The outcome of extracardiac lateral tunnel total cavopulmonary connection with growing conduit using expanded polytetrafluoroethylene graft.

Between July 1991 and March 1993, five children (ages 2 to 6 years) with complex congenital heart disease have undergone a new operation for conversion to the Fontan circulation. This procedure combines a bidirectional Glenn shunt with an extracardiac lateral tunnel (ELT) to carry systemic venous return to the pulmonary arteries (PAs). The ELT was constructed so that the circumference consists of Gore-Tex (2/3) and lateral epicardial atrial wall (1/3). The ELT can be performed with all varieties of single ventricle physiology, as in our patients with tricuspid atresia (n = 3), dextrocardia (n = 1), and situs inversus with levocardia (n = 1). PA reconstruction was required in four patients. At follow-up from 1 to 20 months, all patients are in New York Heart Association Class I and in normal sinus rhythm. Postoperative catheterization has revealed low PA pressures (< or = 12 +/- 1 mmHg) and angiography has shown excellent ELT function with brisk flow into the PAs bilaterally. All patients maintain an O2 saturation > 94% on room air. The advantages of this new extracardiac modification of Fontan's operation are: (1) aortic cross-clamping is not usually required; (2) incorporation of lateral atrial wall in ELT allows for growth while permitting construction of a fenestration or adjustable atrial septal defect in high risk patients; (3) absence of atriotomy and intraatrial suture lines may decrease late risk of arrhythmias; (4) early or late baffle leaks cannot occur; (5) intraatrial obstruction from the baffle cannot occur; (6) coronary sinus remains in low pressure atrium; and (7) hydrodynamic benefits of the total cavopulmonary connection are preserved. We recommend this procedure for patients undergoing surgical conversion to the Fontan circulation.

Novel Modification of Total Cavopulmonary Connection for Isolated Hepatic Vein

The long-term outcome of extra cardiac lateral tunnel total cavopulmonary connection

The optimal Fontan connection: A growing extracardiac lateral tunnel with pedicled pericardium

Extracardiac modification of the Fontan operation without use of prosthetic material

Fontan operation with a viable and growing conduit using pedicled autologous pericardial roll: Serial changes in conduit geometry

Objective To summarize the experience of single or 2-stage total cavo-pulmonary connection (TCPC) to correct the complex congenital heart diseases in children. Methods From December 2003 to November 2009, 88 patients underwent TCPC at this center. Among them, 58 were male and 30 were female. Surgical risks were assessed before surgery. The 41 patients with low surgical risks underwent single stage TCPC, and the other 47 high risk patients were performed 2-stage TCPC. Among the single stage TCPC group,extracardiac conduits were performed on 27 patients,and direct anastomosis between pulmonary trunk and inferior vena cava on the other 14 patients. Among the patients performed 2-stage TCPC, extracardiac conduits were performed on 42 patients, intracardiac conduit on 3, and direct anastomosis between pulmonary trunk and inferior vena cava on the rest 2 patients. Results Six patients died after surgery with overall mortality of 6. 8%,including 5 with single stage TCPC patients (mortality, 12. 2%) and 1 with 2-stage TCPC patient (mortality,2. 1 %). Among the five deaths with single stage TCPC,4 died for severe low cardiac output syndrome,and the other 4 died for respiratory failure. The 1 death with 2-stage TCPC died for severe bleeding when opening the chest during the operation. No significant differences of mechanical ventilating time, duration of chest tube, ICU duration, and post-operative hospital stay were noted between the 2 groups. The 65 survived patients were followed up from 4. 0 months to 6. 5 years. On patient died for sever atrialventricular valve regurgitation and heart failure 8 months later after the surgery. One patient died for multiple organs failure caused by infective endocarditis. Conclusions This study suggests the short-term outcomes of 2-stage TCPC are better than the single stage TCPC. The 2-stage TCPC is recommended to all the patients especially to the patients with 2 or more risk factors. Key words: Heart defects,congenital; Total cavopulmonary connection; Cardiac surgical procedures

Computational fluid dynamics in the evaluation of hemodynamic performance of cavopulmonary connections after the norwood procedure for hypoplastic left heart syndrome

To date, it is not known if 16-20-mm extracardiac conduits are outgrown during somatic growth from childhood to adolescence. This study aims to determine total cavopulmonary connection (TCPC) haemodynamics in adolescent Fontan patients at rest and during simulated exercise and to assess the relationship between conduit size and haemodynamics. Patient-specific, magnetic resonance imaging-based computational fluid dynamic models of the TCPC were performed in 51 extracardiac Fontan patients with 16-20-mm conduits. Power loss, pressure gradient and normalized resistance were quantified in rest and during simulated exercise. The cross-sectional area (CSA) (mean and minimum) of the vessels of the TCPC was determined and normalized for flow rate (mm2/l/min). Peak (predicted) oxygen uptake was assessed. The median age was 16.2 years (Q1-Q3 14.0-18.2). The normalized mean conduit CSA was 35-73% smaller compared to the inferior and superior vena cava, hepatic veins and left/right pulmonary artery (all P < 0.001). The median TCPC pressure gradient was 0.7 mmHg (Q1-Q3 0.5-0.8) and 2.0 (Q1-Q3 1.4-2.6) during rest and simulated exercise, respectively. A moderate-strong inverse non-linear relationship was present between normalized mean conduit CSA and TCPC haemodynamics in rest and exercise. TCPC pressure gradients of ≥1.0 at rest and ≥3.0 mmHg during simulated exercise were observed in patients with a conduit CSA ≤ 45 mm2/l/min and favourable haemodynamics (<1 mmHg during both rest and exercise) in conduits ≥125 mm2/l/min. Normalized TCPC resistance correlated with (predicted) peak oxygen uptake. Extracardiac conduits of 16-20 mm have become relatively undersized in most adolescent Fontan patients leading to suboptimal haemodynamics.

