case presentation: 47 year-old male with sudden weakness,

62
Case Presentation Federico Viganego, M.D. 1/19/07

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Page 1: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case Presentation

Federico Viganego, M.D.

1/19/07

Page 2: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 1-H.H.

• 61 y.o. M recently diagnosed with a right renal mass

• Presented to the hospital with progressive dyspnea and worsening renal function

• A 2D echo is ordered to evaluate the cause of dyspnea..

Page 3: Case Presentation: 47 Year-old Male with Sudden Weakness,

Echocardiogram

Page 4: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 1-H.H. 2D ECHO

• Image 38

• Image 42

• Images 60-66

• Image 69-79

Page 5: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 1-H.H.

• A mass is visualized in the R atrium. The mass is mobile, originates from the IVC, and is compatible with thrombus.

• The RA mass measures 14 x 7 mm

• A MRI of the abdomen is performed..

Page 6: Case Presentation: 47 Year-old Male with Sudden Weakness,

MRI

Page 7: Case Presentation: 47 Year-old Male with Sudden Weakness,

MRI

Page 8: Case Presentation: 47 Year-old Male with Sudden Weakness,

MRI

Page 9: Case Presentation: 47 Year-old Male with Sudden Weakness,

MRI

Page 10: Case Presentation: 47 Year-old Male with Sudden Weakness,

MRI

Page 11: Case Presentation: 47 Year-old Male with Sudden Weakness,

MRI

Page 12: Case Presentation: 47 Year-old Male with Sudden Weakness,

MRI-CORONAL VIEW

Page 13: Case Presentation: 47 Year-old Male with Sudden Weakness,

MRI-SAGITTAL VIEW

Page 14: Case Presentation: 47 Year-old Male with Sudden Weakness,

MRI-SAGITTAL VIEW

Page 15: Case Presentation: 47 Year-old Male with Sudden Weakness,

MRI-SAGITTAL VIEW

Page 16: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 1-H.H.

• A large mass is identified in the right kidney

• A large thrombus is seen in the R renal vein and within the infra-renal IVC extending to the level of iliac bifurcation

• Thrombus is also seen within the IVC extending superiorly to the atrio-caval junction

Page 17: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 1-H.H.

• Pt undergoes embolization of the R renal artery on 12/22 and a repeat embolization of residual renal artery on 1/3

• On 1/11 pt underwent R radical nephrectomy, supradaphragmatic IVC thrombectomy, and splenectomy

Page 18: Case Presentation: 47 Year-old Male with Sudden Weakness,

Cardiac Masses

• Abnormal structure within or immediately adjacent to the heart

Three types of cardiac masses:

• Tumor

• Thrombus

• Vegetation

Page 19: Case Presentation: 47 Year-old Male with Sudden Weakness,

Cardiac Mass-Echocardiography

• Pro: • Can provide both anatomic and physiologic

information about the mass • Noninvasive, relatively inexpensive• Serial studies are feasible• Cons: • Suboptimal image quality in some patients• Relatively narrow field of view (vs.CT/MRI)• Mass versus ultrasound artifact• Mass versus normal structures

Page 20: Case Presentation: 47 Year-old Male with Sudden Weakness,

Diagnosis of Intracardiac Mass

• Excellent image quality

• Identification in more than one acoustic window

• Knowledge of normal structures, normal variants and post-op changes

• Integration of other echo findings (i.e., rheumatic MS and LA thrombus)

• Clinical data

Page 21: Case Presentation: 47 Year-old Male with Sudden Weakness,

Cardiac mass vs. normal structuresRight Atrium

• Crista terminalis

• Chiari network (Eustachian valve remnants)

• Lipomatous hypertrophy of the interatrial septum

• Trabeculation of the RAA

• Atrial suture line (transplant)

