Open Access

ARTICLE

Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study

Shiraz A. Maskatia¹, Christopher J. Petit^2,3, Curtis D. Travers², David J. Goldberg⁴, Lindsay S. Rogers⁴, Andrew C. Glatz⁴, Athar M. Qureshi⁶, Bryan H. Goldstein⁵, Jingning Ao^2,3, Ritu Sachdeva^2,3

1 Division of Pediatric Cardiology, Lucile Packard Children’s Hospital, Stanford University School of Medicine, Palo Alto, California
2 Department of Pediatrics, Division of Pediatric Cardiology, Emory University School of Medicine, Atlanta, Georgia
3 Children’s Healthcare of Atlanta, Sibley Heart Center Cardiology, Atlanta, Georgia
4 Division of Pediatric Cardiology, Children’s Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
5 Division of Cardiology, Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio
6 Division of Pediatric Cardiology, Texas Children’s Hospital, Baylor College of Medicine, Houston, Texas

* Corresponding Author: Shiraz Maskatia, Division of Pediatric Cardiology, Lucile Packard Children’s Hospital, Stanford University School of Medicine, 750 Welch Rd, Suite 325, Palo Alto, CA 94304‐5731, USA. Email:

Congenital Heart Disease 2018, 13(6), 892-902. https://doi.org/10.1111/chd.12671

Abstract

Background: In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echo‐ cardiographic parameters associated with 2V circulation, (2) identify echocardio‐ graphic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions.
Methods: We performed a retrospective analysis of patients who underwent RV de‐ compression for PA/IVS at four centers. We analyzed echocardiograms at baseline, postdecompression, and at follow up (closest to 1‐year or prior to Glenn circulation).
Results: Eighty‐one patients were included. At last follow‐up, 70 (86%) patients had 2V circulations, 7 (9%) had 1.5 ventricle circulations, and 4 (5%) had single ventricle circulations. Follow-up echocardiograms were available in 43 (53%) patients. The ma‐ jority of patients had improved RV systolic function, less tricuspid regurgitation (TR), and more left‐to‐right atrial shunting at a median of 350 days after decompression. Multivariable analysis demonstrated that larger baseline tricuspid valve (TV) z‐score (P = .017), ≥ moderate baseline TR (P = .045) and smaller baseline RV area (P < .001) were associated with larger increases in RV area. Baseline RV area ≥6 cm² /m² had 93% sensitivity and 80% specificity for identifying patients who ultimately achieved 2V circulation. All patients with RV area ≥8 cm² /m² at follow up achieved 2V circula‐ tion. This finding was confirmed in a validation cohort from a separate center (N = 25). Factors associated with achieving RV area ≥8 cm² /m² included larger TV z‐score (P = .004), ≥ moderate baseline TR (P = .031), and ≥ moderate postdecompression pulmonary regurgitation (P = .002).
Conclusions: Patients with PA/IVS and smaller TV annuli are at risk for poor RV growth. Volume‐loading conditions signal increased capacity for growth sufficient for 2V circulation.

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