Analysis of Risk Factors for Early Mortality in Surgical Shunt Palliation:Time for a Change?

Objectives:Over the last decade,neonatal repair has been advocated for many congenital heart diseases.However,specific subgroups of complex congenital heart disease still require temporary palliation for which both surgical and endovascular techniques are currently available.We reviewed our institutional experience with shunt palliation with an emphasis on risk factors for early mortality.Methods:This is a single-center retrospective study on 175 patients undergoing surgery for central shunt or modified Blalock-Taussig shunt.All data were extracted from a prospectively collected computerized database.We identified risk factors for early mortality by uni-and multi-variable analysis.All data were censored at the time of death or shunt take-down operation.Results:Mean age and weight at surgery were 24 days(IQR[7–95])and 3.4 kg(IQR[2.9–4.8]),respectively,with 96 neonates(55%).Most patients had a biventricular heart disease(115 patients,66%),and 51 patients(29.1%)had univentricular heart disease.Thoracotomy was performed in 129 patients(74%).Cardiopulmonary bypass was used in 23 patients(13%).The median intensive care and overall length of stay were 4 days(IQR[2–9])and 18 days(IQR[13–29]),respectively.In-hospital mortality was 8.6%(15/175).By multivariable regression analysis,prematurity(HR 5.6[2.1–14.7]),CPB use(HR 6.7[2.2–18.6]),unplanned<30-day reoperation(HR 3.5[1.2–10])or catheterization(HR 4.5[1.2–16.9])were all significant predictors of early mortality.Conclusions:Procedural-related mortality remains high(8.6%)in surgical shunt palliation.For patients with prematurity,low weight at birth,or if the use of cardiopulmonary bypass is contemplated,alternative endovascular techniques of palliation should be considered together with longitudinal follow-up studies.

Analysis of Pulmonary Arteries Growth after Initial Shunt Palliation in Neonates and Infants

Objective:Despite increasing enthusiasm for neonatal repair,patients with ductal-dependent circulation(pulmonary/systemic)or restrictive pulmonary blood flow still require initial palliation.Ductal stenting has emerged as an endovascular approach whereas modified-Blalock-Taussig and central shunt remain surgical references.In this study,we analyzed the relationship between pulmonary artery growth,sites of shunt connection,or antegrade pulmonary blood flow in surgically placed shunts.The need for secondary catheter-based interventions or pulmonary arterioplasty was also investigated.Methods:A retrospective single-center study analyzing 175 patients undergoing surgery for a central or modified-Blalock-Taussig shunt.Outcome growth variables were right pulmonary artery/left pulmonary artery diameters/Z scores,the indexed sum area(right pulmonary artery+left pulmonary artery),and the pulmonary symmetry index.Three imaging modalities were used:angiography,computed tomography,and echocardiography.Results:At baseline,pulmonary arteries were larger in patients with antegrade pulmonary blood flow(Nakata index 137 vs.114,p=0.047)as well as in patients receiving a modified-Blalock-Taussig shunt(Nakata index 138 vs.84,p<0.001).At the time of shunt takedown,both the right pulmonary artery and left pulmonary artery had normalized their diameter.The Nakata index increased from 134 to 233 mm^(2)/m^(2)(p<0.001).The pulmonary artery index remained stable(0.86)over time.During the inter-stage period,shunt-related pulmonary artery stenosis and juxta-ductal stenosis were diagnosed in 16(10%)and 17 patients(11%),respectively.Conclusions:Surgical shunt palliation allows normal pulmonary artery growth.Pulmonary artery stenosis was either shunt-related(10%)or secondary to juxta-ductal stenosis(11%).Close echographic follow-up allows early diagnosis and treatment of juxta-ductal stenosis.

