Original Article

World Journal for Pediatric and

Congenital Heart Surgery
Pregnancy in Patients with Tetralogy of 1(2) 170-174
ª The Author(s) 2010
Reprints and permission:
Fallot: Outcome and Management sagepub.com/journalsPermissions.nav
DOI: 10.1177/2150135110369685
http://pch.sagepub.com

Harpreet Kaur, MD1, Vanita Suri, MD1, Neelam Aggarwal, MD1,

Seema Chopra, MD1, Rajesh Vijayvergiya, MD, DM2, and
K. K. Talwar, MD, DM2

Abstract
The objective was to evaluate pregnancy outcome in women with tetralogy of Fallot, including impact of corrective cardiac
surgery on pregnancy outcome in a tertiary care referral hospital. The study was a retrospective analysis of maternal and
perinatal outcome in all women with tetralogy of Fallot treated in a cardio-obstetric unit during 1996–2008. Ten women had
21 pregnancies. Of the 10 women, 7 with uncorrected tetralogy of Fallot had 16 pregnancies. Obstetric and cardiac complications
were more frequent in the uncorrected group (70% vs 40% and 40% vs nil, respectively). The frequency of spontaneous abortion
and preterm birth was greater in the uncorrected group (37.5% vs nil and 25% vs nil, respectively). The percentage of babies who
were small for gestational age was 40% in the uncorrected group and 20% in the corrected group. Tetralogy of Fallot carries sub-
stantial risk to mother and fetus. Surgical correction is associated with improved maternal and perinatal outcome. These patients
need detailed prepregnancy evaluation and should be under joint supervision of an obstetrician, a cardiologist, a congenital cardiac
surgeon, and an anesthetist.

Keywords
tetralogy of Fallot, pregnancy outcome, cardiac surgery

Submitted December 26, 2009; Accepted March 7, 2010.

Heart disease constitutes a leading nonobstetric cause of
maternal mortality. Although the prognosis for pregnant
women with heart disease has improved, it still presents a
high-risk pregnancy.1 Recent advances in pediatric cardiology
and cardiac surgery have enabled increasing numbers of
women with congenital heart disease (CHD) to survive into
their childbearing years.2 The most frequently encountered
form of cyanotic CHD in adults is tetralogy of Fallot (TOF),
constituting 10% of cases of CHD and 75% of cases of cyanotic
CHD. Corrective surgery for TOF now permits more than 85%
of such children to survive into adulthood. Pregnancy in unop-
erated women with TOF is reported to have an associated
maternal mortality rate of 3% to 12% as well as a high rate
of perinatal loss (up to 30%). But pregnancy outcome in
women who have undergone reparative surgery is reported to
be nearly the same as for the normal population.3,4 The clinical
presentation of TOF depends upon the severity of right ventri-
cular outflow obstruction.5 There is an increased risk of mater-
nal and fetal complications in women with TOF, especially in
those with uncorrected lesions. Fetal risks include spontaneous
abortion, intrauterine growth restriction (IUGR), prematurity,
perinatal mortality, and congenital heart disease in the fetus.
The frequency of many of these complications increases with
the degree of maternal cyanosis, maternal complications, and
need for anticoagulation.6 Maternal complications include risk
of postpartum hemorrhage and thromboembolism, risk of para-
doxical embolism, congestive cardiac failure (CCF), infective
endocarditis, and arrhythmias. All obstetric and cardiac com-
plications are more likely to occur in patients with uncorrected
lesions, making their management challenging. We present our
data of 21 pregnancies in 10 women with TOF, of which 7
women with uncorrected TOF had 16 pregnancies.
Methods
A retrospective analysis of maternal and perinatal outcome of
pregnancies in women with TOF who were seen in the
cardio-obstetrics unit of Obstetrics and Gynecology at Nehru
Hospital, PGIMER, Chandigarh, India during 1996–2008 was
1
Department of Obstetrics and Gynaecology, Postgraduate Institute of
Medical Education and Research (PGIMER), Chandigarh, India
2
Department of Cardiology, Postgraduate Institute of Medical Education and
Research (PGIMER), Chandigarh, India
Corresponding Author:
Harpreet Kaur, PGIMER, Chandigarh, India 160012
Email: drharpreet_sidhu@hotmail.com, husanmeet@yahoo.co.in

