total correction of tetralogy of fallot in adults:

10
Statistical Analysis Total Correction of Tetralogy of Fallot in Adults: Surgical Results and Long-term Follow-up In-Sook Park, M.D., Robert D. Leachman, M.D., and Denton A. Cooley, M.D. From 1962 to 1983, 117 patients between the ages of 16 and 72 years (mean, 25 years) underwent total correction of tetralogy of Fallot. All patients were operated on because of recent deterioration. Eighty (68%) had had previous surgical palliation. Complications occurred in 38 cases (32%) before total correction and were more frequent in older patients. The hospital mortality was 7.8% and was highest in older patients and those with a previous Potts shunt. Fifty-nine patients were followed up for as long as 23 years (mean, 9.2 years) after total correction. The probability of survival at 5, 10, and 15 years after total correction was 96%, 96%, and 84%, respectively. The actuarial freedom from any significant cardiovascular event at 5, 10, and 15 years was 84%, 73%, and 50%, respectively. Reoperation was required in ten cases (17%), in which there was one operative death. Late death occurred in four cases (6.8%). Left ventricular dysfunction was documented in ten patients (17%), three of whom had mural thrombi and two of whom had cerebrovascular acci- dents. A significant rhythm disorder was present in 13 patients. Fifty-four of the 55 surviving patients were in New York Heart Association (NYHA) functional class I orIl at lastfollow-up. We conclude that 1) total correction of tetralogy of Fallot in adults is associated with a low operative mortality, similar to that in children, and offers symptomatic relief in most cases; 2) the long-term survival is excellent, but late sequelae become more frequent at late follow-up; 3) "corrective" reoperation can be done with a low risk; and 4) most of the survivors are in NYHA functional class I or II and lead normal, productive lives. (Texas Heart Institute Journal 1987; 14:160-169) Key words: Tetralogy of Fallot; heart defects, congenital; heart surgery T OTAL CORRECTION of tetralogy of with tetralogy of Fallot survive to adulthood . Fallot has been practiced for more than without a corrective operation, however, and three decades.' In recent years, most cardiolo- this group presents a unique and challenging gists have recommended total correction at problem for cardiologists. an early age, when anatomically feasible, Several studies2-4 have described the opera- rather than palliation. A number of patients tive results and long-term follow-up of patients From the Department of Cardiology, Texas Heart Institute of St. Luke's Episcopal Hospital, Houston, Texas. 160 Tetralogy of Falot in Adults: Surgery and Folow-up Address for reprints: In-Sook Park, M.D., P.O. Box 20206, Houston, TX 77225. Vol. 14, No. 2, June, 1987

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Statistical Analysis

Total Correction of Tetralogy of Fallot in Adults:Surgical Results and Long-term Follow-up

In-Sook Park, M.D., Robert D. Leachman, M.D.,and Denton A. Cooley, M.D.

From 1962 to 1983, 117 patients between the ages of 16 and 72 years(mean, 25 years) underwent total correction of tetralogy of Fallot. Allpatients were operated on because of recent deterioration. Eighty (68%)had had previous surgical palliation. Complications occurred in 38 cases(32%) before total correction and were more frequent in older patients.The hospital mortality was 7.8% and was highest in older patients andthose with a previous Potts shunt. Fifty-nine patients werefollowed upforas long as 23 years (mean, 9.2 years) after total correction. Theprobabilityof survival at 5, 10, and 15 years after total correction was 96%, 96%,and 84%, respectively. The actuarial freedom from any significantcardiovascular event at 5, 10, and 15 years was 84%, 73%, and 50%,respectively. Reoperation was required in ten cases (17%), in which therewas one operative death. Late death occurred in four cases (6.8%). Leftventricular dysfunction was documented in ten patients (17%), three ofwhom had mural thrombi and two of whom had cerebrovascular acci-dents. A significant rhythm disorder was present in 13 patients. Fifty-fourof the 55 surviving patients were in New York Heart Association (NYHA)functional class I orIl at lastfollow-up. We conclude that 1) total correctionoftetralogy ofFallot in adults is associated with a low operative mortality,similar to that in children, and offers symptomatic relief in most cases;2) the long-term survival is excellent, but late sequelae become morefrequent at late follow-up; 3) "corrective" reoperation can be done witha low risk; and 4) most of the survivors are in NYHAfunctional class I orII and lead normal, productive lives. (Texas Heart Institute Journal1987; 14:160-169)

