Acute Shunt Occlusion Research Articles

C45 MULTI–INSTITUTIONAL ITALIAN EXPERIENCE OF ATRIAL FLOW REGULATOR DEVICE IN CONGENITAL AND ACQUIRED HEART DISEASE

Abstract The Atrial Flow Regulator is a self–expandable nitinol device implanted into the interatrial septum to establish a communication with a predetermined diameter.The creation of such shunt is advisable in several cardiovascular diseases.In pediatric population,the experience is limited to a few case reports describing patients with failing Fontan,pulmonary hypertension,or severe heart failure.We aim to report the preliminary data of Italian registry of AFR implantation in children with heart failure in congenital and acquired heart disease.The goal is to create a local and national register,to monitoring the safety and efficacy of the AFR in pediatric population.The register is prospective and multi–institutional, with 2 centers involved: Pediatric Cardiology department of Padova Hospital and of Bambino Gesù Hospital in Rome,started on the 27/11/2021 and ongoing.AFR implantation was proposed for all patients with symptomatic heart failure,not responding to maximal medical therapy and a history of complex congenital or acquired cardiac diseases.We enrolled 8 patients (median age 124 months)indications were LV systolic dysfunction in 4 patients,restrictive cardiomyopathy with pulmonary hypertension in one, post–operative RV dysfunction after surgical repair of a native TOF and 2 patients with failing Fontan.AFR implantation was successfully reached in all 8 patients.In one case,it was performed a hybrid procedure during pulmonary artery surgical banding, with peratrial approach through the right atrial free wall.Atrial septostomy was needed in 5 cases, in the other 3 cases pre–existing PFO and ASD were used.Balloon pre–dilation was performed in 4 cases.Mean time of procedure was 50 minutes and median radiation exposure dose was 3 Gy/cm2.No complications were reported during the procedure.No acute or sub–acute shunt occlusion or stroke was observed.One patient died of sepsis not related to the procedure;one patient presented a cardiac arrest due to a cerebral ischemia 24 hours after the procedure.Heparin or aspirin were administered to all patients for at least 6 months.Clinical improvement was observed in all patients with an increase in ejection fraction (p=0.02), reduction in LA dilation(p< 0.05)and in HF symptoms(62% of patients improved their NYHA class).AFR implantation is safe and feasible in children with specific congenital and acquired heart diseases,allowing right/left cavities unloading and improvement of hemodynamics and symptoms.

Good Clinical Outcomes in Budd-Chiari Syndrome with Hepatic Vein Occlusion.

Budd-Chiari syndrome (BCS) with hepatic vein (HV) occlusion is manifested by severe liver damage in acute cases and esophageal variceal bleeding or refractory ascites in chronic cases, which is difficult to differentiate from cirrhotic portal hypertension. To evaluate the clinical efficacy and safety of HV angioplasty and transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of BCS with HV occlusion. Between May 1995 and December 2014, 60 patients with HV occlusive BCS underwent HV angioplasty or TIPS. BCS was subacute or chronic in 55 patients and acute in 5 patients. HV angioplasty was performed in 18 patients with HV occlusion, combined HV and IVC angioplasty in 9 patients with HV and IVC occlusion, TIPS in 12 patients with HV occlusion, and modified TIPS in 21 patients with extensive HV occlusion. The interventional procedure was successfully performed in all 60 patients. The portal pressure decreased from 41.23±10.46cmH2O preoperatively to 26.68±6.46cmH2O postoperatively, while the portal flow velocity increased from 14.31±10.43 to 52.16±13.68cm/s in patients undergoing TIPS or modified TIPS. During hospitalization, two patients died from hepatic failure, and acute shunt occlusion occurred in two other patients during subsequent treatment with repeated intervention. During 82.25±46.16months of follow-up, three patients underwent re-intervention with a stenotic shunt, and other three with repeated dilation of the stenotic HV. HV angioplasty and TIPS yield excellent long-term outcomes in patients with HV occlusive BCS.

Role of thrombophilia factors in acute systemic-pulmonary shunt obstruction.

