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Challenging Case Of Pregnancy Associated Cardiomyopathy Of Multifactorial Origin

Author Information

Jagtap S*, Samant PY**, Yadav K***.
(* Senior Resident, ** Professor, *** First Year Resident. Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract
We present a rare case of cardiomyopathy of probable multifactorial origin who presented with severe hypertension and was detected to have hypothyroidism. The additional burden of endocrine and hypertensive disorder added to the complexity of management. Though it was  diagnosed as peripartum cardiomyopathy, a review of diagnosis was required. 

Introduction

Peripartum cardiomyopathy (PPCM), is a disorder causing cardiac failure. It typically affects pregnant women during a period of one month prior to delivery to five months post partum.  Clinical presentations have been found ranging from thromboembolism to cardiac failure and maternal mortality. It has a tendency to recur in subsequent pregnancies and maternal mortality is considered to be as high as 25 to 50 %. Association of hypertension of uncertain duration and hypothyroidism creates diagnostic dilemma, and add to maternal and fetal morbidity. Multidisciplinary approach aids safe management. 

Case Report

A 30 year old primigravida with 32 weeks and 5 days gestation was referred by a peripheral hospital to our tertiary care center in view of pre-eclampsia. 
The patient had complaints of right-sided chest pain since afternoon of the day of admission and breathlessness for 3 days. There were no other complaints like palpitation, or dizziness or loss of consciousness. She also reported bilateral pedal edema since 7th month of pregnancy. There were no complaints of headache, vomiting, epigastric pain, or blurring of vision. She did not reveal history of any other major medical or surgical conditions in the past. She gave no positive family history. She was taking treatment from a private practitioner since first trimester. She was detected to be hypertensive in the 5th month of pregnancy, and was started on tablet labetalol 100 mg twice daily, but she discontinued treatment after a month. 
On examination her general condition was fair. There was no pallor. She was afebrile, there was no remarkable tachycardia. Blood pressure was 110/70 mm of Hg (on antihypertensive medication). No abnormality was detected on auscultation. Bilateral pedal and vulval edema was present. There was no pallor/ icterus/ cyanosis/ clubbing or lymphadenopathy. Deep tendon jerks were normal.
On abdominal examination, uterus was 26-28 weeks in size, relaxed with a single fetus in vertex presentation and fetal heart sounds were regular at 130 beats per minute. Vulval edema was present. On internal examination cervix was long, posterior, uneffaced. Os was closed. 
Her Hemoglobin was 11.5 gm%. Urine albumin was 3+. PT was within normal limits and INR was 0.93. Serum electrolytes were normal. TSH was advised which was found to be 8.09 IU/ml. Renal and liver function tests were normal. Coagulation screen was normal. Renal ultrasound was normal. Screening for APLA syndrome, ANA, ds DNA was negative. Fundoscopy revealed normal findings. Electrocardiogram was reported to have a normal sinus rhythm, normal axis and T wave inversion in leads V2 to V6. Troponin T test was negative. Echocardiogram was indicative of dilated cardiomyopathy with global hypokinesia and moderate mitral regurgitation with ejection fraction of 45 %. On cardiologists advise oral Furosemide and potassium supplementation were added and antihypertensives were continued . 
In view of hyponatremia that was diagnosed as hypervolemic hyponatremia, fluid restriction was advised. Moist oxygen by mask was started for breathlessness. Endocrinologist started her on Levothyroxine 50 mcg/ day. Parenteral betamethasone was given for fetal lung maturity and parenteral ceftriaxone and gentamycin were also started in therapeutic doses. Labetalol, and furosemide were continued.                                                                                                                                                      
Induction of labor was done as blood pressure rose to 170/110 mm Hg. Epidural labor analgesia was given. She delivered a preterm baby weighing1.24 kg who had an Apgar score of 9/10. She was reviewed by cardiologists on day 2 of delivery. Post delivery echocardiogram revealed ejection fraction of 30%. She was continued on bed rest and the same medical management. On day 3 of delivery her SGOT was 134 U/L, alkaline phosphatase was high at 253 IU/L. Observation and avoidance of hypotension/ hypertension, hepatotoxic drugs, hypoglycemia and sepsis was advised. She recovered in a week and was discharged on diuretics, antihypertensives and thyroid supplements. She and her family were counseled about risk of recurrence in subsequent pregnancies and barrier contraception was advised.

