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Hemangioma of the right ventricle causing outflow tract obstruction
J
THoRAc CARDIOVASC SURG
1988;96:307-9
Hemangioma of the right ventricle causing outflow tract obstruction A 3112-year-old child had a murmur of pulmonary stenosis. Echocardiography and cardiac catheterization revealed a pulmonary infundibular obstruction. Magnetic resonance imaging of the heart demonstrated a mass in the interventricular septum. The mass was successfully resected and a pathologic diagnosis of capillary hemangioma was made. Only two previous cases of hemangioma causing right ventricular outflow obstruction have been reported; both of these cases involvedadults. This case represents the first report of a hemangioma causing right ventricular outflow tract obstmction in a child. An exploratory operation with resection is the treatment of choice.
Mark S. Soherman, MD, William H. Plauth, MD, Kevin J. Winn, MD, Gerard C. Forest, PA, Charles R. Hatcher, Jr., MD, and James D. Sink. MD, Atlanta. Ga.
Emary cardiac tumors are rare, with an incidence of 0.0017% to 0.03% in large autopsy series.I? Hemangioma is the most common vascular tumor of the heart, yet constitutes only 2.8% of primary cardiac tumors.' Since the first reported case in 1893, fewer than 50 cases of cardiac hemangioma have been reported in the world literature, the majority having been discovered at autopsy.t' If classified by anatomic location, cardiac tumors arising from the right ventricle are an uncommon subgroup. In 1972, Delaye" reported a case of right ventricular hemangioma and reviewed 62 known cases of primary right ventricular tumor. Of the 56 cases that could he classified histologically, only two (3.6%) were hemangiomas. There have been "two reports of right ventricular hemangioma, in which outflow tract obstruction was initially evident, both in adults. We report the first known case of hemangioma causing right ventricular outflow tract obstruction in a child.
Case report The patient is a 31f2-year-old boy referred to the Henrietta Egleston Hospital for Children for evaluation of a murmur consistent with pulmonary stenosis. The murmur was first noted at birth and was presumed to be innocent.
From the Henrietta Egleston Hospital for Children; the Divisions of Cardiothoracic Surgery and Pediatric Cardiology, Emory University School of Medicine; and Emory Clinic, Atlanta, Ga. Received for publication July 28, 1987. Accepted for publication Dec. 7, 1987. Address for reprints: James D. Sink, MD, Emory University Clinic, 1365 Clifton Rd., N.E. Atlanta, GA 30322.
, Fig. 1. Right ventriculogram in lateral projection demonstrating subvalvular obstruction of the right ventricular outflow tract. Physical examination revealed a long grade 3/6 systolic murmur at the left upper sternal border. Routine laboratory values were within normal limits. Chest x-ray films demonstrated cardiomegaly, and the electrocardiogram showed evidence of right ventricular hypertrophy. Echocardiography revealed right ventricular enlargement, right ventricular hypertrophy, and thickening of the interventricular septum with a mass bulging into the right ventricular outflow tract. Right heart catheterization demonstrated a subvalvular muscular obstruction of the right ventricle, with a 30 mm Hg systolic pressure gradient between the body of the right
307
308
Soberman et al.
Fig. 2. Left ventriculogram in right anterior oblique projection. A vascular blush filling from the left anterior descending coronary artery is noted at the level of the right ventricular outflow tract obstruction.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 3. Cardiac-gated, magnetic resonance image in the transverse plane. TE = 30 msec, 0.5 T magnet. A section made in midsystole shows a localized thickening of the ventricular septum (T) that bulges into both the right ventricle (RV) and the left ventricle (LV). The T2 relaxation time of the tumor was the same as that of the normal myocardium. RA, Right atrium; LA, left atrium. Arrow indicates mitral valve.
Fig. 4. Representative section of resected tissue. Masson trichrome stain of the lesion shows myocardial cell (black) separated by numerous small blood vessels. (Mag. X lOa.) ventricle and the main pulmonary artery (Fig. 1). During the left ventriculogram a vascular blush filling from the left anterior descending coronary artery was evident at the level of obstruction (Fig. 2). Magnetic resonance imaging of the heart confirmed the presence of a mass in the interventricular septum (Fig. 3).
