Pediatric Aortic Valve Disease

The aortic valve is one of four valves (tissue flaps) in the heart that are vital to its normal function. With each heartbeat, the tissue flaps open and shut, working in concert with contraction and relaxation of the heart chambers to propel blood through the heart’s four chambers and to the rest of the circulation.

Heart valves keep blood flowing in a forward direction. The normal heart has four valves:

Left Side

• Mitral valve - allowing flow from left atrium to left ventricle
• Aortic valve - allowing flow from left ventricle to body circulation

Right Side

• Tricuspid valve - allowing flow from right atrium into right ventricle
• Pulmonary valve - allowing flow from right ventricle to lung circulation

A hardened or fused valve, also known as aortic stenosis, leads to obstruction, forcing the left ventricle (the heart’s lower left chamber) to work harder to pump blood into the aorta.

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A leaky aortic valve, also called aortic insufficiency (otherwise, called aortic regurgitation), is a valve that does not shut properly. Therefore, blood leaks back into the left ventricle. The ventricle enlarges in response to accommodate the leaking volume of blood in excess of the usual volume of blood, forcing it to use more effort to expel blood into the body.

Some valve defects have both problems. A stiff valve partially obstructs blood flow from the heart, but since it also does not close properly, blood leaks.

These defects overtax the left ventricle and, with time, the ventricle can fail to perform properly.

Many valve defects occur before birth. Aortic valve disease can be congenital (a child is born with the valve problem) or it may be acquired later in life. A normal aortic valve has three “cusps” or leaflets. Some children are born with a bicuspid valve having only two leaflets. Often times, a bicuspid valve does not require any intervention until much later in adulthood.

Untreated strep throat can develop into rheumatic fever, which in turn, can scar the valve. That is why taking a full course of prescribed antibiotics is vital to treat these symptoms.

In infants, lethargy, poor feeding (or poor food intake) and difficulty breathing may signal a valve problem. Rarely, fainting or chest pain also may indicate a problem for older children.

Though valve disease may have no warning signs, doctors may hear an odd, whooshing sound (or heart murmur) via stethoscope. The following tests may be utilized to find more details:

• An electrocardiogram (EKG or ECG) can check the heart’s electrical action to reveal damage or irregular rhythms.
• Chest X-rays create images of the heart and lungs and can show fluid in the lungs due to valve defects.
• An echocardiogram (echo) uses sound waves (ultrasound) to produce images of the heart and vessels on a screen that reveals whether the heart is pumping properly.
• A cardiac MRI (magnetic resonance imaging) uses radio waves, magnets and a computer to form three-dimensional images of the heart, which can reveal structural abnormalities.
• Cardiac catheterization involves a thin, long tube that is inserted into a blood vessel – usually from the groin – and guided into the heart to obtain direct pressure measurements and angiograms (X-ray images of the heart chambers and valves). It may provide therapy for aortic valve stenosis using balloon-tipped catheters (balloon aortic valvuloplasty).

Aortic valve repair is possible occasionally, although this is uncommon. Young children with aortic stenosis can have their valves opened, either by a balloon procedure or by open surgery; however, this may not always be performed successfully. Older children and young adults can rarely have aortic stenosis repaired surgically. Even less commonly, aortic insufficiency can be repaired surgically. Thus, most people who have significant aortic valve problems require aortic valve replacement.

There are many types of replacement valves – each comes with its own set of advantages and disadvantages. In the table below, the term anticoagulation refers to the need to take "anticlotting" medications. It is imperative that patients maintain careful follow-up so the amount of medication they receive is correct, and sometimes their lifestyle needs to be modified.

Aortic valve disease affects each child differently. Your physician is always your best resource for providing you with the best treatment options specific to your child’s condition.

At Children’s Heart Institute at Children’s Memorial Hermann Hospital, patients with congenital or acquired heart disorders receive hands-on specialized care 24/7 from a team of affiliated physicians and specialty-trained nurses who aim to deliver the best possible outcomes.

Children’s Memorial Hermann Hospital was named one of the top children's hospitals nationally in Cardiology & Heart Surgery by U.S. News & World Report. In addition, Children’s Heart Institute is among the top congenital heart surgery programs in North America for patient care and outcomes, according to the Fall 2019 Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database Report of 118 STS participating programs.

In collaboration with various subspecialties, the affiliated team provides comprehensive care for newborns, children and adolescents, with the ability to transition into adult congenital cardiac care. Team members have the experience and skills necessary to offer innovative treatment methods and specialized services, including, but not limited to:

• Biventricular repairs and biventricular conversions
• Congenital heart optimization
• Full repairs for complex congenital heart defects in newborns
• Hybrid catheterization and surgical procedures
• Minimally invasive transcatheter pulmonary valve (TPV) therapy
• Minimally invasive repairs
• Treatment for adult congenital heart disease
• Valve repairs and preservation

With the Level IV Neonatal Intensive Care Unit (NICU) and a dedicated Children’s Heart Institute Intensive Care Unit at Children’s Memorial Hermann Hospital, critical heart patients have access to quality, specialized care. By utilizing state-of-the-art techniques, the team at Children’s Heart Institute strives to offer patients with the most complex problems the greatest opportunity for a normal life.

If you have any questions, use the online tool below to help us connect with you. To refer a patient or schedule an appointment, please contact our clinic using the information below.

• Pediatric Cardiology Clinic
  The University of Texas Health Science Center Professional Building
  6410 Fannin, Suite 370
  Houston, TX 77030
  Phone: (713) 486-6755 (Appointment Line)
   
• Pediatric and Congenital Heart Surgery Clinic
  The University of Texas Health Science Center Professional Building
  6410 Fannin, Suite 370
  Houston, TX 77030
  Phone: (713) 500-5746
  CMHH-Heart@memorialhermann.org

The Children’s Heart Institute is a collaboration between the affiliated physicians at McGovern Medical School at UTHealth Houston and Children’s Memorial Hermann Hospital. Typically, patients are seen on an outpatient basis at a UT Physicians clinic with all inpatient procedures performed at Children’s Memorial Hermann Hospital.