Glenn sonrası bulboventriküler foramen darlığı saptanan iki tek ventrikül olgusuna cerrahi yaklaşım
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Glenn sonrası bulboventriküler foramen darlığı saptanan iki tek ventrikül olgusuna cerrahi yaklaşım
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Ähnlich wie Glenn sonrası bulboventriküler foramen darlığı saptanan iki tek ventrikül olgusuna cerrahi yaklaşım
Ähnlich wie Glenn sonrası bulboventriküler foramen darlığı saptanan iki tek ventrikül olgusuna cerrahi yaklaşım (20)
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Glenn sonrası bulboventriküler foramen darlığı saptanan iki tek ventrikül olgusuna cerrahi yaklaşım
- 1. Glenn sonrası bulboventriküler foramen darlı ı saptanan iki tekğ ventrikül olgusuna cerrahi yakla ımş Rıza Türköz, Can Vuran, Bülent Sarıta ,ş Canan Ayabakan, Kür ad Tokelş
- 2. Vaka 1Vaka 1 15 aylık kız çocu uğ15 aylık kız çocu uğ 3 aylıkken PAB, AK tamiri ve atrial septektomi (CPB)3 aylıkken PAB, AK tamiri ve atrial septektomi (CPB) 11 aylıkken BDCPS11 aylıkken BDCPS HuzursuzlukHuzursuzluk Fizik muayeneFizik muayene SiyanozSiyanoz ÜfürümÜfürüm Vaka 2Vaka 2 2.5 ya ında kız çocu uş ğ2.5 ya ında kız çocu uş ğ 5 aylıkken BH atrial septektomi ve PAB5 aylıkken BH atrial septektomi ve PAB 10 aylıkken BDCPS10 aylıkken BDCPS Fizik muayene (KKY +)Fizik muayene (KKY +) Periferik dola ım bozuklu u,ş ğPeriferik dola ım bozuklu u,ş ğ Yüzde, gözde ve pretibial ödemYüzde, gözde ve pretibial ödem Takipne, retraksiyonTakipne, retraksiyon KC 2 cm, siyanozKC 2 cm, siyanoz Sol 2. IKA 2/6 sistolik ejeksiyon üfürümüSol 2. IKA 2/6 sistolik ejeksiyon üfürümü KKY sebebiyle yatırıldıKKY sebebiyle yatırıldı OlgularOlgular
- 3. Tanı: EkokardiEkokardiyyografiografi Vaka 1Vaka 1 DILVDILV,, ASD (septektomi)ASD (septektomi) VSD (restriktif 7 mm, 62 mmHg grad)VSD (restriktif 7 mm, 62 mmHg grad) Hipoplastik morf RVHipoplastik morf RV V-A diskordansV-A diskordans L malpozisyon (sol anterior aorta)L malpozisyon (sol anterior aorta) Pulmoner atrazi (sekonder)Pulmoner atrazi (sekonder),, TY (hafif)TY (hafif) Bilateral BDCPS yapılmı (çift SVC)şBilateral BDCPS yapılmı (çift SVC)ş Vaka 2Vaka 2 DILVDILV,, ASD (septektomi)ASD (septektomi) VSD (restriktif 4.4 mm , 39 mmHg gradiyent)VSD (restriktif 4.4 mm , 39 mmHg gradiyent) Hipoplastik morf RVHipoplastik morf RV V-A diskordansV-A diskordans L malpozisyon (sol anterior aorta)L malpozisyon (sol anterior aorta) BDCPS yapılmışBDCPS yapılmış
- 4. Tanı: Kateter veKateter ve anjioanjio Vaka 1Vaka 1 anjio yapılmadıanjio yapılmadı Vaka 2Vaka 2 LA= 19 mmHg, RA=17 mmHg,LA= 19 mmHg, RA=17 mmHg, mLV=195/0-13 mmHg, mRV=130/0-8mLV=195/0-13 mmHg, mRV=130/0-8 mmHg, Ao= 98/64 mmHgmmHg, Ao= 98/64 mmHg VSD düzeyinde 65 mmHg,VSD düzeyinde 65 mmHg, Subaortik düzeyde 32 mmHg gradiyentSubaortik düzeyde 32 mmHg gradiyent
- 5. OperasyonOperasyon Innominat arter, RA ve vena kavaInnominat arter, RA ve vena kava kanükasyonu KPB ve arrest sa landı.ğkanükasyonu KPB ve arrest sa landı.ğ PA disseke edildi ve ligasyon seviyesindenPA disseke edildi ve ligasyon seviyesinden kesildi.kesildi. Distal PA kapatıldı (peç ve primer)Distal PA kapatıldı (peç ve primer) Proksimalde ligasyon düzeyi rezeke edildi.Proksimalde ligasyon düzeyi rezeke edildi. Bir olguda trombüs temizlendiBir olguda trombüs temizlendi Aort ile PA çevresinin 1/3 birbirineAort ile PA çevresinin 1/3 birbirine dikildikten sonra üzerine distal aortadikildikten sonra üzerine distal aorta anastomoz edildi.anastomoz edildi.
