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Reconstruction of the Right Heart in an Elderly Woman With Ebstein’s Anomaly and Severe Heart Failure
CASE REPORT NAKAYAMA ET AL RECONSTRUCTION OF THE RIGHT HEART FOR EBSTEIN’S ANOMALY
Reconstruction of the Right Heart in an Elderly Woman With Ebstein’s Anomaly and Severe Heart Failure Takuya Nakayama, MD, Miki Asano, MD, Kozo Matsumoto, MD, Norikazu Nomura, MD, Takayuki Saito, MD, Akihiro Mizuno, MD, and Akira Mishima, MD Department of Cardiovascular Surgery, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
We report the case of a 53-year-old woman with Ebstein’s anomaly and intractable heart failure who had undergone only tricuspid valve replacement 30 years earlier. She was treated conservatively for 1 month; however, she was placed in the New York Heart Association functional class IV. Therefore, we operated on her with the objective of improving her quality of life and cardiac function. One-and-a-half repair and a second tricuspid valve replacement with right ventriculoplasty were quite effective in ameliorating her critical condition. (Ann Thorac Surg 2007;84:1745– 6) © 2007 by The Society of Thoracic Surgeons
E
bstein’s anomaly is a rare congenital heat disorder characterized by abnormal formation of the tricuspid valve and is often accompanied by various other pathophysiological conditions. Surgery for the tricuspid valve and right ventricle is often required, depending on the grade of the anomaly. Therapy needs to be tailored to each patient and the initial repair undertaken directly influences the prognosis. We reconstructed the right heart in a patient of Ebstein’s anomaly with severe heart failure who had undergone only tricuspid valve replacement 30 years earlier, and we obtained excellent results. A 53-year-old woman was referred to our hospital with a history of acute syncope due to ventricular tachycardia. She had a history of tricuspid valve replacement with a ball valve for Ebstein’s anomaly 30 years earlier. She had been initiated on warfarin potassium at a daily dose of 4 mg as postoperative maintenance anticoagulant therapy and been on bradypacing therapy in the VVI pacing mode for sick sinus syndrome during the previous 5 years. Although the cardiac arrhythmia reversed with defibrillation therapy, her heart failure remained intractable for more than 1 month, even with intensive care. Echocardiography indicated good global function of the prosthetic valve and absence of any pulmonary valve regurgitation. The left ventricular ejection fraction was Accepted for publication June 22, 2007. Address correspondence to Dr Mishima, Department of Cardiovascular Surgery, Nagoya City University Graduate School of Medical Sciences, Kawasumi 1, Mizuho-cho, Mizuho-ku, Nagoya, 467-8601, Japan; e-mail: [email protected].
© 2007 by The Society of Thoracic Surgeons Published by Elsevier Inc
1745
40%, with an enormously dilated right ventricle that was also confirmed by computerized tomography (Fig 1A). We considered that volume reduction and reconstruction of the right ventricle might help in increasing the left ventricular ejection fraction. On opening the pericardium, the mean pulmonary arterial pressure was directly measured at 13 mm Hg. After initiation of standard cardiopulmonary bypass with direct bi-caval cannulation, we noted that the coronary sinus opened into the right atrium and that her mechanical valve was in a normal anatomical position. After detaching the previously implanted valve, we plicated the atrialized right ventricle by using Carpentier’s procedure. In addition, we performed volume reduction of the right atrium by excision of the right atrial appendage and part of the free wall. The tricuspid valve was replaced with a 27-mm CarboMedics valve (CarboMedics Inc, Austin, TX) in the anatomical position. Finally, a bidirectional cavopulmonary shunt was constructed. Cardiopulmonary bypass was successfully discontinued with a moderate dose of inotropic agents. Her postoperative course was uneventful, and she was discharged 30 days after the operation. The postoperative mean pulmonary artery and superior vena cava pressures were 14 and 16 mm Hg, respectively. Computerized tomography and echocardiography showed reduction in the volume of the right ventricle and increase in the volume of the left ventricle (Fig 1B). The left ventricular volume was determined by a radiologist in multi-detector computerized tomographic images [1], and it was found to have increased from 109.7 mL preoperatively to 158.4 mL postoperatively. The left ventricular ejection fraction increased to 75%. The serum brain natriuretic peptide level fell from 750 pg/mL to 250 pg/mL by 1 year after the surgery. Her functional capacity improved to New York Heart Association functional class II.
