Denise C. Joffe

High‐frequency micro‐ultrasound for vascular access in young children – a feasibility study by the High‐frequency UltraSound in Kids studY (HUSKY) group

Cannulation of small arteries and veins in young children can be challenging. Although anesthesiologists frequently use ultrasound for placement of central venous lines and nerve blocks, its use for cannulation of small, peripheral vessels is less helpful. Ultrasound systems (7‐15 MHz) currently used in clinical practice focus poorly at the sub‐10‐mm space and thus lack the resolution to allow accurate ultrasound‐guided cannulation of small vessels. High‐frequency micro‐ultrasound (HFMU) is a new technology that allows higher resolution (15–50 MHz) compared with conventional ultrasound. Limited human studies have been performed thus far with HFMU, and none have been performed in young children or for vascular access.

Coronary Sinus Atrial Septal Defect

A 69-yr-old previously healthy man with a history of atrial fibrillation and an atrial septal defect (ASD) presented for ASD closure and a Maze procedure. On transthoracic echocardiogram (TTE) the patient had a 13 mm defect in the inferoposterior part of the interatrial septum just above the tricuspid annulus with predominantly left-to-right shunting. An inferior sinus venosus ASD or a coronary sinus ASD was suspected. Because the coronary sinus was enlarged, the presence of a left superior vena cava (LSVC) was considered. There was biatrial enlargement and moderate right ventricular (RV) dilation. RV function was normal. The pulmonary veins were not well seen. Calculation of his pulmonary to systemic blood flow ratio was estimated at 1.8:1. On angiography, the defect was described as an inferior sinus venosus type. Intraoperative transesophageal echocardiography (TEE) was performed with the transducer positioned at the midesophageal long axis and aortic valve long axis views. A 13 mm defect at the level of the coronary sinus was demonstrated (Fig. 1A and Video 1; please see video clip available at www.anesthesia-analgesia. org). With the probe turned counterclockwise, rotated to 102 degrees and with color Doppler applied, predominantly left-to-right shunting into the coronary sinus and then the right atrium (RA) was seen (Fig. 1B and Video 2). The coronary sinus was normal in caliber ( 1 cm) in the midesophageal long axis view before its opening into the left atrium (LA). It then enlarged as it opened into the LA, demonstrating an unroofed coronary sinus (Video 1). All pulmonary veins emptied into the LA. There was biatrial, RV, and pulmonary artery enlargement. A contrast study performed by injecting agitated saline into the left arm was negative for a LSVC. All bubbles entered directly into the RA. The remainder of the septum was intact. A bilateral Maze procedure was performed, then the ASD was repaired. A defect from the coronary sinus into the LA was identified which measured 1 cm in diameter. This was repaired by patching or “putting a roof” on the coronary sinus using a piece of pericardium and allowing the coronary sinus effluent to drain normally into the RA. The patient was easily weaned off bypass. On TEE, no flow was seen across the atrial septum and the coronary sinus no longer opened into the LA. ASDs exist in several morphologic varieties that must be characterized, since they are often associated with co-existing congenital abnormalities (Table 1). In addition, the location and associated defects may affect the technique of repair. Isolated coronary sinus ASDs are exceedingly rare congenital cardiac defects. They make up 1% of ASDs. The defect is characterized by a deficiency in the tissue separating the coronary sinus from the LA. This results in partial or complete unroofing of the coronary sinus leading to a predominantly left-to-right shunt through the coronary sinus (LA to coronary sinus to RA). The orifice of the ostium is frequently large because of the increased flow. From the RA side, the defect is located at the level of the coronary sinus ostium and may also include some deficiency in atrial tissue around the ostium. From the LA side, the size can be variable depending on the degree of unroofing of the coronary sinus. In the RA, the location of an inferior sinus venosus defect and a coronary sinus ASD are in close proximity (near the inferior vena cava); however, the defects can be distinguished by the unroofed coronary sinus and additional associated lesions (Table 1). The coronary sinus is best visualized by TEE by starting from the high four chamber view and turning the image plane towards the right heart. The probe is then advanced until the coronary sinus is seen entering the RA adjacent to the tricuspid annulus. Rotation of the probe from 90 to 120 degrees provides a second view of the coronary sinus entering This article has supplementary material on the Web site: www.anesthesia-analgesia.org.

Perioperative morbidity in children with elastin arteriopathy.

