322
CASE REPORT UNGER ET AL GIANT CARDIAC CAVERNOUS HEMANGIOMA
Ann Thorac Surg 2015;100:322–5
FEATURE ARTICLES
Fig 3. (A) The resected specimen. The line shows a cut portion. (B) The tumor tissue near the left innominate vein shows chronic inflammation, with inflammatory cells and fibrosis (hematoxylin and eosin stain, 100).
Recently, we began to explore the usefulness of 3D models produced by rapid prototyping techniques [7]. There had not been any studies describing the use of rapid prototyping in the field of general thoracic surgery until we first reported in October 2013. To the best of our knowledge, this is the first report to describe the 3D printing model of the left innominate vein, which we consider to be more realistic than CT images, to obtain the precise anatomy. We believe that 3D printing techniques can improve the interpretation and knowledge of anatomy for complicated surgeries of the chest.
References 1. Yoshino N, Okada D, Ujiie H, et al. Venous hemangioma of the posterior mediastinum. Ann Thorac Cardiovasc Surg 2012;18:247–50. 2. Akiba T, Morikawa T, Hirayama S, Ohki T. Thymic haemangioma presenting with a left innominate vein aneurysm: insight into the aetiology. Interact Cardiovasc Thorac Surg 2012;15:925–7. 3. McAdams HP, Rosado-de-Christenson ML, Moran CA. Mediastinal hemangioma: radiographic and CT features in 14 patients. Radiology 1994;193:399–402. 4. Papagiannopoulos K, Sheppard MN, Goldstraw P. Thymic hemangioma presenting with recurrent pleural effusion. Ann Thorac Surg 2000;70:297–9. 5. Cohen AJ, Sbasching RJ, Hochholzer L, Lough FC, Albus RA. Mediastinal hemangiomas. Ann Thorac Surg 1987;43:656–9. 6. Burkill GJ, Burn PR, Padley SP. Aneurysm of the left brachiocephalic vein: an unusual cause of mediastinal widening. Br J Radiol 1997;70:837–9. 7. Akiba T, Nakada T, Inagaki T. Three-dimensional printing model of anomalous bronchi before surgery. Ann Thorac Cardiovasc Surg 2013 [Epub ahead of print].
Giant Cardiac Cavernous Hemangioma Eric Unger, MD, Joseph Costic, DO, and Glenn Laub, MD Department of Cardiothoracic Surgery, Drexel University College of Medicine, Philadelphia, Pennsylvania
We report the case of an asymptomatic giant cardiac cavernous hemangioma in a 71-year-old man. The intracardiac mass was discovered incidentally during Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
surveillance for his prostate cancer; however, the patient initially declined intervention. On presentation to our institution 7 years later, the lesion had enlarged significantly, and the patient consented to excision. At surgery, an 8 3 6.5 3 4.8 cm intracardiac mass located on the inferior heart border was excised with an intact capsule through a median sternotomy approach. The patient had an uneventful postoperative course. We discuss the diagnostic workup, treatment, and characteristics of this rare cardiac tumor. (Ann Thorac Surg 2015;100:322–5) Ó 2015 by The Society of Thoracic Surgeons
H
emangiomas are a relatively common soft tissue tumor; however, they account for only 2% of primarily resected cardiac tumors [1]. Rarer still is the finding of a concurrent hemangioma elsewhere in the body. Cavernous hemangiomas represent a subtype characterized by thin-walled, dilated vessels. Their clinical course is unpredictable, and surgical excision is considered both diagnostic and curative.
