Hypoplastic left heart syndrome
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Hypoplastic left heart syndrome
Cardiovascular system
Vascular disorders
Arterial disease
Angina pectoris
Stable angina
Unstable angina
Myocardial infarction
Prinzmetal angina
Coronary steal syndrome
Peripheral artery disease
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Aneurysms
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Renal artery stenosis
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Cushing syndrome
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Human herpesvirus 8 (Kaposi sarcoma)
Angiosarcomas
Congenital heart defects
Truncus arteriosus
Transposition of the great vessels
Total anomalous pulmonary venous return
Tetralogy of Fallot
Hypoplastic left heart syndrome
Patent ductus arteriosus
Ventricular septal defect
Coarctation of the aorta
Atrial septal defect
Cardiac arrhythmias
Atrial flutter
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Premature atrial contraction
Atrioventricular nodal reentrant tachycardia (AVNRT)
Wolff-Parkinson-White syndrome
Ventricular tachycardia
Brugada syndrome
Premature ventricular contraction
Long QT syndrome and Torsade de pointes
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Atrioventricular block
Bundle branch block
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Valvular disorders
Tricuspid valve disease
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Rheumatic heart disease
Pericardial disorders
Pericarditis and pericardial effusion
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Dressler syndrome
Cardiac tumors
Cardiac tumors
Cardiovascular system pathology review
Acyanotic congenital heart defects: Pathology review
Cyanotic congenital heart defects: Pathology review
Atherosclerosis and arteriosclerosis: Pathology review
Coronary artery disease: Pathology review
Peripheral artery disease: Pathology review
Valvular heart disease: Pathology review
Cardiomyopathies: Pathology review
Heart failure: Pathology review
Supraventricular arrhythmias: Pathology review
Ventricular arrhythmias: Pathology review
Heart blocks: Pathology review
Aortic dissections and aneurysms: Pathology review
Pericardial disease: Pathology review
Endocarditis: Pathology review
Hypertension: Pathology review
Shock: Pathology review
Vasculitis: Pathology review
Cardiac and vascular tumors: Pathology review
Dyslipidemias: Pathology review
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Hypoplastic left heart syndrome
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Hypoplastic left heart syndrome
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Rishi Desai, MD, MPHHypo means “beneath” or “under,” and plasia means “molding” or “formation,” so hypoplastic means “under form” or in this case, “underdeveloped.” Therefore, hypoplastic left heart syndrome means that the left side of the heart doesn’t fully develop.
Hypoplastic left heart syndrome, or HLHS, is a congenital heart defect that affects the left side of the heart; it specifically affects the left ventricle and ascending aorta. Also, the aortic valve and mitral valve might be either too small to allow enough blood to flow through, or they might be absent altogether, which is called atresia. The exact mechanism that causes HLHS isn’t known, but one popular theory is that there might be some other primary congenital heart defect that reduces the blood flow through the LV and outflow tract during fetal development, so that part of the heart does not grow and develop normally. This said, HLHS is also often associated with other congenital heart defects, and in particular, with an atrial septal defect, which is an opening between the left and right atria. In fact, not only do these babies usually have this defect, they essentially need it to survive after birth. This is in addition to a patent ductus arteriosus, or PDA, a blood vessel that connects the aorta to the pulmonary artery, which usually closes within a few days after birth.
Let’s switch to a more simplified version of the heart to see what happens with blood flow. So, right here you have the atrial septal defect connecting the left and right atria, and the ductus arteriosus, which connects the aorta to the pulmonary artery. This is a really underdeveloped left ventricle and a smallish aortic and mitral valve.
Sources
- "Robbins Basic Pathology" Elsevier (2017)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
- "Hypoplastic left heart syndrome: from comfort care to long-term survival" Pediatric Research (2016)
- "Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions" Circulation (2016)
- "Opciones quirúrgicas actuales y sus resultados en neonatos con síndrome de corazón izquierdo hipoplásico" Anales de Pediatría (2019)
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