Fetal/Neonatal Pericardial Effusion in Down's Syndrome: Case Report and Review of Literature

Abstract

We report a preterm (35 4/7 weeks) male neonate with Down's syndrome (DS) diagnosed with isolated pericardial effusion (PE) at 20 weeks of gestation. He was born by precipitous delivery, needed no resuscitation and presented within first 24 hours of life with respiratory distress, anemia due to feto-maternal bleed, hypotension, hepatomegaly, and coagulopathy. Postnatal echocardiography confirmed a 5 mm rim of PE without tamponade, normal cardiac structure, and function. He was stabilized with ventilation, packed red cell, fresh frozen plasma, inotropes (dopamine, dobutamine, and adrenaline), and steroid (hydrocortisone). Subsequent evaluation confirmed hypothyroidism, transient myeloproliferative disorder (TMD), hepatic failure due to fibrosis/cirrhosis with portal hypertension, and steroid sensitive hypotension on two occasions possibly due to adrenal insufficiency. PE completely resolved over 2 weeks. In view of progressively worsening liver failure with ascites and portal hypertension, the family opted for palliation. Literature review has been discussed regarding perinatal onset of PE in DS.

Pericardial effusion after congenital heart surgery

“… 18 Down syndrome is associated with a high incidence of PE, which may occur without cardiac surgery and is frequently associated with hypothyroidism, which is also related to PE. 19 , 20 In our study, trisomy 21 (Down syndrome) and 22q.11 deletion (DiGeorge syndrome) were identified as independent risk factors of postoperative PE on multivariable analysis. This finding was partially corroborated by Elias and colleagues, 2 who demonstrated that trisomy 21, but not 22q.11 deletion, was a risk factor of PE after pediatric cardiac surgery.…”

Tamponade and massive pleural effusions secondary to peripherally inserted central catheter in neonates–A complication to be aware of

“…On the other hand, the clinical presentation and the patient's hemodynamics substantially hang on the velocity of fluid accumulation and influence the decision to intervene. When the pericardial effusion accumulates rapidly, or presents in a tamponade, pending tamponade, or is causing significant respiratory or hemodynamic instability, pericardiocentesis is usually performed (18,19).…”

Atypical eosinophils as a biomarker of idiopathic pericardial effusion associated with transient abnormal myelopoiesis in Down syndrome

“…Transient abnormal myelopoiesis (TAM) is a unique neonatal hematological disorder caused by the molecular interaction of trisomy 21 (Down syndrome [DS]) and GATA1 mutation. 1,2 Clinical evidence has shown that pericardial/pleural effusion is one of the most common complications associated with hyperleukocytosis and concurrent organ failure, 3,4 but some develop isolated pericardial effusion in the absence of underlying conditions such as cardiac, metabolic, infectious diseases, and leukemic infiltration. [5][6][7][8][9][10][11][12][13] Herein, we report a female neonate with DS who developed late-onset pericardial effusion associated with clonal hypereosinophilia, and discuss the clinical significance of serial evaluation of peripheral blood images, with a focus on the inflammatory clonal eosinophils for predicting nonhematological complications, such as idiopathic pericardial effusion.…”