Abstract
A 77-year-old female, known hypertensive and dyslipidemic on treatment presented with three episodes of syncope in the last two months. On examination; there was grade 4/6 harsh systolic murmur on the lateral sternal border. Transthoracic echocardiography was difficult because of mesocardia and abnormal rotation of the heart due to enlarged right sided chambers. There is mild left ventricular hypertrophy with normal ejection fraction, no left sided valvular disease. The right ventricle was hypertrophied and dilated with normal RV function. The pulmonary valve was thickened with significant systolic flow aliasing through the valve with significant regurgitation and huge main pulmonary trunk aneurysm (59 mm at its wideset diameter) (Figure 1). Transthoracic approach did not allow a correct alignment of the Doppler CW and the correct estimate of pulmonary valvulopathy; TEE was performed with a correct visualization of the valve in deep transgastric projection at 90 degrees. The valve was thickened, fibrotic, degenerated with systolic doming of leaflets (Figure 2) and peak systolic gradient ∼ 70 mmHg (Figure 3). 3D reconstruction of the valve showed a tricuspid valve (Figure 4) with a valve area ∼ 0.9 cm2 using planimetry in MPR (Figure 5). CT scan was performed which confirmed the main pulmonary trunk aneurysm ∼ 60 mm (Figure 6). Therefore, in light of the clinical and instrumental picture, the patient was referred to heart team discussion for the plan of surgical intervention.
Discussion
According to the ESC guidelines for grown up congenital heart disease in 2010, this pulmonary valve should be intervened upon as it is severe symptomatic PS (1), but there are 2 problems with this case; the first is significant associated PR, so no place for balloon dilatation here, the second problem is the pulmonary artery aneurysm (PAA). The dilemma of management of pulmonary PAA is that all the available data are about aortic aneurysms. Indications for intervention for PAA include: Absolute PAA diameter ≥ 5.5 cm, Increase in the diameter of the aneurysm of ≥ 0.5 cm in 6 mo, Compression of adjacent structures, Thrombus formation in the aneurysm sack, Evidence of valvular pathologies or shunt flow Verification of PAH, Signs of rupture or dissection (2). Surgery could include: Aneurysmorrhaphy only decreases the diameter of the vessel (3). Aneurysmectomy and repair or replacement of the right ventricular outflow tract is commonly used technique recently and mostly suits connective tissue disorders (6). Also, Replacement of the PA and the pulmonary trunk with a conduit (Gore-Tex or Dacron tubes, homografts, or xenografts) starting in the right ventricular outflow tract with replacement of the pulmonary valve (4).
Conclusion
PAA management is currently challenging because there are no clear guidelines on its optimal treatment. The presence of significant pulmonary valve dysfunction could affect the decision making of the associated PAA management.
Abstract P180 Figure.
Ventricular Septation and Outflow Tract Development in Crocodilians Result in Two Aortas With Bicuspid Semilunar Valves
