Treatment of High Output Cardiac Failure by Flow-Adapted Hepatic Artery Banding (FHAB) in Patients with Hereditary Hemorrhagic Telangiectasia

Involvement of abdominal organs in Osler’s disease may lead to the development of hepatic arteriovenous shunts with a dilatation of the hepatic artery. Right and subsequent global heart failure due to cardiac valvular insufficiency, pulmonary artery hypertension, and hepatomegaly as well as increased cardiac output may result. This hyperdynamic hepatic blood flow can be reduced by ligature or banding of the hepatic artery or by orthotopic liver transplantation. We report on two female patients suffering from Osler’s disease (68 and 76 years old) with severe heart insufficiency (NYHA III-IV) caused by the high hepatic shunt volumes. A gradual banding of the hepatic artery directed by intraoperative flow measurement in the hepatic artery and control of the systemic hemodynamics by Swan–Ganz or COLD catheters was performed in these patients. The banding was achieved by encasing the hepatic artery in a PTFE cuff (length, 1.0 cm). The high cardiac output could be reduced from 11.2 to 7.0 l/min and from 10.7 to 6.0 l/min, respectively. The respective hepatic artery flow was reduced from 2.0 to 0.3 l/min and from 4.0 to 0.7 l/min. An improvement of heart insufficiency, a reduction in the severity of the cardiac valvular insufficiency, and a reduction of the pulmonary arterial hypertension could be already observed intraoperatively. One patient died of right cardiac failure after an orthotopic liver transplantation 7 months later. The other one died 3 years after the banding. The banding of the hepatic artery controlled by hepatic arterial flow measurement can be considered as an effective and safe palliative procedure in intrahepatic HHT compared to therapeutic alternatives such as hepatic artery ligation or embolization.     

Effective therapy for hepatic M. Osler with systemic hypercirculation by ligation of the hepatic artery and subsequent liver transplantation

Hereditary hemorrhagic teleangiectasia, or M. Osler (Osler-Weber-Rendu disease), is an autosomal dominant, systemic fibrovascular dysplasia. This may lead to increased liver blood flow from arteriovenous fistulas. A 45-year-old woman with a known M. Osler was admitted for liver transplantation. On admission, exertional dyspnea was the predominant symptom. Radiological investigations revealed multiple intrahepatic arteriovenous fistulas and consecutive high-output heart failure. Laboratory findings revealed remarkably elevated bilirubin and alkaline phosphatase. To alleviate the high-output cardiac failure, the hepatic artery was ligated. Fourteen months later, the patient presented again with increased levels of bilirubin and recurrent bleeding episodes from esophageal varices grade IV. The patient underwent liver transplantation and post-transplant recovery was excellent. A hyperdynamic circulatory state due to a hepatic M. Osler has been treated in several cases by ligation or embolization of the hepatic artery. This procedure, however, is recommended only for patients with normal liver function and carries a considerable risk of bile duct necrosis. Received: 25 November 1997 Accepted: 16 January 1998     

Successful combined liver-heart transplantation in adults: report of three patients and review of the literature

BACKGROUND: Three patients received liver/heart transplantation, and we report their successful outcome. METHODS: Two patients had alcoholic cirrhosis and dilated cardiomyopathy; one had cryptogenic liver disease and idiopathic cardiomyopathy. RESULTS: All patients had evidence of portal hypertension and coagulopathy. The cardiac transplants were performed first. Cardiopulmonary bypass was discontinued in favor of venovenous bypass, and liver transplantation was then performed. All patients developed acute tubular necrosis; two required a brief period of hemodialysis. There was only one episode of acute cellular rejection of the liver. Protocol endomyocardial biopsies in all three patients revealed no evidence of rejection. All patients are currently using low doses of immunosuppressive medications and have normal liver chemistry tests and cardiac function; two patients have mild renal insufficiency. CONCLUSION: In selected patients with severe cardiac dysfunction and advanced liver disease, liver/heart transplantation can be successfully performed even in the face of portal hypertension and coagulopathy.     

Transjugular intrahepatic portosystemic shunts: preliminary results in 25 patients.

