Case of the Week

16yM Marfan Patient - Extent II Open Thoracoabdominal Aortic Aneurysm Repair with CPB and DHCA

CVSA Practitioner: Haley Barrett, DNP, CNOR, CRNFA

Role: First Assistant

Team Composition:

  • Attending Cardiothoracic Surgeon

  • Attending Vascular Surgeon

  • CT Anesthesiologist

  • CT Anesthesiology Fellow

  • CT First Assistant (Dr. Haley)

  • CT Scrub Nurse

  • Vascular Scrub Nurse

  • Perfusionist w/ Assistant

  • 2 Circulating Nurses

  • 2 Scrub Techs

  • Intraoperative Neurophysiologist

Patient Background:

A 16-year-old male patient with history of Marfan Syndrome (MFS) and left pneumothorax underwent a mechanical Bentall procedure for acute aortic dissection. Postoperative echocardiography demonstrated reduced LV function (LVEF 49%) and computed tomography showed patent false lumen. One year later, he developed dyspnea, LVEF was down to 18%, and the diagnosis of dilated cardiomyopathy was made. Four months later, LVEF became <10%. Notably, the diameter of the distal arch increased to 50 mm and that of the supraceliac aorta increased to 62 mm (6.2 cm). Furthermore, he had a recurrent left pneumothorax with a bulla. Although he was seemingly a transplant candidate, the regulation required us to first repair these aortic lesions to enable listing for transplantation. Additionally, even insurance reimbursement for continuous-flow LV assist device (cf-LVAD) is contingent upon listing for transplant. Therefore, to enable transplant and following long-term survival, he was transferred to us for extensive aortic surgery, which comprised an open extent II Thoracoabdominal Aortic Aneurysm repair with significant LV dysfunction. Neurological status was monitored using Motor Evoked Potentials (MEPs) and EEG.

Operative Treatment:

The aorta was exposed via a Stony incision and bypass was established by iliac artery perfusion and right atrium (via femoral vein)/pulmonary artery drainage. We placed a LV apical vent and started cooling. At 18°C, we administered potassium chloride into the pump reservoir and put him on Deep Hypothermic Circulatory Arrest (DHCA) - the head was wrapped in ice. During DHCA, the distal arch was opened, an occlusion balloon was placed in the ascending aorta, and crystalloid cardioplegia was administered. Next, proximal anastomosis was performed under retrograde cerebral circulation, we started side-branch perfusion and rewarming, and iliac perfusion was discontinued. While rewarming, intercostal artery reconstruction was performed, followed by visceral vessel reconstruction. Distal anastomosis was made at the terminal aorta. Weaning was smooth under intra-aortic balloon pump (IABP) support. Finally, thoracic surgeons conducted a bullectomy. On postoperative day (POD) 2, IABP was removed after extubation. Landiolol was started intraoperatively to control heart rate, which was eventually switched to carvedilol. Upon closing, he suddenly developed a severe hypotension and went asystolic. Patient was immediately re-opened and required 18 min of open cardiac massage before ROSC was achieved. Closing was subsequently carried out without incident.

  • Operative Time: 628 min

  • Total Bypass Time: 424 min

  • DHCA Time: 92 min

  • Asystole (PEA) Time: 18 min

Post-Operative Course:

In the post-operative period, the patient experienced severe agitation, personality change, short-term memory lapse (unable to recognize certain family members), left-sided limb weakness and disorientation. Patient was taken for CT scan of the brain. The CT scan demonstrated clear corticomedullary junction of the right hemisphere of the cerebrum. The patient was discharged to a neurological rehabilitation center on post-op day 12 and remains there with no improvement or regain of pre-op neurological function.

Notes and Discussion:

We did not use a superior vena cava cannula for retrograde cerebral circulation when we began deep hypothermic circulatory arrest (DHCA) through the left thoracotomy. Under DHCA, we clamped the descending aorta and maintain lower body perfusion from femoral arterial cannula. Generally, oxygen saturation of venous-return blood in the right atrium is quite high (mostly 100%). Then, we put the patient in the Trendelenburg position and kept central venous pressure at 20 to 25 mm Hg. By these maneuvers, oxygenated venous blood retrogradely circulated the brain, and de-oxygenated his blood, which eventually came out from the aortic arch vessels while under DHCA.

Operative Photos