Exploring the implementation of key nursing roles in children's cardiac services.

BACKGROUND: Children's cardiac nursing roles have changed over the past decade. Royal College of Nursing (RCN) guidance and NHS England standards have been published with the aim of standardising and enhancing nursing care for children and young people with congenital heart disease (CHD) and their families. AIM: To explore the breath of implementation of key nursing roles in children's cardiac services across the UK and Ireland and to determine whether the roles met the RCN guidance and the NHS England standards. METHOD: A cross-sectional survey design was used. The 150 members of the Congenital Cardiac Nurses Association (CCNA) were invited via email to participate and were sent a link to an online survey. FINDINGS: Of the 150 potential respondents, 31 completed the survey. Overall, respondents believed that the RCN guidance had been implemented effectively and that children's cardiac nursing roles matched the RCN's example job descriptions. Respondents' comments suggested that implementation of the NHS England standards had been challenging and that progress in setting up key roles such as lead nurse, cardiac nurse educator and children's cardiac nurse specialist had been slow. Respondents felt that political and financial factors adversely affected recruitment. CONCLUSION: Since publication of the NHS England standards there has been some progress in the implementation, in children's cardiac services, of key nursing roles such as lead nurse and innovative nursing roles such as advanced nurse practitioner and research nurse. The findings of this study have informed the latest edition of the RCN guidance, which now includes the role of senior research nurse.

Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study.

BACKGROUND: late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. METHODS AND RESULTS: we conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. CONCLUSIONS: preoperative clinical and morphological features are important risk factors for postoperative PVO and survival.

Visual Loss Post Ross Procedure in an Adolescent With Newly Diagnosed Mucopolysaccharidosis Type II.

Perioperative visual loss is a rare but serious complication after cardiac surgery. The etiology is not fully understood, and there is no consensus on the optimal management of this condition. A 15-year-old male patient developed severe visual impairment attributed to nonarteritic anterior ischemic optic neuropathy after a Ross aortic root replacement procedure. A new diagnosis of the lysosomal storage disorder, mucopolysaccharidosis type II (Hunter syndrome), was subsequently made, raising questions about the pathogenesis of this devastating postoperative complication.

Total anomalous pulmonary venous connection: outcome of postoperative pulmonary venous obstruction.

OBJECTIVE: Pulmonary venous obstruction (PVO) is an important cause of late mortality in total anomalous pulmonary venous connection (TAPVC). We aimed to describe current practices for the management of postoperative PVO and the efficacy of the different interventional procedures. METHODS: We conducted a retrospective international collaborative population-based study involving 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. Patients with TAPVC born between January 1, 1998, and December 31, 2004, were identified. Patients with functionally univentricular circulation or atrial isomerism were excluded. All available data and images were reviewed. RESULTS: Of 406 patients undergoing repair of TAPVC, 71 (17.5%) had postoperative PVO. The diagnosis was made within 6 months of surgery in 59 (83%) of the 71 patients. In 12, serial imaging documented change in appearance of the pulmonary veins. Good-sized pulmonary veins can progress to diffusely small veins and rarely atresia. Patients presenting after 6 months had less severe disease; all are alive at most recent follow-up. Fifty-six (13.8%) of 406 patients underwent intervention for postoperative PVO: 44 had surgical treatment and 12 had an initial catheter intervention. One half underwent 1 or more reinterventions. Three-year survival for patients with postoperative PVO was 58.7% (95% confidence intervals, 46.2%-69.2%) with a trend that those having a surgical strategy did better (P = .083). Risk factors for death included earlier presentation after TAPVC repair, diffusely small pulmonary veins at presentation of postoperative PVO, and an increased number of lung segments affected by obstruction. CONCLUSIONS: Postoperative PVO tends to appear in the first 6 months after TAPVC repair and can be progressive. Early intervention for PVO may be indicated before irreversible secondary changes occur.