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Ductus Arteriosus Aneurysm in Infancy
CASE REPORTS
Ductus Arteriosus Aneurysm in Infancy Jerry M. Jesseph, M.D., Ph.D., Lynn Mahony, M.D., Donald A. Girod, M.D., and John W. Brown, M.D. ABSTRACT An infant with symptoms of congestive heart failure at 6 months of age was found by cardiac catheterization at the age of 1 year to have a fusiform aneurysm of the ductus arteriosus as well as discrete stenosis of the left pulmonary artery and a secundum atrial septal defect. The aneurysm was resected, and a pericardial patch angioplasty corrected the peripheral pulmonary stenosis. The atrial septal defect was closed in a separate procedure. To our knowledge, this case is one of four ductal aneurysms reported in infants that have been diagnosed before operation and successfully treated. The pathophysiology and literature are reviewed. Aneurysmal dilatation of the ductus arteriosus is a rare condition found in both infants and adults[l]. This lesion has seldom been diagnosed during life because of its clinical manifestations and the mortality due to rupture, thrombosis, embolism, and infection as well as associated lesions (21. Therefore, most authors stress early diagnosis and surgical repair. A 1-year-old female infant had been found to have a heart murmur at birth. There was no history of maternal rubella. Her growth and development were essentially normal until she was 6 months old, when clinical and radiographic signs of congestive heart failure developed. Treatment with digoxin provided good relief of symptoms. At 10 months of age the patient underwent cardiac catheterization, which demonstrated a left-to-right shunt at the atrial level. The pulmonary-systemic flow ratio was 2.62:1, and there was a peak systolic pressure difference of 40 mm Hg between the right ventricle and the right pulmonary artery. It was not possible to enter the left pulmonary artery. Cineangiograms demonstrated aneurysmal dilatation of a widely patent ductus arteriosus (Fig 1)and marked stenosis at the origin of the left pulmonary artery (Fig 2) as well as mild branch stenosis of the right pulmonary artery. In addition, a moderate-sized secundum atrial septal defect was seen. The patient was taken to the operating room. Through a left-sided thoracotomy, a fusiform aneurysm of a patent ductus arteriosus measuring 1.5 cm in diameter and containing no thrombus was resected and the aortic and pulmonary stumps were oversewn. At the same time, a From the Departments of Surgery and Pediatrics, Indiana University School of Medicine, Indianapolis, IN. Accepted for publication Feb 27, 1985. Address reprint requests to Dr.Brown, Cardiovascular Surgery, Emerson Hall, Room 212, 1100 W Michigan St, Indianapolis, IN 46223.
620
pericardial patch angioplasty was performed to correct the left pulmonary artery branch stenosis. Microscopical examination of the aneurysm showed smooth muscle cells widely separated by a myxoid collagenous substance containing numerous fibroblasts, occasional histiocytes, and a paucity of blood vessels. The patient’s postoperative course was uneventful. A follow-up visit in one month showed her to be doing well without evidence of heart failure. Postoperative cardiac catheterization demonstrated a peak systolic pressure difference of 12 mm Hg between the left pulmonary artery and right ventricle. Cineangiograms showed complete relief of the left pulmonary artery stenosis, minimal narrowing of the right pulmonary artery, and no residual ductus arteriosus (Fig 3).
