Pulmonary Artery Aneurysm Associated with Bicuspid Pulmonary Valve

Abstract

A 73-year-old Japanese man was admitted with an asymptomatic pulmonary artery aneurysm. However, chest X-ray and contrast-enhanced thoracic computed tomography revealed a protrusion at the second left branch that in fact was a pulmonary artery aneurysm with a diameter of 50 mm. Transesophageal echocardiography showed a bicuspid pulmonary valve, and cardiac catheterization revealed pulmonary stenosis with a pressure gradient of 45 mmHg, but no other heart diseases were noted. An extremely rare pulmonary artery aneurysm associated with an isolated bicuspid pulmonary valve was diagnosed.

Pulmonary artery aneurysm associated with a non-stenotic bicuspid pulmonic valve: a role for genetics?

“…2 Indications to intervene on a bicuspid valve if stenotic include a PA systolic pressure gradient >64mmHg and right ventricular dysfunction. 1 Although our patient presented without symptoms that could be directly attributed to a bicuspid pulmonic valve and PAA, symptomatic patients can exhibit exertional dyspnea, weakness, cough, and hemoptysis. 2,3 In their case series of 7 patients with isolated bicuspid disease and PAA, Izumida et al found that over half were asymptomatic with diagnosis made on investigation of other diseases.…”

“…Therefore, care should be taken to monitor those with a >2mm increase in size/year, a PA pressure >50mmHg, and a diameter >7.5cm. 1 PAAs that become large enough undetected are eventually found due to sequelae of compression of nearby structures. 2 Indications to intervene on a bicuspid valve if stenotic include a PA systolic pressure gradient >64mmHg and right ventricular dysfunction.…”

“…2,3 In their case series of 7 patients with isolated bicuspid disease and PAA, Izumida et al found that over half were asymptomatic with diagnosis made on investigation of other diseases. 1 Because the patient did not meet size criteria for operative intervention and because symptomatology was not thought to be direct sequelae of physiologic/mass effect of the PAA, the decision was made to continue surveillance with imaging.…”

“…Bicuspid pulmonic valves are quite uncommon, being described in only 0.1% of donor hearts, while PAAs are even more rare, having been found in 8 out of 109,571 autopsies. 1,[5][6][7] This rarity makes it difficult to characterize relationships. Even on the aortic side, despite the robust association between bicuspid aortic valves and thoracic aortic aneurysms, the mechanism still remains unclear.…”

“…8 Abnormal neural crest cell migration could also be a culprit since neural crest cells are involved in both semilunar valve and vascular wall formation. 1 Previously noted relationships between bicuspid valves and PAAs has been attributed to post-stenotic dilation. 3 However, in our patient there was no significant gradient across the bicuspid pulmonic valve, suggesting that hemodynamics are not the primum mobile of this association and that progression can occur in the absence of significant pulmonic stenosis.…”