Embolization of the Anterior Leaflet of the Mitral Valve

diac status was markedly improved from preoperative class 4 to class 2 functional capacity five months after surgery.

Embolization of the Anterior Leaflet of the Mitral Valve*

ACKNOWLEDGMENT: We wish to thank Drs. Ellis Samols and Maurice Nataro, Department of Nuclear Medicine, Veterans Administration Hospital, Louisville, Kentudcy, for performing the radioisotope angiocardiograms.

Benedict S. Maniscalco, M.D., -- DarreD R. Caudill, M.D., and Iverson W. Joines. M.D.

1U:FERENCES 1 Hope J: Diseases of the Heart and Great Vessels, Ed. 3 London, John Churchill, 1839 2 Thurnam J: On aneurysms and especially spontaneous varicose aneurisms of the ascending aorta and sinuses of Valsalva. Trans Med Chir Soc Edinburgh 23:323-384, 1840 3 Jones AM, Langley FA: Aortic sinus aneurysms. Br Heart J 11 :325-341, 1949 4 Oram S, East T: Rupture of aneurysm of aortic sinus (of Valsalva) into the right side of the heart. Br Heart J 17 :541-551, 1955 5 Aletras H, Bjork YO, Cullhed I, et al: Ruptured congenital aneurysm of the sinus of Valsalva with ventricular septal defect. Thorax 18:127-135,1963 6 Perloff JK: Sinus of Valsalva-right heart communications due to congenital aortic sinus defects. Am Heart J 59:318-

321, 1960

7 Kieffer SA, Winchell P: Congenital aneurysms of the aortic sinuses with cardioaortic fistula. Dis Chest 38: 7996,1960 8 Abbott ME: Clinical and developmental study of a case of ruptured aneurysm of the right anterior aortic sinus of Valsalva. In Contributions to Medical and Biological Research (dedicated to Sir William Osler). New York, Paul B. Hoeber, 1919, 914 9 Venning GR: Aneurysms of the sinuses of Valsalva. Am Heart J 42:57-69, 1951 10 Edwards JE, Burchell HB: The pathological anatomy of deficiencies between the aortic root and the heart, including aortic sinus aneurysms. Thorax 12: 125-139, 1957 11 Bjork VO, Bjork L: Aneurysm of the sinus of Valsalva. J Thorac Cardiovasc Surg SO:16-21, 1965 12 Holmes' EC, Bredenburg CE, Brawley RK: Aneurysm of the sinus of Valsalva resulting from bacterial endocarditis. Ann Thorac Surg 15:628-631,1973 13 Besterman EMM, Ooldberg MJ, Sellors TH: Surgical repair of ruptured sinus of Valsalva. Br Med J 2:410-416, 1963 14 Wood P: Disease of the Heart and Circulation. London, Eyre and Spotteswoode, 1950, 524 15 Shepherd SS, Park FR, Kitchell JR: A case of aortopulmonic communication incident to a congenital aortic septal defect: Discussion of embryologic changes involved. Am Heart J 27 :733-738, 1944 16 Nicholson RE: Syndrome of rupture of aortic aneurysm into pulmonary artery: Review of the literature with report of two cases. Ann Int Med 19:286-325, 1943 17 Sawyers JL, Adams JE, Scott HW, Jr: Surgical treatment of aneurysms of the aortic sinuses with aorticoatrial fistula: Experimental and clinical study. Surgery 41 :26-42, 1957 18 Lillehei CW, Stanley P, Varco RL: Surgical treatment of ruptured aneurysms of the sinus of Valsalva. Ann Surg 146:459-472, 1957 19 Taguchi K, Sasaki N, Matsuura Y, et al: Surgical correction of aneurysm of the sinus of Valsalva. A report of fortyfive consecutive patients including eight with total replacement of aortic valve. Am J Cardiol 23:180-191, 1969

CHEST, 65: 5, MAY. 1974

p.

