Valvular heart disease in congenital heart disease: a narrative review

Abstract

Congenital heart defects are the most common type of birth defects, and with recent advances leading to improved survival, there are now over two million infants, children, and adults with congenital heart disease (CHD) in the United States alone (1-3). Many patients suffer from primary congenital valvular lesions or prosthetic/conduit valvular dysfunction. Given the substantial variability within each phenotype of CHD, this review will provide a basic background in embryology to allow the reader to create an adaptable construct in their approach to these patients (Table 1).As the prevalence of CHD grows, it is imperative that providers in all practice settings recognize pathology promptly, coordinate appropriate surveillance, and collaborate with CHD-specialists for optimal treatment. As such, this review provides details on the diagnosis, natural history, and contemporary therapeutic approaches to valvular heart disease in CHD. The information used to write this review was collected via computerized literature searches of electronic databases including U.S. National Library of Medicine (MEDLINE), ScienceDirect, and Google Scholar as well as hand searches of the references of the retrieved literature and discussions with experts in

Transcatheter Interventions for Atrioventricular Dysfunction in Patients with Adult Congenital Heart Disease: An International Case Series

“…Patients with congenital heart disease are surviving into adulthood due to improved surgical and interventional techniques [ 2 ]. With these patients surviving into older age, there is an increasing incidence of valvular dysfunction [ 3 ]. Our results are optimistic in showing that percutaneous valvular interventions are a feasible treatment option in this patient population—patients who are often at high surgical risk and who have had multiple prior cardiac interventions/surgeries.…”

“…The prevalence of patients with congenital heart disease surviving into adulthood is increasing due to medical and surgical advances [ 1 , 2 ]. A significant portion of these patients suffer from valvular dysfunction with ensuing morbidity and mortality [ 3 ]. Many of these patients have undergone multiple prior surgical interventions in childhood and have enhanced risk factors for structural valve deterioration when advancing into adulthood and older age.…”

The Adult Congenital Heart Disease Anatomic and Physiological Classification: Associations with Clinical Outcomes in Patients with Atrial Arrhythmias

“…Our results suggest that PhyS rather than AnatC might be a more important driver of adverse sequelae in ACHD with comorbid AA. Conventional wisdom could explain this observation: individual parameters assessed when scoring PhyS, such as heart failure, pulmonary hypertension, or valvular heart disease, possess an independent prognostic value in either ACHD or AA [ 24 , 25 , 26 , 27 ]. On the other hand, anatomic complexity does not convey any information on the patient’s hemodynamic, functional status, or comorbidities; a patient with atrial septal defect and severe pulmonary hypertension could still be classified as simple anatomic complexity.…”

The Adult Congenital Heart Disease Anatomic and Physiological Classification: Associations with Clinical Outcomes in Patients with Atrial Arrhythmias

“…Our results suggest that PhyS, rather than AnatC might be a more important driver of adverse sequelae in ACHD with comorbid AA. Conventional wisdom could explain this observation: individual parameters assessed when scoring PhyS, such as heart failure, pulmonary hypertension, or valvular heart disease, possess an independent prognostic value in either ACHD or AA [26][27][28][29]. On the other hand, anatomic complexity does not convey any information on the patient's hemodynamic, functional status or comorbidities; a patient with atrial septal defect and severe pulmonary hypertension could still be classified as simple anatomic complexity.…”