title: Echocardiographic Parameters and Outcomes in Primary Fetal Cardiomyopathy.
abstract: OBJECTIVE
  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.
authors.affiliation: Section of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas USA.
authors.forename: David S
authors.initials: DS
authors.lastname: Ezon
authors.affiliation: Section of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas USA.
authors.forename: Nancy A
authors.initials: NA
authors.lastname: Ayres
authors.affiliation: Section of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas USA.
authors.forename: Carolyn A
authors.initials: CA
authors.lastname: Altman
authors.affiliation: Section of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas USA.
authors.forename: Susan W
authors.initials: SW
authors.lastname: Denfield
authors.affiliation: Section of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas USA.
authors.forename: Shaine A
authors.initials: SA
authors.lastname: Morris
authors.affiliation: Section of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas USA.
authors.forename: Shiraz A
authors.initials: SA
authors.lastname: Maskatia
mesh_terms: D000293:Adolescent; D000328:Adult; D009202:Cardiomyopathies / Q000000981:diagnostic imaging* / Q000401:mortality*; D004452:Echocardiography; D005260:Female; D005318:Fetal Heart / Q000000981:diagnostic imaging; D006801:Humans; D011247:Pregnancy; D011256:Pregnancy Outcome / Q000453:epidemiology*; D012189:Retrospective Studies; D013781:Texas / Q000453:epidemiology; D016216:Ultrasonography, Prenatal; D055815:Young Adult
keywords: Tei index; cardiomyopathies; cardiovascular profile score; fetal echocardiography; pediatric ultrasound