10.6084/m9.figshare.7046165.v1
Amber Malhotra
Amber
Malhotra
Vishal Agrawal
Vishal
Agrawal
Kartik Patel
Kartik
Patel
Mausam Shah
Mausam
Shah
Kamal Sharma
Kamal
Sharma
Pranav Sharma
Pranav
Sharma
Sumbul Siddiqui
Sumbul
Siddiqui
Nilesh Oswal
Nilesh
Oswal
Himani Pandya
Himani
Pandya
Ebstein's Anomaly: "The One and a Half Ventricle Heart"
SciELO journals
2018
Ebstein Anomaly
Heart Defects, Congenital
Cardiovascular Surgical Procedures
2018-09-05 02:50:50
Dataset
https://scielo.figshare.com/articles/dataset/Ebstein_s_Anomaly_The_One_and_a_Half_Ventricle_Heart_/7046165
<div><p>Abstract Objective: Ebstein'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). Methods: 22 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. Results: There 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). Conclusion: In 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.</p></div>