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 ...
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