Functional state of patients with heterotaxy syndrome following the Fontan operation

Abstract

The study illustrates a profile of characteristics, medical history, functional health state, and markers of ventricular performance in patients with heterotaxy after the Fontan procedure. Despite obvious anatomic differences, and some differences in echocardiography and heart rhythm, there were no important differences in exercise performance or functional health state between these patients and other survivors of the Fontan procedure.

Improved current era outcomes in patients with heterotaxy syndromes☆

“…Atz and associates in a study carried out by the Pediatric Heart Network investigated the functional state of patients with heterotaxy syndrome following the Fontan operation. Although patients with heterotaxy were taking a greater number of medications when compared to nonheterotaxy patients, the majority of patients reported their general health to be excellent and their life to be unaffected by their disease [21].…”

Outcomes after the Ladd procedure in patients with heterotaxy syndrome, congenital heart disease, and intestinal malrotation

“…Also, in studies dating more than 20 years, there was a very high incidence of cardiac-related death among HS patients [3,7]. These 2 facts make it difficult to approximate the true, present-day, risk of midgut volvulus and mortality from an abdominal event.…”

“…These 2 facts make it difficult to approximate the true, present-day, risk of midgut volvulus and mortality from an abdominal event. Continuing advances in cardiovascular care are resulting in more HS patients surviving and reaching a state of cardiac stability with asymptomatic intestinal malrotation [3,4]. As this population grows, it is becoming more important to devise an evidence-based treatment paradigm.…”

“…Furthermore, no agreement exists concerning whether HS patients should be screened for intestinal malrotation. Because of their complex cardiac anatomy and cardiorespiratory physiology, HS patients are at risk for adverse events during the perioperative period [3]. However, with improving perioperative care for children with HS and congenital heart disease, these risks may be changing [3,4].…”

“…Because of their complex cardiac anatomy and cardiorespiratory physiology, HS patients are at risk for adverse events during the perioperative period [3]. However, with improving perioperative care for children with HS and congenital heart disease, these risks may be changing [3,4]. Thus, the debate remains whether patients with HS and asymptomatic intestinal malrotation should undergo an elective Ladd procedure [4].…”