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Partial anomalous pulmonary venous return

Partial anomalous pulmonary venous return
Literature review current through: Jan 2024.
This topic last updated: Oct 20, 2023.

INTRODUCTION — Partial anomalous pulmonary venous return (PAPVR; also known as partial anomalous pulmonary venous connection [PAPVC]), encompasses a spectrum of congenital cardiovascular anomalies. Blood from one or more pulmonary veins returns abnormally to the right atrium, either directly or indirectly through a variety of systemic venous pathways that connect with the anomalous pulmonary vein.

The anatomic abnormalities that result in PAPVR and the diagnosis and management of PAPVR will be reviewed here. Total anomalous pulmonary venous connection (TAPVC) is discussed separately. (See "Total anomalous pulmonary venous connection".)

PREVALENCE — The estimated prevalence of PAPVR is 0.2 to 0.7 percent [1,2]. The true prevalence may be higher since mild cases may go unrecognized [3-6]. PAPVR is more common in patients with Turner syndrome [7,8]. (See "Clinical manifestations and diagnosis of Turner syndrome", section on 'Cardiovascular disease'.)

PULMONARY VEIN ANATOMIC VARIANTS

Terminology — For congenital anomalies of the pulmonary vein, the terms "anomalous venous return," "anomalous venous connection," and "anomalous venous drainage" are often used synonymously and reflect the same physiology. (See 'Physiology' below.)

However, in the purest anatomic sense, the term "anomalous venous return" is the more general description, while "anomalous venous connection" and "anomalous venous drainage" have distinct meanings, as discussed below. (See 'Partial anomalous pulmonary venous connections' below and 'Partial anomalous pulmonary venous drainage' below.)

Normal variants — Normal variants of pulmonary venous return have no hemodynamic or clinical significance. These variants return blood from the pulmonary circulation directly to the left atrium:

The usual arrangement involves two left and two right pulmonary veins that form separate orifices and connect directly to the posterior wall of the left atrium.

In one common variant, pulmonary veins from the entire right or left lung join to form a single vessel, which then drains to the left atrium, thereby forming a single orifice for an entire lung.

In less common variants, there can be a total of five or more pulmonary vein connections, each draining a correspondingly smaller proportion of the lung but all connecting normally to the left atrium.

Total versus partial anomalous pulmonary venous return — There is a wide range of congenital pulmonary vein arrangements that result in anomalous return of blood from the lung to the heart.

Total anomalous pulmonary venous connection (TAPVC) – TAPVC (also known as total anomalous pulmonary venous return [TAPVR]) is a cyanotic congenital defect in which all four pulmonary veins fail to make their normal connection to the left atrium. This results in drainage of all pulmonary veins into the systemic venous circulation. There are four variants of TAPVC, as summarized in the figure (figure 1) and discussed in detail separately. (See "Total anomalous pulmonary venous connection".)

Partial anomalous pulmonary venous return (PAPVR) – PAPVR occurs when blood from one or more, but not all, of the pulmonary veins returns to the right atrium, either directly (ie, due to an abnormal pulmonary venous connection) or indirectly (ie, through a sinus venosus defect or mispositioned septum primum).

Thus, PAPVR consists of two distinct anatomic subtypes with similar physiology, as discussed in the following sections:

Partial anomalous pulmonary venous connections (see 'Partial anomalous pulmonary venous connections' below)

Partial anomalous pulmonary venous drainage (see 'Partial anomalous pulmonary venous drainage' below)

Partial anomalous pulmonary venous connections — In these defects, the anomalous pulmonary vein connects directly to a systemic vein. This results in left-to-right shunting.

Common variants — In the most common form of partial anomalous venous connection, the left upper pulmonary vein connects to the left innominate vein, which in turn drains into the superior vena cava (SVC) (movie 1).

In another variant, small segments of the right upper lobe connect directly to the SVC, usually at or above the level of the azygous vein. Because of the more superior location of the pulmonary vein connection, this anatomy is different from that of sinus venosus defects. (See 'Sinus venosus defects' below.)

