Clin Res Cardiol (2013) 102:215222
DOI 10.1007/s00392-012-0522-5
ORIGINAL PAPER
Women with congenital heart disease: long-term outcomes
after pregnancy
A. Wacker-Gumann M. Thriemer
M. Yigitbasi F. Berger N. Nagdyman
Received: 22 June 2012 / Accepted: 29 October 2012 / Published online: 21 November 2012
Springer-Verlag Berlin Heidelberg 2012
Abstract
Background Pregnancy in women with congenital car-
diac disease is more frequent due to an increased lifespan
and improved health situations. However, the long-term
outcomes in these women are not known.
Methods We analysed 267 consecutive pregnant women
with congenital heart defects who were seen at the German
Heart Centre Berlin. This retrospective study included
analysis of long-term follow-up data after pregnancy and
standard maternal cardiac, obstetric and neonatal out-
comes. The long-term data (n = 103) were acquired with a
self-assessment questionnaire from each patient. The main
primary outcomes of the study included functional class,
health, work capability and physical activity.
Results The median age of the patients at delivery was
27 years (range 1743 years). The median follow-up of all
patients was 11 years (range 149 years). Twenty-four
percent exhibited complex cardiac defects. Primary long-
term outcomes included good health in 61 % of the
patients. Approximately 68 % worked, and 76 % engaged
in physical activity. Thirty-three percent of the women who
answered the questionnaire demonstrated a decrease in
functional class during pregnancy, but more than two-
thirds of these patients subsequently improved. Secondary
short-term outcomes included a 4 % miscarriage rate and a
A. Wacker-Gumann (&)
M. Yigitbasi
F. Berger

N. Nagdyman
Department of Congenital Heart Disease and Pediatric
Cardiology, Deutsches Herzzentrum Berlin,
Augustenburger Platz 1, 13353 Berlin, Germany
e-mail: annette.wacker@med.uni-tuebingen.de
M. Thriemer
Department of Neonatology, University Childrens Hospital,
Calwerstr.7, 72076 Tubingen, Germany
4 % induced abortion rate. The maternal cardiac data
revealed that 30 % of the patients lost at least one func-
tional class during pregnancy. Onset arrhythmias were
observed in 12 % of the patients. The most prevalent
neonatal complication was premature birth, which was
present in 12 % of the neonates.
Conclusion Two-thirds of the patients tolerated preg-
nancy without cardiovascular complications. Most patients
displayed good long-term health, work capability and
physical activity outcomes. Further prospective controlled
studies are necessary to confirm these results and safely
advise pregnant women.
Keywords Adult congenital heart disease

