Adv. Cardiol., vol. 22, pp. 225-231 (Karger, Basel 1978)
Surgical Implications of Pulmonary Hypertension in Congenital Heart Disease T. KOUCHOUKOS, EUGENE H. BLACKSTONE and W. KIRKLIN
NICHOLAS JOHN
Division of Cardiovascular and Thoracic Surgery, Department of Surgery, University of Alabama Medical Center, Birmingham, Ala.
Pulmonary vascular disease complicates the natural history of a number of congenital cardiac malformations associated with left-to-right shunting, including large ventricular septal defect (YSD), large patent ductus arteriosus, aortapulmonary artery window, complete truncus arteriosus, total anomalous pulmonary venous connection, atrioventricular canal, and double outlet right ventricle without pulmonary stenosis. These malformations can be satisfactorily treated by corrective surgery when the risk of operation is low, the repair is complete, and moderate or severe pulmonary vascular disease is not present postoperatively. The decision to recommend operation depends on knowledge of these factors for each specific lesion. The largest amount of information relating to the effects of these variables on the longterm results of operation, particularly the effect of pulmonary vascular disease, exists for patients with large YSDs, and we shall focus on this lesion in this discussion. The results from these studies should be applicable to the other malformations noted above.
Definition of Pulmonary Vascular Disease
PVR
mean pulmonary artery pressure - mean left atrial pressure pulmonary blood flow/body surface area
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The severity of pulmonary vascular disease and the resistance to flow imposed by it are estimated by calculating the pulmonary vascular resistance (PYR) in units· m 2 •
226
KOUCHOUKOS/BLACKSTONE/KIRKLIN
Table 1. Classification of severity of PVR
Class Nonnal Mild Moderate Severe
Resistance units 9
A classification of PVR is shown in table 1. Patients with moderate or severe disease usually have elevation of the pulmonary arterial pressure (systolic pulmonary artery to systemic artery ratios greater than 0.79) and pulmonary blood flow greater than 1.8 times systemic blood flow.
Effect of Pulmonary Vascular Disease on Results of Surgical Closure of VSD Hospital Mortality In several recent series, the hospital mortality for repair of single large VSDs in patients over 2 years of age has been zero [2]. In general, patients in this age group with severely elevated PVR ( > 10-12 units), pulmonary to systemic blood flow ratios of less than 1.3, and clinical findings to substantiate the hemodynamic data have been considered inoperable. 1.0r 0.9 0.8 £;
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Fig. 1. Probability of hospital death following closure of a lange VSD based upon the combined experience of BLACKSTONE et al. [2] and BARRATI-BoYES et al. [1]. The dotted lines enclose the 70 %confidence bands of the probability curve. The actual proportion of deaths with its 70 % confidence band is shown in each age group of patients. Reproduced by permission from BLACKSTONE et al. [2].
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Age at repair on single VSD, months
Surgical Implications of Pulmonary Hypertension
227
Among patients less than 2 years of age, hospital mortality is approximately 10 % (fig. 1). In a recently reported series of 117 patients with single large defects, preoperative PVR and mean pulmonary artery pressure were poorly related to the incidence of death early postoperatively (p > 0.25) [2]. Similar findings have been noted by SUZUKI et al. [6]. Thus, with current methods of patient selection, and intraoperative and postoperative management, the presence of pulmonary vascular disease does not appear to affect hospital mortality.
Overall Late Results 74 patients having large VSDs completely repaired at the Mayo Clinic between 1955 and 1968 have been evaluated by repeat catheterization 5 or more years after operation [4]. A good late result was defined as survival for 5 or more years with a mean pulmonary artery pressure less than 25 mm Hg. The overall probability of 'surgical cure' (the probability of the patient surviving the operation and early postoperative period and of being alive with a normal or near normal pulmonary artery pressure 5 years later) was derived from the probabilities of hospital and late survival [2]. In this group of patients, age at operation, preoperative PVR, and preoperative mean pulmonary artery pressure were all directly and significantly related to mean pulmonary artery pressure 5 years later. In other words, the younger the patient and the lower the preoperative PVR and pulmonary artery pressure, the greater the probability of a good late result.
