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Cardiomegaly

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Enlarged heart; heart enlargement;

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor in Chief: Cafer Zorkun, M.D., Ph.D. [2]

Overview

Cardiomegaly is defined as an enlargement of the heart above its normal size. Cardiomegaly is generally first detected on chest X ray, and less often it is detected on routine physical examination. Cardiomegaly may be the first sign of an occult systemic or cardiovascular disease.

Pathophysiology

Cardiomegaly involves two main processes in the heart muscle. Hypertrophy causes the heart to enlarge due to thickening to the cardiac muscle, and dilation causes enlargement due to stretching of the heart muscle. Dilation occurs as a result of volume overload in the heart.

Causes

There are many causes for the condition of cardiomegaly, including medications, genetic conditions, endocrine conditions, infectious processes, toxins and iatrogenic causes.

Differentiating Cardiomegaly from other Diseases

There is a large differential for cardiomegaly, as it is a finding that is seen in many conditions. The differential can be narrowed based on the chamber of the heart affected, and whether the cardiomegaly is caused by dilation or by hypertrophy. Other physical findings, imaging studies, and laboratory findings need to be taken into consideration when determining the underlying diagnosis caused the manifestation of an enlarged heart.

Diagnosis

History and Symptoms

The history and symptoms can suggest the underlying cause of cardiomegaly. The patient may have a history of rheumatic heart disease or congenital heart disease, or a history of alcohol abuse which may suggest dilated cardiomyopathy. Symptoms such as chest pain, or other cardiac or respiratory symptoms may help in illiciting the underlying cause.

Physical Examination

A comprehensive physical examination with a thorough cardiac exam will reveal cardiomegaly, and may indicated the nature of the underlying cause. The body habitus of the individual needs to be taken into account when evaluating findings on cardiac examination.

Laboratory Findings

Laboratory tests that should be obtained when a person is suspected or known to have cardiomegaly are: a complete blood count, thyroid stimulating hormone levels, and a blood urea nitrogen. These are common, high yield tests. Other tests can be ordered based on suspicions as to the underlying cause of the cardiomegaly, obtained through patient history and physical examination.

Electrocardiogram

An EKG is a standard method of evaluating a patient with cardiomegaly. It can help to determine the cause, severity, and the specific chamber which is affected. A q wave would indicate an area of muscle death accounting for the enlargement, and ST elevation may suggest a myocardial infarction, myocarditis or pericarditis.

Chest X Ray

Cardiomegaly is easily visualized on chest x ray. Cardiomegaly is traditionally defined as a cardiothoracic ratio that is more than 0.5 on a PA film. Other findings on chest x ray can help to determine the specific chamber that is contributing most to the enlargement of the heart.

Echocardiogram

Echocardiogram recommended for those patients presenting suspected valvular disease, aberrant chamber size, ventricular function, and wall motion abnormalities.

Other Diagnostic Studies

Exercise or pharmacological stress testing may be indicated in those patients suspected of having coronary artery disease. Patients suspected of having valvular disease or coronary artery disease may need a cardiac catheterization for full evaluation following echocardiography.

Treatment

Medical Therapy

There are both acute and chronic pharmacotherapies used to manage the cardiomegaly. Diuretics and ACE inhibitors are the standard therapy for chronic management of cardiomegaly. Acute therapies include digoxin, diuretics, antiarrhythmics, pre-load and after-load reducing medications.

Surgery

In patients who are awaiting a transplant for end-stage symptomatic heart failure, implantable ventricular assist devices may serve as a temporary aid for compensation. Transplantation may be considered in a few cases.

References

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Historical Perspective

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References

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Overview

Cardiomegaly involves two main processes in the heart muscle. Hypertrophy causes the heart to enlarge due to thickening to the cardiac muscle, and dilation causes enlargement due to stretching of the heart muscle. Dilation occurs as a result of volume overload in the heart.

Pathophysiology

The left ventricle can be enlarged from two broad underlying conditions: dilation and hypertrophy.

