Background

Pulmonary Hypertension (PH) occurs in 30-45% of infants with moderate to severe Broncho-pulmonary Dysplasia (BPD) Farrow and Steinhorn 2014). Sildenafil is a potent inhibitor of 5phosphodiesterase acting on cGMP (cyclic guanosine monophosphate), leading to vasodilation of pulmonary vessels, therefore, hypothetically effective in infants with BPD-PH.

Objectives

A clinical question: In preterm infant (Patient) with BPD-PH could sildenafil (Intervention) as compared to no intervention (Control) help reduce length of invasive ventilation (Outcome)?

Methods

Literature search using PICO format yielded studies which were reviewed.

Results

Three trials in Cochrane systematic review (Shah PS and Ohlsson A 2011) showed improved oxygenation index with decreased mortality in term/near term infants, however long term effects weren't explored.

Backes et al (2016) in retrospective study reported inter-hospital variation, with sildenafil reserved for sicker infants (< 29 weeks) with severe disease. There were inconsistencies in diagnosis of PH (echocardiography/ cardiac catheterisation), variation in doses, duration and starting age of treatment.

Wardle et al (2015) in retrospective study demonstrated that in 14 preterm infants (24-28 weeks), systolic PAP (Pulmonary Artery Pressure) was reduced as compared to 5 infants without its use. The study was however small with selection bias.

Conclusion

Use of sildenafil has been investigated in small low powered data in this age group. It is unlicensed in infants < 1 year of age with warning against its use in higher doses. There is no consensus in the dose, duration nor weaning strategy. The evidence found for its use in preterm infant with BPD-PH is limited and further trials should be awaited.

Background

Pulmonary hypoplasia is common in the perinatal period and a significant cause of death in newborn infants. Definitive diagnosis is by histopathology. Clinical diagnosis and management is often challenging. There are no studies describing outcomes of the neonates with clinically suspected pulmonary hypoplasia.

Objectives

Outcomes of preterm and term infants with clinical diagnosis of pulmonary hypoplasia

Methods

Retrospective observational study over a period of 10 years from January 2009 to December 2018. Data was collected from Badger database (Neonatal patient record database) on gestational age, birth weight, ventilator support, use of nitric oxide, CLD ( Chronic Lung Disease), mortality and two year neurodevelopmental outcome

Results

Total number of infants identified with clinical diagnosis of pulmonary hypoplasia: 27; with mortality of 19/26 (73%), survival of 7/26 (26%) 1 outcome unknown. The proportion of infants with associated anomalies and survival: anhydramnios/oligohydramnios due to PROM (Prolonged Rupture of Membranes) (11/5), skeletal dysplasia (2/0), renal anomalies (7/1), multiple congenital anomalies (5/0), postnatal diagnosis of Congenital Diaphragmatic Hernia (1/1). Among the survivors 3/7 had CLD. Two year neurodevelopmental outcome showed, 2 with no or mild delay, 1 with moderate delay and none had severe delay.

Anhydramnios/ Oligohydramnios secondary to PROM: n- 11

Conclusion

In non-surgical tertiary NICU, PROM leading to anhydramnios/oligohydramnios is the most common cause for pulmonary hypoplasia. Mortality remains very high in infants born with pulmonary hypoplasia. Aggressive treatment for pulmonary hypoplasia secondary to PROM and anhydramnios/oligohydramnios is required to improve the outcomes.

Background

Pulmonary Hypertension (PH) occurs in 30-45% of infants with moderate to severe Broncho-pulmonary Dysplasia (BPD) Farrow and Steinhorn 2014). Sildenafil is a potent inhibitor of 5phosphodiesterase acting on cGMP (cyclic guanosine monophosphate), leading to vasodilation of pulmonary vessels, therefore, hypothetically effective in infants with BPD-PH.

Objectives

A clinical question: In preterm infant (Patient) with BPD-PH could sildenafil (Intervention) as compared to no intervention (Control) help reduce length of invasive ventilation (Outcome)?

Methods

Literature search using PICO format yielded studies which were reviewed.

Results

Three trials in Cochrane systematic review (Shah PS and Ohlsson A 2011) showed improved oxygenation index with decreased mortality in term/near term infants, however long term effects weren't explored.

Backes et al (2016) in retrospective study reported inter-hospital variation, with sildenafil reserved for sicker infants (< 29 weeks) with severe disease. There were inconsistencies in diagnosis of PH (echocardiography/ cardiac catheterisation), variation in doses, duration and starting age of treatment.

Wardle et al (2015) in retrospective study demonstrated that in 14 preterm infants (24-28 weeks), systolic PAP (Pulmonary Artery Pressure) was reduced as compared to 5 infants without its use. The study was however small with selection bias.

Conclusion

Use of sildenafil has been investigated in small low powered data in this age group. It is unlicensed in infants < 1 year of age with warning against its use in higher doses. There is no consensus in the dose, duration nor weaning strategy. The evidence found for its use in preterm infant with BPD-PH is limited and further trials should be awaited.

Background

Pulmonary hypoplasia is common in the perinatal period and a significant cause of death in newborn infants. Definitive diagnosis is by histopathology. Clinical diagnosis and management is often challenging. There are no studies describing outcomes of the neonates with clinically suspected pulmonary hypoplasia.

Objectives

Outcomes of preterm and term infants with clinical diagnosis of pulmonary hypoplasia

Methods

Retrospective observational study over a period of 10 years from January 2009 to December 2018. Data was collected from Badger database (Neonatal patient record database) on gestational age, birth weight, ventilator support, use of nitric oxide, CLD ( Chronic Lung Disease), mortality and two year neurodevelopmental outcome

Results

Total number of infants identified with clinical diagnosis of pulmonary hypoplasia: 27; with mortality of 19/26 (73%), survival of 7/26 (26%) 1 outcome unknown. The proportion of infants with associated anomalies and survival: anhydramnios/oligohydramnios due to PROM (Prolonged Rupture of Membranes) (11/5), skeletal dysplasia (2/0), renal anomalies (7/1), multiple congenital anomalies (5/0), postnatal diagnosis of Congenital Diaphragmatic Hernia (1/1). Among the survivors 3/7 had CLD. Two year neurodevelopmental outcome showed, 2 with no or mild delay, 1 with moderate delay and none had severe delay.

Anhydramnios/ Oligohydramnios secondary to PROM: n- 11

Conclusion

In non-surgical tertiary NICU, PROM leading to anhydramnios/oligohydramnios is the most common cause for pulmonary hypoplasia. Mortality remains very high in infants born with pulmonary hypoplasia. Aggressive treatment for pulmonary hypoplasia secondary to PROM and anhydramnios/oligohydramnios is required to improve the outcomes.