Geç simple TGA olgusunda LV destek cihazı ile başarılı arteriyel Switch operasyonu
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Geç simple TGA olgusunda LV destek cihazı ile başarılı arteriyel Switch operasyonu
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Semelhante a Geç simple TGA olgusunda LV destek cihazı ile başarılı arteriyel Switch operasyonu
Semelhante a Geç simple TGA olgusunda LV destek cihazı ile başarılı arteriyel Switch operasyonu (20)
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Geç simple TGA olgusunda LV destek cihazı ile başarılı arteriyel Switch operasyonu
- 1. Geç simple TGA olgusunda LV destek cihazı ile başarılı arteriyel Switch operasyonu Rıza Türköz, Can Vuran, O uz Omay,ğ Canan Ayabakan, Kür ad Tokelş
- 2. VakaVaka 19 günlük kız19 günlük kız çocu uğçocu uğ (40 hf, 3200 gr)(40 hf, 3200 gr) 2 günlük başka merkezde kan alınırken2 günlük başka merkezde kan alınırken siyanozsiyanoz Fizik muayeneFizik muayene SiyanozSiyanoz SSol 3.ol 3. IKAIKA 11/6 sistolik üfürüm/6 sistolik üfürüm OlguOlgu
- 3. Tanı: EkokardiEkokardiyyografiografi TGATGA ASD (küçük, 4 mm)ASD (küçük, 4 mm) TY (hafif)TY (hafif)
- 4. Tanı: Kateter veKateter ve anjioanjio TGATGA ASDASD BAS (sat %58---%84)BAS (sat %58---%84) LA= 7 mmHg, RA=7 mmHg,LA= 7 mmHg, RA=7 mmHg, LV=23/0-9 mmHg,LV=23/0-9 mmHg, RRV=85/0-10V=85/0-10 mmHg, Ao= 62/36 (47)mmHg, Ao= 62/36 (47)
- 5. Tanı: Kateter veKateter ve anjioanjio Anjio göruntusu ???Anjio göruntusu ???
- 6. OperasyonOperasyon Standart aort ve bikavalStandart aort ve bikaval kanükasyon, KPB, PDA divizyonu vekanükasyon, KPB, PDA divizyonu ve arrest sa landığarrest sa landığ Koroner arter anomalisi yoktuKoroner arter anomalisi yoktu Aorta transekte , LAorta transekte , LCxCx ve RCAve RCA buttonbuttonuu çıkarıldıçıkarıldı PA transekte edildi. LeCompte manevrasıPA transekte edildi. LeCompte manevrası yapıldıyapıldı Neoaorta anastomozu sonrası Edward BoveNeoaorta anastomozu sonrası Edward Bove tekniği ile koroner butonlar implante edildi.tekniği ile koroner butonlar implante edildi.
- 7. Operasyon bulgularıOperasyon bulguları Anastomozların tamamlanmasından sonra koronerAnastomozların tamamlanmasından sonra koroner istemisi yoktu.istemisi yoktu. Pompadan çıkılamadıPompadan çıkılamadı Yaklaşık 1 saat pompada CPB miyokard dinlendirildi.Yaklaşık 1 saat pompada CPB miyokard dinlendirildi. LA-Ao santrifugal pompa yerleştirildiLA-Ao santrifugal pompa yerleştirildi LAP ve periton diyaliz kateteri yerleştirildi.LAP ve periton diyaliz kateteri yerleştirildi. Cilt ve sternum açık olarak YBCilt ve sternum açık olarak YB
- 8. 3’lü inotrop verildi3’lü inotrop verildi AdrenalinAdrenalin DopaminDopamin MilrinonMilrinon 5 gün LVAD kalındı5 gün LVAD kalındı Postoperatif 1 gün hipotansiyon ilePostoperatif 1 gün hipotansiyon ile birlikte arrest gelişti.birlikte arrest gelişti. YB açıldı ve yaklaşık 20 dk CPR yapıldı.YB açıldı ve yaklaşık 20 dk CPR yapıldı. LVAD akımı kademeli olarak düşüldü.LVAD akımı kademeli olarak düşüldü. Postop 7 gün dekanüle edilerek. SternumPostop 7 gün dekanüle edilerek. Sternum ve cilt kapatıldı.ve cilt kapatıldı. Yoğun BakımYoğun Bakım bulgularıbulguları
