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Normally, oxygenated blood flows from the lungs to the left atrium through the pulmonary veins. In a case of Total Anomalous Pulmonary Venous Return (TAPVR), the pulmonary veins drain into the right atrium rather than the left atrium. When this happens, the oxygenated blood returning from the lungs mixes with the deoxygenated blood in the right atrium.
Some form of communication between the right and left sides of the heart, usually an atrial septal defect (ASD), must be present in order for oxygenated blood to reach the body. Surgical repair of total anomalous pulmonary venous return is required within the first few months of life. The goal of corrective surgery is to surgically create a connection between the pulmonary veins and the left atrium.
How Total Anomalous Pulmonary Venous Return Differs From Normal Cardiac Anatomy?
If your child has total anomalous pulmonary venous return the structure of his or her heart is different from normal cardiac anatomy.
Heart With Normal Cardiac Anatomy
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When your child has a congenital heart defect, there's usually something wrong with the structure of his or her heart's structure.
The heart is composed of four chambers. The two upper chambers, known as atria, collect blood as it flows back to the heart. The two lower chambers, known as ventricles, pump blood with each heartbeat to the two main arteries (the pulmonary artery and the aorta). The septum is the wall that divides the heart into right and left sides. The atrial septum separates the right and left atria; likewise, the ventricular septum separates the two ventricles.
There are four valves that control the flow of blood through the heart. These flap-like structures allow blood to flow in only one direction. The tricuspid and mitral valves, also known as the atrioventricular valves, separate the upper and lower chambers of the heart. The aortic and pulmonary valves, also known as the arterial valves, separate the ventricles from the main arteries. Oxygen-depleted blood returns from the body and drains into the right atrium via the superior and inferior vena cavas. The blood in the right atrium then passes through the tricuspid valve and enters the right ventricle.
Next, the blood passes through the pulmonary valve, enters the pulmonary artery, and travels to the lungs where it is replenished with oxygen. The oxygen-rich blood returns to the heart via the pulmonary veins, draining into the left atrium. The blood in the left atrium passes through the bicuspid, or mitral, valve and enters the left ventricle.
Finally, the oxygen-rich blood flows through the aortic valve into the aorta and out to the rest of the body.
From Nemours' KidsHealth
- ECG (Electrocardiogram)
- Cardiac Catheterization
- A to Z: Hypoplastic Left Heart Syndrome
- A to Z: Atrial Flutter
- Atrial Septal Defect
- If Your Child Has a Heart Defect
- When Your Child Needs a Heart Transplant
- A to Z: Tetralogy of Fallot
- Tetralogy of Fallot
- A to Z: Patent Ductus Arteriosus (PDA)
- Heart Murmurs and Your Child
- Patent Ductus Arteriosus (PDA)
- Coarctation of the Aorta
- Congenital Heart Defects
- Heart and Circulatory System
- Congenital Heart Defects Special Needs Factsheet
- Ventricular Septal Defect
Trusted External Resources
A to Z: Tetralogy of Fallot
A to Z: Tetralogy of Fallot
The four specific heart defects that make up tetralogy of Fallot (fah-LO) are:
- ventricular septal defect
- pulmonary valve stenosis (narrowing)
- right ventricular hypertrophy
- overriding aorta
More to Know
Babies with tetralogy of Fallot can have cyanosis, which is a bluish-purple color to their skin, lips, and fingernails. This happens when not enough blood reaches the lungs to get oxygen. They may also fail to gain weight, have difficulty feeding or breathing, and have enlarged skin or bones around the fingernails (known as clubbing).
Tetralogy of Fallot develops in utero while the heart is forming. A specific cause hasn't been determined, but babies with certain genetic disorders are at higher risk for developing it. Other risk factors include advanced maternal age and, during pregnancy, poor nutrition, diabetes, or certain viral illnesses.
Keep in Mind
Tetralogy of Fallot is a serious condition that requires young infants to have heart surgery to repair the defect. The good news is that most babies recover fully and thrive into adulthood. They will, however, need to be monitored closely by a heart specialist for the rest of their lives.
All A to Z dictionary entries are regularly reviewed by KidsHealth medical experts.
Date reviewed: April 28, 2017