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Chrousos G. Churg-Strauss syndrome: development of cardiomyopathy during corticosteroid therapy. Am J Dis Child 1982;136:339-44. Davison AG, Thompson PJ, Davies J, Corrin B, Turner-Warwick M. Prominent-pericardial and myocardial lesions in the Churn-Strauss svndrome. Thorax 198338793-5. Wein&ein JM, dhui H, Lane S, Corbett J, Totighi J. ChurgStrauss syndrome (allergic granulomatosis and angiitis): neuro-ophthalmologic manifestations. Arch Ophthalmol 1983; 101:1217-20. Finan MC, Winkelmann RK. Cutaneous extravascular necrotizinz granuloma and lymphocytic lymphoma. Arch Dermatol 1983;119:419-22. Finan MC. Winkelmann RK. The cutaneous extravascular necrotizing granuloma (Churg-Strauss granuloma) and systemic disease: a review of 27 cases. Medicine 1983;62:142-58. Tai P, et al. Deposition of eosinophil cationic protein in granulomas in allergic granulomatosis and vasculitis: the ChurgStrauss svndrome. Br Med J 1984:289:400-2. Lanham “JG, et al. Systemic vasculitis with asthma and eosinophilia. Medicine 1984;63:65-81. Lanham JG, et al. Endomyocardial complications of ChurgStrauss svndrome. Posterad Med J 1985:6:341-4. Kus J, et-al. Lymphocyti subpopulations in allergic granulomatosis (Churg-Strauss syndrome). Chest 1985;87:826-7. Shields CL, et al. Conjunctival involvement in Churg-Strauss syndrome. Am J Ophthalmol 1986;102:601-5. Oh SJ, et al. Eosinophilic vasculitic neuropathy in the ChurgStrauss syndrome. Arthritis Rheum 1986;29:1173-5. Jessurun J, et al. Allergic granulomatosis and angiitis (ChurgStrauss syndrome): report of a case with massive thymic involvement in a nonasthmatic patient. Hum Path01 1986; 17:637-g. Henochowicz S, et al. Necrotizing systemic vasculitis with features of both Wegener’s granulomatosis and Churg-Strauss vasculitis. Arthritis Rheum 1986;29:565-9. MacFayden R, et al. Allergic angiitis of Churg and Strauss syndrome: response to pulse methylprednisolone. Chest i987;91:629-31: Gilbert GJ. Churg-Strauss syndrome: determining adequacy of steroid dosage bv the eosinonhil count. South Med J 1987; 80~266-8.

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Churg J, Strauss L. Allergic granulomatosis: allergic angiitis and periarteritis nodosa. Am J Path01 1951:27:27. 18. Frayha RA, Churg-Strauss syndrome in a child. J Rheumatol 1982;9:807-9. 19. Conn DL, et al. The association of Churg-Strauss vasculitis with temporal arteritis involvement, primary biliary cirrhosis, and polychondritis in a single patient. J Rheumatol 1982;9: 17.

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Rosenberg TF, et al. Allergic granulomatous antiitis (ChurgStrauss syndrome). J Allergy Clin Immunol 1975;55:56-67. 21. Chumbley LC, et al. Allergic granulomatosis and angiitis (Churg-Strauss syndrome): report and analysis of 30 cases. Mayo Clin Proc 1977;52:477-84.

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Persistent left superior vena cava: Unusual wave contour of left jugular vein as the presenting feature Paul Spearman, MD, and Carl V. Leier, Columbus, Ohio

MD.

From the Division of Cardiology, Department Ohio State University College of Medicine.

of Internal

Reprint requests: Hall, 1654 Upham

of Cardiology,

4/4/22915

Carl V. Leier, Dr., Columbus,

MD, Division OH 43210.