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Kaur et al 171

Abbreviations and Acronyms (SGA), and deliveries between 28 and 37 weeks were consid-
ered preterm. All women with TOF who were known to have
APH antepartum hemorrhage become pregnant were included in the study, including those
CCF congestive cardiac failure in whom early termination of pregnancy had occurred. Preg-
CHD congenital heart disease nancy was confirmed with urine pregnancy test and ultrasono-
CVA cerebrovascular accident
graphy. Main outcome measures analyzed were maternal
Hct hematocrit
cardiac complications (CCF, syncope, arrhythmias, infective
IUGR intrauterine growth restriction
endocarditis, cerebrovascular accident), obstetric complica-
LB liveborn
tions (antepartum hemorrhage, postpartum hemorrhage, IUGR,
LVEF left ventricle ejection fraction
MBW mean birth weight
spontaneous abortion, preterm birth), and perinatal outcome
NND neonatal death (live birth, stillbirth, neonatal loss within 28 days of birth, pres-
PA pulmonary artery ence of congenital malformations).
PFO pulmonary flow obstruction
PIH pregnancy-induced hypertension Results
PPH postpartum hemorrhage
RV right ventricle During the study period there were 960 deliveries in women
RVOT right ventricle outflow tract with heart disease, of which 233 deliveries occurred in women
RVOTO right ventricular outflow tract with CHD. Among these, 20 had cyanotic CHD, accounting for
obstruction 8.6% of women with CHD. In the cyanotic group there were 10
SB stillborn women with TOF who had 21 pregnancies. Of these 10 women
SGA small for gestational age of TOF, 9 were registered in the cardio-obstetric clinic and
TOF tetralogy of Fallot 1 was referred in the immediate postpartum period.
VSD ventricular septal defect Table 1 shows a profile of these patients. Age at the time of
pregnancy ranged between 20 and 30 years. Three women had
undergone corrective cardiac surgery prior to index pregnancy.
carried out. Ten women with TOF were identified. All were In 3 women of 7 in the uncorrected group, heart disease was
supervised in the cardio-obstetric clinic under joint supervision diagnosed during index pregnancy only. For the entire group
of obstetrician and cardiologist. Cardiac diagnosis was estab- of 10 women, mean hematocrit was 35.3% (range, 27%-
lished by electrocardiogram and echocardiography. Antenatal 45%). All women without previous surgical correction had
visits were every 2 to 3 weeks until 30 weeks and weekly there- history of dyspnea and cyanosis.
after until delivery. At each visit, fetal growth was assessed and As shown in Table 2, among the obstetrical complications,
maternal assessment of functional grading was done according there was 1 case of pregnancy-induced hypertension in a
to the 1964 criteria of the New York Heart Association. Sever- woman in the corrected group who had gestational hyperten-
ity of the cardiac lesion was assessed by detailed clinical exam- sion at term. There were 4 cases of IUGR in the uncorrected
ination aided by echocardiography. At each visit, women were group and 1 in the corrected group. Four women in the uncor-
advised about the importance of adequate rest, adequate super- rected group had antepartum hemorrhage (APH) including
vision, and oral iron supplementation and were advised to 1 case of placenta previa and 3 case of abruption. Two women
report in case of any worsening of cardiac status. In the third in the uncorrected group had traumatic postpartum hemor-
trimester, hospitalization was advised in the presence of wor- rhage, which included vaginal wall hematoma following
sening of cardiac status, in severe disease, or in the presence forceps delivery and cervical tear. Maternal cardiac complica-
of obstetric indications. Pregnancies were allowed to continue tions were seen only in the uncorrected group, including 1 case
until term, and induction was done for obstetric indications. of syncope at term leading to abruption and intrauterine death,
Otherwise, planned delivery at term was allowed. Prophylaxis 1 case of cerebrovascular accident and left basal ganglia infarct
for infective endocarditis was given to all women during labor. at 28 weeks, and 2 cases of congestive cardiac failure in the
Parenteral analgesia was used for pain relief during labor, and postpartum period. No patient had infective endocarditis or
epidural analgesia was used selectively (3 women). Second arrhythmias.
stage was cut short by forceps/ventouse to avoid cardiac over- Analysis of pregnancy outcomes revealed that the rate of
load related to the Valsalva maneuver. These women were kept spontaneous abortion was 37.5% and preterm delivery 25% in
under observation post partum in an intensive monitoring area. the uncorrected group. In the corrected group there were 100%
Pregnancy outcome, cardiac and obstetric complications, peri- term births. In 1 woman in the uncorrected group, the maternal
natal outcome, and delivery characteristics were noted and diagnosis of TOF was made at 6 weeks gestation. Elective
these were compared in corrected and uncorrected groups. termination of pregnancy was advised because of the severe
Maternal cardiac deterioration, admission to the cardiac care degree of right ventricular outflow obstruction, but the patient
unit, and maternal mortality were noted. All neonates with birth chose to continue the pregnancy. The subsequent course included
weight more than 2 standard deviations below the mean for severe IUGR and oligohydramnios. Induction of labor was done
gestational age were classified as small for gestational age at 34 weeks gestation, resulting in a live baby weighing 800 g.

