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Pulmonary Stenosis
535
LEADING ARTICLES
systemic-pulmonary anastomosis does nothing to relieve the right ventricle ; in fact it may do harm,
THE LANCET LONDON ::
SATURDAY, SEPT. 11,
1954
Pulmonary Stenosis Much confusion has surrounded the subject of simple pulmonary stenosis. Its description under various different names, such as pure pulmonary stenosis," " isolated pulmonary stenosis," and pulmonary stenosis with closed ventricular septum "’ (not to mention the triad of Fallot ’0) has hardly made things easier for the clinician ; and the physical signs and natural1-6history have been misunderstood. The many series published in the past few years show that it probably comprises 10-12-5% of all cases of congenital heart-disease and the may be either valvular or, more rarely, at the infundibulum. ABRAHAMS and WOOD6 have emphasised that simple pulmonary stenosis is distinguishable from more complicated congenital lesions of which it may be a part by the presence of a normal aortic root and the absence of an overriding aorta. If the aortic root is normal the septa may be quite intact, a patent foramen ovale may be present, or either septum may be defective. If there is a patent foramen ovale or septal defect, together with severe stenosis, a right-to-left shunt is liable to occur, in response to a rising pressure on the right side ; and these severe cases, seen after cyanosis has developed, with Fallot’s triad. Mild pulmonary be confused may stenosis causes no symptoms, and the patient may lead an extremely active life, surviving to old age ; whereas in severe stenosis right-heart failure and rapid deterioration may occur in childhood or early adult life. The recognition by BROCKthat obstruction to the right ventricular outflow without an overriding aorta was the primary cause of deterioration led him to undertake pulmonary valvotomy in severe cases. Though cyanosis may occur with a septal the defect, lungs are not primarily ischsemic and a "
"
’’
and BROCK11 has described cases in which he did valvotomy after an anastomosis had caused deteriora. tion with heart-failure. CAMPBELL9 has now reviewed his experience of 100 patients with simple pulmonary stenosis ; his criteria for diagnosis agree largely with those of ABRAHAMS and Woo o, and of DIMOND and LIN 10 who have analysed their clinical findings in 20 cases proved by cardiac catheterisation. A harsh and often loud systolic murmur is always heard in the pulmonary area ; this is usually clearest in the second left space, and a corresponding thrill is present except in the mildest instances (even in these it will usually be appreciated after exercise). Although a diastolic murmur has been reported-it was heard by DIMOND and LIN in 3 cases-its presence, except a after bacterial endocarditis, is point It now stenosis. is generally against uncomplicated recognised that the second heart sound is normally composed of two elements, due to closure of the aortic and of the pulmonary valves. In pulmonary stenosis the intensity of the second sound may be slightly reduced or quite normal, even in severe cases, because it is the aortic component that is heard ; while in mild or moderate cases the practised ear may detect splitting, with diminution of the second element. Thus a second sound which is single and of normal intensity does not rule out severe stenosis. These auscultatory signs are all that may be found in mild or moderate stenosis. A large presystolic or a wave in the jugular venous pulse indicates a severe lesion, and a distinct rightventricular impulse may also be felt in such cases. Radiographic examination is helpful, for a feature of this condition is post-stenotic dilatation of the pulmonary artery, which is absent only in the rarer infundibular stenosis. Such dilatation affects only the main trunk and its two branches ; the -lungfields themselves never show increased vascularity, and, while pulsation may be seen in the pulmonary trunk, a hilar dance is never observed. Statistically, a right-sided aortic arch is against the diagnosis. The electrocardiogram may vary from normal- to the picture of extreme right-ventricular hypertrophy and strain described by MARQUIS,11 who considered that this might occur in severe cases before any clinical evidence of deterioration, and would then be an indication for surgery. CAMPBELL’s results confirm case for in this ; showing T-wave inversion in every leads V—V, cardiac catheterisation showed a rightventricular pressure of over 100 mm. Hg. The converse was not true, for he observed several cases with right-ventricular pressures above 100 mm. Hg but with no T-wave inversion, though this pattern of hypertrophy would presumably develop sooner CAMPBELL regards this pattern of rightor later. ventricular strain as one of the best indications at present for urgent operation. Whereas patients with mild stenosis survive many years, in severe cases operation should not be long deferred ; clinicians may hesitate unduly before
probably;
(pulmonary)
"
"
stenosis always
1. Adams, F.
2. 3.
