An Unusual Suspect in a Case of Left Ventricular Aneurysm
2019; Elsevier BV; Volume: 33; Issue: 8 Linguagem: Inglês
10.1053/j.jvca.2019.01.016
ISSN1532-8422
AutoresDanielle E. Babb, Arnaldo Vera-Arroyo, Yiliam Rodriquez-Blanco, Michael Fabbro,
Tópico(s)Pericarditis and Cardiac Tamponade
ResumoTrue left ventricular aneurysms are most frequently seen after acute transmural myocardial infarction. These aneurysms are distinct from apical left ventricular pseudoaneurysms, which can also be seen in ischemia, and have a different treatment course. A major dilemma for clinicians is using echocardiographic information to make this distinction. Coronary angiography aids in this distinction; however, in the case of normal coronaries alternate etiologies must be considered. The differential for a patient with a left ventricular aneurysm and normal coronaries or no prior cardiac surgery is broad and includes traumatic, infectious and infiltrative causes. In this e-challenge, we present an unusual cause of a left ventricular apical aneurysm in a patient with normal coronary arteries residing in the United States. True left ventricular aneurysms are most frequently seen after acute transmural myocardial infarction. These aneurysms are distinct from apical left ventricular pseudoaneurysms, which can also be seen in ischemia, and have a different treatment course. A major dilemma for clinicians is using echocardiographic information to make this distinction. Coronary angiography aids in this distinction; however, in the case of normal coronaries alternate etiologies must be considered. The differential for a patient with a left ventricular aneurysm and normal coronaries or no prior cardiac surgery is broad and includes traumatic, infectious and infiltrative causes. In this e-challenge, we present an unusual cause of a left ventricular apical aneurysm in a patient with normal coronary arteries residing in the United States. Although the exact incidence is unknown, most left ventricular aneurysms (LVAs) in the United States are related to coronary atherosclerosis, while other common causes include post-cardiac surgical and traumatic causes.1Ba'albaki H.A. Clements S.D. Left ventricular aneurysm: A review.Clin Cardiol. 1989; 12: 5-13Crossref PubMed Scopus (38) Google Scholar Once an aneurysm is suspected on echocardiography, the greatest diagnostic dilemma is differentiating between a true LVA and pseudoaneurysms. Certain echocardiography features favor one diagnosis over the other, and management relies on this determination. Aneurysm location is one of those features. Pseudoaneurysms most commonly are located in the posterior and inferior left ventricular wall, whereas only 3% of true aneurysms are located posteriorly.2Zoffoli G. Mangino D. Venturini A. et al.Diagnosing left ventricular aneurysm from pseudo-aneurysm: A case report and a review in literature.J Cardiothorac Surg. 2009; 4: 1-5Crossref PubMed Scopus (62) Google Scholar Coronary angiography is usually diagnostic and is the next step in management. Combined with echocardiography findings, coronary angiography also helps differentiate true LVA from pseudoaneurysm to guide treatment.2Zoffoli G. Mangino D. Venturini A. et al.Diagnosing left ventricular aneurysm from pseudo-aneurysm: A case report and a review in literature.J Cardiothorac Surg. 2009; 4: 1-5Crossref PubMed Scopus (62) Google Scholar On coronary angiography, a true aneurysm will reveal coronary arteries that cover the thinned ventricular wall. In contrast, a pseudoaneurysm causes the classic draping pattern, such that the coronary arteries do not drape over the defect.1Ba'albaki H.A. Clements S.D. Left ventricular aneurysm: A review.Clin Cardiol. 1989; 12: 5-13Crossref PubMed Scopus (38) Google Scholar Other imaging modalities including computed tomographic angiography and magnetic resonance have been described, but are time-consuming, expensive, and not universally available.2Zoffoli G. Mangino D. Venturini A. et al.Diagnosing left ventricular aneurysm from pseudo-aneurysm: A case report and a review in literature.J Cardiothorac Surg. 2009; 4: 1-5Crossref PubMed Scopus (62) Google Scholar No widely accepted societal guidelines are currently available. In the setting of a normal coronary angiography study, alternate rare diagnoses for LVA must be considered. The patient was A 45-year-old woman emigrated from El Salvador to the United States 18 years prior with a past medical history significant