- Email: [email protected]
Late results of operation for discrete subaortic stenosis
ABSTRACTS
HEMODYNAMIC EVALUATION OF THE FONTAN PROCEDURE AT REST AND WITH EXERCISE William E. Hellenbrand, MD; Hillel Laks, MD; Charles S. Kleinman, MD; Norman S. Talner, MD, Yale University School of Medicine, New Haven, CT Hemodynamic assessment was performed in seven patients (pts) one year following a Fontan procedure. The diagnoses included tricuspid atresia (4), pulmonary atresia (1). mitral atresia (1) and a criss-cross heart (1). Mean age at operation was 15 (g-21 years). At surgery, a 25 mm porcine conduit was interposed between the right atrium (RA) and the right ventricle (RV) in three pts and between the RA and pulmonary artery (PA) in 4. The tricuspid valve was closed in 2 pts and the RA partitioned in one. There was an SVC-RPA anastomosis present in 3 pts. Five of the seven pts were asymptomatic with two in a chronic low output state. Herodynamic evaluation was performed at rest and during supine exercise. At rest, mean cardiac index (CI) was 2.0 L/min/M2 with P.&PA conduits and 2.7 L/&"/M2 with RA-RV conduits. Right atria1 mea" pressure averaged 20 mmHg in R&PA conduits and 10 mmHg in RA-RV conduits. An "a" wave gradient was present across the porcine valve in the RA-RV conduits ranging from 2 to 10 mmHg with no pressure difference in the R&PA conduits Left ventricular ejection fraction was low normal with a mea" of 0.55. During exercise, CI increased from 2.7 to 4.1 L/minJM2. The entire augmentation in systemic flow was dependent on the change in heart rate. Mean right atria1 pressure increased from 11 to 19 mmHg. When changs in cardiac output were related to oxygen consumption during exercise all pts were abnormal with a mea" exercise factor of 4 (nonnal>6). Continuous antegrade flow into the pulmonary arteries was present during systole and diastole in 6 pts. Despite clinical improvement following the Fontan operation abnormal hemodynamics are present at rest and become more significant with exercise.
REST AND EXERCISEHEMODYNAMICS AFTER FONTANPROCEDURE. Giora Ben Shachar, MD, Bradley P. Fuhrman, MD, Yang Wang, MD, Russell V. Lucas, MD, James $. Lock, MD; University of Minnesota, Minneapolis, Minnesota. Sixteen patients, aged 5-26, underwent a Fontan procedure with no operative deaths. Two late deaths were sudden and unexpected. Marked clinical improvement occurred in most survivors; 11 of 14 were NYHAclass I or II. Eleven of the 14 agreed to postop catheterization. Four had developed right-to-left shunts through an oversew" atrioventricular valve ring, invalidating CI measurement. Hemodynamic data on the remaining 7 patients, including 4 atboth rest and exercise, were compared to published results in normals of similar age. None of the exercised patientshad a previous Glenn anastomosis; all were NYHAclass I. N C.G.
Mean RA Pressure
Stroke PA O2 Index SaturaL/min/M2 ml/min/M2 tion(%) C.
I.
