Abstract

Aims

To determine pregnancy outcome and risk factors for adverse events in women with congenital heart disease (CHD) and residual haemodynamic right ventricular (RV) outflow tract (RVOT) lesions.

Methods and results

Pregnancy outcome data for women with CHD and residual RVOT lesions have been recorded since 2001. There were 76 pregnancies in 47 women that continued beyond 24 weeks gestation. At conception 20% had RVOT obstruction, 32% had pulmonary regurgitation (PR) and 49% had mixed RVOT obstruction and PR. Moderate-to-severe PR was present in 30 (39%) and RVOT obstruction ≥30 mmHg in 12 (16%) of pregnancies. Seven pregnancies (9%) were complicated by right heart failure (RHF). No arrhythmias were documented. Predictors for RHF were moderate-to-severe PR in combination with at least one additional risk factor (twin pregnancy, branch pulmonary artery stenosis, RV systolic dysfunction, RV hypertrophy). All patients responded to diuretic treatment and had a good pregnancy outcome without foetal complications.

Conclusion

In patients with CHD and residual RVOT lesions, the outcome of pregnancy is good. Patients with moderate-to-severe PR were at risk for symptomatic RHF only if additional risk factors were present. When treated by a multidisciplinary team, maternal and foetal outcome was good. The general recommendation that pulmonary valve replacement should be undertaken prior to pregnancy in patients with moderate-to-severe PR and RV dilatation needs to be reconsidered.

Introduction

Due to improvements in infant heart surgery and cardiology care, the majority of patients born with congenital heart disease (CHD) now survive to adulthood.1 As a consequence, there is a new cohort of women with complex heart disease who wish to embark on pregnancy. There have been several reports of maternal outcome in CHD within heterogeneous patient populations, but outcome data in relation to specific haemodynamic residua are sparse and the numbers in lesion-specific outcome series are often small.2–10

Two of the commonest CHD lesions are tetralogy of Fallot (TOF) and valvar or subvalvar pulmonary stenosis (PS). The majority of patients will have undergone surgery in infancy or childhood, but these repairs were often palliative and many have important residual haemodynamic lesions of the right ventricular outflow tract (RVOT), either obstruction (RVOTO), pulmonary regurgitation (PR), or both. Over time these haemodynamic lesions cause pathophysiological changes including right ventricular hypertrophy (RVH), right ventricular (RV) dilatation and/or RV dysfunction. Although most pregnancy outcome series have focused on specific underlying CHD diagnostic groups, the spectrum of haemodynamic residua and individual pathophysiological responses to them may vary greatly, and it is these variables which are impacted by the cardiovascular changes associated with pregnancy, irrespective of surgical modifications or interventions.

The literature reports a good pregnancy outcome for those without severe residual RVOTO or PR, but for those with significant residua or those surgically uncorrected, pregnancy is often considered to be contraindicated.2–10 Pre-conceptual counselling and risk assessment in women with heart disease takes into account several factors, in particular the severity of residual haemodynamic lesions, ventricular function, functional class, and co-morbidity, rather than the original congenital diagnosis per se. The aims of our study were therefore to define pregnancy outcome based on the presence and severity of haemodynamic residua and define specific risk factors if any, for adverse events, to add to what is a small evidence base. With increasing experience and expertise in the management of these complex patients during pregnancy, recommendations for intervention prior to pregnancy needs to be continually reassessed, along with what might be potential risk markers for poor outcome.

Methods

Patients

All patients with a diagnosis of CHD and residual RVOT lesions cared for by a specialist multidisciplinary pregnancy team since 2001 at University College London Hospitals were identified from the maternal cardiology database. The multidisciplinary team (MDT) includes dedicated specialists as follows: one specialist grown-up CHD cardiologist, two obstetricians, one anaesthetist, one haematologist and a clinical nurse specialist. Pre-conceptual counselling by the Grown-up congenital heart disease (GUCH) cardiologist takes into account clinical assessment, functional class, haemodynamic residua, ventricular function, and co-morbidity. Residual RVOT lesions comprised RVOT obstruction and/or PR.

Follow-up during pregnancy

After conception all women are seen by the GUCH cardiologist at 14–16 weeks gestation. Thereafter follow-up frequency is individualized based on complexity and risk. At 32–34 weeks a detailed written delivery plan is provided for all patients after discussion with the MDT. Antenatal care is stratified to local or specialist centre care/delivery based on the pre-conception assessment and progress during pregnancy.

