Three-dimensional rendering before and after reconstruction.
Central Message.
LDS is a rare connective tissue disorder with aggressive aortic disease involvement. We present frozen elephant trunk treatment of a transverse arch aneurysm in a pediatric patient with LDS.
We detail a patient diagnosed with Loeys-Dietz syndrome (LDS) diagnosed as a neonate who has undergone 4 major aortic interventions over 15 years. He initially underwent resection of a massive ductus arteriosus at age 10 days and subsequently had a valve-sparing root replacement (custom 20-mm Valsalva graft) and hemiarch replacement for a severely dilated aortic root at age 11 months. He went on to have total arch replacement at age 12 years after progressive dilation of his residual ascending aorta to 4 cm with a z score of 6, which we discuss here.
Surgical Details
A left common carotid artery (CCA) to left subclavian artery (SCA) transposition was performed through a longitudinal neck incision extending to the upper aspect of the sternotomy. The right CCA was then exposed through a right neck incision and a 6-mm polytetrafluoroethylene graft was sewn end-to-side to the right CCA for later arterial perfusion. Redo sternotomy was carried out carefully given the massively dilated aorta. Cardiopulmonary bypass was commenced by arterial cannulation of the polytetrafluoroethylene graft in the right CCA and bicaval venous cannulation. Cooling was commenced to 26 °C. In the meantime, a right atriotomy was fashioned to close a patent foramen ovale and directly cannulate the coronary sinus for retrograde cardioplegia to arrest the heart. Circulatory arrest was initiated with antegrade cerebral perfusion through the right CCA graft, the aneurysmal aorta was opened, and the remainder of cardioplegia was given directly antegrade. The aneurysmal aorta, aortic arch, and arch vessels were dissected to the takeoff of the left SCA resecting the prior hemiarch graft down to the previous root-graft anastomosis. A 20-mm branched soft graft (4-Plex; Vascutek, Inchinnan, Renfrewshire, United Kingdom) was fashioned with a 21 × 10 mm stent graft (Gore TAG) to create the frozen elephant trunk graft as previously described.1 With a proximal landing zone between the origin of the left CCA and left SCA, the stent graft was deployed and subsequently the distal anastomosis was fashioned with a single running Prolene suture, incorporating a felt strip, the aorta, the stented graft, and the soft branched graft. Once completed, circulation was re-established with distal reperfusion through a side arm of the branched graft and cerebral perfusion continued through the right CCA graft. The left CCA was then anastomosed end-to-end to a branch of the soft graft to re-establish bilateral cerebral perfusion. The proximal soft graft was then anastomosed end-to-end to the original root graft. Tricuspid ring valvuloplasty was performed (30-mm AnnuloFlex ring; Carbomedics) with the heart still arrested. While rewarming, the innominate artery anastomosis was created end-to-end to the remaining limb of the branched graft. After de-airing and rewarming, the patient was successfully weaned from the bypass circuit.
Discussion
LDS is a recently described genetic syndrome characterized by triad of arterial tortuosity and aneurysms, hypertelorism, and bifid uvula or cleft palate that results from mutations in receptors for the cytokine transforming growth factor-β.2 With growing experience, it has been recognized as an aggressive connective tissue disorder with increased risk of aortic dissection and aneurysm rupture at an early age and smaller aortic diameters.3,4 Understanding of the natural history of this disease continues to evolve. Total aortic replacement is necessary in about one-fifth of patients with LDS who require intervention.4 Valve-sparing root replacement has gained traction in this population,3 but management of arch disease is still evolving. In this case, the patient had a valve-sparing root and ascending/hemiarch replacement at a very young age (11 months). This repair proved durable, lasting through age 12 years while the remaining native transverse arch continued to degenerate. In planning for this operation, ACP was employed through the carotid artery to facilitate adequate time with circulatory arrest and to secure a site for arterial cannulation in the event of injury during redo sternotomy. The use of stented grafts in the pediatric population certainly has its drawbacks given the rapidly expanding nature of aortic dilation and concern for later failure.5 To get the most proximal landing zone possible and minimize stent extension into the arch, the left carotid-subclavian transposition was performed through the left neck. However, this case illustrates how aggressive the disease can be. Patients can present as early as age 3 months,3 so the treatment paradigm needs a more well-rounded approach that incorporates cardiovascular surgeons who treat children as well as adults. The need for thoracoabdominal repair is not well defined but of patients with LDS requiring surgery, between 4% and 12% require thoracoabdominal repair.4,5 This patient went on to require extent 3 thoracoabdominal repair 3 years later after presenting with a type B dissection beyond his repair. In this case, having the hybrid repair facilitated the subsequent thoracoabdominal approach.
Webcast
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Footnotes
Disclosures: The authors reported no conflicts of interest.
The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.
Supplementary Data
This video outlines the surgical details of the case presented. Video available at: https://www.jtcvs.org/article/S2666-2507(22)00465-5/fulltext.
References
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Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
This video outlines the surgical details of the case presented. Video available at: https://www.jtcvs.org/article/S2666-2507(22)00465-5/fulltext.