Surgical closure of multiple large apical ventricular septal defects: how we do it.

The management of apical ventricular septal defects continues to be challenging because of the difficulty in achieving a complete closure without a left apical ventriculotomy. In this study, we present our innovative technique of closing multiple and/or large muscular apical ventricular septal defects through a right atriotomy. We operated three patients with multiple apical muscular trabecular ventricular septal defects ("Swiss cheese") using a technique that involved exclusion of the right ventricular apex. Their ages ranged between 2 months and 13 years. The VSDs were approached through right atriotomy. The trans right atrial approach using a 5-0 polypropylene purse-string suture or a two-patch procedure is a novel method of closing large apical ventricular septal defects. It was found to be effective with no persistent residual defects and did not have the disadvantages of a ventriculotomy.

The Nikaidoh procedure for complex transposition of the great arteries: short-term follow-up.

BACKGROUND: The surgical treatment for complex forms of d-transposition of the great arteries associated with ventricular septal defect and left ventricular outflow tract obstruction remains controversial. In this study, we describe the classical surgical options - namely, the Rastelli procedure and the "réparation à l'étage ventriculaire" - and present our experience with the modified Nikaidoh procedure with early and short-term follow-up results. METHODS: Between 2007 and 2014, four patients with d-transposition of the great arteries associated with ventricular septal defect and left ventricular outflow tract obstruction underwent surgical repair at our institution by means of a modified Nikaidoh procedure. RESULTS: With a mean follow-up of 4.5 years, survival was 100%, and none of the patients required re-intervention or mechanical circulatory support. There was no recurrence of left ventricular outflow tract obstruction and no aortic valve regurgitation classified as more than mild. Left ventricular function was preserved. CONCLUSIONS: Aortic translocation with the modified Nikaidoh procedure is a safe and effective surgical treatment for certain complex forms of transposition of the great arteries, particularly those associated with ventricular septal defect and left ventricular outflow tract obstruction. It is associated with less need for re-intervention and better morbidity and mortality results in the short- and mid-term follow-up, when compared with the classical alternatives such as the Rastelli procedure.

Pulmonary valve replacement for treatment of severe pulmonary regurgitation in patients with previous tetralogy of Fallot repair surgery.

Tetralogy of Fallot was classically described as the combination of pulmonary stenosis, right ventricle hypertrophy, perimembranous ventricular septal defect and an aortic root that overrides the ventricular septal defect. Full surgical repair, which is usually indicated in toddlers, comprises closure of the ventricular septal defect and alleviation of the pulmonary stenosis. Because there is a wide anatomical spectrum, the cause and severity of the pulmonary stenosis are variable. However, when the pulmonary valve is stenotic and not well-developed, it has to be removed, and one has to accept severe pulmonary regurgitation as a sequela. In the ensuing years, when signs of damage to the right ventricle are proven, pulmonary valve replacement is indicated. We present the case of a 16-year-old patient with tetralogy of Fallot that was corrected during the first year of life. Follow-up demonstrated progressive exercise intolerance and moderate-to-severe right ventricle dilatation. Thus, surgical pulmonary valve replacement was indicated. The surgical approach was through a full median redo sternotomy. Central cardiopulmonary bypass was established with bicaval drainage. The operation was performed under assisted non-clamped circulatory support. The calcified previous pericardial patch was removed, and a 25-mm bioprosthetic valve was implanted in the pulmonary annulus. A new pericardial heterologous patch was used for the transannular pulmonary plasty.

Defibrillation threshold decrease with the supradiaphragmatic extracardiac implantable cardioverter-defibrillator implantation technique.

Despite advances in implantable cardioverter-defibrillator (ICD) technology, the optimal ICD implantation technique for pediatric patients has not yet been established. One increasingly used option is totally extracardiac implantation. However, concern exists about the high defibrillation threshold (DFT) at the moment of implantation or during follow-up. We report the case of a 3-year-old boy with repetitive syncopal idiopathic ventricular tachycardia episodes treated with ICD implantation using the extracardiac technique. Changing device position from abdominal to a supradiaphragmatic, solved unsafe elevated discharge impedance and DFT during follow-up.

A Rare Case of Cardiogenic Shock: Cor Triatriatum With Anomalous Pulmonary Venous Return.

