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A patent ductus arteriosus in patients with transposition of the great arteries is usually beneficial to allow shunting between pulmonary and systemic circulations. However, if the duct is too large, it can cause haemodynamic instability, pulmonary oedema and compromised organ perfusion. We present a neonate in whom a 5 mm short and tubular ductus arteriosus was causing significant cardiac failure with necrotising enterocolitis and liver impairment, leaving him too unstable for the arterial switch operation. At day 14 of life (3.1 kg), the baby underwent successful transcatheter closure using an Amplatzer vascular plug (Abbott, Chicago, Il, USA) delivered through a 5Fr catheter from the aortic side. The procedure was uncomplicated and successful as the neonate was extubated 2 days later. He subsequently underwent successful arterial switch surgery.

The use of Melody valves in the mitral position has been introduced in clinical practice. Stent fracture is a recognized complication of Melody valve implantation in the pulmonary position; however, reports in the mitral position are rare. We present the case of an 8-year-old boy in whom complete fracture of the proximal stent struts occurred, causing acute severe mitral stenosis, and in whom urgent hybrid transapical Melody valve implantation in the fractured Melody valve was performed successfully using a novel modified technique. This modification simplified the implantation, led to reduced time of the procedure, and minimized hemodynamic instability.

Aims COVID-19 has a wide spectrum of clinical presentation, from severe forms that require hospitalization to less severe forms that can be managed at home. An acute myocardial involvement was demonstrated in a large proportion of patients admitted for COVID-19 and may persist in the long term. We evaluated the possible cardiac involvement using echocardiography, comprehensive of right and left ventricular strain, in patients who recovered from SARS-CoV-2 infection (hospitalized or home-treated) comparing them with a population of healthy volunteers. Methods and results Forty-one patients with COVID-19, of which fifteen hospitalized, with no prior heart disease, were compared with 13 healthy volunteers. COVID-19 diagnosis was made by a positive molecular swab. Patients with history of pre-existing heart disease were excluded. The median time from infection to outpatient follow-up was 5.9 months. Numerous echocardiographic parameters were compared by unpaired t-test including left ventricular EF, left ventricular GLS, RV free wall strain, FAC, TAPSE, PAPS, TAPSE/PAPS ratio, RA area, and RV thickness. There was a significant difference in RV free wall strain between hospitalized patients and control (−14.6 ± 2.8% vs. −22 ± 0.7%; P-value 0.03) and between hospitalized and home-treated patients (−14.6 ± 2.8% vs. −19.8 ± 0.9%; P-value 0.03), the difference was not significant between control and home-treated patients (−22 ± 0.7% vs. −19.8 ± 0.8%; P-value 0.09). Between hospitalized and not hospitalized group there was a significant reduction in FAC (38.5 ± 3.2% vs. 44.7 ± 1.3%; P-value 0.03) with an increase of RV end diastolic area (19.9 ± 1.3 cm2 vs. 16.8 ± 0.7 cm2; P-value 0.037) and also of end systolic right atrium area (18.2 ± 1.3 cm2 vs. 15.4 ± 0.5 cm2; P-value 0.01). No difference was observed between hospitalized and home-treated patients in TAPSE (22.38 ± 1.26 mm vs. 23.02 ± 0.68 mm; P-value 0.6) and PAPS (24.3 ± 1.6 mmHg vs. 20.2 ± 1.4 mmHg; P-value 0.07) but there was a borderline significant decrease in right ventricular coupling evaluated with TAPSE/PAPS ratio (0.97 ± 0.08 mm/mmHg vs. 1.29 ± 0.10 mm/mmHg; P-value 0.056) and a significant increase in RV thickness in hospitalized patients (5.32 ± 0.45 mm vs. 3.69 ± 0.24 mm; P-value 0.0014). No significant differences were found between hospitalized and not hospitalized group in left ventricular EF (57.8 ± 1.9% vs. 59.9 ± 1.0%; P-value 0.3) and left ventricular GLS (−15.2 ± 0.6% vs. −16.4 ± 0.4%; P-value 0.1). Conclusions Patients hospitalized for COVID-19 showed a dysfunction in RV parameters at 6 months follow-up compared to non-hospitalized patients. No difference in RV function was found between home treated patients and healthy volunteers. No significant differences in LV function were found among the three groups. These preliminary data confirm a decrease in RV function in more severe COVID-19 infection requiring hospital admission, possibly related to increased pulmonary afterload.

Background Takotsubo syndrome (TTS) is a transient left ventricular dysfunction usually with apical akinesia (classical pattern). Other less frequent variants have been described: the mid-ventricular pattern is characterized by hypokinesia of the mid-left ventricle and hypercontractile apical and basal segments; the inverted or basal pattern is characterized by basal and mid-ventricular segment hypokinesia or akinesia with preserved contractility or hypercontractility of apical segments and finally the focal pattern. There are also biventricular variants and forms with exclusive involvement of the right ventricle. There is a correlation between endocrine disorders and TTS, the one most frequently described is with pheochromocytoma. Catecholamine-mediated myocarditis, focal and diffuse myocardial fibrosis, and myocardial dysfunction are described in pheochromocytoma. Case summary We describe a case of a 69-year-old patient with a recent diagnosis of hypertension and Graves’ disease, hospitalized for persistent chest pain, hypertensive crisis, tachycardia, dyspnoea, and diaphoresis. Thyroid hormones, antibodies to TSH receptors, and hs-troponin I were increased. Electrocardiogram showed sinus tachycardia at 130 b.p.m., first-degree atrioventricular block, signs of left ventricular hypertrophy with inverted T wave in V4–V6. Echocardiogram demonstrated left ventricular apical and para-apical akinesia. Coronary angiography ruled out an obstructive coronary artery disease. Computed tomography angiogram aortic dissection ruled out aortic dissection but incidentally revealed a left adrenal mass compatible with a pheochromocytoma. Plasma and urinary metanephrines were increased. A TTS secondary to pheochromocytoma and hyperthyroidism was diagnosed. Pharmacological treatment included nitrates, urapidil and esmolol IV and methimazole at high doses. Type 2 multiple endocrine neoplasia has been excluded. After a complete haemodynamic stability on 20th day of hospitalization, the patient underwent an adrenalectomy. Discussion High levels of catecholamines in pheochromocytoma can lead to myocardial dysfunction. Similarly, an excess of thyroid hormones with up-regulation of adrenergic system can lead to myocardial dysfunction. These two conditions, if both present, define a high haemodynamic risk profile. How do catecholamines interact with the thyroid gland? The clinical case is of interest as a relationship has been hypothesized between the incretion of plasma catecholamines and Graves’ disease. We suppose an imbalance of the immune system with a predominance of the T helper-type 2 (Th2)-mediated response. Predominance of Th2-mediated immune response may induce humoral immunity causing Graves’ disease. In addition Th2 cytokines are strong inducers of M2 macrophages (alternatively activated) that are involved in autoimmune diseases, myocarditis, and myocardial fibrosis. Knowing the interaction between the cardiovascular system, immune response, and endocrine glands can help define the patient's risk class, possible complications, and follow-up.

