Treatments Hard to Come By...

Take a peak at this entry on the Wall Street Journal blog... it doesn't come as a surprise to patients and families of CHD patients, but it's news all the same:

There is not enough research into CHDs.

What's wrong, however, is the statement that this problem is too small for companies to invest research in. With 1 in 120 (approximately) kids born with heart defects, and a large percentage of those kids needing intervention, you can bet it's big enough to pay attention to.

The article does highlight, though, that there is hope in terms of new research. According to WSJ, the NIH is studying treatments in children with certain heart conditions and the American College of Cardiology is launching a catheter registry that aims to help inform doctors who treat kids. And at least one heart device for kids appears to be on the way: Medtronic’s experimental heart valve for both children and adults who had heart defects at birth.

Baby steps, but we can get there!

Never Give Up

An article published by Associated Press today left me dumbfounded. It reminded me to never, ever give up hope. You've GOT to read this article in its entirety (see summary below). See if you feel the same...

British girl's heart heals itself after transplant

Excerpted from Maria Cheng, Ap Medical Writer – Tue Jul 14, 12:40 am ET

In 1994, when Hannah Clark was eight months old, she developed severe heart failure and doctors put her on a waiting list to get a new heart. But Clark's heart difficulties caused problems with her lungs as well. To avoid a risky heart/lung transplant, doctors took a chance at a new and novel idea.

Sir Magdi Yacoub of Imperial College London thought that said that if Clark's heart was given a time-out, it might be able to recover on its own. So in 1995, Yacoub and others grafted a donor heart from a 5-month-old directly onto Clark's own heart. After four and a half years, both hearts were working fine, so Yacoub and colleagues decided not to take out the extra heart.

However, the powerful anti-rejection drugs Clark was taking caused cancer, which led to chemotherapy. Even when doctors lowered the doses of drugs to suppress Clark's immune system, the cancer spread, and Clark's body eventually rejected the donor heart.

In February 2006, doctors removed Clark's donor heart. Luckily, by that time, Clark's own heart seemed to have fully recovered. Since then, Clark — now 16 years old — has started playing sports, gotten a part-time job, and plans to go back to school in September.

At the moment, doctors aren't sure how that regeneration happens. Some think there are a small number of stem cells in the heart, which may somehow be triggered in crisis situations to heal damaged tissue.

Granted, Hannah's case is a rare and miraculous one, but it does give us a window of hope for the future!

Side note - even if this were to become an option for treatment in the future, there is a serious shortage of donor organs available for such transplants. Consider becoming an organ donor today, and asking your loved ones to do the same!

Great News: Scientists ID'd Heart Stem Cells!

Source: Constance Holden, ScienceNOW Daily News
2 July 2009

I was thrilled to read today that scientists have finally identified the cardiac stem cells that create all of the major cell types in the human heart. In recent years, scientists identified these cells in embryotic mice, but now a team at the Massachusetts General Hospital in Boston have found these same cells (that express the protein Islet 1) in humans. The team reports its work in Nature.

What does this mean? That researchers are one step closer to creating new cardiac stem cells in the heart's major cell types: heart muscle (cardiomyocytes), smooth muscle, and blood vessel lining (endothelium).

Chien, the researcher who made this astounding discovery, cautions that these primordial stem cells could not be used for therapy because they could develop into undesired cell types, but they could be used for disease modeling and drug screening and - most importantly to those of us affected by CHD - further research on congenital heart malformations. Chien speculates that CHD's "may be a stem cell disease" because Islet 1 cells are clustered in areas that are "hot spots" for heart defects.

The future I hope it holds for us? The possibility of growing human "heart parts" (such as strips of muscle or a valve) to assist CHD survivors.

Physical Restriction after Fontan: How do You Know?

A recent article in the American Heart Journal from researchers at the Hospital for Sick Children in Toronto questions the use of physical activity restrictions for children after undergoing the Fontan operation. In the abstract online, the researchers studied how the information regarding restrictions are conveyed from the cardiologist, parent and in the medical charts - often these three sources disagree. Regardless of the cardiologist reported restrictions, 70% of parents reported that their child's activity was restricted. Parents and cardiologists report the same body contact restrictions, but differ for exertion and competition restrictions. Medical charts reflect competitive sport, but not body contact or exertion restrictions. So, what to do if you're a parent?

