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Physicians Update

Winter 2008: Heart

Future Bright for Children with Congenital Heart Disease

Survival rates for pediatric patients with congenital heart disease have improved dramatically in recent years, to the point that for most congenital abnormalities 90-95 percent of patients survive into adulthood—and for many the survival rate is 99 percent or higher. These improvements are the result of a number of advances in the way congenital disease is diagnosed and treated, says Kevin Shannon, M.D., UCLA pediatric cardiologist. Better imaging technology is enabling pediatric cardiologists to detect potential problems and treat them before they become serious concerns. Surgical and postoperative treatment has progressed to the point that two- and three-day hospital stays after open-heart surgery are common. With these dramatic strides in long-term prognosis, Dr. Shannon notes, the focus is shifting to quality of life.

“Many of these children have psychological or social issues associated with the fact that they have scars, they have to take a lot of medications, they frequently miss school because of doctor’s appointments and they can’t always keep up with their peers,” he says.

The issue of multiple appointments is being addressed through the UCLA Medical Home Project, part of a national initiative aiming to improve the coordination of pediatric care so that patients with certain diagnoses requiring chronic care can have multiple needs attended to on each visit, minimizing the frequency of their appointments.

In terms of the actual treatment, Dr. Shannon notes, patients’ quality of life has benefited from a shift toward minimally invasive surgical approaches that reduce the amount of scarring. These include transcatheter techniques for certain procedures, such as closure of atrial septal defects. Pediatric cardiologists are also seeking to minimize pharmacologic interventions by using drugs that are combination therapies and those that have longer half-lives, so that they need to be taken only once per day, rather than two or three times.

“We are now more aggressive about fixing things like leaky valves or small residual shunts,” Dr. Shannon adds, “because we know that even though they may seem to be a minor hemodynamic burden, over 20 to 30 years it will make a difference.” For example, he notes, removing the pulmonary valve as part of repairing tetralogy of Fallot— a congenital anomaly typically characterized by four defects within the heart structures—was found to cause many patients to progress to severe right-ventricular dysfunction as adults, impairing their quality of life. “Now we are much more likely to put in an artificial valve so that we preserve patients’ heart function over the long term,” Dr. Shannon says.

This paradigm has been made possible by the ability to use MRI to image and quantify heart function, particularly of the right ventricle. “We can now see the deleterious effects of not having that valve at a younger age and intervene early to preserve heart function rather than later, when we are just trying to stop the deterioration,” Dr. Shannon explains.

Indeed, thanks to advances in MRI technology, specialists have a minimally invasive technique for obtaining pictures of heart lesions that are equal in quality to those obtained with the more invasive catheterization procedure. This has changed the way many congenital heart disease cases are followed by providing a simpler way to detect changes in the anatomy and function of the heart and its vessels.

“Cardiac MRI and cardiac CT have become extremely valuable in diagnosing and monitoring patients with congenital heart disease,” says Stefan Ruehm, M.D., UCLA radiologist. “With advances in these techniques, we are able to generate high-resolution images that can clearly display the type and severity of the congenital heart defect.”

Recommended Reading

Fenchel M, Saleh R, Dinh H, Lee MH, Nael K, Krishnam M, Ruehm SG, Miller S, Child J, Finn JP. Juvenile and adult congenital heart disease: time-resolved 3D contrast-enhanced MR angiography. Radiology. 2007 Aug; 244(2):399-410.

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