Background Severe pulmonary regurgitation (PR) and progressive right ventricular (RV) dilation and disfunction are common in patients with repaired Tetralogy of Fallot (r-TOF) and should be carefully monitored during the follow up of these patients. In this contest, Echocardiography and Cardiac Magnetic Resonance (CMR) have a complementary diagnostic role. Purpose To correlate Echo and CMR parameters in children ( Methods Paediatric patients with r- TOF with at least moderate PR at the echo evaluation who underwent a CMR study within six months were included by using hospital databases. All patients underwent standard echo-Doppler study including RV end-diastolic area (RVEDA), end-systolic area (RVESA), fractional area change (FAC) and TAPSE; PR was assessed by Color Doppler, continuous-wave (CW) Doppler and derived parameters such as pressure half time (PHT), PR index, ratio of diastolic and systolic time-velocity integrals (DSTVI) of the main pulmonary artery. By speckle tracking we measured also RV global longitudinal strain (RVGLS) and right atrial strain (RAS). All the patients underwent CMR to assess PR and right ventricular volumes and ejection fraction (EF). Of these, 36 patients underwent cardiopulmonary exercise test (CPET). Results Fourty-six children (aged 13.7±3.0 years) were included. Echo derived RV areas correlated significantly with CMR RV volumes (r=0.72, p21.9 cm2/m2 had a good sensitivity (83.3%) and specificity (73.5%) to identify a RV end-diastolic volume (RVEDV) ≥150 ml/m2. No correlation was found among TAPSE, FAC, RVGLS and RVEF calculated by CMR nor between PHT, PR index and DSTVI and PR-RF. Only A' wave velocity showed a significant but modest correlation with CMR RF (r=0.57, p Conclusion In children, flow reversal in pulmonary branches identifies hemodynamically significant PR at CMR. RV area by echocardiogram is a valid first-line parameter to screen RV dilation. Our study suggests that, also for the RV, there is longitudinal systolic dysfunction in presence of preserved RV EF. RAS is the best predictor of peak Vo2 and should be added in the follow up of these patients. Funding Acknowledgement Type of funding source: None

Background Norwood procedure represents the first of three surgical steps toward Hypoplastic left heart syndrome (HLHS) complete palliation. Two are the main surgical techniques allowing the reconstruction of Norwood circulation: the right ventricle-pulmonary artery shunt (RVPAS) and the modified Blalock-Taussing shunt (mBTS). However, still little is known about the impact on right ventricle (RV) function of the required ventriculotomy for the RVPAS. Purpose The aim of the study was to investigate the changes in RV function in HLHS patients after RVPAS vs mBTS. Methods The cohort included 27 consecutive HLHS patients (10 in the modified Blalock-Taussig shunt group and 17 in the RVPAS group) who successfully underwent Norwood procedure in a single tertiary paediatric cardiology centre. Longitudinal strain (LS) and strain rate (LSR), tricuspid annulus peak systolic excursion (TAPSE) and fractional area change (FAC) were evaluated in all patients before Norwood and in three different breakpoints in the steady state after Norwood procedure (30 days after Norwood, 90 days after Norwood, 140 days after Norwood). Results Ventricular loading conditions (diuretic treatment, blood pressure and tricuspid regurgitation) were similar in both groups. No significant differences were found at different time points between RVPAS and mBTS group in terms of LS, LSR, TAPSE and FAC. However, when we compared RV function before and after Norwood procedure, 90 days after the procedure, patients who did not undergo RV ventriculotomy (mBTS group) showed significant improvement in LS compared to pre-surgical assessment (mBTS: +27.35±43.47% vs RVPAS: −8,20±25.25%, p=0,03). This finding was consistent but no longer statistically significant at 140 days after Norwood (mBTS: +13.81±21.99% vs RVPAS: −4.90±27.97%, p=0,12). Conclusion After Norwood procedure mBTS patients showed a significant increase in LS when compared with patients who underwent RVPAS. This finding was consistent but no longer significant at 140 days after Norwood probably because the number of patients was too small to reach a significant level. These data support the use of LS in HLHS patient's evaluation and may be of value to find a patient-tailored timing for the second surgical stage. TAPSE, FAC, LS and LSR trends Funding Acknowledgement Type of funding source: None

Summary Background Cardiac catheterization is the gold-standard modality for investigation of cardiovascular morphology before bidirectional cavopulmonary connection, but requires general anaesthesia and is associated with procedural risk. Aims To assess the diagnostic accuracy and safety of computed tomography in diagnosing great vessel stenosis/hypoplasia compared with cardiac catheterization and surgical findings. Methods Twenty-seven patients (10 after Norwood stage I) underwent computed tomography before surgery between January 2010 and June 2016; 16 of these patients also underwent cardiac catheterization. Proximal and distal pulmonary artery, aortic isthmus and descending aorta measurements, radiation dose and complications were compared via Bland-Altman analyses and correlation coefficients. Results The accuracy of computed tomography in detecting stenosis/hypoplasia of either pulmonary artery was 96.1% compared with surgical findings. For absolute vessel measurements and Z-scores, there was high correlation between computed tomography and angiography at catheterization (r = 0.98 for both) and a low mean bias (0.71 mm and 0.48; respectively). The magnitude of intertechnique differences observed for individual patients was low (95% of the values ranged between −0.9 and 2.3 mm and between −0.7 and 1.7, respectively). Four patients (25%) experienced minor complications from cardiac catheterization, whereas there were no complications from computed tomography. Patients tended to receive a higher radiation dose with cardiac catheterization than with computed tomography, even after exclusion of interventional catheterization procedures (median 2.5 mSv [interquartile range 1.3 to 3.4 mSv] versus median 1.3 mSv [interquartile range 0.9 to 2.6 mSv], respectively; P = 0.13). All computed tomography scans were performed without sedation. Conclusions Computed tomography may replace cardiac catheterization in identification of great vessel stenosis/hypoplasia before bidirectional cavopulmonary connection when no intervention before surgery is required. Computed tomography carries lower morbidity, can be performed without sedation and may be associated with less radiation.

Background Pulmonary regurgitation (PR) and right ventricular (RV) dilatation and disfunction are common in patients with repaired Tetralogy of Fallot (r-TOF). Aims To compare Echo data with the gold standard CMR in a paediatric population of r-TOF with significant PR, to assess the reliability of standard and advanced echo parameters. In addition, to evaluate their correlation with peak oxygen consumption (VO2). Methods and results All patients underwent standard echo-Doppler study, speckle tracking analysis, and CMR to assess PR and RV size and function. Thirty-six patients underwent also cardiopulmonary exercise test. Fourty-six patients (aged 13.7 ± 3.0) were included. Echo derived RV areas correlated with CMR RV volumes (p 21.9 cm2/m2 had a good sensitivity (83.3%) and specificity (73.5%) to identify a RV end-diastolic volume ≥ 150 ml/m2. RVEF was preserved in all patients, while TAPSE was reduced in 78.2% and RVGLS in 60.8%. Flow-reversal in pulmonary branches showed a sensitivity of 95.8% and a specificity of 59.1% to identify CMR pulmonary regurgitant fraction (RF) ≥ 35%. None of the CMR parameters correlated with peak VO2. Among the Echo data only right atrial strain (RAS) correlated with peak VO2. Conclusion In children, flow-reversal in pulmonary branches identifies hemodynamically significant RF with a good sensitivity but poor specificity. RV area by echocardiogram is a valid first-line parameter to screen RV dilation. RV longitudinal systolic dysfunction coexists with a still preserved EF. RAS correlates strongly with peak VO2 and should be added in their follow up.

ObjectivesInfants with symptomatic partial and transitional atrioventricular septal defect undergoing early surgical repair are thought to be at greater risk. However, the outcome and risk profile of this cohort of patients are poorly defined. The aim of this study was to investigate the outcome of symptomatic infants undergoing early repair and to identify risk factors which may predict mortality and reoperation.MethodsThis multicentre study recruited 51 patients (24 female) in three tertiary centres between 2000 and 2015. The inclusion criteria were as follows: (1) partial and transitional atrioventricular septal defect, (2) heart failure unresponsive to treatment, (3) biventricular repair during the first year of life.ResultsMedian age at definitive surgery was 179 (range 0–357) days. Sixteen patients (31%) had unfavourable anatomy of the left atrioventricular valve: dysplastic (n=7), double orifice (n=3), severely deficient valve leaflets (n=1), hypoplastic left atrioventricular orifice and/or mural leaflet (n=3), short/poorly defined chords (n=2). There were three inhospital deaths (5.9%) after primary repair. Eleven patients (22%) were reoperated at a median interval of 40 days (4 days to 5.1 years) for severe left atrioventricular valve regurgitation and/or stenosis. One patient required mechanical replacement of the left atrioventricular valve. After median follow-up of 3.8 years (0.1–11.4 years), all patients were in New York Heart Association (NYHA) class I. In multivariable analysis, unfavourable anatomy of the left atrioventricular valve was the only risk factor associated with left atrioventricular valve reoperation.ConclusionsAlthough surgical repair is successful in the majority of the cases, patients with partial and transitional atrioventricular septal defect undergoing surgical repair during infancy experience significant morbidity and mortality. The reoperation rate is high with unfavourable left atrioventricular valve anatomy.

