A Pregnancy with Corrected Tetralogy of Fallot, What Should

Be Done?

Riza Sufriadi

Mohd. Andalas

, Novita

Obstetrics and Gynecology Department, Syiah Kuala University /Dr.Zainoel Abidin Hospital Banda Aceh, Indonesia

Cardiology Department, Syiah Kuala University-dr. Zainoel Abidin Hospital Banda Aceh, Indonesia

Keywords: Tetralogy of Fallot, Intra Uterine Growth Restriction, Cesarean Section, Vaginal Delivery.

Abstract : The incidence of pregnancy in women with cardiovascular disease is rising. Tetralogy of Fallot (ToF) is the

most common form of cyanotic congenital heart defect. Surgical results after repair of Tetralogy of Fallot

have remained excellent for the last decades, the number of women with ToF reaching reproductive age

following successful repair is increasing. If ToF repaired well, good cardiac function: vaginal delivery is

preferred, and labor induction is safe. Cesarean delivery is limited to obstetrical indication or severe cardiac

lesion. Case Report: The 32-year-old patient G5P1A3 came to the Ante Natal Care clinic of Zainoel Abidin

General Hospital at 40-41 weeks' gestation. The last patient controls at the clinic at 7 months of gestation.

The last ultrasound examination results postdate pregnancy, Intra Uterine Growth Restriction (IUGR), less

amniotic fluid, no any sign of labor. Patient had a history of congenital heart disease known as ToF since

childhood. Patient hasn’t any subjective complaints about heart disease. Patient had received ToF correction

in 2013 and didnot regular checkups in cardiology. Patient has done cesarean section, born female baby

with birth weight 1800 grams, Apgar score 8/9.

1 INTRODUCTION

The prevalence of women with congenital heart disease

increases over time. According to estimates by the

European Society of Cardiology about 1600 patients with

congenital heart disease in the UK enter adolescence each

year, improved medical and surgical management allows

more patients to reach adulthood. More advanced

diagnostic tools allow for early diagnosis with better

sensitivity and accuracy (Deanfield J,2003). Tetralogy of

Fallot (ToF) is a congenital heart disease consisting of

ventricular septal defect (VSD) subaortic membranous

type, overriding aorta, infundibular pulmonary stenosis

(PS) with or without PS valvular and right ventricular

hypertrophy. When accompanied by ASD is called the

pentalogy of fallot (Diller et al,2005). TOF events

represent 10% of cases of congenital heart disease (CHD)

and are the most common cause of cyanotic CHD (Rauch

R,2010). The mortality rate in patients who did not

undergo surgery increased gradually, ranging from 30% at

age 2 years to 50% at 6 years of age. The highest mortality

rate in the first year and then remain constant until the

second decade. No more than 20% of TOF patients can

live up to age 10 and <5-10% of patients live at the end of

the second decade. Most people who survive until age 30

have congestive heart failure (CHF) (Pillutla P,2009). It is

the largest left-to-left shunt, due to obstruction of blood

flow to the lungs where pulmonary vascular resistance is

normal. If this disorder is not corrected, a pregnancy can

be achieved but maternal mortality is still high, and the

fetal loss rate is over 50 percent. After surgery correction

of total defects, maternal mortality is not clear beyond that

of women without heart disease; the chance of offspring to

get heart disease is about 5-10 percent (Anwar.B, 2004).

In a study by Veldtman (2004) founded infants were small

for their gestational age, 86% being born to women with

unrepaired TOF. The fetus was at highest risk when the

mother had a combination of obstetric risk factors and

cardiac risk factors. We were able to demonstrate a

negative correlation between infant birth weight and

maternal unrepaired TOF status, morphologic PA

abnormality, higher RV systolic pressure, and younger age

at primary surgical repair. Unrepaired TOF and the

presence of morphologic PA abnormality (hypoplastic or

disconnected PA or ductal origin of PA) were

independently associated with infant birth weight. Among

infants with low birth weight, 50% of their mothers have

chronic disease that unfavourably affects maternal

placental blood ﬂow. One can speculate that PA

abnormalities, particularly in the presence of pulmonic

Sufriadi, R., Andalas, M. and Novita, .