4.
5.
6.
7.
H., Veasy, L. G., Jorgens, J., Dichl, R., Labree, J. W., Shapiro, M. J., Divan, P. F. J. Pediat. 1951, 38, 431. Allanby, K. D., Campbell, M. Guy’s Hosp. Rep. 1949, 98, 18. Currens, J. H., Kinney, T. D., White, P. D. Amer. Heart J. 1945, 30, 491. Selzer, A., Noble, C. A., Higgins, W. H., Holmes, R. O. Amer. J. Med. 1949, 6, 3. Dow, J. W., Levine, H. D., Elkin, M., Haynes, F. W., Hellems, H. K., Whittenberger, J. W., Ferris, B. G., Goodale, W. T., Harvey, W. P., Eppinger, E. C., Dexter, L. Circulation, 1950, 1, 267. Abrahams, D. G., Wood, P. Brit. Heart J. 1951, 13, 519. Brock, R. C. Brit. med. J. 1949, ii, 399.
8. Breek, R. C., Campbell, M. Brit. Heart J. 1950, 12, 377. 9. Campbell, M. Ibid, 1954, 16, 273. 10. Dimond, E. G., Lin, T. K. Ann. intern. Med. 1954, 40, 1108. 11. Marquis, R. M. Brit. Heart J. 1951, 13, 89.
536 CAMPBELL recommending intracardiac surgery. suggests that the indications for operation include, in addition to the electrocardiographic pattern, a jugular " a wave, severe symptoms, or much cardiac enlargement. Cyanosis with clubbing of the fingers always means severe stenosis, with rising pressure on the right side causing a shunt, usually through the foramen ovale or an atrial septal defect. It seems that patients with moderate or severe stenosis, but without any of these signs, should be seen regularly and, if there is any hint that their condition is deteriorating, should be examined by cardiac catheterisation. Angiocardiography is unlikely to help, for the experienced operator can "
detect with the catheter whether the stenosis is valvular or infundibular, besides recording pressures in each chamber. CAMPBELL believes that operation should not be be long delayed if the patient’s condition is deteriorating and the right-ventricular pressure is 100 mm. Hg or more, while DIMOND and LiN suggest a figure of 75 mm. Certainly the risk of as the patient becomes operation increases alarmingly 8 The results of pulmonary more severely disabled, valvotomy to be published by CAMPBELL may give further information on these problems.
Diabetic Micro-angiopathy INCREASING success in the treatment of primary diabetic symptoms, and the consequent greater longevity of diabetics, accentuate the importance of work on the mechanism of diabetic complications. These involve mainly the vascular and nervous systems, eyes, and kidneys, and it would be satisfying to find explanations that were common to such varied manifestations. The most obvious common factor is the vascular supply, which might be particularly prone to involvement in certain organs because of special local conditions. In advanced vascular disease1 of diabetics all types of blood-vessel are involved, but careful study indicates that capillary lesions may precede sclerosis in other parts of the vascular system.2 In this sense are the capillary changes primary ; and those tissues in which the capillary bed forms the only vascular component, such as the inner nuclear layer of the retina, the glomeruli of the kidney, and the pancreatic islets of Langerhans, are the very ones particularly prone to degenerative change in diabetes. Detailed study of the capillary changes in diabetics thus promises to increase our understanding of the pathogenesis of the complications of diabetes. In two sites of the living body-the conjunctivas and the nail beds-capillaries can be examined with relative ease under normal conditions, using a corneal microscope which can give a magnification of from 16 to 150. It may be assumed that the conjunctival and nail-bed capillaries fairly represent all the capillaries of the subcutaneous layer, but they may well be less affected by diabetes than those in the particular organs subject to the common complications. DITZEL and SAGILD3 studied the conjunctival vessels of 150 diabetics and 90 normal controls. In the diabetics the capillaries were more numerous and elongated, par1. 2. 3.
