Heart transplantation after Fontan: Results from a surgical Fontan cohort.

We performed a retrospective review of outcomes after heart transplantation during long-term follow-up of a surgical cohort of 1138 Fontan patients who were followed at the Mayo Clinic. Follow-up information was obtained from medical records and a clinical questionnaire that was mailed to patients not known to be deceased at the initiation of the study. Forty-four of 1138 Fontan patients with initial or subsequent evaluation at Mayo had cardiac transplantation between 1988 and 2014 (mean age at transplantation was 23.2 ± 12 yr, median was 19.8 yr; mean interval between Fontan and transplantation was 13.0 ± 7.7 yr, median was 13.1 yr). Two patients had combined organ transplantation (one heart-lung, one heart-liver). Twelve of the 44 (27%) patients had PLE prior to transplantation. There was no difference in post-bypass Fontan pressures or incidence of late reoperations for AVV repair/replacement between transplanted and non-transplanted patients. There were 16 (36%) deaths in the transplantation cohort; seven occurred within 30 days of transplantation. Overall one, five, 10, and 15 yr post-transplantation survival was 80%, 72%, 69%, and 55%, respectively. Although this is a challenging group of patients, intermediate-term results suggest that cardiac transplantation remains a reasonable option for patients with a failed Fontan circulation.

Novel Applications of Modified Ultrafiltration and Autologous Priming Techniques to Reduce Blood Product Exposure on ECMO.

Patients needing the assistance of extracorporeal membrane oxygenation (ECMO) are at risk of hemodilution and, in some instances, may require exposure to large amounts of allogeneic blood products. Patient outcomes can be improved by taking steps to reduce transfusions and hemodilution. Currently, modified ultrafiltration (MUF) is used across the world to reduce hemodilution after cardiopulmonary bypass (CPB). Another common technique during bypass initiation is autologous priming. By applying modified versions of these techniques, ECMO patients may potentially benefit. Usually, patients requiring immediate transition from CPB to ECMO are not stable enough to tolerate MUF. Through alterations of the CPB and ECMO circuit tubing, MUF can be performed once on ECMO. Another technique to potentially lower the transfusion requirements for ECMO patients is a complete circuit blood transfer during an ECMO circuit exchange. While selective component changes are preferred if possible, occasionally a complete circuit change must be done. To minimize hemodilution or prevent priming with blood products, the original ECMO circuit's blood can be transferred to the new ECMO circuit before connecting to the patient. Both of these techniques, in our opinion, helped to reduce the number of transfusions that our ECMO patients have seen during these critical time periods.

Mechanical Pulmonary Valve Replacement.

Although most valve operations performed annually address lesions of the aortic or mitral valves, the frequency of pulmonary valve replacement (PVR) is increasing because most patients with congenital heart disease are surviving into the adult years. The vast majority of patients, especially children that require PVR, obtain a tissue valve because of the relative good durability and the lack of a need for anticoagulation. Because the need for repeat operation is inevitable for most patients, and the population of adults with congenital heart disease continues to grow, there are increasing situations in which a mechanical pulmonary prosthesis may be appropriate. Most patients being considered for mechanical PVR have a congenital diagnosis and require multi-valve procedures, and quality of life and need for repeat operation(s) are major issues. Mechanical valves are durable but require anticoagulation, which carries its own inherent set of risks. There are conflicting reports regarding the late outcome of mechanical PVR. There are few reports that indicate that, in the pulmonary position, bileaflet valves are at higher risk for complications compared with monodisc valves; however, the majority of these patients were not anticoagulated with warfarin, but simply maintained on aspirin. There is a growing body of literature documenting low rates of thrombosis or pulmonary prosthesis dysfunction when proper anticoagulation and monitoring are applied.

Ebstein anomaly review: what's now, what's next?

Ebstein anomaly accounts for 1% of all congenital heart disease. It is a right ventricular myopathy with failure of tricuspid valve delamination and highly variable tricuspid valve morphology that usually results in severe regurgitation. It is the only congenital heart lesion that has a range of clinical presentations, from the severely symptomatic neonate to an asymptomatic adult. Neonatal operation has high operative mortality, whereas operation performed beyond infancy and into adulthood has low operative mortality. Late survival and quality of life for hospital survivors are excellent for the majority of patients in all age brackets. Atrial tachyarrhythmias are the most common late complication. There have been more techniques of tricuspid repair reported in the literature than any other congenital or acquired cardiac lesion. This is largely due to the infinite anatomic variability encountered with this anomaly. The cone reconstruction of Ebstein anomaly can achieve near anatomic restoration of the tricuspid valve anatomy. Early and intermediate results with these repairs are promising. Reduced right ventricular function continues to be a challenge for some patients, as is the need for reoperation for recurrent tricuspid regurgitation. The purpose of this article is to outline the current standard of care for diagnosis and treatment of Ebstein anomaly and describe innovative strategies to address poor right ventricular function and associated right-sided heart failure.