Key words: Tetralogy ofFallot; heart defects, congenital; heart surgery

T OTAL CORRECTION of tetralogy of with tetralogy of Fallot survive to adulthood. Fallot has been practiced for more than without a corrective operation, however, and

three decades.' In recent years, most cardiolo- this group presents a unique and challenginggists have recommended total correction at problem for cardiologists.an early age, when anatomically feasible, Several studies2-4 have described the opera-rather than palliation. A number of patients tive results and long-term follow-up of patients

From the Department of Cardiology, Texas Heart Institute of St. Luke's Episcopal Hospital,Houston, Texas.

160 Tetralogy of Falot in Adults: Surgery and Folow-up

Addressfor reprints: In-Sook Park, M.D., P.O. Box 20206, Houston, TX 77225.

Vol. 14, No. 2, June, 1987

TABLE 1. Patient Groups (n = 117)

GroupI 11 III IV

Numberof Patients 70 30 17 117Sex:Male 35 16 12 63Female 35 14 5 54

Age range (y) 16-25 26-35 36-72 16-72Mean age (y) 19.5 29.5 42.0 25.0

with a wide range of ages who have undergonetotal correction of tetralogy of Fallot. To ourknowledge, however, there has been no sys-tematic study comparing the surgical mortalityand morbidity and long-term follow-up aftertotal correction in different age groups. There-fore, this study was designed to assess the earlyoperative results and long-term follow-up ofadults who underwent total correction of tetral-ogy of Fallot at our institution.

PATIENTS AND METHODS

Between 1962 and 1983, 117 patients 16 yearsof age or older underwent total correction oftetralogy of Fallot at the Texas Heart Institute.All were under the care of one or more of theauthors. Only patients with an anatomy typicalof tetralogy of Fallot were included in the study.Those with congenital or acquired pulmonaryatresia, mitral stenosis, restrictive ventricularseptal defect, and absent or abnormal origin ofa pulmonary artery were excluded.

Because it is generally believed that oldertetralogy of Fallot patients have a higher surgicalrisk than younger ones, the patients weredivided into three groups according to theirage at the time of total correction. Group Iincluded 70 patients between 16 and 25 yearsof age; group II comprised 30 patients between26 and 35 years of age; and group III consistedof 17 patients 36 years old or over at the timeof total correction (Table I). The patients'medical records were reviewed, and question-naires were sent to those whose last follow-upwas more than 1 year prior to the end of thestudy period. Eighty patients (68%) had under-gone previous surgical palliation between 6months and 23 years of age (mean, 8.4 years),and total correction was done 3 to 28 years(mean, 17 years) after palliation. Creation of a

Blalock-Taussig shunt was the most frequentpalliative procedure, with creation of a Pottsshunt the second most common (Table 11).

RESULTS

Preoperative StatusMost of the patients were symptomatic

before operation and were in New York HeartAssociation (NYHA) functional class II or Ill.Three (2.5%) were in functional class I, 55(47.0%) were in class II, 57 (49.0%) were inclass III, and two (1.5%) were in class IV. Allwere operated on because of recent worseningof symptoms or onset of complications relatedto tetralogy of Fallot.

Preoperative EventsIn 38 cases (32%), significant medical prob-

lems occurred before total correction; these weremore frequent in group HI (59%) than in groupI (29%) or 11 (27%). Cerebrovascular accident(ten cases) and syncope (ten cases) were themost frequent preoperative complications (TableHI). Congestive heart failure occurred in eightpatients, three of whom also had bacterialendocarditis. These three required urgent correc-tive surgery because of rapid hemodynamicdeterioration. Moderate-to-severe pulmonaryhypertension (pulmonary artery systolic pres-sure greater than half of the systemic pressure)was present in five patients, all of whom hadundergone creation of a previous Potts shunt.The interval between creation of the Pbtts shuntand total correction was slightly, but (from astatistical standpoint) not significantly, longer inthe five patients with pulmonary hypertension(mean, 18.4 years; range, 13 to 28 years) thanin the 14 patients with normal pulmonarypressure (mean, 16.1 years; range, 11 to 25

