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1.
Pediatr Cardiol ; 2024 May 27.
Artículo en Inglés | MEDLINE | ID: mdl-38802599

RESUMEN

The population of adults with congenital heart disease (ACHD) is constantly growing. There seems to be a consensus that these patients are difficult to manage especially if compared to patients with acquired heart disease. The aim of this study is to compare outcomes and results of cardiac surgery in ACHD patients with a reference population of adults with acquired cardiac disease. Retrospective study of 5053 consecutive patients older than 18 years hospitalized for cardiac surgery during a 5-years period in our Institution. Two groups of patients were identified. Group I: 419 patients operated for congenital heart disease; Group II: 4634 patients operated for acquired heart disease. In each Group were identified low, medium, and high-risk patients, according to validated scores. Right ventricular outflow tract surgery was the most frequent procedure in Group I, while coronary artery by-pass grafting was the most common in Group II. Patients with ACHD were younger (37.8 vs. 67.7 years), with higher number of previous operations (32.1% vs. 6.9%), had longer post-ICU hospital stay (11 vs. 8 days) but had lower ICU stay (1 vs. 2 days), shorter assisted mechanical ventilation (12 vs. 14 h) and lower surgical mortality (1 vs. 3.7%) (all p < 0.001). No differences were found in term of post-operative complications (12.4 vs. 15%). The surgical treatment of ACHD patients can be done with excellent results and if compared with acquired cardiac disease patients they have better results with shorter ICU stay and lower mortality.

2.
Pediatr Cardiol ; 44(3): 663-673, 2023 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-35994068

RESUMEN

Aortic valve replacement early in life may be inevitable. Ross operation, until present day, remains the favorite surgical option in pediatrics with irreparable aortic valve disease. Nonetheless, the necessity for re-operation was always its principal limitation due to aortic valve failure or homograft degeneration. We present our 25 years of experience in the pediatric population. From August 1994 until June 2018, 157 children below 18 years underwent the Ross operation. This retrospective review aims at assessing the long-term outcomes, as well as the risk factors for re-operation after Ross procedure. Median age was 10.9 years, of which seven patients were infants, 79 children, and 71 adolescents. The median follow-up time was 14 years. Hospital mortality was 0.6%. Freedom from autograft re-operation for children was 96.7% and 94.1% at 10 and 20 years, respectively; whereas for adolescents, it was 92.6% and 74.9% at 10 and 20 years. For children, freedom from homograft re-operation was 92.5%, 83.5%, and 56.2% at 10, 15, and 20 years; while for adolescents, it was 96.8%, 91.8%, and 86.7% at 10, 15, and 20 years. Homograft size (p = 0.008) and childhood (p = 0.05) were risk factors for homograft re-operation. Pulmonary valve diameter > 24 mm (p = 0.044) and adolescence (p = 0.032) were risk factors for autograft re-operation. Our experience demonstrated excellent early and late survival. While children have preferential outcomes concerning autograft re-operation, those who received a smaller homograft had a higher right-sided re-intervention incidence than adolescents. Pulmonary diameter > 24 mm at surgery was an indicator of future autograft failure.


Asunto(s)
Insuficiencia de la Válvula Aórtica , Estenosis de la Válvula Aórtica , Implantación de Prótesis de Válvulas Cardíacas , Prótesis Valvulares Cardíacas , Válvula Pulmonar , Lactante , Adolescente , Niño , Humanos , Estenosis de la Válvula Aórtica/cirugía , Insuficiencia de la Válvula Aórtica/cirugía , Válvula Aórtica/cirugía , Implantación de Prótesis de Válvulas Cardíacas/métodos , Válvula Pulmonar/cirugía , Resultado del Tratamiento , Reoperación , Estudios de Seguimiento
3.
J Med Syst ; 47(1): 64, 2023 May 17.
Artículo en Inglés | MEDLINE | ID: mdl-37195484

