Persistent atrial standstill, report of three cases

J. ELECTROCARDIOLOGY, 8 (4) 357-362

Persistent Atrial Standstill, Report of Three Cases BY AKIRA KUROKAWA, M.D., AKIRA KURITA, M.D., GENGO KASAI, M.D. AND EIICHI KIMURA, M.D.

SUMMARY Three cases, of which two are brothers, of persistent atrial standstill are reported. The diagnosis was made by the lack of P wave in routine 12 leads and right atrial cavity lead, no response of atrium to electrical stimulation and absence of "a" wave in right atrial pressure curve.

This is a report on three patients showing persistent atrial standstill (PAS).

CASE REPORTS Case 1. A 23-year-old man The patient was found to have bradycardia and cardiac enlargement at the age of six, although he did not complain of any symptom. He suffered from rheumatic fever at the age of 12 and pneumonia at 18. On September 13, 1970 (22 years of age), he suddenly lost consciousness for a few minutes. After the recovery he noticed a right-sided hemiplegia and a disturbance of speech. An ECG t a k e n at that time revealed a marked b r a d y c a r d i a at a r a t e of 29/min, r e g u l a r rhythm and absent P wave, as is seen in Fig. 1. QRS duration was 0.11 sec. On admission to our hospital on J a n u a r y 25, 1971, he still showed marked bradycardia. Blood pressure was measured to be 130/72 mmHg. A systolic ejection m u r m u r of Levine 2 ~ was audible in the apical region. Chest X-ray d e m o n s t r a t e d cardiac e n l a r g e m e n t with a cardiothoracic ratio of 59.2%. Venous pressure was 130 mmH20. There were mild

From the Department of Internal Medicine, Nippon Medical School, Tokyo, Japan. Reprint requests to: Eiichi Kimura, M.D., Department of Internal Medicine, Nippon Medical School, 1-1-5 Sendagi, Bunkyo, Tokyo, Japan, Zip Code 113. 357

disturbance of speech with dextro-position of tongue, right-sided spastic hemiparesis and increased deep tendon reflexes. Muscle atrophy was not present. Blood cell counts and urinalyses were all within normal range. The e l e c t r o l y t e s , e x p r e s s e d as m E q / L , w e r e sodium 143.0, p o t a s s i u m 4.3 and chloride 103.0. The ECG taken on admission was almost the same as that shown in Fig. 1. Exercise and intramuscular injection of 1.0 mg atropine sulfate produced an increase of heart rate to 43/min and 55/min, respectively, but the P wave did not appear. Esophageal and right atrial cavity leads revealed no identifiable atrial electrical activity; the latter is indicated in the upper part of Fig. 2. The lower part of this figure shows the lack of "a" wave in the right atrial pressure curve. No atrial and ventricular excitation were produced by stimulation of the right atrium with a pacing catheter of the intensity of 15 mA, as shown in Fig. 3. On the contrary, stimulation of right ventricle readily produced ventricular activation, but retrograde P wave did not appear. Endomyocardial biopsy was not carried out, because the patient refused further right heart catheterization. The findings described above led us to make the diagnosis of PAS. Because the familial incidence of the disease is known, members of the patient's family were examined and similar findings were found in his brother (Case 2). Case 2. A 21-year-old m a n (a b r o t h e r of Case 1) As in the Case 1, this patient was found to have bradycardia at the age of six. No subjective symptoms, however, have been complained up to the present. An examination carried out on March 28, 1971, revealed abnormal findings in the ECG, including bradycardia with a heart rate of 38/min, lack of P wave and QRS interval of 0.10 sec, as shown in Fig. 4. A systolic m u r m u r of Levine 2 ~ was p r e s e n t in the apical region. Chest X-ray d e m o n s t r a t e d cardiac e n l a r g e m e n t with a cardiothoracic ratio of 57.7%.