Pregnancy outcomes
Long-term survival
Introduction
Congenital heart defects are the most common birth defects
in humans [1]. The number of children with congenital
heart disease who survive to adulthood has increased over
the last decade due to advances in paediatric and inter-
ventional cardiology, intensive care medicine and cardiac
surgery [2]. Therefore, an increasing number of female
patients with congenital heart disease reach reproductive
age [3].
Pregnancy changes the cardiovascular system due to an
increase in blood volume, which impacts even a healthy
womans physiological function. Cardiovascular risk pro-
files are often underestimated [4]. Residual lesions from
corrected or uncorrected congenital heart disease exert
additional effects on the mother and newborn. Therefore,
obstetric, maternal cardiac and neonatal complications are
more prevalent in these patients [5, 6].
123
216
Drenthen and colleagues identified 48 primarily retro-
spective publications of pregnancies in patients with con-
genital cardiac disease (CCD). Obstetric risk factors
included a miscarriage rate of 15 % and an abortion rate of
5 %. The most frequently reported cardiac complications
were heart failure (4.8 %) and arrhythmias (4.5 %). The
neonatal complications primarily included premature
delivery (12 %) and small size for gestational age (14 %)
[5, 6]. The incidence of congenital heart disease in the
offspring was 8 %. In summary, in many women with
congenital heart disease, pregnancy is well tolerated in the
short-term. However, maternal complications are reported
in 12 % of patients.
Limited data on the general health, work capability
and physical activity exist in the long term after preg-
nancy. These data are very important for the medical
surveillance of these patients and the assessment of the
mothers well-being after pregnancy. This knowledge
would be valuable to patients who are considering
another pregnancy. Therefore, this study assessed func-
tional class during and after pregnancy. Furthermore, we
analysed a self-assessment of health, work capability and
physical activity during the longest recorded possible
follow-up period.
Methods
Data collection was completed in 2010. The medical charts
of 267 consecutive pregnant women with CCD who pre-
sented to the German Heart Centre Berlin were retrospec-
tively reviewed by a specialized team.
Baseline parameters during pregnancy were recorded for
all patients. Obstetric data included miscarriage and abor-
tion rates and the mode of delivery. Cardiac data included
functional class, arrhythmia, thromboembolic events and
hypertension. Neonatal data included prematurity, size
with respect to gestational age, congenital heart disease and
offspring mortality (defined as [20 weeks of gestational
age and \1 year postnatal).
Long-term data were collected by mail using a self-
administered nine-item questionnaire. This questionnaire
has been used successfully in adults with congenital heart
disease [7]. Each item was phrased clearly and rated on a
three- to four-point scale. The questionnaires assessed
functional class, health, work capability and physical
activity. Patient functional status was determined using
Perloffs classification [8]. Furthermore, the curtailing of
daily life activities and work capability was evaluated.
Popular and professional physical activities were reported
as frequencies per week, and patients were also asked about
their interest in physical activity. The missing answers of
each patient were registered.
123
Clin Res Cardiol (2013) 102:215222
The ethics committee of the German Heart Centre Berlin
approved the research protocol, and patients received
information about the aim of the study. The subjects par-
ticipated in the study after providing informed consent.
Data analysis
SPSS 18.0 for Windows (IBM) was used for statistical anal-
ysis. Descriptive analysis was used for most analyses.
A Pearsons Chi-squared test was used to analyse the inde-
pendence of two categorical variables. P values \0.05 were
regarded as statistically significant. User-defined missing
values were treated as missing. The statistics were based on all
cases with valid data in the specified range for all variables.
The results refer to the last pregnancy because these data
were the most complete.
Results
Patient characteristics
The median patient age at delivery was 27 years (range
1743 years). Six percent of the women were older than
35 years.
The cohort was stratified by cardiac lesion type. Strati-
fication by cardiac type has been performed in adults with
congenital heart disease previously [7], and this grouping
has been adapted to the New England Regional Infant
Cardiac Program [9]. The patient population was divided
into three groups using a further simplified modification:
Recirculation defects
Atrial septal defect (n = 106), silent ductus arteriosus
(n = 5), atrioventricular septal defect (n = 14) and ven-
tricular septal defect (n = 25);
Valve defects and right/left outflow tract obstructions
Valve defects (n = 29), aortic coarctation (n = 14), left
