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Kinking of the aorta (Pseudocoarctation) with coarctation
Kinking RAMON E. ACEVEDO,
of the Aorta (Pseudocoarctation) with Coarctation* M.D.,? OTTO G. THILENIUS, M.D., PH.D., PETER V. MOULDER, and DONALD E. CASSELS, M.D., F.A.C.C. Chicago,
SEUDOCOARCTATION OF THE descending thoracic aorta is an uncommon anomaly, first described in 1951 by Dotter and Steinberg,’ Souders et at.2 and Robb.3 It was termed pseudocoarctation’ because of certain roentgenologic similarities with simple coarctation of the aorta. Subsequently, other descriptive terms, such as kinking4+ or bucklings*10 of the aorta, have been used. There is general agreement that this anomaly differs from coarctation of the aorta by virtue of absence of a pressure gradient across the kinked segment and lack of collateral circulation. Pseudocoarctation as an isolated lesion has been described in five inIn association with stances in children.“J2 other congenital anomalies, particularly valvular aortic stenosis,13 3 pediatric cases have been reported, 10 years being the youngest documented age. l3 There is no sex prevalence in the reported cases. In contrast, adult type (postductal) coarctation of the aorta (without associated patent ductus arteriosus) is a rather common lesion. It is a localized narrowing of the aorta in the region of the ligamentum arteriosum, not infrequently associated with some degree of hypoplasia of the aortic segment proximal to the region of most pronounced constriction. It is not associated with significant elongation and kinking of the proximal segment. This paper reports the case of a 7 year old boy with both elongation and kinking of the descending thoracic aorta as well as true coarctation and points out that these two anomalies may represent variants of a similar developmental disturbance.
M.D., F.A.C.C.
Illinois
CASE REPORT
P
A 7 year old white boy was admitted to the Bobs Roberts Memorial Hospital for Children in May 1966 for evaluation of a heart murmur. His prenatal and birth histories were unremarkable. He was noted to have a heart murmur that disappeared shortly after birth. Symptoms such as fatigue, diminished exercise tolerance, or leg pain were never noted. Five to six months before admission, the family doctor again noted a heart murmur. Physical examination showed a well developed child. The brachial pulses were full and equal. The femoral pulses were decreased in amplitude and seemed delayed. The blood pressure readings were: right arm, 105/80; left arm, 110/80; right leg, 90/65; left leg, 85/65 mm. Hg. The jugular venous pulse was normal. The cardiac impulse was left ventricular and normal in character. A thrill was not palpated. The first heart sound was normal; the second heart sound was moderately split, the aortic component being slightly louder than the pulmonic component. Neither a third nor a fourth heart sound nor an ejection click was heard. There was a grade 2/6 systolic ejection murmur, best heard at the third left intercostal space and over the suprasternal notch, radiating to the upper back. A diastolic murmur was not heard. The phonocardiogram did not show additional information beyond the auscultatory findings described. Routine laboratory studies were normal. The chest roentgenogram (posteroanterior view, Fig. 1) showed some widening and elevation of the aortic
arch.
The cardiac
silhouette
and pulmonary
vas-
cular markings were within normal limits, Rib notching was not observed. Right and Left Heart Catheterization (Table 7) : Blood oxygen studies showed no evidence of shunts. Cardiac output and pulmonary and systemic resistance values were normal. There were no gradients across the aor-
* From the Departments of Medicine, Surgery and Pediatrics, supported in part by grants from the National Heart Institute, t Postdoctoral Trainee of the National Heart Institute. Address for reprints: Otto G. Thilenius, M.D., Children’s Chicago, Ill. 60614. 442
University of Chicago, Chicago, Ill. National Institutes of Health, Bethesda, Memorial
Hospital,
THE
AMERICAN
2300
N.
JOURNAL
This study Md.
