Updates in the management of congenital heart disease in adult patients

ABSTRACT

Introduction

Adults with congenital heart disease represent a highly diverse, ever-growing population. Optimal approaches to management of problems such as arrhythmia, sudden cardiac death, heart failure, transplant, application of advanced therapies and unrepaired shunt lesions are incompletely established. Efforts to strengthen our understanding of these complex clinical challenges and inform evidence-based practices are ongoing.

Areas Covered

This narrative review summarizes evidence underpinning current approaches to congenital heart disease management while highlighting areas requiring further investigation. A search of literature published in ‘Medline,’ ‘EMBASE,’ and ‘PubMed’ using search terms ‘congenital heart disease,’ ‘arrhythmia,’ ‘sudden cardiac death,’ ‘heart failure,’ ‘heart transplant,’ ‘advanced heart failure therapy,’ ‘ventricular assist device (VAD),’ ‘mechanical circulatory support (MSC),’ ‘intracardiac shunt’ and combinations thereof was undertaken.

Expert Opinion

Application of novel technologies in the diagnosis and management of arrhythmia has and will continue to improve outcomes in this population. Sudden death remains a prevalent problem with many persistent unknowns. Heart failure is a leading cause of morbidity and mortality. Improved access to specialist care, advanced therapies and cardiac transplant is needed. The emerging field of cardio-obstetrics will continue to define state-of-the-art care for the reproductive health of women with heart disease.

KEYWORDS:

• congenital heart disease
• congenital shunts
• fontan circulation
• heart failure
• systemic right ventricle

Article highlights

• The application of novel modalities to augment the success of ablation in patients with complex anatomy hold promise for application in the congenital heart disease population.

• Factors that determine and mitigate patients’ risk of sudden death are increasingly being identified, though a unified approach to risk stratification has yet to be established.

• The prognostic implications of systemic left ventricular dysfunction, and benefits of goal-directed medical therapy in patients with congenital heart disease are increasingly being appreciated.

• Patients with end-stage heart failure syndrome, progressive cyanosis or onset of reversible pulmonary arterial hypertension should be considered for advanced heart failure therapies.

• Mechanical circulatory support should be considered early on for congenital heart failure patients, prior to the progression to end-organ dysfunction.

• Appropriate assessment of the pulmonary vascular resistance is paramount in the management of patients with intracardiac shunts.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.