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\begin{document}

\title{A rare localization and Presentation of cardiac Paraganglioma}



\author[1]{Alessandro Barbone}%
\author[1]{Giosuè Falcetta}%
\author[1]{Giovanni La Canna}%
\author[1]{Lucia Torracca}%
\affil[1]{IRCCS Humanitas Clinical and Research Center}%


\vspace{-1em}



  
  \date{May 05, 2024}


\begingroup
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\selectlanguage{english}
\begin{abstract}
Paragangliomas (PGLs) are rare neuroendocrine tumors that arise from the
autonomic ganglia. A 34-year-old woman was admitted to our department
with palpitation, fatigue and chest pain under stress. Past medical
history included a para-carotid PGL surgically removed 5 years earlier.
Her family history was notable for familiar PGL with SDH mutation. At
stress-echocardiography, was recorded a marked ST-elevation on
precordial-leads, concomitant to new onset anterior septum hypo-kinesis,
and therefore the patient was proposed for coronary angiography.
Although angiographic evidence of atherosclerosis were not recorded, was
found that Left Anterior Descending Coronary Artery gave off some
branches towards a vascular structure (3\selectlanguage{ngerman}×2 cm) in contiguity with the
left coronary sinus of the aortic root. Subsequent Compute-Tomography
and Magnetic-Resonance clarified the anatomical relationships of the
mass with adjacent cardiac structures, in particular the mass was
located behind the pulmonary artery trunk and lateral to aortic root,
lying above the first portion of the anterior descending coronary
artery. Subsequently a careful discussion about the risks and benefits
of all therapeutic opportunity, the open surgical resection was deemed
the best option for the patient despite the complex clinical anatomy.
After standard cannulation for CPB and cardioplegic arrest, transection
of the pulmonary artery and the aorta was performed granting adequate
exposure of the mass and identification of afferent and efferent
vascularization for complete resection though a cleavage plane. To
ensure adequate hemostasis on a such critical area, the continuity of
the pulmonary trunk was ensured only after removing aortic cross-clamp.
The post-operative course was uneventful%
\end{abstract}\selectlanguage{ngerman}%



\sloppy


\textbf{Title: A rare localization and Presentation of cardiac
Paraganglioma}

\textbf{Running Head: Imaging of Cardiac Paraganglioma}

Alessandro Barbone\textsuperscript{1},Giosu\selectlanguage{ngerman}è
Falcetta\textsuperscript{1}Giovanni La Canna\textsuperscript{2}, Lucia
Torracca\textsuperscript{1}

Form the: UO of Cardiac Surgery\textsuperscript{1}: IRCCS~Humanitas
Clinical and Research Center, Via A. Manzoni 56, 20089 Rozzano (MI);

Applied echocardiography service Unit\textsuperscript{2}:
IRCCS~Humanitas Clinical and Research Center, Via A. Manzoni 56, 20089
Rozzano (MI);

Classification:

Key words : Paraganglioma

No conflict of interest is reported by any of the authors

Total Word Count 472

Please Address correspondence to: Alessandro Barbone M.D. PhD.

c/o IRCCS Humanitas Clinical and Research Center

Area Medici Ovest 1

Via A. Manzoni 56 -- 20089 Rozzano (Mi)

Italy

E-Mail:\emph{alessandro.barbone@humanitas.it}

Tel: +39 02 8224 4602

Fax: +39 02 8224 4692

\textbf{Text}

Paragangliomas (PGLs) are rare neuroendocrine tumors that arise from the
autonomic ganglia. The familial PGLs have been associated most commonly
with succinate-dehydrogenase (SDH) complex mutation of the electron
transport chain.(1)

A 34-year-old woman was admitted to our department with palpitation,
fatigue and chest pain under stress. Past medical history included a
para-carotid PGL surgically removed 5 years earlier. Her family history
was notable for familiar PGL with SDH mutation.

At admission was asymptomatic at rest without history of smoking,
hypertension, diabetes, coronary artery disease, or peripheral
atherosclerosis.

At stress-echocardiography, was recorded a marked ST-elevation on
precordial-leads, concomitant to new onset anterior septum hypo-kinesis,
and therefore the patient was proposed for coronary angiography.
Although angiographic evidence of atherosclerosis were not recorded, was
found that Left Anterior Descending Coronary Artery gave off some
branches towards a vascular structure (3×2 cm) in contiguity with the
left coronary sinus of the aortic root (Fg2 + Vid). Subsequent Compute-
Tomography and Magnetic-Resonance clarified the anatomical relationships
of the mass with adjacent cardiac structures (Fig 2 -- 4), in particular
the mass was located behind the pulmonary artery trunk and lateral to
aortic root, lying above the first portion of the anterior descending
coronary artery.

Subsequently a careful discussion about the risks and benefits of all
therapeutic opportunity (2), the open surgical resection was deemed the
best option for the patient despite the complex clinical anatomy(3).

After standard cannulation for CPB and cardioplegic arrest, a
transection of the pulmonary artery and the aorta was performed granting
adequate exposure of the mass and identification of afferent and
efferent vascularization for complete resection though a cleavage plane
(Fig 1). To ensure adequate hemostasis on a such critical area, the
continuity of the pulmonary trunk was ensured only after removing aortic
cross-clamp.

The post-operative course was uneventful.

\textbf{References}

1. Welander J, Söderkvist P, Gimm O. Genetics and clinical
characteristics of hereditary pheochromocytomas and paragangliomas.
2011;18(6):R253.

2. Khan MF, Datta S, Chisti MM, Movahed MR. Cardiac paraganglioma:
Clinical presentation, diagnostic approach and factors affecting short
and long-term outcomes. International Journal of Cardiology
2013;166(2):315-320.

3. Ramlawi B, David EA, Kim MP et al. Contemporary surgical management
of cardiac paragangliomas. The Annals of Thoracic Surgery
2012;93(6):1972-1976.

\textbf{Figure Legend:}

\textbf{Figure 1 (Central Picture):} Surgical View of PGL: to be noted
the transected Aorta and Pulmonary Artery. LAA: Left Atrial Appendage;
in the right upper corner: surgical specimen after removal

Fig 2: Coronary Angiography and Computerized Tomography with 3D
Rendering; arrows are pointing at the PGL

Fig 3: Echocardiographic (Trans-esophageal) (TEE) and Magnetic Resonance
View (MR); arrows pointing at the PGL.

\textbf{Video Legend:}

Coronary angiography\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/Fig1/Fig1}

\end{center}
\end{figure}\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/Fig2/Fig2}

\end{center}
\end{figure}\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/Fig3/Fig3}

\end{center}
\end{figure}

\selectlanguage{english}
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