Transthoracic echocardiography data

For the purpose of this study, all echocardiograms, performed by two experienced CHD cardiac physiologists, were reviewed. Right ventricular measurements were recorded in the apical four-chamber, parasternal long-axis and parasternal short-axis views. The RV to left ventricular (LV) ratio was measured on the apical four-chamber view.11,12 Measurements were summated and normalized for body surface area for a global estimation of RV size. The RV systolic function was assessed by visual estimation and graded normal, mildly, moderately, or severely impaired. Pulmonary regurgitation was graded in severity grade I to grade III (I, mild; II, moderate; III, severe; as indicated by flow reversal in branch pulmonary arteries and pressure half-time <100 ms).13

Outcome measures

The rate of miscarriages and termination of pregnancy before 24 weeks of gestation was recorded. Maternal, obstetric, and neonatal outcomes were assessed. Cardiac complications included death, arrhythmias, heart failure, and hospitalisation for cardiac indication. Right heart failure (RHF) was defined as new elevation of the jugular venous pressure (JVP) (>4 cm above the sternal angle) + symptoms of decreased exercise tolerance ± dyspnoea (a common symptom in those with RVOT lesions due to abnormal interventricular interactions)14 ± pitting peripheral oedema.

Statistical analysis

Statistical analysis was performed using SPSS Version 17.0 (SPSS Inc., Chicago, IL, USA). Mean, standard deviation, median, and range were determined for continuous variables when appropriate; frequencies were determined for nominal variables. Histograms and the Shapiro–Wilk test were used to assess the distributions of continuous variables. For comparison between groups, Student's t-test, the nonparametric Mann–Whitney test, or Fisher's exact test were used as appropriate. A P < 0.05 (two-sided) was considered to be significant.

Results

Baseline characteristics

A total of 93 consecutive pregnancies in 50 patients with CHD and residual RVOT lesions were identified. Seventeen pregnancies ended before 24 weeks of gestation. There were 12 spontaneous miscarriages (in five patients) and 5 elective termination of pregnancy (in four patients). Mean gestational age was 10.3 ± 5.7 weeks and 12.6 ± 4.0 weeks, respectively. The mean age of women with spontaneous miscarriages/termination of pregnancy was significantly lower compared with those who had pregnancies that continued beyond 24 weeks (24.4 ± 5.4 years vs. 28.5 ± 5.5, P = 0.006), while there were no differences in other baseline characteristics.

Ten spontaneous miscarriages occurred in the first and two in the second trimester [both second trimester miscarriages were in the same woman (diagnosis—mild PS) due to cervical insufficiency]. Two patients had termination of unplanned pregnancies while on the waiting list for cardiac surgery (one with unrepaired TOF and one with severe PS).

There were no cardiac complications in the 17 pregnancies that ended before 24 weeks. The study population therefore consists of 76 pregnancies in 47 women that progressed beyond 24 weeks gestation (including one pregnancy with a preterm live born infant at 23 weeks of gestation). Baseline characteristics are shown in Table 1. Previous surgical procedures included isolated pulmonary valvotomy in 26% (including two pregnancies in a patient with previous balloon valvuloplasty), repair including a transannular patch in 17%, TOF repair without transannular patch in 12%, and an RV to pulmonary artery conduit in 28%. Two patients with conduits had undergone additional percutaneous pulmonary stent valve implantation. In 13 pregnancies (17%), no prior surgeries had been performed, 12 with underlying valvar PS, and 1 in a patient with unrepaired TOF. The median time that had lapsed between the last surgical procedure and delivery was 21 (1–37) years.

Table 1

Baseline characteristics in pregnancies that continued beyond 24 weeks of gestation

 Pregnancies (n = 76) 
Age (years)a 28.5 ± 5.5 
Multipara (%) 39 (51) 

 
Primary cardiac defect 
 Tetralogy of Fallota (%) 28 (37) 
 Pulmonary stenosis (%) 34 (45) 
 Pulmonary atresia (%) 5 (7) 
 Absent pulmonary valve syndrome (%) 9 (12) 

