Immediate Therapeutic Outcomes and Medium-term Follow-up of Percutaneous Balloon Pulmonary Valvuloplasty in Infants with Pulmonary Valve Stenosis： A Single-center Retrospective Study

Background： Percutaneous balloon pulmonary valvuloplasty （PBPV） is the preferred therapy for pulmonary valve stenosis （PVS）. This study retrospectively reviewed recent PBPV outcomes in infants with PVS. The aim of this study was to evaluate factors associated with immediate therapeutic outcomes and restenosis during medium-term follow-up. Methods： The study included 158 infants with PVS who underwent PBPV from January 2009 to July 2015. Demographic characteristics and patient records were reviewed, including detailed hospitalization parameters, hemodynamic data before and immediately after balloon dilation, cineangiograms,and echocardiograms before PBPV and at each follow-up.All procedures were performed by more than two experienced operators. Results： Immediately after balloon dilation, the pressure gradient across the pulmonary valve decreased from 73.09 ± 21.89 mmHg （range： 43-151 mmHg） to 24.49 ± 17.00 mmHg （range： 3-92 mmHg; P 〈 0.001） and the right ventricular systolic pressure decreased from 95.34 ± 23.44 mmHg （range： 60-174 mmHg） to 52.07 ± 18.89 mmHg （range： 22-134 mmHg; P 〈 0.001）. Residual transvalvular pressure gradientsof67.31±15.19mmHg（range：50-92mmHg）werefoundin8.2%ofpatients,indicatingpoortherapeuticeffects;6.4%ofpatientshad variable-staged restenosis at follow-up and 3.8% underwent reintervention by balloon dilation or surgical repairs. Further analysis demonstrated that the balloon/annulus ratio showed statistically significant differences（P 〈 0.05） among groups with different therapeutic effects and between the restenosis and no-stenosis groups. Binary logistic regression analysis further revealed that higher balloon/annulus ratio （odds ratio： 0.005, 95% confidence interval： 0-0.39） was an independent protective factor for restenosis. The rate of severe complications was 1.9%. Conclusions： PBPV is a definitive therapy for infants with PVS based on its effectiveness, feasibility, and safety. Restenosis upon medium-term follow-up is relatively rare.

Transcatheter pulmonic valve implantation: Techniques, current roles, and future implications

Right ventricular outflow tract(RVOT)obstruction is present in a variety of congenital heart disease states including tetralogy of Fallot,pulmonary atresia/stenosis and other conotruncal abnormalities etc.After surgical repair,these patients develop RVOT residual abnormalities of pulmonic stenosis and/or insufficiency of their native outflow tract or right ventricle to pulmonary artery conduit.There are also sequelae of other surgeries like the Ross operation for aortic valve disease that lead to right ventricle to pulmonary artery conduit dysfunction.Surgical pulmonic valve replacement(SPVR)has been the mainstay for these patients and is considered standard of care.Transcatheter pulmonic valve implantation(TPVI)was first reported in 2000 and has made strides as a comparable alternative to SPVR,being approved in the United States in 2010.We provide a comprehensive review in this space–indications for TPVI,detailed procedural facets and up-to-date review of the literature regarding outcomes of TPVI.TPVI has been shown to have favorable medium-term outcomes free of reinterventions especially after the adoption of the practice of pre-stenting the RVOT.Procedural mortality and complications are uncommon.With more experience,recognition of risk of dreaded outcomes like coronary compression has improved.Also,conduit rupture is increasingly being managed with transcatheter tools.Questions over endocarditis risk still prevail in the TPVI population.Head-to-head comparisons to SPVR are still limited but available data suggests equivalence.We also discuss newer valve technologies that have limited data currently and may have more applicability for treatment of native dysfunctional RVOT substrates.

Anesthetic management of a child with Cornelia de Lange Syndrome undergoing open heart surgery:A case report

BACKGROUND Cornelia de Lange syndrome(CdLS)is a congenital multisystemic genetic disorder.The expected lifespan of children with this disorder has been prolonged in parallel with the advances in medicine in recent years.However,they still more frequently undergo cardiac surgery.There are some challenges for clinicians when faced with CdLS patients.We present the perioperative management of a child with CdLS undergoing open-heart surgery.CASE SUMMARY Severe pulmonic and subpulmonic valvular stenosis,enlargement of the right side of the heart,mild tricuspid regurgitation,atrial septal defect,and patent ductus arteriosus were diagnosed in a 14-month-old boy with manifested cyanosis,developmental delay,and malnutrition.Attempted balloon valvuloplasty was unsuccessful due to a severe stenotic pulmonary valve,therefore it was decided to perform an open surgical repair.Following a successful and uncomplicated intraoperative course,the patient was extubated on postoperative day 5,and adrenalin and dopamine infusions were gradually decreased and stopped on postoperative days 6 and 10,respectively.Moderate laryngomalacia and suboptimal vocal cord movements were diagnosed,and tracheotomy and percutaneous endoscopic gastrostomy were performed under general anesthesia in the same session at postoperative day 32.The patient was discharged on postoperative day 85 after a challenging postoperative period with additional airway and nutritional problems.CONCLUSION This is the first report of the perioperative anesthetic and clinical management of a CdLS patient undergoing open-heart surgery.