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., F.C.C.P.; and Robert]. Flemma, M.D., F.C.C.P. A total of adults with a variety of congenital heart lesions underwent operation for total correction of their defects. Operative and long-term mortality were and 4 percent, respectively. There has been only one operative death in the pat five years (8 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 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. 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 1-year experience with the surgical treatment of the adult with congenital heart disease. CLINICAL MATERIAL All patients aged 1 years or over who initially had operation for their congenital heart disease in the time period of December 199 to February 194 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 8 percent complete. There were patients, 9 men and 18 women. Ages ranged from 1 to years; the mean age was 4 years. RESULTS The operative (one month) mortality was 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 8 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, 98 Wen Bluemound Road, Milwaukee KAY ET AL percent) had some surgical or residual nonsurgical symptoms which varied from severe congestive heart failure to mild rhythm disturbances. Fifty-four patients ( percent) were grouped in New York Heart Association class or 4 before surgery, but only four ( percent) were grouped in class or 4 after surgery (Table ). Pulmonary hypertension (mean pulmonary blood pressure greater than mm Hg) was present in 9 percent of the patients (Table ). Atrial Septal Defects Table 4 summarizes the data on the group of patients with atrial septal defects. The surgical mortality was percent in 11 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 ( 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 Oval is Type). Three patients died at, 8, and months after operation respectively. All three improved during the postoperative period but died suddenly. Unfortunately, autopsy was not performed. CHEST, 9:, MARCH, 19

Year of Surgery Early deaths 19 19 19 19 19 199 199 Late deaths 194 19 19 19 19 19 19 191 Table I -Summary of Deaths Time after Surgery Lesion* Cause of Death 11 days PAPVR-ASD Pulmonary edema, hemothorax days AVC Congestive heart failure, alcoholic cirrhosis 1 day AVC Adult respiratory distress syndrome 4 hr PS-ASD Myocardial fibrosis days Aortic insuffi- Myocardial infarction cbn::y 4 hr PS-ASD Myocardial fibrosis 4 hr Tetralogy of Patch dehiscence, Fallot pulmonary edema 8 yr Tetralogy of Myocardial fibrosis Fallot mo AVC Bronchopneumonia (schizophrenic) mo Secundum ASD Unknown, no autopsy 8mo Secundum ASD Marfan's syndrome, persistent ASD, no autopsy Unknown, no autopsy Refused to take sodium warfarin (Coumadin), thrombosis of prosthesis Myocardial fibrosis, myocardial infarction Myocardial infarction mo Secundum ASD 4 yr SVA-AVR 18 mo Tetralogy of Fallot mo ASD-ASHD *PAPVR, Partial anomalous pulmonary venous return; ASD, atrial septal defect; A VC, 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 9, 1, 48, 4, and 1 years), and four had residual dyspnea (ages,,, and years). Two patients had immediate postoperative complications; a 4-year-old man had a cerebrovascular accident with partial recovery, and a 9-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 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 percent ( /8). Among the three patients over the age of years, there was one early death and one late death, and one patient was lost to follow-up. Among five patients under 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 1 patients with partial anomalous pulmonary venous return were functional class 1 or before surgery. A -year-old woman with severe pulmonary hypertension died of pulmonary edema and hemothorax on the 11th postoperative day. The other 1 patients showed good improvement (Table ). 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 -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 -year-old man was operated on for repair of Ebstein's anomaly and coexistent atrial septal defect. He did well for months after surgery, but then his prosthetic tricuspid valve underwent thrombotic occli.jsion. The valve was replaced, and the patient did well. A - year-old man with an atrial septal defect, rheumatic mitral and tricuspid valvular disease, and pulmonary hypertension had surgical replacement of his mitral CHEST, 9:, MARCH, 19 SURGERY FOR CONGENITAL HEART DISEASE IN THE ADULT

