Congenital Heart Disease Care Model: Heart Rhythm

Mar 28, 2020 | Ethan McConkey, Moderator | @ethanmcconkey | Comments (5)

This is the fourth entry in our blogpost series that describe the different aspects of the Congenital Heart Disease Center's multi-disciplinary model of care. In this post, the role of Heart Rhythm study in care of CHD patients is examined and explained by Mayo Clinic Rochester electrophysiologist Malini Madhavan, M.B.B.S.

The heart’s rhythm is defined as the electrical activity of the heart that makes the heart muscles squeeze and pump blood. Observing the heart’s rhythm is an integral part of the well-rounded care of a congenital heart disease patient.

A patient is aware of a heart rhythm problem when they start experiencing symptoms, after a chamber of the heart starts beating too fast or slow, generally above 100 beats per minute or below 60 beats per minute. Malini Madhavan, M.B.B.S, an electrophysiologist at Mayo Clinic says that congenital heart disease patients have a higher risk of these abnormalities.

“Patients with adult congenital heart disease are more prone to heart rhythm abnormalities. In fact, it's the second most common cause for illness and hospital admissions in patients with adult congenital heart disease.”

Symptoms of heart rhythm problems are: heart palpitations, which is a sensation of the heart racing; lightheadedness or fainting spells; and increased tiredness or difficulty breathing with activities. Heart rhythm monitoring is recommended when patients experience these symptoms.

Where an irregular heart rhythm occurs, whether it occurs in the top chambers of the heart (atrium), or in the bottom chambers of the heart (ventricle) depends on the specific congenital heart disease diagnosis and the type of procedures performed in the past. The prognosis and treatment for the different arrhythmias will vary based on the type of arrhythmia and the underlying congenital heart disease. If left untreated for a significant period of time, heart rhythm abnormalities can lead to heart failure, stroke, or sudden death. Hence prompt diagnosis and treatment is key.

When a patient has symptoms, a doctor will first listen to the patient’s symptoms and heart sounds and then obtain an electrocardiogram to determine if an arrhythmia (abnormal heart rhythm) is present. If more monitoring is necessary a heart rhythm monitor is given to the patient for 24 hours to a month for the patient to wear continuously to detect problems with the heart rhythm. For patients who have infrequent symptoms that cannot be recorded on a month long monitor, small implanted heart rhythm monitoring devices can provide answers. An improvement in mobile technology gives physicians another tool to potentially use:

“Another advancement in the last five years are smartphone apps that patients can use to record their ECGs. The ECG quality from the smartphone is not the same as what is recorded by medical devices that we give out to patients, but it is of reasonable quality.” She added, “These mobile apps provide an additional diagnostic tool for patients who experience symptoms suspicious for heart rhythm abnormalities.”

The speed and location of the rhythm determines what treatment is appropriate, with treatment tailored to the particular patient and rhythm. Treatment can include medications (anti-arrhythmic drugs) with monitoring from an electrophysiologist. A catheter ablation is another treatment option, which is especially helpful for a particular subset of congenital heart disease patients.

“Patients with congenital heart disease are more prone to arrhythmias due to a combination of their heart disease and scars from prior surgical procedures. The presence of surgical scars can increase the chance of electrical movement around scarred regions leading to arrhythmia” Dr. Madhavan said.

According to Dr. Madhavan, an ablation procedure is a minimally invasive procedure. During this procedure, the physician passes catheters through the veins and arteries of the groin to the heart. The physician then identifies where the previous surgical scars are located and they proceed with an ablation procedure. During the ablation procedure, energy similar to electrocautery heat is used to target the area of the scar that is causing the irregular heartbeat.   Success rates of up to 80 percent are noted for some rhythms.

Another important treatment option, is the implantation of devices called pacemakers and defibrillators. Pacemakers are used to treat slow heart rhythms and defibrillators are specifically for ventricular arrhythmias. Ventricular arrhythmias are fast rhythms from the lower chamber (ventricles), that put a patient at high risk for sudden death. A defibrillator can be life-saving for these patients. Dr. Madhavan elaborated,

“Ventricular arrhythmias are frequently stopped using a shock to the heart. A defibrillator is able to diagnose a ventricular arrhythmia and provide a lifesaving shock in a timely fashion. So for patients who need it, having a defibrillator is like having an EMT with them all the time. The device is able to avoid the delays that occur when emergency medical services are activated and provides timely treatment that preserves the function of vital organs such as the brain. Defibrillators are devices that are most often implanted through minimally invasive procedures, less often they need to be surgically implanted. But these devices pick up on life threatening rhythms within a few seconds and either pace or shock the heart. Once the rhythm is back to normal, a patient’s consciousness is quickly restored within a few minutes.”

Dr. Madhavan concluded by stating the importance of having a team approach: “I take care of the heart rhythm problem, but work very closely with a team of specialists from adult congenital cardiology and cardiovascular surgery. It has to be a team approach.”

HELPFUL LINKS

Interested in more newsfeed posts like this? Go to the Congenital Heart Disease blog.

