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Prenatal Imaging and Therapy of Congenital Heart Disease

Introduction Congenital abnormalities of the heart are the most common birth defects, occurring in approximately 8 of every 1,000 newborns. Approximately 25% of those in whom an abnormality is identified have critical congenital heart disease (CHD) with risk for significant perinatal compromise or death. Despite advances in imaging technology and technique, much CHD remains undetected until after birth. Fortunately, routine prenatal cardiac screening has evolved from…

Surgical Considerations for Congenital Heart Disease

Congenital cardiac defects may be categorized in a variety of ways. One approach is to separate them based on the presence or absence of cyanosis at the time of presentation. Cyanotic lesions are either associated with shunting of deoxygenated blood into the systemic arterial circulation or with severely reduced pulmonary blood flow. These lesions include transposition of the great arteries, tetralogy of Fallot (TOF), truncus arteriosus,…

Pediatric Cardiac Catheterization and Electrophysiology

Pediatric Cardiac Catheterization Introduction Pediatric cardiovascular catheterization laboratories today involve a variety of complex diagnostic and interventional procedures for congenital abnormalities using multiple imaging tools to improve procedural outcome and patient safety. Preprocedure imaging to map the complex anatomy is more routinely performed, including modalities such as computed tomography (CT), cardiac magnetic resonance imaging (cardiac MRI), and three-dimensional (3D) model creation to plan the interventional procedure.…

Magnetic Resonance Imaging for Congenital Heart Disease

Acknowledgment: The editors and the publisher would like to thank Drs. Sadaf T. Bhutta and S. Bruce Greenberg for contributing a chapter on this topic to the prior edition of this work. It has served as the foundation for the current chapter. Cardiac magnetic resonance imaging (MRI) has become integral in the evaluation, treatment planning, and posttreatment surveillance of congenital and acquired heart disease. Although echocardiography…

Pediatric Cardiothoracic Computed Tomographic Angiography

Remarkable advances have occurred in noninvasive imaging evaluation of pediatric cardiothoracic vascular disorders. One such technologic advancement is multidetector array computed tomography angiography (CTA). CTA has become a primary imaging consideration for structural cardiovascular evaluation beginning as early as the newborn period. Attention to technique is fundamental for pediatric CTA. Without optimal or at least sufficient technical performance, diagnostic capabilities may be limited. This technical aspect…

Chest Radiography in Pediatric Cardiovascular Disease

The role of chest radiography in the diagnosis and evaluation of congenital cardiovascular disease continues to evolve. At one time a major tool in the assessment of heart disease, radiography now occupies an ancillary role, with echocardiography serving as the major primary investigation after physical examination, especially in the neonatal period. However, the chest radiograph still may provide the first indication of unsuspected cardiovascular disease, and…

Cardiovascular Anatomy and Segmental Approach to Imaging of Congenital Heart Disease

Because of the large variety of human hearts in nature, a standardized approach and nomenclature is needed to understand and describe cardiac anatomy and physiology in the setting of congenital heart disease. The most widely used approach is called the segmental approach to heart disease, first proposed by Richard Van Praagh in 1972 and later modified by others. It is strongly rooted in embryologic principles, and…

Introductory Embryology

Cardiac Development The major task in cardiac development is to form a four-chambered heart that functions in a coordinated fashion from a straight tube that functions merely by peristalsis. Cardiac development can be thought of as proceeding along various phases: fusion of myocardium and endocardium in the ventral midline to form a simple tube, onset of function, looping to the right side, specification and formation of…

The Diaphragm

Overview The diaphragm is a dome-shaped musculofibrous membrane that separates the thoracic and abdominal cavities and performs an important respiratory function. The diaphragm has a fibrous portion centrally (i.e., a central tendon) surrounded by a peripheral muscular portion. Major structures pass through three openings: caval (inferior vena cava and some right phrenic nerve branches), esophageal (esophagus, vagal nerves, and small esophageal arteries), and aortic (aorta, azygous…

