Accuracy of Micro-Computed Tomography in Post-mortem Evaluation of Fetal Congenital Heart Disease. Comparison Between Post-mortem Micro-CT and Conventional Autopsy.

Aims: Early prenatal diagnosis of congenital heart disease is feasible. Conventional autopsy is the current gold standard method for post-mortem confirmation. Radiologic techniques alternative to conventional autopsy, such as post-mortem micro-computed tomography, have been proposed in case of limited diagnostic accuracy (i.e., early termination of pregnancy, samples of small dimension or of low weight). The aim of the present study was to define accuracy of micro-computed tomography for post-mortem diagnosis of congenital heart disease in gross anatomy samples.Methods and Results: Fetal heart underwent in-utero prenatal echocardiography and ex-vivo post-mortem evaluation by 9 μm resolution micro-computed tomography and conventional autopsy. For each case, 25 indices of cardiac anatomy were studied by post-mortem micro-computed tomography and conventional autopsy; these were used to compare the two post mortem techniques. Ten samples were examined (gestational age between 12 + 4 and 21 + 6 weeks of gestation). Considering comparable indices, agreement between post-mortem micro-computed tomography and conventional autopsy was of 100% and sensitivity and specificity were of 100%. In “challenging specimens,” post-mortem micro-computed tomography diagnoses more indices as compared to conventional autopsy and 84% of “not-diagnostic” indices at conventional autopsy would be diagnostic at post-mortem micro-computed tomography.Conclusion: Micro-computed tomography can be a valid diagnostic alternative to conventional autopsy for post-mortem evaluation of human fetal heart. In addition, it may prove superior to conventional autopsy particularly in cases coming from early termination of pregnancy or in samples of small dimension or of low weight

Thirty years of experience with the Ross operation in children with aortic valve disease and complex LVOT obstruction: Results of a multi-center study

Objective: Progressive autograft and homograft dysfunction with need for later reoperation remain major concerns of the Ross procedure. The aim of the study is to document the outcomes into the third decade after the Ross operation in infants and children. For this purpose, a multi-center study was conducted. Methods: 325 children underwent Ross operation in 10 paediatric units between 1990 and December 2020. Median age at surgery was 10 (IQR 6-15) with 32 patients (10%) under 1 year of life. Indication was aortic stenosis in 114 patients, regurgitation in 116 and mixed lesion in 109. 116 (34%) patients had prior procedures. Root replacement was performed in 223 patients, inclusion cylinder in 33, subcoronary grafting in 20 and Ross-Konno in 63. The primary objective is to analyze the survival rate and re-operation rate on the left and right heart. The secondary objective is to identify risk factors for autograft and homograft failure. Results: There were 7 (2.1%) hospital and 14 late deaths with a median follow-up time of 11 years (IQR: 4,2-17). Survival was 91,6±3,3% and 86%±7% and freedom from any reoperation was 56±10% and 44±13% at 20 and 25 years. 39 (12%) patients had autograft reoperations (10 root replacement, 14 root replacement with valve preservation in 8, ascending aortic replacement in 2 and valve repair in 5). Freedom from autograft reoperation was 75±8% and 70%±11% at 20 and 25 years. 58 children had right heart redo procedures: 6 balloon dilation, 25 trans-catheter pulmonary valve implantation and 26 surgical pulmonary homograft valve replacement (20-year freedom from replacement, 75±5,6%). Prior operation (p=0.031), subcoronary implant (p=0.03) was risk factors for left heart reoperation, while infant age (p=0.001), aortic stenosis as indication to Ross procedure (p=0,049) and use of root technique (p=0.045) were for right heart. The majority (70%) of late survivors were in NYHA class I, 76% free from medication. Conclusions: Ross procedure is a low risk operation for isolated aortic valve or complex left heart obstruction in children, with satisfactory early and late survival. When possible, the Ross operation should be avoided in infants and post-pond after 12 months of age due to the high mortality in this sub-group of patients. In these patients an initial strategy of aortic surgical valvulotomy associated with valve repair should be performed if possible

Late Aortic Valve Rupture After Blunt Chest Trauma

Aortic valve injury after blunt chest trauma due to avulsion of the valve from the annulus or cusp tear commonly results in severe acute regurgitation. Here reported is an unusual case of a 56-year-old woman with multiple traumatic injuries to head, chest, abdomen, and pelvis, in whom massive aortic regurgitation resulting in wide pulse pressure and low cardiac output presented 10 days after admission. The left coronary leaflet explosion was eventually identified at the surgery and managed by valve replacement, due to the extremely weak consistency of the remaining valve tissue. Whether leaflet rupture occurred late after original blunt chest trauma remains conjectural

Improved Outcome of Cardiac Extracorporeal Membrane Oxygenation in Infants and Children Using Magnetic Levitation Centrifugal Pumps.

