CASE REPORTS
Transesophageal Echocardiigraphic Detection of Abnormalities of the Tricuspid Valve in Adul@ Associated with Spontaneous e:gfcTrnernbranous Ventricular Timothy M. Winslow, MD, Rita F. Redberg, MD, Elyse Foster, MD, and Nelson B. Schiller, MD erimembranous defects are the most frequent types of ventricuP lar septal defect. Spontaneous clo-
for the detection of ventricular sep tal aneurysmswas not previously reported.
sure of these defects occurs often in childhood. The mechanismof closure is controversial, but recent pathologic and clinical studiesin children suggest that it occurs by reduplication and adhesion of the septal leaflet of the tricuspid valve to the margins of the defect.1-2Closure of the defect is frequently associated with the formation of a ventricular septal aneurysm, a term that describesan outpouching in the region of the membranous septum.3 The adherent tricuspid valve protrudes into the right ventricle during ventricular systole, accounting for its aneurysmal appearance. Less frequently, prolapseof an aortic valve leaflet reducesthe sizeof the defect and can result in aortic insufficiency.’ Early pathologic studiesdemonstrateddeformity of the tricuspid valve resulting from ventricular septal aneurysm formation.4 Chordal shortening and fusion, and leaflet deformity havebeendemonstrated in necropsy examinations of patients with ventricular septalaneurysms. Functional abnormalities resulting from tricuspid valve involvementin the formation of the aneurysm were recently described in pediatric studies.2 However, these abnormalities have not been described in adults, and the use of transesophageal echocardiography
We report thefindings in 4 adult patients with ventricular septal aneurysms who underwent transesophageal echocardiography (TEE) at our institution. Three patients had a childhood history of ventricular septal defect. The fourth patient was diagnosed as an adult by TEE, and the presence of a membranous ventricular septal defect was confirmed later at autopsy. Three patients had completely closed defects, and the fourth had a small shunt by Doppler color flow mapping. We relate the TEEfindings in these patients to the mechanism of ventricular septal defect closure and subsequent alteration in tricuspid valve function. The index case was a 27-year-old woman who presented with supraventricular tachycardia and had evidence of tricuspid insufficiency on physical examination. A surface echocardiogram showed moderate to severe tricuspid insufficiency and normal pulmonary artery pressure.
A saline solution contrast study was negative for an intracardiac shunt. TEE was performed to determine the etiology of her tricuspid insufficiency and showed that the septal leaflet of the tricuspid valve was involved in the closure of a membranous ventricular septal defect. This resulted in deformity of the leaflet and a ventricular septal aneurysm (Figure I). After review of the records of 520 consecutive outpatient TEE examinations from October 1988 to October 1991, 3 other patients with ventricular septal aneurysms were identified. The clinical and echocardiographic features of the lpatients are summarized in Table I.
It is well-recognized that a sub stantial number of defects in the membranous ventricular septum spontaneously close or diminish in sizeduring childhood. Early observations suggestedthat aneurysm formation was frequently associated with membranousventricular septal defectsin childhood.5Subsequently, aneurysm formation was thought to have a role in spontaneousdiminution in size of membranous defects. Tandon and Edwards6 described their autopsy findings of an aneurysm resulting from adhesionof the septal leaflet of the tricuspid valve to the margin of a membranousdefect resulting in closureof the defect.The role of this mechanismin ventricular septal defect closure was confirmed by later pathologic and echocardio-
From the Division of Medicine, John Henry Mills Echocardiography Laboratory, Cardio vascular ResearchInstitute, University of California, San Francisco,California. Dr. Winslow is a fellow sponsoredby the United States Army, and his current addressis: Moffrtt Haspital, Room 342A, SchocardiographyLaboratory, University of California, 505 Pamassus Avenue, San Francisco, California 94143. Manuscript received November 4, 1991; re vised manuscript received and accepted May 18.1992.
