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going the wrong way…

the case.

56 year old male presents to ED with dyspnoea, cough and small-volume haemoptysis.

He has had gradually worsening orthopnoea & reduced exercise tolerance over the past two weeks which is associated with a non-productive cough. Three hours ago however, he became acutely unwell with the development of sharp, left-sided chest pain.

On examination he is febrile, clammy & diaphoretic. He is tachycardic [120/min] & hypotensive [84/50mmHg] with a pan-systolic murmur at his apex. Auscultation of his chest reveals bilateral [right>>left] coarse crackles.

[DDET What are your differential diagnoses?]

  • Sepsis.
    • Pneumonia
    • Infective endocarditis
    • other…
  • Pulmonary embolism
  • Cardiogenic shock
    • AMI
    • Mitral regurgitation
    • Pericardial effusion
  • Aortic dissection

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[DDET This is his CXR…]

CXR - admission
Predominate right sided infiltrates, particularly perihilar & right-upper lobe.

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[DDET How are you going to put this together?]

You take your trust ultrasound to the bedside, keeping in mind the FALLS protocol…

Screening ECHO:

PLAX, PSAX + subcostal views: LV appears hyperdynamic (even a bit empty) with reasonable contractility.  

Lung Ultrasound:

Lung ultrasound demonstrating numerous B-lines. These were asymmetric (R>>L) and more marked in right upper lobe; suggestive of pulmonary oedema.

Focused Apical Four Chamber:

So…. What are you seeing ?!?!

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[DDET What did we see ?]

In the A4C view the posterior mitral leaflet is seen to be prolapsing into the left atrium.

acute mitral regurgitation
A4C image demonstrating mitral valve prolapse with the posterior mitral leaflet billowing into the left-atrium.

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[DDET The diagnosis…]

Acute mitral regurgitation.

A true surgical emergency, however accurate & timely diagnosis can be difficult.

CAUSES.

  • Chordae or papillary muscle rupture
  • Endocarditis with valve destruction
  • Myocardial ischaemia
  • Acute rheumatic fever with carditis
  • Acute cardiomyopathy
  • Annular dilation with poor coaptation of leaflets
  • Prosthetic valve dysfunction.

CLINICALLY.

The majority of patients with acute mitral regurgitation will present with dyspnoea, haemodynamic instability & symptoms of shock [weakness, dizziness & altered mental state]. Symptoms may also reflect the underlying pathogenesis of the acute regurgitation, however most patients will have no prior history of cardiac disease.

A subset of patients with acute mitral regurgitation may present solely with new-onset dyspnoea & may be diagnosed as a non-cardiac respiratory failure.

The murmur of acute MR is often only a faint systolic murmur, rather than the classic holosystolic heard in chronic MR. This is a result of the rapid equilibration of ventricular & atrial pressures during systole.

ACUTE VS CHRONIC SEVERE REGURGITATION.

Acute vs Chronic MR, acute mitral regurgitation
Comparison of findings in acute & chronic mitral regurgitation. Adapted from Stout & Verrier (3).

CXR findings in Mitral Regurgitation.

  • Typically demonstrates a normal-sized heart with features of pulmonary oedema.
  • Interestingly, [and rarely] acute MR may direct regurgitant flow into a single pulmonary vein resulting in oedema most prominently in that lung segment. This is often [& easily] confused with pneumonia !
acute mitral regurgitation
Unilateral pulmonary oedema. A result of a lateralising MR jet regurgitating into a single pulmonary view. Image courtesy of Attias et al.

ECHO findings in Acute MR.

  • Normal LV size & function
  • Vena contracta >7 mm
  • Decreased aortic valve opening
  • Disrupted mitral valve apparatus
  • Systolic reversal of pulmonary vein flow
  • Vegetation &/or perforation
  • Wall motion abnormalities [ischaemia]

MANAGEMENT.

Surgical = urgent valvuloplasty !!

Medical = support patient prior to valvuloplasty !!

  • Respiratory support:
    • BiPAP
    • Intubation & mechanical ventilation.
  • Haemodynamic support:
    • Cautious fluid administration
    • Vasopressors
    • Consideration for intra-aortic balloon pump counterpulsation.
  • Consider underlying causes especially acute coronary syndrome.
    • ?angiography ± stenting prior to OT.

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[DDET The outcome]

With his haemodynamics heading in the wrong direction, our patient is admitted to Intensive Care for ongoing vasopressor support and intermittent bursts of non-invasive ventilation.

Formal ECHO:
” Severe prolapse of posterior mitral leaflet. Normal LV size and systolic function”.

Taken to theatre on Day 10 of admission for mitral valve annuloplasty repair. Ruptured chordae found and repaired.

Here is his post-operative CXR….

CXR - postop
Post-op mitral valve repair. Note the sub-diaphragmatic gas, a result of an accidental intraoperative diaphragmatic injury.

He continues to do well.

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[DDET References]

  1. Boehmeke, T., & Doliva, R. (2006). Pocket Atlas of Echocardiography. Clinical sciences. Thieme.
  2. Rosenʼs Emergency Medicine. Concepts and Clinical Approach. 7th Edition
  3. Stout, K. K., & Verrier, E. D. (2009). Acute valvular regurgitation. Circulation, 119(25), 3232–3241. doi:10.1161/CIRCULATIONAHA.108.782292
  4. Mokadam, N. A., Stout, K. K., & Verrier, E. D. (2011). Management of acute regurgitation in left-sided cardiac valves. Texas Heart Institute journal / from the Texas Heart Institute of St. Luke“s Episcopal Hospital, Texas Children”s Hospital, 38(1), 9–19.
  5. Reynolds, H. R., & Hochman, J. S. (2008). Cardiogenic Shock: Current Concepts and Improving Outcomes. Circulation, 117(5), 686–697. doi:10.1161/CIRCULATIONAHA.106.613596
  6. Hanson, E. W., Neerhut, R. K., & Lynch, C. (1996). Mitral valve prolapse. Anesthesiology, 85(1), 178–195.
  7. Sutton, M. S. J. (2002). Mitral Valve Prolapse Prevalence and Complications: An Ongoing Dialogue. Circulation, 106(11), 1305–1307. doi:10.1161/01.CIR.0000031759.92250.F3
  8. Gilbert, B. W., Schatz, R. A., VonRamm, O. T., Behar, V. S., & Kisslo, J. A. (1976). Mitral valve prolapse. Two-dimensional echocardiographic and angiographic correlation. Circulation, 54(5), 716–723. doi:10.1161/01.CIR.54.5.716
  9. Raman, S., & Pipavath, S. (2009). Images in clinical medicine. Asymmetric edema of the upper lung due to mitral valvular dysfunction. The New England journal of medicine, 361(5), e6. doi:10.1056/NEJMicm0801147
  10. Attias, D., Mansencal, N., Auvert, B., Vieillard-Baron, A., Delos, A., Lacombe, P., et al. (2010). Prevalence, Characteristics, and Outcomes of Patients Presenting With Cardiogenic Unilateral Pulmonary Edema. Circulation, 122(11), 1109–1115. doi:10.1161/CIRCULATIONAHA.109.934950
  11. Roach, J. M., Stajduhar, K. C., & Torrington, K. G. (1993). Right upper lobe pulmonary edema caused by acute mitral regurgitation. Diagnosis by transesophageal echocardiography. Chest, 103(4), 1286–1288.
  12. Sudden Cardiogenic Shock – Dr Smith’s ECG Blog. April 2014.

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