[Show abstract][Hide abstract] ABSTRACT: We aimed to assess interventricular and right-intraventricular dyssynchrony in patients after tetralogy of Fallot (TOF) repair by two-dimensional (2D) speckle tracking and to identify factors associated with dyssynchrony.
Forty-two patients after TOF repair with a mean age of 19.8 years and 42 age-matched healthy controls were studied. Longitudinal myocardial deformation (strain) and time-to-peak intervals were assessed by 2D speckle tracking and tissue Doppler imaging (TDI) in an apical four-chamber view. Dyssynchrony was defined as delay above 3 standard deviations of mean values in the control group. Magnetic resonance imaging (MRI) was performed for evaluation of ventricular function. Using 2D speckle tracking, 22 patients (52%) showed interventricular dyssynchrony and 16 (38%) had right-intraventricular dyssynchrony. The interventricular delay correlated significantly with right ventricular (RV) strain (r = 0.687, P < 0.001), RV systolic pressure (r = 0.535, P = 0.001), QRS duration (r = 0.466, P = 0.002), RV end-diastolic (r = 0.377, P = 0.018), and RV end-systolic volumes (r = 0.452, P = 0.004) as well as RV ejection fraction (r = -0.378, P = 0.018). Similarly, the right-intraventricular delay correlated significantly with RV strain (r = 0.534, P < 0.001), QRS duration (r = 0.428, P = 0.005), RV end-systolic volume (r = 0.34, P = 0.038), and RV systolic pressure (r = 0.413, P = 0.015). In multivariate regression analysis, reduced RV strain and prolonged QRS duration remained the main determinant factors predicting dyssynchrony. Moreover, 2D speckle tracking and TDI showed a significant correlation in the assessment of the interventricular (r = 0.738, P < 0.001) and right-intraventricular delay (r = 0.747, P < 0.001).
Interventricular and right-intraventricular dyssynchrony are detectable in patients after TOF repair by 2D speckle tracking. Reduced RV myocardial deformation and QRS prolongation are the main factors associated with the observed dyssynchrony.
Full-text · Article · Oct 2010 · European Heart Journal – Cardiovascular Imaging
[Show abstract][Hide abstract] ABSTRACT: We investigated whether a correlation exists between biomarkers of the neurohumoral system and clinical markers in grown-up patients with congenital heart disease (GUCH) and right ventricular function.
Prospective, cross-sectional, multicenter study of 104 GUCH patients (median) 16 years (range 6-43 years) after corrective surgery with RV pressure and/or volume overload and 54 healthy controls. Clinical, functional, and laboratory parameters were assessed. Natriuretic peptide levels were significantly increased in GUCH patients (NTproBNP 101 vs. 25 pg/ml, p < 0.001), but we observed no differences in norepinephrine, aldosterone, angiotensin II and Endothelin-1 levels. NTproBNP correlated significantly with clinical markers such as NYHA classification, prolonged QRS duration and reduced exercise capacity (VO(2) peak) (all p < 0.001), as well as self-reported quality of life (p < 0.001). MRI and echocardiography derived RV volumes were elevated and ejection fraction reduced in the patients (both p < 0.001). Tissue Doppler parameter showed significantly restricted ventricular longitudinal systolic function (longitudinal tricuspid valve movement, 1.7 vs. 2.3 cm, p < 0.001), suggesting stiffness and reduced RV compliance.
In conclusion, grown-up patients with congenital right heart disease NTproBNP correlates well with various clinical markers of RV failure, such as prolongation of QRS duration, exercise capacity, echocardiography and MRI parameters, and quality of life.
Full-text · Article · Oct 2010 · Clinical Research in Cardiology
[Show abstract][Hide abstract] ABSTRACT: The aim of this study was to investigate the hemodynamic profile and heart rhythm in infants who were given intravenous clonidine infusion after prolonged analgesia/sedation following cardiac surgery.