Table Z--Preoperati11e and Poaaoperarive Funetional Clauifieatioa.4eeordift8 to New Yorlc Hearl.4 oeiatioa C,_ajieation Preoperative Class Postoperative Class Lesion Unknown 4 Unknown 4 Dead A trial septal defect Secur.dum 4 4 1 1 1 9 1 Primum 1 1 PAPVR* 1 1 1 1 TAPVR** 1 1 Other lesions 4 Ventricular septal defect 9 Cyanotic 4 4 Acyanotic 4 Pulmonic stenosis Isolated With atrial septal defect Aortic stenosis IHSSt 1 4 Aneurysm of sinus of Valsalva 4 Other valvular lesions Patent ductus arteriosus 9 1 Coarctation of aorta 1 14 1 Total 1 81 8 14 1 1 1 *PAPVR, Partial anomalous pulmonary venous return. **T APVR, Total anomalous pulmonary venous return. tihss, Idiopathic hypertrophic subaortic stenosis. valve and closure of his atrial septal defect. He did Table S--Prellfllenee and lneidenee o/ Pulmoaary well for four years and then developed bacterial Hyperleuon endocarditis on the prosthetic valve. The patient Pulmonary Hypertension responded well to intensive antibiotic therapy and No. of Prevalence Incidence currently is well without evidence of endocarditis. Lesion Patients (No.) (percent) Ventricular Septal Defects Secundum atrial septal defect 8 9 Tables and 4 detail the basic data. Six of the ten PAPVR* 1 1 patients were symptomatic before surgery. There were no operative or late deaths. The one postopera- TAPVR** 1 tive complication was atrioventricular block; in that Other atrial patient a permanent pacemaker was impl~ted. septal defects Postoperative functional result was satisfactory in all Ventricular septal cases. defect 1 1 Aneurysm of sinus of Valsalva There were nine cyanotic and six acyanotic pa- Patent ductus tients in this group. The one operative and two late arteriosus 18 11 deaths (Table 4) occurred in the three oldest pa- Total series of tients (4, 4, and 4 years of age, respectively). patients 18 9 The operative death was because of a technical *P APVR, Partial anomalous pulmonary venous return. problem, but the two late deaths at two and.eight **TAPVR, Total anomalous pulmonary venous return. 8 KAY ET AL CHEST, 9:, MARCH, 19

Atrial septal defects Secundum type Primum type Table 4-Morlfllily, Morbidily, s-, and A e in l.eaion GrouJM Mortality Sex Age (yr) ~ ~ Lesion No. Early Late Morbidity M F <1 1-9 4-49 > Associated with partial anomalous pulmonary venous return Associated with total anomalous pulmonary venous return Associated with other cardiac lesions Ventricular septal defects Cyanotic A cyanotic Pulmonic stenosis Isolated With atrial septal defect Aortic stenosis Idiopathic hypertrophic subaortic stenosis Aneurysm of sinus of Valsalva Other valvular disease Patent ductus arteriosus Aortic coarctation 8 8 1 1 9 18 1 1 4 1 11 4 11 1 1 4 I b 8 9 1 14 years after surgery were the result of progressive biventricular failure. Ninety-three percent ( 14) of the 1 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 mm Hg at rest. There were no operative deaths, and all patients had good results (Table ). 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 ( 1, 1, and 118 mm Hg, respectively), and massive hypertrophy of the right ventricle. The two early deaths were caused by heart failure. Of the six patients with congenital aortic stenosis, three patients had bicuspid aortic valves. Gradients across the valve varied from 48 to 1 mm Hg. All patients were symptomatic (Table ); ages ranged from 1 to 19 years. The three patients with bicuspid valves required aortic valvular replacement, and the other three had commissurotomies. There was no operative or late death. The six patients with idiopathic hypertrophic subaortic stenosis had severe srrnptoms ranging from fatigue (one) to mild congestive heart failure (two) to severe heart failure (three). The outflow gradient varied from to 1 mm Hg. All patients underwent simple trlulsaortic myotomies. Only one patient (the oldest, at 4 years) who was in severe failure before surgery had a poor result, with continued failure after surgery. There were no operative or late deaths. Three of the six patients with aneurysms of the sinus of Valsalva had rupture of the aneurysm into CHEST, 9:, MARCH, 19 SURGERY FOR CONGENITAL HEART DISEASE IN THE ADULT 9

