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      Right Ventricular Compression Observed in Echocardiography from Pectus Excavatum Deformity

      case-report

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          Abstract

          Pectus excavatum exists as varying anatomic deformities and compression of the right heart by the chest wall can lead to patient symptoms including dyspnea and chest pain with exertion. Echocardiography can be difficult but is critical to the evaluation and diagnosis of this patient population. Modifying standard views such as biplane transthoracic and 3-D transesophageal views may be necessary in some patients due to limitations from the abnormal anatomy of the deformed anterior chest wall. Apical four-chamber views when seen clearly can usually visualize any extrinsic compression to the right ventricle of the heart.

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          Anatomical, histologic, and genetic characteristics of congenital chest wall deformities.

          Judith Allen, William A. Ahrens, Nury Steuerwald … (2008)
          There is a large and diverse group of congenital abnormalities of the thorax that manifest as deformities and/or defects of the anterior chest wall and, depending on the severity and concomitant anomalies, may have cardiopulmonary implications. Pectus excavatum, the most common anterior chest deformity, is characterized by sternal depression with corresponding leftward displacement and rotation of the heart. Pectus carinatum, the second most common, exhibits a variety of chest wall protrusions and very diverse clinical manifestations. The cause of these conditions is thought to be abnormal elongation of the costal cartilages. Collagen, as a major structural component of rib cartilage, is implicated by genetic and histologic analysis. Poland syndrome is a unique unilateral chest/hand deficiency that may include rib defects, pectoral muscle deficit, and syndactyly. Cleft sternum is a rare congenital defect resulting from nonfusion of the sternal halves, which leaves the heart unprotected and requires early surgical intervention.
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            Current management of pectus excavatum: a review and update of therapy and treatment recommendations.

            Dawn E. Jaroszewski, David Notrica, Lisa McMahon … (2015)
            Pectus excavatum (PE) is a posterior depression of the sternum and adjacent costal cartilages and is frequently seen by primary care providers. PE accounts for >90% of congenital chest wall deformities. Patients with PE are often dismissed by physicians as having an inconsequential problem; however, it can be more than a cosmetic deformity. Severe cases can cause cardiopulmonary impairment and physiologic limitations. Evidence continues to present that these physiologic impairments may worsen as the patient ages. Data reports improved cardiopulmonary function after repair and marked improvement in psychosocial function. More recent consensus by both the pediatric and thoracic surgical communities validates surgical repair of the significant PE and contradicts arguments that repair is primarily cosmetic. We performed a review of the current literature and treatment recommendations for patients with PE deformities.
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              Demonstrating relief of cardiac compression with the Nuss minimally invasive repair for pectus excavatum.

              C D Coln, J Carrasco, Eric Coln (2006)
              Anatomic and physiological parameters have not been routinely used in the preoperative and postoperative evaluation of pectus excavatum. Most symptomatic patients have had significant subjective improvement after pectus correction. This study is based upon the use of noninvasive upright echocardiography/electrocardiogram (echo/EKG) with exercise to both identify and provide evidence of correction of cardiac abnormalities resulting from pectus excavatum. One hundred twenty-three patients, 99 males and 24 females, ages 5 to 18 years (average, 13 years) underwent Nuss pectus repair. A retrospective review of their medical records was performed. Symptoms related to exertion were present in 106 (86%). The mean Haller chest wall index (CWI) was 4.3 (2.4-10.85). Preoperative echo/EKG with exercise revealed cardiac compression in 117 (95%). A mitral valve abnormality was present in 54 (44%). Six children had no chamber compression but mitral valve prolapse was present in 2 and significant arrhythmias in 4. All patients were asymptomatic after surgery. Postoperative echo/EKG with exercise was performed in 107 (87%). The postoperative echo/EKG was normal in 100 (93% of those studied). Mild persistent mitral valve prolapse existed in 7. There were no postoperative arrhythmias. Twelve (9.8%) patients with low CWI (<3.25) were relieved of chamber compression and had no postoperative arrhythmia. Patent ductus was discovered in 2 patients on their postoperative echos. One closed spontaneously. A child with Marfan syndrome required interventional occlusion. Noninvasive echo/EKG with exercise is beneficial in the evaluation of patients with pectus excavatum and provides objective evidence of improvement postoperatively. It is especially valuable as a physiological indicator of cardiac abnormality in patients with a CWI below 3.25. Patients with mitral valve prolapse need long-term follow-up.
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                Author and article information

                Journal
                Journal ID (nlm-ta): J Cardiovasc Ultrasound
                Journal ID (publisher-id): JCU
                Title: Journal of Cardiovascular Ultrasound
                Publisher: Korean Society of Echocardiography
                ISSN (Print): 1975-4612
                ISSN (Electronic): 2005-9655
                Publication date (Print): December 2011
                Publication date (Electronic): 27 December 2011
                Volume: 19
                Issue: 4
                Pages: 192-195
                Affiliations
                [1 ]Department of Surgery, Division Cardiothoracic Surgery, Mayo Clinic Arizona, Phoenix, AZ, USA.
                [2 ]Department of Medicine, Division of Cardiovascular Diseases, Mayo Clinic Arizona, Phoenix, AZ, USA.
                Author notes
                Address for Correspondence: Dawn E. Jaroszewski, Cardiothoracic Surgery, Mayo Clinic Arizona, 5777 East Mayo Boulevard, Phoenix, AZ 85255, USA. Tel: +1-480-342-2270, Fax: +1-480-342-2269, Jaroszewski.dawn@ 123456mayo.edu
                Article
                DOI: 10.4250/jcu.2011.19.4.192
                PMC ID: 3259543
                PubMed ID: 22259662
                SO-VID: a3b1b24f-42b4-4cc7-bc94-34c3e054c9d5
                Copyright © Copyright © 2011 Korean Society of Echocardiography
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 28 April 2011
                Date revision received : 19 August 2011
                Date accepted : 30 November 2011
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: pectus excavatum,right ventricular compression,echocardiography right heart,chest pain,chest wall deformity
                Data availability:
                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: pectus excavatum, right ventricular compression, echocardiography right heart, chest pain, chest wall deformity

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