Children with elastin arteriopathy (EA), the majority of whom have Williams–Beuren syndrome, are at high risk for sudden death. Case reports suggest that the risk of perioperative cardiac arrest and death is high, but none have reported the frequency or risk factors for morbidity and mortality in an entire cohort of children with EA undergoing anesthesia.

Transitional atrioventricular canal.

A 39-yr-old woman presented with worsening shortness of breath and decreasing exercise tolerance. She was diagnosed with a transitional atrioventricular (AV) canal, a form of congenital heart disease, at age of 25 yr when pregnant with her first child. At that time, she was advised that she would likely need surgery if she became symptomatic. The patient presented to our institution for repair of her defects. Intraoperative transesophageal echocardiography demonstrated mild to moderate right and left atrial and right ventricular dilation. In the midesophageal four-chamber view, a defect in the inferior part of the interatrial septum with predominately left to right low-velocity shunting was seen. The mitral leaflets were thick and tethered at their tips and a central jet of moderate mitral regurgitation (MR) was seen (see Video 1, Supplemental Digital Content 1, http://links.lww.com/AA/A3). In addition, a small jet of MR was seen to originate from the anterior mitral leaflet through a cleft (Fig. 1). In the transgastric basal short-axis view of the left ventricle, a large cleft was seen in the anterior mitral leaflet, dividing the leaflet into two (see Video 2, Supplemental Digital Content 2, http://links.lww.com/AA/A4) (Fig. 2). In the midesophageal aortic valve long-axis view, a chord was seen to arise from the anterior mitral leaflet and insert into the interventricular septum (Fig. 3). This aberrant chord did not result in left ventricular outflow tract (LVOT) obstruction. In the deep transgastric long-axis view, tricuspid valve tissue appeared to be closing what may have been a ventricular septal defect. The rest of the examination was unremarkable. The primum atrial septal defect (ASD) was closed with a patch of pericardium and the mitral valve (MV) was not repaired. With hemodynamic loading conditions similar to her preoperative state, the postpump transesophageal echocardiography demonstrated an overall decrease in the amount of MR, although there was a small increase in the MR through the cleft. No flow was seen through the ASD patch. A transitional canal consists of a primum ASD, a restrictive (small) ventricular septal defect, and a cleft MV. It is thought to result from the abnormal development of the endocardial cushions, which contributes to portions of the atrial and ventricular septum and the septal leaflets of the AV valves. It is considered to be in the spectrum between a primum ASD and a complete AV canal (Table 1). Patients are From the Department of Anesthesiology, University of Washington Medical Center, Seattle, Washington. Accepted for publication February 10, 2009. 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 journal’s Web site (www.anesthesia-analgesia.org). Address correspondence and reprint requests to Denise Joffe, MD, Department of Anesthesiology, Children’s Hospital and Regional Medical Center, 4800 Sand Point Way NE, Seattle, WA 98105. Address e-mail to denise.joffe@seattlechildrens.org. Copyright

Anesthetic Considerations and Management of Transposition of the Great Arteries

Transposition of the great arteries was once an almost uniformly fatal disease in infancy. Six decades of advances in surgical techniques, intraoperative care, and perioperative management have led to at least 90% of patients reaching adulthood, most with a good quality of life. This review summarizes medical and surgical decision making during the neonatal perioperative period, with a special emphasis on factors pertinent to the anesthetic evaluation and care during primary surgical repair of transposition of the great arteries. A review is also provided of anesthetic considerations for noncardiac surgery later in childhood or adulthood, for those survivors of the arterial switch operation, Rastelli procedure, Nikaidoh procedure, and the réparation á l’étage ventriculaire procedure.

An unusual presentation in a patient with Shone's anomaly.

