Impact of early postoperative haemodynamic and laboratory parameters on outcome after the Fontan procedure.

OBJECTIVE: To identify early postoperative haemodynamic and laboratory parameters predicting outcomes following total cavopulmonary connection. METHODS: Patients who underwent total cavopulmonary connection between 2012 and 2021 were evaluated. Serial values of mean pulmonary artery pressure, mean arterial pressure, peripheral oxygen saturation, and lactate levels were collected. The influence of these variables on morbidities was analyzed. Cut-off values were calculated using the receiver operating characteristic analysis. RESULTS: A total of 249 patients were included. All patients had previous bidirectional cavopulmonary shunt. Median age and weight at total cavopulmonary connection were 2.2 (1.8-2.7) years and 11.7 (10.7-13.4) kg, respectively. All patients were extubated in the ICU at a median of 3 (2-5) hours after ICU admission. Postoperative pulmonary artery pressure, around 12 hours after extubation, was significantly associated with chest tube drainage (p = 0.048), chylothorax (p = 0.021), ascites (p = 0.016), and adverse events (p = 0.028). Receiver operating characteristic analysis revealed a cut-off value of 13-15 mmHg for chest tube drainage and chylothorax and 17 mmHg for ascites and adverse events. Mean arterial pressure 1 hour after extubation was associated with prolonged chest tube drainage (p = 0.015) and adverse events (p = 0.008). Peripheral oxygen saturation 6 hours after extubation (p = 0.003) was associated with chest tube duration and peripheral oxygen saturation 1 hour after extubation (p < 0.001) was associated with ascites. Lactate levels on 2nd postoperative day (p = 0.022) were associated with ascites and lactate levels on 1st postoperative day (p = 0.009) were associated with adverse events. CONCLUSIONS: Higher pulmonary artery pressure, lower mean arterial pressure, lower peripheral oxygen saturation, and higher lactate in early postoperative period, around 12 hours after extubation, predicted in-hospital and post-discharge adverse events following total cavopulmonary connection.

Bioactive adrenomedullin (bio-ADM) is associated with endothelial dysfunction in infants and children with complex congenital heart disease undergoing open-heart surgery on cardiopulmonary bypass.

OBJECTIVES: Children with congenital heart disease (CHD) undergoing cardiac surgery on cardiopulmonary bypass (CPB) are at risk for systemic inflammation leading to endothelial dysfunction associated with increased morbidity. Bioactive adrenomedullin (bio-ADM) is a peptide regulating vascular tone and endothelial permeability. The aim of this study was to evaluate the dynamics of plasma bio-ADM in this patient cohort and its role in capillary leak. METHODS: Plasma samples from 73 pediatric CHD patients were collected for bio-ADM measurement at five different timepoints (TP) in the pre-, intra-, and post-operative period. The primary endpoint was a net increase in bio-ADM levels after surgery on CPB. Secondary endpoints included association of bio-ADM levels with clinical signs for endothelial dysfunction. RESULTS: Bio-ADM levels increased after surgery on CPB from pre-operative median of 12 pg/mL (IQR [interquartile range] 12.0-14.8 pg/mL) to a maximum post-operative median of 48.8 pg/mL (IQR 34.5-69.6 pg/mL, p<0.001). Bio-ADM concentrations correlated positively with post-operative volume balance, (r=0.341; p=0.005), increased demand for vasoactive medication (duration: r=0.415; p<0.001; quantity: TP3: r=0.415, p<0.001; TP4: r=0.414, p<0.001), and hydrocortisone treatment for vasoplegia (bio-ADM median [IQR]:129.1 [55.4-139.2] pg/mL vs. 37.9 [25.2-64.6] pg/mL; p=0.034). Patients who required pleural effusion drainage revealed higher bio-ADM levels compared to those who did not (median [IQR]: 66.4 [55.4-90.9] pg/mL vs. 40.2 [28.2-57.0] pg/mL; p<0.001). CONCLUSIONS: Bio-ADM is elevated in children after cardiac surgery and higher levels correlate with clinical signs of capillary leakage. The peptide should be considered as biomarker for endothelial dysfunction and as potential therapeutic target in this indication.

Apixaban for Prevention of Thromboembolism in Pediatric Heart Disease.

