Abstract The conventional upright chest radiograph is obtained with high kilovoltage at maximal inspiration to permit short exposure times, which freeze cardiac motion. Digital chest radiography is performed in a similar manner and provides improved image quality, less overall radiation exposure, increased ease of handling with faster image throughput, and shorter times for image review. The ability to review images electronically at various brightness and contrast levels has decreased the rate of retakes and has provided a much greater dynamic contrast that is helpful for maximizing details of various implanted devices.

Background: The CABANA trial (Catheter Ablation Versus Antiarrhythmic Drug Therapy for Atrial Fibrillation) was a randomized, prospective trial of left atrial catheter ablation versus drug therapy for treatment of atrial fibrillation (AF). As part of CABANA, a prospective imaging substudy was conducted. The main objectives were to describe the patterns of changes in the dimensions of the left atrium and pulmonary veins (PVs), and the relationship between these changes with treatment assignment and clinical outcomes. Methods: Computed tomography or magnetic resonance imaging was acquired at baseline and follow-up in 121 ablation (median follow-up 101 days) and 85 drug patients (median follow-up 97 days). Left atrial volume index, mean PV ostial diameter, and ostial diameters of each PV separately were computed. We examined the relationship between the change from baseline to follow-up with subsequent clinical outcomes (composite of death, disabling stroke, serious bleeding, or cardiac arrest [CABANA primary end point], total mortality or cardiovascular hospitalization, first AF recurrence after the 90-day blanking period, first AF/atrial flutter/atrial tachycardia after the 90-day blanking period) using Cox proportional-hazards models. Results: The median (25th to 75th) change from baseline for left atrial volume index was -7.8 mL/m(2) (-16.4 to 0.2), ablation arm and -3.5 mL/m(2) (-11.4 to 2.6), drug therapy arm. The left atrial volume index decreased in 52.9% of ablation patients versus 40.0% of drug therapy patients. Change for mean PV was -2.7 mm (-4.2 to -1.3) in the ablation arm versus -0.1 mm (-1.5 to 0.8) in the drug therapy arm. Changes in left atrium and PV dimensions had no consistent relationship with the risk of developing the study primary end point. Reductions in left atrial volume index, and in mean PV diameter were associated with decreased risk of AF recurrence. Conclusions: Ablation patients demonstrated more frequent and larger atrial structural changes compared with drug patients. These changes suggest a critical relationship between structural features and AF generation.

IMPORTANCE Catheter ablation is more effective than drug therapy in restoring sinus rhythm in patients with atrial fibrillation (AF), but its incremental effect on long-term quality of life (QOL) is uncertain. OBJECTIVE To determine whether catheter ablation is more beneficial than conventional drug therapy for improving QOL in patients with AF. DESIGN, SETTING, AND PARTICIPANTS An open-label randomized clinical trial of catheter ablation vs drug therapy in 2204 symptomatic patients with AF older than 65 years or 65 years or younger with at least 1 risk factor for stroke. Patients were enrolled from November 2009 to April 2016 from 126 centers in 10 countries. Follow-up ended in December 2017. INTERVENTIONS Pulmonary vein isolation, with additional ablation procedures at the discretion of the investigators, for the catheter ablation group (n = 1108) and standard rhythm and/or rate-control drugs selected and managed by investigators for the drug therapy group (n = 1096). MAIN OUTCOMES AND MEASURES Prespecified co-primary QOL end points at 12 months, including the Atrial Fibrillation Effect on Quality of Life (AFEQT) summary score (range, 0-100; 0 indicates complete disability and 100 indicates no disability; patient-level clinically important difference, >= 5 points) and the Mayo AF-Specific Symptom Inventory (MAFSI) frequency score (range, 0-40; 0 indicates no symptoms and 40 indicates the most severe symptoms; patient-level clinically important difference, <=-1.6 points) and severity score (range, 0-30; 0 indicates no symptoms and 30 indicates the most severe symptoms; patient-level clinically important difference, <=-1.3 points). RESULTS Among 2204 randomized patients (median age, 68 years; 1385 patients [63%] were men, 946 [43%] had paroxysmal AF, and 1256 [57%] had persistent AF), the median follow-up was 48.5 months, and 1968 (89%) completed the trial. The mean AFEQT summary score was more favorable in the catheter ablation group than the drug therapy group at 12 months (86.4 points vs 80.9 points) (adjusted difference, 5.3 points [95% CI, 3.7-6.9]; P <.001). The mean MAFSI frequency score was more favorable for the catheter ablation group than the drug therapy group at 12 months (6.4 points vs 8.1 points) (adjusted difference, -1.7 points [95% CI, -2.3 to -1.2]; P <.001) and the mean MAFSI severity score was more favorable for the catheter ablation group than the drug therapy group at 12 months (5.0 points vs 6.5 points) (adjusted difference, -1.5 points [95% CI, -2.0 to -1.1]; P <.001). CONCLUSIONS AND RELEVANCE Among patients with symptomatic atrial fibrillation, catheter ablation, compared with medical therapy, led to clinically important and significant improvements in quality of life at 12 months. These findings can help guide decisions regarding management of atrial fibrillation.

