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Relation of body habitus to the severity of mitral stenosis in women
March 1,1985
units) is: PVR = PA - LA pressure/CO, where pressures are expressed in mm Hg and cardiac output in literslmin. Multiple linear regression analysis was performed to determine whether any of the independent variables-age, heart rate, LA pressure, PVR, pulmonary artery systolic and diastolic pressures and cardiac output-were significantly related to the dependent variable, PAW pressure. The characteristics of the 49 patients who had elevated PVR and both LA and PAW pressures recorded are shown in Table I. Forty-nine percent were men and 51% were women. All patients had valvular heart disease. Diagnoses and their incidences were mitral regurgitation (Zgpatients), mitral stenosis (28 patients), aortic regurgitation (26 patients), aortic stenosis (20 patients), atherosclerotic heart disease (16patients) and mitral valve prolapse (1 patient). A mean of 2.5 diagnoses per patient was made. A multiple, Linear regression analysis showed that of the variables just listed, only the LA pressure correlated significantly with the PAWpressure. The curve of best fit for the LA-PAWpressure relation, as shown in Figure 1, was described by the formula: PAW pressure = 0.9 X LA pressure + 4.4 (P = 0.84, p
This study demonstrates that the excellent correlation between PAW and LA pressures noted in previous investigations1F2is not altered by the presence of severe elevations in PVR. While the correlation between PAW and LA pressures was excellent, some outlying points were noted (Fig. 1). The scatter in the data may be due in part to the fact that PAW and LA pressure recordings were not simultaneous in all patients. It is of interest that the patient who had the least similarity between PAW and LA pressures (50 and 22 mm Hg, respectively)
Relation of Body Habitus to the Severity of Mitral Stenosis in Women PASQUALE F. NESTICO, MD ABDULMASSIH S. ISKANDRIAN, A-HAMID HAKKI, MD DEMETRIOS KIMBIRIS, MD CHARLES E. BEMIS, MD BERNARD L. SEGAL, MD
Wood described wasting and lean body weight in patients with severe mitral stenosis (MS) who had congestive heart failure. 1~2Cardiac cachexia has been attributed to the catabolic effect of congestive heart failure and to the increased work of breathing.3 This report examines the relation between body habitus and the severity of MS in 79 women. Between January 1977 and June 1983,79 women underwent cardiac catheterization at our institution and had pure or dominant MS. Men were excluded, as were all patients with moderate or severe mitral or aortic regurgitation, aortic stenosis, tricuspid valve disease, associated coronary artery disease and previous cardiac surgery. During the same period, 47 women with normal hemodynamic findings, normal From the Likoff Cardiovascular Institute of Hahnemann University and Hospital, Broad and Vine Streets, Philadelphia, Pennsylvania 19102. Manuscript received May 9, 1984; revised manuscript received November 27, 1984, accepted November 29, 1984.
THE AMERICAN JOURNAL OF CARDIOLOGY Volume 55
057
had the highest PVR (33 Wood units). However, the other four patients with PVR L 10 had PAW and LA measurements within 4 mm Hg of each other. The origin of an elevated PVR in the presence of normal blood flow has not been completely defined, but is thought to be due to a combination of vessel obliteration, with abnormal histology in virtually every element of the circulation, and vascular spasm.3-5 This marked alteration in architecture coupled with vasospasm would be expected to change the rheologic properties of the pulmonary vascular system. Thus, it was of interest to determine if the change in fluid dynamics interferes with the PAW-LA pressure relation. This study suggeststhat the material alterations of the vessels occurring in elevated PVR do not significantly interfere with pressure transmission. Although this study was composed of patients with valvular and ischemic heart disease studied in the catheterization laboratory, it is likely that the PAW-LA pressure relation is valid for other groups with elevated PVR as long as controlled respiration with positive end-expiratory pressure is not present. References DC, Kirklin JW, Wood EH. The relationship between pulmonary artery wedge pressure and left atrial pressure in man. Circ Res 1954;2:
1. Connally
434-440. 2. Luchsinger
PC, Sipp HW, Pate1 DJ. Relationship of pulmonary artery-wedge pressure to left atrial pressure in man. Circ Res 1962;11:315-318. 3. Wagenvoort CA, Wagenvoort N. Pathology of Pulmonary Hypertension. New York: Wiley & Sons: 1977:1-16. 4. Edwards JE. Functronal pathology of the pulmonary vascular tree in congenital cardiac disease. Circulation 1957;15:164-196. * 5. Parker F, Weiss S. The nature and significance of the structural changes in the lungs in mitral stenosis. Am J Pathol 1936;12:573-598.
