Improving medical care and prevention in adults with congenital heart disease—reflections on a global problem—part I: development of congenital cardiology, epidemiology, clinical aspects, heart failure, cardiac arrhythmia

Abstract

Today most patients with congenital heart defects (CHD) survive into adulthood. Unfortunately, despite relevant residua and sequels, follow-up care of adults with congenital heart disease (ACHD) is not performed in specialized and/or certified physicians or centres. Major problems in the long-term course encompass heart failure, cardiac arrhythmias, heart valve disorders, pulmonary vascular disease, infective endocarditis, aortopathy and non-cardiac comorbidities. Many of them manifest themselves differently from acquired heart disease and therapy regimens from general cardiology cannot be transferred directly to CHD. It should be noted that even simple, postoperative heart defects that were until recently considered to be harmless can lead to problems with age, a fact that had not been expected so far. The treatment of ACHD has many special features and requires special expertise. Thereby, it is important that treatment regimens from acquired heart disease are not necessarily transmitted to CHD. While primary care physicians have the important and responsible task to set the course for adequate diagnosis and treatment early and to refer patients to appropriate care in specialized ACHD-facilities, they should actively encourage ACHD to pursue follow-up care in specialized facilities who can provide responsible and advanced advice. This medical update emphasizes the current data on epidemiology, heart failure and cardiac arrhythmia in ACHD.

Facts about the General Medical Care of Adults with Congenital Heart Defects: Experience of a Tertiary Care Center

“…As indicated in the questionnaire, the numerous and serious typical long-term CHD complications seen by PCPs include life-threatening cardiac problems such as heart failure, pulmonary hypertension, arrhythmia, and infective endocarditis. These all have a major impact on morbidity and mortality in ACHD [14,27,28].…”

“…Another issue to bear in mind is that cardiac problems in ACHD often manifest themselves differently from those in acquired heart disease patients. Established treatment regimens for acquired heart defects do not necessarily transfer to CHD [17,28,30].…”

Proposed method for evaluation and categorization of functional capacity of children, adolescents, and adults with cardiac diseases to bring them in existing social justice system by creating the cardiac disability criteria

“…The combined cardiopulmonary dysfunction in these patients limits the capacity to initiate, sustain, or complete even routine activities. Up to 25% of complex CHDs may present with HF in adulthood [4,26,27]. The operated patients may also have suboptimal FC in the presence of residual defects, ventricular dysfunction, chronotropic incompetence, tachyarrhythmia, heart block, SAH, PAH, PVH, prosthesis implantation, post-cardiac transplant deconditioning, IE, and co-existing multi-organ dysfunction [2,[22][23][24][25][28][29][30][31][32][33][34][35][36][37][38][39][40].…”

“…The information obtained from echo or other imaging modalities is pertinent to diagnosis and are ancillary to the overall assessment of functional capacity. Nevertheless, functional capacity as we know is the ability to initiate and sustain accustomed and unaccustomed exercise, indicating the efficiency of the integrated functioning of the cardiovascular-pulmonary unit, in the presence of a cardiac disease [19][20][21][22][23][24][25][26][27][28][29]. Determination of peak VO2/ METs in response to exercise (submaximal treadmill or bicycle exercise testing) or 6-, 9-, and 12-min walk tests are the objective methods described in the literature to assess FC of a patient with the compromised cardiac status [44][45][46][47][48][49][50].…”

“…Need for a multidisciplinary approach for patients going through cardiac rehabilitation programs [3,4,11,12,26,27] A post-intervention, cardiac rehabilitation program improves long-term survival, quality of life, and psychological wellbeing [68][69][70]. These programs must have a cost-effective multidisciplinary approach to handle the multiple issues like treatment of co-morbid factors, patient's (and parent's) education, physiotherapy, neuro-developmental growth, psychiatric problems, speech, visual and hearing impairment, and underlying genetic syndromes [69,70].…”