Global Akinesis: Inadvertent Left Ventricular “Pseudoaneurysmogram”

Case Report

A 65-year old male with a history of bipolar illness had sustained a large myocardial infarction (MI) complicated by Dressler’s syndrome at an outside hospital. Based upon cardiac catheterization findings, surgical intervention was recommended; however, the patient refused, stating: “I refuse surgery. I’ll leave it up to God.” Additionally, the patient also refused to take all medications, but nonetheless had an uneventful post-infarct course. He was then transferred to an inpatient psychiatric facility for treatment of an exacerbated bipolar episode complicated by paranoid delusions. Two months post-MI, the patient was admitted to our emergency room with a 2-week history of pleuritic chest pains and productive cough.
His past medical history was otherwise significant for hyperlipidemia, hypertension, osteoarthritis and bladder tumors status post-multiple excisions. He admitted to former tobacco and alcohol abuse. On transfer, his medications included: ziprasidone 60 mg twice daily, carvedilol 3.125 mg twice daily, furosemide 40 mg daily, aspirin 81 mg daily, potassium chloride 10 mEq daily, atorvastatin 10 mg daily, pantoprazole 40 mg daily and quetiapine 100 mg at bedtime.
On examination, the patient’s blood pressure was 120/71 mmHg, his pulse was 101 beats per minute (sinus tachycardia), his respiratory rate was 24 per minute, his temperature was 98.4ºC, and his oxygen saturation was 96% on room air. Bibasilar scattered pulmonary rales were present. Cardiac examination revealed a laterally displaced point of maximal impulse. A III/VI holosystolic murmur was auscultated at the apex, radiating to the base; no thrill was appreciated. The abdomen was soft without masses, and no peripheral edema was present. Neurologically, he answered all questions appropriately and was oriented to person, place and time. His affect, however, seemed somewhat depressed.
Laboratory data included: brain natiuretic peptide level of 1,050 pg/mL, creatine kinase level of 57 IU/L (with muscle-brain component of 1.5 ng/mL), troponin I level of 0.73 ng/mL, creatine of 1.0 mg/dL, white blood count of 18,700/uL, and hemoglobin of 10.7 g/dL. The electrocardiogram revealed normal sinus rhythm, incomplete right bundle-branch block, Q-waves in the inferior leads and T-wave inversions in the lateral leads. His chest radiograph revealed focal eventration of the left hemidiaphragm but no infiltrate, pulmonary vascular redistribution or effusion. No growth was noted on serial blood or sputum cultures. The patient was treated with intravenous diuresis and levofloxacin antibiotic therapy, and his psychiatric medications were adjusted.
Cardiology consultation was requested for the patient’s significant recent cardiac events. Lisinopril, spironolactone, digoxin and enoxaparin were added to the medical regimen. Review of the previous catheterization revealed a giant inferior left ventricular pseudoaneurysm (PA) (Figure 1). In this “left ventriculogram”, the pigtail catheter was actually placed into the PA, and the image was in fact an unrecognized “peudoaneurysmogram”. The calcified left coronary system could be seen a significant distance above the PA, implying the intervening space was in fact the true left ventricle (LV), as delineated in Figure 2. Based upon this image, the left ventricular ejection fraction (EF) was initially thought to be dismal, since the PA was globally akinetic. The left main coronary artery was moderately stenosed, and the right coronary system was totally occluded, with minimal collateralization.
A subsequent echocardiogram, however, revealed an estimated EF of 30%. A large “jet” emanating from the LV into the inferiorly and a posterolaterally located PA was clearly demonstrated on echocardiographic color Doppler interrogation (Figures 3 and 4). The inferolateral location of the “neck” of the PA is clearly demonstrated in the apical 4-chamber view and the parasternal short axis views, respectively (Figures 5 and 6). In several views, the PA nearly matched the LV in size. Moderate-to-severe mitral and mild-to-moderate tricuspid regurgitation, along with left atrial dilatation, were also noted.
Coronary artery bypass grafting, left ventricular PA resection, and mitral valve repair were once again strongly recommended. Psychiatric consultation deemed the patient competent to make healthcare decisions. Over the next several days, the patient initially refused all intervention, threatened intermittently to sign out against medical advice and required security guard surveillance for violent behavior. Surgery was finally performed on March 23, 2006. Operative findings included a 12 cm inferior and posterolateral PA contained within severe pericardial adhesions, filled with thrombus, and without active flow from the LV (Figure 7). This structure originated from a large “neck” in the LV inferolateral wall. The inferior LV wall was infarcted and scarred, and the coronary arteries were severely calcified. A left internal mammary artery graft was placed to the left anterior descending artery, a reversed saphenous vein graft was placed to the obtuse marginal branch, but the right coronary artery was not bypassable. Mitral ring annuloplasty and endoaneurysmorrhaphy patch repair were also successfully performed. Intraoperatively, the patient exhibited severe coagulopathy requiring voluminous blood product transfusions. The coagulopathy likely occurred due to release of thrombotically active substances from the PA. His postoperative course was protracted and complicated by pneumonia. Unfortunately, the patient and his wife had separated, and she refused to allow him home. He was eventually transferred to an inpatient rehabilitation facility on April 4, 2006, three months after his MI, and experienced an excellent recuperation. Apparently he was right — God was watching over him.

