Loss to Specialist Follow-up in Congenital Heart Disease
Objective To evaluate the scale and clinical importance of loss to follow-up of past patients with serious congenital heart disease, using a common malformation as an example. To better understand the antecedents of loss to specialist follow-up and patients' attitudes to returning.
Design Cohort study using NHS number functionality. Content and thematic analysis of telephone interviews of subset contacted after loss to follow-up.
Patients, intervention and setting Longitudinal follow-up of complete consecutive list of all 1085 UK patients with repair of tetralogy of Fallot from single institution 1964–2009.
Main outcome measures Survival, freedom from late pulmonary valve replacement, loss to specialist follow-up, shortfall in late surgical revisions related to loss to follow-up. Patients' narrative about loss to follow-up.
Results 216 (24%) of patients known to be currently alive appear not to be registered with specialist clinics; some are seen in general cardiology clinics. Their median age is 32 years and median duration of loss to follow-up is 22 years; most had been lost before Adult Congenital services had been consolidated in their present form. 48% of the late deaths to date have occurred in patients not under specialist follow-up. None of those lost to specialist follow-up has had secondary pulmonary valve replacement while 188 patients under specialist care have. Patients lost to specialist follow-up who were contacted by telephone had no knowledge of its availability.
Conclusions Loss to specialist follow-up, typically originating many years ago, impacts patient management.
Open heart surgery for congenital heart disease began in the 1960s in the UK. Its progressive success has meant that since around 2002, adult survivors of surgery outnumber children with congenital heart disease in the UK. The same document estimated that there are around 130 500 adults with 'moderate' or 'complex' congenital heart disease alive in England and Wales today; almost all are survivors of surgery they had as young children. For many complex congenital heart diseases, residual haemodynamic problems can result in late heart failure, reduced exercise capacity, arrhythmias or sudden death and there are risks associated with pregnancy and non-cardiac surgery. Most premature deaths associated with congenital heart disease are now occurring in adults rather than children.
Tetralogy of Fallot is the commonest 'cyanotic' congenital heart disease. Palliative surgery for this malformation became available in the UK in the late 1950s, and intracardiac repairs in the early 1960s. Without surgery, only 4% of children born with tetralogy of Fallot would be alive at 15 years. The intracardiac repair is generally successful in closing the ventricular septal defect but most patients are left with an element of pulmonary obstruction or incompetence. Timely reintervention with insertion of a 'pulmonary' valve in the right ventricular outflow tract may help to prevent or delay late complications. While the exact indications for reintervention are not yet entirely clear, recommendations for pulmonary valve replacement (PVR) may be made on the basis of physiological parameters even if a patient has few symptoms. For patients after Fallot repair, current guidelines stipulate that patients be periodically reviewed by specialists accredited in Adult Congenital Heart Disease (ACHD) in designated centres, though shared-care networks allow for delivery of some care by cardiologists with competencies in ACHD. However, ACHD provisions have been consolidated in their present form for less than 15 years and not all patients who left childhood services before this period have found their way to these services.
In this study, we aimed to use tetralogy of Fallot with its requirement for lifelong surveillance and possible adult reoperation to explore the implications of loss to follow-up of congenital heart disease. For a well-defined cohort, we aimed to establish the proportion of living patients not being followed to the specified standard and to estimate the impact of any shortfall in follow-up coverage on the incidence of secondary reoperations. We also wanted to conduct telephone interviews with patients believed to be lost to follow-up to understand their views on follow-up.
Abstract and Introduction
Abstract
Objective To evaluate the scale and clinical importance of loss to follow-up of past patients with serious congenital heart disease, using a common malformation as an example. To better understand the antecedents of loss to specialist follow-up and patients' attitudes to returning.
Design Cohort study using NHS number functionality. Content and thematic analysis of telephone interviews of subset contacted after loss to follow-up.
Patients, intervention and setting Longitudinal follow-up of complete consecutive list of all 1085 UK patients with repair of tetralogy of Fallot from single institution 1964–2009.
Main outcome measures Survival, freedom from late pulmonary valve replacement, loss to specialist follow-up, shortfall in late surgical revisions related to loss to follow-up. Patients' narrative about loss to follow-up.
Results 216 (24%) of patients known to be currently alive appear not to be registered with specialist clinics; some are seen in general cardiology clinics. Their median age is 32 years and median duration of loss to follow-up is 22 years; most had been lost before Adult Congenital services had been consolidated in their present form. 48% of the late deaths to date have occurred in patients not under specialist follow-up. None of those lost to specialist follow-up has had secondary pulmonary valve replacement while 188 patients under specialist care have. Patients lost to specialist follow-up who were contacted by telephone had no knowledge of its availability.
Conclusions Loss to specialist follow-up, typically originating many years ago, impacts patient management.
Introduction
Open heart surgery for congenital heart disease began in the 1960s in the UK. Its progressive success has meant that since around 2002, adult survivors of surgery outnumber children with congenital heart disease in the UK. The same document estimated that there are around 130 500 adults with 'moderate' or 'complex' congenital heart disease alive in England and Wales today; almost all are survivors of surgery they had as young children. For many complex congenital heart diseases, residual haemodynamic problems can result in late heart failure, reduced exercise capacity, arrhythmias or sudden death and there are risks associated with pregnancy and non-cardiac surgery. Most premature deaths associated with congenital heart disease are now occurring in adults rather than children.
Tetralogy of Fallot is the commonest 'cyanotic' congenital heart disease. Palliative surgery for this malformation became available in the UK in the late 1950s, and intracardiac repairs in the early 1960s. Without surgery, only 4% of children born with tetralogy of Fallot would be alive at 15 years. The intracardiac repair is generally successful in closing the ventricular septal defect but most patients are left with an element of pulmonary obstruction or incompetence. Timely reintervention with insertion of a 'pulmonary' valve in the right ventricular outflow tract may help to prevent or delay late complications. While the exact indications for reintervention are not yet entirely clear, recommendations for pulmonary valve replacement (PVR) may be made on the basis of physiological parameters even if a patient has few symptoms. For patients after Fallot repair, current guidelines stipulate that patients be periodically reviewed by specialists accredited in Adult Congenital Heart Disease (ACHD) in designated centres, though shared-care networks allow for delivery of some care by cardiologists with competencies in ACHD. However, ACHD provisions have been consolidated in their present form for less than 15 years and not all patients who left childhood services before this period have found their way to these services.
In this study, we aimed to use tetralogy of Fallot with its requirement for lifelong surveillance and possible adult reoperation to explore the implications of loss to follow-up of congenital heart disease. For a well-defined cohort, we aimed to establish the proportion of living patients not being followed to the specified standard and to estimate the impact of any shortfall in follow-up coverage on the incidence of secondary reoperations. We also wanted to conduct telephone interviews with patients believed to be lost to follow-up to understand their views on follow-up.
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