Eur Heart J Cardiovasc Imaging Abstracts Supplement, December 2014 doi:10.1093/ehjci/jeu232

CASE-BASED SESSION: UNUSUAL CASES IN CLINICAL PRACTICE

Wednesday 3 December 2014, 09:00–10:30 Location: Agora

Mucopolysaccharidosis type IV B-diagnostic and perioperative implication of Morquio syndrome B G. Dostalova1; Z. Hlubocka1; K. Ravlykova1; V. Rohn1; J. Zeman2; T. Palecek1; A. Linhart1 1 2nd Medical Department - Clinical Department of Cardiology and Angiology of the First Faculty of Med, Prague, Czech Republic; 2General Teaching Hospital, Prague, Czech Republic Mucopolysaccharidosis type IV B (MPS IV B), also known as Morquio syndrome, is a rare inherited disease from a group of lysosomal storage disorders. Estimates of birth prevalence range from less than 1/40,000 to 1/200,000 births. MPS IV B occurs because of a deficiency of the enzyme beta-galactosidase. A deficiency of this enzyme leads to the accumulation of mucopolysaccharides in the whole body with e.g. growth retardation, a prominent lower face, an abnormally short neck, kyphoscoliosis, abnormal and/or a prominent breast bone (pectus carinatum). Also cardiomegaly may also occur. The accumulation in cardiomyocytes causes progressive damage to cells, tissues, and failure of their function. Cardiac manifestation could cause a premature death of the patient. Our clinical case report shows a 60-year old women with symptomatic aortic valve stenosis and severe hypertrophy of the left ventricle. From the childhood there raised suspicion of lysosomal storage disorder because of her clinical features. She had signs of enzymatic storage disorders (as growth retardation with height 140 cm in adulthood, abnormally short neck, kyphoscoliosis, abnormalities of foot and legs, pectus carinatum). The diagnosis of MPS IV, type B in our patient was confirmed by enzyme and genetic screening. The patient suffered from mild dyspnea (NYHA class II), patient’s sister, also with MPS IV, died from sudden cardiac death at her 60. By echocardiographic examination there was significant aortic valve stenosis, with progression during 2 years follow up (AVA 0,67cm2, AVAi 0,45 cm2/m2,PG mean 75 mmHg) and asymetric septal hypertrophy with mild left ventricle outflow tract obstruction at rest. There was no coronary artery disease. MRI of cervical spine showed severe C2/C3 spinal stenosis with special need of perioperative anaesthesiologic care. Aortic valve replacement and septal myectomy were performed without any complications, also postoperative period is so far without any complications. The patient is free of symptoms. Histological examination of the aortic valve tissue and myocytes confirmed also lysosomal storage. Conclusions: Mucopolysaccharidosis type IV B with this combination of cardiac manifestation (severe valve disease and septal hypertrophy with LVOT obstruction) has not been published yet. Cardiologic and echocardiographic assessment should be completed at the time of diagnosis and according to the clinical course at least every 1–2 years thereafter by patients with MPS IV. Cardiac surgery with very careful perioperative monitoring seems to be safe and effective.

32 Three dimensional transesophageal echocardiography in the assessment of left ventricular pseudoaneurysm secondary to mitral valve endocarditis B. Bochard Villanueva1; O. Fabregat-Andres1; R. De La Espriella-Juan1; A. Cubillos-Arango1; M. Ferrando-Beltran1; N. Chacon-Hernandez1; J. Estornell-Erill2; JL. Perez-Bosca1; S. Morell-Cabedo1; R. Paya-Serrano1 1 University General Hospital of Valencia, Department of Cardiology, Valencia, Spain; 2 University General Hospital of Valencia, ERESA, Valencia, Spain A 76 -year-old woman was admitted at the infectious diseases unit of our hospital because of four days remittent fever of unknown origin. She had a history of obesity, hypertension and type 2 diabetes mellitus. Physical examination and chest radiograph were normal. Laboratory tests showed leukocytosis with neutrophilia, C-reactive protein 23 mg/dl and blood cultures were positive for penicillin-sensitive Streptococcus equi. Bi-dimensional and three-dimensional transesophageal echocardiography (2D and 3D TEE) showed a

