- Email: [email protected]
Two-Patch Repair for Atrioventricular Septal Defect With Mitral Aneurysm
Ann Thorac Surg 2009;88:1341–3
References 1. Bland EF, White PD, Garland J. Congenital anomalies of the coronary arteries: report of an unusual case associated with cardiac hypertrophy. Am Heart J 1933;8:787– 801. 2. Keith JD. The anomalous origin of the left coronary artery from the pulmonary artery. Br Heart J 1959;21:149 – 61. 3. Wesselhoeft H, Fawcett JS, Johnson AL. Anomalous origin of the left coronary artery from the pulmonary trunk. Its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases. Circulation 1968;38:403–25. 4. Schwerzmann M, Salehian O, Elliot T, Merchant N, Siu SC, Webb GD. Images in cardiovascular medicine. Anomalous origin of the left coronary artery from the main pulmonary artery in adults: coronary collateralization at its best. Circulation 2004;110:e511–3. 5. Chan RK, Hare DL, Buxton BF. Anomalous left main coronary artery arising from the pulmonary artery in an adult: treatment by internal mammary artery grafting. J Thorac Cardiovasc Surg 1995;109:393– 4. 6. Sabiston DC, Jr, Neill CA, Taussig HB. The direction of blood flow in anomalous left coronary artery arising from the pulmonary artery. Circulation 1960;22:591–7. 7. Backer CL, Stout MJ, Zales VR, et al. Anomalous origin of the left coronary artery. A twenty-year review of surgical management. J Thorac Cardiovasc Surg 1992;103:1049 –57. 8. Collins N, Colman J, Benson L, Hansen M, Merchant N, Horlick E. Successful percutaneous treatment of anomalous left coronary artery from pulmonary artery. Int J Cardiol 2007;122:e29–31.
Two-Patch Repair for Atrioventricular Septal Defect With Mitral Aneurysm Hajime Imura, MD, Shun-ichiro Sakamoto, MD, Yuji Maruyama, MD, Masami Ochi, MD, and Kazuo Shimizu, MD Department of Surgery, Division of Cardiovascular Surgery, Nippon Medical School, Bunkyo-ku, Tokyo, Japan
We experienced an unusual case of partial atrioventricular septal defect in an elderly patient. A preoperative ultrasonic Accepted for publication Feb 13, 2009. Address correspondence to Dr Imura, 1-1-5, Sendagi Bunkyo-ku, Tokyo, 113-8603, Japan; e-mail: [email protected].
© 2009 by The Society of Thoracic Surgeons Published by Elsevier Inc
1341
cardiogram revealed the mitral leaflet pouching toward the right atrium and suggested the presence of a ventricular septal defect underneath the atrioventricular valve. The mitral aneurysm was diagnosed as a septal aneurysm on preoperative ultrasonic cardiogram. A crescent-shaped Dacron patch (InterVascular S. A., La Ciotat Cedex, France) was placed beneath the atrioventricular valve to prevent rupture of the mitral aneurysm and support the anterior mitral leaflet by creating a new annulus. We believe that this is the first report describing this type of mitral aneurysm and its surgical repair. (Ann Thorac Surg 2009;88:1341–3) © 2009 by The Society of Thoracic Surgeons
T
here are numerous variations in anatomy of the atrioventricular connection in atrioventricular septal defects (AVSDs) [1, 2]. Ultrasonic cardiogram (UCG) is the most common and useful examination to evaluate such structures [3]; however, it does not always provide fine detail or allow for accurate diagnosis of pathology. We present an elderly case of partial AVSD with mitral aneurysm in this report. In the present case, mitral aneurysm restricted an inter-atrial shunt mimicking the appearance of a ventricular septal defect and septal aneurysm in the preoperative UCG. Two-patch repair, which is normally performed for complete AVSD, was appropriate for this pathology. A 65-year-old woman was referred to our hospital for the surgical treatment of an atrial septal defect (ASD). The UCG showed a primary ASD and a deficient ventricular septum beneath the atrioventricular valve (Fig 1A). This anomaly was diagnosed as the partial AVSD with septal aneurysm, or the complete form of AVSD with extremely limited ventricular shunt through a membranous septal aneurysm. Moderate mitral regurgitation was seen from the cleft and the central region of the