A retrospective study of transjugular intrahepatic shunts performed between June 1990 and June 1991 is reported. Twelve patients were actively bleeding at the time of the procedure; 12 other patients had had one to five bleeding episodes within the previous 2 weeks, and one patient had massive ascites from Budd-Chiari syndrome. Most patients had severe liver disease: 21 Child's class C, three Child's class B, and one Child's class A. Transjugular intrahepatic shunting was technically successful in all cases. Portal vein pressures were reduced on average from 36 +/- 7 cm H2O to 22 +/- 6 cm H2O. Variceal bleeding ceased after transjugular intrahepatic shunting in all patients who were actively bleeding. Five patients died (30-day mortality, 20%), and eight patients subsequently underwent elective liver transplantation. The transjugular intrahepatic shunts in the 12 other patients have remained patent an average of 5.5 months. Shunt occlusion occurred in three patients at 21, 24, and 102 days, respectively. All three occlusions were successfully reopened with percutaneous techniques, yielding a primary shunt patency of 88% and secondary shunt patency of 100%. Complications included new onset encephalopathy in one patient, which cleared with medical therapy and transient renal failure in one patient. These preliminary data suggest that transjugular intrahepatic shunting is a safe and effective therapy for the short-term treatment of patients with variceal hemorrhage, particularly in patients with severe liver disease awaiting transplantation. The long-term benefit of transjugular intrahepatic shunting awaits further follow-up.     

Arterial steal: an unusual cause for hepatic hypoperfusion after liver transplantation

Case reports of two patients with an unusual cause for a rapid increase in transaminases following liver transplantation are described. In the postoperative course, angiography revealed an arterial, hypoperfusion of the liver due to a steal phenomenon with blood shunting from the hepatic to the splenic artery. In one case, the underlying pathophysiology was a pre-existing filiform stenosis of the celiac trunk with insufficient recruitment of arterial blood from the superior mesenteric artery via the pancreatic arcade. Adequate liver perfusion was restored by simple ligation of the common hepatic artery. In the other case, angiography showed an arteriovenous fistula formation of the splenic vessels and minimal blood flow through the hepatic vessels. This was successfully corrected by angiographic embolization of the splenic artery with metal coils. After therapeutic intervention, both patients rapidly recovered with excellent liver function.     

三次肝移植的临床疗效

目的 探讨三次肝移植的临床疗效.方法 回顾性分析2002年4月至2012年12月武警总医院收治的4例肝胆疾病患者的临床资料.供肝植入均采用经典原位肝移植.术后均采用他克莫司＋吗替麦考酚酯＋激素3联免疫抑制方案.通过移植随访中心随访至2014年5月,了解患者预后并进行用药指导.计量资料采用t检验.结果 4例患者中,3例良性肝脏疾病患者多次行肝移植原因主要为胆道并发症,其次为慢性排斥反应;1例肝癌患者多次肝移植原因主要为肝癌复发.4例患者第1、2次肝移植中位间隔时间为16.0个月,短于第2、3次肝移植的22.5个月.第2次肝移植手术时间为（11.4±1.0）h,第3次肝移植手术时间为（14.1±2.2）h,第2、3次肝移植手术时间比较,差异有统计学意义（t=3.644,P＜0.05）.第2、3次肝移植中位出血量分别为1 300 mL和1 800 mL,中位输血量分别为1 400mL和3 100 mL.4例患者肝移植术后早期肝功能均恢复顺利.4例患者中,有2例3例次[其中1次为术后早期（＜30 d）出现肺部铜绿假单胞菌感染]发生移植后感染,后经过积极抗感染治疗后痊愈.4例患者中,1例于第1次肝移植术后80个月死于肝衰竭;1例于第1次肝移植术后107个月死于肝癌复发合并肺、骨及腹膜后淋巴结等多处转移;另2例于第1次肝移植术后分别生存104个月和26个月.结论 三次肝移植是治疗移植肝胆道并发症及慢性排斥反应的有效方法;如有条件,三次肝移植对肝癌复发患者也能明显延长其生存时间.     