Objectives We present the initial 2-year results of CardioCel® patch (Admedus Regen Pty Ltd, Perth, WA, Australia) implantation in paediatric patients with congenital heart diseases. Methods This was a single-centre retrospective study with prospectively collected data of all patients aged 18 years and under operated for congenital heart disease. The patch was introduced in 2014, with clinical practice committee approval and a special consent in case of an Ozaki procedure. Standard follow-up was performed with systematic clinical exams and echocardiograms. In case of reoperation or graft failure, the patch was removed and sent for a histological examination. Results Between March 2014 and April 2016, 101 patients had surgical repair using a CardioCel patch. The mean age was 22 (±36.3) months, and the mean weight was 9.7 (±10.3) kg. No infections and no intraoperative implantation difficulties were associated with the patch. The median follow-up period was 212 (range 4-726) days. The overall 30-day postoperative mortality was 3.8% (n = 4), none of which were related to graft failure. Five children were reoperated because of graft failure, 4 of whom had the patch implanted for aortic and were aged less than 10 days. The indications for patch implantation in the aortic position were aortopulmonary window, truncus arteriosus, coarctation and aortic arch hypoplasia repair. The median time between the first and the second operation for graft failure was 245 (range 5-480) days. Conclusions Our experience shows that the patch is well tolerated in the septal, valvar and pulmonary artery positions. However, we experienced graft failures in infants in the aortic position.

Introduction Hypoplastic Left Heart Syndrome (HLHS) has high mortality and morbidity and systemic right ventricle (RV) dysfunction may play a key-role. Study aim is to evaluate the accuracy of speckle-tracking echocardiographic (STE) assessment of RV deformation and 2D standard echo parameters in predicting outcome in HLHS patients. Methods We studied 27 HLHS patients (17 male) who successfully completed Norwood palliation. All the patients underwent in-hospital interstage stay. Serial echocardiographic assessment was performed: baseline, one-month after Norwood, three-months after Norwood, one-week before bidirectional cavopulmonary anastomosis (BCPA) and two-months after BCPA. From the apical view we measured: tricuspid annulus peak systolic excursion (TAPSE), fractional area change (FAC), longitudinal strain (LS) and strain rate (LSR). Results After a mean follow-up of 1.18 (± 1.16) years, 8 out of 27 of the included patients met the composite endpoint of death/heart transplant (HT). At pre-Norwood assessment, there was no difference in echo measurements between survivors and patients with events. In death/HT group TAPSE and LS declined already one-month after Norwood procedure: TAPSE ≤5 mm had good sensitivity (85.71%) and moderate specificity (63.16%) for death/HT (AUC = 0.767); a decrease of LS > 8.7% vs baseline showed 100% sensitivity and 84.21% specificity for death/HT (AUC = 0.910). At multivariate analysis, one-month-after-Norwood LS drop >8.7% was the best predictor of outcome (P = 0.01). Conclusions RV dysfunction in HLHS carries prognostic value. Our findings encourage serial measurements of RV function to identify the subgroup of HLHS patients at higher risk. In our experience, ∆ LS showed the best predictive value.

Funding Acknowledgements EACVI Training Grant Introduction Children with Hypoplastic Left Heart Syndrome (HLHS) have a high mortality (up to 95%) and morbidity. Systemic right ventricle (RV) dysfunction plays a key-role in their outcome. Purpose The aim of this study is to evaluate the accuracy of speckle-tracking echocardiographic (STE) assessment of RV deformation and 2D standard echo parameters in predicting death and need for heart transplantation (HT) in HLHS patients. Methods 31 patients with diagnosis of HLHS successfully completed Norwood (n = 29) or comprehensive Norwood stage II (n = 2) at our Institution between 2015 and 2019. Survival at 6 months was 90.32%, survival at 18 months was 85.72%. We studied 29 HLHS patients (17 male). Patients with HLHS variant (n = 2) were excluded. All the studied patients underwent in-hospital interstage stay. Serial echocardiographic assessment was performed in all the included patients (baseline, one month after Norwood, three months after Norwood, one week before bidirectional cavopulmonary anastomosis [BCPA] and two months after BCPA). From the apical view we measured: tricuspid annulus peak systolic excursion (TAPSE), fractional area change (FAC), longitudinal strain (LS) and strain rate (LSR). Results After a mean follow-up of 1.83 ± 1.16 years, 8 out of 29 of the included patients met the composite endpoint of death/HT. At pre-Norwood assessment, there was no statistical difference in echo measurements between survivors and patients who reached the endpoint. In death/HT group TAPSE and LS declined already at one-month after Norwood procedure evaluation. At one-month evaluation, a TAPSE ≤ 5 mm had a good sensitivity for death/HT (85.71%) and a moderate specificity (66.67%), with an area under the curve of 0.789. Always at one-month evaluation, a Δ LS ([{baseline LS – one-month post Norwood LS}/ baseline LS] *100) of 8.7% showed a 100% sensitivity and good specificity (80.95%) for death/HT, with an area under the curve of 0.888. Multivariate analysis showed that one-month-after-Norwood Δ LS was the best predictor of worse outcome (p = 0.02). Conclusions HLHS patients with Δ LS of > 8.7% at one-month after Norwood procedure had a high likelihood of death or HT. These data encourage the routine use of LS to monitor cardiac function in HLHS patients. Abstract 1233 Figure. HLHS LS and trend of TAPSE and LS.

Large conductive arteries undergo to structural modifications by aging, eventually leading to increased vascular stiffness. As consequence, cardiovascular hemodynamic changes by increasing central blood pressure which may be also associated to the remodelling of peripheral resistance arteries that contribute to increase further the central vascular stiffness and blood pressure. These modifications resemble the ones that has been shown in essential hypertension, thus a condition of "early vascular aging" has been described in hypertensive patients. Since hypertension related target organs, particularly the heart, face aortic blood pressure rather than brachial blood pressure, it has been recently suggested that central blood pressure and other parameters of large arteries' stiffness, including pulse wave velocity (PWV), may better correlate with subclinical organ damage and might be useful to assess the cardiovascular risk of patients beyond the traditional risk factors. Different devices have been validated to measure central blood pressure and PWV, and are currently available for clinical use. The increasing application of these tools in clinical practice could improve the management of hypertensive patients by better defining the cardiovascular risk and address the antihypertensive therapy.

