Samad has a Medtronic pacemaker. It is placed on his right lower ventrical area, just under his
rib-cage.

The Medtronic EnRhythm® pacemaker is a small, implantable medical device prescribed for people with a slow or weak heart rhythm to stimulate the heart muscle with precisely timed pulses of electricity. The EnRhythm pacemaker, which delivers electricity to both the right atrium and right ventricle, promotes natural heart activity by significantly reducing unnecessary pacing in the heart’s right ventricle when normal heart conduction is present by automatically switching to pace the right atrium. The EnRhythm device is the first-ever pacemaker to offer this exclusive feature, called MVP® or Managed Ventricular Pacing. With MVP, the device can be programmed to deliver pacing pulses to the right ventricle less than 5 percent of the time, compared to 50 percent or more with typical dual-chamber pacing. This is an important clinical benefit since clinical studies have shown that unnecessary pacing in the right ventricle can increase the risk for heart failure and atrial fibrillation.

Every 45 days, Samad is seen at the pacemaker clinic at the Children's Hospital of Philadelphia. He has a rountine 1)ECG, a 2)Pacemaker Interrogation and every 6 months he has an 3)ECHOCARDIOGRAM.

1) An electrocardiogram or ECG allows us to measure the electrical activity in your child's heart. We perform an ECG by attaching electrodes (small patches) to your child's chest, arms and legs and connecting the electrodes to the ECG machine using lead wires. The ECG machine produces a printout or tracing that we examine to see if it varies from a normal tracing.

The ECG helps us to determine whether your child might have certain heart problems. If your child has had a pacemaker implanted, we may use an ECG to monitor the pacemaker's function. We may also use an ECG to help us monitor the effectiveness of a heart medication your child is taking or to check his heart after he has had a heart procedure such as cardiac catheterization, heart surgery or an electrophysiology study.

We will usually take a baseline or resting ECG as part of your child's physical examination. If your child has an exercise test, we compare the exercise ECG with the baseline or resting ECG to see if any changes occur with increasing stress on his or her heart

2) During an interrogation, a small magnetic device is placed overtop of the pacemaker site. It's linked to a computer which can communicate with the pacemaker using radio waves.  Using a programmer we can "interrogate" the pacemaker and determine how efficiently it's pacing the different chambers of the heart, the remaining life of the pacemakers battery and if there have been any unusual events such as a life-threatening arrhythmia that the pacemaker picked up (even if the patient was unaware that they may have occurred).  In addition, aside from just checking a pacemaker, we can reprogram the unit to behave more ideally for the individual patients problem.  This periodic fine tuning of the pacemaker will help maximize the benefit that the patient gets from cardiac pacing and also help maximize the pacers battery life because we will adjust the pacemakers energy delivery to more finely match the amount required for that particular patient. 

In a few years, Samad will have to undergo yet another surgery to have his pacemaker replaced because the unit that he has now, which is about the size of a fifty cent piece, will no longer support his growing heart and body.

3) During an echocardiographic study (a cardiac ultrasound or echo), we use very high frequency sound waves to form a moving, two-dimensional picture of your child's heart on a television screen. This enables us to:

• Identify any abnormalities in the structure of your child's heart
• Evaluate how the heart muscle is functioning
• Measure the speed of blood flow through the heart
• Estimate blood pressure in the different chambers of the heart

Most of the time the pictures are taken using a noninvasive technique called transthoracic echocardiography, during which a small instrument (a transducer), is placed on your child's skin. Sometimes the pictures are taken from inside your child's esophagus using an invasive technique called transesophageal echocardiography. Echocardiography studies can be performed on children of any age and size. Echos are also used to examine the heart of unborn babies suspected of having congenital heart disease

Samad has been diagnosed with a Congenital Heart Disease (CHD) called Prolonged QT Syndrome.

What is Long QT Syndrome?

Long QT Syndrome (LQTS) is a medical condition resulting from an abnormality in the electrical system of the heart.

