Cardiac Tumors Flashcards
(25 cards)
What is the most commonly occurring tumor found in the pediatric population?
Cardiac rhabdomyoma
Rhabdomyomas may occur in multiple organs and are associated with Tuberous Sclerosis.
What genetic syndrome is associated with cardiac rhabdomyomas?
Tuberous Sclerosis
Tuberous Sclerosis is a genetic, autosomal dominant syndrome.
What percentage of patients with Tuberous Sclerosis develop cardiac rhabdomyomas?
50%
This statistic highlights the strong association between Tuberous Sclerosis and cardiac rhabdomyomas.
How are rhabdomyomas classified in terms of their growth and behavior?
Benign abnormal growth of cardiac myocytes
Rhabdomyomas may become problematic if their growth inhabits critical intracardiac locations.
What complications can arise from the growth of cardiac rhabdomyomas?
Obstruction (inflow/outflow) and/or potentially lethal dysrhythmias
These complications can occur if rhabdomyomas grow in critical intracardiac locations.
Do cardiac rhabdomyomas typically metastasize?
No
Historically, cardiac rhabdomyomas do not metastasize and often regress over time.
What is the survival rate for patients with cardiac rhabdomyomas?
81-92%
This survival rate reflects the typical outcome for patients with rhabdomyomas.
What is the preferred diagnostic tool for detecting cardiac rhabdomyomas?
Echocardiography
Echocardiography is used for detection and evaluation of progression and regression of cardiac rhabdomyomas.
Can rhabdomyomas be diagnosed prenatally?
Yes
Rhabdomyomas may be diagnosed both pre or postnatally.
How do rhabdomyomas typically appear on echocardiography?
Well defined, homogenous, hyperechoic masses
They range in size and may be isolated or diffuse.
Where are rhabdomyomas most commonly found in the heart?
Ventricular myocardium
Rhabdomyomas may also be found in the atria, cavoatrial junction, and epicardial surface.
What percentage of primary cardiac tumors in infancy and childhood are benign?
Approximately 70%
The morbidity rate for these tumors is around 0.27% to 0.8%.
What type of tumor accounts for around 2% to 5% of all benign tumors in the heart?
Cardiac fibromas
Cardiac fibromas arise from heart fibroblasts or myofibroblasts.
What is the most common location for fibromas in the heart?
Ventricular septum
Fibromas may occasionally be found in the ventricular free wall.
What complications can arise from cardiac fibromas?
- Inflow tract obstruction
- Outflow tract obstruction
- Valvular dysfunction
- Ventricular arrhythmias
In surgical series, where do cardiac fibromas rank among cardiac tumors in infancy?
First
They are the second most common cardiac tumors in infancy overall.
What imaging technique is used to demonstrate a hypoperfused tumor core in cardiac fibromas?
Gadolinium enhanced MRA and first pass perfusion imaging
True or False: Cardiac fibromas are the most common cardiac tumors in infancy.
False
They are the second most common cardiac tumors in infancy.
What is the autopsy frequency of cardiac tumors in children?
0.001% to 0.30%
This indicates how rare these tumors are in the pediatric population.
What are the most common primary cardiac tumors in children?
Benign tumors such as:
* Rhabdomyoma
* Myxoma
* Teratoma
* Fibroma
Most primary cardiac tumors in children are benign.
What is the incidence of papillary fibroelastoma in children?
0.13% in autopsy series
This tumor is the third most common in adults but very rare in children.
Which structures do cardiac tumors typically involve in children?
Left-sided valvular structures
These tumors can lead to significant complications.
What serious complications can be associated with cardiac tumors in children?
- Myocardial infarction
- Stroke
- Sudden death
These complications highlight the potential severity of cardiac tumors.
What is the range of clinical presentations for children with cardiac tumors?
From asymptomatic patients to those with:
* Sequelae from outflow tract obstruction
* Systemic emboli
* Pulmonary emboli
The symptoms can vary widely depending on the tumor’s impact.
