Jervell and Lange-Nielsen syndrome – What are the signs and symptoms of Long QT syndrome ?

Long QT syndrome
Long QT syndrome

Long QT syndrome causes problems with the electrical activity of the heart. It’s uncommon, occurring in around 1 in every 2,000 people.

Long QT syndrome is often the result of a faulty gene that’s inherited from one of your parents. The abnormal gene causes an imbalance in the chemicals that create the electric impulses in your heart.

The syndrome can also be caused by medicines for other conditions.

Symptoms of long QT syndrome

There are usually no physical signs of long QT syndrome, and some people don’t experience any symptoms.

The most common symptoms are blackouts or seizures caused by the interruptions to the heart’s rhythm.

Some people with long QT syndrome find that their heart sometimes suddenly beats in an abnormally fast, uncontrollable way. This type of arrhythmia is called “torsade de pointes”.

When this happens, the heart can’t pump blood properly and the brain is temporarily starved of oxygen, causing the person to temporarily pass out. The heart’s rhythm usually returns to normal within a minute or so and the person regains consciousness.

These episodes can start at any age and may be triggered by:

stress

a sudden noise – such as an alarm bell

strenuous exercise – particularly swimming

a slow heart rate during sleep

However, there isn’t always a trigger.

Sometimes, the abnormal heart rhythm persists, leading to ventricular fibrillation (a rapid, uncoordinated series of contractions). This rarely reverts to normal without medication and, if it isn’t electrically corrected, usually causes the heart to stop pumping (cardiac arrest) and death.

Dial 999 to request an ambulance if someone with long QT syndrome suddenly collapses. Perform cardiopulmonary resuscitation (CPR) until medical help arrives.

Long QT syndrome is a leading cause of sudden cardiac death in young, otherwise healthy people, and is often thought to be an underlying cause of sudden infant death syndrome (SIDS).

What causes long QT syndrome?

To understand the underlying cause of long QT syndrome, it’s important to know how the heart cells work.

On the surface of each heart muscle cell are tiny pores, or ion channels. These open and close to let electrically charged sodium, calcium and potassium atoms (ions) flow into and out of the cells.

This passage of ions generates the heart’s electrical activity. The electrical signal spreads from the top of the heart to the bottom, causing the heart to contract and pump blood.

In most cases of long QT syndrome, the flow of potassium ions out of the heart muscle’s cells is delayed. This means that after each heartbeat, your heart can take longer to reset itself.

Inherited long QT syndrome

Long QT syndrome is often inherited from a parent as a faulty gene. The abnormal gene affects the proteins that make up the ion channels in the heart cells. The ion channels may not work well, or there may not be enough of them, which disrupts the heart’s electrical activity.

Cardiac Risk in the Young has published a list of medications that people with long QT syndrome should avoid.

Drug-induced long QT syndrome usually happens in people with an inherited higher risk of developing it, such as those with slight genetic heart defects.

For more information, you can read the Sudden Arrhythmic Death Sydrome’s (SADS UK) guide about acquired, drug-induced long QT syndrome (PDF, 158kb).

What does ‘long QT’ mean?

Every time your heart beats, it produces tiny electrical signals. An electrocardiogram (ECG) machine traces these signals on paper – a typical pattern is shown below.

As the graph shows, each heartbeat is mapped as five distinct electrical waves – P, Q, R, S and T. The part of the pattern from Q to T represents the electrical activity of the heart’s lower chambers, or ventricles.

In people with long QT syndrome, this QT interval lasts abnormally long. In other words, it takes longer for the heart cells in the ventricles to recharge after each heartbeat. This can upset the timing of the heartbeat and may trigger an abnormally fast heart rhythm.

Diagnosing long QT syndrome

If your GP thinks you have long QT syndrome after assessing your symptoms, they may recommend that you have an ECG and refer you to a heart specialist (cardiologist).

