159. ACHD: Coarctation of the Aorta with Dr. Ari Cedars

CardioNerds (Amit Goyal and Daniel Ambinder),  ACHD series co-chair Dr. Agnes Koczo (UPMC), and episode FIT lead, Dr. Natasha Wolfe (Washington University) join Dr. Ari Cedars   (Director of the Adult Congenital Heart Disease Program at Johns Hopkins) for a discussion about coarctation of the aorta.  

In this episode we discuss the presentation and management of unrepaired and repaired coarctation of the aorta in adults. We discuss the unique underlying congenital anatomy of coarctation and how that impacts physiology, clinical presentation, and diagnostic findings. We discuss the importance of long-term routine follow-up and screening of patients (including those who have been “repaired”) for common complications such as hypertension, re-coarctation, and aneurysm development. We end with a discussion of treatment options for coarctation and its complications.

Audio editing by CardioNerds Academy Intern, Dr. Maryam Barkhordarian.

The CardioNerds Adult Congenital Heart Disease (ACHD) series provides a comprehensive curriculum to dive deep into the labyrinthine world of congenital heart disease with the aim of empowering every CardioNerd to help improve the lives of people living with congenital heart disease. This series is multi-institutional collaborative project made possible by contributions of stellar fellow leads and expert faculty from several programs, led by series co-chairs, Dr. Josh Saef, Dr. Agnes Koczo, and Dr. Dan Clark.

The CardioNerds Adult Congenital Heart Disease Series is developed in collaboration with the Adult Congenital Heart Association, The CHiP Network, and Heart University. See more

Claim free CME for enjoying this episode! Disclosures: None

Pearls • Notes • References • Guest Profiles • Production Team

CardioNerds Adult Congenital Heart Disease PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll

CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron!

Pearls

Coarctation of the aorta can occur as a discrete stenosis or as a long and hypoplastic hypoplastic aortic arch segment. Most commonly it is a discrete stenosis located at the insertion site of the ductus arteriosus just distal to the left subclavian artery.Three quarters of patients with coarctation of the aorta also have a bicuspid aortic valve.Hypertension is the most common long-term complication of coarctation of the aorta, whether repaired or unrepaired. Unrepaired coarctation is a rare cause of secondary hypertension in young adults with a difference in upper extremity and lower extremity BP by ≥ 20 mmHg. Systemic hypertension may not be consistently identifiable at rest in those with repaired coarctation, thus guidelines recommend ambulatory blood pressure monitoring or stress testing to identify hypertension with exertion.Chest and brain imaging via CT or MRI should be done every 5-10 years to screen for other long-term complications including re-coarctation (rate ~11%), aortic aneurysm development (higher risk in those with concurrent bicuspid aortic valve), pseudoaneurysm, aortic dissection, and cerebral aneurysms.Repair of coarctation or re-coarctation is indicated for patients who are hypertensive with a BP gradient ≥ 20 mmHg (Class I recommendation). Catheter-based stenting is the preferred approach when technically feasible.

Show notes

1. What is the proposed embryologic origin of coarctation of the aorta?

The aortic arch and its branches develop at 6-8 weeks fetal gestation. We all start with six aortic arches that go on to become the great arteries of the head and neck. The 4th arch forms the thoracic aortic arch and isthmus. The 6th arch persists as the proximal pulmonary arteries and ductus arteriosus. Thoracic aortic coarctation is therefore a manifestation of abnormal embryologic development of the 4th and 6th arches.There are two main theories regarding how aortic coarctation occurs.