Abcd2

Abcd2 and stroke risk

Cardiac papillary fibroelastoma: A comprehensive

 

Cardiac papillary fibroelastoma: A comprehensive

analysis of 725 cases

Ramesh M. Gowda, MD,a Ijaz A. Khan, MD, FACP, FACC,b Chandra K. Nair, MD, FACP, FACC,b

Nirav J. Mehta, MD,b Balendu C. Vasavada, MD, FACC,a and Terrence J. Sacchi, MD, FACCa Omaha, Neb, and

Brooklyn, NY

 

(Am Heart J 2003;146:404–10.)

 

Seven hundred twenty-five cases of CPF were identified. Males comprised 55% of

patients. Highest prevalence was in the 8th decade of life. The valvular surface was the predominant locations of tumor.

The most commonly involved valve was the aortic valve, followed by the mitral valve. The left ventricle was the predominant

nonvalvular site involved. No clear risk factor for development of CPF has been reported. Size of the tumor varied

from 2 mm to 70 mm. Clinically, CPFs have presented with transient ischemic attack, stroke, myocardial infarction, sudden

death, heart failure, presyncope, syncope, pulmonary embolism, blindness, and peripheral embolism. Tumor mobility

was the only independent predictor of CPF-related death or nonfatal embolization. Symptomatic patients should be

treated surgically because the successful complete resection of CPF is curative and the long-term postoperative prognosis

is excellent. The symptomatic patients who are not surgical candidates could be offered long-term oral anticoagulation,

although no randomized controlled data are available on its efficacy. Asymptomatic patients could be treated surgically if

the tumor is mobile, as the tumor mobility is the independent predictor of death or nonfatal embolization. Asymptomatic

patients with nonmobile CPF could be followed-up closely with periodic clinical evaluation and echocardiography, and

receive surgical intervention when symptoms develop or the tumor becomes mobile.

 

Additional pearls from this review:

 

1. 

Cardiac

papillary fibroelastomas (CPF), the second most

common primary cardiac tumors, are benign endocardial

papillomas predominantly affecting the cardiac

valves, and account for three fourths of all cardiac valvular

tumors. They consist of a small, highly papillary,

pedunculated and avascular tumor, covered by a single

layer of endothelium, containing variable amounts of

fine elastic fibrils arranged in whorls in a hyaline

stroma.

2.

Although CPF are rare and histologically benign

tumors, they may result in life-threatening complications,

such as stroke, acute valve dysfunction, embolism,

and sudden death.

3.

The most common clinical presentation was of transient

ischemic attack or stroke (n 120 patients). The

other manifestations were angina (n 49 patients),

myocardial infarction (n 28 patients), sudden death

(n 21 patients), heart failure (n 24 patients), presyncope

or syncope (n 12 patients), pulmonary

embolism (n 3 patients), blindness (n 7 patients),

mesenteric ischemia (n 2 patients), peripheral emboli

(n 3 patients), and renal infarction (n 1 patient).

In patients with mitral valve tumors, stroke was

the most common clinical presentation, but in patients

with aortic valve tumors, sudden death and myocardial

infarction were the 2 most common presentations. In

a large number of patients, tumors were identified as

incidental findings at autopsy (n 209 patients) or at

surgery for other cardiac reasons (n 9 patients).

4.

The major immunophenotypic

difference between CPF and cardiac myxoma was the

frequent presence of muscle-specific actin in the stellate

cells of the stroma in cardiac myxoma but not in

CPF.

5. 

Because most CPF are located in the left heart (95%),

systemic embolism in particular is frequent. In a majority

of CPF cases with embolism, the cerebral arteries,

including retinal arteries, are affected, and transient

ischemic attack, stroke, and sudden visual loss have

been reported

6.

The classic

diastolic tumor plop, which is heard in one third of

patients with atrial myxoma, has not been described

with CPF, except in 1 case where a tricuspid valve

CPF was associated with a tumor plop.46

7. Diferential diagnostic analysis:

Cardiac myxoma

is a predominant left atrial tumor, and is usually attached

to the atrial septum by a stalk. Histologically,

myxoma differs from CPF by presence of polygonal

 

myxoma cells and blood vessels within papillae.125

The papillae of CPF are devoid of blood vessels. Calretinin

staining may be a useful way to distinguish between

myxoma and CPF.218 Cardiac fibroma, which is

a separate entity, frequently demonstrates calcification

and cystic degeneration. Cardiac rhabdomyomas are

predominant myocardial neoplasms seen in infants and

children.214 Metastatic tumors of the heart are more

frequent than the primary tumors.1 Unlike CPF, malignant

tumors commonly involve the pericardium and

myocardium, and are usually accompanied by systemic

symptoms. However, with both primary and metastatic

tumors, the clinical course may be complicated by emboli.

Ljevak J, Mismas A, Bazina A, et al.
An infrequent type of stroke with an unusual cause and successful therapy: basilar artery occlusion caused by a cardiac papillary fibroelastoma recanalized 12 hours after onset [In Process Citation]
Intern Med (Japan), 2013, 52(2) p277-9

 

take home points: Again, BA can be recanalized later than other vessels; this tumor type is very sensitive to lysis

Etiology and treatment of ischaemic stroke in patients with (sup)-thalassemia major.

Eur J Neurol 2011 Dec;18(12):1426-8    (ISSN: 1468-1331)

 

BACKGROUND AND PURPOSE: Although hypercoagulability-induced thromboembolism is generally accepted as cause of cerebral ischaemia in thalassemic patients, cardiogenic embolism has been recently suggested as another possible stroke etiology. METHODS: We present four adult (sup)-thalassemia major patients with manifest cardiac involvement who suffered territorial strokes. RESULTS: In the presence of siderotoxic cardiomyopathy and arrhythmia, we assumed cardiogenic embolism as etiology of stroke and initiated oral anticoagulation as preventive medication. Two of our patients were the first (sup)-thalassemia major patients who underwent successful thrombolysis with rtPA. CONCLUSIONS: Cardioembolism seems to be the cause of stroke in cases of (sup)-thalassemia major. Thrombolysis can be applied in the setting of acute brain ischaemia in such high risk patients. [ 2011 The Author(s). European Journal of Neurology 2011 EFNS.].

 

Comment:  Take home points

1.  Thal cases can be cardioembolic not just hypercoagulable and may benefit from anticoagulation

2.  Thrombolysis might help in some cases