The mechanismsunderlying such damage have not beenestablished.Concomitant factors including vitamin deficiencies, hypertension and associatedbehavior such as drug abuse and cigarette smoking may be relevant. Both alcohol and its acetaldehyde and fatty acid ethyl estermetabolitescan damagecardiac muscledirectly.9,10 Individual sensitivity to alcohol may be derived from hereditary factors and malnutrition, possibly involving established relations between alcohol, nutrition and the immune system.*1 Men appear to be more prone than women to the cardiotoxic effectsof alcohol,12an observation borne out by our own series. Until now, the evidence that heart failure due to alcohol is reversiblehas been basedon casereports2and series3,4that have often failed to establish alcohol as the sole causeof left ventricular dysfunction. Some groups4 have not usedcoronary angiography in all their patients to exclude ischemic heart disease(a serious drawback given that ischemic heart diseaseis often found in alcoholics13).In at least 1 report, patients with known coronary artery diseaseand hypertension were actually included in the alcoholic heart muscle disease group.7 Similarly, no investigators have consistently performed endomyocardial biopsy to exclude specific heart muscle disorders. The endomyocardial biopsy findings in alcoholic heart muscle diseaseare not pathognomonic, but the presenceof fatty degeneration,myocyte hypertrophy and necrosis,and interstitial fibrosis are consistentwith the diagnosis.14In this series, there was no histologic evidence of myocarditis and only nonspecific features were found. Diagnostic shortcomingsprobably explain the confusion in published reports regarding the natural history of alcoholic heart muscle diseaseand the scatteredreports of failure to improve after abstinencefrom alcoho1.3>7 We

intraoperative Balloon Valvuloplasty Stenosis in Neonates

believe that our serieshas helped to define the natural history of alcoholic heart muscle diseaseand show conclusively that it is a reversiblecondition. Patients with this disorder should therefore be encouragedand helped to abstain from alcohol. Acknowledgment: We thank Jean Cunningham for her expert secretarial help.

1. Fink R, Marjot DH, Rosalki SB. Detection of alcoholic cardiomyopathy by serum enzyme and isoenzyme determination. Ann Clin Biochem 1979;16:65-66. 2. Agatston AS, Snow ME, Samet P. Regression of severe alcoholic cardiomyopathy after abstinence of 10 weeks. Alcoholism: Clin Exp Res 1986;10:386-387. 3. Wang R, Mallon J, Alterman AI, McLellan AT. Alcohol and dilated cardiomyopathy: incidence and correlation with clinical outcome. J Subs? Abuse Treat 1987;4:209-213. .4. Francis GS, Johnson TH, Ziesche S, Berg M, Boos& P, Cohn JN. Marked spontaneous improvement in ejection fraction in patients with congestive heart failure. Am J Med 1990;89:303-306. 5. W&he WH. A Practical Treatise on the Diseases of the Heart and Great Vessels, Including the Principles of Their Physical Diagnosis. London: Smith, Elder, 1873. 6. Urbane-Marquez A, Estruch R, Navarro-Lopez F, Grau JM, Mont L, Rubin E. The effects of alcoholism on skeletal and cardiac muscle. N Engl J Med 1989;320:409-415. 7. Kinney EL, Wright RJ, Caldwell JW. Risk factors in alcoholic cardiomyopathy. Angioiogy 1989;40:270-275. 8. E&s I, Piros G, Forster T, Csanady M. Alcohol-induced congestive cardiomyopathy in adult turkeys: effects on myocardial antioxidant defence systems. Basic Res Cardiol 1987;82:551-556. 9. Pus&in S, Robin E. Adenosine diphosphate effect on contractility of human muscle actomyosin: inhibition by ethanol and acetaldehyde. Science 1975; 188:1319-1320. 10. Lange LG, Kinnunen PM. Cardiovascular effects of alcohol. Adu Alcohol Subst Abuse 1987;6:47-52. 11. Dunne FJ. Alcohol and the immune system. A causative agent in altering host defence mechanisms. Br h-fed J 1989;298:543-544. 12. Wu CF, Sudhakar M, Jaferi G, Ahmed SS, Regan TJ. Preclinical cardiomyopathy in chronic alcoholics: a sex difference. Am Heart J 1976,91:281-286. 13. Ross EM, Roberts WC. Severe atherosclerotic coronary artery disease, healed myocardial infarction and chronic congestive heart failure: analysis of 8 1 patients studied at necropsy. Am J Cardiol 1986;57:44-50. 14. Davies MJ. The cardiomyopathies: a review of terminology, pathology and pathogenesis. Histopathology 1984;8:363-393.