Tetralogy of Faflot in Adults: Surgery and FoUow-up 161lexas Heart Institute Journal

TABLE II. Previous, Palliative Procedures

Number ofProcedure Patients

One Blalock-Taussig shunt 49Potts shunt 16Two Blalock-Taussig shunts 4Blalock-Taussig and Potts shunts 3Waterston-Cooley shunt 1Other types of shunt 2Pulmonary valvotomy 4Exploratory thoracotomy only 1

TOTAL 80

TABLE Ill. Preoperative Complications*

Number ofComplication Patients %

Cerebrovascular accident 10 8.5Syncope or near syncope 10 8.5Congestive heart failure 8 6.8Pulmonary hypertension 5 4.2Bacterial endocarditis 4 3.4Symptomatic rhythm disorder 4 3.4Brain abscess 3 2.6Hemoptysis 3 2.6Symptomatic gout 2 1.8Aneurysmal dilatation of pulmonary

artery with Potts shunt 2 1.8Aortic insufficiency 1 0.9

TOTAL 52

*Some patients had more than one preoperative complication.

years). Four patients had a symptomatic rhythmdisorder, and three had a brain abscess. Signifi-cant hemoptysis occurred in three patients, twoof whom had a previous Potts shunt with pul-monary hypertension and one of whom had aBlalock-Taussig shunt with normal pulmonarypressure. Symptomatic gout was present in twocases, both from group III. Two patients hadaneurysmal dilatation of the left pulmonaryartery associated with a Potts shunt, one withnormal and the other with elevated pulmonary

pressure. Aortic regurgitation was observed inone patient.

Hospital Morbidity and MortalityNine patients died within 30 days after total

correction (hospital mortality, 7.8%). The causeof hospital death was bleeding in three cases,congestive heart failure with low cardiac outputin two cases, malignant ventricular dysrhyth-mias in two cases, adult respiratory distresssyndrome with disseminated intravascular

162 Tetralogy of Fallot in Adults: Surgery and Follow-up Vol. 14, No. 2, June, 1987

TABLE IV. Outcome in Patients with a Potts Shunt

Died at operation (1) Bleeding fromPotts shunt

Normal PAP(14) Survived operation (13)

Patients with a Died at operation (2)* Bleeding,Potts shunt (19) ventricular

fibrillation

PH (5)

Survived (3)** No follow-up (1)-Follow-up cath (2)

Both with normal PAP

Cath = cardiac catheterization; PAP = pulmonary artery pressure; PH = pulmonary hypertension.*These two patients had pulmonary hypertension owing to elevated pulmonary vascular resistance duringpreoperative cardiac catheterization.**These three patients had pulmonary hypertension owing to high pulmonary blood flow during pre-operative cardiac catheterization.

coagulopathy in one case, and perioperativecerebrovascular accident in one case. Thehospital mortality was examined with regard toage at the time of total correction, type ofprevious shunt operation, and preoperativehemoglobin level. The mortality was higher ingroup HI (two deaths, 13%) than in group I (fivedeaths, 7%) or H (two deaths, 7%). It was alsohigher in patients with a Potts shunt (threedeaths, 18.8%) than in those with a Blalock-Taussig shunt (three deaths, 5.6%) or without aprevious shunt (two deaths, 5.4%). Of the threepatients with a Pbtts shunt who died after totalcorrection, two had pulmonary hypertensionwith elevated pulmonary vascular resistance,and one had a normal pulmonary artery pressure(Table IV). The preoperative hemoglobin levelwas similar for survivors (mean, 18.4 gm/dl)and nonsurvivors (mean, 18.7 gm/dl). Amongthe survivors, immediate postoperative compli-cations included excessive bleeding requiring anexploratory thoracotomy for hemostasis in 13cases (12.0%), a sternal wound infection in threecases (2.8%), complete heart block requiring

implantation of a permanent pacemaker in twocases (1.8%), a urinary tract infection in twocases (1.8%), supraventricular tachycardia intwo cases (1.8%), and a pulmonary embolism inone case (0.9%).

