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\begin{document}

\title{Bridge to second double lung transplant with an extracorporeal carbon
dioxide removal system in situs inversus patient}



\author[1]{Masashi Furukawa}%
\author[1]{Ernest Chan}%
\author[1]{Pablo Sanchez}%
\affil[1]{University of Pittsburgh Department of Cardiothoracic Surgery}%


\vspace{-1em}



  
  \date{May 24, 2024}


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\begin{abstract}
We report a first case with the use of extracorporeal carbon dioxide
removal system as a bridge to re-do lung transplant in complete situs
inversus patient. A 29-year-old female with Kartagener syndrome and
complete situs inversus underwent a double lung transplant for end stage
lung disease. Within one year after transplant the patient had primarily
hypercapnic respiratory failure with radiographic signs of chronic lung
allograft dysfunction. To optimize her nutritional status and muscle
strength before re-do lung transplantation, we decided to bridge her
with an extracorporeal carbon dioxide removal system due to anatomical
difficulty. She was listed and underwent an uneventful re-do double lung
transplant with cardiopulmonary support.%
\end{abstract}%



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\textbf{INTRODUCTION}

The use of extracorporeal life support (ECLS) as a bridge to lung
transplantation has increased over the last decade, however its use in
re-do lung transplantation is limited due to poor
outcomes\textsuperscript{1,2}. Extracorporeal circulation with a single
duo-lumen cannula provides the advantage of more comfortable
mobilization particularly in patients in which we expect a longer bridge
to transplant. The Hemolung Respiratory Assisted System (HRAS) (ALung
Technologies, Pittsburgh PA) is a venous-venous extracorporeal carbon
dioxide removal (ECCO\textsubscript{2}R) system that allows for
effective removal of carbon dioxide via a small French double lumen
catheter\textsuperscript{2,3}.

In this report the decision to bridge our patient with an HRAS was based
on primary hypercapnic respiratory failure and anatomical limitations
due to her complete situs inversus.

\textbf{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 (PaCO\textsubscript{2}) 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
ECCO\textsubscript{2}R, the patient's PaCO\textsubscript{2} improved by
adjusting sweep between 7-9 L/min resulting in a carbon dioxide
(CO\textsubscript{2}) 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 \textsuperscript{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
HRAS\textsuperscript{2}. Bonnin et al. reported using HRAS for severe
chronic obstructive pulmonary disease bridge to lung transplant;
transplant was performed 31 days after induction of
HRAS\textsuperscript{3}. HRAS has also been reported to improve acidosis
in acute respiratory failure from coronavirus disease
2019\textsuperscript{8}.

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 dextrocardia\textsuperscript{9}. 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
CO\textsubscript{2} 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 ECCO\textsubscript{2}R 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 ECCO\textsubscript{2}R as a bridge to lung re-transplantation
in a patient with complete situs inversus.

\textbf{References}

1. Tipograf Y, Salna M, Minko E, et al. Outcomes of Extracorporeal
Membrane Oxygenation as a Bridge to Lung Transplantation. \emph{Ann
Thorac Surg.} 2019;107(5):1456-1463.

2. Bermudez CA, Zaldonis D, Fan MH, Pilewski JM, Crespo MM. Prolonged
Use of the Hemolung Respiratory Assist System as a Bridge to Redo Lung
Transplantation. \emph{Ann Thorac Surg.} 2015;100(6):2330-2333.

3. Bonin F, Sommerwerck U, Lund LW, Teschler H. Avoidance of intubation
during acute exacerbation of chronic obstructive pulmonary disease for a
lung transplant candidate using extracorporeal carbon dioxide removal
with the Hemolung. \emph{J Thorac Cardiovasc Surg.} 2013;145(5):e43-44.

4. Yamamoto H, Sugimoto S, Miyoshi K, Otani S, Yamane M, Toyooka S. Lung
transplantation for Kartagener syndrome: technical aspects and
morphological adaptation of the transplanted lungs. \emph{Gen Thorac
Cardiovasc Surg.} 2021;69(3):588-592.

5. Lucius Lee KF, Michael Hsin KY, Alva Sit KY, Cally Ho KL, Timmy Au
WK. Bilateral sequential lung transplantation in Kartagener
syndrome.\emph{JTCVS Tech.} 2020;3:406-408.

6. de Castro CCB, Dos Reis FP, de Carvalho GVS, et al. Technical
Challenges in Lung Transplantation of Kartagener Syndrome Recipients: A
Unique Team Experience With 12 Patients. \emph{Transplant
Proc.}2020;52(5):1384-1387.

7. Gauthier JM, Takahashi T, Bierhals AJ, et al. Technical
Considerations for Lung Transplantation in Kartagener's
Syndrome.\emph{Ann Thorac Surg.} 2019;107(5):e337-e339.

8. Akkanti B, Jagpal S, Darwish R, et al. Physiologic Improvement in
Respiratory Acidosis Using Extracorporeal Co2 Removal With Hemolung
Respiratory Assist System in the Management of Severe Respiratory
Failure From Coronavirus Disease 2019. \emph{Crit Care
Explor.}2021;3(3):e0372.

9. Rajab TK, Kim T, Keller S, Mallidi H. Management of a young patient
with dextrocardia, atrial septal defect, and Eisenmenger syndrome with
venous-venous extracorporeal membrane oxygenation and heart-lung
transplantation. \emph{J Card Surg.} 2019;34(10):1114-1116.

Figure 1, A: Hemolung, B: X-ray with Hemolung, C: X-ray at discharge\selectlanguage{english}
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Figure 2:

Top: CO2 removal and sweep gas flow during 8 days of Hemolung support.

Bottom: Patient's PaCO2 during 8 days of Hemolung support.\selectlanguage{english}
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