Review

Proceedings of Singapore Healthcare

Volume 32: 1–7
Treatment considerations in total anomalous © The Author(s) 2023
Article reuse guidelines:
pulmonary venous connection sagepub.com/journals-permissions
DOI: 10.1177/20101058231188865
journals.sagepub.com/home/psh

Eka Prasetya Budi Mulia1,2  and Mahrus A. Rahman3

Abstract
Background: Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic abnormality that accounts for
about 1%–3% of congenital heart disease malformations. TAPVC is a condition in which there is no direct connection between
all four pulmonary veins and the left atrium but makes abnormal connections to the right atrium or systemic venous system.
TAPVC caused a high mortality rate of 80% in the first year of life, and 50% of them die within 3 months after birth without
intervention.
Objectives: The aim of this review is to elucidate the various treatment considerations of TAPVC.
Methods: A literature search was conducted on PubMed, ScienceDirect and Google Scholar using various combinations of
keywords related to treatment of TAPVC. The citations from all selected articles were reviewed for additional studies.
Results and Conclusion: TAPVC intervention, including medical and surgical, is tailored to each type of TAPVC. Catheter-
based interventions are frequently used to temporize neonates and provide time to optimize patients medically prior to
definite repair. Corrective surgery is required for all patients with this condition. Several latest catheter-based or surgical
intervention technique modifications have also been reported.

Keywords
Congenital heart disease, cyanotic, pulmonary vein, surgical, total anomalous pulmonary venous connection

Introduction we narratively review the current therapeutic consider-

Total anomalous pulmonary venous connection (TAPVC),
also referred to as total anomalous pulmonary venous
return/drainage (TAPVR/TAPVD), is a rare but hetero-
geneous anomaly, accounting for 1% to 3% of cases of
congenital heart disease.1,2 This anomaly is characterized
by the failure of confluent pulmonary veins (PV) to be
absorbed into the dorsal part of the left atrium (LA) and in
combination with a persistent connection to the systemic
venous system. Historically, TAPVC caused a high mor-
tality rate of 80% in the first year of life, and 50% of them
die within 3 months after birth without intervention. 3
Advances in surgical technique, increased diagnostic ac-
curacy, and changes in perioperative management have
contributed to the reduction in perioperative mortality,
although several factors such as surgical repair of the
neonate, preoperative pulmonary venous obstruction
(PVO), mixed anatomic variations, single ventricular
physiology, and heterotaxy remain important risk factors
for poor postoperative survival.1,4 In this literature review,
ations, including postoperative follow-up of TAPVC.
Anatomy and classification
The classification of the most widely used type of TAPVC is
based on the location of the PV fusion, which was first
1
Department of Cardiology and Vascular Medicine, Faculty of Medicine,
Universitas Airlangga - Dr Soetomo General Hospital, Surabaya, Indonesia
2
Department of Cardiology and Vascular Medicine, Dr R. Soetrasno General
Hospital, Rembang, Indonesia
3
Pediatric Cardiology Division, Department of Child Health, Faculty of
Medicine, Universitas Airlangga - Dr Soetomo General Hospital, Surabaya,
Indonesia
Corresponding Author:
Eka Prasetya Budi Mulia, Department of Cardiology and Vascular Medicine,
Faculty of Medicine, Universitas Airlangga-Dr. Soetomo General Hospital,
Surabaya, Indonesia Jalan Mayjen Prof. Dr Moestopo No.6-8, Surabaya
60286, Indonesia.
Email: eka.prasetya.budi-2017@fk.unair.ac.id

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2 Proceedings of Singapore Healthcare