Comment The ductus arteriosus is a derivative of the sixth aortic arch. It connects the pulmonary artery and the descending aorta during fetal life, and provides a shunt to carry blood away from the pulmonary vascular bed. In most instances the ductus closes during the first day of life by means of intrinsic muscular contraction. The initiating event for this closure is incompletely understood. Anatomical obliteration of the ductus proceeds over the next several weeks, with fibrosis and degeneration of the cellular elements leaving the ligamentum arteriosum. Closure begins at the pulmonary end, and the aortic end closes last [l]. Sometimes this natural process of involution fails to occur, leaving a patent ductus arteriosus. More rarely, aneurysmal dilatation of the ductus occurs. Both of these aberrations can have severe consequences for the patient and have been of considerable interest to cardiologists and surgeons. [2]. Since Martin [3] described ductal aneurysm in 1827,71 such lesions have been reported, to our knowledge [4]. In 1972, Falcone and colleagues [2] reviewed and categorized 61 ductal aneurysms. Their grouping of aneurysms into infantile, childhood, and adult types showed an overall complication rate of 43% and a resultant mortality of 31%. The complications found were rupture, embolism, and infection along with associated malformations, and the incidence of these complications was greatest in the infantile group. No correct antemortem diagnosis had been made in an infant at that time. In 1975, Ferlic and co-workers [5] reported the successful excision of a symptomatic ductus aneurysm in an infant after preoperative angiographic diagnosis. One year earlier, Heikkinen and Simila [6] had reported on 2 newborns with ductal aneurysms who underwent operation for the finding of a mediastinal mass. Infantile aneurysms diagnosed preoperatively were also reported
621
Case Report: Jesseph et al: Ductus Arteriosus Aneurysm in lnfancy
Fig 3 . Postoperative study showing widely patent left pulmonary artery and no evidence of the ductus. Fig I . Aortic injection demonstrating a saccular aneurysm of the ductus arteriosus (D).
Fig 2 . Pulmonary trunk with arrow marking severe stenosis of the left pulmona y artery.
by Rutishauser and associates [7] in 1977 and Kirks and colleagues [8] in 1980. Of the 64 infantile aneurysms reported, most have been in infants less than 2 months of age. Fifty-eight aneurysms were diagnosed at autopsy, and only 3 patients underwent successful operation with a preoperative diagnosis. To our knowledge, our patient represents the fourth such case reported. Several theories have been proposed to explain the
pathogenesis of ductal aneurysms [l-91. Some aneurysms have an obvious cause, such as trauma or infection, but the etiology of spontaneous ductal aneurysms is not known. It has been suggested that aneurysmal dilatation of the ductus may be part of the natural involutional process [6]. This idea is appealing, since the pulmonary end of the ductus is known to close first, subjecting the structurally weak “ductus diverticulum” to the high pressures of the aorta. In 1947, Taussig (91 stated that this was the only mechanism by which ductal aneurysms were formed. However, in patients such as ours who have an aneurysm of a patent ductus, this argument does not hold true. Aneurysmal dilatation of the ductus may result from an inherent structural abnormality. In patients with persistent patency of the ductus arteriosus, there is an additional subendothelial elastic lamina covering the intimal cushions [lo]. In our patient, histological examination of the ductus showed myxoid degeneration, a finding similar to that found in some connective tissue diseases such as Marfan’s syndrome. Although there was no evidence of connective tissue disease in our patient, it seems likely that a primary structural abnormality predisposed the ductus arteriosus to both persistent patency and aneurysm formation.
References 1. Cruickshank B, Marquis RM:Spontaneous aneurysm of the ductus arteriosus: a review and report of the tenth adult case. Am J Med 25:140, 1958 2. Falcone MW, Perloff JK, Roberts WC: Aneurysm of the nonpatent ductus arteriosus. Am J Cardiol29:422, 1972 3. Martin: Dilatation aneurysmal du canal artenel. Bull Soc Anat Paris 217, 1827 4. Japko L, Skolnick L, Morecki R, Gartner L Saccular aneurysm of ductus arteriosus. NY State J Med 80:1970-1971, 1980
622 The Annals of Thoracic Surgery Vol 40 No 6 December 1985
5. Ferlic RM, Hofschire PJ, Mooring PK. Ruptured ductus ar-
teriosus aneurysm in an infant report of a survivor. Ann Thorac Surg 20456, 1975 6. Heikkinen ES, S i a ES Aneurysm of the ductus arteriosus in infancy: report of two surgically corrected cases. J Pediatr Surg 7392, 1972 7. Rutishauser M, Ronen G, Wyler F Aneurysm of the nonpatent ductus arteriosus in the newborn. Acta Paediatr Scand 66549, 1977
8. Kirks PR, McCook TA, Serwer GA, Oldham HN: Aneurysm of the ductus arteriosus in the neonate. AJR 1x573, 1980 9. Taussig HB: Congenital malformationsof the Heart. New York, Commonwealth Fund, 1947, p 346 10. Gittenberger-deGroot AC Ductus arteriosus: histological
observations. In Godman M, Marquis RM (eds). Pediatric Cardiology: Heart Disease in the Newborn. Edinburgh, Churchill Livingstone, 1978, vol2, pp 4-14
REVIEW OF RECENT BOOKS
Diseases of the Aorta Including an Atlas of Angiographic Pathology and Surgical Technique E. Stanley Crawford and John L. Crazuford Baltimore, Williams 6 Wilkins, 1984 400 pp, illustrated, $135.00
Congenital Heart Surgery: Current Techniques and Controversies Edited by Anthony L. Moulton, M . D . Pasadena, CA, Appleton Davies, 1984 347 pp, illustrated, $97.50
Reviewed by Giacomo A . Dehria, M . D .