RESUMEN

In this paper, we present an exploratory study on the potential impact of holographic heart models and mixed reality technology on medical training, and in particular in teaching complex Congenital Heart Diseases (CHD) to medical students. Fifty-nine medical students were randomly allocated into three groups. Each participant in each group received a 30-minute lecture on a CHD condition interpretation and transcatheter treatment with different instructional tools. The participants of the first group attended a lecture in which traditional slides were projected onto a flat screen (group "regular slideware", RS). The second group was shown slides incorporating videos of holographic anatomical models (group "holographic videos", HV). Finally, those in the third group wore immersive, head-mounted devices (HMD) to interact directly with holographic anatomical models (group "mixed reality", MR). At the end of the lecture, the members of each group were asked to fill in a multiple-choice questionnaire aimed at evaluating their topic proficiency, as a proxy to evaluate the effectiveness of the training session (in terms of acquired notions); participants from group MR were also asked to fill in a questionnaire regarding the recommendability and usability of the MS Hololens HMDs, as a proxy of satisfaction regarding its use experience (UX). The findings show promising results for usability and user acceptance.


Asunto(s)
Cardiopatías Congénitas , Estudiantes de Medicina , Humanos , Aprendizaje
4.
Eur Heart J ; 42(26): 2527-2535, 2021 07 08.
Artículo en Inglés | MEDLINE | ID: mdl-34021343

RESUMEN

Adult congenital heart disease (ACHD) patients represent a growing population with increasing use of acute emergency department (ED) care. Providing comprehensive ED care necessitates an understanding of the most common clinical scenarios to improve morbidity and mortality in this population. The aim of this position document is to provide a consensus regarding the management of the most common clinical scenarios of ACHD patients presenting to the ED.


Asunto(s)
Medicina de Emergencia , Cardiopatías Congénitas , Cirugía Torácica , Adulto , Consenso , Servicio de Urgencia en Hospital , Cardiopatías Congénitas/cirugía , Humanos
5.
Thorac Cardiovasc Surg ; 68(2): 99-106, 2020 03.
Artículo en Inglés | MEDLINE | ID: mdl-30021241

RESUMEN

BACKGROUND: The management of patients with mechanical heart valves remains a major concern in populations with limited resources and medical facilities. This study reports the clinical outcomes of patients who underwent mechanical valve implantation in a sub-Saharan center over an 8-year period. METHODS: A total of 291 mechanical valves were implanted in 233 patients in our institution between February 2008 and June 2016. A total of 117 patients underwent mitral valve replacement (MVR, 50.2%), 57 had aortic valve replacement (AVR, 24.4%), and 59 underwent both AVR and MVR (double valve replacement [DVR], 25.7%). The mean age at surgery was 27.6 ± 13.4 years (range, 7-62 years). Rheumatic etiology was found in 80.6% of the patients. Hospital mortality, late deaths, and valve-related events were reviewed at follow-up (839 patient-years, range: 1-9.4 years, complete in 93%). RESULTS: The 30-day mortality was 4.7% (11/233). The overall survival at 1 and 6 years for the whole cohort was 88.8 ± 2.1% and 78.7 ± 3.3%, respectively. The 6-year survival for AVR, MVR, and DVR was 89.3 ± 4.8%, 73.2 ± 5.4%, and 79.3 ± 5.8%, respectively (p = 0.15). The freedom from neurologic events and anticoagulation-related bleeding at 6 years was 93.1 ± 2.1% and 78.9 ± 3.7%, respectively. No patient had reoperation at follow-up. No case of prosthetic valve thrombosis was identified. Eight full-term pregnancies were reported. CONCLUSION: This preliminary experience reports acceptable midterm results after mechanical heart valve implantation in our region. Both accurate surgical evaluation and strategies, either financial or social, facilitating patient's education and medical assistance are crucial to ensure good results. Long-term follow-up and further studies comparing current nonthrombogenic options are warranted to draw reliable conclusions.


