A rare case of heart failure: Right atrial invasion by hepatocellular carcinoma

A 68-year-old man presented at the emergency department for an episode of syncope. He described thoracic discomfort, orthopnea and lower extremity oedema over the last 2 months. The physical examination disclosed a painless abdominal distension and a systolic heart murmur on auscultation. The patient reports heavy alcohol consumption for years.

The electrocardiogram showed ventricular and supraventricular arrhythmias, without signs of myocardial infarction. The transthoracic echocardiogram depicted a tumour occupying approximately 80% of the right atrium, causing impairing of the cardiac contractility with consequent hemodynamic signs of right heart failure (figure 1). The lesion seemed to come from vena cava. For further characterization, a computed tomography (CT) was performed, which revealed signs of hepatic cirrhosis with a large lesion located in the segment II, invading and extending superiorly through the left hepatic vein and the inferior vena cava, with the tumour thrombus reaching and growing inside the right atrium. This lesion had a heterogeneous hyperenhancement in the arterial phase and washout effect in the portal and delayed phases, as well as a pseudo-capsule (figure 2A-E).

CT scan findings are typical of hepatocellular carcinoma (HCC), later confirmed by liver biopsy (figure 3).

HCC is the fifth most common malignant neoplasm worldwide and the most common form of primary hepatic tumour. It is typically diagnosed in late middle age or elderly adults and more prevalent in males [1-3].

HCC arises almost exclusively in the context of underlying chronic liver disease, most commonly related to viral hepatitis and alcohol [3].

Imaging plays a crucial role in diagnosis, staging and treatment of HCC, with the main role of CT and MRI to detect major criteria based on LI-RADS classification, that determines the probability of HCC in high-risk patients, like the typical pattern of enhancement and the pseudo-capsule both presented in our case (figure 2A-E) [3,4].

HCC tends to spread through intrahepatic blood vessels, lymphatics or direct infiltration. Although this tumour is highly prone to vascular invasion, an intra-cardiac direct involvement via hepatic vein and inferior vena cava is rare, even further if the first clinical presentation is based on heart failure symptoms as seen in our case, with only very few reports in the literature [5,6].

Most cardiac metastases are usually clinically silent, although, in advanced cases, can occur signs and symptoms of heart failure secondary to inflow obstruction and impaired cardiac contractility. It can result in pulmonary embolism or even sudden cardiac death [6,7].

The cardiac involvement in patients with HCC is a major predictor of mortality, with a median survival ranging from 1 to 4 months. Echocardiography is the initial imaging test for the detection of cardiac metastasis, although cardiac MRI and cardiac CT may help further characterize and delineate the extent of both cardiac and extracardiac disease. In addition, on cardiac MRI, an assessment of perfusion after administration of gadolinium can be useful in differentiating malignant from benign cardiac masses, whereas late-enhancement techniques with long inversion recovery (450–600 ms) can be helpful in the identification of thrombus. Contrast-enhanced imaging with a long inversion recovery time will accentuate hypointense intracardiac thrombus and will allow differentiation from metastatic tumors or identification of thrombus associated with these tumours [8].

Although there is no consensus on its management, some studies report that surgical intervention might result in better survival than transcatheter arterial chemoembolization (TACE), systemic chemotherapy or radiation [6,9].

In our case, a multidisciplinary team decided to proceed to TACE (figure 2F-H). However, this proceed was unsucceeded and the patient has undergone surgery due to the high risk of sudden death, removing partially the tumour thrombus combined with left hepatectomy. Unfortunately, the patient died weeks after the surgery.

To conclude, the teaching points of this case are:

• Despite the high propensity of HCC for both direct and distant vascular spread, cardiac invasion is considered an unusual and rare type of presentation.

• Any patient with chronic liver disease or known HCC who presents new-onset of cardiac symptoms should raise clinical suspicion of cardiac invasion.

The prognosis of HCC with intra-cardiac involvement is very poor and can culminate in sudden cardiac arrest.