Ambulatory Intravenous Inotropic Support and or Levosimendan in Pediatric and Congenital Heart Failure: Safety, Survival, Improvement, or Transplantation.

End-stage heart failure (HF) frequently needs continuous inotropic support in hospital and has high morbidity and mortality in absence of heart transplantation. This study reports outcome, efficacy, and safety of continuous ambulatory inotropes (AI) and/or periodic levosimendan (LS) infusions in pediatric HF patients. The study included 27 patients, median age 9.4 (0.1-26.1) years, with severe HF (6 myocarditis, 13 dilated cardiomyopathy, 2 restrictive cardiomyopathy, 6 repaired congenital heart disease). Dobutamine and milrinone AI were administered in 21 patients through a permanent central catheter for median duration 1.0 (0.3-3.7) years. Additionally, 14 AI patients and the remaining 6 study patients received periodic LS infusions for median duration 1.1 (0.2-4.2) years. During median follow-up 2.1 (0.3-21.3) years, 4 patients died of worsening HF after 0.8-2.1 years AI, 6 patients underwent heart transplantation with only 3 survivors, while the rest remained stable out of the hospital with complications 4 line infections treated with antibiotics and 4 catheter reinsertions due to dislodgement. Severe pulmonary hypertension was reversed with AI in 2 patients, allowing successful heart-only transplantation. Therapy with AI was discontinued after 1.4-0.4 years in 6 improved myocarditis and 3 cardiomyopathy patients without deterioration. In conclusion, prolonged AI and/or LS infusions in HF are safe and beneficial even in small infants, allowing stabilization and reasonable social and family life out of the hospital. It may provide precious time for heart transplantation or myocardial remodeling, improvement, and possible discontinuation even after long periods of support.

Pulmonary vasodilator therapy in tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals: case series and review of literature.

We present the use of pulmonary vasodilators in three adult patients with unrepaired tetralogy of Fallot, pulmonary atresia, aortopulmonary collaterals, and segmental pulmonary arterial hypertension. Patients improved by 1-2 NYHA classes with modest exercise-tolerance increase, and remained stable without side effects during 2.5, 10, and 14 years. Literature review revealed five studies with pulmonary vasodilators in heterogeneous, mostly repaired patient populations.

Descending aorta to right atrial fistula: Transcatheter embolization of a very rare anomaly with coils.

Key Clinical Message: Descending aorta to right atrial (RA) fistula is a rare and distinct clinical entity mimicking patent ductus arteriosus (PDA) and it may lead to rapid development of pulmonary vascular disease. Correct diagnosis and treatment, especially in the presence of other congenital heart defects, is very important. Interventional management is the treatment of choice. Abstract: We present a case report of a trisomy 21 infant with atrial and ventricular septal defects and small patent ductus arteriosus (PDA) complicated by the presence of descending aorta to right atrial (RA) fistula with large left to right shunt leading to rapid increase in pulmonary vascular resistance. Transcatheter occlusion of the fistula followed by closure of the PDA with Nit-Occlud coil systems led to decreased pulmonary pressure and resistance permitting successful surgical repair of the patient's intracardiac defects with good outcome over 3 years of follow-up. Descending aorta to RA fistula is a rare and distinct clinical entity mimicking PDA and its correct diagnosis and treatment, especially in the presence of other congenital heart defects, is very important as it may lead to rapid development of pulmonary vascular disease.

Left ventricular geometry and systolic function improvement after percutaneous closure of aortic prosthetic paravalvular leak.

Paravalvular leak (PVL) represents a potential complication of valve replacement surgery. The 'gold standard' treatment for patients with severe prosthetic dysfunction, hemolysis or symptoms, is surgery. Reoperative valve surgery for such patients is related to high morbidity and mortality rate. The percutaneous closure of PVL is feasible and safe, when performed at experienced interventional cardiologic centers. Herein, the case is reported of a patient who showed a significant improvement in left ventricular systolic function after transcatheter closure of an aortic prosthetic PVL with an Amplatzer duct occluder II (ADO II). A review of the current literature is also provided.

Arrhythmias in Patients with Congenital Heart Disease: An Ongoing Morbidity.

