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Ann Thorac Surg 2000;69:S180-S190
© 2000 The Society of Thoracic Surgeons

Congenital Heart Surgery Nomenclature and Database Project: pediatric cardiomyopathies and end-stage congenital heart disease

^a Division of Cardiothoracic Surgery, University of California, Davis School of Medicine, Sacramento, California, USA

Address reprint requests to Dr Delius, Div of Cardiothoracic Surgery, UC Davis Medical Center, 4301 X St, Rm 2250, Sacramento, CA 95817
e-mail: ralph.delius{at}ucdmc.ucdavis.edu

Presented at the International Nomenclature and Database Conferences for Pediatric Cardiac Surgery, 1998–1999.

Abstract

The extant nomenclature for cardiomyopathy is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A functional classification based on pathophysiology is proposed. Cardiomyopathy is subdivided into: dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy, right ventricular cardiomyopathy, and end-stage congenital heart disease. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

I. Background

Cardiomyopathy is a term loosely applied to a wide spectrum of cardiac diseases in which the predominant feature is poor myocardial function in the absence of any anatomic abnormalities. Other cardiac pathologies, such as ischemic, valvular, or congenital heart disease, which can indirectly lead to abnormally functioning heart muscle, are usually excluded, although the term "ischemic cardiomyopathy" is occasionally used in patients with poor left ventricular function due to coronary artery disease. Accurate data regarding the prevalence of cardiomyopathies in children are not currently available, but probably represent less than 1% of all children admitted to a hospital for cardiac disease [1].

The most widely quoted classification of cardiomyopathies has been that promoted by the World Health Organization [2]. In this classification scheme, the term cardiomyopathy is reserved for entities in which diseased myocardium is the predominant feature, with no gross structural basis for cardiac dysfunction. Furthermore, the term is further restricted to diseases that are of unknown cause. Diseases that affect the myocardium but are of known cause or are part of a systemic disorder are called specific heart muscle diseases [3]. This distinction, although technically accurate, is often difficult to determine and, as a consequence, a classification scheme that is primarily based on functional status rather than etiology has come into general use. This pathophysiologic classification, first proposed by Goodwin and Oakley [4], has been found to be more clinically useful and divides cardiomyopathies into three relatively easily distinguishable entities: (1) dilated, (2) hypertrophic, and (3) restrictive cardiomyopathies.

Dilated cardiomyopathies (also referred to as congestive cardiomyopathies) are characterized by ventricular dilatation and systolic dysfunction. Presenting symptoms are typically those of congestive heart failure.

Hypertrophic cardiomyopathies demonstrate physiologically inappropriate hypertrophy of the left ventricle. This hypertrophy is often asymmetric, often affecting the septum to a greater degree than the free walls of the left ventricle. Systolic function is usually normal, or even hyperkinetic, while diastolic dysfunction is often present. Varying degrees of left ventricular outflow tract obstruction are often present. Patients with hypertrophic cardiomyopathy almost never present with congestive heart failure as a first symptom. Typical initial symptoms include angina, dyspnea, palpitations, and syncope [1].

Restrictive cardiomyopathies (also referred to as obliterative cardiomyopathies) are marked by diastolic dysfunction. The left ventricle is usually normal sized or small. Systolic function is usually preserved. Marked hypertrophy is absent, although mild thickening may be observed. The clinical presentation is virtually identical to constrictive pericarditis.

Some overlap may exist between these categories; for instance, diastolic dysfunction is a feature of both hypertrophic and restrictive cardiomyopathies. However, in most cases, a relatively clear distinction can be made.

Another problematic group of patients are those with congenital heart disease and end stage heart failure, who do not have any (or any further) surgically correctable lesions. These patients by definition cannot be classified as having a classic cardiomyopathy, but constitute a large portion of pediatric patients undergoing consideration for heart transplantation. These difficult-to-classify patients will be included in this chapter. Although these patients do not have a classic cardiomyopathy, they will be considered part of the cardiomyopathy group in the hierarchical nomenclature system.

II. Analysis: a unified cardiomyopathies and end-stage congenital heart disease nomenclature system

Cardiomyopathy
Any nosology of a diverse group of pathologies should preferably have distinctions that are clear and reproductive from institution to institution. Given this, we will argue that a functional classification scheme, with a minor modification, is preferable to a classification system based on etiology. The World Health Organization (WHO) classification, in which a primary demarcation is made between idiopathic cardiomyopathies and secondary cardiomyopathies, is often clinically difficult to apply. The presenting symptoms of a given cardiomyopathy are often similar, if not identical to, those of one of the specific heart muscle diseases [3]. In turn, the pathophysiologic features of a wide range of specific heart muscle diseases are identical. The distinguishing pathologic features of a primary cardiomyopathy versus a specific heart muscle disease are sometimes difficult to discern, and the distinction between the two entities may be based, in part, on the local expertise available. Under this classification, confusing situations will arise in distinguishing primary and secondary cardiomyopathy. For instance, systemic amyloidosis with myocardial involvement would be considered amyloid disease of the heart, while amyloidosis in which cardiac involvement is the only manifestation would be considered a primary restrictive cardiomyopathy [5]. Finally, as new powerful genetic methods are used to study the various forms of cardiomyopathy, the term "idiopathic" will become increasingly archaic. Heart diseases formerly under the rubric of idiopathic cardiomyopathy are now known to be caused by mitochondrial disorders, abnormal dystrophin, and so forth. As such, distinction between idiopathic and secondary cardiomyopathies will be a moving target over time.

