Histiocytosis X Cor Pulmonale

Report:

Sinus tachycardia 102/min

Right atrial abnormality

Left atrial abnormality

Right axis deviation +110o

RSR’S’ in V1

Poor R wave progression

Right ventricular hypertrophy

Comment:

Most patients with a trace like this would have emphysema, but cardiac response to pulmonary hypertension of any cause is of course quite stereotyped. Histiocytosis X often presents with a dramatic CXR in still asymptomatic patients, but pulmonary fibrosis and lung destruction follow.

The LAA here (large PTF in V1) may be – and probably is – an expression of right atrial enlargement.

Below (Fig 45a) is a tracing taken two months later (still waiting for the heart-lung transplant), looking unchanged apart from the unusually prominent P waves in V4-6.

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