Abstract
A case of congenital absence of both aortic and pulmonary valves with severe heart failure detected prenatally by cross-sectional and pulsed and colour Doppler echocardiography is reported in small for gestational age male fetus in 17th week of gestation. Additional double outlet right ventricle, hypoplastic left ventricle, and ventricular septal defect, as well as multiple extracardiac anomalies, were found by prenatal echocardiographic investigation and confirmed by necropsy examination. Retrograde diastolic Doppler waveforms retrieved from pulmonary artery, aorta, and umbilical arteries revealed massive insufficiency throughout both the great arteries, which eliminated diastolic placental perfusion, documented by absent anterograde diastolic flow in the umbilical vein. These prenatal echocardiographic findings may contribute to an understanding of the mechanism of rapid and progressive heart failure and growth retardation in the fetus. Severe cardiac failure may explain why congenital aplasia of both the aortic and the pulmonary valves has not been described postnatally, and only two fetal cases revealed by necropsy have been published.
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- Bierman F. Z., Yeh M. N., Swersky S., Martin E., Wigger J. H., Fox H. Absence of the aortic valve: antenatal and postnatal two-dimensional and Doppler echocardiographic features. J Am Coll Cardiol. 1984 Mar;3(3):833–837. doi: 10.1016/s0735-1097(84)80261-2. [DOI] [PubMed] [Google Scholar]
- Emmanoulides G. C., Thanopoulos B., Siassi B., Fishbein M. "Agenesis" of ductus arteriosus associated with the syndrome of tetralogy of Fallot and absent pulmonary valve. Am J Cardiol. 1976 Mar 4;37(3):403–409. doi: 10.1016/0002-9149(76)90290-3. [DOI] [PubMed] [Google Scholar]
- Fouron J. C., Sahn D. J., Bender R., Block R., Schneider H., Fromberger P., Hagen-Ansert S., Daily P. O. Prenatal diagnosis and circulatory characteristics in tetralogy of Fallot with absent pulmonary valve. Am J Cardiol. 1989 Sep 1;64(8):547–549. doi: 10.1016/0002-9149(89)90442-6. [DOI] [PubMed] [Google Scholar]
- Hartwig N. G., Vermeij-Keers C., De Vries H. E., Gittenberger-De Groot A. C. Aplasia of semilunar valve leaflets: two case reports and developmental aspects. Pediatr Cardiol. 1991 Apr;12(2):114–117. doi: 10.1007/BF02238416. [DOI] [PubMed] [Google Scholar]
- Lin A. E., Chin A. J. Absent aortic valve: a complex anomaly. Pediatr Cardiol. 1990 Oct;11(4):195–198. doi: 10.1007/BF02238366. [DOI] [PubMed] [Google Scholar]
- Miyabara S., Ando M., Yoshida K., Saito N., Sugihara H. Absent aortic and pulmonary valves: investigation of three fetal cases with cystic hygroma and review of the literature. Heart Vessels. 1994;9(1):49–55. doi: 10.1007/BF01744495. [DOI] [PubMed] [Google Scholar]
- Rein A. J., Singer R., Simcha A. Prenatal diagnosis of tetralogy of Fallot with absence of the leaflets of the pulmonic valve. Int J Cardiol. 1992 Feb;34(2):211–213. doi: 10.1016/0167-5273(92)90158-y. [DOI] [PubMed] [Google Scholar]