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\begin{document}

\title{Heart-Lung Transplant in Congenitally Corrected Transposition of the
Great Arteries and Dextrocardia Patient}



\author[1]{Magdy El-Sayed Ahmed}%
\author[1]{Mathew Thomas}%
\author[1]{Ian  Makey}%
\author[1]{Archer Martin}%
\author[1]{David  Erasmus }%
\author[1]{Basar Sareyyupoglu}%
\author[1]{Kevin Landolfo}%
\author[1]{Si  Pham}%
\affil[1]{Mayo Clinic Florida}%


\vspace{-1em}



  
  \date{May 26, 2024}


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\selectlanguage{english}
\begin{abstract}
Background and aim of the study: Congenitally corrected transposition of
the great arteries (cc-TGA) is a rare type of congenital heart disease
and heart-lung transplant (HLTx) is the potential solution if the
patient developed severe biventricular failure.. Aim of the study is to
present a challenging case of cc-TGA with dextrocardia managed by HLTx.
Methods: A 53-year-old male patient was presented to our institution
with biventricular failure. The echocardiogram revealed cc-TGA,
dextrocardia with situs solitus, atrioventricular discordance and
ventriculoatrial discordance, severe systemic and sub-pulmonary
atrioventricular valves regurgitation, and severe pulmonary
hypertension. He underwent heart-lung transplant. Results: The patient
was discharged on postoperative day 25 on room air with normal left
ventricular ejection fraction. Conclusion: This case highlights the
complexity of preoperative and intraoperative management of cc-TGA, and
demonstrates the challenges which the surgeons and anesthesiologists may
face during the procedure of HLTx.%
\end{abstract}%



\sloppy


\textbf{Introduction}

Congenitally corrected transposition of the great arteries (cc-TGA) is a
rare type of congenital heart disease with an incidence of less than 1\%
of infants with congenital heart disease\textsuperscript{1}. Other terms
have been used to describe this congenital anomaly such as L-TGA, double
discordance and ventricular inversion. The first heart-lung
transplantation (HLTx) was performed at Stanford University in
1981.\textsuperscript{2} Pulmonary vascular disease and cystic fibrosis
were the main indications for HLTx initially. However, in the recent
years, the indication has been expanded to include congenital heart
disease and idiopathic pulmonary artery
hypertension.\textsuperscript{3}We report a rare case of cc-TGA with
dextrocardia presenting with biventricular failure who underwent HLTx.

\textbf{Materials and Methods}

A 53-year-old male patient presented to our institution with the
clinical picture of biventricular failure. The echocardiogram revealed
cc-TGA, dextrocardia with situs solitus, atrioventricular discordance
and ventriculoatrial discordance (figure 1A\&B), severe systemic
(morphologic tricuspid valve) and sub-pulmonary atrioventricular
(morphologic mitral valve) valves regurgitation, and severe pulmonary
hypertension (PH) (mean pulmonary artery pressure: 51 mmHg). His past
medical history was remarkable for complete heart block mandating
pacemaker implantation, and atrial fibrillation. The patient was
evaluated and was deemed a candidate for HLTx. A 25-year-old male donor
who died of hemorrhagic stroke became available and the en-bloc
heart-lung was recovered by an experienced team (figure 2A).

The patient underwent HLTx via a clamshell incision. Because of
hemodynamic instability, we commenced cardiopulmonary bypass (CPB) with
an aortic cannula and a dual-stage venous cannula placed in the right
atrium. After the heart was decompressed, the venous cannulation was
converted from atrial into bi-caval configuration.

