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Pregnancy Outcome In a Case Of Aortoarteritis With Single Kidney

Author Information

Sinha S*, Warke W**, Mayadeo NM***
(* Third year resident, **Associate Professor,*** Professor, Department of Obstetrics and Gynecology, Seth G. S. Medical College & KEM Hospital, Mumbai, India.)

Abstract

Aortoarteritis is a rare chronic granulomatous disease affecting the aorta and/ or its major branches in females of reproductive age group. It is usually associated with adverse pregnancy outcome. The maternal and fetal outcome in a case of aortoarteritis further complicated by unilateral nephrectomy is presented in this case report.

Introduction

Takayasu’s aortoarteritis is also known as “PULSELESS DISEASE” or “MARTORELLS SYNDROME”.[1] It involves the aorta and/ or its major branches. Women under the age of 40 years are mostly affected, the condition being more common in Asian population.[2] Wall thickening, fibrosis, stenosis, aneurysm and thrombus formation can be seen as sequela to vessel inflammation. Diagnosis is made on presence of 3 out of 6 criteria given by American College Of Rheumatology.[3] Symptoms are varied and reflect end organ ischemia such as vertigo, limb claudication, hypertension, blindness and congestive cardiac failure. The arterial media is destroyed during acute inflammation and lead to aneurysm formation.[4] Worsening of disease is not seen during pregnancy, but complications such as intrauterine growth restriction, fetal death, hypertension, superimposed preeclampsia, heart failure and aneurysm rupture have been reported.[5] Clinical groups were defined by Ishikawa based on the natural history, complications of the disease and their severity at the time of diagnosis.[6] These complications are Takayasu retinopathy, secondary hypertension, aortic regurgitation, and aneurysm formation.

Case Report

A 32 year old primigravida married since 7 years conceived spontaneously, known case of aortoarteritis since childhood with left sided nephrectomy came to our outpatient department at 25 weeks of gestation for antenatal registration. She had a past history of hospitalization at 11 years of age for fever with palpitation where BP recorded was 200/140mm Hg and urine albumin was +3. She was then referred to a higher center for further management. Her serum creatinine was 6.5mg%, ECG was suggestive of left ventricular hypertrophy and left small contracted kidney was noted on ultrasonography. DTPA scan indicated 82% function of right kidney 18% function of left kidney. Aortogram indicated left renal artery and lower abdominal aorta stenosis suggestive of aortoarteritis. Left sided nephrectomy was done and the patient was discharged on tablet Captopril 6.25 mg od. She stopped the drug on her own after 1 year and did not follow up. She presented in our outpatient department with facial puffiness, BP of 140/90 mm Hg, bilateral pedal edema and urine albumin +2. Bilateral pulses were regular, symmetrical without any radio radial or radio femoral delay. All routine investigations were within normal limits except serum TSH 6.58 µIU/ mL and serum creatinine was 1.1 mg%. The patient was admitted and nephrology, rheumatology, cardiology, ophthalmology and endocrinology references were taken. She was started on tablet alphadopa 500mg tid, tablet labetalol 50mg bd, tablet thyronorm 75mg od and low dose aspirin. As BP was not controlled tab amlodipine 5mg od was also added. 2D Echocardiography was suggestive of ejection fraction of 60% with trivial mitral regurgitation and no evidence of coarctation of aorta. Doppler of the abdominal aorta showed a normal vessel wall and lumen with regular flow. Obstetric Doppler indicated amniotic fluid index of 6 cm, estimated fetal weight of 850 gms with increased S/D ratio at 28 weeks of gestation. Weekly obstetric Doppler, BP and serum creatinine monitoring was done. Antenatal steroids were given. At 32 weeks of gestation pregnancy was terminated in view of absent diastolic flow in umbilical artery on Doppler and increased creatinine of 1.7 mg%. Elective LSCS under spinal with epidural anaesthesia was performed and female child of 1.08 kg having APGAR 9/10 was delivered. Her postoperative course was uneventful and was discharged on 5th postoperative day. Baby was admitted in NICU and discharged after 1 month with baby weight of 1.6 kg.

Discussion

Our patient had involvement of left renal artery due to aortoarteritis at an early age leading to poor renal function and severe hypertention. Removal of affected kidney led to normalization of BP. The disease then went into remission. Due to increasing strain on single kidney during pregnancy preeclampsia developed. Proper and timely management of preeclampsia is crucial for good maternal and fetal outcome.
Aortoarteritis does not adversely affect fertility or appear to exacerbate during pregnancy. However management of hypertension is required for favorable pregnancy outcome. Pregnancy is associated with several changes in renal physiology. The glomerular filtration rate increases by up to 50% above baseline levels.[7] Protein excretion during pregnancy is physiological which is further aggravated particularly towards the end of pregnancy in patients with proteinuric renal disease. This leads to either de novo or worsening of pre existing hypertension. Shekhtman et al found decrease in function of the solitary kidney during pregnancy, but renal insufficiency was seldom seen. Obstetric complications were noticed more frequently compared to women with two kidneys like gestosis 28%, threatened abortion 18%, premature delivery 25%.[8]

Conclusion

Careful assessment, treatment of complications, regular antenatal followup and multidisciplinary approach involving obstetrician, cardiologist, rheumatologist, nephrologist, anesthetist and neonatologist in women with aortoarteritis can improve maternal and fetal outcome.

References
  1. Lupi-Herrera E, Sánchez-Torres G, Marcushamer J, Mispireta J, Horwitz S, Vela JE. Takayasu's arteritis. Clinical study of 107 cases.Am Heart J. 1977 Jan;93(1):94-103.
  2. Langford CA, Fauci AS.The Vasculitis Syndromes.In Fauci A, Braunwald E, Kasper D, Hauser S, Longo D, Jameson Loscalzo J.editors in Harrison's Principles of Internal Medicine, 17/e. New York: McGraw-Hill Medical, 2008.
  3. Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990 Aug;33(8):1129-34.
  4. De Jesús GR, d’Oliveira ICC, dos Santos FC, Rodrigues G, Klumb EM, de Jesús NR, et al. Pregnancy may Aggravate Arterial Hypertension in Women with Takayasu arteritis. IMAJ 2012; 14: 724-728.
  5. 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.
  6. Ishikawa K. Natural history and classification of occlusive thromboaortopathy (Takayasu's disease).Circulation 1978; 57(1): 27–35. 
  7. Davison JM, Dunlop W. Renal hemodynamics and tubular function normal human pregnancy. Kidney International. 1980 Aug;18(2):152–161.
  8. Shekhtman MM, Petrova SB. Pregnancy and labor in females with solitary kidney. Ter Arkh. 2000; 72(6):39-42.
Citation

Sinha S, Warke W, Mayadeo NM. Pregnancy Outcome In a Case Of Aortoarteritis With Single Kidney JPGO 2016. Volume 3 No. 2. Available from: http://www.jpgo.org/2016/01/pregnancy-outcome-in-case-of.html