- 9. BY Postop 12 günde düzeldi.BY Postop 12 günde düzeldi. Postoperatif 20 gün ekstübe edildi.Postoperatif 20 gün ekstübe edildi. Cilt açıklığı…………………Cilt açıklığı………………… Yoğun BakımYoğun Bakım bulgularıbulguları
- 10. VSD’ler LV tarafından kapatıldı.VSD’ler LV tarafından kapatıldı. Kleft kapatıldı.Kleft kapatıldı. Küçük tek atrium ve hepatikKüçük tek atrium ve hepatik venlerin PV yakınlı ı hepatikğvenlerin PV yakınlı ı hepatikğ venlerin sa AV kapa ağ ğvenlerin sa AV kapa ağ ğ yyöönlendirilmesini engelliyordu.nlendirilmesini engelliyordu. LSVC (hemiazigos) sı ır perikardığLSVC (hemiazigos) sı ır perikardığ ile sa AV kapa a yönlendirildi.ğ ğile sa AV kapa a yönlendirildi.ğ ğ Atriotomi sı ır perikardı peçiğAtriotomi sı ır perikardı peçiğ kullanıp geni letilerek kapatıldı.şkullanıp geni letilerek kapatıldı.ş Operasyon bulgularıOperasyon bulguları
- 13. OperasyonOperasyon Aortik klemp : dkAortik klemp : dk KPB : dkKPB : dk Blok ve pacemakerBlok ve pacemaker LAP 9-10 mmHg ve Ao 60-65 mmHgLAP 9-10 mmHg ve Ao 60-65 mmHg Periton diyaliz kateteriPeriton diyaliz kateteri notropİnotropİ Milrinon 0.5Milrinon 0.5 µµg/kg/dkg/kg/dk Dopamin 5Dopamin 5 µµg/kg/dkg/kg/dk Adrenalin 0.05Adrenalin 0.05 µµg/kg/dkg/kg/dk
- 14. PostoperatifPostoperatif Postoperatif . gün ekstübePostoperatif . gün ekstübe Postoperatif . gün servisPostoperatif . gün servis Postoperatif 9. gün taburcuPostoperatif 9. gün taburcu edildi.edildi. Postoperatif O2 sat %93-98Postoperatif O2 sat %93-98 arasında de i iyordu.ğ şarasında de i iyordu.ğ ş
- 15. zlemİzlemİ KontKontrol Ekokardiografisinde Küçük rezüdüv VSD Buffle gradiyent yok LAV kapakta minimal yetmezlikLAV kapakta minimal yetmezlik Olguların izlemiOlguların izlemi fonksiyonel kapasite iyifonksiyonel kapasite iyi
- 16. TartısmaTartısma zomerik olgularda tedavisiİzomerik olgularda tedavisiİ Fontan operasyonuFontan operasyonu Biventriküler tamirBiventriküler tamir Ekstrakardiak ve intrakardiak ili kilerinşEkstrakardiak ve intrakardiak ili kilerinş septasyonuseptasyonu
- 17. TartısmaTartısma LA anormal sistemik venöz dönü ünşLA anormal sistemik venöz dönü ünş düzeltilmesindedüzeltilmesinde Ekstrakardiak reimplantasyonEkstrakardiak reimplantasyon ntraatrial baffleİntraatrial baffleİ Basit ligasyonBasit ligasyon Cava-pulmoner anastomozCava-pulmoner anastomoz
- 18. TartısmaTartısma ntraatrial buffle sonrasıİntraatrial buffle sonrasıİ obstruksiyon erken dönem mortalitesiobstruksiyon erken dönem mortalitesi dı ında %25 bildirilmi tirş şdı ında %25 bildirilmi tirş ş 11 .. somerik, erken ya ve küçük atriumdaİ şsomerik, erken ya ve küçük atriumdaİ ş oran çok daha fazlaoran çok daha fazla 1 Trusler GA. Results with the Mustard operation in simple transposition of the great arteries 1963-1985. Ann Surg. 1987;206:251-60.
- 19. TartısmaTartısma Hepatik venöz akım sat %65-70 veHepatik venöz akım sat %65-70 ve KCKC vucudun total O2 tüketiminin %20 sini alırvucudun total O2 tüketiminin %20 sini alır11 .. Hepatik ven sistemik dolaşıma dreneHepatik ven sistemik dolaşıma drene olduğunda Arteriel O2 sat %93-94 olmasıolduğunda Arteriel O2 sat %93-94 olması beklenir.beklenir. 1 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.
