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The cervical aortic arch is a rare congenital vascular abnormality related to the anomalous development of the aortic arch. We present the case of a 6-year-old patient with a large aneurysmal cervical aortic arch who underwent surgical correction and arch reconstruction. Surgical repair was indicated based on the risk of progressive dilation and rupture, aiming to restore correct geometry and hemodynamics. We evaluated preoperative and postoperative hemodynamics using computational fluid dynamics simulations, and we also identified, within the repaired region, an area that remains affected by greater turbulent flow, requiring follow-up surveillance.

Cartridges used for US service rifles from the Spanish-American War to those used in Operation Desert Storm are presented. These are the .30-40 Krag, the .30 for the 1903 Springfield rifle, the .30-'06, the .30 carbine, the 7.62 NATO and the 5.56 mm used since the Vietnam War.

Coronary artery anomalies are a rare form of congenital heart disease.

Irbesartan, a long acting selective angiotensin-1 receptor inhibitor, in Marfan syndrome might reduce aortic dilatation, which is associated with dissection and rupture. We aimed to determine the effects of irbesartan on the rate of aortic dilatation in children and adults with Marfan syndrome.

Congenital sinus of Valsalva aneurysm is a rare disorder, mostly involving the right and noncoronary sinuses, in which intracardiac rupture is more likely foreseen into the right chambers. Because of the unfavorable prognosis, which includes heart failure and sudden death, detection itself is an indication for treatment, which may be performed either surgically or percutaneously. We present a case of a four-year-old patient with aorto-right atrial tunnel, in which a transcatheter attempt of closure was performed, complicated by new onset of aortic valve regurgitation, requiring surgical intervention.

In spite of the magnificent improvement in Fontan operation results in the last two decades, there are still some concerns regarding the unfavourable early outcomes that may follow Fontan completion.

AbstractBackgroundIn 2007, a partnership was initiated between a small-volume paediatric cardiac surgery unit located in Las Palmas de Gran Canaria, Spain, and a large-volume cardiac surgery unit located in Milan, Italy. The main goal of this partnership was to provide surgical treatment to children with CHD in the Canary Islands.MethodsAn operative algorithm for performing surgery in elective, urgent, and emergency cases was adopted by the this joint programme. Demographic and in-hospital variables were collected from the medical records of all the patients who had undergone surgical intervention for CHD from January, 2009 to March, 2013. Data were introduced into the congenital database of the European Congenital Heart Surgeons Association Congenital Database and the database was interrogated.ResultsIn total, 65 surgical mission trips were performed during the period of this study. The European Congenital Heart Surgeons Association Congenital Database documented 214 total patients with a mean age at operation of 36.45 months, 316 procedures in total with 198 cardiopulmonary bypass cases, 46 non-cardiopulmonary bypass cases, 26 cardiovascular cases without cardiopulmonary bypass, 22 miscellaneous other types of cases, 16 interventional cardiology cases, six thoracic cases, one non-cardiac, non-thoracic procedure on a cardiac patient with cardiac anaesthesia, and one extracorporeal membrane oxygenation case. The 30-day mortality was 6.07% (13 patients).ConclusionsA joint programme between a small-volume centre and a large-volume centre may represent a valid and reproducible model for safe paediatric cardiac surgery in the context of a peripheral region.

Introduction:Anomalous Aortic Origin of Coronary Artery (AAOCA) with intramural (IM) segment is associated with risk of sudden cardiac death (SCD). The pathogenic mechanism is believed to be the IM compression caused by the aorta expansion during effort. However, the IM compression occurrence and magnitude during the cardiac cycle remain unknown.Hypothesis:We hypothesized that the IM segment is subjected to dynamic compression during different phases of cardiac cycle with a luminal area and roundness smaller in systole than in end-diastole.Methods:Lumen dynamics was derived from IVUS pullbacks of N=35 AAOCA (N=23 with IM tract) after retrospective image-based gating to identify cardiac cycles. The cyclic change of area (A) and roundness (R=minimum/maximum lumen axis) of ostial, distal IM, and extramural (EM) tracts were described after manual lumen segmentation. The systolic phase was identified, in each patient, by estimating normal PQ and QT intervals from image-based gating. Results are presented with median and interquartile range. One-tailed Wilcoxon signed-rank test (p<0.025) assessed differences between systolic and diastolic phases in each tract.Results:A systolic reduction of roundness was observed in ostium [Rsystole= 0.5 (0.2), Rdiastole= 0.6 (0.3), flattening: -7% (10), p=0.01] and distal IM segment [Rsystole= 0.7 (0.2), Rdiastole= 0.8 (0.2), flattening: -6% (16), p=0.004]. No-IM segments showed normal behaviour and remained circular during the entire cardiac cycle. The distal IM had an area reduction during systole [Asystole= 8.3 (2.3) mm2, Adiastole= 8.1 (2.9) mm2, narrowing: -4% (11), p=0.009] that was not observed in all other segments (Figure 1).Conclusion:AAOCA with IM segment has pathological dynamic compression mainly in systole. Studying AAOCA behaviour with IVUS during the cardiac cycle may help to evaluate and quantify the severity of the narrowing.

