Fabrizio Gandolfo

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.

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.

Plastic Bronchitis After Extracardiac Fontan Operation

BACKGROUND We discuss 4 cases of plastic bronchitis (PB) after Fontan procedure observed at our Institution, with the aim to identify an effective treatment and possible factors favoring the onset of PB. Plastic bronchitis is an uncommon disease characterized by recurrent formation of large pale bronchial casts obstructing the tracheobronchial tree. The treatment includes inhaled or systemic steroids, aerosolized mucolytics, bronchoscopic lavage, direct bronchoscopic extraction and, in few reports, aerosolized fibrinolytic and pulmonary vasodilators. METHODS Four cases of PB after Fontan procedure occurred in our center from January 2008 to January 2012. RESULTS All patients showed preserved ventricle function and underwent embolization of systemic-pulmonary collaterals prior to a Fontan procedure and all had chylothorax in the perioperative period. Two patients died; 1 of massive thrombosis of the superior vena cava and right pulmonary artery and the other of acute asphyxiation and refractory cardiac failure. CONCLUSIONS The exact causes of PB in Fontan patients remain unknown. An early diagnosis and a multiple therapy with steroids, mucolytics, pulmonary vasodilators, and aerosolized tissue plasminogen activator may be effective.

One-stage repair of aberrant left brachiocephalic artery and coarctation of the aorta in right aortic arch

The combination of right aortic arch with aberrant left brachiocephalic artery and aortic coarctation is very rare. Here, we report the case of a 3.1-kg neonate with multiple malformations who received detailed preoperative anatomical definition by chest computed tomography (CT) scan and eventually underwent one-stage repair at the age of 17 days. The surgical technique included left brachiocephalic artery detachment and its end-to-end anastomosis to the branch of a monofurcated pulmonary homograft used to treat the aortic arch coarctation simultaneously. Postoperative control chest CT scan performed 3 months postoperatively revealed optimal repair. Considerations on imaging and surgical technique are reported.

Managing major vessel injuries with a Fogarty catheter during chest re-opening in children

Injury of structures, leading to a major bleeding during chest opening, is a severe and potentially life-threatening complication, especially in redo cardiac surgery, both in adults and children. In three paediatric redo operations performed via midline sternotomy, we managed this complication successfully and uneventfully by using an inflated Fogarty catheter to plug the blood leak from the injured vessel before repairing the lesion under direct vision in a bloodless surgical field. Herein we report in detail the technique used and a comment on our experience.

The Use of Berlin Heart EXCOR VAD in Children Less than 10 kg: A Single Center Experience

Objective: Despite the improvement in ventricular assist device (VAD) therapy in adults and in adolescents, in infant population only Berlin Heart EXCOR (BHE) is licensed as long term VAD to bridge children to Heart Transplantation (HTx). Particularly demanding in terms of morbidity and mortality are smallest patients namely the ones implanted in the first year of life or with a lower body surface area. This work aims at retrospective reviewing a single center experience in using BHE in children with a body weight under 10 kg. Methods: Data of all pediatric patients under 10 kg undergoing BHE implantation in our institution from March 2002 to March 2016 were retrospectively reviewed. Results: Of the 30 patients enrolled in the study, 53% were male, 87% were affected by a dilated cardiomyopathy with an average weight and age at the implantation of 6.75 ± 2.16 Kg and 11.57 ± 10.12 months, respectively. Three patients (10%) required a BIVAD implantation. After the implantation, 7 patients (23%) required re-intervention for bleeding and 9 patients (30%) experienced BHE cannulas infection. A total of 56 BHE pump were changed for thrombus formation (1.86 BHE pump for patient). The average duration of VAD support was 132.8 ± 94.4 days. Twenty patients (67%) were successfully transplanted and 10 patients (33%) died: 7 for major neurological complication and 3 for sepsis. Conclusion: Mechanical support in smaller children with end stage heart failure is an effective strategy for bridging patients to HTx. The need for BIVAD was relegated, in the last years, only to restrictive cardiomiopathy. Further efforts are required in small infants to improve anticoagulation strategy to reduce neurological events and BHE pump changes.

Perigraft seroma mimicking cardiac tamponade after implantation of Berlin Heart EXCOR device in an infant

Development of serous fluid leakage around polytetrafluoroethylene (PTFE) grafts used to perform systemicto-pulmonary arterial shunt (modified Blalock-Taussig shunt) has been reported previously. Occasionally, an excessive amount of serous fluid will persistently drain through the interstices of the fabric. We report an unusual case of pericardial seroma in an infant developing around the outflow cannula of a new model of Berlin Heart left ventricular assistance device (EXCOR; Berlin Heart GmbH, Berlin, Germany), which includes a PTFE component. This seroma led to a initial diagnosis of cardiac tamponade and necessitated surgical revision.

Cardiovascular Magnetic Resonance in patients with repaired Tetralogy of Fallot: the goal standard in preoperative assessment and follow up of injectable pulmonary valve implantation

Background Severe pulmonary regurgitation, progressive dilatation and dysfunction of the right ventricle are the most frequent reason of late morbidity post Fallot repair. Pulmonary valve replacement is often indicated in these patients. BioIntegral Injectable pulmonary valve (IPV) is an innovative less invasive technique, often done off cardiopulmonary bypass (CPB). Cardiovascular Magnetic Resonance (CMR) is fundamental tool to assess patient suitability for IPV insertion and in the clinical follow up.