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\begin{document}

\title{Percutaneous Coronary Intervention for Ventricular Fibrillation in the
setting of an Anomalous Right Coronary Artery}



\author[1]{Danish Saleh}%
\author[1]{Eric Cantey}%
\author[2]{Emily Marogi}%
\author[1]{Benjamin Freed}%
\author[1]{Bradley Knight}%
\author[1]{Roger de Freitas}%
\author[1]{Ranya Sweis}%
\author[1]{James Flaherty}%
\affil[1]{Northwestern University Feinberg School of Medicine}%
\affil[2]{University of California San Francisco School of Medicine}%


\vspace{-1em}



  
  \date{May 28, 2024}


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\selectlanguage{english}
\begin{abstract}
We present a case of a young male who presented in urosepsis and
developed ventricular fibrillation with cardiac arrest. Work-up revealed
a hemodynamically significant anomalous aortic origin of the
right-coronary artery. Patient underwent revascularization with
percutaneous coronary intervention. Herein, we introduce the case and
decision making in our interventional approach.%
\end{abstract}%



\sloppy


Title: Percutaneous Coronary Intervention for Ventricular Fibrillation
in the setting of an Anomalous Right Coronary Artery

Danish Saleh MD PhD\textsuperscript{a}, Eric P Cantey MD
MS\textsuperscript{a}, Emily P Marogi MD\textsuperscript{b}, Benjamin H
Freed MD\textsuperscript{a}, Bradley P Knight MD\textsuperscript{a},
Roger A de Freitas MD\textsuperscript{a}, Ranya N Sweis MD
MS\textsuperscript{a}, and James D Flaherty MD\textsuperscript{a}

\textsuperscript{a}Department of Medicine, Division of Cardiology,
Northwestern University Feinberg School of Medicine, Chicago
IL.\textsuperscript{b}Department of Medicine, University of California
San Francisco School of Medicine, San Francisco CA.

Keywords/phrases: Anomalous right-coronary artery (aRCA), anomalous
coronary artery, ventricular fibrillation, ischemia, percutaneous
coronary intervention (PCI)

\emph{Words: 1487 (including references and figure legends, excluding
abstract and title page)}

\emph{References 10; Figures 5.}

CONSENT STATEMENT

Written informed consent was obtained from the patient to publish this
report in accordance with the journal's patient consent policy.

Corresponding author:

Danish Saleh MD, PhD Department of Medicine, Division of Cardiology
Feinberg School of Medicine at Northwestern University 676 North St.
Clair Street, Arkes Suite 600, Chicago IL 60611 Office: 312-695-0070
Direct: 832-492-5512 Email: danish.saleh@nm.org

ABSTRACT

We present a case of a young male who presented in urosepsis and
developed ventricular fibrillation with cardiac arrest. Work-up revealed
a hemodynamically significant anomalous aortic origin of the
right-coronary artery. Patient underwent revascularization with
percutaneous coronary intervention. Herein, we introduce the case and
decision making in our interventional approach.

HISTORY OF PRESENTATION

Our patient is a twenty-nine-year-old bedbound male with C4 quadriplegia
and a history of atrial fibrillation, who was found to be critically ill
with a urinary tract infection, prompting hospital admission. On the
fifth day, the patient developed cardiac arrest with ventricular
fibrillation. Return of spontaneous circulation (ROSC) was achieved
after defibrillation. Patient was supported with electrolyte repletion,
anti-arrhythmic therapy, and transvenous pacemaker placement with
rapid-ventricular pacing. Targeted-temperature management was enacted
per protocol.

PHYSICAL EXAMINATION

On admission, cardiovascular exam was without tachycardia and/or obvious
murmurs though the rhythm appeared irregular. Patient had shallow
breaths and poor inspiratory effort. Abdomen was diffusely tender, and
lower extremities contracted.