BACKGROUND: Children with heart disease frequently require anticoagulation for thromboprophylaxis. Current standard of care (SOC), vitamin K antagonists or low-molecular-weight heparin, has significant disadvantages. OBJECTIVES: The authors sought to describe safety, pharmacokinetics (PK), pharmacodynamics, and efficacy of apixaban, an oral, direct factor Xa inhibitor, for prevention of thromboembolism in children with congenital or acquired heart disease. METHODS: Phase 2, open-label trial in children (ages, 28 days to <18 years) with heart disease requiring thromboprophylaxis. Randomization 2:1 apixaban or SOC for 1 year with intention-to-treat analysis. PRIMARY ENDPOINT: a composite of adjudicated major or clinically relevant nonmajor bleeding. Secondary endpoints: PK, pharmacodynamics, quality of life, and exploration of efficacy. RESULTS: From 2017 to 2021, 192 participants were randomized, 129 apixaban and 63 SOC. Diagnoses included single ventricle (74%), Kawasaki disease (14%), and other heart disease (12%). One apixaban participant (0.8%) and 3 with SOC (4.8%) had major or clinically relevant nonmajor bleeding (% difference -4.0 [95% CI: -12.8 to 0.8]). Apixaban incidence rate for all bleeding events was nearly twice the rate of SOC (100.0 vs 58.2 per 100 person-years), driven by 12 participants with ≥4 minor bleeding events. No thromboembolic events or deaths occurred in either arm. Apixaban pediatric PK steady-state exposures were consistent with adult levels. CONCLUSIONS: In this pediatric multinational, randomized trial, bleeding and thromboembolism were infrequent on apixaban and SOC. Apixaban PK data correlated well with adult trials that demonstrated efficacy. These results support the use of apixaban as an alternative to SOC for thromboprophylaxis in pediatric heart disease. (A Study of the Safety and Pharmacokinetics of Apixaban Versus Vitamin K Antagonist [VKA] or Low Molecular Weight Heparin [LMWH] in Pediatric Subjects With Congenital or Acquired Heart Disease Requiring Anticoagulation; NCT02981472).

Clinical and haemodynamic variables associated with intensive care unit length of stay and early adverse outcomes after the Norwood procedure.

OBJECTIVES: This study was performed to determine the clinical and haemodynamic variables associated with early adverse outcomes after the neonatal Norwood procedure. METHODS: Patients who underwent the neonatal Norwood procedure between 2001 and 2019 were included. The patient diagnosis, morphological characteristics and haemodynamic parameters were analysed to identify factors associated with length of stay (LOS) in the intensive care unit (ICU) and mortality during the stay. RESULTS: A total of 322 patients were depicted. The median age and weight at the Norwood procedure were 9 days and 3.2 kg, respectively. Certain morphological and preoperative parameters, such as birth weight below 2.5 kg, restrictive atrial septal defect, extracardiac anomalies and the diameter of the ascending aorta, were found to be associated with the LOS in the ICU. Analysis using early postoperative haemodynamic variables revealed that systolic arterial pressure, diastolic arterial pressure, serum lactate levels and reduced ventricular function at 2 days postoperatively were associated with the LOS in the ICU. Birth weight <2.5 kg (P = 0.010), a restrictive atrial septal defect (P = 0.001) and smaller ascending aorta (P = 0.039) were associated with death in the ICU. Reduced ventricular function, lower systolic aortic pressure and higher lactate levels at various time points (P < 0.05) were also associated with ICU deaths. The LOS in the ICU was significantly associated with late mortality (P < 0.001, Hazard Ratio (HR) = 1.015). CONCLUSIONS: The LOS in the ICU after the Norwood procedure was predicted by early postoperative haemodynamic variables, suggesting that good early postoperative haemodynamics determine early recovery. A prolonged stay in the ICU after the Norwood procedure was associated with late mortality.

Factors influencing length of intensive care unit stay following a bidirectional cavopulmonary shunt.

