Showkat Haji, M.D. Lawrence O’Meallie, M.D.

1. Cath ConferenceAugust 31,2004Cardiac tumors Showkat Haji, M.D. Lawrence O’Meallie, M.D.

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

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

4. Characteristic Thrombus Tumor 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. – 2nd ed

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

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

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

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

9. Cardiac tumors (Incidence of benign tumors)

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

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

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

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

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

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

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