Pediatric Tricuspid Atresia Differential Diagnoses

  • Author: P Syamasundar Rao; Chief Editor: Stuart Berger, MD   more...
 
Updated: Oct 15, 2011
 
 

Differential Diagnoses

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Contributor Information and Disclosures
Author

P Syamasundar Rao  MD, Professor of Pediatrics, Division of Cardiology, University of Texas Medical School at Houston; Director, Division of Pediatric Cardiology, Children's Memorial Hermann Hospital; Professor of Pediatrics, MD Anderson Cancer Center, University of Texas

P Syamasundar Rao is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, American Pediatric Society, Medical Association of Georgia, Society for Cardiac Angiography and Interventions, Society for Pediatric Research, Southern Society for Pediatric Research, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Paul M Seib, MD  Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital

Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Ameeta Martin, MD  Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine

Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD  Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

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Cardiac specimen from a patient with the muscular type of tricuspid atresia. The right atrium was opened by cutting through the right atrial appendage (RAA). Note the dimple (arrow) in the floor of the right atrium with muscle fibers radiating around it. An atrial septal defect (ASD) is also shown. From Rao PS, Levy JM, Nikicicz E, Gilbert-Barness EF. Tricuspid atresia: association with persistent truncus arteriosus. Am Heart J 1991, 122:829, with permission.
Systemic arterial saturations. Left ventricular (LV) and aortic (Ao) values are plotted against the pulmonary-to-systemic blood-flow ratio (Qp:Qs). Both type I and type II anatomy are included. Note the curvilinear relationship between the parameters. At low Qp:Qs levels, a slight increase in Qp:Qs produces large increase in systemic oxygen saturation; at high Qp:Qs levels, a further increase does not produce a notable increase in oxygen saturation. The ideal Qp:Qs appears to be 1.5-2.5, which results in oxygen saturations in the low 80s. From Rao PS. Cardiac catheterization in tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. 2nd ed. Mt Kisco, NY: Futura Publishing Co: 1982:153, with permission.
Geographic prevalence of tricuspid atresia by continent. Note that prevalences are similar on all continents except for Australia. This lone exception is thought to be related to the small sample size from Australia. CHD = congenital heart defects; TA = tricuspid atresia. From Rao PS. Demographic features of tricuspid atresia. In: Rao PS, ed, Tricuspid Atresia. 2nd ed. Mt Kisco, NY: Futura Publishing Co; 1992:23, with permission.
Actuarial survival curves from 3 reported clinical series compiled by Dick and Rosenthal (1992) show a high initial mortality rate in the first year of life, a plateau between the first year and middle of the second decade of life, and a second increase in the mortality rate after the middle of the second decade of life; this second rise is presumably related to impaired left ventricular function. From Dick M and Rosenthal A. The clinical profile of tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. Mt Kisco, NY: Futura Publishing Co; 1982:83, with permission.
Actuarial survival rate of 100 patients who had tricuspid atresia and who were undergoing a Fontan operation at the Hospital for Sick Children, Toronto, in 1975-1989. The survival rate 5 years after the operation is 70%. From Freedom RM, et al. The Fontan procedure for patients with tricuspid atresia: long-term follow-up. In: Rao PS, ed. Tricuspid Atresia. 2nd ed. Mt Kisco, NY: Futura Publishing Co; 1992:377, with permission.
Use of electrocardiographic mean QRS vector (axis) in the frontal plane for the differential diagnosis of a cyanotic newborn with decreased pulmonary blood flow. Associated ventricular hypertrophy patterns and decreased right ventricular (RV) forces are also helpful. From Rao PS. Management of neonate with suspected serious heart disease. King Faisal Spec Hosp Med J 1984 (4):209, with permission.
Frontal plane mean QRS vector in 308 patients, plotted by anatomic type. Most patients with tricuspid atresia type I (normally related great arteries) have an abnormally superior vector, also called left axis deviation. Only one half of patients with tricuspid atresia type II have an abnormally superior vector. Most patients with tricuspid atresia type III (subtype A) have an inferiorly oriented frontal plane vector. From Rao PS, Kulungara RJ, Boineau JP. Electrovectorcardiographic features of tricuspid atresia. In: Rao PS, ed, Tricuspid Atresia. 2nd ed. Mt Kisco, NY: Futura Publishing Co; 1992:141, with permission.
Subcostal 4-chamber 2-dimensional echocardiographic view of a neonate with tricuspid atresia shows an enlarged left ventricle (LV), a small right ventricle (RV), and a dense band of echoes where the tricuspid valve echo should be. Atrial and ventricular septal defects and the mitral valve are also seen. Note the attachment of the anterior leaflet of the detectable atrioventricular valve to the left side of the interatrial septum. Reprinted from Rao, Fetal and Neonatal Cardiology, 1990, with permission from Elsevier Science.
Selected cineangiographic frames from superior vena caval (SVC) and right atrial (RA) frontal angiography in 2 patients. Note sequential opacification of the left atrium (LA) and left ventricle (LV) without opacification of the right ventricle. The RA on the right, the LA superiorly, and the LV on the left form a nonopacified right ventricular window (arrow). This is a classic appearance of the muscular variety of tricuspid atresia. From Rao PS. Tricuspid atresia: anatomy, imaging, and natural history. In: Brawnwald E, Freedom RM, eds. Atlas of Heart Disease: Congenital Heart Disease. Vol 12. Philadelphia, PA: Current Medicine; 1997:14.1 with permission.
Selective superior vena caval (SVC) injection for a 4-chamber projection (hepatoclavicular) shows tricuspid atresia and filling of the left atrium (LA) through a somewhat restrictive atrial septal defect (arrows). Note the retrograde filling of the coronary sinus (CS). RA = right atrium. From Schwartz DC, Rao PS. Angiography in tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. 2nd ed. Mt Kisco, NY: Futura Publishing Co; 1992:223, with permission.
 
 
 
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