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

Abstract

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 Conclusions: The management of TA in pregnancy is still a challenge. A multidisciplinary team involving obstetrician, cardiologist, rheumatologist and anaesthetist is absolutely needed to get an optimal development of the pregnancy. The risk of complication on the mother and fetus might occur any time. Adequately controlled blood pressure and fetal monitoring might result for better outcome. Birth delivery (pervaginam or perabdominal) is still a problem according to obstetrical condition, general condition of the mother and other additional factors. Careful assessment, treatment of TA complication, and regular antenatal follow-up improve maternal and fetal outcome.