[Show abstract][Hide abstract] ABSTRACT: Primary repair of pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collaterals based on
single-stage unifocalization was first reported in 1995. From a midline approach, all collaterals are extensively dissected,
translocated in front of the oesophagus and/or the trachea, when required, and directly anastomosed to each other or to the
native pulmonary arteries, whenever present, without interposition of prosthetic material. The need for concomitant VSD closure
is assessed intraoperatively with a pulmonary flow study according to a standardized protocol. Pulmonary blood supply is established
by valved conduit interposition in all patients, regardless of the suitability for VSD closure. Palliation with systemic-pulmonary
shunt is reserved for selected cases. Between 1994 and 2015, 94 patients with a median age of 1.09 years (range 0.03–19) underwent
single-stage unifocalization at our institution. In 78 (82.1%) of them, an intraoperative pulmonary flow study was utilized
to assess acceptability for concomitant VSD closure, which was accomplished in 69 cases (73%). Intraoperatively, following
VSD closure, the mean right ventricle-to-aortic pressure ratio was 0.49 ± 0.14. The overall mortality rate was 11.2% (n = 10), with an 82% survival at 12.5 years. At a median follow-up interval of 5.8 years, the right ventricle-to-aortic pressure
ratio did not differ significantly from the early postoperative phase. The surgical results of primary repair of PA with VSD
and major aortopulmonary collaterals based on single-stage unifocalization and an intraoperative pulmonary flow study are
satisfactory and durable, despite the need for repeated percutaneous or surgical reinterventions.
Preview · Article · Jan 2016 · Multimedia Manual of Cardiothoracic Surgery
[Show abstract][Hide abstract] ABSTRACT: The study aim was to analyze the safety and longevity of cryopreserved homografts used for primary right ventricular outflow tract reconstruction (RVOTR), and to compare the outcome using either standard or bicuspidalized allografts.
Between February 2000 and September 2014, a total of 53 patients underwent primary RVOTR using either a standard (n = 40) or a bicuspidalized (n = 13) cryopreserved homograft. The median age at surgery was 15.5 months (range: 1-419.06 months), and bodyweight was 8.5 kg (range: 4.1-71 kg). The median standard homograft size was 17.5 mm (range: 10-25 mm), while the median bicuspidalized homograft size was 16 mm (range: 14-22 mm). Follow up was complete in 91.4% of patients, with a median duration of 30.11 months (range: 0.26-161.26 months). Data analysis included primary diagnosis, type of surgery, age at surgery, size of conduit, need for reintervention, and survival. Predetermined primary outcomes were represented by survival and freedom from conduit reintervention.
Two patients with standard cryopreserved homograft died. during the early postoperative period (3.7%). No deaths were conduit-related. The five-year survival rate and ten-year freedom from reintervention were 91% (range: 74.7-97.2%) and 53.6% (range: 97-33.2%), respectively. RV-PA conduit replacement was performed in 14 patients (26.4%) at a median interval of 44.5 months (range: 14.93-162.46 months). Among these patients, four children (30.7%) received bicuspidalized homografts, and 10 (25%) received standard homografts. Causes of reintervention were conduit stenosis in six cases (43%), severe homograft valve regurgitation in two (14.2%), conduit stenosis and homograft valve regurgitation in two (14.2%), and stenosis of distal anastomosis involving pulmonary bifurcation in four (28.6%). Univariate analyses showed a longer freedom from reintervention for bicuspidalized compared to standard homograft (p = 0.03).
The results obtained suggested that bicuspidalized homograft performance compares well with that of standard allografts in terms of freedom from reintervention. Bicuspidalized homograft use is strongly indicated for primary RVOTR in small children, when a standard homograft of appropriate size is not available.
No preview · Article · Jul 2015 · The Journal of heart valve disease
[Show abstract][Hide abstract] ABSTRACT: Objective
To analyze the effectiveness and the results of the use of a vacuum-assisted closure (VAC) system for the treatment of complex sternal wounds in newborns after cardiac surgery.Methods
From May 2008 until December 2012, six patients developed post-sternotomy wound problems (large defects of epithelialization or mediastinitis), which were treated with a VAC system. Median age at the time of institution of VAC was 24.5 days (range 16 to 65 days). Median time of treatment was 14 days (range 3 to 42 days).ResultsAll patients were newborns and all underwent delayed sternal closure after cardiac surgery. The indications for using the VAC system were: mediastinitis in two patients (33.3%) and impairment of healing without signs of infection in four (66.7%). All children after VAC therapy achieved healing of the sternal wound. VAC therapy was started with high negative pressures (−125 mmHg) continuously then switched to an intermittent modality in all patients.ConclusionVAC system with high negative pressure is safe, effective, and is a well-tolerated therapy in newborns with complex sternal wounds.