ventricular outflow tract obstruction (n = 7), and right
ventricular outflow tract obstruction (n = 2); and
Complex cardiac defects
Tetralogy of Fallot (n = 24), transposition of the great
arteries (n = 15), complex heart lesions (i.e., single ven-
tricle) (n = 13), moderate Ebsteins aberration (n = 6),
pulmonic valve atresia (n = 5), and truncus arteriosus
communis (n = 2).
The baseline characteristics of all patients are docu-
mented in Table 1.
216
Drenthen and colleagues identified 48 primarily retro-
spective publications of pregnancies in patients with con-
genital cardiac disease (CCD). Obstetric risk factors
included a miscarriage rate of 15 % and an abortion rate of
5 %. The most frequently reported cardiac complications
were heart failure (4.8 %) and arrhythmias (4.5 %). The
neonatal complications primarily included premature
delivery (12 %) and small size for gestational age (14 %)
[5, 6]. The incidence of congenital heart disease in the
offspring was 8 %. In summary, in many women with
congenital heart disease, pregnancy is well tolerated in the
short-term. However, maternal complications are reported
in 12 % of patients.
Limited data on the general health, work capability
and physical activity exist in the long term after preg-
nancy. These data are very important for the medical
surveillance of these patients and the assessment of the
mothers well-being after pregnancy. This knowledge
would be valuable to patients who are considering
another pregnancy. Therefore, this study assessed func-
tional class during and after pregnancy. Furthermore, we
analysed a self-assessment of health, work capability and
physical activity during the longest recorded possible
follow-up period.
Methods
Data collection was completed in 2010. The medical charts
of 267 consecutive pregnant women with CCD who pre-
sented to the German Heart Centre Berlin were retrospec-
tively reviewed by a specialized team.
Baseline parameters during pregnancy were recorded for
all patients. Obstetric data included miscarriage and abor-
tion rates and the mode of delivery. Cardiac data included
functional class, arrhythmia, thromboembolic events and
hypertension. Neonatal data included prematurity, size
with respect to gestational age, congenital heart disease and
offspring mortality (defined as [20 weeks of gestational
age and \1 year postnatal).
Long-term data were collected by mail using a self-
administered nine-item questionnaire. This questionnaire
has been used successfully in adults with congenital heart
disease [7]. Each item was phrased clearly and rated on a
three- to four-point scale. The questionnaires assessed
functional class, health, work capability and physical
activity. Patient functional status was determined using
Perloffs classification [8]. Furthermore, the curtailing of
daily life activities and work capability was evaluated.
Popular and professional physical activities were reported
as frequencies per week, and patients were also asked about
their interest in physical activity. The missing answers of
each patient were registered.
123
Clin Res Cardiol (2013) 102:215222
The ethics committee of the German Heart Centre Berlin
approved the research protocol, and patients received
information about the aim of the study. The subjects par-
ticipated in the study after providing informed consent.
Data analysis
SPSS 18.0 for Windows (IBM) was used for statistical anal-
ysis. Descriptive analysis was used for most analyses.
A Pearsons Chi-squared test was used to analyse the inde-
pendence of two categorical variables. P values \0.05 were
regarded as statistically significant. User-defined missing
values were treated as missing. The statistics were based on all
cases with valid data in the specified range for all variables.
The results refer to the last pregnancy because these data
were the most complete.
Results
Patient characteristics
The median patient age at delivery was 27 years (range
1743 years). Six percent of the women were older than
35 years.
The cohort was stratified by cardiac lesion type. Strati-
fication by cardiac type has been performed in adults with
congenital heart disease previously [7], and this grouping
has been adapted to the New England Regional Infant
Cardiac Program [9]. The patient population was divided
into three groups using a further simplified modification:
Recirculation defects
Atrial septal defect (n = 106), silent ductus arteriosus
(n = 5), atrioventricular septal defect (n = 14) and ven-
tricular septal defect (n = 25);
Valve defects and right/left outflow tract obstructions
Valve defects (n = 29), aortic coarctation (n = 14), left
ventricular outflow tract obstruction (n = 7), and right
ventricular outflow tract obstruction (n = 2); and
Complex cardiac defects
Tetralogy of Fallot (n = 24), transposition of the great
arteries (n = 15), complex heart lesions (i.e., single ven-
tricle) (n = 13), moderate Ebsteins aberration (n = 6),
pulmonic valve atresia (n = 5), and truncus arteriosus
communis (n = 2).
The baseline characteristics of all patients are docu-
mented in Table 1.
Clin Res Cardiol (2013) 102:215222 217

