Hypoplastic Left Heart Syndrome

Heart With Hypoplastic Left Heart Syndrome

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Hypoplasia is defined as underdevelopment of a tissue or organ, usually due to a deficiency in the number of cells. Hypoplastic left heart syndrome is the underdevelopment of the left side of the heart, including the left atrium and ventricle, the mitral valve, the aortic valve, and the aorta.

In some cases an associated ASD allows blood returning from the lungs to flow through the opening in the septum from the left to the right atrium. The mixed blood enters the right ventricle and is then pumped into the pulmonary artery. The blood reaches the aorta through a patent ductus arteriosus, which is kept open by intravenous medication. This heart defect is fatal within the first days or months of life without treatment.

Treatment Options

Options for treatment include a series of three operations collectively known as the Norwood Procedure or heart transplantation. The goal of the Norwood Procedure is to direct deoxygenated blood directly to the lungs and utilize the functional right heart to pump oxygenated blood to the body.

 
Stage I Norwood Procedure

The first stage, performed in the first week of life, is known as the Stage I Norwood Procedure. The connection between the right ventricle and the branch pulmonary arteries is broken and the main pulmonary artery and the small aorta are connected and augmented to create a new, larger aorta. Next, a small tube (shunt) is placed between the aorta and the right branch pulmonary artery to allow for blood flow to the lungs.

Post-Modified Stage I Norwood Procedure

The modified Stage 1 Norwood Procedure connects the Pulmonary artery to the Right Ventricle using a shunt. The underdeveloped Aorta is reconstructed and enlarged.

The right ventricle is converted into a common systemic (to the body) ventricle. The oxygenated and de-oxygenated blood mix in the right atrium and right ventricle and is then dispersed out to the body, through the reconstructed aorta, and to the lungs through the RV to PA shunt and pulmonary artery.

The purpose of the modified Stage 1 Norwood Procedure is to allow blood to circulate in a controlled manner throughout the body, without obstruction.

 
Hemi-Fontan Procedure (Second Stage)

The Hemi-Fontan procedure is the second of three operations for children with hypoplastic left heart syndrome and other types of single ventricle physiology. This procedure is generally performed at 6 months of age. The Hemi-Fontan consists of anastomosis of the superior vena cava (SVC) to the right pulmonary artery, augmentation of the branch pulmonary arteries and patch closure of the communication between the superior vena cava and the right atrium.

After the Hemi-Fontan procedure, the blue blood returning from the upper body through the SVC is immediately diverted to the lungs, without passing through the heart. This blood becomes oxygenated in the lungs and returns to the left atrium. This red or oxygenated blood then passes through the atrial communication into the right atrium. The deoxygenated blood from the lower body enters the right atrium through the inferior vena cava (IVC); there it mixes with the oxygenated blood from lungs. The mixed blood then passes into the right ventricle and is pumped out into the reconstructed aorta to supply the body. The importance of this procedure is that it relieves the single ventricle of having to pump an excess volume of blood. Prior to this procedure, the ventricle is pumping both to the body and to the lungs. Following the Hemi-Fontan, the ventricle pumps only to the body, since the lung is supplied with blood flow directly from the superior vena cava.

 
Third and Final Stage

The third and final stage is performed at approximately 12 months of age. During this procedure, the deoxygenated blood of the lower half of the heart is directed to the lungs. This is done by channeling the blood of the inferior vena cava through the right atrium to the right branch pulmonary artery.


What Is Normal Cardiac Anatomy?

When your child has a congenital heart defect, there's usually something wrong with the structure of his or her heart's structure.

 
Learn More About 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.

Arrhythmias

An arrhythmia  is an abnormal heart rhythm. Most arrythmias are caused by an electrical "short circuit" in the heart.

The heart normally beats in a consistent pattern, but an arrhythmia can make it beat too slowly, too quickly, or irregularly. This erratic pumping can lead to a variety of symptoms, including fatigue, dizziness, and chest pain.

Many arrhythmias don't need medical care, but some can pose a health problem and need to be evaluated and treated by a doctor.

What Causes Arrhythmias?

A unique electrical conduction system in the heart causes it to beat in its regular rhythm. 

The electrical signals start from a group of cells called the sinus node, located in the right atrium. The sinus node acts as the heart's pacemaker and makes sure the heart is beating at a normal and consistent rate. The sinus node normally speeds up the heart rate in response to things like exercise, emotions, and stress, and slows the heart rate during sleep.

But sometimes the electrical signals don't "communicate" properly with the heart muscle, and the heart can start beating in an abnormal rhythm — this is an arrhythmia (also called dysrhythmia).

Arrhythmias also can be due to chemical imbalances in the blood; infections; diseases that irritate the heart; medicines (prescription, over-the-counter, and some herbal remedies); injuries to the heart from chest trauma or heart surgery; use of illegal drugs, alcohol, or tobacco; caffeine; and stress.

Arrhythmias can be temporary or permanent. An arrhythmia can be congenital (meaning a baby is born with it) or happen later.

Signs and Symptoms

Arrhythmias make the heart beat less effectively, interrupting blood flow to the brain and the rest of the body. When the heart beats too fast, its chambers can't fill with enough blood. When it beats too slowly or irregularly, it can't pump enough blood out to the body.

