Objective: To assess the safety and efficacy of transcatheter fenestration closure following Fontan procedure with an atrial septal occluder. Methods: This is a retrospective study. The study sample consists of all consecutive patients who underwent closure of a fenestrated Fontan baffle at Shanghai Children's Medical Center Affiliated to Shanghai Jiaotong University School of Medicine between June 2002 and December 2019. The indications of Fontan fenestration closure included that normal ventricular function, targeted drugs for pulmonary hypertension and positive inotropic drugs were not required prior the procedure; and the Fontan circuit pressure was less than 16 mmHg (1 mmHg=0.133 kPa) and no more than a 2 mmHg increase during test occlusion of the fenestration. Electrocardiogram and echocardiography were reviewed at 24 hours, 1, 3, 6 months and annually thereafter post procedure. Follow-up information including clinical events and complications related to Fontan procedure was recorded. Results: A total of 11 patients, including 6 males and 5 females, aged (8.9±3.7) years old were included. The types of Fontan were extracardiac conduits (7 cases) and intra-atrial duct (4 cases). The interval between percutaneous fenestration closure and the Fontan procedure was (5.1±2.9) years. One patient reported recurrent headache after Fontan procedure. Successful fenestration occlusion with atrial septal occluder was achieved in all patients. Compared with prior closure, Fontan circuit pressure ((12.72±1.90) mmHg vs. (12.36±1.63) mmHg, P<0.05), and aortic oxygen saturation ((95.11±3.11)% vs. (86.35±7.26)%, P<0.01) were increased. There were no procedural complications. At a median follow-up of (3.8±1.2) years, there was no residual leak and evidence of stenosis within the Fontan circuit in all patient. No complication was observed during follow-up. One patient with preoperative headache did not have recurrent headache after closure. Conclusions: If the Fontan pressure is acceptable by test occlusion during the catheterization procedure, Fontan fenestration could be occluded with the atrial septum defect device. It is a safe and effective procedure, and could be used for occlusion of Fontan fenestration with varying sizes and morphologies.
Child ; Male ; Female ; Humans ; Child, Preschool ; Septal Occluder Device ; Retrospective Studies ; Atrial Fibrillation ; Cardiac Catheterization/methods* ; China ; Fontan Procedure/methods* ; Treatment Outcome

The management of pulmonary atresia with intact ventricular septum (PA/IVS) remains controversial. The goal of separating systematic and pulmonary circulation can be achieved by biventricular or uni-ventricular (Fontan or one and a half ventricle repair) strategies. Although outcomes have been improved, these surgical procedures are still associated with high mortality and morbidity. An optimal strategy for definitive repair has yet to be defined. We searched databases for genetically randomized controlled trials (RCTs) comparing biventricular with uni-ventricular repair for patient with PA/IVS. Data extraction and quality assessment were performed following the guidelines of the Cochrane Collaboration. Primary outcome measures were overall survival, and secondary criteria included exercise function, arrhythmia-free survival and treatment-related mortality. A total number of 669 primary citations were screened for relevant studies. Detailed analysis revealed that no RCTs were found to adequately address the research question and no systematic meta-analysis would have been carried out. Nevertheless, several retrospective analyses and case series addressed the question of finding right balance between biventricular and uni-ventricular repair for patient with PA/IVS. In this review, we will discuss the currently available data.
Arrhythmias, Cardiac ; physiopathology ; prevention & control ; Cardiac Valve Annuloplasty ; methods ; mortality ; Fontan Procedure ; methods ; mortality ; Heart Defects, Congenital ; mortality ; pathology ; surgery ; Heart Ventricles ; abnormalities ; pathology ; surgery ; Humans ; Pulmonary Atresia ; mortality ; pathology ; surgery ; Retrospective Studies ; Survival Analysis ; Treatment Outcome

BACKGROUNDThe present study was aimed to compare the effects of bilateral and unilateral bidirectional superior cavopulmonary shunt (b-BDG and u-BDG) on pulmonary artery growth and clinical outcomes.

METHODSThe 51 subjects enrolled in this study were divided into two groups: those receiving b-BDG (n = 21) and those receiving u-BDG (n = 30). Clinical records were reviewed retrospectively at a mean of 43.3 months after BDG procedures. Chi square and t-tests were performed to analyze the data.