Systemic-pulmonary shunts are widely used in initial palliation in cyanotic congenital heart disease. The incidence and the relationship between acute shunt obstruction and thrombophilia are not precisely defined. The aim of this study was to determine the frequency of shunt obstruction in the early postoperative period, and to define the frequency and presence of thrombophilia factors in patients treated for acute shunt thrombosis. Between October 2010 and October 2012, 77 patients who had systemic-pulmonary shunt operation were included in this prospective study. Patients who developed shunt obstruction were examined in terms of inherited and acquired thrombophilia factors. Median patient age was 61 days and median weight was 4.3 kg. Thirty-three patients were neonates. Diameter of the Gore-Tex grafts used for the shunt ranged from 3 mm to 5 mm. Acute shunt occlusion rate was 10% (8/77), and all of these occurred in the first 24 h. Thrombophilia was found in three of eight patients who underwent intervention (surgical and/or transcatheter) due to shunt thrombosis (presence of anti-phospholipid antibodies, n = 1; protein C deficiency, n = 1; and factor V Leiden mutation, n = 1) and only one patient died. Acute shunt obstruction developed in 10% of patients who underwent systemic-pulmonary shunt, and emergency surgery or transcatheter intervention can be life saving in this context. Acute shunt obstruction can occur due to mechanical and hemodynamic problems, but clinicians should also consider and evaluate thrombophilia factors.

Transcatheter stenting of the systemic-to-pulmonary artery shunt: A 7-year experience from a single tertiary center.

Systemic-to-pulmonary artery shunt (SPS) dysfunction can be deleterious in shunt dependent patients and traditionally have undergone surgical revision. Data on transcatheter stenting of SPS is limited. We sought to evaluate feasibility, safety and outcomes of stenting SPS. Retrospective review of all patients who underwent transcatheter SPS stenting from 1/2006 to 12/2013. Of 229 surgically implanted SPS, 25 transcatheter stent interventions were performed in 22 patients. The majority had pulmonary atresia (n = 9) or HLHS (n = 10). Their median age was 4 ms (range 10 days to 4 years) and median weight 4.9 kg (range 3-14). Nine had a central and 15 had a BT shunt with a median shunt size of 3.75 mm (range 3-6). The interval from shunt placement to intervention was 1.9 ms (range 4 days-3.8 years). The indication for intervention was increasing cyanosis in10 patients and delaying final repair in 9. Two patients were on ECMO at the time of intervention. The median shunt diameter increased from 2.3 to 4.1 mm and oxygen saturation from 72 to 85% (P < 0.001). No intra-procedural complications were encountered. One patient died from aspiration (autopsy demonstrated a patent shunt), 13 progressed to repair or next stage, 6 remain palliated with shunts as they are deemed unfit for the next stage and 2 are awaiting surgery. Stenting of systemic to pulmonary artery shunt is a safe and effective procedure and avoids surgical re-intervention. It can be performed both as a rescue procedure in patients with acute shunt occlusion and as an elective procedure to palliate patients not yet suitable for subsequent corrective or staged repair.

Central Shunt Procedures for Complex Congenital Heart Diseases

The study was designed to analyze the results of central shunt procedure using Gore-Tex grafts for treatment of patients with complex congenital heart diseases. A Gore-Tex graft was implanted using an end-to-side anastomosis technique connecting the aorta with the pulmonary artery. The graft size was determined based on the patients' body weight. Artery growth and percutaneous plasma oxygen were examined pre- and postoperatively. The procedure was performed without cardiopulmonary bypass in 96 of 110 cases. Shunt sizes from 3.5 to 6.0 mm were employed. After operation, the oxygen saturations increased significantly from 65.2% ± 7.3 to 84.1% ± 3.8 (p < 0.05). Both left and right pulmonary arteries were found to grow significantly in size, from 4.9 ± 1.8 to 7.5 ± 2.0 mm and from 6.1 ± 2.3 to 8.0 ± 4.7 mm, respectively (p < 0.05). Early mortality was 5.5% (6/110). Major shunt-related complications included congestive heart failure (1.8%, 2/110) and acute shunt occlusion (1.8%, 2/110). Median follow-up was 18 months (range 6 to 61), with late mortality of 3.8% (4/104) one month postoperation. The central shunt increases oxygen saturation and improves pulmonary artery development effectively with a relatively low incidence of congestive heart failure, acute occlusion, and pulmonary distortion. The adequate postoperation survival, low morbidity and mortality, and less technical difficulty of this procedure make it a more desirable treatment for complex heart diseases.

