- Email: [email protected]
Accessory flaplike tissue causing ventricular outflow obstruction
J THORAC
CARDIOVASC SURG
80:211-216, 1980
Accessory flaplike tissue causing ventricular outflow obstruction In three cases. two in children and one in a young adult. ventricular outflow obstruction was caused by a valvelike flap of accessory endocardial tissue. The angiocardiographic feature was that of a narrow. radiolucent. crescent-shaped or linear filling defect corresponding to the site of obstruction. Resection of the accessory tissue was performed in each case at the time of correction of associated conditions. In one case (a woman 20 years of age) the obstructing membrane was in the outflow tract of the right ventricle. and a ventricular septal defect was associated. In each of the other two cases the outflow tract of the left ventricle was the site of obstruction. In one (a /4-month-old boy). an ostium primum type of atrial septal defect and cleft mitral valve were associated; in the remaining case (a 7-year-old boy) complete transposition and ventricular septal defect were also present. In the latter case a Mustard procedure was performed and was followed by death. Successful results were obtained in the first two patients.
Antoinette S. Gomes, M.D., P. H. Nath, M.D., Amarjit Singh, M.D., Russell V. Lucas,
Jr.,
M.D., Kurt Amplatz, M.D., Demetre M. Nicoloff, M.D., and
Jesse E. Edwards, M.D., Minneapolis and St. Paul. Minn.
Ventricular outflow tract obstruction resulting from accessory endocardial tissue is uncommon. The anomaly may be recognized in ventriculograms, and relief of obstruction is possible through resection of the accessory tissue. Since the condition is uncommon and yet curable, it is our purpose to describe three cases in which the anomaly was identified angiocardiographically and treated surgically.
Case reports CAS E 1. A white, female subject with a cardiac murmur since birth and exertional cyanosis was first studied in 1955 at the University of Minnesota Hospitals at the age of 3 years and found to have a left-to-right shunt and pulmonary stenosis. A second cardiac catheterization in 1957 demonstrated the shunt to be at the ventricular level. The catheter could not be advanced into the pulmonary artery. No angiography was
From the Departmentsof Radiology. Pediatrics, Surgery and Pathology, University of Minnesota. Minneapolis; the Department of Pathology, United Hospitals-Miller Division, SI. Paul; and the Children's Health Center. Minneapolis, Minn. This study was supported by Public Health Service Research Grant 5 ROI HL05694 from the National Heart, Lung and Blood Institute, and by the Saint Paul Foundation, SI. Paul, Minn. Received for publication Nov. 27, 1979. Accepted for publication Jan. 14, 1980. Address for reprints: Jesse E. Edwards. M.D., Department of Pathology, United Hospitals-Miller Division, 125 W. College Ave., SI. Paul. Minn. 55102.
performed because of arrhythmias. The patient was lost to follow-up until 1972 when, at the age of 20 years, she presented with a spontaneous abortion. Physical examination revealed no cyanosis but there was bilateral clubbing of fingers. There was a Grade 4/6 pan systolic murmur at the left sternal border. Cardiac catheterization at this time revealed the right ventricular systolic pressure to be at systemic level, and there was a normal pressure in the pulmonary artery. A bidirectional shunt at the ventricular level was also identified. Angiography showed a crescent-shaped, linear filling defect in the outflow tract of the right ventricle (Fig. I). The patient was operated upon. When the right ventricle was opened, a large membranous ventricular septal defect and anomalous muscle bundles were found. The pulmonary valve was normal in appearance. A windsock-like flap was found in relation to the septal leaflet of the tricuspid valve and appeared to attach to abnormal chordae that inserted into the rim of the ventricular septal defect and to a nearby papillary muscle. This abnormal flap appeared to be capable of prolapsing into the outflow tract of the right ventricle and through the pulmonary valve orifice. The flap was excised and the ventricular septal defect closed. The patient has subsequently done well. Two months following the operation cardiac catheterization revealed the following pressures: right ventricle, 32/2-13 mm Hg; pulmonary trunk, 32/14 mm Hg. The excised flap was thin and cusplike. Histologically, it contained a core of loose connective tissue having the appearance of valvular spongiosa. To one side of this layer was a layer of dense compact collagen, and on the other side was a thinner layer composed of moderately delicate collagen (Fig. 2). CASE 2. A 14-month-old, black, male infant with intesti-
0022-5223/80/080211 +06$00.60/0 © 1980 The C. V. Mosby Co.
