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Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries is a rare congenital heart lesion in which pulmonary blood supply may arise from different segments of the aorta. We report an unusual case of a newborn with a major collateral artery originating from the proximal ascending aorta. Successful reparative surgery was undertaken.

Double outlet right atrium (DORA) is a rare congenital heart disease in which the right atrium opens into both ventricles. The reduced leftward motion of the interventricular septum causes a malalignment between the atrial and the ventricular septum at the cardiac crux, which is the pathognomonic feature of this heart defect. We describe a case of significant exertional desaturation in an adult patient who was diagnosed with DORA, restrictive right ventricle, and anomalous tricuspid valve. Subsequently, the patient underwent one-and-a-half ventricular palliation.

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.

We report a case of a newborn, affected by tuberous sclerosis complex, with a prenatally diagnosed giant cardiac rhabdomyoma associated with a large renal angiomyolipoma presenting as a duct-depending lesion not treatable by surgery. After receiving everolimus, a mammalian target of rapamycin inhibitor, we observed a rapid, significant, and durable reduction of both lesions without remarkable side effects.

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.

In congenital non-Ebstein anomalies of the tricuspid valve, the septal leaflet is often involved and tethered. We describe a standardized approach to address septal leaflet tethering by concomitant augmentation of the anterior and posterior leaflets.

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

In congenital non-Ebstein anomalies of the tricuspid valve, the septal leaflet is often involved and tethered. We describe a standardized approach to address septal leaflet tethering by concomitant augmentation of the anterior and posterior leaflets.

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.

The optimal management of patients with tetralogy of Fallot has to consider the individual intra-cardiac anatomy as the most important variable, together with the age and the body weight of the patient. In any case the potential advantages of a primary early repair should be weighted against the experience and expertise of the individual centre and/or surgical team in dealing with tetralogy of Fallot and with neonates and infants. The best results are achieved by very carefully adapting the surgical technique to the individual morphology of the right ventricular outflow tract and of the pulmonary arteries. The details of the established surgical management for each component of the surgical repair are analysed and described. Over a period of 12years (from 1993 to 2005) 318 consecutive patients with tetralogy of Fallot underwent repair with one hospital death (1/318=0.3% mortality).

Firstly described by W. Ebstein in 1866, the Ebstein anomaly (EA) is composed of a wide spectrum of malformations, so that every valve taken into consideration is a unique valve. EA is rare, accounting for approximately 1-5 cases every 200,000 live births and representing less than 1 % of all CHD, but is the most frequent congenital lesion of the tricuspid valve (TV). Natural history of patients with EA is poor. Early presentation in life is associated with high percentage of mortality, while patients who survived the neonatal period have a limited life expectancy. Deaths usually occur for arrhythmic events and heart failure. From the embryological point of view, the anomaly is related to a failure of delamination of the TV leaflets which reflects with a characteristic pattern of morphological anomalies involving the TV and the right ventricle (RV). This cohort of abnormalities could be associated with rhythm disturbances and other cardiac defects. The disease may have a clinical neonatal presentation which corresponds to a severe RV and TV dysfunction or may present later in life, concurrently with an increasing degree of TV regurgitation and RV dilatation. Since the pioneer period of cardiac surgery, many techniques have been described to address the TV and RV anomalies including TV replacement which is still an option, despite reconstructive techniques being now preferred due to the improvement of the early and long-term results. The aim of the repairing techniques is to restore a normal tricuspid valve function, to preserve the right ventricular contractility, and to decrease the risk of rhythm disturbances. Results are associated with the severity of the disease, the surgeon experience, and the perioperative care of the patient.