- 10. OperasyonOperasyon Aortik klemp : 38, 90 dkAortik klemp : 38, 90 dk KPB : 102, 186 dkKPB : 102, 186 dk
- 11. PostoperatifPostoperatif Postoperatif 0.- 2. gün ekstübePostoperatif 0.- 2. gün ekstübe Postoperatif 1. - 3. gün servisPostoperatif 1. - 3. gün servis Postoperatif 5.-12. gün taburcuPostoperatif 5.-12. gün taburcu edildi.edildi.
- 12. KontKontrol Ekokardiografisinde Vaka 1Vaka 1 DSK anastomoz bölgesinde 10 mmHg grad Restriktif VSD grad 5 mmHg AY ve PY saptanmadı Vaka 2Vaka 2 DSK anastomoz bölgesinde gradiyent yokDSK anastomoz bölgesinde gradiyent yok Subaortik/aortik grad 11 mmHgSubaortik/aortik grad 11 mmHg AY ve PY saptanmadı EF %51-55 saptandı.EF %51-55 saptandı.
- 13. TartısmaTartısma Single Ventrikülde Subaortik DarlıkSingle Ventrikülde Subaortik Darlık DILV ve VA diskordanstaDILV ve VA diskordansta TA li TGA olgularıTA li TGA olguları Darlık LokalizasyonuDarlık Lokalizasyonu BVFBVF Kas yapılarınaKas yapılarına AV kapa a ba lı yapılar ileğ ğAV kapa a ba lı yapılar ileğ ğ
- 15. J Am Coll Cardiol. 1992 Jan;19(1):142-8. Bulboventricular foramen size in infants with double- inlet left ventricle or tricuspid atresia with transposed great arteries: influence on initial palliative operation and rate of growth. Matitiau A, et al Department of Cardiology, Children's Hospital, Boston, Massachusetts 02115. Bulboventricular foramen obstruction may complicate the management of patients with single left ventricle. Bulboventricular foramen size was measured in 28 neonates and infants greater than 5 months old and followed up for 2 to 5 years in those patients whose only systemic outflow was through the foramen. The bulboventricular foramen was measured in two planes by two-dimensional echocardiography, its area calculated and indexed to body surface area. One patient died before surgical treatment. The mean initial bulboventricular foramen area index was 0.94 cm2/m2 in 12 patients (Group A) in whom the foramen was bypassed as the first procedure in early infancy. The remaining 15 patients underwent other palliative operations but the bulboventricular foramen continued to serve as the systemic outflow tract. There was one surgical death. Six (Group B) of the 14 survivors developed bulboventricular foramen obstruction during follow-up (mean initial bulboventricular foramen area index 1.75 cm2/m2). The remaining eight patients (Group C) did not develop obstruction during follow-up and had an initial bulboventricular foramen larger than that in the other two groups (mean initial bulboventricular foramen area index 3.95 cm2/m2). All patients with an initial bulboventricular foramen area index less than 2 cm2 /m2 who did not undergo early bulboventricular foramen bypass developed late obstruction.