the right atrium. The other three patients had an aneurysm of the right, left, and noncoronary cusps, respectively. Before surgery the only patient without longstanding symptoms was an 18-year-old woman with acute rupture of her aneurysm into the right atrium. Two patients with unruptured aneurysms were symptomatic because of coexistent aortic insufficiency; these two patients required aortic valvular replacement. After surgery, all patients except one were improved or asymptomatic (Table ). One patient did well with his prosthetic valve for four years, but then refused to take his anticoagulant medication and died of complications related to emboli from the valve. Two other patients with congenital valvular lesions, a 1-year-old man with aortic insufficiency and a 1-year-old girl with mitral stenosis, underwent surgery. The patient with aortic insufficiency had hypoplasia of the aortic annulus with supravalvular and valvular aortic stenosis. He underwent an aortic valvular replacement and extensive repair of the supravalvular aortic obstruction; he died of a myocardial infarct after surgery. The patient with mitral stenosis did well after valvulotomy. Extracardiac Lesions Eighteen patients had repair of a patent ductus arteriosus. Ages ranged from 1 to years. Before surgery, nine patients were asymptomatic; after surgery, all but one were asymptomatic. There were no deaths. Two patients had hypertensive (balancedpressure) ducti and severe pulmonary hypertension. Seventeen patients had resection of coarctation of the aorta. The major presenting symptom was hypertension ( 14 patients}, usually discovered at the time of "routine physical examination." Two patients also had symptoms associated with decreased blood flow to their legs, and two were dyspneic. Ages ranged from 1 to years, with a mean of years. The coarctation began at the usual "adult" location just at or distal to the subclavian artery in all cases. Gradients (when measured) varied from 44 to 8 mm Hg. Except for one woman with an unexplained elevation of blood pressure in her right arm, all patients were asymptomatic and had normal blood pressure after surgery. DISCUSSION Congenital heart lesions may be classified into the following two broad categories based upon the pathologic anatomy: ( 1) those with obstruction to flow, and ( ) those with either intracardiac or extracardiac shunts. The sequelae of these two classes of lesions is different. Obstructive lesions (pulmonic stenosis, aortic stenosis, coarctation of the aorta, and 8 KAY ET AL idiopathic hypertrophic subaortic stenosis) cause damage by increasing ventricular work. This results in ventricular hypertrophy or dilatation and progresses to diminished myocardial reserve, congestive heart failure, and myocardial fibrosis. Surgical treatment is directed at relieving the obstruction to flow. After relief of the obstruction, the ventricular workload is decreased and cardiac function is immediately improved. Surgical intervention, therefore, is directed at preservation of the myocardium. Because this myocardial deterioration is a continuous proc~s. we like to repair the lesion early to prevent further loss of myocardium. Shunts present a different problem. In these lesions ( atrial septal defect, ventricular septal defect, patent ductus arteriosus, or ruptured aneurysm of the sinus of V alsalva), a persistent left-to-right shunt increases both pulmonary blood flow and right ventricular work. Whereas reduction of the outflow gradient in an obstructive lesion ~ produce immediate hemodynamic improvement, once severe pulmonary vascular changes have occurred, they may be irreversible. After the shunt has been closed, any residual pulmonary vascular resistance will represent increased right ventricular work. If significant, this increased resistance will lead to further myocardial deterioration. For this reason, it is imperative to correct the defect before irreversible fixed increases in pulmonary resistance have developed. We do not advocate repair of these lesions when fixed pu!monary changes have progressed to the point of shunt reversal, because we do not believe that we can help these people either hemodynamically or symptomatically. Our biggest problem has arisen in the patients with combined obstructive and shunt lesions (tetralogy of Fallot or