2I I
The Journal of
2 12
Gomes et al.
Thoracic and Cardiovascular Surgery
Fig. 2. Ca se I . Photomicrograph of flap excised surgically . There is a central core of loose, spongiosa-like tissue . On one side (I eft) is a den se layer of compact collagen; on the other side of the spongiosa-like layer is a thinner layer of connective tissue, the latter being composed of moderately delicate co llagen. (Elastic tissue stain; x 35 .)
Fig. 1. Case I . Right ventriculogram in (a) frontal view and lateral view. Beneath the pulmonary valve there is a crescent-shaped, linear filling defect (arrows) . More superiorIy, the pulmonary valve is normal. There is no evidence of a right-to-left shunt.
( b)
nal malrot ation. obstructing duodenal diaphragm, and cardiomegaly was examined at the Children 's Health Center of Minneapolis when he presented with congestive heart failure and pneumonia . A cardiac murmur had been present since birth . Physical examination re vealed a tachypneic, lethargic infant. There was a palpable thrill and an increased S2' A Grade 4/6 holosystolic murmur was heard over the entire precordium and was loudest along the left sternal border. An S, was pre sent. The electrocardiogram revealed left axis deviation and severe biventricular hypertrophy . Cardiac catheterization revealed continuity of the inferior vena cava with the right azygo s ve in. The catheter then passed from the right atrium , acros s the tricuspid valve. into the right ventricle and from the right ventricle to the left ventricle. Right atrial pre ssures were norm al and the right ventricular systo lic pressure was 70 to 80 mm Hg . The left ventricular apical systolic pressure was markedly elevated . being 160 mm Hg. Retrograde arterial catheterization revealed discrete obstruction in the sub aortic region of the left ventricle . A peakto-peak systolic gradient of 90 mm Hg was present between the left ventricular apex and left ventricular outflow tract. One month later , a seco nd card iac catheterization showed a persistent left superior vena cava draining into the coronary sinus
Volume 80 Number 2
Ventricular outflow obstruction
21 3
August. 1980
Fig. 4. Case 2. Photomicrograph of excised . valvelike flap of tissue from subaortic region. One extremity is nodular and composed of loose connective tissue . (Hematoxylin and eosin; X7 .)
Fig. 3. Case 2. Left ventriculography in frontal view . a . Systolic phase. There is a goose-neck deformity of the left ventricle and beneath the aortic valve is a crescent-shaped filling defect (arrows). The mitral valve is competent. b. Diastolic phase . A goose-neck deformity is apparent ; the subaortic filling defect noted in the systolic phase is not visualized .
and a left-to-right shunt at the atrial level. Angiography revealed an enlarged left ventricle with a clear-cut. goose-neck deformity . There was a discrete , crescent-shaped, membranous obstruction about 0.5 cm below a normal-appearing aortic valve (Fig . 3). The membrane appeared to be attached to the anterior portion of the mitral valve on one side and the ventricular septum on the other. There was no mitral regurgitation . At operation, an ostium prim urn atrial septal defect with cleft mitral valve was found . In the subarotic region of the left ventricle a nodular. f1aplike structure , measuring about 4 by 1.2 em , was found attached to the ventricular aspect of the anterior mitral leaflet. This was considered to have caused the subaortic stenosi s and was excised . A mitral valve annuloplasty was performed and a pericardial patch was used to close the atrial septal defect. Histologic examination of the excised tissue revealed a valvelike structure . One extremity showed nodular thickening formed by loose cellular connective tissue (Fig. 4) . Because of postoperative complications . including the presence of mitral insufficiency. two postoperative invasive studies were done and showed no signs of significant residual subaortic stenosi s.
The Journal of
2I4
Gomes et al.