This study suggests that parents are left with uncertainty about physical activity, and rightly so, given the significant variance in the sources of information they receive.Primary care doctors are often not included in the loop (medical records don't reflect the restrictions for exertion and body contact).

Cardiologists need to work to ensure that activity restrictions are accurately conveyed, which will enable parents and primary care providers to confidently encourage the active lifestyles. And parents, talk to your cardiologists - don't take the advice of the primary doctor (though he or she may be correct). While your primary care physician is likely a wonderful, well rounded person, the specialist is better able to answer the questions specific to your child's condition.

Mapping the Heart: Cross-functional Research Leads to Breakthrough

Source: http://www.bjhcim.co.uk/news/2009/n904026.htm

Imagine if imaging of the earth's inner core could lead through to a breakthrough in cardiology... and you will get an idea of what is happening at Bournemouth University.

A computer modeling program was created by Professor Nick Petford to study how liquid metal flows through cracks during planet formation. “For a long time people thought the flow of liquid iron along the edge of grains and through narrow channels and cracks was not possible. NERC funding allowed me to develop a technique for importing object images of rock slices into a software package and then run a fluid-flow computer simulation to look at how liquid metal inside a meteorite moves around under pressure.” Then he realized that the software could be used to do so much more...

Working with a heart radiographer at the Royal Bournemouth Hospital, he used the same simulation technology to look at the blood flow in a diseased human heart. They used data from an actual MRI scan, adjusted the viscosity and density of the fluid to reflect that of human blood, then solved the equations to discover where a blockage existed.

The MRI had not shown an obvious blockage, but utilizing the software, they predicted, exactly, where a clot was located. Just imagine the implications for future studies in this area and earlier diagnoses for patients!

A detailed article regarding this research can be found at: www.nerc.ac.uk/planetearth.

Stem Cells: What Does it Mean for Us?

By now you've likely heard the news - Obama has lifted the ban on stem cell research, which will open the way for many new lines of embryonic stem cells. It's controversial to many, but what does it REALLY mean in terms of research and innovation for the creation of heart tissue?

First, let's clarify what, exactly, embryonic stem cells are. Human embryonic stem cells are our body's most versatile cells, possessing the potential to develop into any cell type in our bodies (with the exception of a placenta). Already such cells have been used to form heart tissues and valves. Embryonic stem cells are created during in vitro fertilization for persons with reproductive issues. Blastocysts are formed, from which the cells are harvested. The permission of the donors are given to donate these cells for research. Only 21 lines of these cells have been researched since 2001, due to a ban Bush placed on further research beyond the existing lines.

In a press release dated March 9, the International Society for Stem Cell Research (ISSCR) applauds President Barack Obama’s executive order, citing its potential to relieve suffering caused by diseases and conditions (including those caused by heart defects). The new policy allows scientists to utilize the many hundreds of valuable lines created since 2001, and relieves them from the substantial hurdles in duplicating equipment and other resources in order to separate privately- or state-funded stem cell research from federal government-funded efforts.

It's a hot topic most of us don't want to discuss in public. But it's a discussion we need to have. What do you think? Does the announcement restore the "integrity to the relationship between politics and science that has been traditional in the U.S.,” as asserted by Irving Weissman, President-Elect of the ISSCR? Would you be a willing recipient of the tissues and valves created in this manner?

The Care Behind the Kids!

Mark O'Shea and the Staff of the Pediatric Heart Institute at Vanderbilt Children's Hospital (Nashville, Tennessee) are back! But this is not a music video... it's a nine minute look at the Doctors, Nurses, and Staff of the Heart Institute and what they do. We present this to you on Valentine's Day with love and best wishes from both Kim and Steve!

Put down the Vicks!