coronary arteriography and no valvular heart disease underwent cardiac catheterization and these normal women served as control subjects. Each patient underwent combined left- and right-sided cardiac catheterization. Simultaneous left ventricular and pulmonary artery wedge pressures were recorded. Cardiac output was measured by the Fick method. Each patient also underwent left ventriculography, aortography and selective coronary arteriography. The mitral valve area was calculated by the Gorlin formula.4 The diastolic filling period was measured between the crossover points of the pulmonary artery wedge and the ventricular pressure tracings.
TABLE I
Relevant Data in Women with Mitral Stenosis and in Control Subjects Mitral Stenosis (n = 74) Mean f SD
Age (years) BSA (M2) Weight (pounds) Height (inches) WTIHT (pounds/inches) Body mass index (kg/M’.5) PAW-LV (mm Ha) MVA (cmq “’ MVA index (cm2/M2)
52 f 1.65 f
13 0.19
140 f 32
Control Subjects (n = 47) Mean f SD 56f 1.76 f
12 0.9
161 f 38
P Go3
o.doo9
63.6 f 2.2 f
3.2 0.5
63.5 f 2.5 f
2.4 0.6
o.oNoso5
31.0 f
6.8
35.8 f
8.0
0.0004
15f7 1.20 f 0.62 0.73 f
0.41
.. ..
... .
BSA = body surface area; HT = height; MVA = mitral valve area; NS = not significant; PAW-LV = mean diastolic pressure gradient across the mitral valve; SD = standard deviation; WT = weight.
BRIEF REPORTS
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.
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$la-,<
.
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.
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6 $
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90
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150
210
270
I
90
150
Body Weight (lb81
FIGURE 1. Correlation valve
between
270
I:0 90
Body Weight (Ibs)
body weight and (A) mitral valve area, (B) cardiac output and (C) mean diastolic pressure gradient across the mitral
(PAW-LV).
The body mass index5 was derived as follows: w/h1.5, where w = weight (in kilograms) and h equals height (in meters) to the power function of 1.5. Data are presented as mean f standard deviation. The significance of group differences was compared by analysis of variance with the UCLA-BMDPstatistical package. The chi-square test (Yates-corrected) was used to assess the differences in different groups. Linear regression analysis was used to assess the relation between two variables. The level of statistical significance was p X0.05. The patients were 26 to 75 years old (mean 52). They weighed 83 to 299pounds (mean 139 f 33). The body surface area ranged from 1.17 to 2.35 m2 (1.6 f 0.19). The weightto-height ratio ranged from 1.33 to 4.54 pounds/inch (2.19 f 0.50). The body-mass index ranged between 18.5 and 62.6 kg/m1.5 (31.0 f 6.8). The pertinent hemodynamic data are listed in Table I.
0
q
MVA >, 1 .Ocm* MVA < 1 .Ocm*
80 d ;6O I?
210
Body Weight (lb@
* ,. ‘: ::‘“‘-2*,___ * ..U.. --s. . . Y=-0.03x +19.7 ... ... ,=-a18 .. . P:Ns . . .
-
Compared to patients with MS, control women had higher body weight, body surface area, weight-to-height ratio and body mass index. Body weight correlated weakly but significantly with cardiac output and mitral valve area, and inversely but insignificantly with mean pressure gradient across the mitral valve (Fig. 1). The lack of a close correlation between the body weight and the severity of MS may be due to the wide variation in cardiac output and pressure difference across the mitral valve among patients with lean body habitus. The relation between the body habitus and the severity of MS is shown in Figure 2. No correlation existed between body weight and mitral valve area index (r = 0.06).
