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Hepatic hernia resembling cardiac tumor
729
Right-sided hepatic hernia of normal left lobe resembling cardiac tumor Hiroshi Ono,1,2 Hiroki Nagamine,1,2 Akifumi Toyoda,2,4 Nobutaka Shimizu,2 Tatsuo Katori2,5 and Masahiko Sugiyama3 Department of Cardiology, National Medical Center for Children and Mothers, Departments of 2Pediatrics and 3Pediatric Surgery, Graduate School of Medicine, University of Tokyo, 4Department of Pediatrics, Teikyo University and 5Department of Pediatrics, Kanto Central Hospital of the Mutual Aid Association of Public School Teachers, Tokyo, Japan 1
Abstract
A 3-year-old boy was referred for cardiac tumor diagnosed on transthoracic echocardiography (TTE) at another hospital. The tumor appeared to be in the right atrium and obstructed inferior vena cava flow. TTE, enhanced computed tomography (CT) and angiography were done to confirm diagnosis. Subsequently, cardiac tumor was ruled out and he was diagnosed with a very rare condition of hepatic hernia containing a normal left lobe. When cardiac tumor is suspected in the right atrium on the basis of TTE, enhanced CT or magnetic resonance imaging should be done for definitive diagnosis.
Key words cardiac tumor, diaphragmatic hernia, hepatic hernia. Hepatic hernia is diaphragmatic hernia that involves only the liver. This condition may be congenital or may arise from traumatic rupture of the diaphragm. Hepatic hernia containing a normal left lobe is a particularly rare condition,1 and has the potential to encroach on the heart, causing cardiac compression. Therefore, hepatic hernia is often mistaken for cardiac tumor. In this report, we describe a case of hepatic hernia with normal left lobe initially misdiagnosed as right atrial tumor on transthoracic echocardiography (TTE).
Case report The patient was born at 33 weeks of gestation with a younger twin sibling. Birthweight was 1546 g. He had hypoplasia cerebellum and abnormal gyrus. At 1 year of age he developed respiratory syncytial virus encephalitis, which resulted in severe mental retardation and tetraplegia. Prior to the present hospital admission, a doctor was first consulted due to fever. Three days after that the patient experienced facial edema that prompted a visit to a general hospital, where chest X-ray, blood examination and TTE were performed. A large mass in the right atrium (RA), which was the cause of the obstruction to inferior vena cava (IVC) flow, was identified on TTE. The patient was transferred to The University of Tokyo Hospital, Tokyo, at 3 years of age with a diagnosis of cardiac tumor in the RA that caused a mild obstruction to IVC flow. On physical examination, his heart sounds were normal in intensity with no murmur and extracardiac sounds. Peripheral edema was observed, especially in the facial area. White blood cell (WBC) count was low. There was mild liver dysfunction and elevated Correspondence: Hiroshi Ono, MD, Department of Cardiology, National Medical Center for Children and Mothers, 2-10-1 Okura, Setagaya-ku, Tokyo 157-8535, Japan. Email: [email protected] Received 25 April 2014; revised 24 October 2014; accepted 17 November 2014. doi: 10.1111/ped.12579
creatinine phosphokinase (CPK) and C-reactive protein (CRP). Viral infection was initially suspected on blood chemistry. B-type natriuretic peptide (BNP) rose to 169.5 pg/mL. Obvious abnormal findings were not detected on chest X-ray or electrocardiogram. A large, round mass in the RA was confirmed on TTE (Fig. 1). The mass was 25 mm in diameter. Further TTE showed that the mass extended to the liver, therefore enhanced computed tomography (CT) was performed, which showed that the mass was part of the liver (Fig. 2): a so-called hepatic hernia. Tumor markers human chorionic gonadotrophin (HCG), β-HCG, α-fetoprotein, CA19-9 and CA125 were all measured and found to be in the normal range.
RV
LA RA
Fig. 1 Large, high-echogenicity mass (25 × 25 mm : arrows) in the right atrium (RA) on transthoracic echocardiography. LA, left atrium; RV, right ventricle. © 2015 Japan Pediatric Society
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H Ono et al.
