Partial Anomalous Pulmonary Venous Connection Follow-up

Updated: Jan 27, 2015
  • Author: Monesha Gupta, MD, MBBS, FAAP, FACC, FASE; Chief Editor: Stuart Berger, MD  more...
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Follow-up

Further Outpatient Care

Intermittent follow-up to assess right heart size and pressures and cardiac function and rhythm is necessary in patients who did not undergo surgical treatment. With a significant shunt, the pulmonary artery pressures can be elevated, and pulmonary vascular resistance can increase with age.

Postoperatively, possible obstruction of the pulmonary veins and superior vena cava (SVC) should be evaluated with echocardiography.

Regular electrocardiography (ECG) and 24-hour ambulatory ECG are also indicated to monitor for atrial arrhythmias.

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Further Inpatient Care

Routine postoperative care of the patient who has undergone cardiac surgery for partial anomalous pulmonary venous connection (PAPVC) should be performed. Pain control should be optimal to reduce the risk of atelectasis.

Anticipate early extubation unless contraindications are recognized; these include excessive chest tube drainage, hemodynamic instability, and oversedation. Encourage early mobilization. Monitor for atrial flutter, atrial fibrillation, and sinus node dysfunction.

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Transfer

Patients should be transferred to an institution skilled in pediatric cardiology and pediatric cardiac surgery for assessment and treatment.

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Deterrence/Prevention

PAPVC is a congenital cardiac defect with no known cause and possibly has a multifactorial origin, including a genetic component. It is also seen with Turner syndrome.

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Complications

No significant complications develop in pediatric patients who do not undergo surgery for a sinus venosus atrial septal defect (ASD) repair with internal patch technique to include the right upper pulmonary vein in the left atrium. However, other types of repair may be associated with complications such as SVC syndrome, pulmonary venous obstruction, atrial arrhythmias, and sick sinus syndrome.

Arrhythmias may occur in adults with unrepaired PAPVC secondary to chronic right atrial enlargement due to volume overload.

Cardiac failure and, very rarely, pulmonary hypertension may develop in adults.

Patients with scimitar syndrome may be asymptomatic or may have symptoms related to lung pathology, including recurrent pneumonias.

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Prognosis

Prognosis is excellent for patients with PAPVC. The perioperative mortality rate is comparable to that for ASD repair (< 0.1%).

Prognosis becomes more guarded if the lesion is undetected for a long period and if complications, particularly pulmonary hypertension, develop.

The only postoperative death in the series by Gustafson et al involved a woman with pulmonary hypertension. [6]

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Patient Education

Advise patient and parents regarding long-term risks if a large shunt remains unrepaired. If pulmonary hypertension has developed, outline the risks of pregnancy, including death.

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