Systolic Blood Pressure, Cardiac Index and Eisenmenger Syndrome are Predictors of Mortality in Pulmonary Arterial Hypertension-associated with Congenital Heart Disease: An Analysis from the COHARD-PH registry
Abstract
Background: Pulmonary arterial hypertension (PAH) is a complication of left-to-right intracardiac shunt congenital heart disease (LtR-shunt CHD). There are several known predictors of mortality in PAH patients, however predictors of mortality in LtR-shunt CHD-associated PAH need to be validated.
Objectives: We aimed to investigate the predictors of mortality among adult LtR-shunt CHD-associated PAH patients.
Methods: This research was a retrospective cohort study that included adult patients with LtR-shunt CHD-associated PAH retrieved from the COHARD-PH registry. Several baseline variables were selected as potential predictors of mortality, namely (1) clinical data: WHO-functional class, SaO2, 6-min walking distance, systolic blood pressure, and Eisenmenger syndrome; (2) laboratory data: hemoglobin and NT-pro BNP levels; (3) echocardiography data: pericardial effusion, defect size, and TAPSE; and (4) hemodynamic data: right atrial pressure, cardiac output and index, SvO2, and flow ratio. The mortality outcome was assessed from the cohort registry.
Results: A total of 124 subjects with LtR-shunt CHD-associated PAH were included. Sixteen subjects (12.9%) died during the follow-up period. The baseline variables which showed significant association with mortality were lower systolic blood pressure, Eisenmenger syndrome, higher NT-pro BNP level, and lower cardiac output. The multivariable analysis showed that systolic blood pressure <100 mmHg (OR 10.99; 95% CI 2.54-47.51, p=0.001), cardiac index <2.5 L/min/m2 (OR 8.13; 95% CI: 1.59-42.28, p=0.011) and Eisenmenger syndrome (OR 3.87; 95%CI: 1.06-14.07) were the independent predictors for mortality.
Conclusions: The systolic blood pressure <100 mmHg, cardiac index <2.5 L/min/m2, and Eisenmenger syndrome were independent predictors of mortality among adults with LtR-shunt CHD-associated PAH.
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References
2. McLaughlin VV, Archer SL, Badesch DB, et al. 2009. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol 2009;53:1573-619.
3. Galie N, Hoeper MM, Humbert M, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2009;30: 2493– 2537.
4. Montani D, Gunther S, Dorfmuller P, et al. Pulmonary arterial hypertension. Orphanet J of Rare Diseas 2013;8:97.
5. Dinarti LK, Hartopo AB, Kusuma AD, Satwiko MG, Hadwiono MR, Pradana AD, Anggrahini DW. The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry: a descriptive study from single-center hospital registry of adult congenital heart disease and pulmonary hypertension in Indonesia. BMC Cardiovasc Disord. 2020 Apr 7;20(1):163.
6. Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, Williams PG, Souza R. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019 Jan 24;53(1):1801913.
7. Li X, Sun X, Huang Y, et al. Simplified risk stratification for pulmonary arterial hypertension associated with connective tissue disease. Clin Rheumatol 2019;38(12):3619-3626.
8. Oliver JM, Gallego P, Gonzales AE, et al. Risk factor for excess mortality in adults with congenital heart diseases. Eur Heart J 2017 [E-pub ahead of print]; doi: 10.1093/eurheartj/ehw590.
9. Vijarnsorn C, Durongpisitkul K, Chungsomprasong P, et al. Contemporary survival of patients with pulmonary arterial hypertension and congenital systemic to pulmonary shunts. PLoS ONE 2018 [E-pub ahead of print]; doi: 10.1371/journal.pone.0195092.
10. Nickel N, Golpon H, Greer M, et al. The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 2012;39:589–596.
11. Campo A, Mathai SC, Pevec JL, et al. Outcomes of hospitalisation for right heart failure in pulmonary arterial hypertension. Eur Respir J 2011 [E-pub ahead of print]; doi: 10.1183/09031936.00148310.
12. Ciarka A, Doan V, Velez-Roa S, et al. Prognostic significance of sympathetic nervous system activation in pulmonary arterial hypertension. Am J Respir Crit Care Med 2010; 181: 1269–1275.
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