The association between tricuspid regurgitation velocity and 5‐year survival in a <scp>N</scp>orth <scp>W</scp>est <scp>L</scp>ondon population of patients with sickle cell disease in the <scp>U</scp>nited <scp>K</scp>ingdom

Cabrita, Inês Zimbarra; Mohammed, A.; Layton, Mark; Ghorashian, Sara; Gilmore, Annette; Cho, Gavin; Howard, Jo; Anie, Kofi A.; Desforges, L; Bassett, Paul; Grapsa, Julia; Howard, L.; Mahalingam, Gaia; Dawson, David M.; Pinto, Fausto J.; Nihoyannopoulos, Petros; Davies, Sally C.; Gibbs, J. Simon R.

doi:10.1111/bjh.12391

Cited by 28 publications

(21 citation statements)

References 30 publications

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“…Forty full‐text articles were assessed for eligibility (Figure 1). Fifteen (8 RHC and 7 echocardiography) studies were included in the meta‐analysis 2, 3, 4, 5, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20. The characteristics of the included studies are shown in Table 2.…”

Section: Resultsmentioning

confidence: 99%

“…Studies included in this analysis are Valerio et al,10 Heresi et al,11 Kovacs et al,5 Suzuki et al,12 Maron et al,3 Takahashi et al,13 Douschan et al,4 Assad et al,2 Abramson et al,14 Kjaergaard et al,15 Shalaby et al,16 Lam et al,17 Damy et al,18 Cabrita et al,19 and Choudhary et al 20. CI indicates confidence interval; M‐H, Mantel‐Haenszel; RHC, right heart catheterization.…”

Section: Resultsmentioning

confidence: 99%

“…Studies included in this analysis are Heresi et al,11 Suzuki et al,12 Maron et al,3 Takahashi et al,13 Assad et al,2 Shalaby et al,16 Cabrita et al,19 and Choudhary et al 20. CI indicates confidence interval; PH, pulmonary hypertension; IV, Inverse Variance; RHC, right heart catheterization.…”

Section: Resultsmentioning

confidence: 99%

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Mild Pulmonary Hypertension Is Associated With Increased Mortality: A Systematic Review and Meta‐Analysis

Kolte

Lakshmanan

Jankowich

et al. 2018

JAHA

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BackgroundRecent studies have demonstrated a continuum in clinical risk related to mean pulmonary artery pressure that begins at >19 mm Hg, which is below the traditional threshold used to define pulmonary hypertension (PH) of 25 mm Hg. Because of the implications on patient diagnosis and prognosis, the generalizability and validity of these data need further confirmation.Methods and ResultsDatabases were searched from inception through January 31, 2018, to identify studies comparing all‐cause mortality between patients with mildly elevated mean pulmonary artery pressure near but <25 mm Hg versus the referent group. The meta‐analysis included 15 nonrandomized studies and 16 482 patients (7451 [45.2%] with measured or calculated mean pulmonary artery pressure of 19–24 mm Hg by right heart catheterization [n=6037] and echocardiography [n=1414] [mild PH]). The mean duration of follow‐up was 5.2 years. Compared with the referent group, mild PH was associated with an increased risk of mortality (risk ratio, 1.52; 95% confidence interval, 1.32–1.74; P<0.001; I2=47%). Secondary analysis using risk‐adjusted time‐to‐event estimates showed a similar result (hazard ratio, 1.19; 95% confidence interval, 1.09–1.31; P<0.001; I2=42%). The findings were consistent between subgroups of right heart catheterization and echocardiography studies (P interaction>0.05). There was evidence of publication bias; however, this did not influence the risk estimate (Duval and Tweedie's trim and fill adjusted risk ratio, 1.34; 95% confidence interval, 1.15–1.56).ConclusionsThe risk of mortality is increased in patients with mild PH, defined as measured or calculated mean pulmonary artery pressure >19 mm Hg. These data emphasize a need for diagnosing patients with mild PH with consideration to enrollment in PH clinical studies investigating pharmacological and nonpharmacological interventions to attenuate clinical risk and improve outcomes.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Mild Pulmonary Hypertension Is Associated With Increased Mortality: A Systematic Review and Meta‐Analysis

Kolte

Lakshmanan

Jankowich

et al. 2018

JAHA

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“…Diastolic dysfunction is an independent risk factor for early mortality in SCD(7,8). In our patients, diastolic dysfunction was associated with LA dilation, which was the most severely and disproportionately enlarged heart chamber.…”

