Volume 3, Case 11
Loren G. Yamamoto, MD, MPH
Kapiolani Medical Center For Women And Children
University of Hawaiʻi John A. Burns School of Medicine
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.