Successful Management of Takayasu’s Arteritis with Pulmonary Hypertension in Pregnancy: A Rare Case

摘要

Introduction: Takayasu’s arteritis (TA) is a rare systemic, chronic inflam-matory, progressive, idiopathic disease of aorta and its main branches. Taka-yasu’s arteritis causes narrowing, occlusion and aneurysm of arteries. The eti-ology of TA is still unknown. It may be autoimmune or genetic in origin or infective diseases. It is commonly found in Asia and oriental countries. Worldwide annual incidence is 1.2 - 2.6 cases/million. Women are affected in 80% - 90% of cases with age of onset below 40 years. TA does not worsen or improve with pregnancy but has adverse effect on pregnancy in the form of abortion, Intrauterine fetal death, superimposed preeclampsia, Intrauterine growth restriction, abruption and congestive heart failure. Because the disease is common in women of childbearing age, management of pregnancy in these patients becomes important; however optimal management for pregnant pa-tients with this disease has not yet been established. Due to the manifold car-diovascular complications that can occur in the course of the disease, man-agement of pregnancies in TA patients is a challenge for the clinician. An in-terdisciplinary collaboration of obstetrician, rheumatologist, cardiologist and anaesthetist is necessary to improve maternal and fetal prognosis. This was the first reported case of TA in pregnancy in Hasan Sadikin Hospital with superimposed preeclampsia and pulmonary hypertension. Materials and Methods: The patient, G1P0A0 was diagnosed as TA since one year ago, and when the patient was pregnant, the patient routinely came to obstetric and rheumatology clinics. Angiographic examination showed dilatation of aorta ascendant, narrowing of aorta descendent at 8 thoracal vertebrae, and arcus aorta, abdominal aorta and the branches were without abnormalities that lead to type IIb TA. On physical examination blood pressure was 200/100 mmHg on the right arm, and 140/100 mm Hg on the left arm, right hand pulse 120 x/m, left hand pulse 113 x/m, respiration rate 20 x/m, temperature 36 ° C. Laboratory values showed Elevated C-reactive protein 6.8 Mg/dl, anemia hemoglobin 10.5 g/dL, elevated rate of sedimentation of blood 36 mm/hours, and proteinuria + 2. The results of other examinations: Glucose, Ureum, Creatinine, GOT, GPT and electrolytes were in normal limits. Thoracal examination/rontgen showed revealed cardiomegaly. Echocardiogram at 20 - 21 weeks gestational age showed an ejection fraction of 55%, hypertensive heart disease and pulmonal hypertension. The ultrasound examination at 36 - 37 weeks’ gestation showed a 2066 g estimated weight (IUGR simetris). Doppler of the umbilical artery showed a resistance index (RI) of 0.49, pulsatility index (PI) 0.70 with a positive end diastolic flow (EDF). Doppler of the median cerebry media artery showed a resistance index (RI) of 0.72, pulsatility index (PI) 1.26. Amniotic Fluid Index 11, 43 cm, Placenta anterior. The pregnancy was controlled tightly in our clinic. During pregnancy in the first trimester, the patient got methylprednisolone 1 × 4 mg, Azathioprin 1 × 50 mg, folic acid 1 × 1 mg. Additional therapy at second trimester were calcium 1000 mg, multivitamin 1 x/day, methyldopa 3 × 250 mg and Acetylsalicylic acid 1 × 80 mg. She was scheduled for a cesarean section at 37 - 38 weeks of pregnancy. Results: According to the American College of Rheumatology (ACR) 1990, our patient fulfilled 4 of 6 TA criteria: age < 40 years, decreased pulsation of left brachial arteries, systolic pressure difference of 50 mmHg between arm, and the arteriographic examination showed narrowing of aorta descendent at 8 thoracal vertebra. She was in stage 2a because she had hypertension, without retinopathy, aneurysm and aortic insufficiency. The patient was monitored with multidisciplinary approach along with the obstetrician, rheumatologist and cardiologist. During antenatal period they followed up periodically with a very strict BP monitoring and fetal growth monitoring. Blood Pressure control was of importance as increase can cause rupture of aneurysms and falls in Blood Pressure could lead to cerebral ischemia in the mother. The risk of fetal death is greatest in the third trimester, especially in those with uncontrolled hypertension. Complication during pregnancy is chronic hypertension superimposed preeclampsia, symmetric IUGR, pulmonal hypertension and hypertension heart disease. The Hypertension may be due to decreased elasticity and narrowing of aorta and abnormal function of carotid and aortic sinus baroreceptor and symmetric IUGR may be impaired by placental blood flow. The mode of delivery in patients with TA is determined by the hemodynamic status of the mother and for obstetrical indications. The patient was scheduled for cesarean section at aterm gestation age, but as 36 weeks she got parturient and emergencies caesarean section was done with epidural anaesthesia by bupivacaine followed by intensive care afterwards. The baby was born with 2180 grams birth wei