Tricuspid valve disease with significant tricuspid insufficiency in the fetus: diagnosis and outcome

Department of Pediatrics, Division University of California-San Diego Medical Center, California.

The echocardiographic studies and clinical course of 27 fetuses (mean gestational age 26.9 weeks) diagnosed in utero with tricuspid valve disease and significant tricuspid regurgitation were reviewed. The diagnosis of Ebstein's anomaly was made in 17 of the fetuses, 7 had tricuspid valve dysplasia with poorly developed but normally attached leaflets and 2 had an unguarded tricuspid valve orifice with little or no identifiable tricuspid tissue. One fetus was excluded from data analysis because a more complex heart lesion was documented at autopsy. All fetuses had massive right atrial dilation and most who were serially studied had progressive right-sided cardiomegaly. Hydrops fetalis was found in six cases and atrial flutter in five. Associated cardiac lesions included pulmonary stenosis in five cases and pulmonary atresia in six. Four fetuses with normal forward pulmonary artery flow at the initial examination were found at subsequent study to have retrograde pulmonary artery and ductal flow in association with the development of pulmonary stenosis (n = 1) and pulmonary atresia (n = 3). On review of the clinical course of the 23 fetuses (excluding 3 with elective abortion), 48% of the fetuses died in utero and 35% who were liveborn died despite vigorous medical and, when necessary, surgical management, many of whom had severe congestive heart failure. Of the four infants who survived the neonatal period, three had a benign neonatal course, all of whom were diagnosed with mild to moderate Ebstein's anomaly; only one had pulmonary outflow obstruction. An additional finding at autopsy was significant lung hypoplasia documented in 10 of 19 autopsy reports.(ABSTRACT TRUNCATED AT 250 WORDS)

This article has been cited by other articles:

				U. T. Truong, S. R. Hintz, and T. A. Tacy Ebstein Anomaly in the Fetus: Preparing for the Perfect Storm NeoReviews, May 1, 2011; 12(5): e260 - e269. [Abstract] [Full Text] [PDF]

				T. Shinkawa, A. C. Polimenakos, C. A. Gomez-Fifer, J. R. Charpie, J. C. Hirsch, E. J. Devaney, E. L. Bove, and R. G. Ohye Management and long-term outcome of neonatal Ebstein anomaly J. Thorac. Cardiovasc. Surg., February 1, 2010; 139(2): 354 - 358. [Abstract] [Full Text] [PDF]

				Y. Kaneko, T. Takeda, H. Yoda, J. Kobayashi, N. Takada, K. Tsuchiya, and R. H. Anderson Right Ventricular Plication in a Neonate With an Unguarded Tricuspid Valvar Orifice Ann. Thorac. Surg., January 1, 2009; 87(1): 305 - 307. [Abstract] [Full Text] [PDF]

				S. Paranon and P. Acar Ebstein's anomaly of the tricuspid valve: from fetus to adult Heart, February 1, 2008; 94(2): 237 - 243. [Full Text] [PDF]

				L. Kapusta, R. M. Eveleigh, S. E. Poulino, M. E. Rijlaarsdam, G. J. du Marchie Sarvaas, J. L. Strengers, T. Delhaas, C. L. de Korte, T. Feuth, and W. A. Helbing Ebstein's anomaly: factors associated with death in childhood and adolescence: a multi-centre, long-term study Eur. Heart J., November 1, 2007; 28(21): 2661 - 2666. [Abstract] [Full Text] [PDF]

				P. V. Anagnostopoulos, N. Alphonso, L. Nolke, L. K. Hornberger, G. W. Raff, A. Azakie, and T. R. Karl Neonatal Mitral and Tricuspid Valve Repair for In Utero Papillary Muscle Rupture Ann. Thorac. Surg., April 1, 2007; 83(4): 1458 - 1462. [Abstract] [Full Text] [PDF]

				I. M. Vettraino, R. Huang, and C. H. Comstock The Normal Offset of the Tricuspid Septal Leaflet in the Fetus J. Ultrasound Med., October 1, 2002; 21(10): 1099 - 1104. [Abstract] [Full Text] [PDF]

				J. Lougheed, B. G. Sinclair, K. Fung Kee Fung, J.-L. Bigras, G. Ryan, J. F. Smallhorn, and L. K. Hornberger Acquired right ventricular outflow tract obstruction in the recipient twin in twin-twin transfusion syndrome J. Am. Coll. Cardiol., November 1, 2001; 38(5): 1533 - 1538. [Abstract] [Full Text] [PDF]

				V Fesslova', S Nava, L Villa, and the Fetal Cardiology Study Group of the Italian So Evolution and long term outcome in cases with fetal diagnosis of congenital heart disease: Italian multicentre study Heart, November 1, 1999; 82(5): 594 - 599. [Abstract] [Full Text]

				Y V Maeno, C Boutin, L K Hornberger, B W McCrindle, T Cavalle-Garrido, G Gladman, and J F Smallhorn Prenatal diagnosis of right ventricular outflow tract obstruction with intact ventricular septum, and detection of ventriculocoronary connections Heart, June 1, 1999; 81(6): 661 - 668. [Abstract] [Full Text]

				S. M. Marianeschi, D. B. McElhinney, V. M. Reddy, N. H. Silverman, and F. L. Hanley Alternative approach to the repair of Ebstein's malformation: intracardiac repair with ventricular unloading Ann. Thorac. Surg., November 1, 1998; 66(5): 1546 - 1550. [Abstract] [Full Text] [PDF]

				J. A. M. van Son, V. Falk, M. D. Black, G. S. Haas, and F. W. Mohr Conversion of complex neonatal Ebstein's anomaly into functional tricuspid or pulmonary atresia Eur J Cardiothorac Surg, March 1, 1998; 13(3): 280 - 285. [Abstract] [Full Text] [PDF]

				M. Endo, M. Ohmi, K. Sato, T. Tanaka, H. Kakizawa, and K. Tabayashi Tricuspid Valve Closure for Neonatal Ebstein's Anomaly Ann. Thorac. Surg., February 1, 1998; 65(2): 540 - 540. [Abstract] [Full Text] [PDF]