PAST MEDICAL HISTORY

Medical history is notable for C4 quadriplegia secondary to a
motor-vehicle accident, autonomic-dysfunction, neurogenic-bladder,
decubitus ulcers, and recurrent urinary-tract and bone-infections (L
ischial tuberosity osteomyelitis) requiring intravenous antibiotics.
Patient had atrial fibrillation which was previously managed with
propafenone and metoprolol; patient had discontinued these medications a
month prior to admission.

DIFFERENTIAL DIAGNOSIS

The differential diagnosis for this patient's ventricular fibrillation
included myocardial ischemia, structural heart-disease including
myocardial scar and/or severe pump dysfunction, congenital and acquired
QT-prolonging conditions, severe electrolyte disturbances especially
hypokalemia and/or hypomagnesemia, and hemodynamic or metabolic stress
in the setting of acute illness.

PERTINENT DATA

At the time of arrest, labs were notable for potassium of 3.0 mEq/L with
hemoglobin at baseline (11.7 g/dl). Tele-strips at the time of arrest
demonstrated R-on-T phenomenon with Ventricular Fibrillation (Figure 1).
Admission high-sensitivity troponin was 12 and initial EKG showed atrial
fibrillation alternating with SR and PVCs with a QTc of 431ms (Figure
2a). On admission, two-dimensional transthoracic echocardiography was
notable for mildly decreased left ventricular systolic function, and an
ejection fraction of 50\% with beat-to-beat variation due to frequent
ectopy.

INVESTIGATIONS \& MANAGEMENT

Post-arrest EKG was notable for ventricular quadrigeminy and QTc of
554ms for sinus beats (Figure 2b) without clear evidence of ischemia.
Echocardiogram revealed a depressed ejection-fraction of 21\% in the
immediate post-arrest period which improved to 40\% within one week.
Cardiac MRI demonstrated a moderately reduced ejection-fraction of 40\%
without evidence of myocardial fibrosis. Coronary CT angiogram revealed
an anomalous right-coronary artery (aRCA), emerging from the left-main,
with significant narrowing between the aortic and pulmonary trunks
(Figure 3a, 3b). Calculation of Fractional Flow Reserve (FFR) of the RCA
demonstrated a hemodynamically significant anatomic lesion (FFR = 0.51;
Figure 3c). Coronary angiogram confirmed significant narrowing of aRCA
emerging from the left-main (Figure 4a). Intravascular ultrasound (IVUS)
demonstrated a proximal aRCA with extrinsic compression by aortic and
pulmonary trunks and ellipse-like cross-section with minimal luminal
area of 3.4 mm\textsuperscript{2}. Distally, the aRCA had a luminal area
of 8.7 mm\textsuperscript{2} (Figure 4b, 4c). The patient underwent PCI
to the ostial and proximal RCA with a Xience\textsuperscript{TM} 3.25 x
28mm drug-eluting stent. Post-intervention, IVUS demonstrated
rectification of extrinsic compression and recovery of luminal
cross-section area (7mm\textsuperscript{2}) associating with TIMI 3 flow
on angiogram (Figure 5a, 5b). Post-intervention, the patient was managed
with dual-antiplatelet therapy and anticoagulation (for atrial
fibrillation and PICC-associated thrombus). Prior to discharge, the
patient was transitioned to oral amiodarone and an implantable
cardioverter defibrillator (ICD) was placed for secondary prevention.
Critical illness and possible osteomyelitis was managed with continued
antibiotics.

DISCUSSION

Anomalous aortic origin of the left-coronary artery (AAOLCA) or
right-coronary artery (AAORCA) have independently been linked to sudden
cardiac death in young individuals {[}1, 2, 3{]}. Myocardial ischemia is
thought to occur during periods of stress, such as physical activity or
periods of heightened metabolic demand, as increased myocardial demand
cannot be met due to anatomic obstruction, including vessel compression
by adjacent structures (ie: aorta and pulmonary trunk), acute-angle
take-off, and/or intramural coronary penetration {[}4{]}. Nevertheless,
these lesions are observed infrequently and have only been reported in
0.1-1\% of births {[}5{]}.

