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\begin{document}

\title{Efficient Approach to Superior Vena Cava Baffle Stenosis Following the
Mustard Procedure: Expanding Role of a Mechanical Rotating Dilator
Sheath for Lead Extraction}



\author[1]{Chi Chi Do-Nguyen}%
\author[2]{Alexander Ochman}%
\author[1]{Maxwell Kilcoyne}%
\author[2]{Richard Kovach}%
\author[3]{Boban Abraham}%
\author[2]{Pedram Kazemian}%
\author[2]{Lynn McGrath}%
\author[3]{Randy Stevens}%
\affil[1]{Philadelphia College of Osteopathic Medicine}%
\affil[2]{Deborah Heart and Lung Center}%
\affil[3]{Saint Christopher's Hospital for Children}%


\vspace{-1em}



  
  \date{May 26, 2024}


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\selectlanguage{english}
\begin{abstract}
Introduction: In adult congenital patients with transposition of the
great arteries originally treated with the Mustard (atrial switch)
procedure, the most common reason for re-intervention is baffle
stenosis. This may be exacerbated by permanent transvenous pacemaker
lead placement across the baffle. Case Report: A 47-year-old female
status post Mustard procedure performed at 15 months old presented with
a high-grade stenosis of the superior vena cava (SVC) baffle from the
SVC to the left atrium, with a nonfunctional permanent pacemaker lead
passing through the baffle. A mechanical rotating dilator sheath was
used for attempted lead extraction, relieving the baffle stenosis almost
completely as a secondary effect, prior to the placement of a 10 x 27 mm
Visipro balloon expandable stent in the SVC baffle. Conclusions: Use of
the mechanical rotating dilator sheath is an evolving treatment strategy
in adult congenital heart disease to minimize the risk of bleeding,
trauma to surrounding structures, and death. Its ability to fully
alleviate baffle stenosis even when full lead extraction is not feasible
or is associated with significant procedural risk further demonstrates
its expanded role in this patient population. A multidisciplinary
approach and great diligence must be employed to avoid potential
complications.%
\end{abstract}%



\sloppy


\textbf{INTRODUCTION}

In adult congenital patients with transposition of the great arteries
(TGA) originally treated with the Mustard (atrial switch) procedure,
long term complications include baffle stenosis, arrhythmias, and sudden
death.\textsuperscript{1} The re-intervention rate for these patients is
as high as 9.7\%, with the most common reason being baffle
stenosis.\textsuperscript{2} Permanent pacemakers are required in this
patient population for management of symptomatic sinus node or AV node
dysfunction and the presence of transvenous leads across the baffle can
increase stenosis rates up to 58\%.\textsuperscript{1,3} This report
describes a case of multidisciplinary lead extraction and recanalization
of an occluded superior vena cava (SVC) baffle using a mechanical
rotating dilator sheath in a patient with a prior Mustard procedure and
retained left atrium (LA) pacemaker lead.

\textbf{CASE REPORT}

A 47-year-old female with a past medical history notable for complete
d-TGA and an extensive interventional history (\textbf{Table 1} )
presented with SVC syndrome. An echocardiogram revealed a high-grade
stenosis of the SVC baffle from the SVC to the LA, with a mean gradient
of 5 mmHg and remaining luminal area of approximately 1 mm. A
nonfunctional permanent pacemaker lead passing through the baffle and
located in the LA was contributing to the obstruction and the patient
was scheduled for extraction of the LA lead and balloon angioplasty and
stenting of the SVC baffle.

\emph{Surgical Planning}

In a hybrid operating room, 6 and 7 French (Fr) sheaths were advanced
percutaneously using a micropuncture technique into the right common
femoral artery and vein, respectively.~A 6 Fr sheath and a 10 Fr multi
lumen venous sheath was advanced similarly into the left common femoral
artery and vein, respectively. In the event emergent bypass was needed,
a 4 Fr sheath was advanced into the left superficial femoral artery and
a 9 Fr sheath was advanced under ultrasound guidance into the right
internal jugular (RIJ) vein.~The patient was then partially
anticoagulated.

Cardiac catheterization demonstrated no flow through the SVC baffle,
with venous drainage from the upper body traveling through a massively
dilated hemi-azygous vein in communication with the inferior vena cava
(IVC), which drains into the LA via the IVC baffle. The IVC baffle had
no signs of obstructive disease (\textbf{Figure 1} ).

\emph{Multidisciplinary Lead Extraction and Baffle Obstruction Relief}

Manual traction of the non-functioning LA lead demonstrated complete
attachment to the subclavian vein and SVC. To increase the safety of the
extraction procedure and to provide hemostasis in case of an SVC tear,
an occlusive balloon (Bridge Occlusion Balloon 590-001, Philips) was
deployed in the SVC by way of the right internal jugular vein. Attempt
retracting the active fixation mechanism of the lead was unsuccessful. A
locking stylet and short-length mechanical dilating rotator sheath (9F
TightRail Sub-C 560-009, Philips) was used to lyse the adhesions from
the entrance of the lead into the subclavian vein. The dilator was
exchanged for a long rotational sheath (9F TightRail 545-509, Philips)
(\textbf{Figure 2} ) and the adhesions for the rest of the lead from the
turn of the SVC into the baffle were released. The tip of the lead was
firmly embedded inside the atrial baffle and could not be safely
extracted despite multiple attempts (\textbf{Figure 3A} ).~The use of
the mechanical dilating rotator sheath almost completely relieved the
stenosis at the baffle ostium as a side effect (\textbf{Figure 3B} ).
Due to resolution of stenosis, lack of progress, and an increasing risk
of perforation, a decision was made not to proceed with complete
extraction of the lead. The lead was cut and capped near the entry into
the subclavian vein and the procedural site was closed. The patient was
then fully anticoagulated.

