- Email: [email protected]
Persistent atrial standstill documented over a 22-year period
404
February 1996 American Heart Journal
Bensaid
Fig. 2. Magneticres•nanceimagingdem•nstratingmass••cated•nrightatria•wa••(•).Rightatria•mass h a s d i s a p p e a r e d 3 months after c h e m o t h e r a p y (B). RA, Right atrium; RV, right ventricle; AO, aorta; p u l m o n a r y artery; M, m a s s .
with obstruction or embolic phenomenon. I n t r a m y o c a r d i a l t u m o r s can produce a r r y t h m i a s or congestive h e a r t failure, w h e r e a s pericardia] t u m o r s can cause cardiac tamponade. Initially, our p a t i e n t ' s symptoms of facial edema, cough, a n d d y s p n e a on excertion caused us to a t t r i b u t e h e r symptoms to h e a r t failure, although results ofechocardiography a n d lung perfusion s c h i n t i g r a p h y revealed cardiac tamponade a n d a p u l m o n a r y embolism, respectively. The t r e a t m e n t of p a t i e n t s with l y m p h o m a involving the h e a r t u s u a l l y does not significantly differ from the t r e a t m e n t of p a t i e n t s with l y m p h o m a without cardiac involvement. However, d r a i n a g e of a pericardial effusion or t u m o r resection m a y be a p p r o p r i a t e u n d e r certain circumstances. Because the survival of p a t i e n t s with l y m p h o m a and cardiac lesions is poor, early diagnosis and t r e a t m e n t are required. A n d r e s s et a].3 have reported t h a t performing a t r a n s v e n o u s biopsy is useful for obtaining tissue for diagnostic purposes a n d avoids the need for surgical exploration. In our case a histologic diagnosis of the right a t r i a l t u m o r was m a d e after tissue obtained by t r a n s v e n o u s biopsy with TEE guidance was analyzed. Because both TEE a n d magnetic resonance i m a g i n g d e m o n s t r a t e d intrathoracic l y m p h adenopathy, the p a t i e n t u n d e r w e n t chemotherapy. The right a t r i a l m a s s decreased in size after six cycles of chemotherapy, and the p a t i e n t ' s symptoms disappeared. In such cases a n d in p a t i e n t s in whom an intracavitary m a s s is present, t r a n s v e n o u s biopsy m a y be the preferred m e t h o d for m a k i n g a diagnosis. I n t r a c a r d i a c biopsy u s u a l l y is performed u n d e r fluoroscopic guidance; however, this procedure limits the sites in the cardiac cavity from which samples can be obtained. Recently S a l k a et al. 4 h a v e r e p o r t e d t h a t TEE is useful in guiding the biotome during a t r a n s v e n o u s i n t r a c a r d i a c t u m o r biopsy. I n this case of a right a t r i a l mass, biopsy guided by fluoroscopy alone m a y have been hazardous, unfruitful, or both. Thus TEE was useful in m a k i n g diagnoses based on tissue analysis in p a t i e n t s with a right a t r i a l m a s s .
