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Published on: May 11, 2015
Pulmonary hypertension in sickle cell disease
Guilherme Fonseca1, Rogerio Souza
1aDepartment of Hematology bDepartment of Pulmonary, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil.
Pulmonary hypertension is a common complication in sickle cell disease (SCD), significantly impacting patient prognosis. Accurate diagnosis requires invasive hemodynamics, as echocardiogram limitations exist for SCD-associated pulmonary hypertension.
Area of Science:
- Cardiology
- Hematology
- Pulmonology
Background:
- Pulmonary hypertension is a serious chronic complication of sickle cell disease (SCD).
- It significantly influences the overall prognosis for individuals with SCD.
Purpose of the Study:
- To review the prevalence and prognostic importance of pulmonary hypertension in SCD.
- To highlight diagnostic challenges and pathophysiological complexities.
Main Methods:
- Review of recent literature on SCD-associated pulmonary hypertension.
- Emphasis on limitations of echocardiography.
- Necessity of invasive hemodynamic assessment for diagnosis.
Main Results:
- Echocardiogram has limitations for diagnosing SCD-associated pulmonary hypertension, necessitating invasive hemodynamics.
- Hemodynamic profile in SCD-associated pulmonary hypertension shows elevated cardiac output and low pulmonary vascular resistance, differing from pulmonary arterial hypertension.
- Both precapillary and postcapillary profiles can occur, indicating complex pathophysiology.
- Any form of pulmonary hypertension worsens prognosis in SCD.
Conclusions:
- Pulmonary hypertension is a prevalent complication of SCD with diverse underlying mechanisms.
- Further research is needed to support aggressive management of the baseline condition and targeted pulmonary arterial hypertension therapies in SCD patients.
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