One may argue that etiologic factors are important in any classification scheme and ought to be retained. However, we feel that if these factors are included they should follow an initial classification based on the pathophysiologic feature described in the introduction.

A functional classification system based on pathophysiology has much support. It is already widely in use, easy to understand, and fairly reproducible. Although some features between the categories overlap, most clinicians are usually able to easily classify patients based on the pathophysiologic presentations. Etiologic factors may be known or unknown with this classification plan. In most cases, classification can be generally made by echocardiography. Cardiac catheterization and endomyocardial biopsy may occasionally provide additional clinically useful information.

The categories of dilated, hypertrophic, and restrictive cardiomyopathies cover most of the range of patients presenting with primary myocardial dysfunction. However, there is a group of patients not covered under this classification scheme that arguably should be included and are not categorizable under the current system, namely those with right ventricular cardiomyopathy. The category of right ventricular cardiomyopathy includes arrhythmogenic right ventricular dysplasia (a condition in which there is partial or total replacement of right ventricular muscle by adipose or fibrous tissue) [6], Uhl’s syndrome, and spongiform cardiomyopathy. These entities are not easily placed into one of the three existing categories. We propose that patients with a cardiomyopathy or syndrome largely confined to the right ventricle be classified as having a right ventricular cardiomyopathy.

End-stage congenital heart disease
Patients with severe heart failure and congenital heart disease are often considered for cardiac transplantation if no contraindications are present. It can be reasonably inferred that these patients do not have a surgically correctable lesion. It seems feasible that the underlying diagnosis and pathophysiology may have an impact on the short- and long-term outcome of cardiac transplantation. For instance, collateral pulmonary blood flow is more likely to be a concern in patients who have undergone a Fontan-type operation, while patients with two ventricles [5] may have more concerns regarding pulmonary vascular disease. As a consequence, we have elected to divide this group of patients into broad pathophysiologic categories, namely [7, 8, 9]:

1. Patients with no history of previous cardiac surgery
   
2. Patients who have had a palliative procedure only (in most cases a single ventricle)
   
3. Patients with single-ventricle physiology who have had a Fontan procedure
   
4. Patients with two-ventricle physiology, divided into:
   

   Patients with two-ventricle physiology with an extracardiac conduit
   

   Patients with two-ventricle physiology without an extracardiac conduit
   

   
   

Patients can be easily categorized, and the effect of the pathophysiology of the original heart defect and subsequent surgical procedures on cardiac transplantation results should be relatively simple to assess.

Cardiomyopathy hierarchy level 1

Cardiomyopathy

Cardiomyopathy hierarchy level 1 definitions
Cardiomyopathy: Myocardial abnormality in which there is systolic and/or diastolic dysfunction in the absence of any structural abnormalities or in the presence of structural congenital heart disease without any (or any further) surgically correctable lesions.

Cardiomyopathy hierarchy level 2
Cardiomyopathy, not otherwise specified (NOS)

Cardiomyopathy, Dilated

Cardiomyopathy, Hypertrophic

Cardiomyopathy, Restrictive

Cardiomyopathy, Right ventricular

Cardiomyopathy, End-stage congenital heart disease

Cardiomyopathy hierarchy level 2 definitions

Cardiomyopathy, NOS

A cardiomyopathy not further described (NOS = not otherwise specified).

Dilated cardiomyopathy. Synonyms: Congestive cardiomyopathy

Cardiomyopathy characterized by enlargement of one or both ventricles and systolic contractile dysfunction.

Hypertrophic cardiomyopathy. Synonyms: Idiopathic hypertrophic subaortic stenosis

Asymmetric septal hypertrophy

Hypertrophic obstructive cardiomyopathy

Cardiomyopathy characterized by physiologically inappropriate hypertrophy and normal ventricular systolic function.

Restrictive cardiomyopathy

Synonyms: Obliterative cardiomyopathy

Cardiomyopathy characterized by ventricular diastolic dysfunction, elevated end diastolic pressures, normal systolic function, and no significant hypertrophy or dilation.

Right ventricular cardiomyopathy

Synonyms: Arrhythmogenic right ventricular dysplasia

Cardiomyopathy characterized by right ventricular failure or dysfunction in the absence of other anatomic abnormalities.

