The Complete Reference for Scimitar Syndrome

Anatomy, Epidemiology, Diagnosis and Treatment

Vladimiro Vida

• Publisher: Academic Press
• Year: 2017
• Topic: Medicine
• Print ISBN:
  9780128104064
• Subtopic: Cardiology
• eBook ISBN:
  9780128104071

The Complete Reference for Scimitar Syndrome: Anatomy, Epidemiology, Diagnosis and Treatment gives the complete picture of this rare syndrome that is usually treated with tactics such as imaging. The book goes beyond imaging, providing a pathological and clinical description of this rare syndrome. All aspects beginning with development, clinical presentation, current surgical options (including recent techniques) and indications to treat patients based on current results coming from multicenter study experiences are included. This book is a must-have reference for all cardiologists, cardiac surgeons, thoracic surgeons, pathologists, physicians, residents and students involved in Scimitar Syndrome cases. - Includes diagnostic features and surgical techniques based on the international scimitar registry - Gives a complete picture of the various aspects of this rare congenital heart malformation that focuses on recent therapeutic results - Provides insights into current medical and surgical outcomes

Part A

Scimitar Syndrome

Outline

Chapter 1. Introduction and Definition

Chapter 2. Historical Notes

Chapter 3. Embryology

Chapter 4. Anatomy and Histology

Chapter 5. Epidemiology and Physiopathology of Scimitar Syndrome

Chapter 6. Diagnosis and Imaging

Chapter 7. Natural History and Medical Treatment

Chapter 8. Cardiac Angiography and Interventional Therapy

Chapter 9. Surgical Treatment and Outcomes

Chapter 1

Introduction and Definition

Vladimiro L. Vida University of Padua, Padua, Italy

Abstract

Scimitar syndrome (SS) is a rare congenital heart defect (CHD) characterized by anomalous venous drainage of part or all of the right lung to the inferior vena cava, variable right lung hypoplasia, and variable systemic blood supply to part of the right lung.

Keywords

Congenital heart disease; Pulmonary hypoplasia; Scimitar syndrome

1.1. Introduction

In 2006–07, I spent a period of time at the Children’s Hospital, Harvard Medical School, Boston, as “senior fellow” and “chief resident” in cardiac surgery. At that time, Dr. PJ Del Nido, chief of the pediatric cardiac surgery unit called me to his office because he needed some language translation with an Italian lady, Rita Martini, who came into touch with him because of her rare congenital heart disease, the scimitar syndrome (SS).

After that, Rita told me that she was confused because in Italy she was not able to find someone who could help her in the management and treatment of her heart malformation; furthermore, she was not able to find a consistent scientific document treating the various aspects of her rare cardiac malformation. For this reason, she decided to get in touch with the chief of one of the leading pediatric and congenital heart centers in North America and she ended up coming over to Boston for her treatment. In those days I had the privilege to assist Dr. Del Nido during the operation and to take part in Rita’s postoperative care till discharge.

Rita is an active and brilliant lady, and after coming back from the United States she decided to found a nonprofit association in Italy called “Amici della Scimitarra, ONLUS” (translated: friends of scimitar association) to create a network among patients with this rare disease and their families in order to share information, experiences, and knowledge.

Once I finished my training period in the United States, I decided to get more information on SS and I embarked on a couple of multiinstitutional studies, at a national and international basis, to evaluate surgical results and follow-up outcomes of these patients, and eventually in a multicenter study evaluating the clinical outcome of patients with SS who didn’t undergo surgical correction of their anomalous pulmonary venous connection. Eventually I decided to coordinate a group of international experts about the anatomy, embryology, diagnosis, and treatment of this rare syndrome, putting together their knowledge to build up a consistent document on this topic, treating also other rare pulmonary vascular malformations such as pulmonary sequestration, horseshoe lung, anomalous arterious supply to the normal lung, and the persistent primitive hepatic venous plexus.