In particular, if blackouts have occurred during exercise, or if there’s a family history of sudden cardiac death below the age of 40, specialist assessment is needed.

An ECG is a test that records your heart’s rhythm and electrical activity. If you have long QT syndrome, the trace of the QT section will be longer than normal.

During an ECG, sticky pads called electrodes are stuck on your arms, legs and chest, and connected by wires to an ECG machine. The test may need to be carried out while you exercise on a treadmill, as well as during rest.

Genetic testing may be needed to identify the defective gene that may be causing long QT syndrome. It can also help to determine which family members may have inherited the defective gene and need clinical assessment.

Treating long QT syndrome

Most people with inherited long QT syndrome will need treatment with medicines. Beta-blockers, such as propranolol or nadolol, may be prescribed to help control irregular heartbeats and slow down your heart rate.

If your symptoms are frequent or severe, and you have a high risk of having a life-threatening arrhythmia, you may need to have a pacemaker or implantable cardioverter defibrillator (ICD) fitted.

Like pacemakers, ICDs are small battery-powered devices. If the ICD senses that the heart is beating at a potentially dangerous abnormal rate, it will deliver an electric shock that returns the heart rhythm to normal.

In some cases of long QT syndrome, surgery may be needed to control the flow of chemicals into the heart.

If you have long QT syndrome caused by taking medication, your medication will be reviewed. It may be possible to prescribe alternative medication that doesn’t adversely affect your QT interval.

Living with long QT syndrome

With appropriate treatment, such as medication or surgery, it should be possible to lead a relatively normal lifestyle. However, you may need to make some lifestyle adjustments to reduce your risk of having blackouts.

For example, your doctor may advise you not to exercise strenuously or play contact sports, and to try to avoid startling noises, such as alarm clocks or telephones with loud ringtones. Avoiding stressful situations may also be recommended.

Your doctor may prescribe potassium supplements or suggest that you increase the amount of potassium-rich foods in your diet. Good sources of potassium include:

fruit – such as bananas

vegetables

pulses

nuts and seeds

milk

fish

shellfish

beef

chicken

turkey

bread

Always tell medical staff that you have long QT syndrome. Any new medication, both prescription or over-the-counter, will need to be carefully checked to see if it’s suitable for you.

First look at new home for Alder Hey Children’s Hospital – watch our video and read the interview.


  • Children’s designs inspire Europe’s first hospital in a park.

  • £250m scheme includes a brand new state of the art hospital along with a dedicated research, education and innovation facility.

Alder Hey Children’s Hospital will start its move into a brand new state of the art facility tomorrow.

New Alder Hey Hospital Liverpool. Images by Gareth Jones
New Alder Hey Hospital Liverpool.
Images by Gareth Jones
The new ‘Alder Hey in the Park’ will feature a uniquely designed children’s hospital alongside a dedicated children’s research and innovation facility, creating a leading-edge Centre for children’s healthcare and research. The entire hospital will relocate during a five day move from 2nd to 6th October.

The new state-of-the-art hospital within ‘Alder Hey in the Park’, has been inspired and designed with the help of children and young people.

It will have 270 beds, including 48 critical care beds for patients in ICU, HDU and Burns, together with 16 digitally enhanced operating theatres. The majority of children and their families will have their own room and en-suite facilities; with each ward equipped with its own kitchen providing freshly cooked food to order.

Europe’s only hospital in a park, the unique design of the building will provide a pleasant healing environment for children and young people. Almost all bedrooms have park views and patients will have easy access to play areas on and outside their ward. Alder Hey’s old buildings will be demolished and replaced by parkland, leaving a new hospital sitting in the heart of Springfield Park, with views of green space or parkland from every patient’s window.


 

The designs for the new world class facility have been inspired by children. Over 900 young people taking part in the initial consultation; with one patient, Eleanor Brogan, inspiring the architects final design with her drawing. Alder Hey in the Park has been designed to meet the expectations of patients who wanted to be looked after in a place that was fun, helped them recover quickly and most importantly didn’t look like a hospital. A Children and Young People’s Design Group have continued to have their say throughout the design process on everything from the colour of their room, to the artwork displayed in the new hospital and what the wards should look like.

