Summary
Background
Ebstein anomaly is an uncommon congenital cardiac lesion that may be associated with cyanosis, arrhythmias and right heart dysfunction. Investigation into patient characteristics and outcomes in pregnant women with Ebstein anomaly has been limited.
Aims
To characterize patient characteristics and clinical events for pregnant women with Ebstein anomaly during hospitalization for delivery in the USA; also, to determine the effect of Ebstein anomaly on maternal clinical outcomes and individual predictors of poor outcome at time of delivery.
Methods
We screened the Healthcare Cost and Utilization Project’s National Inpatient Sample for hospital admissions of pregnant women for delivery (vaginal or caesarean section) in the USA from 2003–2012, and identified a cohort of 7,850,381. Clinical characteristics and maternal outcomes were identified in those with and without Ebstein anomaly. The primary outcome of interest was major adverse cardiac events (MACE), a composite of in-hospital death, acute myocardial infarction, cerebrovascular events, embolic events, cardiac complications of labour and delivery heart failure or arrhythmia.
Results
Our study population consisted of 82 hospitalizations of pregnant women with Ebstein anomaly and 7,850,299 without. The Ebstein cohort more frequently had ostium secundum-type atrial septal defect and/or patent foramen ovale and anomalous atrioventricular excitation ( P < 0.001 for both). The MACE rate was significantly higher among Ebstein patients ( P < 0.001). Preterm delivery, postpartum haemorrhage and caesarean delivery occurred more frequently among the Ebstein cohort (19.5% vs 7.2%, 8.5% vs 2.8% and 47.6% vs 31.1%, respectively; P ≤ 0.001). In a multivariable analysis, anomalous atrioventricular excitation (odds ratio [OR] 21.75, 95% confidence interval [CI] 1.03–457.91) and preterm delivery (OR 11.71, 95% CI 1.39–98.89) were associated with MACE among those with Ebstein anomaly.
Conclusions
Pregnant women with Ebstein anomaly are at higher risk of MACE during pregnancy and delivery. Preterm delivery occurred more frequently in women with Ebstein anomaly.
Résumé
Contexte
L’anomalie d’Ebstein est une cardiopathie congénitale rare, qui peut être révélée par une cyanose, des arythmies et une dysfonction du cœur droit. Les données concernant les caractéristiques de ces patients, ainsi que les modalités évolutives ont été peu étudiées.
Objectifs
Caractériser le profil clinique et évolutif des femmes porteuses d’une anomalie d’Ebstein au cours de la grossesse, pendant l’hospitalisation de délivrance et déterminer par ailleurs l’influence d’une anomalie d’Ebstein sur le pronostic maternel ainsi que sur les prédicteurs d’évènements au cours de la délivrance.
Méthode
La base des admissions hospitalières « Healthcare Cost and utilization project’s national inpatient sample » a été utilisée, recueillie aux États-Unis entre 2003 et 2012, et portant sur les délivrances quel que soit le mode, par les voies naturelles ou par césarienne. Une cohorte de 7 850 381 patientes a été ainsi constituée. Les caractéristiques cliniques et le suivi maternel ont été identifiés selon qu’il y avait ou non une anomalie d’Ebstein préexistante. Le critère de jugement cardiovasculaire composite incluait : décès hospitalier, infarctus du myocarde, évènements cérébro-vasculaires, évènements emboliques, insuffisance cardiaque ou arythmie lors du travail et au décours de la délivrance.