Fig. 2 Large mass (*) resembling cardiac tumor in the right atrium (RA) on (a) enhanced computed tomography and (b) transthoracic echocardiography (subcostal view). The mass was suspected to contain normal left lobe of the liver. IVC, inferior vena cava.
a
b
*
liver
liver
Given that IVC flow was obstructed on TTE, cardiac catheter examination was therefore done. The mass was compressing the RA and IVC, causing blood flow to be mildly obstructed (Fig. 3). The mean pressure gradient between the IVC and RA was 2 mmHg. There were no abnormal arteries in the descending aorta or common hepatic artery to the mass. Subsequently, cardiac tumor was ruled out and the patient was diagnosed with hepatic hernia containing a normal left lobe. We opted for close observation instead of surgical intervention. His fever was eventually brought down and facial edema regressed promptly. WBC count, asparate aminotransferase and alanine aminotransferase, CPK, CRP and BNP returned to normal over time.
Discussion At present there are only a few reports of hepatic hernia, a rare condition that is usually mistaken for cardiac tumor.1 The most prominent cause of hepatic hernia is traumatic rupture of the diaphragm. In the present case, however, there was no episode of
*
IVC
*
RA
trauma or abdominal compression. Hence, the hepatic hernia was most likely congenital or due to fragility of the diaphragm. Although hepatic hernia resembling RA mass has been reported before, this is the first time that hepatic hernia containing a normal left lobe in childhood has been described. Cardiac masses that are actually not cardiac tumor include intra-cardiac masses and extra-cardiac masses. The former encompasses thrombus and septic vegetation, while the latter encompasses mediastinal mass, metastatic tumor and hiatus hernia.2–6 The clinical presentations of hiatus hernia-induced cardiac compression are tamponade, obstruction, respiratory failure, acute heart failure, and hemodynamic collapse and so on.4 In the present case, there was mild obstruction to the IVC flow due to compression of the hernia. Although facial edema was present, we did not think it was related to the hernia because it was resolved without therapy for IVC or atrial compression. Although TTE is a useful tool for the detection of both intracardiac masses and extra-cardiac mediastinal masses attached to the heart,6 it does not allow definitive diagnosis of conditions such as hiatus hernia or mediastinal mass (tumor or cyst) adjacent to the heart.7 And although the present patient was referred with a diagnosis of cardiac tumor, the diagnosis of hepatic hernia was eventually confirmed on enhanced CT. The consumption of a carbonated beverage prior to TTE has been reported to be useful for distinguishing cardiac mass from hiatus hernia.8 In the present case, given that the stomach was not involved, such a practice would not have been helpful. Conclusion
This is the first infant case of hepatic hernia mimicking right atrial tumor. When cardiac tumor in the RA is suspected on the basis of TTE, enhanced CT or magnetic resonance imaging is useful in establishing a definitive diagnosis, as seen in the present case in which hepatic hernia was initially misdiagnosed as cardiac tumor.
Acknowledgments
Fig. 3 Angiography of the inferior vena cava (IVC). The mass in the right atrium (*) interfered mildly with IVC flow. © 2015 Japan Pediatric Society
We thank Dr Julian Tang from the Department of Education for Clinical Research, National Center for Child Health and Development, for assistance with the manuscript. The authors state no conflicts of interest.
Hepatic hernia resembling cardiac tumor References 1 Fujimoto S, Nakagawa Y, Mizuno R, Dohi K, Otsuji H, Nakano H. A case of hepatic hernia with echocardiographic findings simulating right atrial tumor. J. Am. Soc. Echocardiogr. 1998; 11: 680–82. 2 Chan J, Manning WJ, Appelbaum E, Smith P, Rice K. Large hiatal hernia mimicking left atrial mass: A multimodality diagnosis. J. Am. Coll. Cardiol. 2009; 54: 569. 3 Ker J, Van Beljon J. Diaphragmatic hernia mimicking an atrial mass: A two-dimensional echocardiographic pitfall and a cause of postprandial syncope. Cardiovasc. J. South Afr. 2004; 15: 182–3. 4 Baerman JM, Hogan L, Swiryn S. Diaphragmatic hernia producing symptoms and signs of a left atrial mass. Am. Heart J. 1988; 116: 198–200.