PA,
REFERENCES
1. Petersen CD, RobinsonWA,Kurnick JE. Involvementofthe heart and pericardium in malignant Iymphomas.Am J Med Sci 1976;272:161-5. 2. Chou ST, Arkles LB, Gill GD, Pinkus N, Parkin A, Hicks JD. Primary lymphoma of the heart: a case report. Cancer 1983;52:744-7. 3. Andress JD, Polish LB, Donald MC, Hossack KF. Transvenous biopsy diagnosis of cardiac lymphoma in an AIDS patient. AM HEARTJ 1989;118:421-3 4. Salka S, SiegelR, Sagar KB. Transvenous biopsyofintracardiac tumor under transesophageal echocardiographicguidance.AMHEARTJ 1993; 125:1782-4
Persistent atrial standstill documented over a 22-year period J u l i e n Bensaid, MD Limoges, France P e r s i s t e n t a t r i a l standstill (PAS) is a very r a r e a r r h y t h m i a with a n incidence of 1 in every 125,000 electrocardiograms. 1 Its current diagnostic criteria include m a i n l y a junctional b r a d y c a r d i a with a b s e n t P waves both on surface a n d i n t r a c a v i t a r y leads in combination with lack of response of the a t r i a to direct electrical stimulation. 2 This a r r h y t h m i a is caused by a fibro-fatty degeneration of the a t r i a l muscle and is known to occur in patients with longs t a n d i n g cardiac disease, diabetes mellitus, amyloidosis, or Ebstein's anomaly a n d in 30% of p a t i e n t s with EmeryDreifuss m u s c u l a r d y s t r o p h y (EDMD). 3 This p a r t i c u l a r m u s c u l a r disease is characterized by (1) early contractures of the elbows, Achilles tendons, and posterior cervical From the Department of Cardiology,University Hospital of Limoges. Reprint requests: Julien Bensaid, MD, Servicede Cardiologie,HSpitalDupuytren, 2, Avenue Martin Luther King, 87042 LimogesCedex, France. AMHEARTJ 1996;131:404-7. Copyrigi ~© 1996 by Mosby-YearBook, Inc. 0002-8703/96/$5.00 + 0 4/4/68060
Volume 131, Number 2 American Heart Journal
Bensaid
405
Fig. 1. A, Electrocardiogram recorded for first time in 1971 with h e a r t r a t e of 40 beats/rain. P waves were absent. B, Electrocardiogram recorded in 1982. T e m p o r a r y inhibition of p a c e m a k e r previously i m p l a n t e d caused 5-second p a u s e followed by j u n c t i o n a l r h y t h m of 40 beats/min. C, Electrocardiogram recorded in 1992. S a m e j u n c t i o n a l r h y t h m of 40 beats/rain was obtained after t e m p o r a r y a d m i n i s t r a t i o n of transcutaneous electrical currents (small spikes on left). W h e n these currents were stopped, p a c e m a k e r s t a r t e d to function a g a i n (right). muscles, (2) slowly progressive muscle w a s t i n g a n d weakhess with a h u m e r a l - p e r o n e a l distribution, and (3) a card i o m y o p a t h y with a t r i a l or a t r i o v e n t r i c u l a r conduction defects t h a t m i g h t r e s u l t in sudden d e a t h in the absence of a p a c e m a k e r implantation. 4, 5 We report the case of a 36-year-old w o m a n w i t h a n EDMD who h a d a documented PAS over a period of 22 years. The p a t i e n t was first seen at t h e age of 15 y e a r s after she h a d h a d two syacopal attacks. The surface leads displayed a junctional b r a d y c a r d i a at a r a t e of 40 beats/min a n d abs e n t P waves (Fig 1, A). An His-bundle electrogram confirmed the junctional r h y t h m (Fig 2, A). A n intracardiac electrode showed no spontaneous right a t r i a l activity and a lack of response of the a t r i u m to pacing (Fig 2, B). The lack of a t r i a l systolic contraction was confirmed by the absence of "a" waves on the j u g u l a r venous pulse, on the right a t r i a l p r e s s u r e