Endovascular evaluation and treatment of intrahepatic portosystemic shunts in dogs: 100 cases (2001–2011)

To evaluate short- and long-term outcome following endovascular treatment of intrahepatic portosystemic shunts in dogs. Retrospective case series. 100 dogs. All patients had angiographic evaluation with or without endovascular shunt attenuation. The medical records were reviewed for pertinent data, complications, outcome, and survival time. 95 dogs with congenital intrahepatic portosystemic shunts received 111 procedures (83% [79/95] had 1 treatment, and 17% [16/95] had > 1 treatment; 5 dogs had no treatment because of excessive portal venous pressure-central venous pressure gradients). Angiography identified 38 right, 33 left, and 19 central divisional single shunts (n = 90) and 10 complex or multiple shunts. Partial shunt attenuation was performed in 92 dogs by means of caval stent placement and insertion of thrombogenic coils within the shunt, and 3 had complete acute shunt occlusion. Major intraoperative complications (3/111 [3%]) included temporary severe portal hypertension in 2 dogs and gastrointestinal hemorrhage in 1 dog. Major postoperative (< 1 week after surgery) complications (14/111 [13%]) included seizures or hepatoencephalopathy (7/111 [6%]), cardiac arrest (2/111 [2%]), jugular site bleeding (2/111 [2%]), pneumonia (1/111 [1%]), suspected portal hypertension (1/111 [1%]), and acute death (1/111 [1%]). Median follow-up time was 958 days (range, 0 to 3,411 days). Median survival time for treated dogs was 2,204 days (range, 0 to 3,411 days). Outcome was considered excellent (57/86 [66%]) or fair (13/86 [15%]) in 70 of 86 (81%) treated dogs. Results suggested that endovascular treatment of intrahepatic shunts in dogs may result in lower morbidity and mortality rates, with similar success rates, compared with previously reported outcomes for open surgical procedures. Gastrointestinal ulceration was a common finding among this population of dogs, and lifelong gastroprotectant medications are now recommended.

Favorable clinical outcome using a covered stent following transjugular intrahepatic portosystemic shunt in patients with portal hypertension

To compare retrospectively the clinical outcomes in patients treated with transjugular intrahepatic portosystemic shunt (TIPS) using the novel polytetrafluoroethylene-covered stents (Fluency) and bare stents. Sixty consecutive patients with portal hypertension treated with TIPS from April 2007 to April 2009 were included. TIPS creation was performed with Fluency stent grafts in 30 patients (group A) and with bare stents in 30 patients (group B). Liver function, TIPS patency and clinical outcomes were evaluated every 3 months after procedures. During hospitalization, there were no cases of hepatic encephalopathy (HE) and recurrence of variceal bleedings. Acute shunt occlusion was found in one patient in each group. Follow-ups were performed in group A with average time of 6.16 +/- 3.89 months and in group B with 8.34 +/- 4.42 months. The rates of recurrent bleeding, shunt occlusion, HE and mortality were 0.03, 0.0, 16.7 and 0% in group A, and 20.0, 30.0, 20.0 and 13.3% in group B, respectively. There was no difference of HE between group A and group B. The decrease of portal pressure and portosystemic pressure gradient, and the increase of portal flow were 34.1 and 23.3%, 60.0 and 52.8%, and 189.5 and 111.1% in group A and B, respectively. There were no differences of liver function between group A and B. The Fluency stent graft is relatively safe and effective in TIPS creation, with a high patency rate compared with bare stents.

