Postablation Right Atrial Dissection in Ebstein's Anomaly.
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ABSTRACT:
SUBMITTER: Hassan F
PROVIDER: S-EPMC6058392 | biostudies-literature | 2018 Feb
REPOSITORIES: biostudies-literature
Publications
Postablation Right Atrial Dissection in Ebstein's Anomaly.
Hassan Fatemat F Patel Akash A Reddy Vadiyala Mohan VM Nawaytou Hythem H
CASE (Philadelphia, Pa.) 20171115 1
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Project description:IntroductionDespite the clinical benefits of the cone operation for Ebstein's anomaly, significant right ventricular (RV) dysfunction is frequently seen immediately after the procedure and if persistent may portend worse long-term outcomes. In this study we sought to evaluate the predictors of RV dysfunction after the cone operation using preoperative CMR.MethodsThis was a retrospective review of 26 consecutive patients who had the cone operation. Patients with significant RV dysfunction (RVD), defined as moderate or severe dysfunction by discharge echocardiogram, were compared to patients with no or mild dysfunction (no RVD).ResultsThe median age at the operation was 12.2 years (interquartile range (IQR): 4.9-31.7 years). Eighteen patients (69%) had RVD. Patients with RVD had worse preoperative RV ejection fraction (36 ± 15 vs 49 ± 11%, p = 0.02) and a larger cardiothoracic (CT) index (44 ± 8 vs 37 ± 6, p = 0.03). The tricuspid valve was more severely abnormal in the RVD group with higher rotational angle (45 ± 17 vs 23 ± 10°, 0.03) and higher displacement index (39 ± 18 vs 23 ± 12%, p = 0.02). RVD associated with a higher vasoactive inotropic score (P < 0.01) and a trend towards a longer intensive care stay (p = 0.07).ConclusionRVD is common after the cone operation and associated with higher need for postoperative inotropes. Predictors include lower preoperative RV ejection fraction, a more dilated heart and more severe tricuspid valve abnormality. Preoperative CMR is an important tool in preoperative assessment and helps predict RVD.
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Project description:Ebstein's anomaly is a congenital malformation characterised by tricuspid valve pathology with right heart enlargement. Cases of Ebstein's anomaly can vary widely in severity, anatomy and presentation. In this article, we presented three cases of Ebstein's anomaly and discussed the presentation as well as electrocardiographic (ECG) changes. Patients may first present to their primary care physicians with cardiac symptoms such as reduced effort tolerance together with an abnormal ECG. ECG changes suggestive of right heart enlargement are important in the initial consideration and eventual formal diagnosis of the condition.
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Project description:Endomyocardial fibrosis is a rare disease that is seen most commonly in tropical countries. It usually presents with characteristics of right-heart failure. Herein, we report the case of a 14-year-old adolescent boy who experienced endomyocardial fibrosis. Upon transthoracic echocardiography, the condition was mistakenly diagnosed as Ebstein's anomaly of the tricuspid valve. Sixteen months after undergoing tricuspid annuloplasty and receiving a bidirectional Glenn shunt, the patient showed no echocardiographic evidence of valvular regurgitation. We discuss imaging and surgical techniques that enable the diagnosis and treatment of endomyocardial fibrosis.
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Project description:The simultaneous occurrence of an atrioventricular canal defect (AVCD) and Ebstein's anomaly is extremely rare, occurring in less than 0.5% of all patients with AVCD. Only 22 cases are described in the literature. This patient's antenatal diagnosis of both Ebstein's anomaly and partial AVCD was made at 25 weeks of gestation. The delivery was organized in a tertiary center. The initial neonatal course was difficult but with adequate treatment, a rapid improvement allowed for a gap of almost 2 years before a complete surgical repair including a cone tricuspid plasty. To our knowledge, this is the first case of antenatal diagnosis, with carefully tailored delivery, neonatal care and subsequent follow-up before indication for successful surgery.
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Project description:In Ebstein's anomaly, maximal expansion in the atrialized right ventricle (RV) occurs during early diastole, whereas that of the functional RV occurs in late diastole, resulting in diastolic dyssynchronous expansion (DSE). We quantitatively assessed DSE and identified preoperative factors correlated with persistent DSE after surgery. Seventeen patients diagnosed with Ebstein's anomaly in whom transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) images available were retrospectively analyzed for quantitative DSE assessment and 10 patients who underwent surgery and postoperative TTE available were additionally analyzed for postoperative DSE. Severity of DSE was assessed by the time difference of maximal expansion between the atrialized and functional RV divided by the cardiac cycle length × 100 ("DSE index"). Relations between DSE and, clinical, electrophysiologic parameters and the severity of tricuspid valve (TV) tethering (the RV length / tethering height during diastole: "Tethering index") were assessed. In total patients, median DSE index and tethering index were 30.3 and 2.1 respectively, and the DSE index was correlated with tethering index (rs = 0.664, P = 0.004). In 10 patients who underwent surgery, this association remained after surgery and at 2-year follow up. Tethering index ≥2.5 separated patients with and without persistent DSE. In conclusion, DSE exists in Ebstein's anomaly. DSE index is related to the tethering index and DSE persists postoperatively if tethering index ≥ 2.5. As the persistent DSE might possibly impede the optimal recovery of RV function after surgery, severity of TV tethering should take into account in considering surgery.
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Project description:Traditional definitions of Ebstein's anomaly (EA) and left ventricular noncompaction (LVNC), two rare congenital heart defects (CHDs), confine disease to either the right or left heart, respectively. Around 15-29% of patients with EA, which has a prevalence of 1 in 20,000 live births, commonly manifest with LVNC. While individual EA or LVNC literature is extensive, relatively little discussion is devoted to the joint appearance of EA and LVNC (EA/LVNC), which poses a higher risk of poor clinical outcomes. We queried PubMed, Medline, and Web of Science for all peer-reviewed publications from inception to February 2022 that discuss EA/LVNC and found 58 unique articles written in English. Here, we summarize and extrapolate commonalities in clinical and genetic understanding of EA/LVNC to date. We additionally postulate involvement of shared developmental pathways that may lead to this combined disease. Anatomical variation in EA/LVNC encompasses characteristics of both CHDs, including tricuspid valve displacement, right heart dilatation, and left ventricular trabeculation, and dictates clinical presentation in both age and severity. Disease treatment is non-specific, ranging from symptomatic management to invasive surgery. Apart from a few variant associations, mainly in sarcomeric genes MYH7 and TPM1, the genetic etiology and pathogenesis of EA/LVNC remain largely unknown.
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