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 6:06:29 PM
Late Mortality CARTMILL et al. [3], in a review of 447 patients with VSD operated upon between 1960 and 1965, reported 2 late deaths (0.9 %) in 218 patients with preoperative systolic pulmonary artery to systemic artery pressure ratios of less than 0.75. Among patients in whom this ratio was greater than 0.75, 2 died of 59 with preoperative pulmonary/systemic resistance ratios less than 0.45 (3.4 %),3 among 57 with resistance ratios between 0.45 and 0.75 (5.3 %) and 2 of 6 patients with ratios greater than 0.75 (33 %). Using probability of event analysis, probability curves of late death (within 5 years of operation), according to the preoperative PVR and pulmonary arterial pressure, have been constructed [2] (fig. 2). The probability of late death is higher the higher the PVR and the mean pulmonary arterial pressures are preoperatively. It approaches 33 % when these values are severely elevated. Similar results in patients operated upon after 2 years of age have been reported by FRIEDLI et al. [5].
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The probability curve for surgical cure, as defined above, for patients with single large VSD and, for comparison, the natural history of VSD which is the probability of long-term survival with ultimate spontaneous closure of the defect related to age, are shown in figure 3. The probability of surgical cure after primary repair of a single large VSD is clearly better than the probability of spontaneous cure in patients over 3-4 months of age. The probability of surgical cure begins to decline as age at operation exceeds 18-24 months. The probability of surgical sure, combining age at operation and the preoperative PVR, is shown in figure 4. When this resistance is about
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 6:06:29 PM
Fig. 2. Probability of late death (within 5 years of operation) according to PVR preoperatively. The actual proportions of death obtained from CARTMILL et a/. [3] are shown with their 70 %confidence limits. The dotted lines enclose the 70 %confidence bands of the probability curve. b Probability of late death according to preoperative pulmonary artery pressure. Reproduced by permission from BLACKSTONE et a/. [2].
229
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Age at consideration of operation for single large VSD, months
Fig. 3. Probability of overall 'surgical cure' according to age (months) at the time of repair for patients with a single large VSD. Also shown is the 'natural history' (see text). The dotted lines enclose the 70 % confidence bands. Reproduced by permission from BLACKSTONE
et al. [2].
4 units' m2, the probability of surgical cure is highest (about 92 %) for patients operated upon at 27 months of age. When the resistance is 8 units, the highest probability of surgical cure (87%) is obtained when the operation is done at 6 months of age. When the preoperative resistance is 12 units, the probability of surgical cure is highest (80%) when the operation is done early in life.
Reduction of hospital mortality in recent years for primary repair of single large VSD in infants has altered the optimal age for elective operation. Patients with large VSD not requiring operation on an urgent basis because of cardiac failure or recurrent pulmonary complications, should be operated upon before the age of about 2 years. When PVR is low « 4-5 units' m2), the highest probability of surgical cure occurs when operation is performed at about 27 months of age. If PVR rises during the waiting period, optimal age for operation is reassessed based upon the new value for pulmonary resistance. When PVR is moderately elevated (8 units), the highest probability of surgical cure will result if the operation is performed within the first 6 months of life. Because of the somewhat higher hospital mortality when operation is performed under 3 months of age and because of the likelihood
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 6:06:29 PM
Therapeutic Implications
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Fig. 4. Probability of overall 'surgical cure' (vertical axis) in single large VSD according to age at operation in patients with preoperative PVR of 4 units' m2 (a), 8 units' m 2 (b), and 12 units' m 2 (c) (with 70 % confidence bands). Reproduced by permission from BLACKSTONE et al. [2].
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 6:06:29 PM
.~
Surgical Implications of Pulmonary Hypertension
231
of spontaneous closure of the defect in some patients, operation should be deferred until the age of 6 months. When PVR is severely elevated, but pulmonary blood flow remains 1.3 times systemic blood flow or greater, elective operation between 3 and 6 months of age is advisable. Patients with large single VSD first seen at older ages should have prompt operation if the pulmonary/systemic blood flow ratio is 1.3 or greater. The probability of surgical cure in these older patients is less when PVR is elevated than it would have been if operation were performed in infancy.
Summary The effects of pulmonary vascular disease on the results of surgical closure of single large ventricular septal defects are reviewed. Hospital mortality in infants was not affected by the presence of preoperative pulmonary vascular disease. The late results were clearly related to age at operation, preoperative pulmonary vascular resistance, and pulmonary artery pressure. Probability of event analysis allows selection of the optimal age for elective repair of large ventricular septal defects.