This study was retrospective and, thus, may not provide an explanation of the mechanism of the relation between severity of MS and body habitus. It is not known whether severe MS results in weight loss caused by cachexia of cardiac diseaseor simply that overweight women with MS may be symptomatic early in the course of the disease. Because obesity involves an increase in body surface area and an increase in flow, this may result in an increase in transvalvular gradient and pulmonary venous pressure, and hence more severe symptoms. According to the Gorlin formula, a 50% increasein cardiac output may result in a more than 200% increase in the transvalvular gradient.6 On the other hand, weight loss may result in lower cardiac output and transvalvular pressure gradient, although the anatomic severity of MS remains the same. Our results show that body weight was related to cardiac output and mitral valve area, but not to mitral valve area index. Thus, women who weigh 150 pounds or more, or who have a body surface area of 1.75 m2 or more or body mass index of 33 kg/m1.5 or more, seldom have severe MS. The MS in these patients is more likely to be mild or moderate. Conversely, the severity of MS cannot be predicted in women with lean body habitus. Acknowledgment: We thank Eric M. Umile and Angela Parmley for their assistance in preparing this manuscript.
3150
<150
(SZ,
al.75
BSA (m*)
C2.3
>,2.3
WT/HT (IbUnch)
<33
>,33
WTIHT I5 (kg/m 1.5)
FIGURE 2. Severity of mitral stenosis in patients with different body habitus. BSA = body surface area; MVA = mitral valve area; WT = body weight; WTIHT = ratio of weight to height; WTlHT’.5 = body mass index.
References 1. Wood P. An appreciation of mitral’stenosis. I. Clinical features. Br Med J 1954;1:1051-1063. 2. Wood P. Diseases of the heart and circulation. 2nd ad. Philadelphia: JB Liooincott. 1956:515-561. 3. Abichek N, Shelburne JC, Perloff JK. Clinical aspects of rheumatic valvular disease. Prog Cadiovasc Dis 1973;15:491-537.
March I,1985
4. Gorlin R, Gorlln SG. Hydraulic formula for calculation of the area of the stenotic mitral valve, other cardiac valves and central circulatory shunts. Am Heart J 1951;41:1-29. 6. Simpoulos AP, van lttalie TB. Body weight, health and longevity. Ann Intern
Patent Ductus Arteriosus, Atrial Septal Defect and Branch Pulmonary Artery Stenosis: A Possible New Syndrome LYNN MAHONY, MD WILLIAM E. SCULLY, MD DAVID D. WEAVER, MD DONALD A. GIROD, MD JOHN W. BROWN, MD RANDALL L. CALDWFLL, MD ROGER A. HURWITZ, MD
This report describes 3 family members affected by a constellation of congenital heart defects with possible autosomal dominant transmission. Patient 1: The proband was a g-month-old girl referred for evaluation of congestive heart failure and associated poor weight gain. She was acyanotic and had no dysmorphic features. Physical examination was consistent with patent ductus arteriosus (DA). Cardiac catheterization showed left-to-right shunting at both atria1 and great artery levels with a pulmonary-to-systemic blood flow ratio of 2.6:1. There was evidence of branch pulmonary artery (PA) stenosis; the systolic pressure in the right ventricle was 70% more than that in the left ventricle. Angiograms showed a secundum From the Departments of Pediatrics, Medical Genetics and Surgery, Indiana University School of Medicine, Indianapolis, Indiana 46223. Manuscript received September 10, 1984; revised manuscript received November 14, 1984, accepted November 16, 1984.
FIGURE 1. Angiogram after injection of contrast material in the main pulmonary artery of patient 1 shows marked stenosis at the origin of the left main pulmonary artery (arrow).
THE AMERICAN
JOURNAL
OF CARDIOLOGY
Volume 55
859
Med 1984;100:285-295.
