Singer Amy Grant, 59, underwent heart surgery last week to correct a rare congenital heart condition. "She had open-heart surgery to correct a condition from birth the doctors discovered during a heart checkup called PAPVR," Grant's representative . "Thankfully the doctor said it could not have gone better. We're praying for a full and easy recovery over the next days, weeks, and months to come."
PAPVR stands for partial anomalous pulmonary venous return, a condition where some of the pulmonary veins return to the right side of the heart, instead of going to the left atrium.
The "Every Heartbeat" singer was diagnosed in February when she underwent a battery of tests to check her heart. This was done prophylactically because Grant's father had a history of heart disease. Grant was asymptomatic at the time. In a in February (which happened to be Heart Awareness Month) Grant gave a shout-out to her doctor for discovering the condition and reported that "instead of concerts and camping trips this summer, I am going to take care of my heart." She also urged fans to take care of their hearts as well.
What is PAPVR?
Under normal conditions, oxygen-poor blood returns to the right atrium of the heart via the inferior vena cava and superior vena cava (SVC). After passing through the tricuspid valve into the right ventricle, the blood passes through the pulmonary arteries into the lungs, where it becomes oxygenated. The oxygen-rich blood returns to the heart by the pulmonary veins which enter the left atrium. The blood then passes through the mitral valve into the left ventricle. From there it is pumped into the aorta to be delivered to the body.
PAPVR is sometimes called partial anomalous pulmonary venous connection (PAPVC). In this condition, one or more of the pulmonary veins return to the right side of the heart. These vessels can connect directly into the right atrium or can connect to other venous pathways such as the inferior vena cava or the innominate vein.
Occasionally all four pulmonary veins connect to the right side of the heart. This is called total anomalous pulmonary venous return (TAPVR). It is a cyanotic congenital defect which has an incidence of 0.6 to 1.2 per 10,000 live births. These infants are typically ill at birth and undergo surgical repair as soon as they are clinically stable to do so.
The incidence of PAPVR is estimated at 0.4-0.7%. However, these results are based on autopsy studies, which may underestimate the actual prevalence as many patients with the disorder are asymptomatic. There is no evidence of racial predilection for PAPVR, but it does occur more frequently in females. There is no known genetic predisposition.
PAPVR occurs twice as often with vessels from the right lung than from the left. The most common form is one in which an upper pulmonary vessel connects to the right atrium or SVC. Eighty to ninety percent of PAPVR cases also have an atrial septal defect (ASD), most commonly of the sinus venosus type (an ASD that involves the venous inflow of either the SVC or the inferior vena cava).
Pathophysiology and Symptoms
In PAPVR, because the anomalous pulmonary veins are returning oxygenated blood to the right side of the heart, most patients are asymptomatic. However, this oxygenated blood does increase the amount of blood that flows through the right side of the heart. How big a shunt occurs depends on the number of anomalous vessels, and whether an ASD with left to right shunt is present.
Over a period of many years, the increased blood flow to the right side of the heart can cause right atrial prominence and ventricular enlargement. The consequences of this may include an increased risk of arrhythmia, right-sided heart failure, and even pulmonary hypertension.
Although many patients with PAPVR are asymptomatic, the condition may be detected during a routine physical examination when patients are noted to have a heart murmur and undergo echocardiogram. Older patients may be symptomatic with symptoms of shortness of breath, arrhythmias, chest pain, and heart failure.
Diagnosis
Echocardiography is the most frequently used method for an initial diagnosis of PAPVR. Increasingly, cardiac magnetic resonance imaging is being used for further delineation of the abnormalities, particularly if surgical intervention is being considered.
Treatment
Patients who are asymptomatic with small left to right shunts usually do not require any intervention.
Surgical repair is the definitive treatment for those with PAPVR with clinically significant left to right shunts and symptomatic patients. The aim is to surgically reroute the anomalous vessels to the left atrium. The specific procedure depends on the site of the anomalous vein(s) and whether there are additional cardiac abnormalities also being addressed.
For patients with PAPVR involving the SVC, repair techniques include internal patches, with or without SVC enlargement and the Warden technique -- a procedure where the SVC is divided, the upper segment of the SVC is connected to the right atrial appendage, and the lower SVC is redirected to the left atrium.
Overall the prognosis of patients with PAPVR (including those who undergo surgical repair) is excellent. The prognosis is more guarded in patients who have developed pulmonary hypertension.
Michele R. Berman, MD, and Mark S. Boguski, MD, PhD, are a wife and husband team of physicians who have trained and taught at some of the top medical schools in the country, including Harvard, Johns Hopkins, and Washington University in St. Louis. Their mission is both a journalistic and educational one: to report on common diseases affecting uncommon people and summarize the evidence-based medicine behind the headlines.