Thorac ic and Cardiovascular Surgery
A p
Fig. 5. Case 3. Right ventric ulogram. a, Frontal view. There is op aci fication of both gre at vessel s. There is a linear , membranous filling defect in the subpulmo nary are a ( arro ws) . b, Lateral view . There is op acific ation of both ventricles and both gre at vessels . Beneath the pulm onary valve is a platelike, thin filling defect (a rro ws) s imilar to the one observed in the frontal view . A. Aorta . P , Pulm on ary trunk. Two months postoperati vely the left ventricular pressu res were 10010-16 mm Hg and the asce nd ing ao rtic pressu res were 93/ 60 mm Hg . At the age of 3 Y2 years the pressures we re as follows : left ventricle, 12017-10 mm Hg; ascending aorta , Il l/56 mm Hg . CAS E 3. A white, male infant was cyanotic at birth. When he was 7 weeks of age diagnoses of transpo sition of the great vessels , subpulmo nary stenosis, and ventricular septal defect were made at the University of Minn esota Hospitals. Transvenous atria l septostomy (Rashkind pro cedure) was followed by improvement in degree of cyanosis. Cardi ac catheterization at the age of 2 yea rs re vealed equal systolic pressur es in the anatomic rig ht ventricl e and aorta . Th e pressure in the pulmonary trunk was 20/6 mm Hg. During pullback to the left (pulmonary) ventricle, the pressure rose to 40 mm Hg sys to lic in the subpulmonary reg ion and 90/7 mm Hg in the body of the ventricle . The ca theter had reach ed the pulmonary arter y by passage from right atrium to left atrium to anatom ic left (pulmo nary) ventricle . Thi s indi cated that the grad ient was indeed subpulmonary and not ac ros s the ventricular septal defe ct. An ang iog ram performed by injection into the anatomic right ventricle show ed op acifica tio n of both ventricles . A radio lucent filling defect was observed ex tend ing from the area of the m itral valve into the subpulmo nary outflow regi on of the left ventricle (Fig. 5). Op eration was initially deferred because of the child's stable co ndition . Howe ver , at the age o f 7 yea rs the child was restudie d and operated upon. At operatio n, prior to the perform ance of a Mu stard proce dure , right ventriculotomy was done. Through the ventricular
septal defect a flap was ob served in the left ventricular outflow tract ; this was excised and the ventric ular septal defec t was closed. Postope rat ively , the child manifested co mplications associa ted with obstruction to pulm onary venous flow by the baffle of the Mustard procedure and d ied in the early postoperat ive peri od . At autopsy, the preoperati ve anatomic diagnoses were co nfirmed . No residual tissue was found in the left vent ricular outflow tract. The excised tissue was represented by a valvelike flap. Histolo gic exa mi nation showed the basic struc ture of valvular tissue . There was a central core of spongiosa material and, on each side of it , there was fairl y den se co llage nous tissue . Several cordi ike strands of coll agen inserted into the tissue (Fig . 6).
Comment Accessory tissue or redundant valvular tissue as a cause of ventricular outflow obstruction is a welldescribed occurrence in necrop sy serie s, 1- 3 It has been described as a cause of subpulmonary obstruction in complete transposition of the great vessels": 5 and in corrected transposition of the great vessels, 1 and as a cause of subaortic steno sis in the normall y oriented heart. 2, 6 Layman and Edwards " described the autopsy findings in a patient with transpo sition of the great vessels in whom the tricuspid septal leaflet orig inated from the
Volume 80 Number 2 August, 1980
Ventricular outflow obstruction
21 5
Fig. 6. Case 3. Photomicrograph of flap excised from the subpulmonary region. The basic structure is similar to that of valvular tissue. Two cordlike structures insert into it. (Elastic tissue stain; x 23.) rim of a ventricular septal defect, bulged into the outflow tract of the left ventricle, and thereby contributed to subpulmonary stenosis . In another series, Riemenschneider and associates" described two cases of complete transposition of the great vessels, in each of which a poorly defined filling defect in the left ventricular outflow tract was seen in ventriculograms . The anatomic and hemodynamic significance of these angiographic abnormalities had not been fully appreciated preoperatively . Both patients were treated with atrial septostomy but showed progressive clinical deterioration characteristic of left ventricular outflow tract obstruction. At autopsy, in each case, redundant valvular tissue was found arising from the septal tricuspid leaflet protruding through a ventricular septal defect into the left ventricular outflow tract and causing severe subpulmonary obstruction. Rastelli , Wallace , and Ongley' reported on the autopsy findings in a patient with situs inversus, transposition of the great vessels, ventricular septal defect, and subpulmonary stenosis who died following a Mustard procedure and pulmonary valvulotomy. Autopsy showed redundant fibrous tissue arising from the mitral valve, extending through the ventricular septal defect, and inserting into the anatomic right ventricle. That part of the tissue in the anatomic left ventricle and an additional band of redundant endocardial tissue contributed to subpulmonary obstruction . Subpulmonary obstruction caused by accessory tissue in three cases of corrected transposition of the great vessels was reported"by Levy and associates .' In one of their cases, angiocardiography at the age of 5 years was described as showing persistent narrowing beneath the