OBJECTIVES: The goal of this study was to identify the risk factors for prolonged length of stay (LOS) in the intensive care unit (ICU) after a bidirectional cavopulmonary shunt (BCPS) procedure and its impact on the number of deaths. METHODS: In total, 556 patients who underwent BCPS between January 1998 and December 2019 were included in the study. RESULTS: Eighteen patients died while in the ICU, and 35 died after discharge from the ICU. Reduced ventricular function was significantly associated with death during the ICU stay (P = 0.002). In patients who were discharged alive from the ICU, LOS in the ICU [hazard ratio (HR) 1.04, 95% confidence interval (CI) 1.02-1.06; P < 0.001] and a dominant right ventricle (HR 2.41, 95% CI 1.03-6.63; P = 0.04) were independent risk factors for death. Receiver operating characteristic analysis identified a cut-off value for length of ICU stay of 19 days. Mean pulmonary artery pressure (HR 1.03, 95% CI 1.01-1.05; P = 0.04) was a significant risk factor for a prolonged ICU stay. CONCLUSIONS: Prolonged LOS in the ICU with a cut-off value of 19 days after BCPS was a significant risk factor for mortality. High pulmonary artery pressure at BCPS was a significant risk factor for a prolonged ICU stay.

Early extubation improves outcome following extracardiac total cavopulmonary connection.

OBJECTIVES: The aim of this study was to investigate the impact of an early extubation strategy on the outcome following extracardiac total cavopulmonary connection. METHODS: From 1999 through 2017, 458 patients underwent extracardiac total cavopulmonary connection; 257 (56%) patients were managed with an early extubation strategy adopted in 2009 (group A). Their outcome was compared with those of 201 (44%) patients treated before 2009 (group B). In group A, the outcome of unstable patients, defined as >75th percentile for volume administered and inotrope scores, was compared with those of stable patients. RESULTS: Ventilation time (median: 4 h vs 16 h, P < 0.001), fluid volume administered during the first 24 h (mean: 110 ml/kg vs 164 ml/kg, P = 0.003), chest tube duration (median: 3 days vs 4 days, P = 0.028) and length of intensive care unit stay (median: 6 days vs 7 days, P = 0.001) were less in group A than in group B. The reintubation rate (7% vs 6%, P = 0.547) and early mortality (0.8% vs 1.5%, P = 0.465) were similar between groups. The 80 unstable group A patients received more inotropic support (P < 0.001) and fluid volume (P < 0.001) than stable patients, but the ventilation time (6 h vs 5 h, P = 0.220), the reintubation rate (10% vs 6%, P = 0.283) and the length of intensive care unit stay (7 days vs 6 days, P = 0.590) were similar. In unstable patients, mean arterial pressure before extubation was significantly lower than stable patients (P = 0.001). However, mean arterial pressure in unstable patients increased significantly (P < 0.001) soon after extubation, and became similar to the value in stable patients. CONCLUSIONS: Early extubation following extracardiac total cavopulmonary connection improves postoperative haemodynamics and recovery regardless of the initial haemodynamic status.

Predicted clinical factors associated with the intensive care unit length of stay after total cavopulmonary connection.

OBJECTIVES: A longer length of stay (LOS) in the intensive care unit (ICU) after the total cavopulmonary connection (TCPC) is thought to be a predictive sign of late Fontan failure. This study was performed to determine the clinical risk factors for ICU LOS. METHODS: In total, 483 patients who underwent a TCPC between May 1994 and December 2016 were included the study. Patients' main diagnosis, morphologic characteristics, palliative procedures, hemodynamic parameters, and perioperative variables, were analyzed to identify risk factors influencing ICU stay based on Cox regression. Causes of longer ICU LOS and the impact of ICU LOS on late outcomes were evaluated. RESULTS: Age at TCPC, type of TCPC, and fenestration at TCPC did not affect the ICU LOS. With multivariable model, hypoplastic left heart syndrome (P = .001) and anomalous systemic venous drainage (P < .001) were identified as independent morphologic risk factors for prolonged ICU LOS. Of hemodynamic variables, preoperative high transpulmonary gradient (P = .037), and low aortic oxygen saturation (P = .031) were risks for longer ICU LOS. Of postoperative variables, pleural effusion (P < .001), chylothorax (P = .001), ascites (P < .001), and infection (P = .028) were risks for longer ICU LOS. The ICU LOS was found to be significantly associated with late mortality (P < .001) and late cardiac reoperation (P = .007). CONCLUSIONS: Patients with hypoplastic left heart syndrome and anomalous systemic venous drainage had longer ICU LOS. Extended cyanosis and elevated pulmonary artery pressure affect the ICU LOS. Special care should be provided during the initial postoperative phase in patients with such risk factors.

