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\begin{document}

\title{Ectopia cordis in an adult patient with COVID-19: A case report and
literature review}



\author[1]{Kamal AL SHAMIRI}%
\author[1]{Abdulilah Albriek}%
\author[1]{Tariq Farrag}%
\author[2]{MOSTAFA AL SHAMIRI}%
\affil[1]{King Saud Medical City}%
\affil[2]{King Saud University College of Medicine}%


\vspace{-1em}



  
  \date{May 24, 2024}


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\selectlanguage{english}
\begin{abstract}
Ectopia cordis (EC) is a rare congenital condition characterized by a
partial or complete defect of the anterior chest wall. It is associated
with ventricular and atrial septal defects (ASD),. This study aimed to
explore the cardiac manifestations of EC complicated by coronavirus
disease 2019 (COVID-19).%
\end{abstract}%



\sloppy


Ectopia cordis in an adult patient with COVID-19: A case report and
literature review



Kamal M. Alshamiri\textsuperscript{1}, Abdulilah Z.
Albriek\textsuperscript{1}, Tariq W. Farrag\textsuperscript{1}, Mostafa
Q. Alshamiri\textsuperscript{2}

\textsuperscript{1}Radiology Department, King Saud Medical City, Riyadh,
Saudi Arabia.

\textsuperscript{2}Cardiac Sciences Department, College of Medicine,
King Saud University, Riyadh, Saudi Arabia.

\textbf{Ethics approval and patient consent statements}

The study was conducted at a tertiary care hospital in Saudi Arabia,
with full authorization by the hospital. The patient's relative provided
informed consent for the publication of this case. In the review of
articles, the included studies were cleared with respect to the ethical
handling of patients and related data.

\textbf{Funding statement and conflict of interest disclosure}

The authors have no conflicts of interest to disclosure.

Corresponding author

Mostafa Q. Alshamiri

King saud university medical college

Cardiac sciences department

Po Box 7805, Riyadh 11472

Email mshamiri@hotmail.com

Kingdome of Saudi Arabia

Phone +966504135042

Kamal M. Alshamiri\textsuperscript{1}, Email alshamirikamal@gmail.com

Abdulilah Z. Albriek\textsuperscript{1}, Email Dr.albriek@gmail.com

Tariq W. Farrag , Email ew3atensa1@gmail.com

\textbf{Abstract}

Ectopia cordis (EC) is a rare congenital condition characterized by a
partial or complete defect of the anterior chest wall. It is associated
with ventricular and atrial septal defects (ASD), Ebstein's anomaly,
truncus arteriosus, transposition of the great vessels, tetralogy of
Fallot, and hypoplastic left heart syndrome. This study aimed to explore
the cardiac manifestations of EC complicated by coronavirus disease 2019
(COVID-19). A 23-year-old male, born with EC, was admitted to the
hospital for acute cough and fever. The patient was diagnosed with EC
and ASD by computed tomography and COVID-19 via a polymerase chain
reaction swab test. Patients with ECs rarely survive till adulthood.
However, due to the rarity of this syndrome, upon literature review, we
did not find a case of EC with concurrent COVID-19 infection. The
patient underwent the required investigations and conventional treatment
such as fluid resuscitation, antibiotics administration, and full code
cardiopulmonary resuscitation. The interventions performed were
unsuccessful, and the patient died. This case demonstrates a patient who
lived with EC and its associated cardiac anomalies but died of COVID-19
and its complications despite full resuscitation attempts. Our findings
suggest that patients with EC may survive to adulthood if they have an
incomplete EC, fewer intracardiac defects except for ASD, and an absence
of an omphalocele.

Key clinical message

Ectopia Cordis (EC) is a rare anomaly of unknown etiology rarely
surviving to adulthood. We described EC who died of COVID-19 and its
complications despite full resuscitation. patients with EC may survive
to adulthood if they have an incomplete EC, fewer intracardiac , and an
absence of an omphalocele.

