Key result
Surgical closure of PDA and VSD in a 37-year-old woman with Noonan's syndrome eliminated her susceptibility to infections, although her cardiac function did not significantly improve.
Why the study?
Patent ductus arteriosus is extremely rare in Noonan syndrome patients without pulmonary stenosis, and the effect of radical surgery in such cases is uncertain.
Case Report (n=1)
In a rare case of Noonan syndrome with PDA, VSD, and dilated-type cardiomyopathy, radical surgery resolved susceptibility to infection but did not clearly improve heart failure symptoms, highlighting the need for careful consideration of surgical indications.
Noonan 症候群は比較的希な疾患で肺動脈狭窄 (以下PS) を伴わず, 動脈管開存症 (以下PDA) を伴うものは極めて希である. 今回著者らは, 37歳女性の Noonan 症候群のPDAおよび心室中隔欠損 (以下VSD) の根治手術例を経験した. 本症候群に肥大型心筋症 (以下HCM) もあると報告されているが, 自験例は拡張型の様相をした心筋症を呈し, 典型的なHCMは認められなかった. 術後の心不全の軽減や臨床症状の改善は明らかには認められなかったが易感染性は消失した. 本例の根治手術の効果の不確かさは心筋症の関与を示唆し, 手術適応についての検討と今後の観察が必要であると思われた.
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市原 et al. (1999) conducted a case report in Noonan's syndrome with patent ductus arteriosus, ventricular septal defect, and cardiomyopathy (n=1). Surgical closure of PDA and VSD was evaluated on Cardiac function and clinical symptoms. Surgical closure of PDA and VSD in a 37-year-old woman with Noonan's syndrome eliminated her susceptibility to infections, although her cardiac function did not significantly improve.
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