Preliminary Evaluation of Hemodynamic Effects of Fontan Palliation on Renal Artery Using Computational Fluid Dynamics

Background:The assessment of renal function is important to the prognosis of patients needing Fontan palliation due to the reconstructed compromised circulation.To know the relationship between the kidney perfusion and hemodynamic characteristics during surgical design could reduce the risk of acute kidney injury(AKI)and the postoperative complications.However,the issue is still unsolved because the current clinical evaluation methods are unable to predict the hemodynamic changes in renal artery(RA).Methods:We reconstructed a three-dimensional(3D)vascular model of a patient requiring Fontan palliation.The technique of computational fluid dynamics(CFD)was utilized to explore the changes of RA hemodynamics under different possible blood flow rates.The relationship between the kidney perfusion and hemodynamic characteristics was investigated.Results:The calculated results indicated the declined tendency of the pressure and pressure drop as the flow rate decreased.When the flow rate decreased to two-thirds of its baseline,both the pressure of left renal artery(LRA)and the pressure of right renal artery(RRA)dipped below 50%,and the pressure of RRA fell more quickly than that of LRA.Uneven distribution of WSS was observed on the trunk of RA,and the lowest WSS was found at the distal of RA.The average WSS in RA dropped to around 50%as the flow rate reached one-third of its baseline.Conclusions:As a promising approach,CFD can be utilized to quantitatively evaluate the hemodynamic characteristics of RA and contribute to offsetting the drawbacks of clinical assessments of renal function,to help realize better prognosis for the patients with Fontan palliation.

Comparison of 2D and 4D Flow MRI Measurements for Hemodynamic Evaluation of the Fontan Palliation

Background:The assessment of Fontan circuit’sflow is traditionally evaluated by multiple through-plane phase-contrast MRI acquisitions(2Dflow),while recently,a single volumetric 4D-flow MRI acquisition is emerging as a comprehensive tool for the hemodynamic evaluation in congenital heart diseases.Purpose:To compare 2D and 4D-flow MRI measurements in patients after Fontan palliation and to evaluate parameters affecting potential dis-agreement.Methods:39 patients after Fontan palliation(23 males,age 22±11 years)who underwent cardiac MRI with 2D and 4D-flow MRI acquisition were included in the study.In all patients,bloodflow quantification in the Fontan circuit and aorta by 2Dflow and by 4Dflow MRI acquisition blinding to the 2D results was per-formed.The agreement between 2D and 4D-flow MRI was calculated as the intraclass correlation coefficient(ICC).The mean absolute differences between 4D and 2Dflows were analyzed using linear regression models.Results:4D-flow MRI acquisition time was slightly lower than 2D(7.6±1.8 min vs.9.4±3.3 min,p=0.03).Flow was slightly predominant in the right pulmonary artery(58%of total pulmonaryflow).Conduit/tunnel-pul-monary arteriesflow accounted for 60%of the Fontan circuit.Agreement between 2D and 4D was overall good-to-excellent from ICC:0.81795%CI:0.637–0.907 to 0.93295%CI:0.866–0.965.There was no significant influ-ence of evaluated parameters on the agreement on 4D and 2Dflow.Conclusions:4D-flow MRI represents a valid tool in Fontan’sflow quantification.Further larger studies are needed to confirm our results and to evaluate the impact of advanced 4D-flow MRI parameters on the prognostic stratification in patients after Fontan palliation.

Current role of palliative interventions in advanced pancreatic cancer

Pancreatic adenocarcinoma is the third leading cause of cancer death in the United States. Unfortunately, at diagnosis, most patients are not candidates for curative resection. Surgical palliation, a procedure performed with the intention of relieving symptoms or improving quality of life, comes to the forefront of management. This article reviews the palliative management of unresectable pancreatic cancer, including obstructive jaundice, duodenal obstruction and pain control with celiac plexus block. Although surgical bypasses for both biliary and duodenal obstructions usually achieve good technical success, they result in considerable perioperative morbidity and mortality, even when performed laparoscopically. The effectiveness of selfexpanding metal stents for biliary drainage is excellent with low morbidity. Surgical gastrojejunostomy for duodenal obstruction appears to be best for patients with a life expectancy of greater than 2 mo while endoscopic stenting has been shown to be feasible with good symptom relief in those with a shorter life expectancy. Regardless of the palliative procedure performed, all physicians involved must be adequately trained in end of life management to ensure the best possible care for patients.