A 23-yr-old woman with Shone’s anomaly presented to her cardiologist because of worsening exercise tolerance and a history of mitral regurgitation (MR). Her preoperative transthoracic echocardiogram demonstrated severe left ventricle (LV) dilation, global LV hypokinesis, and an ejection fraction of 44%. The left atrial size measured 4 cm. There was a parachute mitral valve (MV) with a large lateral papillary muscle and marked chordal redundancy with systolic anterior motion of the MV without prolapse or LV outflow tract obstruction. There was moderate (3 ) MR with an anteriorly directed jet, and blunted systolic flow in the pulmonary veins. The vena contracta measured 5 mm, and a dense continuous wave mitral regurgitant Doppler signal was seen. There was moderate tricuspid regurgitation. The estimated systolic pulmonary artery pressure was 50 mm Hg. The aortic valve was bicuspid with minimal aortic insufficiency and no aortic stenosis. A cardiac catheterization confirmed the echocardiogram findings and she was sent to the operating room for MV repair or replacement. Intraoperative transesophageal echocardiography (TEE) demonstrated moderate to severe MR with a central jet (Video 1; please see video clip available at www.anesthesia-analgesia.org). Her MV and subvalvulvar apparatus were extremely redundant. (Video 2; please see video clip available at www.anesthesiaanalgesia.org). The mitral annulus measured 3.8 cm. Doppler interrogation of mitral inflow velocities using pulse wave Doppler demonstrated an E wave A wave velocity, E wave velocity of 1.3 m/s, and a deceleration time (DT) of 32 ms (Fig. 1). Continuous wave Doppler demonstrated a mean transmitral pressure gradient of 1.6 m/s and a dense jet of MR (Fig. 2). TEE clearly demonstrated redundant MV tissue in the LV outflow tract without LV outflow tract obstruction. On surgical inspection, her chordae were noted to be matted together in a tangled web (Fig. 3). She had one papillary muscle connected to the anterolateral LV wall. She underwent a MV replacement with a #31 bileaflet valve prosthesis with preservation of the subvalvular apparatus. Shone’s anomaly is a syndrome of left heart obstructive lesions and MV disease. The components of the complex as originally described consist of coarctation of the aorta, subaortic stenosis, a parachute MV, and a supra annular mitral ring. In addition, these patients may have a component of endocardial fibroelastosis which manifests as restrictive ventricular function, and some have a degree of LV hypoplasia which can contribute to morbidity and mortality. In Shone’s anomaly, the MV may be a parachute valve and may also have features of congenital mitral stenosis. As detailed in a previous echocardiogram report, a parachute MV consists of a single papillary muscle that creates an abnormally sized orifice and interferes with normal MV function. In congenital mitral stenosis, the valve can be thick and dysplastic. Chordae are short, thick and fused and form an extensive web. This results in obliteration of the interchordal spaces causing mitral stenosis. MR may exist with these malformations but is not the major problem. Her transmitral Doppler flows were consistent with severe restrictive LV physiology. The E wave velocity was much larger than the A wave velocity and there was a rapid and steep decline in the velocity of flow with a DT of 32 ms. The short DT is consistent with restrictive LV physiology without This article has supplementary material on the Web site: www.anesthesia-analgesia.org.

Perioperative and Anesthetic Considerations in Total Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection (TAPVC) is a potentially devastating form of congenital heart disease in which all pulmonary blood flow returns to the systemic venous circulation rather than the left atrium. Anomalous pulmonary venous flow may be obstructed at birth, and affected infants present with severe cyanosis and poor cardiac output unresponsive to standard resuscitation with prostaglandin. Obstructed TAPVC remains one of the few indications for emergent neonatal cardiac surgery. This review will discuss the physiology and perioperative management of isolated TAPVC without associated cardiac lesions.

Perioperative and Anesthetic Considerations in Truncus Arteriosus

Truncus arteriosus is a congenital cardiac lesion in which failure of embryonic truncal septation results in a single semilunar valve and single arterial trunk providing both pulmonary and systemic circulations. Most patients with this lesion are symptomatic in the neonatal period with cyanosis and/or congestive heart failure and undergo complete repair in the first weeks of life. This review will focus on the anatomy, physiology, and perioperative anesthetic management of patients with truncus arteriosus.

Perioperative and Anesthetic Considerations in Atrioventricular Septal Defect

Atrioventricular septal defect results from a failure of normal endocardial cushion fusion during embryologic cardiac development. This developmental aberration results in defects in the atrial and/or ventricular septum and malformation of the atrioventricular valves. The pathophysiology of atrioventricular septal defect is variable, and ranges from mild left to right shunting similar to a simple atrial septal defect to complex single-ventricle heart disease. This review focuses on the spectrum of atrioventricular septal defect from partial to complete, without associated cardiac defects.

Perioperative and Anesthetic Considerations in Ebstein’s Anomaly

Ebstein’s anomaly is a complex and heterogeneous form of congenital heart disease characterized by malformation and apical displacement of the tricuspid valve leaflets. Patients may present at any time from the neonatal period to adulthood with symptoms ranging from cardiac failure and cyanosis to paroxysmal arrhythmias. Depending on the timing of presentation, various surgical options are available for the management of symptomatic patients. This review will discuss the perioperative and anesthetic management of patients with Ebstein’s anomaly with reference to the more common surgical approaches.