How Long Will It Take To Recover From PAPVR & How Long Do The Symptoms Last?

The prognosis of PAPVR is excellent with a perioperative mortality rate of <0.1%.⁽¹⁾

In about 80-90% of the cases, PAPVR is associated with the atrial septal defect.⁽¹⁾

If PAPVR is detected at an early stage when no complications (such as right heart failure or pulmonary hypertension) have developed, the prognosis is excellent. The definitive treatment for symptomatic PAPVR is the surgical correction of the anomalous vein and defect.⁽¹⁾

Asymptomatic cases do not require any treatment. There is also no need for any special diet or restriction on diet or activity. However, it is important to have regular follow-up with a cardiologist or cardiothoracic surgeon. In patients who did not opt for surgery, it is important to monitor the right heart size, cardiac function, pressure, rhythm and development of arrhythmias, and right heart failure. If the left to right shunt is clinically significant then there is an increased chance of development of pulmonary hypertension with advancing age.⁽¹⁾

Follow-up is also necessary for patients who underwent surgery. They need to have regular ECG and 24-hour ambulatory ECG to assess atrial arrhythmias, possible pulmonary veins, and superior vena cava obstruction.⁽¹⁾

Certain complications can develop after surgery including SVC syndrome, atrial arrhythmias, pulmonary venous obstruction, and sick sinus syndrome. Scimitar syndrome patients may be asymptomatic or develop symptoms associated with lung pathology, such as recurrent pneumonia. If there is a development of pulmonary hypertension then there is an increased risk of death. However, PAPVR is associated with excellent peri-operative mortality of 0.1%, which is comparable to the atrial septal defect. The prognosis decreases if the defect goes undetected for a long time or if pulmonary hypertension develops.⁽¹⁾

The development of symptoms and complications depends on various factors. The primary factor is based on the number of anomalous pulmonary venous connection. Some doctors believe that when 50% or more of the pulmonary veins are abnormal then only PAPVR is clinically significant otherwise the patient may be asymptomatic. In most of the cases, only one of the pulmonary veins is anomalous; therefore, there will be no clinical signs and symptoms. However, PAPVR is mostly associated with the atrial septal defect, which might complicate the scenario causing the additional left to right shunting. Initially, mainly in childhood, the disease goes undetected; however, it becomes symptomatic in adulthood due to continuous pulmonary venous return to the right side of the heart leading to right atrial and ventricular dilatation. This leads to an increased risk of right heart failure, pulmonary hypertension, cardiac arrhythmia, and associated complications.⁽¹⁾

The associated symptoms mainly present in adulthood. In children, cardiac murmurs or intolerance to exercise may be noted. Symptoms include shortness of breath, chest pain, peripheral edema, hemoptysis, and pulmonary hypertension.⁽¹⁾

As the name suggests, PAPVR (Partial Anomalous Pulmonary Venous Return) is an abnormal connection of some of the pulmonary veins. In this, the pulmonary veins, instead of draining into the left atrium, make an abnormal connection with the right side of the heart and drain oxygenated blood into either the right atrium, superior vena cava, inferior vena cava, innominate vein, coronary sinus, and on rare occasions, cavae or left subclavian vein. It is a rare congenital defect of the heart, which is associated with sinus venosus atrial septal defect (ASD) in 80-90% of the cases. The anomaly of the right lung pulmonary vein is twice more common than that of the left lung.⁽¹⁾

PAPVR can be subdivided into several types depending on the various connections it makes and on its anatomical location. It includes:

PAPVR associated with ASD (in 80-90% of the cases)

Isolated PAPVR without associated ASD

Scimitar syndrome (anomalous connection of right pulmonary vein to inferior vena cava, lung sequestration, right lung hypoplasia and dextroposition of the heart to the right side)

Heterotaxia syndrome (PAPVR with compound congenital heart disease)

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Also Read:

• What Is Echocardiographic Diagnosis Of PAPVR?
• What Is PAPVR Repair & What Are The Repair Complications?
• What Leads To PAPVR & Can It Be Cured?