Table 1 Baseline characteristics of all patients before pregnancy 40

p=0.008
Characteristics Recirculation Valve Complex Total Groups:
defects defects cardiac 35
recirculation defects
(n = 150) and outflow defects
tract (n = 65) valve defects and outflow tract
30 obstructions
obstructions
(n = 52) complex defects
25

Percentage
Mean age 26 ? 4.1 27 ? 4.5 28 ? 4.8
(years) SD
20
Native defect (n) 90 22 9 121
Operation (n) 60 30 56 146
15
Surgically 58 30 49 137
corrected (n)
Palliative 2 0 7 9 10
treatment (n)
Digitalis (n) 2 1 12 15 5
b-blocker (n) 5 6 3 14
Anticoagulation 1 0 1 2 0
therapy (n) worse in FC arrhythmia thromboembolic hypertension
TIA (n) 0 0 1 1 events
Events
Cerebral insult (n) 1 0 5 6
Fig. 1 Maternal short-term outcome data on functional class,
arrhythmias, thromboembolic events and hypertension
Short-term outcome data
In these patients loss of functional class was related to the
Obstetric outcomes pregnancy itself.
Twelve percent (n = 31) of the patients had a docu-
The miscarriage rate in our study population was 4 %, and mented arrhythmia. Among these patients, 71 % did not
the induced abortion rate was 4 %. Sixty percent of the require medical treatment. These patients generally
abortions were performed because of maternal cardiac exhibited extrasystole. Nine patients required medical
complications. treatment during pregnancy. Seven of the nine arrhythmias
Twenty-seven percent of the newborns were delivered by that required treatment were supraventricular in origin,
Caesarean section (C-section), which is equal to the typical including one instance of WolffParkinsonWhite syn-
C-section rate in Germany (30 %) [10]. C-sections were drome that was diagnosed in a patient with a corrected
performed for the following reasons: 37 % for maternal Ebsteins anomaly. The remaining two patients exhibited
cardiac complications, 23 % for obstetrical reasons, 17 % the following complications: one patient with a surgically
for foetal complications and 23 % for unclear reasons. repaired aortic stenosis had a ventricular arrhythmia, and
one patient with tetralogy of Fallot without residual lesions
Maternal cardiac outcomes had a cardiac arrest with successful resuscitation immedi-
ately after C-section. Whether anaesthesia or congenital
Maternal cardiac outcomes of all patients In total, 78 % cardiac defect caused this event is not clearly known
(n = 209) of patients displayed uncomplicated pregnan- because resuscitation was performed at another hospital.
cies. No patients died during or immediately after preg- Thromboembolic events, cerebral insults and deep vein
nancy. The loss of functional class (30 %) and arrhythmia thrombosis were observed in five patients (2 %). Four
(12 %) were the primary maternal cardiac symptoms patients suffered from complex cardiac defects. Two of
(Fig. 1]. these patients exhibited a transposition of the great arteries,
Sixty-seven percent of the patients were functional class which was surgically corrected. One patient displayed
(FC) I prior to pregnancy, 32 % were FC II and 1 % were pulmonary valve atresia with a ventricular septal defect,
FC III. Patients were seen regular prior, during and within and one patient exhibited a single functional ventricle.
1 year after pregnancy. During pregnancy, 50 % of patients Both of these patients were palliative cases. One patient in
were FC I, 35 % were FC II, 13 % were FC III, and 2 % the recirculation defect group exhibited a native sinus
were FC IV. In total, 30 % of the patients lost at least one venous defect.
FC and 12 % lost two FCs. These findings correlated with Hypertension ([140/90 mmHg) was observed in 5 % of
the echocardiographic findings, except for five patients. the patients. In pregnancy, hypertension is mainly related

123
218 Clin Res Cardiol (2013) 102:215222

to endothelial dysfunction [11]. Hypertension, related to 80 Groups

the cardiac defect, was discovered in one patient with a patients without contraindications
native coarctation of the aortic arch. The diagnosis was 70
patients with contraindications
primarily determined during pregnancy. This patient had a
hypertensive crisis ([180/100 mmHg) during the peripar- 60
tum period.
50

Percentage
Maternal cardiac outcomes of patients
with contraindications 40

30
Sixteen of our patients became pregnant despite at least one
of the following contraindications [12]: FC C III (n = 7),
20
Eisenmenger disease (n = 3), moderate/severe pulmonary
hypertension (RVSP [ 50 mmHg) (n = 11), cyanosis
10
(SpO2 \ 80 %) (n = 8) and severe pulmonic valve steno-
sis (n = 1). Sixty-two percent of patients with contraindi-
0
cations were in the complex heart defect group. worse in FC arrhythmia thromboembolic hypertension
The miscarriage rate was 12 % in patients with contra- event
indications, and the induced abortion rate was 31 %. Events
Sixty-two percent of patients displayed a cardiovascular Fig. 2 Comparison of cardiac complications in patients with and
complication, which was defined as heart failure, arrhyth- without contraindications
mia, hypertension, a thromboembolic event or death within
1 year after pregnancy. 25
p=0.003
Half of the patients experienced neonatal complications,
Groups
such as prematurity, small size for gestational age and
20 recirculation defects
offspring mortality.
valve defects and outflowtract
Cardiac complications in patients with contraindications obstructions
were compared to patients without contraindications
Percentage

15 complex defects
(Fig. 2). Arrhythmia and hypertension were not observed
in patients with contraindications, but thromboembolic
events were identified in 12 % of patients with contrain- 10
dications and 1 % without contraindications. A reduction
in one FC was observed in 68 % of patients with contra-
5
indications and 24 % without contraindications.

Neonatal outcomes
Eighty-two percent of the neonates were delivered without
complications.
Premature birth (12 %) and small size for gestational
age were the most prevalent neonatal complications. Pre-
maturity was highest in the group with complex defects.
In addition, 15 of the preterm infants were too small for
their gestational age.
Congenital cardiac defects were observed in 3 % of the
newborns, but newborns were not routinely evaluated for
cardiac defects. The maternal defects included tetralogy of
Fallot (n = 1), Ebsteins anomaly (n = 1), transposition
of the great arteries (n = 1), pulmonary valve stenosis
(n = 1), coarctation of the aortic arch (n = 1) and atrial
(n = 3) and ventricular septal defects (n = 1). The off-
spring mortality rate was 1.1 % (Fig. 3).
0
preterm SGA preterm and SGA offspring
mortality
Neonatal outcome
Fig. 3 Neonatal short-term outcome data
Maternal long-term outcome data
158 patients were contacted by written questionnaire.
However, 4 % of the patients died in the interim, and 36 %
of the patients were lost to follow-up due to missing con-
tact data (marriage, movement, etc.). Sixty-five percent of
the patients who were contacted answered the question-
naire. 55 patients with a median age of 40 years (range
2167) refused to answer the questionnaire (complex
defects n = 12; valve defects/outflow tract obstructions
n = 9; recirculation defects n = 34).