A youag WOlllD pre8eIlted to OlD' bo8pital ID florid IIlOIUU'y edema and with the stiplata of acute bacterial endocarditis. Murmun of mitnI reaurgibltloa . .d 80rtk reaurgitatloa were present. WbiIe on IIDtIhiotk therapy, the patient developed sips and symptoms of left femoral artery occlusion. ~ ~nmlDatioD revealed that the specimen removed at embolectomy contalDed tiline from the mitnlI valve. Prompt surgkallDterventloD with mitral valve replacement required ID attempting to correct the hemodynamic derangements.

w.

emboli are a well-recognized feature of bacSystemic terial endocarditis, and usually consist of vegetations,

clot, or calcium fragments. We recently saw a patient who experienced sudden peripheral arterial occlusion secondary to embolization of the anterior leaflet of the mitral valve itself, a manifestation not previously described in the medical literature. CASE REPoRT

A 35-year-old woman was admitted to Georgia Baptist Hospital with a two-week history of malaise and fatigability. Four days prior to admission, she experienced the onset of increasing dyspnea, orthopnea, paroxysmal nocturnal dyspnea, and hemoptysis. The history was unremarkable for weight loss, jaundice, melena, hematuria, skin rashes, joint symptoms, or recent dental procedures. Although she was known to have a heart murmur since age 18, there was no evidence of prior rheumatic fever and she had never been placed on prophylactic antibiotics. One day prior to admission, she received a penicillin injection from her family physician. Admission examination revealed a young woman in severe respiratory distress with a respiratory rate of SO/min. The blood pressure was 114/64 mm Hg, the pulse was regular at 140 beats/min, and the temperature was 98.6- F. Splinter hemorrhages were present in the nail beds;" petechiae were present in the mucous membranes, soft palate, conjunctivae, and skin of the upper and lower extremities. Roth spots were observed in the optic fundi. The neck veins were markedly distended and a prominent dicrotic notch was palpable in the carotid artery. Crepitant rAles were heard in all lungfields. The precordium was hyperdynamic. The first heart sound was soft and the pulmonic closure sound was accentuated. A grade 3/6 holosystolic murmur was present at the apex with radiation to the axilla, the dorsal spine, and the cranium; a loud summation gallop was also present. No extraeardiac sounds were heard. The liver and spleen were not felt. There was 2+ pitting edema of the lower extremities. All peripheral pulses were moderately accentuated. The neurologic examination was unremarkable. After obtaining blood cultures (which subsequently grew Streptococcus viridans), antimicrobial therapy was begun -From the Departments of Medicine, Emory University School of Medicine (Division of Cardiology) and Georgia Baptist Hospital and the Atlanta Heart and Lung CliDic, Atlanta. --Fellow in Medicine (Cardiology) Emory University School of Medicine. Reprint requests: Dr. Caudill, 272 Boulevard, NE, Atlanta 30312

EMBOLIZATION OF ANTERIOR LEAFLET OF MITRAL VALVE 571

FIGURE 1. Injection of dye into the left ventricle ( LV) reveals severe regurgitation of the dye into the left atrium ( LA). The arrow points to the prolapsed anterior leaflet of the mitral valve.

with 20 million units of intravenous penicillin and 2 grams of intramuscular streptomycin per 24 hours. The pulmonary edema was vigorously treated with digitalis and diuretics and subsequently a murmur of aortic regurgitation was heard. On the sixth hospital day, the patient underwent cardiac catheterization. Mild to moderate aortic regurgitation was demonstrated with injection of the aortic root. Left ventriculography demonstrated good left ventricular function, severe mitral regurgitation and massive dilatation of the left atrium. The anterior leaflet of the mitral valve prolapsed into the left atrium with ventricular systole (Fig 1). On the 11th hospital day, the patient suddenly developed signs and symptoms of left femoral artery occlusion. An emergency embolectomy was performed without complication. At surgery the common femoral artery was acutely

FIGURE 3. Mitral Valve (1.5s) This photograph of the mitral valve shows distortion of shortening and thickening with fusion of chordae tendineae consistent with rheumatic valvular disease. The bacterial endocarditis has produced a circular defect in the anterior leaflet which is partially covered by the arching dome-like incomplete aneurysm. The margins of the aneurysm are soft and pulpy with an irregular pattern. The definitive site of origin of the embolic fragment is not identified.