mf3 mm@ Fontan: Rest 7 2+1 14+3* 2.3+0.3* 29+6* 64+10* Pre-exercise 4 2Tl 14T2* 2.47b.3* 3OT8* 68’;9 4 ST5 267b* 4.8rl.2* 33-;14* Exercise 32T7* 23 Normal rest 4;i 4.4ro.7 53TlO 76+4 ,I exercise 23 353 8.OF1.3 57711 SOT10 At rest, the CI (Fick) was low due to a small stroke index, despite normal PA pressures and a minimal conduit gradient (CG). Exercise resultedinmarkedPAdesaturation, a small rise in CI, and the appearanceofa variable (5-13) but significant CG. Despite substantial clinical improvement after Fontan procedure, these data demonstrate a decreased CI both at rest and at exercise. Further, marked RA hypertension and functional conduit obstruction occur with exercise, even in Patients with favorable clinical results. The long-term impiications of these results are unknown. (Data a&mean + S.D. *p
432
February 1981
The American Journal of CARDIOLOGY
LATE RESULTS OF OPERATION FOR DISCRETE SUBAORTIC STENOSIS Glenn R. Barnhart, M.D.: Michael Jones, M.D.; Andrew G. Morrow, M.D., National Heart Institute, Bethesda, Md. Between 1956 and 1979 we performed complete resection of the fibrous ring in 53 patients with discrete subaortic stenosis. Six patients had associated anomalies: PDA, VSD, RV outflow obstruction, and/or Ao to RV fistula. Five patients suffered perioperative deaths; 4 were during our very early experience. Excluding 12 foreign patients, late follow-up evaluations including cardiac catheterizations were performed at our institution for all 36 survivors 1 to 24 years (mean 12.2 years) postoperatively. Gradients preoperatively averaged 98 mm Hg (35-190x gradients at 2 to 168 months (mean 18 months) postop averaged 38 mm Hq (o-200). Eight patients had residual gradients over 50 mm Hg, of whom 6 had the tunnel form of LV outflow obstruction. There were 6 late cardiac deaths. Of the lonqterm survivors 23 of 30 are asymptomatic. Six patients have required reoperation, and 3 patients have developed bacterial endocarditis. Aortic regurgitation was present in 22 patients preop; 5 additional patients have it late postop. Cardiothoracic ratios averaged 0.53 preop and 0.44 late postop. ECG criteria for LVH were present in 26 patients preop and in 10 late postop. Average echocardiographic LV systolic and diastolic dimensions were normal late postop, 30 and 49 mm, respectively. Actuarial survival is 94% at 5 years (n=28), 87% at 10 years (n=22), and 81% at 20 years (r-1=7).Survival without adverse cardiac abnormalities (reoperation, bacterial endocarditis, residual gradient-50 mm Hq, cardiothoracic ratio z-than 0.60, or definite LVH by ECG criteria) is similar at 5 years (54%) and at 10 years (55%). We conclude that resection relieves symptoms and obstruction and gives satisfactory late results for most patients with discrete snbaortic stenosis, but residual cardiac abnormalities require continuing long-term follow-up.
TETRALOGY OF FALLGT: 11 YEAR EXPERIENCE IN NEW ENGLAND Lucy P. Buckley, MD and Donald C. Fyler, MD, FACC, New England Regional Infant Cardiac Program, Children's Hospital Medical Center, Boston, MA Over the past 11 years, 407 of 4,505(9%) infants born in New England with critical congenital heart disease had tetraloqy of Fallot(TF) as their major cardiac malformation. Despite a 25% drop in birthrate, the number of new patients over the years did not vary and case discovery increased from .18 to .25 per 1000 live births. Overall, 102 of 407(25%) infants died before their first birthday and the loss was twice as great among the 87 infants admitted within the first 48 hours of life. As expected, mortality was greater among those with associated cardiac lesions: coronary abnormalities(20), absent pulmonary valve(l3), absent pulmonary artery(E), arch anomalies (101 aortic and mitral problems(9), congenital complete heart black(3), total anomalous pulmonary venous return(2), car triatriatum (l), aorticopulmonary window(l), juxtaposition atria1 appendages(l) and Ebstein's anomaly(l). When the 118 of 407 (29%) "high risk" infants with low birthweight (<2.5kq) and/or severe associated anomalies were excluded, first year medical mortality was halved but surgical mortality was unchanged. Among the 313 infants with TF, 187 (60%) were managed surgically: operative mortality was 17%(18 of 108) for palliative shunts and 26%(19 of 74) for primary repair. Mortality was significantly higher among the 94 infants with pulmonary atresia with ventricular septal defect: shunt mortality was 30%(16 of 54) and 2 of 3 died at attempted repair. Of the 45 infants operated in 1968-69, 42 were shunted and 14(31%) died, whereas of the 43 infants operated in 1977-78, 25 were repaired and 4(9%) died.Comparison of surgical experience over time suggests that primary repair of TF syndrome in the first year of life is superior to palliative shunting and later repair.