End-stage congenital heart disease

Systolic and/or diastolic dysfunction leading to class IV symptoms in patients who have congenital heart disease but do not have any (or any further) surgically correctable lesions.

Cardiomyopathy hierarchy level 3

Cardiomyopathy, NOS

Cardiomyopathy, Dilated, NOS

Cardiomyopathy, Dilated, Primary

Cardiomyopathy, Dilated, Secondary

Cardiomyopathy, Hypertrophic, NOS

Cardiomyopathy, Hypertrophic, Obstructive

Cardiomyopathy, Hypertrophic, Nonobstructive

Cardiomyopathy, Restrictive, NOS

Cardiomyopathy, Restrictive, Myocardial

Cardiomyopathy, Restrictive, Endomyocardial

Cardiomyopathy, Right ventricular, NOS

Cardiomyopathy, Right ventricular, Arrhythmogenic right ventricular dysplasia

Cardiomyopathy, Right ventricular, Uhl’s syndrome

Cardiomyopathy, Right ventricular, Spongiform cardiomyopathy

Cardiomyopathy, End-stage congenital heart disease, NOS

Cardiomyopathy, End-stage congenital heart disease, Status after no prior cardiac surgery

Cardiomyopathy, End-stage congenital heart disease, Status after palliative procedure(s)

Cardiomyopathy, End-stage congenital heart disease, Status after single ventricle repair

Cardiomyopathy, End-stage congenital heart disease, Status after biventricular repair

Cardiomyopathy hierarchy level 3 definitions

Cardiomyopathy, hypertrophic, NOS

Hypertrophic cardiomyopathy not further described.

Cardiomyopathy, hypertrophic, obstructive

Hypertrophic cardiomyopathy in the presence of a pressure gradient across left ventricular outflow tract.

Cardiomyopathy, hypertrophic, nonobstructive

Hypertrophic cardiomyopathy in the absence of a pressure gradient across left ventricular outflow tract.

Cardiomyopathy, end-stage congenital heart disease, NOS

End-stage congenital heart disease not further described.

Cardiomyopathy, end-stage congenital heart disease, status after no prior cardiac surgery

End-stage congenital heart disease in a patient with no history of prior cardiac surgery.

Cardiomyopathy, end-stage congenital heart disease, status after palliative procedure(s)

End-stage congenital heart disease in a patient with any prior noncorrective procedure designed to increase or decrease pulmonary blood flow or increase pulmonary systemic mixing (example: Blalock-Taussig shunt, pulmonary artery band, atrial septectomy) who has not had a prior correction using any application of the Fontan principle.

Cardiomyopathy, end-stage congenital heart disease, status after single-ventricle repair

Synonyms:

Fontan procedure

Total cavopulmonary connection

End-stage congenital heart disease in a patient with any prior correction using any application of the Fontan principle.

Cardiomyopathy, end-stage congenital heart disease, status after biventricular repair

Synonyms: Two-ventricle repair

End-stage congenital heart disease in a patient with any prior repair in which two ventricles are in series.

