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Diverticulum of the heart
Diverticulum
of the Heart
Part of the Syndrome of Congenital Midline Thoracic JOSEPH W. ROBERT J. HALL,
EDGETT, JR.,
and Abdominal
LT. COL., Mc,t
WILLIAM
E. FISHBACK,
COL., MC, MALCOLM
LVashington,
Cardiac and Defects*
P. NELSON,
LT. COL., MC,
MAJ., MC and EDWARD D.
J.
JAHNKE,
COL., MC
C.
A case of coexistent congenital midline thoracoabdominal and cardiac defects is presented. The cardiac lesions consisted of a large ventricular septal defect and a muscular diverticulum of the left ventricle. Refractory congestive heart failure was controlled by banding of the pulmonary artery, and the muscular diverticulum was resected. Such cases are rare but important since the defects are treatable. The clinical features, diagnosis, prognosis and treatment are discussed.
A
SYNDROME
OF ASSOCIATED
CONGENITAL
and 100 in the legs. Pulse rate was lbO/min. Respiratory rate was 50/min., and there was flaring of the alae nasae. The chest was symmetrically formed with an extremely wide angle between the lower rib margin and the sternum. The sternal length appeared abnormally short and at its distal end was a 2 by 2 cm. bluish, pulsatile mass covered by a thin, translucent skin (Fig. 1). Its pulsations were synchronous with precordial activity, and pressure on the mass provoked frequent premature ventricular contractions (Fig. 2). Percussion of the cardiac borders revealed dullness extending about 4 cm. on both sides of the low sternum, and cardiac pulsations could be palpated in the same zone. On auscultation a grade 3/6 holosystolic murmur was heard with maximal intensity in the fourth intercostal space along the left sternal border. Two components of the second sound were present, but it was not possible to define their respiratory relation. An exaggerated third heart sound and middiastolic flow rumble were present at the apex. The lungs were normal. The liver was enlarged, with its lower edge palpable 3 cm. below the right costal margin. An electrocardiogram showed sinus tachycardia and biventricular hypertrophy. The frontal plane P vector suggesting normal atria1 situs and route of was +60°, atrial depolarization. Chest roentgenograms showed an enlarged cardiac silhouette with dextroposition. There was moderate increase in the pulmonary vascularity. Cardiac Catheterization and Angiocardiography: The child was considered to have congestive heart failure,
DE-
diverticulum, intracardiac defects, and midline thoracoabdominal defects was first reported by Cantrell et al.’ Because of the infrequent occurrence of this potentially lethal syndrome, specific guidelines regarding its diagnosis and treatment are not firmly established. The following case is presented to review briefly the clinical features of this complex lesion and to discuss the diagnosis, prognosis, and surgical treatment of this entity. FECTS
including
muscular
ventricular
CASE REPORT A 9 day old Caucasian male child was referred to Walter Reed General Hospital for cardiac evaluation. He was the product of a full term, uncomplicated gestation and spontaneous delivery. At birth an umbilical hernia and an omphalocele were noted and these defects were repaired on the first day of life. At surgery the omphalocele was found to contain loops of bowel. The omphalocele was excised and the umbilical hernia repaired by apposing the fascia of the rectus abdominus muscles. No defects of the diaphragm were identified. On the third postoperative day, the appearance of a cardiac murmur and a pulsatile epigastric mass prompted referral to this hospital. Physical examination revealed a well nourished Caucasian infant with mild respiratory distress. Blood pressure (flush technic) was 90 mm. Hg in the arms
* From the Cardiology and Thoracic Surgical Services, Walter Reed General Hospital, Washington, D. C. Manuscript received June 7, 1968, accepted February 28, 1969. t Present address and address for reprints: Joseph W. Edgett, Jr., LT. COL., MC, Department of the Army, Fitzsimons General Hospital, Denver, Colo. 80240. 580
THE
AMERICAN
JOURNAL
OF CARDIOLOGY
Diverticulum
