Bhalla, Sanjeev

Eghtesady, Pirooz

Ludbrook, Philip A

Martinez, Sara C

Texas Heart Institute Journal

English

PubMed

Sara C. Martinez

Pirooz Eghtesady

Sanjeev Bhalla

Philip A. Ludbrook

Crossref

Scimitar Syndrome: Multimodal Imaging Before and After Repair

Digital Commons@Becker

Washington University School of MedicineDigital Commons@BeckerOpen Access Publications2015Scimitar syndrome: Multimodal imaging beforeand after repairSara C. MartinezWashington University School of Medicine in St. LouisPirooz EghtesadyWashington University School of Medicine in St. LouisSanjeev BhallaWashington University School of Medicine in St. LouisPhilip A. LudbrookWashington University School of Medicine in St. LouisFollow this and additional works at: https://digitalcommons.wustl.edu/open_access_pubsThis Open Access Publication is brought to you for free and open access by Digital Commons@Becker. It has been accepted for inclusion in OpenAccess Publications by an authorized administrator of Digital Commons@Becker. For more information, please contact engeszer@wustl.edu.Recommended CitationMartinez, Sara C.; Eghtesady, Pirooz; Bhalla, Sanjeev; and Ludbrook, Philip A., ,"Scimitar syndrome: Multimodal imaging before andafter repair." Texas Heart Institute Journal.,. . (2015).https://digitalcommons.wustl.edu/open_access_pubs/8136Texas Heart Institute Journal http://dx.doi.org/10.14503/THIJ-14-4462      593© 2015 by the Texas Heart ® Institute, HoustonScimitar Syndrome:Multimodal Imaging Before and After RepairA 19-year-old woman was noted on an incidental chest radiograph to have dextroposition of the heart secondary to a hypoplastic right lung, with a lin-ear opacity to the right of the right atrium extending below the diaphragm (Fig. 1). Although the patient was asymptomatic, a subcostal view via transthoracic echocardiography showed substantial dilation of the right atrium and right ventricle, a pulmonary artery systolic pressure of 27 mmHg, and an abnormal vessel and flow entering a dilated inferior vena cava (IVC) (Fig. 2). Subsequent cardiac catheterization and angiography of the right pulmonary artery revealed complete anomalous right pulmonary venous (PV) drainage from the right lung to the infradiaphragmatic IVC with a pulmonic-to-systemic flow ratio (Qp:Qs) of 2.11:1 (Fig. 3). Cardiac magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) were per-formed before and after gadofosveset-trisodium contrast administration. This nonin-Images inCardiovascularMedicineSara C. Martinez, MD, PhDPirooz Eghtesady, MD, PhDSanjeev Bhalla, MDPhilip A. Ludbrook, MDSection Editor: Raymond F. Stainback, MD, Department of Adult Cardiology, Texas Heart Institute, 6624 Fannin St., Suite 2480, Houston, TX 77030From: Division of Cardio-vascular Medicine, Depart-ment of Internal Medicine (Drs. Ludbrook and Marti-nez), Section of Pediatric Cardiothoracic Surgery, Department of Surgery (Dr. Eghtesady), and Cardio-thoracic Imaging Section, Department of Radiology (Dr. Bhalla), Washington University School of Medi-cine, St. Louis, Missouri 63110Address for reprints: Sara C. Martinez, MD, PhD, Division of Cardiovascular Medicine, Department of Internal Medicine, 660 S. Euclid Ave., Box 8086, Washington Univer-sity School of Medicine, St. Louis, MO 63110E-mail: smartinez@wustl.eduFig. 1  Frontal chest radio-graph shows a hypoplastic lung with cardiac dextro-position and a linear opacity to the right of the atrium, extending below the diaphragm (arrows).Fig. 2  Transthoracic echo-cardiogram in color-flow Doppler mode (subcostal view) shows Doppler inflow (red) of the anomalous right pulmonary vein as it drains into the inferior vena cava.  Supplemental motion image is available for Figure 2.Volume 42, Number 6, 2015594      Scimitar Syndrome: Multimodal Imaging Before and After Repairvasive study revealed anomalous drainage of the entire right lung into the infrahepatic IVC with a calculated Qp:Qs ratio of 2.1:1, confirmed the prior results, and suggested no anomalous pulmonary arterial supply, left PV drainage, horseshoe lung, or accessory diaphragm (Fig. 4). Because of substantial shunting, the scimitar right PV was repaired by means of an end-to-side anas-tomosis of the right PV to the left atrium adjacent to the atrial septum (Fig. 5). An outpatient postoperative MRI revealed the surgical connection, a decrease in right ventricular cavity size, and a Qp:Qs of 1:1 (Fig. 6). Postoperatively, the patient did well.Fig. 3  Right pulmonary arteriogram (anteroposterior projection) shows anomalous pulmonary venous drainage of contrast medi-um into the inferior vena cava, inferior to the diaphragm (arrows).  Supplemental motion image is available for Figure 3.Fig. 4  Cardiac magnetic resonance image with contrast medium (posterior view in 3-dimensional rendering) defines the entire right pulmonary venous drainage to the infrahepatic inferior vena cava (arrows).Fig. 5  Postoperative frontal chest radiograph shows sternotomy wires and an unchanged heart size, with the cardiac apex lying to the right of the midline. Note the small basilar atelectasis.Fig. 6  Cardiac magnetic resonance angiograms with contrast medium. A) Anterior view shows the surgical connection be-tween the right pulmonary vein and the left atrium. B) Posterior view, in 3-dimensional rendering. Note the postoperative changes associated with repair of the partial anomalous pulmonary venous return: in particular, the surgical connection of the right pulmonary vein to the left atrium.ABTexas Heart Institute Journal Scimitar Syndrome: Multimodal Imaging Before and After Repair      595CommentThe term “scimitar syndrome” in these patients is de-rived from the resemblance of the anomalous right pulmonary venous course to a curved sword of Mid-dle Eastern origin, known as a scimitar. The tip of the “sword” is the downward, anastomotic point of the PV entry to the IVC below the diaphragm. Symptoms can arise from this abnormal anastomosis, should obstruc-tion occur. In experienced and multidisciplinary con-genital heart disease centers, the use of 3-dimensional MRI and MRA enables exquisite anatomic definition in both the pre- and postoperative conditions, distinct from that afforded by ultrasonography and conven-tional angiography. The calculation of shunt ratios, comparable to the calculations enabled by catheteriza-tion, allows MRI and MRA to assist clinicians in decid-ing between surgical and conservative management of patients who have anomalous PV drainage—perhaps sparing them the morbidity of invasive and ionizing procedures.

Scimitar syndrome: Multimodal imaging before and after repair

Scimitar syndrome: Multimodal imaging before and after repair

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