August, 1984

426 Brief Communications American Heart Journal

reported clinically. The right ventricle became acutely Guillain-Barri? syndrome (GBS) is an acquired demyelin-
ischemic while the left ventricular blood supply was ative neuropathy occurring in an acute form.’ The average
unaffected, since the circumflex artery was dominant. annual incidence is 1.7 per 100,000 population,* and
This explains the absenceof inferior wall infarction on the usually presents with a symmetrical rapidly ascending
ECG, the maintenanceof normal left ventricular function paralysis which may lead to respiratory failure and death.
at rest and during the stress of atria1 pacing, and the A wide variety of cardiovascular complications have been
absenceof a significant hemodynamic disturbance. The described in GBS. They include hypertension, hypoten-
diagnosis of RVI, and especially isolated RVI, may be sion, focal myocarditis, and various arrhythmias.Y-”How-
difficult when right heart failure is not part of the clinical ever, requirement of pacemakerhas beenrare and Asbury
picture. Moreover, the diagnosisis usually not considered et al.‘:’ were the first to use one in 1969. So far, four
in the absenceof ECG evidence of diaphragmatic infarc- successfulcasesrequiring demandtransvenouspacemaker
tion and may be missedwithout investigations such as from 5 to 18 days have been reported.6,“.y We describe a
radionuclide scintigraphy, echocardiography, cardiac case of GBS requiring permanent demand pacemaker
catheterization, and coronary angiography. RVI shouldbe implantation for prolongedand recurrent periods of spon-
considered in the differential diagnosisof patients pre- taneousasystole.To our knowledge,permanent pacemak-
senting with recurrent unexplained ventricular tachyarr- er implantation has not been reported in this condition.
hythmias. Patients may otherwise be classifiedas having A 34-year-old housewife was admitted on August 24,
idiopathic ventricular tachycardia, cardiomyopathy, or 1981, with weaknessof all extremities and inability to
right ventricular dysplasia.5,” walk. She had had full use of all limbs the night before
REFERENCES
admission.When shewoke up, there wasnumbnessin the
1. Isner JM, Roberts WC: Right ventricular infarction compli-
left finger tips and she was unable to walk properly. The
cating left ventricular infarction secondary to coronary heart weaknessand numbnessincreasedand becamequadriple-
disease. Frequency, location, associated findings and signifi- gic in less than 24 hours. On admission, she appeared
cance from analysis of 236 necropsy patients with acute or anxious but alert and fully oriented. She weighed 277
healed myocardial infarction. Am J Cardiol 42:885, 1978. pounds with a height of 64 inches. The temperature was
2. Lore11 B. Leinbach RC. Pohost GM. Gold HK. Dinsmore RE.
Hutter AM Jr, Pastor; JO, Desanctis RW: Right ventricular normal, pulse was84/min and regular, blood pressurewas
infarction. Clinical diagnosis and differentiation from cardiac 120/70mm Hg, and respirations were 16/min. Her cardiac
tamponade and pericardial constriction. Am J Cardiol and other systemic examinations were essentially unre-
43:465, 1979. markable except for marked truncal weaknesswith intact
3I. Wartman WB, Hellerstein HK: The incidence of heart dis-
ease in 2.000 consecutive autopsies. Ann Intern Med 28:41,
diaphragm, flaccid paralysis of the legs, a strength of 3/5
1948. in both biceps and flexors of the neck. Symptoms pro-
4. Lloyd EA, Gersh BJ, Kennelly BM: Hemodynamic spectrum gressedrapidly and she was intubated 17 hours after
of “dominant” right ventricular infarction in 19 patients. Am admissionbecauseof respiratory difficulties. A tracheos-
J Cardiol 48:1016, 1981.
tomy was performed for aspiration of secretions and
5. Rossi P, Massumi A, Gillette P, Hall RJ: Arrhythmogenic
right ventricular dysplasia: Clinical features, diagnostic tech- ventilatory support and continuous cardiac monitoring
niques and current management. AM HEART J 103:415. wasdone in the intensive care unit. By 3:00P.M.on August
1982. 25, she had become totally respirator-dependent and
6. Sclarovsky S, Zafrir N, Strasberg B, Kracoff 0, Lewin RF, could respond to questions only by blinking her eyes.
Arditi A, Rosen KM, Agmon J: Ventricular fibrillation com-
plicating temporary ventricular pacing in acute myocardial Admission chest x-ray examination and ECG were unre-
infarction: Significance of right ventricular infarction. Am J markable. For 72 hours after admission,her resting heart
Cardiol 48:1160, 1981. rate varied from 60 to 120 per minute with fluctuating
temperature of 38 to 39’ C. On August 29 at 4:30 A.M., an
episodeof spontaneoussinus bradycardia of 4Olmin was
observed (fifth day after onset of neurologic symptom’s)
with a period of sinus arrest and asystole for 15 seconds;
this responded quickly to intitiation of cardiac massage,
Bradycardia and asystole requiring which restored sinus rhythm without any medications.
permanent pacemaker in Guillain-Barr6 Thereafter periods of bradycardia and asystole were
syndrome observed intermittently during endotracheal suctioning.
Intravenous atropine, 1 mg, increasedheart rate, usually
to 80 to 90 per minute, and reduced the frequency and
Dev Narayan, M.D., Milch T. C. Huang, M.D., and
duration of asystolic periods. Subsequently, shewasgiven
P. K. Mathew, M.D. Rochester, N.Y.
continuous infusion of isoproterenol to maintain heart
rate at 80 to 90 per minute; at times she required an
infusion rate ashigh as 36 pg/min (Fig. 1). Still, bradycar-
From the Division of Cardiology, Department of Medicine, St. Mary’s
Hospital; and the University of Rochester School of Medicine and Den- dia and asystole were again observed during suctioning
tistry. procedures as well as spontaneously. Placement, of a
Reprint requests: P. K. Mathew, M.D., Cardiology Unit. St. Mary’s temporary demand pacemaker was initially considered,
Hospital, 89 Genesee St., Rochester, NY 14611. but becauseof the danger of infection, medical manage-
Volume 108
Number 2 Brief Communications 427

