Single ventricle defects form a subgroup of critical congenital heart disorders. Before 1968, most affected babies struggled to live past infancy. That is when Francois Marie Fontan, M.D., and Guillermo Kreutzer, M.D., revolutionized treatment for these patients. The palliative Fontan-Kreutzer, or “Fontan” operation effectively separated the circulations in a single-functioning ventricle system, giving these newborns a real chance at long-term survival.

Destiny F. Chau, M.D.

Advances in modern medicine since then have further improved their life expectancy. Today, patients with Fontan circulation are getting married, having babies, enjoying careers – and showing up in the O.R.

“Although rare, most anesthesiologists will encounter single-ventricle physiology patients at some point in their practice. These patients are at much higher risks for perioperative complications and have unique anesthetic, surgical and resuscitative considerations,” said Destiny F. Chau, M.D., Pediatric Cardiac Anesthesiologist at the Children’s Hospital of the King’s Daughters. Dr. Chau will lead Sunday’s session “Emergent Exploratory Laparotomy for a Teenager with Multisystemic Injuries, Gasoline Contact Burns and Only Half of a Heart.”

Newborns with a single ventricle are often managed via three staged surgeries. First, infants undergo the Norwood and then the Bidirectional Glenn procedures. Finally, around 2 to 4 years old, the Fontan surgery completely separates the pulmonary and systemic circulations, allowing the patients to have normal oxygen saturations.

After that, cardiologists monitor and fine-tune the Fontan physiology for as long as possible. Unfortunately, some patients will eventually require a heart transplantation.

“Anesthesiologists in every setting need to be familiar with these patients’ unique perioperative needs,” said Dr. Chau. “They will present to the O.R. for surgeries related to their cardiac condition, as well as routine procedures such as cholecystectomies and EGDs. They will come to the labor and delivery ward, to the chronic pain clinic, to the ED and ICUs.”

During the session, Dr. Chau will describe the case of a trauma victim who has Fontan physiology. For example, in a trauma setting, it’s not always easy to identify single ventricle patients. Some Fontan patients wear a medical alert bracelet. If there’s no bracelet and the patient is unconscious, physicians must rely on family members or medical clues.

“The best indicators are a midline chest scar and other scars in the chest area indicating past thoracic/cardiac surgery,” Dr. Chau said. “The scars and poor hemodynamics will prompt an anesthesiologist to investigate for cardiac problems. This can be accomplished by getting an echocardiographic screening of the heart, which would show abnormal cardiac anatomy and clue their care team into congenital cardiac physiology.”

When assessing a patient with Fontan circulation, it’s important to look for comorbidities, as it is a chronic disease. Years of surviving with a reconfigured heart takes a toll on every organ system.

“Anesthesiologists must also understand that some routine resuscitative maneuvers used for patients with normal cardiac anatomy and physiology may not be helpful and may actually be detrimental for these patients,” Dr. Chau said.

While it’s ideal for patients with single ventricle physiology to be cared for in centers with advanced cardiac expertise and resources, emergency and geographic factors do not always make that possible. If local resources are not suited for the entire scope of care, plans for proper disposition should be made after temporizing their acute needs. Dr. Chau hopes to enlighten more anesthesiologists about this special patient category.

“I love the strong spirit and resilience of these patients and their families,” she said. “I am happy to raise awareness regarding their unique health needs.”