Characteristics of Infants With Congenital Diaphragmatic Hernia Who Need Follow-Up of Pulmonary Hypertension
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Published on: July 18, 2014
Ulrike S Kraemer1,2, Lisette Leeuwen1, Thomas B Krasemann2
1Intensive Care and Department of Pediatric Surgery, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
Insights
Pulmonary hypertension (PH) persists in less than 10% of congenital diaphragmatic hernia (CDH) patients within the first year of life. Follow-up PH monitoring is recommended for CDH patients with discharge echocardiographic signs or medication treatment.
Area of Science:
- Pediatrics
- Cardiology
- Neonatology
Background:
- Pulmonary hypertension (PH) is a significant cause of mortality and morbidity in infants with congenital diaphragmatic hernia (CDH).
- The persistence or recurrence of PH during the first year of life in CDH patients remains unclear.
Purpose of the Study:
- To investigate the persistence and recurrence of pulmonary hypertension in infants with congenital diaphragmatic hernia during the first year of life.
- To identify characteristics associated with persistent PH in CDH patients.
Main Methods:
- A prospective longitudinal follow-up study was conducted.
- Fifty-two CDH patients were monitored.
- Pulmonary hypertension was assessed using echocardiography and electrocardiography at 6 and 12 months of age.
Main Results:
- Less than 10% of CDH patients exhibited persistent PH at 6 and/or 12 months.
- Patients with persistent PH required longer mechanical ventilation and hospital stays.
- Persistent PH patients received more inhaled nitric oxide, sildenafil, and bosentan treatments.
Conclusions:
- Persistent PH is uncommon in CDH patients within the first year of life.
- Follow-up PH assessment should be targeted towards CDH patients with echocardiographic findings or medication use for PH at hospital discharge.
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.
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