Cardiectomy was performed, including complete removal of right and left
atria, leaving a stump of atrial tissue on the inferior vena cava. The
recipient trachea was resected one tracheal ring above the carina. The
left recurrent laryngeal and the vagus nerves were identified and
preserved during dissection. The two phrenic nerves were identified and
protected by leaving pericardial flaps on each side. Tracheal
anastomosis was performed first followed by the IVC, aorta and then the
superior vena cava. The aortic cross clamp was removed and the patient
was safely weaned from the CPB. Inotropic support was initiated using
Dobutamine at 5 mcg/kg/min and epinephrine at 0.05 mcg/kg/min. At the
end of the procedure, the patient was taken to the intensive care unit
in a stable condition.

The ischemic time was 296~minutes The CPB time was 280~minutes.

The standard immunosuppression protocol was initiated:

1- Methylprednisone 1 g IV intraoperatively, then 125 mg bid for the
first day, then 1mg/kg/day in divided doses for 3 days then prednisone
20 mg daily for 4 weeks then 10 mg daily.

2- Mycophenolate 1g IV intraoperatively, followed by 500 mg bid. Dose
was temporarily increased to 1000 mg bid on POD 18 with suspicion of
humoral rejection.

3- Single dose induction with Alemtuzumab 30 mg IV administered on POD
1.

4- Tacrolimus 0.15mg/kg/day dose titrated to achieve level of 6-10
mcg/L.

On postoperative day 2 the patient was extubated and ambulation was
initiated. The patient progressed steadily and ready for discharge,
however on POD 18, he developed pleural effusion and lung infiltrates.
Transbronchial lung biopsy revealed focal vasculitis. The diagnosis of
possible humoral rejection was considered and the patient received a
single dose of 500 mg, methylprednisone and 5 daily sessions of plasma
exchange. Also he developed gastroparesis which completely resolved
before discharge. Postoperative chest radiograph showed the heart and
lungs in the proper position (figure 2B).

\textbf{Results}

The patient was discharged on postoperative day 25 on room air with left
ventricular ejection fraction of 60-65\%.

\textbf{Discussion}

Over the past 20 years, the number of HLTx has been decreasing from 145
in 1999 to 45 in 2019 and gradually replaced with bilateral lung
transplant (BLTx).\textsuperscript{4} It has been an ongoing debate on
whether to perform HLTs or BLTx for patients with
PH.\textsuperscript{5}HLTx is recommended to patients with congenital
heart disease and Eisenminger syndrome, severe right ventricular (RV)
dysfunction, and severe left ventricular dysfunction. On the other hand,
BLTx provides comparable outcomes, moreover, it offers the advantage of
better organ sharing. Given the long waiting time, patients with PH and
simple cardiac anomalies such as atrial septal defect, patent ductus
arteriosus or perimembranous ventricular septal defect can be
successfully managed with a combination of BLTx and cardiac repair. The
cut-off values for BLTx can be approximated at 10-25\% RVEF and 32-55\%
LVEF. For values less than this cut-off, HLTx is
recommended.\textsuperscript{5}

We are reporting a successful HLTx in a patient with cc-TGA and
dextrocardia. Our patient was presented with advanced cardiac problems
including severe PH, severe systemic and sub-pulmonary atrioventricular
valves regurgitation, complete heart block and biventricular failure. In
our patient, the RV was the systemic ventricle and its failure
attributed to the severely incompetent systemic valve and the afterload
systemic pressure. Hence, BLTx will not improve the condition of the
failing systemic RV and the HLTx was deemed the suitable option. Based
on the aforementioned findings, the multidisciplinary team recommended
HLTx procedure. HLTx is technically a well-established surgical
procedure, however, our case demonstrated unique surgical challenges due
to the complexity of the congenital cardiac anomaly (cc-TGA and
dextrocardia) of the recipient. The combination of the cc-TGA and
dextrocardia is extremely rare. To the best of our knowledge, only one
case of cc-TGA and dextrocardia has been reported by Takemoto et al. in
the literature which was managed medically (i.e. no
HLTx).\textsuperscript{6} This combination posed challenges for both the
surgeon and anesthesiologist during the preoperative preparation and
during the transplant procedure. The strategy of the approach,
cannulation, and the technique was quite different from the standard
HLTx. Giving the fact that both the superior and inferior vena cava were
presented posteriorly, venous cannulation was initiated via dual stage
venous cannula placed in the right atrium transitioned to bi-caval
configuration after decompressing the heart.