A 71-year-old man with asthma, hypertension, dyslipidemia, and prostate cancer status post–cryosurgery and radiotherapy, was found to have a cardiac mass on the inferior border of the heart during his follow-up prostate cancer surveillance by computed tomography imaging, as well as a 2.4 cm hepatic segment VII lesion. Magnetic resonance imaging further characterized the hepatic lesion as a 6.6 4.7 4.9 cm hypointense T1-weighted and hyperintense T2-weighted lesion at the interatrial septum (Fig 1). The lesion moderately compressed the left atrium and was suspected to be a sarcoma. The patient was asymptomatic from his cardiac mass and declined intervention. Fine-needle aspiration of the hepatic lesion revealed a cavernous hemangioma, which was thought to not require further treatment. As part of a cardiac workup 7 years later, magnetic resonance imaging of the chest showed that the mass had enlarged to 8.7 6.3 5.9 cm, and was located just Accepted for publication Aug 15, 2014. Address correspondence to Dr Unger, 245 N 15th St, MS413, Philadelphia, PA 19102; e-mail: [email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.08.056
Ann Thorac Surg 2015;100:322–5
CASE REPORT UNGER ET AL GIANT CARDIAC CAVERNOUS HEMANGIOMA
323
Fig 1. (A) T2-weighted axial image and (B) coronal image from the initial magnetic resonance imaging. (C, D) The mass was enlarged on repeat magnetic resonance imaging performed 7 years later. On all images, an encapsulated large mass, marked by asterisks, is seen in relation to the cardiac chambers, with mass effect on the right atrium.
FEATURE ARTICLES
inferior to the left atrium, with a resultant mass effect on the esophagus and atria (Fig 1). The mass had intermediate signal intensity on T1-weighted imaging, hyperintensity on T2-weighted imaging, hyperintensity on fat-saturated images, and a heterogeneous appearance on contrast imaging. The hepatic cavernous hemangioma was minimally enlarged (2.7 2.5 1.7 cm). Transesophageal echocardiography suggested an encapsulated mass that was confluent with the right ventricle, mild to moderate mitral regurgitation, trace tricuspid regurgitation, and an ejection fraction of 60% to 65% (Fig 2). Given the tumor growth, uncertain diagnosis, and relationship to the surrounding structures, it was recommended that the tumor be excised. At surgery, a median sternotomy approach was used, and the pericardium was opened. The mass was found to be intracardiac, located under the epicardial surface of the inferior heart border, and extended posteriorly to the level of the atrium (Fig 3). After the inferior heart border was retracted, a plane free from vascularity was developed, and the tumor was able to be shelled out from the surrounding myocardium. The tumor was excised intact (Fig 3). After hemostasis, mediastinal drains were placed and the chest was closed. Postoperatively, the patient made an uneventful recovery except for a brief episode of atrial fibrillation. He was discharged home in stable condition
on postoperative day 4. On pathology, the 8 6.5 4.8 cm, 78 g specimen was confirmed to be a cavernous hemangioma (Fig 4). The tumor was surrounded by fibrous tissue, and it was excised with an intact capsule.
Fig 2. The mass is marked in this echocardiographic image with an asterisk. The mitral valve (MV) and tricuspid valve (TV) are also visualized.
CASE REPORT UNGER ET AL GIANT CARDIAC CAVERNOUS HEMANGIOMA
Ann Thorac Surg 2015;100:322–5
FEATURE ARTICLES
324
Fig 3. (A) The cavernous hemangioma is present under the epicardial surface, at the inferior border of the heart. (B) The gross specimen reveals an intact capsule.
Comment Cardiac hemangiomas are rare benign cardiac accounting for 2% of primarily resected cardiac These tumors can present at any age and have male predominance [1]. Although most
Fig 4. Hematoxylin-and-eosin stained slide (original magnification, 4), showing the prominence of thin-walled, dilated vessels.
