e-Herz: Case study Herz 2014 DOI 10.1007/s00059-014-4076-3 Received: 17 November 2013 Accepted: 4 February 2014 © Urban & Vogel 2014
e-Herz
C. Kilit · M. Oylumlu · A. Doğan · B. Amasyalı Department of Cardiology, Faculty of Medicine, Dumlupınar University, Kütahya
Cor triatriatum dexter in a patient with pectus excavatum A rare cause of right heart failure
Cor triatriatum dexter (CTD) is an extremely rare congenital anomaly in which the right atrium is divided into two chambers by a membrane caused by persistence of the right valve of the sinus venosus [1]. The persistence of the right leaflet of the venous sinus valve causes a wide range of defects including a prominent Eustachian valve, the Chiari network, and CTD [2]. It has an estimated incidence of around 0.025% of all congenital heart diseases and is frequently associated with rightsided defects caused by abnormal fetal circulation. Among these, the most frequent are stenosis or atresia of the pulmonary valve, tricuspid valve abnormalities, and atrial septal defects [3]. A patent fora-
men ovale (PFO) has been shown previously in neonatal and adult patients with CTD [4, 5]. However, CTD and PFO together in a patient with pectus excavatum (PE), which is the most common chest wall deformity affecting 1–300/1,000 individuals, has not been reported in the literature [6].
Case report A 58-year-old man was admitted to the cardiology clinic complaining of dyspnea and palpitations and presented with bilateral ankle and leg edema and jugular vein distension. The patient had a history of chronic obstructive pulmonary disease
Fig. 1 8 Photograph of the patient. Sternal depression is seen in the middle of the chest
and also had an anterior chest wall deformity of pectus excavatum (PE) (. Fig. 1). The cardiac rhythm was atrial fibrillation with rapid ventricular response. An initial transthoracic echocardiogram showed a dilated right atrium with a membrane dividing the right atrium into two chambers. The left heart was normal (. Fig. 2). Transesophageal echocardiography showed a membrane arising from the ostium of the inferior vena cava that was attached to the interatrial septum just below the level of the fossa ovalis (. Fig. 3). The valve measured 0.8 cm in its greatest thickness and partitioned the right atrium into two chambers. These findings were consistent with CTD. Col-
Fig. 2 8 Transthoracic apical four-chamber view demonstrating the right atrium divided into two chambers by a membrane. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle Herz 2014
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e-Herz: Case study
Fig. 3 8 Transesophageal echocardiogram demonstrating a membrane in the right atrium. RA right atrium
Fig. 4 8 Transesophageal color Doppler echocardiogram demonstrating a patent foramen ovale and shunt from the left to right atrium. IAS interatrial septum, PFO patent foramen ovale, RA right atrium
Fig. 5 9 Transesophageal color Doppler echocardiogram showing a foramen in the membrane allowing blood flow between two chambers. LA left atrium, RA right atrium
or Doppler echocardiography (. Fig. 4) showed a PFO with a continuous blood flow from the left to the right atrium and a foramen in the membrane attaining a diameter of 1.5 cm at the widest point (. Fig. 5). After diagnosis the membrane appeared to have a hemodynamic significance and required treatment. Because of severe chronic obstructive pulmonary disease and chest wall deformity, surgical treatment was assessed to carry a high mortality risk so it was decided to attempt medicinal treatment. Bronchodilators, parenteral diuretics, and anticoagulation were administered and after the treatment the patient’s complaints significantly improved. The patient was discharged from hospital with diuretic and anticoagulation treatment.
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Herz 2014
Discussion A CTD is an extremely rare congenital anomaly and in most cases the anomaly is detected at autopsy, either as an isolated finding in an otherwise normal heart or accompanying other congenital heart lesions [7, 8]. The most common anterior chest deformity of PE is characterized by sternal depression with corresponding left-sided displacement and rotation of the heart [9]. It is usually seen as an isolated congenital abnormality; however, it is occasionally associated with cardiac abnormalities, such as atrial and ventricular septal defects and tetralogy of Fallot [10]. In a study examining the association between cardiac and sternal anomalies in a series of 2,000 cases of congenital heart
disease, the incidence of PE was found to be 2.5% [11]. Neither CTD nor cor triatriatum sinister have been previously reported in a patient with PE. A CTD has varying clinical manifestations depending on the degree of partitioning or septation of the right atrium [2]. When the septation is mild, the condition is often asymptomatic and is an incidental finding frequently made at postmortem examination; however, more severe septation can cause right-sided heart failure and elevated central venous pressure due to obstruction of the tricuspid valve, the right ventricular outflow tract, and the inferior vena cava. Furthermore, it can lead to trapped catheters, supraventricular arrhythmia, and embolisms. A CTD can be diagnosed by echocardiography and magnetic resonance imaging, although caution is needed since a prominent Eustachian valve can simulate this defect [3, 12]. Either of these imaging techniques can show the right atrium divided into two chambers, a posterior chamber into which the vena cava drains and an anterior one that contains the atrial appendage. Asymptomatic patients should not be treated unless referred for heart surgery for other reasons. In symptomatic patients with significant obstruction, the treatment of choice is surgical resection although percutaneous correction of the membrane has been proposed as an alternative to surgical treatment [13]. Moreover, CTD was
treated successfully in animals by using cutting balloon catheterization and balloon dilation methods [14, 15].
