Heart Transplantation Following Fontan Failure: Long-Term Survival Analysis.

Objectives: Fontan circulation presents significant challenges for patients with congenital heart disease, often necessitating heart transplantation (HTX) due to deteriorating functionality across multiple organ systems. However, the impact of prior Fontan palliation on HTX outcomes remains poorly understood, with early mortality rates suggesting a heightened risk. The aim of our study is to evaluate the long-term results after heart transplantation in patients with univentricular congenital heart disease previously palliated with Fontan circulation. Methods: A retrospective analysis was conducted on patients who underwent HTX for congenital heart disease. Patients were categorized into two groups based on the pre-HTX circulation pathway: the Failing Fontan Group (FFG) and the Biventricular Congenital Group (BCG). Data were collected from patients between 1987 and 2018. Early and late outcomes, including survival rates, were assessed and critically analyzed. Results: Of the 66 patients, 29 (43%) had a failing Fontan palliation (FFG), and 37 had biventricular congenital diseases (BCG) before heart transplantation. Early mortality (30-day) was not statistically different between the two group. The overall survival rate was 82.6 ± 13.9% at 1 year, 79.0 ± 14.9% at 5 years, 67.2 ± 17.6% at 10 years and 63.2 ± 18.2 ± at 15 years for the FFG, and 86.1 ±11.4% at 1 year, 79.5 ± 13.7% at 5 years, 75.7 ± 14.9% at 10 years, 75.7 ± 14.9% at 15 years for the BCG, with no statistically significant difference (Mantel Cox p value: 0.69, 0.89, 0.52 and 0.39, respectively). Regarding Cox-regression analysis, the long-term survival rate was not affected either by previous Fontan surgery or by the era of heart transplantation (before vs. after the year 2000). Conclusions: Although heart transplantation after Fontan palliation showed a higher risk in the early post-operative period, the medium- and long-term survival rates are comparable with biventricular circulation patients. Despite the failing Fontan patients being a challenging set of candidates for transplantation, it is a reasonable option in their treatment.

An alternative valve for mitral valve replacement in young children: using an NO-REACT® INJECTABLE BIOPULMONIC™ prosthesis as a mitral valve replacement in a 14-month-old child.

Mitral valve replacement in infants is challenging and there are limited alternative valves available. Since the Boston group published their first report on alternative valves for mitral valve replacement in infants, there has been a growth in the literature on the topic, mostly based on the use of a stented bovine jugular vein graft (Melody® valve). The challenges of the Melody valve are firstly in its length of 28 mm unexpanded, which has the potential to cause left ventricular outflow tract obstruction, and secondly, the valve needs mechanical dilatation, which is laborious. A modified No-React® Injectable Biopulmonic™ Prosthesis (Bio Integral Surgical, Inc., Mississauga, ON, Canada) which is shorter (19 mm) and simpler in that it is self-expanding was implanted in a 14-month-old child to replace her mitral valve. The operation was successful and the short-term function of the prosthesis is good.

Analysis of a Cooperation and Interventional Model in Humanitarian Medicine.

Background: Every year, around 15 million children, in developing countries, die or develop life-long disabilities because of congenital cardiac diseases. In this report we measure the effect of a pediatric cardiac surgery humanitarian project on the health of the individual and on the potential influence this has on the countries economy and its growing health services. Methods: We collected and analyzed data from the Italian NGO, Mission Bambini's database, including all congenital cardiac missions undertaken in Cambodia between 2012 and 2019. DALY's (Disability Adjusted Life Years) saved by the humanitarian mission were estimated and used to reflect on the impact this had on the populations economy. Progression in the local medical teams skills emulated the advancements made in the health sector of the region. Results: Between 2012 and 2019, 128 patients underwent a congenital cardiac operation at Angkor Hospital for Children at Siem Reap, Cambodia. The median age was 6 years. The majority of the pathologies included VSD, TOF, ASD. The mean Aristotle's Complexity Score was 6. Post-operative mortality was 0.8% (1/128). The cost-effectiveness analysis identified 5.360 DALY's saved by surgery. The competency of the local team was progressive with them being able to handle more complex cases on subsequent missions. Conclusion: In developing Countries, performing congenital cardiac surgery cases can be carried out successfully with improvement in both the economy and the health system of the country by increasing the years and the quality of life of the working population and developing the expertise of the regional team.

