Results & Discussion
In 2014, the PA was 4.2 cm, in 2016 it was 4.6 cm, and in 2018 it was 4.7 cm, despite the absence of any hemodynamically significant pulmonary stenosis. Transesophageal echocardiogram from 2018 obtained for LAA occlusion device monitoring noted EF 65% with normal biventricular function, a trileaflet aortic valve, and normal Sinus of Valsalva (2.9cm)/ascending aorta (3.1cm). It did not have descriptors of PA pressures/size. Follow-up computed tomography angiography (CTA) reported a bicuspid pulmonic valve without stenosis and PA dilation to 4.7cm (Figures 1, 2).
While the risk of rupture and dissection with PAAs is less than that of aortic aneurysms, it has been reported. 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.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.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.