tumors, tumors. a slight cardiac
neoplasms are incidentally discovered, these lesions have the potential to cause significant morbidity and mortality secondary to their spatial relation to adjacent structures. Presentations include arrhythmias, conduction disturbances, congestive heart failure, coronary insufficiency, chest pain, cough, and dysphagia. Death due to conduction disturbances or rupture and tamponade is rare [2]. Cardiac hemangiomas are well-circumscribed tumors occurring in the epicardium, myocardium, or endocardium, and have been associated with all cardiac chambers. Hemangiomas can be classified into three groups based on the morphologic pattern of their vascular channels, endothelial cells, and supporting stroma: capillary hemangiomas are composed of small capillarylike vessels, arteriovenous hemangiomas have thick-walled dysplastic vessels, and cavernous hemangiomas contain thin-walled dilated vessels [3]. Histologically, they are composed of proliferations of endothelial cells that form vascular spaces or channels, and are identical to hemangiomas that form elsewhere in the body. Radiographic findings in this case are characteristic of cavernous hemangiomas. On an unenhanced computed tomography scan, hemangiomas are typically well circumscribed and homogeneous in attenuation. Given their vascular nature, they enhance with contrast administration. Cardiac magnetic resonance imaging is superior to computed tomography for soft-tissue characterization, and provides information regarding the enhancement pattern, invasion, and a functional assessment. As in our patient, cardiac hemangiomas are typically isointense to the myocardium on T1-weighted images and hyperintense on T2-weighted images [3]. Although echocardiography provides the benefit of dynamic assessments of cardiac tumors without ionizing radiation, the findings are inconsistent between cavernous hemangiomas. On ultrasonography, these lesions vary in their echogenicity, and are characterized by slow blood flow that may not been seen on duplex Doppler studies [4]. Echocardiography did prove useful for further defining the tumor’s spatial relationships. Although not obtained in this case, coronary angiography does not show marked enhancement in cavernous hemangiomas, as these lesions have large vascular channels with slower blood velocities. In contrast, arteriovenous and capillary hemangiomas reveal a characteristic “tumor blush” [3]. The natural history for cardiac hemangiomas is unpredictable. They may spontaneously regress, stop growing, or proliferate [2]. Complete surgical resection is considered curative, although rare instances of recurrence are reported [4]. Chalet and associates [5] reported a case of a high-grade angiosarcoma excised from the left atrium of a 44-year-old woman 7 years after cardiac hemangioma excision [5]. Although most reported cardiac cavernous hemangiomas are found in isolation, concurrent hemangiomas have been reported. Yang and colleagues [5] reported a patient with a single cardiac hemangioma and multiple pulmonary cavernous hemangiomas discovered on the workup for pericardial and pleural effusions. These led to cardiac and pulmonary failure that progressed to multisystem organ failure and
Ann Thorac Surg 2015;100:325–6
death 2 months after presentation [6]. The decision to operate on our patient was based on the increasing size, uncertain diagnosis, and the inconsistencies in tumor location across imaging modalities. The size of the tumor we excised was among the largest reported cardiac hemangiomas, which is in itself a rare tumor. Our patient also had the rare finding of a second cavernous hemangioma in his liver. Large cardiac tumors, increasing size, symptoms, and an unclear diagnosis are all indications for excision. Although imaging and patient history may suggest a diagnosis of hemangioma, definitive diagnosis and treatment are achieved by surgical excision.
