Four Cases of Right-Sided Bundle-Branch Block and One Case of Atrioventricular Block in Three Generations of a Family

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British Heart Journal, I970, 32, 50I-504. Br Heart J: first published as 10.1136/hrt.32.4.501 on 1 July 1970. Downloaded from

Four cases of right-sided bundle-branch block and one case of atrioventricular block in three generations of a family

Erik E. Simonsen and Edi G. Madsen From the Clinical Physiological Laboratory, The Municipal and County Hospital, Odense, Denmark

Four cases of complete right bundle-branch block and one case of atrioventricular heart block, all occuring within three generations of the same family, are described. The 4 patients with bundle- branch block had no symptoms, whereas the patient with atrioventricular heart block suffered Adams-Stokes attacksfrom the age of 13 and died at 47 years of age. A 33-year-old man with bundle-branch block had a normal electrocardiogram at the age of 7, suggesting that inherited bundle-branch block does not necessarily manifest itself during thefirst years of life.

Familial incidence of disturbances of conduc- Later in 1927 she was admitted to the Univer- tion in the heart has been described on rare sity Hospital in Copenhagen. The diagnosis of occasions. The authors have found reports atrioventricular block with Adams-Stokes attacks of 3I cases of atrioventricular block in ii was established. Objective investigation revealed a normally developed girl with no dyspnoea, families http://heart.bmj.com/ (Wendkos and Study, I947; Connor cyanosis, or signs of cardiac insufficiency. A sys- et al., I959; Wallgren and Agorio, I960; Lynch tolic murmur was heard in the second intercostal and Engle, I96I; Stephan, I96I; Combrink, space on the left side. The blood pressure was Davis, and Snyman, I962; Crittenden, Latta, go/60 mm. Hg. On 22 November I927 the elec- and Ticinovich, I964; Khorsandian, Mogha- trocardiogram showed third degree atrioventricu- dam, and Miiller, I964; Gazes et al., I965; lar block, and on 9 January I928 sinus rhythm Tsagaris, Bustamante, and Friesendorff, I967; with PR interval of OI4 sec. In both these electro- Veracochea et al., I967). In two of these cardiograms, the QRS complexes were normal. Until 1944, the patient had Adams-Stokes families (Combrink et al., I962; Gazes et al., on September 28, 2021 by guest. Protected copyright. four seizures at intervals of I to 2 years. After I944, I965), and three cases of right-sided these increased in frequency, and from 1951 she bundle-branch block, respectively, were en- had attacks at intervals of 2 to 4 weeks. In 1956 countered in other members of the families. she was treated with ephedrine hydrochloride Right-sided bundle-branch block was des- 20 mg. x 5 without any effect on the pulse rate or cribed in three and five cases in two families the Adams-Stokes seizures. (Mosetti, I954; Trivella and Chella, I960), Between attacks she had no cardiac symptoms. and left-sided bundle-branch block was At the age of 20 and 2I she had two normal preg- described in two and four cases in two nancies. At the age of 40, a therapeutic abortion families (Martinez Aparicio, I95I; DeForest, was performed on account of Adams-Stokes attacks. The frequency of the seizures did not I956). The present paper presents four cases increase during pregnancies. of right-sided bundle-branch block and one In I944, 195i, and 1956 she was admitted to case of atrioventricular block in three genera- the Medical Department B in Odense on account tions of the same family. of Adams-Stokes seizures. The stethoscopic findings were unchanged. The blood pressure varied Case reports between I40/90 and 110/70 mm. Hg. Neither Case I The patient was a 48-year-old female cardiac decompensation nor other complicating cashier. She was healthy until the age of I3 years, conditions were found. The appearance of the when she had to give up Girl Guide activities on electrocardiogram varied during all the periods account of recurrent syncope. In 1927, at i6 years in hospital. she was admitted to the local hospital because of In I944, the patient had third