Résultats
Cette étude a pris en considération 82 hospitalisations de femmes enceintes porteuses de l’anomalie d’Ebstein, comparées à 7 850 299 exemptes de cette cardiopathie congénitale. La cohorte Ebstein comportait plus fréquemment une communication interauriculaire ostium secondum et/ou un foramen ovale perméable ainsi que des anomalies de l’excitabilité atrioventriculaire ( p < 0,001). Le taux d’évènements cardiovasculaires était significativement plus élevé chez les femmes porteuses de l’anomalie d’Ebstein ( p < 0,001). La délivrance préterme, les hémorragies du postpartum, la délivrance par césarienne survenaient plus fréquemment chez les femmes porteuses de l’anomalie d’Ebstein (19,5 % versus 7,2 % ; 8,5 % versus 2,8 % et 47,6 % versus 31,1 % respectivement, p < 0,001). L’analyse multivariée a montré que l’anomalie de l’excitabilité atrioventriculaire ( odds ratio 21,75, IC : 95 % 1,03–457,91) et la délivrance préterme (OR : 11,71, IC : 95 % 1,39–98,89) étaient associées de façon significative à la survenue d’un évènement cardiaque majeur parmi les patientes porteuses de l’anomalie d’Ebstein.
Conclusion
Les femmes enceintes porteuses de l’anomalie d’Ebstein sont donc exposées à un risque accru d’évènements cardiovasculaires pendant la grossesse et lors de la délivrance. La délivrance préterme survient plus fréquemment chez les femmes porteuses de cette cardiopathie congénitale rare.
Background
Ebstein anomaly is a rare congenital cardiac abnormality, characterized by a functionally and morphologically abnormal tricuspid valve and right ventricle. Ebstein anomaly encompasses a wide anatomical spectrum of abnormalities of the tricuspid valve leaflets and right ventricle, including a large anterior tricuspid leaflet, usually attached to the tricuspid valve annulus, which can be redundant and fenestrated. The posterior and septal leaflets are displaced apically, accounting for “atrialization” of the right ventricle and dilation of the tricuspid annulus, with resultant tricuspid regurgitation and enlargement of the right atrium. Concomitant lesions are often reported, and include atrial septal defect, patent foramen ovale and ventricular pre-excitation . Clinical symptoms may include cyanosis, right heart failure, arrhythmias, decreased exercise tolerance, fatigue, sudden cardiac death and paradoxical emboli, particularly in adulthood .
Women born with Ebstein anomaly frequently reach childbearing age and desire pregnancy. However, little contemporary information is available about the maternal risks of pregnancy and delivery to counsel such patients . Adverse maternal cardiovascular outcomes and neonatal events can include maternal death, heart failure, arrhythmia, embolic events, pre-eclampsia/eclampsia, preterm labour and delivery and neonatal death.
In this study, we sought to characterize patient characteristics and clinical events for contemporary pregnant women with Ebstein anomaly during hospitalization for delivery in the USA; in addition, we aimed to determine the effect of Ebstein anomaly on their maternal clinical outcomes and individual predictors of poor outcome at the time of delivery.
Methods
Data source
We used data from the 2003–2012 National Inpatient Sample (NIS), collected by the Agency for Healthcare Research and Quality’s Healthcare Cost and Utilization Project (HCUP), which is the largest all-payer inpatient publicly available database in the USA . The NIS provides annual information on approximately 8 million inpatient stays from about 1000 hospitals, and estimates a 20% stratified sample from a sampling frame that comprises 90% of acute care hospital admissions in the USA. International Classification of Diseases, Ninth Revision (ICD-9) codes were used to identify a cohort of pregnant women hospitalized for delivery, defined as any discharge record with a normal delivery or other indications for care in pregnancy, labour and delivery-related diagnoses (ICD-9 codes 650–659 and V27.4–V27.9) or a delivery-related procedure (ICD-9 codes 72–75) or early onset of delivery, delivered, with or without mention of antepartum condition (ICD-9 codes 644.2, 644.20, 644.21).
Study population
We identified a cohort of 7,850,381 hospitalizations of pregnant women admitted for delivery (vaginal or caesarean section) at hospitals in the USA. The sample population was broadly separated into patients with Ebstein anomaly and those without (the control group), defined by the presence or absence of the ICD-9 code 746.2 for Ebstein anomaly. Data from 2003 were not used for the control group because of coding complexities inherent within the NIS 2003 database.