Central MessageCT-guided transthoracic biopsy of cardiac masses is a solution to obtain tissue safely in specific scenarios; however, preparation should be made to prepare for postbiopsy complications.See Commentary on page e119. CT-guided transthoracic biopsy of cardiac masses is a solution to obtain tissue safely in specific scenarios; however, preparation should be made to prepare for postbiopsy complications. See Commentary on page e119. Obtaining a tissue diagnosis for unresectable intracardiac tumors can be a dilemma. Although open cardiac biopsy is often technically feasible, it may delay the start of appropriate systemic treatment. Although endoluminal catheter-directed biopsy is helpful in many cases, it may not produce diagnostic samples; especially in tumors with reactive surrounding capsules. We present an infrequently described, image-guided biopsy technique adapted to sample heart masses that can minimize morbidity and allow the timely initiation of systemic therapy. A 73-year-old man presented with a right ventricular mass discovered on a routine surveillance computed tomographic (CT) study for moderately differentiated pT3b hepatocellular carcinoma (HCC) after definitive resection through a right partial hepatectomy 3 years previously without chemotherapy or radiation therapy. The patient was free of symptoms and otherwise had no medical problems. He was initially treated with enoxaparin for a presumed right ventricular thrombus. The mass did not resolve according to serial echocardiograms, however, which showed normal left and right ventricular systolic ejection fractions, no valvular disease, and mildly elevated velocities around the mass. Subsequently, cardiac magnetic resonance imaging (Figure 1) demonstrated an enhancing mass infiltrating the anterior right ventricle and right ventricular outflow tract (RVOT). Primary considerations were metastatic HCC, angiosarcoma, and lymphoma. Serum liver function tests and α-fetoprotein were normal. An abdominal magnetic resonance imaging study yielded negative results for recurrent HCC, and a fludeoxyglucose F 18 (INN fludeoxyglucose [18F]) positron emission tomographic and CT study only showed hypermetabolism in the right heart mass, without lesions of concern for metastasis throughout the torso. Endomyocardial biopsy of the mass through a right internal jugular vein catheterization with a 7F sheath, transthoracic ultrasound guidance, and a 50-cm bioptome device only showed interstitial fibrosis within 6 samples (Figure 2, C). A multidisciplinary discussion among representatives from cardiology, oncology, cardiac surgery, and radiology ultimately resulted in the decision to pursue a CT-guided percutaneous transthoracic biopsy. The decision was based on several factors, including probable fibrous capsule of the mass, which precluded a catheter biopsy approach; the unresectability of the mass; and the mass location, along with the delay in initiation of chemotherapy and morbidity associated with an open surgical biopsy. The coronary vessels were mapped before the procedure by means of image review with an expert in cardiac imaging.Figure 2Computed tomographically guided transthoracic biopsy of an aggressive right ventricular outflow tract mass after nondiagnostic endovascular bioptome biopsy in a 73-year-old man with a history of hepatocellular carcinoma. A window between the sternum and the left ribs allowed the introduction of an introducer needle into the right ventricular outflow tract mass. A, Computed tomographic angiography pulmonary embolism protocol confirm the targeted position of the introducer needle within the mass. The needle was slightly withdrawn before 3 core biopsy samples (18 gauge and 2.0 cm long) were obtained. B, A repeat computed tomographic angiogram 10 minutes after removal of the introducer needle excludes early complications. C, Endomyocardial biopsy from the previous endovascular approach showing a nondiagnostic sample with nonspecific cardiac hypertrophy and fibrosis (hematoxylin and eosin, 40× original magnification). D and E, On-site evaluation of the computed tomographically guided transthoracic core biopsy demonstrates loosely cohesive clusters of malignant cells with prominent nucleoli and anisonucleosis (D; air-dried cytologic preparation, 400× original magnification), malignant cells with a nodular growth pattern and eosinophilic cytoplasm (E; hematoxylin and eosin stain, 200× original magnification), and immunohistochemical confirmation of hepatocellular primary tumor (F; hepatocyte immunohistochemistry, 200× original magnification).View Large Image Figure ViewerDownload Hi-res image Download (PPT) Within the interventional CT suite, the patient was placed under general anesthesia, with extracorporeal membrane oxygenation on standby. A cardiac surgeon and expert in ultrasound placement of pericardial drains were on standby. The pathology team was present for rapid on-site cytologic evaluation (Figure 2, D). Patient respirations had a significant impact on shifting the RVOT mass, so manipulation of the needle and firing the biopsy were performed on ventilator end-expiration. A 17/18-gauge introducer needle was advanced into the RVOT component of the mass with the guidance of intermittent stationary CT images of the mass, and the location was confirmed with pulmonary embolism protocol enhanced CT (Figure 2, A). A total of 3 biopsy samples, each 18 gauge and 2.0 cm long, were obtained with a spring-loaded core biopsy device. Care was taken to avoid penetrating the endocardium with the biopsies by aligning the introducer needle within the central aspect of the mass, measuring biopsy length before biopsy, and allowing a plane of soft tissue between the biopsy tip and luminal margin of mass. Pathology confirmed metastatic HCC (Figure 2, E and F). The introducer needle was then removed on ventilator end-expiration. Ten minutes after the biopsy, a repeat CT excluded postbiopsy complications (Figure 2, B). An annotated step-by-step video of the procedure is provided (Video 1). No complications were evident on a postbiopsy transthoracic echocardiogram performed in the CT suite. The patient was free of symptoms after recovering from anesthesia. He was admitted overnight for observation and was discharged the next day after an uneventful evening. To our knowledge, we present here the first case of CT-guided RVOT mass biopsy. Transluminal catheter directed endomyocardial approaches with either fluoroscopic or ultrasound guidance are well-established biopsy techniques.1From A.M. Maleszewski J.J. Rihal C.S. Current status of endomyocardial biopsy.Mayo Clin Proc. 2011; 86: 1095-1102Abstract Full Text Full Text PDF PubMed Scopus (163) Google Scholar Alternatives, however, include ultrasound- and CT-guided percutaneous transthoracic approaches, which may be valuable in select cases.2Daliri A. Oehring K. Moosdorf R.G. Franke F.E. Kalinowski M. Zahedi F. et al.Percutaneous left atrial cardiac biopsy with CT fluoroscopy guidance.J Vasc Interv Radiol. 2007; 18: 909-913Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar, 3Gorospe L. Cabañero-Sánchez A. Muñoz-Molina G.M. Ayala-Carbonero A.M. Fernández-Méndez M.A. Computed tomography-guided core needle biopsy of cardiac angiosarcoma.Arq Bras Cardiol. 2018; 110 (Portuguese): 493-494Google Scholar, 4Nakata A. Hirota S. Takazukura E. Primary cardiac lymphoma diagnosed by percutaneous needle biopsy.Int J Cardiol. 1998; 65: 201-203Abstract Full Text Full Text PDF PubMed Scopus (21) Google Scholar, 5Yamagami T. Kato T. Tanaka O. Hirota T. Ito K. Nishimura M. et al.Percutaneous needle biopsy under CT fluoroscopic guidance for cardiac tumor during continuous intravenous injection of contrast material.J Vasc Interv Radiol. 2005; 16: 559-561Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar In this case, CT-guided transthoracic biopsy proved safe and successful after catheter-directed endomyocardial biopsy had been ineffective in diagnosing isolated HCC metastasis to the right ventricle and RVOT, which is an incredibly rare lesion.6Kim S.B. Shin Y.C. Kwon S.U. Isolated metastasis of hepatocellular carcinoma in the right ventricle: a case report.Medicine (Baltimore). 2016; 95: e5544Crossref Scopus (5) Google Scholar The clinical presentation of the cardiac metastasis in our case mirrors descriptions in the literature: symptom-free patients who had undergone partial hepatectomy for HCC with no hepatic recurrence. Those patients were treated with chemotherapy, and with surgical resection in 1 case.6Kim S.B. Shin Y.C. Kwon S.U. Isolated metastasis of hepatocellular carcinoma in the right ventricle: a case report.Medicine (Baltimore). 2016; 95: e5544Crossref Scopus (5) Google Scholar A β-blocker was not used in our case, because introducer needle manipulation could not be coordinated with the cardiac cycle. Neither transesophageal nor transthoracic echocardiography was utilized because of the suboptimal insonification angle of the biopsy needle; the use of a spring-loaded biopsy device, precluding control of biopsy depth with ultrasound; and the desire to minimize time with introducer needle in the cardiac mass between modalities. Concurrent use of ultrasound, however, may be helpful in different clinical scenarios. The use of a hybrid operating room with C-arm fluoroscopic cone-beam CT was considered and decided against, in response to worries about reaching prohibitive levels of patient radiation exposure and poor image quality relative to standard CT. Cardiac pacing pads were not preemptively placed in this case, because the mass was distant from the primary conduction system; however, such placement should be considered. Our patient was admitted overnight for observation with telemetry because of unknown potential complications; however, this observation period may be overly cautious. A transthoracic CT-guided approach may offer several advantages relative to catheter-directed jaw-type bioptome approaches, including the absence of the need for vascular access; a larger sample size; the sampling of tissue from the central portion of the mass rather than the endocardial aspect, which may be fibrotic; the large anatomic field of view with options to cross lung and bone (particularly the sternum) if needed; precise control of the biopsy needle; and the ability to visualize complications globally immediately after the biopsy. In addition, samples from CT biopsy are amenable to rapid on-site cytologic evaluation, which is challenging with endomyocardial biopsies because the myocytes do not tend to exfoliate onto slides. Endoluminal approaches are well established, however, and have the advantages of postbiopsy bleeding back into the blood pool and an approach that avoids crossing vital structures, such as epicardial arteries. Furthermore, new endoluminal techniques with a core biopsy system (traditionally used for transvenous liver biopsies) and transthoracic or intravascular ultrasound guidance are being described, with histopathologic diagnosis achieved in 72% of cases and the most common complication (7%) being ventricular arrhythmias requiring cardioversion.7Sze D.Y. Lee D.P. Hofmann L.V. Petersen B. Biopsy of cardiac masses using a stabilized intracardiac echocardiography-guided system.J Vasc Interv Radiol. 2008; 19: 1662-1667Abstract Full Text Full Text PDF PubMed Scopus (12) Google Scholar, 8Reddy G. Maor E. Bois M.C. Chandrasekaran K. Rihal C.S. Nishimura R.A. et al.Percutaneous transcatheter biopsy for intracardiac mass diagnosis.EuroIntervention. 2017; 13: e1436-e1443Crossref Scopus (5) Google Scholar Although CT-guided transthoracic biopsy of RVOT cardiac masses is novel, this case illustrates that it is feasible. Masses in the anterior pericardium and right and left atria (when large) may also be optimal targets for a transthoracic CT biopsy. Potential complications of CT-guided transthoracic biopsy include pneumothorax, hemopericardium, air embolism, induction of cardiac arrhythmias, and vascular injury, particularly to the internal thoracic and epicardial arteries. Percutaneous transthoracic biopsy is believed to carry a greater risk of hemorrhage than endovascular biopsy, and preparations must be made to address hemopericardium as an emergency.2Daliri A. Oehring K. Moosdorf R.G. Franke F.E. Kalinowski M. Zahedi F. et al.Percutaneous left atrial cardiac biopsy with CT fluoroscopy guidance.J Vasc Interv Radiol. 2007; 18: 909-913Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar Although transluminal catheter-directed endomyocardial biopsies are currently the standard of care, CT-guided approaches should be considered and may be the optimal technique for large, lobulated cardiac masses that would be biopsied without penetrating the endocardium.2Daliri A. Oehring K. Moosdorf R.G. Franke F.E. Kalinowski M. Zahedi F. et al.Percutaneous left atrial cardiac biopsy with CT fluoroscopy guidance.J Vasc Interv Radiol. 2007; 18: 909-913Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar This minimally invasive technique allowed our patient to proceed with chemotherapy 2 days after his biopsy. More data are needed regarding CT-guided approaches, however, and every case should be carefully scrutinized, with extra attention to epicardial arteries, cardiac valves, and expected location of the conduction system along the biopsy needle path. eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiJiZGQyZWVhODA4NTI4ZjdiZmJlMjIxM2I2NDY5OGI5NSIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjQ0NzQyNDc0fQ.huNEjTY3Wi7jOWiX001TUtIrz5RJQOLCvOMUzGTJw_2vmPKW_1Q2PFoV0n3Ibi5xfk-630GKQg7fq5dFCw_SM9t-utUD41Wiy-_osiRCUh-0afctHR_eDr6S7vn8CueKgkv1jVjkAi9GY08DSIU-qFBFQl-OMrypWVFW43PLfNjbYjOt5M-X1yHyVnyqw5RC8SFgjptv91or3sUtPRn-4JCxdmf_x7f6sEVSppXt5B7Q-rNIFXgoLxc5rPLusIfcavMdaOSeq4pzKudI9Hfbnt68XxFAOGl9gYJ8rqUzZWZaOqQsUP4KZStci2Wek1Zmh1QsbLfmXYetuUDDhxDlcg Download .mp4 (60.78 MB) Help with .mp4 files Video 1A slide show details the computed tomographically (CT) guided cardiac biopsy technique step by step, from the procedure planning to the removal of the introducer needle. ECG, Electrocardiography, ECMO, extracorporeal membrane oxygenation; MRI, magnetic resonance imaging; RVOT, right ventricular outflow tract. Video available at: https://www.jtcvs.org/article/S0022-5223(19)31156-0/fulltext.

