Pulmonary hypertension is associated with worse survival in hypertrophic cardiomyopathy

Ong, Kevin; Geske, Jeffrey B.; Hebl, Virginia B.; Nishimura, Rick A.; Schaff, Hartzell V.; Ackerman, Michael J.; Klarich, Kyle W.; Siontis, Konstantinos C.; Coutinho, Thais; Dearani, Joseph A.; Ommen, Steve R.; Gersh, Bernard J.

doi:10.1093/ehjci/jew024

Cited by 70 publications

(48 citation statements)

References 34 publications

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“…6 A more recent study reported that pulmonary hypertension, as measured with Doppler echocardiography, predicted HCM-related death. 21 The present study reports, for the first time, that elevated RAP is associated with increased risk of death in HCM patients. The association remained significant after adjusting for possible confounding factors.…”

Section: Discussionsupporting

confidence: 49%

Clinical Correlates and Prognostic Value of Elevated Right Atrial Pressure in Patients With Hypertrophic Cardiomyopathy

McCullough

Fifer

Mohajer

et al. 2018

Circ J

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Background:The clinical characteristics associated with elevated right atrial pressure (RAP) in hypertrophic cardiomyopathy (HCM) are unknown. Few data exist as to whether elevated RAP has prognostic implications in patients with HCM. This study investigated the clinical correlates and prognostic value of elevated RAP in HCM. Methods and Results:This retrospective cohort study was performed on 180 patients with HCM who underwent right heart catheterization between 1997 and 2014. Elevated RAP was defined as >8 mmHg. Baseline characteristics, mean pulmonary artery pressure, and mean pulmonary capillary wedge pressure (PCWP) were assessed for association with elevated RAP. The predictive value of elevated RAP for all-cause mortality and the development of atrial fibrillation (AF), ventricular tachycardia/fibrillation (VT/VF), and stroke was evaluated. Elevated RAP was associated with higher New York Heart Association class, dyspnea on exertion, orthopnea, edema, jugular venous distention, larger left atrial size, right ventricular hypertrophy, higher pulmonary artery pressure, and higher PCWP. RAP independently predicted all-cause mortality (adjusted hazard ratio [aHR] 2.18 per 5-mmHg increase, 95% confidence interval [CI] 1.05-4.50, P=0.04) and incident AF (aHR 1.85 per 5-mmHg increase, 95% CI 1.20-2.85, P=0.005). Elevated RAP did not predict VT/VF (P=0.36) or stroke (P=0.28). Conclusions:Elevated RAP in patients with HCM is associated with left-sided heart failure and is an independent predictor of all-cause mortality and new-onset AF.

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Section: Discussionsupporting

confidence: 49%

Clinical Correlates and Prognostic Value of Elevated Right Atrial Pressure in Patients With Hypertrophic Cardiomyopathy

McCullough

Fifer

Mohajer

et al. 2018

Circ J

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“…4 On the other hand, we have the unique opportunity to report here the role of pulmonary hypertension in advanced heart failure with cardiopulmonary hemodynamics measured directly at cardiac catheterization. 13 The study group comprises a consecutive cohort of patients undergoing surgical septal myectomy (or alcohol septal ablation) to relieve outflow tract obstruction and mitigate heart failure symptoms, at a single HCM tertiary referral institution.…”

Section: Discussionmentioning

confidence: 99%

“…Prior reports characterizing pulmonary artery pressure dynamics in obstructive HCM patients have relied largely on estimates derived from Doppler echocardiography studies, and attributed adverse consequences (i.e., all-cause mortality) to pulmonary hypertension. 3,4 Therefore, in the present analysis we have re-visited clinical issues surrounding pulmonary hypertension in a unique cohort of HCM patients using hemodynamic data obtained at cardiac catheterization.…”

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confidence: 99%

Mechanism of Progressive Heart Failure and Significance of Pulmonary Hypertension in Obstructive Hypertrophic Cardiomyopathy

Covella

Rowin

Hill

et al. 2017

Circ: Heart Failure

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Background There are limited data regarding the prevalence, pathophysiology and management implications of pulmonary hypertension in patients with obstructive hypertrophic cardiomyopathy (HCM) and advanced heart failure. Methods and Results To assess the clinical significance of measured cardiopulmonary hemodynamics in HCM patients with heart failure, we retrospectively assessed right heart catheterization data in 162 consecutive patients with outflow tract gradients (median [interquartile range]) (90 [70–110] mmHg), 59±11 years old, and 49% male, predominately NYHA Class III/IV status. Pulmonary hypertension (mean pulmonary artery pressure [mPAP] ≥25 mmHg) was present in 82 patients (51%), including 29 (18%) regarded as moderate-severe (mPAP ≥35 mmHg) and 28 (34%) that also had increased pulmonary vascular resistance (PVR) >3.0 WU. The pulmonary artery wedge pressure (PAWP) was ≤15 mmHg in 54%, indicating that left atrial hypertension was absent in a majority of patients. Notably, 9 patients (11%) met hemodynamic criteria for pre-capillary pulmonary hypertension (mPAP ≥25 mmHg, PVR >3.0 WU, PAWP ≤15 mmHg). Over a median follow-up of 327 [90–743] days after surgical myectomy (or alcohol septal ablation), 92% and 95% of patients with or without preoperative pulmonary hypertension, respectively, were asymptomatic or mildly symptomatic. One postoperative death occurred in a 59-year old woman with acute respiratory failure and mPAP=65 mmHg. Conclusions Pulmonary hypertension was common in obstructive HCM patients with advanced heart failure. While possibly a contributor to pre-operative heart failure, pulmonary hypertension did not significantly influence clinical and surgical outcome. Notably, a novel patient subgroup was identified with resting invasive hemodynamics consistent with pulmonary vascular disease.

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“…Chest CT was not done as the radiation dose of the high resolutional CT which is needed for the proper evaluation of parenchymal lung disease expose the person to high radiation dose approaching 100 times and should be reserved for selective and proper diagnosis of cases like interstial fibrosis or patients with immunedeficiency [26]. In our study we excluded Hypertrophic obstructive cardiomyopathy (HOCM) cases as there is more evidences of associated pulmonary hypertension with HOCM which was found to be associated with increased mortality in HOCM patients [27]. Abnormal thyroid function whether thyroiditis or hyperthyroidism also excluded from the study as there is high association with the pulmonary hypertension which is reversed when the thyroid function returns to the normal [28].…”

Section: Discussionmentioning

confidence: 99%

Study the Pulmonary Hypertension among Heavy Smokers Young Adult Males before the Clinical Evidences of Chronic Lung Disease

Naser¹,

Hadi²,

Ibrahim³

et al. 2017

J Clin Exp Cardiolog

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Pulmonary hypertension is associated with worse survival in hypertrophic cardiomyopathy

Abstract: Over one-third of adults evaluated in our referral HCM clinic demonstrated concomitant PH, and this was associated with increased mortality except in those with obstructive HCM who underwent SRT.

Cited by 70 publications

References 34 publications

Clinical Correlates and Prognostic Value of Elevated Right Atrial Pressure in Patients With Hypertrophic Cardiomyopathy

Clinical Correlates and Prognostic Value of Elevated Right Atrial Pressure in Patients With Hypertrophic Cardiomyopathy

Mechanism of Progressive Heart Failure and Significance of Pulmonary Hypertension in Obstructive Hypertrophic Cardiomyopathy

Study the Pulmonary Hypertension among Heavy Smokers Young Adult Males before the Clinical Evidences of Chronic Lung Disease

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