mitral valve. Systolic pressure in the pulmonary artery was 38 (approximately 35% of systemic pressure), and the ratio of pulmonary artery flow to systemic flow was 2.6. During the operation, the pathologic anatomy was investigated by way of a right atriotomy under ordinary cardiopulmonary bypass and cardioplegic arrest. The ventricular septum under the atrioventricular valve was protruded, and the anterior half of the tricuspid septal leaflet was absent (Fig 2A). A mitral cleft was seen between the ventricular septum and its free margin. First, we closed this cleft and inserted a tube into the left ventricle. Once the left ventricle was volume loaded, the mitral portion was bulged and tense, whereas the tricuspid portion did not show such changes (Fig 2B). No major cordal support was recognized beneath the bulged mitral leaflet. Thus, we suspected central mitral regurgitation was due to this unsupported mitral leaflet. To exclude the aneurysmal area from a new mitral leaflet and obtain adequate mitral coaptation, we elected to attach a Dacron patch (InterVascular S. A., La Ciotat Cedex, France) beneath the atrioventricular valve. For ASD closure, a strip of Dacron (3-mm wide) was attached to an autologous quadrangu0003-4975/09/$36.00 doi:10.1016/j.athoracsur.2009.02.057
FEATURE ARTICLES
graft or internal mammary artery grafting, and (2) reimplantation of the left main artery to the aorta directly or indirectly through a pulmonary artery tunnel or through an internal iliac artery graft [5]. Ligation of the left main artery was performed in 1959 [6]. This technique was shown to higher early and late mortality rates [7]. The high mortality was believed to be due to the fact that postoperatively the patients have one-vessel system. Subsequently, ligation of the left main artery with revascularization of the left coronary system was introduced and has been successful. Reimplantation of the left main artery to the aorta may not be possible because of anatomical reasons. Ligation of the anomalous artery with coronary artery bypass grafting has been the preferred approach in adults, and reimplantation of the left main coronary artery into the aorta has been the most frequently used surgical technique in the pediatric population [7]. Percutaneous ALCAPA closure using an Amplatzer Vascular Occluder has been recently reported [8].
CASE REPORT IMURA ET AL MITRAL ANEURYSM IN PARTIAL AVSD
1342
CASE REPORT IMURA ET AL MITRAL ANEURYSM IN PARTIAL AVSD
Ann Thorac Surg 2009;88:1341–3
appearance of ventricular septal defect covered with a membranous tissue in UCG. Therefore, preoperative diagnosis was difficult and problematic. In fact, a previous report contained similar UCG observations, although a different diagnosis was reached [6].
FEATURE ARTICLES
Fig 1. Transesophageal echocardiographic findings of (A) a mitral aneurysm seen on the ventricular septum. (B) Shown after two-patch repair.
lar pericardial patch. The Dacron edge was sutured to the atrioventricular valve. Thus, a two-patch repair was carried out. When the pericardial patch was built on the atrioventricular valve, the line of stitches on the mitral valve was determined to exclude the aneurysmal region from the new mitral valve. Because the regurgitation from the new mitral valve was trivial at this point, the ASD was closed with a pericardial patch (Figs 1B and 2C). To minimize tricuspid regurgitation, the accessory anterior leaflet was attached to the patch and covered the anterior septal area. The patient had a good postoperative course. Her UCG showed less than mild mitral and tricuspid regurgitation after 1 year.
Comment We believe that this is the first report presenting mitral aneurysm, most of which were previously reported as a complication or sequela of endocarditis [4, 5], occurring in an AVSD patient. In the present case, a mitral aneurysm extended toward the right ventricle, mimicking the
Fig 2. Surgical views through the right atriotomy (A) before repair. (B) Mitral aneurysm apparent with volume loading of the left ventricle. (C) Shown after two-patch repair.
