Total Anomalous Venous Return

History

Total anomalous pulmonary venous return (TAPVR) comprises approximately 2.6% of all congenital heart diseases
Almost all types of TAPVR become symptomatic during the first year of life and present with shortness of breath,
cyanosis, or both. The mortality rate of TAPVR in the first year of life is 80%.2 Surgical correction of TAPVR entails
ligation of the abnormal connection between the pulmonary and systemic veins, re-routing
the pulmonary veins into the left atrium and closing the interatrial communication. Following this procedure,
obstruction to the pulmonary venous flow has been reported, especially during infancy. However, superior vena
cava (SVC) obstruction due to neoseptation is an extremely uncommon complication. There are very few reports of
SVC obstruction following repair of TAPVR. Balloon dilatation and stenting of the SVC baffle has been described for
the SVC syndrome following the Mustard and Senning procedures.

Definition

Abnormal development of the pulmonary veins may result in either partial or complete
anomalous drainage back into the systemic venous circulation.

Total anomalous pulmonary venous return (TAPVR) is a congenital disorder characterized by

total mixing of systemic venous blood and pulmonary venous blood within the heart. This
complete mixing of venous blood produces cyanosis. In contrast, partial anomalous pulmonary
venous return (PAPVR) is usually an acyanotic condition.

Symptoms

The infant may appear to be critically ill and may display the following symptoms:

 Lethargy
 Poor feeding
 Rapid breathing
 Poor growth
 Frequent respiratory infections
 Cyanosis (blue discoloration of the skin)

Note: Sometimes, no symptoms may be present in infancy or early childhood.

Exams and Tests

 ECG shows signs of enlargement of the ventricles (ventricular hypertrophy).

 X-ray of the chest shows a normal to small heart with fluid in the lungs.
 Echocardiogram usually defines the attachment of pulmonary vessels.
 Cardiac catheterization can provide definitive diagnosis by showing abnormal
attachments of the blood vessels.
 MRI of the heart can show the connections between the pulmonary vessels.
 Diagnosis is suspected by chest x-ray and established by 2-dimensional echocardiography with
color flow and Doppler studies. Cardiac catheterization is rarely necessary; occasionally, cardiac
MRI or CT angiography may need to be done to better delineate the anatomy of pulmonary
venous return.

Chest x-ray shows a small heart and severe diffuse pulmonary edema when there is pulmonary
venous obstruction; otherwise, there is cardiomegaly with increased pulmonary vascular
markings. ECG shows right axis deviation, right ventricular hypertrophy, and occasionally right
atrial enlargement.

Physical examination

- parasternal lift
- a single, loud 2nd heart sound (S2)
- hear murmurs

Medical

Medical management, particularly the management of patients with partial anomalous

pulmonary venous return, does not differ significantly from that of patients with atrial septal
defect (ASD). Patients with small shunts are usually asymptomatic and require no treatment. On
the other hand, patients with increased pulmonary blood flow, pulmonary hypertension, and
congestive heart failure may initially be managed with diuretics but soon require surgical
correction.

Surgery

For infants with total anomalous pulmonary venous return (TAPVR), surgical correction is
indicated. Before surgery, infants may be stabilized with prostaglandin E1 to dilate the ductus
venosus and the ductus arteriosus. Some patients may require balloon atrial septostomy;
however, this is of little or no benefit when pulmonary venous obstruction is present.

The common pulmonary venous trunk is anastomosed directly to the left atrium; the ASD is
closed; and the connection to the systemic venous circuit is interrupted.

For patients with partial anomalous pulmonary venous return (PAPVR), different

approaches may be used. For example, anomalous pulmonary veins that connect to the
brachiocephalic venous system or the superior vena cava usually are of adequate length to allow
resection and reattachment to the left atrial appendage. For patients in whom the right inferior
pulmonary veins connect to the right atrium or the inferior vena cava, correction may be
achieved by placement of a contoured intracardiac patch to redirect the blood into left atrium
through an ASD.