Asunto(s)
Enfermedades de las Válvulas Cardíacas/cirugía , Implantación de Prótesis de Válvulas Cardíacas/instrumentación , Prótesis Valvulares Cardíacas , Válvulas Cardíacas/cirugía , Adolescente , Adulto , Camerún , Niño , Femenino , Enfermedades de las Válvulas Cardíacas/diagnóstico por imagen , Enfermedades de las Válvulas Cardíacas/mortalidad , Enfermedades de las Válvulas Cardíacas/fisiopatología , Implantación de Prótesis de Válvulas Cardíacas/efectos adversos , Implantación de Prótesis de Válvulas Cardíacas/mortalidad , Válvulas Cardíacas/diagnóstico por imagen , Válvulas Cardíacas/fisiopatología , Mortalidad Hospitalaria , Humanos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/mortalidad , Embarazo , Estudios Retrospectivos , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento , Adulto Joven
6.
Perfusion ; 34(8): 629-636, 2019 11.
Artículo en Inglés | MEDLINE | ID: mdl-31250738

RESUMEN

INTRODUCTION: Low-weight (<10 kg) children undergoing cardiac surgery with cardiopulmonary bypass are prone to dilution and consumption of soluble coagulation factors and fibrinogen. Low levels of fibrinogen may represent a possible cause of severe postoperative chest drain blood loss. The present study investigates the association between post-cardiopulmonary bypass fibrinogen levels and postoperative chest drain blood loss and severe bleeding, aiming to identify possible cut-off values to trigger specific interventions. METHODS: Prospective cohort study on 77 patients weighing <10 kg undergoing cardiac surgery with cardiopulmonary bypass. Haemostasis and coagulation data were collected before surgery (standard tests and thromboelastometry), after protamine (thromboelastometry) and at the arrival in the intensive care unit (standard tests). The primary outcome variable was severe bleeding (chest drain blood loss >30 ml kg-1/24h). RESULTS: Factors being independently associated with severe bleeding were the international normalized ratio and the fibrinogen levels at the arrival in the intensive care unit. Once corrected for other confounders, fibrinogen levels had an odds ratio of 0.2 (95% confidence interval = 0.011-0.54) per 1 gL-1 for severe bleeding. The discrimination power was fair (area under the curve = 0.770). The best cut-off value was identified at a fibrinogen level of 150 mg dL-1, with a sensitivity of 52%, a specificity of 85% and a positive predictive value of 60% for severe bleeding. CONCLUSION: Both a prolonged international normalized ratio and low fibrinogen levels were predictive for severe bleeding, underscoring the role of coagulation factors dilution and consumption in this specific patient population.


Asunto(s)
Fibrinógeno/análisis , Hemorragia Posoperatoria/sangre , Coagulación Sanguínea , Peso Corporal , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Puente Cardiopulmonar/efectos adversos , Drenaje/efectos adversos , Humanos , Lactante , Hemorragia Posoperatoria/etiología , Hemorragia Posoperatoria/terapia , Estudios Prospectivos
7.
Int J Mol Sci ; 20(24)2019 Dec 11.
Artículo en Inglés | MEDLINE | ID: mdl-31835735

RESUMEN

Alagille syndrome (AGS) is an autosomal-dominant disorder characterized by various degrees of abnormalities in the liver, heart, eyes, vertebrae, kidneys, face, vasculature, skeleton, and pancreas. This case report describes a newborn child exhibiting a congenital neural tube defect and peculiar craniofacial appearance characterized by a prominent forehead, deep-set eyes, bulbous nasal tip, and subtle upper lip. Just a few hours after birth, congenital heart disease was suspected for cyanosis and confirmed by heart evaluation. In particular, echocardiography indicated pulmonary atresia with ventricular septal defect with severe hypoplasia of the pulmonary branches (1.5 mm), large patent ductus arteriosus and several major aortopulmonary collateral arteries. Due to the association of peculiar craniofacial appearance and congenital heart disease, a form of Alagille syndrome was suspected. In addition, on the fifth day after birth, the patient developed jaundice, had acholic stools, and high levels of conjugated bilirubin and gamma-glutamyltransferase (GGT) were detected in the blood. Genetic testing revealed the novel variant c.802del in a single copy of the JAG1 gene. No variants in the NOTCH2 gene were detected. To the best of our knowledge, this is the first clinical description of a congenital neural tube defect in a molecularly confirmed Alagille patient. This work demonstrates a novel pathogenic heterozygous JAG1 mutation is associated with an atypical form of Alagille syndrome, suggesting an increased risk for neural tube defects compared to other Alagille patients.


