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JTCVS Open Dec 2023To describe the surgical outcomes in neonates and infants who had surgery for Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD).
OBJECTIVE
To describe the surgical outcomes in neonates and infants who had surgery for Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD).
METHODS
Retrospective chart review for all patients who underwent surgery for EA or TVD during the index hospitalization after birth at our institution from January 2005 to February 2023.
RESULTS
Fifteen symptomatic neonates and infants who had surgery for EA or TVD were included, 8 with EA and 7 with TVD. Eleven patients (73%) and 3 patients (20%) required preoperative inotropes and extracorporeal membrane oxygenation, respectively. Nine patients (60%) had a Starnes procedure and 6 patients (40%) had tricuspid valve repair (TVr). Mortality at last follow-up was 27% overall (n = 4/15), 22% after Starnes (n = 2/9) and 33% after TVr (n = 2/6), without a significant difference despite a greater-risk profile in the Starnes group. Postoperative day 1 lactate level was associated with mortality on Cox regression (hazard ratio, 1.45; = .01). Three of 9 patients who had a Starnes procedure were or will be converted to a cone repair (1.5/2-ventricle repair).
CONCLUSIONS
Mortality after surgery for EA or TVD during the index hospitalization after birth is still significant in the current era and is associated with a greater lactate level at postoperative day 1. The Starnes procedure and TVr had comparable outcomes despite a greater-risk profile in the Starnes group. An initial single-ventricle approach does not preclude conversion to biventricular or 1.5-ventricle repair.
PubMed: 38204669
DOI: 10.1016/j.xjon.2023.08.007 -
Open Heart Aug 2023Ebstein's anomaly is a rare congenital cardiac condition and data regarding pregnancy outcomes in this patient group are scarce. We evaluated the maternal and perinatal...
OBJECTIVE
Ebstein's anomaly is a rare congenital cardiac condition and data regarding pregnancy outcomes in this patient group are scarce. We evaluated the maternal and perinatal risks of pregnancy in 81 women with Ebstein's anomaly.
METHODS
The Registry of Pregnancy and Cardiac disease is a prospective global registry of pregnancies in women with structural cardiac disease. Pregnancy outcomes in women with Ebstein's anomaly were examined. The primary outcome was the occurrence of a major adverse cardiac event (MACE) defined as maternal mortality, heart failure, arrhythmia, thromboembolic event or endocarditis. Secondary endpoints were obstetric and perinatal outcomes and the influence of pregnancy on tricuspid valve regurgitation as well as right atrial and ventricular dimensions.
RESULTS
In the 81 women with Ebstein's anomaly (mean age 29.7±6.1 years, 46.9% nulliparous), MACE occurred in 8 (9.9%) pregnancies, mostly heart failure (n=6). There were no maternal deaths. Prepregnancy signs of heart failure were predictive for MACE. Almost half of the women were delivered by caesarean section (45.7%) and preterm delivery occurred in 24.7%. Neonatal mortality was 2.5% and 4.9% of the infants had congenital heart disease. In the subgroup in which prepregnancy and postpregnancy data were available, there was no difference in tricuspid valve regurgitation grade or right atrial and ventricular dimensions before and after pregnancy.
CONCLUSIONS
Most women with Ebstein's anomaly tolerate pregnancy well, but women with prepregnancy signs of heart failure are at higher risk for MACE during pregnancy and should be counselled accordingly.
Topics: Infant, Newborn; Infant; Humans; Female; Pregnancy; Young Adult; Adult; Ebstein Anomaly; Pregnancy Outcome; Tricuspid Valve Insufficiency; Prospective Studies; Cesarean Section; Atrial Fibrillation; Retrospective Studies; Heart Defects, Congenital; Heart Failure; Registries
PubMed: 37550057
DOI: 10.1136/openhrt-2023-002406 -
Journal of Neonatal-perinatal Medicine 2024A circular shunt is a poor prognostic factor associated with Ebstein's anomaly. Targeting the constriction of the ductus arteriosus (DA) in order to limit or resolve the...
