British Heart journal, I974, 36, 409-4I2.

Association of Wolff-Parkinson-White syndromewith congenital abnormalities of hands and feet

A. P. Niarchos', R. Finn, H. N. Cohen, and N. J. BuchFrom Royal Southern Hospital, Caryl Street, Liverpool

A female patient, 56 years old, with type A Wolff-Parkinson-White (WPW) syndrome and congenitalskeletal abnormalities of the hands and feet is described. Though all four limbs were affected, the patient'smain disability was caused by the defects of the right hand: this was short and hypoplastic, with hypoplasticphalanges, absent nails, syndactyly, and fibrous constriction rings on the fingers. She had no other evidence,apartfrom the WPW syndrome, ofcongenital heart disease. Chromosome studies were normal.

To our knowledge an association of the Wolff-Parkinson-White (WPW) syndrome with con-

genital skeletal abnormalities of the hands and feethas not been previously described.

Case report

A female patient, 56 years old, was admitted in February1973 because of a tachycardia associated with breathless-ness for the past 24 hours. She gave a history of a similarepisode, which occurred soon after the delivery of herdaughter, in I958, when the patient was 41 years old.Since then she had experienced short paroxysms ofpalpitation on several occasions, but these were notsufficiently prolonged to require any treatment. Inaddition she gave a history of having abnormalities ofboth hands and feet since birth (see below). There wasno history of diabetes, angina, hypertension, or anyother cardiac illnesses. The patient's mother had no ill-nesses during her pregnancy, had not taken any medi-cation, and the delivery of her daughter (patient) wasnormal. A history of two sudden deaths was present inthe family (see below).

Cardiovascular systemA regular tachycardia, I86 a minute, was present. Therewere no murmurs or other extra sounds. There was noevidence of cardiac hypertrophy. The jugular venouspressure was slightly raised, and fine basal crepitationswere heard at the bases of the lungs. The blood pressurewas 120/80mmHg. The electrocardiogram revealed a

nodal tachycardia of I87 a minute (Fig. I). A chestradiograph showed pulmonary oedema. Frusemide and

'Present address: Section on Hypertension, Henry Ford Hos-pital, 2799 W. Grand Blvd, Detroit, Michigan 48202, U.S.A.

digoxin were given intravenously but the tachycardiaeventually reverted to sinus rhythm with intravenouspractolol. The electrocardiogram now showed a type AWPW syndrome (Fig. i). A second chest radiographtaken when the patient was in sinus rhythm showedresolution of the pulmonary oedema. The heart and lungfields were normal; there was no evidence of congenitalheart disease.

Skeletal abnormalitiesa) Clinical findings The right hand was the mostseverely affected (Fig. 2) ofthe four limbs, and accountedfor most of her disability. The thumb was normal. Aconstriction ring was present at the base of the indexfinger which in addition had flexion deformity. Syn-dactyly and rudimentary phalanges with hypoplastic nailswere present in the middle and ring fingers. Small pitswere present in the dorsal and palmar aspects betweenthese two digits. The middle phalanx of the little fingerwas rudimentary, and the nail was absent. On the lefthand constriction rings were present at the base andmiddle of the middle phalanges of the index finger andmiddle finger. A constriction ring was present at the headof the middle phalanx of the ring finger. Hallux valguswas present on the right foot, the nail of the big toe wasabsent, the third and fourth toes were short and fusedwithout nails, and the nail of the fifth toe was rudi-mentary. The big toe of the left foot was short withhallux valgus deformity, the second toe was short, andrudimentary nails were present on the fourth and fifthtoes.

b) Radiological findings A radiograph of the righthand showed that the middle and distal phalanges of thethird and fourth fingers were absent, while the distalphalanges of the index and little fingers were very

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410 Niarchos, Finn, Cohen, and Buch

hypoplastic. The fourth and fifth metacarpals werehypoplastic (Fig. 2). The terminal phalanx of the leftmiddle finger was short. A radiograph of the right footshowed that the second and third phalanges of thesecond, third, fourth, and fifth toes,.-and the terminalphalanx of the big toe were absent, while the firstphalanx of all toes except the big toe were hypoplastic.The left foot was clubbed with similar, but less severeabnormalities.

Other abnormalitiesHypertelorism, prognathism with widely spaced teeth,and high arched palate were present. The left calf was3 cm thinner than the right.

Laboratory testsA full blood count was normal. The maximal values ofthe SGOT and LDH were normal (I5 and 62 inter-national units, respectively). Blood glucose, urea, uricacid, and electrolytes were normal. Cholesterol andlipoprotein electrophoresis were normal. Chromosomeanalysis on peripheral blood was normnal.

Family studyIn spite of a history of sudden deaths from heartdisease of the father~and brother of the propositus atthe age Of 2-5 and 23, respectively, no further in-information was available to confirm with certaintythe type of heart disease. The mother of the pro-POsitus is 78 years old. Her chest radiograph andelectrocardiogram are normal. The terminal phal-anges of the middle and ring fingers are hypoplasticon the right hand, and the left thumb is somewhatelongated. Minor right hand abnormalities wereprobably present in the brother of the propositus.The daughter of the propositus is 15 years old andand perfectly healthy, her chest radiograph electro-cardiogram being normal.