BACKGROUND:Spontaneous coronary artery dissection (SCAD) is associated with extracoronary vascular abnormalities, which depending on type and location may warrant treatment or provide additional diagnostic or prognostic information about this uncommon entity. Fibromuscular dysplasia (FMD), aneurysms, and dissections have been detected in multiple vascular territories by magnetic resonance angiography, CT angiography (CTA), and catheter angiography. The optimal modality to detect extracoronary vascular abnormalities is unknown. We highlight the technique and feasibility of a novel CTA protocol to detect extracoronary vascular abnormalities in these patients, incorporating patient safety and convenience.METHODS:The complete CTA protocol consisting of a single CTA of the neck, chest, abdomen, and pelvis was performed on 39 SCAD outpatients. All examinations were performed with 200 mL of low-osmolar contrast agent and used radiation dose modulation techniques. Average volume CT dose index was 9 mGy for the chest, abdomen, and pelvis portions and 21 mGy for the neck portion. Studies were independently reviewed by 2 senior vascular radiologists.RESULTS:Two patients had nondiagnostic CTA neck evaluation because of technical acquisition errors. Extracoronary vascular abnormalities were detected in 27 of 39 patients (69%). Catheter angiography detected brachial artery FMD in 1 patient, a vascular bed not included in the SCAD CTA protocol. Extracoronary vascular abnormalities were common, including FMD, aneurysms, dissection, and aortic tortuosity, and were seen in the iliac (36%), carotid and/or vertebral (31%), splanchnic (10%), and renal (26%) arteries and in the thoracic and/or abdominal aorta (10%).CONCLUSIONS:The frequency of extracoronary vascular abnormalities and extent of territories identified the CTA protocol in our cohort are high. A tailored CTA may be the optimal imaging technique for detecting extracoronary vascular abnormalities in patients with suspected underlying vasculopathy. Although the clinical significance of extracoronary vascular abnormalities remains unclear, detection of these abnormalities has identified patients in whom cerebral imaging and serial monitoring have been recommended.

Computed tomography-myocardial perfusion imaging (CT-MPI) with or without coronary CT angiography (cCTA) is a novel non-invasive method for the assessment of coronary artery disease (CAD) but it is not well established how its diagnostic performance compares to conventional coronary angiography (CA

Papillary fibroelastomas (PFEs) are benign cardiac tumors arising from endocardium. They are commonly found on valvular surfaces and average 1.0–1.5 cm in size. Though often asymptomatic, PFEs can lead to potentially severe complications, primarily due to their embolic potential. Surgical resection is recommended for all symptomatic or large PFEs. We report the case of a patient presenting with cardiovascular symptoms who was found to have a very large aortic valve PFE, as diagnosed by histopathologic examination following surgical resection. Multimodality cardiovascular imaging demonstrates the classic morphologic findings, including a pedunculated appearance and oscillating “frond-like” surface projections.

Pericardial disease can be challenging to diagnose, and imaging can play a useful role in confirming or even suggesting the diagnosis. Computed tomography (CT) is a particularly appealing option for investigating pericardial disease in many patients because the differential diagnosis for symptoms of acute pericarditis or constrictive pericarditis often includes other diseases which are also well assessed with CT. In addition, many patients will have findings of pericardial disease manifest on CT imaging for other suspected diseases, and these findings can be missed if careful attention is not paid to the pericardium. CT also can play an important role in evaluating specific pericardial lesions, such as cysts, tumors, and abscesses. We will review findings of various pericardial diseases on CT with illustrative cases.

