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OBJECTIVES Energy demand and supply need to be balanced to preserve myocardial function during paediatric cardiac surgery. After a latent aerobic period, cardiac cells try to maintain energy production by anaerobic metabolism and by extracting oxygen from the given cardioplegic solution. Myocardial oxygen consumption (MVO2) changes gradually during the administration of cardioplegia. METHODS MVO2 was measured during cardioplegic perfusion in patients younger than 6 months of age (group N: neonates; group I: infants), with a body weight less than 10 kg. Histidine-tryptophan-ketoglutarate crystalloid solution was used for myocardial protection and was administered during a 5-min interval. To measure pO2 values during cardioplegic arrest, a sample of the cardioplegic fluid was taken from the inflow line before infusion. Three fluid samples were taken from the coronary venous effluent 1, 3 and 5 min after the onset of cardioplegia administration. MVO2 was calculated using the Fick principle. RESULTS The mean age of group N was 0.2 ± 0.09 versus 4.5 ± 1.1 months in group I. The mean weight was 3.1 ± 0.2 versus 5.7 ± 1.6 kg, respectively. MVO2 decreased similarly in both groups (min 1: 0.16 ± 0.07 vs 0.36 ± 0.1 ml/min; min 3: 0.08 ± 0.04 vs 0.17 ± 0.09 ml/min; min 5: 0.05 ± 0.04 vs 0.07 ± 0.05 ml/min). CONCLUSIONS We studied MVO2 alterations after aortic cross-clamping and during delivery of cardioplegia in neonates and infants undergoing cardiac surgery. Extended cardioplegic perfusion significantly reduces energy turnover in hearts because the balance procedures are both volume- and above all time-dependent. A reduction in MVO2 indicates the necessity of a prolonged cardioplegic perfusion time to achieve optimized myocardial protection.

Highlights • Cardiac rhabdomyomas may alter intracardiac electrical conduction. • Rhabdomyoma arrhythmias are caused by isolated atrial depolarization disturbances. • Destruction of conduction system has been described in diffuse rhabdomyomatosis., Introduction Rhabdomyomas are the most frequent cardiac tumors in children. Furthermore, they are often associated to tuberous sclerosis complex, an autosomal dominant neurocutaneous disorder characterized by tumor-like malformations that involve many organ systems. Presentation of the case We describe a rare ECG pattern in a severe case of neonatal tuberous sclerosis complex. Discussion In the presence of significant rhabdomyomatosis related to tuberous sclerosis, multiple clusters of rhabdomyoma-like cells can infiltrate the myocardium, with increased fibrosis areas. Conclusion Considering the fact that rhabdomyomas often show spontaneous regression, close follow-up is sufficient in hemodynamically stable cases. Destruction of the conduction system, with arrhythmias as consequence, can be the presenting feature of diffuse rhabdomyomatosis.

OBJECTIVES: We describe the way we treated 7 children with critical long-term complications after metallic balloon-expandable stenting in the left mainstem bronchus. METHODS: Endoscopic follow-up included a first bronchoscopy 3 weeks after stenting, then monthly for 3 months, every 4-6 months up to 1 year and at scheduled times to calibrate stent diameter up to final calibration. When major complications occurred, patients underwent chest computed tomographic angiography. RESULTS: In 1 of the 7 children (median age 2.8 years), metallic left bronchial stenting served as a bridge to surgery. After a median 4-year follow-up, all 7 children experienced recurrent stent ovalizations with stent breakage in 3 and erosion in 1. In 4 children, computed tomographic angiography showed abundant peribronchial fibrous tissue, in 2 left mediastinal rotation and in 1 displacement along the left bronchus after pulmonary re-expansion as the cause of stent-related complication. Of the 7 children, 6 underwent surgery (5 posterior aortopexy and 1 section of the ligamentum arteriosus) and 3 required nitinol stents placement within the metallic ones. One patient completed the follow-up, and 1 patient was lost to follow-up. All 5 remaining children still have permanent bronchial stents in place, patent and re-epithelialized after a median 10.5-year follow-up. There were no deaths. CONCLUSIONS: Satisfactory anatomical relationships when children have stents placed in the left mainstem bronchus alone do not guarantee the final success. Several mechanisms intervene to cause critical stent-related complications in children during growth. Permanent metallic stents should be used carefully, and only in selected patients.

