Idiopathic dilatation of the right atrium: a not so benign entity.

Idiopathic dilatation of the right atrium is an isolated enlargement of the right atrium in the absence of other cardiac lesions. This rare anomaly has a clinical spectrum ranging from asymptomatic to heart failure or even sudden death. It can be associated with atrial arrhythmias and thrombus formation. Antiplatelet therapy is prescribed in most cases reported in the literature, and reduction plasty is indicated when there is rapid growth of the right atrium, compression of adjacent structures, or refractory arrhythmias. We report four cases of idiopathic dilatation of the right atrium diagnosed during prenatal screening. We describe the intrauterine course and management in postnatal life until early childhood.

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.

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.

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)

[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.

Successful neonatal double switch in symptomatic patients with congenitally corrected transposition of the great arteries.

Anatomic repair is the standard surgical approach to congenitally corrected transposition of the great arteries. However, timing to perform the procedure remains controversial. We present 2 cases of congenitally corrected transposition of the great arteries and Ebstein's-like anomaly of the tricuspid valve presenting with heart failure. Both cases had successful anatomic repair during the neonatal period.

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.

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

Biventricular repair of complete atrioventricular canal, double-outlet right ventricle and common atrium using a modified double switch technique. A valid alternative to univentricular procedure.

An eleven month-old child underwent a successful modified double switch operation for total correction of complete atrioventricular canal, double outlet right ventricle, noncommitted ventricular septal defect, pulmonary stenosis, common atrium and anomalous left superior vena cava to an unroofed coronary sinus. We describe the technique of modified double switch operation, utilizing an atrial switch combined with a Rastelli type reconstruction between the venous ventricle and the pulmonary artery.

[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.

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

Presentamos nuestra experiencia en el cierre percutáneo de la comunicación interventricular (CIV) con dispositivo Amplatzer muscular en 4 pacientes. El primer caso se trataba del cierre de una CIV apical amplia, adicional a la CIV subarterial de un tronco arterioso tipo I, con el objetivo de evitar la ventriculotomía apical en la corrección quirúrgica completa posterior. En el segundo caso, la CIV estaba asociada a una transposición de grandes arterias congénitamente corregida con dextrocardia, situs inversus y bloqueo auriculoventricular, portador de marcapasos desde el nacimiento. La CIV se cerró con el fin de descargar al ventrículo derecho (sistémico). En el tercer caso, la CIV era posquirúrgica en un paciente con ventrículo derecho de doble salida en el que con anterioridad se habían realizado 3 intervenciones quirúrgicas. El último paciente tenía una CIV muscular aislada. El cierre fue completo en todos los casos. No hubo complicaciones. El cierre percutáneo de la CIV puede hacerse como procedimiento de elección primaria, previo a la cirugía o como complemento de ésta (AU)

[Analysis of factors that can influence the appearance of acute heart transplant failure]. / Análisis de los factores que pueden influir en la aparición del fallo agudo del corazón trasplantado.

INTRODUCTION AND OBJECTIVE: Acute graft failure (AGF) is defined as significant failure of myocardial function in a newly implanted heart. The aim of the present study was to investigate a series of factors related to heart transplantation (HT) in relation to AGF. MATERIAL AND METHOD: In a study of 287 consecutive HTs performed over a 14-year period, AGF was defined when: a) the surgeon observed ventricular dysfunction before closing the sternotomy; b) various inotropic drugs were required at high doses in the first days after surgery, or c) ventricular dysfunction was identified by routine echocardiography in the immediate postoperative period. Statistical analysis comprised a descriptive and univariate comparative study, followed by multivariate analysis based on application of a logistical regression model. RESULTS: The incidence of AGF was 22%. Predictors of AGF were female donor status (OR = 2.2; 95% CI, 1.2-4.4; p = 0.02), a disproportion of more than 20% in donor-recipient body weight (OR = 2.2; 95% CI, 1.1-4.3; p = 0.02), and background ischemic heart disease (OR = 2.5; 95% CI, 5.5-1.1; p = 0.03) or valve pathology (OR = 5.0; 95% CI, 7.0-1.5; p = 0.01). CONCLUSIONS: AGF is a frequent pathology, which was present in 22% of our heart transplantation patients. Among the modifiable factors related to AGF was a clear disproportion in body weight and the size of grafts from female donors. Unmodifiable factors related to AGF were ischemic heart disease and valvular heart disease as a cause of heart transplantation.