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Case Reports
. 2013 Mar-Apr;65(2):191-3.
doi: 10.1016/j.ihj.2012.10.010. Epub 2012 Oct 29.

Absent pulmonary valve syndrome with tetralogy of Fallot detected at an early gestational age of 27 weeks - a case report

Ashok N Bhupali  1 , Kiran B Patankar, Sayi Prasad, Jeetendra K Patil, Ajitey Tamhane
Affiliations
Case Reports

Absent pulmonary valve syndrome with tetralogy of Fallot detected at an early gestational age of 27 weeks - a case report

Ashok N Bhupali et al. Indian Heart J. 2013 Mar-Apr.

Abstract

Objective: Absent pulmonary valve syndrome (APVS) is a rare congenital anomaly, usually seen in association with a ventricular septal defect. It has been reported to occur in 3-6% of cases of tetralogy of Fallot (TOF). In this case report we discuss a case of absent pulmonary valve syndrome with tetralogy of Fallot that was detected in utero by fetal echocardiography at 27 weeks of gestation.

Case: A 20-year-old pregnant woman at 27 weeks of gestation referred to our Institute. She has no consanguineous history. We diagnosed the case as tetralogy of Fallot with absent pulmonary valves in fetal echocardiographic study.

Conclusion: We conclude that when a paracardiac cystic, pulsatile lesion with dilated pulmonary arteries are seen in the fetus in utero then other features associated with the syndrome, such as TOF and the presence or absence of the ductus arteriosus should be looked for. In our case there was no ductus arteriosus.

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Figures

Fig. 1
Fig. 1
An oblique coronal image of the fetal thorax reveals the aneurysmally dilated main pulmonary artery and absence of pulmonary valve echoes (arrow).
Fig. 2
Fig. 2
An axial color Doppler image of the right ventricular outflow tract (arrows) shows the dilated pulmonary artery (PA) and the right ventricle (RV) with a mixed hue of colors suggesting to-and-fro random flow between the right ventricle and the pulmonary artery.
Fig. 3
Fig. 3
Sagittal image of the fetal thorax reveals a large subaortic VSD (arrow) between the right ventricle (RV) and the left ventricle (LV). Aorta (AO) is seen arising from the LV.
Fig. 4
Fig. 4
CW Doppler interrogation of RVOT/MPA showing pulmonary regurgitation.
See this image and copyright information in PMC

References

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