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Angina caused by systolic compression of the left coronary artery as a result of pseudoaneurysm of the mitral-aortic intervalvular fibrosa
Volume 129, Number 2 American Heart Journal
operative course. Careful multiple suprasternal and high parasternal sweeps document the anatomy of the vascular ring and the spatial relation of the vessels that comprise the ring. 4 Color flow Doppler makes it easier to follow the arterial branches and differentiate the veins from the arterial vessels of the upper mediastinum and base of the neck. Furthermore, Doppler echocardiography allows reliable assessment of the hemodynamics of this defect. The combined use of detailed two-dimensional echocardiographic sweeps and color flow Doppler should allow comprehensive anatomic and physiologic evaluation of the truncus arteriosus and any associated aortic arch anomaly. We thank Hewlett-Packard Co. (Andover, Mass.) for funding the publication of the color illustrations in this article.
REFERENCES
1. CalderL, Van Praagh R, Van Praagh S, Sears WP, CorwinR, LevyA, Keith JD, Paul MH. Truncus arteriosus communis:clinical,angiocardiographic, and pathologic findings in 100 patients. AM HEART J 1976;92:23-8. 2. HanleyFL, HeinemannMK, Jonas RA, MayerJE Jr., Cook NR, Wessel DL, Castaneda-AR.Repair of truncus arteriosus in the neonate.J Thorac Cardiovasc Surg 1993;105:1047-56. 3. Murdison KA, Andrews BA, Chin AJ. Ultrasonographicdisplay of complex vascular rings. J Am Cell Cardlol 1990;15:1645-53. 4. Butte F, Lucas RV Jr., Edwards JE. Persistent truncus arteriosus: pathologic anatomyin 54 cases. Pediatr Cardiol 1986;7:95-101. 5. Di DonateRM, FyfeDA, Puga FJ, DanielsonGK, Ritter DG, Edwards WD, McGoonDC. Fifteen-yearexperiencewith surgicalrepair of truncus arteriosus. J Thorac CardiovascSurg 1985;89:414-22. 6. BinetJP, LangloisJ. Aortic arch anomaliesin childrenand infants.J Thorac CardiovascSurg 1977;73:248-52.
Angina caused by systolic compression of the left coronary artery as a result of pseudoaneurysm of the mitral-aortic intervalvular fibrosa Deepak K. Parashara, MD, Larry E. Jacobs, MD, Morris N. Kotler, MD, Shahriar Yazdanfar, MD, Scott R. Spielman, MD, Sean F. Janzer, MD, and Charles E. Bemis, MD Philadelphia, Pa.
Pseudoaneurysm formation of the mitral-aortic intervalvular fibrosa (MAIVF) is a well-recognized complication of aortic valve endocarditis. 1 Mitral-aortic intervalvular fibrosa (MAIVF) pseudoaneurysm may rupture into the
From the Department of Medicine,Divisionof CardiovascularDiseases, Albert Einstein Medical Center, Temple UniversitySchool of Medicine. Supported in part by the Women'sLeague for Medical Research, Albert Einstein Medical Center. Reprmt requests: Larry E. Jacobs, MD, Divisionof Cardiology,Suite 331, KleinBuilding,AlbertEinsteinMedicalCenter,5401YorkRoad, Philadelphia, PA 19141. AMHEARTJ 1995;129:417-21. Copyright ® 1995 by Mosby-YearBook, Inc. 0002-8703/95/$3.00+ 0 4/4/60396
Parashara et al.
