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Right Ventricle in Structural and Functional Left Heart Failure in Children

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The Right Ventricle in Health and Disease

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Abstract

In this chapter we describe the role of the right ventricle in structural and functional left ventricular failure in children. The ventriculo-ventricular interaction of the sub-pulmonary and sub-aortic ventricle is discussed in the context of (1) the right ventricle in left heart failure with decreased ejection fraction, (2) the right ventricle in restrictive cardiomyopathy or a borderline left ventricle and heart failure with preserved ejection fraction.

Novel therapeutic strategies are presented: pulmonary artery banding in dilated cardiomyopathy in young children, hybrid approaches combining atrioseptostomy and reverse Potts-shunt in patients with borderline left ventricle and right ventricular failure as well as atrioseptostomy in out-of-proportion left atrial pressure to improve the function of restrictive cardiomyopathy or left ventricular diastolic dysfunction.

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Author information

Authors and Affiliations

  1. Department of Pediatric Cardiology, Justus Liebig University, Feulgenstrasse 10-12, Giessen, Hessen, Germany

    Dietmar Schranz

  2. Pediatric Heart Center, Justus-Liebig-University, Feulgenstr 10-12, Giessen, Germany

    Heiner Latus M.D. & Christian Apitz M.D.

Authors
  1. Dietmar Schranz
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  2. Heiner Latus M.D.
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  3. Christian Apitz M.D.
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Corresponding author

Correspondence to Dietmar Schranz .

Editor information

Editors and Affiliations

  1. Department of Medicine, Virginia Commonwealth University, Richmond, Virginia, USA

    Norbert F. Voelkel

  2. Pediatric Heart Center, Justus-Liebig University, Giessen, Germany

    Dietmar Schranz

Electronic Supplementary Material

Below is the link to the electronic supplementary material.

2D-ECHO images of an infant with severe dilated cardiomyopathy (DCM) and severe mitral regurgitation, but preserved systolic right ventricular function. The enormous dilatation of the left ventricle (z-score +7) leads to a ventricular septum shift to the right with consecutive compression of the right ventricular dimension and inhibiting diastolic inflow despite small PFO (persistent foramen ovale); dilated left ventricle (left); and color Doppler of mitral regurgitation, and PFO flow (right). (AVI 8050 kb)

Four-chamber MRI images of an infant with severe DCM before (left), and following 4 months follow-up after PAB; clinical functional class improvement of Ross class IV to class II was associated by the left ventricular structural and functional improvement. The enormous dilatation of the impaired, dyssynchron LV function with compression of the right ventricle (left) changed by PAB to a better filled right ventricle despite hypertophic reaction, and reduction of LV preload (normalized left atrium dimension), together with IVS left-side shifting improved LV ejection fraction accompanied by synchrony of the contraction of the less dilated but more hypertrophied LV, respectively; the left-sided hypertrophy involved as expected the IVS, but surprisingly the LV posterior free wall, too. (AVI 1902 kb)

310219_1_En_7_MOESM3_ESM.avi

Four-chamber MRI imaging is shown of a newborn with borderline left ventricle before duct stenting and bilateral pulmonary banding was performed as a first palliation. (AVI 1163 kb)

310219_1_En_7_MOESM4a_ESM.avi

Angiographies of a stented duct show the right-to-left shunt ((a) in LAO 30° view, and (b) in lateral 90°) in an infant with borderline left ventricluar structures and associated suprasystemic pulmonary artery pressures (PAPs). Duct stenting allowed right ventricular decompression and clinical improvement; months later when a left-to-right shunt across the stented duct was observed the stent was percutaneously closed by an Amplatzer duct occluder followed by surgical repair of the mitral valve stenosis, sub-aortic stenosis and closure of a small ventricular septal defect, respectively. (AVI 761 kb)

310219_1_En_7_MOESM4b_ESM.avi

(AVI 1091 kb)

(a) Transesophageal imaging shows an extreme dilated left atrium caused by a restrictive left ventricle with preserved ejection fraction. (b) Shows an interatrial communication which was performed in the same patient (a) to influence the post-capillary induced pulmonary hypertension as well as atrial fibrillation by slightly decompression of the left atrium. Three months later PAPs were normalized. (AVI 6440 kb)

(AVI 9823 kb)

310219_1_En_7_MOESM6_ESM.avi

Demonstrates stent placement within the interatrial septum as one part as a strategy to treat end-staged suprasystemic pulmonary hypertension with right ventricular failure caused by left ventricular structural disease (see text). (AVI 5598 kb)

Video 7.3

Four-chamber MRI imaging is shown of a newborn with borderline left ventricle before duct stenting and bilateral pulmonary banding was performed as a first palliation. (AVI 1163 kb)

Video 7.4

Angiographies of a stented duct show the right-to-left shunt ((a) in LAO 30° view, and (b) in lateral 90°) in an infant with borderline left ventricluar structures and associated suprasystemic pulmonary artery pressures (PAPs). Duct stenting allowed right ventricular decompression and clinical improvement; months later when a left-to-right shunt across the stented duct was observed the stent was percutaneously closed by an Amplatzer duct occluder followed by surgical repair of the mitral valve stenosis, sub-aortic stenosis and closure of a small ventricular septal defect, respectively. (AVI 761 kb)

(AVI 1091 kb)

Video 7.5

(a) Transesophageal imaging shows an extreme dilated left atrium caused by a restrictive left ventricle with preserved ejection fraction. (b) Shows an interatrial communication which was performed in the same patient (a) to influence the post-capillary induced pulmonary hypertension as well as atrial fibrillation by slightly decompression of the left atrium. Three months later PAPs were normalized. (AVI 6440 kb)

Video 7.6

Demonstrates stent placement within the interatrial septum as one part as a strategy to treat end-staged suprasystemic pulmonary hypertension with right ventricular failure caused by left ventricular structural disease (see text). (AVI 5598 kb)

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Schranz, D., Latus, H., Apitz, C. (2015). Right Ventricle in Structural and Functional Left Heart Failure in Children. In: Voelkel, N., Schranz, D. (eds) The Right Ventricle in Health and Disease. Respiratory Medicine. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-1065-6_7

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  • DOI: https://doi.org/10.1007/978-1-4939-1065-6_7

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  • Online ISBN: 978-1-4939-1065-6

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