Lundbaek, K. Lancet, 1954, i, 377. Ditzel, J. New Engl. J. Med. 1954, 250, 541. Ditzel, J., Sagild, U. Ibid. p. 587.
in their venous parts, forming an irregular network. It was also noted that the blood within them was often in large aggregates which tended seriously to obstruct the rate of flow. The distension of the venules, sometimes to two or more times their normal diameter, contributed further to the sluggish flow. It is said that aggregation of red blood-cells or sludging does not occur to any significant degree in healthy people, although it is common in many diseases 4 ; and such gross distension of the venules was not seen in the 90 controls. Capillary micro-aneurysms, so characteristic of diabetic retinopathy, were no more common in diabetic conjunctivae than in the normals. These studies of the living are reinforced by a formidable number of examinations of dead tissues. 5-8 Thus, the elongation and angular course of capillaries has been noted in diabetic retinze, 58 where in addition BALLANTYNE and LOWENSTEIN9 described the micro-aneurysms in the venous segments of the capillaries. It is from these micro-aneurysms that red cells may escape to produce haemorrhages,1O and their subsequent hyalinisation and fusion produces the typical waxy exudates of diabetic retinopathy. In the glomeruli of the kidney, too, there is proliferation and hyalinisation of the capillary walls." Parts of the capillaries may be dilated,12 and, with the eye of faith, micro-aneurysms may be made out.13 Patients with these capillary changes in the fundi are extremely likely to have them in the kidneys as well, although they probably occur rather later there.l0 Hyalinisation of the islets of Langerhans, a common finding in older diabetics,14 may represent a similar process. There are accounts of the presence 15 16 or 7 10 of such absence changes in other tissues. Besides these morphological changes in the capillaries there is also evidence for abnormal capillary function in diabetes. Some workers 17 18 have reported increased capillary fragility, measured usually by a tourniquet test; and FREHNER 15 noted increased capillary permeability using the method of fluorescein diffusion. The descriptive stage of study of these changes is well advanced, but there are many gaps in our understanding of how and why they occur. Is, for instance, the distension of the venules and capill. aries due to mechanical obstruction or to active GIBSON 19 believes that endothelial dilatation ? proliferation causes the venous distension and that the capillary micro-aneurysms develop later. In this he is supported by BECKER and POST,20 who found capillary changes, including micro-aneurysms, in the retinae of eyes with naturally occurring occlusion of
ticularly
"
"
conflicting
Knisely, M. H., Bloch, E. H., Eliot, T. S., Warner, L. Science, 1947, 106, 431. 5. Friedenwald, J. S. Amer. J. Ophthal. 1949, 32, 487. 6. Ashton, N. Brit. J. Ophthal. 1950, 34, 38. 7. Friedenwald, J. S. Amer. J. Ophthal. 1950, 33, 1187. 8. Ashton, N. Postgrad. med. J. 1950, 26, 391. 9. Ballantyne. A. J., Lowenstein, A. Trans. ophthal. Soc. U.K. 1944, 63, 95. 10. Ashton, N. Brit. J. Ophthal. 1949, 33, 407. 11. Kimmelstiel, P., Wilson, C. Amer. J. Path. 1936, 12, 83. 12. Allen, A. C. Arch. Path. 1941, 32, 33. 13. Friedenwald, J. S. Trans. Amer. Ass. Ophthal. 1948, 53, 73. 14. Opie, E. L. Special Cytology. New York, 1928. 15. Frehner, H. U. Thesis. University of Zürich, 1950. 16. McCulloch, J. C., Pashby, T. J. Brit. J. Ophthal. 1950, 34, 495 17. Hauum, S. Acta ophthal., Kbh. 1939, suppl. 16, p. 3. 18. Barnes, R. H. Amer. J. med. Sci. 1950, 219, 368. 19. Gibson, G. G. See Beetham, W. P. Trans. Amer. Ophthal. Soc. 4.
1951, 48, 205. B., Post, L. T. jun. Amer. J. Ophthal. 1951, 34, 677.
