Surgical treatment of a pulmonary artery aneurysm due to a regurgitant quadricuspid pulmonary valve

Abstract

We report the case of a patient with a pulmonary artery (PA) aneurysm secondary to a regurgitant quadricuspid pulmonary valve, in which both lesions were successfully repaired. The patient, a 16-year old boy, was known to have had pulmonary regurgitation and progressive dilation of the PA for years. He was operated on when he developed symptoms of effort, a dilated right ventricle and a PA of 55 mm. The quadricuspid pulmonary valve was an intraoperative finding. It had a dilated annulus, two normal cusps, a third with a 'sparrow-nest' configuration and the fourth was severely hypoplastic (which explained the valve regurgitation). The valve was repaired through tricuspidisation of the quadricuspid pulmonary valve (annular plication at the level of the hypoplastic cusp, freeing of excedentary tissue from the 'sparrow-nest' cusp and its reattachment to the plicated annulus). The aneurysm was treated through a reduction pulmonary arterioplasty. Early assessment showed the minimal regurgitation of the valve and a normal diameter PA. The outcome was uneventful, with a stable correction after 44 months of follow-up. To our knowledge, this is the first repair of a quadricuspid pulmonary valve, through tricuspidisation.

Aneurysms of the Pulmonary Artery

“…6 Many patients with PAA also present with pulmonary valve regurgitation, and even though it is more plausible that it is a consequence of annulus dilatation by the PAA, it may also be an independent etiologic factor in the formation of a PAA. 3,10 Patients with the congenital malformation of an absent pulmonary valve syndrome represent a PAA subset that might link pulmonary valve regurgitation to PAA formation ( Figure 1). Absent pulmonary valve syndrome has been described as a rare variant of tetralogy of Fallot but has also been associated with ventricular septal defects and Uhl anomaly and very seldom occurs as an isolated congenital heart defect.…”

“…19 However, some authors have suggested that the Hughes-Stovin syndrome is a cardiovascular manifestation of the Behçet syndrome and therefore part of the same disease process. 18,19 PA hypertension (PAH) is an important cause of PAA formation 10,14,20 and has been suggested to be a clinical symptom of an existing PAA. 21 PAH is a clinical syndrome characterized by an increase in pulmonary vascular resistance leading to failure of the right side of the heart and ultimately to death.…”

“…10,34,35,44 In case of symptomatic pulmonary valve regurgitation and right ventricular dilatation, the timing of surgical intervention should be determined by changes in right ventricular size and function rather than the size of the PAA itself. 10,34 Moreover, in cases of PAH or an underlying tissue disease, surgery seems to be the treatment of choice. 20 However, for this patient group with an aggressive underlying cause of the PAA such as patients presenting with idiopathic PAH who already have a very limited life span despite the introduction of new pharmaceutical therapy options, a more careful therapeutic approach seems reasonable.…”

“…Today, aneurysmectomy and repair or replacement of the right ventricular outflow tract seem to be the methods of choice. 10,34 Moreover, aneurysmectomy is the only feasible treatment for patients with connective tissue disorders. 34 With respect to extension of the PAA, different replacement strategies are possible.…”

“…This can be performed with Gore-Tex or Dacron tubes, homografts, or xenografts (porcine aortic grafts or bovine jugular conduits). 10,34 Another possible option may be a valve-sparing surgical approach comparable to the David procedure. However, in case of involvement of the pulmonary valve, repair or replacement should seriously be considered for relief of right ventricular volume overload and hemodynamic burden on the vessel wall.…”