Cardiomyopathy hierarchy level 4

Cardiomyopathy, NOS

Cardiomyopathy, Dilated, NOS

Cardiomyopathy, Dilated, Primary, NOS

Cardiomyopathy, Dilated, Primary, Idiopathic

Cardiomyopathy, Dilated, Secondary, NOS

Cardiomyopathy, Dilated, Secondary, Infectious

Cardiomyopathy, Dilated, Secondary, Metabolic

Cardiomyopathy, Dilated, Secondary, General systemic

Cardiomyopathy, Dilated, Secondary, Genetic

Cardiomyopathy, Dilated, Secondary, Ischemic

Cardiomyopathy, Dilated, Secondary, Toxic

Cardiomyopathy, Dilated, Secondary, Nutritional

Cardiomyopathy, Hypertrophic, NOS

Cardiomyopathy, Hypertrophic, Obstructive, NOS

Cardiomyopathy, Hypertrophic, Obstructive, Primary

Cardiomyopathy, Hypertrophic, Obstructive, Secondary

Cardiomyopathy, Hypertrophic, Nonobstructive, NOS

Cardiomyopathy, Hypertrophic, Nonobstructive, Primary

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary

Cardiomyopathy, Restrictive, NOS

Cardiomyopathy, Restrictive, Myocardial, NOS^*

Cardiomyopathy, Restrictive, Myocardial, Idiopathic

Cardiomyopathy, Restrictive, Myocardial, Amyloidosis

Cardiomyopathy, Restrictive, Myocardial, Gaucher disease

Cardiomyopathy, Restrictive, Myocardial, Glycogen storage disease

Cardiomyopathy, Restrictive, Myocardial, Hemochromatosis

Cardiomyopathy, Restrictive, Myocardial, Hurler disease

Cardiomyopathy, Restrictive, Myocardial, Sarcoidosis

Cardiomyopathy, Restrictive, Myocardial, Scleroderma

Cardiomyopathy, Restrictive, Endomyocardial, NOS

Cardiomyopathy, Restrictive, Endomyocardial, Anthracycline toxicity

Cardiomyopathy, Restrictive, Endomyocardial, Carcinoid

Cardiomyopathy, Restrictive, Endomyocardial, Endomyocardial fibrosis

Cardiomyopathy, Restrictive, Endomyocardial, Hypereosinophilic syndrome

Cardiomyopathy, Restrictive, Endomyocardial, Pseudoxanthoma elasticum

Cardiomyopathy, Restrictive, Endomyocardial, Radiation

Cardiomyopathy, Right ventricular, NOS

Cardiomyopathy, Right ventricular, Arrhythmogenic Right ventricular dysplasia

Cardiomyopathy, Right ventricular, Uhl’s syndrome

Cardiomyopathy, Right ventricular, Spongiform cardiomyopathy

Cardiomyopathy, End-stage congenital heart disease, NOS

Cardiomyopathy, End-stage congenital heart disease, Status after no prior cardiac surgery

Cardiomyopathy, End-stage congenital heart disease, Status after palliative procedure(s)

Cardiomyopathy, End-stage congenital heart disease, Status after single ventricle repair

Cardiomyopathy, End-stage congenital heart disease, Status after biventricular repair, NOS

Cardiomyopathy, End-stage congenital heart disease, Status after biventricular repair, With an extracardiac conduit

Cardiomyopathy, End-stage congenital heart disease, Status after biventricular repair, Without an extracardiac conduit

Cardiomyopathy hierarchy level 4 definitions

Cardiomyopathy, end-stage congenital heart disease, status after biventricular repair, with an extracardiac conduit: Any repair in which two ventricles are in series and a valved conduit was used (example: truncus arteriosus repair).

Cardiomyopathy, end-stage congenital heart disease, status after biventricular repair, without an extracardiac conduit: Any repair in which two ventricles are in series and in which a valved conduit was not used (example: arterial switch).

Cardiomyopathy hierarchy level 5

Cardiomyopathy, NOS

Cardiomyopathy, Dilated, NOS

Cardiomyopathy, Dilated, Primary, NOS

Cardiomyopathy, Dilated, Primary, Idiopathic

Cardiomyopathy, Dilated, Secondary, NOS

Cardiomyopathy, Dilated, Secondary, Infectious, NOS

Cardiomyopathy, Dilated, Secondary, Infectious, Viral, NOS

Cardiomyopathy, Dilated, Secondary, Infectious, Viral, Coxsackievirus B and A

Cardiomyopathy, Dilated, Secondary, Infectious, Viral, Echovirus

Cardiomyopathy, Dilated, Secondary, Infectious, Viral, Adenovirus

Cardiomyopathy, Dilated, Secondary, Infectious, Viral, Rubella

Cardiomyopathy, Dilated, Secondary, Infectious, Bacterial, NOS

Cardiomyopathy, Dilated, Secondary, Infectious, Bacterial, Diphtheria

Cardiomyopathy, Dilated, Secondary, Infectious, Bacterial, Pneumococcal

Cardiomyopathy, Dilated, Secondary, Infectious, Fungal, NOS

Cardiomyopathy, Dilated, Secondary, Infectious, Fungal, Candidiasis

Cardiomyopathy, Dilated, Secondary, Infectious, Fungal, Aspergillosis

Cardiomyopathy, Dilated, Secondary, Infectious, Protozoal, NOS

Cardiomyopathy, Dilated, Secondary, Infectious, Protozoal, American trypanosomiasis (Chagas disease)

Cardiomyopathy, Dilated, Secondary, Infectious, Protozoal, Toxoplasmosis

Cardiomyopathy, Dilated, Secondary, Infectious, Spirochetal, NOS

Cardiomyopathy, Dilated, Secondary, Infectious, Spirochetal, Lyme disease

Cardiomyopathy, Dilated, Secondary, Metabolic, NOS

Cardiomyopathy, Dilated, Secondary, Metabolic, Endocrine, NOS

Cardiomyopathy, Dilated, Secondary, Metabolic, Endocrine, Thyrotoxicosis

Cardiomyopathy, Dilated, Secondary, Metabolic, Endocrine, Hypothyroidism

Cardiomyopathy, Dilated, Secondary, Metabolic, Catecholamine cardiomyopathy

Cardiomyopathy, Dilated, Secondary, General systemic, NOS

Cardiomyopathy, Dilated, Secondary, General systemic, Connective tissue disorders, NOS

Cardiomyopathy, Dilated, Secondary, General systemic, Connective tissue disorders, Systemic lupus