Neutrophil gelatinase-associated lipocalin reflects inflammation and is not a reliable renal biomarker in neonates and infants after cardiopulmonary bypass: a prospective case-control study.

Introduction Acute kidney injury is a frequent complication after cardiac surgery with cardiopulmonary bypass in infants. Neutrophil gelatinase-associated lipocalin has been suggested to be a promising early biomarker of impending acute kidney injury. On the other hand, neutrophil gelatinase-associated lipocalin has been shown to be elevated in systemic inflammatory diseases without renal impairment. In this secondary analysis of data from our previous study on acute kidney injury after infant cardiac surgery, our hypothesis was that neutrophil gelatinase-associated lipocalin may be associated with surgery-related inflammation. METHODS: We prospectively enrolled 59 neonates and infants undergoing cardiopulmonary bypass surgery for CHD and measured neutrophil gelatinase-associated lipocalin in plasma and urine and interleukin-6 in the plasma. Values were correlated with postoperative acute kidney injury according to the paediatric Renal-Injury-Failure-Loss-Endstage classification. RESULTS: Overall, 48% (28/59) of patients developed acute kidney injury. Of these, 50% (14/28) were classified as injury and 11% (3/28) received renal replacement therapy. Both plasma and urinary neutrophil gelatinase-associated lipocalin values were not correlated with acute kidney injury occurrence. Plasma neutrophil gelatinase-associated lipocalin showed a strong correlation with interleukin-6. Urinary neutrophil gelatinase-associated lipocalin values correlated with cardiopulmonary bypass time. CONCLUSION: Our results suggest that plasma and urinary neutrophil gelatinase-associated lipocalin values are not reliable indicators of impending acute kidney injury in neonates and infants after cardiac surgery with cardiopulmonary bypass. Inflammation may have a major impact on plasma neutrophil gelatinase-associated lipocalin values in infant cardiac surgery. Urinary neutrophil gelatinase-associated lipocalin may add little prognostic value over cardiopulmonary bypass time.

Early postoperative extubation of unstable patients following total cavopulmonary connection: impact on circulation and outcome.

OBJECTIVES: We aimed to investigate whether early postoperative extubation following the Fontan operation is universally feasible and can be used as a management tool in unstable patients. METHODS: All patients undergoing the Fontan operation in our centre between 2004 and 2013 (n=253) were analysed. Until 2008, patients were extubated according to standard criteria and comprised group 1. Group 2 included all patients presenting after 2009, when early extubation was always aimed regardless of the haemodynamic status. Patients who exceeded the 75th percentiles for volume requirements and inotrope scores for the respective group were defined as unstable. Comparisons of outcomes between groups and subgroups and analysis of the changes in haemodynamic and treatment parameters with extubation in unstable patients after 2009 were performed. RESULTS: Compared with group 1, patients from group 2 were ventilated for shorter duration (p<0.001), had similar re-intubation rates (p=0.50), and needed less volume (p=0.01). In group 2, the unstable patients were not ventilated for longer durations (p=0.19), but had higher re-intubation rates (p=0.03) than the stable patients. Compared with the unstable patients from group 1, the unstable patients from group 2 were ventilated for shorter duration (p<0.001), had similar re-intubation rates (p=0.66), and needed less volume (p=0.006). There was a significant acute and sustained increase in mean arterial pressure with extubation and a parallel reduction in volume requirements and inotrope scores in the unstable patients from group 2. CONCLUSIONS: Timely extubation is universally applicable following the Fontan operation. Early postoperative extubation can be valuable for improving Fontan haemodynamics.

Intraoperative renal near-infrared spectroscopy indicates developing acute kidney injury in infants undergoing cardiac surgery with cardiopulmonary bypass: a case-control study.