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172 World Journal for Pediatric and Congenital Heart Surgery 1(2)

Table 1. Patient Profiles (N ¼ 10)

Patient Age, Time of Hct

No. y Parity Corrected/Not Diagnosis (%) Echocardiography Findings

1 22 1 Not corrected Index 36 VSD with 50% aortic override, LVEF 64%, severe pulmonary
pregnancy stenosis
2 20 1 Not corrected Index 36 Large (14 mm) VSD with 50% aortic override, RV thickened with
pregnancy doming
3 22 1 Not corrected Prior to 33 TOF with good-sized PA, normal LVEF
pregnancy
4 23 2 Not corrected Prior to 36 Large VSD with 50% aortic override, RVOT gradient 104 mm Hg,
pregnancy 3 major aortopulmonary collaterals seen
5 28 2 Not corrected Prior to 27 Large (15 mm) subaortic VSD with bidirectional flow, 50% aortic
pregnancy override, severe RVOTO
6 29 2 Not corrected Prior to 45 Large (20 mm) VSD with 40% aortic override, RVOT gradient
pregnancy 78 mm Hg, normal LV function
7 25 2 Not corrected Index 33 TOF with good-sized PA, normal LVEF
pregnancy
8 32 2 Corrected Prior to 38 RA/RV mildly dilated, no residual VSD/RVOTO, LVEF normal
pregnancy
9 22 1 Corrected, patch closure, Prior to 39 No residual RVOTO/VSD
pulmonary valvotomy pregnancy
10 26 2 Corrected, VSD patch closure, Prior to 30 No residual RVOTO/VSD
infundibular resection, pregnancy
closure of small PFO

Hct, hematocrit; LVEF, left ventricle ejection fraction; PA, pulmonary artery; PFO, pulmonary flow obstruction; RV, right ventricle; RVOT, right ventricle outflow
tract; RVOTO, right ventricular outflow tract obstruction; VSD, ventricular septal defect.

Table 2. Pregnancy Complications

Obstetric Cardiac

IUGR PIH APH PPH Total CCF Syncope CVA Total

Uncorrected (n ¼ 10)a 4 (40)b 0 4 (40)b 2 (20) 7 (70) 2 (20) 1 (10) 1 (10) 4 (40)
Corrected (n ¼ 5) 1 (20) 1 (20) 0 0 2 (40) 0 0 0 0

APH, antepartum hemorrhage; CCF, congestive cardiac failure; CVA, cerebrovascular accident; IUGR, intrauterine growth restriction; PIH, pregnancy-induced
hypertension; PPH, postpartum hemorrhage. Table values are n (%). Total no. of pregnancies ¼ 21; uncorrected ¼ 16a, corrected ¼ 5.
a
6 women had spontaneous abortion.
b
3 women had both APH and IUGR.

