the most common cardiac tumor and are characterized by several distinguishing features. About 75% of the time, they arise from the interatrial septum near the fossa ovalis in the left atrium.296 Most others will develop in the right atrium, but, less commonly, they can arise from valvular surfaces and the walls of other cardiac chambers. Macroscopically, these tumors are pedunculated with a gelatinous consistency, and the surface may be smooth (65%), villous, or friable.291 Size varies greatly with these tumors and ranges from 1 to 15 cm in diameter. Internally, myxomas are heterogeneous and often contain hemorrhage, cysts, necrosis, or calcification. Histologically, these tumors contain cells that arise from a multipotent


Surgery_Schwartz

Surgery_Schwartz. 1 to 15 cm in diameter. Internally, myxomas are heterogeneous and often contain hemorrhage, cysts, necrosis, or calcification. Histologically, these tumors contain cells that arise from a multipotent mesenchyme and are contained within a mucopolysaccharide stroma.297While the majority of myxomas occur spontaneously with the highest incidence in women aged 40 to 60 years old, approx-imately 7% of cases are familial as part of Carney complex.291 Carney complex is an autosomal dominant disorder character-ized by two or more of the following conditions: atrial and extra-cardiac myxomas, schwannomas, cutaneous lentiginosis, spotty pigmentation, myxoid fibroadenomas of the breast, endocrine overactivity (pituitary adenomas or primary adrenal hyperplasia with Cushing’s syndrome), and testicular tumors. Compared to sporadic myxomas, those that occur as part of Carney complex are more commonly found in the right atrium (37% vs. 18%) or one of the ventricles (25% vs. 0%), more often


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Surgery_Schwartz. testicular tumors. Compared to sporadic myxomas, those that occur as part of Carney complex are more commonly found in the right atrium (37% vs. 18%) or one of the ventricles (25% vs. 0%), more often multicentric (33% vs. 6%) and more likely to recur (20% vs. 3%).296 They also present earlier at an average age of 24 years old (range 4–48 years).Pathophysiology. Larger tumors are more likely to be asso-ciated with cardiovascular symptoms from obstruction, and embolic symptoms tend to occur from organized thrombi pres-ent on friable or villous tumors (Fig. 21-16). The relative fre-quencies of symptoms was illustrated by a series of 112 patients who reported cardiovascular symptoms (67%), most commonly resembling mitral valve obstruction; systemic embolization (29%); neurologic deficits (20%); and constitutional symptoms (34%).291 Similar incidences of symptoms have been reported in other large studies.Treatment. Cardiac myxomas should be promptly excised after diagnosis due to the


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Surgery_Schwartz. (20%); and constitutional symptoms (34%).291 Similar incidences of symptoms have been reported in other large studies.Treatment. Cardiac myxomas should be promptly excised after diagnosis due to the significant risk of embolization and cardiovascular complications, including sudden death. Resec-tion may be performed through either a median sternotomy or 9Brunicardi_Ch21_p0801-p0852.indd 84201/03/19 5:32 PM 843ACQUIRED HEART DISEASECHAPTER 21Figure 21-16. Massive left atrial myxoma. A. Intraoperative echocardiogram of a large left atrial mass, diagnosed preoperatively as a left atrial myxoma. The mass can be seen prolapsing through the mitral valve orifice causing intermittent symptoms of mitral stenosis. B. The resected specimen. The neck of the mass that was obstructing the mitral orifice is clearly delineated.a minimally invasive right thoracotomy while on cardiopulmo-nary bypass. Care is taken not to manipulate the tumor before cross clamping of the aorta in order to avoid


Surgery_Schwartz

Surgery_Schwartz. orifice is clearly delineated.a minimally invasive right thoracotomy while on cardiopulmo-nary bypass. Care is taken not to manipulate the tumor before cross clamping of the aorta in order to avoid embolization. Left atrial tumors may be approached through a standard left atriotomy.298 Exposure of large tumors attached to the interatrial septum may be facilitated by an additional parallel incision in the right atrium, but this is rarely necessary. An ideal resection encompasses both the tumor and a portion of the cardiac wall or interatrial septum to which it is attached. In order to prevent recurrence, a full thickness excision of the attachment site is preferred, but partial thickness excisions and cryoablation of the base have been performed with good late results.298 The defect created in the atrial septum can either be repaired primarily or with a small patch. Finally, patients with valvular involvement may require additional valvular reconstruction or replacement, and rare cases


