Diagnostic Radiology
•
Congenital Diverticulum of the Right and Left Ventricles 1
•
George T. Bandow, M.D., George G. Rowe, M.D., and Andrew B. Crummy, M.D. An asymptomatic congenital cardiac diverticulum was seen on a routine chest film in a 45-year-old man. Rightatrial and left ventricular cineangiography showedthat the diverticulum communicated independently with both ventricles. There was no evidence of heart failure, arrhythmia, or mural thrombus, and surgerywas not deemednecessary. INDEX TERM:
Heart, abnormalities
Radiology 117:19-20, October 1975
• • A N ASYMPTOMATIC congenital cardiac diverticulum was seen H originating from both ventricles on a routine chest film. Its unique nature was defined by cineangiography. CASE REPORT A 45-year-old man without personal or family history of heart disease fell and sustained a soft-tissue injury. Physical examination and a 12-lead electrocardiogram were normal. A cardiac series showed a normal-sized heart with an abnormal contour which suggested a ventricular aneurysm (Fig. 1). Cardiac catheterization showed normal pressures, dye curves, and indices of cardiac function. Right atrial cineangiography demonstrated half of a diverticulum arising anterolaterally from the right ventricular outflow tract (Fig. 2); the other half of the same diverticulum filled from the left ventricle. Left ventriculography demonstrated that the diverticulum arose anterolaterally from a broad base. In diastole, the trabeculated left diverticular cavity measured 4 X 2.8 em and no filling defects were identified (Fig. 3, A). Ventricular systole produced nearly complete emptying of the diverticulum through a sharply constricting orifice which remained open (Fig. 3, B). Normal coronary arteriograms showed the left anterior descending artery arching over the apex of the diverticulum (Fig. 4). We concluded that the patient had an asymptomatic congenital diverticulum of the right and left ventricles without shunt, thrombus, or muscular dysfunction.
DISCUSSION Diverticula of the ventricle are rare (1) and difficult to differentiate from aneurysms. Treistman et al. (6) define a congenital aneurysm as an isolated asymptomatic lesion at the apex or base of the heart, while an extension of the ventricular cavity through the apex associated with a midline thoracoabdominal defect is considered a diverticulum. The point of connection to the ventricle is usually narrow. Until recently, outpouchings of the ventricle were classified by most authors as diverticula regardless of their specific anatomy, the age of the patient at time of diagnosis, or association with other congenital anomalies, while the term aneurysm was reserved for acquired defects of the ventricular wall. "Congenital" diverticula may be found in either ventricle and usually occur in early childhood as part of the syndrome of multiple cardiac and thoraco-abdominal defects (2). A different group of diverticula are found in Africans, which are distinguished by their clinical course, position, and association with vascular disease. The present case was unique, since the angiographic appearance revealed characteristics of both a diverticulum and an aneurysm: it was biventricular and presented as an isolated anomaly in an asymptomatic adult. We found fewer than 50 cases of congenital diverticulum of the left ventricle in our review of the literature. They have been seen in patients as old as 38 years of age but are typically recognized in infants who have died suddenly because of rupture of the diverticulum, congestive. heart failure, or complications of associated congenital defects (4-6). Some asymptomatic diverticula are discovered incidentally. Equal numbers occur as an isolated defect, usually in the left ventricular apex, or as part of the syndrome of antero-midline thoraco-abdominal defects. Only four right-ventricle diverticula have been reported (3, 4). These were associated with patent ductus arteriosus, truncus arteriosus, or ventricular septal defect but not with other midline congenital anomalies. One patient died of spontaneous rupture and the others were operated on at the time of recognition. The cases are too few to tell whether a diverticulum in the low pressure system carries the same risk of rupture as one on the left side. In the present case the diverticu-
Fig.1. A and B. Postero-anterior (A) and lateral (B) films of the chest show a barium-filled esophagus and a normal-sized heart. In the frontal projection, the superior portion of the left heart border is prominent, while in the lateral view the abnormality is noted to extend anteriorly. 1 From the Departments of Medicine (G.T.B., G.G.R.) and Radiology (A. B. C.), University of Wisconsin Medical School, Madison, Wise. Accepted for publication in March 1975. sjh
19
GEORGE T. SANDOW AND OTHERS
20
October1975
Fig. 2. A and B. Frame from a cineangiogram taken in the right anterior oblique position (A). The right ventricle (RV) is in diastole and the diverticulum (D) is full. In the systolic frame (B), the diverticulum has contracted behind the right ventricular outflow tract and is no longer seen. PA = pulmonary artery. C. Cineangiographic frame from the postero-anterior projection shows the right ventricle (RV) and diverticulum (D) in systole. In this view the diverticulum overlies the pulmonary artery (PA) and is harder to delineate. Fig. 3. A and B. Left ventriculograms in the 45° right anterior oblique position during diastole (A) and systole (B). A broad-based diverticulum (DIV) is seen arising from the left ventricle (L V)' During systole there is nearly complete emptying via a narrow orifice. The left anterior descending coronary artery (AD) passes anteriorly over the diverticulum. Fig. 4. Left coronary arteriogram in the 30° right anterior oblique position. The proximal portion of the anterior descending coronary artery (AD) is bowed upward. All vessels are free of disease. CIR = circumflex coronary artery.
