Ebstein's anomaly

I recently had the opportunity to treat a patient with some very unusual and complex conditions during my cardiopulmonary placement. This patient had been admitted with pneumonia, but had an underlying history of several previous admissions for pneumonia (since he was seven, and is now in his 60's) and cardiological anomalies, one of which was Ebstein's anomaly.

Ebstein's anomaly relates to the tricuspid valve (between R atrium and ventricle) being congenitally displaced down towards the apex of the heart. This reduces the size of the ventricle and obviously relates to poor contractility and output from the R side of the heart. There can also be a patent foramen ovale creating shunt between L and R sides of the heart, as well as enlargement of the anterior leaflet of the tricuspid valve which affects regurgitation of blood into the atria. All of these lead to a raised JVP due to the decreased effectiveness of the R side of the heart. This patient definitely had a raised JVP. He also had peripheral vasoconstriction (to the point that his feet were purple and hypothermic) and extreme tachycardia. He also had shortness of breath, especially on exertion and fatigue, all of which is typical of a patient with Ebstein's.

I was treating this patient for his chest by using the ACBT in sitting. The auscultatory findings indicated greater secretion retention in the L lung, so after doing ACBT in sitting and reauscultating, I decided to treat him in R side ly. The patient lay on his L easily but rolling over gave us both a shock. He immediately groaned, closed his eyes and raised his hand over them, and began restlessly moving on his back. He said he felt 'bad' and had to sit up. In this time he became hypoxamic, increased tachycardia, very dark colouring in his face and exhibited dizziness and distress. I was very concerned and grabbed the nurse coordinating and his own nurse. He stabilised very quickly in his SpO2, alertness and became calm quite quickly once sitting up. We mutually agreed I should leave him to recover, while the nurses proceeded to use a "bear hugger" (warm air body bag) to reduce the hypothermia.

Well. Why did it happen? Cyanosis is a resulting problem for patients with Ebsteins, especially with a severely leaking valve. I have not been able to find any information as to why L side ly was the trigger and not R side lying. Could it be the pulmonary venous volume and pressure shift by the changed position from sitting to sidelying and rolling over? Could it be a poor orthostatic adjustment? I'm not sure but it was a critical situation for a moment and I would like to know more about the mechanics for this occurance and whether it is normal or not. If any one has any other ideas relating to the incident, I'd appreciate them. Perhaps there are better ways to treat such a patient that avoid this situation, or perhaps it was a unique one. Otherwise, I hope you learned something interesting about an interesting condition. Either way, however, for your patients with unstable conditions, where cardiological, pulmonary etc, it is very important to keep monitoring their vitals - things can change very quickly!!!!!