No preview · Article · Nov 2014 · Journal of Cardiac Surgery
[Show abstract][Hide abstract] ABSTRACT: Objective Children undergoing Ross operation were expected to have longer autograft, but shorter homograft durability compared with adults. In order to define the outcome in the second decade after Ross operation in children, a nationwide review of 23 years of experience was undertaken.
Methods 305 children underwent Ross operation in 11 paediatric units between 1990 and 2012. Age at surgery was 9.4±5.7 years, indication aortic stenosis in 103 patients, regurgitation in 109 and mixed lesion in 93. 116 (38%) patients had prior procedures. Root replacement was performed in 201 patients, inclusion cylinder in 14, subcoronary grafting in 17 and Ross–Konno in 73.
Results There were 10 (3.3%) hospital and 12 late deaths (median follow-up 8.7 years). Survival was 93±2% and 89±3% and freedom from any reoperation was 76±3% and 67±6% at 10 and 15 years. 34 children had autograft 37 reoperations (25 replacement, 12 repair): three required transplantation after reoperation. Freedom from autograft reoperation was 86±3% and 75±6% at 10 and 15 years. 32 children had right heart redo procedures, and only 25 (78%) conduit replacements (15-year freedom from replacement, 89±4%). Prior operation (p=0.031), subcoronary implant (p=0.025) and concomitant surgical procedure (p=0.004) were risk factors for left heart reoperation, while infant age (p=0.015) was for right heart. The majority (87%) of late survivors were in NYHA class I, 68% free from medication and six women had pregnancies.
Conclusions Despite low hospital risk and satisfactory late survival, paediatric Ross operation bears substantial valve-related morbidity in the first two decades. Contrary to expectation, autograft reoperation is more common than homograft.
[Show abstract][Hide abstract] ABSTRACT: Haemolysis is known to occur during surgery on cardiopulmonary bypass (CPB) and to be responsible for kidney injury. The aim of this study was to assess, in a cohort of infants, the reference levels of free haemoglobin (fHb) and their change over time postoperatively; the predicting variables of haemolysis in the intraoperative phase; and the association between fHb and renal function.
A retrospective analysis in infants undergoing surgery on CPB was conducted. Children with preoperative renal dysfunction and need for extracorporeal membrane oxygenation support were excluded. fHb was sampled before and after CPB and on the first 2 postoperative days (POD).
Twenty-two patients with a median (interquartile) age of 111 (63-184) days and Aristotle score of 8 (6.4-9) were enrolled. fHb had a baseline value of 29 (24-41) mg/dl, peaked to 75 (65-109) mg/dl at CPB weaning and returned to 35 (30-55) mg/dl on POD 2 (P <0.0001). The median normalized index of haemolysis was 0.15 (0.09-0.19) g of fHb per 100 l of pumped blood. A multivariable regression model showed that, at CPB weaning, fHb levels were independently associated with left atrial venting flow (P = 0.02), and that CPB time remained the only independent variable (P = 0.034), when left atrial venting was excluded from the analysis. Acute kidney injury (AKI) occurred in 10 patients (45%). fHb levels in the 48 post-CPB hours were not significantly different between AKI and non-AKI patients: However, a significant correlation was present between creatinine on POD1 and CPBw-fHb (r = 0.48; P = 0.045); and between cystatin C on POD1 and CPBw-fHb (r = 0.58; P = 0.02).
A high rate of fHb is released during paediatric surgery with CPB in infants. fHb mainly depends on the left atrial venting flow rate and CPB duration. However, such peaks of fHb levels were not associated with renal dysfunction.
No preview · Article · May 2014 · Interactive Cardiovascular and Thoracic Surgery
[Show abstract][Hide abstract] ABSTRACT: To investigate the clinical manifestations at diagnosis and during follow-up in patients with 22q11.2 deletion syndrome to better define the natural history of the disease.
A retrospective and prospective multicenter study was conducted with 228 patients in the context of the Italian Network for Primary Immunodeficiencies. Clinical diagnosis was confirmed by cytogenetic or molecular analysis.
The cohort consisted of 112 males and 116 females; median age at diagnosis was 4 months (range 0 to 36 years 10 months). The diagnosis was made before 2 years of age in 71% of patients, predominantly related to the presence of heart anomalies and neonatal hypocalcemia. In patients diagnosed after 2 years of age, clinical features such as speech and language impairment, developmental delay, minor cardiac defects, recurrent infections, and facial features were the main elements leading to diagnosis. During follow-up (available for 172 patients), the frequency of autoimmune manifestations (P = .015) and speech disorders (P = .002) increased. After a median follow-up of 43 months, the survival probability was 0.92 at 15 years from diagnosis.
Our data show a delay in the diagnosis of 22q11.2 deletion syndrome with noncardiac symptoms. This study provides guidelines for pediatricians and specialists for early identification of cases that can be confirmed by genetic testing, which would permit the provision of appropriate clinical management.