OBJECTIVES The interstage mortality rate after a Norwood stage 1 operation remains 12–20% in current series. In-hospital interstage facilitates escalation of care, possibly improving outcome. METHODS A retrospective study was designed for hypoplastic left heart syndrome (HLHS) and HLHS variants, offering an in-hospital stay after the Norwood operation until the completion of stage 2. Daily and weekly examinations were conducted systematically, including two-dimensional and speckle-tracking echocardiography. Primary end points included aggregate survival until the completion of stage 2 and interstage freedom from escalation of care. Moreover, we calculated the sensitivity and specificity of speckle-tracking echocardiographic myocardial deformation in predicting death/transplant after the Norwood procedure. RESULTS Between 2015 and 2019, 33 neonates with HLHS (24) or HLHS variants (9) underwent Norwood stage 1 (31) or hybrid palliation followed by a comprehensive stage 2 operation (2). Stage 1 Norwood–Sano was preferred in 18 (54.5%) neonates; the classic Norwood with Blalock–Taussig shunt was performed in 13 (39.4%) neonates. The Norwood stage 1 30-day mortality rate was 6.2%. The in-hospital interstage strategy was implemented after Norwood stage 1 with a 3.4% interstage mortality rate. The aggregate Norwood stage 1 and interstage Kaplan–Meier survival rate was 90.6 ± 5.2%. Escalation of care was necessary for 5 (17.2%) patients at 2.5 ± 1.2 months during the interstage for compromising atrial arrhythmias (2), Sano-shunt stenosis (1) and pneumonia requiring a high-frequency oscillator (2); there were no deaths. A bidirectional Glenn (25) or a comprehensive-Norwood stage 2 (2) was completed in 27 patients at 4.7 ± 1.2 months with a 92.6% survival rate. The overall Kaplan–Meier survival rate is 80.9 ± 7.0% at 4.3 years (mean 25.3 ± 15.7 months). An 8.7% Δ longitudinal strain 30 days after Norwood stage 1 had 100% sensitivity and 81% specificity for death/transplant. CONCLUSIONS In-hospital interstage facilitates escalation of care, which seems efficacious in reducing interstage Norwood deaths. A significant reduction of longitudinal strain after Norwood stage 1 is a strong predictor of poor outcome.

Endocarditis is life threatening after percutaneous pulmonary valve implantation. We report a patient with acute heart failure caused by obstructive pulmonary vegetation in a Melody valve. Because of severe right ventricular dysfunction, immediate cardiac surgery was contraindicated. The patient underwent trans-catheter implantation of a covered stent to relieve obstruction. Following an initially good outcome, surgery was performed 2 weeks later. This was complicated by left main coronary artery tear and fatal bleeding. Despite covered stent was an effective bridge, surgery remains extremely challenging in such cases with important surrounding inflammation and fibrosis as well as potential proximity of coronary arteries.

Few patients experience migraines after transcatheter closure of secundum atrial septal defects with nitinol devices. These migraines are usually treated with analgesics and resolve after a few months as the device endothelializes. This report describes the case of a 16-year-old male patient who required surgical explantation of the device 6 years after closure because of debilitating headaches. He had a grade 1 reaction to nickel after skin testing. Intraoperatively, the device had not fully endothelialized. Immediately after removal of the device, his headaches completely resolved. Long-term nickel allergy may cause severe migraine secondary to a lack of endothelialization of a device. Patients with extreme cases may require surgical removal of the device.

Background Accurate risk stratification of patients with Ebstein's anomaly (EA) is crucial. Aim of the study was to assess the prognostic value of echocardiography, including 2D speckle tracking (STE) derived myocardial deformation indices, for predicting outcome in pediatric and young adult unrepaired EA patients. Methods Fifty consecutive EA patients (1 day–18 years, 52% males) underwent echocardiography and were followed for a mean follow-up of 60 ± 41 months for clinical outcome (ventricular tachyarrhythmia, heart failure, need for surgery and/or death). Clinical and instrumental features of EA patients with stable disease were compared with those of EA patients with progressive disease. Results Twenty-four (48%) EA patients had progressive disease. A more severe grade of tricuspid valve (TV) displacement [59.7 mm/m2 (IQR 27.5–83) vs 28.4 mm/m2 (IQR 17.5–47); p = 0.002], a lower functional right ventricle (RV) fractional area change (FAC) (29.2 ± 7.7% vs 36.7 ± 9.6%; p = 0.004), a higher Celermajer index [0.8 (IQR 0.7–0.98) vs 0.55 (IQR 0.4–0.7); p = 0.000], a lower functional RV-longitudinal strain (−10.2 ± 6.2% vs −16.2 ± 7.3%; p = 0.003) and a lower right atrium peak systolic strain (RA-PALS) (25.2 ± 13.5% vs 36.3 ± 12.5%; p = 0.004) were detected in progressive disease group compared to stable one, respectively. Functional RV-FAC and RA-PALS were independent predictors of progressive disease at multivariate analysis. Conclusion Our study demonstrated for the first time the prognostic role of RV-FAC and RA-PALS in a long-term follow-up of EA young patients. A complete echocardiographic evaluation should be regular part in the evaluation and risk-stratification of EA children.

Mitral disease in children is profoundly debilitating with limited therapeutic options and poor outcome. Surgical implantation of a modified expandable stented valve (Melody, Medtronic, Minneapolis, Minnesota) in the mitral position has become an alternative to a mechanical/biological prosthesis [(1

Aims Net clinical benefit of long-term oral anticoagulation therapy (OAT) continuation after successful atrial fibrillation (AF) ablation is still controversial. To evaluate long-term thromboembolic (TE) and haemorrhagic events incidence according to OAT strategy used after AF transcatheter ablation. Methods and results Three months after AF ablation, OAT was discontinued in patients with CHADS2 ≤ 1 if no recurrences were documented, while OAT was maintained in patients with CHADS2 ≥ 2 regardless of AF recurrences. CHA2DS2VASc and HAS-BLED scores have been retrospectively evaluated. Seven hundred and sixty-six patients were followed for a median of 60.5 months. Six (6/267 = 2.2%) and five (5/499 = 1%) TE events occurred in the ON and the OFF-OAT patients, respectively ( P = 0.145), all in concomitance with the AF recurrence. CHADS2 and CHA2DS2VASc ≥ 2 were associated with high TE incidence ( P = 0.047 and P = 0.020). Among patients with a CHADS2 score of 0 or 1, a CHA2DS2VASc score ≥ 2 was predictive of TE events ( P = 0.014). Overall, the incidence of the TE events in patients with CHA2DS2VASc ≥ 2 was 0.6 per 100 patient-years whereas seven haemorrhagic events occurred, all of them in the ON-OAT patients (7/267 = 2.6%). Conclusion Patients with AF undergoing transcatheter ablation have a lower incidence of TE events as compared with the general AF population, regardless of OAT maintenance. The unpredictable risk of AF recurrence, mandate the routine use of the CHADS2, CHA2DS2VASc, and HAS-BLED scores to guide clinical decision regarding OAT management in this peculiar setting of patients. The potential protective role of rhythm control strategy in the TE events needs to be confirmed by future large randomized trials.

OBJECTIVES: Fontan failure (FF) represents a growing and challenging indication for paediatric orthotopic heart transplantation (OHT). The aim of this study was to identify predictors of the best mid-term outcome in OHT after FF.METHODS: Twenty-year multi-institutional retrospective analysis on OHT for FF.RESULTS: Between 1991 and 2011, 61 patients, mean age 15.0 +/- 9.7 years, underwent OHT for failing atriopulmonary connection (17 patients = 27.8%) or total cavopulmonary connection (44 patients = 72.2%). Modality of FF included arrhythmia (14.8%), complex obstructions in the Fontan circuit (16.4%), protein-losing enteropathy (PLE) (22.9%), impaired ventricular function (31.1%) or a combination of the above (14.8%). The mean time interval between Fontan completion and OHT was 10.7 +/- 6.6 years. Early FF occurred in 18%, requiring OHT 0.8 +/- 0.5 years after Fontan. The hospital mortality rate was 18.3%, mainly secondary to infection (36.4%) and graft failure (27.3%). The mean follow-up was 66.8 +/- 54.2 months. The overall Kaplan-Meier survival estimate was 81.9 +/- 1.8% at 1 year, 73 +/- 2.7% at 5 years and 56.8 +/- 4.3% at 10 years. The Kaplan-Meier 5-year survival estimate was 82.3 +/- 5.9% in late FF and 32.7 +/- 15.0% in early FF (P = 0.0007). Late FF with poor ventricular function exhibited a 91.5 +/- 5.8% 5-year OHT survival. PLE was cured in 77.7% of hospital survivors, but the 5-year Kaplan-Meier survival estimate in PLE was 46.3 +/- 14.4 vs 84.3 +/- 5.5% in non-PLE (P = 0.0147). Cox proportional hazards identified early FF (P = 0.0005), complex Fontan pathway obstruction (P = 0.0043) and PLE (P = 0.0033) as independent predictors of 5-year mortality.CONCLUSIONS: OHT is an excellent surgical option for late FF with impaired ventricular function. Protein dispersion improves with OHT, but PLE negatively affects the mid-term OHT outcome, mainly for early infective complications.