- 20. TartısmaTartısma Bulboventriküler foramen darlı ındağBulboventriküler foramen darlı ındağ BVF rezeksiyonuBVF rezeksiyonu DezavantajlarıDezavantajları AV blokAV blok Ventrikül disfonksiyonuVentrikül disfonksiyonu Rekürent ve rezudüv obstrüksiyonRekürent ve rezudüv obstrüksiyon Ventriküler anevrizmaVentriküler anevrizma Arterial Switch operasyonuArterial Switch operasyonu DSK operasyonuDSK operasyonu AvantajlarıAvantajları Outflow da dü ük gradiyentşOutflow da dü ük gradiyentş Blok riski yokBlok riski yok Reoperasyon insidansı dü ükşReoperasyon insidansı dü ükş DezavantajıDezavantajı Semiulnar kapaklarda yetmezlikSemiulnar kapaklarda yetmezlik
- 22. TartısmaTartısma BDCPS ve Fontan sonrası strok insidensi %2-4BDCPS ve Fontan sonrası strok insidensi %2-4 PolisitemiPolisitemi ntrakardiak R-L antİ şntrakardiak R-L antİ ş Koagülasyon bozuklu uğKoagülasyon bozuklu uğ Prostatik materyalProstatik materyal ntrakardiak trombüsİntrakardiak trombüsİ AritmiAritmi Ba lanmı PA kökü !!!!(antikoagülasyon)ğ şBa lanmı PA kökü !!!!(antikoagülasyon)ğ ş Strok önlemek için kapa ın dikilmesiğ Homogreft takılma zorlu u ve uzun dönem sonuçlarığ
- 23. SonuçSonuç Aortanın bulboventrikülerAortanın bulboventriküler foramenden çıktı ı singleğforamenden çıktı ı singleğ ventrikül olgularında erkenventrikül olgularında erken dönemde LVOTO olu mamasınaşdönemde LVOTO olu mamasınaş ra men ileriki dönemde olu abilir.ğ şra men ileriki dönemde olu abilir.ğ ş Bu olgularda DSK operasyonuBu olgularda DSK operasyonu LVOTO gidermek için tercihLVOTO gidermek için tercih edilmesi gereken bir yöntemdiredilmesi gereken bir yöntemdir..
Hinweis der Redaktion
- YD döneminde sarılık nedeniyle hastaneye yatırılmış
- She had TGA, VSD, foramen ovale and LVOTO. The 2.5 mm VSD was located in the trabecular outlet region and the LVOTO was caused by the posterior malalignment of the outlet septum. She also had a bicuspid pulmonary valve which was not stenotic. There was 65 mmHg Doppler gradient across the LVOT
- Her iki olgu Fonksiyonel kapasitesi iyi olarak izlenmektedir.
- Introduction Outcome after biventricular repair of atrial isomerism associated with complex cardiac anomalies is improved with the advancements in preoperative, anesthetic, surgical, and postoperative management (1). However the complex malformations and anomalous venous drainage with heterotaxy syndromes may pose a significant surgical challenge (2). Several different approaches have been used to correct the anomalous drainage of the left superior vena cava (LSVC) to the left atrium. These include extracardiac reimplantation techniques, intraatrial baffles, simple ligations, and cavopulmonary anastomoses (2). In a small heart, where the orifices of the systemic and pulmonary veins are very close to each other, the creation of an intraatrial baffle may be very difficult and may lead to early baffle stenosis. Extracardiac techniques may frequently be inapplicable. In these circumstances uncommon techniques may need to be experienced. In this report, we present an infant with dextrocardia, anomalous systemic venous return, left atrial isomerism, partial atrioventricular septal defect, common atrium, and muscular ventricular septal defects (VSD), who underwent an intraatrial baffle repair of anomalous systemic venous return excluding hepatic venous drainage. Comment Many patients with heterotaxy syndromes will require a Fontan operation due to the complexity of the anatomy, while only a subset of patients will be good candidates for biventricular repair (1). The systemic venous system manifests a wide spectrum of structural abnormalities in these patients, and may significantly alter the surgical management of specific lesions. Principles of repair include the division of any extracardiac communication and intracardiac septation of the venous return from pulmonary and systemic veins. Intracardiac and extracardiac repair have been used to correct the left superior vena caval drainage to the left AV valve with no connecting vein (4). The intracardiac approach, however, may be technically difficult in a small atrium where the pulmonary and systemic vein orifices are close to each other. Baffle complications including p ulmonary and the systemic venous obstruction as a late complication after atrial switch operation has been reported as 25% (5). This rate is expected to be much higher in isomeric hearts, especially when this operation is performed early and in a small atrium. Since early baffle obstruction is the major concern, extracardiac repair is more