\documentclass[10pt]{article}

\usepackage{fullpage}
\usepackage{setspace}
\usepackage{parskip}
\usepackage{titlesec}
\usepackage[section]{placeins}
\usepackage{xcolor}
\usepackage{breakcites}
\usepackage{lineno}
\usepackage{hyphenat}





\PassOptionsToPackage{hyphens}{url}
\usepackage[colorlinks = true,
            linkcolor = blue,
            urlcolor  = blue,
            citecolor = blue,
            anchorcolor = blue]{hyperref}
\usepackage{etoolbox}
\makeatletter
\patchcmd\@combinedblfloats{\box\@outputbox}{\unvbox\@outputbox}{}{%
  \errmessage{\noexpand\@combinedblfloats could not be patched}%
}%
\makeatother


\usepackage{natbib}




\renewenvironment{abstract}
  {{\bfseries\noindent{\abstractname}\par\nobreak}\footnotesize}
  {\bigskip}

\titlespacing{\section}{0pt}{*3}{*1}
\titlespacing{\subsection}{0pt}{*2}{*0.5}
\titlespacing{\subsubsection}{0pt}{*1.5}{0pt}


\usepackage{authblk}


\usepackage{graphicx}
\usepackage[space]{grffile}
\usepackage{latexsym}
\usepackage{textcomp}
\usepackage{longtable}
\usepackage{tabulary}
\usepackage{booktabs,array,multirow}
\usepackage{amsfonts,amsmath,amssymb}
\providecommand\citet{\cite}
\providecommand\citep{\cite}
\providecommand\citealt{\cite}
% You can conditionalize code for latexml or normal latex using this.
\newif\iflatexml\latexmlfalse
\providecommand{\tightlist}{\setlength{\itemsep}{0pt}\setlength{\parskip}{0pt}}%

\AtBeginDocument{\DeclareGraphicsExtensions{.pdf,.PDF,.eps,.EPS,.png,.PNG,.tif,.TIF,.jpg,.JPG,.jpeg,.JPEG}}

\usepackage[utf8]{inputenc}
\usepackage[english]{babel}



\usepackage{float}








\begin{document}

\title{Congenital Atresia of the Left Main coronary artery: new presentation in
a septuagenarian}



\author[1]{Shabnam Cyclewala}%
\author[2]{Taimoor Khan}%
\author[3]{Mohammad Salmasi}%
\author[2]{Toufan Bahrami}%
\affil[1]{Barts Health NHS Trust}%
\affil[2]{Harefield Hospital}%
\affil[3]{Imperial College London}%


\vspace{-1em}



  
  \date{May 28, 2024}


\begingroup
\let\center\flushleft
\let\endcenter\endflushleft
\maketitle
\endgroup





\selectlanguage{english}
\begin{abstract}
Congenital anomalous anatomy and course of coronary arteries are common
however atresia of LMS is extremely a rare condition. Fewer than 50
cases of congenital atresia of left main stem coronary artery have been
reported in the literature. The ostium or trunk of the left coronary
artery is atretic, and the left coronary artery and its branches are
supplied by the right coronary artery via coronary collaterals in a
retrograde direction. The pattern of presentation varies depending on
the age and anatomy of the patient. According to the literature these
cases present at a very early age with signs of heart failure whereas
adults may present with angina or shortness of breath. Its clinical
signs and symptoms often resemble anomalous origin of the left coronary
artery from the pulmonary artery and endomyocardial fibrosis. In
literature there are only five adult cases reported with congenital
atresia of LMS and an interesting fact is that one out of those five
cases was diagnosed on autopsy to have atresia of LMS. That patient was
a female at age of 76 years who died of reasons other than heart
symptoms. Therefore it may be inferred that the range of symptoms vary
from being completely asymptomatic to overt heart failure.%
\end{abstract}%



\sloppy


\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image1/image1}

\end{center}
\end{figure}

\textbf{INTRODUCTION}

Variations in coronary anatomy are extremely common, including
alterations in left/right dominance, dual anatomy and recessive vessels.
For the left main stem (LMS), the commonest anomaly is either a short
vessel or absent vessel (i.e. the LAD and circumflex emerge as a
bifurcation at the left coronary ostia).

However, atresia of LMS is an extremely a rare condition: here, the left
system is supplied completely by the right coronary artery (RCA) via
retrograde collateral circulation. According to the literature these
cases present at a very early age with signs of heart failure, and
rarely in adults.

\textbf{CASE REPORT}

We report a case of congenital atresia of LMS in a 75-year-old male,
presenting with acute severe chest pain on exertion, without prior
cardiac symptoms whatsoever. His risk factors included hypertension,
hypercholesterolemia and smoking.

The electrocardiogram demonstrated ST elevation in the anterior leads,
and cardiac enzymes were positive, leading to emergent coronary
angiogram. The latter demonstrated what was initially thought to be
severe LMS stenosis with retrograde filling through right coronary
artery (Figure 1). There were failed attempts to engage the LMS during
aortic root injection. An ectatic right coronary artery was visualised
which filled the the left side retrogradely.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image2/image2}

\end{center}
\end{figure}

\textbf{Fig 1. No filling of the LMS during aortic root injection.}

There was suspicion of a separate narrowing in the collateralizing
vessel and disease in the posterior descending artery. The computerized
tomography (CT) coronary angiogram refuted the initial diagnosis, and
instead confirmed the absence the LMS stump. A gated angiogram and a 3D
complete reconstruction confirmed atresia of the LMS. The most
interesting finding was that the LAD was supported by a conus branch of
the RCA antegradely, while a collateral from the left ventricular branch
of the RCA was feeding the LAD from apex of the heart. There was a tight
narrowing in the antegrade feeding vessel and a calcified lesion at the
junction of collaterals with the LAD.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image3/image3}

\end{center}
\end{figure}

\textbf{Fig 2. Retrograde filling of LAD by RCA injection, through
collaterals.}\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image4/image4}

\end{center}
\end{figure}

.