Other less common forms include anomalous pulmonary vein connections to the coronary sinus, azygous vein, or the inferior vena cava (IVC).

Scimitar syndrome — Scimitar syndrome is a variant partial anomalous venous connection in which part or even the entire right lung is drained by right pulmonary veins that connect anomalously to the IVC (image 1 and movie 2). The affected lung and its associated airways, which are drained by the scimitar vein, are often hypoplastic or have unusual bronchial or vascular distributions. Sequestration as well as aortopulmonary collateral vessels may also involve the affected lung. (See "Bronchopulmonary sequestration".)

Other cardiac defects are commonly seen and often include hypoplasia of the left heart or aorta [9].

Left-sided scimitar syndrome has also been described, in which some or all of the left pulmonary veins connect to the right-sided IVC [10].

Partial anomalous pulmonary venous drainage — Partial anomalous pulmonary venous drainage is a subtype of PAPVR in which the pulmonary veins connect directly to the left atrium in the usual anatomic location. However, due to an intracardiac defect such as a sinus venosus defect or malposition of the septum primum, physiologically there is a left-to-right intracardiac shunt. Oxygenated blood from one or more pulmonary veins is delivered to only the right atrium or to both the left and right atria simultaneously.

Sinus venosus defects — Normally, the right upper pulmonary vein courses behind the SVC and part of the right atrium before connecting to the left atrium. A sinus venosus defect occurs when there is an absence or unroofing of the wall that normally separates the right upper pulmonary vein from the SVC (movie 3 and movie 4) [11]. This anomaly results in the right pulmonary vein draining into the SVC or into both the right and left atria. In some affected individuals, additional pulmonary veins may drain into to the SVC. (See "Isolated atrial septal defects (ASDs) in children: Classification, clinical features, and diagnosis", section on 'Sinus venosus defects'.)

Inferior sinus venosus defects are an uncommon form of interatrial communications that can lead to the impression of anomalously draining right pulmonary vein(s) [12].

Malposition of the septum primum — Anomalous drainage of one or more normally connecting pulmonary veins results from leftward malposition of the septum primum [13]. In this rare condition, the posterior and/or superior attachments of the atrial septum primum are shifted so far leftward that the pulmonary veins from the right lung drain into the right atrium rather than to the left atrium. This anomaly rarely occurs in isolation. It is usually associated with complex congenital heart conditions, such as hypoplastic left heart syndrome and heterotaxy syndrome [13]. (See "Hypoplastic left heart syndrome: Anatomy, clinical features, and diagnosis" and "Heterotaxy (isomerism of the atrial appendages): Anatomy, clinical features, and diagnosis".)

PHYSIOLOGY — PAPVR results in left-to-right shunting (ie, recirculation of oxygenated blood through the pulmonary circulation). (See "Pathophysiology of left-to-right shunts".)

For patients with isolated PAPVR (ie, without other associated cardiac anomalies), factors that determine the degree of shunting include [14]:

Number and size of anomalous pulmonary veins involved – Shunting from PAPVR involving a single pulmonary vein is usually hemodynamically insignificant, and affected patients are generally asymptomatic without evidence of chamber enlargement on echocardiogram. Patients with two or more anomalous connecting veins may have more significant shunting and may have enlargement of the right atrium, right ventricle, and pulmonary arteries.

Which pulmonary segments or lobes the anomalous veins drain since the distribution of blood flow to each lung segment/lobe varies.

Relative resistance of the normal and abnormal pulmonary veins and compliance of the respective receiving chambers [14].

CLINICAL MANIFESTATIONS — Although PAPVR can present as an isolated abnormality, it more commonly occurs with other cardiac abnormalities, most often an atrial septal defect (ASD). The severity of clinical signs and symptoms is related to the degree of left-to-right shunting and the presence of other associated cardiac and pulmonary defects.