Palliation:Hilar cholangiocarcinoma

Hilar cholangiocarcinomas are common tumors of the bile duct that are often unresectable at presentation. Palliation, therefore, remains the goal in the majority of these patients. Palliative treatment is particularly indicated in the presence of cholangitis and pruritus but is often also offered for high-grade jaundice and abdominal pain. Endoscopic drainage by placing stents at endoscopic retrograde cholangio-pancreatography(ERCP) is usually the preferred modality of palliation. However, for advanced disease, percutaneous stenting has been shown to be superior to endoscopic stenting. Endosonography-guided biliary drainage is emerging as an alternative technique, particularly when ERCP is not possible or fails. Metal stents are usually preferred over plastic stents, both for ERCP and for percutaneous bili-ary drainage. There is no consensus as to whether it is necessary to place multiple stents within advanced hi-lar blocks or whether unilateral stenting would suffice. However, recent data have suggested that, contrary to previous belief, it is useful to drain more than 50% of the liver volume for favorable long-term results. In the presence of cholangitis, it is beneficial to drain all of the obstructed biliary segments. Surgical bypass plays a limited role in palliation and is offered primarily as asegment Ⅲ bypass if, during a laparotomy for resec-tion, the tumor is found to be unresectable. Photody-namic therapy and, more recently, radiofrequency abla-tion have been used as adjuvant therapies to improve the results of biliary stenting. The exact technique to be used for palliation is guided by the extent of the bili-ary involvement(Bismuth class) and the availability of local expertise.

Photodynamic therapy:Palliation and endoscopic technique in cholangiocarcinoma

Cholangiocarcinoma is the primary malignancy arising from the biliary epithelium.The disease is marked by jaundice,cholestasis,and cholangitis.Over 50 percent of patients present with advanced stage disease,precluding curative surgical resection as an option of treatment.Prognosis is poor,and survival has been limited even after biliary decompression.Palliative management has become the standard of care for unresectable disease and has evolved to include an endoscopic approach. Photodynamic therapy(PDT)consists of administration of a photosensitizer followed by local irradiation with laser therapy.Several studies conducted in Europe and the United States have shown a marked improvement in the symptoms of cholestasis,survival,and quality of life.This article summarizes the published experience regarding PDT for cholangiocarcinoma and the steps required to administer this therapy safely.

Quality of survival in patients treated for malignant biliary obstruction caused by unresectable pancreatic head cancer: surgical versus non-surgical palliation

BACKGROUND:Appropriate palliation for unresectable pancreatic head cancer is most important.This study was undertaken to compare the survival of patients with biliary obstruction caused by unresectable pancreatic head cancer after surgical and non-surgical palliation. METHODS:We retrospectively reviewed 69 patients who underwent palliative treatment for unresectable pancreatic head cancer.Fifty-two patients with locally advanced disease (local vascular invasion)and 17 with distant metastatic disease were included.The patients were divided into two groups,surgical and non-surgical palliation. RESULTS:Thirty-eight patients underwent biliary bypass surgery and 31 had percutaneous transhepatic biliary drainage(PTBD).There was no significant difference in the early complications,successful biliary drainage, recurrent jaundice,and 30-day mortality between surgical palliation and PTBD.However,in 52 patients whose tumor was unresectable secondary to local vascular invasion,the rate of recurrent jaundice after successful surgical biliary palliation was lower than that in patients who had non- surgical palliation(P<0.05).The patients who underwent surgical palliation had a longer hospital-free survival rate(P<0.001),although they had a longer postoperative hospital stay(P=0.004)during the first admission period.CONCLUSIONS:In patients with preoperative evaluations showing potentially resectable tumors and/or no metastatic lesions,surgical exploration should be performed.Thus,in patients who have unresectable cancer or limited metastatic disease on exploration,surgical palliation should be performed for longer survival and better quality of survival.

