Sacubitril/Valsartan in Adult Congenital Heart Disease Patients With Chronic Heart Failure - A Single Centre Case Series and Call for an International Registry.

BACKGROUND: An improvement in life expectancy in patients suffering from adult congenital heart disease (ACHD) has corresponded with a rise in heart failure incidence within this group. An area that has not been addressed in ACHD heart failure guidelines has been the use of combined inhibition of angiotensin receptor-neprolysin pathways. This case series sought to demonstrate tolerability and 6-month outcome measures of sacubitril/valsartan use in ACHD patients with a severely impaired systemic ventricle. METHODS: A prospective cohort analysis of ACHD patients between December 2016 and September 2017 with severe systemic ventricular systolic dysfunction, New York Heart Association (NYHA) class II-III symptoms and eligible for commencement on a sacubitril/valsartan regime was undertaken. RESULTS: Five (5) consecutive patients were included in this cohort review, 80% male, mean age 41.8 (±19) years and mean systemic ventricular ejection fraction 27% (±3.6%). Two (2) patients with pre-existing D-TGA and atrial baffle repair, one patient with Tetralogy of Fallot repair and pulmonary valve replacement (PVR), one patient with left atrial isomerism and partial atrioventricular (AV) canal defect repair and mitral valve replacement (MVR) and the last patient had biventricular repair for pulmonary atresia with MVR and PVR. Forty per cent (40%) of patients had a systemic right ventricle. All patients had NYHA functional class of ≥II, were on optimal tolerated doses ACE-I or ARB prior to sacubitril/valsartan combination. Six (6) months post commencement of sacubitril/valsartan patients experienced a mean improvement of one functional class. CONCLUSIONS: Our experience suggests that sacubitril/valsartan therapy is well tolerated in ACHD heart failure patients and is associated with improvement in functional class.

Adult congenital heart disease nurse coordination: Essential skills and role in optimizing team-based care a position statement from the International Society for Adult Congenital Heart Disease (ISACHD).

BACKGROUND: Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with congenital heart disease (CHD) worldwide. Among ISACHD's objectives is to "promote a holistic team-based approach to the care of the adult with CHD that is comprehensive, patient-centered, and interdisciplinary" (http://www.isachd.org). This emphasis on team-based care reflects the fact that adults with CHD constitute a heterogeneous population with a wide spectrum of disease complexity, frequent association with other organ involvement, and varied co-morbidities and psychosocial issues. METHODS: Recognizing the vital role of the adult CHD (ACHD) nurse coordinator (ACHD-NC) in optimizing team-based care, ISACHD established a task force to elucidate and provide guidance on the roles and responsibilities of the ACHD-NC. Acknowledging that nursing roles can vary widely from region to region based on factors such as credentials, scopes of practice, regulations, and local culture and tradition, an international panel was assembled with experts from North America, Europe, East Asia, and Oceania. The writing committee was tasked with reviewing key aspects of the ACHD-NC's role in team-based ACHD care. RESULTS/CONCLUSION: The resulting ISACHD position statement addresses the ACHD-NC's role and skills required in organizing, coordinating, and facilitating the care of adults with CHD, holistic assessment of the ACHD patient, patient education and counseling, and support for self-care management and self-advocacy.

Outcomes Following Melody Transcatheter Pulmonary Valve Implantation for Right Ventricular Outflow Tract Dysfunction in Repaired Congenital Heart Disease: First Reported Australian Single Centre Experience.

BACKGROUND: Transcatheter pulmonary valve implantation (TPVI) with the Melody® transcatheter pulmonary valve (TPV) has demonstrated good haemodynamic and clinical outcomes in the treatment of right ventricular outflow tract (RVOT) conduit dysfunction in patients with repaired congenital heart disease CHD. We present the first Australian single centre experience of patients treated with Melody TPV. METHOD: A prospective, observational registry was developed to monitor clinical and haemodynamic outcomes in patients with RVOT dysfunction treated with the Melody TPV (Medtronic Inc, Minneapolis, United States). RESULTS: Seventeen patients underwent TPVI with Melody TPV at The Prince Charles Hospital between January 2009 and February 2016 with a median (range) age of 34 (R: 15-60). Fifteen (88%) were NYHA Class 2 dyspnoea and 11 (59%) had corrected Tetralogy of Fallot. Indication for TPVI was stenosis in eight (47%), regurgitation in two (12%) and mixed dysfunction in seven (41%). Device implantation was successful in all patients. Peak RVOT gradient was significantly reduced and there was no significant regurgitation post procedure. There was one (6%) major procedural adverse event and two (12%) major adverse events at last recorded follow-up. There were no patient deaths. Follow-up cardiac magnetic resonance imaging revealed a significant reduction in indexed right ventricular end diastolic volume. CONCLUSION: This study confirms the safety and effectiveness of TPVI with Melody TPV for RVOT dysfunction in repaired CHD.

A retrospective study of judicial encounters of young adults with congenital heart disease.

Young people with congenital heart disease are often marginalised and may compensate for restricted physical and social abilities by substance abuse or risk-taking behaviour. We report on the judicial encounters of patients in an Adult Congenital Heart unit. Fifteen patients had court appearances and all were male. Seven served jail sentences, including four indigenous adolescents. All had multiple interventions for their cardiac conditions and four are now deceased. The majority of patients had mental health issues and substance misuse. These patients need psychological and medical support, and help with judicial authorities. Recommendations for ongoing care are provided.