Antonio Amodeo

Extracardiac Fontan operation for complex cardiac anomalies: Seven years' experience

METHODS Between 1988 and 1995, 60 patients with complex cardiac anomalies underwent a total extracardiac cavopulmonary connection, a combination of a bidirectional cavopulmonary anastomosis with an extracardiac conduit interposition between the inferior vena cava and pulmonary arteries, except in one patient in whom direct anastomosis was possible. In 40 patients the total extracardiac cavopulmonary connection followed preliminary bidirectional cavopulmonary anastomosis, associated with a modified Damus-Kaye-Stansel anastomosis in 16. The conduits were constructed of Dacron fabric (n = 34), homografts (n = 3), and polytetrafluoroethylene (n = 22). RESULTS Total early failure rate was 15% (n = 9). Six patients died, and three more had conduit takedown owing to pulmonary artery stenosis and hypoplasia (n = 2) and severe atrioventricular valve regurgitation (n = 1). Two other patients required anastomosis revision owing to stricture. In a mean follow-up of 48 months (6 to 86 months) there were no late deaths (actuarial 5-year survival 88% +/- 4%); 52 of 54 patients are in New York Heart Association class I or II. Two patients required pulmonary artery balloon dilation or stent implantation, or both, after total extracardiac cavopulmonary connection. Late tachyarrhythmias were detected in four of 54 patients: two had sick sinus syndrome with flutter necessitating a pacemaker implantation and two had recurrent flutter (actuarial 5-year arrhythmia-free rate 92% +/- 4%). Conduit patency was evaluated by serial magnetic resonance imaging studies. Preliminary data showed a 17.8% +/- 7.6% mean reduction in conduit internal diameter during the first 6 months after total extracardiac cavopulmonary connection, with no progression over the next 5 years. CONCLUSION These results demonstrate that the total extracardiac cavopulmonary connection provides good early and midterm results and may reduce the prevalence of late arrhythmias in patients undergoing the Fontan operation.

Mechanical Assist Device as a Bridge to Heart Transplantation in Children Less Than 10 Kilograms

BACKGROUND Despite the remarkable advances with the use of ventricular assist devices (VAD) in adults, pneumatic pulsatile support in children is still limited. We report a retrospective review of our experience in very small children (<10 kg of body weight). METHODS Ten consecutive children weighing less than 10 kg were offered mechanical support with Berlin Heart (Berlin Heart AG, Berlin, Germany) as a bridge to heart transplant from March 2002 to March 2010. RESULTS The median patient age was 10.4 months (38 days to 2.2 years). The median patient weight was 6.4 kg (2.9 to 10 kg). Prior to VAD implantation, all children were managed by multiple intravenous inotropes and mechanical ventilation (8) or extracorporeal membrane oxygenation (2). The median pre-VAD pulmonary vascular resistance index was 5.7 Woods units/m(2). Three patients required biventricular mechanical support, but in all other cases a single left VAD proved sufficient. The median duration of VAD support was 61 days (2 to 168 days). Four deaths occurred; from stroke in three and sepsis in one. Five patients were successfully bridged to heart transplantation after a median duration of mechanical support of 89 days (37 to 168 days) and another is still waiting a suitable organ after 77 days of VAD support. There were no complications related to postoperative bleeding. Five patients required at least one pump change. Of 5 patients undergoing heart transplant, 3 developed an extremely elevated (>60%) panel reactive antibody by enzyme-linked immunosorbent assay, confirmed by Luminex (Luminex Corp, Austin, TX). All 3 experienced at least one acute episode of rejection in the first month after heart transplant, needing plasmapheresis. The survival rate after heart transplantation was 100% with a median follow-up of 7.5 months. CONCLUSIONS Mechanical support in very small children with end-stage heart failure is an effective strategy of bridge to heart transplantation with a reasonable mortality rate. The high rate of complications suggests to optimize indications and timing of VAD implantation.

Left ventricular assist device in Duchenne Cardiomyopathy: Can we change the natural history of cardiac disease?

End stage dilated cardiomyopathy (DCM) is currently one of the most challenging elements in the management of patients affected by Duchenne muscular dystrophy [1]. DCM is a complication of Duchenne muscular dystrophy, and leads to advanced heart failure and premature death [2,3]. Until the last decade, cardiomyopathy inDuchennemuscular dystrophy accounted for only 20% of deaths because respiratory failure occurred earlier than cardiac failure. Due to recent technological advances, respiratory care has greatly improved and life expectancy has increased to 30–40 years. The efficacy of standard heart failure treatment for improving the clinical outcome of these patients has been proven, but neverthelessmore than40%will die of heart failure [2]. Duchenne syndrome has generally been considered a contraindication for cardiac transplantation due to the associated progressive skeletal myopathy leading to limited functional capacity [4]. This concern has resulted in a reluctance to offer cardiac transplantation to these patients in an era of donor shortage. The recent advances in left ventricular assist devices, used as destination therapy, have made feasible the use of such devices for the treatment of DCM in Duchenne patients. We present two adolescents with Duchenne muscular dystrophy admitted to our department because of acute heart failure, for which a ventricular assistant device (VAD) was decided as destination therapy. Case 1. A 15 year-old boy who, during the last year, experienced three episodes of acute cardiac failure despite maximal medical treatment, was admitted to our ICU with intractable cardiac failure unresponsive to high dosage inotropes. We elected to implant, as destination therapy, a left VAD (Jarvik 2000) with the pedestal inserted into the skull. The patient needed daily treatmentwith a coughmachine and