This is a single center retrospective review. A total of 542 cardiovascular surgical procedures in infants aged 0-24 months with congenital heart disease were performed between 01/2003 and 12/2005 at the Deutsches Herzzentrum in Berlin. The majority received no long-term analgesia/sedation, but 50 (9%) of these infants received clonidine (dosed at 0.18-3.6 microg.kg(-1).h(-1)) for sedation and to reduce withdrawal symptoms such as CNS hyperactivation, hypertension, tachycardia, and fever. The hospital records of these infants were studied.
Fifty infants (median age 5.0 months, median body weight 5.3 kg, 32 males/18 females) received prolonged analgesia/sedation to ensure hemodynamic stability. Clonidine infusion started on day 5 (median) after surgery. During clonidine treatment we found an age-related normalized profile of hemodynamic parameters with a reduction of heart rate and mean arterial pressure from the upper norm to the mean within 24 h (P < 0.001). In no case did clonidine cause low blood pressure resulting in additional therapy to reach the target blood pressure. There were no adverse effects on cardiac rhythm, especially no onset of atrioventricular block. Midazolam, fentanyl, and other opioids could be ended on day 4 of clonidine treatment.
Although off-label, it is feasible to use clonidine infusions in infants in the PICU setting after cardiac surgery without hemodynamic problems arising.
[Show abstract][Hide abstract] ABSTRACT: Patent ductus arteriosus (PDA) is frequent in neonates with gestational age of less than 28 wk. Clinical and echocardiographic signs define hemodynamic significance of PDA, but do not reveal the need for PDA intervention in the first days of life. B-type natriuretic peptide (BNP) has been proposed as a screening tool for PDA in preterm infants. To determine whether BNP can predict the need for PDA intervention, plasma BNP was measured by chemiluminescence immunoassay in 67 preterm infants <28 wk (median 26) on the second day of life in a prospective blinded study. PDA intervention was based on specified clinical and echocardiographic findings. Twenty-four patients (intervention group) received treatment for PDA and 43 patients (controls) remained without intervention. BNP concentrations were higher in the intervention (median 1069 pg/mL) than in the control group (247 pg/mL, p < 0.001). BNP correlated positively with ductal size (R = 0.46, p < 0.001) and atrial/aortic root ratio (R = 0.54, p < 0.001). In conclusion, plasma BNP proved to be a good predictor for ductus intervention (area under the curve: 0.86) with the best cutoff at 550 pg/mL on the second day of life in ventilated infants less than 28 wk gestation (sensitivity: 83%; specificity: 86%).
Full-text · Article · Apr 2008 · Pediatric Research
[Show abstract][Hide abstract] ABSTRACT: The purpose of this study was to determine the effect of mid-term mechanical circulatory support on the natriuretic hormone system in children and to assess whether such changes are associated with myocardial recovery.
Serial blood samples were collected from 19 children (median age 10.8 years, range 0.2 to 17.5 years), all supported with a pulsatile ventricular assist device (Berlin Heart EXCOR; median support time 55 days). Levels of NT-proBNP were analyzed before and 7 and 30 days after device implantation. In addition, we determined levels of mid-region proANP (MR-proANP) and BNP in 13 of the 19 children.
The actuarial survival rate to discharge home was 84%. Two children could be weaned from the system, 14 reached heart transplantation, and 3 died during mechanical circulatory support. Serial measurements of NT-proBNP, BNP and MR-proANP showed a significant down-regulation of all three natriuretic peptides within the first week of support and a further decrease between Days 7 and 30. The lowest NT-proBNP level while on the device (250 pg/ml) was found in the child later weaned, who reached normal levels (71 pg/ml) within 12 weeks after weaning.
Extremely high levels of natriuretic peptides reflect the severity of myocardial failure before device implantation. During mechanical support, the decline of natriuretic peptides appears to be a helpful additional tool in the pre-selection of potential weaning candidates.
Full-text · Article · Mar 2008 · The Journal of heart and lung transplantation: the official publication of the International Society for Heart Transplantation
[Show abstract][Hide abstract] ABSTRACT: Diaphragmatic palsy (DP) is a rare but severe complication after surgery for congenital heart disease. Transthoracic diaphragmatic plication is an effective means of treatment for those with respiratory impairment due to DP, but little is known about the mid-term effects of diaphragmatic plication.
