© 2015, Wiley Periodicals, Inc. DOI: 10.1111/echo.12923
Echocardiography
A Case Report of Double-Chambered Right Ventricle Associated with Subaterial Ventricular Septal Defect and Rupture of Right Coronary Sinus Aneurysm Guobing Hu, M.D., Xiangming Zhu, M.D., Guojie Li, M.D., Feng Jiang, M.D., and Jing Li, M.D. Department of Ultrasound, the First Affiliated Hospital of Wannan Medical College, Wuhu, Anhui China
Double-chambered right ventricle (DCRV) is a rare congenital heart disease in which the right ventricle (RV) is divided into two chambers by anomalous muscle bundles. Here, we report a case of DCRV associated with subarterial ventricular septal defect (VSD) and rupture of right coronary sinus aneurysm (RCSA); the patient was diagnosed by echocardiography and later confirmed by surgical operation. (Echocardiography 2015;32:1438–1440) Key words: double-chambered right ventricle, ventricular septal defect, rupture, coronary sinus aneurysm, echocardiography Double-chambered right ventricle is a congenital deformity of the right ventricular outflow tract obstruction caused by anomalous muscle bundles that divide the right ventricle into a proximal high-pressure chamber and a distal low-pressure chamber.1 Most cases of DCRV are usually diagnosed and repaired during childhood; some patients are asymptomatic and are referred for evaluation of a cardiac murmur, while other patients can present with syncope, cyanosis, palpitation, and exertional dyspnea.2 It is very rare that some cases remain asymptomatic until adulthood; these patients always present with unusual symptoms. Chest pain, endocarditis, syncope, and decreased exercise tolerance are the common presentation.3,4 In this report, we describe the case of a 41-year-old female who presented with DCRV associated with subaterial ventricular septal defect and rupture of right coronary sinus aneurysm. Case Report: A 41-year-old female patient presented with chest pain, dyspnea, and intermittent fever for 3 months, and the patient was diagnosed as VSD and right coronary sinus aneurysm by echocardiography in local hospital, then she was referred to our hospital, on physical examination, the patient’s temperature was 36.5°C, heart rate 84/ min, blood pressure 108/63 mmHg, auscultation Address for correspondence and reprint requests: Guobing Hu, Department of Ultrasound, the First Affiliated Hospital of Wannan Medical College, Wuhu 241001, Anhui, China. Fax: +00865535738279; E-mail: [email protected]
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revealed a harsh 5/6 pansystolic ejection murmur and 4/6 pandiastolic murmur along the left sternal border, loudest at the third and fourth left intercostal space. Electrocardiography showed left ventricular and right ventricular hypertrophy, left and right atrial enlargement. Transthoracic echocardiography (TTE) revealed left and right atrium, left and right ventricle enlargement, a VSD with a 90 mmHg pressure gradient was observed 16 mm in diameter (Fig. 1), and RCSA prolapsed into the RV during systole and diastole. Twodimensional transthoracic echocardiography detected a rupture of the RCSA 6 mm in diameter on the parasternal long-axis view, and color Doppler showed moderate shunt from aorta to right ventricle and mild aortic valve regurgitation (Fig. 2). TTE also displayed hypertrophied muscle bundles protruding from the interventricular septum to the right ventricular free wall and dividing the right ventricle into two chambers (Fig. 3). Continuous-wave Doppler revealed a pressure gradient of 101 mmHg between the proximal high-pressure chamber and the distal low-pressure chamber. Mild tricuspid regurgitation was detected by color Doppler, and a pressure gradient of 74 mmHg could been displayed using continuous-wave Doppler. The patient was referred to department of cardiac surgery for surgical correction. The surgery performed with the patient included VSD closure, resection of the anomalous muscle bundle, and repair of aortic sinus aneurysm. The postoperative course was uneventful, echocardiography showed successful resection of the muscle bundle, and hemodynamic parameters were normal, without residual VSD shunt.
DCRV Associated with VSD and Rupture of RCSA
Figure 1. Left ventricle long-axis view displayed subarterial VSD A. continuous-wave Doppler showed a 90 mmHg pressure gradient B. Ao = aorta; LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.
Figure 2. Left ventricle long-axis view displayed rupture of right coronary sinus aneurysm (RCSA) and mild aortic valve regurgitation.
