A case of cardiac angiosarcoma presenting as pericardial tamponade

Abstract

Objective-To evaluate the eVects of one year's treatment with beraprost, an orally active prostacyclin analogue, in patients with severe pulmonary hypertension. Patients-13 patients with severe pulmonary hypertension. This was primary in nine, thromboembolic in three, and caused by Eisenmenger syndrome in one. Methods-All patients underwent right heart catheterisation. Mean (SD) right atrial pressure was 5 (3) mm Hg, mean pulmonary artery pressure was 48 (12) mm Hg, cardiac index was 2.6 (0.8) l/min/m 2 , and mixed venous oxygen saturation was 68 (7)%. Beraprost was started at the dose of 20 µg three to four times a day (1 µg/kg/day), increasing after one month to 40 µg three to four times a day (2 µg/kg/day), with further increases of 20 µg three to four times a day in case of clinical deterioration. Main outcome measures-New York Heart Association (NYHA) functional class, exercise capacity measured by distance walked in six minutes, and systolic pulmonary pressure (by echocardiography) were evaluated at baseline, after one month's treatment, and then every three months for a year. Results-After the first month of treatment, NYHA class decreased from 3.4 (0.7) to 2.9 (0.7) (p < 0.05), the six minute walking distance increased from 213 (64) to 276 (101) m (p < 0.05), and systolic pulmonary artery pressure decreased from 93 (15) to 85 (18) mm Hg (NS). One patient died after 40 days from refractory right heart failure, and another was lost for follow up at six months. The 11 remaining patients had persistent improvements in functional class and exercise capacity and a significant decrease in systolic pulmonary artery pressure in the period from 1-12 months. Side eVects were minor. Conclusions-Oral administration of beraprost may result in long lasting clinical and haemodynamic improvements in patients with severe pulmonary hypertension. (Heart 2001;86:661-665)

Cardiac Angiosarcoma

“…On physical examination splitting of S1, heart murmurs, increased jugular venous pressure, peripheral edema, superior vena cava syndrome, hepatomegaly, and ascites can be found [ 6 ]. Signs and symptoms are nonspecific, leading to delayed diagnosis and worse prognosis [ 7 ].…”

Angiosarcoma Presenting as Syncope

“…The right atrium is the most common site of the tumor. 1,2,3 Prognosis is typically poor and is usually less than 1 year. Aggressive behavior and delayed diagnosis on account of non-specifi c symptoms results in the disease being diagnosed usually only after regional spread has occurred, unlike in our case.…”

“…Farah et al have reported a similar case presenting with pericardial tamponade and syncope. 1 The lateral wall of the right atrium is the most common site, the septum being spared in most cases. 3,4 Our case was consistent with the classical description.…”

“…The fl uid was found to be an exudate, negative for acid-fast bacilli and malignant cells. 1 On repeat echocardiography and transesophageal echocardiography (TEE), the mass appeared very vascular and did not show any attachment to the inter-atrial septum. 1 The mass was seen extending to the lumen of the right atrium.…”

“…On echocardiography, massive pericardial effusion with a right atrial mass attached to the lateral wall was seen (Figure 1). 1 A quantity of 1000 mL of sero-sanguinous fl uid was tapped and sent for biochemical, microbiological and cytological studies. The fl uid was found to be an exudate, negative for acid-fast bacilli and malignant cells.…”