Three dimensional Spatio Temporal Imaging Correlation (STIC) in
the diagnosis of isolated infra-cardiac total anomalous pulmonary venous
connection (TAPVC) in fetal life
STIC in prenatal TAPVC diagnosis
Mani Ram Krishna and Usha Nandhini Sennaiyan
Tiny Hearts Fetal and Pediatric Cardiac Clinic
Thanjavur
Keywords: TAPVC, STIC, Fetal echocardiogram
Correspondence to
Dr Mani Ram Krishna
Tiny Hearts Fetal and Pediatric Cardiac Clinic
No 7, V.O.C. Nagar,
Thanjavur – 613007
E-mail:
mann_comp@hotmail.com
Conflict of Interest: None
Funding involved in the study: None
Total anomalous pulmonary venous connection (TAPVC) is a critical
congenital heart disease (CHD) and failure to recognise the condition in
the first few days of life can result in significant morbidity and
mortality. However, the standard obstetric screening views of the heart
are often normal in TAPVC resulting in missed prenatal diagnosis(1).
With improvement in resolution of ultrasound imaging and the advent of
advanced imaging modalities including spatio-temporal imaging
correlation (STIC), the condition is increasing recognised on prenatal
ultrasound(2). We report a fetus with infra-cardiac TAPVC in whom STIC
imaging permitted excellent visualisation of the infra-cardiac drainage
as well as the potential site of obstruction.
A 24 year old prime gravida mother was referred to us for a detailed
evaluation after coarctation of aorta was suspected on the anomaly scan.
The gestational age was 26 weeks at the time of assessment. An anomaly
scan had been performed 3 weeks earlier. This reported ventricular
disproportion with a small left ventricle and a suspicion of coarctation
of aorta. The parents were no-consanguineous. Both the parents did not
report any chronic medical illness and there was no family history of
congenital heart diseases. The mother was not on any medications except
iron and frolic acid supplements. The nuchal translucency was reported
to be normal on a 12 week ultrasound and the combined first trimester
screening revealed a low risk for chromosomal aneuploidies.
A detailed fetal evaluation was performed. The imaging was performed on
a Philips Epiq Elite ultrasound machine using a V9-2 volume transducer.
The fetal growth and fetal as well as maternal Doppler evaluation was
normal. There were no important extra-cardiac anomalies with usual
arrangement of the abdominal organs. The cardiac mass was in the left
side of the chest. There was a single right sided superior caval vein
which drained into the right atrium. The inferior caval vein was intact
and drained into the right atrium. The pulmonary venous drainage to the
left atrium (LA) could not be reliably imaged. The atrioventricular
connections were concordant. There was ventricular asymmetry with a
smaller left ventricle (LV) (Fig 1 A). However, the LV formed the apex
of the cardiac mass. The ventriculoarterial connections were concordant.
The outflows were unconstructed. The aortic arch was left sided with
normal branching pattern. There was no posterior shelf or substrate for
coarctation. The pulmonary veins appeared to drain through a descending
vertical vein (Fig 1 B) into the portal venous system suggesting
infra-cardiac total anomalous pulmonary venous connection (TAPVC). Three
dimensional (3D) colour Doppler imaging was obtained by using
spatial-temporal imaging correlation (STIC) and the obtained image was
rendered to demonstrate the pulmonary venous drainage. This showed flow
acceleration at the site of drainage of the vertical vein to the left
portal vein close to the ductus venous suggesting possible obstruction
(Fig 2 and Video 1).
The family were counselled about the probable need for emergency
surgical repair soon after birth and the need to plan delivery in a
pediatric cardiac surgical facility. The family were also counselled
about the excellent long term outcomes after TAPVC repair
TAPVC is a rare CHD with an incidence of approximately 8 per 100,000
live births(3). Neonates with the obstructed form of the disease often
become very symptomatic in the first few days of life. Prenatal
diagnosis probably has the highest impact in TAPVC among all critical
CHD. During prenatal life, only 10% of the combined cardiac output
flows through the pulmonary veins. Hence, the pulmonary veins are small
and often difficult to image. The diagnosis of TAPVC in prenatal
ultrasound was traditionally established by a combination of “soft
findings” including mild ventricular disproportion with a smaller LV,
increased retrocardiac space between the LA and the descending aorta and
the visualisation of an additional vessel on the three chamber view in
cases of supra-cardiac TAPVC(4-6). In a review of 424 children with
TAPVC operated between 1998 and 2004 in all cardiac centres in United
Kingdom, only 1.9% were diagnosed prenatally(7). Ganesan and colleagues
reviewed their experience with prenatal diagnosis in 26 cases of TAPVC
of which only 4 were isolated TAPVC while 22 were associated with right
isomerism. They described the utility of pulse Doppler evaluation of the
pulmonary veins in addition to two dimensional imaging in establishing
the diagnosis(1).
3D fetal echocardiography using STIC has been shown to be superior to
conventional two dimensional ultrasound in the diagnosis of anomalous
pulmonary veins. Zhang and colleagues reported their experience with
prenatal diagnosis of TAPVC. Among 31 fetuses with adequate quality 2D
and 3D imaging in whom the prenatal suspicion of TAPVC was confirmed by
postnatal ultrasound or fetal autopsy, the diagnosis was identified only
on 3D echocardiogram in 5 cases (16%) establishing the additional
utility of STIC imaging(8). Although the diagnosis of TAPVC was
established by 2 ultrasound in our case, STIC allowed us to delineate
the course of the anomalous venous drainage and also identify the site
of potential obstruction.
References
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Legends
Figure 1 A: Four chamber view of the fetal heart demonstrating mild
ventricular disporoportion with the left ventricle smaller than the
right ventricle. The left ventricle, however, still forms the apex of
the heart (LV- left ventricle, RV- right ventricle)
Figure 1 B: Color compare imaging of the fetal thorax and abdomen
demonstrating the descending vertical vein (green arrow) draining into
the abdomen
Figure 2: Still image of 4D STIC imaging demonstrating the drainage of
the vertical vein into the protal system as well as the site of
obstruction of the vertical vein at its draiange into the portal system
(IVC- inferior caval vein)
Video 1: 4D STIC imaging demonstrating the anomalous pulmonary venous
drainage to the portal system. There is flow acceleration at the site of
entry into the portal vein which represent a potential site pf pulmonary
venous obstruction