Da Silva's cone repair for Ebstein's anomaly: effect on right ventricular size and function

Lange, Rüdiger; Burri, Melchior; Eschenbach, Lena Katharina; Badiu, Catalin Constantin; Silva, José Pedro da; Nagdyman, Nicole; Fratz, Sohrab; Hörer, Jürgen; Kühn, Andrea A.; Schreiber, Christian; Vogt, Manfred

doi:10.1093/ejcts/ezu472

Cited by 47 publications

(33 citation statements)

References 21 publications

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“…As in our work, prior studies have found that the cone operation leads to a decrease in RV EDV [ 6 , 12 – 14 ]. The effect on RV EF is less consistent with some investigations showing no change [ 6 , 12 ] as we found, and others showing a decrease [ 13 , 14 ]. This discrepancy may reflect variations among the studies in the indications for surgery and timing of the postoperative evaluation, and requires further study.…”

Section: Discussionsupporting

confidence: 79%

Impact of the cone operation on left ventricular size, function, and dyssynchrony in Ebstein anomaly: a cardiovascular magnetic resonance study

Beroukhim

Jing

Harrild

et al. 2018

Journal of Cardiovascular Magnetic Resonance

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BackgroundIn addition to tricuspid regurgitation (TR) and right ventricular (RV) enlargement, patients with Ebstein anomaly are at risk for left ventricular (LV) dysfunction and dyssynchrony. We studied the impact of the cone tricuspid valve reconstruction operation on LV size, function, and dyssynchrony.MethodsAll Ebstein anomaly patients who had both pre- and postoperative cardiovascular magnetic resonance (CMR) studies were retrospectively identified. From cine images, RV and LV volumes and ejection fractions (EF) were calculated, and LV circumferential and longitudinal strain were measured by feature tracking. To quantify LV dyssynchrony, temporal offsets (TOs) were computed among segmental circumferential strain versus time curves using cross-correlation analysis and patient-specific reference curves. An LV dyssynchrony index was calculated as the standard deviation of the TOs.ResultsTwenty patients (65% female) were included with a median age at cone operation of 16 years, and a median time between pre- and postoperative CMR of 2.8 years. Postoperatively, there was a decline in the TR fraction (56 ± 19% vs. 5 ± 4%, p < 0.001), RV end-diastolic volume (EDV) (242 ± 110 ml/m2 vs. 137 ± 82 ml/m2, p < 0.001), and RV stroke volume (SV) (101 ± 35 vs. 51 ± 7 ml/m2, p < 0.001). RV EF was unchanged. Conversely, there was an increase in both LV EDV (68 ± 13 vs. 85 ± 13 ml/m2, p < 0.001) and LV stroke volume (37 ± 8 vs. 48 ± 6 ml/m2, p < 0.001). There was no change in LV EF, or global circumferential and longitudinal strain but basal septal circumferential strain improved (16 ± 7% vs. 22 ± 5%, p = 0.04). LV contraction become more synchronous (dyssynchrony index: 32 ± 17 vs. 21 ± 9 msec, p = 0.02), and the extent correlated with the reduction in RV EDV and TR.ConclusionsIn patients with the Ebstein anomaly, the cone operation led to reduced TR and RV stroke volume, increased LV stroke volume, improved LV basal septal strain, and improved LV synchrony. Our data demonstrates that the detrimental effect of the RV on LV function can be mitigated by the cone operation.

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Section: Discussionsupporting

confidence: 79%

Impact of the cone operation on left ventricular size, function, and dyssynchrony in Ebstein anomaly: a cardiovascular magnetic resonance study

Beroukhim

Jing

Harrild

et al. 2018

Journal of Cardiovascular Magnetic Resonance

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“…Today, if surgery is indicated, most patients with Ebstein's anomaly are managed with a cone operation 3 at our centre with very good results. 4 However, in patients in whom tricuspid valve reconstruction results in unacceptable haemodynamics, a cavo-pulmonary shunt, hence a so-called 1 ½ circulation, may be helpful as it was in our patient. 2,5,6 Severe intrapulmonary shunts with subsequent increasing arterial cyanosis are a known long-term complication after a classical Glenn operation or a Kawashima procedure, where the hepatic blood does not reach parts of the pulmonary arteries.…”