i
c :
ISOPibTEiENCiL ILV
_’ :

Fig. 1. A, Spontaneous bradycardia on the fifth day after the onset of neurologic symptoms. B,
Bradycardia and asystole during atropine administration, 1 mg intravenously, every 4 hours. C,
Bradycardia and asystole while receiving isoproterenol, 20 rglmin intravenously.

ment using atropine and isoproterenol was attempted in episodes probably represent bursts of periodic unbalanced
order to avoid transvenous intracardiac manipulations. neurohumoral activity of the autonomic nervous system.
Since there was no improvement in her neurologic status Similar observations have been made by LichtenfeldP
even after 8 weeks, with spontaneous occurrence of brady- These may account for sudden death in these patients.
cardia and asystole, a permanent intracardiac demand The condition can be managed by using a temporary
programmable pacemaker was implanted. After implanta- demand pacemaker, since such activity is usually tran-
tion, frequent pacemaker activity was observed when the sient and reversible. However, lack of improvement in
rate was programmed to the lowest level, even 6 months neurologic functions even after 8 weeks and problems of
after implantation. Only minimal improvement in neuro- associated infections, instability of temporary pacemaker
logic function has taken place during this period and the wire position, and difficulties in constantly monitoring
patient continues to be respirator-dependent. continuous intravenous medications for long periods
Autonomic dysfunction is common in GBS and mani- prompted the use of permanent pacemaker implantation
fests as either excessive or inadequate activity evidenced in our patient. Pacemaker activity has been frequently
by an inappropriate heart rate response, beat-to-beat observed ever since, when the rate was programmed to the
variation, postural hypotension, profuse perspirations, lowest setting even 6 months after implantation.
and hypertensive reaction.4, lo.l1 Incidence of cardiac
arrhythmia is common in CBS. Greenland and Griggs? REFERENCES
noted at least one arrhythmia in 13 out of 16 patients 1. Asbury AK: Diagnostic considerations in Guillain-Barre Syn-
studied, and they also found significant correlation drome. Ann Neurol 9 (Suppl):l, 1981.
2. Kennedy RH, Danielson MA, Mulder DW, et al: Guillain-
between autonomic nervous dysfunction and atrioventric-
Barre Syndrome: A 42-year epidemiologic and clinical study.
ular block rhythms. Temporary pacemaker implantation Mayo Clin Proc 53:33, 1978.
was done in two of their patients because of sinus arrest. 3. Mitchell PL, Meilman E: The mechanism of hypertension in
In our patient, apart from sinus bradycardia, short epi- Guillain-Barre Syndrome. Am J Med 42:986, 1967.
sodes of sinus tachycardia and periods of asystole, no 4. Lichtenfeld P: Autonomic dysfunction in the Guillain-Barre
Syndrome. Am J Med 50:772, 1971.
other arrhythmias or ECG abnormalities were noted 5. Haymaker W, Kernohan JW: The Landry-Guillain-Barre
during the entire course of the disease, and junctional or Syndrome. Medicine (Baltimore) 28:59, 1949.
ventricular escape mechanism was consistently absent 6. Greenland P, Griggs RC: Arrhythmic complications in the
during all episodes of sinus arrest. Bradycardia, asystole, Guillain-Barre Syndrome. Arch Intern Med 140:1053, 1980.
7. Bredin CP: Guillain-Barre Syndrome:,The unsolved cardio-
and various cardiac arrhythmias are well known during vascular problems. Ir J Med Sci 146:273, 1977.
endotracheal suctioning.14 Hypoxia, metabolic distur- a. Emmons PR, Blume WT, DuShane JW: Cardiac monitoring
bances, myocarditis, and often associated pneumonias are and demand pacemaker in Guillain-Barre Syndrome. Arch
thought to be major inducing factors. However, reflex Neurol 32:59, 1975.
9. Favre H, Foex P, Guggisberg M: Use of demand pacemaker in
bradycardia mediated by excessive parasympathetic stim- a case of Guillain-Barre Syndrome. Lancet 1:1062, 1970.
ulation is the most likely explanation in our patient. 10. Frison JC, Sanchez L, Garnacho A, et al: Heart rate varia-
Despite atropine and isoproterenol, spontaneous periods tions in the Guillain-Barre Syndrome. Br Med J 281:649,
of bradycardia and asystole were still observed. Such 1980.
 August, 1984
428 Brief Communications American Heart Journal