Extracorporeal membrane oxygenation (ECMO) has traditionally been and, for the most part, still is being performed using roller pumps. Use of first-generation centrifugal pumps has yielded controversial outcomes, perhaps due to mechanical properties of the same and the ensuing risk of hemolysis and renal morbidity. Latest-generation centrifugal pumps, using magnetic levitation (ML), exhibit mechanical properties which may have overcome limitations of first-generation devices. This retrospective study aimed to assess the safety and efficacy of veno-arterial (V-A) ECMO for cardiac indications in neonates, infants, and children, using standard (SP) and latest-generation ML centrifugal pumps. Between 2002 and 2014, 33 consecutive neonates, infants, and young children were supported using V-A ECMO for cardiac indications. There were 21 males and 12 females, with median age of 29 days (4 days-5 years) and a median body weight of 3.2\u2009kg (1.9-18\u2009kg). Indication for V-A ECMO were acute circulatory collapse in ICU or ward after cardiac repair in 16 (49%) patients, failure to wean after repair of complex congenital heart disease in 9 (27%), fulminant myocarditis in 4 (12%), preoperative sepsis in 2 (6%), and refractory tachy-arrhythmias in 2 (6%). Central cannulation was used in 27 (81%) patients and peripheral in 6. Seven (21%) patients were supported with SP and 26 (79%) with ML centrifugal pumps. Median duration of support was 82\u2009h (range 24-672\u2009h), with 26 (79%) patients weaned from support. Three patients required a second ECMO run but died on support. Seventeen (51%) patients required peritoneal dialysis for acute renal failure. Overall survival to discharge was 39% (13/33 patients). All patients with fulminant myocarditis and with refractory arrhythmias were weaned, and five (83%) survived, whereas no patient supported for sepsis survived. Risk factors for hospital mortality included lower (<2.5\u2009kg) body weight (P\u2009=\u20090.02) and rescue ECMO after cardiac repair (P\u2009=\u20090.03). During a median follow-up of 34 months (range 4-62 months), there were three (23%) late deaths and two late survivors with neurological sequelae. Weaning rate (5/7 vs. 21/26, P\u2009=\u2009NS) and prevalence of renal failure requiring dialysis (4/7 vs. 13/26, P\u2009=\u2009NS) were comparable between SP and ML ECMO groups. Patients supported with ML had a trend toward higher hospital survival (1/7 vs. 12/26, P\u2009=\u20090.07) and significantly higher late survival (0/7 vs. 10/26, P\u2009=\u20090.05). The present experience shows that V-A ECMO for cardiac indications using centrifugal pumps in infants and children yields outcomes absolutely comparable to international registry (ELSO) data using mostly roller pumps. Although changes in practice may have contributed to these results, use of ML centrifugal pumps appears to further improve end-organ recovery and hospital and late survival

Twenty-Year Outcome After Right Ventricular Outflow Tract Repair Using Heterotopic Pulmonary Conduits in Infants and Children

Durability of pulmonary conduits (PCs) used for reconstruction of the right ventricular outflow tract (RVOT) may be affected by a variety of factors. Among these, the technique used for PC implantation, whether in orthotopic or heterotopic position, strictly dependent upon the underlying anatomy, has been suggested to influence long-term outcome after RVOT repair. To determine the outcome of heterotopic implantation in infants and children treated at our institution, late results of heterotopic PC in non-Ross patients were analyzed and compared with data of orthotopic PC in age-matched pediatric Ross patients operated during the same time period. Between November 1991 and January 2015, 58 infants and children, 32 male and 26 female, with a median age of 9.4 years (range 1 day-18 years) underwent implantation of heterotopic PC (31 homografts [HG] and 27 xenografts [XG]) for reconstruction of RVOT. Median age in the XG group was significantly lower than in the HG group (0.9 vs. 13.4 years, P\u2009=\u20090.01), while male/female ratio was similar. Fifty (86%) patients had undergone one or more prior cardiac operations, while 32 (55%) required associated procedures during PC implantation. Comparison with data in 305 children and with a median age of 9.4 years, receiving orthotopic PC between 1990 and 2012 (Italian Pediatric Ross Registry), was undertaken. Descriptive, univariate, and Kaplan-Meier analysis defined outcome. There were three (5.2%) early and five (9.0%) late deaths, during a median follow-up of 7.6 years (range 2 months-23 years). Patients having XG had trend toward higher hospital mortality (2/27 vs. 1/31, P\u2009=\u20090.2), but similar late mortality (2/24 vs. 3/30, P\u2009=\u20090.3). Overall survival was 88 and 62%, while freedom from PC replacement was 49 and 21%, at 10 and 20 years, respectively. The latter proved significantly worse than freedom from orthotopic PC replacement, which was 94\u2009\ub1\u20092 and 70\u2009\ub1\u20099% at 10 and 20 years (P\u2009=\u20090.02). When stratified for type of heterotopic PC, late survival proved comparable (81 and 81% for XG vs. 92 and 60% for HG, at 10 and 20 years, respectively, P\u2009=\u20090.7). However, freedom from PC replacement was significantly higher in patients with heterotopic HG (21 and 5% for XG vs. 63 and 48% for HG, at 10 and 20 years, respectively, P\u2009=\u20090.001). RVOT repair using either XG or HG in heterotopic position is a safe procedure associated with low hospital mortality and satisfactory late survival. Freedom from reoperation is significantly lower than that observed in age-matched children having orthotopic HG. Freedom from reoperation in heterotopic XG is poorer than in HG, although different baseline demography may have influenced this finding