Highlights • In the foetus a tumour can be noted on a routine antenatal anomaly scan as an intracardiac mass. • In postnatal life cardiac tumours may affect the integrity and function of the adjacent cardiac structures leading to severely compromised blood flow. • Because of the potential life-threatening sequelae of cardiac myxoma, treatment consists of urgent surgical resection., Introduction Cardiac myxoma is a benign neoplasm representing the most common primary cardiac tumor in adults, however it is unusual in neonates. It is represented by an endocardial mass that occupies the cardiac chamber. Although the majority of myxomas are attached to the fossa ovalis of the interatrial septum, they also attach to the walls of the cardiac chambers and to valve leaflets surfaces. Approximately 75% of myxomas are found in the left atrium, 20% are located in the right atrium, and rarely in the ventricles. Presentation of case We describe a rare case of neonatal cardiac myxoma arising from interatrial septum, causing significant mechanical obstruction to blood flow through tricuspid valve, in an otherwise normal newborn. The patient underwent successful excision of the myxoma with an uneventful recovery [1]. Discussion Cardiac tumors are rare in children, thus an understanding of the common types of benign and malignant paediatric cardiac tumors and their imaging features, is important because the epidemiology and tumor types differ from those encountered in adults. Conclusion Large neonatal myxoma is exceptionally rare and even more infrequent is the surgical excision in the first day of life.

Adrenocortical carcinoma is a rare malignancy. Due to late diagnosis and no adequate effective adjuvant treatment, prognosis remains poor. Only approximately 30% of these malignancies are confined to the adrenal gland when they are diagnosed, as these tumors tend to be found years after their genesis. Cardiac involvement of adrenal carcinoma is very rare. We report a rare case of a 7-year-old female with right adrenal cortical carcinoma, involving the right-side heart.

OBJECTIVES The aim of this multicentric study was to outline surgical indications and evaluate mid-term outcomes of porcine extracellular matrix (ECM) in surgery for congenital heart disease (CHD). METHODS The use of ECM was categorized into four major groups: A, valve repair; B, septal reconstruction; C, arterial plasty; D, other use. Primary endpoints of analysis were reintervention (either surgical or interventional) when related to ECM, and functional ECM failure. Secondary endpoints were evidence of calcification and of persistent inflammation at follow-up. RESULTS One hundred and three patients (M/F = 61/42, median age 19.7 months, 1 day-62 years) underwent surgical repair for CHD. Among ECM use categories, 38 patients were in Group A, 16 in Group B, 71 in Group C and 7 in Group D. There were neither complications nor deaths related to ECM. At a median follow-up of 23.3 months (0.3–55.2), 19 patients underwent reoperation (ECM-related in 6); 11 patients underwent interventional cardiology procedures (ECM-related in 8). Reinterventions were significantly more frequent on the aortic valve (surgical, P = 0.0056) and pulmonary arteries (interventional, P = 0.0159). In addition, interventional procedures on pulmonary arteries were significantly more frequent in infants

Background. The increasing use of off-pump bypass grafting (OPCABG), requires an evaluation of its effects on left ventricular (LV) performance. Methods. In 8 patients with multivessel coronary disease who were undergoing to off-pump coronary artery bypass grafting, LV performance was analyzed from the pressure-volume (P-V) plane by the conductance catheter technique. Measurements were performed at base line, after the exposure of the vessels, after the application of the stabilization system, and at the end of the procedure. Results. No significant changes in heart rate, LV end-systolic volume, LV end-diastolic pressure, mean pulmonary artery, and mean systemic blood pressure were observed in the various stages of the procedure. Cardiac index decreased during left anterior descending coronary artery grafting after application of the stabilizer with a concomitant decrease in LV end-diastolic volume, together with decreases in LV peak negative -dP/dt and increases in τ, indicating an impairment of LV relaxation but without a change in preload recruitable stroke work, indicating preserved LV contractile state. Exposure of posterior and lateral vessels induced a decrease in cardiac index and preload recruitable stroke work without a decrease in LV preload, indicating a decrease in LV contractile state together with a decrease in peak -dP/dt and increase in τ, indicating an impairment in LV relaxation. Conclusions. Off-pump coronary artery bypass grafting can be performed without decreasing LV performance. Major cardiac displacement like that used for posterior and lateral exposure induces acutely significant decrease in LV contractile state. © 2002 by The Society of Thoracic Surgeons.

