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Uncorrected d-Transposition of Great Arteries in Pregnancy

Author Information

Supriya Poonia*, Vibha More**, M. N. Satia***
(*Third Year Resident, ** Assistant Professor, *** Professor. Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

Congenital heart diseases (CHD) are the most common birth defects. They affect approximately 0.8% of all live births.[1] They are an important cause of maternal and perinatal morbidity and mortality. We present an important case of 22 years primigravida with previously diagnosed but uncorrected case of d-Transposition of Great arteries (TGA) who delivered full term low birth weight baby at 38 weeks of gestation.

Introduction

TGA accounts for 5-7 % of congenital heart disease.[2]   TGA is a birth defect in which two main outlet arteries – aorta and pulmonary artery are transposed i.e. switched in position. In babies with TGA deoxygenated blood enters the right side of heart and instead of going to the lungs, is directly pumped into systemic circulation through aorta. Often babies with d-TGA have other congenital heart defects, such as ventricular septal defect (VSD) or atrial septal defect (ASD) or patent ductus arteriosus (PDA) which are necessary for mixing of blood between two parallel circulations and for survival. Because a baby with TGA cannot maintain oxygen saturation and requires surgery immediately after birth it is considered as a critical congenital heart disease.
Physiological changes of pregnancy may mimic or mask heart disease. Echocardiography (ECHO) is the first investigation of choice. Many women of TGA are reaching reproductive age group owing to improved survival because of surgical treatment in childhood or may be for the first time picked up later in life due to compensation by other associated lesions.[3]   Preconception counselling and skilful management with multidisciplinary approach of obstetrician, cardiologist, anaesthetist and neonatologist plays an important role in successful pregnancy outcome.[4]

Case Report

A 22-year-old primigravida, married for 2 years, was referred at 19.5 weeks of gestation with complaints of dyspnea on excretion NYHA class II. There was a history of easy fatigability since childhood and delayed milestones. Cardiac evaluation was done 1 year ago and the patient was diagnosed to have d-TGA, started on metoprolol 25 mg and advised surgery. She was not compliant to treatment. No further treatment details were available. In present pregnancy she was hospitalised for further evaluation. On admission general condition was moderate, vital parameter were normal, clubbing and peripheral cyanosis were present; oxygen saturation was between 80-85%. On cardiovascular examination showed left parasternal heave, loud second heart sound, pansystolic murmur, and ejection systolic murmur at aortic and pulmonary area.  Respiratory and CNS examination was normal. On obstetric examination the uterus was corresponding to 20 weeks of gestation.  Cardiology opinion was taken. 2D ECHO was done, which showed cyanotic heart disease, situs solitus with double outlet right ventricle d- TGA, ostium secundum atrial septal defect (ASD) with bidirectional shunt, subaortic ventricular septal defect (VSD) with bidirectional shunt, severe infundibular and valvular pulmonary stenosis, and large patent ductus arteriosus (PDA).  Electrocardiograph showed normal sinus rhythm, right axis deviation, tall P wave with right atrial enlargement. She was advised to continue metoprolol and was also started on tablet propanolol 20 mg q8h. Obstetric ultrasonography, fetal anomaly scan and fetal 2D-ECHO were normal. She had secondary polycythaemia with haemoglobin 18 g/dL, packed cell volume (PCV) 53. Hematologist advised to maintain her PCV below 55.  She was advised regular follow-up at antenatal, hamatology and cardiology outpatient departments. At 29 and 32 weeks of gestation the patient had complaints of headache, dizziness, and myalgia. The PCV was 65 and 68. After haematology opinion isovolumic phlebotomy was done. At 34 weeks of gestation she was advised admission in view of intrauterine growth restriction but she did not get admitted. At 38.5 weeks she went into spontaneous labour and was transferred to cardiac intensive care unit. Infective endocarditis prophylaxis was given. Epidural analgesia with fentanyl 25 mcg and 0.1% bupivacaine 6 ml was given. She progressed uneventfully and forceps application was done to cut short second stage of labour. She delivered a female baby weighing 1.8 kg with Apgar score of 9/10. Intravenous diuretics were specifically avoided postdelivey to maintain preload to systemic ventricle. She was monitored in cardiac intensive care unit and discharged on seventh day after delivery. She was counselled to avoid further pregnancy and advised to follow up 6 weeks after delivery in cardiology OPD for Rastelli Repair. Her husband was counselled for vasectomy.