mobile mass of 5 mm in the posterior mitral commissure and diagnosis of infective endocarditis (IE) was established. The clinical course was favorable with intravenous ceftriaxone and the patient was discharged home within fourteen days to continue intravenous antimicrobial treatment for four weeks. Subsequently, follow-up 2D and 3D TEE showed a pulsatile perivalvular echo-free space of 32 × 23 mm with a narrow orifice communicating to the left ventricle at posterior mitral subannular position consistent with pseudoaneurysm. The real-time three-dimensional TEE allowed us to see its relationship with neighboring structures (Figure). No impaired function of mitral valve was observed. As part of preoperative study, coronary computed tomography angiogram was performed confirming these findings and showed no significant coronary stenosis. With this, surgery was indicated and a bovine pericardium circular patch of 2 × 2 cm with stitches and continuous sutures was implanted and reinforced with a second patch with BioGluew. The immediate postoperative period was uneventful being discharged within ten days.

Abstract 32 Figure.

33 Real time 3D transesophageal echocardiographic guidance of mechanical mitral valve perivalvular leak closure with ventricular septal defect occluder device from a transapical approach: a case report A. Mediratta; E. Retzer; J. Decara; L. Weinert; AP. Shah; R M. Lang The University of Chicago Medical Center, Chicago, United States of America Perivalvular leak (PVL) is a complication that affects 1%-20% of heart valve surgeries. Small PVL can be asymptomatic while larger defects can cause hemolytic anemia, heart failure or bacteremia. Surgery for PVL has a high morbidity and mortality; however, a percutaneous approach can be used to occlude defects with high surgical risks. Real-time imaging is critical for intraprocedural guidance. 72 year old with rheumatic heart disease with mechanical mitral and aortic valve replacement presenting with anemia and congestive heart failure. Cardiac exam revealed an irregularly irregular rate and rhythm, a holosystolic murmur at the apex radiating to the back with elevated JVP. Laboratory data was consistent with hemolytic anemia. TTE showed eccentric mitral valve regurgitation suspicious for PVL, TEE showed PVL (panel A) from partial dehiscence of mechanical mitral valve ring at the 9 o’clock position. Due to the vicinity of the defect and the mechanical aortic valve, a transapical approach was chosen over either a trans-septal or aortic approach. 3D TEE acquisition of mitral valve allowed localization of PV defect and assisted in guiding the wire thru the PVL (panel B). After crossing with a wire, a 6 mm VSD occluder device was placed in the defect (panel C). TEE showed valve malfunction of the mechanical AV evidenced by an increased gradient. Fluoroscopy confirmed entrapment of leaflet with PVL occluder. The device was removed and exchanged for a 4 mm VSD occluder device which was successfully deployed. Finally, apical views from TTE guided closure of the transapical access site using a muscular VSD occluder.

Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2014. For permissions please email: [email protected]

Downloaded from http://ehjcimaging.oxfordjournals.org/ by guest on June 6, 2016

31

ii2

Abstracts

Multi-modality imaging can provide useful complementary real-time data for successful guidance of a percutaneous repair of mechanical mitral valve PVL.

Abstract 33 Figure.

hospital stay he had empyema and underwent decortication of empyema with open thoracotomy. On admission the patient was asymptomatic, blood pressure was 100/70 mmHg, heart rate was 86 beats/min, S1 and S2 were rhythmic, S3 and S4 were absent, apical III/VI pansystolic and mesocardiac II/VI pansystolic murmurs were heard on cardiac examination. Transthoracic echocardiogram revealed severe left ventricular systolic dysfunction, left ventricular aneurysm, dense echo-contrast in left ventricle, biatrial and left ventricular dilatation and moderate tricuspid regurgitation, EF was 24%. In summary, this is a patient in class III NYHA secondary to ischemic cardiomyopathy– currently hospitalized on maximal medical therapy, EF ,30% and LVESVI .120 ml/m2 (Picture) which are poor prognostic factors for aneurysmectomy operation. Favorable factors were the patients age and asymptomatic state. He finally underwent surgical anterior ventricular restoration (SAVER) but did not survive and died during the operation.