References
Delayed Diagnosis of Q Fever: Finally Diagnosed After Elective Cardiac Surgery Robert Tyler, MRCS, Hannah Povey, MBChB, Sumita Pai, FRCPath, and Catherine Sudarshan, MD, FRCS(CTh) Departments of Cardiothoracic Surgery and Clinical Microbiology, Papworth Hospital NHS Foundation Trust, Papworth Everard, Cambridge, United Kingdom
Q fever is a bacterial infection caused by Coxiella burnetti. It can cause both acute and chronic illness. Chronic QF can present as a variety of clinical syndromes. A common and critical manifestation is endocarditis which can present atypically and is easily missed. This case describes a man who, after extensive investigation for splenomegaly and pancytopenia by several specialties, was finally diagnosed with Q fever endocarditis after unexpected aortic valve abnormalities found during elective cardiac surgery. Several factors contributed to diagnostic delay including aspects of clinical assessment Accepted for publication Aug 25, 2014. Address correspondence to Dr Tyler, Department of Cardiothoracic Surgery, Papworth Hospital, Cambridgeshire CB3 3RE, United Kingdom; e-mail: [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
325
and radiologic findings. Vigilance is essential for diagnosis and prompt initiation of effective treatment. (Ann Thorac Surg 2015;100:325–6) Ó 2015 by The Society of Thoracic Surgeons
T
he bacterial infection called Q fever (QF) is caused by the strict intracellular organism Coxiella burnetti. The common sources of human infection include farm animals such as cattle, sheep, and goats, along with wild animals such as roe-deer [1]. Humans are infected by the inhalation of aerosolized bacteria after exposure to infected products of conception or fomites. QF is often wrongly assumed to be a rarity when in actual fact approximately 5% of the United Kingdom’s general population have serologic evidence of previous exposure, rising to around 15% in farmers [2]. Acute QF is often asymptomatic but may present as a self-limiting illness with flulike symptoms, pneumonia, and hepatitis [2]. Approximately 1% to 5% of persons infected will have chronic QF, which may be quiescent for many years, finally presenting decades after initial infection [3]. Chronic QF can present as a variety of clinical syndromes but most commonly presents as culture negative endocarditis (60% to 70% cases) [2, 3]. Patients at particular risk of having chronic QF include those who have existing valvulopathy, are immunosuppressed, or are pregnant at the time of initial infection. Chronic QF can cause a variety of nonspecific clinical syndromes. Endocarditis caused by QF often presents insidiously and may lack the classical features of endocarditis. Consequently it often proves to be a diagnostic challenge that, without prompt recognition and initiation of effective treatment, can lead to severe and potentially fatal illness [3]. We present a patient who, despite extensive investigation for splenomegaly and pancytopenia, was finally diagnosed with chronic QF as a result of elective cardiac surgery. Infective endocarditis was suspected in the operating theater during elective aortic valve replacement. The causative agent was made apparent by serology, undertaken as a part of the culture negative endocarditis workup, with results positive for QF. A 53-year-old postman was being investigated for splenomegaly and mild pancytopenia. He was otherwise well, with no history of fever or malaise, although he reported very minor weight loss over the preceding 6 months. Medical history included nephrolithiasis. Further investigations, including computed tomography skeletal survey and trephine bone marrow biopsy, were normal. Despite an extensive workup and discussion by several specialties a diagnosis was not reached; the patient was kept under outpatient review pending further investigation. During assessment he complained of gradually worsening exertional dyspnea and was noted to have a cardiac murmur suggestive of mixed aortic valve disease with predominant aortic regurgitation. Transesophageal echocardiography revealed severe aortic regurgitation and moderate aortic stenosis. No vegetations or abscesses were visualized. The left ventricle was severely dilated with normal systolic function. Computed tomography 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.08.055
FEATURE ARTICLES
1. Jain D, Maleszewski JJ, Halushka MK. Benign cardiac tumors and tumorlike conditions. Ann Diagn Pathol 2010;14:215–30. 2. Thomas JE, Eror AT, Kenney M, et al. Asymptomatic right atrial cavernous hemangioma: a case report and review of the literature. Cardiovasc Pathol 2004;13:341–4. 3. Grebenc ML, Rosado de Christenson ML, Burke AP, et al. Primary cardiac and pericardial neoplasms: radiologicpathologic correlation. Radiographics 2000;20:1073–103. 4. Kim TK, Jang HJ, Wilson SR. Hepatic neoplasms: features on grayscale and contrast enhanced ultrasound. Ultrasound Clin 2007;2:333–54. 5. Chalet Y, Mace L, Franc B, Neveux JY, Lancelin B. Angiosarcoma 7 years after surgical excision of histiocytoid haemangioma in left atrium. Lancet 1993;341:1217. 6. Yang L, Dai J, Xiao Y, Cheng H, Ruan Q. Cardiac cavernous hemangioma and multiple pulmonary hemangiomas. Ann Thorac Surg 2014;97:687–9.
