Session Explores Primary Causes of Sudden Cardiac Death

Although relatively infrequent, the two primary causes of sudden cardiac death—hypertrophic cardiomyopathy (HCM) and anomalous aortic origin of a coronary artery (AAOCA)—can be difficult to manage. Surgeons often have difficulty mastering reproducible techniques that maximize benefits and minimize risk.

Adult Congenital Heart Disease Symposium: Surgical Management of Hypertrophic Cardiomyopathy and Anomalous Aortic Origin of a Coronary Artery in Children and Adults

Sunday, January 28
8:00 a.m. – 12:00 p.m.
Floridian Ballroom B-C

The Adult Congenital Heart Disease Symposium will focus on these two challenging conditions through invited lectures, a panel discussion, and questions from attendees.

“Our goal is for the Symposium to be of interest to a broad audience,” said Stephanie M. Fuller, MD, of Children’s Hospital of Philadelphia, who is one of the Symposium moderators. “We tried to select diseases that cover the life span. These conditions are appealing to both adult cardiac surgeons and congenital heart surgeons.”


Surgical myectomy is the criterion standard for most patients with obstructive HCM and drug-refractory symptoms. “A question for many surgeons is how to do an adequate myectomy to relieve obstruction, especially when left ventricular outflow tract obstruction is present,” said James S. Tweddell, MD, of Cincinnati Children’s Hospital, who is another Symposium moderator.

Although it’s rare, Dr. Tweddell said an individual with HCM may have a resting diastolic defect with no obstruction. “For these patients, we need to determine the role of cavity enlargement,” he said.

Experts on myectomy will describe different surgical techniques, including basal septal myectomy, the transventricular approach for midventricular obstruction, and cavity enlargement using an apical approach for nonobstructive HCM.

Added to the challenges of surgery is identifying individuals who are high risk for conventional surgery. “Alcohol ablation is an option for high-risk patients, but the outcomes are less predictable. Surgeons must understand risk stratification and appropriate referral for ablation,” explained Dr. Tweddell.


AAOCA is increasingly being recognized, primarily because of better detection by echocardiography. But the optimal management of individuals with AAOCA is controversial, leaving many surgeons uncertain of the best way to manage the condition.

“If the patient is asymptomatic, what do you do? AAOCA often occurs in teenagers, a particularly challenging group of patients because of the high risk of sudden cardiac death, which is so tragic,” said Dr. Tweddell. “Do you let teenagers with this condition play sports? How do you identify the condition in advance, and what is the best management strategy?”

Experts will try to answer these questions by offering information on imaging and nomenclature, different management approaches for adults and children, surgical unroofing, the current role of bypass grafting, and when it’s best to operate on the anomalous right coronary artery.