Maurer SJ, Stöckemann K, Pujol C, Hörer J, Ewert P, Tutarel O.
J Clin Med. 2020 Dec 17;9(12):4071. doi: 10.3390/jcm9124071.
PMID: 33348628 Free PMC article.
Take Home Points:
- In patients over age 40, with congenital heart disease and pulmonary hypertension, a quarter died during the follow up period of 4 years.
- Creatinine and NT pro-BNP were the only factors significantly associated with the primary end-point.
Commentary by Dr. Helen Parry (Leeds, UK), section editor of ACHD Journal Watch: There is currently relatively little research looking at the natural history and prognostic factors in pulmonary arterial hypertension in older adults with congenital heart disease. This study focuses on this cohort of patients over 40 years of age.
All patients at the German Heart Centre in Munich with a diagnosis of congenital heart disease and pulmonary arterial hypertension (PAH) above the age of 40 were included. Pulmonary arterial hypertension was diagnosed based on echo, MRI and/ or catheter studies. The patients were sub-categorised as:
- Shunt lesions
- Complex congenital heart disease
- Segmental PAH, i.e. patients with major aorto-pulmonary collaterals (MAPCAs)
The primary end-point was all-cause mortality. Variables examined included:
- NYHA score
- Echo assessment of left and right ventricular function
- Presence of arrhythmia
- NTpro-BNP
- Creatinine
- Arrhythmia
- Presence of Down’s syndrome
- Use of advanced PAH therapies.
Univariate analysis was used to assess whether these variable were associated with death (Students’ t test for continuous variables and Chi squared test for categorical variables). Multivariate analysis by Cox proportional hazard regression modelling was used to assess their relative significance. P-value <0.05 was classed as significant.
Results:
Sixty-five patients were included; 70.8% had a shunt lesion, 18.5% complex CHD and 10.8% segmental PAH. Median follow-up was 4.2 years. Atrial arrhythmia occurred in 23.1% and ventricular arrhythmia in 9.2%. Sixteen patients (24.6%) died during the follow up period.
Univariate analysis showed an association between both creatinine and NT-pro-BNP and all-cause mortality:
|
Variable |
Univariate HR (95%CI) |
Univariate p-value |
Multivariate HR (95% CI) |
Multivariate p-value |
|
Creatinine |
12.76 (2.05–79.32) |
0.0063 |
16.28 (2.23–118.69 |
0.0059 |
|
NT pro-BNP |
3.54 (1.08–11.64) |
0.037 |
4.08 (1.16–14.41) |
0.0289 |
Conclusions:
A quarter of the patients above 40 years of age with combined congenital heart disease and PAH died during the follow up period. Creatinine and NT pro-BNP were the only factors significantly associated with the primary end-point.
Positive aspects of the study:
- There is very little in the literature about this group of patients so this study was a useful contribution.
- The identified predictors of poor prognosis, namely, raised creatinine and raised NT pro BNP can be relatively easily measured routinely in most centres.
- May contribute towards counselling regarding in certain patients.
Negative aspects of the study:
- Small sample size.
- The majority of patients had shunt lesions so it is difficult to extrapolate the results to the other groups as they were relatively under-represented, could survival be worse or better in these groups? The sample sizes were too small to draw confident conclusions.
- The study does not really help to guide treatment in these patients. The questions are posed: should we try to improve creatinine and NT pro-BNP in these patients and will this improve their prognosis? NT pro-BNP is generally improved by increasing heart failure therapies, many of which are nephrotoxic so likely to increase creatinine.
- Follow up period varied significantly from 1.2 years to 7 years.