Alongside the new hospital, Alder Hey has also built an innovative Research and Education Facility. The new facility will place Alder Hey and Liverpool as a national and international leader in the development of safer and more effective medicines for children and young people. The new facility will allow researchers and clinicians to work with industry to develop safer, better medicines and therapies for children to use in the NHS and throughout the world.

Alder Hey Children’s Charity is helping to transform this NHS hospital into a world-class facility. The Charity has already raised over £20m towards the new hospital, providing lifesaving equipment, funding for vital research and patient experience initiatives. Alder Hey Children’s Charity continues to raise much needed funds to create a truly patient friendly environment and support cutting edge innovation and research.

 

Alder Hey is one of the UK and Europe’s largest children’s hospitals and celebrated its centenary last

New Alder Hey Hospital Liverpool. Images by Gareth Jones
New Alder Hey Hospital Liverpool.
Images by Gareth Jones
year. During its 101 year history, the hospital has led the way in a number of significant medical breakthroughs and advancements including being the first hospital to test Penicillin, saving a child from pneumonia in 1944; the first hospital to establish a neonatal unit in the UK; the first to cure the UK’s most commonly encountered congenital heart defect; as well as pioneering various splints and appliances including the Thomas Splint.

The move to the new building has been meticulously planned over the past 18 months to ensure a safe and smooth transition for Alder Hey patients and their families. All non-emergency activity during the move to the new hospital will be reduced. However, the hospital’s Emergency Department will remain open, officially relocating to its new home in the early hours of Sunday 4th October.

As a specialist hospital, Alder Hey currently provides 275,000 episodes of care to children and young people every year as one of Europe’s biggest and busiest centres of excellence for children with cancer, heart, spinal and brain disease.

Watch this video for a sneak peak of the new Alder Hey hospital’s state of the art facilities.

We interviewed Rick Turnick of Alder Hey to tell us more.

PATIENTTALK.ORG Can you tell me a little bit about the history of the hospital?

Rick Turnick – The old hospital was built in 1914 so it’s just over 100 years old. Originally as a children’s hospital but then during both the 1st World War and the 2nd World War we took injured soldiers as well. Obviously since the 2nd World War it’s been a children’s hospital. It’s grown into one of the biggest and busiest in the UK and Europe. During that time we were the first children’s hospital to use penicillin. We were the first children’s hospital worldwide to develop general anaesthesia safely in children and the first in the world to have a purpose built unit to deal with surgery and new born babies.

PATIENTTALK.ORG What is the role of the charity?

New Alder Hey Hospital Liverpool. Images by Gareth Jones
New Alder Hey Hospital Liverpool.
Images by Gareth Jones
Rick Turnick – The charity’s technically a separate organization although it’s housed within the building, both the old one and the new one but the new hospital has been funded mainly through NHS money. What the charity does is they are able to enhance the facilities, so for instance we’ve developed a system of ward based chefs in the new hospital so the children will have bespoke meals cooked for them when they want them, things that they want to eat , we’ve been able to upgrade the beds that we are able to provide, both the ordinary beds in the main ward but also on the critical care unit, they’ve helped us make sure that our operating theatres are absolutely cutting edge and they have helped with developing systems within the operating theatres and also other things like our hydrotherapy pool, the garden that’s both outside and inside the new hospital and the sort of entertainment we are able to offer the children in their cubicles and outside as well, the arts program.

PATIENTTALK.ORG And who will the hospitals serve?

Rick Turnick – Well we have all the children’s beds for the north Liverpool north Merseyside complex so that’s children who are just local with acute illnesses but we also serve a regional population in north west of England but we take patients from all over the UK we take patients from Ireland, we take patients from other parts of Europe and further afield as well.