pulse, and on the M-mode anterior m i t r a l leaflet echogram. The p a t i e n t h a d r e m a i n e d well for 7 years w i t h oral a d m i n i s t r a t i o n of isoproterenol. Nevertheless in 1978 she h a d h a d a n embolic stroke of the right middle cerebral a r t e r y for which oral a n t i c o a g u l a n t t r e a t m e n t was prescribed. A p e r m a n e n t v e n t r i c u l a r inhibited p a c e m a k e r was i m p l a n t e d because t h e h e a r t r a t e h a d decreased to 31 beats/min. In 1981, a t t h e age of 25 years, the p a t i e n t gave b i r t h to a n o r m a l child. In 1982 she h a d a
progressive muscle weakness and a t r o p h y with a humeral-peroneal distribution suggesting the possibility of an EDMD. A t this time electrocardiography displayed, after t e m p o r a r y inhibition of the pacemaker, the same junctional r h y t h m a t a r a t e of 40 beats/min with absent P waves after a 5-second pause (Fig 1, B). The ventricular inhibited p a c e m a k e r was replaced by a new one of the same type. Ten y e a r s l a t e r the p a t i e n t h a d the same muscle deficits, and electrocardiography showed the same junctional r h y t h m after p a c e m a k e r inhibition by t r a n s c u t a n e o u s electrical currents (Fig 1, C). The patient's m o t h e r was known to have m u s c u l a r dystrophy with PAS for which a v e n t r i c u l a r inhibited pacem a k e r was inserted. A t the age of 9 y e a r s the patient's son, who h a d been n o r m a l at birth, h a d skeletal muscle weakness a n d contractures typical of EDMD. W h e n he was 13 years old a r a t e - a d a p t i v e dual-chamber p a c e m a k e r was implanted, because an electrophysiological s t u d y h a d displayed a severe sinus node dysfunction (Fig. 3). The peculiarity of this case lies in the fact that, as in the case described by Woolliscroft a n d Tuna, 6 PAS preceded the clinical onset of m u s c u l a r dystrophy. Moreover, the presence of PAS was proved by several electrocardiograms recorded r e p e a t e d l y during a 22-year course, which is the longest experience reported so far.
February.1996
406
Bensaid
American Heart Journal
Fig. 2. A, His bundle electrogram (HIS) with heart volume interval of 30 msec displaying junctional escape rhythm for 40 beats/min. No atrial electrical activity was present. B, Atrial pacing (S) with 15 mA intensity was unsuccessful.
Fig. 3. Electrophysiologic study performed on patient's son at age of 13 years showed abnormal response of sinus node to overdrive pacing. After right atrial pacing with cycle length of 600 msec (S-S) was done, sinus nodal discharge was suppressed for 4150 msec. Corrected sinus node recovery time was markedly prolonged to 2900 msec. AH interval, 90 msec; HV interval, 40 msec. V2 V5 V6, precordial leads.
Volume 131, Number 2 American Heart Journal
REFERENCES
1. Allensworth DC, Rice CI, Lowe GW. Persistent atrial standstill in a family with myocardial disease. Am J Med 1969;47:775-84. 2. Bloomfield DA, Sinclair-Smith BC. Persistent atrial standstill. Am J Med 1965;39:335-9. 3. Bensaid J, Vallat JM, Virot P. Can electrocardiogram help to differentiate Emery-Dreifuss muscular dystrophy from other muscular dystrophies? [Abstract] J Am Coll Cardiol 1995, II2A. 4. Emery AEH. Emery-Dreifuss muscular dystrophy and other related disorders. Br Med Bull 1989;45:772-87. 5. Marshall TM, Huckell VF. Atrial paralysis in a patient with EmeryDreifuss muscular dystrophy. Pace 1992;15:135-40. 6. Woolliscroi~ J, Tuna N. Pex~nanent atrial standstill: the clinical spectrum. Am J Cardiol 1982;49:2037-41.
