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PRENAT CARDIO. 2013 MAR;3(1):22-27
Ewa Góra et. al.
Case Report
ANOMALOUS PULMONARY VENOUS RETURN WITH STENOSIS IN COMPLEX
LEFT HEART DEFECT - LETHAL ANOMALY - 3 CASES REPORT.
Authors:
Ewa Góra1, Hanna Moczulska2,3, Ewelina Litwińska4, Maciej Słodki2,5, Maria Respondek – Liberska2
NZOZ”Sonomedika”, 2 Department for of Diagnosis and Prevention of Congenital Defects, Polish Mother’s Memorial Hospital, Research
Institute in Lodz & Medical University of Lodz (ZDiPWW, ICZMP) 93-338 Łodz, ul. Rzgowska 281/289, 3 Department of Genetic , Medical
Univesity in Lodz, 4 Department of Perinatology and Gynecology , Polish Mother’s Memorial Hospital, Research Institute in Lodz
5
Institute of Health Science of Public High School in Plock
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PRENAT CARDIO 2013 MAR;3(1):22-27
DOI 10.12847/03134
Abstract
In prenatal cardiology center 3 cases of anomalous pulmonary venous return with pulmonary venous stenosis were
diagnosed on the basis of continues Doppler blood flow (V max >100 cm/sec). In each of 3 cases left heart defect
was diagnosed at 29th, 35th and 36th week of gestation. In each case newborn died on the 1st or 2nd day of life before
qualification to cardiac surgery. At present, anomalous pulmonary venous return with pulmonary venous stenosis and
complex heart defect in fetus seems to be lethal cardiac defect.
Key words: anomalous pulmonary venous return, pulmonary venous stenosis, left heart defect, lethal cardiac defect.
INTRODUCTION
was registered by continues Doppler. In subsequent
two examinations these findings were confirmed and
additionally holosystolic tricuspid valve insufficiency was
detected (2 m/s) (Graph.1).
Abnormal pulmonary venous return was first described
as total anomalous pulmonary venous return (TAPVR)
by Willson in 17891. In this cardiac defect pulmonary
At 39 th week of gestation
veins do not enter into left atrium,
there was spontaneous delivery.
but to different parts of systemic
Female newborn’s birth weight
venous tract.
How to cite this article:
was 2900 grams and Apgar was
It leeds to life risk of newborn,
Góra E, Moczulska H, Janiak K,
4/7. Despite prostin infusion and
especially when outlets of
Słodki M, Respondek-Liberska M.
intensive care on the 2nd day of
pulmonary veins are stenotic. In
Anomalous pulmonary venous return with
life the newborn died. Autopsy
literature we can find only few
stenosis in complex left heart defect –
revealed the same diagnosis
case reports of prenatal diagnosis
lethal anomaly – 3 cases report.
as in prenatal examination
of TAPVR but none with venous
Prenat Cardio. 2013 Mar;3(1):22-27
(two chamber heart: common
stenosis. We would like to present
(morphologicaly right) ventricle,
3 cases, based on our own
residual left atrium, total anomalous pulmonary venous
experience of Prenatal Cardiology Center of ICZMP in
return to persistent left superior vena cava, dilatation of
Lodz, indicating that pulmonary vein stenosis in fetus
pulmonary trunk and its branches with widly opened
with left heart defect may suggest lethal cardiac defect.
ductus arteriosus.
Case report 1 (year 1995)
Case report 2 (year 2006)
Gravida 2, para 2 with high risk pregnancy with family
A 36-year old woman, gravida 2 para 1, with high risk
history of mild aortic valve insufficiency (C1 – male
of aneuploidy in threefold test, after amniopunction
without surgical treatment) was admitted to our unit at
with fetuses Robertson translocation - 45, XX,der(14;15)
28th week of gestation. Echocardiography showed left
(q10;q10 ) was admitted to our unit to perform fetal
heart hypoplasia (HLHS) with double outlet right ventricle
echocardiography due to ascites, pericardial effusion and
(DORV) and total anomalous pulmonary venous return
abnormal four chamber view suggesting left heart defect .
to common collector flowing into persistant left superior
At 28 th week of gestation by targeted echocardiography
vena cava (LSVC). Behind left atrium wall abnormal
we confirmed situs solitus, cardiomegaly (HA/CA 0,5),
flow in venous vessel (accelerated to V max 100 cm/s)
pericardial effusion, aortic valve atresia with restriction of
Corresponding author: [email protected]
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Anomalous pulmonary venous return with stenosis in complex left heart defect – lethal anomaly – 3 cases report.
foramen ovale (FOC) and additionally anomalous
pulmonary venous return with collector was detected.
This anomalous confluence formed conglomerate in
pericardium in region of left atrium imitating tumor (Fot.1).