123
Clin Res Cardiol (2013) 102:215222 219

Table 2 Characteristics of the patients who answered the 80

questionnaire Groups
Characteristics Complex Valve defects Recirculation 70
defects and outflow defects
recirculation defects
(n = 30) tract (n = 48)
obstructions
60 valve defects and outflow tract
(n = 25)
obstructions
Median age of patients 38 (2760) 40 (3157) 44 (2167) complex defects
(range in years) 50

Percentage
Antiarrhythmic 1 1 1
therapy (n)
40
Anticoagulation (n) 1 3 3
Medical treatment for 1 0 2
pulmonary arterial 30
hypertension (n)

20
Median follow-up of all patients was 11 years (range
149 years). Follow-up data was calculated with reference
10
to the last pregnancy.
85 % of the patients answered all questions. The char-
acteristics of this patient cohort are shown in Table 2. 0
healthy and healthy but not ill and not not answered
capable capable capable
Health and work capability Health and capability

Sixty-one percent of patients considered themselves heal-
thy and capable, and 28 % patients regarded themselves as
healthy but not completely capable. Six percent considered
themselves ill, and the remaining patients (5 %) did not
answer this question. Figure 4 demonstrates that patients
with recirculation and valve defects or outflow tract
obstructions assessed themselves as healthy and capable,
and only half of the patients with complex cardiac defects
considered themselves healthy.
Sixty-eight percent of the patients worked. Eighteen
percent worked but experienced impairment in their daily
life activities. Seven percent were unable to work and
experienced impairment in all daily life activities. One
percent required special care. In addition, 6 % of the
patients did not answer this question.
Physical activity
Twenty percent of patients performed regular physical
activity more than once per week (recirculation defects
n = 12, valve defects and outflow tract obstructions n = 5,
complex cardiac defects n = 4). Fifty-six percent of
patients performed physical activity at least casually (\1/
week).
No patients participated in competitive sports. Twenty
percent of the patients were uninterested in physical activ-
ity, and this answer was given by 33 % of patients with
complex cardiac defects in contrast to 10 % with recircu-
lation defects. Four percent did not answer this question.
Fig. 4 Health and capability during the long-term follow-up
Self-assessment of functional class
Thirty-three percent of the patients whose cardiac condi-
tion worsened during pregnancy answered the question-
naire (38 % recirculation defects, 26 % valve defects and
outflow tract obstructions, 36 % complex cardiac defects).
Seventy percent of the respondents regained their pre-
vious functional class or improved. Twenty-one percent of
patients were stable on the lower level, 6 % worsened, and
3 % did not answer the question.
Patients with functional class worsening included one
patient with a postoperative left ventricular outflow tract
obstruction who developed an arrhythmia and one patient
with an atrioseptal defect that was surgically corrected
10 years after delivery. The self-assessment of functional
class correlated with physical activity.
Death in the follow-up period
Ten patients died within the first 10 years after delivery
(median age 46 years). Sixty percent of the patients who
died exhibited complex cardiac defects. Two patients died
within 1 year after delivery. One patient with a double inlet
left ventricle and AP shunt worsened after delivery. This
patient refused a heart transplant despite severe heart
failure, and she died 3 months after delivery. The other
patient had an operated coarctation of the aortic arch and a

