Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
Pediatric Cardiology and Cardiac Surgery 37(1): 44-50 (2021)

症例報告Case Report

肥大型心筋症を合併した心房中隔欠損症に対して開窓付閉鎖術が奏功した1例Fenestrated Closure of an Atrial Septal Defect for Left Ventricular Diastolic Dysfunction in an Early Infant with Hypertrophic Cardiomyopathy

1兵庫県立こども病院 循環器内科Departments of Cardiology, Hyogo Prefectural Kobe Children’s Hospital ◇ Hyogo, Japan

2兵庫県立こども病院 小児集中治療科Pediatric Critical Care Medicine, Hyogo Prefectural Kobe Children’s Hospital ◇ Hyogo, Japan

受付日:2020年3月26日Received: March 26, 2020
受理日:2020年9月9日Accepted: September 9, 2020
発行日:2021年4月1日Published: April 1, 2021

心房中隔欠損(ASD)はまれに乳児期早期に重症化することがある.また肥大型心筋症(HCM)は左室拡張障害を伴い,先天性心疾患合併例においては予後不良とされる.今回HCMを合併したASDに対して乳児期早期に治療介入が必要と判断し,開窓付閉鎖術が有効であったので報告する.症例は基礎疾患にCardio-Facio-Cutaneous症候群,心疾患にASD, HCMをもつ5か月女児で,発熱と痙攣を主訴に受診し,左室拡張障害による肺うっ血に伴う循環不全と診断した.利尿剤投与のみでは改善せず,一酸化窒素吸入によって右心系縮小と左心系拡大が得られて改善したため,肺高血圧症の急性増悪が心室間連関による右室拡大に伴う左室圧排を引き起こし,左室拡張障害の増悪による低心拍出性心不全を来したと考察した.入院18日目に施行した心臓カテーテル検査では,肺体血流比(Qp/Qs)=6.1/2.4=2.6,肺血管抵抗2.1 Wood unit·m2を認め,ASDを介する高度な左右短絡も低心拍出量の一因と考えられたため,4 mm開窓付ePTFEパッチによるASD部分閉鎖を施行して左右短絡の減少を図った.術後評価カテーテル検査では,左房圧は許容範囲で,左右短絡減少と心拍出量増加を認めて良好な血行動態を示した.また,心房中隔開窓部の閉鎖試験により左室拡張末期圧の上昇を認めたことから開窓は適切であると評価した.左室拡張障害を伴うASDは早期に治療介入が必要となることがあり,そうした場合に開窓付ASD閉鎖術は安全かつ有効であると考える.

Hypertrophic cardiomyopathy (HCM) is generally accompanied with left ventricular diastolic dysfunction, leading to a poor prognosis in patients with congenital heart diseases. An atrial septal defect (ASD) rarely needs surgical intervention in infancy. However, we report a case of HCM-complicated ASD that required fenestrated ASD closure in early infancy. A 5-month-old girl with cardiofaciocutaneous syndrome was transferred to our institution because of severe lung congestion triggered by fever and convulsions. Echocardiography revealed right ventricle enlargement secondary to left ventricular diastolic dysfunction and severe pulmonary hypertension. She was intubated and supported with mechanical ventilation, with nitric oxide inhalation. Immediately, her ventricular interdependence and circulatory dynamics improved. On hospitalization day 18, cardiac catheterization showed pulmonary hypertension improvement with high pulmonary blood flow and low cardiac output. However, she had severe lung congestion triggered by a repeat convulsion on day 60. Thus, we performed ASD partial closure with 4 mm fenestration. Postoperative cardiac catheterization showed a good control of pulmonary blood flow, with a Qp/Qs of 1.3. In conclusion, fenestrated ASD closure is a safe and effective treatment option for left ventricular diastolic dysfunction in early infants with ASD and HCM.

Key words: atrial septal defect; hypertrophic cardiomyopathy; diastolic dysfunction; fenestration

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