Successfully reported this slideshow.
We use your LinkedIn profile and activity data to personalize ads and to show you more relevant ads. You can change your ad preferences anytime.

Samir rafla giant-cell myocarditis - an autoimmune-mediated form of myocarditis


Published on

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.

Published in: Health & Medicine
  • Be the first to comment

  • Be the first to like this

Samir rafla giant-cell myocarditis - an autoimmune-mediated form of myocarditis

  1. 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. 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. 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. 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. 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. 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. 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. 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. 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"!