A deficient cordal structure and leaflet malformation are always present in the atrioventricular valve of AVSD [7]. We speculate that aneurysmal change was developed under the condition of high pressure loading to the unsupported area. Although most mitral aneurysms have been reported as complications of endocarditis, the present case was likely due to a different pathology that necessitated a unique surgical procedure. Distinguishing mitral and tricuspid tissues in the atrioventricular valve was difficult. However, once the left ventricle was volume loaded, only the mitral portion of the atrioventricular valve was bulged; thereafter, the mitral and tricuspid areas were easily identified and the aneurysmal portion of the mitral leaflet could be recognized. To determine the size of the Dacron patch (DuPont) and its attachment line on the mitral leaflet, the anterior mitral leaflet had to be bulged. Although this technique is not normally used for partial AVSD, we attached a Dacron patch underneath the atrioventricular valve to secure the closure of the shunt from the left ventricle, exclude the aneurysmal region from the mitral valve, normalize the height of the atrioventricular valve, and obtain adequate mitral coaptation by pushing back the mitral leaflet to the left side. Because left atrioventricular valve regurgitation is an unresolved problem [8], creation of a new mitral annulus has certain benefits for further mitral surgery. The prosthetic mitral ring can be easily placed on this new annulus without injuring the conduction system. Because the annulus should be sufficiently solid for possible subsequent prosthetic annular ring or valve surgeries, we used a Dacron strip on the pericardial patch for ASD closure.
References 1. Taguchi K, Sasaki N, Okii Y, Matsuura Y, Hirao M. Surgical experience with persistent common atrioventricular canal in a series of eighty-two patients. J Thorac Cardiovasc Surg 1968; 55:501–17. 2. Sakakibara S, Yokoyama M, Konno S, Kudo T, Hatsune K. A consideration of operative methods for correction of endocardial cushion defect. Ann Thorac Surg 1971;12:419 –27. 3. Hagler DJ, Tajik AJ, Seward JB, Mair DD, Ritter DG. Real-time wide-angle sector echocardiography: atrioventricular canal defects. Circulation 1979;59:140 –50. 4. Halkos ME, Symbas JD, Felner JM, Symbas PN. Aneurysm of the mitral valve: a rare complication of aortic valve endocarditis. Ann Thorac Surg 2004;78:e65– 6. 5. Takayama T, Teramura M, Sakai H, et al. Perforated mitral aneurysm associated with Libman-Sacks endocarditis. Inter Med 2008;47:1605– 8. 6. Toh N, Kanzaki H, Nakatani S, et al. Partial atrioventricular septal defect assessed by real-time three-dimensional echocardiography: a case report. J Cardiol 2007;50:379 – 82. 7. Kanani M, Elliott M, Cook A, Juraszek A, Devine W, Anderson RH. Late incompetence of the left atrioventricular valve after repair of atrioventricular septal defects: the morphologic perspective. J Thorac Cardiovasc Surg 2006;132:640 – 6. 8. Murashita T, Kubota T, Oba J, Aoki T, Matano J, Yasuda K. Left atrioventricular valve regurgitation after repair of incomplete atrioventricular septal defect. Ann Thorac Surg 2004;77: 2157– 62. © 2009 by The Society of Thoracic Surgeons Published by Elsevier Inc
CASE REPORT AL-KHALDI ET AL OPERATION FOR ARTERIAL TORTUOSITY SYNDROME
1343
Successful Surgical Pulmonary Artery Reconstruction in Arterial Tortuosity Syndrome Abdulaziz Al-Khaldi, MD, Abdulmohsen Alharbi, MD, Omar Tamimi, MD, and Yasser Mohammed, MD Divisions of Cardiac Surgery, Cardiac Anesthesia, and Pediatric Cardiology, King Abdulaziz Medical City, Riyadh, Saudi Arabia
