The timing of follow-up at ACHD centers varies according to the complexity of heart disease. Individuals with simple CHD should be evaluated at an ACHD center at least once to determine the need for further follow-up. Patients with moderate and complex CHD must be monitored at a minimum of every 12 to 24 months, whereas very complex CHD should be monitored every 6 to 12 months.23 The frequency with which the young adult population moves may hinder adequate continuity of care and long-term follow up; a searchable directory of ACHD clinics in the United States and Canada can be found at www.achaheart.org/your-heart/clinic-directory/clinic-listings/.
Managing Specific Issues in the Transitioning Patient
Arrhythmias and Heart Failure
As mentioned, arrhythmias in the ACHD population are extremely common, the most frequent being atrial arrhythmias, especially in patients who have undergone single-ventricle repairs. Patients with late repair of an atrial septal defect have a high incidence of supraventricular tachycardia, which can be treated with catheter ablation procedures.45,46 Pacemaker implantation is another therapeutic option, especially in those who have undergone atrial surgery (ie, Mustard or Senning repairs). In these individuals, particularly in adolescents, abdominal implantation of a pacemaker generator may lessen the psychological impact of the external appearance of the pacemaker. In this population avoiding blunt contact sports (ie, tackle football, wrestling) is also important.28 It is critical that adult and pediatric electrophysiologists work together in the care and management of these complex, recurrent arrhythmias.
As noted above, many ACHD patients will require surgical or catheter-based interventions (as high as 40% in 1 study),47 and many encounter late-onset morbidity as a sequela of interventions earlier in life or as a result of failure of these interventions. The key for adult cardiologists and ACHD providers is delineating the reversible causes (eg, residual shunts, progressive valve regurgitation, and recoarctation) through routine intermittent surveillance, including echocardiograms, magnetic resonance imaging, and cardiac catherization, so that heart failure and arrhythmias in these patients can be identified, treated, and even prevented.
Pregnancy
Pregnancy is the most common reason for women to reenter care. Pregnancy is associated with significant hemodynamic changes, resulting in an increase in cardiac output to up to 150% of pre-pregnancy levels at 32 weeks, and up to 180% during labor. The outcome of pregnancy in patients with CHD is favorable in most instances provided that functional class systemic ventricular function is good. Accordingly, pregnancy is contraindicated in instances of severe pulmonary arterial hypertension (eg, Eisenmenger’s physiology), systemic ventricular dysfunction, and severe left-sided obstructions (eg, aortic or mitral stenosis). It is therefore imperative for health care providers to address the risks of pregnancy and the need for contraception with women who have CHD and are of reproductive age. The AHA advises beginning this conversation at 12 years of age and recommends that counseling be provided by health care providers knowledgeable in both CHD and adolescent health.27 Given the thrombotic potential of estrogen-containing contraception, the selection of contraception for women with ACHD who are seeking birth control requires discussion between the health care provider and patient. Though there have been limited studies performed on the use of contraception in women with CHD, a British working group has developed a consensus statement regarding contraceptive use in women with heart disease based on the World Health Organization format.48,49
Surgical Procedures
The need for operative interventions and re-interventions, both cardiac and noncardiac, in many CHD populations is considerable. Regardless of the type of procedure, these patients should receive a comprehensive preoperative risk assessment as well as appropriate intraoperative and postoperative management, ideally at a center equipped to meet their unique needs. Approaching the surgical procedure under the guidance of an interdisciplinary team that includes an ACHD specialist, anesthesiologist, and surgeon ensures that critical issues for appropriate management are not overlooked.
The preoperative risk assessment should be aimed at identifying and minimizing major risk factors. Historical factors to consider include the congenital lesion, outcomes of prior surgeries, history of syncope or arrhythmias, and the presence of pulmonary disease, among others.27 If the patient has a pacemaker or defibrillator, this should be interrogated prior to the planned procedure to ensure proper functioning. The preoperative evaluation should include consultation with a cardiologist experienced in the care of adolescents with CHD. Cardiac medications should be continued until the time of surgery and restarted as soon after the procedure as possible. Periods without anticoagulation should be minimized if indicated at baseline, and may require substituting warfarin with heparin in the preoperative period. The need for endocarditis prophylaxis must be considered as well; antibacterial prophylaxis prior to dental surgery, respiratory tract procedures, and procedures on infected skin and musculoskeletal structures is recommended in individuals with prosthetic heart valves, previous infective endocarditis, unrepaired CHD, repaired CHD with prosthetic material for the first 6 months after surgery, repaired CHD with residual defects, and valvulopathy after cardiac transplantation.50
Fluid management is important intraoperatively and post procedure, particularly in individuals who are preload dependent at baseline (eg, patients who have had Fontan palliation). Mechanical ventilation strategies with high positive end-expiratory pressure and tidal volume may decrease systemic venous return and should be monitored closely. Early mobilization and pulmonary toilet post extubation is advised to avoid pulmonary infection.
