Objective
The goal of the study was to examine risks in pregnancy in patients with Ebstein anomaly.
Study Design
Data were examined retrospectively for 13 patients (27 pregnancies, 21 live births) with Ebstein anomaly during pregnancy who were treated at our institution from 1985 to 2011. The associated anomalies in these patients were atrial septal defect (ASD) (n = 4) and the Wolff-Parkinson-White syndrome (n = 6).
Results
Before pregnancy, 2 patients underwent ASD closure and 1 received tricuspid valve replacement (TVR). In all patients, the cardiothoracic ratio increased from 55.1 at conception to 57.0 during pregnancy and 58.0 postpartum ( P < .05). Cesarean sections were performed in 3 cases: 1 with ventricular tachycardia and orthopnea (New York Heart Association [NYHA] III) preterm; at full term, and the third in a patient with a mechanical tricuspid valve who developed maternal cerebellum hemorrhage at 27 weeks. The baby died of prematurity in the third case. In all other cases (20 of 21), neonatal prognoses were good without congenital heart diseases. There were 6 spontaneous abortions. Recurrent paroxysmal supraventricular tachycardia occurred during pregnancy in 2 cases and was treated with adenosine triphosphate or verapamil. In 17 pregnancies, NYHA remained in class I and all had full-term vaginal delivery.
Conclusion
Maternal and fetal outcomes are good in patients with Ebstein anomaly and NYHA class I. However, pregnancy in Ebstein anomaly can be complicated with tachyarrhythmia or cardiac failure. In post-TVR cases, meticulous care is required for these complications during pregnancy and delivery.
Ebstein anomaly occurs in about 0.5% of cases of congenital heart disease. This congenital malformation is characterized by downward displacement of tricuspid valve into the right ventricle because of an anomalous attachment of septal and posterior leaflets. The abnormally situated tricuspid orifice divides the right ventricle into a proximal atrialized segement and a distal functional small ventricular chamber. Ebstein anomaly usually revealed tricuspid regurgitation with right atrial enlargement and is often associated with atrial septal defect (ASD) and the Wolff-Parkinson-White (WPW) syndrome type that leads to cyanosis and arrhythmias such as supraventricular tachyarrhythmia.
Many women with Ebstein anomaly reach child-bearing age, but there is little information about the risks of pregnancy and delivery, with only 2 reports of a relatively large series of pregnancy complicated with Ebstein anomaly.
In these reports, there were a total of 56 patients and 153 pregnancies, in which miscarriage occurred in 16%, neonatal death in 2%, preeclampsia in 1%, preterm delivery in 29%, vaginal delivery in 90%, heart failure of New York Heart Association (NYHA) class III or above in 0.7%, and life-threatening arrhythmia in 0.7%. Thus, pregnancy in women with Ebstein anomaly is generally well tolerated, but some patients are undiagnosed until the onset of symptoms such as dyspnea and atrial flutter in the latter half of the pregnancy. Furthermore, some patients with Ebstein anomaly are asymptomatic, even after multiple deliveries, and the condition may not be diagnosed until the age of 50 years or later.
The variety of symptoms and disease severity and differences in complications such as cyanosis, arrhythmia, and stroke make a uniform evaluation difficult. Therefore, the current retrospective study of pregnancy complicated with Ebstein anomaly was carried out to determine factors that influence maternal and fetal prognosis.
Materials and Methods
Maternal and fetal mortality and morbidity in 13 patients with Ebstein anomaly (27 pregnancies) were examined as a case series based on charts in our hospital from January 1985 to December 2011. Cases associated with congenital heart diseases other than ASD and patent foramen ovale were excluded. Cardiac function was evaluated by echocardiography performed before pregnancy, at conception, in each trimester, and postpartum. Cardiothoracic ratio (CTR) was measured on chest X-rays taken before pregnancy, in the third trimester, postpartum, and at other times if needed. A Holter electrocardiogram was recorded in each trimester and postpartum. Vaginal delivery was attempted for women with spontaneous labor, whereas cesarean section was selected for those with a need for early delivery for both fetal and maternal indications. The NYHA classification was used to evaluate cardiac status.
Data collection
Data were collected for maternal age; age at diagnosis; CTR; grade of tricuspid valve regurgitation (TR); NYHA functional class during and after pregnancy; delivery mode (cesarean section or vaginal delivery); treatment before, during, and after delivery; time of delivery (gestational weeks); and birthweight. Echocardiography was performed for all babies to evaluate associated congenital heart diseases.
Statistical analysis
For continuous variables, a Student t test was performed for analysis of normally distributed data; otherwise a Wilcoxon test was used. All statistical analyses were performed using JMP 7 (SAS Institute, Cary, NC). P < .05 was considered statistically significant.