Presentation

Isolated PAPVR — Patients with PAPVR as an isolated finding (ie, without an associated ASD or other cardiac or pulmonary abnormalities) are usually asymptomatic unless there is a large amount of left-to-right shunting (ie, ratio of pulmonary-to-systemic blood flow [Qp:Qs] >1.5) [15]. This is a relatively uncommon scenario. For example, in one series of nearly 300 patients with PAPVR referred for surgical repair, patients with isolated PAPVR with intact atrial septum accounted for only 15 percent of patients requiring repair [16].

The degree of shunting and likelihood of developing symptoms depends on the number of pulmonary veins involved:

Individuals with a single anomalous pulmonary vein are usually asymptomatic. In these patients, the defect may be identified as an incidental finding on imaging performed for another reason [3-6]. However, symptoms can develop in adulthood [17].

Patients with multiple anomalous pulmonary veins are more likely to have clinically significant left-to-right shunting. The presentation is generally similar to that of an isolated ASD or PAPVR associated with an ASD. (See 'PAPVR with an atrial septal defect' below.)

PAPVR with an atrial septal defect — Most patients with PAPVR have an associated ASD. In these patients, the presentation is similar to that of an isolated ASD. (See "Isolated atrial septal defects (ASDs) in children: Classification, clinical features, and diagnosis", section on 'Clinical features'.)

Patients with a small degree of shunting are usually asymptomatic and are often identified incidentally by a murmur detected on physical examination.

Patients with moderate to large shunts may be asymptomatic in childhood. However, as left-to-right shunting increases with age, symptoms generally develop by early adulthood. Initial symptoms may include dyspnea and fatigue, which can progress to overt heart failure. (See "Clinical manifestations and diagnosis of atrial septal defects in adults".)

In symptomatic patients, the severity of findings depends upon the degree of shunting and the presence of pulmonary hypertension. Clinical findings include dyspnea on exertion, recurrent pneumonia, and heart failure.

PAPVR in association with complex CHD — PAPVR can occur as part of more complex congenital heart disease (eg, heterotaxy syndrome, hypoplastic left heart syndrome, other single ventricle variants). In these patients, the presentation is largely dependent on the more severe defect. (See "Heterotaxy (isomerism of the atrial appendages): Anatomy, clinical features, and diagnosis" and "Hypoplastic left heart syndrome: Anatomy, clinical features, and diagnosis".)

Scimitar syndrome — Scimitar syndrome can present in infancy or it may present later in childhood or adulthood.

Presentation in infancy – Patients who present as infants have more severe symptoms related to heart failure and other cardiac and pulmonary conditions [18,19]. Symptoms in infants with scimitar syndrome include tachypnea, poor feeding, failure to thrive, cyanosis, and lethargy. Symptomatic infants generally have a poorer prognosis than patients who present later in childhood or as adults [9]. At the time of diagnosis, most affected infants have pulmonary hypertension. (See 'Outcome' below.)

Later presentation – Many patients who present later in childhood or adulthood are asymptomatic and the abnormality is identified as an incidental finding on chest radiograph (image 2). For patients who present with symptoms, common presenting symptoms include dyspnea, wheezing, cough, exercise intolerance, and recurrent pneumonia [18,20].

Patients with scimitar syndrome often have other associated anomalies, including ASD, pulmonary sequestration, hypoplastic lung, dextrocardia, pulmonary vein stenosis, and other cardiac anomalies (eg, coarctation of the aorta, ventricular septal defect, and patent ductus arteriosus) [18]. (See 'Scimitar syndrome' above and "Bronchopulmonary sequestration".)

Physical examination — The physical findings are directly related to the degree of left-to-right shunting and are similar to those seen in patients with an ASD. (See "Isolated atrial septal defects (ASDs) in children: Classification, clinical features, and diagnosis", section on 'Physical examination'.)

The following findings may be detected:

A precordial bulge due to right atrial and ventricular enlargement.

The aortic and pulmonary components of the second heart sound are generally widely split and fixed. In PAPVR without an ASD, the time interval between the aortic and pulmonary components may not be as prominent and can sometimes sound normal.