Geometric analysis of the left atrium and pulmonary veins is important for studying reverse structural remodeling following cardiac ablation therapy. It has been shown that the left atrium decreases in volume and the pulmonary vein ostia decrease in diameter following ablation therapy. Most analysis techniques, however, require laborious manual tracing of image cross-sections. Pulmonary vein diameters are typically measured at the junction between the left atrium and pulmonary veins, called the pulmonary vein ostia, with manually drawn lines on volume renderings or on image cross-sections. In this work, we describe a technique for making semi-automatic measurements of the left atrium and pulmonary vein ostial diameters from high resolution CT scans and multi-phase datasets. The left atrium and pulmonary veins are segmented from a CT volume using a 3D volume approach and cut planes are interactively positioned to separate the pulmonary veins from the body of the left atrium. The cut plane is also used to compute the pulmonary vein ostial diameter. Validation experiments are presented which demonstrate the ability to repeatedly measure left atrial volume and pulmonary vein diameters from high resolution CT scans, as well as the feasibility of this approach for analyzing dynamic, multi-phase datasets. In the high resolution CT scans the left atrial volume measurements show high repeatability with approximately 4% intra-rater repeatability and 8% inter-rater repeatability. Intra-and inter-rater repeatability for pulmonary vein diameter measurements range from approximately 2 to 4 mm. For the multi-phase CT datasets, differences in left atrial volumes between a standard slice-by-slice approach and the proposed 3D volume approach are small, with percent differences on the order of 3% to 6%.

Patients with atrial fibrillation undergo structural remodeling resulting in increased pulmonary vein sizes. Studies have demonstrated that these changes are reversible following successful ablation therapy. To date, analyses of pulmonary vein structure have focused on measurements at the pulmonary vein ostia, and the full extent of reverse remodeling along the length of the pulmonary veins has not yet been fully characterized.An automated, three-dimensional method is proposed that quantifies pulmonary vein geometry starting at the ostia and extending several centimeters into the veins. A centerline is tracked along the length of the pulmonary vein, and orthogonal planes are computed along the curve. The method was validated against manual measurements on each of the four pulmonary veins for 10 subjects. The proposed methodology was used to analyze the pulmonary veins in 21 patients undergoing cardiac ablation therapy with preoperative and postoperative computed tomographic scans.Validation results demonstrated that the automated measurements closely followed the manual measurements, with an overall mean difference of 11.50 mm(2). Significant differences in cross-sectional area at the two time points were observed at all pulmonary vein ostia and extending for 2.0 cm (excluding the 0.5-cm interval) into the left inferior pulmonary vein, 3.5 cm into the left superior pulmonary vein, and 2.0 cm into the right superior pulmonary vein.Quantitative analysis along the length of the pulmonary veins can be accomplished using centerline tracking and measurements from orthogonal planes along the curve. The patient study demonstrated that reverse structural remodeling following ablation therapy occurs not only at the ostia but for several centimeters extending into the pulmonary veins.