Patient characteristics and outcome measures
All patient and hospital characteristics were obtained from the NIS. Demographic and medical history data extracted included maternal age, race, insurance status, annual income, delivery at teaching hospital, hospital region, diabetes mellitus (ICD-9 code 250), multiple gestation (ICD-9 codes 651.70, 651.71, 651.73,651.80, 651.81, 651.83, 651.90, 651.91,651.93, 652.60, 652.61, 652.63, V91.90, V91.91, V91.92, V91.99), ostium secundum-type atrial septal defect and patent foramen ovale (ICD-9 code 745.5), cyanosis (ICD-9 code 782.5) and anomalous atrioventricular excitation, which encompasses Wolff-Parkinson-White syndrome (ICD-9 code 426.7). Characteristics of valvular disease were also studied (mitral stenosis, ICD-9 code 394.0; mitral stenosis with insufficiency, ICD-9 code 394.2; tricuspid valve, ICD-9 code 397.0; non-congenital diseases of tricuspid valve, ICD-9 code 397.0; congenital tricuspid atresia and stenosis, ICD-9 code 746.1; aortic valve disease, ICD-9 code 424.1). Cardiomyopathy rates were assessed and separated into: hypertrophic cardiomyopathy (ICD-9 codes 425.1–425.18); peripartum cardiomyopathy (ICD-9 codes 674.50–674.54); and other cardiomyopathies (which include disorders such as endomyocardial fibrosis, other primary cardiomyopathy, alcoholic cardiomyopathy, cardiomyopathy in other diseases classified elsewhere and secondary cardiomyopathy unspecified; ICD-9 codes 425.0 and 425.2–425.9).
The primary outcome of interest was major adverse cardiac events (MACE), defined as a composite of in-hospital death (HCUP variable for death Y/N), acute myocardial infarction (ICD-9 codes 410 and 411), cerebrovascular events (ICD-9 codes 431, 433–436), pulmonary embolism (ICD-9 code 515.1), arterial embolism (ICD-9 code 444), artheroembolism (ICD-9 code 445), obstetrical pulmonary embolism (ICD-9 code 673), cardiac complications of anaesthesia or other sedation in labour and delivery (ICD-9 code 668.1), heart failure (total heart failure, ICD-9 code 428; congestive heart failure, ICD-9 code 428.0; left heart failure, ICD-9 code 428.1; systolic heart failure, ICD-9 code 428.2; diastolic heart failure, ICD-9 code 428.3; combined systolic and diastolic heart failure, ICD-9 code 428.4; unspecified heart failure, ICD-9 code 428.9), cardiac arrhythmia (total cardiac dysrhythmia, ICD-9 code 427; paroxysmal supraventricular tachycardia, ICD-9 code 427.0; paroxysmal ventricular tachycardia, ICD-9 code 427.1; unspecified paroxysmal tachycardia, ICD-9 code 427.2; atrial fibrillation and flutter, ICD-9 code 427.3; atrial fibrillation, ICD-9 code 427.31; atrial flutter, ICD-9 code 427.32; ventricular fibrillation and flutter, ICD-9 code 427.4; ventricular fibrillation, ICD-9 code 427.41; ventricular flutter, ICD-9 code 427.2), cardiac arrest (ICD-9 code 427.5), stroke (ICD-9 codes 434.0–435.9) and endocarditis (ICD-9 codes 421.0–421.9 and 424.90–424.99). Other outcomes of interest studied included transient hypertension of pregnancy (ICD-9 codes 642.30–642.34), mild pre-eclampsia (ICD-9 codes 642.40–642.44), severe pre-eclampsia (ICD-9 codes 642.50–642.54), eclampsia complicating pregnancy/childbirth (ICD-9 codes 642.60–642.64), postpartum haemorrhage (ICD-9 code 666) and caesarean delivery (ICD-9 code 74). Preterm labour (ICD-9 codes 644.0–644.13) and preterm delivery (ICD-9 codes 644.2–644.21) rates were also analysed. In situations in which fewer than 10 events or individuals/pregnancies are reported, HCUP NIS recommends reporting “≤ 10” in order to protect individuals and the HCUP database resource itself.