introduced by Darling et al. in 1957. TAPVC is divided into
four types: (1) Supracardiac, (2) Cardiac, (3) Infracardiac,
and (4) Mixed type TAPVC (Figure 1).5–7
(1) Supracardiac TAPVC (type 1)
The prevalence of supracardiac TAPVC is 45%–55%.
This type of TAPVC occurs when the common pulmonary
venous drainage leads to a superior part of the heart, namely
the innominate vein, superior vena cava (SVC), or azygous
vein via the vertical vein. Although obstruction is not fre-
quent, obstruction can also occur due to compression of the
vertical vein by the left main bronchus and left pulmonary
artery (PA) or narrowing of the vertical venous base towards
the innominate vein.6,8
(2) Cardiac TAPVC (type 2)
Cardiac TAPVC occurs in approximately 20%–30%
of cases. Pulmonary venous drainage into the coronary
sinus or, in some cases, directly to the right atrium
(RA); therefore, the coronary sinus is often dilated.
Cardiac obstruction is not usually found; however, it can
occur in the common PV or ostium of the coronary sinus
or PVs.7–9
(3) Infracardiac TAPVC (type 3)
Infracardiac TAPVC occurs in approximately 25% of
cases. Pulmonary venous drainage goes to the descending
vertical vein anterior to the esophagus through the diaphragm
(esophageal hiatus) to the portal vein or ductus venosus.6,7
This picture of pulmonary venous drainage is often called the
“inverted Christmas tree”, where the PVs exit the pulmonary
hilus in an oblique direction like tree branches. This type is
often accompanied by obstruction at the level of the dia-
phragm or ductus venosus. Therefore, patients with this type
are susceptible to hypoxemia and pulmonary hypertension
(PH) pre and postoperatively.8,9
(4) Mixed type TAPVC (type 4)
Mixed type TAPVC occurs in approximately 5% of cases.
Abnormalities of this type may include a combination of
components from the previous type. The right lung and left
lung have different venous drainage from one another.8,9 The

Figure 1. TAPVC anatomical classification. (a) Supracardiac TAPVC. RPV and LPV form a horizontal pulmonary venous confluence that
connects to the LIV through a vertical vein; (b) Cardiac TAPVC. pulmonary venous confluence connects to CS; (c) Infracardiac TAPVC.
Pulmonary veins form a vertical confluence that descends below the diaphragm and joins the portal vein; (d) Mixed type TAPVC. In this
example, LPV connect to LIV, and RPV to CS. CS: coronary sinus; HV: hepatic veins; LIV: left innominate vein; LPV: left pulmonary veins; PV:
portal vein; RPV: right pulmonary veins; SV: splenic vein; SMV: superior mesenteric vein. (from: John Wiley and Sons, Echocardiography in
Pediatric and Congenital Heart Disease: From Fetus to Adult, 3rd Edition by Lai et al., 2021).
Mulia and Rahman
mixed variant is often associated with other major structural
heart defects and can have obstructions at multiple levels.
The most common mixed connections are the left PV con-
nection to the vertical vein leading to the left innominate vein
and the right PV connection to the RA or coronary sinus.7
Management
Medicals
Intensive diuretics should be given to prevent pulmonary
volume overload conditions, especially in TAPVC without
PVO. Infants with severe pulmonary edema (due to in-
fracardiac type or other types with obstruction) should be
intubated, sedated, or even paralyzed if necessary before
emergency surgery. Metabolic acidosis, a fairly frequent
complication, must be corrected immediately. In cases of
TAPVC with PH, as in the infracardiac type, prostaglandins
(PGE1) can also be given to keep the ductus open and
maintain adequate systemic flow. However, a large patent
ductus can also increase the degree of cyanosis.6,10
Obstructive TAPVC may present with PH originating
from the PVs (post-capillary). Administration of inhaled
nitric oxide (iNO) in this condition is not recommended.11
Sudden pulmonary vasodilation with potent pulmonary va-
sodilators tends to be ineffective, non-physiologic, and may
cause sudden and severe worsening.11,12 However, iNO has a
vital role in postoperative care because PH can persist even if
the obstruction is resolved postoperatively.13–15Significant
reductions in pulmonary vascular resistance (PVR) and PA
pressure can be seen when iNO is administered
postoperatively.16
Catheter-based interventions
Catheter-based interventions are frequently used to temporize
neonates and provide time to optimize patients medically
prior to definite repair.17 Balloon or blade atrial septostomy
(BAS) is performed if the interatrial defect is small (re-
strictive), while surgery cannot be performed immediately.
However, this procedure is not very useful if the venous
anomaly is obstructed.6,10
Percutaneous PV stent placement in vertical vein ob-
struction in supracardiac and infracardiac TAPVC were most
Figure 2. Percutaneous intervention using coronary stent via a guiding catheter demonstrating successful dilation of the obstructed vertical
vein in supracardiac TAPVC. (from: John Wiley and Sons, Catheterization and Cardiovascular Interventions, Stenting of the vertical vein in
obstructed total anomalous pulmonary venous return as rescue procedure in a neonate, Lo-A-Njoe et al., 2006; 67(5): 668-670, https://doi.
org/10.1002/ccd.20715).
3
commonly reported (Figure 2).18–22 Repeated angioplasty for
in-stent restenosis has also been reported.23 Case report of
stent placement between the pulmonary confluence and the
LA as a bridge to surgery has also been described.24
Chamberlain et al. reported successful palliation in prema-
ture and low birth weight infants by serial catheter-based
interventions as a viable management option to allow them to
achieve an acceptable weight and maturity for surgical re-
pair.25 Sometimes, the vertical vein was deliberately not
operated on in order to prevent a PH crisis, with embolization
with a coil or plug to be performed if the right heart volume
load increased.26–28
Extracorporeal membrane oxygenation (ECMO) is an-
other intervention used to temporize neonates until the di-
agnosis is confirmed or when medical management is unable
to correct severe hypoxemia, acidosis, and hemodynamic
instability; and interventions or surgical repair are
arranged.22,29,30 ECMO has allowed surgical correction even
in the most severely ill patients. Postoperatively, ECMO can
also be useful for those with residual PH or aid in cardiac
recovery.17
Surgical
Indication and Timing. Corrective surgery is required for all
patients with this condition. There is no palliative procedure.
All infants with PVobstruction should be operated on as soon
as possible after diagnosis. Infants who do not have PVO but
have uncontrolled heart failure are usually operated on when
stable on a semi-elective basis. TAPVC without obstruction
or severe heart failure can be corrected at neonates or delayed
until 3–6 months of age.6,17
Procedure and technique. Although procedures vary with the
location of the anomalous drainage, all procedures are
intended to redirect the PVs to the LA (Figure 3). The
principle of surgery is to provide a connection from PV to
the LA, interrupt the connection with the systemic venous
system, close the interatrial defect, and other specific
actions depending on the type of TAPVC anomaly.6,8,17
The surgical technique varies from surgeon to surgeon;
some use the RA approach to reach the LA, and others
directly reach the LA posterior wall. Several support the
use of deep hypothermic circulatory arrest (18°-20°C).6
4 Proceedings of Singapore Healthcare