Our 43 years old son was born with Transposition of the Great Arteries and had a complete anatomical repair in 1977 at the Mayo Clinic by the late
Dr. Dwight Mc.Goon in an innovative procedure of those years. Since then he had 6 additional open heart surgeries, the last of them to install at once
3 mechanical valves..
He is stable now, even though about 5 years ago, he started with sever Rhythm problems, initially as Ventricle Tachycardia, and later also Atrial Fibrillation. We live in New York, and when the first signs of the Tachycardia started, we rushed to a top Medical center in NY, and despite their efforts, they were not able to find the sources of the Rhythm issues, and were therefore not able to perform the necessary Ablations to eliminate those sources. A decision was made to fly with him with a fixed wing Air Ambulance to the Mayo Clinic, where his Adult Congenital Cardiologist Dr. Heidi Connolly, arranged for
Dr. Samuel Asirvatham, a top World recognized Electrophysiologist to take over. Our son had a lenghty Ablation procedure and Dr. Asirvatham and his team, did not gave up, and eventually managed after a series of Ablation procedures to stabilize his Rhythm, and place a Defibrillator/Pacer.
Dr. Asirvatham and Dr. Connolly, are not only Top Physicians in their fields, but exceptional Human Beings, with compassion, humility, and deep commitment to their patients well being! We call them our "Guardian Angels".
Our son is doing well, and we have no doubt, that his survival through all his life challenges, is thanks to the dedicated and experienced people working
at the Mayo Clinic.

REPLY

I was diagnosed with LGL when I was 32 years old and have been on beta blockers ever since. Started with Inderal and then went to Corgard (nadalol) when it came available many years ago. I have recently had to have a new cardiologist due to them retiring. The new and younger cardiologist want me to go on blood thinners and say that they no longer diagnose the problem as LGL but call it Afib. I am now 79 years old and have had excellent experience with the beta blockers, and am afraid of blood thinners due to having had bronchiecstatis (sp) and bled from it a couple of times. I have was diagnosed with asthma 6 years ago and use Brovana and Alvesco daily. Do you not diagnose LGL anymore? Thanks. baz10

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@baz10

I was diagnosed with LGL when I was 32 years old and have been on beta blockers ever since. Started with Inderal and then went to Corgard (nadalol) when it came available many years ago. I have recently had to have a new cardiologist due to them retiring. The new and younger cardiologist want me to go on blood thinners and say that they no longer diagnose the problem as LGL but call it Afib. I am now 79 years old and have had excellent experience with the beta blockers, and am afraid of blood thinners due to having had bronchiecstatis (sp) and bled from it a couple of times. I have was diagnosed with asthma 6 years ago and use Brovana and Alvesco daily. Do you not diagnose LGL anymore? Thanks. baz10

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Lown-Ganong-Levine syndrome is an extremely rare conduction disorder characterized by a short PR interval (less than or equal to 120 ms) with normal QRS complex on electrocardiogram associated with the occurrence of episodes of atrial tachyarrythmias (e.g. atrial fibrillation, atrial tachycardia). Wolff-Parkinson-White (WPW) syndrome is the best known LGL syndrome.

You may wish to connect with others in this discussion group:
– Wolff-Parkinson-White (WPW) syndrome https://connect.mayoclinic.org/discussion/wolff-parkinson-white-syndrome/

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@colleenyoung

Lown-Ganong-Levine syndrome is an extremely rare conduction disorder characterized by a short PR interval (less than or equal to 120 ms) with normal QRS complex on electrocardiogram associated with the occurrence of episodes of atrial tachyarrythmias (e.g. atrial fibrillation, atrial tachycardia). Wolff-Parkinson-White (WPW) syndrome is the best known LGL syndrome.

You may wish to connect with others in this discussion group:
– Wolff-Parkinson-White (WPW) syndrome https://connect.mayoclinic.org/discussion/wolff-parkinson-white-syndrome/

Jump to this post

Thank you for your reply. I have searched the Lown-Ganong-Levine syndrome and understood it is different from the WPW syndrome and that ablation will not help. Hmm? Is that incorrect?

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@baz10

Thank you for your reply. I have searched the Lown-Ganong-Levine syndrome and understood it is different from the WPW syndrome and that ablation will not help. Hmm? Is that incorrect?

Jump to this post

@baz10, your question is beyond my knowledge. However, I found this explanation online:
"The main distinguishing feature between LGL and WPW syndromes is that the accessory pathway in LGL syndrome connects distally to the normal conduction pathway (bundle of His), and in WPW the accessory pathway connects to the ventricular myocardium. Thus, both disease states can have a short PR interval, however LGL syndrome will not have a delta wave or widened QRS complex as seen in WPW syndrome. This is because ventricular activation occurs normally in LGL syndrome instead of occurring from myocyte to myocyte as seen in WPW.

Similar arrhythmias occur with LGL syndrome and WPW syndrome, including atrioventricular reentrant tachycardia (AVRT) and rapidly conducting atrial fibrillation. Treatment is similar to WPW and procainamide is frequently utilized. Ablation of the accessory pathway is more difficult in LGL syndrome compared to WPW syndrome since it is located very close to the AV node and there is risk of causing complete heart block necessitating permanent pacemaker implantation." https://www.healio.com/cardiology/learn-the-heart/ecg-review/ecg-topic-reviews-and-criteria/lown-ganong-levine-syndrome-review

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