Discussion

The incidence of cardiac disease is less than 1 % in pregnancy. TGA is a one of the critical congenital cyanotic heart disease. TGA can be of 2 types: d – TGA, also called as complete TGA, is the most common type. In this the aorta arises from the morphological right ventricle (RV), and the pulmonary artery arises from the morphological left ventricle (LV), the “d-” refers to the dextro position of the bulboventricular loop. The other type is l-TGA, also called as congenitally corrected TGA, in which the right atrium enters the left ventricle, which gives rise to the pulmonary artery, and the left atrium communicates with the right ventricle, which gives rise to the aorta. Patients who have some route for oxygenated blood to enter from left to right side of heart like VSD and pulmonary vascular disease may reach adulthood without corrective surgery due to Eisenmenger physiology. In cyanotic heart disease, chronic hypoxia causes increased erythropoiesis which leads to increased red cell mass (Polycythaemia) and in turn increased oxygen carrying capacity of blood and heamatocrit (viscosity). Increased viscosity negates oxygen carrying capacity. Phlebotomy is to be done only if symptoms of hyperviscosity such as headache, dizziness, fatigue, chest pain, myalgia, paraesthesiae, depression are present.[4] TGA is associated with common cardiovascular complication during pregnancy such as arrhythmia, deterioration of NYHA class, thromboembolism, endocarditis, heart failure and obstetrical complications such as preeclampsia and eclampsia, premature rupture of membranes, preterm labour, prolonged second stage of labor, postpartum haemorrhage and small for gestation age infants ,fetal and  perinatal mortality.[5]   Risk of CHD recurrence in infant is <1%.[3] .Low oxygen saturation <85% is associated with low live births< 12%.
It is important to conduct proper preconception counselling regarding risk of pregnancy to her health, risk of inheriting the defect and skilful management throughout pregnancy and delivery.[6] Function of right ventricle and degree of tricuspid regurgitation plays a significant role in predicting the increased volume overload and leads to irreversible right ventricle dilatation.[7] Periodic follow up with 2D ECHO in third trimester is done to look for worsening in systemic ventricular function; increasing atrioventricular valve or tricuspid regurgitation. In this case d-TGA was picked up in early twenties and was not surgically corrected till delivery. Surgical procedures like atrial (Mustard and Senning procedure) and arterial switch, which are reserved for first few months of life couldn’t be done hence Rastelli procedure was advised. Rastelli procedure is done when d-TGA coexists with a large subaortic VSD and pulmonary stenosis. A patch is placed to direct blood from the left ventricle to the aorta through the VSD. The pulmonary valve is over sewn, and continuity is established between the RV and the pulmonary artery by means of a valved conduit. This is a case where uncorrected d -TGA was successfully managed conservatively in tertiary care centre with multidisciplinary approach and advised surgical correction after delivery.

References

1.      Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39:1890–1900.
2.      Ferencz C, Rubin JD, Loffredo CA, Epidemiology of Congenital Heart Disease: The Baltimore-Washington Infant Study, 1981–1989. Mount Kisco, NY: Futura Publishing Company; 1993.
3.      Warnes CA. Transposition of great arteries. Circulation. 2006;114:2699-2709
4.      Kontras SB, Bodenbender JG, Craenen J. Hyperviscosity in congenital heart disease. J Paediatr 1970;76:214–220
5.      Siu SC, Colman JM, Sorensen S, Smallhorn JF, Farine D, Amankwah KS, et al. Adverse cardiac and neonatal outcomes are more common in pregnant patients with cardiac disease Circulation. 2002;105:2179-2184.
6.      Cardiac disease in pregnancy. ACOG technical bulletin number 168--June 1992. Int J Gynaecol Obstet.1993;41(3):298–306
7.      Guedes A, Mercier LA, Leduc L, Berube L, Marcotte F, Dore A. Impact of pregnancy on the systemic right ventricle after a Mustard operation for transposition of the great arteries. J Am Coll Cardiol. 2004;44:433– 437.

Citation

Poonia S, More V, Satia MN. Uncorrected d-transposition of great arteries in pregnancy. JPGO 2014 Volume 1 Number 7 Available from: http://www.jpgo.org/2014/07/uncorrected-d-transposition-of-great.html