INTRODUCTION: Acute kidney injury (AKI) is a frequent complication after cardiac surgery with cardiopulmonary bypass in infants. Renal near-infrared spectroscopy (NIRS) is used to evaluate regional oximetry in a non-invasive continuous real-time fashion, and reflects tissue perfusion. The aim of this study was to evaluate the relationship between renal oximetry and development of AKI in the operative and post-operative setting in infants undergoing cardiopulmonary bypass surgery. METHODS: In this prospective study, we enrolled 59 infants undergoing cardiopulmonary bypass surgery for congenital heart disease for univentricular (n = 26) or biventricular (n = 33) repair. Renal NIRS was continuously measured intraoperatively and for at least 24 hours postoperatively and analysed for the intraoperative and first 12 hours, first 24 hours and first 48 hours postoperatively. The renal oximetry values were correlated with the paediatric risk, injury, failure, loss, end (pRIFLE) classification for AKI, renal biomarkers and the postoperative course. RESULTS: Twenty-eight (48%) infants developed AKI based on pRIFLE classification. Already during intraoperative renal oximetry and further in the first 12 hours, 24 hours and 48 hours postoperatively, significantly lower renal oximetry values in AKI patients compared with patients with normal renal function were recorded (P < 0.05). Of the 28 patients who developed AKI, 3 (11%) needed renal replacement therapy and 2 (7%) died. In the non-AKI group, no deaths occurred. Infants with decreased renal oximetry values developed significantly higher lactate levels 24 hours after surgery. Cystatin C was a late parameter of AKI, and neutrophil gelatinase-associated lipocalin values were not correlated with AKI occurrence. CONCLUSION: Our results suggest that prolonged low renal oximetry values during cardiac surgery correlate with the development of AKI and may be superior to conventional biochemical markers. Renal NIRS might be a promising non-invasive tool of multimodal monitoring of kidney function and developing AKI in infants undergoing cardiac surgery with cardiopulmonary bypass.

A Comparative Histopathological Study of Heparin Coated and Uncoated Polytetrafluoroethylene Shunts in Children With Congenital Heart Defect.

OBJECTIVE: Recently, heparin coated polytetrafluoroethylene (PTFE) shunts are available and are believed to improve inherent shunt problems such as thrombosis and excessive and incomplete neointima formation or occlusion. We aimed at comparing the potential histopathological differences in the neointima (in) between uncoated (UCS) PTFE shunts and heparin coated (HCS) PTFE shunts. MATERIALS AND METHODS: Thirteen shunts (six UCS and seven HCS) were analyzed. The specimens were fixed in formalin, embedded in paraffin or in methylmethacrylate, and characterized by standard and immunohistochemical staining. The thickness of pseudointima proliferation was graded as follows: 0 = no cell layers, 1 = few layers <100 µm, 2 = partial layers >100 µm, 3 = complete layers <300 µm, 4 = complete layers >300 µm, and 5 = occlusion. RESULTS: Mean shunt size was 3.4 ± 0.2 mm in UCS and 3.1 ± 0.2 mm in HCS (P = .053). Mean time of implantation was 163 ± 75 days in UCS and 97 ± 52 days in HCS (P = .091). There were no significant differences in the proportion of patients with functionally single ventricle, body surface area, age at implantation, or implantation type, between both groups. Shunt occlusion did not occur. Unplanned shunt explantation due to cyanosis was performed in one patient in each group. Partial thrombus formation was observed in one UCS (P = .462). There was complete endothelialization in 50% of UCS and 86% of HCS (P = .266). The grade of pseudointima proliferation was 1.8 ± 0.4 in UCS and 1.7 ± 0.5 in HCS (P = .646). CONCLUSIONS: The histopathological workup of PTFE shunts revealed equally partial endothelialization and discrete pseudointima proliferation in both the groups. The process of endothelialization may be faster in HCS.

Continuous infusion of clonidine in ventilated newborns and infants: a randomized controlled trial.

OBJECTIVES: To assess the influence of an infusion of clonidine 1 µg/kg/hr on fentanyl and midazolam requirement in ventilated newborns and infants. DESIGN: Prospective, double-blind, randomized controlled multicenter trial. Controlled trials.com/ISRCTN77772144. SETTING: Twenty-eight level 3 German PICUs/neonatal ICUs. PATIENTS: Ventilated newborns and infants: stratum I (1-28 d), stratum II, (29-120 d), and stratum III (121 d to 2 yr). INTERVENTIONS: Patients received clonidine 1 µg/kg/hr or placebo on day 4 after intubation. Fentanyl and midazolam were adjusted to achieve a defined level of analgesia and sedation according to Hartwig score. MEASUREMENTS AND MAIN RESULTS: Two hundred nineteen infants were randomized; 212 received study medication, 69.7% were ventilated in the postoperative care and 30.3% for other reasons. Primary endpoint: consumption of fentanyl and midazolam in the 72 hours following the onset of study medication (main observation period) in the overall study population. The confirmatory analysis of the overall population showed no difference in the consumption of fentanyl and midazolam. Explorative age-stratified analysis demonstrated that in stratum I (n = 112) the clonidine group had a significantly lower consumption of fentanyl (clonidine: 2.1 ± 1.8 µg/kg/hr, placebo: 3.2 ± 3.1 µg/kg/hr; p = 0.032) and midazolam (clonidine: 113.0 ± 100.1 µg/kg/hr, placebo: 180.2 ± 204.0 µg/kg/hr; p = 0.030). Strata II (n = 43) and III (n = 46) showed no statistical difference. Sedation and withdrawal-scores were significantly lower in the clonidine group of stratum I (p < 0.001). Frequency of severe adverse events did not differ between groups. CONCLUSIONS: Clonidine 1 µg/kg/hr in ventilated newborns reduced fentanyl and midazolam demand with deeper levels of analgesia and sedation without substantial side effects. This was not demonstrated in older infants, possibly due to lower clonidine serum levels.

