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Case Study
An interesting case of straight back syndrome and review of the literature
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314539335 aan.sagepub.com
Pavan Soleti1, Bivin Wilson2, Arani Rajabathar Vijayakumar1, Paulraj Ignatius Sudhakar Chakravarthi1 and Chilakala Gopinath Reddy1
Abstract Straight back syndrome is characterized by loss of the normal upper thoracic kyphosis, leading to a reduced anteroposterior diameter and squashing of the heart. Most patients are asymptomatic; if symptomatic, chest pain and palpitations are most common. On examination, the abnormal clinical findings simulate organic heart disease that needs to be ruled out by echocardiography and cardiac catheterization. A lateral chest radiograph is diagnostic. This condition is commonly associated with mitral valve prolapse and bicuspid aortic valve. We describe an interesting case of straight back syndrome with all the classic and rarely reported clinical findings.
Keywords Heart murmurs, Mitral valve prolapse, Straight back, Thoracic vertebrae
Introduction Straight back syndrome (SBS), also known as flat-chest syndrome, is characterized by loss of the normal upper thoracic kyphosis, leading to a reduced anteroposterior diameter and squashing of the heart. It was first reported by Rawlings in 1960.1 It has been suggested that the deformity occurs in intrauterine life during the eighth week, before ossification of the bodies of the vertebrae occur. SBS has an autosomal dominant inheritance with antigenic determinants located on chromosome 6.2 Patients are usually referred to a cardiologist due to a murmur detected during routine cardiac examination. SBS is commonly associated with mitral valve prolapse (MVP) and bicuspid aortic valve.2,3 Here we present an interesting case of SBS with all the classic as well as the rarely reported findings.
Case report A 24-year-old slim-built male software engineer presented to us for a pre-employment assessment. He had a history of dyspnea on exertion (New York Heart Association class II). Cardiovascular examination revealed visible pulsations over the left 3rd
intercostal space with a normal apical impulse and a systolic thrill in the pulmonary area. On auscultation, the second heart sound revealed a loud pulmonary component with a wide and fixed split, and there was a grade V ejection systolic murmur in the pulmonary area. A clinical diagnosis of atrial septal defect with associated pulmonary stenosis was considered. An electrocardiogram revealed left-axis deviation and incomplete right bundle branch block (Figure 1). A chest radiograph in frontal view was normal. Transthoracic and transesophageal echocardiography was normal except for MVP of the anterior leaflet with no mitral regurgitation. At this time, we suspected a diagnosis of SBS, thus a lateral chest radiograph was requested. It revealed straightening of the upper thoracic spine with loss of normal kyphosis, and the measurements satisfied 1 Department of General Medicine, G Kuppuswamy Naidu Memorial Hospital, Coimbatore, India 2 Department of Cardiology, G Kuppuswamy Naidu Memorial Hospital, Coimbatore, India
Corresponding author: Bivin Wilson, G Kuppuswamy Naidu Memorial Hospital, PN Palayam Coimbatore 641037, India. Email: [email protected]
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[1–3] [PREPRINTER stage]
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Asian Cardiovascular & Thoracic Annals 0(0)
Figure 1. Twelve-lead electrocardiogram showing RSR’ pattern in V1 and left anterior hemiblock.
the diagnostic criteria proposed by Davies and colleagues2 (Figure 2) and DeLeon and colleagues4 Figure 3). Thus we confirmed the diagnosis of SBS, reassured the patient, and advised medical follow-up.
