Congenital Heart Disease

Transcription

1 Congenital Heart Disease Morphological and Functional Assessment Hideaki Senzaki Satoshi Yasukochi Editors 123

2 Congenital Heart Disease

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4 Hideaki Senzaki Satoshi Yasukochi Editors Congenital Heart Disease Morphological and Functional Assessment

5 Editors Hideaki Senzaki Department of Pediatric Cardiology Saitama Medical Center Saitama Medical University Kawagoe, Japan Satoshi Yasukochi Heart Center/Pediatric Cardiology Nagano Children s Hospital Azumino, Japan ISBN ISBN (ebook) DOI / Springer Tokyo Heidelberg New York Dordrecht London Library of Congress Control Number: Springer Japan 2015 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. Exempted from this legal reservation are brief excerpts in connection with reviews or scholarly analysis or material supplied specifically for the purpose of being entered and executed on a computer system, for exclusive use by the purchaser of the work. Duplication of this publication or parts thereof is permitted only under the provisions of the Copyright Law of the Publisher s location, in its current version, and permission for use must always be obtained from Springer. Permissions for use may be obtained through RightsLink at the Copyright Clearance Center. Violations are liable to prosecution under the respective Copyright Law. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. While the advice and information in this book are believed to be true and accurate at the date of publication, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. Printed on acid-free paper Springer is part of Springer Science+Business Media (

6 Preface Anatomical or morphological abnormalities in congenital heart disease (CHD) are generally accompanied by abnormal loading conditions, which in turn cause functional ventricular and vascular impairments that may also exist independently of anatomical abnormalities, however. Interactions between morphological and functional abnormalities are important determinants of underlying pathophysiology and cause clinical symptoms in CHD. Therefore, detailed and precise assessment of morphology and function is essential to better understand and treat this disease. Recent advances in technology have provided useful tools for this purpose, and novel findings are accumulating. This book, entitled Congenital Heart Disease: Morphological and Functional Assessment, comprehensively covers the latest information about ventricular vascular morphology and function in CHD, as evaluated by numerous innovative methodologies, including echocardiography, magnetic resonance imaging (MRI), computed tomography (CT), and cardiac catheterization. In Chaps. 1 4 (Part I), the concept of morphological assessment by threedimensional echocardiography, MRI, and high-resolution multi-slice CT scanning is introduced by world-leading Japanese experts. Chapters 1 and 2 describe echocardiographic assessment, using various different approaches, of intra-cardiac anatomy, with particular focus on valvular anatomy, in detail. Three-dimensional CT and MRI are highly useful tools for assessing extra- and intra-cardiac structures and their anatomical relationships, as outlined in Chaps. 3 and 4. In Chaps. 5 8 (Part II), new frontiers in the assessment of cardiovascular function in CHD are presented by world-renowned experts in the field. While cardiac catheterization provides detailed information about cardiovascular function based on pressure measurements, as discussed in Chaps. 5 and 6, echocardiography and MRI provide detailed flowbased as well as myocardial motion-based information on cardiovascular function, as described in Chaps. 7 and 8. The information contained in each chapter will provide researchers and clinicians with invaluable knowledge of this field, and should help to deepen their understanding of CHD. It is my great honor to edit this book with my respected v

7 vi Preface friend, Dr. Satoshi Yasukochi, and to invite world-leading Japanese pediatric cardiologists as authors for each chapter. Finally, as a token of affection and gratitude, I dedicate this book to my father, Dr. Mamoru Senzaki, who died peacefully on April 6th, 2012, surrounded by his family. Arigatou, Otousan. Kawagoe, Japan May 20, 2014 Hideaki Senzaki

8 Contents Part I Morphological Assessment of Congenital Heart Disease 1 Real-Time Three-Dimensional (3D) Echocardiography for Diagnosis and Treatment of Congenital Heart Diseases in Practical Medicine: Transepicardial and Transesophageal Approach... 3 Kiyohiro Takigiku 2 Assessment of Atrioventricular Valve Anatomy and Function in Congenital Heart Diseases Using Three-Dimensional Echocardiography Masaki Nii 3 Assessment of Intracardiac Anatomy by Magnetic Resonance Imaging Satoshi Yasukochi 4 Assessment of Extracardiac and Intracardiac Anatomy by MD-CT Kenji Waki Part II Functional Assessment of Congenital Heart Disease 5 Assessment of Ventricular Function Using the Pressure-Volume Relationship Satoshi Masutani and Hideaki Senzaki 6 Assessment of Vascular Function by Using Cardiac Catheterization Hirofumi Saiki and Hideaki Senzaki vii