No abstract available

Objective: Augmentation of pulmonary blood flow is the only surgical treatment to reduce symptoms of cyanosis in patients with cavopulmonary connection unsuitable for Fontan operation. Brachial arteriovenous fistula is a good option to increase pulmonary blood flow. In this report, we analyze its possible consequences on ventricular function. Methods: Six patients considered unsuitable for a Fontan completion because of poor ventricular function underwent brachial arteriovenous fistula. The fistula was performed with the interposition of a 4-mm gore-tex conduit between the brachial vein and the brachial artery. Results: The mean oxygen saturation increased from 76.8 ± 1.9% to 84.1 ± 1.4% (p < 0.01); hemoglobin and hematocrit decreased from 21.13 ± 0.7 g/dL to 18.12 ± 0.5 g/dL (p < 0.01) and from 63.3 ± 2.7% to 54.2 ± 0.7% (p < 0.01), respectively. Mean ejection fraction and shortening fraction increased from 38.6 ± 1.5% to 49.5 ± 2.3% (p < 0.01) and from 23.3 ± 1.8% to 28.8 ± 1.3% (p < 0.01), respectively. A Fontan completion and takedown of the fistula was then accomplished, within six years, with no mortality or major complications. At a mean follow-up of 15 months, all six patients are clinically in good condition. Conclusion: In patients with a cavopulmonary connection and poor ventricular function, the brachial arteriovenous fistula may play a role in increasing oxygen saturation, optimizing ventricular preload, and reducing blood viscosity and pulmonary arteriovenous malformations. This approach might represent an intermediate step to rescue patients previously considered unsuitable or at high risk for Fontan operation. © 2011 Wiley Periodicals, Inc.

The modified Blalock-Taussig shunt is a surgical option in cyanotic patients. In our Institute heparin infusion therapy in the early postoperative period is used to reduce the risk of shunt thrombosis. This may produce haemorrhagic complications. Herein we describe the effect of a multidisciplinary audit to reduce the risk of haemorrhagic complications. Between January 2005 and December 2009, 49 patients received a modified Blalock-Taussig shunt and anticoagulation therapy until the second administration of an antiplatelet drug. Four patients (8.1%) experienced a haemorrhagic event. A multidisciplinary audit was organized to analyze our anticoagulation protocol. The cohort of patients was divided into group 1 and 2: patients without and with haemorrhagic events respectively. Group 2 was characterized by a low level of anticoagulation in the first postoperative day and received a dose of antithrombin III and an increase in heparin infusion. The result was excessive anticoagulation, evidenced by a significant increase in the aPTT from 44 to 138 sec (in Group 1: from 88 to 54 sec). Retrospectively the nursing staff found that these patients presented clinical signs heralding more significant bleeding. The clinical audit depicted a difference between the two groups. Group 2 was initially characterized by heparin resistance and was consequently treated. The nursing staff found that retrospectively there were clinical signs heralding bleeding and created a risk score. Finally the analysis of this data produced a change in the institutional anticoagulation protocol and created a medical and nursing combined protocol for postoperative anticoagulation screening. Since then, the haemorrhagic complications have been reduced significantly.

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.

Proponents of a telemetrically adjustable pulmonary artery band (PAB) device have cited simplified postoperative management and shortened length of stay as advantages associated with that technology. This report concerns a recent experience with both conventional pulmonary artery banding (conv-PAB) and the telemetrically adjustable PAB FloWatch (FW-PAB). From January 2005 through December 2008, 19 consecutive infants underwent either conv-PAB (8 patients, mean age 3.5 months, mean weight 4.1 kg) or FW-PAB (11 patients, mean age 2.6 months, mean weight 3.1 kg). Indications for PAB were left ventricular retraining (1 patient in FW-PAB), palliation prior to biventricular repair (7 patients in conv-PAB and 10 in FW-PAB group), and staged univentricular repair (1 patient in conv-PAB). In-hospital mortality was 0%. In the FW-PAB group, 1 FloWatch device was removed because of hemodynamic compromise related to the bulk of the device. There were no major complications in the conv-PAB group and no differences between groups with respect to postoperative ventilation time or length of stay in the intensive care unit or in hospital. In the FW-PAB group, a mean of 3.1 ± 1.7 regulations per patient were undertaken. Of the regulations, 85% (29/34) were adjustments to tighten the device, and 15% (5/34) were to loosen it. During follow-up, 8 patients underwent intracardiac repair and pulmonary artery debanding: 4 in the conv-PAB group and 4 in the FW-PAB group. The course of patients in both groups after PAB were similar. Major differences in length of stay and resource utilization were not apparent. © 2010, SAGE Publications. All rights reserved.