Corresponding address C. Kilit Department of Cardiology, Faculty of Medicine, Dumlupınar University 100. Yıl Mahallesi, Dumlupınar Bulvarı, Beylikkent Sitesi, No: 48/D, Daire 6, 43100 Kütahya Turkey [email protected]
Compliance with ethical guidelines
11. Sánchez Cascos A (1989) Association of cardiac and sternal malformations. An Esp Pediatr 30:272–274 12. Lee YS, Kim KS, Lee JB et al (2007) Cor triatriatum dexter assessed by three-dimensional echocardiography reconstruction in two adult patients. Echocardiography 24:991–994 13. Savas V, Samyn J, Schreiber TL et al (1991) Cor triatriatum dexter: recognition and percutaneous transluminal correction. Cathet Cardiovasc Diagn 23:183–186 14. Leblanc N, Defrancesco TC, Adams AK et al (2012) Cutting balloon catheterization for interventional treatment of cor triatriatum dexter: 2 cases. J Vet Cardiol 14:525–530 15. López-Alvarez J, Dukes-McEwan J, Martin MW et al (2011) Balloon dilation of an imperforate cor triatriatum dexter in a Golden Retriever with concurrent double-chambered right ventricle and subsequent evaluation by cardiac magnetic resonance imaging. J Vet Cardiol 13:211–218
Conflict of interest. C. Kilit, M. Oylumlu, A. Doğan, and B. Amasyalı state that there are no conflicts of interest. The accompanying manuscript does not include studies on humans or animals.
References 1. Hansing CE, Young WP, Rowe GG (1972) Cor triatriatum dexter. Persistent right sinus venosus valve. Am J Cardiol 30:559–564 2. García López JC, Sánchez Pérez I, Cazzaniga M et al (2005) Unusual cyanosing heart defect due to supravalvular tricuspid obstruction in an infant. Rev Esp Cardiol 58:1470–1472 3. Eroglu ST, Yildirir A, Simsek V et al (2004) Cor triatriatum dexter, atrial septal defect, and Ebstein’s anomaly in an adult given a diagnosis by transthoracic and transesophageal echocardiography: a case report. J Am Soc Echocardiogr 17:780–782 4. Kessel-Schaefer A, Linka A, Pretre R, Buser P (2006) Inferior sinus venosus defect associated with incomplete cor triatriatum dexter and patent foramen ovale. Eur J Echocardiogr 7:239–242 5. Hoye DJ, Wilson EC, Fyfe DA, Guzzetta NA (2010) Cor triatriatum dexter: a rare cause of neonatal cyanosis. Anesth Analg 110:716–718 6. Schaerer D, Virbalas J, Willis E et al (2013) Pectus excavatum in children with laryngomalacia. Int J Pediatr Otorhinolaryngol 77:1721–1723 7. Muñoz Castellanos L, Kari Nivon M, García Arenal F, Salinas CH (1991) The pathological remnants of the right sinus venosus valve. A presentation of 6 hearts of the cor tritriatum dexter type. Arch Inst Cardiol Mex 61:293–301 8. Doucette J, Knoblich R (1963) Persistent right valve of the sinus venosus. So-called cor triatriatum dextrum: review of the literature and report of a case. Arch Pathol 75:105–112 9. Fokin AA, Steuerwald NM, Ahrens WA, Allen KE (2009) Anatomical, histologic, and genetic characteristics of congenital chest wall deformities. Semin Thorac Cardiovasc Surg 21:44–57 10. DeLeon MM, Magliato KE, Roughneen PT et al (1997) Simultaneous repair of pectus excavatum and congenital heart disease. Ann Thorac Surg 64:557–559
Herz 2014
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e-Herz
C. Kilit · M. Oylumlu · A. Doğan · B. Amasyalı Department of Cardiology, Faculty of Medicine, Dumlupınar University, Kütahya
Cor triatriatum dexter in a patient with pectus excavatum A rare cause of right heart failure
Cor triatriatum dexter (CTD) is an extremely rare congenital anomaly in which the right atrium is divided into two chambers by a membrane caused by persistence of the right valve of the sinus venosus [1]. The persistence of the right leaflet of the venous sinus valve causes a wide range of defects including a prominent Eustachian valve, the Chiari network, and CTD [2]. It has an estimated incidence of around 0.025% of all congenital heart diseases and is frequently associated with rightsided defects caused by abnormal fetal circulation. Among these, the most frequent are stenosis or atresia of the pulmonary valve, tricuspid valve abnormalities, and atrial septal defects [3]. A patent fora-
men ovale (PFO) has been shown previously in neonatal and adult patients with CTD [4, 5]. However, CTD and PFO together in a patient with pectus excavatum (PE), which is the most common chest wall deformity affecting 1–300/1,000 individuals, has not been reported in the literature [6].