A Pregnancy with Corrected Tetralogy of Fallot, What Should Be Done?.

DOI: 10.5220/0008792102190222

In Proceedings of the 2nd Syiah Kuala International Conference on Medicine and Health Sciences (SKIC-MHS 2018), pages 219-222

ISBN: 978-989-758-438-1

219

regurgitation, may adversely affect augmentation of

maternal cardiac output at rest or during exercise. This

may result in depressed placental blood ﬂow and,

subsequently, intrauterine growth retardation (Veldtman

GR, 2004.). About 10% of women with a history of ToF

correction have short-term cardiovascular complications

during pregnancy. Supraventricular and ventricular

arrhythmias are well-known long-term sequelae of

intracardiac repair of ToF. The use of cardiac medication

before pregnancy strongly predicted cardiovascular events.

The incidence of SGA is higher than in the general

population. The premature birth rate also was higher.

However, the long-term effects of pregnancy are poorly

researched Balci (2011) about 10% of women with a

history of ToF correction have short-term cardiovascular

complications during pregnancy. Supraventricular and

ventricular arrhythmias are well-known long-term

sequelae of intracardiac repair of ToF. The use of cardiac

medication before pregnancy strongly predicted

cardiovascular events. The incidence of SGA is higher

than in the general population. The premature birth rate

also was higher. However, the long-term effects of

pregnancy are poorly researched (John Lynn Jefferies,

2016).

2 CASE REPORT

The 32-year-old pregnant women with G5P1A3 came to

the Antenatal Care clinic of Zainoel Abidin Hospital at 40-

41 weeks' gestation. The last patient controls at the clinic

at 7 months of gestation, last ultrasound examination

results in polyclinic were said postdate pregnancy, Intra

Uterine Growth Restriction (IUGR), less amniotic fluid.

The patient does not sign of labor or Premature rupture of

membranes. Fetal movement is felt active. The history of

flour albous is recognized, but not itchy and odorless.

Fever during pregnancy is refused. Micturition and

defecation within normal limits. Patients had a history of

congenital heart disease known as ToF. Patients had

received TOF correction in 2013. Menstrual history within

normal limits. Patients had abortion 3 times (1, 2 and 3

pregnancies), 2-year-old child with BBL 2,500 grams,

born with Cesarean section due to transverse lie

presentation.

Examination of vital signs within normal limits with

normal nutritional status. Blood pressure was 120/80, pulls

80 beat/ min and no murmur or gallop. Laboratory

examination within normal limits. The Echocardiography

results: mild atrial regurgitation, ejection fraction 45%,

septal dyskinetic: corresponded to Chronic Heart Failure,

in general good clinical condition, and tolerance of

cesarean section with moderate risk. Obstetric findings: no

dilatation of cervix with head presentation, uterus was not

contractions, fundal height 25 cm. Ultrasound examination

obtained a single live fetus head percentage with

gestational age 40-41 weeks, estimated fetal weight 1813

gram, intra uterine growth restriction accompanied by

oligohydramnios. Cardiotocography finding was

reassuring with baseline 145 beat/minute, good variability

and no decelerations. Patient has done cesarean section,

born female baby with birth weight 1800 gr, Apgar score

8/9.

3 DISCUSSIONS

The prevalence of women with congenital heart disease

increases over time. According to estimates by the

European Society of Cardiology about 1600 patients with

congenital heart disease in the UK enter adolescence each

year, improved medical and surgical management allows

more patients to reach adulthood. More advanced

diagnostic tools allow for early diagnosis with better

sensitivity and accuracy (Deanfield J,2003).

Nowadays, over than 60th year since the first

successful intracardiac repair of tetralogy of Fallot (ToF).

The repair largely consists of ventricular septal defect

closure and the relief of variable forms of right ventricular

(RV) obstruction, and usually resulting in free pulmonary

insufficiency (PI). Early in the experience with TOF

repair, attention was on quantity of life (palliative).

