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\begin{document}

\title{A Rare Case of Supracardiac Total Anomalous Pulmonary Venous Return}



\author[1]{Jianjun Tang}%
\author[1]{Qingdan Hu}%
\author[1]{Jun Yao}%
\author[1]{Lin Hu}%
\author[1]{Shenghua Zhou}%
\author[1]{Mingxian Chen}%
\affil[1]{Second Xiangya Hospital}%


\vspace{-1em}



  
  \date{May 15, 2024}


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\selectlanguage{english}
\begin{abstract}
A 46-year-old female presented with a 3-month history of palpitations.
Chest radiograph presented a `calabash' configuration. Cardiac CT
revealed supracardiac total anomalous pulmonary venous return, whereby
all the pulmonary veins drain into vertical vein and finally to the
superior vena cava. Cardiac catheterisation was consistent with
anomalous pulmonary venous.%
\end{abstract}%



\sloppy


\textbf{Introduction}

Total anomalous pulmonary venous return (TAPVR) is a rare congenital
heart defect. Anatomically, TAPVR caused by the failure of connection
between left atrium and the common pulmonary vein, while resulting in
persisting communication between the pulmonary and systemic veins (1).
The incidence of TAPVR is 0.05 to 0.09 per 1000 live births accounting
for only 1.5\% of children with congenital heart disease. Patients with
TAPVR are usually symptomatic at a very young age, and less than 7\% of
them with surgical correction can survive into adulthood (2). Herein, we
reported a TAPVR adult patient underwent a successful surgical repair.

\textbf{Case presentation}

A 46-year-old woman presented to our hospital with a history of a
palpitations and exertional dyspnea for 3 months. She was incidentally
found to have a heart murmur during cough treatment at local hospital in
December 2019. However, she did not look for further inspection.
Palpitations correlated with atrial flutter on Holter monitoring. She
was referred to our hospital for further cardiac assessment and
management.

Physical examination showed a blood pressure of 105/68 mmHg in left arm,
a heart rate of 106 bpm, and an oxygen saturation of 96\%--98\% under
room air between the upper and lower limbs. Her jugular venous
pulsations were normal. Auscultation revealed that her lungs were clean.
Cardiac examination found a right ventricular heave, but no thrills.
Cardiac auscultation revealed normal heart sounds, with no added sounds.
There was no hepatomegaly, peripheral clubbing, or edema.

\textbf{Investigations}

Chest radiograph showed cardiac enlargement resembles a classic
`calabash' configuration (Figure 1A and 1B). A CT scan further presented
anomalous pulmonary vein (PV) and to check any remaining PV drainage
into the left atrium.

Three-dimensional images further delineated the supracardiac TAPVR in
coronal view (Figure 2A) and sagittal view (Figure 2B). It found several
pulmonary veins draining separately into the collecting vein. The
collecting vein, located above the left atrium, ascended and formed a
dilated vertical vein. The vertical vein drained into the superior vena
cava (SVC) by a dilated left innominate vein was observed. Other CT
findings include no pulmonary venous obstruction, a large atrial septal
defect (ASD), cardiomegaly with the dilated right ventricular forming
the right lateral heart border.

Cardiac catheterisation revealed common drainage of pulmonary veins into
a collecting vein. This vein ascended and formed a left innominate vein
and finally drained into the SVC (Figure 2C and 2D). Haemodynamic data
obtained as follows: aortic pressure 74/48mm Hg, main pulmonary artery
pressure 28/9 (11) mm Hg, right ventricular (RV) pressure 11/4 (7) mm
Hg. The elevated RV pressure with a pressure gradient of 24 mm Hg was
also suggestive of pulmonary stenosis. Oxygen saturation from arterial
blood gas was 96.0\% under room air. The calculated pulmonary vascular
resistance (PVR) was 0.87 wood unit.

The patient was referred for surgical repair. She underwent a successful
surgical correction of TAPVC and ASD close. At procedure, she was
diagnosed with supracardiac TAPVC and found excessively dilated SVC,
innominate vein, and right heart chamber. The pulmonary artery was
larger than the aorta (ratio=2.5:1). The large ASD was measured at 31mm
\selectlanguage{ngerman}× 22mm diameter and the vertical vein was at 22mm × 26 mm diameter. The
LA was connected to the PV confluence through the anastomosis. A patch
material has been used to close the large ASD. Patient was discharged
well on the sixth postoperative day. No palpitations and exertional
dyspnea during the half year fellow-up.

\textbf{Discussion}

TAPVC is a rare congenital heart defect. The incidence rate was 5 to 9
per 100 000 live births. The most common type of TAPVC is supracardiac
TAPVC, accounting for approximately half of all TAPVC patients (Figure
3). Patients with TAPVC are usually symptomatic at a very young age, and
less than 7\% of them with surgical correction can survive into
adulthood. The clinical presentation of TAPVC is mainly determined by
the degree of pulmonary venous drainage obstruction and the magnitude of
the left-to-right shunt (3, 4). Our patient lived with no symptoms until
found at her 46-year-old because of palpitations and exertional dyspnea
caused by atrial flutter.