No, not Michael Vick, the former NFL quarterback... that subject is an entirely different post for an entirely different blog! We're talking about Vicks VapoRub, which can be found in nearly every medicine cabinet in America. I bought a bottle myself the last time I was at the drugstore. We all have memories of our moms coating our chests with VapoRub when we were sick. Recent research has proven that we were all doing it wrong.

"The ingredients in Vicks can be irritants, causing the body to produce more mucus to protect the airway," said Bruce K. Rubin, MD, of Wake Forest University. While that's not good for anyone, it's especially not good for small children. And even though the report doesn't mention this, it can't be a good thing for someone with any airway issues... including a Congenital Heart Defect.

The study showed that VapoRub increased mucus secreation 59% in healthy, uninfected lungs. Even when a child is healthy, that much mucus could lead to airway inflammation and irritation, possibly closing the airway. Also, VapoRub doesn't actually make it easier to breathe. The menthol triggers a cold sensation as it enters the nasal cavities, which tricks the brain into believing there is increased airflow.

Links for CHD Families

Here are several very important links for you to read today. Read them online, download them, or print the file, whichever you choose. If you try to download the file as a .pdf document, you won't be able to... the writers are trying to protect their work, you can read the details on the download page. But you can print the entire file, which is just as good! The only bad thing is that these links are from medical journals, written by doctors for other doctors. It's almost 100% medical terminology, and you'll probably have to have an interpreter.

Tetralogy of Fallot (ToF) in a seven year old girl

Some thoughts on Tetralogy of Fallot (ToF) surgery

The Fontan Procedure... what's down the road?

This next link refers to the Glenn Shunt, and has a very good illustration. The illustration, however, is of the Classic Glenn Shunt, which was first used in the 1950's. The modern version, the Bi-directional Glenn Shunt, was first described in 1966 and not popular until the 1970's.

Click the .pdf button to be taken to a three page file concerning both versions of the Glenn Shunt. This is important reading for anyone who has a Glenn!

This link has more layers of information than a seven layer Chocolate Cake! Above the illustration, click the heading labeled "Contents"; there you can find 40+ links to heart defects, surgical procedures, and case studies. There is lots of information here, and not just on the Glenn Shunt.

Stem Cells May Help Repair Valve Defects!

Bloomberg Science, Nov. 10 -- Cardiologists at the University Hospital of Munich believe that umbilical cord blood, rich in stem cells, may bre the raw material needed to repair the hearts of thousands of children born each year with defective heart valves. The researchers reported the findings at the November 10 annual meeting of the American Heart Association. They believe they are 5 to 7 years away from transplanting new valves created from cord blood with patient's faulty valves.

The Munich researchers are ready to begin trials in lambs, using collected cells (frozen for preservation) to seed into biodegradable polymer scaffolds. Thus far, eight bio-engineered valves have acted much like natural heart valves when tested for blood flow and pressure. The scaffolds will disolve over time, leaving behind a fully formed structure made from the cells, they assert. The next step will be to transplant the heart valves into hearts of young lambs, watching them to see how they grow and function over time. The experiments are hoped to begin next year.

Click here to read more...

No Drop in IQ Seen After Bypass

ScienceDaily, Nov 10, 2008 -- Good news for older children facing cardiopulmonary bypass, or CPB. Scientists at the Cardiac Center of Children's Hospital recently conducted a study of children aged five to 18 who underwent heart surgery while on a heart-lung machine to circulate their blood. The research found that the use of CPB does not cause short-term neurological problems in children and teenagers after surgery for less complex heart defects (such as valves or ASDs/VSDs), according to pediatric researchers. The new finding contrasts favorably with previous studies that showed adverse neurological effects after newborn surgery for more complex heart conditions.

Click here to read more...

Online Discussion on Adult CHD to be held October 2

Next Thursday, October 2, 2008 at 12 noon (EST) Cleveland Clinic will host an online health chat on Adult Congenital Heart Disease.

During this chat, Dr. Richard Krasuski, Director of Adult Congenital Heart Disease Services and a staff cardiologist in the Section of Clinical Cardiology, Department of Cardiovascular Medicine at Cleveland Clinic Heart and Vascular Institute, explores the treatment options available for adults living with a congenital heart disease. More information on this chat can be found at http://www.clevelandclinic.org/health/ChatReg/Krasuski.html.

