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ECHO ROUNDS

Transesophageal Echocardiography Images of Dynamic


Subpulmonic Left Ventricular Outflow Obstruction in an
Adult Patient After a Remote Senning Procedure for
d-Transposition of Great Arteries
Edward Gologorsky, MD, FASE,* Angela Gologorsky, MD, Marco Ricci, MD, PhD,
and Eliot R. Rosenkranz, MD

29-year-old man presented to the operating room


for laser-assisted removal and replacement of a fractured ventricular lead of an automatic implantable
cardioversion device (AICD). His medical history was significant for a Senning procedure for d-transposition of the great
arteries (d-TGA) at the age of 4 years, and placement of an
AICD for recurrent ventricular arrhythmias 4 years ago.
Subsequent to the AICD placement, the patient had remained
in good health, with some shortness of breath upon mild
physical exertion or excitement. His medications included
aspirin, enalapril, metoprolol, and digoxin. Informed consent
was obtained for this presentation.
Transesophageal echocardiography was requested by
the surgeon to monitor the development of a pericardial
effusion after removal of the old wire from the thinned left
ventricle. General anesthesia was induced with fentanyl,
etomidate, and rocuronium and maintained with isoflurane. Imaging of the midesophageal (ME) 4-chamber view
revealed a Senning baffle separating the pulmonary venous
and systemic atria (Video 1, see Supplemental Digital
Content 1, http://links.lww.com/AA/A121, Loop 1; see
Appendix for video caption). The systemic (morphologically right) ventricle was hypertrophied and dilated, with
mildly globally decreased systolic function. The pulmonic
(morphologically left) ventricle appeared thinned but functionally preserved (Video 1, http://links.lww.com/AA/A121,
Loop 2; see Appendix for video caption). Moderate tricuspid
insufficiency and mild mitral insufficiency were noted.
The imaging plane orthogonal to the ME 4-chamber
view (rotated to 101) demonstrated the aorta anterior and
parallel to the pulmonary artery. Whereas flow through the
aortic valve appeared normal, pulmonary artery flow was
turbulent (Video 1, http://links.lww.com/AA/A121, Loop
3; see Appendix for video caption).
Further rotation to the ME long-axis view revealed
systolic bulging of the basal segments of the interventricular septum into the left ventricular (pulmonary) outflow

tract (Video 2, see Supplemental Digital Content 2,


http://links.lww.com/AA/A122, Loop 4; see Appendix for
video caption), resulting in its narrowing and systolic flow
turbulence (Fig. 1) (Video 2, http://links.lww.com/AA/A122,
Loop 5; see Appendix for video caption). Aligning the
Doppler pulsed-wave beam along the narrowed left ventricular outflow tract, and sampling proximal to the pulmonic valve, revealed a peak subpulmonary dynamic gradient of 60 mm Hg (Fig. 2), consistent with a moderate
degree of pulmonary flow obstruction. Dexmedetomidine
infusion was initiated to prevent emergence excitement; a
normovolemic state was maintained and judicious small
doses of phenylephrine were used to prevent systemic
hypotension. The patient remained hemodynamically
stable through the entire perioperative period and was
discharged home the same day in satisfactory condition.

DISCUSSION
d-TGA is defined by atrioventricular concordance and
ventriculoarterial discordance. The aorta originates from
the right ventricle (RV), anterior and to the right of the
pulmonary artery, whereas the left ventricle drives
the pulmonary circulation, parallel to the systemic. In these
patients, survival depends on blood mixing between the
systemic and pulmonary flows; before the Senning procedure, the 1-year mortality rate for d-TGA approached 90%.
Atrial switch procedures redirect pulmonary venous return
through the tricuspid valve to the (systemic) RV and
systemic venous return through the mitral valve to the left
(pulmonary) ventricle (Fig. 3). In the Senning operation, the