and administration of digitalis and a diuretic resulted in some improvement. On the ninth hospital day (at age 18 days), cardiac catheterization and biplane angiocardiography were performed with the following findings: dextroposition of the heart, normal atria1 situs, a ventricular septal defect and a large left to right intracardiac shunt (pulmonary to systemic flow ratio greater than 5 : 1). Angiocardiography revealed a normally sized and placed pulmonary artery and left atrium. With opacification of the left ventricle a 2 by 2 cm. diverticulum was seen extending from the apex of the left ventricle (Fig. 3). The left to right intracardiac shunt was demonstrated by the recirculation of contrast material through the pulmonary artery. Injection of contrast material into the root of the aorta showed no evidence of a patent ductus arteriosus. Surgical Treatment: Because of persistent cardiac decompensation, regarded as primarily due to the magnitude of the left to right intracardiac shunt, the patient was considered a candidate for banding of the
Figure 2. VOLUME
24,
OCTOBER
1969
of the Heart
581
‘Ihis \vas accomplished without pulmonary artery. complication on the fifteenth hospital day, and the clinical signs of congestive heart failure disappeared. Surgical repair of the ventricular diverticulum was planned as a second procedure, so that optimal cardiac compensation might be achieved prior to this operation. On the thirty-fifth hospital day (at age 44 days) resection of the diverticulum was accomplished. At operation, the following defects were identified: a foreshortened sternum and a defect in the inferior pericardium through which a muscular diverticulum of the apex of the left ventricle passed to present in the epigastrium. The diaphragm was intact and normal. 7’he diverticulum was resected, and the pericardium closed. The patient’s postoperative course was uncomplicated and he was discharged from the hospital at age 58 days. He was seen in follow-up at 6 months of age. Growth and development had been slow but definite and there had been no intercurrent evidence of cardiac decompensation. Continued observation is
Continuous rhythm strip showing effect of pressure on the epigastric
mass.
Edgett et al.
582
Fil[ure 3. An,&mzrdio.pxn. A, frontal virw; B, lateral vkw. site of ventricular diverticulum.
planned with ultimate closure of the ventricular septal defect and removal of the constricting pulmonary artery band.
DISCUSSION Diverticulum and aneurysm of the ventricle are terms that have, in the past, been used interchangeably. The cause may be a congenital defect or an acquired lesion. Of the defects considered to be of congenital origin, Chesler et al2 had proposed a division into two groups: muscular and fibrous diverticula of the left ventricle. The congenital fibrous variety differ from the muscular diverticula in several ways: (1) they never occur with an associated midline thoracoabdominal defect, (2) they usually communicate with the left ventricle at the level of the mitral annulus, and (3) the majority of known cases have occurred in Negroes. Muscular diverticula of the ventricle are distinctly different and are the subject of this report. Muscular diverticulum of the ventricle was first reported in 1866.3 Since that time, 25 cases have been reported.‘*4-14 Although it was initially considered a form of ectopia cordis, Cantrell et al.’ in 1958 proposed that the heart was not ectopic and that muscular ventricular diverticulum was a part of a group of congenital defects, including (1) malrotation of the heart with dextroposition; (2) intracardiac anomalies including ventricular septal defects; (3) a deficient lower sternum; (4) an anterior diaphragmatic defect; and (5) midline abdominal defects with diastasis recti and umbilical hernias. Clinical Features: This complex of congenital defects usually presents in the newborn as a pulsatile epigastric mass with varying midline
Arrows designate
abdominal and thoracic defects. There is no apparent sex predilection. The spectrum of midline thoracoabdominal defects extends from an isolated umbilical hernia to defects with a foreshortened sternum, absence of the anterior one third of the diaphragm, diastasis recti and an omphalocele. Intracardiac defects have consisted of ventricular septal defects or tricuspid atresia. Pericardial defects are usually over the inferior aspect of the heart. The ventricular diverticula arise most frequently from the apex of the left ventricle, but have projected from the right ventricle and in 1 case from both ventricles. Diagnosis: Because of the multiplicity of congenital defects, anatomic clarification