With the aging of congenital heart disease (CHD) patients, the burden of arrhythmias is expanding. Atrial arrhythmias, especially intra-atrial reentrant tachycardia and atrial fibrillation, are the most prevalent forms of arrhythmia. Managing comorbidities, such as obesity, using pharmacotherapy, including antiarrhythmics and anticoagulants, and ablation therapy has become the cornerstone of arrhythmia management. Ventricular tachycardias are also not rare; however, except for tetralogy of Fallot patients, recommendations for the use of implantable cardioverter defibrillators for primary prevention in other CHD patients are still not well established. Patients with CHD might also present with atrioventricular blockages because of their anatomy or following a surgical procedure. The scope of this article is to review the current knowledge and discuss the future directions regarding arrhythmia management in CHD patients.

Efficiency and Safety of Patent Ductus Arteriosus Surgical Ligation in Extremely Low Birth Weight Infants Without Chest Tube Placement.

Patent ductus arteriosus (PDA) has been associated with increased morbidity and mortality in preterm infants. Surgical ligation (SL) is generally performed in symptomatic infants when medical management is contraindicated or has failed. We retrospectively reviewed our institution's experience in surgical management of PDA for extremely low birth weight (ELBW) infants without chest tube placement assessing its efficiency and safety. We evaluated 17 consecutive ELBW infants undergoing SL for symptomatic PDA (January 2012-January 2018) with subsequent follow-up for 6 months postdischarge. Patients consisted of 9 (53%) females and 8 (47%) males. Mean gestational age (GA) at birth was 27.9 ± 2.1 weeks. Median values for surgical age (SA) from birth to operation was 10 days (interquartile range [IQR]: 8-12); PDA diameter 3.4 mm (IQR: 3.2-3.5); surgical weight (SW) 750 g (IQR: 680-850); and days of mechanical ventilation (DMV) as estimated by Kaplan-Meier curve 22 days (95% confidence interval: 14.2-29.8). We observed a statistically significant negative association between DMV and GA at birth (rho = - 0.587, p = 0.017), SA (rho = - 0.629, p = 0.009) and SW (rho = - 0.737, p = 0.001). One patient experienced left laryngeal nerve palsy confirmed by laryngoscopy. Otherwise, there were no adverse events to include surgical-related mortality, recurrence of PDA, or need for chest tube placement during follow-up. SL of PDA in ELBW infants without chest tube placement is both efficient and safe. Universal consensus recommendations for the management of PDA in ELBW neonates are needed. Further study is required regarding the use of the less invasive option of percutaneous PDA closure in ELBW infants.

The Ross-Konno procedure as reoperative treatment in a young adult with congenital aortic stenosis.

Mechanical and biological prostheses are valid options when aortic valve replacement is necessary. The Ross procedure is also an alternative solution, especially for young patients. We describe the case of a young patient with congenital aortic stenosis and bicuspid aortic valve who presented with dyspnea on exertion. An open commissurotomy was performed, and within 8 months the patient developed recurrent symptoms of severe aortic stenosis. He underwent redo sternotomy and a Ross-Konno procedure with an uneventful recovery.

Evaluating the Effectiveness of Stenting for Aortic Coarctation.

BACKGROUND: Coarctation of the aorta (CoA) is a congenital cardiovascular malformation involving narrowing of the thoracic aorta just distal to the left subclavian artery. The aim of our study was to evaluate the hemodynamic effects of endovascular treatment for CoA by using invasive aortic catheterization. METHODS: All patients with CoA who underwent treatment by aortic stent implantation between September 1, 2003, and February 1, 2019, at the "Onassis Cardiac Surgery Center," in Athens, Greece, were evaluated. Patients were treated with either bare (uncovered) Cheatham-Platinum (bCP) or covered Cheatham-Platinum (cCP) stent implantations. Invasive aortic pressure measurements were recorded before and after the endovascular intervention. RESULTS: A total of 48, eight zig CP stents, comprising 24 bCP and 24 cCP stents were implanted in 47 patients. The mean aortic diameter (mm) at the CoA lesion increased from 9.7 ± 3.3 to 19.2 ± 2.9 mm (p <0.01) after the endovascular procedure. The invasive mean blood pressure (BP; mm Hg) from catheterization in the descending aorta increased (before = 114.2 ± 12.8 vs. after = 135.5 ± 28.1; p <0.01), while the invasive mean BP (mm Hg) from catheterization in the ascending aorta was decreased (before = 156.8 ± 25.0 vs. after = 138.4 ± 27.5; p <0.01) after the intervention. The mean aortic BP gradient decreased in both types of stents after intervention (BP gradient among patients with cCP stents = 30.9 +/- 23.6 mmHg and BP gradient among patients with bCP stents = 38.0 +/-23.1 mmHg). However, there was no statistically significant difference between the two types of stents; p = 0.36. CONCLUSIONS: Invasive aortic catheterization provided evidence that endovascular stenting with either bare or covered stents is efficient in treating patients with CoA.