Comment Ebstein’s anomaly is a congenital malformation characterized by downward displacement of the septal and posterior leaflets into the right ventricle and hypoplasia of the tricuspid valve, resulting in an atrialized right ventricle [2]. The pathophysiology is tricuspid valvular insufficiency and pendular blood flow in the right heart. Patients with Ebstein’s anomaly are relatively more susceptible to heart failure because of the dilated atrialized right ventricle, which often reduces the preload of the left side of the heart and compresses the left ventricle. Moreover, the functional inflow into the right ventricle is very small due to abnormalities of the subtricuspid valve apparatus [3]. Therefore, Ebstein’s anomaly is a complex disease of the tricuspid valve and right ventricle that must be treated according to individual need. Carpentier and colleagues [3] and Danielson and colleagues [4] proposed tricuspid valvuloplasty or valve replacement in the anatomical normal position and plication of the atrialized ventricle as surgical treatment for Ebstein’s anomaly. On the other hand, Hetzer and colleagues [5] reported that tricuspid valve replacement alone may be sufficient, because incorporation of the atrialized chamber 0003-4975/07/$32.00 doi:10.1016/j.athoracsur.2007.06.065
FEATURE ARTICLES
Ann Thorac Surg 2007;84:1745– 6
1746
CASE REPORT IMAMURA ET AL HYPOPLASTIC LEFT HEART SYNDROME WITH HETEROTAXY SYNDROME
Ann Thorac Surg 2007;84:1746 – 8
Fig 1. Transverse computerized tomographic images at the level of the ninth thoracic vertebra showing release of compression and increase in the volume of the left ventricle after surgery. Open circles with dotted lines indicates the left ventricle. The preoperative and postoperative left ventricular volume was (A) 109.7 mL and (B) 158.4 mL, respectively.
FEATURE ARTICLES
into the contracting right ventricle may be beneficial in allowing sufficient right ventricular filling during diastole and could stimulate the remaining musculature in the atrialized chamber wall to hypertrophy and contribute to right ventricular contraction. Good results were reported after a follow-up of 10 to 103 months in that study [5]. However, the observation in our case suggests that in the long term, leaving the atrialized chamber in situ does not necessarily yield a good outcome. To treat the intractable heart failure in our patient, we considered it necessary to release the compression on the left ventricle and to protect the dysfunctional right ventricle from volume overload. In general, the bidirectional Glenn shunt is recognized as a step operation for a staged Fontan procedure, which is known to improve cyanosis and reduce the ventricular volume load in children with a single ventricle. Marianeschi and colleagues [6] suggested that an additional bidirectional Glenn shunt after repair or replacement of the tricuspid valve may be used as a surgical option for Ebstein’s anomaly patients with severe heart failure because of the reduced volume overload of the fragile right ventricle. They reported improvement of the exercise tolerance and right cardiac function after a follow-up of 2 to 24 months in their patients. However, there were no elderly or reoperation cases in their series. The condition of the pulmonary vascular bed determines the feasibility of a right heart bypass operation, including the Glenn shunt. Pulmonary hemodynamics as evaluated by pulmonary artery pressure, pulmonary-tosystemic flow ratio, and pulmonary artery index is essential to determine the feasibility of a right heart bypass operation [7]. In this case, right heart catheterization could not be conducted for investigating the pulmonary hemodynamics because of the earlier tricuspid valve replacement. However, we were certain that the pulmonary circulation could be established by one-and-a-half ventricular repair combined with right ventriculoplasty because of some favorable indices, such as a low mean pulmonary artery pressure of 13 mm Hg, absence of severe dilatation of the intrapulmonary vessels, and absence of pulmonary and prosthetic valve regurgitation. Postoperatively, the mean pressure in the inferior vena cava was 6 mm Hg. Echocardiography showed reduced dilatation of the right heart and improvement of the left ventricular ejection fraction. Finally, her quality of life improved and her serum brain natriuretic peptide level decreased. In conclusion, it is suggested that the Glenn © 2007 by The Society of Thoracic Surgeons Published by Elsevier Inc
shunt combined with repair of the tricuspid valve and the right ventricle may be a valuable surgical option for Ebstein’s anomaly patients with intractable heart failure.