123
220
right ventricular outflow tract obstruction without residual
lesions. Her newborn died due to mitral valve stenosis and
coarctation of the aortic arch. The cause of the death was
not known.
Maternal cardiac outcomes in patients
with contraindications
Eighteen percent of patients with contraindications for
pregnancy died, and 57 % were lost to follow-up. Twenty-
five percent answered the questionnaire.
All of the patients with contraindications who answered
the questionnaire had pulmonary hypertension. Two
patients improved; one patient with pulmonary valve atresia
in a palliative situation improved from FC IV to FC II, and
one patient with a closed atrial septal defect improved from
FC III to FC I. One patient with a closed ventricular septal
defect declined from FC II to FC III. One patient with a
double inlet left ventricle did not answer this question.
Three patients considered themselves healthy but not
completely capable. One patient was healthy and capable.
Discussion
The main finding in this study was that most of the women
with congenital cardiac disease had uncomplicated
pregnancies.
The patient cohort was stratified by cardiac lesion type.
This stratification has been performed previously in adults
with congenital heart disease [7]. We simplified the cohort
to evaluate the short-term and long-term follow-up in
simpler and more complex cardiac defects.
The short-term outcome data for obstetric, maternal
cardiac and neonatal complications in our study are com-
parable to those of previously published studies [5, 6, 13].
The miscarriage rate was lower than in previous studies
[14], but patients with complex cardiac defects displayed
the highest miscarriage rate.
Outcome data on maternal cardiac complications indi-
cated that two-thirds of the patients had no cardiovascular
events [5, 6, 14]. In addition to previous published studies
we found, that the loss of functional class before and in
pregnancy was the main cardiac symptom. Most of these
patients exhibited complex defects. The reasons for dete-
rioration included a low ventricular function and pulmon-
ary hypertension. Burden of pregnancy side effects might
furthermore influenced the results, as some patients
(n = 5) felt worse compared to objective cardiac findings
(echocardiography, etc.).
Prematurity and small sizes for gestational age were the
main neonatal complications [15, 16]. Most premature
newborns and the newborns that were too small for their
123
Clin Res Cardiol (2013) 102:215222
gestational age were born to women with complex cardiac
defects. These neonatal issues were likely due to the
mothers higher haemoglobin and lower oxygen levels.
Furthermore, the reduced cardiac output in some of the
women might have led to reduced uteroplacental blood
circulation, which negatively influenced foetal accommo-
dation. Induced delivery due to maternal cardiac compli-
cations was another reason.
Two-thirds of the patients reported good outcomes in the
self-assessments of health, work capability and physical
activity in the long-term follow-up period. The health self-
assessment correlated with physical activity.
Thirty-three percent of the women who answered the
questionnaire displayed a decrease in functional class
during pregnancy, but more than two-thirds of these
patients subsequently improved. These outcome data were
certainly influenced by an improvement of the management
and surveillance of these rare defects in adults over the last
years. And, furthermore, patients adopt to the chronic heart
diseases which influenced the subjective perception. But as
physical activity correlated with the health self-assessment,
the overestimation of ones health, compared to clinical
assessments might be low. In addition, previous published
studies in adults with congenital heart disease demon-
strated a positive correlation of activity and exercise
capacity [17, 18].
Half of the patients with contraindications for pregnancy
exhibited cardiovascular complications, which may be due
to the presence of pulmonary hypertension in most patients
[19]. New advanced therapies for pulmonary arterial
hypertension and optimization of pharmacotherapy have
emerged in the past decade, and progress in high-risk preg-
nancy management has been made. However, maternal
mortality remains high [2023]. Another reason for cardio-
vascular complications may be the inability of these patients
to increase cardiac output, which leads to heart failure.
Hypercoagulability and decreased systemic vascular resis-
tance further increase these risks [24]. The results of cardiac