We performed a study in 24 patients with history of DP. Diaphragm movement was assessed using ultrasound. Patients with DP who were old enough were additionally followed-up with lung function and exercise testing. A group of patients with similar age, diagnoses and operations served as controls.
Ultrasound showed that in the majority of cases with history of DP the paralysed diaphragm was static, independently of whether it was plicated or not. Patients with DP had a more restrictive lung function pattern (VC: 54.3 vs. 76.4% predicted, p<0.001; FEV(1): 58.4 vs. 86.2% predicted, p<0.001) and a lower exercise capacity compared with the control group (peak VO2: 24.5 vs. 31.3 ml/kg/min, p=0.03). Comparing patients with and without plication for DP, only a tendency towards lower lung function values in patients after diaphragmatic plication, but no differences regarding exercise capacity, could be found.
Our results provide evidence that DP is a serious surgical complication with a reduction in lung function and exercise capacity, even at mid-term follow-up; however, diaphragmatic plication, a useful tool in treating post-surgical DP in children with respiratory impairment, seems to be without mid-term risk in terms of recovery of phrenic nerve function, lung function values, and exercise capacity.
Full-text · Article · Nov 2007 · Intensive Care Medicine
[Show abstract][Hide abstract] ABSTRACT: Abstract Pulmonary hypertension was diagnosed in a former conjoined twin presenting with a ventricular septal defect, moderate congestive heart failure, and dyspnea with hypercapnia due to instable bony chest. He underwent successful corrective cardiac surgery and chest wall reconstruction at the age of 12 months. Subsequently, mechanical ventilation was performed for three months to prevent right heart failure and pulmonary hypertension until thoracic stability allowed the tracheostoma to be closed.
No preview · Article · Jul 2007 · Journal of Cardiac Surgery
[Show abstract][Hide abstract] ABSTRACT: Pediatric size pneumatically driven extracorporeal ventricular assist devices (VAD) for infants and small children were introduced into clinical routine in 1992. In the initial period, the results in infants were poor. Since then, several improvements have been introduced with regard to the cannulas, connectors, heparin coating of the blood pump inner surface, anticoagulant treatment and coagulation monitoring, and earlier decision-making in favor of pump implantation before irreversible shock has set in.
Since 1990 and as of January 1, 2005, 62 Berlin Heart Excor systems have been implanted in patients below 18 years of age at our institution. The patients were divided into two groups according to the period of treatment: period 1, devices implanted between 1990 and 1998 (n = 34), and period 2, devices implanted between 1999 and 2004 (n = 28). We compared our experience during the earlier and later periods.
There were no significant differences in the preoperative patient data between the two periods except for time of support (17.9 +/- 27.7 days versus 53.2 +/- 83.9 days, p = 0.001). In period 1, more patients needed a biventricular VAD whereas in period 2, more patients were effectively treated with a left VAD (p = 0.05). In the later period, the chest could be primarily closed in a significantly higher percentage of infants (0% versus 89%, p = 0.012), and more infants could be extubated on the VAD (0% versus 55%, p = 0.16). Discharge from the hospital after either weaning from the system or heart transplantation was achieved for 35% in period 1 and for 68% in period 2 (p = 0.029). Whereas in period 1 there were no survivors in the group of children younger than 1 year old, during period 2, survival in this age group was similar to that of the two groups of older children (p = 0.024). There was a significant improvement in the discharge rate in period 2 in patients with cardiomyopathy (43% versus 76%, p = 0.045) and postcardiotomy heart failure (0% versus 57%, p = 0.01).
Earlier implantation of VADs, heparin coating of the blood pumps, and substantial modifications in cannula design, anticoagulation, and the coagulation monitoring regimen have led to a significant increase in the survival and discharge rate, especially among children under 1 year of age. The pediatric size Berlin Heart Excor VAD is a valuable option as a bridge to heart transplantation or recovery for children suffering from cardiogenic shock.
