tricuspid atresia in adults

REVIEWS

Tricuspid Atresia in Adults

WILLIAM PATTERSON, MD, PhD, FACCWILLIAM A . BAXLEY, MD, FACCROBERT B . KARP, MD, FACCBENIGNO SOTO, MD, FACCLIONEL L . BARGERON, MD, FACC

Birmingham, Alabama

From The Departments of Medicine, Surgery,Radiology and Pediatrics, University of AlabamaMedical Center, Birmingham, Alabama . This in-vestigation was supported in part by Grants SCOR5-P50-HL17667 and Program Project 2-P01-FL11310 from the National Heart, Lung, and BloodInstitute, National Institutes of Health, Bethesda,Maryland; The Vocational Rehabilitation Service,Birmingham, Alabama ; and the U.S . VeteransHospital, Birmingham, Alabama . Manuscript re-ceived February 17, 1981 ; revised manuscriptreceived July 7, 1981, accepted July 14, 1981 .

Address for reprints : William A . Bailey, MD,Cardiology Division, University Hospital, Bir-mingham, Alabama 35294 .

Congenital atresia of the tricuspid valve is still uncommon in adult patients .However, increasingly successful palliative surgery in children now hasincreased its incidence after age 15 years. This investigation updates theclinical features of this disease in adults in light of modern diagnostic andsurgical techniques . The data on all 18 adults with tricuspid atresia havingangiography after age 15 years at this Institution since 1970 were re-viewed. The patients' ages ranged up to 45 years; 12 had had previouspalliative surgery . Left cineventriculography, particularly biplane, withthe long axial view (60 0 left anterior oblique with cranial angulation) isthe most important diagnostic mode and reveals the ventricular and greatvessel relations . According to standard classification, 11 patients had typeI anatomy (normal great arterial relations), 4 type II (transposed greatarteries) and 2 type Ill ("corrected transposition of the great arteries") .One patient with inverted ventricles could not be classified . Associatedadditional congenital defects were uncommon. On the basis of these data,a new anatomic classification of tricuspid atresia is given which en-compasses all possible atrial-ventricular-great arterial combinations .

Seven patients had further surgery after study, including two proceduresof the Fontan type (right atrium to pulmonary arterial conduit) . Follow-updata on all 18 patients revealed two deaths (one early after operation,one late after study without further surgery) . The remaining 16 patientssurvive 2 to 120 months after study. Four patients had naturally balancedpulmonary and systemic circulations and have survived to ages 21 to 41years without surgery. Prudent surgical decision based on accurate an-atomic diagnosis and the need for optimal effective pulmonary blood flowmay result in a relatively optimistic prognosis in adults with this dis-ease .

Tricuspid atresia is a severe, complex and uncommon cyanotic congenitalheart defect, encountered at birth in 1 to 3 percent of cases of congenitalheart disease .' ,' It never appears as an isolated anomaly ; as such it wouldbe incompatible with life. The following features are present in all cases :(1) atresia of the communication between an atrial chamber and themorphologic right ventricle, (2) an atrial and ventricular septal defect,(3) an enlarged mitral valve and morphologic left ventricle, and (4) hy-poplasia of the morphologic right ventricle . Features that vary include(1) size of the ventricular septal defect, (2) anatomic positions of theventricles, (3) spatial relations and connections of the great vessels, and(4) the presence or absence of obstruction to pulmonary blood flow . Inaddition, tricuspid atresia has been interpreted as a form of univen-tricular heart . :'

Survival in patients with this disorder is primarily related to the ad-equacy and efficiency of pulmonary blood flow, 4'r' and surgical proce-dures in children have been increasingly successful in improving thishemodynamic variable. Thus, a larger number of patients with thisdisorder now survive to adulthood .°=' Although there is a body of pub-lished data concerning childhood tricuspid atresia, little material isavailable concerning the adult forms . Adult patients are often evaluatedby adult cardiologists less familiar with complex congenital disease than

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TRICUSPID ATRESIA IN ADULTS-PATTERSON ET AL .

TABLE I

Clinical Features of 18 Patients with Tricuspid Atresia

Hct = hematocrit ; Mod = moderate; NYHA = New York Heart Association ; PA = pulmonary arterial .

their pediatrician colleagues. Furthermore, recent ad-

vances in angiographic equipment and techniques8 '9

now make precise anatomic evaluation possible even inlarge adults, so that more rational surgical decisions can

be made. The present report is a review of the clinicalfeatures of 18 patients with tricuspid atresia undergoingangiographic studies at the University of Alabama afterage 15 years. Particular emphasis is placed on the ana-tomic classification and the optimal angiographic for-

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mat for detailed diagnosis in adults, the available sur-gical options and survival data .