RESULTSLeft and right pulmonary artery diameters increased 27% - 37% in both groups. The pulmonary artery index increased 37.2% after b-BDG and 27.0% after u-BDG. b-BDG patients experienced a significant decrease in mean hemoglobin concentration and hematocrit value, and a correlated change in postoperative diameter of left pulmonary artery (LPA) and pulmonary artery index (y = 0.2719, x = -1.8278; R = 0.564, P = 0.008). The change ratio of hemoglobin and postoperative LPA were also correlated in b-BDG patients (y = -0.0522x + 0.3539; R = -0.479, P = 0.028). Only one b-BDG patient versus twelve u-BDG patients needed total cavopulmonary connections 31.8 months after BDG surgery (P = 0.0074). Moreover, only one (4.8%) b-BDG patient but eight u-BDG patients (26.7%) developed pulmonary arteriovenous malformations.

CONCLUSIONSb-BDG increases bilateral pulmonary blood flow and promotes growth of bilateral pulmonary arteries, with preferable physiological outcomes to u-BDG. Results may imply that subsequent Fontan repair may not always be needed.

Cardiopulmonary Bypass ; methods ; Child ; Child, Preschool ; Female ; Fontan Procedure ; methods ; Heart Defects, Congenital ; physiopathology ; surgery ; Humans ; Infant ; Lung ; pathology ; surgery ; Male ; Pulmonary Circulation ; Retrospective Studies ; Treatment Outcome

BACKGROUNDThe fenestration function is by allowing a right-to-left shunt resulting in an increased cardiac index, associated with mild arterial oxygen desaturation. Subsequent transcatheter fenestration closure can be performed after haemodynamic assessment. The purpose of this study was to compare the outcomes of extracardiac connection (EC) with or without fenestration.

METHODSNinety-five consecutive patients diagnosed with univentricular heart disease underwent EC using Gore-Tax conduits at the Department of Children's Heart Center, Justus-Liebig-University Giessen Germany from June 1996 to July 2007. According to EC with or without fenestration, the patients were assigned to two groups (group A with fenestration and group B without fenestration). Mortality, effusions, postoperative mean pulmonary artery pressure, postoperative oxygen saturation, postoperative thrombosis, postoperative neurological problems, and the postoperative loss of sinus rhythm were compared. In group A, 23 patients had fenestration closed interventionally after a mean time of 20-22 months.

RESULTSMortality and postoperative mean pulmonary artery pressure in group B (3 and (15.1 +/- 3.4) mmHg, respectively) were significantly higher than group A (0 and (13.2 +/- 2.8) mmHg, respectively). Postoperative oxygen saturation, postoperative thrombosis, postoperative neurological problems, and the postoperative loss of sinus rhythm did not differ between cohorts.

CONCLUSIONSFenestrating an extracardiac tunnel seems to improve acute postoperative mortality by rising cardiac output. The induced right-to-left shunt shows no morbidity postoperatively. If a stabilized chronic hemodynamic situation is achieved, an interventional closure of the fenestration can be performed to advance the arterial saturation and improve the exercise tolerance of the patients.

Adolescent ; Child ; Child, Preschool ; Fontan Procedure ; adverse effects ; methods ; mortality ; Heart Defects, Congenital ; physiopathology ; surgery ; Humans ; Infant ; Pulmonary Artery ; physiopathology

OBJECTIVENoninvasive method for estimating the pulmonary vascular resistance (PVR) was used in patients waiting for two staged Fontan procedure to observe the relationship between estimated PVR and surgical results.

METHODSThirty-three candidate patients for two staged Fontan procedures were randomly selected for this trial. Preoperative hemoglobin (HGB), hematocrit (HCT) and pulse oxygen saturation (SpO2) were measured. Estimated PVRs were then calculated by the regression equations. The cases were divided into three groups of low risk group with PVR less than 2.4 wood, high risk group with PVR between 2.4 to 3.2 wood, and extremely high risk group with PVR more than 3.2 wood. The incidences of postoperative low cardiac output and multi-organ failure were compared. Simultaneously, the cases without or with postoperative severe low cardiac output and multi-organ failure after operation were divided into two groups. The preoperative HGB, HCT and estimated PVR among the groups were analyzed.

