Lacerda Junior, Orly O

Machado, Renato A

Marchiori, Edson

Mello, Ricardo A F

Nacif, Marcelo Souto

Sibley, Christophe T

English

PubMed

Mello, Ricardo A. F.

Lacerda Junior, Orly O.

Sibley, Christophe T.

Machado, Renato A.

Cadernos Espinosanos (E-Journal)

LETTER TO THE EDITORDouble-chambered left ventricle in an adult:diagnosis by CMRIMarcelo Souto Nacif,I,II Ricardo A. F. Mello,III Orly O. Lacerda Junior,IV Christophe T. Sibley,IRenato A. Machado,V Edson MarchioriVIINational Institutes of Health - Radiology - Clinical Center, Bethesda, Maryland, United States. II Johns Hopkins School of Medicine - Cardiology, Baltimore,Maryland, United States. IIIUniversidade Federal do Espı´rito, Vito´ria, Espirito Santo, Brazil. IVUNIMED (CIAS) – Cardiology, Vito´ria, Espirito Santo, Brazil.VUNIMED (CIAS) – Radiology, Vito´ria, Espirito Santo, Brazil. VIUniversidade Federal do Rio de Janeiro – Radiology, Rio de Janeiro, Rio de Janeiro, Brazil.Email: msnacif@gmail.comSite: www.msnacif.med.brINTRODUCTIONOutpouching of the left ventricle is a rare condition withheterogeneous causes ranging from congenital abnormal-ities, such as diverticula or muscle bands, to complicationssecondary to myocardial infarction, such as aneurysm andpseudoaneurysm. Distinguishing among these etiologies ischallenging but of great clinical importance given the widerange of risks and implications involved.1–3Double chambered is a term that has been used todescribe the subdivision of a ventricle as a result of ano-malous septum or muscle bundle. Subdivision of the leftventricular cavity is a rare cardiac anomaly compared withsubdivision of the right ventricle.3Cardiac magnetic resonance imaging (CMRI) is useful inthe assessment of associated conditions and to better under-stand the disease, characterizing the diagnosis. CMRI candemonstrate changes in the ventricular contractility, pre-sence or absence of fibrosis and is useful in the follow-up ofpatients with double-chambered left ventricle (DCLV).3,4We describe a case of DCLV, highlighting the importanceof CMRI, clinical evolution, and outcome.CASE PRESENTATIONA 29-year-old sedentary man presented to the emergencyroom an hour after developing chest pain that radiated tothe inner side of the left arm. The patient was administeredacetylsalicylic acid and nitrate and symptoms soonimproved. The patient had no family history of coronaryartery disease (CAD), had smoked a pack of cigarettes a dayfor the past 10 years, and was obese and hypertensive (140/95 mmHg). Physical examination revealed no cardiacmurmur and the electrocardiogram (EKG) was normal,but plasma cardiac necrosis markers were elevated.Although thoracic window acquisition was difficult, echo-cardiogram examination revealed no abnormalities andglobal LV function was normal. After the pain hadsubsided, the patient was discharged and directed toconsult a cardiologist.Based on the necrosis marker changes and presence oftype-B pain on that day, the cardiologist recommended astress test, but the patient was unable to meet the physicalrequirements for accurate evaluation. In the absence of astress test, an invasive coronary angiography (ICA) wasrequested. ICA revealed normal coronary arteries, butventriculography showed an outpouching on the leftventricle that was thought to be an aneurysm (Fig. 1).Further investigation was performed in a 1.5-Tesla MRIscanner (Avanto, Siemens Medical Solutions, Erlangen,Germany) with a four-element phased-array cardiac coilusing perfusion, cine-Steady-state free precession (SSFP)and delayed-enhancement sequences. MRI results con-firmed the presence of the pouch, appending to theposterior and lateral wall of the left ventricle (Fig. 2). Itmeasured 5.8 cm in the long-axis view and 2.4 cm in theshort axis.As seen in Fig. 3, the pouch was separate from the apex.The wall thickness of the thinner segment of LV2 was 5 mmin end diastole, a little bit less than the remaining leftventricular myocardium.The majority of the pouch was found to be contractile. Inthe short-axis view, the myocardium separating bothchambers and a systolic flow jet leaving the pouch andentering the primary LV chamber were noted.The cine sequences showed normal left ventricularcontraction (ejection fraction 51%). The pouch exhibitednormal systolic contraction