Rate of vaginal delivery was 80% in the uncorrected group Discussion

(two thirds being preterm deliveries) and 20% in the corrected
group. The rate of cesarean section was higher in the corrected
group (80% vs 20%). Cesarean sections were done for obstetrical
indications: fetal distress in 2 women, nonprogress of labor in 1,
fetal malpresentation in 1 in the corrected group, and placenta pre-
via and heart disease per se in 1 woman in the uncorrected group.
Perinatal Outcome
In the corrected group there were 100% term live births. In the
uncorrected group the rate of preterm birth was 40% and SGA
was 40%. There was 1 stillbirth and 1 neonatal death in the
uncorrected group. Mean birth weight (2.17 kg vs 1.62 kg) was
higher in corrected group (Table 3). No baby was recognized as
having a congenital anomaly, including congenital cardiac
malformations.
Cyanotic heart disease during pregnancy carries a risk of
maternal and perinatal complications. Hemodynamic changes
of pregnancy may cause clinical deterioration due to increase
in right to left shunt and cyanosis. These volume shifts are
increased at the time of delivery, and sudden blood loss may
exaggerate these changes. Obstetrical hemorrhage, if it occurs,
will put more strain on an already compromised heart and sud-
den blood loss can lead to further increase in right-to-left shunt
and cyanosis with its attendant complications. Poor prognostic
factors in TOF include maternal hematocrit greater than 60%,
oxygen saturation less than 80%, and clinical history of synco-
pal attacks.4,7 Pregnancy in these women carries a high risk of
spontaneous abortion, preterm birth, and low birth weight.3 The
potential predictors of maternal morbidity include functional
class, ventricular dysfunction, significant arrhythmias, cyano-
sis, severe outflow tract obstruction, pulmonary hypertension,