 
Residual functional lesions 
 Subvalvar/valvar pulmonary stenosis (%) 15 (20) 
 Pulmonary regurgitation (%) 24 (32) 
 Mixed pulmonary regurgitation/RVOT obstruction (%) 37 (49) 
 RVOT obstruction with systolic gradient ≥30 mmHg (%) 12 (16) 
 Branch pulmonary artery stenosis 6 (8) 
 Pulmonary regurgitation ≥ grade II (%) 30 (39) 
 Right ventricular dilatation (%) 35 (46) 
 Right ventricular systolic dysfunction (%) 8 (11) 
 Pregnancies (n = 76) 
Age (years)a 28.5 ± 5.5 
Multipara (%) 39 (51) 

 
Primary cardiac defect 
 Tetralogy of Fallota (%) 28 (37) 
 Pulmonary stenosis (%) 34 (45) 
 Pulmonary atresia (%) 5 (7) 
 Absent pulmonary valve syndrome (%) 9 (12) 

 
Residual functional lesions 
 Subvalvar/valvar pulmonary stenosis (%) 15 (20) 
 Pulmonary regurgitation (%) 24 (32) 
 Mixed pulmonary regurgitation/RVOT obstruction (%) 37 (49) 
 RVOT obstruction with systolic gradient ≥30 mmHg (%) 12 (16) 
 Branch pulmonary artery stenosis 6 (8) 
 Pulmonary regurgitation ≥ grade II (%) 30 (39) 
 Right ventricular dilatation (%) 35 (46) 
 Right ventricular systolic dysfunction (%) 8 (11) 

aIncluding one pregnancy in a women with unrepaired tetralogy of Fallot.

Residual RVOT haemodynamic lesions at the time of conception are summarised in Table 1. Moderate-to-severe PR was present in 30 (39%) and moderate-to-severe PS in 12 (16%) pregnancies. Moderate or severe RV dilatation was present in 19 (25%). Right ventricular systolic dysfunction was present in eight (11%) pregnancies. Two patients had mildly impaired LV systolic function. Concomitant branch pulmonary artery stenosis (BPS) was found in five patients with a total of six pregnancies. No patient had a history of stroke or heart failure and all were in sinus rhythm. Medical co-morbidity included bronchial asthma in nine, epilepsy in three, rheumatoid arthritis in one, and minor haematological disorders in four pregnancies (sickle cell trait in one and Thalassaemia trait in three). In one pregnancy, the patient was known to have 22q11 micro-deletion syndrome and Noonan syndrome in another.

Maternal outcome

Arrhythmias

In six pregnancies (9%), a prior history of sustained tachyarrhythmia (four supraventricular, two ventricular tachycardias) was noted; however, no arrhythmias were documented during pregnancy or post-partum.

Heart failure

In seven pregnancies (9%) (seven patients) symptomatic RHF developed. The characteristics of these seven pregnancies are outlined in Table 2. All seven patients had moderate-to-severe PR. Two had RV systolic dysfunction (mild1 and moderate1), four had RVH, and five had concomitant BPS. There were two multiple pregnancies with twins. All patients who developed RHF reported good exercise tolerance prior to pregnancy with objective exercise data available for six [Bruce protocol exercise test: exercise duration 10:51 ± 1:46 min, mean workload of 12.6 ± 2.45 metabolic equivalents (METS), range 10–16 METS]. Two patients were multiparous. One had two previous uneventful pregnancies (9 and 10 years earlier) but the severity of PR had increased in the intervening years, from grades I–II to grade III. The other had an uneventful pregnancy only 12 months prior when PR was grade II, but since then the severity had increased to grade III. The mean onset of symptoms of RHF was 26.9 ± 5.7 weeks gestation (range: 21–33 weeks). Prompt treatment with oral furosemide improved the signs and symptoms of RHF for five of the seven patients. Two patients, however, required hospital admission for intravenous diuretic therapy. One of these converted to oral furosemide and continued to term, while the other patient with a twin pregnancy underwent planned caesarean section at 32 weeks gestation for increasing symptoms and signs of RHF. The birth weights of her twins were 1620 and 1643 g. Both infants did well with no residual handicap at 18-month follow-up. All seven patients with RHF were assessed 1–5 months post-partum with clinical examination and echocardiography. All had returned to their pre-pregnancy functional status except one, who reported a decreased exercise capacity at 3 months follow-up. However, by 12 months post-partum she was again asymptomatic and completed 12:22 min of the Bruce protocol exercise test (unchanged from pre-pregnancy testing). Post-partum echocardiographic measurements were unchanged in all seven patients compared with baseline and at a mean post-partum follow-up of 26 ± 7.3 months, only one patient has subsequently undergone surgical pulmonary valve replacement (18 months after delivery).

