Developmental lung diseases associated with pulmonary hypertension
| Developmental defect | Vascular pathology |
|---|---|
| Alveolar capillary dysplasia (ACD) with or without misalignments of veins (MPV) | Genetic disorder associated with an abnormal development of the capillary vascular system around the alveoli of the lungs |
| Bronchopulmonary dysplasia (BPD) | Prenatal and postnatal impact of exogenous risk factors on a structural and functional immature lung lead to postnatal impairment of angiogenesis and alveolarisation associated with abnormal vascular function (increased tone, altered reactivity, impaired metabolism) and structure (smooth muscle cell proliferation, altered extra cellular matrix structure) |
| Congenital diaphragmatic hernia | Developmental defect leading to severe vascular remodelling and rarefication of the vascular bed |
| Lung hypoplasia (primary and secondary) | Genetic abnormalities or severe reduction in amniotic fluid leading to reduced prenatal alveolar and vascular development |
| Pulmonary interstitial glycogenosis | Rare non-lethal paediatric form of interstitial lung disease, possible male predominance. Infants present with respiratory distress. Histological characteristics are the accumulation of monoparticulate glycogen in interstitial cells and associated lung growth abnormalities affecting all lung structures |
| Pulmonary alveolar proteinosis | Rare lung disease in which abnormal accumulation of surfactant occurs within the alveoli, interfering with gas exchange and affecting lung growth. Possible cause anti-GM-CSF autoantibodies |
| Pulmonary lymphangiectasia | Rare developmental pulmonary disorder characterised by pulmonary subpleural, interlobar, perivascular and peribronchial lymphatic dilatation |
| Surfactant protein (SP) abnormalities (SP-B and SP-C deficiency, ATP binding cassette A3 mutation, thyroid transcription factor 1/Nkx2.1 homeobox mutation) | Genetic inheritance of surfactant deficiency leading to impaired lung development |