Double-Outlet Left Ventricle

Magnetic resonance imaging data of a 20-year-old male patient was used to produce, to the best of our knowledge, the first cast model of a beating heart. Imaging was performed at the German Heart Institute Berlin, Department of Congenital Heart Disease and Pediatric Cardiology; data were transferred to the Department of Medical and Biological Informatics (German Cancer Research Center, Heidelberg). The patient had the diagnoses of dextrocardia, atrial and visceral situs inversus, L-looped ventricles, and L-malposition of the great arteries, with a large perimembranous ventricular septal defect and pulmonary atresia. A 3D whole-heart, navigator-corrected MRI data set …

A 4-month-old girl was referred for cyanosis and echocardiographic suspicion of Ebstein anomaly. Medical history had been uneventful thus far. At clinical examination, she was restless and cyanosed but did not show respiratory distress, cardiac failure, or failure to thrive. Cardiac and lung auscultation were normal except for tachycardia (150 to 180 bpm). Femoral pulses were present. Oxygen saturation on pulse oximetry oscillated between 55% and 70%. ECG showed abnormal right atrial hypertrophy (Figure 1). Chest x-ray showed no cardiomegaly but reduced pulmonary blood flow (Figure 2). Repeat echocardiography showed normal situs and connections. There was a large patent foramen ovale with mainly right to left shunt. The tricuspid valve annulus size was normal (13.1 mm; Z score=+0.2). First images suggested a restrictive opening of the tricuspid valve, which appeared shifted toward the apex, as seen in Ebstein disease (Figure 3). There was, however, hardly any tricuspid valve regurgitation. Close analysis showed normal insertions of the leaflets, precluding the diagnosis of Ebstein disease. Slow-motion analysis showed during diastole a very large and thick membrane arising from the right atrium, protruding toward the right …

A 72-year-old man was referred because of suspected infective endocarditis. Fifteen years earlier, he had undergone mitral valve replacement with a 33-mm Hancock valve for severe mitral regurgitation. Three months ago, he presented with a first episode of congestive heart failure caused by severe mitral regurgitation reflecting degeneration of the bioprosthetic valve without sign of endocarditis. Because of a high surgical risk based on an estimated Euroscore I of 42.96% and The Society of Thoracic Surgeons (STS) score of 29% (main comorbidity is an advanced Parkinson disease), valve-in-valve implantation was planned. A 29-mm Edwards Sapien 3 (S3) balloon-expandable valve was successfully implanted through transapical puncture of the left ventricle (LV) after direct surgical exposure by mini thoracotomy. Patient was discharged 10 days after the procedure without complications. On his admission, the patient was febrile and examination revealed a purulent discharge at the site of thoracotomy. Transthoracic echocardiography (TTE) and transesophageal echocardiography showed a 15-mm mobile vegetation on the leaflet of the S3 (Movie I in the Data Supplement) and a pseudoaneurysm at the apex of the left ventricle (LVPA) flowing by a large apical defect (Figure 1; Movie II in the Data Supplement). Computed tomographic (CT) scanning confirmed the LVPA free from any thrombus and identified the surgical suture tip in its deep (Figures 1 and 2; Movie III in the Data Supplement). 18F positron-emission tomography demonstrated an uptake on the S3 and LVPA (Figure 2). Blood cultures were positive for Staphylococcus aureus . Antibiotherapy with co-trimoxazole for 6 weeks and clindamycin for 1 week was started, and a quick clinical and biological …

A 19-year-old man with no medical history was involved in a motorcycle accident with chest trauma and rapid deceleration. No injury was apparent initially, but 3 weeks later, he developed dyspnea and was referred to our echocardiography unit for assessment of a new 4/6 systolic heart murmur. 2D echocardiography (Figure 1) and cardiac magnetic resonance (CMR) …

