Skip to Main Content




A 29-year-old man was born a "blue baby" and found to have d-transposition of the great arteries (D-TGA). A balloon atrial septostomy (BAS) was performed as an infant, followed by the Mustard (atrial baffle) procedure. He did well for several years and did not require further surgical or percutaneous interventions. He was lost to follow-up for several years and ultimately presented to a local emergency room at 24 years of age, with atrial flutter and a rapid ventricular rate. He was electrically cardioverted back to sinus rhythm. At the time, an echocardiogram demonstrated mild systemic (right ventricular) dysfunction and concerns for narrowing of the superior vena cava (SVC) limb of the systemic venous baffle. Cardiac magnetic resonance imaging (MRI) subsequently demonstrated a mildly dilated and hypertrophied systemic right ventricle (RV) with mild global systolic dysfunction (estimated RV ejection fraction [EF] of 43%). The thin-walled left ventricle (LV) had normal size and function (LVEF of 63%). He was taken to the cardiac catheterization laboratory where SVC baffle limb stenosis was confirmed. A 36-mm max LD stent expanded to 34 mm was deployed to relieve the baffle stenosis. An electrophysiology (EP) study was also performed and revealed both atrial flutter and ventricular tachycardia. Given this, a transvenous implantable cardioverter-defibrillator (ICD) was placed for primary prevention of sudden cardiac death (SCD). He did well from 24 to 29 years of age with no major concerns and maintained regular follow-up with the Adult Congenital Heart Disease (ACHD) clinic. He maintained a regular job, got married, had two children, and exercised regularly. Closer to his 29th birthday, he noted that he was becoming "fatigued" with running 2 to 3 miles. He had rare episodes of palpitations associated with lightheadedness though he never had frank syncope or an ICD discharge. A Cardio-Pulmonary Exercise Test (CPET) was done to screen for desaturations and arrhythmias with exercise. This revealed a baseline oxygen saturation of 92% on room air with significant desaturation to 86% while running. There were no rhythm concerns. A repeat echocardiogram was done that showed a systemic venous baffle leak. He underwent cardiac catheterization with removal of the ICD leads, placement of a covered stent to relieve the baffle leak, and ICD lead replacement. Since this procedure, he has been doing well. He remains fully saturated, continues to work, and exercises often with no concerns.




  • This case highlights several important concepts for the long-term complications and management of adult patients who have had surgical palliation for D-TGA as an infant/child.

  • These include concerns for both atrial and ventricular arrhythmias including the need for ICD placement to prevent SCD.

  • These patients can also undergo both baffle leak and stenosis for which directed transcatheter interventions can be successfully performed in the cardiac catheterization laboratory.

  • Most importantly this case highlights the importance of long-term follow-up and management in patients with complex congenital heart disease like D-TGA, so they may continue to ...

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.


About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessCardiology Full Site: One-Year Subscription

Connect to the full suite of AccessCardiology content and resources including textbooks such as Hurst's the Heart and Cardiology Clinical Questions, a unique library of multimedia, including heart imaging, an integrated drug database, and more.

$595 USD
Buy Now

Pay Per View: Timed Access to all of AccessCardiology

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.