AbstractIntroductionPatients with repaired tetralogy of Fallot have good long-term survival but less is known about the subjectively assessed quality of life or objectively measured functional status of those who have not required subsequent pulmonary valve replacement. We assessed these parameters in a group of children and adults free from pulmonary valve replacement after tetralogy of Fallot repair.Methods and resultsA random sample of 50 subjects – 16 children and 34 adults, aged 4.1–56.7 years – who had undergone tetralogy of Fallot repair and were free from subsequent pulmonary valve replacement underwent cardiopulmonary exercise testing and completed standardised questionnaires assessing health-related quality of life and resilience. Patients were generally asymptomatic (median New York Heart Association class = I). Exercise capacity was within two standard deviations of normal for most children and adults (mean z VO2max: 0.20 ± 1.5; mean z VE/VCO2: −0.9 ± 1.3). Children reported a total health-related quality of life score similar to healthy norms (78 ± 10 versus 84 ± 1, p = 0.73). Adult survivors also reported quality of life scores comparable to healthy norms. Resilience was highly correlated with all domains of health-related quality of life (r = 0.713, p < 0.0001).ConclusionsPatients who have undergone tetralogy of Fallot repair in childhood and have not required pulmonary valve replacement have a good long-term health-related quality of life. The finding that patients with greater resilience had better health-related quality of life suggests that it may be beneficial to implement interventions to foster resilience.

Maternal antenatal therapy with glucocorticoids (GC) is routinely used to prevent lung immaturity. The potential harmful effects on other organs, including in particular the central nervous system (CNS), are still controversial. In the present review we aimed to investigate: i) the beneficial and detrimental effects of antenatal GC treatment in both human and animal models; ii) the potential usefulness of biochemical markers such as calcium binding proteins (S100B, synaptophysin) and cytoskeletal protein of neurons and dendrites (MAP2) in the perinatal period, and iii) whether the assessment of brain markers in different biological fluids could constitute a promising tool for the monitoring of CNS function and/or developmental in fetuses and newborns whose mothers assumed GC antenatally.

Aims We aimed to assess the impact of surgical pulmonary valve replacement (PVR) for severe pulmonary regurgitation (PR) on biventricular function and its effect on exercise capacity. Methods and results Seventy-three patients (mean age 23.6 ± 11.5 years, 47 females) underwent surgical PVR for PR. Echocardiogram and magnetic resonance imaging to assess ventricular size and function, and a cardiopulmonary exercise test were performed before, and 1-year post-surgery. Median New York Heart Association class improved from 2 to 1 but peak oxygen uptake (VO2) did not change. Left ventricular (LV) cardiac output increased from 3.2 ± 0.9 to 3.5 ± 0.7 L/min (P = 0.003). However, this was not associated with increased trans-mitral velocities (▵E = −0.13, P = 0.004; ▵A = 0.03, P = 0.395), or increased heart rate (−0.002%, P = 0.993). Trans-tricuspid rapid right ventricular (RV) filling increased significantly, whereas early diastolic myocardial velocity in RV wall decreased (E velocity: 0.57 ± 0.14–0.65 ± 0.21, P = 0.034; and E/e′ from 6.7 ± 1.9 to 14.8 ± 7.0, P < 0.0001). RV and LV late diastolic velocities and their ratio to early velocities (A, a′, E/A, and e′/a′) correlated with pre- and/or post-PVR peak VO2. No correlations were found between indexes of systolic function and peak VO2, either before or after surgery. Doppler evidence of restrictive RV physiology resolved after elimination of PR. Conclusion Surgical PVR for PR improves RV filling and increases left ventricular stroke volume, however, this could not be demonstrated by conventional Doppler echocardiography. Diastolic ventricular function was associated with exercise capacity. Because of its load dependency, E/e′ ratio failed in assessing diastolic function. Pre-systolic flow in pulmonary trunk in presence of severe PR does not determine intrinsic myocardial stiffness.