The population of neonates and children with congenital heart defects presents about a 30% prevalence of associated genetic syndrome or additional extracardiac anomalies and may show an increased risk of death or major complication at cardiac surgery. Since a well-defined pattern of combined cardiac and extracardiac anomalies may be found in relation to specific genetic defects, correct understanding of the genetic issues may help improving diagnosis, surgical approach and final outcome of these patients. Hereby we review the medical and surgical issues correlated to the genetic asset in patients with congenital heart defects and genetic syndromes, including trisomy 21, deletion 22q11, Noonan/LEOPARD, Turner, Marfan and Williams syndromes. Recognition of specific surgical risk factors can lead to the preparation of specific diagnostic and perioperative protocols in order to reduce operative mortality and morbidity.

OBJECTIVE: The objective of this European multicenter study was to report surgical outcomes of Fontan takedown, Fontan conversion and heart transplantation (HTX) for failing Fontan patients in terms of all-cause mortality and (re-)HTX.METHODS: A retrospective international study was conducted by the European Congenital Heart Surgeons Association among 22 member centres. Outcome of surgery to address failing Fontan was collected in 225 patients among which were patients with Fontan takedown (n=38; 17%), Fontan conversion (n=137; 61%) or HTX (n=50; 22%).RESULTS: The most prevalent indication for failing Fontan surgery was arrhythmia (43.6%), but indications differed across the surgical groups (pCONCLUSIONS: Takedown surgery for failing Fontan is mostly performed in the early postoperative phase, with a high risk of mortality. There is no difference in survival after Fontan conversion or HTX.

We present a case of a 71-year-old Caucasian male with a ventriculo-atrial defect due to infective endocarditis, originating from his aortic root near a bioprosthetic aortic valve, implanted 4 years earlier. Ventriculo-atrial defects are rare and can occur after endocarditis with abscess formation, usually in native tissue. We report a ventriculo-atrial defect due to a paravalvular aortic prosthetic defect, secondary to inflammation, a novel third type of a Gerbode defect. Case presentation, clinical decision making and surgical approach are discusses in this report.

Clinical Outcome of 193 Extracardiac Fontan Procedure Patients: The First 15 Years Salvatore Giannico, Fatma Hammad, Antonio Amodeo, Guido Michielon, Fabrizio Drago, Attilio Turchetta, Roberto Di Donato, Stephen P. Sanders The clinical outcome of 193 early survivors of an extracardiac Fontan procedure is reported. The overall survival was 85% at 15 years. A total of 127 of 165 mid-term survivors (77%) are in good clinical conditions with no major late complications. The incidence of late arrhythmias (11%), obstructions of the extracardiac conduit (3%), and re-interventions (12.7%) seems acceptably low. These promising data suggest that the extracardiac Fontan procedure is a suitable therapeutic option in patients with a single ventricle. Objectives We sought to evaluate the mid-term outcome of hospital survivors with extracardiac Fontan circulation. Background Few data exist about the mid-term and long-term results of the extracardiac Fontan operation. Methods From November 1988 to November 2003, 221 patients underwent an extracardiac Fontan procedure as primary (9 patients) or secondary (212 patients) palliation, at a mean age of 72.2 months (range 13.1 to 131.3 months). A total of 165 of 193 early survivors underwent programmed noninvasive follow-up evaluations and at least one cardiac catheterization. Results The overall survival, including operative deaths, was 85% at 15 years. Freedom from late failure among hospital survivors is 92% at 15 years. A total of 127 of 165 survivors (77%) were in New York Heart Association functional class I. The incidence of late major problems was 24% (42 major problems in 36 of 165 patients): 19 patients had arrhythmias (11%), 5 patients had obstruction of the extracardiac conduit (3%) and 6 of the left pulmonary artery (3.5%), and 5 patients experienced ventricular failure (3%), leading to heart transplantation in 3 patients. Protein-losing enteropathy was found in two patients (1%). The incidence of late re-interventions was 12.7% (21 of 165 patients, including 15 epicardial pacemaker implantations). Four patients died (2.3%), two after heart transplantation. Conclusions After 15 years of follow-up, the overall survival, the functional status, and the cardiopulmonary performance of survivors of the extracardiac Fontan procedure compare favorably with other series of patients who underwent the lateral tunnel approach. The incidence of late deaths, obstructions of the cavopulmonary pathway, re-interventions, and arrhythmias is lower than that reported late after other Fontan-type operations.

Objectives: To evaluate late outcome of non-isomeric total anomalous pulmonary venous connection (TAPVC) repair, controlling for anatomic subtypes and surgical technique. Methods: Between 1983 and 2001, 89 patients (median age 54 days) underwent repair for supracardiac (38), cardiac (26), infracardiac (16) or mixed (nine) TAPVC. Ten patients (11.2%) presented associated anomalies other than PDA. Twenty-eight patients (31.5%) were emergencies, due to obstructed drainage. Supracardiac and infracardiac TAPVC repair included the double-patch technique with left atrial enlargement in 29 patients and side-to-side anastomosis between the pulmonary venous (PV) confluence and the left atrium in 29 patients. Coronary sinus unroofing was preferred for cardiac TAPVC repair. Total follow-up was 727.16 patient-years (mean 8.55 years, 98.8% complete). Results: Early mortality was 7.86% (7/89). Ten patients (11.2%) underwent reintervention, including reoperation (eight), balloon dilation (one) and intraoperative stents placement (one), for anastomotic (four) or diffuse PV stenosis (six), with four late deaths. Kaplan‐Meier survival is 87.3 ^ 0.036 SE% at 18.07 years with no difference according to anatomic type or surgical technique. Freedom from PV reintervention for operative survivors is 86.7 ^ 0.052 SE% at 18.07 years. Cox proportional hazard indicates associated anomalies (P ¼ 0:008) and reoperation for intrinsic PV stenosis (P ¼ 0:034) as independent predictors of mortality. According to logistic analysis, preoperative obstruction predicts higher risk of reintervention for intrinsic PV stenosis (P ¼ 0:022), while the double-patch technique increased the risk of late arrhythmias (P ¼ 0:005). Conclusions: Side-to-side anastomosis provides excellent results for TAPVC repair while left atrial enlargement procedures appear to be associated with higher risk of late arrhythmias. Although early and aggressive reintervention for recurrent PV obstruction is mandatory, intrinsic PV stenosis remains a predictor of adverse outcome. q 2002 Elsevier Science B.V. All rights reserved.

We present a case of a 71-year-old Caucasian male with a ventriculo-atrial defect due to infective endocarditis, originating from his aortic root near a bioprosthetic aortic valve, implanted 4 years earlier. Ventriculo-atrial defects are rare and can occur after endocarditis with abscess formation, usually in native tissue. We report a ventriculo-atrial defect due to a paravalvular aortic prosthetic defect, secondary to inflammation, a novel third type of a Gerbode defect. Case presentation, clinical decision making and surgical approach are discusses in this report. Electronic supplementary material The online version of this article (doi:10.1186/s13019-014-0137-1) contains supplementary material, which is available to authorized users.