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Surgery_Schwartz. in the atrial septum can either be repaired primarily or with a small patch. Finally, patients with valvular involvement may require additional valvular reconstruction or replacement, and rare cases of cardiac autotransplantation (with atrial recon-struction) or transplantation have been reported as strategies for complex cases of recurrent atrial myxoma.299,300Shortand long-term results following excision are excellent for benign cardiac myxomas. Operative mortality is low, and the probability of disease-free survival at 20 years has been reported to be as high as 92% for benign, sporadic myxomas.291,298 Risk of recurrence is significantly higher for familial cases. Other risk factors for recurrence include younger age, smaller tumor mass, and ventricular tumor location.301Other Benign Cardiac TumorsThere are several benign cardiac tumors apart from myxomas that are infrequent but have distinct pathophysiologic features.292 Papillary fibroelastomas are the second most common primary


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Surgery_Schwartz. TumorsThere are several benign cardiac tumors apart from myxomas that are infrequent but have distinct pathophysiologic features.292 Papillary fibroelastomas are the second most common primary cardiac tumor, representing approximately 8% of all cases. These tumors typically occur in more elderly patients; are small (<1 cm in diameter) sessile, pedunculated masses that arise from the mitral or aortic valves; and frequently result in embo-lization. Fibroelastomas can almost always be resected with preservation of the native valve leaflets, and cryoablation of the valve leaflet after resection can help prevent recurrence. Lipomas are encapsulated tumors that usually arise from the epicardium and remain asymptomatic in most patients. Heman-giomas, which may arise from any cardiac structure, including the pericardium, account for 2% of benign cardiac tumors, and atrioventricular node tumors, which often lead to sudden cardiac death from heart block and ventricular fibrillation, are


Surgery_Schwartz

Surgery_Schwartz. including the pericardium, account for 2% of benign cardiac tumors, and atrioventricular node tumors, which often lead to sudden cardiac death from heart block and ventricular fibrillation, are exceedingly rare.In children, rhabdomyomas are the most common pri-mary cardiac tumor, whereas fibromas are the most commonly resected cardiac tumor. Rhabdomyomas are myocardial hamar-tomas that are often multicentric in the ventricles. About 50% of cases are associated with tuberous sclerosis, and while resection is occasionally necessary, most disappear spontaneously. Fibro-mas are congenital lesions that one-third of the time are found in children younger than 1-year old. These tumors, conversely, are ordinarily solitary lesions found in the inner interventricu-lar septum, and they may present with heart failure, cyanosis, arrhythmias, syncopal episodes, chest pain, or sudden cardiac death.Malignant Cardiac TumorsPrimary cardiac malignancies are very rare, but when they occur they tend to


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Surgery_Schwartz. with heart failure, cyanosis, arrhythmias, syncopal episodes, chest pain, or sudden cardiac death.Malignant Cardiac TumorsPrimary cardiac malignancies are very rare, but when they occur they tend to have a right-sided predominance and frequently demonstrate extracardiac extension and involvement.292,302 Malignant cardiac tumors include intimal sarcoma, angiosar-coma, osteosarcoma, leiomyosarcoma, rhabdomyosarcoma, liposarcoma, and primary cardiac lymphomas. Intimal sarcoma is the most common subtype.303 Angiosarcomas are aggressive, rapidly invading adjacent structures, and 47% to 89% of patients present with lung, liver, or brain metastases by the time of diag-nosis. Leiomyosarcomas are sessile masses with a mucous appearance that are typically found in the posterior wall of the left atrium. Rhabdomyosarcomas are bulky (>10 cm in diam-eter) tumors that usually occur in children and do not have a predilection for any particular chamber. They frequently invade nearby cardiac structures


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Surgery_Schwartz. Rhabdomyosarcomas are bulky (>10 cm in diam-eter) tumors that usually occur in children and do not have a predilection for any particular chamber. They frequently invade nearby cardiac structures and are multicentric in 60% of cases. Finally, while not as frequent as secondary cardiac lymphomas, primary cardiac lymphomas are increasing in frequency due to lymphoproliferative disorders caused by Epstein-Barr virus in immunosuppressed patients. The absence of necrotic foci in lymphomas can be used to differentiate these tumors from car-diac sarcomas.Metastatic Cardiac TumorsCardiac metastases have been found in approximately 10% of autopsies performed for malignant disease.292 Secondary cardiac tumors, unlike primary tumors, are therefore relatively common. They may arise from direct extension of mediastinal tumors, hematological spread, intracavitary extension from the inferior vena cava or lymphatic extension, although the latter is the most common mechanism.While they can occur with