Endoscopic palliation of malignant biliary stricture

Malignant biliary strictures often present late after the window for curative resection has elapsed. In such patients, the goal of therapy is typically focused on palliation. While historically, palliative measures were performed surgically, the advent of endoscopic intervention offers minimally invasive options to provide relief of symptoms, improve quality of life, and in some cases, increase survival of these patients. Some of these therapies, such as endoscopic biliary decompression, have become mainstays of treatment for decades, whereas newer modalities, including radiofrequency ablation, and photodynamic therapy offer additional options for patients with incurable biliary malignancies.

Surgical palliation of gastric outlet obstruction in advanced malignancy

Gastric outlet obstruction(GOO) is a common problem associated with advanced malignancies of the upper gastrointestinal tract.Palliative treatment of patients' symptoms who present with GOO is an important aspect of their care.Surgical palliation of malignancy is defined as a procedure performed with the intention of relieving symptoms caused by an advanced malignancy or improving quality of life.Palliative treatment for GOO includes operative(open and laparoscopic gastrojejunostomy) and non-operative(endoscopic stenting) options.The performance status and medical condition of the patient,the extent of the cancer,the patients prognosis,the availability of a curative procedure,the natural history of symptoms of the disease(primary and secondary),the durability of the procedure,and the quality of life and life expectancy of the patient should always be considered when choosing treatment for any patient with advanced malignancy.Gastrojejunostomy appears to be associated with better long term symptom relief while stenting appears to be associated with lower immediate procedure related morbidity.

The Role of Laparoscopy and Laparoscopic Ultrasound in Staging and in Palliation of Upper Gastro-Intestinal Malignancies: The Egyptian National Cancer Institute Experience

Background: Surgical resection of upper gastrointestinal malignancies occasionally carries substantial morbidity due to inaccurate pre-operative staging. The potential to prevent needless laparotomy by means of precise staging is the pouring force behind the use of diagnostic laparoscopy (DL). Objective: To assess the role of laparoscopy and laparoscopic ultrasound (LUS) in proper staging of upper gastro intestinal malignancies, and in potential palliation in advanced cases for pain (by neurolytic celiac plexus block) or gastric outlet obstruction (by laparoscopic bypass surgery). Study design: In this prospective study, 62 patients with lower esophageal, gastric and peri-pancreatic carcinomas were joined after written informed consent. All patients were examined with laparoscopy and LUS with the help of frozen section analysis to any doubtful metastatic site, peritoneal fluid and ascitic fluid analysis. Results: DL helped us to avoid needless laparotomy in 22.5% of patients, reducing its post-operative complications, hospital stay and cost. DL also helped us to do palliative management either in the form of gastric bypass or laparoscopic celiac plexus block. Conclusion: we praise the use of DL as a safe, effective and complimentary method to the other routine imaging modalities, in proper staging and palliation for upper gastrointestinal malignancies.

A Rare Case of Successive Pregnancies in a Woman with Complex Cyanotic Congenital Heart Disease after Glenn Palliation

The bidirectional Glenn procedure is an integral step in the optimal palliation for single ventricular physiology in many forms of complex congenital heart disease. An increasing number of women who have undergone this connection in childhood are now reaching childbearing years. Low pulmonary blood flow and volume over load on the single ventricle pose several problems during pregnancy. We are reporting a 33-year-old lady with congenital tricuspid atresia and mild pulmonary stenosis who had late Bidirectional Glenn procedure with pulmonary forward flow and later underwent six successful pregnancies, with delivery of six low birth weight babies with no reported complications.

Assessing Patient Outcomes after Palliative Radiotherapy Using IG-IMRT

Purpose: To assess clinical outcomes after using IG-IMRT for palliation among patients with advanced cancers. Methods: Patients with advanced and/or metastatic cancers were treated on our Tomo-PAL (Tomotherapy?-Planning and Administration Linked) protocol using helical TomoTherapy? and evaluated to assess clinical efficacy of treatment as well as to assess side effects. Results: A total of 40 patients were treated to 40 sites from Feb 2007 to May 2009. There were 25 men and 15 women with a median age of 70 years (range 16 - 94). Pain and bleeding were the most common symptoms being palliated (80% and 12.5% respectively). The dose prescribed ranged from 5 - 25 Gy in 1 - 5 fractions. A qualitative improvement in symptoms was documented in 82% of patients (75% partial relief and 7% complete relief) and major side effects were not encountered. Conclusions: IG-IMRT can be used for palliation and produces response rates that compare favourably with those reported in the published literature.

