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Related Concept Videos

Development of the Heart01:27

Development of the Heart

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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.
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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.
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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.
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Related Experiment Video

Updated: Jun 29, 2025

Direct Re-implantation of Left Coronary Artery into the Aorta in Adults with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery ALCAPA
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ALCAPA in Children with Complex Congenital Heart Disease: A Multicenter Study.

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
PubMed
Summary

Complex anomalous left coronary artery from the pulmonary artery (ALCAPA) in children is rare and often undiagnosed preoperatively. These complex cases experience significantly higher rates of ECMO, CPR, and operative mortality compared to isolated ALCAPA.

Keywords:
Congenital heart diseaseCongenital heart surgeryCoronary anomaly

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Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease Surgery
  • Medical Diagnostics

Background:

  • Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect.
  • Complex ALCAPA involves associated cardiac anomalies, potentially complicating diagnosis and treatment.
  • Understanding outcomes in complex ALCAPA is crucial for surgical planning and patient management.

Purpose of the Study:

  • To characterize the clinical presentation and surgical outcomes of children with complex ALCAPA.
  • To compare the outcomes of complex ALCAPA cases with those of isolated ALCAPA.
  • To identify factors contributing to morbidity and mortality in complex ALCAPA.

Main Methods:

  • Retrospective analysis of pediatric ALCAPA cases undergoing cardiac surgery from 2009-2018 across 21 centers.
  • Inclusion of 258 patients with ALCAPA, identifying 10 cases (3.9%) as complex.
  • Statistical comparison of outcomes between complex and isolated ALCAPA groups using Fisher's exact test.

Main Results:

  • Complex ALCAPA was associated with various cardiac lesions including HLHS, scimitar syndrome, VSD with aortic coarctation, and others.
  • Preoperative diagnosis was rare in complex ALCAPA (1/10), with many diagnosed intraoperatively or postoperatively.
  • Complex ALCAPA patients showed significantly higher rates of ECMO support (50% vs. 12%), CPR (30% vs. 6%), and operative mortality (50% vs. 3%) compared to isolated ALCAPA.

Conclusions:

  • Complex ALCAPA is an uncommon but severe condition with a high rate of delayed diagnosis.
  • Surgical repair of complex ALCAPA is associated with substantially increased postoperative morbidity and mortality.
  • Improved preoperative diagnostic strategies for complex ALCAPA are warranted to improve patient outcomes.