Surgery for Congenital Heart Disease in the Adult* Harold Kay, M.D.; Derward Lepley, ]r., M.D.; Michael E. Korns, M.D., F.C.C.P.; Alfred]. Tector, M.D., F.C.C.P.; and Robert]. Flemma, M.D., F.C.C.P.

A total of 205 adults with a variety of congenital heart lesions underwent operation for total correction of their defects. Operative and long-term mortality were 3 and 4 percent, respectively. There has been only one operative death in the pat five years (85 patients). While most defects were repaired with good hemodynamic and symptomatic improvement, the three lesions ~ciated with the worst results were cyanotic tetralogy of Fallot, severe

I mproved

diagnostic and therapeutic methods have caused an increase in the number of patients undergoing repair of congenital heart defects during infancy and childhood; however, some patients survive to adulthood without diagnosis or correction of their congenital heart disease. This article discusses our 15-year experience with the surgical treatment of the adult with congenital heart disease. CLINICAL MATERIAL

All patients aged 16 years or over who initially had operation for their congenital heart disease in the time period of December 1959 to February 1974 are included. Excluded are all cases in which a previous procedure had been performed. All patients had cardiac catheterization studies, and nearly all had cineangiographic studies. Follow-up information was gathered from clinical records, with additional information from patient questionnaires. Patient follow-up was 85 percent complete. There were 205 patients, 97 men and 108 women. Ages ranged from 16 to 72 years; the mean age was 34 years. RESULTS

The operative (one month) mortality was 3 percent (seven deaths). There were eight late deaths ( 4 percent) (Table 1). During the past five years, there has been one operative death ( 1 percent) among 85 patients. Thirty-seven patients ( 18 percent) were asymptomatic before surgery. After surgery, 19 ( 9

°From

the Departments of Thoracic and Cardiovascular Surgery and Patholo~l the Medical College of Wisconsin Affiliated Hospitals, Milwaukee. Manuscript received February 19; revision accepted SeptemberS. Reprint requens: Dr. Lepley, 9800 Wen Bluemound Road, Milwaukee 53226

356 KAY ET AL

pulmonic stenosis compHcated by atrial septal defect, and ostium primum atrial septal defect. Myocardial failure due to end-stage myocardial fibrosis was the major cause of operative mortality. Myocardial fibrosis and irreversible pulmonary changes seemed to be the two factors Hmiting operative correction. An aggressive operative approach seems justified based on this study.

percent) had some surgical or residual nonsurgical symptoms which varied from severe congestive heart failure to mild rhythm disturbances. Fifty-four patients ( 26 percent) were grouped in New York Heart Association class 3 or 4 before surgery, but only four ( 2 percent) were grouped in class 3 or 4 after surgery (Table 2). Pulmonary hypertension (mean pulmonary blood pressure greater than 30 mm Hg) was present in 9 percent of the patients (Table 3). Atrial Septal Defects Table 4 summarizes the data on the group of patients with atrial septal defects. The surgical mortality was 3 percent in 115 patients. Two of the three operative deaths occurred in patients with ostium primum defects, and the other occurred in a patient with partial anomalous venous return. Thirteen patients ( 11 percent) of those with atrial septal defects had pulmonary hypertension (Table 4). Repair of secundum defects was by reapproximation of the edges ( 75 cases), pericardia! patch (five cases), or Dacron patch (two cases). Repair of ostium primum defects was by Dacron or Teflon patch (five cases) or by pericardia! patch (three cases). Anomalous venous return was corrected by pericardia! baffie ( 11 cases), Dacron baffie (six cases), or reimplantation of anomalous veins ( two cases ) . Secundum Defects (Defects of Fossa Ovalis Type). Three patients died at 30, 8, and 5 months after operation respectively. All three improved during the postoperative period but died suddenly. Unfortunately, autopsy was not performed. CHEST, 69: 3, MARCH, 1976

Table I -Summary of Deaths Year of Surgery

Time after Surgery

Lesion*

Early deaths 1965 11 days PAPVR-ASD 1966 1966 1966 1966 1969 1969 Late deaths 1964

32 days AVC 1 day

AVC

24 hr PS-ASD 3 days Aortic insufficbn::y 24 hr PS-ASD 24 hr Tetralogy of Fallot 8 yr