Palliative shunts were widely used to permit repair at a

safer, older age. The dividend from a full relief of

obstruction included excellent function for decades for

patients formerly suffering from morbid or lethal disease

(Bichell, 2014). This case report explained about the

women 32 years old had a history of congenital heart

disease known as ToF. Patients had received ToF

correction in 2013. Echocardiography results: mild atrial

regurgitation, ejection fraction 45%, septal dyskinetic,

corresponded to Chronic Heart Failure, in general good

clinical condition, and tolerance of cesarean section with

moderate risk. This patient has been successful repaired of

tetralogy of fallot and good functional status was classified

in a NYHA classes I and II. Improved survival of patients

with congenital heart disease now means that there are

more adults than children with repaired congenital disease

in developed countries. Medium-term survival of repaired

tetralogy of Fallot (rToF) has been documented to be

good, with 20-year survival rates at or above 90% (Dennis

M, 2017).

Women with corrected ToF have risk of

cardiovascular and obstetric events during pregnancy.

Most events are well treatable. The incidence of offspring

events is markedly increased. Cardiovascular and

offspring outcomes are strongly related with the use of

cardiac medication before pregnancy. Surgical status

before pregnancy also appears to predict pregnancy

outcome. This patient had successful repaired of tetralogy

of fallot and good functional status was classified in as

women in NYHA classes I and II. Improved survival of

patients with congenital heart disease now means that

there are more adults than children with repaired

congenital disease in developed countries. Medium-term

survival of repaired tetralogy of Fallot (rToF) has been

documented to be good, with 20-year survival rates at or

above 90% (Dennis M, 2017).

SKIC-MHS 2018 - The 2nd Syiah Kuala International Conference on Medicine and Health Sciences

220

The 32-year-old patient G5P1A3 came to the clinic at

40-41 weeks' gestation. The last patient controls at the

clinic at 7 months of gestation. Patient with bad history of

antenatal care, has been abortion 3 times (1, 2 and 3

pregnancies). Higher prevalence of miscarriage relevant

with research by Balci at al: from 157 pregnancies in 74 of

these patients with post corrected ToF, 19% ended in a

miscarriage and 2,5% in an elective abortion (Balci,2011).

Its similar correlation with research by Pedersen at al; that

outcomes of pregnancy, and fertility, in a series of women

who underwent surgery for tetralogy of fallot, prevalence

of spontaneous abortion is 15% (Pedersen LM, 2008

Aug;18).

During normal pregnancy and delivery, there are

dramatic alterations in cardiovascular physiology.

Systemic vascular resistance falls, blood volume increases,

cardiac output increases secondary to increased heart rate

and stroke volume, and a physiological left ventricular

hypertrophy occurs. Pregnancy in unrepaired TOF carries

a major risk of maternal complications, including heart

failure, arrhythmia and endocarditis, which can give rise to

fetal problems including miscarriage and preterm labour.

The risk is particularly high when the average systemic

oxygen saturation falls below 85%. In repaired tetralogy

of Fallot (rTOF), the risk of pregnancy is dependent on the

degree of residual haemodynamic impairment. When a

good repair has been achieved, pregnancy is usually well

tolerated in the absence of pregnancy complications such

as pre-eclampsia. However, in women with residual

shunts, right ventricular outﬂow obstruction and/or right

ventricular dysfunction, the increased overload volume of

pregnancy can lead to heart failure and arrhythmias.

(Veldtman GR, 2004.). We not founded adverse of cardiac

events in this patient such as arrythmia.

Ultrasound examination obtained a single live fetus

head percentage with gestational age 40-41 weeks,

estimated fetal weight 1813 gram, intrauterine growth

restriction accompanied by oligohydramnios.