for Critical Aortic Valvular

Steven R. Neish, MD, Martin P. O’Laughlin, MD, Michael R. Nihill, MD, David A. Ott, MD, and Denton A. Cooley, MD ritical aortic stenosis(AS) in the neonaterepresents a particularly severe form of AS where normal compensatory mechanisms are unable to maintain homeostasis,evenat rest. Critical AS may be defined as aortic valve stenosis with congestive heart

C

From the Lillie Frank Abercrombie Section of Cardiology, Department of Pediatrics, Baylor College of Medicine, Texas Children’s Hospital, 6621 Fannin, Houston, Texas 77030, and the Department of Surgery, Texas Heart Institute, Houston, Texas. Dr. Neish’s address is: Pediatric Cardiology, Fitzsimons Army Medical Center, Aurora, Colorado 80045. Manuscript received March 5, 1991; revised manuscript received and accepted May 24,199l.

failure and signs of decreasedsystemiccardiac output. l When patients presentin infancy with aortic valve stenosis and congestiveheart failure, medical managementis frequently unsuccessful.Various surgical proceduresusing both open and closed heart techniques have been attemptedto relievecritical AS in infancy.2-7Percutaneous transluminal balloon valvuloplasty has been performed in patients with critical AS in infancy.7 This procedureis technically difficult and at times impossible. The use of balloon valvuloplasty in the operating room has beendescribedfor the treatment of AS.*sQThe techBRIEF REPORTS 807

FIGURE 1. Parasternal m-axis echocardiagram demonstrating left ventricular hypertrophy and dilation, thickened aortic valve, and poststenotic dilation of ascending aorta (AO). LA = left atrium; LV = left ventricle; RV = right ventricle.

nical aspectsof intraopcrative balloon valvuloplasty and the range of application are still being investigated. Reported complications include severeaortic regurgitation and disruption of an aortic valve cusp. This report describesthe useof intraoperative balloon valvuloplasty in 4 neonateswho presentedwith critical AS in the first week of life. Four patients presented in the first 24 hours of life with symptoms due to critical AS. The initial sign in each patient was respiratory distress. Three patients were cyanotic and a systolic ejection murmur was heard in all 4. An ejection click was noted in only 1 patient. Each patient had signs of congestive heart failure. Three patients required inotropic support with dopamine or dobutamine before valvuloplasty. The diagnosis of aortic valve stenosis was confirmed by 2-dimensional and Doppler echocardiogra-

FIGURE 2. Left ventricular an&gram demonstrating ened, doming aortic valve with small orifice. 808

thiik-

THE AMERICAN JOURNAL OF CARDIOLOGY VOLUME 68

phy. Each patient had a thickened aortic valve with abnormal valve movement and left ventricular hypertrophy (Figure I). The ascending aorta was dilated. Two patients had left ventricular dilation with decreased left ventricular shortening fraction. The diameter of the aortic anulus was measured by 2dimensional echocardiography. The peak velocity across the aortic valve was measured by Doppler echocardiography. The velocity was increased and the flow was turbulent in all patients. The peak velocity was 4.5 m/s in the first patient, 5.5 m/s in the second, 3.5 m/s in the third and 23.0 m/s in the fourth. The thirdpatient had the most depressedventricularfunction before valvuloplasty. Cardiac catheterization was performed in the jrst 2 patients. A thickened, doming, stenotic aortic valve was demonstrated during catheterization (Figure 2). There was ventricular hypertrophy and poststenotic dilation of the ascending aorta. Percutaneous balloon aortic valvuloplasty was attempted in both patients. It

FIGURE 3. Technique for transventricular balloon valvuloplasty. Left, balloon is introduced through stab wound in left ventricular apex. Right, balloon is positioned across aortic valve anukis and inflated. SEPTEMBER 15, 1991

was impossible to cross the aortic valve with a guidewire from the aorta into the left ventricle in either patient. Therefore, balloon valvuloplasty could not be performed in the usual retrograde manner. In 1 patient, a guidewire was advancedfrom thefemoral vein across the atria1 septum into the left ventricle and across the aortic valve. However, it was impossible to manipulate the valvuloplasty catheter across the aortic valve.

