Orthotopic Heart and Combined Heart Liver Transplantation: the Ultimate Treatment Option for Failing Fontan Physiology.

Purpose of the Review: This is a comprehensive update on failing Fontan physiology and the role of heart and combined heart and liver transplantation in the current era. Recent Findings: Single ventricle physiology encompasses a series of rare congenital cardiac abnormalities that are characterized by absence of or hypoplasia of one ventricle. This effectively results in a single ventricular pumping chamber. These abnormalities are rarely compatible with long-term survival if left without surgical palliation in the first few years of life. Surgical treatment of single ventricle physiology has evolved over the past 60 years and is characterized by numerous creative innovations. These include the development of arteriopulmonary shunts, the evolution of partial cavopulmonary connections, and the eventual development of the "Fontan" operation. Regardless of the type of Fontan modification, the long-term consequences of the Fontan operation are predominantly related to chronic central venous hypertension and the multi-organ consequences thereof. Atrial arrhythmias can further compromise this circulation.Patients with single ventricle physiology represent a special sub-segment of congenital cardiac transplants and are arguably the most challenging patients considered for transplantation. Summary: This review describes in detail the challenges and opportunities of heart and liver transplantation in Fontan patients, as viewed and managed by the experienced team at the Ahmanson/UCLA Adult Congenital Heart Center.

Perioperative Echocardiography in the Adult With Congenital Heart Disease.

Survival of patients with congenital heart disease has significantly improved over the last 2 decades, confronting interventionalists, surgeons, anesthesiologists, cardiologists, and intensivists with often unfamiliar complex pathophysiology in the perioperative setting. Aside from cardiac catheterization, echocardiography has become the main imaging modality in the hospitalized adult with congenital heart disease. The great variety of congenital lesions and their prior surgical management challenges practitioners to generate optimal imaging, reporting, and interpretation of these complex anatomic structures. Standardization of echocardiographic studies can not only provide significant benefits in the surveillance of these patients, but also facilitate understanding of pathophysiologic mechanism and assist clinical management in the perioperative setting. Knowledge in obtaining and interpreting uniform imaging protocols is essential for the perioperative clinician. In this publication, the authors review current international consensus recommendations on echocardiographic imaging of adults with congenital heart disease and describe the fundamental components by specific lesion. The authors will emphasize key aspects pertinent to the clinical management when imaging these patients in the perioperative setting. The goal of this review is to familiarize the perioperative physician on how to structure and standardize echocardiographic image acquisition of congenital heart disease anatomy for optimal clinical management.

Critical Care of Patients After Pulmonary Thromboendarterectomy.

Pulmonary thromboendarterectomy (PTE) remains the only curative surgery for patients with chronic thromboembolic pulmonary hypertension (CTEPH). Postoperative intensive care unit care challenges providers with unique disease physiology, operative sequelae, and the potential for detrimental complications. Central concerns in patients with CTEPH immediately after PTE relate to neurologic, pulmonary, hemodynamic, and hematologic aspects. Institutional experience in critical care for the CTEPH population, a multidisciplinary team approach, patient risk assessment, and integration of current concepts in critical care determine outcomes after PTE surgery. In this review, the authors will focus on specific aspects unique to this population, with integration of current available evidence and future directions. The goal of this review is to provide the cardiac anesthesiologist and intensivist with a comprehensive understanding of postoperative physiology, potential complications, and contemporary intensive care unit management immediately after pulmonary endarterectomy.

Regional Left Ventricular Myocardial Dysfunction After Cardiac Surgery Characterized by 3-Dimensional Strain.

BACKGROUND: Three-dimensional (3D) strain is an echocardiographic modality that can characterize left ventricular (LV) function with greater accuracy than ejection fraction. While decreases in global strain have been used to predict outcomes after cardiac surgery, changes in regional 3D longitudinal, circumferential, radial, and area strain have not been well described. The primary aim of this study was to define differential patterns in regional LV dysfunction after cardiac surgery using 3D speckle tracking strain imaging. Our secondary aim was to investigate whether changes in regional strain can predict postoperative outcomes, including length of intensive care unit stay and 1-year event-free survival. METHODS: In this prospective clinical study, demographic, operative, echocardiographic, and clinical outcome data were collected on 182 patients undergoing aortic valve replacement, mitral valve repair or replacement, coronary artery bypass graft, and combined cardiac surgery. Three-dimensional transthoracic echocardiograms were performed preoperatively and on the second to fourth postoperative day. Blinded analysis was performed for LV regional longitudinal, circumferential, radial, and area strain in the 17-segment model. RESULTS: Regional 3D longitudinal, circumferential, radial, and area strains were associated with differential patterns of myocardial dysfunction, depending on the surgical procedure performed and strain measure. Patients undergoing mitral valve repair or replacement had reduced function in the majority of myocardial segments, followed by coronary artery bypass graft, while patients undergoing aortic valve replacement had reduced function localized only to apical segments. After all types of cardiac surgery, segmental function in apical segments was reduced to a greater extent as compared to basal segments. Greater decrements in regional function were seen in circumferential and area strain, while smaller decrements were observed in longitudinal strain in all surgical patients. Both preoperative regional strain and change in regional strain preoperatively to postoperatively were correlated with reduced 1-year event-free survival, while postoperative strain was not predictive of outcomes. Only preoperative strain values were predictive of intensive care unit length of stay. CONCLUSIONS: Changes in regional myocardial function, measured by 3D strain, varied by surgical procedure and strain type. Differences in regional LV function, from presurgery to postsurgery, were associated with worsened 1-year event-free survival. These findings suggest that postoperative changes in myocardial function are heterogeneous in nature, depending on the surgical procedure, and that these changes may have long-term impacts on outcome. Therefore, 3D regional strain may be used to identify patients at risk for worsened postoperative outcomes, allowing early interventions to mitigate risk.

