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Introduction: Pulmonary atresia with intact ventricular septum and critical pulmonary stenosis in newborns encompasses a wide spectrum of disease, including cases with significant right ventricular hypoplasia and coronary artery to right ventricle fistulae, which may be considered a contraindication for decompression of the right ventricle. The aim of this study was to review the middle- and long-term outcomes of these patients over 20 years and identify differential factors between both groups, including patients with coronary artery fistulae., Patients and Methods: We performed a descriptive retrospective study by identifying all patients that received a diagnosis of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis between January 1996 and January 2018. We collected and analysed data regarding right ventricular morphology, surgical management, percutaneous intervention and medium- and long-term outcomes., Results: 51 patients were admitted. A total of 9 patients (17.6%) died during the followup. None of the deceased patients had coronary artery to right ventricle fistulae. The median length of follow up in the 42 survivors was 8.9 years (1-16). The functional class based on the latest revision of the New York Heart Association classification was 1.2 for the overall sample. Survivors of critical pulmonary stenosis had a functional class of 1.1, and survivors of pulmonary atresia with intact ventricular septum a functional class of 1.6. There were no differences based on the presence or absence of coronary artery to right ventricle fistulae., Conclusions: Coronary artery to right ventricle fistulae may not be a contraindication for biventricular strategy. Patients with critical pulmonary stenosis had better outcomes compared to patients with pulmonary atresia with intact ventricular septum. The aggressive strategy of opening the pulmonary valve early on was associated with a good overall survival and correlated to a good functional class., (Copyright © 2018. Publicado por Elsevier España, S.L.U.)

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.

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., (© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Introduction and Objectives: Transposition of the great arteries is a prevalent congenital heart defect with a high survival rate and a good long-term outcome, especially if managed with early surgical intervention during the neonatal period. In this study, our main objective was to describe patient characteristics and outcomes and to identify possible predictors of early and long-term morbidity and mortality., Methods: Retrospective analysis through review of clinical and surgical charts of patients with transposition of the great arteries admitted to the service of neonatology during 2000-2011., Results: The study included 136 patients; 119 of them had undergone corrective surgery during the neonatal period. Patients were divided into 3 groups: group I, 81 cases of isolated transposition; group II, 24 cases with ventricular septal defect; and group III, 31 with "complex" transposition of the great arteries. The overall postoperative survival was 96.7% (115 of 119 patients); no patients from group I died after surgery. Duration of surgery, intubation, inotropic treatment, and length of stay were higher in patients in groups II and III. The overall survival rate after an average of 6 years of follow-up was 90.4% 123 of 136 patients, with no deaths after discharge in group I. The most frequent residual defect during cardiac follow-up was supravalvular pulmonary stenosis, in 33 of 113 patients that had follow-up data., Conclusions: In our study, the survival rate was high in patients with transposition of great arteries and especially in those undergoing arterial switch. The number of subsequent residual heart defects was low., (Copyright © 2013 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.)

Cardiac tumours are rare, especially in children, and most of them are benign. Myxomas are unusual in children, being more common among adults. They are usually located in the left atrium, with 25% appearing in the right. The clinical signs and symptoms depend mainly on where the tumour is located. A feature of these tumours is that they can be accompanied by constitutional symptoms and laboratory abnormalities. Echocardiography is the study of choice, and a prompt resection is required to prevent serious complications. We present a case of a 10 year-old girl diagnosed with right atrial myxoma who presented with a fever, myalgia, asthenia and laboratory abnormalities. Diagnosis was made by echocardiography, and the early surgical resection of the tumour ran smoothly and showed a good postoperative recovery., (Copyright © 2012 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.)

We report the first case published in Spain of a palliative anatomic correction associated with aortic arch repair in a neonate with (S,D,L)-transposition of the great arteries, multiple ventricular septal defects, and severe hypoplasia of the right ventricle with subaortic obstruction and hypoplasic aortic arch with coarctation. A one stage palliative surgery on cardiopulmonary bypass was performed with reconstruction of the aortic arch and an arterial switch procedure which obtained a satisfactory result. The principle of this operation is to switch the subaortic obstruction into a subpulmonary obstruction and reconstruct a large natural aortic root from the principal ventricle. The right ventricle-pulmonary artery continuity may promote growth of the right ventricle with the possibility of a future biventricular repair. We conclude that this operation, when used by surgical teams experienced with arterial switch surgery, is the best treatment for the complex newborn group with single ventricles or severe ventricular disbalance, ventriculoarterial discordance and stablished subaortic stenosis.