Type
Respiratory
Hourly respiratory assessment to include presence of bilateral breath sounds, quality of breath sounds, and
evaluation of bilateral chest expansion
Monitor oxygenation and ventilation through arterial blood gas measurements and pulse oximetry
Monitor ventilator settings, parameters, and mean airway pressure measurements
Ensure proper securement of artificial airway per institutional protocol
Maintain patency of the artificial airway per institutional protocol
Ensure provision of ordered respiratory treatments
Obtain chest radiographs as ordered

Cardiac
Ongoing assessment of hemodynamic parameters: heart rate, blood pressure, and cardiac parameters (right atrial
pressure, central venous pressure, left atrial pressure, or pulmonary artery pressure)
Ongoing assessment of markers of cardiac output: near infrared spectroscopy readings, urine output, level of
consciousness, central and peripheral perfusion, capillary refill, and quality of pulses
Monitor and maintain intravenous access for inotropic infusions

Fluid, renal, and electrolytes

Ensure correct administration of inotropic infusions and monitor for patient’s response to inotropic infusions
Strict, hourly intake and output measurements with cumulative 24-hour totals
Monitor and document urine output hourly; notify medical team of decrease in output (<1 mL/kg per hour)
Obtain samples for and monitor results of laboratory chemistries ordered
Notify medical management team of abnormal electrolyte results such as hyperkalemia
Replace electrolytes (eg, sodium, potassium, calcium, or magnesium) as ordered
Provide renal replacement therapy (hemofiltration or peritoneal dialysis) as ordered

Nutrition
Administer parental or enteral nutrition as ordered
Obtain samples for and monitor results of laboratory chemistries (eg, albumin levels) for markers of nutritional status
Monitor tolerance of enteral feedings: stool output, emesis, abdominal distention, and metabolic laboratory values

Hematology
Monitor and report postoperative bleeding such as chest tube output (>5 mL/kg per hour)
Obtain, monitor, and notify surgical team of abnormal coagulation laboratory values

Infectious
Disease
Monitor physical assessment findings that are indicative of signs/symptoms of infection
Monitor daily complete blood cell count for trends
Maintain strict sterile surgical procedure and full barrier precautions for all patient procedures
Administer antibiotics as ordered by the medical team
Obtain samples for and monitor results of surveillance cultures as ordered

Endocrine
Obtain and report abnormal laboratory findings consistent with hypothyroidism, adrenal insufficiency, and/or
hyperglycemia/hypoglycemia
Maintain glycemic control in the form of insulin infusion as ordered by the medical team
Obtain glucose measurements hourly when patient is receiving an insulin infusion and titrate according to medical
team orders and parameters (eg, glucose goal of 100-150 mg/dL)

Pain control and

Sedation
Assess and monitor the patient for adequate pain control and sedation
Administer opioids in the form of infusions and as-needed boluses for pain management as ordered
Administer sedation in the form of infusions and as-needed boluses as ordered
Notify medication team for needed patient changes in pain management and sedation
Provide a daily paralytic holiday if the patient is undergoing chemical paralysis in the form of an infusion to provide
for an adequate neurological assessment after clearance of the paralytic agent

Preventive
Interventions
Pressure sore: Ongoing shift assessments with appropriate skin assessment tools
Use pressure-relief devices
Turn patient every 2 hours when hemodynamically stable
Notify medical team of skin changes associated with development of pressure sores
Eye care: Monitor and prevent corneal abrasions with an ordered preventive lubricant and complete eyelid
closure if patient undergoing chemical paralysis

Parent
Interventions
Provide ongoing clinical updates on child’s status
Provide referrals for parental support such as child life specialists, social workers, chaplains, and palliative care as
needed
Use a patient care delivery model such as Family-Centered Care to meet the needs of each family individually and promote good
outcomes