\textbf{Introduction}

During embryonic development, the cartilage bars of the sternum may fail
to develop properly, leading to a sternal cleft. This results in a
condition called ectopia cordis (EC), wherein the heart is outside the
chest wall with either complete or partial pericardial coverage. It is
extremely rare, and most patients do not survive till adulthood.
Furthermore, an association of coronavirus disease 2019 (COVID-19)
infection with this condition has not been reported. However, a
preexisting cardiovascular disease (CVD) or a viral complication on the
heart has been known to increase the risk, mortality, and morbidity of
COVID-19\textsuperscript{1-6}. This study aimed to present the clinical
data of a patient with EC who survived till adulthood and got infected
with COVID-19. The study also reviewed relevant literature to explore
the cardiac manifestations of EC complicated by COVID-19.

\textbf{Methods}

The review of literature was conducted utilizing databases from PubMed,
Science Direct, and Google Scholar to ensure all relevant reports on EC
have been included. Headings, subjects, and medical subject terms, as
well as different combinations of basic search on EC, were used to
search these databases regarding cardiac anomalies and COVID-19.

Data were extracted if they met the inclusion criteria, which include
the following:

\begin{enumerate}
\tightlist
\item
  Published scientific papers that focus on cases of EC
\item
  Systematic studies and medical case reports that are published in
  English
\item
  Associated cardiac anomaly and COVID-19 infections.
\item
  Published data between 1925 and 2020.
\end{enumerate}

All compatible data based on the inclusion criteria were reviewed and
summarized by at least two independent reviewers.

\textbf{Case history, investigations, treatment, outcome, and follow-up}

A 23-year-old male with EC presented to the emergency department
complaining of shortness of breath, fever, and cough of four days
duration. He had a history of contact with a person, who was COVID-19
positive.

Upon examination, the patient was febrile (38\textsuperscript{o}C),
generally ill with sinus tachycardia, central cyanosis, and oxygen
desaturation on room air. Further examination revealed a bulged
pulsatile mass covered with skin. Loud heart sounds and a systolic
murmur were heard over the heart. An examination revealed coarse
inspiratory crepitation all over the chest. The patient had a normal
neurologic condition. The patient's condition deteriorated rapidly which
required intubation and ventilation. Chest radiography revealed
bilateral patchy opacities in both lungs in the background of
questionable pulmonary edema (Figure 1). A bedside echocardiogram showed
atrial septal defect (ASD), right ventricular dilatation, and normal
left ventricular ejection fraction. A polymerase chain reaction (PCR)
swab test was performed in addition to other laboratory workups. CT
pulmonary angiogram was performed as pulmonary embolism was considered.
It revealed no acute pulmonary embolism but found thoracic EC and
secundum ASD (Figure 2). Ground glass opacities with peripheral
distribution in computed tomography lung window image raise the
possibility of underlying viral infection (Figure 3).The patient was
admitted to the intensive care unit by the time the PCR swab tested
positive. Troponin, creatinine phosphokinase, and renal profiles were
increased. Despite full medical care with guidance from a local protocol
for the care of COVID-19 patients, the patient did not respond and
continued to be hypotensive. He still had low oxygen saturation despite
being on a mechanical ventilator. He remained febrile, with a
temperature reaching 41\selectlanguage{ngerman}°C and an increased renal function profile
indicating multi-organs involvement. He had a cardiac arrest requiring
10 minutes of cardiopulmonary resuscitation (CPR) until steady pulse was
retained. He was administered high-dose inotropic support, but 45
minutes later, he had another cardiac arrest requiring an hour of CPR.
The patient was not able to recover and was declared dead.

The causes of death were postulated to be COVID-19 infection associated
with acute renal injury, acute respiratory distress syndrome, and septic
shock.

\textbf{Discussion}

Our case was a patient with a rare cardiac anomaly who acquired COVID-19
from contact with a person who was confirmed to be COVID-19 positive. He
had contact with this person 10 days before presentation to the
emergency department with symptoms compatible with COVID-19. The patient
succumbed within a few hours of the medical encounter with a cascade of
oxygenation and renal function deterioration that ended in shock and
cardiopulmonary arrest. Imaging excluded pulmonary thrombosis to explain
the fulminant hospital course.

