Adult CHD

This article provides a broad overview of key concepts and more commonly encountered critical illness presentations in adult congenital heart disease (ACHD) patients. General principles are discussed, and the need for ACHD subspecialty consultation is emphasized. ACHD is categorized based on hemodynamic profile, and common clinical presentations are reviewed, including common pitfalls. Many ACHD lesions are associated with predictable complications, and awareness of these associations can guide evaluation and management, which are listed in this article...

The number of patients with congenital heart disease (CHD) undergoing ambulatory surgery is increasing. Deciding whether a CHD patient is suitable for an ambulatory procedure is still challenging. Several factors must be considered, including the type of planned procedure, the complexity of the underlying pathology, the American Society of Anesthesiologists' Physical Status classification of the patient, and other patient-specific factors, including comorbidity, chronic complications of CHD, medication, coagulation disorders, and issues related to the presence of a pacemaker (PM) or cardioverter-defibrillator...

There have been no published prospective randomized clinical trials that have: (1) established an association between invasive dental and nondental invasive procedures and risk of infective endocarditis; or (2) defined the efficacy and safety of antibiotic prophylaxis administered in the setting of invasive procedures in the prevention of infective endocarditis in high-risk patients. Moreover, previous observational studies that examined the association of nondental invasive procedures with the risk of infective endocarditis have been limited by inadequate sample size...

There are marked variations in the incidence of sudden cardiac death (SCD) and in the substrates for ventricular arrhythmias (VAs) across the gamut of congenital heart defects. In this 2-part review, patients with higher-risk forms of congenital heart disease (CHD) were conceptually categorized into those with discrete anatomic isthmuses for macro-reentrant ventricular tachycardia (VT) (Group A) and those with more diffuse or less well-defined substrates (Group B) that include patchy or extensive myocardial fibrosis...

Patients with congenital heart disease associated with a higher risk for ventricular arrhythmias (VA) and sudden cardiac death (SCD) can be divided conceptually into those with discrete mechanisms for reentrant monomorphic ventricular tachycardia (VT) (Group A) and those with more diffuse substrates (Group B). Part I of this review addresses Group A lesions, which predominantly consist of tetralogy of Fallot and related variants. Well-defined anatomic isthmuses for reentrant monomorphic VT are interposed between surgical scars and the pulmonary or tricuspid annulus...

BACKGROUND: Congenital heart anomalies are the most common type of organ malformation, affecting approximately 1% of all newborn infants. More than 90% of these children now survive into adulthood. They need to be cared for by specialists for adults with congenital heart disease (ACHD), as well as by family physicians, internists, and cardiologists who are adequately versed in the basic management of persons with this lifelong condition. METHODS: This review is based on pertinent publications retrieved by a selective literature search, including guidelines and consensus statements from Germany and abroad...

PURPOSE OF REVIEW: A growing number of adult patients with congenital heart disease (ACHD) are entering the healthcare system as a result of advances in the diagnosis and management of congenital heart defects. Heart failure is a common final pathway for this diverse patient population, representing the leading cause of mortality in ACHD patients. Herein, we review present guideline-directed management of heart failure in ACHD patients. RECENT FINDINGS: There exists a dearth of data to guide management of ACHD-related heart failure...

Together, heart failure and arrhythmia represent the most important cardiovascular sources of morbidity and mortality among adults with congenital heart disease (ACHDs). Although traditionally conceptualized as operating within 2 distinct clinical silos, these scenarios frequently coexist within the same individual; consequently the mechanistic, therapeutic, and prognostic overlap between them demands increased recognition. In fact, given the near ubiquity of heart failure and arrhythmia among ACHDs, there is perhaps no other arena within cardiology where this critical intersection is more frequently observed...

Owing to the great advances in the care for children with congenital heart disease by paediatric cardiac surgeons and cardiologists, there are ever increasing numbers of patients with congenital heart disease who reach adult life. At some stage during the late teenage years or soon after, these patients 'transition' from paediatric cardiac care to surveillance by cardiologists who look after adults. Many such specialists, however, are more familiar with commoner acquired heart problems such as coronary disease, heart failure, and arrhythmia in structurally normal hearts and less familiar with congenital heart disease...

The epidemiology of congenital heart disease (CHD) has changed in the past 50 years as a result of an increase in the prevalence and survival rate of CHD. In particular, mortality in patients with CHD has changed dramatically since the latter half of the twentieth century as a result of more timely diagnosis and the development of interventions for CHD that have prolonged life. As patients with CHD age, the disease burden shifts away from the heart and towards acquired cardiovascular and systemic complications...