Methods

Study patients : Clinical data for all 18 patients with tri-cuspid atresia undergoing complete evaluation with angio-graphic studies at age 15 years or older were analyzed. Thesepatients were evaluated for possible surgery at the University

FIGURE 1. Left ventriculograms in a patient with right-sided tricuspid atresia, shus solitus of the atria, levocardia and left aortic arch (type I anatomy 10 ).A, lateral view and B, frontal view . The enlarged left ventricle (LV) is located posterior and to the left of the small right ventricle (RV) . Note the absenceof a connection between the right atrium and right ventricle. The aorta (AO) arises from the left ventricle and the pulmonary artery (PA) from theright ventricle . The septa) communication between the right and left ventricles (arrows) is enough to maintain good flow through the pulmonaryarteries .

CaseAge (yr)& Sex

AnatomicType Cyanosis

FunctionalClass(NYHA)

Hct(%)

ArterialOxygen(%)

Mean PAPressure(mm Hg)

1 15F IB Mod II 50 842 15M IIB Mod III 54 803 16M IB Mod 111 614 17F IIIB Mod II 53 805 21M IA Mod II 686 23F IB Mod II 77 74 157 34M IIB Mod II 60 78 168 18F IA Mild III 54 70 279 22M IB Mod II 60 80

10 23M IC Mild III 52 82 3511 27F (?) B None I 92 1112 29M IB Severe III 7913 29M 1113 Mod II 70 7314 45M IB Mod II 63 81 2715 18F IIB None I 85 2516 27M IIIB Mod li 60 76 2017 35F IC Mild II 58 79 1218 38F IC Mild II 45 83 20

of Alabama from 1970 through 1980 . The anatomy was ini-tially classified by the standard scheme modified from thatof Keith et al . 10 emphasizing the atrioventricular (A-V) andventriculoarterial connections . Patients with type I tricuspidatresia have atrial situs solitus, normally related ventricles andnormally connected great arteries (ventriculoarterial con-cordance) . Patients with type II have associated complete

A

FIGURE 3 . Left ventriculogram in frontal (A) and lateral (B) projections in a patient with right-sided tricuspid atresia, situs solitus of the atria, levocardiaand left aortic arch (type II anatomy with transposition of the great arteries) . The left ventricle (LV) is located posterior and to the left of a small rightventricle (RV). The mitral valve (black arrowheads) connects the left ventricle with the left atrium . The pulmonary artery (PA) originates from theleft ventricle. A large septa) defect (white arrow$) connects the right and left ventricles and provides a large amount of blood to the systemic circulation .The aorta (AC) arises from the small right ventricle. The aorta and pulmonary artery are in anteroposterior relation and parallel .

110

Ir

FIGURE 2. Left ventriculogram in a patient with atresia of the right tricuspid valve and atresia of the pulmonary artery . The aorta (AD) originatesfrom both ventricles, overriding the ventricular septum . A small right ventricle (RV) is located anteriorly and to the right . A, long axial view and B,elongated right anterior oblique view . The left ventricle (LV) is connected with the left atrium through a mitral valve (mv) . The anterior aspect ofthe left ventricle in B is formed by the septum, which appears in profile . A ventricular septa) defect (arrow) joins the left ventricle and the smallright ventricular chamber (RV) . In other angiograms, the pulmonary artery was visualized through collateral arteries originating from the upper de-scending aorta as a nonconfluent structure . Diagnosis: atrial solitus, concordant atrioventricular relation in parallel fashion ; absence of the righttricuspid valve ; single outlet of the heart ; aorta originating from both ventricles ; pulmonary atresia with nonconfluent pulmonary branches .

TRICUSPID ATRESIA IN ADULTS-PATTERSON ET AL,

transposition of the great arteries or ventriculoarterial dis-cordance. Those with type III tricuspid atresia have both ANand ventriculoarterial discordance . (The term "associatedcorrected transposition of the great arteries" has also beenused for this type .) The three types are subclassified accordingto the degree of obstruction to pulmonary flow : Subtype A,pulmonary atresia; subtype B, pulmonary or subpulmonary


1 43

TRICUSPID ATRESIA IN ADULTS- PATTERSON ET AL .

stenosis, and subtype C, no obstruction to pulmonary flow .Angiocardiography: All angiographic data were studied

in detail to determine the best . radiographic technique forprecise diagnosis, with definition of surgical anatomy theforemost consideration . The adequacy of the standard ana-tomic classification system was then evaluated in light of thishigh quality angiography. Surgical procedures both before andafter study here were reviewed and follow-up contact wasmade for survival information .

Results

Clinical diagnostic data (Table I) : The 18 patientsranged in age from 15 to 45 years at the time of cathe-terization study, and the sex distribution was nearlyequal. All were studied because of progressive cardiacsymptoms or uncertainty regarding the diagnosis . Twohad survived bacterial endocarditis and one a para-doxical embolus to the brain . Twelve patients had hadone or more previous palliative surgical procedures .Sixteen patients had resting cyanosis and significantfunctional limitation in their daily activities . None hadsyncope or chest pain .

