Pulmonary Hypertension Risk in Patients with Hemoglobin H Disease: Low Incidence and Absence of Correlation with Splenectomy

Yin, Xiaolin; Zhang, Xinhua; Wu, Zhao-Hui; Zhao, Dong-Hai; Zhou, Yali; Yu, Yue-Hua; Tie-niu, Liu; Fang, Su-Ping; Zhou, Tianhong; Wang, Li; Huang, Jie

doi:10.1159/000347177

Cited by 13 publications

(7 citation statements)

References 26 publications

(36 reference statements)

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“…An elevation of pulmonary vascular resistance cannot be excluded, but is not necessary to explain the mild elevation of TRV seen in the 5 subjects with elevated TRV in our study. Consistent with our findings, a recent study has also reported a very low incidence of possible pulmonary hypertension in HbH subjects [34]. Why pulmonary hypertension appears to be much less common in HbH disease compared to in TI is not known, but nor is it fully understood why pulmonary hypertension is more likely in TI than TM.…”

Section: Discussionsupporting

confidence: 92%

Benign Cardiac Effects of Hemoglobin H Disease

Sheeran

Bowden²,

Pasricha³

et al. 2016

Acta Haematol

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Background/Aims: Hemoglobin H (HbH) disease is associated with iron overload, but whether this results in serious cardiac or vascular sequelae is unresolved. Methods: We identified 39 adult subjects (age 42 ± 12 years, 13 men) with HbH disease who had undergone echocardiography, 27 of whom had also undergone cardiac and liver magnetic resonance assessment of iron loading using T2*-weighted imaging. Results: None of the subjects had a history of heart failure or arrhythmias. There were 13/39 subjects with a ferritin level within the sex-based normal range and only 4/39 had ferritin >1,000 ng/ml. Left ventricular (LV) and left atrial dilatation was common, but LV ejection fraction was normal (≥55%) in all subjects. Age was positively correlated with log ferritin in the 27 nontransfused subjects (r = 0.43) and was inversely correlated with the transmitral E wave and E/A ratio (r = -0.69 and r = -0.79, respectively), but no relation of log ferritin with E or E/A was evident. The peak tricuspid regurgitation velocity was normal in 24/29 subjects for whom this was obtained, and it was no more than mildly elevated in the other 5. None of the tested subjects had an abnormal cardiac T2* reading, but half had evidence of liver iron loading. Conclusion: No myocardial iron loading or serious cardiac or vascular sequelae were identified in this cohort with HbH disease.

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Section: Discussionsupporting

confidence: 92%

Benign Cardiac Effects of Hemoglobin H Disease

Sheeran

Bowden²,

Pasricha³

et al. 2016

Acta Haematol

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“…In one study examining pulmonary hemodynamic measurements by echocardiogram in α‐thalassemia patients, only 3/80 (4%) patients with either HgH or Bart's disease had echocardiographic evidence of PH as defined by a TRV greater than 2.9 m/s . In the largest study of HgH patients, out of 198 patients, 13 patients (7%) had TRVs greater than 2.5 m/s as measured by echocardiogram, and only three (2%) of those had TRVs greater than 3.0 m/s . Interestingly, splenectomy was negatively correlated with an elevated TRV, which conflicts with findings in splenectomized β‐thalassemia subjects.…”

Section: Thalassemiasmentioning

confidence: 99%

Pulmonary hypertension associated with thalassemia syndromes

Fraidenburg

Machado

2016

Annals of the New York Academy of Sciences

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Chronic hemolytic anemia has increasingly been identified as an important risk factor for the development of pulmonary hypertension. Within the thalassemia syndromes, there are multiple mechanisms, both distinct and overlapping, by which pulmonary hypertension develops and that differ among β-thalassemia major or intermedia patients. Pulmonary hypertension in β-thalassemia major correlates with the severity of hemolysis, yet in patients whose disease is well treated with chronic transfusion therapy, the development of pulmonary hypertension can be related to cardiac dysfunction and the subsequent toxic effects of iron overload rather than hemolysis. β-thalassemia intermedia, on the other hand, has a higher incidence of pulmonary hypertension owing to the low level of hemolysis that exists over years without the requirement for frequent transfusions, while splenectomy is shown to play an important role in both types. Standard therapies such as chronic transfusion have been shown to mitigate pulmonary hypertension, and appropriate chelation therapy can avoid the toxic effects of iron overload, yet is not indicated in many patients. Limited evidence exists for the use of pulmonary vasodilators or other therapies, such as L-carnitine, to treat pulmonary hypertension associated with thalassemia. Here we review the most recent findings regarding the pathogenic mechanisms, epidemiology, presentation, diagnosis, and treatment of pulmonary hypertension in thalassemia syndromes.

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“…Some studies regarding certain conditions did not show a link (e.g., hemoglobin H disease, hereditary spherocytosis, idiopathic thrombocytopenic purpura) or reported conflicting results (thalassemia) (6)(7)(8)(9)(10)(11)(12)(13)(14)(15). However, many of these studies involved a smaller number of subjects and used noninvasive means to screen for pulmonary hypertension, mainly echocardiography.…”

Section: Literature Reviewmentioning

confidence: 99%

“…The contribution of post-splenectomy thrombocytosis to the observed propensity for coagulation is controversial (6,8,20,36,42,89,106,107). Thrombocytosis is not necessary to develop thromboembolic complications after splenectomy and alone does not explain the procoagulant state (24,27,32,89).…”

Section: The Role Of Post-splenectomy Thrombocytosismentioning

confidence: 99%

Review of the Association between Splenectomy and Chronic Thromboembolic Pulmonary Hypertension

Kimmig

Palevsky

2016

Annals ATS

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Recent evidence suggests that there may be a link between splenectomy and the later development of pulmonary hypertension, in particular World Health Organization group IV pulmonary hypertension (chronic thromboembolic pulmonary hypertension). Epidemiological studies have demonstrated an odds ratio as high as 18 for the development of chronic thromboembolic pulmonary hypertension after splenectomy in comparison with matched control subjects who have not undergone splenectomy. The mechanisms governing the association between removal of the spleen and the subsequent development of chronic thromboembolic pulmonary hypertension remain incompletely understood; however, recent advances in understanding of coagulation homeostasis have shed some light on this association. Splenectomy increases the risk of venous thromboembolic disease, a necessary precursor of chronic thromboembolic pulmonary hypertension, by generating a prothrombotic state. This prothrombotic state likely results from a reduction in the removal of circulating procoagulant factors from the bloodstream after splenectomy. Although much is to be learned, circulating microparticles have emerged as the most likely mediator for the development of thrombosis after splenectomy. Apparently because of a reduction in reticuloendothelial cell clearance, microparticle levels are elevated in patients after splenectomy. Elevated circulating microparticle levels have been linked to thromboembolism and pulmonary hypertension in a dose-dependent fashion. It is important for health care providers to be aware of the link between splenectomy and chronic thromboembolic pulmonary hypertension. We are optimistic that clarification of the exact mechanisms that govern this association will yield clinical guidelines and potential treatments.

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Pulmonary Hypertension Risk in Patients with Hemoglobin H Disease: Low Incidence and Absence of Correlation with Splenectomy

Cited by 13 publications

References 26 publications

Benign Cardiac Effects of Hemoglobin H Disease

Benign Cardiac Effects of Hemoglobin H Disease

Pulmonary hypertension associated with thalassemia syndromes

Review of the Association between Splenectomy and Chronic Thromboembolic Pulmonary Hypertension

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