Pulmonary Artery Hypertension: Fifty Years Following Pneumonectomy in Infancy

Area of Science:

• Cardiology
• Pulmonology
• Vascular Medicine

Background:

• Pulmonary hypertension (PH) is a serious vascular complication that can arise after major lung resection.
• Pneumonectomy, the removal of an entire lung, significantly increases the risk of developing PH and impacts functional status.
• Long-term consequences of pneumonectomy, including PH developing decades later, require careful consideration.

Purpose of the Study:

• To describe the diagnosis and management of moderate pulmonary hypertension (PH) in a patient presenting over 50 years after a unilateral pneumonectomy.
• To highlight the potential for symptom improvement in long-standing PH post-pneumonectomy with targeted treatment.
• To discuss the implications of delayed-onset PH following major lung resection.

Main Methods:

• Case report detailing the diagnostic process for moderate pulmonary hypertension (PH).
• Management strategies employed, including off-label use of WHO group 1 treatments.
• Long-term follow-up of a patient who underwent total unilateral pneumonectomy in infancy.

Main Results:

• Diagnosis of moderate pulmonary hypertension (PH) confirmed more than 50 years after unilateral pneumonectomy.
• Significant improvement in PH symptoms and functional status observed.
• Successful management achieved through off-label administration of WHO group 1 targeted therapy.

Conclusions:

• Pulmonary hypertension (PH) can manifest decades after pneumonectomy, significantly impacting patient health.
• Off-label use of WHO group 1 treatments may offer a viable therapeutic option for selected PH patients post-pneumonectomy.
• Early recognition and tailored management are crucial for improving outcomes in patients with delayed-onset PH.