Cardiac enlargement was documented by physicalexamination in 12 of the 18 patients. Systolic murmursof varying intensity were heard in all patients, usuallymaximally along the left sternal border, and a bruit fromthe previous shunting operation was present in 9 of the12 who had had these surgical procedures .Pulmonary blood flow as revealed by plain chest

X-ray study varied depending in part on a history ofprevious palliative surgery . The cardiac silhouette in theX-ray film was increased in transverse diameter in 15of the 18 patients .

FIGURE 4 . Angiograms in a patient with atresia of a left tricuspid valve (type III), situs solitus of the atria, dextrocardia and left aortic arch . A, rightventriculogram In frontal view . The morphologic right ventricle (RV) is seen on the left (and inferior to the left ventricle as revealed in comparingwith view in B). The aorta (AO) originates from the right ventricle through a well developed infundibular septum and ventriculoinfundibular fold . Theright ventricle is connected with the left ventricle through a large ventricular septal defect not on profile in this angiogram (arrowheads) . No inlet(tricuspid) valve Is connected with this ventricular chamber . B, left ventriculogram in frontal view . The left ventricle (LV) is connected with the rightatrium through a large mitral valve (outlined by the catheter) . The pulmonary artery (PA) arises from the left ventricle . The main pulmonary arteryis located to the right of the aorta . The right and left pulmonary arteries are enlarged . C, levophase of the left ventriculogram of B . The left atrium(LA) is well outlined and connects with the right atrium (RA) through a large atrial septal defect (ASD) . The connection between the left atrium andthe inferiorly located right ventricle (see A), is absent (arrowheads), showing the afresla of the left tricuspid valve .

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The electrocardiogram showed left ventricular hy-pertrophy by voltage criteria in 15 of the 18 patients,with an associated T wave "strain" pattern in 5 of these15 . Eight had left axis deviation. Right axis deviationwas present in the two patients with type III tricuspidatresia. All but two patients showed electrocardio-graphic evidence of right or left atrial enlargement., orboth .

Arterial oxygen saturation generally reflected thedegree of cyanosis, and the hematocrit was greater than55 percent in 10 of the 16 patients in whom values couldbe retrieved . Mean pulmonary arterial pressure wasincreased above 20 mm Hg in 4 of the 10 patients inwhom the pulmonary artery was entered ; however, nonehad a pressure greater than 35 mm Hg . No patient wasjudged to have excessive pulmonary vascular resis-tance .

Angiographic classification (Fig. 1 to 4): Elevenpatients were categorized by angiography as having typeI tricuspid atresia, 4 type II and 2 type III . One patient(Case 11) did not fit this classification, because he hadA-V discordance or inverted ventricles (the right atriumleading through a mitral valve into the left ventricle,with atresia of the opposite atrioventricular valve ; hehad concordant ventricular-great vessel connections)(Fig . 5) .Associated congenital anomalies: Associated

anomalies were uncommon in this group of patients .Two patients had patent ductus arteriosus. There wasone occurrence each of primum atrial septal defect andjuxtaposition of the atrial appendages . One patient withtype III tricuspid atresia had dextrocardia and a per-sistent left superior vena caves Four patients had left

ventricular dysfunction as revealed by decreased ejec-tion fraction and elevated ventricular filling pressure .The condition was apparently secondary to the volumeoverload from a previous palliative shunting procedurein three of these four . Also, three of these were amongthe only four patients over age 30 years . Two patientswith a previous Glenn superior vena cava-pulmonaryarterial shunt had thromboses in some pulmonary ar-terial branches. One patient had an incompletelyfashioned Glenn shunt with some flow from the superiorvena cava to the right atrium . All patients had evidenceof reduced pulmonary blood flow some time during theircourse, and none had excessive pulmonary blood flow


(the three patients with subtype C, without associatedpulmonary stenosis, had restrictive ventricular septaldefects limiting flow) . All four patients with discordantA-V relations (type Il or associated transposition of thegreat arteries) had pulmonary stenosis (subtype B) butall also had an "overriding aorta," so that none hadaortic or subaortic stenosis . Pulmonary atresia wasencountered in only two patients ; in both, the aortaoriginated from the left ventricle.

Surgical data (Table II) : The previous proceduresfor surgical palliation in these patients varied and do notnecessarily reflect current practice at this institution .Palliative operations had been performed in 12 of the

FIGURE 5 . Left ventricular angiograms In a patient with atresia of a left tricuspid valve, situs solitus of the atria, levocardia and left aortic arch .A, frontal projection . The left ventricle (LV) receives blood from the left atrium through a large mitral valve . The aorta (AO) arises from the left ventricle .There Is septa) defect (ad) which connects the left ventricle with the small right ventricle (RV) . The pulmonary artery (PA) arises from the smallrudimentary right ventricle which Is located anteriorly and to the left . B, levophase of the left ventriculogram, frontal view . The left atrium (LA) isconnected with the left ventricle through a mitral valve and with the right atrium through an atrial septa) defect . C, lateral view . The left ventricleis a large chamber located posterior to the small rudimentary right ventricle . Septal defects (white arrows) connect the left and right ventricles .The aorta and pulmonary artery arise in concordant ventriculoarterial connection . The aorta is posterior to and to the right of the pulmonary artery .D, levophase of the angiogram in C . The left atrium is connected with the left ventricle through the mitral valve . Diagnosis : situs solitus of the atria,discordant atrioventricular relation, with atresia of the left tricuspid valve and ventriculoarterial concordance .