Statistical analysis
Univariate analyses were used to compare demographics, medical history and clinical outcomes of patients in each year. Data were summarized by descriptive statistics. Categorical variables are presented as percentages and were compared using the χ 2 test. Continuous variables are presented as means ± standard deviations and were compared using one-way analysis of variance. Multivariable regression analysis was performed to determine the association of Ebstein anomaly at delivery with MACE. A separate multivariable logistic regression was performed to examine the association of all women at delivery with MACE. Predictor variables included all factors that had a P value < 0.1 in the univariate models. Odds ratios (ORs) and 95% confidence intervals (CIs) are reported. STATA 12 (StataCorp LP, College Station, TX, USA) was used for data analysis and a two-tailed P value ≤ 0.05 was regarded as statistically significant.
Results
Patient characteristics
Among the 7,850,381 patients who comprised the study population, 82 had Ebstein anomaly. Demographic characteristics for pregnant women delivering with Ebstein anomaly and the control group are summarized in Table 1 . In the Ebstein group, the mean age was 27.6 years, 60.6% were white, 22.7% were Hispanic, 38.3% were Medicaid recipients, 50.6% were privately insured and 24.4% delivered at a teaching hospital. Patient characteristics appeared to be similar between the two groups; proportionally more patients had Medicare insurance ( P = 0.03). Clinical characteristics are summarized in Table 2 . Of the women with Ebstein anomaly, 6.1% were coded for ostium secundum-type atrial septal defect and/or patent foramen ovale (vs < 0.001%; P < 0.001) and 9.8% for anomalous atrioventricular excitation (vs < 0.001%; P < 0.001). There were no cyanotic patients in the Ebstein group, defined as central cyanosis by ICD-9 coding.
| Ebstein group | Control group | P | |
|---|---|---|---|
| ( n = 82) | ( n = 7,850,299) | ||
| Age (years) | 27.64 ± 6.4 | 27.64 ± 6.2 | 0.99 |
| Race | 0.43 | ||
| White | 40 (60.6) | 3,293,728 (51.8) | 0.21 |
| Black | ≤ 10 a | 876,909 (13.8) | 0.68 |
| Hispanic | 15 (22.7) | 1,507,851 (23.7) | 0.83 |
| Other | ≤ 10 | 678,209 (10.7) | 0.11 |
| Insurance | 0.12 | ||
| Private, including HMO | 41 (50.6) | 3,961,480 (50.6) | 0.93 |
| Medicare | ≤ 10 | 48,847 (0.6) | 0.04 |
| Medicaid | 31 (38.3) | 3,339,897 (42.6) | 0.39 |
| Other Insurance | ≤ 10 | 486,228 (6.2) | 0.38 |
| Annual income | 0.05 | ||
| $1–24,999 | 21 (25.6) | 2,110,422 (26.9) | 0.80 |
| $25,000–34,999 | 16 (19.5) | 1,928,921 (24.6) | 0.29 |
| $35,000–44,999 | 17 (20.7) | 1,878,340 (23.9) | 0.50 |
| $45,000 or more | 23 (28.1) | 1,780,222 (22.7) | 0.25 |
| Teaching hospital | 20 (24.4) | 1,400,503 (17.8) | 0.12 |
| USA hospital region | 0.53 | ||
| Northeast | 12 (14.6) | 1,277,124 (16.3) | 0.69 |
| Midwest | 14 (17.1) | 1,626,767 (20.7) | 0.42 |
| South | 30 (36.6) | 2,990,222 (38.1) | 0.78 |
| West | 26 (31.7) | 1,956,186 (24.9) | 0.16 |
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