inflamed with considerable local edema. A large (3 em x 1 em gray-red organized clot was removed with immediate restoration of excellent forward and backward How. Subsequent pathologic examination demonstrated that the surgical specimen contained tissue from the mitral valve (Fig 2). The following day she underwent surgery and mitral valve replacement. The mid-portion of the anterior leaflet of the mitral valve was deformed by a large perforated aneurysm. Along the free edge of the perforation an area of discontinuity of tissue was noted and felt to represent the embolized portion of the mitral valve. Small vegetations were evident on the aneurysm, free edges of the anterior and posterior leaflets, and some chordae tendinae. Mild shortening and thickening of the chordae was also present (Fig 3). The postoperative course was uncomplicated until the 14th hospital day when she developed myoclonic seizures and a diffuse petechial rash. A clinical diagnosis of systemic fat emboli complicated by disseminated intravascular coagulopathy was made and therapy was begun with intravenous heparin and high dose steroids. A left hemiparesis became apparent the following day. On the 16th hospital day the murmur of aortic regurgitation was louder, the BUN rose to 109 mg percent, and the temperature rose to 104· F. She remained clinically stable and began to show signs of neurologic improvement until she unexpectedly suffered respiratory arrest on the 20th hospital day. With progressive neurologic deterioration, the patient died on the 22nd hospital day. DISCUSSION

FIGURE 2. The comparison of the fragment obtained from embolectomy (A, upper) and a portion of the aneurysm of the mitral valve (B, lower) reveals the similar endothelial covering, laminated collagenous bundIes and cellularity that allows the conclusion that the embolic fragment had its origin from the aneurysm of the mitral valve. 2A-Embolus x50 Massons; 2B-Aneurysm xl28 Massoos'.

512 MANISCALCO, CAUDill, JOINES

Peripheral arterial embolization is a common occurrence in acute bacterial endocarditis.t'? Hickie" reported 279 patients with bacterial endocarditis and 131 deaths, 54 of which were due to systemic embolization. Buckbinder and Roberts- reported an autopsy series of left sided valvular endocarditis with gross infarcts in 29 (65 percent) of the 45 patients. The left sided location and friable nature of endocardial vegetations are the key factors responsible for systemic emboli in acute bacterial endocarditis. Coulshed and eolleages- emphasized the significance of embolism

CHEST, 65: 5, MAY, 1974

in mitral valve disease. In a series of 839 cases, 18.7 percent had systemic emboli. Conclusions from this large series were: 1) atrial fibrillation is the main cause of systemic embolism; 2) embolism is as common with mitral regurgitation as with mitral stenosis; 3) mitral valve calcification predisposes to operative emboli; 4) atrial appendage size has no influence on the occurrence of systemic embolism. Review of the literature has not revealed a reported case of peripheral migration of the mitral valve. That this may occur with endocarditis and aneurysm formation of the mitral valve is easily appreciated when the pathogenesis of valve aneurysm formation is reviewed. 5 As a result of valvulitis, granulation and scar tissue are exposed to a constantly maintained intraventricular pressure with dilatation and protrusion of the valve into the left atrium. The aneurysm may obstruct the mitral orifice producing mitral stenosis or perforate, causing incompetence. Perforation of the aneurysm allows embolization of valve fragments, vegetations and clots. The aneurysms characteristically 'involve the anterior leaflet of the mitral valve, particularly in the presence of aortic regurgitation." The serious hemodynamic changes attendant with acute mitral regurgitation due to bacterial endocarditis demand judicious medical management directed to control of congestive heart failure and sterilization of the infective process. Prompt surgical intervention must be considered if response to the measures is not achieved. The prudence of removing the nidus of infection as well as hemodynamic correction by surgical means is well documented. 7 - 9 ACKNOWLEDGMENTS: The authors wish to express their appreciation to Dr. William C. Maloy, Director, Cardiac Catheterization laboratory, Georgia Baptist Hospital, for supplying the catheterization data and cineangiographic pictures; to Dr. Lester Forbes, Department of Pathology Georgia Baptist Hospital, for preparation and evaluation of the pathologic tissue specimens; and to Drs. Gerald F. Fletcher and John D. Cantwell for their critical reviews and helpful suggestions in the preparation of this manuscript.