Left pulmonary artery banding to repair ipsilateral diffuse pulmonary arteriovenous fistula

ABSTRACT: Congenital pulmonary arteriovenous fistula (PAVF) is a rare disease which causes hypoxemia by shunting deoxygenated blood from the pulmonary artery into pulmonary venous return. Lung transplantation is the most effective therapy to treat severe, diffuse PAVF. However, the availability of lungs for transplantation is limited in most parts in the world. For patients with diffuse PAVF affecting only one side of the lungs, ipsilateral pulmonary artery banding (PAB) is an effective treatment, but not yet standard of care. We report successful treatment of a patient with diffuse left-sided PAVF with PAB. We believe that PAB is an effective therapy for severe unilateral PAVF and may serve as a bridge to lung transplantation.     

Cardiac dysfunction during liver transplantation: incidence and preoperative predictors

BACKGROUND: The aim was to investigate the cardiac response during liver transplantation (LT) and analyze its relationship with clinical factors, echocardiographic, and hemodynamic findings. METHODS: All patients undergoing LT for cirrhosis from 1998 to 2004 were included. Clinical data, comprehensive echocardiography, hepatic, and right heart hemodynamic measurements were analyzed. During LT patients underwent continuous right-heart pressure monitorization. Measurements 10 min after reperfusion were compared with baseline values. Abnormal cardiac response was defined as a decrease in left ventricular stroke work index despite a rise in pulmonary wedge capillary pressure. Predictors of abnormal cardiac response were investigated using logistic regression. RESULTS: Data were available from 209 patients (mean age 52 (9) yrs; Child A 27; B 93; C 89) with a mean model for end-stage liver disease score 16.3 (4.7). Abnormal cardiac response was observed in 47 (22.5%) patients after reperfusion. Patients who developed this response had hyponatremia, lower central venous pressure, lower pulmonary artery pressure, and lower pulmonary wedged capillary pressure. Abnormal cardiac response was related to a longer postoperative intubation time. CONCLUSION: Abnormal cardiac response is observed during LT and may be a manifestation of occult cirrhotic cardiomyopathy. This finding is underestimated with usual diagnostic tools and could be related to indirect signs of circulatory dysfunction of advanced liver disease.     

动静脉内瘘关闭修复术在肾移植中的应用

目的：探讨动静脉内瘘关闭修复术对心功能的影响,以扩大同种异体肾移植适应证。方法：对８例长期血液透析反复出现心力衰竭者在肾移植前５个月行动静脉内瘘关闭修复术。结果：心腔恢复正常７例,心腔内径在正常上限１例。肾移植成功６例,肝功能衰竭死亡１例,自发性肾破裂作肾切除１例。结论：肾移植前先行动静脉内瘘关闭修复术有利于改善患者心、肺功能,扩大手术适应证,减少并发症,为肾移植创造有利条件。     

肝动脉栓塞术后的肝内外胆道损毁性病变

目的 探讨经导管肝动态栓塞术后对胆道损伤的病因、治疗及预防。方法 总结５例因肝动脉栓塞引起的胆道损伤患者,其中４例为肝血管瘤经导管动脉栓塞术后,１例为手术时结扎肝动脉注射ＴＨ胶后。动物实验观察向大鼠肝动脉内注射乙醇复制血管栓塞剂对肝脏的损伤。结果 ５例患者均有胆道毁坏性病变,甚至造成胆汁性肝硬化,４例进行胆管空肠吻合及胆道支撑。实验结果证实肝动脉注射血管硬化剂无水乙醇后可以引起邻近汇管区肝脏局部坏     

Perioperative management of combined heart–liver transplantation in patients with cirrhosis,renal insufficiency,or pulmonary hypertension

Eyraud D, Ben Menna M, Vaillant JC, Kitajima K, Lebray P, Pavie A, Poynard T, Coriat P, Hannoun L. Perioperative management of combined heart–liver transplantation in patients with cirrhosis, renal insufficiency, or pulmonary hypertension.
Clin Transplant 2011: 25: 228–234. © 2010 John Wiley & Sons A/S. Abstract: Stating the main problem: Only few reports have detailed perioperative management and outcome of combined heart and liver transplantation (CHLT), and none describe the long‐term renal function. Methods: Three patients presented clinical signs of cardiomyopathy with reduced ejection fraction and proven cirrhosis with evidence of portal hypertension. Two of them presented renal failure, and the other pulmonary hypertension. After cardiac transplantation and closure of the sternum, liver transplantation was performed using systematically venovenous double‐limb (portal and caval) bypass. Results: Mean cold ischemic time for heart and liver was 2 h 46 min and 12 h 47 min, respectively. Intraoperative hemodynamics remained grossly stable during surgery. Mean transfusions were 12 red blood cell packs. All three patients received anti‐R‐Il2 antibodies at post‐operative day 1 and 4. Mean plasma creatinine concentration was 90 ± 8 μmol/L one yr post‐CHLT, vs 160 ± 62 μmol/L pre‐CHLT. All three patients are alive with functional grafts after a mean follow‐up of 26 months (12–38). Conclusion: CHLT could be performed safely through two consecutive and independent usual procedures. Perioperative hemodynamic stability, minimal blood loss, and routine splanchnic decompression are probably major determinants of a favorable outcome and good long‐term renal function.     