Reviewed by John G . Jacobson, M . D .
Diseases of the Aorta by E. Stanley and John L. Crawford is an atlas of angiographic pathology and surgical technique drawn from the authors’ long and probably unmatched experience with these dramatic surgical conditions. Chapters focus on particular disease processes and their impact on the aorta at each level. The first two, and largest, chapters discuss degenerative aneurysms and aortic dissection. The remaining ten chapters complete the review and include subjects as diverse as aortic infection and Marfan’s syndrome. Each section includes a concise, complete text review and an up-to-date bibliography. These are excellent and provide all the information usually required by an experienced surgeon. The remaining pages are filled with extraordinary, highquality color illustrations of disease processes and surgical technique, along with clear and easy-to-interpret high-resolution angiograms made before and after repair. All illustrations are uncluttered by either labels or lines and can be used as technical aids, reminders to residents, or examples for patients and their families. The array of operations described and the quality of the illustrations are unrivaled in my experience. Management of obscure but important conditions such as aorta-pulmonary artery fistula or primary tumors of the aorta are covered as completely as a straightforward repair of an abdominal aortic aneurysm. The book is a treasure trove of tricks and maneuvers, and I, at least, was unable to imagine a variation in aortic pathology that was not included. It will be apparent to any cardiovascular surgeon examining this book that it belongs in his or her collection. It is surgically accurate, beautifully illustrated, completely referenced, and a pleasure to hold and read. In fact, the quality is so high as perhaps to independently raise the standard for all subsequent atlases. Certainly it will remain the basic reference text on diseases of the aorta for many years in the future.
In recent decades the repair of many congenital cardiac lesions has become standardized and highly successful. Congenital Heart Surgery: Current Techniques and Controversies focuses on the difficult areas that remain with us in which a concensus does not exist and in which uniformly excellent results have remained an elusive goal. An outgrowth of a symposium, this compact volume retains the excitement of that meeting. Prominent surgeons from around the world share their progressive experiences-some encouraging, some disappointing-in seven sections. Trusler, Brom, Yacoub, Stark, and others describe large series with details of “how to do it” as well as results in “Transposition of the Great Arteries.” Parenzan, Ebert, and Danielson speak of spectacular progress in “Truncus Arteriosus.” “Atrioventricular Septal Defect” is addressed by Bjork, Danielson, and Carpentier. Pacifico, Fontan, Kreutzer, Danielson, and others present their findingsin “Tricuspid Atresia/UniventricularHeart.” The other sections, “Coarctation and Ventricular Septal Defect,” “Perfusion and Hypothermia,” and ”Pulmonary Atresia with Intact Ventricular Septum,” contain contributions from uniquely qualified authors. In many of the sections, Robert Anderson has provided provocative discussions of pathology with challenging concepts for nomenclature and conceptual constructs. Dr. Moulton, the editor, has assembled the concise, wellillustrated chapters and updated results in the interval between presentation and publication, giving the book a current perspective. Clinicians who care for patients with the malformations addressed in this book will enjoy and benefit from reading it, and students seeking an introduction to complex congenital heart disease will find the volume a ready starting place. We are all in debt to Dr. Moulton for preserving the scientific data and mood from the symposium in this text.