is mandatory before formulating a plan of surgical correction. Cardiac catheterization should be accomplished if a coexisting intracardiac defect is suspected and will allow a specific diagnosis in most cases. The ventricular diverticulum can be clearly delineated by angiocardiography as demonstrated in our case. Percutaneous injection of the contrast material directly into the lumen of the diverticulum has been suggested but, in our opinion, would seem an unwarranted risk. Prognosis: The importance of early treatment is emphasized by the fact that in 14 of the reported cases the patients died in the first four years of life, with 9 deaths occurring in the first two months of life. The cause of death has been attributed variously to congestive heart failure, diverticulum rupture, or associated congenital defects. Lowe et al.” speculated that rupture is secondary to the excessive pressure developed in the cavity of the diverticulum. The pressure is THE AMERICAN
JOURNAL
OF
CARDIOLOGY
Diverticulum clc\.t,lopcd because contraction of the ventricular wall, which precedes contraction of the diverticulun~, occludes the opening of the diverticulum and, with its subsequent contraction, the pressure within the diverticulum is exerted against a closed orifice. LSurgical Correction: Treatment of the ventricular diverticulum has been accomplished in two ways. The diverticulum may be replaced in the perirardial sac and the defect in this membraneclosed. The alternate method consists of resection of the diverticulum.‘5 To date, 13 patients have been operated upon. Nine of these had resection, 1 replacement, and 3 died at operation. Of the latter 3, 1 experienced cardiac arrest shortly after the incision was made and could 1 experienced cardiac not be resuscitated,’ arrest after resection of the diverticulum and could not be resuscitated,’ and the final patient, who had coexisting tricuspid atresia,2 died several hours after a subclavian to pulmonary artery anastomosis. The preferable approach would seem to be resection. REFERENCES 1. CANTRELL, J. R., HALLER, J. A. and RAVITCH, M. M. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg., Gynec. &? Obst., 107:603, 1958. 2. CHESLER, M. B., TUCKER, R. B. K. and BARLOW, Subvalvular and apical left ventricular J. B. aneurysms in the Bantu as a source of systemic emboli. Circulation, 35:1156, 1967.
VOLUME 24, OCTOBER 1969
of the Heart
S83
3. ABRAHAM&D. G., BARTON,C. .I., COCKSHO.,“,‘,W. P., EDINGTON. G. M. and WEAVF.R, E. J. M. i\nnular subvalvular left ventricular aneurysms. Qual-t..J. Med., 31:345, 1962. 4. SKAPINKER, S. Diverticulum of the left ventricle of the heart. Arch. Surg., 631629, 1951. 5. SWUER, A. J., MAUSS, I. H. and ROSENRLAT.~,P. Congenital diverticulosis of left ventricle. Atn. .J. Dir. Child., 79:111, 1950. Rupture des Herzes bei einem 6. VON SYDOW. Kindle. Jahrb f. Kinderh, 47 :437, 1866. 7. TAUSSIG, H. B. Congenital Malformations of the Heart. 11. Specific Malformations, pp. 270-73. Cambridge, 1960. Harvard University Press. 8. POTTS, W. .J. Congenital diverticulum of the left ventricle; case report. Surgery, 33:301, 1953. 9. CRITTENDEN,I. H., ADAMS,F. H. and MULDER, D. G. A syndrome featuring defects of the heart, sternum, diaphragm, and anterior abdominal wall. Cirrufation, 20:396, 1959. 10. EL-AKKARI, S. Diverticulum of the left ventricle, report of a case. Arch. Dir. Childhood, 40:545, 1965. 11. Lowe, J. B., WILLIAMS, J. C. P., ROBB, D. and COLE, D. Congenital diverticulum of the left ventricle. &it. Heart J., 21:101, 1959. 12. PARSONS,C. Ventricular extension into the abdominal wall. &it. Heart J., 19:34, 1957. Nederl. 13. FORMYNE, P. Aangeboren Hartzgebreken. tijdschr. gmeesk, 94:2704, 1950. 14. SNELLEN, H. A., DANKMEIJER, J., BRUIUS, C. and CALLISTER, R. M. Saccular elongation of tbe left ventricle into the abdominal wall with persistence of the anterior mesocardium and ventricular septal defects. Cardiologia, 211562, 1952. 15. MULDER, D. G., CRITTENDEN,I. N. and ADAMS, F. II. Complete repair of a syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart; excision of left ventricular diverticulum. Surgery, 151: 113, 1960.
of the Heart
Part of the Syndrome of Congenital Midline Thoracic JOSEPH W. ROBERT J. HALL,
EDGETT, JR.,
and Abdominal
LT. COL., Mc,t
WILLIAM
E. FISHBACK,
COL., MC, MALCOLM
LVashington,
Cardiac and Defects*
P. NELSON,
LT. COL., MC,
MAJ., MC and EDWARD D.