Discussion

Background and epidemiology. Dr. Jesse Edwards provided early pathological descriptions of PAs.¹ These resulted from incomplete myocardial free wall rupture, with subsequent containment by the surrounding tissue. Perhaps the most dreaded complication of MI is LV free wall rupture and tamponade, which, in one series, was associated with 17% of all fatal MIs.² After cardiogenic shock, it is the second leading cause of inhospital post-MI mortality.³ However, when such a rupture is sealed by the pericardium or an overlying hematoma, a PA results. The classic sac-like appearance is characterized by a narrow neck or orifice, usually defined echocardiographically as a diameter less than half that of the widest sac diameter.⁴ Nathaniel et al classified 4 patterns of myocardial rupture. Both Types III and IV are associated with narrow orifices. Type III, corresponding to the generally-recognized LV PA, refers to a contained rupture enclosed by either pericardium or thrombus. In addition, they also include Type IV, a near-transmural rupture contained by a thin epicardial sac, as a variant of PA. Although distinguishable only by pathological examination, there are scattered data to suggest possible improved outcome associated with the slightly thicker-walled Type IV rupture.⁵
Anatomically, there is no clear predilection of PA for any LV segment. While some series reported more anterior PAs,⁶ others found an inferior predominance,⁷ as seen in our patient. The vast majority of true aneurysms, however, are located posteriorly, likely because anterior wall ruptures are usually fatal.⁸ One series reported PAs in 4% of all MIs and in 23% of fatal MIs. In postinfarct patients, PAs are 5 times more common than septal ruptures.⁹ Delay in reperfusion, as seen in our patient, is associated with Dressler’s syndrome and subsequent PA formation; however, the incidence appears to be declining, owing to modern expedited reperfusion strategies.¹⁰
Frances and coworkers reported that one-third of PAs in their review were postoperative, usually following mitral valve replacement.7 Other surgeries less commonly complicated by PAs include right ventricular outflow tract repair, Tetralogy of Fallot repair and pulmonic valvotomy.¹¹ Kanna and colleagues reported successful resection of a giant anterior PA which developed 1 year after fibrin glue repair of a previous free wall rupture.¹² Rarely, infectious etiologies have been implicated. A large PA was reported in a 4-year-old girl who had developed purulent Staphylococcus aureus pericarditis.¹³ Postinfectious endocarditis PA is associated with a high risk of rupture and death.¹⁴
Presentation. The history of a patient presenting with PA may often include a recent MI or cardiac surgery. In their PA series, Frances and others reported the most common presenting symptoms to include: congestive heart failure, chest pain, dyspnea, cerebrovascular accident, arrhythmia, back or shoulder pain and death.⁷ Reinecke and colleagues reported a patient who developed Dressler’s syndrome 2 weeks postinfarct and a large PA 6 weeks subsequently.¹⁵ The classic new “to-and-fro” systolic murmur may be absent in a large number of patients, and when present, may be mistaken for mitral regurgitation.¹⁶ Electrocardiographic findings are often unrevealing. In Frances’ series of electrocardiograms in 290 patients with PAs, only 20% demonstrated ST-segment elevations; most had only nonspecific ST and T-wave changes. The most common chest radiographic finding was cardiomegaly.⁷ A high clinical index of suspicion, therefore, is required to pursue further, more definitive diagnostic testing.
Diagnosis. Cardiac catheterization with left ventriculography remains the gold standard for PA diagnosis. In addition, concomitant coronary angiography is often required for preoperative delineation of coronary anatomy. Care should be taken, however, to avoid direct high-pressure contrast injection into the PA due to the risk of catastrophic rupture. Despite its massive size, the PA found in our patient was likely fairly stable, given the uneventful course after the inadvertent “LV PAgram”. As illustrated by the present case, landmarks such as coronary calcifications can aid in confirming the true cardiac chamber.
Cardiac magnetic resonance imaging (CMRI), cardiac computerized tomography, transesophageal echocardiography and three-dimensional echocardiography also provide excellent noninvasive visualization. In 1991, Kahn and coworkers described the characteristic loss of epicardial fat at the orifice seen on CMRI. However, low myocardial signal from post-MI scar seen in a true aneurysm may appear similar.¹⁷ Although the easiest and perhaps most commonly performed imaging modality for PA detection, two-dimensional echocardiography is the least accurate. In one series, definitive diagnosis was made echocardiographically in only 26% of patients. The median PA and orifice diameters were reported to be 6 and 2 cm, respectively.⁷ Nanda et al proposed the neck:sac ratio of 0.5 or less as an echocardiographic criterion.⁴
