Circuit E
STATION 1
This station assesses your ability to elicit clinical signs:
STATION 2
This station assesses your ability to elicit clinical signs:
STATION 3
This station assesses your ability to elicit clinical signs:
STATION 4
This station assesses your ability to elicit clinical signs:
STATION 5
This station assesses your ability to elicit clinical signs:
STATION 6
This station assesses your ability to assess specifically requested areas in a child with a developmental problem:
STATION 7
This station assesses your ability to communicate appropriate, factually correct information in an effective way within the emotional context of the clinical setting:
STATION 8
This station assesses your ability to communicate appropriate, factually correct information in an effective way within the emotional context of the clinical setting:
STATION 9
This station assesses your ability to take a focused history and explain to the parent your diagnosis or differential management plan:
COMMENTS ON STATION 1
DIAGNOSIS: PULMONARY STENOSIS (PS)
These findings suggest a diagnosis of pulmonary stenosis and in particular with the stenosis being at the level of the valve (in view of the click). In the exam diagnosis of this murmur would be entirely dependent on your being able to localise a systolic murmur to the pulmonary area. The click is an added bonus which will clinch the diagnosis but may not be picked up (apparently best heard at the third left intercostal space in expiration.) Textbooks also suggest the presence of a right ventricular heave (this will be felt at the left sternal border).
Please see table below for investigations and management of PS.
NOONAN’S SYNDROME
• Facial dysmorphism, e.g. hypertelorism, down-slanting palpebral fissures, webbed neck, triangular facies, ptosis
Investigations: | |
CXR | Often normal but may see a prominent pulmonary artery or decreased pulmonary vascular markings in more severe disease |
ECG | Normal if mild. If moderate to severe – right axis deviation and right ventricular hypertrophy In Noonan’s you get a superior axis |
ECHO | A gradient of > 40 mmHg would indicate a need for surgery or the right ventricular pressure is > 60 mmHg |
Management: | |
Multidisciplinary | Cardiologist, local paediatrician – local and tertiary referral centre |
Conservative/medical | Adequate nutrition and growth May only need clinical review and no need for surgery if mild May need diuretics if associated significant shunts Need alprostadil (PGE1) in the presence of cyanotic congenital heart disease during the neonatal period Prophylaxis during surgical procedures |
Surgical | Cardiac catheterisation Balloon valvuloplasty is the corrective treatment of choice |
Associated conditions include: | Noonan’s syndrome Tetralogy of Fallot |