Dr. Krasuski's specialty interests include adult congenital heart disease including atrial septal defects, patent foramen ovale, ventricular septal defects, coarctation of the aorta, Ebstein anomaly, Eisenmenger syndrome, transposition of the great arteries, Tetralogy of Fallot and patent ductus arteriosus. He also specializes in pulmonary hypertension, mitral valve disease, cardiac catheterization, transesophageal echocardiography and mitral, aortic and pulmonic valvuloplasty.

Research Yeilds Answers on Aortic Arch Defect Causes

By The Pittsburgh Tribune-Review
Tuesday, July 8, 2008

Research led by University of Pittsburgh scientists is the first to describe how two common congenital circulatory problems form, the university announced today.

The team found that a gene called unc45a plays a critical and previously unknown role in the formation of aortic arch vessels. The vessels contribute to several of the body's major arteries and often develop improperly, causing a wide range of vascular defects.

The team also found that arteriovenous malformations, or AVMs, happen when an artery fuses with a vein, diverting blood flow, and result from genetic and physiological factors. Previously doctors had believed its origins were solely genetic.

The research is published in the journal Developmental Biology. Scientists at Georgetown University Medical Center and the National Institutes of Health also participated.

Stereoscopic Glasses May Aid Beating-Heart Surgery

Source: iTNews, 10 June 2008

In a recent study, the three-dimensional glasses have been used in a successful operation on pigs with a common form of congenital heart disease. The newly-tested stereoscopic glasses enable depth perception by splitting computer images in two, and cocking them at slightly different angles to provide surgeons with ultrasound images of the beating heart as a hologram.

Researchers expect that clinical trials of beating-heart surgery with the patching system could begin in children with ASDs this year.

$1 Million Gift to Support Earlier CHD Detection

Washington, DC (PRWEB ) May 15, 2008 -- The Elsie & Marvin Dekelboum Family Foundation has announced a gift of $1 million to Children’s National Medical Center. The gift will allow the Children’s National Heart Institute to launch a Congenital Heart Defect Screening Study. Children’s National will name a three-bed bay in the new Cardiac Intensive Care Unit in honor of the Elsie & Marvin Dekelboum Family Foundation.

The heart defect screening study will focus on the effectiveness of an inexpensive device called a pulse oxymeter that could help identify more than 80 percent of life-threatening congenital heart defects. If initial findings are validated, the Dekelboum gift will enable Children’s National to promote its use in hospitals across the country and around the world – improving early detection and health outcomes for children everywhere.

Send Flowers to Mom & Help Raise Funds for Research!

LINCOLNSHIRE, Ill., May 5 /PRNewswire/

The Children's Heart Foundation today announced a new partnership with ProFlowers(R) -- the online fresh flower marketplace -- just in time for Mother's Day! Shoppers can visit http://www.proflowers.com/CHF and choose from a variety of beautiful, fresh-from-the-field flower arrangements. All year long, whenever flowers are purchased through the CHF page on ProFlowers, ten dollars from each purchase will benefit The Children's Heart Foundation (check out their new website!).

"Sending flowers to the mothers and grandmothers in your life is a great way to show your appreciation and celebrate their love -- and now it also can help raise much-needed funding to find better treatments and, hopefully someday a cure, for congenital heart defects" said William Foley, Executive Director, The Children's Heart Foundation.

The Children's Heart Foundation is the country's leading organization solely committed to congenital heart defect (CHD) research funding and is comprised of parents, family members and friends of those affected by a congenital heart defect. The goal of The Children's Heart Foundation is to increase awareness, support the recently diagnosed through educational materials and continue funding the most promising research to find better treatments.