9 viii Contents 7 Assessment of Ventricular-Vascular Function by Echocardiography Manatomo Toyono 8 Assessment of Hemodynamics by Magnetic Resonance Imaging Masaya Sugimoto Index

10 Part I Morphological Assessment of Congenital Heart Disease

11 Chapter 1 Real-Time Three-Dimensional (3D) Echocardiography for Diagnosis and Treatment of Congenital Heart Diseases in Practical Medicine: Transepicardial and Transesophageal Approach Kiyohiro Takigiku Abstract How to use real-time three-dimensional (3D) echocardiography for diagnosis and treatment of congenital heart diseases? To obtain morphological details of complicated intracardiac structure is one of the most effective usages, when planning for cardiac surgery, such as an intracardiac rerouting through ventricular septal defect in patients with double-outlet right ventricle, valvuloplasty for complicated atrioventricular valve regurgitation, or release for intracardiac stenotic lesions. Especially, intraoperative transpericardial 3D echocardiography performed by putting the 3D probe on the pericardium directly enables us to get the good quality images with a high S/N ratio in pediatric patients less than 20 kg of body weight, for whom transesophageal 3D echocardiography cannot be applied. This approach also enables both cardiovascular surgeons and cardiologists to share the surgeon s view in the operating room quickly. Moreover, transesophageal 3D echocardiography is also useful for the decision for the indication of catheter intervention and monitoring the procedure of the percutaneous closure of atrial septal defect, as well as to diagnose the abnormal morphology and guide the intracardiac surgery in children more than 20 kg of body weight with congenital heart disease. Keywords Congenital heart disease Transesophageal 3D echocardiography Transpericardial 3D echocardiography K. Takigiku, MD, PhD (*) Department of Pediatric Cardiology, Nagano Children s Hospital, 3100 Toyoshina, Azumino, Nagano , Japan tackymr2@me.com Springer Japan 2015 H. Senzaki, S. Yasukochi (eds.), Congenital Heart Disease, DOI / _1 3

12 4 K. Takigiku 1.1 Introduction The ultrasonic diagnostic device, three-dimensional (3D) probe, and analysis workstation have developed their function, and then revolutionary advancement has been achieved. The diagnostic device has a sophisticated image processing capability and the probe is equipped with functions such as the matrix array, multi-wave transmission and reception, and multi-focusing, which enable a simple and sensitive 3D reconstruction. These are the major factors that helped the real-time 3D echocardiography increase the use opportunities in clinical practice. The 3D workstation echo scan in the 1990s used the following methods: the ECG-gated multi-cross-sectional images were obtained first, which were then consolidated to reconstruct the volume data and divided into an optional cut plain to visualize the intracardiac structure that was necessary for diagnosis [1]. Thus, it took several tens of seconds to obtain the multi-cross-sectional images (Fig. 1.1). Regarding children, moreover, it was problematic in terms of quality of images even after collecting volume data over time and reconstructing based on them because of their fast heat rate and difficulty of breath-holding compared with adults. In other words, the stitches caused by heartbeat synchronization and the gaps caused by respiratory fluctuation can affect the quality of the images significantly. In addition, enormous amount of time was necessary for off-line image analysis (the right lower panel in Fig. 1.1). Therefore, 3D echocardiography was rarely used for the diagnosis of a complicated form of congenital heart disease in the actual clinical practice. However, recently, high-resolution volume datasets have to be collected in any direction from a single to a few heartbeat datasets, when using the real-time 3D echocardiography. The development of the high-frequency 3D probe for children and the improvements of image quality, even if, by using the low-frequency probe have contributed to the issue. In addition, since the performance improvement of the analysis workstation has enabled the volume data analysis in the extremely short period of time, it has become possible to visualize the optimum cross section as well. The following are the good examples of clinical applications of the real-time 3DE for congenital heart diseases. 1. As the guide for surgical repair: Intracardiac route creation via ventricular septal defect (VSD) with double-outlet right ventricle and valvuloplasty for the complicated atrioventricular valve insufficiency 2. As the guide and monitoring for the percutaneous catheter closure of atrial septal defect or ventricular septal defect 3. As the 3D functional analysis of volume and wall motion both in the left and right ventricle and quantitative evaluation of the dynamic morphological of the atrioventricular valve leaflets in congenital heart disease Once the methodology is established, when it comes to children whose echo windows are easy to obtain and have relatively clear images, it is evident that the