In this case, this patient was particularly unusual in that she has been
living without symptom until her nearly fifth decade of life without the
need of medication. Two factors, the absence of the pulmonary venous
obstruction and a large ASD, contribute to the long-time survival for
this patient in supracardiac TAPVR. It explains the lack of cyanosis and
normal blood pressure in this patient. Cardiac CT provides accurate
anatomical delineation of pulmonary venous pathways and their
connections (5). According to the CT scan of this patient, no
unobstructed pulmonary venous circulation was observed. Cardiac
catheterization showed no pulmonary over-circulation and a normal
cardiac output. Cardiac CT scan and catheterization showed this patient
had no pulmonary venous obstruction and a large ASD.

The classical `calabash' configuration on the chest radiograph is
uncommonly observed in supracardiac TAPVR. The SVC and right heart
dilation formed right-sided configuration and the dilated innominate
vein mainly consist of this left-sided configuration. There is also
non-specific appearance of chest radiograph in some patients, and then
used as such CT scan and echocardiography for accurate diagnosis of
TAPVR. This patient was firstly reported as classical `calabash'
appearance on the chest radiograph. And was diagnosed by CT scan and
echocardiography. Cardiac CT scan presented venous system enlargement
but aortic system disuse atrophy. Cardiac hemodynamic assessment in
patient showed no pulmonary hypertension and for further surgical
repair.

Surgical repair is immediate need in most cases once the diagnosis of
TAPVC is made. The surgical mortality is less than 5\% when repair is
performed in patients without obstructed pulmonary veins. This patient
successfully underwent TAPVC surgical correction and closed ASD.
Although surgery results in a normal circulation (the pulmonary veins
returning normally to the left atrium), she appeared pulmonary edema
because of aortic disuse atrophy and relatively excessive cardiac
output. Several factors predict the poor outcomes such as pulmonary
venous obstruction before repair, a younger age at repair, pulmonary
vein size and univentricular heart (6). However, the long-term outcome
after surgical repair of TAPVC is also excellent.

\textbf{The authors declare no conflicts of interest.}

\textbf{Informed consent:} Written informed consent was obtained from
this patient.

\textbf{Reference}

\begin{enumerate}
\tightlist
\item
  Verma M, Pandey NN, Kumar S, Saxena A. Beating the odds: a rare case
  of supracardiac total anomalous pulmonary venous return (TAPVR) in an
  adult patient. BMJ Case Rep 2018; 2018: bcr2017221074.
\item
  Wu FM, Emani SM, Landzberg MJ, Valente AM. Rare Case of Undiagnosed
  Supracardiac Total Anomalous Pulmonary Venous Return in an Adult.
  Circulation 2014; 130: 1205--1207.
\item
  Mitsubishi T. Successful repair of total anomalous pulmonary venous
  drainage (supracardiac type): the adult case in Japan (57-year-old
  female). Jpn J Thorac Surg 1986; 39:837.
\item
  Wetzel U, Scholtz W, Bogunovic N, Körfer J, Haas NA, Blanz U, et al.
  Successful correction of a total anomalous venous connection in a
  63-year-old male--case report and review of the literature. Congenit
  Heart Dis 2010; 5: 470--475.
\item
  Vyas HV, Greenberg SB, Krishnamurthy R. MR imaging and CT evaluation
  of congenital pulmonary vein abnormalities in neonates and infants.
  Radiographics 2012; 32: 87--98.
\item
  Karamlou T, Gurofsky R, Al Sukhni E, Coles JG, Williams WG, Caldarone
  CA, et al. Factors associated with mortality and reoperation in 377
  children with total anomalous pulmonary venous connection. Circulation
  2007; 115: 1591--1598.
\end{enumerate}

\textbf{Figure legends:}

\begin{enumerate}
\tightlist
\item
  \textbf{Figure 1.} Chest radiograph (A and B). A huge ``calabash''
  heart presented as appearance according to chest radiograph.
  TAPVR=total anomalous pulmonary venous return.
\item
  \textbf{Figure 2.} A three-dimensional cardiac CT scan (A and B) and
  cardiac catheter angiogram (C and D) further delineated the
  supracardiac TAPVR. Pulmonary veins drained into a collecting vein.
  Later followed by left upper pulmonary vein to form an anomalous
  dilated vertical vein which eventually drains into left innominate
  vein and superior vena cava.
\item
  \textbf{Figure 3.} Images of normal heart and supracardiac TAPVR were
  presented.
\end{enumerate}\selectlanguage{english}
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