- •The ventricular assist device (VAD) therapy is getting common in advanced heart failure treatment for bridge to transplant and destination therapy.
- •The adult congenital heart disease (ACHD) patients with advanced heart failure had less access to the VAD therapy and the heart transplant in the past.
- •The ACHD patients have similar survival and improvement of quality of life after left VAD implantation compared to non-ACHD patients.
As long-term surgical outcome of congenital heart disease has continued to improve, most pediatric patients with congenital heart disease are able to reach adulthood. However, adult congenital heart disease (ACHD) patients have increased risk of arrhythmia, valvular diseases, infectious endocarditis, and heart failure. The end-stage ACHD patients with advanced heart failure may require mechanical circulatory support to improve the heart failure symptoms or to recover from circulatory collapse, and may eventually aim to heart transplant or destination therapy. In general, long-term mechanical support for dilated cardiomyopathy or ischemic cardiomyopathy has been achieved with left ventricular assist device with excellent survival outcomes and improved quality of life. However, the ventricular assist device for end-stage ACHD patients can be challenging due to patient-specific anatomical feature, multiple histories of surgical and catheter-based interventions and possible multiple end-organ dysfunctions, and offered less frequently compared to non-ACHD patients.
The Interagency Registry for Mechanically Assisted Circulatory Support data published recently showed that ACHD patients receiving long-term mechanical circulatory support consisted <1 % of all registrants and had higher mortality after mechanical support than non-ACHD patients. However, the ACHD patients supported with left ventricular assist device had similar survival with non-ACHD patients and a large proportion of the mortality difference between ACHD and non-ACHD patients seemed to result from operative and perioperative factors. Therefore, the ventricular assist device therapy can be an excellent treatment for selected ACHD patients.
In this paper, we describe the current status of ventricular assist device support for end-stage ACHD patients and consideration to the future.
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 Cardiology
Already a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
- Mortality in adult congenital heart disease: analysis of outcomes and risk stratification.J Cardiothorac Vasc Anesth. 2022; 36: 3379-3388
- Heart failure related to adult congenital heart disease: prevalence, outcome and risk factors.ESC Heart Fail. 2021; 8: 2940-2950
- The society of thoracic surgeons intermacs database annual report: evolving indications, outcomes, and scientific partnerships.J Heart Lung Transplant. 2019; 38: 114-126
- Discontinuity of cardiac follow-up in young people with congenital heart disease transitioning to adulthood: a systematic review and meta-analysis.J Am Heart Assoc. 2021; 10e019552
- Development of circulatory-renal limitations to angiotensin-converting enzyme inhibitors identifies patients with severe heart failure and early mortality.J Am Coll Cardiol. 2003; 41: 2029-2035
- The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: thirty-eighth adult lung transplantation report - 2021; focus on recipient characteristics.J Heart Lung Transplant. 2021; 40: 1060-1072
- The artificial heart program: current status and history.in: Hogness J.R. VanAntwerp M. The artificial heart: prototypes, policies, and patients. National Academy Press, Washington, D.C1991: 14-25
- A history of devices as an alternative to heart transplantation.Heart Fail Clin. 2014; 10: S1-S12
- Berlin heart EXCOR pediatric ventricular assist device for bridge to heart transplantation in US children.Circulation. 2013; 127: 1702-1711
- Long-term use of a left ventricular assist device for end-stage heart failure.N Engl J Med. 2001; 345: 1435-1443
- Advanced heart failure treated with continuous-flow left ventricular assist device.N Engl J Med. 2009; 361: 2241-2251
- A fully magnetically levitated circulatory pump for advanced heart failure.N Engl J Med. 2017; 376: 440-450
- A fully magnetically levitated left ventricular assist device - final report.N Engl J Med. 2019; 380: 1618-1627
- The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: thirty-fifth adult heart transplantation report-2018; focus theme: multiorgan transplantation.J Heart Lung Transplant. 2018; 37: 1155-1168
- The Society of Thoracic Surgeons Intermacs 2020 annual report.Ann Thorac Surg. 2021; 111: 778-792
- Does small size matter with continuous flow devices?: analysis of the INTERMACS database of adults with BSA ≤1.5 m2. JACC.Heart Fail. 2017; 5: 123-131
- Outcomes of pediatric patients supported with continuous-flow ventricular assist devices: a report from the Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS).J Heart Lung Transplant. 2016; 35: 585-590
- Early experience with the HeartMate 3 continuous-flow ventricular assist device in pediatric patients and patients with congenital heart disease: a multicenter registry analysis.J Heart Lung Transplant. 2020; 39: 573-579
- Greater admissions, mortality and cost of heart failure in adults with congenital heart disease.Heart. 2021; 107: 807-813
- Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow-up at a large tertiary centre.Circulation. 2015; 132: 2118-2125
- Hospitalization trends and health resource use for adult congenital heart disease-related heart failure.J Am Heart Assoc. 2018; 7e008775
- Clinical outcomes during admissions for heart failure among adults with congenital heart disease.J Am Heart Assoc. 2019; 8e012595
- Morbidity and mortality risk factors in adults with congenital heart disease undergoing cardiac reoperations.Ann Thorac Surg. 2009; 88: 1284-1289
- Waitlist outcomes for adults with congenital heart disease listed for heart transplantation in the US.J Am Coll Cardiol. 2016; 68: 908-917
- Outcomes of heart transplantation in adult congenital heart disease with prior intracardiac repair.Ann Thorac Surg. 2021; 112: 846-853
- Organ allocation in adults with congenital heart disease listed for heart transplant: impact of ventricular assist devices.J Heart Lung Transplant. 2013; 32: 1059-1064
- Implantable left ventricular assist device: indications, eligibility and current outcomes.Heart. 2022; 108: 233-241
- Mechanical support for the failing single ventricle at pre-Fontan stage: current state of the field and future directions.Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2021; 24: 10-18
- First use of HeartMate 3 in a failing Fontan circulation.Ann Thorac Surg. 2018; 106: e233-e234
- Ventricular assist device implantation late after double switch operation for L-transposition of the great arteries.Ann Thorac Surg. 2014; 98: e109-e111
- Mechanical support for patients with congenitally corrected transposition of the great arteries and end-stage ventricular dysfunction.Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2019; 22: 66-73
- Heartmate 3 as a bridge to heart transplantation in a patient with congenitally corrected transposition of the great arteries: a case report.J Cardiothorac Surg. 2022; 17: 54
- Outcomes following implantation of mechanical circulatory support in adults with congenital heart disease: an analysis of the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS).J Heart Lung Transplant. 2018; 37: 89-99
- An Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) analysis of hospitalization, functional status, and mortality after mechanical circulatory support in adults with congenital heart disease.J Heart Lung Transplant. 2018; 37: 619-630
- Third Annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) Report: preimplant characteristics and outcomes.Ann Thorac Surg. 2019; 107: 993-1004
- Systemic ventricular assist device support in Fontan patients: a report by ACTION.J Heart Lung Transplant. 2021; 40: 368-376
Published online: October 28, 2022
Accepted: September 14, 2022
Received: September 2, 2022
© 2022 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved. All rights reserved.