Valve problems in children with heart disease: What patients and families should know

My name is Dr. Joseph Dearani, and I am the Chair of Cardiovascular Surgery at the Mayo Clinic, with an area of expertise in pediatric and congenital heart disease. So today, I'd like to talk about valve problems in children with heart disease. What would be important for patients and families to know? We'll focus on the leaky valve, or the regurgitate valve, as the physicians call it. Mitral, tricuspid, and aortic valves are the classic valves that could have a leaky problem that would require treatment. Any of them could consist of a variety of diagnoses that range from an actual structural abnormality of the valve, such as Ebstein's anomaly of the tricuspid valve, or congenital, mitral, or tricuspid regurgitation, mitral or tricuspid valves, as they relate to the atrial ventricular septal defect or atrial ventricular canal defect.

Pulmonary atresia with intact ventricular septum can have tricuspid valve problems. And there can also be the bicuspid aortic valve which an individual may be born with. And that value may be vulnerable to narrowing or leakage. Finally, there actually can be iatrogenic injury of a valve during the repair of another lesion. For example, injury to the tricuspid valve during the course of closure of a ventricular septal defect.

There can be valve problems related to dilation of the annulus, which may be a secondary problem to an abnormality with the ventricle. And in the current era, now, there are many congenital lesions that eventually require placement of a pacemaker or an internal cardio defibrillator lead. And these can also create regurgitation. So a very wide range of lesions.

Now the important thing from a patient or family standpoint, and from a physician's standpoint, is the timing of surgery. And this can be actually quite difficult, particularly in children, because many of these leaky valve lesions have minimal symptoms. Sometimes symptoms may not even be present. And it's important to know that there could be quite advanced disease present even in the absence of symptoms.

So what would symptoms include? The classic symptoms with leaky valves would be shortness of breath, fatigue, or in a very young child it may be failure to thrive, that is, poor weight gain. Symptoms may be very subtle, not keeping up with peers, more naps in the afternoon, going to bed earlier in the evening, something that only a parent might notice. Or patients may be completely asymptomatic and actually be doing everything quite well.

In any situation, we do an echocardiogram. And an echocardiogram really helps provide information that also helps with timing of the operation. Importantly, it will give us information about whether the ventricle is dilated or not, and whether the ventricle has any dysfunction or not. The presence of either of those two things would clearly prompt the need for intervention.

When ventricular function gets dilated, and then the atria, the receiving chambers get dilated, then arrhythmias may become present. And the onset or progression of arrhythmias also can precipitate the need for operation. Finally, if the surgeon is quite confident with their ability to repair the valve, even earlier operation may be considered in an effort to avoid abnormality subsequently developing with the ventricle.

Other imaging studies besides Echo, which includes two-dimensional and three-dimensional Echo, is really ideal to determine anatomy of the valve. It really is a roadmap for the surgeon to determine whether or not they can repair the valve. This often is complemented with either magnetic resonance imaging, or CT imaging, which gives important function about the ventricle, particularly the right ventricle, how large it is, and what the function of it is.

Now there are many techniques of repair. And very importantly, the success of repair has a high correlation with surgeon experience. You should specifically ask your surgeon how many he or she has done. There are a variety of repair techniques that get tailored toward the specific abnormality. Techniques might include mobilization of one or more leaflets. It might include augmenting, that is, increasing the size of a leaflet. It may involve placement of artificial cords, that is, artificial strings to take the place of some that may be absent or broken. And some include reducing the size of the annulus, sort of like tightening a belt around your waist. Sometimes this is done with suture alone. Sometimes this is done with artificial rings or bands.

As mentioned earlier, arrhythmia commonly may coexist. And if arrhythmias are present, then an arrhythmia procedure, often referred to as the Maze procedure, should also be added at the time of valve repair.

Now there can be some curveballs with all of these problems, particularly when patients are referred late for surgery. And there are other strategies that should be in the armamentarium of the surgeon when ventricular function is below normal. Some of these may involve re-routing the blood. Re-routing the plumbing, so to speak, of blood going back to the heart, so that the ventricle that a struggling has less of a workload to deal with. The most common re-routing procedure would be the bi-directional Glenn shunt, where blood going back to the heart is diverted directly to the lungs in an effort to relieve the burden of the ventricle that is struggling.

And then, of course, there's medical therapy. Medical therapy in the perioperative period, but also, importantly, medical therapy in the long-term that would be driven and navigated by the cardiologist.

I can't emphasize the importance of a team approach, a multi-disciplinary approach, that is usually run by the surgeon, cardiologist, and anesthesiologist, but a wide variety of other allied health care professionals, individuals in radiology and imaging that all play a part in the care of these children.

The risk of surgery is largely determined by ventricular function and, in general terms, it is low when ventricular function is normal. An important piece of information for patients and families to know is that, when you are fixing a leaky valve the immediate response to ventricular function is actually to go down. That is to say, ventricular function gets worse initially, and then generally gets better with time. Hopefully, it returns to normal. But this is in a large part due to what the function of the ventricle is before surgery. Again, emphasizing the importance of proper timing of operation. Late results, that is survival, are also largely determined by ventricular function. Common questions are, how long will I live? Or how long will my child live? And will there be a need for other operations?

Generally speaking, survival is optimized when valves can be repaired as opposed to being replaced. But also, there is going to be a high probability for subsequent repair procedures down the road, depending upon how many have been done previous, and what the nature of the specific problem is. So in general, valve repair is preferred. Late survival is beneficial. There's low incidence of infection. And everything is optimized when ventricular function is preserved. Valve replacement may eventually be necessary. And there should be reasonable and sensible attempts at repair in the beginning and on repeated occasions before resorting to replacement.

We, of course, in our practice, have extensive experience with repair of all valves, mitral, tricuspid, and aortic valves. We emphasize the team approach, surgeon experience, and the importance of lifelong oversight cannot be overemphasized by the cardiologist who knows what to look for, knows how to monitor it, and knows when to refer for surgery. If anyone is interested in trying to obtain a consultation with either a surgeon or a cardiologist with your child who has been told that they have a valve problem that needs intervention, please feel free to let us know. We would be happy to review any information and provide recommendations accordingly. Thanks for listening.