The patient was taken to the operating room where total cardiopulmonary bypass was initiated with systemic hypothermia. The aorta was cross-clamped and cardioplegia given. A vertical ventriculotomy was made, and hypertrophied muscle bundles were excised from the parietal and septal bands. The infundibular septum appeared quite hypertrophied, and mus-
Volume 96 Number 2 August 1988
de was excised from this area. Additional cardioplegia was given, and vigorous outputof the cardioplegia was noted from the bed of resected muscle. The ventriculotomy was closed with a pericardial patch. The patient tolerated the procedure well, without complications. Postoperative echocardiography demonstrated some residual septal thickening, with normal flow across the right ventricular outflow tract.The patient is doing well 1 yearafter operation. Pathology. The pathology specimen consisted of multiple fragments of right ventricular myocardium with no grossly evident tumor (Fig. 4). Microscopically the tissue consisted of myocardial cells, between which large numbers of small blood vessels were insinuated. The capillary-sized blood vessels were lined by endothelial cells with large nuclei. There were more nuclei thanwould appearin normal vessels. No separate tumor mass was present, but insome areasdiffusely infiltrating blood vessels constituted approximately 50% of the area of the lesion. The diagnosis of capillary hemangioma with diffuse infiltration was made. Discussion
Hemangioma of the heart is an extremely rare entity, with the world literature containing fewer than 50 reported cases. These lesions may involve the endocardium, myocardium, or epicardium and have been described in all heart chambers and the pericardium. Subendocardial hemangiomas most commonly involve the right ventricle or right atrium.' The mode of presentation varies, depending on the size and location of the tumor.s ' In 1964, Nicks? reported the case of a 20-year-old woman with a 17-year history of dyspnea and the recent onset of nonexertional chest pain and ankle edema. A grade 4/6 murmur was present at the left upper sternal border. Cardiac catheterization demonstrated pulmonary infundibular stenosis. At operation a 4 em rounded vascular lesion was identified in the outflow tract of the right ventricle and resected. Microscopically the tumor was angiomatous in appearance, with heart muscle and adipose tissue also present. It was termed a vascular hamartoma. which could be classified as a hemangioma by current standards. In 1972, Delaye" reported a case of hemangioma in a 23-year-old man. The patient had dyspnea on exertion, lower extremity edema, and chest pain. On physical examination a grade 2/6 systolic murmur was present at the left upper sternal border. Catheterization revealed a systolic pressure gradient between the right ventricle and pulmonary artery. A mobile infundibular mass was noted, which obstructed the right ventricular outflow tract during systole. At operation a pedunculated tumor of the right ventricular infundibulum was successfully resected. Histologically the tumor was a capillary hemangioma.
Hemangioma of the right ventricle 3 0 9
Although rare, a cardiac tumor, including hemangioma, must be considered in the differential diagnosis of patients exhibiting cardiac symptoms. These tumors may mimic valvular obstruction, coronary artery disease, or conduction abnormalities. A thorough evaluation utilizing echocardiography and cineangiography when appropriate, combined with clinical suspicion, will usually suggest if not confirm a diagnosis of cardiac neoplasm. Surgery is indicated in these patients to both confirm the diagnosis and provide optimal treatment. The natural history of cardiac hemangioma is unpredictable because of the erratic behavior of the tumor. The tumor may involute, stop growing, or proliferate indefinitely, and it mayor may not produce symptoms.v' Unresectable tumors have been treated with radiation, but the number of cases is small and data extremely limited.' Resection is recommended if technically feasible. Magnetic resonance imaging in our patient provided more detailed images of the tumor than other available diagnostic modalities, although a firm diagnosis was not arrived at until surgical exploration and pathologic examination of the resected tissue were accomplished. Magnetic resonance imaging may prove to be the method of choice for the diagnosis of cardiac tumors. We plan to follow this patient closely with serial magnetic resonance imaging and echocardiography to monitor for tumor recurrence. The assistance of Murray Baron, MD, Emory University School of Medicine, Atlanta, is greatly appreciated. REFERENCES I. Silverman NA. Primary cardiac tumors. Ann Surg 1980;191 :127-38. 2. Tabry IF, Nassar VH, Rizk G, et al. Cavernous hemangiomaof the heart. J THoRAc CARDlOVAS SURG 1975;69:41520. 3. Novitzky D, Rose AG, Morgan JA, Barnard CN. Primary
cardiac haemangiomas. A report of 2 cases. S Afr Moo J 1984;66:267-70.
4. Baroldi G, Colombo F, Manion We. Benign primary
hemangioma of the right atrium of the heart. Moo Ann DC 1967;36:287-90. 5. Sulayman R, Cassels DE. Myocardial coronary hemangiomatous tumors in children. Chest 1975;68:113-5. 6. Delaye J, Crassard P, Segneuric A, et al. Les tumeurs primitives du ventricule droit. A propos d'un cas d'hemangio-endotheliome benin opere avec sucres. J Med Lyon 1972;53:483-94.