417
pericardium,2, 3 causing fatal cardiac tamponade, or may rupture into the left atrium, 1, 4, 5 causing mitral regurgitation. In some instances the pseudoaneurysm of MAIVF remains intact 6, 7 and appears as a pulsatile cavity with systolic expansion. We report on a patient with angina in the presence of an expanding pseudoaneurysm of the MAIVF after aortic valve replacement for aortic valve endocarditis. The presumed mechanism of angina was compression of the left coronary artery by the pseudoaneurysm of the MAIVF. A 59-year-old m a n with a history of peripheral vascular disease, percutaneous femoral artery angioplasty, and 40year history of two-pack cigarette smoking had sudden acute onset of nonexertional, substernal chest pain radiating to both shoulders. For the previous week the patient had had weakness, myalgia, anorexia, fever, night sweats, and upper respiratory tract symptoms. Physical examination revealed a temperature of 39 ° C, pulse rate of 72 beats/min and a blood pressure of 136/78 m m Hg. The skin showed no sign of needle marks, and throat examination was benign. There was no jugular venous distension, and carotid artery was 2+ palpable without bruit. The cardiac examination revealed a III/VI diastolic m u r m u r in the aortic area. The lung and abdominal examinations revealed nothing remarkable. The electrocardiogram during chest pain showed 1.5 m m ST-segment depression in the inferolateral leads that resolved with one sublingual nitroglycerine tablet. Myocardial infarction was ruled out with serial cardiac enzyme blood determinations. Transthoracic echocardiography revealed moderate to severe aortic regurgitation with hyperdynamic left ventricular function. Aortography demonstrated 4+ aortic regurgitation and a shaggy, irregular left coronary cusp. Coronary cineangiography showed normal coronary arteries without obstructive disease. Transesophageal echocardiography revealed a mobile shaggy mass measuring 1.5 cm attached to the left coronary cusp. Multiple blood cultures were positive for a-hemolytic streptococcus, and the patient was treated with intravenous vancomycin 1 gm every 12 hours. Results of a detailed work-up for source of infection were negative. The patient underwent aortic valve replacement surgery on the fifth day of hospitalization for intractable congestive heart failure. The aortic valve was trileaflet, with a 1.5 cm pedunculated mass present on the left coronary cusp with erosion into the annulus. The postoperative course was unremarkable, and intravenous antibiotic therapy was continued for 6 weeks. The patient was discharged home on oral anticoagulant therapy. Three months after his discharge from hospital, the patient complained of bilateral decrease in acuity of vision. Fundoscopic examination revealed evidence of bilateral retinal artery microembolization. Performed as part of the work-up for source of microemboli, transesophageal echocardiography demonstrated a 3.0 × 2.0 cm pseudoaneurysm of MAIVF, with linear mobile thin filamentous structures seen in the pseudoaneurysm. Persantine 25 mg thrice daily and aspirin 80 mg daily were added to treatment. No further visual symptoms were noted. Six months later the patient had typical exertional angina. An exercise thallium test was performed. The patient achieved
418 Par~hara
February 1995 American Heart Journal
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85 % of the predicted maximal heart rate for his age with 7 METS workload, and he had typical anginal pain that was relieved with rest. The electrocardiogram was positive for ischemia in the inferolateral and anterior leads. Thallium scan showed extensive myocardial perfusion abnormalities involving the inferolateral, anteroseptal, and apical segments with complete redistribution (Fig. 1). Aortography performed in multiple projections revealed a 4 cm pulsatile donut-shaped pseudoaneurysm on the left side of the aortic root. In the left lateral projection the pseudoaneurysm was visualized on the left side of the aortic root, extending posteriorly under the left main coronary artery and left circumflex coronary artery but above the left atrium (Fig. 2). Coronary cineangiogram demonstrated that the left main coronary artery had no obstructive disease but was compressed to >50 % of its diameter during systole by the systolic expansion of the pseudoaneurysm (Fig. 3). The left circumflex coronary artery was free of disease, and a long proximal segment of the artery was completely occluded in systole by the expanding pseudoaneurysm. The left anterior descending coronary artery and the right coronary artery showed no obstructive disease, and there was no compression by the pseudoaneu-
rysm. Transesophageal echocardiogram demonstrated a 4.0 x 2.0 cm pulsatile donut-shaped pseudoaneurysm of MAIVF on the left side of the aortic root below the left atrium and between the left ventricular outflow tract and the main pulmonary artery (Fig. 4). The left main coronary artery and left circumflex artery were seen to be obliterated with systolic expansion of pseudoaneurysm against the main pulmonary artery. Surgery was performed at another institution. The angiographic and transesophageal findings were confirmed. The pseudoaneurysm was closed, and the aortic annulus was sutured to the St. Jude valve. On the seventh postoperative day, the patient suddenly collapsed in the bathroom and had cardiac arrest. An autopsy performed at the other institution showed severe s~enosis of the left main coronary artery caused by intimal fibroplasia and total occlusion with recent thrombus formation. A large recent myocardial infarction was also present. Aortic valve endocarditis can produce perforation and destruction of the aortic leaflet, which leads to severe acute aortic regurgitation that generally results in hemodynamic deterioration and left ventricular failure, s The aortic regurgitant jet emanating from the infected aortic valve directed toward the subaortic structures may cause seeding
Volume 129, Number 2
419
Parashara et aI.
American Heart Journal
.