Obstructive total anomalous pulmonary venous return (TAPVR) is a potentially life-threatening clinical condition and a surgical emergency. Left-sided partial anomalous pulmonary venous return (PAPVR) in association with a subdivided left atrium, also known as cor triatriatum sinister (CTS), is a very rare malformation that can mimic obstructive TAPVR. We present a case of a newborn with clinical manifestation of cardiogenic shock caused by CTS and PAPVR.

Patent Ductus Arteriosus Banding for Circular Shunting After Pulmonary Valvuloplasty.

We report two cases of newborns with critical pulmonary stenosis having intact ventricular septum, who underwent pulmonary valve balloon valvuloplasty followed by banding of a patent ductus arteriosus. Transcatheter pulmonary valvuloplasty was performed one week after delivery. Following the procedure, both developed "circular shunting" as a consequence of left-to-right ductal flow and pulmonary regurgitation. This in turn caused increased blood flow into a dysfunctional right ventricle and low systemic cardiac output syndrome. The PDA banding was performed urgently as a rescue measure in order to restore systemic flow while still maintaining some duct-dependent pulmonary blood flow. This approach resolved the circular shunting. Outcome was favorable in both the patients.

[Aortic origin of the left pulmonary artery in an infant with Fallot's tetralogy]. / Origen aórtico de arteria pulmonar izquierda en neonato con tetralogía de Fallot.

We report the case of a male neonate who had a prenatal diagnosis of Fallot's tetralogy. He presented with respiratory distress during the second week of life. Chest x-ray showed an enlarged right ventricle and pulmonary edema. Echocardiography demonstrated characteristic features of Fallot's tetralogy. However, cardiac catheterization disclosed that, in this case of Fallot's tetralogy, the left pulmonary artery had an anomalous origin in the ascending aorta. At 23 days of age, the patient underwent total surgical correction of the defects, during which a direct anastomosis was employed without conduit placement. 10 days later, he was discharged. We present the results of a literature review of the epidemiological, physiopathological, clinical, diagnostic, and surgical characteristics of this rare condition.

[Percutaneous closure of ventricular septal defect with an Amplatzer device]. / Cierre percutáneo de la comunicación interventricular con dispositivo Amplatzer.

We present a series of 4 patients in whom a ventricular septal defect (VSD) was closed with an Amplatzer muscular VSD device during cardiac catheterization. In one patient with type I truncus arteriosus and subarterial VSD, closure of a wide apical defect was done to allow further surgical correction while avoiding left ventricular ventriculotomy. The second patient had congenitally corrected transposition of the great arteries with complete A-V block and a pacemaker implanted from birth. Our intention was unload the systemic anatomical right ventricle. The third patient had had 3 heart surgeries to correct a double outlet right ventricle. She had a residual ventricular septal defect which was large enough to cause heart failure. The last patient had an isolated muscular ventricular septal defect. Percutaneous closure was successful in all patients, and there were no complications. Percutaneous closure of the defect may be used as a primary procedure, before surgery, or as a complementary procedure after surgery.

Neonatal Ross-Konno operation and endocardial fibroelastosis resection after foetal percutaneous aortic valve balloon valvuloplasty: a complex approach to rescue the left heart.

We report a case of a patient who presented with aortic stenosis and a borderline left ventricle during foetal life. A balloon aortic valve valvuloplasty was performed in uterus, and in the postnatal period for relief of the left ventricular outflow tract obstruction followed by a Ross-Konno procedure with fibroelastosis resection. These successful interventions allowed left ventricular growth and the conversion to a biventricular circulation after a single-stage surgery.

Palliative switch: a surgical decision after hybrid procedure.

The first-stage palliation of newborns with single-ventricle anatomy and transposed great arteries can be very challenging when associated with systemic ventricular outflow obstruction and aortic arch obstruction. Often, the initial intervention is a stage I Norwood procedure. We present the case of a newborn with double inlet left ventricle, discordant ventriculoarterial connection with restrictive ventricular foramen, and severe aortic arch obstruction. A hybrid procedure was performed initially as a means of addressing hemodynamic instability. Three months later, a palliative arterial switch procedure was performed as an alternative to the combined Norwood-Glenn procedure. Palliative arterial switch combined with arch reconstruction can be an effective surgical option in these complex, challenging patients.

[Surgical treatment of anomalous left pulmonary artery]. / Tratamiento quirúrgico del origen anómalo de la arteria pulmonar izquierda.