The outcomes of surgical repair for diffuse pulmonary artery stenoses due to arterial tortuosity syndrome are unknown. We report a 1-year-old girl with tortuosity syndrome who presented with suprasystemic right ventricular pressure due to distortion and stenoses of the pulmonary artery branches. We describe the operative findings of this unique pathology and the surgical techniques used to completely reconstruct the pulmonary arterial tree, with normalization of the right ventricular pressure. (Ann Thorac Surg 2009;88:1343–5) © 2009 by The Society of Thoracic Surgeons
S
evere right ventricular (RV) hypertension due to pulmonary artery stenosis (PAS) is a serious condition that can cause RV dysfunction, tricuspid insufficiency, and ultimately, death if not corrected. Although PAS is potentially correctable, lesions involving the lobar and segmental branches continue to be technically challenging. We present our experience with single-stage complete surgical intraparenchymal pulmonary artery (PA) reconstruction in a case of arterial tortuosity syndrome (ATS) and diffuse bilateral PAS. A 1-year-old girl presented with failure to thrive. Her medical history included repair of a right-sided diaphragmatic hernia, and she also had umbilical and inguinal hernias. The physical examination revealed low arterial saturation on room air (80%), low body weight for age (5.4 kg), hepatomegaly, increased joint laxity, RV heave, and systolic murmur in the lower left parasternal border. Echocardiography showed severe dilatation of the RV, with significant dysfunction (Fig 1A) and severe tricuspid regurgitation, with an estimated RV systolic pressure of 80 mm Hg. Cardiac catheterization showed extensive tortuosity of the aorta and the great arteries, aneurysmal enlargement of the main PA, tortuosity of all PA branches with long segment stenoses, and kinking of the intraparenchymal arteries (Fig 1B). The measured RV pressure was suprasystemic. We decided to proceed with a single-stage bilateral reconstruction of the pulmonary arteries through a me-
Accepted for publication Feb 9, 2009. Address correspondence to Dr Al-Khaldi, Division of Cardiac Surgery, King Abdulaziz Medical City, PO Box 22490, Riyadh, 11426, Saudi Arabia; e-mail: [email protected].
0003-4975/09/$36.00 doi:10.1016/j.athoracsur.2009.02.020
FEATURE ARTICLES
Ann Thorac Surg 2009;88:1343–5
References 1. Bland EF, White PD, Garland J. Congenital anomalies of the coronary arteries: report of an unusual case associated with cardiac hypertrophy. Am Heart J 1933;8:787– 801. 2. Keith JD. The anomalous origin of the left coronary artery from the pulmonary artery. Br Heart J 1959;21:149 – 61. 3. Wesselhoeft H, Fawcett JS, Johnson AL. Anomalous origin of the left coronary artery from the pulmonary trunk. Its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases. Circulation 1968;38:403–25. 4. Schwerzmann M, Salehian O, Elliot T, Merchant N, Siu SC, Webb GD. Images in cardiovascular medicine. Anomalous origin of the left coronary artery from the main pulmonary artery in adults: coronary collateralization at its best. Circulation 2004;110:e511–3. 5. Chan RK, Hare DL, Buxton BF. Anomalous left main coronary artery arising from the pulmonary artery in an adult: treatment by internal mammary artery grafting. J Thorac Cardiovasc Surg 1995;109:393– 4. 6. Sabiston DC, Jr, Neill CA, Taussig HB. The direction of blood flow in anomalous left coronary artery arising from the pulmonary artery. Circulation 1960;22:591–7. 7. Backer CL, Stout MJ, Zales VR, et al. Anomalous origin of the left coronary artery. A twenty-year review of surgical management. J Thorac Cardiovasc Surg 1992;103:1049 –57. 8. Collins N, Colman J, Benson L, Hansen M, Merchant N, Horlick E. Successful percutaneous treatment of anomalous left coronary artery from pulmonary artery. Int J Cardiol 2007;122:e29–31.
