German Registry for Cardiac Operations and Interventions in Patients with Congenital Heart Disease: Report 2020-Comprehensive Data from 6 Years of Experience.

BACKGROUND: Based on a quality assurance initiative of the German Society for Thoracic and Cardiovascular Surgery (DGTHG) and the German Society for Pediatric Cardiology and Congenital Heart Defects (DGPK), a voluntary registry was founded for assessment of treatment and outcomes of patients with congenital heart disease in Germany. This evaluation by the German Registry for cardiac operations and interventions in patients with congenital heart disease reports the data and the outcome over a 6-year period in patients undergoing invasive treatment. METHODS: This real-world database collects clinical characteristics, in-hospital complications, and medium-term outcome of patients who underwent cardiac surgical and interventional procedures within the prospective, all-comers registry. Patients were followed-up for up to 90 days. RESULTS: In the period from 2013 to 2018, a total of 35,730 patients, 39,875 cases, respectively 46,700 procedures were included at up to 31 German institutions. The cases could be subcategorized according to the treatment intention into 21,027 (52.7%) isolated operations, 17,259 (43.3%) isolated interventions, and 1,589 (4.0%) with multiple procedures. Of these, 4,708 (11.8%) were performed in neonates, 10,047 (25.2%) in infants, 19,351 (48.5%) in children of 1 to 18 years, and 5,769 (14.5%) in adults. Also, 15,845 (33.9%) cases could be allocated to so-called index procedures which underwent a more detailed evaluation to enable meaningful comparability. The mean unadjusted in-hospital mortality of all cases in our registry ranged from 0.3% in patients with isolated interventions and 2.0% in patients with surgical procedures up to 9.1% in patients undergoing multiple procedures. CONCLUSION: This annually updated registry of both scientific societies represents voluntary public reporting by accumulating actual information for surgical and interventional procedures in patients with congenital heart disease (CHD) in Germany. It describes advancements in cardiac medicine and is a basis for internal and external quality assurance for all participating institutions. In addition, the registry demonstrates that in Germany, both interventional and surgical procedures for treatment of CHD are offered with high medical quality.

Acquired von Willebrand syndrome in congenital heart disease surgery: results from an observational case-series.

Essentials Bleeding complications during congenital heart disease surgery in neonatal age are very common. We report the perioperative incidence of acquired von Willebrand syndrome (aVWS) in 12 infants. aVWS was detected in 8 out of 12 neonates and infants intraoperatively after cardiopulmonary bypass. Ten patients received von Willebrand factor concentrate intraoperatively and tolerated it well. SUMMARY: Background Cardiac surgery of the newborn and infant with complex congenital heart disease (CHD) is associated with a high rate of intraoperative bleeding complications. CHD-related anatomic features such as valve stenoses or patent arterial ducts can lead to enhanced shear stress in the blood stream and thus cause acquired von Willebrand syndrome (aVWS). Objective To evaluate the intraoperative incidence and impact of aVWS after cardiopulmonary bypass (CPB) in neonates and infants with complex CHD. Patients/Methods We conducted a survey of patients aged < 12 months undergoing complex cardiac surgery in our tertiary referral center. Twelve patients, whose blood samples were analyzed for aVWS before CPB and immediately after discontinuation of CPB on a routine basis, were eligible for the analysis. von Willebrand factor antigen (VWF:Ag), ristocetin cofactor activity (VWF:RCo), collagen binding activity (VWF:CB), VWF:multimers and factor VIII activity (FVIII:C) were determined. Results aVWS was diagnosed by VWF multimer analysis in 10 out of 12 patients (83%) prior to surgery and intraoperatively at the end of CPB in 8 out of 12 patients (66%). Ten patients received VWF/FVIII concentrate intraoperatively as individual treatment attempts during uncontrolled bleeding. They tolerated it well without intraoperative thrombotic events. One patient suffered a transient postoperative cerebral sinuous vein thrombosis. Conclusions aVWS is of underestimated incidence in complex CHD surgery. These data may offer a new approach to reduce the risk of severe bleedings and to achieve hemostasis during high-risk pediatric cardiac surgery by tailoring the substitution with von Willebrand factor concentrate.