A2.8-kg newborn girl was referred to our hospital for tetralogy of Fallot with a right aortic arch. ECG failed to provide a clear anatomic definition of the pulmonary blood supply. Cardiac catheterization disclosed a right aortic arch giving rise to 4 separate branches in the following sequence: right common carotid artery, right vertebral artery, right subclavian artery, and aberrant left subclavian artery (Figure 1). An isolated left common carotid artery, draining into the left pulmonary artery, was visualized by retrograde filling after selective injection into the right common carotid artery (Figure 2). Such a rare anomaly creates a left-to-right shunt at the arterial level, which becomes more significant when the pulmonary artery pressure lowers because of spontaneous or surgical right ductal closure. Isolation of the common carotid artery occurs only when the homolateral subclavian artery is aberrant so that the innominate artery cannot be formed. A bilateral patent ductus arteriosus connected the aortic isthmus to the right pulmonary artery and the isolated vessel to the left pulmonary artery. The main pulmonary artery and its branches were confirmed to be moderately and diffusely hypoplastic. Anomalous origin of the left anterior descending coronary artery from the right coronary artery was visualized. Via median sternotomy (Figure 3), the baby underwent aortic arch implantation of the isolated left carotid artery and 3-mm polytetrafluoroethylene systemic-to-pulmonary shunt interposition between the ascending aorta and the left pulmonary artery (Figure 4). Complete repair was deferred because of the coronary anomaly and the small size of the pulmonary arteries. The thymic gland was absent, thus supporting the clinical findings typical of DiGeorge syndrome. The patient survived the operation and is currently awaiting complete repair.

Complete ectopia cordis is a very rare condition. With few exceptions, it is uniformly considered fatal. Death usually occurs within the first days of life as a result of infection, cardiac failure, or hypoxemia. We report the case of a male infant with Cantrell’s syndrome. There was complete thoracic exteriorization of the heart, with cephalic orientation of the cardiac apex (Figure 1). The cardiac surface was covered by …

A 42-year-old man experiencing nonischemic cardiomyopathy with severely reduced left ventricular function and advanced heart failure met the criteria for primary prophylactic implantation of an implantable cardioverter-defibrillator (ICD). A subcutaneous ICD (S-ICD; EMBLEM; Boston Scientific, Marlborough, MA) was implanted at a secondary center in November 2015 with the lead (3401; Boston Scientific) tunnelled in left parasternal position. Before implantation, surface ECG screening was performed following the manufacturer’s instructions. A few weeks later, the patient was transferred to Hannover Medical School because of heart failure deterioration (New York Heart Association class IV). After careful evaluation, a continuous-flow left ventricular assist device (LVAD; HeartMate 3; Thoratec, Pleasanton, CA) was implanted using conventional sternotomy.1 Approximately 1 hour after LVAD implantation, the patient received 31 S-ICD shocks. The device was immediately deactivated. Interrogation of the S-ICD revealed normal sinus rhythm during the shocks. However, R waves were diminished and superimposed by electric …