Natural History of Kawashima Palliation in Single-Ventricle and Interrupted Inferior Vena Cava Heart Disease in China 11 Years Result

Background:The long-term outcomes of patients treated with a Kawashima procedure and keeping the antegrade pulmonary blood flow(AnPBF)in single-ventricle(SV)and interrupted inferior vena cava(IVC)heart disease is still uncertain as yet.Methods:We investigated 18 patients who underwent the Kawashima procedure with SV physiology and an interrupted IVC between January 2009 and June 2018,perioperative,operative and postoperative characteristics were recorded.Results:A total of 18 patients underwent the Kawashima procedure at a median age of 2.7 years(range 0.5–24.7 years),of which 12(66.7%)were male and 6(33.3%)were female.The mean saturation was 76.2±8.5%in preoperative period and 94.2±2.2%in postoperative period.All patients had kept AnPBF.The median duration of mechanical ventilation was 12 h(range 2.5–22.5 h)and the median duration of pleural drainage was 5 days(range 2–27 days).The median hospital stay was 9 days(range 6–70 days).There was no operative death and no mortality was seen in early postoperative period.Follow-up was 100%completed,with an average follow-up period of 6.1±2.7 years(range 1–11 years).4 patients died during the follow-up.The overall 5 and 10 years’survival rates estimated by Kaplan-Meier method were 88%and 68%,respectively.Although there were no significant differences in the duration of postoperative follow-up between the death group and the survival group(p>0.05),the major systemic ventricular end-diastolic diameter(SVEDD)(p=0.018)and the degree of AVVR(p=0.001)showed significant difference between the two groups.The diameters of main pulmonary artery showed significant growth in both the death group(p=0.015)and the survival group(p=0.012)over time.SVEDD had no significant increase in the survival group(p=0.665)but was significantly larger in the death group(p=0.014).Multivariable risk factors of late mortality in patients treated with Kawashima procedure were follow-up AVVR(p=0.044;HR:3.124;95%CI:1.030–9.473)and SVEDD(p=0.031;HR:9.766;95%CI:1.226–77.8).14 patients(100%)were all in New York Heart Association(NYHA)functional class I and the mean saturation was 93±2%at last follow-up.Only one patient finished Fontan completion.Conclusions:The Kawashima procedure with AnPBF can be safely performed with acceptable early and long outcomes.Although some previous studies have shown the risk of pulmonary arteriovenous malformations(PAVMS)after Kawashima procedure in the mid-and long-term,our findings are in contradiction with it.No PAVMs occurred in all the survivors.Kawashima procedure with open AnPBF may be a good option for unsuitable Fontan candidates.

Palliative Senning Procedure: Management of dTGA with VSD and PVOD in an Adolescent

Patients with D-TGA with atrial level mixing of systemic and pulmonary blood, the morphological left ventricle supports the low pressure pulmonary circulation and regresses in mass and volume as time passes. The LV once regressed is unable to support the high pressure systemic circulation after ASO. These patients are candidates for early ASO, preferably within 3 weeks of life. As age progresses, the scope of these surgical procedures skews and an atrial switch remains the only option to improve quality of life. In Atrial switch, the pulmonary and systemic circulation is returned into an “in series” circulation and cyanosis is obliterated.

