Neonatal pulmonary artery thrombosis

Abstract

Pulmonary artery thrombosis in neonates is a rare entity. We describe two neonates with this diagnosis; their presentation, evaluation, and management. These cases highlight the importance of this differential diagnosis when evaluating the cyanotic neonate.

Respiratory failure secondary to congenital pulmonary arterial thrombus with lung dysplasia

“…Although alike in initial presentation, in the case of PAT, obtaining an echocardiography will quickly reveal a structurally normal heart. There are a few reported cases similar to our patient, where PAT initially presented as PHTN, with increased RV pressures secondary to significant resistance through the occluded PAs 4 5. Like our patient, these cases were refractory to multiple therapies which would usually treat the pathophysiology of PHTN.…”

“…In our patient, based on presentation, it is suspected the thrombus existed in utero, potentially causing secondary anatomic changes in the development of the cystic lung. Three prior cases of PAT with associated lung hypoplasia or dysplasia have been reported in the recent literature 1 4 5. In all three cases, the infants were identified to have significantly occlusive thrombi within the LPA in conjunction with lung hypoplasia or cavities.…”

“…However, considering embryologic lung development is supplied by the bronchial arteries originating from the thoracic aorta, a PAT does not entirely explain a pulmonary infarction and subsequent dysplasia. Existing theories regarding this association include (1) embryonic vascular injury causing both thrombosis and lung hypoplasia and (2) abnormal development of the organ and vasculature, with subsequent thrombosis 4…”

Pulmonary Artery Thrombus Presenting as Cyanotic Cardiac Defect in a Neonate

“…There are few case reports about neonatal thrombosis including pulmonary artery(4) and also of aorta (5) .With large pulmonary artery thrombus, presentation is with respiratory failure and cyanosis while with thrombus in aorta is with coarctation of aorta (4,5).The current data on modes of presentation in the neonates with pulmonary artery thrombosis are very limited.…”

“…Other risk factors are sepsis, polycythemia, maternal diabetes, asphyxia, and inherited thrombophilias (1)(2)(3). Neonate presenting with isolated pulmonary artery thrombosis with severe pulmonary arterial hypertension is a rare event (4). We are reporting such a term neonate presenting with congestive heart failure (CHF) and cyanosis and diagnosed as thrombus involving main and left pulmonary artery.…”