According to the 2016 ISHLT report included the data of patients
underwent HLTx in 2004--2014, the survival rates at 1, 2, 5, and 10
years are 63\%, 52\%, 45\%, and 32\%, respectively, and a median
survival of 5.8 years.\textsuperscript{7} As of today, 15 months
postoperatively, the patient is doing well.

\textbf{Conclusion}

This case highlights the complexity of preoperative and intraoperative
management of cc-TGA, moreover, it demonstrates the challenges which the
surgeons and anesthesiologists may face during the procedure of HLTx.

\textbf{Author Contributions}

Concept/design: MME, MT, SMP

Data analysis/interpretation: MME, MT, AKM, DBE, JCLM, KPL, SMP

Drafting article: MME

Critical revision of article: MME, MT, BS, AKM, IAM, DBE, KPL, SMP

Approval of article: MME, MT, AKM, IAM, BS, DBE, KPL, SMP

Statistics: N/A

Data collection: MME

\textbf{References}

\begin{enumerate}
\tightlist
\item
  Bjarke BB, Kidd BSL. Congenitally corrected transposition of the great
  arteries: a clinical study of 101 cases. Acta Paediatr Scand
  1976;65:153--160.
\item
  Reitz BA, Wallwork JL, Hunt SA, et al. Heart-lung transplantation:
  successful therapy for patients with pulmonary vascular disease. N
  Engl J Med 1982;306:557-564.
\item
  Yusen RD, Edwards LB, Kucheryavaya AY, et al. The registry of the
  International Society for Heart and Lung Transplantation: thirty-first
  adult lung and heart-lung transplant report--2014; focus theme:
  retransplantation. J Heart Lung Transplant 2014;33:1009-1024.
\item
  Hill C, Maxwell B, Boulate D, et al. Heart-lung vs. double-lung
  transplantation for idiopathic pulmonary arterial hypertension. Clin
  Transplant 2015;29:1067-1075.
\item
  Olland A, Falcoz P, Canuet M, Massard G. Should we perform
  bilateral-lung or heart-lung transplantation for patients with
  pulmonary hypertension? Interact CardioVasc Thorac Surg
  2013;17:166-170.
\item
  Takemoto M, Aoki K, Noma M, Yamamoto H. Congenitally corrected
  transposition of the great arteries with dextrocardia. Clin
  Cardiol.~2006;~29(1):44.
\item
  Yusen RD, Edwards LB, Dipchand AI, Goldfarb SB, Kucheryavaya AY,
  Levvey BJ, et al. The Registry of the International Society for
  Heartand Lung Transplantation: Thirty-third Adult Lungand Heart--Lung
  Transplant Report---2016; Focus Theme: Primary Diagnostic Indications
  for Transplant. J Heart Lung Tranplant. 2016;35:1170-1184.
\end{enumerate}

\textbf{Figure Legends}

\textbf{Figure 1:} Preoperative chest radiograph showing the
dextrocardia anomaly (A), and diagram showing anatomy of the
congenitally corrected transposition of the great arteries with
dextrocardia and situs solitus. RV: right ventricle, LV: left ventricle,
RA: right atrium, LA: left atrium, IVC: inferior vena cava, SVC:
superior vena cava (B)

\textbf{Figure 2} : Intraoperative photo showing the en bloc heart and
lung after recovery (A), and postoperative chest radiograph showing the
transplanted heart and lungs in the proper position (B).\selectlanguage{english}
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\includegraphics[width=0.70\columnwidth]{figures/Fig/Fig}

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\includegraphics[width=0.70\columnwidth]{figures/Fih/Fih}

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