References
2
3 4
5
6
BARRATI-BoYES, B. G.; NEUTZE, J. M.; CLARKSON, P. M.; SHARDEY, G. C., and BRANDT, P. W. T.: Repair of ventricular septal defect in the first two years of life using profound hypothermia circulatory arrest technics. Ann. Surg. 184: 376-390 (1976). BLACKSTONE, E. H.; KIRKLIN, J. W.; BRADLEY, E. L.; DUSHANE, J. W., and ApPELBAUM, A.: Optimal age and results in repair of large ventricular septal defects. J. thorac. cardiovasc. Surg. 72: 661-679 (1976). CARTMILL, T. B.; DUSHANE, J.W.; McGOON, D. C., and .KmKLIN, J.W.: Results of repair of ventricular septal defect. J. thorac. cardiovasc. Surg. 52: 486-499 (1966). DUSHANE, J. W. and KIRKLIN, J. W.: Late results of the repair of ventricular septal defect on pulmonary vascular disease; in KIRKLIN Advances in cardiovascular surgery, pp. 9-16 (Grune & Stratton, New York 1973). FRIEDLI, B.; KInD, B. S. L.; MUSTARD, W. T., and KErrn, J. D.: Ventricular septal defect with increased pulmonary vascular resistance. Late results of surgical closure. Am. J. Cardiol. 33: 403-409 (1974). SUZUKI, Y.; ISHIZAWA, E.; TANAKA, S.; ITOH, T.; SATOH, K.; KOIZUMI, S.; TADOKORO, M.; HORIUCHI, T.; SATOH, T., and KANOH, 1.: Surgical treatment of large ventricular septal defect with pulmonary hypertension in the first 24 months of life. Ann. thorac. Surg. 22 :229-234 (1976).
N. T. KOUCHOUKOS, MD, Professor of Surgery, Division of Cardiovascular and Thoracic Surgery, Department of Surgery, University of Alabama Medical Center, Birmingham, Ala. (USA).
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 6:06:29 PM
2
Surgical Implications of Pulmonary Hypertension in Congenital Heart Disease T. KOUCHOUKOS, EUGENE H. BLACKSTONE and W. KIRKLIN
NICHOLAS JOHN
Division of Cardiovascular and Thoracic Surgery, Department of Surgery, University of Alabama Medical Center, Birmingham, Ala.
Pulmonary vascular disease complicates the natural history of a number of congenital cardiac malformations associated with left-to-right shunting, including large ventricular septal defect (YSD), large patent ductus arteriosus, aortapulmonary artery window, complete truncus arteriosus, total anomalous pulmonary venous connection, atrioventricular canal, and double outlet right ventricle without pulmonary stenosis. These malformations can be satisfactorily treated by corrective surgery when the risk of operation is low, the repair is complete, and moderate or severe pulmonary vascular disease is not present postoperatively. The decision to recommend operation depends on knowledge of these factors for each specific lesion. The largest amount of information relating to the effects of these variables on the longterm results of operation, particularly the effect of pulmonary vascular disease, exists for patients with large YSDs, and we shall focus on this lesion in this discussion. The results from these studies should be applicable to the other malformations noted above.
Definition of Pulmonary Vascular Disease
PVR
mean pulmonary artery pressure - mean left atrial pressure pulmonary blood flow/body surface area
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 6:06:29 PM
The severity of pulmonary vascular disease and the resistance to flow imposed by it are estimated by calculating the pulmonary vascular resistance (PYR) in units· m 2 •
226
KOUCHOUKOS/BLACKSTONE/KIRKLIN
Table 1. Classification of severity of PVR
Class Nonnal Mild Moderate Severe
Resistance units 9
A classification of PVR is shown in table 1. Patients with moderate or severe disease usually have elevation of the pulmonary arterial pressure (systolic pulmonary artery to systemic artery ratios greater than 0.79) and pulmonary blood flow greater than 1.8 times systemic blood flow.
Effect of Pulmonary Vascular Disease on Results of Surgical Closure of VSD Hospital Mortality In several recent series, the hospital mortality for repair of single large VSDs in patients over 2 years of age has been zero [2]. In general, patients in this age group with severely elevated PVR ( > 10-12 units), pulmonary to systemic blood flow ratios of less than 1.3, and clinical findings to substantiate the hemodynamic data have been considered inoperable. 1.0r 0.9 0.8 £;
i
0.7
"0 0.6
~
Co
0.5
~ 0.4
.r::.
