The latest JACC mini-spotlight issue focuses on the new 2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease (ACHD), which replaces and updates the 2018 recommendations for monitoring, counseling and treating ACHD.

As JACC Editor-in-Chief Harlan M. Krumholz, MD, SM, FACC, writes in an Editor’s Page, major advances in ACHD treatment have substantially extended life expectancy and shifted the scientific conversation: “When survival was rare, the focus was rescue. When survival becomes the norm, the focus must shift to stewardship. Stewardship requires systems, not just expertise.”

“Few areas of cardiology so clearly illustrate both the promise and the responsibility created by success,” he adds, citing ongoing challenges of delivering hyperspecialized care at scale, confronting patient drop-off and workforce shortages, and generating evidence in a small, heterogeneous patient population. He writes the issue reflects “the collective effort of a field that has learned to organize itself around lifelong care rather than episodic intervention.”

“Our goal is to spark a transformation in the access and delivery of ACHD care, in addition to elevating the scientific discoveries necessary to meet the evolving needs of this growing patient population,” writes JACC Associate Editor Anne Marie Valente, MD, FACC, framing the guideline as the end result of a long journey that began when JACC published the 22nd Bethesda Conference Report, Congenital Heart Disease After Childhood: An Expanding Patient Population in 1991. Other expert voices provide a variety of insights on the guideline and putting it into practice.

The Future of Adult Congenital Heart Care in the United States: Are We Keeping Up With the Pace of Progress? In this commentary, Curt J. Daniels, MD, FACC; Ariane Marelli, MD, FACC; and Jeannette P. Lin, MD, FACC, chart the past, present and future of ACHD care in the U.S., with only 500 board-certified ACHD cardiologists and 56 Adult Congenital Heart Association-accredited Comprehensive Care Centers to care for a patient population currently at 1.69 million and expected to grow to 2.1 million by 2050. Addressing this expanding gap in a manner consistent with the ACHD guideline will “require coordinated national strategies to increase provider capacity, improve regional access and innovate care delivery models,” summarized in a figure outlining strategies in this effort.

What Has Changed Since the First ACC/ AHA Guidelines for the Management of Adult Congenital Heart Disease in 2008? In this commentary, Carole A. Warnes, MD, FACC, and Anne Marie Valente, MD, FACC, survey the changing landscape in ACHD over the past 18 years, noting that more cardiologists have trained in this field, more centers emerged across the country, shared decision-making is supported and percutaneous therapies have revolutionized its management. The guideline recommendation for all patients with ACHD to have at least one evaluation by an ACHD cardiologist is highlighted. They provide a detailed look at changes in heart failure and transplant; secundum atrial septal defect and pulmonary arterial hypertension; single ventricle and Fontan; tetralogy of Fallot, and reproductive health. “We anticipate the incorporation of multicenter trials, precision medicine and artificial intelligence to inform our future management strategies,” they conclude. “This, combined with continued evolution and utilization of percutaneous therapies in ACHD patients, will hopefully enhance outcomes for this most deserving population.”

2025 ACC/AHA/HRS/ISACHD/SCAI Guideline Recommendations for the Treatment of Adult Congenital Heart Disease and Comparison With the 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: As with all aging adults, those with ACHD likely will develop valvular heart disease (VHD) along with other cardiovascular and cerebrovascular diseases. In this commentary, Patrick T. O’Gara, MD, MACC, compares the 2025 ACHD and 2020 VHD guideline (now in revision) to help “facilitate individualized application of selected recommendations.” In highlighting the importance of the multidisciplinary heart team overall, he notes that the “dynamics and composition” of the team and the “iterative need for longitudinal shared decision-making” may be of even greater importance among ACHD patients with VHD because of the “prospect of multiple valve interventions over their lifetime.” O’Gara discusses guideline recommendations for bicuspid aortic valve disease, tricuspid valve disease and pulmonic valve disease, as well as lifetime management considerations.

The ACHD Guideline: A Surgeon’s Perspective: As a congenital cardiac surgeon, Emile Bacha, MD, FACC, writes that, “ACHD management is first and foremost a team sport, with a linchpin of ACHD cardiac surgery and cardiology.” In this regard, he notes that as stated in the guideline, a key component is intrasurgical collaboration, which can improve surgical planning and outcomes. The importance of earlier surgical referral, the need for formally trained ACHD surgeons, broader use of advanced imaging and mechanical support, along with emerging unanswered questions are discussed.

2025 Adults With Congenital Heart Disease Guideline From the Early-Career Perspective: Lidija B. McGrath, MD; Katherine Salciccioli, MD, FACC; Adam Small, MD, FACC; and Jill M. Steiner, MD, MS, FACC, write in this commentary from the perspective of when “the safety net of attending oversight disappears.” They suggest that pairing the guideline document alongside the electronic health record could be a “valuable decision aid in the clinic…solidifying good practice habits and preventing outdated or harmful care.” They highlight the benefit provided by clear connections between recommendations for therapeutic interventions and rationale, enhanced figures and simplified tables, as well as exploring guidance gaps and potential future directions, including a greater focus on enhancing quality of life and long-term care goals.

Adapting the 2025 ACC/AHA/HRS/ ISACHD/SCAI Guideline for Management of Adults With Congenital Heart Disease to Low Resource Settings: Babar S. Hasan, MBBS, and Raman Krishna Kumar, MD, DM, in this commentary propose  a number of actions to help adapt the guideline recommendations to the local context in low- and middle-income countries to address their specific challenges, such as a different profile of ACHD, not understanding the unique needs of ACHD patients and severe resource constraints. The core goal is the same as everywhere: lifelong, quality care for ACHD patients.

Visit JACC’s ACHD Guideline Hub for the full document, as well its Guideline-at-a-Glance and other clinical resources to put the guideline into practice. Be sure to download ACC’s Guideline Clinical App which now includes the ACHD guideline. Also visit the CardioSmart CHD Hub for patient-focused resources.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Congenital Heart Disease

Keywords: Heart Defects, Congenital, Long-Term Care