The retrospective study of transjugular intrahepatic portosystemic shunt with covered or uncovered stents in patients with portal hypertension

To retrospectively compare the clinical outcome in patients with portal hypertension treated with transjugular intrahepatic portosystemic shunt (TIPS) using Fluency stent-graft (PTFE-covered stents) or bare stents. Approval of study and treatment protocol and waiver of informed consent for the retrospective study were obtained from institutional review board. Informed consent was obtained from each patient before procedure. Sixty consecutive patients with portal hypertension treated with TIPS from April 2007 to April 2009 were included. TIPS creation was performed with Fluency stent-graft in 30 patients (group A) and with bare stents in 30 patients (group B). Liver function, TIPS patency and clinical outcome were evaluated every 3 months. During hospitalization, there was no hepatic encephalopathy and recurrency of variceal bleeding.Acute shunt occlusion was observed in one patient with group A and another patient with group B.Follow-up was performed with average time of (6.2 +/- 3.9) months in group A and (8.3 +/- 4.4) months in group B. The rates of recurrent bleeding, acute shunt occlusion, hepatic encephalopathy and death were 3.3% and 20.0%, 0 and 30.0%, 16.7% and 20.0%, 0 and 13.3% in group A and B. The rates of recurrent bleeding, acute shunt occlusion and death in group A was lower than those in group B. There was no difference of hepatic encephalopathy between group A and B. The decrease of portal pressure and portosystemic pressure gradient, and the increase of portal flow and shunt flow in group A were higher than those in group B. There were no difference of liver function, ammonia and MELD between group A and B. Fluency stent-graft is safe and effective in TIPS creation, with high patency rate. Covered-stent can improve the clinical outcome of portal hypertension.

Combined TIPS with portal-azygous disconnection improved the long term clinical outcome in portal hypertension patients

The results of TIPS and the combined TIPS and portal-azygous disconnection for portal hypertension and variceal bleeding were evaluated. 358 patients with portal hypertension were admitted to our clinical ward because of variceal bleeding. 263 patients underwent TIPS and 95 patients with combined TIPS and portal-azygous disconnection. Portal hemodynamics was evaluated by pressure measurements, venography and Doppler ultrasound before and 2 weeks after the procedure. The rates of shunt patency, rebleeding, encephalopathy and survival were observed during the follow-up period from 1 to 10 years. The portal pressure and HVPG were decreased significantly after TIPS. TIPS procedure was successfully performed in 97.50% patients. During 1 month after treatment, acute shunt occlusion occurred in 3.42% patients with TIPS and there were no occluded shunts in patients with combined TIPS and portal-azygous disconnection. Encephalopathy was observed in 36.50% patients with TIPS and 18.95% with combined TIPS and portal-azygous disconnection. Recurrent variceal bleeding was documented in 6.46% patients with TIPS and none of patients with combined TIPS and azygous portal disconnection. Thirty-three patients with TIPS and two patients with combined TIPS and portal-azygous disconnection died. During follow-up periods, the patency of shunts in patients with TIPS and patients combined TIPS and azygous portal disconnection was 68.47, 43.84 and 87.06, 57.65% in 12 and 24 months after operation, respectively. The rates of rebleeding, and encephalopathy in patients with TIPS and patients with combined TIPS and azygous portal disconnection were 17.95, 31.79 and 7.04, 16.47%, respectively. The survival rate in 1, 5, 10 years in patients with TIPS and patients combined TIPS and azygous portal disconnection was 87.68, 51.23, 39.90 and 94.12, 81.18, 76.47%. Combined TIPS and portal-azygous disconnection can improve the effect of TIPS for portal hypertension.