Impact of COVID-19 Pandemic on the Italian Humanitarian Congenital Cardiac Surgery Activity: What No One Tells You.

More than 4 millions of children with congenital heart disease (CHD) are waiting for cardiac surgery around the world. Few of these patients are treated only thanks to the support of many non-governmental organizations (NGOs). Starting in December 2019, the so-called coronavirus disease 2019 (COVID-19) has rapidly become a worldwide pandemic and has dramatically impacted on all the international humanitarian activities for congenital heart disease. We analyzed data from all the Italian congenital cardiac surgery centers with the aim to quantify the impact of the pandemic on their charities. Fifteen Italian centers participated in the study and contributed to data collection. We analyzed and compared data regarding humanitarian activities carried out abroad and on site from two periods: year 2019 (pre-COVID-19) and year 2020 (COVID-19 pandemic). In 2019, 53 international missions were carried out by Italian congenital cardiac surgeons, resulting in the treatment of 471 CHD patients. In the same period 11 Italian cardiac centers operated on 251 foreign patients in Italy. In 2020, the pandemic led to a reduction of this activity by 96% for the surgery performed overseas and 86% for the interventions carried out in Italy. In conclusion our study shows the important quantitative impact of the pandemic on the Italian humanitarian cardiac surgical activity overseas and in Italy. This shocking result highlights the failure of the systems adopted so far to solve the problem of CHD in developing countries.

Mid- to long-term follow-up of pulmonary valve replacement with BioIntegral injectable valve.

OBJECTIVES: Chronic pulmonary valve (PV) regurgitation is a common late sequela after repair of congenital heart diseases like tetralogy of Fallot or pulmonary stenosis, leading to right ventricular dilatation and failure and increased late morbidity and mortality. Timely reoperation may lead to a complete right ventricular recovery. An injectable PV allows pulmonary valve replacement, with or without cardiopulmonary bypass, under direct observation, thereby minimizing the impact of surgery on cardiac function. The aim of this study was to evaluate the feasibility and mid- to long-term clinical outcomes with this device. METHODS: From April 2007 to October 2019, a total of 85 symptomatic patients with severe pulmonary regurgitation or pulmonary stenosis underwent pulmonary valve replacement with an injectable stented pulmonary prosthesis. Data were collected from the international proctoring registry. Mean patient age was 26.7 years. The underlying diagnosis was repaired tetralogy of Fallot in 69.4% patients; moderate or severe pulmonary regurgitation was present in 72.9%. All patients had echocardiographic scans before the operation and during the follow-up period. A total of 54.1% patients also had preoperative/postoperative cardiac magnetic resonance imaging (MRI) or catheterization; 25.9% had off-pump implants. In 53% patients, pulmonary valve replacement was associated with the repair of other cardiac defects. RESULTS: Minor postoperative complications were observed in 10.8% patients. The overall mortality rate was 2.3%; mortality after valve replacement was linked to a severe cardiac insufficiency and it was not related to a prosthesis failure; 1 prosthesis was explanted from 1 patient because of endocarditis, and 6% of patients developed PV stenosis; minor complications occurred in 4.8%. The mean follow-up period was 4.8 years (2 months-12.7 years); 42% of the patients were followed for more than 5 years. Follow-up echocardiography and cardiac MRI showed a significant reduction in RV size and low gradients across the PV. CONCLUSIONS: An injectable PV may be implanted without cardiopulmonary bypass and in a hybrid operating theatre with minimal surgical impact. The bioprosthesis, available up to large sizes, has a low profile, laminar flow and no risk of coronary artery compression. Incidence of endocarditis is rare. The lack of a suture ring permits the implant of a relatively larger prosthesis, thereby avoiding a right ventricular outflow tract obstruction. This device permits future percutaneous valve-in-valve procedures, if needed. Results concerning durability are encouraging, and mid- to long-term haemodynamic performance is excellent.

Lombardy regional urgent reorganization for congenital cardiac patients following the Covid-19 pandemic.

