Postoperative Outcomes of Fontan Operation in a Multicenter Italian Study. How Far Have We Gone? Early Outcomes After Fontan Operation.

Despite the clinical results of the Fontan operation have certainly improved, it still presents with an inherent surgical risk of death and early morbidities. This is a retrospective clinical study of children undergoing Fontan operation in 9 congenital cardiac centers in Italy between 1990 and 2023. Clinical and surgical data were collected via a dedicated RedCap database. Primary outcome was cohort's mortality, also considering different decades, while secondary outcomes were postoperative complications and reintervention. In the last 3 decades, there were 897 patients undergoing Fontan operation, M/F 512/384, median age: 4.5 years (IQR 3.3-6.4), median weight 16 kg (IQR 14-22). A first palliation was deemed necessary in 710 patients (80%), and most patients underwent a staged Fontan (93%); an extracardiac conduit was used in 790 patients (88%). Postoperative complications (mild to severe) occurred in 410 patients (46%), and early reinterventions were required in 66 patients (7.5%). Overall operative mortality was 1.7% (15 patients). Age at Fontan greater than 4 years was associated with an early need for transcatheter reintervention (adj p value = 0.037) and a higher incidence of postoperative complications (adj p value = 0.017). The Fontan operation has seen significant improvements in immediate outcomes, notably a remarkable reduction in overall mortality to just 1.35% in the last decade. While minor complications have remained steady, there has been a substantial decrease in major early complications, deaths, and the need for reinterventions. Notably, patients aged over 4 years seem to face a higher risk of postoperative morbidity, underscoring the critical role of age in preoperative assessment and management strategies for Fontan patients.

Global Cardiac Surgery-Accessibility to Cardiac Surgery in Developing Countries: Objectives, Challenges, and Solutions.

Cardiac surgery is a modern science in the history of medicine. The impact of cardiac disease, in terms of treatment and prognosis, has made this discipline indispensable to global health. In recent decades, the greatest investment has been dispensed to technological and material improvements to increase life expectancy. This surgery must address different epidemiological aspects dictated by the geography and economic-social conditions of the global populations. For this reason, it is progressively important to address the cardiac surgery accessibility disparity. Many scientific papers and international meetings have studied how cardiac surgery can be more accessible in various countries around the world. In this review, we analyze all the challenges, solutions, and suggestions that can make this surgery accessible to the entire global population, with the purpose of reducing its disparity across all seven continents. For a long time, high-income countries have invested in technological capabilities and experimental advancements without caring about unequal access in the rest of the world. We believe that it is time to reverse this growth trajectory, placing the accessibility and distribution of surgical science as a priority, which is significant for the right to health of all people worldwide. This is the real new challenge in cardiosurgery.

Hybrid Palliation for Hypoplastic Borderline Left Ventricle: One More Chance to Biventricular Repair.

Treatment options for hypoplastic borderline left ventricle (LV) are critically dependent on the development of the LV itself and include different types of univentricular palliation or biventricular repair performed at birth. Since hybrid palliation allows deferring major surgery to 4-6 months, in borderline cases, the decision can be postponed until the LV has expressed its growth potential. We aimed to evaluate anatomic modifications of borderline LV after hybrid palliation. We retrospectively reviewed data from 45 consecutive patients with hypoplastic LV who underwent hybrid palliation at birth between 2011 and 2015. Sixteen patients (mean weight 3.15 Kg) exhibited borderline LV and were considered for potential LV growth. After 5 months, five patients underwent univentricular palliation (Group 1), eight biventricular repairs (Group 2) and three died before surgery. Echocardiograms of Groups 1 and 2 were reviewed, comparing LV structures at birth and after 5 months. Although, at birth, all LV measurements were far below the normal limits, after 5 months, LV mass in Group 2 was almost normal, while in Group 1, no growth was evident. However, aortic root diameter and long axis ratio were significantly higher in Group 2 already at birth. Hybrid palliation can be positively considered as a "bridge-to-decision" for borderline LV. Echocardiography plays a key role in monitoring the growth of borderline LV.

Prognosticating Mortality of Primary Cardiogenic Shock Requiring Extracorporeal Life Support: The RESCUE Score.

We aimed to identify prognostic laboratory markers during extracorporeal life support (ECLS) in patients with primary refractory cardiogenic shock (RCS) and to create a preliminary specific mortality score. All 208 consecutive subjects admitted for primary RCS and treated with ECLS between January-2009 and December-2018 were retrospectively analyzed. Multivariate regression analysis on laboratory markers during the first nine days of ECLS was used to develop a "Refractory End-stage Shock CUred with Ecls" (RESCUE) score. Serum creatinine (OR = 3.72, 95%CI: 2.01-6.88), direct bilirubin (OR = 1.40, 95%CI: 1.05-1.8), and platelet count (OR = 0.62, 95%CI: 0.42-0.94) were independent predictors of in-hospital mortality and were included in the score. The mean AUC was 0.763 (95%CI: 0.698-0.828) in the development cohort and 0.729 (95%CI: 0.664-0.794) in the bootstrap internal validation cohort. The RESCUE score represents a novel promising instrument to predict early mortality during the first critical days of ECLS and to help in properly guiding the therapeutic decision-making process.