Two-Patch Repair for Atrioventricular Septal Defect With Mitral Aneurysm Hajime Imura, MD, Shun-ichiro Sakamoto, MD, Yuji Maruyama, MD, Masami Ochi, MD, and Kazuo Shimizu, MD Department of Surgery, Division of Cardiovascular Surgery, Nippon Medical School, Bunkyo-ku, Tokyo, Japan
We experienced an unusual case of partial atrioventricular septal defect in an elderly patient. A preoperative ultrasonic Accepted for publication Feb 13, 2009. Address correspondence to Dr Imura, 1-1-5, Sendagi Bunkyo-ku, Tokyo, 113-8603, Japan; e-mail: [email protected].
© 2009 by The Society of Thoracic Surgeons Published by Elsevier Inc
1341
cardiogram revealed the mitral leaflet pouching toward the right atrium and suggested the presence of a ventricular septal defect underneath the atrioventricular valve. The mitral aneurysm was diagnosed as a septal aneurysm on preoperative ultrasonic cardiogram. A crescent-shaped Dacron patch (InterVascular S. A., La Ciotat Cedex, France) was placed beneath the atrioventricular valve to prevent rupture of the mitral aneurysm and support the anterior mitral leaflet by creating a new annulus. We believe that this is the first report describing this type of mitral aneurysm and its surgical repair. (Ann Thorac Surg 2009;88:1341–3) © 2009 by The Society of Thoracic Surgeons
T
here are numerous variations in anatomy of the atrioventricular connection in atrioventricular septal defects (AVSDs) [1, 2]. Ultrasonic cardiogram (UCG) is the most common and useful examination to evaluate such structures [3]; however, it does not always provide fine detail or allow for accurate diagnosis of pathology. We present an elderly case of partial AVSD with mitral aneurysm in this report. In the present case, mitral aneurysm restricted an inter-atrial shunt mimicking the appearance of a ventricular septal defect and septal aneurysm in the preoperative UCG. Two-patch repair, which is normally performed for complete AVSD, was appropriate for this pathology. A 65-year-old woman was referred to our hospital for the surgical treatment of an atrial septal defect (ASD). The UCG showed a primary ASD and a deficient ventricular septum beneath the atrioventricular valve (Fig 1A). This anomaly was diagnosed as the partial AVSD with septal aneurysm, or the complete form of AVSD with extremely limited ventricular shunt through a membranous septal aneurysm. Moderate mitral regurgitation was seen from the cleft and the central region of the mitral valve. Systolic pressure in the pulmonary artery was 38 (approximately 35% of systemic pressure), and the ratio of pulmonary artery flow to systemic flow was 2.6. During the operation, the pathologic anatomy was investigated by way of a right atriotomy under ordinary cardiopulmonary bypass and cardioplegic arrest. The ventricular septum under the atrioventricular valve was protruded, and the anterior half of the tricuspid septal leaflet was absent (Fig 2A). A mitral cleft was seen between the ventricular septum and its free margin. First, we closed this cleft and inserted a tube into the left ventricle. Once the left ventricle was volume loaded, the mitral portion was bulged and tense, whereas the tricuspid portion did not show such changes (Fig 2B). No major cordal support was recognized beneath the bulged mitral leaflet. Thus, we suspected central mitral regurgitation was due to this unsupported mitral leaflet. To exclude the aneurysmal area from a new mitral leaflet and obtain adequate mitral coaptation, we elected to attach a Dacron patch (InterVascular S. A., La Ciotat Cedex, France) beneath the atrioventricular valve. For ASD closure, a strip of Dacron (3-mm wide) was attached to an autologous quadrangu0003-4975/09/$36.00 doi:10.1016/j.athoracsur.2009.02.057
FEATURE ARTICLES
graft or internal mammary artery grafting, and (2) reimplantation of the left main artery to the aorta directly or indirectly through a pulmonary artery tunnel or through an internal iliac artery graft [5]. Ligation of the left main artery was performed in 1959 [6]. This technique was shown to higher early and late mortality rates [7]. The high mortality was believed to be due to the fact that postoperatively the patients have one-vessel system. Subsequently, ligation of the left main artery with revascularization of the left coronary system was introduced and has been successful. Reimplantation of the left main artery to the aorta may not be possible because of anatomical reasons. Ligation of the anomalous artery with coronary artery bypass grafting has been the preferred approach in adults, and reimplantation of the left main coronary artery into the aorta has been the most frequently used surgical technique in the pediatric population [7]. Percutaneous ALCAPA closure using an Amplatzer Vascular Occluder has been recently reported [8].
