Paper # 008

Versión en Español

Purkinje cell hamartoma (Histiocytoid cardiomyopathy). Study of a case in an 18 months infant.

Marcial Garcia-Rojo, Carlos Gamallo,Felipe Moreno

[Title] [Introduction] [Materials & Methods] [Results] [Pictures] [Discussion] [Bibliography]

[Definition] [Clinical] [Microscopy] [Techniques] [Other findings] [Interpretation]

LITERATURE REVIEW. Anatomic Pathology

Pathological studies performed in Purkinje hamartoma are autopsies and endo-myocardial biopsies^(8,9,21,32). There are some reports about biopsies of skeletal muscle, that normally show no abnormality⁽¹⁵⁾.

At autopsies, they are usually well developed, well-nourished^(4,5), exept in some rare cases⁽⁴⁾.

Cultures aure usually negatives. Postmortem sections of right and left ventricles and of mesenteric lymph nodes showed no growth in viral studies(4). No viruses were isolated from postportem blood, myocardium, kidney, lung, liver and spleen(2).

Postmortem kidney was screened for heavy metals (cadmium, cobalt, chromium, copper, iron, nickel, tin, zinc, lead). Dithizone reaction for mercury was performed on postmortem liver. All values were within accepted normal ranges.

Heart:

Gross findings:

• Normal^(2,3,4,16).
• Hypertrophy^{(1,3-5,8,12,14,15,20,22,23,25-27,30,32)}. Biventricular, more pronounced in left ventricle^(14,22).
• Left ventricle dilatation^(3,4,5).
• The heart was soft, flabby⁽³⁾.

Yellowish-brown, ill defined, multifocal plaques os small nodules are found at endocardium, subendocadium or myocardium⁽¹⁹⁾.

Valves:

1.- Yellowish-brown, small nodules on (or near) cardiac valves^(3,7,11,13), ocasionaly on all 4 cardiac valves⁽¹³⁾ but generally on:

Mitral valve (2-4 mm)

From the base of the mitral valve towards the medial junction of the cusps⁽¹¹⁾.

Posterior mitral leaflet^(7,12,20), sometines extending onto the inferior surface of this leaflet and laterally to the aortic valve⁽²⁰⁾.

Auricular side ⁽³⁰⁾.

In the base of the anterior cusp were several small yellowish-brown plaque-like thickening visible on both surfaces and extending into the adjacent endocardium at the base of the septum, just reaching the base of the right coronary cusp of the aortic valve⁽¹¹⁾.

A multilobulated nodule(4x5mm) adjacent to the membranous portion of the ventricular septum near the base of the noncoronary cusp of the aortic valve⁽³⁾.

One patient had several plaques and small nodules, 1 mm in diameter, below the annulus of the pulmonic valve⁽³⁾.

Tricúspide:

• Several plaques and small nodules, 1 mm in diameter, along the annulus of the tricuspid valve 5 mm above its closing margin⁽³⁾.
• A nodule in the right atrium, just above the septal leaflet of the tricuspid valve⁽⁷⁾.

2.- Slight thickening of the mitral valve^(3,11,20).

3.- The cardiac valves may be unremarkable⁽¹³⁾.

 

Miocardio:

Areas of yellow-white discoloration of myocardium or gray-whitish areas that affected left ventricular subendocardium ^{(3,8,13,14,16,26,27)}.

Affecting:

• Papillary muscles(^(8,12,16).
• Papillary insertions of chordae tendinae^(8,12,13,16).
• Basal part of the interventricular septum⁽¹²⁾.
• left ventricular septum^(14,20,27).
• Interatrial septum ⁽¹⁴⁾.
• Posterior left atrium⁽¹⁴⁾.
• Right chambers^{(8,12-14,27,30)}.
• Left ventricular epicadium, with confluent groups of tiny(up to 0,3 cm) nodules^{(8,9,12,14,27)}.
• Severe, extensive yellow-white discoloration of the left ventricular subendocardium that only a few, small focal areas retain their normal color⁽³⁾.
• All chambers^(12,35).

Myocardium may appear pale, but firm⁽¹¹⁾, with no gross lesion^(4,11,24) except hypertrophy⁽²²⁾ .