Aneurysmal sac located between right pulmonary artery and left atrium

Abstract

Clinical Summary A 6-year-old boy had dyspnea and severe cyanosis, which first appeared at 6 months of age, and was admitted to our hospital. Mucocutaneous telengiectasias were not present. A negative pathergy test ruled out Behcet’s disease. His echocardiography revealed an aneurysmal sac behind the LA, which was in continuity with the LA via a large orifice and absent adjunctive defects. Angiography demonstrated a wide connection between the sac and LA and a large, single feeding artery (afferent) originating from the right PA without an efferent vessel from the sac (Figure 1, a). Cardiac 16-slice computerized tomographic angiography showed that PV orifices were not in relation with the sac and no additional PAVMs were present. All the diagnostic tests supported the absence of thrombi or calcium. A right thoracotomy through the fourth intercostal space was performed, and an aneurysm was found posteroinferior of the right lower lobe. It was not covered with parenchyma and was adhered loosely to surrounding lung tissue. The feeding artery was found at superomedioposterior aspect of aneurysm and ligated and divided (Figure 1, b). A Satinsky clamp was placed over the transition line and the aneurysm was opened. There were no intraluminal thrombi. Following resection of the aneurysm wall, the LA was oversewn. The patient’s postoperative course was uneventful. Pathologic examination of the sac wall revealed aneurysm tissue originated from vessel wall. The patient was discharged from the hospital on postoperative day 6 with an arterial saturation of 97% in room air. At postoperative month 6, his functional capacity was in New York Heart Association class I and he was still fully saturated.