Adolescent and Young Adult Presentation of CHD
Eisenmenger Syndrome
- Severe pulmonary hypertension (leading to shunt reversal) due to a left-to-right intracardiac shunt
- As a consequence of a large left-to-right shunt for many years, the pulmonary vascular resistance will slowly rise. Eventually, the shunt will reverse and the patient will become cyanosed, typically at about 15-20 years of age (Eisenmenger Syndrome).
- Common cause: unrestricted and unrepaired VSD
- Patients are usually stable for many years, with typical survival into the 4th or 5th decade of life. However, they may then present with some of the complications of a right-to-left shunt and of pulmonary hypertension.
- Patients with Eisenmenger Syndrome can present with:
(i) Syncope
- This may be the first presenting feature in a child or young adult with pulmonary hypertension. It should be differentiated from teenagers who present with neurocardiogenic syncope or vasovagal syncope.
- Clinical examination may reveal cyanosis, a loud P2 and a right ventricular heave.
- When Eisenmenger syndrome develops, the associated cardiac murmur may disappear
- ECG: Right ventricular hypertrophy
- Chest Radiograph: Prominent pulmonary vessels
(ii) Exercise intolerance and asthma or COPD or breathlessness on exertion
This is often the next insidious symptom of pulmonary hypertension, due to Eisenmenger syndrome. Non-wheezing asthma is the cardinal feature. Again, full history, clinical examination and ECG assist in the detection of such patients. Generally, the first presentation of severe asthma should prompt the physician to obtain an ECG after initial stabilization.
(iii) Haemoptysis
This is often a preterminal event in pulmonary hypertension or Eisenmenger Syndrome. It may occur with a chest infection or when there is severe right heart failure.
(iv) During Pregnancy
Those with pulmonary hypertension due to any cause are usually advised not to become pregnant. If this does occur, it is lethal in 50% of cases and termination is generally advised.