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TABLE IISurgery and Survival

1 46

B-T = Blalock-Taussig shunt ; GL = Glenn procedure ; MP = modified Potts procedure; PA = pulmonary artery .

18 patients . Initial procedures in the patients as childrenless than 10 years of age had included six Blalock-Taussig anastomoses from the subclavian to the pul-monary artery, one Potts aortopulmonary anastomosis,three Glenn shunts from the superior vena cava to theright pulmonary artery and one pulmonary valvuloto-my. Subsequent operations that had been required 1 to14 years after the initial palliative procedure includedone Blalock and one Glenn shunt and six modified Pottsanastomoses (distal end of the left pulmonary artery toside of the descending aorta) . One patient had under-gone three operations before study at this institution .Two patients had had the initial palliative procedure

A B

POSSIBLE GREAT ARTERIES CONNECTIONS

!'Concordant] Discordant) Double Outlet Single Oulletl

FIGURE 6 . New proposed scheme of anatomic classification for tri-cuspid atresia . This scheme permits all varieties of atrioventricular andventriculoarterial relations to be included . A, situs solitus with con-cordant atrioventricular connections . B, situs solitus with discordantatrioventricular connections . L A = left atrium ; LV = left ventricle ; RA= right atrium ; RV = right ventricle .


during adulthood after survival to age 16 years withoutsurgery.Seven patients underwent further surgery after

study here. In three of these this was a third surgicalprocedure . Multiple types of procedures were utilizedincluding a modified Potts anastomosis, a Blalock-Taussig procedure, and vein and Gore-Tex® grafts fromthe aorta to the pulmonary artery . One patient had aFontan procedure with a right atrial to pulmonary ar-terial conduit. One patient received a Dacron® conduitfrom the right atria] appendage to the right ventricularinfundibulum (modified Fontan procedure) 14 yearsafter an initial Blalock shunt. In one of two patients(Case 4) with both A-V and ventricular-great vesseldiscordance (type III tricuspid atresia or associated"corrected transposition" of the great arteries) spon-taneous closure of a Blalock anastomosis had developed ;adequate pulmonary blood flow was reestablished byatrial septectomy and pulmonary valvotomy.

Survival data (Table II) : These data were availablein all 18 patients. Sixteen of the 18 patients were alive2 to 120 months (mean 49) after evaluation. Of sevenwho had surgery after study, one, aged 34 years, diedfrom unrecognized cardiac tamponade I month after aFontan procedure and the other six are alive 18 to 120months postoperatively. Of the 11 who had no surgeryafter study, 1 died nearly 4 years later. Her anomaly hadbeen judged adequately palliated by two previous pro-cedures, and she died from cardiac complications as-sociated with gynecologic surgery . The remaining 10patients survive 2 to 77 months after study. Six of these10 had previous palliative surgical procedures ; however,

Case

Ages (yr) and Types ofPrevious Operations Subsequent

OperationAfter Study

SubsequentSurvival(mo)1 2 3

Patients With Surgery After Study1 1 (&T) Modified Fontan Alive (25)2 1 (GL) 8 (MP) Gore-Text graft, aorta to left PA Alive (18)3 1 (GL) 12 (MP) Vein graft to left PA Alive (46)4 2 (6-T) Atrial septectomy & pulmonary valvotomy Alive (110)5 2 (B-T) 12 (GL) Modified Potts Alive (89)6 None B-T Alive (120)7 None Fontan Died (1)

Patients With No Surgery After Study8 2 (B-T) 3 (MP) None Died (46)9 1(B-T) None Alive (27)

10 6 (GL) 20 (MP) None Alive (48)11 7 (B-T) None Alive (77)12 2 (B-T) 12 (B-T) 19 (MP) None Alive (29)13 16 (GL) 24 (B-T) None Alive (52)14 16 (B-T) None Alive (11)15 None None Alive (60)16 None None Alive (40)17 None None Alive (2)18 None None Alive (36)

FIGURE 7 . Glenn anastomosis, left innominate vein(LIV) angiograms . A, frontal and B, lateral views . Theright superior vena cava (RSVC) has been connectedto the right pulmonary artery (RPA) in end-to-endfashion (arrows) . Collateral veins bypassing theanastomosis are now present from a left superiorvena cave (LS) to the right atrium through the coronarysinus (CS) .

4 had none and are surviving at ages 21 to 41 years, stillwithout surgery . (Furthermore, anatomic types I, II andIII are represented by these four patients .)