CASE REPORT TYLER ET AL DELAYED DIAGNOSIS OF Q FEVER
CASE REPORT UNGER ET AL GIANT CARDIAC CAVERNOUS HEMANGIOMA
Ann Thorac Surg 2015;100:322–5
FEATURE ARTICLES
Fig 3. (A) The resected specimen. The line shows a cut portion. (B) The tumor tissue near the left innominate vein shows chronic inflammation, with inflammatory cells and fibrosis (hematoxylin and eosin stain, 100).
Recently, we began to explore the usefulness of 3D models produced by rapid prototyping techniques [7]. There had not been any studies describing the use of rapid prototyping in the field of general thoracic surgery until we first reported in October 2013. To the best of our knowledge, this is the first report to describe the 3D printing model of the left innominate vein, which we consider to be more realistic than CT images, to obtain the precise anatomy. We believe that 3D printing techniques can improve the interpretation and knowledge of anatomy for complicated surgeries of the chest.
References 1. Yoshino N, Okada D, Ujiie H, et al. Venous hemangioma of the posterior mediastinum. Ann Thorac Cardiovasc Surg 2012;18:247–50. 2. Akiba T, Morikawa T, Hirayama S, Ohki T. Thymic haemangioma presenting with a left innominate vein aneurysm: insight into the aetiology. Interact Cardiovasc Thorac Surg 2012;15:925–7. 3. McAdams HP, Rosado-de-Christenson ML, Moran CA. Mediastinal hemangioma: radiographic and CT features in 14 patients. Radiology 1994;193:399–402. 4. Papagiannopoulos K, Sheppard MN, Goldstraw P. Thymic hemangioma presenting with recurrent pleural effusion. Ann Thorac Surg 2000;70:297–9. 5. Cohen AJ, Sbasching RJ, Hochholzer L, Lough FC, Albus RA. Mediastinal hemangiomas. Ann Thorac Surg 1987;43:656–9. 6. Burkill GJ, Burn PR, Padley SP. Aneurysm of the left brachiocephalic vein: an unusual cause of mediastinal widening. Br J Radiol 1997;70:837–9. 7. Akiba T, Nakada T, Inagaki T. Three-dimensional printing model of anomalous bronchi before surgery. Ann Thorac Cardiovasc Surg 2013 [Epub ahead of print].
Giant Cardiac Cavernous Hemangioma Eric Unger, MD, Joseph Costic, DO, and Glenn Laub, MD Department of Cardiothoracic Surgery, Drexel University College of Medicine, Philadelphia, Pennsylvania
We report the case of an asymptomatic giant cardiac cavernous hemangioma in a 71-year-old man. The intracardiac mass was discovered incidentally during Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
surveillance for his prostate cancer; however, the patient initially declined intervention. On presentation to our institution 7 years later, the lesion had enlarged significantly, and the patient consented to excision. At surgery, an 8 3 6.5 3 4.8 cm intracardiac mass located on the inferior heart border was excised with an intact capsule through a median sternotomy approach. The patient had an uneventful postoperative course. We discuss the diagnostic workup, treatment, and characteristics of this rare cardiac tumor. (Ann Thorac Surg 2015;100:322–5) Ó 2015 by The Society of Thoracic Surgeons
H
emangiomas are a relatively common soft tissue tumor; however, they account for only 2% of primarily resected cardiac tumors [1]. Rarer still is the finding of a concurrent hemangioma elsewhere in the body. Cavernous hemangiomas represent a subtype characterized by thin-walled, dilated vessels. Their clinical course is unpredictable, and surgical excision is considered both diagnostic and curative.
