Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ Key Features +++ Essentials of Diagnosis ++ Marked cyanosis present from birth ECG with left axis deviation, right atrial enlargement, and left ventricular hypertrophy (LVH) +++ General Considerations ++ There is complete atresia of the tricuspid valve with no direct communication between the righ tatrium and the right ventricle (RV) There are two types atresia based on the relationship of the great arteries: normally related or transposed great arteries Because there is no flow to the RV, development of the RV depends on the presence of a ventricular left-to-right shunt Severe hypoplasia of the RV occurs when there is no ventricular septal defect (VSD) or when the VSD is small +++ Clinical Findings ++ Cyanosis Growth and development are poor Exhaustion during feedings Tachypnea Dyspnea Heart failure (HF) may develop in patients with increased pulmonary blood flow A murmur from the VSD is heard best at the lower left sternal border Digital clubbing is present in older children with long-standing cyanosis +++ Diagnosis +++ Imaging ++ Radiography Heart is slightly to markedly enlarged Main pulmonary artery segment is usually small or absent Size of the right atrium is moderately to massively enlarged, depending on the size of the communication at the atrial level Pulmonary vascular markings Usually decreased May be increased if pulmonary blood flow is not restricted by the VSD or pulmonary stenosis Echocardiography Two-dimensional echocardiography is diagnostic Color-flow Doppler imaging can help identify atrial level shunting and levels of restriction of pulmonary blood flow +++ Diagnostic Procedures ++ Electrocardiography Shows marked left axis deviation P waves are tall and peaked, indicative of right atrial hypertrophy LVH or LV dominance is found in almost all cases RV forces are usually low or absent Cardiac catheterization and angiocardiography Reveals a right-to-left shunt at the atrial level Right atrial pressure is increased if the atrial septal defect (ASD) is restrictive LV and systemic pressures are normal Catheter cannot be passed through the tricuspid valve from the right atrium to the RV A balloon atrial septostomy is performed if a restrictive patent foramen ovale (PFO) or ASD is present +++ Treatment ++ In infants with unrestricted pulmonary blood flow, conventional anticongestive therapy with diuretics and afterload reduction should be given until the infant begins to outgrow the VSD In infants with diminished pulmonary blood flow, PGE1 is given until an aortopulmonary shunt (BT shunt or ductal stent) can be performed. Sometimes, a pulmonary artery band is needed to protect the pulmonary bed from excessive flow and development of pulmonary vascular disease A Glenn procedure (superior vena cava to pulmonary artery anastomosis) is done with takedown of the aortopulmonary/BT shunt at 4–6 months when saturations begin to fall Completion of the Fontan procedure (redirection of inferior vena ... Your Access profile is currently affiliated with [InstitutionA] and is in the process of switching affiliations to [InstitutionB]. Please select how you would like to proceed. Keep the current affiliation with [InstitutionA] and continue with the Access profile sign in process Switch affiliation to [InstitutionB] and continue with the Access profile sign in process Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Error: Incorrect UserName or Password Username Error: Please enter User Name Password Error: Please enter Password Sign in Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free profile for additional features.