Koh Y, Imai Y, Kurosawa H, Sawatari K, Kawada M, Matsuo K, Takeuchi K, Terada M, Yamagishi M, Nagatsu M
Department of Pediatric Cardiovascular Surgery, Heat Institute of Japan, Tokyo Women's Medical College, Japan.
Nihon Kyobu Geka Gakkai Zasshi. 1993 Mar;41(3):409-16.
Between 1986 and 1990, fourteen patients with univentricular atrioventricular connection and subaortic stenosis underwent surgical treatment. The patients consisted of 7 cases of double inlet left ventricle, 4 with double inlet right ventricle, 3 with tricuspid atresia. The palliative operation was performed in 6 infants ranging in age from 17 days to 6 months. Four patients with mild subaortic stenosis underwent pulmonary artery banding, in two patients this was combined with repair of coarctation of the aorta. Two patients with severe subaortic stenosis underwent the Norwood operation. There were no operative deaths. One of two patients who underwent the Fontan operation 2 years after the Norwood operation died later. The definitive operation was performed in 8 children ranging in age from 4 to 11 years. Five of these 8 patients had previous pulmonary artery banding. Five children with double inlet left ventricle underwent septation combined with enlargement of bulboventricular foramen. Postoperatively all remained in sinus rhythm and had no pressure gradient between Aorta and left ventricle. A Fontan operation combined with a Damus operation was performed in 2 children, 1 of double inlet right ventricle and 1 of tricuspid atresia. In both cases, postoperative angiogram showed no pulmonary incompetence. One patient underwent enlargement of bulboventricular foramen after a Fontan operation. All survived later. Young infants and neonates with severe subaortic stenosis can survive by the Norwood operation. Infants with mild subaortic stenosis, although can survive by pulmonary artery banding, should be closely followed for the development of subaortic stenosis. For relief of subaortic stenosis, enlargement of bulboventricular foramen may be effective in septation.(ABSTRACT TRUNCATED AT 250 WORDS)
1986年至1990年间，14例单心室房室连接合并主动脉瓣下狭窄的患者接受了手术治疗。患者包括7例左心室双入口、4例右心室双入口、3例三尖瓣闭锁。6例年龄在17天至6个月的婴儿接受了姑息性手术。4例轻度主动脉瓣下狭窄患者接受了肺动脉环扎术，其中2例合并主动脉缩窄修复术。2例重度主动脉瓣下狭窄患者接受了诺伍德手术。无手术死亡病例。在诺伍德手术后2年接受Fontan手术的2例患者中有1例后来死亡。8例年龄在4至11岁的儿童接受了根治性手术。这8例患者中有5例曾接受过肺动脉环扎术。5例左心室双入口患儿接受了房间隔造口术并扩大球室孔。术后所有患者均维持窦性心律，主动脉与左心室之间无压力阶差。2例患儿接受了Fontan手术联合Damus手术，1例右心室双入口，1例三尖瓣闭锁。2例术后血管造影均显示无肺动脉瓣关闭不全。1例患者在Fontan手术后接受了球室孔扩大术。所有患者后来均存活。患有重度主动脉瓣下狭窄的婴幼儿可通过诺伍德手术存活。患有轻度主动脉瓣下狭窄的婴儿，虽然可通过肺动脉环扎术存活，但应密切随访主动脉瓣下狭窄的进展情况。为缓解主动脉瓣下狭窄，扩大球室孔在房间隔造口术中可能有效。（摘要截断于250字）