Left Ventricular Dilation

Left ventricular dilation can occurs as a result of volume overload. Conditions that cause volume overload can be further broken down as follows:

Left Ventricular Hypertrophy

Left ventricular hypertrophy occur due to factors that can cause the heart to work harder than normal. Cardiac hypertrophy is seen in the following condtions:

Pathology

Gross Pathology

Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

Biventricular Hypertrophy


Biventricular Hypertrophy


Gross excellent example of concentric left ventricular hypertrophy


Left Ventricular Hypertrophy: Gross natural color anterior view intact heart showing disproportionate size of left ventricle by its inferior extent much below the right ventricle apex (quite good example)


Myocardial Infarct: Gross natural color apical section showing large left ventricle infarct and right ventricular hypertrophy


Right ventricular hypertrophy


Right ventricular enlargement due to a patent ductus arteriosus in a patient with hyaline membrane disease


References


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Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Mugilan Poongkunran M.B.B.S [2]

Overview

Cardiomegaly is often caused by hypertension, but it can also occur as a result of other heart conditions, such as congestive heart failure. Common causes of cardiomegaly due to dilation of the myocardium (not hypertrophy) are dilated cardiomyopathy (non-ischemic cardiomyopathy) and ischemic heart disease.

Causes

Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.

Common Causes

Causes by Organ System

Cardiovascular ALCAPA, aortic dissection, aortic regurgitation, aortic stenosis, arrhythmia, atrial septal defect, carcinoid heart disease, cardiac myxoma, cardiofaciocutaneous syndrome, chronic thromboebolic pulmonary hypertension, coarctation of aorta, congenital heart disease, congestive heart failure, cor pulmonale, Costello syndrome, Ebstein’s anomaly, Eisenmenger’s syndrome, endocardial fibroelastosis, endocarditis, familial hypertrophic cardiomyopathy, heart attack, hypertension, hypertrichotic osteochondrodysplasia, idiopathic dilated cardiomyopathy, ischemic heart disease, Kawasaki disease, mitral regurgitation, mitral valve prolapse, myocardial infarction, myocarditis, Naxos disease, patent ductus arteriosus, pericardial effusion, pericarditis, peripartum cardiomyopathy, polyarteritis nodosa, pulmonary embolism, pulmonary hypertension, pulmonary regurgitation, pulmonary stenosis, rheumatic fever, Salih myopathy, tetralogy of fallot, transposition of great vessels, tricuspid regurgitation, tricuspid prolapse, valvular heart disease, ventricular aneurysm, ventricular septal defect, X-linked dilated cardiomyopathy
Chemical / poisoning Beryllium, carbon monoxide, cobalt, ethylene glycol
Dermatologic Cardiofaciocutaneous syndrome, Costello syndrome, hypertrichotic osteochondrodysplasia, multiple lentigines syndrome, Naxos disease
Drug Side Effect Anagrelide, anastrozole, aspirin, atenolol, bleomycin, carfilzomib, certolizumab pegol, didanosine, disopyramide, donepezil, doxorubicin, dronedarone, ethanol, fludrocortisone, hydrocortisone, lapatinib ditosylate, letrozole, liposomal amphotericin B, lithium, mecasermin (recombinant human insulin-like growth factor I), oprelvekin, phenothiazines, phenylbutazone, pindolol, pramipexole, prednisolone, procainamide, propranolol, ropinirole, rosiglitazone, sunitinib malate, synthetic androgens, trastuzumab, triamcinolone , troglitazone, verapamil, zidovudine
Ear Nose Throat No underlying causes
Endocrine Acromegaly, carcinoid heart disease, cushing syndrome, diabetes, hyperthyroidism, hypothyroidism
Environmental No underlying causes
Gastroenterologic Mulibrey nanism
