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Echocardiographic Parameters and Outcomes in Primary Fetal Cardiomyopathy
Author(s) -
Ezon David S.,
Ayres Nancy A.,
Altman Carolyn A.,
Denfield Susan W.,
Morris Shaine A.,
Maskatia Shiraz A.
Publication year - 2016
Publication title -
journal of ultrasound in medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.574
H-Index - 91
eISSN - 1550-9613
pISSN - 0278-4297
DOI - 10.7863/ultra.15.05059
Subject(s) - medicine , fetal echocardiography , cardiology , gestational age , fetus , cardiomyopathy , interquartile range , heart failure , pregnancy , prenatal diagnosis , genetics , biology
Objectives Primary fetal cardiomyopathy is a rare entity, with a poor prognosis. We sought to describe its echocardiographic characteristics and outcomes. Methods We performed a 12‐year retrospective review of fetuses with primary cardiomyopathy. Results Of more than 6000 fetuses evaluated, 25 met criteria for primary cardiomyopathy, and 18 had sufficient echocardiographic and pregnancy outcome data for inclusion. At echocardiography, the median gestational age was 29.6 weeks (range, 21.0–36.4 weeks); median cardiovascular profile score was 6 (range, 1–9); median right ventricular Tei index was 0.52 (range, 0.32–0.94); and median left ventricular Tei index was 0.40 (range, 0.15–0.88). Two had fetal demise, and 16 survived to delivery. The median cardiovascular profile score in those with fetal demise was 3.0 and in those who survived to delivery was 6.5 (range, 3–9; P = .14). The median right ventricular Tei index in those with fetal demise was 0.39 and in those surviving to delivery was 0.53 (range, 0.38–0.94; P = .49). The median left ventricular Tei index in those with fetal demise was 0.29 and in those surviving to delivery was 0.42 (range, 0.15–0.88; P = .50). Sixty‐day survival was available in 11 of 16 fetuses. In addition to the 2 with fetal demise, 4 had postnatal demise, and 5 were alive at a median follow‐up of 39 months. Hydrops ( P = .01), skin edema ( P = .01), and mild or greater mitral regurgitation ( P = .02) were associated with fetal or postnatal demise, with a trend toward an association between moderate or greater tricuspid regurgitation ( P = .07) and fetal or postnatal demise. Conclusions Hydrops and atrioventricular valvar regurgitation are ominous signs in primary fetal cardiomyopathy. Although other commonly used methods for assessing cardiovascular performance may help in diagnosing primary cardiomyopathy, these data suggest limited predictive value.