Showkat Haji, M.D. Lawrence O’Meallie, M.D. Showkat Haji, M.D. Lawrence O’Meallie, M.D. Cath Conference August 31,2004 Cardiac tumors Cath Conference August 31,2004 Cardiac tumors

24 y/o wm transferred from St. Tammany Hospital due to 24 y/o wm transferred from St. Tammany Hospital due to multi-organ failure multi-organ failure Eight months before this admission patient was Eight months before this admission patient was found to have a mediastinal mass measuring found to have a mediastinal mass measuring 21x18x31cm with transthoracic needle biopsy 21x18x31cm with transthoracic needle biopsy confirming Non Hodgkins lymphoma. There was no confirming Non Hodgkins lymphoma. There was no other involvement from the lymphoma. Cardiac function other involvement from the lymphoma. Cardiac function by echocardiogram was normal. by echocardiogram was normal. Pt received 8 cycles of CH(Hydrea-Doxorubicin)OP Pt received 8 cycles of CH(Hydrea-Doxorubicin)OP 24 y/o wm transferred from St. Tammany Hospital due to 24 y/o wm transferred from St. Tammany Hospital due to multi-organ failure multi-organ failure Eight months before this admission patient was Eight months before this admission patient was found to have a mediastinal mass measuring found to have a mediastinal mass measuring 21x18x31cm with transthoracic needle biopsy 21x18x31cm with transthoracic needle biopsy confirming Non Hodgkins lymphoma. There was no confirming Non Hodgkins lymphoma. There was no other involvement from the lymphoma. Cardiac function other involvement from the lymphoma. Cardiac function by echocardiogram was normal. by echocardiogram was normal. Pt received 8 cycles of CH(Hydrea-Doxorubicin)OP Pt received 8 cycles of CH(Hydrea-Doxorubicin)OP Case 1 Case 1

Three weeks prior to this admission, pt felt tired and Three weeks prior to this admission, pt felt tired and short of breath and was admitted to St tamany short of breath and was admitted to St tamany hospital with multi-organ failure. Pt was in respiratory hospital with multi-organ failure. Pt was in respiratory failure, renal failure and had evidence of shock liver. failure, renal failure and had evidence of shock liver. Echocardiogram showed LV systolic dysfunction and Echocardiogram showed LV systolic dysfunction and a LV mass. a LV mass. on arrival at Tulane pt was sedated, intubated and on arrival at Tulane pt was sedated, intubated and hemodynamically stable. hemodynamically stable. Labs showed a WBC count of 22,000, plt 198,000 Labs showed a WBC count of 22,000, plt 198,000 Creatinine of 2.1, Bilirubin 6.4, AST 14758, ALT 5015, Creatinine of 2.1, Bilirubin 6.4, AST 14758, ALT 5015, Alk phos 105, Troponin of 3.0. Alk phos 105, Troponin of 3.0. Echocardiogram was obtained. Echocardiogram was obtained. Three weeks prior to this admission, pt felt tired and Three weeks prior to this admission, pt felt tired and short of breath and was admitted to St tamany short of breath and was admitted to St tamany hospital with multi-organ failure. Pt was in respiratory hospital with multi-organ failure. Pt was in respiratory failure, renal failure and had evidence of shock liver. failure, renal failure and had evidence of shock liver. Echocardiogram showed LV systolic dysfunction and Echocardiogram showed LV systolic dysfunction and a LV mass. a LV mass. on arrival at Tulane pt was sedated, intubated and on arrival at Tulane pt was sedated, intubated and hemodynamically stable. hemodynamically stable. Labs showed a WBC count of 22,000, plt 198,000 Labs showed a WBC count of 22,000, plt 198,000 Creatinine of 2.1, Bilirubin 6.4, AST 14758, ALT 5015, Creatinine of 2.1, Bilirubin 6.4, AST 14758, ALT 5015, Alk phos 105, Troponin of 3.0. Alk phos 105, Troponin of 3.0. Echocardiogram was obtained. Echocardiogram was obtained. Case 1 Case 1