Non-invasive measurement of renal perfusion and oxygen metabolism to predict postoperative acute kidney injury in neonates and infants after cardiopulmonary bypass surgery.

BACKGROUND: The pathophysiology of acute kidney injury (AKI) after cardiopulmonary bypass surgery for congenital heart disease is not completely understood. The aim of this study was to carry out a prospective analysis of the diagnostic value of non-invasive monitoring of renal oxygenation and microcirculation by combining laser Doppler flowmetry and tissue spectrometry. METHODS: In 50 neonates and infants who underwent repair (n = 31) or neonatal palliation (n = 19) of congenital heart disease with cardiopulmonary bypass, renal oxygenation, and microcirculatory flow, the approximate renal metabolic rate of oxygen and Doppler-based renal resistive index were determined after surgery. Correlations between these parameters and the occurrence of AKI according to the Pediatric Risk, Injury, Failure, Loss, End Stage Renal Disease criteria were investigated. RESULTS: Acute kidney injury occurred in 45% of patients after repair and in 32% after palliation. Renal oxygenation was significantly lower and the approximate renal metabolic rate of oxygen significantly higher in patients with AKI (P < 0.05). The microcirculatory flow was significantly higher in patients with AKI after neonatal palliation (P < 0.05), whereas renal resistive index was significantly higher in patients with AKI after repair (P < 0.05). The sensitivity of renal oxygenation, metabolic rate of oxygen, microcirculation, and resistive index in predicting AKI was 78-80, 73-78, 64-83, and 71-74%, respectively, with a specificity of 63-65, 54-75, 64-78, and 46-74% (area under the curve: 0.73-0.75, 0.68-0.83, 0.52-0.68, and 0.60-0.75), respectively. CONCLUSIONS: Monitoring of renal oxygen metabolism allows early prediction of AKI in infants after cardiac surgery. In contrast, renal resistive index does not allow prediction of AKI after neonatal palliation with aortopulmonary shunt establishment.

Efficacy and safety of zero-fluoroscopy ablation for supraventricular tachycardias. Use of optional contact force measurement for zero-fluoroscopy ablation in a clinical routine setting.

BACKGROUND: Conventional catheter ablation of cardiac arrhythmias is associated with radiation risks for patients and laboratory personnel. Widespread use of zero-fluoroscopic catheter ablation in clinical routine is limited by safety concerns. This study investigated the feasibility of zero-fluoroscopy catheter ablation using a three-dimensional mapping system and optional catheter contact force technology for an all-comers collective. PATIENTS AND METHODS: The study comprised 184 patients; 91 patients, including 29 pediatric patients, underwent a zero-fluoroscopic electrophysiology (EP) study using the EnSite NavX system with real-time visualization of all electrodes. These patients were matched to a control group, which was treated using fluoroscopy in the same period. Inclusion criteria were documented supraventricular tachycardia or a history of symptomatic paroxysmal supraventricular tachycardia. Transseptal access, if necessary, was achieved under transesophageal echocardiographic guidance for ablation of left-sided arrhythmias. Radiofrequency (using optional contact force measurement) or a cryotechnique was used for ablation. RESULTS: We observed no major acute complications. There were no significant differences between the two groups in the follow-up period. CONCLUSION: Zero-fluoroscopic catheter ablation is generally feasible in right-sided cardiac arrhythmias. Safety concerns regarding left atrial substrates or children can be overcome with optional real-time contact force measurement.

Safety, efficacy and response to a hydrocortisone rescue therapy protocol in children with refractory hypotension after cardiopulmonal bypass.