Recently, a sutureless technique has been increasingly
used to prevent postoperative PV stenosis/obstruction
(Figure 4).17,31 A meta-analysis by Wu et al. demon-
strated that a lower occurrence rate of postoperative PVO
and reoperation due to PVO were associated with su-
tureless techniques than conventional surgery. Meanwhile,
hospitalization time and postoperative mortality were not
statistically different between the two surgical
approaches.32
In supracardiac type, a side-to-side anastomosis is per-
formed between the common pulmonary venous sinus and
the LA. Vertical vein ligation and ASD closure were per-
formed using a cloth patch (Figure 3(A)).6,17
Repair of cardiac type TAPVC to the RA involves ex-
cision of the atrial septum, where the PV opening is then
closed using a patch. In addition, the PVs drain all the way to
the LA (Figure 3(B)). ASD may have to be enlarged. While in
cardiac type TAPVC to the coronary sinus, an incision is
made in the anterior wall of the coronary sinus (unroofing) to
make a connection between the coronary sinus and the LA.
Subsequently, the ASD and coronary sinus ostium were
closed using a single patch (Figure 3(C)). This procedure
results in drainage of coronary sinus blood with low oxygen
saturation into LA.6,17,33
In infracardiac type, a large vertical anastomose was
performed between the common pulmonary venous sinus and
the LA. Afterward, the common PV leading to the abdominal
cavity was ligated at a level above the diaphragm
(Figure 3(D)).6,17
At the time of TAPVC repair, the surgeon must decide
whether the vertical vein should be ligated. The unligated
vertical vein can act as an import-decompression conduit for
the non-compliance small LV and can help minimize the
consequences of a postoperative PH crisis. An unligated
vertical vein can lead to a significant left-right shunt, al-
though in most cases, the hemodynamic consequences may
be minor,34,35 and most will close spontaneously on their
own.17
A novel surgical technique was successfully described by
Mehta et al. in a rarely combined supracardiac and in-
fracardiac drainage of TAPVC (mixed type) without a
common pulmonary venous chamber, in which a common
pulmonary venous chamber was created, and anastomosis of
this chamber to the LA was combined with interruption of

Figure 3. Surgical approaches to various types of TAPVC. (a) Supracardiac type; (b) Cardiac type to right atrium; (c) Cardiac type to
coronary sinus; (d) Infracardiac type. (from: Elsevier Science & Technology Journals, Park’s Pediatric Cardiology for Practitioners, 7th
Edition by Park and Salamat, 2021).