CharacteristicThrombusTumor Vegetation Location LA (especially when enlarged or associated with MV disease) LV (in setting of reduced systolic function or segmental wall abnormalities) Atria (myxoma), more frequent in LA Myocardium Pericardium Valves Usually valvular Occasionally on ventricular wall Appearance Usually discrete and somewhat spherical in shape or laminated against LV apex or LA wall Various: may be circumscribed or may be irregular Irregular shape, attached to the proximal (upstream) side of the valve with motion independent from the valve Associated findings Underlying etiology usually evident (atrial fibrillation) LV systolic dysfunction or segmental wall motion abnormalities (exception eosinophilic heart disease) MV disease with LA enlargement Intracardiac obstruction depending on site of tumor Arrhythmias Pericardial effusion, which usually is bloody Valvular regurgitation usually present Clinically: fever, systemic signs of endocarditis, positive blood cultures Distinguishing Characteristics of Intracardiac Masses Adapted from the Textbook of clinical echocardiography/ Catherine M. Otto. – 2 nd ed

PET scan showed a superior mediastinal mass without PET scan showed a superior mediastinal mass without involvement of cardiac structures. There were no other involvement of cardiac structures. There were no other sites of involvement. sites of involvement. Gallium scan showed hot spots in the upper and middle Gallium scan showed hot spots in the upper and middle mediastinum consistent with upper mediastinal mass mediastinum consistent with upper mediastinal mass saving the cardiologists the pain of a definitive diagnosis saving the cardiologists the pain of a definitive diagnosis of the LV mass. of the LV mass. Pt was treated with Rituximab and the mass in the LV Pt was treated with Rituximab and the mass in the LV was presumed to be a LV thrombus. was presumed to be a LV thrombus. Pt was aggressively treated for heart failure with the Pt was aggressively treated for heart failure with the heart failure cocktail. Amiodarone was started for NSVT heart failure cocktail. Amiodarone was started for NSVT PET scan showed a superior mediastinal mass without PET scan showed a superior mediastinal mass without involvement of cardiac structures. There were no other involvement of cardiac structures. There were no other sites of involvement. sites of involvement. Gallium scan showed hot spots in the upper and middle Gallium scan showed hot spots in the upper and middle mediastinum consistent with upper mediastinal mass mediastinum consistent with upper mediastinal mass saving the cardiologists the pain of a definitive diagnosis saving the cardiologists the pain of a definitive diagnosis of the LV mass. of the LV mass. Pt was treated with Rituximab and the mass in the LV Pt was treated with Rituximab and the mass in the LV was presumed to be a LV thrombus. was presumed to be a LV thrombus. Pt was aggressively treated for heart failure with the Pt was aggressively treated for heart failure with the heart failure cocktail. Amiodarone was started for NSVT heart failure cocktail. Amiodarone was started for NSVT Case 1 Case 1

28 year-old African American male with history of HIV 28 year-old African American male with history of HIV diagnosed in 1998 and a CD4 count of 1,050, underwent diagnosed in 1998 and a CD4 count of 1,050, underwent a Bilroth II procedure for a perforated peptic ulcer. a Bilroth II procedure for a perforated peptic ulcer. Due to persistent abdominal pain a laparotomy was Due to persistent abdominal pain a laparotomy was done a week later and demonstrated significant done a week later and demonstrated significant lymphadenopathy lymphadenopathy A pathology report of an intra abdominal lymph node A pathology report of an intra abdominal lymph node was consistent with non-Hodgkin Burkitt’s lymphoma. was consistent with non-Hodgkin Burkitt’s lymphoma. 28 year-old African American male with history of HIV 28 year-old African American male with history of HIV diagnosed in 1998 and a CD4 count of 1,050, underwent diagnosed in 1998 and a CD4 count of 1,050, underwent a Bilroth II procedure for a perforated peptic ulcer. a Bilroth II procedure for a perforated peptic ulcer. Due to persistent abdominal pain a laparotomy was Due to persistent abdominal pain a laparotomy was done a week later and demonstrated significant done a week later and demonstrated significant lymphadenopathy lymphadenopathy A pathology report of an intra abdominal lymph node A pathology report of an intra abdominal lymph node was consistent with non-Hodgkin Burkitt’s lymphoma. was consistent with non-Hodgkin Burkitt’s lymphoma. Case 2 Case 2