Individualized management of hepatic diseases in hereditary hemorrhagic telangiectasia

Liver involvement in patients with hereditary hemorrhagic telangiectasia (HHT) has not been fully characterized in China. The clinical manifestations, imaging studies, results of treatment in six patients and symptomatic liver involvement were analyzed. Patients included three women and three men with age from 35 to 62 years old. Two patients presented with shortness of breath, one patient with anemia and splenomegaly, and one with chronic gastrointestinal bleeding; the remaining two were asymptomatic. CT and CT angiography (CTA) showed arterioportal and arteriovenous shunting in liver. CTA showed at least one enlarged hepatic artery in all patients. One patient received ligation of the enlarged arteries with subsequent disappearance of symptoms at 56-month follow-up. The patient with gastrointestinal bleeding received interventional embolotherapy and resolved; interventional therapy to embolize the enlarged hepatic arteries was unsuccessful in another patient and the patient died of heart failure and liver dysfunction 38 months later. The patient with splenomegaly received a splenectomy and bandage of an enlarged hepatic artery. One of the two patients with no symptoms died of liver dysfunction 41 months after diagnosis. The other showed abnormal liver function and ascites, and traditional Chinese medicinal herb was used with no effect 21 months later. The symptoms disappeared after systemic medical treatment. Individualized and active therapy is advantageous and proper for patients with HHT.     

The role of oxidative stress in the development of pulmonary arteriovenous malformations after cavopulmonary anastomosis

BACKGROUND: Cavopulmonary anastomosis is used for palliation of cyanotic heart disease. Clinically significant pulmonary arteriovenous malformations occur in up to 25% of patients after surgical intervention. Cavopulmonary anastomosis creates several modifications to pulmonary physiology that may contribute to the development of pulmonary arteriovenous malformations, including reduced pulmonary blood flow and the exclusion of inferior vena caval effluent. OBJECTIVE: By comparing the expression of angiogenic and stress-related proteins after cavopulmonary anastomosis and pulmonary artery banding, we sought to determine which genes were upregulated independent of reduced pulmonary blood flow. METHODS: Lambs aged 35 to 45 days were placed into 1 of 3 groups: cavopulmonary anastomosis (n = 6), pulmonary artery banding (n = 6), and sham control (n = 6) animals. In our model pulmonary arteriovenous malformations are detectable by means of bubble-contrast echocardiography 8 weeks after cavopulmonary anastomosis. Lung tissue was harvested for Western blotting at 2 and 5 weeks after surgery. RESULTS: Cavopulmonary anastomosis and pulmonary artery banding both increased angiogenic gene expression, but only cavopulmonary anastomosis induced the expression of endothelial stress-related genes. Vascular endothelial growth factor was upregulated 2.5-fold after both cavopulmonary anastomosis (P =.002) and pulmonary artery banding (P =.007). Only cavopulmonary anastomosis upregulated 2 stress-related genes, HO1 and GLUT1, 2.7-fold (P =.002) and 3.8-fold (P =.03), respectively. Hypoxia-inducible factor was upregulated 4-fold (P =.003) after cavopulmonary anastomosis. Pulmonary artery banding failed to induce the increased expression of any of these proteins. CONCLUSIONS: Reduced pulmonary blood flow induces a pulmonary angiogenic response but not an endothelial stress response. These results suggest that oxidative stress is more relevant to the formation of pulmonary arteriovenous malformations than angiogenic signaling alone because pulmonary artery banding does not result in pulmonary arteriovenous malformations. Oxidative stress of the pulmonary endothelium resulting from cavopulmonary anastomosis may predispose the affected vasculature to arteriovenous shunting.     

