Hemochromatosis echocardiography or ultrasound: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Hemochromatosis}} | {{Hemochromatosis}} | ||
{{CMG}}; {{AE}}{{SKA}} | {{CMG}} {{shyam}}; {{AE}}{{SKA}} | ||
==Overview== | ==Overview== | ||
On echocardiogram left ventricular hypertrophy can be seen. On ultrasound the | On echocardiogram, left ventricular hypertrophy can be seen. On ultrasound the iron deposits in the liver usually do not alter liver echogenicity. If ultrasonographic liver abnormalities are present, they are usually secondary to cirrhosis. | ||
==Echocardiogram== | ==Echocardiogram== | ||
Based on the history, specific tests might be done in order to monitor organ dysfunction, such as an [[echocardiogram]] for [[heart failure]].<ref name="pmid2959141">{{cite journal| author=Olson LJ, Baldus WP, Tajik AJ| title=Echocardiographic features of idiopathic hemochromatosis. | journal=Am J Cardiol | year= 1987 | volume= 60 | issue= 10 | pages= 885-9 | pmid=2959141 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2959141 }}</ref> | Based on the history, specific tests might be done in order to monitor organ dysfunction, such as an [[echocardiogram]] for [[heart failure]].<ref name="pmid2959141">{{cite journal| author=Olson LJ, Baldus WP, Tajik AJ| title=Echocardiographic features of idiopathic hemochromatosis. | journal=Am J Cardiol | year= 1987 | volume= 60 | issue= 10 | pages= 885-9 | pmid=2959141 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2959141 }}</ref> | ||
* It is used to diagnose the involvement of heart and stage the progress of cardiac function. | * It is used to diagnose the involvement of heart and stage the progress of cardiac function. | ||
* Echocardiography plays a role in the diagnosis and follow up during treatment. | * Echocardiography plays a role in the diagnosis and follow up during treatment. | ||
* Findings are non-specific, with a restrictive or dilated cardiomyopathy profile. | * Findings are non-specific, with a restrictive or dilated cardiomyopathy profile. | ||
* Mild left ventricular dilatation, normal or mildly increased wall thickness, biatrial enlargement. Right ventricular involvement with normal size and increased wall thickness. | * Mild left ventricular dilatation, normal or mildly increased wall thickness, biatrial enlargement. [[Right ventricle|Right ventricular]] involvement with normal size and increased wall thickness. | ||
* Left ventricular diastolic function – precedes systolic dysfunction. The degree of impairment correlates with the severity of iron overload, with a restrictive filling pattern in advanced stages Palka et al.<ref name="pmid18609062">{{cite journal| author=Davidsen ES, Omvik P, Hervig T, Gerdts E| title=Left ventricular diastolic function in patients with treated haemochromatosis. | journal=Scand Cardiovasc J | year= 2009 | volume= 43 | issue= 1 | pages= 32-8 | pmid=18609062 | doi=10.1080/14017430802203811 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18609062 }}</ref> | * [[Left ventricular]] diastolic function – precedes systolic dysfunction. The degree of impairment correlates with the severity of iron overload, with a restrictive filling pattern in advanced stages Palka et al.<ref name="pmid18609062">{{cite journal| author=Davidsen ES, Omvik P, Hervig T, Gerdts E| title=Left ventricular diastolic function in patients with treated haemochromatosis. | journal=Scand Cardiovasc J | year= 2009 | volume= 43 | issue= 1 | pages= 32-8 | pmid=18609062 | doi=10.1080/14017430802203811 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18609062 }}</ref> | ||
* Left ventricular systolic function – usually preserved in early stages, with regional and global dysfunction in later stages, when iron overload reaches a critical level. | * [[Left ventricle|Left ventricular]] systolic function – usually preserved in early stages, with regional and global dysfunction in later stages, when iron overload reaches a critical level. | ||
* Tissue | * Tissue doppler imaging for subclinical myocardial involvement - studies have shown reduced longitudinal systolic and early diastolic velocities to be early signs of disease. In addition, a reduced increase of basal septal systolic velocities during exercise was found when compared to normal.<ref name="pmid22811786">{{cite journal| author=Barbero U, Destefanis P, Pozzi R, Longo F, Piga A| title=Exercise Stress Echocardiography with Tissue Doppler Imaging (TDI) Detects Early Systolic Dysfunction in Beta-Thalassemia Major Patients without Cardiac Iron Overload. | journal=Mediterr J Hematol Infect Dis | year= 2012 | volume= 4 | issue= 1 | pages= e2012037 | pmid=22811786 | doi=10.4084/MJHID.2012.037 | pmc=3395699 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22811786 }}</ref> | ||
* Left atrial contractile function, particularly active emptying fraction, was studied as a very early echocardiographic sign of abnormal diastolic function in asymptomatic patients.<ref name="pmid19889655">{{cite journal| author=Nihoyannopoulos P, Dawson D| title=Restrictive cardiomyopathies. | journal=Eur J Echocardiogr | year= 2009 | volume= 10 | issue= 8 | pages= iii23-33 | pmid=19889655 | doi=10.1093/ejechocard/jep156 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19889655 }}</ref> | * Left atrial contractile function, particularly active emptying fraction, was studied as a very early echocardiographic sign of abnormal diastolic function in asymptomatic patients.<ref name="pmid19889655">{{cite journal| author=Nihoyannopoulos P, Dawson D| title=Restrictive cardiomyopathies. | journal=Eur J Echocardiogr | year= 2009 | volume= 10 | issue= 8 | pages= iii23-33 | pmid=19889655 | doi=10.1093/ejechocard/jep156 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19889655 }}</ref> | ||
