Systemic Venous Hypertension and Low Output Are Prevalent at Catheterization in Adults with Pulmonary Atresia and Intact Ventricular Septum Regardless of Repair Strategy

Published:October 21, 2021DOI:
      Patients with pulmonary atresia and intact ventricular septum (PA-IVS) require intervention early in life, and most survive to a definitive procedure of either Fontan circulation or right ventricle to pulmonary artery (RV-PA) repair. It remains unknown how surgical strategy impacts hemodynamics and comorbidities in adults. Retrospective analysis of adults (age ≥18 years) with PA-IVS undergoing hemodynamic catheterization at Mayo Clinic, MN between January 2000 through January 2020 was performed. 14 patients in the RV-PA group (71% biventricular, 29% 1.5 ventricle repair) and 19 post-Fontan patients [9 lateral tunnel (48%), 6 atriopulmonary (32%), and 4 extracardiac (21%)] were identified. Median age was 29 (21, 34) years. There were no differences in demographics and laboratory data (including MELD-XI) between groups. All patients assessed for liver disease had evidence of hepatic congestion or cirrhosis (14 in the Fontan group and 4 in the RV-PA group). Invasive hemodynamics were comparable between groups with the Fontan and RV-PA groups having similar systemic venous pressure (15.7±4.4 vs. 14.3±6.2, p = .44) and cardiac output (2.2±0.6 vs. 2.0±0.4 L/min/m2, p = .23). There was no difference in transplant-free survival (p = .92; 5-year transplant-free survival RV-PA 84%, Fontan 80%). Hemodynamic derangements, namely elevated systemic venous pressure and low cardiac output, are prevalent in patients with PA-IVS undergoing cardiac catheterization regardless of surgical strategy.

      Graphical Abstract



      CI (cardiac index), PA-IVS (pulmonary atresia with intact ventricular septum), PVRi (pulmonary vascular resistance index), RV (right ventricle), RV-PA (right ventricle to pulmonary artery)
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      Linked Article

      • Commentary: Liver: The Forgotten Organ in Adults With Congenital Heart Disease
        Seminars in Thoracic and Cardiovascular SurgeryVol. 34Issue 4
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          In the current issue of Seminars in Thoracic and Cardiovascular Surgery, Jain and colleagues1 reported on systemic venous hypertension and low cardiac output in adults with pulmonary atresia and intact ventricular septum (PA-IVS) after surgical repair.
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      • Commentary: 1V, 1.5V, 2V – Does It Really Matter?
        Seminars in Thoracic and Cardiovascular SurgeryVol. 34Issue 4
        • Preview
          Management of patients with pulmonary atresia and intact ventricular septum (PAIVS) varies widely depending on anatomic characteristics and institutional preference.1 Patients with right ventricle-dependent coronary circulation2 are managed as single-ventricle patients. For patients without right ventricle-dependent coronary circulation (Fig. 1), we (as many others) have advocated a staged approach with initial establishment of continuity between the right ventricle (RV) and pulmonary artery (PA) (with or without an aortopulmonary shunt) to promote RV growth, followed by an “RV overhaul” to enlarge the ventricular cavity by resecting muscle bundles.
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