Table 2

Characteristics of patients, who developed right heart failure during pregnancy

Patient Original diagnosis Type of corrective surgery Grade of pulmonary regurgitation Additional cardiac lesions Special obstetric conditions 
Tetralogy of Fallot Transannular patch repair III Mild right pulmonary artery stenosis, mild RVH, moderate RV systolic dysfunction  
Tetralogy of Fallot Pulmonary valvotomy, RVOT patch not crossing the valve ring III Mild left pulmonary artery stenosis, mild left ventricular systolic dysfunction  
Tetralogy of Fallot Transannular patch repair, subsequent RV–PA homograft conduit III Severe stenosis of left pulmonary artery  
Valvar pulmonary stenosis Pulmonary valvotomy III Restrictive RV physiology, RVH Twin pregnancy 
Tetralogy of Fallot Transannular patch repair, subsequent RV–PA homograft conduit III Occluded left pulmonary artery, RVH  
Valvar pulmonary stenosis Pulmonary valvotomy III – Twin pregnancy 
Valvar pulmonary stenosis, ventricular septal defect, left pulmonary artery from aorta Repair with RV–PA homograft conduit and reconnection of the left pulmonary artery to the main pulmonary artery II Right and left pulmonary artery stenosis, left-sided pulmonary hypertension, RVH, mild RV systolic dysfunction  
Patient Original diagnosis Type of corrective surgery Grade of pulmonary regurgitation Additional cardiac lesions Special obstetric conditions 
Tetralogy of Fallot Transannular patch repair III Mild right pulmonary artery stenosis, mild RVH, moderate RV systolic dysfunction  
Tetralogy of Fallot Pulmonary valvotomy, RVOT patch not crossing the valve ring III Mild left pulmonary artery stenosis, mild left ventricular systolic dysfunction  
Tetralogy of Fallot Transannular patch repair, subsequent RV–PA homograft conduit III Severe stenosis of left pulmonary artery  
Valvar pulmonary stenosis Pulmonary valvotomy III Restrictive RV physiology, RVH Twin pregnancy 
Tetralogy of Fallot Transannular patch repair, subsequent RV–PA homograft conduit III Occluded left pulmonary artery, RVH  
Valvar pulmonary stenosis Pulmonary valvotomy III – Twin pregnancy 
Valvar pulmonary stenosis, ventricular septal defect, left pulmonary artery from aorta Repair with RV–PA homograft conduit and reconnection of the left pulmonary artery to the main pulmonary artery II Right and left pulmonary artery stenosis, left-sided pulmonary hypertension, RVH, mild RV systolic dysfunction  

RVH, right ventricular hypertrophy; RV, right ventricle; RVOT, right ventricular outflow tract; RV–PA: right ventricular to pulmonary artery.

Predictors of right heart failure

A comparison of baseline characteristics in patients with and without RHF is given in Table 3. All patients with RHF had moderate (n = 1) or severe PR (n = 6). On univariate analysis, twin pregnancy and BPS were significantly associated with RHF and a trend was noted for RVH. For further analysis, we defined additional risk factors as the presence of one or more of the following: twin pregnancy, BPS, RVH, or RV systolic dysfunction. In those 30 pregnancies (39%) with moderate-to-severe PR at baseline, the mean number of risk factors was significantly higher in patients with symptomatic RHF (1.71 vs. 0.26 risk factors, P = 0.003). In patients with PR ≥ grade II, the presence of at least one risk factor was a pre-requisite for developing symptomatic RHF (Table 4). Right ventricular dimensions were not predictive for developing RHF in patients with moderate-to-severe PR.

Table 3

Comparison of baseline characteristics in patients with and without right heart failure during pregnancy

 Right heart failure (n = 7) No right heart failure (n = 69) P-value 
Baseline characteristics 
 Age at delivery (years) 27.2 ± 7.0 28.7 ± 5.4 0.5 
 Oxygen Saturation (%) 98.7 ± 1.1 97.9 ± 1.6 0.14 
 Twin pregnancy (%) 2 (29) 1 (1) 0.02 
 Multipara (%) 2 (29) 37 (54) 0.26 