Dilated cardiomyopathy associated with chronic consumption of phendimetrazine Carlo Rostagno, MD, PhD, Sabina Caciolli, MD, Massimo Felici, MD, Franca Gori, MD, and Gian Gastone Neri Serneri, MD Firenze, Italy The use of amphetamines and amphetamine-like drugs, like the use of other sympathomimetic drugs, has been associated with severe cardiovascular syndromes such as myocardial infarction, arrhythmias, rupture of ascending aorta, endocarditis, and dilated cardiomyopathy. 1, 2 This report presents the case of a young woman affected by dilated cardiomyopathy with severe impairment of left ventricular function apparently induced by long-term administration of an amphetamine-like drug, phendimetrazine. Discontinuation of the drug resulted in almost complete reversal of cardiac abnormalities within 7 months. A 37-year-old woman was admitted to our clinic because of increasing exertional dyspnea and peripheral edema. The patient had been well until she was 16 years old, when she had a weight loss of 15 kg resulting from a strict diet. She had been taking oral contraceptives (ciproteron and ethinylestradiol) since the age of 24 years. From the age of 30 years she had been taking an amphetamine-like drug (phendimetrazine) (105 mg twice a day) as an anorexiant. One month before hospitalization an increasing exertional dyspnea with progressive peripheral edema developed. Two weeks before admission the patient had palpitations at rest. Because of a worsening dyspnea that was present during minimal exertion and the presence of epigastric discomfort, the patient was admitted to our clinic. The patient was severely ill with tachycardia (120 beats/min), tachypnea (respirations 24/min), and cyanosis. Gallop rhythm and a high-frequency olo-systolic murmur at the
From Istituto di Clinica Medica e Cardiologia, Universit~ degli Studi di Firenze. Reprint requests: Carlo Rostagno, MD, PhD, Institute of Clinica Medica I and Cardiologia, University of Florence, V.le Morgagni 85, Florence, Italy. AM HEARTJ 1996;131:407-9. Copyright © 1996 by Mosby-Year Book, Inc. 0002-8703/96/$5.00 + 0 4]4/68065
Bensaid
407
apex were audible. Thoracic examination revealed dullness with absent tactile fremitus at both pulmonary bases. Peripheral edema was present. At hospital admittance electrocardiography showed sinus tachycardia, right axial deviation, initial signs of left atrial enlargement, and diffuse nonspecific repolarization abnormalities. Central venous pressure was 23 cm H20 with 5 cm H20 hepatogiugular reflux. An echocardiogram revealed increased right and left ventricle diameters with a diffuse impairment of left ventricular contractility (D% = 16) (Fig. 1). The Doppler examination revealed a systolic flow in the left atrium (+++) and in the right atrium (++++); the inferior vena cava was dilated (30 mm), and respiratory excursions were not appreciable. Vigorous treatment with diuretics, digoxin, and angiotensin-converting enzyme inhibitors was started with rapid symptomatic improvement and disappearance of pleural effusion and peripheral edema. Moreover, amiodarone was prescribed because of complex ventricular arrhythmias at Holter monitoring. A radionuclide angiography revealed a left ventricular dilation. End diastolic volume was 215 ml, and ejection fraction was 35%. At cardiac catheterization, performed several days after hospital admission, pulmonary wedge pressure was 12 mm Hg, pulmonary artery pressure was 21/8 mm Hg (mean 13), end diastolic left ventricular pressure was 11 mm Hg, and aortic pressure was 108/69 mm Hg (mean 84). Cardiac index was 2.7 L/m2. Total pulmonary resistances were 4.8 UW/mq, and vascular pulmonary resistances were 1.5 UW/mq. The angiographic examination revealed normal coronary arteriograms and a dilated left ventricular chamber with marked, diffuse hypokynesia (ejection fraction = 20%). A regurgitant mitral flow +++ caused by left ventricular dilation was appreciable. Endomyocardial biopsy showed a fibrous thickening of endocardium, interstitial fibrosis, and hypertrophy of myofibrils without any sign of inflammatory reaction (Fig. 2). The patient was discharged on diuretic, digoxin, and angiotensin-converting enzyme inhibitors treatment. After discharge the patient totally abstained from phendimetrazine use and remained without symptoms. Seven months later a repeated echocardiogram demonstrated a significant decrease of right and left ventricular diameters (diastolic left ventricular diameter decreased from 67 to 56 mm) and iraprovement of left ventricular contractility (D% = 32) (Fig. 1). At radionuclide angiography ejection fraction increased to 55%, and end diastolic volume decreased to 150 ml. At the present time the patient is in New York Heart Association functional class I with normal exertional capacity (Weber functional class A, anaerobic threshold 21.2 ml/kg/ rain). Amphetamines and amphetamine-like drugs such as phendimetrazine are widely used as anorexiants. These sympathomimetic drugs act by releasing noradrenaline from sympathetic nerve endings and blocking its reuptake at the presynaptic receptors, thus resulting in peripheral a- and ~-adrenergic receptors stimulation. Moreover they have a complex, powerful, stimulant action on the central nervous system. Acute cardiovascular effects of sympathomimetic drugs include increased cardiac contractility,
February 1996 American Heart Journal
Bensaid
Fig. 2. Magneticres•nanceimagingdem•nstratingmass••cated•nrightatria•wa••(•).Rightatria•mass h a s d i s a p p e a r e d 3 months after c h e m o t h e r a p y (B). RA, Right atrium; RV, right ventricle; AO, aorta; p u l m o n a r y artery; M, m a s s .