A systolic ejection murmur is often heard along the left sternal border due to the increased amount of blood crossing the pulmonary valve in patients with moderate to large left-to-right shunting.

A low-pitch diastolic rumble, representing flow across the tricuspid valve, may be heard at the left lower sternal border in patients with a large shunt.

Chest radiograph and ECG findings — Findings on chest radiography and electrocardiogram (ECG) may be suggestive of pulmonary overcirculation from left-to-right shunting; however, these are nonspecific findings.

Chest radiography – The following radiographic findings may be seen in patients with PAPVR, depending on the degree of left-to-right shunting:

Cardiomegaly including right atrial prominence and right ventricular enlargement, which may be seen as retrosternal fullness on a lateral view, is suggestive of increased shunting.

Increased pulmonary vascular markings provide evidence of significant left-to-right shunting.

If the anomalous vein drains into the superior vena cava (SVC) or innominate vein, the superior mediastinal shadow may appear wide or the SVC shadow prominent.

In scimitar syndrome, the anomalous vein is often identifiable as it courses towards the diaphragm and the inferior vena cava (IVC), giving the characteristic "scimitar" appearance (image 2). (See 'Scimitar syndrome' above.)

Electrocardiogram – The ECG in patients with PAPVR may be normal or may demonstrate evidence of right heart enlargement and/or right ventricular hypertrophy. These changes may include:

Right axis deviation of the frontal plane QRS complex

Evidence of right atrial enlargement with an increased amplitude of the P wave (waveform 1)

Right ventricular hypertrophy with tall R waves in the right precordial leads (V1, V2, V3R, and V4R) and deep S waves in the left precordial leads (V5 and V6) (waveform 2)

qR pattern in the right precordial leads, which suggests volume-related right ventricular hypertrophy

In patients with sinus venosus defects, the P wave axis may be deviated leftward with a negative P wave in lead III [21]. (See "ECG tutorial: Chamber enlargement and hypertrophy".)

DIAGNOSIS — The diagnosis of PAPVR is made with cardiovascular imaging. Echocardiography may be sufficient for making the diagnosis in some cases, particularly in smaller/younger patients. In other cases, magnetic resonance imaging (MRI), computed tomography (CT), or cardiac catheterization are required to fully characterize the anatomy.

Echocardiography — Echocardiography is the most frequently used method for an initial diagnosis of PAPVR. PAPVR should be considered when the echocardiogram demonstrates unexpected or unexplained right atrial or right ventricular enlargement (ie, not explained by another structural abnormality such as a secundum atrial septal defect [ASD]). In cases with right heart enlargement, PAPVR is inferred if there are fewer than four pulmonary veins connecting to the left atrium, although one must consider the possibility of a single pulmonary vein draining an entire lung.

In addition, PAPVR should be considered if the superior vena cava (SVC), innominate vein, inferior vena cava (IVC), or coronary sinus appear dilated, and no other clear explanations are present. In these cases, the anomalous pulmonary vein may connect directly to the dilated structures.

Color flow mapping and other Doppler techniques may aid the identification of anomalous connections to the innominate vein, SVC and IVC, or right atrium (movie 3) [22]. Transesophageal echocardiography (TEE) is more sensitive than transthoracic echocardiography (TTE) in detecting PAPVR (movie 3 and movie 4) and heart chamber enlargement [23,24]. However, the superior-most aspect of the SVC and the innominate veins are better evaluated with TTE.

A potential limitation of echocardiography is the availability of acoustic windows. Vessels that cross behind large airways such as the mainstem bronchi are masked due to attenuation of the ultrasound beams. In some patients, especially those with chronic lung disease, the anterior and medial margins of the lungs can mask the areas of interest. Distinguishing between a secundum ASD and a sinus venosus defect, with or without anomalous pulmonary vein connections, is often challenging. While TEE can circumvent some of the issues with poor acoustic windows, it also can be subject to the same problems as TTE when the airways obscure areas of interest.