Implantation of a left ventricular assist device as a destination therapy in Duchenne muscular dystrophy patients with end stage cardiac failure: management and lessons learned.

Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder, characterized by progressive skeletal muscle weakness, loss of ambulation, and death secondary to cardiac or respiratory failure. End-stage dilated cardiomyopathy (DCM) is a frequent finding in DMD patients, they are rarely candidates for cardiac transplantation. Recently, the use of ventricular assist devices as a destination therapy (DT) as an alternative to cardiac transplantation in DMD patients has been described. Preoperative planning and patient selection play a significant role in the successful postoperative course of these patients. We describe the preoperative, intraoperative and postoperative management of Jarvik 2000 implantation in 4 DMD pediatric (age range 12-17 years) patients. We also describe the complications that may occur. The most frequent were bleeding and difficulty in weaning from mechanical ventilation. Our standard protocol includes: 1) preoperative multidisciplinary evaluation and selection, 2) preoperative and postoperative non-invasive ventilation and cough machine cycles, 3) intraoperative use of near infrared spectroscopy (NIRS) and transesophageal echocardiography, 4) attention on surgical blood loss, use of tranexamic acid and prothrombin complexes, 5) early extubation and 6) avoiding the use of nasogastric feeding tubes and nasal temperature probes. Our case reports describe the use of Jarvik 2000 as a destination therapy in young patients emphasizing the use of ventricular assist devices as a new therapeutic option in DMD.

Ventricular assist device in univentricular heart physiology

The use of mechanical cardiac assistance is well established as a bridge to orthotopic heart transplantation (OHT) or to recovery for patients with congestive heart failure, however, the experience in single ventricle (SV) physiology is still limited. We report two cases of mechanical assistance in patients with SV physiology: a 2-year old male with hypoplastic left heart syndrome who underwent Norwood Stage I and II followed by HF and a 4-year old female with a univentricular heart who developed a severe right ventricular dysfunction 2 years after a cavopulmonary shunt. Mechanical support utilizing ventricular assist devices (VADs) is considered a valid tool to bridge patients with congestive heart failure to either OHT or to recovery. Increasing experience and improved outcomes utilizing this technology in children with biventricular hearts have led to considering employing these devices in failing SV treatment. We present 2 cases of terminally ill children with SV who were assisted with a VAD.

Inflammatory cytokines in pediatric cardiac surgery and variable effect of the hemofiltration process.

Cardiac surgery with cardiopulmonary bypass (CPB) elicits an inflammatory response and has a multitude of biological consequences, ranging from subclinical organ dysfunction to severe multiorgan failure. Pediatric patients are more prone to have a reaction that can jeopardize their outcome. Cytokines are supposed to be important mediators in this response: limiting their circulating levels is, therefore, appealing. We investigated the pattern of cytokine release during pediatric operation for congenital heart anomalies in 20 patients, and the effect of hemofiltration. Tumor necrosis factor a (TNF-α) was elevated after anesthesia induction and showed significant decrease during CPB. Hemofiltration reduced its concentration, but the effect disappeared on the following day. Interleukin-1 (IL-1) increased slowly at the end of CPB and hemofiltration had no effect. Interleukin-6 (IL-6) showed a tendency toward augmentation during rewarming and hemofiltration did not significantly affect the course. Soluble interleukin-6 receptor (sIL-6r) had a pattern similar to TNF-α, but hemofiltration had no effect. On the other hand, interleukin-8 (IL-8) behaved like IL-6. Our findings suggest that baseline clinical status, anesthetic drugs, and maneuvers before incision may elicit a cytokine response, whereas rewarming is a critical phase of CPB. Hemofiltration is effective in removal of TNF-α, but its role is debatable for the control of IL-1, IL-6, sIL-6r and IL-8 levels.

