Ventricular Assist Device Use in Patients With Single-Ventricle Circulation

Ventricular assist devices (VADs) are being increasingly used to support patients with congenital heart disease and single-ventricle physiology. Because of their unique anatomy and physiology, special consideration must be used to provide effective mechanical circulatory support for each individual patient. This can include alternative cannulation techniques, strategies to balance cardiac output to the systemic and pulmonary circulations from a single ventricle, or the use of continuous vs pulsatile VADs for better ventricular offloading. In this article we review the etiology of single-ventricle failure, VAD options for support, cannulation strategies, post-VAD management considerations, and outcomes at each of the 3 stages of palliation.     

Cardiovascular magnetic resonance imaging for valvular heart disease

Valvular heart disease (VHD) is a clinically important diagnosis, with significant associated morbidity and mortality. Multiple imaging modalities exist to characterize valvular and associated cardiac anatomy. Cardiovascular magnetic resonance (CMR) has emerged as a comprehensive noninvasive imaging modality for VHD. With use of well-established, standardized imaging sequences, CMR can accurately and precisely diagnose valvular structural abnormalities, assess severity of regurgitant and stenotic lesions, and potentially define patient prognosis. This article reviews the clinical applications of CMR in assessment of VHD.     

Prenatal Diagnosis and Management of Single-Ventricle Heart Disease

In the current era, most single-ventricle heart disease (SVHD) is diagnosed prenatally by means of fetal echocardiography. Disparities exist, however, by socioeconomic status and remote location, which require further attention. Prenatal diagnosis affords the opportunity to counsel expectant parents regarding the life-long course of children with SVHD, including the stages of single-ventricle palliation and challenges of the Fontan circulation; to discuss pregnancy management options; and to optimise delivery planning and perinatal care. Prognosis may be refined by specific features on the fetal echocardiogram, such as ventricular morphology, total anomalous pulmonary venous return, and atrioventricular valve regurgitation. Expectant mothers should be referred for evaluation of extracardiac anomalies and/or a genetic syndrome, which also significantly affect outcome. Fetuses with SVHD should be cared for by a multidisciplinary team and ideally delivered at term at or near a cardiac surgical center. Serial echocardiograms refine the anticipated postnatal physiology to optimise transitional care, including the need for prostaglandin or urgent atrial septal intervention in fetuses with hypoplastic left heart syndrome. In selected patients, there may be a role for fetal cardiac intervention to improve mortality or achieve a biventricular circulation after birth. Together, these strategies enhance the preoperative status of the neonate. Recent advances in fetal cardiovascular magnetic resonance imaging have focused on studying the relationships between cardiovascular physiology and fetal growth and development. These novel techniques allow for the exploration of the physiologic effects of SVHD on the brain and open avenues for the investigation of neuroprotective therapies.     

The Contemporary Role of Cardiac Computed Tomography and Cardiac Magnetic Resonance Imaging in the Diagnosis and Management of Pericardial Diseases

Pericardial syndromes encompass different clinical conditions from acute pericarditis to idiopathic chronic pericardial effusion. Transthoracic echocardiography is the first and most important initial diagnostic imaging modality in most patients affected by pericardial disease. However, cardiac computed tomography (CCT) and cardiac magnetic resonance imaging (CMR) have recently gained a pivotal role in cardiology, and recent reports have supported the role of both of these advanced techniques in the evaluation and guiding therapy of pericardial disease. Most promising is the capability of CMR to identify the presence of pericardial inflammation, carrying both diagnostic and prognostic value in the setting of recurrent and chronic pericarditis. In addition, CCT permits accurate evaluation of the presence and extension of pericardial calcification, providing important information in confirming the diagnosis of constrictive pericarditis and during the preprocedural planning for patients undergoing pericardiectomy. Both CCT and CMR require specific expertise, especially for the evaluation of pericardial disease. The aim of the present review is to provide physicians an updated overview of CCT and CMR in pericardial disease, focusing on technical issues, recent research findings, and potential clinical applications.     

Considerations in Critical-Care and Anesthetic Management of Adult Patients Living With Fontan Circulation

The Fontan procedure is a staged palliation for various complex congenital cardiac lesions, including tricuspid atresia, pulmonary atresia, hypoplastic left heart syndrome, and double-inlet left ventricle, all of which involve a functional single-ventricle physiology. The complexity of the patients’ original anatomy combined with the anatomic and physiologic consequences of the Fontan circulation creates challenges. Teens and adults living with Fontan palliation will need perioperative support for noncardiac surgery, peripartum management for labour and delivery, interventions related to their structural heart disease, electrophysiology procedures, pacemakers, cardioversions, cardiac surgery, transplantation, and advanced mechanical support. This review focuses on the anesthetic and intensive care unit (ICU) management of these patients during their perioperative journey, with an emphasis on the continuity of preintervention planning, referral pathways, and postintervention ICU management. Requests for recipes and doses of medications are frequent; however, as in normal anesthesia and ICU practice, the method of anesthesia and dosing are dependent on the presenting medical/surgical conditions and the underlying anatomy and physiologic reserve. A patient with Fontan palliation in their early 20s attending school full-time with a cavopulmonary connection is likely to have more reserve than a patient in their late 40s with an atriopulmonary Fontan at home waiting for a heart transplant. Each case will require an anesthetic and critical care plan tailored to the situation. The critical care environment is a natural extension of the anesthetic management of a patient, with complex considerations for a patient with Fontan palliation.     