The extracardiac conduit type of total cavopulmonary connection (TCPC) is the most common variation of the modified Fontan operation in current use. For patients with some forms of complex anatomy (eg, dextrocardia in situs solitus or asplenia syndrome), we have adopted a different technique: interposition of an intra-atrial conduit between the inferior vena cava (IVC) and the superior vena cava–right pulmonary artery (SVC-RPA) connection. We report our experience with six patients.

Data comparing the effectiveness of high-frequency oscillatory ventilation and of conventional mechanical ventilation in the treatment of respiratory distress syndrome of very low birth weight infants are, to date, still matter of debate. We investigated the effects of first intention high-frequency oscillatory ventilation or conventional mechanical ventilation support on selected primary and secondary outcomes in very low birth weight infants complicated by respiratory distress syndrome in which antenatal glucocorticoid prophylaxis was not performed.

AbstractObjectivesClassical Atriopulmonary Fontan connections tend to fail in the long term due to progressive anastomotic site obstruction, right atrial enlargement, and refractory atrial arrhythmias. Conversion to total cavopulmonary connection with concomitant arrhythmia surgery is a promising treatment but optimal timing of the procedure remains controversial.MethodsBetween the years 2002 and 2009, 15 patients with a median age of 26.2 (12–43) years underwent Fontan conversion operation with concomitant arrhythmia surgery. All were symptomatic and 14 out of the 15 patients had refractory arrhythmias. The duration of pre-operative arrhythmia and the outcome of surgery were correlated to study the impact of delay in surgical intervention on post-operative survival and arrhythmia control.ResultsThere were two patients who died in the early post-operative period (13.3%). At the mid-term follow-up, 53 (20–86) months, late atrial arrhythmias had recurred in two of the 13 surviving patients (15.30%) and one patient developed late sinus node dysfunction. The need for anti-arrhythmic drugs decreased considerably from 93.5% to 15.3% on mid-term follow-up. There was no late death or need for cardiac transplantation. The duration of arrhythmia before surgery was prolonged for more than 10 years in patients who died as well as in those who had complications like late recurrence of arrhythmias, dependence on anti-arrhythmic medications, and worsening of ventricular dysfunction.ConclusionsFontan conversion is a well-established treatment option for salvaging the failing atriopulmonary connections. Concomitant arrhythmia surgery effectively resolves the refractory atrial arrhythmias and improves survival, but we need to optimise the timing of Fontan conversion to improve the long-term outcome.

Near Infrared Spectroscopy (NIRS) is an emerging technique for brain oxygenation monitoring in newborns complicated by acute and chronic hypoxia. However, data regarding cerebral oxygenation normal values are still lacking and matter of debate. Therefore, we investigate whether NIRS parameters in healthy preterm/term infants are gestational age and delivery modalities dependent and correlated with standard monitoring parameters. From January to December 2007, 100 healthy newborns with gestational age from 30 to 42 weeks gestation were evaluated. Routine laboratory variables, daily clinical and neurological evaluation and ultrasound imaging were performed. The regional cerebral oxygen saturation (rSO2) and fractional cerebral tissue oxygen extraction (FTOE) were measured by NIRS in the first 6- hours after birth. Data were recorded by MetaVision ICU X-Edition software and analyzed by SPSS statistical package. rSO2 and FTOE correlated (R-0.77; R0.41; P<0.01, for both) with gestational age. Highest rSO2 and the lowest FTOE peaks (P<0.001, for all) were found at 30-33 wks when compared with other monitoring periods. From 34 wks onwards, rSO2 progressively decreased and FTOE increased reaching their lower dip/peak (P<0.001, for all) at 38-39 weeks. rSO2 and FTOE values were significantly different (P<0.05, for both) between preterm and term newborns when corrected for delivery modality. rSO2 correlated (P<0.001 for all) with heart (r0.63), respiratory (r-0.58) rate, and with arterial oxygen saturation (r0.65). In conclusion, in the first 6-hours after birth cerebral oxygenation in healthy newborns is gestational age-dependent and correlated with routine parameters. NIRS reference curve could be particularly useful in sick newborns brain monitoring.

