Abstract 16456: Is Trisomy 21 a Risk Factor for Rapid Progression of Pulmonary Arteriopathy? -Revisit Histopathological Characteristics Based on 282 Lung Biopsy Specimens
Background: Pulmonary hypertension (PH) is clinically known to be more progressive in trisomy 21 patients than non-trisomy 21, the same seems to be applicable to pulmonary arteriopathy. However, pulmonary arteriopathic lesions in trisomy 21 patients have not been fully characterized histopathologically.
Methods: A retrospective review of our lung biopsy registry identified 282 patients diagnosed as having ventricular septal defect or atrioventricular septal defect (AVSD). 188 patients were trisomy 21 (group D), and the remaining 94 were non-trisomy 21(group N). Preoperative catheterization data and postoperative histopathological data were collected. We evaluated the degree of progression of pulmonary arteriopathy using the previously established histomorphometrical scoring system, the index of pulmonary vascular disease (IPVD).
Results: In comparisons of group D versus N, age at lung biopsy was 3 and 7 months (D vs. N, p < 0.0001). The proportions of patients diagnosed with AVSD were 42% and 12%, respectively (p < 0.0001). Preoperative catheterization revealed pulmonary arterial pressure (PAP) to be 70.5 ± 13.4 mmHg vs. 72.7 ± 18.8 mmHg (p = 0.36) and pulmonary vascular resistance to be 6.5 ± 3.8 unit m2 vs 6.9 ± 4.8 unit m2 (p = 0.46). No difference was observed in the proportion of patients assessed as having irreversible intimal lesions (22.9% vs 24.5%, p = 0.77). IPVDs were essentially the same between the groups (1.05 vs 1.1, p = 0.09). When the lung specimens retrieved at the age of 1 year were compared, irreversible intimal lesions were more frequently detected in group D than in group N (42% vs. 7%, p = 0.047). However, after propensity score matching of patients’ backgrounds (n = 56 in each group), no difference was observed in IPVD (p = 0.86) and the ratio of irreversible intimal changes between groups (p = 0.36). Furthermore, multivariate analysis identified age (p < 0.0001) and PAP (p = 0.0009) as the only risk factors for pulmonary arteriopathy.
Conclusions: Accelerated progression of pulmonary arteriopathy per se may not be directly associated with a chromosomal anomaly of trisomy 21. Factors other than pulmonary arteriopathy might have an impact on the pronounced progression of clinical PH in trisomy 21 patients.
Author Disclosures: N. Masaki: None. Y. Saiki: None. M. Endo: None. K. Maeda: None. K. Watanabe: None. S. Takahara: None. K. Sakatsume: None. K. Hosoyama: None. Y. Hayatsu: None. S. Kawatsu: None. I. Yoshioka: None. S. Masuda: None. K. Kumagai: None. O. Adachi: None. M. Akiyama: None. S. Kawamoto: None. Y. Saiki: None.
- © 2016 by American Heart Association, Inc.