Section: K Gendera Et Almentioning

confidence: 65%

Spontaneous closure of arterio-venous pulmonary fistulas by redirection of hepatic venous blood 9 years after Glenn anastomosis in a 12-year-old girl

2019

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We report on a 12-year-old girl with Ebstein’s anomaly after a unidirectional Glenn procedure with surgical ligation of the proximal right pulmonary artery, who suffered from significant central cyanosis caused by multiple arterio-venous fistulas in the right lung. The continuity between the right pulmonary artery and the pulmonary trunk was restored with the use of radiofrequency perforation and consecutive covered stent implantation.

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“…In recent years, many authors have published successful management of Ebstein's anomaly using biventricular approaches [19-21] . In our series, the residual RV after plication of ARV was 70% of the expected indexed RV volume, which precludes a biventricular repair.…”

Section: Discussionmentioning

confidence: 99%

“…In our series, the residual RV after plication of ARV was 70% of the expected indexed RV volume, which precludes a biventricular repair. Lange et al [21] have not added a BCPS to their repair, probably because of a different subset of patients in their series, as their postoperative RV end diastolic volume was close to normal. However, they left an interatrial communication of 5-6 mm in their patients, which would act as a trigger to decompress a failing RV.…”

Section: Discussionmentioning

confidence: 99%

Ebstein’s Anomaly: “The One and a Half Ventricle Heart”

Malhotra¹,

Agrawal²,

Patel³

et al. 2018

Braz J Cardiovasc Surg

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ObjectiveEbstein's anomaly remains a relatively ignored disease. Lying in the 'No Man's land' between congenital and valve surgeons, it largely remains inadequately studied. We report our short-term results of treating it as a 'one and a half ventricle heart' and propose that the true tricuspid annulus (TTA) 'Z' score be used as an objective criterion for estimation of 'functional' right ventricle (RV).Methods22 consecutive patients undergoing surgery for Ebstein's anomaly were studied. Echocardiography was performed to assess the type and severity of the disease, tricuspid annular dimension and its 'Z' score. Patients were operated by a modification of the cone repair, with addition of annuloplasty, bidirectional cavopulmonary shunt (BCPS) and right reduction atrioplasty to provide a comprehensive repair. TTA 'Z' score was correlated later with postplication indexed residual RV volume.ResultsThere was one (4.5%) early and no late postoperative death. There was a significant reduction in tricuspid regurgitation grading (3.40±0.65 to 1.22±0.42, P<0.001). Residual RV volume reduced to 71.96±3.8% of the expected volume and there was a significant negative correlation (rho −0.83) between TTA 'Z' score and indexed residual RV volume. During the follow-up of 20.54±7.62 months, the functional class improved from 2.59±0.7 to 1.34±0.52 (P<0.001).ConclusionIn Ebstein's anomaly, a higher TTA 'Z' score correlates with a lower postplication indexed residual RV volume. Hence, a complete trileaflet repair with offloading of RV by BCPS (when the TTA 'Z' score is >2) is recommended. The short-term outcomes of our technique are promising.

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Da Silva's cone repair for Ebstein's anomaly: effect on right ventricular size and function

Abstract: Da Silva's cone repair for Ebstein's anomaly creates excellent valve function in all patients. Consecutively, the size of the RV decreases and the antegrade net stroke volume increases 6 months after the operation.

Cited by 47 publications

References 21 publications

Impact of the cone operation on left ventricular size, function, and dyssynchrony in Ebstein anomaly: a cardiovascular magnetic resonance study

Impact of the cone operation on left ventricular size, function, and dyssynchrony in Ebstein anomaly: a cardiovascular magnetic resonance study

Spontaneous closure of arterio-venous pulmonary fistulas by redirection of hepatic venous blood 9 years after Glenn anastomosis in a 12-year-old girl

Ebstein’s Anomaly: “The One and a Half Ventricle Heart”

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