Genetic 1p36 deletion syndrome, Alstrom syndrome, Barth syndrome, Becker muscular dystrophy, Beckwith–Wiedemann syndrome, cardiofaciocutaneous syndrome, carnitine palmitoyl transferase deficiency, congenital generalized lipodystrophy, Costello syndrome, Danon disease, desmin-related myofibrillar myopathy, Duchenne muscular dystrophy, Emery-Dreifuss muscular dystrophy, Fabry disease, familial hypertrophic cardiomyopathy, Friedreich ataxia, fucosidosis, Gaucher disease, glycogenosis type 2b, hemochromatosis, hereditary spherocytosis, Hunter syndrome, hypertrichotic osteochondrodysplasia, infantile sialic acid storage disorder, isobutyryl-CoA dehydrogenase deficiency, Laing distal myopathy, long-chain acyl-CoA dehydrogenase deficiency, malonyl-CoA decarboxylase deficiency, McLeod neuroacanthocytosis syndrome, MELAS, Morquio syndrome, mucopolysaccharidosis type IIIB, Hurler syndrome, Marfan’s syndrome, Mulibrey nanism, multiple lentigines syndrome, myotonic dystrophy, Naxos disease, Noonan syndrome, Pompe’s disease, Refsum disease, Salih myopathy, Sandhoff disease, sickle cell disease, Singleton-Merten syndrome, Tay-Sachs’ disease, thalassemia, very long-chain acyl-CoA dehydrogenase deficiency, X-linked dilated cardiomyopathy, Yunis-Varon syndrome
Hematologic Anemia, hemochromatosis, hereditary spherocytosis, McLeod neuroacanthocytosis syndrome, sickle cell disease, thalassemia
Iatrogenic Water overload, postradiation, cardiac catheterization, cardiopulmonary resuscitation, heart surgery, percutaneous coronary intervention, postpericardiotomy syndrome
Infectious Disease Brucellosis, chagas disease, coxsackie virus, cryptococcosis, cytomegalovirus, diphtheria, echovirus, Epstein-Barr, hepatitis, histoplasmosis, HIV, Lyme disease, psittacosis, rheumatic fever, rickettsial disease, schistosomiasis, toxoplasmosis, trichinosis, trypanosomiasis, typhoid fever, varicella
Musculoskeletal / Ortho Becker muscular dystrophy, desmin-related myofibrillar myopathy, Duchenne muscular dystrophy, Emery-Dreifuss muscular dystrophy, X-linked, hypertrichotic osteochondrodysplasia, Laing distal myopathy, Mulibrey nanism, myotonic dystrophy, Salih myopathy, Yunis-Varon syndrome
Neurologic Costello syndrome, Friedreich ataxia, Mulibrey nanism
Nutritional / Metabolic Alstrom syndrome, Barth syndrome, beri-beri, carnitine palmitoyltransferase deficiency, Fabry disease, fucosidosis, Gaucher disease, glycogenosis type 2b, Hunter syndrome, infantile sialic acid storage disorder, isobutyryl-CoA dehydrogenase deficiency, kwashiorkor, long-chain acyl-CoA dehydrogenase deficiency, malonyl-CoA decarboxylase deficiency, Morquio syndrome, mucopolysaccharidosis type IIIB, Pompe’s disease, Refsum disease, Sandhoff disease, selenium deficiency, Tay-Sachs’ disease
Obstetric/Gynecologic Hemolytic disease of the newborn, infants born to diabetic mothers, peripartum cardiomyopathy, peripartum heart failure, pregnancy, twin-to-twin transfusion syndrome
Oncologic Carcinoid heart disease, cardiac myxoma, lymphadenopathy, mediastinal mass, pheochromocytoma
Opthalmologic Mulibrey nanism, Alstrom syndrome
Overdose / Toxicity Anastrozole, lithium, mecasermin (recombinant human insulin-like growth factor I), olanzapine, oprelvekin, phenothiazines, phenylbutazone, pindolol, procainamide, rosiglitazone, sunitinib malate, trastuzumab, troglitazone, verapamil, zidovudine
Psychiatric Takotsubo cardiomyopathy
Pulmonary Coal worker pneumoconiosis, COPD, cor pulmonale, fibrosing mediastinitis, pulmonary embolism, pulmonary hypertension, sleep apnea
Renal / Electrolyte Hypocalcemia, hypophosphatemia, renal failure, uremia
Rheum / Immune / Allergy Amyloidosis, systemic lupus erythematosus
Sexual No underlying causes
Trauma Blunt or penetrating chest trauma, esophogeal perforation
Urologic No underlying causes
Dental No underlying causes
Miscellaneous Alcoholism, athletes

