Cardiology

Pulmonary stenosis

Follow Up Questions/Answers

Can you present your findings and take me through your list of differential diagnoses?
o This patient has:
o An ejection systolic murmur which is audible throughout the precordium
o A midline sternotomy scar with no other added scars
o No other peripheral stigmata of cardiovascular disease.
o Given this lady’s age and the findings of examination, she has a congenital
abnormality, of which there are a number of differentials:
o Aortic valve disease, which may have led her to have had a
bioprosthetic aortic valve replacement
o Pulmonary valve disease, which may have led to pulmonary valve
replacement
o Tetralogy of Fallot, leading to overriding aorta right ventricular failure, a
ventricular septal defect and pulmonary stenosis. However, this is
unlikely given her equal pulses and lack of a lateral thoracotomy scar
o Transposition of the great vessels, which have in turn been repaired.
How would you confirm your diagnosis?
o I would like to:
o Take a thorough history from the patient
o Examine their respiratory and abdominal systems
o Obtain her previous operation notes to get further information on what
surgery she’s had done
o Do an ECG
o Do a chest X-ray
o Do an echocardiogram
o Do a cardiac MRI scan. If there were any further abnormalities seen on the
cardiac MRI scan, she might be referred for cardiac catheterisation.
How would you manage this lady’s long-term follow-up?
o I would like this lady to be followed up on a regular basis in the
cardiology clinic
o She would need regular echocardiography to monitor any valvular
abnormalities that she might have
o I would also like to counsel this lady about endocarditis prophylaxis, given my
concern about possible valve disease.
Cardiology

What are the causes of pulmonary stenosis?

o The causes of pulmonary stenosis are primarily congenital causes,
which can simply be pulmonary stenosis on its own, or be associated with other
congenital conditions:
o Tetralogy of Fallot
o Williams or Noonan syndrome.
o There are also infective causes:
o Infective endocarditis
o Rheumatic fever
o Carcinoid syndrome.
How would you manage pulmonary valve disease?
o I would like to treat the underlying cause, particularly if it’s due to
infection.
o Manage the disease as necessary going forward, particularly if there’s a
stenosis, for which I would consider balloon valvuloplasty.
o If there’s evidence of pulmonary regurgitation, in some cases patients are
referred for valve replacement surgery.
Cardiology

Pulmonary stenosis

Key Words and Phrases

The clinical symptoms of pulmonary stenosis are variable depending on

its severity. Mild to moderate stenosis is generally haemodynamically well tolerated and
is not associated with cardiac symptoms. Severe to critical stenosis results in clinical
symptoms such as dyspnoea, exercise intolerance, fatigue from poor cardiac output,
syncope, cyanosis - if a right-to-left shunt via a patent foramen ovale or atrial or
ventricular septal defect is present, or even sudden death.
The cause of the stenosis can be divided according to the level of valve obstruction. The
commonest site is at the valve itself, and the most frequent cause of congenital heart
disease, possibly due to malformation of the bulbus cordis or fetal endocarditis.
Notably, pulmonary stenosis makes up about 10% of all congenital heart disease.
Genetic causes of valvular obstruction include Noonan syndrome and
Alagille syndrome, which is a rare autosomal-dominant disorder characterised by
cardiac, liver, kidney, eye and skeletal abnormalities, with a typical facial appearance
of a broad forehead, deep-set eyes and pointy chin. Acquired causes of valvular
stenosis include carcinoid syndrome and rheumatic heart disease.
Sub-valvular obstruction occurs in Tetralogy of Fallot, which co-exists
with an overriding aorta, right ventricular hypertrophy, and a ventricular septal defect.

Supra-valvular obstruction can also occur with Tetralogy of Fallot, Noonan, Alagille, and
LEOPARD syndromes, together with congenital rubella syndrome and Williams
syndrome.
The main pathophysiological consequence of pulmonary stenosis is right ventricular strain
and an increase in right ventricular pressure.
Once the diagnosis is suspected, based on clinical findings of an ejection systolic murmur in
the pulmonary area heard best on inspiration, it is confirmed by echocardiography with
attention focused on the valve gradient and the right ventricular systolic pressure.
These parameters can be used to categorise patients into mild, moderate, or severe
disease, which directly determines their treatment. The majority of adult patients with
valvular pulmonary stenosis have mild stenosis with a gradient less than 36mmHg and are
asymptomatic. Their stenosis rarely progresses or requires treatment, and a repeat echo
is recommended only every five years.
Patients with gradients between 36 and 64 mmHg have moderate stenosis and
frequently develop dyspnoea and fatigue, while those with gradients greater than
64mmHg have severe stenosis and may develop early right ventricular failure and
cyanosis.

The treatment for moderate or severe disease includes percutaneous pulmonary balloon
valvuloplasty or surgery. Trials of percutaneous valves are ongoing.
Cardiology

The incidence of infective endocarditis in patients with pulmonic stenosis is very low,
but still a little more than twice that in the general population. Most patients with
valvular heart disease, including those with pulmonic stenosis, are not included in this
group unless they have had prior endocarditis or have a prosthetic valve.