Full-text · Article · Oct 2006 · The Annals of thoracic surgery
[Show abstract][Hide abstract] ABSTRACT: Within the last 16 years, the Berlin Heart EXCOR systems have been used for circulatory support in 73 infants and children up to 17 years of age (mean, 7 years) with severe circulatory failure resistant to pharmacological therapy at our institution. These were patients with cardiomyopathy, fulminant myocarditis, end-stage congenital cardiac defects, and acute heart failure after congenital heart surgery. Mean EXCOR support time was 36 days (range, 1 to 420 days). Forty-four patients (62%) survived up to transplantation or after weaning and 37 (51%) of them, including 9 infants, were discharged home. These results in patients with very advanced disease have improved significantly during the past few years as the result of technical developments and growing experience in the treatment of patients on the device and in postoperative care. The following article gives answers to five questions frequently asked about intensive care unit treatment while on the device, anticoagulation and additional medical support during left ventricular or biventricular support, the daily treatment of the children, and options for the weaning procedure.
[Show abstract][Hide abstract] ABSTRACT: We examined the clinical impact of diaphragmatic palsy (DP) as a result of phrenic nerve injury following cardiothoracic surgery, specifically its effects on morbidity and mortality, early regeneration capacity of the phrenic nerve, and role of surgical diaphragmatic plication.
A retrospective case control study was performed in 74 children with DP and 74 matched controls after cardiothoracic surgery within the past 14 years.
Following 5,128 surgical procedures in children (aged under 18 years) we found an incidence of DP of 1.4%. There were no differences in mortality between the groups, and the cause of death was not related to DP or plication in any of the patients. However, patients with diaphragm impairment had significantly longer duration of mechanical ventilation (median 3 days vs. 1), ICU stay (7 days vs. 3.5), duration of hospital stay (16 days vs. 12), and for antibiotic treatment (16 days vs. 7). Because of prolonged respiratory problems 40 children (54%) underwent surgical diaphragmatic plication to flatten the diaphragm in its inspiratory position. In children with DP younger age was a strong predictor for plication (median 3.8 months vs. 12.1).
Especially in newborns and young infants with DP the length of mechanical ventilation, ICU stay, and hospital stay are prolonged. Early spontaneous recovery of the phrenic nerve is rare. In cases of respiratory impairment early transthoracic diaphragmatic plication is an effective means of treatment.
Full-text · Article · Sep 2006 · Intensive Care Medicine
[Show abstract][Hide abstract] ABSTRACT: Although considerable progress has been made on ventricular assist devices (VAD) for adults, pneumatic pulsatile circulatory support in young infants is still limited. There is a need for long-term ventricular assist devices to bridge the failing myocardium of young children until recovery or transplantation. Miniaturized devices and innovative modalities need to be optimized. We report on our experience.
From 1/1992 to 6/2004, 18 infants (6 male/12 female) under 1 year of age were treated with the Berlin Heart Excor VAD. The infants were divided into two groups, depending on the year of treatment. Group A consists of eight infants resuscitated and supported with a pulsatile pneumatic ventricular assist device between 1992 and 1998 and group B consists of 10 infants treated between 1999 and 6/2004. With the pediatric-sized Berlin Heart we used miniaturized extracorporeal pneumatically driven blood pumps, the lowest stroke volume being 10 ml.
In 18 children, age 3-345 (median 147) days, artificial replacement of heart function was applied for long-term support (1-64, median 10 days) as a life-saving measure in our hospital. Nine had LVAD and nine BVAD support. All were in cardiogenic shock with multiorgan failure; three had fulminant myocarditis, four cardiomyopathy, and one chronic stage of congenital heart disease. Five children were weaned from the system, three reached heart transplantation, and 10 died on the VAD. There were no differences between groups A and B regarding age, body weight or diagnosis, but the duration of mechanical support differed: Group A, median 2, range 1-16 days; group B, median 12, range 1-100 days. Since 1999 (group B), the survival rate of our small infants has increased to 70% whereas none of the infants in group A survived to be discharged.
The outcome of VAD support in small infants is no longer inferior to that of adult support, now optimized cannulas, modified anticoagulation and optimized surgical and intensive care management have been established.