Causes in Alphabetical Order

Cardiomegaly causes developed by WikiDoc.org

References

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Differentiating Cardiomegaly from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: pseudo-cardiomegaly; pseudocardiomegaly

Overview

Cardiomegaly must be distinguished from pseudo-cardiomegaly, a scenario in which the apparent size of the heart is artificially increased. Disorders and conditions which falsely increase the apparent size of the heart include straight back syndrome, a disorder in which the heart is “pancaked” or compressed; and obtaining the chest x-ray during expiration at which time the diameter of the lungs is decreased and the relative diameter of the heart and aorta is increased.

Diseases From Which Cardiomegaly Must Be Distinguished

References


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Epidemiology and Demographics

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References

Risk factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Cardiomegaly (enlarged heart) can be caused by conditions that increase the workload of heart i,e to pump harder than usual or that damage the heart muscle. Established risk factors for cardiomegaly include family history of cardiomegaly, high blood pressure, high cholesterol, obesity, smoking, and coronary artery disease.

Risk Factors

Patients have a greater risk of developing an enlarged heart if they have any of the following risk factors:

  • Age: Age as an risk factor of cardiomegaly has been controversial. Some studies have shown no increase in heart size with age if hypertension and other heart diseases are carefully excluded.[1] However elderly people are at increased risk for developing cardiomegaly because of other comorbid conditions.
  • Ethnicity : African American
  • Gender : Males have an increased risk than females for developing cardiomegaly.
  • Alcohol: Heavy alcoholism has significant direct cardiac effects and also results in hypertensive heart disease[2]
  • Congenital heart disease: Genetic condition that affects the structure of heart increases the risk for developing cardiomegaly, especially when the condition is not treated at early stage of life.
  • Family history of heart disease: A family history of cardiomyopathy increases the risk of deveopling cardiomegaly.
  • High blood pressure: Blood pressure >140/90 mmHg results in remodeling of left ventricular myocardium.[3]
  • Hyperlipidemia: Lipid and lipoprotein abnormalities are common in the general population and are regarded as a modifiable risk factor for cardiovascular diseases due to their contribution to atherosclerosis.
  • Obesity: Excess weight contributed significantly to formation of structural and geometric changes of left ventricle and left atrium.
  • Physical exercise: Intense, prolonged endurance and strength training can cause the heart to adapt to the extra workload.
  • Tobacco smoking: Smoking tends to increase blood cholesterol levels and contributes to the risk of developing heart disease.
  • Valvular heart disease : Valves may be damaged by a variety of conditions leading to stenosis, regurgitation or prolapse. Any of these conditions may cause the heart to enlarge.

References

  1. Hodkinson I, Pomerance A, Hodkinson HM (1979). “Heart size in the elderly: a clinicopathological study”. J R Soc Med. 72 (1): 13–6. PMC 1436754. PMID 162207.
  2. Marmot MG, Elliott P, Shipley MJ, Dyer AR, Ueshima H, Beevers DG; et al. (1994). “Alcohol and blood pressure: the INTERSALT study”. BMJ. 308 (6939): 1263–7. PMC 2540174. PMID 7802765.
  3. Plotnikova IV, Sokolov AA, Kovalev IA, Bezliak VV (2012). “[Remodeling of left ventricular myocardium at early stages of essential arterial hypertension in adolescents]”. Kardiologiia. 52 (12): 28–33. PMID 23237438.
Screening

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References

Natural History, Complications and Prognosis

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Diagnosis

Diagnosis

History and Symptoms | Physical examination | Laboratory Findings | Electrocardiogram | Chest X ray | MRI | CT | Echocardiogpraphy or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical therapy | Surgery | Prevention | Cost-Effectiveness of Therapy | Future or Investigational therapies

Case Studies

Case Studies

Case #1


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