Elsevier

Progress in Cardiovascular Diseases

Volume 61, Issues 3–4, September–October 2018, Pages 377-381
Progress in Cardiovascular Diseases

When Should Adult Congenital Heart Disease Patients be Considered for Transplant and Deciding Which Organs to Transplant☆

https://doi.org/10.1016/j.pcad.2018.09.004Get rights and content

Abstract

The number of adult congenital heart disease (ACHD) patients continues to increase. Because of multiple related factors such as aging, residual cardiac lesions and prior palliative procedures, advanced heart failure (HF) is increasingly prevalent in this population. Consequently, there is an emerging need to determine which patients are best suited for advanced cardiac therapies, including heart transplantation (HT) and mechanical circulatory support. Unfortunately, optimizing patient selection for these therapies is complicated by patient heterogeneity, variable HF presentation across lesion-type, and a paucity of outcome data. The lack of patient specific data and the increasing number of ACHD patients with end-stage HF identifies a need to more precisely stratify risk and determine appropriate timing for referral. As such, this article will discuss the clinical recognition and classification of advanced HF in the ACHD patient populations and review current data regarding HT outcomes. Following, key considerations regarding the timing of HT in specific forms of ACHD, the role of device therapy, and when dual organ transplantation should be considered will be reviewed. Finally, existing knowledge gaps and key research needs will be highlighted.

Section snippets

Presentation and classification of HF in ACHD patients

Because of the heterogeneity implicit in the ACHD population, the presentation of HF can vary dramatically. Patients with simple forms of ACHD, such as isolated valvular disease or repaired shunting lesions, may present with similar HF symptoms as the population at large. However, patients with complex forms of ACHD often fail to exhibit signs and symptoms classically associated with ventricular dysfunction.8 Decades of abnormal cardiac anatomy and physiology can lead to compensatory mechanisms

HT outcomes in ACHD patients

The increasing number of ACHD patients facing end-stage HF identifies an opportunity to stratify risk more precisely and preempt complications to improve mortality and quality of life (QoL). As such, post-HT outcomes are crucial to understanding ongoing areas of uncertainty in patient selection and timing of referral. The majority of HT outcome data in the ACHD population is derived from registry data or retrospective, single center studies.16., 17., 18., 19. Consequently, available data either

General indications for HT

There are no evidence-based guidelines to help guide medical therapy for advanced HF and there are no consensus recommendations regarding indications for HT in the ACHD population. Transplant referral decisions are made on a case-by-case basis either by the patient's primary ACHD cardiologist or in tandem with providers from advanced HF. Generally, transplant evaluation is sought in patients with progressive symptomology and/or a poor prognosis when medical therapy, non-transplant cardiac

The adult Fontan patient

Historically, adult Fontan patents have higher post-HT mortality than other ACHD diagnoses.27 Whether this indicates an implicit tendency towards worse outcomes as a consequence of chronic systemic venous hypertension, is indicative of poor patient selection, or is a result of poor referral timing remains to be determined. Much of the difficulty in defining the appropriate indications for transplant for patients with prolonged Fontan circulation is a consequence of the different clinical

The systemic right ventricle (SRV)

The SRV features prominently in multiple forms of ACHD and includes both patients with single and biventricular circulation.34 Overall, the prognosis of the systemic right ventricle is highly variable, with some patients requiring transplant in their second and third decades and others achieving a near normal lifestyle. Patients with a failing SRV often present similarly to patients with HF and a morphologic left ventricle; however, long-term exposure to systemic pressure and residual lesions

Eisenmenger's syndrome and combined HT-lung transplant

The role of HT-lung transplant for Eisenmenger's syndrome and select patients with complex ACHD and pulmonary hypertension remains unclear. Despite uncertainty regarding its application in the ACHD population, combined HT-lung transplant remains the definitive treatment for Eisenmenger's syndrome, accounting for nearly one third of all combined HT-lung transplants to date. However, because clinical stability can often be achieved with pulmonary vasodilators and medical surveillance alone, the

Mechanical support

Utilization of MCS in ACHD patients with advanced HF as a bridge to HT or as destination therapy deserves consideration in select clinical scenarios. ACHD patients are more likely to die while waiting for HT after being listed and are more likely to be delisted secondary to clinical decline.40 Increased use of MCS in the ACHD population may help partially ameliorate these issues by improving prioritization on the wait list and providing hemodynamic support to listed ACHD patients with worsening

Future directions

With improved collaboration, multidisciplinary research to improve outcomes in ACHD patients with advanced HF is possible. Clearly, collaborative, phenotypically enriched datasets are necessary to determine best practices for patient selection and transplant referral. However, the combination of organ scarcity and the increasing population need for HT as an end-stage therapy creates a situation where organ allocation will be increasingly competitive. Novel methods of treatment are necessary to

Conclusions

The number of ACHD patients who require HT continues to grow. Patient selection, timing of referral, and indications for dual organ transplant remain largely dependent on institutional factors with insufficient data to allow for standardization across sites. ACHD patients have higher perioperative and early post-HT mortality when compared to non-ACHD patients. Ongoing efforts to promote multi-center collaboration and advance clinical research may allow for the development of lesion-specific

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    ☆

    The authors have no conflicts of interest to report.

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