20. Becker,
LEADING ARTICLES
systemic-pulmonary anastomosis does nothing to relieve the right ventricle ; in fact it may do harm,
THE LANCET LONDON ::
SATURDAY, SEPT. 11,
1954
Pulmonary Stenosis Much confusion has surrounded the subject of simple pulmonary stenosis. Its description under various different names, such as pure pulmonary stenosis," " isolated pulmonary stenosis," and pulmonary stenosis with closed ventricular septum "’ (not to mention the triad of Fallot ’0) has hardly made things easier for the clinician ; and the physical signs and natural1-6history have been misunderstood. The many series published in the past few years show that it probably comprises 10-12-5% of all cases of congenital heart-disease and the may be either valvular or, more rarely, at the infundibulum. ABRAHAMS and WOOD6 have emphasised that simple pulmonary stenosis is distinguishable from more complicated congenital lesions of which it may be a part by the presence of a normal aortic root and the absence of an overriding aorta. If the aortic root is normal the septa may be quite intact, a patent foramen ovale may be present, or either septum may be defective. If there is a patent foramen ovale or septal defect, together with severe stenosis, a right-to-left shunt is liable to occur, in response to a rising pressure on the right side ; and these severe cases, seen after cyanosis has developed, with Fallot’s triad. Mild pulmonary be confused may stenosis causes no symptoms, and the patient may lead an extremely active life, surviving to old age ; whereas in severe stenosis right-heart failure and rapid deterioration may occur in childhood or early adult life. The recognition by BROCKthat obstruction to the right ventricular outflow without an overriding aorta was the primary cause of deterioration led him to undertake pulmonary valvotomy in severe cases. Though cyanosis may occur with a septal the defect, lungs are not primarily ischsemic and a "
"
’’
and BROCK11 has described cases in which he did valvotomy after an anastomosis had caused deteriora. tion with heart-failure. CAMPBELL9 has now reviewed his experience of 100 patients with simple pulmonary stenosis ; his criteria for diagnosis agree largely with those of ABRAHAMS and Woo o, and of DIMOND and LIN 10 who have analysed their clinical findings in 20 cases proved by cardiac catheterisation. A harsh and often loud systolic murmur is always heard in the pulmonary area ; this is usually clearest in the second left space, and a corresponding thrill is present except in the mildest instances (even in these it will usually be appreciated after exercise). Although a diastolic murmur has been reported-it was heard by DIMOND and LIN in 3 cases-its presence, except a after bacterial endocarditis, is point It now stenosis. is generally against uncomplicated recognised that the second heart sound is normally composed of two elements, due to closure of the aortic and of the pulmonary valves. In pulmonary stenosis the intensity of the second sound may be slightly reduced or quite normal, even in severe cases, because it is the aortic component that is heard ; while in mild or moderate cases the practised ear may detect splitting, with diminution of the second element. Thus a second sound which is single and of normal intensity does not rule out severe stenosis. These auscultatory signs are all that may be found in mild or moderate stenosis. A large presystolic or a wave in the jugular venous pulse indicates a severe lesion, and a distinct rightventricular impulse may also be felt in such cases. Radiographic examination is helpful, for a feature of this condition is post-stenotic dilatation of the pulmonary artery, which is absent only in the rarer infundibular stenosis. Such dilatation affects only the main trunk and its two branches ; the -lungfields themselves never show increased vascularity, and, while pulsation may be seen in the pulmonary trunk, a hilar dance is never observed. Statistically, a right-sided aortic arch is against the diagnosis. The electrocardiogram may vary from normal- to the picture of extreme right-ventricular hypertrophy and strain described by MARQUIS,11 who considered that this might occur in severe cases before any clinical evidence of deterioration, and would then be an indication for surgery. CAMPBELL’s results confirm case for in this ; showing T-wave inversion in every leads V—V, cardiac catheterisation showed a rightventricular pressure of over 100 mm. Hg. The converse was not true, for he observed several cases with right-ventricular pressures above 100 mm. Hg but with no T-wave inversion, though this pattern of hypertrophy would presumably develop sooner CAMPBELL regards this pattern of rightor later. ventricular strain as one of the best indications at present for urgent operation. Whereas patients with mild stenosis survive many years, in severe cases operation should not be long deferred ; clinicians may hesitate unduly before
probably;
(pulmonary)
"
"
stenosis always
1. Adams, F.
2. 3.
