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Adult congenital heart disease: left-sided obstructive lesions
  1. Christiane Haeffele
  1. Division of Cardiovascular Medicine, Stanford University, Stanford, California, USA
  1. Correspondence to Dr Christiane Haeffele, Stanford University, Stanford, CA 94304, USA; haeffele{at}stanford.edu

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Learning objectives

  • To know the categories of left-sided obstructive lesions.

  • To be able to recognise the presentation of a left-sided obstruction in adults.

  • To know the long-term follow-up requirements of care of left-sided obstructive lesions.

  • To know when a defect requires invasive correction.

Introduction

Congenital obstruction of the left heart encompasses a range of stenotic lesions that can occur in isolation or as a series of multilevel obstructions. Left ventricular outflow tract (LVOT) defects typically include subaortic stenosis (SubAS), valvular aortic stenosis, supravalvular aortic stenosis (SupAS) and coarctation of the aorta (CoA). They are often associated with other congenital anomalies, including left-sided obstruction due to left atrial obstruction or congenital mitral stenosis. The constellation of a series of left-sided obstructive lesions, including supravalvular mitral ring (SVMR), parachute mitral valve, SubAS, CoA, and bicuspid aortic valve (BAV), is known as the Shone syndrome. Shone syndrome is rare, with only case reports and small case series published. This article is intended as a contemporary review of the causes, manifestations, treatments and outcomes of left-sided obstruction in the context of a patient with adult congenital heart disease (ACHD).

Obstruction to left ventricular inflow leads to higher atrial filling pressures and can predispose to pulmonary oedema. Chronic elevation in atrial filling pressures leads to elevated pulmonary venous pressures. Over time, chronic pulmonary venous hypertension results in pulmonary vascular remodelling, and pulmonary arterial hypertension can develop (WHO group II pulmonary hypertension).

Obstruction to left ventricular outflow causes a pressure load on the left ventricle, leading to adverse remodelling and hypertrophy as compensation. However, the increase in ventricular mass over time may be insufficient to offset wall stress, resulting in left ventricular systolic dysfunction and decreased cardiac output.1 Chronic pressure load also causes diastolic dysfunction and myocardial damage. Some studies suggest that the proximity of the obstruction to …

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Footnotes

  • Twitter @CHaeffele_MD

  • Contributors CH planned, researched and wrote the review of the subject described in the article.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

  • Provenance and peer review Not commissioned; internally peer reviewed.

  • Author note References which include a * are considered to be key references.

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