A 10 x 27 mm Visipro balloon expandable stent was advanced into the RIJ
vein, positioned safely across the stenotic segment of the baffle, and
deployed with a 14-mm balloon (\textbf{Figure 3C} ). Repeat angiography
demonstrated very brisk flow from the SVC through the baffle with no
residual pressure gradient (\textbf{Figure 3D} ).~Anticoagulation was
reversed and the stent delivery system was removed.

At her one month follow up, the patient remained on dofetilide and
Eliquis for rate control and anticoagulation, respectively, with no
symptoms nor complications.

\textbf{DISCUSSION}

Stenosis of the SVC baffle occurs in up to 40\% of patients treated with
the Mustard procedure, with risk factors including a Dacron baffle, a
tortuous baffle course, baffle leaks, and a young age at
operation.\textsuperscript{1,2,4,5} With the increase in permanent
pacemaker insertion in this patient population, transvenous lead
placements across the baffle may increase stenosis rates to up to
58\%.\textsuperscript{1,3} Baffle stenosis can lead to severe
complications, such as persistent hemodynamic load, hypoxia,
thromboembolic events, and SVC syndrome, requiring
re-intervention.\textsuperscript{3}

Transcatheter interventions have been the preferred therapeutic option
in transvenous lead extraction and relief of baffle obstructions,
including laser lead extraction or radiofrequency perforation followed
by angioplasty and stent placement.\textsuperscript{2-4,6,7} Laser lead
extraction uses pulsatile ultraviolet light to dissolve fibrous tissue,
whereas radiofrequency perforation uses rapid
heating.\textsuperscript{6-8} However, both methods are associated with
risks of cardiac wall perforation and damage to surrounding structures
or the conduction system.\textsuperscript{6,7}

In adult congenital heart disease (CHD), use of the mechanical rotating
dilator sheath is an evolving treatment strategy to minimize the risk of
bleeding, trauma to surrounding structures, and death. The baffle
stenosis is typically more compliant than native tissue stenosis.
Therefore, great diligence is required with the mechanical rotating
dilator sheath to avoid over-dilation and puncture through the baffle
wall.\textsuperscript{2} Intraprocedural angiography aids in guiding
dilatation and maintaining safety. Complete transvenous lead extraction
in CHD patients has been successful in up to 92\% of patients in prior
studies, with failures attributed to calcified adhesions or active
fixation.\textsuperscript{3,6,9,10} In our case, we recognized the risks
associated with completely removing the nonfunctional LA lead.
Therefore, extraction was halted.

A second benefit of using the mechanical rotating dilator sheath was its
ability to fully alleviate the SVC baffle stenosis. A stent may not have
been necessary after attempted lead extraction in this case due to the
low gradient shown after adhesiolysis alone. However, recognizing that
delaying baffle stenting may lead to rapid thrombosis, especially after
extensive adhesiolysis, we continued with deployment of the
balloon-expandable stent.\textsuperscript{3} Optimal stent placement is
crucial for future pacemaker lead placement, which may be necessary in
the case of this patient, as well as the benefits of decreased baffle
gradients, increased baffle diameter, relief of clinical symptoms, and
delay of the need for re-intervention.\textsuperscript{2}

A multi-disciplinary team approach including an interventional
cardiology, electrophysiology, and cardiothoracic surgical team that are
familiar with adult CHD is crucial in managing these complex cardiac
cases safely. Prophylactic measures are taken to avoid complications,
such as intubation, full sedation, and placement of a transesophageal
echocardiogram (TEE) probe. During the procedure, obtaining access from
the bilateral femoral arteries and veins allows for accurate
localization of the baffle from above and below, as well as continuous
imaging during stent placement.\textsuperscript{2} Further, obtaining
access for emergent bypass and placing a sheath and balloon in the right
internal jugular vein for emergent tamponade are necessary in procedural
planning, as the periprocedural mortality rate for baffle-related
re-intervention can be as high as
29\%.\textsuperscript{2,5,11}Additionally, the extent and timing of
anticoagulation is pre-planned to avoid excessive bleeding throughout
the course of the combined procedure.

\textbf{CONCLUSION}

In patients with TGA previously treated with the Mustard procedure, the
high prevalence of long term complications due to transvenous pacing and
baffle stenosis leads to clinically challenging situations. This report
represents the outcomes of a unique, safe, and feasible strategy used in
dilation of baffle stenosis using a mechanical rotating dilator sheath,
followed by balloon-expandable stenting of the SVC baffle with a jailed
non-functioning LA lead. Further studies are needed to assess the
efficacy and safety of the mechanical rotating dilator sheath in other
settings of CHD.

\textbf{REFERENCES}

\begin{enumerate}
\tightlist
\item
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\item
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\item
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\item
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\item
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\item
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\item
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\item
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\item
  TightRail rotating mechanical dilator sheath product brochure. San
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  Accessed April 13, 2020.
\end{enumerate}\selectlanguage{english}
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