Tricuspid Atresia

and hepatic disease as a possible manifestation of a 1-antitrypsin deficiency. In addition, it should be noted that

hepatic disease may be present", even though the profile of hepatic function is normal A liver biopsy with appropriate staining techniques is advisable in aD such cases. ACKNOWLEDGMENT: Pbenotyping studies were dooe through the courtesy of R. M. Iammarino, M.D., of the Clinical Laboratory, University Health Ceuta- of Pittsburgh; and in the Jabor8tory of studies for veriBcation were Hopkins University, BaltiRichard Talamo, M.D., of J more.

cr:oamed

REFERENCES 1 Eribsoo S: Studies in alpbat antitrypsin defideDcy. Acta Med Scand 177:1-85, 1965 2 Hutchison DCS, Coole PJL. Barter CE, et al: Pu1mooary emphysema and alphat antitrypsin de8ciency. Br Med J 1:689-694, 1971 3 Jooes MC, Thomas GO: Alphat antitrypsin defiaeDcy and pu)monary emphysema. Thorax 26:651-862., 1971 4 Sharp HL, Bridges RA, Krivit W: Cirrhosis associated with a]phat antitrypsin deBcieDcy: A previously umecognized discxder. J Lab Clin Med 73:934-939, 1969 5 Johnson AM, Alper CA: Deficiency of alphat antitrypsin in chiJdhood liver disease. Pediatics 46:921-WS, 1970 6 Porter CA, Mowat PJL, et al: AJphat antitrypsin de8ciency and DfJODatal hepatitis. Br Med J 3:435-439, 1972 1 Cohen :n., Rubin PE, Echevarria RE: AJphat aotitrypsin deficiency, emphysema and cirrhosis in an adult. Ann Intern Med 78:227-232, 1973 8 Campra JL. Craig JR, Peters RL, et al: Cirrhosis associated with partial de8ciency of alphat antitrypsin in adult. Ann Intern Med 78:233-238, 1973 9 Loogstreth GF, Weitzmali SA, BrowniDg RJ, et al: BroncbiectasU and homozygous alphat-autibypsin de6cJeDcy. Chest ff1 :233-235, 1915 10 Glasgow JFT, L)'Dcll MJ, Hercz A, et al: Alphat aut:itrypsin deficiency in association with both cirrhosis and chronic obstructive hmg diaeue in two siba. Am J Med 54:181-194, 1973 11 Gherardi GH: Alphat antitrypsin deficiency and its effecb on the liver. Hum Patbol 2:173-175, 1971 12 lshalc KG, }enis EH, ManbaD ML, et al: Cirrhosis of the liver associated with a)pha.t antitrypsin de8ciency. Arch Patbol94:445-454, 1912 13 KUIIW.' P, I.anca""H'-8mith M, Cook PJL. et al: Alphat antitrypsin de8ciency in chroDic liver disease and a report of cinhosis aDd empbyaema in adult members of a family. Br Med J 1:366-367,1974 14 Fagerhol MK: Ira Mittman C (eel): Proteolysis aud PulIDODUY Emph,.ema. New York, Acadenric Press, IDe, 1972, pp 145-149 15 Alper CA, Jolm8oo AM: Immn... Jimioo e1eclropboresis: A tedmique far the study of protein polymorphism. Voa Sang 17:445, 1969 16 Lieberman J: Alpba.l antitrypsin de8ciency. Med CJin North Am '$7:891-706, 1973 17 Fieldman G, Biguon J, Chabinfan P, et al: Hepatocytes ultrastructural cbauges in alpbal antitrypsin de8ciency. GutroenlmoJogy ff1: 1214-J.D4, 1975 18 Berg NO, Eriksson S: Liver dtseue in adulb with alphat antitrypsin de&c.ieDcy. N Eng} J Med fJJ7: 1284-1287, 1972 19 Lieberman J, Geidulis L, Klotz SD: A oew deficient var. iant of alphat antitrypsin ( MDuuu). Am Rev Respir Dis 113:31-36, 1976

538 SIIAIIATZADEII ET AL

Tricuspid Atresia* A Review of 68 Cases All N. Shariatzadeh, M.D., F.C.C.P.; Harold King, M.D., F.C.C.P.; Doratlld Girod, M.D.; and H. B. Shumaclcer, M.D. Sbty-el&ht patlems with tricuspid atresia were seen at I....._ Uaivenity dlll'iD& tile .-st 30 yan. Lolli term IUrYivon treated wldloat opendioll emeqed from oar renew. ID tbe8e, tbere was a combiuatioa of tricaspld ldresla ........... lllidoa of tbe ...- vaaels.