Volume 47
HEMODYNAMIC EVALUATION OF THE FONTAN PROCEDURE AT REST AND WITH EXERCISE William E. Hellenbrand, MD; Hillel Laks, MD; Charles S. Kleinman, MD; Norman S. Talner, MD, Yale University School of Medicine, New Haven, CT Hemodynamic assessment was performed in seven patients (pts) one year following a Fontan procedure. The diagnoses included tricuspid atresia (4), pulmonary atresia (1). mitral atresia (1) and a criss-cross heart (1). Mean age at operation was 15 (g-21 years). At surgery, a 25 mm porcine conduit was interposed between the right atrium (RA) and the right ventricle (RV) in three pts and between the RA and pulmonary artery (PA) in 4. The tricuspid valve was closed in 2 pts and the RA partitioned in one. There was an SVC-RPA anastomosis present in 3 pts. Five of the seven pts were asymptomatic with two in a chronic low output state. Herodynamic evaluation was performed at rest and during supine exercise. At rest, mean cardiac index (CI) was 2.0 L/min/M2 with P.&PA conduits and 2.7 L/&"/M2 with RA-RV conduits. Right atria1 mea" pressure averaged 20 mmHg in R&PA conduits and 10 mmHg in RA-RV conduits. An "a" wave gradient was present across the porcine valve in the RA-RV conduits ranging from 2 to 10 mmHg with no pressure difference in the R&PA conduits Left ventricular ejection fraction was low normal with a mea" of 0.55. During exercise, CI increased from 2.7 to 4.1 L/minJM2. The entire augmentation in systemic flow was dependent on the change in heart rate. Mean right atria1 pressure increased from 11 to 19 mmHg. When changs in cardiac output were related to oxygen consumption during exercise all pts were abnormal with a mea" exercise factor of 4 (nonnal>6). Continuous antegrade flow into the pulmonary arteries was present during systole and diastole in 6 pts. Despite clinical improvement following the Fontan operation abnormal hemodynamics are present at rest and become more significant with exercise.
REST AND EXERCISEHEMODYNAMICS AFTER FONTANPROCEDURE. Giora Ben Shachar, MD, Bradley P. Fuhrman, MD, Yang Wang, MD, Russell V. Lucas, MD, James $. Lock, MD; University of Minnesota, Minneapolis, Minnesota. Sixteen patients, aged 5-26, underwent a Fontan procedure with no operative deaths. Two late deaths were sudden and unexpected. Marked clinical improvement occurred in most survivors; 11 of 14 were NYHAclass I or II. Eleven of the 14 agreed to postop catheterization. Four had developed right-to-left shunts through an oversew" atrioventricular valve ring, invalidating CI measurement. Hemodynamic data on the remaining 7 patients, including 4 atboth rest and exercise, were compared to published results in normals of similar age. None of the exercised patientshad a previous Glenn anastomosis; all were NYHAclass I. N C.G.
Mean RA Pressure
Stroke PA O2 Index SaturaL/min/M2 ml/min/M2 tion(%) C.
I.