6. Hakki A-H, lskandrian AS, Bemis CE, Kimbiris D, Mintz GS, Sepal BL, Brice C. A simplified valve formula for calculation of stenotic cardiac valve areas. Circulation 1981;63:1050-1055.
atria1 septal defect (ASD) and bilateral branch PA stenosis with a discrete region of severe stenosis of the left PA (Fig. 1). The distal left PA was hypoplastic. The DA was patent and a large ductal aneurysm was present (Fig. 2). The patient underwent ligation of the DA, closure of the ASD and patch angioplasty of the left PA. Patient 2: The brother of patient 1 developed symptoms of congestive heart failure at 3 months of age. Cardiac catheterization showed an ASD, a discrete stenosis at the origin of the left PA and a dilated and patent DA. Patient 3: The 27-year-old mother of patients 1 and 2 underwent repair of a patent DA and closure of an ASD at 2 years of age, She was entirely asymptomatic. Physical examination was normal except for a short,grade 216 systolic murmur at the left sternal border that radiated well to the back and was consistent with peripheral pulmonary stenosis. An extensive review of the family history showed no other known cases of congenital heart disease. There was no evidence of other dysmorphic features such as dislocated lenses, hyperextensible joints, excessive stretchability of the skin or Marfan habitus in any of the patients or other family members.
The presence of both discrete stenosis at the origin of the left PA and DA aneurysm is of interest because the proximal left PA is derived from the proximal left sixth aortic arch and the DA is derived from the distal left sixth arch. Although this does not explain the presence of the ASD, it is possible that an abnormality of the left sixth aortic arch resulted in at least some of the defects in these patients. The occurrence of these congenital heart defects in a mother and both of her children is suggestive of an autosomal dominant mode of inheritance, although
FIGURE 2. Sixty-degree left anterior oblique view of the aortogram in patient 1 shows an aneurysm of the ductus arteriosus (arrow).
units) is: PVR = PA - LA pressure/CO, where pressures are expressed in mm Hg and cardiac output in literslmin. Multiple linear regression analysis was performed to determine whether any of the independent variables-age, heart rate, LA pressure, PVR, pulmonary artery systolic and diastolic pressures and cardiac output-were significantly related to the dependent variable, PAW pressure. The characteristics of the 49 patients who had elevated PVR and both LA and PAW pressures recorded are shown in Table I. Forty-nine percent were men and 51% were women. All patients had valvular heart disease. Diagnoses and their incidences were mitral regurgitation (Zgpatients), mitral stenosis (28 patients), aortic regurgitation (26 patients), aortic stenosis (20 patients), atherosclerotic heart disease (16patients) and mitral valve prolapse (1 patient). A mean of 2.5 diagnoses per patient was made. A multiple, Linear regression analysis showed that of the variables just listed, only the LA pressure correlated significantly with the PAWpressure. The curve of best fit for the LA-PAWpressure relation, as shown in Figure 1, was described by the formula: PAW pressure = 0.9 X LA pressure + 4.4 (P = 0.84, p
This study demonstrates that the excellent correlation between PAW and LA pressures noted in previous investigations1F2is not altered by the presence of severe elevations in PVR. While the correlation between PAW and LA pressures was excellent, some outlying points were noted (Fig. 1). The scatter in the data may be due in part to the fact that PAW and LA pressure recordings were not simultaneous in all patients. It is of interest that the patient who had the least similarity between PAW and LA pressures (50 and 22 mm Hg, respectively)
Relation of Body Habitus to the Severity of Mitral Stenosis in Women PASQUALE F. NESTICO, MD ABDULMASSIH S. ISKANDRIAN, A-HAMID HAKKI, MD DEMETRIOS KIMBIRIS, MD CHARLES E. BEMIS, MD BERNARD L. SEGAL, MD
Wood described wasting and lean body weight in patients with severe mitral stenosis (MS) who had congestive heart failure. 1~2Cardiac cachexia has been attributed to the catabolic effect of congestive heart failure and to the increased work of breathing.3 This report examines the relation between body habitus and the severity of MS in 79 women. Between January 1977 and June 1983,79 women underwent cardiac catheterization at our institution and had pure or dominant MS. Men were excluded, as were all patients with moderate or severe mitral or aortic regurgitation, aortic stenosis, tricuspid valve disease, associated coronary artery disease and previous cardiac surgery. During the same period, 47 women with normal hemodynamic findings, normal From the Likoff Cardiovascular Institute of Hahnemann University and Hospital, Broad and Vine Streets, Philadelphia, Pennsylvania 19102. Manuscript received May 9, 1984; revised manuscript received November 27, 1984, accepted November 29, 1984.