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Kaur et al
Table 3. Perinatal Outcome (N ¼ 15)
LB, n (%) SB, n (%) Preterm, n (%)
Uncorrected (n ¼ 10) 9 (90) 1 (10) 4 (40)
Corrected (n ¼ 5) 5 (100) 0 0
Total 14 1 4
LB, liveborn; MBW, mean birth weight; NND, neonatal death; SB, stillborn; SGA, small for gestational age.
and need for anticoagulation.3,7 Pregnancy in corrected TOF is
comparable to that in the general population,8,9 but each patient
needs to be individually assessed for the ability to tolerate
hemodynamic changes during pregnancy. Corrective cardiac
surgery improves both maternal and perinatal outcome by
improving hemodynamic parameters, improving placental per-
fusion, and reducing the likelihood of cardiac complications.
Correction of cyanosis is associated with improved birth
weight and higher rate of term births. Various studies have
shown pregnancy outcome to be improved following corrective
surgery.3,4 Patients who have total correction of TOF generally
tolerate pregnancy well with only minimally increased risk to
mother and infant. Patients who have only palliative proce-
dures still carry a significantly increased risk of cardiac dete-
rioration during their pregnancies. The greatest risk, however,
is carried by the untreated group.5 In our series, pregnancy out-
come was better in the corrected group, although the number of
subjects was very small, making it impossible to draw any
statistically significant conclusions. Veldtman et al3 studied
43 women with TOF who had 112 pregnancies, of which
82 (73%) were successful. Among these 43 women, 8 women
had unrepaired TOF at the time of their 20 successful pregnan-
cies; 5 of these 8 were cyanotic at the time of their 12 pregnan-
cies. Of 43 women, 16 patients had spontaneous abortion
(27%) and 1 stillbirth at term (a mother with unrepaired TOF).
Of the 7 infants who were SGA, 5 (71%) were born to women
with unrepaired TOF. Of the 30 spontaneous abortions, 3
occurred in 2 women with unrepaired TOF.3 Meijer et al10
described 63 pregnancies in 29 patients with corrected TOF.
There were 13 instances of spontaneous abortion (20.6%) and
50 successful pregnancies. Of the 29 women in this series, 6
women (12%) developed cardiac complications. Eight children
(17%) were small for gestational age, 5 (11%) were born pre-
mature, and 2 (4.3%) had CHD (1 with TOF).
Given the potential for complications associated with
pregnancy, women with TOF should be counseled prior to preg-
nancy. During preconception counseling, risk factors should be
assessed. Whenever possible, heart defects should be surgically
corrected before conception. Antenatal care should be a coordi-
nated approach including an obstetrician, a cardiologist, a
perinatologist, and an anesthetist. Besides needing routine
antenatal care, these patients require more frequent antenatal
examination, restriction of physical activity, iron supplementa-
tion, periodic arterial blood gas analysis, and fetal surveillance.
Planned delivery should be the aim to optimize intrapartum care.
In the absence of specific obstetric indications, vaginal delivery
is preferred over caesarean section. Second stage should be cut
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173
SGA, n (%) NND, n (%) MBW, kg
4 (40) 1 (10) 1.62 + 0.66
1(20) 0 2.17 + 0.32
5 1
short by application of forceps/vacuum.2,11 Intrapartum oxygen
therapy and continuous maternal and fetal monitoring are
required. Endocarditis prophylaxis should be given and care taken
to avoid sudden hypotension.2,7,11
Surgical repair of TOF has a low perioperative mortality,
and survivors are much improved symptomatically and are able
to cope adequately with demands of pregnancy.8 In a review of
58 pregnancies in 41 women with repaired TOF, Singh et al8
reported that pregnancy outcome for both mother and fetus is
greatly improved with surgical correction prior to pregnancy:
these authors reported a live birth rate of 78% versus 33%,
maternal mortality 0% versus 4% to 12%, and rate of conges-
tive cardiac failure 2% versus 40%. In a study of 9 pregnancies
in patients with TOF reported by Shime et al,4 7 had undergone
prior corrective surgery. Two women had uncorrected TOF.
Their pregnancies resulted in 1 elective abortion and 1 preterm
birth. All 6 continuing pregnancies in women with corrected
TOF went to term with only 1 SGA infant. One woman in this
group had CCF.4 Surgery during pregnancy carries substantial
risk of complications (maternal mortality rate of 5% and peri-
natal mortality up to 30%). Thus, surgery during pregnancy is
generally not advised but if absolutely necessary can be done
after 16 weeks. For symptomatic patients in early pregnancy,
elective termination and subsequent surgical repair can be
offered.2,4
Maximum hemodynamic changes can occur during the
postpartum period, and patients should be monitored in an
intensive care unit. Prior to discharge, the relative risks of birth
control measures, sterilization procedures, and future pregnan-
cies should be discussed.2,7,12
Limitations
Certain limitations are inherent in any retrospective analysis.
The major limitation of this study is the small number of
patients. This precludes the possibility of reaching any statisti-
cally significant conclusions. In addition, we have no details
regarding serial arterial blood gas measurement or objective
parameters for diagnosis of cardiac failure individually in every
patient. Still, this single-center cohort study gives an overview
of our experience regarding management of pregnant patients
with TOF in a large obstetric referral center.
Conclusions
Because maternal morbidity and mortality from other causes
has decreased, the relative importance of cyanotic heart disease
173
174
in pregnancy is increasing. TOF carries high risk to mother and
fetus, and surgical correction improves maternal and perinatal
outcome. Because these patients are at risk for complicated
problems, we emphasize the critical importance of comprehen-
sive prepregnancy evaluation, the benefit of corrective surgery
prior to pregnancy, and the role of effective contraception.
These pregnancies should be monitored under joint supervision
of an obstetrician, a cardiologist, a congenital cardiac surgeon,
and an anesthetist. Intensive monitoring during intrapartum and
postpartum periods is required.
Declaration of Conflicting Interests
The author(s) declared no conflicts of interest with respect to the
authorship and/or publication of this article.
Funding
The author(s) received no financial support for the research and/or
authorship of this article.
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