Results
There were 27 pregnancies and 21 live births in 13 patients during the study period. Six pregnancies were spontaneously aborted before 10 weeks of gestation. The median (range) ages at the time of initial symptoms, diagnosis of Ebstein anomaly, and delivery were 10 (0–30), 21 (0–38), and 32 (25–29) years old, respectively ( Table 1 ). In 2 patients, heart disease was not diagnosed at the time of conception (cases 7 and 8). Four patients had ASD, 6 had WPW syndrome, and 1 had both of these conditions. No patients had cyanosis.
| Case | Associated lesions | Age at onset, mo | Age at initial diagnosis, mo | Age at delivery, y | |
|---|---|---|---|---|---|
| 1 | 9 | 9 | 25 | ||
| 2 | ASD | 3 | 5 | 35, 37 | |
| 3 | ASD | WPW | 5 | 26 | 35 |
| 4 | ASD | 6 | 28 | 31 | |
| 5 | 6 | 10 | 28 | ||
| 6 | ASD | 7 | 7 | 30, 33 | |
| 7 | WPW | 7 | 35 | 35, 39 | |
| 8 | WPW | 18 | 38 | 38 | |
| 9 | WPW | 10 | 10 | 31 | |
| 10 | WPW | 13 | 21 | 25, 29 | |
| 11 | WPW | 22 | 22 | 26, 28, 36 | |
| 12 | 25 | 25 | 27, 36 | ||
| 13 | 30 | 30 | 32, 36 | ||
The patients with ASD developed symptoms of Ebstein anomaly earlier than those with WPW (5.3 vs 11.2 years old, P < .05), but there was no difference in the age of diagnosis (12.2 vs 10.2 years old, P = not significant) or delivery (33.5 vs 32.2 years old, P = not significant). The median time of delivery was 37.9 (27–39.4) gestational weeks, and the neonatal weight was 2664 (1063–3590) g ( Figure 1 ). There were 2 early deliveries at 27 and 28 weeks, respectively, and in these 2 cases, the babies were extremely low birthweight infants (between 1000 and 1499 g) ( Figure 1 ). There was 1 small-for-gestational-age baby. The other 20 babies were appropriate for gestational age. No babies had congenital heart diseases. One baby born with a birthweight of 1063 g at 27 weeks died of prematurity. All other babies showed normal growth and no neurological disability.
Treatment before pregnancy
Ablation therapy for paroximal supraventricular tachycardia (PSVT) accompanied with WPW syndrome) was performed in 2 cases before pregnancy (cases 9 and 11) ( Table 2 ), and there was no other arrhythmia event during and after delivery. One patient (case 2) with mechanical tricuspid valve replacement (TVR) developed maternal cerebral hemorrhage at 27 weeks of gestation and cesarean section was performed.
| Case | TR before pregnancy | CTR (%) in 1st pregnancy | Delivery week | CV event | Treatment before pregnancy | ||
|---|---|---|---|---|---|---|---|
| Before | Third | Post | |||||
| 2 | Mild | 53 | 27 | 58 | 27 | CHF | ASD closure, TVR (3 y) |
| 9 | Mild | 46 | 39 | 53 | 39 | PSVT | Ablation (15 y) |
| 11 | Mild | 42 | 39 | 52 | 39 | PSVT | Ablation (22 y) |
| During pregnancy | |||||||
| 1 | Mod | 62 | 28 | 66 | 28 | CHF | Furosemide, oxygen |
| 3 | Mod | 58 | 37 | 58 | 37 | CHF | Furosemide, oxygen |
| 7 | Mod | nd | 39 | 62 | 39 | PSVT | Verapamil |
| 10 | Mild | 47 | 39 | 55 | 39 | PSVT | ATP |
| After pregnancy | |||||||
| 4 | Sev | 55 | 37 | 62 | 37 | CHF | ASD closure, TVP |
| 6 | Mod | 59 | 37 | 58 | 37 | CHF | Furosemide |
| 12 | Sev | 62 | 37 | 61 | 37 | CHF | Furosemide |
| 5 | Mod | 54 | 38 | 52 | 38 | No | None |
| 8 | Mod | 55 | 38 | 58 | 38 | No | None |
| 13 | Mod | 57 | 61 | 58 | 37 | No | None |
Treatment during pregnancy
Nonsustained ventricular tachycardia (NSVT) was terminated with bisoprolol fumarate (case 1). Diuretics and oxygen were administered when dyspnea caused by cardiac failure occurred (cases 1 and 3) ( Table 2 ). Two patients had recurrent PSVT during pregnancy (cases 7 and 10). Each arrhythmia was treated effectively with verapamil or adenosine triphosphate. Each patient underwent ablation therapy after delivery.
Treatment after delivery
The patient with ASD and severe TR (case 4) developed cardiac failure (NYHA class worsened to III) at 16 years after delivery, and ASD closure and tricuspid valvoplasty were performed ( Table 2 ). In 2 cases, diuretics were given because of worsening of cardiac failure (cases 6 and 12).
Change in CTR
The median (range) CTR significantly increased from before to during pregnancy (55.1% [42–62%] vs 57.0% [51–72%], P < .05) and from before pregnancy to after delivery (55.1% [42–62%] vs 58.0% [52–62%], P < .05) ( Figure 2 ).