- 20. SonuçSonuç Küçük atriumlu kompleks kardiak anomalilerdeKüçük atriumlu kompleks kardiak anomalilerde hepatik venoz drenaj olmaksızın anormal SVDhepatik venoz drenaj olmaksızın anormal SVD düzeltilmesi basit ve etkin bir yöntem olabilir.düzeltilmesi basit ve etkin bir yöntem olabilir. AncakAncak düşük O2 saturasyonu daha ileri yaştadüşük O2 saturasyonu daha ileri yaşta eforu kısıtlaması problem olabilir.eforu kısıtlaması problem olabilir. Biventriküler tamir sonrası PAVM gelişmesi ?Biventriküler tamir sonrası PAVM gelişmesi ? Olgu semptomatik olursa ileri yaşta dahaOlgu semptomatik olursa ileri yaşta daha kolaylıkla düzeltilebilir.kolaylıkla düzeltilebilir.
Notas do Editor
- Intraatrial Baffle Repair of Anomalous Systemic Venous Return Excluding Hepatic Venous Drainage in Heterotaxy Syndrome Running Head: Repair of Anomalous Systemic Venous Return in Heterotaxy Syndrome Rıza Turkoz MD1 Canan Ayabakan MD2 Can Vuran MD1 Oğuz Omay MD1 Uygar Yoruker MD1 Kurşad Tokel MD2 Baskent University, İstanbul Teaching and Medical Research Center, Department of Cardiovascular Surgery1, Department of Pediatric Cardiology2, İstanbul/Turkey Key words: CHD, heterotaxy, atrium, Word count: 1327 Corresponding author: Rıza Turkoz MD Baskent Universitesi İstanbul Uygulama ve Araştırma Hastanesi Altunizade Istanbul / Turkey Tel: +90 216 5541500-2001 Fax: +90 216 6519858 E-mail: [email_address] Abstract A 7-month-old patient with heterotaxy syndrome had partial atrioventricular septal defect and interrupted inferior vena cava with hemiazygos continuation to a left superior vena cava. The left-side of the common atrium, receiving all the venous drainage, was in connection with the left ventricle and the aorta. The small atrium and the proximity of the pulmonary and hepatic vein orifices precluded complete baffling. We describe an intraatrial baffle repair of anomalous systemic venous return excluding hepatic venous drainage. This resulted in good oxygenation postoperatively, with oxygen saturation ranging from 93% to 98%. 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 . Brown JW, Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW. Pulmonary arteriovenous malformations in children after the Kawashima operation. Ann Thorac Surg. 2005;80:1592-6. Fig 1. The schematic drawing of the heart preoperatively: The case had left atrial isomerism with common atrium. Left superior vena cava receiving the hemiazygos vein, all pulmonary veins and the hepatic vein drained into the left side of the atrium. LSVC= left superior vena cava; HA= hemiazygos vein; PV= pulmonary veins; HV= hepatic vein; LV= Left ventricle; RV= right ventricle Fig 2. The schematic drawing of the heart after intraatrial baffle placement: A patch of bovine pericardium is sutured around the orifice of LSVC to route the hemiazygous and the superior vena caval effluent to the right AV valve. The hepatic vein and the pulmonary veins are left to drain through the left AV valve. IB= intraatrial baffle; LSVC= left superior vena cava; HA= hemiazygos vein; PV= pulmonary veins; HV= hepatic vein; LV= Left ventricle; RV= right ventricle
- Case Report A 7-month-old male baby, weighing 6.2 kg, presented to the hospital with dyspnea and failure to thrive. Cardiovascular examination revealed a right-sided apical impulse and a grade II/IV pansystolic murmur at the right lower sternal border. Chest roentgenogram showed dextrocardia and cardiomegaly.
- Echocardiographic study revealed dextrocardia, left atrial isomerism, partial atrioventricular septal defect (PASVD) with common atrium and two small muscular VSD’s. He had interrupted inferior vena cava, persistence of the hemiazygos vein draining into the left side of the common atrium via LSVC. There was no coronary sinus or right superior vena cava. The pulmonary veins and the hepatic veins drained directly to the left side of the common atrium.
- Cardiac catheterization confirmed the diagnosis and detailed the anomalous venous return.
- Cardiac catheterization confirmed the diagnosis and detailed the anomalous venous return.
- At surgery both appendages had the left atrial appendage morphology and were not juxtaposed. Left side of the common atrium was anterior, whereas the right side of the atrium was situated posterior. The aorta was posterior and to the right of the pulmonary artery.
