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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
- If Your Child Has a Heart Defect
- When Your Child Needs a Heart Transplant
- Heart and Circulatory System
- Congenital Heart Defects Special Needs Factsheet
- Heart Murmurs
- Coarctation of the Aorta
- Congenital Heart Defects
- Atrial Septal Defect
- A to Z: Tetralogy of Fallot
- A to Z: Atrial Flutter
- Patent Ductus Arteriosus (PDA)
- Ventricular Septal Defect
- Tetralogy of Fallot
- A to Z: Patent Ductus Arteriosus (PDA)
- A to Z: Hypoplastic Left Heart Syndrome
Trusted External Resources
A to Z: Hypoplastic Left Heart Syndrome
A to Z: Hypoplastic Left Heart Syndrome
May also be called: HLHS
Hypoplastic (HI-puh-plas-tik) left heart syndrome (HLHS) is a rare and serious defect in which the structures of the left side of the heart (the left ventricle, the mitral valve, and the aortic valve) are underdeveloped. As a result, blood cannot be adequately pumped throughout the body.
More to Know
Hypoplastic left heart syndrome is present at birth (congenital) and has no known cause. It is usually diagnosed within the first few days of life. Without surgery to repair the heart, HLHS is fatal.
Symptoms of HLHS include blue or gray skin color (cyanosis), rapid breathing, pounding heart, shortness of breath, cold hands and feet, weak pulse, unusual drowsiness and poor feeding.
Once diagnosed with HLHS, most babies will undergo a series of three operations beginning shortly after birth and ending at 3 years of age. For some babies, heart transplant is the best option if a donated organ is available.
Keep in Mind
While many babies now survive HLHS, they need to be monitored closely by a cardiologist for the rest of their lives. Many complications can occur with HLHS and sometimes additional heart surgery or even heart transplant may be necessary as children born with HLHS get older.
All A to Z dictionary entries are regularly reviewed by KidsHealth medical experts.
Date reviewed: September 05, 2017