In preterm infants, neurological signs and clinical manifestations of brain damage are limited criteria for diagnosis of neurologic sequelae. Early indicators of brain damage are needed and currently some specific biochemical markers of brain injury are investigated to assess regional brain damage after perinatal asphyxia in neonates. In this study Protein S-100 (PS-100) and Neuron Specific Enolase (NSE) serum levels were studied serially during the perinatal period in preterm neonates with perinatal asphyxia as markers of glial and neuronal damage respectively. Thirty outborn preterm infants with perinatal asphyxia were studied at 3, 24, 48 hours and 7 days of life. According to Apgar scores at 1 and cord blood pH and lacticidemia (LA), patients were divided in two groups: 15 of them (GA 33±1.2 wk, BW 1790±383 g) with severe asphyxia (Apgar <4, pH7.0±0.08, LA 6.29±0.79 mM/L) and 15 (GA 32±1.8 wk, BW 1810±290 g) with mild asphyxia (Apgar between 4-6, pH 7.18±0.05, LA 2.59±0.61 mM/L). Ten gestational age matched healthy preterm neonates were studied as control group. Cerebral ultrasound examinations (7 MHz) were performed at birth and repeated at 3 weeks of life. The results of this study show that neonates with severe asphyxia at any time had significantly more elevated mean serum levels of both markers compared to the group with mild asphyxia and to the control group (p<0.05). The values of control group were also significantly lower in comparison with that of mild asphyxia. In neonates with severe asphyxia, NSE values decreased constantly from birth to the seventh day of life, while PS-100 showed a different pattern increasing progressively between 3 h and 7 days. In neonates with mild asphyxia serum values of both markers showed decreasing levels through the study period. The results of this study suggest that perinatal asphyxia is associated with the release of different brain cellular proteins in the blood of preterm infants with different time course indicating specific regional cellular injury. The more elevated levels of NSE at birth found in the newborns with severe asphyxia could be considered as an early biomarker of neuronal necrotic damage in the ischaemic phase of perinatal cerebral hypoxic-ischaemic insult; progressive increase of PS-100 during the first week of life in the same neonates could be expression of apoptotic damage of glial cells occurring in the reperfusion phase of cerebral ischaemia.

Hypoxia-ischemia constitutes a risk in infants by altering cerebral blood flow regulatory mechanisms and causing loss of cerebral vascular auto-regulation. Hypotension, cerebral ischemia, and reperfusion are the main events involved in vascular auto-regulation leading to cell death and tissue damage. Reperfusion could be critical since organ damage, particularly of the brain, may be amplified during this period. An exaggerated activation of vasoactive agents of calcium mediated effects could be responsible for reperfusion injury, which, in turns, leads to cerebral hemorrhage and damage. These dramatic phenomena represent a common repertoire in infants complicated by perinatal acute or chronic hypoxia or cardiovascular disorders treated by risky procedures such as open heart surgery and cardiopulmonary by-pass (CPB). To date, despite accurate perinatal and intra-operative monitoring, the post-insult period is crucial, since clinical symptoms and monitoring parameters may be of no avail and therapeutic window for pharmacological intervention (6-12 hours) may be limited, at a time when brain damage is already occurring. Therefore, the measurement of circulating biochemical markers of brain damage, such as vasoactive agents and nervous tissue peptides is eagerly awaited in clinical practice to detect high risk infants. The present review is aimed at investigating the role as circulating biochemical markers such as adrenomedullin, a vasoactive peptide; S100B, a calcium binding protein, activin A, a glycoprotein; neuronal specific enolase (NSE), a dimeric isoenzyme; glial fibrillary acid protein (GFAP), a astroglial protein, in the cascade of events leading to ischemia reperfusion injury in infants complicated by perinatal asphyxia or cardiovascular disorders requiring risky therapeutic strategies such as CPB and/or extracorporeal membrane oxygenation.

Global population aging and greater age-related incidence of ischemic, degenerative and calcific valve disease have led to an increasing number of very elderly patients being referred for valve surgery. However, their preoperative risk factors, and in-hospital and long-term outcomes have not been thoroughly investigated.