HomeCirculationVol. 108, No. 6Concomitant T-Wave Alternans and Pulsus Alternans in a Child With Long-QT Syndrome Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessReview ArticlePDF/EPUBConcomitant T-Wave Alternans and Pulsus Alternans in a Child With Long-QT Syndrome Bert Suys, MD, Rudi De Paep, MD, Katrien François, MD and Daniel De Wolf, MD, PhD Bert SuysBert Suys From the Departments of Pediatric Cardiology (B.S., D.D.W.), Intensive Care (R.D.P.), and Cardiac Surgery (K.F.), University Hospitals, Antwerp and Ghent, Belgium. Search for more papers by this author , Rudi De PaepRudi De Paep From the Departments of Pediatric Cardiology (B.S., D.D.W.), Intensive Care (R.D.P.), and Cardiac Surgery (K.F.), University Hospitals, Antwerp and Ghent, Belgium. Search for more papers by this author , Katrien FrançoisKatrien François From the Departments of Pediatric Cardiology (B.S., D.D.W.), Intensive Care (R.D.P.), and Cardiac Surgery (K.F.), University Hospitals, Antwerp and Ghent, Belgium. Search for more papers by this author and Daniel De WolfDaniel De Wolf From the Departments of Pediatric Cardiology (B.S., D.D.W.), Intensive Care (R.D.P.), and Cardiac Surgery (K.F.), University Hospitals, Antwerp and Ghent, Belgium. Search for more papers by this author Originally published12 Aug 2003https://doi.org/10.1161/01.CIR.0000079312.63225.AECirculation. 2003;108:e36A13-month-old girl was hospitalized for cardiogenic shock and convulsions after a few days of fever and malaise. She was known to have long-QT syndrome and titers for adenovirus. Her myocardial function was poor, and myocarditis was suspected. ECG monitoring showed extremely long QT and QTc with T-wave alternans. A simultaneous arterial pressure curve showed pulsus alternans (Figure 1). The occurrence of concomitant electrical alternans and mechanical alternans is extremely rare; the former is related to action potential duration changes and is a marker for electrical instability, and the latter is explained by hemodynamic alterations. Gradually, a pseudo 2:1 AV block developed (extreme prolongation of ventricular refractoriness), with bradycardia and hypotension (Figure 2). After 10 days of ventilation and massive inotropic support, she was extubated and recovered slowly. Before leaving the hospital, the patient had a pacemaker-defibrillator with epipericardial patch surgically implanted (Figure 3), and β-blockade reinstalled. Download figureDownload PowerPointFigure 1. Prolonged QT and QTc intervals, T wave alternans, and pulsus alternans.Download figureDownload PowerPointFigure 2. Pseudo 2:1 AV block with hypotension.Download figureDownload PowerPointFigure 3. Chest x-ray study showing pacemaker–internal cardioverter-defibrillator with epipericardial patch; the battery was implanted in the perirenal space.FootnotesCorrespondence to Bert E. Suys, Congenital and Pediatric Cardiology, University Hospital Antwerp, Wilrijkstraat 10, 2650 Edegem, Belgium. E-mail [email protected] Previous Back to top Next FiguresReferencesRelatedDetailsCited By Deogire A and Hamde S (2015) A collaborative approach to improve TWA detection 2015 International Conference on Industrial Instrumentation and Control (ICIC), 10.1109/IIC.2015.7150747, 978-1-4799-7165-7, (247-251) Xie Y, Hu G, Sato D, Weiss J, Garfinkel A and Qu Z (2007) Dispersion of Refractoriness and Induction of Reentry due to Chaos Synchronization in a Model of Cardiac Tissue, Physical Review Letters, 10.1103/PhysRevLett.99.118101, 99:11 Lansky A, Costa R, Mintz G, Tsuchiya Y, Midei M, Cox D, O’Shaughnessy C, Applegate R, Cannon L, Mooney M, Farah A, Tannenbaum M, Yakubov S, Kereiakes D, Wong S, Kaplan B, Cristea E, Stone G, Leon M, Knopf W and O’Neill W (2004) Non–Polymer-Based Paclitaxel-Coated Coronary Stents for the Treatment of Patients With De Novo Coronary Lesions, Circulation, 109:16, (1948-1954), Online publication date: 27-Apr-2004. August 12, 2003Vol 108, Issue 6 Advertisement Article InformationMetrics https://doi.org/10.1161/01.CIR.0000079312.63225.AEPMID: 12912796 Originally publishedAugust 12, 2003 PDF download Advertisement

Unrepairable Infant Mitral Valve: An Unexpected Case of Decompensated Heart Failure.

A previously healthy 39-year-old man was admitted to our department with biventricular cardiac decompensation (New York Heart Association class IV) and acute onset of angina pectoris after a flulike disease 1 week earlier. The ECG on admission displayed ST elevations in II, III, and aVF. Troponin T on admission was increased (2.20 mg/L, normal value<0.03 mg/L), with increased creatine phosphokinase (503 U/L; normal value<171 U/L) and creatine phosphokinase myocardial band (45 U/L; normal value=10 U/L). N-terminal pro-brain natriuretic peptide was highly elevated (15,848 pg/mL; normal value<125 pg/mL). Coronary artery disease was excluded by coronary angiography. Multiple nonsustained ventricular tachycardias were recorded by Holter monitoring and telemetry. Endomyocardial biopsies obtained from the right ventricular septum demonstrated active myocarditis by histological investigations according to the Dallas criteria (Figure 1A). By immunohistological staining of endomyocardial biopsies1 and quantification using digital image analysis, highly increased focal lymphocytic (CD3+: 267.4/mm2, CD11a/LFA-1+: 498.9/mm2; Figure 1B) and macrophage infiltrates (CD11b/Mac-1+: 481.6/mm2), sarcolemmal human leukocyte antigen class I expression (area fraction of human leukocyte antigen class I=35.6%; Figure 1C), and focal abundance …

