日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

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.