by Dr Ben Land
A 34 year old female with a history of Congenital Lymphangiomatosis presented to the ED with progressive SOB on exertion over a few weeks. She had previously been walking to work and cycling at level 8 on an exercise bike, but now was SOB after walking only a few metres. She had generalised chest pain and subjective fevers. She then had a single episode of haemoptysis, and estimated around 100mls of frank blood. She became acutely SOB after this. On ambulance arrival she was distressed, pale and clammy with SATs of 50% on air, which improved with 15L 02 via a Hudson mask.
Background history of Lymphangiomatosis: a rare disease characterised by abnormal proliferation of the lymphatic system, leading to chylous effusions. She had a peritoneal venous shunt placed a few years ago. She had a history of recurrent PE’s, and was taking Apixaban. She also had a chronic right pleural effusion.
On ED arrival she appeared pale and unwell, with markedly increased work of breathing. She was only able to talk in interrupted sentences and had a RR of 38. Her pulse was 84, with BP 97/70 and temp was 37.7. Chest exam revealed dullness in the right base. Her ECG showed a right axis deviation.
Portable chest X-ray showing her known chronic right pleural effusion, and also left upper lobe opacities compatible with scarring. Her shunt is visible with the tip situated within the right atrium.
Here are some clips of her bedside echo:
Parasternal long axis showing reasonable LV function. At a glance you can see that the anterior leaflet of the mitral valve nearly touches the septum in systole, which is a marker of pretty good LV function. On this view the RV outflow tract is dilated. In this view RV should be 1:1:1 with aorta and LA.
Parasternal short axis view which again shows hyperdynamic LV function: also note the D shaped LV due to straightening of the IV septum. The right ventricle isn't seen perfectly here, but is moderately dilated (should be 0.6:1- RV:LV, but in this case appears at least 1:1 or more).
The subcostal views demonstrated a hypoechoic mass in the RA which is closely associated with the tip of her peritoneal venous shunt. The differential for this is thrombus or vegetation associated with the shunt or the tricuspid valve. However, atrial myxoma should also be considered.
Note the vigorous movement of the tricuspid valve annulus, which suggests preserved RV function despite dilatation. A TAPSE >16mm (A4C) would further define this as normal. The RV dilatation is likely acute in this case as the RV free wall is thin. Features of chronic RV dysfunction are thick RV free wall (subcostal >6mm), pronounced RV wall trabeculation, RVSP >60mmHg, PAT <90ms. Comparison with a previous echo would be helpful.
Here's an IVC view again demonstrating the right atrial thrombus:
You can also see the right sided pleural effusion containing some abnormal lung tissue with B-lines, it's in the bottom left part of the screen here. We could (and might) write a separate blog post about her lung US which also demonstrated some great signs!
From the bedside echo, we made a diagnosis of RA thrombus with likely acute pulmonary embolism causing acute RV dilatation.
This patient went on to have a CTPA which confirmed the presence of non-occlusive and occlusive segmental and subsegmental PE involving all lung segments. The finding of right atrial thrombus may increase the mortality in patients with PE(1), and is associated with increased haemodynamic instability. Treatment options are the same as any PE including anticoagulation or thrombolysis, but surgical thrombectomy may also be considered for large clots(2). Our patient was already taking Apixaban and after some initial treatment was stable in ED. Given her complex history she was transferred to her usual treating team for further management.
Many thanks to Amaali for providing expert insight and editorial review, and to Rachel who allowed me to do the ultrasound on her patient. Please send any questions, comments, suggestions or feedback to myself or Amaali.
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