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BACKGROUND: Right mini-thoracotomy cardiac surgery has been recognized as a safe and effective procedure, with remarkable early and long-terms outcomes. However, most of the literature is focused on mitral valve surgery and few studies report on the minimally invasive approach applied to congenital disease. Aim of this study was to review our experience on patients with grown-up congenital heart (GUCH) undergoing right mini-thoracotomy cardiac surgery. METHODS: Data of patients with GUCH undergoing right mini-thoracotomy cardiac surgery from 2006 to 2019 were retrospectively analyzed. Inclusion criteria were atrial septal defect, partial anomalous pulmonary venous return, partial atrioventricular septal defect, and mitral or tricuspid valve dysfunction in congenital heart diseases. RESULTS: During the study period 127 patients with GUCH underwent right mini-thoracotomy cardiac surgery. Mean age was 43.6 years and more than 60% were females; diagnosis was atrial septal defect in 57 cases (44.9%); 24 patients were redo (18.9%). No cases of stroke and major vascular complications were reported. Conversion to sternotomy was required in one case (0.8%). No residual shunts or valves dysfunction were recorded at the postoperative echocardiographic evaluation. Perioperative mortality was 1.6%. CONCLUSIONS: Right mini-thoracotomy cardiac surgery in selected patients with GUCH allows to avoid the big scar of the sternotomy approach and to accelerate the recovery in a young population. Moreover, in redo cases, it allows the surgeon to reach the heart and the aorta avoiding the well-known risks of a re-sternotomy procedure.
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Cardiopatias Congênitas , Toracotomia , Adulto , Feminino , Cardiopatias Congênitas/cirurgia , Humanos , Masculino , Procedimentos Cirúrgicos Minimamente Invasivos , Valva Mitral/diagnóstico por imagem , Valva Mitral/cirurgia , Estudos Retrospectivos , Esternotomia , Resultado do TratamentoRESUMO
BACKGROUND AND AIM OF THE STUDY: Outcomes after repair of tetralogy of Fallot (TOF) are good with either a transventricular (TV) or transatrial (TA) approach. We sought to determine if there is a relationship between the TV or TA approach and right ventricular (RV) function, and the role of residual pulmonary regurgitation (PR) on the long-term outcomes. METHODS: This was a retrospective cohort multicentric study on survivors after surgical repair of TOF (TA versus TV approach, ±transannular patch) between 1990 and 2004. All patients underwent magnetic resonance imaging to assess RV volume, function, and PR. Patients were matched for length of follow-up and age. Clinical adverse events were retrieved from institutional databases. RESULTS: Seventy-nine patients (TA/TV = 37/42, median age 0.3 and 1.0 yrs, respectively) were included. At a median follow-up of 16.6 years (12.5-20.3), there were no differences in freedom from reintervention (either catheter or surgical), RV volumes, function, and PR between the TA and TV groups. Pulmonary valve (PV) replacement was significantly less frequent in the TA subgroup (P = 0.033) and patients with a preserved PV showed significantly lower RV volumes and less adverse events at follow-up. CONCLUSIONS: There is no significant difference in RV volumes and function between the TA and TV. However, the TA approach seems to be protective against PV replacement in the long-term. When PV is not preserved at repair, residual pulmonary regurgitation is a significant cause of late RV dysfunction and dilation, and is associated with a higher rate of late adverse events.
