Hypoplastic left heart syndrome (HLHS) is a congenital Congenital Chorioretinitis heart defect that consists of the underdevelopment, or hypoplasia, of the left side of the heart in various degrees. The most notable feature of HLHS is the reduced size and functionality of the left ventricle (LV). Also, HLHS is associated with stenosis, hypoplasia, or atresia of the vessels or atrioventricular valves on the left side of the heart. A mixture of genetic factors and altered fetal blood flow Blood flow Blood flow refers to the movement of a certain volume of blood through the vasculature over a given unit of time (e.g., mL per minute). Vascular Resistance, Flow, and Mean Arterial Pressure causes HLHS. Hypoplastic left heart syndrome presents once the ductus arteriosus Ductus arteriosus A fetal blood vessel connecting the pulmonary artery with the descending aorta. Patent Ductus Arteriosus (PDA) closes physiologically as tachypnea Tachypnea Increased respiratory rate. Pulmonary Examination, cyanosis Cyanosis A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule. Pulmonary Examination, heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR), and cardiogenic shock Shock Shock is a life-threatening condition associated with impaired circulation that results in tissue hypoxia. The different types of shock are based on the underlying cause: distributive (↑ cardiac output (CO), ↓ systemic vascular resistance (SVR)), cardiogenic (↓ CO, ↑ SVR), hypovolemic (↓ CO, ↑ SVR), obstructive (↓ CO), and mixed. Types of Shock. Diagnosis can be made pre- or postnatally via echocardiogram Echocardiogram Transposition of the Great Vessels. Once detected, surgical treatment is the 1st-line therapy, done in 3 stages.
Last updated: Sep 26, 2022
Hypoplastic left heart syndrome (HLHS) is characterized by the underdevelopment of the left side of the heart. The components of HLHS include:
There are 3 types of HLHS based on the morphology of the cardiac valves:
The following genetic syndromes are associated with the development of HLHS:
The following conditions are differential diagnoses of HLHS:
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