Single Ventricle Conditions

The term single ventricle refers to a group of various congenital heart defects in which one of the ventricles does not form properly during fetal development, causing it to become very small, or hypoplastic. The most common defect in babies born with single ventricles is known as Hypoplastic Left Heart Syndrome (HLHS).

Most babies with HLHS will require surgery in the first few days of life as well as around 4-6 months and 2-4 years of age. These surgeries are necessary and life-saving; however even after surgery these babies continue to be very fragile, especially between the first and second operations. This time between the first and second operations is known specifically as the "interstage period."

In addition to performing the life-saving surgeries necessary to help these babies survive, the University of Maryland Children's Heart Program also has a special clinic for babies during the interstage period known as the Single Ventricle Clinic. Just as important as the life-saving surgeries, the post-operative care a baby receives has a major impact on their survival outcomes.

The Single Ventricle Clinic is comprised of a dedicated, multidisciplinary group of specialists including cardiologists, advanced practice nurses, nutritionists, and social workers, staff this clinic to help achieve the best possible outcomes for these babies. Interstage babies are seen in the Single Ventricle Clinic on a weekly basis to ensure that their ongoing cardiac problems are addressed, that they are feeding and gaining weight appropriately, developing properly, and that the families have the support that they need during this tenuous time. The Children's Heart team also works closely with community pediatricians who care for these babies so they can help them receive the necessary developmental care and preventative medicine to grown and thrive.

In addition to the Single Ventricle Clinic, babies in the interstage period are enrolled in the home monitoring program. In this program, families are sent home with a scale and a pulse ox monitor so that they can measure their baby's weight and oxygen saturation on a daily basis. Families maintain weekly communication with advanced practice nurses to report these measurements. Together with the families, the Children's Heart is able to detect subtle changes in weight or oxygen saturation, which may indicate a bigger problem, allowing physicians to intervene quickly if necessary. Additionally, families are sent home with comprehensive health binders and "Red Flag" checklists so they are fully prepared to care for their baby home and have their child's health information at their fingertips.

The University of Maryland Children's Heart Program is part of a bigger nationwide collaborative known as the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC) whose goal is to improve survival during the fragile interstage period. Together with many hospitals across the country, the Children's Heart Program is examining the best practices and creating standardized protocols to care for these babies in hopes of decreasing mortality rates and achieving the best possible outcomes for all babies.

To learn more about the National Pediatric Cardiology Quality Improvement Collaborative, visit the Parents' website:

To make an appointment with the Children's Heart Program, please call 410-328-4FIT (4348) or 1-800-373-4111 (referring physicians only).

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