CASE REPORTS 967
TABLE I Mechanism of Perimembranous Ventricular Septal Defect Closure Mechanism of VSD Closure
Age (yr) & Sex
VSA Detected by TTE
Reduplication and/or Adhesion TV
Aortic Leaflet Prolapse
27F 31M* 20F 28M
0 0 0 +
+ + + +
0 0 0 0
graphic studies. In a necropsyseries by Anderson et al,’ 26 of 69 membranousventricular septal defects(38%) had evidenceof at least partial closure. The tricuspid valve was responsible for closure by either reduplication of tissueor adhesionof the septal leaflet to the defect in 22 of 26 cases (85%). Prolapse of an aortic valve leaflet was the mechanismfor reduction of the defect size in 3 of 26 cases ( 12%).The mechanismby which the tricuspid valve closes the defect is postulated to involve endothelial roughening of the tricuspid valve and the margins of the defect by the left to right shunt. Eventual adhesionof the surfacesoccursand closesthe defect.7 Despite the recognition that the tricuspid valve is involved in the closure of membranous ventricular septal defects,few studies have eval-
Tricuspid Insufficiency 3-4+ 2+
1+ 0
uated the resulting functional abnormalities. Eshaghpouret al* usedphonocardiography to demonstrate the associationof tricuspid insufficiency with ventricular septalaneurysmformation and speculated that insufliciency may result from a mechanical disturbance caused by involvement of the tricuspid valve in aneurysm formation. Hornberger et al9 recently observed 6 patients with significant diminution of ventricular septal defect size on serial surface echocardiographic examinations. They found a progressiveincrease in tricuspid insufficiency as the defect became smaller. Finally, Magherini et al2reported that significant tricuspid insufficiency was detected by pulse wave Doppler examination in 78%of patients with “restrictive” tissue in the area of a membranousventricular septal defect. They concluded
that tricuspid valve dysfunction was the result of the septal leaflet being involved in the closure of the membranous defect. All these studies were performed in children. There have been no studies in adults. Echocardiography is the most sensitive method of identifying ventricular septal aneurysms.lo Our findings suggest that TEE, with its superior image resolution, is an even more sensitivemethod than transthoracic echocardiographyfor their detection. Furthermore, TEE enabled better visualization of the anatomic structures that formed the ventricular septal aneurysms in these patients. In each patient, the tricuspid valve could be seenforming the aneurysm and closing the ventricular septal defect.In 3 patients,reduplication of the septal leaflet could clearly be seen(Figure 2). The resulting tricuspid valve deformity was associated with at least moderate tricuspid insufficiency in 2 of 4 patients. No patient had prolapse of an aortic leaflet or aortic insufficiency detected. These findings are similar to results reported in the pediatric literature. In adults, tricuspid valve participation in membranousventricular sep tal defect closure may be a more frequent cause of isolated tricuspid insufficiency than has been appreciated. We concludethat TEE is a sensitive method for the detection of ventricular septal aneurysms. Furthermore, tricuspid insufficiency can result from closure of membranous ventricular septaldefects.TEE to detect a ventricular septal aneurysm should be consideredin patients with unexplained tricuspid insufficiency, particularly if there is history of a childhood murmur. 1. Anderson RH, Lenox CC, Zuberbuhler JR. Mechanismsof closureof perimembranousventricolar septal defect. Am J Cardiol 1983;52:341-345. 2. Magherini A, Urciuolo A, TommassiniCR, Boldrini R, Rossi M, Iadevaia A, Parri A, Milio C, Romani C. Restrictive tissuein the area of perimembranous ventricular septal defect. Cross-sectional and Doppler echocardiographicstudy. Eur Hearf J 1990;11:601-610. 3. Ramaciotti C, Keren A, Silverman NH. Importance of (perimembranous)ventricular septal aneurysm in the natural history of isolatedperimembranousventricular septaldefect.Am J Cardiol1986;57: 268-272.
4. Chsler E, Koms ME, Edwards JE. Anomaliesof the tricuspid valve, including pouches,resembling
968
THE AMERICAN JOURNAL OF CARDIOLOGY VOLUME 70
OCTOBER 1. 1992
aneurysmsof the membranousventricular septum. Am J Cardiol 1968;21:661-668.
5. Freedom RM, White RD. Pieroni DR. Varghese PJ, Kroveta LJ, Rowe RD. The natural history of the x-called aneurysm of the membranousventricular septum in childhood. Circularion 1974;49:375-384. 6. Tandon R, EdwardsJE. Aneurysmlike formations in relation to membranousventricular septum. Circulation 1973;47:1089-1097.
7. Guntheroth WG. Ductus arteriosusand ventricular septaldefect in the adult. In: Parmley WW, Chatterjee K, cd. Cardiology, volume 2. Philadelphia:J.B. Lippincott, 19892. 8. EshaghpourE, Kawai N, Linhart JW. Tricuspid insufficiency associatedwith aneurysm of the ventricular septum.Pediatrics 1978;61:586-592. 9. Hornberger LK, Sahn DJ, Krabill KA, Sherman FS, SwenssonRE, Pesonen E, Hagen-Ansert S,
Chung KJ. Elucidation of the natural history of ventricular septal defects by serial Doppler color Bow mappingstudies.J Am Co/l Cardiol 1989;13:111l1118. 10. Canale JM, Sahn DJ, Valdes-Cruz LM, Allen HD, Goldberg SJ, Ovitt TW. Accuracy of two-dimensionalechocardiographyin the detection of aneurysms of the ventricular septum. Am Heart J 1981;101:255-259.