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The p a t i e n t most likely has p e r s i s t e n t atrial standstill, even though further examination m a y be required to make a definite diagnosis. Case 3. A 78-year-old woman The patient had no remarkable family history. She suffered from diphtheria when she was 2 years old. At the age of about 15, she became aware of bradycardia, although no subjective symptoms were present. On October 26, 1972, she suddenly lost consciousness for approximately 10 min while engaged in sedentary work. Two similar fits were experienced since then. Fig. 5 shows an ECG t a k e n on October 29, 1972. P wave was absent in all leads. M a r k e d p r o l o n g a t i o n of QT interval was observed in addition to the ventricular extrasystoles. When she was admitted to our hospital on November 1, 1972, her heart rate was 52/min, showing slight irregularity. Blood pressure was 172/90 m m H g and venous pressure 110 mmI-I20. A systolic m u r m u r of Levine 3 ~ was heard in the left 4th intercostal space parasternally. Chest X-ray revealed a very large cardiac silhouette. The electrolytes, expressed as mEq/L, were sodium 145.0, potassium 4.2 and chloride 103.0. J. E L E C T R O C A R D I O L O G Y ,

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Repeat ECG t a k e n after hospitalization still showed no P wave in any lead, but abnormal elongation of QT interval and extrasystoles were not observed. Intravenous injection of 0.5 rag. atropine sulfate resulted in nothing o t h e r t h a n an increase of heart rate to 78/min. P wave was lacking also in both esophageal and right atrial cavity leads, and r i g h t a t r i a l pressure curve showed no "a" wave. Stimulation of right a t r i u m with a pacing catheter did not cause any excitation, even when the intensity of the stimulus was increased to 20 mA, as shown in Fig. 6. As shown in Fig. 6, His bundle electrogram exhibited t h a t the V wave was preceded by His spike, indicating a r h y t h m originating from proximal His bundle. The H-V interval was prolonged to 65 to 75 msec. On the basis of these findings, the diagnosis of PAS was confirmed.

DISCUSSION Atrial standstill is classified into three types:~temporary, terminal and persistent. According to the review of Jouve et al,2 38 cases with atrial standstill were reported be-

fore 1967, including all types. P e r s i s t e n t atrial standstill is a r a t h e r rare event. In addition to 15 cases T M reviewed by Bensaid et al, 3 16 cases are f o u n d in t h e w o r l d literature, 12-19 including our three patients. Characteristic features of PAS comprise the lack of P wave and increase in heart rate without appearance of P wave after injection of a t r o p i n e sulfate or exercise. In r e c e n t years, the lack of P wave even in right atrial cavity lead, no response to the stimulation of atrium and absence o f " a " wave in right atrial pressure curve have been added to the diagnostic criteria. Such an electrical silence of atrium m a y indicate t h a t PAS is not a disorder of the conduction system but an abnormality of atrial muscle itself and t h a t it m u s t be different from sick sinus syndrome. Underlying or complicating diseases of PAS so far alluded to include facioscapulohumeral muscular dystrophy and amyloidosis. In autopsy, Chavez et al 4 found old myocardial infarction with perivascular and interstitial i n f l a m m a t i o n in a t r i u m and m a r k e d degeneration of SA node. Rosen et al ~4described the presence of atrial degeneration of unknown origin in their autopsied case. In this case transient prolongation of H-V interval J. ELECTROCARDIOLOGY, VOL. 8, NO. 4, 1975

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was observed and ascribed to t h e fibrosis surr o u n d i n g t h e b u n d l e of His. S u c h prolongation of H-V i n t e r v a l in His b u n d l e electrog r a m was found also in our Case 3. In the p r e s e n t paper, Cases 1 and 2 are brothers. Similar cases were reported by A l l e n s w o r t h et al, s N a g l e et a116 and A r i t a et al. 19 In A l l e n s w o r t h ' s cases amyloidosis was suspected as an u n d e r l y i n g disease, while in N a g l e ' s and Arita's cases no etiologic factor was d e m o n s t r a t e d . In our cases also no evid e n c e i n d i c a t i n g a n y etiologic disease was found. It m a y be n o t e w o r t h y t h a t n i n e cases h a v e b e e n r e p o r t e d f r o m J a p a n a m o n g 31 cases s t a t e d above. T h e r e were some d o c u m e n t e d cases of PAS in w h i c h s y s t e m i c embolism, A d a m s - S t o k e s s y n d r o m e or h e a r t failure developed. Cases 1 and 3 h a d episodes of A d a m s - S t o k e s - l i k e attacks, a l t h o u g h cerebral e m b o l i s m could not be excluded. T h e s e p a t i e n t s refused to h a v e a n artificial p a c e m a k e r i m p l a n t e d .