Asunto(s)
Síndrome de Alagille/genética , Eliminación de Gen , Proteína Jagged-1/genética , Síndrome de Alagille/sangre , Síndrome de Alagille/diagnóstico por imagen , Secuencia de Bases , Bilirrubina/sangre , Electrocardiografía , Femenino , Humanos , Recién Nacido , Masculino , Linaje , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/patología
8.
Europace ; 20(11): 1719-1753, 2018 11 01.
Artículo en Inglés | MEDLINE | ID: mdl-29579186

RESUMEN

The population of patients with congenital heart disease (CHD) is continuously increasing with more and more patients reaching adulthood. A significant portion of these young adults will suffer from arrhythmias due to the underlying congenital heart defect itself or as a sequela of interventional or surgical treatment. The medical community will encounter an increasing challenge as even most of the individuals with complex congenital heart defects nowadays become young adults. Within the past 20 years, management of patients with arrhythmias has gained remarkable progress including pharmacological treatment, catheter ablation, and device therapy. Catheter ablation in patients with CHD has paralleled the advances of this technology in pediatric and adult patients with structurally normal hearts. Growing experience and introduction of new techniques like the 3D mapping systems into clinical practice have been particularly beneficial for this growing population of patients with abnormal cardiac anatomy and physiology. Finally, device therapies allowing maintanence of chronotropic competence and AV conduction, improving haemodynamics by cardiac resynchronization, and preventing sudden death are increasingly used. For pharmacological therapy, ablation procedures, and device therapy decision making requires a deep understanding of the individual pathological anatomy and physiology as well as detailed knowledge on natural history and long-term prognosis of our patients. Composing expert opinions from cardiology and paediatric cardiology as well as from non-invasive and invasive electrophysiology this position paper was designed to state the art in management of young individuals with congenital heart defects and arrhythmias.


Asunto(s)
Arritmias Cardíacas , Procedimientos Quirúrgicos Cardíacos , Cardiología , Muerte Súbita Cardíaca , Cardiopatías Congénitas , Manejo de Atención al Paciente , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/etiología , Arritmias Cardíacas/terapia , Terapia de Resincronización Cardíaca/métodos , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Procedimientos Quirúrgicos Cardíacos/métodos , Cardiología/métodos , Cardiología/tendencias , Ablación por Catéter/métodos , Muerte Súbita Cardíaca/etiología , Muerte Súbita Cardíaca/prevención & control , Desfibriladores Implantables , Técnicas Electrofisiológicas Cardíacas/métodos , Europa (Continente) , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/cirugía , Humanos , Manejo de Atención al Paciente/métodos , Manejo de Atención al Paciente/normas , Adulto Joven
9.
Artif Organs ; 42(9): 911-917, 2018 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-30094862

RESUMEN

Adult congenital heart disease (ACHD) patients are at risk for end-stage heart failure; heart transplantation (Htx) represents the only definitive therapy available although not easily achievable for all patients. The study aims to assess the pathway difficulties and outcomes of ACHD patients with end-stage heart failure referred for Htx evaluation. This is a single center retrospective study on ACHD patients with end-stage heart failure referred to Htx evaluation from 2004 to 2015. Demographic data, medical history, failure modality, and follow-up were obtained from patient charts. End-points were Htx list enrollment, transplant, and survival. Statistical analysis was performed comparing patients listed and not listed. There were 21 ACHD patients with end-stage heart failure referred to Htx evaluation. Transplant listing was declined for 12 (57%) meanwhile 9 patients were listed. Htx was successfully achieved in 3 patients after 24 and 36 months, respectively. Three patients are still on the wait list and three died while waiting, with a listed group mortality of 33.3% (3/9). Mortality occurred in first 18 months after Htx list enrollment. Not listed group mortality was 50% (6/12) and occurred after a median time of 17.5 months (IQR: 9-23 months). There was no difference in survival (P = 0.574) between listed and not listed (89, 63, and 63% vs. 83, 56, and 47% at 12-24-48 months). Follow-up median duration was 27 months (IQR: 14-56 months). Heart transplant listing for ACHD patients with end-stage heart failure is hard to obtain. Almost 2/3 of the patients were declined. Survival for these patients is reduced severely either in waiting list for transplant or excluded indicating the potential need of mechanical circulatory support as bridge to transplant or as destination therapy to improve survival likelihood.


