Multimodality imaging of anomalous pulmonary veins

Abstract

Partial anomalous pulmonary venous connection (PAPVC) is an extremely rare congenital condition where one or more of the pulmonary veins are connected to the venous circulation. Although initially suspected with unexplained right ventricular enlargement on transthoracic echocardiography (TTE), cardiac MRI is able to delineate the anatomical variant. We present a case of a 65-year-old male diagnosed with left sided PAPVC using multimodality cardiac imaging. Case ReportA 65-year-old male with diabetes, hyperlipidemia, and hypertension presented with palpitations in the absence of dyspnea, chest pain, nor syncope. On physical examination, the patient was hypertensive with a blood pressure of 167/125 mmHg. The jugular venous pressure (JVP) and carotid contour were normal. There was evidence of a parasternal lift, consistent with right ventricular (RV) enlargement with no evidence of cardiac murmurs. Transthoracic echocardiography (TTE) confirmed moderate RV enlargement with preserved systolic function. A small patent foramen ovale (PFO) was identified with left-to-right shunting on color Doppler ( Figure 1A and 1B, Additional File 1, Movie 1A and Additional File 2, Movie 1B). Transesophageal echocardiography (TEE) confirmed the left-to-right shunt across the PFO by color Doppler with intermittent right-to-left flow following administration of agitated saline contrast ( Figure 1C, Additional File 3, Movie 1C). However, the degree of RV enlargement on both TTE and TEE was disproportionate to the interatrial shunt. Cardiac magnetic resonance imaging (CMR) demonstrated a congenital partial anomalous connection of both left pulmonary veins to the innominate vein as the etiology of RV enlargement ( Figure 1D).

Wait, treat and see: echocardiographic monitoring of brain-dead potential donors with stunned heart

“…At least in theory, the stress echo approach might also be applied to these potential heart donors with left ventricular abnormalities, since viability response during stress echo effectively recognizes viable tissue with non-fixed response, as opposed to necrotic response with scar and fixed wall motion abnormalities following inotropic challenge with either dobutamine or dipyridamole [ 29 ]. Information on resting function, viability and ischemia can all be obtained in a one-stop shop with pharmacologic stress echocardiography in a bedside, low-cost, and radiation-free approach [ 30 ].…”

Anomalous unilateral single pulmonary vein versus scimitar syndrome: Comparison of two paediatric cases and a review of the literature

“…Scimitar syndrome is an anomaly of pulmonary venous drainage into the venous circulation, resulting in a left‐to‐right shunt. A variant of PAPVD, partial anomalous pulmonary venous connection or partial anomalous pulmonary venous return, the syndrome has a prevalence of 0.4–0.7% 3–6 . The syndrome is named for the anomalous vessel's resemblance to the scimitar, which is a Turkish sword with a curved blade.…”

“…The vessel usually drains the entire right lung, courses anterior to the hilum and pierces the diaphragm before draining into the IVC 1 . Also known as hypoplastic right lung syndrome, the anomalous pulmonary vein in scimitar syndrome is characteristically associated with an atrial septal defect, hypoplastic right lung and systemic arterial supply to the basal segments of the right lung 1,4,5,7,8 . While scimitar syndrome is characteristically right‐sided, two left‐sided scimitar veins have been described 9,10 …”

“…Conventional diagnostic techniques, such as the plain chest radiograph, often have difficulty distinguishing the abnormal scimitar veins from adjacent normal vessels. MRI 4,8,14,15 and CT angiography 3,7,8,16 are increasingly being used to confirm the diagnosis. Nordmeyer et al 15 .…”

“…recently used flow‐sensitive four‐dimensional magnetic resonance imaging on six patients to visualise the blood flow of selective aberrantly draining pulmonary veins. Quantification of the degree of left‐to‐right shunt in patients with scimitar syndrome is important in deciding upon intervention for these patients 4,8,15,16 …”