RESULTSThe rates of postoperative severe low cardiac output and multi-organ failure were 10.0%, 32.4% and 63.6% for the patients of low, high and extremely high risk groups respectively. The difference was significant (P < 0.01). The preoperative HGB, HCT, SpO2 and PVR were all significantly different between the patients without or with postoperative severe low cardiac output and multi-organ failure. The PVR (HGB) were (2.53 +/- 0.56) wood and (3.24 +/- 0.58) wood respectively. The PVR (HCT) were (2.59 +/- 0.58) wood and (3.21 +/- 0.79) wood respectively. The PVR (SpO2) were (2.22 +/- 0.55) wood and (2.93 +/- 0.58) wood, respectively. The differences were all significant (t = 3.25, 2.52 and 3.33 respectively, P < 0.01 or 0.05).

CONCLUSIONSPreoperative estimated PVRs calculated by HGB, HCT and SpO2 were comparable with the postoperative results. Thus, the invasive preoperative estimation of pulmonary vascular resistance could be a method in estimating the surgical indication and predicting the surgical result for two stage Fontan procedure.

Adolescent ; Cardiovascular Abnormalities ; physiopathology ; surgery ; Child ; Child, Preschool ; Contraindications ; Female ; Fontan Procedure ; methods ; Humans ; Male ; Treatment Outcome ; Vascular Resistance

Single ventricle disease is a complex congenital heart anomaly with a high operative mortality. In the past few years, much progress had been made in surgical treatment of single ventricle. However, some problems in the diagnosis and treatment still remained. Based on the author's experience, the concept, morphology, and pathophysiology were reviewed and indications of various surgical techniques, problems during the operation and post-operation with their strategies were discussed in this article. Most single ventricular diseases are treated by Fontan series surgery; in which better results can be achieved through extra-cardiac conduct. Ventricular separation which is better than Fontan series surgery can be performed on those qualified. If the pulmonary vessels are maldeveloped, systematic to pulmonary shunt is preferred to promote the development of pulmonary vessels. When pulmonary hypertension occurs, Banding procedure is suggested to prevent pathologic changes of pulmonary vessels. The development of pulmonary vessels and total pulmonary resistance varies greatly from patients at different ages. Choosing appropriate patients is the key to obtain satisfactory outcome.The pulmonary vessel resistance and cardiac function status are the most important factors for successful operation.
Cardiac Surgical Procedures ; methods ; Child ; Child, Preschool ; Female ; Fontan Procedure ; methods ; Heart Defects, Congenital ; surgery ; Heart Ventricles ; abnormalities ; surgery ; Humans ; Infant ; Male ; Pulmonary Artery ; surgery

OBJECTIVETo analyze 68 pediatric cases with functional univentricle heart who underwent bidirectional Glenn procedure during from April 1998 to December 2005.

METHODSThere were 47 males and 21 females in this group, aged from 5 months to 14 years old and weighed from 6.7 to 30.0 kg. Among them, 39 cases were received bidirectional Glenn procedure on the right side, 13 cases on the left side and 16 cases on both sides. Three cases had the pulmonary artery banded; one case had the pulmonary artery ligated;one case had the original A-P shunt cut off; six cases had the PDA ligated; four cases had the MAPCAs cut off; one case had TAPVC corrected contemporarily; two cases of PAPVC were also corrected; four cases had the atrial-ventricular valve repaired.

RESULTSThree cases died. The mortality was 4.4%. The mean post-operative pressure of super vena cava was (15.9 +/- 2.4) mm Hg (1 mm Hg = 0.133 kPa), higher than the pre-operative one (8.3 +/- 1.8) mm Hg (P < 0.01). The mean post operative SpO(2) was (89.3 +/- 4.2)%, higher than the pre-operative one (78.4 +/- 6.0)% (P < 0.01).

CONCLUSIONSBidirectional Glenn procedure is of satisfied effect on surgical treatment for functional univentricle heart. The persistent forward flow from pulmonary artery should be reserved in bidirectional Glenn procedure.