Asunto(s)
Cardiopatías Congénitas/cirugía , Insuficiencia Cardíaca/cirugía , Trasplante de Corazón , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Tiempo , Listas de Espera , Adulto Joven
10.
Europace ; 19(9): 1542-1548, 2017 Sep 01.
Artículo en Inglés | MEDLINE | ID: mdl-27738072

RESUMEN

AIMS: Supraventricular arrhythmias are a major cause of morbidity and mortality in adult patients with congenital heart disease (CHD). Intraoperative ablation offers an alternative for patients who failed ablation procedures or are requiring concomitant surgical intervention. We present our long-term results with the surgical treatment of arrhythmias in adults with CHD (ACHD) undergoing elective cardiac surgery and the clinical predictors for arrhythmia recurrence. METHODS AND RESULTS: Between 2002 and 2013, 80 consecutive patients with CHD, mean age of 39 years, underwent intraoperative ablation with monopolar irrigated radiofrequency during cardiac surgery procedures. Significant clinical predictors of arrhythmia recurrence were determined by univariate analysis. We performed 47 right-sided Maze procedures, and 33 Cox-Maze III procedures. In 75 survivors, the ablation was effective immediately. Over an average follow-up period of 72 months (12-155 months), arrhythmias recurred in nine (20%) patients after right-sided Maze, and in six (19%) patients after Cox-Maze III. Eleven patients were controlled with medical therapy, three underwent catheter ablation of the arrhythmia, and one required a permanent pacemaker. Preoperative arrhythmia length ≥3 years (P ≤ 0.001), tetralogy of Fallot (P ≤ 0.006), and preoperative atrial fibrillation (P ≤ 0.016) were associated with recurrence arrhythmia. Conversely, NYHA class <3 (P ≤ 0.047) was associated with a lower risk of recurrence. CONCLUSION: Surgical treatment of unresponsive supraventricular arrhythmia during concomitant cardiac surgery in ACHD is a safe and effective procedure. Freedom from arrhythmias recurrence is 75% after 6 years of follow-up. Long-term recurrence of arrhythmia in these patients seems to be strongly correlated to preoperative arrhythmia duration.


Asunto(s)
Fibrilación Atrial/cirugía , Aleteo Atrial/cirugía , Ablación por Catéter , Cardiopatías Congénitas/cirugía , Taquicardia Supraventricular/cirugía , Adolescente , Adulto , Anciano , Fibrilación Atrial/diagnóstico , Fibrilación Atrial/etiología , Fibrilación Atrial/fisiopatología , Aleteo Atrial/diagnóstico , Aleteo Atrial/etiología , Aleteo Atrial/fisiopatología , Ablación por Catéter/efectos adversos , Supervivencia sin Enfermedad , Femenino , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/fisiopatología , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Modelos de Riesgos Proporcionales , Recurrencia , Estudios Retrospectivos , Factores de Riesgo , Taquicardia Supraventricular/diagnóstico , Taquicardia Supraventricular/etiología , Taquicardia Supraventricular/fisiopatología , Factores de Tiempo , Resultado del Tratamiento , Adulto Joven
11.
Cardiol Young ; 27(2): 273-283, 2017 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-27086665

RESUMEN

BACKGROUND: In 2007, a partnership was initiated between a small-volume paediatric cardiac surgery unit located in Las Palmas de Gran Canaria, Spain, and a large-volume cardiac surgery unit located in Milan, Italy. The main goal of this partnership was to provide surgical treatment to children with CHD in the Canary Islands. METHODS: An operative algorithm for performing surgery in elective, urgent, and emergency cases was adopted by the this joint programme. Demographic and in-hospital variables were collected from the medical records of all the patients who had undergone surgical intervention for CHD from January, 2009 to March, 2013. Data were introduced into the congenital database of the European Congenital Heart Surgeons Association Congenital Database and the database was interrogated. RESULTS: In total, 65 surgical mission trips were performed during the period of this study. The European Congenital Heart Surgeons Association Congenital Database documented 214 total patients with a mean age at operation of 36.45 months, 316 procedures in total with 198 cardiopulmonary bypass cases, 46 non-cardiopulmonary bypass cases, 26 cardiovascular cases without cardiopulmonary bypass, 22 miscellaneous other types of cases, 16 interventional cardiology cases, six thoracic cases, one non-cardiac, non-thoracic procedure on a cardiac patient with cardiac anaesthesia, and one extracorporeal membrane oxygenation case. The 30-day mortality was 6.07% (13 patients). CONCLUSIONS: A joint programme between a small-volume centre and a large-volume centre may represent a valid and reproducible model for safe paediatric cardiac surgery in the context of a peripheral region.