Prenatal diagnosis of PAPVR by fetal echocardiography is feasible and has been reported in a large tertiary care center [25,26], although its predictive value remains to be fully defined. (See "Congenital heart disease: Prenatal screening, diagnosis, and management", section on 'Advanced fetal cardiac evaluation'.)

Magnetic resonance imaging — Cardiovascular MRI (CMR) is becoming widely available as a cardiovascular diagnostic technique used to diagnose and characterize congenital heart disease, including PAPVR [27,28]. (See "Clinical utility of cardiovascular magnetic resonance imaging".)

The optimal use of CMR is in larger/older cooperative children who can lie still and hold their breath on command. CMR imaging in young children or anxious patients often requires sedation or even general anesthesia.

When echocardiography is equivocal or imaging quality is limited (eg, due to poor windows), CMR can provide additional information, including quantitation of heart chamber volumes, ventricular mass, and blood flow through the great vessels [29,30].

There are several CMR techniques that are particularly useful in the diagnosis of PAPVR:

Magnetic resonance angiography typically uses gadolinium-based intravenous contrast agents and provides enhanced visualization of the pulmonary vasculature, including the anomalous pulmonary vein(s) (image 1). In patients with renal failure, CMR is capable of viewing vascular structures without intravenous contrast, though the diagnostic accuracy may be reduced.

Phase-contrast velocity mapping is a CMR technique that is used to noninvasively measure blood flow. It can derive the ratio of pulmonary to systemic blood flow (Qp:Qs), which is a quantitative measure of left-to-right shunting [31,32]. (See 'Physiology' above.)

Computed tomography — Cardiac CT can be used to diagnose or confirm PAPVR [33]. CT has several advantages over other imaging modalities. It provides more detailed anatomic information than echocardiography and, like CMR, is not limited by narrow acoustic windows. CT is more readily available than CMR. It takes less time to perform CT and therefore it usually does not require anesthesia or sedation in younger patients. Unlike CMR, intravenous contrast is absolutely necessary to provide anatomic detail, including visualization of the pulmonary vasculature. The benefits of CT must be balanced with the known long-term risks of exposure to ionizing radiation, especially in small children [34-36]. However, modern scanners use lower doses of ionizing radiation.

Cardiac catheterization — Although cardiac catheterization can establish a definitive diagnosis of PAPVR, its role as a diagnostic tool for this condition has largely been replaced by less invasive diagnostic modalities (ie, echocardiography, MRI, and CT). For patients with PAPVR, catheterization is most commonly used if the patient requires therapeutic intervention (eg, occlusion of small anomalous connections). In addition, cardiac catheterization is used to obtain additional hemodynamic information, such as pulmonary vascular resistance, cardiac output, and ventricular pressures. Shunt volume can be calculated by oximetry, but some authors have questioned its reliability in PAPVR [37] since mixed venous saturation can be difficult to measure, especially if the anomalous vein drains directly into the SVC.

MANAGEMENT

Management approach — Our general approach to management is as follows:

Asymptomatic patients with hemodynamically insignificant left-to-right shunt – Asymptomatic patients with small PAPVR-related left-to-right shunts without evidence of right heart volume overload generally do not require intervention, as the defect has no significant clinical impact and life expectancy without correction appears to be normal [14].

Patients with symptoms and/or hemodynamically significant left-to-right shunt – For patients with signs and symptoms attributable to right heart volume overload and those with clinically significant left-to-right shunts, surgery is the definitive treatment. Transcatheter occlusion may be used to reduce the shunting in selected cases.

We define hemodynamically significant left-to-right shunt as a ratio of pulmonary to systemic blood flow (Qp:Qs) ≥2:1. However, practice varies and other experts use lower thresholds. For example, the American Heart Association 2018 adult congenital heart disease management guidelines use a threshold of Qp:Qs >1.5:1 [38].

Patients with recurrent pulmonary infections – In many centers, patients with PAPVR who have recurrent pulmonary infections without an alternative identified etiology are also considered candidates for surgical repair. Management of patients with scimitar syndrome who have recurrent pulmonary infections in the setting of pulmonary sequestration is discussed separately. (See "Bronchopulmonary sequestration", section on 'Management'.)