We report our experience with the surgical treatment of anomalous origin of the left pulmonary artery in eight children between 2004 and 2009. The congenital heart disease most frequently associated with this condition was patent ductus arteriosus. Surgery was carried out with extracorporeal circulation in five children, and without, in three. The anomalous pulmonary artery was divided and translocated to the main pulmonary artery. One patient died soon after surgery because of hemodynamic instability and another died later because of respiratory complications. The other patients progressed satisfactorily during follow-up: the reimplanted artery remained patent in all cases and respiratory symptoms improved. However, one patient required endoscopic treatment.

Treatment of complex coarctation of aorta with hypoplastic transverse aortic arch using left carotid artery flap.

Coarctation of aorta associated with severe hypoplastic aortic arch and ductus arteriosus dependent, often combined with complex cardiac malformations, should be looked upon as bordering of hypoplastic left heart syndrome. The crucial and first objective is the adequate reconstruction of aortic arch, continuing with the repair of cardiac malformations. The surgical treatment making resection of the coarctation segment combined with left carotid flap plasty is a surgical alternative, useful in patients with this complex anatomic variant.

Device migration in hybrid technique for apical muscular ventricular septal defects closure.

Muscular ventricular septal defects still require complex surgical procedures for their repair. We have used a hybrid approach for closure of these ventricular septal defects in patients needing open-heart surgery. It consists of the deployment of a ventricular septal occluder, as used in transcatheter procedures inside the defect under direct vision after cardiopulmonary bypass establishment. Through this paper, we report a case to illustrate a new and simple technique to avoid one of the most dramatic complications after this procedure: the migration of the closure device.

Tratamiento quirúrgico del origen anómalo de la arteria pulmonar izquierda / Surgical treatment of anomalous left pulmonary artery

Presentamos nuestra experiencia en el tratamiento quirúrgico del origen anómalo de la arteria pulmonar izquierda en 8 niños, intervenidos entre 2004 y 2009. La cardiopatía asociada más frecuentemente fue la persistencia del conducto arterioso. A 5 se realizó cirugía con circulación extracorpórea y a 3, sin ella. Se realizó división de la arteria pulmonar anómala y translocación al tronco de la arteria pulmonar. Hubo un fallecimiento de forma temprana por inestabilidad hemodinámica y una muerte más tardía por complicaciones respiratorias. La evolución de los pacientes en seguimiento ha sido satisfactoria con permeabilidad de la arteria reimplantada en todos los casos y mejoría de la clínica respiratoria; uno de ellos precisó de técnicas endoscópicas (AU)

We report our experience with the surgical treatment of anomalous origin of the left pulmonary artery in eight children between 2004 and 2009. The congenital heart disease most frequently associated with this condition was patent ductus arteriosus. Surgery was carried out with extracorporeal circulation in five children, and without, in three. The anomalous pulmonary artery was divided and translocated to the main pulmonary artery. One patient died soon after surgery because of hemodynamic instability and another died later because of respiratory complications. The other patients progressed satisfactorily during follow-up: the reimplanted artery remained patent in all cases and respiratory symptoms improved. However, one patient required endoscopic treatment (AU)

Sling de la arteria pulmonar / Pulmonary artery sling

Introducción. El nacimiento anómalo de la arteria pulmonarizquierda (sling de la arteria pulmonar) es una malformacióncardiaca en la que la arteria pulmonar izquierdanace de la derecha y se sitúa entre tráquea y esófago. Provocasíntomas respiratorios como consecuencia de la compresióndel bronquio principal derecho y la tráquea. Caso clínico. Lactante de 7 meses de edad con episodios de dificultad respiratoria desde los 3 meses de vida catalogados de bronquitis. Diagnosticado de sling de la arteria pulmonar, se realizó cirugía correctora con desinserción de la API y anastomosis directa al lado izquierdo del tronco de la arteria pulmonar. Comentarios. El origen anómalo de la arteria pulmonar izquierda o sling de la arteria pulmonar es un raro anillo vascular que requiere un alto índice de sospecha en lactantes con sintomatología respiratoria recurrente. El diagnóstico precoz de esta enfermedad es crucial, porque puede conducir a un severo compromiso respiratorio que puede amenazar la vida e incluso producir la muerte antes del año de edad. Una adecuada evaluación de las anomalías asociadas del árbol traqueo-bronquial es importante para decidir la cirugía y mejorar el pronóstico. Revisamos la literatura de esta rara entidad, en sus aspectos epidemiológicos, fisiopatológicos, clínicos, diagnósticos y quirúrgicos