J. ELECTROCARDIOLOGY, VOL. 8, NO. 4, 1975

REFERENCES 1. BLOOMFIELD, D A AND SINCLAIR-SMITH, B C: Persistent atrial standstill. Am J Med 39:335, 1965 2. JOUVE, A, DELAAGE, M, TORRESANI, J, NICOLAI, P, HEUILLET, G E T PINAS, E: La paralysie auriculaire. Arch Mal Coeur 60:865, 1967 3. BENSAID, J, GUIZE, L, FERNANDEZ, F, SCEBAT, L ET LENEGRE, J: Block sino-auriculaire chronique et paralysie auriculaire permanente. Acta Cardiol 28:3, 1973 4. CHAVEZ, I, BRUMLIK, J v SODI-PALLARES, D: Sobre un caso extraordinario de par~lisis auricular permanente, con degeneraci6n del novo de Keith y Flack. Arch Inst Card Mexico 16:159, 1946 5. RAYNAUD, R, JOBARD, P, BROCHIER, M, FAUCHIER, J P, BENATRE, A ET RAYNAUD, P H: Paralysie auriculaire chronique et oreillette papyracee. Ann Cardiol Angeiol 17:117, 1968 6. CAPONNETTO,S, PASTORONI, C AND T1RELLI,G:

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9. 10. 11.

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Persistent atrial standstill in a patient affected with facio-scapulohumeral dystrophy. Cardiologia 53:341, 1968 RAPPELLI, A, GAGNA, G E FABRIS, F: Standstill atriale permanente in un paziente affeto da distrofia muscolare progressiva e da diabete mellito. Min Cardioang 17:63, 1969 JOUVE, A, AMICHOT, J L, PRUDHOMME, F ET PICARD, J P: Paralysie auriculaire (a propos d'un nouveau cas avec electrocardiogramme endocavitaire). Marseille Medical 106:431, 1967 ALLENSWORTH,D C, RICE, C J AND LOWE, G W: Persistent atrial standstill in a family with myocardial disease. Am J Med 47:775, 1969 PATTON, R, DAMATO, A N, BERKOWITZ, W D, LAV, S H AND STEIN, E: The electrically silent atrium. J Electrocardiol 3:239, 1970 DERRIDA, J, FORMAN, J, FOUCHARD, J E T DEGEORGE, M: La paralysie auriculaire chronique. A p r o p o s d'un observation. Coeur Med Int 3:467, 1971 WADA, M, TAKADA, C AND MISE, J: A case report of atrial standstill. J a p Circulation J 30:543, 1966 NASI, C E LEGNANI,L: Paralisi atriale permanente con molteplici centri idioventricolari de sostituzione. Cardiol Prat 17:472, 1966

14. ROSEN, K M, RAHIMTOOLA,S H, GUNNAR, R M AND LEV, M: Transient and persistent atrial standstill with His bundle lesions: Electrophysiologic and pathologic correlations. Circulation 44:220, 1971 15. HASHIBA, K, MOCHINAGA, S, HUJIWARA, C, KONDO, H, HUKAYA, M AND OTSUBO, M: Persistent atrial standstill and U wave abnormalities in a case with scapuloperoneal amyotrophy. Jap Circulation J 44:220, 1971 16. NAGLE, R E, SMITH, B AND WILLIAMS, D O: Familial atrial cardiomyopathy with heart block. (abstr.) Proceeding of the British Cardiac Society. Br Heart J 34:205, 1972 17. BALDWIN, B J, TALLEY, R C, JOHNSON, C AND NUTTER, D O: Permanent paralysis of the atrium in a patient with facioscapulohumeral muscular dystrophy. Am J Cardiol 31:649, 1973 18. MIZUNO, Y, YASUI, S, TOBATA, I, TOYAMA, J, ANDO, K, WATANABE,T, HISHIDA, H, HAYASHI, H, INO, M, SAWADA, K, ITO, A, KOIKE, Y, HUTAMURA, Y, YOKOTA,M, TANAHASHI,Y AND TSUZUKI,J: Electrophysiologic and histological studies on patients with atrial standstill. The 70th Annual Session o f J a p Soc Int Med, 1973 19. ARITA, M, YAMAGUCHI,M, NAGASUE, S: A family of myocardiopathy with marked bradycardia. Shinzo 2:59, 1970 (in Japanese).

J. ELECTROCARDIOLOGY. VOL. 8. NO. 4. 1975