□
CASE REPORT
□
An Adult Case of Polysplenia Syndrome Associated with Sinus Node Dysfunction, Dextrocardia, and Systemic Venous Anomalies Kentaro Fukuda, Toshihito Onda, Yuki Kimura, Seiji Miura, Rie Matsumori, Yoshiyuki Masaki, Akihisa Nishino, Kenji Inoue, Yasumasa Fujiwara and Masataka Sumiyoshi
Abstract A 54-year-old woman was referred to our hospital for symptomatic sinus bradyarrhythmia with a sinus pause of 8 seconds. She was diagnosed with dextrocardia during childhood and discovered to have heterotaxy syndrome when she had an appendectomy during her teenager years. Chest and abdominal examinations by computed tomography showed multiple spleens located on the right side and abnormal drainages of the superior and inferior vena cava. Left isomerism was diagnosed by bilaterally bilobed lungs. Because of a patent bilateral superior vena cava, pacemaker leads were implanted using the right cephalic vein approach. Her fainting symptoms disappeared after pacemaker implantation. Key words: polysplenia, sinus node dysfunction, left isomerism, venous anomaly, dextrocardia, cardiac pacing (Intern Med 54: 1071-1074, 2015) (DOI: 10.2169/internalmedicine.54.3354)
Introduction Polysplenia syndrome is a rare congenital anomaly that may be found incidentally in adults who undergo abdominal computed tomography (CT) (1). We herein describe an adult case of polysplenia syndrome associated with sinus node dysfunction which required cardiac pacemaker implantation.
Case Report The patient was a 54-year-old woman employed as a cleaning attendant on bullet trains. She had been experiencing fainting sensations upon exertion for the past 2 years. This symptom had occurred only once or twice a month in the previous 2 years, but the frequency had increased to several times a week over the past year and continued up until presentation. However, no syncope occurred. When she was referred to a nearby clinic for phlegmon in her leg, sinus
bradycardia (36 bpm) was incidentally discovered. Ambulatory Holter electrocardiography (ECG) monitoring performed at this clinic revealed a sinus pause of 8 seconds after cessation of atrial fibrillation (AF) and flutter (AFL) with a fainting sensation (Fig. 1). Therefore, the patient was referred to our hospital with a diagnosis of symptomatic sinus node dysfunction. She had been diagnosed with dextrocardia during childhood. She was discovered to have heterotaxy during an appendectomy in her teens. She also had a cholecystectomy for cholecystitis in her twenties. Her family history was unremarkable without any congenital anomaly or heart disease. On admission, she was small and slightly obese with a height of 151 cm and body weight of 61.4 kg (BMI of 26.9). Her blood pressure and pulse rate were 98/46 mmHg and 30 bpm with a regular rhythm, respectively. On her physical examination, a systolic murmur was audible at the apex with a grade of Levine 2/6. No hepatomegaly or edema at her low extremities was observed. A blood exami-
Department of Cardiology, Juntendo University Nerima Hospital, Japan Received for publication May 24, 2014; Accepted for publication September 21, 2014 Correspondence to Dr. Masataka Sumiyoshi, [email protected]
1071
Intern Med 54: 1071-1074, 2015
DOI: 10.2169/internalmedicine.54.3354
Figure 1. Ambulatory Holter ECG monitoring revealed a sinus pause of 8 seconds with a fainting sensation.
Figure 2. A: A chest X-ray showed cardiomegaly, a cardiothoracic rate of 56%, and dextrocardia. B: Abdominal computed tomography (CT) revealed multiple spleens located on the right side (arrow).
Figure 3. The 12-lead ECG on admission showed marked bradycardia (36 bpm) with junctional escapes.
nation was unremarkable except for a mild elevation of her serum NTproBNP at 279 pg/mL. A chest X-ray showed cardiomegaly, a cardiothoracic ratio (CTR) of 56%, and dextrocardia but no lung edema (Fig. 2A). We performed echocardiography, which showed no cardiac anomaly or valvular
disease but dilatation of the coronary sinus. The 12-lead ECG on admission revealed marked bradycardia with junctional escapes (Fig. 3). Although she had dextrocardia, the P wave vector was positive in II, III, aVF, and negative in I and both aVL and aVR, which suggested origin in the upper
1072
Intern Med 54: 1071-1074, 2015
DOI: 10.2169/internalmedicine.54.3354
Figure 4. Left isomerism was diagnosed by bilaterally bilobed lungs on chest CT.