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Procedimentos Cirúrgicos Cardíacos/métodos , Átrios do Coração/cirurgia , Ventrículos do Coração/fisiopatologia , Ventrículos do Coração/cirurgia , Tetralogia de Fallot/fisiopatologia , Tetralogia de Fallot/cirurgia , Feminino , Humanos , Lactente , Masculino , Valva Pulmonar , Fatores de Tempo , Resultado do TratamentoRESUMO
BACKGROUND: Data regarding long-term outcome after percutaneous closure of left superior caval vein draining into the left atrium are lacking. The aim of the present study was to report the long-term follow-up by using contrast-enhanced CT. METHODS: In all, three patients underwent percutaneous closure of left superior caval vein draining into the left atrium between 2005 and 2015. All of them were evaluated clinically and underwent contrast-enhanced CT. RESULTS: In one patient, the Amplatzer® Septal Occluder was used. In two patients, the Amplatzer® Vascular Plug type-1 was preferred: the device size/LSVC diameter ratio was 1.7 in the child and 1.2 in the adult. There were no early-onset or long-term onset complications. CT was performed 1, 2, and 10 years after the procedure, respectively. Complete occlusion of the vessel was documented in all. After 10 years since the procedure, CT revealed a persistent trivial residual shunt through the accessory hemiazygos vein in one patient, in whom the device was implanted above its drainage into the left superior caval vein. When an Amplatzer® Vascular Plug type-1 is oversized compared with the venous vessel diameter, it immediately assumes a dog-bone shape that disappears early to regain its shape memory and nominal size. CONCLUSIONS: Percutaneous occlusion of left superior caval vein draining into the left atrium has excellent early and long-term outcomes. The optimal implantation of the device is below the drainage of the accessory hemiazygos vein, when present. The device might be oversized compared with the left superior caval vein diameter according to the age of the patient.
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Procedimentos Cirúrgicos Cardiovasculares/métodos , Átrios do Coração/anormalidades , Dispositivo para Oclusão Septal , Cirurgia Assistida por Computador/métodos , Malformações Vasculares/cirurgia , Veia Cava Superior/anormalidades , Adulto , Criança , Pré-Escolar , Ecocardiografia , Feminino , Fluoroscopia , Seguimentos , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/cirurgia , Humanos , Masculino , Fatores de Tempo , Tomografia Computadorizada por Raios X , Malformações Vasculares/diagnóstico , Veia Cava Superior/diagnóstico por imagem , Veia Cava Superior/cirurgiaRESUMO
We report two cases with levoatriocardinal vein and partial anomalous pulmonary venous drainage in left-sided obstructive lesions. This association may be difficult to recognise by echocardiography. Cardiac CT and MRI were crucial to define the diagnosis and to tailor the best therapeutic option.
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Anormalidades Múltiplas/cirurgia , Cardiopatias Congênitas/cirurgia , Veias Pulmonares/anormalidades , Veias Pulmonares/cirurgia , Criança , Humanos , Recém-Nascido , MasculinoRESUMO
INTRODUCTION: A systemic inflammatory response is triggered in patients undergoing cardiothoracic surgery with cardiopulmonary bypass (CPB). This response is particularly evident in pediatric patients, especially those of low weight and after undergoing long CPB, and can severely impair the surgical result. Adsorptive blood purification techniques have been proposed to limit this systemic inflammatory response. To test its efficacy, we added the hemoadsorption filter Jafron HA 380 to CPB in a much compromised pediatric patient who underwent heart transplantation. METHODS: A 10-year-old single ventricle patient previously treated with Fontan operation was listed for heart transplantation due to the evidence of failing Fontan condition. He experienced many episodes of cardiac arrest and underwent heart transplantation in much compromised general and hemodynamic conditions. The hemoadsorption filter Jafron HA 380 was used for all the duration of CPB, and the inflammatory biomarker interleukin 6 (IL-6) was assayed. RESULTS: Postoperative outcome was uneventful and comparable to that of elective pediatric heart transplantation. IL-6 levels showed an impressive postoperative reduction, and after 2 days, the IL-6 level was comparable with a typical uneventful post-transplant course. CONCLUSIONS: The use of hemoadsorption filter can contribute to improve the pediatric transplant results, especially in very high-risk patients.