* Increased pulmonary pressures at rest and during exercise due to elevated left ventricular filling pressures. | * Increased pulmonary pressures at rest and during exercise due to elevated left ventricular filling pressures. | ||
* '''Iron overload cardiomyopathy''': | * '''Iron overload cardiomyopathy''': | ||
** Hereditary (primary) hemochromatosis, an autosomal recessive disorder characterized by gene mutations involving iron metabolism, leading to increase iron uptake in the gastrointestinal tract. | ** Hereditary (primary) hemochromatosis, an [[autosomal recessive]] disorder characterized by gene mutations involving iron metabolism, leading to increase iron uptake in the [[gastrointestinal tract]]. | ||
** Transfusional hemochromatosis secondary to chronic blood transfusions in patients with ineffective erythropoiesis (increased catabolism) as found in thalassemia or sickle cell anemia. | ** Transfusional hemochromatosis secondary to chronic blood transfusions in patients with ineffective [[erythropoiesis]] (increased catabolism) as found in [[thalassemia]] or [[sickle cell anemia]]. | ||
==Ultrasound== | ==Ultrasound== | ||
On ultrasound the | On ultrasound the iron deposits in the liver usually do not alter liver echogenicity. If ultrasonographic liver abnormalities are present, they are usually secondary to cirrhosis. | ||
==References== | ==References== | ||
Latest revision as of 18:27, 1 January 2019
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Shyam Patel [2]; Associate Editor(s)-in-Chief: Sunny Kumar MD [3]
Overview
On echocardiogram, left ventricular hypertrophy can be seen. On ultrasound the iron deposits in the liver usually do not alter liver echogenicity. If ultrasonographic liver abnormalities are present, they are usually secondary to cirrhosis.
Echocardiogram
Based on the history, specific tests might be done in order to monitor organ dysfunction, such as an echocardiogram for heart failure.[1]
- It is used to diagnose the involvement of heart and stage the progress of cardiac function.
- Echocardiography plays a role in the diagnosis and follow up during treatment.
- Findings are non-specific, with a restrictive or dilated cardiomyopathy profile.
- Mild left ventricular dilatation, normal or mildly increased wall thickness, biatrial enlargement. Right ventricular involvement with normal size and increased wall thickness.
- Left ventricular diastolic function – precedes systolic dysfunction. The degree of impairment correlates with the severity of iron overload, with a restrictive filling pattern in advanced stages Palka et al.[2]
- Left ventricular systolic function – usually preserved in early stages, with regional and global dysfunction in later stages, when iron overload reaches a critical level.
- Tissue doppler imaging for subclinical myocardial involvement - studies have shown reduced longitudinal systolic and early diastolic velocities to be early signs of disease. In addition, a reduced increase of basal septal systolic velocities during exercise was found when compared to normal.[3]
- Left atrial contractile function, particularly active emptying fraction, was studied as a very early echocardiographic sign of abnormal diastolic function in asymptomatic patients.[4]
- Increased pulmonary pressures at rest and during exercise due to elevated left ventricular filling pressures.
- Iron overload cardiomyopathy:
- Hereditary (primary) hemochromatosis, an autosomal recessive disorder characterized by gene mutations involving iron metabolism, leading to increase iron uptake in the gastrointestinal tract.
- Transfusional hemochromatosis secondary to chronic blood transfusions in patients with ineffective erythropoiesis (increased catabolism) as found in thalassemia or sickle cell anemia.
Ultrasound
On ultrasound the iron deposits in the liver usually do not alter liver echogenicity. If ultrasonographic liver abnormalities are present, they are usually secondary to cirrhosis.
References
- ↑ Olson LJ, Baldus WP, Tajik AJ (1987). "Echocardiographic features of idiopathic hemochromatosis". Am J Cardiol. 60 (10): 885–9. PMID 2959141.
- ↑ Davidsen ES, Omvik P, Hervig T, Gerdts E (2009). "Left ventricular diastolic function in patients with treated haemochromatosis". Scand Cardiovasc J. 43 (1): 32–8. doi:10.1080/14017430802203811. PMID 18609062.
- ↑ Barbero U, Destefanis P, Pozzi R, Longo F, Piga A (2012). "Exercise Stress Echocardiography with Tissue Doppler Imaging (TDI) Detects Early Systolic Dysfunction in Beta-Thalassemia Major Patients without Cardiac Iron Overload". Mediterr J Hematol Infect Dis. 4 (1): e2012037. doi:10.4084/MJHID.2012.037. PMC 3395699. PMID 22811786.
- ↑ Nihoyannopoulos P, Dawson D (2009). "Restrictive cardiomyopathies". Eur J Echocardiogr. 10 (8): iii23–33. doi:10.1093/ejechocard/jep156. PMID 19889655.