Case report A 58-year-old man was admitted to the cardiology clinic complaining of dyspnea and palpitations and presented with bilateral ankle and leg edema and jugular vein distension. The patient had a history of chronic obstructive pulmonary disease
Fig. 1 8 Photograph of the patient. Sternal depression is seen in the middle of the chest
and also had an anterior chest wall deformity of pectus excavatum (PE) (. Fig. 1). The cardiac rhythm was atrial fibrillation with rapid ventricular response. An initial transthoracic echocardiogram showed a dilated right atrium with a membrane dividing the right atrium into two chambers. The left heart was normal (. Fig. 2). Transesophageal echocardiography showed a membrane arising from the ostium of the inferior vena cava that was attached to the interatrial septum just below the level of the fossa ovalis (. Fig. 3). The valve measured 0.8 cm in its greatest thickness and partitioned the right atrium into two chambers. These findings were consistent with CTD. Col-
Fig. 2 8 Transthoracic apical four-chamber view demonstrating the right atrium divided into two chambers by a membrane. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle Herz 2014
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e-Herz: Case study
Fig. 3 8 Transesophageal echocardiogram demonstrating a membrane in the right atrium. RA right atrium
Fig. 4 8 Transesophageal color Doppler echocardiogram demonstrating a patent foramen ovale and shunt from the left to right atrium. IAS interatrial septum, PFO patent foramen ovale, RA right atrium
Fig. 5 9 Transesophageal color Doppler echocardiogram showing a foramen in the membrane allowing blood flow between two chambers. LA left atrium, RA right atrium
or Doppler echocardiography (. Fig. 4) showed a PFO with a continuous blood flow from the left to the right atrium and a foramen in the membrane attaining a diameter of 1.5 cm at the widest point (. Fig. 5). After diagnosis the membrane appeared to have a hemodynamic significance and required treatment. Because of severe chronic obstructive pulmonary disease and chest wall deformity, surgical treatment was assessed to carry a high mortality risk so it was decided to attempt medicinal treatment. Bronchodilators, parenteral diuretics, and anticoagulation were administered and after the treatment the patient’s complaints significantly improved. The patient was discharged from hospital with diuretic and anticoagulation treatment.
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Herz 2014
Discussion A CTD is an extremely rare congenital anomaly and in most cases the anomaly is detected at autopsy, either as an isolated finding in an otherwise normal heart or accompanying other congenital heart lesions [7, 8]. The most common anterior chest deformity of PE is characterized by sternal depression with corresponding left-sided displacement and rotation of the heart [9]. It is usually seen as an isolated congenital abnormality; however, it is occasionally associated with cardiac abnormalities, such as atrial and ventricular septal defects and tetralogy of Fallot [10]. In a study examining the association between cardiac and sternal anomalies in a series of 2,000 cases of congenital heart
disease, the incidence of PE was found to be 2.5% [11]. Neither CTD nor cor triatriatum sinister have been previously reported in a patient with PE. A CTD has varying clinical manifestations depending on the degree of partitioning or septation of the right atrium [2]. When the septation is mild, the condition is often asymptomatic and is an incidental finding frequently made at postmortem examination; however, more severe septation can cause right-sided heart failure and elevated central venous pressure due to obstruction of the tricuspid valve, the right ventricular outflow tract, and the inferior vena cava. Furthermore, it can lead to trapped catheters, supraventricular arrhythmia, and embolisms. A CTD can be diagnosed by echocardiography and magnetic resonance imaging, although caution is needed since a prominent Eustachian valve can simulate this defect [3, 12]. Either of these imaging techniques can show the right atrium divided into two chambers, a posterior chamber into which the vena cava drains and an anterior one that contains the atrial appendage. Asymptomatic patients should not be treated unless referred for heart surgery for other reasons. In symptomatic patients with significant obstruction, the treatment of choice is surgical resection although percutaneous correction of the membrane has been proposed as an alternative to surgical treatment [13]. Moreover, CTD was
treated successfully in animals by using cutting balloon catheterization and balloon dilation methods [14, 15].
