W. Aaron Kay

Permanent nonselective His bundle pacing in an adult with L-transposition of the great arteries and complete AV block

We report the placement of a permanent transvenous nonselective His bundle pacing lead in conjunction with a transvenous pacemaker/implantable cardioverter‐defibrillator in an adult with Levo‐Transposition of the Great Arteries (L‐TGA) and a stenotic coronary sinus (CS) ostium, which would not accommodate a transvenous left ventricular (LV) pacing lead. Nonselective His bundle pacing provided a nearly identical ventricular activation pattern in this previously unpaced patient. Many L‐TGA patients will have an eventual need for permanent pacing and, given the challenges of CS cannulation, His bundle pacing may represent a preferred modality rather than pure morphologic LV pacing or surgical systemic ventricular lead placement to achieve optimal electrical synchrony.

Erratum to: At the Heart of the Pregnancy: What Prenatal and Cardiovascular Genetic Counselors Need to Know about Maternal Heart Disease

1 Department of Internal Medicine, The Ohio State University, Columbus, OH, USA 2 Human Genetics Division, The Ohio State University, 306 BRT, 460 W. 12th Ave, Columbus, OH 43210, USA 3 Department of Human Genetics, University of Michigan, Ann Arbor, MI, USA 4 Allegheny General Hospital, Pittsburgh, PA, USA 5 Institute of Genetic Medicine, Johns Hopkins University, Baltimore, MD, USA 6 Division of Cardiology, Johns Hopkins University, Baltimore, MD, USA 7 Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA

Molecular and Genetic Insights into Thoracic Aortic Dilation in Conotruncal Heart Defects

Thoracic aortic dilation (AD) has commonly been described in conotruncal defects (CTDs), such as tetralogy of Fallot, double outlet right ventricle and transposition of the great arteries, and truncus arteriosus. Several theories for this have been devised, but fairly recent data indicate that there is likely an underlying histologic abnormality, similar to that seen in Marfan and other connective tissue disease. The majority of aortic dissection in the general population occurs after the age of 45 years, and there have been very few case reports of aortic dissection in CTD. Given advances in cardiac surgery and increasing survival over the past several decades, there has been rising concern that, as patients who have survived surgical correction of these defects age, there may be increased morbidity and mortality due to aortic dissection and aortic regurgitation. This review discusses the most recent developments in research into AD in CTD, including associated genetic mutations.

Long Term Consequences of the Fontan Procedure and How to Manage Them

In 1971, Fontan and Baudet described a surgical technique for successful palliation of patients with tricuspid atresia. Subsequently, this technique has been applied to treat most forms of functional single ventricles and has become the current standard of care for long-term palliation of all patients with single ventricle congenital heart disease. Since 1971, the Fontan procedure has undergone several variations. These patients require lifelong management including a thorough knowledge of their anatomic substrate, hemodynamic status, management of rhythm and ventricular function along with multi organ evaluation. As these patients enter middle age, there is increasing awareness regarding the long-term complications and mortality. This review highlights the long-term outcomes of the Fontan procedure and management of late sequelae.