7. Nicks R. Hamartoma of the right ventricle. J THoRAc CARDlOVAS SURG 1964;47:762-8.
THoRAc CARDIOVASC SURG
1988;96:307-9
Hemangioma of the right ventricle causing outflow tract obstruction A 3112-year-old child had a murmur of pulmonary stenosis. Echocardiography and cardiac catheterization revealed a pulmonary infundibular obstruction. Magnetic resonance imaging of the heart demonstrated a mass in the interventricular septum. The mass was successfully resected and a pathologic diagnosis of capillary hemangioma was made. Only two previous cases of hemangioma causing right ventricular outflow obstruction have been reported; both of these cases involvedadults. This case represents the first report of a hemangioma causing right ventricular outflow tract obstmction in a child. An exploratory operation with resection is the treatment of choice.
Mark S. Soherman, MD, William H. Plauth, MD, Kevin J. Winn, MD, Gerard C. Forest, PA, Charles R. Hatcher, Jr., MD, and James D. Sink. MD, Atlanta. Ga.
Emary cardiac tumors are rare, with an incidence of 0.0017% to 0.03% in large autopsy series.I? Hemangioma is the most common vascular tumor of the heart, yet constitutes only 2.8% of primary cardiac tumors.' Since the first reported case in 1893, fewer than 50 cases of cardiac hemangioma have been reported in the world literature, the majority having been discovered at autopsy.t' If classified by anatomic location, cardiac tumors arising from the right ventricle are an uncommon subgroup. In 1972, Delaye" reported a case of right ventricular hemangioma and reviewed 62 known cases of primary right ventricular tumor. Of the 56 cases that could he classified histologically, only two (3.6%) were hemangiomas. There have been "two reports of right ventricular hemangioma, in which outflow tract obstruction was initially evident, both in adults. We report the first known case of hemangioma causing right ventricular outflow tract obstruction in a child.
Case report The patient is a 31f2-year-old boy referred to the Henrietta Egleston Hospital for Children for evaluation of a murmur consistent with pulmonary stenosis. The murmur was first noted at birth and was presumed to be innocent.
From the Henrietta Egleston Hospital for Children; the Divisions of Cardiothoracic Surgery and Pediatric Cardiology, Emory University School of Medicine; and Emory Clinic, Atlanta, Ga. Received for publication July 28, 1987. Accepted for publication Dec. 7, 1987. Address for reprints: James D. Sink, MD, Emory University Clinic, 1365 Clifton Rd., N.E. Atlanta, GA 30322.
, Fig. 1. Right ventriculogram in lateral projection demonstrating subvalvular obstruction of the right ventricular outflow tract. Physical examination revealed a long grade 3/6 systolic murmur at the left upper sternal border. Routine laboratory values were within normal limits. Chest x-ray films demonstrated cardiomegaly, and the electrocardiogram showed evidence of right ventricular hypertrophy. Echocardiography revealed right ventricular enlargement, right ventricular hypertrophy, and thickening of the interventricular septum with a mass bulging into the right ventricular outflow tract. Right heart catheterization demonstrated a subvalvular muscular obstruction of the right ventricle, with a 30 mm Hg systolic pressure gradient between the body of the right
307
308
Soberman et al.
Fig. 2. Left ventriculogram in right anterior oblique projection. A vascular blush filling from the left anterior descending coronary artery is noted at the level of the right ventricular outflow tract obstruction.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 3. Cardiac-gated, magnetic resonance image in the transverse plane. TE = 30 msec, 0.5 T magnet. A section made in midsystole shows a localized thickening of the ventricular septum (T) that bulges into both the right ventricle (RV) and the left ventricle (LV). The T2 relaxation time of the tumor was the same as that of the normal myocardium. RA, Right atrium; LA, left atrium. Arrow indicates mitral valve.
Fig. 4. Representative section of resected tissue. Masson trichrome stain of the lesion shows myocardial cell (black) separated by numerous small blood vessels. (Mag. X lOa.) ventricle and the main pulmonary artery (Fig. 1). During the left ventriculogram a vascular blush filling from the left anterior descending coronary artery was evident at the level of obstruction (Fig. 2). Magnetic resonance imaging of the heart confirmed the presence of a mass in the interventricular septum (Fig. 3).
