sildenafil-citrate and Respiratory-Insufficiency

While diagnoses of hypoxemic respiratory failure (HRF) and pulmonary hypertension (PH) in preterm infants may be based on criteria similar to those in term infants, management approaches often differ. In preterm infants, HRF can be classified as 'early' or 'late' based on an arbitrary threshold of 28 postnatal days. Among preterm infants with late HRF, the pulmonary vascular abnormalities associated with bronchopulmonary dysplasia (BPD) represent a therapeutic challenge for clinicians. Surfactant, inhaled nitric oxide (iNO), sildenafil, prostacyclin and endothelin receptor blockers have been used to manage infants with both early and late HRF. However, evidence is lacking for most therapies currently in use. Chronic oral sildenafil therapy for BPD-associated PH has demonstrated some preliminary efficacy. A favorable response to iNO has been documented in some preterm infants with early PH following premature prolonged rupture of membranes and oligohydramnios. Management is complicated by a lack of clear demarcation between interventions designed to manage respiratory distress syndrome, prevent BPD and treat HRF. Heterogeneity in clinical phenotype, pathobiology and genomic underpinnings of BPD pose challenges for evidence-based management recommendations. Greater insight into the spectrum of disease phenotypes represented by BPD can optimize existing therapies and promote development of new treatments. In addition, better understanding of an individual's phenotype, genotype and biomarkers may suggest targeted personalized interventions. Initiatives such as the Prematurity and Respiratory Outcomes Program provide a framework to address these challenges using genetic, environmental, physiological and clinical data as well as large repositories of patient samples.

Topics: Administration, Inhalation; Biomarkers; Bronchodilator Agents; Bronchopulmonary Dysplasia; Gestational Age; Humans; Infant, Newborn; Infant, Premature; Lung; Nitric Oxide; Persistent Fetal Circulation Syndrome; Pulmonary Surfactants; Randomized Controlled Trials as Topic; Respiration, Artificial; Respiratory Insufficiency; Sildenafil Citrate; Vasodilator Agents

Lymphatic malformations account for approximately 5% of benign tumors encountered during the neonatal period. Most often treated by surgical resection or sclerotherapy, phosphodiesterase inhibitors have recently emerged as a potential treatment modality for lymphatic malformations. Treatment with phosphodiesterase inhibitors may be particularly useful when patients are not candidates for surgical resection or sclerotherapy. We report a case of giant bilateral lymphatic malformations diagnosed prenatally and treated postnatally with sildenafil.

Topics: Adult; Fatal Outcome; Female; Humans; Infant, Newborn; Lymphatic Abnormalities; Male; Phosphodiesterase 5 Inhibitors; Piperazines; Pregnancy; Purines; Respiratory Insufficiency; Sclerotherapy; Sildenafil Citrate; Sulfones; Ultrasonography, Prenatal

Sildenafil, usually a well-tolerated drug traditionally used for erectile dysfunction (ED), was recently approved for pulmonary arterial hypertension. In the literature, there are few cases of hemoptysis following sildenafil use for ED; however, to our knowledge, we are reporting the first case of hemoptysis following sildenafil use for pulmonary hypertension. We are documenting a case of a 90-year-old male patient who was admitted to the intensive care unit with hemoptysis and respiratory failure two weeks after he was started on sildenafil.

Topics: Aged, 80 and over; Anti-Inflammatory Agents, Non-Steroidal; Aspirin; Drug Therapy, Combination; Hemoptysis; Humans; Hypertension, Pulmonary; Intensive Care Units; Male; Patient Admission; Piperazines; Purines; Respiratory Insufficiency; Sildenafil Citrate; Sulfones; Vasodilator Agents

A 23-week-old baby, born at 26(+2) weeks, presented to the hospital with critical respiratory failure, which was impossible to stabilize. She had unstable oxygen saturations between 35% and 95%. A presumptive diagnosis of bronchopulmonary dysplasia with associated pulmonary hypertensive crisis was made. In the absence of inhaled nitric oxide, 2 oral doses of 1 mg/kg sildenafil were given, with a dramatic improvement 30 to 45 minutes later. Her oxygenation index fell from 43 to 14. She made a full recovery and was discharged from the hospital 2 weeks later.

Topics: Administration, Oral; Bronchopulmonary Dysplasia; Dose-Response Relationship, Drug; Drug Administration Schedule; Female; Heart Arrest; Humans; Hypertension, Pulmonary; Infant; Infant, Newborn; Infant, Premature, Diseases; Intensive Care Units, Neonatal; Intubation, Gastrointestinal; Oxygen; Phosphodiesterase 5 Inhibitors; Piperazines; Purines; Respiratory Insufficiency; Sildenafil Citrate; Sulfones; Vasodilator Agents

We describe the case of a woman who presented to the intensive care unit with acute respiratory failure that required mechanical ventilation. She had severe pulmonary hypertension secondary to interstitial lung disease, and her history included sarcoidosis and tuberculosis. She was dependent on inhaled nitric oxide (INO) to maintain safe arterial oxygen saturation and could not be weaned from mechanical ventilation. Echocardiography revealed a patent foramen ovale with substantial right-to-left shunt, which probably contributed to her hypoxemia. Sildenafil enabled weaning from INO and substantially reduced the flow through the patent foramen ovale. She was successfully extubated and discharged home. To our knowledge, this is the first report of weaning from INO and mechanical ventilation in a patient with both severe secondary pulmonary hypertension and a right-to-left shunt through a patent foramen ovale.

Topics: Aged; Bronchodilator Agents; Comorbidity; Dyspnea; Female; Foramen Ovale, Patent; Humans; Hypertension, Pulmonary; Nitric Oxide; Phosphodiesterase 5 Inhibitors; Piperazines; Purines; Respiratory Insufficiency; Sarcoidosis, Pulmonary; Sildenafil Citrate; Sulfones; Vasodilator Agents; Ventilator Weaning

Post-pneumonectomy respiratory failure is a devastating complication of resection for lung cancer. As proven therapy is limited, we successfully employed a novel medication silfenadil that has been effective in the treatment of pulmonary hypertension.

Topics: Carcinoma, Bronchogenic; Female; Humans; Lung Neoplasms; Middle Aged; Nitric Oxide; Nitric Oxide Donors; Piperazines; Pneumonectomy; Purines; Respiratory Distress Syndrome; Respiratory Insufficiency; Sildenafil Citrate; Sulfones; Treatment Outcome

Topics: Adaptation, Physiological; Age Factors; Blood Circulation; Contraindications; Depression; Heart Failure; Humans; Male; Piperazines; Purines; Respiratory Insufficiency; Risk Assessment; Sildenafil Citrate; Sulfones; Vasodilator Agents