with obstruction or embolic phenomenon. I n t r a m y o c a r d i a l t u m o r s can produce a r r y t h m i a s or congestive h e a r t failure, w h e r e a s pericardia] t u m o r s can cause cardiac tamponade. Initially, our p a t i e n t ' s symptoms of facial edema, cough, a n d d y s p n e a on excertion caused us to a t t r i b u t e h e r symptoms to h e a r t failure, although results ofechocardiography a n d lung perfusion s c h i n t i g r a p h y revealed cardiac tamponade a n d a p u l m o n a r y embolism, respectively. The t r e a t m e n t of p a t i e n t s with l y m p h o m a involving the h e a r t u s u a l l y does not significantly differ from the t r e a t m e n t of p a t i e n t s with l y m p h o m a without cardiac involvement. However, d r a i n a g e of a pericardial effusion or t u m o r resection m a y be a p p r o p r i a t e u n d e r certain circumstances. Because the survival of p a t i e n t s with l y m p h o m a and cardiac lesions is poor, early diagnosis and t r e a t m e n t are required. A n d r e s s et a].3 have reported t h a t performing a t r a n s v e n o u s biopsy is useful for obtaining tissue for diagnostic purposes a n d avoids the need for surgical exploration. In our case a histologic diagnosis of the right a t r i a l t u m o r was m a d e after tissue obtained by t r a n s v e n o u s biopsy with TEE guidance was analyzed. Because both TEE a n d magnetic resonance i m a g i n g d e m o n s t r a t e d intrathoracic l y m p h adenopathy, the p a t i e n t u n d e r w e n t chemotherapy. The right a t r i a l m a s s decreased in size after six cycles of chemotherapy, and the p a t i e n t ' s symptoms disappeared. In such cases a n d in p a t i e n t s in whom an intracavitary m a s s is present, t r a n s v e n o u s biopsy m a y be the preferred m e t h o d for m a k i n g a diagnosis. I n t r a c a r d i a c biopsy u s u a l l y is performed u n d e r fluoroscopic guidance; however, this procedure limits the sites in the cardiac cavity from which samples can be obtained. Recently S a l k a et al. 4 h a v e r e p o r t e d t h a t TEE is useful in guiding the biotome during a t r a n s v e n o u s i n t r a c a r d i a c t u m o r biopsy. I n this case of a right a t r i a l mass, biopsy guided by fluoroscopy alone m a y have been hazardous, unfruitful, or both. Thus TEE was useful in m a k i n g diagnoses based on tissue analysis in p a t i e n t s with a right a t r i a l m a s s .