Little is known about which paediatric patients respond to hydrocortisone rescue therapy (HRT) with improvement of haemodynamic stability in refractory hypotension after cardiopulmonal bypass. Data were gathered retrospectively from children who received HRT in refractory hypotension after cardiopulmonary bypass in the period from 2000 to 2010. One hundred and sixty-six out of 1,273 children, 150 <1 year and 16 >1 year were enrolled. HRT improved haemodynamics significantly, increased blood pressure, decreased the vasoactive-inotropic score and plasma lactate concentrations in all children >1 year and in 82 % (123 out of 150) of the infants <1 year. Non-responders <1 year were significantly younger, lighter, mostly male infants and had longer cardiopulmonary bypass support time. Serum lactate and paediatric risk of mortality score were significantly higher in non-responders at time of initiation of HRT. Mortality was significantly higher in non-responders versus responders (2.44 vs. 13.5 %; p = 0.0008). HRT caused no adverse effects like electrolyte disturbances or hyperglycaemia. HRT in refractory hypotension after paediatric cardiac surgery is safe but not all infants <1 year show haemodynamic response to HRT. Non-response to HRT is associated with significantly higher mortality.

[Contact force control - the key to safe zero-fluoroscopy catheter ablation of atrioventricular nodal reentrant tachycardia]. / Die Anpresskraftkontrolle als Schlüssel zur sicheren strahlenfreien Katheterablation der AV-Knoten-Reentrytachykardie.

BACKGROUND: Atrioventricular nodal reentrant tachycardia (AVNRT) is a frequent supraventricular tachycardia in children and young adults. Despite favourable success rates of catheter ablation, conventional fluoroscopic catheter guidance is associated with risks of low-dose ionizing radiation for the patient and the personnel. Here we describe a technique for zero-fluoroscopy catheter ablation using contact force technology. PATIENTS AND METHODS: Zero-fluoroscopy catheter ablation was attempted in 12 patients with AVNRT (median age 20 years; range 11-75 years). An ablation catheter with integrated contact force sensor and a nonfluoroscopic electroanatomical mapping system was used for visualization of cardiovascular structures. Mean contact forces during mapping and ablation were restricted to an upper limit of 50 g to avoid cardiovascular injuries. RESULTS: Zero-fluoroscopy catheter ablation was performed successfully and uneventfully in all patients. There were no arrhythmia recurrences during a median follow-up of 6.2 months (range 2.7-12.8). CONCLUSION: Zero-fluoroscopy catheter ablation of AVNRT is possible and appears simple yet safe, when a nonfluoroscopic electroanatomical mapping system is used in combination with an ablation catheter with integrated contact force sensor. The presented technique could thus be easily employed in most electrophysiological laboratories.

Cryorecanalization of an obstructed bronchial stent in a 12-year-old boy.

We report a 12-year-old boy who underwent stent implantation into the left main bronchus at the age of 9.5 years for palliation of severe bronchial stenosis. He had developed complete obstruction of the stent by granulation tissue resulting in respiratory deterioration and the requirement of mechanical ventilation. The stent obstruction was treated at the age of 11.5 years by cryorecanalization. In this technique, cooling of the tip of the cryoprobe is used to induce adherence of the obstructing tissue allowing the subsequent removal of tissue particles by retraction of the probe. Under general anesthesia, a miniaturized cryoprobe was advanced via the working channel of a flexible bronchoscope. Repeat maneuvers of freezing and retraction resulted in complete recanalization of the stent. Repeat bronchoscopies 4 and 12 weeks later revealed recurrent formation of some granulation tissue which was removed by repeat cryorecanalization. Seven months after the initial procedure there was a complete patency of the stent. According to our experience, cryorecanalization is a safe and effective alternative for the treatment of stent obstruction by granulation tissue. Due to the introduction of a miniaturized probe, this method is well applicable in children since it can be performed via the working channel of a flexible pediatric bronchoscope.

Giant infantile fibroma of the right ventricle - surgical debulking and tumor plication.

Cardiac fibromas are rare lesions which occur predominantly in infants and children. In a 2-week-old premature infant with progressive exertional dyspnea, a huge cardiac tumor (5.0 x 4.5 x 5.0 cm) obstructing the right ventricle was diagnosed. Due to tumor progression with resulting obstruction of the right ventricular outflow tract (RVOT), surgery became necessary at 6 months. The tumor was partially resected, creating a crater-like defect, and the resection margins were subsequently plicated. Histological examination confirmed infantile fibroma. The combination of early diagnosis, the time and opportunity for cardiac development and immediate excision once symptoms occur is supposed to improve survival.

Sepsis and lung abscess following malposition of an umbilical vein catheter in a neonate.