degree atrioven- syncope, with a pulse rate of 24 a minute. tricular block and bundle-branch block on the left Received 2a November I969. side. In 1951, sinus rhythm and right-sided 502 Simonsen and Madsen Br Heart J: first published as 10.1136/hrt.32.4.501 on 1 July 1970. Downloaded from bundle-branch block were present. On 7 January I956 the patient had second degree atrioventricular block, with normal QRS complexes, while, the I next day, third degree atrioventricular block and S:= AS;+I,. right-sided bundle-branch block were found, and I BI three days later, sinus rhythm and right-sided III wow ..:S bundle-branch block (Fig. I). X-ray examination of the heart in I956 revealed that the cardiothoracic ratio was 56. The increase in size was localized to the left ventricle IF~~~~~~~~~~~~~w.ii S.a; while the right ventricle and the pulmonary arch were normal. She died in i959 in her home at the age of 48 years presumably during an Adams-Stokes seizure. No necropsy was performed. Case 2 This patient, a housewife aged 38 years, was the daughter of Case I. She had always been FI G. I Electrocardiogram from Case I. The healthy, was capable of doing ordinary housework, upper one was recorded in 1951 and shows and could ride a bicycle without discomfort. She sinus rhythm and right-sided bundle-branch had two normal pregnancies at the ages of 23 and block. The lower one was recorded in I956 and 26 years. shows second degree atrioventricular block, with Investigations in I968 showed a normal woman. normal QRS complexes. Stethoscopic examination of the heart was normal and the blood pressure was II0/80 mm. Hg. Electrocardiographic examination revealed right- Case 5 This patient, the daughter of a sister of sided bundle-branch block (Fig. 2). X-ray examin- Case i, was a 2i-year-old typist and had always ation of the heart was normal. been healthy. She was capable of doing ordinary housework and could ride a bicycle without Case 3 This patient, a hospital porter aged 37 discomfort. years, was the son of Case i. He had never had Objective investigation in 1968 revealed a slen- rheumatic fever, diphtheria, or tuberculosis. der woman with neither dyspnoea nor cyanosis. At the age of 7 years, he was admitted on Stethoscopic examination of the heart was normal account of malaise. Objective investigation, and the blood was mm. pressure II5/65 Hg. http://heart.bmj.com/ electrocardiographic and x-ray examination of the Electrocardiogram showed sinus rhythm and thorax were normal. right-sided bundle-branch block (Fig. 2). Radio- In I965, at the age of 33 years, he was sub- graphic examination of the heart was normal. mitted to operation for acute appendicitis. During this episode the electrocardiogram indicated right- Family data The father of Case i died at the sided bundle-branch block. He had never had age of 68 years from myelomatosis, and the symptoms of cardiac disease and was able to fulfil mother died at the age of 28 years, according to his duties as hospital porter. the family, from Spanish influenza. No electro- Investigation in I968 showed a slender man. cardiographic recordings had been made in either Stethoscopic examination of the heart was normal, on September 28, 2021 by guest. Protected copyright. and the blood pressure was I20/80 mm. Hg. Electrocardiogram recorded at rest and during FIG. 2 Electrocardiogram showing sinus exercise showed sinus rhythm and right-sided rhythm and right-sided bundle-branch block bundle-branch block (Fig. 2). X-ray examination from Cases 2, 3, 4, and S. ofthe heart was normal. Right- and left-sided cardiac catheterization and angiocardiography indicated nothing abnormal. 9. :;W Case 4 She was the second of Case 3's children. V In I967, at the age of 7 years, she was admitted to the Pediatric Department on account of a systolic A murmur. She had always been healthy. -4S II The patient was a little thin, but otherwise --A,...... normally developed girl. Stethoscopic examination of the heart revealed a systolic murmur of III first degree in the second left intercostal space, A_ and the second heart sound was constantly re- duplicated. The blood pressure was 95/60 mm.