A 71-year-old man with asthma, hypertension, dyslipidemia, and prostate cancer status post–cryosurgery and radiotherapy, was found to have a cardiac mass on the inferior border of the heart during his follow-up prostate cancer surveillance by computed tomography imaging, as well as a 2.4 cm hepatic segment VII lesion. Magnetic resonance imaging further characterized the hepatic lesion as a 6.6 4.7 4.9 cm hypointense T1-weighted and hyperintense T2-weighted lesion at the interatrial septum (Fig 1). The lesion moderately compressed the left atrium and was suspected to be a sarcoma. The patient was asymptomatic from his cardiac mass and declined intervention. Fine-needle aspiration of the hepatic lesion revealed a cavernous hemangioma, which was thought to not require further treatment. As part of a cardiac workup 7 years later, magnetic resonance imaging of the chest showed that the mass had enlarged to 8.7 6.3 5.9 cm, and was located just Accepted for publication Aug 15, 2014. Address correspondence to Dr Unger, 245 N 15th St, MS413, Philadelphia, PA 19102; e-mail: [email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.08.056
Ann Thorac Surg 2015;100:322–5
CASE REPORT UNGER ET AL GIANT CARDIAC CAVERNOUS HEMANGIOMA
323
Fig 1. (A) T2-weighted axial image and (B) coronal image from the initial magnetic resonance imaging. (C, D) The mass was enlarged on repeat magnetic resonance imaging performed 7 years later. On all images, an encapsulated large mass, marked by asterisks, is seen in relation to the cardiac chambers, with mass effect on the right atrium.
FEATURE ARTICLES
inferior to the left atrium, with a resultant mass effect on the esophagus and atria (Fig 1). The mass had intermediate signal intensity on T1-weighted imaging, hyperintensity on T2-weighted imaging, hyperintensity on fat-saturated images, and a heterogeneous appearance on contrast imaging. The hepatic cavernous hemangioma was minimally enlarged (2.7 2.5 1.7 cm). Transesophageal echocardiography suggested an encapsulated mass that was confluent with the right ventricle, mild to moderate mitral regurgitation, trace tricuspid regurgitation, and an ejection fraction of 60% to 65% (Fig 2). Given the tumor growth, uncertain diagnosis, and relationship to the surrounding structures, it was recommended that the tumor be excised. At surgery, a median sternotomy approach was used, and the pericardium was opened. The mass was found to be intracardiac, located under the epicardial surface of the inferior heart border, and extended posteriorly to the level of the atrium (Fig 3). After the inferior heart border was retracted, a plane free from vascularity was developed, and the tumor was able to be shelled out from the surrounding myocardium. The tumor was excised intact (Fig 3). After hemostasis, mediastinal drains were placed and the chest was closed. Postoperatively, the patient made an uneventful recovery except for a brief episode of atrial fibrillation. He was discharged home in stable condition
on postoperative day 4. On pathology, the 8 6.5 4.8 cm, 78 g specimen was confirmed to be a cavernous hemangioma (Fig 4). The tumor was surrounded by fibrous tissue, and it was excised with an intact capsule.
Fig 2. The mass is marked in this echocardiographic image with an asterisk. The mitral valve (MV) and tricuspid valve (TV) are also visualized.
CASE REPORT UNGER ET AL GIANT CARDIAC CAVERNOUS HEMANGIOMA
Ann Thorac Surg 2015;100:322–5
FEATURE ARTICLES
324
Fig 3. (A) The cavernous hemangioma is present under the epicardial surface, at the inferior border of the heart. (B) The gross specimen reveals an intact capsule.
Comment Cardiac hemangiomas are rare benign cardiac accounting for 2% of primarily resected cardiac These tumors can present at any age and have male predominance [1]. Although most
Fig 4. Hematoxylin-and-eosin stained slide (original magnification, 4), showing the prominence of thin-walled, dilated vessels.
