Abstract
This chapter reviews the basics of anomalous pulmonary venous return. The author reviews the signs and symptoms as well as the anatomic definitions of the various forms of anomalous venous return. Anesthetic concerns of caring for patients with anomalous venous return are discussed.
A one-hour-old male is severely tachypneic. He is the product of a full-term spontaneous vaginal delivery to a healthy 27-year-old G1 now P1 mother with good prenatal care. In the delivery room, Apgar scores were 7 at one and five minutes. Central and peripheral cyanosis were present and did not improve with supplemental oxygen.
Diagnosis
What Are the Important Diagnoses to Consider?
Differential diagnosis for this patient is broad and includes:
transient tachypnea of the newborn;
meconium aspiration;
pulmonary hypertension;
congenital heart disease.
Life-threatening diagnoses such as total anomalous pulmonary venous return (TAPVR) should be ruled out prior to evaluation for less severe disorders.
Is Routine Newborn Pulse Oximetry Screening Useful in Identifying TAPVR?
Not necessarily. Neonates with TAPVR may not be cyanotic at birth. However, pulse oximetry will detect even suboptimal oxygen saturation without frank or severe cyanosis.
Screening pulse oximetry is performed to detect infants with potentially life-threatening duct-dependent lesions or lesions requiring an invasive procedure within the first 28 days of life.
What Clinical Signs and Symptoms are Present in Children with TAPVR?
The signs and symptoms depend of the presence or absence of pulmonary venous obstruction. In patients with obstruction they may include:
Soft murmur over the vertical vein;
Cool to cold extremities;
Cyanosis;
Tachypnea;
Hepatomegaly (infracardiac location of anomalous veins);
Hypotension.
Describe the Anatomy of Pulmonary Venous Drainage in TAPVR
Total anomalous pulmonary venous return is a rare condition in which all pulmonary venous blood empties into the systemic circulation or directly into the right atrium. A right-to-left shunt at the atrial level maintains systemic cardiac output. This may be in the form of a patent foramen ovale (PFO) or atrial septal defect (ASD).
There are four anatomic variants of TAPVR and these are classified according to their relationship with the systemic veins.
Supracardiac TAPVR.
The pulmonary veins retain their connection to the embryologic cardinal veins. Pulmonary veins from the left and right lung coalesce and continue as a vertical vein that drains into the right superior vena cava (SVC), azygous vein, or the left SVC. Supracardiac location is the most common variant and is present in nearly 50% of patients with TAPVR (Figure 66.1).
Cardiac TAPVR.
The PVs retain their connection to the embryologic cardinal veins. The veins drain into the right heart directly via the right atrium or via the coronary sinus.
Infracardiac TAPVR.
The pulmonary veins (PV) retain their connection to the embryologic umbilicovitelline veins. Pulmonary veins from the left and right lung coalesce and continue as a vertical vein that travels inferiorly through the mediastinum, through the diaphragm, and inserts into the portal vein or inferior vena cava (IVC) below the diaphragm. Due to their lengthy course, infracardiac TAPVR has the highest risk of becoming obstructed.
Mixed TAPVR.
Sites of pulmonary venous drainage in mixed TAPVR are variable as the name suggests. Commonly the left and right pulmonary veins have separate drainage patterns. These drainage patterns may involve supra-cardiac, cardiac, or infracardiac locations.
Figure 66.1 Drawing of supracardiac anomalous venous return. The pulmonary veins coalesce posterior to the heart and travel cranially via the vertical vein, eventually draining into the right atrium: IVC, inferior vena cava; SVC, superior vena cava; RA, right atrium; TV, tricuspid valve; RV, right ventricle; PV, pulmonary valve; MPA, main pulmonary artery; LA, left atrium; AoV, aortic valve; LV, left ventricle; MV, mitral valve; PVs, pulmonary veins; VV, vertical vein; IV, innominate vein. Image courtesy of the Centers for Disease Control and Prevention, USA
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