Section: Discussionmentioning

confidence: 99%

“…In addition, the degree of PH, which is often mild in individuals with SCD, does not readily explain the full extent of cardiac morbidity and frequency of early mortality in SCD(7). Nevertheless, TRV, pulmonary pressure and diastolic dysfunction are clearly associated with increased mortality and sudden death in SCD(2,7,8). The causes of cardiac-related mortality in the absence of apparently clinically significant PH have remained unclear.…”

Section: Introductionmentioning

confidence: 99%

Cardiomyopathy With Restrictive Physiology in Sickle Cell Disease

Niss¹,

Quinn²,

Lane³

et al. 2016

JACC: Cardiovascular Imaging

108

137

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Objective To identify a unifying cardiac pathophysiology that explains the cardiac pathology in SCD. Background Cardiopulmonary complications, the leading cause of adult mortality in sickle cell disease (SCD), are associated with heart chamber dilation, diastolic dysfunction, elevated tricuspid regurgitant jet velocity (TRV) and pulmonary hypertension (PH). However, no unifying cardiac pathophysiology has been identified to explain these findings. Methods In a two-part study, we first examined SCD patients who underwent screening echocardiography during steady state at our institution. We then conducted a meta-analysis of cardiac studies in SCD. Results In the 134 SCD patients studied (median age 11 years), a significant enlargement of the left atrial volume was present (z-score 3.1, P=0.002), shortening fraction (SF) was normal (37.6 ± 4.7%), and lateral and septal ratios of mitral velocity to early diastolic velocity of the mitral annulus (E/e′) were severely abnormal in 8% and 14% of patients, respectively, indicating impaired diastolic function. Both TRV and lateral E/e′ correlated with enlarged left atrial volume in SCD (P=0.003 and P=0.006, respectively). Meta-analysis of 68 studies confirmed significant left atrial diameter enlargement in SCD patients compared to controls, evidence of diastolic dysfunction and enlarged left ventricular end-diastolic dimension with normal SF. The majority of patients with catheter-confirmed PH had mild pulmonary venous hypertension consistent with restrictive cardiac physiology. Conclusions Patients with SCD have a unique cardiomyopathy with restrictive physiology that is superimposed on hyperdynamic physiology, and is characterized by diastolic dysfunction, left atrial dilation and normal systolic function. This results in mild, secondary, pulmonary venous hypertension and elevated TRV. Sudden death is common in other forms of restrictive cardiomyopathy. Our finding of this unique restrictive cardiomyopathy may explain the increased mortality and sudden death seen in patients with SCD with mildly elevated TRV.

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Daily pain in adults with sickle cell disease—a different perspective

et al. 2017

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Previous reports demonstrated that patients with sickle cell disease (SCD) experience pain on more than half of the observed days. Yet, these high incidences do not seem to match observations in our population. In this prospective cohort study, we aimed to assess the frequency and characteristics of daily, self-reported pain among adult SCD patients in the Netherlands. Consecutive patients were enrolled during routine outpatient visits and followed up to 6 months. A total of 55 patients completed 5,982 diary observation days. Median age was 27 years (IQR 23-43). Patients reported SCD related pain on 17% of the observed days; on 13% of these days this pain was not defined as a painful crisis, while 3% was reported as a painful crisis but managed at home, and on 1% of the observed days patients were admitted to the hospital. Analgesics were used on 52% of days with pain with a relatively infrequent use of oral opioids (9% of pain days). This first European study on pain in SCD indicates that pain appears to be significantly less frequent in our population as compared to previous study cohorts from the United States, and may be more representative for current SCD populations in other Western countries. Besides a more widespread use of hydroxycarbamide in modern disease management, differences in organization and accessibility of healthcare between countries may also explain this discrepancy.

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The association between tricuspid regurgitation velocity and 5‐year survival in a North West London population of patients with sickle cell disease in the United Kingdom

Cited by 28 publications

References 30 publications

Mild Pulmonary Hypertension Is Associated With Increased Mortality: A Systematic Review and Meta‐Analysis

Mild Pulmonary Hypertension Is Associated With Increased Mortality: A Systematic Review and Meta‐Analysis

Cardiomyopathy With Restrictive Physiology in Sickle Cell Disease

Daily pain in adults with sickle cell disease—a different perspective

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