Figure 5. Chest and abdominal examination using CT. CT with intravenous contrast material revealed abnormal drainages of the superior and inferior vena cava (SVC and IVC): the double SVC (arrows in A) draining into the right atrium (RA) separately and the double IVC (arrows in B) meeting together at the hepatic level and draining into the RA.
right atrium. The patient was diagnosed with sick sinus syndrome (SSS), and we thus decided to implant a cardiac pacemaker. Before implantation of the cardiac pacemaker, we performed chest and abdominal CT. We found multiple
spleens located on the right side (Fig. 2B, arrow). Left isomerism was also diagnosed by bilaterally bilobed lungs (Fig. 4). We diagnosed the patient with polysplenia syndrome and heterotaxia associated with SSS. Because
1073
Intern Med 54: 1071-1074, 2015
DOI: 10.2169/internalmedicine.54.3354
polysplenia syndrome is typically associated with various venous anomalies, we performed CT with intravenous contrast material which revealed abnormal drainages of the superior and inferior vena cava (SVC and IVC): the right SVC drained directly into the right atrium, whereas the left SVC drained into the right atrium via the coronary sinus (Fig. 5A). Bilateral IVC met together at the hepatic level and drained into the right atrium (Fig. 5B). Because of a patent right SVC, pacemaker leads were easily implanted using the right cephalic vein approach. Atrial and ventricular leads were fixed at the right atrial free wall and the right ventricular apex using active fixation leads, respectively. After pacemaker implantation, we did not treat her AF/AFL because of well-controlled heart rates and no AF/AFL symptoms. In addition, she had no risk factors for thromboembolic events of AF; her CHADS2 score was zero. The patient’s fainting symptoms disappeared after pacemaker implantation.
to the development of the right appendage; therefore, in right isomerism two sinus nodes are usually found; conversely, a sinus node tends to be hypoplastic or absent in left isomerism (4), which may have been the cause of sinus node dysfunction in our patient. The Pitx2 gene, which has recently been reported to play a crucial role for left-right asymmetry in the sinus venous myocardium (5), may contribute to the occurrence of sinus node dysfunction in left isomerism. Patients with polysplenia syndrome usually have some abnormal venous drainage (1). These abnormal systemic venous channels may pose technical difficulties for transvenous pacing (6). In our case, PLSVC was present; however, we could easily implant transvenous pacing leads because of the presence of double SVC. In patients with polysplenia syndrome, anatomical information of venous drainage, especially in SVC, is essential for transvenous pacemaker implantation. The authors state that they have no Conflict of Interest (COI).
Discussion Polysplenia syndrome is a type of situs ambiguous characterized by left isomerism (bilateral left sidedness) (1). This syndrome is commonly known to be associated with various cardiac anomalies, abnormal venous drainages, and abdominal heterotaxia, etc. The majority of patients with polysplenia syndrome have been reported to die by the age of 5 years due to severe cardiac anomalies, but less than 10% of patients have normal hearts or only minor cardiac defects and reach adulthood without symptoms (1). In such cases, polysplenia syndrome is often discovered incidentally during adulthood when the patient undergoes abdominal CT, such as in our patient. Our patient had an almost normal heart anatomy except for persistent left superior vena cava (PLSVC) and dextrocardia. Sinus node dysfunction is a common arrhythmia in patients with polysplenia syndrome because of bilateral left sidedness (left isomerism) (2, 3). The development of the sinus node tissue is strongly related
References 1. Gayer G, Apter S, Jonas T, et al. Polysplenia syndrome detected in adulthood: report of eight cases and review of the literature. Abdom Imaging 24: 178-184, 1999. 2. Wu M-H, Wang J-K, Lin J-L, et al. Cardiac rhythm disturbances in patients with left atrial isomerism. PACE 24: 1631-1638, 2001. 3. Ferrero P, Massa R, Amellone C, Trevi G. Sinus node dysfunction associated with left atrial isomerism. J Cardiovasc Med 9: 953956, 2008. 4. Ho SY, Seo J-W, Brown NA, et al. Morphology of the sinus node in human and mouse hearts with isomerism of the atrial appendages. Br Heart J 74: 437-442, 1995. 5. Ammirabile G, Tessari A, Pignataro V, et al. Pitx2 confers left morphological, molecular, and functional identity to the sinus venosus myocardium. Cardiovasc Res 93: 291-301, 2012. 6. Frogoudaki A, Sutton R, Gatzoulis MA. Pacing for adult patients with left atrial isomerism: efficacy and technical considerations. Europace 5: 189-193, 2003.
