pulm stenosis

Question 1

causes

Answer 1

• congenital - by far the most common. Thick deformed bicuspid or tricuspid valves, sometimes associated with Noonan’s syndrome, William’s syndrome, or Fallot’s tetralogy. Peripheral and valvular pulmonary stenosis may occur in rubella syndrome
• rheumatic involvement of the pulmonary valve - rare
• carcinoid tumours of the small bowel with hepatic metastases - these are associated with acquired thickening of valves on the right side of the heart

Question 2

s/s

Answer 2

• usually asymptomatic, presenting only with a murmur.
• if severe obstruction, the cardiac output may be reduced and exertional syncope occasionally can result.
• longstanding obstruction may result in right ventricular failure and therefore necessitate a high filling pressure - right atrial pressure.
• patient appears normal unless there is a recognisable syndrome in association.
• pulse and blood pressure are normal.
• JVP - a large a wave signifies atrial hypertrophy.
• may be a parasternal heave due to right ventricular hypertrophy - and maybe a thrill in the pulmonary area.
• auscultation:
  
  • pulmonary ejection click - the click disappears on inspiration in severe stenosis.
  • a loud pulmonary ejection murmur - loudest to left of upper sternum, with radiation to the left shoulder.
  • second heart sound - with significant obstruction the P2 is delayed and soft.

Question 3

ix

Answer 3

Electrocardiogram:

• P pulmonale (RA hypertrophy)
• RV hypertrophy

Chest radiology:

• prominent pulmonary artery - post-stenotic dilatation
• ventricular mass is normal unless there is right ventricular failure
• right atrial dilatation

Echo / Doppler scan:

• the abnormal valve can be visualised and the severity of the stenosis can be estimated.

Question 4

rx

Answer 4

If the right ventricular pressure is greater than 60 mmHg then treatment is indicated, even if the patient is asymptomatic.

Balloon dilatation of the pulmonary valve is performed during cardiac catheterization. This technique has replaced surgical pulmonary valvotomy as the treatment of choice.

Question 5

relative incidence of most common congenital heart defects

Answer 5

Incidence of congenital heart defects is 8/1000 live births.

• ventricular septal defect 32.5%
• pulmonary stenosis 7.6%
• persistent ductus arteriosus 11.9%
• atrial septal defect 5.9%
• coarctation of the aorta 6.3%
• aortic stenosis 5.1%
• tetralogy of Fallot 5.9%
• transposition of great arteries 5.0%