 
Cardiac characteristics 
 Original cardiac diagnosis 
  Tetralogy of Fallot/pulmonary atresia/absent pulmonary valve syndrome (%) 5 (71) 37 (54) 0.69 
  Pulmonary stenosis (%) 2 (29) 32 (46) 
 Previous cardiac surgery (%) 7 (100) 56 (81) 0.59 
 Age at corrective surgery (years) 2.2 (2–17) 4.2 (0–17) 0.53 
 NYHA functional class 
  NYHA I (%) 3 (43) 45 (65) 0.24 
  NYHA II (%) 4 (57) 23 (33) 
 History of arrhythmia (%) 1 (14) 6 (9) 0.51 
  QRS width (ms) 132 (90–190) 133 (70–180) 0.42 
 Residual lesions at conception 
  Pulmonary regurgitation ≥ grade II (%) 7 (100) 23 (33) 0.002 
  Pulmonary valve stenosis ≥ 30 mmHg (%) 12 (17) 0.588 
  Right ventricular systolic dysfunction (%) 2 (29) 6 (9) 0.16 
  Tricuspid regurgitation ≥ grade 2 (%) 1 (14) 3 (4) 0.33 
  Right ventricular hypertrophy (%) 4 (57) 10 (14) 0.09 
  Branch pulmonary artery stenosis or occlusion (%) 5 (71) 1 (1) <0.001 
  Any risk factorsa (%) 7 (100) 14 (20) 0.001 
 Right ventricular dimensions on echocardiography 
  RVin/LVin 1.01 ± 0.11 0.95 ± 0.18 0.38 
  (RV-4CV + RV-PLAX + RVOT)/BSA (cm/m27.0 ± 0.8 6.2 ± 1.0 0.12 
 Right heart failure (n = 7) No right heart failure (n = 69) P-value 
Baseline characteristics 
 Age at delivery (years) 27.2 ± 7.0 28.7 ± 5.4 0.5 
 Oxygen Saturation (%) 98.7 ± 1.1 97.9 ± 1.6 0.14 
 Twin pregnancy (%) 2 (29) 1 (1) 0.02 
 Multipara (%) 2 (29) 37 (54) 0.26 

 
Cardiac characteristics 
 Original cardiac diagnosis 
  Tetralogy of Fallot/pulmonary atresia/absent pulmonary valve syndrome (%) 5 (71) 37 (54) 0.69 
  Pulmonary stenosis (%) 2 (29) 32 (46) 
 Previous cardiac surgery (%) 7 (100) 56 (81) 0.59 
 Age at corrective surgery (years) 2.2 (2–17) 4.2 (0–17) 0.53 
 NYHA functional class 
  NYHA I (%) 3 (43) 45 (65) 0.24 
  NYHA II (%) 4 (57) 23 (33) 
 History of arrhythmia (%) 1 (14) 6 (9) 0.51 
  QRS width (ms) 132 (90–190) 133 (70–180) 0.42 
 Residual lesions at conception 
  Pulmonary regurgitation ≥ grade II (%) 7 (100) 23 (33) 0.002 
  Pulmonary valve stenosis ≥ 30 mmHg (%) 12 (17) 0.588 
  Right ventricular systolic dysfunction (%) 2 (29) 6 (9) 0.16 
  Tricuspid regurgitation ≥ grade 2 (%) 1 (14) 3 (4) 0.33 
  Right ventricular hypertrophy (%) 4 (57) 10 (14) 0.09 
  Branch pulmonary artery stenosis or occlusion (%) 5 (71) 1 (1) <0.001 
  Any risk factorsa (%) 7 (100) 14 (20) 0.001 
 Right ventricular dimensions on echocardiography 
  RVin/LVin 1.01 ± 0.11 0.95 ± 0.18 0.38 
  (RV-4CV + RV-PLAX + RVOT)/BSA (cm/m27.0 ± 0.8 6.2 ± 1.0 0.12 

NYHA, New York Heart Association; RVin, right ventricular inflow diameter; LVin, left ventricular inflow diameter; RV-4CV, right ventricular end-diastolic inflow diameter in apical four-chamber view; RV-PLAX, right ventricular end-diastolic diameter in parasternal long-axis view; RVOT, right ventricular end-diastolic outflow tract diameter in short-axis view; BSA, body surface area.

aOne or more of the following: twin pregnancy, branch pulmonary artery stenosis, right ventricular systolic dysfunction, right ventricular hypertrophy.

Bold values signify statistically significant differences.