Heart transplantation in biventricular congenital heart disease: indications, techniques, and outcomes.

Heart transplantation is an accepted therapeutic modality for end-stage congenital heart disease for both biventricular and univentricular anomalies. Many transplant centers have pushed the limits of transplantation to include patients with high pulmonary vascular resistance, high panel reactive antibodies, positive cross-matches, and ABO-incompatibility. Excellent results have been possible, particularly with the development of improved diagnostic and therapeutic algorithms to prevent and treat rejection, infection, and post-transplant lymphoproliferative disease. Late graft failure and chronic rejection remain vexing problems. The vast majority of patients with biventricular congenital heart disease have undergone prior cardiac surgical procedures. Indications for transplantation in this subgroup are primarily progressive refractory heart failure following prior cardiac surgical reconstructive procedures. Contraindications to transplantation mimic those for other forms of end-stage heart disease. A determination of pulmonary vascular resistance is important in listing patients with biventricular congenital heart disease for heart transplantation. Modifications in the implant technique are necessary and vary depending on underlying recipient anatomy. Risk factors for perioperative outcomes in patients with biventricular congenital heart disease include the need for reoperation, the degree of anatomic reconstruction necessary during the implant procedure, and the degree of antibody sensitization, in addition to a number of other recipient and donor factors. Postoperative outcomes and survival are very good but remain inferior to those with cardiomyopathy in most series. In conclusion, patients with end-stage biventricular congenital heart disease represent a complex group of patients for heart transplantation, and require careful evaluation and management to ensure optimal outcomes.

Device closure of muscular ventricular septal defects in infants less than one year of age using the Amplatzer devices: feasibility and outcome.

OBJECTIVES: In this study, we evaluated the feasibility, safety, and outcome of device closure of muscular ventricular septal defects (mVSD) in infants less than 1 year of age using the Amplatzer devices. BACKGROUND: Closure of mVSDs continues to represent a surgical challenge with significant morbidity. Hence, device closure is becoming an attractive and reliable alternative. However, little is known about the feasibility of this procedure in small infants. METHODS: Between July 1999 and September 2006, device closure of mVSD was attempted in 20 infants ranging in age from 3 days to 12 months (median +/- SD; 4.6 +/- 3.8) and in weight from 3.2 to 8.9 kg (4.6 +/- 1.9) under TEE guidance by percutaneous or hybrid (perventricular) techniques. The size of the VSD as assessed by TEE ranged from 3 to 11 mm (6.0 +/- 2.2) and the Qp:Qs ratio ranged from 0.7 to 8.8 (2.8 +/- 2.3). RESULTS: The device was successfully placed in 19/20 infants and it ranged in size from 4 to 14 mm (8.0 +/- 2.6). It was percutaneously deployed in 11/19 and by the hybrid approach in 8/19. There were 30 devices placed in 19 infants with multiple devices placed in 5/19 infants. Fluoroscopy times ranged from 11 to 136 min (41 +/- 28) and procedure times ranged from 57 to 291 min (178 +/- 68). The success rate as defined by complete closure or a trivial shunt was 84% immediately and 100% at 1-year follow-up. Major complications occurred in 4/20 patients: wire perforation and hemopericardium (n = 1), device migration (n = 1), transient electromechanical dissociation (n = 1), and mediastinitis (n = 1). At a median follow-up of 3.8 years, all patients improved and had no hemodynamically significant VSDs. No major complications were encountered in patients with multiple devices. CONCLUSION: In infants less than 1 year of age, percutaneous and perventricular device closure of mVSDs is technically feasible and highly effective with low morbidity. Long term safety and efficacy needs to be assessed.

Modified technique for the surgical treatment of severe tricuspid valve deformity in Ebstein's anomaly.

Distortion of septal and atrial anatomy in Ebstein's anomaly places the atrioventricular node and His bundle at risk for injury at operation. We present a novel technique for creating a robust neo-annulus, remote from conduction tissue, adding to the armamentarium of techniques available for the reconstruction or replacement of the tricuspid valve. Three cases are described. All the patients were in sinus rhythm, with competent native or bioprosthetic valves, at short-term follow-up.

The presentation and management of vascular rings: an otolaryngology perspective.