If the body doesn't get the supply of blood it needs to run smoothly, a person might have:

  • dizziness
  • fatigue
  • lightheadedness
  • weakness
  • palpitations (a feeling of fluttering or pounding in the chest)
  • shortness of breath
  • chest pain
  • fainting

Arrhythmias can be constant, but most come and go at random. Some cause no detectable symptoms at all. In these cases, the arrhythmia is only found during a physical examination or a heart function test.

What's a Normal Heart Rate?

Heart rate is measured by counting the number of beats per minute. Someone's normal heart rate depends on things like the person's age and whether he or she leads an active lifestyle. 

The resting heart rate decreases as kids get older. Typical normal resting heart rate ranges are:

  • babies (birth to 3 months of age): 100–150 beats per minute
  • kids 1–3 years old: 70–110 beats per minute
  • kids by age 12: 55–85 beats per minute

A doctor can determine whether a heart rate is abnormally fast or slow, depending on a person's situation. An older child or adult with a slow heart rate, for example, might have symptoms when the heart rate drops below 50 beats per minute. But trained athletes have a lower resting heart rate, so a slow heart rate in them isn't considered abnormal if it causes no symptoms.

Types of Arrhythmias

There are several types of arrhythmias, including:

Premature Atrial Contraction (PAC) and Premature Ventricular Contraction (PVC)

Premature contractions are usually considered minor arrhythmias. The person may feel a fluttering or pounding in the chest caused by an early or extra beat. PACs and PVCs are very common, and are what happens when it feels like your heart "skips" a beat. Actually, the heart doesn't skip a beat — an extra beat comes sooner than normal. Occasional premature beats are common and considered normal, but in some cases they can be a sign of an underlying medical problem or heart condition.

Tachycardias

A tachycardia is an abnormally fast heartbeat. Tachycardias fall into two major categories — supraventricular and ventricular:

  • Supraventricular tachycardia (SVT) is characterized by bursts of fast heartbeats that start in the upper chambers of the heart. These can happen suddenly and last anywhere from a few seconds to several days. Treatment is usually recommended if SVTs are long-lasting or happen often.
  • Ventricular tachycardia is a serious but uncommon condition that starts in the lower chambers of the heart and can be dangerous.

Bradycardias

A bradycardia is an abnormally slow heartbeat. Bradycardias can be due to:

  • Sinus node dysfunction, when the heart's sinus node isn't working correctly, usually after surgery to correct a congenital heart defect.
  • Heart block, when electrical impulses can't make their way from the upper to lower chambers of the heart. It's often caused by a congenital heart defect, but also can be due to disease or injury.

Diagnosing Arrhythmias

Doctors use several tools to diagnose arrhythmias. It's very important to know a child's medical history and give this information to the doctor. The doctor will use the medical history, along with a physical exam, to begin the evaluation.

If an arrhythmia is suspected, the doctor will order an electrocardiogram (EKG) to measure the heart's electrical activity. For this painless test, the child will lie down and have small metal tabs (called electrodes) fixed to the skin with sticky papers. The electrodes have wires attached to them, which connect to the EKG machine. The electrical signals from the heart are then briefly recorded, usually for just 10 seconds. This information is sent to a computer, where it's interpreted and drawn as a graph.

These types of EKG tests might be recommended:

  • Resting EKG. This measures resting heart rate and rhythm, and lasts about a minute.
  • Exercise EKG (also called a stress test). This measures heart rate and rhythm during exercising, like riding a stationary bicycle or walking on a treadmill.
  • Signal-average EKG. This is much like a resting EKG, but monitors the heartbeat for about 15–20 minutes.
  • Holter monitor. This EKG is done over a long period of time, usually 24 hours or more. The electrodes are fixed to the chest, and the wires are attached to a portable EKG recorder. The child is encouraged to continue normal daily activities, but must be careful to not get the electrodes wet (for example, no swimming, showering, or activities that cause a lot of sweating).

    The two kinds of Holter monitoring are: continuous recording, which means the EKG is on throughout the entire monitoring period; and event monitoring, which means data is recorded only when the child feels symptoms and then turns the Holter monitor on.

Treating Arrhythmias

Many arrhythmias don't need treatment. For those that do, these options might be used:

  • Medicine. Doctors may prescribe anti-arrhythmic medicines depending on the type of arrhythmia and other considerations. Sometimes, these can increase symptoms and cause side effects, so the patient will be closely watched by the doctor.
  • Pacemakers. A pacemaker is a small battery-operated device implanted into the body (near the collarbone) through a surgical procedure. Connected to the heart by a wire, a pacemaker can detect if the heart rate is too slow and send electrical signals to speed up the heartbeat.
  • Defibrillators. A small battery-operated implantable cardioverter defibrillator (ICD) is surgically placed near the left collarbone. Wires run from the defibrillator to the heart. The ICD senses if the heart has a dangerously fast or irregular rhythm and sends an electrical signal to restore a normal heartbeat.
  • Catheter ablation. A catheter (a long, thin wire) is guided through a vein in the leg to the heart. Arrhythmias often are caused by microscopic defects in the heart muscle. Once the problem area of the heart is pinpointed, the catheter heats or freezes the defective muscle cells and destroys them.
  • Surgery. Surgery is usually recommended only if all other options have failed. The child will be put under anesthesia, and a surgeon will remove the tissue causing the arrhythmia.

When to Call the Doctor

Many arrhythmias are minor and aren't a significant health threat. But some can indicate a more serious problem. If your child has symptoms of an arrhythmia, call your doctor.

Reviewed by: Steven B. Ritz, MD, MSEd
Date reviewed: June 14, 2017