Follow-up DataFollow-up data were available for 59 patients.

Most of the patients for whom follow-up datawere not available were from outside the UnitedStates. The duration of follow-up ranged from 6months to 23 years (mean, 9.2 years) after totalcorrection. Significant postoperative events arelisted in Table V. Of the 59 patients with follow-up information, 39 (66%) were free of latedeath, reoperation, or significant symptoms.Among the 27 patients with a follow-up longerthan 10 years, only 14 (52%) were free of theseevents. The probability of survival at 5, 10, and15 years after total correction was 96%, 96%,and 84%, respectively. The actuarial freedomfrom significant complicating cardiovascularevents at 5, 10, and 15 years was 84%, 73%, and50%, respectively (Fig. 1).

Tetralogy of Falot in Adults: Surgery and FoUow-up 163Texas Heart Institute Journal

TABLE V. Postoperative Events*

Number ofEvent Patients %

Reoperation 10 17.0Late death 4 6.8Left ventricular dysfunction 10 17.0Significant rhythm disorder 13 22.0Cerebrovascular accident 4 6.8Aortic insufficiency 2 3.4Pregnancy 9**

*Some patients had more than one postoperative event.**Seventeen pregnancies occurred in nine patients.

Postoperative Cardiac CatheterizationPostoperative cardiac catheterization was

performed in 23 "selected" patients who hadmajor cardiovascular symptoms or who werethought to have abnormal hemodynamic resultson the basis of noninvasive studies (Fig. 2).According to the hemodynamic criteria pub-lished by Garson and associates, 2,5 for classify-ing the results of total correction, eight patients(35%) had an excellent result (right ventricularpressure <40 mm Hg and no residual left-to-right shunt or a small left-to-right shunt with aQp:Qs ratio <1.3:1.0), five patients (22%) hada good result (right ventricular pressure between40 and 59 mm Hg and/or Qp:Qs ratio < 1.5:1.0),and ten patients (43%) had a poor result (rightventricular pressure .60 mm Hg and/or Qp:Qsratio . 1.5:1.0 or pulmonary hypertension).Of the 13 patients who met the hemodynamic

criteria for a good or an excellent result, how-ever, seven (54%) had a significant postop-erative cardiac abnormality including severeventricular dysfunction (six cases) and leftventricular outflow obstruction owing to asubaortic membrane (one case). These sevenpatients are represented by the cross-hatchedarea in Figure 2. In patients with poor results(except for one patient with pulmonary hyper-tension), residual abnormalities could be cor-rected by means of reoperation. Three patientshad severe pulmonary hypertension (which wasthought to be secondary to a large Potts shuntpreoperatively) and survived total correction.Two of these had postoperative hemodynamic

studies, which showed a dramatic reduction inpulmonnary artery pressure. In both cases, thepulmonary artery pressure gradually decreasedover the ensuing 13 postoperative years until itbecame completely normal.

ReoperationTen patients required reoperation 1 month

to 19 years after initial total correction. Theindications and procedures were as follows:closure of a residual atrial septal defect and aprevious Blalock-Taussig shunt (two cases),closure of a residual ventricular septal defect(two cases), closure of a persistent systemic-to-pulmonary artery shunt and azygos veingrafting (one case), resection of residual pulmo-nary stenosis (one case), repair of a ventricularseptal defect and pulmonary stenosis (one case),repair of a residual ventricular septal defect andmitral regurgitation (one case), closure of apersistent Potts shunt (one case), and resectionof a subaortic membrane (one case). One patientdied at the time of reoperation (operative mortal-ity, 10%). Of the nine who survived reoperation,seven showed marked hemodynamic improve-ment. One patient required two more operationsin order to close a persistent ventricular septaldefect after the initial total correction of tetral-ogy of Fallot. One patient, whose azygos veingraft remained patent despite reoperation, died14 years later (at the age of 34 years) of severeventricular dysfunction and congestive heartfailure, which had been present before the initialtotal correction.

