Turnerâs Syndrome
Report:
Sinus rhythm 91/min
Right atrial abnormality, P congenitale type
Right axis deviation +140o
Right ventricular hypertrophy
Diffuse T wave changes
Comment:
Women with Turnerâs syndrome have a range of ECG abnormalities, including T wave changes, QT prolongation, accelerated conduction and RAD. The last appears particularly associated with anomalous pulmonary venous connection, usually to left-sided SVC50. Other ECG manifestations may be secondary to associated anomalies like bicuspid aortic valve, coarctation or (seemingly essential) hypertension.
This case, contrary to my expectation, had no shunts. She was cyanosed from chronic hypoventilation, with chronic cor pulmonale and RVH. The T wave changes may be reflecting more than RVH alone. And yet, I am not sure the clinicians have got it right: I have never seen a patient with this degree of RVH and P congenitale from presumably central hypoventilation. Her shunt (obviously leading to Eisenmenger situation) may be quite elusive.
63. 24 year old man with recent syncopal episodes, about to be discharged home and die
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