Full-text · Article · Mar 2014 · The Journal of pediatrics
[Show abstract][Hide abstract] ABSTRACT: An intrapericardial vacuolated mass compressing and displacing the heart was diagnosed by echocardiography in a foetus of 22 weeks gestation. The birth was induced for early signs of foetal distress at 29 weeks and, after two initial pericardial evacuation procedures, the tumour was resected radically 7 days after birth at a weight of 1.55 kg. Mass histology showed teratoma associated with yolk sac tumour. We comment on the overall approach adopted after foetal diagnosis and the histopathological features of the tumour, and try to draw conclusions on patient outcome data.
No preview · Article · Jan 2014 · Cardiology in the Young
[Show abstract][Hide abstract] ABSTRACT: Bleeding during and after cardiac surgery is a major issue in pediatric patients. A prospective cohort study was conducted to evaluate the effect of a commercially available prothrombin complex (Confidex) administered in cardiac surgery after weaning from cardiopulmonary bypass of infants with nonsurgical bleeding. In this study, 14 patients younger than 1 year received a Confidex bolus and were matched with 11 patients of a similar age who did not receive the drug. The preoperative coagulation profile was similar in the two groups. No side effects, including anaphylaxis or thrombotic events, were observed. The numbers of units of packed red blood cells and fresh frozen plasma administered both intra- and postoperatively were similar. The postoperative coagulation examination results and thromboelastographic parameters did not differ significantly between the two groups. However, the Confidex patients bled significantly less than the control subjects during the first 24 postoperative hours. The median volume of drained blood was 0.0 ml/kg h (range 0-1.9 ml/kg h) compared with 1.9 ml/kg h (range 1-3 ml/kg h) (p = 0.009). At least one unit of packed red blood cells in the postoperative phase was required by 2 patients (14 %) in the Confidex group and six patients (54 %) in the control group (odds ratio [OR], 0.13; 95 % confidence interval [CI], 0.02-0.9; p = 0.03). The median duration of mechanical ventilation was 3 days (range 2-4 days) in the Confidex group and 4 days (range 0-8 days) in the control group (p = 0.66). The median stay in the intensive care unit was 6 days (range 5-9 days) in the Confidex group and 7 days (range 4-12 days) in the control group (p = 0.88). The use of Confidex for infants undergoing cardiac surgery was safe and effective. It reduced postoperative bleeding and allowed fewer units of packed red blood cells to be infused in the postoperative phase without major side effects.
No preview · Article · Jul 2013 · Pediatric Cardiology
[Show abstract][Hide abstract] ABSTRACT: 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.
No preview · Article · May 2013 · Interactive Cardiovascular and Thoracic Surgery
[Show abstract][Hide abstract] ABSTRACT: OBJECTIVE:: Modified ultrafiltration is commonly used in pediatric cardiac surgery. Although its clinical benefits are currently debated, modified ultrafiltration has proved to improve mean arterial pressure in the first postoperative hours. Aim of our study was to measure cardiac index, stroke volume index, and mean arterial pressure modification before and after modified ultrafiltration by means of Pressure Recording Analytical Method. DESIGN:: Single-center prospective observational cohort study. SETTING:: Pediatric cardiac surgery operating room. PATIENTS:: Children below 20 kg that are included in the "pediatric" mode of Pressure Recording Analytical Method. MEASUREMENTS AND MAIN RESULTS:: Forty patients were enrolled in this study. Median age, weight, and body surface area at surgery were 3 months (interquartile range, 10 days to 3.5 years), 5.6 (3.1-15) kg, and 0.31 (0.21-0.56), respectively. During the modified ultrafiltration procedure, a median volume of 17 mL/kg (11-25) was ultrafiltered and a median volume of 11 mL/kg (6-17) was reinfused with a median final modified ultrafiltration balance of -0.15 mL/kg (-4.0 to 0.1). By univariate analyses, there was a 10% increase in postmodified ultrafiltration mean, systolic and diastolic pressures (p = 0.01), stroke volume index (p = 0.02), and cardiac index (p = 0.001) without significant changes in heart rate, central (left and right) venous pressures, stroke volume variation, and inotropic score. By multivariate analysis, when controlling for cardiopulmonary bypass time and age at surgery, cardiac index variation was independently associated with lower preoperative body surface area (beta coefficient -5.5, p = 0.04). CONCLUSIONS:: According to Pressure Recording Analytical Method assessment, modified ultrafiltration acutely improves myocardial function, as shown by a 10% increase of systemic arterial pressure, stroke volume index, and cardiac index. This effect is more pronounced in smaller sized patients.
No preview · Article · Apr 2013 · Pediatric Critical Care Medicine
[Show abstract][Hide abstract] ABSTRACT: 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.
Preview · Article · Mar 2013 · Interactive Cardiovascular and Thoracic Surgery