Sergio Filippelli

Determinants of outcome after surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

OBJECTIVESnIdentification of variables influencing surgical outcome in patients treated for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.nnnMETHODSnA total of 90 consecutive patients (median age, 12 months; range, 20 days to 35 years), who had primarily undergone either 1-stage unifocalization (nxa0=xa069) or palliation to promote native pulmonary arterial development (nxa0=xa021), were studied. Chromosome 22q11 deletion had occurred in 37% of the cases. Ventricular septal defect closure was accomplished in 70 patients (78%), with a mean postoperative right/left ventricular pressure ratio of 0.48xa0± 0.14.nnnRESULTSnThe rate of 14-year survival, freedom from conduit reintervention, and freedom from percutaneous intervention on the pulmonary arteries was 75%, 46%, and 52%, respectively. At a median interval of 95 months (range, 1.5-164 months), the right/left ventricular pressure ratio did not differ significantly from early postoperatively. Univariate analysis showed that an absence of confluent intrapericardial pulmonary arteries favorably affected the postoperative right/left ventricular pressure ratio after ventricular septal defect closure (Pxa0=xa0.04). Kaplan-Meier estimates showed age of 30 days or younger (Pxa0=xa0.0004) and weight of 3 kg or less (Pxa0=xa0.0004) at unifocalization and chromosome 22q11 deletion (Pxa0=xa0.001) significantly affected survival. Chromosome 22q11 deletion was significantly associated with mortality, even in the Cox regression model (hazard ratio, 8.26; Pxa0=xa0.003). Finally, ventricular septal defect closure during single-stage and single/multiple-stage procedures significantly correlated with both early (Pxa0=xa0.0013 and Pxa0<xa0.00001, respectively) and overall (Pxa0=xa0.013 and Pxa0=xa0.0007, respectively) survival.nnnCONCLUSIONSnThe results of surgery were satisfactory and durable, despite the need for repeated percutaneous or surgical reinterventions. The outcomes were negatively affected by neonatal age and low body weight and positively affected by simultaneous or staged ventricular septal defect closure. Finally, chromosome 22q11 deletion remained an independent variable affecting survival.

Mechanically Assisted Total Cavopulmonary Connection With an Axial Flow Pump: Computational and In Vivo Study

A relevant number of patients undergoing total cavopulmonary connection (TCPC) experience heart failure (HF). Heart transplant is then the final option when all other treatments fail. The axial flow blood pumps are now the state of the art; however, there is little experience in low-pressure circuits, such as support of the right ventricle or even a TCPC circulation. A new T-shaped model of mechanically assisted TCPC using the Jarvik Child 2000 axial pump, (flow rates between 1 and 3u2009L/m in a range of 5000-9000u2009rpm) was designed, simulated numerically, and then tested in animals. Eight sheep (42-45u2009kg) were studied: two pilot studies, four pump-supported (PS) TCPC for 3u2009h, and two not pump-supported (NPS) TCPC. In the PS, the axial pump was set to maintain the baseline cardiac output (CO). Pressures, CO, systemic and pulmonary vascular resistance, lactate levels, and blood gases were recorded for 3u2009h. Computational fluid dynamics (CFD) study allows us to set the feasible operating condition and the safety margins to minimize the venous collapse risk. In the NPS animals, a circulatory deterioration, with increasing lactate level, occurred rapidly. In the PS animals, there was a stable cardiac index of 2.7u2009±u20091.4u2009L/min/m(2), central venous pressure of 12.3u2009±u20091u2009mmu2009Hg, and a mean pulmonary artery pressure (PAP) of 18.1u2009±u20096 after 3u2009h of support up to 9000u2009rpm. systemic vascular resistance (SVR), pulmonary vascular resistance (PVR), blood gasses, and arterial lactate levels remained stable to baseline values. No caval collapse occurred. A new pediatric axial flow pump provides normal CO and physiologic stability in a new T-shaped model of TCPC in sheep, in vivo. CFD and in vivo data showed that this experimental arrangement will allow us to evaluate the potential for mechanical support in patients with Fontan failure avoiding major adverse events.

Repair of incompetent truncal valves: early and mid-term results.

