Left and Right Ventricular Function in Fetal Tetralogy of Fallot with Absent Pulmonary Valve

ABSTRACT

Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is a rare form of congenital heart disease with a high risk of perinatal mortality, particularly when diagnosed before birth. We aimed to assess whether global left and right ventricular function in fetal TOF/APV, using the Tei index, correlate with outcome. We reviewed the fetal echocardiogram and clinical outcome of eight fetuses diagnosed with TOF/APV. Of the eight cases, four developed cardiovascular compromise, leading to intrauterine death in two fetuses and neonatal demise in two fetuses, and four fetuses survived the neonatal period. The right ventricular (RV) Tei index was significantly greater and the left ventricular (LV) Tei index tended to be greater in nonsurvivors compared with survivors with TOF/APV (RV Tei, 0.90 ± 0.17 versus 0.30 ± 0.28, p < 0.05; LV Tei, 0.97 ± 0.42 versus 0.54 ± 0.21). The global LV and RV function can be affected in TOF/APV. Furthermore, more severe pulmonary insufficiency and worse biventricular function as assessed by Tei index likely contribute to the high perinatal mortality associated with this disease.

KEYWORDS

Tei index - absent pulmonary valve - fetal echocardiography

REFERENCES

• 1 Razavi R S, Sharland G K, Simpson J M. Prenatal diagnosis by echocardiogram and outcome absent pulmonary valve syndrome.  Am J Cardiol. 2003;  91 429-432
• 2 Moon-Grady A J, Tracy T A, Brook M M, Hanley F L, Silverman N H. Value of clinical and echocardiographic fetures in predicting outcome in the fetus, infant, and child with tetralogy of Fallot with absent pulmonary valve complex.  Am J Cardiol. 2002;  89 1280-1285
• 3 Calan N A, Kan J S. Prenatal diagnosis of tetralogy of Fallot with absent pulmonary valve.  Am J Perinatol. 1991;  8 15-17
• 4 McDonnell B E, Raff G W, Gaynor J W et al.. Outcome after repair of tetralogy of Fallot with absent pulmonary valve.  Ann Thorac Surg. 1999;  67 1391-1396
• 5 Dodge-Khatami A, Backer C L, Holinger L D, Baden H P, Mavroudis C. Complete repair of tetralogy of Fallot with absent pulmonary valve including the role of airway stenting.  J Card Surg. 1999;  14 82-91
• 6 Tei C, Dujardin K S, Hodge D O, Kyle R A, Tajik A J, Seward J B. Doppler index combining systolic and diastolic myocardial performance: clinical value in cardiac amyloidosis.  J Am Coll Cardiol. 1996;  28 658-664
• 7 Abd E I, Rahman M Y, Abdul-Khaliq H et al.. Value of the new Doppler-derived myocardial performance index for the evaluation of right and left ventricular function following repair of tetralogy of Fallot.  Pediatr Cardiol. 2002;  23 502-507
• 8 Sahn D J, Lange L, Allen H D et al.. Quantitative real-time cross-sectional echocardiography in the developing normal human fetus and newborn.  Circulation. 1980;  62 588-597
• 9 Reed K L, Meijboom E J, Sahn D J, Scagneli S A, Valdes-Cruz L M, Shenker L. Cardiac Doppler flow velocities in human fetuses.  Circulation. 1986;  73 41-46
• 10 Satomi G, Kanda S, Katayama H, Yasukochi S, Nakamura K, Takao A. Ebstein's anomaly with or without pulmonary atresia.  Echocardiography. 1989;  6 509-515
• 11 Emmanouilides G C, Thanopoulos B, Siassi B, Fishbein M. Abenesis of the ductus arteriosus associated with the syndrome of tetralogy of Fallot and absent pulmonary valve.  Am J Cardiol. 1976;  37 403-409
• 12 Lakier J B, Stanger P, Heymann M A, Hoffman J I, Rudolph A M. Tetralogy Fallot with absent pulmonary valve. Natural history and hemodynamic considerations.  Circulation. 1974;  50 167-175
• 13 Fouron J C, Sahn D, Bender R et al.. Prenatal diagnosis and circulatory characteristics in tetralogy of Fallot with absent pulmonary valve.  Am J Cardiol. 1989;  64 547-549
• 14 Hiraishi S, Bargeron L M, Isabel-Jones J B, Emmanouilides G C, Friedman W F, Jarmakani J M. Ventricular and pulmonary artery volumes in patients with absent pulmonary valve: factors affecting the natural course.  Circulation. 1983;  67 183-190
• 15 Uebing A, Fischer G, Benthge M et al.. Influence of the pulmonary annulus diameter on pulmonary regurgitation and right ventricular pressure load after repair of tetralogy of Fallot.  Heart. 2002;  88 510-514
• 16 Helbing W A, Niexen R A, Le Cessie S et al.. Right ventricular diastolic function in children with pulmonary regurgitation after repair of tetralogy of Fallot: volumetric evaluation by magnetic resonance velocity mapping.  J Am Coll Cardiol. 1996;  28 1827-1835