Author Information
Venkateswaran S*, Chauhan AR**
(* Second year Resident, ** Professor, Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)
Abstract
Takayasu aortoarteritis (TA) is a condition that causes narrowing, occlusion and aneurysm of medium and large arteries. Pregnancies in such cases are associated with severe life threatening systemic complications, and hence require super-specialty management and future contraception counseling. We present a case of a 30 year old primigravida with rheumatic heart disease with severe aortic stenosis in a diagnosed case of TA. Pregnancy was continued with multidisciplinary care and the patient delivered by a lower segment cesarean section at term for obstetric indication.
Introduction
Takayasu aortoarteritis (TA) is a chronic idiopathic large vessel vasculitis. It primarily involves the aorta and its branches, coronaries and pulmonary arteries. It is also known commonly as “young female arteritis” owing to the demographic distribution; and “pulseless disease” as it causes progressive inflammation, narrowing, scarring and abnormal ballooning within the aorta and its major branches. It shares some histologic features with giant cell arteritis, which is the other major large-vessel vasculitis. Affected individuals are usually women in reproductive age group and of Asian origin. The incidence is 2.6 cases per million population per year.[1] TA was earlier suspected to be an infection, later identified as an autoimmune condition. Genetic factors may also have an important role.[2] The inflammatory process has been thought to be initiated by cell mediated immunity. Pro-inflammatory cytokines like tumor necrosis factor (TNF)-alpha, interferon (IFN) gamma and interleukins are associated with granuloma formation on large vessel walls.[3] Successful management during pregnancy requires a multidisciplinary approach with obstetrician, cardiologist and rheumatologist because of multiple systemic complications associated with the disease.[4]
Case Report
A 30 year old primigravida presented at 26 weeks of gestation to our OPD. She was a diagnosed case of TA since the last 10 years. Rheumatic heart disease with aortic regurgitation (AR) had been diagnosed 6 years ago; aortic valve replacement with bioprosthetic valve (Epic® St. Jude’s valve) was done within a year of diagnosing AR. She had no complaints and was regularly following up with a cardiologist. She was on oral prednisolone 20 mg, metoprolol 50 mg and diuretic (furesemide 40 mg), all once daily. A repeat echocardiogram was done after she conceived for evaluation of persistently raised blood pressure in both upper limbs; and it was suggestive of severe restenosis of the aortic valve and type 2 diastolic dysfunction. Patient was then referred to us for further multidisciplinary management.
On examination her general condition was fair. She was afebrile and had no recent history of acute febrile illness. Her radial pulse was 80 beats/ minute and of good volume in the left radial artery but was felt feebly in the right radial artery. Blood pressure in the left brachial artery was 165/ 80 mm Hg and right brachial artery was 178/ 80 mm Hg. On cardiovascular auscultation, both heart sounds were heard normally and there was a pansystolic murmur. Respiratory system was normal. There was no neurological or vascular deficit in any limbs. Per abdomen, uterus was 24 - 26 weeks’ size, fetal heart sounds were heard and regular, uterus was relaxed. Per vaginally os was closed and uneffaced.
Complete hemogram was normal with a hemoglobin of 12.5 gm %, adequate total leucocyte and platelet counts. Her plasma sugars, liver, renal and thyroid function tests were within normal limits. Her CRP level was 21.7 mg/ dL. All autoimmune markers (ANA, dsDNA, anti ß2 glycoprotein, lupus anticoagulant, anti-phospholipid antibody) were negative. Fetal malformation scan was normal.
Cardiologist repeated a 2D echocardiogram which showed very severe aortic stenosis with a valve area of 0.6 square cm, mild mitral regurgitation, mild tricuspid regurgitation and mild pulmonary hypertension. Shortening of second stage of labor was advised due to severe degenerative stenosis of the bioprosthetic aortic valve. She was asked to continue all medicines. Rheumatologist advised to continue prednisolone 20 mg daily for 6 weeks, followed by tapering of dose by 2.5 mg every 15 days. Tablet labetalol 100 mg twice daily and nifedipine 20 mg twice daily were started and were continued throughout pregnancy.
Her blood pressure was closely monitored and she was watched for development of preeclampsia. Obstetric ultrasound with Doppler studies were done serially (weekly) for early detection of intrauterine growth restriction. At 37 weeks of gestation, she had prelabour rupture of membranes with poor Bishop score, and was unwilling for induction of labor. Hence emergency lower segment cesarean section was done under spinal anesthesia. She delivered a healthy female neonate of 2.5 kg birth weight with Apgar score of 9/10. The LSCS was uneventful with 600 ml of blood loss. In the immediate postoperative period she developed acute breathlessness due to severe hypovolemia and pulmonary edema. She was then intubated on he operation table itself and shifted to cardiac intensive care unit for observation. Diuretics and inotrope support were started and she was extubated within 24 hours. She had a gradual recovery was shifted to the ward after 5 days. She started breastfeeding and was discharged after cardiac and rheumatologic opinion and advised to continue antihypertensives and steroids in tapering dose.