164 Tetralogy of Fallot in Adults: Surgery and Follow-up Vol. 14, No. 2, June, 1987

Probability of Survival and Freedomfrom any Event in Hospital Survivors

Survival(59)

'15)

(9) (5)

10 12 14 16 18 20

Years after Operation

Flg. 1 Probability of survival and freedom from major cardiovascular events among hospital survivors. Thenumbers in parentheses denote the number ofpatients followed up to the corresponding year after total correction.

Late DeathLate death occurred in four cases (6.8%). One

patient died of ventricular dysfunction andcongestive heart failure 14 years after totalcorrection. A second patient died at 22 years ofage (2 years after total correction), at the time ofreoperation to close a persistent Blalock-Taussigshunt and an atrial septal defect. A third patientdied at 31 years of age (15 years after totalcorrection), of multiple problems including leftventricular dysfunction with mural thrombus,severe systemic hypertension, and ventriculardysrhythmia. Afourth patient died at 72 years ofage (3 months after total correction). This patienthad led a fairly normal life without palliation butwas advised to have total correction at age 72years because ofrecent clinical deterioration. Heimproved somewhat after the operation but died3 months later of renal failure and congestiveheart failure, probably secondary to long-standing systemic hypertension and hypoxemia.

Left Ventricular DysfunctionLeft ventricular dysfunction was documented

in ten cases (nine by means of ventricular angiog-

raphy and one by means of a nuclear study), 2 to23 years after total correction. The mean age atthe time of total correction in this subset was 27years (range, 16 to 44 years); this was similar tothe mean age of the entire group. Four patientshad had previous palliative surgery. Nine were

evaluated by means of postoperative cardiaccatheterization: five of these were shown to havea good-to-excellent result, and four had a poor

result. One patient was considered to have a

good result on the basis of noninvasive studies.Left ventricular mural thrombi were seen on two-dimensional echocardiography and/or angiog-raphy in three patients, two ofwhom subsequentlyhad a cerebrovascular accident.

Significant Rhythm DisorderThirteen patients (22%) had significant rhythm

disorder during the follow-up period. These were

subjective complaints such as palpitations,frequent irregular beats, or supraventricular or

ventricular tachycardia as documented by a Holtermonitor. Age at total correction (mean, 25.5 years;range, 16 to 42 years) was similar to that of the

entire group. Of these 13 patients, eight under-

Tetralogy of Fallot in Adults: Surgery and FoUow-up 165

cJC)a)a.

Texas Heart Institute Journal

Postoperative Catheterizationin Selected Group of Patients

(N = 23)

ExcellentResultsN = 8(35%)

RVP < 40 mm HcQp:Qs< 1.3:1.0

N = 7Good-ExcelleTOF Repair

LV DysfunctionSub Ao Membrar

oo-o-o--ooN ~~..............R

~ ~~~~~~~~~~~~~~~~~~~~~ 0

xx ::: :::::::::::::::::::::::

x\\\xXxXxx\xXxXxxr ......................

_ x .........................xxNNNNNNNx x x x x; x

\xX\\\\\\ x x x x x x x x x x *\x - - o o. ooo o o

(6 xx xx _ x x x x x x, ./\\\ xx

x xxx x x x-XxMAx xxxxxx x\ .....................................i&woeNY Y x Y x x x xx .M...................................

vxX> x xx xx x x x x x x x x x x xnt~~~~~~ XN; M x x x x >s4xxx* - -

6 \ x xx x x xx x x x x x x x x

Pooresults 9 Reop=10 1.51 PH

(43%)WVP - 60 mm Hglp:Qs - 1.5:1.0 PH

GoodResultsN = 5(22%)

VP 40-59 mm HgQp:Qs < 1.5:1.0

Fig. 2 Results of postoperative cardiac catheterization in a "selected" group of 23 patients. The cross-hatched areais not a fourth subgroup but represents seven patients who had either good or excellent results after total correction,but who were found to have left ventricular dysfunction or subaortic membrane.LV = left ventricular; PH = pulmonary hypertension; Qp:Qs = ratio of pulmonary to systemic blood flow; reop= reoperations; RVP = right ventricular pressure; Sub Ao = subaortic; TOF = tetralogy of Fallot.

went postoperative cardiac catheterization, andonly one was shown to have an excellent result.The remaining seven had poor hemodynamicresults and/or ventricular dysfunction.