Asunto(s)
Procedimientos Quirúrgicos Cardíacos/métodos , Cardiología/organización & administración , Cardiopatías Congénitas/cirugía , Evaluación de Resultado en la Atención de Salud , Sociedades Médicas , Especialidades Quirúrgicas/organización & administración , Preescolar , Europa (Continente) , Femenino , Humanos , Masculino , Estudios Retrospectivos
12.
Eur Heart J Suppl ; 18(Suppl E): E19-E21, 2016 Apr 28.
Artículo en Inglés | MEDLINE | ID: mdl-28533711

RESUMEN

Mucormycosis is an angioinvasive infection caused by ubiquitous filamentous fungi of the order Mucorales. We describe a case of rhinocerebral mucormycosis presented in a paediatric patient after cardiac surgery correction of a complete atrioventricular canal defect. The rhinocerebral form of mucormycosis in our patient presented as an important epistaxis that needed immediate intubation due to blood inhalation. Furthermore, due to the worsening of pulmonary function, the patient also needed mechanical support with veno-arterial extracorporeal membrane oxygenation. The patient died as a consequence of a disseminated form of fungal infection. We describe our experience of this rare opportunistic infection and we think that early recognition of the disease could help in proper management.

13.
Eur Heart J Suppl ; 18(Suppl E): E15-E18, 2016 Apr 28.
Artículo en Inglés | MEDLINE | ID: mdl-28533710

RESUMEN

Thanks to the improvement of surgical and interventional cardiac procedures, the majority of children with congenital heart defects (CHD) can now be expected to reach adulthood. The number of adults with congenital heart disease (ACHD) will inevitably increase, and recent data affirm that in Europe, we are actually faced with an estimated patient population of 2.3 million. These patients, particularly the adults with moderate and highly complex CHD, can be very difficult to manage, and should be treated in few experienced and specialized 'grown-up congenital heart disease' (GUCH) units, concentrating resources, patients, funding, and professional experiences. On 2000, we created a GUCH unit in our hospital that rapidly became a referral centre in Italy for number and complexity of patients treated. The present study is a presentation of how is organized our GUCH unit and a brief overview of our more recent experience with ACHD patients.

14.
Eur Heart J Suppl ; 18(Suppl E): E79-E85, 2016 Apr 28.
Artículo en Inglés | MEDLINE | ID: mdl-28533721

RESUMEN

Extracorporeal Membrane Oxygenation (ECMO) represents a useful tool to support the lungs and the heart when all conventional therapies failed and the patients are at risk of death. While the Extracorporeal Life Support Organization (ELSO) collects data from different institutions that joined the Registry and reports overall outcome, individual centres often collide with results below expectations, either in adults and in paediatric population. Some authors suggest that poor outcomes could be overcome with a programme dedicated to ECMO, with specialized professionals adequately trained on ECMO and with a consistent number of procedures. In 2012, The IRCCS PSD ECMO Programme was instituted with the specific aim of achieving better results than hitherto obtained. After only 1 year of activity, the results justified the programme, with a better survival rate for each group investigated, particularly in adults, but surprisingly in paediatrics too, where the results were better than what reported by ELSO. Although the number of patients treated with ECMO is still growing up, the effects of the ECMO programme continue to exert a positive action on outcome even now. The present article reports data on survival, blood loss, and blood consumption during ECMO in the last few years at our institution.

