CASE PRESENTATION
A 29-year-old female with Kartagener syndrome and complete situs
inversus underwent a double lung transplant for end stage lung disease.
Her postoperative course was uneventful. Within 6 months of transplant,
she experienced an aspiration event leading to a persistent decline in
her lung function within the first year. She ultimately was hospitalized
requiring mechanical ventilation and a tracheostomy. The patient had
primarily hypercapnic respiratory failure with radiographic signs of
chronic
lung allograft dysfunction
(bronchitis obliterans syndrome). At the time of admission, the patient
ventilatory rate was 30 breaths/min and her arterial carbon dioxide
tension (PaCO2) was 80 mmHg. Her increased respiratory
work resulted in high calorie consumption with a significant impact on
her body mass index (16.5 to 14.4 in 6 months). To optimize her
nutritional status and muscle strength before re-do lung
transplantation, we decided to bridge her with extracorporeal membrane
oxygenation (ECMO). Initially, our intention was to use a dual-lumen
cannula to facilitate mobility but due to her anatomy we decided that an
Avalon or Crescent cannula was not suitable as the outflow jet will be
directed against the atrial wall. We decided to proceed with the HRAS
after emergency FDA authorization. A dual-lumen
15.5Fr catheter was placed via the
left internal jugular vein into the superior vena cava
(Figure 1A, 1B). After the
initiation of ECCO2R, the patient’s
PaCO2 improved by adjusting sweep between 7-9 L/min
resulting in a carbon dioxide (CO2) removal of 80-90
ml/min (Figure 2). We used
bivalirudin for anticoagulation and targeted goal was activated partial
thrombin time 45-60 s. Three days after placing the HRAS, she was listed
for re-do double lung transplantation. As she waited for a viable donor
offer, she was awake, able to get out of bed, stand up and actively
participate in rehabilitation program. In addition, she stopped losing
weight, her BMI plateau at 15 and her prealbumin at 20
mg/dl. Donor lungs became
available 8 days after the initiation support.
After
induction of general anesthesia, the HRAS was removed after confirming
that the patient was hemodynamically stable, and cardiopulmonary bypass
was introduced when the donor lungs arrived in the operating room.
She underwent an uneventful re-do
double lung transplant.
After
transplantation, the patient developed an increased need for oxygen,
potentially due to extensive volume and product resuscitation and
ischemic reperfusion injury. We decided to put her on venous-venous ECMO
(VV-ECMO). Her chest was left open due to coagulopathy for 3 days. We
decannulated VV-ECMO on postoperative day 7. She was discharged 43 days
after lung transplantation (Figure 1C).
DISCUSSION
Lung transplantation in patients with Kartagener’s syndrome and situs
inversus challenges include: dextrocardia with discrepancies between
donor and recipient vessels and airways 4-7. In
addition, the chest cavity mismatch between the donor right lung between
and the recipient’s dextrocardia can result in right lower lobe
atelectasis and kinks of the pulmonary artery. In the present report we
did not experience any anastomotic or postoperative challenges. We only
preserved donor pulmonary arteries to remain long enough for
anastomosis.
We
previously reported a case of using HRAS to bridge re-do double lung
transplant; transplant was performed 20 days after the induction of
HRAS2. Bonnin et al. reported using HRAS for severe
chronic obstructive pulmonary disease bridge to lung transplant;
transplant was performed 31 days after induction of
HRAS3. HRAS has also been reported to improve acidosis
in acute respiratory failure from coronavirus disease
20198.
Rajab et al. reported the use of VV-ECMO via a right internal jugular
Avalon cannula as a bridge to heart and lung transplantation in a
patient with dextrocardia9. In our patient this was
not an option because her superior and inferior vena cava were on the
left side and her tricuspid valve was facing right. We decided to
proceed with the Hemolung because our patient mainly required
CO2 removal to reduce ventilatory need, reduce work of
breathing and reduce calory consumption. In addition, the 15.5Fr was
easy to place inside the right atrium via the left internal jugular
vein.
This experience demonstrates that ECCO2R can be safely
and effectively used to bridge lung transplant patients with hypercapnic
respiratory failure in which anatomical challenges limit cannulation
options. To our knowledge this may
represent the first experience with the use of ECCO2R as
a bridge to lung re-transplantation in a patient with complete situs
inversus.