Glucose/nitric oxide/treprostinil

Abstract

Interstitial pulmonary oedema and lack of efficacy: case report A female neonate [exact age at reaction onset not stated] developed interstitial pulmonary oedema during treatment with nitric oxide. Additionally, she exhibited lack of efficacy during treatment with treprostinil and nitric oxide for pulmonary hypertension, and glucose for hypoglycaemia [not all dosages stated; duration of treatment to reaction onset not stated]. The female neonate was intubated on day 1 of life due to progressive respiratory distress and received unspecified surfactant for presumed respiratory distress syndrome. Echocardiogram findings indicated suprasystemic pulmonary artery pressure; therefore, inhaled nitric oxide therapy was started on day 1 of life for suspected persistent pulmonary hypertension of newborn. However, she remained hypoxic with lower post-ductal than pre-ductal saturations and eventually developed hypoxaemic respiratory failure (lack of efficacy). Postnatal echocardiogram revealed possible aortic arch coarctation. A CT angiography on day 7 of life ruled out coarctation and revealed prominent interstitial pulmonary oedema, which was attributed to nitric oxide. Initially, interstitial lung disease was presumed to be the cause of pulmonary hypertension. The neonate started receiving SC treprostinil 4 ng/kg/min on day 8 of life. The dose of treprostinil was further up-titrated to 30 ng/kg/min due to lack of significant response (lack of efficacy). For ILD, she received empirical treatment with methylprednisolone for 3 days, resulting in significant improvement in pulmonary oedema on day 14 of life. However, she continued to experience pulmonary hypertensive crises with profound hypoxia and bradycardia. She required increased sedation and intermittent paralysis. On day 17 of life, she underwent cardiac catheterisation which showed severe pulmonary arterial hypertension, increased pulmonary vascular resistance and significant vascular reactivity. She also received inotropic support with phenylephrine, epinephrine and vasopressin followed by unspecified systemic steroids. She remained sedated with fentanyl and dexmedetomidine. Additionally, she had repeated episodes of hypoglycaemia, which could not be controlled despite high rates of glucose infusion (lack of efficacy). Therefore, she was treated with octreotide. Hypoglycaemia was considered to be due to hyperinsulinism related to intrauterine growth restriction. Based on the findings of exome with copy number analysis, she was diagnosed with alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV). Considering the lethal outcome of her disease, her family withdrew care on day 26 of life. Eventually, she died of ACD/MPV. Postmortem further confirmed the characteristic histologic pattern of ACD/MPV.