New Directives in Cardiac Imaging: Imaging the Adult With Congenital Heart Disease

Advances in pediatric surgical and interventional techniques and medical care over the past 50 years have revolutionized the care of children with congenital heart disease. Survival to adulthood is now expected and, as such, there is a growing population of adults which is exceeding the pediatric population with congenital heart disease. Noninvasive cardiac imaging with modalities such as echocardiography, computed tomography, and cardiac magnetic resonance imaging are integral to the care of adults with congenital heart disease. These modalities are used for diagnosis, surveillance for complications late after surgery and catheter-based interventions, and in decision-making for medical, interventional, and surgical therapies. In this review we will discuss noninvasive imaging modalities used to assess congenital cardiac lesions, imaging strategies for select congenital lesions, and comment on the future of cardiac imaging in congenital heart disease.     

Cardiac and pericardial tumors: A potential application of positron emission tomography-magnetic resonance imaging

Cardiac and pericardial masses may be neoplastic, benign and malignant, non-neoplastic such as thrombus or simple pericardial cysts, or normal variants cardiac structure can also be a diagnostic challenge. Currently, there are several imaging modalities for diagnosis of cardiac masses; each technique has its inherent advantages and disadvantages. Echocardiography, is typically the initial test utilizes in such cases, Echocardiography is considered the test of choice for evaluation and detection of cardiac mass, it is widely available, portable, with no ionizing radiation and provides comprehensive evaluation of cardiac function and valves, however, echocardiography is not very helpful in many cases such as evaluation of extracardiac extension of mass, poor tissue characterization, and it is non diagnostic in some cases. Cross sectional imaging with cardiac computed tomography provides a three dimensional data set with excellent spatial resolution but utilizes ionizing radiation, intravenous iodinated contrast and relatively limited functional evaluation of the heart. Cardiac magnetic resonance imaging(CMR) has excellent contrast resolution that allows superior soft tissue characterization. CMR offers comprehensive evaluation of morphology, function, tissue characterization. The great benefits of CMR make CMR a highly useful tool in the assessment of cardiac masses.(Fluorine 18) fluorodeoxygluocse(FDG) positron emission tomography(PET) has become a corner stone in several oncological application such as tumor staging, restaging, treatment efficiency, FDG is a very useful imaging modality in evaluation of cardiac masses. A recent advance in the imaging technology has been the development of integrated PET-MRI system that utilizes the advantages of PET and MRI in a single examination. FDG PET-MRI provides complementary information on evaluation of cardiac masses. The purpose of this review is to provide several clinical scenarios on the incremental value of PET and MRI in the evaluation of cardiac masses.     

Assessment of myocardial ischemia and viability using cardiac magnetic resonance

In the past decade, cardiac magnetic resonance (CMR) has evolved dramatically. Its clinical applications are now a major tool in the diagnosis and prognostic assessment of patients with ischemic heart disease. CMR can be used for detection and quantification of ischemia and for viability assessment using different techniques that are now well validated. Scar can be easily detected using contrast enhancement (late gadolinium enhancement). Ischemia detection is usually achieved with stress CMR techniques, whereas prediction for the recovery of function (detection of dysfunctional but viable myocardial segments) can be deduced from scar and stress imaging. Although determination of which approach is better may depend on the population group, the major advantage of CMR is the ability to integrate different information about anatomy, wall motion, myocardial perfusion, and tissue characterization in a single comprehensive examination.     

Occlusion of the coronary sinus ostium: a danger for cavopulmonary anastomosis.

Coronary sinus (CS) ostial obstruction decompressing through a left superior vena cava is a rare finding and presents unique risks to patients requiring a Glenn anastomosis for single-ventricle palliation. Preoperative diagnosis is imperative in order to avoid inadvertent ligation or coil occlusion. We report three cases with single-ventricle physiology and CS obstruction identified at cardiac catheterization. Balloon occlusion angiography proved valuable in making the diagnosis.     