Cardiomyopathy, Dilated, Secondary, General systemic, Connective tissue disorders, Juvenile rheumatoid arthritis

Cardiomyopathy, Dilated, Secondary, General systemic, Connective tissue disorders, Polyarteritis nodosa

Cardiomyopathy, Dilated, Secondary, General systemic, Connective tissue disorders, Kawasaki disease

Cardiomyopathy, Dilated, Secondary, General systemic, Connective tissue disorders, Pseudoxanthoma elasticum

Cardiomyopathy, Dilated, Secondary, General systemic, Nonconnective tissue disorders, NOS

Cardiomyopathy, Dilated, Secondary, General systemic, Nonconnective tissue disorders, Peripartum cardiomyopathy

Cardiomyopathy, Dilated, Secondary, General systemic, Nonconnective tissue disorders, Osteogenesis imperfecta

Cardiomyopathy, Dilated, Secondary, Genetic, NOS

Cardiomyopathy, Dilated, Secondary, Gentic, Muscular dystrophies and myopathies, NOS

Cardiomyopathy, Dilated, Secondary, Genetic, Muscular dystrophies and myopathies, juvenile progressive (Duchenne)

Cardiomyopathy, Dilated, Secondary, Genetic, Muscular dystrophies and myopathies, myotonic dystrophy (Steinert)

Cardiomyopathy, Dilated, Secondary, Genetic, Muscular dystrophies and myopathies, limb-girdle (Erb)

Cardiomyopathy, Dilated, Secondary, Genetic, Muscular dystrophies and myopathies, cardioskeletal myopathy (Barth syndrome)

Cardiomyopathy, Dilated, Secondary, Genetic, Muscular dystrophies and myopathies, X-linked cardiomyopathy

Cardiomyopathy, Dilated, Secondary, Genetic, Muscular dystrophies and myopathies, juvenile progressive spinal muscular atrophy (Kugelberg-Welander)

Cardiomyopathy, Dilated, Secondary, Ischemic, NOS

Cardiomyopathy, Dilated, Secondary, Ischemic, Congenital coronary artery malformation

Cardiomyopathy, Dilated, Secondary, Ischemic, Familial hypercholesterolemia

Cardiomyopathy, Dilated, Secondary, Ischemic, Post vasculitis (Kawasaki)

Cardiomyopathy, Dilated, Secondary, Toxic, NOS

Cardiomyopathy, Dilated, Secondary, Toxic, Sulfonamides, NOS

Cardiomyopathy, Dilated, Secondary, Toxic, Sulfonamides, Penicillin

Cardiomyopathy, Dilated, Secondary, Toxic, Anthracyclines

Cardiomyopathy, Dilated, Secondary, Toxic, Iron (hemachromatosis)

Cardiomyopathy, Dilated, Secondary, Toxic, Chloramphenicol

Cardiomyopathy, Dilated, Secondary, Nutritional, NOS

Cardiomyopathy, Dilated, Secondary, Nutritional, Kwashiorkor

Cardiomyopathy, Dilated, Secondary, Nutritional, Beriberi

Cardiomyopathy, Hypertrophic, NOS

Cardiomyopathy, Hypertrophic, Obstructive, NOS

Cardiomyopathy, Hypertrophic, Obstructive, Primary

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, NOS

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, Metabolic, NOS

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, Metabolic, NOS

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, Metabolic, carnitine deficiency

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, Metabolic, Debrancher enzyme deficiency (GSD III)

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, Metabolic, Fabry disease

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, Metabolic, Fucosidosis, type I

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, Metabolic, Hunter syndrome

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, Metabolic, Hurler-Scheie syndrome

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, Metabolic, Hurler syndrome

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, Metabolic, I-cell disease

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, Metabolic, Infant of a diabetic mother (IDM)

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, Metabolic, Mannosidosis

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, Metabolic, Phosphorylase b kinase deficiency

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, Metabolic, Pompe disease (GSD II)

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, Metabolic, Selenium deficiency

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, NOS

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, Beckwith-Wiedemann syndrome

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, cardio-facial-cutaneous syndrome

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, Friedreich ataxia

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, LEOPARD syndrome

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, Noonan syndrome

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, mitochondrial myopathy, NOS

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, mitochondrial myopathy, Cardiomyopathy with cataracts

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, mitochondrial myopathy, fatal infantile cardiomyopathy

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, mitochondrial myopathy, Histiocytoid cardiomyopathy, NOS

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, mitochondrial myopathy, Histiocytoid cardiomyopathy, Cytochrome b deficiency

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, mitochondrial myopathy, Histiocytoid cardiomyopathy, Cytochrome aa3 deficiency

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, mitochondrial myopathy, Histiocytoid cardiomyopathy, Cytochrome c-reductase deficiency

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, Mitochondrial myopathy, Leigh syndrome

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, mitochondrial myopathy, Maternally inherited myopathy and cardiomyopathy