CHD and Neurodevelopmental Risks

Risk of attention and behavior problems for kids with CHD
Children's Hospital of Philadelphia
Public Release, 7 April 2008

Schoolchildren who required surgery as infants for congenital heart disease (CHD) run a significant risk of having problems with inattention and hyperactivity, and often require remedial services in school. “These children are at risk for academic and behavior problems, and our findings reinforce how important it is to provide them with ongoing follow-up and neurodevelopmental screening,” said study leader Amanda J. Shillingford, M.D., a pediatric cardiologist at The Children’s Hospital of Philadelphia.

The researchers, who reported their findings in the April issue of Pediatrics, studied a group of 109 children, aged five to 10, who had undergone cardiac surgery for complex congenital heart disease at Children’s Hospital when they were newborns. Of that group, 53 children--nearly half of them--were receiving remedial services at school, and 15 percent were in special education classrooms.

Based on questionnaire responses from their parents and teachers, although the majority of the children with CHD scored in the normal range, the rates of high-risk scores for inattention and hyperactivity were three to four times greater than those found in the general population.

Previous studies at The Children’s Hospital of Philadelphia and other centers found that school-aged children with complex CHD tended to have normal cognitive abilities but were at risk for problems in visual and motor skills, as well as impairments in speech, language and executive functioning (executive functioning refers to capacities for attention, planning, decision-making and problem-solving).

“As survival rates have improved,” said Shillingford, “the important longer-term issue is quality of life for patients and their families as they reach school age and beyond. We hope our findings will help raise awareness among parents, teachers and physicians about the children’s risk of neurodevelopmental problems.” Shillingford added that a next step for researchers is to conduct larger, multicenter studies, with more formalized diagnostic tools, and to develop formalized follow-up protocols for these children. Such follow-up programs are currently being designed at Children’s Hospital.

New Techniques for Stenting in Adults with CHD

A pioneering paper was presented at last week’s Society of Interventioanl Radiology meeting, in Washington, DC by Dr John Moriarty, Specialist Registrar in Dublin’s Mater Hospital. The Mater is the only place in Ireland and one of a few places in Europe that places pulmonary valves in adult patients via stenting. This procedure, which is not yet licensed in the US, was examined with CT during Dr Moriarty’s presentation.

Moving beyond diagnosis and into therapy, interventional radiologists have new imaging techniques and new ways of getting into organs which, when combined, give an extremely powerful new tool that is changing the course of care for patients, in quality as well as quantity of life.

Posted in Research and Education on 29 March 2008

Transposition of the Great Arteries (TGA) and Genetics

The American Heart Association posted a new "Ask the Pediatric Cardiologist" question today on DTGA and genetic factors, including increased risk of having children in the future who also have heart defects. For information, click here. According to this source...

For a couple who's had one child with DTGA and has no other family members with transposition, the chance of having another child with DTGA increases slightly to about 18 in 1,000 or 1.8 percent. This is also about the same chance of this baby being born with a form of congenital heart disease other than DTGA. This gives a greater than 98 percent chance that the baby will be born with a normal heart.

Canadian Research News

For the complete article from LabCanada.com, please click here.

SickKids Foundation and the University of Toronto announced today a $2 million dollar donation from BMO Financial Group, which establishes the BMO Financial Group Chair in Cardiology. World-renowned SickKids clinician and researcher, and University of Toronto professor in paediatrics, Dr Andrew Redington has been named the inaugural chair holder.

The $2 million gift will allow Dr Redington to continue to pursue research and contribute to the understanding of congenital heart disease and heart function. Dr Redington's group is currently studying a novel technique developed at SickKids, that has already been shown to protect the heart and other organs from damage due to restricted blood flow during heart surgery.

"Through BMO's generosity and Dr Redington's vision, today's announcement effectively solidifies SickKids as a world leader in children's cardiac medicine," said Mary Jo Haddad, president and CEO at SickKids. "An endowed chair for our head of cardiology is a powerful tool in both retaining people like Dr Redington while recruiting others with similar credentials in the years to come."

Under The Labatt Family Heart Centre headed by Dr Redington, SickKids currently performs 80% of all children's heart transplants in Canada and cares for 80% of all the children in Ontario with congenital heart defects. More than 60% of children requiring heart surgery are under one year of age, and almost a quarter of children requiring surgery are in their first month of life.