title: [Bronchopulmonary dysplasia-associated pulmonary arterial hypertension of very preterm infants].
abstract: Bronchopulmonary dysplasia (BPD) of very preterm infants is a multifactorial chronic lung disease and its incidence has not decreased despite improvements in neonatal intensive care, including lung protective strategies. Pulmonary hypertension (PH) can complicate the course of BPD. Mortality in infants with BPD-associated PH is thought to be very high, but its incidence is unknown and a standard diagnostic and therapeutic strategy has not been well defined. In this article, we will first describe the current knowledge on the BPD-associated PH and the current treatments available for this pathology. We will then present the HTP-DBP Study, carried out in Paris (France) starting in 2012. The diagnosis of PH is suspected on echocardiographic criteria, but cardiac catheterization is considered the gold standard for diagnosis and evaluation of the severity of PH. Moreover, pulmonary vasoreactivity testing is used to guide the management of patients with PH. The pathogenesis of BPD-associated PH is poorly understood and even less is known about appropriate therapy. Today, optimizing ventilation and reducing the pulmonary vascular tone with specific pulmonary vasodilatator drugs are the main goals in treating HTP-associated DBP. Animal studies and a few clinical studies suggest that medications targeting the nitric oxide (NO) signaling pathway (NO inhalation, oral sildenafil citrate) could be effective treatments for BPD-associated PH, but they have not been approved for this indication. The HTP-DBP study is a French multicenter prospective observational study. The objective is to evaluate the frequency of BPD-associated PH, to describe its physiopathology, its severity (morbidity and mortality), and the effectiveness of current treatments.
authors.affiliation: Service de maternité, réanimation, soins intensifs et pédiatrie néonatales, hôpital Necker-Enfants-Malades, AP-HP, Paris, France. petitmeau@hotmail.com
authors.forename: V
authors.initials: V
authors.lastname: Meau-Petit
authors.forename: G
authors.initials: G
authors.lastname: Thouvenin
authors.forename: N
authors.initials: N
authors.lastname: Guillemot-Lambert
authors.forename: V
authors.initials: V
authors.lastname: Champion
authors.forename: I
authors.initials: I
authors.lastname: Tillous-Borde
authors.forename: F
authors.initials: F
authors.lastname: Flamein
authors.forename: L
authors.initials: L
authors.lastname: de Saint Blanquat
authors.forename: S
authors.initials: S
authors.lastname: Essouri
authors.forename: J
authors.initials: J
authors.lastname: Guilbert
authors.forename: N
authors.initials: N
authors.lastname: Nathan
authors.forename: I
authors.initials: I
authors.lastname: Guellec
authors.forename: S
authors.initials: S
authors.lastname: Kout
authors.forename: R
authors.initials: R
authors.lastname: Epaud
authors.forename: M
authors.initials: M
authors.lastname: Lévy
mesh_terms: D000280:Administration, Inhalation; D001993:Bronchodilator Agents / Q000008:administration & dosage; D001997:Bronchopulmonary Dysplasia / Q000150:complications / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D006328:Cardiac Catheterization; D065627:Familial Primary Pulmonary Hypertension; D005602:France / Q000453:epidemiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D015994:Incidence; D062071:Infant, Extremely Premature; D007231:Infant, Newborn; D009569:Nitric Oxide / Q000008:administration & dosage; D010879:Piperazines / Q000008:administration & dosage; D011175:Positive-Pressure Respiration; D011446:Prospective Studies; D011687:Purines / Q000008:administration & dosage; D012307:Risk Factors; D012720:Severity of Illness Index; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000008:administration & dosage; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage
chemical_list: D001993:Bronchodilator Agents; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D009569:Nitric Oxide; D000068677:Sildenafil Citrate