suitable in these patients whenever possible. In our case, extracardiac repair was not possible because the posterior position of the right-sided appendage precluded division of the LSVC and its anastamosis to the right sided atrial appendage. Simple ligation was not an option either, since the patient had single SVC. LSVC to left pulmonary artery anastamosis could not be performed due to high mean pulmonary artery pressure. Therefore, intracardiac repair was preferred despite a small common atrium and close proximity of the pulmonary and systemic vein orifices. Including the hepatic veins in the systemic venous baffle would create a risk of baffle obstruction and pulmonary vein stenosis, hence the hepatic veins were left to drain into systemic circulation along with the pulmonary veins. Autologous pericardial patch is known to shrink in the long term after Mustard type repair and baffle obstructions are common (6). Therefore a patch of bovine pericardium was used to create the intraatrial baffle and to augment the atrium from the atriotomy site. The hepatic venous system is the final common pathway of hepatic arterial and portal venous blood after sinusoidal mixing in normal liver. It is thus the drainage tract of the entire splanchnic circulation. Under normal condition, hepatic venous blood is about two-thirds saturated with oxygen and the liver accounts for some 20% of the total oxygen consumption of the body (7). So when the hepatic veins drain into the systemic circulation, the arterial oxygen saturation is expected to drop to 93-94%. Our patient had similar saturation recordings postoperatively. In patients having single ventricle physiology, excluding the hepatic venous effluent from the pulmonary circulation may cause pulmonary arteriovenous malformations to develop after cavapulmonary shunt. Patients with left atrial isomerism, especially those who undergo the Kawashima operation with an interrupted inferior vena cava and azygous continuation may have rapid development of pulmonary arteriovenous malformations, incidence of which has been reported to be as high as 58% within 5 years of operation (8). The pathophysiology of pulmonary arteriovenous malformations is not well understood. Absence of pulsatile blood flow has also been implicated in the development of pulmonary arteriovenous malformations. It is not yet known whether excluding the hepatic effluent from the pulmonary circulation after a biventricular repair will cause pulmonary arteriovenous malformation in the log term. An intraatrial baffle for anomalous systemic venous return without hepatic venous drainage is a simple and an effective solution in complex cardiac anomalies with small atria because it avoids crowding of the long intraatrial baffles for systemic and hepatic veins. Slightly lower oxygen saturation may cause dyspnea on exertion as our patient becomes physically more active. Pulmonary arteriovenous malformations may be another consequence in the long term. If the patient becomes symptomatic or more cyanotic the enlarged atrium created in the first operation may facilitate easier rerouting of the hepatic veins to the pulmonary circulation in a following operation. References Lim HG, Bacha EA, Marx GR, Marshall A, Fynn-Thompson F, Mayer JE, Del Nido P, Pigula FA. Biventricular repair in patients with heterotaxy syndrome. J Thorac Cardiovasc Surg. 2009;137:371-9. Palacios-Macedo AX, Fraser CD Jr. Correction of anomalous systemic venous drainage in heterotaxy syndrome. Ann Thorac Surg. 1997;64:235-7. Zimand S, Benjamin P, Frand M, Mishaly D, Smolinsky AK, Hegesh J. Left superior vena cava to the left atrium: do we have to change the traditional approach? Ann Thorac Surg. 1999;68:1869-71. Vargas FJ. Reconstructive methods for anomalous systemic venous return: surgical management of persistent left superior vena cava. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2008;11:31-8. Trusler GA, Williams WG, Duncan KF, Hesslein PS, Benson LN, Freedom RM, Izukawa T, Olley PM. Results with the Mustard operation in simple transposition of the great arteries 1963-1985. Ann Surg. 1987;206:251-60. Hörer J, Herrmann F, Schreiber C, Cleuziou J, Prodan Z, Vogt M, Holper K, Lange R. How well are patients doing up to 30 years after a mustard operation? Thorac Cardiovasc Surg. 2007;55:359-64. Mathie RT, Wheatly AM, Blumgart LH. Liver blood flow: physiology, measurement and clinical relevance. In: Blumgart LH, eds. Surgery of the liver and biliary tract. 2nd ed London:Churchill Livingstone;1994:95-110 .