From the *Department of Anesthesiology, Jackson Memorial Hospital/


University of Miami, Miller School of Medicine, Miami; Department of
Anesthesiology, Memorial Regional Hospital East, Hollywood; and Division of Cardiothoracic Surgery, University of Miami, Miller School of
Medicine/Jackson Memorial Hospital, Miami, Florida.
Accepted for publication February 6, 2010.
Supplemental digital content is available for this article. Direct URL citations
appear in the printed text and are provided in the HTML and PDF versions
of this article on the journals Web site (www.anesthesia-analgesia.org).
Address correspondence to Edward Gologorsky, MD, FASE, Department of
Anesthesiology, Jackson Memorial Hospital/University of Miami, Miller
School of Medicine, 1611 NW 12th Ave., C-300, Miami, FL 33136. Address
e-mail to egologorsky@med.maimi.edu.
Reprints will not be available from the author.
Copyright 2010 International Anesthesia Research Society
DOI: 10.1213/ANE.0b013e3181db7973

July 2010 Volume 111 Number 1

Figure 1. Turbulent subpulmonic flow in midesophageal long-axis


view. Left ventricular outflow tract is narrowed by bulging of basal
interventricular septal segments (wide arrow) toward the anterior
mitral valve leaflet (narrow arrow). RV right ventricle; PA
pulmonary artery.
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ECHO ROUNDS

Figure 2. Pulse-wave Doppler interrogation of the subpulmonic flow


reveals a moderately severe gradient close to 60 mm Hg.

pathway is created from a right atrial free wall flap and


the interatrial septum; the Mustard procedure involves the
creation of a pericardial baffle. Both are considered palliative for d-TGA, which is replaced by the definitive arterial
switch operations. Despite low perioperative mortality, the
long-term prognosis after atrial switch procedures may be
complicated by RV failure, sinus node dysfunction, tricuspid insufficiency, venous (both systemic and pulmonary)

obstruction, arrhythmias, and sudden death.1,3 RV dysfunction can progress insidiously in an asymptomatic patient to the point of failure.2
Echocardiographic assessment of RV function is performed in ME (rotating the imaging plane from the
4-chamber view to RV inflow-outflow to left ventricular
long axis) and transgastric views (short axis and RV
inflow). The aorta is usually visualized arising from the
RV anterior and to the right of the pulmonary artery. RV
morphology is characterized by the absence of the fibrous
continuity between the tricuspid and aortic valves (subaortic conus). The ME long-axis view allows for the appreciation of fibrous continuity between the anterior mitral valve
leaflet and the pulmonic valve, which may contribute to the
LV outflow tract obstruction by basal interventricular septal segments.
Left ventricular outflow (subpulmonary) obstruction
after atrial switch procedures is not rare.4 As seen in our
patient, right-to-left systolic bulging of the interventricular
septum can cause dynamic subpulmonic narrowing, limiting pulmonary flow and contributing to exercise/
excitement-induced dyspnea. The severity of the pulmonary flow obstruction, based on the peak systolic gradient,
is graded as trivial if 25 mm Hg, mild if 50 mm Hg.,
moderate if 50 to 79 mm Hg, and severe if 80 mm Hg.
Similar to hypertrophic obstructive cardiomyopathy,5

Figure 3. A, The systemic venous return is


routed via a Senning pathway through the
mitral valve into the left ventricle and further
into the pulmonary artery. B, The pulmonary
venous return is routed via a Senning pathway
through the tricuspid valve into the right ventricle and into the systemic circulation. C, A
schematic diagram of the blood flow after a
Senning procedure. RV right ventricle; LV
left ventricle; TV tricuspid valve; MV mitral
valve; IVC inferior vena cava; SVC superior
vena cava; PV pulmonary veins.