origin of the pulmonary trunk . Review of Fig . 5 of that paper reveals a crescent-shaped filling defect similar to those observed in our cases . At operation through a right-sided ventriculotomy, accessory tissue was seen bulging into the subpulmonary region of the anatomic left ventricle . Because the exact origin, structure, and function of this accessory tissue remained uncertain, the tissue was not removed. At autopsy, an umbrellalike formation of accessory tissue, attached by chordalike strands to pulmonary and atrioventricular valvular tissue and to the edges of a related ventricular septal defect, protruded into and obstructed the subpulmonary region of the right-sided anatomic left ventricle. In the third patient of Levy and associates, I an adult with corrected transposition of the great vessels, situs inversus, and atrial septal defect, angiography of the venous ventricle showed a filling defect in the outflow tract. At operation, the basis for this subpulmonary filling defect was specifically sought through a pulmonary arteriotomy. Yet it was not identified, presumably because it lay flattened against the atrioventricular leaflet from which it arose . The cusplike formation of accessory valvular tissue attached to the ventricular aspect of the anterior leaflet of the venous atrioventricular valve was clearly identified at autopsy. Sellers, Lillehei, and Edwards" described two cases in which, at autopsy, accessory tricuspid tissue was observed to protrude through a ventricular septal defect into the subaortic area, possibly causing subaortic obstruction. In a third case, multiple bellows-like masses of accessory tissue, attached to the anterior mitral leaflet, protruded into the left ventricle and, when expanded, caused subaortic stenosis . At operation,
The Journal of
216
Gomes et al,
these collections had evidently been collapsed and had not been identified. In another case of subaortic outflow obstruction caused by accessory tissue, that of MacLean, Culligan, and Kane," the tissue arose as a diverticulum from the ventricular surface of the anterior mitral leaflet, producing ball-valve obstruction in the left ventricular outflow tract. This obstruction was identified at operation and the responsible tissue was resected. Preoperative ventriculography had not been performed. In a case with tetralogy of Fallot, Neufeld and associates t described an accessory, flaplike process of the tricuspid valve that obstructed the ventricular septal defect. This gave rise to features like those in pulmonary stenosis with intact ventricular septum. In our cases, the basis for ventricular outflow obstruction was appreciated preoperatively. The unusual filling defects seen on angiography were felt to represent the cause of obstruction, impressions which were confirmed at operation. Surgical relief of the obstruction was obtained in each case. The roentgenographic appearance of obstruction caused by redundant tissue is unusual but appears to be relatively similar on ventriculography in our three cases and the other cases in the literature where adequate angiography has been available. 1-5 The angiocardiographic feature is that of a narrow, linear, or crescent-shaped defect in the zone corresponding to the site of obstruction.
Thoracic and Cardiovascular Surgery
Relief of ventricular outflow obstruction can be accomplished by resecting the accessory tissue. REFERENCES
2
3
4
5
6
7
Levy MJ, Lillehei CW, Elliott LP, Carey LS, Adams P Jr, Edwards JE: Accessory valvular tissue causing subpulmonary stenosis in corrected transposition of great vessels. Circulation 27:494-502, 1963 Sellers RD, Lillehei CW, Edwards JE: Subaortic stenosis caused by anomalies of the atrioventricular valves. J THORAC CARDIOVASC SURG 48:289-302, 1964 Layman TE, Edwards JE: Anomalies of the cardiac valves associated with complete transposition of the great vessels. Am J Cardiol 19:247-255, 1967 Rastelli GC, Wallace RB, Ongley PA: Complete repair of transposition of the great arteries with pulmonary stenosis. A review and report of a case corrected by using a new surgical technique. Circulation 39:83-95, 1969 Riemenschneider TA, Goldberg SJ, Ruttenberg HD, Gyepes MT: Subpulmonic obstruction in complete (d) transposition produced by redundant tricuspid tissue. Circulation 39:603-609, 1969 MacLean LD, Culligan JA, Kane DJ: Subaortic stenosis due to accessory tissue on the mitral valve. J THORAC CARDIOVASC SURG 45:382-388, 1963 Neufeld HN, McGoon DC, DuShane JW, Edwards JE: Tetralogy of Fallot with anomalous tricuspid valve simulating pulmonary stenosis with intact septum. Circulation 22:1083-1090, 1960
CARDIOVASC SURG
80:211-216, 1980
Accessory flaplike tissue causing ventricular outflow obstruction In three cases. two in children and one in a young adult. ventricular outflow obstruction was caused by a valvelike flap of accessory endocardial tissue. The angiocardiographic feature was that of a narrow. radiolucent. crescent-shaped or linear filling defect corresponding to the site of obstruction. Resection of the accessory tissue was performed in each case at the time of correction of associated conditions. In one case (a woman 20 years of age) the obstructing membrane was in the outflow tract of the right ventricle. and a ventricular septal defect was associated. In each of the other two cases the outflow tract of the left ventricle was the site of obstruction. In one (a /4-month-old boy). an ostium primum type of atrial septal defect and cleft mitral valve were associated; in the remaining case (a 7-year-old boy) complete transposition and ventricular septal defect were also present. In the latter case a Mustard procedure was performed and was followed by death. Successful results were obtained in the first two patients.