Although major breakthroughs in the field of pediatric cardiology, cardiac surgery, intervention, and overall care improved the outlook of congenital heart disease, Eisenmenger syndrome (ES) is still encountered and remains a complex clinical entity with multisystem involvement, including secondary erythrocytosis, increased thrombotic and bleeding diathesis, high arrhythmogenic risk, progressive heart failure, and premature death. Clearly, care for ES is best delivered in multidisciplinary expert centers. In this review, we discuss the considerable recent progress in understanding the complex pathophysiology of ES, means of prognostication, and improvement in clinical outcomes achieved with pulmonary arterial hypertension-targeted therapies...

Congenital heart disease is one of the most common causes of death derived from malformations. Historically, its treatment has depended on timely diagnosis and early pharmacological and surgical interventions. Survival rates for patients with this disease have increased, primarily due to advancements in therapeutic choices, but mortality remains high. Since this disease is a time-sensitive pathology, pharmacological interventions are needed to improve clinical outcomes. Therefore, we analyzed the applications, dosage, and side effects of drugs currently used for treating congenital heart disease...

OBJECTIVE: Current data on intensive care unit (ICU) admissions in patients with adult congenital heart disease (ACHD) are limited and focus on admissions after elective cardiac surgery. This study describes non-elective ICU admissions in patients with ACHD. METHODS: A retrospective matched cohort study was performed from January 2000 until December 2015 in a tertiary care centre ICU (there was no cardiac care unit). Primary outcomes were short-term (during hospital stay or <30 days after discharge) and long-term (>30 days after discharge until end of follow-up) mortality...

Pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) affects 5% to 10% of adults with CHD and is associated with significant morbidity and mortality. PAH-CHD develops as a consequence of intracardiac or extracardiac systemic-to-pulmonary shunts that lead to pulmonary vascular remodeling through a pathologic process that is similar to other causes of PAH. Eisenmenger syndrome is the most severe phenotype of PAH-CHD and is characterized by severe elevation in pulmonary vascular resistance, with shunt reversal causing hypoxemia and central cyanosis...

Maternal morbidity and mortality as a result of cardiac disease is increasing in the United States. Safe management of pregnancy in women with heart disease requires appropriate anesthetic, cardiac, and obstetric care. The anesthesiologist should risk stratify pregnant patients based upon cardiac disease etiology and severity in order to determine the appropriate type of hospital and location within the hospital for delivery and anesthetic management. Increased intrapartum hemodynamic monitoring may be necessary and neuraxial analgesia and anesthesia is typically appropriate...

PURPOSE: Adults with congenital heart disease (ACHD) are a rapidly growing population with ever-increasing complexity, and intensive care unit (ICU) management is often necessary. This review summarizes common cardiovascular and non-cardiovascular complications in ACHD and provides a framework for ICU care. RECENT FINDINGS: Heart failure is the leading cause of hospitalization and mortality in ACHD. Varied anatomy and repairs, as well as differing physiological complications, limit generalized application of management algorithms...

Presently, with increasing survival of patients with congenital heart disease (CHD), pulmonary arterial hypertension (PAH) associated with CHD is commonly encountered in children and adults. This increased prevalence is seen despite significant advances in early diagnosis and surgical correction of patients with structural CHD. PAH is the cause of significant morbidity and mortality in these patients and comes in many forms. With the increased availability of targeted therapies for PAH, there is hope for improved hemodynamics, exercise capacity, quality of life, and possibly survival for these patients...

OBJECTIVE: Pharmacological options for patients with a failing systemic right ventricle (RV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are not well defined. This study aims to investigate the feasibility and effects of sacubitril/valsartan treatment in a single-centre cohort of patients. METHODS: Data on all consecutive adult patients (n=20, mean age 46 years, 50% women) with a failing systemic RV in a biventricular circulation treated with sacubitril/valsartan in our centre are reported...

We are witnessing an unparalleled pandemic caused by the novel Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) associated with coronavirus disease 2019 (COVID-19). Current data show that SARS-CoV-2 results in mild flu-like symptoms in the majority of healthy and young patients affected. Nevertheless, the severity of COVID-19 respiratory syndrome and the risk of adverse or catastrophic outcomes are increased in patients with pre-existing cardiovascular disease. Patients with adult congenital heart disease (ACHD)-by definition-have underlying cardiovascular disease...

Women with congenital heart disease are pursuing pregnancy in increasing numbers. Counseling about genetic transmission, medication management, maternal and fetal risks, and maternal longevity should be initiated well before pregnancy is considered. Although preconception medical and surgical optimization as well as coordinated multidisciplinary care throughout pregnancy decrease maternal and fetal risks, the rate of complications remains increased compared with the general population. Lesion-specific risk stratification and care throughout pregnancy further improve outcomes and decrease unnecessary interventions...