Automatic implantable cardioverter defibrillator implantation for malignant ventricular arrhythmias associated with congential heart disease

tional antiarrhythmic drug therapy. This patient has re- well tolerated during short-term administration. Further mained well over a follow-up period of 18 months. Al- studies will be necessaryto determine its long-term effithough he hascontinued to show episodesof nonsustained cacy and safety in patients with sustained VT. VT on 24-hour ambulatory recordings during follow-up, theseepisodesare shorter, slower and less frequent than Colatsky TJ, Bird LB, Jurkiewicz NK, Wendt RL. Cellular electrophysiology during the baselinestate when his VT wasvirtually inces- of1. the new antiarrhythmic agent recainam (WY-42,362) in canine cardiac Porkinsant. In patients in whom VT remained inducible, re- je fibers. J Cardiovasc Pharmacol 1987;9:435-444. cainam significantly slowed the mean VT rate from 194 2. Colatsky TJ, Bird LB, Knowles JA. Cardiac electrophysiology of the antiarrhythmic agent recainam (WY-42,362) in anesthetized dogs: relation to plasma to 157 beats/min (p <0.05). However, none of these and myocardial concentrations. J Cardiouasc Pharmacol 1988:11:308-316. patients was treated for a long term with recainam be- 3. Bergey JL, Sulkowski T, Much DR, Wendt RL. Ant&rhythmic, hemodynamcause this partial responsewas deemed inadequate. A ic and cardiac electrophysiological evaluation of N-(2,6-dimethylphenyI)-N’-[3(WY-42,362). Arzneimittelforschung 1983; notable observation in our study was the absenceof sig- (1-methylethylamino)-propyllurea 33:1258-1268. nificant side effects. Recainam was well tolerated by all 4. Anastasiou-Nana MI, Anderson JL, Hampton EM, Nanas JN, Heath BM. Recainam, a potent new antiarrhythmic agent: effects on complex ventricular 10patients during short-term administration and by the 1 arrhythmias. JACC 1986:8:427-435. patient in the long-term study. In particular, no patient 5. Anderson JL, Anastasiou-Nana MI, Heath BM, Menlove RL, Nanas JN, developedsigns or symptoms of congestiveheart failure. Friedman J. Efficacy of recainam, a new antiarrhythmic drug, for control of arrhythmias. Am J Cardiol 1987;60:281-287. Theseresults are similar to those found in previous stud- ventricular 6. Donoso R, Anastasiou-Nana MI, Bartholomew MB, Anderson JL. Long-term ies of ventricular premature complex suppression.4-6 evaluation of recainam: a highly effective new ant&rhythmic with negligible side In conclusion, recainam may occasionally suppress effects. .I Electrophysiol 1988;2:104-113. F, Nelson SD, Kou WH, Pratley R, Schmaltz S, de Buitleir M, Halter the induction of sustainedVT in patients who have failed 7.JB.Morady Electrophysiologic effects of epinephrine in humans. JACC 1988;l I:1 235to respondto other antiarrhythmic drugs. It appearsto be 1244.

Automatic Malignant Disease

Implantable Ventricular

Cardioverter Arrhythmias

Defibrillator Implantation for Associated with Congential Heart

Mary A. Kral, PA-C, Henry M. Spotnitz, MD, Alan Hordof, MD, J. Thomas Bigger, Jr., MD, Jonathan S. Steinberg, MD, and Frank D. Livelli, Jr., MD he prognosis for patients with congenital cardiac T abnormalities can be improved by surgical correction of hemodynamics. With’ or without2 surgery, sudden

