Dyspnea delivered by the stork


No, the title has nothing to do with ornithology, but read on and you will discover how the case is related to the stork.
 
The stork arrives

Here is the story of a patient who was referred to our clinic because of shortness of breath. Her name is Carla, she is 49 years old, and has considered herself healthy up until now.


Storks live and breed in Austria during the summer.They
typically build their nests on chimney tops in the region of
Neusiedlersee - a lake area in the eastern part of Austria.
In the winter they migrate to Africa.

 
Ten days ago Carla gave birth to a healthy child in a smaller hospital. Her labor was complicated by placenta accreta. She developed major bleeding and severe anemia, which necessitated a Caesarean section. After the Caesarean section she was given packed red blood cells and underwent curettage. Bleeding could be controlled and the patient was stabilized shortly after the operation. The brighter side: Carla and her daughter were discharged after a week of observation. Both were doing fine at the time.

After the stork left
A few days later the patient developed dyspnea. Her clinical condition deteriorated rapidly and she was admitted to our hospital. At this time Carla was in pre-shock. She had severe dyspnea, her oxygen saturation was 89% and her blood pressure 90 / 45 mmHg. What was the problem? Was her condition related to the previous bleeding?

Tracing the path of the stork
That’s what we thought initially. However, arterial blood gas analysis showed us that we were wrong. Besides, the patient's red blood count was in the normal range. Her ECG showed sinus tachycardia, which is not unusual in a state of shock. This was her chest x-ray at admission:
 

The chest X-ray indicates pulmonary congestion, an enlarged
heart, and pleural effusion.

So maybe she did have a heart problem after all? Obviously, the next step was to perform echocardiography. Surprise, surprise: this is what we found:
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The parasternal long-axis view showed reduced left ventricular
function. Note the accumulation of fluid posterior to the heart
and also posterior to the aorta. This is pleural effusion.
 
It was evident that she had heart failure. But why? Could it be a valve problem?


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Parasternal long-axis view showing mild to moderate mitral
regurgitation.

 
Regurgitation was not severe enough to explain heart failure. But what about left ventricular function?
 

This atypical four-chamber view shows diffusely reduced left
ventricular function. Note that the left ventricle is not very
large. This indicates “acute onset” of left ventricular
dysfunction.

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Reduced LVF is also seen on the two-chamber view.
 
Poor left ventricular function certainly explains her symptoms.


What the stork had done:
What an unexpected finding in a relatively young patient without a history of heart disease. One could summarize the findings as follows: “Sudden onset of cardiomyopathy with acute heart failure shortly after labor.” We are sure some of you know the diagnosis: Peripartum cardiomyopathy (CMP).
 
What is peripartum cardiomyopathy?

Peripartum cardiomyopathy is a rare cause of heart failure that affects women late in pregnancy or early puerperium. It usually develops between the last weeks of gestation and about 5 months post partum. Establishment of the diagnosis requires four criteria:
  • development of heart failure in the last month of pregnancy or within five months of delivery,
  • absence of any other identifiable cause of heart failure,
  • absence of heart disease prior to the last month of pregnancy,
  • LV systolic dysfunction.
Although the true etiology remains unclear, several risk factors for the disease have been identified:
  • age over 30 years
  • multiparity
  • African descent
  • pregnancy with multiple fetuses
  • a history of preeclampsia, eclampsia, or postpartum hypertension
  • maternal cocaine abuse
  • long-term oral tocolytic therapy with beta adrenergic agonists such as terbutaline
The treatment options are quite similar to those for regular heart failure, including angiotensin inhibition,diuretics, digoxin, vasodilators, beta-blockers, and aldosterone antagonists. Treatment also includes the management of arrhythmia and anticoagulation. Heart transplantation may be used as the last resort.
 
Beating the stork back?

Carla was stabilized with “medication for heart failure”. Her left ventricular function improved and she could be transferred to the normal ward quite early. She was discharged two weeks later. Here is her echo at discharge:

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Parasternal short axis: radial function has definitely
improved.
 
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However, global left ventricular function is still not normal
(4-chamber view).
 
Will the stork return?

Maybe she had CMP all along, and her pregnancy merely unmasked the problem. However, considering her good condition before pregnancy and the rapid onset of symptoms, this seems rather unlikely.
Could some form of Takutsubo cardiomyopathy triggered by the stress of a Caesarean section and bleeding have led to cardiomyopathy? Frankly, we don’t know.
As a rule of thumb: if the patient's LVF normalizes rapidly after the treatment, one may anticipate a favorable prognosis. However, this only happens in about 50% of patients.
Yours Andi Schober & the 123sonography team

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A wonderful example of how echo plays an important role in emergency medicine. Watch out for more case from our ER team and more of emergency medicine in general. For those of you who wish to learn more about emergency ultrasound: please visit 3rd Rock Ultrasound (http://www.emergencyultrasound.com/) or our website at 123sonography.com
Yours Thomas

 
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