Especially for Families re. Children with BAV

Valve Replacement Forums

Help Support Valve Replacement Forums:

This site may earn a commission from merchant affiliate links, including eBay, Amazon, and others.

Arlyss

Well-known member
Joined
Nov 7, 2002
Messages
447
Location
southern California
Sometimes when a BAV is found in one family member, parents wonder when they should have their children screened. There are many good reasons why it is best to know - and one of them is the risk of infection - endocarditis.

According to the following paper by C. Ward "Clinical Significance of a Bicuspid Aortic Valve", infective endocarditis statistics show endocarditis is the greatest threat to children and young adults, accounting for 55% of the deaths under age 30 compared to only 13% over age 70.

http://heart.bmjjournals.com/cgi/content/full/83/1/81

Here is a link to an account of the tragic experience of a family in England who lost their 10 year old son to endocarditis - inside his chest was an undetected BAV.

http://www.bicuspidfoundation.com/Memories_of_Sahib_Singh_Vaseer.html

Perhaps Sahib's story may help save another child's life.

Arlyss
 
Found this article interesting.....

Screen Siblings, Parents of Infants with Severe Heart Abnormalities

HOUSTON, TX -- September 7, 2004 -- Brothers and sisters as well as parents of infants born with severe, life-threatening abnormalities of the left side of the heart should be screened for less severe, but related, heart problems, said researchers at Baylor College of Medicine (BCM) in Houston.

In a new study in the September issue of the journal Pediatrics, Drs. Jeffrey Towbin, BCM professor of pediatrics at BCM, and John Belmont, BCM professor of molecular and human genetics, found that these first degree relatives of children with severe defects of the left side of the heart had a fivefold increased risk of having an abnormality called bicuspid aortic valve.


The finding solidifies the currently held belief that such disorders are related to several genes - as few as two and as many as six, said Belmont.
?What this tells us, is that multiple other relatives including parents and siblings are at risk for progressive heart disease and that they should be screened by a cardiologist,? said Towbin, also chief of cardiology at Texas Children?s Hospital. At present, specialists do not recommend screening of close relatives of these children with severe heart defects.


A normal aortic valve has three cusps or ?flaps? that open and close to regulate the flow of blood. A bicuspid valve has only two. Individuals with this disorder are predisposed to heart valve infections and a narrowing of the valve later in life.


Approximately 0.9 percent of people in the general population have bicuspid aortic valve, but the rate among brothers, sisters and parents of these infants is five times greater, said Belmont.


?This is an impressive statistic, as a significant number of these individuals will develop symptomatic disease later in life, and could require catheter or surgical procedures on the heart,? Towbin said.


The infants? severe heart defects included congenital aortic valve stenosis, coarctation of the aorta and hypoplastic left heart syndrome.


Congenital aortic valve stenosis involves a narrowing of the valve that impedes the pumping of blood from the heart. Coarctation of the aorta is a narrowing of the aorta, the largest artery in the body. Hypoplastic left heart syndrome means that the left side of the heart did not develop properly and cannot adequately pump blood to the rest of the body. In their study, Belmont and Towbin evaluated ultrasound-generated images of the hearts of 278 relatives of 113 children. Thirteen had bicuspid aortic valve and 32 had a variety of abnormalities involving the left side of the heart.


Belmont said it is difficult to determine whether the 32 other abnormalities found represent an increase because data do not exist that show how common they are in the general population.


?For many of those individuals, the problems are medically significant and need to be followed up by a cardiologist,? he said. ?Before, we would have completely ignored these people. Now we know they need medical follow-up.?


Others who participated in the research include Drs. Mark B. Lewin, Kim L. McBride, Ricardo Pignatelli, Ana Combes, Andres Menesses, Wilbur Lam and Louis I. Bezold and Susan Fernbach, all of BCM and Dr. Norman Kaplan of Research Triangle Park, NC.



SOURCE: Baylor College of Medicine
 
Thanks Arlyss...

condolences to Sahibs' family...what a lovely young fellow and a terrible loss for the family

I think prevention of Endocarditis is probably the main reason I am so glad I know about our boys and their BAV's. Sure they may never have an infection but there is always that odd chance they could get one which is where I as a parent would find it very very hard to excuse myself for not investigating/screening them so we can protect them as much as possible.
 

Latest posts

Back
Top