Discussion

Clinical FeaturesThe results of this review indicate that adults with

tricuspid atresia are generally referred for study andconsideration of surgery because of weakness, inap-propriate dyspnea and cyanosis with various degrees offunctional disability. Fluid retention, chest pain syn-dromes and syncope are not common . However, it isimportant for all adults with complex congenital heartdisease to have a complete catheterization/angiographicstudy to establish the basic pathoanatomic and physi-ologic features, regardless of symptoms . Even if a studywere done in childhood, time-related changes in theheart, plus improvement in both diagnostic and surgicaltechniques, often indicate a need for restudy . Thisconcept was exemplified by two patients in this series,one of whom had had early palliative surgery, who hadno cyanosis at rest and were not limited in daily activi-ties .

Physical examination in adult tricuspid atresiatypically reveals left ventricular enlargement, althoughit was absent in one third of the patients in this series .Systolic murmurs are common . Additional abnormali-ties related to previous surgical shunts include widenedpulse pressure, further left ventricular enlargement andcontinuous murmurs over the involved area.

The electrocardiogram in adults with tricuspidatresia, as exemplified in this series, characteristicallydisplays left axis deviation, left ventricular hypertrophyand nonspecific abnormalities in T wave contour .'°-1aNormal or right axis deviation in tricuspid atresia hasbeen described when there is coexisting transpositionof the great arteries . 12,14-1 s Accordingly, the two personsmanifesting right axis deviation had type III tricuspid

TRICUSPID ATRESIA IN AOULTS-PATTERSON ET AL .

atresia, one in association with dextrocardia . The plainchest X- ray film typically shows a normal cardiac size.Pulmonary vascularity is usually diminished, but thesefeatures are modified by palliative shunt procedureswhich will augment pulmonary vascularity and leftventricular size .

Characteristic features of tricuspid atresia definedby echocardiography, particularly the sector scan type,include: absence of a tricuspid valve echo, small anteriorright ventricular chamber, posterior mitral valve echowith large diastolic anterior excursion and mitral-semilunar valve continuity . 17 Echocardiograms per-formed in three patients in this series demonstratedthese findings . However, no systolic prolapse of the

FIGURE 8 . Glenn anastomosis . Left innominate vein (LIV) angiogram(same case as Figure 7, after reoperation) . The collateral veins seenin Figure 7 have been ligated to improve right lung perfusion. Abbre-viations as in Figure 7 .

January 1982 The American Journal of Cardiology Volume 49 147


FIGURE 9 . Fontan anastomosis . Right atriogram (A) and angiogram ofthe outlet chamber (B) . The morphologic right atrium (RA) has beenconnected to the pulmonary artery (PA) . The anastomosis was alsoconnected to the outlet chamber (OCH) (rudimentary right ventricle) .The pulmonary artery is well demonstrated without regurgitation intothe vans cava . L = left pulmonary artery; R = right pulmonary ar-tery .

mitral leaflets was seen, as has occasionally been ob-served elsewhere. 14

AngiocardiographyAll patients included in this series had cardiac cath-

eterization and angiographic studies at the time ofpresentation as adults . High quality angiography,preferably biplane, is the most important mode ofprecise diagnosis and classification. Desirable angio-graphic techniques include the use of large bore cathe-ters, angled views8. 9 and, occasionally, large film se-quences. Figures 1 to 5 show representative angiocar-diogramsin tricuspid atresia .Methodology: At this institution, we prefer use of a

size 10 French or larger National Institutes of Healthcatheter (side holes only) . Such a catheter can best beplaced in the heart through an open saphenous venot-omy, and can be manipulated if necessary with a tip-deflecting guidewire. Passage across the atrial defectpermits study of the left heart chambers . This largecatheter allows high contrast angiography to be per-formed safely in most patients . Typically, 60 to 80 mlof contrast material is injected in the left ventricle over2 to 4 seconds. Injections are made in the right atriumand elsewhere as necessary to define the patency ofpreviously created surgical shunts or to diagnose othercoexistent congenital or acquired defects . As in otherforms of complex congenital heart disease, two separatecatheterization studies several days apart may be re-quired. Such an approach protects the patient fromexcessive doses of contrast material and from a pro-longed procedure. The first study is planned aftercareful evaluation of all noninvasive data and permitsgross anatomic diagnosis . The second study followssurgical consultation and clarifies any remaining ana-tomic or physiologic details including pulmonary vas-