accepted that the prognosis for patients Jt withgenerally infancy poor. Most die biruspid atresia is

in

is

from problems stemming from inadequate pulmonary blood flow. Palliative surgery has been associated with a high mortality. A recent review of patients with this congenital heart abnormality, observed and treated at this institution, confirms these generaDy held impressions in patients with tricuspid atresia and normally-related great arteries. However, patients with triruspid atresia and transposition of the great arteries fared much better. Many have not required operative intervention and have lived a relatively long life. CuN!CAL MATERIAL

A total of 68 patients with documented bicuspid atresia were seen at the Indiana University Medical Center in the past 30 years. Thirty-seven were boys and 31 girls. Anum-

ber of other anomalies were revealed by cineangiocardiographic data. These included venbicular septal defects in 60, transposition of great vessels in 22, patent ductus arteriosus in 12, anomalous pulmonary venous return in 4, and rare anomalies such as interrupted aortic arch and truncus arteriosus. Of importance was the presence or absence of

associated

transposition of the great vessels. In the 46 patients without transposition, 39 required creation of some form of systemic-pulmonary shunt with the overall hospital mortality of 27 percent. BJalock-Taussing shunt carried the lowest mortality ( 12 percent) and was the most frequently used operation. Waterson-Cooley shunt was used in smaller infants (mortality, 70 percent), and the Glenn shunt in larger children (mortality, 20 percent). The remaining seven were treated medically. Four of the seven died in infancy and one died several years later. Two are alive and doing fairly well (Table 1 ) . Of the 22 patients with associated transposition, nine were treated with one of several surgical procedures. Six died during or following operative intervention and three are still alive. The problem of concern in this group was increased pu)monary blood flow which required pulmonary artery banding. A few required systemic-to-pulmonary shunt. Of interest was a subgroup of 13 who were treated medically; there were only two deaths (Table 2) in this group. •From the Departments of Thoracic and Cardiovascular Surgery, and Pediatric Cardiology, Indiana University Medical Center, IndianapolU . Eoonstora, 800 A~ Reprirat requem: Dr. S~. Illmoii601JJ2

CHEST, 71: 4, APRIL, 1977

Ductus

Age

Size of VSD

Pulmonary Circuit

Arterio8us

16 yrs

large

pulmonary stenosis

closed

76%

Alive

16 yrs

large

normal

patent

58%

Dead

7yrs

moderate

normal

closed

80%

Alive

9

moderate

normal

patent

57%

Dead

4 mos

none

small

patent

54%

Dead

3

none

small

patent

68%

Dead

moderate

small

closed

?

Dead

11108

lll08

15 days

Preeent

Status

somewhat mu.scu1ar right atrium, a slit-like and hypoplastic right venbicle and finally, complete atresia of the tricuspid valve. The presence of a venbicular septal defect ( 90-95 percent) allows for a varying degree of development of right ventricular out8ow tract~ and it is the size of this defect which determines the large builc of puhnonary blood flow. Our review would suggest that patients with bicuspid atresia and transposition of great vesseJs frequently have a systemic-to-pulmonary blood flow ratio that is compatible with a relatively good prognosis. A few long-term survivors with this combination of defects have been reported in the literature. Jordan ahd Sanders" in 1966 reviewed the world literature and found 19. They added two: one, age 33, with transposition and a ventricular septal defect, the other, age 57, with ventricular septal defect and subpuhnonary

DiscuSSION

Tricuspid atresia is a rare congenital anomaly. Abbott1 found the incidence to be 2.1 percent in postmortem examination of 1,000 patients with congenital heart disease. It is thought to occur when the ventricular septum fails to shift to the left in the developmental stage. 2 The anatomic classification is somewhat confusing. The most widely accepted theory is that of Edwards and Burchell. 8 They described two types: one with, and the other without transposition of the great vessels. Subgroups were later ddined which divided the two major groups into classifications by varying size of pulmonary conduits, ranging from complete atresia to a nearly normal right ventricular outflow ~ pulmo~ valve and artery. Common to all forms of bicuspid atresia is the presence of interabial communication, an enlarged and Table 2---TidiCeen Pfdlenu ~RIA~

Systemic(), Saturation

Tf'fPUporlalioa of*Great Y __,. T,_,. N,..,_..,;WJIT

Systemic Ot

Size of VSD

Pulmonary Circuit

28yrs

large

subpulmonic stenosis

cloeed

87%

Alive

17 yrs

large

normal

closed

72%

Alive

closed

80%

Alive

closed

78%

Alive

Age

15 yrs

moderate

normal

15 yt'8

large

stenosis, left

Ductus

A.rteri08U8

pulmonary artery

Saturation

Preeent

Status

14 yrs

moderate

normal

closed

80%

Alive

13 yrs

moderate

normal

patent

64%

Alive

7yrs

large

normal

e1oeed

85%

Alive

7yrs

small

normal

closed

78%

Alive

4yrs

large

pulmonary valve stenoais

cloeed

82%

Alive

4yrs

large

normal

closed

78%

Alive

2yrs

large

normal

closed

74%

Alive

3moe

large

pulmonary valve stenosis

closed

80%

Dead

normal

patent

87%

Dead (Interrupted aortic arch, renal anomalies)

