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Address correspondence to Madan Mohan Maddali, MD, Senior Consultant in Anesthesia, National Heart Center, Royal Hospital, P.B. No: 1331, P.C: 111, Seeb, Muscat, Sultanate of Oman.
A 10-MONTH-OLD BOY (weight: 5.8 kg; height: 66 cm; body mass index: 13) was taken
to the cardiac catheterization laboratory before the creation of a bidirectional Glenn
shunt. At 4 days of age, transthoracic echocardiography done for the evaluation of
persistent cyanosis revealed heterotaxy (left atrial isomerism), situs ambiguous with
levocardia, bilateral superior vena cava, atrial septal defect (9 mm), partial anomalous
right pulmonary venous return to the morphologic right atrium, discordant atrioventricular
connection and concordant ventriculoarterial connection, with a hypoplastic right
ventricle. The child underwent stenting of the patent ductus arteriosus and balloon
atrial septostomy on the 11th day of life. Pulmonary artery banding was done at the
age of 1 month. During the current admission, a computerized tomography scan revealed
the complex connections of the great vessels (Fig 1). A 4-chamber transesophageal echocardiography view displayed the morphologic left
atrium, which was on the right side, and multiple coronary artery fistulae opening
into an enlarged chamber located superiorly (Fig 2; Video 1). The atrial septal defect could be visualized in the bicaval view (Fig 3; Video 2). A modified midesophageal 4-chamber view displayed a large superior chamber,
as well as the atrial septal defect (Fig 4). Agitated saline was injected into a right upper extremity vein. The agitated saline
appeared first in the right internal jugular vein and then appeared in the upper atrial
chamber, right side atrium (morphologic left atrium), followed by an appearance in
the right ventricle (Fig 5, A-D; Video 3). Slight retraction of the probe from the 4-chamber view displayed
a right superior vena cava on the right side that was moving into close juxtaposition
with the morphologic left atrium (Fig 6; Video 4). Correlation with computerized tomography findings revealed the identity
of the structure seen by transesophageal echocardiography into which the right superior
vena cava and the coronary artery fistulae were draining. What is the diagnosis?
Fig. 1The (A) coronal and the (B) sagittal plane computerized tomography images depicting
a cannula in the left internal jugular vein and the cardiac structures. Ao, aorta;
ASD, atrial septal defect; CS, coronary sinus; LIJV, left internal jugular vein; LV,
left ventricle; mLA, morphologic left atrium; mRA, morphologic right atrium; PDA,
patent ductus arteriosus; LPA, left pulmonary artery; RIJV, right internal jugular
vein; RPA, right pulmonary artery.
Fig. 2(A) Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image with
(B) color Doppler displaying an enlarged chamber (white query sign) with multiple coronary artery fistulae opening into the chamber. mLA, morphologic
left atrium; RV, right ventricle; TV, tricuspid valve
Fig. 4Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image with
color-flow Doppler displaying the atrial septal defect and a large upper chamber marked
by white query sign.
Fig. 5Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image displaying
(A) the agitated saline echo contrast injected into an upper limb vein entering the
right superior vena cava, (B) the coronary sinus and the morphologic right atrium
on the right side, (C) the morphologic left atrium on the right side, and (D) the
right ventricle.
Fig. 6Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image displaying
the right superior vena cava and morphologic right atrium in close proximity. ASD,
atrial septal defect; LAA, morphologic left atrial appendage; mLeft atrium, morphologic
left atrium; mR, morphologic right atrium; RSVC, right superior vena cava; RV, right
ventricle; TV, tricuspid valve.
