Liver hemangiomatosis with hyperdynamic right heart failure and Kasabach-Merritt Syndrome

71-year-old female patient with dyspnea and episodes of oppressive chest pain with minimum effort that disappeared after 2-3 minutes. No previous history. Physical examination showed intense systolic murmur in the aortic area, hepatomegaly and mediocre general condition. Laboratory tests revealed anaemia, low platelet count, coagulation disorder and increased total bilirubin.

Ultrasonography showed hepatomegaly with multiple focal lesions, mainly hypoechogenic and cystic with a diffuse distribution (Figure 1). 

For further characterization, CT and MR scans were performed. On one hand, an enlarged liver with lobulated contours and nodular lesions with progressive enhancement were found on CT (Figure 2). On the other hand, MR showed a diffuse involvement of the liver with very high signal on T2WI and progressive contrast enhancement following the vascular pool (Figure 3) as well as hepatic arterial enlargement with increased flow (2640mL/min). 

Cardiac MRI was also performed demonstrating hyperdynamic systolic function with global cardiomegaly suggesting a systemic arteriovenous fistula.

Hemagiomatosis is a very rare condition characterized by the substitution of hepatic parenchyma with poorly defined lesions formed by dilated and irregular vessels that replace the normal parenchyma [1]. Aetiology is unknown and the definitive diagnosis is histological, even though, imaging diagnosis can be assumed with a very high level of certainty when image studies show typical findings. 

Symptoms can be produced due to compression of adjacent organs and episodes of acute pain can be found in case of intralesional bleeding.

One of the main known complications is Kasabach-Merritt syndrome, a localized form of disseminated intravascular coagulopathy (DIC) [2], defined as thrombocytopenia, microangiopathic hemolytic anaemia and consumptive coagulopathy [3]. 

Hemangiomas have been known to induce high-output heart failure as a result of left-to-right systemic shunting [4] due to the lesion itself secondary to the lowering of the systemic vascular resistance and a compensatory rise cardiac output. This is a rare presentation. 

Hepatic impairment associated with right heart failure implies very high mortality. Liver transplantation is the only possible alternative, which in our case, was rejected due to the advanced stage of the cardiovascular affection.

On ultrasound, hemangiomas are normally hyperechogenic and without Doppler signal because of the low flow. However, there is a form of high flow hemangioma in which it is possible

to register vascular flow, dilated arteries and portal flow inversion. These high-flow

hemangiomas are more frequently hypoechogenic and show a hyperechogenic ring. In CT scan they are hypodense, with a typical pattern of intense peripheral and nodular enhancement with progressive centripetal filling; although this depends on the size, since big ones can enhance incompletely [1].

MR is the imaging method of choice. On T1WI, hemangiomas display low signal intensity and on T2WI they are strikingly homogeneous, clearly demarcated from the adjacent parenchyma, with very high signal intensity. The enhancement is similar to that seen on CT.

In conclusion, diffuse hemangiomatosis, is a very rare entity characterized by the presence of large hemangiomas all through the liver parenchyma. In the worst scenario, it can lead to liver failure, Kasabach-Merritt syndrome and hemodynamic compromise with cardiac failure. It is of paramount importance to make an early diagnosis in order to try to proceed to a transplant since no other treatment has demonstrated useful. Image findings can lead to a confident diagnosis when histological sample is not available due to bleeding risk. 

Written informed patient consent for publication has been obtained.