OBJECTIVESnTo analyse the factors associated with in-hospital mortality and mid-term significant neoaortic valve regurgitation (AR) after truncal valve (TV) repair.nnnMETHODSnEleven children underwent TV repair at our institution from July 1999 to March 2012. All children presented significant preoperative TV regurgitation. Valve anatomy was quadricuspid in 7 (64%) patients and tricuspid in 4 (36%). The median age and weight at surgery were 29.6 (range 0.3-173.2) months and 12 (range 2.2-49) kg, respectively. Repair included bicuspidalization through the approximation of two leaflets associated with triangular resection of the opposite one (n = 2, 18%), or either bicuspidalization or tricuspidalization of the TV through excision of one leaflet and related sinus of Valsalva (n = 9, 82%). In 3 patients, repair was associated with coronary detachment before cusp removal, followed by coronary reimplantation.nnnRESULTSnIn-hospital death occurred in 2 (18%) patients. Factors associated with hospital mortality were age <1 year (P = 0.05), weight <3 kg (P = 0.02) and longer cross-clamping time (P = 0.008). Follow-up was complete for all patients [median follow-up time: 52.2 (range 132.2-2.5) months]. Mid-term significant AR occurred in 4 patients (45%, moderate in 2 and severe in 2). One with severe AR underwent successful valve replacement 4 months postoperatively, leading to freedom from reintervention of 91%. Freedom from significant AR was 76.2 (33.2-93.5) and 60.9 (20.2-85.6) at 1 and 2 years, respectively. There was a trend towards longer freedom from mid-term significant AR for patients who underwent cusp removal compared with those who did not (P = 0.07).nnnCONCLUSIONSnTV repair in children can be performed safely with fairly good and durable results. Cusp removal might decrease the rate of severe AR on mid-term follow-up.

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.

Evolution of Biventricular Loading Condition in Pediatric LVAD Patient: A Prospective and Observational Study

The aim of this study was to describe the echocardiographic trend of left ventricular (LV) and right ventricular (RV) function after implantation of a pulsatile flow left ventricular assist device (LVAD) in children. From 2013 to 2016, we prospectively evaluated 13 consecutive pediatric Berlin Heart EXCOR LVAD patients. Clinical and echocardiographic data were collected at baseline, within 24 h after implantation and monthly until LVAD explant. Median age and weight at the implantation was 8 (4-23) months and 5 (4.6-8.3) kg at the time of implantation, respectively. All were affected by dilated cardiomyopathy. Average LVAD support time was 226.2u2009±u2009121.2 days. Nine (70%) were transplanted, 4 (30%) died. LV end-systolic and end-diastolic volumes were reduced until the follow up of two months (Pu2009=u20090.019 and Pu2009=u20090.001). A progressive increase in RV dimensions was observed. After 4 months of follow up, RV fractional area change worsening was statistically related with the deterioration of LV unloading (Pu2009=u20090.0036). Four patients needed prolonged inotropic support for RV failure. Pulsatile LVAD in pediatrics is followed by an early and mid-term LV unloading, as expressed by a decrease in LV volumes and diameters at echocardiogram. The effects of unloading do not remain stable at long term follow up. RV function improved in the acute phase, but a progressive dilatation of RV was noted over time. In some patients, RV failure might lead to the need of an increase of inotropic support at long term follow up.

Evolution of Ventricular Energetics in the Different Stages of Palliation of Hypoplastic Left Heart Syndrome: A Retrospective Clinical Study

Hyperplastic left heart syndrome (HLHS) patients are palliated by creating a Fontan-type circulation passing from different surgical stages. The aim of this work is to describe the evolution of ventricular energetics parameters in HLHS patients during the different stages of palliation including the hybrid, the Norwood, the bidirectional Glenn (BDG), and the Fontan procedures. We conducted a retrospective clinical study enrolling all HLHS patients surgically treated with hybrid procedure and/or Norwood and/or BDG and/or Fontan operation from 2011 to 2016 collecting echocardiographic and hemodynamic data. Measured data were used to calculate energetic variables such as ventricular elastances, external and internal work, ventriculo-arterial coupling and cardiac mechanical efficiency. From 2010 to 2016, a total of 29 HLHS patients undergoing cardiac catheterization after hybrid (nxa0=xa07) or Norwood (nxa0=xa06) or Glenn (nxa0=xa08) or Fontan (nxa0=xa08) procedure were retrospectively enrolled. Ventricular volumes were significantly higher in the Norwood circulation than in the hybrid circulation (pxa0=xa00.03) with a progressive decrement from the first stage to the Fontan completion. Ventricular elastances were lower in the Norwood circulation than in the hybrid circulation and progressively increased passing from the first stage to the Fontan completion. The arterial elastance and Rtot increased in the Fontan circulation. The ventricular work progressively increased. Finally, the ventricular efficiency improves passing from the first to the last stage of palliation. The use of ventricular energetic parameters could lead to a more complete evaluation of such complex patients to better understand their adaptation to different pathophysiological conditions.