• Pacer wire, Swan-Ganz catheter, CVC, etc

Page 22: Case Presentation: 47 Year-old Male with Sudden Weakness,

Eustachian valve remnants

• Persistent portions of embryologic valves of sinus venosus

• Junction of IVC/SVC with RA

• Typically mobile

• May be extensive → Chiari network

• Do not extend to cross the tricuspid valve

Page 23: Case Presentation: 47 Year-old Male with Sudden Weakness,

Chiari Network

Page 24: Case Presentation: 47 Year-old Male with Sudden Weakness,

Pacer Wire

Page 25: Case Presentation: 47 Year-old Male with Sudden Weakness,

Right-sided thrombi

• Rarely form in situ

• Most commonly embolized from venous source

• May be ‘entrapped’ in TV or RV structures

• Indwelling catheters or pacer wires

• Better characterized with TEE

• If mobile, differential include Eustachian valve remnants

Page 26: Case Presentation: 47 Year-old Male with Sudden Weakness,

Risk of embolization

Higher: • Irregular shape• Protruding in the

cavity• Mobile• Seen in multiple

projections

Lower:• Flat• Immobile• Seen in single

projections

Page 27: Case Presentation: 47 Year-old Male with Sudden Weakness,

Distingushing intracardiac massesCharacteristic Thrombus Tumor Vegetation

Location LA (esp. if enlarged or MV disease)

LA (myxoma)

Myocardium

Pericardium

Valves

Usually valvular

Occasionally on ventricular wall or Chiari network

LV (↓EF or segmental wma)

Appearance Usually discrete and somewhat spherical or laminated against LV apex or LA wall

Various: may be circumscribed or irregular

Irregular shape, attached to prox side (upstream) of the valve with motion independent from the valve

Associated findings Underlying etiology usually evident

Intracardiac obstruction depending on site of tumor

Valvular regurgitation usually present

LV systolic dysfn or segmental wma

Fever, systemic signs of endocarditis, positive blood cultures

MV disease with LAE

Page 28: Case Presentation: 47 Year-old Male with Sudden Weakness,

Cardiac mass vs. normal structuresLeft Atrium

• Dilated coronary sinus (persistent L superior vena cava)

• Raphe between L superior pulmonary vein and LAA

• Atrial suture line (transplant)

• Beam-width artifact from calcified aortic valve, AV prosthesis, etc.

• Interatrial septal aneurysm

Page 29: Case Presentation: 47 Year-old Male with Sudden Weakness,

Cardiac mass vs. normal structuresLeft Ventricle

• Papillary muscles

• Left ventricular web (aberrant chordae)

• Prominent apical trabeculations

• Prominent mitral annular calcification

Page 30: Case Presentation: 47 Year-old Male with Sudden Weakness,

Cardiac mass vs. normal structures

Right Ventricle:• Moderator band• Papillary muscles• Swan-Ganz catheter

or pacer wire

Aortic Valve:• Nodules of Arantius• Lambl’s excrescenses• Base of valve leaflet

seen en face in diastole

Page 31: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 2-D.J.

• 74 y.o. F presents with progressively worsening dyspnea and bilateral pleural effusions. Recently hospitalised for repeated syncopal episodes.

• PMH: HTN, CAD, TIAs, Rheumatoid arthritis

• MEDS: Methotrexate, Toprol, HCTZ, Lisinopril

• SOCIAL: 40 pack-year tobacco hx

Page 32: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 2-D.J. Echo

• Image 10

• Image 15

• Images 39-40 and 44

• Images 45-51

• Image 58

Page 33: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 2-D.J. 2D Echo

• Normal LV with normal to hyperdynamic systolic function

• Mild LVH• Severe MAC. Moderate MS (MV area by

pressure half-time=1.5 cm2, mean gradient=11 mmHg)

• Calcified aortic valve with moderate AS by continuity equation (AVA 1.4 cm2)