Intrahepatic arterioportal fistula following conservative treatment of a traumatic liver rupture: case report.

A 16-year-old girl with severe liver injury complicated by the development of an intrahepatic arterial aneurysm and arteriovenous fistula is presented. Nonsurgical treatment combining close observation and repeat hepatic arterial embolization was successful.     

西罗莫司在肝移植术后钙调素类抑制剂相关肾损病人中的应用

目的 探讨西罗莫司对肝移植术后钙调素类免疫抑制剂相关肾功能损害病人的肾功能的改善作用及安全性.方法 对11例肝移植术后出现钙调素类免疫抑制剂相关肾损害病人进行西罗莫司转换治疗,同时减少或完全停止钙调素类免疫抑制剂的应用.观察转换治疗后病人的肾功能、肝功能、急性排斥反应的发生及药物副作用等情况.结果 随访至今所有病人均存活,随访时间6～23个月.转化治疗后所有病人的肾功能均有不同程度的改善,6个月后血肌酐从(163.8±47.9)μmol/L降为(108.1±26.6)μtmol/L(P<0.05);除1例病人出现转氨酶升高,加用钙调素类免疫抑制剂后恢复正常外,其余病人肝功能无明显变化;药物副作用有高脂血症、贫血、溃疡型口疮等.结论 西罗莫司可以安全地应用于肝移植术后钙调素类免疫抑制剂相关肾功能损害的病人,改善病人的肾功能,同时对移植肝功能无明显影响.     

Heart insufficiency in traumatic arteriovenous fistulas

Objective: To analyse the incidence, clinical presentation, and outcome of heart insufficiency in patients with chronic arteriovenous fistulas (AVF). Methodology: From 1991 to 2000 we treated 49 patients with traumatic AVF. The present study included 19 patients with AVF present for 6 months or longer, as it was presumed that these AVF will have an impact on cardiac overload. There were 16 male and three female patients with a mean age of 36 years (17–59). The time from injury to admission varied from 6 months to 33 years. Cardiological examination checked for dyspnoea and palpitation. ECG, chest X‐ray and ECHO‐cardiography were also performed. All AVF were deleted, magistral vessels reconstructed, non‐magistral obliterated, either surgically or by percutaneous embolization. Results: Mean follow up was 44.2 months (3–93). Serious heart insufficiency was seen in two patients only, with AVF in the subclavial vessels. Both suffered from serious heart disease prior to the therapeutic procedures that resulted in iatrogenic AVF, so it was difficult to connect heart insufficiency to the AVF alone. Despite surgical closure of AVF and intensive medical treatment, signs of heart insufficiency remained in both patients. Signs of cardiac overload were seen in six patients with long‐standing AVF in major vessels. Except for one patient who refused surgical closure of femoral AVF, the remaining five were symptom free for cardiac overload during the follow‐up period. Conclusion: In our series, heart insufficiency was an infrequent complication in long‐standing AVF, even when major vessels were involved.     

Management of aortopulmonary collaterals in children following cardiac transplantation for complex congenital heart disease.

BACKGROUND: Heart transplantation (HTx) is increasingly utilized as therapy for end-stage cyanotic congenital heart disease. This study investigates the presence and impact of aortopulmonary collaterals (APCs) associated with cyanotic heart disease on the early post-operative course of patients undergoing transplantation. High output cardiac failure due to residual aortopulmonary collaterals can affect outcome following heart transplantation. METHODS: Seven patients with hemodynamically significant APCs post-transplant were identified among 40 patients with cyanotic congenital heart disease undergoing HTx. The peri- and intra-operative courses of these patients were reviewed. All 7 patients required prolonged inotropic support despite normal ventricular function and no allograft rejection; 5 were ventilator-dependent due to significant pulmonary vascular congestion. Selective angiography demonstrated the presence of significant aortopulmonary collaterals at 7 to 19 days post-transplant. Coil embolization of aortopulmonary collaterals was performed in all patients; a mean of 6 (2 to 16) vessels/patient were embolized. RESULTS: After embolization, pulmonary edema resolved and heart size normalized in all patients; inotropic support was weaned within 2 to 10 days in 5 patients. One patient developed transient renal failure secondary to excessive contrast load and another had enterococcal sepsis within 24 hours after the procedure. All patients were asymptomatic from 4 to 10 years of follow-up post-HTx. CONCLUSIONS: Aortopulmonary collaterals should be considered a cause of early donor heart failure in children following HTx for cyanotic congenital heart disease. Early detection and treatment of aortopulmonary collaterals by coil embolization is necessary to improve the post-transplant course in these complex patients.     