Congenital heart diseases are corrected early in life, so the surgical procedure has to maintain the maximum potential for growth. The quest for the ideal material for cardiac tissue repair is still ongoing. Here, we describe our preliminary experience with an extracellular matrix for cardiac and vascular tissue repair. Between August 2009 and April 2011, 26 patients underwent cardiac surgery using the CorMatrix patch for vascular repair (10 pulmonary artery, four ascending aorta, three aortic arch and one right ventricular outflow tract) or for valve reconstruction (five aortic, two tricuspid, one mitral and one pulmonary valve); in four cases, the repair was associated with pericardial closure using the same patch. There were no deaths, and at a mean follow-up of 13.2months, there was no evidence of patch-related complications either in the vascular position or when used for valve repair. The ideal material for cardiac tissue repair should be haemostatic and resistant to tearing and calcification, and possibly one that will not induce an inflammatory cascade. In the last 25months, we used an extracellular matrix for cardiac and vascular tissue repair. The patch is advocated to induce the patient's own tissue regeneration. The early results are encouraging, but a longer follow-up is needed to understand the real potential of this material. © 2011 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.

Severe pericardial effusion is a rare complication of bacterial pneumonia and it usually disappears under medical treatment. Herein we report a case of a girl with a congenital immunodeficient syndrome and bacterial pneumonia, who developed recurrent and life-threatening pericardial effusion refractory to medical treatment. She was finally treated with pericardiectomy. Copyright © 2010 Andrea Quarti et al.

Many surgical techniques have been described either to repair and to replace the aortic valve. Among the paediatric population the potential for growth has to be preserved and valve reconstruction is therefore of great importance. In the last two years 25 consecutive patients, mean age 8.6 years, with aortic valve disease, underwent aortic valve repair for aortic regurgitation (AR) (10 patients), aortic valve stenosis (5 patients) or mixed lesion (10 patients). None of the patients died neither during hospitalization nor at follow-up (median 9.25 months). In the aortic stenosis (AS) group, one patient required reoperation (re-repair). None of the patients in the AR group developed more than mild AS and mild AR during follow-up. Nine out of 10 patients of the mixed lesion group had no or trivial AR at the follow-up. Left ventricular dimension decreased in all patients after repair. With a better understanding of the causes of AS or AR and the adoption of different techniques, often used in multiple association, we believe that aortic valve repair can be achieved in most patients with a normal left ventricular outflow tract. © 2010 Published by European Association for Cardio-Thoracic Surgery.

A case of anomalous origin of the left coronary artery from the pulmonary artery in a patient with the origin of the coronary opposite to the aorta is reported. Between many surgical options we conclude to reestablish a double coronary system reconnecting the coronary through a conduit created with a pulmonary wall baffle and an autologous pericardial patch.

Here we present our simplified technique of cannulation of the brachiocephalic trunk for cardiopulmonary bypass and antegrade cerebral perfusion institution.

Echinococcosis is a parasitic disease that usually involves lungs and liver. Occasionally, it localizes in the heart (less than 2% of cases). We present a case of an adult patient with cardiac echinococcosis complicated by ventricular tachycardia. The diagnosis, based on transthoracic two-dimensional echocardiography, magnetic resonance imaging (MRI), and computerized tomography (CT), was confirmed by surgery.

Cardiac perforation after transcatheter closure of a patent foramen ovale is a very rare complication. We report a case of left atrial wall perforation and aortic root erosion occurring 16 months after implantation of an Amplatzer patent foramen ovale occluder that required emergent surgery. © 2006 The Society of Thoracic Surgeons.

Plastic bronchitis is a rare complication of a variety of respiratory diseases and congenital heart disease surgery, particularly Fontan procedure. Bronchial casts with rubber-like consistency develop acutely and may cause severe life-threatening respiratory distress. The management of plastic bronchitis is yet not well defined. Early intermittent, self-administered nebulization of tissue plasminogen activator was found to be effective in preventing deterioration of acute respiratory symptoms in a patient with primary ciliary dyskinesia and recurrent cast formation. Further investigation into new therapeutic strategies for this devastating disease is advocated. © 2014 Colaneri et al.; licensee BioMed Central Ltd.