[Septal Myectomy for Obstructive Hypertrophic Cardiomyopathy Due to Noonan Syndrome;Report of a Case].

A 15-year-old boy with Noonan syndrome, who had been diagnosed with hypertrophic cardiomyopathy (HCM) at 4 and treated by drugs, was referred to our hospital because of progression of left ventricular outflow tract obstruction (LVOTO). Transthoracic echocardiogram (TTE) revealed left ventricular hypertrophy( LVH), LVOTO( systolic gradient:102 mmHg), and systolic anterior motion of the mitral valve( SAM) with mild mitral regurgitation(MR). We performed septal myectomy by transaortic and transapical approaches. Postoperative TTE revealed significant reduction in left ventricular outflow tract (LVOT) gradient (9 mmHg), resolution of SAM, and reduction in MR grade. The postoperative course was uneventful except for transient atrial tachyarrhythmia. Myectomy for HCM in a patient with Noonan syndrome is rare. This case suggests that myectomy is useful for the patients with Noonan syndrome.

[Aorta-left Main Trunk Interposition for Adult Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Using a Prosthetic Graft;Report of a Case].

A 70-year-old woman with back pain was diagnosed with adult anomalous origin of the left coronary artery from the pulmonary artery [Bland-White-Garland (BWG) syndrome]. Preoperative transthoracic echocardiography showed diffuse hypokinesia of the left ventricle and mild mitral valve regurgitation. Coronary angiography revealed dilatation of both coronary arteries, but no aneurysms. The left coronary ostium was removed as buttons of the pulmonary arterial wall, and a prosthetic graft (8 mm ePTFE graft) was interposed between the ascending aorta and coronary button. The pulmonary arterial wall was repaired with a bovine pericardium. Postoperative angiography showed good flow of the graft. The antegrade flow provided by this simple technique will allow feasible percutaneous coronary intervention in the future.

Three Year Follow-Up of a Vein Patch Repair for a Coronary Artery Saccular Aneurysm of the Left Main Bifurcation.

This report describes a case of surgical treatment for a coronary artery saccular aneurysm of the left main bifurcation. A coronary artery saccular aneurysm (7 mm × 10 mm) and three vessel disease, including the left main trunk, were diagnosed by coronary angiography. A surgical resection and saphenous vein patch repair of the left main coronary artery aneurysm was performed concomitantly with coronary artery bypass grafting. The pathological findings of the aneurysm clarified that the aneurysm wall was atrophic and extremely thin because of a collapsed trilaminar arterial structure due to atherosclerosis. A coronary computed tomographic scan revealed no aneurysmal formation in the patent left main trunk and patent grafts 3 years after surgery.