CASE REPORT IMURA ET AL MITRAL ANEURYSM IN PARTIAL AVSD
1342
CASE REPORT IMURA ET AL MITRAL ANEURYSM IN PARTIAL AVSD
Ann Thorac Surg 2009;88:1341–3
appearance of ventricular septal defect covered with a membranous tissue in UCG. Therefore, preoperative diagnosis was difficult and problematic. In fact, a previous report contained similar UCG observations, although a different diagnosis was reached [6].
FEATURE ARTICLES
Fig 1. Transesophageal echocardiographic findings of (A) a mitral aneurysm seen on the ventricular septum. (B) Shown after two-patch repair.
lar pericardial patch. The Dacron edge was sutured to the atrioventricular valve. Thus, a two-patch repair was carried out. When the pericardial patch was built on the atrioventricular valve, the line of stitches on the mitral valve was determined to exclude the aneurysmal region from the new mitral valve. Because the regurgitation from the new mitral valve was trivial at this point, the ASD was closed with a pericardial patch (Figs 1B and 2C). To minimize tricuspid regurgitation, the accessory anterior leaflet was attached to the patch and covered the anterior septal area. The patient had a good postoperative course. Her UCG showed less than mild mitral and tricuspid regurgitation after 1 year.
Comment We believe that this is the first report presenting mitral aneurysm, most of which were previously reported as a complication or sequela of endocarditis [4, 5], occurring in an AVSD patient. In the present case, a mitral aneurysm extended toward the right ventricle, mimicking the
Fig 2. Surgical views through the right atriotomy (A) before repair. (B) Mitral aneurysm apparent with volume loading of the left ventricle. (C) Shown after two-patch repair.
A deficient cordal structure and leaflet malformation are always present in the atrioventricular valve of AVSD [7]. We speculate that aneurysmal change was developed under the condition of high pressure loading to the unsupported area. Although most mitral aneurysms have been reported as complications of endocarditis, the present case was likely due to a different pathology that necessitated a unique surgical procedure. Distinguishing mitral and tricuspid tissues in the atrioventricular valve was difficult. However, once the left ventricle was volume loaded, only the mitral portion of the atrioventricular valve was bulged; thereafter, the mitral and tricuspid areas were easily identified and the aneurysmal portion of the mitral leaflet could be recognized. To determine the size of the Dacron patch (DuPont) and its attachment line on the mitral leaflet, the anterior mitral leaflet had to be bulged. Although this technique is not normally used for partial AVSD, we attached a Dacron patch underneath the atrioventricular valve to secure the closure of the shunt from the left ventricle, exclude the aneurysmal region from the mitral valve, normalize the height of the atrioventricular valve, and obtain adequate mitral coaptation by pushing back the mitral leaflet to the left side. Because left atrioventricular valve regurgitation is an unresolved problem [8], creation of a new mitral annulus has certain benefits for further mitral surgery. The prosthetic mitral ring can be easily placed on this new annulus without injuring the conduction system. Because the annulus should be sufficiently solid for possible subsequent prosthetic annular ring or valve surgeries, we used a Dacron strip on the pericardial patch for ASD closure.