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Técnica de Fontan , Transplante de Coração , Criança , Humanos , Masculino , Ponte Cardiopulmonar/métodos , Técnica de Fontan/métodos , Cardiopatias Congênitas/cirurgia , Interleucina-6/sangueRESUMO
OBJECTIVES: The use of ventricular assist devices (VADs) in children is increasing. However, absolute numbers in individual centres and countries remain small. Collaborative efforts such as the Paedi-European Registry for Patients with Mechanical Circulatory Support (EUROMACS) are therefore essential for combining international experience with paediatric VADs. Our goal was to present the results from the fourth Paedi-EUROMACS report. METHODS: All paediatric (<19 years) patients from the EUROMACS database supported by a VAD were included. Patients were stratified into a congenital heart disease (CHD) group and a group with a non-congenital aetiology. End points included mortality, a transplant and recovery. Cox proportional hazard models were used to explore associated factors for mortality, cerebrovascular accident and pump thrombosis. RESULTS: A total of 590 primary implants were included. The congenital group was significantly younger (2.5 vs 8.0 years, respectively, P < 0.001) and was more commonly supported by a pulsatile flow device (73.5% vs 59.9%, P < 0.001). Mortality was significantly higher in the congenital group (30.8% vs 20.4%, P = 0.009) than in the non-congenital group. However, in multivariable analyses, CHD was not significantly associated with mortality [hazard ratio (HR) 1.285; confidence interval (CI) 0.8111-2.036, P = 0.740]. Pump thrombosis was the most frequently reported adverse event (377 events in 132 patients; 0.925 events per patient-year) and was significantly associated with body surface area (HR 0.524, CI 0.333-0.823, P = 0.005), CHD (HR 1.641, CI 1.054-2.555, P = 0.028) and pulsatile flow support (HR 2.345, CI 1.406-3.910, P = 0.001) in multivariable analyses. CONCLUSIONS: This fourth Paedi-EUROMACS report highlights the increasing use of paediatric VADs. The patient populations with congenital and non-congenital aetiologies exhibit distinct characteristics and clinical outcomes.
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Coração Auxiliar , Sistema de Registros , Humanos , Sistema de Registros/estatística & dados numéricos , Coração Auxiliar/estatística & dados numéricos , Coração Auxiliar/efeitos adversos , Criança , Pré-Escolar , Masculino , Feminino , Lactente , Europa (Continente)/epidemiologia , Adolescente , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/mortalidade , Insuficiência Cardíaca/mortalidade , Insuficiência Cardíaca/cirurgia , Recém-NascidoRESUMO
BACKGROUND AND AIM OF THE STUDY: Decellularized xenogeneic pulmonary valves have been introduced for right ventricular outflow tract (RVOT) reconstruction in congenital heart disease. In the present study, the intermediate-term results from three institutions were analyzed. METHODS: Between January 2006 and September 2008, a total of 61 patients (median age 7 years; range: 9 days to 50 years; median body weight 21 kg; range: 1.9-140 kg) underwent RVOT reconstruction with either the Matrix P (n = 9) or Matrix P Plus (n = 52) tissue-engineered conduit. Eighteen patients underwent surgery in infancy, and 31 patients had previously undergone one or more RVOT interventions or operations. RESULTS: The valve sizes ranged from 11 to 27mm. Five patients died during the hospital stay or within three months, from non-valve-related causes; hence, the early mortality was 8.2%. No deaths occurred during the follow up period. Reoperation due to valve failure became necessary in four patients; three patients underwent RVOT interventions due to distal anastomotic stenosis, and six reinterventions were performed distal to the valve due to hypoplastic branch pulmonary arteries. Patients with valve implantation during infancy showed a composite freedom from valve-related reoperation, catheter intervention or valve dysfunction (defined as dP(max) > 40 mmHg) of 87% at one and three years postoperatively. Both, computed tomography and magnetic resonance imaging studies demonstrated normal structural features, with no evidence of calcification. CONCLUSION: The Matrix P/Matrix Plus conduit represents a viable alternative for RVOT reconstruction in patients with congenital heart disease. The intermediate-term performance of the conduits was favorable compared to that of other currently available implants.
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Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas/cirurgia , Valva Pulmonar/transplante , Adolescente , Adulto , Animais , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Criança , Pré-Escolar , Feminino , Alemanha , Cardiopatias Congênitas/mortalidade , Mortalidade Hospitalar , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/patologia , Reoperação , Suínos , Fatores de Tempo , Tomografia Computadorizada por Raios X , Transplante Heterólogo , Resultado do TratamentoRESUMO
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.