Patients undergoing surgery for repair of other major cardiac lesions – Repair of PAPVR may be undertaken during surgical repair of other major cardiac lesions, depending upon the surgical risk of repair and level and degree of shunting.

Surgery — The surgical technique varies depending upon the anatomy (particularly where the pulmonary veins connect and whether there are other associated cardiac anomalies), local expertise, and practice preferences.

Surgical repair focuses on closing the atrial septal defect, if present, and redirecting the anomalous pulmonary veins into the left atrium. Care is taken to minimize the risk of injury to the sinus node or its blood supply and to minimize the risk of long-term pulmonary venous or superior vena cava obstruction.

Surgical repair is simpler when the anomalous pulmonary veins are close to the right atrium. These defects can usually be repaired with a technique in which the anomalous pulmonary venous drainage is redirected with a patch through the atrial septal defect into the left atrium. However, repair is more complex for high pulmonary venous connections (ie, when the pulmonary veins enter the superior vena cava above the cavoatrial junction). Various techniques have been described using prosthetic grafts, pericardial patches, or atrial wall flaps to baffle the anomalous pulmonary veins into the left atrium. Alternatively, an cavoatrial anastomosis technique (the Warden procedure) may be used [39,40].

The likelihood of a successful procedure and its potential benefit must be weighed against the risks of the intervention itself. For example, surgical correction in infants and toddlers is more technically challenging than in larger patients.

OUTCOME — PAPVR encompasses a heterogenous group of congenital cardiovascular anomalies. Long-term outcomes vary considerably depending upon the anatomy of the pulmonary venous connections, whether there are associated cardiac or pulmonary defects, and the patient’s age at presentation and repair.

PAPVR in isolation or with associated atrial septal defect (ASD) – Most patients with PAPVR (either as an isolated defect or in association with an ASD) who undergo repair in childhood have a low risk of mortality or major long-term morbidities [41-43]. Potential long-term complications after surgical repair include late venous obstruction and sinus node dysfunction, particularly in patients who undergo surgical incisions across the superior cavoatrial junction [44-47].

PAPVR associated with complex congenital heart disease (CHD) – For children with PAPVR in association with complex CHD, long-term outcomes are largely determined by the more severe defect (eg, heterotaxy syndrome, hypoplastic left heart syndrome). (See "Hypoplastic left heart syndrome: Management and outcome", section on 'Outcome' and "Heterotaxy (isomerism of the atrial appendages): Management and outcome", section on 'Prognosis'.)

Scimitar syndrome – Outcomes for patients with scimitar syndrome depend on the severity and timing of presentation. Patients who present in infancy are generally more severely affected. These patients are at considerable risk of mortality and long-term morbidity [48-50]. Mortality is highest for patients who have preoperative pulmonary hypertension. In a case series of 80 patients with scimitar syndrome managed at a single center, survival for the entire cohort was 76 percent over a median follow-up of 6.2 years [49]. Survival was considerably lower for patients diagnosed at age <1 year compared with those who presented later (68 versus 85 percent, respectively). Most of the nonsurviving children had additional CHD defects, which were severe in many cases. Preoperative pulmonary hypertension and left pulmonary vein stenosis were identified as risk factors for mortality in this series.

SUMMARY AND RECOMMENDATIONS

Anatomy – Partial anomalous pulmonary venous return (PAPVR) encompasses a heterogenous group of congenital cardiovascular anomalies that are caused by the abnormal return of one or more, but not all, of the pulmonary veins to the right side of the heart. (See 'Pulmonary vein anatomic variants' above.)

Scimitar syndrome is a variant of PAPVR in which the right lung is drained by right pulmonary veins that connect anomalously to the inferior vena cava (IVC) (image 1 and movie 2). The affected lung and its associated airways are often hypoplastic with abnormal bronchial anatomy (ie, bronchopulmonary sequestration). (See 'Scimitar syndrome' above and "Bronchopulmonary sequestration".)