Introduction. The anomalous left pulmonary artery(pulmonary artery sling) is a congenital condition in whichthe left pulmonary artery arises from the right pulmonaryartery forming a sling between the trachea and the esophagusand leading to respiratory symptoms by compressionof the right main stem bronchus and trachea.Case Report. A 7 month old infant presented with respiratorydistress since 3 months of age, for which he hadbeen diagnosed with bronchitis. The diagnosis of pulmonaryartery sling was made and he underwent a surgicalrepair with detachment of the aberrant left pulmonary arteryand reimplantation to the left side of the main pulmonaryartery.Comments. The anomalous origin of the left pulmonaryartery known as pulmonary artery sling is a rare formof vascular ring that requires a high index of suspicion ininfants with recurrent respiratory symptomatology. Theearly recognition of this condition is crucial because thesepatients may experience life-threatening respiratory compromiseand early death. An adequate assessment of theanomalies of the tracheobronchial tree is very importantin order to improve surgical planning and prognosis. Theauthors review the literature and discuss the epidemiology,pathophysiology, clinical presentation and diagnosis,and surgical aspects of this anomaly

Origen aórtico de arteria pulmonar izquierda en neonato con tetralogía de Fallot / Aortic origin of the left pulmonary artery in an infant with Fallot's tetralogy

Presentamos el caso de un neonato con diagnóstico prenatal de tetralogía de Fallot. Durante la segunda semana de vida presentó dificultad respiratoria; en la radiografía de tórax se observó un aumento del ventrículo derecho con edema pulmonar. La ecocardiografía mostró los hallazgos de la tetralogía de Fallot. En el cateterismo cardíaco se encontró además un origen anómalo de la arteria pulmonar izquierda desde la aorta ascendente. A los 23 días se realizó cirugía correctora con anastomosis directa sin utilizar interposición de conducto. Fue dado de alta hospitalaria 10 días después. Revisamos la literatura médica de esta rara entidad, en sus hallazgos epidemiológicos, fisiopatológicos, clínicos, diagnósticos y quirúrgicos (AU)

We report the case of a male neonate who had a prenatal diagnosis of Fallot's tetralogy. He presented with respiratory distress during the second week of life. Chest x-ray showed an enlarged right ventricle and pulmonary edema. Echocardiography demonstrated characteristic features of Fallot's tetralogy. However, cardiac catheterization disclosed that, in this case of Fallot's tetralogy, the left pulmonary artery had an anomalous origin in the ascending aorta. At 23 days of age, the patient underwent total surgical correction of the defects, during which a direct anastomosis was employed without conduit placement. 10 days later, he was discharged. We present the results of a literature review of the epidemiological, physiopathological, clinical, diagnostic, and surgical characteristics of this rare condition (AU)

Uso de oxigenación por membrana extracorpórea (ECMO) en un caso de shock séptico neonatal por neumococo / Use of extracorporeal membrane oxygenation in a case of pneumococcal neonatal septic

Introducción. La sepsis neonatal por Streptococcus pneumoniae es una causa muy infrecuente de enfermedad invasiva en el recién nacido, conlleva una elevada mortalidad y en ocasiones puede beneficiarse del tratamiento con oxigenación por membrana extracorpórea (ECMO). Observación clínica. Neonato a término de sexo masculino, fruto de una cesárea urgente por sospecha de pérdida del bienestar fetal que presenta, a las pocas horas de vida, fracaso respiratorio y hemodinámico progresivo que no responde al tratamiento convencional intensivo. En el hemocultivo crece Streptococcus pneumoniae. Requiere tratamiento con oxigenación por membrana extracorpórea (ECMO) venoarterial durante 8 días, presentando una evolución favorable y consiguiendo la supervivencia libre de secuelas hasta la actualidad. Comentarios. La ECMO está indicada en aquellos pacientes que tienen un alto riesgo de muerte por fracaso respiratorio o cardiorespiratorio que no responde al tratamiento médico exhaustivo. Ha demostrado una disminución de la mortalidad frente al tratamiento médico convencional sin incrementar las secuelas en los supervivientes. El tratamiento adecuado de la coagulopatía del shock antes de iniciar la ECMO y el manejo minucioso de la heparinización una vez ésta es aplicada permite el tratamiento de los neonatos sépticos con éxito. Es fundamental el contacto precoz con el centro de referencia para valorar la indicación de la técnica y el momento oportuno para la realización del traslado (AU)

ECMO pediàtric. A propòsit de dos casos / ECMO pediátrico. A propósito de dos casos / Pediatric ECMO: report of two cases