Figure 4. Surgical repair for TAPVC. Conventional repair (pulmonary vein insert with potential suture line stenosis) and sutureless repair
(pulmonary vein insert) techniques are shown. (from: Elsevier, The Journal of Thoracic and Cardiovascular Surgery, Primary sutureless
repair for “simple” total anomalous pulmonary venous connection: Midterm results in a single institution, Yanagawa et al., 2011; 141(6):
1346-1354, https://doi.org/10.1016/j.jtcvs.2010.10.056).
Mulia and Rahman
both the ascending and descending anomalous connections.36
Another palliative technique in low birth weight newborns
suffering from critical obstructive supracardiac TAPVC by
establishing anastomosis without cardiopulmonary bypass
(CPB) between pulmonary venous confluence and LA ap-
pendage (Sarmast – Takriti Shunt) was successfully
reported.37
Postoperative mortality. The surgery mortality rate is between
5% and 10% for infants without PVO and will be higher by
20% in the infracardiac type with obstruction. The most
common causes of postoperative mortality are PH and PV
stenosis due to its closure. Seale et al. reported a 3-year
survival of 85%, with independent risk factors for death
consisting of early age at surgery, hypoplastic/stenotic PVs,
complex cardiac lesions, postoperative PH, and postoperative
PVO. Mortality from PV stenosis alone reached 40%, where
the risk factors for postoperative PV stenosis include the
degree of preoperative morphological abnormalities and the
absence of common confluence in the PV structure.6,10,38,39
Xiang et al. reported that the survival rates of repaired mixed
type at 3 and 5 years were 90.9% ± 8.7%.40 The anatomical
type of TAPVC is less correlated with surgical outcome, but
supracardiac TAPVC has a better outcome than other
types.1,41
Surgical complications
Several surgical complications include paroxysmal PH,
which is associated with a small left heart and poor com-
pliance, with consequent heart failure and pulmonary edema,
which may require prolonged postoperative respiratory
support.6 Postoperative arrhythmias are usually atrial ar-
rhythmias, including sick sinus syndrome/sinus node
dysfunction.42,43 Obstruction at the anastomotic site or PV
stenosis is rare, ranging from 10%–15%.1,6,38,44 Reoperation
is required in approximately 10 to 15% of patients with
isolated TAPVC with individual PV stenosis, or surgical
anastomosis is the main reason for reoperation.13,38,45–47 In
addition, neurodevelopmental delay and growth retardation
may also occur post-surgery.48
Post-surgery follow-up. Evaluation at 1 month and every 6 to
12 months are recommended for long-term (late) surgical
complications such as PVO and atrial arrhythmias. PVO at
the site of the anastomosis or the appearance of delayed PV
stenosis may occur in about 10% of patients and require
reoperation. These complications are usually seen within 6 to
12 months after improvement.17,38 The possibility of PV
stenosis requires cardiac catheterization and angiocardiog-
raphy. Follow-up was continued for 5 years if there were no
residual defects and PH.49 Some patients may have atrial
arrhythmias, including sick sinus syndrome, which require
medical treatment or pacemaker therapy. Periodic arrhythmia
screening is not routinely recommended in asymptomatic
children with repaired TAPVC. Periodic Holter may be
considered in asymptomatic adolescents. Activity restriction
is usually not necessary unless PV obstruction or PH de-
velops. Prophylactic measures of infective endocarditis
should be considered in the first 6 months after surgery, after
which it is no longer necessary. Children with TAPVC should
5
undergo appropriate surveillance, screening, and/or referral
for neurodevelopmental disorders.6,49,50
Conclusion
TAPVC is a rare and severe cyanotic congenital heart disease
with a prevalence of 1%–3% of all congenital heart diseases.
The universal TAPVC classification is divided into supra-
cardiac, cardiac, intracardiac, and mixed type TAPVC.
TAPVC management in medical, catheter-based, and surgical
procedures should be tailored for each type of TAPVC, taking
into account the patient’s clinical condition, including the
current intervention procedure.
Author contributions
EPBM contributed to conceptualization, validation, investigation,
writing and preparation of the original draft, and visualization. MAR
contributed to conceptualization, validation, resources, writing,
reviewing, and editing of the manuscript, visualization, and
supervision.
Declaration of conflicting interests
The author(s) declared no potential conflicts of interest with respect
to the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, au-
thorship, and/or publication of this article.
ORCID iD
Eka Prasetya Budi Mulia  https://orcid.org/0000-0002-2681-7743
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