HomeCirculationVol. 126, No. 12Levoatriocardinal Vein With Partial Anomalous Venous Return and a Bidirectional Shunt Free AccessBrief ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessBrief ReportPDF/EPUBLevoatriocardinal Vein With Partial Anomalous Venous Return and a Bidirectional Shunt Ethany L. Cullen, MD, Jerome F. Breen, MD, Charanjit S. Rihal, MD, Robert D. Simari, MD and Naser M. Ammash, MD Ethany L. CullenEthany L. Cullen From the Department of Radiology (E.L.C., J.F.B.) and the Division of Cardiovascular Diseases (C.S.R., R.D.S., N.M.A.), Mayo Clinic, Rochester, MN. , Jerome F. BreenJerome F. Breen From the Department of Radiology (E.L.C., J.F.B.) and the Division of Cardiovascular Diseases (C.S.R., R.D.S., N.M.A.), Mayo Clinic, Rochester, MN. , Charanjit S. RihalCharanjit S. Rihal From the Department of Radiology (E.L.C., J.F.B.) and the Division of Cardiovascular Diseases (C.S.R., R.D.S., N.M.A.), Mayo Clinic, Rochester, MN. , Robert D. SimariRobert D. Simari From the Department of Radiology (E.L.C., J.F.B.) and the Division of Cardiovascular Diseases (C.S.R., R.D.S., N.M.A.), Mayo Clinic, Rochester, MN. and Naser M. AmmashNaser M. Ammash From the Department of Radiology (E.L.C., J.F.B.) and the Division of Cardiovascular Diseases (C.S.R., R.D.S., N.M.A.), Mayo Clinic, Rochester, MN. Originally published18 Sep 2012https://doi.org/10.1161/CIRCULATIONAHA.111.085555Circulation. 2012;126:e174–e177A 52-year-old woman with a history of systemic lupus erythematosus presented to our institution with nonhealing ulcers on her left lower extremity and toes. She had no cardiac symptoms, with a normal ECG and chest radiograph (Figures 1 and 2). As part of her evaluation she underwent a transesophageal echocardiogram to look for endocarditis, which demonstrated a right-to-left shunt with Valsalva that appeared to originate from the vicinity of the lateral aspect of the left atrium (Movie I in the online-only Data Supplement). There was also a vascular structure seen adjacent to the aorta that typically would represent a remnant of the cardinal vein, such as a persistent left superior vena cava. There were no findings of endocarditis.Download figureDownload PowerPointFigure 1. ECG with normal sinus rhythm.Download figureDownload PowerPointFigure 2. Chest radiograph demonstrates normal heart size and pulmonary vascularity.A magnetic resonance angiogram was performed to further evaluate the origin of the right-to-left shunt and demonstrated a levoatriocardinal vein that drained the left upper lobe pulmonary veins. This levoatriocardinal vein connected the left innominate vein with the left atrium via the left superior pulmonary vein (Figure 3). The flow in the levoatriocardinal vein was demonstrated to be primarily directed cephalad, creating a left-to-right shunt, as would be expected. However, cine phase contrast imaging demonstrated a reversal of flow in the levoatriocardinal vein after Valsalva (Figure 4). This was the source of the right-to-left shunt detected on transesophageal echo. Transthoracic echo confirmed the bidirectional flow in the levoatriocardinal vein (Figures 5 and 6, Movies II and III in the online-only Data Supplement). There were no findings of volume overload in the right heart because the left pulmonary veins were still connected to the left atrium, with only partial drainage of these veins into the innominate vein.Download figureDownload PowerPointFigure 3. Four reformatted images from a gadolinium-enhanced magnetic resonance angiogram demonstrate the levoatriocardinal vein (short arrows) draining the left upper lobe pulmonary veins, which also connects to the left atrium (L) via the left superior pulmonary vein (open arrow). i indicates innominate vein.Download figureDownload PowerPointFigure 4. Magnetic resonance images from a cine phase contrast examination showing reversal of flow in the levoatriocardinal vein during Valsalva. Arrows indicate the levoatriocardinal vein. At rest the vein is black indicating flow toward the patient's head. With valsalva the flow reverses and is displayed as white, which indicates flow direction toward the patient's feet.Download figureDownload PowerPointFigure 5. Color Doppler images of the levoatriocardinal vein flow at rest (top) and reversal of the levoatriocardinal vein flow during Valsalva (bottom).Download figureDownload PowerPointFigure 6. Spectral Doppler demonstrates the changes in the levoatriocardinal vein flow at rest (left), with Valsalva (middle), and with release of Valsalva (right).Further clinical history revealed that the patient was having clusters of transient ischemic attacks, most recently after coughing spells during hospitalization for pneumonia while she had an intravenous line in her left arm. This raised the possibility of paradoxical embolism due to reversal of the shunt by severe coughing.This anomaly was determined to be treated best using percutaneous embolization of the levoatriocardinal vein. The planning angiogram nicely demonstrated the patient's variant anatomy (Movie IV in the online-only Data Supplement). The levoatriocardinal vein was closed with a 12-mm Amplatzer vascular plug (AVP II) (Figure 7 and Movie V in the online-only Data Supplement). This successfully occluded the levoatriocardinal vein and eliminated the right-to-left and left-to-right shunts. Follow-up transthoracic echo demonstrated complete occlusion of the levoatriocardinal vein with no residual right-to-left shunt.Download figureDownload PowerPointFigure 7. After deployment of the Amplatz vascular plug into the levoatriocardinal vein.As described by McIntosh1 in 1926, the levoatriocardinal vein is an abnormal persistent connection between the pulmonary venous system and the cardinal (systemic) venous system through the splanchnic plexus of the embryonic foregut. It is most commonly associated with left obstructive lesions such as mitral atresia, hypoplastic left heart, and cor triatriatum. Approximately 50 cases have been reported in the literature, only 3 of which had otherwise normal cardiac anatomy.2–4Patients with levoatriocardinal veins and no other cardiovascular abnormality presented much later in life (aged 5–52 years) than those with left obstructive lesions (aged <4 months). Two of these patients, aged 154 and 24 years,2 were being evaluated for clinically suspected atrial septal defects. The third patient, aged 5 years, was being evaluated for an asymptomatic murmur.3 Our patient had no cardiac symptoms at the time of her transesophageal echocardiogram.Levoatriocardinal vein is a rare anomaly that can be seen in patients with otherwise normal cardiac anatomy, although it is more common in patients with other left obstructive abnormalities. It should be considered in the differential diagnosis of abnormal vascular structures adjacent to the aorta along with a left superior vena cava and a vertical vein with partial anomalous pulmonary venous return.DisclosuresNone.FootnotesThe online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.111.085555/-/DC1.Correspondence to Ethany L. Cullen, MD, Mayo Clinic, 200 First St. SW, Rochester, MN 55902. E-mail cullen.[email protected]eduReferences1. McIntosh C. Cor biatriutum triloculare. Am Heart J. 1926; 1:735–744.CrossrefGoogle Scholar2. Odemis E, Akdeniz C, Saygili OB, Karaci AR. Levoatriocardinal vein with normal intracardiac anatomy and pulmonary venous return. Ann Pediatr Cardiol. 2011; 4:183–185.CrossrefMedlineGoogle Scholar3. Jaecklin T, Beghetti M, Didier D. Levoatriocardinal vein without cardiac malformation and normal pulmonary venous return. Heart. 2003; 89:1444.CrossrefMedlineGoogle Scholar4. Bernstein HS, Moore P, Stanger P, Silverman NH. The levoatriocardinal vein: morphology and echocardiographic identification of the pulmonary–systemic connection. J Am Coll Cardiol. 1995; 26:995–1001.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Sheth M (2021) Congenital Pulmonary Venous Anomalies CT and MRI in Congenital Heart Diseases, 10.1007/978-981-15-6755-1_11, (237-263), . Tuccillo A, Giordano M, Gaio G, Carrozza M, Cappelli-Bigazzi M, Morelli C, Ait-Ali L, Festa P and Russo M (2021) Trans-catheter closure of a rare cause of pre-tricuspid left-to-right shunt: A "double" levoatriocardinal vein without left heart obstructive lesions, Journal of Cardiology Cases, 10.1016/j.jccase.2020.09.002, 23:2, (65-68), Online publication date: 1-Feb-2021. Mercan I, Akyuz M, Guven B and Isik O (2021) Levoatrial Cardinal Vein: Occluder Embolization and Complication Management, Journal of Chest Surgery, 10.5090/jcs.20.037, 54:3, (214-217), Online publication date: 5-Jun-2021. Yahia Sabri Y, Moawed M, Ahmed Taymour T and Foad Tadros S (2018) Role of MDCT in evaluation of congenital and acquired anomalies of pulmonary venous drainage, The Egyptian Journal of Radiology and Nuclear Medicine, 10.1016/j.ejrnm.2018.01.013, 49:3, (624-630), Online publication date: 1-Sep-2018. Moodley S, Duncan W and Gandhi S (2016) Levoatriocardinal vein in D-transposition of the great arteries, Cardiology in the Young, 10.1017/S1047951116000652, 26:6, (1235-1237), Online publication date: 1-Aug-2016. Marini D, Agnoletti G and Pace Napoleone C (2015) Levoatriocardinal vein and partial anomalous pulmonary vein drainage in left-sided obstructive CHDs: diagnostic and surgical implications, Cardiology in the Young, 10.1017/S1047951115002425, 26:4, (811-814), Online publication date: 1-Apr-2016. Saremi F and Ho S (2016) Extracardiac Pulmonary–Systemic Connection via Persistent Levoatriocardinal Vein in Adults, Annals of Vascular Surgery, 10.1016/j.avsg.2015.12.018, 34, (269.e1-269.e7), Online publication date: 1-Jul-2016. Agarwal P, Mahani M, Lu J and Dorfman A (2015) Levoatriocardinal Vein and Mimics: Spectrum of Imaging Findings, American Journal of Roentgenology, 10.2214/AJR.15.14365, 205:2, (W162-W171), Online publication date: 1-Aug-2015. Maemura S, Ishizuka M, Nakata R, Motozawa Y, Yamamoto K, Takizawa M, Uozumi H and Ikenouchi H (2014) Pulmonary Hypertension Caused by Persistent Anomalous Vertical Vein Bridging the Left Subclavian Vein and Left Atrium With Hypertrophic Cardiomyopathy, Circulation, 10.1161/CIRCULATIONAHA.114.007639, 130:18, Online publication date: 28-Oct-2014. Luciano D, Laux D, Boudjemline Y, Hascoët S, Lusson J, Sorensen C, Ovaert C, Kreitmann B, Van Praagh R and Fraisse A (2013) Transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium, International Journal of Cardiology, 10.1016/j.ijcard.2013.10.061, 170:2, (221-226), Online publication date: 1-Dec-2013. September 18, 2012Vol 126, Issue 12 Advertisement Article InformationMetrics © 2012 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.111.085555PMID: 22988048 Originally publishedSeptember 18, 2012 PDF download Advertisement SubjectsComputerized Tomography (CT)Congenital Heart DiseaseEchocardiography