pulmonic stenosis with atrial septal defect). We have seen little hemodynamic or symptomatic improvement after repair of tetralogy of Fallot in patients over the age of 4 years. Similarly, the degree of right ventricular myocardial fibrosis in patients with severe pulmonic stenosis and atrial septal defects seems to be a limiting factor in operative repair of the lesion. The only way we can help these patients is with earlier diagnosis. Atrial Septal Defect The most common congenital heart defect in the adult is an atrial septal defect of the fossa ovalis type. 11-1 In our series, these patients fell into two categories, the young relatively asymptomatic patient and the older symptomatic patient. The fact that the middle-aged patient with an atrial septal defect has increasing symptoms is well CHEST, 9:, MARCH, 19

known. 8 9 11 14 1 The patient has increasing problems related to increased pulmonary vascular obliterative changes and serious arrhythmias. In this series, 4 percent (nine) of the 14 patients over the age of years in this subgroup had congestive heart failure, and none was asymptomatic. Surgery resulted in good functional improvement (Table ). We believe that we can offer this older group of patients significant symptomatic improvement Our results are consistent with other reports in the literature which confirm good functional improvement in the older group, even with severe preoperative symptoms; 9 1 1 1 however, it should be pointed out that atrial arrhythmias of a severe degree may persist after surgical correction. Sixty percent (nine) of the 1 patients under the age of 1 with the ostium secundum type of atrial septal defect were asymptomatic. There have been no operative deaths in the subgroup of patients with uncomplicated secundum type of atrial septal defects. We advocate early correction in any patient with a 1.:1 or larger left-to-right shunt or any evidence of increased pulmonary vascular resistance. Although a more complicated lesion, the ostium primum defect can be repaired with satisfactory results. 1 Our younger patients seemed to show better functional improvement than the older patients. We see no advantage in withholding surgery from this subgroup of patients. Atrial septal defect complicated by anomalous venous drainage was not associated with any increased morbidity or mortality. Our indications for surgery are the same as for an uncomplicated atrial septal defect. We operated on four patients with a combination of coronary atherosclerosis and secundum type of atrial septal defect. Their major symptoms (angina and congestive heart failure) were related to the coronary arterial disease. We repair atrial septal defects whenever they are present at the time of myocardial revascularization surgery. In summary, we believe that almost all patients with atrial septal defect should have surgical repair. We would exclude those with trivial left-to-right shunts ( < 1.:1) and those with severe obliterative pulmonary vascular disease and bidirectional shunting. Ventricular Septal Defect Ventricular septal defects are one of the most common congenital heart defects, 1 yet only percent of our patients had this lesion. Three factors reduce the number of adult ventricular septal defects: ( 1) frequent repair during infancy, ( ) frequent spontaneous closure, and ( ) high mortality in the untreated newborn. As a result, the patients who survive to be adults have ventricular septal defects which are hemodynamically less significant. The complications associated with medical management of patients with ventricular septal defects are the same as for atrial septal defects, namely, increased pulmonary resistance, heart failure, and paradoxic embolus. We advocate an early aggressive approach for any patient over 1 years old with an unrepaired, hemodynamically significant ventricular septal defect. Patients with tetralogy of Fallot have the greatest longevity of patients with any form of cyanotic congenital heart disease;1 however, without surgery, less than percent survive for ten years. 