Case Report: "Smart Palliation" and "Clepsydra Shape": A new approach in complex congenital heart disease.

A limiting factor in using vascular conduits in the pediatric/newborn population is their inability to grow. Many complex congenital heart diseases require palliative surgery, but using rigid and nonexpandable conduits does not allow the structures to grow and anticipates the need for redo surgery. In newborns, a way to increase the palliation time according to the patient's growth is desirable. In recent years, expandable shunts (exGraft™ PECA) have been developed. According to recent material studies, a shunt could increase diameter after endovascular balloon dilatation. In this case report, we describe the first case of endovascular Blalock-Thomas-Taussig shunt (mBT) shunt expansion in a Tetralogy of Fallot / atrial-ventricular Septal Defect complete (TOFAVSDc) patient with trisomy 21 who went to palliative treatment for tracheomalacia (noncardiac lesion association), severe pulmonary arteries hypoplasia, and low weight. This case introduces the "Smart Palliation concept" in the clinical scenario of selected growing patients where the lifetime of the Blalock-Thomas-Taussig (BT) shunt, anatomic substrates, and complexity of clinical status may require an additional palliation time. The limitation of endovascular conduit expansion is the fragility of the anastomosis site. The anastomosis site is a lesser strength structure of the conduit, and dilatation could develop procedure complications. For this reason, in this paper, we introduced our project design: a new technique (Clepsydra Shape) that consists, before surgical implantation, of pre-expansion of the proximal and distal anastomotic parts of the shunt to obtain an increase of 30% in size of both anastomotic sides, preventing stress- and stretch-related lesion of future balloon dilatation.

Use of rapid-deployment aortic valve prosthesis and patch reconstruction in complex endocarditis.

We describe the case of a 59-year-old female affected by aortic extensive endocarditis with communication between aortic annulus and right cavities, treated with double pericardial patch reconstruction and rapid-deployment aortic valve prosthesis implantation.

Arterial switch operation for transposition of the great arteries: A single-centre 32-year experience.

OBJECTIVE: Arterial switch operation (ASO) is nowadays the standard of care for neonates with D-transposition of the great arteries (D-TGA). We evaluated our early and late results with ASO for D-TGA. MATERIALS AND METHODS: We collected data on 267 patients with D-TGA, either with intact ventricular septum (D-TGA/IVS; n = 182, 68%) and with other associated congenital heart malformations (complex D-TGA; n = 85, 32%) that underwent ASO between January 1987 and July 2018. RESULTS: Median age at ASO was 8 days (interquartile range [IQR], 6-12 days). Fifteen patients (5.6%) died in hospital (6/182 with D-TGA/IVS, 3.3% and 9/85 with complex D-TGA, 11%; P = 0.02). Median follow-up time was 10.2 years (IQR, 3.7-18 years). There were 2 (0.8%) late sudden deaths. Overall survival at 10 and 20 years was 94% and 93%, respectively. Thirty-five patients (14%) required either reoperations or reinterventions, mainly for right ventricular outflow tract obstruction (n = 28, 11%). Freedom from reoperation/reintervention at 10 and 20 years was 87% and 78%, respectively. All patients were in NYHA I at latest clinical examination. Six over 173 patients (3.4%) who underwent a postoperative evaluation of their coronaries presented acquired anomalies. Forty-four patients (17%) who performed a cardiopulmonary exercise testing (CPET) have a predicted VO 2 comparable to normal peers. CONCLUSIONS: The results of ASO for D-TGA are excellent, with a fairly low mortality and reoperation/reintervention rate. Functional capacity evaluated with CPET is comparable to normal peers. Continuous follow-up for detecting asymptomatic acquired coronary artery disease is mandatory. A reassessment of competitive sport eligibility criteria for specific D-TGA patients should be considered.

Impact of 3D printing on the surgical management of tracheal stenosis associated to pulmonary sling: a case report.

Congenital tracheal stenosis (CTS) is rare lesions frequently associated with pulmonary sling (PS). Despite the recent improvement in the results by the introduction of slide tracheoplasty (ST) and multidisciplinary approach, surgical management remains difficult and several authors, for this reason, debate for a conservative approach in these cases. Preoperative planning, require hence the use of the most innovative technology to gain the best possible result. This report describes a complicated, unsuccessful surgically treated case of CTS associated to PS. Based on this result and in order to improve the outcomes, we have built a 3D model of patient's trachea simulating preoperative surgical planning. We have hypnotized that having the model preoperatively the choice of surgical technique would have been different. Since that case, 3D models are introduced in our practice and built before tracheal or cardiac cases considered to have complex anatomy. Future investigations are required but at the moment this experience confirms the utility of 3D model in the evaluation of children considered for tracheal surgery.