Connie Tsao Non-invasive Conference April 7, 2010

Outline  Non-tumors Normal Variants Catheters Thrombotic disease Infective endocarditis  Cardiac tumors Epidemiology Clinical Manifestations Primary Cardiac Tumors ○ Benign ○ Malignant Metastatic Tumors

Non-tumors

Normal Variants  Structural variants False tendon: fibrous/fibromuscular Eustachian valve Chiari network  Prosthetic material Catheters Pacing wires Cardiac assist devices

Arrhythmogenic potential?  Series of 15 patients with idiopathic LV tachycardia vs. controls referred for echo  All ILVT had false tendon from IL wall- septum 2/3 of these >2 mm  34/671 (5%) of controls had false tendon Oriented across LV <2 mm Thakur RK, Circ 1996

Epidemiology  In FHS Original and Offspring cohort: 101 participants with LV false tendons (2% of population) Kenchaiah S et al, JASE 2009

 Associated with: Lower BMI Innocent murmur ECG-LVH  Not associated with ventricular ectopy, or other ECG abnormalities  No excess mortality in 7.7±1.6 yrs follow-up Kenchaiah S et al, JASE 2009

Eustachian valve

Persistent Eustachian valve  Case reports of association between Eustachian valve and PFO  In 306 pts referred for TEE (211 for cryptogenic CVA): 143/211 (68%) of cryptogenic stroke group had EV 31/95 (33%) of controls had EV 70% of pts with EV had PFO  ? Effect of flow on increasing patency of PFO Strotmann JM, Heart 2001 Schuchlenz HW, JASE 2004

Chiari Network  Hans Chiari, 1897: 11 pts, fibrous network in RA  Remnant of right valve of sinus venosus Directed IVC flow through fossa ovalis to LA Incomplete resorption  1-4% in autopsy studies

Chiari network and PFO  1436 pts consecutive pts referred for TEE  Prevalence 29/1436 (2%)  Chiari network present in: 24/522 (4.6%) referred for paradoxical embolus 5/913 (0.5%) controls  PFO present in: 24/29 (83%) with Chiari 44/160 (28%) controls  Significant R-L shunt by agitated saline in 1/3 with Chiari Schneider B, et al, JACC 1995

Prosthetic Material

Impella

Intracardiac Thrombi  Accounts for 15-20% strokes Major source: LA thrombi (>45% cases) ○ LA thrombi detected by TEE: Acute AF: 14% Chronic AF: 27% AF with clinical thromboembolism: 43% Other: Aorta, valve prostheses, inter-atrial septum aneurysm  LV thrombi Post-MI Significant LV dysfunction Stoddard MF et al, JACC 1995; Manning WJ et al, Ann Int Med 1995

LAA masses

LV Thrombus

Same patient, LGE

LV Thrombus: Value of LGE-CMR  784 consecutive pts with LVEF <50%  Thrombus detection: 37 (4.7%) by cine-CMR 55 (7%) by LGE-CMR  Pathologic correlation in 8 pts, LV thrombus in 5 All 5 detected by LGE-CMR 2 detected by cine-CMR  Cine CMR missed small intracavity and mural thrombi Weinsaft JW et al, JACC 2008

LV Thrombus: Contrast Echo vs CMR  121 pts post MI or clinical heart failure  TTE, contrast-TTE, LGE-CMR  LV thrombus in 24 pts by LGE-CMR Larger infarcts, aneurysm, lower LVEF  TTE sensitivity 33%, Contrast TTE: 61% Low LVEF predictor of thrombus detection by CMR  Thrombi detected by DE-CMR vs contrast echo: mural, small apical  Close agreement with contrast echo (k=0.79) Weinsaft JW et al, JACC Imaging 2009

Asymptomatic 50 year old man SSFPFirst pass perfusion Hoey ED et al, Clin Radiol 2009

Cardiac Tumors

 Majority (>75%) are benign  Rare; incidence of < % in autopsy studies Primary cardiac tumors

Primary Benign Tumors

Braunwald’s Heart Disease, 7 th Ed.

