Clinical Manifestations
An abnormal sound heard through a stethoscope caused by turbulent blood flow through the narrowed right ventricular outflow tract. It is described as harsh and systolic, heard loudest at the upper left sternal border, often accompanied by a single second heart sound.
What is a Heart Murmur?
On a chest x-ray, the heart will have a distinct shape due to the thickened ventricle and narrowed or missing pulmonary artery segment.
What is Boot-Shaped Appearance?
An invasive diagnostic procedure that uses a thin, flexible tube inserted into blood vessels to measure pressures and oxygen levels in the pulmonary and systemic circulations, evaluate shunt direction and volume, and map coronary or pulmonary artery anatomy. This is typically reserved for high-risk or complex cases where non-invasive imaging is insufficient.
What is Cardiac Catheterization?
A synthetic tube device used to improve oxygen levels by connecting the subclavian artery to the pulmonary artery. Used as a temporary fix for this condition.
What is Blalock-Taussig-Thomas Shunt?
French physician who published a revolutionary description of the disease that established and outlined the major defects in the disease.
What is Étienne-Louis Arthur Fallot?
A sudden, acute episode in which an infant becomes profoundly cyanotic, extremely restless or limp, and gasps for air, triggered by events like crying, feeding, waking from sleep that cause a rapid drop in pulmonary blood flow.
What is Hypercyanotic?
A heart defect that consists of a hole in the septum of the heart that separates the two lower chambers of the heart allowing deoxygenated blood to mix with oxygenated blood.
What is Ventricular Septal Defect?
A prenatal imaging study performed around the midpoint of pregnancy using ultrasound focused specifically on the structure of the developing fetus's heart. This is capable of detecting structural abnormalities such as a ventricular septal defect and overriding aorta before birth in approximately 60% of cases.
What is a Fetal Echocardiogram?
A minimally invasive cardiac catheterization procedure where a tiny metal mesh tube (stent) is inserted into the ductus arteriosus of a newborn to keep it open?
What is PDA stentinging catheterization?
The year the first palliative surgery (only addressing relieving symptoms, not a total fix) for this condition was performed using a shunt to create a connection between the subclavian artery and pulmonary artery to improve oxygenation.
What is 1944?
A physical finding in which the fingertips or toes become wide and rounded, with nail beds that curve downward, developing in patients after years without treatment due to chronic low blood oxygen.
What is Clubbing?
A heart defect where the Aorta sits directly above a hole in the wall separating the two lower chambers of the heart. Instead of rising solely from the left ventricle, the aorta overrides both the left and right ventricles, allowing it to receive blood from both. As a result, the aorta delivers oxygen-poor blood to the body, which should receive only oxygen-rich blood.
What is Overriding Aorta?
An electrical tracing of the heart's activity that characteristically shows right axis deviation and prominent right ventricular forces, with tall R waves in anterior leads and deep S waves in lateral leads. These findings do not normalize over time as they would in a healthy newborn.
What is an Electrocardiogram?
A surgeon patches the hole between the lower heart chambers and repairs or replaces the pulmonary valve. The surgeon may also remove thickened muscle below the pulmonary valve or widen the smaller lung arteries.
What is Complete Intracardiac Repair?
The year the first successful total repair fixing all anatomical heart defects in the condition was performed.
What is 1954?
A chronic condition in which the body overproduces red blood cells in response to long-term low oxygen levels in the blood.
What is Polycynthemia?
A heart condition characterized by a narrowed or obstructed pulmonary valve, which restricts blood flow from the right ventricle to the pulmonary artery.
What is Pulmonary Stenosis?
A non-invasive, painless newborn screening tool that detects low blood oxygen saturation and can identify undiagnosed cases of this condition before symptoms become apparent.
What is Pulse Oximetry?
The timeframe in which a patient with this condition typically undergoes open-heart surgery to repair the defects of the heart?
What is 4-6 months after birth?
A limitation, imperfection, or shortcoming that causes a product, process, or system to fail to meet requirements or standards?
What is a Defect?
A bluish or purplish discoloration of the skin, lips, and nail beds caused by an insufficient amount of oxygen in the blood being pumped throughout the body.
What is Cyanosis?
Abnormal thickening and enlargement of the heart's lower right chamber muscle that reduces the ability of the heart to pump effectively.
What is Right Ventricular Hypertrophy?
The gold-standard, non-invasive imaging test used to definitively diagnose this condition. It uses sound waves to visualize the four structural heart defects, measure the pressure gradient across obstructions, and assess ventricular function.
What is an Echocardiogram?
A medication used to block the effects of adrenaline (epinephrine), causing the heart to beat slower and with less force.
What are Beta-Blockers?
A condition, trait, or abnormality that is present before or at birth.
What is Congenital?