Discussion SBS is a benign skeletal deformity of the thorax, which may masquerade as heart disease. Patients are usually asymptomatic, and if symptomatic, chest pain and palpitations are most commonly reported.2 In 1956, DeLeon and colleagues4 first proposed a diagnostic criterion: a ratio of the anteroposterior diameter measured at T8 level to the transverse diameter of the chest
(AAN)
[1–3] [PREPRINTER stage]
Case Study
An interesting case of straight back syndrome and review of the literature
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314539335 aan.sagepub.com
Pavan Soleti1, Bivin Wilson2, Arani Rajabathar Vijayakumar1, Paulraj Ignatius Sudhakar Chakravarthi1 and Chilakala Gopinath Reddy1
Abstract Straight back syndrome is characterized by loss of the normal upper thoracic kyphosis, leading to a reduced anteroposterior diameter and squashing of the heart. Most patients are asymptomatic; if symptomatic, chest pain and palpitations are most common. On examination, the abnormal clinical findings simulate organic heart disease that needs to be ruled out by echocardiography and cardiac catheterization. A lateral chest radiograph is diagnostic. This condition is commonly associated with mitral valve prolapse and bicuspid aortic valve. We describe an interesting case of straight back syndrome with all the classic and rarely reported clinical findings.
Keywords Heart murmurs, Mitral valve prolapse, Straight back, Thoracic vertebrae
Introduction Straight back syndrome (SBS), also known as flat-chest syndrome, is characterized by loss of the normal upper thoracic kyphosis, leading to a reduced anteroposterior diameter and squashing of the heart. It was first reported by Rawlings in 1960.1 It has been suggested that the deformity occurs in intrauterine life during the eighth week, before ossification of the bodies of the vertebrae occur. SBS has an autosomal dominant inheritance with antigenic determinants located on chromosome 6.2 Patients are usually referred to a cardiologist due to a murmur detected during routine cardiac examination. SBS is commonly associated with mitral valve prolapse (MVP) and bicuspid aortic valve.2,3 Here we present an interesting case of SBS with all the classic as well as the rarely reported findings.
Case report A 24-year-old slim-built male software engineer presented to us for a pre-employment assessment. He had a history of dyspnea on exertion (New York Heart Association class II). Cardiovascular examination revealed visible pulsations over the left 3rd
intercostal space with a normal apical impulse and a systolic thrill in the pulmonary area. On auscultation, the second heart sound revealed a loud pulmonary component with a wide and fixed split, and there was a grade V ejection systolic murmur in the pulmonary area. A clinical diagnosis of atrial septal defect with associated pulmonary stenosis was considered. An electrocardiogram revealed left-axis deviation and incomplete right bundle branch block (Figure 1). A chest radiograph in frontal view was normal. Transthoracic and transesophageal echocardiography was normal except for MVP of the anterior leaflet with no mitral regurgitation. At this time, we suspected a diagnosis of SBS, thus a lateral chest radiograph was requested. It revealed straightening of the upper thoracic spine with loss of normal kyphosis, and the measurements satisfied 1 Department of General Medicine, G Kuppuswamy Naidu Memorial Hospital, Coimbatore, India 2 Department of Cardiology, G Kuppuswamy Naidu Memorial Hospital, Coimbatore, India
Corresponding author: Bivin Wilson, G Kuppuswamy Naidu Memorial Hospital, PN Palayam Coimbatore 641037, India. Email: [email protected]
Downloaded from aan.sagepub.com at UQ Library on June 22, 2015
XML Template (2014) [2.6.2014–7:09pm] //blrnas3/cenpro/ApplicationFiles/Journals/SAGE/3B2/AANJ/Vol00000/140126/APPFile/SG-AANJ140126.3d
(AAN)
[1–3] [PREPRINTER stage]
2
Asian Cardiovascular & Thoracic Annals 0(0)
Figure 1. Twelve-lead electrocardiogram showing RSR’ pattern in V1 and left anterior hemiblock.
the diagnostic criteria proposed by Davies and colleagues2 (Figure 2) and DeLeon and colleagues4 Figure 3). Thus we confirmed the diagnosis of SBS, reassured the patient, and advised medical follow-up.
Discussion SBS is a benign skeletal deformity of the thorax, which may masquerade as heart disease. Patients are usually asymptomatic, and if symptomatic, chest pain and palpitations are most commonly reported.2 In 1956, DeLeon and colleagues4 first proposed a diagnostic criterion: a ratio of the anteroposterior diameter measured at T8 level to the transverse diameter of the chest