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BACKGROUND: Rare diseases are chronic and life-threatening disorders affecting < 1 person every 2,000. For most of them, clinical symptoms and signs can be observed at birth or childhood. Approximately 80% of all rare diseases have a genetic background and most of them are monogenic conditions. In addition, while the majority of these diseases is still incurable, early diagnosis and specific treatment can improve patients' quality of life. Transplantation is among the therapeutic options and represents the definitive treatment for end-stage organ failure, both in children and adults. The aim of this paper was to analyze, in a large cohort of Italian patients, the main rare genetic diseases that led to organ transplantation, specifically pointing the attention on the pediatric cohort. RESULTS: To the purpose of our analysis, we considered heart, lung, liver and kidney transplants included in the Transplant Registry (TR) of the Italian National Transplantation Center in the 2002-2019 timeframe. Overall, 49,404 recipients were enrolled in the cohort, 5.1% of whom in the pediatric age. For 40,909 (82.8%) transplant recipients, a disease diagnosis was available, of which 38,615 in the adult cohort, while 8,495 patients (17.2%) were undiagnosed. There were 128 disease categories, and of these, 117 were listed in the main rare disease databases. In the pediatric cohort, 2,294 (5.6%) patients had a disease diagnosis: of the 2,126 (92.7%) patients affected by a rare disease, 1,402 (61.1%) presented with a monogenic condition. As expected, the frequencies of pathologies leading to organ failure were different between the pediatric and the adult cohort. Moreover, the pediatric group was characterized, compared to the adult one, by an overall better survival of the graft at ten years after transplant, with the only exception of lung transplants. When comparing survival considering rare vs non-rare diseases or rare and monogenic vs rare non-monogenic conditions, no differences were highlighted for kidney and lung transplants, while rare diseases had a better survival in liver as opposed to heart transplants. CONCLUSIONS: This work represents the first national survey analyzing the main genetic causes and frequencies of rare and/or monogenic diseases leading to organ failure and requiring transplantation both in adults and children.
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Transplante de Rim , Transplante de Órgãos , Criança , Humanos , Itália , Qualidade de Vida , Sistema de Registros , TransplantadosRESUMO
OBJECTIVES: Healthcare systems worldwide have been overburdened by the coronavirus disease 2019 (COVID-19) outbreak. Accordingly, hospitals had to implement strategies to profoundly reshape both non-COVID-19 medical care and surgical activities. Knowledge about the impact of the COVID-19 pandemic on cardiac surgery practice is pivotal. The goal of the present study was to describe the changes in cardiac surgery practices during the health emergency at the national level. METHODS: A 26-question web-enabled survey including all adult cardiac surgery units in Italy was conducted to assess how their clinical practice changed during the national lockdown. Data were compared to data from the corresponding period in 2019. RESULTS: All but 2 centres (94.9%) adopted specific protocols to screen patients and personnel. A significant reduction in the number of dedicated cardiac intensive care unit beds (-35.4%) and operating rooms (-29.2%), along with healthcare personnel reallocation to COVID departments (nurses -15.4%, anaesthesiologists -7.7%), was noted. Overall adult cardiac surgery volumes were dramatically reduced (1734 procedures vs 3447; P < 0.001), with a significant drop in elective procedures [580 (33.4%) vs 2420 (70.2%)]. CONCLUSIONS: This national survey found major changes in cardiac surgery practice as a response to the COVID-19 pandemic. This experience should lead to the development of permanent systems-based plans to face possible future pandemics. These data may effectively help policy decision-making in prioritizing healthcare resource reallocation during the ongoing pandemic and once the healthcare emergency is over.
