CASE REPORTS

Supravalvular Aortic Stenosis After Replacement of the Ascending Aorta lsidre Vilacosta, MD, Asunci6n Camino, MD, Jose Albert0 San Romdn, MD, Maria JesClsRolUn, MD, Ramiro de la Llana, MD, Manuel Gil, MD, and Luis Sfinchez Harguindey, MD he compositegraft technique for T replacement of the ascending aorta in patients with aortic dissec tion or ascending aortic aneurysm has becomewidely acceptecL1Pseudoaneurysm formation secondaryto dehiscenceof the suture line at the coronary anastomosisand proximal or distal graft anastomosiscan occur. This potentially lethal complication has been reported to occur in 7 to 25%of patients with compositegrafts and requires early detection and management.2We describea patient in whom a huge anfractuous and partially thrombosed pseudoaneurysm around an ascendingaortic Dacron@ graft resulted in external compression and severe obstruction of the graft with congestive heart failure. Successful surgical correction was performed. Different imaging techniques for the diagnosisof pseudoaneurysm were used. The few reported casesare revised. A 56-year-old man was referred to our hospital for evaluation of a systolic precordial murmur and congestive heart failure. Approximately 3 months before referral, the patient experienced the onset of dyspnea on exertion and mild peripheral edema. During this period, he developed substernal chest pain, orthopnea, palpitations and paroxysmal nocturnal dyspnea. Two years before admission, he underwent surgery for an acute type A aortic dissection, and a 30 mm Dacron graft was placed in the ascending aorta. The aneurysmal ascending aorta was wrapped around the graft and closed. The patient’s postoperative course was complicated by hypotension, respiratory failure and anoxic ischemic encephalopathy. He had no history of hypertension.

On admission to our hospital, findings on physical examination revealed an alert man in mild distress. Blood pressure was 110165 mm Hg in the right arm and 80160 mm Hg, in the left arm. Pulse rate was 96 beatslmin with a regular rhythm; respiratory rate was 30 beatslmin. Jugular venous pressure was increased and bibasilar rales were detected. Coarse systolic vibrations werefelt in the carotid arterial pulse. On palpation, the cardiac apical impulse was sustained and a presystolic distention was both visible and palpable. On auscultation, there was a grade 316 midsystolic murmur at the base of the heart that was exceptionally well transmitted to the jugular notch and along the carotid vessels, and a 216 holosystolic murmur over the apex. A prominent S4 was noted. The abdomen was normal and mild ankle edema (1 +) was present bilaterally. An electrocardiogram showed sinus rhythm, left atria1 enlargement and left ventricular hypertrophy. A chest roentgenogram disclosed a prominent left atrium, mild dilata-

tion of the ascending aorta, small bilateral pleural effusions and prominent pulmonary vasculature. Transthoracic 2-dimensional echocardiography demonstrated a hypertrophied and mildly dilated left ventricle, mild reduction in systolic function with an ejection fraction of 40% and generalized hypokinesia. Mild aortic and moderate mitral regurgitation were noted. The ascending aorta was dilated and a small echo-free space between the aortic graft and the wall of the aorta was seen; transthoracic color flow examination could not clearly identify jlow in the echo-free space. A systolic jet through the ascending aorta with a maximal velocity of 4.9 m/s was recorded by continuous-wave Doppler. A transesophageal echocardiographic study allowed the diagnosis of aortic pseudoaneurysm, demonstrating flow into the echofree space around the aortic graft and also a slit-like narrowing of the aortic Dacron tube (Figure 1). Aortography and left ventricular a@ography were performed with the following results: large and anfractuous pseudoaneurysm of the ascending aorta (Figure 2, a and b), proximal graft dehiscence, mild ventricular dilatation, and reduced left ventricular systolic function. A pullback pressure tracing from the left ventricle to the proximal ascending aorta demonstrated absence of a