Objectives: To compare surgical as opposed to percutaneous interventional closure of isolated atrial septal defects in the oval fossa in terms of hospital stay, efficacy, and complications, and to study the respective role of the two techniques in current practice. Methods: Between January 1998 and April 2004, 126 out of 1210 patients treated at our institution for closure of an isolated defect in the oval fossa were aged less than 6 years. The mean age of these 126 patients at procedure was 4.2 plus or minus 1 year. The ratio of females to males was 74 to 52. Results: Of the patients, 62% were treated successfully using a percutaneous approach. The groups treated surgically or percutaneously did not differ for age, gender, or indications for treatment. No deaths occurred. The rates of total and major complications were higher in the group undergoing surgical closure, at 34% versus 9%, p less than 0.0001, and 10.5% versus 1%, p equal to 0.01, respectively. Embolisation of the device requiring subsequent surgery occurred in 1% of patients. The stay in hospital was shorter in those closed percutaneously, at 3.2 plus or minus 0.9 days versus 6.8 plus or minus 2.8 days, p equal to 0.0001. During a mean follow-up of 3.4 plus or minus 1.9 years, no major complications occurred in either group, and symptoms improved significantly in both groups. Additional sequels occurred in 2 patients who had major complications subsequent to surgical closure. Conclusions: Even in young children, it is both feasible and safe to close defects in the oval fossa percutaneously. Compared to surgical closure, the transcatheter approach allows a shorter stay in hospital, and has a lower rate of complications. Early and mid-term follow-up has confirmed the safety and efficacy of both techniques.

Ischemic functional mitral regurgitation following ischemic cardiomyopathy is a secondary phenomenon to ventricular dilation, and therapeutic approaches to this complication are not uniform. Solutions to improve mitral function include either mitral repair or observing the effects of coronary revascularization and/or ventricular rebuilding during surgical ventricular restoration (SVR). The present study of 108 patients (comprising 18% of our 588 SVR population) reports the effects of mitral repair following SVR and CABG by comparing geometric, functional, hemodynamic and outcome changes to SVR patients without mitral repair. The degree of mitral regurgitation went from 2.9 ± 1.2 before to 0.7 ± 0.7 after SVR and mitral repair. SVR improved EF from 29 ± 7% to 34 ± 10% p0.001; reduced end diastolic volume from 243 ± 74 to 163 ± 53 ml and end systolic volume from 170 ± 63 to 107 ± 41 ml, p0.000. Ventricular size and shape geometric measurements improved in all patients, either with and without mitral repair. SVR improved tenting and papillary muscle width between muscle heads in all patients, but alterations in mitral annular size improved only following mitral repair. Preoperative mitral regurgitation occurred in patients with larger ventricular volume and lower ejection fraction and was an independent predictor of operative mortality risk.

In this prospective cohort study we addressed the clinical impact of a reduced anticoagulation protocol on the hospital outcome of patients undergoing coronary revascularization with cardiopulmonary bypass.364 consecutive low to moderate risk patients scheduled for elective isolated coronary operations were admitted to the study. 184 patients (Control Group) received conventional open circuits and full systemic anticoagulation (target activated clotting time 480 seconds); 180 patients (Intraoperative ECMO group) received closed, phosphorylcholine coated circuits and a reduced systemic heparin dose (target activated clotting time 320 seconds).Patients of the Intraoperative ECMO group had less requirement for allogeneic blood products (odds ratio 0.55, 95% confidence interval 0.34–0.92, p= 0.02), a significant containment of blood loss (374 ± 278 mL vs. 463 ± 321 mL in Control group, p= 0.005) a lower postoperative peak serum creatinine levels (1.19 ± 0.48 mg/dL vs. 1.41 ± 0.94 mg/dL in Control group, p= 0.048), and a significant lower rate of severe morbidity (odds ratio 0.27, 95% confidence interval 0.09–0.81, p= 0.02). A reduction of systemic anticoagulation is feasible with a non-heparin-bonded, closed biocompatible circuit, and results in a significant improvement of the outcome of low to moderate risk coronary patients.

Objectives:Functional mitral regurgitation in ischemic cardiomyopathy carries a poor prognosis, and its surgical management remains problematic and controversial. The aim of this study was to report the results of our surgical approach to patients who have had myocardial infarctions and have ventricular dilatation, mitral regurgitation, reduced pump function, pulmonary hypertension and coronary artery disease. This surgical approach consists of endoventricular mitral repair without prosthetic ring, ventricular reconstruction with or without patch, and coronary artery bypass grafting. Patients:Forty-six patients (aged 64 ± 10 years) with previous anterior transmural myocardial infarction and mitral regurgitation comprised the study group. Indication for surgery was heart failure in 93% of cases; 25 patients were in New York Heart Association functional class IV and 17 were in class III. Mitral regurgitation was moderate to severe in 32 cases (69%). Results:All patients underwent coronary artery bypass grafting, with a mean of 3.2 ± 1.3 grafts. Associated aortic valve replacement was performed in 4 cases. Global operative mortality rate was 15.2%. End-diastolic and end-systolic volumes significantly decreased after surgery (from 140 ± 40 to 98 ± 36 mL/m2and from 98 ± 32 to 63 ± 22 mL/m2, respectively, P=.001). Systolic pulmonary pressure decreased significantly (from 55 ± 13 to 43 ± 16 mm Hg, P=.001). Ejection fraction did not change significantly. Postoperative mitral regurgitation was absent or minimal in 84% of cases; 1 patient had severe mitral regurgitation necessitating valve replacement. New York Heart Association functional class significantly improved. The mean preoperative functional class was 3.4 ± 0.6 (median 3, range 2-4); after the operation, this decreased to 1.9 ± 0.7 (median 2, range 1-3, P<.001). Cumulative survival at a 30-month follow-up was 63%. Conclusions:Our aggressive, combined surgical approach is aimed at correcting the three components of ischemic cardiomyopathy: relieving ischemia, reducing left ventricular wall tension by decreasing left ventricular volumes, and reducing volume overload and pulmonary hypertension by repairing the mitral valve. Despite a relatively high perioperative mortality rate, surviving patients benefitted from the operation, with improved clinical functional class and thus quality of life.

