What Happens To Untreated PAPVR & When To Go To A Doctor For It?

Partial Anomalous Pulmonary Venous Return (PAPVR) is a rare congenital disorder of the heart(1).

The right lung PAPVR is twice more common than the left lung PAPVR(1).

What Happens To Untreated PAPVR?

PAPVR has an excellent prognosis for asymptomatic cases and there is no need for treatment in such cases. The peri-operative mortality rate is <0.1%, which is comparable to the atrial septal defect. The prognosis decreases in individuals who are not diagnosed for a long period and if complications pursue especially pulmonary hypertension. In pulmonary hypertensive females, there are risks during pregnancy that may include death(1).

When To Go To A Doctor For PAPVR?

Management is needed for symptomatic cases. Adults with heart failure may be managed with diuretics, beta-blockers, and cardiac glycosides. Although, definitive care for symptomatic PAPVR patients is surgical repair of the defect; however, there are controversies regarding its indication. There are two schools of thought; one believes that all children with PAPVR should undergo surgical repair due to very low mortality and morbidity post-surgery. The other school of thought believes that surgery should only be undertaken when there is significant left-to-right shunt (Qp:Qs is 2:1 or greater) or if an entire lung has abnormal pulmonary veins(1).

The operative technique depends on the site of the abnormal vein/veins. The repair technique for PAPVR to the superior vena cava (SVC) may either include internal patch techniques with or without SVC enlargement or Warden technique (caval division technique with atriocaval anastomosis). It is important to follow up pediatric patients with internal patch techniques for complications such as obstruction of the SVC with SVC syndrome, obstruction of the pulmonary veins, sick sinus syndrome, and supraventricular tachyarrhythmias(1).

There is no diet or activity/exercise restriction for the patients. However, patients should consult a cardiologist and cardiothoracic surgeon. It is important to visit the cardiologist/cardiothoracic surgeon for a follow up as needed to monitor the right heart size, pressure, rhythm, and cardiac function in patients who did not opt for surgical repair. Monitoring of atrial arrhythmias with ECG and 24-hour ambulatory ECG should be done regularly as directed by the physician. Adults with unrepaired PAPVR may develop arrhythmias, cardiac failure, and on rare occasions pulmonary hypertension. In postoperative patients, it is important to monitor for complications such as obstruction of the pulmonary veins and superior vena cava. Scimitar syndrome patients may either be asymptomatic or may develop symptoms of lung pathology, such as recurrent pneumonia(1).

Partial Anomalous Pulmonary Venous Return (PAPVR) is a rare congenital (by birth) heart disorder in which a few pulmonary veins blood is drained to the right atrium instead of the left atrium. This leads to the flow of oxygenated blood from pulmonary veins to the deoxygenated systemic circulation. Generally, only a single pulmonary vein has a defect and it is only a rare incidence in which all the pulmonary veins from one lung have a defect. PAPVR is very similar to TAPVR (Total Anomalous Pulmonary Venous Return), except in TAPVR all or most pulmonary veins have an abnormal connection with the right atrium(1).

Types Of PAPVR

The association of PAPVR is twice more common with the right lung than with the left lung and usually, PAPVR of right upper pulmonary vein draining into the right atrium or the superior vena cava (SVC) is seen. In 80-90% of the cases, this PAPVR is associated with atrial septal defect of sinus venosus type(1).

The pulmonary vein can also drain into the inferior vena cava (IVC) seen in Scimitar syndrome (also known as Halasz syndrome, hypogenetic lung syndrome, congenital pulmonary venolobar syndrome). It mostly involves the right lung that may show hypoplasia of the lung due to sequestered lobe of the lung, and dextrapositioning of the heart(1).

Isolated PAPVR or PAPVR without atrial septal defect is also noted, but it is quite rare. PAPVR with complex congenital heart disease is associated with left atrial isomerism in which there is a common atrium(1).

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