complications in women without contraindications were
compared to those of women with contraindications to
determine whether the complications in the general cohort
are primarily associated with the group with contraindica-
tions for pregnancy. Arrhythmia and hypertension were not
observed in patients with contraindications, but other com-
plications, such as thromboembolic events and a loss of
functional class, were frequent. Therefore, the general cohort
might be associated with a lower incidence of thromboem-
bolic events and have better outcomes interims of functional
class, excluding patients with contraindications.
For the long-term outcomes, 18 % of the patients with
contraindications died and 56 % were lost to follow-up.
Only 25 % of these patients answered the questionnaire.
All of these patients suffered from pulmonary hypertension.
Clin Res Cardiol (2013) 102:215222
The high risk of cardiovascular complications, poor neonatal
outcomes and poor long-term follow-up outcomes suggests
that advisement against pregnancy is justified due to com-
plications in pregnancy and the long-term health outcomes
in this group [20, 25, 26].
Several study limitations related to the retrospective
design must be considered. Medical approaches have
changed in the past decades, and these changes may have
influenced the results in many different aspects. Underre-
porting is possible because additional information might
have been lost. A control group and an objective evaluation
were also missing, but this was not possible due to the
historical manner. Another limitation may be the positive
selection in the long-term follow-up group because
only patients in good health may have answered the
questionnaire.
Despite these limitations, the study analysed long-term
outcomes after pregnancy in women with congenital car-
diac disease in a relatively large number of patients, which
has not been previously investigated.
Conclusion
Two-thirds of the patients tolerated pregnancy without
cardiovascular complications emphasizing the fact that
more than half of the women had simple cardiac defects as
it was a single-centre study. Most patients displayed good
long-term health, work capability and physical activity
outcomes, but lost to follow-up has to be considered.
In patients with contraindications, the high risk of car-
diovascular complications, poor neonatal outcomes and
poor long-term follow-up outcomes suggests that advise-
ment against pregnancy is justified due to complications in
pregnancy and the long-term health outcomes in this group.
Further prospective controlled long-term follow-up studies
are necessary to confirm these results and safely advise
pregnant women.
Acknowledgments We thank Mrs Julia Stein (M.Sc.) of the
Deutsches Herzzentrum Berlin for her advice in the statistical analysis
of this study. We also thank all of the women who supported the study
by answering the questionnaire.
References
1. Schwedler G, Lindinger A, Lange PE, Sax U, Olchvary J, Peters
B, Bauer U, Hense HW (2011) Frequency and spectrum of
congenital heart defects among live births in Germany: a study of
the Competence Network for Congenital Heart Defects. Clin Res
Cardiol 100(12):11111117
2. Khairy P, Ionescu-Ittu R, Mackie A, Abrahamovicz M, Pilote L,
Marelli AJ (2010) Changing mortality in congenital heart disease.
J Am Coll Cardiol 56:11491157
221
3. Moons P, Budts W, Costermans E, Huyghe E, Pieper PG,
Drenthen W (2009) Pregnancy related behaviour of women with
congenital heart disease: room for behavioural interventions.
Congenit Heart Dis 4:348355
4. Rademacher W, Lauten A, Lauten A, Ragoschke-Schumm A,
Figulla HR (2010) Postpartum unmasking of a severe triple-
vessel-disease with acute myocardial infarction. Clin Res Cardiol
99(7):463466
5. Drenthen W, Pieper PG, Roos-Hesselinek JW (2007) Outcome of
pregnancy in women with congenital heart disease: a literature
review. J Am Coll Cardiol 49:23032311
6. Drenthen W, Boersema E, Balci A, Moons P, Roos-Hesselink
JW, Mulder BJM, on behalf of the ZAHARA investigators et al
(2010) Predictors of pregnancy complications in women with
congenital heart disease. Eur Heart J 31:21242132
7. Wacker A, Kaemmerer H, Hollweck R, Hauser M, Deutsch MA,
Brodherr-Heberlein S, Eicken A, Hess J (2005) Outcome of
operated and unoperated adults with congenital cardiac disease
lost to follow-up for more than five years. Am J Cardiol
95:776779
8. Perloff JK, Child JS (1998) Congenital heart disease in adults,
2nd edn. WB Saunders Company, Philadelphia
9. Fyler DC, Buckely LP, Hellenbrand WE, Cohne HE, Kirklin JW,
Nadas AS, Cartier JM, Breitbart MH (1980) Report of the New
