Clinical History A 62-year-old woman presented with recurrent syncope. Her history was notable for hypertension, hyperlipidemia, asymptomatic coronary artery disease, and a colectomy secondary to diverticulitis. A transthoracic cardiac ultrasound (TTE) revealed no evidence of significant structural heart disease. An MR angiogram of the brain suggested the possibility of partial anomalous pulmonary venous return, and a cardiac CTA was requested to confirm the diagnosis.
CTA revealed partial anomalous pulmonary venous return of the entire left upper lobe including the lingula, to the left innominate vein via a vertical vein. The left innominate vein drains into the SVC and into the right atrium creating an effective left to right shunt. The left lower lobe pulmonary vein drains conventionally into the left atrium.
(Click on image to enlarge)
Figure 1: Left superior pulmonary vein (LSPV) drains into the innominate vein (*) and subsequently into the superior vena cava (SVC).
Figure 2: 3-D reconstructed image demonstrating the left superior pulmonary vein (LSPV) draining into the inomminate vein (**) and superior vena cava (*) and then into the right atrium (RA).
Figure 3: Single left inferior pulmonary vein (LIPV) draining the left lower lobe is seen draining into the left atrium (LA).
Figure 4: Axial maximum intensity projection demonstrates the left atrium (LA) receiving a left lower lobe pulmonary vein (*). Left atrial appendage (**). There is no left superior pulmonary vein.
Partial anomalous pulmonary venous return (PAPVR) encompasses a group of congenital cardiovascular anomalies in which one or more pulmonary veins drain into the right atrium. PAPVR may occasionally present as an incidental finding in an asymptomatic patient. Symptomatic patients usually present with a spectrum of symptoms including supraventricular tachycardia, exertional dyspnea, and chronic fatigue. The extent of symptoms and physiologic disturbances depends primarily on the degree of shunting, the number of anomalous veins, associated valvular abnormalities, and the presence of concomitant cardiac or pulmonary disease. Surgical correction can be considered for symptomatic patients with a pulmonary to systemic (Qp:Qs) blood ﬂow ratio exceeding 1.5 because of their higher likelihood of progression to pulmonary hypertension and right ventricular failure.
Concomitant cardiovascular anomalies, including hard to detect sinus venous atrial septal defects may be present in up to 80% of cases and should be searched for diligently. Cardiac CT has the combination of excellent spatial resolution and a large field-of-view, and is thus ideally suited for the delineation of associated cardiovascular anomalies.
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Ho ML, Balla S et al. MDCT of partial anomalous pulmonary venous return (PAPVR) in adults. J Thoracic Imaging 2009; 24:89-95.
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Ammash NM, Seward JB et al. partial anomalous pulmonary venous connection: diagnosis by transesophageal echocardiography. JACC 1997, 29:1351-58.
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Suhny Abbara, MD
MGH Department of Radiology
Wilfred Mamuya, MD, PhD
MGH Department of Cardiology