Author Affiliations: From the Department of Cardiology, Lahey Clinic Medical Center, Tufts University School of Medicine, Burlington, Massachusetts. Disclosure: Dr. Piemonte is a member of the speaker’s bureau for Boston Scientific Corporation. Manuscript submitted March 10, 2008, provisional acceptance given June 3, 2008, manuscript accepted June 11, 2008. Address for correspondence: Wael F. Al-Husami, MD, Department of Cardiology, Lahey Clinic Medical Center, Tufts University School of Medicine, 41 Mall Road, Burlington, MA 01805. E-mail: email@example.com
ABSTRACT: Children with congenital heart disease represent 1% of live births, and it is estimated that millions will reach adulthood. Advances in cardiac surgery for the treatment of pediatric congenital heart disease have allowed many of these patients to enjoy a long life. Adults with a history of repaired congenital heart disease present different and unique challenges to the adult interventional cardiologist. This is a case report of a patient who presented with pseudoaneurysm of a repaired coarctation of the aorta, illustrating the need for the interventional cardiologist to be innovative in using the many occluding devices available in the laboratory. Our case demonstrates the technical feasibility of occluding the pseudoaneurysm tube graft used for the correction of the coarctation of the aorta. This procedure can be performed safely and successfully in expert hands.
J INVASIVE CARDIOL 2008;20:E293–E295
Children with congenital heart disease represent 1% of live births, and it is estimated that millions will reach adulthood. Advances in cardiac surgery for the treatment of pediatric congenital heart disease have allowed many of these patients to enjoy a long life. Adults with history of repaired congenital heart disease present different and unique challenges to the adult interventional cardiologist.
This is a case report of a patient who presented with pseudoaneurysm of a repaired coarctation of the aorta, illustrating the need for the interventional cardiologist to be innovative in using the many occluding devices available in the laboratory. Our case demonstrates the technical feasibility of occluding the pseudoaneurysm tube graft used for the correction of the coarctation of the aorta. This procedure can be performed safely and successfully in expert hands.
Case Report. A 41 year-old female with a history of congenital coarctation of the aorta had undergone surgical repair at the age of 19. She developed recurrent coarctation requiring a tube graft between the left subclavian artery and the descending aorta below the level of the coarctation at the age of 30. The patient remained asymptomatic until the current presentation when she experienced squeezing chest discomfort while at rest. The initial assessment with transthoracic echocardiography revealed normal left ventricular function, no regional wall motion abnormalities and a normal ascending aorta. The myocardial perfusion scan was normal. A 3-dimensional multiplanar chest computed tomographic (CT) scan revealed a small pseudoaneurysm at the anastomosis between the left subclavian artery and the descending aortic bypass graft. Cardiothoracic surgery was consulted and conservative management was recommended. A follow-up visit and chest CT angiographic (CTA) scan were scheduled for 6 months later.
Despite the recommendation of the cardiothoracic surgeon, the patient missed her follow-up visit. Over the course of several months, she developed bilateral leg weakness with moderate walking, which rapidly disappeared with rest and was very reminiscent of her symptoms prior to the initial surgical repair of the coarctation. Physical examination showed equal blood pressure in both arms. A chest CTA scan demonstrated enlargement of the pseudoaneurysm to 2.5 cm. There was also an aortic narrowing at the site of her previous coarctation. To further study the anatomical and hemodynamic changes, the patient underwent arch aortography. A 5 Fr pigtail catheter was advanced easily across the area of coarctation to the ascending aorta (Figure 1B). Ascending aortography revealed evidence of a mild coarctation immediately beyond the takeoff of the left subclavian artery, with evidence of a patent graft from the mid-subclavian artery to the descending aorta beyond the coarctation. There was a pseudoaneurysm at the site of the proximal anastomosis (Figure 1A). Note the post-stenotic aortic dilatation below line b (Figure 1). No collateral circulation was observed. Transcoarctation gradients were obtained with a balloon occluding the flow within the left subclavian artery. There was no gradient across the coarctation. Surgical pseudoaneurysm repair was thought to be high-risk due the prior two surgical operations and the likelihood of excessive scar tissue formation. The patient was referred to the cardiac catheterization laboratory for potential endovascular repair. Informed consent was obtained from the patient.