731
5 Koskinas KC, Oikonomou K, Karapatsoudi E, Makridis P. Echocardiographic manifestation of hiatus hernia simulating a left atrial mass: Case report. Cardiovasc. Ultrasound 2008; 6: 46. 6 D’Cruz IA, Feghali N, Gross CM. Echocardiographic manifestations of mediastinal masses compressing or encroaching on the heart. Echocardiography 1994; 11: 523–33. 7 Nishimura RA, Tajik AJ, Schattenberg TT, Seward JB. Diaphragmatic hernia mimicking an atrial mass: A two-dimensional echocardiographic pitfall. J. Am. Coll. Cardiol. 1985; 5: 992–5. 8 Smelley M, Lang RM. Large mass impinging on the left atrium: Diagnostic value of a new cocktail. J. Am. Soc. Echocardiogr. 2007; 20: 1414.e5–7.
Pulmonary arterial hypertension associated with chronic active Epstein–Barr virus infection Yutaka Fukuda,1 Nobuo Momoi,2 Mitsuko Akaihata,1 Katsutoshi Nagasawa,1 Masaki Mitomo,2 Yoshimichi Aoyagi,2 Kisei Endoh2 and Mitsuaki Hosoya2 1 Department of Pediatrics, Takeda General Hospital and 2Department of Pediatrics, Fukushima Medical University, Fukushima, Japan Abstract
Chronic active Epstein–Barr virus (EBV) infection (CAEBV), characterized by persistent infectious mononucleosis-like symptoms, can lead to cardiovascular complications including coronary artery aneurysm or myocarditis. Here, we present the case of an 11-year-old boy with pulmonary arterial hypertension (PAH) and junctional ectopic tachycardia associated with CAEBV. The patient did not have any major symptoms attributed to CAEBV, such as fever, lymphadenopathy or splenomegaly when the PAH developed. Mild liver dysfunction was found at the first examination, and it persisted. Two years after the PAH symptoms appeared, CAEBV was evident, based on deteriorated liver function, hepatosplenomegaly, and coronary artery aneurysms. CAEBV should be considered as a cause of secondary PAH, particularly when liver dysfunction coexists.
Key words chronic active Epstein–Barr virus infection, coronary artery aneurysm, junctional ectopic tachycardia, pulmonary arterial hypertension. Chronic active Epstein–Barr virus (EBV) infection (CAEBV) is characterized by chronic or recurrent infectious mononucleosislike symptoms, such as fever, lymphadenopathy, hepatitis and hepatosplenomegaly.1,2 A severe form of CAEBV involves a lymphoproliferative disorder that is induced by clonal expansion of EBV-infected T or natural killer (NK) cells, and is prevalent in East Asian countries.2,3 CAEBV often leads to serious complications such as virus-associated hemophagocytic syndrome, disseminated intravascular coagulation, interstitial pneumonia, liver failure and cardiovascular disease. Coronary artery aneurysm and myocarditis are the major cardiovascular complications correlated with poor prognosis of CAEBV.3,4 Here, we report a rare Correspondence: Yutaka Fukuda, MD, Department of Pediatrics, Takeda General Hospital, 3-27 Yamagamachi Aizu-Wakamatsu City, Fukushima 965-8585, Japan. Email: [email protected] Received 17 June 2014; revised 13 October 2014; accepted 17 November 2014. doi: 10.1111/ped.12578
case of pulmonary arterial hypertension (PAH) and junctional ectopic tachycardia (JET), symptoms of which developed prior to the diagnosis of CAEBV.
