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Congenit Heart Dis. 2018 Nov;13(6):892-902. doi: 10.1111/chd.12671. Epub 2018 Sep 21.

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.

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



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 echocardiographic parameters associated with 2V circulation, (2) identify echocardiographic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions.


We performed a retrospective analysis of patients who underwent RV decompression 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).


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 majority 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 circulation. 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).


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.


catheter-based intervention; congenital heart disease; pediatric echocardiography; pulmonary atresia intact ventricular septum

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