BACKGROUND: Umbilical vein catheterisation is a common and useful procedure in the care of critically ill newborns, but several complications may occur. PATIENTS: We report on a newborn, who developed sepsis and necrotizing pneumonia of the right middle lobe due to extended spectrum beta lactamase (ESBL) Klebsiella following malposition of an umbilical vein catheter into a right pulmonary vein. RESULTS: Due to failed conservative treatment the child required lobectomy of the right middle lobe. CONCLUSION: Our case highlights the importance of exact determination of the position of central catheters by x-ray and/or ultrasound immediately after placement.

Comparison of imaging techniques in the diagnosis of bridging bronchus.

Bridging bronchus (BB) is a rare, congenital bronchial anomaly that is frequently associated with congenital cardiac malformations, especially left pulmonary artery sling. It represents an anomalous bronchus to the right originating from the left main bronchus. Discrimination from other bronchial anomalies is important, since BB is frequently associated with bronchial stenoses due to abnormal cartilage rings. This case study describes the findings of bronchoscopy, bronchography and multidetector computed tomography (MDCT) in three patients. Bronchoscopy was helpful in the description of the severity and length of bronchial stenoses. However, it was not possible to establish a diagnosis of BB based on this method in two patients, since it is difficult or even impossible to differentiate the bifurcation from the pseudocarina. It was not possible to establish the correct diagnosis in all patients based on bronchography or MDCT. MDCT was able to depict the relationship of bronchial and vascular structures, which is particularly important in patients with pulmonary artery sling. Multidetector computed tomography is preferable to bronchography as it is less invasive and due to its short acquisition time it can be performed in children with severe respiratory disease. In the current authors' experience, detection of cartilage rings still requires flexible bronchoscopy.

[Systematic evaluation of the proximal cardiac veins for echocardiographic diagnosis of total anomalous pulmonary venous connection]. / Nichtinvasive Diagnostik der totalen Lungenvenenfehleinmündung durch systematische echokardiografische Darstellung der herznahen Venen.

PURPOSE: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiovascular malformation that is characterized by an absent connection of the pulmonary veins to the left atrium. Echocardiographic differentiation of TAPVC and persistent pulmonary hypertension of the newborn (PPHN) can be a great diagnostic challenge. The aim of our study was the assessment of a systematic echocardiographic approach to evaluate the feasibility and reliability of noninvasive diagnosis of TAPVC. METHODS: Between January 1995 and January 2005, 15 consecutive patients with isolated TAPVC were diagnosed in our institution. 5 patients had supracardiac type, 5 cardiac type, 3 infracardiac and 2 had mixed type of TAPVC. Since 2001 preoperative diagnosis was performed exclusively by echocardiography (8/15 patients). RESULTS: TAPVC could be diagnosed correctly in all cases using a systematic echocardiographic approach with careful investigation of the proximal cardiac veins. Infracardiac type ofTAPVC was diagnosed using systematic evaluation of the liver with visualization of a pathological vessel with venous flow directed away from the heart. In all cases the echocardiographic findings were confirmed during surgery. CONCLUSION: Echocardiography with Doppler and color Doppler sonography is an effective method for noninvasive diagnosis of TAPVC. The presented systematic echocardiographic approach permits reliable noninvasive differentiation of TAPVC and PPHN.

[The use of implantable cardioverter-defibrillators (ICD) in children and adolescents]. / ICD-Schrittmachertherapie bei Kindern und Jugendlichen.

