Asian Cardiovasc Thorac Ann 2007;15:66-68
© 2007 Asia Publishing EXchange Ltd
Intimal Cardiac Sarcoma in a Pregnant Woman
Ho Sung Son, MD,
Kyung Sun, MD,
Jae Seung Jung, MD,
Sung Min Park, MD,
Sung Ho Lee, MD,
Kwang Taik Kim, MD
Department of Thoracic and Cardiovascular Surgery, Korea University Medical Center, Seoul, South Korea
For reprint information contact: Kyung Sun, MD Tel: 82 2 920 5559 Fax: 82 2 927 3104 Email: ksunmd{at}kumc.or.kr, Dept. of Thoracic and Cardiovascular Surgery, Korea University Medical School, 126-1 Anam-dong 5-ga, Sungbuk-gu, Seoul 136-705, South Korea.
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ABSTRACT
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A 29-year-old female patient in the 28th week of pregnancy was diagnosed with a cardiac tumor. We performed tumor excision and mitral valve replacement, and her baby was born successfully with cesarean section on the 9th day after cardiac surgery. After this event, 8 cycles of chemotherapy were administered. After 10 months, metastatic intimal sarcoma of the right ovary developed, and a right salpingo-oophorectomy and omentectomy was performed. However, the patient died of sudden onset of intractable ventricular fibrillation.
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INTRODUCTION
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Primary cardiac sarcoma is very rare. In most cases, the therapy for cardiac malignancy is a combination of surgical resection with added chemotherapy or radiotherapy. However, curative therapy is very difficult to achieve because of incomplete resection and a poor response to chemotherapy or radiotherapy. We report herein a case of intimal cardiac sarcoma in the left atrium of a pregnant woman. The tumor mass originated from the anterior mitral valve annulus and had invaded the mitral valve, left atrium and left ventricle. The pathologic findings were confirmed as intimal sarcoma (unclassified type).
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CASE REPORT
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A 29-year-old female patient was referred to us during the 28th week of pregnancy suffering from severe dyspnea. These symptoms had exacerbated during the previous 10 days. She had been receiving routine antenatal care at a private obstetrical clinic, and had not experienced any cardiac problems or symptoms of dyspnea before the pregnancy, or during antenatal care. On physical examination, her neck veins were engorged and the heart rate was 120 beats·min–1 with an irregular rhythm (Figure 1
). The patient’s blood pressure was 120/80 mm Hg and her respiratory rate was 48 breaths·min–1.Auscultation revealed coarse breathing sounds on both lower lung fields and a diastolic rumbling murmur on the left fourth intercostal space at the mid-clavicle line. Chest radiography showed increased pulmonary vascular markings, bilateral pleural effusions and severe cardiomegaly. Echocardiography showed a 24 x 52 mm echogenic mass and severe mitral steno-insufficiency. The mass was mobile and irregular, and was attached to the anterior mitral leaflet. The systolic pulmonary arterial pressure was calculated as 112 mm Hg. The low density tumor mass in the left atrium was confirmed on preoperative chest computerized tomography (CT) (Figure 2).
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Figure 1. Preoperative electrocardiographic findings. Sinus arrhythmia was demonstrated in the preoperative electrocardiography.
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Figure 2. Chest CT of the patient; (A) The tumor mass in the left atrium was demonstrated in the preoperative chest CT (arrow); (B) Chest CT showed that the tumor mass had not enlarged 10 months after the operation.
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On obstetrical examination, the fetus was in a breech presentation with body weight appropriate for gestational age (AGA). The fetal heart beat and activity were within normal limits. Because of intractable pulmonary congestion and dyspnea, we decided to remove the cardiac mass. The operation was done with a median sternotomy. Cardiopulmonary bypass (CPB) was maintained with normothermia and the maternal mean arterial pressure was kept above 70 mm Hg during CPB. The operative findings revealed that the mass originated from the anterior mitral valve annulus and had metastasized to the mitral valve, left atrium and left ventricle. Its size was measured as 5 cm x 2 cm (Figure 3). The mass was incompletely resected due to annulus invasion of the mitral valve. Mitral valve replacement was performed with a 27 mm ON-X mechanical valve, and a modified De Vega tricuspid annuloplasty was also done. After the operation, anticoagulation therapy was started with low molecular heparin 7200 IU·day–1, injected into the subcutaneous layer. The baby was born successfully with transabdominal delivery by cesarean section on the 9th postoperative day (GA 29+5 wks). The baby was of normal appearance and health.