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cular resistance . At this institution, we oftenprefer thecatheterization study to be performed under generalanesthesia in patients with severe cyanotic disease, withattendance by an anesthesiologist specializing in cardiacdisease. Such a practice, complete with tracheal intu-bation, mechanical ventilation and urinary catheter-ization, enhances patient safety and comfort, cardio-vascular stability, fluid balance, and postcatheterizationrecovery.Angiographic anatomy: The angiographic anatomy

of tricuspid atresia in the setting of a concordant A-Vrelation with situs solitus of the atria and levocardia hasthe following features as revealed in the long axial view(Fig. 2A) . This projection is 60 ° left anterior obliquewith 30° cranial angulation, and facilitates correct an-atomic diagnosis. The left ventricle is seen as a largechamber located posteriorly and to the left . The tra-becular septum is seen anteriorly and to the left . Theinfundibular septum, which separates the subaorticfrom subpulmonary outflow tracts, is usually on linewith the trabecular portion . The ventricular septal de-fect (outlet foramen) is seen in the junction betweenthese two portions of the septum . The small right ven-tricle, usually anterior, has uniform contractions whichinclude the entire wall . The outflow tract of this smallchamber supports the pulmonary valve . The pulmonaryartery is located anterior and to the left of the aorta . Theleft ventricle is usually enlarged with an enlarged mitralvalve, supporting the aorta . (When transposition of thegreat arteries is present, the aorta is located anterior tothe pulmonary artery; the pulmonary artery arises fromthe left ventricle .) The mitral valve is located posteriorlyunderneath the aortic valve . The anterior leaflet is inrelation with the left and noncoronary aortic cusp . Thisview shows that the ventricular septum in its entireaspect is important for the detection of additionalventricular septal defects .

A further special view is the elongated right anterioroblique view (Fig . 2B). This projection includes caudalangulation to bring the long ventricular axis into profile .The left ventricle is located to the left of the right ven-tricle . The aortic valve is seen above the mitral valve .The posterior contour is obscured by the opacified rightventricle. The ascending aorta is seen to the right of thepulmonary artery . The small right ventricle is locatedto the right and inferiorly, connected to the left ventriclethrough a ventricular septal defect. The pulmonaryartery and its branches are well depicted in this view,allowing the identification of peripheral pulmonarystenosis.

Associated congenital cardiac anomalies were un-common in this series, as noted elsewhere.la,n-2s Ofparticular interest were two patients with type IA, eachwith adequate pulmonary blood flow through a patentductus arteriosus so that a palliative procedure was notcarried out until age 2 years, as described in their pre-vious hospital records .

Anatomic ClassificationIn this group of adult patients, as in pediatric se-

ries,4s,1o,14,19,26-28 the most frequently observed ana-

tomic type was IB, with normally related great arteriesand pulmonary stenosis. Less common were type IA(pulmonary atresia) and types II and III with transpo-sition complexes. One patient (Case 11) did not fit intothe standard classification . She had situs solitus of theatria with unusual connections and relations of theventricles. The right atrium was connected with theright-sided left ventricle through a initial valve; thesmall right ventricle was left-sided in potential con-nection with the left atrium, but the tricuspid valve wasabsent. However, because the great arteries were inconcordant connection, this was not "corrected trans-position ." The aorta originated from the left ventricleand the pulmonary artery from the right ventricle,which was left-sided in potential connection with theleft atrium, but the tricuspid valve was absent . Thetricuspid atresia in this patient was not type f, IT orIll .

Therefore, it appears that tricuspid atresia mightbe better classified by its sequential chamber local-ization (Fig . 6) . This new classification is based on de-scription of atrioventricular and ventricular-great vesselrelations, and can further be defined by describing atrialsitus. It constitutes a modification and expansion of theoriginal classification by Keith et al . 10 Such a proposedclassification includes all potential anatomic types oftricuspid atresia, both those described herein or earlieras well as those not yet reported . Thus, the most com-mon type in this series is described as situs solitus, withconcordant A-V and concordant ventricular-great ar-terial relations . An example of a type of tricuspid atresianot present in this series, or not reported yet to ourknowledge, would be situs inversus of the atria withdouble outlet right ventricle. This new classificationtherefore permits more concise and complete anatomicdescription of this disease .

Surgical Considerations

Diminished pulmonary blood flow: In contrast tosome other forms of complex cyanotic congenital heartdisease in the adult, tricuspid atresia cannot be cor-rected in the usual anatomic and physiologic sense be-cause of the prohibitively small size of the right ven-tricle, absent tricuspid ring and associated structures .However, a variety of surgical procedures have beenincreasingly beneficial in reducing cyanosis and en-hancing effort tolerance and survival. 27,29-35 A primarysurgical consideration in tricuspid atresia is that thehemodynamic defect responsible for disability anddeath is usually low effective pulmonary blood flow.Physiologically as well as anatomically, tricuspid atresiamay be likened to single ventricle3 ; that is, there are twopotential paths of exit of mixed oxygenated and unox-ygenated blood from the heart ; the aorta and the pul-monary artery . The ratio of flows to each route dependslargely on downstream resistance. Because pulmonarystenosis, an underdeveloped right ventricle and smallpulmonary arteries are the common features of tricuspidatresia, resistance to flow in this direction is usuallyhigh, causing inadequate pulmonary blood flow and


cyanosis . Thus, the major clinical symptoms in tricuspidatresia stern from cyanosis and pot ycythemia, with fa-tigue and weakness predominant,'L ' .i 1,36,3'