The role of nontransplant procedures for sclerosing cholangitis

Twenty-five of 38 patients with sclerosing cholangitis underwent operative therapy at our institution. Seven patients with primarily extrahepatic obstruction had biliary bypass procedures and maintained normal liver function for 1 to 96 months. Biliary procedures were performed in 11 patients with combined intrahepatic and extrahepatic disease. Seven patients underwent subsequent liver transplantation because of deteriorating hepatic function, and two patients died before transplantation could be performed. Although there were no significant differences in outcome of liver transplantation whether or not a biliary procedure had been performed previously, previous biliary tract procedures influenced the type of biliary reconstruction performed, and two complications occurred as direct results of prior operations. Nontransplant procedures should be restricted to those patients with primarily extrahepatic obstruction, whereas liver transplantation should be considered the initial procedure of choice for patients with diffuse sclerosing cholangitis.     

Hepatic arterial embolization for massive bleeding from an intrahepatic artery pseudoaneurysm using N‐butyl‐2‐cyanoacrylate after living donor liver transplantation

Parenchymal pseudoaneurysm of the hepatic arteries with massive intraperitoneal bleeding is rare but a serious life‐threatening complication when it occurs following liver transplantation. We report a case of an adult postliving donor liver transplant recipient who developed massive subcapsular bleeding combined with massive right pleural effusion from ruptured multiple small intrahepatic arteries, which developed from a pseudoaneurysm that was treated by hepatic arterial embolization. Successful embolization was performed via a percutaneous trans‐catheter approach by depositing 20–25%N‐butyl‐2‐cyanoacrylate (NBCA) through the multiple small intrahepatic arteries into the pseudoaneurysm. Complete occlusion of the feeding arteries and pseudoaneurysm cavity resulted to immediate cessation of bleeding. There was no re‐bleeding; and normal liver graft function was noted postembolization. Hepatic arterial embolization with NBCA can be used as treatment for postliver transplant peripheral intrahepatic artery pseudoaneurysm bleeding.     

Reverse cardiac remodelling in patients with primary pulmonary hypertension after isolated lung transplantation.

OBJECTIVE: Pulmonary hypertension eventually leads to severe distortion of the cardiac geometry with consequent impact on cardiac function. The purpose of this study was to prove reverse cardiac remodelling after isolated bilateral lung transplantation (LuTX) in patients with advanced primary pulmonary hypertension (PPH) and severe alterations of cardiac morphology and function. METHODS: In the period of 2000-2002 17 (10 female, seven male) patients with advanced PPH underwent isolated bilateral LuTX. Median age was 30 years (range 16-53). All patients were in NYHA III or IV, most of them with intractable ascites, established renal impairment, malnutrition and immobility, continuously deteriorating despite various forms of pharmacological treatment including i.v. and inhalative prostacyclin, diuretics, Ca-antagonists, bosentan and catecholamines. Echocardiography and Doppler echocardiography measurements were performed before and 3 months after transplantation. Left and right ventricular diameters and function were assessed and tricuspid valve regurgitation was determined. RESULTS: Mortality after 3 months was 17.5% (cerebral bleeding, multi-organ failure and diffuse myocardial infarction in one patient each). Three months after LuTX the 14 surviving patients were in NYHA I or II. Echocardiography showed normal left ventricular function and markedly improved right ventricular function with normal size of the RV. The leftward shifted flattened interventricular septum had returned in its physiological position and the high-grade tricuspid insufficiency had disappeared in all patients. CONCLUSIONS: Advanced alterations of cardiac morphology and function normalize completely and pre-existing tricuspid insufficiency disappears in PPH patients after isolated bilateral LuTX. Quality of life is excellent. Therefore, LuTX is preferred and safe in patients with advanced PPH even with severe cardiac dysfunction.