Antoinette S. Gomes, M.D., P. H. Nath, M.D., Amarjit Singh, M.D., Russell V. Lucas,
Jr.,
M.D., Kurt Amplatz, M.D., Demetre M. Nicoloff, M.D., and
Jesse E. Edwards, M.D., Minneapolis and St. Paul. Minn.
Ventricular outflow tract obstruction resulting from accessory endocardial tissue is uncommon. The anomaly may be recognized in ventriculograms, and relief of obstruction is possible through resection of the accessory tissue. Since the condition is uncommon and yet curable, it is our purpose to describe three cases in which the anomaly was identified angiocardiographically and treated surgically.
Case reports CAS E 1. A white, female subject with a cardiac murmur since birth and exertional cyanosis was first studied in 1955 at the University of Minnesota Hospitals at the age of 3 years and found to have a left-to-right shunt and pulmonary stenosis. A second cardiac catheterization in 1957 demonstrated the shunt to be at the ventricular level. The catheter could not be advanced into the pulmonary artery. No angiography was
From the Departmentsof Radiology. Pediatrics, Surgery and Pathology, University of Minnesota. Minneapolis; the Department of Pathology, United Hospitals-Miller Division, SI. Paul; and the Children's Health Center. Minneapolis, Minn. This study was supported by Public Health Service Research Grant 5 ROI HL05694 from the National Heart, Lung and Blood Institute, and by the Saint Paul Foundation, SI. Paul, Minn. Received for publication Nov. 27, 1979. Accepted for publication Jan. 14, 1980. Address for reprints: Jesse E. Edwards. M.D., Department of Pathology, United Hospitals-Miller Division, 125 W. College Ave., SI. Paul. Minn. 55102.
performed because of arrhythmias. The patient was lost to follow-up until 1972 when, at the age of 20 years, she presented with a spontaneous abortion. Physical examination revealed no cyanosis but there was bilateral clubbing of fingers. There was a Grade 4/6 pan systolic murmur at the left sternal border. Cardiac catheterization at this time revealed the right ventricular systolic pressure to be at systemic level, and there was a normal pressure in the pulmonary artery. A bidirectional shunt at the ventricular level was also identified. Angiography showed a crescent-shaped, linear filling defect in the outflow tract of the right ventricle (Fig. I). The patient was operated upon. When the right ventricle was opened, a large membranous ventricular septal defect and anomalous muscle bundles were found. The pulmonary valve was normal in appearance. A windsock-like flap was found in relation to the septal leaflet of the tricuspid valve and appeared to attach to abnormal chordae that inserted into the rim of the ventricular septal defect and to a nearby papillary muscle. This abnormal flap appeared to be capable of prolapsing into the outflow tract of the right ventricle and through the pulmonary valve orifice. The flap was excised and the ventricular septal defect closed. The patient has subsequently done well. Two months following the operation cardiac catheterization revealed the following pressures: right ventricle, 32/2-13 mm Hg; pulmonary trunk, 32/14 mm Hg. The excised flap was thin and cusplike. Histologically, it contained a core of loose connective tissue having the appearance of valvular spongiosa. To one side of this layer was a layer of dense compact collagen, and on the other side was a thinner layer composed of moderately delicate collagen (Fig. 2). CASE 2. A 14-month-old, black, male infant with intesti-
0022-5223/80/080211 +06$00.60/0 © 1980 The C. V. Mosby Co.