Advances in diagnosis,treatment and palliation of cholangiocarcinoma:1990-2009

Several advances in diagnosis,treatment and palliation of cholangiocarcinoma(CC)have occurred in the last decades.A multidisciplinary approach to this disease is therefore recommended.CC is a relatively rare tumor and the main risk factors are:chronic inflammation, genetic predisposition and congenital abnormalities of the biliary tree.While the incidence of intra-hepatic CC is increasing,the incidence of extra-hepatic CC is trending down.The only curative treatment for CC is surgical resection with negative margins.Liver transplantation has been proposed only for selected patients with hilar CC that cannot be resected who have no metastatic disease after a period of neoadjuvant chemo-radiation therapy.Magnetic resonance imaging/magnetic resonance cholangiopancreatography,positron emission tomography scan,endoscopic ultrasound and computed tomography scans are the most frequently used modalities for diagnosis and tumor staging.Adjuvant therapy,palliative chemotherapy and radiotherapy have been relatively ineffective for inoperable CC.For most of these patients biliary stenting provides effective palliation.Photodynamic therapy is an emerging palliative treatment that seems to provide pain relief,improve biliary patency and increase survival. The clinical utility of other emerging therapies such as transarterial chemoembolization,hepatic arterial chemoinfusion and high intensity intraductal ultrasound needs further study.

Advances in diagnosis, treatment and palliation of pancreatic carcinoma: 1990-2010

Several advances in genetics, diagnosis and palliation of pancreatic cancer (PC) have occurred in the last decades. A multidisciplinary approach to this disease is therefore recommended. PC is relatively common as it is the fourth leading cause of cancer related mortality. Most patients present with obstructive jaundice, epigastric or back pain, weight loss and anorexia. Despite improvements in diagnostic modalities, the majority of cases are still detected in advanced stages. The only curative treatment for PC remains surgical resection. No more than 20% of patients are candidates for surgery at the time of diagnosis and survival remains quite poor as adjuvant therapies are not very effective. A small percentage of patients with borderline non-resectable PC might benefit from neo-adjuvant chemoradiation therapy enabling them to undergo resection; however, randomized controlled studies are needed to prove the benefits of this strategy. Patients with unresectable PC benefit from palliative interventions such as biliary decompression and celiac plexus block. Further clinical trials to evaluate new chemo and radiation protocols as well as identification of genetic markers for PC are needed to improve the overall survival of patients affected by PC, as the current overall 5-year survival rate of patients affected by PC is still less than 5%. The aim of this article is to review the most recent high quality literature on this topic.

Self-expanding metal stents for the palliation of malignant gastric outlet obstruction in patients with peritoneal carcinomatosis

AIM To evaluate the efficacy of self-expanding metal stents(SEMS) for the palliation of malignant gastric outlet obstruction in patients with and without peritoneal carcinomatosis(PC).METHODS We performed a retrospective analysis of 62 patients who underwent SEMS placement for treatment of malignant gastroduodenal obstruction at our hospital over a six-year period. Stents were deployed through the scope under combined fluoroscopic and endoscopic guidance. Technical success was defined as successful stent placement and expansion. Clinical success was defined as an improvement in the obstructive symptoms and discharge from hospital without additional parenteral nutrition. According to carcinomatosis status, patients were assigned into groups with or without evidence of peritoneal disease.RESULTS In most cases, obstruction was caused by pancreatic(47%) or gastric cancer(23%). Technical success was achieved in 96.8%(60/62), clinical success in 79%(49/62) of all patients. Signs of carcinomatosis were identified in 27 patients(43.5%). The diagnosis was confirmed by pathology or previous operation in 7 patients(11.2%) and suspected by CT, MRI or ultrasound in 20 patients(32.2%). Presence of carcinomatosis was associated with a significantly lower clinical success rate compared to patients with no evidence of peritoneal disease(66.7% vs 88.6%, P = 0.036). There was no significant difference in overall survival between patients with or without PC(median 48 d vs 70 d, P = 0.21), but patients showed significantly longer survival after clinical success of SEMS placement compared to those experiencing clinical failure(median 14.5 d vs 75 d, P = 0.0003).CONCLUSION Given the limited therapeutic options and a clinical success rate of at least 66.7%, we believe that SEMS are a reasonable treatment option in patients with malignant gastric outlet obstruction with peritoneal carcinomatosis.

