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\begin{document}

\title{Successful Venoplasty of Superior Vena Cava Stenosis in a Patient with a
Total Artificial Heart after Orthotopic Heart Transplantation due to
Primary Graft Failure}



\author[1]{Anita Phancao}%
\author[2]{Aly El Banayosy}%
\author[2]{Stephen Lee}%
\author[2]{David Vanhooser}%
\author[2]{Michael Harper}%
\author[2]{Douglas Hortsmanshof}%
\author[2]{James Long}%
\author[2]{Michael  M Koerner}%
\affil[1]{University of Miami Department of Medicine}%
\affil[2]{INTEGRIS Baptist Medical Center}%


\vspace{-1em}



  
  \date{May 27, 2024}


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\selectlanguage{english}
\begin{abstract}
Background: With the limited number of available suitable donor hearts
resulting in plateaued numbers of heart transplantations, short- and
long-term mechanical circulatory support devices, including the
implantation of total artificial hearts (TAH) are modalities that are
increasingly being used as treatment options for patients with end-stage
heart failure. The superior vena cava syndrome has been described in
this context in various disease processes. We report successful
venoplasty for superior vena cava syndrome in a patient with a TAH. Case
Presentation: A 65 years old man with a history of non-ischemic
cardiomyopathy had received a left ventricular assist device, and then
two years later underwent orthotopic heart transplantation using the
bicaval anastomosis technique. The post procedural course was
complicated by primary graft failure, resulting in the need for the
implantation of a TAH. About 5 months after the TAH implantation he
started to develop complications such as volume retention, swelling of
the upper extremities, and was diagnosed to have a superior vena cava
syndrome. The patient underwent a successful venoplasty of his superior
vena cava by interventional radiology with resolution of upper body
edema, normalization of renal and liver function. Conclusion: Potential
fatal complications caused by catheter or wire entrapment in the right
sided mechanical valve of a TAH have been reported. We describe a safe
method for the treatment of superior vena cava syndrome in patients with
TAH.%
\end{abstract}%



\sloppy


\textbf{Background}

A total artificial heart (TAH) is a form of heart replacement therapy
that provides cardiocirculatory support in end-stage heart failure being
used as a bridge to heart transplantation or destination
therapy\textsuperscript{1,2}. Due to limited availability of acceptable
donor hearts, left ventricular assist devices (LVADs), biventricular
assist devices, and TAHs are becoming increasingly used as options for
the treatment of end-stage heart failure if conventional treatment is
failing.

Superior vena cava syndrome (SVCS) is a condition in which the superior
vena cava (SVC) is partially blocked or compressed, leading to a
constellation of symptoms including upper extremity swelling, shortness
of breath, coughing, hoarseness, as well as more serious complications
such as cerebral edema, confusion, multi organ dysfunction, and
coma\textsuperscript{3}.

SVCS is most commonly recognized in patients with
malignancies\textsuperscript{4}, and has also been reported to be caused
by infections\textsuperscript{5}, multiple catherization of the venous
system, pacemaker placement, and heart
transplantation\textsuperscript{3,6 -8}. Management of SVCS is dependent
on the severity of disease as well as underlying etiology. Percutaneous
intervention for SVCS is a treatment modality which has provided
successful results\textsuperscript{3}. Although there have been reports
of percutaneous intervention for relief of SVCS in those with heart
transplantation\textsuperscript{6,9}, so far there has only been one
case being reported in the literature in regard to the treatment of SVCS
in a patient with TAH, although this was caused by external compression
of the SVC due to bleeding and tamponade\textsuperscript{10}.

\textbf{Case Presentation}

A 65 year old man with a history of chronic renal insufficiency, dual
chamber AICD, non-ischemic cardiomyopathy, and end-stage heart failure
received a non-pulsatile LVAD (HeartMate 3\selectlanguage{ngerman}®, Abbott, Boston, MA) as a
bridge to heart transplantation.

The patient did well while under LVAD support. After two years he was
re-evaluated and subsequently listed for heart transplantation. He
received an appropriately matched donor heart which was implanted using
the bicaval anastomosis technique. Unfortunately his orthotopic heart
transplantation was complicated by severe primary graft failure
requiring the placement of bilateral centrifugal pumps (Centrimags®,
Abbott, Pleasanton, CA). His donor heart did not recover and therefore
he received an implantation of a 70 cc TAH (CardioWest temporary Total
Artificial Heart System; SynCardia Systems®, Inc, Tucson, AZ). He had a
prolonged hospitalization during which he required the placement of a
temporary dialysis catheter for continuous renal replacement therapy.
This procedure was done under continuous fluoroscopy to avoid any
life-threatening complications such as entrapment of the guide wire
and/or line into the right sided mechanical valve of his TAH. In the
following time, his renal function eventually recovered but he
chronically required high dosages of diuretics. At that time he also
experienced episodes of confusion, agitation, emotional lability, and
short episodes of expressive aphasia. The computed tomography of his
head only noted a small subarachnoid hemorrhage in the left frontal
lobe. The patient recovered and was discharged home.

