Clinical scenario: Cystic fibrosis | bronchiectasis | raised JVP | loud P2
A 13-year-old boy with cystic fibrosis is admitted with progressive exertional dyspnea over the past 6 months. He has a history of recurrent Pseudomonas aeruginosa chest infections and extensive bronchiectasis. His chronic productive cough is unchanged, but his exercise tolerance has markedly deteriorated.
On examination:
- Oxygen saturation: 88% on room air
- Digital clubbing
- Central cyanosis
- Raised jugular venous pressure
- Parasternal heave
- A loud pulmonary component of the second heart sound (loud P2)
- Bilateral coarse crackles over both lung fields
- Mild hepatomegaly and bilateral ankle edema
Chest X-ray shows bilateral cystic bronchiectatic changes without a new area of consolidation.
Which complication has most likely developed?
A. Allergic bronchopulmonary aspergillosis
B. Acute pulmonary embolism
C. Pulmonary hypertension with cor pulmonale
D. Left ventricular failure
E. Tension pneumothorax
Correct answer & Explanation:
Correct answer: C. Pulmonary hypertension with cor pulmonale
Explanation:
This child has advanced cystic fibrosis with extensive bronchiectasis and chronic hypoxemia. The new findings are not due to worsening infection but indicate pulmonary hypertension progressing to cor pulmonale.
The key clues are:
- Progressive exertional dyspnea despite no new infiltrate
- Persistent hypoxemia
- Loud P2
- Parasternal heave (right ventricular hypertrophy)
- Raised JVP
- Hepatomegaly
- Peripheral edema
These are classic signs of right-sided heart failure secondary to pulmonary hypertension.
Why other options are incorrect:
- A. Allergic bronchopulmonary aspergillosis: Usually presents with worsening wheeze, eosinophilia, elevated IgE, and new pulmonary infiltrates rather than right heart failure.
- B. Acute pulmonary embolism: Presents with acute pleuritic chest pain and sudden deterioration; it is uncommon in children with CF.
- D. Left ventricular failure: Would cause pulmonary edema and is not suggested by the examination findings.
- E. Tension pneumothorax: Causes acute respiratory distress with unilateral absent breath sounds and tracheal deviation.
Exam pearl:
In a child with bronchiectasis or cystic fibrosis, the development of:
- Loud P2
- Parasternal heave
- Raised JVP
- Hepatomegaly
- Peripheral edema
should immediately suggest pulmonary hypertension with cor pulmonale.