mf3 mm@ Fontan: Rest 7 2+1 14+3* 2.3+0.3* 29+6* 64+10* Pre-exercise 4 2Tl 14T2* 2.47b.3* 3OT8* 68’;9 4 ST5 267b* 4.8rl.2* 33-;14* Exercise 32T7* 23 Normal rest 4;i 4.4ro.7 53TlO 76+4 ,I exercise 23 353 8.OF1.3 57711 SOT10 At rest, the CI (Fick) was low due to a small stroke index, despite normal PA pressures and a minimal conduit gradient (CG). Exercise resultedinmarkedPAdesaturation, a small rise in CI, and the appearanceofa variable (5-13) but significant CG. Despite substantial clinical improvement after Fontan procedure, these data demonstrate a decreased CI both at rest and at exercise. Further, marked RA hypertension and functional conduit obstruction occur with exercise, even in Patients with favorable clinical results. The long-term impiications of these results are unknown. (Data a&mean + S.D. *p
432
February 1981
The American Journal of CARDIOLOGY
LATE RESULTS OF OPERATION FOR DISCRETE SUBAORTIC STENOSIS Glenn R. Barnhart, M.D.: Michael Jones, M.D.; Andrew G. Morrow, M.D., National Heart Institute, Bethesda, Md. Between 1956 and 1979 we performed complete resection of the fibrous ring in 53 patients with discrete subaortic stenosis. Six patients had associated anomalies: PDA, VSD, RV outflow obstruction, and/or Ao to RV fistula. Five patients suffered perioperative deaths; 4 were during our very early experience. Excluding 12 foreign patients, late follow-up evaluations including cardiac catheterizations were performed at our institution for all 36 survivors 1 to 24 years (mean 12.2 years) postoperatively. Gradients preoperatively averaged 98 mm Hg (35-190x gradients at 2 to 168 months (mean 18 months) postop averaged 38 mm Hq (o-200). Eight patients had residual gradients over 50 mm Hg, of whom 6 had the tunnel form of LV outflow obstruction. There were 6 late cardiac deaths. Of the lonqterm survivors 23 of 30 are asymptomatic. Six patients have required reoperation, and 3 patients have developed bacterial endocarditis. Aortic regurgitation was present in 22 patients preop; 5 additional patients have it late postop. Cardiothoracic ratios averaged 0.53 preop and 0.44 late postop. ECG criteria for LVH were present in 26 patients preop and in 10 late postop. Average echocardiographic LV systolic and diastolic dimensions were normal late postop, 30 and 49 mm, respectively. Actuarial survival is 94% at 5 years (n=28), 87% at 10 years (n=22), and 81% at 20 years (r-1=7).Survival without adverse cardiac abnormalities (reoperation, bacterial endocarditis, residual gradient-50 mm Hq, cardiothoracic ratio z-than 0.60, or definite LVH by ECG criteria) is similar at 5 years (54%) and at 10 years (55%). We conclude that resection relieves symptoms and obstruction and gives satisfactory late results for most patients with discrete snbaortic stenosis, but residual cardiac abnormalities require continuing long-term follow-up.
TETRALOGY OF FALLGT: 11 YEAR EXPERIENCE IN NEW ENGLAND Lucy P. Buckley, MD and Donald C. Fyler, MD, FACC, New England Regional Infant Cardiac Program, Children's Hospital Medical Center, Boston, MA Over the past 11 years, 407 of 4,505(9%) infants born in New England with critical congenital heart disease had tetraloqy of Fallot(TF) as their major cardiac malformation. Despite a 25% drop in birthrate, the number of new patients over the years did not vary and case discovery increased from .18 to .25 per 1000 live births. Overall, 102 of 407(25%) infants died before their first birthday and the loss was twice as great among the 87 infants admitted within the first 48 hours of life. As expected, mortality was greater among those with associated cardiac lesions: coronary abnormalities(20), absent pulmonary valve(l3), absent pulmonary artery(E), arch anomalies (101 aortic and mitral problems(9), congenital complete heart black(3), total anomalous pulmonary venous return(2), car triatriatum (l), aorticopulmonary window(l), juxtaposition atria1 appendages(l) and Ebstein's anomaly(l). When the 118 of 407 (29%) "high risk" infants with low birthweight (<2.5kq) and/or severe associated anomalies were excluded, first year medical mortality was halved but surgical mortality was unchanged. Among the 313 infants with TF, 187 (60%) were managed surgically: operative mortality was 17%(18 of 108) for palliative shunts and 26%(19 of 74) for primary repair. Mortality was significantly higher among the 94 infants with pulmonary atresia with ventricular septal defect: shunt mortality was 30%(16 of 54) and 2 of 3 died at attempted repair. Of the 45 infants operated in 1968-69, 42 were shunted and 14(31%) died, whereas of the 43 infants operated in 1977-78, 25 were repaired and 4(9%) died.Comparison of surgical experience over time suggests that primary repair of TF syndrome in the first year of life is superior to palliative shunting and later repair.
Volume 47