Quick Fire Cases
Malenka M. Bissell, MD, PhD
Clinical lecturer
University of Leeds, United Kingdom
Lisa Ferrie, MSc, BSc
Biomedical Engineer
Leeds Teaching Hospitals NHS, United Kingdom
Rawan Abuzinadah, MSc
PhD student
University of Leeds, Leeds Institute of Cardiovascular and Metabolic Medicine (LICAMM), United Kingdom
Julian Tonge
Lead Congenital Cardiac MRI Radiographer
Leeds Teaching Hospitals NHS, United Kingdom
David A. Broadbent, PhD
Principal Clinical Scientist (MRI Physics)
Leeds Institute of Cardiovascular and Metabolic Medicine, United Kingdom
Sven Plein, MD PhD
Professor of Cardiovascular Imaging
University of Leeds, United Kingdom
Shuba Barwick
Consultant Paediatric Cardiologist
Leeds teaching hospitals trust, United Kingdom
Elspeth Brown
Consultant Paediatric Cardiologist
Leeds teaching hospitals trust, United Kingdom
Chris Oakley, MD
Consultant Paediatric Cardiology
Leeds teaching hospitals, United Kingdom
Malenka M. Bissell, MD, PhD
Clinical lecturer
University of Leeds, United Kingdom
Case 1 was referred for third trimester fetal CMR due to concerns with pulmonary drainage in the background of hypoplastic left heart syndrome (HLHS) on fetal echocardiogram.
Case 2 was a late diagnosis of pregnancy and HLHS with restricted inter-atrial septum but possible ascending vein on fetal echocardiogram.
Diagnostic Techniques and Their Most Important Findings:
Both cases underwent fetal CMR using HASTE image stacks in 5 different planes. These stacks were reconstructed into 3D MPRs using Northh Medical slice to volume reconstruction tool. Mimics Innovation Suite (V 26.0, Materialise, Belgium) was used to segment the fetal volume, lung volume and cardiac anatomy.
Case 1 showed HLHS with anomalous right pulmonary venous return with a single, non-dilated vein draining into the IVC just at the junction to the right atrium. Additionally, an arterial vessel was arising from the descending aorta into the right lung. The right lung volume was reduced (48% of expected). Postnatal CT confirmed a very abnormal hypoplastic right lung with only 1 lobe with abnormal pulmonary venous drainage and the additional arterial feeding vessel from the descending aorta to the abnormal right lung. Therefore, baby was not a candidate for the surgical single ventricle pathway.
Case 2 showed HLHS with partially abnormal pulmonary venous drainage. All pulmonary veins were dilated. 2 right pulmonary veins drained into the left atrium (LA). The lower left pulmonary vein was also draining into the LA. The middle and upper left pulmonary veins did not drain into the LA but via a vertical vein into the innominate vein. The lower left pulmonary vein was connected to this vertical vein via two connecting vessels. No classical levoatriocardinal vein was seen. Lung volumes were normal but lung parenchyma appeared hyperdense with the appearance of nutmeg lung pattern. The findings were confirmed on postnatal fetal echocardiogram and therefore, this baby was also not a candidate for the surgical single ventricle pathway.
Learning Points from this Case:
A levoatriocardinal vein usually is an additional vessel arising from the left atrium with all pulmonary veins entering into the LA. In case 2 this was not the case with the left middle and upper vein draining via the therefore vertical vein into the SVC. The 2 collateral vessels between the left lower pulmonary vein and vertical vein likely formed in response to the severely increased left atrial pressures in the setting of a restricted inter-atrial septum.