Chicago, ZL
Loma Linda, CA
Ductus Arteriosus Aneurysm in Infancy Jerry M. Jesseph, M.D., Ph.D., Lynn Mahony, M.D., Donald A. Girod, M.D., and John W. Brown, M.D. ABSTRACT An infant with symptoms of congestive heart failure at 6 months of age was found by cardiac catheterization at the age of 1 year to have a fusiform aneurysm of the ductus arteriosus as well as discrete stenosis of the left pulmonary artery and a secundum atrial septal defect. The aneurysm was resected, and a pericardial patch angioplasty corrected the peripheral pulmonary stenosis. The atrial septal defect was closed in a separate procedure. To our knowledge, this case is one of four ductal aneurysms reported in infants that have been diagnosed before operation and successfully treated. The pathophysiology and literature are reviewed. Aneurysmal dilatation of the ductus arteriosus is a rare condition found in both infants and adults[l]. This lesion has seldom been diagnosed during life because of its clinical manifestations and the mortality due to rupture, thrombosis, embolism, and infection as well as associated lesions (21. Therefore, most authors stress early diagnosis and surgical repair. A 1-year-old female infant had been found to have a heart murmur at birth. There was no history of maternal rubella. Her growth and development were essentially normal until she was 6 months old, when clinical and radiographic signs of congestive heart failure developed. Treatment with digoxin provided good relief of symptoms. At 10 months of age the patient underwent cardiac catheterization, which demonstrated a left-to-right shunt at the atrial level. The pulmonary-systemic flow ratio was 2.62:1, and there was a peak systolic pressure difference of 40 mm Hg between the right ventricle and the right pulmonary artery. It was not possible to enter the left pulmonary artery. Cineangiograms demonstrated aneurysmal dilatation of a widely patent ductus arteriosus (Fig 1)and marked stenosis at the origin of the left pulmonary artery (Fig 2) as well as mild branch stenosis of the right pulmonary artery. In addition, a moderate-sized secundum atrial septal defect was seen. The patient was taken to the operating room. Through a left-sided thoracotomy, a fusiform aneurysm of a patent ductus arteriosus measuring 1.5 cm in diameter and containing no thrombus was resected and the aortic and pulmonary stumps were oversewn. At the same time, a From the Departments of Surgery and Pediatrics, Indiana University School of Medicine, Indianapolis, IN. Accepted for publication Feb 27, 1985. Address reprint requests to Dr.Brown, Cardiovascular Surgery, Emerson Hall, Room 212, 1100 W Michigan St, Indianapolis, IN 46223.
620
pericardial patch angioplasty was performed to correct the left pulmonary artery branch stenosis. Microscopical examination of the aneurysm showed smooth muscle cells widely separated by a myxoid collagenous substance containing numerous fibroblasts, occasional histiocytes, and a paucity of blood vessels. The patient’s postoperative course was uneventful. A follow-up visit in one month showed her to be doing well without evidence of heart failure. Postoperative cardiac catheterization demonstrated a peak systolic pressure difference of 12 mm Hg between the left pulmonary artery and right ventricle. Cineangiograms showed complete relief of the left pulmonary artery stenosis, minimal narrowing of the right pulmonary artery, and no residual ductus arteriosus (Fig 3).