fu:FERENCES 1 Buckbinder NA, Roberts WC: Left-sided valvular active infective endocarditis. Am J Med 53:20-35, 1972 2 Hickie JD: Bacterial endocarditis in Sydney, 1950-1959. Med J Aust 1:929, 1961 3 Morgan WI.., Bland EF: Bacterial endocarditis in the antibiotic era, with special reference to the later complications. Circulation 19:753, 1959 4 Coulshed W, Epstein EJ, McKendrick CS, et al: Systemic embolism in mitral valve disease. Br Heart J 32:26-34, 1970 5 Saphir 0, Leroy EP: True aneurysms of the mitral valve in subacute bacterial endocarditis. Am J Pathol24:83, 1948 6 Symbas PN, Baldwin BJ, Schlant RC, et al: Unusual complications of bacterial endocarditis. Br Heart J 33:664670,1971 7 Wallace AG, Young WG Jr, Osterhout S: Treatment of acute bacterial endocarditis by valve excision and replacement. Circulation 31:450, 1965 8 Windsor HM, Golding LA, Shanahan MX: Cardiac surgery in bacterial endocarditis. J Thorac Cardiovasc Surg 64:282290, 1972 9 Windsor HM, Shanahan MS: Emergency valve replacement in bacterial endocarditis. Thorax 22:25-53, 1967

CHEST, 65: 5, MAY, 1974

Right Coronary Arterial Spasm Causing Prinzmetal's Variant Angina * John S. Schroeder, M.D.,·· James·F. Silverman, M.D.,··

and Donald C. Harrison, M.D.t

Severe spasm of the right coronary artery "'. documeDteel during the second of three Injections duriDg coronary arteriography in a 42-year-old man with a five-year history of Prinzmetars variant angina. Only mild atherosclerosis « 25 percent Darrowlng) of the artery before and after the spasm "'. noted. Repeat coronary arteriography two years later confirmed the minimal disease In tbe right coronary artery, despite continned occasional severe disabDity from angina. NitroglyceriD Invariably relieveel the pain and isosorblde cIinitnde takeD prophylactically' _ been very effective in preventing episodes of Docturnal angina. These 0bservations snpport the concept that coronary spasm is one etiology of ischemic pain In variant angina. pathophysiology of Prinzmetal's variant angina' T heremains unclear since he initially proposed increased

tonus of a large narrowed coronary artery as the precipitating cause. 1 The significant features of this variant form of angina include its occurrence at rest, frequently on a cyclical basis during the day or night, and not precipitated by exercise. The ECG during chest pain shows marked ST elevation rather than ST depression. Hemodynamic observations in a small patient group have confirmed that increased myocardial work is not the apparent etiology for these transient anginal attacks which are accompanied by a reversible myocardial injury pattern on the electrocardiogram." Review of recent studies of coronary arteriography in variant angina pectoris patients shows two apparent patient groups: one group with severe proximal occlusive coronary artery disease and the other with normal or minimally diseased coronary vessels. 3 - 8 The purpose of this report is to document a case of Prinzmetal's variant angina secondary to diffuse spasm of the right coronary artery with longterm follow-up and repeat arteriography. Its possible relationships to other forms of r~st angina with and without ST elevation will be discussed. CASE

REPoRT

A 42-year-old Negro man was admitted to Stanford University Hospital in May, 1972, because of increasing severity and frequency of crushing substernal chest pain. He had noted occasional pain, primarily nocturnal, since 1966. The pain usually resolved spontaneously after awakening and had never occurred with exertion. In the several months prior to admission he had noted an increasing number of attacks while asleep, ranging from two to ten per night, in addition to episodes while resting. Several episodes of chest pain oc·From the Divisions of Cardiology and Radi~, Stanford University School of Medicine, Stanford, . omia. This work was supported in part by NIH Grants Nos. HL-5709 and HL-5866. ••Assistant Professor of Medicine. tWilliam G. Irwin Professor of Medicine. Reprint requests: Publications Editor, 701 Welch Road, Suite 3303, PaloAlto, California94304

RIGHT CORONARY ARTERIAL SPASM 513