- After standard aortic cannulation and venous cannulation through LSVC and hepatic veins, cardiopulmonary bypass was begun. The heart was arrested with blood cardioplegic solution. The operation was performed from the left side of the operating table. The left side of the common atrium was incised parallel to the atrioventricular groove. The atrial septum was completely absent and common atrium was small. LSVC, hepatic vein and all pulmonary veins drained into the left side of the atrium. The hepatic vein orifice was very close to the pulmonary venous orifices. Coronary sinus was absent. The left ventricle was anterior and to the left, the right ventricle was posterior and to the right. PAVSD with a large cleft in the left atrioventricular valve was noted. The left-side of the common atrium, receiving all the venous drainage, was in connection with the left ventricle and the aorta. The VSD’s were closed from the left side of the septum through the left AV valve and the cleft was closed with interrupted sutures. The small common atrium and the proximity of the pulmonary and hepatic vein orifices precluded baffling the hepatic vein to the right AV valve. Therefore a patch of bovine pericardium was sutured around the orifice of LSVC and posterior wall of the atrium to route the hemiazygous and the superior vena caval effluent to the right AV valve. Thus only a LSCV baffle was used; the hepatic vein and the pulmonary veins were left to drain through the left AV valve to the aorta. In order to prevent baffle obstruction and pulmonary vein stenosis, the atrium was enlarged with an ellipse shaped bovine pericardium interposed to the atriotomy site.
- After standard aortic cannulation and venous cannulation through LSVC and hepatic veins, cardiopulmonary bypass was begun. The heart was arrested with blood cardioplegic solution. The operation was performed from the left side of the operating table. The left side of the common atrium was incised parallel to the atrioventricular groove. The atrial septum was completely absent and common atrium was small. LSVC, hepatic vein and all pulmonary veins drained into the left side of the atrium. The hepatic vein orifice was very close to the pulmonary venous orifices. Coronary sinus was absent. The left ventricle was anterior and to the left, the right ventricle was posterior and to the right. PAVSD with a large cleft in the left atrioventricular valve was noted. The left-side of the common atrium, receiving all the venous drainage, was in connection with the left ventricle and the aorta. The VSD’s were closed from the left side of the septum through the left AV valve and the cleft was closed with interrupted sutures. The small common atrium and the proximity of the pulmonary and hepatic vein orifices precluded baffling the hepatic vein to the right AV valve. Therefore a patch of bovine pericardium was sutured around the orifice of LSVC and posterior wall of the atrium to route the hemiazygous and the superior vena caval effluent to the right AV valve. Thus only a LSCV baffle was used; the hepatic vein and the pulmonary veins were left to drain through the left AV valve to the aorta. In order to prevent baffle obstruction and pulmonary vein stenosis, the atrium was enlarged with an ellipse shaped bovine pericardium interposed to the atriotomy site.
- After standard aortic cannulation and venous cannulation through LSVC and hepatic veins, cardiopulmonary bypass was begun. The heart was arrested with blood cardioplegic solution. The operation was performed from the left side of the operating table. The left side of the common atrium was incised parallel to the atrioventricular groove. The atrial septum was completely absent and common atrium was small. LSVC, hepatic vein and all pulmonary veins drained into the left side of the atrium. The hepatic vein orifice was very close to the pulmonary venous orifices. Coronary sinus was absent. The left ventricle was anterior and to the left, the right ventricle was posterior and to the right. PAVSD with a large cleft in the left atrioventricular valve was noted. The left-side of the common atrium, receiving all the venous drainage, was in connection with the left ventricle and the aorta. The VSD’s were closed from the left side of the septum through the left AV valve and the cleft was closed with interrupted sutures. The small common atrium and the proximity of the pulmonary and hepatic vein orifices precluded baffling the hepatic vein to the right AV valve. Therefore a patch of bovine pericardium was sutured around the orifice of LSVC and posterior wall of the atrium to route the hemiazygous and the superior vena caval effluent to the right AV valve. Thus only a LSCV baffle was used; the hepatic vein and the pulmonary veins were left to drain through the left AV valve to the aorta. In order to prevent baffle obstruction and pulmonary vein stenosis, the atrium was enlarged with an ellipse shaped bovine pericardium interposed to the atriotomy site.
- The patient underwent an uneventful postoperative course, and the oxygen saturation ranged from 93% to 98%. Postoperative echocardiography demonstrated mild regurgitation in the left atrioventricular valve, small residual muscular ventricular septal defect, no baffle obstruction and good ventricular function. The patient was discharged 9 days after surgery.
- The patient underwent an uneventful postoperative course, and the oxygen saturation ranged from 93% to 98%. Postoperative echocardiography demonstrated mild regurgitation in the left atrioventricular valve, small residual muscular ventricular septal defect, no baffle obstruction and good ventricular function. The patient was discharged 9 days after surgery.
- 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 .
- 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.