MALIGNANT ANGIOSARCOMA OF THE RIGHT ATRIU M DURING PREGNANCY - DIAGNOSTIC AND THERAPEUTIC PROBLEMS

Primary tumors of the heart, in contrast to secondary metastatic tumor s show a very rare occurrence. About 25% of all primary cardiac tumors are malignant with angiosarcomas being the most frequent type of tumo r. The clinical presentation include pericardial tamponade by hemorrha gic pericardial effusion, right heart failure, lung embolism and thora cal pain. Diagnostic procedures include echocardiography, magnetic res onance imaging, CT scan, angiography, pericardioscopy and finally surg ical exploration. We report on a 27-year old pregnant woman (23rd week ) with a malignant primary angiosarcoma of the right atrium. The patie nt 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 echocard iography, magnetic resonance imaging and finally angiography of the ri ght heart with tumor biopsy were performed. Histological examination d id not show any signs of malignancy. After corticosteroids pericardial effusion disappeared. Surgical exploration of the tumor was not perfo rmed because of the pregnancy. Two weeks later the patient underwent e mergency surgery because of progressive heart failure due to obstructi on of the tricuspidal valve by the tumor. Thereby intrauterine death o f the fetus occurred. The tumor was removed and the right atrium was r econstructed 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 patie nt died 20 months after onset of symptoms. This is a rare case of the very unusual coincidence of pregnancy and a primary malignant heart tu mor.