Aortic Valve Repair in Pediatric Patients: 30 Years Single Center Experience.

BACKGROUND: Valve repair is the procedure of choice for congenital aortic valve disease. With increasing experience, the surgical armamentarium broadened from simple commissurotomy to more complex techniques. We report our 30-year experience with pediatric aortic valve repair. METHODS: A retrospective chart review of all patients aged less than 18 years who underwent aortic valve repair from May 1985 to April 2020 was conducted. Mortality was cross-checked with the national health insurance database (96% complete mortality follow-up in April 2020). Primary study endpoints were survival and incidence of reoperations. RESULTS: From May 1985 until April 2020, 126 patients underwent aortic valve repair at a median age of 1.8 years (interquartile range, 0.2-10). Early mortality was 5.6% (7 of 126). All early deaths occurred in neonates with critical aortic stenosis undergoing commissurotomy. No early deaths were observed after 2002. Kaplan-Meier estimated survival was 90.8% (95% CI, 84.0-94.8) at 10 years, 86.9% (95% CI, 78.7-92.2) at 20 years, and 83.5% (95% CI, 71.7-90.6) at 30 years. The cumulative incidence of aortic valve replacement was 37% (95% CI, 27.7-46.3) at 10 years, 62.2% (95% CI, 50.1-72.1) at 20 years, and 67.4% (51.2-79.2) at 30 years. Nine patients had undergone re-repair of the aortic valve. The majority of valve replacements were Ross procedures. CONCLUSIONS: Our results support a repair-first strategy for patients with congenital heart disease and underline that aortic valve reconstruction can be a successful long-term solution. Longevity did not differ between aortic valve commissurotomy and complex aortic valve reconstruction.

Long-term outcomes after surgical repair of subvalvular aortic stenosis in pediatric patients.

Objectives: Subvalvular aortic stenosis (SAS) can occur as discrete or tunnel-like obstruction of the left ventricular outflow tract and as progressive disease often leads to aortic valve regurgitation. We report our 30-year single-center experience after surgical repair of SAS. Methods: A retrospective chart review of all patients aged < 18 years, who underwent surgical repair of SAS from May 1985 to April 2020, was conducted. Mortality was cross-checked with the national health insurance database (93.8% complete mortality follow-up in April 2020). Survival and competing risks analysis were used to analyze the primary endpoints survival and incidence of reoperations. Results: From May 1985 until April 2020 103 patients (median age 5.5 years) underwent surgical repair of SAS. Survival was 90.8% at 10 years and 88.7% at 20 and 30 years. Age < 1 year at time of surgery, Shone's complex, mitral stenosis and concomitant mitral valve surgery were associated with mortality. The cumulative incidence of reoperation for SAS was 21.6% at 10 years, 28.2% at 20 and 30 years. The incidence of reoperation for SAS did not differ between the myectomy, membrane resection and combined myectomy and membrane resection groups. The cumulative incidence of reoperation on the aortic valve was 13.5% at 20 years. Conclusion: Recurrence rate of SAS is not to be neglected, though surgical repair of subaortic stenosis has good long-term results. Patients who needed a combined membrane resection and septal myectomy are not more prone to recurrence than patients who underwent solitaire myectomy or membrane resection.

Copeptin: Prognostic Relevance as a Perioperative Marker in Pediatric Cardiac Surgery.

BACKGROUND: Copeptin is a cleavage product of vasopressin. This study aimed to figure out if copeptin would be a suitable biomarker in patients with congenital heart disease in the postoperative course. METHODS: The primary outcome endpoint of this study was the change in copeptin concentration perioperatively in patients with congenital heart disease after surgery, with the use of a cardiopulmonary bypass. Three blood samples were taken from 81 patients up to 6 years of age in order to evaluate changes in copeptin concentration. RESULTS: Significant increase of copeptin concentration was shown between the first and second blood draws as well as between the first and third blood draws (Ps < .001). Additionally, positive and significant correlations (r ≥ .27) between the cardiopulmonary bypass times, The Society of Thoracic Surgeons and European Association for Cardio-Thoracic Surgery mortality category, the inotropic score, the duration of mechanical ventilation, the length of stay at the intensive care unit (ICU), the length of stay at the hospital, and the preoperative as well as the ICU copeptin levels were found. CONCLUSIONS: Copeptin showed a tendency to predict the clinical outcome of patients after congenital heart surgery. Patients with higher copeptin levels underwent more complex procedures, had longer cardiopulmonary bypass times, required more catecholamine support, needed longer time of invasive ventilation, and had longer overall stay and ICU stay.

Performing central venous catheters in neonates and small infants undergoing cardiac surgery using a wireless transducer for ultrasound guidance: a prospective, observational pilot study.

