2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines | Circulation
PH Risk Grows Over Time With COA, Or Narrowing Of Aorta: Study
People with coarctation of aorta, or COA — a condition marked by narrowing where the body's largest blood vessel connects to the heart — are at high risk of developing pulmonary hypertension (PH), and this risk increases as time goes on, a study showed.
The data also showed that PH progression is associated with heightened mortality risk among people with COA, emphasizing a need for better treatment strategies in this patient population, according to the researchers.
"These findings underscore the clinical importance of [PH] in COA and [support] the need for new strategies for prevention and treatment of [heart conditions that increase PH risk]," the researchers wrote, noting that new therapies "should, in turn, slow the pace of [PH] progression in this [patient] population."
The study, "Temporal changes in prevalence and severity of pulmonary hypertension, and relationship to outcomes in coarctation of aorta," was published in the journal IJC Heart & Vasculature.
COA, marked by narrowing of aorta, already tied to higher PH riskCOA, a type of birth defect, occurs when the aorta — the large blood vessel that carries oxygen-rich blood from the heart out toward the rest of the body — is abnormally pinched or narrowed.
Previous studies have shown that people with COA are at increased risk of developing PH, which is marked by abnormally high pressure in pulmonary arteries, or the vessels that carry blood through the lungs. PH makes the heart's right ventricle work harder to pump blood into the lungs.
However, whether PH risk in COA patients changes over time remained unclear.
To learn more, a team of researchers in the U.S. Retrospectively analyzed data from 392 people with COA who were treated at Mayo Clinic from January 2003 to December 2017. Each of the patients underwent two or more echocardiograms — a technique used to image the heart and that can be used to assess PH — five or more years apart. The patients' median age at the first echocardiogram was 35 years. Among them, 61% were men.
At the initial assessment, nearly 1 in 5 patients (19%) met criteria for PH, meaning they had right ventricular systolic pressure — known as RVSP, this measure estimates the pressure in pulmonary arteries — higher than 40 mmHg.
At the second assessment years later, more than 1 in 4 patients (27%) met PH criteria, implying that the risk of PH increases with time in COA patients.
RVSP values, which are commonly used as a proxy of PH severity, also increased significantly over time (by a mean of 5.6 mmHg), implying that PH may get more severe as time goes on in people with COA.
Statistical analyses showed that older age was significantly associated with an increased risk of PH in people with COA. Other heart conditions, such as atrial fibrillation, or an irregular heartbeat that begins in the heart's upper chambers, as well as signs of left heart problems, also were significantly tied to a higher PH risk.
During a median follow-up of nearly 5.5 years, 50 patients (13%) died.
PH progression is clinically relevant because of its association with mortality in patients with COA.
Statistical analyses adjusted for potential influencing factors showed that for every 5 mmHg increase in RVSP between the first and second echocardiogram — reflecting increased pressure in pulmonary arteries — there was a 19% increase in the risk of death.
Also, a significantly lower proportion of patients experiencing greater PH progression, or highest RVSP increase, were estimated to be alive after 10 years relative to those with less PH progression, or lowest RVSP increase (82% vs. 95%).
These findings highlighted that "PH progression was associated with all-cause mortality, independent of [PH] severity at [first] echocardiogram," the researchers wrote.
This "demonstrates that PH progression is clinically relevant because of its association with mortality in patients with COA," and underscores the importance of strategies to prevent PH in people with COA, the team concluded.
Pulmonary Hypertension Subtypes Show Distinct PA Flow Hemodynamics
Investigators used 4D flow cardiovascular magnetic resonance imaging to search for differences between pulmonary artery (PA) remodeling in pulmonary arterial hypertension and other types of pulmonary hypertension.
Advanced imaging technology can help clinicians better understand pulmonary artery (PA) remodeling and its relation to different types of pulmonary hypertension (PH), according to a new report. The study was published in the journal Pulmonary Circulation.1
The analysis found that patients with pulmonary arterial hypertension (PAH) had distinct PA flow characteristics compared with those with heart failure with preserved ejection fraction and pulmonary hypertension (PH-HFpEF). These findings suggest the PA remodeling process differs by PH etiology, the authors explained.
Patients with PAH had distinct PA flow characteristics compared with those with PH-HFpEF.Image credit: Thirawat - stock.Adobe.Com
Corresponding author James D. Thomas, MD, of Northwestern University, and colleagues, noted that PH subtypes—including PAH (Group 1) and PH due to left heart disease (Group 2 PH)—are definable by hemodynamic indices.
Group 2 PH is the most common type of PH, and Thomas and colleagues noted that many patients with Group 2 PH meet the criteria for PH-HFpEF, which itself can be subdivided into isolated postcapillary PH and combined pre- and postcapillary PH.
Yet differentiating between the different subtypes can be difficult and invasive. "While these subtypes are distinguishable in advanced disease, early differentiation often requires precise catheterization," they wrote.
One possible solution the authors presented is the use of 4D flow cardiovascular magnetic resonance imaging (4D-flow CMR). It allows for comprehensive assessment of blood flow velocities in the PA, thereby helping clinicians track the progression of PH. The imaging method also makes it possible to track physiological parameters, helping clinicians better characterize vascular remodeling.
Recent research has highlighted the role of advanced MRI to track hemodynamic changes in pulmonary circulation,2 Thomas and colleagues noted. Yet, while studies have examined its ability to track right ventricle (RV) and PA flow, little attention has been devoted toward identifying potential associations between PA flow features and PH etiologies, they said.
The investigators identified 13 patients with PAH and 15 patients with PH-HFpEF and performed echocardiography, 4D-flow CMR, and right heart catheterization on each. They then compared several parameters, including right ventricular outflow tract (RVOT) flow and main pulmonary artery (MPA) hemodynamics, including peak velocity and mean and maximum wall shear stress (WSS).
They found that mean PA pressure and pulmonary vascular resistance (PVR) were higher in patients with PAH. Eight of the 13 patients with PAH also had RVOT systolic notching, compared with 0 patients in the PH-HFpEF group. RVOT acceleration time was shorter in the PAH group, and people with PAH had lower MPA peak velocity, mean WSS, and maximal WSS. Thomas and colleagues also found PVR was negatively correlated with MPA mean WSS.
"These findings align with RHC and echocardiography results, showing higher PVR, more notching patterns, and altered RVOT flow in PAH patients," Thomas and colleagues wrote. "These changes are well-known indicators of pulmonary vascular load and RV function."
The investigators said their findings confirm that PA remodeling in PAH and PH-HFpEF are significantly different due to differences in flow characteristics. They added, though, that some patients with advanced PH-HFpEF and significant PA remodeling may benefit from drugs initially developed to treat PAH.
Thomas and colleagues noted their results should be interpreted with caution due to the study's small sample size. They also explained that their analysis was limited to PA flow, and thus did not capture changes at the cellular level that might further elucidate overall PA remodeling.
Still, they said these early data suggest MPA WSS may serve as an important novel indicator of PA remodeling in patients with PH.
References:
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