2I I
The Journal of
2 12
Gomes et al.
Thoracic and Cardiovascular Surgery
Fig. 2. Ca se I . Photomicrograph of flap excised surgically . There is a central core of loose, spongiosa-like tissue . On one side (I eft) is a den se layer of compact collagen; on the other side of the spongiosa-like layer is a thinner layer of connective tissue, the latter being composed of moderately delicate co llagen. (Elastic tissue stain; x 35 .)
Fig. 1. Case I . Right ventriculogram in (a) frontal view and lateral view. Beneath the pulmonary valve there is a crescent-shaped, linear filling defect (arrows) . More superiorIy, the pulmonary valve is normal. There is no evidence of a right-to-left shunt.
( b)
nal malrot ation. obstructing duodenal diaphragm, and cardiomegaly was examined at the Children 's Health Center of Minneapolis when he presented with congestive heart failure and pneumonia . A cardiac murmur had been present since birth . Physical examination re vealed a tachypneic, lethargic infant. There was a palpable thrill and an increased S2' A Grade 4/6 holosystolic murmur was heard over the entire precordium and was loudest along the left sternal border. An S, was pre sent. The electrocardiogram revealed left axis deviation and severe biventricular hypertrophy . Cardiac catheterization revealed continuity of the inferior vena cava with the right azygo s ve in. The catheter then passed from the right atrium , acros s the tricuspid valve. into the right ventricle and from the right ventricle to the left ventricle. Right atrial pre ssures were norm al and the right ventricular systo lic pressure was 70 to 80 mm Hg . The left ventricular apical systolic pressure was markedly elevated . being 160 mm Hg. Retrograde arterial catheterization revealed discrete obstruction in the sub aortic region of the left ventricle . A peakto-peak systolic gradient of 90 mm Hg was present between the left ventricular apex and left ventricular outflow tract. One month later , a seco nd card iac catheterization showed a persistent left superior vena cava draining into the coronary sinus
Volume 80 Number 2
Ventricular outflow obstruction
21 3
August. 1980
Fig. 4. Case 2. Photomicrograph of excised . valvelike flap of tissue from subaortic region. One extremity is nodular and composed of loose connective tissue . (Hematoxylin and eosin; X7 .)
Fig. 3. Case 2. Left ventriculography in frontal view . a . Systolic phase. There is a goose-neck deformity of the left ventricle and beneath the aortic valve is a crescent-shaped filling defect (arrows). The mitral valve is competent. b. Diastolic phase . A goose-neck deformity is apparent ; the subaortic filling defect noted in the systolic phase is not visualized .
and a left-to-right shunt at the atrial level. Angiography revealed an enlarged left ventricle with a clear-cut. goose-neck deformity . There was a discrete , crescent-shaped, membranous obstruction about 0.5 cm below a normal-appearing aortic valve (Fig . 3). The membrane appeared to be attached to the anterior portion of the mitral valve on one side and the ventricular septum on the other. There was no mitral regurgitation . At operation, an ostium prim urn atrial septal defect with cleft mitral valve was found . In the subarotic region of the left ventricle a nodular. f1aplike structure , measuring about 4 by 1.2 em , was found attached to the ventricular aspect of the anterior mitral leaflet. This was considered to have caused the subaortic stenosi s and was excised . A mitral valve annuloplasty was performed and a pericardial patch was used to close the atrial septal defect. Histologic examination of the excised tissue revealed a valvelike structure . One extremity showed nodular thickening formed by loose cellular connective tissue (Fig. 4) . Because of postoperative complications . including the presence of mitral insufficiency. two postoperative invasive studies were done and showed no signs of significant residual subaortic stenosi s.
The Journal of
2I4
Gomes et al.