FDXR Gene Expression after in Vivo Radiation Exposure of Pediatric Patients Undergoing Interventional Cardiology Procedures.

BACKGROUND: Ferredoxin reductase (FDXR) has already been reported as a promising biomarker for estimating radiation doses in radiotherapy. This study aimed to investigate the responsiveness of FDXR on pediatric population exposed to ionizing radiation (X-rays) during pediatric interventional cardiology (IC) procedures. PATIENTS AND METHODS: Peripheral blood was collected by venipuncture from 24 pediatric donors before and 24 hours after the IC procedure. To estimate the effective dose, demographic data and Air Kerma-Area Product (PKA) were recorded for each patient. The relative quantification (RQ) of the FDXR gene in irradiated patient blood samples compared to the non-irradiated blood samples was determined using qPCR analysis. The relative values of FDXR were log- transformed. RESULTS: The effective dose ranged from 0.002 mSv to 8.004 mSv. Over this radiation exposure range, the FDXR gene expression varied randomly with the effective dose. Up-regulation in FDXR expression was observed in 17 patients and down-regulation in 7 patients. CONCLUSIONS: Further studies in a larger cohort of pediatric patients along with the record of clinical data are needed to determine whether FDXR gene expression is an effective biomarker for radiation exposure estimation in pediatric imaging.

Acute Kidney Injury Predictors and Outcomes after Cardiac Surgery in Children with Congenital Heart Disease: An Observational Cohort Study.

Acute Kidney Injury (AKI) commonly complicates cardiac surgery in children with congenital heart disease (CHD). In this study we assessed incidence, risk factors, and outcomes of postoperative AKI, while testing the hypothesis that, depending on the underlying diagnosis, there would be significant differences in AKI incidence among different diagnostic groups. We conducted an observational cohort study of children with CHD undergoing cardiac surgery in a single tertiary center between January 2019 and August 2021 (n = 362). Kidney Disease Improving Global Outcome (KDIGO) criteria were used to determine the incidence of postoperative AKI. Diagnosis was incorporated into multivariate models using an anatomic-based CHD classification system. Overall survival was estimated using Kaplan−Meier curves. Log-rank test and adjusted Cox proportional hazard modelling were used to test for differences in survival distributions and determine AKI effect on survival function, respectively. AKI occurred in 70 (19.3%), with 21.4% in-hospital mortality for AKI group. Younger age, lower weight, longer cardiopulmonary bypass time, preoperative mechanical ventilation and diagnostic category were associated with postoperative AKI. Resolution rate was 92.7% prior to hospital discharge for survivors. AKI was associated with longer duration of mechanical ventilation, ICU and hospital length of stay. AKI patients had significantly higher probability of all-cause mortality postoperatively when compared to the non-AKI group (log-rank test, p < 0.001). Adjusted hazard ratio for AKI versus non-AKI group was 11.08 (95% CI 2.45−50.01; p = 0.002). Diagnostic category was associated with cardiac surgery-related AKI in children with CHD, a finding supporting the development of lesion specific models for risk stratification. Postoperative AKI had detrimental impact on clinical outcomes and was associated with decreased survival to hospital discharge.

The impact of cardiovascular comorbidities associated with risk for left heart disease on idiopathic pulmonary arterial hypertension: Data from the Hellenic Pulmonary Hypertension Registry (HOPE).

Whereas younger female patients were diagnosed with idiopathic pulmonary arterial hypertension (IPAH) in 1980s, it is now frequently encountered in elderly patients with cardiovascular comorbidities (CVCs) associated with increased risk for left heart disease. We present data until November 2019 regarding specific features and clinical outcomes of IPAH population from the Hellenic Pulmonary Hypertension Registry (HOPE). Patients were divided into two groups based on the presence of ≥ or <3 CVCs, arterial hypertension, diabetes mellitus, obesity, presence of coronary artery disease, or atrial fibrillation. Overall, 77 patients with IPAH (55.1 [interquartile range, IQR: 24.1] years, 62.8% women) have been recorded. Fifteen patients (19.2%) had ≥3 CVCs, while 25 (32%) were over 65 years old. Patients with ≥3 CVCs were older, presented an almost equal female to male ratio, walked less in 6-min walk test, and had lower mean arterial pulmonary pressure and pulmonary vascular resistance at baseline than patients with less CVCs. Fewer patients with ≥3 CVCs received PAH-specific treatment compared to patients with less comorbidities (n = 11 [73.3%] versus n = 58 [95.5%], p = 0.02). During a median follow-up period of 3.8 (IQR: 2.7) years, 18 patients died (all-cause mortality 24.3%). Male sex and older age were independent predictors of mortality and/or lung transplantation, while CVCs did not have a significant impact on clinical outcomes. In this nationwide, register-based study, the epidemiology of IPAH involves older patients with CVCs, who seem to have less hemodynamic compromise, but worse functional impairment and are treated less aggressively with PAH pharmacotherapy.