tumors, tumors. a slight cardiac
neoplasms are incidentally discovered, these lesions have the potential to cause significant morbidity and mortality secondary to their spatial relation to adjacent structures. Presentations include arrhythmias, conduction disturbances, congestive heart failure, coronary insufficiency, chest pain, cough, and dysphagia. Death due to conduction disturbances or rupture and tamponade is rare [2]. Cardiac hemangiomas are well-circumscribed tumors occurring in the epicardium, myocardium, or endocardium, and have been associated with all cardiac chambers. Hemangiomas can be classified into three groups based on the morphologic pattern of their vascular channels, endothelial cells, and supporting stroma: capillary hemangiomas are composed of small capillarylike vessels, arteriovenous hemangiomas have thick-walled dysplastic vessels, and cavernous hemangiomas contain thin-walled dilated vessels [3]. Histologically, they are composed of proliferations of endothelial cells that form vascular spaces or channels, and are identical to hemangiomas that form elsewhere in the body. Radiographic findings in this case are characteristic of cavernous hemangiomas. On an unenhanced computed tomography scan, hemangiomas are typically well circumscribed and homogeneous in attenuation. Given their vascular nature, they enhance with contrast administration. Cardiac magnetic resonance imaging is superior to computed tomography for soft-tissue characterization, and provides information regarding the enhancement pattern, invasion, and a functional assessment. As in our patient, cardiac hemangiomas are typically isointense to the myocardium on T1-weighted images and hyperintense on T2-weighted images [3]. Although echocardiography provides the benefit of dynamic assessments of cardiac tumors without ionizing radiation, the findings are inconsistent between cavernous hemangiomas. On ultrasonography, these lesions vary in their echogenicity, and are characterized by slow blood flow that may not been seen on duplex Doppler studies [4]. Echocardiography did prove useful for further defining the tumor’s spatial relationships. Although not obtained in this case, coronary angiography does not show marked enhancement in cavernous hemangiomas, as these lesions have large vascular channels with slower blood velocities. In contrast, arteriovenous and capillary hemangiomas reveal a characteristic “tumor blush” [3]. The natural history for cardiac hemangiomas is unpredictable. They may spontaneously regress, stop growing, or proliferate [2]. Complete surgical resection is considered curative, although rare instances of recurrence are reported [4]. Chalet and associates [5] reported a case of a high-grade angiosarcoma excised from the left atrium of a 44-year-old woman 7 years after cardiac hemangioma excision [5]. Although most reported cardiac cavernous hemangiomas are found in isolation, concurrent hemangiomas have been reported. Yang and colleagues [5] reported a patient with a single cardiac hemangioma and multiple pulmonary cavernous hemangiomas discovered on the workup for pericardial and pleural effusions. These led to cardiac and pulmonary failure that progressed to multisystem organ failure and
Ann Thorac Surg 2015;100:325–6
death 2 months after presentation [6]. The decision to operate on our patient was based on the increasing size, uncertain diagnosis, and the inconsistencies in tumor location across imaging modalities. The size of the tumor we excised was among the largest reported cardiac hemangiomas, which is in itself a rare tumor. Our patient also had the rare finding of a second cavernous hemangioma in his liver. Large cardiac tumors, increasing size, symptoms, and an unclear diagnosis are all indications for excision. Although imaging and patient history may suggest a diagnosis of hemangioma, definitive diagnosis and treatment are achieved by surgical excision.
