Author + information
- Received March 8, 2017
- Revision received February 5, 2018
- Accepted March 1, 2018
- Published online July 2, 2018.
- Bo Xu, MBBS (Hons)a,∗ (, )
- Jorge Betancor, MDa,
- Paul C. Cremer, MDa,
- Rahul Renapurkar, MDb,
- Gosta B. Pettersson, MDc,
- Craig R. Asher, MDd and
- L. Leonardo Rodriguez, MDa
- aSection of Cardiovascular Imaging, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio
- bDepartment of Diagnostic Radiology, Imaging Institute, Cleveland Clinic, Cleveland, Ohio
- cDepartment of Thoracic and Cardiovascular Surgery, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio
- dDepartment of Cardiovascular Medicine, Heart and Vascular Institute, Cleveland Clinic Florida, Weston, Florida
- ↵∗Address for correspondence:
Dr. Bo Xu, Section of Cardiovascular Imaging, Heart and Vascular Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, Ohio 44195.
- cardiac magnetic resonance imaging
- multidetector cardiac computed tomography
- multimodality imaging
- unroofed coronary sinus
Unroofed coronary sinus (CS) defects are rare anomalies that result from embryologic developmental abnormalities, leading to partial or complete absence of the common wall between the CS and the left atrium. They account for less than 1% of atrial septal defects, having a strong association with a persistent left superior vena cava, and are often difficult to diagnose. Delayed diagnosis may lead to right ventricular enlargement and failure, and pulmonary hypertension. Here, we present the multimodality cardiovascular imaging findings of unroofed CS defects (Figures 1, 2, 3, 4, and 5)⇓⇓⇓⇓⇓. For simplicity, unroofed CS defects are classified as types I, II, and III, referring to complete unroofing, partial unroofing of the mid-portion, and partial unroofing of the terminal portion, respectively. Echocardiography is the first-line imaging modality, whereas multidetector cardiac computed tomography provides detailed anatomical information, when echocardiography is not diagnostic. Cardiac magnetic resonance imaging is useful as an adjunct, particularly when quantification of intracardiac shunting is required (Figure 6).
The authors have reported that they have no relationships relevant to the contents of this paper to disclose. Drs. Xu and Betancor contributed equally to this work and are joint first authors.
- Received March 8, 2017.
- Revision received February 5, 2018.
- Accepted March 1, 2018.
- 2018 American College of Cardiology Foundation