CASE REPORTS
969
Transesophageal Echocardiigraphic Detection of Abnormalities of the Tricuspid Valve in Adul@ Associated with Spontaneous e:gfcTrnernbranous Ventricular Timothy M. Winslow, MD, Rita F. Redberg, MD, Elyse Foster, MD, and Nelson B. Schiller, MD erimembranous defects are the most frequent types of ventricuP lar septal defect. Spontaneous clo-
for the detection of ventricular sep tal aneurysmswas not previously reported.
sure of these defects occurs often in childhood. The mechanismof closure is controversial, but recent pathologic and clinical studiesin children suggest that it occurs by reduplication and adhesion of the septal leaflet of the tricuspid valve to the margins of the defect.1-2Closure of the defect is frequently associated with the formation of a ventricular septal aneurysm, a term that describesan outpouching in the region of the membranous septum.3 The adherent tricuspid valve protrudes into the right ventricle during ventricular systole, accounting for its aneurysmal appearance. Less frequently, prolapseof an aortic valve leaflet reducesthe sizeof the defect and can result in aortic insufficiency.’ Early pathologic studiesdemonstrateddeformity of the tricuspid valve resulting from ventricular septal aneurysm formation.4 Chordal shortening and fusion, and leaflet deformity havebeendemonstrated in necropsy examinations of patients with ventricular septalaneurysms. Functional abnormalities resulting from tricuspid valve involvementin the formation of the aneurysm were recently described in pediatric studies.2 However, these abnormalities have not been described in adults, and the use of transesophageal echocardiography
We report thefindings in 4 adult patients with ventricular septal aneurysms who underwent transesophageal echocardiography (TEE) at our institution. Three patients had a childhood history of ventricular septal defect. The fourth patient was diagnosed as an adult by TEE, and the presence of a membranous ventricular septal defect was confirmed later at autopsy. Three patients had completely closed defects, and the fourth had a small shunt by Doppler color flow mapping. We relate the TEEfindings in these patients to the mechanism of ventricular septal defect closure and subsequent alteration in tricuspid valve function. The index case was a 27-year-old woman who presented with supraventricular tachycardia and had evidence of tricuspid insufficiency on physical examination. A surface echocardiogram showed moderate to severe tricuspid insufficiency and normal pulmonary artery pressure.
A saline solution contrast study was negative for an intracardiac shunt. TEE was performed to determine the etiology of her tricuspid insufficiency and showed that the septal leaflet of the tricuspid valve was involved in the closure of a membranous ventricular septal defect. This resulted in deformity of the leaflet and a ventricular septal aneurysm (Figure I). After review of the records of 520 consecutive outpatient TEE examinations from October 1988 to October 1991, 3 other patients with ventricular septal aneurysms were identified. The clinical and echocardiographic features of the lpatients are summarized in Table I.
It is well-recognized that a sub stantial number of defects in the membranous ventricular septum spontaneously close or diminish in sizeduring childhood. Early observations suggestedthat aneurysm formation was frequently associated with membranousventricular septal defectsin childhood.5Subsequently, aneurysm formation was thought to have a role in spontaneousdiminution in size of membranous defects. Tandon and Edwards6 described their autopsy findings of an aneurysm resulting from adhesionof the septal leaflet of the tricuspid valve to the margin of a membranousdefect resulting in closureof the defect.The role of this mechanismin ventricular septal defect closure was confirmed by later pathologic and echocardio-
From the Division of Medicine, John Henry Mills Echocardiography Laboratory, Cardio vascular ResearchInstitute, University of California, San Francisco,California. Dr. Winslow is a fellow sponsoredby the United States Army, and his current addressis: Moffrtt Haspital, Room 342A, SchocardiographyLaboratory, University of California, 505 Pamassus Avenue, San Francisco, California 94143. Manuscript received November 4, 1991; re vised manuscript received and accepted May 18.1992.