To describe findings of patients with surgically confirmed pericardial disease on state of the art MR sequences. Retrospective review was performed for patients who underwent pericardiectomy and preoperative MR over a 5 year period ending in 2009. Patients' records were reviewed to confirm the diagnosis of chronic recurrent pericarditis, constrictive pericarditis, or pericardial tumor. MR imaging findings of pericardial thickness, IVC diameter, presence or absence of pericardial or pleural effusion, pericardial edema, pericardial enhancement, and septal "bounce" were recorded. Patients with constriction had a larger IVC diameter (3.1 ± 0.4 cm) than patients with recurrent pain and no constriction (2.0 ± 0.4 cm). Mean pericardial thickness for the 16 patients with chronic recurrent pericarditis but no evidence of constriction was 4.8 ± 2.9 mm. Mean pericardial thickness for patients with constriction was 9.2 ± 7.0 cm with calcification, and 4.6 ± 2.1 cm without calcification. 94% of patients with chronic recurrent pericarditis had gadolinium enhancement of the pericardium, while 76% of patients with constriction had pericardial enhancement. Septal "bounce" was present in 19% of chronic recurrent pericarditis cases and 86% of constriction cases. 5 patients had a pericardial neoplasm, 1 of which was not identified preoperatively. State of the art MR techniques can identify significant and distinct findings in patients with chronic recurrent pericarditis, constrictive pericarditis, and pericardial tumors.

HomeCirculationVol. 124, No. 22Left Main Coronary Artery to Biatrial Fistula Associated With Severe Functional Mitral Valve Regurgitation Free AccessBrief ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessBrief ReportPDF/EPUBLeft Main Coronary Artery to Biatrial Fistula Associated With Severe Functional Mitral Valve Regurgitation Rakesh M. Suri, Alexis K. Okoh, Raul E. Espinosa, Jerome F. Breen, Kent H. Rehfeldt and Rick A. Nishimura Rakesh M. SuriRakesh M. Suri From the Division of Cardiovascular Surgery (R.M.S., A.K.O.), Division of Cardiovascular Diseases (R.E.E., R.A.N.), Department of Radiology (J.F.B.), and Division of Cardiovascular and Thoracic Anesthesia (K.H.R.), Mayo Clinic, Rochester, Minnesota. , Alexis K. OkohAlexis K. Okoh From the Division of Cardiovascular Surgery (R.M.S., A.K.O.), Division of Cardiovascular Diseases (R.E.E., R.A.N.), Department of Radiology (J.F.B.), and Division of Cardiovascular and Thoracic Anesthesia (K.H.R.), Mayo Clinic, Rochester, Minnesota. , Raul E. EspinosaRaul E. Espinosa From the Division of Cardiovascular Surgery (R.M.S., A.K.O.), Division of Cardiovascular Diseases (R.E.E., R.A.N.), Department of Radiology (J.F.B.), and Division of Cardiovascular and Thoracic Anesthesia (K.H.R.), Mayo Clinic, Rochester, Minnesota. , Jerome F. BreenJerome F. Breen From the Division of Cardiovascular Surgery (R.M.S., A.K.O.), Division of Cardiovascular Diseases (R.E.E., R.A.N.), Department of Radiology (J.F.B.), and Division of Cardiovascular and Thoracic Anesthesia (K.H.R.), Mayo Clinic, Rochester, Minnesota. , Kent H. RehfeldtKent H. Rehfeldt From the Division of Cardiovascular Surgery (R.M.S., A.K.O.), Division of Cardiovascular Diseases (R.E.E., R.A.N.), Department of Radiology (J.F.B.), and Division of Cardiovascular and Thoracic Anesthesia (K.H.R.), Mayo Clinic, Rochester, Minnesota. and Rick A. NishimuraRick A. Nishimura From the Division of Cardiovascular Surgery (R.M.S., A.K.O.), Division of Cardiovascular Diseases (R.E.E., R.A.N.), Department of Radiology (J.F.B.), and Division of Cardiovascular and Thoracic Anesthesia (K.H.R.), Mayo Clinic, Rochester, Minnesota. Originally published29 Nov 2011https://doi.org/10.1161/CIRCULATIONAHA.111.032706Circulation. 2011;124:2456–2457A 67-year-old woman had progressive dyspnea, a loud murmur of mitral regurgitation, and a separate, continuous cardiac murmur suggesting shunt. Electrocardiography-gated computed tomography demonstrated a 7-cm aneurysm of the left main coronary artery with possible connection to the right atrium (Figure, A), along with a thin tissue boundary between the aneurysm (Figure, B) and left atrium, raising the possibility of secondary erosion (Figure, C, arrow). Echocardiography revealed severe functional mitral regurgitation, presumably due to volume overload (Figure, D and Movie I in the online-only Data Supplement). Flow was seen between the aneurysm and the left atrium (Figure, E and Movie II in the online-only Data Supplement). Intraoperative evaluation identified a large fistula at the left main coronary artery trifurcation (Figure, F), multiple erosions into the left atrium dome (Figure, G), and a communication to the right atrium (Figure, H) near the superior vena cava–right atrium junction. The origin of the fistula was identified, resected, and patched with bovine pericardium. The mitral valve was repaired, and communications to the left atrium/right atrium were closed. Postoperative transesophageal echocardiography (Figure, I and Movie III in the online-only Data Supplement) and computed tomography (Figure, J) demonstrated the absence of aneurysm and fistulous connections. The patient's postoperative course was uneventful.Download figureDownload PowerPointFigure. A, Electrocardiography-gated computed tomography (CT) showing the aneurysm (*). B, Electrocardiography-gated CT showing a thin tissue rim between the aneurysm (*) and the LA. C, Electrocardiography-gated CT showing a gap (arrow) in the thin tissue rim between the aneurysm (*) and the LA. D, Preoperative transthoracic echocardiogram showing annular dilatation, mitral leaflet malcoaptation, and severe functional mitral valve regurgitation. E, Preoperative transesophageal echocardiogram demonstrating flow between the aneurysm and the LA. F, Intraoperative photograph showing further bifurcation of the left main coronary artery, demonstrated with probes passed down the left anterior descending and circumflex coronary arteries. G, Intraoperative photograph demonstrating erosion of the fistula into the dome of the LA. H, Intraoperative photograph demonstrating orifice of the fistula into the RA near the junction with the superior vena cava. I, Transesophageal echocardiogram showing absence of flow between the aneurysm and the LA after surgical correction. J, Postoperative electrocardiography-gated CT showing repair and absence of aneurysm. * indicates the aneurysm; CT, computed tomography; LA, left atrium; RA, right atrium.DisclosuresThe Division of Cardiovascular Surgery has a research grant entitled "Randomized biological aortic valve replacement" funded by Edwards Lifesciences, St. Jude Medical, and Sorin Group equally. Research funding to the Division within the past year include: AtriCure Inc, Boehringer Ingelheim, Bolton Medical, Carbomedics/Sorin Group, Edwards Lifesciences, Jarvik Heart, LAAX Inc, Medtronic, St. Jude Medical, Schering-Plough, Terumo Heart, The Medicines Company, Thoratec, and WL Gore and Associates. Current technology licensing agreements are with St. Jude Medical and Sorin Group.FootnotesThe online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:/10.1161/CIRCULATIONAHA.111.032706/-/DC1.Correspondence to Rakesh M. Suri, MD, Division of Cardiovascular Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55905. E-mail suri.rakesh@mayo.edu. eLetters(0)eLetters should relate to an article recently published in the journal and are not a forum for providing unpublished data. Comments are reviewed for appropriate use of tone and language. Comments are not peer-reviewed. Acceptable comments are posted to the journal website only. Comments are not published in an issue and are not indexed in PubMed. Comments should be no longer than 500 words and will only be posted online. References are limited to 10. Authors of the article cited in the comment will be invited to reply, as appropriate.Comments and feedback on AHA/ASA Scientific Statements and Guidelines should be directed to the AHA/ASA Manuscript Oversight Committee via its Correspondence page.Sign In to Submit a Response to This Article Previous Back to top Next FiguresReferencesRelatedDetails November 29, 2011Vol 124, Issue 22 Advertisement Article InformationMetrics © 2011 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.111.032706PMID: 22125191 Originally publishedNovember 29, 2011 PDF download Advertisement SubjectsCardiovascular SurgeryEchocardiography

