Fast-track Evaluation for Adults with Congenital Heart Disease

One-of-a-kind program provides single-day evaluation and consultation with senior ACHD specialists.

Adults who had childhood surgery for Tetralogy of Fallot or pulmonary stenosis should continue to be evaluated to ensure that their hearts are functioning well and to determine whether any further treatment may be needed. A new fast-track program at the Schneeweiss Adult Congenital Heart Center now makes it possible for patients to obtain thorough, sophisticated testing and expert consultation in a single visit.

Why the need for this special program?

Thirty years ago, the first generation of children to undergo successful repairs of their heart defects on a wide scale represented a new era in cardiac care. For the first time, surgery provided new lifesaving options that allowed many to live relatively normal lives and progress to adulthood and beyond.

But it turns out that many of these young adults, once considered fully cured, have developed previously unforeseen problems as a result of those early repairs. Marlon S. Rosenbaum, MDDirector of the Schneeweiss Adult Congenital Heart Center, explains, "Surgeons used to think that after early surgery to repair their defects, children with Tetralogy of Fallot or pulmonary stenosis would be fine thereafter. During the last decade, however, we have learned that many patients do have consequences of early surgery that are important to address."

Childhood surgery for these conditions typically involved enlarging a narrowed pulmonary valve, which resulted in a leakage of blood back into the right ventricle. Over decades, such leaks can cause progressive enlargement of the ventricle, interfering with its function. The treatment is to replace the pulmonary valve before there is irreparable damage to the right ventricle.

"A large segment of patients with Tetralogy of Fallot, and increasing numbers of those with pulmonary stenosis, are now requiring pulmonary valve replacement," says Dr. Rosenbaum. Left untreated, this problem can lead to irreversible failure of the right ventricle, an additional leak in the tricuspid valve, arrhythmias, heart failure, and fluid retention. If the heart becomes too enlarged and cannot recover, heart transplantation may be the only option.

Proper evaluation and timely intervention with valve replacement is highly effective in reducing the size of the right ventricle and generally leads to a 30% reduction in the size of this chamber. "Although we continue to refine the timing for pulmonary valve replacement, we do know that too many patients are still being referred with excessively large right ventricles that should have been addressed earlier with pulmonary valve replacement," says Dr. Rosenbaum.

Pulmonary valve stenosis is a heart valve disorder in which outflow of blood from the right ventricle of the heart is obstructed at the level of the pulmonic valve. The most common cause of pulmonary valve stenosis is congenital heart disease.The problem is two-fold. Across much of the country, patients with Tetralogy of Fallot or other congenital heart diseases often follow up with cardiologists who may be unfamiliar with unique sequelae that occur after childhood heart surgery, explains Dr. Rosenbaum. Patients and their cardiologists alike frequently do not recognize symptoms that could indicate a problem because patients have lived with their conditions their entire lives. Difficulty with stairs, or being unable to run, for instance, just seem normal. Simply put, most cardiologists are not comfortable advising their adult congenital heart patients regarding the need to replace the pulmonary valve.

Moreover, even if cardiologists recognize the need for further evaluation, the process of determining whether a patient may need pulmonary valve replacement typically requires multiple visits for a consultation, congenital echocardiography, cardiac MRI, cardiopulmonary stress test, and sometimes a cardiac catheterization. This multi-stage process is cumbersome, and for people living far from an adult congenital heart center that offers this expertise, too costly or time consuming to arrange.

But as Dr. Rosenbaum explains, "It is extremely important to know if your right ventricle is excessively large and not pumping well, or if other potentially dangerous problems are brewing."

The Fast-Track Evaluation Program

In order to make it possible for patients to obtain evaluation in an expedited fashion, and to provide the experienced decision-making necessary for optimal care, the Schneeweiss Adult Congenital Heart Center at Columbia has established an innovative, first of its kind, fast-track evaluation program. When a patient with Tetralogy of Fallot or pulmonary stenosis calls to schedule an evaluation, this sets in motion a discussion with our nurse practitioner, validation of insurance, and a request for pertinent test results. Then, during a single visit scheduled within two to three weeks of the initial call, the patient comes to the center for the three major tests followed by consultation with a senior adult congenital heart specialist to review the test results and discuss treatment needs. "Although this process involves a lot of coordination on our end to get these studies performed and interpreted in one day, it will be a great advantage for the patient who seeks expert advice during a day trip to New York City. At the end of the day, we are able to tell the patient what he or she needs to know," says Dr. Rosenbaum.

In addition to evaluating the need for pulmonary valve replacement, the testing will identify related problems such as a leak in the tricuspid valve, narrowing of a pulmonary artery, or history of arrhythmias. Up to 20% of patients who underwent repair for Tetralogy of Fallot now have atrial arrhythmias, according to Dr. Rosenbaum. Treatment options range from medication, catheter ablation or arrhythmia surgery during valve replacement.

"It is an exciting time to practice adult congenital heart disease," says Dr. Rosenbaum."Advances in catheter-based technology such as percutaneous pulmonary valve replacement and a better understanding of late outcomes are changing the way ACHD is practiced at the major programs. Fast-track is our attempt to provide ACHD patients easy access to cutting edge technology and senior-level expertise."

The Schneeweiss Adult Congenital Heart Center includes specialists in echocardiography, MRI, electrophysiology, and exercise testing. To learn more or to request an appointment, please visit: or call: 212.305.6936.