'b
0.3
~O.2
:n
~ 0.1
~ °0~~6--~~~~'8--~~~~3~0--~36~~4~2~~~~~4~~60·
Fig. 1. Probability of hospital death following closure of a lange VSD based upon the combined experience of BLACKSTONE et al. [2] and BARRATI-BoYES et al. [1]. The dotted lines enclose the 70 %confidence bands of the probability curve. The actual proportion of deaths with its 70 % confidence band is shown in each age group of patients. Reproduced by permission from BLACKSTONE et al. [2].
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 6:06:29 PM
Age at repair on single VSD, months
Surgical Implications of Pulmonary Hypertension
227
Among patients less than 2 years of age, hospital mortality is approximately 10 % (fig. 1). In a recently reported series of 117 patients with single large defects, preoperative PVR and mean pulmonary artery pressure were poorly related to the incidence of death early postoperatively (p > 0.25) [2]. Similar findings have been noted by SUZUKI et al. [6]. Thus, with current methods of patient selection, and intraoperative and postoperative management, the presence of pulmonary vascular disease does not appear to affect hospital mortality.
Overall Late Results 74 patients having large VSDs completely repaired at the Mayo Clinic between 1955 and 1968 have been evaluated by repeat catheterization 5 or more years after operation [4]. A good late result was defined as survival for 5 or more years with a mean pulmonary artery pressure less than 25 mm Hg. The overall probability of 'surgical cure' (the probability of the patient surviving the operation and early postoperative period and of being alive with a normal or near normal pulmonary artery pressure 5 years later) was derived from the probabilities of hospital and late survival [2]. In this group of patients, age at operation, preoperative PVR, and preoperative mean pulmonary artery pressure were all directly and significantly related to mean pulmonary artery pressure 5 years later. In other words, the younger the patient and the lower the preoperative PVR and pulmonary artery pressure, the greater the probability of a good late result.
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 6:06:29 PM
Late Mortality CARTMILL et al. [3], in a review of 447 patients with VSD operated upon between 1960 and 1965, reported 2 late deaths (0.9 %) in 218 patients with preoperative systolic pulmonary artery to systemic artery pressure ratios of less than 0.75. Among patients in whom this ratio was greater than 0.75, 2 died of 59 with preoperative pulmonary/systemic resistance ratios less than 0.45 (3.4 %),3 among 57 with resistance ratios between 0.45 and 0.75 (5.3 %) and 2 of 6 patients with ratios greater than 0.75 (33 %). Using probability of event analysis, probability curves of late death (within 5 years of operation), according to the preoperative PVR and pulmonary arterial pressure, have been constructed [2] (fig. 2). The probability of late death is higher the higher the PVR and the mean pulmonary arterial pressures are preoperatively. It approaches 33 % when these values are severely elevated. Similar results in patients operated upon after 2 years of age have been reported by FRIEDLI et al. [5].
228
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80
PPJ; preoperatively, mm Hg
The probability curve for surgical cure, as defined above, for patients with single large VSD and, for comparison, the natural history of VSD which is the probability of long-term survival with ultimate spontaneous closure of the defect related to age, are shown in figure 3. The probability of surgical cure after primary repair of a single large VSD is clearly better than the probability of spontaneous cure in patients over 3-4 months of age. The probability of surgical cure begins to decline as age at operation exceeds 18-24 months. The probability of surgical sure, combining age at operation and the preoperative PVR, is shown in figure 4. When this resistance is about
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 6:06:29 PM
Fig. 2. Probability of late death (within 5 years of operation) according to PVR preoperatively. The actual proportions of death obtained from CARTMILL et a/. [3] are shown with their 70 %confidence limits. The dotted lines enclose the 70 %confidence bands of the probability curve. b Probability of late death according to preoperative pulmonary artery pressure. Reproduced by permission from BLACKSTONE et a/. [2].
229
Surgical Implications of Pulmonary Hypertension to ..~ 09
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..............
...............
.................
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---- _____ •• ________________ __ 6
~
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~
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Age at consideration of operation for single large VSD, months
Fig. 3. Probability of overall 'surgical cure' according to age (months) at the time of repair for patients with a single large VSD. Also shown is the 'natural history' (see text). The dotted lines enclose the 70 % confidence bands. Reproduced by permission from BLACKSTONE
et al. [2].
