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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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Outcome of palliative surgical correction for single ventricle physiology with echocardiography as the only preoperative assessment tool
From CHEST, 10/1/05 by Annette Santiago

PURPOSE: Single ventricle physiology occurs when one of the two ventricles is sufficiently small that a series circuit is incompatible with survival. Traditionally, all patients with single ventricle physiology undergo cardiac catheterization before palliative surgical correction. Cardiac catheterization carries small but appreciable risks, including arrhythmias, vascular damage, radiation exposure, patient discomfort, and parental anxiety. Additionally, in the current atmosphere of cost containment, the role of preoperative cardiac catheterization versus echocardiography assessment should be reevaluated. We postulate that by solely, using commonly acquired echocardiographic information, patients with single ventricle physiology can undergo palliative surgical correction with bi-Glenn or Fontan procedure and safely avoid preoperative cardiac catheterization.

METHODS: We retrospectively reviewed the medical records of all the patients who underwent biGlenn and Fontan procedure from April 1995 through June 2003 at the Intensive Care Unit of the Centro Cardiovascular de Puerto Rico y del Caribe. Inclusion criteria included all children with univentricular physiology who had a complete preoperative echocardiogram performed within two weeks of surgery and who underwent palliative surgical corrections in the selected study period.

RESULTS: A total of 37 patients were identified to meet the criteria. The most common preoperative diagnosis was Tricuspid Atresia (n= 15), followed by Single Ventricle (n=13) and Pulmonary Atresia (n=9). There was no discordance between echocardiography and surgical findings. Bi-Glenn was performed in thirty-two patients. One patient of the biGlenn group died for an overall group survival of 96.8%. Twenty patients underwent Fontan procedure. Seventeen patients had undergone prior Glenn shunt. The overall group survival was 100%.

CONCLUSION: Echocardiography is a valuable diagnostic tool if performed after a careful physical examination. It can provide the information needed for a complete and accurate diagnosis of congenital heart diseases.

CLINICAL IMPLICATIONS: We conclude that morbidity and mortality after single ventricle palliative correction is not affected by echocardiography as the only preoperative assessment tool and that routine use of cardiac catheterism with its implicated complications and higher costs may be avoided.

DISCLOSURE: Annette Santiago, None.

Annette Santiago MD * Enrique Carrion MD Cid Quintana MD University of Puerto Rico, San Juan, Puerto Rico

COPYRIGHT 2005 American College of Chest Physicians
COPYRIGHT 2005 Gale Group

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