Double-outlet right ventricle falls under the category of congenital heart disease known as conotruncal defects, which possess abnormal ventriculoarterial relationships.1 For complex cases, the surgeon must determine whether the left ventricle and one of the great arteries can be aligned using the ventricular septal defect to construct an unobstructed pathway or baffle, resulting in a 2-ventricle repair.2 Creation of the baffle can be complicated by anatomic obstructions because of prominent conal septum, straddling atrioventricular valve attachments, or location of the ventricular septal defect in the inlet septum, remote from any great artery. Three-dimensional (3D) printing has been applied in the management of many different congenital heart diseases.3 In this specific patient population, in whom communicating the complex intracardiac anatomy to the surgeon is so critical, the use of 3D modeling and printing is invaluable. We used this approach in a patient with dextrocardia, complex double-outlet right ventricle (S,L,A)1 and supratricuspid ring. She underwent pulmonary artery banding in infancy and had been doing relatively well clinically; so that any further surgical intervention was deferred until she was 8 years old. Although she was growing well and required no medication, …

An 80-year-old woman was admitted to our institution because of shortness of breath (New York Heart Association grade III) and stable angina pectoris (Canadian Cardiovascular Society [CCS] grade II). Two years ago, the patient had a posterior wall infarction successfully treated by percutaneous coronary intervention and stent implantation. A low-gradient aortic stenosis with an aortic valve area of 1.1 cm2 was also diagnosed but was considered to be clinically insignificant at the time. Because of the progression of symptoms within the last 2 years, a re-evaluation of coronary and aortic valve disease was performed. Coronary angiography revealed a 1-vessel coronary disease with a patent stent in the right coronary artery without any evidence of restenosis. The left ventricular ejection fraction was only moderately impaired (55%). However, the aortic valve area, as determined invasively, had decreased to 0.5 cm2. This was confirmed by echocardiography; the cusps of the aortic valve were calcified and their motion was impaired (Supplemental Data Movie 1). On the basis of these findings, the necessity of an aortic valve replacement was discussed. However, because the patient was anxious and refused conventional aortic valve …

A 63-year-old white man with a 6-month history of progressive exertional dyspnea was referred for evaluation. In 1997, he presented an episode of unconsciousness as first symptom of a cardiac disease. In 2003, arterial hypertension, as well as atrioventricular block Mobitz type I, was diagnosed. A worsening of the biventricular heart failure over the last 2 years led to his admission at our clinic in 2008, with dyspnea at rest and bilateral pleural effusions. At admission, the patient appeared to be well, with a blood pressure of 142/76 mm Hg and a pulse of 97 bpm. Jugular venous pulse was not elevated, but mild bilateral edema of the lower extremities was noted. The lungs were clear to auscultation, with attenuation on the right side because of pleural effusion. The patient’s symptoms improved slightly through pharmacological therapy, but he remained in New York Heart Association functional class III heart failure. Laboratory studies revealed normal blood cell counts and electrolyte panel, with signs of load on the right side of the heart. The highest B-type natriuretic peptide was 818.6 pg/mL (normal, <100 pg/mL). The 24-hour urine protein collection was outside of normal limits at 0.27 g (normal, <0.15 g), and glomerular filtration rate according to the modified …

A 42-year-old man without a past cardiovascular history arrived in the emergency department reporting progressive chest pain, abdominal discomfort, dyspnea at rest, and orthopnea of 4 days duration. An abdominal ultrasound revealed significant pericardial and bilateral pleural effusion, with hepatomegaly and ascites of a probable congestive nature. A transthoracic echocardiogram showed severe pericardial effusion with data of hemodynamic compromise, and a right retroauricular mass anchored to the posterior atrial wall (Figures 1A and 2; Movies I and II in the online-only Data Supplement). Figure 1. A , Apical 4-chamber view transthoracic echocardiogram showing concentric severe pericardial effusion (PE), right (RV) and left (LV) ventricle, and tumor (arrow). B , CT scan showing tumor mass (arrow) attached to the right atrium (RA). C , Apical 4-chamber view with color flow Doppler demonstrating in the right chambers flow acceleration (arrow) secondary to extrinsic tumor compression. D , CT scan …

Ahealthy 30-year-old man who had not had any prior medical or surgical history presented to the emergency department with substernal pain for 2 days that was sharp and exacerbated by inspiration and recumbency. Chest radiograph (Figure 1A) showed mild left atrial enlargement. The ECG showed normal findings. The next day, the patient complained of sudden onset of class 3 dyspnea and frothy blood-tinged sputum. Follow-up …