这些缺陷包括发育异常和/或狭窄的肺动脉瓣、肥厚型心肌病、间隔缺损及法洛四联症。[30]Shaw AC, Kalidas K, Crosby AH, et al. The natural history of Noonan syndrome: a long-term follow-up study. Arch Dis Child. 2007 Feb;92(2):128-32.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2083343/http://www.ncbi.nlm.nih.gov/pubmed/16990350?tool=bestpractice.com[52]Burch M, Sharland M, Shinebourne E, et al. Cardiologic abnormalities in Noonan syndrome: phenotypic diagnosis and echocardiographic assessment of 118 patients. J Am Coll Cardiol. 1993 Oct;22(4):1189-92.http://www.sciencedirect.com/science/article/pii/0735109793904365?via%3Dihubhttp://www.ncbi.nlm.nih.gov/pubmed/8409059?tool=bestpractice.com[53]Ishizawa A, Oho S, Dodo H, et al. Cardiovascular abnormalities in Noonan syndrome: the clinical findings and treatments. Acta Paediatr Jpn. 1996 Feb;38(1):84-90.http://www.ncbi.nlm.nih.gov/pubmed/8992869?tool=bestpractice.com[54]Noonan JA, O'Connor W. Noonan syndrome: a clinical description emphasizing the cardiac findings. Acta Paediatr Jpn. 1996 Feb;38(1):76-83.http://www.ncbi.nlm.nih.gov/pubmed/8992867?tool=bestpractice.com
诊断时需要进行全面的心血管评估。应以发现的特殊异常来指导随后的治疗。
球囊瓣膜成形术可能对肺动脉瓣狭窄和/或发育不良有效，但可能需要瓣膜置换术。[53]Ishizawa A, Oho S, Dodo H, et al. Cardiovascular abnormalities in Noonan syndrome: the clinical findings and treatments. Acta Paediatr Jpn. 1996 Feb;38(1):84-90.http://www.ncbi.nlm.nih.gov/pubmed/8992869?tool=bestpractice.com 肥厚型心肌病可通过 β-受体阻滞剂或钙通道阻滞剂进行治疗，但偶尔需要进行外科肌瘤切除术。[53]Ishizawa A, Oho S, Dodo H, et al. Cardiovascular abnormalities in Noonan syndrome: the clinical findings and treatments. Acta Paediatr Jpn. 1996 Feb;38(1):84-90.http://www.ncbi.nlm.nih.gov/pubmed/8992869?tool=bestpractice.com[54]Noonan JA, O'Connor W. Noonan syndrome: a clinical description emphasizing the cardiac findings. Acta Paediatr Jpn. 1996 Feb;38(1):76-83.http://www.ncbi.nlm.nih.gov/pubmed/8992867?tool=bestpractice.com
心脏治疗护理应持续至成年，因为与该缺陷相关的并发症发病率和死亡率可持续存在。[30]Shaw AC, Kalidas K, Crosby AH, et al. The natural history of Noonan syndrome: a long-term follow-up study. Arch Dis Child. 2007 Feb;92(2):128-32.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2083343/http://www.ncbi.nlm.nih.gov/pubmed/16990350?tool=bestpractice.com[53]Ishizawa A, Oho S, Dodo H, et al. Cardiovascular abnormalities in Noonan syndrome: the clinical findings and treatments. Acta Paediatr Jpn. 1996 Feb;38(1):84-90.http://www.ncbi.nlm.nih.gov/pubmed/8992869?tool=bestpractice.com