without any regional wallmotion abnormality.After injection of Gadolinium - diethylenetriamine penta-acetic acid (Gd-DTPA), the first pass perfusion in the short-axis view displayed normal myocardial perfusion. Lateenhancement of the short and long axes revealed nomyocardial scarring.The patient was diagnosed with a congenital DCLV. Hewas instructed to undergo diet and exercise and follow upwith a cardiologist to ensure that symptoms and cardiacfunction remain under control.DISCUSSIONDCLV is characterized by the division of the ventricularchamber into two chambers by abnormal muscular tissue. Itis best differentiated from left ventricular aneurysms andpseudoaneurysms by the fact that the double-chamberedCopyright  2010 CLINICS – This is an Open Access article distributed underthe terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided theoriginal work is properly cited.CLINICS 2010;65(12):1393-1395 DOI:10.1590/S1807-593220100012000281393ventricle exhibits contractile motion during systole. Ventri-cular aneurysm lacks complete layering of the ventricularwall, and thus expands slightly due to the increased pres-sure during systole.5,6The differentiation between double-chambered left andright ventricles is clear as they have different pathophy-siology. Double-chambered right ventricle (DCRV) is morecommon and often presents with murmur and exertionaldyspnea. Studies have found that DCRV is associatedwith septal defects, tetralogy of Fallot, and transpositionof the great arteries. Conversely, DCLV is commonlyasymptomatic. DCRV is often caused by a progressivethickening of the right ventricular septum due to thepresence of anomalous muscle bundles. This causes apressure gradient, and two chambers in series develop. Incontrast, the chambers of a DCLV are in parallel andpresent less of a pressure gradient, as both contractsynchronously. The DCLV etiology is less well known,but the anomaly is thought to be congenital and non-progressive.4,7,8Usually, DCLV is incidentally found in the course of anevaluation for other cardiovascular abnormalities. As this isan extremely rare finding, no definite data regarding theprognosis, outcomes and potential complications, such asrisk of embolism, of DCLV are available. It is generallybelieved that DCLV poses little risk to the patient.Treatment, if any, is usually guided by the presence ofother associated abnormalities.2,9,10The CMRI with the normal delayed enhancement imagessuggested that the patient had not suffered a myocardialinfarction. Additionally, there were no signs of fibrosis onthe myocardium, even near the outpouching. This rules outthe possibility that the pouch was formed as a result oftrauma or infarction, and suggests that the pouch’s origin iscongenital. Through examination of the four-chambersequence, the pouch is seen to have a fully developedmyocardial wall that contracts synchronously with the restof the myocardium. During systole, one area of the pouch’smyocardial wall appears thinner, but the delayed enhance-ment image verifies that the wall muscle is in fact fullydeveloped in that region. This excludes the possibility ofone aneurysm.4,6,11,12We believe the misdiagnosis was probably due to a poorthoracic window that did not yield proper diagnostic-quality images of this condition.There is not too much data stating the importance of othernon-invasive methods but it is reasonable to assume thatechocardiography and computed tomography can aid indetection of double-chambered ventricles. However, MRIallows a better delineation of this condition because of itshigher spatial resolution and the ability for tissue character-ization, especially regarding the differentiation betweenfibrosis and normal myocardium, which would not be easilyachievable on echocardiography and CT.Because of the rarity of DCLV, few data exist ontreatments and outcomes. Surgical excision of the accessorychamber with interposition patch reconstruction and car-diac transplant are two valid options.4,5In the case presented, normal ejection fraction and wallmotion are positive signs, suggesting that the patient’scondition is not immediately life threatening, eliminatingthe need for surgical intervention. One concern is thepossibility that a clot will form in the minor ventricle,although in the patient’s case