Discussion

Peripartum cardiomyopathy is called diagnosis of exclusion where all other conditions causing cardiac failure are ruled out. A met analysis by Bello et al found pooled prevalence PIH of 22% in PPCM as compared to 5% in normal pregnancy and prevalence of hypertension during pregnancy of 37% in PPCM.[1] Our patient was hypertensive since the fifth month of pregnancy and a treatment defaulter.
Johnson Coyle and colleagues stated that myocardial infarction, sepsis, severe pregnancy induced hypertension, structural cardiac diseases and other factors that may cause cardiomyopathy must be ruled out before labeling a case as PPCM.[2] Both hypo and hyperthyroidism are considered as causative factors in cardiomyopathy though mechanisms differ. Hypothyroidism causes low output cardiomyopathy by affecting alfa myosin chains and affecting ionic channels.[3] Our patient was detected to have hypothyroidism which could have contributed to her cardiomyopathy.
Besides the diagnostic dilemma; the challenge was to deliver her safely. After delivery deranged liver function required careful monitoring and management.
Epidural analgesia and anesthesia help in PPCM cases as those permit gradual induction of anesthesia. Also there is minimal fluctuation in hemodynamic parameters.[4] Epidural induced sympathectomy improves cardiac condition during labor.[5] Multidisciplinary management and epidural analgesia helped our patient go through labor smoothly. Our patient had mild hepatic derangement, which recovered over a few days. Hepatic congestion and cellular failure induced by cardiac failure is reported in cases of PPCM. Fussell reported a case of fulminant hepatic failure that recovered after treating previously undiagnosed peripartum cardiomyopathy.[6] 

Conclusion

It is important to consider and investigate inflammatory, metabolic, cardiological and other factors in cases of peripartum cardiomyopathy so that accurate treatment is initiated and chance of recurrence in subsequent pregnancies reduce. For safe transition through induced or spontaneous labor epidural analgesia is a safe intervention.

References
  1. Bello N, Rendon I. Arany Z. The relationship between pre-eclampsia and peripartum cardiomyopathy: a systematic review and meta-analysis. J Am Coll Cardiol. 2013;62(18):1715-1723. 
  2. Johnson-Coyle L, Jensen L, Sobey A. Peripartum Cardiomyopathy: Review and Practice Guidelines. Am J Crit Care 2012;21(2): 89-98
  3. Patel H, Madanieh R, Kosmas CE, Vatti SK, Vittorio TJ. Reversible Cardiomyopathies. Clin Med Insights Cardiol. 2015;9(Suppl 2):7-14.
  4. Dutt A, Agarwal A, Chatterji R, Ahmed F.  Anesthetic management for caesarean section in a case of peripartum cardiomyopathy. Anesth Essays Res. 2013; 7(2): 273–275.
  5. Bhakta P, Biswas BK, Banerjee B. Peripartum Cardiomyopathy: Review of the Literature. Yonsei Med J. 2007;48(5):731-47.
  6. Fussell KM, Awad JA, Ware LB. Case of fulminant hepatic failure due to unrecognized peripartum cardiomyopathy. Crit Care Med. 2005;33(4):891-3.

Citation

Jagtap S, Samant PY, Yadav K. Challenging Case Of Pregnancy Associated Cardiomyopathy Of Multifactorial Origin. JPGO 2018. Volume 5 No.1. Available from: http://www.jpgo.org/2018/01/challenging-case-of-pregnancy.html