Changing epidemiology and mortality in adult congenital heart disease: looking into the future

Advances in surgical and medical treatment of children born with congenital heart disease have led to a growing number of adult survivors, particularly to a growing number of adults with complex congenital heart disease. Childhood mortality has continuously decreased over the last few decades and mortality has shifted almost entirely to adulthood. However, most patients are not cured and many remain at risk of premature death. The extent of excess mortality among individual congenital disease entities is not well defined. In this article we outline the current demographics of adults with selected congenital heart lesions. Based on these contemporary patient cohorts, we delineate future changes in patient demographics. A better understanding of these trends may help in the optimal planning of future resource allocation for medical care and optimal planning of multicenter research, for this novel and growing population of young, chronically ill adults.     

The adult with congenital heart disease

Internal medicine cardiovascular specialists and internists are going to be seeing increasing numbers of adult patients with congenital heart malformations, and most of them will have had one or more surgical or therapeutic catheter procedures. The nonsurgical cases will have either benign defects or important but inoperable anomalies, or the physician may encounter the rare case which, though amenable to surgery, has escaped operation until adult life. Among patients with postoperative congenital heart disease, a few are totally cured and require no special follow-up or any special precautions, e.g., those with certain cases of atrial septal defect of ostium secundum types, patent ductus arteriosus without pulmonary hypertension, and fully repaired total anomaly of pulmonary venous return. Others who are very much improved by definitive repair have residual defect for which surgical treatment was not attempted, e.g., bicuspid aortic valve in the patient who had surgical repair of coarctation or the patient with persistent cleft of a mitral valve leaflet for which no repair was attempted at the time of closure of the ostium primum type of atrial septal defect. Some patients have had incomplete and unsuccessful repair of a defect but yet are symptomatically improved compared with their preoperative status, e.g., those with tetralogy of Fallot with loss of right-to-left shunting by closure of the ventricular septal defect but persistent right ventricular outflow tract or pulmonary artery branch obstruction. Patients with persistently high intracardiac pressure from unrelieved obstruction are at higher risk for sudden death than they were preoperatively, especially those with a high grade of ventricular ectopy. Despite symptomatic improvement compared with the preoperative status, such cases must have reoperation, if feasible, as a means to prevent a sudden catastrophe. Patients with congenital malformations have symptoms that are distinctly different from those with acquired heart disease. Furthermore, their reactions to their problems and to their lives and work in relation to others are often colored by their restricted and overprotected childhoods, which were often filled with doctor visits, several admissions to the hospital, one or more operations, and usually several heart catheterizations. Otherwise, these patients are capable of the same level of achievement intellectually, and only a few have important defects of other systems that would interfere with their roles as independent and productive adults. The problem arises as to what group of physicians will care for these patients in the future.(ABSTRACT TRUNCATED AT 400 WORDS)     

复杂畸形成人先天性心脏病的外科治疗分析

目的 对167例复杂畸形成人先天性心脏病（ACHD）病例的外科治疗结果进行总结分析。方法 对阜外医院2012年1月~ 2015年2月共167例复杂畸形ACHD患者的病种、手术术式及术后早期结果进行总结和分析。结果 完成矫治手术135例,姑息手术30例,二次手术心包开窗术2例,发病最多的前3位病种依次为法洛氏四联症（TOF）,部分型肺静脉异位引流（PAPVC）和完全型心内膜垫缺损TECD。术后死亡2例,死亡率1.19%。术后并发症36例,发生率21.6%。术后患者呼吸机辅助平均时间（26±13）h。ICU治疗时间（3.2±1.6）d。结论 虽然复杂畸形ACHD手术存在一定难度,但手术术式及围术期处理得当,仍可获得满意的效果。     

Care of the adult with congenital heart disease

Opinion statement  The population of adults with congenital heart disease (CHD) is growing to the extent that there are now more adults than children with CHD. As this population ages, these patients will need optimal medical care, will be at risk for clinically significant noncardiac comorbidities, and will require methods to decrease adverse outcomes. Recognition of extracardiac risk factors and a multidisciplinary approach to management are essential in caring for this unique group of patients.     