Clinical course. PA rupture, the most serious complication, has been reported in one series to occur in 30–45% of patients.⁶ Risk factors for rupture include: advanced age (>60 years), female gender, history of hypertension, recent large MIs, lack of collateral coronary flow, lack of previous angina or MI, delayed reperfusion (>7 hours), symptoms of pericarditis and prominent electrocardiographic ST-segment abnormalities. In addition, agitation and forceful emesis have also been linked to rupture, likely secondary to increased intrathoracic pressure.⁷ O’Rourke divides ruptures into either acute or subacute types. The former is characterized by tamponade with cardiac arrest, electrical mechanical dissociation (pulseless electrical activity) and usually death. Patients with the latter type may not have cardiac arrest and can frequently be successfully resuscitated.¹⁸ It has been proposed that these more stable PAs may represent Nathaniel Type IV variants.⁵ One unusual complication of a PA to left atrial fistula was reported by Walters and Caplin in a patient 3 years after an inferoposterior MI.¹⁹
Some studies, however, have reported favorable outcomes with conservative observation in the asymptomatic patient. In 1963, Hurst and coworkers reported over 6-year survival in a patient with a PA status following a large posterolateral MI.²⁰ Thirteen of 19 patients in one series survived more than 4 years after diagnosis.³ Moreno and colleagues followed 9 patients with asymptomatic LV PAs for a mean of 3.8 years. They reported 1- and 4-year survivals to be 88.9% and 74.1%, respectively. Although 3 patients suffered ischemic strokes, no patients experienced a fatal rupture. Based upon these results, the authors recommended close medical follow up with anticoagulation therapy to reduce the embolic risk. They advocated surgical intervention only in the very young patient with preserved LV function and few or no comorbid conditions.²¹
Yeo and coauthors reported 3.2-year mean survival for 5 of 6 medically-treated PA patients. Of the 2 who suffered cardiac deaths, neither had PA rupture. One case report described a 70-year-old male who remained alive 12 years after diagnosis; it was unclear, however, whether this PA was postinfarct. The authors suggested that the patient’s reduced LV function may have allowed for lower pressures within the PA.²² Hung and colleagues reported a patient scheduled for coronary bypass surgery with PA repair. Intraoperatively, however, they found a well-organized thrombus sealing off the PA; in addition, the myocardium surrounding the PA was necrotic, making suturing impractical. Eight months later, with conservative therapy, the patient remained well, with brisk flow still demonstrable from the LV into the PA.²³ Similarly, in their review, Natarajan et al found medical therapy not to be associated with significant rupture risk.²⁴ Other studies, however, have found less favorable prognoses associated with conservative therapy, reporting up to 48% mortality within 1 week of diagnosis.⁷
Therapy. For the symptomatic patient with PA, especially when rupture appears imminent, surgical repair is emergent and clearly the treatment of choice. For the asymptomatic patient, however, the decision has traditionally been less clear. Aside from standard post-MI and heart failure medical regimens, there is little pharmacological therapy aimed specifically at PAs. While steroids and nonsteroidal anti-inflammatory agents have been recommended, there is concern that these drugs may actually increase the risk of rupture, and must therefore be administered with caution.²⁵ Certainly, given the improved modern surgical mortality rate, surgical intervention remains the mainstay of definitive treatment. PAs can often be directly sutured without the need for pledgets or patches, making the repair easier than that of true aneurysms.²⁶ The reported postoperative mortality rate ranges from 13–29%, believed by many experts to be acceptable when weighed against the possibility of fatal rupture.^24,27,28 Surgical authorities are optimistic that further improvements in technique will lower the rate to <10%.²⁹
In this case, the recommendation for surgical intervention was based upon both the need for bypass grafting and mitral valve repair as well as the PA’s dimensions. Although the incidence of post-MI PAs appears to be declining due to widespread urgent reperfusion strategies, our patient, due to his unusual psychosocial circumstances, clearly did not benefit from these advancements.

