A new prospective study shows that, most infants with congenital diaphragmatic hernia (CDH) do not require follow-up for pulmonary hypertension (PH),

“Long-term follow-up in CDH patients is essential but not all infants will need structural echocardiographic assessments after discharge,” Dr. Hanneke Ijsselstijn of Erasmus MC-Sophia Children’s Hospital in Rotterdam, the Netherlands, reported. “Patients at risk for developing pulmonary hypertension or with persistent pulmonary hypertension at discharge should nevertheless be followed in a standardized way.”

Persistent PH is a leading cause of mortality and morbidity in children with CDH, but the course of the illness is poorly understood, Dr. Ijsselstijn et al. note in Pediatric Critical Care Medicine, online. To investigate, they followed 52 CDH patients admitted to their hospital in 2010-2014.

Forty-nine underwent an echocardiogram at hospital admission, at a median of one day old, and 37 (71%) had PH. At six and 12 months’ follow-up, four infants had a persistent PH. These patients had spent a long time on mechanical ventilation and had a longer hospital stay. They were also more likely to be on nitric oxide, sildenafil and bosentan, likely due to the severity of their illness, Dr. Ijsselstijn said.

“In the Netherlands, all children with CDH who are discharged home will attend structured longitudinal follow-up with assessments of growth, neurodevelopment, pulmonary condition, exercise testing until the transition of care in adolescence and adulthood,” Dr. Ijsselstijn said. “At present, we are working at a European level to develop a methodology for standardization of follow-up in CDH patients.”

At her center, she added, all CDH patients who have an elevation of pulmonary arterial pressure at discharge on echocardiography, and those on medication, receive echo follow-up.

“Within the CDH EURO Consortium we aim to start the CoDiNOS trial (NTR6982) this year and we expect to gather additional information on the early course of PH in CDH with echocardiography in a large number of patients,” Dr. Ijsselstijn said.