Table 4

Presence of significant pulmonary regurgitation and additional haemodynamic or obstetric risk factors in pregnancies complicated by symptomatic right heart failure and those without

  No right ventricular failure (n = 69) Symptomatic right ventricular failure (n = 7) 
Mild or no PR (n = 46) No additional risk factorsa 38 – 
Additional risk factorsa – 

 
Moderate-to-severe PR (n = 30) No additional risk factorsa 17 – 
Additional risk factorsa 
  No right ventricular failure (n = 69) Symptomatic right ventricular failure (n = 7) 
Mild or no PR (n = 46) No additional risk factorsa 38 – 
Additional risk factorsa – 

 
Moderate-to-severe PR (n = 30) No additional risk factorsa 17 – 
Additional risk factorsa 

PR, pulmonary regurgitation.

aOne or more of the following: twin pregnancy, branch pulmonary artery stenosis, RV systolic dysfunction, RV hypertrophy.

Obstetric outcome

Of the 76 pregnancies that continued beyond 24 weeks of gestation, 46 (61%) were delivered at the local obstetric unit and 30 (39%) at the specialist centre. The mean gestational age at delivery was 37.9 ± 3.8 weeks. Caesarean section was performed for 24 deliveries (32%), with 23 of the 24 (96%) for an obstetric indication and 50% as emergencies. Only one patient had a planned caesarean section for a maternal cardiac indication (patient 6 in Table 2). In 13 pregnancies (17%) obstetric complications were recorded, including four (5%) with cervical insufficiency, two (3%) with systemic hypertension, two (3%) with pre-eclampsia, two (3%) with post-partum haemorrhage, two (3%) with retained placental products, and one (1%) with cholestasis of pregnancy.

Neonatal outcome

The 76 completed pregnancies included three twin pregnancies, with a total of 79 infants. There were two stillbirths, one at 24 weeks gestation (cause unknown) in a patient with mild PS and the other at 34 weeks due to placental insufficiency in a patient with mild PR. Of the 77 live births, the mean gestational age at delivery was 38 ± 3.4 weeks (range 23–42 weeks). Thirteen infants (17%) were born preterm at a mean gestational age of 32 ± 3.9 weeks, with a mean birth weight of 1983 ± 746 g (range 530–2824 g). One preterm live born infant (23 weeks gestation) died 3 days later as a consequence of prematurity, all others survived. Gestational age at delivery and birth weight were not significantly different in pregnancies complicated by RHF, though direct comparison is not possible, as numbers are confounded by the higher rate of twin pregnancies in the RHF group.

Four infants (5%) were born with CHD. One mother with congenital PS and twin pregnancy had a male infant with TOF, requiring surgical correction. Another patient with repaired TOF had an infant with valvar PS, managed conservatively. Both infants were diagnosed prenatally by foetal echocardiography. One pre-term infant born at 29 weeks of gestation underwent surgical closure of a persistent ductus arteriosus during the first year of life and one mother with absent pulmonary valve syndrome had a daughter with a small atrial septal defect, which closed spontaneously in her first year of life.

Discussion

In this study, we have focused on residual haemodynamic lesions of the RVOT and assessed their impact on pregnancy outcome in women with CHD. Our study has shown that pregnancy is well tolerated with good outcome in the majority of women with residual RVOT lesions, but a small number do experience complications, including symptomatic RHF. Identifying these ‘at-risk’ individuals prior to pregnancy is essential for optimal outcome.15

All patients who developed RHF had moderate-to-severe PR, with an additional haemodynamic or obstetric risk factor, namely BPS or multiple pregnancy. Of note, the degree of RV dilatation was not a predictor for developing RHF, nor was the presence of RV systolic dysfunction. Given the trend to a higher rate of RVH in the group who developed RHF, one might speculate that hypertrophy causes a decrease in RV compliance, impairing the ability of the RV to accommodate the increase in circulating blood volume associated with pregnancy. However, true restrictive RV physiology was documented in only one patient who developed RHF, suggesting that more subtle echocardiographic markers of RV compliance need to be examined in order to explain this phenomenon. With specialist multidisciplinary care and frequent review by a specialist GUCH cardiologist, all patients who developed RHF were diagnosed and treated promptly with furosemide and there were no major adverse events during pregnancy or post-partum.