The patient was taken to the operating room where total cardiopulmonary bypass was initiated with systemic hypothermia. The aorta was cross-clamped and cardioplegia given. A vertical ventriculotomy was made, and hypertrophied muscle bundles were excised from the parietal and septal bands. The infundibular septum appeared quite hypertrophied, and mus-
Volume 96 Number 2 August 1988
de was excised from this area. Additional cardioplegia was given, and vigorous outputof the cardioplegia was noted from the bed of resected muscle. The ventriculotomy was closed with a pericardial patch. The patient tolerated the procedure well, without complications. Postoperative echocardiography demonstrated some residual septal thickening, with normal flow across the right ventricular outflow tract.The patient is doing well 1 yearafter operation. Pathology. The pathology specimen consisted of multiple fragments of right ventricular myocardium with no grossly evident tumor (Fig. 4). Microscopically the tissue consisted of myocardial cells, between which large numbers of small blood vessels were insinuated. The capillary-sized blood vessels were lined by endothelial cells with large nuclei. There were more nuclei thanwould appearin normal vessels. No separate tumor mass was present, but insome areasdiffusely infiltrating blood vessels constituted approximately 50% of the area of the lesion. The diagnosis of capillary hemangioma with diffuse infiltration was made. Discussion
Hemangioma of the heart is an extremely rare entity, with the world literature containing fewer than 50 reported cases. These lesions may involve the endocardium, myocardium, or epicardium and have been described in all heart chambers and the pericardium. Subendocardial hemangiomas most commonly involve the right ventricle or right atrium.' The mode of presentation varies, depending on the size and location of the tumor.s ' In 1964, Nicks? reported the case of a 20-year-old woman with a 17-year history of dyspnea and the recent onset of nonexertional chest pain and ankle edema. A grade 4/6 murmur was present at the left upper sternal border. Cardiac catheterization demonstrated pulmonary infundibular stenosis. At operation a 4 em rounded vascular lesion was identified in the outflow tract of the right ventricle and resected. Microscopically the tumor was angiomatous in appearance, with heart muscle and adipose tissue also present. It was termed a vascular hamartoma. which could be classified as a hemangioma by current standards. In 1972, Delaye" reported a case of hemangioma in a 23-year-old man. The patient had dyspnea on exertion, lower extremity edema, and chest pain. On physical examination a grade 2/6 systolic murmur was present at the left upper sternal border. Catheterization revealed a systolic pressure gradient between the right ventricle and pulmonary artery. A mobile infundibular mass was noted, which obstructed the right ventricular outflow tract during systole. At operation a pedunculated tumor of the right ventricular infundibulum was successfully resected. Histologically the tumor was a capillary hemangioma.
Hemangioma of the right ventricle 3 0 9
Although rare, a cardiac tumor, including hemangioma, must be considered in the differential diagnosis of patients exhibiting cardiac symptoms. These tumors may mimic valvular obstruction, coronary artery disease, or conduction abnormalities. A thorough evaluation utilizing echocardiography and cineangiography when appropriate, combined with clinical suspicion, will usually suggest if not confirm a diagnosis of cardiac neoplasm. Surgery is indicated in these patients to both confirm the diagnosis and provide optimal treatment. The natural history of cardiac hemangioma is unpredictable because of the erratic behavior of the tumor. The tumor may involute, stop growing, or proliferate indefinitely, and it mayor may not produce symptoms.v' Unresectable tumors have been treated with radiation, but the number of cases is small and data extremely limited.' Resection is recommended if technically feasible. Magnetic resonance imaging in our patient provided more detailed images of the tumor than other available diagnostic modalities, although a firm diagnosis was not arrived at until surgical exploration and pathologic examination of the resected tissue were accomplished. Magnetic resonance imaging may prove to be the method of choice for the diagnosis of cardiac tumors. We plan to follow this patient closely with serial magnetic resonance imaging and echocardiography to monitor for tumor recurrence. The assistance of Murray Baron, MD, Emory University School of Medicine, Atlanta, is greatly appreciated. REFERENCES I. Silverman NA. Primary cardiac tumors. Ann Surg 1980;191 :127-38. 2. Tabry IF, Nassar VH, Rizk G, et al. Cavernous hemangiomaof the heart. J THoRAc CARDlOVAS SURG 1975;69:41520. 3. Novitzky D, Rose AG, Morgan JA, Barnard CN. Primary
cardiac haemangiomas. A report of 2 cases. S Afr Moo J 1984;66:267-70.
4. Baroldi G, Colombo F, Manion We. Benign primary
hemangioma of the right atrium of the heart. Moo Ann DC 1967;36:287-90. 5. Sulayman R, Cassels DE. Myocardial coronary hemangiomatous tumors in children. Chest 1975;68:113-5. 6. Delaye J, Crassard P, Segneuric A, et al. Les tumeurs primitives du ventricule droit. A propos d'un cas d'hemangio-endotheliome benin opere avec sucres. J Med Lyon 1972;53:483-94.
7. Nicks R. Hamartoma of the right ventricle. J THoRAc CARDlOVAS SURG 1964;47:762-8.