Cirugía de cono en la enfermedad de Ebstein. Primeros casos / The cone technique for Ebstein Repair. First cases

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Beating-heart aortic arch surgery in neonates and infants.

OBJECTIVES: Aortic arch repair has been shifted from deep hypothermia plus circulatory arrest to cerebral perfusion at tepid temperatures. A step forward is a simultaneous brain-coronary perfusion, allowing beating-heart arch surgery. METHODS: A 'Y' cannula from the arterial line delivers oxygenated blood to brain and heart. The arch is repaired on a beating heart at 25°C. Intracardiac repair is performed after running cardioplegia through the root line. Fifty patients are classified into 3 groups: A, Norwood (8 neonates); B, aortic arch (14 children) and C, aortic arch plus intracardiac repair (28 patients). Associated anomalies in Group C are as follows: ventricular septal defect (10), arterial switch (5), atrial septal defect (4), cor triatriatum (3), aortic commissurotomy (2), comprehensive repair (2), ostium primum (1) and Yasui (1). RESULTS: The mean bypass time was 161 ± 54.44 (range 93-312) min. Mean brain-coronary perfusion was 37.26 ± 10.54 (18-60) min. Mean coronary ischaemia was 31 ± 32.40 (0-160) min. The heart was not arrested in Group B patients. Follow-up was complete for a mean of 30 (1-48) months. Four patients died in the postoperative period. Two required angioplasty for recoarctation. CONCLUSIONS: Selective brain-coronary perfusion is feasible and easy to switch to conventional cardioplegia delivery. Coronary ischaemia can be notably reduced and even 0 min in isolated arch surgery.

Myocarditis, flail tricuspid valve, and normal rhythm: an exceptional form of neonatal cardiac lupus.

Neonatal cardiac lupus is a rare, passively acquired autoimmune disease. We report a case of in utero myocarditis, confirmed postnatally, with papillary muscle rupture and severe tricuspid regurgitation after birth in the absence of conduction disturbances. Tricuspid repair was successfully performed with polytetrafluoroethylene neochordae. In this article, we discuss the pathophysiology, medical and surgical management, and implications at follow-up in this unique scenario.

Pediatric Heart Transplantation With Lecompte Maneuver Owing to Extremely Oversized Donor Allograft.

The techniques and outcomes of heart transplantation in the pediatric population continue to improve over the years, although the supply of organs remains limited. Donor-to-recipient size matching is critical, especially in neonates and small infants. We present a novel strategy for heart transplantation that includes the Lecompte maneuver because of the features of the donor allograft available in a 8-month-old patient with a cardiac fibroma. We discuss the basis principles for extending the indication of this procedure to exceptional transplantation scenarios and describe the results at long-term follow-up.

How to set-up a program of minimally-invasive surgery for congenital heart defects.

BACKGROUND: Mid-line sternotomy is the commonest incision for cardiac surgery. Alternative approaches are becoming fashionable in many centres, amidst some reluctance because of learning curves and overall complexity. Our recent experience in starting a new program on minimally invasive pediatric cardiac surgery is presented. The rationale for a stepwise onset and the short-medium term results for a three-year span are displayed. METHODS: A three-step schedule is planned: First, an experienced surgeon (A) starts performing simple cases. Second, new surgeons (B, C, D, E) are introduced to the minimally invasive techniques according to their own proficiency and skills. Third, the new adopters are enhanced to suggest and develop further minimally invasive approaches. Two quality markers are defined: conversion rate and complications. RESULTS: In part one, surgeon A performs sub-mammary, axillary and lower mini-sternotomy approaches for simple cardiac defects. In part two, surgeons B, C, D and E are customly introduced to such incisions. In part three, new approaches such as upper mini-sternotomy, postero-lateral thoracotomy and video-assisted mini-thoracotomy are introduced after being suggested and developed by surgeons B, C and E, as well as an algorithm to match cardiac conditions and age/weight to a given alternative approach. The conversion rate is one out of 148 patients. Two major complications were recorded, none of them related to our alternative approach. Four minor complications linked to the new incision were registered. The minimally invasive to mid-line sternotomy ratio rose from 20% in the first year to 40% in the third year. CONCLUSIONS: Minimally invasive pediatric cardiac surgery is becoming a common procedure worldwide. Our schedule to set up a program proves beneficial. The three-step approach has been successful in our experience, allowing a tailored training for every new surgeon and enhancing the enthusiasm in developing further strategies on their own. Recording conversion-rates and complications stands for quality standards. A twofold increase in minimally invasive procedures was observed in two years. The short-medium term results after three years are excellent.

