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Prenatal ultrasonic diagnosis of absent pulmonary valve syndrome: A case report.

Abstract Absent pulmonary valve syndrome (APVS) is a rare congenital heart disease that is often associated with tetralogy of Fallot (TOF). Here, we report 2 cases of APVS associated with TOF diagnosed via fetal echocardiography and discuss their specific ultrasonographic characteristics.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title medicine
Publication Year Start




PMID- 28858090
OWN - NLM
STAT- In-Process
DA  - 20170831
LR  - 20170831
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Linking)
VI  - 96
IP  - 35
DP  - 2017 Sep
TI  - Prenatal ultrasonic diagnosis of absent pulmonary valve syndrome: A case report.
PG  - e7747
LID - 10.1097/MD.0000000000007747 [doi]
AB  - RATIONALE: Absent pulmonary valve syndrome (APVS) is a rare congenital heart
      disease that is often associated with tetralogy of Fallot (TOF). Here, we report 
      2 cases of APVS associated with TOF diagnosed via fetal echocardiography and
      discuss their specific ultrasonographic characteristics. PATIENT CONCERNS: Two
      pregnant women with suspicion of fetal heart anomaly were referred from their
      local hospitals to our hospital for fetal malformation screening and detailed
      fetal echocardiography. Color and spectral Doppler flow imaging were utilized to 
      evaluate the axis, size, situs, cardiac chambers, and both inflow and outflow
      tracts of the heart as well as the great arteries. Both cases had a severe
      dilatation of the pulmonary trunk and its branches and an absence or dysplasia of
      the pulmonary valve, which was associated with subaortic ventricular septal
      defect (VSD) with an overriding aorta. In addition, the fetus in case 1 showed a 
      patent ductus arteriosus, and the fetus in case 2 showed arterial duct agenesis. 
      Furthermore, color Doppler flow imaging showed a bi-directional multicolored flow
      signal in the pulmonary valve ring. DIAGNOSES: Both fetuses were diagnosed with
      APVS associated with TOF. INTERVENTIONS: No therapeutic intervention was
      performed. OUTCOMES: On the request of the pregnant women and their families,
      both fetuses were aborted. LESSONS: Although APVS is a rare congenital heart
      disease and often associated with TOF, it has an overall poor prognosis.
      Nowadays, it can be easily diagnosed via ultrasonography because of its typical
      ultrasonographic features, such as aneurysmal dilatation of pulmonary artery,
      massive regurgitation of the pulmonary valve, VSD, and an overriding aorta.
      Therefore, early fetal echocardiography screening should be performed for every
      fetus.
FAU - Zhang, Wen-Jun
AU  - Zhang WJ
AD  - Department of Medical Ultrasound, Taihe Hospital, Hubei University of Medicine,
      Shiyan, Hubei, China.
FAU - Zhang, Zhong-Lei
AU  - Zhang ZL
FAU - Chang, Jun-Jie
AU  - Chang JJ
FAU - Song, Xiao-Yu
AU  - Song XY
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
EDAT- 2017/09/01 06:00
MHDA- 2017/09/01 06:00
CRDT- 2017/09/01 06:00
AID - 10.1097/MD.0000000000007747 [doi]
AID - 00005792-201709010-00014 [pii]
PST - ppublish
SO  - Medicine (Baltimore). 2017 Sep;96(35):e7747. doi: 10.1097/MD.0000000000007747.