Adolescent ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Fontan Procedure ; methods ; Heart Defects, Congenital ; surgery ; Humans ; Infant ; Male ; Retrospective Studies ; Tricuspid Atresia ; surgery

OBJECTIVETo study cardiopulmonary physiology during exercise in patients after extracardiac total cavopulmonary connection (ECTCPC).

METHODSTwenty-six patients were studied after ECTCPC by exercise testing with bicycle treadmill protocol. Heart rate (HR), blood pressure (BP), respiratory frequency (RF) and pulse oxygen saturation (SpO(2)) were measured continuously; twenty-six patients suffered from Fallot 4 underwent biventricular repair were also studied as control group.

RESULTSIn ECTCPC group, HR, BP, SpO(2) and RF all increased with exercise below 3 grade; when exceed 4 grade, BP, SpO(2) decreased and RF kept increasing. Compared with control group, HR, RF were higher (t = 2.13, P < 0.05; t = 2.31, P < 0.05), SpO(2) was lower (t = 2.46, P < 0.05) under the quiescent condition; When exceed 3 grade, HR, BP, SpO(2) decreased more significantly, but RF increased continuously. In fenestration group after ECTCPC, HR reached the top at 5 grades, but in group without fenestration it reached the top at 3 grades; In the whole process of exercising, RF kept higher and SpO(2) kept lower in fenestration group.

CONCLUSIONSThe ECTCPC patients showed obviously exercise limitation. Totally bypass of sinoatrial node in this operation may have some adverse effects on the integer regulation of HR.

Adolescent ; Adult ; Child ; Child, Preschool ; Exercise Test ; Female ; Follow-Up Studies ; Fontan Procedure ; methods ; Heart Defects, Congenital ; physiopathology ; surgery ; Humans ; Male ; Physical Endurance ; physiology ; Postoperative Period

OBJECTIVETo report the experience of extracardiac conduit total cavopulmonary connection (ECTCPC) in surgical treatment of complex congenital heart diseases.

METHODSFrom 1998 to 2006, 68 patients underwent ECTCPC for complex congenital heart diseases. Among them, 45 had functional univentricle with transposition of the great artery (TGA) and pulmonary artery valve stenosis, 19 had tricuspid atresia with hypoplasia of right ventricle, 4 had Ebstein's anomaly with hypoplasia of right ventricle. Six had left superior vena cava, 18 had received Bidirectional Glenn operation; Fifty-seven cases were performed under cardiopulmonary bypass with general anesthesia and hypothermia, 11 cases were performed without cardiopulmonary bypass.

RESULTSThere were two death, the mortality was 2.9%. All patients were followed up from 1 to 8 years with no clinical symptoms and have been doing well. The arterial oxygen saturation was 90% - 96%, the cardiac function were in NYHA class I - II.

CONCLUSIONThe extra cardiac conduit TCPC is a simple procedure and superior to other type of Fontan procedure in most patients.

Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Fontan Procedure ; methods ; Heart Defects, Congenital ; surgery ; Humans ; Male ; Treatment Outcome

We report our experience in 13 patients who underwent transcatheter closure of Fontan fenestration with the Cook(R) detachable coils. These patients underwent the extracardiac type Fontan operation with a short conduit fenestration (n=7) or lateral tunnel type with a punch-hole fenestration (n=6). Fenestration closure was done at the mean age of 5.1+/-2.4 yr, average of 32 months after the Fontan operation. We used one to three coils depending on the fenestration type, size, and residual shunt. Aortic oxygen saturations increased by an average of 5.4 (2-9)% and mean pressures in the Fontan circuit increased by an average of 2.1 (0-6) mmHg. During fol-low-up (median of 23 months), five patients (4 in extracardiac, 1 in lateral tunnel) had complete occlusion of the fenestration on echocardiography. There was no immediate or late complication. Transcatheter closure of fenestration in Fontan operation using the Cook(R) detachable coil is a safe and feasible technique. However, the coil was ineffective for closure of a punch-hole fenestration in the lateral tunnel type operation. In the conduit type fenestration, some modification of fenestration method instead of a short conduit for coil closure or use of new device is necessary to increase complete closure rate.
*Prostheses and Implants ; Male ; Humans ; *Heart Catheterization ; Fontan Procedure/instrumentation/*methods ; Female ; Child, Preschool ; Child