Primary
tumors of the heart, in contrast to secondary metastatic
tumors show a very rare occurrence. About 25% of all primary
cardiac tumors are malignant with
angiosarcomas being the most frequent type of
tumor. The clinical presentation include
pericardial tamponade by hemorrhagic
pericardial effusion, right
heart failure, lung
embolism and thoracal
pain. Diagnostic procedures include echocardiography, magnetic resonance imaging, CT scan, angiography, pericardioscopy and finally surgical exploration. We report on a 27-year old pregnant woman (23rd week) with a malignant primary
angiosarcoma of the right atrium. The patient was admitted to the hospital with progressive shortening of breath due to
pericardial tamponade from
pericardial effusion. A
tumor of the right atrium was detected by echocardiography. Diagnostic procedures had to consider the pregnancy of the patient. Transesophageal echocardiography, magnetic resonance imaging and finally angiography of the right heart with
tumor biopsy were performed. Histological examination did not show any signs of
malignancy. After
corticosteroids pericardial effusion disappeared. Surgical exploration of the
tumor was not performed because of the pregnancy. Two weeks later the patient underwent emergency surgery because of progressive
heart failure due to obstruction of the tricuspidal valve by the
tumor. Thereby intrauterine death of the fetus occurred. The
tumor was removed and the right atrium was reconstructed using a
Dacron patch.
Adjuvant chemotherapy was performed. Eight months later the
tumor recurred and
heart transplantation was performed. two months later pulmonary
metastases were found. the patient died 20 months after onset of symptoms. This is a rare case of the very unusual coincidence of pregnancy and a primary malignant
heart tumor.