Each patient was taken to the operating room for valvuloplasty in the first week of life. The heart was exposed through a midline sternotomy. Neither cardiopulmonary bypass nor venous inflow occlusion was performed. A purse-string suture was placed in the anterior apical surface of the left ventricle (Figure 3). A stab wound was made in the left ventricle inside the area circumscribed by the purse-string suture. A conventional angioplasty balloon, with a diameter 1 to 2 mm larger than the aortic valve anulus as measured during the preoperative echocardiogram, was chosen for valvuloplasty. The balloon catheter was inserted into the ventricle and advanced across the aortic valve. Proper position of the balloon catheter was confirmed by the surgeon by palpation at and above the aortic valve anulus. The balloon was inflated to the maxima1 recommended pressure 2 to 4 times for 3 to 6 seconds per inflation. The angioplasty balloon was removed and the ventriculotomy was repaired. After valvuloplasty and initial stabilization, echocardiography was repeated. Table I compares the peak velocity before valvuloplasty with that after valvuloplasty. The peak velocities after valvuloplasty were between 1.5 and 2.7 m/s. In the operating room, 2 patients had ventricular fibrillation requiring electrical defibrillation. Another patient had no intraoperative arrhythmias, but had several episodesof nonsustained ventricular tachycardia postoperatively and was treated successfully with mexilitene. Each patient had mild aortic regurgitation detected by Doppler echocardiography after valvuloplasty, although only 1 patient had aortic regurgitation by auscultation during the early postoperative period. Each patient had clinical improvement after surgery with improved peripheral perfusion and decreased respiratory distress. Three patients continued to have signs and symptoms of mild congestive heart failure. One patient was discharged from the hospital receiving digoxin, and 2 were discharged receiving digoxin and diuretics for the treatment of congestive heart failure. The fourth patient was discharged receiving no medications. The patients have beenfollowedfor 2 to 18 months after valvuloplasty. In the first patient, who had ventricular tachycardia during the early postoperative

TABLE I Peak Velocity Measured by Continuous-Wave at the Aortic Valve

Doppler

Peak Velocity (m/s)

Pt. No.

1 2 3 4

Before Valvuloplasty

After Valvuloplasty

4.5 5.5 3.5 23.0

1.5 2.5 2.3 2.7

period, mexilitene was stopped after 5 months and digoxin was discontinued after 6 months. This patient is asymptomatic 18 months after valvuloplasty, has mild AS and no aortic regurgitation by physical examination, and has mild aortic regurgitation by Doppler echocardiography. The second patient had persistent signs and symptoms of congestive heart failure, and continued to require digoxin and diuretits. Thirteen months after valvuloplasty, this patient developed endocarditis and progressive aortic regurgitation, and died. Before death, the patient had mild AS by physical examination and echocardiography. The third patient is asymptomatic 12 months after valvuloplasty. This patient continues to be treated with digoxin andfurosemide, has no aortic regurgitation by physical examination, and has moderate AS and mild aortic regurgitation by Doppler echocardiography. The fourth patient is asymptomatic 2 months after valvuloplasty and is receiving no medications. This patient has no aortic regurgitation by physical examination, and has moderate AS and mild aortic regurgitation by Doppler echocardiography. None of the 4 patients has any echocardiographic evidenceof left ventricular aneurysm formation at the site of the repaired stab wound in the left ventricle. Intraoperative balloon valvuloplasty was feasibleeven when percutaneousballoon valvuloplasty was impossible. The obstruction was relieved by balloon valvuloplasty and only mild aortic regurgitation was created. This is different from the resultsreportedby Phillips et a1.9They reported 4 casesof severevalve damage and regurgitation after operative balloon dilation. They used angioplasty balloons up to 8 mm larger in diameter than the aortic valve anulus as measured during necropsy. Our technique differed in that we used angioplasty balloons that were only 1 to 2 mm larger than the estimatedvalve anulus diameter as measuredduring preoperativeechocardiography. This allowed adequate relief of stenosis with an acceptabledegree of aortic regurgitation. The forcesusedto openthe stenoticorifice are applied in a radial fashion during balloon valvuloplasty. Theoretically, this will result in lessvalve disruption than a Hegar dilator that applies force to the valve in a linear fashion. The balloon catheter can be placed precisely acrossthe BRIEF REPORTS 809