13 1 Real-Time Three-Dimensional (3D) Echocardiography for Diagnosis... 5 Fig D echocardiography by ECG-gated rotational device. The upper figures show how to obtain the actual image by using ECG-gated rotational device. The probe is placed on the patient from subxiphoid window. The lower right figure shows the 3D echocardiography in a case with complete atrioventricular septal defect by the system that rotates by 2 s and collects the images of every heartbeat to reconstruct. RA right atrium, RV right ventricle, ASD(I) primum atrial septal defect, CAVV common atrioventricular valve real-time 3D echocardiography would become even more useful for the understanding of the complicated anatomical structure than the adult s cardiovascular diseases. In this chapter, I would like to discuss mainly how to use the transpericardial real-time 3D echocardiography as a guide for surgical repair in the actual clinical practice. In addition, I also would like to outline the usage of the transesophageal real-time 3D echocardiography as the guide and monitoring for the percutaneous catheter closure D Display of the Intracardiac Structure Roll as a tool for detailed diagnosis and a guide for surgery. Since 3D display of the intracardiac structure enables to understand the anatomically abnormal findings, it is an extremely useful method for considering the hemodynamic status and the operative procedure for intracardiac surgical repair

14 6 K. Takigiku [2]. It can be utilized for various surgeries such as closure operation for the multiple or complicated VSDs, creation of the intracardiac route via ventricular defect with double-outlet right ventricle and transposition of the great arteries, release for stenotic or obstructive lesions such as the left of right ventricular outflow tract and the pulmonary vein, and the atrioventricular valvuloplasty for congenital heart disease. One major point of the 3D display as a guide of the congenital heart disease is how to present it to surgeons. Creating the images from the surgeon s standing point, i.e., surgeon s view, would serve as the base of communication between cardiologists and cardiac surgeons and also help surgeons understand with ease Transpericardial 3D Echocardiography Although transthoracic 3D echocardiography has a certain level of diagnostic accuracy, we perform intraoperative transpericardial 3D echography with the aim to construct the good quality of images that would have more diagnostic accuracy and would be helpful for surgery [3]. In adult patients having severe mitral regurgitation, it is difficult to perform the detailed guide for mitral valvuloplasty by using transthoracic 3D echocardiography, while the transesophageal 3D echocardiography is better than that. Since the body weight of the most children with congenital heart disease, who undergo intracardiac repair that needs a cardiopulmonary bypass, is less than 15 kg, it is impossible to insert the probe of the current transesophageal 3D echocardiography. For the children for whom the transesophageal probe cannot be used, the transpericardial approach is probably the best 3D echocardiography currently because it produces best images and high sensitivity. Specifically, the images can be obtained by applying the 3D probe directly on the pericardium or heart under thoracotomy. Volume data will be obtained at the full-volume mode using 3D probe by temporarily shutting off the artificial respirator only when the breathing movement is influential. Of course, clearer images with higher resolution and better S/N ratio than transthoracic 3D echocardiography will be obtained (Fig. 1.2) Accommodation of Images Collecting high-quality volume data is the key to obtain the 3D images with higher quality. The first step is to select the appropriate probe. For the children with body weight less than 20 kg, it might be better to use a 3D probe with as high frequency as possible (more than 7 MHz). For those with body more than that, a 3D probe with 5 MHz frequency should be used. First, capture the 2D images. Then, decide where to put the center for collecting the 3D images. It is important to put the probe from the window that can best visualize the target lesion. It might be better to confirm whether the whole target sites are visualized properly by the biplane mode, the