Case report A 6-year-old Japanese boy visited hospital to undergo investigation of abnormal findings on school electrocardiogram (ECG) screening. Complete right bundle branch block (CRBBB; Fig. 1a) was noted on ECG. Echocardiography was performed to check cardiac function and anatomy, but no abnormalities were noted. Five years later, at the age of 11, he visited hospital again complaining of motion fatigue. ECG findings differed from those of 5 years earlier. The presence of CRBBB with a tall R wave in V1 and right axis deviation of +180° were considered to be suggestive of right ventricular hypertrophy (Fig. 1b). Echocardiographic findings including dilation of the right atrium and right ventricle chamber, and abnormal shape of the © 2015 Japan Pediatric Society
Hepatic hernia resembling cardiac tumor
729
Right-sided hepatic hernia of normal left lobe resembling cardiac tumor Hiroshi Ono,1,2 Hiroki Nagamine,1,2 Akifumi Toyoda,2,4 Nobutaka Shimizu,2 Tatsuo Katori2,5 and Masahiko Sugiyama3 Department of Cardiology, National Medical Center for Children and Mothers, Departments of 2Pediatrics and 3Pediatric Surgery, Graduate School of Medicine, University of Tokyo, 4Department of Pediatrics, Teikyo University and 5Department of Pediatrics, Kanto Central Hospital of the Mutual Aid Association of Public School Teachers, Tokyo, Japan 1
Abstract
A 3-year-old boy was referred for cardiac tumor diagnosed on transthoracic echocardiography (TTE) at another hospital. The tumor appeared to be in the right atrium and obstructed inferior vena cava flow. TTE, enhanced computed tomography (CT) and angiography were done to confirm diagnosis. Subsequently, cardiac tumor was ruled out and he was diagnosed with a very rare condition of hepatic hernia containing a normal left lobe. When cardiac tumor is suspected in the right atrium on the basis of TTE, enhanced CT or magnetic resonance imaging should be done for definitive diagnosis.
Key words cardiac tumor, diaphragmatic hernia, hepatic hernia. Hepatic hernia is diaphragmatic hernia that involves only the liver. This condition may be congenital or may arise from traumatic rupture of the diaphragm. Hepatic hernia containing a normal left lobe is a particularly rare condition,1 and has the potential to encroach on the heart, causing cardiac compression. Therefore, hepatic hernia is often mistaken for cardiac tumor. In this report, we describe a case of hepatic hernia with normal left lobe initially misdiagnosed as right atrial tumor on transthoracic echocardiography (TTE).
Case report The patient was born at 33 weeks of gestation with a younger twin sibling. Birthweight was 1546 g. He had hypoplasia cerebellum and abnormal gyrus. At 1 year of age he developed respiratory syncytial virus encephalitis, which resulted in severe mental retardation and tetraplegia. Prior to the present hospital admission, a doctor was first consulted due to fever. Three days after that the patient experienced facial edema that prompted a visit to a general hospital, where chest X-ray, blood examination and TTE were performed. A large mass in the right atrium (RA), which was the cause of the obstruction to inferior vena cava (IVC) flow, was identified on TTE. The patient was transferred to The University of Tokyo Hospital, Tokyo, at 3 years of age with a diagnosis of cardiac tumor in the RA that caused a mild obstruction to IVC flow. On physical examination, his heart sounds were normal in intensity with no murmur and extracardiac sounds. Peripheral edema was observed, especially in the facial area. White blood cell (WBC) count was low. There was mild liver dysfunction and elevated Correspondence: Hiroshi Ono, MD, Department of Cardiology, National Medical Center for Children and Mothers, 2-10-1 Okura, Setagaya-ku, Tokyo 157-8535, Japan. Email: [email protected] Received 25 April 2014; revised 24 October 2014; accepted 17 November 2014. doi: 10.1111/ped.12579
creatinine phosphokinase (CPK) and C-reactive protein (CRP). Viral infection was initially suspected on blood chemistry. B-type natriuretic peptide (BNP) rose to 169.5 pg/mL. Obvious abnormal findings were not detected on chest X-ray or electrocardiogram. A large, round mass in the RA was confirmed on TTE (Fig. 1). The mass was 25 mm in diameter. Further TTE showed that the mass extended to the liver, therefore enhanced computed tomography (CT) was performed, which showed that the mass was part of the liver (Fig. 2): a so-called hepatic hernia. Tumor markers human chorionic gonadotrophin (HCG), β-HCG, α-fetoprotein, CA19-9 and CA125 were all measured and found to be in the normal range.
RV
LA RA
Fig. 1 Large, high-echogenicity mass (25 × 25 mm : arrows) in the right atrium (RA) on transthoracic echocardiography. LA, left atrium; RV, right ventricle. © 2015 Japan Pediatric Society
730
H Ono et al.
