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ECHO 2013 / Cardiac Tumors and Masses

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CPF: Left Ventricular Chambers

CPF: Clinical Signs and Symptoms

Asymptomatic in vast majority

incidental finding on surgically excised valves or necrospy

CVA due to embolization of the papillary fronds of the tumor itself or from a superimposed thrombus can occur.

Risk factors: size (≥ 1 cm), mobility, location (left sided lesions)

Rare reports of angina or sudden cardiac death from coronary ostial occlusion

CPF: Management

Symptoms

yes

no

surgical candidate

R side

L side

 

 

 

recommendations NOT based on

 

no

randomized controlled data

high risk features

yes

Clinical f/u

Clinical f/u

 

Consider a/c

 

surgery

no

yes

Consider a/c

 

 

 

Sun et al, Circulation 2001; 103:2687-2693 Gowda et al, AHJ 2003; 146:404-410

low surgical risk

yes

no

surgery

Clinical f/u

 

 

Consider a/c

Majority of cases (83%) can be safely resected by valve sparing, simple shave excision. Valve repair or replacement was rarely necessary.

Low surgical mortality (<2%)

No tumor recurrence and no tumor-related late mortality or morbidity during a follow-up period lasting up to 8.3 years.

Lambl’s Excrescences

Fine thread like strands arising on the line of closure (contact surface) of heart valves

Found in 70-80% of older adults; often multiple

Pathogenesis

Degenerative wear and tear of the valvular endocardial surfaces where the valve margins contact

Association with embolization - controversial

RHABDOMYOMA

Most common primary cardiac tumor in the pediatric age group

approximately 3/4 occur in < 1 year of age

Strongly associated with tuberous sclerosis (familial syndrome of systemic hemartoma)

Derived from cardiac muscle; may actually be cardiac hemartoma or malformation rather than true neoplasm

RHABDOMYOMA: Gross Pathology

Yellow-gray; circumscribed

Range from 1mm to several cm in diameter

Locations

90% are multiple

equal frequency in LV/RV/septum

1/3 also involve atria

LV

 

mostly intramural with intracavitary extension in 50% of cases

Rhabdomyoma

Fetal Ultrasound: In-Utero

Rhabdomyoma

One month after birth

RHABDOMYOMA: Clinical Aspects

Obstruction: inflow or outflow

Arrythmia

AV block or incessant VT

sudden death

Presence of multiple nodular masses in several chambers on 2D echo is diagnostic

Limited ability to grow and tend to regress

Surgery only indicated for symptomatic patients