• Mild to moderate TR. Severe PHTN

Page 34: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 2-D.J. TEE

Page 35: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 2-D.J. TEE

Page 36: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 2-D.J. cath

Page 37: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 2-D.J. cath

• RH cath: RA pressures 8 mmHg, RV 31/6, PAP 57/25,

• Simultaneous pressures: PCWP 24 mmHg, LVEDP=8-11 mmHg, trans-mitral valve gradient 13-16 mmHg

• CO=2.67 L/min (thermodilution)• CI= 1.75 L/min/m2• Coronary angio: LAD 60-70% mid, LCX minor

lum irreg, RCA 50-60% prox-mid• LV-gram= normal LV filling and LV fn, MR2+• Normal ascending aortogram

Page 38: Case Presentation: 47 Year-old Male with Sudden Weakness,

CASE 2-D.J.-2D ECHO recent

Page 39: Case Presentation: 47 Year-old Male with Sudden Weakness,

Mitral StenosisRheumatic mitral stenosis.

There are severe valvular changes, including marked fibrosis and calcification of the mitral valve leaflets and severe chordal thickening and fusion into pillars of fibrous tissue.

(From Becker AE, Anderson RH [eds]: Cardiac Pathology: An Integrated Text and Colour Atlas. New York, Raven Press, 1983, p 4.3.)

Page 40: Case Presentation: 47 Year-old Male with Sudden Weakness,

Hemodynamics

• Schematic representation of left ventricular (LV), aortic, and left atrial (LA) pressures, showing normal relationships and alterations with mild and severe mitral stenosis (MS). Corresponding classic auscultatory signs of MS are shown at the bottom. Compared with mild MS, with severe MS the higher left atrial v wave causes earlier pressure crossover and earlier mitral valve (MV) opening, leading to a shorter time interval between aortic valve (AV) closure and the opening snap (OS). The higher left atrial end-diastolic pressure with severe MS also results in later closure of the mitral valve. With severe MS, the diastolic rumble becomes longer and there is accentuation of the pulmonic component (P2) of the second heart sound (S2) in relation to the aortic component (A2).

Page 41: Case Presentation: 47 Year-old Male with Sudden Weakness,

Classification of severity of MS

Mitral stenosis

Mild Moderate Severe

Mean Gradient* (mmHg)

<5 5-10 >10

Pulmonary artery systolic pressure (mmHg)

<30 30-50 >50

Valve area (cm2) >1.5 1.0-1.5 <1.0

*valve gradients are flow dependent and when used to assess severity of valve stenosis should be assessed with knowledge of cardiac output or forward flow across the valve.

Page 42: Case Presentation: 47 Year-old Male with Sudden Weakness,

Natural history of MSNatural history of 159 patients

with isolated mitral stenosis (solid blue line) or mitral regurgitation (solid purple line) who were not operated on (even though the operation was indicated) compared with patients treated with valve replacement for mitral stenosis (dashed blue line) or mitral regurgitation (dashed purple line). The expected survival rate in the absence of mitral valve disease is indicated by the upper curve (dashed black line).

(From Horstkotte D, Niehues R, Strauer BE: Pathomorphological aspects, aetiology, and natural history of acquired mitral valve stenosis. Eur Heart J 12[Suppl]:55-60, 1991.)

Page 43: Case Presentation: 47 Year-old Male with Sudden Weakness,

Echo in MS

Page 44: Case Presentation: 47 Year-old Male with Sudden Weakness,

Evaluation of MS by Echo

• Valve anatomy, mobility and calcification

• Mean trans-mitral pressure gradient

• 2D Echo mitral valve area (planimetry)

• Doppler pressure half-time area

• Pulmonary artery pressures (TR jet and IVC)

• Coexisting MR

Page 45: Case Presentation: 47 Year-old Male with Sudden Weakness,

MV Morphology by 2D EchoThe Wilkins Score

• Intended for predicting the likelihood of success of balloon valvulopasty

• Total valve score will be in the range of 0 to 16

• Scores ≤8 associated with an optimal outcome from percutaneous valvuloplasty

• Scores of ≥12 are associated with a poor outcome.