Radiation Exposure in Pediatric Interventional Procedures.

The article is part of the series of articles on radiation protection. You can find further articles in the special section of the CVIR issue. The expanding applications of interventional procedures coupled with the potential harmful effects of ionizing radiation highlight the need to assess the delivered radiation dose and establish an effective radiation protection program, particularly in the radiosensitive pediatric population. Given the complexity and heterogeneity of interventional procedures as well as the unique characteristics of children, the management of radiation dose is proving to be quite challenging. The aim of the current article is to provide an overview of the radiation exposure in pediatric patients during interventional procedures focusing on the importance of radiation protection in the pediatric population, the reported radiation doses and the techniques of minimizing radiation dose.

Physical activity levels and self-efficacy of Greek children with congenital heart disease compared to their healthy peers.

OBJECTIVE: It remains a challenge for children with congenital heart disease (CHD) to develop healthy lifestyle behaviors. This study investigates the interrelationship of physical activity and self-efficacy levels in Greek children with corrected CHD and compares them with those of healthy controls. METHODS: A prospective cross-sectional study of 76 patients with operated CHD and 78 healthy volunteers (n = 154) was conducted. The Self Efficacy Scale and Previous Day Physical Activity Recall self-reporting questionnaires were used to assess self-efficacy and physical activity, respectively. Calculated metabolic equivalent of task (MET) provided an indication of participation in moderate and vigorous physical activities. RESULTS: Mean physical self-efficacy in children with CHD was 29.01 ± 7.28, while that in healthy volunteers was 29.04 ± 6.60 (p = 0.076). Average physical activity levels-METs in children with CHD (2.38 ± 0.85) were significantly lower than those in controls (3.1 ± 0.95). Physical self-efficacy and physical activity were positively correlated (r = 0.515, p = 0.001), thus indicating that children with higher self-efficacy engaged in more vigorous physical activities. In children with CHD, self-efficacy was quite important in predicting physical activity, thus explaining almost » of the variability in observed activities. CONCLUSION: The low physical activity levels exhibited by Greek children with corrected CHD can lead to a sedentary adult lifestyle. Improving physical self-efficacy may help encourage them and consequently increase their physical activity levels. School-based education initiatives and athletic programs can play an important role in improving this.

Fatal cardiac arrest due to an unusual fibroma of the pulmonary valve.

Fibroma, the second most common tumor in childhood, usually is a large solitary mass involving the ventricular septum or the left and rarely the right free ventricular wall. An unusual case of a 5.2-year-old boy with a large mass originating from the pulmonary valve is reported. The patient presented for evaluation of a murmur. Echocardiography and cardiac magnetic resonance imaging showed a large tumor extending from the distal right ventricular outflow tract through the pulmonary valve and into the main pulmonary artery causing moderate obstruction. The patient had a fatal cardiac arrest while awaiting surgery. The postmortem examination proved the tumor to be a fibroma.

Hybrid procedures for complex congenital cardiac lesions.

BACKGROUND: We present an alternative treatment employing a hybrid approach used in 3 patients with congenital heart disease. The goal was to provide optimal therapy by minimizing the potentially harmful effects of methods that accompany conventional surgical procedures. METHODS: Two patients aged 4 and 6 months underwent beating-heart closure of a muscular ventricular septal defect (VSD) with an occluding device. In addition, an 8-year-old patient with supraaortic, main, and branch pulmonary artery (PA) stenosis underwent conventional surgical patch augmentation of the ascending aorta and the main PA and intraoperative stenting of the branch PA stenoses. RESULTS: No patient deaths occurred. One patient developed a postoperative pneumothorax. Median intensive care unit and hospital stays for the VSD patients were 1 and 5 days and for the other patients 2 and 20 days, respectively. At median follow-up of 25 months, all patients were well and had required no further interventions. CONCLUSIONS: Patients with muscular VSD can currently be treated with the hybrid approach. Intraoperative PA stenting in addition to conventional surgical repair can be performed safely and may be complementary in patients with complex lesions.