Objective:To investigate the perioperative changes of antithrombin III (AT-III) activity using reduced systemic heparinization and the possible role of AT-III in determining a better postoperative outcome. Design:Prospective randomized study. Setting:University hospital. Participants:Patients undergoing elective coronary revascularization with cardiopulmonary bypass (n = 90). Interventions:Of patients, 30 were treated with heparin-coated circuits and reduced systemic heparinization; 30, with heparin-coated circuits and full systemic heparinization; 30, with conventional circuits and full systemic heparinization. Measurements and Main Results:Heparin-coated circuits with full systemic heparinization did not exert any effect on coagulation parameters. Low systemic heparinization resulted in a significantly (p< 0.01) higher hematocrit value on arrival in the intensive care unit and in significantly higher values of AT-III activity during cardiopulmonary bypass (66 ± 12% v57.4 ± 13% and 59.1 ± 12% in the full systemic heparinization groups; p< 0.05), on arrival in the intensive care unit (69.7 ± 13% v60.7 ± 13% and 60.8 ± 11% in the full systemic heparinization groups; p< 0.01), and on the first postoperative day (81.3 ± 15% v67.4 ± 13% and 70.2 ± 12% in the full systemic heparinization groups; p< 0.01). No differences were observed in the clinical outcome. Conclusion:Reducing systemic heparinization determines an AT-III-saving effect that could be responsible for the decrease in thromboembolic complications already observed by other authors. It induces higher hematocrit levels immediately after the operation, probably reducing the unmeasurable intraoperative blood loss. Copyright 2002, Elsevier Science (USA). All rights reserved.

The objective of this study was to evaluate growth in infants with d-transposition of the great arteries (d-TGA) who underwent switch operation (anatomical correction) in the early neonatal period. Growth data (at birth and 1, 3, 6, 12, and 24 months of age) were retrieved in 23 infants with d-TGA who survived the operation without major complications. Measures were transformed into z scores and compared with normative data for age. Following surgery, infants showed low z scores for weight, height, and head circumference. Weight and height showed a trend to reach normal ranges between 6 and 12 months of age, and the z scores were positive at 24 months. z scores for head circumference were still negative at 24 months of age. No dietary interventions were implemented during this period, and the infants were fed their own mothers' milk or, before discharge, high-calorie, preterm formula. Following anatomical correction for d-TGA in the early neonatal period, infant growth shows a characteristic pattern, with parameters normalized by 12 months of age in most cases. Head growth shows a different pattern, although gross motor development seems unaffected.

We studied 316 patients undergoing cardiopulmonary bypass for coronary artery surgery in order to determine perioperative risk factors for postoperative renal dysfunctionA preliminary univariate analysis was performed by χ2analysis for categorical data and Mann-Whitney U-test for continuous variables to detect significant correlations between each risk factor and the occurrence of moderate or severe renal dysfunction. Subsequently, a multiple logistic regression was applied to the three risk factors identified as predictive for severe renal dysfunction. Low cardiac output syndrome and need for banked blood transfusions combined with a low haematocrit value during cardiopulmonary bypass increase the probability of severe renal dysfunction in the postoperative course.