4.
5.
6.
7.
H., Veasy, L. G., Jorgens, J., Dichl, R., Labree, J. W., Shapiro, M. J., Divan, P. F. J. Pediat. 1951, 38, 431. Allanby, K. D., Campbell, M. Guy’s Hosp. Rep. 1949, 98, 18. Currens, J. H., Kinney, T. D., White, P. D. Amer. Heart J. 1945, 30, 491. Selzer, A., Noble, C. A., Higgins, W. H., Holmes, R. O. Amer. J. Med. 1949, 6, 3. Dow, J. W., Levine, H. D., Elkin, M., Haynes, F. W., Hellems, H. K., Whittenberger, J. W., Ferris, B. G., Goodale, W. T., Harvey, W. P., Eppinger, E. C., Dexter, L. Circulation, 1950, 1, 267. Abrahams, D. G., Wood, P. Brit. Heart J. 1951, 13, 519. Brock, R. C. Brit. med. J. 1949, ii, 399.
8. Breek, R. C., Campbell, M. Brit. Heart J. 1950, 12, 377. 9. Campbell, M. Ibid, 1954, 16, 273. 10. Dimond, E. G., Lin, T. K. Ann. intern. Med. 1954, 40, 1108. 11. Marquis, R. M. Brit. Heart J. 1951, 13, 89.
536 CAMPBELL recommending intracardiac surgery. suggests that the indications for operation include, in addition to the electrocardiographic pattern, a jugular " a wave, severe symptoms, or much cardiac enlargement. Cyanosis with clubbing of the fingers always means severe stenosis, with rising pressure on the right side causing a shunt, usually through the foramen ovale or an atrial septal defect. It seems that patients with moderate or severe stenosis, but without any of these signs, should be seen regularly and, if there is any hint that their condition is deteriorating, should be examined by cardiac catheterisation. Angiocardiography is unlikely to help, for the experienced operator can "
detect with the catheter whether the stenosis is valvular or infundibular, besides recording pressures in each chamber. CAMPBELL believes that operation should not be be long delayed if the patient’s condition is deteriorating and the right-ventricular pressure is 100 mm. Hg or more, while DIMOND and LiN suggest a figure of 75 mm. Certainly the risk of as the patient becomes operation increases alarmingly 8 The results of pulmonary more severely disabled, valvotomy to be published by CAMPBELL may give further information on these problems.
Diabetic Micro-angiopathy INCREASING success in the treatment of primary diabetic symptoms, and the consequent greater longevity of diabetics, accentuate the importance of work on the mechanism of diabetic complications. These involve mainly the vascular and nervous systems, eyes, and kidneys, and it would be satisfying to find explanations that were common to such varied manifestations. The most obvious common factor is the vascular supply, which might be particularly prone to involvement in certain organs because of special local conditions. In advanced vascular disease1 of diabetics all types of blood-vessel are involved, but careful study indicates that capillary lesions may precede sclerosis in other parts of the vascular system.2 In this sense are the capillary changes primary ; and those tissues in which the capillary bed forms the only vascular component, such as the inner nuclear layer of the retina, the glomeruli of the kidney, and the pancreatic islets of Langerhans, are the very ones particularly prone to degenerative change in diabetes. Detailed study of the capillary changes in diabetics thus promises to increase our understanding of the pathogenesis of the complications of diabetes. In two sites of the living body-the conjunctivas and the nail beds-capillaries can be examined with relative ease under normal conditions, using a corneal microscope which can give a magnification of from 16 to 150. It may be assumed that the conjunctival and nail-bed capillaries fairly represent all the capillaries of the subcutaneous layer, but they may well be less affected by diabetes than those in the particular organs subject to the common complications. DITZEL and SAGILD3 studied the conjunctival vessels of 150 diabetics and 90 normal controls. In the diabetics the capillaries were more numerous and elongated, par1. 2. 3.