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Surgery_Schwartz. of mediastinal tumors, hematological spread, intracavitary extension from the inferior vena cava or lymphatic extension, although the latter is the most common mechanism.While they can occur with most any primary tumor, they are generally observed late in the course of disease. Malignant melanomas have a high potential for cardiac involvement, but Brunicardi_Ch21_p0801-p0852.indd 84301/03/19 5:32 PM 844SPECIFIC CONSIDERATIONSPART IIother soft tissue tumors such as lung cancer, breast cancer, sarcomas, renal carcinoma, esophageal cancer, hepatocellu-lar carcinoma, and thyroid cancer may all progress to cardiac involvement. Cardiac metastases may also develop from leuke-mia and lymphoma in 25% to 40% of cases.304Metastatic cardiac tumors are typically found in random locations, excluding the valvular tissue where lymphatics are absent, and they may be multifocal or diffusely extend along the epicardial surface. Signs of malignant cardiac involvement in cancer patients include


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Surgery_Schwartz. the valvular tissue where lymphatics are absent, and they may be multifocal or diffusely extend along the epicardial surface. Signs of malignant cardiac involvement in cancer patients include pericardial effusion or tamponade, tachyarrhythmias, and heart failure symptoms. Workup is simi-lar to other cardiac tumors. Treatment is generally with com-bined chemotherapy and radiation and is rarely effective.REFERENCESEntries highlighted in bright blue are key references. 1. Task Force for the Diagnosis and Management of Syncope; European Society of Cardiology (ESC); European Heart Rhythm Association (EHRA); et al. Guidelines for the diag-nosis and management of syncope (version 2009). Eur Heart J. 2009;30(21):2631-2671. 2. Braunwald E, Bonow RO. Braunwald’s Heart Disease: A Textbook of Cardiovascular Medicine, 9th ed. Philadel-phia: Saunders; 2012. 3. New York Heart Association. Diseases of the Heart and Blood Vessels: Nomenclature and Criteria for Diagnosis, 6th ed. Boston: Little,


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Surgery_Schwartz. Medicine, 9th ed. Philadel-phia: Saunders; 2012. 3. New York Heart Association. Diseases of the Heart and Blood Vessels: Nomenclature and Criteria for Diagnosis, 6th ed. Boston: Little, Brown; 1964. 4. Lee DH, Buth KJ, Martin BJ, Yip AM, Hirsch GM. Frail patients are at increased risk for mortality and pro-longed institutional care after cardiac surgery. Circulation. 2010;121(8):973-978. 5. Smilowitz NR, Gupta N, Ramakrishna H, Guo Y, Berger JS, Bangalore S. Perioperative major adverse cardiovascular and cerebrovascular events associated with noncardiac surgery. JAMA Cardiol. 2017;2(2):181-187. 6. Nishimura RA, Otto CM, Bonow RO, et al. 2017 AHA/ACC focused update of the 2014 AHA/ACC guideline for the man-agement of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on clinical practice guidelines. J Am Coll Cardiol. 2017;70(2):252-289. 7. Fleisher LA, Fleischmann KE, Auerbach AD, et al. 2014 ACC/AHA guideline on


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Surgery_Schwartz. Cardiology/American Heart Association Task Force on clinical practice guidelines. J Am Coll Cardiol. 2017;70(2):252-289. 7. Fleisher LA, Fleischmann KE, Auerbach AD, et al. 2014 ACC/AHA guideline on perioperative cardiovascular eval-uation and management of patients undergoing noncardiac surgery: a report of the American College of Cardiology/American Heart Association Task Force on practice guide-lines. J Am Coll Cardiol. 2014;64(22):e77-e137. 8. Klocke FJ, Baird MG, Lorell BH, et al. ACC/AHA/ASNC guide-lines for the clinical use of cardiac radionuclide imaging— executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/ASNC Committee to Revise the 1995 Guidelines for the Clinical Use of Cardiac Radionuclide Imaging). Circulation. 2003;108(11):1404-1418. 9. Badheka AO, Hendel RC. Radionuclide cardiac stress testing. Curr Opin Cardiol. 2011;26(5):370-378. 10. Bax JJ, Boogers MM, Schuijf JD. Nuclear imaging in


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