Aortic outflow tract obstruction can complicate the clinical course and surgical management of patients with heterotaxy syndromes, but its anatomic basis has not been described in detail. In 20 postmortem cases with asplenia ( n = 4) or polysplenia ( n = 16), the anatomic causes of aortic outflow tract obstruction were absence of the subaortic conus in association with (1) narrowing of the subaortic outflow tract between the conal septum anteriorly and the common atrioventricular (AV) valve posteriorly in six (30%) patients; (2) aortic valvar atresia in four (25%), three with asplenia and one with polysplenia; (3) redundant AV valve leaflets in four (20%); (4) excessive AV valve fibrous tissue in four (20%); (5) marked hypoplasia of the mitral valve and left ventricle in two (10%); and (6) aneurysm of membranous septum in one (5%). One patient belonged to group (1) and (4). Aortic outflow tract obstruction was much more common with polysplenia (28%) than with asplenia (4%) (p < 0.001). (Am Heart J 1997;133:558-68.)

Objectives: To evaluate midterm results of mechanical valves in pulmonary position in patients with pulmonary regurgitation and right ventricular dysfunction as an alternative to bioprostheses. Patients: Mechanical valves (six tilting disc valves and two bileaflet valves) were implanted in eight patients previously operated on for tetralogy of Fallot (n = 7) and truncus arteriosus (n = 1), with severe right ventricular dysfunction caused by massive pulmonary regurgitation. Results: All patients survived prosthesis implantation and are currently well. At follow-up (3 months to 9 years), they do not show signs of valve failure, and right ventricular function has dramatically improved in all but one, who still shows moderate ventricular hypokinesia. Conclusion: After operative correction of congenital heart defects in selected patients who show severe dysfunction of the right ventricle caused by pulmonary regurgitation/stenosis, mechanical valves may represent an alternative to bioprosthetic valves. The selection of the valve type is still a matter of debate. However, according to literature data, complications seem to have occurred only in patients with bileaflet mechanical valves in the pulmonary position, whereas no thromboembolic episodes or valve failure is reported in subjects with tilting disc valves in the right ventricular outflow. Tilting disc valves might perform better in the right ventricular outflow than bileaflet valves. (J Thorac Cardiovasc Surg 1998;115:1074-9)

AbstractCoronary artery compression is a rare and potentially fatal complication after percutaneous pulmonary valve implantation. We report on a case of an acute antero-septal non-ST myocardial infarction secondary to the partial laceration of the conduit and the creation of a thrombus giving an extrinsic compression of left anterior descendent coronary artery after Melody valve implantation.

Pericardial cysts are intrathoracic lesions usually considered to be congenital and less frequently to be acquired. They are normally found incidentally upon chest radiography, computed tomography (CT)-scan or echocardiography. They are usually asymptomatic and have a benign behavior although sometimes they can cause clinical symptoms and diagnosis can be uncertain. We present, herein, the case of a 51-year-old male with a history of atrial arrhythmia resistant to transcatheter ablation therapies with an accidental finding of a pericardial cyst adherent to the lateral wall of the right atrium at the emerging superior vena cava. The patient was studied with transesophageal echocardiography, CT-scan and cardiovascular magnetic resonance with the evidence of dimensional increasing of the cyst at seriate controls. Considering this, in the absence of a definitive diagnosis and suspecting a link between the mass and the arrhythmia, the cyst was surgically removed through median sternotomy, off-pump on a beating heart. Postoperative course was unremarkable. Histopathologic examination confirmed it was a mesothelial pericardial cyst. At 12 months of follow-up the patient is doing well and he is in sinus rhythm. In conclusion, we believe that, although the majority of pericardial cysts need only radiological and clinical follow-up, surgical resection should be performed when the patient is symptomatic and when diagnosis is uncertain.

Medical advancements have significantly improved survival of patients with Congenital Heart Disease (CHD). Consequently, an aging CHD population are more susceptible to cardiac-related complications and co-morbidities.

Recent studies have suggested the beneficial effects of GH treatment in patients with dilated cardiomyopathy. We have treated with recombinant human growth hormone (rhGH) a 6-year-old female with a complex congenital heart defect (severe tricuspid hypoplasia and malposition of the great arteries), who developed a progressive dilated cardiomyopathy of unknown etiology. rhGH treatment (0,1 U/kg/day, for 3 months) did not improve cardiac function, nor clinical symptoms, although we have no clear explanations for this. However, a trial with rhGH may be offered to children with dilated cardiomyopathy and waiting for heart transplantation.

A seven-month-old girl had subaortic stenosis caused by a single intracavitary rhabdomyoma unassociated with tuberous sclerosis. Diagnosis was formulated on the basis of two-dimensional echocardiography and Doppler technique findings alone, and surgery was successful.