EC is a rare congenital defect with an estimated prevalence of 5.5--7.9
per million live births\textsuperscript{7}. The first report of EC was
in 1706\textsuperscript{8}. The cause of EC is unknown, and most cases
are sporadic and usually observed in newborns or
children\textsuperscript{9}. Byron classified EC into four types:
cervical, thoracic, thoracoabdominal, and abdominal\textsuperscript{10}.
Thoracic and thoracoabdominal EC account for about 85\% of the
cases\textsuperscript{11}. Several congenital cardiac anomalies have
been reported to be associated with EC in 82.2\% of
cases\textsuperscript{12}, including ventricular defect and ASD,
Ebstein's anomaly, truncus arteriosus, transposition of the great
vessels, tetralogy of Fallot, and hypoplastic left heart
syndrome\textsuperscript{13}. ASD occurred in 53\% of the
cases\textsuperscript{14}. Our patient was documented to have ASD by CT
angiography.

The heart can often be seen as a pulsating mass through the skin. In a
partial EC, as seen in our patient, the heart is covered by the
pericardium. Meanwhile, in a complete thoracic EC, the heart is
displaced outside the thoracic cavity without a pericardial
cover\textsuperscript{15,16}.

A few patients with pulmonary arterial hypertension and chronic
thromboembolic pulmonary hypertension (CTEPH) suffer from COVID-19. The
case fatality rate of these patients was higher that that in the general
population\textsuperscript{17}. However, in our case, there was no
evidence of CTEPH on CT angiography.

The prognosis of thoracic EC is generally poor, and those who survive
usually had fewer cardiac defects\textsuperscript{18}. The morbidity and
mortality of EC are reduced when there is no concurrent omphalocele,
such as in the case of our patient. An omphalocele is a sac containing
organs, such as the bowel, liver, etc., that remains outside the
abdominal cavity\textsuperscript{19}.

There are no reports in the literature of adults with EC. Our patient
may have survived until adulthood because his EC was only partial, had
fewer intracardiac associated defects except for an ASD, and absence of
an omphalocele.

Without a co-existing COVID-19 infection, the treatment for this
condition would be surgical repair. The first repair for EC was
attempted in 1925 by Cutler and Wilens\textsuperscript{20}. Koop (1975)
reported the first successful repair of thoracic EC\textsuperscript{21}.
In our case, our patient was in a severe condition due to the concurrent
COVID-19 infection. There was no time to manage the patient's cardiac
anomaly.

In conclusion, EC and its associated anomalies are rare. Most patients
do not survive to adulthood. Survival to adulthood is associated with
fewer associated anomalies. However, a co-existing COVID-19 infection
leads to a poor prognosis for any CVD and much more for complex cardiac
anomalies such as EC. This report highlights the fact that EC may
present with a clinical combination of spectral cardiac defects, and
survival to adulthood may depend on the number of associated cardiac
anomalies and having a partial rather than complete EC. The authors hope
that this article sheds light on cases of patients with rare congenital
cardiac anomalies who subsequently get infected with COVID-19. This may
help this special group of patients as we can ensure to provide them
with early treatment and refer them to an advanced care facility to
avoid death.

\emph{\textbf{Acknowledgments}: The} authors acknowledge the support of
the medical imaging administration at King Saud Medical City.

\textbf{References}

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\textbf{Figure legends}

\textbf{Figure 1.} A frontal chest radiograph reveals bilateral patchy
opacities throughout both lungs suggestive of ongoing infectious process

\textbf{Figure 2.} An enhanced computed tomography scan of the chest was
performed to evaluate shortness of breath and chest pain. This shows
partial ectopia cordis with right ventricular outflow tract herniation.
The heart is seen extending through a midline defect in the sternum and
lying outside the thorax, covered only by the skin. Atrial septal defect
is also noted.

\textbf{Figure 3.} Lung window image of enhanced chest computed
tomography scan reveals scattered ground-glass opacities and patchy lung
consolidation with the peripheral distribution. Viral infection was
considered.

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\selectlanguage{english}
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