Establishing a nationwide pediatric heart transplantation program with mid-term results comparable to worldwide data - The Czech experience.

BACKGROUND: Heart transplantation (HTx) is an established therapeutic option for children with end-stage heart failure. Comprehensive pediatric nationwide HTx program was introduced in 2014 in the Czech Republic. The aim of this study was to evaluate its mid-term characteristics and outcomes and to compare them with international data. METHODS: Retrospective observational study, including all patients who underwent HTx from June 2014 till December 2022. Data from the institutional database were used for descriptive statistics and survival analyses. RESULTS: A total of 30 HTx were performed in 29 patients with congenital heart disease (CHD, N = 15, single ventricular physiology in 10 patients) and cardiomyopathy (CMP, N = 14). Ten patients were bridged to HTx by durable left ventricular assist devices (LVADs) for a mean duration of 104 (SD 89) days. There was one early and one late death during median follow-up of 3.3 (IQR 1.3-6.1) years. Survival probability at 5 years after HTx was 93%. Two patients underwent re-transplantation (one of them in an adult center). Significant rejection-free survival at 1, 3, and 6 years after HTx was 76%, 63%, and 63%, respectively. CONCLUSIONS: The introduced pediatric HTx program reflects the complexity of the treated population, with half of the patients having complex CHD and one-third being bridged to HTx by LVADs. Mid-term results are comparable to worldwide data. The data confirm the possibility of establishing a successful nationwide pediatric HTx program in a relatively small population country with well-developed pediatric cardiovascular care and other transplantation programs.

Impact of liver fibrosis and nodules formation on hemodynamics in young adults after total cavopulmonary connection. A magnetic resonance study.

Background: The aim of this study was to analyze the relation between the hepatic fibrosis markers, liver morphology and hemodynamics assessed by magnetic resonance imaging (MRI) after total cavopulmonary connection (TCPC). Materials and methods: Adult patients after TCPC performed in childhood between 1993 and 2003 are the subjects of this observational study. The follow-up protocol consisted of clinical and echocardiographic examination, liver elastography, cardiopulmonary exercise test, MRI hemodynamics and liver morphology assessment and direct enhanced liver fibrosis (ELF) test. Results: The cohort consisted of 39 patients (46% female) with a median age at study 26 (IQR 23-28) years and interval from TCPC 21 (IQR 20-23) years. There was no correlation between ELF test and any MRI variables, but procollagen III amino-terminal peptide (PIIINP), a single component of ELF test, correlated significantly with ventricular end-diastolic volume (r = 0.33; p = 0.042) and inferior vena cava flow (r = 0.47; p = 0.003). Fifteen (38%) patients with liver nodules had compared to other 24 patients higher end-diastolic volume (ml/m2) 102.8 ± 20.0 vs. 88.2 ± 17.7; p = 0.023, respectively. PIIINP correlated significantly with inferior vena cava flow (r = 0.56; p = 0.030) and with end-diastolic volume (r = 0.53; p = 0.043), but only in patients with liver nodules. Conclusion: Gradual progression of liver fibrosis, particularly hepatic arterialization caused by liver nodules formation, increases inferior vena cava flow and subsequent ventricular volume overload may further compromise single ventricle functional reserve in adult patients after TCPC.

Structural Integrity and Cellular Viability of Cryopreserved Allograft Heart Valves in Right Ventricular Outflow Tract Reconstruction: Correlation of Histopathological Changes with Donor Characteristics and Preservation Times

Abstract Introduction: Cryopreserved allograft heart valves (CAHV) show longer event-free survival compared to other types of protheses. However, all patients develop early and/or late allograft failure. Negative predictors are clinical, and there is a lack of evidence whether they correspond with the microscopic structure of CAHV. We assessed histopathological signs of structural degeneration, degree of cellular preservation, and presence of antigen-presenting cells (APC) in CAHV and correlated the changes with donor clinical characteristics, cryopreservation times, and CAHV types and diameters. Methods: Fifty-seven CAHV (48 pulmonary, nine aortic) used for transplantation between November/2017 and May/2019 were included. Donor variables were age, gender, blood group, height, weight, and body surface area (BSA). Types and diameters of CAHV, cold ischemia time, period from decontamination to cryopreservation, and cryopreservation time were recorded. During surgery, arterial wall (n=56) and valvar cusp (n=20) samples were obtained from the CAHV and subjected to microscopy. Microscopic structure was assessed using basic staining methods and immunohistochemistry (IHC). Results: Most of the samples showed signs of degeneration, usually of mild degree, and markedly reduced cellular preservation, more pronounced in aortic CAHV, correlating with arterial APC counts in both basic staining and IHC. There was also a correlation between the degree of degeneration of arterial samples and age, height, weight, and BSA of the donors. These findings were independent of preservation times. Conclusion: CAHV show markedly reduced cellular preservation negatively correlating with the numbers of APC. More preserved CAHV may be therefore prone to stronger immune rejection.