Split-graft technique in neonatal heart transplant for aortic atresia.

We describe a neonate with aortic atresia and hypoplastic aorta, listed for heart transplant after extracorporeal membrane oxygenation resuscitation and ductal stenting. The donor aorta was detached from the graft, after an isolated arch reconstruction prior to the transplant itself in a routine fashion. To the best of our knowledge, this is the first reported case of neonatal arch reconstruction before transplantation performed with grafts from the same donor in a split-way strategy.

Batista Procedure for a Coronary Anomaly in an Infant: Long-Term Follow-Up.

A partial left ventriculectomy (Batista operation) is extremely unusual in infants. Follow-up data are unknown. We report the findings at long-term follow-up in an infant after the Batista procedure, following reimplantation of an anomalous coronary artery. The initial approach and options for management are discussed, along with late cardiac remodeling. doi: 10.1111/jocs.12775 (J Card Surg 2016;31:556-558).

15 años de cirugía cardiaca infantil miniinvasiva; evolución y tendencias / 15 years of minimally invasive paediatric cardiac surgery; development and trends

INTRODUCCIÓN: Los abordajes miniinvasivos en cirugía cardiaca infantil no son habituales. Pocos grupos presentan programas y experiencia, quedando limitados a cardiopatías simples. Presentamos una casuística recopilada a lo largo de 15 años y más de 200 pacientes. MATERIAL Y MÉTODOS: En el año 2000 comenzamos un programa de acceso submamario, ampliándolo progresivamente a casos complejos y edades menores. En 2009 incorporamos el abordaje axilar, con idénticas pautas. En 2013 iniciamos la cirugía por miniesternotomía inferior, ampliando la cartera de servicios. Entre julio del 2000 y diciembre del 2014 se intervino a 203 pacientes: 102 por vía submamaria, 50 por axilar, 44 por miniesternotomía inferior, 4 por toracotomía lateral-posterior y 3 por miniesternotomía superior. RESULTADOS: Por patologías, la más frecuente fue la comunicación interauricular ostium secundum (128), seguida del seno venoso (20), comunicación interventricular (20), ostium primum (16) y otras (19). Un caso fue reconvertido a esternotomía. No hubo eventos neurológicos. Las medias de edad fueron 7,8/3,7 y 1,8 años, con medias de peso de 28,1/16,1 y 9,4 kg en los accesos submamario, axilar y miniesternotomía, respectivamente. Los resultados estéticos han sido excelentes. CONCLUSIONES: Tras 15 años de experiencia acumulada, la cirugía miniinvasiva es segura y eficaz, con resultados estéticos excelentes. La introducción gradual de diferentes accesos alternativos permite establecer pautas de aprendizaje. La versatilidad de accesos (submamario, axilar, miniesternotomía) facilita la selección de abordajes en función de cardiopatía y edad/peso