Lundbaek, K. Lancet, 1954, i, 377. Ditzel, J. New Engl. J. Med. 1954, 250, 541. Ditzel, J., Sagild, U. Ibid. p. 587.
in their venous parts, forming an irregular network. It was also noted that the blood within them was often in large aggregates which tended seriously to obstruct the rate of flow. The distension of the venules, sometimes to two or more times their normal diameter, contributed further to the sluggish flow. It is said that aggregation of red blood-cells or sludging does not occur to any significant degree in healthy people, although it is common in many diseases 4 ; and such gross distension of the venules was not seen in the 90 controls. Capillary micro-aneurysms, so characteristic of diabetic retinopathy, were no more common in diabetic conjunctivae than in the normals. These studies of the living are reinforced by a formidable number of examinations of dead tissues. 5-8 Thus, the elongation and angular course of capillaries has been noted in diabetic retinze, 58 where in addition BALLANTYNE and LOWENSTEIN9 described the micro-aneurysms in the venous segments of the capillaries. It is from these micro-aneurysms that red cells may escape to produce haemorrhages,1O and their subsequent hyalinisation and fusion produces the typical waxy exudates of diabetic retinopathy. In the glomeruli of the kidney, too, there is proliferation and hyalinisation of the capillary walls." Parts of the capillaries may be dilated,12 and, with the eye of faith, micro-aneurysms may be made out.13 Patients with these capillary changes in the fundi are extremely likely to have them in the kidneys as well, although they probably occur rather later there.l0 Hyalinisation of the islets of Langerhans, a common finding in older diabetics,14 may represent a similar process. There are accounts of the presence 15 16 or 7 10 of such absence changes in other tissues. Besides these morphological changes in the capillaries there is also evidence for abnormal capillary function in diabetes. Some workers 17 18 have reported increased capillary fragility, measured usually by a tourniquet test; and FREHNER 15 noted increased capillary permeability using the method of fluorescein diffusion. The descriptive stage of study of these changes is well advanced, but there are many gaps in our understanding of how and why they occur. Is, for instance, the distension of the venules and capill. aries due to mechanical obstruction or to active GIBSON 19 believes that endothelial dilatation ? proliferation causes the venous distension and that the capillary micro-aneurysms develop later. In this he is supported by BECKER and POST,20 who found capillary changes, including micro-aneurysms, in the retinae of eyes with naturally occurring occlusion of
ticularly
"
"
conflicting
Knisely, M. H., Bloch, E. H., Eliot, T. S., Warner, L. Science, 1947, 106, 431. 5. Friedenwald, J. S. Amer. J. Ophthal. 1949, 32, 487. 6. Ashton, N. Brit. J. Ophthal. 1950, 34, 38. 7. Friedenwald, J. S. Amer. J. Ophthal. 1950, 33, 1187. 8. Ashton, N. Postgrad. med. J. 1950, 26, 391. 9. Ballantyne. A. J., Lowenstein, A. Trans. ophthal. Soc. U.K. 1944, 63, 95. 10. Ashton, N. Brit. J. Ophthal. 1949, 33, 407. 11. Kimmelstiel, P., Wilson, C. Amer. J. Path. 1936, 12, 83. 12. Allen, A. C. Arch. Path. 1941, 32, 33. 13. Friedenwald, J. S. Trans. Amer. Ass. Ophthal. 1948, 53, 73. 14. Opie, E. L. Special Cytology. New York, 1928. 15. Frehner, H. U. Thesis. University of Zürich, 1950. 16. McCulloch, J. C., Pashby, T. J. Brit. J. Ophthal. 1950, 34, 495 17. Hauum, S. Acta ophthal., Kbh. 1939, suppl. 16, p. 3. 18. Barnes, R. H. Amer. J. med. Sci. 1950, 219, 368. 19. Gibson, G. G. See Beetham, W. P. Trans. Amer. Ophthal. Soc. 4.
1951, 48, 205. B., Post, L. T. jun. Amer. J. Ophthal. 1951, 34, 677.
20. Becker,