OBJECTIVE: To review the presentation and natural history of children with vascular rings and present management guidelines. METHODS: Retrospective study of tertiary care pediatric medical center charts from 1991 to 2002. RESULTS: There were 37 males and 27 females with a diagnosis of vascular rings. At presentation, 91% of patients had airway symptoms and 47% had esophageal symptoms. Airway symptoms included stridor (63%), recurrent respiratory infections (47%), respiratory distress (19%), and cough (17%). The most common esophageal symptom was dysphagia (27%). Pre-operative studies included: echocardiography (96%), chest X-ray (93%), barium swallow (75%), magnetic resonance imaging (MRI) (60%), and computerized tomography (CT) scan of the chest (59%). Surgical management included open (n=25) and thoracoscopic (n=39) approach. Complications included recurrent laryngeal nerve injury in five patients (8%). CONCLUSION: Children with vascular rings present with respiratory and/or feeding difficulty. The evaluation should include chest X-ray, echocardiography, and barium swallow. Direct laryngoscopy and bronchoscopy are recommended to assess the degree of compression of the airway and/or esophagus, tracheomalacia, and vocal fold motion prior to intervention. Indication for surgical release is given when the diagnosis is made and can be assisted by advanced radiology studies. Surgical options include minimally invasive techniques involving either thoracoscopic or robotic-assisted repairs, as well as open procedures involving thoracotomy.

Single-ventricle palliation for high-risk neonates: the emergence of an alternative hybrid stage I strategy.

BACKGROUND: Survival after stage I palliation for hypoplastic left heart syndrome or related anomalies remains poor in high-risk neonates. We hypothesized that a less invasive hybrid approach would be beneficial in this patient population. METHODS: The hybrid stage I procedure was performed in the catheterization laboratory. Via a median sternotomy, both branch pulmonary arteries were banded, and a ductal stent was delivered via a main pulmonary artery puncture and positioned under fluoroscopic guidance. RESULTS: Between October 2003 and June 2005, 14 high-risk neonates underwent a hybrid stage I procedure. Eleven of 14 had hypoplastic left heart syndrome. Two also underwent peratrial atrial septal stenting, and 5 required percutaneous atrial stenting later. Two neonates with an intact or highly restrictive atrial septum had emergency percutaneous atrial stent placement. Hospital survival was 11 (78.5%) of 14. One patient required extracorporeal membrane oxygenation support for intraoperative cardiac arrest. He underwent cardiac transplantation but died later of sepsis. One patient died of ductal stent embolization, and a third died of progressive cardiac dysfunction. The first 4 patients required pulmonary artery band revisions. There were none after we modified our technique and added branch pulmonary artery angiograms. There were 2 interstage deaths from atrial stent occlusion and from preductal retrograde coarctation. Eight patients underwent stage II procedures, consisting of aortic arch reconstruction, atrial septectomy, and cavopulmonary shunt. Two patients died after stage II. One patient is awaiting stage II. CONCLUSIONS: The hybrid stage I palliation is a valid option in high-risk neonates. As experience is accrued, it may become the preferred alternative. However, in aortic atresia, the development of preductal retrograde coarctation is a significant problem.

Totally endoscopic robotic-assisted repair of patent ductus arteriosus and vascular ring in children.

BACKGROUND: This study reports on our initial experience with robotically assisted patent ductus arteriosus (PDA) closure and vascular ring division in children. METHODS: From April 2002 to May 2004, 15 patients underwent PDA closure (n = 9) and vascular ring repair (n = 6) by a totally endoscopic approach, utilizing the Da Vinci robotic system. The mean age of the patients was 8.3 +/- 4.7 years (range, 3 to 18) and the mean weight, 35.5 +/- 19.0 kg (range, 14.1 to 77.0 kg). Three thoracoscopic trocars were used to accommodate the endoscopic camera and two surgical instruments with an additional small incision for lung retraction. After dissection by the surgeon seated at the master console, PDA ligation with clips or division of the atretic arch and ductal ligament was performed. RESULTS: Total operative times were 170 +/- 46 minutes (PDA) and 167 +/- 48 minutes (vascular ring). One patient with vascular ring was converted to thoracotomy because of dense adhesions due to previous surgery. Precise and easy surgical maneuver was possible with the articulated surgical instruments and three-dimensional visualization in 14 patients. Intraoperative transesophageal echocardiography confirmed no persistent shunt in all PDA patients. No laryngeal nerve injury and hemorrhage were noted. All patients were extubated in the operating room. Median length of postoperative hospital stay was 1.5 days. CONCLUSIONS: Robotically assisted PDA closure and vascular ring division is a feasible and safe procedure. Future technologic improvement, including smaller instrument size and incorporation of tactile feedback, may permit application of this technique to even younger infants and intracardiac repairs.