Bovine valved xenograft conduits in the extracardiac Fontan procedure

Hemodynamic Consequences of an Undersized Extracardiac Conduit in an Adult Fontan Patient Revealed by 4-Dimensional Flow Magnetic Resonance Imaging.

“The treatment of congenital heart disease is unsatisfactory. As a rule, nothing can be done to improve patients symptomatically; in some instances digitalis may be of help.” — —L. Emmett Holt, MD, 1933 On August 26, 1938, Dr Robert Gross performed the first successful surgery for a congenital heart defect by closing a patent ductus arteriosus in a 7-year-old girl. The event marked the beginning of an interventional approach to congenital heart disease (CHD) that forever banished the sort of pessimism expressed by Dr Holt in his classic pediatric textbook only 5 years earlier.1 Subsequent diagnostic and operative innovations ultimately led to surgical solutions for nearly all anatomic heart defects, allowing the vast majority of infants born with CHD in the modern era to survive into adulthood. However, improved hemodynamic longevity has exposed alternate sources of morbidity and mortality for this population, central among which are cardiac rhythm disorders. In some instances, arrhythmias are intrinsic to the CHD lesion itself, but in most cases, they arise as an unintended consequence of prior corrective surgery whenever patches and suture lines function in conjunction with hypertrophy and fibrosis to create the substrate for reentrant tachycardias. It is fortunate that improved understanding of rhythm abnormalities in CHD has coincided with the emergence of interventional electrophysiological techniques. Catheter ablation, arrhythmia surgery, pacemakers, and implantable defibrillators have now become indispensable treatment options for this group. It is the purpose of this article to review the application of these tools to CHD with an emphasis on unique technical challenges. Congenital heart defects occur in roughly 0.8% of live births, and in 30% to 50% of cases, the malformations are severe enough to warrant ≥1 surgical procedures during early childhood.2 Arrhythmia mechanisms vary according to the underlying anatomic defect and method of surgical repair. The …

BackgroundVery few studies have investigated the blood flow velocity from the inferior vena cava (IVC) to the pulmonary artery following the Fontan operation using an extra-cardiac conduit (ECC). No studies at all have investigated the velocity immediately after the circulation is established. The purpose of this retrospective study was to find an acceptable flow velocity at the ECC following the completion of a total cavo-pulmonary connection (TCPC) via transesophageal echocardiography.FindingsWe measured the mean velocity (m-V) of the blood flow proximal to the anastomosis between the IVC and ECC in eight patients and compared the results with theoretically predicted values based on assumptions regarding the cardiac output, the ratio of the IVC flow to the superior vena cava flow, and the cross-sectional form of the ECC. Mean velocities ranging from about 15 to 60 cm/s were detected in the absence of any observable stenosis. The measured m-V was significantly faster than the predicted value in our study, both collectively and in every patient individually. The shrinking and compression of the ECC might account for the faster velocities measured in our cases.ConclusionThe observed range of m-V at the ECC, about 15-60cm/s, may be acceptable for the establishment of TCPC circulation.

Total cavopulmonary connection with an extracardiac conduit: experience with 100 patients

PurposeDetails on the hemodynamic differences among Fontan operations remain unclear according to respiratory and cardiac cycles. This study was undertaken to investigate hemodynamic characteristics in different types of Fontan circulation by quantification of blood flow with the combined influence of cardiac and respiratory cycles.Materials and MethodsThirty-five patients [10 atriopulmonary connections (APC), 13 lateral tunnels (LT), and 12 extracardiac conduits (ECC)] were evaluated, and parameters were measured in the superior vena cava, inferior vena cava (IVC), hepatic vein (HV), baffles, conduits, and left and right pulmonary artery. Pulsatility index (PIx), respiratory variability index (RVI), net antegrade flow integral (NAFI), and inspiratory/expiratory blood flow (IQ/EQ) were measured by intravascular Doppler echocardiography.ResultsThe PIx between APC and total cavopulmonary connection (TCPC; LT and ECC) showed significant differences at all interrogation points regardless of respiratory cycles. The PIxs of HVs and IVCs in APC significantly increased, compared with that in LT and ECC, and the RVI between APC and TCPC showed significant differences at all interrogation points (p<0.05). The NAFI and IQ/EQ between APC and TCPC showed significant differences at some interrogation points (p<0.05).ConclusionPatients with different types of Fontan circulation show different hemodynamic characteristics in various areas of the Fontan tract, which may lead to different risks causing long-term complications. We believe the novel parameters developed in this study may be used to determine flow characteristics and may serve as a clinical basis of management in patients after Fontan operations.