3mos

small

CHEST, 71: 4, APRIL, 1977

(No Auwpey)

TRICUSPID ATRESIA 531

stenosis. The longest survivor in our group is a 28-yearold man who bas led a moderately active life. Ventricular septal defect was present in the subgroup with transposition of the great vessels. It was of a moderate-to-large size in the majority of patients. It was the presence of this defect which allowed for a nearnormal pulmonary blood flow. It appears that although the patients in this group were more anatomically derange
1 Abbott ME: Atlas of Congenital Cardiac Disease. New York, American Heart Association, 1936 2 Nadas AS, Fyler OC: Pediatric audiology (3rd ed). Philadelphia, Saunders, 1972, p 298 ~ Edwards JE, Burchell HB: Congenital tricuspid atresia: a classification. Med Clin N Am 33:1177, 1949 :4 Jordan JC, Sanders CA: Tricuspid atresia with prolonged survival, a report of two cases with a review of the world literature. Am J Cardiol 18:112, 1966 ~ Tandon R, Edwards JE: Tricuspid atresia. J Tborac Cardiovas Surg f57:530, 1974

Clinical Pulmonary Tuberculosis in an Asthmatic Patient using a Steroid Aerosol*

include: oral candidiasis, which responds well to topical antifungal medications; 2 voice loss and hoarseness, which may represent a local steroid myopathy;" and exacerbation of rhinitis or eczema, which previously was controlled by use of systemic steroids. 5 If dosage of systemic steroids is reduced, symptoms of steroid withdrawal and adrenal insufBciency may occur. 4.5 This report suggests another possible complication of steroid aerosols. A patient who developed pulmonary tuberculosis while receiving a steroid aerosol for treatment of asthma is described. CASE

REPoRT

A 43-year-old housewife was referred to National Jewish Hospital and Research Center ( NJHRC) in July, 1974 for control of asthma of nine years' duration. Her symptoms were perennial and exacerbated by respiratory infections and irritant exposure. Since November, 1973 she had become steroid-dependent, receiving dexamethasone 0.6 to 1.5 mg daily. Isoniazid ( INH) was also given for tuberculosis chemoprophylaxis, but was discontinued because of gastrointestinal distress. A tuberculin skin test was not applied before starting the INH. She had no history of prior tuberculosis exposure, infection or skin tests. Physical examination was unremarkable except for her cushingoid appearance, expiratory wheezes throughout the chest, and lower extremity proximal muscle weakness. Chest roentgenograms revealed scattered calcifications. Pulmonary function tests showed obstructive airways disease responsive to administration of bronchodilators. Histamine and methacholine inhalations fell within the asthmatic range. 8 Immediate hypersensitivity skin tests gave negative results. Her intolerance to the conventional bronchodilators made daily steroid use her mainstay of therapy, but she experienced side effects, including gastrointestinal disturbances, depres-

Douglas ]. Horton, M.D. •• and Sheldon L. Spector, M.D., F.C.C.P.t

A patieat who W8l receivblg a steroid aeroeol for treatment of asthma deYeloped diJdcal ,.._,_., tuherculotds. Coatiaaed adadDisandioa of the steroid 8ei'OIOI aloD& with aatitaberealolis chealothe111py did aot adYenely illluellee ...... of the piiiiDoaary ...._

corticosteroid (steroid) aerosols T hewithhalogenated degree of topical activity relative their

high to their systemic effects have proved helpful in the management of certain patients with reversible obstructive airways disease (asthma) .1 •2 With efferove doses, adrenocortical function is usually not suppressed, and the adverse effects of systemic steroids are avoided. 8 Complications of administration of steroid aerosols •From the Department of Medicine, Section of Allergy, Clinical Immunology, and Chest Diseases, National Jewish Hospital and Research Center, Denver. Supported in part by Allergic Disease Center Grant No. Al-10398. • •Fellow, Allergy and Clinical Immunology. t Head, Section of ADergy and Clinical Immunology. Reprint requests: Dr. Horton, 3800 East Colfax, Denver 80!?JJ6

540 HORTON, SPECTOR

FIGURE 1. Chest roentgenogram of November 9, 1974, showing scattered calcifications in the lung fields.

CHEST, 71: 4, APRIL, 1977