Children’s
was
Plaza,
OF CARDIOLOGY
Kinking
1. Frontal chest roentgenogram and elevation of the aortic arch. demonstrated. FIG.
of the Aorta
shows both widening Rib notching is not
tic, mitral, pulmonic, or tricuspid valve. However, a significant pressure difference was found between the femoral and right brachial arteries. The cineangiocardiogram with injection into the root of the aorta (Fig. 2) showed elongation and kinking of the proximal descending thoracic aorta and a moderate degree of poststenotic dilatation. Evidence of increased collateral circulation was absent. Operative Findings and Procedure: On July 19, 1966, the child underwent surgery. The proximal part of the descending aorta was elongated and kinked in an ‘.S”-shaped figure. The ligamenturn arteriosum was attached to the lower convexity of this “S.” There was a mild dilatation of the aorta just beyond the isthmus. After the kinked segment was dissected free of all adjacent tissue, including division of the ligamentum arteriosum, a systolic pressure gradient of only 3 to 4 mm. Hg remained across this segment; the mean pressures were identical (Fig. 3). However, the pressure pulse of the distal aorta showed delay of the anacrotic limb. After resection of this segment and end-to-end anastomosis, the delay in the
Pertinent
Cardiac (May
TABLE I Catheterization 18, 1966)
Data
0 xYFn
Pulmonarv artew Right brachial &cry Right femoral artery Left ventricle Femoral artery Left ventricle Aorta (ascending) ED = end-diastolic. Figures in parentheses VOLUME
21.
MARCH
Pressure (mm. Hg)
Oxygen Saturation
COlttent (vol. %)
21/13 (17) 118/64 (85) 89/62 (75) llO/... 96/69 110/o-11 ED 116/72
73.7% 96.0% 95.5%
10.91 14.27 14.17
= mean pressure.
1968
:::
with
343
Coarctation
inscription of the anacrotic limb of the distal aortic pulse was no longer observed (Fig. 3). The child recovered uneventfully and had identical blood prrssure in arms and legs thereafter. Surgical Specimen: The resected segment of aorta was “S”-shaped and 2.5 cm. long. The proximal end was 1 cm. in diameter, and the distal end 1.2 cm. .4t the midportion, the outside diameter was constricted to 0.5 cm. At the outer curvature near one end of the specimen was the patent orifice of the ductus arteriosus, 0.1 cm. in diameter. The intimal surface of the aorta was smooth, but at its constricted midpoint a spur or fold of aortic wall protruded into the lumen. Microscopically, the spur consisted of a localized thickening of the aortic wall media with a dense network of elastic fibers, but without fibrosis or significant alteration of the intima. The adjacent parts of the aortic wall had a normal histologic appearance. DISCUSSION
In this paper, the term “coarctation” (of the aorta) refers to a simple, localized narrowing of the aorta without associated cardiovascular lesions, in particular without an associated patent ductus arteriosus or hypoplasia of the aortic arch. Pseudocoarctation, on the other hand, is an elongation and kinking of the descending thoracic aorta without true narrowing. By definition, the differentiation between these anomalies rests upon the presence of a pressure gradient and increased collateral circulation in coarctation and their absence in pseudocoarctation. Furthermore, coarctation is not accompanied by a significant degree of elongation of the preductal portion of the aorta, but pseudocoarctation characteristically has marked elongation and kinking of this segment. Both anomalies may have poststenotic dilation of the thoracic aorta. Although pseudocoarctation is a roentgenographic diagnosis, not accompanied by clinical symptoms in childhood, coarctation has the well known clinical profile. The patient reported in this paper had features of both coarctation and pseudocoarctation. The typical difference in blood pressure between arms (110/80 mm. Hg) and legs (90/65 mm. Hg), the catheterization data and, finally, the surgical specimen were diagnostic of coarctation. The angiogram, however, showed the marked elongation and folding of the thoracic aorta typical of pseudocoarctation. This “S”shaped folding appeared even more pronounced when seen in situ during the thoracotomy. The pressure gradient across the abnormal aortic segment disappeared after the tortuous segment was freed of its surrounding tissue and was thus
Acevedo et al.