11. Roseman E, Aring CD: Infectious polyneuritis. Medicine (reduced nicotinamide adenine dinucleotide), we have
(Baltimore) 20:463, 1941. developed an apparatus which allowscontinuous monitor-
12. Stewart IM: Arrhythmias in the Guillain-Barre Syndrome.
Lancet 1:665, 1973. ing of the NADH to nicotinamide adenine dinucleotide
13. Asbury AK, Arnason BG, Adams RD: The inflammatory (NAD) ratio of the endomyocardium in intact animals.
lesion in idiopathic polyneuritis: Its role in pathogenesis. The principle of this device (Fig. 1) is illumination of
Medicine 48:173, 1969. cardiac tissue, in situ, through an optical fiber at a special
14. Shim C, Fine N, Fernandez R, et al: Cardiac arrhythmias wavelength (~340 nm) which inducesemissionof fluores-
resulting from tracheal suctioning. Ann Intern Med 71:1149,
1969. cent light by NADH and thus from cardiac tissue. The
emitted light is collected by the optical fiber and transmit-
ted to photoreceivers; the amount of light measuredis
directly related to the NADH to NAD ratio of the
Cardiac metabolism monitored by endomyocardium in the zone of examination, as previous-
fiberoptic laser fluorimeter ly demonstrated by Chanceet al.’ A reference light of 586
nm is back-scattered by cardiac tissues, giving a signal
Guy Renault, M.D., Elizabeth Raynal, related to tissue-bloodcontent, to compensatethe fluores-
Martine Sinet, M.D., Martine Muffat-Joly, cent signal for variations induced by blood circulating into
Jean-Marie Vallois, Jean-Pierre Berthier, Ph.D., the tissue. These signals are processed,according to an
Jean Cornillault, and Bruno Godard. experimentally determined relationship, in a Hewlett-
Paris, Villetaneuse, and Marcoussis, France Packard HP85 microcomputer.?Pulsed laser sources(ni-
trogen and dye lasers)have properties which make them
especiallysuitable for this application; the short duration
of pulses(-5 nsec)allows delivery of high peak power to
The need to appreciate alterations of cardiac metabolism the tissue while the mean power delivered is low, thus
in ischemic heart diseasehas not been fully satisfied by preventing the occurrenceof photochemical artefact. Fig.
the measurementof lactate extraction rate in the coronary 2 showsthe tracings of F (fluorescent light) and I (back-
sinus. On the basis of in vivo fluorimetry of NADH scattered light) recorded by right catheterization from an
intact dog. The tip of the fiber, introduced in a catheter
through the jugular vein, wasplaced againstthe endomyo-
From INSERM U.13. H6pital Claude Bernard; Laboratoire de Physique
cardium at the apex of the right ventricle. Anoxia (100%
des Lasers; and CILAS. N, breathing for 30 seconds)was produced when the
Reprint requests: Guy Renault, M.D., INSERM U. 13, HBpital Claude recording began; femoral artery PO, was 34 mm Hg just
Bernard, 10, Ave. de la Porte d’Aubervilliers, 75944 Paris, 19, France. before the rise of intra-tissue NADH.

NITROGEN DYE
(\
LASER
tn

PhO
805lllll
Or 586nm

Fig. 1. Principle of the apparatus. The optical fiber transmits laser energy (nitrogen 1aser:NADH
excitation; dye 1aser:referencelight) to the tissue, collects the fluorescent and back-scattered light from
the tissue, and then transmits this light to the optical system. PM = measuring photomultipliers;
PhD = control photodiodes.