References 1. Taguchi K, Sasaki N, Okii Y, Matsuura Y, Hirao M. Surgical experience with persistent common atrioventricular canal in a series of eighty-two patients. J Thorac Cardiovasc Surg 1968; 55:501–17. 2. Sakakibara S, Yokoyama M, Konno S, Kudo T, Hatsune K. A consideration of operative methods for correction of endocardial cushion defect. Ann Thorac Surg 1971;12:419 –27. 3. Hagler DJ, Tajik AJ, Seward JB, Mair DD, Ritter DG. Real-time wide-angle sector echocardiography: atrioventricular canal defects. Circulation 1979;59:140 –50. 4. Halkos ME, Symbas JD, Felner JM, Symbas PN. Aneurysm of the mitral valve: a rare complication of aortic valve endocarditis. Ann Thorac Surg 2004;78:e65– 6. 5. Takayama T, Teramura M, Sakai H, et al. Perforated mitral aneurysm associated with Libman-Sacks endocarditis. Inter Med 2008;47:1605– 8. 6. Toh N, Kanzaki H, Nakatani S, et al. Partial atrioventricular septal defect assessed by real-time three-dimensional echocardiography: a case report. J Cardiol 2007;50:379 – 82. 7. Kanani M, Elliott M, Cook A, Juraszek A, Devine W, Anderson RH. Late incompetence of the left atrioventricular valve after repair of atrioventricular septal defects: the morphologic perspective. J Thorac Cardiovasc Surg 2006;132:640 – 6. 8. Murashita T, Kubota T, Oba J, Aoki T, Matano J, Yasuda K. Left atrioventricular valve regurgitation after repair of incomplete atrioventricular septal defect. Ann Thorac Surg 2004;77: 2157– 62. © 2009 by The Society of Thoracic Surgeons Published by Elsevier Inc
CASE REPORT AL-KHALDI ET AL OPERATION FOR ARTERIAL TORTUOSITY SYNDROME
1343
Successful Surgical Pulmonary Artery Reconstruction in Arterial Tortuosity Syndrome Abdulaziz Al-Khaldi, MD, Abdulmohsen Alharbi, MD, Omar Tamimi, MD, and Yasser Mohammed, MD Divisions of Cardiac Surgery, Cardiac Anesthesia, and Pediatric Cardiology, King Abdulaziz Medical City, Riyadh, Saudi Arabia
The outcomes of surgical repair for diffuse pulmonary artery stenoses due to arterial tortuosity syndrome are unknown. We report a 1-year-old girl with tortuosity syndrome who presented with suprasystemic right ventricular pressure due to distortion and stenoses of the pulmonary artery branches. We describe the operative findings of this unique pathology and the surgical techniques used to completely reconstruct the pulmonary arterial tree, with normalization of the right ventricular pressure. (Ann Thorac Surg 2009;88:1343–5) © 2009 by The Society of Thoracic Surgeons
S
evere right ventricular (RV) hypertension due to pulmonary artery stenosis (PAS) is a serious condition that can cause RV dysfunction, tricuspid insufficiency, and ultimately, death if not corrected. Although PAS is potentially correctable, lesions involving the lobar and segmental branches continue to be technically challenging. We present our experience with single-stage complete surgical intraparenchymal pulmonary artery (PA) reconstruction in a case of arterial tortuosity syndrome (ATS) and diffuse bilateral PAS. A 1-year-old girl presented with failure to thrive. Her medical history included repair of a right-sided diaphragmatic hernia, and she also had umbilical and inguinal hernias. The physical examination revealed low arterial saturation on room air (80%), low body weight for age (5.4 kg), hepatomegaly, increased joint laxity, RV heave, and systolic murmur in the lower left parasternal border. Echocardiography showed severe dilatation of the RV, with significant dysfunction (Fig 1A) and severe tricuspid regurgitation, with an estimated RV systolic pressure of 80 mm Hg. Cardiac catheterization showed extensive tortuosity of the aorta and the great arteries, aneurysmal enlargement of the main PA, tortuosity of all PA branches with long segment stenoses, and kinking of the intraparenchymal arteries (Fig 1B). The measured RV pressure was suprasystemic. We decided to proceed with a single-stage bilateral reconstruction of the pulmonary arteries through a me-
Accepted for publication Feb 9, 2009. Address correspondence to Dr Al-Khaldi, Division of Cardiac Surgery, King Abdulaziz Medical City, PO Box 22490, Riyadh, 11426, Saudi Arabia; e-mail: [email protected].
0003-4975/09/$36.00 doi:10.1016/j.athoracsur.2009.02.020
FEATURE ARTICLES
Ann Thorac Surg 2009;88:1343–5