F‘rc. 2. Relrograde thorocic aortogrom in right posterior ohltpe projection demonstrating elongation of the aorta with a kinked segment in the proximal descending aorta. There is no evidence of collateral circulation.
allowed to expand to some degree. This finding appears to prove that the pseudocoarctation component of this child contributed to his clinical picture of coarctation. Of course, the delayed upstroke of aortic pulse distal to the coarctation disappeared only after resection, a finding typical for coarctation. Mild narrowing of the aortic isthmus as seen in this child does not cause a pressure gradient but may be only an incidental roentgenologic diagnosis. The pres-
BEFORE
mm.Hg
FIG. 3. Pressure tracings of the aorta proximal and distal to the narrowed segment. The tracings were recorded at the time of operation (after complete dissection of the kinked portion of the aorta) immediately before and after resectioll of the coarctation.
ence of a tight kink, however, altered the blood flow across the coarcted segment to such an estent that the constriction becalne clinicall) apparent. With one exception, a 31 year old patient’” with pseudocoarctation without pressure gradient, who upon autopsy also had a mild coarctation, the association of the two anomalies has not been reported. Nasser and HelmenlZ recently reported on 2 children with pseudocoarctation with systolic pressure gradients of 10 and 20 mm. Hg, respectively; however, associated coarctation was not invoked as the cause for this pressure differential. The observation that the two lesions may occur together raises again the question whether they are pathogenetically related. Bruwer and Burchells do not see such a relation but mention an unusually short ligamenturn arteriosmn or an unusually long aortic arch as possible causes for pseudocoarctation. Steinberg and Hagstrom” believe that the two lesions are related and that they represent different results of uneven growth of the aortic wall. Barry’s discussion15 of the embryology of the aortic arch derivatives is of great interest in this respect. During the early development of the fetus, the left subclavian artery (seventh dorsal intersegmental artery) originates from the aorta below the ductus arteriosus; the latter joins the left dorsal aortic root approximately at the level of the fourth segment. The subsequent caudal migration of the heart and the increase in size of the lesser and greater curvature of the aortic arch arc associated with an uneven growth of the third to tenth segments of the left dorsal aortic root. Thus, in the newborn infant, the left subclavian artery arises at or slightly above the aortic AFTER I
PRYX’MAL looq$J?+hy-
PROXIMAL
DISTAL
0 PHASIO
MEAN
PHASIC
I THE
AMERICAN
JOURNAL
MEAN OF CARDIOLOGY
Kinking
of the Aorta with Coarctation
In our opinion, nlouth of the ductus arteriosus. a disturbance in the degree of this longitudinal ,yrowth differential in the region of the third to tenth segments of the dorsal aortic root could lead to an excessive elongation of the aorta, thus resulting in the typical “S”-shaped deforInation of pseudocoarctation. By contrast, postductal coarctation has a decreased circumference at the level of the eighth segment (entrance of the ductus arteriosus). The etiology of this decrease in circumference is unknown. The hypotheses relating it to a short ligamenturn arteriosum or a retarded circumferential growth, or to the normal involution of the right eighth segment represent attempts to explain the given fact of a decreased vessel diameter. Although a pathogenetic relation between pseudocoarctation and coarctation appears likely, it still remains to be proved. Pseudocoarctation does not require medical therapy or limitation of physical exercise, certainly not in children. Whether surgical excision of the elongated aortic segment is indicated is not clear. Development of atherosclerotic changes may be favored by eddies that presumably exist in the kinked region of this abnormally shaped aorta, but this may not have clinical significance. The patient reported on here was advised to undergo surgery because the associated coarctation, although mild in itself, was thought to induce further hemodynamic changes in, and close to, the narrowed segment which possibly would increase obstruction at a later age. Surgery thus has a preventive character, and no general guidelines can be stated. SUMMARY
Pseudocoarctation and coarctation of the aorta in a 7 year old boy are reported. This association is very unusual, particularly in a young child. A possible pathogenetic relation between these two anomalies is discussed.