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, mitochondrial myopathy, MELAS

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, mitochondrial myopathy, MERRF

Cardiomyopathy, Hypertrophic, Obstructive, Secondary, HCM associated with syndromes, Mitochondrial myopathy, NADH-coenzyme Q reductase deficiency

Cardiomyopathy, Hypertrophic, Nonobstructive, NOS

Cardiomyopathy, Hypertrophic, Nonobstructive, Primary

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, NOS

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, Metabolic, NOS

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, Metabolic, Carnitine deficiency

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, Metabolic, Debrancher enzyme deficiency (GSD III)

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, Metabolic, Fabry disease

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, Metabolic, Fucosidosis, type I

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, Metabolic, Hunter syndrome

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, Metabolic, Hurler-Scheie syndrome

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, Metabolic, Hurler syndrome

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, Metabolic, I-cell disease

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, Metabolic, Infant of a diabetic mother (IDM)

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, Metabolic, Mannosidosis

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, Metabolic, Phosphorylase b kinase deficiency

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, Metabolic, Pompe disease (GSD II)

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, Metabolic, Selenium deficiency

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, NOS

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Beckwith-Wiedemann syndrome

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Cardio-facial-cutaneous syndrome

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Friedreich ataxia

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, LEOPARD syndrome

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Noonan syndrome

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Mitochondrial myopathy, NOS

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Mitochondrial myopathy, cardiomyopathy with cataracts

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Mitochondrial myopathy, Fatal infantile cardiomyopathy

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Mitochondrial myopathy, Histiocytoid cardiomyopathy, NOS

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Mitochondrial myopathy, Histiocytoid cardiomyopathy, Cytochrome b deficiency

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Mitrochondrial myopathy, Histiocytoid cardiomyopathy, Cytochrome aa3 deficiency

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Mitochondrial myopathy, Histiocytoid cardiomyopathy, Cytochrome c-reductase deficiency

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Mitochondrial myopathy, Leigh syndrome

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Mitochondrial myopathy, Maternally inherited myopathy and cardiomyopathy

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Mitochondrial myopathy, MELAS

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Mitochondrial myopathy, MERRF

Cardiomyopathy, Hypertrophic, Nonobstructive, Secondary, HCM associated with syndromes, Mitochondrial myopathy, NADH-coenzyme Q reductase deficiency

Cardiomyopathy, Restrictive, NOS

Cardiomyopathy, Restrictive, Myocardial, NOS*

Cardiomyopathy, Restrictive, Myocardial, Idiopathic

Cardiomyopathy, Restrictive, Myocardial, Amyloidosis

Cardiomyopathy, Restrictive, Myocardial, Gaucher disease

Cardiomyopathy, Restrictive, Myocardial, Glycogen storage disease

Cardiomyopathy, Restrictive, Myocardial, Hemochromatosis

Cardiomyopathy, Restrictive, Myocardial, Hurler disease

Cardiomyopathy, Restrictive, Myocardial, Sarcoidosis

Cardiomyopathy, Restrictive, Myocardial, Scleroderma

Cardiomyopathy, Restrictive, Endomyocardial, NOS

Cardiomyopathy, Restrictive, Endomyocardial, Anthracycline toxicity

Cardiomyopathy, Restrictive, Endomyocardial, Carcinoid

Cardiomyopathy, Restrictive, Endomyocardial, Endomyocardial fibrosis

Cardiomyopathy, Restrictive, Endomyocardial, Hypereosinophilic syndrome

Cardiomyopathy, Restrictive, Endomyocardial, Pseudoxanthoma elasticum

Cardiomyopathy, Restrictive, Endomyocardial, Radiation

Cardiomyopathy, Right ventricular, NOS

Cardiomyopathy, Right ventricular, Arrhythmogenic right ventricular dysplasia

Cardiomyopathy, Right ventricular, Uhl’s syndrome

Cardiomyopathy, Right ventricular, Spongiform cardiomyopathy

Cardiomyopathy, End-stage congenital heart disease, NOS

Cardiomyopathy, End-stage congenital heart disease, Status after no prior cardiac surgery

Cardiomyopathy, End-stage congenital heart disease, Status after palliative procedure(s)

Cardiomyopathy, End-stage congenital heart disease, Status after single ventricle repair

Cardiomyopathy, End-stage congenital heart disease, Status after biventricular repair, NOS

Cardiomyopathy, End-stage congenital heart disease, Status after biventricular repair, With an extracardiac conduit

Cardiomyopathy, End-stage congenital heart disease, Status after biventricular repair, Without an extracardiac conduit

Hierarchy level 5 definitions

MELAS: An acronym for mitochrondrial myopathy, encephalopathy, lactacidosis, and stroke.

MERRF: Mitochrondial epilepsy with ragged red muscle fibers.

NADH: Nicotinamide adenine dinucleotide.

