Download Ebstein TypeB TV case - ASA 2013

A case report: Anesthesia management of an Ebstein Type B tricuspid valve malformation in an Adult
Mical S. Duvall MD (1) , Pushpa Koyyalamudi MD (1), Lucas M. Duvall MD (2)
1. Louisiana State University Health Sciences Center, Department of Anesthesiology , Shreveport, LA
2. Louisiana State University Health Sciences Center, Department of Surgery, Division of Cardiothoracic Surgery, Shreveport, LA
Intervention/Treatment
Modality
Introduction/Purpose
Conclusions
.
Ebstein’s anomaly (EA) occurs in 1:210,000 live
births and accounts for less than 1% of all
congenital heart disease. It is a malformation of
the Tricuspid valve (TV) associated with a wide
spectrum of abnormalities often involving the
right atrium and ventricle (1). We present a case
of anesthetic management during tricuspid valve
replacement, of a patient with an Epstein type B
malformation.
Patient Description
A 58 year-old gentleman with history of
hypertension, paroxysmal atrial fibrillation,
ascites, jugular venous distension and severe
Tricuspid regurgitation (TR), presented for
Tricuspid
valve
replacement.
The
Electrocardiogram showed sinus bradycardia
with first degree AV block. Following placement
of a radial artery pressure-monitoring cannula,
anesthesia was induced with midazolam and
sufenanil. Central venous access was obtained
and pulmonary artery catheter placement was
successful without any evidence of arrhythmia.
Transesophageal
echocardiogram
(TEE)
revealed apical displacement of septal leaflet of
TV with severe TR, severely dilated right atrium
and right ventricle. Interventricular septum
displayed diastolic bowing toward left ventricle
with severely reduced left ventricular end
diastolic volume and a normal EF. There was no
evidence of Atrial septal defect . Initial CVP of 20
mmHg and normal Pulmonary artery pressures
(PAP) were noted. No complications were
observed in the pre-bypass period.
1. Ebstein’s Anamoly is a rare congenital heart
disease involving the Tricuspid valve with Tricuspid
regurgitation and variable degree of Right sided
chamber enlargement and dysfunction
2. The goals of Anesthetic management of these
patients should include interventions to improve right
ventricular contractility and avoid increases in RV
afterload.
3. Placement of PA catheter can be technically
difficult and care should be taken to avoid
arrhythmias.
4. CVP is typically elevated in these patients and
peri-operative fluid management can be challenging.
5. TEE can accurately identify the lesion, its severity
and associated cardiac defects. Intra-operative TEE
is a valuable tool to help guide intravenous fluid
therapy and pharmacologic
interventions by
assessing the right and left ventricular preload and
changes in ventricular function.
Surgical findings included a partial delamination
defect of septal leaflet and some atrialization of
Right Ventricle. Tricuspid valve was replaced
with a
bio-prosthetic valve and reduction
atrialoplasty was done.
Separation from cardiopulmonary bypass was
accomplished with minimal use of inotropes
(Epinephrine 0.04 mcg/kg/min) and vasodilators
(Nitroglycerin 2-3 mcg/kg/min). CVP decreased
to 7–10 mmHg with a mean PAP of 15-20
mmHg. Patient required AV pacing at 80
beats/min for junctional bradycardia. TEE
revealed a normal functioning Tricuspid valve
Bio-prosthesis with no residual regurgitation.
The right ventricle remained dilated with
improved function. The patient was extubated
on the first postoperative day. He subsequently
required permanent pacemaker for persistant
complete heart block.
Discussion
The clinical manifestations of Ebstein’s anamoly
in the adult depend on several factors, including
the extent of TV leaflet distortion, degree of TR,
right atrial pressure, and presence of a right to
left atrial shunt. Echocardiography can
accurately identify the lesion, its severity and
associated cardiac defects (2). The anesthetic
management of patient with EA should include
interventions to improve right ventricular
contractility and decrease afterload. TEE can
give real time information as to the influence of
pharmaceutical interventions on ventricular
function and in patients with elevated CVP helps
guide intravenous fluid therapy by assessing the
right and left ventricular preload.
References
TEE 4-chamber view with severe enlargement of Right atrium and Right ventricle. Dilated Tricuspid valve Annulus and Apical
Displacement of Septal leaflet is seen.
1. Ann Thoracic Surgery 2000; 69: 106-117
2. Current Treatment Options in Cardiovascular Medicine
(2012) 14:594–607
Acknowledgments/Conflict of Interest
The authors would like to thank the University as well as the
Departments of Anesthesiology and Surgery for the time
and funding necessary to see this study through to
completion.
The Authors have no current Conflicts of Interest.
,
TEE ME RV inflow-outflow view showing severe Tricuspid Regurgitation