Ultrasound of the Week #040


Case:

A 32 year old male dialysis patient presented to ED following a near-syncopal episode at home, 6 hours after being dialysed.  In the department he reported feeling ‘back to normal’, had no other symptoms and his haemodynamics were within normal range.

He had a bedside echo in the ED with concern he had been aggressively dialysed and would be hypovolaemic.

Question: What are these views?  What do they show?  What abnormalities can you identify?  Is there anything that could have contributed to his presenting complaint?

Answer and Explanation:

The views demonstrated are the Parasternal Long Axis (PLAX), Parasternal Short Axis (PSAX), Apical 4 Chamber (A4Ch), Subcostal (SC) and Subcostal (SC IVC).

There is a lot going on here!

  • PLAX: The LV walls appear grossly thickened (LV Hypertrophy) with a small LV cavity.  The LV function appears visually normal.  There is a notable pericardial effusion. 
  • PSAX:  The LV contraction appears good and there are no obvious RWMAs.  The MV opens well.  The RV free wall appears thickened (RV Hypertrophy.)
  • A4Ch:  The pericardial effusion is very clear, and there is some collapse of the RA free wall. It is unclear whether this is during systole or diastole (as no ECG leads were attached). 
  • SC: Again the 4 chambers can be assessed and the above findings noted.  The pericardial effusion and RA free wall collapse is again evident.
  • SC IVC:  The IVC is dilated with little to no variation throughout the respiratory cycle.

In all views, the Myocardium appears quite bright, ‘granular’ or ‘sparkling’ in appearance.  This is characteristic of cardiac amyloidosis (see below).

Summary:
LVH with a small LV cavity and visually normal LV function.
Granular/’sparkiling’ myocardium
RVH with visually normal longitudinal function
Large pericardial effusion
Plethoric IVC

Conclusion: Large pericardial effusion with RA free wall collapse raising suspicion of pericardial tamponade.  ‘Granular’ myocardium suggestive of cardiac amyloidosis.


Pericardial Tamponade:

For an explanation of the basics of focussed echo in cardiac tamponade and the physiology behind this, see Ultrasound of the Week #024. Remember, the key is that the presence of pericardial fluid is an “anatomical diagnosis” whilst cardiac tamponade is a “physiological diagnosis” which can only really be proven retrospectively after an observed improvement in physiological parameters following pericardial decompression. 

Given the presentation and echo findings, this highlighted the suspicion of tamponade and this gentleman was admitted under the renal team and seen by cardiology for a formal echocardiogram and consideration of pericardiocentesis.


Cardiac Amyloidosis:

Knowledge of the echocardiographic features of cardiac amyloidosis and diagnosis are outside the scope of this case and not generally pertinent to Emergency Physicians.  However, some of the more obvious visual findings are listed below for interest and are well demonstrated in this case[1,2,3]:

  • LV, IVS and RV thickening
  • ‘Granular’ or ‘sparkling’ appearance of the myocardium secondary to amyloid deposits
  • Normal or reduced LVIDd
  • Pericardial effusion (usually small)

References:

  1. Siqueira-Filho AG, Cunha CL, Tajik AJ, Seward JB, Schattenberg TT, Giuliani ER (1981). “M-mode and two-dimensional echocardiographic features in cardiac amyloidosis”. Circulation. 63 (1): 188–96. PMID 7438392.
  2. Nishikawa H, Nishiyama S, Nishimura S; et al. (1988). “Echocardiographic findings in nine patients with cardiac amyloidosis: their correlation with necropsy findings”. Journal of Cardiology. 18 (1): 121–33. PMID 3221306.
  3. Hamer JP, Janssen S, van Rijswijk MH, Lie KI (1992). “Amyloid cardiomyopathy in systemic non-hereditary amyloidosis. Clinical, echocardiographic and electrocardiographic findings in 30 patients with AA and 24 patients with AL amyloidosis”. European Heart Journal. 13 (5): 623–7. PMID 1618203.

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