4 units' m2, the probability of surgical cure is highest (about 92 %) for patients operated upon at 27 months of age. When the resistance is 8 units, the highest probability of surgical cure (87%) is obtained when the operation is done at 6 months of age. When the preoperative resistance is 12 units, the probability of surgical cure is highest (80%) when the operation is done early in life.
Reduction of hospital mortality in recent years for primary repair of single large VSD in infants has altered the optimal age for elective operation. Patients with large VSD not requiring operation on an urgent basis because of cardiac failure or recurrent pulmonary complications, should be operated upon before the age of about 2 years. When PVR is low « 4-5 units' m2), the highest probability of surgical cure occurs when operation is performed at about 27 months of age. If PVR rises during the waiting period, optimal age for operation is reassessed based upon the new value for pulmonary resistance. When PVR is moderately elevated (8 units), the highest probability of surgical cure will result if the operation is performed within the first 6 months of life. Because of the somewhat higher hospital mortality when operation is performed under 3 months of age and because of the likelihood
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 6:06:29 PM
Therapeutic Implications
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Fig. 4. Probability of overall 'surgical cure' (vertical axis) in single large VSD according to age at operation in patients with preoperative PVR of 4 units' m2 (a), 8 units' m 2 (b), and 12 units' m 2 (c) (with 70 % confidence bands). Reproduced by permission from BLACKSTONE et al. [2].
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 6:06:29 PM
.~
Surgical Implications of Pulmonary Hypertension
231
of spontaneous closure of the defect in some patients, operation should be deferred until the age of 6 months. When PVR is severely elevated, but pulmonary blood flow remains 1.3 times systemic blood flow or greater, elective operation between 3 and 6 months of age is advisable. Patients with large single VSD first seen at older ages should have prompt operation if the pulmonary/systemic blood flow ratio is 1.3 or greater. The probability of surgical cure in these older patients is less when PVR is elevated than it would have been if operation were performed in infancy.
Summary The effects of pulmonary vascular disease on the results of surgical closure of single large ventricular septal defects are reviewed. Hospital mortality in infants was not affected by the presence of preoperative pulmonary vascular disease. The late results were clearly related to age at operation, preoperative pulmonary vascular resistance, and pulmonary artery pressure. Probability of event analysis allows selection of the optimal age for elective repair of large ventricular septal defects.
References
2
3 4
5
6
BARRATI-BoYES, B. G.; NEUTZE, J. M.; CLARKSON, P. M.; SHARDEY, G. C., and BRANDT, P. W. T.: Repair of ventricular septal defect in the first two years of life using profound hypothermia circulatory arrest technics. Ann. Surg. 184: 376-390 (1976). BLACKSTONE, E. H.; KIRKLIN, J. W.; BRADLEY, E. L.; DUSHANE, J. W., and ApPELBAUM, A.: Optimal age and results in repair of large ventricular septal defects. J. thorac. cardiovasc. Surg. 72: 661-679 (1976). CARTMILL, T. B.; DUSHANE, J.W.; McGOON, D. C., and .KmKLIN, J.W.: Results of repair of ventricular septal defect. J. thorac. cardiovasc. Surg. 52: 486-499 (1966). DUSHANE, J. W. and KIRKLIN, J. W.: Late results of the repair of ventricular septal defect on pulmonary vascular disease; in KIRKLIN Advances in cardiovascular surgery, pp. 9-16 (Grune & Stratton, New York 1973). FRIEDLI, B.; KInD, B. S. L.; MUSTARD, W. T., and KErrn, J. D.: Ventricular septal defect with increased pulmonary vascular resistance. Late results of surgical closure. Am. J. Cardiol. 33: 403-409 (1974). SUZUKI, Y.; ISHIZAWA, E.; TANAKA, S.; ITOH, T.; SATOH, K.; KOIZUMI, S.; TADOKORO, M.; HORIUCHI, T.; SATOH, T., and KANOH, 1.: Surgical treatment of large ventricular septal defect with pulmonary hypertension in the first 24 months of life. Ann. thorac. Surg. 22 :229-234 (1976).
N. T. KOUCHOUKOS, MD, Professor of Surgery, Division of Cardiovascular and Thoracic Surgery, Department of Surgery, University of Alabama Medical Center, Birmingham, Ala. (USA).
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 6:06:29 PM
2