VOLUME
21, MARCH
1968
44.i
hZKNOWI,EDGMENl
We are grateful to Dr. Klaus Ranniger for the anqiographic study and to Dr. Francis L. .&her for tht, dcscription of the surgical specimen. REFERENCES 1. DOTTER, C. and STEINBERG, I. ;\ngiocardiography. p. 181. New York, 1951. Paul B. Hoeber. 2. SOUDERS,C. R., PEARSON,C.M. and ADAMS, H.D. An aortic deformity simulating mediastinal tumor: A subclinical form of coarctation. I)i.r. UIPS~. 20: 35, 1951. 3. ROBB, G. P. An Atlas of Angiocardiography Prepared for The American Registry of Pathology, .4rmed Forces Institute of Pathology (American Registry of Pathology, National Research Council), Washington, D. C., 1951. 4. DI GUGLIELMO, L. and GATTADAURO, M. Kinking of the aorta: Report of two cases. Acta radial., 44: 121, 1955. 5. BRUWER, A. J. and BURCHELL, H. B. Kinking of aortic arch (pseudocoarctation, subclinical coarctation). J.A.M.A., 162: 1445, 1956. 6. VAUGHAN, B. F. Kinking of aortic arch. &it. J. Radial., 29: 516, 1956. 7. PATTINSON, J. N. and GRAINGER, 1~. G. Congenital kinking of the aortic arch. &it. Heart J., 21: 555, 1959. 8. SHAW, V. V., SHAH, K. D., DASTUR, K. N. and SHAH, R. L. Kinking of aortic arch with aneurysma1 dilatation. Dir. Chest, 50: 101, 1966. 9. STEVENS, G. M. Buckling of the aortic arch (pseudocoarctation, kinking) : A roentgenographic entity. Radiology, 70: 67, 1957. 10. STEINBERG, I. and HAGSTROM, J. W. C. Congenital aortic valvular stenosis and pseudocoarctation (“kinking, buckling”) of the arch of the aorta. Circulation, 25: 545, 1962. 11. GUDBJERC, C. E. and PETERSON, 0. Coarctation of the aorta: Relation between roentgenographic and hemodynamic findings. Radiology, 75 : 399, 1960. 12. NASSER, W. K. and HELMEN, C. Kinking of the aortic arch (pseudocoarctation). Ann. Int. Med., 64: 971, 1966. 13. STEINBERG, I. Anomalies (pseudocoarctation) of the arch of the aorta. Am. J. Roentgenol., 88: 73, 1962. 14. EDMUNDS, L. H., JR., MCCLENATHAN, J. E. and HUFNAGEL, C. A. Subclinical coarctation of the aorta. Ann. Sup., 156: 180, 1962. 15. BARRY, A. The aortic arch derivatives in the human adult. Amt. Rec., 111: 221, 1951.
of the Aorta (Pseudocoarctation) with Coarctation* M.D.,? OTTO G. THILENIUS, M.D., PH.D., PETER V. MOULDER, and DONALD E. CASSELS, M.D., F.A.C.C. Chicago,
SEUDOCOARCTATION OF THE descending thoracic aorta is an uncommon anomaly, first described in 1951 by Dotter and Steinberg,’ Souders et at.2 and Robb.3 It was termed pseudocoarctation’ because of certain roentgenologic similarities with simple coarctation of the aorta. Subsequently, other descriptive terms, such as kinking4+ or bucklings*10 of the aorta, have been used. There is general agreement that this anomaly differs from coarctation of the aorta by virtue of absence of a pressure gradient across the kinked segment and lack of collateral circulation. Pseudocoarctation as an isolated lesion has been described in five inIn association with stances in children.“J2 other congenital anomalies, particularly valvular aortic stenosis,13 3 pediatric cases have been reported, 10 years being the youngest documented age. l3 There is no sex prevalence in the reported cases. In contrast, adult type (postductal) coarctation of the aorta (without associated patent ductus arteriosus) is a rather common lesion. It is a localized narrowing of the aorta in the region of the ligamentum arteriosum, not infrequently associated with some degree of hypoplasia of the aortic segment proximal to the region of most pronounced constriction. It is not associated with significant elongation and kinking of the proximal segment. This paper reports the case of a 7 year old boy with both elongation and kinking of the descending thoracic aorta as well as true coarctation and points out that these two anomalies may represent variants of a similar developmental disturbance.
M.D., F.A.C.C.