Hospitalisations for heart failure predict mortality in pulmonary hypertension related to congenital heart disease.

OBJECTIVE: Despite the progress in the management of patients with adult congenital heart disease (ACHD), a significant proportion of patients still develop pulmonary hypertension (PH). We aimed to highlight the rate of the complications in PH-ACHD and the predicting factors of cumulative mortality risk in this population. METHODS: Data were obtained from the cohort of the national registry of ACHD in Greece from February 2012 until January 2018. RESULTS: Overall, 65 patients receiving PH-specific therapy were included (mean age 46.1±14.4 years, 64.6% females). Heavily symptomatic (New York Heart Association (NYHA) class III/IV) were 53.8% of patients. The majority received monotherapy, while combination therapy was administered in 41.5% of patients. Cardiac arrhythmia was reported in 30.8%, endocarditis in 1.5%, stroke in 4.6%, pulmonary arterial thrombosis in 6.2%, haemoptysis in 3.1% and hospitalisation due to heart failure (HF) in 23.1%. Over a median follow-up of 3 years (range 1-6), 12 (18.5%) patients died. On univariate Cox regression analysis history of HF hospitalisation emerged as a strong predictor of mortality (HR 8.91, 95% CI 2.64 to 30.02, p<0.001), which remained significant after adjustment for age and for NYHA functional class. CONCLUSIONS: Long-term complications are common among patients with PH-ACHD. Hospitalisations for HF predict mortality and should be considered in the risk stratification of this population.

Epidemiology and initial management of pulmonary arterial hypertension: real-world data from the Hellenic pulmOnary hyPertension rEgistry (HOPE).

Pulmonary arterial hypertension (PAH) is a heterogenous clinical entity with poor prognosis, despite recent major pharmacological advances. To increase awareness about the pathophysiology, epidemiology, and management of the disease, large national registries are required. The Hellenic pulmOnary hyPertension rEgistry (HOPE) was launched in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. Baseline epidemiologic, diagnostic, and initial treatment data of consecutive patients with PAH are presented in this article. In total, 231 patients with PAH were enrolled from January 2015 until April 2018. At baseline, about half of patients with PAH were in World Health Organization functional class II. The majority of patients with PAH (56.7%) were at intermediate 1-year mortality risk, while more than one-third were low-risk patients, according to an abbreviated risk stratification score. Half of patients with PAH were on monotherapy, 38.9% received combination therapy, while prostanoids were used only in 12.1% of patients. In conclusion, baseline data of the Greek PAH population share common characteristics, but also have some differences with other registries, the most prominent being a better functional capacity. This may reflect earlier diagnosis of PAH that in conjunction with the increased proportion of patients with atypical PAH could partially explain the preference for monotherapy and the limited use of prostanoids in Greece. Nevertheless, early, advanced specific therapy is strongly recommended.

Assessment of bone mineral density and markers of bone turnover in children under long-term oral anticoagulant therapy.

Oral anticoagulants antagonize vitamin K action and potentially impair the carboxylation of osteocalcin, a protein essential for normal bone matrix formation. In the present study, bone mineral density (BMD) and bone turnover markers were evaluated in 23 children under long-term oral anticoagulant therapy. BMD of the lumbar spine was assessed (Dual Energy x-ray Absorptiometry) and reported as z score. Osteoblast [bone alkaline phosphatase, osteocalcin (Gla-Oc), amino-terminal procollagen 1 extension peptide] and osteoclast (urinary calcium and deoxypyridinoline, serum cross-linked C telopeptide) activity markers were measured. Vitamin D {[25(OH) D], parathormone, calcium, phosphorus, magnesium} and vitamin K status [factors II, VII, IX, X, protein C, protein S, undercarboxylated osteocalcin (Glu-Oc)] were determined. The above parameters were also evaluated in 25 healthy controls. Patients presented with higher levels in Glu-Oc, parathormone, and bone resorption markers, lower levels in bone formation markers and 25(OH) D, whereas 52% of them showed signs of osteopenia (-1>BMD z score>-2.5). Statistical analysis demonstrated that anticoagulant therapy was an independent predictor of alterations in Glu-Oc, Gla-Oc, bone alkaline phosphatase, amino-terminal procollagen 1 extension peptide, and serum cross-linked C telopeptide levels. It seems that long-term use of coumarin derivatives may cause osteopenia in children with the risk of developing osteoporosis later in life.