Cardiotocography finding was baseline 145, good

variability and no decelerations. In pregnancies with

cardiovascular events, significantly smaller for gestational

age children were born. In this case, ultrasound

examination results Intra Uterine Growth Restriction, low

infant birth weight was related to the maternal state of

women who had not undergone reparative surgery or to

morphologic pulmonary artery abnormalities. In a research

infant who were small for gestational age, 71% were born

to women with untreated TOF (Child JS, 2004). The

incidence of SGA (19%) is also higher than in the general

population although it is lower than the 35% recently

mentioned by Gelson et al. The use of maternal cardiac

medication before pregnancy was the most important

predictor of offspring outcome. Maternal hemodynamic

abnormalities as well as direct effects of maternal

cardiovascular medication may undermine placental blood

flow and induce placental insufficiency with subsequent

intrauterine growth restriction resulting in children born

SGA as well as in premature birth. The strong association

between maternal cardiovascular events and SGA points

in this direction. Palliative surgery before correction

appears to influence offspring outcome negatively. Some

neonatological outcomes were high mortality percentage,

partially due to prematurely born babies. It was also

notices that new-borns were born with low body weight

for their age which was closely related to frequency of

negative cardiovascular outcome during pregnancy which

can lead to hemodynamic changes and placenta

insufficiency as a result (Balci,2011).

Relief from pain and apprehension is important.

Although intravenous analgesics provide satisfactory pain

relief for some women, continuous epidural analgesia is

recommended for most the major problem with

conduction analgesia is maternal hypotension is especially

dangerous in women with intracardiac shunts in whom ow

may be reversed. Blood passes from right to left within the

heart or aorta and thereby bypasses the lungs. Hypotension

can also be life-threatening if there is pulmonary arterial

hypertension or aortic stenosis because ventricular output

is dependent on adequate preload. In women with these

conditions, narcotic conduction analgesia or general

anesthesia may be preferable. (Cunningham, 2018)

For vaginal delivery in women with only mild

cardiovascular compromise, epidural analgesia given with

intravenous sedation often succes. is has been shown to

minimize intrapartum cardiac output uctuations and allows

forceps or vacuum-assisted delivery. Subarachnoid

blockade is not generally recommended in women with

significant heart disease. For cesarean delivery, epidural

analgesia is preferred by most clinicians with caveats for

its use with pulmonary arterial hypertension. Finally,

general endotracheal anaesthesia with thiopental,

succinylcholine, nitrous oxide, and at least 30-percent

oxygen has also proved satisfactory (Cunningham, 2018).

Heart rate, stroke volume, cardiac output, and mean

arterial pressure increase further during labor and in the

immediate postpartum period and should be monitored

closely. Fluid intake and output and pulse oximetry

readings should also be carefully reviewed. Lateral

positioning and adequate pain control can reduce maternal

tachycardia and increase cardiac output. There is no

consensus on intrapartum invasive hemodynamic

monitoring, but women with New York Heart Association

class III or IV disease may be candidates. Operative

assistance with the second stage of labor is recommended

to decrease maternal cardiac work. The immediate

postpartum period is especially critical for the patient with

cardiovascular disease. Blood loss must be minimized, and

blood pressure maintained, but congestive failure from

fluid overload must also be avoided (John, T Queeman,

Catherine Y.Spong, Charles J.Lockwood, 2015).

Based on Simpson review (2012) recommends

cesarean delivery for women with the following: (1)

dilated aortic root >4 cm or aortic aneurysm; (2) acute

severe congestive heart failure; (3) recent myocardial

infarction; (4) severe symptomatic aortic stenosis; (5)

warfarin administration within 2 weeks of delivery; and

(6) need for emergency valve replacement immediately

after delivery. Although we agree with most of these, we

have some caveats (Cunningham, 2018). The indication of

Caesarean section in this patient by Obstetric indication.

A Pregnancy with Corrected Tetralogy of Fallot, What Should Be Done?

221

4 CONCLUSSIONS

Vaginal delivery is preferred, and labor induction is

usually safe with collaborating care by multiple specialist.

Caesarean delivery is limited to obstetrical indications,

and considerations are given for the specific cardiac lesion

or inavailability of teamwork and general support

facilities.

REFERENCES

Agarwal N, Gupta M, Singh N, et al. Successful

Management of Pregnancy in Uncorrected Tetralogy

of Fallot with Pulmonary Atresia. Journal of

Obstetrics and Gynaecology of India. 2015;65(6):417–

9, n.d.

Al-Aqeedi RF, Alnabti A, Al-Ani F, et al. Successful

Delivery by a Cesarean Section in a Parturient with

Severe Dilated Cardiomyopathy an Implantable

Cardioverter Defibrillator and a Repaired Tetralogy of

Fallot. Heart Views. 2011 Jan-Mar;12(1):26–31, n.d.