THE AMERICAN JOURNAL OF CARDIOLOGY Volume 55
057
had the highest PVR (33 Wood units). However, the other four patients with PVR L 10 had PAW and LA measurements within 4 mm Hg of each other. The origin of an elevated PVR in the presence of normal blood flow has not been completely defined, but is thought to be due to a combination of vessel obliteration, with abnormal histology in virtually every element of the circulation, and vascular spasm.3-5 This marked alteration in architecture coupled with vasospasm would be expected to change the rheologic properties of the pulmonary vascular system. Thus, it was of interest to determine if the change in fluid dynamics interferes with the PAW-LA pressure relation. This study suggeststhat the material alterations of the vessels occurring in elevated PVR do not significantly interfere with pressure transmission. Although this study was composed of patients with valvular and ischemic heart disease studied in the catheterization laboratory, it is likely that the PAW-LA pressure relation is valid for other groups with elevated PVR as long as controlled respiration with positive end-expiratory pressure is not present. References DC, Kirklin JW, Wood EH. The relationship between pulmonary artery wedge pressure and left atrial pressure in man. Circ Res 1954;2:
1. Connally
434-440. 2. Luchsinger
PC, Sipp HW, Pate1 DJ. Relationship of pulmonary artery-wedge pressure to left atrial pressure in man. Circ Res 1962;11:315-318. 3. Wagenvoort CA, Wagenvoort N. Pathology of Pulmonary Hypertension. New York: Wiley & Sons: 1977:1-16. 4. Edwards JE. Functronal pathology of the pulmonary vascular tree in congenital cardiac disease. Circulation 1957;15:164-196. * 5. Parker F, Weiss S. The nature and significance of the structural changes in the lungs in mitral stenosis. Am J Pathol 1936;12:573-598.
coronary arteriography and no valvular heart disease underwent cardiac catheterization and these normal women served as control subjects. Each patient underwent combined left- and right-sided cardiac catheterization. Simultaneous left ventricular and pulmonary artery wedge pressures were recorded. Cardiac output was measured by the Fick method. Each patient also underwent left ventriculography, aortography and selective coronary arteriography. The mitral valve area was calculated by the Gorlin formula.4 The diastolic filling period was measured between the crossover points of the pulmonary artery wedge and the ventricular pressure tracings.
TABLE I
Relevant Data in Women with Mitral Stenosis and in Control Subjects Mitral Stenosis (n = 74) Mean f SD
Age (years) BSA (M2) Weight (pounds) Height (inches) WTIHT (pounds/inches) Body mass index (kg/M’.5) PAW-LV (mm Ha) MVA (cmq “’ MVA index (cm2/M2)
52 f 1.65 f
13 0.19
140 f 32
Control Subjects (n = 47) Mean f SD 56f 1.76 f
12 0.9
161 f 38
P Go3
o.doo9
63.6 f 2.2 f
3.2 0.5
63.5 f 2.5 f
2.4 0.6
o.oNoso5
31.0 f
6.8
35.8 f
8.0
0.0004
15f7 1.20 f 0.62 0.73 f
0.41
.. ..
... .
BSA = body surface area; HT = height; MVA = mitral valve area; NS = not significant; PAW-LV = mean diastolic pressure gradient across the mitral valve; SD = standard deviation; WT = weight.
