Flail anterior tricuspid valve leaflet in a neonate: association with maternal antiphospholipid syndrome

Abstract

This brief report describes a case of flail anterior tricuspid valve leaflet in a neonate associated with maternal antiphospholipid syndrome. Fetal echocardiography at 27 weeks of gestation showed competent atrioventricular valves with biventricular echogenic chordae. Fetal distress was noted at delivery, and echocardiography showed a flail anterior leaflet of the tricuspid valve with severe regurgitation. Possible causation and implications of maternal antiphospholipid syndrome are discussed.

Endocardial Fibroelastosis as an Independent Predictor of Atrioventricular Valve Rupture in Maternal Autoimmune Antibody Exposed Fetus: A Systematic Review with Clinicopathologic Analysis

“…A total of 12 cases of acute valvar rupture attributable to antenatal exposure to maternal autoantibodies were analyzed ( Table 1 ). This includes Case A from our experience (numbered as Case 1) and 11 patients extracted from 10 reports (numbered Cases 2–10) [ 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 ]. Shiraishi et al reported cases of acute MR in infants, of which two cases were attributable to maternal autoimmunity [ 15 ].…”

“…Furthermore, 42% (5/12) of mothers (Cases 2–4, 8, and 12) had known autoimmune disease [ 9 , 10 , 16 , 17 ], specifically Sjogren’s syndrome, affecting two mothers (Cases 3 and 4) [ 10 , 17 ]. Two mothers had previously affected children (Cases 3 and 7) [ 10 , 16 , 17 ] and one had a sibling that was affected subsequently (Case 10) [ 14 ]. Three mothers had normal pregnancies prior to the affected fetus (Cases 1,2, and 12) [ 9 ].…”

“…Three mothers had normal pregnancies prior to the affected fetus (Cases 1,2, and 12) [ 9 ]. While all mothers were associated with positive Ro titers, only five (42%) had additional positive La antibodies (Cases 6 and 8–10) [ 12 , 13 , 14 , 16 ].…”

“…Of the 12 patients with valve rupture, 4 patients (Cases 1–4) had both MV and TV rupture (33%) [ 9 , 10 , 17 ]. Although six (50%) patients had isolated TV rupture (Cases 7–10) [ 8 , 9 , 11 , 12 , 14 , 16 ], only two (17%) (Cases 5 and 6) had an isolated MV rupture [ 13 , 15 ] ( Figure 10 a). Table 2 describes the antenatal echocardiographic features of these cases as reported.…”

“…Table 2 describes the antenatal echocardiographic features of these cases as reported. The changes in EFE were noted in nine (75%) patients (Cases 2–4, 7–9, and 11) antenatally [ 8 , 9 , 10 , 11 , 12 , 16 , 17 ]. These changes were noted in patients as early as 19 weeks of gestational age (GA) in three cases (Cases 2, 3, and 8) [ 9 , 10 , 16 ] and as late as 24 weeks (Case 4) [ 17 ].…”