15 1 Real-Time Three-Dimensional (3D) Echocardiography for Diagnosis... 7 Fig. 1.2 The actual procedure of 3D pericardial echocardiography volume dataset can be acquired to put the 3D probe directly on the pericardium under thoracotomy, using 3D probe by temporarily shutting off the artificial respirator only when the breathing movement is influential. Then good quality of reconstructed images with higher resolution and better S/N ratio compared to the images by transthoracic 3D echocardiography can be obtained multi-slice mode, and tilting the probe. Adjustment such as gain and dynamic range should be controlled on the 2D images. One of the keys to success is to put the probe on the pericardium tightly Volume Data Collection After adjusting the 2D images, start to work on the 3D image collection. It is important to capture the images considering what kind of 3D images you would like to compose at the end. For example, suppose that the disease is VSD. Figure 1.3 is the schema to observe the interventricular septum that was cut out from the right atrium and the right ventricular free wall at the frontal plane. Based on the anatomical relationship among the defect, the tricuspid valve, the pulmonary valve, and the aortic valve, it can be classified into perimembranous VSD, doubly committed VSD, trabecular VSD, and inflow septal VSD. If the 3D volume data is cut similarly as in this figure, it would help to determine what kind of approach would be appropriate to close the VSD, or whether it should be performed from the pulmonary artery or the tricuspid valve, or under which leaflet of tricuspid valve the

16 8 K. Takigiku Inflow septal Subpulmonary Outlet muscular Perimembranous Doubly committed Trabecular Inlet muscular Fig. 1.3 Type of the ventricular septal defects Fig. 1.4 The comparison between the transpericardial 3D image with the ventricular septal defect and the surgical finding. RA right atrium, PA pulmonary artery, RV right ventricle, VSD ventricular septal defect defect exists. It might be better to understand the anatomical knowledge and representative operative procedures regarding the VSD before data acquisition. Figure 1.4 shows the comparison between the transpericardial 3D image with the VSD and the surgical finding. Similarly as in the surgical findings, the defect is seen in perimembranous portion, nearly the upper part of the septal leaflet of the tricuspid valve. In addition, the chorda tendinea of the tricuspid valve appears to cross over the defect, which can be clearly confirmed by the 3D echocardiography. To close the defect, it used to be necessary to make a resection avoiding the chorda tendinea [3]. In the assessment of the double-outlet right ventricle and the VSD that is porous and more complicated, the positional relationship among the pulmonary valve, the aortic valve, the tricuspid valve, and the abnormal chorda tendinea in addition to the size or the number becomes even more important in order to

17 1 Real-Time Three-Dimensional (3D) Echocardiography for Diagnosis... 9 determine the method of closing the defect and forming the intracardiac route. Therefore, the volume data should be collected so that not only the VSD but also all the surrounding large vessels and atrioventricular valves would be included. The volume rate at the time of collecting should be over 40 Hz when the heart rate is around 100 bpm. Since a wide angle becomes necessary due to the necessity of including the surrounding structures, capturing in the full-volume mode integrated with ECG-synchronized multiple slices (heartbeats) could maintain the beamline density higher than capturing the single heartbeat with low volume rate Cropping The next step is to create the images viewed from the surgeon s position, so-called surgeon s view. To that end, it is necessary to understand the anatomical features of the disease and representative operative procedures and keep in mind how to proceed with the cropping to make the cut-plane. Here is an example of the actual cropping case of the double-outlet right ventricle. Figure 1.5 shows a case with VSD in double-outlet right ventricle. The 2D echography reveals that the aorta is located in the right posterior and the pulmonary artery in the left anterior while the VSD exists subpulmonary. In Fig. 1.6, the 3D image by cropping of the right ventricular free wall of the transpericardial volume data in this case is visualized. An abnormal muscle bundle that separates the large VSD into the right and left halves exists from the center of the VSD to the right ventricular free wall. It was diagnosed as the subpulmonary VSD and the subaortic VSD, so-called multiple VSDs. Figure 1.6 is the view of the VSD from the pulmonary artery side, which is the surgeon s view of the opened pulmonary artery. The VSD under the aortic valve is invisible due to the abnormal muscle bundle. The surgical findings shown in Fig. 1.7 are completely consistent with the preoperative echo findings when the pulmonary artery is opened. The VSD that is inserted with forceps was the one under the pulmonary valve, while the other one is not accessible being blocked by Fig D echocardiography in a case with double-outlet right ventricle. Ao aorta, PA pulmonary artery, LA left atrium, RA right atrium, LV left ventricle, VSD ventricular septal defect