Background-Constrictive pericarditis (CP) is a disabling disease, and usually requires pericardiectomy to relieve heart failure. Reversible CP has been described, but there is no known method to predict the reversibility. Pericardial inflammation may be a marker for reversibility. As a pilot study, we assessed whether cardiac magnetic resonance imaging pericardial late gadolinium enhancement (LGE) and inflammatory biomarkers could predict the reversibility of CP after antiinflammatory therapy.Method and Results-Twenty-nine CP patients received antiinflammatory medications after cardiac magnetic resonance imaging. Fourteen patients had resolution of CP, whereas 15 patients had persistent CP after 13 months of follow-up. Baseline LGE pericardial thickness was greater in the group with reversible CP than in the persistent CP group (4 +/- 1 versus 2 +/- 1 mm, P=0.001). Qualitative intensity of pericardial LGE was moderate or severe in 93% of the group with reversible CP and in 33% of the persistent CP group (P=0.002). Cardiac magnetic resonance imaging LGE pericardial thickness >= 3 mm had 86% sensitivity and 80% specificity to predict CP reversibility. The group with reversible CP also had higher baseline C-reactive protein and erythrocyte sedimentation rate than the persistent CP group (59 +/- 52 versus 12 +/- 14 mg/L, P=0.04 and 49 +/- 25 versus 15 +/- 16 mm/h, P=0.04, respectively). Antiinflammatory therapy was associated with a reduction in C-reactive protein, erythrocyte sedimentation rate, and pericardial LGE in the group with reversible CP but not in the persistent CP group.Conclusions-Reversible CP was associated with pericardial and systemic inflammation. Antiinflammatory therapy was associated with a reduction in pericardial and systemic inflammation and LGE pericardial thickness, with resolution of CP physiology and symptoms. Further studies in a larger number of patients are needed. (Circulation. 2011;124:1830-1837.)

We report a case of lipoma in the right infraclavicular and axillary area compressing subclavian vein there by presenting with upper extremity deep venous thrombosis (UEDVT) and persistent symptoms of venous congestion. Patient was also found to be a heterozygous carrier of prothrombin 20210 gene mutation. Surgical excision of lipomatous tissue performed after 6 months of anticoagulation resulted in a complete resolution of symptoms.

Important features of cardiac masses can be clearly delineated on cardiac computed tomography (CT) imaging. This modality is useful in identifying the presence of a mass, its relationship with cardiac and extracardiac structures, and the features that distinguish one type of mass from another A multimodality approach to the evaluation of cardiac tumors is advocated, with the use of echocardiography, CT imaging and magnetic resonance imaging as appropriately indicated. In this article, various cardiac masses are described, including pseudotumors and true cardiac neoplasms, and the CT imaging findings that may be useful in distinguishing these rare entities are presented.