III. Nomenclature for cardiomyopathies treatment options

Cardiomyopathy treatment hierarchy level 1

Dilated cardiomyopathy

Transplant, heart

Permanent cardiac assist device

Temporary cardiac assist device

Partial left ventriculectomy (LV volume reduction surgery) (Batista procedure)

Dynamic cardiomyoplasty

Hypertrophic cardiomyopathy

Transplant, Heart

Myotomy, Left ventricle

Myomectomy, Left ventricle

Mitral valve replacement

Valve-sparing Konno procedure

DDD pacemaker

Restrictive cardiomyopathy

Transplant, Heart

Permanent cardiac assist device

Temporary cardiac assist device

Right ventricular cardiomyopathy

Transplant, Heart

Automatic implantable cardioversion device (AICD)

Fontan procedure

Arrhythmia ablation

End-stage congenital heart disease

Transplant, Heart

Transplant, Heart and lung(s)

Permanent cardiac assist device

Temporary cardiac assist device

Dynamic cardiomyoplasty

Arrhythmia ablation

Cardiomyopathy treatment hierarchy level 2

Transplant, Heart

Transplant, Heart, NOS

Transplant, Heart, Orthotopic: allograft

Transplant, Heart, Orthotopic: xenograft

Transplant, Heart, Heterotopic: allograft

Transplant, Heart, Heterotopic: xenograft

Permanent cardiac assist device

Total artificial heart

Left ventricular assist device

Right ventricular assist device

Biventricular ventricular assist device

Temporary cardiac assist device

Extracorporeal membrane oxygenation (ECMO)

Left ventricular assist device

Right ventricular assist device

Biventricular assist device

Extracorporeal membrane oxygenation (ECMO): transplant, heart

Left ventricular assist device: transplant, heart

Right ventricular assist device: transplant, heart

Biventricular assist device: transplant, heart

Partial left ventriculectomy (LV volume reduction surgery) (Batista procedure): no further subdivision

Dynamic cardiomyoplasty: no further subdivision

Myotomy, left ventricle: no further subdivision

Myomectomy, left ventricle: no further subdivision

Mitral valve replacement: see the "Diseases of the Mitral Valve" article in this publication

Valve spring Konno procedure: see the "Diseases of the Aortic Valve" article in this publication

Pacemaker implantation, Permanent, Transvenous, Atrial and ventricular leads, DDD

Pacemaker implantation, Permanent, Transvenous, Atrial and ventricular leads, DDDR

AICD: see the "Arrhythmias" article in this publication

Fontan procedure: see the "Single Ventricle" article in this publication

Arrhythmia ablation: see the "Arrhythmias" and "Therapeutic Cardiac Catheter Interventions" articles in this publication

Transplant, Heart and lung(s): see the "End-Stage Lung Disease" article in this publication

Additional comment on therapeutic options
To be complete, designations regarding allograft versus xenograft and orthotopic versus heterotopic are considered. However, the technology for xenografting may not be imminent at this point in time, and the number of transplants currently placed in a heterotopic position is vanishingly small.

IV. Diagnosis and procedure short lists

Diagnosis Short List

Cardiomyopathy

PROCEDURE SHORT LIST

Transplant, Heart

Transplant, Heart and Lung

Partial left ventriculectomy (LV volume reduction surgery) (Batista)

Valve replacement, Mitral (MVR)

Konno procedure

Pacemaker implantation, Permanent

Pacemaker procedure

ICD (AICD) implantation

ICD (AICD) ([automatic] implantable cardioverter defibrillator) procedure

Arrhythmia surgery - atrial, Surgical ablation

Arrhythmia surgery - ventricular surgical ablation

Fontan, Atrio-pulmonary connection

Fontan, Atrio-ventricular connection

Fontan, TCPC, Lateral tunnel, Fenestrated

Fontan, TCPC, Lateral tunnel, Nonfenestrated

Fontan, TCPC, Lateral tunnel, NOS

Fontan, TCPC, External conduit, Fenestrated

Fontan, TCPC, External conduit, Nonfenestrated

Fontan, TCPC, External conduit, NOS

Fontan, Other

Fontan, NOS

V. Potential diagnostic related risk factors

Preoperative risk variables
Demographic data

Age at surgery

Weight at surgery

Gender

Race

Blood type

Preoperative risk factors

Preoperative pulmonary vascular resistance

Preoperative left ventricular end diastolic pressure

Preoperative wedge pressure

Preoperative left atrial pressure

Preoperative right atrial pressure

Preoperative pressure gradient across left ventricular outflow tract

Preoperative ejection fraction

Preoperative shortening fraction

Preoperative ECMO

Preoperative mechanical ventricular assist

Preoperative inotropes

Preoperative nitric oxide

Preoperative mechanical ventilation

Preoperative medications (diuretics, digoxin, antiarrhythmics, afterload reduction agents)

Noncardiac anomalies

Associated cardiac anomalies (listed separately)

Waiting time on transplant list

Intraoperative risk variables

Donor graft ischemic time

Donor blood type

Donor cause of death

Donor weight

Graft preservation solution

Distance to donor

Prior sternotomy

Cardiopulmonary bypass time

Aortic cross-clamp time

Cardioplegia type

Circulatory arrest time

Modified ultrafiltration use

Heparin-coated bypass circuit

Postoperative risk variables

Early graft failure

Definition: Cardiac failure within 1 month after transplant not attributed to rejection or surgical technical problems.

