Predictors of adverse clinical outcome after hospitalisation for decompensated heart failure in ACHD patients
Authors: Krishnathasan K , Constantine A , Kempny A , Rafiq I , Gatzoulis M A , Heng E L , Montanaro C , Babu-Narayan S V , Li W , Dimopoulos K 
Published: October 2021

Background

As the ACHD population ages, an increasing number of patients are being admitted for heart failure. Management is complicated by the limited evidence on pharmacological and other therapies and lack of risk stratification tools. Improved surgical and medical treatment options in this patient cohort has improved life expectancy.

Purpose

Assess the mortality and rate of rehospitalisation in ACHD patients following admission with decompensated HF and identify clinical predictors of outcome.

Methods

All ACHD patients admitted for HF requiring IV diuresis from February 2016 to December 2020 in a single tertiary centre were included. Cox analysis was used to identify predictors of death after the index admission.

Results

91 patients were included: 44.0% female, 45.8±14.2 years. The most frequent underlying diagnoses were: 15.4% with primary valve or left ventricular outflow tract disease, 12.1% with transposition of the great arteries post-atrial switch, 9.9% with tetralogy of Fallot and 30.8% had a systemic right ventricle. Most (56.7%) patients had CHD of “great” complexity according to the Bethesda classification, whereas no patients had “simple” CHD. Cyanosis was present in 26.4%.

Heart failure was predominantly related to systemic ventricular dysfunction in 35.2%, subpulmonary ventricular dysfunction in 25.3%, biventricular dysfunction in 24.2% and univentricular dysfunction in 15.4%. Pulmonary hypertension was present in most (58.2%) patients, only half (46.0%) of these had pre-capillary pulmonary hypertension.

On admission, approximately half of patients were on standard heart failure therapy: 48.4% ACE-inhibitors or angiotensin receptor blockers, 54.9% beta-blockers, 54.9% mineralocorticoid receptor antagonists and 2% on sacubitril/valsartan.

In-hospital mortality was only 3.3% in this high-risk population. During a median follow-up of 22.1 [0.2–58.4] months, 37.5% patients were rehospitalised for HF and 37.5% patients died (22.7% mortality at 1 year). On univariable Cox analysis, the following parameters were predictors of mortality: pulmonary hypertension, cyanosis, unoperated or palliated CHD, chronic kidney disease (CKD), hyponatraemia, mineralocorticoid receptor antagonist use on admission, admission BNP&gt;350ng/L and a maximum daily inpatient loop diuretic requirement &gt;160mg (furosemide equivalents). On multivariable Cox analysis, pulmonary hypertension, cyanosis, hyponatraemia and CKD were independent predictors of mortality; patients with ≥2 of these risk factors had an 8-fold higher mortality (figure).

Conclusion

ACHD patients hospitalised for decompensated HF have a poor outlook with a quarter of patients dying within a median &lt;2 years from discharge. We present clinical parameters that can identify patients at high risk of an adverse outcome who should be targeted for aggressive monitoring and advanced HF therapies, including transplantation.

Funding Acknowledgement

Type of funding sources: None. Figure 1