lum communicated independently with both right and left ventricles. Rupture and peripheral embolization from mural thrombi are known complications; when the diverticulum is in the subvalvular position, aortic and mitral insufficiency can also occur (1). An overall mortality of 7 % has been reported; however, surgical correction has improved the prognosis (7). When the diverticulum is thin-walled, connected to the ventricle by a narrow neck, seen as part of the syndrome of combined congenital abnormalities, contains thrombi, or is associated with congestive heart failure, the indication for surgery is clear. Our patient's diverticulum was bicameral, thick-walled and emptied well with systole. Since he was asymptomatic at age 45, surgery was not done.
heart. Part of the syndrome of congenital cardiac and midline thoracic and abdominal defects. Am J Cardiol 24:580-583, Oct 1969 3. Farnsworth PB, Lefkowitz M, Shehadi W, et al: Spontaneous rupture of fibrous diverticulum of the right ventricle. Occurrence in an infant with persistent truncus arteriosus. Am J Dis Child 123: 248-250, Mar 1972 4. Kavanagh-Gray 0: Right ventricular diverticulum. Can Med Assoc J 105:1055-1056, 20 Nov 1971 5. Pettersson G, Bergstrom T: A case of ruptured diverticulum of the left ventricle with hemopericardium in a neonate, treated successfully by surgery. Scand J Thorac Cardiovasc Surg 3:203-206,
1969 6. Treistman B, Cooley DA, Lufschanowski R, et al: Diverticulum or aneurysm of left ventricle. Am J Cardiol 32:119-123, Jul
1973 7. Verger P, Bricaud H, Fontan F, et al: Le diverticule congEmital du ventricule gauche: propos d'un cas opere avec sucoss. Ann Pediatr 17:24-30, 2 Jan 1970
a
REFERENCES 1. Chesler E, Tucker RBK, Barlow JB: Subvalvular and apical left ventricular aneurysms in the Bantu as a source of systemic emboiL CirculatiOn 35: 1156-1162, Jun 1967 2. Edgett JW Jr, Nelson WP, Hall RJ, et al: Diverticulum of the
Andrew B. Crummy, M.D. University of Wisconsin Hospitals 1300 University Ave. Madison, Wisc. 53706
•
Congenital Diverticulum of the Right and Left Ventricles 1
•
George T. Bandow, M.D., George G. Rowe, M.D., and Andrew B. Crummy, M.D. An asymptomatic congenital cardiac diverticulum was seen on a routine chest film in a 45-year-old man. Rightatrial and left ventricular cineangiography showedthat the diverticulum communicated independently with both ventricles. There was no evidence of heart failure, arrhythmia, or mural thrombus, and surgerywas not deemednecessary. INDEX TERM:
Heart, abnormalities
Radiology 117:19-20, October 1975
• • A N ASYMPTOMATIC congenital cardiac diverticulum was seen H originating from both ventricles on a routine chest film. Its unique nature was defined by cineangiography. CASE REPORT A 45-year-old man without personal or family history of heart disease fell and sustained a soft-tissue injury. Physical examination and a 12-lead electrocardiogram were normal. A cardiac series showed a normal-sized heart with an abnormal contour which suggested a ventricular aneurysm (Fig. 1). Cardiac catheterization showed normal pressures, dye curves, and indices of cardiac function. Right atrial cineangiography demonstrated half of a diverticulum arising anterolaterally from the right ventricular outflow tract (Fig. 2); the other half of the same diverticulum filled from the left ventricle. Left ventriculography demonstrated that the diverticulum arose anterolaterally from a broad base. In diastole, the trabeculated left diverticular cavity measured 4 X 2.8 em and no filling defects were identified (Fig. 3, A). Ventricular systole produced nearly complete emptying of the diverticulum through a sharply constricting orifice which remained open (Fig. 3, B). Normal coronary arteriograms showed the left anterior descending artery arching over the apex of the diverticulum (Fig. 4). We concluded that the patient had an asymptomatic congenital diverticulum of the right and left ventricles without shunt, thrombus, or muscular dysfunction.