An abdominal and chest computed tomography An abdominal and chest computed tomography demonstrated mediastinal lymphadenopathy with demonstrated mediastinal lymphadenopathy with invasion of cardiac structures invasion of cardiac structures An echocardiogram showed biatrial masses, which An echocardiogram showed biatrial masses, which were non-mobile and attached to the roof of both atria. were non-mobile and attached to the roof of both atria. Moderate pericardial effusion, left atrial enlargement Moderate pericardial effusion, left atrial enlargement and left ventricular hypertrophy were also present and left ventricular hypertrophy were also present Systolic function was preserved. Systolic function was preserved. A combination chemotherapy regimen consisting of A combination chemotherapy regimen consisting of rituximab, cytarabine, vincristine and prednisone was rituximab, cytarabine, vincristine and prednisone was initiated. initiated. An abdominal and chest computed tomography An abdominal and chest computed tomography demonstrated mediastinal lymphadenopathy with demonstrated mediastinal lymphadenopathy with invasion of cardiac structures invasion of cardiac structures An echocardiogram showed biatrial masses, which An echocardiogram showed biatrial masses, which were non-mobile and attached to the roof of both atria. were non-mobile and attached to the roof of both atria. Moderate pericardial effusion, left atrial enlargement Moderate pericardial effusion, left atrial enlargement and left ventricular hypertrophy were also present and left ventricular hypertrophy were also present Systolic function was preserved. Systolic function was preserved. A combination chemotherapy regimen consisting of A combination chemotherapy regimen consisting of rituximab, cytarabine, vincristine and prednisone was rituximab, cytarabine, vincristine and prednisone was initiated. initiated. Case 2 Case 2

Primary cardiac tumors are rare, with an autopsy Primary cardiac tumors are rare, with an autopsy incidence of 0.05% incidence of 0.05% A quarter of primary tumors are malignant, the vast A quarter of primary tumors are malignant, the vast majority being angiosarcomas or Rhabdomyosarcomas majority being angiosarcomas or Rhabdomyosarcomas Metastatic tumors are more common than primary (30:1) Metastatic tumors are more common than primary (30:1) Neoplasms may involve only the endocardium, Neoplasms may involve only the endocardium, myocardium or the epicardium or various combinations myocardium or the epicardium or various combinations Neoplasms limited to parietal pericardium without Neoplasms limited to parietal pericardium without extension into the epicardium are not considered cardiac extension into the epicardium are not considered cardiac neoplasms neoplasms Primary cardiac tumors are rare, with an autopsy Primary cardiac tumors are rare, with an autopsy incidence of 0.05% incidence of 0.05% A quarter of primary tumors are malignant, the vast A quarter of primary tumors are malignant, the vast majority being angiosarcomas or Rhabdomyosarcomas majority being angiosarcomas or Rhabdomyosarcomas Metastatic tumors are more common than primary (30:1) Metastatic tumors are more common than primary (30:1) Neoplasms may involve only the endocardium, Neoplasms may involve only the endocardium, myocardium or the epicardium or various combinations myocardium or the epicardium or various combinations Neoplasms limited to parietal pericardium without Neoplasms limited to parietal pericardium without extension into the epicardium are not considered cardiac extension into the epicardium are not considered cardiac neoplasms neoplasms Cardiac tumors Cardiac tumors

Cardiac tumors (Incidence of benign tumors) Cardiac tumors (Incidence of benign tumors) Incidence % Incidence % Adults Children Adults Children MyxomaLipoma Pappilary Fibroelastoma AngiomaFibromaHaemangiomaRhabdomyomaTeratoma <1 <

Cardiac tumors (General clinical features) Cardiac tumors (General clinical features) Atrial myxomas may cause systemic symptoms such as fatigue, Atrial myxomas may cause systemic symptoms such as fatigue, fever, erythematous rash, arthralgia, myalgia and wt. loss fever, erythematous rash, arthralgia, myalgia and wt. loss Elevations in ESR, CRP and globulin levels Elevations in ESR, CRP and globulin levels Irrespective of size and site. Irrespective of size and site. Clinical features common to all tumors include Clinical features common to all tumors include Embolization: Tumor or an attached thrombus may dislodge Embolization: Tumor or an attached thrombus may dislodge Multiple small emboli may mimic vasculitis Multiple small emboli may mimic vasculitis Obstruction: Mimic valvular stenosis. May cause outflow Obstruction: Mimic valvular stenosis. May cause outflow obstruction leading to chest pain, breathlessness or syncope obstruction leading to chest pain, breathlessness or syncope Arrhythmias: AV blocks, V. Tachycardia Arrhythmias: AV blocks, V. Tachycardia Initial presentation may be with sudden death. Initial presentation may be with sudden death.