J.
JAHNKE,
COL., MC
C.
A case of coexistent congenital midline thoracoabdominal and cardiac defects is presented. The cardiac lesions consisted of a large ventricular septal defect and a muscular diverticulum of the left ventricle. Refractory congestive heart failure was controlled by banding of the pulmonary artery, and the muscular diverticulum was resected. Such cases are rare but important since the defects are treatable. The clinical features, diagnosis, prognosis and treatment are discussed.
A
SYNDROME
OF ASSOCIATED
CONGENITAL
and 100 in the legs. Pulse rate was lbO/min. Respiratory rate was 50/min., and there was flaring of the alae nasae. The chest was symmetrically formed with an extremely wide angle between the lower rib margin and the sternum. The sternal length appeared abnormally short and at its distal end was a 2 by 2 cm. bluish, pulsatile mass covered by a thin, translucent skin (Fig. 1). Its pulsations were synchronous with precordial activity, and pressure on the mass provoked frequent premature ventricular contractions (Fig. 2). Percussion of the cardiac borders revealed dullness extending about 4 cm. on both sides of the low sternum, and cardiac pulsations could be palpated in the same zone. On auscultation a grade 3/6 holosystolic murmur was heard with maximal intensity in the fourth intercostal space along the left sternal border. Two components of the second sound were present, but it was not possible to define their respiratory relation. An exaggerated third heart sound and middiastolic flow rumble were present at the apex. The lungs were normal. The liver was enlarged, with its lower edge palpable 3 cm. below the right costal margin. An electrocardiogram showed sinus tachycardia and biventricular hypertrophy. The frontal plane P vector suggesting normal atria1 situs and route of was +60°, atrial depolarization. Chest roentgenograms showed an enlarged cardiac silhouette with dextroposition. There was moderate increase in the pulmonary vascularity. Cardiac Catheterization and Angiocardiography: The child was considered to have congestive heart failure,
DE-
diverticulum, intracardiac defects, and midline thoracoabdominal defects was first reported by Cantrell et al.’ Because of the infrequent occurrence of this potentially lethal syndrome, specific guidelines regarding its diagnosis and treatment are not firmly established. The following case is presented to review briefly the clinical features of this complex lesion and to discuss the diagnosis, prognosis, and surgical treatment of this entity. FECTS
including
muscular
ventricular
CASE REPORT A 9 day old Caucasian male child was referred to Walter Reed General Hospital for cardiac evaluation. He was the product of a full term, uncomplicated gestation and spontaneous delivery. At birth an umbilical hernia and an omphalocele were noted and these defects were repaired on the first day of life. At surgery the omphalocele was found to contain loops of bowel. The omphalocele was excised and the umbilical hernia repaired by apposing the fascia of the rectus abdominus muscles. No defects of the diaphragm were identified. On the third postoperative day, the appearance of a cardiac murmur and a pulsatile epigastric mass prompted referral to this hospital. Physical examination revealed a well nourished Caucasian infant with mild respiratory distress. Blood pressure (flush technic) was 90 mm. Hg in the arms
* From the Cardiology and Thoracic Surgical Services, Walter Reed General Hospital, Washington, D. C. Manuscript received June 7, 1968, accepted February 28, 1969. t Present address and address for reprints: Joseph W. Edgett, Jr., LT. COL., MC, Department of the Army, Fitzsimons General Hospital, Denver, Colo. 80240. 580
THE
AMERICAN
JOURNAL
OF CARDIOLOGY
Diverticulum