DISCUSSION Diverticula of the ventricle are rare (1) and difficult to differentiate from aneurysms. Treistman et al. (6) define a congenital aneurysm as an isolated asymptomatic lesion at the apex or base of the heart, while an extension of the ventricular cavity through the apex associated with a midline thoracoabdominal defect is considered a diverticulum. The point of connection to the ventricle is usually narrow. Until recently, outpouchings of the ventricle were classified by most authors as diverticula regardless of their specific anatomy, the age of the patient at time of diagnosis, or association with other congenital anomalies, while the term aneurysm was reserved for acquired defects of the ventricular wall. "Congenital" diverticula may be found in either ventricle and usually occur in early childhood as part of the syndrome of multiple cardiac and thoraco-abdominal defects (2). A different group of diverticula are found in Africans, which are distinguished by their clinical course, position, and association with vascular disease. The present case was unique, since the angiographic appearance revealed characteristics of both a diverticulum and an aneurysm: it was biventricular and presented as an isolated anomaly in an asymptomatic adult. We found fewer than 50 cases of congenital diverticulum of the left ventricle in our review of the literature. They have been seen in patients as old as 38 years of age but are typically recognized in infants who have died suddenly because of rupture of the diverticulum, congestive. heart failure, or complications of associated congenital defects (4-6). Some asymptomatic diverticula are discovered incidentally. Equal numbers occur as an isolated defect, usually in the left ventricular apex, or as part of the syndrome of antero-midline thoraco-abdominal defects. Only four right-ventricle diverticula have been reported (3, 4). These were associated with patent ductus arteriosus, truncus arteriosus, or ventricular septal defect but not with other midline congenital anomalies. One patient died of spontaneous rupture and the others were operated on at the time of recognition. The cases are too few to tell whether a diverticulum in the low pressure system carries the same risk of rupture as one on the left side. In the present case the diverticu-
Fig.1. A and B. Postero-anterior (A) and lateral (B) films of the chest show a barium-filled esophagus and a normal-sized heart. In the frontal projection, the superior portion of the left heart border is prominent, while in the lateral view the abnormality is noted to extend anteriorly. 1 From the Departments of Medicine (G.T.B., G.G.R.) and Radiology (A. B. C.), University of Wisconsin Medical School, Madison, Wise. Accepted for publication in March 1975. sjh
19
GEORGE T. SANDOW AND OTHERS
20
October1975
Fig. 2. A and B. Frame from a cineangiogram taken in the right anterior oblique position (A). The right ventricle (RV) is in diastole and the diverticulum (D) is full. In the systolic frame (B), the diverticulum has contracted behind the right ventricular outflow tract and is no longer seen. PA = pulmonary artery. C. Cineangiographic frame from the postero-anterior projection shows the right ventricle (RV) and diverticulum (D) in systole. In this view the diverticulum overlies the pulmonary artery (PA) and is harder to delineate. Fig. 3. A and B. Left ventriculograms in the 45° right anterior oblique position during diastole (A) and systole (B). A broad-based diverticulum (DIV) is seen arising from the left ventricle (L V)' During systole there is nearly complete emptying via a narrow orifice. The left anterior descending coronary artery (AD) passes anteriorly over the diverticulum. Fig. 4. Left coronary arteriogram in the 30° right anterior oblique position. The proximal portion of the anterior descending coronary artery (AD) is bowed upward. All vessels are free of disease. CIR = circumflex coronary artery.
lum communicated independently with both right and left ventricles. Rupture and peripheral embolization from mural thrombi are known complications; when the diverticulum is in the subvalvular position, aortic and mitral insufficiency can also occur (1). An overall mortality of 7 % has been reported; however, surgical correction has improved the prognosis (7). When the diverticulum is thin-walled, connected to the ventricle by a narrow neck, seen as part of the syndrome of combined congenital abnormalities, contains thrombi, or is associated with congestive heart failure, the indication for surgery is clear. Our patient's diverticulum was bicameral, thick-walled and emptied well with systole. Since he was asymptomatic at age 45, surgery was not done.
heart. Part of the syndrome of congenital cardiac and midline thoracic and abdominal defects. Am J Cardiol 24:580-583, Oct 1969 3. Farnsworth PB, Lefkowitz M, Shehadi W, et al: Spontaneous rupture of fibrous diverticulum of the right ventricle. Occurrence in an infant with persistent truncus arteriosus. Am J Dis Child 123: 248-250, Mar 1972 4. Kavanagh-Gray 0: Right ventricular diverticulum. Can Med Assoc J 105:1055-1056, 20 Nov 1971 5. Pettersson G, Bergstrom T: A case of ruptured diverticulum of the left ventricle with hemopericardium in a neonate, treated successfully by surgery. Scand J Thorac Cardiovasc Surg 3:203-206,
1969 6. Treistman B, Cooley DA, Lufschanowski R, et al: Diverticulum or aneurysm of left ventricle. Am J Cardiol 32:119-123, Jul
1973 7. Verger P, Bricaud H, Fontan F, et al: Le diverticule congEmital du ventricule gauche: propos d'un cas opere avec sucoss. Ann Pediatr 17:24-30, 2 Jan 1970
a
REFERENCES 1. Chesler E, Tucker RBK, Barlow JB: Subvalvular and apical left ventricular aneurysms in the Bantu as a source of systemic emboiL CirculatiOn 35: 1156-1162, Jun 1967 2. Edgett JW Jr, Nelson WP, Hall RJ, et al: Diverticulum of the
Andrew B. Crummy, M.D. University of Wisconsin Hospitals 1300 University Ave. Madison, Wisc. 53706