arrest during daily activities. Arrhythmias were not inducible in the electrophysiology laboratory to guide therapy. One of these patients (no. 4) experienced nearly death from malignant ventricular arrhythmias is an im- fatal failure of empiric management at another hospital. portant potential problem in congenital heart disease. AICD implantation was recommended as more likely to Pharmacologic arrhythmia control may be limited by provide long-term survival than empiric drug therapy or side effects,noncompliance or unreliable indexes predic- cardiac transplantation. Direct surgical ablation was not tive of drug efficacy. The automatic implantable cardio- attempted because of inability to map the site of origin of verter difibrillator (AICD) is a potential alternative. The the ventricular arrhythmias. Preoperative evaluation included catheter studies of incidence of arrhythmic death is <2% per year following AICD implant3 in adults with ventricular arrhythmias electrophysiology and hemodynamics, as well as exerrefractory to medical management. This successrate fa- cise testing to define maximal heart rate. The surgical vors AICD therapy in selected patients with congenital approach was dictated by factors including residual heart disease.We present 4 such cases (Table I) and pathologic anatomy and physiology, surgical adhesions, discusstheir management. shunts and prostheses. The need for accessory pathway The first patient (no. 1) experienced no spontaneous ablation (no. I), aortic coarctation repair (no. 2) and arrhythmia. Intervention was prompted by sudden death transvenous pacemaker insertion (no. 3) were specific in 2 siblings within 2 years. Electrophysiologic study issues in these patients. A bipolar dual chamber pacing revealed ventricular fibrillation during both atria1 and system, physical separation of the pacing electrodes ventricular stimulation. A left paraseptal accessory from AICD rate sensing electrodes and reduced pacing pathway with short anterograde refractory period was amplitude inpatient no. 3 avoided undesirablepacemakalso demonstrated. Intervention was required in the re- er-AICD interaction.4 Since defibrillation thresholds can increase postopermaining 3 patients (nos. 2, 3 and 4) because of cardiac atively, AICDfunction was retested I week after implanFrom the Departments of Surgery, Pediatrics and Medicine, Columbia tation. AICD failure during this test inpatient no. 2 was Presbyterian Medical Center, 630 West 168th Street, New York, New corrected by implantation of a transvenous spring-coil York 10032.This study was supported in part by grant HL-22894 from the US Public Health Service, Bethesda, Maryland. Manuscript re- electrode, although patch-coil systems are usually less ceivedJuly 13,1988; revisedmanuscript receivedand acceptedNovem- effective than large patch systems.j Postpericardiotomy syndrome in 2 patients (nos. 1 and 4) responded to antiber 7, 1988. 118

THE AMERICAN JOURNAL OF CARDIOLOGY VOLUME 63

TABLE I Clinical

Data: Automatic

Implantable

Cardioverter

Defibrillator

(AICD)

Implantation

in Congenital

Previous Pt

Age*, Sex

&ease

Arr

EP

Op

1 2 3 4

15, 14, 19, 25,

FHC, ACP T, DORV,Co T,VSD, PS,SS Uhl’s

0 + + +

VF 0 0 0

0 P,C P, P, C 0

M M F F

Heart

Disease

AICD Rx

Implant Date

Operation (incision)

Gen (n)

Prop Prop PA Mult

4/12/85 11/8/85 6/21/85 11/30/84

AICD. AICD. .AICD, AICD

2 2 2 3

APA (MS) Co (PL, TV) PM (PL. TV) (MS)

Rate ‘li9 197 171 160

Disch (n) 1 0

Status A A D A

>I7 37

* !Jge at time of implant. = corrective open heart surgery; Co = coarctation of the aorta; D = A = alwe: ACP = accessory ConductIon pathway; APA = accessory pathway ablation; &rr = clinical,arrest;.C de@; Disch = cumulative AICD pulse Count; DORV = double outlet right ventricle; EP = inducible ventricular arrhythmia at electrophysiology study; FHC = familial hypertrophic cardiomyopathy; Gen = generators implanted; MS = median stern&my; Mult = multiple; Op = operation; P = palliative (e.g., shunts); PA = procalnamide; PL = posterolateral thoracotomy; PM = pacemaker; Prop = propranoloi; PS = pulmon~c stenosis: Rate = AICD rate cutoff; Rx = drug therapy; SS = sick sinus syndrome; T = transposition of the great leads; Uhl’s = Uhl’s anomaly; VF = ventricular fibrillation; VSD = ventricular septal ,defect; + = present; 0 = absent. Vessels; N = b3~SV~~oUS