England regional infant cardiac program. Pediatrics 65:377461
10. Statistisches Bundesamt Deutschland (2010)
11. Karthikeyan VJ, Blann AD, Baghdadi S, Lane DA, Gareth
Beevers D, Lip GY (2011) Endothelial dysfunction in hyperten-
sion in pregnancy: associations between circulating endothelial
cells, circulating progenitor cells and plasma von Willebrand
factor. Clin Res Cardiol 100(6):531537
12. Baumgartner H, Bonhoeffer P, De Groot NM, de Haan F,
Deanfield JE, Galie N, Task Force on the Management of Grown-
up Congenital Heart Disease of the European Society of Cardi-
ology (ESC); Association for European Paediatric Cardiology
(AEPC) et al (2010) ESC Guidelines for the management of
grown-up congenital heart disease (new version 2010). Eur Heart
J 31:29152957
13. Siu SC, Colman JM, Sorensen S, Smallhorn JF, Farine D,
Amankwah KS et al (2002) Adverse neonatal and cardiac
outcomes are more common in pregnant women with cardiac
disease. Circulation 105:21792184
14. Karamlou T, Diggs BS, McCrindle BW, Welke KF (2011)
A growing problem: maternal death and peripartum compli-
cations are higher in women with grown-up congenital heart
disease. Ann Thorac Surg 92:21932198
15. Gelson E, Curry R, Gatzoulis MA, Swan L, Lupton M, Steer P
et al (2011) Effect of maternal heart disease on fetal growth.
Obstet Gynecol 117:886891
16. Josefsson A, Kernell K, Nielsen NE, Bladh M, Sydsjo G (2011)
Reproductive patterns and pregnancy outcomes in women with
congenital heart diseasea Swedish population-based study.
Acta Obstet Gynecol Scand 90:659665
17. Lemmer J, Heise G, Rentzsch A, Boettler P, Kuehne T, Dubowy
KO, Peters B, Lemmer B, Hager A, German Competence Net-
work for Congenital Heart Defects (2011) Right ventricular
function in grown-up patients after correction of congenital right
heart disease. Clin Res Cardiol 100(4):289296
18. Muller J, Hess J, Hager A (2012) Daily physical activity in adults
with congenital heart disease is positively correlated with exer-
cise capacity but not with quality of life. Clin Res Cardiol 101(1):
5561
19. European Society of Gynecology; Association for European
Paediatric Cardiology; German Society for Gender Medicine;
Authors/Task Force Members, Regitz-Zagrosek V, Blomstrom
Lundqvist C, Borghi C, Cifkova R, Ferreira R, Foidart JM,
123
222
Gibbs JS, Gohlke-Baerwolf C, Gorenek B, Iung B, Kirby M,
Maas AH, Morais J, Nihoyannopoulos P, Pieper PG, Presbitero P,
Roos-Hesselink JW, Schaufelberger M, Seeland U, Torracca L;
ESC Committee for Practice Guidelines, Bax J, Auricchio A,
Baumgartner H, Ceconi C, Dean V, Deaton C, Fagard R, Funck-
Brentano C, Hasdai D, Hoes A, Knuuti J, Kolh P, McDonagh T,
Moulin C, Poldermans D, Popescu BA, Reiner Z, Sechtem U,
Sirnes PA, Torbicki A, Vahanian A, Windecker S; Document
Reviewers, Baumgartner H, Deaton C, Aguiar C, Al-Attar N,
Garcia AA, Antoniou A, Coman I, Elkayam U, Gomez-Sanchez
MA, Gotcheva N, Hilfiker-Kleiner D, Kiss RG, Kitsiou A,
Konings KT, Lip GY, Manolis A, Mebaaza A, Mintale I, Morice
MC, Mulder BJ, Pasquet A, Price S, Priori SG, Salvador MJ,
Shotan A, Silversides CK, Skouby SO, Stein JI, Tornos P,
Vejlstrup N, Walker F, Warnes C (2011) ESC Guidelines on the
management of cardiovascular diseases during pregnancy: the
Task Force on the Management of Cardiovascular Diseases
during Pregnancy of the European Society of Cardiology (ESC).
Eur Heart J 32(24):314797
20. Bedard E, Dimopoulos K, Gatzoulis MA (2009) Has there been
any progress made on pregnancy outcomes among women with
pulmonary arterial hypertension? Eur Heart J 30:256265
123
Clin Res Cardiol (2013) 102:215222
21. Jas X, Olsson KM, Barbera JA, Blanco I, Torbicki A, Peacock A,
Vizza CD et al (2012) Pregnancy outcomes in pulmonary arterial
hypertension in the modern management era. Eur Respir J
40(4):881885
22. Seyfarth HJ, Halank M, Wilkens H, Schafers HJ, Ewert R, Riedel
M, Schuster E, Pankau H, Hammerschmidt S, Wirtz H (2010)
Standard PAH therapy improves long term survival in CTEPH
patients. Clin Res Cardiol 99(9):553556
23. Franke J, Wolter JS, Meme L, Keppler J, Tschierschke R,
Katus HA, Zugck C (2012) Optimization of pharmacotherapy in
chronic heart failure: is heart rate adequately addressed? Clin Res
Cardiol [Epub ahead of print]
24. Pieper PG, Hoedermis ES (2011) Pregnancy in women with
pulmonary hypertension. Neth Heart J 19:504508
25. Almange C (2002) Pregnancy and congenital heart disease. Arch
Mal Coeur Vaiss 95:10401044
26. Regitz-Zagrosek V, Gohlke-Barwolf C, Geibel-Zehender A,
Haass M, Kaemmerer H, Kruck I, Nienaber C (2008) Heart
diseases in pregnancy. Clin Res Cardiol 97:630665