DiscussionIt has been well established that the 'WPW syn-drome is due to the presence of a congenital anom-alous and accessory atrioventricular pathway. Theevidence for this concept is based on electrocardio-graphic data., experimental studies., observationsduring cardiac catheterization and atrial pacing, andhistological studies. All this evidence was discussedin detail by Schamroth (I97I). In addition data fromHis bundle electrograms (Castillo and Castellanos,1970; Roelandt et al.., 11973)., and reports of success-ful surgical interruption of the bundle of Kent(Cobb et al., i968) support this concept. Further-more., the even distribution of the WPW syndromein all age groups., its presence in children and new-born infants., and its famnilial incidence and associa-tion with other forms of congenital heart disease,

strongly support a congenital origin (Schamroth,1971). It is very unlikely that the coexistence of theWPW syndrome and skeletal abnormalities in ourcase was due to a mere coincidence, since the inci-dence of the syndrome has been estimated as i6per thousand in the normal population (Averill,Fosmoe, and Lamb, i96o), while hand abnormal-ities were recorded in less than i per cent Of 430

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FIG. i Electrocardiogram showing nodal tachy-cardia and type A Wolff-Parkinson-White syndrome.

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Association of Wolff-Parkinson-White syndrome with congenital abnormalities of hands and feet 411

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FIG. 2 The right hand is small with hypoplasticfingers and nails. Syndactyly and constrictionrings are present. Hypoplastic toes are present on the rightfoot. For details, see text.

consecutive newborn infants with musculoskeletaldeformities but without heart disease (Bick, I960).The aetiology of most congenital defects, in-

cluding cardiac, is not known. Chromosomalanalysis was normal in our patient. Normal chromo-some studies, however, do not exclude a partialdeletion or inversion, changes which cannot bedetected with present techniques. It is of interest,that, not only in this case but also in the well-established cardiovascular-sekeletal syndromes (Holtand Oram, I960; McKusick, I966; Emanuel, 1970;Krikler, I973; Neill, I972), the hands are mostcommonly and more severely affected, while thefeet are very infrequently affected, and when so,show very minor defects. An embryological explana-

tion has been suggested for this preferential involve-ment by Holt and Oram (I960). The limb budsappear in the embryo during the fourth week, at thetime when the primitive heart tube is beginning todifferentiate, and the main development of bothlimbs and heart takes place simultaneously in thenext two weeks. The upper limb buds appear firstand their growth is completed earlier than that ofthe lower limbs (Arey, I965).The present case certainly has a similar distri-

bution of skeletal abnormalities, but whether itshould be considered as a variant of the cardio-vascular-skeletal syndromes, in which the con-genital heart lesion is the anomalous atrioventricularpathway, is too early to predict; the hypothesis is

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412 Niarchos, Finn, Cohen, and Buch

nevertheless under investigation in a larger group ofpati-nts with the WPW syndrome.

We are very grateful to Dr. S. Walker ofthe CytogeneticsUnit of the University of Liverpool for the chromosomestudies.

ReferencesArey, L. B. (i965). Developmental Anatomy, 7th ed. W. B.

Saunders, Philadelphia and London.Averill, K. H., Fosmoe, R. G., and Lamb, L. E. (ig60).

Electrocardiographic findings in 67,375 asymptomaticsubjects. IV. Wolff-Parkinson-White syndrome. AmericanJournal of Cardiology, 6, I08.

Bick, E. M. (I960). Congenital deformities of musculoskeletalsystem noted in the newborn. American Journal of Diseasesof Children, 0OO, 86I.

Castillo, C. A., and Castellanos, A., Jr. (1970). His bundlerecordings in patients with reciprocating tachycardias andWolff-Parkinson-White syndrome. Circulation, 42, 27I.

Cobb, F. R., Blemenschein, S. D., Sealy, W. C., Boineau,J. P., Wagner, G. S., and Wallace, A. G. (I968). Successfulsurgical interruption of the bundle of Kent in a patient

with Wolff-Parkinson-White syndrome. Circulation, 38,ioi8.

Emanuel, R. (I970). Genetics and congenital heart disease.British Heart3Journal, 32, 28i.

Holt, M., and Oram, S. (I960). Familial heart disease withskeletal malformations. British Heart3Journal, 22, 236.

Crikler, D. M. (I973). The cardiovascular system. In-ClinicalGenetics, 2nd ed., p. 409. Ed. by A. Sorsby, Butterworths,London.

McKusick, V. A. (I966). Heritable Disorders of ConnectiveTissue, 3rd ed. C. V. Mosby, Saint Louis.

Neill, C. A. (I972). Etiology of congenital heart disease. InPediatric Cardiology, Cardiovascular Clinics, 4: No. 3,p. I37. Ed. by M. A. Engle. Davis, Philadelphia.

Roelandt, J., Schamroth, L., Draulans, J., and Hugenholtz,P. G. (i973). Functional characteristics of the Wolff-Parkinson-White bypass. A study of six patients with Hisbundle electrograms. American Heart3Journal, 85, 260.

Schamroth, L. (I97i). The Disorders of Cardiac Rhythm,p. 233. Blackwell Scientific Publications, Oxford andEdinburgh.

Requests for reprints to Dr. A. P. Niarchos, Section onHypertension, Henry Ford Hospital, 2799 W. GrandBlvd, Detroit, Michigan 48202, U.S.A.

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