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Fig. 3. A, Left coronary artery cineangiogram in left lateral projection in systole shows 60 % narrowing of distal left main coronary artery and complete obliteration of proximal left circumflex coronary artery. B, Left coronary artery cineangiogram in left lateral projection in diastole shows normal patency of proximal left main coronary artery and left circumflex coronary artery.
of infection of the subaortic structures. 1, s Alternatively, the subaortic structures may be infected by direct extension of the infection from the infected aortic valve. 1, s Anatomically, the subaortic structures consist of an anterior mitral leaflet, which lies in a somewhat medial position subjacent to the posterior aortic root, 1, 3 and the MAIVF, a fibrous interannular tissue between the base of the anterior mitral leaflet and the left and noncoronary aortic cusp.l, 4 The MAIVF is relatively avascular and offers little resistance to infection. Infection is the most common cause of pseudoaneurysm of MAIVF. Blunt chest trauma has also
been implicated as a cause of the MAIVF pseudoaneurysm, 9 and it has been suggested that MAIVF is congenitally weakened in these patients. 2 Infection of the MAIVF may result in formation of abscess or pseudoaneurysm or a direct communication from left ventricular outflow into the left atrium. 1 A review of the literature shows that pseudoaneurysm of the MAIVF may rupture into the pericardial sac and result in fatal hemopericardium. 2, 3 It may rupture into the left atrium with systolic shunting of the blood from the left ventricular outflow tract into the left atrium 4, 5 with resultant supraannular mitral regurgitation
420
February 1995 American Heart Journal
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Fig. 4. A, Transesophageal echocardiography (TEE) in transverse plane shows relation of pseudoaneurysm of MAIVF to left atrial appendage (APP), left atrium (LA), and St. Jude aortic prosthetic valve (AO V). B, Transesophageal echocardiography in longitudinal plane shows pseudoaneurysm of MAIVF in relation to left ventricular outflow tract (LVOT) and main pulmonary artery (PA). LV, Left ventricular.
and an eccentric jet directed toward the lateral left atrial wall. 1 This pseudoaneurysm may remain intact, 6, 7 appearing as an echo-free cavity in systole, posterior to the aortic root. In our patient, we angiographicaUy demonstrated systolic compression of the left main coronary artery to >50 % of its diameter and complete occlusion of the left circumflex artery by the pseudoaneurysm of the MAIVF. Systolic compression of coronary arteries occurred as a result of systolic expansion of the pseudoaneurysm impinging on the left main artery and the left circumflex coronary artery against the main pulmonary artery. The physiologic significance of the observed compression of coronary arteries resulted in typical exertional anginal symptoms and the
demonstration of positive exercise electrocardiogram and extensive ischemia on exercise thallium-201 single-photon emission scintigraphy. The exact mechanism of ischemia caused by systolic compression of the coronary artery with pseudoaneurysm of the mitral aortic intravalvutar fibrosa is unclear. It is likely that beat-to-beat systolic impingement led to fibroplasia of the left main coronary artery and subsequent thrombus formation, culminating in the untimely death of this patient. Angiography failed to visualize severe stenosis of the left main artery due to intimal fibroplasia as confirmed by autopsy. This is consistent with prior reports, which demonstrate that angiographic determination of the percentage of stenosis of the left main coronary artery are often inaccurate and imprecise, l°-12 Had
Volume 129, Number 2 American Heart Journal
intravascular ultrasound of the left main coronary artery been performed, it is likely that the intravascular ultrasound would have confirmed left main stenosis. 13 In addition, systolic compression of the left main coronary artery may have further aggravated ischemia in our patient by the following mechanism. The normal pattern of the epicardial coronary blood flow is characterized by predominance of flow during diastole. In the presence of significant aortic regurgitation, the systolic coronary blood flow increases and diastolic coronary blood flow decreases. 14 Thus in our patient, who had 4+ aortic regurgitation resulting in increased dependence on systolic coronary flow, systolic compression of the left main coronary artery may well have further exacerbated ischemia. REFERENCES
1. Karalis DG, Bansal RC, Hauck AJ, Ross J J, Applegate PM, Jutzy KR, Mintz GS, Krishnaswamy C. Transesophageal echocardiographic recognition of subaortic complication in aortic valve endocarditis. Circulation 1992;86:353-62. 2. Qizibash 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;32:110-3. 3. Chesler E, Korns ME, Porter GE, Reyes CN, Edwards JE. False aneurysm of the left ventricle secondary to bacterial endocarditis with perforation of the mitral-aortic intervalvular fibrosa. Circulation 1968;37: 518-23. 4. Schwartz DR, Belkin RN, Pucillo AL, Burleson PD, Fish BG, Polley RW, Weiss MB, Herman MV. Aneurysm of the mitral-aortic fibrosa complicating infective endocarditis: preoperative characterization by two-dimensional and color Doppler echocardiography, magnetic resonance imaging, and cineangiography. AM HEART J 1990;119:196-9. 