Classic Triad of Symptoms  Intracardiac obstruction: Dyspnea, orthopnea, pulmonary edema Presyncope/syncope Angina, claudication  Systemic embolization: CVA, retinal artery emboli Emboli to extremities  Constitutional symptoms: fever, fatigue, weight loss, arthalgia

Myxoma  Mean age 50 years at diagnosis  F>M (60-70%)  80% in left atrium, 15% in right atrium Can occur in ventricles  90% solitary, 7% Carney complex  Average size 5-6 cm  Attachment to fossa ovalis

 Pedunculated, gelatinous  Friable/villous surface (1/3)  emboli  Histology: Mesenchymal cells in mucopolysaccharide stroma  Production of VEGF  angiogenesis

Clinical manifestations  Factors: size, anatomic location  Pulmonary venous or mitral valve obstruction  Stroke/neurologic deficits  Systemic embolization  Constitutional symptoms: fever, weight loss Anemia, elevated ESR, leukocytosis ↑IL-6, inflammatory factors

Imaging  Echo Prolapsing mass across MV/TV Identification of point of attachment  CMR Heterogeneous appearance on T1W, T2W images Patchy LGE  CT Low attenuation mass, no enhancement Calcification in 10-15%

T1W post gadolinium

T2W

58 year old man with dyspnea

Treatment  Resection Including surrounding septum at attachment  Surgical mortality <5%  Risk for atrial arrhythmias  Recurrence in 2-5%  Recurrence in Carney complex 12-22%

Papillary Fibroelastoma  Incidence % in autopsies  Mean age 60 years  Mean size 9 mm (2-70 mm)  80-90% on valvular endocardium, AV 36%> MV 29%> TV 11% > PV 7% Downstream side  Histology: fibromyxoid core, rim of elastic fibers covered by endothelial cells Distinction from Lambl’s excrescence

Clinical manifestations  Embolization: tumor or thrombus CVA/TIA PE Peripheral embolization  MI, angina  Sudden cardiac death  Syncope  1/3 of patients asymptomatic

Imaging  TTE can miss due to size  CMR not ideal due to high mobility Well-circumscribed nodule on T1W, T2W LGE reported  Distinction from vegetation No significant valvular regurgitation Location away from valvular free edge

29 year old woman with incidentally discovered mass… Parthenakis F et al, Cardiovasc Ultrasound 2009

Treatment  Observation: small, nonmobile tumors  Surgical resection: Any embolic events Highly mobile >1 cm  No recurrences known Sun JP et al, Circ 2001

Lipoma  Slow-growing  Mature adipose tissue  Sub-endocardial (50%) Broad based attachment Growth into adjacent chambers  Myocardial (25%)  Sub-epicardial (25%) Narrow attachment point Growth into pericardial space  Valvular attachment rare  Lipomatous hypertrophy of IAS Older, obese Associated with CAD (Chaowalit N et al, Chest 2007)

Clinical manifestations/Treatment  Most asymptomatic Invasion into tissue  arrhythmias, conduction block ↑size  obstruction  Resection recommended (continued growth) Lipomatous hypertrophy of IAS: no resection unless significant clinical sxs

Imaging  Echo: variable appearance Spares fossa ovalis  CMR + CT: corresponds to fat signal  CMR Bright on T1W + T2W images Uniform suppression by fat sat No soft tissue component/ LGE  CT Homogenous fat attenuation

Lipoma Leu HB et al, Eur Heart J 2004

35 yo woman with AF, mass on TTE T2W BBT1W BB Lack of LGE T2W fat sat Hoey ED et al, Clin Radiol 2009

Rhabdomyoma  Most common primary cardiac tumor in children Most <1 year of age  80-90% association with tuberous sclerosis  Most regress spontaneously  Arrhythmias Heart block, VT