Fig. 2 Large mass (*) resembling cardiac tumor in the right atrium (RA) on (a) enhanced computed tomography and (b) transthoracic echocardiography (subcostal view). The mass was suspected to contain normal left lobe of the liver. IVC, inferior vena cava.
a
b
*
liver
liver
Given that IVC flow was obstructed on TTE, cardiac catheter examination was therefore done. The mass was compressing the RA and IVC, causing blood flow to be mildly obstructed (Fig. 3). The mean pressure gradient between the IVC and RA was 2 mmHg. There were no abnormal arteries in the descending aorta or common hepatic artery to the mass. Subsequently, cardiac tumor was ruled out and the patient was diagnosed with hepatic hernia containing a normal left lobe. We opted for close observation instead of surgical intervention. His fever was eventually brought down and facial edema regressed promptly. WBC count, asparate aminotransferase and alanine aminotransferase, CPK, CRP and BNP returned to normal over time.
Discussion At present there are only a few reports of hepatic hernia, a rare condition that is usually mistaken for cardiac tumor.1 The most prominent cause of hepatic hernia is traumatic rupture of the diaphragm. In the present case, however, there was no episode of
*
IVC
*
RA
trauma or abdominal compression. Hence, the hepatic hernia was most likely congenital or due to fragility of the diaphragm. Although hepatic hernia resembling RA mass has been reported before, this is the first time that hepatic hernia containing a normal left lobe in childhood has been described. Cardiac masses that are actually not cardiac tumor include intra-cardiac masses and extra-cardiac masses. The former encompasses thrombus and septic vegetation, while the latter encompasses mediastinal mass, metastatic tumor and hiatus hernia.2–6 The clinical presentations of hiatus hernia-induced cardiac compression are tamponade, obstruction, respiratory failure, acute heart failure, and hemodynamic collapse and so on.4 In the present case, there was mild obstruction to the IVC flow due to compression of the hernia. Although facial edema was present, we did not think it was related to the hernia because it was resolved without therapy for IVC or atrial compression. Although TTE is a useful tool for the detection of both intracardiac masses and extra-cardiac mediastinal masses attached to the heart,6 it does not allow definitive diagnosis of conditions such as hiatus hernia or mediastinal mass (tumor or cyst) adjacent to the heart.7 And although the present patient was referred with a diagnosis of cardiac tumor, the diagnosis of hepatic hernia was eventually confirmed on enhanced CT. The consumption of a carbonated beverage prior to TTE has been reported to be useful for distinguishing cardiac mass from hiatus hernia.8 In the present case, given that the stomach was not involved, such a practice would not have been helpful. Conclusion
This is the first infant case of hepatic hernia mimicking right atrial tumor. When cardiac tumor in the RA is suspected on the basis of TTE, enhanced CT or magnetic resonance imaging is useful in establishing a definitive diagnosis, as seen in the present case in which hepatic hernia was initially misdiagnosed as cardiac tumor.
Acknowledgments
Fig. 3 Angiography of the inferior vena cava (IVC). The mass in the right atrium (*) interfered mildly with IVC flow. © 2015 Japan Pediatric Society
We thank Dr Julian Tang from the Department of Education for Clinical Research, National Center for Child Health and Development, for assistance with the manuscript. The authors state no conflicts of interest.
Hepatic hernia resembling cardiac tumor References 1 Fujimoto S, Nakagawa Y, Mizuno R, Dohi K, Otsuji H, Nakano H. A case of hepatic hernia with echocardiographic findings simulating right atrial tumor. J. Am. Soc. Echocardiogr. 1998; 11: 680–82. 2 Chan J, Manning WJ, Appelbaum E, Smith P, Rice K. Large hiatal hernia mimicking left atrial mass: A multimodality diagnosis. J. Am. Coll. Cardiol. 2009; 54: 569. 3 Ker J, Van Beljon J. Diaphragmatic hernia mimicking an atrial mass: A two-dimensional echocardiographic pitfall and a cause of postprandial syncope. Cardiovasc. J. South Afr. 2004; 15: 182–3. 4 Baerman JM, Hogan L, Swiryn S. Diaphragmatic hernia producing symptoms and signs of a left atrial mass. Am. Heart J. 1988; 116: 198–200.