for hypertriglyceridemia. She presented to her outpatient primary care physician with complaints of nonradiating chest pain associated with left arm numbness, dizziness, intermittent dyspnea at rest, and 2-pillow orthopnea for 3 months. Transthoracic echocardiography revealed a left ventricle ejection fraction of 30% to 35%, a large left ventricular apical aneurysm, and heart failure of unknown etiology (New York Heart Association class I). She was admitted to the authors’ institution to determine the cause of heart failure and for further management. On admission, transesophageal echocardiography was performed, which again demonstrated a left apical ventricular aneurysm with a reduced ejection fraction (Figs. 1 and 2). Coronary angiography was performed, which demonstrated completely normal coronaries.Fig 2Four-chamber view demonstrating apical aneurysm.View Large Image Figure ViewerDownload Hi-res image Download (PPT) With an ischemic cause of heart failure less likely, what other causes should be considered to explain the findings in Figs. 1 and 2? Given that the patient was symptomatic, the decision was made to resect the aneurysm electively. Intraoperative findings revealed a dyskinetic apex with significant enlargement (Fig 3). The patient was placed on cardiopulmonary bypass, and resection was accomplished by plicating the aneurysm with a beating heart (Fig 4). The patient met extubation criteria at the end of surgery, and inotropes were weaned off after several days. To confirm previous exposure, Trypanosoma titers were sent postoperatively and resulted in positive titers. The patient likely acquired the acute infection 20 to 30 years ago that then progressed to Chagas cardiomyopathy (CCM). Most patients at the authors’ institution in Miami, Florida are Latin American immigrants, thus the authors maintain a high level of suspicion for this disease. After surgery, the patient's heart failure symptoms were managed aggressively and she eventually was discharged. At postoperative day 60, her left ventricle ejection fraction slightly improved to 40% to 45%. Although at postoperative day 90, she continued to report shortness of breath with moderate physical exertion.Fig 4Intraoperative aneurysm resection.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Chagas disease acquired its name from the Brazilian doctor Carlos Chagas, who discovered it in 1909.3Aufderheide A.C. Salo W. Madden M. et al.A 9,000-year record of Chagas’ disease.Proc Natl Acad Sci U S A. 2004; 101: 2034-2039Crossref PubMed Scopus (293) Google Scholar Yet it is not a new disease. Evidence of the disease can be found in Chinchorro mummies in the Chilean desert that date back 9,000 years.3Aufderheide A.C. Salo W. Madden M. et al.A 9,000-year record of Chagas’ disease.Proc Natl Acad Sci U S A. 2004; 101: 2034-2039Crossref PubMed Scopus (293) Google Scholar Chagas disease is caused by the protozoan Trypanosoma cruzi (T cruzi) and is endemic to Latin America; however, with migration Chagas disease is becoming a global burden. In Latin America, an estimated 6 million people are infected with T cruzi; 300,000 of those people now reside in the United States, and 30,000 to 45,000 of these patients have cardiomyopathy. Infected insect vectors, “kissing” bugs, transmit the protozoa to humans through their feces, contaminating the mucous membranes of people typically residing in poor rural housing.4Bocchi E.A. Bestetti R.B. Scanavacca M.I. et al.Chronic Chagas heart disease management: From etiology to cardiomyopathy treatment.J Am Coll Cardiol. 2017; 70: 1510-1524Crossref PubMed Scopus (116) Google Scholar, 5Sasagawa E. de Aguilar A.V.G. de Ramírez M.A.H. et al.Acute chagas disease in El Salvador 2000-2012 - Need for surveillance and control.Mem Inst Oswaldo Cruz. 2014; 109: 256-258Crossref PubMed Scopus (10) Google Scholar Other forms of transmission have been described. Clinicians need to maintain suspicion for patients with a history of travel to endemic countries, Latin American immigrants, patients with a history of blood transfusions, and patients with a family history, as vertical transmission is possible.6Gascón J. Albajar P. Cañas E. et al.Diagnosis, management, and treatment of chronic Chagas’ heart disease in areas where Trypanosoma cruzi infection is not endemic.Rev Española Cardiol English Ed. 2007; 60: 285-293Crossref PubMed Scopus (104) Google Scholar Once contracted, a brief acute phase characterized by a nonspecific febrile illness lasting a few weeks may occur.4Bocchi E.A. Bestetti R.B. Scanavacca M.I. et al.Chronic Chagas heart disease management: From etiology to cardiomyopathy treatment.J Am Coll Cardiol. 