Comment The ductus arteriosus is a derivative of the sixth aortic arch. It connects the pulmonary artery and the descending aorta during fetal life, and provides a shunt to carry blood away from the pulmonary vascular bed. In most instances the ductus closes during the first day of life by means of intrinsic muscular contraction. The initiating event for this closure is incompletely understood. Anatomical obliteration of the ductus proceeds over the next several weeks, with fibrosis and degeneration of the cellular elements leaving the ligamentum arteriosum. Closure begins at the pulmonary end, and the aortic end closes last [l]. Sometimes this natural process of involution fails to occur, leaving a patent ductus arteriosus. More rarely, aneurysmal dilatation of the ductus occurs. Both of these aberrations can have severe consequences for the patient and have been of considerable interest to cardiologists and surgeons. [2]. Since Martin [3] described ductal aneurysm in 1827,71 such lesions have been reported, to our knowledge [4]. In 1972, Falcone and colleagues [2] reviewed and categorized 61 ductal aneurysms. Their grouping of aneurysms into infantile, childhood, and adult types showed an overall complication rate of 43% and a resultant mortality of 31%. The complications found were rupture, embolism, and infection along with associated malformations, and the incidence of these complications was greatest in the infantile group. No correct antemortem diagnosis had been made in an infant at that time. In 1975, Ferlic and co-workers [5] reported the successful excision of a symptomatic ductus aneurysm in an infant after preoperative angiographic diagnosis. One year earlier, Heikkinen and Simila [6] had reported on 2 newborns with ductal aneurysms who underwent operation for the finding of a mediastinal mass. Infantile aneurysms diagnosed preoperatively were also reported
621
Case Report: Jesseph et al: Ductus Arteriosus Aneurysm in lnfancy
Fig 3 . Postoperative study showing widely patent left pulmonary artery and no evidence of the ductus. Fig I . Aortic injection demonstrating a saccular aneurysm of the ductus arteriosus (D).
Fig 2 . Pulmonary trunk with arrow marking severe stenosis of the left pulmona y artery.
by Rutishauser and associates [7] in 1977 and Kirks and colleagues [8] in 1980. Of the 64 infantile aneurysms reported, most have been in infants less than 2 months of age. Fifty-eight aneurysms were diagnosed at autopsy, and only 3 patients underwent successful operation with a preoperative diagnosis. To our knowledge, our patient represents the fourth such case reported. Several theories have been proposed to explain the
pathogenesis of ductal aneurysms [l-91. Some aneurysms have an obvious cause, such as trauma or infection, but the etiology of spontaneous ductal aneurysms is not known. It has been suggested that aneurysmal dilatation of the ductus may be part of the natural involutional process [6]. This idea is appealing, since the pulmonary end of the ductus is known to close first, subjecting the structurally weak “ductus diverticulum” to the high pressures of the aorta. In 1947, Taussig (91 stated that this was the only mechanism by which ductal aneurysms were formed. However, in patients such as ours who have an aneurysm of a patent ductus, this argument does not hold true. Aneurysmal dilatation of the ductus may result from an inherent structural abnormality. In patients with persistent patency of the ductus arteriosus, there is an additional subendothelial elastic lamina covering the intimal cushions [lo]. In our patient, histological examination of the ductus showed myxoid degeneration, a finding similar to that found in some connective tissue diseases such as Marfan’s syndrome. Although there was no evidence of connective tissue disease in our patient, it seems likely that a primary structural abnormality predisposed the ductus arteriosus to both persistent patency and aneurysm formation.
References 1. Cruickshank B, Marquis RM:Spontaneous aneurysm of the ductus arteriosus: a review and report of the tenth adult case. Am J Med 25:140, 1958 2. Falcone MW, Perloff JK, Roberts WC: Aneurysm of the nonpatent ductus arteriosus. Am J Cardiol29:422, 1972 3. Martin: Dilatation aneurysmal du canal artenel. Bull Soc Anat Paris 217, 1827 4. Japko L, Skolnick L, Morecki R, Gartner L Saccular aneurysm of ductus arteriosus. NY State J Med 80:1970-1971, 1980
622 The Annals of Thoracic Surgery Vol 40 No 6 December 1985
5. Ferlic RM, Hofschire PJ, Mooring PK. Ruptured ductus ar-
teriosus aneurysm in an infant report of a survivor. Ann Thorac Surg 20456, 1975 6. Heikkinen ES, S i a ES Aneurysm of the ductus arteriosus in infancy: report of two surgically corrected cases. J Pediatr Surg 7392, 1972 7. Rutishauser M, Ronen G, Wyler F Aneurysm of the nonpatent ductus arteriosus in the newborn. Acta Paediatr Scand 66549, 1977
8. Kirks PR, McCook TA, Serwer GA, Oldham HN: Aneurysm of the ductus arteriosus in the neonate. AJR 1x573, 1980 9. Taussig HB: Congenital malformationsof the Heart. New York, Commonwealth Fund, 1947, p 346 10. Gittenberger-deGroot AC Ductus arteriosus: histological
observations. In Godman M, Marquis RM (eds). Pediatric Cardiology: Heart Disease in the Newborn. Edinburgh, Churchill Livingstone, 1978, vol2, pp 4-14
REVIEW OF RECENT BOOKS
Diseases of the Aorta Including an Atlas of Angiographic Pathology and Surgical Technique E. Stanley Crawford and John L. Crazuford Baltimore, Williams 6 Wilkins, 1984 400 pp, illustrated, $135.00
Congenital Heart Surgery: Current Techniques and Controversies Edited by Anthony L. Moulton, M . D . Pasadena, CA, Appleton Davies, 1984 347 pp, illustrated, $97.50
Reviewed by Giacomo A . Dehria, M . D .