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COVID-19 , Procedimentos Cirúrgicos Cardíacos , Controle de Doenças Transmissíveis , Humanos , Itália , Pandemias , SARS-CoV-2RESUMO
Anomalous drainage of the inferior vena cava into the left atrium is a rare congenital condition. A 20-year-old girl was referred for recurrent transient ischemic attacks. Transthoracic echocardiography revealed a large ostium secundum atrial septal defect, and computed tomography showed anomalous drainage of the inferior vena cava into the left atrium. Through a right mini-thoracotomy, the opening of the inferior vena cava into the atrium was identified under the inferior edge of the interatrial septum, draining into the left atrium, and redirected to the right atrium, using a pericardial patch to reconstruct the atrial septum. Postoperative course was uneventful. Right mini-thoracotomy approach was effective in correcting the anomalous drainage of the inferior vena cava into the left atrium.
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Procedimentos Cirúrgicos Cardíacos/métodos , Átrios do Coração/anormalidades , Cardiopatias Congênitas/cirurgia , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Veia Cava Inferior/anormalidades , Ecocardiografia , Feminino , Átrios do Coração/cirurgia , Cardiopatias Congênitas/diagnóstico , Humanos , Tomografia Computadorizada por Raios X , Veia Cava Inferior/cirurgia , Adulto JovemRESUMO
Protein-losing enteropathy and chyle leakage may lead to severe malnutrition in heart transplantation for failing Fontan. Nutritional management may be challenging from defining nutrient needs to diagnosis of malnutrition enteropathy, and expertise is necessary. Body composition and hematological nutritional indices may help define malnutrition severity and guide nutritional strategy.
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OBJECTIVES: Italy has been one of the countries most severely affected by the coronavirus disease 2019 (COVID-19). The Italian government was forced to introduce quarantine measures quickly, and all elective health services were stopped or postponed. This emergency has dramatically changed the management of paediatric and adult patients with congenital heart disease. We analysed data from 14 Italian congenital cardiac surgery centres during lockdown, focusing on the impact of the pandemic on surgical activity, patients and healthcare providers and resource allocation. METHODS: Fourteen centres participated in this study. The period analysed was from 9 March to 4 May. We collected data on the involvement of the hospitals in the treatment of patients with COVID-19 and on limitations on regular activity and on the contagion among patients and healthcare providers. RESULTS: Four hospitals (29%) remained COVID-19 free, whereas 10 had a 39% reduction in the number of beds for surgical patients, especially in the northern area. Two hundred sixty-three surgical procedures were performed: 20% elective, 62% urgent, 10% emergency and 3% life-saving. Hospital mortality was 0.4%. Compared to 2019, the reduction in surgical activity was 52%. No patients operated on had positive test results before surgery for severe acute respiratory syndrome coronavirus 2, the virus responsible for COVID-19. Three patients were infected during the postoperative period. Twenty-nine nurses and 12 doctors were infected. Overall, 80% of our infected healthcare providers were in northern centres. CONCLUSIONS: Our study shows that the pandemic had a different impact on the various Italian congenital cardiac surgery centres based on the different patterns of spread of the virus across the country. During the lockdown, the system was able to satisfy all emergency clinical needs with excellent results.