BACKGROUND: Neonates and small infants with congenital cardiac disease undergoing cardiac surgery represent major challenges facing paediatric anaesthesia and perioperative medicine. AIMS: We here aimed to investigate the success rates in performing ultrasound (US) guided central venous catheter insertion (CVC) in neonates and small infants undergoing cardiac surgery, and to evaluate the practicability and feasibility of thereby using a novel wireless US transducer (WUST). METHODS: Thirty neonates and small infants with a maximum body weight of 10 kg and need for CVC before cardiac surgery were included in this observational trial and were subdivided into two groups according to their weight: < 5 kg and ≥ 5 kg. Cannulation success, failure rate, essential procedure related time periods, and complications were recorded and the clinical utility of the WUST was assessed by a 5-point Likert scale. RESULTS: In total, CVC-insertion was successful in 27 (90%) of the patients and the first attempt was successful in 24 (78%) of patients. Success rates of CVC were 80% < 5 kg and 100% ≥5 kg. Comparing the two groups we found a clear trend towards longer needle insertion time in patients weighing < 5 kg (33 [28-69] vs. 24 [15-37]s, P = .07), whereas, the total time for catheter insertion and the duration of the whole procedure were similar in both groups (199 [167-228] vs. 178 [138-234] and 720[538-818] vs. 660 [562-833]s. In total, we report 3 (10%) cases of local hematoma as procedure-related complications. Assessments of the WUST revealed very good survey results for all parameters of practicability and handling (all ratings between 4.5 and 5.0). CONCLUSION: Although difficulties in CVC-placement seem to relate to vessel size and patient's weight, US guided CVC-insertion represents a valuable, fast, and safe intervention in neonates and small children undergoing cardiac surgery. Using the WUST is feasible for this clinical application and may aid in efforts aiming to optimize perioperative care. TRIAL REGISTRATION: Wireless US-guided CVC placement in infants; Clinicaltrials.gov: NCT04597021 ; Date of Registration: 21October, 2020; retrospectively registered.

Implanting the HeartMate 6 (total artificial heart).

The HeartMate 3 is a ventricular assist device that supports the heart with a centrifugal continuous flow. It contains a fully levitated rotor to minimize hemolysis and was initially designed as an apical intrapericardial implant. It can be used as a bridge to a transplant, to recovery, or to destination therapy. After we excise the ventricles, we implant 2 HeartMate 3 devices as a total artificial heart (HeartMate 6). The patient was 35 years old when the devices were implanted and had been diagnosed with Yamaguchi syndrome (apical hypertrophic cardiomyopathy) at 13 years of age. Being listed for a transplant was not an option due to secondary pulmonary hypertension. Furthermore, the conventional method of apically implanting a left ventricular assist device was not possible due to the underlying pathology. A HeartMate 6 implant as a bridge to transplant therapy was planned. Additionally, a CardioMEMS HF System was implanted to monitor the pulmonary artery pressure. The video tutorial provides step-by-step instructions for implanting 2 HeartMate 3 devices as a total artificial heart.

Surgical stented transcatheter valve-in-valve implantation in atrioventricular position in children.

The Melody valve (Medtronic, Minneapolis, MN, USA) is a stented bovine jugular vein graft that was primarily approved for transcatheter implantation in a pulmonary valve position. The prosthetic valve can also be implanted in an atrioventricular position in infants and young children, and in these cases it must be modified appropriately.  In this tutorial we demonstrate the surgical preparation of a stented transcatheter Melody valve for implantation in the atrioventricular position. Additionally, we present a safe and effective method for surgical valve-in-valve implantation in a 3-year-old patient with hypoplastic left heart syndrome.

Implantation of the Berlin Heart EXCOR ventricular assist device.

The Berlin Heart EXCOR® is a mechanical, pulsatile ventricular assist device. The paracorporeal assist device is pneumatically driven and can be used for long-term bridging therapy of one or both ventricles (LVAD, BIVAD, RVAD). It is specifically designed for pediatric patients and can be used in neonates as well as juveniles and adults.  The infant presented in this video tutorial was diagnosed with myocarditis leading to end-stage heart failure and severe mitral valve regurgitation. A bridging therapy was indicated and a Berlin Heart EXCOR® VAD was implanted. This tutorial provides detailed insight on how to perform this procedure. In addition, a safe and effective way of extending the outflow cannula is demonstrated.

Hemostatic consequences of a non-fresh or reconstituted whole blood small volume cardiopulmonary bypass prime in neonates and infants.

OBJECTIVES: Despite aggressive measures to miniaturize the cardiopulmonary bypass (CPB) circuit in neonates and infants, the CPB prime volume is often at least as large as the patients' blood volume. We conducted an observational study to characterize the hemostatic consequences of a CPB prime consisting of either non-fresh or reconstituted whole blood. METHODS: Hematocrit, fibrinogen, platelet count, plasminogen, anti-thrombin III (AT-III), and factors (F) II, V, VII, IX, and X of 30 neonates and infants undergoing cardiac surgery with CPB utilizing either a non-fresh or reconstituted whole blood prime were prospectively evaluated at eight time points. Following protamine administration, microvascular bleeding was treated by protocol. RESULTS: The hemostatic composition of the CPB prime was the same following the use of either non-fresh or reconstituted whole blood. The CPB prime platelet count (mean +/- SD) was 5.87 +/- 2.84 x 10(3) microl(-1) when compared to a preoperative platelet count of 298 +/- 142 x 10(3) microl(-1) (P < 0.0001). Twenty patients received 17.3 +/- 9.2 ml x kg(-1) (0.86 +/- 0.46 units x kg(-1)) of platelets with significant improvement in platelet count. Nine patients received 16.7 +/- 13.4 ml x kg(-1) (0.84 +/- 0.67 units x kg(-1)) of cryoprecipitate with significant improvements in FVIII and fibrinogen. CONCLUSIONS: Non-fresh or reconstituted whole blood as a component of a small volume CPB prime in neonates and infants induces clinically significant dilutional thrombocytopenia in conjunction with less significant reductions in fibrinogen, FII, FV, FVII, FVIII, FIX, FX, plasminogen, and AT-III.