Transcatheter recanalization of acutely occluded modified systemic to pulmonary artery shunts in infancy

There are few data on successful recanalization of acutely occluded modified aortopulmonary shunts in infants in whom the major component of pulmonary flow is shunt-dependent. Seven infants (age range 6 days to 7 months) with modified (polytetrafluoroethylene) aortopulmonary shunts (central shunt n = 6, modified Blalock-Taussig shunt n = 1), in whom acute shunt occlusion was documented following surgery (interval from shunt surgery 2-6 days) underwent emergency cardiac catheterization with a view to reopening the shunt. Anterograde right ventricle to pulmonary artery flow was present in three patient; two other had additional aortopulmonary collateral vessels, and intravenous prostaglandin E (PGE1) infusion was restarted in three infants to achieve or maintain ductal patency. All occluded shunts could be crossed with a 4F endhole catheter and various guidewire combinations. Balloon dilation was performed using a balloon with the same nominal diameter as the shunt (5 or 6 mm). All shunts were successfully recanalized. Two infants with recurrent cyanotic episodes underwent stent implantation in the right ventricular outflow tract (RVOT) during the same procedure. One infant required shunt revision 24 h following balloon angioplasty, during which a small clot was evacuated from the distal end of the shunt. An immediate improvement in oxygen saturation could be documented in all patients. In the remaining six infants, shunt patency was confirmed at follow-up, until further definitive repair. One infant died of non-cardiac causes, while awaiting definitive repair. This small series demonstrates that acutely occluded shunts can be successfully recanalized, and that patency can be maintained until further definitive surgery in the majority of infants.

Venoarterial Extracorporeal Membrane Oxygenation (VA-ECMO) in Pediatric Cardiac Support

Resuscitation extracorporeal membrane oxygenation (R-ECMO) was introduced at our institution in July 2002. We reviewed the use of venoarterial (VA)-ECMO for cardiac diagnoses at our institution. Retrospective analysis of patients on VA-ECMO for cardiac failure was performed. Survival was defined as discharge from hospital. Twenty-seven patients were supported with VA-ECMO (median age, 27 days; range, 1 to 640 days; median weight, 3.8 kg; range, 1.8 to 11.3 kg). Diagnoses were cardiomyopathy-myocarditis (CMM) in 8 (30%), systemic-to-pulmonary artery shunt-dependent single ventricle (SV) in 12 (44%), postcardiotomy for biventricular repair (BiV) in 6 (22%), and arrhythmia in 1 (4%). Sixteen of 27 patients survived (59%). Seven of 8 CMM patients survived (88%); 6 (75%) bridged to cardiac recovery, 1 to transplant (13%), and 1 death (13%). Seven of 12 SV patients survived (58%). The SV ECMO indications: post-Norwood ventricular dysfunction (n = 3, 2 deaths), postoperative cardiac failure (n = 6, 2 deaths), respiratory failure (n = 1, 1 death), and acute shunt occlusion (n = 2, 0 deaths). One of 6 BiV patients survived (17%). The BiV ECMO indications: failure to wean from CPB (n = 3, 3 deaths), postoperative cardiac failure (n = 2, 2 deaths), and pulmonary hypertension (n = 1, 0 deaths). Fifteen patients (56%) underwent cardiopulmonary resuscitation during ECMO cannulation. Eleven of 15 R-ECMO patients (73%) survived versus 5 of 12 non-R-ECMO patients (42%, p = 0.13). Median duration of R-ECMO: 66 hours (range, 18 to 179) versus 145 hours (range, 43 to 986, p = 0.01) for non-R-ECMO. Resuscitation extracorporeal membrane oxygenation is an appropriate application in pediatric patients with cardiac disease. Single ventricle patients experiencing cardiopulmonary collapse and CMM patients have favorable outcomes. Failure to wean from CPB and postoperative ventricular failure are higher risk indications.

Transjugular Intrahepatic Portosystemic Shunts through Hepatic Neoplasms

Transjugular intrahepatic portosystemic shunts (TIPS) were successfully placed through hepatic malignancy, without technical complication, in nine patients. A mean follow-up of 229 days (range, 53-391 days) was available in six patients. Ascites improved in three patients and variceal hemorrhage did not recur in another three patients. Hepatic encephalopathy occurred in three patients. Three acute shunt occlusions and two deaths occurred within 30 days of TIPS placement. Shunts that traverse malignancy can be created safely without increased technical complication. Although there is a high rate of acute shunt occlusion, hepatic neoplasm alone should not be a contraindication for TIPS placement.