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Tricuspid atresia

Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic or an absence of the right ventricle. more...

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Because of the lack of an A-V connection, an atrial septal defect (ASD) must be present to maintain blood flow. Also, since there is a lack of a right ventricle there must be a way to pump blood into the pulmonary arteries, and this is accomplished by a ventricular septal defect (VSD).

Blood is mixed in the left atrium. Because the only way the pulmonary circulation receives blood is through the VSD, a patent ductus arteriosus is usually also formed to increase pulmonary flow.

Clinical manifestations

  • progressive cyanosis
  • poor feeding
  • tachypnea over the first 2 weeks of life
  • holosystolic murmur due to the VSD
  • superior axis and left ventricular hypertrophy (since it must pump blood to both the pulmonary and systemic systems)
  • normal heart size

Treatment

  • PGE1 to maintain patent ductus arteriosus
  • modified Blalock-Taussig shunt to maintain pulmonary blood flow by placing a Gortex conduit between the subclavian artery and the pulmonary artery.
  • cavopulmonary anastomosis (hemi-Fontan or bidirectional Glenn) to provide stable pulmonary flow
  • Fontan procedure to redirect inferior vena cava and hepatic vein flow into the pulmonary circulation

External Links

  • Tricuspid Atresia information from Seattle Children's Hospital Heart Center

Read more at Wikipedia.org


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Elevated NT, normal karyotype = good prognosis: 11-14 weeks' gestation - Obstetrics
From OB/GYN News, 11/1/03 by Miriam E. Tucker

PARIS -- Most karyotypically normal fetuses with elevated nuchal translucency at 11-14 weeks will be born without major abnormalities, Dr. Katia Bilardo reported at the 13th World Congress on Ultrasound in Obstetrics and Gynecology.

Sonographic assessment of nuchal translucency (NT) at 11-14 weeks' gestation is a recognized effective screening method for trisomy 21 and other chromosomal abnormalities. Up to 80% of aneuploid fetuses have increased NT, but so do 3%-5% of karyotypically normal fetuses, said Dr. Bilardo of the Academic Medical Centre, Amsterdam.

Over a 9-year period, 67% of 560 fetuses with elevated NT at 11-14 weeks had normal karyotypes. Of those 376, 81% were normal (detected by subsequent scans or after birth) while 19% had major adverse outcomes, including structural anomalies in 7.2%, spontaneous abortion/intrauterine death in 4.3%, genetic syndromes in 4.0%, and voluntary termination of pregnancy in 3.5%.

The likelihood of abnormality increased with NT thickness, from 8% with NT of 3.5 mm to 30% at 5.5 mm to 78% at 6.5 mm and above, she said at the conference, also sponsored by the International Society of Ultrasound in Obstetrics and Gynecology.

Cardiac defects--including ventricular and atrial septal defects, tricuspid atresia, and double outlet right ventricle--were diagnosed in 13 fetuses/infants, a finding that parallels the experience of other groups.

NT may also be a way to screen for major heart defects, Dr. Bilardo said.

Of 312 surviving fetuses who had normal second scans at 20 weeks, just 3% were born with abnormalities.

COPYRIGHT 2003 International Medical News Group
COPYRIGHT 2003 Gale Group

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