PAPVR is a rare congenital defect of the heart found in around 0.4-0.7% postmortem cases(1).
The incidence of PAPVR is comparatively higher in females than males(1).
How Dangerous Is PAPVR?
When diagnosed at the right time, PAPVR is a correctable disease that can be corrected with surgical intervention and repair of the defect. The prognosis associated with surgery is also excellent with a mortality rate of around 0.1%. If the condition is not surgically corrected then a regular follow-up of the patients is necessary to prevent the development of complications, which mainly include right-sided heart failure and pulmonary hypertension. They can be managed with diuretics, beta-blockers, and cardiac glycosides. The development of pulmonary hypertension makes the situation more complex and the prognosis also decreases, but a study by Gustafson et al. reported only a single postoperative death of a female patient suffering from pulmonary hypertension. All in all, pulmonary hypertension is also a rare phenomenon if the patient is regularly followed up and monitored(1).
Is PAPVR Contagious?
Partial Anomalous Pulmonary Venous Return (PAPVR) or Partial Anomalous Pulmonary Venous Connection PAPVC) is not contagious, but a congenital defect, meaning, it is present by birth and does not spread to others by contact. Complementary to its name, in PAPVR some of the pulmonary vein/veins connection become anomalous. Pulmonary veins normally carry oxygenated blood from the lungs to the left side of the heart (left atrium) and pump it to the systemic circulation through the aorta. However, in PAPVR the connection of the pulmonary veins becomes abnormal and connects to the right side of the heart. Pulmonary vein/veins may connect either to the right atrium, superior vena cava, inferior vena cava, innominate vein, coronary sinus, left subclavian vein, and cavae. The most common abnormal connection is of the right lung, which is two times more common and of the right upper pulmonary vein to the right atrium or superior vena cava(1).
What Are The Different Types Of PAPVR?
PAPVR can make different connections depending if it is affecting the right lung or the left lung. The different types of PAPVR include:
- PAPVR with Atrial Septal Defect (ASD) of sinus venosus type (the most common type accounting for nearly 80-90% of all cases)
- Isolated PAPVR (PAPVR without atrial septal defect) is quite rare
- PAPVR in heterotaxia syndromes
Scimitar syndrome (PAPVR of the right pulmonary vein to the inferior vena cava, right lung sequestration and hypoplasia, dextra positioning of the heart to the right side, and abnormal collateral arterial vessel)
Epidemiology Of PAPVR
The epidemiological data of PAPVR is gathered from postmortem cases and it is prevalent in around 0.4-0.7% cases making it a rare phenomenon. Since most of these cases were asymptomatic; the actual incidence of the disease is even lower. It is mostly found in females than males and is also associated with other congenital diseases, such as Turner’s syndrome. Although PAPVR is a congenital defect presenting since the time of birth, clinically it becomes significant in adulthood. The mortality and morbidity associated with the disease are quite low as death due to the disease is only seen in adults and the diagnosis of the disease has been done only postmortem. PAPVR associated morbidity is mostly due to arrhythmias, right-sided heart failure, and pulmonary hypertension, which also occurs in adults only(1).
What Are The Symptoms Of PAPVR?
PAPVR may continue to be clinically asymptomatic to being symptomatic. The presence of symptoms mainly depends on the size of the anomaly and the number of anomalous veins. Usually, only one vein is affected, so people may remain asymptomatic throughout their life.
Children are usually asymptomatic and may only present with cardiac murmurs or intolerance to exercise. The continuous right heart overload over years and pulmonary hypertension cause various symptoms. Also, the atrial septal defect may complicate the situation. Adults may complain of chest pain, shortness of breath, palpitations, edema of the peripheries, hemoptysis, and chest infections(1).
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