Illinois
CASE REPORT
P
A 7 year old white boy was admitted to the Bobs Roberts Memorial Hospital for Children in May 1966 for evaluation of a heart murmur. His prenatal and birth histories were unremarkable. He was noted to have a heart murmur that disappeared shortly after birth. Symptoms such as fatigue, diminished exercise tolerance, or leg pain were never noted. Five to six months before admission, the family doctor again noted a heart murmur. Physical examination showed a well developed child. The brachial pulses were full and equal. The femoral pulses were decreased in amplitude and seemed delayed. The blood pressure readings were: right arm, 105/80; left arm, 110/80; right leg, 90/65; left leg, 85/65 mm. Hg. The jugular venous pulse was normal. The cardiac impulse was left ventricular and normal in character. A thrill was not palpated. The first heart sound was normal; the second heart sound was moderately split, the aortic component being slightly louder than the pulmonic component. Neither a third nor a fourth heart sound nor an ejection click was heard. There was a grade 2/6 systolic ejection murmur, best heard at the third left intercostal space and over the suprasternal notch, radiating to the upper back. A diastolic murmur was not heard. The phonocardiogram did not show additional information beyond the auscultatory findings described. Routine laboratory studies were normal. The chest roentgenogram (posteroanterior view, Fig. 1) showed some widening and elevation of the aortic
arch.
The cardiac
silhouette
and pulmonary
vas-
cular markings were within normal limits, Rib notching was not observed. Right and Left Heart Catheterization (Table 7) : Blood oxygen studies showed no evidence of shunts. Cardiac output and pulmonary and systemic resistance values were normal. There were no gradients across the aor-
* From the Departments of Medicine, Surgery and Pediatrics, supported in part by grants from the National Heart Institute, t Postdoctoral Trainee of the National Heart Institute. Address for reprints: Otto G. Thilenius, M.D., Children’s Chicago, Ill. 60614. 442
University of Chicago, Chicago, Ill. National Institutes of Health, Bethesda, Memorial
Hospital,
THE
AMERICAN
2300
N.
JOURNAL
This study Md.
Children’s
was
Plaza,
OF CARDIOLOGY
Kinking
1. Frontal chest roentgenogram and elevation of the aortic arch. demonstrated. FIG.
of the Aorta
shows both widening Rib notching is not
tic, mitral, pulmonic, or tricuspid valve. However, a significant pressure difference was found between the femoral and right brachial arteries. The cineangiocardiogram with injection into the root of the aorta (Fig. 2) showed elongation and kinking of the proximal descending thoracic aorta and a moderate degree of poststenotic dilatation. Evidence of increased collateral circulation was absent. Operative Findings and Procedure: On July 19, 1966, the child underwent surgery. The proximal part of the descending aorta was elongated and kinked in an ‘.S”-shaped figure. The ligamenturn arteriosum was attached to the lower convexity of this “S.” There was a mild dilatation of the aorta just beyond the isthmus. After the kinked segment was dissected free of all adjacent tissue, including division of the ligamentum arteriosum, a systolic pressure gradient of only 3 to 4 mm. Hg remained across this segment; the mean pressures were identical (Fig. 3). However, the pressure pulse of the distal aorta showed delay of the anacrotic limb. After resection of this segment and end-to-end anastomosis, the delay in the
Pertinent
Cardiac (May
TABLE I Catheterization 18, 1966)
Data
0 xYFn
Pulmonarv artew Right brachial &cry Right femoral artery Left ventricle Femoral artery Left ventricle Aorta (ascending) ED = end-diastolic. Figures in parentheses VOLUME
21.
MARCH
Pressure (mm. Hg)
Oxygen Saturation
COlttent (vol. %)
21/13 (17) 118/64 (85) 89/62 (75) llO/... 96/69 110/o-11 ED 116/72
73.7% 96.0% 95.5%
10.91 14.27 14.17
= mean pressure.