The Pleura

The pleura is a serous membrane consisting of two layers, the visceral and the parietal pleura. The parietal pleura covers the inner aspect of the chest wall and the diaphragm. The visceral pleura is strongly adherent to the surface of the lungs and interlobar fissures. In normal conditions, no imaging study can visualize the pleural space because pleural membranes are only 0.2 to 0.4 mm thick, and…

The Chest Wall

The chest wall provides support and protection to the various thoracic vascular and nonvascular structures. In addition, it allows the important physiologic motion of the lungs and airways. The chest wall is less rigid and more cartilaginous in children than in adults and consists of several fundamental structural components, including bones (ribs, sternum, and vertebrae), nerves, muscles, vessels, and subcutaneous soft tissues. Within these structural components,…

Pulmonary Manifestations of Systemic Disease

Many systemic diseases of childhood involve the pulmonary parenchyma. The pulmonary manifestations may in fact prompt the patient or parents to seek medical attention and frequently a chest radiograph is obtained early in the illness. Therefore the radiologist can play an important role in directing the diagnostic workup by recognizing that the pulmonary findings reflect an underlying condition. In some patients, such as those with pulmonary…

Diffuse Lung Disease

The pediatric diffuse lung disorders, also known as childhood interstitial lung diseases (chILDs), comprise a rare and heterogeneous spectrum of progressive and often lethal pulmonary pathology. They are characterized by otherwise unexplained respiratory signs and symptoms, hypoxemia, and widespread pulmonary opacities on imaging. The diseases are not limited to the interstitium and may affect the alveoli, airways, blood vessels, lymphatic channels, and pleural spaces. Although much…

Neoplasia

Pulmonary neoplasm is much less common in children than in adults. Affected children may present clinically with respiratory symptoms, or a pulmonary neoplasm may be detected incidentally on a chest radiograph in an asymptomatic child. In a recent series of 204 pediatric lung tumors, the ratio of primary benign to primary malignant to secondary malignant pulmonary neoplasms (i.e., metastases) is 1.4 : 1:11.6. Primary lung tumors represent only…

Pulmonary Infection

Pneumonia and other pulmonary infections involving the lower respiratory tract continue to be the most common cause of illness in children, affecting over 150 million children under 5 years of age each year worldwide, with 20 million related US hospitalizations annually. Despite the decrease in hospitalizations for pediatric pneumonia since the introduction of the pneumococcal conjugate vaccine in 2000, the rates of pneumonia presenting in the…

Congenital Lung Anomalies

Congenital lung anomalies represent a heterogeneous group of pulmonary developmental disorders that affect the lung parenchyma, its arterial supply, venous drainage, or a combination thereof. The reported incidence of congenital lung anomalies ranges from 30 to 42 cases per 100,000 population. Although prenatal sonography, fetal magnetic resonance imaging (MRI) and advances in postnatal imaging have enhanced our understanding of congenital lung anomalies, substantial controversy continues regarding…

Lower Large Airway Disease

Overview Disorders of the lower large airways occur frequently in the pediatric population and have the potential to be life threatening. Because these disorders are often associated with nonspecific clinical symptoms, the diagnosis frequently is missed or delayed, particularly in infants and young children. After careful investigation of the clinical history and physical examination, imaging evaluation is the next management step. Imaging plays an important role…

Upper Airway Disease

The upper airway, by definition, lies above the thoracic inlet. The anomalies and abnormalities involving the upper airway in children are common and varied. They include a combination of congenital and acquired morphologic, neuromuscular, bony, and cartilaginous abnormalities. The anatomic considerations that predispose children to acute respiratory distress, even with only partial obstruction, are as follows: (1) The nasopharynx is narrower than in adults, (2) the…

Prenatal Imaging and Intervention

With the advances in ultrasound (US) and magnetic resonance imaging (MRI), thoracic lesions are now commonly detected before birth. The most common congenital thoracic lesions include congenital diaphragmatic hernia (CDH); congenital bronchopulmonary malformations, which represent a group of lung anomalies, including congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration (BPS), and congenital lobar overinflation (CLO); and congenital hydrothorax. Additionally, congenital high airway obstruction (CHAOS), which is caused…