PA,
REFERENCES
1. Petersen CD, RobinsonWA,Kurnick JE. Involvementofthe heart and pericardium in malignant Iymphomas.Am J Med Sci 1976;272:161-5. 2. Chou ST, Arkles LB, Gill GD, Pinkus N, Parkin A, Hicks JD. Primary lymphoma of the heart: a case report. Cancer 1983;52:744-7. 3. Andress JD, Polish LB, Donald MC, Hossack KF. Transvenous biopsy diagnosis of cardiac lymphoma in an AIDS patient. AM HEARTJ 1989;118:421-3 4. Salka S, SiegelR, Sagar KB. Transvenous biopsyofintracardiac tumor under transesophageal echocardiographicguidance.AMHEARTJ 1993; 125:1782-4
Persistent atrial standstill documented over a 22-year period J u l i e n Bensaid, MD Limoges, France P e r s i s t e n t a t r i a l standstill (PAS) is a very r a r e a r r h y t h m i a with a n incidence of 1 in every 125,000 electrocardiograms. 1 Its current diagnostic criteria include m a i n l y a junctional b r a d y c a r d i a with a b s e n t P waves both on surface a n d i n t r a c a v i t a r y leads in combination with lack of response of the a t r i a to direct electrical stimulation. 2 This a r r h y t h m i a is caused by a fibro-fatty degeneration of the a t r i a l muscle and is known to occur in patients with longs t a n d i n g cardiac disease, diabetes mellitus, amyloidosis, or Ebstein's anomaly a n d in 30% of p a t i e n t s with EmeryDreifuss m u s c u l a r d y s t r o p h y (EDMD). 3 This p a r t i c u l a r m u s c u l a r disease is characterized by (1) early contractures of the elbows, Achilles tendons, and posterior cervical From the Department of Cardiology,University Hospital of Limoges. Reprint requests: Julien Bensaid, MD, Servicede Cardiologie,HSpitalDupuytren, 2, Avenue Martin Luther King, 87042 LimogesCedex, France. AMHEARTJ 1996;131:404-7. Copyrigi ~© 1996 by Mosby-YearBook, Inc. 0002-8703/96/$5.00 + 0 4/4/68060
Volume 131, Number 2 American Heart Journal
Bensaid
405
Fig. 1. A, Electrocardiogram recorded for first time in 1971 with h e a r t r a t e of 40 beats/rain. P waves were absent. B, Electrocardiogram recorded in 1982. T e m p o r a r y inhibition of p a c e m a k e r previously i m p l a n t e d caused 5-second p a u s e followed by j u n c t i o n a l r h y t h m of 40 beats/min. C, Electrocardiogram recorded in 1992. S a m e j u n c t i o n a l r h y t h m of 40 beats/rain was obtained after t e m p o r a r y a d m i n i s t r a t i o n of transcutaneous electrical currents (small spikes on left). W h e n these currents were stopped, p a c e m a k e r s t a r t e d to function a g a i n (right). muscles, (2) slowly progressive muscle w a s t i n g a n d weakhess with a h u m e r a l - p e r o n e a l distribution, and (3) a card i o m y o p a t h y with a t r i a l or a t r i o v e n t r i c u l a r conduction defects t h a t m i g h t r e s u l t in sudden d e a t h in the absence of a p a c e m a k e r implantation. 4, 5 We report the case of a 36-year-old w o m a n w i t h a n EDMD who h a d a documented PAS over a period of 22 years. The p a t i e n t was first seen at t h e age of 15 y e a r s after she h a d h a d two syacopal attacks. The surface leads displayed a junctional b r a d y c a r d i a at a r a t e of 40 beats/min a n d abs e n t P waves (Fig 1, A). An His-bundle electrogram confirmed the junctional r h y t h m (Fig 2, A). A n intracardiac electrode showed no spontaneous right a t r i a l activity and a lack of response of the a t r i u m to pacing (Fig 2, B). The lack of a t r i a l systolic contraction was confirmed by the absence of "a" waves on the j u g u l a r venous pulse, on the right a t r i a l p r e s s u r e pulse, and on the M-mode anterior m i t r a l leaflet echogram. The p a t i e n t h a d r e m a i n e d well for 7 years w i t h oral a d m i n i s t r a t i o n of isoproterenol. Nevertheless in 1978 she h a d h a d a n embolic stroke of the right middle cerebral a r t e r y for which oral a n t i c o a g u l a n t t r e a t m e n t was prescribed. A p e r m a n e n t v e n t r i c u l a r inhibited p a c e m a k e r was i m p l a n t e d because t h e h e a r t r a t e h a d decreased to 31 beats/min. In 1981, a t t h e age of 25 years, the p a t i e n t gave b i r t h to a n o r m a l child. In 1982 she h a d a
progressive muscle weakness and a t r o p h y with a humeral-peroneal distribution suggesting the possibility of an EDMD. A t this time electrocardiography displayed, after t e m p o r a r y inhibition of the pacemaker, the same junctional r h y t h m a t a r a t e of 40 beats/min with absent P waves after a 5-second pause (Fig 1, B). The ventricular inhibited p a c e m a k e r was replaced by a new one of the same type. Ten y e a r s l a t e r the p a t i e n t h a d the same muscle deficits, and electrocardiography showed the same junctional r h y t h m after p a c e m a k e r inhibition by t r a n s c u t a n e o u s electrical currents (Fig 1, C). The patient's m o t h e r was known to have m u s c u l a r dystrophy with PAS for which a v e n t r i c u l a r inhibited pacem a k e r was inserted. A t the age of 9 y e a r s the patient's son, who h a d been n o r m a l at birth, h a d skeletal muscle weakness a n d contractures typical of EDMD. W h e n he was 13 years old a r a t e - a d a p t i v e dual-chamber p a c e m a k e r was implanted, because an electrophysiological s t u d y h a d displayed a severe sinus node dysfunction (Fig. 3). The peculiarity of this case lies in the fact that, as in the case described by Woolliscroft a n d Tuna, 6 PAS preceded the clinical onset of m u s c u l a r dystrophy. Moreover, the presence of PAS was proved by several electrocardiograms recorded r e p e a t e d l y during a 22-year course, which is the longest experience reported so far.