1 Nine patients in our series were cyanotic before surgery, and six were not. One-third of the patients in the cyanotic group died; none of those in the acyanotic group died. All survivors were New York Heart Association functional class 1 or. Our mortality in the three patients over the age of 4 with tetralogy of Fallot was 1 percent. We believe that the only way to reduce the morbidity and mortality of this lesion is by early diagnosis and early surgery. Ventricular Outflow Obstruction and Valvular Disease As discussed earlier, obstructive lesions must be approached aggressively to interrupt the progression of myocardial damage. We favor pulmonary valvulotomy in patients who have a gradient of more than mm Hg or whose quality of life might be severely curtailed because of the lesion ( eg, a young athlete). Our experience with the combined lesions of pulmonic stenosis and atrial septal defect has not been good. This combination of lesions seems to exact an especially high toll. Despite the fact that our experience with this combination of lesions has been poor, we believe that surgical treatment has a role. Symptomatic patients with aortic stenosis and a gradient of mm Hg or more should undergo surgery. We prefer commissurotomy if possible, rather than replacing the valve. Our experience with idiopathic hypertrophic subaortic stenosis, both in this series and in our total experience, 18 has been gratifying. Simple transaortic myotomy has been sufficient in almost all of our cases. Indications for surgery are similar to those for aortic valvular stenosis. Ruptured aneurysm of the sinus of Valsalva can be a significant cause of left-to-right shunt. The three patients in this series had 1.: 1, 1. : 1, and : 1 CHEST, 9:, MARCH, 19 SURGERY FOR CONGENITAL HEART DISEASE IN THE ADULT 81

left-to-right shunts. Two patients with unruptured aneurysm had associated rheumatic aortic valvular insufficiency and required valvular replacement. We feel that all patients with aneurysms of the sinus of Valsalva should have repair. Ertracardiac Lesions Patent ductus arteriosus was the second most common lesion in this series. Survival to the eighth decade has been reported;19 however, large shunts may lead to occlusive pulmonary vascular changes. We advocate repair whenever there is significant shunting ( > 1.: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. 1 We believe that a gradient of 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 : 19-, 194 Kanjuh VI, Edwards JE: A review of congenital anomalies of the heart and great vessels according to functional categories. Pediatr Clin North Am 11:-1, 194 Mark H, Young D: Congenital heart disease in the adult. Am J Cardioll:9-, 19 4 Johnson WD, Dawes R, Walker JA, et al: Congenital heart disease in adults. Am J Surg 111:8-8, 19 Landry AB, Knight HF, Hulme SH: Atrial septal defect in the elderly. Geriatrics :11-11, 19 Cohn LH, Collins JJ Jr: Surgical treatment of congenital heart disease in adults. Chest 4:-, 19 Bedford DE: The anatomical types of atrial septal defect. Am J Cardiol:8-4, 19 8 Brest AN: Congenital heart disease in adults. Cardiovasc Clin :8-, 19 9 Gault JH, Morrow AG, Gay W, et al: Atrial septal defect in patients over the age of 4 years. Circulation :1-, 198 1 Coles JC, Gergely NE, Bottigliero JB: Congenital heart disease in the adult. Arch Surg 89:1-1, 194 11 Gerbode F, Kerth W, Sabar EF, et al: The operative treatment of congenital heart lesions in adults. J Thorac Cardiovasc Surg 48:1-1, 194 1 Fisher JM, Wilson WR, Theilen EO: Recognition of congenital heart disease in the fifth to eighth decades of life. Circulation :84-8, 1 1 Fontana RS, Edwards JE: Congenital Cardiac Disease: A Review of Cases Studied Pathologically. Philadelphia, WB Saunders Co, 19 14 Wolf PS, Vogel JHK, Pryor R, et al: Atrial septal defect in patients over 4 years of age. Br Heart J : 114-1, 198 1 Richmond DE, Lowe JB, Barratt-Bayes BG: Results of surgical repair of atrial septal defects in the middle aged and elderly. Thorax 4:-4, 199 1 Cooley DA: Results of surgical treatment of atrial septal defect. Am J Cardiol:-1, 19 1 Higgins CB, Mulder : in the adult. Am J Cardiol9:8-84, 19 18 Schmahl TM, Flemma RJ, Tector AJ, et al: Surgical management of idiopathic hypertrophic subaortic stenosis. Ann Thorac Surg 18:4-44, 194 19 Fishman L, Silverthorne MC: Persistent patent ductus arteriosus in the aged. Am Heart J 9:-9, 19 ANNOUNCEMENT AMERICAN COLLEGE OF CHEST PHYSICIANS POSTGRADUATE COURSE Environmental Pulmonary Disease June 1..1, 19 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 8. 8 KAY ET AL CHEST, 9:, MARCH, 19