1965

22 mo

1966 1967

30 mo 8mo

1967 1967

5mo 4 yr

1970

18 mo

1971

7 mo

Tetralogy of Fallot AVC

Cause of Death Pulmonary edema, hemothorax Congestive heart failure, alcoholic cirrhosis Adult respiratory distress syndrome Myocardial fibrosis Myocardial infarction Myocardial fibrosis Patch dehiscence, pulmonary edema Myocardial fibrosis

Bronchopneumonia (schizophrenic) Secundum ASD Unknown, no autopsy Secundum ASD Marfan's syndrome, persistent ASD, no autopsy Secundum ASD Unknown, no autopsy Refused to take SVA-AVR sodium warfarin (Coumadin), thrombosis of prosthesis Myocardial fibrosis, Tetralogy of Fallot myocardial infarction Myocardial infarction ASD-ASHD

*PAPVR, Partial anomalous pulmonary venous return; ASD, atrial septal defect; AVC, atrioventricular communication defect (primum ASD and cleft mitral valve); PS, pulmonic stenosis; SVA-AVR, sinus of Valsalva aneurysm and aortic valvular replacement; and ASHD, arteriosclerotic heart disease.

In addition, nine patients continued to have problems following operation; five had supraventricular tachycardias (ages 59, 51, 48, 34, and 31 years), and four had residual dyspnea (ages 62, 52, 37, and 27 years). Two patients had immediate postoperative complications; a 40-year-old man had a cerebrovascular accident with partial recovery, and a 59-yearold man had a pulmonary embolus but recovered. Primum Defects (Persistent Common Atrioventricular Canal). The subgroup of eight patients with ostium primum defects includes a variety of congenital malformations involving the atrial and ventricular septa and portions of the tricuspid and mitral valves. The classification of Tandon et aP is used in this report. There were two cases of complete atrioventricular canal, three cases of partial atrioventricular canal, and three cases in which only

CHEST, 69: 3, MARCH, 1976

the septum appeared to be involved. There was an associated atrial septal defect at the fossa ovalis in two cases. Sixty-three percent (five) of the eight patients in this subgroup were symptomatic before surgery. Symptoms included dyspnea on exertion (two patients), congestive heart failure (one patient), paradoxic embolus (one patient), and recurrent pneumonitis (one patient). The operative mortality in this subgroup was 25 percent ( 2/8). Among the three patients over the age of 30 years, there was one early death and one late death, and one patient was lost to follow-up. Among five patients under 30 years of age, there was one early death, one patient who remained symptomatic, and three asymptomatic patients (New York Heart Association class 1 ) . Anomalous Pulmonary Venous Retum. Seventeen percent of the patients with atrial septal defects had coexistent anomalous pulmonary venous return. Fourteen had complete drainage of the right lung into either the superior vena cava or the right atrium. In three other cases, the right superior pulmonary vein drained into the junction of the superior vena cava and the right atrium, with the rest of the drainage in the usual manner. There were two cases of total anomalous pulmonary venous return, one each of the supracardiac and intracardiac types; both survived and are well. Fifteen of 17 patients with partial anomalous pulmonary venous return were functional class 1 or 2 before surgery. A 20-year-old woman with severe pulmonary hypertension died of pulmonary edema and hemothorax on the 11th postoperative day. The other 16 patients showed good improvement (Table 2). Other Types of Atrial Septal Defects. Four patients with coexistent atrial septal defects and coronary atherosclerosis are included. Their major symptoms were angina pectoris and congestive heart failure. All had coronary bypass surgery at the time of repair of the atrial septal defects. A 65-year-old man with severe, three-vessel, coronary arterial disease and an atrial septal defect died seven months after the operation. Two other patients with atrial septal defects are included in this subgroup. A cyanotic 36-year-old man was operated on for repair of Ebstein's anomaly and coexistent atrial septal defect. He did well for 20 months after surgery, but then his prosthetic tricuspid valve underwent thrombotic occli.Jsion. The valve was replaced, and the patient did well. A 62year-old man with an atrial septal defect, rheumatic mitral and tricuspid valvular disease, and pulmonary hypertension had surgical replacement of his mitral

SURGERY FOR CONGENITAL HEART DISEASE IN THE ADULT 357

Table Z--Preoperati11e and Poaaoperarive Funetional Clauifieatioa .4eeordift8 to New Yorlc Hearl .4•oeiatioa C,_ajieation Preoperative Class Lesion

Unknown

Atrial septal defect Secur.dum Primum PAPVR* TAPVR** Other lesions

4 0 0 0 0

Ventricular septal defect Tetralogy of Fallot Cyanotic Acyanotic Pulmonic stenosis Isolated With atrial septal defect Aortic stenosis