February.1996
406
Bensaid
American Heart Journal
Fig. 2. A, His bundle electrogram (HIS) with heart volume interval of 30 msec displaying junctional escape rhythm for 40 beats/min. No atrial electrical activity was present. B, Atrial pacing (S) with 15 mA intensity was unsuccessful.
Fig. 3. Electrophysiologic study performed on patient's son at age of 13 years showed abnormal response of sinus node to overdrive pacing. After right atrial pacing with cycle length of 600 msec (S-S) was done, sinus nodal discharge was suppressed for 4150 msec. Corrected sinus node recovery time was markedly prolonged to 2900 msec. AH interval, 90 msec; HV interval, 40 msec. V2 V5 V6, precordial leads.
Volume 131, Number 2 American Heart Journal
REFERENCES
1. Allensworth DC, Rice CI, Lowe GW. Persistent atrial standstill in a family with myocardial disease. Am J Med 1969;47:775-84. 2. Bloomfield DA, Sinclair-Smith BC. Persistent atrial standstill. Am J Med 1965;39:335-9. 3. Bensaid J, Vallat JM, Virot P. Can electrocardiogram help to differentiate Emery-Dreifuss muscular dystrophy from other muscular dystrophies? [Abstract] J Am Coll Cardiol 1995, II2A. 4. Emery AEH. Emery-Dreifuss muscular dystrophy and other related disorders. Br Med Bull 1989;45:772-87. 5. Marshall TM, Huckell VF. Atrial paralysis in a patient with EmeryDreifuss muscular dystrophy. Pace 1992;15:135-40. 6. Woolliscroi~ J, Tuna N. Pex~nanent atrial standstill: the clinical spectrum. Am J Cardiol 1982;49:2037-41.