Discussion: DCRV is always associated with cardiac anomalies, including atrial septal defect, tetralogy of Fallot, complete or corrected transposition of the great arteries, Ebstein anomaly, pulmonary valve stenosis, anomalous pulmonary venous drainage5 and perimembranous VSD is the most common defect6 but in this patient, the associated defect was a very large subarterial VSD which is always seen in Asians7; this type of VSD was located under the aortic valve, connected to the low-pressure chamber of RV, so the transseptal pressure gradient across VSD was relatively great. Rupture of RCSA is often associated with VSD; therefore, it is also required to be identified from prolapse of right coronary valve (PRCV) associated with VSD. Great infundibular VSD is often associated with prolapse of PRCV, severe PRCV can enter the right ventricular outflow tract (RVOT) and cause obstruction, auscultation can reveal both diastolic and systolic murmur, two-
dimensional echocardiography can show tumorlike structure protruding into the RVOT, Doppler ultrasound can display systolic abnormal blood flow from the VSD and severe diastolic aortic valve regurgitation, but the tumor-like protrusion comes from the right coronary valve, and it is located below the aortic annulus. In our case, the prolapsed structure came from RCSA, and it is located up the aortic annulus. It can be always difficult to obtain the right ventricular outflow tract image on TTE in adults because of the RV’s irregular shape, retrosternal location.5 So, our patient who was missed diagnosis of DCRV in local hospital might be related to the following factors: Firstly, lacking of understanding of the disease, the ultrasonic doctor’s experience is limited, only observed the large VSD and ignored the observation of the anomalous muscle bundles in the right ventricle. Secondly, because of not skilled, the ultrasonic doctor failed to scan the anomalous muscle bundles in the right ventricle, or took the abnormal muscle bundles as normal bundles. Thirdly, not appropriate gain adjustment of the ultrasound machine, poor imaging of two-dimensional TTE, and anamorphose of color flow signal can cause misdiagnosis.8 Transesophageal echocardiography (TEE) provides better visualization of the RV than TTE does.9 Other noninvasive imaging methods include contrast computed tomography (CT) and magnetic resonance imaging (MRI), especially MRI has excellent spatial resolution and can provide three-dimensional visualization of the RV outflow tract.10 Our case was only diagnosed only by TTE and later was confirmed by surgical operation which included resection of the obstructive muscle bundle, repairment of aortic sinus aneurysm replacement, and VSD closure. The patient survived with surgical treatment and without complications during the follow-up period. 1439
Hu, et al.
Figure 3. C. Parasternal four chamber view and RVOT view displayed abnormal muscle bundles dividing RV into two chambers. Arrow showed blood flow during A. systole and D. diastole. B. Continuous-wave Doppler showed a 101 mmHg pressure gradient. RVOT = right ventricular outlet trace.
To reduce misdiagnosis of complex congenital heart diseases and provide accurate preoperative diagnosis for clinical heart surgical operation, ultrasonic doctors should have a profound hemodynamic knowledge and perform multisection, multi-angle observation. References 1. Alva C, Ho SY, Lincoln CR, et al: The nature of the obstructive muscular bundles in double-chambered right ventricle. J Thorac Cardiovasc Surg 1999;117:1180–1189. 2. Cil E, Saraclar M, Ozkutlu S, et al: Double-chambered right ventricle: experience with 52 cases. Int J Cardiol 1995;50:19–29. 3. Hoffman P, Wojcik AW, Rozanski J, et al: The role of echocardiography in diagnosing double chambered right ventricle in adults. Heart 2004;90:789–793. 4. Hachiro Y, Takagi N, Koyanagi T, et al: Repair of doublechambered right ventricle: surgical results and long-term follow-up. Ann Thorac Surg 2001;72:1520–1522.
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5. Choi YJ, Park SW: Characteristics of double-chambered right ventricle in adult patients. Korean J Intern Med 2010;25:147–153. 6. Oliver JM, Garrido A, Gonzalez A, et al: Rapid progression of midventricular obstruction in adults with doublechambered right ventricle. J Thorac Cardiovasc Surg 2003;126:711–717. 7. Cheung YF, Chiu CS, Yung TC, et al: Impact of preoperative aortic cusp prolapse on long-term outcome after surgical closure of subarterial ventricular septal defect. Ann Thorac Surg 2002;73:622–627. 8. Iii COS, Wienecke MM, Fyfe DA: Color flow Doppler in the diagnosis of double-chambered right ventricle. Echocardiography 2007;11:173–178. 9. Chang RY, Kou CH, Rim RS, et al: Transesophageal echocardiographic image of double-chambered right ventricle. J Am Soc Echocardiogr 1996;9:347–352. 10. Sato Y, Matsumoto N, Matsuo S, et al: Double-chambered right ventricle: depiction at three-dimensional whole heart magnetic resonance imaging. Int J Cardiol 2007;119:e14–e16.