Structural Integrity and Cellular Viability of Cryopreserved Allograft Heart Valves in Right Ventricular Outflow Tract Reconstruction: Correlation of Histopathological Changes with Donor Characteristics and Preservation Times.

INTRODUCTION: Cryopreserved allograft heart valves (CAHV) show longer event-free survival compared to other types of protheses. However, all patients develop early and/or late allograft failure. Negative predictors are clinical, and there is a lack of evidence whether they correspond with the microscopic structure of CAHV. We assessed histopathological signs of structural degeneration, degree of cellular preservation, and presence of antigen-presenting cells (APC) in CAHV and correlated the changes with donor clinical characteristics, cryopreservation times, and CAHV types and diameters. METHODS: Fifty-seven CAHV (48 pulmonary, nine aortic) used for transplantation between November/2017 and May/2019 were included. Donor variables were age, gender, blood group, height, weight, and body surface area (BSA). Types and diameters of CAHV, cold ischemia time, period from decontamination to cryopreservation, and cryopreservation time were recorded. During surgery, arterial wall (n=56) and valvar cusp (n=20) samples were obtained from the CAHV and subjected to microscopy. Microscopic structure was assessed using basic staining methods and immunohistochemistry (IHC). RESULTS: Most of the samples showed signs of degeneration, usually of mild degree, and markedly reduced cellular preservation, more pronounced in aortic CAHV, correlating with arterial APC counts in both basic staining and IHC. There was also a correlation between the degree of degeneration of arterial samples and age, height, weight, and BSA of the donors. These findings were independent of preservation times. CONCLUSION: CAHV show markedly reduced cellular preservation negatively correlating with the numbers of APC. More preserved CAHV may be therefore prone to stronger immune rejection.

Long-Term Survival and Freedom From Coronary Artery Reintervention After Arterial Switch Operation for Transposition of the Great Arteries: A Population-Based Nationwide Study.

Background The aim of this study was to evaluate long-term survival and freedom from coronary artery reintervention after the arterial switch operation (ASO). Methods and Results This single-center nationwide retrospective study included consecutive children who underwent ASO between 1990 and 2016 (n=605). Long-term outcomes were obtained by cross-mapping individual data with the National Death Registry and the National Registry of Cardiovascular Interventions for adults. A control group was randomly retrieved at a 1:10 ratio from the National Birth and Death Registries. Early mortality was 3.3% and late mortality was 1.7% during a median follow-up of 10 (interquartile range, 5-16) years. The probability of overall survival at 20 years after ASO was 94.9% compared with 99.5% in the background population (hazard ratio [HR] 15.6; 95% CI, 8.9-27.5, P<0.001). Independent multivariable predictors of worse survival were an intramural coronary artery (HR, 5.2; 95% CI, 1.8-15.2, P=0.002) and period of ASO 1990 to 1999 (HR, 4.6; 95% CI, 1.5-13.6, P<0.001). Fourteen patients (2.3%) required 16 coronary artery reoperations. Freedom from coronary artery reintervention at 20 years after ASO was 96%. The only independent multivariable predictor associated with a higher hazard for coronary artery reintervention was an intramural coronary artery (HR, 33.9; 95% CI, 11.8-97.5, P<0.001). Conclusions Long-term survival after ASO is excellent. Coronary artery reinterventions are rare. An intramural coronary artery was an independent predictor associated with a higher risk for coronary artery reintervention and death, regardless of the surgical period.

Aislamiento de la arteria subclavia derecha: reparación miniinvasiva / Isolation of the right subclavian artery. Mini-invasive repair

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Initial experience with polytetrafluoroethylene leaflet extensions for aortic valve repair.