34

31 2 13 0

13 21 1 2

Unknown 10 1 1 3

0

35

7 2 0 0 0

62 2 16 2 4

0

9

2

34

9 0 0 0 0

1 1 0 0 0

0 0 0 0

3 3 1 0

0

0

0

0 0

0 0

3 0

0

0

0

0

0

0

2

0

3 4

2 0

4

0

4 5

2

0

5

0

0

0

6

0

0

0

0

6

0

0

0

0

2

1

0

4

2

0

0

0

0

4

0

3

0 0

Aneurysm of sinus of Valsalva

0

Other valvular lesions

0

Patent ductus arteriosus

39 1 13

0

5

0

2

0

0 5 14 57

2 81

0 26

2 0

Dead

0 0

IHSSt

Coarctation of aorta Total

24 3 2 0 0

2

Postoperative Class

0

0

0

0

2

15

0 28

2 14

15 155

0 17

0

0

0

0

0

0 3

0

0 15

*PAPVR, Partial anomalous pulmonary venous return. **TAPVR, Total anomalous pulmonary venous return. tiHSS, Idiopathic hypertrophic subaortic stenosis.

valve and closure of his atrial septal defect. He did well for four years and then developed bacterial endocarditis on the prosthetic valve. The patient responded well to intensive antibiotic therapy and currently is well without evidence of endocarditis. Ventricular Septal Defects

Tables 2 and 4 detail the basic data. Six of the ten patients were symptomatic before surgery. There were no operative or late deaths. The one postoperative complication was atrioventricular block; in that patient a permanent pacemaker was impl~ted. Postoperative functional result was satisfactory in all cases. Tetralogy of Fallot

There were nine cyanotic and six acyanotic patients in this group. The one operative and two late deaths (Table 4) occurred in the three oldest patients (54, 47, and 42 years of age, respectively). The operative death was because of a technical problem, but the two late deaths at two and .eight 358 KAY ET AL

Table S--Prellfllenee and lneidenee o/ Pulmoaary Hyperleuon Pulmonary Hypertension Lesion

No. of Patients

Prevalence (No.)

Incidence (percent)

Secundum atrial septal defect

82

7

9

PAPVR*

17

2

12

TAPVR**

2

2

100

Other atrial septal defects

6

2

33

Ventricular septal defect

10

Aneurysm of sinus of Valsalva

6

2

33

Patent ductus arteriosus

18

2

11

Total series of patients

205

18

9

10

*PAPVR, Partial anomalous pulmonary venous return. **TAPVR, Total anomalous pulmonary venous return.

CHEST, 69: 3, MARCH, 1976

Table 4-Morlfllily, Morbidily,

s-, and

A•e in l.eaion GrouJM

Mortality Lesion Atrial septal defects Secundum type Primum type

No.

Early

82

0

8

2

Associated with partial anomalous pulmonary venous return

17

Associated with total anomalous pulmonary venous return

2

0

Associated with other cardiac lesions

6

0

Ventricular septal defects

10

0

Tetralogy of Fallot Cyanotic Acyanotic

~

Late Morbidity

MF

>50

14

16

30

22

0

3

5

2

3

2

0

0

6

11

0

0

0

2

2

0

6

2

2

4

2

4

0

0

6

0

0

0

I

1

6

0

0

0

b

5

3

2

0

0

0

3

Aortic stenosis

6

0

0

0

5

Idiopathic hypertrophic subaortic stenosis

6

0

0

Aneurysm of sinus of Valsalva

6

0

Other valvular disease

2

Aortic coarctation

17

0

0

years after surgery were the result of progressive biventricular failure. Ninety-three percent ( 14) of the 15 patients in this group were symptomatic before surgery. After surgery, six patients were asymptomatic without residual shunt (including two women with a total of six pregnancies), four patients were asymptomatic with small residual shunts, and two patients were asymptomatic but had minor arrhythmias.