The “QT” interval refers to a specific measurement on the electrocardiogram, the record of the electrical activity of a person’s heart. A “QT” interval is measured in seconds or in milliseconds. “Long QT” means the QT interval lasts longer than normal.

A prolonged QT interval occurs when the cells of the ventricles (the lower chambers of the heart) don’t electrically reset at the same time or take longer than normal to reset. This can allow for an arrhythmia, or abnormal heart rhythm, to occur that can be potentially lethal. However, there are some QT intervals that are at the borderline duration that still may be considered normal. A person has LQTS when there is abnormal prolongation of the QT interval that can cause certain symptoms, such as fainting.

In most cases LQTS is inherited, or caused by a genetic mutation that is passed down through a family. It can also be caused by certain medications. LQTS can manifest in different forms and can be difficult to diagnose.

What are the symptoms?

LQTS can cause:

• fainting, especially as a result of acute emotions (such as anger or surprise), exercise, menses (menstruation), or swimming in cold water
• seizures
• ventricular arrhythmias (abnormal heart rhythms originating in the lower chambers)
• sudden cardiac arrest

Sometimes children or adults have no symptoms until they experience sudden cardiac arrest. At the same rate, not all patients who have fainting have LQTS.

How is it diagnosed?

If a child has repeated fainting episodes, or other symptoms, a pediatrician may refer him or her to a pediatric cardiologist. It is not unusual for individuals with LQTS to have no symptoms until the teenage or early adult years.

LQTS is diagnosed with an electrocardiogram. Evaluation often includes a Holter monitor and exercise stress testing.

Because the condition is usually inherited, your cardiologist may consider genetic testing and counseling. If a child has LQTS, brothers, sisters, parents and other family members may also have it.

What are the treatment options?

If a person has LQTS and isn’t treated, the risk of sudden death over a lifetime is high.

A person with LQTS may have to:

• take medications called beta blockers
• avoid triggers that may cause an arrhythmia event
• avoid medications that can prolong the QT interval

Beta blockers significantly reduce the incidence of arrhythmias and sudden cardiac arrest. In some cases, however, an implantable cardioverter-defibrillator (ICD), a special pacemaker that can interrupt arrhythmias, is necessary to stop these arrhythmias. Implanting this device requires surgery.

Some children with LQTS have to limit competitive sports and other exercise. It is particularly dangerous for a person with LQTS to have low levels of electrolytes (sodium and potassium are two examples of electrolytes). Remaining hydrated by drinking water or sports drinks (which contain electrolytes) is important, particularly before, during and after exercise.

Treatment may depend on which type of LQTS your child has. The three major types of LQTS are:

**SAMAD HAS LQT1 & LQT2**

• LQT1: Individuals with LQT1 are more likely to have symptoms with exercise, although symptoms can occur with emotional stress and even when resting. It can be treated effectively with beta blocker medication in most patients. It is important for individuals with LQT1 to maintain normal potassium levels and lifestyle modifications in addition to taking their medications.
• LQT2: Individuals with LQT2 are more likely to have symptoms with emotional stress, but symptoms can also occur during exercise and when resting. LQT2 can be treated moderately well with beta blocker medication, but treatment may be improved by also maintaining a good potassium level. Of the three major LQTS types, LQT2 is most affected by medications that further prolong the QT interval.
• LQT3: Individuals with LQT3 are more likely to have symptoms during rest. LQT3 is less well treated with beta blocker medication than other types of LQTS, but beta blocker medication is still effective in many of these patients. LQT3 patients with symptoms may need to take another medication in addition to beta blockers. Some patients may need an implantable cardioverter-defibrillator (ICD).

What kind of follow-up care is required?

Your child won’t outgrow LQTS. He or she will have to continue to take beta blockers, or continue other appropriate treatment, and will require life-long care by a cardiologist. In most cases, an annual checkup will be sufficient.

Most children and adults with LQTS lead healthy, normal lives. It is very important that they and their families be aware of the signs of sudden cardiac arrest, so that they can get help immediately if needed.