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COVID-19/prevenção & controle , Procedimentos Cirúrgicos Cardíacos/tendências , Alocação de Recursos para a Atenção à Saúde/tendências , Acessibilidade aos Serviços de Saúde/tendências , Cardiopatias Congênitas/cirurgia , COVID-19/epidemiologia , Infecção Hospitalar/epidemiologia , Infecção Hospitalar/prevenção & controle , Procedimentos Cirúrgicos Eletivos/tendências , Emergências , Alocação de Recursos para a Atenção à Saúde/métodos , Alocação de Recursos para a Atenção à Saúde/organização & administração , Pesquisas sobre Atenção à Saúde , Política de Saúde , Acessibilidade aos Serviços de Saúde/organização & administração , Humanos , Controle de Infecções/métodos , Itália/epidemiologia , Doenças Profissionais/epidemiologia , Doenças Profissionais/prevenção & controle , Pandemias , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/prevenção & controle , QuarentenaRESUMO
OBJECTIVES: Repair of Tetralogy of Fallot (TOF) has currently excellent results with either transventricular or transatrial approach. However, it is unclear as to which has better late outcomes and what role of residual pulmonary valve (PV) regurgitation in the long term is. We report on late clinical outcomes after repair in a large series of patients with TOF, focusing on the type of surgical technique. METHODS: This analysis is a retrospective multicentre study on patients undergoing TOF repair in infancy. The exclusion criteria of the study were TOF with pulmonary atresia or absent PV. RESULTS: We selected 720 patients who had undergone TOF repair (median age 5.7 months, interquartile range 3.7-11.7). Preoperative cyanotic spells occurred in 18%. A transatrial repair was performed in 433 (60.1%) patients. The PV was preserved in 249 (35%) patients, while the right ventricular outflow tract was reconstructed with a transannular patch (60.4%) or a conduit (4.6%) in the rest of the patients. At a median follow-up of 4 years (range 1-21, 86% complete), 10 (1.6%) patients died, while 39 (6.3%) patients required surgical reoperation and 72 (11.7%) patients required an interventional procedure. The propensity match analysis showed that the incidence of postoperative complications and adverse events at follow-up were significantly increased in patients undergoing transventricular approach repair with transannular patch (P = 0.006) and PV preservation was a significant protective factor against postoperative complications (P = 0.009, odds ratio 0.5) and late adverse events (P = 0.022). CONCLUSIONS: Surgical repair of TOF in infancy is a safe procedure, with good late clinical outcomes. However, transatrial approach and PV preservation at repair are associated with lower early and late morbidity.
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Procedimentos Cirúrgicos Cardíacos , Valva Pulmonar , Tetralogia de Fallot , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Seguimentos , Humanos , Lactente , Reoperação , Estudos Retrospectivos , Tetralogia de Fallot/cirurgia , Resultado do TratamentoRESUMO
Although few cases of bacteremia or sepsis caused by probiotics have been reported, it is important to consider their pathogenic potential, especially in some categories of patients. We report a case of Bifidobacterium spp bacteremia in a child with heart disease, undergoing probiotic supplementation to prevent antibiotic-associated diarrhea.
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Bacteriemia/microbiologia , Infecções por Bifidobacteriales/microbiologia , Bifidobacterium longum , Insuficiência Cardíaca/complicações , Probióticos/efeitos adversos , Antibacterianos/uso terapêutico , Bacteriemia/tratamento farmacológico , Infecções por Bifidobacteriales/tratamento farmacológico , Bifidobacterium longum/isolamento & purificação , Esquema de Medicação , Oxigenação por Membrana Extracorpórea , Feminino , Febre/microbiologia , Insuficiência Cardíaca/microbiologia , Comunicação Interatrial/complicações , Humanos , Lactente , Insuficiência da Valva Mitral/congênitoRESUMO
Capillary leak syndrome is a critical condition occasionally occurring posttransplant and is characterized by acute endothelial hyperpermeability leading to systemic protein-rich fluid extravasation and consequent hypovolemia, hypoperfusion, and acute kidney injury. Treatment is merely supportive and is based on osmotic drugs, diuretics, continuous renal replacement therapy, and surgical drainage. However, removal of the underlying inflammatory cause is mandatory to achieve stable resolution. Herein, we report the first successful treatment with colchicine in 2 life-threatening pediatric cases of capillary leak syndrome with renal failure occurring after transplant (heart and bone marrow) and unresponsive to any other line of therapy. Both cases were only palliated by supportive therapy and revealed an impressively rapid response to colchicine both in terms of diuresis and clinical condition recovery, allowing for the cessation of renal replacement therapy in a few hours. In both patients, colchicine was temporarily discontinued for transient leukopenia (attributed to an additive effect with mycophenolate mofetil), resulting in extravasation, and renal failure recurrence was restored only after colchicine reintroduction. Although the association of colchicine with an immunosuppressive drug was formerly contraindicated, no other adverse events were noted when using a minimized dose. Both patients are now maintaining a good renal function without recurrence of extravasation after 6 months of follow-up. In conclusion, this strikingly positive experience forces physicians to consider this old and cost-effective drug as a new, powerful rescue tool in such critical cases.