Two consecutive open heart operations in a small child with heparin-induced thrombocytopenia type II using anticoagulation with heparin and tirofiban.

We report the use of the platelet glycoprotein IIb/IIIa antagonist tirofiban for cardiac surgery in a small child with active heparin-induced thrombocytopenia type II, including description of the postoperative course.

Neonatal balloon aortic valvuloplasty-predictive value of current risk score algorithms for treatment strategies.

BACKGROUND: Neonatal valvular aortic stenosis (AoS) represents a spectrum of different degrees of hypoplasia and malformation of all left heart structures. Uncertainty exists on threshold values for biventricular circulation of newborns with critical AoS. Our aim was to assess the predictive value of current risk scores for treatment strategies in critical AoS. METHODS AND RESULTS: The echocardiograms of all newborns with AoS treated by balloon valvuloplasty (AoVP) or Norwood operation between January 2006 and September 2008 were reviewed retrospectively and the Rhodes-, Colan-score and the univentricular repair survival advantage (UVR-SA) tool were applied. The results were compared to the actual outcome. Out of 28 patients 19 were treated by an initial AoVP and nine by an initial Norwood operation. In three a secondary Norwood operation was done. According to the Rhodes-score 24 patients should have been treated by a univentricular strategy but 12 of them (50%) live with biventricular circulation. The Colan-score resulted in 19 univentricular decisions and 7 (37%) of these patients now live with biventricular circulation. Applying the UVR-SA tool 2/12 (17%) patients predicted for a univentricular strategy received successful biventricular circulation and 2/16 (12%) of the suggested biventricular patients have a univentricular circulation. Hence, 14/28 (50%) patients had discordant treatment decisions. CONCLUSION: No prospectively tested criteria for patient selection (biventricular vs. univentricular) are available for critically ill newborns with AoS. Retrospective application of the current risk scores showed unsatisfactory results. Treatment decisions are based on local experience and expertise.

Transcutaneous Melody valve implantation in "tricuspid position" after a Fontan Björk (RA-RV homograft) operation results in biventricular circulation.

A modified Fontan operation (Fontan Björk [Bjork VO, Olin CL, Bjarke BB, Thoren CA. Right atrial-right ventricular anastomosis for correction of tricuspid atresia. J Thorac Cardiovasc Surg 1979;77:452--8]) for absent right atrio-ventricular connection was done by using a homograft between the right atrium and the right ventricle in some patients. We present the case of a 31-year-old patient, 21 years after a modified Fontan operation with severe homograft dysfunction. A percutaneous "tricuspid valve" implantation was performed with a Melody valve resulting in a good functional result.

Management of emergencies in adults with congenital cardiac disease.