Medicine,

The

669 Means

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The persistence of a left superior vena cava is a congenital anomaly present in 0.3 % to 0.5 % of the general population. The diagnosis should be suspected when the left jugular vein is distended to a higher level than the right,‘, 2 although the diagnosis is usually made at cardiac catheterization or during cardiac surgery. The presence of this congenital anomaly may also be suspected when a dilated coronary sinus is noted on two-dimensional echocardiography and is confirmed by contrast echocardiographic techniques.3 We describe a patient with a persistent left superior vena cava who presented with an unusual wave contour of the left jugular vein. We propose that certain characteristics of the left jugular waveform are indicative and probably pathognomonic for the presence of a persistent left superior vena cava. A 64-year-old man with a history of tachyarrhythmias was hospitalized for alteration of antiarrhythmic therapy. The patient had a regular pulse of 84 beatslmin and a blood pressure of 102/74 mm Hg. At an angulation of 30 degrees, the left jugular pulsations were more prominent than the right, and demonstrated at least four distinct waves. The right jugular vein moved with A and V waves. A left paramediastinal venous crescent was present on chest x-ray film. Cardiac catheterization demonstrated a persistent left superior vena cava continuous with an enlarged coronary sinus draining into the right atrium (Fig. 1, A). The posterior left atria1 wall was contiguous with the left superior vena cava (Fig. 1, B). Left atria1 systole clearly indented the left superior vena cava. In addition, angiographic dye was noted to reverse its direction within the anomalous vessel (i.e., moving cephalad) during ventricular systole. The simultaneous right and left jugular venous pulse recordings are shown in Fig. 2, A. The right jugular tracings show A and V waves and a small C wave. The leftsided tracings demonstrate four distinct waves, as well as a smaller fifth wave at the bottom of the X descent. The C and V waves of the left-sided tracing coincide with their right-sided counterparts. There are two waves in the leftsided tracing that occur simultaneously with the rightsided A wave; these have been designated A and A’. The fifth wave (following the C wave) on the left has no rightsided counterpart, and conincides with ventricular contraction (Fig. 2, B). The A wave is produced by rising pressure in the right atrium accompanying atria1 contraction. The occurrence of a double A wave on the left represents the time delay in the left atria1 contraction,4 such that the initial A wave on the left is produced by contraction of the right atrium, while a second wave (A’) is produced by left atria1 contraction (Fig. 2). In the patient we describe, the expression of the A’ wave in the left jugular pulse is a manifestation of the direct transmission of the left atria1 contraction to the dilated coronary sinus-left superior vena cava system because of the close anatomic relationship (Fig. 1, B). The right venous system meanwhile senses only the “normal” atrial contraction. The origin of the small venous wave following the C wave is less clear. This systolic wave is likely produced by the effect of ventricular contraction on the markedly enlarged coronary sinus. We were able to demonstrate angiographically compression of the enlarged coronary sinus with ventricular systole, causing a temporary

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Brief Communications

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October 1990 Heart Journal

Fig. 1. A, Angiographic demonstration of the enlarged coronary sinus(C’S)communicating with the persistent left superior vena cava (arrow). B, Indentation of the left superior vena cava by the posterior wall of the left atrium (arrow).

reversal of flow (cephalad) in the left superior vena cava. This explanation is further supported by the simultaneous recording of the left jugular wavesand the apexcardiogram (Fig. 2, B). The existenceof a left superior vena cava draining into the coronary sinusdoesnot of itself result in any hemodynamic compromise,as venousblood is returned appropriately to the right atrium. Still, the identification of this anomaly is important for several reasons.A left superior vena cava is often accompaniedby other congenital anomalies such as atria1 septal defect, anomalouspulmonary venous return, and tetralogy of Fallot.5v6 Electrical instability and arrhythmias have been associated with this entity.7 Technical difficulties in venous-to-cardiac catheterization may occur whenthe procedureisattempted from the left jugular, subclavian, or arm veins. A far higher incidence of supraventricular ectopy and tachycardia may

occur during the procedure.” Finally, serioustechnical difficulties may be encountered during open-heart surgery if the existence of a left superior vena cava is not suspected preoperatively.5 The presenceof a persistent left superior vena cava can be suspected on the basis of a characteristic physical examination and radiologic findings. Jugular veins that are more prominent on the left are a useful physical sign and a left paramediastinal venous crescentis often present on the chest x-ray film. The patient presented herein manifested both of thesefindings, but also showeda markedly different pattern of jugular venouswaves on the left compared with the right. It is possiblethat these neck vein findings may be present in only a subsetof patients with a persistent left superior vena cava. Nevertheless, when present they represent important if not pathognomonic evidence for the presenceof this congenital anomaly.

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Fig. 2. A, Simultaneousrecording of the left (LJ) and right (RJ) jugular wave forms. Asterisk marks the fifth wave noted on the left jugular recording. Seetext for description. B, Simultaneousrecording of the left jugular vein (LJ) and left ventricular apex (Apex). Asterisk marks the fifth wave noted on the left jug-

ular recording. See text for description.

REFERENCES

1. Coleman AL. Diagnosis of left superior vena cava by clinical inspection, a new physical sign. AM HEART J 1967;73:115-20. 2. Horwitz S, Esquival JA, Attie F, Lupi EH, Espino-Vela J.

Clinical diagnosis of persistent left superior vena cava by observation of jugular pulses. AMERICAN HEART JOURNAL 1973; 86:759-63. 3. Armstrong W. Congenital heart disease. In: Feigenbaum H, ed.