A summary of baseline characteristics and pregnancy outcome in all reported series2–7,9 of comparable patient groups is given in Table 5. When summated, the total number of reported pregnancies amounts to 444 with an overall good pregnancy outcome and no reported maternal deaths. In three series, moderate-to-severe PR was found to be a risk factor for adverse outcome.3,6,7 Compared with our series, the number of patients with severe PR in these studies was, however, smaller3,7 or was not reported.6 Furthermore, all of these studies focused on the underlying congenital heart defects, with less analysis of residual haemodynamic lesions at the time of conception. The high rate of significant PR in our cohort may be explained by the fact that some consider moderate–severe PR to contraindicate pregnancy, with many recommending pre-pregnancy pulmonary valve replacement, even in asymptomatic patients, while we do not take this view.10 In contrast to other series we also observed no complication of cardiac arrhythmia, despite a high prevalence of prior arrhythmia in our study population.2,3,6,7 One might speculate that this is due to random chance, or a difference in baseline patient characteristics and arrhythmia substrate, but it also might be due to the fact that fluid overload as evidenced by elevation of JVP was avoided, by intervening early with diuretic therapy and up-titrating diuretics as needed to maintain normal JVP.

Table 5

Cardiac complications in different series of patients with congenital heart disease and residual right ventricular outflow tract lesions

Author Patient characteristics Na Cardiac complications Comments 
Singh et al.5 Repaired tetralogy of Fallot 31b None No details about residual lesions 

 
Zuber et al.c4 Repaired tetralogy of Fallot 37 None No details about residual lesions 

 
Siu et al.c2 
  • Repaired tetralogy of Fallot (n = 53)

  • Unrepaired pulmonary stenosis (n = 35)

  • Repaired pulmonary stenosis (n = 23)

 
111 
  • Stroke (n = 1)

  • Bradycardia (n = 1)

  • Supraventricular tachycardia (n = 3)

  • Ventricular tachycardia (n = 3)

 
No details about residual lesions 

 
Veldtman et al.6 
  • Repaired tetralogy of Fallot (n = 74)

  • Unrepaired tetralogy of Fallot (n = 8)

 
82 
  • Supraventricular tachycardia (n = 2)

  • Heart failure (n = 2)

  • Progressive right ventricular dilatation in one patient with severe PR

 
Predictors for adverse outcome:
  • Left ventricular dysfunction

  • Severe pulmonary hypertension

  • Severe PR with right ventricular dysfunction

 

 
Meijer et al.7 Repaired tetralogy of Fallot
  • Severe PR (n = 14)

 
50 
  • Supraventricular tachycardia (n = 3)

  • Ventricular tachycardia (n = 3)

  • Right heart failure (n = 2)

 
Both patients with right heart failure had severe PR and developed supraventricular tachycardia 

 
Khairy et al.c3 
  • Repaired tetralogy of Fallot ( n = 15)

  • Pulmonary stenosis (n = 3)

Residua at time of conception:
  • Moderate-to-severe PR (n = 18)

  • Pulmonary stenosis >30 mmHg (n = 11)

 
18 
  • Pulmonary oedema (n = 5)

    • Only in repaired Tetralogy of Fallot with severe residual PR

    • Three had right ventricular dysfunction

  • Non-sustained ventricular tachycardia (n = 3)

 
Predictors for adverse outcome:
  • Severe PR: odds ratio 4.6

  • RV dysfunction: odds ratio 7.7

 

 
Pedersen et al.9 Repaired tetralogy of Fallot (n = 25)
  • PR (n = 5)

 
39 
  • None

 
  • No details about severity of residual haemodynamic lesions

  • Low rate of transannular patch repair

  • Low rate of residual PR

 

 
Our series Congenital heart disease with residual right ventricular outflow tract lesions 76 
  • Right ventricular failure (n = 7)

  • No arrhythmias

 
Only the combination of significant PR with additional risk factors was predictive of right heart failure 
Author Patient characteristics Na Cardiac complications Comments 
Singh et al.5 Repaired tetralogy of Fallot 31b None No details about residual lesions 

 
Zuber et al.c4 Repaired tetralogy of Fallot 37 None No details about residual lesions 

 
Siu et al.c2 
  • Repaired tetralogy of Fallot (n = 53)

  • Unrepaired pulmonary stenosis (n = 35)

  • Repaired pulmonary stenosis (n = 23)

 
111 
  • Stroke (n = 1)

  • Bradycardia (n = 1)

  • Supraventricular tachycardia (n = 3)

  • Ventricular tachycardia (n = 3)