Flow in pulmonary veins was abnormal high up to 100
cm/sec. Systemic peripheral flows (UMB A,V; MCA) were
normal with regular fetal heart rate.
After patient couselling transplacental pharmacological
treatment with Digoxine was undertaken with
echocardiological monitoring. In next 2 weeks not only
there was no improvement in fetal circulation but also
total foramen ovale closure and opening of numerous
ventricular septum defects with right-left flows were
detected (Graph.2).
Pict.1. Schematic drawing of the fetal heart case nr 1
These findings forced us to withdrawal transplacental
pharmacology. At 36th week of gestation hydrothorax and
right pyelectasis were detected. One week later cesarean
section was performed because of premature labour
and female newborn was born with birth weight of 2400
grams and Apgar score of 5. Baby died the same day
because of circulatory and respiratory failure. Autopsy
confirmed prenatal diagnosis: closure of foramen ovale,
excess of ventricular septum and anomalous “receiver”
of pulmonary veins with numerous overgrowth diverticuli
in region of left atrium.
Case report 3 (year 2012)
In 35-year old woman, gravida 2, para 2 (C1- healthy
female), in low risk pregnancy, three ultrasonographical
examinations were performed at 10th, 14th, 19th week
of gestation described as normal. At 34 th week of
gestation patient was referred to our unit for detailed
echocardiography due to abnormal four chamber view.
We described:
In 2D presentation: situs solitus, abnormal axis of
the heart - 91°, normal heart size (HA/HC 0,3, AP 36
mm); four chamber view with 2 symmetrical atria and
2 asymmetrical ventricles – thick, small contracted LV,
dominant RV; FO of 3,2 mm ( small) with left-right blood
flow; interventrical septum without defect; connections A-V
and V-A concordant; mediastinum abnormal: 2 vessels
(PV – 11 mm, SVC – 6 mm); thymus present; wide ductal
arch was present but aortic arch was difficult to assess
with Y sign indicating aortic arch interruption. Descending
aorta was 7 mm; systemic veins flow with wider superior
vena cava (SVC – 6 mm, IVC – 3,4 mm); anomalous
pulmonary venous return with collector deflecting to left
atrium, probably with inflow to SVC.
In 2DD +CD presentation: intracardiac flows: tricuspid
valve insufficiency, normal pulmonary valve flow ; bilateral
blood flow in narrowed (2 mm) pulmonary veins; UMB
A,V flow normal with regular fetal heart rate (140/min).
Pict.2. Schematic drawing of the fetal heart case nr 2
Based on these findings diagnosis was established such
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PRENAT CARDIO. 2013 MAR;3(1):22-27
Ewa Góra et. al.
day he died of circulatory and respiratory failure.
He was excused from autopsy on parents’ request.
Specification of the 3 cases data are presented
in Table 1.
DISCUSSION
The incidence of total anomalous pulmonary
venous return (TAPVR) is 1:17000 life born and
accounts for 1,5 % of all congenital heart defects.
The condition appears in 1% of cases operated
on in pediatric cardiosurgical centres and can be
found in 4% newborns during autopsy. This heart
defect is one of the most difficult anomalies to
detect prenatally what was confirmed in UOG in
2012 where only 8 (1,9%) of 424 TAPVR cases
were diagnosed in fetuses before the delivery2.
We registered 3 cases of TAPVR with obstruction
in our centre (Table 1). All these cases ended
up with death in 1st or 2nd day of newborns life.
We recorded a very special Doppler tracings in
each case with typical sound of turbulent blood
flows. These anomalies are extremely rare (that is
why we presented also an individual year of each
diagnosis) and prenatal diagnosis very essential to
predict early demise of the newborn (in contrast to
similar heart defects without anomalous pulmonary
venous connection, which can be operated on in
2nd – 3rd week after delivery).
Pict.3. Schmatic drawing of the fetal heart case nr 3
a s :
Generally we oblige to Darling’s 3 classification
of total anomalous pulmonary venous return which
hypoplastic left heart (HLHS),
tricuspid insufficiency (TR),
interrupted aortic arch (IAA
type B) and partial anomalous
pulmonary venous return
(PAPVR) (supracardiac)
probably with inflow to SVC
and collector “closing”
FO (Graph 3). Circulatory
system was efficient in fetus,
cardiovascular profile score
was 10.
Cesarean section was
performed at 37th week of
gestation after amniotic
sac rupture and contraction
appearance. Male birth
weight was 2570 g (10th – 25th
percentile range) and Apgar
score 9. After admission to
Intensive Care Unit newborn
was followed a course of
Prostin 0,01 µg/kg/min. Next
Fot.1. 4 ch fetal heart with pericardial effusion
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Anomalous pulmonary venous return with stenosis in complex left heart defect – lethal anomaly – 3 cases report.