5. Bansal RC, Graham BM, Jutzy KR, Shakudo M, Shah PM. Left ventricular outflow tract to left atrial communication secondary to rupture of mitral-aortic intervalvular fibrosa in infective endocarditis; diagnosis by transesophageal echocardiography and color flow imaging. J Am Coll Cardiol 1990;15:499-504. 6. Meyerowitz CB, Jacobs LE, Kotler MN, Ioli AW, Wertheimer JH. Four-year follow-up of a pseudoaneurysm of the mitral-aortic fibrosa. AM HEART J 1991;122:589-92. 7. Reid CL, McKay C, Kawanishi DT, Edwards C, Rahimtoola SH, Chandraratna PAN. False aneurysm of mitral-aortic intervalvular fibrosa: diagnosis by two-dimensional contrast echocardiography at cardiac catheterization. Am J Cardiol 1983;51:1801-2. 8. Gonzalez-Lavin L, Lise M, Ross D. The importance of the "jet lesion" in bacterial endocarditis involving the left heart. J Thorac Cardiovasc Surg 1970;59:185-92. 9. Bansal RC, Moloney PM, Marsa RJ, Jacobson JG. Echoeardiographic features of a mycotic aneurysm of the left ventricular outflow tract caused by perforation of mitral-aortic intervalvular fibrosa. Circulation 1983;67:930-4. 10. Katritsis D, Wegg-Peoloe M. Limitation of the coronary angiography: an underestimated problem? Clin Cardiol 1991;14:20-4. 11. MARCUS ML, Skorton DJ, Johnson MR, Colloins SM, Harrison DG, Kerber RE. Visual estimates of percent diameter coronary stenosis: "a battered gold standard." J Am Coll Cardiol 1988;11:882-5. 12. Isner JM, Kishel J, Kent KM, Ronan JA, Ross AM, Roberts WC. Accuracy of angiographic determination of left main coronary arterial narrowing: angiographic-histologic correlative analysis in 28 patients. Circulation 1981;63:1056-64. 13. Parashara DK, Jacobs LE, Ledly GS, Yazdanfar S, Oline J, Kotler MN. Intravascular ultrasound for angiographically indeterminant left main coronary artery disease. Echocardiography 1994;11:65-9. 14. Matuso S, Tsuruta M, Hayano M, Imamura Y, Eguchi Y, Tokushima T, Tsuji S. Phasic coronary artery flow velocity determined by doppler flowmeter catheter in aortic stenosis and aortic regurgitation. Am J Cardiol 1988;62:917-22.
Pearl
421
Familial occurrence of peripartum cardiomyopathy William Pearl, MD El Paso, Texas
Peripartum cardiomyopathy is a condition of unknown cause. It is initially seen as heart failure during the last month of pregnancy or within 5 months after delivery. Increased age, multiparity, twinning, and toxemia of pregnancy are risk factors. 1 A 24-year-old white woman of Mexican extraction had had progressive dyspnea on exertion since the delivery of her third child. She had increasing cardiomegaly and congestive heart failure. Seventeen days after delivery, she died in ventricular fibrillation from which she could not be resuscitated. Postmortem examination demonstrated a 520 gm heart with dilation of both ventricles, the left greater than the right. The coronary arteries did not have significant atherosclerosis. The myocardium demonstrated interstitial edema, minimal fibrosis, and no evidence of inflammation. Her family history was remarkable in that her mother died of a cardiomyopathy at 34 years of age 1 week after giving birth to her fifth child. The patient's only sibling, a sister, also died of a cardiomyopathy at age 26. She had congestive heart failure 2 days after giving birth to her third child, and she died 15 months later. Three previous reports 24 describe five kinships having more than one member with peripartum cardiomyopathy. Pierce et al. 2 reported that 3 of 17 patients with postpartum heart failure had a definite family history of the same condition. Massad et al. 3 reported a 16-year-old girl who had biopsy-proven cardiomyopathy after a molar pregnancy. Her sister underwent cardiac transplantation for peripartum cardiomyopathy. Voss et al. 4 reported on a patient who died of peripartum cardiomyopathy, as had her mother and two of her six sisters. The familial occurrence of peripartum cardiomyopathy suggests its cause has a genetic component. Dilated cardiomyopathy--unassociated with pregnancy--is often familial 5 and has been documented in the male relatives of women with peripartum cardiomyopathy.6 Unfortunately, it is not possible to retrospectively determine whether patients diagnosed as having peripartum cardiomyopathy were entirely healthy before becoming pregnant. Children and siblings of women with peripartum cardiomyopathy should be advised to have periodic echocardiograms, especially before a planned pregnancy.
From the Division of Pediatric Cardiology, University of Texas Medical Branch. Reprint requests: William Pearl, MD, Pediatric Cardiology, Children's Hospital, 301 University Blvd., Galveston, TX 77555-0367. AM HEARTJ 1995;129:421-2, Copyright © 1995 by Mosby-Year Book, Inc. 0002-8703/95/$3.00 + 0 4/4/60399
operative course. Careful multiple suprasternal and high parasternal sweeps document the anatomy of the vascular ring and the spatial relation of the vessels that comprise the ring. 4 Color flow Doppler makes it easier to follow the arterial branches and differentiate the veins from the arterial vessels of the upper mediastinum and base of the neck. Furthermore, Doppler echocardiography allows reliable assessment of the hemodynamics of this defect. The combined use of detailed two-dimensional echocardiographic sweeps and color flow Doppler should allow comprehensive anatomic and physiologic evaluation of the truncus arteriosus and any associated aortic arch anomaly. We thank Hewlett-Packard Co. (Andover, Mass.) for funding the publication of the color illustrations in this article.
