Congenital Heart Defects

What are congenital heart defects?

Congenital (kon-JEN-i-tal) heart defects are problems with the heart's structure that are present at birth. These defects can involve the interior walls of the heart, valves inside the heart, or the arteries and veins that carry blood to the heart or out to the body. Congenital heart defects change the normal flow of blood through the heart.

There are many different types of congenital heart defects. They range from simple defects with no symptoms to complex defects with severe, life-threatening symptoms.

Congenital heart defects are the most common type of birth defect, affecting 8 of every 1,000 newborns. Each year, more than 35,000 babies in the United States are born with congenital heart defects. Most of these defects are simple conditions that are easily fixed or need no treatment.

A small number of babies are born with complex congenital heart defects that need special medical attention soon after birth. Over the past few decades, the diagnosis and treatment of these complex defects has greatly improved.

As a result, almost all children with complex heart defects grow to adulthood and can live active, productive lives because their heart defects have been effectively treated.

Most people with complex heart defects continue to need special heart care throughout their lives. They may need to pay special attention to certain issues that their condition could affect, such as health insurance, employment, pregnancy and contraception, and preventing infection during routine health procedures. Today in the United States, about 1 million adults are living with congenital heart defects.

How the heart works

To understand congenital heart defects, it's helpful to know how the normal heart works.

Your child's heart is a muscle about the size of his or her fist. It works like a pump and beats 100,000 times a day.

The heart has two sides, separated by an inner wall called the septum. The right side of the heart pumps blood to the lungs to pick up oxygen. Then, oxygen-rich blood returns from the lungs to the left side of the heart, and the left side pumps it to the body.

The heart has four chambers and four valves and is connected to various blood vessels. Veins are the blood vessels that carry blood from the body to the heart. Arteries are the blood vessels that carry blood away from the heart to the body.

The illustration shows a cross-section of a healthy heart and its inside structures. The blue arrow shows the direction in which oxygen-poor blood flows from the body to the lungs. The red arrow shows the direction in which oxygen-rich blood flows from the lungs to the rest of the body.

Picture of a Healthy Heart Cross-Section

Heart Chambers

The heart has four chambers or "rooms."

  • The atria (AY-tree-uh) are the two upper chambers that collect blood as it comes into the heart.

  • The ventricles (VEN-trih-kuls) are the two lower chambers that pump blood out of the heart to the lungs or other parts of the body.

Heart Valves

Four valves control the flow of blood from the atria to the ventricles and from the ventricles into the two large arteries connected to the heart.

  • The tricuspid (tri-CUSS-pid) valve is in the right side of the heart, between the right atrium and the right ventricle.

  • The pulmonary (PULL-mun-ary) valve is in the right side of the heart, between the right ventricle and the entrance to the pulmonary artery, which carries blood to the lungs.

  • The mitral (MI-trul) valve is in the left side of the heart, between the left atrium and the left ventricle.

  • The aortic (ay-OR-tik) valve is in the left side of the heart, between the left ventricle and the entrance to the aorta, the artery that carries blood to the body.

Valves are like doors that open and close. They open to allow blood to flow through to the next chamber or to one of the arteries, and then they shut to keep blood from flowing backward.

When the heart's valves open and close, they make a "lub-DUB" sound that a doctor can hear using a stethoscope.

  • The first sound - the "lub" - is made by the mitral and tricuspid valves closing at the beginning of systole (SIS-toe-lee). Systole is when the ventricles contract, or squeeze, and pump blood out of the heart.

  • The second sound - the "DUB" - is made by the aortic and pulmonary valves closing at beginning of diastole (di-AS-toe-lee). Diastole is when the ventricles relax and fill with blood pumped into them by the atria.

Arteries

The arteries are major blood vessels connected to your heart.

  • The pulmonary artery carries blood pumped from the right side of the heart to the lungs to pick up a fresh supply of oxygen.

  • The aorta is the main artery that carries oxygen-rich blood pumped from the left side of the heart out to the body.

  • The coronary arteries are the other important arteries attached to the heart. They carry oxygen-rich blood from the aorta to the heart muscle, which must have its own blood supply to function.

Veins

The veins are also major blood vessels connected to your heart.

  • The pulmonary veins carry oxygen-rich blood from the lungs to the left side of the heart so it can be pumped out to the body.

  • The vena cava is a large vein that carries oxygen-poor blood from the body back to the heart.



Patient Comments

Viewers share their comments

Congenital Heart Defects - Types Question: Please describe the type of congenital heart defect you have, and your experience.
Congenital Heart Defects - Symptoms Question: What symptoms did you experience with your congenital heart defect?
Congenital Heart Defects - Treatment Question: What treatment did you receive for your congenital heart defect?

Ectopia Cordis

Medical Author: David Perlstein, MD, MBA, FAAP
Medical Editor: Melissa Conrad Stöppler, MD

When I heard about Ryan Marquiss and saw his smiling 3 year old toddler face I was pretty much amazed, but pleased. Ectopia cordis is an exceedingly rare defect in which the heart, in part or in its entirety, forms outside the chest cavity. It is considered as one of the less common cardiac malpositions (defects characterized by the heart or great vessels developing in the wrong place). When I say rare, I mean rare. Ectopia cordis occurs in 5-7 per one million live births and is associated with an extremely high mortality rate. There are five different types of ectopia cordis that are defined by the position of the heart outside the body. Prior to the era of ultrasound, ectopia cordis was not diagnosed prenatally but after delivery. Today most cases of ectopia cordis are diagnosed in the second trimester. So, how is it that Ryan Marquiss is doing as well as he is?

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