Introduction and Objectives: Anatomic correction of transposition of the great arteries in simple and complex form is theoretically the only procedure which offers the possibility of real cure to the problem. We report there our operative results and mid-term follow-up with this approach., Methods: From June 1989 to February 1994, anatomical surgical correction was performed in 41 patients in the neonatal period, 31 with simple TGA and 9 patients with transposition associated with ventricular septal defect. We report our preoperative management, anatomic findings and surgical technique., Results: Our initial experience with the arterial repair was associated with a high operative mortality. The actuarial survival rate was 74% at 56 months. Hospital mortality of the last 31 patients was 12.9%. Intermediate-term follow-up was 18 months. No late deaths. One patient underwent reoperation for supravalvular pulmonary stenosis. All survivors are in functional class I. The only significant risk factor of in-hospital mortality was the time of surgery (learning curve)., Conclusion: The initial experience with arterial repair is associated with a difficult learning curve. Primary repair must be performed during the newborn period in simple and complex transpositions. Mid-term results are very encouraging. Longer follow-up is necessary to secure this technique and to render obsolete all other types of repair.

Introduction: It is unclear after review the literature, whether the techniques of retrograde cardioplegia provides more optimal myocardial protection in the setting of coronary surgery that is possible with conventional antegrade cardioplegia, due to inhomogeneous patient subset that appears in clinical trials., Patients and Methods: The present clinical study, prospective and randomized was undertaken in 40 patients undergoing high risk myocardial revascularization to compare the efficacy of both techniques. Inclusion criterias were: age > 65 years, unstable angor, multivessel disease and impaired ventricular function. One control group received cardioplegia through the aortic root, and the study group received combined antegrade-retrograde cardioplegia with perfusion into the right atrium. In both groups the vehicle of cardioplegia was oxygenated blood., Results: There were 3 deaths, two in the control group and another one in the study group. Overall operative mortality was 7.3%. Left main coronary stenosis was present in the 2 patients dying in the control group. There were no significant differences between the two groups with regard to the operative and postoperative parameters analyzed; trials to wean off cardiopulmonary bypass, inotropic support, intra-aortic balloon counterpulsation, perioperative myocardial infarction, and levels of myocardial enzymes. There were higher incidence of temporal heart block in patients in study group., Conclusions: It was difficult to identify any appreciable difference in the clinical efficacy of these two methods of myocardial protection. We review the literature, trying to identify the "better" alternative in the setting of coronary revascularization and different patient subset.

Anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital malformation associated with a high mortality during the first year of life. Up to 1990, 75 cases have been reported in the literature. We are reporting 2 cases diagnosed and operated before 3 months of age. Repair was facilitated by the use of deep hypothermic circulatory arrest. The duration of follow up is 5 and 6 years, respectively. Pre and postoperative cardiac catheterization pressure data and angiograms are shown. We review the literature, presenting the physiopathological, embryological and surgical features of this rare congenital anomaly.

We reviewed our experience with replacement of the ascending aorta and aortic valve with a composite graft and reimplantation of coronary arteries to the tube graft during 8 years interval from April, 1982, to April 1990. 24 patients underwent repair, the mean age was 49.83 years. Annuloaortic ectasia was the most common indication (58.33%), followed by aortic dissection (acute or chronic). Emergency operation was carried out in nine patients with aortic dissection (37.5%) and elective in 15. The mean duration of cardiopulmonary bypass was 118 +/- 4 minutes and of aortic clamping 83.85 +/- 2 minutes. Hospital mortality was 4.17%, reoperation for hemorrhage was 12.5% and perioperative morbidity for other causes was 34.7%. There were one late death. 20 patients were follow-up with a total of 638 patients-months (two patients excluded with insufficient follow-up and one late death). At last follow-up 14 patients were in functional class I. Eight year actuarial survival for the 24 patients was 91%. We believe that replacement of the ascending aorta and aortic valve with a composite graft and coronary arteries reimplantation to the tube graft is more than one satisfactory alternative to supracoronary graft replacement and aortic valve replacement. It offers the advantage of excluding all abnormal aortic tissue, eliminating the risk for later development of complications in the non excluded disease aorta. It supposes the method of choice for patients with anuloaortic-ectasia, aneurysms of the sinuses of Valsalva with aortic insufficiency, and aortic dissection with proximal affectation of coronary arteries and aortic valve.

We made a retrospective analysis of 43 cases of primary tumor of the small intestine, 28 benign and 15 malignant, diagnosed and treated in our hospital over a period of 18 years. A preoperative diagnosis was reached in 13 cases (30%), while in 37.2% the tumor was a casual finding during operation by other methods. The remaining 32.5% debuted as acute abdomen and were an emergency surgical indication. Simple tumoral resection was practiced in 19 cases (44.1%) of benign tumor, intestinal resection with end-to-end anastomosis in 22 cases (51%) and only biopsy in two cases (4.6%). Coadjuvant chemotherapy was given to five patients (11.5%) and radiotherapy to one (2.3%). The one-year survival for malignant tumors was 73%, and only 18% (2 cases) survived more than 5 years after the operation).