2017; 70: 1510-1524Crossref PubMed Scopus (116) Google Scholar Rarely, acute myocarditis may occur at this time. After this acute illness, patients typically remain asymptomatic for decades, causing significant diagnostic challenges for clinicians. CCM is the most serious complication of chronic disease, and develops in 20% to 40% of patients.7Nunes M.C.P. Badano L.P. Marin-Neto J.A. et al.Multimodality imaging evaluation of Chagas disease: An expert consensus of Brazilian Cardiovascular Imaging Department (DIC) and the European Association of Cardiovascular Imaging (EACVI).Eur Hear J Cardiovasc Imaging. 2018; 19: 459-460Crossref PubMed Scopus (38) Google Scholar It is within this patient group that LVA may manifest.8Taleb M.M. An infrequent cause of apical ventricular aneurysm in the United States.Heart Views. 2017; 18: 54-57Crossref PubMed Google Scholar Apical aneurysms are found on echocardiography in greater than 50% of symptomatic patients with CCM compared to asymptomatic patients. The first diagnostic dilemma for echocardiographers in ventricular aneurysm cases is differentiating between true LVA and pseudoaneurysms or congenital diverticulum. This distinction is essential, because pseudoaneurysms can represent urgent clinical scenarios and avoidance of thrombolytics in these patients is prudent. This is crucial because both LVA and pseudoaneurysms are seen most commonly in myocardial ischemia.2Zoffoli G. Mangino D. Venturini A. et al.Diagnosing left ventricular aneurysm from pseudo-aneurysm: A case report and a review in literature.J Cardiothorac Surg. 2009; 4: 1-5Crossref PubMed Scopus (62) Google Scholar Pseudoaneurysms are caused by true myocardial wall disruptions with blood being retained often by thin adherent pericardial segments. True aneurysms involve saccular dilation of an intact ventricular wall. Pseudoaneurysms tend to occur in a posterior or posterior-lateral position, which only is seen in a small percentage (3%) of all aneurysm cases.2Zoffoli G. Mangino D. Venturini A. et al.Diagnosing left ventricular aneurysm from pseudo-aneurysm: A case report and a review in literature.J Cardiothorac Surg. 2009; 4: 1-5Crossref PubMed Scopus (62) Google Scholar Additionally, pseudoaneurysms tend to have narrow openings with larger aneurysmal bodies. Some studies have suggested using criteria comparing the neck opening width to the aneurysmal sac width, with small ratios (<0.5) being suggestive of pseudoaneurysm cases.9Gatewood R.P. Nanda N.C. Differentiation of left ventricular pseudoaneurysm from true aneurysm with two dimensional echocardiography.Am J Cardiol. 1980; 46: 869-878Abstract Full Text PDF PubMed Scopus (149) Google Scholar Congenital diverticulum is an appendage of tissue most commonly found in the left ventricle wall and normally has a hook-like appearance.10Ohlow M.A. Congenital left ventricular aneurysms and diverticula: Definition, pathophysiology, clinical relevance and treatment.Cardiology. 2006; 106: 63-72Crossref PubMed Scopus (134) Google Scholar Congenital diverticulum are recognized easily by their normal synchronous contractile function, because they contain all 3 layers of myocardium.11Yesin M. Kalçık M. Alizade E. et al.Multimodality imaging of a left ventricular aneurysm in a patient with normal coronary arteries: Unusual localization.Echocardiography. 2017; 34: 1110-1111Crossref PubMed Scopus (3) Google Scholar Coronary angiography, the next step in management, can provide additional clues because pseudoaneurysms are devoid of any coronary flow in the sac wall. Once the diagnosis of a true aneurysm is established, consideration for etiologies is important, particularly in cases with normal coronary angiography. In patients with normal coronaries, the differential diagnoses include trauma, Takotsubo cardiomyopathy, hypertrophic cardiomyopathies, focal myocarditis, glycogen storage disease, Chagas disease, sarcoidosis, amyloidosis, and congenital or idiopathic etiologies.11Yesin M. Kalçık M. Alizade E. et al.Multimodality imaging of a left ventricular aneurysm in a patient with normal coronary arteries: Unusual localization.Echocardiography. 2017; 34: 1110-1111Crossref PubMed Scopus (3) Google Scholar, 12Leucker T.M. Agrawal V. Rahim H. et al.Idiopathic “true” left ventricular aneurysm.J Cardiol Cases. 