Anwar, B. Kehamilan dan Penyakit Jantung. Universitas

Sumatera Utara. 2004, n.d.

Balci A, Drenthen W, Mulder BJ, et al. Pregnancy in

women with corrected tetralogy of Fallot: occurence

and predictors of adverse events. Am Heart J. 2011

Feb;161(2):307–13, n.d.

Balci, A. e. a., 2011. Pregnancy in women with corrected

tetralogy of Fallot: Occurrence and predictors of

adverse events. American Heart Journal , Volume ,

Issue , , 161( 2), pp. 307 - 313.

Bichell, D. P., 2014. Fourth Decade After Repair of

Tetralogy of Fallot. ahajournals.org, 130(Circulation),

pp. 1931-1932.

Cassater D, et al. The effects of pregnancy on right

ventricular remodeling in women with repaired

tetralogy of Fallot. Int J Cardiol 2013;168:1847–1852,

n.d.

Cauldwell, M. et al. Effect of Pregnancy on Ventricular

Aortic Dimensions in Repaired TOF. JAHA. 2017

10(16):1-16, n.d.

Child and JS, a. a., 2004. Fallot’s Tetralogy and

Pregnancy;Prognostication and Prophesy. Journal of

the American College of Cardiology, 44,(1), pp. 181-

Chiu SN, Wu MH, Su MJ, et al. Coexisting

mutations/polymorphisms of the long QT syndrome

genes in patients with repaired Tetralogy of Fallot are

associated with the risks of life-threatening events.

Hum Genet. 2012 Aug. 131(8):1295-304, n.d.

Cunningham, F. G., 2018. Williams obstetrics. New York:

McGraw-Hill.

Deanfield J, Thaulow E, Warnes C, Webb G, Kolbel F,

Hoffman A, et al. Management of grownup congenital

heart disease. Eur Heart J. 2003; 24:1035–84, n.d.

Dennis M, M. B. K. I. e. a., 2017. Adults with repaired

tetralogy: low mortality but high morbidity up to

middle age. Open Heart , 4:(000564), p. e000564..

Diller GP, Kempny A, Liodakis E, et al. Left ventricular

longitudinal function predicts life-threatening

ventricular arrhythmia and death in adults with

repaired tetralogy of fallot. Circulation. 2012 May 22.

125(20):2440-6, n.d.

John Lynn Jefferies, 2016. Mode of Delivery and

Pregnancy Outcome in Women with Congenital Heart

Disease. PLoS ONE, p. 11(12).

John, T Queeman, Catherine Y.Spong, Charles

J.Lockwood, 2015. Protocol for high risk

Pregnancies: evidence based approach, sixth edition.

oxford UK: Wiley Blackwell.

Meah VL, Cockcroft JR, Backx K, Shave R, Stohr EJ.

Cardiac output and related haemodynamics during

pregnancy: a series of meta-analyses. Heart.

2016;102:518–526, n.d.

Meijer JM, P. P. D. W. e. a., 2005. Pregnancy, fertility,

and recurrence risk in corrected tetralogy of Fallot.

heart, 91(6), pp. 801-805..

Pedersen LM, 2008 Aug;18. Outcomes of pregnancy in

women with tetralogy of Fallot. Cardiol Young.,

4(Epub 2008 Jun 18), pp. 423-9.

Pillutla P, Shetty KD, Foster E. Mortality associated with

adult congenital heart disease: Trends in the US

population from 1979 to 2005. Am Heart J. 2009 Nov.

158(5):874-9 , n.d.

Rauch R, Hofbeck M, Zweier C, et al. Comprehensive

genotype-phenotype analysis in 230 patients with

tetralogy of Fallot. J Med Genet. 2010 May.

47(5):321-31, n.d.

Sudoyo AW, Setiyohadi B, Alwi I, Simadibrata M, Setiati

S (Ed). Buku Ajar Ilmu Penyakit Dalam. Ed. 6, Jakarta

: InternaPublishing, 2015, n.d.

Veldtman GR, 2004.. Outcomes of pregnancy in women

with tetralogy of Fallot. J Am Coll Cardio, Volume

44, p. 174–80.

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