\textbf{Fig 3. Blind end of Left main coronary artery on CT angiography}

The patient remained completely asymptomatic in the hospital which
raised concerns whether chest pain was due to LMS atresia or one of the
potentially culprit lesions in this complex coronary system. To confirm
LAD territory ischemia, dobutamine stress echocardiography was
conducted, identifying a remarkable decline in contractility of the
anterior wall on stress and dilatation in left ventricular dimensions.

The initial clinical inclination was to offer the patient medical
management, being asymptomatic, and then intervene at a later stage were
symptoms to arise. However, following further investigation with
magnetic resonance imaging (MRI), this identified a considerable loss of
contractility of the anterior wall, septum and apical segments of the
heart with full viability in all segments.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image5/image5}

\end{center}
\end{figure}

\textbf{Fig 4. 3D reconstruction depicting atretic ostium left coronary
artery and tortous left anterior descending}

Following further multi-disciplinary discussion, the patient was offered
off-pump coronary artery bypass grafting.

The patient received a left internal mammary-to-LAD graft and a
saphenous vein graft to the posterior descending artery. The
postoperative recovery of the patient was uneventful and he was
discharged home with complete resolution of his angina symptoms.

\textbf{DISCUSSION}

Only five adult cases of congenital LMS atresia have been reported
previously in the literature, and interestingly, one case was only
diagnosed on autopsy\textsuperscript{1 2}. This handful of adult cases
have confirmed that strong collateralization of the arterial tree let
them skip a turn up at an early age.\textsuperscript{3,~4}The range of
symptoms vary from being completely asymptomatic to overt heart
failure.\textsuperscript{5} Important differential diagnoses also
include origin of the LMS from the pulmonary artery and endomyocardial
fibrosis.\textsuperscript{3,6}

Our case report depicts the chain of investigations which are required
for diagnosis of coronary atresia and determination of the culprit
lesions jeopardizing heart muscle function. Kate et.al emphasized the
limited role of invasive coronary angiography in this important
diagnosis. Cardiac MR angiography and multi-slice computed tomography
(MSCT) gives a 3d visualization and are potentially more powerful
modalities to identify this diagnosis. \textsuperscript{7}

In the present case report, directed investigations played an essential
role in committing to the correct treatment. Whereas gated CT
angiography provided the actual coronary anatomy, stress
echocardiography determined the true origin of ischemia and guided
subsequent therapy. This suggests that, in the case of demonstrable
myocardial compromise, prompt revascularization is the optimal therapy
for patients with LMS atresia.\textsuperscript{1}

\textbf{REFERENCES}

\begin{enumerate}
\tightlist
\item
  A Musiani 1, C Cernigliaro, M Sansa, D Maselli, C De Gasperis. Left
  main coronary artery atresia: literature review and therapeutical
  considerations. Eur J Cardiothorac Surg.~1997 Mar;11(3):505-14.~doi:
  10.1016/s1010-7940(96)01121-9.
\item
  Cortes M., Roldan C.A., Clegg S. Unusual congenital coronary artery
  anomaly in a young adult presenting as sudden cardiac arrest. BMJ Case
  Rep. 2018;24:2018.
\item
  Choudhry S,~Raja MH,~Maadullah M.~A rare congenital cardiovascular
  abnormality presenting as respiratory distress in an infant.~\emph{J
  Pak Med Assoc} ~2012;~\textbf{62} :~969--~971.
\item
  Xianghong Luo MD Zhaojun Li MD Bin Geng MD. Congenital Atresia of the
  Left Main Coronary Artery: Preliminary Diagnosis by Echocardiography.
  2013 https://doi.org/10.7863/ultra.32.11.2045
\item
  Ibrahim A, Power SP, Kelly P. Anomalous left anterior descending
  artery arising from the pulmonary trunk: a rare cause of angina BMJ
  Case Rep. 2017 Sep 7;2017:bcr2017221618. doi:
  10.1136/bcr-2017-221618.PMID: 28882941
\item
  Petersen JA,~Karlo CA,~Alkadhi H,~Gaemperli O,~Biaggi P.~Anomalous
  origin of the right coronary artery from the pulmonary artery: a rare
  finding in an asymptomatic man.~\emph{Eur J Echocardiogr}
  ~2011;~\textbf{12} :~884.
\item
  Ten Kate G.J., Weustink A.C., De Feyter P.J. Coronary artery anomalies
  detected by MSCT-coronary angiography in the adult.~\emph{Netherlands
  Heart J.~} 2008;16:369--375.\textbf{Figure legends}
\end{enumerate}

Fig 1. No filling of the LMS during aortic root injection.

Fig 2. Retrograde filling of LAD by RCA injection, through collaterals.

Fig 3. Blind end of Left main coronary artery on CT angiography

Fig 4. 3D reconstruction depicting atretic ostium left coronary artery
and tortous left anterior descending

\selectlanguage{english}
\FloatBarrier
\end{document}

