Lydia has acute myocarditis caused by a viral infection and/or her autoimmune disorder (Crohn's disease). Maybe she even had giant-cell myocarditis - an autoimmune-mediated form of myocarditis marked by sudden and severe onset.

1. On the other side of the fence
Dec 27th, 2013 Bea Marzluf
Emergency Echo
The patient is the one with the disease
Doctors and nurses don’t get sick. Right? It’s the patient who falls ill. They are on the
receiving end of health care; we certainly are not. But what if we are on the other side
of the fence? What if we are the patient? I felt quite uncomfortable when I was called to
one of our intensive care units to perform an echo on Lydia, a 26-year old nurse. Why
would someone younger than I was require admission to an ICU? When I first saw her I
was shocked: Lydia was literally in multiple organ failure. She was hypotensive, and
required hemodialysis, high levels of oxygen insufflation, and volume support.
Critically ill
Lydia was diagnosed with Crohn's disease six years earlier. However, everything was
stable from thispoint of view. She told me that her "problem" had started with nausea,
vomiting, and breathing difficulties. She was brought to a local hospital and then
transferred to our ICU the next day for the treatment of acute kidney and liver failure.
One of the treatment options was acute liver transplantation! At this point, the cause of
her problem was still unclear. Would echo shed light on this mysterious case?
Four-chamber view on admission shows poor
left ventricular function.
Would you have suspected this? This is her short-axis view:

2. Parasternal short-axis view on admission:
poor left ventricular function and a pericardial effusion.
Her ejection fraction (EF) was only 31% and her left ventricular function was diffusely
reduced. The same was true of her right ventricular function. Does this mean that
Lydia's has acute heart failure? Is she in cardiogenic shock? To truly understand her
condition and determine whether this is acute or a chronic heart failure, we need to
obtain more information from the echo. First, look at the size of her ventricle. The enddiastolic diameter was 51 mm. The ventricle is just mildly dilated; it had not had time to
compensate. What about the Doppler spectrum of mitral inflow?

3. Diastolic mitral inflow pattern. E/A fusion, but the E wave
appears to be very high. Very steep deceleration.
The mitral inflow pattern shows fusion of the E/A wave due to tachycardia. It looks as if
the E wave is very high. A restrictive filling pattern is present. This denotes high filling
pressures. You don’t even need a stethoscope to know that she has pulmonary
congestion. She also had a very low stroke volume (35 ml) - no wonder her heart rate
was so high. It is a way of compensating for low stroke volume. All of these findings
confirm our suspicion: this is an acute - not a chronic - form of heart failure.
But why?
At this point it is very difficult to know why she developed acute heart failure. Let us go
through a checklist of possible causes:
Coronary artery disease? No. She is young, has no risk factors, and there are no
“regional wall motion abnormalities”.
Takutsubo? No. Lydia has no history of acute emotional stress, and the typical
pattern of apical ballooning is absent.
Toxic cardiomyopathy? No. She had no pertinent history and all her urine and
blood tests were negative.
Valvular heart disease? No. She did have “mild to moderate” functional mitral
regurgitation, but no structural abnormalities of the valves were seen. So this cannot be
the primary cause of heart failure.

4. Functional - mild to moderate mitral regurgitation.
So what are we left with?
Acute myocarditis
You probably noticed that a small pericardial effusion was present. This, and the fact
that no other (likely) possibility is left, strongly suggest fulminant myocarditis. Another
cornerstone of our theory: biventricular dysfunction is by no means uncommon in
myocarditis. Later we discovered that her troponin levels were increased. Lydia told us
that she had experienced a respiratory infection 8 weeks earlier. Was it related to her
condition? My suspicion: Lydia has acute myocarditis caused by a viral infection and/or
her autoimmune disorder (Crohn's disease). Maybe she even had giant-cell myocarditis
- an autoimmune-mediated form of myocarditis marked by sudden and severe onset.
Ultimately only a biopsy can provide the answer.
What is clear
We found the reason for acute liver and renal failure. I deliberately didn’t tell you earlier
that Lydia's liver parameters pointed to an ischemic cause of hepatic failure. Of course
her NT-pro BNP was also massively increased. She received inotropic support and
standard heart failure therapy. But Lydia was still on the "edge". Will treatment be
effective? Will she make it? Don’t forget: acute myocarditis is associated with high
mortality (i.e. giant cell myocarditis). To answer this question we performed serial echo
studies. Here is the echo on day four:

5. Apical four-chamber view on day 4: how is LVF now?
What do you think of ventricular function now - three days later? Are you satisfied?
From bad to worse
Of course we didn’t like what we saw. Her EF had deteriorated further - it was now
below 20%. One would certainly expect her pulmonary pressure to be high at this
point, but this was not the case.

6. Spectral Doppler tracing of tricuspid regurgitation on day 4.
The maximal velocity is just mildly elevated: 2.8 m/s.
Her systolic pulmonary pressure (sPAP) was in the range of 36-41 mmHg - in fact, lower
than it had been earlier. Right ventricular function was so poor now that it was unable to
generate high pulmonary pressures. It is not surprising that Lydia’s clinical condition
had deteriorated even further. Her oxygen demand was on the rise, she developed
pleural effusions, and her pulmonary congestion increased. Her central venous
saturation was just 35%. What now? What would you suggest? We initiated treatment
with alprostadil in addition to levosimendan and inotropic agents, and put her on the
urgent list for heart transplantation.
Miracles happen
We were happy to see that Lydia did recover slowly during the next three weeks. Left
ventricular function, vital parameters and lab values (including NT-Pro BNP) gradually
improved. When we performed the echo shown below, Lydia was fully mobile. Her
hepatic and renal function was normal. She was ready to be discharged after 27 long
days in the hospital.

7. Apical four-chamber view on day 27.
Parasternal short-axis view on day 27.
Left ventricular function has greatly improved.
She still has a small residual pericardial effusion.
I think the improvement is astonishing in such a short period of time. Her ejection
fraction improved to 44%. Look at right ventricular function: it is completely normal

8. now. Here is a direct comparison between day 4 and 27:
Apical 4-chamber view, comparison between admission
and day 27. What an improvement.
Do you need more proof that she is truly doing fine now? Okay, here is the mitral inflow
signal:
Normal mitral inflow pattern.
She has normal diastolic function. The E/A ratio is 1.6.
The E/E´ratio was 6. Her filling pressures are completely normal now.
What about mitral regurgitation?

9. As you can see, it is practically gone. There must be
less annular dilatation now.
Our side of the fence
As you can imagine, we were all very happy to see her smile and wave us goodbye
when we gave her final confirmation of her recovery. We were curios to see whether her
left ventricular function had fully returned to normal - to confirm that she truly was on
“our side of the fence”. After all she is one of "us"!