Babies with the congenital heart defect hypoplastic left heart syndrome receive specialized care from our expert team.

患有左心发育不全综合征(HLHS)的婴儿——一种罕见的, complex heart defect that is present at birth — need comprehensive care from an experienced team to meet their unique needs throughout their treatment.

我们治疗HLHS的方法

圣霍普金斯大学所有儿童心脏研究所的研究小组. 彼得堡, 佛罗里达, 包括专业的儿科心脏病专家, 心脏外科医生, 胎儿心脏专家, 心脏重症监护医师等为您的宝宝提供专家, 从诊断到治疗的关爱, 跟踪照顾到成年. 

左心发育不良综合征(HLHS), the left side of the heart is critically underdeveloped and cannot effectively pump blood to the body, leaving the right side of the heart to pump blood to both the lungs and the rest of the body. 在HLHS中，左下腔可能太小，或者根本没有发育. 心脏左侧的瓣膜也不能正常工作, 离开心脏的大动脉比正常情况要小.

所有的婴儿出生时都有动脉导管未闭, which is an opening between the pulmonary artery and the aorta that allows blood to flow directly from the heart to the lungs. This typically closes after birth but babies with HLHS are given medication to prevent this closure because the PDA helps provide additional blood flow to help keep them stable before surgery. 

Hypoplastic left heart syndrome may be diagnosed before your baby is born with a fetal echocardiogram. A fetal echocardiogram is a type of ultrasound used to take pictures of a baby’s heart before birth to diagnose various types of congenital heart defects. 如果你的产科医生在超声检查后对宝宝的心脏有担忧, 你可能会被推荐给专科医生做胎儿超声心动图检查.

在约翰霍普金斯儿童医院，我们的 胎儿心脏计划 team provides expert diagnosis using fetal echocardiogram as well as management before birth of congenital heart defects like hypoplastic left heart syndrome.

有时婴儿在出生后才被诊断出来. Babies with HLHS are usually seriously ill right after birth and will have lower oxygen levels. 婴儿将接受超声心动图检查，以确认HLHS的诊断. 超声心动图是一种超声波，可以拍摄婴儿心脏的运动图像.

左心发育不良综合征的治疗

所有患有HLHS的婴儿都需要在不同的时间做三次手术. 这被称为“分阶段重建”.“修复HLHS的手术是诺伍德手术, 格伦手术, 和方丹程序. 这些手术是由我们委员会认证的心脏外科医生进行的. 了解更多关于心脏手术团队的信息.

诺伍德手术

第一个手术是诺伍德手术，在出生后的第一周内进行. In babies with HLHS, the left ventricle and aorta are too small to pump blood to the body. 诺伍德手术是为了让右心室能够将血液泵入身体. This involves rebuilding the aorta by joining it with the bottom part of the pulmonary artery and placing a shunt that will take blood to the lungs.

在诺伍德手术后，你宝宝的氧含量仍然会低于正常水平, 但这个过程有助于稳定他们的氧气水平. The goal for patients after the 诺伍德手术 is to be able to eat well enough to gain weight.

Some babies may have difficulty eating and may need a feeding tube to help them get enough calories to grow. 如果需要的话, your baby’s care team will discuss with you the type of feeding tube that best meets your child’s needs and how to care for them with the tube at home. 在诺伍德手术之后, your baby will see our team for follow-up visits typically every two weeks until their second surgery.

格伦过程

格伦手术是修复HLHS的第二次手术. 这种手术通常在患者4-6个月大时进行. Before surgery your baby will have a cardiac MRI or catheterization procedure so that doctors can examine how your baby’s heart is functioning to make sure they are ready for surgery. 用于导管插入术, 很长一段, 被称为导管的细管被引导穿过血管来检查心脏. 这些程序由我们的团队执行 介入心脏病学项目.

格伦手术, the surgeon will disconnect the superior vena cava (which usually carries blood back to the heart from the head, 武器, and upper body) from the heart and connect it to the branch pulmonary arteries so that blood will now flow from the upper body directly to the lungs. 这使得单心室不需要那么辛苦地工作. 格伦手术期间, 外科医生也会移除在诺伍德手术中放置的分流器.

而婴儿的氧含量会继续异常, 格伦手术进一步稳定了血液流向肺部的方式.

Fontan过程

修复HLHS的第三种手术是Fontan手术, 这通常是在孩子大一点的时候做的, 2-5岁, 这取决于你孩子的个人情况. 和格伦手术之前类似, a catheterization procedure will be performed so that doctors can make sure that your child is a good candidate for the operation.

For the Fontan过程 the surgeon will connect the inferior vena cava (a vein that carries blood from the abdomen and lower body back to the heart, 这样它就可以进入肺部供氧)通过一根管子进入肺动脉. 外科医生通常也会在导管和右心房之间开一个小孔, 叫做开窗, 这样一些血液就会回流到心脏, 减轻肺部的压力. The Fontan过程 is done so that the single ventricle only pumps oxygenated blood to the body, so that blood with oxygen and blood without oxygen no longer mixes as it flows through the body.

在手术间隙照顾你的孩子

每次手术后，您的宝宝将由医院的团队照顾 心血管重症监护病房(CVICU). CVICU团队包括儿科心脏病专家, 心脏外科医生, 心脏重症监护医师, respiratory therapists and other pediatric experts who care for our patients after surgery.

在家的风险最高的时间是在第一次和第二次手术之间. Your baby will be monitored by the team in our Cardiac High Risk Monitoring Program during this period. The program provides seamless coordination of care and a consistent point of contact for your family during this time.

We provide families with education and technology so that they can track their baby’s health and progress between visits, allowing our team to receive real-time updates about your baby’s vital signs such as oxygen levels, 喂奶, 以及其他重要信息. Families also have access to the team through a 24-hour dedicated phone line for any questions or concerns that may arise. 了解更多关于心脏高危监测项目.

后续护理

第三次手术后, 患有HLHS的儿童将在其一生中继续与心脏病专家进行随访. 丰坦法是为了尽可能提高孩子的氧含量, 但是血液流经身体的方式仍然是不正常的. 他们的氧气水平需要在他们的一生中被监测, 患有HLHS的儿童在以后的生活中可能会出现并发症.

他们通常一生都在服药, 他们以后可能需要额外的手术, 在某些情况下包括心脏移植. Other long-term complications may include issues with the liver or swelling or the buildup of fluid in the abdomen or extremities. Johns Hopkins All Children’s Hospital has a Fontan clinic to provide specialized care for children who have had this procedure.

Patients are also recommended to have developmental evaluations between ages 9-12 months and may need care from other specialists later. Our team can refer your child to other specialists throughout Johns Hopkins All Children’s as needed.

儿科患者也可以像成人一样继续接受我们的专家的护理 成人先天性心脏病计划. Adults who were treated for HLHS as children benefit from seeing a provider who is familiar with the Fontan过程 in particular and understands how congenital heart defects can impact health long term.