REFERENCES
1. CalderL, Van Praagh R, Van Praagh S, Sears WP, CorwinR, LevyA, Keith JD, Paul MH. Truncus arteriosus communis:clinical,angiocardiographic, and pathologic findings in 100 patients. AM HEART J 1976;92:23-8. 2. HanleyFL, HeinemannMK, Jonas RA, MayerJE Jr., Cook NR, Wessel DL, Castaneda-AR.Repair of truncus arteriosus in the neonate.J Thorac Cardiovasc Surg 1993;105:1047-56. 3. Murdison KA, Andrews BA, Chin AJ. Ultrasonographicdisplay of complex vascular rings. J Am Cell Cardlol 1990;15:1645-53. 4. Butte F, Lucas RV Jr., Edwards JE. Persistent truncus arteriosus: pathologic anatomyin 54 cases. Pediatr Cardiol 1986;7:95-101. 5. Di DonateRM, FyfeDA, Puga FJ, DanielsonGK, Ritter DG, Edwards WD, McGoonDC. Fifteen-yearexperiencewith surgicalrepair of truncus arteriosus. J Thorac CardiovascSurg 1985;89:414-22. 6. BinetJP, LangloisJ. Aortic arch anomaliesin childrenand infants.J Thorac CardiovascSurg 1977;73:248-52.
Angina caused by systolic compression of the left coronary artery as a result of pseudoaneurysm of the mitral-aortic intervalvular fibrosa Deepak K. Parashara, MD, Larry E. Jacobs, MD, Morris N. Kotler, MD, Shahriar Yazdanfar, MD, Scott R. Spielman, MD, Sean F. Janzer, MD, and Charles E. Bemis, MD Philadelphia, Pa.
Pseudoaneurysm formation of the mitral-aortic intervalvular fibrosa (MAIVF) is a well-recognized complication of aortic valve endocarditis. 1 Mitral-aortic intervalvular fibrosa (MAIVF) pseudoaneurysm may rupture into the
From the Department of Medicine,Divisionof CardiovascularDiseases, Albert Einstein Medical Center, Temple UniversitySchool of Medicine. Supported in part by the Women'sLeague for Medical Research, Albert Einstein Medical Center. Reprmt requests: Larry E. Jacobs, MD, Divisionof Cardiology,Suite 331, KleinBuilding,AlbertEinsteinMedicalCenter,5401YorkRoad, Philadelphia, PA 19141. AMHEARTJ 1995;129:417-21. Copyright ® 1995 by Mosby-YearBook, Inc. 0002-8703/95/$3.00+ 0 4/4/60396
Parashara et al.
417
pericardium,2, 3 causing fatal cardiac tamponade, or may rupture into the left atrium, 1, 4, 5 causing mitral regurgitation. In some instances the pseudoaneurysm of MAIVF remains intact 6, 7 and appears as a pulsatile cavity with systolic expansion. We report on a patient with angina in the presence of an expanding pseudoaneurysm of the MAIVF after aortic valve replacement for aortic valve endocarditis. The presumed mechanism of angina was compression of the left coronary artery by the pseudoaneurysm of the MAIVF. A 59-year-old m a n with a history of peripheral vascular disease, percutaneous femoral artery angioplasty, and 40year history of two-pack cigarette smoking had sudden acute onset of nonexertional, substernal chest pain radiating to both shoulders. For the previous week the patient had had weakness, myalgia, anorexia, fever, night sweats, and upper respiratory tract symptoms. Physical examination revealed a temperature of 39 ° C, pulse rate of 72 beats/min and a blood pressure of 136/78 m m Hg. The skin showed no sign of needle marks, and throat examination was benign. There was no jugular venous distension, and carotid artery was 2+ palpable without bruit. The cardiac examination revealed a III/VI diastolic m u r m u r in the aortic area. The lung and abdominal examinations revealed nothing remarkable. The electrocardiogram during chest pain showed 1.5 m m ST-segment depression in the inferolateral leads that resolved with one sublingual nitroglycerine tablet. Myocardial infarction was ruled out with serial cardiac enzyme blood determinations. Transthoracic echocardiography revealed moderate to severe aortic regurgitation with hyperdynamic left ventricular function. Aortography demonstrated 4+ aortic regurgitation and a shaggy, irregular left coronary cusp. Coronary cineangiography showed normal coronary arteries without obstructive disease. Transesophageal echocardiography revealed a mobile shaggy mass measuring 1.5 cm attached to the left coronary cusp. Multiple blood cultures were positive for a-hemolytic streptococcus, and the patient was treated with intravenous vancomycin 1 gm every 12 hours. Results of a detailed work-up for source of infection were negative. The patient underwent aortic valve replacement surgery on the fifth day of hospitalization for intractable congestive heart failure. The aortic valve was trileaflet, with a 1.5 cm pedunculated mass present on the left coronary cusp with erosion into the annulus. The postoperative course was unremarkable, and intravenous antibiotic therapy was continued for 6 weeks. The patient was discharged home on oral anticoagulant therapy. Three months after his discharge from hospital, the patient complained of bilateral decrease in acuity of vision. Fundoscopic examination revealed evidence of bilateral retinal artery microembolization. Performed as part of the work-up for source of microemboli, transesophageal echocardiography demonstrated a 3.0 × 2.0 cm pseudoaneurysm of MAIVF, with linear mobile thin filamentous structures seen in the pseudoaneurysm. Persantine 25 mg thrice daily and aspirin 80 mg daily were added to treatment. No further visual symptoms were noted. Six months later the patient had typical exertional angina. An exercise thallium test was performed. The patient achieved
418 Par~hara
February 1995 American Heart Journal
e t al.
