Cardiovascular Journal of Africa: Vol 29 No 2 (March/April 2018)

75 / 84

CARDIOVASCULAR JOURNAL OF AFRICA • Volume 29, No 2, March/April 2018 AFRICA e5
Case Report
Partial anomalous pulmonary venous connection with
accessory pulmonary veins
Vimalarani Arulselvam, Neale N Kalis, Suad R Al Amer
Abstract
We present a case of a six-year-old boy with complex partial
anomalous pulmonary venous connections with accessory
pulmonary veins, where multi-detector computed tomogra-
phy proved crucial for accurate identification prior to plan-
ning for surgical correction.
Keywords: partial anomalous pulmonary venous connections,
accessory pulmonary veins
Submitted 13/12/16, accepted 4/4/17
Cardiovasc J Afr 2018; 29: e5–e7
DOI: 10.5830/CVJA-2017-022
Partial anomalous pulmonary venous connection is a rare
congenital cardiovascular condition in which some but not
all of the pulmonary veins drain into the systemic circulation
rather than into the left atrium. Although the pulmonary venous
anatomy can be evaluated by echocardiography and cardiac
catheterisation, non-invasive modalities such as multi-detector
computed tomography and magnetic resonance imaging now
play a crucial role in characterisation of the pulmonary veins.
We report on a case of partial anomalous pulmonary venous
connection of the left superior pulmonary vein with bilateral
accessory pulmonary veins.
Case report
A six-year-old child who underwent aortic coarctation repair
at two years of age was referred to us. He was asymptomatic
and weighed 23 kg, with normal oxygen saturation in room air.
There was no significant limb blood pressure gradient between
the upper and lower limbs. His left radial and brachial pulses
were absent. The cardiovascular examination revealed a grade
2/6 systolic murmur.
Chest X-ray showed mild cardiomegaly. Electrocardiography
revealed right atrial and right ventricular enlargement.
Echocardiography confirmed a dilated right atrium, ventricle
and pulmonary arteries. The estimated right ventricular systolic
pressure was 45 mmHg. There was a 20-mmHg gradient across
the descending aorta. Evaluation of the pulmonary veins showed
two right-sided veins draining normally into the left atrium and
one left-sided pulmonary vein connecting to the vertical vein and
draining into a dilated innominate vein and superior vena cava.
Multi-detector computed tomography (Fig. 1A, B) confirmed
normal drainage of the right upper and lower pulmonary veins,
a small left middle pulmonary vein, and left lower pulmonary
veins draining into the upper poles of the left atrium.
A large right-sided accessory pulmonary vein drained from
the right upper lobe lung. This accessory pulmonary vein was
dilated and had a long superior course to the left side of the
heart before joining the left upper pulmonary vein, which made
a U-turn around the left pulmonary artery. After joining, both
drained superiorly into the innominate vein via a dilated vertical
vein, which drained into the dilated right-sided superior vena
cava (Fig. 2A, B). Furthermore, the lower branch of this right-
sided anomalous accessory pulmonary vein was connected to the
right lower pulmonary vein (Fig. 1A, B).
The patient was scheduled for surgical redirection of the
anomalous pulmonary venous drainage to the left atrium.
Discussion
The typical pattern of four pulmonary veins with well-
differentiated ostia is seen in 60 to 70% of the population.1
Atypical anatomical patterns are found in approximately 38% of
the population,2 hence it is important to be familiar with them.
The prevalence of partial anomalous pulmonary venous
connections is 0.4 to 0.6%.3 Patients with partial anomalous
pulmonary venous connections are often asymptomatic and are
detected incidentally. If the anomaly compromises 50% or more of
the pulmonary venous flow, it may become clinically significant.
Various normal patterns and variations have been described
in studies of pulmonary vein anatomy.1,2 Anatomical variants
on the left side are relatively simple, basically consisting of
convergence of the left pulmonary veins into a common trunk
that drains into the left atrium. Two subtypes of this variant
occur: a short or a long left common trunk. The short left
common trunk is the second most common normal anatomical
pattern, occurring in 15% of the population.
Anatomical variants on the right side are less common and
more complex, with one or more accessory veins that have their
Mohammed bin Khalifa bin Salman Al-Khalifa Cardiac
Centre, Bahrain Defense Forces Hospital, Kingdom of
Bahrain
Vimalarani Arulselvam, MB BS, DNB (Paeds), FNB (Paed
Cardiology)
Neale N Kalis, MB ChB, MMed (Paeds), FCP (Paeds) SA,
Suad R Al Amer, MD, DCh, SSC-P, SF (Paed Cardiology)