Page 46: Case Presentation: 47 Year-old Male with Sudden Weakness,

“Doming” of anterior leaflet

(From Bach DS: Rheumatic mitral stenosis. N Engl J Med 337:31, 1997.)

Page 47: Case Presentation: 47 Year-old Male with Sudden Weakness,

Mean trans-mitral pressure gradient by Doppler

• Simplified Bernoulli equation:

ΔP= 4 v2

• Measurement of the Velocity Time Integral of a continuous wave Doppler recording of the entire period of mitral inflow.

• Depends on transmitral flow rate

Page 48: Case Presentation: 47 Year-old Male with Sudden Weakness,

Pitfalls of Pressure Gradient

• Intercept angle between MS jet and ultrasound beam

• Beat-to-beat variability in AF

• Dependence on trans-mitral volume flow rate (i.e., exercise, MR, etc.)

Page 49: Case Presentation: 47 Year-old Male with Sudden Weakness,

2D Echo Mitral Valve Area

• Planimetry of short-axis of the MV orifice

• Tracing of the iner edge of the valve

• Validated by comparison with valve area at surgery

• Requires adequate image quality

Page 50: Case Presentation: 47 Year-old Male with Sudden Weakness,

Pitfalls of 2D Valve Area

• Image orientation

• Tomographic plane

• 2D gain settings

• Intra and inter-observer variability in planimetry of orifice

• Poor acoustic access

• Deformed valve anatomy after valvuloplasty

Page 51: Case Presentation: 47 Year-old Male with Sudden Weakness,

Doppler pressure half-time area

• The smaller the MV orifice, the slower the rate of pressure decline

• T1/2= time for the peak transmitral pressure to halve (in msecs)

• Empiric formula: MVA= 220/T1/2

• Affected by ventricular compliance and cardiac output

Page 52: Case Presentation: 47 Year-old Male with Sudden Weakness,

Pitfalls of Pressure Halftime

• Definition of Vmax and early diastolic deceleration slope

• Nonlinear early diastolic slope

• Sinus rhythm with a wave superimposed on early diastolic slope

• Influence of coexisting AI

• Changing LV and LA compliances esp. after commissurotomy

Page 53: Case Presentation: 47 Year-old Male with Sudden Weakness,

Common Echo Findings in MS

• Left atrial enlargement and thrombus

• Pulmonary hypertension

• Mitral regurgitation

• Coexisting valvular disease (aortic and/or tricuspid)

• Small LV, normal systolic function

Page 54: Case Presentation: 47 Year-old Male with Sudden Weakness,

FIN

Page 55: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 2-K.W.

• 43 y.o. M who is s/p closure of VSD at Ochsner Clinic at age 13

• Presented to clinic with increasing fatigue

• A 2D ECHO and a TEE are performed..

Page 56: Case Presentation: 47 Year-old Male with Sudden Weakness,

TEE

Page 57: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 2-K.W. cath

Page 58: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 3-P.S.

• 42 y.o. F with Ehlers-Danlos syndrome who is s/p closure of ASD at age 7

• More recently, she developed increasing dyspnea and fatigue

• She also has history of atrial fibrillation

• Patient undergoes TEE and cardiac catheterization

Page 59: Case Presentation: 47 Year-old Male with Sudden Weakness,

TEE

Page 60: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 3-P.S. LHC

Page 61: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 3-P.S.

• LHC: normal coronaries. AoP 110/65 LVEDP 22, LV-gram normal LV function, 3+MR

• RHC: No step-up of venous o2 saturation sugesting residual ASD. PCWP 6 PAP 22/7 RV 23/6

• CO thermodilution 5.63 L/min

• CI 2.76 L/min/m2

Page 62: Case Presentation: 47 Year-old Male with Sudden Weakness,

Case 3-P.S.

• Pt underwent mitral valve repair and modified maze procedure on 6/16 by Dr. Piggott

• At follow-up on 8/24, notable improvement of patient’s dyspnea on exertion is reported