患有复杂先天性心脏病的儿童的ALCAPA:一个多中心研究
Saul Flores1, Christine M Riley2, Peter Sassalos3
1Division of Critical Care and Cardiology, Department of Pediatrics, Texas Children's HospitalBaylor College of Medicine, 6651 Main Street, 14th Floor, Houston, TX, 77030, USA. saul.flores2@bcm.edu.
Pediatric cardiology
|April 3, 2024
概括
从肺动脉出发的复杂异常左冠状动脉 (ALCAPA) 在儿童中很少见,并且通常在手术前未被诊断出来. 与孤立的ALCAPA相比,这些复杂病例的ECMO,CPR和手术死亡率显著更高.
科学领域:
- 儿童心脏病学 儿童心脏病学
- 生产性心脏病 手术 手术 生产性心脏病
- 医学诊断 医学诊断 医学诊断
背景情况:
- 来自肺动脉的异常左冠状动脉 (ALCAPA) 是一种罕见的先天性心脏缺陷.
- 复杂ALCAPA涉及相关的心脏异常,可能使诊断和治疗复杂化.
- 了解复杂的ALCAPA的结果对于手术规划和患者管理至关重要.
研究的目的:
- 描述患有复杂ALCAPA的儿童的临床表现和手术结果.
- 将复杂的ALCAPA病例的结果与孤立的ALCAPA病例的结果进行比较.
- 确定导致复杂ALCAPA的发病率和死亡率的因素.
主要方法:
- 从2009年至2018年间在21个中心接受心脏手术的儿科ALCAPA病例的回顾性分析.
- 包括258名ALCAPA患者,确定10例 (3.9%) 是复杂的.
- 复杂和孤立的ALCAPA组之间的结果的统计比较,使用费舍尔的精确测试.
主要成果:
- 复杂的ALCAPA与各种心脏病变有关,包括HLHS,scimitar综合征,有大动脉缩的VSD等.
- 在复杂的ALCAPA中,术前诊断是罕见的 (1/10),许多人被诊断为术内或术后.
- 与单独的ALCAPA相比,复杂ALCAPA患者的ECMO支持率 (50%对12%),心肺复苏率 (30%对6%) 和手术死亡率 (50%对3%) 显着更高.
结论:
- 复杂ALCAPA是一种罕见但严重的疾病,诊断延迟率高.
- 复杂ALCAPA的手术修复与术后发病率和死亡率大幅增加有关.
- 改善复杂ALCAPA的手术前诊断策略是有必要的,以改善患者的治疗结果.
相关概念视频
Development of the Heart
929
The development of the human heart, a crucial organ, commences from the mesoderm on the 18th or 19th day after fertilization. This process initiates in the cardiogenic area, a group of mesodermal cells at the embryo's head end, which evolves into elongated strands known as cardiogenic cords. These cords undergo a transformation to form hollow-centered endocardial tubes.
As the embryo undergoes lateral folding, these paired tubes approach each other, merging into a single primitive heart...
As the embryo undergoes lateral folding, these paired tubes approach each other, merging into a single primitive heart...
929
Heart Valves
4.6K
The human heart is a complex organ with an intricate system of valves that regulate blood flow. There are two main types of valves: atrioventricular (AV) valves and semilunar valves.
The AV valves prevent the backflow of blood from the ventricles to the atria during ventricular contraction. These valves function with the assistance of the chordae tendineae and papillary muscles. When the ventricles are relaxed, the chordae tendineae are slack, allowing blood to flow from the atria into the...
The AV valves prevent the backflow of blood from the ventricles to the atria during ventricular contraction. These valves function with the assistance of the chordae tendineae and papillary muscles. When the ventricles are relaxed, the chordae tendineae are slack, allowing blood to flow from the atria into the...
4.6K
Imbalances in Cardiac Output
1.4K
The heart's primary function is to pump blood throughout the body, maintaining a balance between blood sent out (cardiac output) and blood returning (venous return). If this balance is disrupted, it can result in congestive heart failure (CHF), a severe condition where the heart becomes an inefficient pump, leading to inadequate blood circulation.
CHF can occur due to the failure of either side of the heart. Left-side failure leads to pulmonary congestion—the right side continues to send...
CHF can occur due to the failure of either side of the heart. Left-side failure leads to pulmonary congestion—the right side continues to send...
1.4K
Fetal Circulation
958
Fetal circulation is a unique system that facilitates the exchange of gases, nutrients, and waste products between the developing fetus and the mother. This intricate process takes place through a special organ called the placenta.
Two umbilical arteries transport blood from the fetus to the placenta. At the placenta, the blood absorbs oxygen and nutrients while simultaneously eliminating waste products. This oxygen-enriched and nutrient-rich blood then returns to the fetus through one...
Two umbilical arteries transport blood from the fetus to the placenta. At the placenta, the blood absorbs oxygen and nutrients while simultaneously eliminating waste products. This oxygen-enriched and nutrient-rich blood then returns to the fetus through one...
958