Three months after TAH implantation he was admitted with shortness of
breath, weight gain, low fill volumes and low cardiac output readings on
his TAH. Facial and upper extremity swelling was noted. A central line
was placed under continuous imaging with projection of its tip just on
the upper part of the SVC. The readings of his central venous pressure
were exceeding values of 20 cm of H\textsubscript{2}O. Due to
significant hypertension he was treated with multiple antihypertensive
medications and diuretics.

Subsequently he was readmitted monthly to bimonthly with various
problems such as epistaxis and labile mood swings. During all these
admissions edema of his upper extremities, primarily left sided edema,
and facial swelling had been noted. During his entire post TAH
implantation course, he did receive multiple fluoroscopy guided central
lines, as well as temporary dialysis catheter placements for volume
removal due to diuretic resistance. The tips of the guide wires and
lines were, intentionally, never inserted deeper than just into the
upper part of the SVC to avoid the possibility of a wire and or a line
entrapment into the TAH. A subclavian access was considered as
contraindicated due to the risk that lifting his arm may generate the
possibility that the tip of the line could advance deep enough getting
entrapped in the right sided valve. The central venous pressures were
always noted to be significantly elevated and considered to be caused by
renal insufficiency in a patient with questionable compliance and
intermittent need for intravenous diuretics and/or renal replacement
therapy.

Due to the patient's recurrent clinical presentations, an additional
component such as SVCS was suspected, although venous ultrasound studies
of his upper extremities did not reveal any deep venous thrombosis or
occlusion. During one of his admissions for volume retention, a
temporary dialysis catheter placement was performed by interventional
radiology with findings consistent of severe stenosis of the SVC. The
major concern for a possible venoplasty was that the treatment option
carried the possibility for the guide wire not to pass immediately
through the stenosis and curve above the stenosis without passing
through the opening, which could eventually build up kinetic energy
during the attempt to correct its position and unintentionally suddenly
flip through the stenosis vested with additional kinetic energy and
length when straightened. This uncontrolled move may hypothetically
deviate the wire into the right sided valve of the TAH rather than to
stay in the patient's SVC or inferior vena cava. Under risk benefit
analysis and informed consent by the patient and his proxies, the
decision was made for venoplasty of the SVC stenosis by interventional
radiology under fluoroscopy guidance. The pressure gradient within the
SVC was measured above the stenosis at 30 mmHg and below the stenosis at
18 mmHg (Figure 1, 2). The patient's SVC was serially dilated with 10
mm, 14 mm, and 18 mm diameter angioplasty balloons (Figures 3, 4). The
12 mmHg gradient was decreased finally down to 3 mmHg after angioplasty
(Figure 5). The patient's symptoms including the swelling of his upper
extremities, shortness of breath, and hoarseness all improved post
procedure. He has been discharged and will be evaluated for combined
heart/kidney transplantation.

\textbf{Conclusion}

Durable mechanical circulatory support is becoming increasingly used due
to the shortage of available or suitable donor hearts for end-stage
heart failure. External and intravascular mechanical complications will
arise with increased use of these devices, the use of the bicaval
anastomosis technique for orthotopic heart transplantation, as well as
placement of central venous catheters.

Entrapment of guide wires, catheters or central lines into the right
ventricular inflow valves of a TAH is a fatal complication in the
management of this patient population\textsuperscript{11}. Although
stent placement for SVCS caused by external compression due to bleeding
and tamponade in TAH has previously been described\textsuperscript{10},
the risk of stent migration in endovascular stenting in those with
SVCS\textsuperscript{9} could create lethal outcomes in this population.
In life-threatening situations such as profound hypoxic lung dysfunction
and adult respiratory distress syndrome after TAH
implantation\textsuperscript{12,13} invasive instrumentation with the
help of guide wires has been performed successfully. Here we describe a
safe and feasible treatment option in TAH patients with hemodynamically
relevant SVCS caused by primarily vascular related impairment with
venoplasty of the SVC.

The development of SVCS after advanced cardiac therapy therapies,
including TAH and heart transplantation may have multiple contributing
factors. In patients with TAH after heart transplantation, the
development of a SVC stenosis may have a higher incidence due to
remaining donor heart tissue that may be a focus of immune related
changes with the development of scarring and/or necrosis, especially if
immunosuppressive therapy is stopped after the graft has been removed
and TAH implanted. Patients with the need of TAH after orthotopic heart
transplantation, multiple manipulations due to central line placement in
the SVC, defibrillator or pacemaker insertion and manipulations, heart
transplant recipients with bicaval rather than biatrial anastomosis
between the recipient and the donor heart, all may have a higher
tendency to develop SVCS although this remains a topic of clinical
observation and discussion.