Introducció. Les tècniques de suport vital extracorpori (ECMO) s’han demostrat altament eficaces per al tractament de la insuficiència respiratòria o cardiorespiratòria, del nadó, sense resposta al tractament convencional. Sense aquesta tècnica, la mortalitat supera el 80% dels casos. Des de l’any 1984, s’han registrat a l’ELSO (Extracorporeal Life Support Organization) un total de 14.700 casos neonatals, amb una supervivència del 80%. A l’edat pediàtrica, l’experiència és molt inferior, amb un total de 1.723 casos registrats i una supervivència global del 50%. Observació clínica. Al nostre hospital, hem tingut l’oportunitat de tractar dues malaltes en edat pediàtrica: 1r cas, nena de 3 anys, afecta de sèpsia per pneumococ, amb afectació pulmonar bilateral, i insuficiència cardiorespiratòria refractària al tractament convencional; 2n cas, nena d’1 any d’edat, afecta d’insuficiència respiratòria severa en el postoperatori immediat, de la reparació completa d’una Tetralogia de Fallot. En tots dos casos es van aplicar tècniques de suport venoarterial (ECMO V-A), durant 10 i 4 dies respectivament, i en un d’ells es van reconstruir els vasos, en el moment de la decanulació. Les dos malaltes van ser donades d’alta hospitalària, en bon estat i sense seqüeles. Es tracta dels primers casos en edat pediàtrica tractats amb èxit a Espanya. Comentaris. El tractament amb ECMO pot ser una tècnica de suport eficaç en el maneig de la insuficiència cardiorespiratòria refractària al tractament convencional, millorant la supervivència d’aquests malalts crítics (AU)

Introducción. Las técnicas de soporte vital extracorpóreo (ECMO), para el manejo del fallo respiratorio o cardiorrespiratorio del recién nacido, sin respuesta al tratamiento convencional, han demostrado su elevada eficacia. La mortalidad de estos pacientes sin ECMO es superior al 80% de los casos. Desde 1984, se han recogido un total de 14.700 casos registrados en la ELSO (Extracorporeal Life Support Organization), con una supervivencia global del 80%. La experiencia clínica en la edad pediátrica es mucho menor, con 1.723 casos registrados y una supervivencia global del 50%. Observación clínica. En nuestro hospital, hemos tratado dos pacientes en edad pediátrica: 1 caso, niña de 3 años, afecta de una sepsis por neumococo, con afectación pulmonar severa y fallo cardiorrespiratorio refractario; 2º caso niña de 1 año que presenta insuficiencia respiratoria grave en el postoperatorio inmediato de la reparación completa de una Tetralogía de Fallot. En ambos casos se aplicaron técnicas de soporte veno-arterial (ECMO V-A) y en uno de ellos se procedió a la reconstrucción vascular en el momento de la decanulación. Ambas pacientes fueron dadas de alta hospitalaria, en buen estado y sin secuelas. Se trata de los primeros pacientes, en edad pediátrica, tratados con éxito con esta técnica, en España. Comentarios. El tratamiento con ECMO puede ser un soporte eficaz pàra el fallo cardíaorrespiratorio refractario al tratamiento convencional, aumentando la supervivencia de estos pacientes críticos (AU)

Introduction. The techniques of extracorporeal life oxygenation (ECMO) have shown their efficacy in the acute management of neonatal respiratory or cardio-respiratory distress syndrome unresponsive to conventional therapy. Mortality in this group of patients without the use of ECMO is greater than 80%. Since 1984, 14,700 neonatal cases have been registered at the Extracorporeal Life Support Organization (ELSO), with an overall survival of 80%. The pediatric experience is much less extensive, with 1,723 recorded cases, and an overall survival of 50%. Clinical observation. We have treated two pediatric patients with ECMO. The first case was a 3 year-old girl with pneumococal sepsis and acute respiratory distress syndrome, which was unresponsive to conventional therapy; the second case was a 1 year-old girl who developed an acute and refractory respiratory failure in the immediate postoperative course of a complete repair of Tetralogy of Fallot. In both patients, the severity of the respiratory failure warranted veno-arterial ECMO, with good response. They were weaned at 10 and 4 days, respectively, with vascular repair at the time of decanulation in the second case. Both patients recovered completely and were eventually discharged without complications. Comments. ECMO therapy can be an effective support for the management of postoperative cardiac and respiratory failure refractory to medical management (AU)