Advertisement
Editorial Comment|Articles in Press

Austin Powers’ Steamroller: Lessons Learned to Benefit Patients with Congenital Heart Disease

Published:December 30, 2021DOI:https://doi.org/10.1016/j.cardfail.2021.12.015
      A structural crisis is brewing in advanced care for patients with congenital heart disease (CHD)—specifically, single-ventricle patients palliated by the Fontan procedure. The largest study evaluating management practices in pediatric cardiac teams found that 93% of providers believe that after the Fontan procedure, patients “will eventually have signs/symptoms of heart failure (HF) and will need a heart transplant (HT) at some point in their lives.”1 Despite this, the majority either disagreed about (45%) or were undecided about (24%) “whether routine evaluation by a HF/HT cardiologist is needed.” This may be, in part, attributable to the lack of an HF/HT subspecialty in the American College of Pediatrics, but these findings highlight a concerning disconnect in the minds of providers caring for patients undergoing the Fontan procedure, for whom the providers’ preparation seems incongruous in terms of the anticipated endpoint. This disconnect would likely be even worse had the survey been conducted in adult providers, given that the providers of care for adults with congenital heart disease (ACHD) have limited required HF/HT training, and adult HF/HT cardiologists have little required training in CHD.
      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 access
      One-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 Journal of Cardiac Failure
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Sadat-Hossieny S
        • Karamlou T
        • Marino BS
        • Gossett JG
        • Schumacher K
        • Patel A
        • et al.
        Contemporary provider management practices and attitudes towards referral for advanced heart failure therapies in Fontan patients across North America.
        J Card Fail. 2022; (in press)
        • Kempny A
        • Dimopoulos K
        • Uebing A
        • Moceri P
        • Swan L
        • Gatzoulis MA
        • et al.
        Reference values for exercise limitations among adults with congenital heart disease: relation to activities of daily life–single-centre experience and review of published data.
        Eur Heart J. 2012; 33: 1386-1396
        • Diller GP
        • Giardini A
        • Dimopoulos K
        • Gargiulo G
        • Müller J
        • Derrick G
        • et al.
        Predictors of morbidity and mortality in contemporary Fontan patients: results from a multicenter study including cardiopulmonary exercise testing in 321 patients.
        Eur Heart J. 2010; 31: 3073-3083
        • Mancini DM
        • Eisen H
        • Kussmaul W
        • Mull R
        • Edmunds Jr, LH
        • Wilson JR
        Value of peak exercise oxygen consumption for optimal timing of cardiac transplantation in ambulatory patients with heart failure.
        Circulation. 1991; 83: 778-786
      1. Schumacher K, Kindel S, ACTION ACHD/HF Awareness & Fontan Committee Members. Considerations for advanced heart failure consultation in Fontan patients: guidance for primary cardiologists. https://www.actionlearningnetwork.org/s/Failing-Fontan-Referral-Document-10_21_19.pdf. [Accessed December 14, 2021 ].

      2. Cedars A & ACTION ACHD/HF Awareness & Fontan Committee Members. Considerations for advanced heart failure consultation in congenital heart disease (CHD) patients: guidance for primary cardiologists. https://www.actionlearningnetwork.org/s/CHD-Referral-Document-2_6_20.pdf. Accessed December 14, 2021.

        • Rychik J
        • Atz AM
        • Celermajer DS
        • Deal BJ
        • Gatzoulis MA
        • Gewillig MH
        • et al.
        Evaluation and management of the child and adult with fontan circulation: a scientific statement from the American Heart Association.
        Circulation. 2019; (CIR0000000000000696)
        • Khairy P
        • Fernandes SM
        • Mayer Jr, JE
        • Triedman JK
        • Walsh EP
        • Lock JE
        • et al.
        Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery.
        Circulation. 2008; 117: 85-92
        • Verheugt CL
        • Uiterwaal CS
        • van der Velde ET
        • Meijboom FJ
        • Pieper PG
        • van Dijk APJ
        • et al.
        Mortality in adult congenital heart disease.
        Eur Heart J. 2010; 31: 1220-1229
      3. ACGME. Program requirements for graduate medical education in adult congenital heart disease. 2020. https://www.acgme.org/Portals/0/PFAssets/ProgramRequirements/153_AdultCongenitalHeartDisease_2020_TCC.pdf?ver=2020-06-26-132332-187. Accessed December 14, 2021.

        • Menachem JN.
        Advanced heart failure in the ACHD population: finding the fellows’ role in a growing field.
        J Am Coll Cardiol. 2017; 69: 1986-1989
      4. ACGME. Program requirements for graduate medical education in pediatric cardiology. 2020. https://www.acgme.org/Portals/0/PFAssets/ProgramRequirements/325_PediatricCardiology_2020.pdf?ver=2020-06-29-162718-490. Accessed December 14, 2021.

      Linked Article