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Cardiovascular Diagnosis and Therapy Jun 2021Patients with congenital heart disease (CHD) are one of the fastest growing populations in cardiology, and valvular pathology is at the center of many congenital... (Review)
Review
Patients with congenital heart disease (CHD) are one of the fastest growing populations in cardiology, and valvular pathology is at the center of many congenital lesions. Derangements in valvular embryology lead to several anomalies prone to dysfunction, each with hemodynamic effects that require appropriate surveillance and management. Surgical innovation has provided new treatments that have improved survival in this population, though has also contributed to esotericism in patients who already have unique anatomic and physiologic considerations. Conduit and prosthesis durability are often monitored collaboratively with general and specialized congenital-focused cardiologists. As such, general cardiologists must become familiar with valvular disease with CHD for appropriate care and referral practices. In this review, we summarize the embryology of the semilunar and atrioventricular (AV) valves as a foundation for understanding the origins of valvular CHD and describe the mechanisms that account for heterogeneity in disease. We then highlight the categories of pathology from the simple (e.g., bicuspid aortic valve, isolated pulmonic stenosis) to the more complex (e.g., Ebstein's anomaly, AV valvular disease in single ventricle circulations) with details on natural history, diagnosis, and contemporary therapeutic approaches. Care for CHD patients requires collaborative effort between providers, both CHD-specialized and not, to achieve optimal patient outcomes.
PubMed: 34295708
DOI: 10.21037/cdt-19-693-b -
HeartRhythm Case Reports Nov 2022
PubMed: 36618597
DOI: 10.1016/j.hrcr.2022.08.011 -
Journal of Pediatric Genetics Dec 2021Trisomy 21 is considered the most common chromosomal aneuploidy, and congenital heart disease (CHD) is highly prevalent and relevant to the morbidity and mortality of...
Trisomy 21 is considered the most common chromosomal aneuploidy, and congenital heart disease (CHD) is highly prevalent and relevant to the morbidity and mortality of these patients. Ebstein anomaly (EA) is a rare CHD characterized by tricuspid valve dysplasia with inferior septal leaflet displacement. Herein, we described a patient with trisomy 21 who presented with EA and discuss the association between the two conditions based on a literature review. We conclude that the concomitant occurrence of both conditions is considered to be rare. These individuals are most frequently diagnosed during birth and childhood, and they usually have a good prognosis, as observed with our patient and is typical for EA patients in general. However, it is important to be aware that electrophysiologic anomalies may also be present.
PubMed: 34849279
DOI: 10.1055/s-0040-1714360 -
Interactive Cardiovascular and Thoracic... Apr 2021Our goal was to evaluate the impact of the adult congenital heart disease anatomical and physiological (ACHD AP) classification system on the surgical management of...
OBJECTIVES
Our goal was to evaluate the impact of the adult congenital heart disease anatomical and physiological (ACHD AP) classification system on the surgical management of Ebstein anomaly (EA) in adult patients.
METHODS
From February 2000 through August 2017, data of patients aged at least 16 years, who underwent primary EA surgery, were retrospectively evaluated. The cohort was divided in 2 groups according to their ACHD AP classification: the moderate EA group (IIB, IIC) and the severe EA group (IID). Survival, freedom from reoperation and freedom from occurrence of major adverse advents were estimated.
RESULTS
There were 33 patients (21 women, 12 men). Eighteen belonged to the moderate group, 15 to the severe group. There were 12 female patients (80%) in the severe group. Patients in the moderate group were younger than those in the severe group (P = 0.02): 32 ± 12 vs 44 ± 15 years old. Thirty tricuspid valve repairs and 3 replacements were performed. Repair was mainly performed in the moderate group (P = 0.02). Overall survival was 90.1 ± 5.4% at 9 months after the operation and did not change in the later follow-up period. It was 100% for patients in the moderate group and 80.0 ± 10.3% in the severe group (P = 0.07), and 75.0 ± 12.5% for female patients of in the severe group compared to 100% for the remaining patients (P = 0.025). Survival free from major adverse events, including reoperation, at 10 years was 60.0 ± 12.6% in the moderate and 38.1% ± 12.9% in the severe group (P = 0.03). No patient in the moderate group evolved to be in the severe group at late follow-up.
CONCLUSION
Adult EA patients should undergo surgery earlier when they are still in the moderate ACHD AP classification.
Topics: Cardiac Surgical Procedures; Ebstein Anomaly; Female; Heart Defects, Congenital; Humans; Male; Retrospective Studies; Treatment Outcome; Tricuspid Valve Insufficiency
PubMed: 33313770
DOI: 10.1093/icvts/ivaa294 -
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 -
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 -
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 -
JTCVS Techniques Sep 2020
PubMed: 34317896
DOI: 10.1016/j.xjtc.2020.05.033 -
The Korean Journal of Thoracic and... Oct 2020Ebstein anomaly is a rare congenital heart malformation typically involving the tricuspid valve and the right ventricle that has a wide range of anatomical and...
Ebstein anomaly is a rare congenital heart malformation typically involving the tricuspid valve and the right ventricle that has a wide range of anatomical and pathophysiological presentations. Various surgical repair techniques for Ebstein anomaly have been reported because of its near-infinite anatomical variability. Cone repair for Ebstein anomaly can achieve nearly anatomical reconstruction of the tricuspid valve with promising outcomes. In this article, the surgical techniques for cone repair in adult patients with Ebstein anomaly are described in detail, and clinical experiences and technically challenging cases are presented.
PubMed: 33020344
DOI: 10.5090/kjtcs.20.113