Distribution of ABO phenotypes in patients with congenital cardiac defects.

BACKGROUND: Cardiac anomalies are among the most frequent congenital malformations, but the basic underlying causes for most cardiac defects remains undetermined. Some 40 years ago, a higher incidence of blood group B was reported in a small number of African-American children with congenital cardiac defects. In this study, we sought to re-evaluate this association using a larger population. METHODS AND RESULTS: We collected data from 1985 patients undergoing cardiac surgery from July, 2000, through December, 2004. We divided the patients into 6 subgroups according to their diagnosis. We then compared the prevalence of ABO phenotypes between the patients and the general population of the United States of America by chi-square analysis. There were no significant differences in the distribution of the ABO phenotypes amongst the subgroups of those with congenital cardiac disease, or any for subgroup compared to the general population. CONCLUSION: While statistical significance is influenced by the size of the population within the United States of America and the small numbers within each of our subgroups of patients with congenital cardiac disease, we have been unable to show any relationship between the distribution of ABO phenotypes and the existence of congenital cardiac disease.

Evaluation of the coagulation system in children with two-ventricle congenital heart disease.

BACKGROUND: Multiple coagulation factor abnormalities involving both procoagulant and anticoagulant proteins have been described in children with single-ventricle physiology. This study used age-matched controls to evaluate coagulation factors in children with two-ventricle congenital heart disease (CHD). METHODS: Coagulation factors were assayed in 120 patients with CHD, divided into four age groups: group 1, 0 to 3 months; group 2, 3 to 12 months; group 3, 12 to 48 months; and group 4, older than 48 months. Healthy children without CHD were assayed as controls. Concentration of factors II, V, VII, VIII, IX, and X; protein C and S, plasminogen, and antithrombin III, were measured by standard assays. Normal ranges were determined by the empirical 95% confidence intervals. RESULTS: Significant reductions were found in mean levels of both procoagulant and anticoagulant factors in patients in groups 1, 2, and 3 compared with controls, but no differences were found in group 4. In group 1, all variables had significantly lower concentrations except fibrinogen and protein S; in group 2, all variables had significantly lower concentrations except for fibrinogen, factors VIII and IX, and plasminogen and protein S; and in group 3, all variables had significantly lower concentrations except fibrinogen, factors VIII and IX, and antithrombin III, plasminogen, and protein S. CONCLUSIONS: Neonates and infants with two-ventricle CHD have lower levels of procoagulant and anticoagulant factors compared with aged-matched controls approaching normal levels in children aged older than 4 years. These coagulation factor abnormalities are similar to those described in patients with single-ventricle physiology.

Pre-existing anticardiolipin antibodies and development of restenosis after coronary balloon angioplasty: predictive value for a complicated course?

Immune mechanisms play a critical role in cardiovascular disease. Cardiolipins are candidate autoantigens with a prothrombotic activity of their corresponding antibodies. We investigated the influence of pre-existing immunoglobulin (Ig)M and IgG anticardiolipin (aCL) antibodies on restenosis after coronary balloon angioplasty and their interaction with tissue plasminogen activator, plasminogen activator inhibitor type-1, von Willebrand factor and lipoprotein (a) in 132 patients with stable angina pectoris using immunoassays. Thirty percent of patients developed angiographically proven restenosis estimated by three independent experienced angiographers; 12% of all patients developed recurrent restenoses at the same site during a follow-up period of 2 years. Circulating IgM aCL antibodies categorized by quartiles predicted recurrent restenoses (logistic regression, for trend P < 0.04) with an increase of relative risk (RR) per quartile of 2.09. The predictive value of IgM aCL antibodies was unchanged adjusting for established cardiovascular risk factors (P = 0.028, RR = 2.69), extent of coronary artery disease (P = 0.014, RR = 2.73) and inflammatory parameters (P = 0.025, RR = 2.79), but lost significance adjusting for other prothrombotic parameters (P = 0.24, RR = 1.76). IgM aCL antibodies positively correlated with lipoprotein (a) (r = 0.23, P = 0.04). However, there was no significant interaction between their influences on recurrent restenoses. The other prothrombotic parameters did not predict single or recurrent restenoses. In conclusion, IgM aCL antibodies may help to identify a group of patients at high risk for recurrent restenoses after coronary balloon angioplasty.