Quadricuspid pulmonary valve and left pulmonary artery aneurysm in an asymptomatic patient assessed by cardiovascular MRI

Netherlands Heart Journal, Apr 2011

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.

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Quadricuspid pulmonary valve and left pulmonary artery aneurysm in an asymptomatic patient assessed by cardiovascular MRI

G. J. Nollen 0 J. Kodde 0 A. M. Beek 0 J. C. J. Res 0 A. C. van Rossum 0 0 Present Address: G. J. Nollen Ikazia Hospital , Montessoriweg 1, 3083 AN, Rotterdam, the Netherlands 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 (arrow) congenital abnormalities, as patent ductus arteriosus, atrial or ventricular septal defects or aortic valve abnormalities [1]. 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) [2]. A quadricuspid pulmonary valve is usually clinically quiescent, and significant valvular stenosis or regurgitation rarely occur [3]. Aneurysm of the pulmonary arteries are also rare, found in approximately 1:14,000 autopsies [4]. 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 [5]. 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 [6]. 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 [7]. 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. Open Access This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited.


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G. J. Nollen, J. Kodde, A. M. Beek, J. C. J. Res, A. C. van Rossum. Quadricuspid pulmonary valve and left pulmonary artery aneurysm in an asymptomatic patient assessed by cardiovascular MRI, Netherlands Heart Journal, 2011, 196-198, DOI: 10.1007/s12471-011-0115-3