Although the prenatal diagnosis of heart anomalies has improved dramatically during the last 2 decades, the diagnosis of heart anomalies remains a challenge. Tricuspid stenosis has not been previously diagnosed in the early second trimester. The sonographic signs of early detection of tricuspid stenosis at 15 weeks of gestation included normal sized right atrium, small right ventricle, narrow pulmonary artery, and diminished flow through the tricuspid valve. The diagnosis was confirmed by postabortal examination. In this case report we describe, for the first time, early prenatal diagnosis of tricuspid stenosis at 15 weeks’ gestational age.
Tricuspid stenosis (TS) is a rare form of congenital heart disease; it is a cyanotic heart disease because of decreased pulmonary flow. The valve dysfunction can result from primary or secondary causes. Secondary TS is caused by rheumatic heart disease, congenital abnormalities, carcinoid disease, pacemaker catheters, and metabolic or enzymatic abnormalities such as Fabry’s or Whipple’s disease. In this case report, we describe, for the first time, early prenatal diagnosis of tricuspid stenosis at 15 weeks’ gestational age.
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Case Report
In Israel, early vaginal sonography for the detection of fetal structural malformations is a common practice, and the majority of pregnant women undergo this scan between 14 and 16 weeks of gestation. A 28 year old healthy primigravida presented at 15 weeks of a spontaneous pregnancy for early transvaginal ultrasound screening. The patient has prophylactically taken folic acid and denied exposure to any teratogenic drugs. Her family history was unremarkable.
The ultrasound examination showed a normal left ventricle and a small right ventricle ( Figure 1 and Video 1 ), normal flow to the left ventricle, narrow main pulmonary artery with reversed flow ( Figure 2 and Video 2 ), and absence of inferior vena cava with azygos continuation. The right atrium and right ventricle were connected by a narrow tunnel through which severely decreased blood flow passed through the tricuspid valve ( Figure 3 and Video 3 ). An ultrasound examination at 18 gestational weeks confirmed the same structural defects and revealed mild pericardial effusion. The patient opted to terminate the pregnancy at 18 weeks of gestation. The postabortal examination confirmed our diagnosis of tricuspid stenosis ( Figure 4 ). There was a normal left-sided spleen and the possible diagnosis of heterotaxy was excluded.