At cardiac follow up post-delivery, she has been advised a re-do aortic valve replacement due to severe aortic stenosis, restricted aortic valve movement and hypokinesia at the left ventricular base.
Discussion
More than 150 pregnancies in women with TA have been published. Most common complication seen is hypertension (in around 30% pregnancies) with 20% developing preeclampsia. Myocardial infarction, aortic aneurysm and dissection, pulmonary embolism and such other serious maternal complications have been reported. Intrauterine growth restriction due to poor placental perfusion is seen in 20% pregnancies, and intrauterine fetal demise in 8% pregnancies.[4] In uncomplicated cases of TA in pregnancy, the entire duration of pregnancy and delivery are usually uneventful. In our patient, due to severe aortic stenosis as a consequence of degeneration of the biprosthetic valve, patient decompensated in the peripartum period. Newer bioprosthetic valves have a longer life than older ones, ranging from 8 to 20 years. The main advantage is that anticoagulation is not required; however in younger patients they are not preferred mainly due to the need for repeat surgery.With suitable anticoagulation, mechanical valves are preferred in young patients < 40 years of age, especially in low resource settings where repeat surgery may be difficult.[5] Decision for cesarean section or trial of normal labor is up to the treating obstetrician.
Pregnancy complications, including pregnancy loss and preterm birth, are higher among women with all forms of vasculitis.[6] Pregnant women without active Takayasu arteritis have a low risk of developing a cardiovascular event. For women with chronic hypertension, it is important to note the development of preeclampsia, fetal growth restriction and abruption.[7]
Blood pressure can increase significantly during labor. In the presence of subclavian stenosis, blood pressure measurements may be inaccurate by an arm cuff. Internal blood pressure monitoring may be required in these cases. Elective cesarean section may be prudent for these patients. Anesthesia in cases of TA is challenging as uncontrolled hypertension and end-organ dysfunction need to be avoided. General as well as regional anesthesia may be used; newer reports suggest the safety of low-dose spinal anesthesia with close monitoring.[8]
Conclusion
Thus it is seen that TA is a common condition in women of reproductive age group, these pregnancies should be regarded as extremely high-risk and given adequate multidisciplinary care. Combined efforts of the obstetrician, cardiologist and rheumatologist can result in successful maternal and perinatal outcome.
References
- Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: a review. J Clin Pathol. 2002; 55(7): 481-6.
- Ogino H, Matsuda H, Minatoya K, Sasaki H, Tanaka H, Matsumura Y et al. Overview of late outcome of medical and surgical treatment for Takayasu arteritis. Circulation. 2008; 118(25): 2738-47.
- Goel R, Kabeerdoss J, Ram B, Prakash JAJ, Babji S, Nair A et al. Serum Cytokine Profile in Asian Indian Patients with Takayasu Arteritis and its Association with Disease Activity. The Open Rheumatol J. 2017; 11: 23-9.
- Hauenstein E, Frank H, Bauer JS, Schneider KT, Fischer T. Takayasu’s arteritis in pregnancy: review of literature and discussion. J Perinat Med. 2010; 38(1): 55-62.
- Choudhary SK, Talwar S, Airan B. Choice of prosthetic heart valve in a developing country. Heart Asia. 2016; 8(1): 65-72.
- Machen L, Clowse ME. Vasculitis and Pregnancy. Rheum Dis Clin North Am. 2017;43(2): 239-47.
- Tanaka H, Tanaka K, Kamiya C, Iwanaga N, Yoshimatsu J. Analysis of pregnancies in women with Takayasu arteritis: complication of Takayasu arteritis involving obstetric or cardiovascular events. J Obstet Gynaecol Res. 2014; 40(9): 2031-6.
- Dutta B, Pandey R, Darlong V, Garg R. Low-dose spinal anaesthesia for a parturient with Takayasu's arteritis undergoing emergency caesarean section. Singapore Med J. 2010; 51(6): e111-3.
Venkateswaran S, Chauhan AR. Pregnancy With Takayasu Arteritis And Heart Disease. JPGO 2017. Volume 4 No.8. Available from: http://www.jpgo.org/2017/08/pregnancy-with-takayasu-arteritis-and.html