15.
Eur Heart J Suppl ; 18(Suppl E): E72-E78, 2016 Apr 28.
Artículo en Inglés | MEDLINE | ID: mdl-28533720

RESUMEN

Cooperation activity in training programmes promoted by IRCCS Policlinico San Donato and Bambini Cardiopatici nel Mondo Association is a model of cooperation between people as an alternative intervention in promoting the right to healthcare, especially offering programmes of training and medical care on cardiovascular and congenital heart disease. This new strategy, implemented in several developing countries, has absolutely contributed to the improvement of the medical services concerning the diagnostic and surgical approach in the treatment of paediatric and adult cardiovascular disease. To strengthen this kind of activity, both IRCCS Policlinico San Donato and Bambini Cardiopatici nel Mondo have introduced a global perspective aiming at the realization of surgical missions 'in situ', building new cardiac surgery units in collaboration with the local partners, which are mainly university hospitals. They, furthermore, support financially the scholarship and accommodation in favour of medical and paramedical staff.

16.
Pediatr Med Chir ; 37(2): pmc.2015.120, 2015 Sep 29.
Artículo en Inglés | MEDLINE | ID: mdl-26429123

RESUMEN

Williams-Beuren syndrome (WBS) is a genetic disorder caused by elastin gene deletions, and is characterized by cardiovascular malformations, primarily including supravalvular aortic stenosis and peripheral pulmonary stenosis. We report a case of a neonate who developed severe discrete aortic coarctation, underwent multiple surgical interventions, and was subsequently diagnosed with WBS. Severe discrete aortic coarctation is a rare event in WBS newborns. An abnormally thick aortic wall is present in these patients and is the basis of the failure of the classical approach towards coarctation repair, which consists of end-to-end anastomosis as first surgical choice. Our case, and a very few similar previously documented cases, have all demonstrated recoarctation, which only aortic patch implantation was able to successfully repair. In light of this, we would also like to underline the importance of early WBS diagnosis. Therefore, even in mild syndromic phenotype such as low birth weight or facial dysmorphism that raise the suspicion of a genetic syndrome, it is advisable to perform fluorescent in situ hybridization analysis rather than merely karyotypic one.


Asunto(s)
Coartación Aórtica/etiología , Elastina/genética , Síndrome de Williams/fisiopatología , Coartación Aórtica/genética , Femenino , Eliminación de Gen , Humanos , Hibridación Fluorescente in Situ , Recién Nacido , Síndrome de Williams/diagnóstico , Síndrome de Williams/genética
17.
Cardiol Young ; 24(2): 275-82, 2014 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-23534397

RESUMEN

OBJECTIVES: The purpose of this study was to investigate whether the objective medical parameters related to congenital heart disease and patients' ratings of cardiac disease severity were related to psychological well-being and illness behaviour during the pre-operative period. METHODS: A total of 143 patients (63 male patients; 16-73 years old) with congenital heart disease evaluated the severity of their cardiac condition using a numerical rating scale ranging from 0, indicating the least severe condition, to 100, indicating the most severe condition. Psychological well-being was assessed using the Psychological General Well-Being Index (total score ≤ 60 indicating severe distress) and illness behaviour using the Illness Behavior Questionnaire. RESULTS: Pre-operative psychological well-being was not related to the objective medical parameters reflecting cardiac disease severity. In contrast, total psychological well-being scores correlated significantly with patients' subjective ratings of disease severity (p < 0.001). When compared with the reference values from the Italian population, the mean scores of the patients on psychological well-being were similar. As regards the Illness Behavior Questionnaire, the scores on denial were higher and those on hypochondria were lower compared with other hospitalised patients. CONCLUSIONS: This study shows that the perception of cardiac disease severity, and not the medical parameters in congenital heart disease, is related to the patients' pre-operative psychological state. Thus, more importance needs to be given to assessing the patients' pre-operative perception and psychological state independently of cardiac severity. Targeted interventions with regard to the cardiac condition are recommended.