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Echocardiography. 4th ed. Philadelphia: Lea & Febiger, 1986:367. Luisada AA, Singhal A, Kim K. The jugular and hepatic tracings in normal subjects and in conduction defects. Acta Cardiol 1983;38:405-24. Gensini GG, Caldini P, Casaccio F, Blount SG. Persistent left superior vena cava. Am J Cardiol 1959;4:677-85. Fraser RS, Dvorkin J, Rossall R, Eiden R. Left superior vena cava. A review of associated congenital heart lesions, catheterization data and roentgenologic findings. Am J Cardiol 1961;31:711-16. James TN, Marshall TK, Edwards JE. Cardiac electrical instability in the presence of a left superior vena cava. Circulation 1976:54:689-97.

Pseudoaneurysm of a Shiley composite aortic valve and graft prosthesis Barbara Kong, MD, J. David Ogilby, MD, Richard Poynton, RT, CNMT. Philadelphia,

Pa.

The spectrum of manifestations of Marfan’s syndrome makesassessment of diseasestatus a clinical and diagnostic challenge.The defective collagensynthesisresults in a high prevalence of abnormalities in the aortic root, and many of patients with Marfan’s syndrome require aortic aneurysmrepair. The predilection for recurrent aneurysm formation and dissection mandates close postoperative follow-up for detection of these life-threatening disorders. We describethe useof echocardiographyand magnetic resonanceimaging (MRI) in the characterization of a presudoaneurysmof a composite ascendingaortic graft in a young man with Marfan’s syndrome. The patient wasfirst seenin 1974at the ageof 20 with annuloaortic ectasiathat From the Philadelphia adelphia,

Pa.

Reprint byterian

requests: Medical

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J. David Center,

Institute,

Presbyterian

Medical

Ogilby, MD, Philadelphia Heart 39th and Market St., Philadelphia,

Center,

Phil-

Institute, PresPA 19104.

October 1990 Heart Journal

required placement of a compositeaortic valve and graft. He did well until the ageof 30 when he wasadmitted with an acute type III aortic dissection.His hospital coursewas complicated by sepsisand new aortic insufficiency. After 6 weeks of antibiotic therapy, he underwent an uncomplicated resection of a 20 cm infrarenal abdominal aneurysm emergingfrom the falselumen of his aorta. At that time the infected composite graft was also replaced, and debridement of the infected aortic annulus was carried out. A descendingthoracic aortic aneurysm was left unrepaired becauseof the other extensive surgery required. After surgery, his descendingthoracic aortic aneurysmwasfollowed up by serial computed tomographic scans,which recently showed an increasein diameter to 9 cm. He was free of symptoms. Physical examination revealed a young man with Marfanoid appearance.Brachial blood pressureswere equal. Cardiac examination was consistent with a normal functioning prosthetic aortic valve. A right femoral arteriovenousfistula (from a previous catheterization site) was present, and the area of previously resectedleft radial artery aneurysm wasnoted. Chest radiographsrevealed cardiomegaly with enlargementof the descendingaorta and a paratracheal density, which appeared to compressthe lower trachea. Two-dimensional and M-mode echocardiography performed on a Hewlett-Packard phased array system (Hewlett-Packard Co., Medical Products GR, Andover, Mass.) with a 2.5 MHz transducer revealed a massively dilated descendingaorta, a prosthetic aortic valve, and an ascendingaortic graft. Around the graft wasfound a 4.3 x 4 cm encapsulated space that exhibited a lowvelocity “smoke” pattern on two-dimensionalexamination (Fig. 1). The entrance from the graft to this area could not be elucidated. Conventional pulsed-wave and color flow Doppler examination of this area was unrevealing. Evidence of aortic insufficiency wasalso noted. MRI was performed (Fig. 2) with a Picker scanner(Picker International Inc., Healthcare Products, Mayfield Village, Ohio), that operated at 0.5 T with Tl weighted imagesin the coronal, transaxial, and oblique sagittal planes with TR = R- R interval and TE = 20 msec.The ascendingaortic graft was identified as well as a massthat partially surrounded the

Fig. 1. Two-dimensional echocardiogramin the right parasternal view demonstrating “smoke” swirling within a 4.3 X 4 cm encapsulatedpseudoaneurysmfPsA) adjacent to the intraluminal graft (AoG).

Persistent left superior vena cava: unusual wave contour of left jugular vein as the presenting feature.

Volume Number 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 120 4 Brief Communications Chrousos G. Churg-Strauss syndrome: development...
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