INTRODUCTION: The minimally invasive approach is seldom reported in paediatric cardiac surgery. Teams gathering experience are scarce, with programs focused on simple cases. The experience is presented on a series of over 200 cases operated on in the past 15 years. MATERIAL AND METHODS: A sub-mammary approach program was started in 2000, which was gradually extended to include more complex and younger patients. The axillary incision was adopted in 2009, following the same steps. In 2013, the mini-sternotomy incision was introduced, increasing our armamentarium. From July 2000 until December 2014, 203 patients were operated on. The sub-mammary approach was used in 102 cases, axillary in 50 patients, mini-sternotomy in 44, postero-lateral thoracotomy in 4 cases, and upper mini-sternotomy in 3. RESULTS: By diagnosis, ostium secundum atrial septal defect was the most common (128), followed by sinus venosus (20), ventricular septal defect (20), ostium primum (16), and others (19). One patient was converted to sternotomy. No neurological events were detected. The mean age was 7.8/3.7 and 1.8 years, and the mean weight was 28.1/16.1 and 9.4 Kg. in the sub-mammary, axillary and mini-sternotomy approaches, respectively. The aesthetic results were excellent. CONCLUSIONS: Based on our 15 years of experience, minimally invasive surgery is safe and yields excellent cosmetic results. The gradual introduction of alternative approaches (sub-mammary, axillary, mini-sternotomy) allowed us to set-up guidelines and learning curves. The wide range of incisions enables the most appropriate one to be selected depending on age/weight and cardiac condition

Combined Ablation and Exclusion of the Left Atrial Appendage in a Pediatric Patient: A Minimally Invasive Simplified Approach.

We present the first reported case of totally video-assisted thoracoscopic surgery for combined ablation and external exclusion without appendectomy of the left atrial appendage. We encourage the use of video-assisted thoracoscopic surgery for a combined procedure of epicardial radiofrequency ablation and external exclusion without appendectomy to achieve a safe and complete electrical isolation of the left atrial appendage in children with focal atrial tachycardia. In our opinion, the external exclusion of the appendage has several advantages: it presents minimal risk of bleeding (especially in the pediatric population), and the clip can be retrieved if the circumflex artery is distorted.

End-stage ischemic heart failure and Williams-Beuren syndrome: A unique scenario for pediatric heart transplantation.

WBS is a rare disorder caused by mutations in the chromosomal sub-band 7q11.23 involving the elastin gene. The clinical features (craniofacial, developmental, and cardiovascular abnormalities) are variable. The association with cardiac anomalies is a well-recognized feature, and SVAS is the most common cardiac defect found. End-stage ischemic heart disease is unusual in this setting but when it occurs, OHT remains the final therapeutic option. This decision can be difficult to determine, and it must be tailored to the individual patient based on the clinical status and concomitant cardiovascular and multisystem lesions. To date, no cases of OHT in patients with WBS have been described. We present a 14-month-old patient with WBS who developed severe LV dysfunction secondary to ischemia following a complex staged surgery for SVAS repair. He underwent successful OHT with no post-operative complications, and at three-month follow-up, he remains asymptomatic on standard immunosuppressive therapy. This case constitutes the first demonstration that OHT may be indicated for extended survival in selected children with WBS and we discuss the basic principles for extending the indication for OHT to this scenario as well as the particularities for post-transplant care.

[15 years of minimally invasive paediatric cardiac surgery; development and trends]. / 15 años de cirugía cardiaca infantil miniinvasiva; evolución y tendencias.

INTRODUCTION: The minimally invasive approach is seldom reported in paediatric cardiac surgery. Teams gathering experience are scarce, with programs focused on simple cases. The experience is presented on a series of over 200 cases operated on in the past 15 years. MATERIAL AND METHODS: A sub-mammary approach program was started in 2000, which was gradually extended to include more complex and younger patients. The axillary incision was adopted in 2009, following the same steps. In 2013, the mini-sternotomy incision was introduced, increasing our armamentarium. From July 2000 until December 2014, 203 patients were operated on. The sub-mammary approach was used in 102 cases, axillary in 50 patients, mini-sternotomy in 44, postero-lateral thoracotomy in 4 cases, and upper mini-sternotomy in 3. RESULTS: By diagnosis, ostium secundum atrial septal defect was the most common (128), followed by sinus venosus (20), ventricular septal defect (20), ostium primum (16), and others (19). One patient was converted to sternotomy. No neurological events were detected. The mean age was 7.8/3.7 and 1.8 years, and the mean weight was 28.1/16.1 and 9.4 Kg. in the sub-mammary, axillary and mini-sternotomy approaches, respectively. The aesthetic results were excellent. CONCLUSIONS: Based on our 15 years of experience, minimally invasive surgery is safe and yields excellent cosmetic results. The gradual introduction of alternative approaches (sub-mammary, axillary, mini-sternotomy) allowed us to set-up guidelines and learning curves. The wide range of incisions enables the most appropriate one to be selected depending on age/weight and cardiac condition.