Echocardiography
A Case Report of Double-Chambered Right Ventricle Associated with Subaterial Ventricular Septal Defect and Rupture of Right Coronary Sinus Aneurysm Guobing Hu, M.D., Xiangming Zhu, M.D., Guojie Li, M.D., Feng Jiang, M.D., and Jing Li, M.D. Department of Ultrasound, the First Affiliated Hospital of Wannan Medical College, Wuhu, Anhui China
Double-chambered right ventricle (DCRV) is a rare congenital heart disease in which the right ventricle (RV) is divided into two chambers by anomalous muscle bundles. Here, we report a case of DCRV associated with subarterial ventricular septal defect (VSD) and rupture of right coronary sinus aneurysm (RCSA); the patient was diagnosed by echocardiography and later confirmed by surgical operation. (Echocardiography 2015;32:1438–1440) Key words: double-chambered right ventricle, ventricular septal defect, rupture, coronary sinus aneurysm, echocardiography Double-chambered right ventricle is a congenital deformity of the right ventricular outflow tract obstruction caused by anomalous muscle bundles that divide the right ventricle into a proximal high-pressure chamber and a distal low-pressure chamber.1 Most cases of DCRV are usually diagnosed and repaired during childhood; some patients are asymptomatic and are referred for evaluation of a cardiac murmur, while other patients can present with syncope, cyanosis, palpitation, and exertional dyspnea.2 It is very rare that some cases remain asymptomatic until adulthood; these patients always present with unusual symptoms. Chest pain, endocarditis, syncope, and decreased exercise tolerance are the common presentation.3,4 In this report, we describe the case of a 41-year-old female who presented with DCRV associated with subaterial ventricular septal defect and rupture of right coronary sinus aneurysm. Case Report: A 41-year-old female patient presented with chest pain, dyspnea, and intermittent fever for 3 months, and the patient was diagnosed as VSD and right coronary sinus aneurysm by echocardiography in local hospital, then she was referred to our hospital, on physical examination, the patient’s temperature was 36.5°C, heart rate 84/ min, blood pressure 108/63 mmHg, auscultation Address for correspondence and reprint requests: Guobing Hu, Department of Ultrasound, the First Affiliated Hospital of Wannan Medical College, Wuhu 241001, Anhui, China. Fax: +00865535738279; E-mail: [email protected]
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revealed a harsh 5/6 pansystolic ejection murmur and 4/6 pandiastolic murmur along the left sternal border, loudest at the third and fourth left intercostal space. Electrocardiography showed left ventricular and right ventricular hypertrophy, left and right atrial enlargement. Transthoracic echocardiography (TTE) revealed left and right atrium, left and right ventricle enlargement, a VSD with a 90 mmHg pressure gradient was observed 16 mm in diameter (Fig. 1), and RCSA prolapsed into the RV during systole and diastole. Twodimensional transthoracic echocardiography detected a rupture of the RCSA 6 mm in diameter on the parasternal long-axis view, and color Doppler showed moderate shunt from aorta to right ventricle and mild aortic valve regurgitation (Fig. 2). TTE also displayed hypertrophied muscle bundles protruding from the interventricular septum to the right ventricular free wall and dividing the right ventricle into two chambers (Fig. 3). Continuous-wave Doppler revealed a pressure gradient of 101 mmHg between the proximal high-pressure chamber and the distal low-pressure chamber. Mild tricuspid regurgitation was detected by color Doppler, and a pressure gradient of 74 mmHg could been displayed using continuous-wave Doppler. The patient was referred to department of cardiac surgery for surgical correction. The surgery performed with the patient included VSD closure, resection of the anomalous muscle bundle, and repair of aortic sinus aneurysm. The postoperative course was uneventful, echocardiography showed successful resection of the muscle bundle, and hemodynamic parameters were normal, without residual VSD shunt.
DCRV Associated with VSD and Rupture of RCSA
Figure 1. Left ventricle long-axis view displayed subarterial VSD A. continuous-wave Doppler showed a 90 mmHg pressure gradient B. Ao = aorta; LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.
Figure 2. Left ventricle long-axis view displayed rupture of right coronary sinus aneurysm (RCSA) and mild aortic valve regurgitation.