J STqESs
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l
, , ,
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~~. Tq~qLt. TU[q TF-qlr-J Fig. 1. Polar maps of exercise thallium-201 single-photon emission computed tomographic imaging at peak stress (top left) and after recovery (right) and reversibility computed (lower left). Orientation in images is left, septum; right, lateral left ventricular wall bottom, inferior left ventricular wail; top, anterior left ventricular wall.
85 % of the predicted maximal heart rate for his age with 7 METS workload, and he had typical anginal pain that was relieved with rest. The electrocardiogram was positive for ischemia in the inferolateral and anterior leads. Thallium scan showed extensive myocardial perfusion abnormalities involving the inferolateral, anteroseptal, and apical segments with complete redistribution (Fig. 1). Aortography performed in multiple projections revealed a 4 cm pulsatile donut-shaped pseudoaneurysm on the left side of the aortic root. In the left lateral projection the pseudoaneurysm was visualized on the left side of the aortic root, extending posteriorly under the left main coronary artery and left circumflex coronary artery but above the left atrium (Fig. 2). Coronary cineangiogram demonstrated that the left main coronary artery had no obstructive disease but was compressed to >50 % of its diameter during systole by the systolic expansion of the pseudoaneurysm (Fig. 3). The left circumflex coronary artery was free of disease, and a long proximal segment of the artery was completely occluded in systole by the expanding pseudoaneurysm. The left anterior descending coronary artery and the right coronary artery showed no obstructive disease, and there was no compression by the pseudoaneu-
rysm. Transesophageal echocardiogram demonstrated a 4.0 x 2.0 cm pulsatile donut-shaped pseudoaneurysm of MAIVF on the left side of the aortic root below the left atrium and between the left ventricular outflow tract and the main pulmonary artery (Fig. 4). The left main coronary artery and left circumflex artery were seen to be obliterated with systolic expansion of pseudoaneurysm against the main pulmonary artery. Surgery was performed at another institution. The angiographic and transesophageal findings were confirmed. The pseudoaneurysm was closed, and the aortic annulus was sutured to the St. Jude valve. On the seventh postoperative day, the patient suddenly collapsed in the bathroom and had cardiac arrest. An autopsy performed at the other institution showed severe s~enosis of the left main coronary artery caused by intimal fibroplasia and total occlusion with recent thrombus formation. A large recent myocardial infarction was also present. Aortic valve endocarditis can produce perforation and destruction of the aortic leaflet, which leads to severe acute aortic regurgitation that generally results in hemodynamic deterioration and left ventricular failure, s The aortic regurgitant jet emanating from the infected aortic valve directed toward the subaortic structures may cause seeding
Volume 129, Number 2
419
Parashara et aI.
American Heart Journal
.
d
~.~:
•
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F i g . 2. A, Aortogram performed in left lateral projection in systole shows systolic expansion of pseudoaneurysm of the MAIVF with complete obliteration of proximal left circumflex coronary artery. B, Aortogram performed in left lateral projection in diastole shows normal patency of proximal left circumflex coronary artery with pseudoaneurysm of MAIVF.