References 1. Juergens KU, Seifarth H, Maintz D, et al. MDCT determination of volume and function of the left ventricle: are short-axis image reformations necessary? Am J Radiol 2006;186(6 Suppl 2):S371– 8. 2. Becker AE, Becker MJ, Edwards JE. Pathologic spectrum of dysplasia of the tricuspid valve. Features in common with Ebstein’s malformation. Arch Pathol 1971;91:167–78. 3. Carpentier A, Chauvaud S, Mac´e L, et al. A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1988;96:92–101. 4. Danielson GK, Driscoll DJ, Mair DD, Warnes CA, Oliver WC Jr. Operative treatment of Ebstein’s anomaly. J Thorac Cardiovasc Surg 1992;104:1195–202. 5. Hetzer R, Nagdyman N, Ewert P, et al. A modified repair technique for tricuspid incompetence in Ebstein’s anomaly. J Thorac Cardiovasc Surg 1998;115:857– 68. 6. Marianeschi SM, McElhinney DB, Reddy VM, Silverman NH, Hanley FL. Alternative approach to the repair of Ebstein’s malformation: intracardiac repair with ventricular unloading. Ann Thorac Surg 1998;66:1546 –50. 7. Chowdhury UK, Airan B, Sharma R, et al. One and a half ventricle repair with pulsatile bidirectional Glenn: results and guidelines for patient selection. Ann Thorac Surg 2001;71: 1995–2002.
Hypoplastic Left Heart Syndrome, Interrupted Inferior Vena Cava, Biliary Atresia Michiaki Imamura, MD, PhD, Umesh Dyamenahalli, MD, Ritu Sachdeva, MD, Evan R. Kokoska, MD, and Robert D. B. Jaquiss, MD Divisions of Pediatric Cardiothoracic Surgery, Pediatric Cardiology, and Pediatric General Surgery, University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, Little Rock, Arkansas
A neonate was diagnosed with hypoplastic left heart syndrome, heterotaxy syndrome, and interrupted inferior Accepted for publication May 29, 2007. Address correspondence to Dr Imamura, Division of Pediatric Cardiothoracic Surgery, Arkansas Children’s Hospital, 800 Marshall St, Slot 677, Little Rock, AR 72202; e-mail: [email protected].
0003-4975/07/$32.00 doi:10.1016/j.athoracsur.2007.05.068
Reconstruction of the Right Heart in an Elderly Woman With Ebstein’s Anomaly and Severe Heart Failure Takuya Nakayama, MD, Miki Asano, MD, Kozo Matsumoto, MD, Norikazu Nomura, MD, Takayuki Saito, MD, Akihiro Mizuno, MD, and Akira Mishima, MD Department of Cardiovascular Surgery, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
We report the case of a 53-year-old woman with Ebstein’s anomaly and intractable heart failure who had undergone only tricuspid valve replacement 30 years earlier. She was treated conservatively for 1 month; however, she was placed in the New York Heart Association functional class IV. Therefore, we operated on her with the objective of improving her quality of life and cardiac function. One-and-a-half repair and a second tricuspid valve replacement with right ventriculoplasty were quite effective in ameliorating her critical condition. (Ann Thorac Surg 2007;84:1745– 6) © 2007 by The Society of Thoracic Surgeons
E
bstein’s anomaly is a rare congenital heat disorder characterized by abnormal formation of the tricuspid valve and is often accompanied by various other pathophysiological conditions. Surgery for the tricuspid valve and right ventricle is often required, depending on the grade of the anomaly. Therapy needs to be tailored to each patient and the initial repair undertaken directly influences the prognosis. We reconstructed the right heart in a patient of Ebstein’s anomaly with severe heart failure who had undergone only tricuspid valve replacement 30 years earlier, and we obtained excellent results. A 53-year-old woman was referred to our hospital with a history of acute syncope due to ventricular tachycardia. She had a history of tricuspid valve replacement with a ball valve for Ebstein’s anomaly 30 years earlier. She had been initiated on warfarin potassium at a daily dose of 4 mg as postoperative maintenance anticoagulant therapy and been on bradypacing therapy in the VVI pacing mode for sick sinus syndrome during the previous 5 years. Although the cardiac arrhythmia reversed with defibrillation therapy, her heart failure remained intractable for more than 1 month, even with intensive care. Echocardiography indicated good global function of the prosthetic valve and absence of any pulmonary valve regurgitation. The left ventricular ejection fraction was Accepted for publication June 22, 2007. Address correspondence to Dr Mishima, Department of Cardiovascular Surgery, Nagoya City University Graduate School of Medical Sciences, Kawasumi 1, Mizuho-cho, Mizuho-ku, Nagoya, 467-8601, Japan; e-mail: [email protected].