References
Delayed Diagnosis of Q Fever: Finally Diagnosed After Elective Cardiac Surgery Robert Tyler, MRCS, Hannah Povey, MBChB, Sumita Pai, FRCPath, and Catherine Sudarshan, MD, FRCS(CTh) Departments of Cardiothoracic Surgery and Clinical Microbiology, Papworth Hospital NHS Foundation Trust, Papworth Everard, Cambridge, United Kingdom
Q fever is a bacterial infection caused by Coxiella burnetti. It can cause both acute and chronic illness. Chronic QF can present as a variety of clinical syndromes. A common and critical manifestation is endocarditis which can present atypically and is easily missed. This case describes a man who, after extensive investigation for splenomegaly and pancytopenia by several specialties, was finally diagnosed with Q fever endocarditis after unexpected aortic valve abnormalities found during elective cardiac surgery. Several factors contributed to diagnostic delay including aspects of clinical assessment Accepted for publication Aug 25, 2014. Address correspondence to Dr Tyler, Department of Cardiothoracic Surgery, Papworth Hospital, Cambridgeshire CB3 3RE, United Kingdom; e-mail: [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
325
and radiologic findings. Vigilance is essential for diagnosis and prompt initiation of effective treatment. (Ann Thorac Surg 2015;100:325–6) Ó 2015 by The Society of Thoracic Surgeons
T
he bacterial infection called Q fever (QF) is caused by the strict intracellular organism Coxiella burnetti. The common sources of human infection include farm animals such as cattle, sheep, and goats, along with wild animals such as roe-deer [1]. Humans are infected by the inhalation of aerosolized bacteria after exposure to infected products of conception or fomites. QF is often wrongly assumed to be a rarity when in actual fact approximately 5% of the United Kingdom’s general population have serologic evidence of previous exposure, rising to around 15% in farmers [2]. Acute QF is often asymptomatic but may present as a self-limiting illness with flulike symptoms, pneumonia, and hepatitis [2]. Approximately 1% to 5% of persons infected will have chronic QF, which may be quiescent for many years, finally presenting decades after initial infection [3]. Chronic QF can present as a variety of clinical syndromes but most commonly presents as culture negative endocarditis (60% to 70% cases) [2, 3]. Patients at particular risk of having chronic QF include those who have existing valvulopathy, are immunosuppressed, or are pregnant at the time of initial infection. Chronic QF can cause a variety of nonspecific clinical syndromes. Endocarditis caused by QF often presents insidiously and may lack the classical features of endocarditis. Consequently it often proves to be a diagnostic challenge that, without prompt recognition and initiation of effective treatment, can lead to severe and potentially fatal illness [3]. We present a patient who, despite extensive investigation for splenomegaly and pancytopenia, was finally diagnosed with chronic QF as a result of elective cardiac surgery. Infective endocarditis was suspected in the operating theater during elective aortic valve replacement. The causative agent was made apparent by serology, undertaken as a part of the culture negative endocarditis workup, with results positive for QF. A 53-year-old postman was being investigated for splenomegaly and mild pancytopenia. He was otherwise well, with no history of fever or malaise, although he reported very minor weight loss over the preceding 6 months. Medical history included nephrolithiasis. Further investigations, including computed tomography skeletal survey and trephine bone marrow biopsy, were normal. Despite an extensive workup and discussion by several specialties a diagnosis was not reached; the patient was kept under outpatient review pending further investigation. During assessment he complained of gradually worsening exertional dyspnea and was noted to have a cardiac murmur suggestive of mixed aortic valve disease with predominant aortic regurgitation. Transesophageal echocardiography revealed severe aortic regurgitation and moderate aortic stenosis. No vegetations or abscesses were visualized. The left ventricle was severely dilated with normal systolic function. Computed tomography 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.08.055
FEATURE ARTICLES
1. Jain D, Maleszewski JJ, Halushka MK. Benign cardiac tumors and tumorlike conditions. Ann Diagn Pathol 2010;14:215–30. 2. Thomas JE, Eror AT, Kenney M, et al. Asymptomatic right atrial cavernous hemangioma: a case report and review of the literature. Cardiovasc Pathol 2004;13:341–4. 3. Grebenc ML, Rosado de Christenson ML, Burke AP, et al. Primary cardiac and pericardial neoplasms: radiologicpathologic correlation. Radiographics 2000;20:1073–103. 4. Kim TK, Jang HJ, Wilson SR. Hepatic neoplasms: features on grayscale and contrast enhanced ultrasound. Ultrasound Clin 2007;2:333–54. 5. Chalet Y, Mace L, Franc B, Neveux JY, Lancelin B. Angiosarcoma 7 years after surgical excision of histiocytoid haemangioma in left atrium. Lancet 1993;341:1217. 6. Yang L, Dai J, Xiao Y, Cheng H, Ruan Q. Cardiac cavernous hemangioma and multiple pulmonary hemangiomas. Ann Thorac Surg 2014;97:687–9.
CASE REPORT TYLER ET AL DELAYED DIAGNOSIS OF Q FEVER
