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\begin{document}

\title{Ten-year survival with Heart Mate-II in situs viscerum inversus.}



\author[1]{Alessandra Francica}%
\author[1]{Francesco Onorati}%
\author[1]{Livio San Biagio}%
\author[1]{Leonardo Gottin}%
\author[1]{Giuseppe Faggian}%
\affil[1]{Integrated University Hospital of Verona}%


\vspace{-1em}



  
  \date{May 17, 2024}


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\selectlanguage{english}
\begin{abstract}
End-stage heart failure (ESHF) represents the leading cause of death for
adult congenital heart disease (ACHD) and heart transplant (HT) remains
the first-line treatment. Although many patients are ineligible for
transplantation due to advanced comorbidities, left ventricle assist
devices (LVAD) is considered a valid option. Herein we present a case of
dextrocardia in situs viscerum inversus totalis (SVIT) who was implanted
with LVAD HeartMate II ten years ago. To the best of our knowledge, this
was the first case of dextrocardia treated with a totally intrathoracic
device and he is to date the longest survivor with a HeartMate II in
SVIT.%
\end{abstract}%



\sloppy


\textbf{Case report}

A 58-year-old man with ischemic cardiomyopathy in SVIT underwent LVAD
(HeartMate II; Thoratec Corporation, Pleasanton, CA) implant on November
2009. The postoperative course was uncomplicated and he was discharged
home in healthy condition.

In the following years he remained in stable clinical conditions (NYHA
class I-II) with a good quality of life (KCCQ=79). LVAD parameters were
always stable. He was regularly followed-up at our Centre (Figure 1). Up
to ten years after surgery, chest X-ray never reported signs of
pulmonary congestion (Figure 2).

No events of LVAD thrombosis or device dysfunction occurred ever. During
time he developed a progressive chronic renal failure with preserved
urinary output, now in stage IIIb KDOQI (eGFR 40 ml/min). During the
last year, the patient needed a new hospitalization for recurrent severe
gastrointestinal bleeding, requiring multiple endoscopic treatments,
prolonged discontinuation of antiplatelet and anticoagulant regimens
(15-days during which the INR was always [?]1.4), and transitory
administration of octeotride and fresh frozen plasma. Despite that, no
thrombotic event occurred, and no LVAD dysfunction developed. Subsequent
postoperative course complicated further with pneumonia, successfully
treated with antibiotic therapy and non-invasive positive-pressure
ventilation. The patient successfully recovered and is next to be
discharged in rehabilitation after 4 months of hospitalization.

Last echocardiography (after 123 months from LVAD implant) showed normal
inflow and outflow doppler-signals, mild aortic valve regurgitation, and
a moderate right ventricular dysfunction with mild tricuspid
regurgitation. Despite that, the patient was hemodynamically stable in
NYHA class II, with stable LVAD parameters.

Because of the overall successful postoperative course and the good
hemodynamic stability, HT was not offered during years.

\textbf{Comment}

ESHF in patient with CHD who survive into adulthood remains today a
challenge for both HT and VAD implantation, because of the complex
pathophysiology and potential comorbidities.

To date, publications regarding outcomes and use of LVADs in ACHD
patients are limited.

Of the 16,182 adult patients in the Interagency Registry for
Mechanically Assisted Circulatory Support (INTERMACS) database, only 126
(0.8\%) have CHD.\textsuperscript{1} Additional analysis on outcomes
shows that ACHD patients with LVADs have similar survival, adverse event
rates and similar improvements in functional capacity and quality of
life compared with non-ACHD patients.\textsuperscript{6} Actually,
INTERMACS registry \textsuperscript{1} shows an overall-survival about
70\% at 24 months after implantation of LVADs in ACHD patients, but no
data are available on survival beyond 24 post-operative months.

Other literature data on VAD-surgery in ACHD patients consist mainly of
case-reports or small series. Of note, besides our patient who reported
the first implant ever of a HeartMate II in SVIT\textsuperscript{2},
other HeartMate II implantations in adults with SVIT are only reported
twice: Ariyachaipanich et al.\textsuperscript{3} described a case of
right ventricular support with HeartMate II in a 56-year-old man with a
history of SVIT as destination therapy (DT). The patient had an
uncomplicated postoperative course and did not require any readmission
during the first year after surgery. No data were available beyond the
first postoperative year. Menachem et al.\textsuperscript{4} reported a
case of a 75-year-old female with SVIT implanted with HeartMate II as
DT. She expired early because of neurologic complications.

To the best of our knowledge therefore, this is the first documented
case of 10-year survival after HeartMate II implantation in a patient
with SVIT. Interestingly enough, long-term follow-up was quite
uneventful for more than 8 years, and no device failure, pump
thrombosis, or stroke occurred ever during ten-year follow-up, allowing
an overall excellent quality of life for a decade. Of note, the
prolonged withdrawal of both anti-platelet and anti-thrombotic regimens
during the last haemorrhagic event did not led to VAD thrombosis or
device malfunction, nor to thromboembolic events, possibly in light of a
long-lasting complete formation of neo-intimal tissue
with~endothelialization of the inner surfaces of the device.

In conclusion, ESHF remains nowadays the leading cause of death for ACHD
patients. The ease availability of LVADs provides an elegant solution to
these complex scenarios, sometimes allowing long-lasting and consistent
results as in the case reported. The recent availability of new
generations of these devices can possibly raise the rod further in the
next future.

\textbf{Bibliography}

1. VanderPluym CJ et al. Outcomes following implantation of mechanical
circulatory support in adults with congenital heart disease: An analysis
of the Interagency Registry for Mechanically Assisted Circulatory
Support (INTERMACS). J Hear Lung Transplant. 2018. 2. Faggian G et al.
LVAD in situs viscerum inversus totalis. J Hear Lung Transplant, Vol 30,
No 12, December 2011 3. Ariyachaipanich A, Mudd JO, Gelow J, Song HK.
Long-term systemic right ventricular support in transposition and
dextrocardia. J Thorac Cardiovasc Surg. 2012 3. Menachem JN et al.
Initial Experience of Left Ventricular Assist Device Support for Adult
Patients with Transposition of the Great Vessels. Congenit Heart Dis.
2015 5.Cedars A et al. An Interagency Registry for Mechanically Assisted
Circulatory Support (INTERMACS) analysis of hospitalization, functional
status, and mortality after mechanical circulatory support in adults
with congenital heart disease. J Hear Lung Transplant. 2018.

\textbf{Figure Legends}

1. 3D-recontruction at ten years after implantation. 2. Chest X-ray
after ten years from implantation.\selectlanguage{english}
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\includegraphics[width=0.70\columnwidth]{figures/1/1}

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\includegraphics[width=0.70\columnwidth]{figures/2/2}

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