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Lung pathology in infants with severe pulmonary hypertension and cardiac disease
Insights
Severe septitis, a lung condition in infants with congenital heart disease, significantly impacts survival. This finding highlights septitis as a critical prognostic factor over pulmonary vascular changes in early infancy.
Area of Science:
- Pediatric Cardiology
- Pulmonary Hypertension
- Infant Pathology
Background:
- Congenital heart disease (CHD) in infants often presents with severe pulmonary hypertension (PH).
- Respiratory distress is a common complication in these infants.
- Pulmonary vascular changes are typically assessed in managing PH.
Purpose of the Study:
- To investigate the pathological lung changes in infants with CHD and PH.
- To correlate lung pathology with clinical outcomes after surgical correction.
- To identify key prognostic factors in early infantile PH.
Main Methods:
- Lung biopsy specimens from 39 infants (<12 months) with CHD and severe PH were analyzed.
- Pathological findings were compared with clinical courses and surgical outcomes.
- Septitis was classified as mild, moderate, or severe based on lymphoid infiltration and alveolar septal thickening.
Main Results:
- A common finding was "septitis" (lymphoid infiltration and alveolar septal thickening).
- Pulmonary vascular obstructive changes were generally within Grade 2.
- Survival rates were significantly lower in infants with severe septitis (3/19) compared to moderate (10/12) or mild (8/8) septitis.
Conclusions:
- Septitis is a critical pathological finding in infants with PH and CHD.
- The severity of septitis, not pulmonary vascular obstruction, is strongly correlated with postoperative survival.
- Age and pulmonary hypertension severity are linked to septitis grade, suggesting its central role in prognosis.
Abstract:
Lung biopsy specimens were taken from 39 infants younger than 12 months of age with congenital heart disease and severe pulmonary hypertension (Pp/Ps greater than or equal to 0.75) accompanied by respiratory distress. The pathological change in lung specimens and clinical courses were compared. These 39 infants underwent surgical treatment of patent ductus arteriosus (PDA), seven patients; ventricular septal defect (VSD), 13 patients; and complex heart anomaly, 19 patients. The common pathological findings of the lung specimens taken from these infants were lymphoid cellular infiltration and thickening of the alveolar septum, which we have called "septitis" in the present study. In most cases pulmonary vascular obstructive change was within Grade 2 of the Health-Edwards criteria. Septitis was classified into three categories: mild, moderate, and severe. Only three of the 19 infants with severe septitis survived postoperatively, whereas 10 of the 12 infants with moderate septitis and all eight with mild septitis could be successfully weaned. The cause of septitis remains unidentified. We have found the patient's age and pulmonary hypertension to be closely related to the grade of septitis in this study. Septitis plays a much more important role than pulmonary vascular obstructive change in the prognosis of pulmonary hypertensive heart disease in early infancy.