and administration of digitalis and a diuretic resulted in some improvement. On the ninth hospital day (at age 18 days), cardiac catheterization and biplane angiocardiography were performed with the following findings: dextroposition of the heart, normal atria1 situs, a ventricular septal defect and a large left to right intracardiac shunt (pulmonary to systemic flow ratio greater than 5 : 1). Angiocardiography revealed a normally sized and placed pulmonary artery and left atrium. With opacification of the left ventricle a 2 by 2 cm. diverticulum was seen extending from the apex of the left ventricle (Fig. 3). The left to right intracardiac shunt was demonstrated by the recirculation of contrast material through the pulmonary artery. Injection of contrast material into the root of the aorta showed no evidence of a patent ductus arteriosus. Surgical Treatment: Because of persistent cardiac decompensation, regarded as primarily due to the magnitude of the left to right intracardiac shunt, the patient was considered a candidate for banding of the
Figure 2. VOLUME
24,
OCTOBER
1969
of the Heart
581
‘Ihis \vas accomplished without pulmonary artery. complication on the fifteenth hospital day, and the clinical signs of congestive heart failure disappeared. Surgical repair of the ventricular diverticulum was planned as a second procedure, so that optimal cardiac compensation might be achieved prior to this operation. On the thirty-fifth hospital day (at age 44 days) resection of the diverticulum was accomplished. At operation, the following defects were identified: a foreshortened sternum and a defect in the inferior pericardium through which a muscular diverticulum of the apex of the left ventricle passed to present in the epigastrium. The diaphragm was intact and normal. 7’he diverticulum was resected, and the pericardium closed. The patient’s postoperative course was uncomplicated and he was discharged from the hospital at age 58 days. He was seen in follow-up at 6 months of age. Growth and development had been slow but definite and there had been no intercurrent evidence of cardiac decompensation. Continued observation is
Continuous rhythm strip showing effect of pressure on the epigastric
mass.
Edgett et al.
582
Fil[ure 3. An,&mzrdio.pxn. A, frontal virw; B, lateral vkw. site of ventricular diverticulum.
planned with ultimate closure of the ventricular septal defect and removal of the constricting pulmonary artery band.
DISCUSSION Diverticulum and aneurysm of the ventricle are terms that have, in the past, been used interchangeably. The cause may be a congenital defect or an acquired lesion. Of the defects considered to be of congenital origin, Chesler et al2 had proposed a division into two groups: muscular and fibrous diverticula of the left ventricle. The congenital fibrous variety differ from the muscular diverticula in several ways: (1) they never occur with an associated midline thoracoabdominal defect, (2) they usually communicate with the left ventricle at the level of the mitral annulus, and (3) the majority of known cases have occurred in Negroes. Muscular diverticula of the ventricle are distinctly different and are the subject of this report. Muscular diverticulum of the ventricle was first reported in 1866.3 Since that time, 25 cases have been reported.‘*4-14 Although it was initially considered a form of ectopia cordis, Cantrell et al.’ in 1958 proposed that the heart was not ectopic and that muscular ventricular diverticulum was a part of a group of congenital defects, including (1) malrotation of the heart with dextroposition; (2) intracardiac anomalies including ventricular septal defects; (3) a deficient lower sternum; (4) an anterior diaphragmatic defect; and (5) midline abdominal defects with diastasis recti and umbilical hernias. Clinical Features: This complex of congenital defects usually presents in the newborn as a pulsatile epigastric mass with varying midline
Arrows designate
abdominal and thoracic defects. There is no apparent sex predilection. The spectrum of midline thoracoabdominal defects extends from an isolated umbilical hernia to defects with a foreshortened sternum, absence of the anterior one third of the diaphragm, diastasis recti and an omphalocele. Intracardiac defects have consisted of ventricular septal defects or tricuspid atresia. Pericardial defects are usually over the inferior aspect of the heart. The ventricular diverticula arise most frequently from the apex of the left ventricle, but have projected from the right ventricle and in 1 case from both ventricles. Diagnosis: Because of the