Three-dimensional echocardiography-guided beating-heart surgery without cardiopulmonary bypass: a feasibility study.

BACKGROUND: There is no current acceptable approach for intracardiac beating-heart interventions. We have adapted real-time 3-dimensional echocardiography with specialized instrumentation to facilitate beating-heart repair of atrial septal defects and mitral valve plasty to investigate the feasibility of real-time 3-dimensional echocardiography-guided cardiac surgery. METHODS: In experiment I a modified real-time 3-dimensional echocardiography system with x4 matrix transducer was compared with 2-dimensional echocardiography in the performance of common surgical tasks. Completion times, deviation from an ideal trajectory, and an echogenic target were measured. In experiment II porcine atrial septal defects were closed with an original semiautomatic suturing device (n = 4) and with a 5-mm endoscopic stapler and a pericardial or polytetrafluoroethylene patch (n = 4). In experiment III a pulsatile porcine mitral valve model was developed, and suture placement through the anterior and posterior mitral leaflets was performed (n = 8). During all experiments, the operator was blinded to the target and operated on only with ultrasonic guidance. RESULTS: In experiment I, compared with 2-dimensional echocardiographic guidance, completion times improved by 21% ( P <.01) with high-trajectory accuracy, and suture deviation was significantly smaller (2-dimensional echocardiography, 5.4 +/- 2.7 mm; 3-dimensional echocardiography, 1.7 +/- 0.7 mm; P <.05) in real-time 3-dimensional echocardiography-guided tasks. In experiments II and III in both atrial septal defect closure and mitral valve plasty, real-time 3-dimensional echocardiography provided satisfactory images and sufficient anatomic detail for suturing and patch deployment. All surgical tasks were successfully performed with accuracy. CONCLUSIONS: Real-time 3-dimensional echocardiography provides adequate imaging and anatomic detail to act as a sole guide for surgical task performance. These initial experiments demonstrate the feasibility of beating-heart direct or patch closure of atrial septal defects and mitral valve plasty without cardiopulmonary bypass.

Three-dimensional echocardiography-guided atrial septectomy: an experimental study.

OBJECTIVES: The surgical feasibility of beating-heart atrial septectomy under the guidance of two different types of real-time 3-dimensional echocardiography systems was examined. METHODS: A modified real-time 3-dimensional echocardiography system with a x4 matrix transducer (Sonos 7500; Philips Medical Systems, Andover, Mass) or a mechanical 1-dimensional array transducer (SSD-5500; Aloka Co, Ltd, Tokyo, Japan) was used. Small porcine atrial septal defects (n = 8) were enlarged with a Kerrison bone punch in the tank model. In the animal studies, small atrial septal defects (n = 8) were enlarged with the same device through a transatrial port. In both experiments, the area of the atrial septal defect measured by real-time 3-dimensional echocardiography was compared with the area measured directly. RESULTS: Real-time 3-dimensional echocardiography provided satisfactory images and sufficient anatomic detail for the atrial septectomy in both experimental settings. All the atrial septal defects were successfully enlarged; an increase of as much as 293% of the preoperative atrial septal defect area was achieved in the tank experiment, and an increase of as great as 449% of the preoperative area was achieved in the animal experiment. The size of the atrial shunt was increased significantly after the atrial septectomy relative to that before the surgery (P <.0001). The percentage enlargement of the atrial septal defect measured by real-time 3-dimensional echocardiography was strongly correlated with that measured directly (both r(2) = 0.997, P <.0001) Bland-Altman analysis showed close agreement between the results obtained by the two measurement methods in both models. CONCLUSIONS: Real-time 3-dimensional echocardiography provides satisfactory images and sufficient anatomic detail for atrial septectomy. This experiment demonstrates the surgical feasibility of a beating-heart intracardiac procedure such as atrial septectomy under real-time 3-dimensional echocardiographic guidance.

Composite aortic root replacement with a bovine pericardial valve conduit.

In order to offer selected patients undergoing composite aortic root replacement the advantages of a tissue valve, we have constructed conduits intraoperatively by suturing a stented bovine pericardial valve (Edwards Life-sciences LLC, Irvine, CA) inside of a Dacron tube graft (Boston Scientific Corp, Natick, MA). The conduit is quickly made from readily available materials, is easily implanted, and can accommodate any of the anatomic situations encountered in repair of aortic root aneurysms. It is particularly suitable for patients 65 years of age and older.