BRIEF REPORTS
650
C . 30-
l . . .
z . i24-
”
4..
.
. .
$la-,<
.
m
m
E
.
.
*-
-.._.
.
. . *
-pk
6 $
,2-
P 6L
90
I
I
I
150
210
270
I
90
150
Body Weight (lb81
FIGURE 1. Correlation valve
between
270
I:0 90
Body Weight (Ibs)
body weight and (A) mitral valve area, (B) cardiac output and (C) mean diastolic pressure gradient across the mitral
(PAW-LV).
The body mass index5 was derived as follows: w/h1.5, where w = weight (in kilograms) and h equals height (in meters) to the power function of 1.5. Data are presented as mean f standard deviation. The significance of group differences was compared by analysis of variance with the UCLA-BMDPstatistical package. The chi-square test (Yates-corrected) was used to assess the differences in different groups. Linear regression analysis was used to assess the relation between two variables. The level of statistical significance was p X0.05. The patients were 26 to 75 years old (mean 52). They weighed 83 to 299pounds (mean 139 f 33). The body surface area ranged from 1.17 to 2.35 m2 (1.6 f 0.19). The weightto-height ratio ranged from 1.33 to 4.54 pounds/inch (2.19 f 0.50). The body-mass index ranged between 18.5 and 62.6 kg/m1.5 (31.0 f 6.8). The pertinent hemodynamic data are listed in Table I.
0
q
MVA >, 1 .Ocm* MVA < 1 .Ocm*
80 d ;6O I?
210
Body Weight (lb@
* ,. ‘: ::‘“‘-2*,___ * ..U.. --s. . . Y=-0.03x +19.7 ... ... ,=-a18 .. . P:Ns . . .
-
Compared to patients with MS, control women had higher body weight, body surface area, weight-to-height ratio and body mass index. Body weight correlated weakly but significantly with cardiac output and mitral valve area, and inversely but insignificantly with mean pressure gradient across the mitral valve (Fig. 1). The lack of a close correlation between the body weight and the severity of MS may be due to the wide variation in cardiac output and pressure difference across the mitral valve among patients with lean body habitus. The relation between the body habitus and the severity of MS is shown in Figure 2. No correlation existed between body weight and mitral valve area index (r = 0.06).
This study was retrospective and, thus, may not provide an explanation of the mechanism of the relation between severity of MS and body habitus. It is not known whether severe MS results in weight loss caused by cachexia of cardiac diseaseor simply that overweight women with MS may be symptomatic early in the course of the disease. Because obesity involves an increase in body surface area and an increase in flow, this may result in an increase in transvalvular gradient and pulmonary venous pressure, and hence more severe symptoms. According to the Gorlin formula, a 50% increasein cardiac output may result in a more than 200% increase in the transvalvular gradient.6 On the other hand, weight loss may result in lower cardiac output and transvalvular pressure gradient, although the anatomic severity of MS remains the same. Our results show that body weight was related to cardiac output and mitral valve area, but not to mitral valve area index. Thus, women who weigh 150 pounds or more, or who have a body surface area of 1.75 m2 or more or body mass index of 33 kg/m1.5 or more, seldom have severe MS. The MS in these patients is more likely to be mild or moderate. Conversely, the severity of MS cannot be predicted in women with lean body habitus. Acknowledgment: We thank Eric M. Umile and Angela Parmley for their assistance in preparing this manuscript.
3150
<150
(SZ,
al.75
BSA (m*)
C2.3
>,2.3
WT/HT (IbUnch)
<33
>,33
WTIHT I5 (kg/m 1.5)
FIGURE 2. Severity of mitral stenosis in patients with different body habitus. BSA = body surface area; MVA = mitral valve area; WT = body weight; WTIHT = ratio of weight to height; WTlHT’.5 = body mass index.