Objective: Mitral valve prolapse is a rare condition and represents a challenge for cardiac surgeons. Many techniques have been described to treat the wide spectrum of abnormalities causing mitral valve regurgitation but the low incidence of these abnormalities is the cause of the relatively poor experience of many institutions. Methods: From April 2005 to September 2006, five patients underwent isolated mitral valve repair with the edge-to-edge technique for anterior or bileaflet prolapse. Three patients also had a Gore-Tex strip to reinforce the posterior annulus, whereas two had a 26-size soft incomplete ring annuloplasty. Results: There was no death and none of the patients required reoperation. None of the patients had mitral regurgitation more than 1+ at a mean follow-up of 30 months. None of the patients had mitral stenosis or any valve-related event. Conclusion: The edge-to-edge technique is a well tolerated and useful technique for the anterior or bileaflet prolapse of the mitral valve. Although many techniques have been described with good results, in many anatomical settings and in small patients, edge-to-edge is a good option and might not interfere with the growth of the valve. Early results are encouraging but a longer follow-up is needed to evaluate the long-term prognosis. © 2009 Italian Federation of Cardiology.

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.

The association of a bicuspid aortic valve (BAV) with abnormalities of the proximal thoracic aorta, including dilatation, aneurysm and dissection, has been previously described, leading to the hypothesis of a common underlying developmental defect involving the aortic valve and the aortic wall. Consequently, any patient with BAV should receive a careful assessment not only of the valve function, but also of the aortic root and the ascending aorta. Dilatation of the proximal thoracic aorta is a common finding in patients with BAV and is believed to be related to aortic rupture and dissection. Because progressive dilatation can occur, careful long-term surveillance of the aortic dimensions is required. Prophylactic surgical repair of the dilated aorta should be recommended more aggressively for patients with BAV than for those with a tricuspid aortic valve. However, the optimal timing of aortic surgery in BAV patients remains uncertain because of the limited data available on the natural history of asymptomatic aortic dilatation. © 2006 Italian Federation of Cardiology.

A persistent left superior vena cava draining into the left atrium may produce a symptomatic right-to-left shunt. Although intra-atrial rerouting techniques, in patients with no connecting vein, have proved to be reliable and successful, in many cases the extracardiac repair is preferable. We report a case of a 5-month-old patient with a not connected left superior vena cava draining into the left atrium, associated with atrial septal defect and partial anomalous pulmonary venous connection. The correction has been achieved by rerouting the pulmonary venous return into the left atrium and by transposition of the left vena cava on the right appendage.

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

The development of minimally invasive cardiac surgery has shown good clinical results with shorter recovery time and better cosmetic results. The introduction of the robotic systems can further reduce the surgical trauma and improve the surgical dexterity. We report seven cases of complete closed chest atrial septal defect closure using the "da Vinci" Surgical System (Intuitive Surgical, Mountain View, CA).Following peripheral cannulation for cardiopulmonary bypass (CPB), aortic occlusion and cardioplegia delivery, five patients with atrial septal defect (ASD) and two patients with patent forame ovale (PFO) with atrial septal aneurysm (ASA) were successfully treated using the robotic system. Two robotic arms and an endoscopic camera were inserted through ports in the right hemithorax and an accessory port was placed for blood suction and ancillary instruments insertion. The defect closure was carried out with interrupted stitches in one patient and with a continuous suture in the others.Mean cardiopulmonary bypass and cross clamp time were 101.8 +/- 39.6 and 63.4 +/- 21.9 minutes respectively. Extubation was carried out within the seventh postoperative hour. All patients returned to normal lifestyle in one week.Complete closed chest ASD closure can be carried out using robotic technique with rapid postoperative recovery and excellent cosmetic result.

The case of a 2-year-old infant admitted to our Institution with a diagnosis of severe aortic valve stenosis is presented. After a balloon valvuloplasty with no results in terms of gradient reduction, an echocardiogram showed a moderate mitral regurgitation. The patient underwent surgical repair of both the aortic and mitral valves. Inspection of the mitral valve showed a 5-mm hole in the posterior leaflet at the P2 scallop. Probably, the dilation tore a secondary cord, pulling away a piece of the leaflet. A quadrangular resection was performed with good results. © Springer Science+Business Media, LLC 2011.

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.