Strategies to Aid Successful Transition of Adolescents with Congenital Heart Disease: A Systematic Review.

The majority of patients born with congenital heart disease (CHD) need lifelong surveillance with serial clinical attendance and examinations. However, loss of follow-up (namely no documented follow-up for 3 years or more) is a recognised common problem since it is often related to remarkable worsening in the health of CHD patients with increased morbidity and mortality. Transitioning from paediatric to adult care has proven to be the most vulnerable point in the care of these subjects. As such, a systematic review was carried out to ask the following questions: What is the percentage of loss of follow-up worldwide? Are there regional fluctuations in the percentage? Is there a link between loss of follow-up and the complexity of CHD? What strategies should be employed to lower the risk of discontinuity in care? The most recent worldwide averaged loss of follow-up is 26.1%, with significant fluctuations across continents and countries. This percentage is even higher (31.9%) when one includes all untraceable patients, presuming that they are not having any cardiac follow-up. The highest discontinuity of care was reported in the USA and in patients with simple CHD. Planning the rules of transition seems to be one of the most reliable tools to minimise the number of CHD patients who are lost in transition. Recalling patients, with general practitioners who are crucial in readdressing half of the lost to follow-up CHD patients to adult CHD specialists, and a good relationship between paediatric cardiologists and the adult CHD team are two other valuable strategies in aiding successful transition.

Idiopathic Pulmonary Arterial Hypertension in Paediatrics Represents Still a Serious Challenge: A Case Series Study.

INTRODUCTION: Paediatric pulmonary hypertension (PH) represents a heterogeneous illness that is responsible for high morbidity and mortality if left without treatment. Idiopathic pulmonary arterial hypertension (IPAH) is a subtype of PAH rarely seen in paediatrics. Limited long-term data are available. METHODS: Over a period of 20 years, 10 paediatric patients were enrolled at two tertiary centres. Their clinical, echocardiographic, and right heart catheterisation (RHC) features and outcome were evaluated. RESULTS: The mean age at first diagnosis was 5.7 ± 5.7 years. The age at the last follow-up was 12.4 ± 6.1 years. The average follow-up was 6.6 ± 0.8 years. There was a female prevalence of 60% (p < 0.05) in this case series. Regarding the NYHA functional class, 80% of IPAH subjects were in class III or IV. The mean saturation was 91 ± 5%. In this regard, 70% of the patients were on a combination of three drugs, with sildenafil (90%) included. On echocardiography, longitudinal right ventricular contractility (TAPSE) was slightly reduced (13.4 ± 2.6 mm), whilst RVSP was severely elevated (101 ± 19 mmHg). The RHC data showed that mPAP was 61.8 ± 23.1 mmHg (p = 0.0017 with RVSP on echocardiography), mRAP was 10.7 ± 3.8 mmHg, CI was 2.6 ± 1 L·min-1·m-2, PVRi was 16.8 ± 12.6 WU·m2, and SVO2 was 63.6 ± 14.8%. Regarding the outcome, two male IPAH patients (20%) died, and 50% underwent lung transplant or were on transplant assessment or already on the waiting list for lung transplantation. One patient underwent a ductus arteriosus stenting (reverse Potts shunt) and another underwent atrial septostomy and stenting. CONCLUSIONS: Notwithstanding the progress in medical therapy, IPAH continues to represent a serious challenge, particularly in the paediatric population, with the need for lung transplantation and significant mortality.

Planning Transition of Care for Adolescents Affected by Congenital Heart Disease: The Irish National Pathway.

At some point in their life, adolescent patients with a congenital heart disease (CHD) transition from paediatric services to adult care facilities. The process is not without any risks, as it is often linked with a significantly progressive deterioration in adolescents' health and loss of follow-up. In fact, transition patients often encounter troubles in finding a care giver who is comfortable managing their condition, or in re-establishing trust with the new care provider. Planning the rules of transition is pivotal in preventing these risks. Unfortunately, the American and European guidelines on CHD provide just generic statements about transition. In a recently published worldwide inter-societies consensus document, a hybrid model of transition, which should be adapted for use in high- and low- resource settings, has been suggested. Currently, in literature there are a few models of transition for CHD patients, but they are by far local models and cannot be generalized to other regions or countries. This paper describes the Irish model for transition of care of CHD patients. Due to the peculiarity of the healthcare organization in the Republic of Ireland, which is centralized with one main referral centre for paediatric cardiology (in Dublin, with a few smaller satellite centres all around, according to the "hub and spoke" model) and one centre for adult with CHD (in Dublin), the model can be considered as a national one and the first to be released in the old continent.

Ventricular tachycardia triggered by the first dose of an adenoviral vector-based COVID-19 vaccine in an adult patient with congenital heart disease.

A unique adverse event of adenoviral COVID-19 vaccine in an adult patient with congenital heart disease is reported.

Micra pacemaker in adult congenital heart disease patients: A case series.

INTRODUCTION: Implantation of transvenous endocardial or epicardial pacemakers presents specific challenges in adult congenital heart disease (ACHD) patients. Micra leadless permanent pacemakers (Micra PPM) may overcome some of these difficulties. METHODS: Fifteen ACHD patients who underwent Micra PPM insertion were retrospectively evaluated. RESULTS: Males 53.3%. Mean age at study: 37.5 ± 10.7 years. Mean age at Micra PPM insertion: 35.5 ± 11.0 years. Mean follow-up so far: 2.0 ± 0.3 years. Concerning the ACHD patients, 6.7% had a simple defect, 66.6% had a moderately complex defect, 26.7% were complex. Four patients (26.7%) had a previous PPM implantation. Three patients (20%) had a systemic right ventricle. Two patients (13.3%) had a single ventricle physiology. Five (33.3%) had Trisomy 21. The most commonly used Micra PPM modality was single chamber ventricular pacemaker (73.3%). Mean threshold post implantation was 0.48 V [range: 0.25-1.13 V], while mean threshold at 6 months control was 0.60 V [range: 0.38-1.13 V] (p = ns). Mean R wave postimplantation was 10.3 V [range: 3.25-19.4 V], whilst mean R wave at 6 months follow-up was 10.1 V [range: 3.5-19.0 V] (p = ns). No major peri and postprocedural complications were encountered. CONCLUSIONS: since ACHD patients are living longer and surviving into adulthood, the incidence of conduction disorders continues to increase, as part of the natural history of some lesions or as early or late complication of surgery. The Micra leadless PPM can be successfully implanted in ACHD patients and have significant theoretical advantages. They should be considered when transvenous and epicardial pacing are either contraindicated or represent an otherwise suboptimal approach.

Cardiac MRI prior to liver biopsy in a Fontan patient: A case report.

The management of adults with complex congenital heart disease requires a multidisciplinary team (Heart Team).