References 1. Wood P. An appreciation of mitral’stenosis. I. Clinical features. Br Med J 1954;1:1051-1063. 2. Wood P. Diseases of the heart and circulation. 2nd ad. Philadelphia: JB Liooincott. 1956:515-561. 3. Abichek N, Shelburne JC, Perloff JK. Clinical aspects of rheumatic valvular disease. Prog Cadiovasc Dis 1973;15:491-537.
March I,1985
4. Gorlin R, Gorlln SG. Hydraulic formula for calculation of the area of the stenotic mitral valve, other cardiac valves and central circulatory shunts. Am Heart J 1951;41:1-29. 6. Simpoulos AP, van lttalie TB. Body weight, health and longevity. Ann Intern
Patent Ductus Arteriosus, Atrial Septal Defect and Branch Pulmonary Artery Stenosis: A Possible New Syndrome LYNN MAHONY, MD WILLIAM E. SCULLY, MD DAVID D. WEAVER, MD DONALD A. GIROD, MD JOHN W. BROWN, MD RANDALL L. CALDWFLL, MD ROGER A. HURWITZ, MD
This report describes 3 family members affected by a constellation of congenital heart defects with possible autosomal dominant transmission. Patient 1: The proband was a g-month-old girl referred for evaluation of congestive heart failure and associated poor weight gain. She was acyanotic and had no dysmorphic features. Physical examination was consistent with patent ductus arteriosus (DA). Cardiac catheterization showed left-to-right shunting at both atria1 and great artery levels with a pulmonary-to-systemic blood flow ratio of 2.6:1. There was evidence of branch pulmonary artery (PA) stenosis; the systolic pressure in the right ventricle was 70% more than that in the left ventricle. Angiograms showed a secundum From the Departments of Pediatrics, Medical Genetics and Surgery, Indiana University School of Medicine, Indianapolis, Indiana 46223. Manuscript received September 10, 1984; revised manuscript received November 14, 1984, accepted November 16, 1984.
FIGURE 1. Angiogram after injection of contrast material in the main pulmonary artery of patient 1 shows marked stenosis at the origin of the left main pulmonary artery (arrow).
THE AMERICAN
JOURNAL
OF CARDIOLOGY
Volume 55
859
Med 1984;100:285-295.
6. Hakki A-H, lskandrian AS, Bemis CE, Kimbiris D, Mintz GS, Sepal BL, Brice C. A simplified valve formula for calculation of stenotic cardiac valve areas. Circulation 1981;63:1050-1055.
atria1 septal defect (ASD) and bilateral branch PA stenosis with a discrete region of severe stenosis of the left PA (Fig. 1). The distal left PA was hypoplastic. The DA was patent and a large ductal aneurysm was present (Fig. 2). The patient underwent ligation of the DA, closure of the ASD and patch angioplasty of the left PA. Patient 2: The brother of patient 1 developed symptoms of congestive heart failure at 3 months of age. Cardiac catheterization showed an ASD, a discrete stenosis at the origin of the left PA and a dilated and patent DA. Patient 3: The 27-year-old mother of patients 1 and 2 underwent repair of a patent DA and closure of an ASD at 2 years of age, She was entirely asymptomatic. Physical examination was normal except for a short,grade 216 systolic murmur at the left sternal border that radiated well to the back and was consistent with peripheral pulmonary stenosis. An extensive review of the family history showed no other known cases of congenital heart disease. There was no evidence of other dysmorphic features such as dislocated lenses, hyperextensible joints, excessive stretchability of the skin or Marfan habitus in any of the patients or other family members.
The presence of both discrete stenosis at the origin of the left PA and DA aneurysm is of interest because the proximal left PA is derived from the proximal left sixth aortic arch and the DA is derived from the distal left sixth arch. Although this does not explain the presence of the ASD, it is possible that an abnormality of the left sixth aortic arch resulted in at least some of the defects in these patients. The occurrence of these congenital heart defects in a mother and both of her children is suggestive of an autosomal dominant mode of inheritance, although
FIGURE 2. Sixty-degree left anterior oblique view of the aortogram in patient 1 shows an aneurysm of the ductus arteriosus (arrow).