Infection

Rejection

Immunosuppressive regimen

Induction

Maintenance

Postoperative mechanical support

VAD

ECMO

IABP

Postoperative nitric oxide

Length of ventilation

Length of intensive care unit (ICU) stay

Length of hospital stay

Blood products required

Residual left ventricular outflow gradient

VI. Database studies and outcome analysis

Potential heart transplant data set
Demographics data fields

Name

Member number

Date of birth

Medical record number

Gender

Race

Blood type

Age at surgery

Weight at surgery

Body surface area at surgery

Preoperative data fields

Preoperative pulmonary vascular resistance

Preoperative left ventricular end diastolic pressure

Preoperative wedge pressure

Preoperative left atrial pressure

Preoperative right atrial pressure

Preoperative pressure gradient across left ventricular outflow tract

Preoperative ejection fraction

Preoperative shortening fraction

Preoperative ECMO

Preoperative mechanical ventricular assist

Preoperative inotropes

Preoperative nitric oxide

Preoperative mechanical ventilation

Preoperative medications (diuretics, digoxin, antiarrhythmics, afterload reduction agents)

Noncardiac anomalies

Associated cardiac anomalies (listed separately)

Waiting time on transplant list

Operative data fields

Admit date

Discharge date

Surgery date

Status

Elective

Urgent (no discharge without surgery)

Emergent

Salvage

ASA classification

Prior sternotomy (yes or no)

Noncardiac abnormalities (yes or no)

Diagnosis (ICD-9)

Procedure (CPT)

Surgeon

Assistant

Cardiopulmonary bypass time

Aortic cross-clamp time

Cardioplegia type

Circulatory arrest time

Modified ultrafiltration use

Heparin-coated bypass circuit

Donor graft ischemic time

Donor blood type

Donor cause of death

Donor weight

Donor body surface area

Graft preservation solution

Distance to donor

Postoperative data fields

Early graft failure

Infection: yes or no

Date infection identified

Organism causing infection

Site of infection

Rejection: yes or no

Date of rejection episode

Hyperacute, acute, or chronic

Grade of infection

Immunosuppressive regimen

Induction

Maintenance

Postoperative mechanical support

VAD

ECMO

IABP

Postoperative nitric oxide

Length of ventilation

Length of ICU stay

Length of hospital stay

Blood products required

Residual left ventricular outflow gradient

Other complications: yes or no

Specific complications with ICD-9 codes

Complication dates

Mortality: yes or no

Mortality: in operating theater

Mortality: before discharge

Mortality: after discharge

Mortality: < 30 days

Mortality: 30 days

Cause of death

Footnotes

^* Modified from: Wynne J, Braunwald E. The cardiomyopathies and myocarditides: toxic, chemical and physical damage to the heart. In: Braunwald E, ed. Heart disease, 4th ed, Philadelphia: WB Saunders Company, 1992:1415.

Modified from: Denfield SW, Gajarski RJ, Towbin JA. Cardiomyopathies. In: Garson, Jr A, Bricker JT, Fisher DJ, Neish SR, eds. The science and practice of pediatric cardiology, 2nd ed, Baltimore: Williams & Wilkins, 1998:1852.

Modified from: Denfield SW, Gajarski RJ, Towbin JA. Cardiomyopathies. In: Garson, Jr A, Bricker JT, Fisher DJ, Neish SR, eds. The science and practice of pediatric cardiology, 2nd ed, Baltimore: Williams & Wilkins, 1998:1868.

References

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2. World Health Organization. Report of the WHO/IFSC task force on the definition and classification of cardiac myopathies. Br Heart J 1980;44:672-673.[Free Full Text]
3. Wynne J., Braunwald E. The cardiomyopathies and myocarditides. In: Braunwald E., ed. Heart disease, 4th ed Philadelphia: WB Saunders Company, 1992:1394-1450.
4. Goodwin J.F., Oakley C.M. The cardiomyopathies. Br Heart J 1972;34:545-549.[Free Full Text]
5. Goodwin J.F. Overview and classification of the cardiomyopathies. In: Shaver J.A., ed. Cardiomyopathies. Philadelphia: FA Davis, 1998:3-7.
6. Thiene G., Nava A., Corrado D., et al. Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med 1988;318:129-133.[Abstract]
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