CASE REPORTS 967
TABLE I Mechanism of Perimembranous Ventricular Septal Defect Closure Mechanism of VSD Closure
Age (yr) & Sex
VSA Detected by TTE
Reduplication and/or Adhesion TV
Aortic Leaflet Prolapse
27F 31M* 20F 28M
0 0 0 +
+ + + +
0 0 0 0
graphic studies. In a necropsyseries by Anderson et al,’ 26 of 69 membranousventricular septal defects(38%) had evidenceof at least partial closure. The tricuspid valve was responsible for closure by either reduplication of tissueor adhesionof the septal leaflet to the defect in 22 of 26 cases (85%). Prolapse of an aortic valve leaflet was the mechanismfor reduction of the defect size in 3 of 26 cases ( 12%).The mechanismby which the tricuspid valve closes the defect is postulated to involve endothelial roughening of the tricuspid valve and the margins of the defect by the left to right shunt. Eventual adhesionof the surfacesoccursand closesthe defect.7 Despite the recognition that the tricuspid valve is involved in the closure of membranous ventricular septal defects,few studies have eval-
Tricuspid Insufficiency 3-4+ 2+
1+ 0
uated the resulting functional abnormalities. Eshaghpouret al* usedphonocardiography to demonstrate the associationof tricuspid insufficiency with ventricular septalaneurysmformation and speculated that insufliciency may result from a mechanical disturbance caused by involvement of the tricuspid valve in aneurysm formation. Hornberger et al9 recently observed 6 patients with significant diminution of ventricular septal defect size on serial surface echocardiographic examinations. They found a progressiveincrease in tricuspid insufficiency as the defect became smaller. Finally, Magherini et al2reported that significant tricuspid insufficiency was detected by pulse wave Doppler examination in 78%of patients with “restrictive” tissue in the area of a membranousventricular septal defect. They concluded
that tricuspid valve dysfunction was the result of the septal leaflet being involved in the closure of the membranous defect. All these studies were performed in children. There have been no studies in adults. Echocardiography is the most sensitive method of identifying ventricular septal aneurysms.lo Our findings suggest that TEE, with its superior image resolution, is an even more sensitivemethod than transthoracic echocardiographyfor their detection. Furthermore, TEE enabled better visualization of the anatomic structures that formed the ventricular septal aneurysms in these patients. In each patient, the tricuspid valve could be seenforming the aneurysm and closing the ventricular septal defect.In 3 patients,reduplication of the septal leaflet could clearly be seen(Figure 2). The resulting tricuspid valve deformity was associated with at least moderate tricuspid insufficiency in 2 of 4 patients. No patient had prolapse of an aortic leaflet or aortic insufficiency detected. These findings are similar to results reported in the pediatric literature. In adults, tricuspid valve participation in membranousventricular sep tal defect closure may be a more frequent cause of isolated tricuspid insufficiency than has been appreciated. We concludethat TEE is a sensitive method for the detection of ventricular septal aneurysms. Furthermore, tricuspid insufficiency can result from closure of membranous ventricular septaldefects.TEE to detect a ventricular septal aneurysm should be consideredin patients with unexplained tricuspid insufficiency, particularly if there is history of a childhood murmur. 1. Anderson RH, Lenox CC, Zuberbuhler JR. Mechanismsof closureof perimembranousventricolar septal defect. Am J Cardiol 1983;52:341-345. 2. Magherini A, Urciuolo A, TommassiniCR, Boldrini R, Rossi M, Iadevaia A, Parri A, Milio C, Romani C. Restrictive tissuein the area of perimembranous ventricular septal defect. Cross-sectional and Doppler echocardiographicstudy. Eur Hearf J 1990;11:601-610. 3. Ramaciotti C, Keren A, Silverman NH. Importance of (perimembranous)ventricular septal aneurysm in the natural history of isolatedperimembranousventricular septaldefect.Am J Cardiol1986;57: 268-272.
4. Chsler E, Koms ME, Edwards JE. Anomaliesof the tricuspid valve, including pouches,resembling
968
THE AMERICAN JOURNAL OF CARDIOLOGY VOLUME 70
OCTOBER 1. 1992
aneurysmsof the membranousventricular septum. Am J Cardiol 1968;21:661-668.
5. Freedom RM, White RD. Pieroni DR. Varghese PJ, Kroveta LJ, Rowe RD. The natural history of the x-called aneurysm of the membranousventricular septum in childhood. Circularion 1974;49:375-384. 6. Tandon R, EdwardsJE. Aneurysmlike formations in relation to membranousventricular septum. Circulation 1973;47:1089-1097.
7. Guntheroth WG. Ductus arteriosusand ventricular septaldefect in the adult. In: Parmley WW, Chatterjee K, cd. Cardiology, volume 2. Philadelphia:J.B. Lippincott, 19892. 8. EshaghpourE, Kawai N, Linhart JW. Tricuspid insufficiency associatedwith aneurysm of the ventricular septum.Pediatrics 1978;61:586-592. 9. Hornberger LK, Sahn DJ, Krabill KA, Sherman FS, SwenssonRE, Pesonen E, Hagen-Ansert S,
Chung KJ. Elucidation of the natural history of ventricular septal defects by serial Doppler color Bow mappingstudies.J Am Co/l Cardiol 1989;13:111l1118. 10. Canale JM, Sahn DJ, Valdes-Cruz LM, Allen HD, Goldberg SJ, Ovitt TW. Accuracy of two-dimensionalechocardiographyin the detection of aneurysms of the ventricular septum. Am Heart J 1981;101:255-259.
CASE REPORTS
969