Departments of Cardiology and Cardiac Surgery, Hospital Universitario San Carlos, Madrid, Spain. Dr. Viimsta’s current addressis: Alchtara 57, 6Y, 28006 Madrid, Spain. Manuscript received April 6, 1992; revised manuscript rekved June 19, 1992, and accepted June 22. CASE REPORTS 1505

gradient. However, betweenthe aortic pseudoaneurysm and the distal ascending aorta, proximal to the takeoff of the innominate artery, a 95 mm Hg gradient was recorded. The dissectionflap wasvisualized in the descending aorta with persistence of flow in both lumen. Computed tomography of the chest indicated the presenceof a supravalvular aortic pseudoaneurysm which was partially thrombosed, resulting in external compressionof the aortic graft (Figure 3, a and b). Thepatient was taken to the operating room and placed on cardiopulmonary bypass. The aorta was cross-clamped and opened. There was a thrombus between the prosthetic graft and the aorta that had beensutured outside the graft resulting in compressionof the graft with severestenosis.Apartial dehiscenceof the proximal graft anastomosiswas detected.The graft wasremovedand a newDacron graft was placed. The ascending aorta was wrapped around the graft and closed. The patient’s postoperative course was uncomplicated and 2 years after his secondsurgical procedure remains asymptomatic. This report illustrates a very unusual and life-threatening complication following the repair of aortic dissection: suture line dehiscence, pseudoaneurysmformation, pseudo aneurysm thrombosis, and external compression and severe stenosis of the ascending aortic graft. Three casesof supravalvular aortic stenosis following replacementof the ascending aorta have been reported: 2 patients with Marfan’s syndrome and ascendingaortic aneurysm and 1 after repair of aortic dksection.3-5Two of these patients had acute obstruction of the ascendingaorta after its repair. Acute supravalvular aortic stenosisoccurred asa result of bleeding into the spacebetweenthe tube and the ascendingaorta usedto wrap the tube. In 1case,needledrainageof this spacecombined with autotransfusion of the aspiratedblood allowed time for reoperation.3Crosby et al4 reported a patient who developedsupravalvular aortic stenosisboth during and 15 months after surgery. A hematoma was found between the prosthetic tube and the aorta sutured

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THE AMERICAN JOURNAL OF CARDIOLOGY VOLUME 70

DECEMBER 1. 1992

outside the tube. When the association of hypotension with elevatedleft atrial pressuresoccursin the immediate postoperative period of patients after replacement of the ascending aorta, consideration should be given to this complication. Our patient and all patients reported with this complication had this hemodynamic state after surgery. The patient in this re port had a physical examination similar to patients with congenital supravalvular aortic stenosis.The disparity in pulsesmay be related to the tendency of a jet stream to adhere to a vesselwall (Coanda effect) and preferential blood flow into the innominate artery. The diagnosis of this complication is important for early surgical intervention. Various imaging methods for a correct diagnosiswere used in this patient including transthorao ic echocardiography,transesophage al echocardiography, aortography and computed tomography. Each technique provided useful information for an accurate preoperative di-

agnosis. Echocardiographic techniques can diagnosethe presenceof pseudoaneurysmcomplicating aortic grafts, and complementaortography and computedtomography in the assessmentof patients before surgical intervention.2v6Special efforts should be made to prevent pseudoaneurysm in patients with aortic grafts and to minim& formation of thrombus between the prosthetic tube and the aortic wrap. Kouchoukoset al1 studied 127 patients with compositeaortic grafts - 103 with the inclusion technique (performing the distal anastomosisinside the intact aorta and wrapping the aortic wall snugly around the aortic graft), and 24 without the inclusion technique (preclotting the prosthesiswith whole blood or albumin plus autcclaving) - and found 9 patients with pseudoaneurysms secondaryto dehiscenceat the coronary ostia or aortic suture lines; reoperation was required in 8. The incision technique was used in all 9 patients.’ These investigators* did not find significant compressionof