Cerebrovascular AccidentCerebrovascular accidents occurred in four

cases, 10 to 18 years after total correction. Allof these patients were known to have postoperativeleft ventricular dysfunction, and two had muralthrombi on ventricular angiography. The cerebro-vascular accidents were most likely due to sys-temic embolization from ventricular thrombi. Twoof these patients also had other symptoms thatsuggested systemic embolization.

Aortic Insuffi'ciencyOne patient with known preoperative aor-

tic insufficiency continued to have evidence ofmild aortic insufficiency after total correction.

Another patient developed mild aortic insuffi-ciency postoperatively and was also found tohave a residual ventricular septal defect 2 yearsafter total correction.

PregnancyDuring the postoperative period, 17 preg-

nancies occurred in nine patients, with thefollowing outcome: twelve normal infants, onepremature birth, three spontaneous abortions,and one infant with tetralogy of Fallot.

Functional Status of the Surviving PatientsFifty-five patients were alive at the last

follow-up (Fig. 3). Forty-five (82%) were inNYHA class I, and ten were in class II or III.No patient was in class IV. Most of the surviv-ing patients were leading productive lives,both at home and professionally.

166 Tetralogy of Fallot in Adults: Surgery and Follow-up

x x x xx x x

x x x xx x x

Vol. 14, No. 2, June, 1987

Functional Statusof Survivors(N = 55)

BeforeTotal Correction

NYHAFunctional Class

I (1)

31 (28)

N (25)

IV (1)

LastFollow-up

(1)-+...-,(45)

(9)

(1)

(0)

Fig. 3 Functional status of the 55 surviving patients. Most of the patients who survived the operation had markedsymptomatic relief.

DISCUSSION

Although in recent years almost all car-diologists have recommended that patients withtetralogy of Fallot undergo total correctionduring childhood, a number of patients surviveinto adulthood without a corrective operation.Undoubtedly, these adults, who may or may nothave had a previous palliative procedure, willdevelop a severe cardiovascular disability. Theypresent a challenging problem to internists andcardiologists involved in their care.The natural history of untreated tetralogy

of Fallot patients is well known, and preopera-tive complications in our series resembled thosedescribed elsewhere 6: cerebrovascular acci-dent, syncope, congestive heart failure, bacterialendocarditis, dysrhythmia, and brain abscess.As expected, these preoperative complicationswere more common in older patients. We believethat one of our patients, a 72-year-old man, wasthe oldest patient with tetralogy of Fallot everto survive beyond 70 years of age without pal-liation and the only patient to undergo success-ful total correction at that age.The hospital mortality in our adult patients

(7.8%) was similar to that reported by many

other investigators 7-9 and did not differ fromthe hospital mortality reported in children.Patients with a previous Pbtts shunt had a muchhigher hospital mortality, as shown by otherinvestigators i0i2; this was probably due topulmonary hypertension with pulmonary vascu-lar obstructive disease and surgical difficulty inclosing the shunt.

Previous investigators'3"14 have reported thatthe development of pulmonary hypertensionwith pulmonary vascular obstructive diseaseafter creation of a Potts shunt is related to theduration of the shunt. Two of our patients witha Potts shunt who had preoperative pulmonaryhypertension secondary to elevated pulmonaryvascular resistance died after total correction.They had had a Potts shunt for 16 and 20 years,respectively, before undergoing total correction.The unusually high incidence of excessive

postoperative bleeding requiring reexplorationin our group of adults with tetralogy of Fallotreflects the fact that these patients, who hadbeen subject to life-long cyanosis and polycy-themia, frequently had some degree of coagu-lopathy,'5 as well as numerous collateral vesselsto the lungs, which were the source of bleedingin most cases.