Preview · Article · Sep 2005 · European Journal of Cardio-Thoracic Surgery
[Show abstract][Hide abstract] ABSTRACT: The introduction of the Fontan operation for single ventricle physiology was based on the dual principle of the pulmonary blood flow. It is postulated that normal breathing movements are necessary for passive blood flow into the lungs. We compared patients with and without palsy of the phrenic nerve regarding the sufficiency of Fontan hemodynamics.
We analyzed 85 consecutive patients, who were available for follow-up after completion of their total cavopulmonary connection (TCPC) between February 1992 and February 2003. The median age at TCPC completion was 4.3 (range 1.3-37) years. Sixty were operated on with an extracardiac conduit and 25 with a lateral tunnel. Fifty patients underwent postoperative heart catheterization with contrast angiography. The diagnosis of diaphragm paralysis was made using echocardiography, fluoroscopy and X-ray examination. Surgical diaphragm plication was performed in 13 patients (Four before and nine after Fontan operation) at a median of 2.2 years after the diagnosis.
Twenty-one patients developed fixed palsy of the phrenic nerve during a total of 225 operations before and including completion of TCPC. There were no differences in the incidence of phrenic nerve paralysis between small children (aged <3 years) and older patients or between patients with the extracardiac and intracardiac Fontan procedures. There were no differences in the duration of mechanical ventilation. However, prolonged pleural effusions and a hospital stay of longer than 2 weeks were noted more frequently in patients with palsy (P<0.05). During the median follow-up of 4.6 (range: 0.7-11.4) years significantly more patients with phrenic nerve palsy developed chronic ascites compared to those without palsy (8 of 20 vs. 2 of 65; P<0.001).
Phrenic nerve palsy was recognized as a risk factor for suboptimal Fontan hemodynamics due to the hindrance of passive venous blood flow. Patients with phrenic nerve palsy have a longer hospital stay and a higher incidence of prolonged pleural effusions and of chronic ascites, than those without. Early diaphragm plication may be favorable to optimize the Fontan circuit in these patients. Completion of the TCPC in patients with diaphragm paralysis should be viewed critically.
Full-text · Article · Apr 2005 · European Journal of Cardio-Thoracic Surgery
[Show abstract][Hide abstract] ABSTRACT: Different mechanical circulatory support (MCS) systems are used in children with intractable heart failure. However, the need for anticoagulation leads to hemorrhage with subsequent use of blood products. We compared the coagulation disorders and the need for blood products in children treated either with extracorporeal membrane oxygenation (ECMO) or with the Berlin Heart pulsatile pneumatic ventricular assist device.
We retrospectively reviewed the first 8-day course of 64 children who were on MCS for more than 2 days between 1990 and 2002. Thirty children (median age 7.4 years, weight 25.5 kg) received Berlin Heart support and 34 children (median age 1.8 years, weight 9.2 kg) ECMO. Anticoagulation was accomplished by continuous infusion of heparin. Red blood cell count, platelet count, aPTT, AT III, fibrinogen, and ACT were measured regularly. Depending on blood loss and the coagulation disorder, red blood cells, fresh frozen plasma, platelets, and AT III were substituted.
There were no preoperative differences in hematological parameters between the two groups. In the Berlin Heart group platelet transfusion was 4.3 ml x kg x day vs 24.6 ml x kg x day in the ECMO group. Red blood cell substitution was 17.2 vs 60.3 ml.kg.day. Fresh frozen plasma substitution was 8.5 ml x kg x day vs 46.9 ml x kg x day (P<0.001). Even in the congenital heart defect subgroups, when MCS was implanted without recent cardiotomy, the differences were significant. Nevertheless, the mean daily values for hemoglobin, platelets, and fibrinogen were lower in the ECMO group. There was lower overall mortality in the Berlin Heart group.
Compared to ECMO, use of the Berlin Heart in children results in less blood loss and lower consumption of red blood cells, platelets, and fresh frozen plasma.
Full-text · Article · Sep 2004 · Intensive Care Medicine