Corresponding address C. Kilit Department of Cardiology, Faculty of Medicine, Dumlupınar University 100. Yıl Mahallesi, Dumlupınar Bulvarı, Beylikkent Sitesi, No: 48/D, Daire 6, 43100 Kütahya Turkey [email protected]
Compliance with ethical guidelines
11. Sánchez Cascos A (1989) Association of cardiac and sternal malformations. An Esp Pediatr 30:272–274 12. Lee YS, Kim KS, Lee JB et al (2007) Cor triatriatum dexter assessed by three-dimensional echocardiography reconstruction in two adult patients. Echocardiography 24:991–994 13. Savas V, Samyn J, Schreiber TL et al (1991) Cor triatriatum dexter: recognition and percutaneous transluminal correction. Cathet Cardiovasc Diagn 23:183–186 14. Leblanc N, Defrancesco TC, Adams AK et al (2012) Cutting balloon catheterization for interventional treatment of cor triatriatum dexter: 2 cases. J Vet Cardiol 14:525–530 15. López-Alvarez J, Dukes-McEwan J, Martin MW et al (2011) Balloon dilation of an imperforate cor triatriatum dexter in a Golden Retriever with concurrent double-chambered right ventricle and subsequent evaluation by cardiac magnetic resonance imaging. J Vet Cardiol 13:211–218
Conflict of interest. C. Kilit, M. Oylumlu, A. Doğan, and B. Amasyalı state that there are no conflicts of interest. The accompanying manuscript does not include studies on humans or animals.
References 1. Hansing CE, Young WP, Rowe GG (1972) Cor triatriatum dexter. Persistent right sinus venosus valve. Am J Cardiol 30:559–564 2. García López JC, Sánchez Pérez I, Cazzaniga M et al (2005) Unusual cyanosing heart defect due to supravalvular tricuspid obstruction in an infant. Rev Esp Cardiol 58:1470–1472 3. Eroglu ST, Yildirir A, Simsek V et al (2004) Cor triatriatum dexter, atrial septal defect, and Ebstein’s anomaly in an adult given a diagnosis by transthoracic and transesophageal echocardiography: a case report. J Am Soc Echocardiogr 17:780–782 4. Kessel-Schaefer A, Linka A, Pretre R, Buser P (2006) Inferior sinus venosus defect associated with incomplete cor triatriatum dexter and patent foramen ovale. Eur J Echocardiogr 7:239–242 5. Hoye DJ, Wilson EC, Fyfe DA, Guzzetta NA (2010) Cor triatriatum dexter: a rare cause of neonatal cyanosis. Anesth Analg 110:716–718 6. Schaerer D, Virbalas J, Willis E et al (2013) Pectus excavatum in children with laryngomalacia. Int J Pediatr Otorhinolaryngol 77:1721–1723 7. Muñoz Castellanos L, Kari Nivon M, García Arenal F, Salinas CH (1991) The pathological remnants of the right sinus venosus valve. A presentation of 6 hearts of the cor tritriatum dexter type. Arch Inst Cardiol Mex 61:293–301 8. Doucette J, Knoblich R (1963) Persistent right valve of the sinus venosus. So-called cor triatriatum dextrum: review of the literature and report of a case. Arch Pathol 75:105–112 9. Fokin AA, Steuerwald NM, Ahrens WA, Allen KE (2009) Anatomical, histologic, and genetic characteristics of congenital chest wall deformities. Semin Thorac Cardiovasc Surg 21:44–57 10. DeLeon MM, Magliato KE, Roughneen PT et al (1997) Simultaneous repair of pectus excavatum and congenital heart disease. Ann Thorac Surg 64:557–559
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