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Síndrome de Vazamento Capilar/tratamento farmacológico , Colchicina/administração & dosagem , Transplante de Coração/efeitos adversos , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Insuficiência Renal/tratamento farmacológico , Moduladores de Tubulina/administração & dosagem , Adolescente , Síndrome de Vazamento Capilar/diagnóstico , Síndrome de Vazamento Capilar/etiologia , Criança , Feminino , Transplante de Coração/tendências , Transplante de Células-Tronco Hematopoéticas/tendências , Humanos , Masculino , Insuficiência Renal/diagnóstico , Insuficiência Renal/etiologiaRESUMO
OBJECTIVES: The population of ageing adults with congenital heart disease (ACHD) is increasing; surgery in these patients presents major difficulties in management. A great debate has developed about whether these patients should be cared for at an adult or paediatric hospital and by an acquired or congenital cardiac surgeon. We analysed data of the surgical treatment of ACHD from the Italian cardiac surgery centres in 2016, focusing on the type of surgery performed, where these patients were operated on and by whom. METHODS: Ninety-two Italian cardiac surgery centres were contacted and 70 centres participated in this study. We collected data on the types of cardiac operations performed in congenital heart defect patients older than 18 years. In 2016, a total of 913 patients with ACHD were operated on: 440 by congenital cardiac surgeons (group I) in centres with paediatric and adult cardiac surgery units, and 473 by adult cardiac surgeons (group II) in centres with exclusively adult cardiac surgery units. RESULTS: Pathologies of the right ventricular outflow tract were the most frequent diseases treated in group I and pathologies of the left ventricular outflow tract in group II. Group I included more complex and heterogeneous cases than group II. Surgery for ACHD represented 12% of the activity of congenital cardiac surgeons and only 1% of the activity of adult cardiac surgeons. CONCLUSIONS: In Italy, ACHD patients are operated on both by congenital and adult cardiac surgeons. Congenital cardiac surgeons working in centres with both paediatric and adult cardiac surgery are more involved with ACHD patients and with more complex cases.
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Anomalous origin of coronary arteries is a rare congenital defect and usually presents as an isolated defect. In only 5% of cases, it may be associated with other cardiac anomalies. In this article, we present a case of a newborn with a very complex congenital cardiac disease accompanied with an anomalous origin of an additional coronary artery.
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Anomalias dos Vasos Coronários/patologia , Cardiopatias Congênitas/patologia , Cardiopatias Congênitas/cirurgia , Artéria Pulmonar/anormalidades , Aorta/anormalidades , Aorta/cirurgia , Aorta Torácica/anormalidades , Aorta Torácica/cirurgia , Anomalias dos Vasos Coronários/cirurgia , Humanos , Recém-Nascido , Artéria Pulmonar/cirurgiaRESUMO
The management of failing Fontan patients with mechanical circulatory support has been reported with unsatisfactory results. A 5-year-old girl, listed for heart transplantation for failing Fontan, underwent rescue extracorporeal membrane oxygenation support. As it was necessary to move the patient to the Berlin Heart Excor, a step-by-step approach was designed to obtain a gradual adaptation to this form of support. A central extracorporeal membrane oxygenation, with a Berlin Heart apical and aortic cannulae, was implanted leaving the previously positioned femoral vein cannula in parallel with the outflow apical one. The femoral vein cannula was removed when the lungs recovered from the effects of surgical trauma. Four days later, the Fontan fenestration was closed, and after 4 more days, a gradual reduction of gas support was planned without problems. Oxygenator was then removed, and 4 days later, a 30-ml Berlin Heart EXCOR ventricle replaced the centrifugal pump. This sequential approach, which has never been described before, can improve the results of the ventricular assist device support in failing Fontan patients, because it allows a gradual adaptation, even in patients with damaged lungs.