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Pulmonary hypertension associated with congenital heart disease 

Pulmonary hypertension associated with congenital heart disease
Pulmonary hypertension associated with congenital heart disease

Ryan Coleman

, and Corey Chartan

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date: 23 April 2021

The spectrum of pulmonary hypertension associated with congenital heart disease (PH-CHD) is broad and complex. The most common aetiologies are related to left-to-right shunts, resulting in volume loading of the pulmonary circulation (precapillary pulmonary hypertension) and left-sided obstructive disease (post-capillary pulmonary hypertension). However, in addition to these basic classifications are patients with absent pulmonary arteries, large septal defects, duct-dependent lesions, cardiomyopathies, and, perhaps the most challenging, patients with single ventricle physiology lacking a subpulmonic ventricle. It is therefore imperative that physicians taking care of children with PH-CHD are well versed not only in the different physiologies present in these children, but also in those that result from their respective surgical palliations as well and how these flows and pressures relate to their pulmonary vascular disease burden. This chapter presents the case of a child with trisomy 21 and congenital heart disease who presented late to medical care and required significant modulation of her pulmonary vascular bed to successfully undergo surgical correction. The child’s clinical course, an overview of PH-CHD and its diagnosis and management, and important therapeutic considerations are presented.

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