Epidemiology of adult congenital heart disease among the general population in Kuwait

BackgroundAdult congenital heart disease (ACHD) is a highly underrepresented entity in medical literature, especially in the middle‐eastern region.HypothesisThis study is the first to assess the prevalence of adult congenital heart disease among the population of Kuwait.MethodsAfter a retrospective register review of patients in Kuwait being followed up in the chest diseases hospital was conducted, patients who fit the inclusion criteria were enrolled in the study. Using the American College of Cardiology Task Force 1 of the 32nd Bethesda conference classification of the severity of ACHD, the patients were classified into those with simple, moderate, and complex congenital heart diseases. The age and gender of the patients, as well as the type repair performed, and the residual cardiac findings were recorded to assess the association between the complexity and residuals. Associations were assessed using STATA 15.ResultsA total of 611 patients were evaluated over a period of 18 months. The youngest participant was 20 years of age, and the oldest participant was 88 years old. Male participants with moderate congenital heart disease class were more common in our study population. Patients with complex congenital heart disease have more residual cardiac lesion than the moderate or simple groups. Almost (70%) of patients with complex cardiac anomalies have undergone either partial or complete repair. The most prevalent cardiac defect was atrial septal defect (21.5%). Tetralogy of Fallot was the most prevalent defect in the moderate group, representing (13%) of the group. The most prevalent anomaly in the complex group was double outlet right ventricle (DORV) representing (15.38%).ConclusionAdult Congenital heart disease is a growing entity of heart disease due to advanced repair techniques. This population requires registries to document cases and assign specialists for the management and care of this special group of patients.Highlights

• First database of adult congenital heart disease in Kuwait.
• The most prevalent heart defect was ASD in Kuwait.
• TOF was the most prevalent defect in the moderate group; and DORV was the most prevalent in the complex group.
• Patients with moderate ACHD tended to have a more complete repair than those in the complex group.

     

Transition and transfer from pediatric to adult care of the young adult with complex congenital heart disease

This article focuses first on the process of transition and transfer of care of young adults with complex congenital heart disease. It defines the transition process and briefly discusses its history. It reviews the important aspects of transition, outlines the key elements of a successful transition program, and provides a curriculum appropriate for the young adult with congenital heart disease. Finally, it identifies the barriers to transfer of care, discusses the importance of a policy on timing, outlines the components of adult provider services that may be needed, and reviews the steps to an orderly transfer process.     

Right atrial size relates to right ventricular end-diastolic pressure in an adult population with congenital heart disease

Aim: Noninvasive markers of right ventricular (RV) diastolic dysfunction are limited by their lack of reproducibility and accuracy. We tested the hypothesis that right atrial (RA) size measured by echocardiography was correlated to invasive parameters of RV diastolic filling. Methods and Results: We studied 31 consecutive adult patients with congenital heart disease. From 2D echocardiography images, we measured maximal RA long‐axis and short‐axis lengths, area and volume. We compared each of these measures to right ventricular end‐diastolic pressure (RVEDP) and mean right atrial pressure (mRAP) measured by right heart catheterization. RA long‐axis, short‐axis, area, and volume correlated significantly with RVEDP (r = 0.78, P < 0.001; r = 0.61, P < 0.001; r = 0.79, P < 0.001; and r = 0.75, P < 0.001, respectively) and mRAP (r = 0.66, P < 0.001; r = 0.56, P = 0.002; r = 0.70, P < 0.001; r = 0.68, P < 0.001, respectively). Single cut points for each echocardiographic parameter demonstrated reasonable accuracy to rule‐in and rule‐out RVEDP ≥7 mm Hg (sensitivity = 74%, specificity = 82%, positive LR = 4.1, negative LR = 0.32 for RA long‐axis of 49 mm; sensitivity = 89%, specificity = 82%, positive LR = 4.9, negative LR = 0.12 for RA area of 14 cm²; sensitivity = 89%, specificity = 82%, positive LR = 4.9, negative LR = 0.13 for RA volume of 37 mL). Conclusion: RA size measured by echocardiography is strongly correlated to invasive parameters of RV diastolic filling and predicts high RV end‐diastolic pressure. (Echocardiography 2011;28:109‐116)     

Evaluation of athletes with complex congenital heart disease

As a result of improvements in congenital heart surgery, there are more adults alive today with congenital heart disease (CHD) than children. Individuals with cardiac birth defects may be able to participate in physical activities but require proper cardiovascular evaluation. The American Heart Association and American College of Cardiology released guidelines in 2015 for athletes with cardiovascular abnormalities. The guidelines express that although restriction from competitive athletics may be indicated for some, the majority of individuals with CHD can and should engage in some form of physical activity. This case study demonstrates the importance of combining all aspects of history, physical examination, ECG, and imaging modalities to evaluate cardiac anatomy and function in young athletes with complex CHD.