 
No details about residual lesions 

 
Veldtman et al.6 
  • Repaired tetralogy of Fallot (n = 74)

  • Unrepaired tetralogy of Fallot (n = 8)

 
82 
  • Supraventricular tachycardia (n = 2)

  • Heart failure (n = 2)

  • Progressive right ventricular dilatation in one patient with severe PR

 
Predictors for adverse outcome:
  • Left ventricular dysfunction

  • Severe pulmonary hypertension

  • Severe PR with right ventricular dysfunction

 

 
Meijer et al.7 Repaired tetralogy of Fallot
  • Severe PR (n = 14)

 
50 
  • Supraventricular tachycardia (n = 3)

  • Ventricular tachycardia (n = 3)

  • Right heart failure (n = 2)

 
Both patients with right heart failure had severe PR and developed supraventricular tachycardia 

 
Khairy et al.c3 
  • Repaired tetralogy of Fallot ( n = 15)

  • Pulmonary stenosis (n = 3)

Residua at time of conception:
  • Moderate-to-severe PR (n = 18)

  • Pulmonary stenosis >30 mmHg (n = 11)

 
18 
  • Pulmonary oedema (n = 5)

    • Only in repaired Tetralogy of Fallot with severe residual PR

    • Three had right ventricular dysfunction

  • Non-sustained ventricular tachycardia (n = 3)

 
Predictors for adverse outcome:
  • Severe PR: odds ratio 4.6

  • RV dysfunction: odds ratio 7.7

 

 
Pedersen et al.9 Repaired tetralogy of Fallot (n = 25)
  • PR (n = 5)

 
39 
  • None

 
  • No details about severity of residual haemodynamic lesions

  • Low rate of transannular patch repair

  • Low rate of residual PR

 

 
Our series Congenital heart disease with residual right ventricular outflow tract lesions 76 
  • Right ventricular failure (n = 7)

  • No arrhythmias

 
Only the combination of significant PR with additional risk factors was predictive of right heart failure 

PR, pulmonary regurgitation; RV, right ventricle.

aPregnancies that continued beyond 24 weeks gestation.bIn this study, a total of 40 successful pregnancies are reported but only in 31 cases detailed information was available.cThese studies included patients with other congenital and acquired heart diseases.

Based on our findings, the recommendation for prophylactic pulmonary valve replacement in patients with moderate-to-severe PR prior to pregnancy should be reconsidered. In our opinion, if these patients are cared for by a specialist MDT with frequent cardiology reviews, there is a negligible pregnancy risk for those with no additional haemodynamic risk factors, and even for patients with additional risk factors, the risk of developing RHF must be weighed against the higher and more definite risk of what is often repeat cardiac surgery. This is particularly so, as early intervention with diuretics is usually successful and complications are rare.

Although the mode of follow-up may slightly differ between different high-risk obstetric programmes, this will likely not have a significant impact on overall outcome and our data strongly underscore the need for such programmes.

Study limitations

The primary use of echocardiography for assessment of the right ventricle and the lack of a pre-defined protocol for the echocardiographic analysis of RV function is a study weakness especially as diastolic function would appear to be as important as systolic function in this patient group. By integrating measurements of multiple echocardiographic views and reanalysing of images by experienced echocardiographers, we tried to overcome some of these limitations.

Although we believe that haemodynamic residua are more important than the underlying anatomical diagnosis with regard to pregnancy outcome, the latter might have an influence on the development of complications and our study could not examine this effect due to the heterogeneity of the study population.

Although only the combination of significant PR with additional risk factors was predictive for symptomatic RV failure, the number of outcomes was too small for more sophisticated statistical analysis, particularly for logistic regression models of single haemodynamic risk factors.

Several patients had multiple pregnancies. Because of the small sample size we did not, however, adjust for intra-patient correlation.

Conclusion

In patients with CHD and residual RVOT lesions, the outcome of pregnancy is good. Patients with moderate-to-severe PR were at risk for symptomatic RHF only if additional risk factors were present. When treated by an MDT, maternal and foetal outcome was good even in patients who developed RHF. A general recommendation for pulmonary valve replacement prior to pregnancy in patients with moderate-to-severe PR would appear unjustified.

Acknowledgement

We wish to thank Adrienne Kovacs from the Toronto Congenital Cardiac Centre for Adults for her help with statistical analysis.

Conflict of interest: none declared.

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