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Open in new tab Download slide Open in new tab Download slide 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Hypertension and reduced HRV are frequent in heart-transplanted recipients. We studied 26 young recipients to investigate the relationship between BP and HRV during simultaneous 24-h monitoring. Presence of CAV was considered. All HRV measures were significantly lower than normal values. Significant correlations were found between mean daytime systolic BP and the rMSSD (p = 0.04), and mean daytime DBP and SDANN for all 5-min segments (p = 0.03) and between rMSSD and mean nighttime DBP (p = 0.03). Four patients were hypertensive during daytime, seven had a reduced nocturnal fall and two had a nocturnal rise in BP. Eight patients showed severe CAV grade (grade IV) on the Stanford scale, 13 moderate (grade III) and five mild (grade I-II). After a follow-up time of 30 months, four of the 13 patients (30%) with CAV grade III showed an increase to grade IV and all showed abnormalities of both HRV and ABPM patterns. The relationship between HRV abnormalities and arterial hypertension and CAV should be further explored. [ABSTRACT FROM AUTHOR]

Summary: 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. [Copyright &y& Elsevier]

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 (26°C). 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 < 0.01). The groups differed significantly for plasma levels of ANF during CPB and postoperatively (p < 0.05), but did not differ prebypass (p = 0.08). There was no correlation in either group between ANF release and central venous pressure, natriuresis and diuresis. There was only a borderline relationship between ANF concentration and diuresis after CPB in Group I. 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. [ABSTRACT FROM AUTHOR]

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 (p=0.032). Regression logistic modelling indicated failing Fontan circulation as predictor of higher mortality after OHT (p=0.041). 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. [Copyright &y& Elsevier]

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=0.008) and reoperation for intrinsic PV stenosis (P=0.034) as independent predictors of mortality. According to logistic analysis, preoperative obstruction predicts higher risk of reintervention for intrinsic PV stenosis (P=0.022), while the double-patch technique increased the risk of late arrhythmias (P=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. [Copyright &y& Elsevier]

Despite the remarkable advances with the use of ventricular assist devices (VAD) in adults, pneumatic pulsatile support in children is still limited. We report on our experience in the pediatric population. A retrospective review of 17 consecutive children offered mechanical support with Berlin Heart as a bridge to heart transplant from February 2002 to April 2010 was conducted. The median patient age was 3.9 years (75 days to 13.3 years). The median patient weight was 14.1 kg (2.9-43 kg). Before VAD implantation, all children were managed by multiple intravenous inotropes and mechanical ventilation (14) or extracorporeal membrane oxygenation (3). All patients had right ventricular dysfunction. Nine patients required biventricular mechanical support (BVAD), but in all other cases a single left ventricular assist device proved sufficient (47%). The median duration of VAD support was 47 days (1-168 days). The median pre-VAD pulmonary vascular resistance index (Rpi) was 5.7 WU/m (3.5 to 14.4 WU/m). Eleven patients (65%) were successfully bridged to heart transplantation after a median duration of mechanical support of 68 days (6-168 days). Six deaths occurred (35%), three for neurological complications, one for sepsis, and two others for device malfunctioning. Since 2007, the survival rate of our patients has increased from 43% to 80%, and the need for BVAD has decreased from 86% to 30%. In two patients with Rpi >10 WU/m, unresponsive to pulmonary vasodilatator therapy, Rpi dropped to 2.2 and 2 WU/m after 40 and 23 days of BVAD support, respectively. Seven patients (41%) required at least one pump change. Of 11 patients undergoing heart transplant, four developed an extremely elevated (>60%) panel reactive antibody by enzyme-linked immunosorbent assay, confirmed by Luminex. All of them 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 25.4 months (6 days to 7.7 years). Mechanical support in children with end-stage heart failure is an effective strategy as a bridge to heart transplantation with a reasonable morbidity and mortality. BVAD support may offer an additional means to reverse extremely elevated pulmonary vascular resistance. [ABSTRACT FROM AUTHOR]

Pulmonary regurgitation (PR) and right ventricular (RV) dilatation and disfunction are common in patients with repaired Tetralogy of Fallot (r-TOF). 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 (VO 2). 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 <.0001, r = 0.72). RV end-diastolic area > 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 VO 2. Among the Echo data only right atrial strain (RAS) correlated with peak VO 2. 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 VO 2 and should be added in their follow up. • In children with repaired Tetralogy of Fallot RV area by echocardiogram is a valid first line parameter to screen RV dilation as compared with CMR. • Right ventricle longitudinal systolic dysfunction coexists with a still preserved RV EF. • Flow-reversal in pulmonary branches identifies PR RF ≥ 35% at CMR. • The only parameter correlated to peak Vo2 max in children with r-TOF is right atrial strain [ABSTRACT FROM AUTHOR]

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. 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). 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). 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. • Hypoplastic left heart syndrome still carries high mortality and morbidity. • Systemic right ventricle dysfunction is one of the major causes of the poor outcome. • Speckle tracking echocardiography is accurate in right ventricular function assessment. • Serial evaluations may precociously identify patients with poor prognosis. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Abstract 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. Highlights • Accurate risk stratification of Ebstein's anomaly (EA) patients is crucial. • Echocardiographic parameters may help in predicting outcome in EA patients, • Speckle tracking echo should be a regular part in the evaluation of EA children, [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [Copyright &y& Elsevier]

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 α (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. [ABSTRACT FROM AUTHOR]

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. [Copyright &y& Elsevier]