2015; 12: 202-204Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar, 13Yalçinkaya E. Çelik M. Bugan B. Aneurysm associated with normal coronary arteries: A diagnostic dilemma.Int J Cardiol. 2013; 168: 5096Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar, 14Chowdhury U.K. Seth S. Sheil A. et al.Successful aneurysmectomy of a congenital apical left ventricular aneurysm.Texas Heart Inst J. 2009; 36: 331-333PubMed Google Scholar Many of these diagnoses may be suspected based on patient history, clinical scenario, or other echocardiographic findings. CCM is unique in that it often is the only manifestation of chronic Chagas. Apical aneurysms in the setting of dilated cardiomyopathy and normal coronaries are correlated strongly with Chagas’ disease, especially in patients with a history of potential exposure. Aneurysms in Chagas’ disease may have some specific echocardiography findings suggestive of the diagnosis.15Acquatella H. Echocardiography in Chagas heart disease.Circulation. 2007; 115: 1124-1131Crossref PubMed Scopus (160) Google Scholar Echocardiographic findings consistent with Chagas include regional wall motion abnormalities, particularly inferior wall hypokinesis, in the absence of coronary disease. Aneurysms also may occur uniquely in the inferior wall (up to 20% of the time) as opposed to the apex or anterior wall as seen in true aneurysm secondary to ischemic events. Global left ventricle dilation and diffuse hypokinesis also are seen in advanced disease.15Acquatella H. Echocardiography in Chagas heart disease.Circulation. 2007; 115: 1124-1131Crossref PubMed Scopus (160) Google Scholar Further imaging modalities such as left ventriculography, computed tomographic angiography, and cardiac magnetic resonance (CMR) imaging with gadolinium also may be helpful. CMR is particularly useful because it is a low-risk, noninvasive tool that assesses myocardial function and tissue. CMR may favor certain cardiomyopathies based on the delayed gadolinium enhancement pattern.13Yalçinkaya E. Çelik M. Bugan B. Aneurysm associated with normal coronary arteries: A diagnostic dilemma.Int J Cardiol. 2013; 168: 5096Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar Delayed gadolinium enhancement appears subendocardial to transmural for a myocardial infarction, and a mid-myocardial pattern suggests myocarditis pathologies. A minimal delayed gadolinium enhancement pattern alludes to Takotsubo cardiomyopathy, while an epicardial pattern to the left ventricular free wall suggests Chagas disease.13Yalçinkaya E. Çelik M. Bugan B. Aneurysm associated with normal coronary arteries: A diagnostic dilemma.Int J Cardiol. 2013; 168: 5096Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar, 16Sechtem U. Mahrholdt H. Vogelsberg H. Cardiac magnetic resonance in myocardial disease.Heart. 2007; 93: 1520-1527Crossref PubMed Scopus (38) Google Scholar Treatment is aimed at early diagnosis, imaging surveillance, and, once Chagas has progressed to CCM, treatment of heart failure symptoms. Advanced CCM often requires surgical interventions including ablation procedures, automatic implantable cardioverter-defibrillator and pacemaker implantation, aneurysm resection, left ventricular assist devices, bridging extracorporeal membrane oxygenation, and, lastly, heart transplantation.6Gascón J. Albajar P. Cañas E. et al.Diagnosis, management, and treatment of chronic Chagas’ heart disease in areas where Trypanosoma cruzi infection is not endemic.Rev Española Cardiol English Ed. 2007; 60: 285-293Crossref PubMed Scopus (104) Google Scholar In nonendemic countries like the United States, recognition of cardiac involvement by CCM likely is underappreciated and diagnosis remains challenging. With increasing Latin American immigration, physicians need to maintain a high notion for this disease when patients present with new-onset nonischemic cardiomyopathy with or without a left ventricular apical aneurysm. Chagas Disease and the Kissing Bug: An Invisible GiantJournal of Cardiothoracic and Vascular AnesthesiaVol. 33Issue 8Preview“Triatoma Infestans is how I am formally called, But I have many nicknames all over Brazil,Bicudo, Barbeiro, or Chupão, I am the famous bug. (...) (...) Places that I like to make my home Cracks in cob walls or unfinished furniture In every place that I arrive, I find my cozy spot And my offspring are raised.”1 Full-Text PDF
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