A circular shunt is a poor prognostic factor associated with Ebstein's anomaly. Targeting the constriction of the ductus arteriosus (DA) in order to limit or resolve the circular shunt, has been shown to improve fetal outcomes. Prenatal non-steroidal anti-inflammatory drugs (NSAIDs) have been known to constrict the DA. Recently, prenatal NSAIDs have been used for that purpose in the treatment of circular shunt. Limited research shows that it may be an effective treatment leading to improved fetal outcomes. In this article, we did an extensive review of literature to describe this therapy's effectiveness and outcomes. 82% of fetuses were able to achieve ductal constriction with prenatal NSAID therapy. For fetuses who achieved ductal constriction, fetal demise was less likely (6%) when compared to those who were unable to achieve the same (50%). Of all the fetuses with hydrops, 50% had resoluation of hydrops with prenatal NSAID treatment.
Topics: Pregnancy; Female; Humans; Ebstein Anomaly; Anti-Inflammatory Agents, Non-Steroidal; Ductus Arteriosus, Patent; Ductus Arteriosus; Edema
PubMed: 38217614
DOI: 10.3233/NPM-230040 -
JTCVS Open Dec 2023In this study, we investigated the clinical characteristics of patients with Ebstein anomaly and left ventricular outflow tract (LVOT) obstruction as well as possible...
OBJECTIVE
In this study, we investigated the clinical characteristics of patients with Ebstein anomaly and left ventricular outflow tract (LVOT) obstruction as well as possible mechanisms of obstruction as assessed by 2-dimensional echocardiography.
METHODS
We queried our institutional echocardiography and surgical databases for patients with the diagnosis of Ebstein anomaly seen from 1985 through 2022. Fourteen patients had the additional diagnosis of LVOT obstruction identified from transthoracic echocardiography.
RESULTS
The mean age of the 14 patients at the time that LVOT obstruction was identified was 51.9 ± 9.9 years, and 11 (78%) were female. LVOT obstruction (mean gradient 61.0 ± 25.7 mm Hg) was documented at the time of initial diagnosis of Ebstein anomaly for 8, and in the remaining, was recognized on follow-up imaging. Dynamic LVOT obstruction with systolic anterior motion (SAM) was the most common mechanism and was mostly accompanied by leftward septal movement and septal hypertrophy. Seven of the 9 patients with SAM had the diagnosis of hypertrophic cardiomyopathy. Leftward bowing of the septum appeared to be the sole cause of LVOT obstruction in 3 and a contributing factor to SAM in 4. Among the 13 patients who underwent an operation for Ebstein anomaly, 7 had concomitant septal myectomy and 3 had a subsequent procedure.
CONCLUSIONS
There are multiple mechanisms of LVOT obstruction in patients with Ebstein anomaly, including SAM and leftward bowing of the interventricular septum, which may exist alone or in combination. Septal myectomy at the time of tricuspid valvuloplasty is safe and effectively reduces gradients in the outflow tract and may prevent the need for subsequent reintervention.
PubMed: 38204684
DOI: 10.1016/j.xjon.2023.10.024 -
Medical Ultrasonography Sep 2023
Topics: Humans; Ebstein Anomaly; Atrial Fibrillation; Heart Atria; Thrombosis
PubMed: 37778027
DOI: 10.11152/mu-4236 -
Korean Circulation Journal Feb 2024
PubMed: 38346696
DOI: 10.4070/kcj.2024.0007 -
Heart Rhythm O2 Mar 2024
PubMed: 38560370
DOI: 10.1016/j.hroo.2023.11.005 -
Journal of the American Heart... Jan 2024The increasing prevalence of atrial fibrillation (AF) in adults with congenital heart disease raises significant questions regarding its management. The unique... (Review)
Review
The increasing prevalence of atrial fibrillation (AF) in adults with congenital heart disease raises significant questions regarding its management. The unique underlying anatomic and physiological background further adds to the difficulty in eliminating the AF burden in these patients. Herein, we provide an overview of the current knowledge on the pathophysiology and risk factors for AF in adult congenital heart disease, with a special focus on the existing challenges in AF ablation. Emerging imaging modalities and ablation techniques might have a role to play. Evidence regarding the safety and efficacy of AF ablation in adult congenital heart disease is summarized, especially for patients with an atrial septal defect, Ebstein anomaly of the tricuspid valve, tetralogy of Fallot, and Fontan circulation. Finally, any remaining gaps in knowledge and potential areas of future research are highlighted.