Ascending aortography and selective left subclavian angiography were performed to characterize the pseudoaneurysm and its relation to the adjacent vessels. Multiple angiographic views were obtained. However, we were not able to identify a discrete pseudoaneurysm neck that could be coiled or safely occluded with an Amplatzer device. It was clear that neither approach would be effective.
Therefore, a 12 x 30 mm WallGraft stent (Boston Scientific Corp., Natick, Massachusetts) was advanced to the proximal left subclavian artery occluding the flow into the bypass graft (Figure 2). Repeat ascending aortography with runoff was performed revealing residual antegrade flow (Figure 3). We electively staged the procedure to avoid excessive contrast exposure. One month later, the patient was brought back to the cardiac catheterization laboratory for exclusion of the tube graft and repair of the endoleak. The previously-detected Type I endoleak was repaired using a Gianturco-Grifka vascular coil that passed through an 8 Fr Mullins sheath. During attempted detachment, the bag of coils dislodged and was precariously perched on top of the Mullins sheath. A 15 mm snare was advanced to the descending aorta from the contralateral femoral artery and successfully snared the coils.
Another 15 mm snare was advanced into the left subclavian artery through the endoleak and down to the distal anastomosis of the bypass graft, and the vascular device was then re-snared (Figure 4). The devices were then pulled back to the proximal end of the bypass graft, and the distal snare was released. A 16 mm vascular plug was delivered through a Mullins sheath into the distal end of the bypass graft to prevent embolization of the Gianturco-Grifka vascular device and to occlude the tube graft. Repeat ascending aortography revealed near-total occlusion of flow in the vascular bypass graft (Figure 5). The residual coarctation was found to be hemodynamically insignificant, with a minimal gradient noted after closure of the graft. The patient tolerated the procedure well, with no complications. Two years later, the patient underwent chest CTA, which demonstrated a thrombosed pseudoaneurysm that had decreased in size, as well as a thrombosed bypass graft.
Discussion. In 1945, the first successful surgical repair of a coarctation of the aorta was performed.1 During that early operative era, recurrence rates varied from 20–86%.2–4 In response to that problem, prosthetic patch aortoplasty was introduced in 1957 by Vossschulte.5 Pseudoaneurysms at the anastomotic site, however, have been reported as complications after prosthetic patch aortoplasty repair. The rate of late aneurysm formation after Dacron patch aortoplasty range from 5–38%.6 The etiology of pseudoaneurysm formation after patch aortoplasty could be due to a change in hemodynamics resulting from the different tensile strengths of a Dacron patch and the posterior aortic wall, with the pulsatile waveform being directed to the posterior aortic wall by the inflexible anterior patch.7,8 Knyshov et al reviewed 48 patients who had aortic aneurysms at the site of the repair of coarctation of the aorta and found that the outcome of conservative treatment remains unpredictable and was associated with a 100% rate of rupture within 15 years in this single-center experience.9 On the other hand, repeat surgery is associated with a 14% in-hospital mortality rate due to peripheral or central nervous system injury or bleeding.10 The aortic dilatation noticed in Figure 1 requires continued follow up. We recommend an annual chest CT scan with contrast to ensure that no significant changes have occurred.
In recent years, minimally invasive percutaneous endovascular repair of anastomotic pseudoaneurysms has become acceptable as a safe and effective alternative to surgery or observation. These published case reports or small case series have indicated promising results and insignificant complications. Bell and colleagues treated 3 patients after previous patch aortoplasty, and 1 patient after left subclavian aortoplasty with the CORE TAG Thoracic Endoprosthesis.11 The procedures were technically successful in all cases without any mortality. During follow up (3–29 months), all aneurysms remained excluded without evidence of endoleaks or stent migration.6,11 In our case, we were able to demonstrate successful endovascular closure of the previously-placed tube graft with depressurization and isolation of pseudoaneurysms at the site of the anastomosis. Minimally invasive percutaneous endovascular repair of pseudoaneurysms can present a safer alternative to repeated aortic surgery.