Pacemaker Lead Placement via Transmural Approach in an Adult With Palliated Single Ventricle Heart Disease

Given the lack of systemic venous return to the heart, palliated single ventricle patients frequently require epicardial pacemaker implantation for management of dysrhythmias including sinus node dysfunction, atrial arrhythmias, and heart block. Repeated device hardware replacement, frequently required due to high lead thresholds or other device failure, is a challenging and significant problem for this population. 3-dimensional imaging can assist in delineating the cardiac anatomy allowing for novel approaches to intervention. We review a patient with extracardiac Fontan circulation who underwent placement of an endocardial atrial pacemaker lead via a transmural approach with a 3D-printed model used for procedural guidance.

Impact of Maternal–Fetal Environment on Mortality in Children With Single Ventricle Heart Disease

BACKGROUND Children with single ventricle heart disease have significant morbidity and mortality. The maternal–fetal environment (MFE) may adversely impact outcomes after neonatal cardiac surgery. We hypothesized that impaired MFE would be associated with an increased risk of death after stage 1 Norwood reconstruction. METHODS AND RESULTS We performed a retrospective cohort study of children with hypoplastic left heart syndrome (and anatomic variants) who underwent stage 1 Norwood reconstruction between 2008 and 2018. Impaired MFE was defined as maternal gestational hypertension, preeclampsia, gestational diabetes, and/or smoking during pregnancy. Cox proportional hazards regression models were used to investigate the association between impaired MFE and death while adjusting for confounders. Hospital length of stay was assessed with the competing risk of in‐hospital death. In 273 children, the median age at stage 1 Norwood reconstruction was 4 days (interquartile range [IQR], 3–6 days). A total of 72 children (26%) were exposed to an impaired MFE; they had more preterm births (18% versus 7%) and a greater percentage with low birth weights <2.5 kg (18% versus 4%) than those without impaired MFE. Impaired MFE was associated with a higher risk of death (hazard ratio [HR], 6.05; 95% CI, 3.59–10.21; P <0.001) after adjusting for age at surgery, Hispanic ethnicity, genetic syndrome, cardiac diagnosis, surgeon, and birth era. Children with impaired MFE had almost double the risk of prolonged hospital stay (HR, 1.95; 95% CI, 1.41–2.70; P <0.001). CONCLUSIONS Children exposed to an impaired MFE had a higher risk of death following stage 1 Norwood reconstruction. Prenatal exposures are potentially modifiable factors that can be targeted to improve outcomes after pediatric cardiac surgery.

Polymorphism of folate metabolism genes and thrombotic complications in patients with functionally single ventricle

Aim. To analyze the relationships between the carriage of polymorphic variants in the folate metabolism genes and the development of thrombotic complications in patients with single ventricle (SV) during surgical treatment.Material and Methods. A total of 102 children with SV were examined in the performed research. All patients underwent surgical hemodynamic correction of congenital heart disease (CHD). According to a retrospective chart review, thrombosis was diagnosed in 12.7 % of the examined patients with SV. The analysis of polymorphism in the MTR A2756G enzyme gene revealed significant differences between the groups of patients with a history of thrombosis and without it.Results. We found that the risk of developing thrombosis was associated with the carriage of homozygous genotype 2756AA of the MTR enzyme gene (OR = 11.21; 95% CI: 1.39–89.96; p = 0.023).

Results of aortopulmonary collateral arteries coil embolization in pre-fontan patients

Background: Aortopulmonary collateral arteries (APCAs) are common pulmonary blood supply in the group of congenital heart disease with functionally single ventricle. APCAs causes loss of aortic outflow, increase in pulmonary arterial hydrostatic pressure, reducing blood flow from the SVC and IVC to the pulmonary artery (in patients after Glenn and Fontan operations). Therefore, the closure of APCAs is vital in the management of single ventricle patients before and during Fontan operation. The technique of percutaneous APCAs occlusion with coils is a new, effective and better method of control of APCAs, excluding the need for surgical ligation of the APCAs.. Objectives: To evaluate results of aortopulmonary collateral arteries coil embolization in pre-fontan patients Methods: This is the descriptive clinical and retrospective study Result: 29 patients with average age of 7.9 underwent cardiac catheterization with APCAs coil embolization successfully before the Fontan surgery. Complication rate was very low. This method effectively reduced mean pulmonary artery pressure with statistical significance. The results of Fontan surgery of this group were better than that in other studies in the rate of diaphragmatic paralysis and chylous effusion Conclusions: The technique of percutaneous APCAs occlusion with coils before Fontan surgery is safe, effective in reducing mean pulmonary artery pressure and improving results of Fontan surgery.

REHABILITATION OF STROKE IN CYANOTIC CONGENITAL SINGLE VENTRICLE HEART DEFECT PATIENT

Congenital heart disease consists of various conditions including tetralogy of Fallot, ventricular septal defect, Epstein’s anomaly, single ventricle, etc. Among these single ventricles is one of the gravest forms of cyanotic congenital heart disease. The cardiac diagnosis is associated with an increased risk of stroke among children. Pediatric arterial ischemic stroke (AIS) is an important cause of neurologic disease in children causing disability. The 14-year-old patient came to the hospital was presented with left side hemiplegia with severe exercise intolerance due to congenital heart disease. The patient has been advised to undergo Fontan procedure for single-ventricle condition 3 years back, but due to poor socioeconomic status, parents of patients refused to do so. The primary goal was to improve bed mobility and trunk balance without developing symptoms of exercise intolerance. The intervention was started with deep and segmental breathing exercises. Proprioceptive neuromuscular facilitation and constrained induced movement therapy were added in the program in addition to passive and active movements, bed mobility, functional reeducation, trunk control exercises, and balance exercises. Combinations of all of the above therapeutic approaches lead to increased functional independence in the patient. This case reports the effectiveness of a rehabilitation program for pediatric arterial ischemic stroke with preventive guidelines for exercise intolerance.