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AJP Rep 2012; 02(01): 051-054
DOI: 10.1055/s-0032-1316464
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Absent Pulmonary Valve Syndrome with Left Hemitruncus

Ashraf M. Aly
1   Division of Pediatric Cardiology, Department of Pediatrics, University of Texas Medical Branch-Galveston, Galveston, Texas
,
Deborah A. Reid
2   Division of Maternal Fetal Medicine, Department of Obstetrics and Gynecology, University of Texas Medical Branch-Galveston, Galveston, Texas
,
Amyn K. Jiwani
1   Division of Pediatric Cardiology, Department of Pediatrics, University of Texas Medical Branch-Galveston, Galveston, Texas
,
Pooja H. Desai
1   Division of Pediatric Cardiology, Department of Pediatrics, University of Texas Medical Branch-Galveston, Galveston, Texas
› Author Affiliations
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Publication History

04 January 2012

03 March 2012

Publication Date:
16 July 2012 (online)

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Abstract

Absent pulmonary valve syndrome (APVS) is a rare congenital heart defect, usually associated with tetralogy of Fallot (TOF), although other associations have been described. A pregnant woman was referred to fetal echocardiography clinic from the Maternal Fetal Medicine department due to abnormal findings on routine antenatal ultrasound, showing a pulsatile cystic mass above the left atrium and a suspected TOF. A fetal echocardiogram confirmed the presence of TOF/APVS. The pulsatile cystic mass was the aneurysmally dilated main pulmonary artery. The exact origin of the left pulmonary artery (LPA) was not clearly established prenatally. A postnatal echocardiogram of the neonate showed an abnormal origin of the LPA from the ascending aorta (hemitruncus). The neonate subsequently underwent surgical repair with a good outcome. We present a novel case of a TOF/APVS associated with an abnormal origin of the LPA from the ascending aorta.

Keywords

tetralogy of Fallot - absent pulmonary valve - left hemitruncus - fetal echocardiography
 

  • References

  • 1 Galindo A, Gutiérrez-Larraya F, Martínez JM , et al. Prenatal diagnosis and outcome for fetuses with congenital absence of the pulmonary valve. Ultrasound Obstet Gynecol 2006; 28: 32-39
    CrossrefPubMedSearch in Google Scholar
  • 2 Bharati AH, Naware A, Merchant SA. Absent pulmonary valve syndrome with tetralogy of Fallot and associated dextrocardia detected at an early gestational age of 26 weeks. Indian J Radiol Imaging 2008; 18: 352-354
    Thieme ConnectPubMedSearch in Google Scholar
  • 3 Kikuchi S, Yokozawa M. Absent pulmonary valve associated with tetralogy of Fallot and complete atrioventricular septal defect: report of a case. Ann Thorac Cardiovasc Surg 2005; 11: 44-47
    PubMedSearch in Google Scholar
  • 4 Yeager SB, Van Der Velde ME, Waters BL, Sanders SP. Prenatal role of the ductus arteriosus in absent pulmonary valve syndrome. Echocardiography 2002; 19: 489-493
    CrossrefPubMedSearch in Google Scholar
  • 5 Becker R, Schmitz L, Guschmann M, Wegner RD, Stiemer B, Entezami M. Prenatal diagnosis of familial absent pulmonary valve syndrome: case report and review of the literature. Ultrasound Obstet Gynecol 2001; 17: 263-267
    CrossrefPubMedSearch in Google Scholar