731
5 Koskinas KC, Oikonomou K, Karapatsoudi E, Makridis P. Echocardiographic manifestation of hiatus hernia simulating a left atrial mass: Case report. Cardiovasc. Ultrasound 2008; 6: 46. 6 D’Cruz IA, Feghali N, Gross CM. Echocardiographic manifestations of mediastinal masses compressing or encroaching on the heart. Echocardiography 1994; 11: 523–33. 7 Nishimura RA, Tajik AJ, Schattenberg TT, Seward JB. Diaphragmatic hernia mimicking an atrial mass: A two-dimensional echocardiographic pitfall. J. Am. Coll. Cardiol. 1985; 5: 992–5. 8 Smelley M, Lang RM. Large mass impinging on the left atrium: Diagnostic value of a new cocktail. J. Am. Soc. Echocardiogr. 2007; 20: 1414.e5–7.
Pulmonary arterial hypertension associated with chronic active Epstein–Barr virus infection Yutaka Fukuda,1 Nobuo Momoi,2 Mitsuko Akaihata,1 Katsutoshi Nagasawa,1 Masaki Mitomo,2 Yoshimichi Aoyagi,2 Kisei Endoh2 and Mitsuaki Hosoya2 1 Department of Pediatrics, Takeda General Hospital and 2Department of Pediatrics, Fukushima Medical University, Fukushima, Japan Abstract
Chronic active Epstein–Barr virus (EBV) infection (CAEBV), characterized by persistent infectious mononucleosis-like symptoms, can lead to cardiovascular complications including coronary artery aneurysm or myocarditis. Here, we present the case of an 11-year-old boy with pulmonary arterial hypertension (PAH) and junctional ectopic tachycardia associated with CAEBV. The patient did not have any major symptoms attributed to CAEBV, such as fever, lymphadenopathy or splenomegaly when the PAH developed. Mild liver dysfunction was found at the first examination, and it persisted. Two years after the PAH symptoms appeared, CAEBV was evident, based on deteriorated liver function, hepatosplenomegaly, and coronary artery aneurysms. CAEBV should be considered as a cause of secondary PAH, particularly when liver dysfunction coexists.
Key words chronic active Epstein–Barr virus infection, coronary artery aneurysm, junctional ectopic tachycardia, pulmonary arterial hypertension. Chronic active Epstein–Barr virus (EBV) infection (CAEBV) is characterized by chronic or recurrent infectious mononucleosislike symptoms, such as fever, lymphadenopathy, hepatitis and hepatosplenomegaly.1,2 A severe form of CAEBV involves a lymphoproliferative disorder that is induced by clonal expansion of EBV-infected T or natural killer (NK) cells, and is prevalent in East Asian countries.2,3 CAEBV often leads to serious complications such as virus-associated hemophagocytic syndrome, disseminated intravascular coagulation, interstitial pneumonia, liver failure and cardiovascular disease. Coronary artery aneurysm and myocarditis are the major cardiovascular complications correlated with poor prognosis of CAEBV.3,4 Here, we report a rare Correspondence: Yutaka Fukuda, MD, Department of Pediatrics, Takeda General Hospital, 3-27 Yamagamachi Aizu-Wakamatsu City, Fukushima 965-8585, Japan. Email: [email protected] Received 17 June 2014; revised 13 October 2014; accepted 17 November 2014. doi: 10.1111/ped.12578
case of pulmonary arterial hypertension (PAH) and junctional ectopic tachycardia (JET), symptoms of which developed prior to the diagnosis of CAEBV.
Case report A 6-year-old Japanese boy visited hospital to undergo investigation of abnormal findings on school electrocardiogram (ECG) screening. Complete right bundle branch block (CRBBB; Fig. 1a) was noted on ECG. Echocardiography was performed to check cardiac function and anatomy, but no abnormalities were noted. Five years later, at the age of 11, he visited hospital again complaining of motion fatigue. ECG findings differed from those of 5 years earlier. The presence of CRBBB with a tall R wave in V1 and right axis deviation of +180° were considered to be suggestive of right ventricular hypertrophy (Fig. 1b). Echocardiographic findings including dilation of the right atrium and right ventricle chamber, and abnormal shape of the © 2015 Japan Pediatric Society