Asunto(s)
Actitud Frente a la Salud , Cardiopatías Congénitas/psicología , Conducta de Enfermedad , Periodo Preoperatorio , Estrés Psicológico/psicología , Adolescente , Adulto , Anciano , Negación en Psicología , Femenino , Cardiopatías Congénitas/fisiopatología , Cardiopatías Congénitas/cirugía , Humanos , Hipocondriasis/psicología , Masculino , Persona de Mediana Edad , Satisfacción Personal , Calidad de Vida , Índice de Severidad de la Enfermedad , Encuestas y Cuestionarios , Adulto Joven
18.
Heart ; 110(4): 235-244, 2024 Jan 29.
Artículo en Inglés | MEDLINE | ID: mdl-37487694

RESUMEN

Although survival has significantly improved in the last four decades, the diagnosis of Ebstein's anomaly is still associated with a 20-fold increased risk of mortality, which generally drops after neonatal period and increases subtly thereafter. With increasing age of presentation, appropriate timing of intervention is challenged by a wide spectrum of disease and paucity of data on patient-tailored interventional strategies. The present review sought to shed light on the wide grey zone of post-neonatal Ebstein's manifestations, highlighting current gaps and achievements in knowledge for adequate risk assessment and appropriate therapeutic strategy.A 'wait-and-see' approach has been adopted in many circumstances, though its efficacy is now questioned by the awareness that Ebstein's anomaly is not a benign disease, even when asymptomatic. Moreover, older age at intervention showed a negative impact on post-surgical outcome.In order to tackle the extreme heterogeneity of Ebstein's anomaly, this review displays the multimodality imaging assessment necessary for a proper anatomical classification and the multidisciplinary approach needed for a comprehensive risk stratification and monitoring strategy. Currently available predictors of clinical outcome are summarised for both operated and unoperated patients, with the aim of supporting the decisional process on the choice of appropriate therapy and optimal timing for intervention.


Asunto(s)
Anomalía de Ebstein , Recién Nacido , Niño , Adulto , Humanos , Anomalía de Ebstein/diagnóstico por imagen , Anomalía de Ebstein/cirugía , Medición de Riesgo , Imagen Multimodal
19.
Diagnostics (Basel) ; 14(5)2024 Feb 21.
Artículo en Inglés | MEDLINE | ID: mdl-38472942

RESUMEN

We describe a case of a voluminous rhabdomyoma (R) detected by fetal echocardiography at 32 weeks' gestation (w.g.) obstructing the left ventricular inflow and aortic outflow tract, with a moderate aortic gradient at birth, not needing immediate surgery. At follow-up, the mass progressively regressed, leaving the aortic valve partly damaged, with a gradient that increased to a maximum of 100 mmHg at 9 years. The girl was then operated on successfully by a plasty of the aortic valve. The literature regarding R is discussed.

20.
J Cardiovasc Med (Hagerstown) ; 25(1): 76-87, 2024 01 01.
Artículo en Inglés | MEDLINE | ID: mdl-38079284

RESUMEN

AIMS: Congenital heart diseases (CHDs) often show a complex 3D anatomy that must be well understood to assess the pathophysiological consequences and to guide therapy. Three-dimensional imaging technologies have the potential to enhance the physician's comprehension of such spatially complex anatomies. Unfortunately, due to the new introduction in clinical practice, there is no evidence on the current applications. We conducted a survey to examine how 3D technologies are currently used among CHD European centres. METHODS: Data were collected using an online self-administered survey via SurveyMonkey. The questionnaire was sent via e-mail and the responses were collected between January and June 2022. RESULTS: Ninety-eight centres correctly completed the survey. Of these, 22 regularly perform 3D rotational angiography, 43 have the availability to print in-silico models, and 22 have the possibility to visualize holographic imaging/virtual reality. The costs were mostly covered by the hospital or the department of financial resources. CONCLUSION: From our survey, it emerges that these technologies are quite spread across Europe, despite not being part of a routine practice. In addition, there are still not enough data supporting the improvement of clinical management for CHD patients. For this reason, further studies are needed to develop clinical recommendations for the use of 3D imaging technologies in medical practice.


Asunto(s)
Cardiopatías Congénitas , Humanos , Cardiopatías Congénitas/diagnóstico por imagen , Imagenología Tridimensional , Encuestas y Cuestionarios , Impresión Tridimensional , Modelos Anatómicos
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