good blood flow through thecommunication gap lessens that risk. The patient wasdischarged and told to follow up with his cardiologist; 7months of follow up have now passed without any clinicalsymptoms.Figure 1 – Ventriculography from the right anterior oblique caudal view. A, Arrows showing the first pass of the contrast media fromthe LV1 to the LV2. B, Systole. C, Diastole. The LV2 was not part of the apex (*) of the LV1.Figure 2 – Cardiac magnetic resonance imaging with theperfusion technique on the same slice position of one short axisview. A, The gadolinium is first inside of the LV1. LV2 (*) does notfill in with contrast. B, The second moment shows that thegadolinium fills the LV2 but with less concentration than LV1. C,The third moment both cavities has the same signal intensity. D,There is no perfusion defect on the lateral wall of the LV2 andalso at the whole myocardium.Double-chambered left ventricle in an adultNacif MS et al.CLINICS 2010;65(12):1393-13951394REFERENCES1. Akdemir R, Balci MM, Ozer C, Karcaaltincaba M, Acikel S.Quadrichambered ventricles. Cardiol J. 2010;17:303–5.2. Koz C, Yokusoglu M, Uzun M, Baysan O, Bulakbasi N. Double-chambered left ventricle with nonsustained ventricular tachycardia.Anadolu Kardiyol Derg. 2009;9:E5.3. Breithardt OA, Ropers D, Seeliger T, Schmid A, Von Erffa J, Garlichs C,et al. A heart within the heart: double-chambered left ventricle.Eur J Echocardiogr. 2008;9:739–41, doi: 10.1093/ejechocard/jen158.4. Kilner PJ, Geva T, Kaemmerer H, Trindade PT, Schwitter J, Webb GD.Recommendations for cardiovascular magnetic resonance in adults withcongenital heart disease from the respective working groups of TheEuropean Society of Cardiology. Eur Heart J. 2010;31:794–805, doi: 10.1093/eurheartj/ehp586.5. Awad SM, Patel AS, Polimenakos A, Braun R, Abdulla RI. LeftVentricular accessory chamber: a case report and review of the literature.Pediatr Cardiol. 2009;30:1022–5, doi: 10.1007/s00246-009-9482-6.6. Gerlis LM, Partridge JB, Fiddler GI, Williams G, Scott O. Two chamberedleft ventricle. Three new varieties. Br Heart J. 1981;46:278–84, doi: 10.1136/hrt.46.3.278.7. Choi YJ, Park SW. Characteristics of double-chambered right ventricle inadult patients. Korean J Intern Med. 2010;25:147–53, doi: 10.3904/kjim.2010.25.2.147.8. Truong QA, Yared K, Maurovich-Horvat P, Siegel E, Cubeddu RJ, KingME, et al. Images in cardiovascular medicine. Double-chambered rightventricle and situs inversus with dextrocardia. Circulation. 2010;121:E229–32, doi: 10.1161/CIR.0b013e3181d56ebd.9. Kay PH, Rigby M, Mulholland HC. Congenital double chambered leftventricle treated by exclusion of accessory chamber. Br Heart J. 1983;49:195–8, doi: 10.1136/hrt.49.2.195.10. Partington SL, Ali B, Daly RP, Koplan BA, Lilly LS, Solomon SD, et al.Images in cardiovascular medicine. Initial presentation of an accessoryleft ventricle in a patient with syncope. Circulation. 2010,121:E401–3, doi:10.1161/CIR.0b013e3181e036bf.11. Sanz J, Rius T, Kuschnir P, Macaluso F, Fuster V, Poon M. Images Incardiovascular medicine. Double-chambered left ventricle: completecharacterization by cardiac magnetic resonance and multidetector-rowcomputed tomography. Circulation. 2004;110:E502–3, doi: 10.1161/01.CIR.0000147273.67609.7D.12. Harikrishnan S, Sivasankaran S, Tharakan J. Double chamberedleft ventricle. Int J Cardiol. 2002;82:59–61, doi: 10.1016/S0167-5273(01)00570-8.Figure 3 – Cardiac magnetic resonance imaging (MRI) with the cine (A, B, D, and E) and late enhancement (C and F) techniques. A, Cine-MRI, short-axis view at the apical portion of the left ventricle (LV) showing both cavities. B, Cine-MRI, short axis view at the middleportion of LV showing both cavities. C, Delayed enhancement, short-axis view at the middle portion of LV without scar/fibrosis. D, Cine-MRI four-chamber view at diastole showing both cavities. E, Cine-MRI, four-chamber view at systole showing the thickening of thelateral wall of the LV2. F, Delayed enhancement, four-chamber view without scar/fibrosis.CLINICS 2010;65(12):1393-1395 Double-chambered left ventricle in an adultNacif MS et al.1395

Double-chambered left ventricle in an adult: diagnosis by CMRI

Abstract

Similar works

Full text

Available Versions

Cadernos Espinosanos (E-Journal)

Directory of Open Access Journals