OBJECTIVE: By the end of February 2020, the COVID-19 pandemic infection had spread in Northern Italy, with thousands of patients infected. In Lombardy, the most affected area, the majority of public and private hospitals were dedicated to caring for COVID-19 patients and were organized following the 'Hub-and-Spoke' model for other medical specialties, like cardiac surgery and interventional procedures for congenital cardiac disease (CHD). Here, we report how the congenital cardiac care system was modified in Lombardy and the first results of this organization. METHODS: We describe a modified 'Hub-and-Spoke' model - that involves 59 birthplaces and three specialized Congenital Cardiac Centers -- and how the hub center organized his activity. We also reported the data of the consecutive cases hospitalized during this period. RESULTS: From 9 March to 15 April, we performed: a total of 21 cardiac surgeries, 4 diagnostic catheterizations, 3 CT scans, and 2 CMR. In three cases with prenatal diagnosis, the birth was scheduled. The spoke centers referred to our center six congenital cardiac cases. The postop ExtraCorporeal Membrane Oxygenation support was required in two cases; one case died. None of these patients nor their parents or accompanying person was found to be COVID-19-positive; 2 pediatric intensivists were found to be COVID-19-positive, and needed hospitalization without mechanical ventilation; 13 nurses had positive COVID swabs (4 with symptoms), and were managed and isolated at home. CONCLUSION: Our preliminary data suggest that the model adopted met the immediate needs with a good outcome without increased mortality, nor COVID-19 exposure for the patients who underwent procedures.

The unnatural history of failing univentricular hearts: outcomes up to 25 years after heart transplantation.

OBJECTIVES: Heart transplantation (HTx) in children with a univentricular physiology is a challenge. In this study, we aimed to investigate the early and late survival as well as the causes of death of HTx recipients at different stages of univentricular palliation. METHODS: Between January 1987 and September 2015, 40 orthotropic cardiac transplants were performed in 38 children with univentricular hearts at our institution. Outcomes were reviewed using medical records and transplant databases. RESULTS: For the purposes of this analysis, patients were divided into 3 subgroups according to their stage of palliation: Stage 1 (n = 10, 26%), Stage 2 (n = 5, 13%) and Fontan (n = 23, 61%). The median age at HTx was 15.2 years (range 0-38). The median follow-up time after transplantation was 8.7 years (range 0-25.4). Indications for transplant were ventricular dysfunction in 25 (66%) patients, protein losing enteropathy in 10 patients transplanted in Fontan (26%) and refractory arrhythmias in 3 (8%) patients with an atriopulmonary connection. Total mortality was 42% (4.84/100 patient-years), and total early mortality was 21%. Overall survival at 1, 10 and 20 years was 73% (95% confidence interval 56-84%), 58% (95% confidence interval 40-72%) and 49% (95% confidence interval 30-65%), respectively. CONCLUSIONS: HTx is a feasible option for patients with failing univentricular circulation, and although the mortality rate is high, this rate is still comparable to that in patients undergoing HTx for other congenital and non-congenital heart diseases.

[Transposition of the great arteries]. / La trasposizione delle grandi arterie.

Dextro-transposition of the great arteries with intact ventricular septum (d-TGA) is the most frequent cyanotic congenital heart disease in neonates. In newborns affected by d-TGA, the pulmonary and systemic circulations are in parallel instead of being in series. The survival of babies affected by d-TGA is related to the level of mixing at the patent foramen ovale. The diagnosis of d-TGA is an indication for treatment due to scarce survival beyond the neonatal period if not corrected. The current surgical approach is the anatomical correction with the arterial switch operation, which has excellent early and medium-term results. In this review, we discuss treatment of d-TGA and the arterial switch operation, the medium- and long-term results following surgery, and the reasons that have led the arterial switch operation to its popularity compared to the former physiological correction of d-TGA, i.e. the Senning and Mustard procedures.

Partial anomalous connection of both superior pulmonary veins.

Several patterns of anomalous pulmonary venous drainage have been described in the literature, and bilateral partial pulmonary anomalous vein connection (PAPVC) has been described as a rare congenital cardiac anomaly. We report an unusual type of bilateral PAPVC, involving both the superior right and left pulmonary veins draining into the left brachiocephalic vein in a young adult who was symptomatic with dyspnea and a dry cough.

Large left atrial myxoma with severe mitral regurgitation: the inverted T-shaped biatrial incision revisited.

Cardiac myxomas are primary cardiac tumors. In some cases of atrial myxoma, the standard left atriotomy alone does not enable safe tumor resection and easy access to the mitral valve. We report the cases of 2 patients with huge left atrial myxomas associated with severe mitral valve regurgitation who underwent an inverted T-shaped biatrial incision for tumor excision and mitral repair. This approach reduces tumor fragmentation and permits good mitral valve exposure.