Carol Lam, Presenter: Nothing to Disclose

Laura Jimenez-Juan MD, Abstract Co-Author: Nothing to Disclose

Rachel Wald MD, Abstract Co-Author: Nothing to Disclose

Elsie Nguyen MD, Abstract Co-Author: Nothing to Disclose

Sebastian Ley, Abstract Co-Author: Nothing to Disclose

Narinder S. Paul MD, Abstract Co-Author: Research support, Toshiba Corporation

Bernd J. Wintersperger MD, Abstract Co-Author: Speakers Bureau, Bayer AG Speakers Bureau, Siemens AG

Andrew Michael Dominic Crean MD, Abstract Co-Author: Nothing to Disclose

To document cardiac MR (CMR) imaging findings and surgical correlation in a cohort of patients presenting in adult life for repair of partial anomalous pulmonary venous return (PAPVR) and/or sinus venosus defect (SVD).

Imaging reports and operative notes of patients (a) diagnosed with PAPVR and/or SVD, (b) repaired surgically, and (c) with CMR prior to surgery were reviewed. 

50 consecutive patients were included. 32 patients had PAPVR and superior (S-) SVD, 2 patients had PAPVR and inferior (I-) SVD, 4 patients had PAPVR and a secundum atrial septal defect, 9 patients had PAPVR only and 3 patients had I-SVD alone. The median age at surgery was 44 (17 – 74) years and 54% of the cohort was female. 60% were Caucasian, 18% were Asian or Middle Eastern, 16% were of uncertain ethnicity and 6% were Afro-Caribbean. Almost 20% of the cohort were immigrants to Canada. The commonest symptoms at presentation were palpitations (46%), breathlessness (38%) chest pain (20%) and presyncope (18%). However in 15 out of 50 patients the anomaly was discovered incidentally.  Associated CV anomalies were common including dual superior vena cavae (14%) and secundum atrial septal defects (8%). Mean right ventricular end diastolic volume (EDV) by CMR was 332±81ml and mean RV ejection fraction (EF) was 55±6%. There was no significant difference in RV EDV between those with PAPVR alone vs those with combined PAPVR & SVD (322±70ml vs 334±87ml). Mean left ventricular (LV) EF was 60±6% for all patients combined. Compared to CMR, transesophageal echo (n = 17) and transthoracic echo (n = 25) underestimated right ventricular dilatation by at least one grade in 41% and 36% of patients, respectively Shunt fraction was significantly higher as measured by CMR phase contrast than invasive angiography (2.8:1 vs 2.3:1 p<0.01) although neither correlated well with RV EDV. There was no relationship between age at surgery and RV EDV. The presence or absence of PAPVR±SVD was correctly ascertained in 48/50 cases for PAPVR and 49/50 cases for SVD compared to the surgical reference standard.

CMR was highly accurate for the identification of PAPVR±SVD. Echocardiography underestimated RV size perhaps contributing to delayed recognition. The RV remodelling may occur in adolescence or early adult life since there was no correlation between age and RV EDV.

CMR is accurate for diagnosis of PAPVR±SVD in adults.  

Lam, C, Jimenez-Juan, L, Wald, R, Nguyen, E, Ley, S, Paul, N, Wintersperger, B, Crean, A, Cardiac Magnetic Resonance Imaging Findings in Late-presenting PAPVR and Sinus Venosus Defect in 50 Consecutive Adult Patients Undergoing Surgical Repair.  Radiological Society of North America 2011 Scientific Assembly and Annual Meeting, November 26 - December 2, 2011 ,Chicago IL. http://archive.rsna.org/2011/11013285.html