BACKGROUND: The compelling safety and efficacy data in numerous large, blinded trials on adult patients, and the progress in device- and leadtechnology have led to increasing use of implantable cardioverter defibrillators in pediatric patients. The purpose of our study was to assess the efficacy and safety of ICD in the pediatric age group of a tertiary referral centre. PATIENTS AND METHODS: Between March 1998 and October 2003 12 patients underwent ICD-implantation. The mean age at implantation was 14,8 years with a range between 10-17 years. The underlying cardiac disorders included long QT-syndrome in 4 patients, ventricular fibrillation in 3 patients, dilated cardiomyopathy in 4 patients, and congenital heart disease in 1 patient (pulmonary atresia with ventricular septal defect after Rastelli repair). All patients received a transvenous ICD-system (VVI-ICD in 4 patients, DDD-ICD in 8 patients). RESULTS: The mean follow up was 35 months (6-68 months). During this period there were no severe complications nor mortality. We haven't seen infections, thromboembolic complications or lead-perforations. 2 patients (17 %) received appropriate DC-shocks, 1 patient (8 %) received an inappropriate DC-shock. 10 patients (83 %) had no malignant ventricular arrhythmia under medical therapy. 2 patients (17 %) required revision because of lead-dysfunction. In 2 patients with DCM the device was explanted during orthotopic heart transplantation. CONCLUSIONS: Our data demonstrate that advances in device- and leadtechnology have resulted in a decrease of severe complications in the pediatric age group. We conclude that ICD-implantation represents a safe and effective therapy for children and adolescents with lifethreatening ventricular dysrhythmias. Since it represents an invasive therapy, indication should be confined to patients with lifethreatening dysrhythmias according to the guidelines of the American Heart Association.

Recanalisation of bilateral superior vena cava after total cavopulmonary connection. Interventional occlusion with the Amplatzer VSD Occluder.

UNLABELLED: Formation of systemic to pulmonary venous or systemic venous left atrial collaterals frequently occurs in patients after Glenn or Fontan-type operations. Embolization with detachable metal coils is the therapy of choice for the closure of small vessels. These devices however are not appropriate for the occlusion of large collaterals, e. g. recanalized bilateral caval veins. We report two patients who presented late after Fontan-type operations with a gradual decrease in oxygen saturation due to recanalisation of bilateral caval veins. Interventional closure of these large veins was carried out successfully with the use of 8 mm Amplatzer muscular VSD Occluders, resulting in an increase of arterial oxygen saturations. CONCLUSION: The closure of recanalized bilateral superior caval veins after Fontan procedures is possible without technical problems by means of the Amplatzer muscular VSD Occluder. In order to avoid future formation of venous collaterals via the azygos or hemiazgos system, the occluder should be placed in the vena cava below the orifice of the azygos/ hemiazygos vein.

Excision of the coronary orifices in arterial switch operation: "O" like obstructive and "U" like unobstructive?

BACKGROUND: Stenoses of the neo-pulmonary artery (NPA) may complicate follow-up of the arterial switch operation (ASO). It is unknown whether the type of patch covering the coronary excision defects ("O"- or "U"-shaped) might influence this complication. METHODS: Echocardiographically and invasively measured NPA pressure-gradients were evaluated retrospectively in 95 children after ASO. Median follow-up was 5.8 years. Defects had been covered with pericardial patches: O/O and U/U (left/right) 34 x each, and 27 x in mixed combinations. The frequency of NPA stenoses requiring re-interventions was registered. RESULTS: Median of the peak instantaneous echocardiographic pressure gradient was 23 mmHg (interquartile range, IQR: 16 - 49, n = 34) in O/O, and 19 mmHg (IQR: 13 - 23, n = 34) in U/U; p < 0.034, t-test. Invasively measured gradients were 49 mmHg (IQR: 17 - 65, n = 12) in O/O, and 12 mmHg (IQR: 7-21, n = 28) in U/U; p < 0.001. One child per O/O- and U/U-group underwent balloon angioplasty of the neo-pulmonary root. Five children of the O/O-group had to undergo repeat surgery, whereas only one child in the U/U-group required repeat surgery ( p < 0.34). CONCLUSIONS: The preferential type of covering the NPA coronary excision sites in ASO should be U-shaped.

[Aortic root abscess without involvement of the aortic valve: diagnosis and therapy in a 2.5-year-old child]. / Aortenwurzelabszess ohne Beteiligung der Aortenklappe: Diagnostik und Therapie bei einer 2 1/2-jährigen Patientin.