Discussion: DCRV is always associated with cardiac anomalies, including atrial septal defect, tetralogy of Fallot, complete or corrected transposition of the great arteries, Ebstein anomaly, pulmonary valve stenosis, anomalous pulmonary venous drainage5 and perimembranous VSD is the most common defect6 but in this patient, the associated defect was a very large subarterial VSD which is always seen in Asians7; this type of VSD was located under the aortic valve, connected to the low-pressure chamber of RV, so the transseptal pressure gradient across VSD was relatively great. Rupture of RCSA is often associated with VSD; therefore, it is also required to be identified from prolapse of right coronary valve (PRCV) associated with VSD. Great infundibular VSD is often associated with prolapse of PRCV, severe PRCV can enter the right ventricular outflow tract (RVOT) and cause obstruction, auscultation can reveal both diastolic and systolic murmur, two-
dimensional echocardiography can show tumorlike structure protruding into the RVOT, Doppler ultrasound can display systolic abnormal blood flow from the VSD and severe diastolic aortic valve regurgitation, but the tumor-like protrusion comes from the right coronary valve, and it is located below the aortic annulus. In our case, the prolapsed structure came from RCSA, and it is located up the aortic annulus. It can be always difficult to obtain the right ventricular outflow tract image on TTE in adults because of the RV’s irregular shape, retrosternal location.5 So, our patient who was missed diagnosis of DCRV in local hospital might be related to the following factors: Firstly, lacking of understanding of the disease, the ultrasonic doctor’s experience is limited, only observed the large VSD and ignored the observation of the anomalous muscle bundles in the right ventricle. Secondly, because of not skilled, the ultrasonic doctor failed to scan the anomalous muscle bundles in the right ventricle, or took the abnormal muscle bundles as normal bundles. Thirdly, not appropriate gain adjustment of the ultrasound machine, poor imaging of two-dimensional TTE, and anamorphose of color flow signal can cause misdiagnosis.8 Transesophageal echocardiography (TEE) provides better visualization of the RV than TTE does.9 Other noninvasive imaging methods include contrast computed tomography (CT) and magnetic resonance imaging (MRI), especially MRI has excellent spatial resolution and can provide three-dimensional visualization of the RV outflow tract.10 Our case was only diagnosed only by TTE and later was confirmed by surgical operation which included resection of the obstructive muscle bundle, repairment of aortic sinus aneurysm replacement, and VSD closure. The patient survived with surgical treatment and without complications during the follow-up period. 1439
Hu, et al.
Figure 3. C. Parasternal four chamber view and RVOT view displayed abnormal muscle bundles dividing RV into two chambers. Arrow showed blood flow during A. systole and D. diastole. B. Continuous-wave Doppler showed a 101 mmHg pressure gradient. RVOT = right ventricular outlet trace.
To reduce misdiagnosis of complex congenital heart diseases and provide accurate preoperative diagnosis for clinical heart surgical operation, ultrasonic doctors should have a profound hemodynamic knowledge and perform multisection, multi-angle observation. References 1. Alva C, Ho SY, Lincoln CR, et al: The nature of the obstructive muscular bundles in double-chambered right ventricle. J Thorac Cardiovasc Surg 1999;117:1180–1189. 2. Cil E, Saraclar M, Ozkutlu S, et al: Double-chambered right ventricle: experience with 52 cases. Int J Cardiol 1995;50:19–29. 3. Hoffman P, Wojcik AW, Rozanski J, et al: The role of echocardiography in diagnosing double chambered right ventricle in adults. Heart 2004;90:789–793. 4. Hachiro Y, Takagi N, Koyanagi T, et al: Repair of doublechambered right ventricle: surgical results and long-term follow-up. Ann Thorac Surg 2001;72:1520–1522.
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5. Choi YJ, Park SW: Characteristics of double-chambered right ventricle in adult patients. Korean J Intern Med 2010;25:147–153. 6. Oliver JM, Garrido A, Gonzalez A, et al: Rapid progression of midventricular obstruction in adults with doublechambered right ventricle. J Thorac Cardiovasc Surg 2003;126:711–717. 7. Cheung YF, Chiu CS, Yung TC, et al: Impact of preoperative aortic cusp prolapse on long-term outcome after surgical closure of subarterial ventricular septal defect. Ann Thorac Surg 2002;73:622–627. 8. Iii COS, Wienecke MM, Fyfe DA: Color flow Doppler in the diagnosis of double-chambered right ventricle. Echocardiography 2007;11:173–178. 9. Chang RY, Kou CH, Rim RS, et al: Transesophageal echocardiographic image of double-chambered right ventricle. J Am Soc Echocardiogr 1996;9:347–352. 10. Sato Y, Matsumoto N, Matsuo S, et al: Double-chambered right ventricle: depiction at three-dimensional whole heart magnetic resonance imaging. Int J Cardiol 2007;119:e14–e16.