Profound “Pacemaker Syndrome” Hypertrophic Cardiomyopathy

in

Jay N. Gross, MD, Theodore N. Keltz, MD, Jerome A. Cooper, MD, Sheldon Breitbart, MD, and Seymour Furman, MD ypertrophic cardiomyopathy (HC) results in depressedcardiH ac output and pulmonary congestion from decreasedleft ventricular diastolic compliance,and in a large subset of patients is further complicated by dynamic left ventricular outflow tract obstruction. Completeatrioventricular (AV) block can induce severe hemodynamic compromiseand is particularly dangerousin patients with structural heart disease.The incidence of spontaneousAV block in patients with HC is unknown but is the subject of numerous clinical and electrophysiologic reports.‘-” The From the Departments of Medicine and Cardiothoracic Surgery, Montefiore Medical Center. 111 East 210th Street. Bronx. New York iO467. Manuscript received A&l 9, 1992; revised manuscript received June 16, 1992, and accepted June 17.

use of /3 blockers and calcium channel antagonists in patients with HC may independently induce rhythms that compromise normal AV synchrony. “Pacemaker syndrome” refers to the occurrenceof symptomsin patients managed with singlerate ventricular pacing who develop the adverse electrophysiologic and hemodynamic consequencesof the loss of AV synchrony.12Rarely has serious hemodynamic compromisebeen noted. This syndromeis similar to the hemodynamic events described in 3 patients with HC and profound he modynamic impairment due to the loss of AV synchrony. PATIENT I: An 80-year-old woman with a history of a precordial murmur and left bundle branch block was hospitalized with dizziness,chestpain and complete heart

the graft by thrombus formation be tween the aortic wall and the graft. To reduce the possibility of pseudoaneurysmand supravalvular aortic stenosisin patients with aortic grafts, preclotting the graft, as describedby Kouchoukos et al, is recommended.

1. Kouchoukos NT, Mar&d WG Jr, WedigeStecherTA. Eleven-yearexperier~~ with composite graft replacementof the ascendingaorta and aortic valve. J Thorax CordiaMse Surg 1986;92:691-705. 2. BarhetseasJ, Crawford Es, Safi HJ, Cc&i JS, Quinones MA, Zoghbi WA Doppler echaxrdi* graphicevaluationof pseudoaneurysms complicating compositegrafts of the ascendingaorta. Circularion 1992;85:212-222. 3. KIopp E, Hauer J, zinncr M, Brawley R. Acute supravalvuIar stenosisfollowing replacementof the aortic vaIve and ascendingaorta in a patient with Marfan’s syndrome:report of a case.Surgery 1978; 84:292-294. 4. Crosby IK, Ashcraft WC, Reed WA. Surgery of proximal aorta in Marfan’s syndrome.J Thorac Curdiovarc Surg 1973;66:75-81. 5. Chiiholm RJ, Baker CB, SalernoTA. Obstruction of the ascendingaorta following repair of aortic diisection.Am Heart J 1991;122:1771-1772. 6. JacobsNM, Godwin JD, Wolfe WG, Moore AV, Breiman RS, Korobkin M. Evaluation of the grafted ascendingaorta with computed tomography. Complications caused by suture dehiicencz. Radiology 1982;145:749-753.

block. Despite temporary ventricular pacing, her clinical course deteriorated and was complicated by persistent hypotension, respiratory failure, myocardial infarction and “‘shock liver.” A grade 316 systolic ejection murmur that increased markedly with Valsalva waspresent. No significant coronary arterial narrowing wasdetectedon angiography. A 90 mm Hg left ventricular outjlow gradient during ventricular pacing was detected,which resolved during transient episodesof intact AV conduction or when temporary AV sequential pacing was instituted (Figure I). Implantation of a permanent dual-chamber (DDD) pacemaker resulted in total clinical resolution. After implantation, noninvasive hemodynamic assessmentconfirmed this patient’s persistent dependance on AV synchrony (Figure 2). PATIENT 2: A 58-year-old woman with a history ofplasma cell dyscrasia and HC treated with verapamil was hospitalized because of intermittent chest pain and dyspnea. Symptoms wereobservedto develop

CASE REPORTS 1507

Supravalvular aortic stenosis after replacement of the ascending aorta.

CASE REPORTS Supravalvular Aortic Stenosis After Replacement of the Ascending Aorta lsidre Vilacosta, MD, Asunci6n Camino, MD, Jose Albert0 San Romdn...
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