Stomach-interposed cholecystogastrojejunostomy: A palliative approach for periampullary carcinoma

AIM: For patients of periampullary carcinoma found to be unresectable at the time of laparotomy, surgical palliation is the primary choice of treatment. Satisfactory palliation to maximize the quality of life with low morbidity and mortality is the gold standard for a good procedure.Our aim is to explore such a procedure as an alternative to the traditional ones.METHODS: A modified double-bypass procedure is performed by, in addition to the usual gastrojejunostomy,implanting a mushroom catheter from the gall bladder into the jejunum through the interposed stomach as an internal drainage. A retrospective review was performed including 22 patients with incurable periampullary carcinomas who underwent this surgery.RESULTS: Both jaundice and impaired liver function improved significantly after surgery. No postoperative deaths, cholangitis, gastrojejunal, biliary anastomotic leaks, recurrent jaundice or late gastric outlet obstruction occurred. Delayed gastric emptying occurred in two patients. The total surgical time was 150±26 min. The estimated blood loss was 160±25 mL. The mean length of hospital stay after surgery was 22±6 d. The mean survival was 8 mo (range 1.5-18 mo).CONCLUSION: In patients of unresectable periampullary malignancies, stomach-interposed cholecystogastrojejunostomy is a safe, simple and efficient technique for palliation.

Clinical results of combined palliative procedures for cyanotic congenital heart defects with intractable hypoplasia of pulmonary arteries

Background Congenital heart defects with intractable hypoplasia of the pulmonary arteries without intercourse or with intercourse stenosis is unsuitable for surgical correction or regular palliative procedures. We reported our experience with combined palliative procedures for congenital heart defects with intractable hypoplasia pulmonary arteries. Methods From 2001 to 2012, a total of 41 patients with cyanotic congenital heart defects and intractable hypoplasia of the pulmonary arteries underwent surgical procedures. From among them, 31 patients had pulmonary atresia with ventricular septal defect （VSD） and the other 10 cases had complicated congenital heart defects with pulmonary stenosis. Different kinds of palliative procedures were performed according to the morphology of the right and left pulmonary arteries in every patient. If the pulmonary artery was well developed, a Glenn procedure was performed. A modified Blalock-Taussig shunt or modified Waterston shunt was performed if pulmonary arteries were hypoplastic. If the pulmonary arteries were severely hypoplastic, a Melbourne shunt was performed. Systemic pulmonary artery shunts were performed bilaterally in 25 cases. A systemic-pulmonary shunt was performed on one side and a Glenn procedure was performed contralaterally in 16 cases. Major aortopulmonary collateral arteries were unifocalized in six cases, ligated in two cases and interventionally embolized in two cases. There was one early death because of cardiac arrest and the hospital mortality was 2.4%. Results Five patients suffered from postoperative low cardiac output syndrome, three had perfusion of the lungs, and two pulmonary infections. Systemic pulmonary shunts were repeated after the original operation in three cases due to the occlusion of conduits. The mean follow-up time was 25 months. The pre- and the post-operation left pulmonary indices were （8.13±3.68） vs. （14.9±6.21） mm2/m2. The pre- and post-operation right pulmonary indices were （12.7±8.13） vs. （17.7±7.78） mm2/m2. The pre- and post-operational pulmonary indices were （20.87±9.43） vs. （32.6±11.7） mm2/m2. They were all significantly increased （P 〈0.001）. The diameter of the pulmonary artery increased after the modified Blalock-Taussig shunt （（5.51±0.94） mm2/m2 pre-operation vs. （7.01±1.97） mm2/m2 post-operation）, the modified Waterston shunt （（5.70±3.96） mm2/m2 pre-operation vs. （9.17±3.62） mm2/m2 post-operation） and the Melbourne shunt （（2.17±0.41） mm2/m2 pre-operation vs. （7.35±2.49） mm2/m2 post-operation） （all P 〈0.05）. Bilateral pulmonary arteries developed well as compared to their pre-operation development. Hemoglobin decreased from （194±27） to （174±24） g/L （P 〈0.05） and peripheral oxygen saturation increased from （65±11）% to （84±6）% （P 〈0.001 ）. During the follow-up of 27 to 49 months, ultimate complete repair was performed in four cases and one patient underwent a Glenn procedure. Conclusions The procedures should be considered on a case to case basis in patients having hypoplasia of the pulmonary arteries with cyanotic congenital heart defects. Combined palliative operations could be an adequate strategic treatment.