36 Right sided emboli in transit - a catch-22 situation T. Felice; M. Mercieca Balbi; K. Yamagata; H. Felice Mater Dei Hospital, Msida, Malta Introduction: Pulmonary emboli are a common cause of cardiac arrest, however free floating right heart thrombi are rarely noted on transthoracic echocardiography (TTE). Case Presentation Summary: Our case revolves around a 76 year old woman, previously healthy, who presented for investigation of epigastric pain. An oesophagogastroscopy

35 Unusual history of a patient with subsequent giant left ventricular aneurisms IA. Altun1; GG. Guz2; FA. Akin1; NK. Kose1; IA. Ilknur Altun3 1 Mugla Sitki Kocman University, Faculty of Medicine, Cardiology, Mugla, Turkey; 2istanbul university, faculty of medicine, department of cardiology, istanbul, Turkey; 3Mugla Sitki Kocman Training and Research Hospital, Radiology, Mugla, Turkey A 65-year-old male patient referred to our clinic after revealing left ventricular aneurysm on transthoracic echocardiogram. His past medical history is significant for myocardial infarction. 2 years ago coronary revascularization (left internal mammary artery to left anterior descending artery, and two saphenous vein grafts from the aorta to an obtuse marginal and right coronary artery, respectively) and ventricular septal defect (VSD) repair operation have been performed. After 2 months from this procedure he had a fever complaints and operated with diagnosis of mediastinitis. 9 months later after the last procedure left ventricular inferoposterior aneurysm and thrombus revealed on transthoracic echocardiogram. Posterior aneurysmectomy and thrombectomi was performed. . During the

Eur Heart J Cardiovasc Imaging Abstracts Supplement, December 2014

Abstract 36 Figure. Large right atrial thrombus

Downloaded from http://ehjcimaging.oxfordjournals.org/ by guest on June 6, 2016

Abstract 35 Figure. Cardiac MRI

Abstracts (OGD) showed the presence of a duodenal ulcer with no active bleeding. During the OGD the patient had a run of multifocal atrial tachycardia and was referred for cardiology admission. During her admission, the patient had an episode of sudden onset narrow complex tachycardia followed by collapse. On examination the patient was unresponsive, cyanotic with an unrecordable blood pressure. No evidence of DVT was present. A short cycle of chest compressions was performed. Return of spontaneous circulation was achieved after a few minutes, however the patient remained haemodynamically unstable. Inotropes were commenced and a bed-side TTE was performed which showed biventricular dilatation and severe impairment, flattening of the intraventricular septum, and large floating

ii3

thrombi in the right atrium prolapsing across the tricuspid valve. A CT pulmonary angiogram confirmed the presence of pulmonary embolism. Thrombolytic therapy was contraindicated in view of her duodenal ulcer. The patient was started on unfractionated heparin, and an intravenous proton-pump inhibitor. Insertion of an inferior vena caval filter was contraindicated in view of possible thrombus dislodgement and further pulmonary embolism. After 48 hours of heparinisation, the patient remained stable and a repeat TTE showed resolution of the right atrial thrombi. Conclusion: Our case presents a complex and challenging situation of cardiopulmonary arrest, emphasizing the importance of TTE in acute care.

Downloaded from http://ehjcimaging.oxfordjournals.org/ by guest on June 6, 2016

Eur Heart J Cardiovasc Imaging Abstracts Supplement, December 2014

The Eighteenth Annual Meeting of the European Association of Echocardiography, December 3-6, 2014, Vienna, Austria.

The Eighteenth Annual Meeting of the European Association of Echocardiography, December 3-6, 2014, Vienna, Austria. - PDF Download Free
192KB Sizes 0 Downloads 9 Views