Myxomas Myxomas 90 % are left atrial 90 % are left atrial 90 % are solitary 90 % are solitary Associated facial freckling should raise the possibility of Carney Associated facial freckling should raise the possibility of Carney complex, in which case family members should be screened. complex, in which case family members should be screened. Clinical features may mimic infective endocarditis, vasculitis Clinical features may mimic infective endocarditis, vasculitis or other inflammatory disorders or other inflammatory disorders Differentiation from an intra-atrial thrombus may be important. Differentiation from an intra-atrial thrombus may be important. Surgical resection is advisable as soon as possible after diag Surgical resection is advisable as soon as possible after diag as the risk of embolization is high as the risk of embolization is high Recurrence is possible. Long term ECHO f/u is recommended Recurrence is possible. Long term ECHO f/u is recommended

Other benign cardiac tumors Other benign cardiac tumors Pappilary fibroelastomas have a high incidence of embolization Pappilary fibroelastomas have a high incidence of embolization Rhabdomyoma is the most common neoplasm of childhood, Rhabdomyoma is the most common neoplasm of childhood, often seen in association with tuberous sclerosis. Spontaneous often seen in association with tuberous sclerosis. Spontaneous tumor resolution is common tumor resolution is common Lipomas are encapsulated and usually asymptomatic Lipomas are encapsulated and usually asymptomatic Angiomas are extremely rare. Angiomas are extremely rare.

Malignant primary cardiac tumors Malignant primary cardiac tumors The majority of malignant primary cardiac tumors are sarcomas The majority of malignant primary cardiac tumors are sarcomas (95%) usually angiosarcomas or rhabdomyosarcomas. (95%) usually angiosarcomas or rhabdomyosarcomas. Most malignant primary cardiac tumors are right atrial in location Most malignant primary cardiac tumors are right atrial in location The incidence of primary intracardiac lymphomas is increasing, The incidence of primary intracardiac lymphomas is increasing, seen as part of AIDS. seen as part of AIDS. Treatment is rarely curative and survival is poor. Treatment is rarely curative and survival is poor.

Secondary cardiac tumors Secondary cardiac tumors Secondary cardiac tumors are usually epicardial and Secondary cardiac tumors are usually epicardial and asymptomatic. asymptomatic. Metastasis is rarely limited solely to the heart. Metastasis is rarely limited solely to the heart. Pericardial effusion is common Pericardial effusion is common Melanoma, leukemia and lymphoma are most commonly Melanoma, leukemia and lymphoma are most commonly associated with metastasis to the heart. associated with metastasis to the heart. Carcinoma of the lung is the most commonly encountered Carcinoma of the lung is the most commonly encountered metastatic tumor followed by cancer of breast, lymphoma and metastatic tumor followed by cancer of breast, lymphoma and leukemia. leukemia.

Non neoplastic conditions Non neoplastic conditions Pericardial cyst: These are frequent benign tumors of the Pericardial cyst: These are frequent benign tumors of the pericardium. They are asymptomatic and found on a routine pericardium. They are asymptomatic and found on a routine chest radiograph. chest radiograph. Lipomatous hypertrophy of the atrial atrium: Extremely Lipomatous hypertrophy of the atrial atrium: Extremely common condition almost exclusively in patients above 50. common condition almost exclusively in patients above 50. Limited to obese people. Limited to obese people. Atrial septum may be as thick as 7 cm. Atrial septum may be as thick as 7 cm. May present as atrial arrhythmias. May present as atrial arrhythmias. Treatment is simply wt. loss. Treatment is simply wt. loss. Thrombi: Sometimes biopsy is the only way to differentiate. Thrombi: Sometimes biopsy is the only way to differentiate. Cardiac hemorrhages: Thrombocytopenia if persistent, can Cardiac hemorrhages: Thrombocytopenia if persistent, can result in focal epi, myocardial and endocardial hemorrhage. result in focal epi, myocardial and endocardial hemorrhage.

Heart and malignant lymphoma Heart and malignant lymphoma Primary lymphomas of the heart are very rare, although Primary lymphomas of the heart are very rare, although incidence has been rising as they are seen as part of AIDS. incidence has been rising as they are seen as part of AIDS. Chief presentation is with intractable heart failure. Chief presentation is with intractable heart failure. Secondary involvement is more common Secondary involvement is more common 20% of patients dying of lymphoma have cardiac involvement. 20% of patients dying of lymphoma have cardiac involvement. Parietal pericardium is involved in most of the patients Parietal pericardium is involved in most of the patients Pericardial effusions are common Pericardial effusions are common Usually associated with diffuse metastasis Usually associated with diffuse metastasis