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Mucormycosis is an angioinvasive infection caused by ubiquitous filamentous fungi of the order Mucorales. We describe a case of rhinocerebral mucormycosis presented in a paediatric patient after cardiac surgery correction of a complete atrioventricular canal defect. The rhinocerebral form of mucormycosis in our patient presented as an important epistaxis that needed immediate intubation due to blood inhalation. Furthermore, due to the worsening of pulmonary function, the patient also needed mechanical support with veno-arterial extracorporeal membrane oxygenation. The patient died as a consequence of a disseminated form of fungal infection. We describe our experience of this rare opportunistic infection and we think that early recognition of the disease could help in proper management.

Transcatheter aortic valve replacement determined a paradigm shift in the treatment of high-risk patients with severe symptomatic aortic valve stenosis. Notwithstanding the impressive results of the first-generation prostheses, a fast-paced technological evolution is taking place to overcome their limitations, in particular the vascular access damage and the paravalvular leak. Nowadays, with the availability of several different devices, the expert operator can select the right prosthesis for the specific anatomical and clinical situation. As ‘One does not fit all’, the ‘Tailored TAVR Approach’ we describe will conceivably become the future of this therapy.

Cooperation activity in training programmes promoted by IRCCS Policlinico San Donato and Bambini Cardiopatici nel Mondo Association is a model of cooperation between people as an alternative intervention in promoting the right to healthcare, especially offering programmes of training and medical care on cardiovascular and congenital heart disease. This new strategy, implemented in several developing countries, has absolutely contributed to the improvement of the medical services concerning the diagnostic and surgical approach in the treatment of paediatric and adult cardiovascular disease. To strengthen this kind of activity, both IRCCS Policlinico San Donato and Bambini Cardiopatici nel Mondo have introduced a global perspective aiming at the realization of surgical missions ‘in situ’, building new cardiac surgery units in collaboration with the local partners, which are mainly university hospitals. They, furthermore, support financially the scholarship and accommodation in favour of medical and paramedical staff.

Thanks to the improvement of surgical and interventional cardiac procedures, the majority of children with congenital heart defects (CHD) can now be expected to reach adulthood. The number of adults with congenital heart disease (ACHD) will inevitably increase, and recent data affirm that in Europe, we are actually faced with an estimated patient population of 2.3 million. These patients, particularly the adults with moderate and highly complex CHD, can be very difficult to manage, and should be treated in few experienced and specialized ‘grown-up congenital heart disease’ (GUCH) units, concentrating resources, patients, funding, and professional experiences. On 2000, we created a GUCH unit in our hospital that rapidly became a referral centre in Italy for number and complexity of patients treated. The present study is a presentation of how is organized our GUCH unit and a brief overview of our more recent experience with ACHD patients.

The Thoracic Aortic Research Center (TARC) of the IRCCS Policlinico San Donato (PSD) aims to promote research on thoracic aortic diseases, to disclose the scientific knowledge and clinical experience and to develop new scientific paths within the Hospital and the aortic community, in collaboration with other national and international centres. Thoracic Aortic Research Center collaborates with many centres in both Europe (e.g. University of Utrecht, the Netherlands) and the USA (e.g. University of Michigan). This has led to multiple highly regarded publications in respected cardiovascular journals and has led to several PhD programmes resulting in doctorate degrees. Within Italy, in association with the Bioengineering School of the University of Pavia, TARC has founded the “BETA-lab” (Biomechanics for Endovascular Treatment of the Aorta laboratory), where MDs, Bioengineers, and PhD fellows conduct experimental studies using in vitro/ex vivo models of the physiologic aorta and aortic diseases. Furthermore, a database (iCardiocloud) where the medical imaging of cardiovascular patients from the PSD is structured, for in silico analysis utilizing computational fluid dynamics, and in vitro studies using also 3D printed aortic models. With the role of principal investigator or co-investigator, TARC at PSD has been participating in other several projects, including the International Registry of Acute Aortic Dissection, the International Aortic Arch Surgery Study Group, the European Registry of Endovascular Aortic Repair Complications, the ADSORB and ASSIST trials, and the GREAT registry. International collaborations have included also studies on predictors of aortic growth after dissection with the Yale University and University of Virginia, and on aortic biomarkers with the University of Tokyo.

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Congenital aortopulmonary window is a rare inborn cardiac malformation that should be surgically treated as soon as the diagnosis is made usually during infancy. We report a successful surgical treatment of a 23-year-old male patient with a big type III aortopulmonary window.