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
Graphical Abstract
Keywords
Abbreviations:
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)To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Seminars in Thoracic and Cardiovascular SurgeryAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum.Heart. 2019; 105: 1007-1013
- Pulmonary atresia with intact ventricular septum: from fetus to adult: congenital heart disease.Heart. 2008; 94: 1350-1357
- Natural history of pulmonary atresia with intact ventricular septum and right-ventricle-dependent coronary circulation managed by the single-ventricle approach.Ann Thorac Surg. 2006; 81: 2250-2258
- Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum.J Thorac Cardiovasc Surg. 2004; 127 (discussion 1007-1008): 1000-1007
- Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study.J thorac cardiovasc surg. 2005; 130: 1071
- Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum: a Congenital Heart Surgeons Society study.J thorac cardiovasc surg. 2013; 145 (e1013): 1018-1027
- Pulmonary atresia with intact ventricular septum with borderline tricuspid valve: how small is too small.Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2019; 22: 27-31
- MELD-XI: a rational approach to "sickest first" liver transplantation in cirrhotic patients requiring anticoagulant therapy.Liver Transpl. 2007; 13: 30-37
- Prognostic value of hepatorenal function by modified model for end-stage liver disease (MELD) score in patients undergoing tricuspid annuloplasty.J Am Heart Assoc. 2018; 7: 1-11
- Prognostic Role of Hepatorenal Function Indexes in Patients With Ebstein Anomaly.J Am Coll Cardiol. 2020; 76: 2968-2976
- Heart failure and liver disease: cardiohepatic interactions.JACC Heart Fail. 2019; 7: 87-97
- Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery.Circulation. 2008; 117: 85-92
- Evaluation and management of the child and adult with fontan circulation: a scientific statement from the american heart association.Circulation. 2019; 140: 234-284
- 40-Year follow-up after the fontan operation: long-term outcomes of 1,052 patients.J Am Coll Cardiol. 2015; 66: 1700-1710
- The fontan procedure for pulmonary atresia with intact ventricular septum: operative and late results.J Am Coll Cardiol. 1997; 29: 1359-1364
- Independent and additive prognostic value of right ventricular systolic function and pulmonary artery pressure in patients with chronic heart failure.J Am Coll Cardiol. 2001; 37: 183-188
- Relationship of right- to left-sided ventricular filling pressures in advanced heart failure: insights from the ESCAPE trial.Circ Heart Fail. 2013; 6: 264-270
- Late clinical features of patients with pulmonary atresia or critical pulmonary stenosis with intact ventricular septum after biventricular repair.Ann Thorac Surg. 2012; 94 (discussion 841): 833-841
- Long-term outcome for right heart function after biventricular repair of pulmonary atresia and intact ventricular septum.Jpn J Thorac Cardiovasc Surg. 2000; 48: 145-152
- Impaired cardiac reserve in asymptomatic patients with moderate pulmonary restenosis late after relief of severe pulmonary stenosis: evidence for diastolic dysfunction.Int J Cardiol. 2013; 167: 2836-2840
- Prognostic implications of inferior vena cava haemodynamics in ambulatory patients with tetralogy of Fallot.ESC Heart Fail. 2020; 7: 2589-2596
- Clinical outcomes of adult survivors of pulmonary atresia with intact ventricular septum.Int J Cardiol. 2012; 161: 13-17
- Restrictive right ventricular physiology and right ventricular fibrosis as assessed by cardiac magnetic resonance and exercise capacity after biventricular repair of pulmonary atresia and intact ventricular septum.Clin Cardiol. 2010; 33: 104-110
- Antegrade diastolic pulmonary arterial flow as a marker of right ventricular restriction after complete repair of pulmonary atresia with intact septum and critical pulmonary valvar stenosis.Cardiol Young. 1992; 2: 382-386
- Pulmonary Vascular Reserve in Fontan Patients: Looking Upstream for the True Heart of the Matter.J Am Coll Cardiol. 2020; 76: 2764-2767
- The Fontan circulation after 45 years: update in physiology.Heart. 2016; 102: 1081-1086
- Hemodynamic and clinical implications of impaired pulmonary vascular reserve in the fontan circulation.J Am Coll Cardiol. 2020; 76: 2755-2763
- Differences in right ventricular physiologic response to chronic volume load in patients with repaired pulmonary atresia intact ventricular septum/critical pulmonary stenosis versus tetralogy of fallot.Pediatr Cardiol. 2019; 40: 526-536
- Determinants of exercise function following univentricular versus biventricular repair for pulmonary atresia/intact ventricular septum.Am J Cardiol. 2006; 97: 1638-1643
- Exercise performance after surgery for pulmonary atresia and intact ventricular septum.Pediatr Cardiol. 2009; 30: 752-762
- Congestive hepatopathy.Clin Liver Dis (Hoboken). 2016; 8: 68-71
- The liver in circulatory Disturbances.Clin Liver Dis. 2019; 23: 209-220
- Surveillance for liver complications after the Fontan procedure.Congenit Heart Dis. 2017; 12: 124-132
- Relation of tricuspid regurgitation to liver stiffness measured by transient elastography in patients with left-sided cardiac valve disease.Am J Cardiol. 2016; 117: 640-646
- Fontan-associated liver disease: screening, management, and transplant considerations.Circulation. 2020; 142: 591-604
Article info
Publication history
Published online: October 21, 2021
Footnotes
Informed Consent: All patients provided consent for the use of their medical records for research.
Funding: None.
Conflicts of Interest: None.
Identification
Copyright
© 2021 Elsevier Inc. All rights reserved.
ScienceDirect
Access this article on ScienceDirectLinked Article
- Commentary: Liver: The Forgotten Organ in Adults With Congenital Heart DiseaseSeminars in Thoracic and Cardiovascular SurgeryVol. 34Issue 4
- Commentary: 1V, 1.5V, 2V – Does It Really Matter?Seminars in Thoracic and Cardiovascular SurgeryVol. 34Issue 4
- PreviewManagement 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.
- Full-Text
- Preview
