Abstract

Case Presentation: A 5 day old, term infant born to a 39-year-old G1P0 with a history of diet-controlled gestational diabetes mellitus presented with hypernatremic dehydration in the setting of poor feeding. On hospital day 2, the infant suffered a bradycardic cardiac arrest requiring 2 minutes of CPR prior to return of spontaneous circulation. An echocardiogram (Figure 1A) revealed moderately depressed right ventricular (RV) function, elevated RV pressure and a large mobile mass extending from the main pulmonary artery (PA) into the left PA with obstruction of flow. D-dimer was markedly elevated at 2740 ng/mL. A subsequent CT angiogram revealed acute pulmonary emboli in the distal left and right branch PAs (Figure 1B). Additional imaging was obtained and showed dural venous sinus, left portal vein and right cephalic vein thromboses. The infant was managed with a heparin infusion and supportive therapy for the RV dysfunction and pulmonary hypertension including mechanical ventilation, inhaled nitric oxide as well as epinephrine and milrinone infusions. Over the course of two weeks, the infant improved clinically with normalization of RV function. Discussion: Neonatal pulmonary arterial thrombosis is an exceedingly rare phenomenon. The majority of cases described in the literature are associated with either an inherited thrombophilia or with underlying congenital heart disease, which were not found to be present in this case. However, this patient presented with severe dehydration and a history of maternal diabetes, both of which are risk factors for neonatal thrombosis. Fortunately, this patient responded to treatment with anticoagulation alone and did not require more invasive therapies, such as surgical thrombectomy or systemic and catheter-directed thrombolysis. Although rare, neonatal pulmonary arterial thrombosis can occur and delays in recognition and treatment may lead to significant RV dysfunction, morbidity, and even death.

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