1968
:::
with
343
Coarctation
inscription of the anacrotic limb of the distal aortic pulse was no longer observed (Fig. 3). The child recovered uneventfully and had identical blood prrssure in arms and legs thereafter. Surgical Specimen: The resected segment of aorta was “S”-shaped and 2.5 cm. long. The proximal end was 1 cm. in diameter, and the distal end 1.2 cm. .4t the midportion, the outside diameter was constricted to 0.5 cm. At the outer curvature near one end of the specimen was the patent orifice of the ductus arteriosus, 0.1 cm. in diameter. The intimal surface of the aorta was smooth, but at its constricted midpoint a spur or fold of aortic wall protruded into the lumen. Microscopically, the spur consisted of a localized thickening of the aortic wall media with a dense network of elastic fibers, but without fibrosis or significant alteration of the intima. The adjacent parts of the aortic wall had a normal histologic appearance. DISCUSSION
In this paper, the term “coarctation” (of the aorta) refers to a simple, localized narrowing of the aorta without associated cardiovascular lesions, in particular without an associated patent ductus arteriosus or hypoplasia of the aortic arch. Pseudocoarctation, on the other hand, is an elongation and kinking of the descending thoracic aorta without true narrowing. By definition, the differentiation between these anomalies rests upon the presence of a pressure gradient and increased collateral circulation in coarctation and their absence in pseudocoarctation. Furthermore, coarctation is not accompanied by a significant degree of elongation of the preductal portion of the aorta, but pseudocoarctation characteristically has marked elongation and kinking of this segment. Both anomalies may have poststenotic dilation of the thoracic aorta. Although pseudocoarctation is a roentgenographic diagnosis, not accompanied by clinical symptoms in childhood, coarctation has the well known clinical profile. The patient reported in this paper had features of both coarctation and pseudocoarctation. The typical difference in blood pressure between arms (110/80 mm. Hg) and legs (90/65 mm. Hg), the catheterization data and, finally, the surgical specimen were diagnostic of coarctation. The angiogram, however, showed the marked elongation and folding of the thoracic aorta typical of pseudocoarctation. This “S”shaped folding appeared even more pronounced when seen in situ during the thoracotomy. The pressure gradient across the abnormal aortic segment disappeared after the tortuous segment was freed of its surrounding tissue and was thus
Acevedo et al.
F‘rc. 2. Relrograde thorocic aortogrom in right posterior ohltpe projection demonstrating elongation of the aorta with a kinked segment in the proximal descending aorta. There is no evidence of collateral circulation.
allowed to expand to some degree. This finding appears to prove that the pseudocoarctation component of this child contributed to his clinical picture of coarctation. Of course, the delayed upstroke of aortic pulse distal to the coarctation disappeared only after resection, a finding typical for coarctation. Mild narrowing of the aortic isthmus as seen in this child does not cause a pressure gradient but may be only an incidental roentgenologic diagnosis. The pres-
BEFORE
mm.Hg
FIG. 3. Pressure tracings of the aorta proximal and distal to the narrowed segment. The tracings were recorded at the time of operation (after complete dissection of the kinked portion of the aorta) immediately before and after resectioll of the coarctation.
ence of a tight kink, however, altered the blood flow across the coarcted segment to such an estent that the constriction becalne clinicall) apparent. With one exception, a 31 year old patient’” with pseudocoarctation without pressure gradient, who upon autopsy also had a mild coarctation, the association of the two anomalies has not been reported. Nasser and HelmenlZ recently reported on 2 children with pseudocoarctation with systolic pressure gradients of 10 and 20 mm. Hg, respectively; however, associated coarctation was not invoked as the cause for this pressure differential. The observation that the two lesions may occur together raises again the question whether they are pathogenetically related. Bruwer and Burchells do not see such a relation but mention an unusually short ligamenturn arteriosmn or an unusually long aortic arch as possible causes for pseudocoarctation. Steinberg and Hagstrom” believe that the two lesions are related and that they represent different results of uneven growth of the aortic wall. Barry’s discussion15 of the embryology of the aortic arch derivatives is of great interest in this respect. During the early development of the fetus, the left subclavian artery (seventh dorsal intersegmental artery) originates from the aorta below the ductus arteriosus; the latter joins the left dorsal aortic root approximately at the level of the fourth segment. The subsequent caudal migration of the heart and the increase in size of the lesser and greater curvature of the aortic arch arc associated with an uneven growth of the third to tenth segments of the left dorsal aortic root. Thus, in the newborn infant, the left subclavian artery arises at or slightly above the aortic AFTER I