Topics: Humans; Adult; Heart Defects, Congenital; Atrial Fibrillation; Heart Septal Defects, Atrial; Catheter Ablation; Ebstein Anomaly
PubMed: 38193287
DOI: 10.1161/JAHA.123.032102 -
Pediatric Cardiology Mar 2024The underlying invasive hemodynamics and physiology in Ebstein anomaly (EA) are poorly understood. Moreover, the hemodynamic impact of tricuspid valve intervention in EA...
The underlying invasive hemodynamics and physiology in Ebstein anomaly (EA) are poorly understood. Moreover, the hemodynamic impact of tricuspid valve intervention in EA has not been well studied. Retrospective cohort of 52 adults with repaired and 36 with unrepaired EA undergoing right heart catheterization at Mayo Clinic, MN between 1993 and 2021. "Repaired" EA was defined as prior tricuspid valve repair and/or replacement (83% post-tricuspid valve replacement). Repaired patients were younger than those with unrepaired EA (41.3 ± 16.0 versus 50.6 ± 15.6 years, p = 0.008) and had a lower prevalence of ≥ moderate native or prosthetic tricuspid regurgitation (67% versus 81%, p = 0.01). Right atrial (RA) pressure was higher among patients with repaired EA than in unrepaired disease [13 (11; 18) versus 10 (8; 15) mmHg; p = 0.02], but these differences were no longer present when adjusting for ≥ moderate right ventricular systolic dysfunction and ≥ moderate tricuspid regurgitation. Cardiac index (Qs) was lower among those with unrepaired EA than in repaired EA (1.9 ± 0.7 versus 2.3 ± 0.6 l/min/m, p = 0.01), even after adjusting for similar confounders. During a follow-up of 8.6 (3.2-13.3) years, 16 (18%) patients died. Systolic pulmonary artery pressure was independently associated with all-cause mortality. In summary, higher cardiac indices were found in those with repaired EA compared to those with unrepaired disease. RA hypertension was prevalent in both groups and no differences in right filling pressures were found between groups after adjusting for potential confounders. Elevation in pulmonary pressures was independently associated with survival. The use of pulmonary vasomodulators in EA requires further investigation.
Topics: Adult; Humans; Ebstein Anomaly; Retrospective Studies; Tricuspid Valve Insufficiency; Prognosis; Cardiac Catheterization
PubMed: 38019300
DOI: 10.1007/s00246-023-03337-0 -
Pediatric and Developmental Pathology :... May 2024Ebstein anomaly (EA) is a rare congenital heart defect characterized by abnormal development of the tricuspid valve (TV) and right ventricular myocardium. This study...
Ebstein anomaly (EA) is a rare congenital heart defect characterized by abnormal development of the tricuspid valve (TV) and right ventricular myocardium. This study documents 2 dramatic cases of fetal EA characterized by hydrops and cardiomegaly, leading to intrauterine or early neonatal death. These clinical outcomes were associated with morphological abnormalities including severe tricuspid regurgitation, unguarded TV orifice, pulmonary atresia, and flattened right ventricular myocardium. This study highlights that these adverse anatomical features may result in unfavorable clinical outcomes in fetal EA. While timely identification of such features by prenatal ultrasound is crucial for providing accurate prognostic stratification and guiding treatment decisions, fetopsy may be necessary to discern EA among the spectrum of right-heart anomalies.
PubMed: 38762771
DOI: 10.1177/10935266241250235