Is Trisomy 21 a Risk Factor for Rapid Progression of Pulmonary Arteriopathy? ― Revisiting Histopathological Characteristics Using 282 Lung Biopsy Specimens
Background:Pulmonary hypertension (PH) is more progressive in trisomy 21 patients. However, pulmonary arteriopathic lesions in these patients have not been fully characterized histopathologically.Methods and Results:A retrospective review of a lung biopsy registry identified 282 patients: 188 patien...
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| Published in | Circulation Journal Vol. 82; no. 6; pp. 1682 - 1687 |
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| Main Authors | , , , , , , , |
| Format | Journal Article |
| Language | English |
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The Japanese Circulation Society
25.05.2018
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| Abstract | Background:Pulmonary hypertension (PH) is more progressive in trisomy 21 patients. However, pulmonary arteriopathic lesions in these patients have not been fully characterized histopathologically.Methods and Results:A retrospective review of a lung biopsy registry identified 282 patients: 188 patients with trisomy 21 (Group D) and 94 without (Group N). The mean age at lung biopsy was 3 and 7 months (P<0.0001). Pulmonary arterial pressure (PAP) and pulmonary vascular resistance were similar between the 2 groups. There were no significant differences in the proportion of patients with irreversible intimal lesions or the index of pulmonary vascular disease (IPVD; a measure of the degree of pulmonary arteriopathy progression) between the 2 groups. In addition, after propensity score matching for patient background (n=43 in each group), there were no significant differences in IPVD (P=0.29) or the ratio of irreversible intimal changes between the D and N groups (P=0.39). Multivariate analysis identified age (P<0.0001) and PAP (P=0.03) as the only risk factors for progression of pulmonary arteriopathy.Conclusions:Histopathologically, early progression of pulmonary arteriopathy in patients with trisomy 21 was not proved compared with patients without trisomy 21. Although we cannot exclude the possibility of bias in the Group D and N patients who were slated for lung biopsy, factors other than pulmonary arteriopathy may affect the marked progression of clinical PH in trisomy 21 patients. |
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| AbstractList | Pulmonary hypertension (PH) is more progressive in trisomy 21 patients. However, pulmonary arteriopathic lesions in these patients have not been fully characterized histopathologically.BACKGROUNDPulmonary hypertension (PH) is more progressive in trisomy 21 patients. However, pulmonary arteriopathic lesions in these patients have not been fully characterized histopathologically.A retrospective review of a lung biopsy registry identified 282 patients: 188 patients with trisomy 21 (Group D) and 94 without (Group N). The mean age at lung biopsy was 3 and 7 months (P<0.0001). Pulmonary arterial pressure (PAP) and pulmonary vascular resistance were similar between the 2 groups. There were no significant differences in the proportion of patients with irreversible intimal lesions or the index of pulmonary vascular disease (IPVD; a measure of the degree of pulmonary arteriopathy progression) between the 2 groups. In addition, after propensity score matching for patient background (n=43 in each group), there were no significant differences in IPVD (P=0.29) or the ratio of irreversible intimal changes between the D and N groups (P=0.39). Multivariate analysis identified age (P<0.0001) and PAP (P=0.03) as the only risk factors for progression of pulmonary arteriopathy.METHODS AND RESULTSA retrospective review of a lung biopsy registry identified 282 patients: 188 patients with trisomy 21 (Group D) and 94 without (Group N). The mean age at lung biopsy was 3 and 7 months (P<0.0001). Pulmonary arterial pressure (PAP) and pulmonary vascular resistance were similar between the 2 groups. There were no significant differences in the proportion of patients with irreversible intimal lesions or the index of pulmonary vascular disease (IPVD; a measure of the degree of pulmonary arteriopathy progression) between the 2 groups. In addition, after propensity score matching for patient background (n=43 in each group), there were no significant differences in IPVD (P=0.29) or the ratio of irreversible intimal changes between the D and N groups (P=0.39). Multivariate analysis identified age (P<0.0001) and PAP (P=0.03) as the only risk factors for progression of pulmonary arteriopathy.Histopathologically, early