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4. 5. 6.
7.
American
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
4/4/22904
Heart
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).
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1003
Fig. 3. Line drawing showing preoperative pathology of
pseudoaneurysm,composite graft, and descendingaortic dissection.
Fig. 2. Top, Diagram: Pseudoaneurysm,PsA; intralumi-
nal graft, AoG; internal mammary artery, ZMA; descending aorta, DAo; right pulmonary artery, RPA. Middle, MRI imagewith Tl weighting. The massivelyenlargeddescending aorta (DAo) with low-velocity flow, intraluminal graft (AoG) with a signal void indicative of high-velocity flow and a surrounding mass(pseudoaneurysm).Bottom, Transaxial MRI imagein which the FLOWCDIF gradient refocused echo pulse sequenceis used to examine flow areas with moving blood (e.g., descendingaorta, pulmonary artery, mammary arteries are shown asregionsof high signal intensity signifying flow in the pseudoaneurysm).
graft, Useof the FLOWCDIF gradient refocusedechopulse sequencedemonstrated flow in this region. This combination of findings, which confirmed the suspicionof pseudoaneurysm, had not previously been described with MRI techniques. A descendingaortic aneurysm that measured 9.4 x 10 cm in width was also described. A preoperative thoracic aortogram showed minor widening of the right coronary insertion site and an aneurysmat the origin of the left main coronary artery. The diagnosis of pseudoaneurysmwasconfirmed by demonstration of contrast flow
past the left coronary ostealaneurysminto the periascending aortic graft space.Dissection of the descendingaorta wasalso noted. Fig. 3 diagrams the pathology of pseudoaneurysm and the dissection of descendingaortic aneurysm. The patient was taken to the operating room for repair of the descendingaortic aneurysm but unfortunately died in the early postoperative period. No postmortem examination was granted. The incidence of pseudoaneurysmformation after composite graft placement has not been systematically investigated; however,a study by Nath et a1.lon 15patients with compositegrafts reported that 4 of 12subjectswho underwent postoperative angiogramsdeveloped late pseudoaneurysm. Three pseudoaneurysmswere at the site of coronary ostia, and the remaining one was at the distal graft anastomosis.None of the patients carried the diagnosisof Marfan’s syndrome. In another study by Marvasti et a1.,2 15 patients with compositegrafts underwent angiography at a meanpostoperative time of 54 months. Two members of this group were found to have a pseudoaneurysmat the right coronary anastomosis.Although the natural history of aortic pseudoaneurysmsis not known, certainly death can be a result.3 Surgical therapy is often performed. In general, this complication is diagnosed by angiographic demonstration of contrast opacification in the periaortic space.Contrast opacification is often small and is limited to systole,thus cineangiographicdisplay is preferred to cut film. Computedtomography hasalsodemonstratedpseudoaneurysms.4MRI hasparticular strength in the evaluation of the aorta and has been found to be a useful tool in follow-up of patients after aortic repair. Two-dimensional echocardiography can also be useful.
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The opening of a pseudoaneurysm into the ascending aorta has been detected by two-dimensional echocardiographic examination in one case reporL5 Flow from an aortic pseudoaneurysm into the right atrium was documented with color Doppler by Hoadley.6 To our knowledge this is the first time that flow within a pseudoaneurysm was seen by “smoke” or swirling clouds of densities on echocardiogram that have been correlated with stagnant, very lowvelocity blood. Given this finding it is not surprising that pulsed-wave and color Doppler examinations, which are better suited for higher flow velocities, could not demonstrate flow in the pseudoaneurysm. Pseudoaneurysm formation is not an uncommon complication of composite aortic graft placement. This case demonstrates the usefulness of Doppler echocardiography and MRI in the diagnosis of pseudoaneurysm in a composite aortic graft. REFERENCES
1. Nath PH, Zollikofer C, Castaneda-Zuniga WR, et al. Radiological evaluation of composite aortic grafts. Radiology 1979: 131:43-51. 2. Marvasti MA, Parker FB, Randall PA, Witwer GA. Composite graft replacement of the ascending aorta and aortic valve. J Thorac Cardiovasc Surg 1988;95:924-8. 3. Crawford, ES. Marfan’s syndrome. Broad spectral surgical treatment cardiovascular manifestations. Ann Surg 1983;198: 487-505. 4. Probst P, Baur HR, Leupi F, et al. Postoperative evaluation of composite aortic grafts: comparison of angiography and CT. Br J Radio1 1983;56:797-804. 5. Wendel CH, Cornman CR, Dianzumba SB. Diagnosis of pseudoaneurysm of the ascending aorta by pulsed Doppler cross sectional echocardiography. Br Heart J 1985;53:567-70. 6. Hoadley SD. Aortic pseudoaneurysm with right atria1 communication shown by Doppler color flow imaging. AM HEARI J 1987:114:1535-36.