Other features that may further decrease pulmonaryblood flow include progressive pulmonary stenosis orclosure of the ventricular septal defect .38A restrictiveventricular septal defect may progressively close, evenin adulthood . 39 Three patients in this series had a re-strictive ventricular septal defect . Pulmonary vascularresistance may progress to impede pulmonary flow,particularly in patients who have had a surgicallycreated systemic to pulmonary arterial shunt, whichinitially results in high pulmonary flow . No patient inthe series had excessive pulmonary vascular resistance,implying that this complication usually proves lethalbefore adulthood. Other shunt-related complicationsthat may diminish pulmonary blood flow are sponta-neous closure; kinking, distortion or stenosis of thepulmonary artery developing at the site of anastomosis ;and the development of collateral channels around aGlenn superior vena cava to pulmonary arterial anas-tomosis, which may occur in any of these settings as itdid in two patients in this series .Excessive pulmonary blood flow: In contrast to

decreased pulmonary blood flow, excessive flow mayoccasionally occur in tricuspid atresia. This may resultnaturally, in the absence of pulmonary stenosis or arestrictive ventricular septal defect (anatomic subtypeC rather than A or B, according to the classification ofKeith et al . 10 ) . However, none of the patients in ouradult series had this form of defect. More commonly,excessive pulmonary blood flow may occur after an in-ordinately large surgically created systemic to pulmo-nary arterial shunt . Complications of high pulmonaryblood flow include heart failure and the potential de-velopment of increased pulmonary vascular resistance .Decreased ventricular function, present in three of thefour oldest patients in our series, may occur with age aspart of the natural history of this disease . 36 Increasedleft ventricular size, resulting from this surgically in-duced volume overload with or without ventriculardysfunction, is often reflected by cardiomegaly onphysical examination and chest X-ray study, and leftventricular hypertrophy on electrocardiography . Thus,heart failure rather than cyanosis often proves theultimate clinical problem in these postoperative pa-tients .

Great arterial relations : Additional factors thatmay alter the effective pulmonary blood flow in tri-cuspid atresia are the great arterial relations . In ven-triculoarterial discordance (transposed great arteries),aortic flow rather than pulmonary flow may be impededby a restrictive septal defect and diminutive right ven-tricle and semilunar valve . This may be a lethal occur-rence in childhood and was not observed in our series .Other variations not observed here include double-outlet ventricle or single outlet (truncus arteriosus) .Finally, the degree of mixing of unoxygenated and ox-ygenated blood within the heart may not be complete ;the streaming effect may result in selectively high or lowoxygenation levels in blood flowing to the lungs .


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Surgical OptionsIt is apparent from review of these hemodynamic

factors that longevity in tricuspid atresia depends on:the adequacy of pulmonary blood flow, the absence ofhigh pulmonary vascular resistance, the degree ofmixing of unoxygenated and oxygenated blood in theleft ventricle, and the absence of associated congenitalor acquired abnormalities. The chance occurrence of anoptimal balance of these factors may result in thesepersons presenting to the physician in adult life for thefirst time. Six of the 18 patients in this series, aged 18to 38 years, so presented to this institution withoutprevious surgery . Thus, it is important for the adultcardiologist to be familiar with the surgical options andsurvival possibilities in this disorder, as well as to realizethe importance of detailed, precise diagnosis . Thesesurgical options are designed to improve the abnormalhemodynamics described herein and include the fol-lowing :

1 . Aorta to pulmonary arterial anastomoses :These were originally developed to provide palliationin tetralogy of Fallot but can be utilized in other con-ditions with inadequate pulmonary blood flow . 33 Thepresence of a true pulmonary artery is a prerequisite,although it may be small or underdeveloped . Theseprocedures include the original Blalock-Taussig sub-clavian artery to pulmonary arterial (end to side)anastomosis, the Potts descending aorta to left pul-monary arterial (side to side) anastomosis, the Water-ston ascending aorta to right pulmonary arterial (sideto side) anatomosis, and construction of other anasto-moses with vein, Gore-Tex or other material . Thephysical examination, chest X-ray film, electrocardio-gram and data from other studies may be altered bythese shunts. Thus, features similar to patent ductusarteriosus with left ventricular enlargement may appear .Complications of these procedures include the devel-opment of pulmonary vascular disease associated withpulmonary overcirculation and heart failure from leftventricular volume overload if the shunt is large . 36These complications occur most commonly with thePotts and Waterston type procedures . The ipsilateralupper limb may suffer from ischemia if the Blalock-Taussig procedure is performed in an adult . Sponta-neous closure of the Blalock-Taussig anastomosis mayoccur, or the surgery may cause kinking or distortion ofthe pulmonary artery . However, 15 of these types ofprocedures had previously been performed in 12 pa-tients in this series . After evaluation here as adults, fourpatients received such a shunt. As shown by the survivaldata, these procedures can be considered generallysuccessful .

