Intrauterine growth restriction is not associated with decreased exercise capacity in adolescents with congenital heart disease

Annually, there > 30,000 infants are born with congenital heart defects; in different populations, the prevalence of congenital heart disease (CHD) varies from 2.4 to 14.15%. Women with CHD planning pregnancy are at increased risk of heart failure, arrhythmias, cerebrovascular disease, and embolism. In such patients, pregnancy course is complicated by intrauterine growth restriction, pre-eclampsia, and preterm birth. Their newborns generally have a low birth weight and high risk of congenital malformations including heart defects. European Society of Cardiology (ESC) developed risk assessment-based guidelines to optimise the management of pregnant women with CHD. This approach requires a cooperation of obstetrician-gynecologists, general practitioners, and cardiologists.

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Tetrasomy of 11q13.4-q14.3 due to an intrachromosomal triplication associated with paternal uniparental isodisomy for 11q14.3-qter, intrauterine growth restriction, developmental delay, corpus callosum dysgenesis, microcephaly, congenital heart defects and facial dysmorphism

Taiwanese Journal of Obstetrics and Gynecology ◽

10.1016/j.tjog.2020.11.027 ◽

2021 ◽

Vol 60 (1) ◽

pp. 169-172

Author(s):

Chih-Ping Chen ◽

Shuan-Pei Lin ◽

Schu-Rern Chern ◽

Peih-Shan Wu ◽

Shin-Wen Chen ◽

...

Keyword(s):

Corpus Callosum ◽

Developmental Delay ◽

Congenital Heart Defects ◽

Intrauterine Growth Restriction ◽

Congenital Heart ◽

Heart Defects ◽

Growth Restriction ◽

Facial Dysmorphism ◽

Intrauterine Growth ◽

Uniparental Isodisomy

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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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