CMR and CT of the Patient With Cardiac Devices: Safety,Efficacy, and Optimization Strategies

Cardiac magnetic resonance (CMR) and cardiac CT (CCT) have evolved into powerful diagnostic tools in the evaluation of patients with cardiovascular diseases. However, the use of these imaging techniques poses potential safety concerns for patients with implanted cardiac devices. These concerns result from the potential for electromagnetic interaction between the device and the CMR field or CCT x-ray radiation, which could lead to device heating, malfunction, or dislocation. Additionally, the presence of cardiac devices may induce significant image artifacts due to local magnetic field inhomogeneities (CMR) or photon starvation/beam hardening (CCT). In this review summarizes the safety issues regarding imaging in patients with cardiac devices. Optimization strategies to mitigate image artifacts and to improve imaging efficacy are discussed.     

Vascular Imaging in Diabetes

Diabetes is a global epidemic affecting individuals of all socioeconomic backgrounds. Despite intensive efforts, morbidity and mortality secondary to the micro- and macrovascular complications remain unacceptably high. As a result, the use of imaging modalities to determine the underlying pathophysiology, early onset of complications, and disease progression has become an integral component of the management of such individuals. Echocardiography, stress echocardiography, and nuclear imaging have been the mainstay of noninvasive cardiovascular imaging tools to detect myocardial ischemia, but newer modalities such as cardiac MRI, cardiac CT, and PET imaging provide incremental information not available with standard imaging. While vascular imaging to detect cerebrovascular and peripheral arterial disease non-invasively has traditionally used ultrasound, CT- and MRI-based techniques are increasingly being employed. In this review, we will provide an outline of recent studies utilizing non-invasive imaging techniques to assist in disease diagnosis as well as monitoring disease progression. In addition, we will review the evidence for newer modalities such as MR spectroscopy, 3D intravascular ultrasound, and optical coherence tomography that provide exquisite detail of metabolic function and coronary anatomy not available with standard imaging, but that have not yet become mainstream.     

Imaging and quantifying valvular heart disease using magnetic resonance techniques

Opinion statement Echocardiography remains the cornerstone of noninvasive valvular heart disease evaluation. There are instances where MRI can be of use. Aside from the obvious advantage where limited acoustic windows are present, cardiac magnetic resonance (CMR) allows for imaging in any desired plane, and advantage can be taken of the ability to align with any regurgitant or stenotic flow jet. The high spatial resolution and contrast allow for accurate detail of valvular anatomy, but it must be remembered that the images represent a composite of eight to 12 heart cycles. For visualizing multiple valvular abnormalities simultaneously, cardiac MRI has a distinct advantage. Finally, a CMR valvular examination can be combined with accurate assessments of left and right ventricular function, myocardial stress perfusion imaging, and detailed viability determinations in a single examination. This provides a comprehensive presurgical evaluation of cardiac physiology.     

Cardiac Imaging in the Geriatric Population: What Do We Think We Know,and What Do We Need to Learn?

Cardiac imaging plays an important role in coronary artery disease (CAD), congestive heart failure (HF) and valvular heart disease (VHD) in the elderly. Imaging defines the structure and function of the cardiac system, refining the understanding of patients' anatomy and physiology and informing a host of clinical care decisions, including prognosis. Yet there is a paucity of evidence to guide the rational use of many imaging modalities in patients of advanced age, a population with considerable clinical heterogeneity, high prevalence and burden of cardiovascular disease (CVD) and atypical presentations of CVD. This paper discusses important considerations for cardiac imaging for older adults, particularly in regard to CAD, VHD and HF, and then presents domains for future research to produce data that would inform clinical care guidelines, appropriate use criteria and imaging lab protocols to address the unique needs of the fast-growing elderly population.     

Cardiac magnetic resonance imaging in Turner syndrome

Girls and women with Turner syndrome (TS) have a highly increased morbidity as the result of cardiovascular disease, both congenital and acquired. Increased clinical use of cardiac magnetic resonance imaging (CMR) in patients with TS over recent years has allowed for characterization of disease not always possible with standard imaging modalities, such as echocardiography (echo). In this review, we discuss the current literature regarding CMR in patients with TS and guidelines for its use.     

Staged single-ventricle palliation in an infant with hemoglobin SC disease

Staged single-ventricle palliation is used to treat many cyanotic congenital heart diseases. Hemoglobin sickle cell disease is associated with anemia and significant vascular sickling sequelae, which increase the risk associated with single-ventricle palliation. To our knowledge, there are no reports in the English-language medical literature of single-ventricle palliation having been performed on a patient who had either sickle cell anemia or sickle cell-hemoglobin C disease. Herein, we discuss our clinical and surgical management of an infant with tricuspid atresia type IA and hemoglobin sickle cell disease who survived single-ventricle palliative procedures through the 2nd stage of a bidirectional Glenn anastomosis.     

