A previously healthy 6-week old female is brought to 
the E.D. 10 minutes after experiencing a sudden onset 
of difficulty breathing.
     Exam:  VS T37 (tympanic), P160, R60.  A blood 
pressure is not recorded.  Oxygen saturation 90% in 
room air.  She is tired, pale appearing, and in moderate 
respiratory distress.  Oxygen saturation improves to 
99% on oxygen by mask.  Her skin is mottled.  Capillary 
refill time is about 4 seconds.  Eyes clear.  Oral mucosa 
moist.  Neck supple.  Heart tachycardic and regular.  
There is a grade 3/6 systolic murmur.  No gallops are 
heard.  Lungs clear with good aeration.  Abdomen 
distended and firm.  Severe hepatomegaly is noted.  
There is a 4 cm strawberry hemangioma over the right 
flank region.  Pulses are slightly weak.
     A nasogastric tube is inserted.  Blood work is drawn 
and a chest/abdomen radiograph is obtained in the E.D.

View AP radiograph.

     The chest portion of the radiograph shows 
cardiomegaly.  Although the central pulmonary 
vascularity may be slightly prominent, the lungs are 
largely obscured by the cardiomegaly.  The abdomen is 
distended, and there is a paucity of bowel gas.  There is 
a suggestion of an abdominal mass.
     She was not noted to have any signs or symptoms 
of cardiac disease prior to this incident.  A CT scan of 
the abdomen is obtained.

View abdominal CT scan.

     There are lobular vascular masses in the liver with 
extreme hepatomegaly extending into the pelvis.  These 
are most likely hepatic cavernous hemangiomas.  There 
is a large cyst within the liver.  It is unclear whether 
there is significant hemorrhage into the cyst.

CBC  WBC  7.9,  74% lymphs, 10% monos, 16% segs,
     Hgb 8.6, Hct 25.3, platelets 234,000.

Questions:
     1.  Does this patient have congestive heart failure ?
     2.  Is this patient euvolemic or hypovolemic ?
     3.  Depending on your answer to the above, would 
you administer volume expanding fluids and/or red 
blood cells ?

     The cardiomegaly noted on the chest radiograph is 
quite prominent.  However, it should be noted that 
cardiomegaly does not always equate with cardiogenic 
congestive heart failure, especially in this patient who 
has no previous known history of cardiac disease.  
Cardiomegaly seen on a chest radiograph could also be 
due to pericardial fluid or high-output (non-cardiogenic) 
congestive heart failure.  The cardiac silhouette may 
also appear to be enlarged if the lungs are hypoplastic 
or if the film is taken during exhalation.
     In our patient's case, the presence of a murmur 
suggests the possibility of an anatomic cardiac lesion, 
however, it could also be due to high-output failure.  
The multiple vascular lesions in the liver are consistent 
with high-output congestive heart failure due to 
excessive arterio-venous shunting.  The large 
abdominal mass may be significantly compressing the 
thoracic cavity so that the radiograph in essence is 
similar to an expiratory view.  This may give the heart 
an enlarged appearance when, in fact, it is not
enlarged.
     Based on the clinical information thus far, it is 
difficult to determine with certainty the magnitude of 
congestive heart failure, if any.  An echocardiogram 
would be useful in this situation.
     A stat echocardiogram is performed.  It shows slight 
enlargement of the left atrium and left ventricle.  
Contractility is normal.  This study was able to rule out 
cardiogenic causes of congestive heart failure.  The 
slight enlargement in the chamber sizes indicates that 
some degree of congestive heart failure (CHF) is 
present.  It is probably high-output in nature due to 
excessive arterio-venous shunting.

     Now that we have determined that there is some 
degree of high-output CHF, is this patient euvolemic or 
hypovolemic and should we administer volume 
expanding therapies (fluids and/or red blood cells) to 
her?  It is unclear what is responsible for her acute 
deterioration.  Her anemia may be due to hemolysis or 
hemorrhage.  However, it is unclear whether this has 
worsened acutely, or whether this has occurred slowly.  
Regardless, she is anemic and in failure.  However, the 
high-output CHF makes correcting her hemoglobin 
more complication prone.  Excessive volume expansion 
in a patient with CHF of any type can result in acute 
deterioration, despite correcting the anemia and/or 
hypovolemia.  Red blood cells are administered to her 
cautiously.  She is also treated simultaneously with 
digoxin and furosemide.
     She stabilized well and was transferred to the 
intensive care unit.  She continued to have difficulties in 
maintaining her fluid balance despite intensive care 
measures.  She was transferred to a liver 
transplantation center for selective embolization therapy 
or transplantation.