The optimal approach to ensure durable myocardial perfusion in the
setting of a clinical and/or a hemodynamically significant anomalous
coronary artery lesion remains unclear. The 2018 AHA/ACC guidelines for
the management of congenital heart disease in adults recommend surgery
(Class I, Level B-NR) for any AAOCA with myocardial ischemia or AAOLCA
without evidence of ischemia (Class IIa/Level C-LD) {[}6{]}. Similarly,
The American Association for Thoracic Surgeons (AATS) recommend surgery
(Class I/Level B) in patients with asymptomatic or symptomatic AAOLCA or
symptomatic AAORCA {[}7{]}. Surgical approaches include coronary
`unroofing,' coronary re-implantation with and/or neo-orifice generation
in the correct coronary sinus, pulmonary artery translocation, and
coronary-artery bypass grafting (CABG). The AATS advises consideration
of PCI for adults with high surgical risk (Class IIb/Level C) {[}7{]}.

In patients with AAORCA, PCI has been shown to be an effective and
low-risk alternative to surgery in limited long-term studies {[}8, 9{]}.
Indeed, PCI-associated remodeling increases coronary lumen area and
features resolution of ischemia {[}10{]}. Historically, surgical
approaches have been preferred to percutaneous approaches in pediatric
patients because of anticipated cardiac/coronary growth and concern for
long-term stent-associated complications and/or durability. Although the
safety and durability of PCI in the pediatric population has yet to be
examined, emerging data supports consideration of PCI in the adults with
AAOCA and high surgical risk {[}7, 8, 9, 10{]}.

FOLLOW UP

There were no further ventricular arrhythmias and/or ICD discharges.

CONCLUSIONS

We suspect that ventricular arrhythmia was the manifestation of ischemia
in the setting of congenital flow-limiting stenosis within the
right-coronary artery. The ventricular fibrillation threshold was likely
lowered by a hypermetabolic state of sepsis, hypotension, and underlying
electrolyte abnormalities (hypokalemia). In this young adult with
quadriplegia who was a poor candidate for post-surgical rehabilitation,
a minimally invasive approach with PCI offered a solution to maintain
coronary patency, mitigate myocardial ischemia, and lower the risk of
recurrent cardiac arrest.

\par\null

Funding: None

Acknowledgements: None

Disclosures/Conflicts of Interest: 1. Dr. Knight has received research
grants from and has served as a consultant for Abbott, Biotronik, Boston
Scientific, and Medtronic. 2. Dr. Sweis is a part of the Speakers'
Bureau with Edwards Lifesciences.

Ethics Statement: We confirm that this manuscript has not been published
elsewhere and is not under consideration by another journal. We have
removed all identifiable information from this case study for academic
and educational purposes. Accordingly, this body of work does not
constitute `research' and Institutional Review Board (IRB) approval was
not required or obtained. All authors have approved the manuscript and
agree with submission to \emph{Clinical Case Reports.}

Author Contributions: D.S. wrote and revised the manuscript and prepared
the figures. E.P.C. aided in manuscript revision and figure preparation.
E.P.M. aided in writing the manuscript. B.H.F, B.P.K, R.A.dF, and R.N.S.
revised the manuscript. J.D.F. wrote, reviewed, and revised the
manuscript.

Data availability statement: Data sharing is not applicable to this
article as no new data were created or analyzed in this study.

REFERENCES

{[}1{]} Angelini, Paul. Coronary Artery Anomalies: An Entity in Search
of Identity. Circulation. March 2007. PMID: 17353457.

{[}2{]} Cheitlin MD, De Castro M, \& McAllister HA. Sudden death as a
complication of anomalous left coronary origin from the anterior sinus
of Valsalva, A not-so-minor congenital anomaly. Circulation. 1974. PMID:
4419670.