Echocardiographic diagnosis of rheumatic cardiopathy affecting all four cardiac valves Manuel Angel Bandin, MD, Jesus Vargas-Barron, MD, Candace Keirns, MD, Angel Romero-Cardenas, MD, Manuel Villegas, MD, and Alfonso Buendia, MD. Mexico City, Mexico
Damage to the pulmonic valve by rheumatic fever is extremely rare. When it occurs, as a rule it coexists with alterations of the other valves. The few reports in the literature of patients with rheumatic lesions of all four heart valves correspond to cases diagnosed at autopsy’, 2 or by cardiac catheterization.3,4 The purpose of this communiFrom ologia
the Department Ignacio Chavez.
of Echocardiography,
Instituto
National
Reprint requests: Jesus Vargas-Barron, MD, Department ography, Instituto National de Cardiolagia Ignacio Chavez. No. 1, Tlalpan 14080, Mexico, D.F.. Mexico.
414/22683
de Cardiof Echocardi.Juan Badiano
Table
I. Catheterization
S’>,stolic/ Diastolic (mm Hgi
RA RV MPA PWP LV Ao RA, Right pulmonary
October 1990 Heart Journal
data
Mean
Systolic/ Diastolic (mm Hg)
Mean
14 :36/l 21/18 I x/4 70152 atrium; RV. right ventricle; capillary wedge pressure;
20 19 49 60
14 90/6 1718
11
17017 82/48
62
MPA, main pulmonary artery; LV, left ventricle; Ao, aorta.
PWP.
cation is to describe the findings in a patient with rheumatic cardiopathy affecting the four valves diagnosed by echocardiography with Doppler and corroborated in a hemodynamic study and by surgery. A 19-year-old woman had suffered rheumatic fever at age 7. Two years later, cardiac catheterization was performed in the Instituto National de Cardiologia Ignacio Chavez. An aortic transvalvular pressure gradient of 103 mm Hg was found; resting pulmonary capillary pressure was 19 mm Hg and increased to 30 mm Hg during an episode of atria1 flutter (Table I). Mitral stenosis, aortic stenosis, minimal aortic regurgitation, and moderate tricuspid regurgitation were diagnosed. At that time a transpulmonary pressure gradient of 13 mm Hg was present. When the patient was 10 years old (1980), a surgical correction of the valve lesions was performed involving aortic commissurotomy (valvular area 0.4 cm2 with fusion of the three commissures), mitral commissurotomy (valvular area 0.8 cm2 with fibrotic leaflets and subvalvular thickening), and tricuspid valve reconstruction. No postoperative complications occurred, although a chest x-ray film taken several months later demonstrated second degree cardiomegaly. Nine years later, despite the patient’s remaining in New York Heart Association (NYHA) functional class I, radiologic studies showed an increase to third degree cardiomegaly. In April 1989, a second cardiac catheterization was performed (Table I), which demonstrated stenosis of all four heart valves. The patient refused another surgical intervention. Six months later (October 1989), an echocardiographic study (Sonoline C.F.. Siemens Medical Systems Inc., Iselin, N.J.) with continuous wave and color-coded Doppler was performed because of exertional dyspnea and orthopnea, and it showed signs of inactive rheumatic cardiopathy. Calcification of the mitral valve leaflets and thickening of the subvalvular apparatus were evident in two-dimensional images. With Doppler, diastolic valve area calculated by pressure half time (PHT) was 1.46 cm2, and mild mitral regurgitation was detected (Fig. 1). The aortic valve lesion was predominantly and severely stenotic. An instantahous peak gradient of 156 mm Hg was calculated from the b oppler registry using the simplified Bernoulli equation. A mild to moderate aortic regurgitation coexisted (Fig. 2). The tricuspid lesion was predominantly stenotic with a