Evidence of Advanced Pulmonary Vascular Remodeling in Obstructive Hypertrophic Cardiomyopathy With Pulmonary Hypertension

Maron, Bradley A.; Kleiner, David E.; Arons, Elena; Wertheim, Bradley M.; Sharma, Nirmal; Haley, Kathleen J.; Samokhin, Andriy O.; Rowin, Ethan J.; Maron, Martin S.; Rosing, Douglas R.; Maron, Barry J.

doi:10.1016/j.chest.2022.09.040

Cited by 9 publications

(3 citation statements)

References 22 publications

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“…This finding was independent of the PAWP, with increased mortality identified in patients with both pre-and post-capillary PH. Pathologic data again confirmed the findings of adverse vascular remodeling in patients with lower PVR beginning approximately at 1.8-2 WU [35].…”

Section: Pulmonary Vascular Resistancesupporting

confidence: 72%

Updated Clinical Classification and Hemodynamic Definitions of Pulmonary Hypertension and Its Clinical Implications

Kularatne,

Gerges,

Jevnikar

et al. 2024

JCDD

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Pulmonary hypertension (PH) refers to a pathologic elevation of the mean pulmonary artery pressure (mPAP) and is associated with increased morbidity and mortality in a wide range of medical conditions. These conditions are classified according to similarities in pathophysiology and management in addition to their invasive hemodynamic profiles. The 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension present the newest clinical classification system and includes significant updates to the hemodynamic definitions. Pulmonary hypertension is now hemodynamically defined as an mPAP > 20 mmHg, reduced from the previous threshold of ≥25 mmHg, due to important insights from both normative and prognostic data. Pulmonary vascular resistance has been extended into the definition of pre-capillary pulmonary hypertension, with an updated threshold of >2 Wood Units (WU), to help differentiate pulmonary vascular disease from other causes of increased mPAP. Exercise pulmonary hypertension has been reintroduced into the hemodynamic definitions and is defined by an mPAP/cardiac output slope of >3 mmHg/L/min between rest and exercise. While these new hemodynamic thresholds will have a significant impact on the diagnosis of pulmonary hypertension, no evidence-based treatments are available for patients with mPAP between 21–24 mmHg and/or PVR between 2–3 WU or with exercise PH. This review highlights the evidence underlying these major changes and their implications on the diagnosis and management of patients with pulmonary hypertension.

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Section: Pulmonary Vascular Resistancesupporting

confidence: 72%

Updated Clinical Classification and Hemodynamic Definitions of Pulmonary Hypertension and Its Clinical Implications

Kularatne,

Gerges,

Jevnikar

et al. 2024

JCDD

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show abstract

“…as part of heart transplant evaluation), and at times use of inhaled nitric oxide if there is prominent precapillary component. Chronic elevation of left heart filling pressure can lead to remodeling of the pulmonary arterioles and venules [15,16], contributing to further increase in pulmonary vascular resistance and less reversibility. In this article, we review recent advances in the understanding of optimal approach to the diagnosis and management of pulmonary hypertension accompanying left heart disease.…”

Section: Introductionmentioning

confidence: 99%

Group 2 pulmonary hypertension: from diagnosis to treatment

Frantz

2023

Current Opinion in Pulmonary Medicine

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Purpose of review Pulmonary hypertension associated with left heart disease (PHLHD) is common and associated with adverse prognosis. Proper diagnosis is critical to avoid inappropriate treatment. Practical approaches to noninvasive diagnosis are available, though invasive hemodynamics including volume loading or exercise are often necessary for definitive diagnosis. Treatment strategies and research in the field is rapidly evolving. Recent findings Combined pre and post capillary pulmonary hypertension is associated with remodeling of the pulmonary vasculature that is proportional to the severity of the pulmonary hypertension. Even quite mild elevation of pulmonary vascular resistance (PVR), (greater than 2.0 Wood units (WU)) is associated with adverse outcome. Guideline-directed medical therapy (GDMT) for left heart failure has rapidly evolved, including approval of SGLT2 inhibitors for treatment of heart failure with preserved ejection fraction (HFpEF). However, full implementation of GDMT is lagging. Utilization of implanted pulmonary artery pressure monitoring can facilitate patient management in selected patient phenotypes and has been utilized to demonstrate improvement in pulmonary pressures with emerging therapies in HFpEF including SGLT2 inhibitors and sacubitril/valsartan. A variety of shunt devices to create a left-to-right shunt in order to decompress the left heart are available or undergoing clinical trials. There is concern that there could be adverse response to such devices in patients who have even mild elevation of pulmonary vascular resistance. Sleep disordered breathing is common in PHLHD and should be aggressively sought out and treated. Summary Even mild elevation in pulmonary vascular resistance is associated with adverse outcome. A systematic approach to diagnosis of PHLHD is essential. Guideline-directed medical therapy for PHLHD prioritizes optimal management of the left heart disease. Pulmonary artery pressure sensors are useful in selected patients.

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“… 18 , 19 In well‐phenotyped populations at risk for PAH 20 or PH from primary cardiac or lung disease, 21 mPAP >20 mm Hg and PVR >2.0 WU are associated with impaired exercise tolerance or other measures of PH disease burden. In patients with mPAP >20 mm Hg and PVR >2.0 WU, pathogenic changes are also observed to the pulmonary vasculature, 22 whereas pathogenic changes to RV size and function 23 , 24 are often indicative of increased RV afterload, 25 itself an independent risk factor for right heart failure progression. 4 These collective observations indicate that mild PH is pathogenic per se, and associated adverse outcome is unlikely attributable solely to comorbidities.…”

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confidence: 99%

Revised Definition of Pulmonary Hypertension and Approach to Management: A Clinical Primer

Maron

2023

JAHA

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The definition of pulmonary hypertension (PH) has changed recently based, in part, on contemporary outcome data and to focus on early disease detection. Now, PH includes patients with mean pulmonary artery pressure >20 mm Hg measured by right heart catheterization. In contrast to the classical era, pulmonary vascular resistance >2.0 Wood units is also used for diagnosis and prognostication. These lowered thresholds aim to identify patients early in the disease course, which is important because delay to diagnosis of PH is common and linked to elevated morbidity and shortened lifespan. This clinical primer highlights key changes in diagnosis and approach to PH management, focusing on concepts that are likely to be encountered frequently in general practice. Specifically, this includes hemodynamic assessment of at‐risk patients, pharmacotherapeutic management of pulmonary arterial hypertension, approach to PH in patients with heart failure with preserved ejection fraction, and newly established indications for early referral to PH centers to prompt comanagement of patients with pulmonary vascular disease experts.

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Evidence of Advanced Pulmonary Vascular Remodeling in Obstructive Hypertrophic Cardiomyopathy With Pulmonary Hypertension

Cited by 9 publications

References 22 publications

Updated Clinical Classification and Hemodynamic Definitions of Pulmonary Hypertension and Its Clinical Implications

Updated Clinical Classification and Hemodynamic Definitions of Pulmonary Hypertension and Its Clinical Implications

Group 2 pulmonary hypertension: from diagnosis to treatment

Revised Definition of Pulmonary Hypertension and Approach to Management: A Clinical Primer

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