Characteristics of infants with congenital diaphragmatic hernia who need follow-up of pulmonary hypertension

Ulrike S. Kraemer, Lisette Leeuwen, Thomas B. Krasemann, René M.H. Wijnen, Dick Tibboel, Hanneke IJsselstijn*

*Bijbehorende auteur voor dit werk

OnderzoeksoutputAcademicpeer review

15 Citaten (Scopus)


Objectives: Pulmonary hypertension is one of the main causes of mortality and morbidity in patients with congenital diaphragmatic hernia. Currently, it is unknown whether pulmonary hypertension persists or recurs during the first year of life.

Design: Prospective longitudinal follow-up study.

Setting: Tertiary university hospital.

Patients: Fifty-two congenital diaphragmatic hernia patients admitted between 2010 and 2014.

Interventions: None.

Measurements and Main Results: Pulmonary hypertension was measured using echocardiography and electrocardiography at 6 and 12 months old. Characteristics of patients with persistent pulmonary hypertension were compared with those of patients without persistent pulmonary hypertension. At follow-up, pulmonary hypertension persisted in four patients: at 6 months old, in three patients (patients A-C), and at 12 months old, in two patients (patients C and D). Patients with persistent pulmonary hypertension had a longer duration of mechanical ventilation (median 77 d [interquartile range, 49-181 d] vs median 8 d [interquartile range, 5-15 d]; p = 0.002) and hospital stay (median 331 d [interquartile range, 198-407 d) vs median 33 d (interquartile range, 16-59 d]; p = 0.003) than patients without persistent pulmonary hypertension. The proportion of patients with persistent pulmonary hypertension (n = 4) treated with inhaled nitric oxide (100% vs 31%; p = 0.01), sildenafil (100% vs 15%; p = 0.001), and bosentan (100% vs 6%; p < 0.001) during initial hospital stay was higher than that of patients without persistent pulmonary hypertension (n = 48). At 6 months, all patients with persistent pulmonary hypertension were tube-fed and treated with supplemental oxygen and sildenafil.

Conclusions: Less than 10% of congenital diaphragmatic hernia patients had persistent pulmonary hypertension at ages 6 and/ or 12 months. Follow-up for pulmonary hypertension should be reserved for congenital diaphragmatic hernia patients with echocardiographic signs of persistent pulmonary hypertension at hospital discharge and/or those treated with medication for pulmonary hypertension at hospital discharge.

Originele taal-2English
Pagina's (van-tot)e219-e226
Aantal pagina's8
TijdschriftPediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies
Nummer van het tijdschrift5
StatusPublished - mei-2018
Extern gepubliceerdJa

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