PATIENTTALK.ORG Why a children’s hospital rather than one for all ages?

Rick Turnick – Well children have different diseases from adults so the reason for a children’s hospital rather than an adult or all ages one means that we can make sure that the environment is safe and effective for their treatment, it means that we can shape the whole ethos and ambiance of the hospital around children and their families rather than having it as a sort of bolt on extra to an adult type hospital.

PATIENTTALK.ORG How will the new building improve healthcare?

Rick Turnick – Well it will improve healthcare by the fact we have been able too not only make everything cutting edge technology at the top end but also that we are able to ensure that our patient flows work properly and the other main factor is when you are working in a 100 year old building keeping it clean just is a mammoth task and at the new hospital we’ve designed it so that infection control and prevention will be one of our key strategies to really nail them and get it absolutely the best.

PATIENTTALK.ORG What conditions will be treated?

Rick Turnick – The conditions that we treat will be everything that a child might have the misfortune to suffer from, from the usual sort of respiratory tract problems that occur in winter through to the most severe forms of cancer, children’s heart surgery, to treating tiny babies, a whole range of children will be treated at Alder Hey.

PATIENTTALK.ORG And what’s the difference between a children’s ICU and a normal ICU?
Rick Turnick – With children’s ICU again its dealing with different diseases so everything is focused, the staff are trained in the treatment of different illnesses, the equipment’s has to be scaled up or down because we treat everything from tiny new-born babies weighing a couple of pounds through to adolescents who are sort of adults sized so it’s got to be much more flexible then an adult ICU and the range of illnesses that you deal with on a children’s ICU is very, very wide as opposed to an adult ICU where you really only have fewer conditions that you have to treat.

PATIENTTALK.ORG And last and final question, what are the current research projects?

Rick Turnick – The current research projects will be focused around our adjacent new research & education building, we host the NAHR medicines for children’s program for the whole of the UK, we’ve got research projects in every area of children’s medicine but practically through our cancer, our oncology, that’s been very strong. hydro therapy pool Orthopaedics, neurosurgery, rheumatology really everything. We want to weave research through the whole hospital in everything we do.

Check out these 11 fascinating facts about the human heart


human heart facts

If you want to know how incredibly important – and complex – the human heart is, check out this interesting infographic:
11 Fascinating Facts About the Human Heart
. Use the embed code to share it on your website or visit our infographic page for the high-res version.

<img src="http://media.mercola.com/assets/images/infographic/human-heart-facts.jpg" alt="human heart facts" border="0" style="max-width:100%; min-width:300px; margin: 0 auto 20px auto; display:block;"><p style="max-width:800px; min-width:300px; margin:0 auto; text-align:center;">If you want to know how incredibly important - and complex - the human heart is, check out this interesting infographic: <a href="http://www.mercola.com/infographics/human-heart-facts.htm"><strong>11 Fascinating Facts About the Human Heart.</a></strong> Visit our infographic page for the high-res version.


Donating an organ! Would you consider becoming an organ donor? Tell us your opinion?


Would you consider donating an organ?
Would you consider donating an organ?

It is a pretty regular thing in the UK , at least, for a news item on the need for more people to donate their organs after their death.  Currently organs that can be donated include :-

  • kidneys
  • liver
  • heart
  • lungs
  • small bowel
  • pancreas

Tissues that can be donated include:

  • the cornea (the transparent layer at the front of the eye)
  • bone
  • skin
  • heart valves
  • tendons
  • cartilage

In the UK and other countries there are plan afoot to make to a presumed consent or opt out system of organ donation.  This means that we will have to state that we do not want our organs to be used for transplantation surgery in the event of our passing as opposed to making a positive choice to donate.

We are really interested in what our readers think.  It would be great if you could take our poll below.

If you have received an organ from a donor it would be great if you could share your story in the comments box below.  Feel free to share any links that you think will be of interest to our readers.

Many thanks in advance.