Prof Julene S Carvalho Head of Brompton Centre for Fetal Cardiology

Consultant Fetal and Paediatric Cardiologist Professor of Practice, Fetal Cardiology

Molecular & Clinical Sciences Research Institute, SGUL

Big aorta, big pulmonary artery

London 24 January 2020

How big is too big and does it matter?

Vessel size

Big aorta and pulmonary artery

Objectives

• To exclude structural heart disease

• To appreciate relative sizes of the aorta and PA

• To make objective measurements

• To have a plan

Big aorta and pulmonary artery

Objectives

• To appreciate relative sizes of the aorta and PA

PAAo SVC

RT

LT

Vessel number Vessel size Vessel arrangement Vessel alignment

PAAo

SVC

Right

Left

Ant

Post

Big aorta and pulmonary artery

Objectives

• To appreciate relative sizes of the aorta and PA

• To make objective measurements

http://fetal.parameterz.com

http://fetal.parameterz.com

Big aorta and pulmonary artery

Objectives

• To make objective measurements

• To exclude structural heart disease

Big aorta, small PA or both?Case 1- 36 weeks

Tetralogy of Fallot

Case 2- 19 weeks

Small aorta or big PA?

Coarctation

27 weeks 33 weeks

Coarctation

Case 3- 21 weeks

Big pulmonary artery?

Vmax = 2m/s

Pulmonary stenosis

Case 4- 21 weeks

Big aorta

Aortic aliaising

67cm/s

67cm/s

Ao Vmax = 154cm/s

Valvar aortic stenosis

Valvar pulmonary stenosis … mother: likely Noonan

Case 4- 21 weeks

Referred for ‘big pulmonary artery’

No structural abnormality

What next?

Sonographer referral for ‘big aorta’No structural cardiac abnormality

‘Big-gish’ aortaNo structural cardiac abnormality

Z-score = + 1.86

Family history of aortic aneurysm/ dissection

Patient referred for ‘bicuspid aortic valve’ - 23 weeksNo structural cardiac abnormality - ‘aorta a bit big’?

Bicuspid valve?

Patient referred for ‘bicuspid aortic valve’

Normal looking aortic valve at 31-week follow up

Patient self-referred ‘for reassurance’No structural cardiac abnormality - 20 weeks - prominent PA?

http://fetal.parameterz.com

No structural cardiac abnormality - 20 weeks - prominent PA = 4.6mm?

http://fetal.parameterz.com

Big aorta and pulmonary artery

Objectives

• To exclude structural heart disease

• To have a plan

Big aorta and pulmonary arterySummary

• Family history • Consider bicuspid aortic valve • Consider possibility of aortopathy • Consider possibility of genetic syndrome:

• Exclude obvious cardiac abnormality

• Aortic and pulmonary stenosis • Rescan to exclude minor lesions that may evolve

• May still remain unexplained

• Marfan, other collagen disorders • Noonan syndrome (pulmonary stenosis, ? progression)

• Calculate Z-scores and follow up if > +2

Thank you