Orthotopic Heart Transplantation in an Adult Patient with Heterotaxy Syndrome: Surgical Implications.

Modified techniques for orthotopic heart transplantation are mandatory when complex congenital anomalies are associated in adult patients. An unusual case of a heterotaxy syndrome and dilated cardiomyopathy following mitral ring annuloplasty is presented in a 62-year-old male. Orthotopic cardiac transplantation was performed by using a modified operative strategy: selective peripheral and central venous cannulation according to the thoraco-abdominal venous challenges, biatrial technique, and preservation of venous drainage via the native coronary sinus. We discuss the anatomical features of heterotaxy in adult patients and surgical approaches when heart transplantation is needed.

Surgical Management of Vascular Stents in Pediatric Cardiac Surgery: Clues for a Staged Partnership.

Complex cases undergo step surgical and percutaneous procedures, including stent deployment. Concerns arise on stent removal at latest surgery. Our initial experience is presented. Forty-six stents in 35 patients were partially or totally removed at surgery. Univentricular heart was diagnosed in 20 patients. Stents were previously deployed in: ductus (6), right ventricle outflow tract (12), atrial septal defect (4), right pulmonary artery (4), left pulmonary artery (16), inferior vena cava (2), superior vena cava (1) and ascending aorta (1). Surgical procedures performed: 9 transplants, 6 Fontan, 4 Glenn, 1 comprehensive repair (Norwood + Glenn), 1 Glenn takedown, 8 conduit replacement, 2 Fallot, 2 Rastelli, 1 ventricular septal defect closure and 1 iatrogenic aortopulmonary window. Five ductal stents were clipped. Eleven stents in right ventricle, four ones in atrial septal defect, two in right pulmonary artery, seven in the left pulmonary artery and two in inferior vena cava were completely removed. Two stents in right pulmonary artery, one in superior vena cava, one in ascending aorta and nine in the left pulmonary artery were partially retrieved. Handling the stents in ductus, right ventricle and atrial septal defect was straightforward. On the contrary, stent removal in the ductus (comprehensive case), pulmonary branches, both vena cavae or aorta required short periods of deep hypothermia with circulatory arrest. Surgery over stents is increasing in complex, step procedures. Univentricular hearts are most prevalent. Congenital transplant surgery faces new challenges. Stent removal at the time of surgery may require deep hypothermic circulatory arrest.

Truncus arteriosus, tricuspid atresia and partial anomalous pulmonary venous drainage: a unique form of univentricular heart.

We present the first reported case of truncus arteriosus, tricuspid atresia and partial anomalous pulmonary venous drainage into a retro-aortic innominate vein in a neonate in whom successful palliation was performed using a two-staged procedure. An early repair after presentation along with a re-routing of anomalous pulmonary venous drainage at the time of the second stage should be undertaken to optimize the patient's haemodynamic status prior to a Fontan completion.

Modified Fontan Conversion: An Alternative Technique for Adult Patients.

A need persists for Fontan conversion that provides alternative approaches for the individual anatomical challenges occurring in these unusual and complex adult patients. The pulmonary arteries present unique variations and the surgical technique needs to be intraoperatively addressed. We describe a technique for Fontan conversion for performing the distal anastomosis of the extracardiac conduit, allowing adequate matching to the pulmonary arteries and preserving an optimal flow into the Fontan circuit.

Atresia valvular aórtica o hipoplasia grave y comunicación interventricular: estrategias de corrección biventricular y resultados a medio plazo / Aortic valve atresia or severe hypoplasia and ventricular septal defect: surgical strategies for biventricular repair and mid-term results

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