Conclusion

While LV PAs are relatively uncommon findings, they represent a special group of patients who require vigilant monitoring and treatment. Clinicians must maintain a high index of suspicion when evaluating any patient at risk of PA formation, since the clinical clues are usually subtle, nonspecific or absent. Once the diagnosis is considered, most modern cardiac imaging modalities can readily provide the accurate diagnosis. Future developments in CMRI and cardiac computerized tomographic angiography may provide valuable insight into PA wall stress and aid in identification of the patient at high risk for future rupture. Although commonly recommended, the necessity of surgical intervention in the asymptomatic patient with a PA, especially if small, is still somewhat controversial. Due to the relative infrequency of LV PAs today, large-scale randomized, controlled studies are not logistically feasible to answer the question; therefore, treatment plans must be individualized for each patient.

Acknowledgements. Special thanks to Dr. Jeffrey Miller for his intraoperative photograph and to Ms. Lewana Trifoso, RN, and Mr. Scott Matthewson for their technical assistance.

References

1. Chesler E, Korns ME, Semba T, et al. False aneurysm of the left ventricle following myocardial infarction. Am J Cardiol 1969;23:76–82.
2. Dellborg M, Held P, Swedberg K, et al. Rupture of the myocardium. Occurrence and risk factors. Br Hear J 1985;54:11–16.
3. Figueras J, Cortadellas J, Evangelista A, et al. Medical management of selected patients with left ventricular free wall rupture during acute myocardial infarction. J Am Coll Cardiol 1977;29:512–518.
4. Gatewood R Jr, Nanda NC. Differentiation of left ventricular pseudoaneurysm from true aneurysm with two dimensional echocardiography. Am J Cardiol 1980;46:869–878.
5. Nathaniel C, Lane S, Palma R, et al. Pseudoaneurysm causing partial obliteration of the left atrium: Case report and review. Cathet Cardiovasc Diagn 1996;38:83–86.
6. Davidson KH, Parisi AF, Harrington JJ, et al. Pseudoaneurysm of the left ventricle: an unusual echocardiographic presentation. Review of the literature. Ann Intern Med 1977;86:430–433.
7. Frances C, Romero A, Grady D. Left ventricular pseudoaneurysm. J Am Coll Cardiol 1998;32:557–561.
8. Rittenhouse EA, Sauvage LR, Mansfield PB, et al. False aneurysm of the left ventricle. Report of four cases and review of surgical management. Ann Surg 1979;189:409–415.
9. Ropers D, Achenbach S, Pfeiffer S. Left ventricular pseudoaneurysm following myocardial infarction. Heart 2004;90:555.
10. Shahar A, Hod H, Barabash GM, et al. Disappearance of a syndrome: Dressler’s syndrome in the era of thrombolysis. Cardiology 1994;85:255–258.
11. Sadiq M, Fenton AC, Firmin RK. False aneurysm of the right ventricle outflow tract after total correction of tetralogy of Fallot: Diagnosis by echocardiography and successful repair by neck cannulation of cardiopulmonary bypass. Br Heart J 1994;7:566–568.
