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.
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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