Fig. 3. A, Left coronary artery cineangiogram in left lateral projection in systole shows 60 % narrowing of distal left main coronary artery and complete obliteration of proximal left circumflex coronary artery. B, Left coronary artery cineangiogram in left lateral projection in diastole shows normal patency of proximal left main coronary artery and left circumflex coronary artery.
of infection of the subaortic structures. 1, s Alternatively, the subaortic structures may be infected by direct extension of the infection from the infected aortic valve. 1, s Anatomically, the subaortic structures consist of an anterior mitral leaflet, which lies in a somewhat medial position subjacent to the posterior aortic root, 1, 3 and the MAIVF, a fibrous interannular tissue between the base of the anterior mitral leaflet and the left and noncoronary aortic cusp.l, 4 The MAIVF is relatively avascular and offers little resistance to infection. Infection is the most common cause of pseudoaneurysm of MAIVF. Blunt chest trauma has also
been implicated as a cause of the MAIVF pseudoaneurysm, 9 and it has been suggested that MAIVF is congenitally weakened in these patients. 2 Infection of the MAIVF may result in formation of abscess or pseudoaneurysm or a direct communication from left ventricular outflow into the left atrium. 1 A review of the literature shows that pseudoaneurysm of the MAIVF may rupture into the pericardial sac and result in fatal hemopericardium. 2, 3 It may rupture into the left atrium with systolic shunting of the blood from the left ventricular outflow tract into the left atrium 4, 5 with resultant supraannular mitral regurgitation
420
February 1995 American Heart Journal
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Fig. 4. A, Transesophageal echocardiography (TEE) in transverse plane shows relation of pseudoaneurysm of MAIVF to left atrial appendage (APP), left atrium (LA), and St. Jude aortic prosthetic valve (AO V). B, Transesophageal echocardiography in longitudinal plane shows pseudoaneurysm of MAIVF in relation to left ventricular outflow tract (LVOT) and main pulmonary artery (PA). LV, Left ventricular.
and an eccentric jet directed toward the lateral left atrial wall. 1 This pseudoaneurysm may remain intact, 6, 7 appearing as an echo-free cavity in systole, posterior to the aortic root. In our patient, we angiographicaUy demonstrated systolic compression of the left main coronary artery to >50 % of its diameter and complete occlusion of the left circumflex artery by the pseudoaneurysm of the MAIVF. Systolic compression of coronary arteries occurred as a result of systolic expansion of the pseudoaneurysm impinging on the left main artery and the left circumflex coronary artery against the main pulmonary artery. The physiologic significance of the observed compression of coronary arteries resulted in typical exertional anginal symptoms and the
demonstration of positive exercise electrocardiogram and extensive ischemia on exercise thallium-201 single-photon emission scintigraphy. The exact mechanism of ischemia caused by systolic compression of the coronary artery with pseudoaneurysm of the mitral aortic intravalvutar fibrosa is unclear. It is likely that beat-to-beat systolic impingement led to fibroplasia of the left main coronary artery and subsequent thrombus formation, culminating in the untimely death of this patient. Angiography failed to visualize severe stenosis of the left main artery due to intimal fibroplasia as confirmed by autopsy. This is consistent with prior reports, which demonstrate that angiographic determination of the percentage of stenosis of the left main coronary artery are often inaccurate and imprecise, l°-12 Had
Volume 129, Number 2 American Heart Journal
intravascular ultrasound of the left main coronary artery been performed, it is likely that the intravascular ultrasound would have confirmed left main stenosis. 13 In addition, systolic compression of the left main coronary artery may have further aggravated ischemia in our patient by the following mechanism. The normal pattern of the epicardial coronary blood flow is characterized by predominance of flow during diastole. In the presence of significant aortic regurgitation, the systolic coronary blood flow increases and diastolic coronary blood flow decreases. 14 Thus in our patient, who had 4+ aortic regurgitation resulting in increased dependence on systolic coronary flow, systolic compression of the left main coronary artery may well have further exacerbated ischemia. REFERENCES