\textbf{References}

\textbf{1.} El-Banayosy A, Arusoglu L, Morshuis M, Kizner L, Tenderich
G, Sarnowski P, Milting H, Koerfer R: CardioWest total artificial heart:
Bad Oeynhausen experience. Ann J Thorac Surg 2005; 80(2):548-52.

2. Copeland JG, Copeland H, Gustafson M, Mineburg N, Covington D, Smith
RG, Friedman M: Experience with more than 100 total artificial heart
implants. J Thorac Cardiovasc Surg 2012;143:727-34.

3. Wilson LD, Detterbeck F, Yahalom J. Clinical practice. Superior vena
cava syndrome with malignant causes. N Engl J Med 2007;356(18):1862-9.

4. Lepper PM, Ott SR, Hope H, Schumann C, Stammberger U Bugalho A, Frese
S, Schmucking M, Blumstein NM, Diehm N, Bals R, Jamacher J. Superior
Vena Cava Syndrome in Thoracic Malignancies. Respir Care
2011;56(5):653-666.

5. Nunnelee JD. Superior vena cava syndrome. J Vasc Nurs 2007; 25(1):
2-5 quiz 6

6. Strecker T, Zimmerman I, Heinz M, Rosch J, Agaimy A, Weyan M.
Successful venous angioplasty of superior vena cava syndrome after heart
transplantation. Case Rep Cardiol 2014; 2014:490276.

7. Kashani BS, Ahmadi ZH, Abdi S, Mirhosseini SM, Kianfar AA Niusha S.
Superior vena cava obstruction after heart transplantation. Asian
Cardiovasc Thorac Ann. 2016;24(1):88-90.

8. Metzer C, Lembcke A, Ziemer S, Eddicks S, Witte J, Baumann G, Borges
AC. Pacemaker induced superior vena cava syndrome: clinical evaluation
of long term follow-up. Pacing Clin Electrophysiol 2006;29(12):1346-51.

9. Rejjal L, Al-Taweel O, Alwash H, Choudhry M. Alghrouz M, Khalife W,
Morsy M, Al-Taweel A, Chatila K, Gilani K, Gilani S. Stent migration
during endovascular stenting in superior vena cava syndrome in a patient
with recent heart transplantation. JACC 2019;79(9):2741.

10. Kondruweit M, Strecker T, Seitz T, Uder M, Weyand M, Tandler R,
Stenting of the superior vena cava in a patient with a total artificial
heart. J Thorac Cardiovasc Surg 2008; 135:455-6.

11. Zimmerman H, Coehlo-Anderson R, Slepian M, Smith RG, Sethi G,
Copeland JG: Device malfunction of the CardioWest Total Artificial Heart
secondary to catheter entrapment of the tricuspid valve. ASAIO J
2010;56:481-82.

12. Spiliopoulos S, Dogan G, Guersoy D, Serrano MR, Koerfer R, Tenderich
G: veno-venous extracorporeal membrane oxygenation with a bicaval
dual-lumen catheter in a Syncardia total artificial heart patient. J
Cardiothorac Surg 2013; 8:179.

13. Lick SD, Tran PL, Kazui P, Smith RG, Khalpey ZI: Total Artificial
Heart, augmented by venovenous extracorporeal membrane oxygenation.
ASAIO J 2016;62(4): e35-6.

\textbf{Figure 1:} Angiographic catheter (5 French Berenstein, 65 cm
length, Torcon NB Beacon Tip, Cook, Inc., Bloomington, IN) inserted from
the right common femoral vein through a 9 French vascular sheath.~ The
tip of the catheter is situated in the upper portion of the inferior
vena cava prior to passage through the TAH and into the SVC.

\textbf{Figure 2} : Superior venacavogram using a straight flush
catheter (5 French, 65 cm length, Boston Scientific, Marlborough MA).~A
high-grade stenosis of the superior vena cava is evident.

\textbf{Figure 3} : Angioplasty balloon catheter (14 mm diameter Atlas,
7 Fr, Bard Incorporated Tempe, AZ) positioned within the superior vena
cava.~ Balloon is in the process of being inflated and demonstrates the
site of vena cava stenosis (full inflation of the balloon not depicted).

\textbf{Figure 4} : Further angioplasty performed with a larger diameter
balloon.~Fully inflated 18 mm diameter angioplasty balloon (Atlas, 7 Fr,
Bard Inc., Tempe AZ) across the site of SVC stenosis.

\textbf{Figure 5} : Repeat superior venacavogram shows no
hemodynamically significant residual stenosis.

FIGURE 1\selectlanguage{english}
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\includegraphics[width=0.70\columnwidth]{figures/image3/image3}

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FIGURE 2\selectlanguage{english}
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\end{center}
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FIGURE 3\selectlanguage{english}
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\end{center}
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FIGURE 4\selectlanguage{english}
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\includegraphics[width=0.70\columnwidth]{figures/image6/image6}

\end{center}
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FIGURE 5\selectlanguage{english}
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\includegraphics[width=0.70\columnwidth]{figures/image7/image7}

\end{center}
\end{figure}

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