Hetorotaksi sendromu olan bir olguda hepatik venoz drenajı dahil etmeden anormal sistemik venoz dönüşün düzeltilmesi
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Hetorotaksi sendromu olan bir olguda hepatik venoz drenajı dahil etmeden anormal sistemik venoz dönüşün düzeltilmesi
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Hetorotaksi sendromu olan bir olguda hepatik venoz drenajı dahil etmeden anormal sistemik venoz dönüşün düzeltilmesi
- 1. Hetorotaksi sendromu olan bir olguda hepatik venoz drenajı dahil etmeden anormal sistemik venoz dönü ünş düzeltilmesiRıza Türköz, Can Vuran, O uz Omay,ğ Canan Ayabakan, Kür ad Tokelş
- 2. Vaka 1Vaka 1 77 aylıkaylık erkekerkek çocu uğçocu uğ Nefes darlığı ve gelişme geriliğiNefes darlığı ve gelişme geriliği Fizik muayeneFizik muayene Kalbin apeksine bağlı sağ tarafta vurum.Kalbin apeksine bağlı sağ tarafta vurum. SiyanozSiyanoz SSağ 3.4.ağ 3.4. IKA 2/6 sistolik üfürümIKA 2/6 sistolik üfürüm AC de krepitan ralAC de krepitan ral Vaka 2Vaka 2 3 aylık diabetik anne çocu uğ3 aylık diabetik anne çocu uğ Pnomoni tedavisiPnomoni tedavisi morarma, solunum sıkıntısı YD döneminde sarılıkmorarma, solunum sıkıntısı YD döneminde sarılık veve morarmamorarma 27 gün anjio ve BAS27 gün anjio ve BAS Fizik muayeneFizik muayene SiyanozSiyanoz Sol 2. IKA 3/6 sistolik üfürümSol 2. IKA 3/6 sistolik üfürüm OlguOlgu
- 3. Tanı: EkokardiEkokardiyyografiografi Vaka 1Vaka 1 d-TGAd-TGA VSDVSD musküler-outlet, çok küçükmusküler-outlet, çok küçük:: 2 mm2 mm LVOT’de darlıkLVOT’de darlık septumda posterior malaligment, 58 mmHgseptumda posterior malaligment, 58 mmHg ASD (BAS ba lı)ğASD (BAS ba lı)ğ PDA (çok ince)PDA (çok ince) Vaka 2Vaka 2 d-TGAd-TGA VSDVSD trabekuler-outlet, çok küçüktrabekuler-outlet, çok küçük:: 2.5 mm2.5 mm LVOT’de darlıkLVOT’de darlık septumda posterior malaligment, 65 mmHgseptumda posterior malaligment, 65 mmHg Foromen ovaleForomen ovale Bikuspid pulmoner kapak (stenoz yok)Bikuspid pulmoner kapak (stenoz yok)
- 4. Tanı: Kateter veKateter ve anjioanjio Vaka 1Vaka 1 LA= 7 mmHg, RA=8 mmHg,LA= 7 mmHg, RA=8 mmHg, LV=83/0-8 mmHg, RV=94/0-9 mmHg, Ao= 75/40LV=83/0-8 mmHg, RV=94/0-9 mmHg, Ao= 75/40 mmHgmmHg Aort Satürasyon %54Aort Satürasyon %54 Koroner anjiografiKoroner anjiografi:: normal koroner arterlernormal koroner arterler Vaka 2Vaka 2 LA= 12 mmHg, RA=7 mmHg,LA= 12 mmHg, RA=7 mmHg, LV=64/0-7 mmHg, RV=81/0-10 mmHg, Ao= 70/40LV=64/0-7 mmHg, RV=81/0-10 mmHg, Ao= 70/40 mmHgmmHg Aort Satürasyon %40Aort Satürasyon %40 Koroner anjiografiKoroner anjiografi:: normal koroner arterlernormal koroner arterler
- 5. OperasyonOperasyon Standart aort ve bikaval kanükasyon, KPB,Standart aort ve bikaval kanükasyon, KPB, PDA divizyonu ve arrest sa landığPDA divizyonu ve arrest sa landığ Aorta transekte , L button sirküler çıkarıldıAorta transekte , L button sirküler çıkarıldı Aort kapa ın sol yarısı RV’denğAort kapa ın sol yarısı RV’denğ serbestle tirildi (Ross gibi)şserbestle tirildi (Ross gibi)ş LVOT,LVOT, VSD’den pulmoner kapa a kadar açıldığVSD’den pulmoner kapa a kadar açıldığ ,, Otojen perikard ile Mod Konno yapıldı,Otojen perikard ile Mod Konno yapıldı, Aort kapak tekrar yerine dikildiAort kapak tekrar yerine dikildi Edward Bove tekni i ile arterial switchğEdward Bove tekni i ile arterial switchğ yapıldı.yapıldı.
- 9. OperasyonOperasyon Aortik klemp : 145, 90 dkAortik klemp : 145, 90 dk KPB : 232, 186 dkKPB : 232, 186 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
- 10. PostoperatifPostoperatif Postoperatif 1.- 2. gün ekstübePostoperatif 1.- 2. gün ekstübe Postoperatif 3. - 5. gün servisPostoperatif 3. - 5. gün servis Postoperatif 10. gün taburcuPostoperatif 10. gün taburcu edildiedildi
- 11. zlemİzlemİ KontKontrol Ekokardiografisinde LVOT gradiyent yok RVOT gradiyent yok Olguların izlemiOlguların izlemi fonksiyonel kapasite iyifonksiyonel kapasite iyi
- 13. TartısmaTartısma
- 15. TartısmaTartısma LVOTO tedavisiLVOTO tedavisi Müdahale edilmedenMüdahale edilmeden Transpulmoner rezeksiyonTranspulmoner rezeksiyon RA yoluyla rezeksiyonRA yoluyla rezeksiyon Konno prosedürüKonno prosedürü**
- 18. SonuçSonuç LVOTO ile birlikte TGA olan olgulardaLVOTO ile birlikte TGA olan olgularda rezeksiyon ile birlikte arterial switchrezeksiyon ile birlikte arterial switch ameliyatı ba arılı sonuç vermektedir.şameliyatı ba arılı sonuç vermektedir.ş Ancak rezeksiyonun kolay olmadı ığAncak rezeksiyonun kolay olmadı ığ posterior malalignment olgularındaposterior malalignment olgularında modifiye Konno prosedürünün arterialmodifiye Konno prosedürünün arterial switch operasyonu ile birlikteswitch operasyonu ile birlikte uygulanabilece ini dü ünüyoruz.ğ şuygulanabilece ini dü ünüyoruz.ğ ş
Editor's Notes
- 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. 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. 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.
- 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
- REV: Lecompt tarafından tanımlanan. Tüm infundibular septumun rezeksiyonu ile conduit kullanuılmadan direct olarak RV-PA rekonstrüksiyonudur.
- 10 vakalık bir seride arterial switch takibinde LVOTO görülüyor ve çeşitli müdahaleler yapılıyor. 2 sinde VSD peçinin genişletilmesi uygulanıyor ve birinde AY gelişincce AVR ile birlikte KOnno prosedürü uygulanıyor.
- Bizimde arterial switch ile birlikte rezeksiyon yaptığımız 4 olgu mevcut.