Genetic syndromes occur in 20% of patients with tetralogy of Fallot (TOF). The impact of genetic syndromes on surgical repair of TOF in infancy is still under investigation. METHODS: This retrospective study reviews the outcome of 306 consecutive patients (median age, 5.1 months) who underwent primary (266) or staged (40) repair of TOF between 1994 and 2004. Total follow-up was 1,188 patient-years (mean, 57 months). RESULTS: Genetic syndromes were documented in 85 patients (27.8%), including 22q11 deletion (27), trisomy 21 (13), vertebral, anal, cardiac, tracheoesophageal, renal, and limb abnormalities (VACTERL, 12), and others (33). Hypoplastic pulmonary arteries (PA) were more common in syndromic (19/85 = 22.3%) than nonsyndromic TOF (20/221 = 9.04%) (p < 0.001). Primary repair was performed in 82.4% syndromic and 88.7% nonsyndromic TOF (p = not significant [NS]). Ten-year actuarial survival was 94.1 +/- 2.3% in nonsyndromic and 84.3 +/-4.2% in syndromic TOF (p < 0.001). Ten-year survival was 96.3 +/- 3.6% for del22q11, 100% for trisomy 21, 63.6 +/- 14.5% for VACTERL, and 78.5 +/- 7.3% for patients with other syndromes (p = 0.022). Survival in syndromic TOF with normal PA anatomy was 89.6 +/- 4.2% for primary repair and 85.7 +/- 12.8% for staged repair (p = NS); freedom from reoperation after complete repair was 74.4 +/- 6.4% for primary correction and 56.3 +/- 11.9% for staged repair (p = 0.04). Cox proportional hazard identified the presence of genetic syndrome (p = 0.011) and central PA hypoplasia (p = 0.002) as independent predictors of mortality. CONCLUSIONS: Pulmonary arborization defects and genetic syndromes other than del22q11 or trisomy 21, are associated with worse outcome after correction of TOF. Primary TOF repair in syndromic patients with normal PA anatomy is a valid surgical strategy, with no additional risk for mortality and higher freedom from reintervention.

From January 1988 through to July 1994, 54 consecutive infants underwent an arterial switch operation for simple or more complex forms of complete transposition (concordant atrioventricular and discordant ventriculo-arterial connections). They ranged in age from 2 to 180 days. The Lecompte maneuver was performed in all. In the first 19 patients the harvested sinuses of Valsalva were filled with two separate patches of autologous preserved pericardium, while, in the last 35 patients, a wide pantaloon patch of tanned pericardium was employed. Five babies died within 30 days after the procedure (operative mortality of 9.2%, 70% CL 4–17%). Four survivors were found to have developed a significant supravalvar pulmonary stenosis from 1 month to 21 months postoperatively. Echocardiographic data showed a transpulmonary peak systolic gradient from 60 mmHg to 101 mmHg, with a right-to-left ventricular systolic pressure ratio from 0.65 to 0.9. Reoperation was performed from 8 months to 39 months after the arterial switch procedure. Supravalvar pulmonary stenosis was located at the level of the pulmonary trunk, extending distally in two cases, due to the growth of fibrous scarring tissue with partial calcification. The pulmonary valvar leaflets were involved in two cases. Relief of the obstruction was obtained by insertion of a wide shield-tailored polytetrafluoroethylene patch after making an inverted Y-shaped longitudinal incision in the pulmonary trunk between the anterior sinuses of Valsalva. No mortality occurred at reoperation. Early and midterm echocardiographic measurements showed the effectiveness of this technique, with only trivial or mild residual transpulmonary gradients.

Reoperation rate for residual atrioventricular (AV) valve regurgitation after repair of common atrioventricular canal defect (AVCD) is currently between 5% and 10%. This retrospective analysis evaluates the impact of AV valve anatomy, age at repair, and surgical technique on postoperative AV valve incompetence.Between January 1982 and July 1994, 205 patients with common AVCD underwent surgical repair at our institution. A complete form with common atrioventricular orifice (CCAVCD) was present in 81 patients. One hundred twenty-four had separate AV valves and orifices; 22 of these had a restrictive ventricular septal defect component. Preoperative moderate to severe AV valve regurgitation (AVVR) was present in 74 (36%), with a lower incidence in the CCAVCD group (20/81, 25%) versus the transitional (8/22, 36%) or the partial forms (46/102, 45%) (p = 0.02). The incidence was 12.5% (3/24) for CCAVCD patients less than 4 months of age (p = 0.02). Repair of the left AV valve was performed according to the trifoliate approach in most cases (142/205, 70%). Follow-up was 98% complete.By Kaplan-Meier analysis, survival at 12.3 years was 97.8% +/- 1% in partial AVCD, 95.4% +/- 4% in transitional AVCD, and 73.2% +/- 5% in CCAVCD (median follow-up, 60 months). Freedom from reoperation at 12.3 years was 93.5% +/- 2% for partial AVCD, 76.9 +/- 9% for the transitional form, and 68.3% +/- 5% for CCAVCD: Postoperative moderate to severe AVVR occurred in 42 patients (21%), with lower incidence for CCAVCD (10/81, 12.5%) versus transitional AVCD (8/22, 36%) and partial AVCD (24/102, 24%) (p = 0.02). Postoperative moderate AVVR was found in only 1 patient with CCAVCD less than 4 months of age (p0.01). Nine patients (5%) underwent reoperation for residual postoperative AVVR Valve repair was performed in all with no operative death. By Cox proportional risk multivariate analysis, preoperative AVVR and double orifice "mitral" valve were associated with increased risk of postoperative left AVVR (p0.01), whereas a bifoliate approach appeared to reduce the risk of this event in partial AVCD (p = 0.03).Postoperative AVVR is related to the type of anatomy of the AV valve, to the age at repair, and to the surgical technique employed. Residual AVVR can still be corrected with conservative techniques at low mortality rates. Early repair of common AVCD is associated with a lower incidence of preoperative and postoperative AVVR in CCAVCD and seems to prevent progression of annular dilation and preoperative AVVR in partial AVCD.

Background This report describes our experience with primary correction of tetralogy of Fallot in infants. Methods Fifty-one consecutive infants younger than 6 months underwent primary correction of tetralogy of Fallot between January 1978 and October 1994. Mean age at repair was 4.2 months. Four were neonates. Correction was accomplished through a right ventriculotomy in the first consecutive 22 patients (43%; group A); since 1991, a combined transatrial-transpulmonary approach was used in 29 consecutive patients (57%; group B). A transannular patch was necessary in 33 infants (65%) 16 of group A (73%) and 17 of group B (59%). Results There was one early death from possible left anterior descending coronary artery distortion in group A and no deaths in group B. Two patients required early reoperation for systemic-to-pulmonary artery collateral ligation (postoperative day 6) and permanent pacemaker implantation (postoperative day 30). There were no late deaths. All 50 survivors are currently asymptomatic and in New York Heart Association class I. Three patients required late reoperations 36 months, 30 months, and 13 months after repair for (1) subaortic stenosis and dysfunctioning dysplastic mitral valve, (2) residual pulmonary artery branch stenosis, and (3) residual right ventricular outflow obstruction. Four patients underwent balloon dilation and stent insertion (1 patient) for peripheral pulmonary artery stenosis 1.5 year to 12 years (mean, 5 years) after initial repair. Actuarial freedom from need for reintervention at 4 years was 78.4% in group A and 85.7% in group B. Two-dimensional and Doppler echocardiographic follow-up studies showed a residual mild to moderate pulmonary artery branch stenosis in 4 patients in group A, and a recurrent subaortic stenosis in 1 patient in group A. Right ventricular peak systolic pressure was less than 40 mm Hg in all but 3 asymptomatic patients who had a residual pulmonary artery branch stenosis. Right ventricular end-systolic and end-diastolic volumes showed larger volumes and reduced ejection fraction in group A compared with group B. Conclusions This limited experience with repair of tetralogy of Fallot in patients less than 6 months of age demonstrates that the transatrial-transventricular approach is possible in neonates and young infants with a very low mortality and morbidity and also a low incidence of residual lesions. Follow-up echocardiographic data suggest that right ventricular function is better preserved in those patients who underwent the transatrial-transpulmonary repair.

