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1/10. epoprostenol and home mechanical ventilation for pulmonary hypertension associated with chronic lung disease.

    Pulmonary hypertension (PH) can be associated with bronchopulmonary dysplasia (BPD) of infancy, and mortality in these pediatric patients is high without aggressive medical treatment. Continuous intravenous epoprostenol (prostacyclin) was shown to lower pulmonary artery pressures (PAP) in children with idiopathic pulmonary arterial hypertension (PAH), formerly referred to as primary pulmonary hypertension. We report on the first case of long-term home ventilation in combination with chronic intravenous epoprostenol in a child with severe pulmonary hypertension associated with chronic lung disease. This aggressive combination resulted in significant improvement in pulmonary artery pressures, substantial improvement in quality of life, and eventual discontinuation of home ventilation.
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2/10. Oral sildenafil for treatment of severe pulmonary hypertension in an infant.

    We report the use of oral sildenafil in a 5-month-old preterm infant with severe bronchopulmonary dysplasia and pulmonary arterial hypertension refractory to inhaled nitric oxide treatment, maximal ventilatory support and conventional vasodilator therapy. Sildenafil was prepared as a liquid suspension by the method of trituration and administered via an orogastric tube to the patient. Forty-eight hours after sildenafil treatment, echocardiography revealed that the tricuspid incompetence was substantially diminished and the contractility of both ventricles improved, indicating a marked reduction in pulmonary arterial pressure. Oral sildenafil treatment was continued for 6 months until complete resolution of pulmonary arterial hypertension, and oxygen supplement was weaned off. There was no adverse effect during the treatment period. Oral sildenafil may be useful in reducing pulmonary vascular resistance and can be considered for treatment of severe pulmonary arterial hypertension secondary to bronchopulmonary dysplasia.
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3/10. Effects of supplemental oxygen administration in an infant with pulmonary artery hypertension.

    In patients with pulmonary disease, pulmonary artery hypertension often occurs as a result of pulmonary artery vasoconstriction, primarily from hypoxia and alveolar hypotension. In this report we describe the hemodynamic effects of breathing supplemental oxygen in a child with bronchopulmonary dysplasia and pulmonary artery hypertension. These hemodynamic effects include an improvement in oxygenation, an increase in systemic vascular resistance, and a decrease in the pulmonary vascular resistance. As a direct result of these changes in vascular resistances, alterations of heart rate, cardiac index, stroke volume, aortic pressure, oxygen consumption, and pulmonary artery pressure have been shown to occur. Oxygen is widely used to treat many physiologic conditions. However, during the administration of supplemental oxygen, rarely do we recognize the hemodynamic changes associated with its use. These hemodynamic effects must be clearly understood and appreciated before oxygen administration in any clinical situation.
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4/10. Treatment of pulmonary hypertension with diltiazem in a child with bronchopulmonary dysplasia.

    A two-year-old child dying of pulmonary hypertension and cor pulmonale secondary to bronchopulmonary dysplasia, was demonstrated to have reactive pulmonary hypertension in response to 100% oxygen and isoproterenol infusion. In an attempt to find an oral medication to maintain pulmonary vasodilatation, experimental trials were done using hydralazine, salbutamol, nifedipine and diltiazem. Cardiac index, pulmonary and systemic vascular resistances and intrapulmonary shunts were monitored during the trials. hydralazine, salbutamol and nifedipine were ineffective. diltiazem 2.0 mg given every 6 h resulted in a profound and sustained decrease in pulmonary pressures and resistance, and a reversal of the cor pulmonale.
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5/10. Diagram for easy volume setting of an infant ventilator.

    A pressure-volume diagram with isocompliance lines allows quick, accurate volume setting of the piston-driven infant ventilator Bourns LS. A line parallel with the machine compliance line and an intercept on the ordinate equal to the tidal volume, serves as a guideline for initial volume setting and subsequent adaptation according to the patient's lung compliance. This diagram is particularly useful when the lung compliance is low; changes consistently during controlled ventilation.
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6/10. bronchopulmonary dysplasia in the adult.

