[Malignant angiosarcoma of the right atrium in pregnancy. Diagnostic and therapeutic problems]

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.