Although formation of an aortic root abscess is a frequent complication of aortic valve endocarditis in adults, this complication has been rarely observed in children. In the majority of cases it has been described in children without underlying congenital heart disease. Due to the rarity of this complication, diagnosis and treatment is frequently delayed in childhood. We report a 2 1/2 year old girl who developed pericardial effusion in the course of pneumonia. Echocardiographic examinations, which were performed because of the pericardial effusion, revealed after 6 days the development of a cystic structure posterior to the aortic root. There was a perforation of this aortic root abscess to the left ventricular outflow tract; the aortic and mitral valves however were normal without endocarditic vegetations. Surgery was performed on the 10th day following a rapid increase in the size of the abscess. During surgery the abscess was drained and the perforation to the left ventricle was closed with direct sutures. Intraoperative transesophageal echocardiography confirmed a good surgical result. Blood cultures remained negative; in the material from the abscess however we found staphylococcus aureus. The postoperative course was uneventful. Our case demonstrates the necessity of detailed and repeated echocardiographic examinations in children with possible symptoms of bacterial endocarditis (in our case pericardial effusion) as well as the requirement of cultures of the abscess for identification of the infective organism. Intraoperative transesophageal echocardiography allows exact description of an aortic root abscess, its relation to other cardiac structures and immediate evaluation of the surgical result.

Hemostatic changes following the modified Fontan operation (total cavopulmonary connection).

Thromboembolism is a serious complication after Fontan operation, which may be caused by alterations of the coagulation system. We therefore investigated pro- and anticoagulant factors in 20 patients aged 4 to 21 years, 4 to 63 months following total cavopulmonary connection. Furthermore we compared markers of thrombin activation and fibrinolysis and in vitro clotting and clot-lysis to age-matched healthy subjects. Compared to results of age-matched controls, the Fontan operated individuals had significant decreases in levels of protein C (0.88 U/ml in controls, 0.67 U/ml in patients; p <0.001) and protein S (1.05 in controls, 0.93 U/ml in patients; p <0.05). Moreover, half of the patients had high values of FVIII (>1.5 IU/ml), which are associated with an increased thrombotic risk. These changes may result in enhanced generation of thrombin and plasmin, indicated by our finding of increased thrombin-antithrombin III (TAT) and plasmin-antiplasmin (PAP) levels and a similar trend in prothrombin fragments F1+2. Clot lysis tests, global coagulation tests, red blood cell count, liver enzymes AST, ALT, but not GGT, were generally within the normal ranges.

[Rate of prenatal detection of congenital right heart defects]. / Pränatale Detektionsrate angeborener Rechtsherzfehler.

Congenital right heart lesions (including tetralogy of Fallot, pulmonary valve stenosis, pulmonary atresia with intact ventricular septum, Ebstein's anomaly and dysplastic tricuspid valve) account for about 19% of congenital cardiac anomalies. We performed a retrospective study in order to assess the percentage of patients with significant right heart lesions (requiring therapy in the first year of life), which is detected prenatally and referred to a centre for perinatal treatment. From 1/1990 until 12/1997 congenital right heart lesions were diagnosed in 21 fetuses and 190 infants (211 patients. The majority of patients had tetralogy of Fallot (64%), less frequently we found critical pulmonary valve stenosis (9%), pulmonary atresia with intact ventricular septum (9%), tricuspid atresia (14%) and Ebstein's anomaly or dysplastic tricuspid valve (4%). Prenatally the cardiac anomaly was diagnosed in all 21 cases who were referred to our center (10%). The highest referral and detection rate was found among fetuses with Ebstein's anomaly or dysplastic tricuspid valve (5/8 patients = 63%) followed by fetuses with pulmonary atresia and intact ventricular septum (5/20 = 25%), critical pulmonary stenosis (4/18 = 22%) or tricuspid atresia (4/29 = 14%). The prenatal referral rate was disappointing in children with tetralogy of Fallot (3/136 = 2.2%). A higher prenatal detection rate of congenital right heart lesions can be achieved only by an improvement of prenatal screening including the 4-chamber view and the origin of the great arteries. A first step would be the inclusion of the fetal 4-chamber view into the routine examination during the 18th-20th week of pregnancy (stage 1 of a multistage concept of prenatal screening) and by assessment of the outflow tracts and the great arteries in pregnancies associated with risk factors or anomalies of the fetus (stage 2 and 3 of a multistage concept).