Ⓒ 2015 The Japanese Society of Internal Medicine http://www.naika.or.jp/imonline/index.html
1074
CASE REPORT
□
An Adult Case of Polysplenia Syndrome Associated with Sinus Node Dysfunction, Dextrocardia, and Systemic Venous Anomalies Kentaro Fukuda, Toshihito Onda, Yuki Kimura, Seiji Miura, Rie Matsumori, Yoshiyuki Masaki, Akihisa Nishino, Kenji Inoue, Yasumasa Fujiwara and Masataka Sumiyoshi
Abstract A 54-year-old woman was referred to our hospital for symptomatic sinus bradyarrhythmia with a sinus pause of 8 seconds. She was diagnosed with dextrocardia during childhood and discovered to have heterotaxy syndrome when she had an appendectomy during her teenager years. Chest and abdominal examinations by computed tomography showed multiple spleens located on the right side and abnormal drainages of the superior and inferior vena cava. Left isomerism was diagnosed by bilaterally bilobed lungs. Because of a patent bilateral superior vena cava, pacemaker leads were implanted using the right cephalic vein approach. Her fainting symptoms disappeared after pacemaker implantation. Key words: polysplenia, sinus node dysfunction, left isomerism, venous anomaly, dextrocardia, cardiac pacing (Intern Med 54: 1071-1074, 2015) (DOI: 10.2169/internalmedicine.54.3354)
Introduction Polysplenia syndrome is a rare congenital anomaly that may be found incidentally in adults who undergo abdominal computed tomography (CT) (1). We herein describe an adult case of polysplenia syndrome associated with sinus node dysfunction which required cardiac pacemaker implantation.
Case Report The patient was a 54-year-old woman employed as a cleaning attendant on bullet trains. She had been experiencing fainting sensations upon exertion for the past 2 years. This symptom had occurred only once or twice a month in the previous 2 years, but the frequency had increased to several times a week over the past year and continued up until presentation. However, no syncope occurred. When she was referred to a nearby clinic for phlegmon in her leg, sinus
bradycardia (36 bpm) was incidentally discovered. Ambulatory Holter electrocardiography (ECG) monitoring performed at this clinic revealed a sinus pause of 8 seconds after cessation of atrial fibrillation (AF) and flutter (AFL) with a fainting sensation (Fig. 1). Therefore, the patient was referred to our hospital with a diagnosis of symptomatic sinus node dysfunction. She had been diagnosed with dextrocardia during childhood. She was discovered to have heterotaxy during an appendectomy in her teens. She also had a cholecystectomy for cholecystitis in her twenties. Her family history was unremarkable without any congenital anomaly or heart disease. On admission, she was small and slightly obese with a height of 151 cm and body weight of 61.4 kg (BMI of 26.9). Her blood pressure and pulse rate were 98/46 mmHg and 30 bpm with a regular rhythm, respectively. On her physical examination, a systolic murmur was audible at the apex with a grade of Levine 2/6. No hepatomegaly or edema at her low extremities was observed. A blood exami-
Department of Cardiology, Juntendo University Nerima Hospital, Japan Received for publication May 24, 2014; Accepted for publication September 21, 2014 Correspondence to Dr. Masataka Sumiyoshi, [email protected]
1071
Intern Med 54: 1071-1074, 2015
DOI: 10.2169/internalmedicine.54.3354
Figure 1. Ambulatory Holter ECG monitoring revealed a sinus pause of 8 seconds with a fainting sensation.
Figure 2. A: A chest X-ray showed cardiomegaly, a cardiothoracic rate of 56%, and dextrocardia. B: Abdominal computed tomography (CT) revealed multiple spleens located on the right side (arrow).
Figure 3. The 12-lead ECG on admission showed marked bradycardia (36 bpm) with junctional escapes.
nation was unremarkable except for a mild elevation of her serum NTproBNP at 279 pg/mL. A chest X-ray showed cardiomegaly, a cardiothoracic ratio (CTR) of 56%, and dextrocardia but no lung edema (Fig. 2A). We performed echocardiography, which showed no cardiac anomaly or valvular
disease but dilatation of the coronary sinus. The 12-lead ECG on admission revealed marked bradycardia with junctional escapes (Fig. 3). Although she had dextrocardia, the P wave vector was positive in II, III, aVF, and negative in I and both aVL and aVR, which suggested origin in the upper
1072
Intern Med 54: 1071-1074, 2015
DOI: 10.2169/internalmedicine.54.3354
Figure 4. Left isomerism was diagnosed by bilaterally bilobed lungs on chest CT.