Ventricular Outflow Obstruction and Valvular Disease Seven patients had isolated pulmonic stenosis. Five of the seven were symptomatic, and all but one had gradients of over 60 mm Hg at rest. There were no operative deaths, and all patients had good results (Table 2). Three patients had pulmonic stenosis and atrial septal defect; two of these patients died. All three patients were cyanotic, had bidirectional shunts, the highest right ventricular systolic pressures in this series ( 170, 125, and 118 mm Hg, respectively), and massive hypertrophy of the right

4

2

3

3

0

0

5

7

2

0

0 5

0

0

0

3

0

18

0

3

0

Patent ductus arteriosus

5

7

7

0

10

0

5

0

CHEST, 69: 3, MARCH, 1976

40-49

50

0

With atrial septal defect

21-39

32

6

Pulmonic stenosis Isolated

1.5:1), cardiac decompensation, or evidence of increased pulmonary vascular resistance. In addition to the problem of heart failure, coarctation of the aorta and hypertension are associated with cerebral hemorrhage. 13 We believe that a gradient of 20 mm Hg across a coarctation is hemodynamically significant and is an indication for resection.

lb:FERENCES I Tandon R, MoDer JH, Edwards JE: Unusual longevity in persistent common atrioventricular canal. Circulation 50: 619-626, 1974 2 Kanjuh VI, Edwards JE: A review of congenital anomalies of the heart and great vessels according to functional categories. Pediatr Clin North Am 11:55-105, 1964 3 Mark H, Young D: Congenital heart disease in the adult. Am J Cardioll5:293-302, 1965 4 Johnson WD, Dawes R, Walker JA, et al: Congenital heart disease in adults. Am J Surg 111:830-833, 1966

5 Landry AB, Knight HF, Hulme SH: Atrial septal defect in the elderly. Geriatrics 27:110-117, 1972 6 Cohn LH, Collins JJ Jr: Surgical treatment of congenital heart disease in adults. Chest 64:60-65, 1973 7 Bedford DE: The anatomical types of atrial septal defect. Am J Cardiol5:568-574, 1960 8 Brest AN: Congenital heart disease in adults. Cardiovasc Clin 2:258-265, 1970 9 Gault JH, Morrow AG, Gay W, et al: Atrial septal defect in patients over the age of 40 years. Circulation 37:261272, 1968 10 Coles JC, Gergely NE, Bottigliero JB: Congenital heart disease in the adult. Arch Surg 89:130-133, 1964 11 Gerbode F, Kerth W, Sabar EF, et al: The operative treatment of congenital heart lesions in adults. J Thorac Cardiovasc Surg 48:601-613, 1964 12 Fisher JM, Wilson WR, Theilen EO: Recognition of congenital heart disease in the fifth to eighth decades of life. Circulation 25:84-86, 1002 13 Fontana RS, Edwards JE: Congenital Cardiac Disease: A Review of 357 Cases Studied Pathologically. Philadelphia, WB Saunders Co, 1962 14 Wolf PS, Vogel JHK, Pryor R, et al: Atrial septal defect in patients over 45 years of age. Br Heart J 30: 114-125, 1968 15 Richmond DE, Lowe JB, Barratt-Bayes BG: Results of surgical repair of atrial septal defects in the middle aged and elderly. Thorax 24:536-542, 1969 16 Cooley DA: Results of surgical treatment of atrial septal defect. Am J Cardiol5:605-610, 1960 17 Higgins CB, Mulder 00: Tetralogy of Fallot in the adult. Am J Cardiol29:837-846, 1972 18 Schmahl TM, Flemma RJ, Tector AJ, et al: Surgical management of idiopathic hypertrophic subaortic stenosis. Ann Thorac Surg 18:437-446, 1974 19 Fishman L, Silverthorne MC: Persistent patent ductus arteriosus in the aged. Am Heart J 39:762-769, 1950

ANNOUNCEMENT AMERICAN COLLEGE OF CHEST PHYSICIANS POSTGRADUATE COURSE Environmental Pulmonary Disease June 10..12, 1976 New York, New York This course will be devoted to an in-depth exposition of environmental lung disease. The first session will be devoted to basic and applied science including epidemiology, immunology, physiology, pathology, and mineralogy. The remaining segments will explore clinical, public health and social problems encountered in pneumoconiosis, hypersensitivity lung disease, cancer and obstructive pulmonary disease. For further information·, write Dale E. Braddy, Director of Education, American College of Chest Physicians, 911 Busse Highway, Park Ridge, Illinois 60068.

382 KAY ET AL

CHEST, 69: 3, MARCH, 1976

Surgery for congenital heart disease in the adult.

Surgery for Congenital Heart Disease in the Adult* Harold Kay, M.D.; Derward Lepley, ]r., M.D.; Michael E. Korns, M.D., F.C.C.P.; Alfred]. Tector, M.D...
1MB Sizes 0 Downloads 0 Views