- Introduction Outcome after biventricular repair of atrial isomerism associated with complex cardiac anomalies is improved with the advancements in preoperative, anesthetic, surgical, and postoperative management (1). However the complex malformations and anomalous venous drainage with heterotaxy syndromes may pose a significant surgical challenge (2). Several different approaches have been used to correct the anomalous drainage of the left superior vena cava (LSVC) to the left atrium. These include extracardiac reimplantation techniques, intraatrial baffles, simple ligations, and cavopulmonary anastomoses (2). In a small heart, where the orifices of the systemic and pulmonary veins are very close to each other, the creation of an intraatrial baffle may be very difficult and may lead to early baffle stenosis. Extracardiac techniques may frequently be inapplicable. In these circumstances uncommon techniques may need to be experienced. In this report, we present an infant with dextrocardia, anomalous systemic venous return, left atrial isomerism, partial atrioventricular septal defect, common atrium, and muscular ventricular septal defects (VSD), who underwent an intraatrial baffle repair of anomalous systemic venous return excluding hepatic venous drainage. Comment Many patients with heterotaxy syndromes will require a Fontan operation due to the complexity of the anatomy, while only a subset of patients will be good candidates for biventricular repair (1). The systemic venous system manifests a wide spectrum of structural abnormalities in these patients, and may significantly alter the surgical management of specific lesions. Principles of repair include the division of any extracardiac communication and intracardiac septation of the venous return from pulmonary and systemic veins. Intracardiac and extracardiac repair have been used to correct the left superior vena caval drainage to the left AV valve with no connecting vein (4). The intracardiac approach, however, may be technically difficult in a small atrium where the pulmonary and systemic vein orifices are close to each other. Baffle complications including p ulmonary and the systemic venous obstruction as a late complication after atrial switch operation has been reported as 25% (5). This rate is expected to be much higher in isomeric hearts, especially when this operation is performed early and in a small atrium. Since early baffle obstruction is the major concern, extracardiac repair is more suitable in these patients whenever possible. In our case, extracardiac repair was not possible because the posterior position of the right-sided appendage precluded division of the LSVC and its anastamosis to the right sided atrial appendage. Simple ligation was not an option either, since the patient had single SVC. LSVC to left pulmonary artery anastamosis could not be performed due to high mean pulmonary artery pressure. Therefore, intracardiac repair was preferred despite a small common atrium and close proximity of the pulmonary and systemic vein orifices. Including the hepatic veins in the systemic venous baffle would create a risk of baffle obstruction and pulmonary vein stenosis, hence the hepatic veins were left to drain into systemic circulation along with the pulmonary veins. Autologous pericardial patch is known to shrink in the long term after Mustard type repair and baffle obstructions are common (6). Therefore a patch of bovine pericardium was used to create the intraatrial baffle and to augment the atrium from the atriotomy site. The hepatic venous system is the final common pathway of hepatic arterial and portal venous blood after sinusoidal mixing in normal liver. It is thus the drainage tract of the entire splanchnic circulation. Under normal condition, hepatic venous blood is about two-thirds saturated with oxygen and the liver accounts for some 20% of the total oxygen consumption of the body (7). So when the hepatic veins drain into the systemic circulation, the arterial oxygen saturation is expected to drop to 93-94%. Our patient had similar saturation recordings postoperatively. In patients having single ventricle physiology, excluding the hepatic venous effluent from the pulmonary circulation may cause pulmonary arteriovenous malformations to develop after cavapulmonary shunt. Patients with left atrial isomerism, especially those who undergo the Kawashima operation with an interrupted inferior vena cava and azygous continuation may have rapid development of pulmonary arteriovenous malformations, incidence of which has been reported to be as high as 58% within 5 years of operation (8). The pathophysiology of pulmonary arteriovenous malformations is not well understood. Absence of pulsatile blood flow has also been implicated in the development of pulmonary arteriovenous malformations. It is not yet known whether excluding the hepatic effluent from the pulmonary circulation after a biventricular repair will cause pulmonary arteriovenous malformation in the log term. An intraatrial baffle for anomalous systemic venous return without hepatic venous drainage is a simple and an effective solution in complex cardiac anomalies with small atria because it avoids crowding of the long intraatrial baffles for systemic and hepatic veins. Slightly lower oxygen saturation may cause dyspnea on exertion as our patient becomes physically more active. Pulmonary arteriovenous malformations may be another consequence in the long term. If the patient becomes symptomatic or more cyanotic the enlarged atrium created in the first operation may facilitate easier rerouting of the hepatic veins to the pulmonary circulation in a following operation. References Lim HG, Bacha EA, Marx GR, Marshall A, Fynn-Thompson F, Mayer JE, Del Nido P, Pigula FA. Biventricular repair in patients with heterotaxy syndrome. J Thorac Cardiovasc Surg. 2009;137:371-9. Palacios-Macedo AX, Fraser CD Jr. Correction of anomalous systemic venous drainage in heterotaxy syndrome. Ann Thorac Surg. 1997;64:235-7. Zimand S, Benjamin P, Frand M, Mishaly D, Smolinsky AK, Hegesh J. Left superior vena cava to the left atrium: do we have to change the traditional approach? Ann Thorac Surg. 1999;68:1869-71. Vargas FJ. Reconstructive methods for anomalous systemic venous return: surgical management of persistent left superior vena cava. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2008;11:31-8. Trusler GA, Williams WG, Duncan KF, Hesslein PS, Benson LN, Freedom RM, Izukawa T, Olley PM. Results with the Mustard operation in simple transposition of the great arteries 1963-1985. Ann Surg. 1987;206:251-60. Hörer J, Herrmann F, Schreiber C, Cleuziou J, Prodan Z, Vogt M, Holper K, Lange R. How well are patients doing up to 30 years after a mustard operation? Thorac Cardiovasc Surg. 2007;55:359-64. Mathie RT, Wheatly AM, Blumgart LH. Liver blood flow: physiology, measurement and clinical relevance. In: Blumgart LH, eds. Surgery of the liver and biliary tract. 2nd ed London:Churchill Livingstone;1994:95-110 .