progression of pulmonary arteriopathy in patients with trisomy 21 was not proved compared with patients without trisomy 21. Although we cannot exclude the possibility of bias in the Group D and N patients who were slated for lung biopsy, factors other than pulmonary arteriopathy may affect the marked progression of clinical PH in trisomy 21 patients.CONCLUSIONSHistopathologically, early progression of pulmonary arteriopathy in patients with trisomy 21 was not proved compared with patients without trisomy 21. Although we cannot exclude the possibility of bias in the Group D and N patients who were slated for lung biopsy, factors other than pulmonary arteriopathy may affect the marked progression of clinical PH in trisomy 21 patients. Pulmonary hypertension (PH) is more progressive in trisomy 21 patients. However, pulmonary arteriopathic lesions in these patients have not been fully characterized histopathologically. A retrospective review of a lung biopsy registry identified 282 patients: 188 patients with trisomy 21 (Group D) and 94 without (Group N). The mean age at lung biopsy was 3 and 7 months (P<0.0001). Pulmonary arterial pressure (PAP) and pulmonary vascular resistance were similar between the 2 groups. There were no significant differences in the proportion of patients with irreversible intimal lesions or the index of pulmonary vascular disease (IPVD; a measure of the degree of pulmonary arteriopathy progression) between the 2 groups. In addition, after propensity score matching for patient background (n=43 in each group), there were no significant differences in IPVD (P=0.29) or the ratio of irreversible intimal changes between the D and N groups (P=0.39). Multivariate analysis identified age (P<0.0001) and PAP (P=0.03) as the only risk factors for progression of pulmonary arteriopathy. Histopathologically, early progression of pulmonary arteriopathy in patients with trisomy 21 was not proved compared with patients without trisomy 21. Although we cannot exclude the possibility of bias in the Group D and N patients who were slated for lung biopsy, factors other than pulmonary arteriopathy may affect the marked progression of clinical PH in trisomy 21 patients. Background:Pulmonary hypertension (PH) is more progressive in trisomy 21 patients. However, pulmonary arteriopathic lesions in these patients have not been fully characterized histopathologically.Methods and Results:A retrospective review of a lung biopsy registry identified 282 patients: 188 patients with trisomy 21 (Group D) and 94 without (Group N). The mean age at lung biopsy was 3 and 7 months (P<0.0001). Pulmonary arterial pressure (PAP) and pulmonary vascular resistance were similar between the 2 groups. There were no significant differences in the proportion of patients with irreversible intimal lesions or the index of pulmonary vascular disease (IPVD; a measure of the degree of pulmonary arteriopathy progression) between the 2 groups. In addition, after propensity score matching for patient background (n=43 in each group), there were no significant differences in IPVD (P=0.29) or the ratio of irreversible intimal changes between the D and N groups (P=0.39). Multivariate analysis identified age (P<0.0001) and PAP (P=0.03) as the only risk factors for progression of pulmonary arteriopathy.Conclusions:Histopathologically, early progression of pulmonary arteriopathy in patients with trisomy 21 was not proved compared with patients without trisomy 21. Although we cannot exclude the possibility of bias in the Group D and N patients who were slated for lung biopsy, factors other than pulmonary arteriopathy may affect the marked progression of clinical PH in trisomy 21 patients. |
| Author | Masaki, Naoki Adachi, Osamu Saiki, Yoshikatsu Saiki, Yuriko Endo, Masato Akiyama, Masatoshi Maeda, Kay Kawamoto, Shunsuke |
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| Cites_doi | 10.1161/01.CIR.18.4.533 10.1164/ajrccm.163.3.2004231 10.1161/01.CIR.54.5.805 10.1016/j.ijcard.2011.07.009 10.1016/S0022-3476(75)80142-9 10.1016/0002-9149(83)90665-3 10.1001/archotol.1996.01890210025007 10.1111/ped.12349 10.1016/S0003-4975(98)00776-0 10.1155/2015/230846 10.1056/NEJM198211043071902 10.1016/j.jpeds.2010.07.023 10.1542/peds.58.6.893 10.1002/ajmg.a.36780 10.1002/mrdd.20157 10.1007/s11748-012-0155-7 10.1016/0167-5273(87)90295-6 |