FIGURE 1. A, Posterior anterior chest x-ray, patient no. 4, illustrates epicardial electrodes for rate sensing and large patch electrodes for defibrillation and QRS morphology. 6, patient no. 2: lead placement initially similar to A; subsequently a transvenous spring-coil defibrillation/QRS morphology electrode was added. C, patient no. 3: similar lead configuration plus a dual chamber pacemaker. An additional epicardial pacing electrode, no longer functional, was placed during previous heart surgery.

We conclude that oatients with congenital heart diseaseand malignant ar;hythmias amenableto AICD control representa population of specialinterest in whom the probability of long-term survival may be increased as a result of the unusual efficacy of this form of antiarrhythmic therapy. Our experienceemphasizesthe desirability In our series,3 patients have experiencedAICD dis- of increased AICD battery life and reduced cost consischarges.AICD rescueduring potentially lethal ventricu- tent with anticipated patient longevity. Smaller AICD lar arrhythmias wasdocumentedin 2 patients (nos. 3 and size, as well as improved arrhythmia detection and pro4). Sinus tachycardia during exerciseis believed to have grammable rate limits to minimize inappropriate shocks, would also be desirable. triggered function in the third patient (no. 1). Inconveniencesassociatedwith the AICD have generally beenwell acceptedby our patients. Theseinclude the AICD discharge itself and restrictions on contact sports, driving an automobile and swimming without supervi- 1. Bharati S, Lev M. The myocardium, the conduction system, and general sequelae after surgery for congenital heart disease. In: Engle MA, Pcrloff JK, eds. sion. Follow-up at 1 to 2-month intervals also has been Congenital Heart Disease after Surgery: Benefits, Residua, Sequelae. New York; accepted. Yorke Medical Books, 1983:247-259. One patient did not survive the 42-month mean fol- 2. Pedersen DH, Zipes DP, Foster PR, Troup PR. Ventricular tachycardia and fibrillation in a young population. Circulation 1979;60:988-997. low-up in this series.Factors implicated in her death may ventricular 3. Reid PR, Mirowski MM, Mower MM, Platia EV, Griffith LSC, Watkins L Jr, have included residual pulmonary hypertension. More Bach SM, Imran M, Thomas A. Clinical evaluation of the internal cardioverterin survivors of sudden cardiac death. Am J Cardiol1983;51:16OXimportantly, neurologic injury and a severebipolar per- defibrillator 1613. sonality disorder developedafter her initial cardiac ar- 4. Winkle RA, Stinson EB, Echt DS, Mead RH, Schmidt P. Practical aspects of rest. Theseled to parental ambivalenceregarding intensi- automatic cardioverter/defibrillator implantation. Am HeartJ 1984:108;1335ty of treatment, demonstratedby failure to comply with 5.1346. Troop P, Chapman P, Olinger G, Kleinman L. The implanted defibrillator: recommendedreplacementof a depleted AICD genera- relation of defibrillating lead configuration and clinical variables to defibrillation tor. Finally, hospitalization was refused despitea marked threshold. JACC 1985;6:1315-1321. 6. Steinberg JS, Bigger JT, Livelli FD, Spot&z HM. Recurrence of ventricular increase in frequency of AICD discharges, followed tachyarrhythmias in patients with automatic implantable defibrillators and no shortly by the patient’s death. antiarrhythmic therapy (abstr). Circulation 1987;76(.wppl V):V-461. inflammatory agents. We usually avoid antiarrhythmic therapy following AICD implant6; however, P-adrenergic blockade was required for persistent hypertension (no. 2) and also following inappropriate shock for presumed sinus tachycardia (no. 1). Patient no. 4 required disopyramide to reduce frequency of AICD discharges.

THE AMERICAN

JOURNAL

OF CARDIOLOGY

JANUARY

1, 1989

1