PRYX’MAL looq$J?+hy-
PROXIMAL
DISTAL
0 PHASIO
MEAN
PHASIC
I THE
AMERICAN
JOURNAL
MEAN OF CARDIOLOGY
Kinking
of the Aorta with Coarctation
In our opinion, nlouth of the ductus arteriosus. a disturbance in the degree of this longitudinal ,yrowth differential in the region of the third to tenth segments of the dorsal aortic root could lead to an excessive elongation of the aorta, thus resulting in the typical “S”-shaped deforInation of pseudocoarctation. By contrast, postductal coarctation has a decreased circumference at the level of the eighth segment (entrance of the ductus arteriosus). The etiology of this decrease in circumference is unknown. The hypotheses relating it to a short ligamenturn arteriosum or a retarded circumferential growth, or to the normal involution of the right eighth segment represent attempts to explain the given fact of a decreased vessel diameter. Although a pathogenetic relation between pseudocoarctation and coarctation appears likely, it still remains to be proved. Pseudocoarctation does not require medical therapy or limitation of physical exercise, certainly not in children. Whether surgical excision of the elongated aortic segment is indicated is not clear. Development of atherosclerotic changes may be favored by eddies that presumably exist in the kinked region of this abnormally shaped aorta, but this may not have clinical significance. The patient reported on here was advised to undergo surgery because the associated coarctation, although mild in itself, was thought to induce further hemodynamic changes in, and close to, the narrowed segment which possibly would increase obstruction at a later age. Surgery thus has a preventive character, and no general guidelines can be stated. SUMMARY
Pseudocoarctation and coarctation of the aorta in a 7 year old boy are reported. This association is very unusual, particularly in a young child. A possible pathogenetic relation between these two anomalies is discussed.
VOLUME
21, MARCH
1968
44.i
hZKNOWI,EDGMENl
We are grateful to Dr. Klaus Ranniger for the anqiographic study and to Dr. Francis L. .&her for tht, dcscription of the surgical specimen. REFERENCES 1. DOTTER, C. and STEINBERG, I. ;\ngiocardiography. p. 181. New York, 1951. Paul B. Hoeber. 2. SOUDERS,C. R., PEARSON,C.M. and ADAMS, H.D. An aortic deformity simulating mediastinal tumor: A subclinical form of coarctation. I)i.r. UIPS~. 20: 35, 1951. 3. ROBB, G. P. An Atlas of Angiocardiography Prepared for The American Registry of Pathology, .4rmed Forces Institute of Pathology (American Registry of Pathology, National Research Council), Washington, D. C., 1951. 4. DI GUGLIELMO, L. and GATTADAURO, M. Kinking of the aorta: Report of two cases. Acta radial., 44: 121, 1955. 5. BRUWER, A. J. and BURCHELL, H. B. Kinking of aortic arch (pseudocoarctation, subclinical coarctation). J.A.M.A., 162: 1445, 1956. 6. VAUGHAN, B. F. Kinking of aortic arch. &it. J. Radial., 29: 516, 1956. 7. PATTINSON, J. N. and GRAINGER, 1~. G. Congenital kinking of the aortic arch. &it. Heart J., 21: 555, 1959. 8. SHAW, V. V., SHAH, K. D., DASTUR, K. N. and SHAH, R. L. Kinking of aortic arch with aneurysma1 dilatation. Dir. Chest, 50: 101, 1966. 9. STEVENS, G. M. Buckling of the aortic arch (pseudocoarctation, kinking) : A roentgenographic entity. Radiology, 70: 67, 1957. 10. STEINBERG, I. and HAGSTROM, J. W. C. Congenital aortic valvular stenosis and pseudocoarctation (“kinking, buckling”) of the arch of the aorta. Circulation, 25: 545, 1962. 11. GUDBJERC, C. E. and PETERSON, 0. Coarctation of the aorta: Relation between roentgenographic and hemodynamic findings. Radiology, 75 : 399, 1960. 12. NASSER, W. K. and HELMEN, C. Kinking of the aortic arch (pseudocoarctation). Ann. Int. Med., 64: 971, 1966. 13. STEINBERG, I. Anomalies (pseudocoarctation) of the arch of the aorta. Am. J. Roentgenol., 88: 73, 1962. 14. EDMUNDS, L. H., JR., MCCLENATHAN, J. E. and HUFNAGEL, C. A. Subclinical coarctation of the aorta. Ann. Sup., 156: 180, 1962. 15. BARRY, A. The aortic arch derivatives in the human adult. Amt. Rec., 111: 221, 1951.