[Isolated agenesia of pulmonary artery]
1Unité de médecine infantile, CHU Timone-enfants, 13385 Marseille cedex 05, France.
Insights
Unilateral primitive pulmonary artery agenesis is a rare congenital condition. Pediatric patients diagnosed with this anomaly showed recurrent infections and asthmatic symptoms, but experienced no major complications during long-term follow-up.
Area of Science:
- Pediatric Pulmonology
- Congenital Cardiovascular Anomalies
- Medical Genetics
Background:
- Unilateral primitive agenesis of the pulmonary artery (PAPA) is an exceptionally rare congenital disorder.
- This condition involves the congenital absence of one of the main pulmonary arteries, impacting blood flow to the lungs.
Observation:
- A study reviewed eight pediatric cases (3 boys, 5 girls) with PAPA over an 11-year median follow-up.
- The median age at diagnosis was 4 years.
- Common clinical manifestations included recurrent respiratory tract infections (75%), dyspnea on exertion (50%), and asthmatic symptoms (75%).
Findings:
- Diagnosis was consistently confirmed via chest X-rays and angiography.
- Agenesis of the right pulmonary artery occurred in five cases, while two cases of left pulmonary artery agenesis were associated with abnormal vascular rings.
- Pulmonary function tests revealed bronchial obstruction in two of the six evaluated children.
Implications:
- Long-term clinical follow-up, extending into adulthood for some patients, indicated a lack of severe complications like hemoptysis or pulmonary hypertension.
- This suggests a potentially manageable prognosis for pediatric patients with unilateral primitive pulmonary artery agenesis.
- Further research into the long-term outcomes and management strategies for this rare condition is warranted.
Abstract:
Unilateral primitive agenesia of pulmonary artery is a rare congenital disorder. Experience of three hospital-based pulmonology pediatric units including eight children (three boys and five girls) with such anomaly is reported (11 years median follow-up, range 6 months to 10 years). Median age at diagnosis was 4 years. Clinical features included recurrent respiratory tract infections (75%), effort dyspnea (50%) and, curiously, asthmatic symptoms (75%). Diagnosis was evoked on chest X rays and confirmed by angiography in all cases. Agenesis of the right pulmonary artery was more frequent (five cases). Abnormal vascular ring was associated in two cases of agenesis of the left pulmonary artery. Pulmonary function tests were performed in six children, with bronchial obstruction in two. Regular clinical follow-up, up to adult age in four cases, showed no complication, such as hemoptysis or pulmonary hypertension in our patients.