Case 1
Year
Age of gravida
High risk pregnancy
Case 2
1995
2008
2012
29
36
35
yes
Week of gestation
during diagnosis
Case 3
yes
28
Type of heart defect
HLHS+ DORV+TR
multipe VSD, pericardial effusion
HLHS + IAA + TR
Type of anomalous
pulmonary venous
return
no
28
34
Aortic valve atresis, FO closure,
HLHS + IAA + TR
total /supracardiac
total/cardiac
partial/supracardiac
V max for PV
80 cm/sec
100 cm/sec
100 cm/sec
Pericardial effusion
no
yes
no
Delivery
SN
CS
CS
Weigh of newborn
Apgar
2900
2400
2570
4/7
5
9
Gender
Decease
2nd day
1st day
2nd day
Table 1. Specification of data – 3 cases with anomalous pulmonary venous return from our unit : Department for Diagnosis and Prevention of Congenital Malformations
(Polish Mother’s Memorial Hospital & Medical University of Lodz)
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PRENAT CARDIO. 2013 MAR;3(1):22-27
divide TAPVR into 4 types, depending on site which
pulmonary veins are connect to: supracardiac, cardiac,
infracardiac or multiple. In supracardiac connection (41%)
pulmonary veins join into common vessel and drain into
vena cava, vena azygos or left cephalobrachial truncus.
In cardiac connection (31%) they drain directly to right
atrium or coronary sinus. The infracardiac connection
(24%) can be through common vessel to the inferior vena
cava, hepatic vein, portal vein, or gastric vein. The mixed
type (4%) is a combination of two or three connections
earlier described.
In our cases vein collector was anomalous and crated an
image of “tumor” behind left atrium (case 2) or alteration
bulging into left atrium with pressure on to atrial septum
(case 3).
In current literature there is little data about total
anomalous pulmonary venous return in fetuses because it
is rare and difficult to detect prenatally. There are indirect
echocardiographic criteria, such as: small left atrium,
right ventricular and right atrial dominance and dilated
pulmonary artery. But these signs can appear also in
typical and common heart defects. We should pick up
an anomalous alterations behind left atrium which can
suggest TAPVR4,5,6,7,8,9. Also dilatation of superior vena
cava can support the diagnosis10 as in the case 3.
Diagnosis of TAPVR using only one ultrasonoghraphic
marker can lead to false positive identification.
Characteristic echocardiographic abnormal the 4
chamber view, dominant RV and abnormal the 3VT view
was observed in all cases with left heart defect (2 x HLHS,
1 x aortic atresia with Fo obliteration).
Our findings confirm Al-Naami’s observation, that there
is common appearance TAPVR with other structural heart
defects, especially left heart defects 9.
One of the theories of left heart anomalies (eg. HLHS,
aortic stenosis) is limited flow through foramen ovale 11.
Primary Fo occlusion may lead to right heart enlargement
and right heart failure, cardiomegaly and fetus oedema
(Case 2 – pericardial effusion, hydrothorax). The probable
reason for fetus survival was due to “opening” of multi
VSDs, which enabled right-left flow replacing closed Fo.
The most important in each case was auscultation of
intracardiac flows by electronic stethoscope – Doppler
and registration of abnormal spectra over 100 cm/sec in
left atrium region. These findings should be differentiated
with reverse blood flow in venous vessels of fetus lungs
observed in Fo restriction or other anomalous resistance
in pulmonary circulation.
Traditionally HLHS, AS, aortic valve atresia are qualified
as critical heart defects or planned to Prostin administration
after delivery in order to maintain permeability of arterial
duct till cardiac surgery is performed 10,12,13.
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Ewa Góra et. al.
But described cases were different. These complex heart
defects despite of echocardiographic supervision and
deliveries performed in main obstetric center, caused quick
hemodynamic deterioration and demises of newborns
before surgery.
Similar natural history/course of pulmonary veins
restriction was described by colleagues from Korea14.
Maybe in future prenatal diagnosis of this specific
cardiac condition can lead to stent insertion into described
pulmonary veins restriction just after delivery.
CONCLUSION
At present prenatal anomalous pulmonary venous return
with stenosis in left heart diseases seems to be a features
of rare lethal cardiac defect.
References:
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heart, Philos. Trans. R. Soc. Lond. 1798, 88,346
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Anomalous pulmonary venous return with stenosis in complex left heart defect – lethal anomaly – 3 cases report.
12. Szymkiewicz-Dangel J. Kardiologia Płodu. Zasady diagnostyki
i terapii. Poznań, 2007
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Łódź
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echocardiography of total anomalous pulmonary venous connection
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. Ultrasound in Obstetrics & Gynecology, 2007, Special Issue: 17th
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Conflict of interest: The author declares no conflict of interest and
did not receive any remuneration
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