Reviewed by John G . Jacobson, M . D .
Diseases of the Aorta by E. Stanley and John L. Crawford is an atlas of angiographic pathology and surgical technique drawn from the authors’ long and probably unmatched experience with these dramatic surgical conditions. Chapters focus on particular disease processes and their impact on the aorta at each level. The first two, and largest, chapters discuss degenerative aneurysms and aortic dissection. The remaining ten chapters complete the review and include subjects as diverse as aortic infection and Marfan’s syndrome. Each section includes a concise, complete text review and an up-to-date bibliography. These are excellent and provide all the information usually required by an experienced surgeon. The remaining pages are filled with extraordinary, highquality color illustrations of disease processes and surgical technique, along with clear and easy-to-interpret high-resolution angiograms made before and after repair. All illustrations are uncluttered by either labels or lines and can be used as technical aids, reminders to residents, or examples for patients and their families. The array of operations described and the quality of the illustrations are unrivaled in my experience. Management of obscure but important conditions such as aorta-pulmonary artery fistula or primary tumors of the aorta are covered as completely as a straightforward repair of an abdominal aortic aneurysm. The book is a treasure trove of tricks and maneuvers, and I, at least, was unable to imagine a variation in aortic pathology that was not included. It will be apparent to any cardiovascular surgeon examining this book that it belongs in his or her collection. It is surgically accurate, beautifully illustrated, completely referenced, and a pleasure to hold and read. In fact, the quality is so high as perhaps to independently raise the standard for all subsequent atlases. Certainly it will remain the basic reference text on diseases of the aorta for many years in the future.
In recent decades the repair of many congenital cardiac lesions has become standardized and highly successful. Congenital Heart Surgery: Current Techniques and Controversies focuses on the difficult areas that remain with us in which a concensus does not exist and in which uniformly excellent results have remained an elusive goal. An outgrowth of a symposium, this compact volume retains the excitement of that meeting. Prominent surgeons from around the world share their progressive experiences-some encouraging, some disappointing-in seven sections. Trusler, Brom, Yacoub, Stark, and others describe large series with details of “how to do it” as well as results in “Transposition of the Great Arteries.” Parenzan, Ebert, and Danielson speak of spectacular progress in “Truncus Arteriosus.” “Atrioventricular Septal Defect” is addressed by Bjork, Danielson, and Carpentier. Pacifico, Fontan, Kreutzer, Danielson, and others present their findingsin “Tricuspid Atresia/UniventricularHeart.” The other sections, “Coarctation and Ventricular Septal Defect,” “Perfusion and Hypothermia,” and ”Pulmonary Atresia with Intact Ventricular Septum,” contain contributions from uniquely qualified authors. In many of the sections, Robert Anderson has provided provocative discussions of pathology with challenging concepts for nomenclature and conceptual constructs. Dr. Moulton, the editor, has assembled the concise, wellillustrated chapters and updated results in the interval between presentation and publication, giving the book a current perspective. Clinicians who care for patients with the malformations addressed in this book will enjoy and benefit from reading it, and students seeking an introduction to complex congenital heart disease will find the volume a ready starting place. We are all in debt to Dr. Moulton for preserving the scientific data and mood from the symposium in this text.
Chicago, ZL
Loma Linda, CA