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Figure 3. Operative findings; a 5 cm x 2 cm tumor mass originated from the anterior mitral valve annulus in the left atrium (arrow).
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The pathologic findings for the cardiac mass were confirmed as an unclassified type intimal sarcoma (Figure 4). After the delivery, chemotherapy was commenced. The chemotherapy regimen used was adriamycin, ifosfamide and dacarbazine. After 8 cycles of chemotherapy, the heart residual tumor mass showed no signs of growing, thus, we decided to observe the patient without further treatment as the adriamycin cumulative dose had reached 480 mg·m–2. A huge 9 x 5 cm metastatic intimal sarcoma in the right ovary developed 10 months after the cardiac operation, so a right salpingo-oophorectomy and omentectomy was performed. However, the patient died soon after the operation as the result of sudden onset of intractable ventricular fibrillation.
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Figure 4. Pathologic findings of the cardiac tumor; (A) The tumor is composed of highly mitotic atypical spindle cells forming irregular fascicles (HE x 200); (B) Immunohistochemistry: strong reactivity for vimentin (ABC x 200) was demonstrated.
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DISCUSSION
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Primary cardiac tumors are very rare.1–3 In an autopsy series, their incidence was found to be 0.02%, with one quarter of the cases showing malignant histopathologic features. Seventy-five percent of the primary cardiac tumors are benign. Primary sarcomas of the heart are the second most common primary cardiac tumor after myxomas, accounting for 10% to 20% of the resected cases. Nearly 50% of primary cardiac tumors are located in the left atrium (LA).2 The differential diagnosis between primary cardiac sarcoma and myxoma in the LA is difficult. When attempting to distinguish between primary cardiac sarcoma and myxoma of the LA, the site of origin, nature, extension, and size of tumor are important features. Of these features, the original site of the tumor is the most important factor: myxoma usually originates from the atrial septum and sarcoma generally has a non-septal origin.2,4 It is also very difficult to achieve complete remisson.3 Because the surgical resection margins are rarely free of disease, the tumor shows early metastasis and local recurrence. Mery and associates1 have also reported distant metastases without evidence of local recurrence, as in our case. Although chemotherapy or radiotherapy are indicated for treatment after the surgery, the patient’s prognosis is poor.1,2,4 Life expectancy of these patients is less than one year, and the median survival time ranges from 5 to 11 months.2
The incidence of heart diseases in pregnant women ranges from 1% to 4%.5–7 Mitral valve disease is the most common disease. During cardiac surgery, cardiopulmonary bypass (CPB) could seriously compromise the fetus more than the mother.8 Cardiopulmonary bypass causes alterations in coagulation, the release of vasoactive substances, activation of the complement system, nonpulsatile flow, hypotension and hypothermia. The hypothermia may lead to uterine contraction and reduction of placental blood flow.5,6,8 Pomini et al5 and Parry7 reported that maternal mortality was 2.9% and 1.5%, respectively, and embryo/fetal mortality was 20.2% and 19%, respectively. To reduce fetal mortality and morbidity during open heart surgery on pregnant women, CPB is maintained with a high flow, high pressure (mean blood pressure 60 mm Hg) and normothermia. The hematocrit should also be kept higher than 25% for hyperoxygenation, and for as brief a period as possible.5–7
In conclusion, cardiac malignancy in pregnant women is very rare and is difficult to treat. Surgical resection is the treatment of choice and adequate flow should be maintained to avoid fetal distress during CPB.
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REFERENCES
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- Mery GM, Reardon MJ, Hass J, Lazar J, Hindenburg A. A combined modality approach to recurrent cardiac sarcoma resulting in a prolonged remission: a case report. Chest 2003;123:1766–8.[Medline]
- Ceresoli G, Passoni P, Benussi S, Alfieri O, Dell’Antonio G, Bolognesi A. Primary cardiac sarcoma in pregnancy: a case report and review of the literature. Am J Clin Oncol 1999;22:460–5.[Medline]
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- Arnoni RT, Arnoni AS, Bonini RC, de Almeida AF, Neto CA, Dinkhuysen JJ, et al. Risk factors associated with cardiac surgery during pregnancy. Ann Thorac Surg 2003;76:1605–8.[Abstract/Free Full Text]
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ABSTRACT
INTRODUCTION