Protein-losing enteropathy (PLE), defined as severe loss of serum protein into the intestine, occurs in 4% to 13% of patients after the Fontan procedure. We report a case of PLE reversal after heart transplantation in a 14-year-old boy with Fontan circulation who previously was treated unsuccessfully with medical therapy. The protein loss continued after heart transplantation. We administered total parenteral nutrition to rest the bowel. After 16 months, we observed a gradual decrease in protein loss. The patient is doing well 5 years after heart transplantation and had has a normal serum albumin level.

Background We discuss 4 cases of plastic bronchitis (PB) after Fontan procedure observed at our Institution, with the aim to identify an effective treatment and possible factors favoring the onset of PB. Plastic bronchitis is an uncommon disease characterized by recurrent formation of large pale bronchial casts obstructing the tracheobronchial tree. The treatment includes inhaled or systemic steroids, aerosolized mucolytics, bronchoscopic lavage, direct bronchoscopic extraction and, in few reports, aerosolized fibrinolytic and pulmonary vasodilators. Methods Four cases of PB after Fontan procedure occurred in our center from January 2008 to January 2012. Results All patients showed preserved ventricle function and underwent embolization of systemic-pulmonary collaterals prior to a Fontan procedure and all had chylothorax in the perioperative period. Two patients died; 1 of massive thrombosis of the superior vena cava and right pulmonary artery and the other of acute asphyxiation and refractory cardiac failure. Conclusions The exact causes of PB in Fontan patients remain unknown. An early diagnosis and a multiple therapy with steroids, mucolytics, pulmonary vasodilators, and aerosolized tissue plasminogen activator may be effective.

A total of 775 consecutive patients who survived the first 24 hours after cardiac operation were prospectively studied to assess the prevalence, mortality rate, and main risk factors for development of new acute renal failure. Normal renal function before operation (serum creatinine level less than 1.5 mg/dl) was registered in 734 (94.7 %) patients. Of these, 111 (15.1 %) showed a postoperative renal complication including 84 (11.4%) classified as renal dysfunction (serum creatinine level between 1.5 and 2.5 mg/dl) and 27 (3.7%) as acute renal failure (serum creatinine level higher than 2.5 mg/dl). The mortality rate was 0.8 % in normal patients, 9.5 % in patients with renal dysfunction, and 44.4 % when acute renal failure developed (p

An aortoventriculoplasty (Konno procedure) operation was performed for relief of tunnel-type subaortic stenosis using a Bjork-Shiley valve aortic prosthesis. The mechanical prosthesis thrombosed and cerebral embolism occurred when anticoagulant medication was stopped. The aortic root was successfully reconstructed with a cryopreserved aortic allograft using freehand implant technique. The cryopreserved aortic allograft is an excellent replacement device in the young adult patient in cases of failed prostheses, even in the presence of complex left ventricular outflow tract morphology or previous reconstruction operations.

Parallel advancements in surgical technique, preoperative and postoperative care, as well as a better understanding of physiology in patients with duct-dependent pulmonary or systemic circulation and a functional single ventricle, have led to superb results in staged palliation of most complex congenital heart disease (CHD) [1]. The Fontan procedure and its technical modifications have resulted in markedly improved outcomes of patients with single ventricle anatomy [2,3,4]. The improved early survival has led to an exponential increase of the proportion of Fontan patients surviving long into adolescence and young adulthood [5]. Improved early and late survival has not yet abolished late mortality secondary to myocardial failure, therefore increasing the referrals for cardiac transplantation [6]. Interstage attrition [7] is moreover expected in staged palliation towards completion of a Fontan-type circulation, while Fontan failure represents a growing indication for heart transplantation [8]. Heart transplantation has therefore become the potential “fourth stage” [9] or a possible alternative to a high-risk Fontan operation [10] in a strategy of staged palliation for single ventricle physiology. Heart transplant barely accounts for 16% of pediatric solid organ transplants [11]. The thirteenth official pediatric heart transplantation report- 2010 [11] indicates that pediatric recipients received only 12.5% of the total reported heart transplants worldwide. Congenital heart disease is not only the most common recipient diagnosis, but also the most powerful predictor of 1-year mortality after OHT. Results of orthotopic heart transplantations (OHT) for failing single ventricle physiology are mixed. Some authors advocate excellent early and mid-term survival after OHT for failing Fontan [9], while others suggest that rescue-OHT after failing Fontan seems unwarranted [10]. Moreover, OHT outcome appears to be different according to the surgical staging towards the Fontan operation and surgical technique of Fontan completion [12]. The focus of this report is a complete review of the recent literature on OHT for failing single ventricles, outlining the clinical issues affecting Fontan failure, OHT listing and OHT outcome. These data are endorsed reporting our experience with OHT for failing single ventricle physiology in recent years.

This case describes a young woman with a manifest left inferoparaseptal accessory pathway and previous history of surgical repair of a defect of the ventricular inlet septum in whom a transient complete AV block occurred during radiofrequency ablation performed from the coronary sinus. The presence of a preexisting surgery related AV block unmasked by anomalous pathway ablation is the more reliable explanation for this case.

Despite the remarkable advances with the use of ventricular assist devices (VAD) in adults, pneumatic pulsatile support in children is still limited. We report a retrospective review of our experience in very small children (10 kg of body weight).Ten consecutive children weighing less than 10 kg were offered mechanical support with Berlin Heart (Berlin Heart AG, Berlin, Germany) as a bridge to heart transplant from March 2002 to March 2010.The median patient age was 10.4 months (38 days to 2.2 years). The median patient weight was 6.4 kg (2.9 to 10 kg). Prior to VAD implantation, all children were managed by multiple intravenous inotropes and mechanical ventilation (8) or extracorporeal membrane oxygenation (2). The median pre-VAD pulmonary vascular resistance index was 5.7 Woods units/m(2). Three patients required biventricular mechanical support, but in all other cases a single left VAD proved sufficient. The median duration of VAD support was 61 days (2 to 168 days). Four deaths occurred; from stroke in three and sepsis in one. Five patients were successfully bridged to heart transplantation after a median duration of mechanical support of 89 days (37 to 168 days) and another is still waiting a suitable organ after 77 days of VAD support. There were no complications related to postoperative bleeding. Five patients required at least one pump change. Of 5 patients undergoing heart transplant, 3 developed an extremely elevated (60%) panel reactive antibody by enzyme-linked immunosorbent assay, confirmed by Luminex (Luminex Corp, Austin, TX). All 3 experienced at least one acute episode of rejection in the first month after heart transplant, needing plasmapheresis. The survival rate after heart transplantation was 100% with a median follow-up of 7.5 months.Mechanical support in very small children with end-stage heart failure is an effective strategy of bridge to heart transplantation with a reasonable mortality rate. The high rate of complications suggests to optimize indications and timing of VAD implantation.

