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Radiology
Pulmonary Stenosis and Atresia
Built from Obstetric Imaging
What’s inside
10 sections · 72 slides
Overview
- What this deck covers
The two defects
One valve, two very different problems
- Start with the valve itself
- Where the pulmonary valve sits in the heart
- Pulmonary stenosis in plain words
- The three levels of narrowing
- Pulmonary atresia in plain words
- Why the right ventricle ends up small
- Normal heart beside a narrowed pulmonary valve
- 9%-10%
- Stenosis or atresia: the core difference
- How to tell them apart
Causes and associations
Genes, syndromes, and the company these defects keep
- What causes them
- Syndromes to keep in mind
- Two faces of fetal pulmonary stenosis
- Atresia with a hole in the septum is a different disease
- Two types of atresia, set by the tricuspid valve
- Type I atresia: the small right ventricle
- Type II atresia: the big leaking right ventricle
- Ventriculocoronary fistulas and why they matter
- Where do the fistulas come from
How the fetus and newborn behave
From a silent scan finding to a sick blue baby
- What the fetus and the newborn show
- How a tight valve damages the right heart
- Keeping the duct open after birth
- Two features that predict a bad course
Finding stenosis on ultrasound
Views, valve signs and Doppler
- The order in which you look
- Will the four-chamber view show it
- Signs of a diseased valve
- Narrowed, thickened pulmonary valve on a short-axis view
- What to notice in that picture
- A size rule you can use
- What colour Doppler adds
- Turbulent flow through a mildly narrowed valve
- What pulsed Doppler measures
- Accelerated flow across the pulmonary valve
- What the right ventricle looks like in severe disease
- Thick right ventricle and a leaking tricuspid valve
- What to notice in that four-chamber view
- When flow in the duct turns around
- Normal versus reversed flow at the three vessels and trachea view
- Reversed flow in the pulmonary artery and duct, zoomed
- Stenosis: the classic signs together
Finding atresia on ultrasound
A blocked valve changes every view
- The four-chamber view in type I atresia
- Spotting the coronary fistulas
- The four-chamber view in type II atresia
- Two shapes of the same blockage
- The outflow and three-vessel views in atresia
- Atresia: the classic signs together
- When you cannot tell atresia from critical stenosis
- Another way to look at the valves
What else could it be
Four look-alikes, and the sign that separates each
- Look-alike 1: the same lesions with a hole in the septum
- Look-alike 2: tricuspid atresia
- Look-alike 3: Ebstein anomaly and tricuspid dysplasia
- Look-alike 4: the recipient twin
Management before birth
Testing, follow-up, delivery and fetal intervention
- The work-up once the diagnosis is made
- Why follow-up scans matter so much
- 40%
- Reading those two numbers
- Planning the delivery
- Fetal pulmonary valvuloplasty
Management after birth
Two ventricles if possible, one if not
- Mild stenosis after birth
- The first decision in critical disease
- Which repair route the newborn takes
- The two-ventricle route
- The one-ventricle route
- Living with a one-ventricle circulation
- 90%
Take home
What the referring clinician must remember
- What the referring physician needs to know
- Advice to give the family and the team
- Three points to carry away
- References and suggested reading
- References and suggested reading (continued)
- Obstetric Imaging: Fetal Diagnosis and Care, 2nd Edition