HomeCirculationVol. 120, No. 1Uncommon Variant of Ebstein Anomaly With Tricuspid Stenosis Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessReview ArticlePDF/EPUBUncommon Variant of Ebstein Anomaly With Tricuspid Stenosis Niloufar Tabatabaei, MD, Poomiporn Katanyuwong, MD, Jerome F. Breen, MD, James Glockner, MD, PhD, Joseph A. Dearani, MD, Rami N. Khouzam, MD and Naser M. Ammash, MD Niloufar TabatabaeiNiloufar Tabatabaei From the Divisions of Cardiovascular Disease (N.T., N.M.A.), Pediatric Cardiology (P.K.), Vascular and Interventional Radiology (J.F.B., J.G.), and Cardiovascular Surgery (J.A.D.), Mayo Clinic, St. Mary's Hospital, Rochester, Minn; and Farmington Heart Center, Farmington, Minn (R.N.K.). , Poomiporn KatanyuwongPoomiporn Katanyuwong From the Divisions of Cardiovascular Disease (N.T., N.M.A.), Pediatric Cardiology (P.K.), Vascular and Interventional Radiology (J.F.B., J.G.), and Cardiovascular Surgery (J.A.D.), Mayo Clinic, St. Mary's Hospital, Rochester, Minn; and Farmington Heart Center, Farmington, Minn (R.N.K.). , Jerome F. BreenJerome F. Breen From the Divisions of Cardiovascular Disease (N.T., N.M.A.), Pediatric Cardiology (P.K.), Vascular and Interventional Radiology (J.F.B., J.G.), and Cardiovascular Surgery (J.A.D.), Mayo Clinic, St. Mary's Hospital, Rochester, Minn; and Farmington Heart Center, Farmington, Minn (R.N.K.). , James GlocknerJames Glockner From the Divisions of Cardiovascular Disease (N.T., N.M.A.), Pediatric Cardiology (P.K.), Vascular and Interventional Radiology (J.F.B., J.G.), and Cardiovascular Surgery (J.A.D.), Mayo Clinic, St. Mary's Hospital, Rochester, Minn; and Farmington Heart Center, Farmington, Minn (R.N.K.). , Joseph A. DearaniJoseph A. Dearani From the Divisions of Cardiovascular Disease (N.T., N.M.A.), Pediatric Cardiology (P.K.), Vascular and Interventional Radiology (J.F.B., J.G.), and Cardiovascular Surgery (J.A.D.), Mayo Clinic, St. Mary's Hospital, Rochester, Minn; and Farmington Heart Center, Farmington, Minn (R.N.K.). , Rami N. KhouzamRami N. Khouzam From the Divisions of Cardiovascular Disease (N.T., N.M.A.), Pediatric Cardiology (P.K.), Vascular and Interventional Radiology (J.F.B., J.G.), and Cardiovascular Surgery (J.A.D.), Mayo Clinic, St. Mary's Hospital, Rochester, Minn; and Farmington Heart Center, Farmington, Minn (R.N.K.). and Naser M. AmmashNaser M. Ammash From the Divisions of Cardiovascular Disease (N.T., N.M.A.), Pediatric Cardiology (P.K.), Vascular and Interventional Radiology (J.F.B., J.G.), and Cardiovascular Surgery (J.A.D.), Mayo Clinic, St. Mary's Hospital, Rochester, Minn; and Farmington Heart Center, Farmington, Minn (R.N.K.). Originally published7 Jul 2009https://doi.org/10.1161/CIRCULATIONAHA.108.835652Circulation. 2009;120:e1–e2A 27-year-old previously healthy woman with no prior history of exercise limitations, shortness of breath, cyanosis, or arrhythmia became short of breath 10 months after her second successful pregnancy. Her shortness of breath was at rest accompanied by progressive edema, ascites, and later anasarca. An ECG revealed normal sinus rhythm with right atrial enlargement (Figure 1A), and chest x-ray revealed normal lung parenchyma with mildly enlarged cardiac silhouette (Figure 1B). A transthoracic echocardiogram demonstrated reduced left ventricular size with an ejection fraction estimated at 50%, severe right atrial enlargement, and severe right ventricular enlargement with dysfunction and was suspicious for severe displacement of the tricuspid valve leaflets toward the right ventricular apex, suggestive of Ebstein anomaly (EA) (Figure 2). She responded to intravenous diuresis with improvement in her right-sided heart failure. In light of her dilated, poorly functioning right ventricle, she was started on Coumadin. Repeat transthoracic echocardiogram 2 months later demonstrated displacement of the tricuspid valve into the right ventricular outflow tract with associated tricuspid stenosis (Figure 3, online-only Data Supplement Movie IA). The spectral Doppler analysis demonstrated a mean gradient of 4 to 5 mm Hg. Cardiac magnetic resonance imaging performed to assess right ventricular function demonstrated tricuspid valve displacement into the right ventricular outflow tract below the pulmonary valve with extensive mural thrombus/mass in the right ventricular apex extending into the right ventricular outflow tract (Figure 4, online-only Data Supplement Movie IIA and IIB). Download figureDownload PowerPointFigure 1. A, ECG showing normal sinus rhythm with right atrial enlargement, without evidence for preexcitation or interventricular conduction delay. B, Chest x-ray: anteroposterior view with moderate enlargement of the cardiac silhouette (cardiothoracic ratio of 60%), normal lung parenchyma, and vasculature.Download figureDownload PowerPointFigure 2. Two-dimensional transthoracic echocardiography. Apical 4-chamber view with displaced tricuspid leaflet not completely visualized with possible apical thrombus formation. RA indicates right atrium; LA, left atrium; TV, tricuspid valve; and LV, leaflet valve.Download figureDownload PowerPointFigure 3. Transthoracic echocardiograph: subcostal view with continuous-wave Doppler across displaced tricuspid valve showing diastolic flow consistent with tricuspid valve stenosis.Download figureDownload PowerPointFigure 4. A, Reformatted image from ECG-gated contrast-enhanced magnetic resonance angiography showing atrialized right ventricle (aRV), tricuspid valve (TV) displaced into the right ventricle (RV), and pulmonary outflow with thrombus (*) in the base of the apex. B, Myocardial delayed-enhancement magnetic resonance image showing short-axis image of the atrialized right ventricle with thrombus (*) and the left ventricle (LV).In light of the right-sided heart failure and the findings of EA with tricuspid stenosis, surgical repair was recommended. Intraoperative transesophageal echocardiography confirmed the displacement of the tricuspid valve into the right ventricular outflow tract with associated tricuspid stenosis as noted by color flow Doppler (online-only Data Supplement Movies IIIA and IIIB). The pulmonary and aortic valves were normal. Surgical repair consisted of resection of the stenotic anterior tricuspid leaflet in the right ventricular outflow and removal of a large amount of organized thrombus from the right ventricle (Figure 5A and 5B). A porcine bioprosthetic tricuspid valve was placed, and a right atrial reduction was performed. Furthermore, a bidirectional Glenn shunt was placed to offload the right ventricle. The patient did well postoperatively with resolution of symptoms. Download figureDownload PowerPointFigure 5. A, Surgical view of the tricuspid valve (TV) stenosis. B, Surgical view of thrombus (*) in the atrialized right ventricle and outflow tract.EA is a rare congenital anomaly of the tricuspid valve frequently associated with tricuspid regurgitation, right ventricular dysplasia, and, less frequently, atrial septal defect/patent foramen ovale, Wolf-Parkinson-White syndrome, and pulmonary valve stenosis.1 Patients with EA can present at different ages, depending on its structural and functional severity and associated defects.2 This case represents a rare form of EA with tricuspid stenosis very well tolerated into adulthood until the patient's second pregnancy, which we believe played a role in her subsequent cardiac decompensation. Pregnancy is a known hypercoagulable state that could have precipitated thrombus formation in the relatively akinetic segment of the atrialized portion of the right ventricle, leading to obstruction of the right ventricular outflow tract near the stenotic tricuspid valve and ultimately causing low cardiac output, severe dyspnea, and right-sided heart failure.Transthoracic echocardiography is the initial diagnostic procedure of choice in the assessment of the structural and functional severity of EA and its associated defects. At times, however, a transesophageal echocardiogram is needed to assess for the presence of an atrial shunt. In addition, cardiac magnetic resonance imaging is often used to assess right ventricular size and function, given some of the limitations of echocardiography. The findings on echocardiography and magnetic resonance imaging have a significant impact on the surgical repair and outcome of patients with EA.3The online-only Data Supplement is available with this article at http://circ.ahajournals.org/cgi/content/full/120/1/e1/DC1.AcknowledgmentsWe gratefully acknowledge Mark A. Zangs and Timothy B. Seelinger for their expert technical assistance.DisclosuresNone.FootnotesCorrespondence to Nasser M. Ammash, MD, FACC, Associate Professor of Medicine, Mayo Clinic, St. Mary's Hospital, 2nd St SW GO-138SE, Rochester, MN 55902. E-mail [email protected]References1 Attenhofer Jost CH, Connolly HM, Edwards WD, Hayes D, Warnes CA, Danielson GK. Ebstein's anomaly: review of a multifaceted congenital cardiac condition. Swiss Med Wkly. 2005; 135: 269–281.MedlineGoogle Scholar2 Connolly HM, Warnes CA. Ebstein's anomaly: outcome of pregnancy. J Am Coll Cardiol. 1994; 23: 1194–1198.CrossrefMedlineGoogle Scholar3 Donnelly JE, Brown JM, Radford DJ. Pregnancy outcome and Ebstein's anomaly. Br Heart J. 1991; 66: 368–371.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Khajali Z, Rezaeian N and Ansari Z (2022) Displacement of all three leaflets of tricuspid valve: A rare variant of Ebstein anomaly, Clinical Case Reports, 10.1002/ccr3.5555, 10:3, Online publication date: 1-Mar-2022. Pérez-Riera A, Barbosa-Barros R, Daminello-Raimundo R, de Abreu L and Nikus K The Vectorcardiogram and the Main Dromotropic Disturbances, Current Cardiology Reviews, 10.2174/1573403X16666200810105504, 17:1, (50-59) Holst K, Ammash N and Dearani J (2018) Ebstein Anomaly Diagnosis and Management of Adult Congenital Heart Disease, 10.1016/B978-0-7020-6929-1.00043-5, (442-449), . Perloff J and Marelli A (2012) Ebstein's Anomaly of the Tricuspid Valve Clinical Recognition of Congenital Heart Disease, 10.1016/B978-1-4377-1618-4.00013-4, (176-195), . Khouzam R, Dearani J and Julsrud P (2010) Ebstein's malformation presenting with tricuspid stenosis: 1-year follow-up after surgical repair, Cardiology in the Young, 10.1017/S104795111000003X, 20:1, (80-85), Online publication date: 1-Feb-2010. July 7, 2009Vol 120, Issue 1 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.108.835652PMID: 19581515 Originally publishedJuly 7, 2009 PDF download Advertisement SubjectsComputerized Tomography (CT)Congenital Heart DiseaseEchocardiography