Cardiac CT of Dome-shaped Pulmonary Valve Stenosis

HomeRadiologyVol. 311, No. 2 PreviousNext Reviews and CommentaryFree AccessImages in RadiologyCardiac CT of Dome-shaped Pulmonary Valve StenosisMarco Parillo , Domenico De StefanoMarco Parillo , Domenico De StefanoAuthor AffiliationsFrom the Fondazione Policlinico Universitario Campus Bio-Medico, Via Alvaro del Portillo 200, 00128 Rome, Italy; and Research Unit of Diagnostic Imaging and Interventional Radiology, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Rome, Italy.Address correspondence to M.P. (email: [email protected]).Marco Parillo Domenico De StefanoPublished Online:May 14 2024https://doi.org/10.1148/radiol.233161MoreSectionsPDF ToolsAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookXLinked In Supplemental material is available for this article.A 57-year-old woman was admitted to the cardiology department because of severe congenital pulmonary stenosis. Transthoracic echocardiography showed a dysmorphic pulmonary valve with severe funnel-shaped stenosis (peak gradient, 80 mm Hg). Cardiac CT (CCT) was requested to provide detailed anatomic information regarding the pulmonary valve and the coronary arteries in anticipation of a valvuloplasty procedure.CCT was performed with a 128-section multidetector CT scanner with an optimized imaging protocol. The region of interest of bolus tracking was set in the ascending aorta. We used a split-bolus injection technique: an initial injection of 90 mL of contrast medium at a flow rate of 6 mL/sec, followed by a subsequent injection of 50 mL of mixed bolus (50:50 saline and contrast medium) at a flow rate of 6 mL/sec and a 30-mL saline chaser at a flow rate of 5 mL/sec (1). In this way, it was possible to achieve adequate attenuation of both the right heart chambers and the coronary arteries, despite the pronounced enlargement of the right atrium, avoiding streak artifacts from high-attenuation contrast medium in the right atrial appendage. Furthermore, we employed retrospective multiphasic acquisition, which allowed cine CCT reconstruction (Movies 1, 2). Movie 1: Cine cardiac CT of the right ventricular outflow tract shows the dome shape of pulmonary valve during systole but not diastole; also note the right ventricular outflow obstruction in systole due to the marked right ventricle hypertrophy.Download Original Video (4.1 MB) Movie 2: Axial cine cardiac CT in the pulmonary valve plane shows the movement of the three thickened valve leaflets during the cardiac cycle.Download Original Video (2.9 MB)The main findings of CCT were as follows: diffuse right ventricular hypertrophy (maximum thickness of infundibular wall, 1.2 cm), with reduced diameter of the pulmonary infundibulum in systole; severe right atrial enlargement; a dome-shaped tricuspid pulmonary valve; a main pulmonary artery aneurysm (diameter, 5 cm); and a left main pulmonary artery dilatation (diameter, 3.2 cm) (Figure). The presence of significant stenosis in the coronary circulation was excluded in the same examination. The patient underwent right ventriculography and balloon pulmonary valvuloplasty. Postprocedure transthoracic echocardiography showed a reduction in the peak gradient, and the patient was then discharged.Multiplanar reconstructions from cardiac CT in a 57-year-old woman with pulmonary stenosis. (A) Four-chamber view shows good attenuation of the right chambers (*) with diffuse right ventricular hypertrophy and severe right atrial enlargement. (B, C) Axial views of the infundibulum (circle) show a marked reduction in diameter during (C) systole compared with (B) diastole. (D) Axial view shows a poststenotic aneurysm of the main pulmonary artery with a diameter of 5 cm (solid arrow); a dilatation of the left pulmonary artery is also noted, with a diameter of 3.2 cm (dashed arrow). (E, F) In coronal views of the right ventricular outflow tract, the dome shape of the pulmonary valve (arrow) is visible during (F) systole but not (E) diastole; also note the right ventricular outflow obstruction in systole due to the marked right ventricular hypertrophy. (G) Axial view in the pulmonary supravalvular plane during systole shows the typical restricted central opening of the dome-shaped configuration (arrow).Download as PowerPointCCT plays a critical role not only in the diagnosis of coronary artery disease, but also in the study of valvulopathies, including those involving the pulmonary valve, often referred to as the "neglected valve" because it is the least frequently imaged among the cardiac valves (2).Disclosures of conflicts of interest: M.P. No relevant relationships. D.D.S. No relevant relationships.References1. Saremi F, Gera A, Ho SY, Hijazi ZM, Sánchez-Quintana D. CT and MR imaging of the pulmonary valve. RadioGraphics 2014;34(1):51–71. Link, Google Scholar2. Costantini P, Perone F, Siani A, et al. Multimodality imaging of the neglected valve: role of echocardiography, cardiac magnetic resonance and cardiac computed tomography in pulmonary stenosis and regurgitation. J Imaging 2022;8(10):278. Crossref, Medline, Google ScholarArticle HistoryReceived: Nov 22 2023Revision requested: Jan 8 2024Revision received: Jan 23 2024Accepted: Feb 5 2024Published online: May 14 2024 FiguresReferencesRelatedDetailsRecommended Articles RSNA Education Exhibits RSNA Case Collection Vol. 311, No. 2 Metrics Altmetric Score PDF download