Какой порок развития у плода

Дисплазия трёхстворчатого клапана, с недостаточностью, правосторонний гидроторакс

Tricuspid valve dysplasia with severe tricuspid regurgitation: fetal pulmonary artery size predicts lung viability in the presence of small lung volumes.

Nathan AT1, Marino BS, Dominguez T, Tabbutt S, Nicolson S, Donaghue DD, Spray TL, Rychik J.

Author information

Abstract

Congenital tricuspid valve disease (Ebstein's anomaly, tricuspid valve dysplasia) with severe tricuspid regurgitation and cardiomegaly is associated with poor prognosis. Fetal echocardiography can accurately measure right atrial enlargement, which is associated with a poor prognosis in the fetus with tricuspid valve disease. Fetal lung volumetric assessments have been used in an attempt to predict viability of fetuses using ultrasonogram and prenatal MRI.

We describe a fetus with tricuspid dysplasia, severe tricuspid regurgitation, right atrial enlargement and markedly reduced lung volumes. The early gestational onset of cardiomegaly with bilateral lung compression raised the possibility of severe lung hypoplasia with decreased broncho-alveolar development. Use of fetal echocardiography with measurement of pulmonary artery size combined with prenatal MRI scanning of lung volumes resulted in an improved understanding of this anomaly and directed the management strategy towards a successful Fontan circulation.

2010 S. Karger AG, Basel.

Tricuspid valve dysplasia with regurgitation

Fabrice Cuillier, MD.

Department of Gynecology, Felix Guyon Hospital, 97400 Saint- Denis, Ile de la Reunion, France; tel: 0262 90 55 22; fax : 0262 90 77 30.

Case report

This is a case of a 30-year-old woman, G2 P1, who was referred to our antenatal unit at 23 weeks of gestation to rule out congenital cardiac anomalies.

We observed cardiomegaly, with dilated right ventricle and right atrium. The tricuspid valve was dysplastic. The main pulmonary artery and left and right pulmonary artery seemed narrowed.

The ultrasound scan at 27 weeks of gestation showed markedly dysplastic tricuspid valve. Doppler imaging revealed to and fro flow across the right ventricle and right atrium. The right ventricle was dilated with thin myocardium. The right atrium was markedly dilated due to blood regurgitation from the right ventricle to the right atrium.

Pulmonary artery and both branches were hypoplastic with reverse flow.

The patient decided for amniocentesis which was normal, 46 XX. The screening for fetal infections, TORCH, was negative.
Parents wished to continue in their pregnancy. Patient delivered at term. The baby weighted 3000 grams. The postnatal echocardiogram confirmed the prenatal findings. Unfortunatelly, baby died first day after delivery due to functional pulmonary insufficiency.

Images 1,2: Image 1 shows a 4-chamber view with markedly dilated right atrium. Image 2 shows dialted right atrium and tricuspid valve which is dysplastic.

Images 3,4: Images of the dysplastic tricuspid valve, note the dilated right atrium.

Images 5-8: Doppler flow through the tricuspid valve showing the regurgitation from the right ventricle to the right atrium.

Images 9,10: Image 9 shows main pulmonary artery (AP) with it's branches, it measures 3.8 mm in diameter (aorta: 5.8 mm). Image 10 shows blood flow through the aorta and pulmonary artery. Direction of the flow through the pulmonary artery is different than via aorta, reverse flow in the pulmonary artery.

Images 11,12: Doppler of the flow at the level of the pulmonary valve.