Case Report
A 50-year-old-male with cystic fibrosis and multidrug resistant
pseudomonas, Achromobacter, methicillin resistant Staph aureus, and
recurrent sinusitis was referred for a double lung transplant and
concomitant liver transplant. He was declined for a liver transplant due
to congenital absence of the IVC. We decided to proceed with double lung
transplant only since the synthetic function of the liver was preserved.
Other comorbidities included diabetes, compensated cirrhosis, and HIV.
He required 4L of O2 at rest and 5L with exertion, had a
mean PA pressure of 21 mmHg, an ejection fraction of 65%, and no
significant coronary artery disease. MRI and CT showed an absence of
normal infrahepatic IVC with enlarged lumbar retro peritoneal
collaterals continuing into the azygous. There was normal portal venous
flow and hepatic venous flow into the suprahepatic IVC (figure
1). A suitable donor became available. We thought that it would not
have been prudent to initiate emergent CPB or ECMO intraoperatively
given his anatomy; therefore, the decision was made to insert the Protek
duo prior to the transplantation. For right ventricular and oxygenation
support, a 31-Fr Protek Duo cannula was inserted percutaneously into the
right internal jugular vein using fluoroscopy and transesophageal
echocardiography. The cannula was connected to a modified
cardiopulmonary bypass (CPB) machine, in an Oxy-RVAD configuration. The
right ventricle (RV) remained decompressed and the patient was well
oxygenated throughout the operation. Bilateral sequential lung
transplantation required extensive adhesiolysis. The patient had
previously undergone several chest tube placements bilaterally and
doxycycline pleurodesis due to pneumothoraces. The lungs were adherent
to the chest well, diaphragm, and pericardium. Right and left lung total
ischemic times were 387 and 488 minutes. The Protek duo was left in
place for perioperative oxygenation support. The patient remained on
Oxy-RAVD support after completion of the case due to a hypokinetic RV
and large amount of blood product transfusions. Postoperatively, the CPB
machine was converted to a CentriMag System with an inline Quadrox
oxygenator. Due to the extensive adhesiolysis the chest was left open
and packed. After 48 hrs the patient returned to the OR for chest
washout, closure and tracheostomy (figure 2). The patient was
decannulated bedside from support on postoperative day 11. Initially the
patient was doing very well, however within 3 weeks post lung transplant
he developed recurrent Achromobacter sepsis, renal failure requiring
dialysis, and shock leading to multisystem organ failure. Due to
multiple positive bacterial cultures during broad spectrum antibiotic
coverage, the patient received phage therapy. However, after careful
consideration with the patient, family, palliative care, and medical
ethics, the patient was transitioned to comfort measures only at his
request. He expired 7 weeks after transplantation.