Congestive heart failure life expectancy: Prognosis and stages
In PAH, Main Pulmonary Artery Diameter Predicts Adverse Outcomes
Main pulmonary artery (MPA) diameter is a prognostic indicator in patients with pulmonary arterial hypertension (PAH) without congenital heart disease, according to study findings published in Chest.
Investigators characterized the size and growth of the MPA in patients with PAH without congenital heart disease. The researchers also assessed the prognostic value of MPA relative to existing risk stratification scores. A composite of all-cause mortality, lung transplantation, and hospitalization for right heart failure was the primary outcome.
The study included patients from 2 pulmonary hypertension referral centers in Sydney, Australia, who had pulmonary artery imaging at least once between January 2010 and December 2021. Participants were identified through a retrospective review of electronic medical records of patients with PAH without congenital heart disease who also did not have concurrent chronic thromboembolic PH or vasculitis involving pulmonary circulation.
PAH risk scores based on the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) 2.0, REVEAL Lite 2, and the European Society of Cardiology/European Respiratory Society (ESC/ERS) risk assessment were evaluated using baseline variables. REVEAL 2.0 was used when there were at least 7 parameters available; REVEAL Lite 2 was used when there were at least 3 parameters available, with at least 2 being World Health Organization (WHO) functional class, 6-minute walk distance (6MWD), or N-terminal pro b-type natriuretic peptide (NT-proBNP); and ESC/ERS was used when there were at least 3 parameters available.
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MPA diameter is a significant independent predictor of adverse clinical events in PAH patients without congenital heart disease. It may potentially be a novel prognostic marker in addition to the existing risk scores.
Overall, 351 patients were included in analysis, 70% with contrast-enhanced CTs, 27% with non-contrast enhanced CTs, and 3% with MRIs. Connective tissue disease was the most common PAH subgroup (49%), followed by idiopathic PAH (30%), and portopulmonary hypertension (5%). Echocardiography showed that right ventricular function was normal in 55% of participants, mildly impaired in 17%, moderately impaired in 12%, and severely impaired in 15%. Pericardial effusion was noted in 18% of participants.
Mean pulmonary artery pressure (MPAP) was 43 mmHg at baseline. Mean PA diameter was 35.3 mm at baseline and grew by 0.4 mm per year. The investigators noted 190 primary events across mean 4.0 years follow-up, with MPA diameter as a predictor (hazard ratio [HR], 1.06; 95% CI, 1.04-1.07; P <.001).
After multivariable adjustments for the 3 risk scores and their individual components, MPA diameter remained an independent outcome predictor. Independent of baseline MPA diameter, MPA growth rate predicted the primary endpoint with every 1 mm per year presenting a 79% increased hazard (HR, 1.79; 95% CI, 1.52-2.11; P <.001).
The risk for the primary endpoint at 1 year was similar for MPA alone (area under the receiver-operator characteristic curve [AUC], 0.72) vs the 3 risk scores (AUCs, 0.72-0.75). Risk reclassification (primarily risk downgrading) in nearly a quarter of patients (23%) stemmed from MPA used in addition to REVEAL 2.0.
Study limitations include the retrospective design and the lack of data on causes of mortality.
"MPA diameter is a significant independent predictor of adverse clinical events in PAH patients without congenital heart disease," the investigators concluded. "It may potentially be a novel prognostic marker in addition to the existing risk scores."
How A Durban Hospital Is Leading The Fight Against Congenital Kidney Diseases
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Division Of Pediatric Nephrology
The Division of Pediatric Nephrology in the Department of Pediatrics of Saint Louis University provides comprehensive care for infants, children and adolescents with various forms of kidney or urologic disorders, both acute and chronic.
Members of the division are involved in the teaching of medical students, residents, community physicians and families and participate in clinical research studies. The division is housed at SSM Health Cardinal Glennon Children's Hospital. Our multidisciplinary team includes physicians, renal nurses, a renal dietitian, a renal social worker and a play therapist.
Medical EducationOur educational programs address training of pediatric residents, family practice residents, and medical students, as well as community programs for families of children with renal disease and continuing medical education (CME) activities for community physicians.
Transplant and Clinical ServicesOur division began a renal transplant program in 1983. Since that time, approximately 150 pediatric renal transplants have been performed. The division currently follows approximately 45 renal transplant recipients. Deceased donor, living related, and living unrelated renal transplants are currently performed. We also participate in kidney paired donation (KPD), the donor exchange program for living donors.
The division provides primary management of renal transplant recipients along with members of the transplant surgery service. We also provide fetal consultation through the Fetal Care Institute.
HypertensionOur division has a special interest in hypertension, with a weekly clinic for patients with primary hypertension, and an active Ambulatory BP monitoring program.
The division has a formal transition program for dialysis and transplant patients to aid in the successful transition to adult care.
Clinical Services OverviewOur physician faculty members treat a variety of acute and chronic kidney disorders, including congenital and hereditary disorders involving the kidney and urinary tract, urinary tract infections, kidney stones, hypertension, acute and chronic glomerular and tubular disorders and renal diseases that occur secondary to a variety of systemic diseases, such as systemic lupus erythematosus, diabetes mellitus, and vasculitis.
Learn About Services Offered and Our Clinical Practice
ResearchThe division participates in the North American Pediatric Renal Transplant Cooperative Study, a registry for Transplant, Dialysis, and Chronic Renal Insufficiency patients. We also participate in multi-center studies funded by the NIH and the Midwest Pediatric Nephrology Consortium Study Group, as well as in pharmaceutical studies.
Current Research Multi-Center NIH studies:
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