Fibroma  2 nd most common pediatric cardiac tumor  Fibroblasts interwoven with collagen  Arise in myocardial free wall/septum  LV:RV 5:1  Heart failure: obstruction, valvular dysfunction

Fibroma- Imaging  CMR: Low signal on T1W, T2W Hypovascular on 1 st pass perfusion Homogeneous on LGE  CT Mildly enhancing Up to 50% calcification

32 yo F with recurrent syncope, VT Hoey ED et al, Clin Radiol 2009 T1W BB T2W BB SSFP LGE

Primary Malignant Tumors

Braunwald’s Heart Disease, 7 th Ed.

Overview  Overall 15% of primary cardiac tumors  Sarcomas most common Angiosarcoma Sarcomas with myo- or fibroblastic differentiation Rhabdomyosarcoma  Suggestive imaging findings: Right-sided Broad-based attachment Ill-defined margins Tissue inhomogeneity/ heterogeneous contrast enhancement Size >5 cm Pericardial effusion

Angiosarcoma  Highly aggressive, anaplastic epithelial cells, vascular channels  M>F, peak incidence in 40s  RA involved in 75%  RV, pericardium  Clinical symptoms Right heart failure Tamponade  Metastases in 66-89%  lungs/brain/bone/liver

Imaging  CMR T1 isointense, T2 hyperintense Flow voids = vascular channels Prominent LGE “sunray appearance”  CT Low attenuation/ irregular Heterogenous enhancement

25 year old woman with dyspnea T1W BBT2W, fat suppression Hoey ED et al, Clin Radiol 2009

O’Donnell DH et al, Am J Roentol 2009 T1W BBLGE

63 year old man with chest pain

Treatment  Resection + chemotherapy ↑ survival with complete resection  Transplantation Sarcoma in 15/21 malignancies Mean survival 12 months 7 patients with mean survival 27 mos Gowdamarajan A et al, Curr Opin Cardiol 2000;

 Autotransplantation 8 sarcomas resected ○ 7 atrial, 1 ventricular Median survival 18.5 mos Reardon MJ et al, Ann Thorac Surg 1999, 2006

Lymphoma  Majority aggressive B-cell lymphomas Commonly in immunocompromised  Disseminated non-Hodgkin’s lymphoma more common  Firm, nodular aggregates of lymphoid tissue  Mean age 38 years  Treatment: anthracyclines, monoclonal anti-CD20 antibody

Imaging  Echo characteristic features: RA, pericardial effusion  CMR Isointense on T1W, or hyperintense on T2W Heterogeneous enhancement on LGE  CT Isointense relative to myocardium

T1W LGE T1W T2W LGE

54 yo F with CP, DOE, palpitations

Metastatic Tumors

Overview  Up to 12% of oncology pts at autopsy Most clinically silent  Most common: lung cancer, melanoma  Pericardial effusion common  Multiple masses suggestive  Imaging characteristics Hypointense on T1W (except melanoma: paramagnetic effect of melanin) Hyperintense on T2W Enhancement after gadolinium administration Soft tissue attenuation on CT

Primary MalignancyCardiac Effect LungDirect extension, effusion BreastHematogenous/lymphatic spread, effusion LymphomaLymphatic spread, variable effects GIVariable MelanomaIntracardiac and myocardial Involvement Renal Cell CarcinomaIVC-RA-RV extension, can look like thrombus CarcinoidTricuspid and pulmonic valve abnormalities Braunwald’s Heart Disease, 7 th Ed.

Melanoma

Direct Extension Tumors

Lung cancer

Hepatocellular carcinoma

Renal Cell Carcinoma

Braunwald’s Heart Disease, 7 th Ed.

Summary  Many conditions mimic cardiac masses  Primary cardiac tumors are rare and usually benign  Clinical presentation varies by location and size of mass  TTE and CMR with gadolinium helpful to narrow differential diagnoses  Treatment: surgical resection for bulky tumors/ chemotherapy