Hg. Electrocardiogram revealed sinus rhythm and vi :.: right-sided bundle-branch block. Radiography .., A of the heart showed normal findings. Right-sided .; .. .. cardiac catheterization and angiocardiography CASE 2 CASE 3 CASE 4 CASE 5 revealed normal conditions. *...... i...... : ...... S _ . . ^ Br Heart J: first published as 10.1136/hrt.32.4.501 on 1 July 1970. Downloaded from Familial right-sided bundle-branch block and atrioventricular block 503 of them. The authors have investigated 22 further under the age of I4 years, and 7 adults aged members of this family in view of electrocardio- 25-67 years with an average age of 45 years. graphic changes and evidence of heart disease. Only i of the I7 patients had dyspnoea and Normal conditions were encountered in all. One oedema which disappeared after implanta- member refused to co-operate in the investigation, and no indications for investigation ofthe husband tion of a pacemaker (Tsagaris et al., i967). of Case 5 were considered to be present as this Three of the 17 patients had children after couple were childless (Fig. 3). the diagnosis of atrioventricular block had been established. One patient reported by Khorsandian et al. (I964) completed three Discussion pregnancies without Adams-Stokes seizures, None of our patients have had infections or while, in one of the patients of Gazes et al. other diseases which might cause these con- (I965), the first Adams-Stokes seizure oc- duction disturbances. Therefore these must curred during the sixth pregnancy at the age be considered as genetically determined. of 35 years. Case i of the present series had Like Combrink et al. (I962) and Gazes et Adams-Stokes seizures before she became al. (I965), we find atrioventricular block as pregnant and no change in the incidence of well as right-sided bundle-branch block in seizures occurred during her pregnancies. As families with hereditary conduction disturb- far as can be judged from the cases presented, ances. pregnancy thus does not appear to predispose In Cases i and 2 of the patients with to Adams-Stokes seizures or increased inci- hereditary atrioventricular block described by dence of such attacks in patients with heredit- Tsagaris et al. (I967), alternation between ary atrioventricular block. atrioventricular block and sinus rhythm with Out of the I7 surviving patients with right-sided bundle-branch block was found. hereditary atrioventricular block, i had Among the I9 published cases of hereditary first degree block and 5 had second degree right-sided bundle-branch block, including block. Six of the i:i patients with third degree the present authors' cases, no transition from atrioventricular block had Adams-Stokes sei- sinus rhythm to atrioventricular block was zure and, in 3 of them, pacemakers were observed. It is therefore necessary to record implanted at the ages of 24, 46, and 59 years. several electrocardiograms to differentiate be- Artificial pacemaking has considerably im- tween the two groups of patients. proved the prognosis for patients with atrio- http://heart.bmj.com/ The I9 patients with hereditary right-sided ventricular block and, as such block may be bundle-branch block comprise IO male and 9 hereditary, it is reasonable to investigate female patients. Six of the patients were chil- parents and sibs of young patients with dren under the age of I4 years and 13 were bundle-branch block and atrioventricular adults aged I9-67 years, the average age being block. 38 years. None of the patients had symptoms of cardiac disease and no deaths were de- References scribed. The diagnosis was not established in Combrink, J. M., Davis, W. H., and Snyman, H. W. on September 28, 2021 by guest. Protected copyright. any case at birth, and in Case 3 in the present (i962). Familial bundle-branch block. American series no bundle-branch block was present Heart Journal, 64, 397. at the age of 7 years but was present at the age of 33 years. Hereditary right-sided bundle- FIG. 3 Pedigree offamily. branch block is, therefore, not necessarily congenital. I The 32 published cases, including Case i from this series, with electrocardiographically confirmed atrioventricular block comprise 2I II male and ii female cases. Six of them died in infancy and 8 died between 3-65 years, the average duration of life being 3I years. Of the 8 patients, 7 survived from i day to 3 III years after the first Adams-Stokes seizure. Case i in the present series thus differed from the remainder in that she survived for 35 years after the first Adams-Stokes seizure. A IV further patient died because of failure of an implanted pacemaker. at (28)=Age at death Seventeen of the patients were alive at the (=Not examined 59-Age investigation time of publication, including io children g=Bundle-branch block *=Third degree atrioventricular block Br Heart J: first published as 10.1136/hrt.32.4.501 on 1 July 1970. Downloaded from 504 Simonsen and Madsen

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