Tumors

Question 1

Primary tumors — benign 75%

Answer 1

•Myxomas 24%
- middle aged adults (30 - 60 yrs)
- women > men
- most common cardiac neoplasm in adulthood
- LA 75% > RA 23% > LV/RV 2%
- valvular origin is uncommon
- often attached to fossa ovalis
- mobile, irregular, pedunculated
- protruding fronds of tissue or grape cluster appearance
- TTE usually sufficient — MRI/CT don’t add much (stalk not defined)
- generally sporadic with low recurrence
- ventricular more common in women and children
- occasionally multiple & familial
• Lipomas 10%
- can be found anywhere in the heart (more common extramyocardial or subpericardial)
-lipomatous hypertrophy is a benign condition — fat accumulation of interatrial septum
•Papillary fibroelastomas 8%
- arises on valvular tissue (valve dysfunction uncommon)
- mimics appearance of vegetation w/ similar appearance to Lambl’s excrescences
- often see on downstream side of valve (LV side of MV / aortic side of AV)
- most common in AV and MV but TV and PV reported
- well-demarcated borders w/ homogeneous texture
- most small (99% < 20mm)
- many have mobile stalks
- may be single or multiple
- benign but can cause embolization
•Rhabdomyomas 7%
•Others 5%

Question 2

Primary tumors — malignant 25%

Answer 2

•Sarcomas 95%
-Angiosarcoma 30% (most common primary malignant)
— middle aged men
— predilection for RA
— prognosis very poor
-Rhabdomyosarcoma 10%
— most common cardiac malignancy in infants / children
— usually occurs in ventricular wall
-Lymphomas 5%

Question 3

Secondary tumors (metastatic)

Answer 3

• 20 x’s more common than primary cardiac tumors
•most common primary sources:
-lung
-breast
-melanoma
— uncommon
— involves the heart in 50%
— usual manifestation is sub clinical ‘charcoal’ heart where tumor is on pericardial surface
-leukemia / lymphoma
•often involves pericardium and epicardium
•presence of pericardial effusion in a patient with known malignancy shoulder alert

Question 4

Carcinoid heart disease

Answer 4

•neuroendocrine tumor — secretes serotonin & other vasoactive substances
•arises from GI tract
• > 50% develop carcinoid heart disease
•generally occurs in hepatic mets where large amounts of serotonin & vasoactive hormones can reach the heart before inactivated by MAO in lungs — typically effects right heart
•diffuse collections of thick, pearly white plaques composed of myofibroblasts
•can involve endocardium, valve cusps, leaflets, chambers
•leaflets appear thickened & shortened and progress to retracted & hypo-mobile
•TV regurgitation&raquo_space; stenosis
•PV regurgitation + stenosis (PS gradient > 10 mmHg indication for surgery)
•left side involvement with : intracardiac shunts, extensive primary bronchial carcinoid, when high tumore secretions or poorly controlled disease overwhelm capacity of lungs
•surgical procedures:
- TV replacement operation of choice — repair not an option
- if PV involved then combined TV + PV operation
- balloon valvuloplasty not recommended
- mechanical (thrombus) vs bio prosthetic (premature degeneration)

Question 5

Ebstein’s Anomaly

Answer 5

• < 1% of call CHD
•may be seen in adults w/o previous surgical intervention
•malformation of the TV and RV
•caused by failure of delamination
•septal leaflet of TV apically displaced ( > 8 mm/m^2 BSA )
•anterior TV leaflet redundant with fenestrations and ‘sail-like’
•significant TR
•atrialization of the RV especially on septal side
•associated findings:
- atrial level shunts (ASDs) 80 - 94%
- other congenital anomalies
- LV non-compaction
- RA dilation
- RV dysplasia
- paradoxical systolic expansion of the atrialized RV
- RV volume overload
•surgical treatment
- observation if asymptomatic
- TV repair when feasible
- long term data on repair vs replace unavailable

Question 6

Cor Triatriatum

Answer 6

•rare congenital anomaly
•failure of resorption of common pulmonary veins during embryogenesis
- fibromuscular membrane divides the LA into two chambers
- communication via perforation in the membrane
•sinister > dexter
•key TEE points:
- define anatomy of LA membrane
- assess flow obstruction with CFD
- evaluate pulmonary venous and atrial septal anatomy
- look for associated anomalies : ASD, MR, PLSVC
•primary concern for LV inlet obstruction — MS physiology
•pulmonary venous inflow can be compromised (rare)
•incidental findings : dilemma because surgical excision exposed patient to open-chamber procedure

Question 7

Thrombus

Answer 7

•Virchow’s Triad : stasis, endothelial dysfunction, hypercoagulable state
•appearance varies according to underlying pathology
•serpentine, irregular, mobile — may appear free floating
•mural thrombus : laminated, non homogeneous, immobile
•LV thrombus
- located in regions of abnormal wall motion and regions of stasis
- often more echogenic than underlying myocardium
- TTE > TEE
•LA thrombus
- highest incidence in patients with MS and A-fib
- spontaneous echo contrast

Question 8

Chiari network

Answer 8

• filamentous strands attached to eustachian valve
• often clinically insignificant
• can be involved in pathogens of thromboembolic disease, arrhythmia, and entrapment of percutaneous devices
• associated with PFO and atrial septal aneurysm

Question 9

LV band / false tendon

Answer 9

• normal finding
• fibrous muscular bands stretching across the LV from ventricular septum to papillary muscle or LV free wall
• no connections with valve cusps
• single or multiple
• may be involved in producing innocent murmurs, cardiac rhythm disorders, or LV dysfunction