ACHD patients need referral to specialized care!

Background:

With a worldwide prevalence of 9 per 1000 newborns, congenital heart diseases (CHD) are the most common hereditary illnesses. Survival has increased up to 90% in the last decades¹. In Europe, it is estimated that there are more than 4 million people living with CHD, of which around 2.6 million are adults². Unfortunately, cure is seldom achieved in the treatment of CHD, while residua and sequelae are often encountered. This leads to a new patient group, adults with congenital heart disease (ACHD). While during childhood patients with CHD are commonly treated by pediatric cardiologists, the care model for ACHD patients is often less well established. Consequently, a large proportion of ACHD patients are not under regular follow-up by a cardiologist. However, the extent and the impact of this loss to follow-up were unknown. 

Study summary:

In this article, Diller et al. analysed 24,139 ACHD from the database of one the largest health insurance companies in Germany. Median age was 43 years and almost 60% were female. Most common CHD were simple heart defects in 69.4%, followed by moderate defects in 22.7% and complex defects in 7.9%. The authors reported that 49.7% of ACHD were under cardiology follow-up, while 49.3% were under the care of primary care physicians (PCPs) and 1.1% had no medical controls in a 3-year period. Patients being followed by cardiologists were significantly older (46 vs. 40 years old, p<0.0001) and had more complex cardiac defects (simple: 65% vs. 73.7%; moderate: 25% vs. 20.5%; complex: 10% vs. 5.8%). They also had significantly more cardiac and extra-cardiac comorbidities. The authors analysed the impact of non-specialized medical care for ACHD in terms of death and major adverse events (cardiac or neurology emergency admission, neurological complications, acute myocardial infarction, survived resuscitation and heart transplantation or implantation of an assist device). Overall, specialised cardiologic care was associated with a lower risk of death [hazard ratio (HR) 0.81, 95% CI 0.67–0.98, P = 0.03] and major adverse events (HR 0.85; 95% CI 0.78–0.92, P < 0.001), compared to primary care. Concerning subgroups, cardiology follow-up had a positive impact on the survival of females (HR 0.65; P = 0.005), elderly patients (≥45 years; HR 0.76; P = 0.01) as well as those with moderate or complex CHD (HR 0.73; P = 0.048). For the composite endpoint of major adverse events, cardiology care was beneficial in females (HR 0.88; P = 0.046) as well as males (HR 0.82; P = 0.001), patients ≥45 years (HR 0.77; P < 0.001) and in simple (HR 0.85; P = 0.003), moderate and complex lesions (HR 0.81; P = 0.007).

Discussion:

It is worrisome, that only half of ACHD patients are under follow-up by a cardiologist. The other half were under follow-up only by a PCP, which was associated with significant higher morbidity and mortality. Interestingly, patients who were totally lost to medical follow-up were rare. The problem seems to be that the referral from the PCP to the cardiologist did not occur. Additionally, patients under follow-up by a general cardiologist were also often referred to an ACHD specialist. One could assume that this would lead to additional benefits regarding the outcome of the ACHD patients. But, the effect of an additional treatment of an ACHD specialist on morbidity and mortality of ACHD patients could not be analysed from the current data.

In order to improve access to cardiologist and hence, ACHD care, several measures are necessary:

• The education of our colleagues in primary care, who do an outstanding job, that a referral to a cardiologist does improve the outcome of our ACHD patients.
• Considering, that many ACHD patients receive a cardiology follow-up by general cardiologist without the involvement of ACHD specialists, a better understanding of the specific problems of ACHD patients by general cardiologists should be encouraged.
• We need to provide our patients with a better understanding of their CHD and the need of a cardiology follow-up.

Conclusions:

In conclusion, only one-half of ACHD patients in a large population-based study were under cardiology follow-up which significantly improved morbidity and mortality.