Clinical and Echocardiographic Characteristics After Six Months of Sacubitril/Valsartan in Chagas Heart Disease – a Case Series

INTRODUCTION Incidence of congenital heart disease is 25 per thousand live birth in Bangladesh which is much higher than other countries. Tetralogy of Fallot, the commonest cyanotic heart disease (5%) and some other complex diseases with right ventricular outflow tract abnormality demand surgical correction and revision in many occasions including percutaneous intervention. As a resource constraint country, it was a difficult task to introduce percutaneous pulmonary valve implantation (PPVI) with MelodyTM. However, it was started on 12th December 2012 in Combined Military Hospital Dhaka, Bangladesh and cases performed till October 2019 were included in this series. METHODS Retrospective analysis of six cases who had PPVI with MelodyTM in Combined Military Hospital, Bangladesh. Patient with dysfunctional conduit between right ventricle (RV) and pulmonary artery causing (a) Symptoms of exceptional dyspnoea of various grade (NYHA II,III, IV) (b) RVEVD >150 ml/m2 ±regurgitant fraction >40% (c) RVOT peak instantaneous gradient > 30 mm Hg. (d) RV dysfunction (RVEF<40%) were accepted for the procedure and outcome were analyzed. RESULTS Mean age was 9.56 ± 2.96 years, weight was 28.75 ± 8.61 kg, height was 137.5 ± 17.52 cm. Mean age at surgery was 4.25 ± 2.72 years. Female were 66.66%. Aortic homograft was used in 66.66% cases. Eighteen mm Ensemble was used in four (66.66%) cases and 20 mm and 22 mm in one each. Immediate result was excellent with no residual PS in two cases and negligible residual flow acceleration across pulmonary valve in four cases. No PR seen in all except one. One patient developed Bacterial endocarditis after 3 years and was treated. CONCLUSION Aim of PPVI is to prolong the life expectancy of conduits which were placed surgically from right ventricle to pulmonary artery. In our case series, we found that Melody valve is functioning well without any complications like infective endocarditis or stent fracture. KEYWORDS PPVI, MelodyTM, RVOT, Outcome

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Cardiac transplantation in adults with congenital heart disease: A single center case series

Clinical Transplantation ◽

10.1111/ctr.14430 ◽

2021 ◽

Author(s):

Christopher A. Heid ◽

Raghav Chandra ◽

Charles Liu ◽

Jessica Pruszynski ◽

Mitri K. Khoury ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Cardiac Transplantation ◽

Congenital Heart ◽

Case Series ◽

Single Center

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Contemporary use of Selexipag in pulmonary arterial hypertension associated with congenital heart disease: a case series

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytaa320 ◽

2020 ◽

Author(s):

Sarah Blissett ◽

David Blusztein ◽

Vaikom S Mahadevan

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Pulmonary Arterial Hypertension ◽

Side Effects ◽

Arterial Hypertension ◽

Exercise Capacity ◽

Congenital Heart ◽

Pulmonary Veins ◽

Case Series ◽

Pulmonary Arterial

Abstract Background There are significant risks of parenteral prostacyclin use in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), which may limit their use. Selexipag is an oral, selective prostacyclin analogue that has been shown to reduce disease progression and improve exercise capacity in patients with PAH-CHD. Administering Selexipag in patients with PAH-CHD could potentially overcome some of the risks of parenteral therapy while improving clinical outcomes. Case summary We report five cases highlighting the clinical uses of Selexipag in patients with PAH-CHD. In the first two cases, Selexipag was initiated as part of a Treat-to-close strategy. In the third case, initiation of Selexipag improved symptoms and objective exercise capacity in a patient with Eisenmenger syndrome. In the fourth and fifth cases, rapid cross-titration protocols were used to transition from parenteral prostacyclins to Selexipag. In the fourth case, Selexipag was initiated in the context of significant side effects limiting parenteral prostacyclin use. In the fifth case, Selexipag was used to down-titrate from parenteral prostacyclins following closure of a sinus venosus atrial septal defect and redirection of anomalous pulmonary veins. Discussion Selexipag is a promising oral therapy for patients with at various stages of the spectrum of PAH-CHD to improve symptoms, exercise capacity and, in some cases, haemodynamics. Our cases also highlight practical aspects of Selexipag use including targeting the individualized maximally tolerated dose for each patient, managing side effects and managing dose interruptions.

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A systematic review examining the clinical and health-care outcomes for congenital heart disease patients using home monitoring programmes

Journal of Telemedicine and Telecare ◽

10.1177/1357633x20984052 ◽

2021 ◽

pp. 1357633X2098405

Author(s):

Rachel Crawford ◽

Ciara Hughes ◽

Sonyia McFadden ◽

Jacqui Crawford

Keyword(s):

Systematic Review ◽

Health Care ◽

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Case Series ◽

Home Monitoring ◽

Risk Of Bias ◽

Health Care Outcomes ◽

Care Outcomes

Objectives This review aimed to present the clinical and health-care outcomes for patients with congenital heart disease (CHD) who use home monitoring technologies. Methods Five databases were systematically searched from inception to November 2020 for quantitative studies in this area. Data were extracted using a pre-formatted data-collection table which included information on participants, interventions, outcome measures and results. Risk of bias was determined using the Cochrane Risk of Bias 2 tool for randomised controlled trials (RCTs), the Newcastle–Ottawa Quality Assessment Scale for cohort studies and the Institute of Health Economics quality appraisal checklist for case-series studies. Data synthesis: Twenty-two studies were included in this systematic review, which included four RCTs, 12 cohort studies and six case-series studies. Seventeen studies reported on mortality rates, with 59% reporting that home monitoring programmes were associated with either a significant reduction or trend for lower mortality and 12% reporting that mortality trended higher. Fourteen studies reported on unplanned readmissions/health-care resource use, with 29% of studies reporting that this outcome was significantly decreased or trended lower with home monitoring and 21% reported an increase. Impact on treatment was reported in 15 studies, with 67% of studies finding that either treatment was undertaken significantly earlier or significantly more interventions were undertaken in the home monitoring groups. Conclusion The use of home monitoring programmes may be beneficial in reducing mortality, enabling earlier and more timely detection and treatment of CHD complication. However, currently, this evidence is limited due to weakness in study designs.

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