The Da Silva cone operation after the Starnes procedure for Ebstein's anomaly: New surgical strategy and initial results.
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ABSTRACT:
SUBMITTER: Da Silva JP
PROVIDER: S-EPMC8302946 | biostudies-literature | 2020 Sep
REPOSITORIES: biostudies-literature
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The Da Silva cone operation after the Starnes procedure for Ebstein's anomaly: New surgical strategy and initial results.
Da Silva Jose Pedro JP Viegas Melita M Castro-Medina Mario M Da Fonseca Da Silva Luciana L
JTCVS techniques 20200517
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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.
| S-EPMC11658122 | biostudies-literature
Project description:BackgroundThe volume-outcome relationship is well-known in health care. The Experience Curve, initially developed by the Boston Consulting Group for manufacturing, offers insight on this relationship and has never before been applied to health care.ObjectivesThe purpose of this study was to determine the effect of experience on cost and resource utilization for the Cone procedure.MethodsWe performed a retrospective review of patients who underwent Cone reconstruction for Ebstein's anomaly at Boston Children's Hospital between October 2010 and October 2021. Cardiopulmonary bypass time and aortic cross clamp time over time were evaluated using exponential regression to assess the surgeon-level learning curve. At the hospital level, length of stay and cost over time were assessed using exponential regression.ResultsThere were 115 patients included in the study. Median hospital length of stay was 7.9 days (IQR: 6.4-10.2 days) with a decline of 3.3% per year. Median intensive care unit [ICU] length of stay was 3.2 days (IQR: 2-5.7 days) with a decline of 10.5% per year. Adjusted direct costs indexed to 2020 prices fell by 4% per year. There was no statistically significant change in cardiopulmonary bypass or aortic cross clamp time. In mediation analysis, the reduction in cost was completely accounted for in the decline in ICU length of stay. ICU length of stay was correlated with duration of intubation.ConclusionsIncreasing familiarity with Ebstein's anomaly and Cone reconstruction led to a reduction in resource utilization.
| S-EPMC11299563 | biostudies-literature
Project description:ObjectiveEbstein's anomaly is a rare congenital heart malformation for which surgical and medical management are still controversial. The cone repair has transformed surgical outcomes in many of these patients. We aimed to present our results on the outcomes of patients with Ebstein's anomaly who underwent a cone repair or tricuspid valve replacement.MethodsA total of 85 patients who underwent a cone repair (mean age, 16.5 years) or tricuspid valve replacement (mean age, 40.8 years) between 2006 and 2021 were included. Univariate, multivariate, and Kaplan-Meier analyses were used to evaluate operative and long-term outcomes.ResultsResidual/recurrent greater than mild-to-moderate tricuspid regurgitation at discharge was higher after cone repair compared with tricuspid valve replacement (36% vs 5%; P = .010). However, at last follow-up, the risk of greater than mild-to-moderate tricuspid regurgitation was not different between groups (35% in the cone group vs 37% in the tricuspid valve replacement group; P = .786). The tricuspid valve replacement group had a higher risk of tricuspid valve reoperation (37% vs 9%; P = .005) and tricuspid stenosis (21% vs 0%; P = .002) compared with the cone repair group. Kaplan-Meier freedom from reintervention was 97%, 91%, and 91% at 2, 4, and 6 years after cone repair, respectively, and 84%, 74%, and 68% at 2, 4, and 6 years after tricuspid valve replacement, respectively (P = .0191). At last follow-up, right ventricular function was significantly worse from baseline in the tricuspid valve replacement group (P = .0294). There were no statistical differences between age-stratified cohorts or surgeon volume in the cone repair group.ConclusionsThe cone procedure offers excellent results, with stable tricuspid valve function and low reintervention and death rates at last follow-up. The rate of greater than mild-to-moderate residual tricuspid regurgitation at discharge was higher after cone repair compared with tricuspid valve replacement, but this did not expose the patient to a higher risk of reoperation or death at last follow-up. Tricuspid valve replacement was associated with a significantly higher risk of tricuspid valve reoperation and tricuspid valve stenosis, and worse right ventricular function at last follow-up.
| S-EPMC10328840 | biostudies-literature
Project description:Background and objectiveWe aimed to investigate long-term clinical and echocardiographic outcomes, including tricuspid valve durability, annular growth, and left ventricular reverse remodeling, after modified cone reconstruction in patients with Ebstein's anomaly.MethodsThis was a retrospective analysis of all pediatric patients who underwent modified cone reconstruction for Ebstein's anomaly at a single tertiary center between January 2005 and June 2021.ResultsA total of 14 pediatric patients underwent modified cone reconstruction for Ebstein's anomaly; the median age was 5.8 years (range, 0.01-16.6). There were three patients (21.4%) with Carpentier type B, ten patients with Carpentier type C (71.4%), and one patient with Carpentier type D (7.1%). There was no early or late mortality, arrhythmia, or readmission for heart failure at a 10-year follow-up. There were no cases of more than mild tricuspid stenosis or more than moderate tricuspid regurgitation during the study period, except for one patient with severe tricuspid regurgitation who underwent reoperation. The z value for tricuspid valve annular size significantly decreased immediately after the operation (2.46 vs. -1.15, p<0.001). However, from 1 year to 7 years after surgery, the z values were maintained between -1 and +1. Left ventricular end-systolic volume, end-diastolic volume, and stroke volume increased after surgery and remained elevated until seven years postoperatively.ConclusionsEbstein's anomaly in children can be repaired by modified cone reconstruction with low mortality and morbidity, good tricuspid valve durability, and annular growth relative to somatic growth.
| S-EPMC10864249 | biostudies-literature
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| S-EPMC6058392 | biostudies-literature
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.
| S-EPMC6875802 | biostudies-literature
Project description: Not available
| S-EPMC7501924 | biostudies-literature
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.
| S-EPMC2696495 | biostudies-literature
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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