multiplicity of congenital defects, anatomic clarification is mandatory before formulating a plan of surgical correction. Cardiac catheterization should be accomplished if a coexisting intracardiac defect is suspected and will allow a specific diagnosis in most cases. The ventricular diverticulum can be clearly delineated by angiocardiography as demonstrated in our case. Percutaneous injection of the contrast material directly into the lumen of the diverticulum has been suggested but, in our opinion, would seem an unwarranted risk. Prognosis: The importance of early treatment is emphasized by the fact that in 14 of the reported cases the patients died in the first four years of life, with 9 deaths occurring in the first two months of life. The cause of death has been attributed variously to congestive heart failure, diverticulum rupture, or associated congenital defects. Lowe et al.” speculated that rupture is secondary to the excessive pressure developed in the cavity of the diverticulum. The pressure is THE AMERICAN
JOURNAL
OF
CARDIOLOGY
Diverticulum clc\.t,lopcd because contraction of the ventricular wall, which precedes contraction of the diverticulun~, occludes the opening of the diverticulum and, with its subsequent contraction, the pressure within the diverticulum is exerted against a closed orifice. LSurgical Correction: Treatment of the ventricular diverticulum has been accomplished in two ways. The diverticulum may be replaced in the perirardial sac and the defect in this membraneclosed. The alternate method consists of resection of the diverticulum.‘5 To date, 13 patients have been operated upon. Nine of these had resection, 1 replacement, and 3 died at operation. Of the latter 3, 1 experienced cardiac arrest shortly after the incision was made and could 1 experienced cardiac not be resuscitated,’ arrest after resection of the diverticulum and could not be resuscitated,’ and the final patient, who had coexisting tricuspid atresia,2 died several hours after a subclavian to pulmonary artery anastomosis. The preferable approach would seem to be resection. REFERENCES 1. CANTRELL, J. R., HALLER, J. A. and RAVITCH, M. M. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg., Gynec. &? Obst., 107:603, 1958. 2. CHESLER, M. B., TUCKER, R. B. K. and BARLOW, Subvalvular and apical left ventricular J. B. aneurysms in the Bantu as a source of systemic emboli. Circulation, 35:1156, 1967.
VOLUME 24, OCTOBER 1969
of the Heart
S83
3. ABRAHAM&D. G., BARTON,C. .I., COCKSHO.,“,‘,W. P., EDINGTON. G. M. and WEAVF.R, E. J. M. i\nnular subvalvular left ventricular aneurysms. Qual-t..J. Med., 31:345, 1962. 4. SKAPINKER, S. Diverticulum of the left ventricle of the heart. Arch. Surg., 631629, 1951. 5. SWUER, A. J., MAUSS, I. H. and ROSENRLAT.~,P. Congenital diverticulosis of left ventricle. Atn. .J. Dir. Child., 79:111, 1950. Rupture des Herzes bei einem 6. VON SYDOW. Kindle. Jahrb f. Kinderh, 47 :437, 1866. 7. TAUSSIG, H. B. Congenital Malformations of the Heart. 11. Specific Malformations, pp. 270-73. Cambridge, 1960. Harvard University Press. 8. POTTS, W. .J. Congenital diverticulum of the left ventricle; case report. Surgery, 33:301, 1953. 9. CRITTENDEN,I. H., ADAMS,F. H. and MULDER, D. G. A syndrome featuring defects of the heart, sternum, diaphragm, and anterior abdominal wall. Cirrufation, 20:396, 1959. 10. EL-AKKARI, S. Diverticulum of the left ventricle, report of a case. Arch. Dir. Childhood, 40:545, 1965. 11. Lowe, J. B., WILLIAMS, J. C. P., ROBB, D. and COLE, D. Congenital diverticulum of the left ventricle. &it. Heart J., 21:101, 1959. 12. PARSONS,C. Ventricular extension into the abdominal wall. &it. Heart J., 19:34, 1957. Nederl. 13. FORMYNE, P. Aangeboren Hartzgebreken. tijdschr. gmeesk, 94:2704, 1950. 14. SNELLEN, H. A., DANKMEIJER, J., BRUIUS, C. and CALLISTER, R. M. Saccular elongation of tbe left ventricle into the abdominal wall with persistence of the anterior mesocardium and ventricular septal defects. Cardiologia, 211562, 1952. 15. MULDER, D. G., CRITTENDEN,I. N. and ADAMS, F. II. Complete repair of a syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart; excision of left ventricular diverticulum. Surgery, 151: 113, 1960.