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ANESTHESIA & ANALGESIA

Dynamic Subpulmonic Obstruction in an Adult Patient After a Senning Procedure

medical interventions are directed to maintain left ventricular outflow patency by increasing left ventricular intracavitary pressures and decreasing septal systolic excursion.
The anesthetic plan includes avoidance of catecholamines,
inodilators and positive inotropic drugs, maintenance of
adequate afterload and euvolemia to sustain a slower heart
rate, and prevention of adrenergic stimulation and tachycardia. Surgical options may include pulmonary artery
banding to increase left ventricular afterload and intracavitary pressures with an eye toward decreasing septal
excursion and restoring left ventricular mass (in preparation for a double-switch procedure) and dual-chamber
sequential pacing in attempt to induce an asynchronous
septal contraction.
We hope that this presentation will alert anesthesiologists to this potentially challenging physiology in adult
patients after atrial switch procedures.

APPENDIX: VIDEO CAPTIONS


Video 1, Loop 1: 4-chamber view shows dilated hypertrophied hypocontractile right (systemic) ventricle, normally
functioning left (pulmonic) ventricle; visualized portion of the
Senning baffle appears intact; a wire is seen in the left atrium.
Video 1, Loop 2: short-axis view reveals dilated hypertrophied hypocontractile right (systemic) ventricle and normally functioning left (pulmonic) ventricle.
Video 1, Loop 3: the imaging plane is rotated to 101. An
automatic implantable cardioversion device (AICD) wire is
seen in the left atrium. Senning baffle separates pulmonary
and systemic atria. Aorta is visualized anterior to pulmonary artery, originating from the dilated hypertrophied
right ventricle. Absence of fibrous continuity between the
tricuspid and aortic valves is characteristic of the right
ventricular (RV) morphology. Color Doppler examination

Teaching Points

demonstrates normally functioning aortic valve. Turbulent


flow is noted in pulmonary artery. PA pulmonary artery.
Video 2, Loop 4: midesophageal long-axis view of left
ventricular (LV) outflow tract. Right ventricle is dilated and
hypertrophied. Characteristic of the LV morphology, anterior leaflet of the mitral valve is in fibrous continuity with
the pulmonic valve. Septal segments are seen bulging from
right to left into the LV outflow tract. RV right ventricle;
PA pulmonary artery; MV anterior leaflet of mitral valve.
Video 2, Loop 5: color Doppler examination reveals turbulence of subpulmonic flow.
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By Kent H. Rehfeldt, MD, Martin Stechert, MD, and Martin J. London, MD

In d-transposition of the great arteries (d-TGA), the aorta arises from the right ventricle (RV) and is located anterior and
to the right of the pulmonary artery that arises from the left ventricle (LV). Atrial switch procedures, such as the
Senning, redirect pulmonary venous return at the atrial level through the tricuspid valve, RV, and ultimately into the
systemic circulation.
Transesophageal echocardiography (TEE) is used to identify typical complications associated with atrial switch
operations, such as RV dysfunction, tricuspid regurgitation, systemic and pulmonary venous obstruction, and LV
outflow (subpulmonic) obstruction. TEE evaluation of the LV outflow tract and RV is performed by progressively
increasing the multiplane angle in the midesophageal (ME) position from 4-chamber to RV inflow-outflow and
subsequently LV long-axis views; transgastric short-axis and RV inflow views are also helpful. Pressure gradients are
measured using spectral Doppler.
In this case, ME LV long-axis imaging revealed ventricular septal bulging into the LV outflow tract, which combined with
the anatomic limitation imposed by the mitral-pulmonary fibrous continuity to create moderate subpulmonic
obstruction. The subpulmonic obstruction was confirmed by both color and spectral Doppler examination and
appropriate medical interventions were implemented including maintenance of euvolemia and afterload while avoiding
excessive inotropic stimulation.
Subpulmonic obstruction is not an uncommon finding in patients with d-TGA who are treated with atrial switch
procedures; prompt recognition using 2-dimensional TEE imaging as well as color and spectral Doppler interrogation
facilitate early medical intervention.

July 2010 Volume 111 Number 1

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