Tetralogy of Falot in Adults: Surgery and FoUow-up 167

..-OOOOOP-(1)

nka Heart Institute Journal

Generally, tetralogy of Fallot patients whosurvive total correction should undergo post-operative cardiac catheterization approximately1 year after surgery.2'6 This practice cannotalways be applied, however, particularly in thecase of patients from other countries. In ourseries, postoperative invasive studies were donein only a small number of selected patients whocomplained of cardiac symptoms or had evidentabnormalities, as documented by noninvasivestudies. Patient selection may be the reason whya high percentage had poor results. Although thecriteria used in our study, which were initiallysuggested by Garson and associates2 for classify-ing the hemodynamic results of total correctionin postoperative patients, are convenient and arefrequently used in clinical practice or research,"excellent or good" results do not necessarilyguarantee that patients are in "excellent or good"condition, since halfofour patients with "excellentor good" results had a significant hemodynamicderangement such as ventricular dysfunction withor without mural thrombi and/or an acquiredcardiac lesion.

Reoperation, which was required in ten cases tocorrect residual defects, was associated with a 10%mortality and produced symptomatic relief in 77%of the survivors. Uretzky and associates'7 reportedsimilar findings, with a low reoperative mortalityand good long-term results. Unlike the patients inother reports,18 none of our patients had reopera-tion for isolated pulmonary insufficiency. Thiscondition seems to be well tolerated in mostpatients who do not have an associated resid-ual defect.

Late death occurred in four cases, all relatedto residual hemodynamic abnormalities, the mostimportant of which was ventricular dysfunctionand/or a residual shunt lesion. Among ourpatients, there were no sudden deaths; accordingto other investigators, this is a common mode oflate death in postoperative tetralogy of Fallotpatients and is thought to be related to ventriculardysrhythmia.2 With a longer follow-up, we mighthave detected more cases of sudden death.

Left ventricular dysfunction was seen in tenpostoperative patients and was associated withmural thrombi and cerebrovascular accidents insome cases. Half of these patients had no otherresidual hemodynamic abnormality on postopera-tive cardiac catheterization. Borow and asso-ciates19 have reported a similar finding, which

suggests that latent left ventricular dysfunctionmay occur in tetralogy of Fallot patients whoundergo repair after infancy, despite the absence ofa residual cardiac lesion. Prolonged exposure ofthe left ventricular myocardium to hypoxemia withvolume overloading may predispose to the latedevelopment of left ventricular dysfunction. Earlydefinitive correction oftetralogy ofFallot may helppreserve ventricular function. The high incidenceof ventricular dysfunction in our adult patients,even in those with an "excellent-to-good" result,support this concept of early repair. Ventriculardysfunction might have been detected more fre-quently if we had done systematic prospectiveventricular function studies.

Previous investigations2'20-24 have empha-sized the importance of late postoperative ven-tricular dysrhythmia, particularly with respect toage at total correction, residual hemodynamicabnormalities, late sudden death, and ventric-ular dysfunction. We found a similar trend inour series. Most of our patients with subjectiverhythm disorders had an abnormal hemody-namic result and/or ventricular dysfunction.

Several investigators have observed tetral-ogy of Fallot patients postoperatively over along term. Our results are similar to theirsexcept for a more frequent incidence of latepostoperative events, seemingly due to ourpatients' more advanced age at the time ofcorrective operation. As Hu and colleagues25have shown in the case of patients over 40 yearsof age at the time of correction, most of thesurvivors had marked symptomatic relief andare leading relatively normal, productive lives.We draw the following conclusions:(1) Total correction of tetralogy of Fallot in

adults is associated with a low operative mortal-ity, similar to that in children, and offers markedsymptomatic relief in most cases.

(2) Long-term survival is excellent, but latesequelae become more frequent with longerfollow-up.

(3) "Corrective" reoperation entails a lowoperative risk.

(4) Most survivors are in NYHA functionalclass I or H.We recommend that, whenever anatomically

feasible, adults with tetralogy of Fallot have totalcorrection regardless of the presence or absenceof symptoms and regardless of any previous,palliative procedure.

168 Tetralogy of Fallot in Adults: Surgery and Follow-up Vol. 14, No. 2, June, 1987

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Tetralogy of Fallot in Adults: Surgery and FoUow-up 169Texas Heart ILmstitute Joumal