Figure 5. Chest and abdominal examination using CT. CT with intravenous contrast material revealed abnormal drainages of the superior and inferior vena cava (SVC and IVC): the double SVC (arrows in A) draining into the right atrium (RA) separately and the double IVC (arrows in B) meeting together at the hepatic level and draining into the RA.
right atrium. The patient was diagnosed with sick sinus syndrome (SSS), and we thus decided to implant a cardiac pacemaker. Before implantation of the cardiac pacemaker, we performed chest and abdominal CT. We found multiple
spleens located on the right side (Fig. 2B, arrow). Left isomerism was also diagnosed by bilaterally bilobed lungs (Fig. 4). We diagnosed the patient with polysplenia syndrome and heterotaxia associated with SSS. Because
1073
Intern Med 54: 1071-1074, 2015
DOI: 10.2169/internalmedicine.54.3354
polysplenia syndrome is typically associated with various venous anomalies, we performed CT with intravenous contrast material which revealed abnormal drainages of the superior and inferior vena cava (SVC and IVC): the right SVC drained directly into the right atrium, whereas the left SVC drained into the right atrium via the coronary sinus (Fig. 5A). Bilateral IVC met together at the hepatic level and drained into the right atrium (Fig. 5B). Because of a patent right SVC, pacemaker leads were easily implanted using the right cephalic vein approach. Atrial and ventricular leads were fixed at the right atrial free wall and the right ventricular apex using active fixation leads, respectively. After pacemaker implantation, we did not treat her AF/AFL because of well-controlled heart rates and no AF/AFL symptoms. In addition, she had no risk factors for thromboembolic events of AF; her CHADS2 score was zero. The patient’s fainting symptoms disappeared after pacemaker implantation.
to the development of the right appendage; therefore, in right isomerism two sinus nodes are usually found; conversely, a sinus node tends to be hypoplastic or absent in left isomerism (4), which may have been the cause of sinus node dysfunction in our patient. The Pitx2 gene, which has recently been reported to play a crucial role for left-right asymmetry in the sinus venous myocardium (5), may contribute to the occurrence of sinus node dysfunction in left isomerism. Patients with polysplenia syndrome usually have some abnormal venous drainage (1). These abnormal systemic venous channels may pose technical difficulties for transvenous pacing (6). In our case, PLSVC was present; however, we could easily implant transvenous pacing leads because of the presence of double SVC. In patients with polysplenia syndrome, anatomical information of venous drainage, especially in SVC, is essential for transvenous pacemaker implantation. The authors state that they have no Conflict of Interest (COI).
Discussion Polysplenia syndrome is a type of situs ambiguous characterized by left isomerism (bilateral left sidedness) (1). This syndrome is commonly known to be associated with various cardiac anomalies, abnormal venous drainages, and abdominal heterotaxia, etc. The majority of patients with polysplenia syndrome have been reported to die by the age of 5 years due to severe cardiac anomalies, but less than 10% of patients have normal hearts or only minor cardiac defects and reach adulthood without symptoms (1). In such cases, polysplenia syndrome is often discovered incidentally during adulthood when the patient undergoes abdominal CT, such as in our patient. Our patient had an almost normal heart anatomy except for persistent left superior vena cava (PLSVC) and dextrocardia. Sinus node dysfunction is a common arrhythmia in patients with polysplenia syndrome because of bilateral left sidedness (left isomerism) (2, 3). The development of the sinus node tissue is strongly related
References 1. Gayer G, Apter S, Jonas T, et al. Polysplenia syndrome detected in adulthood: report of eight cases and review of the literature. Abdom Imaging 24: 178-184, 1999. 2. Wu M-H, Wang J-K, Lin J-L, et al. Cardiac rhythm disturbances in patients with left atrial isomerism. PACE 24: 1631-1638, 2001. 3. Ferrero P, Massa R, Amellone C, Trevi G. Sinus node dysfunction associated with left atrial isomerism. J Cardiovasc Med 9: 953956, 2008. 4. Ho SY, Seo J-W, Brown NA, et al. Morphology of the sinus node in human and mouse hearts with isomerism of the atrial appendages. Br Heart J 74: 437-442, 1995. 5. Ammirabile G, Tessari A, Pignataro V, et al. Pitx2 confers left morphological, molecular, and functional identity to the sinus venosus myocardium. Cardiovasc Res 93: 291-301, 2012. 6. Frogoudaki A, Sutton R, Gatzoulis MA. Pacing for adult patients with left atrial isomerism: efficacy and technical considerations. Europace 5: 189-193, 2003.
Ⓒ 2015 The Japanese Society of Internal Medicine http://www.naika.or.jp/imonline/index.html
1074