Thorac ic and Cardiovascular Surgery
A p
Fig. 5. Case 3. Right ventric ulogram. a, Frontal view. There is op aci fication of both gre at vessel s. There is a linear , membranous filling defect in the subpulmo nary are a ( arro ws) . b, Lateral view . There is op acific ation of both ventricles and both gre at vessels . Beneath the pulm onary valve is a platelike, thin filling defect (a rro ws) s imilar to the one observed in the frontal view . A. Aorta . P , Pulm on ary trunk. Two months postoperati vely the left ventricular pressu res were 10010-16 mm Hg and the asce nd ing ao rtic pressu res were 93/ 60 mm Hg . At the age of 3 Y2 years the pressures we re as follows : left ventricle, 12017-10 mm Hg; ascending aorta , Il l/56 mm Hg . CAS E 3. A white, male infant was cyanotic at birth. When he was 7 weeks of age diagnoses of transpo sition of the great vessels , subpulmo nary stenosis, and ventricular septal defect were made at the University of Minn esota Hospitals. Transvenous atria l septostomy (Rashkind pro cedure) was followed by improvement in degree of cyanosis. Cardi ac catheterization at the age of 2 yea rs re vealed equal systolic pressur es in the anatomic rig ht ventricl e and aorta . Th e pressure in the pulmonary trunk was 20/6 mm Hg. During pullback to the left (pulmonary) ventricle, the pressure rose to 40 mm Hg sys to lic in the subpulmonary reg ion and 90/7 mm Hg in the body of the ventricle . The ca theter had reach ed the pulmonary arter y by passage from right atrium to left atrium to anatom ic left (pulmo nary) ventricle . Thi s indi cated that the grad ient was indeed subpulmonary and not ac ros s the ventricular septal defe ct. An ang iog ram performed by injection into the anatomic right ventricle show ed op acifica tio n of both ventricles . A radio lucent filling defect was observed ex tend ing from the area of the m itral valve into the subpulmo nary outflow regi on of the left ventricle (Fig. 5). Op eration was initially deferred because of the child's stable co ndition . Howe ver , at the age o f 7 yea rs the child was restudie d and operated upon. At operatio n, prior to the perform ance of a Mu stard proce dure , right ventriculotomy was done. Through the ventricular
septal defect a flap was ob served in the left ventricular outflow tract ; this was excised and the ventric ular septal defec t was closed. Postope rat ively , the child manifested co mplications associa ted with obstruction to pulm onary venous flow by the baffle of the Mustard procedure and d ied in the early postoperat ive peri od . At autopsy, the preoperati ve anatomic diagnoses were co nfirmed . No residual tissue was found in the left vent ricular outflow tract. The excised tissue was represented by a valvelike flap. Histolo gic exa mi nation showed the basic struc ture of valvular tissue . There was a central core of spongiosa material and, on each side of it , there was fairl y den se co llage nous tissue . Several cordi ike strands of coll agen inserted into the tissue (Fig . 6).
Comment Accessory tissue or redundant valvular tissue as a cause of ventricular outflow obstruction is a welldescribed occurrence in necrop sy serie s, 1- 3 It has been described as a cause of subpulmonary obstruction in complete transposition of the great vessels": 5 and in corrected transposition of the great vessels, 1 and as a cause of subaortic steno sis in the normall y oriented heart. 2, 6 Layman and Edwards " described the autopsy findings in a patient with transpo sition of the great vessels in whom the tricuspid septal leaflet orig inated from the
Volume 80 Number 2 August, 1980
Ventricular outflow obstruction
21 5
Fig. 6. Case 3. Photomicrograph of flap excised from the subpulmonary region. The basic structure is similar to that of valvular tissue. Two cordlike structures insert into it. (Elastic tissue stain; x 23.) rim of a ventricular septal defect, bulged into the outflow tract of the left ventricle, and thereby contributed to subpulmonary stenosis . In another series, Riemenschneider and associates" described two cases of complete transposition of the great vessels, in each of which a poorly defined filling defect in the left ventricular outflow tract was seen in ventriculograms . The anatomic and hemodynamic significance of these angiographic abnormalities had not been fully appreciated preoperatively . Both patients were treated with atrial septostomy but showed progressive clinical deterioration characteristic of left ventricular outflow tract obstruction. At autopsy, in each case, redundant valvular tissue was found arising from the septal tricuspid leaflet protruding through a ventricular septal defect into the left ventricular outflow tract and causing severe subpulmonary obstruction. Rastelli , Wallace , and Ongley' reported on the autopsy findings in a patient with situs inversus, transposition of the great vessels, ventricular septal defect, and subpulmonary stenosis who died following a Mustard procedure and pulmonary valvulotomy. Autopsy showed redundant fibrous tissue arising from the mitral valve, extending through the ventricular septal defect, and inserting into the anatomic right ventricle. That part of the tissue in the anatomic left ventricle and an additional band of redundant endocardial tissue contributed to subpulmonary obstruction . Subpulmonary obstruction caused by accessory tissue in three cases of corrected transposition of the great vessels was reported"by Levy and associates .' In one of their cases, angiocardiography at the age of 5 years was described as showing persistent narrowing beneath the
