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\begin{document}

\title{A Symphony of Anomalies: Isolated Pulmonary Artery Agenesis Meets
Congenital Lung Hypoplasia}



\author[1]{Nida Ansari}%
\author[2]{Sacide S. Ozgur}%
\author[3]{Alan Alcantara}%
\author[3]{Yasmeen Sultana}%
\affil[1]{Saint Joseph's Healthcare System}%
\affil[2]{St Joseph's University Medical Center}%
\affil[3]{St Josephs Regional Medical Center}%


\vspace{-1em}



  
  \date{June 10, 2024}


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\textbf{A Symphony of Anomalies: Isolated Pulmonary Artery Agenesis
Meets Congenital Lung Hypoplasia}

Nida Ansari, D.O.\textsuperscript{1}, Sacide S. Ozgur,
M.D.\textsuperscript{1}, Alan Alcantara, M.D.\textsuperscript{1},
Yasmeen Sultana, M.D.\textsuperscript{1}

\emph{Disclosure of Interest:} The authors report no conflict of
interest. An ethical review is not necessary because this is a case
report. This research received no specific grant from funding agencies
in the public, commercial, or not-for-profit sectors.

\emph{Consent:} As this is a case report, consent was obtained for the
purpose of this paper.

\emph{Acknowledgments:} We would like to acknowledge the patient and his
family for allowing us to share this case with our colleagues.

\emph{Author affiliation:} Ansari, N, Ozgur, S, Alcantara, A, performed
the literature review and wrote the manuscript, and all authors
contributed to the writing, the final editing, and the collection of the
patient's clinical data. All work was performed at St. Joseph's
University Medical Center at the following address:

St. Joseph's University Medical Center Department of Internal Medicine
703 Main Street Paterson, NJ USA 07503 973-754-2000

All authors, including the corresponding author, may be reached using
the aforementioned contact information.

\emph{Emails:}

Nida Ansari: nidaansari.do@gmail.com

Sacide S. Ozgur: r\_ozgurs@sjhmc.org

Alan Alcantara: dr.alcantara3@gmail.com

Yasmeen Sultana: sultanay@sjhmc.org

\emph{Keywords}\textbf{: Pulmonary Hypoplasia, Pulmonary Artery
Agenesis, Congenital, Absent Pulmonary Artery}

\textbf{Key Clinical Message:}

Isolated agenesis of pulmonary arteries with congenital lung hypoplasia
is rare. It can be found in childhood or adulthood if asymptomatic. We
present a patient with congenital right lung hyperplasia with an absent
right pulmonary artery.

\textbf{Case Presentation:}

A 24-year-old Female with a past medical history of intermittent asthma,
right lung hypoplasia, and dextrocardia presented to the Emergency
Department for evaluation of shortness of breath. Upon evaluation via
imaging, she was noted on CT Angiogram (Figures A,B \&C) to have
congenital hypoplasia of the right lung, dextrocardia, and absent right
pulmonary artery and its branches.\selectlanguage{english}
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\textbf{Figure A} : Coronal View of Computer Tomography Angiography of
the Chest demonstrating Absent Pulmonary Artery, Pulmonary Hypoplasia
and Dextrocardia.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image2/image2}

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\end{figure}

\textbf{Figure B\&C:} Axial Views of Computer Tomography Angiography of
the Chest demonstrating Absent Pulmonary Artery, Pulmonary Hypoplasia
and Dextrocardia.

\textbf{Discussion:}

Congenital, isolated agenesis of pulmonary arteries (UAPA) is a
developmental malformation that shows a unilateral absence of the
pulmonary artery without the presence of a congenital heart disease
{[}1{]}. This often leads to pulmonary hypoplasia of the ipsilateral
lung {[}1{]}. UAPA is often classified as congenital or acquired
{[}1{]}. Congenital is defined as a developmental defect or malformation
during embryogenesis {[}1,2{]}. Acquired due to chronic subclinical
infection, typically secondary to tuberculosis, that involves the hilum
of the lung {[}1{]}. UAPA has a bimodal distribution in regard to age of
presentation {[}1{]}. Those who are present in childhood are often
symptomatic as a result of congestive heart failure or pulmonary
hypertension {[}1{]}. Those who present as an adult are often
asymptomatic, typically due to collateral vasculature to the affected
lung {[}1,2{]}. While there are many modalities that can be used for
diagnosis, CT or MR pulmonary angiography is considered the gold
standard {[}1{]}.

\textbf{References:}

Jariwala P, Maturu VN, Christopher J, Jadhav KP. Congenital isolated
unilateral agenesis of pulmonary arteries in adults: Case series and
review. \emph{Indian J Thorac Cardiovasc Surg} . 2021;37(Suppl
1):144-154. doi:10.1007/s12055-020-01032-w.

Cherian SV, Kumar A, Ocazionez D, Estrada-Y-Martin RM, Restrepo CS.
Developmental lung anomalies in adults: A pictorial review.\emph{Respir
Med} . 2019;155:86-96. doi:10.1016/j.rmed.2019.07.011.

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