12. Kanna M, Kakuchi H, Nonogi H. Giant left ventricular pseudoaneurysm following left ventricular rupture caused by myocardial infarction. Heart 2002;88:446.
13. Morales AN, Fereira AG Jr, Ferreira SM. Left ventricular pseudoaneurysm complicating infective pericarditis. Heart 1999;82:393–394.
14. Qizilbash AH, Schwartz CJ. False aneurysm of left ventricle due to perforation of mitral-aortic intervalvular fibrosa with rupture and cardiac tamponade. Rare complication of infective endocarditis. Am J Cardiol 1973;32:110–113.
15. Reinecke H, Wichter T, Weyand M. Left ventricular pseudoaneurysm in a patient with Dressler’s syndrome after myocardial infarction. Heart 1998;80:98–100.
16. Turgeman Y, Antonelli D, Rosenfeld T. Intermittent to-and-fro murmur in cardiac pseudoaneurysm: Doppler echocardiographic findings. Int J Cardiol 1990;26:376–377.
17. Kahn J, Fisher MR. MRI of cardiac pseudoaneurysm and other complications of myocardial infarction. Magn Reson Imaging 1991;9:159–164.
18. O’Rourke MF. Subacute heart rupture following myocardial infarction: Clinical features of a correctable condition. Lancet 1973;ii:124–126.
19. Walters MI, Caplin JL. Atrivoentricular fistula: an unusual complication of a ventricular pseudoaneurysm after myocardial infarction. Heart 1998;79:523–524.
20. Hurst CO, Fine G, Keyes JW. Pseudoaneurysm of the heart. Report of a case and review of literature. Circulation 1963;28:427–436.
21. Moreno R, Gordillo E, Zamorano J, et al. Long term outcome of patients with postinfarction left ventricular pseudoaneurysm. Heart 203;89:1144–1146.
22. Bolognesi R, Cuchini F, Lettieri C, et al. Left ventricular false aneurysm: An unusually prolonged natural history. Cathet Cardiovasc Diagn 1995;36:46–50.
23. Hung M-J, Wang C-H, Cherng W-J. Unruptured left ventricular pseudoaneurysm following myocardial infarction. Heart 1998;80:94–97.
24. Natarajan MK, Salerno TA, Burke B, et al. Chronic false aneurysms of the left ventricle: Management revisited. Can J Cardiol 1994;10:927–931.
25. Silverman HS, Pfeffer MP. Relation between use of anti-inflammatory agents and left ventricular free wall rupture during acute myocardial infarction. Am J Cardiol 1987;59:363–364.
26. Treasure T. False aneurysm of the left ventricle. Heart 1998;80:7–8.
27. Komeda M, David TE. Surgical treatment of postinfarction false aneurysm of the left ventricle. J Thorac Cardiovasc Surg 1993;106:1189–1191.
28. Figueras J, Cortadellas J, Domingo E, et al. Survival following self-limited left ventricular free wall rupture during myocardial infarction. Management difference between patients with or without pseudoaneurysm formation. Int J Cardiol 2001;79:103–111.
29. Bolooki H. Surgical treatment of complications of acute myocardial infarction. JAMA 1990;263:1237–1240.