1.2. Definition

A scimitar is a curved oriental sword with an edge on the convex side (from French “cimaterre,” from Italian “scimitarra,” from Spanish “cimitarra,” and from Persian “simsir”) [1] (Fig. 1.1).

SS is a rare association of congenital cardiopulmonary anomalies consisting of a partial or total anomalous pulmonary venous connection of the right lung to the inferior vena cava, right lung hypoplasia and a systemic arterial supply of variable hemodynamic importance to the right lung [2–5].

Figure 1.1 The curved oriental sword with an edge on the convex side, named scimitar.

The origin of the name “scimitar” comes from the curvilinear shadow along the posterior-anterior chest X-ray, representing the anomalous pulmonary venous connection of the hypoplastic right lung [6,7] (Fig. 1.2).

Figure 1.2 Plain chest X-ray showing the curvilinear shadow along the posterior-anterior on the right hemithorax, representing the anomalous pulmonary venous connection of the hypoplastic right lung.

In fact, the intrathoracic course of the right anomalous pulmonary venous connection, descending toward the inferior vena cava appears as a radio-opaque waning moon gradually widening from the pulmonary hilum toward the cardiophrenic angle.

This syndrome is also known as Halasz syndrome, mirror-image lung syndrome, hypogenetic lung syndrome, epi-bronchial right pulmonary artery syndrome, vena cava bronchovascular syndrome, or congenital pulmonary veno-lobar syndrome [3,8,9].

References

[1] Morris W, ed. American Heritage Dictionary of the English Language. Boston: Houghton Mifflin Co; 1979:1163.

[2] Dupuis C, Charaf L.A, et al. Infantile form of the scimitar syndrome with pulmonary hypertension. Am J Cardiol. 1993;71:1326–1330.

[3] Dupuis C, Charaf L.A, Brevie re G.M, et al. The adult form of scimitar. Am J Cardiol. 1992;70:502–507.

[4] Vida V.L, Padalino M.A, Boccuzzo G, et al. Scimitar syndrome: A European Congenital Heart Surgeons Association (ECHSA) multicentric study. Circulation. 2010;122:1159–1166.

[5] Herlong J.R, Jaggers J.J, Ungerleider R.M. Congenital heart surgery nomenclature and database project: pulmonary venous anomalies. Ann Thorac Surg. 2000;69:56–69.

[6] Dotter C.T, Hardisty N.M, Steinberg I. Anomalous right pulmonary vein entering the inferior vena cava: two cases diagnosed during life by angiocardiography and cardiac catheterization. Am J Med Sci. 1949;218:31–36.

[7] Roehm Jr. J.O, Jue K.L, Amplatz K. Radiographic features of the scimitar syndrome. Radiology. 1966;86(5):856–859.

[8] Aggarwal S, Joshi A, Bolger-Theut S, et al. Meandering inferior vena cava in a child with pseudo-scimitar syndrome. Ann Thorac Surg. 2011;92(4):1528.

[9] Halasz N.A, Halloran K.H, Liebow A.A. Bronchial and arterial anomalies with drainage of the right lung into the inferior vena cava. Circulation. 1956;14:826–846.

Chapter 2

Historical Notes

Gaetano Thiene University of Padua, Padua, Italy

Abstract

From the first observation in 1836 to nowadays, the scimitar syndrome has been described by many eminent authors throughout different countries. Its name was eventually christened in the 1960 and has remained unchanged to underline its specific conformation among pulmonary vascular abnormalities.

Keywords

History; Scimitar syndrome

The first anatomical observation of this peculiar anomalous venous connection was described by George Cooper [1] in the United Kingdom and by Raoul Chassinat in France [2], both in 1836.

George Cooper was an English pathologist who reported the autopsy findings of an “imperfect development of the right lung with malposition of the heart’’ in a 10-month-old girl with right pulmonar...

Table of contents

• Cover image
• Title page
• Table of Contents
• Copyright
• List of Contributors
• Foreword
• Part A. Scimitar Syndrome
• Part B. Pulmonary Vascular Anomalies
• Index