Physiology – PAPVR results in left-to-right shunting (ie, recirculation of oxygenated blood through the pulmonary circulation). The degree of shunting depends upon the number and size of anomalous pulmonary veins. Patients with two or more anomalous pulmonary veins generally have clinically significant left-to-right shunting (ie, ratio of pulmonary-to-systemic blood flow [Qp:Qs] >1.5:1). This may result in enlargement of the right atrium and ventricle. (See 'Physiology' above.)

Presentation – The clinical presentation of PAPVR depends upon the degree of left-to-right shunting and the presence of other associated cardiac and pulmonary defects. Most patients with PAPVR have an associated atrial septal defect (ASD). (See 'Clinical manifestations' above.)

Patients with single anomalous veins generally do not have clinically significant left-to-right shunting. These patients are usually asymptomatic and the defect may be identified as an incidental finding on imaging performed for another reason. (See 'Isolated PAPVR' above.)

Patients with multiple anomalous veins and/or an associated ASD have greater degrees of shunting. These patients may be asymptomatic in early childhood. However, as shunting increases with age, symptoms generally develop by early adulthood. Initial symptoms may include dyspnea and fatigue, which may progress to overt heart failure. (See 'PAPVR with an atrial septal defect' above.)

Infants with scimitar syndrome tend to have earlier and more severe symptoms (heart failure, recurrent pulmonary infections, pulmonary hypertension) and often have associated pulmonary and cardiac anomalies. On chest radiograph, the anomalous vein is often identifiable as it courses towards the diaphragm and the IVC, giving the characteristic "scimitar" appearance (image 2). (See 'Scimitar syndrome' above.)

In some patients, PAPVR occurs as part of more complex congenital heart disease (CHD) such as heterotaxy syndrome, hypoplastic left heart syndrome, or other single ventricle variants. In these cases, the presentation is largely dependent on the more severe CHD defect. (See "Heterotaxy (isomerism of the atrial appendages): Anatomy, clinical features, and diagnosis" and "Hypoplastic left heart syndrome: Anatomy, clinical features, and diagnosis".)

Diagnosis – The diagnosis of PAPVR is made with cardiovascular imaging. Echocardiography may be sufficient for making the diagnosis in some cases, particularly in smaller/younger patients. In other cases, magnetic resonance imaging (MRI), computed tomography (CT), or cardiac catheterization are required to fully characterize the anatomy. (See 'Diagnosis' above.)

Management – Surgery is the definitive treatment for PAPVR. Our general approach to surgical decision-making is as follows (see 'Management' above):

Asymptomatic patients with small left-to-right shunts do not require intervention, as the defect has no significant clinical impact.

For patients with hemodynamically significant left-to-right shunting (ie, Qp:Qs ≥2:1) and/or signs and symptoms attributable to right heart volume overload, we suggest surgical intervention (Grade 2C). Other experts may use a lower threshold for intervention (eg, Qp:Qs >1.5:1). The surgical technique varies depending upon the anatomy, local expertise, and practice preferences. Transcatheter occlusion may be used to reduce the shunting in selected cases.

We also suggest surgical intervention for patients with PAPVR who have recurrent pulmonary infections without an alternative identified etiology (Grade 2C). The role of surgery for patients with recurrent pulmonary infections in the setting of bronchopulmonary sequestration due to scimitar syndrome is discussed separately. (See "Bronchopulmonary sequestration", section on 'Management'.)

In addition, repair of PAPVR may be undertaken during surgical repair of other major CHD lesions, depending upon the surgical risk of repair and level and degree of shunting.

Outcome – PAPVR encompasses a heterogenous group of congenital cardiovascular anomalies. Long-term outcomes vary considerably depending upon the anatomy of the pulmonary venous connections, whether there are associated cardiac or pulmonary defects, and the patient’s age at presentation and repair. (See 'Outcome' above.)

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Topic 5764 Version 22.0

References

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