The aim of the study was to assess the quantity and nature of emergencies affecting adults with congenital cardiac disease (CCD) and evaluate infrastructural requirements for adequate management. There is an increasing number of adults with CCD requiring specialized complex care. This multicenter study evaluated all emergency admissions to 1 of 5 centers for adults with CCD within 1 year. Within 1 year, there were 1,033 admissions of adults with CCD, and 201 (160 patients; age 16 to 71 years) were emergencies. Underlying cardiac anomalies were univentricular heart (22%), complete transposition (14%), tetralogy of Fallot (21%), and others (43%). Seventy percent of patients had undergone previous cardiac surgery. The main reason for acute admission was cardiovascular (arrhythmia, heart failure, syncope, aortic dissection, and endocarditis). Diagnostic procedures most often assigned were echocardiography (n = 223), chest x-ray (n = 95), Holter electrocardiography (n = 85), cardiac catheterization/electrophysiologic study (n = 39), and others (n = 143). Forty-six patients underwent surgery (cardiovascular n = 41, general n = 5) or electrophysiologic treatment (n = 41). One hundred twenty-six of 201 emergencies (63%) required cooperation with another specialized department: surgery (n = 46), internal medicine (n = 42), neurology (n = 12), ophthalmology (n = 6), otorhinolaryngology (n = 5), gynecology (n = 5), psychiatry (n = 4), radiology (n = 3), dermatology (n = 2), and orthopedics (n = 2). In conclusion, physicians and consultants attending adult patients with CCD need a high degree of specialized experience concerning the cardiac anomaly to manage emergencies properly. Furthermore, a wide range of noncardiac diagnostic and therapeutic procedures must be available. Data support the demand for a multidisciplinary approach in specialized centers for adequate care of adults with CCD.

Aortic valvuloplasty in pediatric patients substantially postpones the need for aortic valve surgery: a single-center experience of 188 patients after up to 17.5 years of follow-up.

BACKGROUND: Aortic valvuloplasty (AoVP) is an established procedure regarded as a valid alternative for surgical management of congenital aortic valve stenosis. However, its long-term efficacy in preventing or postponing aortic valve surgery remains uncertain for the individual patient. Therefore, the aim of this study was to study the long-term results of AoVP in pediatric patients and its efficacy in preventing or postponing aortic valve surgery. METHODS AND RESULTS: We reviewed up to 17.5 years of follow-up data of all 188 patients who received AoVP at the Deutsches Herzzentrum München. The patients were divided into those < 1 month of age (group < 1 month; n=68) and those > or = 1 month of age (group > or = 1 month; n=120) at the time of AoVP. After the first and second AoVP, moderate and severe aortic regurgitation developed in 29% and 14%, respectively, of the patients in group < 1 month and in 19% and 29%, respectively, of the patients in group > or = 1 month. Survival after 10 years free from aortic valve surgery was 59% (95% confidence interval, 45 to 73) in group < 1 month and 70% (95% confidence interval, 59 to 81) in group > or = 1 month. CONCLUSIONS: This study shows that the long-term results of AoVP of congenital aortic valve stenosis in pediatric patients and its efficacy in preventing or postponing aortic valve surgery are very good. About two thirds of the patients are free from aortic valve surgery 10 years after AoVP.

Replacement of the systemic atrioventricular valve with a mechanical prosthesis in children aged less than 6 years: late clinical results of survival and subsequent replacement.

OBJECTIVE: We analyzed the survival, clinical course, and role of prosthesis-patient mismatch after systemic atrioventricular valve replacement in children. METHODS: From 1974 to 2006, 69 patients underwent systemic atrioventricular valve replacement (median age 1.2 years, range 1.1 months to 5.4 years), with 17 patients requiring re-replacement of the systemic atrioventricular valve. Prosthesis-patient relationship was analyzed by comparing (1) the prosthetic valve diameter and the predicted annulus diameter based on the body surface area and (2) the prosthetic valve diameter and the measured annulus diameter. RESULTS: Survival was 73% at 1 year and 65% at 5, 10, and 15 years. Age, weight, body surface area, predicted annulus diameter, prior surgery, underlying disease, and ratio of prosthetic valve diameter to body weight were significant predictors of death. Variables associated with re-replacement of the systemic atrioventricular valve were body surface area, prosthetic valve diameter, predicted annulus diameter, and presence of multiple left-sided obstructive lesions. The majority of patients received a prosthesis larger than the predicted annulus diameter. There was good correlation between the prosthetic valve diameter and the measured annulus diameter (r = 0.85). Mismatch, as described by the difference in z scores of prosthetic valve diameter and measured annulus diameter, was not a significant predictor of death or re-replacement of the systemic atrioventricular valve. CONCLUSIONS: Although valve replacement is considered the last therapeutic option after failed attempts of valvuloplasty, long-term outcome is favorable. Selection of the prosthesis is made on the basis of the measured annulus diameter. An elevated ratio of prosthetic valve diameter to body weight is associated with patients with low body weight or a large native annulus in dilated ventricles.