Emergency cardiac imaging: state of the art

Multiple strategies and testing modalities are available to evaluate patients presenting to the emergency department with cardiac complaints. Many provide anatomic and prognostic information about coronary stenosis and long-term out-comes. Although nuclear and stress echo imaging have the ability to predict outcomes in patients in the emergency department population, the newer modalities of cardiac imaging (EBCT, MDCT,and CMR) continue to show promising results and may soon be incorporated into emergency department chest pain centers. Protocols can be developed within an institution to meet the needs of the patient population while minimizing risk and improving outcomes for all patients.     

Imaging Heart Failure: Current and Future Applications

A variety of cardiac imaging tests are used to help manage patients with heart failure (HF). This article reviews current and future HF applications for the major noninvasive imaging modalities: transthoracic echocardiography (TTE), single-photon emission computed tomography (SPECT), positron emission tomography (PET), cardiovascular magnetic resonance (CMR), and computed tomography (CT). TTE is the primary imaging test used in the evaluation of patients with HF, given its widespread availability and reliability in assessing cardiac structure and function. Recent developments in myocardial strain, 3-dimensional TTE, and echo contrast appear to offer superior diagnostic and prognostic information. SPECT imaging is a common method employed to detect ischemia and viability in patients with HF; however, PET offers higher diagnostic accuracy for both. Ongoing study of sympathetic and molecular imaging techniques may enable early disease detection, better risk stratification, and ultimately targeted treatment interventions. CMR provides high-quality information on cardiac structure and function and allows the characterization of myocardial tissue. Myocardial late gadolinium enhancement allows the determination of HF etiology and may predict patient outcomes and treatment response. Cardiac CT has become a reliable means for detecting coronary artery disease, and recent advances have enabled concurrent myocardial function, perfusion, and scar analyses. Overall, available imaging methods provide reliable measures of cardiac performance in HF, and recent advances will allow detection of subclinical disease. More data are needed demonstrating the specific clinical value of imaging methods and particularly subclinical disease detection in large-scale, clinical settings.     

Cardiac magnetic resonance imaging in patients with coronary disease

Opinion statement Cardiac magnetic resonance (CMR) has emerged as a versatile noninvasive tool for the comprehensive evaluation of patients with suspected or established coronary artery disease (CAD). In a single imaging session, CMR can assess left ventricular anatomy and function, myocardial perfusion, viability, and coronary luminal stenosis. Using specific pulse sequences, left ventricular global and regional function can be assessed by cine CMR at rest and in response to inotropic stress; first-pass perfusion quantified by vasodilator stress; myocardial viability evaluated by delayed enhancement imaging and also by functional reserve; and coronary artery stenosis assessed by angiography. All these modalities can be achieved with high spatial resolution and image contrast, without exposure to ionizing radiation, and within a reasonable time frame of about 1 hour of scan time. Also, the imaging planes can be programmed to provide identical views of the heart for each type of image, thereby facilitating intermodality comparisons. There is early but accumulating evidence that the accuracy and prognostic values of many of these modalities are comparable or superior to radionuclide scintigraphy and echocardiography in head-to-head studies. Current limitations unique to CMR include the inability to perform exercise stress testing inside the CMR suite and exclusion of patients with indwelling metallic devices such as defibrillators and pacemakers. Despite these limitations, CMR is unique in its multifaceted approach that can be specifically tailored to the clinical question at hand, making it arguably the best tool for the diagnosis and management of CAD. With the rapid pace of advancement in CMR hardware and pulse sequence technologies, the clinical use of this powerful technique is likely to grow even greater in this area.     

Special Considerations in Critical Care of the Congenital Heart Disease Patient

Outcomes of congenital heart disease have improved markedly over the past 20 years, with survival to adulthood now close to 90%. The mean age of admission to an intensive care unit (ICU) is 40 years. The incidence of hospital and critical care admissions have increased significantly as a consequence of this improved survival. Intensivists are now confronted with the management not only of complex adult congenital heart disease (ACHD) lesions from a cardiac perspective, but also of extracardiac organ consequences of years of abnormal circulation after surgical or palliative correction. Kidney and liver dysfunction and respiratory and hematologic abnormalities are very common in this population. ACHD patients can present to the ICU for a vast number of reasons, classified in this review as medical noncardiac, medical cardiac, and surgical. Community/hospital–acquired infections, cerebrovascular accidents, and respiratory failure, alongside arrhythmias and heart failure, are responsible for medical admissions. Surgical admissions include postoperative management after correction or palliation, but also medical optimisation and work-up for advanced therapies. ICU management of this large heterogeneous group requires a thorough understanding of the pathophysiology in order to apply conventional adult critical care modalities; left ventricular or right ventricular dysfunction, pulmonary hypertension, intracardiac, extracardiac, and palliative surgical shunts can be present and require additional consideration. This review focuses on the pathophysiology, long-term sequelae, and different treatment modalities to supply a framework for the ICU physician caring for these patients. Successful outcome, especially in complex lesions, depends on early involvement of specialised ACHD centres.