2. The Glenn procedure: This is the superior venacava to right pulmonary arterial (end to end) anasto-mosis . 32-34 It has the advantage of more effective pul-monary flow to the right lung (unmixed, unoxygenatedblood) . Furthermore, this procedure does not cause avolume load on the left ventricle . 36 Low pulmonaryvascular resistance is a prerequisite. The superior venacava must be identified angiographically before oper-ation, since it is occasionally located on the left side . 28

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The cardiologist should be aware of the possibility oflate development of collateral venous flow around theanastomosis by way of the azygous or other systems 40

Such collateral vessels defeat the purpose of the anas-tomosis but can be ligated by further surgery . The col-lateral vessels can be demonstrated angiographically bysubclavian venography (Fig . 7) . Figure 8 reveals a wellfunctioning Glenn shunt after ligation of the collateralvessels . Five patients in this series had received a Glennprocedure before evaluation here as adults; no furtherGlenn procedures were done in the patients in this seriesafter evaluation here .

3. Pulmonary arterial banding: This proceduremay be required in patients with excessive pulmonaryflow to prevent heart failure from volume overload . 11It was not performed in our series, but is more com-monly utilized in children. Banding may be combinedwith atrial septectomy if the atrial septal defect is in-adequate for venous transport into the left ven-tricle . 32

4. The Fontan procedure: This procedure has un-dergone many modifications but basically involvesredirecting venous flow from both venae cavae or theright atrium directly to the hypoplastic right ventricle,outflow chamber or pulmonary arteries through avalved or nonvalved conduit29,30,35 The atrial septaldefect is closed as is the outflow foramen (ventricularseptal defect) . The pulmonary valve may or may notremain functional. Thus, this procedure theoreticallyoffers more physiologic correction in terms of effectivepulmonary flow with creation of the normal seriesrather than parallel pulmonary and systemic flow cir-cuits. It is mandatory that such patients have low pul-monary vascular resistance, because right atrial pressureis the perfusion pressure for pulmonary flow. Becauseof the spatial arrangements of the cardiac chambers, theFontan procedure cannot be performed in patients withboth A-V and ventricular-great vessel discordance (typeIII, "corrected transposition of the great vessels") . Also,the development of atrial arrhythmias, particularlyatrial fibrillation, may be poorly tolerated ; however, thereported results of this procedure have generally beengood. 35 Long-term results of the Fontan procedure havebeen less good in patients aged 15 to 36 years than inyounger patients.41 Only two patients in this series hada Fontan procedure ; one died early after operation andthe other has an excellent late result. Figure 9 is an an-giographic depiction of a Fontan anastomosis .

Criteria for subsequent surgical procedure: Ineach case in this series, the decision for or against ad-ditional operation as an adult was based on severalfactors. These included the severity of symptoms andthe patient's ability to tolerate them, the anatomic po-tential for further correction, and the estimated surgicalrisk. Eleven of the 18 patients studied did not meetcriteria for subsequent operation . At present in childrenolder than 5 years and in adults in whom surgery is ad-visable, the Fontan procedure is preferred at this in-stitution. We choose this if pulmonary vascular resis-tance is low, and if the pulmonary valve anulus andpulmonary arteries are of satisfactory diameter as

measured angiographically or at operation .42 If thesecriteria are lacking, a Blalock-Taussig shunt is per-formed. Potts and Waterston anastomoses are notrecommended .

Prognosis

The overall survival data of this series of adult pa-tients offer a somewhat optimistic note, particularly inrelation to earlier studies that have primarily includedchildren. Thus, there were only two deaths in the 18patients with an average follow-up period of more than4 years. Of the seven patients having surgery after study,one died early after operation and the others survive upto 9 years. Of the 11 patients who did not have furthersurgery, one died late and the others survived up to over6 years. Four patients had no surgery either before orafter study here and had relatively good hemodynamicstatus and survival at latest contact at ages 21 to 41years. Previously published survival data are primarilypediatric and include those of Dick et al.4 In 1975 theydescribed an overall survival rate to age 15 years of only

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12. Davachi F, Lucas RV Jr, Moller JH . The electrocardiogram andvectorcardlogram in tricuspid atresia . Correlation with pathologicanatomy. Am J Cardiol 1970 ;15:18-27 .

13. Schatz J, Krongrad E, Maim JR . Left anterior and left posteriorhemiblock in tricuspid atresia and transposition of the great vessels .Circulation 1976 ;54:1010-3 .

14. Edwards JE, Bruchell HB . Congenital tricuspid atresia : a classi-fication . Med Clin North Am 1949 ;33:1177-96 .

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16. Ruttenburg HP, Elliott LP, Anderson RC, Adams P, Tuna P . Con-genital corrected transposition of the great vessels . Correlationof electrocardiograms and vectorcardiograms with associatedcardiac malformations and hemodynamic states . Am J Cardiol1976:17:339-54 .

17 . Seward JB, Talik AJ, Hagler DJ, Ritter DG. Echocardlographic

References

TRICUSPID ATRESIA IN ADULTS- PATTERSON ET AL .

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Acknowledgment

We thank Theresa Carreker for typing the manuscript andJames Craig for assistance with the illustrations .

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tricuspid atresia in adults

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