Nonbacterial Thrombotic Endocarditis Coupled With Fatal Persistent Pulmonary Hypertension of Newborn

Aswathy Rahul, Radhika Sujatha, Sobhakumar Saraswathyamma

Abstract

Nonbacterial thrombotic endocarditis (NBTE) in neonates is a rare phenomenon, which has its origin in intrauterine life. Usually, this condition is an incidental finding while the neonate is getting evaluated for respiratory distress. Such neonates present with clinical and echocardiographic evidence of persistent pulmonary hypertension of newborn (PPHN). Most of the neonates have associated thrombocytopenia without any other features of sepsis. Association of hypoxia, ventricular dyskinesia due to asphyxia, and disturbed hemodynamics with the activation of vasoactive and platelet proaggregatory substances may serve as a pathogenic mechanism for the occurrence of PPHN, thrombocytopenia, and NBTE together. NBTE has a grave prognosis due to thromboembolism, especially to pulmonary circulation. Treating NBTE using early thrombolysis with recombinant tissue plasminogen activator (rt-PA) has been suggested in neonates, but there are limited case reports in this regard. As this condition is extremely rare, there is lack of experience regarding the treatment as well. The rt-PA is a very costly drug, which precludes its use in low-resource settings.

In this case series, we discuss 3 cases of PPHN associated with vegetations in the heart valves. All the 3 cases had a fast and grave outcome. We could not start them on rt-PA due to logistic reasons.