18 10 K. Takigiku Fig D transpericardial echocardiography in a case with double-outlet right ventricle. The view of the ventricular septal defect above the pulmonary valve. Ao aorta, PA pulmonary artery, TV tricuspid valve, LV left ventricle, VSD ventricular septal defect, IVS interventricular septum Fig. 1.7 The surgical findings in a case with double-outlet right ventricle. The relation between subpulmonary VSD and muscle band is completely consistent with the preoperative echo findings in Fig. 1.6, when the pulmonary artery was open. PA pulmonary artery, VSD ventricular septal defect

19 1 Real-Time Three-Dimensional (3D) Echocardiography for Diagnosis the muscle bundle as expected. The VSD under the aortic valve is approached from the tricuspid valve side and a route is created from the left to the right ventricle. On the other hand, patch closure is performed for the VSD under the pulmonary valve from the pulmonary valve side. If the two VSDs have not been found at the preoperative diagnosis, they could not have been closed completely and the patient could not have been disconnected from the cardiopulmonary bypass. Thus, when the VSD is multiple or the shapes of the defect and the surrounded structures are complicated, anatomically detailed diagnosis by optimal cropping of the transpericardial 3D volume data would be very useful for the surgery practically Case Presentation In this chapter, I would like to discuss some actual cases for which the transpericardium 3D displays were effective Postoperative Left Ventricular Outflow Tract Stenosis This is a case in which a left ventricular outflow tract stenosis occurred after intracardiac repair for the double-outlet right ventricle (Fig. 1.8). This 3D image was the one that was cropped looking up the left ventricular outflow tract from the left ventricle side. The fibrous structure sticking out under the aortic valve from the anterior mitral leaflet side and the patch used to close the VSD (the highly bright structure extending from the interventricular septum to the aortic valve) have narrowed the subaortic site and formed a high degree of stenosis. In the surgery, Fig. 1.8 Transepicardial 3D echocardiography in a case with left ventricular outflow tract stenosis after intracardiac repair of the double-outlet right ventricle. Ao aorta, MV mitral valve, TV tricuspid valve, LV left ventricle, VSD ventricular septal defect

20 12 K. Takigiku the fibrous structure was resected from the aorta side and the patch was removed and reapplied in a boat-like shape to secure the wide outflow tract again. Thus, not necessarily only surgeon s view but also the cross section observed from the angle that is difficult to obtain from the usual 2D images can be utilized as a guide of surgical procedures Atrioventricular Valvuloplasty In children with atrioventricular valve disease, it is also very important for valvuloplasty to analyze of the several mechanism of valvular regurgitation, by using the 3D assessment/diagnosis, such as extension of the flap, tethering, contraction of the flap, extension and rupture of the chorda tendinea, and abnormal adhesion of papillary muscle. Collecting volume data enables to cut the valve and the valvular structure as one complex at an optimal cross section or continuous cross sections, and then, the abnormal structure would be grasped accurately. It also works as a guide for surgical repair to identify the abnormal area, the area of the tobe-resected valve, and the need of the artificial chorda tendinea, filling of the valve leaflet, and adaptation of artificial valve replacement surgery. Adult patients mainly undergo the detailed diagnosis using transesophageal 3D echocardiography; however, morphological evaluation of the atrioventricular valve complicated with congenital heart diseases using transpericardial 3D echography is very useful because the valvuloplasty in most patients is performed in infancy, for whom transesophageal 3D echocardiography cannot be applied, and moreover the quality of the transthoracic 3D echocardiographic image is not yet satisfactory. Figure 1.9 shows the transpericardial 2D echocardiographic image of congenital mitral regurgitation while Fig shows the transpericardial 3D echocardiography. When being observed from the left atrial side, the anterior leaflet is short and Fig. 1.9 Transpericardial 2D echocardiography in congenital mitral regurgitation. Ao aorta, LA left atrium, LV left ventricle