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| References_xml | – reference: 6. Yamaki S. Pulmonary vascular disease associated with pulmonary hypertension in 445 patients: Diagnosis from lung biopsy and autopsy. Gen Thorac Cardiovasc Surg 2013; 61: 24–31. – reference: 17. Jacobs IN, Gray RF, Todd NW. Upper airway obstruction in children with Down syndrome. Arch Otolaryngol Head Neck Surg 1996; 122: 945–950. – reference: 3. Chi TPL, Krovetz J. The pulmonary vascular bed in children with Down syndrome. J Pediatr 1975; 86: 533–538. – reference: 16. McDowell KM, Craven DI. Pulmonary complications of Down syndrome during childhood. J Pediatr 2011; 158: 319–325. – reference: 12. Yamaki S, Horiuchi T, Sekino Y. Quantitative analysis of pulmonary vascular disease in simple cardiac anomalies with the Down syndrome. Am J Cardiol 1983; 51: 1502–1506. – reference: 13. Frescura C, Thiene G, Franceschini E, Talenti E, Mazzucco A. Pulmonary vascular disease in infants with complete atrioventricular septal defect. Int J Cardiol 1987; 15: 91–103. – reference: 4. Soudon P, Stijns M, Tremouroux-Wattiez M, Vliers A. Precocity of pulmonary vascular obstruction of Down’s syndrome. Eur J Cardiol 1975; 2: 473–476. – reference: 5. Greenwood RD, Nadas AS. The clinical course of cardiac disease in Down’s syndrome. Pediatrics 1976; 58: 893–897. – reference: 15. Cooney TP, Thurlbeck WM. Pulmonary hypoplasia in Down’s syndrome. N Engl J Med 1982; 307: 1170–1173. – reference: 11. Saji T. Clinical characteristics of pulmonary arterial hypertension associated with Down syndrome. Pediatr Int 2014; 56: 297–303. – reference: 14. D’Alto M, Romeo E, Argiento P, D’Andrea A, Sarubbi B, Correra A, et al. Therapy for pulmonary arterial hypertension due to congenital heart disease and Down’s syndrome. Int J Cardiol 2013; 164: 323–326. – reference: 1. Morris JK, Garne E, Wellesley D, Addor MC, Arriola L, Barisic I, et al. Major congenital anomalies in babies born with Down syndrome: A EUROCAT population-based registry study. Am J Med Genet A 2014; 164A: 2979–2986. – reference: 8. Heath D, Edwards JE. The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation 1958; 18: 533–547. – reference: 2. Sherman SL, Allen EG, Bean LH, Freeman SB. Epidemiology of Down syndrome. Ment Retard Dev Disabil Res Rev 2007; 13: 221–227. – reference: 10. Yamaki S, Abe A, Endo M, Tanaka T, Tabayashi K, Takahashi T. Surgical indication for congenital heart disease with extremely thickened media of small pulmonary arteries. Ann Thorac Surg 1998; 66: 1560–1564. – reference: 18. Uong EC, McDonough JM, Tayag-Kier CE, Zhao H, Haselgrove J, Mahboubi S, et al. Magnetic resonance imaging of the upper airway in children with Down syndrome. Am J Respir Crit Care Med 2001; 163: 731–736. – reference: 7. Maeda K, Saiki Y, Yamaki S. In situ thrombosis of small pulmonary arteries in pulmonary hypertension developing after chemotherapy for malignancy. Pulm Med 2015; 2015: 230846. – reference: 9. Yamaki S, Tezuka F. Quantitative analysis of pulmonary vascular disease in complete transposition of the great arteries. Circulation 1976; 54: 805–809. – ident: 8 doi: 10.1161/01.CIR.18.4.533 – ident: 4 – ident: 18 doi: 10.1164/ajrccm.163.3.2004231 – ident: 9 doi: 10.1161/01.CIR.54.5.805 – ident: 14 doi: 10.1016/j.ijcard.2011.07.009 – ident: 3 doi: 10.1016/S0022-3476(75)80142-9 – ident: 12 doi: 10.1016/0002-9149(83)90665-3 – ident: 17 doi: 10.1001/archotol.1996.01890210025007 – ident: 11 doi: 10.1111/ped.12349 – ident: 10 doi: 10.1016/S0003-4975(98)00776-0 – ident: 7 doi: 10.1155/2015/230846 – ident: 15 doi: 10.1056/NEJM198211043071902 – ident: 16 doi: 10.1016/j.jpeds.2010.07.023 – ident: 5 doi: 10.1542/peds.58.6.893 – ident: 1 doi: 10.1002/ajmg.a.36780 – ident: 2 doi: 10.1002/mrdd.20157 – ident: 6 doi: 10.1007/s11748-012-0155-7 – ident: 13 doi: 10.1016/0167-5273(87)90295-6 – reference: 29760348 - Circ J. 2018 May 25;82(6):1513-1514. doi: 10.1253/circj.CJ-18-0471 |
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| Snippet | Background:Pulmonary hypertension (PH) is more progressive in trisomy 21 patients. However, pulmonary arteriopathic lesions in these patients have not been... Pulmonary hypertension (PH) is more progressive in trisomy 21 patients. However, pulmonary arteriopathic lesions in these patients have not been fully... |
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| SubjectTerms | Age Factors Atrioventricular septal defect Biopsy Blood Pressure Disease Progression Down Syndrome - complications Female Humans Hypertension Infant Male Pulmonary arteriopathy Pulmonary Artery - pathology Pulmonary Artery - physiopathology Retrospective Studies Risk Factors Trisomy 21, Ventricular septal defect Vascular Calcification Vascular Resistance |
| Title | Is Trisomy 21 a Risk Factor for Rapid Progression of Pulmonary Arteriopathy? ― Revisiting Histopathological Characteristics Using 282 Lung Biopsy Specimens |
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