l-Transposition of the Great Arteries

L-TGA diagram

What is it?

A heart in which the lower section is fully reversed.

心脏的这种畸形导致正常血流模式的逆转，因为心脏的左右下腔颠倒了. The I-transposition, however, is less dangerous than a d-transposition because the great arteries are also reversed. 这种“双重逆转”使身体仍然接受富氧血液，而肺部仍然接受贫氧血液.

More information for parents of children with l-transposition

What causes it?

The cause is unknown, but genetic factors may contribute to it.

How does it affect the heart?

In this condition, 血液正常流通，但右心室必须在高于正常的压力下泵血. The right ventricular function may decline over time.

How does it affect my child?

Babies born with l-transposition usually aren't blue. The congenital heart defect may go undetected for a long time. 可能直到成年后充血性心力衰竭才会被诊断出来, heart murmurs and abnormal heart rhythms can develop. When there is a ventricular septal defect 和肺动脉瓣阻塞，婴儿可能脸色发青，经常听到杂音.

Is surgery needed?

大多数没有室间隔缺损或肺动脉瓣阻塞的儿童不需要手术. 有这些问题的儿童可能需要手术来关闭这个洞, relieve the blockage of blood flow to the lungs, and in some cases repair or replace the leaky tricuspid valve. In patients with VSDs, 可能建议进行更复杂的外科手术，称为“双开关”，将富氧血液重新输送到左心室和主动脉，将贫氧血液重新输送到右心室和肺动脉.

Problems your child may have may have

Patients may have problems with heart muscle, tricuspid valve leakage or heart rhythm as they enter adulthood, whether or not they had prior surgery. Like patients with atrial repair of d-TGA, the right ventricle may weaken, leading to congestive heart failure. Abnormally slow heart rhythms may cause fainting or fatigue. The most worrisome slow heart rhythm, complete heart block, is common in l-transposition. Rapid heart rhythms are less common than in d-TGA patients.

Ongoing Care

What will my child need in the future?

Medications to help the heart pump better, 控制液体积聚(利尿剂)和控制血压可能有助于缓解充血性心力衰竭症状. 在心律异常缓慢的情况下可能需要使用起搏器. 如果你正在接受任何类型的非心脏手术或侵入性手术，你也应该咨询心脏病专家在照顾患有先天性心脏病的成年人方面的专业知识.

Medical Follow up

常规检查包括心电图、超声心动图、动态心电图和压力测试. 你的心脏病专家可能会建议你做其他检查，比如核磁共振或心导管检查.

Activity Restrictions

Many patients don't need to limit their activity, 除了避免像举重这样会导致血压升高的活动. However, 如果你孩子的心脏不能正常跳动或者你有心律问题, you may need to limit your activity to your endurance. 你的心脏病专家会帮助确定你的孩子是否需要限制你的活动.

Preventing Endocarditis

有l型大动脉转位的人如果有假瓣膜可能需要预防心内膜炎. In the majority of patients, 没有人工瓣膜，心脏病专家将能够确定患者在进行某些牙科手术之前是否需要继续服用常规抗生素.

Congenital Heart Defect ID sheet

More information for adults with l-transposition

What causes it?

The cause is unknown, but genetic factors may contribute to it.

How does it affect the heart?

In this condition, 血液正常流通，但右心室必须在高于正常的压力下泵血. The right ventricular function may decline over time.

How does it affect me?

When there is a ventricular septal defect 和肺动脉瓣阻塞，婴儿可能脸色发青，经常听到杂音. 除非这些问题在儿童时期得到解决，否则成年患者可能偶尔仍会忧郁.

If l-transposition was repaired in childhood what can I expect?

大多数没有室间隔缺损或肺动脉瓣阻塞的儿童不需要手术. 即使进行了修复，大多数人仍然有一个向主动脉泵血的右心室.

Can further repair be done in adulthood?

A first surgery or repeat surgery may be needed in adulthood. 例如，三尖瓣可能会渗漏，需要更换. 很少有病人能从一种叫做“双开关”的复杂手术中受益."

Problems you may have

Patients may have problems with heart muscle, 三尖瓣渗漏或心律失常时进入生命的第三、四十年, whether or not they had prior surgery. Like patients with atrial repair of d-TGA, the right ventricle may weaken, leading to congestive heart failure. Abnormally slow heart rhythms may cause fainting or fatigue. 最令人担忧的心律缓慢，完全性心脏传导阻滞，常见于l-转位. Rapid heart rhythms are less common than in d-TGA patients.

Ongoing Care

What will I need in the future?

一位在治疗成人先天性心脏病方面具有专业知识的心脏病专家应该定期对你进行评估, at least once a year and more often if you have symptoms. Medications to help the heart pump better, 控制液体积聚(利尿剂)和控制血压可能有助于缓解充血性心力衰竭症状. 在心律异常缓慢的情况下可能需要使用起搏器. 如果你正在接受任何类型的非心脏手术或侵入性手术，你也应该咨询心脏病专家在照顾患有先天性心脏病的成年人方面的专业知识.

Medical Follow up

常规检查包括心电图、超声心动图、动态心电图和压力测试. 你的心脏病专家可能会建议你做其他检查，比如核磁共振或心导管检查. 如果你有心脏起搏器，你需要更频繁地检查心脏起搏器的功能.

Activity Restrictions

Many patients don't need to limit their activity, 除了避免像举重这样会导致血压升高的活动. However, 如果你的心脏不能正常跳动或者你有心律问题, you may need to limit your activity to your endurance. 你的心脏病专家会帮助你确定是否需要限制你的活动.