Dilated cardiomyopathy associated with chronic consumption of phendimetrazine Carlo Rostagno, MD, PhD, Sabina Caciolli, MD, Massimo Felici, MD, Franca Gori, MD, and Gian Gastone Neri Serneri, MD Firenze, Italy The use of amphetamines and amphetamine-like drugs, like the use of other sympathomimetic drugs, has been associated with severe cardiovascular syndromes such as myocardial infarction, arrhythmias, rupture of ascending aorta, endocarditis, and dilated cardiomyopathy. 1, 2 This report presents the case of a young woman affected by dilated cardiomyopathy with severe impairment of left ventricular function apparently induced by long-term administration of an amphetamine-like drug, phendimetrazine. Discontinuation of the drug resulted in almost complete reversal of cardiac abnormalities within 7 months. A 37-year-old woman was admitted to our clinic because of increasing exertional dyspnea and peripheral edema. The patient had been well until she was 16 years old, when she had a weight loss of 15 kg resulting from a strict diet. She had been taking oral contraceptives (ciproteron and ethinylestradiol) since the age of 24 years. From the age of 30 years she had been taking an amphetamine-like drug (phendimetrazine) (105 mg twice a day) as an anorexiant. One month before hospitalization an increasing exertional dyspnea with progressive peripheral edema developed. Two weeks before admission the patient had palpitations at rest. Because of a worsening dyspnea that was present during minimal exertion and the presence of epigastric discomfort, the patient was admitted to our clinic. The patient was severely ill with tachycardia (120 beats/min), tachypnea (respirations 24/min), and cyanosis. Gallop rhythm and a high-frequency olo-systolic murmur at the
From Istituto di Clinica Medica e Cardiologia, Universit~ degli Studi di Firenze. Reprint requests: Carlo Rostagno, MD, PhD, Institute of Clinica Medica I and Cardiologia, University of Florence, V.le Morgagni 85, Florence, Italy. AM HEARTJ 1996;131:407-9. Copyright © 1996 by Mosby-Year Book, Inc. 0002-8703/96/$5.00 + 0 4]4/68065
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apex were audible. Thoracic examination revealed dullness with absent tactile fremitus at both pulmonary bases. Peripheral edema was present. At hospital admittance electrocardiography showed sinus tachycardia, right axial deviation, initial signs of left atrial enlargement, and diffuse nonspecific repolarization abnormalities. Central venous pressure was 23 cm H20 with 5 cm H20 hepatogiugular reflux. An echocardiogram revealed increased right and left ventricle diameters with a diffuse impairment of left ventricular contractility (D% = 16) (Fig. 1). The Doppler examination revealed a systolic flow in the left atrium (+++) and in the right atrium (++++); the inferior vena cava was dilated (30 mm), and respiratory excursions were not appreciable. Vigorous treatment with diuretics, digoxin, and angiotensin-converting enzyme inhibitors was started with rapid symptomatic improvement and disappearance of pleural effusion and peripheral edema. Moreover, amiodarone was prescribed because of complex ventricular arrhythmias at Holter monitoring. A radionuclide angiography revealed a left ventricular dilation. End diastolic volume was 215 ml, and ejection fraction was 35%. At cardiac catheterization, performed several days after hospital admission, pulmonary wedge pressure was 12 mm Hg, pulmonary artery pressure was 21/8 mm Hg (mean 13), end diastolic left ventricular pressure was 11 mm Hg, and aortic pressure was 108/69 mm Hg (mean 84). Cardiac index was 2.7 L/m2. Total pulmonary resistances were 4.8 UW/mq, and vascular pulmonary resistances were 1.5 UW/mq. The angiographic examination revealed normal coronary arteriograms and a dilated left ventricular chamber with marked, diffuse hypokynesia (ejection fraction = 20%). A regurgitant mitral flow +++ caused by left ventricular dilation was appreciable. Endomyocardial biopsy showed a fibrous thickening of endocardium, interstitial fibrosis, and hypertrophy of myofibrils without any sign of inflammatory reaction (Fig. 2). The patient was discharged on diuretic, digoxin, and angiotensin-converting enzyme inhibitors treatment. After discharge the patient totally abstained from phendimetrazine use and remained without symptoms. Seven months later a repeated echocardiogram demonstrated a significant decrease of right and left ventricular diameters (diastolic left ventricular diameter decreased from 67 to 56 mm) and iraprovement of left ventricular contractility (D% = 32) (Fig. 1). At radionuclide angiography ejection fraction increased to 55%, and end diastolic volume decreased to 150 ml. At the present time the patient is in New York Heart Association functional class I with normal exertional capacity (Weber functional class A, anaerobic threshold 21.2 ml/kg/ rain). Amphetamines and amphetamine-like drugs such as phendimetrazine are widely used as anorexiants. These sympathomimetic drugs act by releasing noradrenaline from sympathetic nerve endings and blocking its reuptake at the presynaptic receptors, thus resulting in peripheral a- and ~-adrenergic receptors stimulation. Moreover they have a complex, powerful, stimulant action on the central nervous system. Acute cardiovascular effects of sympathomimetic drugs include increased cardiac contractility,