    We describe 3 patients with adult respiratory distress syndrome that eventuated in a pathologic picture of honeycomb lung and a radiographic picture of variably cystic lung super-imposed on a background of diffuse alveolar infiltrates. All 3 patients had been treated with unusually high pressures of PEEP as well as high concentrations of oxygen for long periods of time (3 to 7 wk). Microscopically, the cystic structures in our patients appeared to be derived from collapse and fibrosis of the alveolar parenchyma with dilatation of the alveolar ducts. We suggest that this process is morphologically and radiographically similar to bronchopulmonary dysplasia as seen in the newborn.
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7/10. Inhaled nitric oxide for a severe respiratory syncytial virus infection in an infant with bronchopulmonary dysplasia.

    OBJECTIVE: To report the first case of ARDS in children treated with nitric oxide (NO) inhalation. methods: A 13-months infant presented with BPD and severe hypoxemia related to RSV infection and ARDS. Inhaled NO was delivered in the ventilatory circuit of a continuous flow ventilator (Babylog 8000, Drager) in a concentration of 20-80 ppm for 7 days. NO and NO2 were continuously monitored (Polyton Draeger). respiratory mechanics were evaluated by using the method of passive inflation by the ventilator. RESULTS: NO inhalation improved oxygenation (tcSaO2) and reduced respiratory system resistance without affecting arterial pressure. NO2 level remained below 5 ppm, and methaemoglobin level below 1%. The child survived without neurologic sequela. CONCLUSIONS: Two mechanisms to explain oxygenation improvement can be suggested: selective improvement in perfusion of ventilated regions and bronchodilation.
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8/10. bronchopulmonary dysplasia: a case report.

    An unusual case of an extremely low birth weight and very preterm infant who developed bronchopulmonary dysplasia is presented. She required artificial (assisted) ventilation via a manual ambubag using a maximum concentration of 60% O2. Despite previous reports implicating mechanical ventilators and elevated peak airway pressures greater than 35cm of water, our infant still developed the disease with O2 delivery from an ambubag. Outcome was favourable. At 16 1/2 months follow-up, she appeared neurologically normal, and despite her prolonged neonatal respiratory problem, had not been troubled by chest disease or hospital readmissions. The observed etiopathogenesis is worthy of consideration in the 'developing world'.
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9/10. tracheobronchomalacia in preterm infants with chronic lung disease.

    tracheobronchomalacia is a treatable cause of persisting ventilatory requirements in the preterm neonate, and warrants a high index of suspicion. Five preterm infants with persisting ventilatory requirements with evidence of tracheobronchomalacia are reported. Four were diagnosed by tracheobronchogram and one by flexible endoscopy. All were successfully managed by continuous positive airway pressure (CPAP) via a tracheostomy. One infant died of unrelated causes. The oldest child in this series at the age of 2 years requires no further ventilatory support. Tracheobronchial anomalies should be considered in all preterm infants with persisting ventilatory requirements.
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10/10. Effect of beta-blockade on symptomatic dexamethasone-induced hypertrophic obstructive cardiomyopathy in premature infants: three case reports and literature review.

    OBJECTIVE: The objective of this study was to assess the efficacy of beta-blockade on clinically significant left ventricular outflow tract obstruction in premature infants treated with dexamethasone because of bronchopulmonary dysplasia. STUDY DESIGN: case reports are presented of three premature infants (mean gestational age 27 weeks) cared for in the intensive care nursery in whom clinically significant septal hypertrophy and left ventricular outflow tract obstruction developed during dexamethasone treatment for bronchopulmonary dysplasia. The infants were treated with oral propranolol. Serial physiologic and echocardiographic parameters were followed. Physiologic data were analyzed with an analysis of variance, with the Kruskal-Wallis test used for nonparametric data. A p value < 0.05 was considered statistically significant. RESULTS: Oral administration of the beta-blocker propranolol resulted in clinical and echocardiographic improvement of the left ventricular outflow tract obstruction. One patient had a lower average heart rate and two patients had lower average mean blood pressure values during propranolol treatment, none of which was clinically significant. None of the patients had worsening of the respiratory status. CONCLUSION: beta-blockade treatment was well tolerated and may be beneficial in relieving symptomatic steroid-induced left ventricular outflow tract obstruction in premature infants.
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