Quadricuspid pulmonary valve and left pulmonary artery aneurysm in an asymptomatic patient assessed by cardiovascular MRI
G. J. Nollen
A. M. Beek
J. C. J. Res
A. C. van Rossum
Present Address: G. J. Nollen Ikazia Hospital
, Montessoriweg 1, 3083 AN, Rotterdam,
We present a coincidental finding of quadricuspid pulmonary valve and left pulmonary artery aneurysm. As both the pulmonary valve and the pulmonary trunk with its main branches are hard to visualise with cardiac ultrasound, most abnormalities described so far are from autopsy series. With the increasing use of CMR and its excellent potential for visualising both pulmonary valve and pulmonary arteries, we believe more cases will be discovered in the near future. Although pulmonary artery aneurysm are rare, timely detection may prevent lethal bleeding.
Fig. 1 Chest radiography in the anteroposterior view showing a
dilated left pulmonary artery
Fig. 2 Cardiac magnetic resonance imaging with balanced
steadystate free precession sequence showing a dilated left pulmonary artery
(asterisk) in axial a, coronal b, and oblique sagittal c view
Fig. 3 Cardiac magnetic resonance imaging with balanced
steadystate free precession sequence showing quadricuspid pulmonary valve
congenital abnormalities, as patent ductus arteriosus, atrial
or ventricular septal defects or aortic valve abnormalities
. Quadricuspid pulmonary valves predominate in males
and are more frequently found than quadricuspid aortic
valves (with a ratio of 2:1, and 9:1, respectively) . A
quadricuspid pulmonary valve is usually clinically
quiescent, and significant valvular stenosis or regurgitation rarely
Aneurysm of the pulmonary arteries are also rare, found
in approximately 1:14,000 autopsies . Of these
aneurysms 80% are located in the main pulmonary artery. Gender
distribution is equal. Congenital structural heart disease
(patent ductus arteriosus, atrial or ventricular septal defects,
pulmonary valve stenosis) accounts for 50% of the cases of
pulmonary artery aneurysm . Other causes are infections
(tuberculosis, mycotic infections or syphilis), connective
tissue disorders (Marfan, Ehlers-Danlos), Behcets
syndrome, pulmonary hypertension, atherosclerosis or trauma.
In a minority of cases, no cause is identified. Local
compression on adjacent structures may produce symptoms
of thoracic pain, dyspnoea or cough. Rupture of the
aneurysm may lead to (fatal) haemoptysis. Management
of pulmonary artery aneurysm remains controversial. Early
surgery has been suggested in case of symptoms or large
aneurysms, especially mycotic aneurysms, which tend to
rupture earlier. In idiopathic aneurysm a more conservative
strategy has been advocated, as these have a lower tendency
to rupture .
In our patient no possible causes for the aneurysm and
no other congenital abnormalities were found, and it was
considered idiopathic. A follow-up CMR will be performed
in 6 months. To our best knowledge, only one case of
quadricuspid pulmonary valve in combination with an
idiopathic pulmonary artery aneurysm has been described
previously . In that case the aneurysm was located in the
main pulmonary artery.
As both the pulmonary valve and the pulmonary trunk with
its main branches are hard to visualise with cardiac ultrasound,
most abnormalities described so far are from autopsy series.
With the increasing use of CMR and its excellent potential of
visualising both the pulmonary valve and pulmonary arteries,
we believe more cases will be discovered in the near future.
Although pulmonary artery aneurysms are rare, timely
detection may prevent lethal bleeding.
Informed consent was obtained from the patient for
publication of this case report and accompanying images.
A copy of the written consent is available for review by the
editor in chief of this journal.
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