OBJECTIVES We undertook to determine the influence of perfusion pressure during hypothermic cardiopulmonary bypass (CPB) on cerebral blood flow (CBF) and cognitive memory outcome at 6 months postoperatively. METHODS Nineteen patients who underwent hypothermic nonpulsatile CPB for elective coronary artery bypass (CAB) surgery were evaluated by (133)Xe measurement of the CBF and by the Incidental Memory Assessment for evaluating cognitive memory (IMTscore), both at baseline before the operation (T(1)) and again at 5 to 6 months postoperatively (T(2)). RESULTS Overall, the mean CBF fell significantly from 39 +/- 5 mL.(100 g)(-1).min(-1) at T(1) to 33 +/- 3 mL.(100 g)(-1).min(-1) at T(2) (P < .001). The decrease in CBF from T(1) to T(2) (DeltaCBF(2-1)) correlated with a significant reduction in the IMTscore from T1 to T2 (DeltaIMTscore(2-1)) (P < .001) and with a mean arterial pressure during CPB (MAPCPB) of

We describe the case of a newborn with isolated noncompaction of the ventricular myocardium, congenital atrioventricular block, and prolonged QT, who developed a dilated cardiomyopathy after 4 months of right ventricular permanent pacing. The implanted system was upgraded to biventricular pacing. Resynchronization therapy was associated with normalized septal motion and shortening of the interventricular delay and, within 2 months after initiation, resulted in markedly improved left ventricular ejection fraction and reduced ventricular volume.

Objective We report our experience with repair of tetralogy of Fallot associated with complete atrioventricular septal defect, addressing in particular the need for a pulmonary valve in the right ventricular outflow tract. Methods Between 1992 and 2006, 33 children with tetralogy of Fallot and complete atrioventricular septal defect were admitted; 26 had Down's syndrome (79%). Thirty-two children had complete repair (18 primary, 14 staged); of the 15 who received initial palliation, 1 died before complete repair. Right ventricular outflow tract obstruction was relieved by transannular patch in 14 cases (42%), infundibular patch with preservation of the pulmonary valve in 7 (21%), and right ventricle–to–pulmonary artery conduit in 11 (33%). Results There were no hospital deaths. Actuarial survival was 96% ± 3.9% at 5 years and 85.9 ± 1.1% at 10 years. Multivariate analysis showed that type of relief of right ventricular outflow tract obstruction did not influence survival ( P = .16), nor did the choice to use a valved conduit ( P = .82). Primary correction ( P = .05) and lower weight at repair ( P = .05) were associated with higher probability of survival. Mean follow-up was 69.3 ± 5.9 months (range 0.2–282 months). There were 2 late deaths. Overall freedom from reoperation was 69% at 5 years and 38% at 10 years. Right ventricular outflow tract reconstruction without use of a valved conduit allowed a significantly higher freedom from reinterventions ( P Conclusions Tetralogy of Fallot associated with complete atrioventricular septal defect can be corrected at low risk with favorable intermediate survival. Use of right ventricle–to–pulmonary artery conduit can be avoided in two thirds of patients with no impact on survival, possibly improving overall freedom from reintervention.

Cardiac surgery with cardiopulmonary bypass (CPB) elicits an inflammatory response and has a multitude of biological consequences, ranging from subclinical organ dysfunction to severe multiorgan failure. Pediatric patients are more prone to have a reaction that can jeopardize their outcome. Cytokines are supposed to be important mediators in this response: limiting their circulating levels is, therefore, appealing. We investigated the pattern of cytokine release during pediatric operation for congenital heart anomalies in 20 patients, and the effect of hemofiltration. Tumor necrosis factor a (TNF-α) was elevated after anesthesia induction and showed significant decrease during CPB. Hemofiltration reduced its concentration, but the effect disappeared on the following day. Interleukin-1 (IL-1) increased slowly at the end of CPB and hemofiltration had no effect. Interleukin-6 (IL-6) showed a tendency toward augmentation during rewarming and hemofiltration did not significantly affect the course. Soluble interleukin-6 receptor (sIL-6r) had a pattern similar to TNF-α, but hemofiltration had no effect. On the other hand, interleukin-8 (IL-8) behaved like IL-6. Our findings suggest that baseline clinical status, anesthetic drugs, and maneuvers before incision may elicit a cytokine response, whereas rewarming is a critical phase of CPB. Hemofiltration is effective in removal of TNF-α, but its role is debatable for the control of IL-1, IL-6, sIL-6r and IL-8 levels.

Background: To evaluate the plasmatic changes of atrial natriuretic factor (ANF) during and after cardiopulmonary bypass (CPB) in normothermia and hypothermia. Methods: Twenty-three patients (n=23) undergoing coronary artery bypass graft surgery were randomly assigned to two groups. In Group I (n=11), the patients underwent operation in normothermia; in Group II (n=12), the operation was performed in hypothermia (268C). Results: Plasma ANF levels were determined after induction of anaesthesia, at the end of CPB and one hour postoperatively. There were no demographic differences between the two groups, diuresis (p=0.90) and natriuresis (p=0.95). Plasma levels of ANF were significantly elevated during and after CPB in both groups (p Conclusion: CPB triggers the production and release of ANF. The present study demonstrates a significantly enhanced ANF release during hypothermia and reperfusion after ischaemia. Thus, these data suggest the protective role of ANF on the hypoxic myocardium, and they confirm that ANF does not play a role in diuresis and natriuresis during and after hypothermic CPB.

Objective: Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for failing single ventricle physiology. Methods: Between 1988 and 2002, 25 patients (mean age 9.3 ^ 7.1 years) underwent OHT for complex congenital heart disease (CHD) with a functional right (15 patients) or left (10 patients) single ventricle. Palliative staging towards Fontan completion had been previously accomplished in 22 patients (88%). Transition to OHT occurred from a shunt stage in 10, a bi-directional cavopulmonary anastomosis (BDG) stage in nine, and after Fontan failure in six patients. Results: Thirty-day survival was 68.0 ^ 9.3% with no additional mortality up to 14.1 years. OHT following BDG staging exhibited 100% long-term survival, as opposed to 66.7 ^ 15.7% for OHT after systemic-to-pulmonary shunt, and 33.3 ^ 19.2% for OHT following failing Fontan ðp ¼ 0:032Þ. Regression logistic modelling indicated failing Fontan circulation as predictor of higher mortality after OHT ðp ¼ 0:041Þ. Reintervention was necessary in four patients 40 ^ 11 months after OHT to address residual superior vena cava (two) and isthmic (two) stenosis. Overall freedom from reintervention was 88.3 ^ 8.1% at 5 years. Conclusions: OHT for structural CHD with single ventricle physiology entails substantial early mortality while BDG enables the best transition to heart transplant. OHT should be considered in the decision-making process as an alternative to Fontan completion in high-risk candidates, since rescue-OHT after failing Fontan seems unwarranted. q 2003 Elsevier B.V. All rights reserved.

Objective— Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for congenital heart disease. Methods— Between 1988 and 2002, 43 patients (mean age 9.1±7.2 years) underwent 44 OHT for complex TGA (6), DORV (4), single ventricle (21), and other end-stage structural heart disease (11). Two discernible ventricular chambers were present in 18 pts (41.8%). Previous reconstructive or palliative procedures had been previously accomplished in 35 pts (83.3%), including atrial switch (5), systemic-to-pulmonary shunts (10), cavopulmonary anastomosis (9), Fontan completion (6), and others (5). Results— 30-day survival for the 2-ventricle subgroup was 94.4±5.4% compared with 67.2±9.5% for the single ventricle subgroup ( P =0.04) (overall 78.6%±3.3%). OHT following single ventricle staging to bi-directional cavopulmonary anastomosis exhibited 100% early survival, as opposed to 62.5±17.1% for OHT after systemic-to-pulmonary shunts, and 33.3±19.2% for OHT following failing Fontan ( P =0.010). HLHS diagnosis (0.0085) and failing Fontan ( P =0.003) were identified as independent predictors of early mortality by regression logistic modeling, while Fontan stage represented the only predictor of overall mortality by Cox proportional hazard. Overall 10-year survival was 54.3±11%. Conclusions— OHT for structural congenital heart disease with single ventricle physiology entails substantial early mortality and bi-directional cavopulmonary anastomosis enables the best transition to heart transplant. OHT should be considered in the decision making process as an alternative to Fontan completion in high-risk candidates, since rescue-OHT after failing Fontan seems unwarranted.