The Ross procedure in patients aged less than 18 years: The midterm results

OBJECTIVE This study reviews a single-center experience with the Ross procedure in infants and young children. METHODS From November 1993 to March 2012, 55 children aged less than 17 years underwent a Ross procedure. The patients ranged in age from 2 days to 17 years (median, 5.9 years). Thirteen patients were infants, and 18 patients were preschool children. The predominant indication for the Ross procedure was aortic stenosis. Twenty-seven patients (49%) with left ventricular outflow tract obstruction underwent a modified Ross-Konno procedure. Twenty-five patients (45%) had undergone 40 previous cardiac procedures. Preoperatively, 3 patients showed severe left ventricular dysfunction, with 2 of the patients requiring intubation and inotropic support. Concomitant procedures were performed in 11 patients (20%). Nine patients underwent mitral valve surgery, and 2 patients underwent subaortic membrane resection. RESULTS Patients were followed up for a median of 66 months (range, 3 months to 17 years). Overall survival at 1, 2, 5, and 10 years was 84.9%. Hospital mortality rate was 13% (7/55 patients). All deaths occurred in neonates or infants, except 1 who was aged less than 4 years. Freedom from reoperation for autograft failure was 100% at 1 year, 96.7% at 5 years, and 73.7% at 10 years. During follow-up, 7 patients required a reoperation on the autograft for dilatation and severe aortic insufficiency. Freedom from reoperation for the right ventricular outflow tract replacement was 56.1% at 10 years. CONCLUSIONS The low rate of autograft failure demonstrates that the Ross procedure is an attractive option for the management of aortic valve disease and complex left ventricular outflow tract obstruction in the pediatric population. However, alternative options must be considered in adolescents and young adults.

Neurological complications during pulsatile ventricular assistance with the Berlin Heart EXCOR in children: incidence and risk factors.

The aim of this study is to describe the incidence of brain injury (BI) in children with end-stage cardiac failure who were supported with the Berlin Heart EXCOR ventricular assist device (VAD) as a bridge to heart transplantation. Between January 2002 and January 2012, all patients <18 years of age who underwent the implantation of the Berlin Heart EXCOR at Bambino Gesú Childrens Hospital were included. A total of 25 patients were included in this study. Median age and weight at implantation were 22.4 months (range 3.6-154.2) and 10 kg (range 4.5-36), respectively. Diagnosis included cardiomyopathy (n = 20) and congenital heart disease (n = 5). Eleven patients received atrial cannulation. Nine patients underwent biventricular assist device support. Seven patients underwent extracorporeal membrane oxygenation before the implantation of the EXCOR VAD. Median duration of VAD support was 51 days (range 2-167). Nine patients had evidence of acute BI including intracranial hemorrhage (n = 5) and cerebral ischemia (n = 4). Freedom from BI at 30, 60, and 90 days from VAD implantation was 80.7, 69.9, and 43.3%, respectively. Weight <10 kg at implantation was significantly associated with BI. BI is a frequent complication among children supported with EXCOR VAD and is associated with lower weight at implantation. However, our data do not support the association between size and BI. Future prospective multicenter studies are warranted to further help understand the etiology and the impact of BI and to improve functional outcomes for children undergoing EXCOR VAD mechanical support.

Total cavopulmonary direct anastomosis: a logical approach in selected patients.

A 2.5-year-old boy with a diagnosis of situs solitus, tricuspid atresia, anatomically corrected malposition of the great arteries (S,D,L), left juxtaposition of atrial appendages, and pulmonary stenosis underwent successful total cava-to-pulmonary connection by means of a superior vena cava-to-pulmonary artery end-to-side anastomosis associated with an inferior vena cava-to-pulmonary artery direct anastomosis. Anatomic features and surgical technique are described.

Left ventricular assist device as destination therapy in cardiac end-stage dystrophinopathies: Midterm results

Objective: We report our experience with the use of a left ventricular assist device (LVAD) as destination therapy (DT) for the management of patients with cardiac end‐stage dystrophinopathies. Methods: From February 2011 to February 2016, 7 patients with dystrophinopathies and dilated cardiomyopathy (DCM) were treated with LVADs at our institution. Median age at surgery was 16.5 years (range, 14.2‐23.4 years). All patients were preoperatively evaluated by a multidisciplinary team approach. Results: All patients survived to hospital discharge. The early postoperative course was characterized by abdominal bleeding (1 patient) and retropharyngeal bleeding (1 patient). Because of abdominal or retropharyngeal bleeding, both required postoperative heparin infusion discontinuation for 35 and 33 days, respectively. Among the late complications, 1 child developed osteolysis and infection at the pedestal site of the device, which required surgical displacement; 1 patient required gastrostomy as a result of poor feeding, and another had a cerebral stroke, which was treated with percutaneous thrombus aspiration. The other 2 patients did not show early or late complications. At a median follow‐up time of 21.7 months (range, 3‐45 months) there have been 3 deaths: 1 patient died of a lung infection after 45 months, 1 died of tracheal bleeding after 29 months, and 1 died of cerebral hemorrhage after 14 months. Conclusions: Our experience indicates that the use of an LVAD as DT in patients with dystrophinopathies with end‐stage DCM is feasible, suggesting that it may be suitable as a palliative therapy for the treatment of these patients with no other therapeutic options.