origin of the pulmonary trunk . Review of Fig . 5 of that paper reveals a crescent-shaped filling defect similar to those observed in our cases . At operation through a right-sided ventriculotomy, accessory tissue was seen bulging into the subpulmonary region of the anatomic left ventricle . Because the exact origin, structure, and function of this accessory tissue remained uncertain, the tissue was not removed. At autopsy, an umbrellalike formation of accessory tissue, attached by chordalike strands to pulmonary and atrioventricular valvular tissue and to the edges of a related ventricular septal defect, protruded into and obstructed the subpulmonary region of the right-sided anatomic left ventricle. In the third patient of Levy and associates, I an adult with corrected transposition of the great vessels, situs inversus, and atrial septal defect, angiography of the venous ventricle showed a filling defect in the outflow tract. At operation, the basis for this subpulmonary filling defect was specifically sought through a pulmonary arteriotomy. Yet it was not identified, presumably because it lay flattened against the atrioventricular leaflet from which it arose . The cusplike formation of accessory valvular tissue attached to the ventricular aspect of the anterior leaflet of the venous atrioventricular valve was clearly identified at autopsy. Sellers, Lillehei, and Edwards" described two cases in which, at autopsy, accessory tricuspid tissue was observed to protrude through a ventricular septal defect into the subaortic area, possibly causing subaortic obstruction. In a third case, multiple bellows-like masses of accessory tissue, attached to the anterior mitral leaflet, protruded into the left ventricle and, when expanded, caused subaortic stenosis . At operation,
The Journal of
216
Gomes et al,
these collections had evidently been collapsed and had not been identified. In another case of subaortic outflow obstruction caused by accessory tissue, that of MacLean, Culligan, and Kane," the tissue arose as a diverticulum from the ventricular surface of the anterior mitral leaflet, producing ball-valve obstruction in the left ventricular outflow tract. This obstruction was identified at operation and the responsible tissue was resected. Preoperative ventriculography had not been performed. In a case with tetralogy of Fallot, Neufeld and associates t described an accessory, flaplike process of the tricuspid valve that obstructed the ventricular septal defect. This gave rise to features like those in pulmonary stenosis with intact ventricular septum. In our cases, the basis for ventricular outflow obstruction was appreciated preoperatively. The unusual filling defects seen on angiography were felt to represent the cause of obstruction, impressions which were confirmed at operation. Surgical relief of the obstruction was obtained in each case. The roentgenographic appearance of obstruction caused by redundant tissue is unusual but appears to be relatively similar on ventriculography in our three cases and the other cases in the literature where adequate angiography has been available. 1-5 The angiocardiographic feature is that of a narrow, linear, or crescent-shaped defect in the zone corresponding to the site of obstruction.
Thoracic and Cardiovascular Surgery
Relief of ventricular outflow obstruction can be accomplished by resecting the accessory tissue. REFERENCES
2
3
4
5
6
7
Levy MJ, Lillehei CW, Elliott LP, Carey LS, Adams P Jr, Edwards JE: Accessory valvular tissue causing subpulmonary stenosis in corrected transposition of great vessels. Circulation 27:494-502, 1963 Sellers RD, Lillehei CW, Edwards JE: Subaortic stenosis caused by anomalies of the atrioventricular valves. J THORAC CARDIOVASC SURG 48:289-302, 1964 Layman TE, Edwards JE: Anomalies of the cardiac valves associated with complete transposition of the great vessels. Am J Cardiol 19:247-255, 1967 Rastelli GC, Wallace RB, Ongley PA: Complete repair of transposition of the great arteries with pulmonary stenosis. A review and report of a case corrected by using a new surgical technique. Circulation 39:83-95, 1969 Riemenschneider TA, Goldberg SJ, Ruttenberg HD, Gyepes MT: Subpulmonic obstruction in complete (d) transposition produced by redundant tricuspid tissue. Circulation 39:603-609, 1969 MacLean LD, Culligan JA, Kane DJ: Subaortic stenosis due to accessory tissue on the mitral valve. J THORAC CARDIOVASC SURG 45:382-388, 1963 Neufeld HN, McGoon DC, DuShane JW, Edwards JE: Tetralogy of Fallot with anomalous tricuspid valve simulating pulmonary stenosis with intact septum. Circulation 22:1083-1090, 1960