Extravascular lung water monitoring for thoracic and lung transplant surgeries.

PURPOSE OF REVIEW: Excessive accumulation of extravascular lung water (EVLW) resulting in pulmonary edema is the most feared complication following thoracic surgery and lung transplant. ICUs have long relied on chest radiography to monitor pulmonary status postoperatively but the increasing recognition of the limitations of bedside plain films has fueled development of newer technologies, which offer earlier detection, quantitative assessments, and can aide in preoperative screening of surgical candidates. In this review, we focus on the emergence of transpulmonary thermodilution (TPTD) and lung ultrasound with a focus on the clinical integration of these modalities into current intraoperative and critical care practices. RECENT FINDINGS: Recent studies demonstrate transpulmonary thermodilution and lung ultrasound provide greater sensitivity and earlier detection of lung water accumulation and are useful to guide clinical management. Assessments from these techniques have predictive value of postoperative outcome. Further, EVLW assessment shows promise as a preoperative screening tool in lung transplant patients. SUMMARY: Monitoring EVLW in the perioperative period offers clinicians a powerful tool to guide fluid therapy and manage pulmonary edema. Both TPTD and lung ultrasound have unique attributes in the care of thoracic surgery and lung transplant patients.

Management of the Critically Ill Adult With Congenital Heart Disease.

Survival of adults with congenital heart disease (CHD) has improved significantly over the last 2 decades, leading to an increase in hospital and intensive care unit (ICU) admissions of these patients. Whereas most of the ICU admissions in the past were related to perioperative management, the incidence of medical emergencies from long-term sequelae of palliative or corrective surgical treatment of these patients is rising. Intensivists now are confronted with patients who not only have complex anatomy after congenital cardiac surgery, but also complex pathophysiology due to decades of living with abnormal cardiac anatomy and diseases of advanced age. Comorbidities affect all organ systems, including cognitive function, pulmonary and cardiovascular systems, liver, and kidneys. Critical care management requires an in-depth understanding of underlying anatomy and pathophysiology in order to apply contemporary concepts of adult ICU care to this population and optimize patient outcomes. In this review, the main CHD lesions and their common surgical management approaches are described, and the sequelae of CHD physiology are discussed. In addition, the effects of chronic comorbidities on the management of critically ill adults are explored, and the adjustments of current ICU management modalities and pharmacology to optimize care are discussed.

Assessment of Pulmonary Edema: Principles and Practice.

Pulmonary edema increasingly is recognized as a perioperative complication affecting outcome. Several risk factors have been identified, including those of cardiogenic origin, such as heart failure or excessive fluid administration, and those related to increased pulmonary capillary permeability secondary to inflammatory mediators. Effective treatment requires prompt diagnosis and early intervention. Consequently, over the past 2 centuries a concentrated effort to develop clinical tools to rapidly diagnose pulmonary edema and track response to treatment has occurred. The ideal properties of such a tool would include high sensitivity and specificity, easy availability, and the ability to diagnose early accumulation of lung water before the development of the full clinical presentation. In addition, clinicians highly value the ability to precisely quantify extravascular lung water accumulation and differentiate hydrostatic from high permeability etiologies of pulmonary edema. In this review, advances in understanding the physiology of extravascular lung water accumulation in health and in disease and the various mechanisms that protect against the development of pulmonary edema under physiologic conditions are discussed. In addition, the various bedside modalities available to diagnose early accumulation of extravascular lung water and pulmonary edema, including chest auscultation, chest roentgenography, lung ultrasonography, and transpulmonary thermodilution, are examined. Furthermore, advantages and limitations of these methods for the operating room and intensive care unit that are critical for proper modality selection in each individual case are explored.