© 2007 by The Society of Thoracic Surgeons Published by Elsevier Inc
1745
40%, with an enormously dilated right ventricle that was also confirmed by computerized tomography (Fig 1A). We considered that volume reduction and reconstruction of the right ventricle might help in increasing the left ventricular ejection fraction. On opening the pericardium, the mean pulmonary arterial pressure was directly measured at 13 mm Hg. After initiation of standard cardiopulmonary bypass with direct bi-caval cannulation, we noted that the coronary sinus opened into the right atrium and that her mechanical valve was in a normal anatomical position. After detaching the previously implanted valve, we plicated the atrialized right ventricle by using Carpentier’s procedure. In addition, we performed volume reduction of the right atrium by excision of the right atrial appendage and part of the free wall. The tricuspid valve was replaced with a 27-mm CarboMedics valve (CarboMedics Inc, Austin, TX) in the anatomical position. Finally, a bidirectional cavopulmonary shunt was constructed. Cardiopulmonary bypass was successfully discontinued with a moderate dose of inotropic agents. Her postoperative course was uneventful, and she was discharged 30 days after the operation. The postoperative mean pulmonary artery and superior vena cava pressures were 14 and 16 mm Hg, respectively. Computerized tomography and echocardiography showed reduction in the volume of the right ventricle and increase in the volume of the left ventricle (Fig 1B). The left ventricular volume was determined by a radiologist in multi-detector computerized tomographic images [1], and it was found to have increased from 109.7 mL preoperatively to 158.4 mL postoperatively. The left ventricular ejection fraction increased to 75%. The serum brain natriuretic peptide level fell from 750 pg/mL to 250 pg/mL by 1 year after the surgery. Her functional capacity improved to New York Heart Association functional class II.
Comment Ebstein’s anomaly is a congenital malformation characterized by downward displacement of the septal and posterior leaflets into the right ventricle and hypoplasia of the tricuspid valve, resulting in an atrialized right ventricle [2]. The pathophysiology is tricuspid valvular insufficiency and pendular blood flow in the right heart. Patients with Ebstein’s anomaly are relatively more susceptible to heart failure because of the dilated atrialized right ventricle, which often reduces the preload of the left side of the heart and compresses the left ventricle. Moreover, the functional inflow into the right ventricle is very small due to abnormalities of the subtricuspid valve apparatus [3]. Therefore, Ebstein’s anomaly is a complex disease of the tricuspid valve and right ventricle that must be treated according to individual need. Carpentier and colleagues [3] and Danielson and colleagues [4] proposed tricuspid valvuloplasty or valve replacement in the anatomical normal position and plication of the atrialized ventricle as surgical treatment for Ebstein’s anomaly. On the other hand, Hetzer and colleagues [5] reported that tricuspid valve replacement alone may be sufficient, because incorporation of the atrialized chamber 0003-4975/07/$32.00 doi:10.1016/j.athoracsur.2007.06.065
FEATURE ARTICLES
Ann Thorac Surg 2007;84:1745– 6
1746
CASE REPORT IMAMURA ET AL HYPOPLASTIC LEFT HEART SYNDROME WITH HETEROTAXY SYNDROME
Ann Thorac Surg 2007;84:1746 – 8
Fig 1. Transverse computerized tomographic images at the level of the ninth thoracic vertebra showing release of compression and increase in the volume of the left ventricle after surgery. Open circles with dotted lines indicates the left ventricle. The preoperative and postoperative left ventricular volume was (A) 109.7 mL and (B) 158.4 mL, respectively.