- Introduction Outcome after biventricular repair of atrial isomerism associated with complex cardiac anomalies is improved with the advancements in preoperative, anesthetic, surgical, and postoperative management (1). However the complex malformations and anomalous venous drainage with heterotaxy syndromes may pose a significant surgical challenge (2). Several different approaches have been used to correct the anomalous drainage of the left superior vena cava (LSVC) to the left atrium. These include extracardiac reimplantation techniques, intraatrial baffles, simple ligations, and cavopulmonary anastomoses (2). In a small heart, where the orifices of the systemic and pulmonary veins are very close to each other, the creation of an intraatrial baffle may be very difficult and may lead to early baffle stenosis. Extracardiac techniques may frequently be inapplicable. In these circumstances uncommon techniques may need to be experienced. In this report, we present an infant with dextrocardia, anomalous systemic venous return, left atrial isomerism, partial atrioventricular septal defect, common atrium, and muscular ventricular septal defects (VSD), who underwent an intraatrial baffle repair of anomalous systemic venous return excluding hepatic venous drainage. Comment Many patients with heterotaxy syndromes will require a Fontan operation due to the complexity of the anatomy, while only a subset of patients will be good candidates for biventricular repair (1). The systemic venous system manifests a wide spectrum of structural abnormalities in these patients, and may significantly alter the surgical management of specific lesions. Principles of repair include the division of any extracardiac communication and intracardiac septation of the venous return from pulmonary and systemic veins. Intracardiac and extracardiac repair have been used to correct the left superior vena caval drainage to the left AV valve with no connecting vein (4). The intracardiac approach, however, may be technically difficult in a small atrium where the pulmonary and systemic vein orifices are close to each other. Baffle complications including p ulmonary and the systemic venous obstruction as a late complication after atrial switch operation has been reported as 25% (5). This rate is expected to be much higher in isomeric hearts, especially when this operation is performed early and in a small atrium. Since early baffle obstruction is the major concern, extracardiac repair is more suitable in these patients whenever possible. In our case, extracardiac repair was not possible because the posterior position of the right-sided appendage precluded division of the LSVC and its anastamosis to the right sided atrial appendage. Simple ligation was not an option either, since the patient had single SVC. LSVC to left pulmonary artery anastamosis could not be performed due to high mean pulmonary artery pressure. Therefore, intracardiac repair was preferred despite a small common atrium and close proximity of the pulmonary and systemic vein orifices. Including the hepatic veins in the systemic venous baffle would create a risk of baffle obstruction and pulmonary vein stenosis, hence the hepatic veins were left to drain into systemic circulation along with the pulmonary veins. Autologous pericardial patch is known to shrink in the long term after Mustard type repair and baffle obstructions are common (6). Therefore a patch of bovine pericardium was used to create the intraatrial baffle and to augment the atrium from the atriotomy site. The hepatic venous system is the final common pathway of hepatic arterial and portal venous blood after sinusoidal mixing in normal liver. It is thus the drainage tract of the entire splanchnic circulation. Under normal condition, hepatic venous blood is about two-thirds saturated with oxygen and the liver accounts for some 20% of the total oxygen consumption of the body (7). So when the hepatic veins drain into the systemic circulation, the arterial oxygen saturation is expected to drop to 93-94%. Our patient had similar saturation recordings postoperatively. In patients having single ventricle physiology, excluding the hepatic venous effluent from the pulmonary circulation may cause pulmonary arteriovenous malformations to develop after cavapulmonary shunt. Patients with left atrial isomerism, especially those who undergo the Kawashima operation with an interrupted inferior vena cava and azygous continuation may have rapid development of pulmonary arteriovenous malformations, incidence of which has been reported to be as high as 58% within 5 years of operation (8). The pathophysiology of pulmonary arteriovenous malformations is not well understood. Absence of pulsatile blood flow has also been implicated in the development of pulmonary arteriovenous malformations. It is not