How to manage the Eisenmenger syndrome

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Abstract

Without an understanding of the physiology of the Eisenmenger state (complex, syndrome or reaction) the management and outcomes of such cases will not be optimal. This is a problem for many patients scattered as occasional curiosities in practices and non-specialised clinics. The patient group with Eisenmenger reaction can lead adequate though symptomatic lives until late middle age or longer, but survival beyond age 50 years demands optimum care and attention from informed cardiologists. The cardiologist in charge should advise on lifestyle and all therapy, particularly for other systems, prevent disaster and educate patient and profession to keep the patient alive.

Introduction

Without an understanding of the physiology of the Eisenmenger state (complex, syndrome or reaction) the management and outcomes will not be optimal. This is a problem for many patients scattered as occasional curiosities in practices and non-specialised clinics.

Wood's definition [1]is the best: “...Pulmonary hypertension at systemic level due to high pulmonary vascular resistance with reversed or bi-directional shunt...”. He commented that “...it matters very little where the shunt happens to be...”. The term “Eisenmenger reaction” is applied to the whole group of patients and associated with various structural congenital cardiac defects. The Eisenmenger syndrome is used for the whole group. When the basic lesion is a ventricular septal defect, as Eisenmenger [2]described, the term Eisenmenger's complex is used.

Section snippets

Anatomy

The physiological state of high pulmonary vascular resistance with right-to-left shunt occurs with many cardiac defects (Fig. 1) with ventricular septal defect the most frequent; rare lesions may be associated with the reaction. Ventricular septal defect may coexist with coarctation, persistent duct, be part of double outlet right ventricle or atrioventricular canal and associated with straddling atrioventricular valves, aortic stenosis and even, occasionally, aorto-pulmonary window. Sometimes

Physiology

The presence of a large defect communicating at ventricular or aorto-pulmonary level means the systolic pulmonary artery and systemic arterial pressure are the same, as there is unrestricted communication between the circulations. When the pulmonary vascular resistance is low or lower, as in infancy and early childhood, the shunt is from left-to-right side. When the pulmonary vascular resistance rises the left-to-right shunt reduces until it becomes bi-directional then permanently

Medical responsibility

The physician/cardiologist caring for the patient with Eisenmenger's syndrome must accept specific responsibilities:

  • 1.

    Understand the underlying physiology and the effects of therapy on the cardiovascular system of patients with Eisenmenger's reaction (strange how many physicians have these patients in their care and do not fulfil this basic need!).

  • 2.

    Alleviate symptoms without adding risk.

  • 3.

    Counsel against special risks.

  • 4.

    Know the natural history.

  • 5.

    Prevent disasters and premature death

  • 6.

    Refer for

Conclusions

The patient group with Eisenmenger reaction can lead adequate though symptomatic lives until late middle age or longer. Survival beyond age 50 years demands optimum care and attention from informed cardiologists. Such patients should not be the occasional interesting patient in a cardiological practice nor for the paediatric cardiologist. The cardiologist in charge requires to “control” and advise on lifestyle and all therapy, particularly for other systems, prevent disaster and educate patient

References (2)

  • Wood, P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt (The Croonian Lectures). Br Med...
  • Eisenmenger, V. Congenital defects of the ventricular septum. Z Clin Med 1897; suppl...

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    All available data are retrospective, with no standardization regarding how hemoptysis is defined. Further, anticoagulation in cyanotic patients requires citrate adjustment for accurate international normalized ratio quantification (9), and improper monitoring has lead to warfarin-related deaths in Eisenmenger syndrome (24). Finally, patients with idiopathic pulmonary arterial hypertension are routinely anticoagulated, but are distinctly different from Eisenmenger patients (2,23,25,26).

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