1. Karalis DG, Bansal RC, Hauck AJ, Ross J J, Applegate PM, Jutzy KR, Mintz GS, Krishnaswamy C. Transesophageal echocardiographic recognition of subaortic complication in aortic valve endocarditis. Circulation 1992;86:353-62. 2. Qizibash 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;32:110-3. 3. Chesler E, Korns ME, Porter GE, Reyes CN, Edwards JE. False aneurysm of the left ventricle secondary to bacterial endocarditis with perforation of the mitral-aortic intervalvular fibrosa. Circulation 1968;37: 518-23. 4. Schwartz DR, Belkin RN, Pucillo AL, Burleson PD, Fish BG, Polley RW, Weiss MB, Herman MV. Aneurysm of the mitral-aortic fibrosa complicating infective endocarditis: preoperative characterization by two-dimensional and color Doppler echocardiography, magnetic resonance imaging, and cineangiography. AM HEART J 1990;119:196-9. 5. Bansal RC, Graham BM, Jutzy KR, Shakudo M, Shah PM. Left ventricular outflow tract to left atrial communication secondary to rupture of mitral-aortic intervalvular fibrosa in infective endocarditis; diagnosis by transesophageal echocardiography and color flow imaging. J Am Coll Cardiol 1990;15:499-504. 6. Meyerowitz CB, Jacobs LE, Kotler MN, Ioli AW, Wertheimer JH. Four-year follow-up of a pseudoaneurysm of the mitral-aortic fibrosa. AM HEART J 1991;122:589-92. 7. Reid CL, McKay C, Kawanishi DT, Edwards C, Rahimtoola SH, Chandraratna PAN. False aneurysm of mitral-aortic intervalvular fibrosa: diagnosis by two-dimensional contrast echocardiography at cardiac catheterization. Am J Cardiol 1983;51:1801-2. 8. Gonzalez-Lavin L, Lise M, Ross D. The importance of the "jet lesion" in bacterial endocarditis involving the left heart. J Thorac Cardiovasc Surg 1970;59:185-92. 9. Bansal RC, Moloney PM, Marsa RJ, Jacobson JG. Echoeardiographic features of a mycotic aneurysm of the left ventricular outflow tract caused by perforation of mitral-aortic intervalvular fibrosa. Circulation 1983;67:930-4. 10. Katritsis D, Wegg-Peoloe M. Limitation of the coronary angiography: an underestimated problem? Clin Cardiol 1991;14:20-4. 11. MARCUS ML, Skorton DJ, Johnson MR, Colloins SM, Harrison DG, Kerber RE. Visual estimates of percent diameter coronary stenosis: "a battered gold standard." J Am Coll Cardiol 1988;11:882-5. 12. Isner JM, Kishel J, Kent KM, Ronan JA, Ross AM, Roberts WC. Accuracy of angiographic determination of left main coronary arterial narrowing: angiographic-histologic correlative analysis in 28 patients. Circulation 1981;63:1056-64. 13. Parashara DK, Jacobs LE, Ledly GS, Yazdanfar S, Oline J, Kotler MN. Intravascular ultrasound for angiographically indeterminant left main coronary artery disease. Echocardiography 1994;11:65-9. 14. Matuso S, Tsuruta M, Hayano M, Imamura Y, Eguchi Y, Tokushima T, Tsuji S. Phasic coronary artery flow velocity determined by doppler flowmeter catheter in aortic stenosis and aortic regurgitation. Am J Cardiol 1988;62:917-22.
Pearl
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Familial occurrence of peripartum cardiomyopathy William Pearl, MD El Paso, Texas
Peripartum cardiomyopathy is a condition of unknown cause. It is initially seen as heart failure during the last month of pregnancy or within 5 months after delivery. Increased age, multiparity, twinning, and toxemia of pregnancy are risk factors. 1 A 24-year-old white woman of Mexican extraction had had progressive dyspnea on exertion since the delivery of her third child. She had increasing cardiomegaly and congestive heart failure. Seventeen days after delivery, she died in ventricular fibrillation from which she could not be resuscitated. Postmortem examination demonstrated a 520 gm heart with dilation of both ventricles, the left greater than the right. The coronary arteries did not have significant atherosclerosis. The myocardium demonstrated interstitial edema, minimal fibrosis, and no evidence of inflammation. Her family history was remarkable in that her mother died of a cardiomyopathy at 34 years of age 1 week after giving birth to her fifth child. The patient's only sibling, a sister, also died of a cardiomyopathy at age 26. She had congestive heart failure 2 days after giving birth to her third child, and she died 15 months later. Three previous reports 24 describe five kinships having more than one member with peripartum cardiomyopathy. Pierce et al. 2 reported that 3 of 17 patients with postpartum heart failure had a definite family history of the same condition. Massad et al. 3 reported a 16-year-old girl who had biopsy-proven cardiomyopathy after a molar pregnancy. Her sister underwent cardiac transplantation for peripartum cardiomyopathy. Voss et al. 4 reported on a patient who died of peripartum cardiomyopathy, as had her mother and two of her six sisters. The familial occurrence of peripartum cardiomyopathy suggests its cause has a genetic component. Dilated cardiomyopathy--unassociated with pregnancy--is often familial 5 and has been documented in the male relatives of women with peripartum cardiomyopathy.6 Unfortunately, it is not possible to retrospectively determine whether patients diagnosed as having peripartum cardiomyopathy were entirely healthy before becoming pregnant. Children and siblings of women with peripartum cardiomyopathy should be advised to have periodic echocardiograms, especially before a planned pregnancy.
From the Division of Pediatric Cardiology, University of Texas Medical Branch. Reprint requests: William Pearl, MD, Pediatric Cardiology, Children's Hospital, 301 University Blvd., Galveston, TX 77555-0367. AM HEARTJ 1995;129:421-2, Copyright © 1995 by Mosby-Year Book, Inc. 0002-8703/95/$3.00 + 0 4/4/60399