yet known whether excluding the hepatic effluent from the pulmonary circulation after a biventricular repair will cause pulmonary arteriovenous malformation in the log term. An intraatrial baffle for anomalous systemic venous return without hepatic venous drainage is a simple and an effective solution in complex cardiac anomalies with small atria because it avoids crowding of the long intraatrial baffles for systemic and hepatic veins. Slightly lower oxygen saturation may cause dyspnea on exertion as our patient becomes physically more active. Pulmonary arteriovenous malformations may be another consequence in the long term. If the patient becomes symptomatic or more cyanotic the enlarged atrium created in the first operation may facilitate easier rerouting of the hepatic veins to the pulmonary circulation in a following operation. References Lim HG, Bacha EA, Marx GR, Marshall A, Fynn-Thompson F, Mayer JE, Del Nido P, Pigula FA. Biventricular repair in patients with heterotaxy syndrome. J Thorac Cardiovasc Surg. 2009;137:371-9. Palacios-Macedo AX, Fraser CD Jr. Correction of anomalous systemic venous drainage in heterotaxy syndrome. Ann Thorac Surg. 1997;64:235-7. Zimand S, Benjamin P, Frand M, Mishaly D, Smolinsky AK, Hegesh J. Left superior vena cava to the left atrium: do we have to change the traditional approach? Ann Thorac Surg. 1999;68:1869-71. Vargas FJ. Reconstructive methods for anomalous systemic venous return: surgical management of persistent left superior vena cava. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2008;11:31-8. Trusler GA, Williams WG, Duncan KF, Hesslein PS, Benson LN, Freedom RM, Izukawa T, Olley PM. Results with the Mustard operation in simple transposition of the great arteries 1963-1985. Ann Surg. 1987;206:251-60. Hörer J, Herrmann F, Schreiber C, Cleuziou J, Prodan Z, Vogt M, Holper K, Lange R. How well are patients doing up to 30 years after a mustard operation? Thorac Cardiovasc Surg. 2007;55:359-64. Mathie RT, Wheatly AM, Blumgart LH. Liver blood flow: physiology, measurement and clinical relevance. In: Blumgart LH, eds. Surgery of the liver and biliary tract. 2nd ed London:Churchill Livingstone;1994:95-110 .
- Introduction Outcome after biventricular repair of atrial isomerism associated with complex cardiac anomalies is improved with the advancements in preoperative, anesthetic, surgical, and postoperative management (1). However the complex malformations and anomalous venous drainage with heterotaxy syndromes may pose a significant surgical challenge (2). Several different approaches have been used to correct the anomalous drainage of the left superior vena cava (LSVC) to the left atrium. These include extracardiac reimplantation techniques, intraatrial baffles, simple ligations, and cavopulmonary anastomoses (2). In a small heart, where the orifices of the systemic and pulmonary veins are very close to each other, the creation of an intraatrial baffle may be very difficult and may lead to early baffle stenosis. Extracardiac techniques may frequently be inapplicable. In these circumstances uncommon techniques may need to be experienced. In this report, we present an infant with dextrocardia, anomalous systemic venous return, left atrial isomerism, partial atrioventricular septal defect, common atrium, and muscular ventricular septal defects (VSD), who underwent an intraatrial baffle repair of anomalous systemic venous return excluding hepatic venous drainage. Comment Many patients with heterotaxy syndromes will require a Fontan operation due to the complexity of the anatomy, while only a subset of patients will be good candidates for biventricular repair (1). The systemic venous system manifests a wide spectrum of structural abnormalities in these patients, and may significantly alter the surgical management of specific lesions. Principles of repair include the division of any extracardiac communication and intracardiac septation of the venous return from pulmonary and systemic veins. Intracardiac and extracardiac repair have been used to correct the left superior vena caval drainage to the left AV valve with no connecting vein (4). The intracardiac approach, however, may be technically difficult in a small atrium where the pulmonary and systemic vein orifices are close to each other. Baffle complications including p ulmonary and the systemic venous obstruction as a late complication after atrial switch operation has been reported as 25% (5). This rate is expected to be much higher in isomeric hearts, especially when this operation is performed early and in a small atrium. Since early baffle obstruction is the major concern, extracardiac repair is more suitable in these patients whenever possible. In our case, extracardiac repair was not possible because the posterior position of the right-sided appendage precluded division