Preliminary Results of Cryoablation for Surgical Treatment of Arrhythmias in Adults With Congenital Heart Disease

Background: Arrhythmias in adult congenital heart disease (ACHD) are responsible for the majority of hospital admissions and 20-25% of late deaths. Since need for further cardiac operations is frequent in ACHD, concomitant arrhythmia surgery represents a strategic treatment modality.Material and Methods: A two-center retrospective study was undertaken on cryoablation of supraventricular arrhythmias in 25 conescutive ACHD patients (16/9, M/F, median age 38.5 years, IQR 38-60) operated between 01/2017 and 12/2020. Nineteen (76%) had undergone one or more previous cardiac operations and 8 (32%) one or more trans-catheter ablations. Indications included Fontan conversion in seven patients, septal defect repair in 6, pulmonary valve replacement in 10 and tricuspid surgery in 2. Open-heart cryoablation included: 4 cavotricuspid isthmus ablations, 19 right-sided Maze for atrial tachycardia/flutter, and 2 Cox-Maze III for atrial fibrillation.Results: There were 2 (8%) hospital deaths, unrelated to cryoablation, due to low cardiac output syndrome. There were no intraoperative complications related to cryoablation. Seven (28%) patients required pace-maker implantation due to post-operative atrioventricular block. All patients were discharged on oral antiarrhythmic and anticoagulantion for 6 months. After a median follow-up of 14 months (IQR 7-27) no late mortality was observed. At follow-up, 16/23 (69%) patients are in stable sinus rhythm, 12 without anti-arrhythmic therapy. Two (8.6%) patients had relapse of arrhythmia. Freedom from arrhythmia was 90.9% and cumulative risk of recurrence was 9.6%.Conclusions: Intraoperative cryoablation is safe and effective procedure. Surgical treatment of arrhythmias should always be considered in ACHD, whenever further open-heart repair is needed

Selective Cerebro-Myocardial Perfusion in Complex Neonatal Aortic Arch Pathology: Midterm Results

Aortic arch repair in newborns and infants has traditionally been accomplished using a period of deep hypothermic circulatory arrest. To reduce neurologic and cardiac dysfunction related to circulatory arrest and myocardial ischemia during complex aortic arch surgery, an alternative and novel strategy for cerebro-myocardial protection was recently developed, where regional low-flow perfusion is combined with controlled and independent coronary perfusion. The aim of the present retrospective study was to assess short-term and mid-term results of selective and independent cerebro-myocardial perfusion in neonatal aortic arch surgery. From April 2008 to August 2015, 28 consecutive neonates underwent aortic arch surgery under cerebro-myocardial perfusion. There were 17 male and 11 female, with median age of 15 days (3-30 days) and median body weight of 3 kg (1.6-4.2 kg), 9 (32%) of whom with low body weight (<2.5 kg). The spectrum of pathologies treated was heterogeneous and included 13 neonates having single-stage biventricular repair (46%), 7 staged biventricular repair (25%), and 8 single-ventricle repair (29%). All operations were performed under moderate hypothermia and with a "beating heart and brain." Average cardiopulmonary bypass time was 131\u2009\ub1\u200964 min (42-310 min). A period of cardiac arrest to complete intra-cardiac repair was required in nine patients (32%), and circulatory arrest in 1 to repair total anomalous pulmonary venous connection. Average time of splanchnic ischemia during cerebro-myocardial perfusion was 30\u2009\ub1\u200911 min (15-69 min). Renal dysfunction, requiring a period of peritoneal dialysis was observed in 10 (36%) patients, while liver dysfunction was noted only in 3 (11%). There were three (11%) early and two late deaths during a median follow-up of 2.9 years (range 6 months-7.7 years), with an actuarial survival of 82% at 7 years. At latest follow-up, no patient showed signs of cardiac or neurologic dysfunction. The present experience shows that a strategy of selective and independent cerebro-myocardial perfusion is safe, versatile, and feasible in high-risk neonates with complex congenital arch pathology. Encouraging outcomes were noted in terms of cardiac and neurological function, with limited end-organ morbidity