{[}3{]} Barth CW 3\textsuperscript{rd} and Roberts WC. Left main
coronary artery originating from the right sinus of Valsalva and
coursing between aorta and pulmonary trunk. JACC. 1986. PMID 3944356.

{[}4{]} Taylor AJ, Rogan KM, \& Virmani R. Sudden cardiac death
associated with isolated congenital coronary artery anomalies. JACC.
1992. PMID: 1512344.

{[}5{]} Brothers JA, Frommelt MA, Jaquiss RDB, Myerburg RJ, Fraser CD
Jr., \& Tweddell JS. Expert consensus guidelines: Anomalous aortic
origin of a coronary artery. The Journal of Thoracic and Cardiovascular
Surgery. June 2017. PMID: 28274557.

{[}6{]} Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS,
Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P,
Landzberg MJ, Saidi A, Valente AM, \& Van Hare GF. 2018 AHA/ACC
guideline for the management of adults with congenital heart disease: a
report of the American college of cardiology/American heart association
task force on clinical practice guidelines. J Am Coll Cardiol. 2019.
PMID: 30121240.

{[}7{]} Angelini P, Walmsley RP, Libreros A, \& Ott DA. Symptomatic
anomalous origination of the left coronary artery from the opposite
sinus of valsalva. Clinical presentations, diagnosis, and surgical
repair. Tex Heart Inst J. 2006. PMID: 16878619.

{[}8{]} Darki A, Motiwala A, Bahos L, Lewis BE, Lopez JJ, Steen LH Jr.,
Mathew V, \& Leya FS. Technical success and long-term outcomes after
anomalous right coronary artery stenting with cardiac computed
tomography angiography correlation. Catheter Cardiovasc Interv. Aug
2020. PMID: 31430026.

{[}9{]} Angelini P, Uribe C, Monge J, Tobis JM, Elayda MA, \& Willerson
JT. Origin of the right coronary artery from the opposite sinus of
valsalva in adults: characterization by intravascular ultrasonography at
baseline and after stent angioplasty. Catheter Cardiovasc Interv. 2015.
PMID: 26178792.

{[}10{]} Aubry P, du Fretay XH, Boudvillain O, Degrell P, \& ANOCOR
Working Group. Place of Angioplasty for Coronary Artery Anomalies with
Interarterial Course. Front. Cardiovasc. Med. Jan, 2021. PMID: 33614737.

FIGURES LEGENDS

Figure 1 A) A 6-lead telemetry rhythm strip showing atrial fibrillation
with natively conducted QRS complexes, aberrantly conducted QRS
complexes, PVCs, and the onset of ventricular fibrillation. B) Telemetry
strip demonstrating ventricular fibrillation.

Figure 2 A) Admission EKG, QTc \textasciitilde{} 431. B) Post-arrest
EKG, QTc \textasciitilde{}554.

Figure 3 A) CT-Coronary Angiogram demonstrating emergence of an aRCA
from the Left Main. B) Dedicated RCA view. C) 3D graphical
reconstruction of coronary anatomy with FFR quantitation of coronary
flow across proximal aRCA stenosis. FFR of RCA \textasciitilde{} 0.51.

Figure 4 A) Pre-intervention coronary angiogram demonstrating aRCA
emerging from the Left Main and region of narrowing. B) Pre-intervention
IVUS of proximal aRCA demonstrating ellipse-like luminal compression
with luminal area of 3.4mm\textsuperscript{2}. Shadowing reflects
anatomy of the aortic and pulmonary trunks. C) Pre-intervention IVUS of
the aRCA distal to proximal narrowing with luminal area of
8.7mm\textsuperscript{2}.

Figure 5 A) Post-intervention coronary angiogram demonstrating Left
Main, Left Coronary Circulation, and aRCA with resolution of proximal
narrowing. B) Post-intervention IVUS of proximal aRCA post PCI with
rectification of luminal integrity and recovery of luminal area
(7mm\textsuperscript{2}). Shadowing reflects anatomy of the aortic and
pulmonary trunks.

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