OBJECTIVES: The purpose of this study is to evaluate our initial experience with aortic valve repair using polytetraflouroethylene (PTFE) leaflet extensions in congenital valvular disease. METHODS: From October 2008 through February 2011, 13 patients underwent aortic valvuloplasty by PTFE leaflet extensions. All valves were repaired in a tri-leaflet configuration using PTFE leaflet extensions. The median age at operation was 14 years (1.8-19.7 years) and the median weight was 58 kg (9.5-86 kg). Previous interventions included balloon valvuloplasty in two patients, aortic valvuloplasty in one and coarctation repair in one patient. Eight (73%) patients had combined aortic stenosis and insufficiency, three (23%) had isolated insufficiency and two (15%) had stenosis only. In 10 (77%) patients, a bicuspid aortic valve was present. RESULTS: The follow-up ranged from 2 to 30 months (mean follow-up 14.8 ± 9 months). At the latest echocardiography follow-up, six patients had none or trace aortic insufficiency, six patients had a mild aortic insufficiency and one patient had a mild-to-moderate insufficiency. The mean aortic insufficiency degree decreased from 1.8 ± 1.2 preoperatively to 0.8 ± 0.6 at the follow-up (P < 0.01). The mean gradient across the aortic valve decreased from 56 ± 40 mmHg preoperatively to 12 ± 13 mmHg at the follow-up (P < 0.0008). All patients are alive. There were no reoperations. The median hospital stay was 9 days (4-21 days). CONCLUSIONS: The use of PTFE leaflet extensions is an effective technique for aortic valve reconstruction in congenital valvular disease. Long-term follow-up is necessary to assess the durability of this type of repair.

A comparison of Blalock-Taussig shunts with and without closure of the ductus arteriosus in neonates with pulmonary atresia.

BACKGROUND: The question of whether to close the patent ductus arteriosus when performing primary modified Blalock-Taussig (MBT) shunt surgery in neonates is still not clearly answered. The aim of this report was to compare the results of closure versus nonclosure of the patent ductus arteriosus during MBT shunt surgery in neonates with pulmonary atresia. METHODS: This retrospective study included neonates with pulmonary atresia who underwent primary MBT shunt surgery through a sternotomy approach at our institution between January 1997 and October 2010. Mortality, resuscitation events, and the need for reintervention within the first 48 postoperative hours were studied as primary outcomes. RESULTS: Sixty-two neonates (mean age 6.9±5.5 days) underwent a MBT procedure. The arterial duct was closed surgically in 31 patients, and left open in 31 patients. Compared with patients in whom the PDA was left open, patients with a surgically closed arterial duct had a higher incidence of resuscitation events (29.0% versus 0%, p=0.0012), reinterventions (35.5% versus 3.2%, p=0.0013), and higher early hospital mortality (9.7% versus 0%, p=0.038). Time to extubation and length of hospital stay did not differ between the two groups (p=0.16 and p=0.73, respectively). A trend toward a higher maximum vasoactive-inotropic score in the group with a closed duct was observed (median 13.5 versus 10, p=0.10). CONCLUSIONS: In newborns with pulmonary atresia, ductal closure during MBT shunt procedure is associated with increased incidence of resuscitation events, need for reintervention, and increased mortality during the early postoperative period.

Ross-Konno operation in children.

The Ross-Konno procedure is an excellent technique for the treatment of complex multilevel left ventricular outflow tract obstruction with severe annular hypoplasia and a dysplastic aortic valve. The operation can be performed earlier in life, thus avoiding repeated surgical reinterventions, which may provide only short-term palliation and potentially exacerbate ventricular function. The Ross-Konno procedure increases our therapeutic choices for neonates or infants with critical aortic stenosis, who show unacceptable results following open valvotomy or balloon valvotomy. The pulmonary autograft demonstrates durability without the likelihood of developing aortic stenosis or progressive dilatation and a low incidence of developing aortic insufficiency. Despite the technically demanding nature of the operation, the Ross-Konno procedure is the method of choice for the multilevel type of left ventricle outflow tract obstruction, especially in newborns and infants.

Hypoplastic left heart syndrome with anomalous origin of left coronary artery from the right pulmonary artery: successful surgical treatment in a neonate.

Anomalous origin of the left coronary artery from the right pulmonary artery in association with hypoplastic left heart syndrome is a rare congenital anomaly. We describe a successful simultaneous surgery for both anomalies during the first stage palliation in a neonate.