P191. Like Father, Like Daughter: Open Surgical Management of Thoracoabdominal Aneurysms in a Father and Daughter with Loeys-Dietz Syndrome
Presented During: POD Abstracts (Available for viewing in the exhibit hall for the duration of the meeting)
Thomas Jefferson University Hospital
philadelphia, PA
United States - Contact Me
Joshua Chen is a 3rd year medical student at Sidney Kimmel Medical College interested in a career in academic cardiothoracic surgery.
Thursday, April 25, 2024: 5:38 PM - 7:00 PM
Sheraton Times Square
Room: Central Park
Description
Objective: We report the successful open surgical management of thoracoabdominal aneurysms (TAAAs) in a father and daughter with Loeys-Dietz Syndrome after failed endovascular repair.
Methods:
Patient 1:
A 36-year-old woman with Loeys-Dietz syndrome and a past history of postpartum type A aortic dissection requiring stage 1 elephant trunk and chronic type B aortic dissection with left carotid to subclavian bypass and subsequent TEVAR all performed at an outside hospital presented with chest pain. The CT scan demonstrated type 1B endoleak and a 9 cm Crawford extent II TAAA (Figure 1).
The patient was taken for open repair. After femoral-femoral cannulation, cardiopulmonary bypass (CPB) was initiated. Under deep hypothermic circulatory arrest (DHCA), the aorta was transected and the stent was explanted. A 26-mm graft was anastomosed proximally to the previous elephant trunk graft and distally to the infrarenal aorta. The renovisceral vessels were reattached using a trifurcated graft.
The patient was extubated after 36 hours. After surgery, the patient developed renal failure requiring temporary dialysis. She was discharged home on POD #16.
Patient 2:
A 62-year-old man with Loeys-Dietz syndrome, who incidentally was the father of Patient 1, presented with back pain. The patient had a prior CABG and AVR and hybrid aortic arch repair with TEVAR performed at an outside hospital. The CT scan demonstrated a type 1B endoleak and a 6.9 cm Crawford extent III TAAA (Figure 1).
After femoral-femoral cannulation, CPB was initiated. Under DHCA, the aorta was transected at the level of the distal stent and a 30-mm graft was anastomosed proximally at the level of the distal stent and then distally at the level of the aortoiliac bifurcation. The renovisceral vessels were selectively perfused and then reattached.
Postoperatively, the patient required a sigmoid colon resection for bowel ischemia. He also developed cholecystitis requiring a percutaneous cholecystostomy drain, renal failure requiring temporary dialysis, and tracheostomy. He was discharged to a rehabilitation facility on POD #57.
Results:
Both cases demonstrate the successful open surgical management of TAAAs in a father and daughter with Loeys-Dietz syndrome after failed endovascular repair.
Conclusions: Young patients with Loeys-Dietz syndrome should undergo open surgical repair of TAAAs to avoid stent-related complications. Early recognition and meticulous surveillance in families with Loeys-Dietz syndrome are absolutely essential.
Authors
joshua chen (1), Vishal Shah (1), Colin King (1), Jacqueline McGee (1), Megary McCoy (1), Jeffrey Zucker (1), Konstadinos Plestis (1)
Institutions
(1) Thomas Jefferson University Hospital, Philadelphia, PA
Methods:
Patient 1:
A 36-year-old woman with Loeys-Dietz syndrome and a past history of postpartum type A aortic dissection requiring stage 1 elephant trunk and chronic type B aortic dissection with left carotid to subclavian bypass and subsequent TEVAR all performed at an outside hospital presented with chest pain. The CT scan demonstrated type 1B endoleak and a 9 cm Crawford extent II TAAA (Figure 1).
The patient was taken for open repair. After femoral-femoral cannulation, cardiopulmonary bypass (CPB) was initiated. Under deep hypothermic circulatory arrest (DHCA), the aorta was transected and the stent was explanted. A 26-mm graft was anastomosed proximally to the previous elephant trunk graft and distally to the infrarenal aorta. The renovisceral vessels were reattached using a trifurcated graft.
The patient was extubated after 36 hours. After surgery, the patient developed renal failure requiring temporary dialysis. She was discharged home on POD #16.
Patient 2:
A 62-year-old man with Loeys-Dietz syndrome, who incidentally was the father of Patient 1, presented with back pain. The patient had a prior CABG and AVR and hybrid aortic arch repair with TEVAR performed at an outside hospital. The CT scan demonstrated a type 1B endoleak and a 6.9 cm Crawford extent III TAAA (Figure 1).
After femoral-femoral cannulation, CPB was initiated. Under DHCA, the aorta was transected at the level of the distal stent and a 30-mm graft was anastomosed proximally at the level of the distal stent and then distally at the level of the aortoiliac bifurcation. The renovisceral vessels were selectively perfused and then reattached.
Postoperatively, the patient required a sigmoid colon resection for bowel ischemia. He also developed cholecystitis requiring a percutaneous cholecystostomy drain, renal failure requiring temporary dialysis, and tracheostomy. He was discharged to a rehabilitation facility on POD #57.
Results:
Both cases demonstrate the successful open surgical management of TAAAs in a father and daughter with Loeys-Dietz syndrome after failed endovascular repair.
Conclusions: Young patients with Loeys-Dietz syndrome should undergo open surgical repair of TAAAs to avoid stent-related complications. Early recognition and meticulous surveillance in families with Loeys-Dietz syndrome are absolutely essential.
Authors
joshua chen (1), Vishal Shah (1), Colin King (1), Jacqueline McGee (1), Megary McCoy (1), Jeffrey Zucker (1), Konstadinos Plestis (1)
Institutions
(1) Thomas Jefferson University Hospital, Philadelphia, PA
Presentation Duration
PODS will be on display in the exhibit hall for the duration of the meeting during exhibit hall hours. PODS will also be available for viewing on the meeting website. There is no formal presentation associated with your POD, but we encourage you to visit the PODS area during breaks to connect with those viewing.