of the LSVC and its anastamosis to the right sided atrial appendage. Simple ligation was not an option either, since the patient had single SVC. LSVC to left pulmonary artery anastamosis could not be performed due to high mean pulmonary artery pressure. Therefore, intracardiac repair was preferred despite a small common atrium and close proximity of the pulmonary and systemic vein orifices. Including the hepatic veins in the systemic venous baffle would create a risk of baffle obstruction and pulmonary vein stenosis, hence the hepatic veins were left to drain into systemic circulation along with the pulmonary veins. Autologous pericardial patch is known to shrink in the long term after Mustard type repair and baffle obstructions are common (6). Therefore a patch of bovine pericardium was used to create the intraatrial baffle and to augment the atrium from the atriotomy site. The hepatic venous system is the final common pathway of hepatic arterial and portal venous blood after sinusoidal mixing in normal liver. It is thus the drainage tract of the entire splanchnic circulation. Under normal condition, hepatic venous blood is about two-thirds saturated with oxygen and the liver accounts for some 20% of the total oxygen consumption of the body (7). So when the hepatic veins drain into the systemic circulation, the arterial oxygen saturation is expected to drop to 93-94%. Our patient had similar saturation recordings postoperatively. In patients having single ventricle physiology, excluding the hepatic venous effluent from the pulmonary circulation may cause pulmonary arteriovenous malformations to develop after cavapulmonary shunt. Patients with left atrial isomerism, especially those who undergo the Kawashima operation with an interrupted inferior vena cava and azygous continuation may have rapid development of pulmonary arteriovenous malformations, incidence of which has been reported to be as high as 58% within 5 years of operation (8). The pathophysiology of pulmonary arteriovenous malformations is not well understood. Absence of pulsatile blood flow has also been implicated in the development of pulmonary arteriovenous malformations. It is not yet known whether excluding the hepatic effluent from the pulmonary circulation after a biventricular repair will cause pulmonary arteriovenous malformation in the log term. An intraatrial baffle for anomalous systemic venous return without hepatic venous drainage is a simple and an effective solution in complex cardiac anomalies with small atria because it avoids crowding of the long intraatrial baffles for systemic and hepatic veins. Slightly lower oxygen saturation may cause dyspnea on exertion as our patient becomes physically more active. Pulmonary arteriovenous malformations may be another consequence in the long term. If the patient becomes symptomatic or more cyanotic the enlarged atrium created in the first operation may facilitate easier rerouting of the hepatic veins to the pulmonary circulation in a following operation. References Lim HG, Bacha EA, Marx GR, Marshall A, Fynn-Thompson F, Mayer JE, Del Nido P, Pigula FA. Biventricular repair in patients with heterotaxy syndrome. J Thorac Cardiovasc Surg. 2009;137:371-9. Palacios-Macedo AX, Fraser CD Jr. Correction of anomalous systemic venous drainage in heterotaxy syndrome. Ann Thorac Surg. 1997;64:235-7. Zimand S, Benjamin P, Frand M, Mishaly D, Smolinsky AK, Hegesh J. Left superior vena cava to the left atrium: do we have to change the traditional approach? Ann Thorac Surg. 1999;68:1869-71. Vargas FJ. Reconstructive methods for anomalous systemic venous return: surgical management of persistent left superior vena cava. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2008;11:31-8. Trusler GA, Williams WG, Duncan KF, Hesslein PS, Benson LN, Freedom RM, Izukawa T, Olley PM. Results with the Mustard operation in simple transposition of the great arteries 1963-1985. Ann Surg. 1987;206:251-60. Hörer J, Herrmann F, Schreiber C, Cleuziou J, Prodan Z, Vogt M, Holper K, Lange R. How well are patients doing up to 30 years after a mustard operation? Thorac Cardiovasc Surg. 2007;55:359-64. Mathie RT, Wheatly AM, Blumgart LH. Liver blood flow: physiology, measurement and clinical relevance. In: Blumgart LH, eds. Surgery of the liver and biliary tract. 2nd ed London:Churchill Livingstone;1994:95-110 .
- Rezeksiyon BVF yanında kas hipertrofisi ve AV kapak yapılarına bağlıda oluştuğundan rezeksiyonu zor ve komplikasyonludur.
- Glenn ve fontan sonrası strok oluşumu ile cerrahi teknik arasında bir ilişki var.
- Bu darlık erken dönemde oluşabileceği gibi BDCPS veya Fontan sonrasıda oluşabilir. Fontan sonrası ventrikül volumunun azalması ile LVOT darlığın gradyentinin daha da arttığı bildirilmiştir. Arzu ederseniz Canan Hanım size preoperatif ve per operatif TEE gösterebilir.