Page 25 - Journal of Structural Heart Disease Volume 4, Issue 3

Original Scienti c Article 82 Table 1. Baseline and follow-up imaging characteristics of 11 pulmonary artery aneurysms (PAA) patients. Mean follow-up duration
is 10 years.
1 M
2 M
3 M
4 F
5 F
6 F
7 F
8 F
9 F
10 F
11 F
65 3.7 30 5 77 4.5 53 3.8 52 4.8 80 7.3 71 3.1 25 2.8 69 5.9 70 4 43 4.6
3.8 12 5 5 5 15 3.8 1 5.3 18 7.5 2 4.3 11 3.9 14 6.5 13 5.4 16 4.7 5
.01 cm/year 0 cm/year .03 cm/year 0 cm/year .03 cm/year 0.1 cm/year 0.1 cm/year 0.08 cm/year 0.05 cm/year 0.09 cm/year 0.02 cm/year
Patient
Gender
Age (years)
Initial PAA size (cm)
PAA size at latest follow-up (cm)
Follow-up dura- tion (years)
Rate of PAA growth (cm/year)
greater than mild pulmonary valve stenosis or regurgi- tation and any of the secondary causes of pulmonary artery aneurysm were excluded. All patients had mild pulmonary valve stenosis and 9/11 had mild pulmo- nary regurgitation. Eight of the eleven patients were female, mean age 57 (range 25-80) (Table 1). Eight of the eleven (73%) were above 50 years of age. Five pa- tients(46%)hadPAA>4cmandallwere≥50years old. PAA size increased at a mean rate of 0.5 cm over a mean follow-up of 10 years, and an increase in PAA size was noted in nine patients. PAA size increased at <0.1 cm/year in all patients (Figure 3). For the assess- ment of PAA size, echocardiography demonstrated a signi cant correlation to CT/cMRI (r=0.93, p<0.001). There were no instances of pulmonary artery dissec- tion or rupture despite 6/11 patients with PAA > 5 cm. Additionally, there were no instances of coronary ar- tery compression and no evidence of progressive pul- monary valvular abnormalities over 10 years.
The mean systolic gradient across the pulmonic valve measured by Doppler was 7.3 (+/- 3 mmHg) and 7/11 patients had evidence of pulmonary valve sys- tolic doming by echocardiography. Ten patients un- derwent cardiac MRI imaging at some point in their care, of these eight had evidence of pulmonary valve doming, and one patient had a bicuspid pulmon- ic valve. A bicuspid aortic valve was present in one patient who also had dilation of the ascending aorta
and whereas another patient had pectus excavatum and mitral valve prolapse without dilation of the aor- ta. Pulmonary ejection sounds were auscultated in eight patients and pulmonary ejection murmurs were heard in all eleven patients. Pulmonic valve clicks or murmurs led to further investigation and discovery of PAA by echocardiography in nine patients. Incidental discovery occurred by chest CT in one patient with frequent pulmonary infections and another patient with a thyroid cyst.
Discussion
Pulmonary arterial aneurysm (PAA) may occur in a variety of settings and is often associated with pul- monary arterial hypertension or congenital cardiac shunts. The condition has been described in patients with pulmonary valve pathology, but long-term data on clinical signi cance and progression is lacking. This study sought to delineate the long-term outcomes in a cohort of adults with no more than mild pulmonary valve dysfunction and PAA. Long-term follow-up sug- gests a benign course without catastrophic compli- cations of rupture or dissection despite 6/11 patients having a PAA ≥ 5 cm. The dilation of the pulmonary artery occurred slowly over time and did not seem to cause any secondary clinical complications such as
Journal of Structural Heart Disease, June 2018
Volume 4, Issue 3:79-84