Can You Die From PAPVR & How Do You Stop It From Spreading?

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

PAPVR, a congenital heart defect, has no known etiology(1).

Can You Die From PAPVR?

Although death from illnesses is possible, it is very rare to die from PAPVR. It has an excellent prognosis and very low morbidity and mortality rates after surgery that is <0.1%. Therefore, it is necessary to diagnose and treat the condition before the development of complications, especially pulmonary hypertension. Although PAPVR can be managed symptomatically with diuretics, beta-blockers, cardiac glycosides, and other medications, the only definitive treatment is surgical repair of the defect(1).

The surgical defect can be corrected with either an internal patch technique with or without superior vena cava enlargement or the caval division technique with anastomosis of atriocaval junction (Warden technique). The choice of technique is dependent on the site of abnormal vein/veins(1).

Surgery may be associated with certain complications, such as pulmonary venous obstruction, arrhythmias, SVC syndrome, and sick sinus syndrome. Complications may also develop in adults with unrepaired PAPVR due to chronic right heart dilation, cardiac failure, and pulmonary hypertension. Therefore, long term follow-up is necessary for repaired PAPVR and unrepaired PAPVR cases, especially for patients who did not undergo surgery, since they have a greater chance of development of right heart failure and pulmonary hypertension. Regular monitoring with ECG and 24-hour ambulatory ECG by a cardiologist or cardiothoracic surgery is necessary to prevent the development of complications that may prove fatal, especially pulmonary hypertension(1).

How To Stop PAPVR From Spreading?

PAPVR has no known cause and it is not associated with any teratogens (infections or drugs) or has any genetic predisposition. However, it is associated with other congenital syndromes, such as Turner’s syndrome. To stop PAPVR from spreading, it is important to diagnose the condition as early as possible and manage the symptomatically or surgically. However, it is very common to misdiagnose the condition due to its rarity and common symptoms that overlap with other conditions. Imaging studies such as chest radiography, echocardiography, MRI, CT scan, and cardiac catheterization are important techniques to diagnose the condition(1).

Partial Anomalous Pulmonary Venous Return (PAPVR) is a congenital heart abnormality of rare origin. PAPVR involves the defective connection of some of the pulmonary veins to the right side of the heart, mainly 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 right upper pulmonary vein to the right atrium or superior vena cava, which is mostly accompanied by an atrial septal defect of sinus venosus type. PAPVR without atrial septal defect is also seen, but it is a very rare phenomenon(1).

PAPVR is so rare that it is noted in only 0.4-0.7% of autopsy cases and the clinical cases are even rarer due to its asymptomatic nature.

Females are more commonly afflicted with this defect and since it is a congenital defect, it is present by birth, but the disease becomes clinically significant only in adulthood with the development of symptoms and complications(1).

Clinical Course Of PAPVR

PAPVR is mostly asymptomatic in children except cardiac murmurs and intolerance to exercise. Some adults may also remain asymptomatic and diagnosed during postmortem since the development of symptoms depends on various factors, such as the number of pulmonary veins affected, presence of other anomalies (atrial septal defect, heterotaxia) and the size and location of the affected veins(1).

Symptoms develop progressively due to continuous blood overload on the right atrium and ventricle and gradual development of the pulmonary vascular obstructive disease. Right-sided heart failure may present clinically as shortness of breath (dyspnea), chest pain, peripheral edema (swelling of ankles, feet, and hands), arrhythmias, and palpitations. The signs include ascites, liver enlargement, and distension of the jugular vein in the neck along with swelling of the ankles. The development of pulmonary hypertension and the atrial septal defect may complicate the disease course(1).

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