FEATURE ARTICLES
into the contracting right ventricle may be beneficial in allowing sufficient right ventricular filling during diastole and could stimulate the remaining musculature in the atrialized chamber wall to hypertrophy and contribute to right ventricular contraction. Good results were reported after a follow-up of 10 to 103 months in that study [5]. However, the observation in our case suggests that in the long term, leaving the atrialized chamber in situ does not necessarily yield a good outcome. To treat the intractable heart failure in our patient, we considered it necessary to release the compression on the left ventricle and to protect the dysfunctional right ventricle from volume overload. In general, the bidirectional Glenn shunt is recognized as a step operation for a staged Fontan procedure, which is known to improve cyanosis and reduce the ventricular volume load in children with a single ventricle. Marianeschi and colleagues [6] suggested that an additional bidirectional Glenn shunt after repair or replacement of the tricuspid valve may be used as a surgical option for Ebstein’s anomaly patients with severe heart failure because of the reduced volume overload of the fragile right ventricle. They reported improvement of the exercise tolerance and right cardiac function after a follow-up of 2 to 24 months in their patients. However, there were no elderly or reoperation cases in their series. The condition of the pulmonary vascular bed determines the feasibility of a right heart bypass operation, including the Glenn shunt. Pulmonary hemodynamics as evaluated by pulmonary artery pressure, pulmonary-tosystemic flow ratio, and pulmonary artery index is essential to determine the feasibility of a right heart bypass operation [7]. In this case, right heart catheterization could not be conducted for investigating the pulmonary hemodynamics because of the earlier tricuspid valve replacement. However, we were certain that the pulmonary circulation could be established by one-and-a-half ventricular repair combined with right ventriculoplasty because of some favorable indices, such as a low mean pulmonary artery pressure of 13 mm Hg, absence of severe dilatation of the intrapulmonary vessels, and absence of pulmonary and prosthetic valve regurgitation. Postoperatively, the mean pressure in the inferior vena cava was 6 mm Hg. Echocardiography showed reduced dilatation of the right heart and improvement of the left ventricular ejection fraction. Finally, her quality of life improved and her serum brain natriuretic peptide level decreased. In conclusion, it is suggested that the Glenn © 2007 by The Society of Thoracic Surgeons Published by Elsevier Inc
shunt combined with repair of the tricuspid valve and the right ventricle may be a valuable surgical option for Ebstein’s anomaly patients with intractable heart failure.
References 1. Juergens KU, Seifarth H, Maintz D, et al. MDCT determination of volume and function of the left ventricle: are short-axis image reformations necessary? Am J Radiol 2006;186(6 Suppl 2):S371– 8. 2. Becker AE, Becker MJ, Edwards JE. Pathologic spectrum of dysplasia of the tricuspid valve. Features in common with Ebstein’s malformation. Arch Pathol 1971;91:167–78. 3. Carpentier A, Chauvaud S, Mac´e L, et al. A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1988;96:92–101. 4. Danielson GK, Driscoll DJ, Mair DD, Warnes CA, Oliver WC Jr. Operative treatment of Ebstein’s anomaly. J Thorac Cardiovasc Surg 1992;104:1195–202. 5. Hetzer R, Nagdyman N, Ewert P, et al. A modified repair technique for tricuspid incompetence in Ebstein’s anomaly. J Thorac Cardiovasc Surg 1998;115:857– 68. 6. Marianeschi SM, McElhinney DB, Reddy VM, Silverman NH, Hanley FL. Alternative approach to the repair of Ebstein’s malformation: intracardiac repair with ventricular unloading. Ann Thorac Surg 1998;66:1546 –50. 7. Chowdhury UK, Airan B, Sharma R, et al. One and a half ventricle repair with pulsatile bidirectional Glenn: results and guidelines for patient selection. Ann Thorac Surg 2001;71: 1995–2002.
Hypoplastic Left Heart Syndrome, Interrupted Inferior Vena Cava, Biliary Atresia Michiaki Imamura, MD, PhD, Umesh Dyamenahalli, MD, Ritu Sachdeva, MD, Evan R. Kokoska, MD, and Robert D. B. Jaquiss, MD Divisions of Pediatric Cardiothoracic Surgery, Pediatric Cardiology, and Pediatric General Surgery, University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, Little Rock, Arkansas
A neonate was diagnosed with hypoplastic left heart syndrome, heterotaxy syndrome, and interrupted inferior Accepted for publication May 29, 2007. Address correspondence to Dr Imamura, Division of Pediatric Cardiothoracic Surgery, Arkansas Children’s Hospital, 800 Marshall St, Slot 677, Little Rock, AR 72202; e-mail: [email protected].
0003-4975/07/$32.00 doi:10.1016/j.athoracsur.2007.05.068