aortic valve when the chestis open,and the movementof consideredas one of the options for the initial treatment the balloon catheter during dilation in relation to the of neonateswith critical AS. aortic valve anulus can be minimized by the surgeon. 1. La&on L. Aortic stenosis: valvular, supravalvular, and fibromuscular subvalvuThis is an advantagewhen comparedwith percutaneous lar. In: Garson A, Bricker JT, McNamara DG, eds. The Science and Practice of transluminal balloon valvuloplasty where catheter move- Pediatric Cardiology. Philadelphia: Lea & Febiger, 1990:1334-l 352. ment during balloon inflation can introduce a potential 2. Keane JF, Bernhard WF, Nadas AS. Aortic stenosis surgery in infancy. Circulation 1975;52:1138-1143. source of valve damage.‘O 3. Kugler JD, Campbell E, Vargo TA, McNamara DG, Hallman GL, Co&y Another complication of percutaneousvalvuloplasty DA. Results of aortic valvotomy in infants with isolated aortic valvular stenosis. J Thorac Cardiovasc Surg 1979;78:553-558. that is avoidedwith the operative technique is damageto 4. Sade RM, Crawford FA, Hohn AR. Inflow occlusion for semilunar valve the femoral arteries during cardiac catheterization. The stenosis. Ann Thorac Surg 1982;33:570-575. 5. Duncan K, Sullivan I, Robinson P, Horvath P, de Leval M, Stark J. Transvenpotential for damage to the femoral vesselsis highest in tricular aortic valvotomy for critical aortic stenosis in infants. J Thorac Cardionewbornsin whom the vesselcaliber is particularly small. “ax Surg 1987;93:546-550. This is important becauseany treatment of severeAS in 6. Brown JW, Stevens LS, Holly S, Robison R, Rodefeld M, Grayson T, Marts B, Caldwell RA, Hurwitz RA, Girod DA, King H. Surgical spectrum of aortic the neonatecan only be viewed asa temporary palliation. stenosis in children: a thirty-year experience with 257 children. Ann Thorac Surg Femoral artery occlusion makes cardiac catheterization 1988;45:393-403. 7. Zeevi B, Keane JF, Castaneda AR, Perry SB, Lock JE. Neonatal critical valvar or percutaneousballoon valvuloplasty, or both, more dif- aortic stenosis: a comparison of surgical and balloon dilation therapy. Circulation ficult as a subsequentprocedure when further interven- 1989;80:831-839. 8. Brown JW, Robison RJ, Wailer BF. Transventricular balloon catheter aortic tion is required at a later age. valvotomy in neonates. Ann Thorac Surg 1985;39:376-378. Cardiopulmonary bypass,hypothermia and systemic 9. Phillips RR, Gerlis LM, Wilson N, Walker DR. Aortic valve damage caused venous inflow occlusion are avoided with this method. by operative balloon dilatation of critical aortic valve stenosis. Er Heart J 1987;57:168-170. Operative time is minimal, and relief of the stenosisis 10. Freedom RF. Balloon therapy of critical aortic stenosis in the neonate: the prompt. Intraoperative balloon valvuloplasty should be therapeutic conundrum resolved? Circulation 1989;80:1087~1088.

Still’s=Like Innocent Murmur Can be Produced by Increasing Aortic Velocity to a Threshold Value Scott E. Klewer, MD, Richard L. Donnerstein, MD, and Stanley J. Goldberg, MD till’s murmur is a musical or vibratory systolic ejection murmur found in many normal schoolaged children and adolescents.Its origin has been attributed to vibration of a cardiac structure during ventricular contraction, turbulent blood flow, or pressure changes across normal valves.’ We previously related Still’s murmur to a small ascendingaorta with concomitant high aortic velocity.2 Functional murmurs are accentuated in high cardiac output states such as fever, exercise and anemia. Dobutamine, a ,Bt agonist with primarily inotropic action at low doses,allows study of high cardiac output states.We investigatedwhether dobutamine infusion could produce a Still’+like murmur in subjects without murmurs at rest and evaluated those factors correlating best with murmur presence.

S

From the Steele Memorial Children’s Research Center, University Heart Center, and the Department of Pediatrics (Cardiology), University of Arizona, College of Medicine, Tucson,Arizona. This study was supportedby the Pediatric Cardiology ResearchFund and the Cancer Research Fund, University of Arizona, College of Medicine, Tucson, Arizona, and by Grant T35HL07479 from the National Heart, Lung, and Blood Institute, Bethesda,Maryland. Manuscript receivedMarch 25, 1991;revisedmanuscript receivedand acceptedMay 23, 1991.

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The study group consisted of 12 normal children and young adults (3 female, 9 male) aged 10 to 22 years (mean age f standard deviation 18 f 4) without audible murmurs at rest or evidenceof cardiac abnormalities by history or examination. Subjects gave consent according to an approved human subjects protocol. Age, height and weight were recorded for each subject and body surface area calculated. Heart rate and blood pressure were measured at baseline and at Sminute intervals throughout the study. A continuous rhythm strip was monitored. Continuous dobutamine infusions of 0.5, 2.5 and 5.0 ug/kg/min were delivered through a peripheral venous catheter. Each infusion was deliveredfor 15 minutes to attain steadystate dobutamine concentrations before echocardiographic and auscultatory examination. Two independent examiners assessedthe presence or absenceof a Still’s-like murmur by auscultation immediately before each echocardiographic examination. A Still’slike murmur was defined as a low-pitched vibratory systolic ejection murmur of grade W/VI intensity heard loudest in the midprecordium near the left ster-

SEPTEMBER 15, 1991